Transpozycja wielkich naczyń

Transpozycja wielkich naczyń
Objawy

Transpozycja wielkich naczyń (TGA) to poważna wrodzona wada serca charakteryzująca się zamianą miejsc aorty i tętnicy płucnej, co prowadzi do rozdzielenia krążenia systemowego i płucnego. Objawy kliniczne, takie jak sinica, tachypnoe, słabe tętno i trudności z karmieniem, pojawiają się zwykle w pierwszych godzinach lub dniach życia, zwłaszcza w przypadku D-TGA. Stopień sinicy zależy od obecności dodatkowych wad serca umożliwiających mieszanie się krwi, takich jak PDA, ASD czy VSD. Nieleczona TGA wiąże się z wysoką śmiertelnością: około 30% pacjentów umiera w pierwszym tygodniu, 50% w pierwszym miesiącu, a 90% przed końcem pierwszego roku życia. Diagnostyka prenatalna i wczesna po urodzeniu, w tym echokardiografia, są kluczowe dla szybkiego rozpoznania i wdrożenia leczenia, które często obejmuje septostomię balonową przedsionkową (BAS) w celu poprawy mieszania krwi.

Symptomy Transpozycji wielkich naczyń

Transpozycja wielkich naczyń (TGA) jest poważną wrodzoną wadą serca, w której dwie główne tętnice wychodzące z serca – aorta i tętnica płucna – są zamienione miejscami. Powoduje to niebezpieczne zaburzenie cyrkulacji krwi, w którym krew utlenowana i nieutlenowana krążą w odrębnych obiegach, co prowadzi do poważnych konsekwencji zdrowotnych. Objawy tej wady serca można zaobserwować już w okresie prenatalnym lub krótko po urodzeniu, w zależności od typu transpozycji i współistniejących wad serca.¹²

Wczesne objawy u noworodków

Większość noworodków z transpozycją wielkich naczyń wykazuje objawy w ciągu pierwszych godzin lub dni życia. Nasilenie objawów zależy od stopnia mieszania się krwi między dwoma obiegami oraz obecności dodatkowych wad serca.¹²

Najczęstszym i najbardziej charakterystycznym objawem jest sinica – niebieskie lub szare zabarwienie skóry, warg i paznokci, spowodowane niewystarczającym natlenowaniem organizmu. Sinica jest widoczna w pierwszych godzinach życia u około połowy niemowląt z TGA, a w ciągu pierwszych dni życia u około 90% z nich.¹² Stopień sinicy zależy od obecności innych wad pozwalających na mieszanie się krwi, takich jak przetrwały przewód tętniczy (PDA), ubytek przegrody międzyprzedsionkowej (ASD) lub ubytek przegrody międzykomorowej (VSD).¹

Inne częste objawy obejmują:¹²³

Problemy z oddychaniem (tachypnoe, duszność)
Słabe tętno
Trudności z karmieniem
Słaby przyrost masy ciała
Przyspieszona akcja serca
Chłodna, wilgotna skóra

Wpływ dodatkowych wad serca na objawy

Nasilenie objawów TGA jest ściśle związane z obecnością dodatkowych wad serca, które mogą umożliwiać mieszanie się krwi utlenowanej z nieutlenowaną:¹²

TGA z nienaruszonym przegrodzeniem międzykomorowym – wyraźna i postępująca sinica w ciągu pierwszych 24 godzin życia jest typowym objawem u niemowląt, jeśli nie występuje znaczące mieszanie się krwi na poziomie przedsionków.¹
TGA z dużym ubytkiem przegrody międzykomorowej (VSD) – niemowlęta mogą początkowo nie wykazywać objawów choroby serca, choć łagodna sinica (szczególnie podczas płaczu) jest często zauważalna. Objawy zastoinowej niewydolności serca (tachypnea, tachykardia, pocenie się i brak przyrostu masy ciała) mogą pojawić się w ciągu pierwszych 3-6 tygodni życia.¹²
TGA z VSD i zwężeniem drogi odpływu lewej komory – niemowlęta często wykazują skrajną sinicę przy urodzeniu, proporcjonalną do stopnia zwężenia drogi odpływu lewej komory (płucnej). Historia kliniczna może przypominać historię niemowlęcia z tetralogią Fallota.¹
TGA z VSD i naczyniową chorobą obturacyjną płuc – postępująca naczyniowa choroba obturacyjna płuc może zapobiec rozwojowi objawów zastoinowej niewydolności serca, pomimo dużego ubytku przegrody międzykomorowej. Najczęściej pacjenci wykazują postępującą sinicę.¹

Przebieg kliniczny bez leczenia

Nieleczona transpozycja wielkich naczyń ma dramatyczny przebieg kliniczny i wysoką śmiertelność:¹²

Około 30% nieleczonych pacjentów umiera w pierwszym tygodniu życia
Około 50% umiera w pierwszym miesiącu
Około 90% umiera przed końcem pierwszego roku życia

W pierwszych godzinach po urodzeniu występuje ciężka sinica, a następnie szybko rozwija się kwasica metaboliczna spowodowana niedotlenieniem tkanek.¹ Bez interwencji chirurgicznej, w miarę zamykania się przewodu tętniczego (który normalnie zamyka się w pierwszych dniach po urodzeniu), sinica staje się bardziej nasilona, co prowadzi do pogłębiającego się niedotlenienia.¹

Fizyczne objawy podmiotowe i przedmiotowe

Podczas badania fizykalnego u noworodków z TGA można zaobserwować:¹²

Uogólnioną sinicę
Szmer sercowy może być nieobecny, chyba że występują wady współistniejące
Drugi ton serca (S2) może być pojedynczy i głośny
Z czasem mogą pojawić się objawy niewydolności serca
U niektórych dzieci może wystąpić palcowanie pałeczkowate (clubbing) palców rąk i stóp jako późny objaw przewlekłej hipoksemii¹

Warianty transpozycji i ich objawy

D-TGA (dekstro-transpozycja wielkich naczyń)

D-TGA (dekstro-transpozycja wielkich naczyń) jest najczęstszym typem TGA i charakteryzuje się całkowitym odwróceniem pozycji aorty i tętnicy płucnej. W tym typie dochodzi do całkowitego rozdzielenia krążenia systemowego i płucnego.¹²

Objawy D-TGA są zazwyczaj widoczne zaraz po urodzeniu lub w ciągu pierwszych dni życia i obejmują:¹²

Nasiloną sinicę
Zaburzenia oddychania
Problemy z karmieniem
Kołatanie serca
Słabe tętno

Ciężkość objawów zależy od tego, czy krew bogata w tlen może docierać do reszty organizmu dzięki mieszaniu się w górnych komorach serca. U niektórych noworodków objawy mogą nie być widoczne od razu, ponieważ małe otwory wokół serca nie zamknęły się jeszcze. Gdy otwory się zamkną, objawy pojawiają się i niemowlę wymaga natychmiastowej pomocy medycznej.¹

L-TGA (lewo-transpozycja wielkich naczyń)

L-TGA (lewo-transpozycja wielkich naczyń) charakteryzuje się odwróceniem pozycji komór serca. W przeciwieństwie do D-TGA, objawy L-TGA zazwyczaj pojawiają się później w dzieciństwie lub nawet w wieku dorosłym.¹²

Objawy L-TGA mogą obejmować:¹²³

Trudności z oddychaniem
Omdlenia lub zawroty głowy
Niestabilne lub wolne bicie serca
Szmer sercowy
Zmęczenie
Kołatanie serca

