Tetralogia fallota
Leczenie

Tetralogia Fallota (ToF) to najczęstsza sinicza wada wrodzona serca, wymagająca korekcji chirurgicznej czterech anatomicznych nieprawidłowości. W przypadku noworodków z ciężką sinicą stosuje się terapię pomostową, w tym infuzję prostaglandyny E1 (0,05-0,1 µg/kg/min) dla utrzymania drożności przewodu tętniczego, beta-blokery (np. propranolol) na napady hipoksemiczne, fenylefrynę w opornych przypadkach oraz morfinę dla zmniejszenia napadów. Paliatywne zabiegi, takie jak zespolenie systemowo-płucne, stentowanie przewodu tętniczego, balonowe poszerzenie zastawki płucnej czy stentowanie drogi odpływu prawej komory, przygotowują pacjentów do całkowitej korekcji, zwykle wykonywanej między 3 a 6 miesiącem życia. Operacja obejmuje zamknięcie ubytku przegrody międzykomorowej, poszerzenie drogi odpływu prawej komory oraz naprawę lub wymianę zastawki płucnej, z zastosowaniem różnych technik chirurgicznych, takich jak plastyka zastawki płucnej, łata przezzastawkowa (TAP) czy operacja Rastelliego.

Leczenie Tetralogii Fallota

Tetralogia Fallota (ToF) to najczęstsza sinicza wrodzona wada serca, która wymaga interwencji chirurgicznej w celu skorygowania czterech współistniejących nieprawidłowości anatomicznych. Leczenie tej złożonej wady serca znacznie ewoluowało na przestrzeni lat, a obecne strategie terapeutyczne zapewniają doskonałe długoterminowe przeżycie pacjentów.12

Leczenie medyczne przed zabiegiem operacyjnym

W przypadku noworodków z tetralogią Fallota, które wykazują ciężką sinicę lub hipoksję, stosuje się terapię pomostową przed właściwą korekcją chirurgiczną. Do metod farmakologicznych należy:12

  • Infuzja prostaglandyny E1 (rozpoczynając od dawki 0,05-0,1 µg/kg/min dożylnie) w celu utrzymania drożności przewodu tętniczego i zwiększenia przepływu krwi przez płuca12
  • Beta-blokery (np. propranolol) w celu zmniejszenia nasilenia i częstości napadów hipoksemicznych12
  • Fenylefryna (Neo-Synephrine) w sytuacjach opornych na leczenie w celu zwiększenia oporu naczyniowego i poprawy przepływu płucnego1
  • Morfina w celu zmniejszenia napadu hipoksemicznego poprzez obniżenie napędu oddechowego1

W przypadku napadów hipoksemicznych stosuje się również niefarmakologiczne metody, takie jak ułożenie dziecka w pozycji z kolanami przyciągniętymi do klatki piersiowej, co zwiększa opór systemowy i zmniejsza przeciek prawo-lewy.12

Leczenie chirurgiczne

Podstawowym sposobem leczenia tetralogii Fallota jest zabieg chirurgiczny. Moment przeprowadzenia operacji oraz rodzaj zabiegu zależą od stanu klinicznego dziecka, anatomii wady i doświadczenia ośrodka.12

Operacje paliatywne

W przypadku noworodków z nasiloną sinicą, niską masą urodzeniową lub niedorozwojem tętnic płucnych, zanim będzie możliwa pełna korekcja, wykonuje się zabiegi paliatywne:12

  • Zespolenie systemowo-płucne (zmodyfikowany zespół Blalocka-Taussiga) – polega na umieszczeniu syntetycznego graftu (rurki) między tętnicą podobojczykową lub aortą a tętnicą płucną, co zwiększa przepływ krwi do płuc12
  • Stentowanie przewodu tętniczego (PDA) – alternatywna metoda paliatywna, umożliwiająca utrzymanie przepływu krwi do płuc poprzez implantację stentu w przewodzie tętniczym12
  • Balonowe poszerzenie zastawki płucnej – paliatywna procedura wykonywana w przypadku zwężenia zastawki płucnej przy minimalnym zwężeniu podzastawkowym12
  • Stentowanie drogi odpływu prawej komory (RVOT) – może być korzystne fizjologicznie w porównaniu z chirurgicznym zespoleniem12

Całkowita korekcja chirurgiczna

Całkowita korekcja tetralogii Fallota jest preferowaną metodą leczenia i zazwyczaj wykonywana jest między 3 a 6 miesiącem życia, choć może być przeprowadzona wcześniej lub później w zależności od stanu klinicznego dziecka.12

Operacja polega na:12

  • Zamknięciu ubytku przegrody międzykomorowej (VSD) za pomocą łaty12
  • Poszerzeniu drogi odpływu prawej komory poprzez usunięcie przerośniętego mięśnia podzastawkowego12
  • Naprawie lub wymianie zwężonej zastawki płucnej12
  • Poszerzeniu mniejszych tętnic płucnych, jeśli jest to konieczne12

Istnieją różne techniki chirurgiczne wykonywania całkowitej korekcji, w tym:12

  • Plastyka zastawki płucnej z zachowaniem zastawki (PVS) – w przypadkach, gdy zwężenie dotyczy głównie mięśnia podzastawkowego1
  • Łata przezzastawkowa (TAP) – stosowana, gdy zwężenie obejmuje zastawkę płucną12
  • Operacja Rastelliego – polegająca na użyciu konduitu (rurki) do połączenia prawej komory z tętnicą płucną, często z zastawką wewnątrz1

W przypadku niektórych pacjentów, u których tętnice wieńcowe przebiegają w sposób uniemożliwiający standardową naprawę, stosuje się alternatywne metody, takie jak umieszczenie konduitu między prawą komorą a tętnicą płucną.1

Leczenie po zabiegu chirurgicznym

Po całkowitej korekcji tetralogii Fallota pacjenci wymagają długoterminowej obserwacji pod kątem potencjalnych powikłań. Najczęstsze problemy występujące po korekcji obejmują:12

Niedomykalność zastawki płucnej

Niedomykalność zastawki płucnej jest najczęstszym długoterminowym powikłaniem po korekcji tetralogii Fallota. Około 40% pacjentów w ciągu 35 lat od pierwotnego zabiegu wymaga wymiany zastawki płucnej (PVR).12

Wskazania do wymiany zastawki płucnej obejmują:12

  • Dysfunkcję prawej komory
  • Poszerzenie prawej komory
  • Niedobór czynnościowy
  • Postępujące zmniejszenie tolerancji wysiłku

Wymiana zastawki płucnej może być przeprowadzona chirurgicznie lub, w niektórych przypadkach, za pomocą metod przezskórnych (np. zastawka Melody, Harmony lub Alterra).12

Zaburzenia rytmu serca

Pacjenci po korekcji tetralogii Fallota mają zwiększone ryzyko wystąpienia zaburzeń rytmu serca, które mogą prowadzić do nagłego zgonu sercowego.12

Leczenie zaburzeń rytmu serca może obejmować:12

  • Farmakoterapię
  • Ablację prądem o częstotliwości radiowej
  • Implantację kardiowertera-defibrylatora (ICD) w przypadku groźnych arytmii komorowych
  • Stymulator serca w przypadku bloku przedsionkowo-komorowego

Inne powikłania wymagające leczenia

Poza niedomykalnością zastawki płucnej i zaburzeniami rytmu serca, pacjenci po korekcji tetralogii Fallota mogą wymagać leczenia następujących stanów:12

  • Resztkowe lub nawracające zwężenie drogi odpływu prawej komory
  • Zwężenie tętnic płucnych, które może być leczone balonoplastyką lub implantacją stentu podczas cewnikowania serca
  • Resztkowy ubytek przegrody międzykomorowej
  • Infekcyjne zapalenie wsierdzia – wymagane może być stosowanie profilaktyki antybiotykowej przed zabiegami dentystycznymi i operacjami

