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. […] Early detection through fetal ultrasound or postnatal echocardiography is crucial, as untreated cases can lead to severe cyanosis, heart failure, and life-threatening complications. […] This course provides healthcare professionals with the knowledge to diagnose and manage pulmonary atresia with ventricular septal defect accurately. […] Participants gain insight into prenatal and postnatal screening techniques, imaging modalities, and individualized surgical strategies based on anatomical presentation. […] The curriculum emphasizes the importance of an interprofessional approach, integrating expertise from pediatric cardiologists, cardiothoracic surgeons, neonatologists, and genetic counselors to ensure comprehensive care.

• #1 Pulmonary atresia with ventricular septal defect – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-atresia-ventricular-septum-defect/diagnosis-treatment/drc-20580526

  Pulmonary atresia with ventricular septal defect, also called PA-VSD, is often diagnosed during pregnancy or soon after birth. […] Tests that may be used to diagnose pulmonary atresia with ventricular septal defect include: […] An echocardiogram of a baby’s heart during pregnancy is called a fetal echocardiogram. It can diagnose pulmonary atresia. […] An ECG can find irregular heartbeats. […] A cardiac CT can help diagnose major aortopulmonary collateral arteries, also called MAPCAs. Knowing about the MAPCAs is important for planning treatment.

• #1 Pulmonary Atresia: Overview, Causes, Treatment

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

  Pulmonary atresia is typically diagnosed in the womb (evident at 18-22 weeks gestation) or shortly after birth. The diagnosis is often made based on prenatal ultrasound findings that show abnormalities in the baby’s heart. After birth, a physical examination may reveal symptoms such as a heart murmur or bluish discoloration of the skin. […] Further diagnostic tests may include: […] Echocardiogram: An echocardiogram uses ultrasound to visualize the heart’s structure and function, confirming the diagnosis and assessing severity. […] Electrocardiogram (EKG): An EKG records the heart’s electrical activity, identifying abnormalities in rhythm or structure. […] Chest X-ray: A chest X-ray shows heart and lung blood vessel size and shape. […] Cardiac catheterization: A cardiac catheterization involves inserting a catheter into a blood vessel and guiding it to the heart for detailed information. […] MRI or CT scan: An MRI scan or CT scan provides detailed heart and blood vessel images.

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

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

  Pulmonary atresia may be diagnosed during pregnancy or soon after the baby is born. […] An ultrasound, a tool that creates pictures of the baby, may detect pulmonary atresia during pregnancy. If the health care provider suspects pulmonary atresia, they can request a fetal echocardiogram to confirm the diagnosis. A fetal echocardiogram is a more detailed ultrasound of the baby’s heart. This test can show problems with the structure of the heart and how the heart is working. […] During a physical examination, a doctor can see the symptoms, such as bluish skin or problems breathing. Using a stethoscope, a doctor will check for a heart murmur (an abnormal „whooshing” sound caused by blood not flowing properly). However, it is not uncommon for a heart murmur to be absent right at birth. […] If a doctor suspects a problem, the health care provider might request an echocardiogram, which is an ultrasound of the heart. Cardiac catheterization can also confirm the diagnosis by looking inside the heart and measuring the blood pressure and oxygen levels. An electrocardiogram (EKG) may also be conducted to confirm the diagnosis. […] Pulmonary atresia can also be detected with newborn pulse oximetry screening. Low levels of oxygen in the blood can be a sign of a critical CHD like pulmonary atresia. Newborn screening using pulse oximetry can identify some infants with pulmonary atresia before they show any symptoms.

• #1 4.5 Pulmonary Valve Atresia (Q22.0) | CDC

  https://archive.cdc.gov/www_cdc_gov/ncbddd/birthdefects/surveillancemanual/chapters/chapter-4/chapter4-5e.html

  Infants with pulmonary atresia (with or without ventricular septal defect) typically present early in the neonatal period with cyanosis and hypoxemia that worsens as the ductus closes. Echocardiography is the key diagnostic procedure, although other imaging techniques, including catheterization, might be necessary to better guide management and care. […] Pulmonary atresia with ventricular septal defect can be associated with deletion 22q11, unlike the form with an intact ventricular septum. […] Pulmonary atresia with ventricular septal defect is in most cases a conotruncal defect and should be grouped with tetralogy of Fallot and not with pulmonary atresia with intact ventricular septum.

