Transpozycja wielkich naczyń – Rokowania, prognozy i postęp choroby

Transpozycja wielkich naczyń
Rokowania, prognozy i postęp choroby

Transpozycja wielkich naczyń (TGA) jest jedną z najczęstszych wrodzonych wad serca wymagających wczesnej interwencji chirurgicznej, z częstością występowania 4/10 000 żywych urodzeń. Rokowanie zależy od anatomicznego podłoża i zastosowanej metody operacyjnej, najczęściej arterial switch operation (ASO) lub procedury Rastelliego w przypadku współistniejącego dużego ubytku przegrody międzykomorowej (VSD) i zwężenia tętnicy płucnej. Bez leczenia śmiertelność wynosi około 30% w pierwszym tygodniu życia, 50% w pierwszym miesiącu i 90% do końca pierwszego roku. Operacja ASO wykonywana w pierwszym tygodniu życia zapewnia ponad 90% przeżywalności okołooperacyjnej oraz ponad 95% przeżywalności po 15-25 latach, z doskonałym długoterminowym wynikiem bez zaburzeń rytmu (około 97% po 25 latach). Jednak u około 50% pacjentów w średnim wieku 22 lat obserwuje się zaburzenia rytmu, a 25% wymaga wszczepienia rozrusznika lub kardiowertera-defibrylatora.

Prognoza i przewidywanie wyników u pacjentów z transpozycją wielkich naczyń

Przeżywalność i rokowanie bez leczenia
Wyniki po leczeniu chirurgicznym
Czynniki wpływające na rokowanie
Powikłania długoterminowe
Długoterminowe rokowanie i jakość życia
Kolejne rozdziały

Prognoza i przewidywanie wyników u pacjentów z transpozycją wielkich naczyń

Transpozycja wielkich naczyń (TGA) to jedna z najczęstszych wrodzonych wad serca wymagających interwencji chirurgicznej w okresie noworodkowym, występująca u 4 noworodków na 10 000 żywych urodzeń w Holandii. Rokowanie w przypadku transpozycji wielkich naczyń zależy od specyficznego podłoża anatomicznego oraz zastosowanej metody chirurgicznej (operacja arterial switch, operacja na poziomie przedsionków lub procedura Rastelliego).¹²

Przeżywalność i rokowanie bez leczenia

W przypadku braku interwencji chirurgicznej rokowanie jest bardzo niekorzystne. Badania wskazują, że śmiertelność u nieleczonych pacjentów wynosi około 30% w pierwszym tygodniu życia, 50% w pierwszym miesiącu i aż 90% do końca pierwszego roku. Pierwszy miesiąc życia stanowi okres największego ryzyka (8% śmiertelności). Jeśli nie zostanie przeprowadzona operacja korekcyjna, oczekiwana długość życia wynosi jedynie kilka miesięcy.³⁴⁵

Wyniki po leczeniu chirurgicznym

Dzięki postępom w diagnostyce oraz technikach medycznych i chirurgicznych, ogólna krótko- i średnioterminowa przeżywalność przekracza 90%. Zabieg chirurgiczny naprawy d-TGA powinien być wykonany w pierwszym tygodniu życia. Najczęściej stosowanymi procedurami są standardowa operacja arterial switch (ASO) oraz procedura Rastelliego, która jest zalecana dla pacjentów z d-TGA, dużym ubytkiem przegrody międzykomorowej (VSD) i zwężeniem tętnicy płucnej.⁶⁷

Wyniki po operacji arterial switch

Ogólna przeżywalność okołooperacyjna po operacji arterial switch przekracza 90%. Wskaźnik przeżywalności po wypisie ze szpitala wynosi ponad 95% nawet po 15-25 latach. Długoterminowe przeżycie bez zaburzeń rytmu serca jest również doskonałe (około 97% po 25 latach). Późna śmiertelność wynika głównie z nagłego zgonu sercowego i zawału mięśnia sercowego.⁸⁹¹⁰

Badania retrospektywne wskazują jednak, że zaburzenia rytmu serca występują u prawie połowy pacjentów w średnim wieku 22 lat, a 25% pacjentów wymaga rozrusznika serca lub wszczepialnego kardiowertera-defibrylatora.¹¹

Wyniki po operacjach na poziomie przedsionków

Ogólna śmiertelność po operacji na poziomie przedsionków (switch przedsionkowy) jest niska, jednak długoterminowa zachorowalność związana z rozszerzeniem i niewydolnością prawej komory (systemowej), niedomykalnością zastawki przedsionkowo-komorowej (trójdzielnej) systemowej oraz przedsionkowymi brady- i tachyarytmiami jest znacząca.¹²

