Tetralogia fallota
Objawy

Tetralogia Fallota, stanowiąca 5-7% wrodzonych wad serca, to złożona wada anatomiczna obejmująca cztery defekty serca, prowadzące do zaburzeń przepływu krwi i hipoksemii. Kluczowym objawem jest sinica, której nasilenie zależy od stopnia zwężenia drogi odpływu z prawej komory do tętnicy płucnej. Charakterystyczne są napady anoksemiczne, pojawiające się najczęściej u niemowląt w wieku 2-4 miesięcy, manifestujące się nagłym nasileniem sinicy, dusznością i niepokojem. W badaniu fizykalnym dominują szmer wyrzutowy o zmiennej głośności, pojedynczy drugi ton serca (S2) oraz palce pałeczkowate. Naturalny przebieg bez leczenia chirurgicznego wiąże się z wysoką śmiertelnością: 33-35% w pierwszym roku życia, 50-60% do 3 roku życia, a do 40 roku życia aż 97% pacjentów nie przeżywa.

Tetralogia Fallota – objawy

Tetralogia Fallota to wrodzona wada serca, która stanowi około 5-7% wszystkich wrodzonych wad serca i jest najczęstszą siniczą wadą serca 1. Jest to złożona wada składająca się z czterech defektów anatomicznych serca występujących jednocześnie, które powodują zaburzenia przepływu krwi przez serce i zmniejszone natlenienie organizmu 23. Nasilenie objawów tetralogii Fallota zależy przede wszystkim od stopnia zwężenia drogi odpływu z prawej komory do tętnicy płucnej, co określa ilość krwi docierającej do płuc w celu natlenienia 45.

Sinica – podstawowy objaw tetralogii Fallota

Najbardziej charakterystycznym objawem tetralogii Fallota jest sinica (cyanoza), czyli niebieskie lub szarawe zabarwienie skóry, warg, błon śluzowych jamy ustnej i nosa oraz paznokci 67. Sinica pojawia się z powodu niedoboru tlenu we krwi krążącej w organizmie – krew uboga w tlen przepływa przez ubytek przegrody międzykomorowej (VSD) z prawej komory serca do lewej, a następnie do aorty i całego ciała 89.

Stopień sinicy jest różny i zależy od nasilenia zwężenia drogi odpływu z prawej komory i tętnicy płucnej 10. U niektórych dzieci sinica jest widoczna bezpośrednio po urodzeniu, szczególnie przy ciężkim zwężeniu tętnicy płucnej. U innych może się rozwinąć stopniowo w ciągu pierwszych tygodni lub miesięcy życia, gdy zwężenie drogi odpływu prawej komory postępuje 11. W łagodniejszych przypadkach, przy minimalnym zwężeniu tętnicy płucnej, dzieci mogą nie wykazywać sinicy (tzw. „różowa tetralogia Fallota”) 109.

Napady anoksemiczne (napady sinicy)

Charakterystycznym objawem tetralogii Fallota są napady anoksemiczne (tzw. „napady sinicze” lub „napady tetralogiczne”), które występują, gdy poziom tlenu we krwi gwałtownie spada 1213. Napady te objawiają się nagłym nasileniem sinicy, trudnościami w oddychaniu i znacznym niepokojem dziecka 6. Najczęściej występują u niemowląt w wieku 2-4 miesięcy 1415 i mogą być wywołane przez:

  • Płacz lub krzyk 16
  • Karmienie 6
  • Defekację 12
  • Wysiłek 14
  • Pobudzenie lub stres 16
  • Odwodnienie lub gorączkę 17

Podczas napadu anoksemicznego dziecko może wykazywać następujące objawy 1819:

  • Nagłe nasilenie sinicy (nasycenie skóry kolorem niebieskim lub szarym)
  • Znaczne trudności w oddychaniu, przyspieszony oddech (hiperwentylacja)
  • Silny niepokój, drażliwość lub płacz
  • Osłabienie, senność
  • Utrata świadomości (omdlenie)
  • W ciężkich przypadkach – drgawki

Napady anoksemiczne są stanem nagłym, wymagającym natychmiastowej interwencji medycznej. Nieleczone mogą prowadzić do niedotlenienia mózgu, udarów mózgu a nawet śmierci 2021. W trakcie napadu szmer sercowy może ulec osłabieniu lub zaniknąć 22.

Inne objawy oddechowe i wysiłkowe

Dzieci z tetralogią Fallota często prezentują różnorodne objawy związane z układem oddechowym i nietolerancją wysiłku 23:

  • Przyspieszony oddech (tachypnoe), zwłaszcza podczas karmienia lub wysiłku 13
  • Duszność 3
  • Łatwe męczenie się podczas aktywności, zabawy lub karmienia 24
  • Przyjmowanie pozycji kucznej podczas odpoczynku po wysiłku w celu zwiększenia przepływu krwi do płuc (u starszych dzieci) 257

Dzieci starsze z nieleczoną tetralogią Fallota mogą przyjmować charakterystyczną pozycję kuczną po wysiłku, co pomaga zwiększyć opór naczyń systemowych, zmniejsza przeciek prawo-lewy i zwiększa przepływ krwi przez płuca 726.

Problemy z karmieniem i rozwój fizyczny

Niemowlęta z tetralogią Fallota często doświadczają problemów z karmieniem i rozwojem fizycznym 27:

  • Trudności podczas karmienia – szybkie męczenie się 7
  • Słaby apetyt 28
  • Niewystarczający przyrost masy ciała (failure to thrive) 311
  • Opóźnienie wzrostu i rozwoju fizycznego 3
  • Opóźnione dojrzewanie (w przypadku nieleczonej wady) 726

Problemy z karmieniem wynikają z szybkiego męczenia się dziecka w związku z zwiększonym wysiłkiem oddechowym i niewydolnością serca. Chroniczne niedotlenienie hamuje prawidłowy wzrost i rozwój dziecka 29.

Inne objawy fizykalne

W badaniu fizykalnym dzieci z tetralogią Fallota można stwierdzić 3026:

  • Szmer sercowy – najczęściej szmer wyrzutowy, słyszalny wzdłuż drogi odpływu prawej komory, którego głośność jest odwrotnie proporcjonalna do stopnia zwężenia (im większe zwężenie, tym cichszy szmer) 22
  • Pojedynczy drugi ton serca (S2) – brak słyszalnego zamknięcia zastawki płucnej 26
  • Palce pałeczkowate (clubbing) – poszerzenie i zaokrąglenie końcówek palców oraz paznokci, pojawiające się z czasem u dzieci z przewlekłą sinicą 3119
  • Chłodna, wilgotna skóra 32

Tetralogia Fallota – progresja choroby

Naturalny przebieg tetralogii Fallota bez leczenia chirurgicznego wiąże się z postępującym pogarszaniem stanu pacjenta i niekorzystnym rokowaniem 3321.