Długoterminowa opieka i monitorowanie

Pacjenci po korekcji tetralogii Fallota wymagają dożywotniej opieki kardiologicznej, najlepiej w ośrodku specjalizującym się w leczeniu wrodzonych wad serca.12

Regularne wizyty kontrolne obejmują:12

  • Badania obrazowe serca (echokardiografia, rezonans magnetyczny) w celu oceny funkcji zastawek i komór
  • Elektrokardiografia do wykrywania zaburzeń rytmu serca
  • Testy wysiłkowe do oceny wydolności fizycznej
  • Badania biomarkerów krwi

Przejście z opieki pediatrycznej do opieki dla dorosłych wymaga współpracy między kardiologami dziecięcymi a specjalistami od wrodzonych wad serca u dorosłych.12

Nowoczesne podejścia terapeutyczne

Przezskórna wymiana zastawki płucnej

Jedną z najważniejszych innowacji w leczeniu długoterminowych powikłań po korekcji tetralogii Fallota jest przezskórna wymiana zastawki płucnej.1 Metoda ta pozwala na implantację zastawki płucnej bez konieczności przeprowadzania otwartej operacji serca, co prowadzi do szybszego powrotu do zdrowia.1

Dostępne systemy przezskórnej wymiany zastawki płucnej obejmują:1

  • Zastawka Melody
  • System Harmony Transcatheter Pulmonary Valve (Medtronic)
  • System Alterra Adaptive Prestent

Długoterminowe powikłania po przezskórnej implantacji zastawki płucnej obejmują złamania stentu i zapalenie wsierdzia.1

Nowe techniki chirurgiczne

Współczesne techniki chirurgiczne stosowane w leczeniu tetralogii Fallota koncentrują się na:12

  • Minimalizacji wentrikulotomii
  • Zachowaniu kompetencji zastawki płucnej
  • Unikaniu istotnego resztkowego zwężenia drogi odpływu prawej komory
  • Hybrydowych procedurach operacyjnych łączących chirurgię z zabiegami przezskórnymi

Wyniki leczenia

Wyniki leczenia tetralogii Fallota znacznie się poprawiły w ostatnich dekadach. Śmiertelność wczesna po całkowitej korekcji wynosi obecnie około 1-3%.12

Długoterminowe wskaźniki przeżycia 30-letniego wzrosły z 68,5% do ponad 90%.12 Większość pacjentów prowadzi aktywne, zdrowe życie po operacji.1

Czynniki wpływające na długoterminowe wyniki obejmują:1

  • Czas przeprowadzenia pierwotnej korekcji
  • Technikę chirurgiczną
  • Stopień niedomykalności zastawki płucnej
  • Obecność zaburzeń rytmu serca
  • Funkcję prawej komory

Perspektywy na przyszłość

Badania nad leczeniem tetralogii Fallota koncentrują się obecnie na:12

  • Opracowaniu całkowicie przezskórnej naprawy tetralogii Fallota jako alternatywy dla operacji chirurgicznej
  • Wykorzystaniu modelowania 3D i nowych materiałów, takich jak bioresorbowalne urządzenia
  • Optymalizacji czasu wymiany zastawki płucnej w celu zapobiegania nieodwracalnej dysfunkcji prawej komory
  • Poprawie trwałości protez zastawek płucnych

Chociaż chirurgiczna naprawa tetralogii Fallota zapewnia doskonałe wyniki w obecnej erze, postępy w kardiologii interwencyjnej mogą w przyszłości umożliwić mniej inwazyjne podejście do leczenia tej złożonej wady serca.1

Podsumowanie leczenia

Leczenie tetralogii Fallota wymaga multidyscyplinarnego podejścia obejmującego kardiologów, kardiochirurgów i specjalistów od kardiologii interwencyjnej. Wczesna diagnoza, odpowiednie leczenie medyczne oraz optymalnie zaplanowana korekcja chirurgiczna umożliwiają większości pacjentów prowadzenie normalnego, aktywnego życia.12

Dożywotnia, regularna opieka kardiologiczna jest niezbędna dla wszystkich pacjentów po korekcji tetralogii Fallota, aby monitorować i leczyć potencjalne długoterminowe powikłania.12

Kolejne rozdziały

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Wybierz kolejny rozdział z menu poniżej, aby otworzyć nową podstronę kompedium wiedzy i uzyskać szczegółowe informację o leku, substancji lub chorobie.

