Raras
Buscar doenças, sintomas, genes...
Comunicação interatrial
ORPHA:1478CID-10 · Q21.1CID-11 · LA8E.YDOENÇA RARA

A comunicação interauricular é uma malformação congênita caracterizada pela comunicação entre as câmaras atriais do coração.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

A comunicação interauricular é uma malformação congênita caracterizada pela comunicação entre as câmaras atriais do coração.

Publicações científicas
478 artigos
Último publicado: 2026 Feb

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-5 / 10 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Europe
Início
Adolescent
+ adult, childhood, elderly
🏥
SUS: Cobertura mínimaScore: 15%
CID-10: Q21.1
🇧🇷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
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Sinais e sintomas

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

Partes do corpo afetadas

❤️
Coração
48 sintomas
🫁
Pulmão
10 sintomas
🦴
Ossos e articulações
2 sintomas
📏
Crescimento
1 sintomas
🧠
Neurológico
1 sintomas
🛡️
Imunológico
1 sintomas

+ 27 sintomas em outras categorias

Características mais comuns

90%prev.
Defeito do septo atrial tipo ostium secundum
Muito frequente (99-80%)
90%prev.
Defeito do septo atrial
Muito frequente (99-80%)
55%prev.
Fibrilação atrial
Frequente (79-30%)
55%prev.
Sopro cardíaco sistólico
Frequente (79-30%)
17%prev.
Bloqueio do ramo direito
Ocasional (29-5%)
17%prev.
Dilatação ventricular direita
Ocasional (29-5%)
91sintomas
Muito frequente (2)
Frequente (2)
Ocasional (13)
Muito raro (3)
Sem dados (71)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 91 características clínicas mais associadas, ordenadas por frequência.

Defeito do septo atrial tipo ostium secundumSecundum atrial septal defect
Muito frequente (99-80%)90%
Defeito do septo atrialAtrial septal defect
Muito frequente (99-80%)90%
Fibrilação atrialAtrial fibrillation
Frequente (79-30%)55%
Sopro cardíaco sistólicoSystolic heart murmur
Frequente (79-30%)55%
Bloqueio do ramo direitoRight bundle branch block
Ocasional (29-5%)17%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico478PubMed
Últimos 10 anos127publicações
Pico202119 papers
Linha do tempo
2026Hoje · 2026🧪 2018Primeiro ensaio clínico📈 2021Ano de pico
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

8 genes identificados com associação a esta condição. Padrão de herança: Autosomal dominant, Not applicable.

MYH6Myosin-6Disease-causing germline mutation(s) inTolerante
FUNÇÃO

Muscle contraction

LOCALIZAÇÃO

Cytoplasm, myofibril

VIAS BIOLÓGICAS (1)
Striated Muscle Contraction
MECANISMO DE DOENÇA

Atrial septal defect 3

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.

EXPRESSÃO TECIDUAL(Tecido-específico)
Coração - Átrio
4609.5 TPM
Coração - Ventrículo esquerdo
334.6 TPM
Músculo esquelético
53.3 TPM
Pituitária
5.7 TPM
Próstata
0.7 TPM
OUTRAS DOENÇAS (7)
atrial septal defect 3dilated cardiomyopathy 1EEhypertrophic cardiomyopathy 14atrial septal defect, ostium secundum type
HGNC:7576UniProt:P13533
TBX20T-box transcription factor TBX20Disease-causing germline mutation(s) inAltamente restrito
FUNÇÃO

Acts as a transcriptional activator and repressor required for cardiac development and may have key roles in the maintenance of functional and structural phenotypes in adult heart

LOCALIZAÇÃO

Nucleus

VIAS BIOLÓGICAS (1)
Cardiogenesis
MECANISMO DE DOENÇA

Atrial septal defect 4

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 defects in septation, chamber growth and valvulogenesis. The disease is not associated with defects in the cardiac conduction system or with non-cardiac abnormalities.