Jeśli występują inne problemy, takie jak ubytek w sercu lub nieprawidłowa zastawka czy naczynie krwionośne, u dziecka może wystąpić szmer sercowy lub inne problemy, które skierują je do opieki medycznej już we wczesnym dzieciństwie.¹ Z czasem L-TGA może prowadzić do niewydolności serca, gdy komora pompująca staje się większa, a mięsień sercowy słabnie.¹

Progresja choroby i powikłania długoterminowe

Powikłania u niemowląt bez operacji

Bez leczenia chirurgicznego transpozycja wielkich naczyń prowadzi do poważnych powikłań:¹²

Niedotlenienie tkanek – jeśli nie dochodzi do mieszania się krwi bogatej w tlen i ubogiej w tlen, powikłanie to prowadzi do śmierci¹
Niewydolność serca – może rozwinąć się z czasem, ponieważ prawa dolna komora serca pompuje pod wyższym ciśnieniem niż zwykle. Obciążenie może sprawić, że mięsień prawej dolnej komory stanie się sztywny lub słaby¹
Kwasica metaboliczna – rozwija się szybko po urodzeniu z powodu niedotlenienia tkanek¹
Policytemia i zespół nadlepkości – długoterminowe powikłania wtórne do przedłużającej się sinicy¹

Powikłania długoterminowe po leczeniu chirurgicznym

Po operacji naprawczej TGA pacjenci mogą rozwinąć różne powikłania długoterminowe, w zależności od rodzaju wykonanego zabiegu:¹²³

Po operacji przełożenia przedsionkowego (Mustard/Senning)

Zaburzenia rytmu serca – arytmie często rozwijają się z czasem i mogą prowadzić do nagłej śmierci sercowej (7-15% wszystkich pacjentów)¹²
Niewydolność serca – pacjenci, którzy przeszli operację przełożenia przedsionkowego, są narażeni na zwiększone ryzyko niewydolności serca w wieku dorosłym, ponieważ zabieg sprawia, że prawa komora pracuje ciężej niż powinna. Badania pokazują, że około 10% pacjentów po operacji Mustard/Senning doświadcza niewydolności serca¹²
Problemy z przegrodami (bafflami) – z czasem przegrody stworzone podczas operacji Mustard/Senning mogą rozwinąć problemy, takie jak przecieki lub zwężenia¹²
Osłabienie funkcji prawej komory – ponieważ prawa komora pozostaje główną komorą pompującą, z czasem może ulec osłabieniu¹

Po operacji przełożenia tętniczego (Jatne/arterial switch)

Zwężenie tętnic – niektórzy pacjenci mogą wymagać dodatkowych zabiegów w celu usunięcia zwężenia w aorcie lub tętnicy płucnej w miejscu pierwotnej operacji¹²
Nieszczelne zastawki – z czasem może dojść do niedomykalności zastawek¹²
Arytmie – ryzyko jest mniejsze niż w przypadku operacji przełożenia przedsionkowego¹
Problemy z tętnicami wieńcowymi – mogą prowadzić do niedokrwienia mięśnia sercowego¹
Poszerzenie aorty – aorta może rozszerzać się (powiększać) w miejscu, w którym została ponownie dołączona do lewej komory¹

Powikłania wspólne dla wszystkich typów operacji

Infekcyjne zapalenie wsierdzia – zakażenie, które występuje, gdy bakterie wnikają do serca. Jest bardziej powszechne u osób z wadami serca, takimi jak TGA¹
Konieczność dodatkowych operacji – niektórzy pacjenci mogą wymagać dodatkowych zabiegów w późniejszym życiu¹
Całkowity blok serca – szczególnie częsty w L-TGA¹²

Prognozy długoterminowe

Dzięki postępom w leczeniu chirurgicznym, długoterminowe prognozy dla pacjentów z TGA znacznie się poprawiły:¹²

Wskaźnik przeżycia po operacji przełożenia tętniczego wynosi ponad 95%¹
Większość dzieci po operacji TGA może prowadzić pełne, zdrowe życie i zazwyczaj może uczestniczyć w normalnych codziennych czynnościach, w tym w sportach, bez ograniczeń¹
Badania wykazały, że 88% pacjentów po operacji przełożenia tętniczego (Jatene) przeżyło 10 lat, bez dalszych zgonów między 10 a 15 rokiem po operacji¹

Jednak wszyscy pacjenci z TGA wymagają dożywotniej opieki kardiologicznej, aby monitorować postępy i unikać innych problemów zdrowotnych lub powikłań.¹² Regularne wizyty kontrolne są niezbędne, ponieważ nowe problemy z sercem mogą rozwijać się bez objawów.¹

Ciąża u kobiet z TGA

U niewielkiej liczby kobiet z TGA podczas ciąży mogą wystąpić nowe problemy związane z niewydolnością serca i/lub rytmem serca.¹ Jednak dla większości osób, które przeszły operację naprawy TGA metodą przełożenia tętniczego, ciąża jest bezpieczna.¹

Ryzyko urodzenia dziecka z wrodzoną wadą serca dla każdej osoby wynosi około 1%. U dzieci urodzonych przez rodzica z wrodzoną wadą serca, taką jak TGA, szansa, że dziecko będzie miało jakikolwiek rodzaj wrodzonej wady serca, wynosi około 3%.¹

Rozpoznanie i różnice diagnostyczne

Transpozycja wielkich naczyń może być zdiagnozowana:¹²

Prenatalnie – podczas rutynowego badania ultrasonograficznego ciąży, choć może zostać przeoczona i czasami potrzebne jest badanie echokardiograficzne płodu wykonane przez specjalistów¹
Po urodzeniu – diagnoza stawiana jest zwykle w pierwszych godzinach lub dniach życia z powodu sinicy lub niskiego poziomu tlenu. Wszystkie noworodki mają przy urodzeniu przetrwały przewód tętniczy (PDA), który może umożliwiać wystarczające mieszanie się krwi, aby zapobiec ciężkiej sinicy. Gdy przewód tętniczy zamyka się, co nastąpi w pierwszych godzinach lub dniach życia, sinica staje się bardziej nasilona¹

Objawy TGA mogą przypominać objawy innych stanów, dlatego ważne jest, aby dziecko zostało zbadane przez lekarza w celu postawienia diagnozy.¹ Objawom tym mogą towarzyszyć także inne problemy zdrowotne, które wymagają dokładnej diagnozy różnicowej.¹

Około jedna trzecia niemowląt urodzonych z TGA ma wyjątkowo niskie poziomy tlenu, które mogą zaszkodzić ich organizmom i będą wymagały pilnej interwencji, zwanej septostomią balonową przedsionkową (BAS), w ciągu kilku godzin od urodzenia.¹

Podsumowanie przebiegu klinicznego

Transpozycja wielkich naczyń jest poważną wrodzoną wadą serca wymagającą szybkiej interwencji medycznej. Objawy u większości pacjentów z D-TGA pojawiają się w pierwszych dniach życia i obejmują głównie sinicę oraz trudności z oddychaniem. Z kolei pacjenci z L-TGA mogą nie doświadczać objawów przez wiele lat, aż do rozwoju niewydolności serca w wieku dorosłym.¹²

Bez leczenia chirurgicznego perspektywy są złe, z 90% śmiertelnością w pierwszym roku życia. Dzięki nowoczesnym technikom chirurgicznym, w szczególności operacji przełożenia tętniczego, ponad 95% pacjentów przeżywa do wieku dorosłego.¹²

Wszyscy pacjenci z TGA wymagają dożywotniej opieki kardiologicznej, aby monitorować potencjalne powikłania, takie jak arytmie, niewydolność serca, problemy z zastawkami czy zwężenia tętnic.¹² Dzięki odpowiedniemu leczeniu i regularnej opiece medycznej, większość osób z TGA może prowadzić aktywne, pełnowartościowe życie.¹

