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Tetralogia de Fallot
ORPHA:3303CID-10 · Q21.3CID-11 · LA88.2OMIM 187500DOENÇA RARA

A Tetralogia de Fallot é uma condição cardíaca congênita (presente desde o nascimento) que se caracteriza por quatro problemas no coração: um buraco na parede que separa os dois ventrículos (as câmaras de baixo do coração), um estreitamento na saída do ventrículo direito (a câmara que bombeia o sangue para os pulmões), a artéria aorta (o principal vaso que leva sangue para o corpo) está mal posicionada, nascendo por cima do buraco entre os ventrículos, e um aumento do músculo do ventrículo direito.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

A Tetralogia de Fallot é uma condição cardíaca congênita (presente desde o nascimento) que se caracteriza por quatro problemas no coração: um buraco na parede que separa os dois ventrículos (as câmaras de baixo do coração), um estreitamento na saída do ventrículo direito (a câmara que bombeia o sangue para os pulmões), a artéria aorta (o principal vaso que leva sangue para o corpo) está mal posicionada, nascendo por cima do buraco entre os ventrículos, e um aumento do músculo do ventrículo direito.

Pesquisas ativas
20 ensaios
97 total registrados no ClinicalTrials.gov
Publicações científicas
11.798 artigos
Último publicado: 2026 Apr 17

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Início
Antenatal
+ neonatal
🏥
SUS: Cobertura mínimaScore: 15%
CID-10: Q21.3
🇧🇷Dados SUS / DATASUS
PROCEDIMENTOS SIGTAP (5)
0202010503
Cariótipo — bandas G, Q ou Rgenetic_test
0202010600
Pesquisa de microdeleções/microduplicações por FISHlab_test
0202010694
Sequenciamento completo do exoma (WES)rehabilitation
0202010260
Dosagem de alfa-fetoproteína
0301070040
Atendimento em reabilitação — doenças raras
Você se identifica com essa condição?
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

😀
Face
2 sintomas
🦴
Ossos e articulações
2 sintomas
📏
Crescimento
2 sintomas
👁️
Olhos
1 sintomas

+ 6 sintomas em outras categorias

Características mais comuns

90%prev.
Testa larga
Muito frequente (99-80%)
90%prev.
Morfologia nasal anormal
Muito frequente (99-80%)
90%prev.
Braquidactilia
Muito frequente (99-80%)
90%prev.
Retardo do crescimento intrauterino
Muito frequente (99-80%)
90%prev.
Clinodactilia do quinto dedo
Muito frequente (99-80%)
55%prev.
Dolicocefalia
Frequente (79-30%)
13sintomas
Muito frequente (5)
Frequente (7)
Sem dados (1)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 13 características clínicas mais associadas, ordenadas por frequência.

Testa largaBroad forehead
Muito frequente (99-80%)90%
Morfologia nasal anormalAbnormal nasal morphology
Muito frequente (99-80%)90%
BraquidactiliaBrachydactyly
Muito frequente (99-80%)90%
Retardo do crescimento intrauterinoIntrauterine growth retardation
Muito frequente (99-80%)90%
Clinodactilia do quinto dedoClinodactyly of the 5th finger
Muito frequente (99-80%)90%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico11.798PubMed
Últimos 10 anos200publicações
Pico2026117 papers
Linha do tempo
2026Hoje · 2026🧪 1986Primeiro ensaio clínico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

Genes associados

13 genes identificados com associação a esta condição. Padrão de herança: Autosomal dominant, Multigenic/multifactorial.

GATA6Transcription factor GATA-6Major susceptibility factor inAltamente restrito
FUNÇÃO

Transcriptional activator (PubMed:19666519, PubMed:22750565, PubMed:22824924, PubMed:27756709). Regulates SEMA3C and PLXNA2 (PubMed:19666519). Involved in gene regulation specifically in the gastric epithelium (PubMed:9315713). May regulate genes that protect epithelial cells from bacterial infection (PubMed:16968778). Involved in bone morphogenetic protein (BMP)-mediated cardiac-specific gene expression (By similarity). Binds to BMP response element (BMPRE) DNA sequences within cardiac activati

LOCALIZAÇÃO

Nucleus

VIAS BIOLÓGICAS (5)
Formation of definitive endodermCardiogenesisDevelopmental Lineage of Multipotent Pancreatic Progenitor CellsSurfactant metabolismFactors involved in megakaryocyte development and platelet production
EXPRESSÃO TECIDUAL(Ubíquo)
Ovário
121.3 TPM
Glândula adrenal
65.6 TPM
Fallopian Tube
61.2 TPM
Aorta
54.6 TPM
Artéria coronária
51.1 TPM
OUTRAS DOENÇAS (11)
conotruncal heart malformationstetralogy of fallotpancreatic hypoplasia-diabetes-congenital heart disease syndromeatrial septal defect 9
HGNC:4174UniProt:Q92908
NKX2-6Homeobox protein Nkx-2.6Major susceptibility factor inTolerante
FUNÇÃO

Acts as a transcriptional activator (PubMed:15649947). In conjunction with NKX2-5, may play a role in both pharyngeal and cardiac embryonic development

LOCALIZAÇÃO

Nucleus

VIAS BIOLÓGICAS (2)
Regulation of gene expression in endocrine-committed (NEUROG3+) progenitor cellsRegulation of gene expression in beta cells
MECANISMO DE DOENÇA

Conotruncal heart malformations

A group of congenital heart defects involving the outflow tracts. Examples include truncus arteriosus communis, double-outlet right ventricle and transposition of great arteries. Truncus arteriosus communis is characterized by a single outflow tract instead of a separate aorta and pulmonary artery. In transposition of the great arteries, the aorta arises from the right ventricle and the pulmonary artery from the left ventricle. In double outlet of the right ventricle, both the pulmonary artery and aorta arise from the right ventricle.

EXPRESSÃO TECIDUAL(Baixa expressão)
Glândula salivar
1.4 TPM
Coração - Átrio
0.7 TPM
Testículo
0.2 TPM
Coração - Ventrículo esquerdo
0.0 TPM
INTERAÇÕES PROTEICAS (1)
OUTRAS DOENÇAS (4)
conotruncal heart malformationsfamilial atrial fibrillationtetralogy of fallotpersistent truncus arteriosus
HGNC:32940UniProt:A6NCS4
GATA5Transcription factor GATA-5Disease-causing germline mutation(s) (loss of function) inTolerante
FUNÇÃO

Transcription factor required during cardiovascular development (PubMed:23289003). Plays an important role in the transcriptional program(s) that underlies smooth muscle cell diversity (By similarity). Binds to the functionally important CEF-1 nuclear protein binding site in the cardiac-specific slow/cardiac troponin C transcriptional enhancer (PubMed:25543888)

LOCALIZAÇÃO

Nucleus

VIAS BIOLÓGICAS (1)
Factors involved in megakaryocyte development and platelet production
MECANISMO DE DOENÇA

Congenital heart defects, multiple types, 5

A disorder characterized by congenital developmental abnormalities involving structures of the heart. Common defects include transposition of the great arteries, aortic stenosis, atrial septal defect, ventricular septal defect, pulmonic stenosis, patent ductus arteriosus, and tetralogy of Fallot. Some patients also have cardiac arrhythmias, which may be due to the anatomic defect itself or to surgical interventions. CHTD5 inheritance can be autosomal dominant or recessive.

