Atrezja płucna z zachowaną przegrodą międzykomorową
Rokowania, prognozy i postęp choroby

Atrezja płucna z zachowaną przegrodą międzykomorową (PAIVS) to rzadka wada serca o częstości około 45/100 000 żywych urodzeń, charakteryzująca się hipoplazją prawego serca i znaczną heterogennością morfologiczną. Przeżycie jednoroczne i pięcioletnie wynosi odpowiednio 70,8% i 63,8%, a w nowszych kohortach wskaźnik przeżycia według zamiaru leczenia osiąga 83,0% przy medianie obserwacji 22,5 miesiąca. Niezależne czynniki ryzyka zgonu to niska masa urodzeniowa (p = 0,024), jednoczęściowa morfologia prawej komory (p = 0,001) oraz obecność poszerzonej prawej komory (p < 0,001). Kluczowym prognostycznie aspektem jest możliwość osiągnięcia krążenia dwukomorowego (BV), co ma istotne znaczenie w poradnictwie prenatalnym i planowaniu leczenia chirurgicznego po urodzeniu.

  1. Prognoza w atrezji płucnej z zachowaną przegrodą międzykomorową
  2. Wskaźniki przeżycia
  3. Niezależne czynniki ryzyka zgonu
  4. Predyktory wyniku dwukomorowego
    1. Parametry echokardiograficzne w prognozowaniu
    2. Systemy predykcyjne wyniku jednokomorowego
  5. Kluczowe znaczenie wielkości prawej komory
  6. Znaczenie diagnostyki prenatalnej
  7. Kierunki przyszłych badań
  8. Wnioski
    1. Kolejne rozdziały

Prognoza w atrezji płucnej z zachowaną przegrodą międzykomorową

Atrezja płucna z zachowaną przegrodą międzykomorową (PAIVS) to rzadka, heterogenna anomalia serca występująca z częstością około 45/100 000 żywych urodzeń. Jest to forma wrodzonej wady serca zazwyczaj związana z hipoplazją struktur prawego serca, charakteryzująca się znaczną heterogennością morfologiczną i często niekorzystnym rokowaniem.12 Określenie czynników ryzyka niekorzystnego wyniku jest istotnym krokiem, jeśli ma nastąpić poprawa rokowania. Prawidłowa ocena prognostyczna ma kluczowe znaczenie w poradnictwie prenatalnym i planowaniu strategii chirurgicznej po urodzeniu.3

Wskaźniki przeżycia

Wyniki leczenia atrezji płucnej z zachowaną przegrodą międzykomorową uległy poprawie na przestrzeni lat, jednak nadal pozostają niepewne. Jednoroczne i pięcioletnie przeżycie wynosi odpowiednio 70,8% i 63,8% według badań populacyjnych.4 Inne badania raportują wskaźniki przeżycia jednego, pięciu i dziesięciu lat w zakresie od 63% do 97%, w zależności od typu krążenia postnatalnego.5 W nowszych kohortach wskaźnik przeżycia według zamiaru leczenia (intention-to-treat) przy medianie obserwacji 22,5 miesiąca wynosił 83,0% (44/53 pacjentów).6

Niezależne czynniki ryzyka zgonu

Na podstawie analizy modelu proporcjonalnego hazardu Coxa, zidentyfikowano kilka niezależnych czynników ryzyka zgonu u pacjentów z PAIVS:7

  • Niska masa urodzeniowa (p = 0,024)
  • Jednoczęściowa morfologia prawej komory (p = 0,001)
  • Obecność poszerzonej prawej komory (p < 0,001)

8

Predyktory wyniku dwukomorowego

Kluczowym elementem prognostycznym w PAIVS jest możliwość osiągnięcia krążenia dwukomorowego (biventricular, BV). Przewidywanie wyniku krążenia stanowi istotny aspekt poradnictwa dla rodziców, zwłaszcza że odsetek terminacji ciąży w przypadkach PAIVS/CPS (krytycznego zwężenia zastawki płucnej) sięga nawet 60%, szczególnie gdy podejrzewany jest wynik jednokomorowy (univentricular, UV).9