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Materiały źródłowe

#1 Transposition of the great arteries – Symptoms and causes – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/transposition-of-the-great-arteries/symptoms-causes/syc-20350589
Transposition of the great arteries (TGA) may be seen in a baby before birth during a routine pregnancy ultrasound. […] Symptoms of transposition of the great arteries after birth include: Blue or gray skin. Depending on the baby’s skin color, these color changes may be harder or easier to see. Weak pulse. Lack of appetite. Poor weight gain. […] Skin color changes may not be noticed right away if a baby with TGA also has other heart problems. This is because the other heart problems may let some oxygen-rich blood move through the body. But as the baby becomes more active, less blood flows through the body. The blue or gray skin color then becomes more noticeable. […] Symptoms are usually noticed during pregnancy, immediately after birth or within a few weeks of birth. Without treatment, serious complications or death can occur. […] But some people with the congenitally corrected type of TGA may not have symptoms for many years.
#1 Transposition of the Great Arteries | Children’s Hospital of Philadelphia
https://www.chop.edu/conditions-diseases/transposition-great-arteries
Transposition of the great arteries (TGA) is a complex congenital heart defect. The symptoms of TGA include: Blue or purple tint to lips, skin and nails (cyanosis), Rapid breathing, Difficulty feeding, poor appetite and poor weight gain. Sometimes TGA is diagnosed a few hours or days after a baby is born. Rarely, a baby may not have visible symptoms for weeks or months. About one-third of babies born with TGA have extremely low oxygen levels that can harm their bodies and will require an urgent intervention, called a balloon atrial septostomy (BAS), within hours of birth. All children with transposition of the great arteries will require open heart surgery to treat the defect. Without surgical repair, most babies with TGA will not survive their first year of life. Most children born with TGA will go on to lead healthy, productive lives, and are usually able to participate in normal day-to-day activities, including sports, without limitations. Children who have had surgical repair of TGA require lifelong care by a cardiologist.
#1 Transposition of the Great Arteries | TGA Heart | Children’s Wisconsin
https://childrenswi.org/medical-care/herma-heart/conditions/transposition-of-the-great-arteries
The obvious TGA symptom is a newborn who becomes cyanotic (blue) in the transitional first day of life when the maternal source of oxygen (from the placenta) is removed. Cyanosis is noted in the first hours of life in about half of the infants with TGA, and within the first days of life in 90 percent of them. The degree of cyanosis is related to the presence of other defects that allow blood to mix, including a patent ductus arteriosus – a fetal connection between the aorta and the pulmonary artery present in the newborn, which usually closes in the first few days after birth. […] The following are the other most common TGA heart symptoms. However, each child may experience symptoms differently. Symptoms may include: Rapid breathing, Labored breathing, Rapid heart rate, Cool, clammy skin.
#1 Transposition of the Great Arteries Signs & Symptoms | Rush | Rush System
https://www.rush.edu/kids/conditions/transposition-great-arteries
Transposition of the great arteries (TGA), also referred to as dextro-transposition of the great arteries or D-TGA, is a congenital heart defect in which the two major vessels that carry blood away from the heart the aorta and the pulmonary artery are switched. It is a relatively rare condition, affecting 20 to 30 infants per 100,000 live births. […] TGA symptoms occur at birth or soon after. Babies with TGA may have a condition called cyanosis, which is bluish looking skin caused by their blood not carrying enough oxygen. Other symptoms may include the following: Problems breathing, Pounding heart, Weak pulse, Trouble eating. […] All babies born with TGA must have surgery to switch and reattach the aorta and pulmonary arteries and reconnect the coronary arteries. Other procedures may be done before surgery to maintain, enlarge or create openings that will allow oxygen-rich blood to get out to the body. […] People born with TGA have lifelong complications. Your child will need regular follow-up visits with a cardiologist to monitor their progress and avoid other health problems or complications.
#1 Transposition of the Great Arteries Clinical Presentation: History, Physical Examination
https://emedicine.medscape.com/article/900574-clinical
Infants with transposition of the great arteries (TGA) are usually born at term, with cyanosis apparent within hours of birth. […] The clinical course and manifestations depend on the extent of intercirculatory mixing and the presence of associated anatomic lesions. Note the following: […] Transposition of the great arteries with intact ventricular septum: Prominent and progressive cyanosis within the first 24 hours of life is the usual finding in infants if no significant mixing at the atrial level is evident. […] Transposition of the great arteries with large ventricular septal defect: Infants may not initially manifest symptoms of heart disease, although mild cyanosis (particularly when crying) is often noted. Signs of congestive heart failure (tachypnea, tachycardia, diaphoresis, and failure to gain weight) may become evident over the first 3-6 weeks as pulmonary blood flow increases.
#1 Transposition of the Great Arteries Clinical Presentation: History, Physical Examination
https://emedicine.medscape.com/article/900574-clinical
Transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction: Infants often present with extreme cyanosis at birth, proportional to the degree of left ventricular (pulmonary) outflow tract obstruction. The clinical history may be similar to that of an infant with tetralogy of Fallot. […] Transposition of the great arteries with ventricular septal defect and pulmonary vascular obstructive disease: Progressively advancing pulmonary vascular obstructive disease can prevent this rare subgroup of patients from developing symptoms of congestive heart failure, despite a large ventricular septal defect. Most often, patients present with progressive cyanosis, despite an early successful palliative procedure.
#1 Transposition of the Great Arteries: Background, Pathophysiology, Etiology
https://emedicine.medscape.com/article/900574-overview
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. […] 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.
#1 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
Severe cyanosis occurs within hours of birth, followed rapidly by metabolic acidosis secondary to poor tissue oxygenation. […] Patients with a moderate or large atrial septal defect, a large ventricular septal defect, a patent ductus arteriosus, or a combination of these tend to have less severe cyanosis, but symptoms and signs of heart failure (eg, tachypnea, dyspnea, tachycardia, diaphoresis, inability to gain weight) may develop during the first weeks of life. […] Except for generalized cyanosis, physical examination is rather unremarkable. Heart murmurs may be absent unless associated anomalies are present. The second heart sound (S2) is single and loud. […] Severe cyanosis occurs within hours of birth, followed rapidly by metabolic acidosis; there are no murmurs unless other anomalies are present.
#1 Transposition of the Great Arteries (TGA) – RCEMLearning
https://www.rcemlearning.co.uk/modules/congenital-heart-disease-in-the-emergency-department/lessons/righttoleft-shunts/topic/transposition-of-the-great-arteries-tga/
Second most common cause of cyanotic CHD diagnosed by 1 year of age. […] The presentation for infants with TGA depends on the degree of blood mixing. […] These infants present with cyanosis at birth because of right-to-left shunting, and the cyanosis becomes more severe when the ductus arteriosus closes. […] Cyanosis is the predominant symptom. Presentation is usually on day 2 of life when ductal closure leads to a marked reduction in mixing of the desaturated and saturated blood. […] Cyanosis may be less severe and presentation is delayed if there is more mixing of blood from associated anomalies, e.g. an ASD. […] In the sick cyanosed neonate, the key is to improve mixing. The outcome depends on the degree of blood mixing, the magnitude of tissue hypoxia, and the ability of the right ventricle to maintain the systemic circulation. Without surgery, most patients die within months.
#1 HIE Multimedia – Transposition of the great arteries
https://adamcertificationdemo.adam.com/content.aspx?productid=117&pid=1&gid=001568
