Atrezja trójdzielna – Rokowania, prognozy i postęp choroby

Atrezja trójdzielna
Rokowania, prognozy i postęp choroby

Atrezja trójdzielna jest złożoną siniczą wrodzoną wadą serca, charakteryzującą się brakiem drożności zastawki trójdzielnej, co bez leczenia prowadzi do wysokiej śmiertelności w pierwszym roku życia. Diagnostyka prenatalna oparta na kompleksowej echokardiografii płodowej umożliwia identyfikację kluczowych czynników rokowniczych, takich jak obecność restrykcyjnego ubytku przegrody międzykomorowej (VSD) oraz zwężenia drogi odpływu z komory, które determinują przeżycie noworodkowe. W przypadku prawidłowego połączenia naczyń wielkich i braku zwężenia tętnicy płucnej, przeżycie 12-miesięczne wynosi nawet 100%, natomiast przy zwężeniu tętnicy płucnej spada do 40%. Wczesne wdrożenie terapii, w tym podawanie prostaglandyny E1 (PGE1) w celu utrzymania drożności przewodu tętniczego (PDA) oraz szybka interwencja chirurgiczna, znacząco poprawiają rokowanie i umożliwiają przeżycie do dorosłości.

Wprowadzenie do prognozy atrezji trójdzielnej
Czynniki wpływające na rokowanie prenatalne

Wady towarzyszące i nieprawidłowości strukturalne
Parametry hemodynamiczne

Rokowanie wczesne po urodzeniu

Przeżycie bez leczenia operacyjnego
Interwencje ratujące życie

Rokowanie długoterminowe po leczeniu operacyjnym

Przeżywalność po procedurze Fontana
Rezultaty etapowego podejścia chirurgicznego
Jakość życia i odległe powikłania

Znaczenie monitorowania i opieki długoterminowej

Monitorowanie domowe
Opieka specjalistyczna

Wpływ wczesnej diagnostyki na prognozę
Podsumowanie prognozy atrezji trójdzielnej

Kolejne rozdziały

Wprowadzenie do prognozy atrezji trójdzielnej

Atrezja trójdzielna (łac. atresia tricuspidalis) jest złożoną siniczą wrodzoną wadą serca, która nieleczona wiąże się z bardzo niekorzystnym rokowaniem. Bez interwencji medycznej większość pacjentów z atrezją trójdzielną umiera w pierwszym roku życia. ¹²³ Postępy w diagnostyce prenatalnej, rozwój technik chirurgicznych oraz nowoczesne procedury monitorowania pacjentów znacząco poprawiły rokowanie w tej grupie chorych, umożliwiając wielu pacjentom przeżycie do wieku dorosłego. ⁴⁵

Czynniki wpływające na rokowanie prenatalne

Dokładna diagnostyka prenatalna atrezji trójdzielnej jest możliwa dzięki zastosowaniu kompleksowej echokardiografii płodowej. Określenie czynników rokowniczych na tym etapie ma kluczowe znaczenie dla planowania dalszego postępowania. ⁶ Do najważniejszych prenatalnych czynników prognostycznych należą:

Wady towarzyszące i nieprawidłowości strukturalne

Obecność restrykcyjnego ubytku przegrody międzykomorowej (VSD) oraz zwężenia drogi odpływu z komory są krytycznymi determinantami wczesnego przeżycia. ⁷⁸ U płodów z prawidłowym połączeniem naczyń wielkich (concordant ventriculo-arterial connection) i bez towarzyszącego zwężenia tętnicy płucnej, przeżycie 12-miesięczne wynosi nawet 100%, podczas gdy tylko 40% płodów ze zwężeniem tętnicy płucnej przeżywa okres noworodkowy. ⁹

Parametry hemodynamiczne

Nieprawidłowe przepływy w przewodzie żylnym (DV – ductus venosus) mogą stanowić dodatkowe markery niekorzystnego rokowania. Zwężenie przepływów wewnątrzsercowych w połączeniu ze złożonymi nieprawidłowościami dróg odpływu stanowią krytyczne determinanty przeżycia. ¹⁰¹¹

Rokowanie wczesne po urodzeniu

Wczesne rozpoznanie atrezji trójdzielnej, szybki i bezpieczny transport do ośrodka kardiologii dziecięcej, nieinwazyjna diagnostyka, dostępność prostaglandyny E1 (PGE1) do utrzymania drożności przewodu tętniczego (PDA) oraz postępy w technikach chirurgicznych i anestezjologicznych znacząco poprawiły rokowanie u tych niemowląt. ¹²

Przeżycie bez leczenia operacyjnego

Nieleczona atrezja trójdzielna jest w większości przypadków śmiertelna. Większość niemowląt z tą wadą nie dożywa pierwszych urodzin bez operacji. ¹³¹⁴¹⁵ Zależnie od stopnia zwężenia dróg odpływu i anomalii towarzyszących, atrezja trójdzielna może być śmiertelna już przy urodzeniu. ¹⁶

Interwencje ratujące życie

Interwencja chirurgiczna jest niezbędna, często w pierwszych dniach lub tygodniach życia, aby przekierować przepływ krwi przez serce. ¹⁷ Wczesna diagnoza i skuteczne interwencje chirurgiczne umożliwiają przeżycie do wieku dorosłego przy zachowaniu dobrej wydolności czynnościowej. ¹⁸

