Materiały źródłowe

• #1 Pulmonary Atresia With Ventricular Septal Defect – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK562277/

  Pulmonary atresia with ventricular septal defect is a rare and complex congenital heart anomaly characterized by the underdevelopment or complete obstruction of the pulmonary valve, preventing blood flow from the right ventricle to the lungs. This condition is often associated with major aortopulmonary collateral arteries, which supply pulmonary circulation without a normal pulmonary artery. Although it shares similarities with tetralogy of Fallot, its anatomical variations make diagnosis and treatment challenging. Early detection through fetal ultrasound or postnatal echocardiography is crucial, as untreated cases can lead to severe cyanosis, heart failure, and life-threatening complications. Surgical intervention, often in multiple stages, is required to establish pulmonary blood flow and improve long-term survival.

• #2 Pulmonary Atresia With Ventricular Septal Defect – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/sites/books/NBK562277/

  Pulmonary atresia with ventricular septal defect is a rare and complex congenital heart anomaly characterized by the underdevelopment or complete obstruction of the pulmonary valve, preventing blood flow from the right ventricle to the lungs. This condition is often associated with major aortopulmonary collateral arteries, which supply pulmonary circulation without a normal pulmonary artery. […] Early detection through fetal ultrasound or postnatal echocardiography is crucial, as untreated cases can lead to severe cyanosis, heart failure, and life-threatening complications. Surgical intervention, often in multiple stages, is required to establish pulmonary blood flow and improve long-term survival. […] The clinical presentation of the PAVSD is highly variable depending on the degree of pulmonary atresia and MAPCAs. Most neonates present with cyanosis and cardiac murmur. Other common symptoms include the following: Central cyanosis may present as bluish discoloration of the face, particularly around the mouth and lips. In severe cases, it may be seen in peripheral limbs as well. Increased respiratory rate or shortness of breath due to poor blood oxygenation. This may not be evident at rest and only at exertion during crying or breastfeeding. Easy fatigability may present as a weak cry, tone loss, and poor breast latching. Failure to thrive and difficulty feeding are also symptoms. […] The physical exam may reveal central cyanosis, a holosystolic murmur at the left sternal border that may radiate to the back or axilla, a single accentuated second heart sound S2, a machine-like continuous murmur of PDA best heard at the upper precordium or the interscapular region on the back, a weak grip, a low weight for age, and lethargy. Peripheral edema, clubbing, and worsening cyanosis may indicate congestive heart failure (CHF), especially if presenting late in life. A small number of neonates/infants present with CHF early in life. They have large MAPCAs with excessive pulmonary blood flow, sometimes requiring mechanical ventilation. These patients require early complete repair, and outcomes are generally favorable because they often have well-developed pulmonary vasculature.

• #3 Pulmonary atresia with ventricular septal defect – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-atresia-ventricular-septum-defect/cdc-20396707

  Pulmonary atresia with ventricular septal defect is life-threatening. A baby with pulmonary atresia eventually doesn’t get enough oxygen. One or more procedures or surgeries are needed to fix the heart. […] Symptoms of pulmonary atresia with ventricular septal defect, also called PA-VSD, may appear at birth or very soon after. They can include: Blue or gray skin. This change may be harder or easier to see depending on skin color. Fast breathing or shortness of breath. Tiredness. Poor feeding. […] If your baby has symptoms of this condition after birth, call a healthcare professional right away.

• #4 Pulmonary atresia with ventricular septal defect | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/pulmonary-atresia-ventricular-septal-defect

  Symptoms of pulmonary atresia with ventricular septal defect, also called PA-VSD, may appear at birth or very soon after. They can include: […] A baby with pulmonary atresia eventually doesn’t get enough oxygen. One or more procedures or surgeries are needed to fix the heart. […] If your baby has symptoms of this condition after birth, call a healthcare professional right away. […] A baby with pulmonary atresia with ventricular septal defect, also called PA-VSD, needs treatment right away. Treatment may include one or more surgeries or procedures.

• #5 Pulmonary Atresia With Ventricular Septal Defect – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK562277/

  The clinical presentation of the PAVSD is highly variable depending on the degree of pulmonary atresia and MAPCAs. Most neonates present with cyanosis and cardiac murmur. Other common symptoms include the following: Central cyanosis may present as bluish discoloration of the face, particularly around the mouth and lips. In severe cases, it may be seen in peripheral limbs as well. Increased respiratory rate or shortness of breath due to poor blood oxygenation. This may not be evident at rest and only at exertion during crying or breastfeeding. Easy fatigability may present as a weak cry, tone loss, and poor breast latching. Failure to thrive and difficulty feeding are also symptoms. […] The physical exam may reveal central cyanosis, a holosystolic murmur at the left sternal border that may radiate to the back or axilla, a single accentuated second heart sound S2, a machine-like continuous murmur of PDA best heard at the upper precordium or the interscapular region on the back, a weak grip, a low weight for age, and lethargy. Peripheral edema, clubbing, and worsening cyanosis may indicate congestive heart failure (CHF), especially if presenting late in life. A small number of neonates/infants present with CHF early in life. They have large MAPCAs with excessive pulmonary blood flow, sometimes requiring mechanical ventilation. These patients require early complete repair, and outcomes are generally favorable because they often have well-developed pulmonary vasculature.

• #6 Pulmonary Atresia (PA) | Causes, Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/patients/child/encyclopedia/defects/pa

  Pulmonary atresia (PA) is a complicated congenital (present at birth) heart defect. It occurs due to abnormal development of the fetal heart in the first trimester of pregnancy. […] Cyanosis (blue color) due to low oxygen levels is often present right after birth. Depending on other structural differences in the heart, it could range from mild to severe. The degree of cyanosis is related to the presence of other defects that allow blood to mix. This includes a patent (open) ductus arteriosus as described above. As the ductus closes the cyanosis will increase. […] The following are the most common symptoms of pulmonary atresia. Each child may have different symptoms. Symptoms may include: cyanosis (blue color around the mouth or of the skin), fast breathing, difficulty breathing, irritability, lethargy (increased sleepiness), pale, cool or clammy skin.

• #7 Pulmonary atresia with ventricular septal defect – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-atresia-ventricular-septum-defect/cdc-20396707

  Pulmonary atresia with ventricular septal defect is life-threatening. A baby with pulmonary atresia eventually doesn’t get enough oxygen. One or more procedures or surgeries are needed to fix the heart. […] Symptoms of pulmonary atresia with ventricular septal defect, also called PA-VSD, may appear at birth or very soon after. They can include: Blue or gray skin. This change may be harder or easier to see depending on skin color. Fast breathing or shortness of breath. Tiredness. Poor feeding. […] If your baby has symptoms of this condition after birth, call a healthcare professional right away.

• #8 Pulmonary Atresia With Ventricular Septal Defect – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK562277/

  The clinical presentation of the PAVSD is highly variable depending on the degree of pulmonary atresia and MAPCAs. Most neonates present with cyanosis and cardiac murmur. Other common symptoms include the following: Central cyanosis may present as bluish discoloration of the face, particularly around the mouth and lips. In severe cases, it may be seen in peripheral limbs as well. Increased respiratory rate or shortness of breath due to poor blood oxygenation. This may not be evident at rest and only at exertion during crying or breastfeeding. Easy fatigability may present as a weak cry, tone loss, and poor breast latching. Failure to thrive and difficulty feeding are also symptoms. […] The physical exam may reveal central cyanosis, a holosystolic murmur at the left sternal border that may radiate to the back or axilla, a single accentuated second heart sound S2, a machine-like continuous murmur of PDA best heard at the upper precordium or the interscapular region on the back, a weak grip, a low weight for age, and lethargy. Peripheral edema, clubbing, and worsening cyanosis may indicate congestive heart failure (CHF), especially if presenting late in life. A small number of neonates/infants present with CHF early in life. They have large MAPCAs with excessive pulmonary blood flow, sometimes requiring mechanical ventilation. These patients require early complete repair, and outcomes are generally favorable because they often have well-developed pulmonary vasculature.

• #9 About Pulmonary Atresia | Congenital Heart Defects (CHDs) | CDC

  https://www.cdc.gov/heart-defects/about/pulmonary-atresia.html

  Babies born with pulmonary atresia will show symptoms at birth or very soon afterwards. They may have a bluish looking skin color, called cyanosis, because their blood doesn’t carry enough oxygen. Infants with pulmonary atresia can have additional symptoms such as: […] Problems breathing […] Ashen or bluish skin color […] Poor feeding […] Extreme sleepiness. […] Most babies with pulmonary atresia will need routine checkups with a heart doctor. A heart doctor will monitor a baby’s progress and check for other health conditions that might develop as they get older. As adults, they may need more surgery or medical care for other possible problems.

