Atrezja płucna
Diagnostyka i diagnoza

Atrezja płucna to wrodzona wada serca charakteryzująca się całkowitym brakiem drożności zastawki płucnej, co uniemożliwia przepływ krwi z prawej komory do tętnicy płucnej. Diagnostyka może być przeprowadzona prenatalnie, głównie w II trymestrze (18-22 tydzień) za pomocą echokardiografii płodowej, w tym technik 3D/STIC, które pozwalają na ocenę struktury serca, obecności hipoplazji prawej komory, braku przepływu przez zastawkę oraz odwrócenia przepływu przez przewód tętniczy. Po urodzeniu rozpoznanie opiera się na objawach klinicznych, takich jak sinica i niska saturacja tlenem (pulsoksymetria), oraz badaniach obrazowych: echokardiogramie, RTG klatki piersiowej, EKG i cewnikowaniu serca, które umożliwiają ocenę anatomii, funkcji prawej komory, obecności VSD, PDA oraz krążenia obocznego (MAPCAs). W diagnostyce stosuje się także wskaźnik McGoon do oceny proporcji tętnic płucnych względem aorty zstępującej, co ma znaczenie prognostyczne i terapeutyczne.

Diagnostyka atrezji płucnej

Atrezja płucna to wrodzona wada serca charakteryzująca się brakiem prawidłowo ukształtowanej zastawki pomiędzy prawą komorą serca a tętnicą płucną, co uniemożliwia przepływ krwi z serca do płuc. Diagnoza tej wady może nastąpić zarówno w okresie prenatalnym, jak i krótko po urodzeniu, a wczesne rozpoznanie jest kluczowe dla szybkiego wdrożenia leczenia ratującego życie.

Diagnostyka prenatalna

Atrezja płucna może zostać wykryta już w okresie prenatalnym podczas rutynowych badań ultrasonograficznych ciąży. Podejrzenie tej wady często pojawia się podczas standardowego badania USG w drugim trymestrze ciąży (18-22 tydzień), gdy widoczne są nieprawidłowości w budowie serca płodu.12

Jeśli istnieją podejrzenia wady serca podczas rutynowego USG, pacjentka kierowana jest na bardziej szczegółowe badanie – płodowe badanie echokardiograficzne (echo płodowe). Jest to specjalistyczne USG serca płodu wykonywane przez kardiologa dziecięcego, które pozwala na dokładną ocenę struktury i funkcji serca, szczególnie zastawki płucnej i przepływu krwi.34

Wczesna diagnoza prenatalna umożliwia:5

  • Zaplanowanie opieki okołoporodowej
  • Konsultacje z zespołem specjalistów przed urodzeniem dziecka
  • Wybór odpowiedniego ośrodka do porodu z dostępem do intensywnej opieki noworodkowej
  • Przygotowanie do natychmiastowej interwencji po urodzeniu

Podczas diagnostyki prenatalnej można zaobserwować charakterystyczne cechy atrezji płucnej:67

  • Brak przepływu krwi przez zastawkę płucną
  • Odwrócenie przepływu krwi przez przewód tętniczy (wsteczny przepływ jest praktycznie zawsze obecny w przypadku atrezji płucnej z nienaruszonym przegrodą międzykomorową)
  • Hipoplazja (niedorozwój) prawej komory
  • Pogrubienie ściany prawej komory

Trójwymiarowa echokardiografia z wykorzystaniem techniki korelacji czasowo-przestrzennej (STIC) może dostarczyć dodatkowych informacji przestrzennych, ukazując szczegółowo duże naczynia, co stanowi cenne uzupełnienie tradycyjnej echokardiografii dwuwymiarowej.8

Diagnostyka po urodzeniu

Większość przypadków atrezji płucnej jest diagnozowana w pierwszych godzinach lub dniach po urodzeniu. Pierwszym sygnałem wskazującym na problem może być sinica (niebieskawa barwa skóry i błon śluzowych) spowodowana niedoborem tlenu we krwi.910

Badanie fizykalne noworodka obejmuje:1112

  • Ocenę koloru skóry – sinica jest głównym objawem atrezji płucnej
  • Osłuchiwanie serca i płuc stetoskopem – lekarz może wykryć szmer serca (nieprawidłowy dźwięk spowodowany turbulentnym przepływem krwi)
  • Ocenę rytmu oddechowego – przyspieszony oddech może wskazywać na problem z sercem

Niektóre noworodki są diagnozowane podczas badania przesiewowego tlenem (pulsoksymetria), które jest wykonywane rutynowo około doby po urodzeniu. Niski poziom tlenu we krwi może wskazywać na krytyczną wrodzoną wadę serca, taką jak atrezja płucna.1314

Badania diagnostyczne

W celu potwierdzenia diagnozy atrezji płucnej wykonuje się szereg badań:1516

Echokardiogram

Echokardiogram (echo) jest podstawowym badaniem w diagnostyce atrezji płucnej. To nieinwazyjne badanie wykorzystuje fale ultradźwiękowe do utworzenia ruchomego obrazu serca i zastawek serca. Pozwala na ocenę:1718

  • Struktury zastawki płucnej
  • Wielkości i funkcji prawej komory
  • Obecności defektów przegrody międzykomorowej (VSD)
  • Obecności i wielkości przewodu tętniczego (PDA)
  • Przepływu krwi przez serce
Pulsoksymetria

To nieinwazyjny test mierzący saturację (nasycenie krwi tlenem) za pomocą małego czujnika umieszczanego na palcu lub stopie dziecka. Niski poziom tlenu we krwi jest charakterystyczny dla atrezji płucnej.1920

Rentgen klatki piersiowej

Badanie RTG klatki piersiowej umożliwia wizualizację struktury serca i płuc. Może wykazać:2122

  • Zmieniony kształt serca
  • Zmniejszony przepływ krwi do płuc
  • Strukturalne problemy związane z atrezją płucną
Elektrokardiogram

Elektrokardiogram (EKG) rejestruje aktywność elektryczną serca. To bezbolesne, nieinwazyjne badanie może wykazać:2324

  • Nieprawidłowy rytm serca
  • Przerost prawej komory
  • Obciążenie mięśnia sercowego
Cewnikowanie serca

Cewnikowanie serca to inwazyjne badanie, które dostarcza szczegółowych informacji o strukturze wewnętrznej serca. Podczas badania:2526

  • Dziecko otrzymuje leki uspokajające
  • Cienki, elastyczny cewnik wprowadzany jest do naczynia krwionośnego w pachwinie i kierowany do serca
  • Wykonywane są pomiary ciśnienia krwi i poziomu tlenu w poszczególnych komorach serca
  • Wstrzykiwany jest środek kontrastowy dla lepszej wizualizacji struktur serca

To badanie pozwala także na ocenę tętnic płucnych i aorty oraz może umożliwić przeprowadzenie pewnych interwencji terapeutycznych podczas tej samej procedury.27