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

Materiały źródłowe

  • #1 Current outcomes and treatment of tetralogy of Fallot
    https://pmc.ncbi.nlm.nih.gov/articles/PMC6719677/
    Tetralogy of Fallot (ToF) is the most common type of cyanotic congenital heart disease. Since the first surgical repair in 1954, treatment has continuously improved. The treatment strategies currently used in the treatment of ToF result in excellent long-term survival (30 year survival ranges from 68.5% to 90.5%). However, residual problems such as right ventricular outflow tract obstruction, pulmonary regurgitation, and (ventricular) arrhythmia are common and often require re-interventions. […] Performing pulmonary valve replacement or relief of pulmonary stenosis before irreversible right ventricular dysfunction occurs is important, but determining the optimal timing of pulmonary valve replacement is challenging for several reasons. […] Different surgical techniques were developed minimizing the extent of the ventriculotomy and trying to preserve competence of the pulmonary valve without causing significant residual RVOTO.
  • #1 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. Therefore, whether to repair tetralogy of Fallot is not often debated. […] Medical management of tetralogy of Fallot spells often includes use of mechanical ventilation, inotropes, and an alpha-agonist such as phenylephrine hydrochloride (Neo-Synephrine) to increase pulmonary blood flow. […] Patients whose condition is refractory to medical management and stabilization require urgent surgical intervention. […] In some centers, these patients are treated with initial surgical palliation with a systemic-to-pulmonary artery shunt and subsequent complete repair, whereas, in other centers, these children are treated with urgent primary complete repair. […] 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.
  • #1 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. […] Definitive treatment is surgical repair. […] For symptomatic neonates, prostaglandin E1 infusion. […] For hypercyanotic spells, knee-chest positioning, calming, oxygen, IV fluids, and sometimes drugs. […] Neonates with severe cyanosis may be palliated with an infusion of prostaglandin E1 (beginning at 0.05 to 0.1 mcg/kg/minute IV) to open the ductus arteriosus and thereby increase pulmonary blood flow. […] Hypercyanotic spells require immediate intervention. […] If the spell persists, standard medical therapy includes morphine, phenylephrine, and beta-blockers (propranolol or esmolol).
  • #1 Tetralogy of fallot medical treatment – wikidoc
    https://www.wikidoc.org/index.php/Tetralogy_of_fallot_medical_treatment
    Although operative repair of tetralogy of Fallot is definitive, medical therapy can be used to manage hypoxic or tet spells. Tet spells cause acute hypoxia and may be treated with beta blockers, morphine, phenylephrine, and oxygen. […] Tet spells cause acute hypoxia and may be treated with: Beta-blockers such as propranolol or esmolol. The beta-blockers causes relaxation of the right ventricular outflow tract and increases blood flow into the pulmonary vessels. Morphine to reduce ventilatory drive. Phenylephrine to increase systemic afterload that in turn increases the flow across right ventricle and the pulmonary artery and decreases right to left shunting. Procedures such as the knee-chest position which increases aortic wave reflection, increasing pressure on the left side of the heart, decreasing the right-to-left shunt thus decreasing the amount of deoxygenated blood entering the systemic circulation. Oxygen is ineffective in treating hypoxic spells as the underlying problem is lack of blood flow through the pulmonary circuit and not alveolar oxygenation. In case all these measures fail, an emergency Blalock-Taussig shunt might be needed. […] Prostaglandins can be administered while awaiting surgery to maintain patency of the ductus arteriosus. […] Antibiotic prophylaxis is suggested to prevent infective endocarditis, particularly in the repaired tetralogy of Fallot patient with a patch.
  • #1 Tetralogy of Fallot
    https://www.webmd.com/heart-disease/tetralogy-fallot
    Surgery is the primary way to correct the heart problem. Your child may be prescribed medication for tet spells. You will also be given information for dealing with future tet spells. […] The child will be placed on their back in the knee-to-chest position to increase aortic resistance. The increased aortic and left ventricular pressure reduces the rush of blood through the septal hole from the right ventricle and improves blood circulation to the lungs, so more red blood reaches the tissues. […] The Blalock-Taussig operation: A palliative procedure performed in smaller infants to increase blood flow to the lungs. This allows the child to grow big enough to have complete surgical repair. […] Total correction: The hole in the ventricular septum (between the ventricles) is closed with a patch and the obstruction to right ventricular outflow, pulmonic stenosis, is opened. These corrections allow blood flow to the lungs for oxygenation before being pumped out into the body.
  • #1 Tetralogy of Fallot – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/tetralogy-of-fallot/diagnosis-treatment/drc-20353482
    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. […] Some babies or young children are given medicine while waiting for surgery to keep blood flowing from the heart to the lungs. […] Surgery used to treat tetralogy of Fallot may include: […] Temporary surgery, also called temporary repair. Some babies with tetralogy of Fallot need a temporary surgery to improve blood flow to the lungs while waiting for open-heart surgery. This type of treatment is called palliative surgery. A surgeon places a tube called a shunt between a large artery that comes off from the aorta and the lung artery. The tube creates a new path for blood to go to the lungs. This surgery may be done if a baby is born early or if the lung arteries aren’t fully developed. The shunt is removed during open-heart surgery to treat tetralogy of Fallot.
  • #1 Tetralogy of Fallot | American Heart Association
    https://www.heart.org/en/health-topics/congenital-heart-defects/about-congenital-heart-defects/tetralogy-of-fallot
    Tetralogy of Fallot is treated surgically. A temporary procedure may be done at first if the baby is small or if there are other problems. Complete repair comes later. Sometimes the first operation is a complete repair. […] In some infants, a shunt operation may be done first to provide adequate blood flow to the lungs. This isn’t open-heart surgery and doesn’t fix the inside of the heart. The shunt is usually a small tube of synthetic material sewn between a body artery (or the aorta) and the pulmonary artery. The shunt is closed when a complete repair is done later. Alternatively, a metal stent can be placed in the ductus arteriosus to provide extra blood flow to the lungs. […] Complete repair tends to be done early in life. The surgeon closes the ventricular septal defect with a patch and opens the right ventricular outflow tract by removing some thickened muscle below the pulmonary valve, repairing or removing the obstructed pulmonary valve and, if needed, enlarging the branch pulmonary arteries that go to each lung.
  • #1 Transcatheter-Based Interventions for Tetralogy of Fallot: Key Points
    https://www.acc.org/Latest-in-Cardiology/ten-points-to-remember/2024/05/16/20/39/transcatheter-based-interventions
    Transcatheter-Based Interventions for Tetralogy of Fallot: Key Points […] The following are key points to remember from a state-of-the-art review on transcatheter-based interventions for tetralogy of Fallot (TOF) across all age groups: […] Initial treatment strategies for TOF can include palliative transcatheter or surgical interventions as well as primary surgical repairs. […] In pediatric patients, transcatheter interventions can be performed after initial palliative or corrective surgery to treat residual lesions, or as palliation including patent arterial duct (PDA) stenting, right ventricular outflow tract (RVOT) stenting, or ballon pulmonary valvuloplasty. […] Recent data suggest that palliative transcatheter interventions can be considered as an alternative to surgical palliation, particularly in low-birth-weight neonate in infants. […] Stenting of the PDA may be performed in infants with ductal-dependent pulmonary blood flow. […] Pulmonary balloon valvuloplasty may be considered as a palliative procedure when there is obstruction primarily at the valve level, and minimal infundibular obstruction. […] Transcatheter RVOT stenting may be physiologically advantageous as compared with surgical shunts. […] Transcatheter pulmonary valve replacement may be considered for adults with tetralogy of Fallot and moderate or greater pulmonary valve regurgitation or pulmonary stenosis with symptoms or evidence of RV dilatation or dysfunction. […] More recently, The Harmony Transcatheter Pulmonary Valve by Medtronic and the Alterra Adaptive Prestent system received FDA approval in 2021. […] Long-term complications after transcatheter pulmonary valve implantation include stent fracture and endocarditis.
  • #1 Current outcomes and treatment of tetralogy of Fallot
    https://pmc.ncbi.nlm.nih.gov/articles/PMC6719677/
    In general, it is thought that earlier primary repair of ToF can limit prolonged exposure to RV pressure loading and reduced oxygen saturations, preserving cardiovascular and brain function. However, there is no consensus on the definition of early versus later repair. […] Symptomatic ToF patients may require an intervention in the neonatal period. […] The superiority of a staged approach versus primary neonatal repair has not been demonstrated. […] Alternatively, palliative balloon dilation of the pulmonary annulus can be used to increase oxygen saturation and promote growth of the pulmonary vasculature and as bridge to later complete repair in selected patients. […] Similarly, RVOT stenting can be used as a palliative strategy or bridge to repair in neonatal life. […] Overall survival following ToF repair has significantly improved in recent eras.