Parametry echokardiograficzne w prognozowaniu

W badaniach prenatalnych, najlepszym pojedynczym parametrem echokardiograficznym do przewidywania wyniku dwukomorowego okazał się zastawki trójdzielnej do zastawki mitralnej” class=”to-tag” data-termid=”120212″>stosunek zastawki trójdzielnej do zastawki mitralnej (TV/MV ratio) w 24. tygodniu ciąży.1011 Wartość TV/MV ratio jest prostym parametrem o wysokiej wartości predykcyjnej, wykazującym pole pod krzywą ROC (AUC) wynoszące 0,93 w przedziałach czasowych 2 i 3 prenatalnego rozwoju (AUC 0,92).12

Systemy predykcyjne wyniku jednokomorowego

Opracowano również bardziej złożone systemy predykcyjne. W jednym z badań stworzono skalę łączącą stosunek TV/MV z obecnością połączeń wieńcowo-komorowych (VCAC) oraz maksymalną prędkością niedomykalności trójdzielnej (TI-Vmax) ≥2,5 m/s. Wynik jednokomorowy (non-BV) był prawidłowo przewidywany we wszystkich przypadkach, gdy spełnione było więcej niż jedno kryterium.1314 Odsetek wyników fałszywie dodatnich dla tej skali wynosi około 17%, co jest zbliżone do wcześniejszych danych i powinno być brane pod uwagę w poradnictwie dla rodziców.15

Kluczowe znaczenie wielkości prawej komory

Podstawą postępowania i ostatecznego wyniku w PAIVS jest ocena wielkości prawej komory (RV).16 Rozmiar zastawki trójdzielnej jest ważnym wskaźnikiem predykcyjnym dla rozwoju prawej komory w kontekście PAIVS.17 Pacjenci prezentują się jako noworodki z sinicą, a ich przeżycie jest zależne od drożności przewodu tętniczego.18

Znaczenie diagnostyki prenatalnej

Podstawowym narzędziem diagnostycznym w PAIVS jest echokardiografia, a inne metody obrazowania zwykle nie są wymagane.19 Dokładna ocena prenatalna pozwala na wczesne poradnictwo dla rodziców i planowanie strategii leczenia. Umożliwia również identyfikację pacjentów z grupy wysokiego ryzyka, co ma istotne znaczenie dla poradnictwa w życiu płodowym i strategii chirurgicznej po urodzeniu.20

Kierunki przyszłych badań

Kryteria interwencji płodowej w przypadkach PAIVS/CPS powinny być dalej oceniane w sposób prospektywny.21 Nadal istnieje potrzeba prowadzenia badań w większych kohortach prenatalnych z PAIVS/CPS, w celu dalszej walidacji parametrów prognostycznych i poprawy strategii leczenia.22

Wnioski

Atrezja płucna z zachowaną przegrodą międzykomorową pozostaje wyzwaniem klinicznym z umiarkowanymi wskaźnikami przeżycia. Zidentyfikowane czynniki ryzyka, takie jak niska masa urodzeniowa, jednoczęściowa morfologia prawej komory i obecność poszerzonej prawej komory, pomagają w stratyfikacji ryzyka pacjentów. Stosunek TV/MV w 24. tygodniu ciąży jest najlepszym pojedynczym parametrem do przewidywania wyniku dwukomorowego, co ma kluczowe znaczenie w poradnictwie prenatalnym. Połączenie kilku parametrów diagnostycznych zwiększa dokładność przewidywania wyniku jednokomorowego, choć należy uwzględnić ryzyko wyników fałszywie dodatnich.2324