Rokowanie długoterminowe po leczeniu operacyjnym

Przeżywalność po procedurze Fontana

Śmiertelność operacyjna związana z procedurą Fontana (ostatnią z trzech etapów leczenia chirurgicznego) jest obecnie konsekwentnie poniżej 2%. ¹⁹ Kompleksowe badanie przeprowadzone przez Sittiwangkul i wsp. w 2004 roku, koncentrujące się na wynikach pacjentów z atrezją trójdzielną (w latach 1971-1999), wykazało wskaźniki przeżycia na poziomie:

82% po 1 roku
72% po 5 latach
61% po 20 latach ²⁰

Nowsze badanie przeprowadzone przez Mery i wsp., analizujące wyniki pacjentów poddanych procedurze Fontana z różnymi patologiami, w tym atrezją trójdzielną, wykazało wskaźnik przeżycia wolnego od przeszczepu na poziomie 92% po 15 latach oraz 87% wolnych od niewydolności krążenia Fontana. ²¹

Według innych źródeł, 20-letnia przeżywalność po procedurze Fontana waha się od 61% do 85%. ²² Pacjenci po procedurze Fontana mogą oczekiwać nawet 30-letniej przeżywalności na poziomie 85%. ²³

Rezultaty etapowego podejścia chirurgicznego

Wyniki etapowego podejścia chirurgicznego u dzieci z atrezją trójdzielną są dobre. Oczekiwane przeżycie po wszystkich trzech etapach wynosi 75-95% w zależności od dokładnych wad i specyfiki operacji dla danego pacjenta. ²⁴ Przeżywalność 1-roczna po operacji Fontana wynosi 85%, a 5-letnia – 78%. ²⁵

Chociaż operacja nie jest leczeniem przyczynowym, krótko- i średnioterminowe rokowanie dla dzieci poddanych zabiegom chirurgicznym jest obiecujące. Rokowanie jest zwykle gorsze dla dzieci operowanych w późniejszym wieku. ²⁶

Jakość życia i odległe powikłania

Długoterminowa jakość życia po operacji Fontana jest dobra. Najstarsi pacjenci są obecnie w czwartej dekadzie życia, jednak mogą wystąpić różne powikłania, takie jak:

Zaburzenia rytmu serca wymagające leczenia farmakologicznego lub wszczepienia stymulatora serca
Problemy płucne prowadzące do zmniejszenia saturacji krwi tlenem
Problemy trawienne spowodowane utratą białka w kale, mogące prowadzić do obrzęków i zatrzymania wody
W niektórych przypadkach konieczność przeszczepu serca i płuc w późniejszym okresie życia ²⁷

Pacjenci po procedurze Fontana mają średnią przewidywaną długość życia wynoszącą 35-40 lat, chociaż mogą wymagać przeszczepu serca we wcześniejszym wieku. ²⁸ Ponieważ procedura eliminuje sinicę, zostaje zniesiona poliglobulina i przeciążenie objętościowe lewej komory, co pozwala oczekiwać dłuższego życia. ²⁹

Znaczenie monitorowania i opieki długoterminowej

Monitorowanie domowe

Lekarze mogą zalecać monitorowanie domowe w celu poprawy wyników leczenia dziecka z atrezją trójdzielną. Rodzice są instruowani w zakresie kontrolowania wagi i poziomu tlenu u dziecka, śledzenia karmienia i podawania leków. ³⁰

Opieka specjalistyczna

Pacjenci z atrezją trójdzielną powinni być monitorowani przez kardiologa przez całe życie ze względu na ryzyko powikłań związanych z ich stanem. ³¹ Postępy w technikach chirurgicznych i innowacje w leczeniu znacząco poprawiły wyniki leczenia operacyjnego. ³²

Wpływ wczesnej diagnostyki na prognozę

Prenatalna diagnoza atrezji trójdzielnej, w tym towarzyszących jej anomalii wewnątrz- i pozasercowych, może być osiągnięta z wysokim stopniem dokładności przy zastosowaniu kompleksowych technik echokardiografii płodowej. ³³ Wczesna identyfikacja wady wpływa na zmniejszenie wczesnej zachorowalności i śmiertelności. ³⁴

Badanie przesiewowe pulsoksymetrią po 3-4 dniu po urodzeniu może wykryć tę wrodzoną wadę serca. ³⁵ Szczegółowe wyjaśnienie wady serca i wymaganego leczenia powinno być przekazane rodzicom w momencie diagnozy i powtarzane w razie potrzeby. ³⁶

Podsumowanie prognozy atrezji trójdzielnej

Ogólna przeżywalność pacjentów z atrezją trójdzielną wynosi około 64%, przy czacie terminacji ciąży na poziomie 12%. Wśród żywo urodzonych niemowląt, przeżywalność w pierwszym roku życia wynosi 72%, co wskazuje, że wczesna terminacja ciąży znacząco wpływa na ogólne wskaźniki przeżycia. ³⁷³⁸

Pacjenci z atrezją trójdzielną, którzy przechodzą wszystkie trzy zabiegi chirurgiczne, doświadczają poprawy objawów: skóra przestaje być sina, ponieważ bogata w tlen krew może krążyć po całym ciele, zwiększa się apetyt i odzyskują energię. ³⁹

Pomimo znaczącego postępu w leczeniu, atrezja trójdzielna pozostaje poważnym schorzeniem wymagającym kompleksowego, wielodyscyplinarnego podejścia przez całe życie pacjenta. Chociaż oczekiwana długość życia wynosi obecnie kilkadziesiąt lat, niezbędne są dalsze badania w celu walidacji czynników prognostycznych i poprawy poradnictwa prognostycznego. ⁴⁰

