Zespół hipoplastycznego lewego serca – Rokowania, prognozy i postęp choroby

Zespół hipoplastycznego lewego serca
Rokowania, prognozy i postęp choroby

Zespół hipoplastycznego lewego serca (HLHS) charakteryzuje się niedorozwojem lewej strony serca, obejmującym lewy przedsionek, zastawkę mitralną, lewą komorę, zastawkę aortalną oraz łuk aorty. Przed latami 80. XX wieku śmiertelność niemowląt z HLHS wynosiła około 95% w pierwszym miesiącu życia. W ostatnich dwóch dekadach wprowadzono wieloetapowe leczenie paliatywne (procedura Norwooda), przeszczepy serca oraz opiekę paliatywną, co znacząco poprawiło rokowanie. W badaniach populacyjnych z Anglii i Walii wskaźniki przeżywalności dla HLHS wynosiły odpowiednio: 84,4% w 1 tygodniu, 76,2% w 1 miesiącu, 63,5% w 1 roku, 58,6% w 5 lat, 54,6% w 10 lat oraz 32,6% w 15 lat. Dla hipoplazji lewej komory z dodatkowymi wadami serca przeżywalność była nieco wyższa, np. 90,9% w 1 tygodniu i 40,3% w 15 lat. Czynniki pogarszające rokowanie to niska masa urodzeniowa (p=0,005), wcześniactwo (p=0,007), płeć żeńska (p=0,01), obecność pozasercowych wad wrodzonych oraz ciężka obstrukcja powrotu żylnego płucnego.

Zespół hipoplastycznego lewego serca – Prognoza i rokowanie

Współczesne wskaźniki przeżywalności
Czynniki wpływające na rokowanie
Metody leczenia a prognoza
Długoterminowe rokowanie i jakość życia
Nowoczesne metody przewidywania rokowania
Podsumowanie tendencji w rokowaniu
Kolejne rozdziały

Zespół hipoplastycznego lewego serca – Prognoza i rokowanie

Zespół hipoplastycznego lewego serca (HLHS) jest jedną z najcięższych wrodzonych wad serca, charakteryzującą się niedorozwojem lewej strony serca z różnym stopniem hipoplazji lewego przedsionka, zastawki mitralnej, lewej komory, zastawki aortalnej i łuku aorty. Przed latami 80. XX wieku, rokowanie dla niemowląt z HLHS było jednoznacznie niepomyślne – śmiertelność w pierwszym miesiącu życia sięgała 95%. Nieleczona wada prowadziła do pewnej śmierci, najczęściej w ciągu pierwszych dwóch tygodni życia.¹²

W ciągu ostatnich dwóch dekad nastąpił znaczący postęp w leczeniu dzieci urodzonych z HLHS, co doprowadziło do istotnej poprawy rokowania. Obecnie istnieją różne opcje terapeutyczne, w tym leczenie paliatywne wieloetapowe, przeszczep serca lub opieka paliatywna bez interwencji chirurgicznej.³⁴

Współczesne wskaźniki przeżywalności

Badania populacyjne prowadzone w Anglii i Walii na 343 przypadkach urodzeń dzieci z HLHS lub hipoplazją lewej komory z towarzyszącymi innymi wadami serca w latach 1998-2012 pokazują znaczną poprawę rokowania. Wskaźniki przeżywalności metodą Kaplana-Meiera dla HLHS wynosiły:⁵⁶

84,4% w wieku 1 tygodnia
76,2% w wieku 1 miesiąca
63,5% w wieku 1 roku
58,6% w wieku 5 lat
54,6% w wieku 10 lat
32,6% w wieku 15 lat

⁷

Dla przypadków hipoplazji lewej komory współwystępującej z dodatkowymi wadami serca, wskaźniki przeżywalności były nieco wyższe:⁸

90,9% w wieku 1 tygodnia
84,9% w wieku 1 miesiąca
73,7% w wieku 1 roku
67,7% w wieku 5 lat
59,2% w wieku 10 lat
40,3% w wieku 15 lat

⁹

Inne badania podają, że aktuarialne przeżycie dla wszystkich niemowląt poddanych etapowym procedurom paliatywnym wynosi od 63% do 80% w wieku jednego roku i od 58% do 72% w wieku pięciu lat.¹⁰ Obecnie wskaźniki przeżywalności po zabiegu paliatywnym przekraczają 70% w ciągu 5 lat.¹¹

Czynniki wpływające na rokowanie

Zidentyfikowano szereg czynników, które wpływają na rokowanie u pacjentów z HLHS:¹²¹³

Wyjściowy stan niemowlęcia – najważniejszy czynnik prognostyczny
Obecność pozasercowych wad wrodzonych – pogarsza rokowanie
Niska masa urodzeniowa – związana z gorszymi wynikami (p = 0,005)
Wcześniactwo – negatywny wpływ na przeżywalność (p = 0,007)
Płeć żeńska – związana z wyższą śmiertelnością (p = 0,01)
Ciężka obstrukcja powrotu żylnego płucnego – pogarsza rokowanie

¹⁴¹⁵

Badania potwierdzają, że niemowlęta o prawidłowej masie urodzeniowej, które nie urodziły się przedwcześnie, osiągają lepsze wyniki niż niemowlęta o niższej masie urodzeniowej.¹⁶