Naturalny przebieg nieleczonej wady

Bez interwencji chirurgicznej tetralogia Fallota prowadzi do postępującego pogorszenia stanu zdrowia i skrócenia długości życia 34. Śmiertelność u nieleczonych pacjentów wynosi 2133:

  • Około 33-35% w pierwszym roku życia
  • Około 50-60% do 3 roku życia
  • Około 89% do 20 roku życia
  • Około 94% do 30 roku życia
  • Około 97% do 40 roku życia

Głównymi przyczynami zgonów u pacjentów nieleczonych chirurgicznie są 21:

  • Napady anoksemiczne (68%)
  • Incydenty naczyniowo-mózgowe (17%)
  • Ropnie mózgu

Z czasem u pacjentów z nieleczoną tetralogią Fallota dochodzi do nasilenia objawów 27:

  • Nasilenie sinicy
  • Częstsze i cięższe napady anoksemiczne
  • Postępujące ograniczenie tolerancji wysiłku
  • Poliglobulia (zwiększenie liczby czerwonych krwinek) jako mechanizm kompensacyjny przy przewlekłej hipoksemii 19
  • Zwiększone ryzyko infekcyjnego zapalenia wsierdzia 3
  • Zwiększone ryzyko zaburzeń rytmu serca (arytmie) 3
  • Przerost prawej komory i ostatecznie niewydolność prawokomorowa 33

Progresja po korekcji chirurgicznej

Większość dzieci z tetralogią Fallota jest poddawana korekcji chirurgicznej w pierwszym roku życia, co znacząco poprawia rokowanie 35. Po udanej operacji następuje 36:

  • Zmniejszenie sinicy lub jej ustąpienie
  • Poprawa utlenowania krwi
  • Ustąpienie napadów anoksemicznych
  • Poprawa tolerancji wysiłku
  • Normalizacja wzrostu i rozwoju fizycznego

W pierwszych dniach po operacji serce może funkcjonować gorzej ze względu na przerost i sztywność prawej komory oraz osłabienie jej kurczliwości po chirurgicznym nacięciu, jednak stan ten zazwyczaj poprawia się w kolejnych dniach 37.

Pomimo skutecznej korekcji chirurgicznej, pacjenci z tetralogią Fallota wymagają dożywotniej opieki kardiologicznej ze względu na możliwe długoterminowe powikłania 3135. Najczęstsze problemy po operacji to:

Niedomykalność zastawki płucnej

Jednym z najczęstszych powikłań po korekcji tetralogii Fallota jest niedomykalność zastawki płucnej (pulmonic regurgitation), która rozwija się u większości pacjentów po operacji 3738. W trakcie operacji zastawka płucna jest często poszerzana w celu usunięcia zwężenia, co prowadzi do jej niepełnego zamykania się i wstecznego przepływu krwi 39.

Konsekwencje niedomykalności zastawki płucnej 4041:

  • Stopniowe powiększanie prawej komory serca
  • Pogorszenie funkcji prawej komory
  • Zmniejszenie tolerancji wysiłku i łatwiejsze męczenie się
  • Zwiększone ryzyko zaburzeń rytmu serca (arytmii)
  • Niedomykalność zastawki trójdzielnej jako konsekwencja powiększenia prawej komory

W ciągu 30 lat po korekcji, około 50% pacjentów będzie wymagało ponownej operacji, najczęściej z powodu niedomykalności zastawki płucnej 38. Wymiana zastawki płucnej wskazana jest, gdy niedomykalność powoduje istotne objawy kliniczne lub prowadzi do powiększenia i dysfunkcji prawej komory 42.

Nawracające zwężenie drogi odpływu prawej komory

U niektórych pacjentów może dojść do ponownego zwężenia drogi odpływu prawej komory lub gałęzi tętnic płucnych, co prowadzi do zwiększonego obciążenia prawej komory 37. Objawy nawracającego zwężenia mogą obejmować 43:

  • Pogorszenie tolerancji wysiłku
  • Duszność wysiłkowa
  • Szmer sercowy
  • Przerost i dysfunkcja prawej komory

Zaburzenia rytmu serca

Pacjenci po korekcji tetralogii Fallota mają zwiększone ryzyko arytmii, które mogą rozwinąć się w dłuższej perspektywie czasowej 44. Około 1/3 dorosłych pacjentów doświadcza zaburzeń rytmu w ciągu 2-3 dekad po operacji 44.

Występujące zaburzenia rytmu serca to 4041:

  • Tachyarytmie przedsionkowe
  • Tachykardia komorowa
  • Zaburzenia przewodzenia

Arytmie mogą powodować 40:

  • Kołatanie serca
  • Zawroty głowy
  • Omdlenia
  • Zwiększone ryzyko nagłego zgonu sercowego (ocenia się, że dotyczy 1-5% pacjentów po korekcji) 44

Problemy rozwojowe i neurologiczne

Niektóre dzieci po operacji tetralogii Fallota mogą mieć problemy z normalnym rozwojem i uczeniem się 45. Może to być związane z:

Rokowanie długoterminowe

Długoterminowe rokowanie dla pacjentów po korekcji tetralogii Fallota jest na ogół dobre, choć nie jest to leczenie całkowicie naprawcze 47. Wskaźniki przeżycia 30-letniego po operacji wynoszą 68,5-90,5% 48.

Większość pacjentów po korekcji chirurgicznej tetralogii Fallota prowadzi aktywne życie z dobrą jakością życia, choć mogą mieć pewne ograniczenia w zakresie intensywnego wysiłku fizycznego lub sportów wyczynowych 49. Jednak dorośli pacjenci po korekcji tetralogii Fallota mają zwiększone ryzyko wystąpienia powikłań sercowo-naczyniowych w młodszym wieku w porównaniu do zdrowej populacji 38.

Po 10-15 latach od reoperacji (np. wymiany zastawki płucnej) większość pacjentów jest wolna od objawów, jednak w piątej dekadzie życia objawy mogą powrócić 44, co może wymagać kolejnych interwencji.

Pacjenci z tetralogią Fallota wymagają dożywotniej specjalistycznej opieki kardiologicznej i regularnych badań kontrolnych, aby monitorować potencjalne odległe powikłania 3550.