Dodatkowe badania obrazowe

W niektórych przypadkach wykonuje się dodatkowe badania obrazowe:282930

  • Tomografia komputerowa (CT) serca – dostarcza szczegółowych obrazów serca i naczyń krwionośnych, pozwala ocenić obecność MAPCAs (głównych tętnic krążenia obocznego aortalno-płucnego)
  • Rezonans magnetyczny (MRI) serca – oferuje dokładne obrazy serca bez narażenia na promieniowanie, umożliwia ocenę funkcji komór i przepływu krwi

Ocena ciężkości atrezji płucnej

Podczas diagnostyki atrezji płucnej istotne jest określenie typu i ciężkości wady, co wpływa na dalsze postępowanie terapeutyczne. Ocena obejmuje:3132

  • Wielkość prawej komory i tętnicy płucnej
  • Zdolność prawej komory do pompowania krwi
  • Obecność lub brak ubytku przegrody międzykomorowej (VSD)
  • Źródła dopływu krwi do płuc
  • Obecność i rozmiar krążenia obocznego aortalno-płucnego

W ocenie proporcjonalnej wielkości tętnic płucnych w stosunku do aorty zstępującej stosuje się wskaźnik McGoon. Oblicza się go sumując średnice prawej i lewej tętnicy płucnej i dzieląc tę sumę przez średnicę aorty zstępującej.33

Różnicowanie typów atrezji płucnej

W trakcie diagnostyki ważne jest rozróżnienie dwóch głównych typów atrezji płucnej:3435

  • Atrezja płucna z nienaruszonym przegrodą międzykomorową (PA/IVS) – charakteryzuje się całkowitą niedrożnością drogi odpływu prawej komory bez połączenia między prawą a lewą komorą.
  • Atrezja płucna z ubytkiem przegrody międzykomorowej (PA/VSD) – często nazywana tetralogią Fallota z atrezją płucną, umożliwia przepływ krwi między komorami przez ubytek przegrody.

Każdy z tych typów wymaga nieco odmiennego podejścia diagnostycznego i terapeutycznego.36

Badania genetyczne

W niektórych przypadkach zalecane są badania genetyczne, zwłaszcza gdy atrezji płucnej towarzyszą inne wady wrodzone. Badania te mogą pomóc w:3738

  • Określeniu przyczyny wady
  • Ocenie ryzyka wystąpienia podobnych wad u przyszłego potomstwa
  • Zidentyfikowaniu zespołów genetycznych związanych z wadami serca

Multidyscyplinarne podejście do diagnostyki

Diagnostyka atrezji płucnej wymaga współpracy wielu specjalistów:3940

  • Kardiologów dziecięcych – specjalizujących się w diagnozowaniu i leczeniu wrodzonych wad serca
  • Neonatologów – zajmujących się opieką nad noworodkami z problemami zdrowotnymi
  • Specjalistów medycyny matczyno-płodowej – w przypadku diagnozy prenatalnej
  • Kardiochirurgów – planujących interwencje chirurgiczne
  • Genetyków – w przypadku podejrzenia zespołu genetycznego

Ośrodki specjalistyczne oferują kompleksową diagnostykę atrezji płucnej, co pozwala na szybkie rozpoznanie wady i wdrożenie odpowiedniego leczenia.41

Znaczenie wczesnej diagnostyki

Szybka diagnoza atrezji płucnej jest kluczowa dla przeżycia dziecka, ponieważ:4243

  • Bez leczenia atrezja płucna jest śmiertelna ze względu na niski poziom tlenu we krwi
  • Wczesna diagnoza pozwala na natychmiastowe podanie prostaglandyny E1 (PGE) po urodzeniu, co utrzymuje drożność przewodu tętniczego
  • Umożliwia zaplanowanie odpowiednich zabiegów chirurgicznych
  • Zwiększa szanse na poprawę krążenia i natlenienia organizmu

Opieka po diagnostyce

Po zdiagnozowaniu atrezji płucnej konieczna jest regularna i długoterminowa kontrola kardiologiczna. Dzieci z atrezją płucną wymagają:4445

  • Regularnych wizyt u kardiologa dziecięcego
  • Okresowych badań obrazowych oceniających efekty leczenia
  • Opieki w ośrodku specjalizującym się w leczeniu wrodzonych wad serca przez całe życie
  • Monitorowania potencjalnych powikłań

Regularna opieka podiagnostyczna może zmniejszyć ryzyko powikłań i poprawić jakość życia pacjentów z atrezją płucną.46

Wyzwania diagnostyczne

Diagnostyka atrezji płucnej może być trudna, szczególnie w przypadku:4748

  • Hipoplastycznej (słabo rozwiniętej) tętnicy płucnej
  • Współistniejących złożonych wad serca
  • Wczesnej ciąży, gdy struktury serca są jeszcze małe

Ze względu na te wyzwania, dokładna ocena anatomii i hemodynamiki serca płodu jest kluczowa dla wykrycia atrezji płucnej, a potwierdzenie diagnozy po urodzeniu jest niezbędne dla pewnej diagnozy.4950

Badania diagnostyczne muszą być wykonywane przez doświadczonych specjalistów w ośrodkach z odpowiednim zapleczem technicznym, co zwiększa dokładność diagnozy i umożliwia optymalne planowanie leczenia.51

Nowoczesne techniki diagnostyczne

Rozwój technologii medycznych pozwala na coraz dokładniejszą diagnostykę atrezji płucnej:5253

  • Zaawansowane techniki echokardiograficzne, w tym echo 3D i 4D
  • Obrazowanie magnetyczno-rezonansowe płodu
  • Tomografia komputerowa o wysokiej rozdzielczości
  • Obrazowanie przepływu metodą Dopplera tkankowego

Te nowoczesne techniki dostarczają bardziej szczegółowych informacji o anatomii i funkcji serca, co umożliwia precyzyjniejsze planowanie leczenia.54

W diagnostyce prenatalnej coraz częściej stosuje się badania przesiewowe w pierwszym trymestrze ciąży, co może pozwolić na wcześniejsze wykrycie niektórych przypadków atrezji płucnej.55

Echokardiografia płodowa pozostaje głównym narzędziem diagnostycznym w wykrywaniu atrezji płucnej w okresie prenatalnym, ale ze względu na postępujący charakter tej wady w łonie matki, konieczne są sekwencyjne badania ultradźwiękowe w drugim i trzecim trymestrze ciąży w celu monitorowania progresji choroby i zmian hemodynamicznych.56

Dokładna diagnoza prenatalna i stratyfikacja ryzyka są kluczowe dla efektywnego poradnictwa prenatalnego, zarządzania okresem okołoporodowym i planowania leczenia po urodzeniu.57

Kompleksowa diagnostyka atrezji płucnej umożliwia wdrożenie optymalnego leczenia, które w wielu przypadkach może znacząco poprawić rokowanie u dzieci z tą ciężką wrodzoną wadą serca.58