Metody leczenia a prognoza

Leczenie paliatywne wieloetapowe

Dzięki rozwojowi etapowego leczenia operacyjnego (procedura Norwooda), rokowanie dla niemowląt z HLHS uległo dramatycznej poprawie. Zabieg ten obejmuje trzy etapy, które mają na celu przekształcenie układu krążenia w funkcjonalny układ z pojedynczą komorą.¹⁷

Obawy dotyczące długoterminowych wyników u niemowląt z HLHS obejmują odpowiednie zachowanie funkcji prawej komory i zastawek, gdy są one narażone na ciśnienie systemowe. Potencjalne ograniczenie stanowi fizjologia pojedynczej komory osiągnięta po wieloetapowej rekonstrukcji.¹⁸

Przeszczep serca

Przeszczep serca oferuje potencjał normalnej fizjologii układu sercowo-naczyniowego, przezwyciężając ograniczenia fizjologii pojedynczej komory. Główne ograniczenia przeszczepu to niedobór dawców i problemy z dopasowaniem rozmiaru od dawcy do biorcy.¹⁹²⁰

Około 65% wszystkich niemowląt umieszczonych na liście oczekujących na przeszczep żyje po 5 latach. Spośród tych, które otrzymały przeszczep, ponad trzy czwarte przeżywa do 5 lat.²¹

Długoterminowe rokowanie i jakość życia

Dzieci po leczeniu HLHS wymagają dożywotnich wizyt kontrolnych co najmniej raz w roku u kardiologa. Wizyty te zapewniają, że serce, płuca i inne narządy nadal funkcjonują prawidłowo. Gdy dziecko staje się dorosłe, przechodzi pod opiekę specjalisty w zakresie wrodzonych wad serca u dorosłych.²²

Długoterminowe rokowanie i jakość życia po operacji pozostają nie w pełni zdefiniowane i wymagają dalszych badań. Jedno z badań wykazało, że większość niemowląt, które przeżyły pierwszy rok, nadal żyła w wieku 18 lat.²³²⁴²⁵

Potencjalne długoterminowe powikłania HLHS mogą obejmować:²⁶

Zmniejszoną wydolność wysiłkową
Problemy z rozwojem
Konieczność przeszczepu serca w późniejszym życiu

²⁷

Nowoczesne metody przewidywania rokowania

Obecnie rozwijane są metody oparte na uczeniu maszynowym (ML) do indywidualnego przewidywania przeżycia bez przeszczepu (TFS) u pacjentów z HLHS. Modele te wykorzystują dane dostępne przed pierwszym etapem leczenia paliatywnego (S1P) i wykazują obiecującą skuteczność w prognozowaniu 5-letniego przeżycia.²⁸

Modele trenowane z cechami z okresu przed S1P, operacji S1P i hospitalizacji S1P wykazywały zależne od czasu pola pod krzywymi (td-AUC) powyżej 0,70 przez 5 lat po S1P. Model uwzględniający cechy z całej hospitalizacji S1P wykazał szczególnie solidną wydajność (td-AUC 0,838; 95% CI 0,836-0,840).²⁹³⁰

Wykorzystując jedynie cechy dostępne w fazie przed S1P, opracowano model prognozujący sześcioletnie TFS z zadowalającą wydajnością kliniczną, z indeksem C wynoszącym 0,692 (95% CI 0,690-0,695).³¹

Podsumowanie tendencji w rokowaniu

Postęp w leczeniu HLHS w ostatnich dziesięcioleciach znacząco poprawił rokowanie. Największe ryzyko śmiertelności u pacjentów z HLHS lub hipoplazją lewej komory z dodatkowymi wadami serca występuje w pierwszym tygodniu życia, chociaż przeżywalność nadal maleje w ciągu 15 lat obserwacji.³²

Wskaźniki przeżycia były wyższe w późniejszej kohorcie (2006-2012 vs 1998-2005), choć w przypadkach HLHS mogły wzrosnąć tylko do 5 roku życia. Badania populacyjne pokazują, że rokowanie związane z HLHS w XXI wieku przewyższa dane z wielu wcześniejszych badań populacyjnych, prawdopodobnie dzięki ulepszeniom w technologiach intensywnej terapii i postępom w technikach chirurgicznych.³³³⁴

Mimo znaczących postępów, wciąż potrzebne są dalsze badania, szczególnie w zakresie naturalnej historii HLHS, diagnostyki prenatalnej, strategii operacyjnych i długoterminowych wyników, aby określić optymalne postępowanie dla poszczególnych niemowląt z tą wadą.³⁵