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  1. 10.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Tetralogy of Fallot – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK513288/
    Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease, representing 5% to 7% of all congenital heart defects. […] Clinical presentation varies based on the severity of the right ventricular outflow tract obstruction (RVOTO), most commonly presenting as a cyanotic neonate. […] Patients with TOF and pulmonary atresia are usually cyanotic in the newborn period, with worsening cyanosis as the ductus arteriosus closes. […] The clinical manifestations of patients with TOF and absent pulmonary valves depend on the severity of respiratory distress, ranging from those with no respiratory distress to those with severe respiratory distress due to trachea and main stem bronchi compression by the massive pulmonary artery branches. […] „Tet spells” or hypercyanotic episodes can occur in infants or toddlers with unrepaired TOF, characterized by tachypnea and hyperpnea and a decrease in the intensity of the cardiac murmur.
  • #2 Tetralogy of Fallot (ToF): Symptoms, Causes & Treatment
    https://my.clevelandclinic.org/health/diseases/22343-tetralogy-of-fallot
    Tetralogy of Fallot is a congenital heart condition, which means its present at birth. ToF makes it hard to get enough oxygen to your body because of four issues in your hearts structure. Surgery in infancy repairs the problems and helps blood flow better, but youll need lifelong follow-ups with a provider. […] A baby born with tetralogy of Fallot has four issues in their heart that cause problems with getting oxygen to their body. […] These issues make it hard for your babys heart to send enough oxygen to their entire body. This matters because your body needs oxygen to function. […] Rarely, some people dont get treatment in childhood and may develop issues in adulthood. […] If your baby has tetralogy of Fallot, your provider will most likely hear a heart murmur when listening to your babys heart.
  • #3 Tetralogy of Fallot (TOF) (for Parents) | Nemours KidsHealth
    https://kidshealth.org/en/parents/tetralogy-of-fallot.html
    Tetralogy of Fallot (fah-LO) is a congenital (present at birth) heart defect. In tetralogy of Fallot (TOF), four related heart defects change the way blood flows to the lungs and through the heart. […] In most cases of tetralogy of Fallot, too little blood goes to the lungs. This low-oxygen (blue) blood then circulates to the rest of the body, and too little oxygen reaches the body tissues. […] Babies with TOF often have cyanosis (sy-eh-NO-sis) a blue or purple tint to the baby’s skin, lips, and fingernails. […] If tetralogy of Fallot isn’t treated, a child may have: cyanosis that gets worse, spells where the blood oxygen levels become very low, dizziness, fainting, or seizures, a higher risk of endocarditis, an infection of the inner layer of the heart, high pressure in the right side of the heart that can cause an irregular heartbeat (an arrhythmia).
  • #4 Tetralogy of Fallot (ToF) – RCEMLearning
    https://www.rcemlearning.co.uk/modules/congenital-heart-disease-in-the-emergency-department/lessons/righttoleft-shunts/topic/tetralogy-of-fallot-tof/
    TOF is the most common cause of cyanotic CHD. […] The greater the degree of obstruction to pulmonary blood flow, the larger the right-to-left shunt and the worse the desaturation. […] Time of manifestation depends on the severity of right ventricular outflow tract obstruction and thus the amount of pulmonary blood flow; severe obstruction results in cyanosis in the newborn period and presents in the first week of life with cyanosis or collapse. […] The degree of RV outflow obstruction determines the age and symptoms at presentation. […] Newborn: Cyanosis at this stage may not be obvious, although a few with severe RVOTO may present with severe cyanosis in the first few days of life (secondary to reduced pulmonary blood flow). […] Older Children: May present with pulmonary over-circulation and CHF (Net left-to-right flow through the VSD)- pink TOF (adequate pulmonary flow).
  • #5 Tetralogy of Fallot – Pediatric Cardiology Associates of Houston
    https://www.kidsheartshouston.com/answers/21859-tetralogy-of-fallot2
    Tetralogy of Fallot is a cyanotic heart defect. The term cyanosis means a bluish discoloration of the skin. The cause of cyanosis is a lower than normal blood oxygen level. Children with tetralogy of Fallot are at risk for cyanosis because the narrowing of blood flow to the lungs in combination with a VSD or hole allows blood in many instances to bypass the lungs and go directly up to the body. The severity of cyanosis, and therefore the severity of symptoms, is determined by the severity of pulmonary stenosis. Babies with more severe pulmonary stenosis are usually very cyanotic at birth and often need immediate attention. Children with less significant pulmonary stenosis may have normal or near-normal blood oxygen levels and may be seemingly fine at birth and for several months thereafter. In this case, the child is often identified by the presence of an asymptomatic heart murmur.
  • #6 Tetralogy of Fallot – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/tetralogy-of-fallot/symptoms-causes/syc-20353477
    Tetralogy of Fallot symptoms depend on how much blood flow is blocked from leaving the heart to go to the lungs. Symptoms may include: […] Babies with tetralogy of Fallot often have blue or gray skin color due to low oxygen levels. […] Some babies with tetralogy of Fallot suddenly develop deep blue or gray skin, nails, and lips. This usually happens when the baby cries, eats or is upset. These episodes are called tet spells. […] Seek medical help if you notice that your baby has these symptoms: Trouble breathing. Bluish color of the skin. Lack of alertness. Seizures. Weakness. More irritable than usual. […] If your baby becomes blue or gray, place your baby on the side and pull the baby’s knees up to the chest. This helps increase blood flow to the lungs. Call 911 or your local emergency number immediately. […] If the heart changes and symptoms are mild, tetralogy of Fallot may not be noticed or diagnosed until adulthood.
  • #7 Tetralogy of Fallot
    https://www.webmd.com/heart-disease/tetralogy-fallot
    Most infants with tetralogy of Fallot develop cyanosis in the first year of life. […] The skin, lips, and mucous membranes inside the mouth and nose take on a noticeably dusky blue color. […] A small number of children with tetralogy of Fallot never turn blue at all, especially if the pulmonary stenosis is mild, the ventricular septal defect is small, or both. […] In some children, the cyanosis is quite subtle and may go undetected for some time. […] Growth and development are slower, especially if the pulmonary stenosis is severe. Puberty may be delayed if the tetralogy is untreated. […] The child usually tires easily and begins panting with any form of exertion. They may play for only a short time before sitting or lying down. […] Once able to walk, the child often assumes a squatting position to catch their breath and then resumes physical activity. Squatting increases the pressure transiently in the aorta and left ventricle, causing less blood to move into the left ventricle, and more out the pulmonary artery to the lungs.
  • #8 Tetralogy of Fallot | American Heart Association
    https://www.heart.org/en/health-topics/congenital-heart-defects/about-congenital-heart-defects/tetralogy-of-fallot
    Infants and young children with unrepaired tetralogy of Fallot are often blue (cyanotic). The reason is that some oxygen-poor blood is pumped to the body through the hole in the wall between the right and left ventricle instead of being pumped to the lungs. […] Most patients are diagnosed with tetralogy of Fallot as infants or young children. Patients with unrepaired tetralogy of Fallot are usually blue (cyanotic). This is true in infants, children and adults with unrepaired tetralogy of Fallot. […] Some long-term problems can include leftover or worsening obstruction between the right pumping chamber and the lung arteries. Children with repaired tetralogy of Fallot have a higher risk of heart rhythm disturbances called arrhythmias. Sometimes these may cause dizziness or fainting. […] The surgery to open up the pulmonary valve often leads to a leaky pulmonary valve. Although this is better tolerated than the original abnormality, the leaky valve can eventually cause problems. There also can be obstructed pathways that are left behind or develop as the patient grows. Both leaky valves and obstructed pathways can cause problems for adult patients with tetralogy of Fallot. In many cases, another surgery to replace the pulmonary valve may be needed in adolescence or adulthood.