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

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

  Other tests are needed to help with the diagnosis. They may include: […] This heart defect may also be diagnosed on fetal echocardiograms. It is one of the cardiac defects that may be found on screening ultrasounds. Early diagnosis of the defect allows for prompt intervention at the time of birth. […] Planning to deliver an infant at a hospital capable of newborn resuscitation is important in improving the chances for a good outcome. […] Echocardiograms can give detailed information of the anatomy of the various cardiac structures affected in this congenital defect. They also give important information about the function of the heart, the heart valves, the size of the atrial septal defect and the size of the patent ductus arteriosus.

• #1 Prenatally diagnosed pulmonary atresia with ventricular septal defect: echocardiography, genetics, associated anomalies and outcome | Heart

  https://heart.bmj.com/content/92/10/1501.short

  Objective: To assess the accuracy of prenatal diagnosis, the association with genetic and extracardiac anomalies, and outcome in fetuses with isolated pulmonary atresia with ventricular septal defect (PA-VSD). […] PA-VSD was correctly diagnosed in 19 of 21 patients (90%) with postnatal or autopsy confirmation of diagnosis. […] Conclusion: PA-VSD can be diagnosed by fetal echocardiography with a high degree of accuracy. However, it can be difficult to determine the morphology of the central pulmonary arteries and to locate the source of pulmonary blood supply.

• #1 Pulmonary Atresia | Boston Children’s Hospital

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

  Pulmonary atresia is type of heart defect that a baby is born with. It occurs when the pulmonary valve — normally located between the right ventricle and the pulmonary artery — doesn’t form properly. This means that blood can’t flow from the heart to the lungs to get oxygen to the body. In some cases, babies with pulmonary atresia may also have a small, or missing, right ventricle that can’t properly pump blood to the lungs. […] Most babies with pulmonary atresia are diagnosed shortly after birth. In some cases, it is diagnosed before birth by a prenatal ultrasound. […] If your baby is born with a bluish tint to the skin or other symptoms of pulmonary atresia, he or she will likely see a cardiologist (heart doctor). […] To diagnose pulmonary atresia, the cardiologist will examine the baby and measure the oxygen level in his or her blood. The cardiologist will also listen for a heart murmur — a noise heard through a stethoscope that’s caused by the turbulence of blood flow. This will give the cardiologist an initial idea of the kind of heart problem your baby may have.

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

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

  Tests performed when diagnosing PA with VSD may include: Cardiac Catheterization: During cardiac catheterization, a small catheter (thin tube) is inserted into a larger blood vessel, typically in the groin, and guided to the heart where blood pressure and oxygen measurements can be taken in the aorta and pulmonary artery as well as the four chambers of the heart. A dye can also be injected through the tube to make both the hearts structure and the MAPCAs more visible on an X-ray. Cardiac MRI or CT Scan: A cardiac MRI or CT scan is used to take more detailed images of the heart to help define the anatomy and detect anomalies, such as when there are MAPCAs. Chest X-Ray: A chest X-ray produces an image of the tissue and bones in the heart and lungs and helps your provider assess the shape, size, and structure of the heart and lungs as well as the aeration of or any congestion in the lungs. Echocardiogram: An echocardiogram uses ultrasound technology to create a moving image of the heart and its valves, allowing your provider to assess the structure and function of the heart. An echocardiogram also helps provide information about blood flow and how well the heart is pumping blood. An echocardiogram is not always the best way to observe MAPCAs. Electrocardiogram (ECG or EKG): An electrocardiogram uses electrodes that are placed on the body to record the electrical activity taking place in the heart. An ECG/EKG test helps detect abnormal rhythms, such as cardiac arrhythmias, stress on the heart, and damage to the heart muscles.

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

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

  Through effective collaboration, healthcare teams enhance early diagnosis, optimize surgical outcomes, and provide long-term management strategies that improve survival and quality of life for affected patients. […] Assess the diagnostic evaluation of pulmonary atresia with ventricular septal defect. […] Evaluate the management options available for pulmonary atresia with ventricular septal defect. […] The following investigations are a part of the comprehensive evaluation of PAVSD: Echocardiography: The gold standard investigation module provides valuable insight into pulmonary valve atresia, overriding of the aorta, VSD, ASD, some MAPCA if present, the pressure gradient across valves, and ejection fraction. […] Angiography: Cardiac catheterization is another important investigation that can provide details of the arterial vasculature’s anatomy, size, and distribution.