EXPRESSÃO TECIDUAL(Tecido-específico)
Testículo
28.3 TPM
Fallopian Tube
26.9 TPM
Bladder
20.8 TPM
Estômago
13.9 TPM
Cervix Endocervix
8.9 TPM
INTERAÇÕES PROTEICAS (5)
OUTRAS DOENÇAS (4)
congenital heart defects, multiple types, 5familial bicuspid aortic valvetetralogy of fallotfamilial atrial fibrillation
HGNC:15802UniProt:Q9BWX5
GATA4Transcription factor GATA-4Disease-causing germline mutation(s) (loss of function) inAltamente restrito
FUNÇÃO

Transcriptional activator that binds to the consensus sequence 5'-AGATAG-3' and plays a key role in cardiac development and function (PubMed:24000169, PubMed:27984724, PubMed:35182466). In cooperation with TBX5, it binds to cardiac super-enhancers and promotes cardiomyocyte gene expression, while it down-regulates endocardial and endothelial gene expression (PubMed:27984724). Involved in bone morphogenetic protein (BMP)-mediated induction of cardiac-specific gene expression. Binds to BMP respons

LOCALIZAÇÃO

Nucleus

VIAS BIOLÓGICAS (10)
Developmental Lineage of Pancreatic Acinar CellsDevelopmental Lineage of Multipotent Pancreatic Progenitor CellsDevelopmental Lineage of Pancreatic Ductal CellsSynthesis, secretion, and inactivation of Glucose-dependent Insulinotropic Polypeptide (GIP)Cardiogenesis
MECANISMO DE DOENÇA

Atrial septal defect 2

A congenital heart malformation characterized by incomplete closure of the wall between the atria resulting in blood flow from the left to the right atria. Patients show other heart abnormalities including ventricular and atrioventricular septal defects, pulmonary valve thickening or insufficiency of the cardiac valves. The disease is not associated with defects in the cardiac conduction system or non-cardiac abnormalities.

EXPRESSÃO TECIDUAL(Tecido-específico)
Ovário
144.1 TPM
Coração - Átrio
53.7 TPM
Testículo
51.1 TPM
Coração - Ventrículo esquerdo
45.2 TPM
Artéria coronária
40.7 TPM
OUTRAS DOENÇAS (12)
tetralogy of fallotventricular septal defect 1testicular anomalies with or without congenital heart diseaseatrial septal defect 2
HGNC:4173UniProt:P43694
TBX1T-box transcription factor TBX1Candidate gene tested inRestrito
FUNÇÃO

Transcription factor that plays a key role in cardiovascular development by promoting pharyngeal arch segmentation during embryonic development (By similarity). Also involved in craniofacial muscle development (By similarity). Together with NKX2-5, acts as a regulator of asymmetric cardiac morphogenesis by promoting expression of PITX2 (By similarity). Acts upstream of TBX1 for the formation of the thymus and parathyroid glands from the third pharyngeal pouch (By similarity). Required for hair f

LOCALIZAÇÃO

Nucleus

VIAS BIOLÓGICAS (1)
Cardiogenesis
VIAS REACTOME (1)
EXPRESSÃO TECIDUAL(Ubíquo)
Testículo
34.8 TPM
Músculo esquelético
27.5 TPM
Tireoide
22.5 TPM
Fallopian Tube
19.1 TPM
Cervix Ectocervix
15.9 TPM
OUTRAS DOENÇAS (9)
tetralogy of fallotvelocardiofacial syndromeDiGeorge syndromeconotruncal heart malformations
HGNC:11592UniProt:O43435
KDRVascular endothelial growth factor receptor 2Major susceptibility factor inAltamente restrito
FUNÇÃO

Tyrosine-protein kinase that acts as a cell-surface receptor for VEGFA, VEGFC and VEGFD. Plays an essential role in the regulation of angiogenesis, vascular development, vascular permeability, and embryonic hematopoiesis. Promotes proliferation, survival, migration and differentiation of endothelial cells. Promotes reorganization of the actin cytoskeleton. Isoforms lacking a transmembrane domain, such as isoform 2 and isoform 3, may function as decoy receptors for VEGFA, VEGFC and/or VEGFD. Isof

LOCALIZAÇÃO

Cell junctionEndoplasmic reticulumCell membraneCytoplasmNucleusCytoplasmic vesicleEarly endosomeSecreted

VIAS BIOLÓGICAS (1)
High laminar flow shear stress activates signaling by PIEZO1 and PECAM1:CDH5:KDR in endothelial cells
MECANISMO DE DOENÇA

Hemangioma, capillary infantile

A condition characterized by dull red, firm, dome-shaped hemangiomas, sharply demarcated from surrounding skin, usually presenting at birth or occurring within the first two or three months of life. They result from highly proliferative, localized growth of capillary endothelium and generally undergo regression and involution without scarring.

EXPRESSÃO TECIDUAL(Ubíquo)
Tireoide
80.1 TPM
Útero
78.6 TPM
Adipose Visceral Omentum
73.7 TPM
Pulmão
50.8 TPM
Mama
47.4 TPM
OUTRAS DOENÇAS (2)
capillary infantile hemangiomatetralogy of fallot
HGNC:6307UniProt:P35968
ZFPM2Zinc finger protein ZFPM2Major susceptibility factor inAltamente restrito
FUNÇÃO

Transcription regulator that plays a central role in heart morphogenesis and development of coronary vessels from epicardium, by regulating genes that are essential during cardiogenesis. Essential cofactor that acts via the formation of a heterodimer with transcription factors of the GATA family GATA4, GATA5 and GATA6. Such heterodimer can both activate or repress transcriptional activity, depending on the cell and promoter context. Also required in gonadal differentiation, possibly be regulatin

LOCALIZAÇÃO

Nucleus

VIAS BIOLÓGICAS (2)
Transcriptional regulation of testis differentiationFactors involved in megakaryocyte development and platelet production
MECANISMO DE DOENÇA

Tetralogy of Fallot

A congenital heart anomaly which consists of pulmonary stenosis, ventricular septal defect, dextroposition of the aorta (aorta is on the right side instead of the left) and hypertrophy of the right ventricle. In this condition, blood from both ventricles (oxygen-rich and oxygen-poor) is pumped into the body often causing cyanosis.

EXPRESSÃO TECIDUAL(Ubíquo)
Cérebro - Hemisfério cerebelar
39.2 TPM
Cerebelo
27.3 TPM
Ovário
27.0 TPM
Fallopian Tube
17.6 TPM
Útero
16.3 TPM
OUTRAS DOENÇAS (5)
tetralogy of fallot46,XY sex reversal 9diaphragmatic hernia 3congenital diaphragmatic hernia
HGNC:16700UniProt:Q8WW38
GJA5Gap junction alpha-5 proteinMajor susceptibility factor inModerado
FUNÇÃO

One gap junction consists of a cluster of closely packed pairs of transmembrane channels, the connexons, through which materials of low MW diffuse from one cell to a neighboring cell

LOCALIZAÇÃO

Cell membraneCell junction, gap junction

VIAS BIOLÓGICAS (1)
Gap junction assembly
MECANISMO DE DOENÇA

Atrial standstill 1

A rare arrhythmia characterized by the absence of electrical and mechanical activity in the atria. Electrocardiographically, it is characterized by bradycardia, the absence of P waves, and a junctional narrow complex escape rhythm.

VIAS REACTOME (1)
EXPRESSÃO TECIDUAL(Ubíquo)
Artéria coronária
39.1 TPM
Artéria tibial
34.6 TPM
Pulmão
32.6 TPM
Aorta
28.9 TPM
Adipose Visceral Omentum
22.7 TPM
OUTRAS DOENÇAS (4)
atrial fibrillation, familial, 11atrial standstill 1tetralogy of fallotfamilial atrial fibrillation
HGNC:4279UniProt:P36382
CITED2Cbp/p300-interacting transactivator 2Major susceptibility factor inAltamente restrito
FUNÇÃO

Transcriptional coactivator of the p300/CBP-mediated transcription complex. Acts as a bridge, linking TFAP2 transcription factors and the p300/CBP transcriptional coactivator complex in order to stimulate TFAP2-mediated transcriptional activation. Positively regulates TGF-beta signaling through its association with the SMAD/p300/CBP-mediated transcriptional coactivator complex. Stimulates the peroxisome proliferator-activated receptors PPARA transcriptional activity. Enhances estrogen-dependent

LOCALIZAÇÃO

Nucleus

VIAS BIOLÓGICAS (4)
Regulation of gene expression by Hypoxia-inducible FactorActivation of the TFAP2 (AP-2) family of transcription factorsTFAP2 (AP-2) family regulates transcription of other transcription factorsFOXO-mediated transcription of cell death genes
MECANISMO DE DOENÇA

Ventricular septal defect 2

A common form of congenital cardiovascular anomaly that may occur alone or in combination with other cardiac malformations. It can affect any portion of the ventricular septum, resulting in abnormal communications between the two lower chambers of the heart. Classification is based on location of the communication, such as perimembranous, inlet, outlet (infundibular), central muscular, marginal muscular, or apical muscular defect. Large defects that go unrepaired may give rise to cardiac enlargement, congestive heart failure, pulmonary hypertension, Eisenmenger's syndrome, delayed fetal brain development, arrhythmias, and even sudden cardiac death.