  • #1 Tetralogy of Fallot – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/tetralogy-of-fallot/diagnosis-treatment/drc-20353482
    Open-heart surgery, called complete repair. People with tetralogy of Fallot need open-heart surgery to completely fix the heart. […] A complete repair is usually done in the first year of life. Rarely, a person may not have surgery in childhood if tetralogy of Fallot goes undiagnosed or if surgery is not available. These adults may still benefit from surgery. […] A complete repair is done in several steps, The surgeon patches the hole between the lower heart chambers and repairs or replaces the pulmonary valve. The surgeon may remove thickened muscle below the pulmonary valve or widen the smaller lung arteries. […] 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.
  • #1 Tetralogy of Fallot (ToF): Symptoms, Causes & Treatment
    https://my.clevelandclinic.org/health/diseases/22343-tetralogy-of-fallot
    To perform a complete repair, a surgeon who specializes in adult congenital heart disease closes the ventricular septal defect with a patch. They open the passageway out of the right ventricle and repair or replace the pulmonary valve. They enlarge the pulmonary arteries to both lungs. Sometimes, they place a tube between the right ventricle and the pulmonary artery to improve blood flow. […] Many adults who undergo repair for a ToF heart defect dont need further surgical treatment. But they should continue to follow up with an adult congenital heart disease specialist for regular monitoring. […] With medical advances, the outlook for babies with tetralogy of Fallot is better than it was in the past. Surgical treatment is very effective in correcting structural defects and blood flow through the heart. With an experienced congenital heart disease surgeon, surgical repair of the defect in adults has a very high success rate. […] After tetralogy of Fallot repair, your child will need regular visits with a provider who specializes in childrens hearts (pediatric cardiologist). Regular care will continue into adulthood.
  • #1 Tetralogy of Fallot (TOF) – Stanford Medicine Children’s Health
    https://www.stanfordchildrens.org/en/topic/default?id=tetralogy-of-fallot-tof-90-P01822
    Treatment will depend on your childs symptoms, age, and general health. It will also depend on how severe the condition is. […] Some children will need a shunt put in for stable pulmonary blood flow until a more permanent repair can be done at a later age. […] All children with TOF need to have surgery to fix it. Most children have it before they turn 1-year-old. Its often done around age 6 months. A team of heart surgeons will do your childs surgery. To fix TOF the doctor may use a patch to close the ventricle septal defect (VSD). Enlarging the right ventricular outflow tract can be done by relieving pulmonary stenosis by removing excessive heart muscle and/or possibly using a patch to enlarge the pulmonary arteries if there is narrowing. […] Fixing the heart defects will allow oxygen-poor blood to travel its normal route. This is through the pulmonary artery to the lungs to pick up oxygen.
  • #1 Tetralogy of Fallot – Child Heart Specialist
    https://www.childheartspecialist.com/london/tetralogy-of-fallot/
    In some children, where the narrowing is mainly due to thick muscle underneath the pulmonary valve, muscle resection in isolation or associated with a patch enlargement might be required. […] In children where the narrowing is mainly across the pulmonary valve, the patch might need to be extended through the valve, trans-annular patch. In some other children, where the main lung artery distal to the valve is narrow or the narrowing extends to the right or the left pulmonary artery, a patch might need to be applied to enlarge those areas. […] In some children the surgeon needs to use a tube, with or without a valve inside, to connect the right sided pumping chamber to the pulmonary artery. This is known as the Rastelli operation. […] In some children complete repair might not be offered as the first procedure for a variety of reasons: the child might be premature, be of small weight or have associated cardiac or extra-cardiac defects. In these children additional procedures, sometimes undertaken urgently, might be required.
  • #1 Tetralogy of Fallot – Wikipedia
    https://en.wikipedia.org/wiki/Tetralogy_of_Fallot
    Tetralogy of fallot is typically treated by open heart surgery in the first year of life. The timing of surgery depends on the baby’s symptoms and size. The procedure involves increasing the size of the pulmonary valve and pulmonary arteries and repairing the ventricular septal defect. In babies who are too small, a temporary surgery may be done with plans for a second surgery when the baby is bigger. With proper care, most people who are affected live to be adults. Long-term problems may include an irregular heart rate and pulmonary regurgitation. […] Total surgical repair of TOF is a curative surgery. Different techniques can be used in performing TOF repair. One method to permit pulmonary blood flow post-birth is the stenting of the ductus arterious (DA) through the inducement of a systemic-to-pulmonary shunt. This surgical approach has an 83% success rate. However, a transatrial, transpulmonary artery approach is used for most cases. The repair consists of two main steps: closure of the VSD with a patch and reconstruction of the right ventricular outflow tract. This open-heart surgery is designed to relieve the right ventricular outflow tract stenosis by careful resection of muscle and to repair the VSD. The right ventricle outflow tract can be reconstructed using mainly 2 procedures: a transannular patch (TAP) or a pulmonary valve-sparing procedure (PVS). The decision on the type of the procedure depends on individual anatomy (especially the size of the pulmonary valve). PVS showed better overall survival, event-free survival and less pulmonary regurgitation at 10, 20 and 30 years after the operation. PVS can be performed with or without ventriculotomy. A study found similar overall and event-free survival and pulmonary regurgitation rate between patients who underwent PVS with ventriculotomy and the ones who did not.
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  • #1 Current outcomes and treatment of tetralogy of Fallot
    https://pmc.ncbi.nlm.nih.gov/articles/PMC6719677/
    Mortality rates at medium-term follow-up have not changed much across the different surgical eras. […] Thirty-five years after ToF repair, PVR will have been performed in about 40% of patients. […] PVR is effective in decreasing RV volumes, reducing TR, decreasing QRS duration, increasing left ventricle (LV) ejection fraction (EF), and improving functional status. […] Current guidelines provide clear indications for re-intervention for residual RVOTO. […] Restoring pulmonary valve function before irreversible RV dysfunction occurs could be important to prevent RV failure. However, the durability of currently used pulmonary prosthetic valves is limited. Therefore, the timing of PVR always is a compromise: It should be timed early enough to prevent irreversible adverse remodeling but late enough to limit the number of re-interventions.
  • #1 Tetralogy of Fallot: Risks and Management in Adulthood – Clinical Advisor
    https://www.clinicaladvisor.com/features/tetralogy-of-fallot-in-adulthood/
    The modified Blalock-Taussig (mBT) shunt is the most commonly used modification and creates an extracardiac connection between the subclavian artery and the ipsilateral pulmonary artery. […] Although long-term outlook is excellent, some patients may need medications (ie antihypertensives, diuretics, and inotropes), heart catheterization, or additional open-heart surgeries. […] Consistent with this study, the most common surgical reintervention is PVR. In patients with severe pulmonary regurgitation, PVR will likely be recommended to prevent progression to right ventricular dilation and dysfunction. […] According to the AHA/ACC guidelines, indications for PVR include ventricular dysfunction, RV dilation, RVOT obstruction, and progressive reduction in exercise tolerance. […] Radiofrequency ablations and automatic implantable cardioverter defibrillators (AICDs) are also common interventions used for patients in the setting of arrhythmias.
  • #1 Tetralogy of Fallot – Seattle Children’s
    https://www.seattlechildrens.org/conditions/tetralogy-of-fallot/
    In the past, some children with tetralogy of Fallot needed open heart surgery later in life to put in a new pulmonary valve. Now, in some cases, we can do this through cardiac catheterization instead. […] We use devices called the Melody, Harmony and Alterra transcatheter heart valves. This means we place a new pulmonary valve through a narrow tube directed into your childs heart. This avoids open heart surgery and leads to quicker recovery.
  • #1 Tetralogy of Fallot | Symptoms, Diagnosis & Treatment
    https://www.cincinnatichildrens.org/health/t/tof
  • #1 Tetralogy of Fallot (TOF) in Adults Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/2035949-treatment
