Wrodzona wada serca u dorosłych
Rokowania, prognozy i postęp choroby
W dobie postępów chirurgii kardiologicznej większość pacjentów z wrodzoną wadą serca (CHD) osiąga wiek dorosły, jednak ich przeżywalność pozostaje niższa niż w populacji ogólnej, a ryzyko nagłych zgonów sercowych jest istotnie podwyższone. Kluczowymi czynnikami prognostycznymi u dorosłych z CHD są złożoność wady (śmiertelność 6,7% vs. 0,1% przy prostych wadach), brak korekcji chirurgicznej (6,1% vs. 0,8% po operacji), obecność sinicy (współczynnik ryzyka 38,1), niewydolność serca oraz hipoalbuminemia (HR 3,37, 95% CI 2,67–4,25). Hipoalbuminemia stanowi silny, niezależny predyktor śmiertelności, niezależny od funkcji nerek i aktywacji neurohormonalnej, co podkreśla konieczność rutynowego monitorowania stężenia albuminy w osoczu u tej populacji.
Prognoza w Wrodzonej Wadzie Serca u Dorosłych
Dzięki znaczącym postępom w chirurgii kardiologicznej większość pacjentów z wrodzoną wadą serca (ang. Congenital Heart Disease, CHD) osiąga obecnie wiek dorosły. Mimo to, przeżywalność w tej grupie pozostaje niższa w porównaniu z populacją ogólną, a nagłe zgony sercowe występują znacznie częściej.1 Chociaż przeżywalność do wieku dorosłego uległa poprawie, to właśnie w dorosłości obserwuje się zwiększoną śmiertelność pacjentów z CHD, co podkreśla konieczność dożywotniej specjalistycznej opieki dla tej wrażliwej populacji.2
Czynniki prognostyczne śmiertelności
Badania wskazują na kilka kluczowych czynników wpływających na rokowanie u dorosłych pacjentów z wrodzoną wadą serca:
- Złożoność wady – pacjenci z złożonymi wadami serca wykazują istotnie wyższą śmiertelność (6,7%) w porównaniu do pacjentów z prostymi wadami (0,1%)3
- Korekcja chirurgiczna – pacjenci bez korekcji chirurgicznej mają wyższy wskaźnik śmiertelności (6,1%) niż osoby po operacji (0,8%)4
- Sinica – w analizie wieloczynnikowej sinica okazała się niezależnym predyktorem śmiertelności (współczynnik ryzyka 38,1)5
- Niewydolność serca – stanowi negatywny predyktor przeżycia6
- Hipoalbuminemia – związana z trzykrotnie zwiększonym ryzykiem zgonu (HR 3,37, 95% CI 2,67 do 4,25)7
Hipoalbuminemia jako marker prognostyczny
Hipoalbuminemia okazuje się być silnym niezależnym czynnikiem prognostycznym u dorosłych pacjentów z CHD. Jej znaczenie jako predyktora śmiertelności jest szczególnie widoczne u pacjentów z umiarkowanymi do złożonych wad serca, którzy znajdują się w grupie zwiększonego ryzyka niepożądanych zdarzeń i mogą odnieść największe korzyści z dokładnych algorytmów stratyfikacji ryzyka.8
Co istotne, hipoalbuminemia zachowuje swoją wartość prognostyczną niezależnie od poziomu kreatyniny i stężenia sodu, sugerując, że niesie informacje prognostyczne wykraczające poza dysfunkcję nerek czy aktywację neurohormonalną.9 Z tego powodu stężenie albuminy w osoczu powinno być elementem rutynowej oceny pacjentów z wrodzoną wadą serca w wieku dorosłym. Niewyjaśniona hipoalbuminemia, nawet u klinicznie stabilnych i skąpoobjawowych pacjentów, powinna skutkować odpowiednimi działaniami mającymi na celu identyfikację przyczyn zaburzeń równowagi albuminy w surowicy, a przede wszystkim prowadzić do ściślejszego monitorowania i wdrożenia środków poprawiających rokowanie.10
Predykcja ryzyka niewydolności serca
Niewydolność serca stanowi istotny problem u pacjentów z wrodzoną wadą serca w wieku dorosłym. Przeglądy systematyczne modeli predykcyjnych wskazują na istotną zmienność w charakterystyce populacji, punktach końcowych oraz analizowanych czynnikach ryzyka.11 Pomimo tych niespójności, kilka czynników konsekwentnie wyróżnia się jako istotne predyktory zdarzeń związanych z niewydolnością serca:
- Peptyd natriuretyczny typu B (BNP) – biomarker pozwalający na ocenę stopnia przeciążenia serca12
- Klasa czynnościowa według New York Heart Association (NYHA) – odzwierciedlająca stopień upośledzenia wydolności fizycznej13
- Charakterystyka wady wrodzonej serca – typ i stopień złożoności wady14
Należy jednak podkreślić, że aktualnie dostępne dowody naukowe są niewystarczające do precyzyjnego profilowania pacjentów z wrodzoną wadą serca w wieku dorosłym pod kątem ryzyka niewydolności serca. Potrzebne są wysokiej jakości badania w celu opracowania unikalnego modelu predykcyjnego dla tej populacji oraz potwierdzenia predykcyjnej roli potencjalnych czynników ryzyka.15
Znaczenie interwencji chirurgicznej
Wcześniejsza korekcja chirurgiczna wady serca wiąże się z lepszą przeżywalnością u dorosłych pacjentów z CHD. Dane wskazują na znacząco niższą śmiertelność u pacjentów po zabiegach kardiochirurgicznych (0,8%) w porównaniu do osób bez interwencji (6,1%).16 Warto jednak zauważyć, że w analizach wieloczynnikowych przebyta operacja kardiochirurgiczna, podobnie jak wiek i płeć, nie stanowi istotnego niezależnego czynnika prognostycznego.17 Może to sugerować, że korzyści z interwencji chirurgicznej są częściowo zależne od innych czynników, takich jak złożoność wady, obecność sinicy czy rozwój niewydolności serca.
Znaczenie długoterminowej obserwacji
Wobec przesunięcia się punktu ciężkości śmiertelności u pacjentów z CHD na wiek dorosły, dożywotnia obserwacja specjalistyczna staje się imperatywem w celu poprawy długoterminowych wyników leczenia i jakości życia tej wrażliwej populacji pacjentów.18 Regularne wizyty kontrolne pozwalają na wczesne wykrycie powikłań, takich jak zaburzenia rytmu serca, niewydolność serca czy pogorszenie funkcji zastawek, co umożliwia wdrożenie odpowiednich interwencji przed rozwinięciem się poważnych konsekwencji.
Stratyfikacja ryzyka oparta na zidentyfikowanych czynnikach prognostycznych powinna być integralną częścią opieki nad dorosłymi pacjentami z wrodzoną wadą serca, pozwalając na indywidualizację częstotliwości wizyt kontrolnych oraz intensywności monitorowania w zależności od profilu ryzyka pacjenta.
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Materiały źródłowe
- #1 Predictors of mortality in adult patients with congenital heart disease – PubMedhttps://pubmed.ncbi.nlm.nih.gov/19653177/
Most patients with congenital heart disease (CHD) reach adulthood thanks to the successful efforts of cardiac surgeons. However, sudden cardiac deaths are significantly more prevalent in this population, and survival is reduced when compared to the general population. The aim of this study is to define the prognostic value of selected clinical parameters to predict mortality in adult CHD patients referred to the specialist outpatient centre. […] Higher mortality was found in the group of patients with complex as opposed to simple CHD (28 [6.7%] vs. 1 [0.1%]; p = 0.00001), in subjects without surgical correction as opposed to those operated on (21 [6.1%] vs. 8 [0.8%], p = 0.00001). […] In multivariate analysis, the independent predictor of mortality was cyanosis (heart rate 38.1). Complexity of lesion (heart rate 6.4) represented a relative risk factor. […] Heart failure and cyanosis are negative predictors of survival in adult patients with CHD. Complexity of the lesion increases the relative risk of mortality. Past cardiac surgery is associated with better survival, but, as with age and gender, it is not a significant prognostic factor.