OUTRAS DOENÇAS (6)
ventricular septal defect 2atrial septal defect 8atrial septal defect, ostium secundum typeatrial septal defect, sinus venosus type
HGNC:1987UniProt:Q99967
GDF1Embryonic growth/differentiation factor 1Major susceptibility factor inTolerante
FUNÇÃO

May mediate cell differentiation events during embryonic development

LOCALIZAÇÃO

Secreted

VIAS BIOLÓGICAS (1)
Signaling by NODAL
MECANISMO DE DOENÇA

Conotruncal heart malformations

A group of congenital heart defects involving the outflow tracts. Examples include truncus arteriosus communis, double-outlet right ventricle and transposition of great arteries. Truncus arteriosus communis is characterized by a single outflow tract instead of a separate aorta and pulmonary artery. In transposition of the great arteries, the aorta arises from the right ventricle and the pulmonary artery from the left ventricle. In double outlet of the right ventricle, both the pulmonary artery and aorta arise from the right ventricle.

VIAS REACTOME (1)
OUTRAS DOENÇAS (4)
congenital heart defects, multiple types, 6right atrial isomerismisolated congenitally uncorrected transposition of the great arteriestetralogy of fallot
HGNC:4214UniProt:P27539
NKX2-5Homeobox protein Nkx-2.5Candidate gene tested inAltamente restrito
FUNÇÃO

Transcription factor required for the development of the heart and the spleen (PubMed:22560297). During heart development, acts as a transcriptional activator of NPPA/ANF in cooperation with GATA4 (By similarity). May cooperate with TBX2 to negatively modulate expression of NPPA/ANF in the atrioventricular canal (By similarity). Binds to the core DNA motif of NPPA promoter (PubMed:22849347, PubMed:26926761). Together with PBX1, required for spleen development through a mechanism that involves CD

LOCALIZAÇÃO

Nucleus

VIAS BIOLÓGICAS (3)
Physiological factorsYAP1- and WWTR1 (TAZ)-stimulated gene expressionCardiogenesis
MECANISMO DE DOENÇA

Atrial septal defect 7, with or without atrioventricular conduction defects

A congenital heart malformation characterized by incomplete closure of the wall between the atria resulting in blood flow from the left to the right atria, and atrioventricular conduction defects in some cases.

EXPRESSÃO TECIDUAL(Tecido-específico)
Coração - Átrio
113.8 TPM
Coração - Ventrículo esquerdo
108.3 TPM
Baço
44.1 TPM
Adipose Visceral Omentum
0.5 TPM
Testículo
0.3 TPM
OUTRAS DOENÇAS (15)
hypoplastic left heart syndrome 2tetralogy of fallotventricular septal defect 3hypothyroidism, congenital, nongoitrous, 5
HGNC:2488UniProt:P52952
FLT4Vascular endothelial growth factor receptor 3Disease-causing germline mutation(s) (loss of function) inAltamente restrito
FUNÇÃO

Tyrosine-protein kinase that acts as a cell-surface receptor for VEGFC and VEGFD, and plays an essential role in adult lymphangiogenesis and in the development of the vascular network and the cardiovascular system during embryonic development. Promotes proliferation, survival and migration of endothelial cells, and regulates angiogenic sprouting. Signaling by activated FLT4 leads to enhanced production of VEGFC, and to a lesser degree VEGFA, thereby creating a positive feedback loop that enhance

LOCALIZAÇÃO

Cell membraneCytoplasmNucleusSecreted

VIAS BIOLÓGICAS (3)
VEGF binds to VEGFR leading to receptor dimerizationNOTCH4 Intracellular Domain Regulates TranscriptionHigh laminar flow shear stress activates signaling by PIEZO1 and PECAM1:CDH5:KDR in endothelial cells
MECANISMO DE DOENÇA

Lymphatic malformation 1

A form of primary lymphedema, a disease characterized by swelling of body parts due to developmental anomalies and functional defects of the lymphatic system. Patients with lymphedema may suffer from recurrent local infections. LMPHM1 is an autosomal dominant form with variable expression and severity. Onset is usually at birth or in early childhood but can occur later. Affected individuals manifest lymphedema, predominantly in the lower limbs, and hypoplasia of lymphatic vessels. Additional features are hemangioma and nail dysplasia or papillomatosis.

EXPRESSÃO TECIDUAL(Ubíquo)
Tireoide
64.0 TPM
Pulmão
34.8 TPM
Baço
31.3 TPM
Mama
28.9 TPM
Adipose Visceral Omentum
28.7 TPM
OUTRAS DOENÇAS (4)
lymphatic malformation 1congenital heart defects, multiple types, 7capillary infantile hemangiomatetralogy of fallot
HGNC:3767UniProt:P35916
JAG1Protein jagged-1Disease-causing germline mutation(s) inAltamente restrito
FUNÇÃO

Ligand for multiple Notch receptors and involved in the mediation of Notch signaling (PubMed:18660822, PubMed:20437614). May be involved in cell-fate decisions during hematopoiesis (PubMed:9462510). Seems to be involved in early and late stages of mammalian cardiovascular development. Inhibits myoblast differentiation (By similarity). Enhances fibroblast growth factor-induced angiogenesis (in vitro)

LOCALIZAÇÃO

MembraneCell membrane

VIAS BIOLÓGICAS (10)
NOTCH2 Activation and Transmission of Signal to the NucleusRAC3 GTPase cycleRAC1 GTPase cycleActivated NOTCH1 Transmits Signal to the NucleusConstitutive Signaling by NOTCH1 PEST Domain Mutants
MECANISMO DE DOENÇA

Alagille syndrome 1

A form of Alagille syndrome, an autosomal dominant multisystem disorder. It is clinically defined by hepatic bile duct paucity and cholestasis in association with cardiac, skeletal, and ophthalmologic manifestations. There are characteristic facial features and less frequent clinical involvement of the renal and vascular systems.

EXPRESSÃO TECIDUAL(Ubíquo)
Artéria tibial
202.9 TPM
Artéria coronária
150.2 TPM
Aorta
133.4 TPM
Skin Sun Exposed Lower leg
102.3 TPM
Vagina
102.2 TPM
OUTRAS DOENÇAS (5)
tetralogy of fallotCharcot-Marie-Tooth disease, axonal, Type 2HHAlagille syndrome due to a JAG1 point mutationdeafness, congenital heart defects, and posterior embryotoxon
HGNC:6188UniProt:P78504

Medicamentos aprovados (FDA)

1 medicamento encontrado nos registros da FDA americana.

💊 Alprostadil (ALPROSTADIL)
Ver no DailyMed/FDA

Variantes genéticas (ClinVar)

1,049 variantes patogênicas registradas no ClinVar.

🧬 JAG1: NM_000214.3(JAG1):c.2273C>T (p.Thr758Ile) ()
🧬 JAG1: NM_000214.3(JAG1):c.2670del (p.Ile890fs) ()
🧬 JAG1: NM_000214.3(JAG1):c.1521_1522del (p.Asn507fs) ()
🧬 JAG1: NM_000214.3(JAG1):c.1349-2A>C ()
🧬 JAG1: NM_000214.3(JAG1):c.1248T>A (p.Cys416Ter) ()
Ver todas no ClinVar

Classificação de variantes (ClinVar)

Distribuição de 394 variantes classificadas pelo ClinVar.

79
295
20
Patogênica (20.1%)
VUS (74.9%)
Benigna (5.1%)
VARIANTES MAIS SIGNIFICATIVAS
NKX2-5: NM_004387.4(NKX2-5):c.334+15C>T [Conflicting classifications of pathogenicity]
GATA6: NM_005257.6(GATA6):c.1135+1G>A [Likely pathogenic]
HEY2: NM_012259.3(HEY2):c.171G>C (p.Glu57Asp) [Pathogenic]
JAG1: NM_000214.3(JAG1):c.138dup (p.Gly47fs) [Likely pathogenic]
JAG1: NM_000214.3(JAG1):c.134_154dup (p.Asn51_Gly52insValAsnGlyGluLeuGlnAsn) [Uncertain significance]

Vias biológicas (Reactome)

39 vias biológicas associadas aos genes desta condição.