Kolejne rozdziały

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Materiały źródłowe

#1 Hypoplastic left heart syndrome: Prognosis and management options
https://pmc.ncbi.nlm.nih.gov/articles/PMC2817797/
Few congenital heart malformations have raised as many surgical, ethical, social and economic issues as the therapy for infants diagnosed with hypoplastic left heart syndrome. […] In the past two decades, palliative surgery and cardiac transplantation have become management options, in addition to comfort care for the infant. […] Surgical therapy provides hope for the survival of these infants, but their long term outcomes are not well defined. […] More investigations to determine the long term outcome following palliative surgery and transplantation are needed before they become the standards of care. […] Before the 1980s, this complex malformation was associated with 95% mortality within the first month of life. […] Untreated, the prognosis for the infant is certain death. […] In the past two decades, there has been remarkable progress in the management of children born with HLHS.
#2 Azthena logo with the word Azthena
https://www.news-medical.net/health/Prognosis-for-Hypoplastic-Left-Heart-Syndrome.aspx
Hypoplastic left heart syndrome (HLHS) is a severe form of congenital heart disease which is always fatal without treatment. […] The outlook for HLHS was uniformly dismal before the 1980s. Most infants succumbed to the lethal effects of a minimal systemic circulation, mostly within the first two weeks of life. […] With the development of palliation staged surgeries (the Norwood surgery), the prognosis has improved dramatically for these infants. […] Survival rates following this staged procedure are above 70% at 5 years at present. […] The most important factor in predicting the ultimate outcome is the initial status of the infant. […] 65% of all babies who are put on a transplant list are alive at 5 years. Of those who receive a transplant, more than three out of four survived at 5 years.
#3 Hypoplastic left heart syndrome: Prognosis and management options
https://pmc.ncbi.nlm.nih.gov/articles/PMC2817797/
Few congenital heart malformations have raised as many surgical, ethical, social and economic issues as the therapy for infants diagnosed with hypoplastic left heart syndrome. […] In the past two decades, palliative surgery and cardiac transplantation have become management options, in addition to comfort care for the infant. […] Surgical therapy provides hope for the survival of these infants, but their long term outcomes are not well defined. […] More investigations to determine the long term outcome following palliative surgery and transplantation are needed before they become the standards of care. […] Before the 1980s, this complex malformation was associated with 95% mortality within the first month of life. […] Untreated, the prognosis for the infant is certain death. […] In the past two decades, there has been remarkable progress in the management of children born with HLHS.
#4 Hypoplastic left heart syndrome: Prognosis and management options
https://pmc.ncbi.nlm.nih.gov/articles/PMC2817797/
The option of palliative or comfort care without aggressive surgical intervention remains, either at home or in hospital. […] Parents and physicians face difficult decisions regarding the appropriate therapeutic intervention for individual infants with HLHS. […] Survival has been shown to be worse in patients with significant noncardiac congenital anomalies, low birth weight and severe obstruction to pulmonary venous return. […] Actuarial survival for all infants with combined staged procedures has been reported to be 63% to 80% at one year of age and 58% to 72% at five years of age. […] Concerns about the long term outcome of infants with HLHS include the adequate preservation of the right ventricle and valvular function when exposed to systemic pressure. […] Cardiac transplantation offers the potential for a normal cardiovascular physiology over the limitations of the single ventricle physiology achieved after multi-stage reconstruction.
#5 Frontiers | The Improved Prognosis of Hypoplastic Left Heart: A Population-Based Register Study of 343 Cases in England and Wales
https://www.frontiersin.org/journals/pediatrics/articles/10.3389/fped.2021.635776/full
The Improved Prognosis of Hypoplastic Left Heart: A Population-Based Register Study of 343 Cases in England and Wales […] Background: Hypoplastic Left Heart Syndrome (HLHS) is a severe congenital heart defect (CHD) characterised by the underdevelopment of the left side of the heart with varying levels of hypoplasia of the left atrium, mitral valve, left ventricle, aortic valve and aortic arch. In the UK, age 12 survival for cases born between 1991 and 1993 was 21%. UK survival estimates corresponding to cases born between 2000 and 2015 were improved at 56%, but survival was examined up to age five only. Contemporary long-term survival estimates play a crucial role in counselling parents following diagnosis. The aim of this study was to report survival estimates up to age 15 for children born with HLHS or hypoplastic left ventricle with additional CHD in England and Wales between 1998 and 2012.
#6 Frontiers | The Improved Prognosis of Hypoplastic Left Heart: A Population-Based Register Study of 343 Cases in England and Wales
https://www.frontiersin.org/journals/pediatrics/articles/10.3389/fped.2021.635776/full
Results: There were 244 cases of HLHS and 99 cases of hypoplastic left ventricle co-occurring with other CHD, with traced survival status. Kaplan-Meier survival estimates for HLHS were 84.4% at age 1 week, 76.2% at 1 month, 63.5% at age 1 year, 58.6% at age 5 years, 54.6% at age 10 years, and 32.6% to age 15 years. The Kaplan-Meier survival estimates for cases of hypoplastic left ventricle co-occurring with additional CHD were 90.9% at age 1 week, 84.9% at 1 month, 73.7% at age 1 year, 67.7% to age 5 years, 59.2% to age 10 years, and 40.3% to age 15 years. Preterm birth (p = 0.007), low birth weight (p = 0.005), and female sex (p = 0.01) were associated with mortality. […] Conclusions: We have shown that prognosis associated with HLHS in the twenty first century exceeds that of many previous population-based studies, likely due to improvements in intensive care technologies and advances in surgical techniques over the last few decades.
