Transpozycja wielkich naczyń

Transpozycja wielkich naczyń
Epidemiologia

Transpozycja wielkich naczyń (TGA), w szczególności jej najczęstsza forma D-TGA, stanowi około 3% wszystkich wrodzonych wad serca i 20% wad siniczych, z częstością występowania 20-30 przypadków na 100 000 żywych urodzeń (około 1 na 3000-5000). Występuje z przewagą u płci męskiej (stosunek 1,5-3:1). Etiopatogeneza jest wieloczynnikowa, z czynnikami ryzyka takimi jak cukrzyca matki, wiek powyżej 40 lat, palenie tytoniu, infekcje wirusowe i alkoholizm. TGA może występować jako wada izolowana (90%) lub z towarzyszącymi anomaliami, najczęściej ubytkiem przegrody międzykomorowej (30-50%), zwężeniem drogi odpływu lewej komory (do 25%) oraz innymi wadami serca. Diagnostyka prenatalna opiera się na echokardiografii płodowej, jednak wykrywalność prenatalna pozostaje niska (<40% w wielu krajach europejskich). Po urodzeniu rozpoznanie wspomaga pulsoksymetria, echokardiografia, EKG i RTG klatki piersiowej.

Epidemiologia transpozycji wielkich naczyń

Transpozycja wielkich naczyń (TGA) to jedna z najczęstszych wrodzonych wad serca o charakterze siniczym, charakteryzująca się nieprawidłowym połączeniem wielkich naczyń z komorami serca. Występuje w kilku postaciach, z których najczęstszą jest D-TGA (dekstro-transpozycja wielkich naczyń), gdzie aorta wychodzi z prawej komory, a tętnica płucna z lewej komory.¹²

Częstotliwość występowania

Częstość występowania D-TGA szacuje się na poziomie 1 na 3000-5000 żywych urodzeń.³¹ Dane epidemiologiczne wskazują, że:

TGA stanowi około 3% wszystkich wrodzonych wad serca (CHD)⁴⁵
Odpowiada za 20% wszystkich siniczych wad serca⁴⁵
Roczna częstość występowania wynosi 20-30 przypadków na 100 000 żywych urodzeń⁶⁷
W Stanach Zjednoczonych rocznie rodzi się około 928 dzieci z D-TGA⁸

Warto zauważyć, że istnieją pewne różnice w szacunkach występowania między różnymi badaniami i regionami geograficznymi – od 10 do 47 przypadków na 10 000 urodzeń, co może wynikać z różnic w systemach raportowania i diagnostyki.⁹

Rozkład płciowy

D-TGA charakteryzuje się wyraźną przewagą występowania u płci męskiej:

Stosunek mężczyzn do kobiet wynosi od 1,5:1 do 3:1³⁴
Około 60-70% przypadków występuje u chłopców⁴⁶
Badania wskazują na występowanie 2-3 razy częściej u chłopców niż u dziewczynek⁹

ccTGA – epidemiologia

Kongenitalna korygowana transpozycja wielkich naczyń (ccTGA lub L-TGA) jest znacznie rzadszą formą wady:

Występuje w około 0,05% wszystkich przypadków wrodzonych wad serca¹
Szacowana częstość to około 1 na 33 000 żywych urodzeń¹¹⁰
W Stanach Zjednoczonych żyje około 5 000-10 000 osób z ccTGA¹¹¹²

Czynniki ryzyka i etiopatogeneza

Etiopatogeneza TGA jest wieloczynnikowa i nie jest w pełni poznana. Zidentyfikowano jednak pewne czynniki ryzyka:¹³

Cukrzyca matki – dzieci matek z cukrzycą mają zwiększone ryzyko rozwoju TGA¹⁴¹³
Zaawansowany wiek matki – wiek powyżej 40 lat uznawany jest za czynnik ryzyka⁴¹⁵
Palenie tytoniu przez matkę w czasie ciąży¹⁵¹⁶
Choroby wirusowe w czasie ciąży, zwłaszcza różyczka⁴
Alkoholizm matki⁴

Warto podkreślić, że D-TGA rzadko występuje rodzinnie, a częstość wad wrodzonych serca u rodzeństwa dzieci z TGA nie różni się istotnie od populacji ogólnej (około 0,3%).¹³ Badania międzynarodowe na dużych kohortach pacjentów z ccTGA zidentyfikowały jednak przypadki występowania rodzinnego tej wady, co sugeruje potencjalny wspólny szlak patogenetyczny związany z genami lateralizacji.¹