VIAS REACTOME (1)
EXPRESSÃO TECIDUAL(Tecido-específico)
Coração - Átrio
20.7 TPM
Bladder
9.8 TPM
Coração - Ventrículo esquerdo
5.4 TPM
Artéria coronária
4.0 TPM
Pituitária
0.9 TPM
OUTRAS DOENÇAS (3)
atrial septal defect 4atrial septal defect, ostium secundum typeleft ventricular noncompaction
HGNC:11598UniProt:Q9UMR3
GATA4Transcription factor GATA-4Disease-causing germline mutation(s) 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
TLL1Tolloid-like protein 1Disease-causing germline mutation(s) inAltamente restrito
FUNÇÃO

Protease which processes procollagen C-propeptides, such as chordin, pro-biglycan and pro-lysyl oxidase. Required for the embryonic development. Predominant protease, which in the development, influences dorsal-ventral patterning and skeletogenesis

LOCALIZAÇÃO

Secreted

VIAS BIOLÓGICAS (4)
Degradation of the extracellular matrixCollagen biosynthesis and modifying enzymesCrosslinking of collagen fibrilsAnchoring fibril formation
MECANISMO DE DOENÇA

Atrial septal defect 6

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.

EXPRESSÃO TECIDUAL(Tecido-específico)
Cérebro - Hemisfério cerebelar
13.9 TPM
Cerebelo
11.4 TPM
Tecido adiposo
5.5 TPM
Mama
4.7 TPM
Adipose Visceral Omentum
4.2 TPM
INTERAÇÕES PROTEICAS (2)
OUTRAS DOENÇAS (3)
atrial septal defect 6atrial septal defect, ostium primum typeatrial septal defect, ostium secundum type
HGNC:11843UniProt:O43897
NKX2-5Homeobox protein Nkx-2.5Disease-causing germline mutation(s) 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
ACTC1Actin, alpha cardiac muscle 1Disease-causing germline mutation(s) inAltamente restrito
FUNÇÃO

Actins are highly conserved proteins that are involved in various types of cell motility and are ubiquitously expressed in all eukaryotic cells

LOCALIZAÇÃO

Cytoplasm, cytoskeleton

VIAS BIOLÓGICAS (5)
Regulation of CDH1 FunctionFormation of the dystrophin-glycoprotein complex (DGC)Striated Muscle ContractionRHOB GTPase cycleRHOA GTPase cycle
MECANISMO DE DOENÇA

Cardiomyopathy, dilated, 1R

A disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.

OUTRAS DOENÇAS (7)
atrial septal defect 5dilated cardiomyopathy 1Rhypertrophic cardiomyopathy 11hypertrophic cardiomyopathy
HGNC:143UniProt:P68032
GATA6Transcription factor GATA-6Disease-causing germline mutation(s) 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
CITED2Cbp/p300-interacting transactivator 2Disease-causing germline mutation(s) 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

Variantes genéticas (ClinVar)

921 variantes patogênicas registradas no ClinVar.

🧬 MYH6: NM_002471.4(MYH6):c.4284G>C (p.Glu1428Asp) ()
🧬 MYH6: NM_002471.4(MYH6):c.2349del (p.Ser784fs) ()
🧬 MYH6: NM_002471.4(MYH6):c.5417C>A (p.Ala1806Asp) ()
🧬 MYH6: NM_002471.4(MYH6):c.2816C>T (p.Thr939Ile) ()
🧬 MYH6: NM_002471.4(MYH6):c.2309G>T (p.Gly770Val) ()
Ver todas no ClinVar

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Comunicação interatrial

🗺️

Selecione um estado ou use sua localização para ver resultados.

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Publicações mais relevantes

📖Melhor nível de evidência: Revisão
Timeline de publicações
128 papers (10 anos)
#1

Immediate Reversal of Hypoxemia With PFO Closure in Carcinoid Heart Disease and Platypnea-Orthodeoxia Syndrome.