#7 Frontiers | The Improved Prognosis of Hypoplastic Left Heart: A Population-Based Register Study of 343 Cases in England and Wales
https://www.frontiersin.org/journals/pediatrics/articles/10.3389/fped.2021.635776/full
Results: There were 244 cases of HLHS and 99 cases of hypoplastic left ventricle co-occurring with other CHD, with traced survival status. Kaplan-Meier survival estimates for HLHS were 84.4% at age 1 week, 76.2% at 1 month, 63.5% at age 1 year, 58.6% at age 5 years, 54.6% at age 10 years, and 32.6% to age 15 years. The Kaplan-Meier survival estimates for cases of hypoplastic left ventricle co-occurring with additional CHD were 90.9% at age 1 week, 84.9% at 1 month, 73.7% at age 1 year, 67.7% to age 5 years, 59.2% to age 10 years, and 40.3% to age 15 years. Preterm birth (p = 0.007), low birth weight (p = 0.005), and female sex (p = 0.01) were associated with mortality. […] Conclusions: We have shown that prognosis associated with HLHS in the twenty first century exceeds that of many previous population-based studies, likely due to improvements in intensive care technologies and advances in surgical techniques over the last few decades.
#8 Frontiers | The Improved Prognosis of Hypoplastic Left Heart: A Population-Based Register Study of 343 Cases in England and Wales
https://www.frontiersin.org/journals/pediatrics/articles/10.3389/fped.2021.635776/full
Results: There were 244 cases of HLHS and 99 cases of hypoplastic left ventricle co-occurring with other CHD, with traced survival status. Kaplan-Meier survival estimates for HLHS were 84.4% at age 1 week, 76.2% at 1 month, 63.5% at age 1 year, 58.6% at age 5 years, 54.6% at age 10 years, and 32.6% to age 15 years. The Kaplan-Meier survival estimates for cases of hypoplastic left ventricle co-occurring with additional CHD were 90.9% at age 1 week, 84.9% at 1 month, 73.7% at age 1 year, 67.7% to age 5 years, 59.2% to age 10 years, and 40.3% to age 15 years. Preterm birth (p = 0.007), low birth weight (p = 0.005), and female sex (p = 0.01) were associated with mortality. […] Conclusions: We have shown that prognosis associated with HLHS in the twenty first century exceeds that of many previous population-based studies, likely due to improvements in intensive care technologies and advances in surgical techniques over the last few decades.
#9 Frontiers | The Improved Prognosis of Hypoplastic Left Heart: A Population-Based Register Study of 343 Cases in England and Wales
https://www.frontiersin.org/journals/pediatrics/articles/10.3389/fped.2021.635776/full
Results: There were 244 cases of HLHS and 99 cases of hypoplastic left ventricle co-occurring with other CHD, with traced survival status. Kaplan-Meier survival estimates for HLHS were 84.4% at age 1 week, 76.2% at 1 month, 63.5% at age 1 year, 58.6% at age 5 years, 54.6% at age 10 years, and 32.6% to age 15 years. The Kaplan-Meier survival estimates for cases of hypoplastic left ventricle co-occurring with additional CHD were 90.9% at age 1 week, 84.9% at 1 month, 73.7% at age 1 year, 67.7% to age 5 years, 59.2% to age 10 years, and 40.3% to age 15 years. Preterm birth (p = 0.007), low birth weight (p = 0.005), and female sex (p = 0.01) were associated with mortality. […] Conclusions: We have shown that prognosis associated with HLHS in the twenty first century exceeds that of many previous population-based studies, likely due to improvements in intensive care technologies and advances in surgical techniques over the last few decades.
#10 Hypoplastic left heart syndrome: Prognosis and management options
https://pmc.ncbi.nlm.nih.gov/articles/PMC2817797/
The option of palliative or comfort care without aggressive surgical intervention remains, either at home or in hospital. […] Parents and physicians face difficult decisions regarding the appropriate therapeutic intervention for individual infants with HLHS. […] Survival has been shown to be worse in patients with significant noncardiac congenital anomalies, low birth weight and severe obstruction to pulmonary venous return. […] Actuarial survival for all infants with combined staged procedures has been reported to be 63% to 80% at one year of age and 58% to 72% at five years of age. […] Concerns about the long term outcome of infants with HLHS include the adequate preservation of the right ventricle and valvular function when exposed to systemic pressure. […] Cardiac transplantation offers the potential for a normal cardiovascular physiology over the limitations of the single ventricle physiology achieved after multi-stage reconstruction.
#11 Azthena logo with the word Azthena
https://www.news-medical.net/health/Prognosis-for-Hypoplastic-Left-Heart-Syndrome.aspx
Hypoplastic left heart syndrome (HLHS) is a severe form of congenital heart disease which is always fatal without treatment. […] The outlook for HLHS was uniformly dismal before the 1980s. Most infants succumbed to the lethal effects of a minimal systemic circulation, mostly within the first two weeks of life. […] With the development of palliation staged surgeries (the Norwood surgery), the prognosis has improved dramatically for these infants. […] Survival rates following this staged procedure are above 70% at 5 years at present. […] The most important factor in predicting the ultimate outcome is the initial status of the infant. […] 65% of all babies who are put on a transplant list are alive at 5 years. Of those who receive a transplant, more than three out of four survived at 5 years.
#12 Hypoplastic left heart syndrome: Prognosis and management options
https://pmc.ncbi.nlm.nih.gov/articles/PMC2817797/