• #1 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/

  Magnetic resonance imaging (MRI) is a useful adjunct to cardiac catheterization in patients with MAPCAs. […] The ultimate goal of the surgical repair strategy is creation of biventricular circulation with unobstructed RV-to-PA continuity and elimination of intracardiac or extracardiac shunts with low resultant right ventricular pressure. […] Unifocalization is a technique in which MAPCAs are disconnected from the descending aorta and anastomosed to a common confluence that can receive blood supply from a stable sourceusually the RV or a systemic shunt. […] The VSD may be closed with Dacron, polytetrafluoroethylene (PTFE), or pericardial patch material. […] An RV-to-PA conduit can be sewn to confluent central native pulmonary arteries or to unifocalized pulmonary vascular segments. […] Lung or heartlung transplantation may be applied selectively for certain patients, generally adolescents or adults, with irreversible pulmonary hypertension.

• #1 Complete Preoperative Evaluation of Pulmonary Atresia with Ventricular Septal Defect with Multi-Detector Computed Tomography | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0146380

  To compare multi-detector computed tomography (MDCT) with cardiac catheterization and transthoracic echocardiography (TTE) in comprehensive evaluation of the global cardiovascular anatomy in patients with pulmonary atresia with ventricular septal defect (PA-VSD). […] MDCT was more accurate than catheterization and TTE in identification of native pulmonary arteries. MDCT is also the most accurate test for delineation of the major aortopulmonary collateral arteries. […] MDCT can correctly delineate the native pulmonary vasculatures and intracardiac defects and may be a reliable method for noninvasive assessment of global cardiovascular abnormalities in patients with PA-VSD. […] Pulmonary atresia with ventricular septal defect (PA-VSD) is defined as a group of cardiac malformation characterized by lack of luminal continuity, and absence of blood flow between right ventricle and pulmonary artery, with egress of blood from right ventricle (RV) occurring through a ventricular septal defect.

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

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

  The McGoon index evaluates the proportional size of the pulmonary arteries compared to the descending aorta in congenital heart conditions. […] The normal value for the NI is 330 30 mm/body surface area and remains consistent across a wide range of body surface areas from infancy through adolescence. […] The major benefit of this approach is that the whole procedure becomes more tolerable by the already feeble neonate, and breaks are available to allow for healing. […] In contrast to staged repair, the other approach is complete repair in 1 surgery. […] A cardiac transplant can be a viable option in patients with a completely atretic pulmonary system or who have failed the surgical corrective measures. […] A final diagnosis requires a comprehensive evaluation, with consultation from departments like pediatric cardiology, pediatric surgery, and geneticists. […] PAVSD is a rare condition that requires prompt referral to pediatric cardiologists when echocardiographic or computed tomography/magnetic resonance imaging scans reveal cardiac defects. […] The outcomes of a PAVSD depend on the time of presentation and extent of severity.

• #1 The Modern Surgical Approach to Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries

  https://www.mdpi.com/2227-9067/9/4/515

  CMR imaging provides also functional information for surgical decision making in patients with PA/VSD/MAPCAs. […] The preoperative Qp:Qs ratio calculated with CMR has been shown to correlate with RV systolic pressure after VSD closure, proving useful in selecting patients for complete repair. […] The goals of surgical treatment are to achieve a single confluence of all sources of pulmonary blood flow (unifocalization) and to complete the repair by closing the VSD and creating a connection between the RV and pulmonary artery (PA). […] The unifocalization technique, particularly in single-stage constitutes the approach of choice advocated by the Stanford group. […] The modern approach to PA/VSD/MAPCAs is the synthesis of decades of experience of the world’s leading centers in the treatment of this disease. The experience gained at our institution confirms that a structured approach to the treatment of this complex lesion is the key to achieve optimal results. […] The ultimate goal is to achieve definitive intracardiac repair which has proved to be the main determinant of both improved survival and adequate RV systolic performance during mid-term follow-up.