Choroby współistniejące

TGA może występować jako izolowana wada lub z towarzyszącymi innymi nieprawidłowościami:

Około 90% przypadków TGA występuje jako izolowana wada⁶¹⁴
W 10% przypadków TGA towarzyszy innym wadom pozasercowym³¹⁷
Najczęstsze towarzyszące wady serca to:
- Ubytek przegrody międzykomorowej (VSD) – występuje w 30-50% przypadków¹⁸¹⁹
- Zwężenie drogi odpływu lewej komory – do 25% przypadków¹⁸
- Nieprawidłowości tętnic wieńcowych¹⁴
- Koarktacja aorty¹⁴
- Zwężenie zastawki płucnej¹⁴

D-TGA rzadko wiąże się z zespołami genetycznymi. Warto zauważyć, że podczas gdy 80% pacjentów z zespołem DiGeorge’a wykazuje delecję 22q11 i wady stożka, rzadko występuje u nich D-TGA.¹³

Nadzór i diagnostyka

Diagnostyka prenatalna

TGA może być wykryta prenatalnie, chociaż odsetek wykrywalności przedurodzeniowej wciąż pozostaje stosunkowo niski:⁹

W wielu krajach europejskich odsetek wykrywalności prenatalnej jest poniżej 40%⁹
W niektórych regionach odsetek ten wynosi mniej niż 10%⁹
TGA jest rzadziej wykrywana prenatalnie niż zespół hipoplazji lewego serca (HLHS), mimo podobnej częstości występowania⁹

Diagnostyka prenatalna TGA opiera się głównie na echokardiografia-plodowa/” title=”echokardiografia płodowa” class=”to-tag” data-termid=”29695″>echokardiografii płodowej, która może wykazać nieprawidłowe połączenia wielkich naczyń z komorami serca.¹⁰²⁰ W przypadku rozpoznania prenatalnego, zalecane są konsultacje specjalistyczne z pediatrycznym kardiologiem, chirurgiem kardiotorakochirurgicznym i neonatologiem, aby omówić rokowanie i postępowanie po urodzeniu.²¹

Diagnostyka poporodowa

W przypadku braku diagnostyki prenatalnej, TGA jest zwykle diagnozowana wkrótce po urodzeniu na podstawie objawów klinicznych i badań diagnostycznych:²²

Badanie pulsoksymetryczne noworodków może zidentyfikować niemowlęta z D-TGA przed wystąpieniem objawów⁸
Echokardiografia jest podstawowym narzędziem diagnostycznym w rozpoznawaniu TGA¹⁸⁷
EKG może wykazywać narastający przerost prawej komory lub obukomorowy przerost mięśnia sercowego²³
RTG klatki piersiowej zazwyczaj ukazuje serce w kształcie jaja, wąskie śródpiersie górne oraz zwiększony rysunek naczyniowy płuc z kardiomegalią²³

Badania obrazowe odgrywają kluczową rolę w diagnostyce i ocenie TGA. Według niedawno opublikowanych wytycznych, wielomodalny obrazowanie ma istotne znaczenie w diagnostyce, planowaniu chirurgicznym i długoterminowym nadzorze pacjentów z TGA.²⁴

Nadzór długterminowy

Pacjenci z TGA wymagają dożywotniego nadzoru kardiologicznego, niezależnie od rodzaju przeprowadzonej operacji naprawczej:⁸²⁵

Regularne wizyty kontrolne u kardiologa w celu monitorowania postępów i unikania powikłań⁸
Badania obrazowe (echokardiografia, tomografia komputerowa) do oceny zmian morfologicznych w aorcie, tętnicy płucnej i tętnicach wieńcowych²⁶
Testy wysiłkowe do oceny funkcji serca²⁵

Amerykańskie Towarzystwo Kardiologiczne i Amerykańskie Kolegium Kardiologiczne klasyfikują TGA jako złożoną wadę serca i zalecają, aby pacjenci byli pod opieką specjalistycznych ośrodków zajmujących się wrodzonymi wadami serca u dorosłych (ACHD).¹¹²⁷

Systemy nadzoru i rejestracji

W wielu krajach funkcjonują systemy nadzoru epidemiologicznego nad wrodzonymi wadami serca, w tym TGA:²⁸