• #10 Pulmonary Atresia With Ventricular Septal Defect – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/sites/books/NBK562277/

  Pulmonary atresia with ventricular septal defect is a rare and complex congenital heart anomaly characterized by the underdevelopment or complete obstruction of the pulmonary valve, preventing blood flow from the right ventricle to the lungs. This condition is often associated with major aortopulmonary collateral arteries, which supply pulmonary circulation without a normal pulmonary artery. […] Early detection through fetal ultrasound or postnatal echocardiography is crucial, as untreated cases can lead to severe cyanosis, heart failure, and life-threatening complications. Surgical intervention, often in multiple stages, is required to establish pulmonary blood flow and improve long-term survival. […] The clinical presentation of the PAVSD is highly variable depending on the degree of pulmonary atresia and MAPCAs. Most neonates present with cyanosis and cardiac murmur. Other common symptoms include the following: Central cyanosis may present as bluish discoloration of the face, particularly around the mouth and lips. In severe cases, it may be seen in peripheral limbs as well. Increased respiratory rate or shortness of breath due to poor blood oxygenation. This may not be evident at rest and only at exertion during crying or breastfeeding. Easy fatigability may present as a weak cry, tone loss, and poor breast latching. Failure to thrive and difficulty feeding are also symptoms. […] The physical exam may reveal central cyanosis, a holosystolic murmur at the left sternal border that may radiate to the back or axilla, a single accentuated second heart sound S2, a machine-like continuous murmur of PDA best heard at the upper precordium or the interscapular region on the back, a weak grip, a low weight for age, and lethargy. Peripheral edema, clubbing, and worsening cyanosis may indicate congestive heart failure (CHF), especially if presenting late in life. A small number of neonates/infants present with CHF early in life. They have large MAPCAs with excessive pulmonary blood flow, sometimes requiring mechanical ventilation. These patients require early complete repair, and outcomes are generally favorable because they often have well-developed pulmonary vasculature.

• #11 Pulmonary Atresia: Symptoms, Causes and Treatment

  https://my.clevelandclinic.org/health/diseases/14779-pulmonary-atresia

  Symptoms of pulmonary atresia often appear within the first few hours or days of a baby’s life and may include: Rapid breathing. A bluish tinge to the skin, especially the lips, fingers and toes. Cool, pale or clammy skin. Breathing problems. Fatigue or tiredness. Irritability. Poor feeding. […] Without treatment, pulmonary atresia is fatal because it makes your oxygen level low. However, when your healthcare provider makes a diagnosis before or shortly after your baby’s birth, they can treat your newborn to improve their oxygen circulation. Your baby may need several surgeries at different ages to keep improving their situation. […] Without having surgery to fix pulmonary atresia with a ventricular septal defect, the survival rate is 50% at age 1 and 8% at 10 years of age. Most people don’t live into their 30s without surgery.

• #12 Pulmonary Atresia With Ventricular Septal Defect – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK562277/

  The clinical presentation of the PAVSD is highly variable depending on the degree of pulmonary atresia and MAPCAs. Most neonates present with cyanosis and cardiac murmur. Other common symptoms include the following: Central cyanosis may present as bluish discoloration of the face, particularly around the mouth and lips. In severe cases, it may be seen in peripheral limbs as well. Increased respiratory rate or shortness of breath due to poor blood oxygenation. This may not be evident at rest and only at exertion during crying or breastfeeding. Easy fatigability may present as a weak cry, tone loss, and poor breast latching. Failure to thrive and difficulty feeding are also symptoms. […] The physical exam may reveal central cyanosis, a holosystolic murmur at the left sternal border that may radiate to the back or axilla, a single accentuated second heart sound S2, a machine-like continuous murmur of PDA best heard at the upper precordium or the interscapular region on the back, a weak grip, a low weight for age, and lethargy. Peripheral edema, clubbing, and worsening cyanosis may indicate congestive heart failure (CHF), especially if presenting late in life. A small number of neonates/infants present with CHF early in life. They have large MAPCAs with excessive pulmonary blood flow, sometimes requiring mechanical ventilation. These patients require early complete repair, and outcomes are generally favorable because they often have well-developed pulmonary vasculature.

• #13 Partners in Care | Pulmonary Atresia With Ventricular Septal Defect…

  https://partnersincare.health/conditions/pulmonary-atresia-with-ventricular-septal-defect

  Symptoms of PA with VSD usually appear in the first few days of life. […] Symptoms of PA with VSD may include: Fast breathing, Poor feeding, Poor weight gain, Skin that appears pale or blue in color (cyanosis), Sleeping more than expected, Sweating during feeding.

• #14 SSA – POMS: DI 23022.585 – Pulmonary Atresia – 08/28/2020

  https://secure.ssa.gov/poms.nSf/lnx/0423022585!OpenDocument&Click=

  Pulmonary atresia is a rare congenital heart disease in which the pulmonary valve does not form properly. The pulmonary valve is an opening on the right side of the heart that regulates blood flow from the right ventricle (right side pumping chamber) to the lungs. Because of this defect, blood from the right side of the heart cannot go to the lungs to pick up oxygen. […] Infants born with this condition also tend to have multiple other cardiac conditions, such as poorly developed tricuspid valves, underdeveloped (hypoplastic) right ventricle, and abnormal blood vessels leading into the heart. Surgery is necessary shortly after birth to form a shunt from the systemic circulation to the pulmonary circulation in order to oxygenate the blood. […] The physical findings for this condition include: Bluish colored skin (cyanosis); Fast breathing; Fatigue; Poor feeding during nursing; Sweating during feeding; and Shortness of breath.

• #15 Pulmonary Atresia With Ventricular Septal Defect – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK562277/

  The clinical presentation of the PAVSD is highly variable depending on the degree of pulmonary atresia and MAPCAs. Most neonates present with cyanosis and cardiac murmur. Other common symptoms include the following: Central cyanosis may present as bluish discoloration of the face, particularly around the mouth and lips. In severe cases, it may be seen in peripheral limbs as well. Increased respiratory rate or shortness of breath due to poor blood oxygenation. This may not be evident at rest and only at exertion during crying or breastfeeding. Easy fatigability may present as a weak cry, tone loss, and poor breast latching. Failure to thrive and difficulty feeding are also symptoms. […] The physical exam may reveal central cyanosis, a holosystolic murmur at the left sternal border that may radiate to the back or axilla, a single accentuated second heart sound S2, a machine-like continuous murmur of PDA best heard at the upper precordium or the interscapular region on the back, a weak grip, a low weight for age, and lethargy. Peripheral edema, clubbing, and worsening cyanosis may indicate congestive heart failure (CHF), especially if presenting late in life. A small number of neonates/infants present with CHF early in life. They have large MAPCAs with excessive pulmonary blood flow, sometimes requiring mechanical ventilation. These patients require early complete repair, and outcomes are generally favorable because they often have well-developed pulmonary vasculature.

• #16 Pulmonary Atresia | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/pulmonary-atresia

  When there is not a ventricular septal defect, children with pulmonary atresia may also have: […] Pulmonary atresia symptoms include: […] Blue or purple tint to lips, skin and nails (cyanosis) […] Shortness of breath […] Difficulty feeding […] Heart murmur — the heart sounds abnormal when a doctor listens with a stethoscope […] In children with pulmonary atresia and ventricular septal defect (VSD), surgery will be necessary to close the VSD. Sometimes the pulmonary arteries are very small. In this case, multiple surgeries and cardiac catheterization may be needed.

• #17 Pulmonary Atresia With Ventricular Septal Defect – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/sites/books/NBK562277/

  Pulmonary atresia with ventricular septal defect is a rare and complex congenital heart anomaly characterized by the underdevelopment or complete obstruction of the pulmonary valve, preventing blood flow from the right ventricle to the lungs. This condition is often associated with major aortopulmonary collateral arteries, which supply pulmonary circulation without a normal pulmonary artery. […] Early detection through fetal ultrasound or postnatal echocardiography is crucial, as untreated cases can lead to severe cyanosis, heart failure, and life-threatening complications. Surgical intervention, often in multiple stages, is required to establish pulmonary blood flow and improve long-term survival. […] The clinical presentation of the PAVSD is highly variable depending on the degree of pulmonary atresia and MAPCAs. Most neonates present with cyanosis and cardiac murmur. Other common symptoms include the following: Central cyanosis may present as bluish discoloration of the face, particularly around the mouth and lips. In severe cases, it may be seen in peripheral limbs as well. Increased respiratory rate or shortness of breath due to poor blood oxygenation. This may not be evident at rest and only at exertion during crying or breastfeeding. Easy fatigability may present as a weak cry, tone loss, and poor breast latching. Failure to thrive and difficulty feeding are also symptoms. […] The physical exam may reveal central cyanosis, a holosystolic murmur at the left sternal border that may radiate to the back or axilla, a single accentuated second heart sound S2, a machine-like continuous murmur of PDA best heard at the upper precordium or the interscapular region on the back, a weak grip, a low weight for age, and lethargy. Peripheral edema, clubbing, and worsening cyanosis may indicate congestive heart failure (CHF), especially if presenting late in life. A small number of neonates/infants present with CHF early in life. They have large MAPCAs with excessive pulmonary blood flow, sometimes requiring mechanical ventilation. These patients require early complete repair, and outcomes are generally favorable because they often have well-developed pulmonary vasculature.