Istnieją znaczące obawy dotyczące zdolności anatomicznej prawej komory do utrzymania krążenia systemowego u pacjentów z TGA, którzy przeszli procedurę Mustarda. W tej procedurze wewnątrzprzedsionkowej anatomiczna prawa komora staje się komorą systemową, odpowiedzialną za pompowanie krwi do głównej tętnicy i zarządzanie ciśnieniami 3-4 razy wyższymi niż w obiegu płucnym, co prowadzi do znacznego obciążenia ciśnieniowego. Z czasem, anatomiczna prawa komora często ma trudności z utrzymaniem krążenia systemowego, prowadząc do stopniowego pogorszenia stanu klinicznego pacjentów po naprawie metodą Mustarda.¹³

Czynniki wpływające na rokowanie

Układ tętnic wieńcowych

Nieprawidłowy układ tętnic wieńcowych może mieć negatywny wpływ na wyniki operacji arterial switch. Badania wykazały, że niezgodność komisuralna (CM) między zastawkami półksiężycowatymi może być związana z nieprawidłowym wzorem tętnic wieńcowych (CA) u pacjentów z transpozycją wielkich naczyń.¹⁴

Współczynnik C-Ratio pomaga kategoryzować stopień niezgodności komisuralnej jako mniejszy (mniej niż 0,31) lub większy (więcej niż 0,31). Wyższy współczynnik C-Ratio przewiduje wyższą częstość występowania nietypowego wzoru tętnic wieńcowych. W badaniach wykazano, że dwie trzecie (67%) pacjentów z większą niezgodnością komisuralną (C-Ratio powyżej 0,31) miało nietypowy wzór tętnic wieńcowych, podczas gdy 88% pacjentów z mniejszą niezgodnością miało typowy wzór tętnic wieńcowych.¹⁵

Czas krążenia pozaustrojowego był znacząco krótszy u pacjentów z typowym wzorem tętnic wieńcowych w porównaniu z pacjentami z nietypowym wzorem. Niezgodność komisuralna i nietypowy wzór tętnic wieńcowych mają negatywny wpływ na wyniki operacji arterial switch.¹⁶

Natlenienie mózgu przed operacją

Obniżone przedoperacyjne nasycenie tlenem w mózgu u noworodków z TGA może wpływać na rozwój neurologiczny. Badania sugerują, że przedłużone niskie przedoperacyjne i pooperacyjne regionalne nasycenie mózgu tlenem (rcSO₂) u noworodków z wrodzoną wadą serca jest związane z uszkodzeniem mózgu i upośledzonym rozwojem neurologicznym.¹⁷

Wcześniejsze badania wykazały tendencję do mniej korzystnego rozwoju neurologicznego u noworodków z przedoperacyjnym rcSO₂ < 35% w porównaniu z noworodkami z wyższym przedoperacyjnym rcSO₂. Balonowa septostomia przedsionkowa (BAS) poprawia nasycenie mózgu tlenem u noworodków z TGA, których natlenowanie może być upośledzone, i może być uznana za interwencję neuroprotekcyjną u tych noworodków. BAS prowadziła do poprawy rcSO₂ do prawdopodobnie bezpieczniejszych wartości mediany 48% 2 godziny po BAS i 64% 24 godziny po BAS.¹⁸¹⁹

Powikłania długoterminowe

Mimo wysokiej przeżywalności po operacjach, u pacjentów z TGA mogą wystąpić różne powikłania długoterminowe.²⁰

Po operacji arterial switch

Niedomykalność chronotropowa (nieprawidłowa reakcja częstości akcji serca na wysiłek)
Zwężenie w miejscu neoaorty nadzastawkowej
Zwężenie neopłucne, tętnic płucnych gałęziowych i ujść tętnic wieńcowych
Postępujące poszerzenie korzenia neoaorty (częste i stanowi czynnik ryzyka niedomykalności zastawki neoaorty)

²¹

Mimo tych potencjalnych powikłań, większość pacjentów utrzymuje prawidłową funkcję skurczową i wydolność wysiłkową.²²

Powikłania neurologiczne

Długoterminowe badania kontrolne wykazują, że 30-50% dzieci w wieku szkolnym z TGA wykazuje pewną formę opóźnienia rozwojowego, w tym opóźnienie motoryki małej i dużej, upośledzenie integracji wzrokowo-ruchowej, trudności językowe i mowne oraz problemy behawioralne.²³

Reinterwencje po leczeniu

Reinterwencje po korekcji anatomicznej ccTGA (wrodzonej skorygowanej transpozycji wielkich naczyń) są częste. W szczególności, wymiany przewodów są szczególnie częste w grupie po operacji Rastelliego z operacją przedsionkową, przy czym większość pacjentów prawdopodobnie będzie wymagać co najmniej jednej wymiany na większy przewód odpowiedni do wymiany przezskórnej.²⁴

Po początkowej reoperacji często wykonuje się wiele przezskórnych wymian, co zmniejsza potrzebę kolejnych operacji. Powikłania związane z przegrodami, takie jak resztkowe przecieki lub zwężenia, są dobrze udokumentowane po operacjach Mustarda lub Senninga i są częstym powodem reoperacji po anatomicznej korekcji ccTGA. Jednak w obecnej erze większość problemów związanych z przegrodami można skutecznie leczyć technikami przezskórnymi, minimalizując potrzebę dodatkowych operacji.²⁵