    However, if the patient has no symptoms and has only mild RV dilatation and normal RV function, then no surgery is indicated. […] Most often, surgeons implant a bioprosthetic valve, of which two types are available: human tissue (homografts) or animal tissue (bovine pericardium or porcine heart valve). […] Some patients continue to have ventricular arrhythmias despite surgical repair; they are at greater risk for sudden death. […] Relatively recently, the use of radiofrequency ablation has become another option to treat arrhythmias in adult patients with tetralogy of Fallot. […] The literature suggests that approximately 5% of individuals with tetralogy of Fallot (TOF) who underwent repair in childhood will need a revision/reoperation at some point. […] Once tetralogy of Fallot has been repaired in infancy or childhood, about 5% of individuals require repair or replacement of the pulmonary valve.
  • #1 Tetralogy of Fallot (TOF): Diagnosis & Treatment | NewYork-Presbyterian
    https://www.nyp.org/heart/congenital-heart-disease/tetralogy-of-fallot-tof/treatment
    They can have a less serious surgery for tetralogy of Fallot called a shunt, which provides an alternate pathway to get additional blood to the lungs and raise oxygen levels. Later, the shunt will be removed when the complete repair is done. […] The survival rate for infants with tetralogy of Fallot surgery is estimated at 97%. Most babies who undergo the procedure go on to lead active, healthy lives. […] Tetralogy of Fallot medications can include antibiotics after surgery and heart medications in the future if other issues, such as arrhythmias, arise. […] In some cases, blood flow to the lungs may still be obstructed. In other cases, the valves on the right side of the heart may leak. These issues may require additional surgery. […] Arrhythmias (irregular heart rhythms) can develop after tetralogy of Fallot repair surgery. Medications, an ablation procedure, or a pacemaker may be recommended by your doctor. […] Continuous care into adulthood. Infants diagnosed and treated for tetralogy of Fallot will require life-long attention from a congenital cardiologist for routine testing and heart-health maintenance.
  • #1 Tetralogy of Fallot – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/tetralogy-of-fallot/diagnosis-treatment/drc-20353482
    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.
  • #1 Tetralogy of Fallot With Pulmonary Stenosis Treatment & Management: Approach Considerations, Conservative Therapy, Transcatheter Interventions
    https://emedicine.medscape.com/article/2063480-treatment
    Patients who are status post repair of tetralogy of Fallot deserve lifelong outpatient follow-up with a focus on early detection of signs of congestive heart failure or functional impairment, as well as ongoing surveillance for cardiac arrhythmias. […] Pulmonary valve insertion or replacement can be performed as treatment for pulmonary insufficiency to improve performance status, optimize hemodynamics, and improve control of arrhythmias.
  • #1 Tetralogy of Fallot – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/tetralogy-of-fallot/symptoms-causes/syc-20353477
    In the modern era, repair of tetralogy of Fallot can be performed with very low mortality, in the neighborhood of 1%, and the late survival and quality of life are excellent for the majority of patients. […] The decision to close the patent foramen ovale is largely determined by patient age and whether a transannular repair was applied. […] The application of a monocusp repair to improve competence of the pulmonary valve can be helpful in this situation and can smooth out the early post-operative period.
  • #1 About Tetralogy of Fallot | Congenital Heart Defects (CHDs) | CDC
    https://www.cdc.gov/heart-defects/about/tetralogy-of-fallot.html
    Tetralogy of Fallot can be treated by surgery soon after the baby is born. During surgery, doctors widen or replace the pulmonary valve and enlarge the passage to the pulmonary artery. They also will place a patch over the VSD to close the hole between the two lower chambers of the heart. These actions will improve blood flow to the lungs and the rest of the body. […] Most infants will live active, healthy lives after surgery. However, they will need routine checkups with a heart doctor to monitor their progress. Providers will check for other health conditions that might develop as they get older. As adults, they may need more surgery or medical care for other possible problems.
  • #1 Treatment of Tetralogy of Fallot (ToF) | Best Hospitals | Clinics | Prices | Booking Health
    https://bookinghealth.com/disease/tetralogy-of-fallot-tof
    The advantage of the palliative intervention is that it gives the opportunity to make the radical correction later. The child’s heart changes after a few months, and the surgery can be performed without the transannular patch of the right ventricular outflow tract. This contributes to the improved survival rates after radical correction. […] In recent years, the results of surgical correction of tetralogy of Fallot have improved significantly. Operative mortality is on average 3%, and in the best hospitals in the world this indicator does not exceed 1%. The prognosis depends not only on the quality of treatment, but also on the condition of the child at the time of the surgical intervention, on the severity of the defect, which is determined by the following aspects: […] Thirty-year survival rates with tetralogy of Fallot in the twentieth century were only 68.5%, and now they have increased to 90.5%. The treatment in the best hospitals in the world provides excellent long-term results, even if patients have to undergo plastic surgery of the right ventricular outflow tract.
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    https://link.springer.com/article/10.1007/s00246-020-02297-z
    The aim of this procedure is to increase the pulmonary blood flow but leaving some stenosis to avoid systemic pressure to be transmitted to the pulmonary bed. […] As mentioned previously, there is no report of a complete transcatheter tetralogy of Fallot repair so far. […] Theoretically both anomalies are can be treated by interventions. Device closure of muscular ventricular septal defects and selected cases of peri-membranous ventricular septal defects have already been established as a mode of treatment. […] Surgical repair of tetralogy of Fallot provides excellent results in the current era. With the recent advances in interventional cardiology in simple cardiac anomalies, the question of complete repair of more complex anomalies like tetralogy of Fallot has been raised. […] With the help of 3D modeling and the development of new materials such as bioresorbable devices, we are convinced that, in coming two decades, many advances will be made to interventionally approach more complex congenital heart disease like the tetralogy of Fallot.
  • #1 Tetralogy of Fallot > Fact Sheets > Yale Medicine
    https://www.yalemedicine.org/conditions/tetralogy-of-fallot
    Treatment includes surgery, medication […] Surgical intervention is used to correct tetralogy of Fallot, but it is usually performed between 2 and 6 months of life. Younger infants may need medication to manage their symptoms until they are old enough for surgery. […] Doctors may prescribe prostaglandins in certain newborns with symptoms of tetralogy of Fallot and severe cyanosis. This medication prevents the ductus arteriosus a special blood vessel that connects the pulmonary artery to the aorta in a fetus from closing during the first week of life. […] Patients who experience tet spells need to be treated quickly. […] When a patient is old enough for surgical repair, doctors perform open-heart surgery. […] Well over 90% of patients who have surgical intervention to correct the condition live into adulthood as healthy, active people.
  • #2 Current outcomes and treatment of tetralogy of… | F1000Research
    https://f1000research.com/articles/8-1530
    Tetralogy of Fallot (ToF) is the most common type of cyanotic congenital heart disease. Since the first surgical repair in 1954, treatment has continuously improved. The treatment strategies currently used in the treatment of ToF result in excellent long-term survival (30 year survival ranges from 68.5% to 90.5%). […] Performing pulmonary valve replacement or relief of pulmonary stenosis before irreversible right ventricular dysfunction occurs is important, but determining the optimal timing of pulmonary valve replacement is challenging for several reasons. […] Different surgical techniques were developed minimizing the extent of the ventriculotomy and trying to preserve competence of the pulmonary valve without causing significant residual RVOTO. […] In general, it is thought that earlier primary repair of ToF can limit prolonged exposure to RV pressure loading and reduced oxygen saturations, preserving cardiovascular and brain function.
  • #2 Tetralogy of fallot medical treatment – wikidoc
    https://www.wikidoc.org/index.php/Tetralogy_of_fallot_medical_treatment
    Although operative repair of tetralogy of Fallot is definitive, medical therapy can be used to manage hypoxic or tet spells. Tet spells cause acute hypoxia and may be treated with beta blockers, morphine, phenylephrine, and oxygen. […] Tet spells cause acute hypoxia and may be treated with: Beta-blockers such as propranolol or esmolol. The beta-blockers causes relaxation of the right ventricular outflow tract and increases blood flow into the pulmonary vessels. Morphine to reduce ventilatory drive. Phenylephrine to increase systemic afterload that in turn increases the flow across right ventricle and the pulmonary artery and decreases right to left shunting. Procedures such as the knee-chest position which increases aortic wave reflection, increasing pressure on the left side of the heart, decreasing the right-to-left shunt thus decreasing the amount of deoxygenated blood entering the systemic circulation. Oxygen is ineffective in treating hypoxic spells as the underlying problem is lack of blood flow through the pulmonary circuit and not alveolar oxygenation. In case all these measures fail, an emergency Blalock-Taussig shunt might be needed. […] Prostaglandins can be administered while awaiting surgery to maintain patency of the ductus arteriosus. […] Antibiotic prophylaxis is suggested to prevent infective endocarditis, particularly in the repaired tetralogy of Fallot patient with a patch.