• #18 Orphanet: Pulmonary atresia with ventricular septal defect

  https://www.orpha.net/en/disease/detail/1207

  Pulmonary atresia with ventricular septal defect (PA-VSD) is a rare cyanotic congenital heart malformation characterized by underdevelopment of the right ventricular outflow tract and atresia of the pulmonary valve, ventricular septal defect (VSD) and pulmonary collateral vessels. Clinical features depend on the anatomic variability of the lesion and patients may be minimally symptomatic, severely cyanotic or may develop congestive heart failure. PA-VSD may represent a severe form of Tetralogy of Fallot. […] Clinical features depend on the anatomic variability of the lesion and patients may be minimally symptomatic, severely cyanotic or may develop congestive heart failure.

• #19 Pulmonary Atresia With Ventricular Septal Defect – MD Searchlight

  https://mdsearchlight.com/heart-health/pulmonary-atresia-with-ventricular-septal-defect/

  Pulmonary atresia with a ventricular septal defect (PAVSD) is a type of heart disease present at birth that can cause a blue or purple tinge to the skin (cyanosis). It happens when parts of the right side of the heart dont develop correctly, causing issues with the valve and muscly wall (pulmonary valve and trunk) that lead to the lungs, and creating a large hole in the muscular wall (ventricular septal defect) separating the hearts lower chambers. Also, the aorta (the hearts main artery) is often misplaced. […] The symptoms and severity of this heart condition can vary greatly, mostly depending on the development of the pulmonary valve and other blood vessels that may develop to make up for the poorly formed valve. […] Some patients with PAVSD are heavily reliant on a particular blood vessel (the arterial duct) for blood to flow to their lungs. These patients can turn blue (become cyanotic) when this blood vessel naturally closes after birth. If these patients are not diagnosed before birth, they may need a specific medicine called prostaglandin. Meanwhile, patients with enough extra blood vessels (major aortopulmonary collateral arteries or MAPCAs) can usually breathe comfortably at birth because these extra vessels ensure that blood reaches the lungs. […] Most newborns with PAVSD show signs of blue skin color and a heart murmur. Here are some of the common symptoms: Blue coloration of the face, especially around the mouth and lips due to poor oxygen supply to the bodys tissues. In severe cases, this can also be seen in the arms and legs. An increased breathing rate or difficulty breathing because of poor blood oxygen level. Sometimes, this isnt noticeable when the baby is resting but becomes evident during crying or breastfeeding. The baby may tire easily, demonstrated by a weak cry or having difficulty breastfeeding due to weakness and lack of energy. Failure to grow or gain weight as expected. […] A few newborns and infants can develop heart failure early in life if they have large MAPCAs leading to excessive blood flow to the lungs. These babies sometimes need mechanical assistance to breathe and should have their heart defect repaired as soon as possible.

• #20 Pulmonary Atresia with Ventricular Septal Defect, and Right Ventricle-to-Pulmonary Artery Conduits | Thoracic Key

  https://thoracickey.com/pulmonary-atresia-with-ventricular-septal-defect-and-right-ventricle-to-pulmonary-artery-conduits/

  Pulmonary atresia with ventricular septal defect (PAVSD) is a congenital cardiac malformation characterized by discontinuity of blood flow from the right ventricle to the pulmonary arteries, a ventricular septal defect (VSD) resulting from anterior deviation of the infundibular (conal) septum, and an overriding aorta. […] The natural history of PAVSD varies significantly depending on the pulmonary artery morphology. Patients presenting with PAVSD that has ductal-dependent pulmonary blood flow experience profound fatal cyanosis in the neonatal period as the ductus begins to close. On the other hand, patients with MAPCAs with mild stenosis of collateral vessels and balanced pulmonary blood flow may exhibit only mild cyanosis. These patients may survive into adolescence or adulthood without repair, although their life expectancy is significantly shorter than that of the normal population.

• #21 Pulmonary Atresia With Ventricular Septal Defect – MD Searchlight

  https://mdsearchlight.com/heart-health/pulmonary-atresia-with-ventricular-septal-defect/

  Pulmonary atresia with a ventricular septal defect (PAVSD) is a type of heart disease present at birth that can cause a blue or purple tinge to the skin (cyanosis). It happens when parts of the right side of the heart dont develop correctly, causing issues with the valve and muscly wall (pulmonary valve and trunk) that lead to the lungs, and creating a large hole in the muscular wall (ventricular septal defect) separating the hearts lower chambers. Also, the aorta (the hearts main artery) is often misplaced. […] The symptoms and severity of this heart condition can vary greatly, mostly depending on the development of the pulmonary valve and other blood vessels that may develop to make up for the poorly formed valve. […] Some patients with PAVSD are heavily reliant on a particular blood vessel (the arterial duct) for blood to flow to their lungs. These patients can turn blue (become cyanotic) when this blood vessel naturally closes after birth. If these patients are not diagnosed before birth, they may need a specific medicine called prostaglandin. Meanwhile, patients with enough extra blood vessels (major aortopulmonary collateral arteries or MAPCAs) can usually breathe comfortably at birth because these extra vessels ensure that blood reaches the lungs. […] Most newborns with PAVSD show signs of blue skin color and a heart murmur. Here are some of the common symptoms: Blue coloration of the face, especially around the mouth and lips due to poor oxygen supply to the bodys tissues. In severe cases, this can also be seen in the arms and legs. An increased breathing rate or difficulty breathing because of poor blood oxygen level. Sometimes, this isnt noticeable when the baby is resting but becomes evident during crying or breastfeeding. The baby may tire easily, demonstrated by a weak cry or having difficulty breastfeeding due to weakness and lack of energy. Failure to grow or gain weight as expected. […] A few newborns and infants can develop heart failure early in life if they have large MAPCAs leading to excessive blood flow to the lungs. These babies sometimes need mechanical assistance to breathe and should have their heart defect repaired as soon as possible.

• #22 Pulmonary Atresia with Ventricular Septal Defect, and Right Ventricle-to-Pulmonary Artery Conduits | Thoracic Key

  https://thoracickey.com/pulmonary-atresia-with-ventricular-septal-defect-and-right-ventricle-to-pulmonary-artery-conduits/

  The clinical presentation also varies according to pulmonary artery morphology. Patients with ductal-dependent pulmonary circulation present with profound cyanosis during the first several weeks of life as the ductus begins to close. Children with aortopulmonary collaterals may present in childhood with cyanosis. Patients with large, unrestrictive collaterals may present with heart failure symptomsfailure to thrive, tachypnea, or feeding intolerance.

• #23 Pulmonary Atresia With Ventricular Septal Defect – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK562277/

  The clinical presentation of the PAVSD is highly variable depending on the degree of pulmonary atresia and MAPCAs. Most neonates present with cyanosis and cardiac murmur. Other common symptoms include the following: Central cyanosis may present as bluish discoloration of the face, particularly around the mouth and lips. In severe cases, it may be seen in peripheral limbs as well. Increased respiratory rate or shortness of breath due to poor blood oxygenation. This may not be evident at rest and only at exertion during crying or breastfeeding. Easy fatigability may present as a weak cry, tone loss, and poor breast latching. Failure to thrive and difficulty feeding are also symptoms. […] The physical exam may reveal central cyanosis, a holosystolic murmur at the left sternal border that may radiate to the back or axilla, a single accentuated second heart sound S2, a machine-like continuous murmur of PDA best heard at the upper precordium or the interscapular region on the back, a weak grip, a low weight for age, and lethargy. Peripheral edema, clubbing, and worsening cyanosis may indicate congestive heart failure (CHF), especially if presenting late in life. A small number of neonates/infants present with CHF early in life. They have large MAPCAs with excessive pulmonary blood flow, sometimes requiring mechanical ventilation. These patients require early complete repair, and outcomes are generally favorable because they often have well-developed pulmonary vasculature.