Kolejne rozdziały

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Materiały źródłowe

#1 Tricuspid Atresia: Symptoms, Treatment & Prognosis
https://my.clevelandclinic.org/health/diseases/14789-tricuspid-atresia
Children with this condition can have complications between stages of repair. A healthcare provider may recommend home monitoring to improve outcomes for your child. Theyll instruct you on checking your babys weight and oxygen level, tracking their feedings and giving them medicine. […] Untreated tricuspid atresia is often fatal. Most babies with this condition wont reach their first birthday without an operation. […] But most children who have the surgeries survive and live to be adults. One study found that most people with tricuspid atresia are alive 20 years later. People who have the Fontan procedure have a life expectancy of 35 to 40 years, although they may need a heart transplant at an earlier age. […] Although surgery isnt a cure, the short-term and intermediate-term outlook for children who have surgery is promising. The outlook is usually worse for children who have surgery later in life.
#2 Tricuspid Atresia > Fact Sheets > Yale Medicine
https://www.yalemedicine.org/conditions/tricuspid-atresia
When a baby is born with tricuspid atresia, surgeryoften within the first few days or weeks of lifeis needed to redirect blood flow through the heart. […] Without treatment, most patients with tricuspid atresia will die during the first year of life. However, with surgical intervention, the chances of longer-term survival increase substantially. For instance, the 20-year survival after a Fontan procedurethe last of a three-stage sequence of surgeries for children with tricuspid atresiaranges from 61% to 85%. […] If tricuspid atresia is left untreated, patients usually die within the first year of life. But patients with tricuspid atresia who undergo all three surgical procedures experience improvement in their symptoms. Their skin stops appearing bluish because oxygen-rich blood is able to circulate throughout the body, their appetite increases, and they regain energy. People who undergo a Fontan procedure, for example, are projected to have a 30-year survival rate of 85%. […] Patients with tricuspid atresia should be monitored by a cardiologist for the rest of their lives due to the risk of complications associated with their condition.
#3 Tricuspid Atresia: Background, Pathophysiology, Etiology
https://emedicine.medscape.com/article/158359-overview
The 1-year survival rate after the Fontan operation is 85%; the 5-year survival rate is 78%. Because the procedure eliminates cyanosis, polycythemia and left ventricular volume overload are relieved; therefore, this population can be expected to live longer. […] Depending on the degree of obstruction and associated anomalies, tricuspid atresia may be lethal at birth. Without repair, the patient rarely survives to adulthood.
#4 Tricuspid Atresia – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK554495/
The majority of unoperated patients face a high risk of mortality within the first year of life. In the current era of early diagnosis and effective surgical interventions, individuals with tricuspid atresia commonly survive well into adulthood, maintaining good functional capacity. Operative mortality associated with the Fontan procedure is consistently below 2%. A comprehensive study conducted by Sittiwangkul et al in 2004, focusing specifically on outcomes in tricuspid atresia patients (between 1971 and 1999), revealed survival rates of 82%, 72%, and 61% at 1, 5, and 20 years, respectively. […] Surgical outcomes have significantly improved due to advancements in surgical techniques and innovations for addressing patients’ conditions. A recent study conducted by Mery et al, examining outcomes of patients undergoing the Fontan procedure for various pathologies, including tricuspid atresia, demonstrated a transplant-free survival rate of 92% at 15 years and an 87% freedom from Fontan failure.
#5 Prenatal Diagnosis and Management of Tricuspid Valve Atresia: A Case Report and Review of Literature
https://www.heraldopenaccess.us/openaccess/prenatal-diagnosis-and-management-of-tricuspid-valve-atresia-a-case-report-and-review-of-literature
Prognosis in untreated TVAtr is poor. Early identification, rapid and safe transport to a pediatric cardiology center, noninvasive diagnosis, availability of PGE1 to keep PDA, and recent advances in anesthesia and surgical techniques have improved the prognosis of these babies. Complications associated with the classic Fontan are decreased with the widespread use of staged TCPC. […] TVAtr is a complex cyanotic critical CHD requiring multiple medical, intervention and surgical treatment. Prenatal diagnosis is available alternating early morbidity and mortality but also offers to parent the alternative of an elective abortion. A detailed explanation of the cardiac defect and treatment required should be given to the parents at the time of diagnosis and repeated as needed. Further, pulse oximetry screening after the 3rd to 4th day postnatal can detect this CHD.
#6 Prenatal Predictors and Early Postnatal Outcomes in Fetuses Diagnosed with Tricuspid Atresia
https://www.mdpi.com/2075-4418/14/24/2855
Prenatal diagnosis of tricuspid atresia (TA), including its associated intra- and extracardiac anomalies, can be achieved with a high degree of accuracy when employing comprehensive fetal echocardiographic techniques. […] The overall survival rate was 64%, while the termination rate was 12%. Among liveborn infants, the survival rate at one year was 72%, indicating that early termination significantly impacted overall survival rates. […] Factors such as restrictive ventricular septal defects and outflow tract obstructions were strongly associated with decreased survival, highlighting the critical role of detailed prenatal evaluation in risk stratification.
#7 Prenatal Predictors and Early Postnatal Outcomes in Fetuses Diagnosed with Tricuspid Atresia
https://pmc.ncbi.nlm.nih.gov/articles/PMC11675844/