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  1. 11.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #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.
  • #2 Pulmonary Atresia: Symptoms, Causes and Treatment
    https://my.clevelandclinic.org/health/diseases/14779-pulmonary-atresia
    Pulmonary atresia is a congenital heart defect, which means its present at birth. […] Pulmonary atresia is a congenital (present at birth) heart defect in which your pulmonary valve doesnt develop normally or stays blocked after birth. […] How is pulmonary atresia diagnosed? […] While youre pregnant, your healthcare provider will do standard screenings to check on the health of the fetus. […] 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.
  • #3 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.
  • #4 Pulmonary Atresia (PA) | Causes, Symptoms, Diagnosis & Treatment
    https://www.cincinnatichildrens.org/patients/child/encyclopedia/defects/pa
    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. […] 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.
  • #5 Pulmonary Atresia | Texas Children’s
    https://www.texaschildrens.org/content/conditions/pulmonary-atresia
    Pulmonary atresia may be detected during pregnancy through a routine fetal echocardiogram (ultrasound of the fetal heart). A diagnosis during pregnancy enables your family and your healthcare team to plan ahead for the specialized treatment and cardiac expertise your baby will need at birth, optimizing outcomes. […] In some cases the condition isn’t diagnosed until after the baby is born. Symptoms in a newborn may include: […] If pulmonary atresia is detected during pregnancy, prompt referral to a fetal cardiac center is important for further evaluation and proper treatment planning. […] At Texas Children’s Fetal Center, we arrange for you to visit as quickly as possible to meet with a team of specialists experienced in diagnosing and treating rare congenital heart defects, including maternal-fetal medicine physicians (OB/GYNs specializing in high-risk pregnancies), fetal and pediatric cardiologists, fetal imaging experts, congenital heart surgeons, neonatologists, and genetic counselors.
  • #6 Prenatal Diagnosis of Pulmonary Atresia With Ventricular Septal Defect and an Aberrant Ductus Arteriosus in a Dextrocardia by Two- and Three-Dimensional Echocardiography: A Case Report
    https://pmc.ncbi.nlm.nih.gov/articles/PMC9283767/
    Prenatal diagnosis of pulmonary atresia is difficult in relative, especially when the pulmonary artery is slim and hypoplastic in development. […] It is difficult to make an accurate diagnosis of pulmonary atresia prenatally, especially when the pulmonary artery is slim and hypoplastic. […] A detailed evaluation of fetal cardiac anatomy and hemodynamics is crucial for the detection of an aberrant ductus arteriosus, which plays an important role in the diagnosis of pulmonary atresia with ventricular septal defect. […] The strategy for the diagnosis of PA-VSD together with the experience for tracing the aberrant DA was summarized in this report. […] The three-dimensional echocardiography with spatio-temporal image correlation technique could provide additional spatial information to show great arteries in detail, which can serve as a supplement to traditional two-dimensional modality and benefit examiners to make an accurate diagnosis.
  • #7 Prenatal Diagnosis of Pulmonary Atresia with Intact Ventricular Septum
    https://www.contemporaryobgyn.net/view/prenatal-diagnosis-pulmonary-atresia-intact-ventricular-septum
    Hypoplasia of the right ventricle is uncommon as an isolated entity. It may result from tricuspid atresia, but is most commonly secondary to pulmonary atresia with intact ventricular septum. […] Pulmonary atresia with intact ventricular septum is rare, accounting for 1-3% of cases of congenital heart disease. It occurs in 0.1 to 0.4 in 10,000 live births. Pulmonary atresia with intact ventricular septum has been reported to occur slightly more frequently in males. […] Fetal echocardiogram revealed a four-chamber heart correctly oriented in the left chest. The right ventricle appeared small and severely hypertrophied. A small pulmonary artery was visualized with an atretic pulmonic valve. Pulsed Doppler distal to the pulmonic valve confirmed absence of flow. […] Because a normal caliber main pulmonary artery does not exclude the diagnosis of pulmonary atresia, demonstrating reversal of blood flow through the ductus is essential. Retrograde blood flow through the ductus is virtually always present in pulmonary atresia with intact ventricular septum. […] The outcome for infants born with pulmonary atresia and intact ventricular septum is poor. They are dependent on a patent ductus arteriosus for survival in the neonatal period. Administration of prostaglandin E1 at birth is essential to maintain patency until surgery can be performed.
  • #8 Prenatal Diagnosis of Pulmonary Atresia With Ventricular Septal Defect and an Aberrant Ductus Arteriosus in a Dextrocardia by Two- and Three-Dimensional Echocardiography: A Case Report
    https://pmc.ncbi.nlm.nih.gov/articles/PMC9283767/
    Prenatal diagnosis of pulmonary atresia is difficult in relative, especially when the pulmonary artery is slim and hypoplastic in development. […] It is difficult to make an accurate diagnosis of pulmonary atresia prenatally, especially when the pulmonary artery is slim and hypoplastic. […] A detailed evaluation of fetal cardiac anatomy and hemodynamics is crucial for the detection of an aberrant ductus arteriosus, which plays an important role in the diagnosis of pulmonary atresia with ventricular septal defect. […] The strategy for the diagnosis of PA-VSD together with the experience for tracing the aberrant DA was summarized in this report. […] The three-dimensional echocardiography with spatio-temporal image correlation technique could provide additional spatial information to show great arteries in detail, which can serve as a supplement to traditional two-dimensional modality and benefit examiners to make an accurate diagnosis.
  • #9 Pulmonary Atresia | Diagnosis | UK Healthcare
    https://ukhealthcare.uky.edu/gill-heart-vascular-institute/conditions/adult-congenital-heart-disease/pulmonary-atresia/diagnosis
    Diagnosing pulmonary atresia involves a series of exams and tests to confirm the condition. […] During a physical exam, healthcare providers assess for: Cyanosis (a blue tint to the skin, lip and nails caused by a shortage of oxygen in the blood), Rapid breathing, Heart murmurs. […] The presence of these symptoms may lead to further evaluation. […] An echocardiogram (echo) records activity of the heart using high-frequency sound waves, or ultrasound. […] A chest X-ray takes pictures of your heart, lungs and arteries. […] An electrocardiogram provides information about your hearts electrical activity. […] Pulse oximetry is a painless test that measures the blood’s oxygen saturation levels. These levels can indicate the presence of a heart defect.
  • #10 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. […] 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. […] Cyanosis is a major sign that there is a problem with your newborn. Your child’s doctor may have also heard a heart murmur during a physical examination. A heart murmur is a noise caused by the turbulence of blood flowing through the openings that allow the blood to mix, or through abnormal valves between the different parts of the heart. […] Other tests are needed to help with the diagnosis. They may include: Chest X-ray – a diagnostic test that uses invisible electromagnetic energy beams to create images of internal tissues, bones, and organs onto film.