• #24 Pulmonary Atresia With Ventricular Septal Defect – MD Searchlight

  https://mdsearchlight.com/heart-health/pulmonary-atresia-with-ventricular-septal-defect/

  Pulmonary atresia with a ventricular septal defect (PAVSD) is a type of heart disease present at birth that can cause a blue or purple tinge to the skin (cyanosis). It happens when parts of the right side of the heart dont develop correctly, causing issues with the valve and muscly wall (pulmonary valve and trunk) that lead to the lungs, and creating a large hole in the muscular wall (ventricular septal defect) separating the hearts lower chambers. Also, the aorta (the hearts main artery) is often misplaced. […] The symptoms and severity of this heart condition can vary greatly, mostly depending on the development of the pulmonary valve and other blood vessels that may develop to make up for the poorly formed valve. […] Some patients with PAVSD are heavily reliant on a particular blood vessel (the arterial duct) for blood to flow to their lungs. These patients can turn blue (become cyanotic) when this blood vessel naturally closes after birth. If these patients are not diagnosed before birth, they may need a specific medicine called prostaglandin. Meanwhile, patients with enough extra blood vessels (major aortopulmonary collateral arteries or MAPCAs) can usually breathe comfortably at birth because these extra vessels ensure that blood reaches the lungs. […] Most newborns with PAVSD show signs of blue skin color and a heart murmur. Here are some of the common symptoms: Blue coloration of the face, especially around the mouth and lips due to poor oxygen supply to the bodys tissues. In severe cases, this can also be seen in the arms and legs. An increased breathing rate or difficulty breathing because of poor blood oxygen level. Sometimes, this isnt noticeable when the baby is resting but becomes evident during crying or breastfeeding. The baby may tire easily, demonstrated by a weak cry or having difficulty breastfeeding due to weakness and lack of energy. Failure to grow or gain weight as expected. […] A few newborns and infants can develop heart failure early in life if they have large MAPCAs leading to excessive blood flow to the lungs. These babies sometimes need mechanical assistance to breathe and should have their heart defect repaired as soon as possible.

• #25 Pulmonary atresia with ventricular septal defect – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-atresia-ventricular-septum-defect/cdc-20396707

  Pulmonary atresia with ventricular septal defect is life-threatening. A baby with pulmonary atresia eventually doesn’t get enough oxygen. One or more procedures or surgeries are needed to fix the heart. […] Symptoms of pulmonary atresia with ventricular septal defect, also called PA-VSD, may appear at birth or very soon after. They can include: Blue or gray skin. This change may be harder or easier to see depending on skin color. Fast breathing or shortness of breath. Tiredness. Poor feeding. […] If your baby has symptoms of this condition after birth, call a healthcare professional right away.

• #26 Pulmonary atresia with ventricular septal defect | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/pulmonary-atresia-with-ventricular-septal-defect?content_id=CON-20380877

  Pulmonary atresia with ventricular septal defect is life-threatening. A baby with pulmonary atresia eventually doesn’t get enough oxygen. […] Symptoms of pulmonary atresia with ventricular septal defect, also called PA-VSD, may appear at birth or very soon after. They can include: Blue or gray skin. This change may be harder or easier to see depending on skin color. Fast breathing or shortness of breath. Tiredness. Poor feeding. […] If your baby has symptoms of this condition after birth, call a healthcare professional right away. […] A baby with pulmonary atresia with ventricular septal defect, also called PA-VSD, needs treatment right away. Treatment may include one or more surgeries or procedures.

• #27 Pulmonary Atresia With Ventricular Septal Defect – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK562277/

  Without surgical correction, about 50% of patients die within the first 2 years of life, and 20-year survival is around 10%. With proper treatment and follow-up, 65% of patients alive at 1 year can live beyond the age of 10. One high-volume center estimated operative mortality at 1.5% to 8%, depending on the procedure performed.

• #28 Pulmonary Atresia With Ventricular Septal Defect – MD Searchlight

  https://mdsearchlight.com/heart-health/pulmonary-atresia-with-ventricular-septal-defect/

  Without proper surgical intervention, around half of patients do not survive past their first 2 years of life, and only around 10% live up to or beyond 20 years. However, with appropriate treatment and regular follow-up care, the outlook improves significantly. About 65% of patients who are alive one year after treatment can live past the age of 10.

• #29 Pulmonary Atresia With Ventricular Septal Defect – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/sites/books/NBK562277/

  Without surgical correction, about 50% of patients die within the first 2 years of life, and 20-year survival is around 10%. With proper treatment and follow-up, 65% of patients alive at 1 year can live beyond the age of 10. […] One high-volume center estimated operative mortality at 1.5% to 8%, depending on the procedure performed.

• #30 Pulmonary Atresia: Symptoms, Causes and Treatment

  https://my.clevelandclinic.org/health/diseases/14779-pulmonary-atresia

  Symptoms of pulmonary atresia often appear within the first few hours or days of a baby’s life and may include: Rapid breathing. A bluish tinge to the skin, especially the lips, fingers and toes. Cool, pale or clammy skin. Breathing problems. Fatigue or tiredness. Irritability. Poor feeding. […] Without treatment, pulmonary atresia is fatal because it makes your oxygen level low. However, when your healthcare provider makes a diagnosis before or shortly after your baby’s birth, they can treat your newborn to improve their oxygen circulation. Your baby may need several surgeries at different ages to keep improving their situation. […] Without having surgery to fix pulmonary atresia with a ventricular septal defect, the survival rate is 50% at age 1 and 8% at 10 years of age. Most people don’t live into their 30s without surgery.

• #31 Pulmonary atresia with ventricular septal defect – Wikipedia

  https://en.wikipedia.org/wiki/Pulmonary_atresia_with_ventricular_septal_defect

  Pulmonary atresia with ventricular septal defect is a rare birth defect characterized by pulmonary valve atresia occurring alongside a defect on the right ventricular outflow tract. The spectrum of symptoms exhibited by children with this condition depends on the severity of the condition, while some barely show symptoms, others might develop complications such as congestive heart failure. In symptomatic children, symptoms become apparent soon after birth, these usually consist of the following: Cyanosis, Breathing difficulties, Feeding difficulties, Exhaustion while being fed, Heart murmur, Excessive daytime sleepiness, Sticky skin. Children with this condition are at a higher risk of developing the following complications: Failure to thrive, Recurrent chest infections, Endocarditis, Epilepsy, Stroke, Arrhythmia, Heart failure, Premature death. Without treatment, it is a highly life-threatening condition, so prognosis is poor. Life expectancy for untreated children with PAVSD is 10 years. Survival rates for untreated people with this defect have been reported to be 50% at the tenth decade and 10% at the twentieth decade, and out of these untreated patients, those who do not have major aortopulmonary arteries have a higher chance of living to their 30s than those who do have them, as the latter have a 40% chance of surviving to the tenth decade and a 20% chance of doing so to the thirtieth decade. Prognosis after surgical intervention is generally good.

• #32 Pulmonary atresia with ventricular septal defect – Wikipedia

  https://en.wikipedia.org/wiki/Pulmonary_atresia_with_ventricular_septal_defect

  Pulmonary atresia with ventricular septal defect is a rare birth defect characterized by pulmonary valve atresia occurring alongside a defect on the right ventricular outflow tract. The spectrum of symptoms exhibited by children with this condition depends on the severity of the condition, while some barely show symptoms, others might develop complications such as congestive heart failure. In symptomatic children, symptoms become apparent soon after birth, these usually consist of the following: Cyanosis, Breathing difficulties, Feeding difficulties, Exhaustion while being fed, Heart murmur, Excessive daytime sleepiness, Sticky skin. Children with this condition are at a higher risk of developing the following complications: Failure to thrive, Recurrent chest infections, Endocarditis, Epilepsy, Stroke, Arrhythmia, Heart failure, Premature death. Without treatment, it is a highly life-threatening condition, so prognosis is poor. Life expectancy for untreated children with PAVSD is 10 years. Survival rates for untreated people with this defect have been reported to be 50% at the tenth decade and 10% at the twentieth decade, and out of these untreated patients, those who do not have major aortopulmonary arteries have a higher chance of living to their 30s than those who do have them, as the latter have a 40% chance of surviving to the tenth decade and a 20% chance of doing so to the thirtieth decade. Prognosis after surgical intervention is generally good.

• #33 SSA – POMS: DI 23022.585 – Pulmonary Atresia – 08/28/2020

  https://secure.ssa.gov/poms.nSf/lnx/0423022585!OpenDocument&Click=

  A diagnosis of pulmonary atresia is usually made shortly after birth. Disease progression is variable based on the severity of the congenital heart defect and the response to medication and surgical interventions. […] Infants surviving into childhood and adults may develop problems with their heart functioning later in life due to congestive heart failure, angina, arrhythmias, cyanosis, and sudden death.

• #34 Pulmonary atresia: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/001091.htm

  Symptoms most often occur in the first few hours of life, although it may take up to a few days. […] Symptoms may include: […] Bluish color to the skin (cyanosis) due to low oxygen level in the blood […] Fast breathing […] Fatigue […] Poor eating habits (babies may get tired while nursing or sweat during feedings) […] Shortness of breath.