Zaleca się oddzielne śledzenie prostych form D-TGA ze względu na heterogenność tej wady²⁸
Monitorowanie częstości występowania TGA – bardzo niska częstość (1 na 10 000 urodzeń) może sugerować niedostateczne rozpoznawanie tej wady²⁸
Śledzenie i ocena przypadków D-TGA z ubytkiem przegrody międzykomorowej i bez niego – bardzo niski odsetek przypadków z VSD może wskazywać na niedostateczne raportowanie lub nieprawidłową dokumentację tej często towarzyszącej wady²⁸

Przykładem systemu nadzoru jest program w stanie Minnesota, który śledzi występowanie TGA wśród żywych urodzeń w wybranych hrabstwach od 2005 roku, a obecnie rozszerzył działanie na cały stan. Na podstawie danych z lat 2014-2018 stwierdzono, że 3 niemowlęta na 10 000 urodzeń rodzi się z TGA, co przekłada się na około 19 dzieci rocznie w tym stanie.²⁹

Wyniki leczenia i przeżywalność

Dzięki postępom w diagnostyce i leczeniu chirurgicznym, rokowanie dla pacjentów z TGA znacznie się poprawiło:²²³⁰

Odsetek pacjentów osiągających wiek dorosły po operacji przełożenia tętnic (arterial switch operation, ASO) w D-TGA sięga 90%, co jest znacznie wyższe niż średni odsetek dla krytycznych wrodzonych wad serca (około 69% pacjentów przeżywa do 18 roku życia)³⁰
W jednym z badań długoterminowych wyników u pacjentów po operacji przełożenia tętnic, 25-letnia przeżywalność wynosiła 95%²²
Badania wykazały niższą śmiertelność w pierwszym roku życia u pacjentów z D-TGA rozpoznaną prenatalnie w porównaniu do przypadków rozpoznanych po urodzeniu³⁰

Warto podkreślić, że bez leczenia TGA jest praktycznie niekompatybilna z długoterminowym przeżyciem z powodu niedostatecznego zaopatrzenia organizmu w tlen.³¹ Jednak dzięki współczesnym metodom diagnostycznym i chirurgicznym, większość pacjentów ma doskonałe rokowanie.²²

Badania pokazują, że większość osób z ccTGA nadal dobrze funkcjonuje – jedno badanie wykazało, że trzy na cztery osoby z izolowaną ccTGA były wolne od niewydolności serca w wieku 45 lat.¹¹