  • #11 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.
  • #12 Pulmonary Atresia | Boston Children’s Hospital
    https://www.childrenshospital.org/conditions/pulmonary-atresia
    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). […] Sometimes pulmonary atresia is first suspected during newborn screening, using pulse oximetry, a painless bedside test that uses a light probe, attached to the hand or foot, to detect the amount of oxygen in the blood. […] 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. […] The cardiologist may order one or more of the following tests to help diagnose pulmonary atresia: Electrocardiogram (ECG or EKG), Echocardiogram (cardiac ultrasound), Cardiac MRI, Cardiac catheterization, Chest X-ray.
  • #13 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.
  • #14 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. […] Doctors can diagnose pulmonary atresia when a baby is in the womb using a fetal echocardiogram (fetal echo). This is a special ultrasound that uses sound waves to view and make pictures of a developing babys heart during pregnancy. The results are interpreted by a pediatric heart doctor (cardiologist) who specializes in fetal congenital heart disease. […] To diagnose this condition, your childs doctor will examine your baby and use a stethoscope to listen to their heart. In children with this condition, doctors often notice cyanosis, and sometimes they hear a heart murmur the sound of blood moving in the heart in a way thats not normal. […] Your child will need an echocardiogram test so the doctor can see how their heart works.
  • #15 Pulmonary Atresia
    https://encyclopedia.nm.org/library/testsprocedures/LabTests/90,P01809
    PA may be found during a routine ultrasound in pregnancy. […] At birth, the healthcare providers and nurses will examine your baby. They will listen to your baby’s heart and lungs with a stethoscope and note any signs of a heart defect. […] Testing for heart defects varies by the child’s age, condition, and other things. Some tests that may be done include: […] Pulse oximetry. This test checks oxygen levels in the blood through a tiny clip or band on the baby’s hand or foot. PA causes low oxygen levels. […] Chest X-ray. A chest X-ray shows pictures of the heart and lungs. It may show structural problems that occur with PA. […] Electrocardiogram (ECG). This test records the electrical activity of the heart. It shows abnormal rhythms and heart muscle stress. […] Echocardiogram (echo). Echo uses sound waves to record a moving picture of the heart and heart valves. This test is the best test to diagnose PA.
  • #16 Pulmonary Atresia
    http://healthlibrary.umcno.org/Conditions/Pregnancy/Tools/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. […] PA may be found during a routine ultrasound in pregnancy. […] At birth, the healthcare providers and nurses will examine your baby. They will listen to your baby’s heart and lungs with a stethoscope and note any signs of a heart defect. […] Testing for heart defects varies by the child’s age, condition, and other things. Some tests that may be done include: […] Pulse oximetry. This test checks oxygen levels in the blood through a tiny clip or band on the baby’s hand or foot. PA causes low oxygen levels. […] Chest X-ray. A chest X-ray shows pictures of the heart and lungs. It may show structural problems that occur with PA.
  • #17 Pulmonary Atresia
    https://encyclopedia.nm.org/library/testsprocedures/LabTests/90,P01809
    PA may be found during a routine ultrasound in pregnancy. […] At birth, the healthcare providers and nurses will examine your baby. They will listen to your baby’s heart and lungs with a stethoscope and note any signs of a heart defect. […] Testing for heart defects varies by the child’s age, condition, and other things. Some tests that may be done include: […] Pulse oximetry. This test checks oxygen levels in the blood through a tiny clip or band on the baby’s hand or foot. PA causes low oxygen levels. […] Chest X-ray. A chest X-ray shows pictures of the heart and lungs. It may show structural problems that occur with PA. […] Electrocardiogram (ECG). This test records the electrical activity of the heart. It shows abnormal rhythms and heart muscle stress. […] Echocardiogram (echo). Echo uses sound waves to record a moving picture of the heart and heart valves. This test is the best test to diagnose PA.
  • #18 Pulmonary Atresia
    http://healthlibrary.umcno.org/Conditions/Pregnancy/Tools/90,P01809
    Electrocardiogram (ECG). This test records the electrical activity of the heart. It shows abnormal rhythms and heart muscle stress. […] Echocardiogram (echo). Echo uses sound waves to record a moving picture of the heart and heart valves. This test is the best test to diagnose PA. […] Cardiac catheterization (heart cath). The baby is sedated with medicine. Then a thin, flexible tube (catheter) is put into a blood vessel in the groin and guided to the heart. A heart cath gives very detailed information about the heart. This includes blood pressure and oxygen measurements in the chambers of the heart, as well as the pulmonary artery and aorta. Contrast dye is also injected to better see the structures in the heart.
  • #19 Pulmonary Atresia: Symptoms, Causes and Treatment
    https://my.clevelandclinic.org/health/diseases/14779-pulmonary-atresia
    Pulmonary atresia is a congenital heart defect, which means its present at birth. […] Pulmonary atresia is a congenital (present at birth) heart defect in which your pulmonary valve doesnt develop normally or stays blocked after birth. […] How is pulmonary atresia diagnosed? […] While youre pregnant, your healthcare provider will do standard screenings to check on the health of the fetus. […] 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.
  • #20 Pulmonary Atresia
    https://encyclopedia.nm.org/library/testsprocedures/LabTests/90,P01809
    PA may be found during a routine ultrasound in pregnancy. […] At birth, the healthcare providers and nurses will examine your baby. They will listen to your baby’s heart and lungs with a stethoscope and note any signs of a heart defect. […] Testing for heart defects varies by the child’s age, condition, and other things. Some tests that may be done include: […] Pulse oximetry. This test checks oxygen levels in the blood through a tiny clip or band on the baby’s hand or foot. PA causes low oxygen levels. […] Chest X-ray. A chest X-ray shows pictures of the heart and lungs. It may show structural problems that occur with PA. […] Electrocardiogram (ECG). This test records the electrical activity of the heart. It shows abnormal rhythms and heart muscle stress. […] Echocardiogram (echo). Echo uses sound waves to record a moving picture of the heart and heart valves. This test is the best test to diagnose PA.
  • #21 Pulmonary Atresia: Symptoms, Causes and Treatment
    https://my.clevelandclinic.org/health/diseases/14779-pulmonary-atresia
    Pulmonary atresia is a congenital heart defect, which means its present at birth. […] Pulmonary atresia is a congenital (present at birth) heart defect in which your pulmonary valve doesnt develop normally or stays blocked after birth. […] How is pulmonary atresia diagnosed? […] While youre pregnant, your healthcare provider will do standard screenings to check on the health of the fetus. […] 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.
  • #22 Pulmonary Atresia
    https://encyclopedia.nm.org/library/testsprocedures/LabTests/90,P01809