  • #2 Tetralogy of Fallot > Fact Sheets > Yale Medicine
    https://www.yalemedicine.org/conditions/tetralogy-of-fallot
    Treatment includes surgery, medication […] Surgical intervention is used to correct tetralogy of Fallot, but it is usually performed between 2 and 6 months of life. Younger infants may need medication to manage their symptoms until they are old enough for surgery. […] Doctors may prescribe prostaglandins in certain newborns with symptoms of tetralogy of Fallot and severe cyanosis. This medication prevents the ductus arteriosus a special blood vessel that connects the pulmonary artery to the aorta in a fetus from closing during the first week of life. […] Patients who experience tet spells need to be treated quickly. […] When a patient is old enough for surgical repair, doctors perform open-heart surgery. […] Well over 90% of patients who have surgical intervention to correct the condition live into adulthood as healthy, active people.
  • #2 Tetralogy of Fallot – Pediatrics – Merck Manual Professional Edition
    https://www.merckmanuals.com/professional/pediatrics/congenital-cardiovascular-anomalies/tetralogy-of-fallot
    Ultimately, if positioning and medications do not relieve the spell or if the infant is rapidly deteriorating, tracheal intubation with muscle paralysis and general anesthesia, extracorporeal membrane oxygenation (ECMO), or urgent surgical intervention may be necessary. […] Complete repair of tetralogy of Fallot consists of patch closure of the ventricular septal defect, widening of the right ventricular outflow tract with muscle resection, pulmonic valvuloplasty and, when warranted, patch augmentation of the main pulmonary artery. […] Surgery is usually done electively at age 2 to 6 months but can be done at any time if symptoms are present or right ventricular outflow tract obstruction is severe. […] Propranolol 0.25 to 1 mg/kg orally every 6 hours (given until surgical repair) may prevent recurrences. Most experts think that even one significant spell indicates the need for expeditious surgical repair.
  • #2 Nursing Basics for Tetralogy of Fallot – Straight A Nursing
    https://straightanursingstudent.com/tetralogy-of-fallot/
    Treatment is aimed at responding appropriately to tet spells, preventing infection, managing heart failure if present, and surgical correction of the anomaly. […] If the child is having a “tet” spell or hypercyanotic episode, the general guidelines are to: […] Draw the child’s knees up to their chest to increase systemic vascular resistance. This increases the amount of blood flow out of the right ventricle and into the pulmonary vasculature. […] Provide oxygen. […] The MD may order additional therapies, including an IV narcotic and fluid bolus of 10-20 ml/kg. The mechanism of how narcotics help is unclear, but the fluids help improve preload and pulmonary blood flow. […] A beta blocker may be used to decrease right-ventricular outflow tract obstruction, thereby increasing pulmonary blood flow.
  • #2 Tetralogy of Fallot (ToF): Symptoms, Causes & Treatment
    https://my.clevelandclinic.org/health/diseases/22343-tetralogy-of-fallot
    Tetralogy of Fallot treatment includes medicine to ease symptoms and surgery to fix the issues. As a first step in tetralogy of Fallot treatment, your childs provider can give them beta-blockers (to improve blood flow) and extra oxygen. Three to six months after birth, your baby can have surgery to make blood move through their heart the way it should. […] Repair options vary depending on your childs condition. If your baby is too small or weak for a full repair, their provider can do a simpler procedure until they can do the complete ToF repair. These techniques can relieve symptoms for a number of years well into adulthood until a provider can do a more complete repair surgery. […] Occasionally, a person reaches adulthood without having had any surgical repair. This isnt common. Specialists recommend they have a complete surgical repair to prevent future complications or sudden death.
  • #2 Tetralogy of Fallot | American Heart Association
    https://www.heart.org/en/health-topics/congenital-heart-defects/about-congenital-heart-defects/tetralogy-of-fallot
    Tetralogy of Fallot is treated surgically. A temporary procedure may be done at first if the baby is small or if there are other problems. Complete repair comes later. Sometimes the first operation is a complete repair. […] In some infants, a shunt operation may be done first to provide adequate blood flow to the lungs. This isn’t open-heart surgery and doesn’t fix the inside of the heart. The shunt is usually a small tube of synthetic material sewn between a body artery (or the aorta) and the pulmonary artery. The shunt is closed when a complete repair is done later. Alternatively, a metal stent can be placed in the ductus arteriosus to provide extra blood flow to the lungs. […] Complete repair tends to be done early in life. The surgeon closes the ventricular septal defect with a patch and opens the right ventricular outflow tract by removing some thickened muscle below the pulmonary valve, repairing or removing the obstructed pulmonary valve and, if needed, enlarging the branch pulmonary arteries that go to each lung.
  • #2 Tetralogy of Fallot: Risks and Management in Adulthood – Clinical Advisor
    https://www.clinicaladvisor.com/features/tetralogy-of-fallot-in-adulthood/
    The modified Blalock-Taussig (mBT) shunt is the most commonly used modification and creates an extracardiac connection between the subclavian artery and the ipsilateral pulmonary artery. […] Although long-term outlook is excellent, some patients may need medications (ie antihypertensives, diuretics, and inotropes), heart catheterization, or additional open-heart surgeries. […] Consistent with this study, the most common surgical reintervention is PVR. In patients with severe pulmonary regurgitation, PVR will likely be recommended to prevent progression to right ventricular dilation and dysfunction. […] According to the AHA/ACC guidelines, indications for PVR include ventricular dysfunction, RV dilation, RVOT obstruction, and progressive reduction in exercise tolerance. […] Radiofrequency ablations and automatic implantable cardioverter defibrillators (AICDs) are also common interventions used for patients in the setting of arrhythmias.
  • #2 Tetralogy of Fallot | Condition Overview | Columbia Surgery
    https://columbiasurgery.org/conditions-and-treatments/tetralogy-of-fallot
    Patent ductus arteriosus (PDA) stent: If the heart cannot deliver blood to the lungs due to a defect in the pulmonary valve, it may be necessary to add a stent, an expandable mesh tube, to open a blood vessel called the patent ductus arteriosus, or PDA. The PDA helps deliver blood during development but closes after birth. By reopening the PDA, some blood flow is restored to the lungs. The defect is then fixed during intracardiac surgical repair at a later time.
  • #2 Tetralogy of Fallot Will be Treated Interventionally Within Two Decades
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7170827/
    Palliative procedures are aimed to improve oxygen saturations, so that the definitive procedures can be performed at a later stage with lesser risk. […] Over the last decade and with the advance of non-invasive imaging (i.e., echocardiography, computed tomography angiography or magnetic resonance imaging), the role of cardiac catheterization in most congenital heart diseases and in tetralogy of Fallot in particular has shifted from diagnostic to interventional. […] A large spectrum of interventions can be performed in tetralogy of Fallot patients. […] After the complete surgical repair, cardiac catheterization can be done to treat residual lesions or long-term complications such as pulmonary artery stenosis (balloon angioplasty or stent implantation), residual or additional ventricular septal defect (closure using devices), or pulmonary valve regurgitation (percutaneous pulmonary valve implantation).
  • #2 Current outcomes and treatment of tetralogy of Fallot
    https://pmc.ncbi.nlm.nih.gov/articles/PMC6719677/
    In general, it is thought that earlier primary repair of ToF can limit prolonged exposure to RV pressure loading and reduced oxygen saturations, preserving cardiovascular and brain function. However, there is no consensus on the definition of early versus later repair. […] Symptomatic ToF patients may require an intervention in the neonatal period. […] The superiority of a staged approach versus primary neonatal repair has not been demonstrated. […] Alternatively, palliative balloon dilation of the pulmonary annulus can be used to increase oxygen saturation and promote growth of the pulmonary vasculature and as bridge to later complete repair in selected patients. […] Similarly, RVOT stenting can be used as a palliative strategy or bridge to repair in neonatal life. […] Overall survival following ToF repair has significantly improved in recent eras.
  • #2 Tetralogy of Fallot – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/tetralogy-of-fallot/symptoms-causes/syc-20353477
    People who are diagnosed with tetralogy of Fallot need surgery to fix the heart. They will need regular health checkups for life. […] The optimum treatment approach remains controversial, but in general, complete repair is advised in the first three to six months of life. […] The surgical goal is complete repair, which consists of ventricular septal defect closure and relief of right ventricular outflow tract obstruction, which is ideally performed with preservation of pulmonary valve function. […] In general, children attend school and can participate in most childhood sport activities without restrictions. Early repair in the first six months of life is the rule, and preservation of the pulmonary valve and minimizing pulmonary regurgitation is the goal. […] The presence of an anomalous left anterior descending coronary artery is usually not a contraindication to complete repair in the current era.