Objective: To assess the prenatal course and early postnatal outcomes of fetuses diagnosed with tricuspid atresia and to identify predictors of survival. […] Tricuspid atresia can be accurately diagnosed in utero with a thorough echocardiographic evaluation. Restrictive VSD and outflow tract obstructions are critical determinants of early survival, while abnormal DV Doppler patterns may serve as additional markers for adverse outcomes. More extensive studies are needed to validate these findings and improve prognostic counseling. […] The survival rates at 3 and 12 months were significantly influenced by the presence of pulmonary stenosis. No intrauterine fetal demise (IUFD) occurred. For fetuses with concordant VA and no associated pulmonary stenosis, survival at 12 months was 100%, whereas only 40% of those with pulmonary stenosis survived beyond the neonatal period.
#8 Prenatal Predictors and Early Postnatal Outcomes in Fetuses Diagnosed with Tricuspid Atresia
https://www.mdpi.com/2075-4418/14/24/2855
Prenatal diagnosis of tricuspid atresia (TA), including its associated intra- and extracardiac anomalies, can be achieved with a high degree of accuracy when employing comprehensive fetal echocardiographic techniques. […] The overall survival rate was 64%, while the termination rate was 12%. Among liveborn infants, the survival rate at one year was 72%, indicating that early termination significantly impacted overall survival rates. […] Factors such as restrictive ventricular septal defects and outflow tract obstructions were strongly associated with decreased survival, highlighting the critical role of detailed prenatal evaluation in risk stratification.
#9 Prenatal Predictors and Early Postnatal Outcomes in Fetuses Diagnosed with Tricuspid Atresia
https://pmc.ncbi.nlm.nih.gov/articles/PMC11675844/
Objective: To assess the prenatal course and early postnatal outcomes of fetuses diagnosed with tricuspid atresia and to identify predictors of survival. […] Tricuspid atresia can be accurately diagnosed in utero with a thorough echocardiographic evaluation. Restrictive VSD and outflow tract obstructions are critical determinants of early survival, while abnormal DV Doppler patterns may serve as additional markers for adverse outcomes. More extensive studies are needed to validate these findings and improve prognostic counseling. […] The survival rates at 3 and 12 months were significantly influenced by the presence of pulmonary stenosis. No intrauterine fetal demise (IUFD) occurred. For fetuses with concordant VA and no associated pulmonary stenosis, survival at 12 months was 100%, whereas only 40% of those with pulmonary stenosis survived beyond the neonatal period.
#10 Prenatal Predictors and Early Postnatal Outcomes in Fetuses Diagnosed with Tricuspid Atresia
https://pmc.ncbi.nlm.nih.gov/articles/PMC11675844/
Objective: To assess the prenatal course and early postnatal outcomes of fetuses diagnosed with tricuspid atresia and to identify predictors of survival. […] Tricuspid atresia can be accurately diagnosed in utero with a thorough echocardiographic evaluation. Restrictive VSD and outflow tract obstructions are critical determinants of early survival, while abnormal DV Doppler patterns may serve as additional markers for adverse outcomes. More extensive studies are needed to validate these findings and improve prognostic counseling. […] The survival rates at 3 and 12 months were significantly influenced by the presence of pulmonary stenosis. No intrauterine fetal demise (IUFD) occurred. For fetuses with concordant VA and no associated pulmonary stenosis, survival at 12 months was 100%, whereas only 40% of those with pulmonary stenosis survived beyond the neonatal period.
#11 Prenatal Predictors and Early Postnatal Outcomes in Fetuses Diagnosed with Tricuspid Atresia
https://pmc.ncbi.nlm.nih.gov/articles/PMC11675844/
The overall survival rate was 64%, while the termination rate was 12%. Among liveborn infants, the survival rate at one year was 72%, indicating that early termination significantly impacted overall survival rates. […] These findings suggest that restrictive intracardiac flow patterns, combined with complex outflow tract abnormalities, are critical determinants of survival, contributing to the discrepancy with other studies.
#12 Prenatal Diagnosis and Management of Tricuspid Valve Atresia: A Case Report and Review of Literature
https://www.heraldopenaccess.us/openaccess/prenatal-diagnosis-and-management-of-tricuspid-valve-atresia-a-case-report-and-review-of-literature
Prognosis in untreated TVAtr is poor. Early identification, rapid and safe transport to a pediatric cardiology center, noninvasive diagnosis, availability of PGE1 to keep PDA, and recent advances in anesthesia and surgical techniques have improved the prognosis of these babies. Complications associated with the classic Fontan are decreased with the widespread use of staged TCPC. […] TVAtr is a complex cyanotic critical CHD requiring multiple medical, intervention and surgical treatment. Prenatal diagnosis is available alternating early morbidity and mortality but also offers to parent the alternative of an elective abortion. A detailed explanation of the cardiac defect and treatment required should be given to the parents at the time of diagnosis and repeated as needed. Further, pulse oximetry screening after the 3rd to 4th day postnatal can detect this CHD.
#13 Tricuspid Atresia: Symptoms, Treatment & Prognosis
https://my.clevelandclinic.org/health/diseases/14789-tricuspid-atresia
Children with this condition can have complications between stages of repair. A healthcare provider may recommend home monitoring to improve outcomes for your child. Theyll instruct you on checking your babys weight and oxygen level, tracking their feedings and giving them medicine. […] Untreated tricuspid atresia is often fatal. Most babies with this condition wont reach their first birthday without an operation. […] But most children who have the surgeries survive and live to be adults. One study found that most people with tricuspid atresia are alive 20 years later. People who have the Fontan procedure have a life expectancy of 35 to 40 years, although they may need a heart transplant at an earlier age. […] Although surgery isnt a cure, the short-term and intermediate-term outlook for children who have surgery is promising. The outlook is usually worse for children who have surgery later in life.
#14 Tricuspid Atresia > Fact Sheets > Yale Medicine
https://www.yalemedicine.org/conditions/tricuspid-atresia