The option of palliative or comfort care without aggressive surgical intervention remains, either at home or in hospital. […] Parents and physicians face difficult decisions regarding the appropriate therapeutic intervention for individual infants with HLHS. […] Survival has been shown to be worse in patients with significant noncardiac congenital anomalies, low birth weight and severe obstruction to pulmonary venous return. […] Actuarial survival for all infants with combined staged procedures has been reported to be 63% to 80% at one year of age and 58% to 72% at five years of age. […] Concerns about the long term outcome of infants with HLHS include the adequate preservation of the right ventricle and valvular function when exposed to systemic pressure. […] Cardiac transplantation offers the potential for a normal cardiovascular physiology over the limitations of the single ventricle physiology achieved after multi-stage reconstruction.
#13 Azthena logo with the word Azthena
https://www.news-medical.net/health/Prognosis-for-Hypoplastic-Left-Heart-Syndrome.aspx
Hypoplastic left heart syndrome (HLHS) is a severe form of congenital heart disease which is always fatal without treatment. […] The outlook for HLHS was uniformly dismal before the 1980s. Most infants succumbed to the lethal effects of a minimal systemic circulation, mostly within the first two weeks of life. […] With the development of palliation staged surgeries (the Norwood surgery), the prognosis has improved dramatically for these infants. […] Survival rates following this staged procedure are above 70% at 5 years at present. […] The most important factor in predicting the ultimate outcome is the initial status of the infant. […] 65% of all babies who are put on a transplant list are alive at 5 years. Of those who receive a transplant, more than three out of four survived at 5 years.
#14 Hypoplastic left heart syndrome: Prognosis and management options
https://pmc.ncbi.nlm.nih.gov/articles/PMC2817797/
The option of palliative or comfort care without aggressive surgical intervention remains, either at home or in hospital. […] Parents and physicians face difficult decisions regarding the appropriate therapeutic intervention for individual infants with HLHS. […] Survival has been shown to be worse in patients with significant noncardiac congenital anomalies, low birth weight and severe obstruction to pulmonary venous return. […] Actuarial survival for all infants with combined staged procedures has been reported to be 63% to 80% at one year of age and 58% to 72% at five years of age. […] Concerns about the long term outcome of infants with HLHS include the adequate preservation of the right ventricle and valvular function when exposed to systemic pressure. […] Cardiac transplantation offers the potential for a normal cardiovascular physiology over the limitations of the single ventricle physiology achieved after multi-stage reconstruction.
#15 Frontiers | The Improved Prognosis of Hypoplastic Left Heart: A Population-Based Register Study of 343 Cases in England and Wales
https://www.frontiersin.org/journals/pediatrics/articles/10.3389/fped.2021.635776/full
Results: There were 244 cases of HLHS and 99 cases of hypoplastic left ventricle co-occurring with other CHD, with traced survival status. Kaplan-Meier survival estimates for HLHS were 84.4% at age 1 week, 76.2% at 1 month, 63.5% at age 1 year, 58.6% at age 5 years, 54.6% at age 10 years, and 32.6% to age 15 years. The Kaplan-Meier survival estimates for cases of hypoplastic left ventricle co-occurring with additional CHD were 90.9% at age 1 week, 84.9% at 1 month, 73.7% at age 1 year, 67.7% to age 5 years, 59.2% to age 10 years, and 40.3% to age 15 years. Preterm birth (p = 0.007), low birth weight (p = 0.005), and female sex (p = 0.01) were associated with mortality. […] Conclusions: We have shown that prognosis associated with HLHS in the twenty first century exceeds that of many previous population-based studies, likely due to improvements in intensive care technologies and advances in surgical techniques over the last few decades.
#16 Hypoplastic Left Heart Syndrome (HLHS): Causes, Symptoms & Treatment
https://my.clevelandclinic.org/health/diseases/12214-hypoplastic-left-heart-syndrome-hlhs
After treatment for hypoplastic left heart syndrome, your child will need lifelong follow-up visits at least once a year with a cardiologist (heart doctor). These visits ensure that their heart, lungs and other organs continue to work properly. When your child becomes an adult, theyll transition to care with a specialist in adult congenital heart disease. […] Without treatment, hypoplastic left heart syndrome is fatal days or weeks after birth. […] With treatment, the prognosis depends on the complexity of your childs heart deformity. Ask your babys healthcare provider about the risks associated with each surgery. […] About 20% to 60% of babies with hypoplastic left heart syndrome survive their first year of life. After that, the survival rate for the next five, 10 and 15 years is about 40%. Babies who have a normal birth weight and arent born prematurely do better than babies with lower birth weights. One study found that most babies who survived their first year were still alive at age 18.
#17 Azthena logo with the word Azthena
https://www.news-medical.net/health/Prognosis-for-Hypoplastic-Left-Heart-Syndrome.aspx
Hypoplastic left heart syndrome (HLHS) is a severe form of congenital heart disease which is always fatal without treatment. […] The outlook for HLHS was uniformly dismal before the 1980s. Most infants succumbed to the lethal effects of a minimal systemic circulation, mostly within the first two weeks of life. […] With the development of palliation staged surgeries (the Norwood surgery), the prognosis has improved dramatically for these infants. […] Survival rates following this staged procedure are above 70% at 5 years at present. […] The most important factor in predicting the ultimate outcome is the initial status of the infant. […] 65% of all babies who are put on a transplant list are alive at 5 years. Of those who receive a transplant, more than three out of four survived at 5 years.
#18 Hypoplastic left heart syndrome: Prognosis and management options