  • #2 Tetralogy of Fallot With Pulmonary Stenosis Treatment & Management: Approach Considerations, Conservative Therapy, Transcatheter Interventions
    https://emedicine.medscape.com/article/2063480-treatment
    The role of palliative surgery for tetralogy of Fallot (TOF) is controversial. Patients whose conditions are refractory to medical management and stabilization require urgent surgical intervention. […] Complete intracardiac repair of tetralogy of Fallot (TOF) can be performed as a single-stage procedure or as a 2-stage approach, with initial systemic-topulmonary artery shunting. […] Complete surgical repair involves closure of the ventricular septal defect (VSD) and relief of the right ventricular (RV) outflow tract obstruction (RVOTO). […] The goals of complete repair are relief of all obstruction to blood flow from the RV to the pulmonary artery and closure of the VSD. […] After surgery, various residual abnormalities may be encountered, ranging from a nearly normal-appearing heart to one in which substantial right ventricular (RV) dysfunction and residual RV outflow tract obstruction (RVOTO).
  • #2 Tetralogy of Fallot – BHF
    https://www.bhf.org.uk/informationsupport/conditions/tetralogy-of-fallot
    Surgery is the only way to treat tetralogy of Fallot. This is usually done in childhood. You may need or have had more than one operation to repair the problems with your heart. If youre worried, or have questions about what procedures youve had, speak to your healthcare team. […] A shunt operation (or temporary operation) is one of the first procedures you may have had if you were born with tetralogy of Fallot. Its a surgery that improves the blood flow to your lungs while you wait for complete repair surgery. Its not always needed, but if it is, its done within the first four to six months of your life. […] Open heart surgery is needed to repair tetralogy of Fallot. The surgery is often done within the first few years of your life. Complete repair surgery usually includes: a patch being sewn over the hole in your heart, the widening of your narrowed pulmonary valve and artery, part of your thickened pulmonary valve may also be removed.
  • #2 Tetralogy of Fallot | Symptoms and Treatment | Dayton, OH
    http://fetaltonewborn.org/tetralogy-fallot/
    Babies diagnosed with TOF will require open-heart surgery to repair all four abnormalities so that the heart can work as normally as possible. The surgery is usually done soon after birth or while your baby is very young. Sometimes more than one surgery is needed. Babies who have continued, severe leakiness of the pulmonary valve may need to have doctors replace the valve. […] Most cases of TOF can be corrected with surgery, although your baby will need regular follow-up visits with a cardiologist. More than 90 percent of babies who have surgery for TOF go on to live healthy lives.
  • #2 Tetralogy of Fallot (TOF) | Causes, symptoms, & treatment | Children’s Wisconsin
    https://childrenswi.org/medical-care/herma-heart/conditions/tetralogy-of-fallot
    Tetralogy of Fallot is very treatable, most often requiring surgery before a child’s first birthday. […] Expert diagnosis is necessary to determine how your child’s heart is affected so that treatment can be customized based on your child’s unique needs. […] Tetralogy of Fallot is treated by surgical repair of the defects. A team of cardiac surgeons performs the surgery, usually before an infant is 1 year old. In many cases, the repair is made at around 6 months of age, or even a little earlier. Repairing the heart defects will allow oxygen-poor (blue) blood to travel its normal route through the pulmonary artery to receive oxygen. […] The operation is performed under general anesthesia, and involves the following: The ventricular septal defect is closed with a patch. The obstructed pathway between the right ventricle and the pulmonary artery is opened and enlarged with a patch. If the pulmonary valve is small, it may be opened as well. […] Most children who have had a tetralogy of Fallot surgical repair will live healthy lives. Activity levels, appetite, and growth will eventually return to normal in most children.
  • #2 Tetralogy of Fallot Repair | Conditions & Treatments | UT Southwestern Medical Center
    https://utswmed.org/conditions-treatments/tetralogy-of-fallot-repair/
    UT Southwestern Medical Center, one of the top cardiothoracic surgery centers in the U.S., offers advanced open-heart surgery to repair the cardiac abnormalities associated with tetralogy of Fallot (TOF). […] Open-heart surgery is required to repair tetralogy of Fallot (TOF), a complex congenital heart condition. Depending upon the precise nature of the condition, surgery to repair it can include ventricular septal defect (VSD) closure, removal of muscle from the right ventricle, repair of the pulmonic valve, or pulmonary artery enlargement. […] Our heart surgeons can perform surgery in small infants to temporarily manage the heart defects, with complete surgical repair to follow when the baby is older. […] We take a collaborative approach, combining cardiologists, interventional cardiologists, and surgeons to develop the best treatment plan for each patient.
  • #2 Tetralogy of Fallot – Wikipedia
    https://en.wikipedia.org/wiki/Tetralogy_of_Fallot
    Tetralogy of fallot is typically treated by open heart surgery in the first year of life. The timing of surgery depends on the baby’s symptoms and size. The procedure involves increasing the size of the pulmonary valve and pulmonary arteries and repairing the ventricular septal defect. In babies who are too small, a temporary surgery may be done with plans for a second surgery when the baby is bigger. With proper care, most people who are affected live to be adults. Long-term problems may include an irregular heart rate and pulmonary regurgitation. […] Total surgical repair of TOF is a curative surgery. Different techniques can be used in performing TOF repair. One method to permit pulmonary blood flow post-birth is the stenting of the ductus arterious (DA) through the inducement of a systemic-to-pulmonary shunt. This surgical approach has an 83% success rate. However, a transatrial, transpulmonary artery approach is used for most cases. The repair consists of two main steps: closure of the VSD with a patch and reconstruction of the right ventricular outflow tract. This open-heart surgery is designed to relieve the right ventricular outflow tract stenosis by careful resection of muscle and to repair the VSD. The right ventricle outflow tract can be reconstructed using mainly 2 procedures: a transannular patch (TAP) or a pulmonary valve-sparing procedure (PVS). The decision on the type of the procedure depends on individual anatomy (especially the size of the pulmonary valve). PVS showed better overall survival, event-free survival and less pulmonary regurgitation at 10, 20 and 30 years after the operation. PVS can be performed with or without ventriculotomy. A study found similar overall and event-free survival and pulmonary regurgitation rate between patients who underwent PVS with ventriculotomy and the ones who did not.
  • #2 Tetralogy of Fallot – Child Heart Specialist
    https://www.childheartspecialist.com/london/tetralogy-of-fallot/
    In some children, where the narrowing is mainly due to thick muscle underneath the pulmonary valve, muscle resection in isolation or associated with a patch enlargement might be required. […] In children where the narrowing is mainly across the pulmonary valve, the patch might need to be extended through the valve, trans-annular patch. In some other children, where the main lung artery distal to the valve is narrow or the narrowing extends to the right or the left pulmonary artery, a patch might need to be applied to enlarge those areas. […] In some children the surgeon needs to use a tube, with or without a valve inside, to connect the right sided pumping chamber to the pulmonary artery. This is known as the Rastelli operation. […] In some children complete repair might not be offered as the first procedure for a variety of reasons: the child might be premature, be of small weight or have associated cardiac or extra-cardiac defects. In these children additional procedures, sometimes undertaken urgently, might be required.
  • #2 Tetralogy of Fallot With Pulmonary Stenosis Treatment & Management: Approach Considerations, Conservative Therapy, Transcatheter Interventions
    https://emedicine.medscape.com/article/2063480-treatment
    Patients who are status post repair of tetralogy of Fallot deserve lifelong outpatient follow-up with a focus on early detection of signs of congestive heart failure or functional impairment, as well as ongoing surveillance for cardiac arrhythmias. […] Pulmonary valve insertion or replacement can be performed as treatment for pulmonary insufficiency to improve performance status, optimize hemodynamics, and improve control of arrhythmias.
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  • #2 Current outcomes and treatment of tetralogy of Fallot
    https://pmc.ncbi.nlm.nih.gov/articles/PMC6719677/