When a baby is born with tricuspid atresia, surgeryoften within the first few days or weeks of lifeis needed to redirect blood flow through the heart. […] Without treatment, most patients with tricuspid atresia will die during the first year of life. However, with surgical intervention, the chances of longer-term survival increase substantially. For instance, the 20-year survival after a Fontan procedurethe last of a three-stage sequence of surgeries for children with tricuspid atresiaranges from 61% to 85%. […] If tricuspid atresia is left untreated, patients usually die within the first year of life. But patients with tricuspid atresia who undergo all three surgical procedures experience improvement in their symptoms. Their skin stops appearing bluish because oxygen-rich blood is able to circulate throughout the body, their appetite increases, and they regain energy. People who undergo a Fontan procedure, for example, are projected to have a 30-year survival rate of 85%. […] Patients with tricuspid atresia should be monitored by a cardiologist for the rest of their lives due to the risk of complications associated with their condition.
#15 Tricuspid Atresia: Background, Pathophysiology, Etiology
https://emedicine.medscape.com/article/158359-overview
The 1-year survival rate after the Fontan operation is 85%; the 5-year survival rate is 78%. Because the procedure eliminates cyanosis, polycythemia and left ventricular volume overload are relieved; therefore, this population can be expected to live longer. […] Depending on the degree of obstruction and associated anomalies, tricuspid atresia may be lethal at birth. Without repair, the patient rarely survives to adulthood.
#16 Tricuspid Atresia: Background, Pathophysiology, Etiology
https://emedicine.medscape.com/article/158359-overview
The 1-year survival rate after the Fontan operation is 85%; the 5-year survival rate is 78%. Because the procedure eliminates cyanosis, polycythemia and left ventricular volume overload are relieved; therefore, this population can be expected to live longer. […] Depending on the degree of obstruction and associated anomalies, tricuspid atresia may be lethal at birth. Without repair, the patient rarely survives to adulthood.
#17 Tricuspid Atresia > Fact Sheets > Yale Medicine
https://www.yalemedicine.org/conditions/tricuspid-atresia
When a baby is born with tricuspid atresia, surgeryoften within the first few days or weeks of lifeis needed to redirect blood flow through the heart. […] Without treatment, most patients with tricuspid atresia will die during the first year of life. However, with surgical intervention, the chances of longer-term survival increase substantially. For instance, the 20-year survival after a Fontan procedurethe last of a three-stage sequence of surgeries for children with tricuspid atresiaranges from 61% to 85%. […] If tricuspid atresia is left untreated, patients usually die within the first year of life. But patients with tricuspid atresia who undergo all three surgical procedures experience improvement in their symptoms. Their skin stops appearing bluish because oxygen-rich blood is able to circulate throughout the body, their appetite increases, and they regain energy. People who undergo a Fontan procedure, for example, are projected to have a 30-year survival rate of 85%. […] Patients with tricuspid atresia should be monitored by a cardiologist for the rest of their lives due to the risk of complications associated with their condition.
#18 Tricuspid Atresia – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK554495/
The majority of unoperated patients face a high risk of mortality within the first year of life. In the current era of early diagnosis and effective surgical interventions, individuals with tricuspid atresia commonly survive well into adulthood, maintaining good functional capacity. Operative mortality associated with the Fontan procedure is consistently below 2%. A comprehensive study conducted by Sittiwangkul et al in 2004, focusing specifically on outcomes in tricuspid atresia patients (between 1971 and 1999), revealed survival rates of 82%, 72%, and 61% at 1, 5, and 20 years, respectively. […] Surgical outcomes have significantly improved due to advancements in surgical techniques and innovations for addressing patients’ conditions. A recent study conducted by Mery et al, examining outcomes of patients undergoing the Fontan procedure for various pathologies, including tricuspid atresia, demonstrated a transplant-free survival rate of 92% at 15 years and an 87% freedom from Fontan failure.
#19 Tricuspid Atresia – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK554495/
The majority of unoperated patients face a high risk of mortality within the first year of life. In the current era of early diagnosis and effective surgical interventions, individuals with tricuspid atresia commonly survive well into adulthood, maintaining good functional capacity. Operative mortality associated with the Fontan procedure is consistently below 2%. A comprehensive study conducted by Sittiwangkul et al in 2004, focusing specifically on outcomes in tricuspid atresia patients (between 1971 and 1999), revealed survival rates of 82%, 72%, and 61% at 1, 5, and 20 years, respectively. […] Surgical outcomes have significantly improved due to advancements in surgical techniques and innovations for addressing patients’ conditions. A recent study conducted by Mery et al, examining outcomes of patients undergoing the Fontan procedure for various pathologies, including tricuspid atresia, demonstrated a transplant-free survival rate of 92% at 15 years and an 87% freedom from Fontan failure.
#20 Tricuspid Atresia – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK554495/
The majority of unoperated patients face a high risk of mortality within the first year of life. In the current era of early diagnosis and effective surgical interventions, individuals with tricuspid atresia commonly survive well into adulthood, maintaining good functional capacity. Operative mortality associated with the Fontan procedure is consistently below 2%. A comprehensive study conducted by Sittiwangkul et al in 2004, focusing specifically on outcomes in tricuspid atresia patients (between 1971 and 1999), revealed survival rates of 82%, 72%, and 61% at 1, 5, and 20 years, respectively. […] Surgical outcomes have significantly improved due to advancements in surgical techniques and innovations for addressing patients’ conditions. A recent study conducted by Mery et al, examining outcomes of patients undergoing the Fontan procedure for various pathologies, including tricuspid atresia, demonstrated a transplant-free survival rate of 92% at 15 years and an 87% freedom from Fontan failure.
#21 Tricuspid Atresia – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK554495/