    PA may be found during a routine ultrasound in pregnancy. […] At birth, the healthcare providers and nurses will examine your baby. They will listen to your baby’s heart and lungs with a stethoscope and note any signs of a heart defect. […] Testing for heart defects varies by the child’s age, condition, and other things. Some tests that may be done include: […] Pulse oximetry. This test checks oxygen levels in the blood through a tiny clip or band on the baby’s hand or foot. PA causes low oxygen levels. […] Chest X-ray. A chest X-ray shows pictures of the heart and lungs. It may show structural problems that occur with PA. […] Electrocardiogram (ECG). This test records the electrical activity of the heart. It shows abnormal rhythms and heart muscle stress. […] Echocardiogram (echo). Echo uses sound waves to record a moving picture of the heart and heart valves. This test is the best test to diagnose PA.
  • #23 Pulmonary Atresia: Symptoms, Causes and Treatment
    https://my.clevelandclinic.org/health/diseases/14779-pulmonary-atresia
    Electrocardiogram (EKG or ECG): A painless and noninvasive way to measure the electrical activity of your babys heart to find out if theres any stress on their heart muscle. […] Echocardiogram: A painless, noninvasive imaging method. It uses harmless sound waves to produce a moving picture of your babys heart and heart valves. […] Cardiac catheterization: An invasive procedure that lets a provider view your babys heart. […] How is pulmonary atresia treated? […] Usually, children with pulmonary valve atresia will need surgery to improve blood flow to their lungs. […] Pulmonary atresia treatment depends on how the condition affects your child and on their: […] General health. […] Age. […] Medical history. […] The type of surgery your baby needs for a pulmonary atresia repair will depend on several factors, including:
  • #24 Pulmonary Atresia
    https://encyclopedia.nm.org/library/testsprocedures/LabTests/90,P01809
    PA may be found during a routine ultrasound in pregnancy. […] At birth, the healthcare providers and nurses will examine your baby. They will listen to your baby’s heart and lungs with a stethoscope and note any signs of a heart defect. […] Testing for heart defects varies by the child’s age, condition, and other things. Some tests that may be done include: […] Pulse oximetry. This test checks oxygen levels in the blood through a tiny clip or band on the baby’s hand or foot. PA causes low oxygen levels. […] Chest X-ray. A chest X-ray shows pictures of the heart and lungs. It may show structural problems that occur with PA. […] Electrocardiogram (ECG). This test records the electrical activity of the heart. It shows abnormal rhythms and heart muscle stress. […] Echocardiogram (echo). Echo uses sound waves to record a moving picture of the heart and heart valves. This test is the best test to diagnose PA.
  • #25 Pulmonary Atresia
    https://encyclopedia.nm.org/library/testsprocedures/LabTests/90,P01809
    Cardiac catheterization (heart cath). The baby is sedated with medicine. Then a thin, flexible tube (catheter) is put into a blood vessel in the groin and guided to the heart. A heart cath gives very detailed information about the heart. This includes blood pressure and oxygen measurements in the chambers of the heart, as well as the pulmonary artery and aorta. Contrast dye is also injected to better see the structures in the heart.
  • #26 Pulmonary Atresia – Stanford Medicine Children’s Health
    https://www.stanfordchildrens.org/en/topic/default?id=pulmonary-atresia-90-P01809
    Cardiac catheterization (heart cath). The baby gets medicine to sedate him or her. Then a thin, flexible tube (catheter) is put into a blood vessel in the groin and guided to the heart. A heart cath gives very detailed information about the heart. This includes blood pressure and oxygen measurements in the chambers of the heart, as well as the pulmonary artery and aorta. Contrast dye is also injected to better see the structures in the heart.
  • #27 Get Pulmonary Valve Atresia Treatment | Cleveland Clinic Children’s
    https://my.clevelandclinic.org/pediatrics/services/pulmonary-valve-atresia-treatment
    A chest X-ray alone wont diagnose pulmonary valve atresia. But this painless test give us information about your babys heart and lungs. Well also use X-rays to help rule out other conditions, like pneumonia. […] If we need to see inside your babys heart, our interventional cardiologists may do a cardiac catheterization. Dont worry. This isnt heart surgery. First, theyll insert a thin tube (catheter) into one of your babys blood vessels (usually in their arm or leg) and then move it through to the inside of their heart. This test measures blood pressure and oxygen levels and gives better details about their congenital heart condition. Sometimes, a temporary intervention is needed to improve your babys condition before heart surgery. This procedure is done during the cardiac catheterization.
  • #28 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.
  • #29 Pulmonary Atresia | Nationwide Children’s Hospital
    https://www.nationwidechildrens.org/conditions/pulmonary-atresia
    Cardiac MRI: A test that uses radio waves, magnets, and a computer to make detailed pictures of the heart and blood vessels. […] Some congenital conditions are caused by a baby’s DNA. […] The doctors may suggest genetic testing to find out more about this. […] Your baby may need help with breathing, extra oxygen, or may need to be started on a medicine called prostaglandin E (PGE, or „prostins”). […] If your baby’s condition is severe, they will need to be transferred to Nationwide Children’s Hospital Cardiothoracic Intensive Care Unit (CTICU) shortly after birth by the Nationwide Children’s Mobile Intensive Care team. […] After surgery, your baby will go to the Cardiothoracic Intensive Care Unit (CTICU). […] Your hospital stay after birth may be a couple of days or as long as weeks to months. […] Your cardiologist will follow up with your baby’s heart needs even after they are discharged from the hospital.
  • #30 Pulmonary artery atresia | Radiology Reference Article | Radiopaedia.org
    https://radiopaedia.org/articles/pulmonary-artery-atresia?lang=us
    Pulmonary artery atresia, sometimes known as pulmonary atresia, is a congenital cardiovascular anomaly where there is complete disruption between the right ventricular outflow tract (RVOT) and the pulmonary trunk. […] Echocardiography can characterize intracardiac anatomy. Has a limited role in assessing pulmonary artery anatomy. […] CT is better than echocardiography for pulmonary artery anatomy. It allows evaluation of the following: length of pulmonary atresia, presence of pulmonary artery confluence, size of main, right, and left pulmonary arteries at the origin and at the hilum, presence of branch pulmonary artery stenosis, sources of pulmonary blood flow to each lung, including the number of bronchopulmonary segments supplied by native pulmonary arteries and the distribution of major aortopulmonary collateral arteries (MAPCAs). […] MRI allows direct visualization of the anomaly. Cine sequences may show a dilated non contracting right ventricle. MR angiography allows detection of aortopulmonary collaterals and patent ductus arteriosus (if present).
  • #31 Pulmonary Atresia: Symptoms, Causes and Treatment
    https://my.clevelandclinic.org/health/diseases/14779-pulmonary-atresia