Transposition of the great arteries (TGA) is a heart defect that occurs from birth (congenital). The two major arteries that carry blood away from the heart — the aorta and the pulmonary artery — are switched (transposed). […] TGA is a cyanotic heart defect. This means there is decreased oxygen in the blood that is pumped from the heart to the rest of the body. […] Symptoms appear at birth or very soon afterward. How bad the symptoms are depends on the type and size of additional heart defects (such as atrial septal defect, ventricular septal defect, or patent ductus arteriosus) and how much the blood can mix between the two abnormal circulations. […] Symptoms may include: Bluish color to the skin (cyanosis) due to low oxygen level in the blood, Clubbing of the fingers or toes, Poor feeding, Shortness of breath. […] The baby’s mouth and skin will be a blue color. […] 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.
#1 Transposition of the Great Arteries | Condition Overview | Columbia Surgery
https://columbiasurgery.org/conditions-and-treatments/transposition-of-the-great-arteries
Complete transposition of the great arteries, or dextro-transposition of the great arteries (D-TGA), is the most common type of TGA, in which the pulmonary artery and aorta are switched entirely in position. This means that not enough oxygen is being delivered to the rest of the body, resulting in a number symptoms, including: […] For D-TGA, surgery is the only treatment for survival. […] Infants with L-TGA may go longer without needing corrective heart surgery.
#1 Transposition of the Great Arteries (TGA)
https://my.clevelandclinic.org/health/diseases/23387-transposition-of-the-great-arteries
Transposition of the great arteries symptoms include: […] Newborns with d-TGA have symptoms of cyanosis (low oxygen) and may also have heart failure. […] Trouble breathing. […] Weak pulse. […] Difficulty feeding. […] Pounding heart. […] Skin or whites of their eyes appear bluish (in light-skinned babies) or grayish (in dark-skinned babies). […] In a baby with the d type of TGA heart problem, signs appear soon after birth. Their severity depends on whether oxygen-rich blood can reach the rest of your babys body by mixing in the upper chambers of their heart. […] Some newborns dont show symptoms right away because small openings around their hearts havent closed yet. Once the holes close, symptoms appear, and your baby requires immediate medical attention. […] While surgery doesnt cure d-transposition of the great arteries, most people with the condition can lead full, healthy lives.
#1 Levo-Transposition of the Great Arteries (for Parents) | Nemours KidsHealth
https://kidshealth.org/en/parents/l-tga.html
Levo-transposition of the great arteries (L-TGA) is when the positions of the hearts ventricles are reversed. Some people with the condition never need treatment. Others develop symptoms and need treatment with medicines and surgery. […] Symptoms usually show up later in childhood or in adulthood. These may include trouble breathing, fainting, or lightheadedness. Sometimes a person may have an unsteady or slow heartbeat. When there are other issues, such as a hole in the heart or an abnormal valve or blood vessel, the baby may have a heart murmur or other issues that bring them to medical attention early in life. […] Over time, this can lead to heart failure when the pumping chamber gets larger, and the heart muscle becomes weak.
#1 Transposition of the great arteries – Hancock Health
https://www.hancockhealth.org/mayo-health-library/transposition-of-the-great-arteries/
Always seek emergency medical help if you notice that anyone develops a blue or gray skin color. […] Possible complications of complete transposition of the great arteries (D-TGA) may include: Not enough oxygen to body tissues. Unless theres some mixing of oxygen-rich blood and oxygen-poor blood within the body, this complication causes death. Heart failure. Heart failure is a condition in which the heart cant pump enough blood to meet the bodys needs. It may develop over time because the right lower heart chamber is pumping under higher pressure than usual. The strain may make the muscle of the right lower chamber stiff or weak. […] Possible complications of congenitally corrected transposition (L-TGA) may include: Reduced heart pumping. In L-TGA, the right lower heart chamber pumps blood to the body. This work is different from what that chamber was designed to do. This can cause changes in how well the heart pumps blood. Complete heart block. The changes in the structure of the heart due to L-TGA can change the electrical signals that tell the heart to beat. A complete heart block occurs if all signals are blocked. Heart valve disease. In L-TGA, the valve between the upper and lower heart chambers the tricuspid valve may not close completely. Blood might move backward through the valve. This condition is called tricuspid valve regurgitation. It can eventually reduce the hearts ability to pump blood. […] After birth, a health care provider may think about a diagnosis of a TGA if the baby has blue or gray skin, a weak pulse, or trouble breathing. The care provider may hear a heart sound, called a murmur, while listening to the babys heart.
#1 D-Transposition of the Great Arteries > Fact Sheets > Yale Medicine
https://www.yalemedicine.org/conditions/d-transposition-of-the-great-arteries
Symptoms include bluish skin, difficulty breathing, poor feeding, pounding heart, weak pulse. […] Newborns experience the following signs and symptoms within the first days of life, including: Bluish skin, Difficulty breathing, Poor feeding habits, A pounding heart, A weak pulse. […] Without surgical intervention, 90% of babies born with d-transposition of the great arteries will die during their first year of life; half will die within the first month. With surgery, over 95% of patients will live into adulthood. […] Patients who have an atrial switch procedure are at increased risk of congestive heart failure during adulthood because the procedure makes the right ventricle work harder than it should. […] Some patients with d-transposition of the great arteries develop arrhythmias (heart rhythm problems) over time. This is less of a risk in patients with the arterial switch procedure compared to those with an atrial switch procedure. Other patients may develop leaky heart valves, or their great arteries may become narrowed over time, requiring additional intervention.
#1
https://www.pted.org/?id=transpositiond4
The untreated patient with this anomaly is not expected to survive to adulthood, with 90% of all affected individuals dying in infancy. […] There is no heart murmur with TGAD unless caused by an associated defect. […] The long-term prognosis for patients who received the Mustard or Senning atrial switch operations is not as good as for those who received an arterial switch. Arrhythmias often develop over time, and are probably responsible for most cases of sudden death that occur (7-15% of all patients, with resuscitation possible in some cases). […] Rastelli patients are also subject to the development of arrhythmias and sudden death. About one quarter will develop left ventricular dysfunction. […] The prognosis for arterial switch (Jatene) patients is very good. A study of 1000 patients showed that 88% survived for 10 years, with no further deaths between 10 and 15 years after surgery.
#1 Transposition of the Great Arteries after Mustard/Senning Repair – ACHA
https://www.achaheart.org/your-heart/educational-qas/types-of-heart-defects/transposition-of-the-great-arteries-after-mustardsenning-repair/
Research reports that about 10% of TGA post-Mustard/Senning patients are now experiencing heart failure. […] If other treatment options are not working, surgical options may be considered. […] In a small number of cases, women with TGA experience new problems with heart failure and/or heart rhythm during pregnancy. […] The American College of Cardiology and the American Heart Association classify transposition of the great arteries as a highly complex heart defect. […] This way you can catch any problems before they start, since new heart problems can develop without symptoms.
#1 Transposition of the Great Arteries after Mustard/Senning Repair – ACHA
https://www.achaheart.org/your-heart/educational-qas/types-of-heart-defects/transposition-of-the-great-arteries-after-mustardsenning-repair/
In transposition of the great arteries (TGA), the hearts two major arteries are reversed. […] Babies born with TGA will die unless there is a hole between the two sides of the heart to allow some red blood to get out to the body. […] Babies born with TGA almost always need surgery to help the heart work more normally. […] There are three kinds of problems that people with a Mustard or Senning repair face: rhythm problems, baffle problems, and pump problems: […] Many TGA patients have inborn problems with the hearts electrical system. […] Up to 25% of Mustard/Senning patients have a pacemaker by adulthood. […] Over time the baffles in the Mustard/Senning repair can develop problems. […] Sometimes these leaks do not need treatment. […] Heart failure symptoms can include feeling extra-tired on exertion, shortness of breath on exertion, and/or retaining fluid.