https://pmc.ncbi.nlm.nih.gov/articles/PMC2817797/
The option of palliative or comfort care without aggressive surgical intervention remains, either at home or in hospital. […] Parents and physicians face difficult decisions regarding the appropriate therapeutic intervention for individual infants with HLHS. […] Survival has been shown to be worse in patients with significant noncardiac congenital anomalies, low birth weight and severe obstruction to pulmonary venous return. […] Actuarial survival for all infants with combined staged procedures has been reported to be 63% to 80% at one year of age and 58% to 72% at five years of age. […] Concerns about the long term outcome of infants with HLHS include the adequate preservation of the right ventricle and valvular function when exposed to systemic pressure. […] Cardiac transplantation offers the potential for a normal cardiovascular physiology over the limitations of the single ventricle physiology achieved after multi-stage reconstruction.
#19 Hypoplastic left heart syndrome: Prognosis and management options
https://pmc.ncbi.nlm.nih.gov/articles/PMC2817797/
The option of palliative or comfort care without aggressive surgical intervention remains, either at home or in hospital. […] Parents and physicians face difficult decisions regarding the appropriate therapeutic intervention for individual infants with HLHS. […] Survival has been shown to be worse in patients with significant noncardiac congenital anomalies, low birth weight and severe obstruction to pulmonary venous return. […] Actuarial survival for all infants with combined staged procedures has been reported to be 63% to 80% at one year of age and 58% to 72% at five years of age. […] Concerns about the long term outcome of infants with HLHS include the adequate preservation of the right ventricle and valvular function when exposed to systemic pressure. […] Cardiac transplantation offers the potential for a normal cardiovascular physiology over the limitations of the single ventricle physiology achieved after multi-stage reconstruction.
#20 Hypoplastic left heart syndrome: Prognosis and management options
https://pmc.ncbi.nlm.nih.gov/articles/PMC2817797/
The major limitations of transplantation are a shortage of donors and size matching from donor to recipient. […] Although prognosis for HLHS is no longer one of certain death, many issues surrounding surgical treatment remain poorly defined. […] The long term outcomes and quality of life following surgery remain poorly defined. […] More information on the natural history of HLHS, its antenatal diagnosis, operative strategies and long term outcome are essential to determine the optimal management for individual infants with HLHS.
#21 Azthena logo with the word Azthena
https://www.news-medical.net/health/Prognosis-for-Hypoplastic-Left-Heart-Syndrome.aspx
Hypoplastic left heart syndrome (HLHS) is a severe form of congenital heart disease which is always fatal without treatment. […] The outlook for HLHS was uniformly dismal before the 1980s. Most infants succumbed to the lethal effects of a minimal systemic circulation, mostly within the first two weeks of life. […] With the development of palliation staged surgeries (the Norwood surgery), the prognosis has improved dramatically for these infants. […] Survival rates following this staged procedure are above 70% at 5 years at present. […] The most important factor in predicting the ultimate outcome is the initial status of the infant. […] 65% of all babies who are put on a transplant list are alive at 5 years. Of those who receive a transplant, more than three out of four survived at 5 years.
#22 Hypoplastic Left Heart Syndrome (HLHS): Causes, Symptoms & Treatment
https://my.clevelandclinic.org/health/diseases/12214-hypoplastic-left-heart-syndrome-hlhs
After treatment for hypoplastic left heart syndrome, your child will need lifelong follow-up visits at least once a year with a cardiologist (heart doctor). These visits ensure that their heart, lungs and other organs continue to work properly. When your child becomes an adult, theyll transition to care with a specialist in adult congenital heart disease. […] Without treatment, hypoplastic left heart syndrome is fatal days or weeks after birth. […] With treatment, the prognosis depends on the complexity of your childs heart deformity. Ask your babys healthcare provider about the risks associated with each surgery. […] About 20% to 60% of babies with hypoplastic left heart syndrome survive their first year of life. After that, the survival rate for the next five, 10 and 15 years is about 40%. Babies who have a normal birth weight and arent born prematurely do better than babies with lower birth weights. One study found that most babies who survived their first year were still alive at age 18.
#23 Hypoplastic left heart syndrome: Prognosis and management options
https://pmc.ncbi.nlm.nih.gov/articles/PMC2817797/
The option of palliative or comfort care without aggressive surgical intervention remains, either at home or in hospital. […] Parents and physicians face difficult decisions regarding the appropriate therapeutic intervention for individual infants with HLHS. […] Survival has been shown to be worse in patients with significant noncardiac congenital anomalies, low birth weight and severe obstruction to pulmonary venous return. […] Actuarial survival for all infants with combined staged procedures has been reported to be 63% to 80% at one year of age and 58% to 72% at five years of age. […] Concerns about the long term outcome of infants with HLHS include the adequate preservation of the right ventricle and valvular function when exposed to systemic pressure. […] Cardiac transplantation offers the potential for a normal cardiovascular physiology over the limitations of the single ventricle physiology achieved after multi-stage reconstruction.
#24 Hypoplastic left heart syndrome: Prognosis and management options
https://pmc.ncbi.nlm.nih.gov/articles/PMC2817797/