  • #9 Tetralogy of Fallot – Wikipedia
    https://en.wikipedia.org/wiki/Tetralogy_of_Fallot
    At birth, children may be asymptomatic or present with many severe symptoms. […] Later in infancy, there are typically episodes of bluish colour to the skin due to a lack of sufficient oxygenation, known as cyanosis. […] When affected babies cry or have a bowel movement, they may undergo a „tet spell” where they turn cyanotic, have difficulty breathing, become limp, and occasionally lose consciousness. […] Other symptoms may include a heart murmur, finger clubbing, and easy tiring upon breastfeeding. […] Depending on the degree of obstruction, symptoms vary from no cyanosis or mild cyanosis to profound cyanosis at birth. […] If the baby is not cyanotic, then it is sometimes referred to as a „pink tet”. […] Other symptoms include a heart murmur which may range from almost imperceptible to very loud, difficulty in feeding, failure to gain weight, retarded growth and physical development, labored breathing (dyspnea) on exertion, clubbing of the fingers and toes, and polycythemia.
  • #10 Tetralogy of Fallot (TOF) | Phoenix Children’s Hospital
    https://phoenixchildrens.org/specialties-conditions/tetralogy-fallot-tof
    Some children with TOF may only have slightly lower than normal oxygen levels in their blood. These children have minimal narrowing of the pulmonary artery. They dont usually have bluish skin (cyanosis). Other children with TOF will have low oxygen levels in their blood. These children have more severe narrowing of the pulmonary artery. They have bluish skin. This is from the low oxygen levels in their blood. […] Symptoms can show up a bit differently in each child. The most common symptom is a bluish color of the skin, lips, and nail beds. This may come on in sudden spells, called TET spells. It happens when blood oxygen level drops quickly. During the spell, babies may have a hard time breathing. They may also be tired and fussy. In the most severe cases, they may lose consciousness. […] The symptoms of TOF may be similar to symptoms caused by other problems. Make sure that your child sees a healthcare provider for a diagnosis.
  • #11 Tetralogy of Fallot – Seattle Children’s
    https://www.seattlechildrens.org/conditions/tetralogy-of-fallot/
    The main effect of tetralogy of Fallot is that your babys blood does not carry as much oxygen as it should. A low oxygen level can make the babys skin, lips and fingernails look blue or purple tinged. This is called cyanosis. […] Cyanosis can range from mild to severe. It depends on how much oxygen-poor (blue) blood makes it to your babys lungs and how much goes out to their body. Some babies with this condition appear bluish at birth. Some do not, but they may become cyanotic later as their pulmonary stenosis gets worse. […] Your baby may also have trouble feeding and gaining weight normally, which doctors call failure to thrive. […] Some babies with tetralogy of Fallot have episodes called tet spells, when they suddenly turn bluish and may faint. These spells are serious. […] Tet spells happen most often in babies between 2 and 4 months old.
  • #12 Tetralogy of Fallot > Fact Sheets > Yale Medicine
    https://www.yalemedicine.org/conditions/tetralogy-of-fallot
    Symptoms include bluish skin, difficulty breathing while eating, poor growth, shortness of breath, heart murmur […] Babies with tetralogy of Fallot may experience tet spells, which are sudden, potentially life-threatening instances when the skin becomes even more bluish, coupled with difficulty breathing; it occurs when babies cry, eat, or have bowel movements. In these moments, more blood than usual may travel through the VSD, resulting in even less oxygen-rich blood traveling throughout the body. Tet spells may cause fainting. […] About two-thirds of babies who dont receive surgical intervention for tetralogy of Fallot die from complications relating to lack of oxygen during tet spells.
  • #13 Tetralogy of Fallot (TOF): Symptoms & Causes | NewYork-Presbyterian
    https://www.nyp.org/heart/congenital-heart-disease/tetralogy-of-fallot-tof
    Tetralogy of Fallot (TOF) is a congenital heart defect that affects infants and young children. It is caused by four heart defects that allow low-oxygen blood to flow from the right side of the heart and throughout the body. […] Common tetralogy of Fallot symptoms include: […] Cyanosis – The babys lips, skin, and nail beds turn blue in color due to low levels of oxygen. If oxygen levels are too low, the baby will stay in the hospital for extra care. […] Rapid breathing or shortness of breath, particularly while playing or during feeding […] Fatigue, problems with feeding, and irritability – These symptoms sometimes occur later, when the tetralogy of Fallot has not yet been repaired, and the pathway to the lungs is getting narrower and tighter. All are signs of lower oxygen levels. […] Babies and young children with tetralogy of Fallot may experience tet spells caused by a sudden drop of oxygen levels in the blood. Tet spell episodes can produce sudden, alarming symptoms, most commonly in infants 2 to 4 months old.
  • #14 Tetralogy of Fallot
    https://www.webmd.com/heart-disease/tetralogy-fallot
    Episodes of extreme blue coloring (called hypercyanosis or simply „tet spells”) occur in many children, usually in the first 2-3 years of life. […] The child suddenly becomes blue, has difficulty breathing, and may become extremely irritable or even faint. […] 20%-70% of children with tetralogy of Fallot experience these spells. […] The spells often happen during feeding, crying, straining, or on awakening in the morning. […] Spells can last from a few minutes to a few hours.
  • #15 Tetralogy of Fallot – Seattle Children’s
    https://www.seattlechildrens.org/conditions/tetralogy-of-fallot/
  • #16 Tetralogy of Fallot – Wikipedia
    https://en.wikipedia.org/wiki/Tetralogy_of_Fallot
    The baby may turn blue with breastfeeding or crying. […] Infants and children with unrepaired tetralogy of Fallot may develop hypercyanotic, or „tet,” spells. […] Patients with prominent subvalvar muscle bundles and/or conal tissue in the right ventricular outflow tract are thought to be at higher risk for hypercyanotic spells. […] These are acute spells characterized by profound cyanosis, often in the setting of agitation or tachycardia, that may progress to loss of consciousness or cardiac arrest if not aborted. […] This may be initiated by any event such as anxiety, pain, dehydration, or fever that leads to an increase in dynamic muscular obstruction of the right ventricular outflow tract. […] The pathophysiology of these episodes is multifactorial; increased sympathetic activation (from pain, agitation, fever, etc.) leads to increased myocardial contractility, which worsens dynamic muscular obstruction of the right ventricular outflow tract, and increased heart rate (tachycardia), which allows less time for right ventricular diastolic filling.
  • #17 Tetralogy of Fallot – Pediatric Cardiology Associates of Houston
    https://www.kidsheartshouston.com/answers/21859-tetralogy-of-fallot2
    One of the risks of tetralogy of Fallot is what are called tet spells. A tet spell is an episode in which a child or infant becomes extremely blue and frequently agitated and out of breath. The spell is caused by a relatively sudden decrease in blood flow to the lungs. Tet spells can be precipitated by a number of things, including dehydration, agitation, or fever. Some children may have spells in the absence of any identifiable cause. Treatment of tet spells includes calming the patient, and administering oxygen or fluids if available. In many instances placing a child in what is called the knee chest position will assist in terminating a spell. […] Even in the absence of symptoms, tetralogy of Fallot ultimately causes significant problems in a number of ways. Long-standing cyanosis causes the body to produce more red blood cells. Over time this can clog the circulation and lead to several long-term negative consequences. In addition, the presence of a hole in the middle of the heart can predispose older patients to strokes and infections. Finally, the presence of long-standing pulmonary stenosis can cause thickening and ultimate failure of the right ventricle.
  • #18 Tetralogy of Fallot | Symptoms, Diagnosis & Treatment
    https://www.cincinnatichildrens.org/health/t/tof