Surfactant metabolism Cardiogenesis Formation of definitive endoderm Factors involved in megakaryocyte development and platelet production Developmental Lineage of Multipotent Pancreatic Progenitor Cells Regulation of gene expression in endocrine-committed (NEUROG3+) progenitor cells YAP1- and WWTR1 (TAZ)-stimulated gene expression Synthesis, secretion, and inactivation of Glucose-dependent Insulinotropic Polypeptide (GIP) Physiological factors Transcriptional regulation of testis differentiation Formation of lateral plate mesoderm Developmental Lineage of Pancreatic Acinar Cells Developmental Lineage of Pancreatic Ductal Cells Neurophilin interactions with VEGF and VEGFR VEGF binds to VEGFR leading to receptor dimerization Integrin cell surface interactions VEGFA-VEGFR2 Pathway VEGFR2 mediated cell proliferation Signaling by membrane-tethered fusions of PDGFRA or PDGFRB High laminar flow shear stress activates signaling by PIEZO1 and PECAM1:CDH5:KDR in endothelial cells Gap junction assembly Regulation of gene expression by Hypoxia-inducible Factor TFAP2 (AP-2) family regulates transcription of other transcription factors Activation of the TFAP2 (AP-2) family of transcription factors FOXO-mediated transcription of cell death genes Signaling by NODAL NOTCH4 Intracellular Domain Regulates Transcription Activated NOTCH1 Transmits Signal to the Nucleus Constitutive Signaling by NOTCH1 PEST Domain Mutants Constitutive Signaling by NOTCH1 t(7;9)(NOTCH1:M1580_K2555) Translocation Mutant Constitutive Signaling by NOTCH1 HD Domain Mutants Constitutive Signaling by NOTCH1 HD+PEST Domain Mutants NOTCH2 Activation and Transmission of Signal to the Nucleus RUNX3 regulates NOTCH signaling RAC1 GTPase cycle RAC3 GTPase cycle NOTCH3 Activation and Transmission of Signal to the Nucleus NOTCH4 Activation and Transmission of Signal to the Nucleus Nephron development

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Pipeline de tratamentos
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Aprovado1
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1Fase 11
·Pré-clínico17
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 20 ensaios
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97 ensaios clínicos encontrados, 20 ativos.

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Publicações mais relevantes

📖Melhor nível de evidência: Revisão
Timeline de publicações
4.505 papers (10 anos)

Mostrando amostra de 200 publicações de um total de 4.505

#1

Utility of three-dimensional echocardiography for evaluating right ventricular size and function and ventricular myocardial deformation in repaired tetralogy of fallot.

PloS one2026

Para pacientes com Tetralogia de Fallot reparada (rTOF), onde a disfunção do ventrículo direito é uma preocupação e a ressonância magnética cardíaca (RMC) tem limitações, o ecocardiograma 3D (3DE) surge como uma ferramenta de monitoramento complementar. Ele demonstrou boa correlação com a RMC para avaliar volumes do ventrículo direito e, importante para médicos, sua análise de deformação miocárdica (strain) foi eficaz em identificar disfunção sistólica nesses pacientes, apoiando seu uso como um recurso valioso no acompanhamento longitudinal.

🇧🇷 traduzido
#2

Genetic Syndromes Do Not Affect Survival but Increase Morbidity in Neonates with Symptomatic Tetralogy of Fallot.

The Journal of pediatrics2026 Mar 09

Este estudo indica que a presença de síndromes genéticas em recém-nascidos com Tetralogia de Fallot sintomática não afeta a taxa de sobrevivência a longo prazo. No entanto, esses pacientes enfrentam maior morbidade, necessitando de internações mais longas em UTI e no hospital total, além de maior probabilidade de alta com sondas de alimentação. Consequentemente, o teste genético é uma ferramenta valiosa para médicos e famílias, fornecendo informações cruciais para o planejamento do cuidado e expectativas clínicas.

🇧🇷 traduzido
#3

Optimal Size of Transannular Patches for Tetralogy of Fallot Repair.

Heart, lung &amp; circulation2026 Mar 10

Este estudo sobre a reparação da Tetralogia de Fallot aponta que o tamanho do remendo transanular utilizado influencia o risco de regurgitação pulmonar (RP) a longo prazo. Em pacientes, um alargamento excessivo do anel da válvula pulmonar, definido por um aumento no escore Z de 4 ou mais em relação ao pré-operatório, foi um fator de risco significativo para RP progressiva. Para médicos, isso sugere que um alargamento menos extenso da válvula pulmonar (aumento do escore Z inferior a 4) está associado a melhores resultados, minimizando esta complicação pós-cirúrgica.

🇧🇷 traduzido
#4

Age-dependent Dynamics of the Electrocardiographic Parameters in Cardiovascular Disease-Free Children.

medRxiv : the preprint server for health sciences2026 Feb 09

Este estudo aprimorou as referências de eletrocardiograma (ECG) em crianças, fornecendo valores mais precisos e com maior resolução etária, especialmente para neonatos, período de rápidas mudanças cardíacas. Para médicos e pacientes, esses dados são cruciais, pois permitem identificar precocemente desvios em condições como a Tetralogia de Fallot não reparada, onde foram encontradas diferenças significativas em parâmetros como frequência cardíaca, PR, QRS e QTc. Essa maior especificidade ajuda a otimizar o diagnóstico e o manejo de doenças cardíacas desde a primeira infância.

🇧🇷 traduzido
#5

Two young athletes with Tetralogy of Fallot-stress echocardiography in therapeutic decision-making: a case report.

European heart journal. Case reports2026 Feb

Em atletas com Tetralogia de Fallot, a excelente capacidade física pode mascarar a deterioração cardíaca precoce, dificultando o diagnóstico com métodos convencionais. A ecocardiografia de estresse (em esforço) surge como uma ferramenta essencial para médicos e pacientes, pois revela disfunções cardíacas ocultas — como a dessincronia biventricular — que seriam imperceptíveis em repouso. Esta avaliação detalhada permite decisões terapêuticas mais precisas, como a indicação de troca valvar, otimizando o acompanhamento e o tratamento para garantir a saúde cardíaca a longo prazo desses pacientes.

🇧🇷 traduzido

Publicações recentes

Ver todas no PubMed

📚 EuropePMC6.874 artigos no totalmostrando 196

2026

Not just palliation: integrating patent ductus arteriosus and right ventricular outflow tract stenting into modern surgical pathways for tetralogy of Fallot.

Cardiology in the young
2026

Left ventricular quantification in repaired tetralogy of Fallot: two-dimensional echocardiography versus cardiovascular magnetic resonance.

The international journal of cardiovascular imaging
2025

Transcatheter Pulmonary Valve Replacement (TPVR): A Saudi Consensus Statement.

Journal of the Saudi Heart Association
2026

Persistent Double Dorsal Aorta and Complex Congenital Heart Disease - A Case Report.

Journal of the Saudi Heart Association
2026

Utility of three-dimensional echocardiography for evaluating right ventricular size and function and ventricular myocardial deformation in repaired tetralogy of fallot.

PloS one
2026

Surgical retrieval and repositioning of malpositioned prosthetic pulmonic valve in a patient with situs inversus dextrocardia and tetralogy of Fallot: a case report.

International journal of surgery case reports
2026

Comments on "Sex differences late after tetralogy of Fallot repair: a systematic review and meta-analysis".

Indian journal of thoracic and cardiovascular surgery
2026

A New Treatment Paradigm for Tetralogy of Fallot/Absent Pulmonary Valve with Significant Airway Compromise - Addressing the Airway First.

The Annals of thoracic surgery
2026

Incidence and Factors Associated with Neurological Complications Following Pediatric Heart Surgery: A Retrospective Study.

Journal of clinical medicine
2026

Isolated Transcatheter Tricuspid Valve Edge-to-Edge Repair in Repaired Tetralogy of Fallot.

JACC. Case reports
2026

Palliative Balloon Pulmonary Valvotomy Enhances Immediate Postoperative Outcomes in Severely Cyanotic Patients With Tetralogy of Fallot.

World journal for pediatric &amp; congenital heart surgery
2025

15O-water PET perfusion in complex congenital heart disease.

European heart journal. Imaging methods and practice
2026

Surgical repair of a child with tetralogy of Fallot with absent left pulmonary artery: a case report.