#1 d-Transposition of the Great Arteries | American Heart Association
https://www.heart.org/en/health-topics/congenital-heart-defects/about-congenital-heart-defects/d-transposition-of-the-great-arteries
Patients with transposition of the great arteries require surgery early in life to survive. […] Without surgery, the only way to survive this condition temporarily is to have leakages that allow some oxygen-rich blood to cross into the oxygen-poor blood for delivery to the body. […] Babies born with transposition are blue. If there are no connections between the right and left side of the heart to allow the oxygen poor and oxygen rich blood to mix, the baby can only survive for a short time (days). Some type of surgical treatment is needed to allow for survival throughout childhood and into adulthood. […] Patients who have undergone the Senning or Mustard procedures may have problems as they enter their twenties and thirties. The surgery in the atrium leaves areas of scar that can result in abnormal heart rhythms. Since the right ventricle remains the heart’s main pumping chamber, it can become weakened, resulting in signs and symptoms of congestive heart failure. About half of patients will develop blockage in the baffles that were created to reroute the blood flow. […] Patients who had the arterial switch operation infrequently may need more surgery to relieve narrowing in the aorta or pulmonary artery where the original surgery was done, or to fix leaky valves.
#1 Transposition of Great Arteries (TGA) – Congenital Heart Disease in Adults – Cardiovascular Diseases – Diseases – McMaster Textbook of Internal Medicine
https://empendium.com/mcmtextbook/chapter/B31.II.2.12.12.
Symptoms: The majority of patients have had previous operations. Rarely, a patient with previously unrecognized isolated congenitally corrected TGA is seen with presyncope or syncope. Patients may develop progressive dyspnea, fatigue, and decreased exercise tolerance as the function of the systemic ventricle deteriorates. Most patients with a previous atrial switch operation develop atrial arrhythmias and may present with palpitations and presyncope. Patients who have had the arterial switch operation may present with signs or symptoms of ischemia related to the coronary artery stenosis.
#1 Transposition of the great arteries (TGA)
https://teens.aboutkidshealth.ca/transposition-of-the-great-arteries-tga
This switch of the great arteries means that blood that is low in oxygen gets pumped around the body instead of blood that is high in oxygen. An open-heart surgery called the arterial switch operation is needed to fix this problem with the heart’s arteries. […] Some patients with TGA may also have other heart problems, such as a ventricular septal defect (VSD), abnormal coronary arteries (blood vessels that carry blood and oxygen into the heart muscle) or pulmonary stenosis. […] Although the arterial switch is major heart surgery, it is a very successful operation. However, some complications can occur. TGA is a lifelong condition so its important to keep seeing your cardiologist. Many complications can be diagnosed before you develop symptoms. […] In a small number of children, the main pulmonary artery or the branch pulmonary arteries may become narrow.
#1 Transposition of the Great Arteries (TGA)
https://my.clevelandclinic.org/health/diseases/23387-transposition-of-the-great-arteries
But people with this congenital condition need lifelong care. […] The survival rate after an arterial switch surgery is more than 95%. […] Your child can live a full life after treatment for transposition of the great arteries. […] After transposition surgery, people have a higher risk for other heart problems. […] Yes, your child may need more surgery later. […] Endocarditis is an infection that happens when bacteria enter your heart. Its more common in people with heart conditions, like TGA. […] See a healthcare provider if your baby has: […] Trouble breathing. […] Weak pulse. […] Bluish or whitish skin around their mouth. […] If you had transposition surgery as a child, you need routine care and evaluation throughout your life.
#1 l-Transposition of the Great Arteries | American Heart Association
https://www.heart.org/en/health-topics/congenital-heart-defects/about-congenital-heart-defects/l-transposition-of-the-great-arteries
Babies born with l-transposition usually aren’t blue. The congenital heart defect may go undetected for a long time. It might not be diagnosed until well into adulthood when congestive heart failure, heart murmurs and abnormal heart rhythms can develop. […] Patients may have problems with heart muscle, tricuspid valve leakage or heart rhythm as they enter adulthood, whether or not they had prior surgery. Like patients with atrial repair of d-TGA, the right ventricle may weaken, leading to congestive heart failure. Abnormally slow heart rhythms may cause fainting or fatigue. The most worrisome slow heart rhythm, complete heart block, is common in l-transposition. Rapid heart rhythms are less common than in d-TGA patients. […] The right ventricular function may decline over time.
#1 About dextro-Transposition of the Great Arteries (d-TGA) | Congenital Heart Defects (CHDs) | CDC
https://www.cdc.gov/heart-defects/about/d-tga.html
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. With proper treatment, most babies with d-TGA grow up to lead healthy, productive lives.
#1 Transposition of the great arteries (TGA)
https://teens.aboutkidshealth.ca/transposition-of-the-great-arteries-tga
Sometimes the aorta can dilate (become larger) at the site where it was reattached to the left ventricle. This can be diagnosed by regular follow up with your cardiologist. Similarly, a leaky aortic valve can be diagnosed before symptoms develop. […] Children who had surgery for TGA are expected to grow and be able to exercise normally. […] For most individuals who had a repair of their TGA with an arterial switch operation pregnancy is safe. […] The risk for any person having a baby with congenital heart disease is about 1%. Babies born to a parent who has congenital heart disease, such as an TGA, the chance that the baby will have any type of congenital heart disease is about 3%.
#1 Transposition of the Great Arteries | Diagnosis & Treatment
https://www.cincinnatichildrens.org/health/t/transposition
Transposition of the great arteries can be diagnosed by a fetal ultrasound. This can be missed on a routine fetal ultrasound. Sometimes, a fetal echocardiogram done by specialists is needed to make a diagnosis of transposition in a fetus. […] After birth, transposition is diagnosed in the first hours or days of life due to cyanosis or low oxygen levels. All babies have a patent ductus arteriosus (PDA) at birth that may allow enough mixing to prevent severe cyanosis. As the ductus arteriosus closes, as it will in the first hours or days of life, cyanosis gets more severe. […] Rapid breathing is seen. This is due to the low oxygen levels in the blood. The infants do look uncomfortable breathing this fast. […] A heart murmur heart murmur is often absent in the first days or weeks of life. If there is a site where blood mixing allows for safe oxygen levels, children will often develop signs and symptoms of congestive heart failure over the course of the first weeks or months of life. […] Untreated, over 50 percent of infants with transposition will die in the first month of life. Ninety percent will die in the first year.
#1 Transposition of the Great Arteries (TGA) – Stanford Medicine Children’s Health
https://www.stanfordchildrens.org/en/topic/default?id=transposition-of-the-great-arteries-tga-90-P01823
Most babies with TGA have blue skin color (cyanosis) in the first hours or days of their lives. […] Other symptoms that can occur include: Fast breathing, Trouble breathing, Fast heart rate, Poor feeding. […] The symptoms of TGA may be similar to symptoms of other conditions. Make sure that your child sees his or her healthcare provider for a diagnosis.
#1 Transposition of the Great Arteries – Children’s Hospital of Orange County
https://choc.org/heart/congenital-heart-defects/transposition-of-the-great-arteries/
Transposition of the great arteries is the second most common congenital heart defect that causes problems in early infancy. TGA occurs in about 3% of all congenital heart defects. […] The most common indication of TGA in a newborn is cyanosis (blueish skin color) in the first day of life. Cyanosis is noted in the first hours of life in about half of the infants with TGA, and within the first days of life in most of them. The degree of cyanosis is related to the presence of other defects that allow blood to mix, including an atrial septal defect (a hole between the top chambers of the heart) and a patent ductus arteriosus (a fetal connection between the aorta and the pulmonary artery present in the newborn, which usually closes in the first few days after birth). […] The following are the other most common symptoms of TGA. However, each child may experience symptoms differently. Symptoms may include: Rapid breathing, Labored breathing, Rapid heart rate, Cool, clammy skin. […] The symptoms of TGA may resemble other medical conditions or heart problems. Always consult your childâs doctor for a diagnosis.