- #2 Long-Term Follow-Up of Adults with Congenital Heart Disease | SpringerLinkhttps://link.springer.com/10.1007/978-3-031-07563-6_97
Survival for individuals with congenital heart disease (CHD) has greatly improved over the past several decades. […] However, while survival has improved to adulthood, mortality in patients with CHD has shifted to adulthood. […] Lifelong follow-up is imperative in order to improve the long-term outcome and quality of life of this vulnerable patient population.
- #3 Predictors of mortality in adult patients with congenital heart disease – PubMedhttps://pubmed.ncbi.nlm.nih.gov/19653177/
Most patients with congenital heart disease (CHD) reach adulthood thanks to the successful efforts of cardiac surgeons. However, sudden cardiac deaths are significantly more prevalent in this population, and survival is reduced when compared to the general population. The aim of this study is to define the prognostic value of selected clinical parameters to predict mortality in adult CHD patients referred to the specialist outpatient centre. […] Higher mortality was found in the group of patients with complex as opposed to simple CHD (28 [6.7%] vs. 1 [0.1%]; p = 0.00001), in subjects without surgical correction as opposed to those operated on (21 [6.1%] vs. 8 [0.8%], p = 0.00001). […] In multivariate analysis, the independent predictor of mortality was cyanosis (heart rate 38.1). Complexity of lesion (heart rate 6.4) represented a relative risk factor. […] Heart failure and cyanosis are negative predictors of survival in adult patients with CHD. Complexity of the lesion increases the relative risk of mortality. Past cardiac surgery is associated with better survival, but, as with age and gender, it is not a significant prognostic factor.
- #4 Predictors of mortality in adult patients with congenital heart disease – PubMedhttps://pubmed.ncbi.nlm.nih.gov/19653177/
Most patients with congenital heart disease (CHD) reach adulthood thanks to the successful efforts of cardiac surgeons. However, sudden cardiac deaths are significantly more prevalent in this population, and survival is reduced when compared to the general population. The aim of this study is to define the prognostic value of selected clinical parameters to predict mortality in adult CHD patients referred to the specialist outpatient centre. […] Higher mortality was found in the group of patients with complex as opposed to simple CHD (28 [6.7%] vs. 1 [0.1%]; p = 0.00001), in subjects without surgical correction as opposed to those operated on (21 [6.1%] vs. 8 [0.8%], p = 0.00001). […] In multivariate analysis, the independent predictor of mortality was cyanosis (heart rate 38.1). Complexity of lesion (heart rate 6.4) represented a relative risk factor. […] Heart failure and cyanosis are negative predictors of survival in adult patients with CHD. Complexity of the lesion increases the relative risk of mortality. Past cardiac surgery is associated with better survival, but, as with age and gender, it is not a significant prognostic factor.
- #5 Predictors of mortality in adult patients with congenital heart disease – PubMedhttps://pubmed.ncbi.nlm.nih.gov/19653177/
Most patients with congenital heart disease (CHD) reach adulthood thanks to the successful efforts of cardiac surgeons. However, sudden cardiac deaths are significantly more prevalent in this population, and survival is reduced when compared to the general population. The aim of this study is to define the prognostic value of selected clinical parameters to predict mortality in adult CHD patients referred to the specialist outpatient centre. […] Higher mortality was found in the group of patients with complex as opposed to simple CHD (28 [6.7%] vs. 1 [0.1%]; p = 0.00001), in subjects without surgical correction as opposed to those operated on (21 [6.1%] vs. 8 [0.8%], p = 0.00001). […] In multivariate analysis, the independent predictor of mortality was cyanosis (heart rate 38.1). Complexity of lesion (heart rate 6.4) represented a relative risk factor. […] Heart failure and cyanosis are negative predictors of survival in adult patients with CHD. Complexity of the lesion increases the relative risk of mortality. Past cardiac surgery is associated with better survival, but, as with age and gender, it is not a significant prognostic factor.