International journal of surgery case reports
2026

Genetic Syndromes Do Not Affect Survival but Increase Morbidity in Neonates with Symptomatic Tetralogy of Fallot.

The Journal of pediatrics
2026

Infective endocarditis in an adult male patient with tetralogy of Fallot physiology secondary to double outlet right ventricle presenting with stroke: a complex presentation with multiorgan dysfunction-a case report.

Journal of medical case reports
2026

Optimal Size of Transannular Patches for Tetralogy of Fallot Repair.

Heart, lung &amp; circulation
2026

Evaluation of the Venus P-valve for transcatheter pulmonary valve replacement in patients with right ventricular outflow tract dysfunction.

Future cardiology
2026

Visualisation of cardiac magnetic resonance-based anatomic models in virtual reality to evaluate candidacy for transcatheter pulmonary valve replacement in surgically palliated right ventricular outflow tracts.

Cardiology in the young
2026

Agreement between single and multiple ROI strategies for hepatic T1 and ECV mapping in tetralogy of Fallot and Fontan circulation.

Abdominal radiology (New York)
2026

Right ventricular outflow tract stenting for obstructive lesions in adult congenital heart disease.

International journal of cardiology. Congenital heart disease
2026

Surgical repair of Tetralogy of Fallot - Long-term outcomes into the second decade after transannular patch repair and annulus-sparing patch repair.

International journal of cardiology. Congenital heart disease
2026

Pulmonary vasomodulators in adults with repaired pulmonary atresia with ventricular septal defect: Single-center experience and review of literature.

International journal of cardiology. Congenital heart disease
2026

Dynamics of matrix metalloproteinases and procollagen type-1 N-terminal propeptide following right ventricular outflow tract reconstruction: the protective mechanism of restrictive physiology.

Cardiology in the young
2026

Transcatheter Pulmonary Artery Intervention in Tetralogy of Fallot With Pulmonary Atresia During Pregnancy.

JACC. Case reports
2026

The use of human decellularized amniotic membrane as pulmonary valve leaflets in right ventricular outflow tract reconstruction - an in vivo proof of concept study.

Frontiers in bioengineering and biotechnology
2026

Transannular patch repair of tetralogy of Fallot.

Asian cardiovascular &amp; thoracic annals
2026

Development of 3D-Printed Congenital Heart Disease Models Using Feasible Low-Cost Workflow - A Potential Tool to Improve Pediatric Cardiology Education.

Arquivos brasileiros de cardiologia
2026

Preoperative decision tree model for predicting pulmonary valve-sparing repair in humanitarian pediatric tetralogy of fallot patients.

Journal of cardiothoracic surgery
2026

Experience with patients presenting with the clinical features of Holt-Oram syndrome: a single center retrospective study.

Journal of cardiothoracic surgery
2026

A Comparison of Treatment Options for Right Ventricular Outflow Tract Obstruction.

The Thoracic and cardiovascular surgeon
2026

In silico Analysis of CHD4 Mutations Reveals Domain-Specific Impacts on Cardiovascular Disorders Among Patients With Rare Diseases.

Human mutation
2026

Late Diagnosis of Unroofed Coronary Sinus Associated with Persistent Left Superior Vena Cava in Patient with Repaired Tetralogy of Fallot: Case Report.

Life (Basel, Switzerland)
2026

Tetralogy of Fallot: Genetic, Epigenetic and Clinical Insights into a Multifactorial Congenital Heart Disease.

Genes
2026

Osteopathic Manipulative Treatment in 564 Children with Congenital Heart Disease: A Project Report.

Children (Basel, Switzerland)
2026

Long-Term Arrhythmic Complications in Patients with Repaired Tetralogy of Fallot: A Single-Center Experience from 1960 to the Present.

Pediatric cardiology
2026

The Hypoxic Link: Paragangliomas in Unrepaired Tetralogy of Fallot.

Radiology. Cardiothoracic imaging
2025

Right aortic arch with cervical origin of left subclavian artery: Unusual arch anomaly associated with tetralogy of Fallot.

Annals of pediatric cardiology
2026

Case Report: A rare case of ANCA-positive Q fever endocarditis-associated glomerulonephritis.

Frontiers in medicine
2026

Incidence, risk factors, and outcome of early post-operative arrhythmia after open-heart surgery in the paediatric cardiac intensive care unit: a retrospective study.

Cardiology in the young
2026

Age-dependent Dynamics of the Electrocardiographic Parameters in Cardiovascular Disease-Free Children.

medRxiv : the preprint server for health sciences
2026

Two young athletes with Tetralogy of Fallot-stress echocardiography in therapeutic decision-making: a case report.

European heart journal. Case reports
2026

Persistent left superior vena cava as a potential arrhythmogenic substrate in a patient with repaired tetralogy of Fallot.

HeartRhythm case reports
2025

Complete sternal cleft with pectus excavatum associated with tetralogy of Fallot.

Annals of pediatric cardiology
2025

Critique on "determining the optimal timing for tetralogy of Fallot management: A meta-analysis of neonatal versus postneonatal repairs".

Annals of pediatric cardiology
2026

Kingella kingae Endocarditis and Septic Pulmonary Emboli in a 12-Year-Old Male With Repaired Tetralogy of Fallot and a Bioprosthetic Pulmonary Valve.

Case reports in cardiology
2026

Late Gadolinium Enhancement in Repaired Tetralogy of Fallot May Overestimate Right Ventricular Myocardial Fibrosis.

Pediatric cardiology
2025

11-Year follow-up of percutaneous pulmonary valve implantation (PPVI) in central South Africa.

Cardiovascular journal of Africa
2026

Successful transcatheter pulmonary valve implantation under elective extracorporeal membrane oxygenation in a patient with tetralogy of Fallot with biventricular failure and ventricular tachycardia: a case report.

European heart journal. Case reports
2026

Esophageal erosion by a PDA occluder device following MAPC closure in tetralogy of Fallot.

Oxford medical case reports
2026

Effect of the Implantation Angle on the Durability of a Bio-Prosthetic Pulmonary Valve in the Tetralogy of Fallot.

Korean circulation journal
2026

Prognostic Value of Cardiac MRI-derived Left Atrial and Biventricular Function in Children with Repaired Tetralogy of Fallot.

Radiology. Cardiothoracic imaging
2026

An alternative surgical approach to redo pulmonary valve replacement: the vertical left axillary thoracotomy.

Cardiology in the young
2026

Development and multicentre validation of an artificial intelligence electrocardiogram model for ventricular remodeling in repaired tetralogy of Fallot.

European heart journal. Digital health
2026

Computed tomography angiography imaging of double superior vena cava in a case of tetralogy of fallot.

Radiology case reports
2026

Long-term Oral Management for 2q37 Deletion Syndrome Patient.

The Bulletin of Tokyo Dental College
2026

[Molecular mechanisms of the TRPV4-RhoA/ROCK1 signaling axis in mediating mechanical stress-induced myocardial fibrosis in fetuses with tetralogy of Fallot].

Zhonghua xin xue guan bing za zhi
2026

Pulmonary Valve Replacement: Update on Timing and Ventricular Remodelling.

Journal of clinical medicine
2026

Genetic Syndromes and Multimorbidity in Adults with Congenital Heart Disease and Heart Failure: Insights from the PATHFINDER-CHD Registry.

Journal of clinical medicine
2026

Extracellular Matrix Tissue Patch for Pulmonary Artery Repair in Pediatric Cardiac Surgery: A Single-Center Experience.

Journal of clinical medicine
2026

Strain Analysis from Transverse CMR Cine Imaging in Congenital Heart Disease: Feasibility, Reproducibility, and Comparison to Global Longitudinal Strain.

Healthcare (Basel, Switzerland)
2026

Ultrasound-guided guidewire maneuver to resolve peritoneal drainage catheter obstruction in an infant: A case report.

Radiology case reports
2026

Arrhythmia Following Congenital Heart Disease Surgery in Oman: Incidence and risk factors - A prospective study.

Sultan Qaboos University medical journal
2025

Impact of right ventricular incision extent on early outcomes after tetralogy of Fallot repair: a two-center retrospective cohort study.

Frontiers in cardiovascular medicine
2026

Safety and Efficacy of HarmonyTM Transcatheter Pulmonary Valve Implantation - Clinical and Hemodynamic Assessment in a Single-Center Japanese Cohort.

