Wrodzona przepuklina przeponowa – Rokowania, prognozy i postęp choroby

Wrodzona przepuklina przeponowa
Rokowania, prognozy i postęp choroby

Wrodzona przepuklina przeponowa (CDH) charakteryzuje się przemieszczeniem zawartości jamy brzusznej do klatki piersiowej przez defekt przepony, co prowadzi do hipoplazji płuc i nadciśnienia płucnego, kluczowych determinantów śmiertelności i chorobowości. Przeżywalność ogólna wynosi około 67%, jednak u niemowląt wymagających ECMO spada do 50-61%. Rokowanie pogarsza się przy wczesnej prenatalnej diagnozie (<24. tygodnia), obecności wątroby w klatce piersiowej (szansa przeżycia 0,21; CI: 0,13-0,35) oraz niskim stosunku płuc do głowy (LHR ≤ 1,0; szansa przeżycia 0,14; CI: 0,10-0,27). Wskaźniki postnatalne, takie jak najwyższy wskaźnik oksygenacji w 1. dniu życia (HOId1) oraz odpowiedź na wziewny tlenek azotu (iNO), są istotnymi predyktorami przeżycia. Narzędzia prognostyczne, w tym SNAP-II, CDHSG-probability survival i skala Brindle, wykazują wysoką skuteczność (statystyka zgodności ~0,8) w ocenie ryzyka śmiertelności.

Wprowadzenie do wrodzonej przepukliny przeponowej
Czynniki prognostyczne i przewidywanie przeżywalności

Prenatalne czynniki prognostyczne
Postnatalne czynniki prognostyczne
Skale predykcyjne

Wskaźniki przeżywalności i rokowanie długoterminowe

Obecne wskaźniki przeżywalności
Szczególne przypadki i ich rokowanie
Czynniki wpływające na rokowanie długoterminowe
Powikłania długoterminowe

Wpływ strategii leczenia na przeżywalność

Strategie wentylacji
Zastosowanie wziewnego tlenku azotu

Podsumowanie czynników rokowniczych

Kolejne rozdziały

Wprowadzenie do wrodzonej przepukliny przeponowej

Wrodzona przepuklina przeponowa (ang. Congenital Diaphragmatic Hernia, CDH) to stan, w którym zawartość jamy brzusznej przedostaje się do klatki piersiowej przez defekt w mięśniu przepony. Jest to stan nagły wymagający pilnej interwencji, aby zapobiec dalszym powikłaniom lub śmierci.¹ Pomimo postępów w opiece neonatologicznej i chirurgicznej, leczenie CDH pozostaje wyzwaniem i brakuje jednoznacznych standardowych wytycznych terapeutycznych. Chociaż niektóre ośrodki raportują wskaźniki śmiertelności na poziomie zaledwie 20%, w przypadku konieczności zastosowania pozaustrojowego natleniania membranowego (ECMO) wskaźnik śmiertelności wzrasta do 50%.²

Ciężkość choroby związana jest ze stopniem hipoplazji płuc i nadciśnienia płucnego, które towarzyszą CDH. Oba te stany ograniczają zdolność noworodków do prawidłowej wentylacji i natlenowania po urodzeniu. Te fizjologiczne zaburzenia upośledzające wymianę gazową są kluczowymi determinantami chorobowości i śmiertelności u niemowląt z CDH.³

Czynniki prognostyczne i przewidywanie przeżywalności

Głównymi determinantami przeżycia u niemowląt z CDH są: obecność towarzyszących anomalii, stopień hipoplazji płuc oraz nadciśnienie płucne. Przypadki wykryte prenatalnie oraz zdiagnozowane przed 24. tygodniem ciąży mają gorsze rokowanie ze względu na wczesny etap rozwoju płuc, na którym wystąpiła anomalia. Śmiertelność w tej grupie sięga nawet 60-80%.⁴

Prenatalne czynniki prognostyczne

Kilka prenatalnych czynników ma istotny wpływ na rokowanie w CDH:

Obecność wątroby w klatce piersiowej w prenatalnym badaniu ultrasonograficznym jest złym czynnikiem prognostycznym dla przeżycia. Szansa przeżycia przy przepuklinie wątroby wynosiła 0,21 (CI: 0,13-0,35).⁵⁶
Stosunek płuc do głowy (LHR) – szansa przeżycia przy LHR ≤ 1,0 wynosiła zaledwie 0,14 (CI: 0,10-0,27).⁷ Płody z przepukliną wątroby do klatki piersiowej i współczynnikiem LHR poniżej 1,0 mają cięższą postać CDH i tym samym gorsze rokowanie.⁸
Obserwowany do oczekiwanego stosunek płuc do głowy (O/E LHR) – niemowlęta z CDH, które zmarły, miały niższy średni O/E LHR (p<0,001).⁹
Wiek ciążowy w momencie diagnozy prenatalnej.¹⁰

Postnatalne czynniki prognostyczne

Zidentyfikowano kilka postnatalnych wskaźników prognostycznych:

Najwyższy wskaźnik oksygenacji w 1. dniu życia (HOId1) był wyższy u niemowląt, które zmarły (p<0,001). HOId1 przewidywał przeżycie po skorygowaniu o wiek ciążowy, ocenę w skali Apgar w 5. minucie i O/E LHR (iloraz szans 0,948, 95% CI: 0,913-0,983).¹¹ Jest to lepszy predyktor przeżycia niż wiek ciążowy i ocena w skali Apgar w 5. minucie.¹²
Najlepszy wskaźnik oksygenacji w 1. dniu (BOId1) po urodzeniu był związany z lepszym przeżyciem u niemowląt z CDH.¹³
Odpowiedź na wziewny tlenek azotu (iNO) – procentowa zmiana w stosunku PaO2/FiO2 po rozpoczęciu podawania iNO była wyższa u niemowląt, które przeżyły (69,4%) w porównaniu do niemowląt, które zmarły (10,2%), p=0,018.¹⁴ Niemowlęta, które pozytywnie zareagowały na iNO, miały większe szanse na przeżycie.¹⁵
Nasilenie nadciśnienia płucnego.¹⁶
Średnica ubytku przepony.¹⁷

Skale predykcyjne

W badaniach zidentyfikowano kilka narzędzi predykcyjnych, które wykazują wysoką skuteczność w przewidywaniu śmiertelności u noworodków z CDH:

SNAP-II (Score for Neonatal Acute Physiology II) – uważana za niezależną od początkowej strategii wentylacji.¹⁸ Wykazuje wysoką statystykę zgodności (C) na poziomie 0,8.¹⁹
CDHSG-probability survival (Congenital Diaphragmatic Hernia Study Group) – ze statystyką zgodności 0,79.²⁰
Skala Brindle – również ze statystyką zgodności 0,8.²¹
Model nomogramowy oparty na sześciu czynnikach: wiek ciążowy w momencie diagnozy prenatalnej, O/E LHR, przepuklina wątroby, nasilenie nadciśnienia płucnego, średnica ubytku i wskaźnik oksygenacji.²²²³

Wskaźniki przeżywalności i rokowanie długoterminowe

Wskaźniki przeżywalności w CDH znacznie się poprawiły w ostatnich latach, chociaż nadal występuje duża zmienność w zależności od ciężkości przypadku i zastosowanego leczenia.