- #6 Predictors of mortality in adult patients with congenital heart disease – PubMedhttps://pubmed.ncbi.nlm.nih.gov/19653177/
Most patients with congenital heart disease (CHD) reach adulthood thanks to the successful efforts of cardiac surgeons. However, sudden cardiac deaths are significantly more prevalent in this population, and survival is reduced when compared to the general population. The aim of this study is to define the prognostic value of selected clinical parameters to predict mortality in adult CHD patients referred to the specialist outpatient centre. […] Higher mortality was found in the group of patients with complex as opposed to simple CHD (28 [6.7%] vs. 1 [0.1%]; p = 0.00001), in subjects without surgical correction as opposed to those operated on (21 [6.1%] vs. 8 [0.8%], p = 0.00001). […] In multivariate analysis, the independent predictor of mortality was cyanosis (heart rate 38.1). Complexity of lesion (heart rate 6.4) represented a relative risk factor. […] Heart failure and cyanosis are negative predictors of survival in adult patients with CHD. Complexity of the lesion increases the relative risk of mortality. Past cardiac surgery is associated with better survival, but, as with age and gender, it is not a significant prognostic factor.
- #7 Hypoalbuminaemia predicts outcome in adult patients with congenital heart disease | Hearthttps://heart.bmj.com/content/101/9/699
Hypoalbuminaemia is common in patients with ACHD and is associated with a threefold increased risk of risk of death. […] Hypoalbuminaemia was a strong predictor of outcome (HR 3.37, 95% CI 2.67 to 4.25, p0.0001). […] Hypoalbuminaemia is a strong independent predictor of mortality. […] Hypoalbuminaemia appeared to be a stronger predictor of outcome in patients with moderate to complex ACHD, who are at risk of adverse events and, thus, are more likely to benefit from accurate risk stratification algorithms. […] Hypoalbuminaemia likely relates to mortality in the ACHD population through various mechanisms. […] Hypoalbuminaemia was a predictor of mortality independent of creatinine levels and sodium concentration, suggesting that it carries prognostic information beyond renal dysfunction or neurohormonal activation.
- #8 Hypoalbuminaemia predicts outcome in adult patients with congenital heart disease | Hearthttps://heart.bmj.com/content/101/9/699
Hypoalbuminaemia is common in patients with ACHD and is associated with a threefold increased risk of risk of death. […] Hypoalbuminaemia was a strong predictor of outcome (HR 3.37, 95% CI 2.67 to 4.25, p0.0001). […] Hypoalbuminaemia is a strong independent predictor of mortality. […] Hypoalbuminaemia appeared to be a stronger predictor of outcome in patients with moderate to complex ACHD, who are at risk of adverse events and, thus, are more likely to benefit from accurate risk stratification algorithms. […] Hypoalbuminaemia likely relates to mortality in the ACHD population through various mechanisms. […] Hypoalbuminaemia was a predictor of mortality independent of creatinine levels and sodium concentration, suggesting that it carries prognostic information beyond renal dysfunction or neurohormonal activation.
- #9 Hypoalbuminaemia predicts outcome in adult patients with congenital heart disease | Hearthttps://heart.bmj.com/content/101/9/699
Hypoalbuminaemia is common in patients with ACHD and is associated with a threefold increased risk of risk of death. […] Hypoalbuminaemia was a strong predictor of outcome (HR 3.37, 95% CI 2.67 to 4.25, p0.0001). […] Hypoalbuminaemia is a strong independent predictor of mortality. […] Hypoalbuminaemia appeared to be a stronger predictor of outcome in patients with moderate to complex ACHD, who are at risk of adverse events and, thus, are more likely to benefit from accurate risk stratification algorithms. […] Hypoalbuminaemia likely relates to mortality in the ACHD population through various mechanisms. […] Hypoalbuminaemia was a predictor of mortality independent of creatinine levels and sodium concentration, suggesting that it carries prognostic information beyond renal dysfunction or neurohormonal activation.