Circulation journal : official journal of the Japanese Circulation Society
2026

Evaluation and application of electrocardiographic age model for children.

Scientific reports
2026

Transcatheter pulmonary valve implantation in patients with repaired tetralogy of Fallot: International evidence and the current status in Japan.

Journal of cardiology
2026

Hybrid rule-based and on-premises LLM pipeline for extracting CMR and CPET metrics from free-text reports in repaired tetralogy of Fallot.

medRxiv : the preprint server for health sciences
2026

Beyond Volume Criteria - Individualized Approach to Pulmonary Regurgitation in Tetralogy of Fallot and Related Diseases.

Circulation journal : official journal of the Japanese Circulation Society
2026

Smaller Right Ventricle Results in Poorer Exercise Performance in Young Patients With Repaired Tetralogy of Fallot.

Circulation journal : official journal of the Japanese Circulation Society
2026

Discovery Beyond the "Undiscovered Country": Re-Exploring the Right Ventricle Through Metabolomics and Cardiac Magnetic Resonance.

JACC. Asia
2026

Reference standards and diagnosis-specific trends in cardiorespiratory fitness in paediatric patients with repaired CHD.

Cardiology in the young
2026

Recurrent left internal carotid artery stenosis in a patient with intrathoracic carotid bifurcation and right aortic arch.

Neuroradiology
2025

A Rare Case of D-transposition of the Great Arteries (TGA) With Ventricular Septal Defect (VSD), Dysplastic Pulmonary Valve (Absent Pulmonary Valve Physiology), and Aortic Valve Stenosis in a Term Neonate.

Cureus
2026

Strategies for preventing arrhythmic sudden death in adult congenital heart disease.

European heart journal supplements : journal of the European Society of Cardiology
2026

Antiarrhythmic Medications in Patients with Adult Congenital Heart Disease.

Cardiac electrophysiology clinics
2026

Incidence, Progression, and Surgical Outcomes of Aortopathies in Adults With Congenital Heart Disease: A Systematic Review.

World journal for pediatric &amp; congenital heart surgery
2026

Influence of Technical Performance Score on Outcomes After Tetralogy of Fallot Repair in a Low-Middle-Income Country.

World journal for pediatric &amp; congenital heart surgery
2026

Atrial Flutter Radiofrequency Ablation After Secondary Surgery in Adult Congenital Heart Disease.

JACC. Case reports
2026

Prognostic value of fetal growth and prenatal functional echocardiography in tetralogy of FALLOT.

Acta obstetricia et gynecologica Scandinavica
2025

Is Cyanosis Exposure Associated with Exercise Capacity or Daily Physical Activity in Children with Complex Congenital Heart Disease: A Cross-Sectional Study.

Children (Basel, Switzerland)
2026

The utility of 4D intracardiac echocardiography in transcatheter pulmonary valve replacement in adult congenital heart disease patients.

International journal of cardiology. Congenital heart disease
2026

Residual shunts after tetralogy of Fallot repair: two case illustrations and echocardiographic insights.

European heart journal. Case reports
2025

Fetal echocardiography: A nine-year experience at a single tertiary center.

Northern clinics of Istanbul
2025

Case Report: Low cardiac output syndrome with multisystem complications following total repair of tetralogy of fallot.

Frontiers in pediatrics
2026

Progression of cardiac remodelling in adolescents and adults with tetralogy of Fallot: Insights from serial magnetic resonance imaging.

Kardiologia polska
2026

Symptomatic Pulmonary Regurgitation Without Right Ventricular Enlargement Accompanied by Biventricular Diastolic Dysfunction in Repaired Tetralogy of Fallot and Related Diseases.

Circulation journal : official journal of the Japanese Circulation Society
2026

Surgical Rescue of Right Ventricular Outflow Tract Stent Malposition With Tricuspid Valve Compromise.

World journal for pediatric &amp; congenital heart surgery
2026

Transcatheter pulmonary valve implantation for re-intervention in recurrent right ventricular outflow tract obstruction.

Journal of cardiology cases
2026

A Rare Neonatal Case of 48,XXYY Syndrome Presenting With Ambiguous Genitalia and Tetralogy of Fallot.

Clinical case reports
2026

Building Lifelong Management for the Right Ventricular Outflow Tract.

JACC. Cardiovascular interventions
2026

Long-Term Outcomes After Melody Transcatheter Pulmonary Valve Implant in Patients Weighing ≤30 kg.

JACC. Cardiovascular interventions
2025

Absent ductus arteriosus in Tetralogy of Fallot: early outcomes from a matched fetal cohort study.

Cardiology in the young
2026

[Long-term management of right ventricular outflow tract dysfunction in repaired tetralogy of Fallot: an interpretation of the 2024 scientific statement from the American Heart Association].

Zhonghua xin xue guan bing za zhi
2025

Assessing liver disease in cyanotic CHD using multiparametric MRI: a call to prevent future burden.

Cardiology in the young
2026

Surgical remodeling techniques for the right ventricle during pulmonary valve replacement.

Turk gogus kalp damar cerrahisi dergisi
2025

Echocardiographic Markers of Right Ventricle Diastolic Dysfunction in Neonates and Infants with Congenital Heart Disease.

Journal of clinical medicine
2026

Minimally Invasive Cardiac Surgery for Treating Tetralogy of Fallot in Children in the Modern Era.

The Annals of thoracic surgery
2026

An extremely rare tetralogy of Fallot with absent pulmonary valve and unilateral absence of the pulmonary artery: a rare report of De Bucket Syndrome.

The Egyptian heart journal : (EHJ) : official bulletin of the Egyptian Society of Cardiology
2026

DNA methylation analysis of NOTCH1 variants reveals the first episignature for non-syndromic congenital heart defects.

Genome medicine
2026

4D Flow Yields Similar Clinical Results Compared To 2D Phase Contrast for Decision Making Regarding Pulmonary Valve Replacement in Repaired Tetralogy of Fallot.

Pediatric cardiology
2026

Importance of clinical practice guidelines for specialized delivery room resuscitation of newborns with prenatally diagnosed critical congenital heart disease.

Journal of perinatology : official journal of the California Perinatal Association
2025

Results of right ventricular outflow tract stenting as a primary palliation in symptomatic children having Fallot type of lesion.

Cardiology in the young
2025

Imaging considerations in tetralogy of Fallot: A comprehensive review.

World journal of radiology
2026

Aplasia Cutis Congenita and Congenital Heart Disease: A Case Report, Highlighting the Limitation of Antenatal Screening.

The American journal of case reports
2025

Pulmonary Valve Replacement-Related Change in Biventricular Global Function Index in Repaired Tetralogy of Fallot.

Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance
2026

A Regional Approach to a Global Challenge: A Case Report of Caudal Anesthesia for Anoplasty in Tetralogy of Fallot With Pulmonary Atresia.

Clinical case reports
2025

Factors Leading to Delay in Surgical Treatment of Paediatric Congenital Heart Diseases in Rural Western India - A Hospital-Based Study.

Nigerian medical journal : journal of the Nigeria Medical Association
2025

Identification of a Novel FLNC Truncating Variant in Fetal Tetralogy of Fallot: A Case Report and Review of the Literature.

Diagnostics (Basel, Switzerland)
2025

Artificial Intelligence in Tetralogy of Fallot: From Prenatal Diagnosis to Lifelong Management: A Narrative Review.

Bioengineering (Basel, Switzerland)
2025

Association of pulmonary arterial end-diastolic forward flow and right heart remodeling in dogs with congenital pulmonic stenosis and precapillary pulmonary hypertension.

Journal of veterinary cardiology : the official journal of the European Society of Veterinary Cardiology
2025

Experiencia de implante percutáneo de válvula pulmonar en un hospital de referencia cardiovascular en Colombia. Serie de casos.

Archivos de cardiologia de Mexico
2026

Tetralogy of Fallot with pulmonary atresia - Role of patent ductus arteriosus stenting as initial palliation.

Asian cardiovascular &amp; thoracic annals
2025

Standardized mortality ratio in adults with congenital heart disease.

European heart journal open
2025

Arrhythmia mechanisms and results of transcatheter ablation therapy in patients diagnosed with CHD: the most difficult and dark side of paediatric electrophysiology.