Długoterminowe rokowanie i jakość życia

Chociaż operacja nie leczy całkowicie d-transpozycji wielkich naczyń, większość osób z tym schorzeniem może prowadzić pełne, zdrowe życie. Jednak osoby z tą wrodzoną wadą wymagają dożywotniej opieki.²⁶

Kardiolodzy (specjaliści od serca) monitorują postępy, aby pomóc w zarządzaniu powikłaniami. Po operacji arterial switch stan większości pacjentów poprawia się, a wskaźnik przeżywalności wynosi ponad 95%. Ten wskaźnik przeżywalności utrzymuje się nawet 25 lat później.²⁷

Około 50% noworodków z transpozycją wielkich naczyń przeżyje 5 lat z doskonałą funkcją, a dodatkowe 15-20% przeżyje z jednym lub więcej upośledzeniami medycznymi.²⁸

Większość niemowląt, które przechodzą operację arterial switch, nie ma objawów po operacji i prowadzi normalne życie. Dziecko może prowadzić pełne życie po leczeniu transpozycji wielkich naczyń. Dla obu typów schorzenia, serce wymaga dożywotniej opieki, w tym regularnych wizyt u kardiologa z doświadczeniem w leczeniu tej wady.²⁹³⁰

Wiele osób, które przeszły operację d-TGA, miało udane ciąże. Zaleca się jednak rozmowę z lekarzem prowadzącym i kardiologiem przed zajściem w ciążę. Może być konieczne znalezienie ośrodka oferującego zarówno usługi położnicze wysokiego ryzyka, jak i specjalistyczną wiedzę w zakresie wrodzonych wad serca u dorosłych.³¹