Obecne wskaźniki przeżywalności

Ogólna przeżywalność dla niemowląt z CDH wynosi około 67%.²⁴
Dla niemowląt wymagających ECMO przeżywalność wynosi około 61%.²⁵
Śmiertelność u pacjentów leczonych ECMO utrzymuje się na poziomie 50%.²⁶
Dzięki postępom w leczeniu CDH ogólna przeżywalność poprawiła się i wynosi 70-90% u niemowląt niewymagających ECMO oraz do 50% u niemowląt poddawanych ECMO.²⁷
Współczesne raporty wskazują, że przeżywalność wynosi od 70 do 90% noworodków.²⁸

Szczególne przypadki i ich rokowanie

Stan kliniczny	Wskaźnik przeżywalności	Uwagi
CDH z wodobrzuszem (hydrops)	43% ogólna	Rzadkie (2,8% wszystkich prenatalnie zdiagnozowanych przypadków)
Hydrops z CDH po operacji	80%	Wyższy wskaźnik niepodejmowania leczenia operacyjnego (45%)
Prawostronny CDH z wodobrzuszem	niższy	Wodobrzusze częściej występuje w prawostronnej CDH (55%)
Prawdziwy hydrops z CDH	22%	Bardzo rzadki (0,35% prenatalnie zdiagnozowanych przypadków CDH)
CDH z wątrobą w klatce piersiowej	21% szans	CI: 0,13-0,35
CDH z LHR ≤ 1.0	14% szans	CI: 0,10-0,27

Dane źródłowe:²⁹³⁰

Czynniki wpływające na rokowanie długoterminowe

Rokowanie długoterminowe zależy od kilku czynników, w tym:³¹

Czy dziecko urodziło się przedwcześnie
Jak ciężki jest stan dziecka po urodzeniu
Wielkość przepukliny
Jakie narządy są zaangażowane
Inne współistniejące choroby

Niemowlęta wymagające dłuższego wspomagania oddechowego lub żywieniowego mają wyższe ryzyko utrzymujących się powikłań, w tym przewlekłej choroby płuc, zaburzeń wzrostu, utraty słuchu i opóźnień rozwojowych.³²

Powikłania długoterminowe

Nawet w przypadku przeżycia, dzieci po leczeniu ciężkiej postaci CDH mogą doświadczać długoterminowych problemów zdrowotnych:³³³⁴

Problemy oddechowe – nawracające infekcje, astma, ograniczona wydolność oddechowa
Zaburzenia wzrostu – wolniejszy przyrost masy ciała i wzrostu
Problemy z karmieniem – trudności z przyjmowaniem pokarmu, refluks żołądkowo-przełykowy
Utrata słuchu – częściej występująca u dzieci po intensywnej terapii
Łagodne zaburzenia poznawcze i uczenia się – mogą wymagać dodatkowego wsparcia edukacyjnego

Wpływ strategii leczenia na przeżywalność

Wprowadzenie określonych strategii terapeutycznych miało znaczący wpływ na poprawę wskaźników przeżywalności w CDH:

Strategie wentylacji

Wprowadzenie strategii delikatnej wentylacji, permisywnej hiperkapni i spontanicznego oddychania miało bardziej znaczący wpływ na przeżywalność niż wentylacja wysokoczęstotliwościowa (HFOV) czy ECMO jako terapia ratunkowa.³⁵ Badania wykazały, że niemowlęta z CDH, które wymagały HFOV przedoperacyjnie, miały prawie zero dni bez wentylacji w pierwszych 28 dniach po urodzeniu.³⁶

Zastosowanie wziewnego tlenku azotu

Chociaż stosowanie wziewnego tlenku azotu (iNO) w leczeniu nadciśnienia płucnego u niemowląt z CDH jest kontrowersyjne, badania wskazują, że:³⁷

iNO poprawiło natlenowanie u niektórych niemowląt z CDH
Pozytywna odpowiedź na iNO była bardziej prawdopodobna u tych, którzy przeżyli
Procentowa zmiana stosunku PaO2/FiO2 po rozpoczęciu iNO była wyższa u niemowląt, które przeżyły (69,4%) w porównaniu do tych, które zmarły (10,2%), p=0,018³⁸

Odpowiedź na wziewny tlenek azotu była zmienna u niemowląt z CDH, ale te, które pozytywnie zareagowały na iNO, miały większe szanse na przeżycie.³⁹⁴⁰

Podsumowanie czynników rokowniczych

Najważniejsze czynniki wpływające na rokowanie w CDH to:⁴¹⁴²

Wiek ciążowy w momencie diagnozy prenatalnej
Obserwowany do oczekiwanego stosunek płuc do głowy (O/E LHR)
Przepuklina wątroby do klatki piersiowej
Nasilenie nadciśnienia płucnego
Średnica ubytku przepony
Wskaźnik oksygenacji (OI)

Pomimo znaczącej poprawy technik chirurgii noworodkowej i intensywnej opieki, wskaźnik śmiertelności noworodków z rozpoznaniem CDH pozostaje bardzo wysoki.⁴³ Jednak należy podkreślić, że istnieje szeroki zakres ciężkości i wyników dla CDH. W najlepszych przypadkach niektóre niemowlęta radzą sobie bardzo dobrze po leczeniu poporodowym, operacji i opiece na oddziale intensywnej terapii noworodka. W najcięższych przypadkach niektóre nie przeżyją pomimo naszych intensywnych wysiłków.⁴⁴

Płody z najlepszego końca spektrum mają doskonałą szansę na prowadzenie całkowicie normalnego życia. Nie wymagają specjalnego postępowania prenatalnego w zakresie czasu lub rodzaju porodu, ale powinny być urodzone w ośrodku perinatologicznym z oddziałem intensywnej opieki noworodkowej poziomu III z wsparciem neonatologicznym i chirurgicznym.⁴⁵

Nawet w przypadku ciężkiej CDH, ponad 70% dotkniętych niemowląt może zostać uratowanych dzięki intensywnemu wsparciu. Jednak mogą wystąpić długoterminowe problemy zdrowotne związane z oddychaniem, karmieniem, wzrostem i rozwojem.⁴⁶