Cardiology in the young
2026

Sex differences late after tetralogy of Fallot repair: a systematic review and meta-analysis.

Indian journal of thoracic and cardiovascular surgery
2026

Z-Drug Use in the First Trimester of Pregnancy and Risk of Congenital Malformations.

JAMA psychiatry
2025

Late Repair of Native Pulmonary Valve for Severe Pulmonary Regurgitation After Transannular Patch Repair in Tetralogy of Fallot.

Annals of thoracic surgery short reports
2026

2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines.

Journal of the American College of Cardiology
2025

Catheter Ablation of Ventricular Arrhythmias in Patients With Congenital Heart Diseases: A Nationwide Prospective Study.

JACC. Clinical electrophysiology
2026

Decision-making to use right ventricle to pulmonary artery conduit: Advantages of the expanded polytetrafluoroethylene valved conduit.

Asian cardiovascular &amp; thoracic annals
2025

Clinical outcomes following surgical intervention for critical congenital heart disease in neonates: a retrospective study in China.

BMC pediatrics
2026

Transcatheter Implantation of Balloon-Expandable Valve in a Repaired Native Tricuspid Valve Without Annuloplasty Ring.

Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography &amp; Interventions
2025

Bleeding and Thrombotic Challenges in Children with uncorrected Cyanotic Congenital Heart Diseases (CCHD)- Experience from a tertiary care pediatric hospital.

Pakistan journal of medical sciences
2025

Antenatal spectrum of congenital heart disease: Experience from a Northern Indian referral unit.

Bioinformation
2025

Valve Creation From Atrial Appendage Tissue for Primary Repair of Tetralogy of Fallot in Infants.

JACC. Case reports
2025

Structural birth defects and leukemia risk in children with Down syndrome.

Scientific reports
2026

Pulsed Field Ablation for Ventricular Arrhythmia in Repaired Tetralogy of Fallot.

JACC. Case reports
2026

Prenatal Exposure to Antiseizure Medications and the Risk of Congenital Anomalies: A Nationwide Population-Based Study in South Korea.

Neurology
2025

Palliative RVOT Stenting Using a Drug-Eluting Coronary Stent in a Preterm Neonate With Severe PS, VSD, and Bilateral Branch PA Stenosis: A Case Report.

Clinical case reports
2025

Imbalance of Fetal Growth Factor Levels in Congenital Heart Disease Pathology: A Systematic Review.

Birth defects research
2025

From repair to right heart failure: Understanding the evolving landscape in tetralogy of fallot.

Heart failure reviews
2026

Percutaneous Edge-to-Edge Repair of Atrioventricular Valves in Congenital Heart Disease: A Multicenter Clinical Experience.

JACC. Advances
2026

Biatrial Flutter in Repaired Tetralogy of Fallot: Complementary Role of Activation and Entrainment Mapping in Understanding the Mechanism of a Complex Atrial Arrhythmia.

JACC. Clinical electrophysiology
2026

Slow Conducting Anatomical Isthmus and VT Inducibility in Repaired Tetralogy of Fallot: It Takes More Than 2 to Tango.

JACC. Clinical electrophysiology
2025

Pulse Oximetry Screening for Critical Congenital Heart Defects: Effectiveness and Implementation across Clinical Settings.

Cardiology and cardiovascular medicine
2026

Managing Unrepaired Tetralogy of Fallot in Pregnancy: A Rare Case Defying Standard Cardiac Guidelines.

JACC. Case reports
2025

Neopulmonary Valve Reconstruction Using Autologous Right Atrial Appendage: Early Single-Center Results.

World journal for pediatric &amp; congenital heart surgery
2025

Integrated Open-Source Framework for Simulation of Transcatheter Pulmonary Valves in Native Right Ventricular Outflow Tracts.

ArXiv
2026

Early-Term Outcomes of Percutanous Pulmonary Valve Implantation With the Meril's Myval Pulmonary Valve-Single Center Experience From Turkey.

Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography &amp; Interventions
2025

Recurrent staphylococcus epidermidis prosthetic valve endocarditis with deep sternal infection in repaired tetralogy of fallot: A case report.

International journal of surgery case reports
2025

Short- and Mid-term Cardiac Ventricular Remodelling After Pulmonary Valve Replacement in Adults With Repaired Tetralogy of Fallot.

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
2025

Exercise Testing and Counseling in Patients with Tetralogy of Fallot: A Guide for the Pediatric and Congenital Cardiologist.

Pediatric cardiology
2026

Preoperative Home Monitoring for Neonates and Young Infants With Tetralogy of Fallot.

The Journal of cardiovascular nursing
2025

Virtual Cath Lab: Versatile Open-Source Simulator for Education and Procedural Planning in Congenital Heart Interventions.

Journal of the Society for Cardiovascular Angiography &amp; Interventions
2025

Clinical characteristics and outcomes of cardiovascular surgery and transcatheter procedures in adults with Down syndrome and congenital heart disease.

International journal of cardiology. Congenital heart disease
2025

Burden of diffuse myocardial fibrosis assessed by cardiac magnetic resonance in repaired tetralogy of Fallot: A systematic review and meta-analysis.

International journal of cardiology. Congenital heart disease
2025

A Case Series of Hypogonadism in 22q11.2 Deletion Syndrome: Is It Time to Check the Gonadal Axis?

Journal of investigative medicine high impact case reports
2025

Vortex formation of cardiac bilateral ventricles in fetuses with tetralogy of fallot assessed by blood speckle-tracking echocardiography.

BMC pregnancy and childbirth
2025

Right Ventricular Volume Unload: Transcatheter Tricuspid Valve Replacement in Congenital Heart Disease.

JACC. Case reports
2025

Mid- and Late-term Left Ventricular Diastolic Function After the Ross Procedure.

Pediatric cardiology
2025

Prevalence and Patterns of Neuro-developmental problems among children with Congenital Heart Diseases attending a tertiary institution in South East Nigeria.

Malawi medical journal : the journal of Medical Association of Malawi
2025

Incidence, Risk Factors and Outcomes of Junctional Ectopic Tachycardia After Tetralogy of Fallot Repair in Pediatric Patients.

Children (Basel, Switzerland)
2025

Importance of Preoperative Left Ventricle Volume Assessment in Asymptomatic Tetralogy of Fallot Infants.

Annals of thoracic and cardiovascular surgery : official journal of the Association of Thoracic and Cardiovascular Surgeons of Asia
2025

Noninvasive Assessment of Right Ventricular-Pulmonary Arterial Coupling in Repaired Tetralogy of Fallot by Magnetic Resonance Imaging.

Journal of the American Heart Association
2026

Ablation in Adult Congenital Heart Disease Using a Dual-Energy, Lattice-Tip, Large-Footprint, Map-and-Ablate Catheter.

JACC. Case reports
2025

Dual Disease Burden: Growing Older with Congenital Heart Disease and Hereditary Metabolic and Connective Tissue Disorders-Data from the PATHFINDER-CHD Registry on Heart Failure.

Geriatrics (Basel, Switzerland)
2025

The relation between Down syndrome and co-occurring conditions in children and young adults: A population-based cohort in Denmark, 1977-2016.

Research in developmental disabilities
2026

Inappropriate antibiotic prophylaxis before dental procedures in congenital heart disease: Insights from a nationwide Korean cohort.

Journal of infection and public health
2025

A Paradigm Shift in Congenital Heart Disease: A Scientometric Portrait of the Rise of Computational Intelligence.

Pediatric cardiology
2025

Parallel Transcatheter Valve and Stent Placement with Subsequent Stent Occlusion to Address a Giant Dysfunctional RVOT in a Patient with Repaired Tetralogy of Fallot.

Pediatric cardiology
2025

Late Outcomes After Infant Repair of Tetralogy of Fallot: Do Not "Burn After Reading".

The Annals of thoracic surgery
2026

Interchangeable measure of pulmonary regurgitation in repaired tetralogy of Fallot: Comparison between serial cardiac CT and MRI.

European journal of radiology
2026

Microaxial Flow Pump-Protected Percutaneous Pulmonary Valve Replacement in High-Risk Patients With Severe Left Ventricular Dysfunction.

JACC. Case reports
2026

Transcatheter Pulmonary Valve Replacement in an Extra-Large RVOT Using a Custom-Made Pulmonary Valve.