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Materiały źródłowe

#1 Effect of balloon atrial septostomy on cerebral oxygenation in neonates with transposition of the great arteries | Pediatric Research
https://www.nature.com/articles/pr2012147
Transposition of the great arteries (TGA) is one of the most common congenital heart diseases requiring neonatal surgical intervention, occurring in 4 neonates per 10,000 live births in The Netherlands (1). […] Although mortality is low, neonates with TGA are at risk of impaired neurodevelopmental outcome. Long-term follow-up demonstrates that 30-50% of school-aged children with TGA show some form of developmental delay, including fine and gross motor delay, impaired visuomotor integration, language and speech difficulties, and behavioral problems (2,3,4,5). […] It has been suggested that a prolonged low pre- and postoperative rcSO2 in neonates with congenital heart disease is associated with brain injury and impaired neurodevelopmental outcome. A previous study of cerebral oxygenation in neonates with TGA undergoing an arterial switch operation showed a trend toward a less favorable neurodevelopmental outcome in neonates with a preoperative rcSO2 < 35%, as compared with neonates with a higher preoperative rcSO2 (15).
#2 Transposition of the Great Arteries: Background, Pathophysiology, Etiology
https://emedicine.medscape.com/article/900574-overview
The prognosis depends on the specific anatomic substrate and type of surgical therapy used (arterial switch operation, atrial switch operation, or Rastelli procedure). […] Overall, perioperative survival following arterial switch operation is greater than 90%. […] In one study, long-term and arrhythmia-free survival was excellent (approximately 97% at 25 years); late mortality was predominantly due to sudden death and myocardial infarction. […] In another retrospective study, arrhythmias occurred in almost half of patients at a median age of 22 years, and 25% of patients received a pacemaker or implantable cardioverterdefibrillator. […] The overall mortality rate following an atrial level switch is low; however, long-term morbidity associated with systemic (right) ventricular dilatation and failure, systemic atrioventricular (tricuspid) valve regurgitation, and atrial bradyarrhythmias and tachyarrhythmias is significant.
#3 Transposition of the Great Arteries: Background, Pathophysiology, Etiology
https://emedicine.medscape.com/article/900574-overview
A subset of patients may experience profound right ventricular failure, but they may do well with left ventricular retraining and late arterial switch. […] After arterial switch operation, sequelae may include chronotropic incompetence and stenosis at the supravalve neoaortic, neopulmonary, branch pulmonary arteries, and coronary artery ostia. […] However, most patients maintain normal systolic function and exercise capacity. […] Progressive neoaortic root dilation is common and is a risk factor for neoaortic valve regurgitation following arterial switch operation. […] Continued surveillance of this population is required. […] The mortality rate in untreated patients is approximately 30% in the first week, 50% in the first month, and 90% by the end of the first year. […] Long-term complications are secondary to prolonged cyanosis and include polycythemia and hyperviscosity syndrome.
#4 Transposition of the Great Arteries – UF Health
https://ufhealth.org/conditions-and-treatments/transposition-of-the-great-arteries
The child’s symptoms will improve after surgery to correct the defect. Most infants who undergo arterial switch do not have symptoms after surgery and live normal lives. If corrective surgery is not performed, the life expectancy is only months.
#5 Prognosis for the newborn with transposition of the great arteries – PubMed
https://pubmed.ncbi.nlm.nih.gov/88173/
To determine the prognosis for the newborn with transposition of the great arteries, the clinical course of 112 consecutive neonates with dextrotransposition was reviewed. The 1st month of life was the period of greatest risk (8 percent mortality rate). Between balloon septostomy and baffle repair, 14 of 103 patients at risk (14 percent) either died or had a cerebrovascular accident. The mortality rate at baffle repair was 14 percent (10 deaths in 71 patients), and there were 3 late postoperative deaths. Actuarial analysis of the data indicates that with this plan of management, approximately 50 percent of newborns with transposition of the great arteries will survive 5 years with excellent function and an additional 15 to 20 percent will survive with one or more medical handicaps.
#6 Transposition of the Great Arteries: Background, Pathophysiology, Etiology
https://emedicine.medscape.com/article/900574-overview
These patients may develop headache, decreased exercise tolerance, and stroke. […] Thrombocytopenia is common in patients with cyanotic congenital heart disease leading to bleeding complications. […] With improved diagnostic, medical, and surgical techniques, the overall short-term and midterm survival rate exceeds 90%. […] Patients with a large ventricular septal defect, a patent ductus arteriosus, or both may have an early predilection for congestive heart failure, as pulmonary vascular resistance falls with increasing age. […] Heart failure may be mitigated in those patients with left ventricular outflow tract (pulmonary) stenosis. […] A small percentage (approximately 5%) of patients with transposition of the great arteries (and often a ventricular septal defect) develop accelerated pulmonary vascular obstructive disease and progressive cyanosis despite surgical repair or palliation. […] Long-term survival in this subgroup is particularly poor.
#7 Transposition of the Great Arteries – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK538434/
Surgical repair for d-TGA should be performed within the first week of life. The two most commonly used procedures are the standard ASO and the Rastelli procedure, which is recommended for patients with d-TGA, a large VSD, and pulmonary stenosis. Studies report a survival rate exceeding 95% at 15 to 25 years post-discharge. […] Postoperative complications can result from the underlying pathophysiology, surgical intervention, or residual defects, leading to adverse effects such as right ventricular dysfunction, tricuspid valve regurgitation, supraventricular arrhythmias, interatrial and interventricular septal dysfunction, and, less commonly, pulmonary hypertension. Despite these potential complications, most treated patients reach adulthood, with a 20-year survival rate nearing 90%. The primary cause of death is sudden cardiac death, followed by anatomical right ventricular dysfunction.