    Tetralogy of Fallot is most often diagnosed in the first few weeks of life due to either a loud murmur or cyanosis. […] As the ductus arteriosus closes, which it typically will in the first days of life, cyanosis can develop or become more severe. […] Rapid breathing in response to low oxygen levels and reduced pulmonary blood flow can occur. […] The arterial oxygen saturation of babies with tetralogy of Fallot can suddenly drop markedly. This phenomenon, called a „tetralogy spell,” usually results from a sudden increased constriction of the outflow tract to the lungs so that pulmonary blood flow is further restricted. […] Children having a tetralogy spell will initially become extremely irritable in response to the critically low oxygen levels, and they may become sleepy or unresponsive if the severe cyanosis persists.
  • #19 Tetralogy of Fallot – WikEM
    https://wikem.org/wiki/Tetralogy_of_Fallot
    Most common cyanotic CHD manifesting in post-infancy period. […] Acute episodes of hypoxia and cyanosis caused by right-to-left shunting across the VSD (’Tet Spells’). […] Patients may present with irritability, agitation, grunting, crying, and central cyanosis. […] During cyanotic spells, there is either increased pulmonary outflow obstruction or decreased systemic vascular resistance leading to right-to-left shunting. […] During the spell there is hypercarbia and hypoxemia (which further increases pulmonary vascular resistance). The process compounds itself creating worsening right-to-left shunting, hyperpnea, right outflow tract obstruction and increased systemic venous return. […] Acute respiratory distress (Tet Spells) due to increased right outflow tract obstruction. […] Chronic hypoxemia causes a compensatory polycythemia and clubbing of the fingers and toes.
  • #20 Tetralogy of Fallot With Pulmonary Stenosis Treatment & Management: Approach Considerations, Conservative Therapy, Transcatheter Interventions
    https://emedicine.medscape.com/article/2063480-treatment
    The prognosis of patients with unrepaired tetralogy of Fallot (TOF) is inferior to the life expectancy of those undergoing repair. […] The nature of the right ventricular (RV) outflow tract obstruction (RVOTO) often dictates the symptoms. In cyanotic tetralogy of Fallot, hypercyanotic episodes (tetralogy of Fallot spells) may occur with agitation or irritability. If the episode is profound, the child develops severe cyanosis, often with hypotension, hemodynamic instability, and altered consciousness. […] Patients whose condition is refractory to medical management and stabilization require urgent surgical intervention. […] In asymptomatic patients, some centers advocate that elective repair be performed from the neonatal period, whereas other centers wait until age 1 year. Most surgeons repair the infant with asymptomatic tetralogy of Fallot between ages 4 and 6 months.
  • #21 Tetralogy of Fallot – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK513288/
    Adult patients with TOF may have dyspnea and exercise intolerance. […] Patients with TOF and absent pulmonary valves usually have to-and-fro systolic and diastolic murmurs and a single-second heart sound. […] The degree of obstructive airway disease among these patients varies markedly. […] Patients with unrepaired TOF have an estimated survival rate of 66% at 1 year of age, 40% at 3 years, 11% at 20 years, 6% at 30 years, and 3% at 40 years. […] Without surgical treatment, the survival rate of patients with TOF without additional cardiac defects is approximately 66% at 1 year of age, 40% at 3 years, 11% at 20 years, 6% at 30 years, and 3% at 40 years. […] The prognosis of these patients is also affected by the need for pulmonary valve replacement due to progressive pulmonary insufficiency, which places them at risk of bacterial endocarditis. […] The most frequent causes of mortality in patients with no surgical intervention include hypoxic spells (68%), cerebrovascular accidents (17%), and brain abscesses.
  • #22 Tetralogy of Fallot – Pediatrics – Merck Manual Professional Edition
    https://www.merckmanuals.com/professional/pediatrics/congenital-cardiovascular-anomalies/tetralogy-of-fallot
    Tetralogy of Fallot consists of 4 features: a large ventricular septal defect, right ventricular outflow tract obstruction and pulmonic valve stenosis, right ventricular hypertrophy, and over-riding of the aorta. Symptoms include cyanosis, dyspnea with feeding, poor growth, and hypercyanotic „tet” spells (sudden, potentially lethal episodes of severe cyanosis). […] Hypercyanotic spells may be precipitated by activity and are characterized by paroxysms of hyperpnea (rapid and deep respirations), irritability and prolonged crying, increasing cyanosis, and decreasing intensity or disappearance of the heart murmur. The spells occur most often in young infants; peak incidence is age 2 to 4 months. A severe spell may lead to limpness, seizures, and occasionally death. […] Manifestations depend on the degree of right ventricle outflow obstruction; severely affected neonates have marked cyanosis, dyspnea with feeding, poor weight gain, and a harsh grade 3 to 5/6 systolic ejection murmur. The murmur comes from the pulmonary stenosis; the VSD shunt is silent.
  • #23 Partners in Care | Tetralogy of Fallot treatment at the Texas Center…
    https://partnersincare.health/conditions/tetralogy-of-fallot
    Children who have tetralogy of Fallot cannot deliver enough oxygenated blood to the body and as a result, the skin, lips, and nails may look blue (cyanosis). […] Tetralogy of Fallot symptoms vary depending on the severity of the defects. […] Tetralogy of Fallot symptoms may include: Blue-tinged skin, lips, and nails when the baby cries or feeds, Cool, clammy skin, Heart murmur, Irritability, Lethargy, Pale, ashen skin, Poor growth, Tet spells, Trouble breathing or feeding. […] Most babies recover well after treatment for tetralogy of Fallot, but there can be lifelong complications, such as an irregular heartbeat, restricted blood flow, or a leaky heart valve.
  • #24 Tetralogy of Fallot Signs & Symptoms | Rush | Rush System
    https://www.rush.edu/kids/conditions/tetralogy-fallot
    In babies and children with tetralogy of Fallot, the blood leaving the heart doesnt get enough oxygen to feed the rest of the body. This can cause a variety of issues, including the following: […] As with many congenital heart defects, signs and symptoms of tetralogy of Fallot typically appear right after or within a few weeks of birth. One of the most common telltale signs is a bluish tint to the skin, lips and fingernails or cyanosis due to low oxygen levels in the blood. […] You may also notice the following issues with your baby: Has difficulty breathing, Grows very tired and limp, Doesnt respond to your voice or touch, Acts fussy, Loses consciousness, Gets very tired during feedings (and, as a result, may not be gaining weight normally), Has clubbed (widened and rounded) fingers. […] Because babies who are treated for tetralogy of Fallot are vulnerable to future heart problems, they require lifelong monitoring and care to stay as healthy as possible.
  • #25 Tetralogy of Fallot: MedlinePlus Medical EncyclopediaLock
    https://medlineplus.gov/ency/article/001567.htm
    Symptoms include: […] Bluish color to the skin (cyanosis) due to low oxygen level in the blood, which gets worse when the baby is upset […] Passing out […] Poor development […] Squatting during episodes of cyanosis (which increases blood flow to the lungs).
  • #26 Fallot’s Tetralogy: Symptoms and Treatment | Doctor
    https://patient.info/doctor/fallots-tetralogy
    Poor feeding, breathlessness and agitation. […] Dyspnoea on exertion (usually after prolonged crying) is common. […] Squatting to rest whilst exercising is characteristic of a right-to-left shunt and presents in an older child. […] Cyanosis occurs and indicates the need for surgical repair. […] Development and puberty may be delayed. […] Hypoxic spells are potentially lethal, unpredictable episodes that occur even in non-cyanotic patients with TOF. These are known as 'tet spells’ and consist of prolonged crying, intense cyanosis and decreased intensity of the murmur of pulmonic stenosis. […] The rare patient may remain marginally and imperceptibly cyanotic, or acyanotic and asymptomatic, into adult life. […] In the now rare situation, an older child or adult with long-standing cyanosis (without surgery) may present with the following signs:
  • #26 Fallot’s Tetralogy: Symptoms and Treatment | Doctor
    https://patient.info/doctor/fallots-tetralogy