The major limitations of transplantation are a shortage of donors and size matching from donor to recipient. […] Although prognosis for HLHS is no longer one of certain death, many issues surrounding surgical treatment remain poorly defined. […] The long term outcomes and quality of life following surgery remain poorly defined. […] More information on the natural history of HLHS, its antenatal diagnosis, operative strategies and long term outcome are essential to determine the optimal management for individual infants with HLHS.
#25 Hypoplastic Left Heart Syndrome (HLHS): Causes, Symptoms & Treatment
https://my.clevelandclinic.org/health/diseases/12214-hypoplastic-left-heart-syndrome-hlhs
After treatment for hypoplastic left heart syndrome, your child will need lifelong follow-up visits at least once a year with a cardiologist (heart doctor). These visits ensure that their heart, lungs and other organs continue to work properly. When your child becomes an adult, theyll transition to care with a specialist in adult congenital heart disease. […] Without treatment, hypoplastic left heart syndrome is fatal days or weeks after birth. […] With treatment, the prognosis depends on the complexity of your childs heart deformity. Ask your babys healthcare provider about the risks associated with each surgery. […] About 20% to 60% of babies with hypoplastic left heart syndrome survive their first year of life. After that, the survival rate for the next five, 10 and 15 years is about 40%. Babies who have a normal birth weight and arent born prematurely do better than babies with lower birth weights. One study found that most babies who survived their first year were still alive at age 18.
#26 Hypoplastic left heart syndrome – Symptoms and causes – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/hypoplastic-left-heart-syndrome/symptoms-causes/syc-20350599
Treatment for hypoplastic left heart syndrome may include medicines, heart surgery or a heart transplant. Advances in care have improved the outlook for babies born with HLHS. […] With proper treatment, many babies with hypoplastic left heart syndrome (HLHS) survive. But they do need many surgeries and can have less energy and other challenges. Complications of HLHS may include: […] Children with HLHS may require a heart transplant later in life. […] I hope that the doctors find the cure for HLHS. We’re all part of the team.
#27 Hypoplastic left heart syndrome – Symptoms and causes – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/hypoplastic-left-heart-syndrome/symptoms-causes/syc-20350599
Treatment for hypoplastic left heart syndrome may include medicines, heart surgery or a heart transplant. Advances in care have improved the outlook for babies born with HLHS. […] With proper treatment, many babies with hypoplastic left heart syndrome (HLHS) survive. But they do need many surgeries and can have less energy and other challenges. Complications of HLHS may include: […] Children with HLHS may require a heart transplant later in life. […] I hope that the doctors find the cure for HLHS. We’re all part of the team.
#28 Using machine learning to predict five-year transplant-free survival among infants with hypoplastic left heart syndrome | Scientific Reports
https://www.nature.com/articles/s41598-024-55285-1
Hypoplastic left heart syndrome (HLHS) is a congenital malformation commonly treated with palliative surgery and is associated with significant morbidity and mortality. […] Individualized prediction of transplant-free survival (TFS) employing machine learning (ML) based analyses of outcomes beyond infancy may provide further valuable insight for families and healthcare providers along the course of a staged palliation. […] Models trained with features from Pre-S1P, S1P operation, and S1P hospitalization all demonstrated time-dependent area under the curves (td-AUC) beyond 0.70 through 5 years following S1P, with a model incorporating features through S1P hospitalization demonstrating particularly robust performance (td-AUC 0.838 (95% CI 0.8360.840)). […] The purpose of this study was to leverage the longer endpoint data of the SVR II study to inform an ML-based model, which can accurately predict individualized transplant-free survival through five years using data available prior to S1P.
#29 Using machine learning to predict five-year transplant-free survival among infants with hypoplastic left heart syndrome | Scientific Reports
https://www.nature.com/articles/s41598-024-55285-1
Hypoplastic left heart syndrome (HLHS) is a congenital malformation commonly treated with palliative surgery and is associated with significant morbidity and mortality. […] Individualized prediction of transplant-free survival (TFS) employing machine learning (ML) based analyses of outcomes beyond infancy may provide further valuable insight for families and healthcare providers along the course of a staged palliation. […] Models trained with features from Pre-S1P, S1P operation, and S1P hospitalization all demonstrated time-dependent area under the curves (td-AUC) beyond 0.70 through 5 years following S1P, with a model incorporating features through S1P hospitalization demonstrating particularly robust performance (td-AUC 0.838 (95% CI 0.8360.840)). […] The purpose of this study was to leverage the longer endpoint data of the SVR II study to inform an ML-based model, which can accurately predict individualized transplant-free survival through five years using data available prior to S1P.
#30 Using machine learning to predict five-year transplant-free survival among infants with hypoplastic left heart syndrome | Scientific Reports
https://www.nature.com/articles/s41598-024-55285-1
We report here a novel, individualized predictor of death or transplant through five years among patients with HLHS following S1P, incorporating both modifiable and nonmodifiable features known in the pre-S1P phase of care into an ML-driven predictive model. […] We also report a series of adaptive models which capture the evolution of individual risk by accounting for nonlinear effects of features associated with successive stages of care, with particularly robust model performance incorporating features through S1P hospitalization. […] Employing only features available in the pre-S1P phase (Phase A), we report here a model predicting six-year TFS with satisfactory clinical performance with a C index of 0.692 (95% CI 0.6900.695). […] Using ML-based survival analysis of publicly available longitudinal data from a randomized controlled clinical trial, we report the development of an individualized series of models predicting the probability of transplant-free survival through five years following S1P.