#8 Transposition of the Great Arteries: Background, Pathophysiology, Etiology
https://emedicine.medscape.com/article/900574-overview
The prognosis depends on the specific anatomic substrate and type of surgical therapy used (arterial switch operation, atrial switch operation, or Rastelli procedure). […] Overall, perioperative survival following arterial switch operation is greater than 90%. […] In one study, long-term and arrhythmia-free survival was excellent (approximately 97% at 25 years); late mortality was predominantly due to sudden death and myocardial infarction. […] In another retrospective study, arrhythmias occurred in almost half of patients at a median age of 22 years, and 25% of patients received a pacemaker or implantable cardioverterdefibrillator. […] The overall mortality rate following an atrial level switch is low; however, long-term morbidity associated with systemic (right) ventricular dilatation and failure, systemic atrioventricular (tricuspid) valve regurgitation, and atrial bradyarrhythmias and tachyarrhythmias is significant.
#9 Transposition of the Great Arteries (TGA)
https://my.clevelandclinic.org/health/diseases/23387-transposition-of-the-great-arteries
Transposition of the great arteries is a congenital heart condition that happens when the two main arteries going away from your heart are in the wrong places. For many babies with this condition, it affects blood oxygen levels and can be life-threatening without surgery to repair it. But the surgery has a very high survival rate. […] While surgery doesn’t cure d-transposition of the great arteries, most people with the condition can lead full, healthy lives. But people with this congenital condition need lifelong care. A cardiologist (heart specialist) monitors your progress to help you manage complications. […] The survival rate after an arterial switch surgery is more than 95%. This survival rate stands even 25 years later. […] Your child can live a full life after treatment for transposition of the great arteries. For either type of the condition, their heart needs lifelong follow-up care, including: Regular appointments with a cardiologist with expertise in this condition. […] Many people who’ve had d-TGA surgery have had successful pregnancies. Talk to your healthcare provider and a cardiologist before getting pregnant. You may need to find a center that offers both high-risk obstetrical services and expertise in adult congenital heart disease.
#10 Transposition of the Great Arteries – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK538434/
Surgical repair for d-TGA should be performed within the first week of life. The two most commonly used procedures are the standard ASO and the Rastelli procedure, which is recommended for patients with d-TGA, a large VSD, and pulmonary stenosis. Studies report a survival rate exceeding 95% at 15 to 25 years post-discharge. […] Postoperative complications can result from the underlying pathophysiology, surgical intervention, or residual defects, leading to adverse effects such as right ventricular dysfunction, tricuspid valve regurgitation, supraventricular arrhythmias, interatrial and interventricular septal dysfunction, and, less commonly, pulmonary hypertension. Despite these potential complications, most treated patients reach adulthood, with a 20-year survival rate nearing 90%. The primary cause of death is sudden cardiac death, followed by anatomical right ventricular dysfunction.
#11 Transposition of the Great Arteries: Background, Pathophysiology, Etiology
https://emedicine.medscape.com/article/900574-overview
The prognosis depends on the specific anatomic substrate and type of surgical therapy used (arterial switch operation, atrial switch operation, or Rastelli procedure). […] Overall, perioperative survival following arterial switch operation is greater than 90%. […] In one study, long-term and arrhythmia-free survival was excellent (approximately 97% at 25 years); late mortality was predominantly due to sudden death and myocardial infarction. […] In another retrospective study, arrhythmias occurred in almost half of patients at a median age of 22 years, and 25% of patients received a pacemaker or implantable cardioverterdefibrillator. […] The overall mortality rate following an atrial level switch is low; however, long-term morbidity associated with systemic (right) ventricular dilatation and failure, systemic atrioventricular (tricuspid) valve regurgitation, and atrial bradyarrhythmias and tachyarrhythmias is significant.
#12 Transposition of the Great Arteries: Background, Pathophysiology, Etiology
https://emedicine.medscape.com/article/900574-overview
The prognosis depends on the specific anatomic substrate and type of surgical therapy used (arterial switch operation, atrial switch operation, or Rastelli procedure). […] Overall, perioperative survival following arterial switch operation is greater than 90%. […] In one study, long-term and arrhythmia-free survival was excellent (approximately 97% at 25 years); late mortality was predominantly due to sudden death and myocardial infarction. […] In another retrospective study, arrhythmias occurred in almost half of patients at a median age of 22 years, and 25% of patients received a pacemaker or implantable cardioverterdefibrillator. […] The overall mortality rate following an atrial level switch is low; however, long-term morbidity associated with systemic (right) ventricular dilatation and failure, systemic atrioventricular (tricuspid) valve regurgitation, and atrial bradyarrhythmias and tachyarrhythmias is significant.
#13 Transposition of the Great Arteries – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK538434/
Significant concerns exist regarding the ability of the anatomical right ventricle to sustain systemic circulation in patients with TGA who have undergone the Mustard procedure. In this intraatrial procedure, the anatomical right ventricle becomes the systemic ventricle, responsible for pumping blood into the main artery and managing pressures 3 to 4 times higher than those in the pulmonary circuit, leading to a considerable pressure load. Over time, the anatomical right ventricle often struggles to maintain systemic circulation, leading to a gradual decline in the clinical condition of patients following Mustard repair. […] In ccTGA, the improved long-term survival associated with anatomic repair must be carefully weighed against the short-term safety of a physiological repair strategy, which focuses solely on correcting associated abnormalities. However, surgical mortality in the physiological repair population is not insignificant. For instance, a series from the Mayo Clinic reported a 3% surgical mortality rate for operations performed after 1986, with an overall mortality rate of 16%. Similarly, a Dutch series by Bogers et al documented a 6.7% mortality rate.