    Cardiac […] Right ventricular predominance on palpation or possibly a bulging left hemithorax. […] Systolic thrill at the lower left sternal border. […] Aortic ejection click. […] A patient without cyanosis has a long, loud, systolic murmur with a thrill along the right ventricular outflow tract (RVOT). […] Single S2 – pulmonary valve closure not heard. […] Systolic ejection murmur – varies in intensity inversely with the degree of RVOT obstruction. […] Cyanotic patients have greater obstruction and a softer murmur. […] General appearance […] Cyanosis and clubbing are variable. […] Scoliosis is common. […] Ophthalmological […] Vessels in the retina appear engorged. […] Respiratory […] Haemoptysis. […] Prognosis […] If left untreated, TOF has a survival rate of 11% at 20 years and 3% at 40 years.18 Untreated TOF also causes delayed growth and development, including delayed puberty.
  • #27 Tetralogy of Fallot Symptoms: A Guide for Parents | Medical Daycare PPEC
    https://www.sparkpediatrics.com/post/tetralogy-of-fallot-symptoms-a-guide-for-parents
    Is your child exhibiting Tetralogy of Fallot symptoms? […] Noticing your baby has a bluish tint to their skin or struggles to catch their breath is incredibly scary. […] The most common symptoms often relate to low oxygen levels. Keep in mind that symptoms can vary, and some might be subtle at first. […] One of the most noticeable signs is cyanosis a bluish tint to your baby’s skin, lips, or fingernails. […] Does your baby seem to breathe much faster than usual (tachypnea) or get easily out of breath? Because the heart has to work harder, babies with TOF often show signs of breathing difficulties. […] Feeding can be exhausting for a baby whose heart and lungs are working overtime. This can lead to feeding difficulties, taking in less milk, and slower weight gain. […] Without treatment, TOF symptoms often become more noticeable as a child grows. They might tire more easily or become bluer during activity.
  • #27 Tetralogy of Fallot Symptoms: A Guide for Parents | Medical Daycare PPEC
    https://www.sparkpediatrics.com/post/tetralogy-of-fallot-symptoms-a-guide-for-parents
    Consistent medical care is key. […] Seek emergency medical help immediately if your child: Has severe bluish skin that doesn’t improve, Has significant trouble breathing, Loses consciousness or becomes very limp, Has a suspected Tet spell. […] Recognizing the signs of Tetralogy of Fallot is the first, brave step in getting your child the care they need.
  • #28 Tetralogy of Fallot | Children’s Hospital Los Angeles
    https://www.chla.org/tetralogy-fallot
    Tetralogy of Fallot symptoms vary and depend on the level of blood flow blockage to the lungs. Babies and children who have Tetralogy of Fallot experience episodes of cyanosis (lack of oxygen). Cyanosis is visible in the skin, lips and fingernails; these areas appear blueish on light skin and greyish on dark skin. […] Other signs and symptoms may include: Shortness of breath and rapid breathing, especially during feeding or exercise; Tiring easily during play or exercise; Heart murmur; Loss of consciousness (fainting); Clubbed fingers and toes—an abnormal, rounded nail bed shape; Poor weight gain; Irritability; Prolonged crying; Tet spells—sudden cyanosis, breathing difficulties and restlessness while crying or feeding (most common in infants).
  • #29 Tetralogy of Fallot | UCSF Department of Surgery
    https://surgery.ucsf.edu/condition/tetralogy-fallot
    In years past, when tetralogy of Fallot wasn’t treated in infancy, children would get very tired during exercise and could faint. This heart defect is now repaired in infancy to prevent symptoms like this. […] Another common sign of tetralogy of Fallot is a heart murmur. A heart murmur is an extra or unusual sound that doctors may hear while listening to the heart. […] Normal growth and development depend on a normal workload for the heart and normal flow of oxygen-rich blood to all parts of the body. Babies who have tetralogy of Fallot may not gain weight or grow as quickly as children who have healthy hearts because they tire easily while feeding. […] Children who have tetralogy of Fallot also may have clubbing. Clubbing is the widening or rounding of the skin or bone around the tips of the fingers.
  • #30 Tetralogy of Fallot: Causes and Symptoms
    https://www.massgeneral.org/children/tetralogy-of-fallot
    Many babies with Tetralogy of Fallot (TOF) will not have symptoms. In more severe cases, babies will have low oxygen levels in their blood and have a blue color to their skin. […] Common symptoms of TOF include: […] Cyanosis (blue color) of the skin, especially around the lips, fingertips or toes […] Breathing faster than usual […] Poor weight gain […] Heart murmur (an extra sound the heart makes when listening with a stethoscope)
  • #31 Tetralogy of Fallot (TOF) (for Parents) | Nemours KidsHealth
    https://kidshealth.org/en/parents/tetralogy-of-fallot.html
    Children whose TOF isn’t fixed usually become increasingly blue over time and have difficulty participating in physical activities (like playing sports). Most babies who have surgery to correct the defect do very well and can participate in normal kid activities. […] Cyanosis is a very common sign. […] A child with TOF might have sudden episodes of deep cyanosis, called „hypercyanotic spells” or „Tet spells,” during crying or feeding. […] Other signs include: heart murmur, fussiness, tiring easily, trouble breathing, rapid heartbeat (palpitations), fainting, „clubbing,” where the skin or bones around the tips of fingers are widened or rounded (this happens over time in a child with cyanosis). […] Doctors repair tetralogy of Fallot through open-heart surgery soon after birth or later in infancy, depending on the baby’s health and weight and severity of defects and symptoms. […] Most babies whose tetralogy of Fallot is repaired do very well. After surgery, they will need regular follow-up visits with a heart specialist, even as adults.
  • #32 Tetralogy of Fallot (TOF) | Causes, symptoms, & treatment | Children’s Wisconsin
    https://childrenswi.org/medical-care/herma-heart/conditions/tetralogy-of-fallot
    Some children become pale or ashen in color, and may have cool, clammy skin. […] The amount of oxygen-poor (blue) blood that passes through the VSD to the left side of the heart varies. If the right ventricle obstruction is severe, or if the pressure in the lungs is high, a large amount of oxygen-poor (blue) blood passes through the VSD, mixes with the oxygen-rich (red) blood in the left ventricle, and is pumped to the body. […] Soon, nearly all the blood in the left ventricle is oxygen-poor (blue). This is an emergency situation, as the body will not have enough oxygen to meet its needs. […] Some situations, such as crying, increase the pressure in the lungs temporarily, and increasing blueness might be noted as a baby with tetralogy of fallot cries. […] The symptoms of tetralogy of fallot may resemble other medical conditions or heart problems. Always consult your child’s physician for a diagnosis.
  • #33 Tetralogy of Fallot – Wikipedia
    https://en.wikipedia.org/wiki/Tetralogy_of_Fallot
    Untreated, tetralogy of Fallot rapidly results in progressive right ventricular hypertrophy due to the increased resistance caused by narrowing of the pulmonary trunk. […] This progresses to heart failure which begins in the right ventricle and often leads to left heart failure and dilated cardiomyopathy. […] Mortality rate depends on the severity of the tetralogy of Fallot. […] If left untreated, TOF carries a 35% mortality rate in the first year of life, and a 50% mortality rate in the first three years of life. […] Patients with untreated TOF rarely progress to adulthood. […] Patients who have undergone total surgical repair of tetralogy of Fallot have improved hemodynamics and often have good to excellent cardiac function after the operation with some to no exercise intolerance.
  • #34 Tetralogy of Fallot (TOF): Symptoms & Causes | NewYork-Presbyterian
    https://www.nyp.org/heart/congenital-heart-disease/tetralogy-of-fallot-tof
    Symptoms of a tet spell can include: […] Skin suddenly turns deep bluish in color […] Breathing difficulties […] Fainting […] Extreme weakness […] If your baby is exhibiting signs of a tet spell, seek medical attention immediately. […] Left untreated, only 3 of 4 babies will be alive at 1 year, only 3 in 10 at 10 years of age, only 1 in 10 at 20 years old, and less than 1 in 20 at age 40 years. […] Those that do survive have a limited quality of life, as the low oxygen levels keep them from being able to do exercise or strenuous work.