    Mortality rates at medium-term follow-up have not changed much across the different surgical eras. […] Thirty-five years after ToF repair, PVR will have been performed in about 40% of patients. […] PVR is effective in decreasing RV volumes, reducing TR, decreasing QRS duration, increasing left ventricle (LV) ejection fraction (EF), and improving functional status. […] Current guidelines provide clear indications for re-intervention for residual RVOTO. […] Restoring pulmonary valve function before irreversible RV dysfunction occurs could be important to prevent RV failure. However, the durability of currently used pulmonary prosthetic valves is limited. Therefore, the timing of PVR always is a compromise: It should be timed early enough to prevent irreversible adverse remodeling but late enough to limit the number of re-interventions.
  • #2 Transcatheter-Based Interventions for Tetralogy of Fallot: Key Points
    https://www.acc.org/Latest-in-Cardiology/ten-points-to-remember/2024/05/16/20/39/transcatheter-based-interventions
    Transcatheter-Based Interventions for Tetralogy of Fallot: Key Points […] The following are key points to remember from a state-of-the-art review on transcatheter-based interventions for tetralogy of Fallot (TOF) across all age groups: […] Initial treatment strategies for TOF can include palliative transcatheter or surgical interventions as well as primary surgical repairs. […] In pediatric patients, transcatheter interventions can be performed after initial palliative or corrective surgery to treat residual lesions, or as palliation including patent arterial duct (PDA) stenting, right ventricular outflow tract (RVOT) stenting, or ballon pulmonary valvuloplasty. […] Recent data suggest that palliative transcatheter interventions can be considered as an alternative to surgical palliation, particularly in low-birth-weight neonate in infants. […] Stenting of the PDA may be performed in infants with ductal-dependent pulmonary blood flow. […] Pulmonary balloon valvuloplasty may be considered as a palliative procedure when there is obstruction primarily at the valve level, and minimal infundibular obstruction. […] Transcatheter RVOT stenting may be physiologically advantageous as compared with surgical shunts. […] Transcatheter pulmonary valve replacement may be considered for adults with tetralogy of Fallot and moderate or greater pulmonary valve regurgitation or pulmonary stenosis with symptoms or evidence of RV dilatation or dysfunction. […] More recently, The Harmony Transcatheter Pulmonary Valve by Medtronic and the Alterra Adaptive Prestent system received FDA approval in 2021. […] Long-term complications after transcatheter pulmonary valve implantation include stent fracture and endocarditis.
  • #2 Tetralogy of Fallot (TOF) in Adults Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/2035949-treatment
    However, if the patient has no symptoms and has only mild RV dilatation and normal RV function, then no surgery is indicated. […] Most often, surgeons implant a bioprosthetic valve, of which two types are available: human tissue (homografts) or animal tissue (bovine pericardium or porcine heart valve). […] Some patients continue to have ventricular arrhythmias despite surgical repair; they are at greater risk for sudden death. […] Relatively recently, the use of radiofrequency ablation has become another option to treat arrhythmias in adult patients with tetralogy of Fallot. […] The literature suggests that approximately 5% of individuals with tetralogy of Fallot (TOF) who underwent repair in childhood will need a revision/reoperation at some point. […] Once tetralogy of Fallot has been repaired in infancy or childhood, about 5% of individuals require repair or replacement of the pulmonary valve.
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  • #2 Tetralogy of Fallot (ToF): Symptoms, Causes & Treatment
    https://my.clevelandclinic.org/health/diseases/22343-tetralogy-of-fallot
    To perform a complete repair, a surgeon who specializes in adult congenital heart disease closes the ventricular septal defect with a patch. They open the passageway out of the right ventricle and repair or replace the pulmonary valve. They enlarge the pulmonary arteries to both lungs. Sometimes, they place a tube between the right ventricle and the pulmonary artery to improve blood flow. […] Many adults who undergo repair for a ToF heart defect dont need further surgical treatment. But they should continue to follow up with an adult congenital heart disease specialist for regular monitoring. […] With medical advances, the outlook for babies with tetralogy of Fallot is better than it was in the past. Surgical treatment is very effective in correcting structural defects and blood flow through the heart. With an experienced congenital heart disease surgeon, surgical repair of the defect in adults has a very high success rate. […] After tetralogy of Fallot repair, your child will need regular visits with a provider who specializes in childrens hearts (pediatric cardiologist). Regular care will continue into adulthood.
  • #2 Tetralogy of Fallot (TOF) | Children’s Hospital of Philadelphia
    https://www.chop.edu/conditions-diseases/tetralogy-fallot
    After surgery, your child will initially recover in the Evelyn and Daniel M. Tabas Cardiac Intensive Care Unit (CICU), where they will receive around-the-clock attention from a team of dedicated cardiac critical care medicine specialists and specialty trained nurses and support staff. As your childs condition improves, they will be transferred to the Cardiac Care Unit and then discharged home. […] Children who have had surgical repair of tetralogy of Fallot will require lifelong care by a cardiologist. At CHOP, our pediatric cardiologists follow patients until they are young adults, coordinating care with the childs primary care physician. Patients will need to carefully follow their doctors’ advice, including continuing any prescribed medications and, in some cases, limiting exercise. […] Adults born with tetralogy of Fallot must continue to see a cardiologist throughout their lives. CHOPs Cardiac Center can help with the transition to an adult cardiologist.
  • #2 Tetralogy of Fallot Repair | Conditions & Treatments | UT Southwestern Medical Center
    https://utswmed.org/conditions-treatments/tetralogy-of-fallot-repair/
    Our surgeons and cardiologists work closely together in hybrid operative procedures, using a combination of surgery and minimally invasive catheter procedures. The team focuses on preserving the patients pulmonary valve and performing the procedure through the atrium and pulmonary artery, rather than open surgery that would require an incision into heart muscle. […] UT Southwestern is also home to a dedicated Cardiac Rehabilitation Program. Cardiac rehabilitation is a critical component of recovery and can prevent future heart disease. […] The surgeon provides specific instructions to the patients parents prior to the tetralogy of Fallot repair procedure, also discussing risks such as bleeding, infection, or adverse reaction to anesthesia. […] Before the surgery begins, a cardiologist starts a transesophageal echocardiogram (TEE) so the surgeon can look at the heart structure during surgery.
  • #2 Treatment of Tetralogy of Fallot (ToF) | Best Hospitals | Clinics | Prices | Booking Health
    https://bookinghealth.com/disease/tetralogy-of-fallot-tof
    The advantage of the palliative intervention is that it gives the opportunity to make the radical correction later. The child’s heart changes after a few months, and the surgery can be performed without the transannular patch of the right ventricular outflow tract. This contributes to the improved survival rates after radical correction. […] In recent years, the results of surgical correction of tetralogy of Fallot have improved significantly. Operative mortality is on average 3%, and in the best hospitals in the world this indicator does not exceed 1%. The prognosis depends not only on the quality of treatment, but also on the condition of the child at the time of the surgical intervention, on the severity of the defect, which is determined by the following aspects: […] Thirty-year survival rates with tetralogy of Fallot in the twentieth century were only 68.5%, and now they have increased to 90.5%. The treatment in the best hospitals in the world provides excellent long-term results, even if patients have to undergo plastic surgery of the right ventricular outflow tract.
  • #2 Tetralogy of Fallot Will be Treated Interventionally Within Two Decades
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7170827/
    In native tetralogy of Fallot, cardiac interventions can also be performed mimicking what is done in surgery but no transcatheter complete repair has been reported so far. […] Palliative approaches aiming to improve oxygenation and pulmonary blood flow are performed on regular basis. […] The aim of this procedure is to increase the pulmonary blood flow but leaving some stenosis to avoid systemic pressure to be transmitted to the pulmonary bed. […] Considering a full transcatheter repair, tetralogy of Fallot should also be seen as a monology. […] By shifting the septum, the right ventricular outflow tract obstruction would be relieved. […] However, because standing in the right ventricular outflow tract, that kind of device would be under very high stress, prone to fracture and might be the substrate for arrhythmias. […] With the recent advances in interventional cardiology in simple cardiac anomalies, the question of complete repair of more complex anomalies like tetralogy of Fallot has been raised.
  • #2 Tetralogy of Fallot – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/tetralogy-of-fallot/symptoms-causes/syc-20353477
    In the modern era, repair of tetralogy of Fallot can be performed with very low mortality, in the neighborhood of 1%, and the late survival and quality of life are excellent for the majority of patients. […] The decision to close the patent foramen ovale is largely determined by patient age and whether a transannular repair was applied. […] The application of a monocusp repair to improve competence of the pulmonary valve can be helpful in this situation and can smooth out the early post-operative period.