#14 Commissural Malalignment as a predictor of coronary artery abnormalities in patients with transposition of great arteries | Journal of Congenital Cardiology | Full Text
https://jcongenitalcardiology.biomedcentral.com/articles/10.1186/s40949-020-00039-7
In patients with transposition of the great arteries (TGA), commissural malalignment (CM) between semilunar valves may be associated with abnormal coronary (CA) pattern. […] The prediction revealed that the CA pattern would most probably be usual when there is a minor commissural malalignment (C-Ratio less than the EL50) and most probably be unusual when there is a major malalignment (C-Ratio is greater than the EL50). […] The C-Ratio helps to categorize the degree of CM as minor (less than 0.31) or major (more than 0.31). A higher C-Ratio predicts a higher incidence of unusual CA pattern. […] The relationship between the unusual CA pattern and CM in patients with TGA was described by Kim et al. in 2003. […] In our study, two thirds (67%) of the patients with major CM (C-Ratio above 0.31) had unusual CA pattern, while 88% of the patients with minor malalignment had usual CA pattern (C-Ratio less than 0.31).
#15 Commissural Malalignment as a predictor of coronary artery abnormalities in patients with transposition of great arteries | Journal of Congenital Cardiology | Full Text
https://jcongenitalcardiology.biomedcentral.com/articles/10.1186/s40949-020-00039-7
In patients with transposition of the great arteries (TGA), commissural malalignment (CM) between semilunar valves may be associated with abnormal coronary (CA) pattern. […] The prediction revealed that the CA pattern would most probably be usual when there is a minor commissural malalignment (C-Ratio less than the EL50) and most probably be unusual when there is a major malalignment (C-Ratio is greater than the EL50). […] The C-Ratio helps to categorize the degree of CM as minor (less than 0.31) or major (more than 0.31). A higher C-Ratio predicts a higher incidence of unusual CA pattern. […] The relationship between the unusual CA pattern and CM in patients with TGA was described by Kim et al. in 2003. […] In our study, two thirds (67%) of the patients with major CM (C-Ratio above 0.31) had unusual CA pattern, while 88% of the patients with minor malalignment had usual CA pattern (C-Ratio less than 0.31).
#16 Commissural Malalignment as a predictor of coronary artery abnormalities in patients with transposition of great arteries | Journal of Congenital Cardiology | Full Text
https://jcongenitalcardiology.biomedcentral.com/articles/10.1186/s40949-020-00039-7
The surgical bypass time was significantly shorter in patients with usual CA pattern compared to those with unusual CA pattern. […] Commissural malalignment in patients with TGA is a good predictor of abnormal CA pattern. The C-Ratio helps to categorize the degree of CM. Patients with C-Ratio more than 0.31 are considered as having major CM and are expected to have unusual CA pattern. CM and unusual CA pattern have negative impact on the outcome of the arterial switch operation.
#17 Effect of balloon atrial septostomy on cerebral oxygenation in neonates with transposition of the great arteries | Pediatric Research
https://www.nature.com/articles/pr2012147
Transposition of the great arteries (TGA) is one of the most common congenital heart diseases requiring neonatal surgical intervention, occurring in 4 neonates per 10,000 live births in The Netherlands (1). […] Although mortality is low, neonates with TGA are at risk of impaired neurodevelopmental outcome. Long-term follow-up demonstrates that 30-50% of school-aged children with TGA show some form of developmental delay, including fine and gross motor delay, impaired visuomotor integration, language and speech difficulties, and behavioral problems (2,3,4,5). […] It has been suggested that a prolonged low pre- and postoperative rcSO2 in neonates with congenital heart disease is associated with brain injury and impaired neurodevelopmental outcome. A previous study of cerebral oxygenation in neonates with TGA undergoing an arterial switch operation showed a trend toward a less favorable neurodevelopmental outcome in neonates with a preoperative rcSO2 < 35%, as compared with neonates with a higher preoperative rcSO2 (15).
#18 Effect of balloon atrial septostomy on cerebral oxygenation in neonates with transposition of the great arteries | Pediatric Research
https://www.nature.com/articles/pr2012147
Transposition of the great arteries (TGA) is one of the most common congenital heart diseases requiring neonatal surgical intervention, occurring in 4 neonates per 10,000 live births in The Netherlands (1). […] Although mortality is low, neonates with TGA are at risk of impaired neurodevelopmental outcome. Long-term follow-up demonstrates that 30-50% of school-aged children with TGA show some form of developmental delay, including fine and gross motor delay, impaired visuomotor integration, language and speech difficulties, and behavioral problems (2,3,4,5). […] It has been suggested that a prolonged low pre- and postoperative rcSO2 in neonates with congenital heart disease is associated with brain injury and impaired neurodevelopmental outcome. A previous study of cerebral oxygenation in neonates with TGA undergoing an arterial switch operation showed a trend toward a less favorable neurodevelopmental outcome in neonates with a preoperative rcSO2 < 35%, as compared with neonates with a higher preoperative rcSO2 (15).
#19 Effect of balloon atrial septostomy on cerebral oxygenation in neonates with transposition of the great arteries | Pediatric Research
https://www.nature.com/articles/pr2012147
Given that BAS led to an improvement of rcSO2 to what are probably more safe median values of 48% at 2h after BAS and 64% at 24h after BAS, BAS could be regarded as a neuroprotective intervention in these neonates. […] BAS improves cerebral oxygen saturation in neonates with TGA whose oxygenation may be impaired and might therefore be considered as a neuroprotective intervention in these neonates. Future long-term outcome studies are warranted to determine predictive values of cerebral oxygen saturation and extraction in neonates with TGA on neurocognitive development.