  • #35 Tetralogy of Fallot – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/tetralogy-of-fallot/diagnosis-treatment/drc-20353482
    Tetralogy of Fallot is often diagnosed soon after birth. Your baby’s skin may look blue or gray. A whooshing sound may be heard when listening to the baby’s heart with a stethoscope. This is called a heart murmur. […] All babies who have tetralogy of Fallot need surgery to fix the heart and improve blood flow. A heart surgeon, called a cardiovascular surgeon, does the surgery. The timing and type of surgery depends on the baby’s overall health and specific heart problems. […] After complete repair, the right lower chamber won’t need to work as hard to pump blood. As a result, the right chamber wall should go back to its usual thickness. The oxygen level in the blood goes up. Symptoms typically get better. […] The long-term survival rates for people who’ve had tetralogy of Fallot surgery continue to improve. […] People with tetralogy of Fallot need lifelong care, preferably from a healthcare team that specializes in heart diseases. The health checkups often include imaging tests to see how well the heart is working. Tests also are done to check for surgery complications.
  • #36 Tetralogy of Fallot | Beacon Health System
    https://www.beaconhealthsystem.org/library/diseases-and-conditions/tetralogy-of-fallot?content_id=CON-20316969
    Tetralogy of Fallot symptoms depend on how much blood flow is blocked from leaving the heart to go to the lungs. Symptoms may include: […] Babies with tetralogy of Fallot often have blue or gray skin color due to low oxygen levels. […] Some babies with tetralogy of Fallot suddenly develop deep blue or gray skin, nails, and lips. This usually happens when the baby cries, eats or is upset. These episodes are called tet spells. […] Serious congenital heart defects are often diagnosed before or soon after your child is born. Seek medical help if you notice that your baby has these symptoms: […] If your baby becomes blue or gray, place your baby on the side and pull the baby’s knees up to the chest. This helps increase blood flow to the lungs. […] The long-term survival rates for people who’ve had tetralogy of Fallot surgery continue to improve. […] After complete repair, the right lower chamber won’t need to work as hard to pump blood. As a result, the right chamber wall should go back to its usual thickness. The oxygen level in the blood goes up. Symptoms typically get better.
  • #37 Tetralogy of Fallot | Symptoms, Diagnosis & Treatment
    https://www.cincinnatichildrens.org/health/t/tof
    Most babies are fairly sick in the first few days after surgery, since the right ventricle is „stiff” from the previous hypertrophy (thickness) and because an incision is made into the muscle of the ventricle, making the muscle temporarily weaker. […] This right ventricular dysfunction usually improves significantly in the days following surgery. […] In a small percentage of children, this pulmonary insufficiency can lead to diminished function of the right ventricle. Symptoms of fatigue, especially with exercise, may develop. […] Patients who have had repair of tetralogy of Fallot can also redevelop a narrowing in the right ventricular outflow area or in the branch (left or right) pulmonary arteries, which will cause the right ventricle to pump at abnormally high pressures. […] Most adult patients with tetralogy of Fallot have had surgery to repair it in childhood. […] Unfortunately, the majority do have issues; the most important one is leakage (regurgitation) of the pulmonary valve. This can enlarge the right heart chambers and lead to limitations in physical activity as well as heart rhythm abnormalities and occasionally sudden cardiac death.
  • #38 Tetralogy of Fallot – Wikipedia
    https://en.wikipedia.org/wiki/Tetralogy_of_Fallot
    Long-term outcome is usually excellent for most patients, however residual post-surgical defects such as pulmonary regurgitation, pulmonary artery stenosis, residual VSD, right ventricular dysfunction, right ventricular outflow tract obstruction may affect life expectancy and increase the need for reoperation. […] Cardiovascular and cerebrovascular complications in patients with repaired CHD such as TOF occur earlier in life compared to healthy subjects. […] Chronic pulmonary regurgitation and right ventricular dilation and dysfunction is also common. […] Within 30 years after correction, 50% of patients will require reoperation. […] The most common cause of reoperation is a leaky pulmonary valve (pulmonary valve insufficiency).
  • #39 Tetralogy of Fallot – ACHA
    https://www.achaheart.org/your-heart/educational-qas/types-of-heart-defects/tetralogy-of-fallot/
    ToF causes blood with low oxygen to mix with blood that is oxygen-rich (red) as it enters and leaves your heart. This can make babies look blue, or cyanotic. This is why newborns are sometimes called blue babies. […] People born with ToF do well over time. Many are now in their 60s, 70s and even 80s. […] A surgical repair does not mean that your heart is permanently fixed. You can develop new heart problems as you age. It is important that you stay in the care of an adult congenital heart disease (ACHD) specialist throughout your life. Talk to your doctor regularly so changes in your heart can be detected early, which helps prevent permanent damage. […] The most common problem in adults with ToF is a leaking pulmonary valve, also called pulmonary regurgitation or PR. During the repair, the doctor stretches the pulmonary valve to open it. This almost always makes the valve leak. As you age, the leak can get worse.
  • #40 Tetralogy of Fallot – ACHA
    https://www.achaheart.org/your-heart/educational-qas/types-of-heart-defects/tetralogy-of-fallot/
    It makes the right side of your heart get bigger, affecting your hearts ability to pump. When your heart does not function as well, it can eventually cause heart failure. It increases the chance of developing a dangerously fast heartbeat in the right ventricle, called ventricular tachycardia or VT. If not treated, VT can lead to sudden death. It can cause rhythm problems in the upper chambers, or atria. The tricuspid valve may leak, called tricuspid regurgitation. If both the tricuspid and pulmonary valves leak, the right ventricle will also grow. It can make your aorta just above the aortic valve too large. This may cause a leak to develop in the aortic valve, called aortic regurgitation. […] Sometimes there are few or no symptoms of a leaky valve. If you notice any of these symptoms, let your ACHD cardiologist know: Rhythm problems, such as a fast heartbeat or feeling your heart flutter. Less energy. Shortness of breath on effort. Swelling in your leg and/or belly.
  • #41 Tetralogy of Fallot | Schneeweiss Adult Congenital Heart Center
    https://www.congenitalheart.cuimc.columbia.edu/conditions-we-treat/tetralogy-fallot
    Most patients with tetralogy of Fallot require cardiac surgery early in childhood. […] Successful surgery eliminates cyanosis, relieves the obstruction between the right ventricle and the pulmonary artery, and improves exercise capacity. […] Most adults who were successfully repaired during childhood continue to do well although ongoing follow up with an adult congenital heart disease specialist is important to address potential long term issues such as arrhythmias, right ventricular function, and abnormalities of the pulmonary valve and pulmonary arteries. […] Adults with tetralogy of Fallot may experience atrial or ventricular arrhythmias. […] These arrhythmias can result in heart racing, diminished stamina, dizziness and fluid retention. […] Following repair of tetralogy of Fallot, most patients are left with a leak in their pulmonary valve, known as pulmonary insufficiency.
  • #42 Tetralogy of Fallot – ACHA
    https://www.achaheart.org/your-heart/educational-qas/types-of-heart-defects/tetralogy-of-fallot/
    Your ACHD care team has tools to determine how severe the leak is. They might even hear the leak by listening to your heart. […] The right time to replace a pulmonary valve varies from person to person. Your ACHD doctor will advise you based on your symptoms, the results of heart tests, and the most recent ACHD care guidelines. Because valve replacements do not last forever, your doctor might want to wait as long as possible without hurting your heart. […] People with ToF can develop health problems that affect people without CHD, such as high blood pressure, high cholesterol, obesity, and sleep apnea. Your ACHD doctor can assess and treat these concerns.