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Materiały źródłowe

#1 Transposition of the Great Arteries – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/sites/books/NBK538434/
D-TGA is a CHD occurring in about 1 in 4000 live births. […] TGA accounts for 3% of all CHDs and 20% of cyanotic heart diseases. […] CcTGA is an uncommon and complex CHD, occurring in about 0.05% of all CHD cases, with an estimated incidence of approximately 1 in every 33,000 live births. […] An international study examining the largest cohort of ccTGA cases identified nonsporadic occurrences, including familial clusters. […] These findings suggest a potential common pathogenetic pathway involving laterality genes in the development of ccTGA.
#2 D-transposition of the great arteries (D-TGA): Anatomy, clinical features, and diagnosis – UpToDate
https://www.uptodate.com/contents/d-transposition-of-the-great-arteries-d-tga-anatomy-clinical-features-and-diagnosis/print
D-transposition of the great arteries (TGA) is a ventriculoarterial discordant lesion in which the aorta arises from the right ventricle and the pulmonary artery from the left ventricle. […] The prevalence of TGA in the United States is estimated to be 2 to 5 per 10,000 live births. TGA accounts for approximately 3 percent of all congenital heart disease (CHD) disorders and almost 20 percent of all cyanotic CHD defects.
#3 Transposition of the great arteries | Orphanet Journal of Rare Diseases | Full Text
https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-3-27
Transposition of the great arteries (TGA), also referred to as complete transposition, is a congenital cardiac malformation characterised by atrioventricular concordance and ventriculoarterial (VA) discordance. The incidence is estimated at 1 in 3,500-5,000 live births, with a male-to-female ratio 1.5 to 3.2:1. […] The hearts with atrioventricular concordance and ventriculoarterial discordance represent 57% of all congenital heart diseases, corresponding to an incidence of 20 to 30 per 100,000 live births. There is a male predominance with a male/female sex ratio that varies, in the literature, from 1.5:1 to 3.2:1. […] In 10% of the cases, this cardiac lesion is associated with other noncardiac malformations.
#4 Transposition of the Great Arteries (TGA) | Doctor
https://patient.info/doctor/transposition-of-the-great-arteries
Transposition is the most common cyanotic congenital heart lesion presenting in the neonate. It constitutes 3% of all congenital heart disease (CHD) and 20% of all neonatal cyanotic CHD. […] The overall annual incidence is 1 in 4,000 live births. […] It is more common in males than in females, with a ratio of about 3:1. […] Maternal factors associated with an increased risk include rubella or other viral illness during pregnancy, alcoholism, maternal age over 40 and diabetes. […] Transposition is rarely associated with syndromes or extracardiac malformations.
#5 D-transposition of the great arteries (D-TGA): Anatomy, clinical features, and diagnosis – UpToDate
https://www.uptodate.com/contents/d-transposition-of-the-great-arteries-d-tga-anatomy-clinical-features-and-diagnosis
D-transposition of the great arteries (D-TGA): Anatomy, clinical features, and diagnosis […] EPIDEMIOLOGY […] The prevalence of TGA in the United States is estimated to be 2 to 5 per 10,000 live births. TGA accounts for approximately 3 percent of all congenital heart disease (CHD) disorders and almost 20 percent of all cyanotic CHD defects.
#6 Transposition of the Great Arteries: Background, Pathophysiology, Etiology
https://emedicine.medscape.com/article/900574-overview
Transposition of the great arteries is the most common etiology for cyanotic congenital heart disease in the newborn. This lesion presents in 5-7% of all patients with congenital heart disease. The overall annual incidence is 20-30 per 100,000 live births, and inheritance is multifactorial. Transposition of the great arteries is isolated in 90% of patients and is rarely associated with syndromes or extracardiac malformations. This congenital heart defect is more common in infants of diabetic mothers. […] No racial predilection is known, but transposition of the great arteries has a 60-70% male predominance. […] Patients with transposition of the great arteries usually present with cyanosis in the newborn period, but clinical manifestations and courses are influenced predominantly by the degree of intercirculatory mixing.
#7 Echocardiographic Evaluation of Transposition of the Great Arteries – Congenital Cardiac Anesthesia Society
https://ccasociety.org/education/echoimage/echocardiographic-evaluation-of-transposition-of-the-great-arteries/
Transposition of the great arteries (TGA) is the most common cyanotic cardiac lesion in newborns, accounting for 5-10% of all congenital heart disease (CHD). The malformation occurs in about 20-30 per 100,000 live births and is seen more often in male babies. Without surgical correction, TGA is almost universally fatal during the first year of life. However, contemporary outcome studies have shown that survival rates after surgical intervention are excellent and that most patients live into adulthood. […] The important role of echocardiography in patients with TGA is well established. The recently published guidelines for multimodality imaging have described in detail the significant contributions of echocardiography in the diagnosis, surgical planning, and long-term surveillance of patients with TGA.