The majority of unoperated patients face a high risk of mortality within the first year of life. In the current era of early diagnosis and effective surgical interventions, individuals with tricuspid atresia commonly survive well into adulthood, maintaining good functional capacity. Operative mortality associated with the Fontan procedure is consistently below 2%. A comprehensive study conducted by Sittiwangkul et al in 2004, focusing specifically on outcomes in tricuspid atresia patients (between 1971 and 1999), revealed survival rates of 82%, 72%, and 61% at 1, 5, and 20 years, respectively. […] Surgical outcomes have significantly improved due to advancements in surgical techniques and innovations for addressing patients’ conditions. A recent study conducted by Mery et al, examining outcomes of patients undergoing the Fontan procedure for various pathologies, including tricuspid atresia, demonstrated a transplant-free survival rate of 92% at 15 years and an 87% freedom from Fontan failure.
#22 Tricuspid Atresia > Fact Sheets > Yale Medicine
https://www.yalemedicine.org/conditions/tricuspid-atresia
When a baby is born with tricuspid atresia, surgeryoften within the first few days or weeks of lifeis needed to redirect blood flow through the heart. […] Without treatment, most patients with tricuspid atresia will die during the first year of life. However, with surgical intervention, the chances of longer-term survival increase substantially. For instance, the 20-year survival after a Fontan procedurethe last of a three-stage sequence of surgeries for children with tricuspid atresiaranges from 61% to 85%. […] If tricuspid atresia is left untreated, patients usually die within the first year of life. But patients with tricuspid atresia who undergo all three surgical procedures experience improvement in their symptoms. Their skin stops appearing bluish because oxygen-rich blood is able to circulate throughout the body, their appetite increases, and they regain energy. People who undergo a Fontan procedure, for example, are projected to have a 30-year survival rate of 85%. […] Patients with tricuspid atresia should be monitored by a cardiologist for the rest of their lives due to the risk of complications associated with their condition.
#23 Tricuspid Atresia > Fact Sheets > Yale Medicine
https://www.yalemedicine.org/conditions/tricuspid-atresia
When a baby is born with tricuspid atresia, surgeryoften within the first few days or weeks of lifeis needed to redirect blood flow through the heart. […] Without treatment, most patients with tricuspid atresia will die during the first year of life. However, with surgical intervention, the chances of longer-term survival increase substantially. For instance, the 20-year survival after a Fontan procedurethe last of a three-stage sequence of surgeries for children with tricuspid atresiaranges from 61% to 85%. […] If tricuspid atresia is left untreated, patients usually die within the first year of life. But patients with tricuspid atresia who undergo all three surgical procedures experience improvement in their symptoms. Their skin stops appearing bluish because oxygen-rich blood is able to circulate throughout the body, their appetite increases, and they regain energy. People who undergo a Fontan procedure, for example, are projected to have a 30-year survival rate of 85%. […] Patients with tricuspid atresia should be monitored by a cardiologist for the rest of their lives due to the risk of complications associated with their condition.
#24 Tricuspid Atresia | Symptoms, Diagnosis & Treatment
https://www.cincinnatichildrens.org/health/t/tricuspid-atresia
The results of this staged approach for children with tricuspid atresia are good. The expected survival through all three stages is 75-95% depending on the exact defects and surgery for any given person. […] The long-term quality of life following the Fontan operation is good. The oldest patients are currently in their 30s. However, several complications may occur. These include heart rhythm problems needing medication or placement of a pacemaker. Patients may also have lung problems leading to a decrease in oxygen in the blood. Digestive problems caused by loss of protein in the stool may be a concern. This may lead to swelling and water retention. For some patients, heart and lung transplantation may be needed later in life.
#25 Tricuspid Atresia: Background, Pathophysiology, Etiology
https://emedicine.medscape.com/article/158359-overview
The 1-year survival rate after the Fontan operation is 85%; the 5-year survival rate is 78%. Because the procedure eliminates cyanosis, polycythemia and left ventricular volume overload are relieved; therefore, this population can be expected to live longer. […] Depending on the degree of obstruction and associated anomalies, tricuspid atresia may be lethal at birth. Without repair, the patient rarely survives to adulthood.
#26 Tricuspid Atresia: Symptoms, Treatment & Prognosis
https://my.clevelandclinic.org/health/diseases/14789-tricuspid-atresia
Children with this condition can have complications between stages of repair. A healthcare provider may recommend home monitoring to improve outcomes for your child. Theyll instruct you on checking your babys weight and oxygen level, tracking their feedings and giving them medicine. […] Untreated tricuspid atresia is often fatal. Most babies with this condition wont reach their first birthday without an operation. […] But most children who have the surgeries survive and live to be adults. One study found that most people with tricuspid atresia are alive 20 years later. People who have the Fontan procedure have a life expectancy of 35 to 40 years, although they may need a heart transplant at an earlier age. […] Although surgery isnt a cure, the short-term and intermediate-term outlook for children who have surgery is promising. The outlook is usually worse for children who have surgery later in life.
#27 Tricuspid Atresia | Symptoms, Diagnosis & Treatment
https://www.cincinnatichildrens.org/health/t/tricuspid-atresia
The results of this staged approach for children with tricuspid atresia are good. The expected survival through all three stages is 75-95% depending on the exact defects and surgery for any given person. […] The long-term quality of life following the Fontan operation is good. The oldest patients are currently in their 30s. However, several complications may occur. These include heart rhythm problems needing medication or placement of a pacemaker. Patients may also have lung problems leading to a decrease in oxygen in the blood. Digestive problems caused by loss of protein in the stool may be a concern. This may lead to swelling and water retention. For some patients, heart and lung transplantation may be needed later in life.