    Electrocardiogram (EKG or ECG): A painless and noninvasive way to measure the electrical activity of your babys heart to find out if theres any stress on their heart muscle. […] Echocardiogram: A painless, noninvasive imaging method. It uses harmless sound waves to produce a moving picture of your babys heart and heart valves. […] Cardiac catheterization: An invasive procedure that lets a provider view your babys heart. […] How is pulmonary atresia treated? […] Usually, children with pulmonary valve atresia will need surgery to improve blood flow to their lungs. […] Pulmonary atresia treatment depends on how the condition affects your child and on their: […] General health. […] Age. […] Medical history. […] The type of surgery your baby needs for a pulmonary atresia repair will depend on several factors, including:
  • #32 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. […] 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. […] Pulse oximetry evaluates oxygen saturation, especially in dark-skinned infants, where cyanosis may be missed during the physical exam.
  • #33 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 calculation involves summing the diameters of the right and left pulmonary arteries and dividing this total by the diameter of the descending aorta. […] Surgical intervention, often in multiple stages, is required to establish pulmonary blood flow and improve long-term survival. […] In patients with a completely atretic pulmonary system or who have failed the surgical corrective measures, a cardiac transplant can be a viable option.
  • #34 Pulmonary atresia with intact ventricular septum (PA/IVS) – UpToDate
    https://www.uptodate.com/contents/pulmonary-atresia-with-intact-ventricular-septum-pa-ivs
    Pulmonary atresia with intact ventricular septum (PA/IVS) is characterized by complete obstruction to right ventricular (RV) outflow with varying degrees of RV and tricuspid valve (TV) hypoplasia. Blood is thus unable to flow from the RV to the pulmonary artery and lungs, and an alternative source of pulmonary blood flow is required for survival. If untreated, PA/IVS is almost always fatal. Outcomes of surgical interventions are improving, with a five-year survival rate of approximately 80 percent. […] The definition, anatomy, physiology, clinical presentation, management (including follow-up care), and outcome of PA/IVS will be reviewed here. […] PA/IVS is a rare congenital cardiac defect that consists of atresia of the pulmonary valve resulting in an absent connection between the right ventricular outflow tract (RVOT) and pulmonary arteries as well as an IVS that allows no connection between the right and left ventricles.
  • #35 Pulmonary atresia with intact ventricular septum (PA/IVS) – UpToDate
    https://www.uptodate.com/contents/pulmonary-atresia-with-intact-ventricular-septum-pa-ivs
    PA/IVS is distinctive from PA with a ventricular septal defect (PA/VSD; often called tetralogy of Fallot [TOF]/PA) and from severe forms of Ebstein anomaly of the tricuspid valve (TV). Although all of these defects can result in complete obstruction of the RVOT, they have different morphologic anatomic features (eg, RV size and function, presence of pulmonary and/or TV abnormalities, and status of the ventricular septum), which significantly impact management decisions. […] PA/IVS features developmental abnormalities of the RV and TV that are „upstream” of pulmonary outflow. The pulmonary arteries may be small, but their architecture and branching patterns are usually otherwise normal.
  • #36 Pulmonary atresia with ventricular septal defect – Wikipedia
    https://en.wikipedia.org/wiki/Pulmonary_atresia_with_ventricular_septal_defect
    There are various ways of diagnosing this congenital heart defect both prenatally and postnatally, these methods include: Ultrasound, Pulse oximetry, Chest X-ray, Echocardiogram, Electrocardiogram, Cardiac catheterization, Cardiac CT scan, Genetic testing (particularly if other systemic birth anomalies are seen alongside the pulmonary atresia and ventricular septal defect). […] Diagnostic method Radiological studies such as chest CT scans.
  • #37 Pulmonary Atresia | Nationwide Children’s Hospital
    https://www.nationwidechildrens.org/conditions/pulmonary-atresia
    Cardiac MRI: A test that uses radio waves, magnets, and a computer to make detailed pictures of the heart and blood vessels. […] Some congenital conditions are caused by a baby’s DNA. […] The doctors may suggest genetic testing to find out more about this. […] Your baby may need help with breathing, extra oxygen, or may need to be started on a medicine called prostaglandin E (PGE, or „prostins”). […] If your baby’s condition is severe, they will need to be transferred to Nationwide Children’s Hospital Cardiothoracic Intensive Care Unit (CTICU) shortly after birth by the Nationwide Children’s Mobile Intensive Care team. […] After surgery, your baby will go to the Cardiothoracic Intensive Care Unit (CTICU). […] Your hospital stay after birth may be a couple of days or as long as weeks to months. […] Your cardiologist will follow up with your baby’s heart needs even after they are discharged from the hospital.
  • #38 Prenatal Diagnosis of Pulmonary Atresia With Ventricular Septal Defect and an Aberrant Ductus Arteriosus in a Dextrocardia by Two- and Three-Dimensional Echocardiography: A Case Report
    https://pmc.ncbi.nlm.nih.gov/articles/PMC9283767/
    It should be mentioned that the coronal plane sectioned through bilateral hilum is useful to find any systemic-to-pulmonary collateral arteries, when present. […] The steps of the diagnosis were summarized in a flowchart. […] The novel 3D technology provides new insights and has the potential to supplement traditional 2D echocardiography by yielding realistic-like images, which may contribute to prenatal counseling between obstetricians and parents. […] Therefore, we strongly recommend that chromosomal tests should be performed for these fetuses. […] The diagnostic approaches and differential diagnosis strategies of PA-VSD are discussed.
  • #39 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.
  • #40 Pulmonary atresia | Children’s Wisconsin
    https://childrenswi.org/medical-care/herma-heart/conditions/pulmonary-atresia
    How is pulmonary atresia diagnosed? […] 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 your newborn. Your child’s physician may have also heard a heart murmur during a physical examination. A heart murmur is simply a noise caused by the turbulence of blood flowing through the openings that allow the blood to mix. […] Other diagnostic tests are needed to help with the diagnosis, and may include the following: […] Chest x-ray – a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film.
  • #41 Pulmonary Atresia | Texas Children’s
    https://www.texaschildrens.org/content/conditions/pulmonary-atresia
    You will undergo additional testing to help us gain more information about the structure and function of your baby’s heart. […] Following a thorough evaluation and assessment, our specialists will meet with you about the results, provide treatment recommendations, and answer any questions your family has, to help you make the most informed decisions regarding your baby’s care and treatment.
  • #42 Pulmonary Atresia: Symptoms, Causes and Treatment
    https://my.clevelandclinic.org/health/diseases/14779-pulmonary-atresia
    The size of their right ventricle and pulmonary artery. […] The ability of their right ventricle to pump blood. […] 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 child will have regular follow-up appointments with their pediatric cardiologist. […] People with pulmonary atresia must remain under the care of a cardiologist for their entire lives. […] Regular follow-up care can reduce or prevent these complications.
  • #43 Prenatal Diagnosis of Pulmonary Atresia with Intact Ventricular Septum
    https://www.contemporaryobgyn.net/view/prenatal-diagnosis-pulmonary-atresia-intact-ventricular-septum