• #1 4.5 Pulmonary Valve Atresia (Q22.0) | CDC

  https://archive.cdc.gov/www_cdc_gov/ncbddd/birthdefects/surveillancemanual/chapters/chapter-4/chapter4-5e.html

  Pulmonary valve atresia is a structural heart anomaly characterized clinically by cyanosis and anatomically by an imperforate pulmonary valve that blocks the flow of blood through the right ventricular outflow tract completely. […] Two main forms must be distinguished, based on whether or not a ventricular septal defect is present. […] In most cases, pulmonary valve atresia with a ventricular septal defect is considered as a conotruncal defect specifically the more severe end of the spectrum of tetralogy of Fallot. […] Pulmonary atresia with intact ventricular septum is a different entity unrelated to conotruncal heart defects. […] Pulmonary valve atresia can be suspected prenatally on a second trimester obstetrican atomic scan, using a combination of four-chamber and outflow tract views, and further characterized by fetal echocardiography. Because of diagnostic challenges, however, postnatal confirmation is necessary for a firm diagnosis.

• #1

  https://link.springer.com/article/10.1007/s10396-017-0809-2

  Pulmonary atresia with ventricular septal defect (PA-VSD) is a rare complex congenital heart defect. Major artery-pulmonary collateral arteries (MAPCAs) are characteristic of PA-VSD. Prenatal diagnosis can be achieved in most cases of PA-VSD with recent advances in echocardiography. However, it is extremely rare that all MAPCAs can be observed on the echocardiograph. Here, we report a case of prenatally diagnosed type C PA-VSD in which all the MAPCAs could be seen on the echocardiograph, with the diagnosis supported by autopsy evidence. […] Accuracy of fetal echocardiography in the differential diagnosis between truncus arteriosus and pulmonary atresia with ventricular septal defect. […] Prenatally diagnosed pulmonary atresia with ventricular septal defect: echocardiography, genetics, associated anomalies and outcome.

• #1 Current era outcomes of pulmonary atresia with ventricular septal defect: A single center cohort in Thailand | Scientific Reports

  https://www.nature.com/articles/s41598-020-61879-2

  Pulmonary atresia with ventricular septal defect (PA/VSD) is a complex cyanotic congenital heart disease with a wide-range of presentations and treatment strategies, depending on the source of pulmonary circulation, anatomy of pulmonary arteries (PAs), and major aortopulmonary collateral arteries (MAPCAs). […] The median age of diagnosis was 0.5 (0-13.8) years. […] The survival rates at 1, 5, and 10 years of age were 95%, 83.7%, and 79.6%, respectively. […] Significant mortality risks were the presence of associated anomalies and non-confluent PAs. […] The present study evaluates survival rates and mortality risks of children who were diagnosed with PA/VSD at Siriraj Hospital in the last decade. […] Overall survival rates of PA/VSD at 1, 5, and 10 years of age, were 95%, 83.7%, and 79.6%, respectively.

• #1 Current era outcomes of pulmonary atresia with ventricular septal defect: A single center cohort in Thailand | Scientific Reports

  https://www.nature.com/articles/s41598-020-61879-2

  In this study, the significant predictors of death were the presence of associated anomalies and non-confluent PAs. […] A multivariate analysis identified two predictors of death: presence of non-confluent PAs (hazard ratio 8.09, 95% CI 1.89-34.40, p-value=0.003) and presence of associated anomalies (hazard ratio 6.50, 95% CI 1.87-22.60, p-value=0.002). […] Children with PA/VSD have had a fairly good survival rate for the past decade.

• #1 Pulmonary Atresia: Symptoms, Causes and Treatment

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

  Usually, the right ventricle is well developed and can pump blood to your babys lungs. Surgery involves: Closing your babys ventricular septal defect. Placing a donated artery and valve between your childs right ventricle and pulmonary artery. This lets blood flow through their right ventricle into their pulmonary artery and to their lungs. […] 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 babys 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 dont live into their 30s without surgery. […] Medical procedures and surgeries can improve your childs condition, but they arent cures.