Kolejne rozdziały

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  1. 13.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Natural history of pulmonary atresia with intact ventricular septum (PAIVS) and critical pulmonary stenosis (CPS) and prediction of outcome | springermedizin.de
    https://www.springermedizin.de/natural-history-of-pulmonary-atresia-with-intact-ventricular-sep/18862602
    Pulmonary atresia with intact ventricular septum (PAIVS) is a rare, heterogenous cardiac anomaly with an incidence of 45/100,000 live births. Outcomes have improved over time, but are still guarded with reported one- and 5-year survival rates of 70-75% and 63-67%, depending on the type of postnatal circulation. The rate of termination of pregnancy (TOP) in PAIVS/CPS is up to 60%, especially when univentricular (UV) outcome is supposed. Therefore, prediction of circulation outcome is an important aspect for parental counselling. […] The best single parameter for BV outcome was tricuspid/mitral valve (TV/MV) ratio (AUC 0.93) in intervals 2 and 3 (AUC 0.92). […] TV/MV ratio as simple parameter has high predictive value. After our score, non-BV outcome was correctly predicted in all cases.
  • #2 Pulmonary atresia with intact ventricular septum: predictors of early and medium-term outcome in a population-based study – PubMed
    https://pubmed.ncbi.nlm.nih.gov/16214522/
    Pulmonary atresia with intact ventricular septum is a form of congenital heart disease usually associated with right-heart hypoplasia, with considerable morphologic heterogeneity and often poor outlook. […] Ascertainment of risk factors for poor outcome is an important step if an improvement in outcome is to be achieved. […] One- and 5-year survival was 70.8% and 63.8%, respectively. […] Results from Cox proportional hazards model analysis showed that low birth weight (P = .024), unipartite right ventricular morphology (P = .001), and the presence of a dilated right ventricle (P .001) were independent risk factors for death. […] This population-based study has shown which features at presentation place an infant in a high-risk group. This is important information for counseling in fetal life and for surgical strategy after birth.
  • #3 Pulmonary atresia with intact ventricular septum: predictors of early and medium-term outcome in a population-based study – PubMed
    https://pubmed.ncbi.nlm.nih.gov/16214522/
    Pulmonary atresia with intact ventricular septum is a form of congenital heart disease usually associated with right-heart hypoplasia, with considerable morphologic heterogeneity and often poor outlook. […] Ascertainment of risk factors for poor outcome is an important step if an improvement in outcome is to be achieved. […] One- and 5-year survival was 70.8% and 63.8%, respectively. […] Results from Cox proportional hazards model analysis showed that low birth weight (P = .024), unipartite right ventricular morphology (P = .001), and the presence of a dilated right ventricle (P .001) were independent risk factors for death. […] This population-based study has shown which features at presentation place an infant in a high-risk group. This is important information for counseling in fetal life and for surgical strategy after birth.
  • #4 Pulmonary atresia with intact ventricular septum: predictors of early and medium-term outcome in a population-based study – PubMed
    https://pubmed.ncbi.nlm.nih.gov/16214522/
    Pulmonary atresia with intact ventricular septum is a form of congenital heart disease usually associated with right-heart hypoplasia, with considerable morphologic heterogeneity and often poor outlook. […] Ascertainment of risk factors for poor outcome is an important step if an improvement in outcome is to be achieved. […] One- and 5-year survival was 70.8% and 63.8%, respectively. […] Results from Cox proportional hazards model analysis showed that low birth weight (P = .024), unipartite right ventricular morphology (P = .001), and the presence of a dilated right ventricle (P .001) were independent risk factors for death. […] This population-based study has shown which features at presentation place an infant in a high-risk group. This is important information for counseling in fetal life and for surgical strategy after birth.
  • #5
    https://link.springer.com/article/10.1007/s00404-020-05929-0
    To analyse prenatal parameters predicting biventricular (BV) outcome in pulmonary atresia with intact ventricular septum/critical pulmonary stenosis (PAIVS/CPS). […] Outcomes have improved over time, but are still guarded with reported one- and 5-year survival rates of 70-75% and 63-67%, depending on the type of postnatal circulation. […] Therefore, prediction of circulation outcome is an important aspect for parental counselling. […] The aim of our study was to describe outcomes in prenatally diagnosed PAIVS/CPS, to analyse foetal echocardiographic parameters for predicting BV outcome and growth rate of these during gestation in the largest cohort to date. […] Intention-to-treat survival rate (median follow-up 22.5 months) was 44/53 (83.0%) which fits to reported one-, 5- and 10-year survival rates ranging from 63 to 97%. […] In our cohort, the best single, echocardiographic parameter to predict BV outcome was TV/MV ratio 24 weeks. […] Non-BV outcome was correctly predicted in all our cases if more than one criteria were fulfilled. […] TV/MV ratio is a simple single parameter with high predictive value.