#28 Tricuspid Atresia: Symptoms, Treatment & Prognosis
https://my.clevelandclinic.org/health/diseases/14789-tricuspid-atresia
Children with this condition can have complications between stages of repair. A healthcare provider may recommend home monitoring to improve outcomes for your child. Theyll instruct you on checking your babys weight and oxygen level, tracking their feedings and giving them medicine. […] Untreated tricuspid atresia is often fatal. Most babies with this condition wont reach their first birthday without an operation. […] But most children who have the surgeries survive and live to be adults. One study found that most people with tricuspid atresia are alive 20 years later. People who have the Fontan procedure have a life expectancy of 35 to 40 years, although they may need a heart transplant at an earlier age. […] Although surgery isnt a cure, the short-term and intermediate-term outlook for children who have surgery is promising. The outlook is usually worse for children who have surgery later in life.
#29 Tricuspid Atresia: Background, Pathophysiology, Etiology
https://emedicine.medscape.com/article/158359-overview
The 1-year survival rate after the Fontan operation is 85%; the 5-year survival rate is 78%. Because the procedure eliminates cyanosis, polycythemia and left ventricular volume overload are relieved; therefore, this population can be expected to live longer. […] Depending on the degree of obstruction and associated anomalies, tricuspid atresia may be lethal at birth. Without repair, the patient rarely survives to adulthood.
#30 Tricuspid Atresia: Symptoms, Treatment & Prognosis
https://my.clevelandclinic.org/health/diseases/14789-tricuspid-atresia
Children with this condition can have complications between stages of repair. A healthcare provider may recommend home monitoring to improve outcomes for your child. Theyll instruct you on checking your babys weight and oxygen level, tracking their feedings and giving them medicine. […] Untreated tricuspid atresia is often fatal. Most babies with this condition wont reach their first birthday without an operation. […] But most children who have the surgeries survive and live to be adults. One study found that most people with tricuspid atresia are alive 20 years later. People who have the Fontan procedure have a life expectancy of 35 to 40 years, although they may need a heart transplant at an earlier age. […] Although surgery isnt a cure, the short-term and intermediate-term outlook for children who have surgery is promising. The outlook is usually worse for children who have surgery later in life.
#31 Tricuspid Atresia > Fact Sheets > Yale Medicine
https://www.yalemedicine.org/conditions/tricuspid-atresia
When a baby is born with tricuspid atresia, surgeryoften within the first few days or weeks of lifeis needed to redirect blood flow through the heart. […] Without treatment, most patients with tricuspid atresia will die during the first year of life. However, with surgical intervention, the chances of longer-term survival increase substantially. For instance, the 20-year survival after a Fontan procedurethe last of a three-stage sequence of surgeries for children with tricuspid atresiaranges from 61% to 85%. […] If tricuspid atresia is left untreated, patients usually die within the first year of life. But patients with tricuspid atresia who undergo all three surgical procedures experience improvement in their symptoms. Their skin stops appearing bluish because oxygen-rich blood is able to circulate throughout the body, their appetite increases, and they regain energy. People who undergo a Fontan procedure, for example, are projected to have a 30-year survival rate of 85%. […] Patients with tricuspid atresia should be monitored by a cardiologist for the rest of their lives due to the risk of complications associated with their condition.
#32 Tricuspid Atresia – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK554495/
The majority of unoperated patients face a high risk of mortality within the first year of life. In the current era of early diagnosis and effective surgical interventions, individuals with tricuspid atresia commonly survive well into adulthood, maintaining good functional capacity. Operative mortality associated with the Fontan procedure is consistently below 2%. A comprehensive study conducted by Sittiwangkul et al in 2004, focusing specifically on outcomes in tricuspid atresia patients (between 1971 and 1999), revealed survival rates of 82%, 72%, and 61% at 1, 5, and 20 years, respectively. […] Surgical outcomes have significantly improved due to advancements in surgical techniques and innovations for addressing patients’ conditions. A recent study conducted by Mery et al, examining outcomes of patients undergoing the Fontan procedure for various pathologies, including tricuspid atresia, demonstrated a transplant-free survival rate of 92% at 15 years and an 87% freedom from Fontan failure.
#33 Prenatal Predictors and Early Postnatal Outcomes in Fetuses Diagnosed with Tricuspid Atresia
https://www.mdpi.com/2075-4418/14/24/2855
Prenatal diagnosis of tricuspid atresia (TA), including its associated intra- and extracardiac anomalies, can be achieved with a high degree of accuracy when employing comprehensive fetal echocardiographic techniques. […] The overall survival rate was 64%, while the termination rate was 12%. Among liveborn infants, the survival rate at one year was 72%, indicating that early termination significantly impacted overall survival rates. […] Factors such as restrictive ventricular septal defects and outflow tract obstructions were strongly associated with decreased survival, highlighting the critical role of detailed prenatal evaluation in risk stratification.
#34 Prenatal Diagnosis and Management of Tricuspid Valve Atresia: A Case Report and Review of Literature
https://www.heraldopenaccess.us/openaccess/prenatal-diagnosis-and-management-of-tricuspid-valve-atresia-a-case-report-and-review-of-literature