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

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

  Assess the diagnostic evaluation of pulmonary atresia with ventricular septal defect. […] The following investigations are a part of the comprehensive evaluation of PAVSD: Echocardiography: The gold standard investigation module provides valuable insight into pulmonary valve atresia, overriding of the aorta, VSD, ASD, some MAPCA if present, the pressure gradient across valves, and ejection fraction. […] The McGoon index evaluates the proportional size of the pulmonary arteries compared to the descending aorta in congenital heart conditions. […] A final diagnosis requires a comprehensive evaluation, with consultation from departments like pediatric cardiology, pediatric surgery, and geneticists. […] PAVSD is a rare condition that requires prompt referral to pediatric cardiologists when echocardiographic or computed tomography/magnetic resonance imaging scans reveal cardiac defects.

• #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. […] 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. […] This course provides healthcare professionals with the knowledge to diagnose and manage pulmonary atresia with ventricular septal defect accurately. Participants gain insight into prenatal and postnatal screening techniques, imaging modalities, and individualized surgical strategies based on anatomical presentation.

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

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

  Other tests are needed to help with the diagnosis. They may include: […] This heart defect may also be diagnosed on fetal echocardiograms. It is one of the cardiac defects that may be found on screening ultrasounds. Early diagnosis of the defect allows for prompt intervention at the time of birth. […] Planning to deliver an infant at a hospital capable of newborn resuscitation is important in improving the chances for a good outcome. […] Echocardiograms can give detailed information of the anatomy of the various cardiac structures affected in this congenital defect. They also give important information about the function of the heart, the heart valves, the size of the atrial septal defect and the size of the patent ductus arteriosus.

• #2 Pulmonary artesia with ventricular septal defect

  https://www.isuog.org/clinical-resources/patient-information-series/patient-information-pregnancy-conditions/heart/pulmonary-artesia-with-ventricular-septal-defect.html

  Pulmonary Atresia with Ventricular Septal Defect (PA-VSD) is a complex congenital heart defect (CHD). It occurs in less than 1 in 10,000 fetuses. […] PA-VSD is diagnosed when pulmonary atresia and VSD occur together. […] Your caregiver will refer you to specialists in fetal cardiology (a maternal-fetal medicine specialist or a pediatric cardiologist) to diagnose as accurately as possible your fetus’s individual anatomic structure and monitor the progress of the defect during the course of your pregnancy. […] Your caregiver may refer you for genetic counseling and genetic testing. […] You will likely be offered tests to look for some of the known genetic changes that can cause PA-VSD. […] In PA-VSD, blood flow from the right ventricle to the pulmonary artery is blocked, so blood is not pumped to the lungs, and a mixture of oxygen-rich and oxygen-poor blood flows out through the VSD to the aorta. […] PA-VSD is treated with cardiac surgery with a goal of creating a channel between the right ventricle and the pulmonary artery and closing the VSD. […] Your caregiver will likely order early targeted fetal scanning to rule out fetal heart defects in subsequent pregnancies.

• #2 Pulmonary atresia with ventricular septal defect – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-atresia-ventricular-septum-defect/diagnosis-treatment/drc-20580526

  Pulmonary atresia with ventricular septal defect, also called PA-VSD, is often diagnosed during pregnancy or soon after birth. […] Tests that may be used to diagnose pulmonary atresia with ventricular septal defect include: […] An echocardiogram of a baby’s heart during pregnancy is called a fetal echocardiogram. It can diagnose pulmonary atresia. […] An ECG can find irregular heartbeats. […] A cardiac CT can help diagnose major aortopulmonary collateral arteries, also called MAPCAs. Knowing about the MAPCAs is important for planning treatment.

• #2 Pulmonary atresia with a ventricular septal defect and left pulmonary artery discontinuity: a case report | Journal of Medical Case Reports | Full Text

  https://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-021-02750-4

  Unilateral pulmonary artery discontinuity is a rare malformation that is associated with other intracardiac abnormalities. […] We report a case of pulmonary atresia with ventricular septal defect (PAVSD) with LPA discontinuity. […] The initial prenatal diagnosis was pulmonary atresia with VSD (PAVSD). […] Therefore, we think the detection of UPAD before surgery would help to decide the timing and planning of the surgery. […] Prenatal detection is important because it aids in the prompt initiation of PGE1 administration to ensure early rehabilitation of the affected lung. […] Therefore, clinicians should examine the route of both pulmonary arteries, regardless of the existence of other intracardiac abnormalities.