#20 Transposition of the Great Arteries: Background, Pathophysiology, Etiology
https://emedicine.medscape.com/article/900574-overview
A subset of patients may experience profound right ventricular failure, but they may do well with left ventricular retraining and late arterial switch. […] After arterial switch operation, sequelae may include chronotropic incompetence and stenosis at the supravalve neoaortic, neopulmonary, branch pulmonary arteries, and coronary artery ostia. […] However, most patients maintain normal systolic function and exercise capacity. […] Progressive neoaortic root dilation is common and is a risk factor for neoaortic valve regurgitation following arterial switch operation. […] Continued surveillance of this population is required. […] The mortality rate in untreated patients is approximately 30% in the first week, 50% in the first month, and 90% by the end of the first year. […] Long-term complications are secondary to prolonged cyanosis and include polycythemia and hyperviscosity syndrome.
#21 Transposition of the Great Arteries: Background, Pathophysiology, Etiology
https://emedicine.medscape.com/article/900574-overview
A subset of patients may experience profound right ventricular failure, but they may do well with left ventricular retraining and late arterial switch. […] After arterial switch operation, sequelae may include chronotropic incompetence and stenosis at the supravalve neoaortic, neopulmonary, branch pulmonary arteries, and coronary artery ostia. […] However, most patients maintain normal systolic function and exercise capacity. […] Progressive neoaortic root dilation is common and is a risk factor for neoaortic valve regurgitation following arterial switch operation. […] Continued surveillance of this population is required. […] The mortality rate in untreated patients is approximately 30% in the first week, 50% in the first month, and 90% by the end of the first year. […] Long-term complications are secondary to prolonged cyanosis and include polycythemia and hyperviscosity syndrome.
#22 Transposition of the Great Arteries: Background, Pathophysiology, Etiology
https://emedicine.medscape.com/article/900574-overview
A subset of patients may experience profound right ventricular failure, but they may do well with left ventricular retraining and late arterial switch. […] After arterial switch operation, sequelae may include chronotropic incompetence and stenosis at the supravalve neoaortic, neopulmonary, branch pulmonary arteries, and coronary artery ostia. […] However, most patients maintain normal systolic function and exercise capacity. […] Progressive neoaortic root dilation is common and is a risk factor for neoaortic valve regurgitation following arterial switch operation. […] Continued surveillance of this population is required. […] The mortality rate in untreated patients is approximately 30% in the first week, 50% in the first month, and 90% by the end of the first year. […] Long-term complications are secondary to prolonged cyanosis and include polycythemia and hyperviscosity syndrome.
#23 Effect of balloon atrial septostomy on cerebral oxygenation in neonates with transposition of the great arteries | Pediatric Research
https://www.nature.com/articles/pr2012147
Transposition of the great arteries (TGA) is one of the most common congenital heart diseases requiring neonatal surgical intervention, occurring in 4 neonates per 10,000 live births in The Netherlands (1). […] Although mortality is low, neonates with TGA are at risk of impaired neurodevelopmental outcome. Long-term follow-up demonstrates that 30-50% of school-aged children with TGA show some form of developmental delay, including fine and gross motor delay, impaired visuomotor integration, language and speech difficulties, and behavioral problems (2,3,4,5). […] It has been suggested that a prolonged low pre- and postoperative rcSO2 in neonates with congenital heart disease is associated with brain injury and impaired neurodevelopmental outcome. A previous study of cerebral oxygenation in neonates with TGA undergoing an arterial switch operation showed a trend toward a less favorable neurodevelopmental outcome in neonates with a preoperative rcSO2 < 35%, as compared with neonates with a higher preoperative rcSO2 (15).
#24 Transposition of the Great Arteries – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK538434/
Reinterventions after anatomic correction for cc-TGA are common. In particular, conduit replacements are especially frequent in the atrial switch-Rastelli cohort, with most patients likely requiring at least one exchange to a larger conduit suitable for transcatheter replacement. After the initial reoperation, procedures such as multiple transcatheter replacements are often performed, reducing the need for further surgeries. Complications related to baffles, such as residual leaks or stenosis, are well-documented after Mustard or Senning operations and are a common reason for reoperations following anatomic repair for ccTGA. However, in the current era, most baffle-related issues can be effectively managed with transcatheter techniques, minimizing the need for additional surgeries. Residual LVOTO is also a known complication following both types of anatomic repair.
#25 Transposition of the Great Arteries – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK538434/
Reinterventions after anatomic correction for cc-TGA are common. In particular, conduit replacements are especially frequent in the atrial switch-Rastelli cohort, with most patients likely requiring at least one exchange to a larger conduit suitable for transcatheter replacement. After the initial reoperation, procedures such as multiple transcatheter replacements are often performed, reducing the need for further surgeries. Complications related to baffles, such as residual leaks or stenosis, are well-documented after Mustard or Senning operations and are a common reason for reoperations following anatomic repair for ccTGA. However, in the current era, most baffle-related issues can be effectively managed with transcatheter techniques, minimizing the need for additional surgeries. Residual LVOTO is also a known complication following both types of anatomic repair.
#26 Transposition of the Great Arteries (TGA)
https://my.clevelandclinic.org/health/diseases/23387-transposition-of-the-great-arteries