Kolejne rozdziały

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Materiały źródłowe

#1 Survival and Predictors of Mortality of Congenital Diaphragmatic Hernia in Newborns at a Tertiary Care Hospital in Saudi Arabia
https://pmc.ncbi.nlm.nih.gov/articles/PMC10946794/
Congenital diaphragmatic hernia (CDH) is a condition where abdominal contents protrude into the chest due to defects in the diaphragm muscle. It is considered an emergency that needs urgent intervention to prevent further complications or death. Our study aimed to estimate survival and evaluate predictors of mortality in newborns with CDH using available prediction tools in the literature. […] The mortality rate was 51.1%. […] One prenatal predictor tool, the lung-to-head ratio, was found to be significant; in addition, three postnatal predictor tools of mortality, SNAP-II, CDHSG-probability survival, and Brindle Score, had the highest concordance (C) statistics of 0.8, 0.79, and 0.8, respectively. […] The most significant predictors of mortality were lung-to-head ratio (prenatally), SNAP-II, CDHSG-probability of survival, and Brindle Score (postnatally).
#2 Congenital Diaphragmatic Hernia | IntechOpen
https://www.intechopen.com/chapters/56039
Despite advances in neonatal and surgical care, the management of congenital diaphragmatic hernia (CDH) remains challenging with no definitive standard treatment guidelines. Several centers report mortality rates as low as 20%, but if extracorporeal membrane oxygenation (ECMO) support is required, the mortality rate rises to 50%. The disease severity is related to the degree of pulmonary hypoplasia and pulmonary hypertension that occurs with CDH. Both conditions decrease the infants ability to ventilate and oxygenate adequately at delivery. These physiologic conditions that impair gas exchange are the important determinants of morbidity and mortality in CDH infants. […] Prognosis: The primary determinants of survival for infants with CDH are the presence of associated anomalies, pulmonary hypoplasia, and pulmonary hypertension. Prenatally discovered cases and cases diagnosed before 24-week gestation have a worse prognosis because of the early stage of the lung development when the anomaly has occurred. Mortality in this group is reported as high as 60-80%.
#3 Congenital Diaphragmatic Hernia | IntechOpen
https://www.intechopen.com/chapters/56039
Despite advances in neonatal and surgical care, the management of congenital diaphragmatic hernia (CDH) remains challenging with no definitive standard treatment guidelines. Several centers report mortality rates as low as 20%, but if extracorporeal membrane oxygenation (ECMO) support is required, the mortality rate rises to 50%. The disease severity is related to the degree of pulmonary hypoplasia and pulmonary hypertension that occurs with CDH. Both conditions decrease the infants ability to ventilate and oxygenate adequately at delivery. These physiologic conditions that impair gas exchange are the important determinants of morbidity and mortality in CDH infants. […] Prognosis: The primary determinants of survival for infants with CDH are the presence of associated anomalies, pulmonary hypoplasia, and pulmonary hypertension. Prenatally discovered cases and cases diagnosed before 24-week gestation have a worse prognosis because of the early stage of the lung development when the anomaly has occurred. Mortality in this group is reported as high as 60-80%.
#4 Congenital Diaphragmatic Hernia | IntechOpen
https://www.intechopen.com/chapters/56039
Despite advances in neonatal and surgical care, the management of congenital diaphragmatic hernia (CDH) remains challenging with no definitive standard treatment guidelines. Several centers report mortality rates as low as 20%, but if extracorporeal membrane oxygenation (ECMO) support is required, the mortality rate rises to 50%. The disease severity is related to the degree of pulmonary hypoplasia and pulmonary hypertension that occurs with CDH. Both conditions decrease the infants ability to ventilate and oxygenate adequately at delivery. These physiologic conditions that impair gas exchange are the important determinants of morbidity and mortality in CDH infants. […] Prognosis: The primary determinants of survival for infants with CDH are the presence of associated anomalies, pulmonary hypoplasia, and pulmonary hypertension. Prenatally discovered cases and cases diagnosed before 24-week gestation have a worse prognosis because of the early stage of the lung development when the anomaly has occurred. Mortality in this group is reported as high as 60-80%.
#5 Congenital Diaphragmatic Hernia | IntechOpen
https://www.intechopen.com/chapters/56039
The presence of liver in the chest on prenatal ultrasound is a poor prognosticator for survival. […] The odds of survival with LHR 1.0 was 0.14 (CI: 0.10-0.27) and with liver herniation on ultrasound it was 0.21 (CI: 0.13-0.35). […] The SNAP 2 score is considered independent of initial ventilation strategy. […] The introduction of gentle ventilation strategies, permissive hypercapnia and spontaneous respiration had a more significant effect on survival than HFOV or ECMO as rescue therapy. […] The overall survival for CDH infants was 67% and for those requiring ECMO, it was 61%. […] The mortality in ECMO-treated patients was 50% in both time periods. […] With advances in CDH management, the overall survival has improved and has been reported between 70 and 90% in non-ECMO infants and up to 50% in infants who undergo ECMO.
#6 Congenital Diaphragmatic Hernia (CDH)
https://fetus.ucsf.edu/cdh/
On the other end of the spectrum, babies with severe CDH and very small lungs can have difficult challenges after birth, and some will not survive. […] Even when the CDH is severe, greater than 70% of affected babies can be saved with intensive support. However, there can be long term health issues related to breathing, feeding, growth and development. […] When CDH is the only problem, we have learned that severity and, thus, outcome is determined by two factors: 1) liver position, and 2) lung-to-head ratio or LHR. […] Fetuses who do not have liver herniated into the chest (liver down) usually have a less severe form of CDH and therefore a better prognosis. […] Fetuses with liver herniated into the chest and a lung-head ratio less than 1.0 have a more severe form of CDH. We can predict that they will have a very difficult time after birth.
#7 Congenital Diaphragmatic Hernia | IntechOpen
https://www.intechopen.com/chapters/56039
The presence of liver in the chest on prenatal ultrasound is a poor prognosticator for survival. […] The odds of survival with LHR 1.0 was 0.14 (CI: 0.10-0.27) and with liver herniation on ultrasound it was 0.21 (CI: 0.13-0.35). […] The SNAP 2 score is considered independent of initial ventilation strategy. […] The introduction of gentle ventilation strategies, permissive hypercapnia and spontaneous respiration had a more significant effect on survival than HFOV or ECMO as rescue therapy. […] The overall survival for CDH infants was 67% and for those requiring ECMO, it was 61%. […] The mortality in ECMO-treated patients was 50% in both time periods. […] With advances in CDH management, the overall survival has improved and has been reported between 70 and 90% in non-ECMO infants and up to 50% in infants who undergo ECMO.
#8 Congenital Diaphragmatic Hernia (CDH)
https://fetus.ucsf.edu/cdh/
On the other end of the spectrum, babies with severe CDH and very small lungs can have difficult challenges after birth, and some will not survive. […] Even when the CDH is severe, greater than 70% of affected babies can be saved with intensive support. However, there can be long term health issues related to breathing, feeding, growth and development. […] When CDH is the only problem, we have learned that severity and, thus, outcome is determined by two factors: 1) liver position, and 2) lung-to-head ratio or LHR. […] Fetuses who do not have liver herniated into the chest (liver down) usually have a less severe form of CDH and therefore a better prognosis. […] Fetuses with liver herniated into the chest and a lung-head ratio less than 1.0 have a more severe form of CDH. We can predict that they will have a very difficult time after birth.