  • #6
    https://link.springer.com/article/10.1007/s00404-020-05929-0
    To analyse prenatal parameters predicting biventricular (BV) outcome in pulmonary atresia with intact ventricular septum/critical pulmonary stenosis (PAIVS/CPS). […] Outcomes have improved over time, but are still guarded with reported one- and 5-year survival rates of 70-75% and 63-67%, depending on the type of postnatal circulation. […] Therefore, prediction of circulation outcome is an important aspect for parental counselling. […] The aim of our study was to describe outcomes in prenatally diagnosed PAIVS/CPS, to analyse foetal echocardiographic parameters for predicting BV outcome and growth rate of these during gestation in the largest cohort to date. […] Intention-to-treat survival rate (median follow-up 22.5 months) was 44/53 (83.0%) which fits to reported one-, 5- and 10-year survival rates ranging from 63 to 97%. […] In our cohort, the best single, echocardiographic parameter to predict BV outcome was TV/MV ratio 24 weeks. […] Non-BV outcome was correctly predicted in all our cases if more than one criteria were fulfilled. […] TV/MV ratio is a simple single parameter with high predictive value.
  • #7 Pulmonary atresia with intact ventricular septum: predictors of early and medium-term outcome in a population-based study – PubMed
    https://pubmed.ncbi.nlm.nih.gov/16214522/
    Pulmonary atresia with intact ventricular septum is a form of congenital heart disease usually associated with right-heart hypoplasia, with considerable morphologic heterogeneity and often poor outlook. […] Ascertainment of risk factors for poor outcome is an important step if an improvement in outcome is to be achieved. […] One- and 5-year survival was 70.8% and 63.8%, respectively. […] Results from Cox proportional hazards model analysis showed that low birth weight (P = .024), unipartite right ventricular morphology (P = .001), and the presence of a dilated right ventricle (P .001) were independent risk factors for death. […] This population-based study has shown which features at presentation place an infant in a high-risk group. This is important information for counseling in fetal life and for surgical strategy after birth.
  • #8 Pulmonary atresia with intact ventricular septum: predictors of early and medium-term outcome in a population-based study – PubMed
    https://pubmed.ncbi.nlm.nih.gov/16214522/
    Pulmonary atresia with intact ventricular septum is a form of congenital heart disease usually associated with right-heart hypoplasia, with considerable morphologic heterogeneity and often poor outlook. […] Ascertainment of risk factors for poor outcome is an important step if an improvement in outcome is to be achieved. […] One- and 5-year survival was 70.8% and 63.8%, respectively. […] Results from Cox proportional hazards model analysis showed that low birth weight (P = .024), unipartite right ventricular morphology (P = .001), and the presence of a dilated right ventricle (P .001) were independent risk factors for death. […] This population-based study has shown which features at presentation place an infant in a high-risk group. This is important information for counseling in fetal life and for surgical strategy after birth.
  • #9 Natural history of pulmonary atresia with intact ventricular septum (PAIVS) and critical pulmonary stenosis (CPS) and prediction of outcome | springermedizin.de
    https://www.springermedizin.de/natural-history-of-pulmonary-atresia-with-intact-ventricular-sep/18862602
    Pulmonary atresia with intact ventricular septum (PAIVS) is a rare, heterogenous cardiac anomaly with an incidence of 45/100,000 live births. Outcomes have improved over time, but are still guarded with reported one- and 5-year survival rates of 70-75% and 63-67%, depending on the type of postnatal circulation. The rate of termination of pregnancy (TOP) in PAIVS/CPS is up to 60%, especially when univentricular (UV) outcome is supposed. Therefore, prediction of circulation outcome is an important aspect for parental counselling. […] The best single parameter for BV outcome was tricuspid/mitral valve (TV/MV) ratio (AUC 0.93) in intervals 2 and 3 (AUC 0.92). […] TV/MV ratio as simple parameter has high predictive value. After our score, non-BV outcome was correctly predicted in all cases.
  • #10 Natural history of pulmonary atresia with intact ventricular septum (PAIVS) and critical pulmonary stenosis (CPS) and prediction of outcome | springermedizin.de