- #10 Hypoalbuminaemia predicts outcome in adult patients with congenital heart disease | Hearthttps://heart.bmj.com/content/101/9/699
Plasma albumin concentration should be a part of the routine assessment of patients with ACHD, and unexplained hypoalbuminaemia, even in clinically stable and oligosymptomatic patients, should trigger appropriate measures to identify underlying causes of serum albumin imbalance, but more importantly lead to closer monitoring and measures aimed at improving prognosis.
- #11 Heart failure risk predictions in adult patients with congenital heart disease: a systematic review – PubMedhttps://pubmed.ncbi.nlm.nih.gov/31350277/
To summarise existing heart failure (HF) risk prediction models and describe the risk factors for HF-related adverse outcomes in adult patients with congenital heart disease (CHD). […] A wide variation in population characteristics, outcome of interest and candidate risk factors was observed between studies. […] Although there were substantial inconsistencies regarding which patient characteristics were predictive of HF-related adverse outcomes, brain natriuretic peptide, New York Heart Association class and CHD lesion characteristics were shown to be important predictors. […] To date, evidence in the published literature is insufficient to accurately profile patients with ACHD. High-quality studies are required to develop a unique ACHD-HF prediction model and confirm the predictive roles of potential risk factors.
- #12 Heart failure risk predictions in adult patients with congenital heart disease: a systematic review – PubMedhttps://pubmed.ncbi.nlm.nih.gov/31350277/
To summarise existing heart failure (HF) risk prediction models and describe the risk factors for HF-related adverse outcomes in adult patients with congenital heart disease (CHD). […] A wide variation in population characteristics, outcome of interest and candidate risk factors was observed between studies. […] Although there were substantial inconsistencies regarding which patient characteristics were predictive of HF-related adverse outcomes, brain natriuretic peptide, New York Heart Association class and CHD lesion characteristics were shown to be important predictors. […] To date, evidence in the published literature is insufficient to accurately profile patients with ACHD. High-quality studies are required to develop a unique ACHD-HF prediction model and confirm the predictive roles of potential risk factors.
- #13 Heart failure risk predictions in adult patients with congenital heart disease: a systematic review – PubMedhttps://pubmed.ncbi.nlm.nih.gov/31350277/
To summarise existing heart failure (HF) risk prediction models and describe the risk factors for HF-related adverse outcomes in adult patients with congenital heart disease (CHD). […] A wide variation in population characteristics, outcome of interest and candidate risk factors was observed between studies. […] Although there were substantial inconsistencies regarding which patient characteristics were predictive of HF-related adverse outcomes, brain natriuretic peptide, New York Heart Association class and CHD lesion characteristics were shown to be important predictors. […] To date, evidence in the published literature is insufficient to accurately profile patients with ACHD. High-quality studies are required to develop a unique ACHD-HF prediction model and confirm the predictive roles of potential risk factors.
- #14 Heart failure risk predictions in adult patients with congenital heart disease: a systematic review – PubMedhttps://pubmed.ncbi.nlm.nih.gov/31350277/
To summarise existing heart failure (HF) risk prediction models and describe the risk factors for HF-related adverse outcomes in adult patients with congenital heart disease (CHD). […] A wide variation in population characteristics, outcome of interest and candidate risk factors was observed between studies. […] Although there were substantial inconsistencies regarding which patient characteristics were predictive of HF-related adverse outcomes, brain natriuretic peptide, New York Heart Association class and CHD lesion characteristics were shown to be important predictors. […] To date, evidence in the published literature is insufficient to accurately profile patients with ACHD. High-quality studies are required to develop a unique ACHD-HF prediction model and confirm the predictive roles of potential risk factors.