#9 Prediction of survival in infants with congenital diaphragmatic hernia and the response to inhaled nitric oxide
https://pmc.ncbi.nlm.nih.gov/articles/PMC9508000/
The use of inhaled nitric oxide (iNO) in treating pulmonary hypertension in infants with congenital diaphragmatic hernia (CDH) is controversial. […] Factors affecting survival were determined. […] The CDH infants who died had a lower median observed to expected lung to head ratio (O/E LHR) (p0.001) and a higher median highest OI on day 1 (HOId1) (p0.001). HOId1 predicted survival after adjusting for gestational age, Apgar score at 5 min and O/E LHR (odds ratio 0.948 (95% confidence intervals 0.9130.983)). […] The highest OI on day 1 predicted survival. iNO improved oxygenation in certain CDH infants and a positive response was more likely in those who survived. […] We have examined predictors of survival in CDH infants including the response to iNO and demonstrated that the highest oxygenation index on day 1 predicted survival (AUCROC =0.908).
#10 Development of a prediction nomogram for 1-month mortality in neonates with congenital diaphragmatic hernia | BMC Surgery | Full Text
https://bmcsurg.biomedcentral.com/articles/10.1186/s12893-024-02479-z
Although many prognostic factors in neonates with congenital diaphragmatic hernia (CDH) have been described, no consensus thus far has been reached on which and how many factors are involved. […] The aim of this study is to analyze the association of multiple prenatal and postnatal factors with 1-month mortality of neonates with CDH and to construct a nomogram prediction model based on significant factors. […] After graded multivariable adjustment, six factors were found to be independently and consistently associated with the significant risk of 1-month mortality in neonates with CDH, including gestational age of prenatal diagnosis, observed-to-expected lung-to-head ratio, liver herniation, severity of pulmonary hypertension, diameter of defect, and oxygen index. […] Our findings provide evidence for the association of six preoperational and intraoperative factors with the risk of 1-month mortality in neonates with CDH, and this association was reinforced in a nomogram model.
#11 Prediction of survival in infants with congenital diaphragmatic hernia and the response to inhaled nitric oxide
https://pmc.ncbi.nlm.nih.gov/articles/PMC9508000/
The use of inhaled nitric oxide (iNO) in treating pulmonary hypertension in infants with congenital diaphragmatic hernia (CDH) is controversial. […] Factors affecting survival were determined. […] The CDH infants who died had a lower median observed to expected lung to head ratio (O/E LHR) (p0.001) and a higher median highest OI on day 1 (HOId1) (p0.001). HOId1 predicted survival after adjusting for gestational age, Apgar score at 5 min and O/E LHR (odds ratio 0.948 (95% confidence intervals 0.9130.983)). […] The highest OI on day 1 predicted survival. iNO improved oxygenation in certain CDH infants and a positive response was more likely in those who survived. […] We have examined predictors of survival in CDH infants including the response to iNO and demonstrated that the highest oxygenation index on day 1 predicted survival (AUCROC =0.908).
#12
https://link.springer.com/article/10.1007/s00431-022-04568-8
The highest OI on day 1 predicted survival. […] The percentage change in the PaO2/FiO2 ratio post iNO initiation was higher in infants who survived (69.4%) compared to infants who died (10.2%), p=0.018. […] We have demonstrated that survival in infants with CDH is related to highest OI on day 1 after birth. Furthermore, we highlight that the response to iNO can predict survival in CDH infants. […] Our study showed that the HOI on day 1 was associated with survival after adjusting for gestational age, Apgar score at 5 min and the O/E LHR. […] The response to inhaled nitric oxide was variable in infants with CDH in our study, but infants who responded to iNO were more likely to survive. […] In conclusion, the highest oxygenation index on day 1 of life was a better predictor of survival than gestational age and the Apgar score at 5 min. Inhaled nitric oxide was associated with improved oxygenation in certain infants with CDH and the response to nitric oxide was better in those infants who survived.
#13 Prediction of survival in infants with congenital diaphragmatic hernia and the response to inhaled nitric oxide
https://pmc.ncbi.nlm.nih.gov/articles/PMC9508000/
Certain infants with CDH responded to iNO and those with a greater response were more likely to survive. […] The best oxygenation index on day 1 (BOId1) after birth was associated with better survival in CDH infants. […] Our study showed that the HOI on day 1 was associated with survival after adjusting for gestational age, Apgar score at 5 min and the O/E LHR. […] Our study showed that CDH infants who required HFO preoperatively had almost no ventilator free days within the first 28 days after birth. […] The response to inhaled nitric oxide was variable in infants with CDH in our study, but infants who responded to iNO were more likely to survive. […] In conclusion, the highest oxygenation index on day 1 of life was a better predictor of survival than gestational age and the Apgar score at 5 min. Inhaled nitric oxide was associated with improved oxygenation in certain infants with CDH and the response to nitric oxide was better in those infants who survived.
#14
https://link.springer.com/article/10.1007/s00431-022-04568-8
The highest OI on day 1 predicted survival. […] The percentage change in the PaO2/FiO2 ratio post iNO initiation was higher in infants who survived (69.4%) compared to infants who died (10.2%), p=0.018. […] We have demonstrated that survival in infants with CDH is related to highest OI on day 1 after birth. Furthermore, we highlight that the response to iNO can predict survival in CDH infants. […] Our study showed that the HOI on day 1 was associated with survival after adjusting for gestational age, Apgar score at 5 min and the O/E LHR. […] The response to inhaled nitric oxide was variable in infants with CDH in our study, but infants who responded to iNO were more likely to survive. […] In conclusion, the highest oxygenation index on day 1 of life was a better predictor of survival than gestational age and the Apgar score at 5 min. Inhaled nitric oxide was associated with improved oxygenation in certain infants with CDH and the response to nitric oxide was better in those infants who survived.
#15 Prediction of survival in infants with congenital diaphragmatic hernia and the response to inhaled nitric oxide
https://pmc.ncbi.nlm.nih.gov/articles/PMC9508000/
Certain infants with CDH responded to iNO and those with a greater response were more likely to survive. […] The best oxygenation index on day 1 (BOId1) after birth was associated with better survival in CDH infants. […] Our study showed that the HOI on day 1 was associated with survival after adjusting for gestational age, Apgar score at 5 min and the O/E LHR. […] Our study showed that CDH infants who required HFO preoperatively had almost no ventilator free days within the first 28 days after birth. […] The response to inhaled nitric oxide was variable in infants with CDH in our study, but infants who responded to iNO were more likely to survive. […] In conclusion, the highest oxygenation index on day 1 of life was a better predictor of survival than gestational age and the Apgar score at 5 min. Inhaled nitric oxide was associated with improved oxygenation in certain infants with CDH and the response to nitric oxide was better in those infants who survived.
#16 Development of a prediction nomogram for 1-month mortality in neonates with congenital diaphragmatic hernia | BMC Surgery | Full Text
https://bmcsurg.biomedcentral.com/articles/10.1186/s12893-024-02479-z