#8 About dextro-Transposition of the Great Arteries (d-TGA) | Congenital Heart Defects (CHDs) | CDC
https://www.cdc.gov/heart-defects/about/d-tga.html
About 1 in 3,957 babies in the United States are born with a d-TGA. This means that about 928 babies are born with d-TGA each year. […] d-TGA may be diagnosed during pregnancy or soon after the baby is born. […] d-TGA can also be detected with newborn pulse oximetry screening. Newborn screening using pulse oximetry can identify some infants with d-TGA before they show any symptoms. […] Infants who have surgical repairs for d-TGA are not cured. They may have lifelong complications. A child or adult with d-TGA will need regular follow-up visits with a cardiologist (a heart doctor) to monitor their progress and avoid complications or other health problems.
#9 Dextro-transposition of great vessels: difficult to detect prenatally, one of the most dangerous and one of the best prognosed – SÅodki – Translational Pediatrics
https://tp.amegroups.org/article/view/94564/html
Dextro-transposition of the great arteries (d-TGA) states about 57% of all congenital heart diseases (CHD), 23 times more in boys, approximately about 2030 cases per 100,000 live birth and could be also detected in twin pregnancies. The difference in prevalence varies between countries and regions between 10 to 47 cases per 10,000 births. The explanation of these differences might be lack of the proper data, probably from the cases which were not detected prenatally and postnatally. […] The percentage of prenatal diagnosis of d-TGA has been growing for many years but it still remains below 50% and is much less often detected prenatally than hypoplastic left heart syndrome (HLHS) despite very similar prevalence. In many European countries prenatal detection rate is below 40%, in some even less than 10%.
#10 Congenitally Corrected Transposition of the Great Arteries | SpringerLink
https://link.springer.com/10.1007/978-1-4471-4999-6_46-2
ccTGA is a rare yet complex congenital cardiac anomaly comprising 0.05% (1/33,000 live births) of all congenital heart defects (Wallis et al., Orphanet J Rare Dis 6:22, 2011; Samanek and Voriskova, Pediatr Cardiol 20(6):411417, 1999; Ferencz et al., Am J Epidemiol 121(1):3136, 1985). […] Prenatal echocardiography and enhanced newborn screening promise to lessen the future incidences of childhood and adult ccTGA diagnoses, yet current treatment algorithms following the late diagnosis of ccTGA remain complex and debated. […] Longitudinal, multicenter studies comparing anatomic and physiologic repair techniques remain limited given variations in surgical technique and long-term management, yet anatomic repair has emerged as the preferred treatment strategy to prevent late-term complications of systemic right ventricular dysfunction (Murtuza et al., J Thorac Cardiovasc Surg 142(6):13481357. e1, 2011; Bogers AJ, Head SJ, de Jong PL, Witsenburg M, Kappetein AP (2010) Long term follow up after surgery in congenitally corrected transposition of the great arteries with a right ventricle in the systemic circulation. J Cardiothorac Surg 5:74).
#11 CCTGA – ACHA
https://www.achaheart.org/your-heart/educational-qas/types-of-heart-defects/cctga/
About 0.5%1% of all babies born with heart defects have CCTGA. This means there are about 5,00010,000 people in the United States with CCTGA. […] We do not know enough about what causes CCTGA. The studies we have now suggest that CCTGA is rarely passed from parent to child. […] The American Heart Association and the American College of Cardiology classify CCTGA as a highly complex heart defect. This means that experts recommend that you get your care at a special ACHD center. […] The good news is that most people with CCTGA continue to do well. One study showed that three out of four patients with isolated CCTGA were free from heart failure at age 45.
#12 Congenitally Corrected Transposition of the Great Arteries | Children’s Hospital of Philadelphia
https://www.chop.edu/conditions-diseases/congenitally-corrected-transposition-great-arteries
Congenitally corrected transposition of the great arteries (CCTGA) is a rare heart defect affecting less than one percent of all people with congenital heart disease (CHD). This means there are only about 5,000 to 10,000 people in the United States with this condition. […] CCTGA can be detected before birth by fetal echocardiography. […] The American College of Cardiology has recommended that CCTGA patients be seen regularly at regional ACHD centers.
#13 Transposition of Great Arteries | IntechOpen
https://www.intechopen.com/chapters/77846
DTGA is not associated with any identifiable syndromes or genetic abnormality. […] It is notable that 80 percent of the patients with DiGeorge syndrome display 22q11 deletion and conotruncal lesions, but they rarely suffer from DTGA. […] TGA in family members is uncommon, and the prevalence of CHD in the siblings of affected babies is not different from the general population at 0.3 percent. […] TGA has a documented association with maternal diabetes mellitus, and it is more common in males to females in a 3:1 ratio in the DTGA form. […] The pathogenesis is multifactorial, and a combination of genetic and environmental factors is believed to play a role.