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

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

  Pulmonary atresia may be diagnosed during pregnancy or soon after the baby is born. […] An ultrasound, a tool that creates pictures of the baby, may detect pulmonary atresia during pregnancy. If the health care provider suspects pulmonary atresia, they can request a fetal echocardiogram to confirm the diagnosis. A fetal echocardiogram is a more detailed ultrasound of the baby’s heart. This test can show problems with the structure of the heart and how the heart is working. […] During a physical examination, a doctor can see the symptoms, such as bluish skin or problems breathing. Using a stethoscope, a doctor will check for a heart murmur (an abnormal „whooshing” sound caused by blood not flowing properly). However, it is not uncommon for a heart murmur to be absent right at birth. […] If a doctor suspects a problem, the health care provider might request an echocardiogram, which is an ultrasound of the heart. Cardiac catheterization can also confirm the diagnosis by looking inside the heart and measuring the blood pressure and oxygen levels. An electrocardiogram (EKG) may also be conducted to confirm the diagnosis. […] Pulmonary atresia can also be detected with newborn pulse oximetry screening. Low levels of oxygen in the blood can be a sign of a critical CHD like pulmonary atresia. Newborn screening using pulse oximetry can identify some infants with pulmonary atresia before they show any symptoms.

• #2 Pulmonary atresia with ventricular septal defect – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube

  https://www.augustahealth.com/disease/pulmonary-atresia-with-ventricular-septal-defect/

  Pulmonary atresia with ventricular septal defect is typically diagnosed at birth or soon after. Tests that may be used to diagnose pulmonary atresia with ventricular septal defect include: […] Pulse oximetry. A small sensor attached to a hand or foot measures the amount of oxygen in the blood. Pulse oximetry is simple and painless. […] Chest X-ray. A chest X-ray shows the shape and size of the heart and lungs. […] Echocardiogram. Sound waves are used to create images of the beating heart. An echocardiogram of the mother’s belly before delivery (fetal echocardiogram) can be used to diagnose pulmonary atresia. […] Electrocardiogram (ECG or EKG). This quick and simple test measures the electrical activity of the heart. Sticky patches (electrodes) with sensors detect the heartbeat. A monitor connected to the wires prints or displays results. An ECG can show how fast or slow the heart is beating. It can help diagnose irregular heart rhythms (arrhythmias).

• #2 Pulmonary Atresia: Symptoms, Causes and Treatment

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

  While youre pregnant, your healthcare provider will do standard screenings to check on the health of the fetus. If they see something of concern on an ultrasound (using harmless sound waves), they can do a fetal echocardiogram. This also uses sound waves to get a closer look at the fetal heart. […] After you have your baby, their healthcare provider will check their heart and lungs to find out if there are any problems. If they hear a heart murmur through a stethoscope, theyll order tests that may include: Pulse oximetry: This noninvasive test tells how much oxygen is in your babys blood. Chest X-ray: An imaging method that uses painless X-rays to get a picture of the inside of your babys chest from the outside. […] The type of surgery your baby needs for a pulmonary atresia repair will depend on several factors, including: The size of their right ventricle and pulmonary artery. The ability of their right ventricle to pump blood.

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

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

  Diagnosis for pulmonary atresia may require some or all of these tests: Echocardiogram (also called „echo” or ultrasound): sound waves create an image of the heart, Electrocardiogram (ECG): a record of the electrical activity of the heart, Chest X-ray, Cardiac catheterization: a thin tube (catheter) is inserted into the heart through the large veins or arteries in the leg, Pulse oximetry: a painless way to monitor the oxygen content of the blood.

• #2 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. […] A pediatric cardiologist and/or a neonatologist may be involved in your child’s care. A pediatric cardiologist specializes in the diagnosis and medical management of congenital heart defects, as well as heart problems that may develop later in childhood. A neonatologist specializes in illnesses affecting newborns, both premature and full-term. […] Cyanosis is a major indication that there is a problem with a newborn. The child’s physician may have also heard a heart murmur during a physical examination. […] Diagnostic testing for congenital heart disease varies by the child’s age and condition. Some tests that may be recommended include the following: Chest X-ray. A diagnostic test that uses X-ray beams to produce images of internal tissues, bones, and organs onto film.