Although many prognostic factors in neonates with congenital diaphragmatic hernia (CDH) have been described, no consensus thus far has been reached on which and how many factors are involved. […] The aim of this study is to analyze the association of multiple prenatal and postnatal factors with 1-month mortality of neonates with CDH and to construct a nomogram prediction model based on significant factors. […] After graded multivariable adjustment, six factors were found to be independently and consistently associated with the significant risk of 1-month mortality in neonates with CDH, including gestational age of prenatal diagnosis, observed-to-expected lung-to-head ratio, liver herniation, severity of pulmonary hypertension, diameter of defect, and oxygen index. […] Our findings provide evidence for the association of six preoperational and intraoperative factors with the risk of 1-month mortality in neonates with CDH, and this association was reinforced in a nomogram model.
#17 Development of a prediction nomogram for 1-month mortality in neonates with congenital diaphragmatic hernia | BMC Surgery | Full Text
https://bmcsurg.biomedcentral.com/articles/10.1186/s12893-024-02479-z
Although many prognostic factors in neonates with congenital diaphragmatic hernia (CDH) have been described, no consensus thus far has been reached on which and how many factors are involved. […] The aim of this study is to analyze the association of multiple prenatal and postnatal factors with 1-month mortality of neonates with CDH and to construct a nomogram prediction model based on significant factors. […] After graded multivariable adjustment, six factors were found to be independently and consistently associated with the significant risk of 1-month mortality in neonates with CDH, including gestational age of prenatal diagnosis, observed-to-expected lung-to-head ratio, liver herniation, severity of pulmonary hypertension, diameter of defect, and oxygen index. […] Our findings provide evidence for the association of six preoperational and intraoperative factors with the risk of 1-month mortality in neonates with CDH, and this association was reinforced in a nomogram model.
#18 Congenital Diaphragmatic Hernia | IntechOpen
https://www.intechopen.com/chapters/56039
The presence of liver in the chest on prenatal ultrasound is a poor prognosticator for survival. […] The odds of survival with LHR 1.0 was 0.14 (CI: 0.10-0.27) and with liver herniation on ultrasound it was 0.21 (CI: 0.13-0.35). […] The SNAP 2 score is considered independent of initial ventilation strategy. […] The introduction of gentle ventilation strategies, permissive hypercapnia and spontaneous respiration had a more significant effect on survival than HFOV or ECMO as rescue therapy. […] The overall survival for CDH infants was 67% and for those requiring ECMO, it was 61%. […] The mortality in ECMO-treated patients was 50% in both time periods. […] With advances in CDH management, the overall survival has improved and has been reported between 70 and 90% in non-ECMO infants and up to 50% in infants who undergo ECMO.
#19 Survival and Predictors of Mortality of Congenital Diaphragmatic Hernia in Newborns at a Tertiary Care Hospital in Saudi Arabia
https://pmc.ncbi.nlm.nih.gov/articles/PMC10946794/
Congenital diaphragmatic hernia (CDH) is a condition where abdominal contents protrude into the chest due to defects in the diaphragm muscle. It is considered an emergency that needs urgent intervention to prevent further complications or death. Our study aimed to estimate survival and evaluate predictors of mortality in newborns with CDH using available prediction tools in the literature. […] The mortality rate was 51.1%. […] One prenatal predictor tool, the lung-to-head ratio, was found to be significant; in addition, three postnatal predictor tools of mortality, SNAP-II, CDHSG-probability survival, and Brindle Score, had the highest concordance (C) statistics of 0.8, 0.79, and 0.8, respectively. […] The most significant predictors of mortality were lung-to-head ratio (prenatally), SNAP-II, CDHSG-probability of survival, and Brindle Score (postnatally).
#20 Survival and Predictors of Mortality of Congenital Diaphragmatic Hernia in Newborns at a Tertiary Care Hospital in Saudi Arabia
https://pmc.ncbi.nlm.nih.gov/articles/PMC10946794/
Congenital diaphragmatic hernia (CDH) is a condition where abdominal contents protrude into the chest due to defects in the diaphragm muscle. It is considered an emergency that needs urgent intervention to prevent further complications or death. Our study aimed to estimate survival and evaluate predictors of mortality in newborns with CDH using available prediction tools in the literature. […] The mortality rate was 51.1%. […] One prenatal predictor tool, the lung-to-head ratio, was found to be significant; in addition, three postnatal predictor tools of mortality, SNAP-II, CDHSG-probability survival, and Brindle Score, had the highest concordance (C) statistics of 0.8, 0.79, and 0.8, respectively. […] The most significant predictors of mortality were lung-to-head ratio (prenatally), SNAP-II, CDHSG-probability of survival, and Brindle Score (postnatally).
#21 Survival and Predictors of Mortality of Congenital Diaphragmatic Hernia in Newborns at a Tertiary Care Hospital in Saudi Arabia
https://pmc.ncbi.nlm.nih.gov/articles/PMC10946794/
Congenital diaphragmatic hernia (CDH) is a condition where abdominal contents protrude into the chest due to defects in the diaphragm muscle. It is considered an emergency that needs urgent intervention to prevent further complications or death. Our study aimed to estimate survival and evaluate predictors of mortality in newborns with CDH using available prediction tools in the literature. […] The mortality rate was 51.1%. […] One prenatal predictor tool, the lung-to-head ratio, was found to be significant; in addition, three postnatal predictor tools of mortality, SNAP-II, CDHSG-probability survival, and Brindle Score, had the highest concordance (C) statistics of 0.8, 0.79, and 0.8, respectively. […] The most significant predictors of mortality were lung-to-head ratio (prenatally), SNAP-II, CDHSG-probability of survival, and Brindle Score (postnatally).
#22 Development of a prediction nomogram for 1-month mortality in neonates with congenital diaphragmatic hernia | BMC Surgery | Full Text
https://bmcsurg.biomedcentral.com/articles/10.1186/s12893-024-02479-z
Although many prognostic factors in neonates with congenital diaphragmatic hernia (CDH) have been described, no consensus thus far has been reached on which and how many factors are involved. […] The aim of this study is to analyze the association of multiple prenatal and postnatal factors with 1-month mortality of neonates with CDH and to construct a nomogram prediction model based on significant factors. […] After graded multivariable adjustment, six factors were found to be independently and consistently associated with the significant risk of 1-month mortality in neonates with CDH, including gestational age of prenatal diagnosis, observed-to-expected lung-to-head ratio, liver herniation, severity of pulmonary hypertension, diameter of defect, and oxygen index. […] Our findings provide evidence for the association of six preoperational and intraoperative factors with the risk of 1-month mortality in neonates with CDH, and this association was reinforced in a nomogram model.
#23 Development of a prediction nomogram for 1-month mortality in neonates with congenital diaphragmatic hernia | BMC Surgery | Full Text
https://bmcsurg.biomedcentral.com/articles/10.1186/s12893-024-02479-z
Despite significant improvement in neonatal surgical techniques and intensive care, the mortality rate of neonates diagnosed with CDH still remains very high. […] The key findings of this study are the identification of six independent factors, including GA of prenatal diagnosis, o/e LHR, liver herniation, severity of PH, diameter of defect, and OI in predicting the significant risk of 1-month mortality in neonates with CDH on the first day of life. […] Taken together, our findings provide evidence for the association of six prenatal and early postnatal factors with the significant risk of 1-month mortality in neonates with CDH, and this association was reinforced in a nomogram model.