#14 Transposition of the great arteries | Radiology Reference Article | Radiopaedia.org
https://radiopaedia.org/articles/transposition-of-the-great-arteries?embed_domain=hackmd.io%2F%40yIPUAFeCSL2JsU8smR5nJQ%2Fbnjhjgjghjghjghfavicon.icoradiopaedia-icon-144.pngfavicon.ico&lang=gb
Transposition of the great arteries accounts for up to 7% of all congenital cardiac anomalies, with an estimated incidence of ~1 in 5000 births. It is an isolated abnormality in 90% of those affected and rarely is associated with a syndrome or an extra-cardiac malformation. It is most common in infants of diabetic mothers. […] Approximately 90% occur as an isolated finding, and extracardiac syndromic associations are rare. Associations have been described with maternal diabetes, congenital coronary arterial anomalies, coarctation of the aorta, right ventricular outflow tract obstruction, left ventricular outflow tract obstruction, and pulmonary stenosis.
#15
https://step2.medbullets.com/cardiovascular/120001/transposition-of-great-vessels
most common cyanotic lesion presenting immediately after birth […] male female […] present at birth […] maternal diabetes […] maternal smoking […] advanced maternal age
#16
https://step1.medbullets.com/cardiovascular/108030/transposition-of-great-vessels
A neonatal boy is born to a 37-year-old mother via normal spontaneous vaginal delivery. His mother has type 2 diabetes and hypertension. Since birth, he has had trouble feeding, seems to be constantly tachypneic, and has increasingly blue lips. He is immediately given prostaglandin E, and an echocardiogram is obtained, revealing transposition of the great vessels. He is scheduled for surgical repair. […] most common cyanotic lesion presenting immediately after birth […] risk factors maternal diabetes […] maternal smoking […] advanced maternal age.
#17 Transposition of Great Arteries | IntechOpen
https://www.intechopen.com/chapters/77846
TGA is the second most common congenital cyanotic heart defect (CCHD) and the commonest one occurring during the 1st week of life. […] According to an estimation, about 1153 infants are born with TGA annually in the USA. The prevalence is assessed to be 2.34.7 per 10,000 live births. […] TGA accounts for approximately 3 percent of all congenital heart disease (CHD) disorders and almost 20 percent of all cyanotic CHD defects. […] Overall, the incidence of DTGA is 510% of all CHDs, whereas that of LTGA is 1%, and 0.02 to 0.07 per 1000 live births. […] About 90% of the cases present as an isolated defect and the disorder is rarely associated with extracardiac anomalies. […] The occurrence of non-cardiac congenital lesions in TGA at 10% is significantly lower than those in other CHDs.
#18 Transposition of the Great Arteries (TGA) – Pediatrics – Merck Manual Professional Edition
https://www.merckmanuals.com/professional/pediatrics/congenital-cardiovascular-anomalies/transposition-of-the-great-arteries-tga
Dextro-transposition of the great arteries accounts for 5 to 7% of congenital heart anomalies. About 30 to 40% of patients have a ventricular septal defect (VSD); up to 25% have left ventricular outflow tract obstruction (either pulmonic valve or subpulmonary stenosis). […] Diagnosis of transposition of the great arteries is suspected clinically, supported by chest x-ray and ECG, and established by 2-dimensional echocardiography with color flow and Doppler studies. […] In dextro-transposition of the great arteries (d-TGA), the aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle, resulting in independent pulmonary and systemic circulations. […] d-TGA is incompatible with life unless mixing of the circulations occurs through an atrial and/or ventricular septal opening, or a patent ductus. […] Severe cyanosis occurs within hours of birth, followed rapidly by metabolic acidosis; there are no murmurs unless other anomalies are present. […] Do definitive surgical repair during the first week of life.
#19 Transposition of the Great Arteries | Concise Medical Knowledge
https://www.lecturio.com/concepts/transposition-of-the-great-vessels/
Transposition of the great arteries is 3% of all cases of congenital heart disease and 20% of cyanotic congenital heart disease. Prevalence is approximately 4 in 10,000 live births. Associated cardiac anomalies include ventricular septal defect in 50% of cases and left ventricle outflow tract obstruction noted in 25% of cases. […] Coronary arteries have variable origins and short course. […] Mitral or tricuspid valve abnormalities are also associated.
#20 Transposition of the Great Arteries | Pediatric Echocardiography
https://pedecho.org/library/fetal/Fet-DTGA
Transposition of the great arteries (D-TGA) is a conotruncal abnormality characterized by discordant ventricular-arterial connections. In D-TGA, the aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle. The aorta is anterior and rightward. The pulmonary and aortic circulations are in parallel instead of in series. […] D-TGA occurs in ~31.5 in 100,000 live births […] D-TGA is the second most common cyanotic heart disease following tetralogy of Fallot […] Male predominance (2:1 male to female ratio) […] Fetal Surveillance: Assess atrial communication, ductal shunting and outflow tracts, valvar regurgitation, aortic arch if concern for hypoplasia/coarctation. If VSD, assess size of VSD and outflow tracts and pulmonary/aortic valves for evidence of hypoplasia/stenosis.