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

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

  Tests performed when diagnosing PA with VSD may include: Cardiac Catheterization: During cardiac catheterization, a small catheter (thin tube) is inserted into a larger blood vessel, typically in the groin, and guided to the heart where blood pressure and oxygen measurements can be taken in the aorta and pulmonary artery as well as the four chambers of the heart. A dye can also be injected through the tube to make both the hearts structure and the MAPCAs more visible on an X-ray. Cardiac MRI or CT Scan: A cardiac MRI or CT scan is used to take more detailed images of the heart to help define the anatomy and detect anomalies, such as when there are MAPCAs. Chest X-Ray: A chest X-ray produces an image of the tissue and bones in the heart and lungs and helps your provider assess the shape, size, and structure of the heart and lungs as well as the aeration of or any congestion in the lungs. Echocardiogram: An echocardiogram uses ultrasound technology to create a moving image of the heart and its valves, allowing your provider to assess the structure and function of the heart. An echocardiogram also helps provide information about blood flow and how well the heart is pumping blood. An echocardiogram is not always the best way to observe MAPCAs. Electrocardiogram (ECG or EKG): An electrocardiogram uses electrodes that are placed on the body to record the electrical activity taking place in the heart. An ECG/EKG test helps detect abnormal rhythms, such as cardiac arrhythmias, stress on the heart, and damage to the heart muscles.

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

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

  Assess the diagnostic evaluation of pulmonary atresia with ventricular septal defect. […] The following investigations are a part of the comprehensive evaluation of PAVSD: Echocardiography: The gold standard investigation module provides valuable insight into pulmonary valve atresia, overriding of the aorta, VSD, ASD, some MAPCA if present, the pressure gradient across valves, and ejection fraction. […] The McGoon index evaluates the proportional size of the pulmonary arteries compared to the descending aorta in congenital heart conditions. […] A final diagnosis requires a comprehensive evaluation, with consultation from departments like pediatric cardiology, pediatric surgery, and geneticists. […] PAVSD is a rare condition that requires prompt referral to pediatric cardiologists when echocardiographic or computed tomography/magnetic resonance imaging scans reveal cardiac defects.

• #2 Pulmonary atresia with ventricular septal defect – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube

  https://www.augustahealth.com/disease/pulmonary-atresia-with-ventricular-septal-defect/

  Cardiac catheterization. A heart doctor (cardiologist) threads a thin tube (catheter) through a blood vessel in the arm or groin to an artery in the heart and injects dye through the catheter. This makes the heart arteries show up more clearly on an X-ray. […] Cardiac CT scan. This test uses a series of X-rays to create pictures of the heart and blood vessels. It can help reveal the shape of the heart and lungs and determine if major aortopulmonary collateral arteries (MAPCAs) are present. Knowing about the MAPCAs is important for planning treatment.

• #2 Complete Preoperative Evaluation of Pulmonary Atresia with Ventricular Septal Defect with Multi-Detector Computed Tomography | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0146380

  The main preoperative goals of imaging are as follows: (a) visualization of the native pulmonary arteries; (b) assessment of the numbers, origin and course of the APCAs; and (c) characterization of the intracardiac malformation. […] Current fast MR imaging and MR angiographic acquisition techniques with bolus injections of contrast material can provide images of the pulmonary vasculature that are similar to those obtained at multi-detector computed tomography (MDCT), but clinical application of MRI is limited because of the following disadvantages. […] Previous studies with small sample size have revealed that MDCT is equivalent or superior to cardiac catheterization for the preoperative and postoperative characterization of pulmonary vasculature. […] The present study, therefore, was retrospectively undertaken to compare MDCT with cardiac catheterization and echocardiography in preoperative evaluation of the global cardiovascular anatomy in patients with PA-VSD.

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

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

  The McGoon index evaluates the proportional size of the pulmonary arteries compared to the descending aorta in congenital heart conditions. […] The normal value for the NI is 330 30 mm/body surface area and remains consistent across a wide range of body surface areas from infancy through adolescence. […] The major benefit of this approach is that the whole procedure becomes more tolerable by the already feeble neonate, and breaks are available to allow for healing. […] In contrast to staged repair, the other approach is complete repair in 1 surgery. […] A cardiac transplant can be a viable option in patients with a completely atretic pulmonary system or who have failed the surgical corrective measures. […] A final diagnosis requires a comprehensive evaluation, with consultation from departments like pediatric cardiology, pediatric surgery, and geneticists. […] PAVSD is a rare condition that requires prompt referral to pediatric cardiologists when echocardiographic or computed tomography/magnetic resonance imaging scans reveal cardiac defects. […] The outcomes of a PAVSD depend on the time of presentation and extent of severity.