#24 Congenital Diaphragmatic Hernia | IntechOpen
https://www.intechopen.com/chapters/56039
The presence of liver in the chest on prenatal ultrasound is a poor prognosticator for survival. […] The odds of survival with LHR 1.0 was 0.14 (CI: 0.10-0.27) and with liver herniation on ultrasound it was 0.21 (CI: 0.13-0.35). […] The SNAP 2 score is considered independent of initial ventilation strategy. […] The introduction of gentle ventilation strategies, permissive hypercapnia and spontaneous respiration had a more significant effect on survival than HFOV or ECMO as rescue therapy. […] The overall survival for CDH infants was 67% and for those requiring ECMO, it was 61%. […] The mortality in ECMO-treated patients was 50% in both time periods. […] With advances in CDH management, the overall survival has improved and has been reported between 70 and 90% in non-ECMO infants and up to 50% in infants who undergo ECMO.
#25 Congenital Diaphragmatic Hernia | IntechOpen
https://www.intechopen.com/chapters/56039
The presence of liver in the chest on prenatal ultrasound is a poor prognosticator for survival. […] The odds of survival with LHR 1.0 was 0.14 (CI: 0.10-0.27) and with liver herniation on ultrasound it was 0.21 (CI: 0.13-0.35). […] The SNAP 2 score is considered independent of initial ventilation strategy. […] The introduction of gentle ventilation strategies, permissive hypercapnia and spontaneous respiration had a more significant effect on survival than HFOV or ECMO as rescue therapy. […] The overall survival for CDH infants was 67% and for those requiring ECMO, it was 61%. […] The mortality in ECMO-treated patients was 50% in both time periods. […] With advances in CDH management, the overall survival has improved and has been reported between 70 and 90% in non-ECMO infants and up to 50% in infants who undergo ECMO.
#26 Congenital Diaphragmatic Hernia | IntechOpen
https://www.intechopen.com/chapters/56039
The presence of liver in the chest on prenatal ultrasound is a poor prognosticator for survival. […] The odds of survival with LHR 1.0 was 0.14 (CI: 0.10-0.27) and with liver herniation on ultrasound it was 0.21 (CI: 0.13-0.35). […] The SNAP 2 score is considered independent of initial ventilation strategy. […] The introduction of gentle ventilation strategies, permissive hypercapnia and spontaneous respiration had a more significant effect on survival than HFOV or ECMO as rescue therapy. […] The overall survival for CDH infants was 67% and for those requiring ECMO, it was 61%. […] The mortality in ECMO-treated patients was 50% in both time periods. […] With advances in CDH management, the overall survival has improved and has been reported between 70 and 90% in non-ECMO infants and up to 50% in infants who undergo ECMO.
#27 Congenital Diaphragmatic Hernia | IntechOpen
https://www.intechopen.com/chapters/56039
The presence of liver in the chest on prenatal ultrasound is a poor prognosticator for survival. […] The odds of survival with LHR 1.0 was 0.14 (CI: 0.10-0.27) and with liver herniation on ultrasound it was 0.21 (CI: 0.13-0.35). […] The SNAP 2 score is considered independent of initial ventilation strategy. […] The introduction of gentle ventilation strategies, permissive hypercapnia and spontaneous respiration had a more significant effect on survival than HFOV or ECMO as rescue therapy. […] The overall survival for CDH infants was 67% and for those requiring ECMO, it was 61%. […] The mortality in ECMO-treated patients was 50% in both time periods. […] With advances in CDH management, the overall survival has improved and has been reported between 70 and 90% in non-ECMO infants and up to 50% in infants who undergo ECMO.
#28 Congenital Diaphragmatic Hernia (CDH): What Parents Need To Know
https://my.clevelandclinic.org/health/diseases/24308-diaphragmatic-hernia
The reported survival rate for babies born with CDH has improved. Between 7 and 9 out of every 10 babies survive. These babies are born critically ill, but for those who make it through the tense early days of their condition, the outlook gets better. Some children may have long-term complications, but theyll still live long and full lives. Continuing advances in medicine improve the odds of both short-term survival and long-term health. […] The longer-term prognosis depends on several factors, including: If your baby was born prematurely, How severe your babys condition is at birth, The size of the hernia, What organs are involved, Other health conditions. […] Babies who require breathing or feeding support for longer have a higher risk of ongoing complications, including chronic lung disease, growth failure, hearing loss and developmental delays. Your childs provider will monitor them closely throughout their early life. […] Though this is a difficult time, you and your newborn will have a team of healthcare providers looking out for you. Theyll support you at every step from the moment of diagnosis through your childs early life.
#29 Hydrops and congenital diaphragmatic hernia: reported incidence and postnatal outcomes. Analysis of the congenital diaphragmatic hernia study group registry | Journal of Perinatology
https://www.nature.com/articles/s41372-024-02010-5
Congenital Diaphragmatic Hernia (CDH) associated with hydrops is rare. The overall survival to discharge for CDH patients with hydrops was 43%. The hydropic CDH group had lower birth weight and gestational age at birth, and increased incidence of right-sided CDH (55%), and rate of non-repair (45%). However, the survival rate for hydropic infants with CDH undergoing surgical repair was 80%. Hydropic CDH is rare, only 2.8% of all prenatally diagnosed cases, and more commonly occurring in right-sided CDH. Survival rates are low, with higher rates of non-repair. However, decision-making regarding goals of care and an aggressive surgical approach in selected cases may result in survival rates comparable to non-hydropic cases. Overall survival rates are still low, ranging from 65 to 85%. Due to the heterogeneity of this malformation, it is difficult to accurately predict outcomes in individual cases. Prenatal factors associated with worse outcome include decreased gestational age or weight at diagnosis, decreased lung volume, thoracic liver location and percent herniation, thoracic stomach position, and the presence of associated structural or chromosomal anomalies. The combination of hydrops and CDH is rare, with only a few series and case reports published, and is generally associated with a worse outcome. The presence of excess fluid in only one compartment is associated with worse outcome. True hydropic CDH is even rarer, only identified in 0.35% of the prenatally diagnosed CDH cases, and more commonly occurring in right-sided CDH. Survival rates for true hydropic CDH are very low: 78% of patients with true hydrops did not survive to discharge.
#30 Congenital Diaphragmatic Hernia | IntechOpen
https://www.intechopen.com/chapters/56039
The presence of liver in the chest on prenatal ultrasound is a poor prognosticator for survival. […] The odds of survival with LHR 1.0 was 0.14 (CI: 0.10-0.27) and with liver herniation on ultrasound it was 0.21 (CI: 0.13-0.35). […] The SNAP 2 score is considered independent of initial ventilation strategy. […] The introduction of gentle ventilation strategies, permissive hypercapnia and spontaneous respiration had a more significant effect on survival than HFOV or ECMO as rescue therapy. […] The overall survival for CDH infants was 67% and for those requiring ECMO, it was 61%. […] The mortality in ECMO-treated patients was 50% in both time periods. […] With advances in CDH management, the overall survival has improved and has been reported between 70 and 90% in non-ECMO infants and up to 50% in infants who undergo ECMO.
#31 Congenital Diaphragmatic Hernia (CDH): What Parents Need To Know