JACC. Case reports2026 Jan 06

Intracardiac shunting via a patent foramen ovale (PFO) is an under-recognized cause of refractory hypoxemia. A 48-year-old woman with metastatic gastric neuroendocrine tumor involving the tricuspid valve presented with recurrent shortness of breath and severe hypoxemia refractory to supplemental oxygen. Computed tomography angiography ruled out pulmonary embolism. Transthoracic echocardiography with a bubble study demonstrated an interatrial communication suggesting a PFO. Right heart catheterization showed bidirectional shunting. Intracardiac echocardiography confirmed the presence of a large PFO. The patient underwent percutaneous PFO closure with an Amplatzer PFO Occluder, resulting in marked improvement in oxygenation and resolution of respiratory failure. This case underscores the importance of considering intracardiac shunts in instances of unexplained refractory hypoxemia and demonstrates the efficacy of percutaneous PFO closure in treating this phenomenon. Consider a PFO in persistent hypoxemia despite a negative pulmonary work-up. Timely percutaneous closure may lead to rapid, sustained improvement.

#2

The Role of Serial Fetal Echocardiography in Postnatal Surgical Decision-Making for Borderline Left Ventricle: A Case Report.

Pediatric reports2026 Feb 02

Background: Borderline left ventricle represents a heterogeneous spectrum of congenital heart disease for which accurate prediction of suitability for biventricular versus univentricular circulation is often difficult. Serial fetal echocardiography may provide dynamic information to support postnatal decision-making. Case Presentation: We report the case of a fetus diagnosed at 32 weeks' gestation with a borderline left ventricle, ventricular disproportion, hypoplastic left-sided structures, ductal-dependent systemic circulation, and a non-restrictive ostium secundum atrial septal defect. Serial fetal echocardiographic evaluations demonstrated stable left ventricular dimensions, preserved systolic function, impaired diastolic relaxation, and absence of endomyocardial fibroelastosis. Postnatal echocardiography confirmed hypoplastic aortic arch and coarctation. Following multidisciplinary evaluation, a biventricular repair strategy was selected. At 14 days of life, the patient underwent aortic arch reconstruction and partial atrial septal defect closure with preservation of a small therapeutic interatrial communication. Postoperative evolution was favorable, with progressive left ventricular growth and preserved function. At 2-year follow-up, echocardiography showed normalized mitral and aortic valve z-scores, good left ventricular systolic performance, and no evidence of myocardial fibrosis. Conclusions: This case highlights the value of serial fetal echocardiography in guiding individualized management of borderline left ventricle. Careful assessment of ventricular function and atrial septal physiology may support selection of a biventricular strategy in selected patients and contribute to favorable mid-term outcomes. Atrioventricular septal defect (AVSD) is categorized into 3 anatomical types: complete, partial, and transitional (or intermediate). Complete AVSDs feature both an atrial and a ventricular septal defect, as well as a common AV valve. Partial AVSDs contain an interatrial communication (primum atrial septal defect) but lack an interventricular communication. Transitional AVSDs are a variant of complete AVSDs, characterized by an interatrial communication and a restrictive interventricular communication. Complete AVSDs can be further classified using the Rastelli classification into types A, B, and C, based on the anatomical variations of the superior bridging leaflet and its attachments to the ventricular septum. Additionally, AVSDs can be categorized as balanced or unbalanced. A balanced AVSD features a single, large AV valve that opens nearly equally into both ventricles, resulting in similarly sized ventricles. Conversely, an unbalanced AVSD has the common valve displaced toward 1 ventricle, resulting in that ventricle being significantly larger. This distinction influences whether a biventricular or single-ventricle repair is recommended. Pathophysiology and clinical presentation vary considerably by AVSD subtype. Mortality approaches 50% within the first year of life among children with unrepaired complete AVSD, most commonly due to congestive heart failure and pneumonia. Survors develop irreversible pulmonary arterial hypertension; unfortunately, long-term survival is low. Surgical management has evolved significantly, ranging from palliative pulmonary artery banding to complete AVSD repair. Early detection through echocardiography, appropriate preoperative care, and meticulous management of the AV valve morphology are essential to ensure optimal long-term survival and functional outcomes in this group.