• #2 Pulmonary atresia: MedlinePlus Medical EncyclopediaLock

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

  Pulmonary atresia may occur with or without a ventricular septal defect (VSD). […] If the person has both problems, the condition is called pulmonary atresia with VSD. This is an extreme form of tetralogy of Fallot. […] The following tests may be ordered: Chest x-ray, Echocardiogram, Electrocardiogram (ECG), Heart catheterization, Pulse oximetry — shows the amount of oxygen in the blood. […] Most cases can be helped with surgery. How well a baby does depends on: Size and connections of the pulmonary artery (the artery that takes blood to the lungs), How well the heart is beating, How well the other heart valves are formed or how much they are leaking. […] Outcome varies because of the different forms of this defect. A baby may need only a single procedure or could need three or more surgeries and have only a single working ventricle.

• #2 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. […] Early detection through fetal ultrasound or postnatal echocardiography is crucial, as untreated cases can lead to severe cyanosis, heart failure, and life-threatening complications. […] This course provides healthcare professionals with the knowledge to diagnose and manage pulmonary atresia with ventricular septal defect accurately. […] Participants gain insight into prenatal and postnatal screening techniques, imaging modalities, and individualized surgical strategies based on anatomical presentation. […] The curriculum emphasizes the importance of an interprofessional approach, integrating expertise from pediatric cardiologists, cardiothoracic surgeons, neonatologists, and genetic counselors to ensure comprehensive care.

• #2 CE Activity | Pulmonary Atresia With Ventricular Septal Defect | Pharmacist

  https://www.statpearls.com/pharmacist/ce/activity/112526

  This course provides healthcare professionals with the knowledge to diagnose and manage pulmonary atresia with ventricular septal defect accurately. Participants gain insight into prenatal and postnatal screening techniques, imaging modalities, and individualized surgical strategies based on anatomical presentation. The curriculum emphasizes the importance of an interprofessional approach, integrating expertise from pediatric cardiologists, cardiothoracic surgeons, neonatologists, and genetic counselors to ensure comprehensive care. Through effective collaboration, healthcare teams enhance early diagnosis, optimize surgical outcomes, and provide long-term management strategies that improve survival and quality of life for affected patients. […] Assess the diagnostic evaluation of pulmonary atresia with ventricular septal defect. […] Evaluate the management options available for pulmonary atresia with ventricular septal defect.

• #2 Current era outcomes of pulmonary atresia with ventricular septal defect: A single center cohort in Thailand | Scientific Reports

  https://www.nature.com/articles/s41598-020-61879-2

  In this study, the significant predictors of death were the presence of associated anomalies and non-confluent PAs. […] A multivariate analysis identified two predictors of death: presence of non-confluent PAs (hazard ratio 8.09, 95% CI 1.89-34.40, p-value=0.003) and presence of associated anomalies (hazard ratio 6.50, 95% CI 1.87-22.60, p-value=0.002). […] Children with PA/VSD have had a fairly good survival rate for the past decade.

• #2

  https://journals.lww.com/mjdy/fulltext/2020/13040/pulmonary_atresia_with_ventricular_septal_defect.23.aspx

  Hence, the diagnosis was some extreme form of TOF with variable combination of pulmonary arterial anatomy. […] CTPA revealed absent RVOT and origin of main pulmonary artery and left pulmonary artery, indicating PA. […] Large arterial channel representing MAPCAs (one on left) was seen arising from the descending thoracic aorta at D6 vertebral level supplying blood to the left upper lobe. […] Final diagnosis in our case was PA-VSD. […] The most important determinant of survival was the presence of adequate but not excessive pulmonary blood supply through the development of MAPCAs. […] Heterogeneity of pulmonary blood supply in PA-VSD patients limits the uniform applicability of management. […] However, the general principles of surgical repair include (1) placement of RV-PA conduit, (2) unifocalization of aorto-pulmonary collaterals connecting more lung segments to APCs, and (3) VSD closure. […] The overall life expectancy in the absence of surgery is reported to be 50% at 1 year of age and 8% at 10 years.

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