Atrezja płucna
Objawy

Atrezja płucna to wrodzona wada serca charakteryzująca się brakiem prawidłowo uformowanej zastawki pnia płucnego, co uniemożliwia przepływ krwi z prawej komory do płuc i prowadzi do ciężkiego niedotlenienia organizmu. Objawy kliniczne pojawiają się zwykle w pierwszych godzinach lub dniach życia i obejmują sinicę, tachypnoe, trudności oddechowe, osłabienie, letarg, słabe przybieranie na wadze oraz szmer serca. Nasilenie symptomów jest często związane z zamknięciem przewodu tętniczego, co dodatkowo ogranicza przepływ krwi do płuc. Bez leczenia wskaźnik przeżycia wynosi około 50% w pierwszym roku życia i spada do 8-10% w wieku 10 lat, a większość pacjentów nie dożywa 30. roku życia. Powikłania nieleczonej atrezji płucnej obejmują niewydolność serca, arytmie, infekcyjne zapalenie wsierdzia, udar mózgu, enteropatię z utratą białka oraz nagłą śmierć.

Atrezja płucna – definicja i mechanizm choroby

Atrezja płucna (ang. Pulmonary atresia) to wrodzona wada serca, która powstaje w pierwszych 8 tygodniach ciąży na skutek nieprawidłowego rozwoju serca płodu. Charakteryzuje się brakiem prawidłowo uformowanej zastawki pnia płucnego, co uniemożliwia przepływ krwi z prawej komory serca do płuc.12 W niektórych przypadkach zastawka może być całkowicie nieobecna, a w innych – zablokowana.3 W rezultacie krew nie może przepływać normalną drogą do płuc, aby pobrać tlen potrzebny dla całego organizmu.

Objawy atrezji płucnej

Objawy atrezji płucnej zazwyczaj pojawiają się w ciągu pierwszych kilku godzin lub dni po urodzeniu, choć w niektórych przypadkach mogą pojawić się dopiero po kilku dniach.456 Główne objawy wynikają z niedotlenienia organizmu i obejmują:

Sinica (cyanoza)

Najbardziej charakterystycznym objawem jest sinica – niebieskawe lub szarawe zabarwienie skóry, szczególnie widoczne na ustach, palcach rąk i nóg. Jest to spowodowane niskim poziomem tlenu we krwi.789 Intensywność sinicy może być różna w zależności od karnacji dziecka i może być trudniejsza do zaobserwowania u niemowląt o ciemniejszej skórze.10

Problemy oddechowe

Niemowlęta z atrezją płucną często wykazują:

  • Przyspieszony oddech (tachypnea)1112
  • Trudności z oddychaniem13
  • Zwiększony wysiłek oddechowy – widoczne jako wciąganie przestrzeni międzyżebrowych6

Zmęczenie i osłabienie

Dzieci z atrezją płucną często wykazują:

  • Nadmierną senność i zmęczenie414
  • Osłabienie i letarg11
  • Szybkie męczenie się podczas wysiłku15

Problemy z karmieniem

Obserwuje się również:

  • Trudności z karmieniem – dziecko szybko się męczy podczas karmienia166
  • Pocenie się podczas karmienia1718
  • Słabe przybieranie na wadze1920

Inne objawy

Dodatkowo u niemowląt z atrezją płucną można zaobserwować:

  • Bladą, chłodną lub wilgotną skórę1412
  • Szmer serca – nieprawidłowe dźwięki serca słyszalne podczas osłuchiwania stetoskopem821
  • Drgawki w cięższych przypadkach1512

Czynnik czasowy objawów

Ważnym aspektem atrezji płucnej jest zależność czasowa występowania objawów. Często objawy stają się wyraźniejsze po zamknięciu przewodu tętniczego (ductus arteriosus), który u płodu zapewnia przepływ krwi między aortą a tętnicą płucną.22 Po urodzeniu ten przewód zwykle zaczyna się zamykać, co w przypadku atrezji płucnej prowadzi do nasilenia objawów z powodu dalszego ograniczenia przepływu krwi do płuc.11 Zatem objawy mogą:

  • Wystąpić natychmiast po urodzeniu6
  • Rozwinąć się w ciągu pierwszych 24 godzin życia23
  • Pojawić się w ciągu kilku dni po urodzeniu, gdy przewód tętniczy zaczyna się zamykać1415