• #35 Pulmonary Atresia – Seattle Children’s

  https://www.seattlechildrens.org/conditions/pulmonary-atresia/

  Most babies with pulmonary atresia show symptoms during the first few hours after birth. They may be diagnosed after having a pulse oximetry screening in their birth hospital when they are about a day old. In some babies, it may take a few days for symptoms to appear. […] If your baby has pulmonary atresia symptoms, they may have these: Skin may look blue or purple tinged, mottled (different shades or colors), grayish or paler than usual; the lips, mouth, gums, fingernails or toenails may look bluish (cyanosis) […] Fast breathing […] Working hard to breathe […] Tiring easily while feeding.

• #36 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Pulmonary-Atresia-Congenital-Heart-Defect.aspx

  Noticeable symptoms in a newborn with pulmonary atresia can become evident within a few hours of birth or they may be delayed for several days, depending on the type and severity of the condition. The symptoms could include: Cyanosis or bluish gray tinged skin, lips or nails […] Shortness of breath or quick breathing […] Getting tired easily or being lethargic […] Not feeding well or getting fatigued while nursing […] Clammy and sweaty skin that is cool to the touch. A number of complications may arise from this condition. The baby will be at risk for seizures, strokes, or heart failure. The baby may also experience delayed growth and development, or infectious endocarditis. It is imperative that pulmonary atresia is identified as soon as possible for the baby to survive. If not treated the condition will prove fatal.

• #37 Pulmonary Atresia With Ventricular Septal Defect – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/sites/books/NBK562277/

  Pulmonary atresia with ventricular septal defect is a rare and complex congenital heart anomaly characterized by the underdevelopment or complete obstruction of the pulmonary valve, preventing blood flow from the right ventricle to the lungs. This condition is often associated with major aortopulmonary collateral arteries, which supply pulmonary circulation without a normal pulmonary artery. […] Early detection through fetal ultrasound or postnatal echocardiography is crucial, as untreated cases can lead to severe cyanosis, heart failure, and life-threatening complications. Surgical intervention, often in multiple stages, is required to establish pulmonary blood flow and improve long-term survival. […] The clinical presentation of the PAVSD is highly variable depending on the degree of pulmonary atresia and MAPCAs. Most neonates present with cyanosis and cardiac murmur. Other common symptoms include the following: Central cyanosis may present as bluish discoloration of the face, particularly around the mouth and lips. In severe cases, it may be seen in peripheral limbs as well. Increased respiratory rate or shortness of breath due to poor blood oxygenation. This may not be evident at rest and only at exertion during crying or breastfeeding. Easy fatigability may present as a weak cry, tone loss, and poor breast latching. Failure to thrive and difficulty feeding are also symptoms. […] The physical exam may reveal central cyanosis, a holosystolic murmur at the left sternal border that may radiate to the back or axilla, a single accentuated second heart sound S2, a machine-like continuous murmur of PDA best heard at the upper precordium or the interscapular region on the back, a weak grip, a low weight for age, and lethargy. Peripheral edema, clubbing, and worsening cyanosis may indicate congestive heart failure (CHF), especially if presenting late in life. A small number of neonates/infants present with CHF early in life. They have large MAPCAs with excessive pulmonary blood flow, sometimes requiring mechanical ventilation. These patients require early complete repair, and outcomes are generally favorable because they often have well-developed pulmonary vasculature.

• #38 Pulmonary atresia | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/pulmonary-atresia

  Symptoms of pulmonary atresia may be seen soon after birth. They can include: […] Blue or gray skin, lips or fingernails due to low oxygen levels. Depending on the skin color, these changes may be harder or easier to see. […] Fast breathing or shortness of breath. […] Tiring easily. […] Not feeding well. […] Pulmonary atresia is most often found soon after birth. If your baby has symptoms of pulmonary atresia after you’ve left the hospital, get medical help right away. […] Without treatment, pulmonary atresia most often leads to death. After surgery for pulmonary atresia, babies need regular health checkups throughout their lives to watch for complications.

• #39 Pulmonary atresia | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/pulmonary-atresia?content_id=CON-20155225

  Symptoms of pulmonary atresia may be seen soon after birth. They can include: […] Blue or gray skin, lips or fingernails due to low oxygen levels. Depending on the skin color, these changes may be harder or easier to see. […] Fast breathing or shortness of breath. […] Tiring easily. […] Not feeding well. […] Pulmonary atresia is a life-threatening condition that needs emergency treatment. Treatment includes surgery to repair the heart and medicines to help the heart work better. […] Without treatment, pulmonary atresia most often leads to death. After surgery for pulmonary atresia, babies need regular health checkups throughout their lives to watch for complications.

• #40 Pulmonary Atresia: Causes, Symptoms, and Treatment – Longmore Clinic

  https://longmoreclinic.org/pulmonary-atresia-causes-symptoms-and-treatment/

  Pulmonary atresia can occur in various forms: […] Pulmonary Atresia with Ventricular Septal Defect (PA-VSD): The pulmonary valve is absent or narrowed, and there is a hole in the ventricular septum, allowing blood to mix between the hearts chambers. […] Symptoms of pulmonary atresia can appear shortly after birth: […] Cyanosis: A blue or purple tint to the skin, lips, and nails due to low oxygen levels in the blood. […] Breathing Difficulties: Shortness of breath, rapid breathing, or labored breathing. […] Fatigue: Tiredness and fatigue during feeding or activity. […] Poor Weight Gain: Difficulty gaining weight and slower growth. […] Heart Murmur: An abnormal heart sound detected during a physical examination. […] If your baby shows signs of pulmonary atresia, such as cyanosis or breathing difficulties, seek medical attention immediately. Early diagnosis and treatment are crucial for improving outcomes.

• #41 Pulmonary Atresia (Tetralogy of Fallot) | Connecticut Children’s

  https://www.connecticutchildrens.org/specialties-conditions/cardiology/conditions/pulmonary-atresia-tetralogy-fallot

  Pulmonary atresia is usually detected before birth, or very soon after a child is born. Here are signs that a newborn may have pulmonary atresia. […] Bluish or greyish lips, skin and nails […] Heart murmur […] Trouble breathing […] Sweaty or clammy skin […] Feeding problems […] Low energy and activity. […] Patients born with pulmonary atresia need care that continues throughout their life, often including additional surgeries or catheter procedures to replace the donor valve and artery.

• #42 Pulmonary Atresia (PA) – Children’s Hospital of Orange County

  https://choc.org/heart/congenital-heart-defects/pulmonary-atresia-pa/

  Pulmonary atresia (PA) is a heart defect that occurs due to abnormal development of the fetal heart during the first eight weeks of pregnancy. […] Symptoms can be noted shortly after birth or several weeks later as the ductus arteriosus closes. The most obvious symptom is blue, or cyanotic, skin in a newborn. […] Although each child may experience symptoms differently, the following are the most common symptoms of pulmonary atresia: Rapid breathing, Difficulty breathing, Poor feeding, Exhaustion, Pale, cool or clammy skin, Blue color of the lips or skin. […] The symptoms of pulmonary atresia may resemble other medical conditions or heart problems, so it is very important to contact the child’s physician if you suspect the child may be experiencing any of these symptoms. […] Because of the low amount of oxygen provided to the body, pulmonary atresia is a heart problem that can ultimately result in cyanosis, or a blue color to the skin from lack of oxygen.

• #43 Pulmonary Atresia | Phoenix Children’s Hospital

  https://phoenixchildrens.org/specialties-conditions/pulmonary-atresia

  Symptoms may happen shortly after birth or later as the ductus arteriosus closes. The most clear symptom is a bluish color of the skin (cyanosis) in a newborn. […] These are other common symptoms: Fast or troubled breathing, Trouble feeding, Weakness and sleepiness, Pale, cool, or moist skin, Seizures. […] Because the blood doesn’t get enough oxygen, every cell in the baby’s body gets less oxygen. A newborn baby with PA cant live long without treatment. […] Without surgery, the heart cant pump oxygenated blood to the body and cant support life.

• #44 Pulmonary Atresia | Phoenix Children’s Hospital

  https://phoenixchildrens.org/specialties-conditions/pulmonary-atresia

  Symptoms may happen shortly after birth or later as the ductus arteriosus closes. The most clear symptom is a bluish color of the skin (cyanosis) in a newborn. […] These are other common symptoms: Fast or troubled breathing, Trouble feeding, Weakness and sleepiness, Pale, cool, or moist skin, Seizures. […] Because the blood doesn’t get enough oxygen, every cell in the baby’s body gets less oxygen. A newborn baby with PA cant live long without treatment. […] Without surgery, the heart cant pump oxygenated blood to the body and cant support life.

• #45 Pulmonary atresia | Children’s Wisconsin

  https://childrenswi.org/medical-care/herma-heart/conditions/pulmonary-atresia

  Symptoms will be noted shortly after birth. The obvious indication of PA is a newborn who becomes cyanotic (blue) in the transitional first day of life when the maternal source of oxygen (from the placenta) is removed. The degree of cyanosis is related to the presence of other defects that allow blood to mix, including a patent (open) ductus arteriosus. […] The following are the most common symptoms of pulmonary atresia. However, each child may experience symptoms differently. Symptoms may include: Rapid breathing, Difficulty breathing, Irritability, Lethargy, Pale, cool, or clammy skin. […] Because of the low amount of oxygen provided to the body, pulmonary atresia is a heart problem that is labeled „blue-baby syndrome.”