- #15 Heart failure risk predictions in adult patients with congenital heart disease: a systematic review – PubMedhttps://pubmed.ncbi.nlm.nih.gov/31350277/
To summarise existing heart failure (HF) risk prediction models and describe the risk factors for HF-related adverse outcomes in adult patients with congenital heart disease (CHD). […] A wide variation in population characteristics, outcome of interest and candidate risk factors was observed between studies. […] Although there were substantial inconsistencies regarding which patient characteristics were predictive of HF-related adverse outcomes, brain natriuretic peptide, New York Heart Association class and CHD lesion characteristics were shown to be important predictors. […] To date, evidence in the published literature is insufficient to accurately profile patients with ACHD. High-quality studies are required to develop a unique ACHD-HF prediction model and confirm the predictive roles of potential risk factors.
- #16 Predictors of mortality in adult patients with congenital heart disease – PubMedhttps://pubmed.ncbi.nlm.nih.gov/19653177/
Most patients with congenital heart disease (CHD) reach adulthood thanks to the successful efforts of cardiac surgeons. However, sudden cardiac deaths are significantly more prevalent in this population, and survival is reduced when compared to the general population. The aim of this study is to define the prognostic value of selected clinical parameters to predict mortality in adult CHD patients referred to the specialist outpatient centre. […] Higher mortality was found in the group of patients with complex as opposed to simple CHD (28 [6.7%] vs. 1 [0.1%]; p = 0.00001), in subjects without surgical correction as opposed to those operated on (21 [6.1%] vs. 8 [0.8%], p = 0.00001). […] In multivariate analysis, the independent predictor of mortality was cyanosis (heart rate 38.1). Complexity of lesion (heart rate 6.4) represented a relative risk factor. […] Heart failure and cyanosis are negative predictors of survival in adult patients with CHD. Complexity of the lesion increases the relative risk of mortality. Past cardiac surgery is associated with better survival, but, as with age and gender, it is not a significant prognostic factor.
- #17 Predictors of mortality in adult patients with congenital heart disease – PubMedhttps://pubmed.ncbi.nlm.nih.gov/19653177/
Most patients with congenital heart disease (CHD) reach adulthood thanks to the successful efforts of cardiac surgeons. However, sudden cardiac deaths are significantly more prevalent in this population, and survival is reduced when compared to the general population. The aim of this study is to define the prognostic value of selected clinical parameters to predict mortality in adult CHD patients referred to the specialist outpatient centre. […] Higher mortality was found in the group of patients with complex as opposed to simple CHD (28 [6.7%] vs. 1 [0.1%]; p = 0.00001), in subjects without surgical correction as opposed to those operated on (21 [6.1%] vs. 8 [0.8%], p = 0.00001). […] In multivariate analysis, the independent predictor of mortality was cyanosis (heart rate 38.1). Complexity of lesion (heart rate 6.4) represented a relative risk factor. […] Heart failure and cyanosis are negative predictors of survival in adult patients with CHD. Complexity of the lesion increases the relative risk of mortality. Past cardiac surgery is associated with better survival, but, as with age and gender, it is not a significant prognostic factor.
- #18 Long-Term Follow-Up of Adults with Congenital Heart Disease | SpringerLinkhttps://link.springer.com/10.1007/978-3-031-07563-6_97
Survival for individuals with congenital heart disease (CHD) has greatly improved over the past several decades. […] However, while survival has improved to adulthood, mortality in patients with CHD has shifted to adulthood. […] Lifelong follow-up is imperative in order to improve the long-term outcome and quality of life of this vulnerable patient population.
Zobacz więcej
niewydolność serca zaburzenie rytmu serca biomarker wydolność fizyczna przeciążenie serca sinica predyktor śmiertelności hipoalbuminemia powikłanie dysfunkcja zastawki model predykcyjny zabieg kardiochirurgiczny wrodzona wada serca dysfunkcja nerek korekcja chirurgiczna skala NYHA nagły zgon sercowy stratyfikacja ryzyka złożona wada serca interwencja chirurgiczna aktywacja neurohormonalna albumina w surowicy czynnik prognostyczny peptyd natriuretyczny typu B