Progresja choroby i rokowanie

Atrezja płucna jest stanem zagrażającym życiu, który bez leczenia prowadzi do śmierci.714 Zaburzenie przepływu krwi do płuc powoduje, że każda komórka ciała dziecka otrzymuje niewystarczającą ilość tlenu, co uniemożliwia prawidłowe funkcjonowanie organizmu.12 Bez interwencji medycznej noworodek z atrezją płucną nie może przeżyć długo.1524

Rokowanie bez leczenia chirurgicznego

Dane dotyczące przeżywalności bez interwencji chirurgicznej są niepokojące:

  • Wskaźnik przeżycia dla nieleczonej atrezji płucnej z ubytkiem przegrody międzykomorowej wynosi około 50% w wieku 1 roku425
  • Spada do około 8-10% w wieku 10 lat426
  • Większość pacjentów bez leczenia nie dożywa 30. roku życia4

W przypadku atrezji płucnej z ubytkiem przegrody międzykomorowej bez leczenia chirurgicznego około 50% pacjentów umiera w ciągu pierwszych 2 lat życia.2627

Potencjalne powikłania

Nieleczona lub niewłaściwie leczona atrezja płucna może prowadzić do szeregu powikłań:

  • Niewydolność serca1527
  • Zaburzenia rytmu serca (arytmie)1826
  • Infekcyjne zapalenie wsierdzia2427
  • Opóźniony wzrost i rozwój2426
  • Udar mózgu i drgawki2827
  • Enteropatia z utratą białka (zastój w wątrobie)15
  • Nagła śmierć1826

Rokowanie z odpowiednim leczeniem

Właściwe leczenie znacząco poprawia rokowanie:

  • Współczesne wskaźniki przeżywalności są znacznie lepsze niż w poprzednich dekadach4
  • Przy odpowiednim leczeniu i obserwacji, 65% pacjentów, którzy przeżyli pierwszy rok, może dożyć wieku powyżej 10 lat26
  • Chirurgiczna korekcja wady ma często doskonałe wyniki, choć zależy to od tego, czy operacja była naprawcza, czy paliatywna29

Konieczność natychmiastowego leczenia i długoterminowej obserwacji

Atrezja płucna wymaga natychmiastowej interwencji medycznej po wystąpieniu objawów.730 Leczenie ma na celu poprawę przepływu krwi do płuc i zazwyczaj obejmuje:

  • Podawanie prostaglandyn (PGE) bezpośrednio po urodzeniu, aby zapobiec zamknięciu przewodu tętniczego i umożliwić przepływ krwi do płuc313
  • Zabiegi chirurgiczne mające na celu poprawę przepływu krwi do płuc1632
  • W niektórych przypadkach konieczne jest przeprowadzenie kilku operacji w różnym wieku dziecka4

Po leczeniu chirurgicznym niemowlęta wymagają regularnych kontroli lekarskich przez całe życie, aby monitorować potencjalne powikłania.79 Długoterminowa opieka powinna być prowadzona w ośrodku specjalizującym się w leczeniu wrodzonych wad serca.1533 W ciągu życia pacjenta często konieczne jest przeprowadzenie kolejnych operacji, szczególnie w celu wymiany przewodów lub rewizji zabiegów paliatywnych.15

Wpływ na rozwój i jakość życia

Dzieci z atrezją płucną mogą doświadczać wyzwań związanych z rozwojem fizycznym, wytrzymałością i ogólnym stanem zdrowia.20 Mogą wymagać specjalistycznego wsparcia edukacyjnego lub dostosowań do aktywności fizycznej. Jednak przy odpowiednim leczeniu większość dzieci z atrezją płucną może prowadzić normalne życie, choć nadal istnieje ryzyko powikłań.28

Należy podkreślić, że wczesna diagnoza i natychmiastowe leczenie są kluczowe dla poprawy rokowania u pacjentów z atrezją płucną.20 Zachęcanie do zdrowego stylu życia i zapewnienie wsparcia emocjonalnego są również istotne dla ogólnego dobrostanu pacjentów.20

Kolejne rozdziały

Zapraszamy do dalszego czytania naszego leksykonu.

Wybierz kolejny rozdział z menu poniżej, aby otworzyć nową podstronę kompedium wiedzy i uzyskać szczegółowe informację o leku, substancji lub chorobie.