• #46 Pulmonary atresia

  https://www.aboutkidshealth.ca/pulmonary-atresia

  With pulmonary atresia, the pulmonary valve cannot open properly, meaning blood can’t flow from the right ventricle to the lungs as it normally would. […] Main symptoms are a murmur, cyanosis, rapid breathing or trouble breathing, irritability, low energy, and clammy skin. […] The degree of symptoms depends on the severity of the defect and the presence of other defects. […] Surgical repair of this condition has an excellent outcome, though this depends on whether the surgery was corrective or palliative. Full repair enables most children to lead full, normal lives.

• #47 Pulmonary Atresia (Tetralogy of Fallot) | Connecticut Children’s

  https://www.connecticutchildrens.org/specialties-conditions/cardiology/conditions/pulmonary-atresia-tetralogy-fallot

  Pulmonary atresia is usually detected before birth, or very soon after a child is born. Here are signs that a newborn may have pulmonary atresia. […] Bluish or greyish lips, skin and nails […] Heart murmur […] Trouble breathing […] Sweaty or clammy skin […] Feeding problems […] Low energy and activity. […] Patients born with pulmonary atresia need care that continues throughout their life, often including additional surgeries or catheter procedures to replace the donor valve and artery.

• #48 What is Pulmonary Atresia? – Healthis Assistance

  https://healthis.com.tr/en/what-is-pulmonary-atresia/

  Pulmonary atresia becomes apparent within the first few hours or days of life. One of the most common symptoms is cyanosis, a bluish tint to the lips and skin caused by low oxygen levels. Babies may also have difficulty breathing, feeding problems, and a rapid heart rate. Depending on the severity of the condition, these symptoms can become more pronounced. […] In newborns, the symptoms of pulmonary atresia usually appear quickly. Babies may struggle to breathe, and their skin may feel cold and clammy. Additionally, poor oxygen levels can lead to feeding difficulties and developmental delays, such as failure to gain weight. […] Children and adolescents who have untreated or inadequately treated pulmonary atresia may display different symptoms as they grow. These can include shortness of breath, fatigue, and an inability to tolerate physical activity. These issues tend to become more noticeable during physical exertion and can also affect the child’s social interactions.

• #49 Pulmonary Atresia: Overview, Causes, Treatment

  https://www.healthline.com/health/pulmonary-atresia

  Pulmonary atresia presents a unique challenge in pediatric cardiology in which the intricate pathways of the heart can be disrupted from the very start. The infants heart finds it difficult to send oxygen-rich blood to the lungs, which is crucial for sustaining life. […] Symptoms of pulmonary atresia can vary depending on the severity of the condition and the presence of other heart irregularities. Common symptoms may include: bluish discoloration of the skin, lips, and nail beds (cyanosis), rapid breathing or shortness of breath, fatigue or weakness, especially during feeding or exertion, poor growth or development, irritability or fussiness, heart murmur (an abnormal sound heard during a heartbeat). […] In severe cases, newborns may develop severe cyanosis and respiratory distress shortly after birth. Symptoms can worsen over time if left untreated, leading to heart failure and other complications.

• #50 Pulmonary Atresia With Ventricular Septal Defect – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK562277/

  The clinical presentation of the PAVSD is highly variable depending on the degree of pulmonary atresia and MAPCAs. Most neonates present with cyanosis and cardiac murmur. Other common symptoms include the following: Central cyanosis may present as bluish discoloration of the face, particularly around the mouth and lips. In severe cases, it may be seen in peripheral limbs as well. Increased respiratory rate or shortness of breath due to poor blood oxygenation. This may not be evident at rest and only at exertion during crying or breastfeeding. Easy fatigability may present as a weak cry, tone loss, and poor breast latching. Failure to thrive and difficulty feeding are also symptoms. […] The physical exam may reveal central cyanosis, a holosystolic murmur at the left sternal border that may radiate to the back or axilla, a single accentuated second heart sound S2, a machine-like continuous murmur of PDA best heard at the upper precordium or the interscapular region on the back, a weak grip, a low weight for age, and lethargy. Peripheral edema, clubbing, and worsening cyanosis may indicate congestive heart failure (CHF), especially if presenting late in life. A small number of neonates/infants present with CHF early in life. They have large MAPCAs with excessive pulmonary blood flow, sometimes requiring mechanical ventilation. These patients require early complete repair, and outcomes are generally favorable because they often have well-developed pulmonary vasculature.

• #51 Orphanet: Pulmonary atresia with ventricular septal defect

  https://www.orpha.net/en/disease/detail/1207

  Pulmonary atresia with ventricular septal defect (PA-VSD) is a rare cyanotic congenital heart malformation characterized by underdevelopment of the right ventricular outflow tract and atresia of the pulmonary valve, ventricular septal defect (VSD) and pulmonary collateral vessels. Clinical features depend on the anatomic variability of the lesion and patients may be minimally symptomatic, severely cyanotic or may develop congestive heart failure. PA-VSD may represent a severe form of Tetralogy of Fallot. […] Clinical features depend on the anatomic variability of the lesion and patients may be minimally symptomatic, severely cyanotic or may develop congestive heart failure.

• #52 Pulmonary Atresia with Ventricular Septal Defect Symptoms, Doctors, Treatments, Advances & More | MediFind

  https://www.medifind.com/conditions/pulmonary-atresia-with-ventricular-septal-defect/4495

  Pulmonary atresia with ventricular septal defect (PA-VSD) is a rare cyanotic congenital heart malformation characterized by underdevelopment of the right ventricular outflow tract and atresia of the pulmonary valve, ventricular septal defect (VSD) and pulmonary collateral vessels. Clinical features depend on the anatomic variability of the lesion and patients may be minimally symptomatic, severely cyanotic or may develop congestive heart failure. […] PA-VSD may represent a severe form of Tetralogy of Fallot.

• #53 Pulmonary Atresia with Ventricular Septal Defect, and Right Ventricle-to-Pulmonary Artery Conduits | Thoracic Key

  https://thoracickey.com/pulmonary-atresia-with-ventricular-septal-defect-and-right-ventricle-to-pulmonary-artery-conduits/

  Pulmonary atresia with ventricular septal defect (PAVSD) is a congenital cardiac malformation characterized by discontinuity of blood flow from the right ventricle to the pulmonary arteries, a ventricular septal defect (VSD) resulting from anterior deviation of the infundibular (conal) septum, and an overriding aorta. […] The natural history of PAVSD varies significantly depending on the pulmonary artery morphology. Patients presenting with PAVSD that has ductal-dependent pulmonary blood flow experience profound fatal cyanosis in the neonatal period as the ductus begins to close. On the other hand, patients with MAPCAs with mild stenosis of collateral vessels and balanced pulmonary blood flow may exhibit only mild cyanosis. These patients may survive into adolescence or adulthood without repair, although their life expectancy is significantly shorter than that of the normal population.

• #54 Pulmonary Atresia with Ventricular Septal Defect, and Right Ventricle-to-Pulmonary Artery Conduits | Thoracic Key

  https://thoracickey.com/pulmonary-atresia-with-ventricular-septal-defect-and-right-ventricle-to-pulmonary-artery-conduits/

  Pulmonary atresia with ventricular septal defect (PAVSD) is a congenital cardiac malformation characterized by discontinuity of blood flow from the right ventricle to the pulmonary arteries, a ventricular septal defect (VSD) resulting from anterior deviation of the infundibular (conal) septum, and an overriding aorta. […] The natural history of PAVSD varies significantly depending on the pulmonary artery morphology. Patients presenting with PAVSD that has ductal-dependent pulmonary blood flow experience profound fatal cyanosis in the neonatal period as the ductus begins to close. On the other hand, patients with MAPCAs with mild stenosis of collateral vessels and balanced pulmonary blood flow may exhibit only mild cyanosis. These patients may survive into adolescence or adulthood without repair, although their life expectancy is significantly shorter than that of the normal population.

• #55 Pulmonary Atresia with Ventricular Septal Defect, and Right Ventricle-to-Pulmonary Artery Conduits | Thoracic Key

  https://thoracickey.com/pulmonary-atresia-with-ventricular-septal-defect-and-right-ventricle-to-pulmonary-artery-conduits/

  The clinical presentation also varies according to pulmonary artery morphology. Patients with ductal-dependent pulmonary circulation present with profound cyanosis during the first several weeks of life as the ductus begins to close. Children with aortopulmonary collaterals may present in childhood with cyanosis. Patients with large, unrestrictive collaterals may present with heart failure symptomsfailure to thrive, tachypnea, or feeding intolerance.