    Hypoplasia of the right ventricle is uncommon as an isolated entity. It may result from tricuspid atresia, but is most commonly secondary to pulmonary atresia with intact ventricular septum. […] Pulmonary atresia with intact ventricular septum is rare, accounting for 1-3% of cases of congenital heart disease. It occurs in 0.1 to 0.4 in 10,000 live births. Pulmonary atresia with intact ventricular septum has been reported to occur slightly more frequently in males. […] Fetal echocardiogram revealed a four-chamber heart correctly oriented in the left chest. The right ventricle appeared small and severely hypertrophied. A small pulmonary artery was visualized with an atretic pulmonic valve. Pulsed Doppler distal to the pulmonic valve confirmed absence of flow. […] Because a normal caliber main pulmonary artery does not exclude the diagnosis of pulmonary atresia, demonstrating reversal of blood flow through the ductus is essential. Retrograde blood flow through the ductus is virtually always present in pulmonary atresia with intact ventricular septum. […] The outcome for infants born with pulmonary atresia and intact ventricular septum is poor. They are dependent on a patent ductus arteriosus for survival in the neonatal period. Administration of prostaglandin E1 at birth is essential to maintain patency until surgery can be performed.
  • #44 Pulmonary Atresia: Symptoms, Causes and Treatment
    https://my.clevelandclinic.org/health/diseases/14779-pulmonary-atresia
    The size of their right ventricle and pulmonary artery. […] The ability of their right ventricle to pump blood. […] 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 child will have regular follow-up appointments with their pediatric cardiologist. […] People with pulmonary atresia must remain under the care of a cardiologist for their entire lives. […] Regular follow-up care can reduce or prevent these complications.
  • #45 Pulmonary Atresia | Valley Children’s Healthcare
    https://www.valleychildrens.org/heart/conditions/pulmonary-atresia
    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. […] PA may be found during a routine ultrasound in pregnancy. […] At birth, the healthcare providers and nurses will examine your baby. They will listen to your baby’s heart and lungs with a stethoscope and note any signs of a heart defect. […] Testing for heart defects varies by the child’s age, condition, and other things. Some tests that may be done include: […] Echocardiogram (echo). Echo uses sound waves to record a moving picture of the heart and heart valves. This test is the best test to diagnose PA. […] Without surgery, the heart cant pump oxygenated blood to the body and cant support life. Surgery can be very successful in those with normal sized tricuspid valves and right ventricles. […] Your child will need regular follow-up care at a center offering pediatric or adult congenital heart care for the rest of their life.
  • #46 Pulmonary Atresia: Symptoms, Causes and Treatment
    https://my.clevelandclinic.org/health/diseases/14779-pulmonary-atresia
    The size of their right ventricle and pulmonary artery. […] The ability of their right ventricle to pump blood. […] 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 child will have regular follow-up appointments with their pediatric cardiologist. […] People with pulmonary atresia must remain under the care of a cardiologist for their entire lives. […] Regular follow-up care can reduce or prevent these complications.
  • #47 Prenatal Diagnosis of Pulmonary Atresia With Ventricular Septal Defect and an Aberrant Ductus Arteriosus in a Dextrocardia by Two- and Three-Dimensional Echocardiography: A Case Report
    https://pmc.ncbi.nlm.nih.gov/articles/PMC9283767/
    Prenatal diagnosis of pulmonary atresia is difficult in relative, especially when the pulmonary artery is slim and hypoplastic in development. […] It is difficult to make an accurate diagnosis of pulmonary atresia prenatally, especially when the pulmonary artery is slim and hypoplastic. […] A detailed evaluation of fetal cardiac anatomy and hemodynamics is crucial for the detection of an aberrant ductus arteriosus, which plays an important role in the diagnosis of pulmonary atresia with ventricular septal defect. […] The strategy for the diagnosis of PA-VSD together with the experience for tracing the aberrant DA was summarized in this report. […] The three-dimensional echocardiography with spatio-temporal image correlation technique could provide additional spatial information to show great arteries in detail, which can serve as a supplement to traditional two-dimensional modality and benefit examiners to make an accurate diagnosis.
  • #48 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 can be suspected prenatally on a second trimester obstetric an 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. […] 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.
  • #49 Prenatal Diagnosis of Pulmonary Atresia With Ventricular Septal Defect and an Aberrant Ductus Arteriosus in a Dextrocardia by Two- and Three-Dimensional Echocardiography: A Case Report
    https://pmc.ncbi.nlm.nih.gov/articles/PMC9283767/
    Prenatal diagnosis of pulmonary atresia is difficult in relative, especially when the pulmonary artery is slim and hypoplastic in development. […] It is difficult to make an accurate diagnosis of pulmonary atresia prenatally, especially when the pulmonary artery is slim and hypoplastic. […] A detailed evaluation of fetal cardiac anatomy and hemodynamics is crucial for the detection of an aberrant ductus arteriosus, which plays an important role in the diagnosis of pulmonary atresia with ventricular septal defect. […] The strategy for the diagnosis of PA-VSD together with the experience for tracing the aberrant DA was summarized in this report. […] The three-dimensional echocardiography with spatio-temporal image correlation technique could provide additional spatial information to show great arteries in detail, which can serve as a supplement to traditional two-dimensional modality and benefit examiners to make an accurate diagnosis.
  • #50 Prenatal diagnosis of pulmonary atresia with intact ventricular septum: a single-center study in China | Cardiovascular Ultrasound | Full Text
    https://cardiovascularultrasound.biomedcentral.com/articles/10.1186/s12947-025-00348-0
    To evaluate the efficacy of prenatal ultrasound in diagnosing pulmonary atresia with intact ventricular septum (PA/IVS). […] This study aims to enhance the understanding of PA/IVS and improve the accuracy of its prenatal diagnosis. […] Fetal echocardiography is an effective tool for diagnosing PA/IVS. While PA/IVS can be diagnosed in early gestation, it remains diagnostical challenging. Given the progressive nature of PA/IVS in utero, sequential ultrasound examinations during the second and third trimesters are essential for monitoring disease progression and hemodynamic changes. Additionally, a comprehensive evaluation for associated intracardiac and extracardiac anomalies should be systematically conducted throughout the pregnancy. […] Accurate prenatal ultrasound diagnosis and risk stratification are therefore critical for effective prenatal counseling, perinatal management, and postnatal treatment planning.
  • #51 Prenatal diagnosis of pulmonary atresia with intact ventricular septum: a single-center study in China | Cardiovascular Ultrasound | Full Text
    https://cardiovascularultrasound.biomedcentral.com/articles/10.1186/s12947-025-00348-0