Transposition of the great arteries is a congenital heart condition that happens when the two main arteries going away from your heart are in the wrong places. For many babies with this condition, it affects blood oxygen levels and can be life-threatening without surgery to repair it. But the surgery has a very high survival rate. […] While surgery doesn’t cure d-transposition of the great arteries, most people with the condition can lead full, healthy lives. But people with this congenital condition need lifelong care. A cardiologist (heart specialist) monitors your progress to help you manage complications. […] The survival rate after an arterial switch surgery is more than 95%. This survival rate stands even 25 years later. […] Your child can live a full life after treatment for transposition of the great arteries. For either type of the condition, their heart needs lifelong follow-up care, including: Regular appointments with a cardiologist with expertise in this condition. […] Many people who’ve had d-TGA surgery have had successful pregnancies. Talk to your healthcare provider and a cardiologist before getting pregnant. You may need to find a center that offers both high-risk obstetrical services and expertise in adult congenital heart disease.
#27 Transposition of the Great Arteries (TGA)
https://my.clevelandclinic.org/health/diseases/23387-transposition-of-the-great-arteries
Transposition of the great arteries is a congenital heart condition that happens when the two main arteries going away from your heart are in the wrong places. For many babies with this condition, it affects blood oxygen levels and can be life-threatening without surgery to repair it. But the surgery has a very high survival rate. […] While surgery doesn’t cure d-transposition of the great arteries, most people with the condition can lead full, healthy lives. But people with this congenital condition need lifelong care. A cardiologist (heart specialist) monitors your progress to help you manage complications. […] The survival rate after an arterial switch surgery is more than 95%. This survival rate stands even 25 years later. […] Your child can live a full life after treatment for transposition of the great arteries. For either type of the condition, their heart needs lifelong follow-up care, including: Regular appointments with a cardiologist with expertise in this condition. […] Many people who’ve had d-TGA surgery have had successful pregnancies. Talk to your healthcare provider and a cardiologist before getting pregnant. You may need to find a center that offers both high-risk obstetrical services and expertise in adult congenital heart disease.
#28 Prognosis for the newborn with transposition of the great arteries – PubMed
https://pubmed.ncbi.nlm.nih.gov/88173/
To determine the prognosis for the newborn with transposition of the great arteries, the clinical course of 112 consecutive neonates with dextrotransposition was reviewed. The 1st month of life was the period of greatest risk (8 percent mortality rate). Between balloon septostomy and baffle repair, 14 of 103 patients at risk (14 percent) either died or had a cerebrovascular accident. The mortality rate at baffle repair was 14 percent (10 deaths in 71 patients), and there were 3 late postoperative deaths. Actuarial analysis of the data indicates that with this plan of management, approximately 50 percent of newborns with transposition of the great arteries will survive 5 years with excellent function and an additional 15 to 20 percent will survive with one or more medical handicaps.
#29 Transposition of the Great Arteries – UF Health
https://ufhealth.org/conditions-and-treatments/transposition-of-the-great-arteries
The child’s symptoms will improve after surgery to correct the defect. Most infants who undergo arterial switch do not have symptoms after surgery and live normal lives. If corrective surgery is not performed, the life expectancy is only months.
#30 Transposition of the Great Arteries (TGA)
https://my.clevelandclinic.org/health/diseases/23387-transposition-of-the-great-arteries
Transposition of the great arteries is a congenital heart condition that happens when the two main arteries going away from your heart are in the wrong places. For many babies with this condition, it affects blood oxygen levels and can be life-threatening without surgery to repair it. But the surgery has a very high survival rate. […] While surgery doesn’t cure d-transposition of the great arteries, most people with the condition can lead full, healthy lives. But people with this congenital condition need lifelong care. A cardiologist (heart specialist) monitors your progress to help you manage complications. […] The survival rate after an arterial switch surgery is more than 95%. This survival rate stands even 25 years later. […] Your child can live a full life after treatment for transposition of the great arteries. For either type of the condition, their heart needs lifelong follow-up care, including: Regular appointments with a cardiologist with expertise in this condition. […] Many people who’ve had d-TGA surgery have had successful pregnancies. Talk to your healthcare provider and a cardiologist before getting pregnant. You may need to find a center that offers both high-risk obstetrical services and expertise in adult congenital heart disease.
#31 Transposition of the Great Arteries (TGA)
https://my.clevelandclinic.org/health/diseases/23387-transposition-of-the-great-arteries
Transposition of the great arteries is a congenital heart condition that happens when the two main arteries going away from your heart are in the wrong places. For many babies with this condition, it affects blood oxygen levels and can be life-threatening without surgery to repair it. But the surgery has a very high survival rate. […] While surgery doesn’t cure d-transposition of the great arteries, most people with the condition can lead full, healthy lives. But people with this congenital condition need lifelong care. A cardiologist (heart specialist) monitors your progress to help you manage complications. […] The survival rate after an arterial switch surgery is more than 95%. This survival rate stands even 25 years later. […] Your child can live a full life after treatment for transposition of the great arteries. For either type of the condition, their heart needs lifelong follow-up care, including: Regular appointments with a cardiologist with expertise in this condition. […] Many people who’ve had d-TGA surgery have had successful pregnancies. Talk to your healthcare provider and a cardiologist before getting pregnant. You may need to find a center that offers both high-risk obstetrical services and expertise in adult congenital heart disease.