https://my.clevelandclinic.org/health/diseases/24308-diaphragmatic-hernia
The reported survival rate for babies born with CDH has improved. Between 7 and 9 out of every 10 babies survive. These babies are born critically ill, but for those who make it through the tense early days of their condition, the outlook gets better. Some children may have long-term complications, but theyll still live long and full lives. Continuing advances in medicine improve the odds of both short-term survival and long-term health. […] The longer-term prognosis depends on several factors, including: If your baby was born prematurely, How severe your babys condition is at birth, The size of the hernia, What organs are involved, Other health conditions. […] Babies who require breathing or feeding support for longer have a higher risk of ongoing complications, including chronic lung disease, growth failure, hearing loss and developmental delays. Your childs provider will monitor them closely throughout their early life. […] Though this is a difficult time, you and your newborn will have a team of healthcare providers looking out for you. Theyll support you at every step from the moment of diagnosis through your childs early life.
#32 Congenital Diaphragmatic Hernia (CDH): What Parents Need To Know
https://my.clevelandclinic.org/health/diseases/24308-diaphragmatic-hernia
The reported survival rate for babies born with CDH has improved. Between 7 and 9 out of every 10 babies survive. These babies are born critically ill, but for those who make it through the tense early days of their condition, the outlook gets better. Some children may have long-term complications, but theyll still live long and full lives. Continuing advances in medicine improve the odds of both short-term survival and long-term health. […] The longer-term prognosis depends on several factors, including: If your baby was born prematurely, How severe your babys condition is at birth, The size of the hernia, What organs are involved, Other health conditions. […] Babies who require breathing or feeding support for longer have a higher risk of ongoing complications, including chronic lung disease, growth failure, hearing loss and developmental delays. Your childs provider will monitor them closely throughout their early life. […] Though this is a difficult time, you and your newborn will have a team of healthcare providers looking out for you. Theyll support you at every step from the moment of diagnosis through your childs early life.
#33 Congenital Diaphragmatic Hernia (CDH)
https://fetus.ucsf.edu/cdh/
On the other end of the spectrum, babies with severe CDH and very small lungs can have difficult challenges after birth, and some will not survive. […] Even when the CDH is severe, greater than 70% of affected babies can be saved with intensive support. However, there can be long term health issues related to breathing, feeding, growth and development. […] When CDH is the only problem, we have learned that severity and, thus, outcome is determined by two factors: 1) liver position, and 2) lung-to-head ratio or LHR. […] Fetuses who do not have liver herniated into the chest (liver down) usually have a less severe form of CDH and therefore a better prognosis. […] Fetuses with liver herniated into the chest and a lung-head ratio less than 1.0 have a more severe form of CDH. We can predict that they will have a very difficult time after birth.
#34 Congenital Diaphragmatic Hernia (CDH)
https://fetus.ucsf.edu/cdh/
Although you can be given a prognosis on the severity of the CDH, until the baby for sure is born, there isn’t a test to perform during pregnancy that can predict lung function. How your baby’s lung’s function will only be determined after they are born. […] The long-term outcome depends on the severity of the CDH and the need for very intensive support. […] Babies who experienced more severe CDH may face challenges which can include mild learning problems, breathing illnesses, hearing loss, and growth problems.
#35 Congenital Diaphragmatic Hernia | IntechOpen
https://www.intechopen.com/chapters/56039
The presence of liver in the chest on prenatal ultrasound is a poor prognosticator for survival. […] The odds of survival with LHR 1.0 was 0.14 (CI: 0.10-0.27) and with liver herniation on ultrasound it was 0.21 (CI: 0.13-0.35). […] The SNAP 2 score is considered independent of initial ventilation strategy. […] The introduction of gentle ventilation strategies, permissive hypercapnia and spontaneous respiration had a more significant effect on survival than HFOV or ECMO as rescue therapy. […] The overall survival for CDH infants was 67% and for those requiring ECMO, it was 61%. […] The mortality in ECMO-treated patients was 50% in both time periods. […] With advances in CDH management, the overall survival has improved and has been reported between 70 and 90% in non-ECMO infants and up to 50% in infants who undergo ECMO.
#36 Prediction of survival in infants with congenital diaphragmatic hernia and the response to inhaled nitric oxide
https://pmc.ncbi.nlm.nih.gov/articles/PMC9508000/
Certain infants with CDH responded to iNO and those with a greater response were more likely to survive. […] The best oxygenation index on day 1 (BOId1) after birth was associated with better survival in CDH infants. […] Our study showed that the HOI on day 1 was associated with survival after adjusting for gestational age, Apgar score at 5 min and the O/E LHR. […] Our study showed that CDH infants who required HFO preoperatively had almost no ventilator free days within the first 28 days after birth. […] The response to inhaled nitric oxide was variable in infants with CDH in our study, but infants who responded to iNO were more likely to survive. […] In conclusion, the highest oxygenation index on day 1 of life was a better predictor of survival than gestational age and the Apgar score at 5 min. Inhaled nitric oxide was associated with improved oxygenation in certain infants with CDH and the response to nitric oxide was better in those infants who survived.
#37 Prediction of survival in infants with congenital diaphragmatic hernia and the response to inhaled nitric oxide
https://pmc.ncbi.nlm.nih.gov/articles/PMC9508000/
The use of inhaled nitric oxide (iNO) in treating pulmonary hypertension in infants with congenital diaphragmatic hernia (CDH) is controversial. […] Factors affecting survival were determined. […] The CDH infants who died had a lower median observed to expected lung to head ratio (O/E LHR) (p0.001) and a higher median highest OI on day 1 (HOId1) (p0.001). HOId1 predicted survival after adjusting for gestational age, Apgar score at 5 min and O/E LHR (odds ratio 0.948 (95% confidence intervals 0.9130.983)). […] The highest OI on day 1 predicted survival. iNO improved oxygenation in certain CDH infants and a positive response was more likely in those who survived. […] We have examined predictors of survival in CDH infants including the response to iNO and demonstrated that the highest oxygenation index on day 1 predicted survival (AUCROC =0.908).
#38
https://link.springer.com/article/10.1007/s00431-022-04568-8
The highest OI on day 1 predicted survival. […] The percentage change in the PaO2/FiO2 ratio post iNO initiation was higher in infants who survived (69.4%) compared to infants who died (10.2%), p=0.018. […] We have demonstrated that survival in infants with CDH is related to highest OI on day 1 after birth. Furthermore, we highlight that the response to iNO can predict survival in CDH infants. […] Our study showed that the HOI on day 1 was associated with survival after adjusting for gestational age, Apgar score at 5 min and the O/E LHR. […] The response to inhaled nitric oxide was variable in infants with CDH in our study, but infants who responded to iNO were more likely to survive. […] In conclusion, the highest oxygenation index on day 1 of life was a better predictor of survival than gestational age and the Apgar score at 5 min. Inhaled nitric oxide was associated with improved oxygenation in certain infants with CDH and the response to nitric oxide was better in those infants who survived.