    Therefore, it remains crucial to investigate how prenatal ultrasound can accurately diagnose PA/IVS throughout pregnancy and enhance the integrated prenatal-to-postnatal management of affected fetuses. […] We analyzed the prenatal ultrasound features of PA/IVS, classified the cases into subtypes, and tracked their pregnancy outcomes. These outcomes were compared with post-labor induction pathological findings or postnatal imaging results to deepen the understanding of PA/IVS and improve the accuracy of prenatal ultrasound diagnosis. […] The early pregnancy detection rate of 8.3% in our cohort aligns with emerging evidence supporting first-trimester cardiac screening. […] The association of NT thickening and abnormal DV flow with subsequent PA/IVS diagnosis suggests these markers warrant thorough fetal echocardiography.
  • #52 Prenatal Diagnosis of Pulmonary Atresia With Ventricular Septal Defect and an Aberrant Ductus Arteriosus in a Dextrocardia by Two- and Three-Dimensional Echocardiography: A Case Report
    https://pmc.ncbi.nlm.nih.gov/articles/PMC9283767/
    It should be mentioned that the coronal plane sectioned through bilateral hilum is useful to find any systemic-to-pulmonary collateral arteries, when present. […] The steps of the diagnosis were summarized in a flowchart. […] The novel 3D technology provides new insights and has the potential to supplement traditional 2D echocardiography by yielding realistic-like images, which may contribute to prenatal counseling between obstetricians and parents. […] Therefore, we strongly recommend that chromosomal tests should be performed for these fetuses. […] The diagnostic approaches and differential diagnosis strategies of PA-VSD are discussed.
  • #53 What is Pulmonary Atresia? – Healthis Assistance
    https://healthis.com.tr/en/what-is-pulmonary-atresia/
    Pulmonary atresia is often diagnosed before birth during routine ultrasound scans. A specialized ultrasound method called fetal echocardiography is used to examine the structural abnormalities of the heart. This technique provides detailed images of the baby’s heart function and blood flow. Prenatal diagnosis helps medical teams quickly plan post-birth interventions, increasing the baby’s chances of survival. […] Several methods are used to diagnose pulmonary atresia. The first step is usually an echocardiogram, which provides a detailed image of the heart and allows for the evaluation of blood flow and valve function. In more advanced cases, additional imaging techniques may be required. […] Echocardiography uses ultrasound waves to create images of the heart. In pulmonary atresia, it helps visualize the heart valves, chambers, and the impact on blood flow. This method is essential for diagnosing the condition and determining whether surgical intervention is needed. […] To get a more detailed view of pulmonary atresia, doctors may use magnetic resonance imaging (MRI) or computed tomography (CT). These techniques offer three-dimensional images of the heart and blood vessels, providing critical information for surgical planning.
  • #54 Prenatal Diagnosis of Pulmonary Atresia With Ventricular Septal Defect and an Aberrant Ductus Arteriosus in a Dextrocardia by Two- and Three-Dimensional Echocardiography: A Case Report
    https://pmc.ncbi.nlm.nih.gov/articles/PMC9283767/
    It should be mentioned that the coronal plane sectioned through bilateral hilum is useful to find any systemic-to-pulmonary collateral arteries, when present. […] The steps of the diagnosis were summarized in a flowchart. […] The novel 3D technology provides new insights and has the potential to supplement traditional 2D echocardiography by yielding realistic-like images, which may contribute to prenatal counseling between obstetricians and parents. […] Therefore, we strongly recommend that chromosomal tests should be performed for these fetuses. […] The diagnostic approaches and differential diagnosis strategies of PA-VSD are discussed.
  • #55 Prenatal diagnosis of pulmonary atresia with intact ventricular septum: a single-center study in China | Cardiovascular Ultrasound | Full Text
    https://cardiovascularultrasound.biomedcentral.com/articles/10.1186/s12947-025-00348-0
    Therefore, it remains crucial to investigate how prenatal ultrasound can accurately diagnose PA/IVS throughout pregnancy and enhance the integrated prenatal-to-postnatal management of affected fetuses. […] We analyzed the prenatal ultrasound features of PA/IVS, classified the cases into subtypes, and tracked their pregnancy outcomes. These outcomes were compared with post-labor induction pathological findings or postnatal imaging results to deepen the understanding of PA/IVS and improve the accuracy of prenatal ultrasound diagnosis. […] The early pregnancy detection rate of 8.3% in our cohort aligns with emerging evidence supporting first-trimester cardiac screening. […] The association of NT thickening and abnormal DV flow with subsequent PA/IVS diagnosis suggests these markers warrant thorough fetal echocardiography.
  • #56 Prenatal diagnosis of pulmonary atresia with intact ventricular septum: a single-center study in China | Cardiovascular Ultrasound | Full Text
    https://cardiovascularultrasound.biomedcentral.com/articles/10.1186/s12947-025-00348-0
    To evaluate the efficacy of prenatal ultrasound in diagnosing pulmonary atresia with intact ventricular septum (PA/IVS). […] This study aims to enhance the understanding of PA/IVS and improve the accuracy of its prenatal diagnosis. […] Fetal echocardiography is an effective tool for diagnosing PA/IVS. While PA/IVS can be diagnosed in early gestation, it remains diagnostical challenging. Given the progressive nature of PA/IVS in utero, sequential ultrasound examinations during the second and third trimesters are essential for monitoring disease progression and hemodynamic changes. Additionally, a comprehensive evaluation for associated intracardiac and extracardiac anomalies should be systematically conducted throughout the pregnancy. […] Accurate prenatal ultrasound diagnosis and risk stratification are therefore critical for effective prenatal counseling, perinatal management, and postnatal treatment planning.
  • #57 Prenatal diagnosis of pulmonary atresia with intact ventricular septum: a single-center study in China | Cardiovascular Ultrasound | Full Text
    https://cardiovascularultrasound.biomedcentral.com/articles/10.1186/s12947-025-00348-0
    To evaluate the efficacy of prenatal ultrasound in diagnosing pulmonary atresia with intact ventricular septum (PA/IVS). […] This study aims to enhance the understanding of PA/IVS and improve the accuracy of its prenatal diagnosis. […] Fetal echocardiography is an effective tool for diagnosing PA/IVS. While PA/IVS can be diagnosed in early gestation, it remains diagnostical challenging. Given the progressive nature of PA/IVS in utero, sequential ultrasound examinations during the second and third trimesters are essential for monitoring disease progression and hemodynamic changes. Additionally, a comprehensive evaluation for associated intracardiac and extracardiac anomalies should be systematically conducted throughout the pregnancy. […] Accurate prenatal ultrasound diagnosis and risk stratification are therefore critical for effective prenatal counseling, perinatal management, and postnatal treatment planning.
  • #58 Pulmonary atresia: Types, causes, symptoms, and more
    https://www.medicalnewstoday.com/articles/pulmonary-atresia
    A baby with pulmonary atresia may need surgery soon after birth. […] The condition is usually noticeable at birth or soon after, and in some cases a doctor may be able to diagnose it during pregnancy. […] Signs and symptoms of pulmonary atresia can include cyanosis, heart murmur, breathing difficulties, feeding difficulties, tiredness, and failure to thrive. […] A baby with pulmonary atresia may need additional surgeries or treatments as they get older.