  1. 10.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 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.
  • #2 Pulmonary Atresia
    https://healthlibrary.aultcare.com/ReturningtoCare/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 regular follow-up care at a center offering pediatric or adult congenital heart care for the rest of their life.
  • #3 Pulmonary Atresia | Norton Children’s
    https://nortonchildrens.com/services/cardiology/conditions/congenital-heart-disease/pulmonary-atresia/
    Pulmonary atresia is a rare birth defect of the pulmonary valve. Sometimes the pulmonary valve is missing completely; other times the valve is blocked. […] Most babies with pulmonary atresia show symptoms during the first few hours of life. However, in some babies, symptoms do not show up until a few days after birth. […] Symptoms may include: Bluish skin tone (cyanosis), Clammy skin, Fast breathing, Poor feeding, Tiring easily while feeding, Working hard to breathe. […] Pulmonary atresia makes babies look blue (cyanotic) because not enough blood flow to the lungs. […] Initial treatment will focus on keeping the ductus arteriosus open with a medication called prostaglandin to keep blood flowing to the lungs. If the baby does not have a ventricular septal defect or a large atrial communication, a balloon atrial septostomy will be emergently performed. This will allow blood on the right side of the heart to get to the left side of the heart to be pumped out to the body. […] Surgery will be needed to create a way to get blood flow to the lungs. Different options include surgically reconstructing the right ventricular outflow tract.
  • #4 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: […] A bluish tinge to the skin, especially the lips, fingers and toes. […] Breathing problems. […] Fatigue or tiredness. […] 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. […] Pulmonary atresia life expectancy varies depending on how severe your child’s condition is and other individual factors. Survival rates are better today than they were in previous decades. […] 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.
  • #5 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.
  • #6 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.
  • #7 Pulmonary atresia – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/pulmonary-atresia/symptoms-causes/syc-20350727
    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. […] Pulmonary atresia happens as the baby’s heart is forming during pregnancy. Certain health conditions or illicit drug use during pregnancy may increase a baby’s risk of pulmonary atresia or other congenital heart defects. Risk factors include: […] 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.
  • #8 Pulmonary Atresia | Children’s Hospital of Philadelphia
    https://www.chop.edu/conditions-diseases/pulmonary-atresia
    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.
  • #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: […] A baby with pulmonary atresia may need surgery or other procedures soon after birth. Therefore, pulmonary atresia is considered a critical congenital heart defect (critical CHD). […] 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 – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/pulmonary-atresia-ventricular-septum-defect/symptoms-causes/syc-20580510
    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. […] 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.
  • #11 Pulmonary Atresia (PA) | Causes, Symptoms, Diagnosis & Treatment
    https://www.cincinnatichildrens.org/patients/child/encyclopedia/defects/pa
    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. […] The symptoms of pulmonary atresia may look like other medical conditions or heart problems. Always talk to your child’s doctor for a diagnosis.
  • #12 Pulmonary Atresia
    https://library.oumedicine.com/Wellness/Cholesterol/Tools/90,P01809
    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.
  • #13 Pulmonary Atresia | Conditions | UCSF Benioff Children’s Hospitals
    https://www.ucsfbenioffchildrens.org/conditions/pulmonary-atresia
    Babies with pulmonary atresia have reduced oxygen in their blood, a condition called hypoxia. They often have a small right ventricle as well. […] Babies with pulmonary atresia may turn blue, a condition called cyanosis; pulmonary atresia may also be called „blue baby syndrome.” Other symptoms include: […] Difficult or rapid breathing […] Lethargy […] Cool sweat.
  • #14 Pulmonary Atresia
    https://pihhealth.staywellknowledgebase.com/Library/Wellness/AtWork/90,P01809
    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.
  • #15 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).
  • #16 Pulmonary Atresia | Boston Children’s Hospital
    https://www.childrenshospital.org/conditions/pulmonary-atresia
    Symptoms of pulmonary atresia are noticeable shortly after a baby’s birth. The most obvious symptom is a bluish tint to the skin, called cyanosis. Other common symptoms include: […] If your baby’s pediatrician notices any of these symptoms, he or she will order testing right away. […] Babies born with pulmonary atresia need medication and surgery to correct the heart defect and improve blood flow to the lungs.
  • #17 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. […] 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. […] 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.
  • #18 SSA – POMS: DI 23022.585 – Pulmonary Atresia – 08/28/2020
    https://secure.ssa.gov/apps10/poms.nsf/lnx/0423022585
    Pulmonary atresia is a rare congenital heart disease in which the pulmonary valve does not form properly. 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. 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.
  • #19 Pulmonary Atresia: Overview, Causes, Treatment
    https://www.healthline.com/health/pulmonary-atresia