  • #43 Tetralogy of Fallot (TOF) in Adults: Practice Essentials, Background, Anatomy
    https://emedicine.medscape.com/article/2035949-overview
    The clinical features of tetralogy of Fallot in the adult are directly related to the severity of the anatomic defects and may include the following: Lack of exercise endurance, Palpitations, Gradual decline in bodily functions. […] Physical findings in adults include the following: Exertional dyspnea, Syncope, Palpitations, Evidence of right-sided heart failure (eg, elevated jugular venous pressure [JVP], ascites, peripheral edema, hepatomegaly), Large „A wave” in JVP tracings, In individuals with pulmonary valve insufficiency: Low-pitched, short diastolic murmur; may have an extra heart sound (ejection click); may have a murmur, RV dysfunction, arrhythmias. […] It is important to understand that corrective surgery for tetralogy of Fallot performed in childhood is not curative surgery. Rather, this procedure only corrects the anatomic defects without changing the progression of the disease or addressing the consequences of using a patch. As a result, these individuals develop new symptoms and eventually present to the cardiology clinic with diverse symptoms.
  • #44 Tetralogy of Fallot (TOF) in Adults: Practice Essentials, Background, Anatomy
    https://emedicine.medscape.com/article/2035949-overview
    With advancing age, children with tetralogy of Fallot who had corrective surgery are usually asymptomatic for the first decade after the initial procedure. Thereafter, they develop adverse myocardial problems, the majority of which involve varying degrees of pulmonary valve insufficiency, which, in turn, can lead to RV overload and RV distention and failure. […] An estimated one third of adults with tetralogy of Fallot experience atrial and ventricular arrhythmias over a period of 2-3 decades. The incidence of sudden death ranges from 6% to 9% in patients who develop these arrhythmias. […] Adult patients with tetralogy of Fallot who undergo surgery again are usually symptom free for 10-15 years, but by the time they reach their fifth decade, even these patients begin to have symptoms. […] Sudden death from ventricular arrhythmia has been reported in 1-5% of patients at a later stage in life, and the cause remains unknown. It has been suspected that ventricular dysfunction may be the cause.
  • #45 Tetralogy of Fallot (TOF) | University Hospitals
    https://www.uhhospitals.org/health-information/health-and-wellness-library/article/diseases-and-conditions—pediatrics/tetralogy-of-fallot-tof
    Some children with TOF may only have slightly lower than normal oxygen levels in their blood. […] Other children with TOF will have low oxygen levels in their blood. These children have more severe narrowing of the pulmonary artery. They have bluish skin. This is from the low oxygen levels in their blood. […] The most common symptom is a bluish color of the skin, lips, and nail beds. This may come on in sudden spells, called TET spells. It happens when blood oxygen level drops quickly. During the spell, babies may have a hard time breathing. They may also be tired and fussy. In the most severe cases, they may lose consciousness. […] After surgery, your child may become tired easily and sleep more. Over time, most children are able to be active. Most children’s appetite and growth become normal soon after surgery. Some children who had surgery for TOF can have problems learning or growing normally.
  • #46 Tetralogy of Fallot Information | Mount Sinai – New York
    https://www.mountsinai.org/health-library/diseases-conditions/tetralogy-of-fallot
    Tetralogy of Fallot is a type of congenital heart defect. Congenital means that it is present at birth. […] Symptoms include: Bluish color to the skin (cyanosis) due to low oxygen level in the blood, which gets worse when the baby is upset. […] Children with tetralogy of Fallot are more likely to have chromosome disorders, such as Down syndrome, Alagille syndrome, and DiGeorge syndrome (a condition that causes heart defects, low calcium levels, and poor immune function). […] Most cases can be corrected with surgery. Babies who have surgery usually do well. More than 90% survive to adulthood and live active, healthy, and productive lives. Without surgery, death often occurs by the time the person reaches age 20. […] Complications may include: Delayed growth and development, Irregular heart rhythms (arrhythmias), Seizures during periods when there is not enough oxygen, Death from cardiac arrest, even after surgical repair. […] Contact your health care provider if new unexplained symptoms develop or the child is having an episode of cyanosis (blue skin).
  • #47 Tetralogy of Fallot (TOF) in Adults: Practice Essentials, Background, Anatomy
    https://emedicine.medscape.com/article/2035949-overview
    Tetralogy of Fallot (TOF) (pronounced te-tral-uh-jee of Fal-oh), one of the most common congenital heart disorders, comprises right ventricular (RV) outflow tract obstruction (infundibular stenosis), ventricular septal defect (VSD), aorta dextroposition (overriding aorta), and RV hypertrophy. The mortality rate in untreated patients reaches 50% by age 6 years, but in the present era of cardiac surgery, children with simple forms of tetralogy of Fallot enjoy good long-term survival with an excellent quality of life. […] However, it is important to understand that corrective surgery for tetralogy of Fallot performed in childhood is not curative surgery. As a result, many children with repaired tetralogy of Fallot survive into adulthood and are seen at cardiology clinics. Some patients who were undiagnosed during childhood may present as adults with a variety of symptoms, and other patients who may only have had a palliative procedure (eg, shunt placement) can also present as adults.
  • #48 Fallot’s Tetralogy: Symptoms and Treatment | Doctor
    https://patient.info/doctor/fallots-tetralogy
    Current surgical treatment produces much improved 30-year survival rates between 68.5-90.5%.19 However residual problems (such as right ventricular outflow tract obstruction and pulmonary regurgitation) are common. They may require further interventions. […] […] […] After 5-20 years following surgery, patients generally have reduced exercise capacity and cardiac output compared with that of healthy individuals.
  • #49 Tetralogy of Fallot (ToF): Symptoms, Causes & Treatment
    https://my.clevelandclinic.org/health/diseases/22343-tetralogy-of-fallot
    Surgery for tetralogy of Fallot creates a normal blood flow path. But you or your child can still have other issues, including: […] Babies with tetralogy of Fallot may be slower to develop and grow. They may need physical, speech or occupational therapy to help them. […] Most babies who have surgery today to fix tetralogy of Fallot will have active lives. But people with repaired ToF typically have some restrictions on certain strenuous activities, like competitive sports. […] Some adults who had tetralogy of Fallot surgery as infants may develop issues that require medicines, medical care or surgery in adulthood. […] If your baby is having a severe tet spell, take them to the closest emergency room. […] Adults whove had initial surgery for ToF as a child should have thorough cardiac evaluations every year or two.
  • #50 Tetralogy of Fallot – BHF
    https://www.bhf.org.uk/informationsupport/conditions/tetralogy-of-fallot
    The symptoms of tetralogy of Fallot are different for adults and children. Symptoms for children may include: […] Symptoms for adults may include: […] Tetralogy of Fallot is a type of congenital heart disease, which means its something youre born with. Many people with tetralogy of Fallot live a normal life, but over time it can cause other problems with your heart like heart failure and high blood pressure. […] A common problem for people with repaired tetralogy of Fallot is a leaky heart valve between the heart and lungs. This is known as pulmonary regurgitation, where your pulmonary valve does not close properly. This lets blood leak back into your heart instead of forwards into your lungs. […] A leaky heart valve puts extra strain on your heart, making it work harder. This can cause symptoms like feeling more tired and out of breath. Over time, it can increase your risk of having a heart attack, stroke and other heart conditions. […] After its been repaired, most people living with tetralogy of Fallot live a normal life. […] Even if you feel well, going to these appointments is important because there may be changes in your heart before you get symptoms.