    https://www.springermedizin.de/natural-history-of-pulmonary-atresia-with-intact-ventricular-sep/18862602
    In our cohort, the best single, echocardiographic parameter to predict BV outcome was TV/MV ratio at 24 weeks. We included TV/MV ratio as best single parameter in combination with presence of VCAC and TI-Vmax2.5 m/s to a score. Non-BV outcome was correctly predicted in all our cases if more than one criteria were fulfilled. False-positive rate of the score is 17%, respectively, which is similar to previous data and should be considered for parental counselling.
  • #11
    https://link.springer.com/article/10.1007/s00404-020-05929-0
    To analyse prenatal parameters predicting biventricular (BV) outcome in pulmonary atresia with intact ventricular septum/critical pulmonary stenosis (PAIVS/CPS). […] Outcomes have improved over time, but are still guarded with reported one- and 5-year survival rates of 70-75% and 63-67%, depending on the type of postnatal circulation. […] Therefore, prediction of circulation outcome is an important aspect for parental counselling. […] The aim of our study was to describe outcomes in prenatally diagnosed PAIVS/CPS, to analyse foetal echocardiographic parameters for predicting BV outcome and growth rate of these during gestation in the largest cohort to date. […] Intention-to-treat survival rate (median follow-up 22.5 months) was 44/53 (83.0%) which fits to reported one-, 5- and 10-year survival rates ranging from 63 to 97%. […] In our cohort, the best single, echocardiographic parameter to predict BV outcome was TV/MV ratio 24 weeks. […] Non-BV outcome was correctly predicted in all our cases if more than one criteria were fulfilled. […] TV/MV ratio is a simple single parameter with high predictive value.
  • #12 Natural history of pulmonary atresia with intact ventricular septum (PAIVS) and critical pulmonary stenosis (CPS) and prediction of outcome | springermedizin.de
    https://www.springermedizin.de/natural-history-of-pulmonary-atresia-with-intact-ventricular-sep/18862602
    Pulmonary atresia with intact ventricular septum (PAIVS) is a rare, heterogenous cardiac anomaly with an incidence of 45/100,000 live births. Outcomes have improved over time, but are still guarded with reported one- and 5-year survival rates of 70-75% and 63-67%, depending on the type of postnatal circulation. The rate of termination of pregnancy (TOP) in PAIVS/CPS is up to 60%, especially when univentricular (UV) outcome is supposed. Therefore, prediction of circulation outcome is an important aspect for parental counselling. […] The best single parameter for BV outcome was tricuspid/mitral valve (TV/MV) ratio (AUC 0.93) in intervals 2 and 3 (AUC 0.92). […] TV/MV ratio as simple parameter has high predictive value. After our score, non-BV outcome was correctly predicted in all cases.
  • #13 Natural history of pulmonary atresia with intact ventricular septum (PAIVS) and critical pulmonary stenosis (CPS) and prediction of outcome | springermedizin.de
    https://www.springermedizin.de/natural-history-of-pulmonary-atresia-with-intact-ventricular-sep/18862602
    In our cohort, the best single, echocardiographic parameter to predict BV outcome was TV/MV ratio at 24 weeks. We included TV/MV ratio as best single parameter in combination with presence of VCAC and TI-Vmax2.5 m/s to a score. Non-BV outcome was correctly predicted in all our cases if more than one criteria were fulfilled. False-positive rate of the score is 17%, respectively, which is similar to previous data and should be considered for parental counselling.
  • #14
    https://link.springer.com/article/10.1007/s00404-020-05929-0
    To analyse prenatal parameters predicting biventricular (BV) outcome in pulmonary atresia with intact ventricular septum/critical pulmonary stenosis (PAIVS/CPS). […] Outcomes have improved over time, but are still guarded with reported one- and 5-year survival rates of 70-75% and 63-67%, depending on the type of postnatal circulation. […] Therefore, prediction of circulation outcome is an important aspect for parental counselling. […] The aim of our study was to describe outcomes in prenatally diagnosed PAIVS/CPS, to analyse foetal echocardiographic parameters for predicting BV outcome and growth rate of these during gestation in the largest cohort to date. […] Intention-to-treat survival rate (median follow-up 22.5 months) was 44/53 (83.0%) which fits to reported one-, 5- and 10-year survival rates ranging from 63 to 97%. […] In our cohort, the best single, echocardiographic parameter to predict BV outcome was TV/MV ratio 24 weeks. […] Non-BV outcome was correctly predicted in all our cases if more than one criteria were fulfilled. […] TV/MV ratio is a simple single parameter with high predictive value.
  • #15 Natural history of pulmonary atresia with intact ventricular septum (PAIVS) and critical pulmonary stenosis (CPS) and prediction of outcome | springermedizin.de
    https://www.springermedizin.de/natural-history-of-pulmonary-atresia-with-intact-ventricular-sep/18862602