    Pulmonary atresia symptoms 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.
  • #20 Pulmonary Atresia: Causes, Symptoms, and Treatment – Longmore Clinic
    https://longmoreclinic.org/pulmonary-atresia-causes-symptoms-and-treatment/
    Pulmonary atresia can occur in various forms: […] 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. […] Children with pulmonary atresia may face challenges related to physical development, endurance, and overall health. They may require specialized educational support or accommodations for physical activities. Encouraging a healthy lifestyle and providing emotional support are crucial for their overall well-being.
  • #21 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.
  • #22 Pulmonary Atresia | The Texas Heart Institute®
    https://www.texasheart.org/heart-health/heart-information-center/topics/pulmonary-atresia/
    The lack of oxygen-rich blood makes the fingers, toes, and lips appear blue, a condition called cyanosis. […] Babies with pulmonary atresia seem normal before birth. This is because a passageway called the ductus arteriosus connects the pulmonary artery to the aorta in the fetal heart. The ductus arteriosus begins to close naturally after birth. In normal babies, a new valve opens that lets blood flow from the right ventricle to the lungs. But in infants with pulmonary atresia, the valve does not open. This means that the only path for blood to reach the lungs will soon begin to close.
  • #23 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.”
  • #24 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.
  • #25 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. […] 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. […] 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.
  • #26 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. […] On top of surgical and anesthesia complications in treated patients, some of the complications of PAVSD are: Congestive heart failure, Reactive erythrocytosis in response to chronic hypoxia, Infective endocarditis due to the aberrant flow of blood, Sepsis either due to infective endocarditis or poor immune system development, Delayed growth and puberty, Arrhythmias, Sudden death.
  • #27 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.
  • #28 Pulmonary atresia – Wikipedia
    https://en.wikipedia.org/wiki/Pulmonary_atresia
    The symptoms/signs of pulmonary atresia that will occur in babies are consistent with cyanosis, some fatigue and some shortness of breath (eating may be a problem as well). […] The prognosis for pulmonary atresia varies for every child. If the condition is left uncorrected it may be fatal, but the prognosis has greatly improved over the years for those with pulmonary atresia. Some factors that affect how well the child does include how well the heart is beating and the condition of the blood vessels that supply the heart. Most cases of pulmonary atresia can be helped with surgery. If the patient’s right ventricle is exceptionally small, many surgeries will be needed in order to help stimulate normal circulation of blood to the heart. If uncorrected, babies with this type of congenital heart disease may only survive for the first few days of life. Many children with pulmonary atresia will go on to lead normal lives, though complications such as endocarditis, stroke and seizures are possible.
  • #29 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.
  • #30 Pulmonary Valve Atresia
    https://www.rwjbh.org/treatment-care/heart-and-vascular-care/diseases-conditions/congenital-heart-disease/pulmonary-valve-atresia/
    If your baby is born with pulmonary atresia, symptoms will be noticeable soon after birth. Your baby’s signs and symptoms may include: A bluish discoloration of the lips and skin caused by low oxygen (cyanosis). Fast breathing or shortness of breath. Easily tiring or being fatigued. Feeding problems. […] Your baby will need urgent medical attention once pulmonary atresia symptoms develop.
  • #31 Pulmonary Atresia with Intact Ventricular Septum (IVS) | Texas Children’s
    https://www.texaschildrens.org/content/conditions/pulmonary-atresia-with-intact-ventricular-septum-ivs
    Babies born with pulmonary atresia don’t have enough oxygen in their blood to support the body’s needs. Treatment at birth is necessary for survival. […] In some cases, the condition isn’t diagnosed until after the baby is born. Symptoms in a newborn may include: […] Bluish tint to the skin, lips and nails (cyanosis) or pale skin, indicating a lack of oxygen in the blood […] Difficulty breathing or rapid breathing […] Fatigue […] Clammy skin […] Feeding problems. […] Treatment is required after birth to improve blood flow to the lungs and meet the baby’s oxygen needs. A medication to prevent the closure of the ductus arteriosus, called prostaglandins (PGE), is started as soon as the baby is born. This medication enables blood to continue to flow to the lungs for oxygen until a more permanent treatment strategy is determined. […] Babies born with pulmonary atresia with intact ventricular septum require lifelong monitoring by cardiologists experienced in the treatment of congenital heart defects.
  • #32 Pulmonary Atresia
    https://healthlibrary.ecuhealth.org/library/Wellness/Behavior/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. […] 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. Your child’s healthcare provider may advise pain medicines such as acetaminophen or ibuprofen to keep your baby comfortable. […] 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.
  • #33 Pulmonary Atresia
    https://healthlibrary.vidanthealth.com/Conditions/Heart/90,P01809
    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. […] Your child will need regular follow-up care at a center offering pediatric or adult congenital heart care for the rest of their life.