#1 Transposition of the great arteries – Diagnosis and treatment – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/transposition-of-the-great-arteries/diagnosis-treatment/drc-20350595
Transposition of the great arteries is most often diagnosed after a baby is born. […] After birth, a health care provider may think about a diagnosis of a TGA if the baby has blue or gray skin, a weak pulse, or trouble breathing. […] Babies born with TGA often have other heart problems. […] After surgery to fix TGA, lifelong care is needed with a provider trained in heart problems present at birth. This type of health care provider is called a congenital cardiologist. […] Every situation is different. But due to advances in surgical treatment, most babies with transposition of the great arteries grow up to lead active lives.
#2 Transposition of the great vessels Information | Mount Sinai – New York
https://www.mountsinai.org/health-library/diseases-conditions/transposition-of-the-great-vessels
Transposition of the great arteries (TGA) is a heart defect that occurs from birth (congenital). The two major arteries that carry blood away from the heart — the aorta and the pulmonary artery — are switched (transposed). […] Symptoms appear at birth or very soon afterward. How bad the symptoms are depends on the type and size of additional heart defects (such as atrial septal defect, ventricular septal defect, or patent ductus arteriosus) and how much the blood can mix between the two abnormal circulations. […] Symptoms may include: Bluish color to the skin (cyanosis) due to low oxygen level in the blood, Clubbing of the fingers or toes, Poor feeding, Shortness of breath.
#2 Transposition of the Great Arteries – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK538434/
Transposition of the great arteries (TGA) is a congenital cardiac defect characterized by an embryological discordance of the aorta and pulmonary trunk. In dextro-transposition of the great arteries (d-TGA), the aorta arises from the right ventricle and the pulmonary trunk from the left ventricle, resulting in 2 parallel circuits that are incompatible with life unless there is a shunt, such as a patent ductus arteriosus (PDA) or septal defect, to allow for blood mixing. Early diagnosis and surgical intervention, such as the arterial switch operation, are essential for optimal outcomes. […] Patients with d-TGA typically present with cyanosis within the first 30 days of life. Completely parallel circulatory circuits are incompatible with life and require a patent ductus arteriosus (PDA) or an atrial (ASD) or ventricular septal defect (VSD) to allow the mixing of oxygenated and deoxygenated blood.
#2 Transposition of the Great Arteries (TGA)
https://my.clevelandclinic.org/health/diseases/23387-transposition-of-the-great-arteries
Transposition of the great arteries symptoms include: […] Newborns with d-TGA have symptoms of cyanosis (low oxygen) and may also have heart failure. […] Trouble breathing. […] Weak pulse. […] Difficulty feeding. […] Pounding heart. […] Skin or whites of their eyes appear bluish (in light-skinned babies) or grayish (in dark-skinned babies). […] In a baby with the d type of TGA heart problem, signs appear soon after birth. Their severity depends on whether oxygen-rich blood can reach the rest of your babys body by mixing in the upper chambers of their heart. […] Some newborns dont show symptoms right away because small openings around their hearts havent closed yet. Once the holes close, symptoms appear, and your baby requires immediate medical attention. […] While surgery doesnt cure d-transposition of the great arteries, most people with the condition can lead full, healthy lives.
#2 Transposition of the Great Arteries | Clinical Heart and Vascular Center | Condition | UT Southwestern Medical Center
https://utswmed.org/conditions-treatments/transposition-of-the-great-arteries/
Transposition of the great arteries is the most common cyanotic (low blood oxygen) congenital heart condition. […] Cyanosis a bluish color to the skin caused by insufficient oxygenation is the primary symptom of TGA, and it is usually noticeable at birth or shortly thereafter. Its severity depends on the size and type of any heart abnormalities and how much oxygen is moving through the patients body: […] TGA with no additional heart defect: Severe cyanosis in the first 24 hours of life […] TGA with a large ventricular septal defect (VSD): Mild cyanosis when crying, increased breathing and heart rates, sweating, and failure to gain weight in the first six weeks of life […] TGA with VSD and obstruction in the left ventricle: Extreme cyanosis at birth […] TGA with VSD and pulmonary artery obstruction: Progressive cyanosis from birth. […] As with most congenital heart defects, the cause of TGA is unknown. Occurring primarily in males, the condition has a 90 percent mortality rate within the first year of life, if left untreated.
#2 Transposition of the Great Arteries (TGA) | Doctor
https://patient.info/doctor/transposition-of-the-great-arteries
Transposition of the great arteries symptoms (presentation) […] Asymptomatic cases surviving to adulthood are reported. […] Infants with TGA with intact septum usually present with cyanosis within the first week of life which may be obvious in some soon after birth. They may otherwise be well and asymptomatic initially although with time they become tachypnoeic and develop respiratory distress. If they are not treated they develop metabolic acidosis and become severely ill. […] […] Those with a large VSD may not be diagnosed until several weeks of age. They usually present with symptoms of congestive heart failure (tachypnoea, tachycardia, sweating and poor feeding) between 4-8 weeks of life. Cyanosis is often minimal. […] […] If there is a VSD and pulmonary stenosis, the presentation can vary depending on the severity of PS. If the PS is severe, the presentation is similar to that of an infant with Fallot’s tetralogy. However, those with poor mixing of blood can present early with cyanosis similar to TGA with intact septum and some with mild PS may present late with heart failure.
#2 Transposition of the Great Arteries (TGA): Diagnosis & Treatment | NewYork-Presbyterian
https://www.nyp.org/pediatrics/heart/transposition-of-the-great-arteries-tga/treatment
Transposition of the great arteries (TGA) is a heart defect generally diagnosed at birth. A newborn will typically have blue skin, difficulty breathing, or a weak pulse. […] An abnormal whooshing sound in the baby’s heart can indicate the presence of TGA. […] Most babies born with TGA require surgery soon after birth. […] Without surgery to correct transposition of the great arteries, the life expectancy rates are low. Over 50% of patients will die in the first month and 90% within their first year. […] More than 90% of babies who undergo TGA surgery grow into adulthood and lead healthy lives but will need lifelong check-ins with a cardiologist.
#2 Transposition of the Great Arteries | Conditions | UCSF Health
https://www.ucsfhealth.org/conditions/transposition-of-the-great-arteries
A baby with transposed arteries is blue, or cyanotic, shortly after birth. The blueness doesn’t go away even if the baby is given extra oxygen. For a baby with transposed arteries to survive, blood flow between the right and left sides of the heart must be increased. These infants rarely survive without surgical intervention so it is extremely rare for a person to grow to adulthood without treatment. […] A complete transposition usually is diagnosed shortly after birth due to the baby’s increasing blue coloration or cyanosis. There may or may not be a heart murmur or extra sound in the heartbeat. […] Currently, permanent treatment requires a surgical procedure to switch the arteries to their proper places. This operation, called an arterial switch operation, is done within the first few weeks of life. It is an open-heart procedure that requires a temporary stopping of the baby’s heart while a heart-lung machine handles respiration and blood circulation. Any abnormal holes between the ventricles or atria also are closed.
#2 Transposition of the Great Arteries Signs & Symptoms | Rush | Rush System
https://www.rush.edu/kids/conditions/transposition-great-arteries