• #56 Pulmonary Atresia with Ventricular Septal Defect, and Right Ventricle-to-Pulmonary Artery Conduits | Thoracic Key

  https://thoracickey.com/pulmonary-atresia-with-ventricular-septal-defect-and-right-ventricle-to-pulmonary-artery-conduits/

  The clinical presentation also varies according to pulmonary artery morphology. Patients with ductal-dependent pulmonary circulation present with profound cyanosis during the first several weeks of life as the ductus begins to close. Children with aortopulmonary collaterals may present in childhood with cyanosis. Patients with large, unrestrictive collaterals may present with heart failure symptomsfailure to thrive, tachypnea, or feeding intolerance.

• #57 Pulmonary Atresia With Ventricular Septal Defect – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK562277/

  The clinical presentation of the PAVSD is highly variable depending on the degree of pulmonary atresia and MAPCAs. Most neonates present with cyanosis and cardiac murmur. Other common symptoms include the following: Central cyanosis may present as bluish discoloration of the face, particularly around the mouth and lips. In severe cases, it may be seen in peripheral limbs as well. Increased respiratory rate or shortness of breath due to poor blood oxygenation. This may not be evident at rest and only at exertion during crying or breastfeeding. Easy fatigability may present as a weak cry, tone loss, and poor breast latching. Failure to thrive and difficulty feeding are also symptoms. […] The physical exam may reveal central cyanosis, a holosystolic murmur at the left sternal border that may radiate to the back or axilla, a single accentuated second heart sound S2, a machine-like continuous murmur of PDA best heard at the upper precordium or the interscapular region on the back, a weak grip, a low weight for age, and lethargy. Peripheral edema, clubbing, and worsening cyanosis may indicate congestive heart failure (CHF), especially if presenting late in life. A small number of neonates/infants present with CHF early in life. They have large MAPCAs with excessive pulmonary blood flow, sometimes requiring mechanical ventilation. These patients require early complete repair, and outcomes are generally favorable because they often have well-developed pulmonary vasculature.

• #58 Pulmonary atresia with ventricular septal defect – Wikipedia

  https://en.wikipedia.org/wiki/Pulmonary_atresia_with_ventricular_septal_defect

  Pulmonary atresia with ventricular septal defect is a rare birth defect characterized by pulmonary valve atresia occurring alongside a defect on the right ventricular outflow tract. The spectrum of symptoms exhibited by children with this condition depends on the severity of the condition, while some barely show symptoms, others might develop complications such as congestive heart failure. In symptomatic children, symptoms become apparent soon after birth, these usually consist of the following: Cyanosis, Breathing difficulties, Feeding difficulties, Exhaustion while being fed, Heart murmur, Excessive daytime sleepiness, Sticky skin. Children with this condition are at a higher risk of developing the following complications: Failure to thrive, Recurrent chest infections, Endocarditis, Epilepsy, Stroke, Arrhythmia, Heart failure, Premature death. Without treatment, it is a highly life-threatening condition, so prognosis is poor. Life expectancy for untreated children with PAVSD is 10 years. Survival rates for untreated people with this defect have been reported to be 50% at the tenth decade and 10% at the twentieth decade, and out of these untreated patients, those who do not have major aortopulmonary arteries have a higher chance of living to their 30s than those who do have them, as the latter have a 40% chance of surviving to the tenth decade and a 20% chance of doing so to the thirtieth decade. Prognosis after surgical intervention is generally good.

• #59 Health Library

  https://www.limamemorial.org/health-library/HIE%20Multimedia-TextOnly/1/001091

  Pulmonary atresia is a form of heart disease in which the pulmonary valve does not form properly. It is present from birth (congenital heart disease). The pulmonary valve is an opening on the right side of the heart that regulates blood flow from the right ventricle (right side pumping chamber) to the lungs. […] In pulmonary atresia, the valve leaflets are fused. This causes a solid sheet of tissue to form where the valve opening should be. Normal blood flow to the lung is blocked as a result. Because of this defect, blood from the right side of the heart is restricted from reaching the lungs to pick up oxygen. […] Symptoms most often occur in the first few hours of life, although it may take up to a few days. […] Symptoms may include: Bluish color to the skin (cyanosis) due to low oxygen level in the blood, Fast breathing, Fatigue, Poor eating habits (babies may get tired while nursing or sweat during feedings), Shortness of breath. […] Complications may include: Delayed growth and development, Seizures, Stroke, Infectious endocarditis, Heart failure, Death. […] Contact your provider if the baby has: Problems breathing, Skin, nails, or lips that appear blue (cyanosis).

• #60 Pediatric Pulmonary Atresia – Conditions and Treatments | Children’s National Hospital

  https://www.childrensnational.org/get-care/health-library/pulmonary-atresia

  The most common symptom is a bluish color of the skin in a newborn. […] Symptoms may happen shortly after birth or later as the ductus arteriosus closes. The most clear symptom is a bluish color of the skin (cyanosis) in a newborn. […] These are other common symptoms: Fast or troubled breathing, Trouble feeding, Weakness and sleepiness, Pale, cool or moist skin, Seizures. […] Because the blood doesn’t get enough oxygen, every cell in the baby’s body gets less oxygen. A newborn baby with PA cant live long without treatment. […] Without surgery, the heart cant pump oxygenated blood to the body and cant support life. […] The outlook varies from child to child. Follow-up care at a center offering pediatric congenital cardiac care should be carried out regularly. It is not unexpected for multiple reoperations to be performed to replace conduits or revise a palliation. […] There is significant risk for progressive development of complications, such as heart failure, dysrhythmias and protein-losing enteropathy (liver congestion).

• #61 Pulmonary Atresia With Ventricular Septal Defect – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK562277/

  Without surgical correction, about 50% of patients die within the first 2 years of life, and 20-year survival is around 10%. With proper treatment and follow-up, 65% of patients alive at 1 year can live beyond the age of 10. One high-volume center estimated operative mortality at 1.5% to 8%, depending on the procedure performed.

• #62 Pulmonary Atresia: Symptoms, Causes and Treatment

  https://my.clevelandclinic.org/health/diseases/14779-pulmonary-atresia

  Symptoms of pulmonary atresia often appear within the first few hours or days of a baby’s life and may include: Rapid breathing. A bluish tinge to the skin, especially the lips, fingers and toes. Cool, pale or clammy skin. Breathing problems. Fatigue or tiredness. Irritability. Poor feeding. […] Without treatment, pulmonary atresia is fatal because it makes your oxygen level low. However, when your healthcare provider makes a diagnosis before or shortly after your baby’s birth, they can treat your newborn to improve their oxygen circulation. Your baby may need several surgeries at different ages to keep improving their situation. […] Without having surgery to fix pulmonary atresia with a ventricular septal defect, the survival rate is 50% at age 1 and 8% at 10 years of age. Most people don’t live into their 30s without surgery.

• #63 Pulmonary atresia with ventricular septal defect – Wikipedia

  https://en.wikipedia.org/wiki/Pulmonary_atresia_with_ventricular_septal_defect

  Pulmonary atresia with ventricular septal defect is a rare birth defect characterized by pulmonary valve atresia occurring alongside a defect on the right ventricular outflow tract. The spectrum of symptoms exhibited by children with this condition depends on the severity of the condition, while some barely show symptoms, others might develop complications such as congestive heart failure. In symptomatic children, symptoms become apparent soon after birth, these usually consist of the following: Cyanosis, Breathing difficulties, Feeding difficulties, Exhaustion while being fed, Heart murmur, Excessive daytime sleepiness, Sticky skin. Children with this condition are at a higher risk of developing the following complications: Failure to thrive, Recurrent chest infections, Endocarditis, Epilepsy, Stroke, Arrhythmia, Heart failure, Premature death. Without treatment, it is a highly life-threatening condition, so prognosis is poor. Life expectancy for untreated children with PAVSD is 10 years. Survival rates for untreated people with this defect have been reported to be 50% at the tenth decade and 10% at the twentieth decade, and out of these untreated patients, those who do not have major aortopulmonary arteries have a higher chance of living to their 30s than those who do have them, as the latter have a 40% chance of surviving to the tenth decade and a 20% chance of doing so to the thirtieth decade. Prognosis after surgical intervention is generally good.

• #64 Pulmonary Atresia With Ventricular Septal Defect – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/sites/books/NBK562277/

  Without surgical correction, about 50% of patients die within the first 2 years of life, and 20-year survival is around 10%. With proper treatment and follow-up, 65% of patients alive at 1 year can live beyond the age of 10. […] One high-volume center estimated operative mortality at 1.5% to 8%, depending on the procedure performed.