#21 Transposition of the Great Arteries (TGA) | Obstetrics & Gynecology
https://www.obgyn.columbia.edu/patient-care/our-centers/center-prenatal-pediatrics/conditions-we-care/transposition-great-arteries-tga
TGA occurs in approximately two to four out of every 10,000 live births. […] Prenatal consultations with pediatric cardiology, pediatric cardiothoracic surgery, and neonatology specialists are recommended to discuss the prognosis and management after birth. […] All newborns with TGA require corrective surgery, which typically occurs within the first week of life.
#22 Transposition of the Great Arteries | Condition Overview | Columbia Surgery
https://columbiasurgery.org/conditions-and-treatments/transposition-of-the-great-arteries
Transposition of the great arteries (TGA) is a rare and complex congenital heart defect in which the two main arteries that carry blood from the heart are switched (transposed). This prevents oxygen-rich blood from being pumped to the rest of the body. Infants born with this condition must receive corrective surgery immediately after birth. […] TGA can be diagnosed during pregnancy or shortly after birth, depending on the severity of the condition and the presence of other heart defects. […] TGA can be diagnosed either during pregnancy or shortly after birth. […] After birth, if the baby is showing any signs of TGA, such as blue skin, lips, or fingernails or rapid breathing, a diagnosis may be made using a number of methods: […] The long-term outlook for TGA is generally excellent. With proper treatment, most patients are able to gain normal heart function and lead healthy, active lives. In one study of long-term outcomes for patients who have undergone arterial switch operations, the most common treatment for TGA, the 25-year survival rate was 95%.
#23 Transposition of the Great Arteries (TGA) – Cardiothoracic Surgery
https://lsom.uthscsa.edu/ct-surgery/patient-care/congenital-heart/conditions-we-treat/transposition-great-arteries-tga/
This is the most common of the cyanotic heart diseases. In transposition of the great arteries, the aorta arises from the right ventricle (RV) and the pulmonary artery arises from the left ventricle (LV). Survival is dependent upon mixing, usually by an atrial level connection or ASD. Up to 50% of patients will also have a VSD. TGA is the most common congenital heart defect requiring early intervention. Currently, anatomic correction is performed early in life using the arterial switch operation. […] The EKG will show increasing RVH or biventricular hypertrophy over time. CXR shows an egg-shaped heart, narrow superior mediastinum, and increased pulmonary vascular markings with cardiomegaly. […] For infants with hemodynamic instability or persistent hypoxemia or acidemia shortly after birth, balloon atrial septostomy, performed percutaneously in the cardiac catheterization lab, may be employed to improve mixing.
#24 Multimodality Imaging Guidelines of Patients with Transposition of the Great Arteries: A Report from the American Society of Echocardiography Developed in Collaboration with the Society for Cardiovascular Magnetic Resonance and the Society of Cardiovascul
https://www.asecho.org/guideline/multimodality-imaging-guidelines-of-patients-with-transposition-of-the-great-arteries-a-report-from-the-american-society-of-echocardiography-developed-in-collaboration-with-the-society-for-cardiovasc/
Transposition of the great arteries (TGA) is a congenital heart defect with ventriculoarterial discordance in which the aorta is aligned with the right ventricle and the pulmonary artery is aligned with the left ventricle. […] The clinical diagnosis and management of patients with TGA has improved dramatically over the past three decades because of the evolution and availability of multiple imaging modalities and strides made in the surgical management of these patients. […] Despite these technical advances, patients with TGA require longterm surveillance because of ongoing anatomic and hemodynamic abnormalities. […] The purpose of this report is to present guidelines for multimodality imaging in this cohort of patients.
#25 Transposition of the Great Arteries | Condition Overview | Columbia Surgery
https://columbiasurgery.org/conditions-and-treatments/transposition-of-the-great-arteries
However, children who receive surgical repair for TGA will require lifelong care and supervision by a cardiologist. Regular follow-ups may include imaging tests to check for any possible defects or complications of the heart as well as exercise stress tests to determine how well the heart is functioning.
#26 Pulmonary, aorta, and coronary arteries post-arterial switch in transposition of great arteries: intermediate-term surveillance utilizing conventional echocardiography and cardiac multislice computed tomography | Italian Journal of Pediatrics | Full Text
https://ijponline.biomedcentral.com/articles/10.1186/s13052-024-01686-x