Transposition of the great arteries (TGA), also referred to as dextro-transposition of the great arteries or D-TGA, is a congenital heart defect in which the two major vessels that carry blood away from the heart the aorta and the pulmonary artery are switched. It is a relatively rare condition, affecting 20 to 30 infants per 100,000 live births. […] TGA symptoms occur at birth or soon after. Babies with TGA may have a condition called cyanosis, which is bluish looking skin caused by their blood not carrying enough oxygen. Other symptoms may include the following: Problems breathing, Pounding heart, Weak pulse, Trouble eating. […] All babies born with TGA must have surgery to switch and reattach the aorta and pulmonary arteries and reconnect the coronary arteries. Other procedures may be done before surgery to maintain, enlarge or create openings that will allow oxygen-rich blood to get out to the body. […] People born with TGA have lifelong complications. Your child will need regular follow-up visits with a cardiologist to monitor their progress and avoid other health problems or complications.
#2 About dextro-Transposition of the Great Arteries (d-TGA) | Congenital Heart Defects (CHDs) | CDC
https://www.cdc.gov/heart-defects/about/d-tga.html
Symptoms for d-TGA occur at birth or very soon afterwards. How severe the symptoms are will depend on whether oxygen-rich blood can get out to the rest of the body. For example, if an infant with d-TGA has an atrial septal defect (ASD), the ASD forms a passageway for some oxygen-rich blood to reach the rest of the body. Therefore, this infant with both defects may not have as severe symptoms as infants whose hearts don’t have any mixing of blood. […] Infants with d-TGA can have a bluish looking skin color because their blood doesn’t carry enough oxygen. This condition is called cyanosis. Infants with d-TGA or other conditions causing cyanosis can have symptoms such as Problems breathing, Pounding heart, Weak pulse, Ashen or bluish skin color, Poor feeding. […] After surgery, medications may be needed to Help the heart pump better, Control blood pressure, Help get rid of extra fluid in the body, Slow down the heart if it is beating too fast.
#2 l-Transposition of the Great Arteries | American Heart Association
https://www.heart.org/en/health-topics/congenital-heart-defects/about-congenital-heart-defects/l-transposition-of-the-great-arteries
Babies born with l-transposition usually aren’t blue. The congenital heart defect may go undetected for a long time. It might not be diagnosed until well into adulthood when congestive heart failure, heart murmurs and abnormal heart rhythms can develop. […] Patients may have problems with heart muscle, tricuspid valve leakage or heart rhythm as they enter adulthood, whether or not they had prior surgery. Like patients with atrial repair of d-TGA, the right ventricle may weaken, leading to congestive heart failure. Abnormally slow heart rhythms may cause fainting or fatigue. The most worrisome slow heart rhythm, complete heart block, is common in l-transposition. Rapid heart rhythms are less common than in d-TGA patients. […] The right ventricular function may decline over time.
#2 L-Transposition of the Great Arteries
https://www.rwjbh.org/treatment-care/heart-and-vascular-care/diseases-conditions/congenital-heart-disease/l-transposition-of-the-great-arteries/
Transposition of the Great Arteries, or TGA, is a congenital birth defect (present at birth) in which the two main arteries carrying blood out of the heart, the main pulmonary artery and the aorta, are switched in position, or transposed. […] Many times this condition is not recognized until the child is older or even as an adult. Your doctor will continue to monitor your condition throughout your life. […] Some people with this condition may not experience symptoms for many years. Some of the most common symptoms include: Fatigue, Heart palpitations, Heart murmur, Shortness of breath, Syncope.
#2
https://www.wkhs.com/health-resources/wk-health-library/disease-condition-information/a-z/transposition-of-the-great-arteries-(tga)
Symptoms of TGA may be noticed just after the birth, or within the first week of life. The baby’s skin may turn a blue color, and the skin may feel cool and clammy. The baby may be short of breath, and may have a faster-than-normal heart rate. And, the baby may have trouble feeding and gaining weight. Without proper treatment, TGA can result in complications such as heart failure, lung damage and death. […] In a heart with TGA, this process does not work correctly. Oxygen-depleted blood from the body is carried to the aorta and sent back to the body without flowing through the lungs. Oxygen-rich blood from the lungs is carried through the pulmonary arteries and sent back to the lungs instead of flowing out to the rest of the body. The body’s cells quickly become starved of oxygen.
#2 d-Transposition of the Great Arteries | American Heart Association
https://www.heart.org/en/health-topics/congenital-heart-defects/about-congenital-heart-defects/d-transposition-of-the-great-arteries
Patients with transposition of the great arteries require surgery early in life to survive. […] Without surgery, the only way to survive this condition temporarily is to have leakages that allow some oxygen-rich blood to cross into the oxygen-poor blood for delivery to the body. […] Babies born with transposition are blue. If there are no connections between the right and left side of the heart to allow the oxygen poor and oxygen rich blood to mix, the baby can only survive for a short time (days). Some type of surgical treatment is needed to allow for survival throughout childhood and into adulthood. […] Patients who have undergone the Senning or Mustard procedures may have problems as they enter their twenties and thirties. The surgery in the atrium leaves areas of scar that can result in abnormal heart rhythms. Since the right ventricle remains the heart’s main pumping chamber, it can become weakened, resulting in signs and symptoms of congestive heart failure. About half of patients will develop blockage in the baffles that were created to reroute the blood flow. […] Patients who had the arterial switch operation infrequently may need more surgery to relieve narrowing in the aorta or pulmonary artery where the original surgery was done, or to fix leaky valves.
#2 D-Transposition of the Great Arteries > Fact Sheets > Yale Medicine
https://www.yalemedicine.org/conditions/d-transposition-of-the-great-arteries
Symptoms include bluish skin, difficulty breathing, poor feeding, pounding heart, weak pulse. […] Newborns experience the following signs and symptoms within the first days of life, including: Bluish skin, Difficulty breathing, Poor feeding habits, A pounding heart, A weak pulse. […] Without surgical intervention, 90% of babies born with d-transposition of the great arteries will die during their first year of life; half will die within the first month. With surgery, over 95% of patients will live into adulthood. […] Patients who have an atrial switch procedure are at increased risk of congestive heart failure during adulthood because the procedure makes the right ventricle work harder than it should. […] Some patients with d-transposition of the great arteries develop arrhythmias (heart rhythm problems) over time. This is less of a risk in patients with the arterial switch procedure compared to those with an atrial switch procedure. Other patients may develop leaky heart valves, or their great arteries may become narrowed over time, requiring additional intervention.
#2 Transposition of the great arteries (TGA) – BHF
https://www.bhf.org.uk/informationsupport/conditions/transposition-of-the-great-arteries
When youre born with TGA, the symptoms you have as a baby can include: blue or grey skin (cyanosis), having little energy, shortness of breath, a weak pulse, long periods of crying, difficulty feeding or gaining weight. […] The symptoms that you have as an adult depend on you and your condition. Factors that can affect the symptoms you have include: whether you were born with other conditions like ASD or VSD, the type of surgery you had to fix TGA. […] Cyanosis is when your skin and lips turn blue or grey due to low levels of oxygen. It can be a side effect or symptom of some congenital heart diseases. It happens with TGA because a small amount of blood high in oxygen is moving around your body. […] As you get older, its common to have problems with your heart. Youre more likely to have issues if your TGA was repaired by an atrial switch or Rastelli procedure. The complications can include: narrowing (stenosis) of the pulmonary artery, leaky heart valves (regurgitation), baffle narrowing or leaking, conduit narrowing or leaking, heart rhythm problems (arrhythmias), weakening of your heart muscle and heart failure. You may need more surgeries in the future to fix these problems, like a leaking valve or narrowed pulmonary artery.
#2 Transposition of the great arteries (TGA)
https://teens.aboutkidshealth.ca/transposition-of-the-great-arteries-tga