• #65 Pulmonary Atresia: Overview, Causes, Treatment

  https://www.healthline.com/health/pulmonary-atresia

  Pulmonary atresia can be a serious condition with a risk of death, especially without appropriate treatment. But with advances in medical and surgical care, the outlook for individuals with pulmonary atresia has improved significantly, and many can lead relatively normal lives with appropriate management. […] The exact survival rate varies, but overall, there has been a significant improvement in survival rates in recent years.

• #66 What Is Pulmonary Atresia With Ventricular Septal Defect?

  https://www.icliniq.com/articles/heart-circulatory-health/pulmonary-atresia-with-ventricular-septal-defect

  The newborns affected with pulmonary atresia with ventricular septal defect require immediate treatment. Treatment procedures may include recommendations for medications, surgery, and other procedures. […] The child should have regular follow-ups with the pediatric cardiologist. This starts after two to four weeks. Few children may need many heart catheterizations or may require special medication. Long-term complications may require: Abnormal heart rhythm. Heart failure. Narrowing of the pulmonary artery. […] Pulmonary atresia is a congenital heart condition that may differ from person to person. This condition can be diagnosed at birth or after birth and can be treated through medications or surgical procedures. The proper medical situation of the child should be known, and regular follow-ups with the doctor are mandatory once the baby returns home. These visits can help the doctor determine any other complications that may develop.

• #67 Pulmonary Atresia: Causes, Symptoms, and Treatment – Longmore Clinic

  https://longmoreclinic.org/pulmonary-atresia-causes-symptoms-and-treatment/

  Treatment for pulmonary atresia depends on the severity of the condition and any associated heart defects. It typically involves surgery to improve blood flow to the lungs: […] Children who have been treated for pulmonary atresia need lifelong follow-up care with a cardiologist. […] Pulmonary atresia may be associated with other heart defects, such as atrial septal defects (ASD) or patent ductus arteriosus (PDA), which can further complicate the condition. […] Children with pulmonary atresia may face challenges related to physical development, endurance, and overall health. […] With early diagnosis and treatment, many children with pulmonary atresia can lead healthy lives.

• #68 Pulmonary Atresia: Causes, Symptoms, and Treatment – Longmore Clinic

  https://longmoreclinic.org/pulmonary-atresia-causes-symptoms-and-treatment/

  Treatment for pulmonary atresia depends on the severity of the condition and any associated heart defects. It typically involves surgery to improve blood flow to the lungs: […] Children who have been treated for pulmonary atresia need lifelong follow-up care with a cardiologist. […] Pulmonary atresia may be associated with other heart defects, such as atrial septal defects (ASD) or patent ductus arteriosus (PDA), which can further complicate the condition. […] Children with pulmonary atresia may face challenges related to physical development, endurance, and overall health. […] With early diagnosis and treatment, many children with pulmonary atresia can lead healthy lives.

• #69 33920 CPT4 – GenHealth.ai

  https://genhealth.ai/code/cpt4/33920-repair-of-pulmonary-atresia-with-ventricular-septal-defect-by-construction-or-replacement-of-conduit-from-right-or-left-ventricle-to-pulmonary-artery

  Pulmonary atresia with ventricular septal defect is a serious congenital heart disorder that prevents blood from properly flowing from the heart to the lungs. […] Severe cyanosis (bluish skin due to lack of oxygen) […] Difficulty breathing or shortness of breath […] Failure to thrive or poor growth in infants […] Diagnosed congenital heart defect with compromised blood flow to the lungs […] Echocardiographic or cardiac catheterization findings indicating PA/VSD. […] Improved oxygenation and overall cardiovascular function […] Relief from symptoms such as cyanosis and breathlessness […] Enhanced quality of life and normal growth in children.

• #70 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Treatment-for-Pulmonary-Atresia.aspx

  Pulmonary atresia affects 1 in 10,000 live births. Babies born with the condition will begin to exhibit symptoms of the associated defects on the day they are born. […] The right ventricle is not too small and the pulmonary valve forms but it is defective. The small-sized pulmonary valve has trouble directing the flow of blood from the heart. […] The outlook is quite positive for a baby who has undergone corrective surgery for pulmonary atresia. Most children recover from the birth defect and will go on to meet all regular developmental milestones.

• #71 Pulmonary Atresia: Causes, Symptoms, and Treatment – Longmore Clinic

  https://longmoreclinic.org/pulmonary-atresia-causes-symptoms-and-treatment/

  Treatment for pulmonary atresia depends on the severity of the condition and any associated heart defects. It typically involves surgery to improve blood flow to the lungs: […] Children who have been treated for pulmonary atresia need lifelong follow-up care with a cardiologist. […] Pulmonary atresia may be associated with other heart defects, such as atrial septal defects (ASD) or patent ductus arteriosus (PDA), which can further complicate the condition. […] Children with pulmonary atresia may face challenges related to physical development, endurance, and overall health. […] With early diagnosis and treatment, many children with pulmonary atresia can lead healthy lives.

• #72 Pulmonary atresia with ventricular septal defect – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-atresia-ventricular-septum-defect/cdc-20396707

  Pulmonary atresia with ventricular septal defect is life-threatening. A baby with pulmonary atresia eventually doesn’t get enough oxygen. One or more procedures or surgeries are needed to fix the heart. […] Symptoms of pulmonary atresia with ventricular septal defect, also called PA-VSD, may appear at birth or very soon after. They can include: Blue or gray skin. This change may be harder or easier to see depending on skin color. Fast breathing or shortness of breath. Tiredness. Poor feeding. […] If your baby has symptoms of this condition after birth, call a healthcare professional right away.

• #73 Health Library

  https://www.limamemorial.org/health-library/HIE%20Multimedia-TextOnly/1/001091

  Pulmonary atresia is a form of heart disease in which the pulmonary valve does not form properly. It is present from birth (congenital heart disease). The pulmonary valve is an opening on the right side of the heart that regulates blood flow from the right ventricle (right side pumping chamber) to the lungs. […] In pulmonary atresia, the valve leaflets are fused. This causes a solid sheet of tissue to form where the valve opening should be. Normal blood flow to the lung is blocked as a result. Because of this defect, blood from the right side of the heart is restricted from reaching the lungs to pick up oxygen. […] Symptoms most often occur in the first few hours of life, although it may take up to a few days. […] Symptoms may include: Bluish color to the skin (cyanosis) due to low oxygen level in the blood, Fast breathing, Fatigue, Poor eating habits (babies may get tired while nursing or sweat during feedings), Shortness of breath. […] Complications may include: Delayed growth and development, Seizures, Stroke, Infectious endocarditis, Heart failure, Death. […] Contact your provider if the baby has: Problems breathing, Skin, nails, or lips that appear blue (cyanosis).

• #74 Pulmonary Atresia

  https://healthlibrary.vidanthealth.com/Conditions/Heart/90,P01809

  Pulmonary atresia (PA) is a heart defect. It happens when the baby’s heart doesnt form as it should in the uterus. This can happen during the first 8 weeks of pregnancy. […] Symptoms may happen shortly after birth or later as the ductus arteriosus closes. The most clear symptom is a bluish color of the skin (cyanosis) in a newborn. […] These are other common symptoms: Fast or troubled breathing, Trouble feeding, Weakness and sleepiness, Pale, cool, or moist skin, Seizures. […] Without surgery, the heart cant pump oxygenated blood to the body and cant support life. […] Your child will need surgery to improve blood flow to the lungs. The type of surgery your child has will depend on whether the tricuspid valve or right ventricle can send enough blood to the lungs. […] After the surgical repair and time for recovery in the hospital, your baby will be able to go home. […] The outlook varies from child to child. Be sure to get regular follow-up care at a center offering pediatric congenital heart care. Your child likely will need more surgery. […] Call your child’s healthcare provider if your child has breathing or feeding problems or has new symptoms.

• #75 Mayo Clinic Health Library – Pulmonary atresia with ventricular septal defect | Swiss Medical Network

  https://www.swissmedical.net/en/healtcare-library/con-20380877

  Symptoms of pulmonary atresia with ventricular septal defect, also called PA-VSD, may appear at birth or very soon after. They can include: […] A baby with pulmonary atresia eventually doesn’t get enough oxygen. One or more procedures or surgeries are needed to fix the heart. […] Pulmonary atresia with ventricular septal defect, also called PA-VSD, is typically found during pregnancy or soon after birth. If your baby has symptoms of this condition after birth, call a healthcare professional right away. […] A baby with pulmonary atresia with ventricular septal defect, also called PA-VSD, needs treatment right away. Treatment may include one or more surgeries or procedures.

• #76 Pulmonary Atresia: Overview, Causes, Treatment

  https://www.healthline.com/health/pulmonary-atresia

  Pulmonary atresia can be a serious condition with a risk of death, especially without appropriate treatment. But with advances in medical and surgical care, the outlook for individuals with pulmonary atresia has improved significantly, and many can lead relatively normal lives with appropriate management. […] The exact survival rate varies, but overall, there has been a significant improvement in survival rates in recent years.

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