JACC. Case reports
2025

The analysis reveals novel hub genes and pathways associated with Tetralogy of Fallot.

Frontiers in cardiovascular medicine
2025

Cardiovascular magnetic resonance 4D flow derived aortic and pulmonary wall shear stress in pediatric patients with repaired tetralogy of Fallot.

Frontiers in pediatrics
2026

Right ventricular outflow tract pseudoaneurysm in repaired of Tetralogy of Fallot.

Revista portuguesa de cardiologia : orgao oficial da Sociedade Portuguesa de Cardiologia = Portuguese journal of cardiology : an official journal of the Portuguese Society of Cardiology
2026

High risk and low incidence diseases: Cyanotic critical congenital heart disease.

The American journal of emergency medicine
2026

Early Outcomes of Self-Expandable Versus Balloon-Expandable Valves for Managing Dysfunctional Right Ventricular Outflow Tracts.

Circulation. Cardiovascular interventions
2025

Changes in right ventricular function on three-dimensional speckle tracking echocardiography after transcatheter pulmonary valve replacement.

Quantitative imaging in medicine and surgery
2025

Role of balloon pulmonary valvuloplasty in symptomatic infants with tetralogy of Fallot awaiting intracardiac repair.

International journal of cardiology. Congenital heart disease
2025

Arrhythmias in Congenital Heart Disease: Tetralogy of Fallot.

Cardiac electrophysiology clinics
2025

Surgical Repair of Tetralogy of Fallot and a Large Congenital Diaphragmatic Hernia in a 16-Week-Old Infant With Pentalogy of Cantrell and a Large Omphalocele: A Case Report.

World journal for pediatric &amp; congenital heart surgery
2025

Case Report: a novel missense variant of FGD5 in a family with tetralogy of Fallot.

Frontiers in genetics
2025

Left Atrial Strain and History of Life-Threatening Arrhythmia in Adults with Repaired Tetralogy of Fallot: A Cardiovascular Magnetic Resonance Study.

Pediatric cardiology
2026

First Reported Case of Simultaneous Transjugular Tricuspid Valve-in-Ring and Pulmonary Valve-in-Valve Implantation.

JACC. Case reports
2025

Adult Congenital Heart Disease Complexity Restratification by Computed Tomography and/or Cardiac Magnetic Resonance.

Echocardiography (Mount Kisco, N.Y.)
2025

Evolution of Surgical Management in Tetralogy of Fallot: A Historical and Contemporary Review.

Heart views : the official journal of the Gulf Heart Association
2025

Timing and Mode of Death Following Treatment of Neonatal Symptomatic Tetralogy of Fallot.

Journal of the American Heart Association
2025

MYH6 in Congenital Heart Defects: A Genotype-Phenotype Characterization in a French Cohort.

Pediatric cardiology
2025

Tracheal Compression on Chest X-ray Leading to the Diagnosis of Right Aortic Arch in 22q11.2 Deletion Syndrome.

Cureus
2025

"Tricuspid valved patch" repair with a polytetrafluoroethylene valved conduit for right ventricular outflow reconstruction.

Annals of thoracic surgery short reports
2026

Associations Between Right Ventricular Remodeling, Exercise and Circulating Metabolites in Volume and Pressure Overload States.

JACC. Asia
2025

Association of preoperative Circulating biomarkers with echocardiographic measures of right ventricular strain five years after tetralogy of fallot repair.

The international journal of cardiovascular imaging
2025

Incidentally detected congenital pericardial defect and anomalous great cardiac venous drainage in a patient of tetralogy of Fallot-a case report.

Indian journal of thoracic and cardiovascular surgery
2025

Concurrence of double aortic arch in tetralogy of Fallot with absent pulmonary valve syndrome.

Indian journal of thoracic and cardiovascular surgery
2025

Tetralogy of Fallot: Multimodality Imaging and Key Historical Contributions to Diagnosis and Treatment.

Echocardiography (Mount Kisco, N.Y.)
2025

Pediatric cardiac surgery in Sudan before the 2023 armed conflict: patterns, outcomes, and quality indicators from a single-center experience in a low-resource setting.

BMC pediatrics
2026

Tetralogy of Fallot and craniosynostosis - differential manifestation in a familial case of CHDED syndrome caused by novel pathogenic PRKD1 variant.

Journal of human genetics
2025

Congenital extrahepatic portosystemic shunts (Abernethy malformation): A report of two cases.

The Journal of international medical research
2025

[Agenesis of the left pulmonary artery with hypoplasia of the homolateral lung].

La Revue du praticien
2025

Invasive fungal infection in a neonate with tetralogy of Fallot after repeated right ventricle outflow tract stenting: diagnosis, treatment, monitoring, and therapeutic success.

Postepy w kardiologii interwencyjnej = Advances in interventional cardiology
2025

Drug-Eluting Balloons Versus Conventional Balloon Angioplasty for Peripheral Pulmonary Artery Stenosis in Childhood.

Pediatric cardiology
2025

Association Between G6PD Deficiency and Congenital Heart Disease Incidence and Hospital Outcomes.

Pediatric cardiology
2025

The Hidden Regulators: MicroRNAs in Pediatric Heart Development and Disease.

Journal of clinical medicine
2026

Risk stratification for sudden death in congenital heart disease: bridging evidence, uncertainty, and individual decision-making.

Current opinion in cardiology
Ver todos os 6.874 no EuropePMC

Associações

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Comunidades

Grupos ativos de quem convive com esta doença aqui no Raras

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Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

Ordenadas pelo número de sintomas em comum.

Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Utility of three-dimensional echocardiography for evaluating right ventricular size and function and ventricular myocardial deformation in repaired tetralogy of fallot.
    PloS one· 2026· PMID 41843576mais citado
  2. Genetic Syndromes Do Not Affect Survival but Increase Morbidity in Neonates with Symptomatic Tetralogy of Fallot.
    The Journal of pediatrics· 2026· PMID 41812746mais citado
  3. Optimal Size of Transannular Patches for Tetralogy of Fallot Repair.
    Heart, lung &amp; circulation· 2026· PMID 41807220mais citado
  4. Age-dependent Dynamics of the Electrocardiographic Parameters in Cardiovascular Disease-Free Children.
    medRxiv : the preprint server for health sciences· 2026· PMID 41728320mais citado
  5. Two young athletes with Tetralogy of Fallot-stress echocardiography in therapeutic decision-making: a case report.
    European heart journal. Case reports· 2026· PMID 41728223mais citado
  6. Comment on "Left ventricular quantification in repaired tetralogy of Fallot: two-dimensional echocardiography versus cardiovascular magnetic resonance".
    Int J Cardiovasc Imaging· 2026· PMID 41996048recente
  7. Single-cell omics uncovers novel pathological mechanisms and therapeutic targets for congenital heart diseases: insights from integrated intercellular communication analysis.
    Stem Cell Res Ther· 2026· PMID 41992274recente
  8. "Correlation of right ventricular volumes and ejection fraction by 3D echo and cardiac MRI in postoperative tetralogy of Fallot patients with free pulmonary valve regurgitation.".
    Indian Heart J· 2026· PMID 41985780recente
  9. Spectrum of Congenital Heart Diseases in Iraqi Patients.
    Eurasian J Med· 2026· PMID 41984424recente
  10. MorphiNet: A Graph Subdivision Network for Adaptive Bi-ventricle Surface Reconstruction.
    IEEE Trans Med Imaging· 2026· PMID 41979944recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:3303(Orphanet)
  2. OMIM OMIM:187500(OMIM)
  3. MONDO:0008542(MONDO)
  4. GARD:2245(GARD (NIH))
  5. Variantes catalogadas(ClinVar)
  6. Busca completa no PubMed(PubMed)
  7. Artigo Wikipedia(Wikipedia)
  8. Q1126831(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Tetralogia de Fallot
Compêndio · Raras BR

Tetralogia de Fallot

ORPHA:3303 · MONDO:0008542
Prevalência
Unknown
Herança
Autosomal dominant, Multigenic/multifactorial
CID-10
Q21.3 · Tetralogia de Fallot
CID-11
Ensaios
20 ativos
Início
Antenatal, Neonatal
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C0039685
EuropePMC
Wikidata
Wikipedia
Papers 10a
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