    In our cohort, the best single, echocardiographic parameter to predict BV outcome was TV/MV ratio at 24 weeks. We included TV/MV ratio as best single parameter in combination with presence of VCAC and TI-Vmax2.5 m/s to a score. Non-BV outcome was correctly predicted in all our cases if more than one criteria were fulfilled. False-positive rate of the score is 17%, respectively, which is similar to previous data and should be considered for parental counselling.
  • #16 Chapter 13 – Pulmonary Atresia with Intact Ventricular Septum | Thoracic Key
    https://thoracickey.com/chapter-13-pulmonary-atresia-with-intact-ventricular-septum/
    This is an unusual condition (accounting for 5 per cent of all cases of pulmonary atresia (PA), although commoner in Eastern Asia) but one with a broad spectrum of treatment options depending on the degree of development of the right ventricle (RV). […] The key to management and the ultimate destination in this condition is based on assessment of the RV size. […] Table 13.1 summarizes the predictive outcome for the RV in the setting of PAIVS based on tricuspid valve size. […] Patients present as newborns with cyanosis, and survival is duct dependent. […] The mainstay of diagnosis is with echocardiography, and other imaging modalities are not usually required.
  • #17 Chapter 13 – Pulmonary Atresia with Intact Ventricular Septum | Thoracic Key
    https://thoracickey.com/chapter-13-pulmonary-atresia-with-intact-ventricular-septum/
    This is an unusual condition (accounting for 5 per cent of all cases of pulmonary atresia (PA), although commoner in Eastern Asia) but one with a broad spectrum of treatment options depending on the degree of development of the right ventricle (RV). […] The key to management and the ultimate destination in this condition is based on assessment of the RV size. […] Table 13.1 summarizes the predictive outcome for the RV in the setting of PAIVS based on tricuspid valve size. […] Patients present as newborns with cyanosis, and survival is duct dependent. […] The mainstay of diagnosis is with echocardiography, and other imaging modalities are not usually required.
  • #18 Chapter 13 – Pulmonary Atresia with Intact Ventricular Septum | Thoracic Key
    https://thoracickey.com/chapter-13-pulmonary-atresia-with-intact-ventricular-septum/
    This is an unusual condition (accounting for 5 per cent of all cases of pulmonary atresia (PA), although commoner in Eastern Asia) but one with a broad spectrum of treatment options depending on the degree of development of the right ventricle (RV). […] The key to management and the ultimate destination in this condition is based on assessment of the RV size. […] Table 13.1 summarizes the predictive outcome for the RV in the setting of PAIVS based on tricuspid valve size. […] Patients present as newborns with cyanosis, and survival is duct dependent. […] The mainstay of diagnosis is with echocardiography, and other imaging modalities are not usually required.
  • #19 Chapter 13 – Pulmonary Atresia with Intact Ventricular Septum | Thoracic Key
    https://thoracickey.com/chapter-13-pulmonary-atresia-with-intact-ventricular-septum/
    This is an unusual condition (accounting for 5 per cent of all cases of pulmonary atresia (PA), although commoner in Eastern Asia) but one with a broad spectrum of treatment options depending on the degree of development of the right ventricle (RV). […] The key to management and the ultimate destination in this condition is based on assessment of the RV size. […] Table 13.1 summarizes the predictive outcome for the RV in the setting of PAIVS based on tricuspid valve size. […] Patients present as newborns with cyanosis, and survival is duct dependent. […] The mainstay of diagnosis is with echocardiography, and other imaging modalities are not usually required.
  • #20 Pulmonary atresia with intact ventricular septum: predictors of early and medium-term outcome in a population-based study – PubMed
    https://pubmed.ncbi.nlm.nih.gov/16214522/
    Pulmonary atresia with intact ventricular septum is a form of congenital heart disease usually associated with right-heart hypoplasia, with considerable morphologic heterogeneity and often poor outlook. […] Ascertainment of risk factors for poor outcome is an important step if an improvement in outcome is to be achieved. […] One- and 5-year survival was 70.8% and 63.8%, respectively. […] Results from Cox proportional hazards model analysis showed that low birth weight (P = .024), unipartite right ventricular morphology (P = .001), and the presence of a dilated right ventricle (P .001) were independent risk factors for death. […] This population-based study has shown which features at presentation place an infant in a high-risk group. This is important information for counseling in fetal life and for surgical strategy after birth.
  • #21 Natural history of pulmonary atresia with intact ventricular septum (PAIVS) and critical pulmonary stenosis (CPS) and prediction of outcome