#39 Prediction of survival in infants with congenital diaphragmatic hernia and the response to inhaled nitric oxide
https://pmc.ncbi.nlm.nih.gov/articles/PMC9508000/
Certain infants with CDH responded to iNO and those with a greater response were more likely to survive. […] The best oxygenation index on day 1 (BOId1) after birth was associated with better survival in CDH infants. […] Our study showed that the HOI on day 1 was associated with survival after adjusting for gestational age, Apgar score at 5 min and the O/E LHR. […] Our study showed that CDH infants who required HFO preoperatively had almost no ventilator free days within the first 28 days after birth. […] The response to inhaled nitric oxide was variable in infants with CDH in our study, but infants who responded to iNO were more likely to survive. […] In conclusion, the highest oxygenation index on day 1 of life was a better predictor of survival than gestational age and the Apgar score at 5 min. Inhaled nitric oxide was associated with improved oxygenation in certain infants with CDH and the response to nitric oxide was better in those infants who survived.
#40
https://link.springer.com/article/10.1007/s00431-022-04568-8
The highest OI on day 1 predicted survival. […] The percentage change in the PaO2/FiO2 ratio post iNO initiation was higher in infants who survived (69.4%) compared to infants who died (10.2%), p=0.018. […] We have demonstrated that survival in infants with CDH is related to highest OI on day 1 after birth. Furthermore, we highlight that the response to iNO can predict survival in CDH infants. […] Our study showed that the HOI on day 1 was associated with survival after adjusting for gestational age, Apgar score at 5 min and the O/E LHR. […] The response to inhaled nitric oxide was variable in infants with CDH in our study, but infants who responded to iNO were more likely to survive. […] In conclusion, the highest oxygenation index on day 1 of life was a better predictor of survival than gestational age and the Apgar score at 5 min. Inhaled nitric oxide was associated with improved oxygenation in certain infants with CDH and the response to nitric oxide was better in those infants who survived.
#41 Development of a prediction nomogram for 1-month mortality in neonates with congenital diaphragmatic hernia | BMC Surgery | Full Text
https://bmcsurg.biomedcentral.com/articles/10.1186/s12893-024-02479-z
Although many prognostic factors in neonates with congenital diaphragmatic hernia (CDH) have been described, no consensus thus far has been reached on which and how many factors are involved. […] The aim of this study is to analyze the association of multiple prenatal and postnatal factors with 1-month mortality of neonates with CDH and to construct a nomogram prediction model based on significant factors. […] After graded multivariable adjustment, six factors were found to be independently and consistently associated with the significant risk of 1-month mortality in neonates with CDH, including gestational age of prenatal diagnosis, observed-to-expected lung-to-head ratio, liver herniation, severity of pulmonary hypertension, diameter of defect, and oxygen index. […] Our findings provide evidence for the association of six preoperational and intraoperative factors with the risk of 1-month mortality in neonates with CDH, and this association was reinforced in a nomogram model.
#42 Development of a prediction nomogram for 1-month mortality in neonates with congenital diaphragmatic hernia | BMC Surgery | Full Text
https://bmcsurg.biomedcentral.com/articles/10.1186/s12893-024-02479-z
Despite significant improvement in neonatal surgical techniques and intensive care, the mortality rate of neonates diagnosed with CDH still remains very high. […] The key findings of this study are the identification of six independent factors, including GA of prenatal diagnosis, o/e LHR, liver herniation, severity of PH, diameter of defect, and OI in predicting the significant risk of 1-month mortality in neonates with CDH on the first day of life. […] Taken together, our findings provide evidence for the association of six prenatal and early postnatal factors with the significant risk of 1-month mortality in neonates with CDH, and this association was reinforced in a nomogram model.
#43 Development of a prediction nomogram for 1-month mortality in neonates with congenital diaphragmatic hernia | BMC Surgery | Full Text
https://bmcsurg.biomedcentral.com/articles/10.1186/s12893-024-02479-z
Despite significant improvement in neonatal surgical techniques and intensive care, the mortality rate of neonates diagnosed with CDH still remains very high. […] The key findings of this study are the identification of six independent factors, including GA of prenatal diagnosis, o/e LHR, liver herniation, severity of PH, diameter of defect, and OI in predicting the significant risk of 1-month mortality in neonates with CDH on the first day of life. […] Taken together, our findings provide evidence for the association of six prenatal and early postnatal factors with the significant risk of 1-month mortality in neonates with CDH, and this association was reinforced in a nomogram model.
#44 Congenital Diaphragmatic Hernia (CDH)
https://fetus.ucsf.edu/cdh/
There is a wide range of severity and outcomes for CDH. In the best cases, some infants do very well with treatment after birth, surgery and care in an intensive care nursery. In the most severe cases, some will not survive no matter how hard we try. And in the middle, some will live normally while others will have a difficult time and may have some handicaps ranging from mild learning problems to breathing and growth problems. How the baby does after birth is determined by how well the lung grows before birth and its function. […] Fetuses on the best end of the spectrum have an excellent chance to lead a perfectly normal life. They do not require special prenatal management in terms of the timing or type of delivery, but should be delivered in a perinatal center with a Level III intensive care nursery with neonatal and pediatric surgery support.
#45 Congenital Diaphragmatic Hernia (CDH)
https://fetus.ucsf.edu/cdh/
There is a wide range of severity and outcomes for CDH. In the best cases, some infants do very well with treatment after birth, surgery and care in an intensive care nursery. In the most severe cases, some will not survive no matter how hard we try. And in the middle, some will live normally while others will have a difficult time and may have some handicaps ranging from mild learning problems to breathing and growth problems. How the baby does after birth is determined by how well the lung grows before birth and its function. […] Fetuses on the best end of the spectrum have an excellent chance to lead a perfectly normal life. They do not require special prenatal management in terms of the timing or type of delivery, but should be delivered in a perinatal center with a Level III intensive care nursery with neonatal and pediatric surgery support.
#46 Congenital Diaphragmatic Hernia (CDH)
https://fetus.ucsf.edu/cdh/
On the other end of the spectrum, babies with severe CDH and very small lungs can have difficult challenges after birth, and some will not survive. […] Even when the CDH is severe, greater than 70% of affected babies can be saved with intensive support. However, there can be long term health issues related to breathing, feeding, growth and development. […] When CDH is the only problem, we have learned that severity and, thus, outcome is determined by two factors: 1) liver position, and 2) lung-to-head ratio or LHR. […] Fetuses who do not have liver herniated into the chest (liver down) usually have a less severe form of CDH and therefore a better prognosis. […] Fetuses with liver herniated into the chest and a lung-head ratio less than 1.0 have a more severe form of CDH. We can predict that they will have a very difficult time after birth.