#31 Using machine learning to predict five-year transplant-free survival among infants with hypoplastic left heart syndrome | Scientific Reports
https://www.nature.com/articles/s41598-024-55285-1
We report here a novel, individualized predictor of death or transplant through five years among patients with HLHS following S1P, incorporating both modifiable and nonmodifiable features known in the pre-S1P phase of care into an ML-driven predictive model. […] We also report a series of adaptive models which capture the evolution of individual risk by accounting for nonlinear effects of features associated with successive stages of care, with particularly robust model performance incorporating features through S1P hospitalization. […] Employing only features available in the pre-S1P phase (Phase A), we report here a model predicting six-year TFS with satisfactory clinical performance with a C index of 0.692 (95% CI 0.6900.695). […] Using ML-based survival analysis of publicly available longitudinal data from a randomized controlled clinical trial, we report the development of an individualized series of models predicting the probability of transplant-free survival through five years following S1P.
#32 Frontiers | The Improved Prognosis of Hypoplastic Left Heart: A Population-Based Register Study of 343 Cases in England and Wales
https://www.frontiersin.org/journals/pediatrics/articles/10.3389/fped.2021.635776/full
The primary aim of this study was to report contemporary survival estimates up to age 15 for children born with HLHS or hypoplastic left ventricle in addition to other CHD subtypes in England and Wales, and to examine risk factors for mortality among these cases. […] Based on this population-based study of 343 traced cases of HLHS or hypoplastic left ventricle with additional CHD born in England and Wales, we have shown that survival to age 5 years in the twenty first century is as high as 59 and 68%, respectively. Survival was slightly greater amongst cases with hypoplastic left ventricle with major CHD compared to HLHS, although the difference was not statistically significant. This study shows that the greatest risk of mortality in those with HLHS or hypoplastic left ventricle and additional CHD occurs during the first week of life, although survival continued to decrease over the 15 years of follow-up. Survival estimates were increased in the later cohort (2006â2012 vs. 1998â2005) although for cases of HLHS they may only have been increased until age 5 years.
#33 Frontiers | The Improved Prognosis of Hypoplastic Left Heart: A Population-Based Register Study of 343 Cases in England and Wales
https://www.frontiersin.org/journals/pediatrics/articles/10.3389/fped.2021.635776/full
Results: There were 244 cases of HLHS and 99 cases of hypoplastic left ventricle co-occurring with other CHD, with traced survival status. Kaplan-Meier survival estimates for HLHS were 84.4% at age 1 week, 76.2% at 1 month, 63.5% at age 1 year, 58.6% at age 5 years, 54.6% at age 10 years, and 32.6% to age 15 years. The Kaplan-Meier survival estimates for cases of hypoplastic left ventricle co-occurring with additional CHD were 90.9% at age 1 week, 84.9% at 1 month, 73.7% at age 1 year, 67.7% to age 5 years, 59.2% to age 10 years, and 40.3% to age 15 years. Preterm birth (p = 0.007), low birth weight (p = 0.005), and female sex (p = 0.01) were associated with mortality. […] Conclusions: We have shown that prognosis associated with HLHS in the twenty first century exceeds that of many previous population-based studies, likely due to improvements in intensive care technologies and advances in surgical techniques over the last few decades.
#34 Frontiers | The Improved Prognosis of Hypoplastic Left Heart: A Population-Based Register Study of 343 Cases in England and Wales
https://www.frontiersin.org/journals/pediatrics/articles/10.3389/fped.2021.635776/full
The primary aim of this study was to report contemporary survival estimates up to age 15 for children born with HLHS or hypoplastic left ventricle in addition to other CHD subtypes in England and Wales, and to examine risk factors for mortality among these cases. […] Based on this population-based study of 343 traced cases of HLHS or hypoplastic left ventricle with additional CHD born in England and Wales, we have shown that survival to age 5 years in the twenty first century is as high as 59 and 68%, respectively. Survival was slightly greater amongst cases with hypoplastic left ventricle with major CHD compared to HLHS, although the difference was not statistically significant. This study shows that the greatest risk of mortality in those with HLHS or hypoplastic left ventricle and additional CHD occurs during the first week of life, although survival continued to decrease over the 15 years of follow-up. Survival estimates were increased in the later cohort (2006â2012 vs. 1998â2005) although for cases of HLHS they may only have been increased until age 5 years.
#35 Hypoplastic left heart syndrome: Prognosis and management options
https://pmc.ncbi.nlm.nih.gov/articles/PMC2817797/
The major limitations of transplantation are a shortage of donors and size matching from donor to recipient. […] Although prognosis for HLHS is no longer one of certain death, many issues surrounding surgical treatment remain poorly defined. […] The long term outcomes and quality of life following surgery remain poorly defined. […] More information on the natural history of HLHS, its antenatal diagnosis, operative strategies and long term outcome are essential to determine the optimal management for individual infants with HLHS.