Prognosis in untreated TVAtr is poor. Early identification, rapid and safe transport to a pediatric cardiology center, noninvasive diagnosis, availability of PGE1 to keep PDA, and recent advances in anesthesia and surgical techniques have improved the prognosis of these babies. Complications associated with the classic Fontan are decreased with the widespread use of staged TCPC. […] TVAtr is a complex cyanotic critical CHD requiring multiple medical, intervention and surgical treatment. Prenatal diagnosis is available alternating early morbidity and mortality but also offers to parent the alternative of an elective abortion. A detailed explanation of the cardiac defect and treatment required should be given to the parents at the time of diagnosis and repeated as needed. Further, pulse oximetry screening after the 3rd to 4th day postnatal can detect this CHD.
#35 Prenatal Diagnosis and Management of Tricuspid Valve Atresia: A Case Report and Review of Literature
https://www.heraldopenaccess.us/openaccess/prenatal-diagnosis-and-management-of-tricuspid-valve-atresia-a-case-report-and-review-of-literature
Prognosis in untreated TVAtr is poor. Early identification, rapid and safe transport to a pediatric cardiology center, noninvasive diagnosis, availability of PGE1 to keep PDA, and recent advances in anesthesia and surgical techniques have improved the prognosis of these babies. Complications associated with the classic Fontan are decreased with the widespread use of staged TCPC. […] TVAtr is a complex cyanotic critical CHD requiring multiple medical, intervention and surgical treatment. Prenatal diagnosis is available alternating early morbidity and mortality but also offers to parent the alternative of an elective abortion. A detailed explanation of the cardiac defect and treatment required should be given to the parents at the time of diagnosis and repeated as needed. Further, pulse oximetry screening after the 3rd to 4th day postnatal can detect this CHD.
#36 Prenatal Diagnosis and Management of Tricuspid Valve Atresia: A Case Report and Review of Literature
https://www.heraldopenaccess.us/openaccess/prenatal-diagnosis-and-management-of-tricuspid-valve-atresia-a-case-report-and-review-of-literature
Prognosis in untreated TVAtr is poor. Early identification, rapid and safe transport to a pediatric cardiology center, noninvasive diagnosis, availability of PGE1 to keep PDA, and recent advances in anesthesia and surgical techniques have improved the prognosis of these babies. Complications associated with the classic Fontan are decreased with the widespread use of staged TCPC. […] TVAtr is a complex cyanotic critical CHD requiring multiple medical, intervention and surgical treatment. Prenatal diagnosis is available alternating early morbidity and mortality but also offers to parent the alternative of an elective abortion. A detailed explanation of the cardiac defect and treatment required should be given to the parents at the time of diagnosis and repeated as needed. Further, pulse oximetry screening after the 3rd to 4th day postnatal can detect this CHD.
#37 Prenatal Predictors and Early Postnatal Outcomes in Fetuses Diagnosed with Tricuspid Atresia
https://www.mdpi.com/2075-4418/14/24/2855
Prenatal diagnosis of tricuspid atresia (TA), including its associated intra- and extracardiac anomalies, can be achieved with a high degree of accuracy when employing comprehensive fetal echocardiographic techniques. […] The overall survival rate was 64%, while the termination rate was 12%. Among liveborn infants, the survival rate at one year was 72%, indicating that early termination significantly impacted overall survival rates. […] Factors such as restrictive ventricular septal defects and outflow tract obstructions were strongly associated with decreased survival, highlighting the critical role of detailed prenatal evaluation in risk stratification.
#38 Prenatal Predictors and Early Postnatal Outcomes in Fetuses Diagnosed with Tricuspid Atresia
https://pmc.ncbi.nlm.nih.gov/articles/PMC11675844/
The overall survival rate was 64%, while the termination rate was 12%. Among liveborn infants, the survival rate at one year was 72%, indicating that early termination significantly impacted overall survival rates. […] These findings suggest that restrictive intracardiac flow patterns, combined with complex outflow tract abnormalities, are critical determinants of survival, contributing to the discrepancy with other studies.
#39 Tricuspid Atresia > Fact Sheets > Yale Medicine
https://www.yalemedicine.org/conditions/tricuspid-atresia
When a baby is born with tricuspid atresia, surgeryoften within the first few days or weeks of lifeis needed to redirect blood flow through the heart. […] Without treatment, most patients with tricuspid atresia will die during the first year of life. However, with surgical intervention, the chances of longer-term survival increase substantially. For instance, the 20-year survival after a Fontan procedurethe last of a three-stage sequence of surgeries for children with tricuspid atresiaranges from 61% to 85%. […] If tricuspid atresia is left untreated, patients usually die within the first year of life. But patients with tricuspid atresia who undergo all three surgical procedures experience improvement in their symptoms. Their skin stops appearing bluish because oxygen-rich blood is able to circulate throughout the body, their appetite increases, and they regain energy. People who undergo a Fontan procedure, for example, are projected to have a 30-year survival rate of 85%. […] Patients with tricuspid atresia should be monitored by a cardiologist for the rest of their lives due to the risk of complications associated with their condition.
#40 Prenatal Predictors and Early Postnatal Outcomes in Fetuses Diagnosed with Tricuspid Atresia
https://pmc.ncbi.nlm.nih.gov/articles/PMC11675844/
Objective: To assess the prenatal course and early postnatal outcomes of fetuses diagnosed with tricuspid atresia and to identify predictors of survival. […] Tricuspid atresia can be accurately diagnosed in utero with a thorough echocardiographic evaluation. Restrictive VSD and outflow tract obstructions are critical determinants of early survival, while abnormal DV Doppler patterns may serve as additional markers for adverse outcomes. More extensive studies are needed to validate these findings and improve prognostic counseling. […] The survival rates at 3 and 12 months were significantly influenced by the presence of pulmonary stenosis. No intrauterine fetal demise (IUFD) occurred. For fetuses with concordant VA and no associated pulmonary stenosis, survival at 12 months was 100%, whereas only 40% of those with pulmonary stenosis survived beyond the neonatal period.