This switch of the great arteries means that blood that is low in oxygen gets pumped around the body instead of blood that is high in oxygen. An open-heart surgery called the arterial switch operation is needed to fix this problem with the heart’s arteries. […] Some patients with TGA may also have other heart problems, such as a ventricular septal defect (VSD), abnormal coronary arteries (blood vessels that carry blood and oxygen into the heart muscle) or pulmonary stenosis. […] Although the arterial switch is major heart surgery, it is a very successful operation. However, some complications can occur. TGA is a lifelong condition so its important to keep seeing your cardiologist. Many complications can be diagnosed before you develop symptoms. […] In a small number of children, the main pulmonary artery or the branch pulmonary arteries may become narrow.
#2 Transposition of the great arteries – Hancock Health
https://www.hancockhealth.org/mayo-health-library/transposition-of-the-great-arteries/
Always seek emergency medical help if you notice that anyone develops a blue or gray skin color. […] Possible complications of complete transposition of the great arteries (D-TGA) may include: Not enough oxygen to body tissues. Unless theres some mixing of oxygen-rich blood and oxygen-poor blood within the body, this complication causes death. Heart failure. Heart failure is a condition in which the heart cant pump enough blood to meet the bodys needs. It may develop over time because the right lower heart chamber is pumping under higher pressure than usual. The strain may make the muscle of the right lower chamber stiff or weak. […] Possible complications of congenitally corrected transposition (L-TGA) may include: Reduced heart pumping. In L-TGA, the right lower heart chamber pumps blood to the body. This work is different from what that chamber was designed to do. This can cause changes in how well the heart pumps blood. Complete heart block. The changes in the structure of the heart due to L-TGA can change the electrical signals that tell the heart to beat. A complete heart block occurs if all signals are blocked. Heart valve disease. In L-TGA, the valve between the upper and lower heart chambers the tricuspid valve may not close completely. Blood might move backward through the valve. This condition is called tricuspid valve regurgitation. It can eventually reduce the hearts ability to pump blood. […] After birth, a health care provider may think about a diagnosis of a TGA if the baby has blue or gray skin, a weak pulse, or trouble breathing. The care provider may hear a heart sound, called a murmur, while listening to the babys heart.
#2 Transposition of the Great Arteries (TGA)
https://my.clevelandclinic.org/health/diseases/23387-transposition-of-the-great-arteries
But people with this congenital condition need lifelong care. […] The survival rate after an arterial switch surgery is more than 95%. […] Your child can live a full life after treatment for transposition of the great arteries. […] After transposition surgery, people have a higher risk for other heart problems. […] Yes, your child may need more surgery later. […] Endocarditis is an infection that happens when bacteria enter your heart. Its more common in people with heart conditions, like TGA. […] See a healthcare provider if your baby has: […] Trouble breathing. […] Weak pulse. […] Bluish or whitish skin around their mouth. […] If you had transposition surgery as a child, you need routine care and evaluation throughout your life.
#2 About dextro-Transposition of the Great Arteries (d-TGA) | Congenital Heart Defects (CHDs) | CDC
https://www.cdc.gov/heart-defects/about/d-tga.html
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. With proper treatment, most babies with d-TGA grow up to lead healthy, productive lives.
#2 Transposition of the Great Arteries | Diagnosis & Treatment
https://www.cincinnatichildrens.org/health/t/transposition
Transposition of the great arteries can be diagnosed by a fetal ultrasound. This can be missed on a routine fetal ultrasound. Sometimes, a fetal echocardiogram done by specialists is needed to make a diagnosis of transposition in a fetus. […] After birth, transposition is diagnosed in the first hours or days of life due to cyanosis or low oxygen levels. All babies have a patent ductus arteriosus (PDA) at birth that may allow enough mixing to prevent severe cyanosis. As the ductus arteriosus closes, as it will in the first hours or days of life, cyanosis gets more severe. […] Rapid breathing is seen. This is due to the low oxygen levels in the blood. The infants do look uncomfortable breathing this fast. […] A heart murmur heart murmur is often absent in the first days or weeks of life. If there is a site where blood mixing allows for safe oxygen levels, children will often develop signs and symptoms of congestive heart failure over the course of the first weeks or months of life. […] Untreated, over 50 percent of infants with transposition will die in the first month of life. Ninety percent will die in the first year.
#2 Levo-Transposition of the Great Arteries (for Parents) | Nemours KidsHealth
https://kidshealth.org/en/parents/l-tga.html
Levo-transposition of the great arteries (L-TGA) is when the positions of the hearts ventricles are reversed. Some people with the condition never need treatment. Others develop symptoms and need treatment with medicines and surgery. […] Symptoms usually show up later in childhood or in adulthood. These may include trouble breathing, fainting, or lightheadedness. Sometimes a person may have an unsteady or slow heartbeat. When there are other issues, such as a hole in the heart or an abnormal valve or blood vessel, the baby may have a heart murmur or other issues that bring them to medical attention early in life. […] Over time, this can lead to heart failure when the pumping chamber gets larger, and the heart muscle becomes weak.
#3 Transposition of the Great Arteries (TGA): Symptoms & Causes | NewYork-Presbyterian
https://www.nyp.org/pediatrics/heart/transposition-of-the-great-arteries-tga
Signs of TGA generally appear at birth or very soon after, depending on the severity of the case. An infant with bluish color or breathing issues is usually diagnosed with TGA within the first week. […] Common transposition of the great arteries symptoms can include: Breathing problems, Weak pulse, Bluish or ashen skin color (cyanosis), Trouble feeding, Poor weight gain. […] If your baby is exhibiting any TGA symptoms, a pediatric cardiologist or heart specialist can provide a diagnosis and treatment.
#3 Transposition of the Great Arteries | UK Healthcare
https://ukhealthcare.uky.edu/gill-heart-vascular-institute/conditions/adult-congenital-heart-disease/transposition-great-arteries
Symptoms of transposition of the great arteries depend on the type. […] Infants with d-TGA usually develop symptoms within their first month, including a heart murmur, rapid breathing or cyanosis, a blue coloring caused by low oxygen levels in the blood. In adulthood, patients often develop additional symptoms due to late surgery complications. Symptoms of complications may include abnormal heart rhythms or signs of congestive heart failure. […] Patients with l-TGA often don’t have symptoms until adulthood. The first symptoms of l-TGA may be signs of early congestive heart failure. Adults with l-TGA can also develop arrhythmias or a heart murmur. […] With surgical treatment, more than 9 in 10 patients with d-TGA live at least 15 years after surgery. […] People with l-TGA are often in their 30s or 40s before diagnosis. Identifying and treating l-TGA as soon as possible can help patients prevent or slow worsening heart failure.
#3 Transposition of the great arteries (TGA) – BHF
https://www.bhf.org.uk/informationsupport/conditions/transposition-of-the-great-arteries
When youre born with TGA, the symptoms you have as a baby can include: blue or grey skin (cyanosis), having little energy, shortness of breath, a weak pulse, long periods of crying, difficulty feeding or gaining weight. […] The symptoms that you have as an adult depend on you and your condition. Factors that can affect the symptoms you have include: whether you were born with other conditions like ASD or VSD, the type of surgery you had to fix TGA. […] Cyanosis is when your skin and lips turn blue or grey due to low levels of oxygen. It can be a side effect or symptom of some congenital heart diseases. It happens with TGA because a small amount of blood high in oxygen is moving around your body. […] As you get older, its common to have problems with your heart. Youre more likely to have issues if your TGA was repaired by an atrial switch or Rastelli procedure. The complications can include: narrowing (stenosis) of the pulmonary artery, leaky heart valves (regurgitation), baffle narrowing or leaking, conduit narrowing or leaking, heart rhythm problems (arrhythmias), weakening of your heart muscle and heart failure. You may need more surgeries in the future to fix these problems, like a leaking valve or narrowed pulmonary artery.