Dextro-transposition of the great arteries (DTGA) is the second most prevalent cyanotic congenital heart disease (CHD). Globally, it accounts for 3.82% of all CHDs, with a prevalence of about 0.3 per 1000 live births. The concerns regarding the arteries fate, neo-pulmonary, and neo-aortic valvular functions warrant persistent surveillance of these patients. Hence, regular follow-up echocardiography (ECHO) post-ASO is currently the standard surveillance tool in most centers. […] Echocardiography alone is not a conclusive surveillance tool for detecting late post-ASO anatomic changes in D-TGA patients. Cardiac MSCT should be considered for comprehensive evaluation on the intermediate-term follow-up post-ASO to accurately track morphologic abnormalities in the aorta, pulmonary, and coronary arteries.
#27 Transposition of the Great Arteries after Arterial Switch or Rastelli Procedure – ACHA
https://www.achaheart.org/your-heart/educational-qas/types-of-heart-defects/transposition-of-the-great-arteries-after-arterial-switch-or-rastelli-procedure/
Transposition of the great arteries (TGA or d-TGA) is a complex congenital heart defect. The two main arteries, the pulmonary artery and aorta, are reversed. It is more common than the other type of TGA CCTGA (also known as l-TGA). […] TGA occurs in between 1 in 3,500 and 5,000 births. It is more common in males than females. […] Both types of repairs have potential problems that require lifelong congenital cardiology follow up. […] The American College of Cardiology and the American Heart Association classify d-TGA as a complex heart defect. This means that experts recommend that you get care for your heart at a special ACHD center.
#28 4.5 Transposition of Great Arteries (Q20.3) | CDC
https://archive.cdc.gov/www_cdc_gov/ncbddd/birthdefects/surveillancemanual/chapters/chapter-4/chapter4-5c.html
d(dextro)-transposition of the great arteries (d-TGA) is a structural heart anomaly characterized clinically by cyanosis (usually) and anatomically by an abnormal origin of the great arteries, such that the aorta exits from the right ventricle (instead of the left) and the pulmonary artery exits from the left ventricle (instead of the right) (see Fig. 4.16). […] Because of this heterogeneity, it is recommended that public health surveillance track separately the simple forms of d-TGA. […] d-TGA occurs with a frequency of approximately 1 in 3000 to 4000 births, and is more common in males. […] For public health surveillance, l-TGA should not be coded with Q20.3 in order to track d-TGA appropriately. […] Track and evaluate cases of d-TGA with and without ventricular septal defect: A very low proportion of cases with ventricular septal defect might indicate that this commonly associated intracardiac anomaly is being underreported or not documented and coded correctly. […] Monitor prevalence: If very low (1 per 10 000 births) it suggests under-ascertainment.
#29 Transposition of the Great Arteries (also called transposition of the great vessels) – MN Dept. of Health
https://www.health.state.mn.us/diseases/cy/transposition.html
Transposition occurs more frequently in mothers who are diabetic, although this risk decreases if the mother’s diabetes is carefully controlled during the pregnancy. […] Our program has been tracking transposition of the great arteries among live births in select counties since 2005 and we now have expanded statewide. […] Using data from births statewide between 2014-2018, we found that 3 babies were born with transposition of the great arteries per 10,000 births. […] Using this data, we estimate about 19 babies are born with transposition of the great arteries every year in Minnesota.
#30 Dextro-transposition of great vessels: difficult to detect prenatally, one of the most dangerous and one of the best prognosed – SÅodki – Translational Pediatrics
https://tp.amegroups.org/article/view/94564/html
The study of van Velzen et al. is one of these which have proofed lower first-year mortality in prenatally diagnosed d-TGA than in those without prenatal diagnosis. In their study group presurgical mortality was present only in postnatal detected d-TGA cases. The percentage of patients reaching to adulthood after the arterial switch operation (ASO) in d-TGA reaches 90% of patients and is much higher than an average percentage for critical CHD, which is about 69% for patients who survive to 18 years of age.
#31 Transposition of the great arteries | PPT
https://www.slideshare.net/slideshow/transposition-of-the-great-arteries-232471856/232471856
Transposition of the great arteries is a serious but rare heart defect present at birth (congenital), in which the two main arteries leaving the heart are reversed (transposed). The condition is also called dextro-transposition of the great arteries. […] Transposition of the Great Arteries Most common form of cyanotic congenital heart disease (5 to 7% of all congenital heart defects) which presents in the newborn period. More common in males. […] In transposition of the great arteries, the aorta is connected to the right ventricle, and the pulmonary artery is connected to the left ventricle – the exact opposite of a normal heart’s anatomy. […] Because of the low amount of oxygen provided to the body, TGA is a heart problem that is labeled „blue-baby syndrome.” […] Transposition of the great arteries accounts for 5-7% of congenital heart diseases and has an annual incidence of 20-30 per 100,000 live births. […] Without treatment, TGA is incompatible with long-term survival due to lack of oxygen supply.