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8164597/
    To analyse prenatal parameters predicting biventricular (BV) outcome in pulmonary atresia with intact ventricular septum/critical pulmonary stenosis (PAIVS/CPS). […] Outcomes have improved over time, but are still guarded with reported one- and 5-year survival rates of 70-75% and 63-67%, depending on the type of postnatal circulation. […] Therefore, prediction of circulation outcome is an important aspect for parental counselling. […] The aim of our study was to describe outcomes in prenatally diagnosed PAIVS/CPS, to analyse foetal echocardiographic parameters for predicting BV outcome and growth rate of these during gestation in the largest cohort to date. […] In our cohort, the best single, echocardiographic parameter to predict BV outcome was TV/MV ratio at 24 weeks. […] Non-BV outcome was correctly predicted in all our cases if more than one criteria were fulfilled. […] To our knowledge, we evaluated one of the largest prenatal cohorts with PAIVS/CPS. TV/MV ratio is a simple single parameter with high predictive value. […] Criteria for foetal intervention should be evaluated further in a prospective manner.
  • #22 Natural history of pulmonary atresia with intact ventricular septum (PAIVS) and critical pulmonary stenosis (CPS) and prediction of outcome
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8164597/
    To analyse prenatal parameters predicting biventricular (BV) outcome in pulmonary atresia with intact ventricular septum/critical pulmonary stenosis (PAIVS/CPS). […] Outcomes have improved over time, but are still guarded with reported one- and 5-year survival rates of 70-75% and 63-67%, depending on the type of postnatal circulation. […] Therefore, prediction of circulation outcome is an important aspect for parental counselling. […] The aim of our study was to describe outcomes in prenatally diagnosed PAIVS/CPS, to analyse foetal echocardiographic parameters for predicting BV outcome and growth rate of these during gestation in the largest cohort to date. […] In our cohort, the best single, echocardiographic parameter to predict BV outcome was TV/MV ratio at 24 weeks. […] Non-BV outcome was correctly predicted in all our cases if more than one criteria were fulfilled. […] To our knowledge, we evaluated one of the largest prenatal cohorts with PAIVS/CPS. TV/MV ratio is a simple single parameter with high predictive value. […] Criteria for foetal intervention should be evaluated further in a prospective manner.
  • #23 Natural history of pulmonary atresia with intact ventricular septum (PAIVS) and critical pulmonary stenosis (CPS) and prediction of outcome | springermedizin.de
    https://www.springermedizin.de/natural-history-of-pulmonary-atresia-with-intact-ventricular-sep/18862602
    In our cohort, the best single, echocardiographic parameter to predict BV outcome was TV/MV ratio at 24 weeks. We included TV/MV ratio as best single parameter in combination with presence of VCAC and TI-Vmax2.5 m/s to a score. Non-BV outcome was correctly predicted in all our cases if more than one criteria were fulfilled. False-positive rate of the score is 17%, respectively, which is similar to previous data and should be considered for parental counselling.
  • #24
    https://link.springer.com/article/10.1007/s00404-020-05929-0
    To analyse prenatal parameters predicting biventricular (BV) outcome in pulmonary atresia with intact ventricular septum/critical pulmonary stenosis (PAIVS/CPS). […] Outcomes have improved over time, but are still guarded with reported one- and 5-year survival rates of 70-75% and 63-67%, depending on the type of postnatal circulation. […] Therefore, prediction of circulation outcome is an important aspect for parental counselling. […] The aim of our study was to describe outcomes in prenatally diagnosed PAIVS/CPS, to analyse foetal echocardiographic parameters for predicting BV outcome and growth rate of these during gestation in the largest cohort to date. […] Intention-to-treat survival rate (median follow-up 22.5 months) was 44/53 (83.0%) which fits to reported one-, 5- and 10-year survival rates ranging from 63 to 97%. […] In our cohort, the best single, echocardiographic parameter to predict BV outcome was TV/MV ratio 24 weeks. […] Non-BV outcome was correctly predicted in all our cases if more than one criteria were fulfilled. […] TV/MV ratio is a simple single parameter with high predictive value.

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niedomykalność trójdzielna interwencja płodowa zastawka trójdzielna poradnictwo prenatalne krążenie jednokomorowe drożność przewodu tętniczego sinica krążenie dwukomorowe wrodzona wada serca heterogenność morfologiczna krytyczne zwężenie zastawki płucnej atrezja płucna z zachowaną przegrodą międzykomorowa echokardiografia krzywa ROC połączenia wieńcowo-komorowe stratyfikacja ryzyka model proporcjonalnego hazardu Coxa