#3

Hybrid Versus Percutaneous Left Atrial Decompression in Infants With Hypoplastic Left Heart Variants and an Intact or Highly Restrictive Atrial Septum: A Multicenter PICES Study.

Circulation. Cardiovascular interventions2025 Mar

Neonates with hypoplastic left heart syndrome variants with an intact or highly restrictive atrial septum (HLH-IAS) require immediate postnatal intervention to survive. Emergent left atrial decompression (LAD) via a percutaneous or hybrid approach is standard, but the comparative effectiveness and outcomes of these approaches remain underexplored. A multicenter retrospective analysis of all neonates with HLH-IAS who underwent LAD in the first 36 hours of life from January 2009 to March 2020 at 14 North American congenital cardiac programs was conducted. Patient and procedural characteristics and patient outcomes were compared between hybrid and percutaneous LAD. Among 128 patients with HLH-IAS, 105 (80%) underwent percutaneous LAD and 23 (17%) hybrid LAD. No significant differences were observed in preprocedural characteristics such as birth weight (3.1±0.6 versus 3.2±0.6 kg; P=0.453), presence of a baseline interatrial communication (66% versus 61%; P=0.659), interatrial communication gradient (14.4±6.9 versus 12.7±4.6 mm Hg; P=0.456), or lowest pH (7.13±0.14 versus 7.09±0.15; P=0.193). Hybrid LAD resulted in a shorter time from birth to procedure (120 versus 52 minutes; P=0.005) and to atrial septal defect creation (55 versus 29 minutes; P=0.002). All hybrid LAD were technically successful, although 11 (10%) percutaneous LAD were unsuccessful with 7 requiring conversion to surgical septectomy. There was a trend toward fewer serious adverse events with hybrid LAD (36% versus 17%; P=0.141). Most patients (67%) who underwent hybrid LAD had simultaneous pulmonary artery band placement and 21% of patients with percutaneous LAD required reoperation for pulmonary artery band. Long-term survival rates were comparably low between groups, with a 30% transplant-free survival at a median 8-month follow-up. In patients with HLH-IAS, hybrid LAD allows for faster atrial septal defect creation, has a higher technical success rate, and potentially fewer procedural serious adverse events compared with percutaneous LAD, alongside facilitating simultaneous pulmonary artery band. Our findings underscore the poor prognosis for patients with HLH-IAS despite intervention, highlighting the need for better treatment strategies.

#4

Biventricular Interatrial Shunt With Desaturation After Atrial Septal Defect Repair.

JACC. Case reports2025 Nov 19

Patients after atrial septal defect (ASD) repair with residual shunt, elevated pulmonary arterial pressure or arrhythmias, and those repaired at adult age should be followed on a regular basis. This case describes a 43-year-old female with previous ASD repair, who presented with breathlessness, tiredness, and evidence of desaturation on exercise with a mildly positive bubble echo. While local investigations were inconclusive, diagnostic tests performed at our center revealed an inferior sinus venosus ASD-type defect with bidirectional flow, even though neither right-heart dilatation, pulmonary hypertension nor hemodynamically significant shunt was identified. The patient was offered surgical repair of the residual interatrial communication to give the patient symptomatic benefit. This case demonstrates a rare presentation of a post-ASD repair patient with bidirectional shunt without right-heart overload and no pulmonary hypertension. Patients with repaired congenital defects should be followed up by specialized congenital services at reasonable interval based on the lesion and the results of the repair.

#5

A novel approach to preparation of neonates with transposition of great arteries with intact ventricular septum for successful arterial switch operation: Combined balloon atrial septostomy and patent ductus arteriosus stenting.

Medical journal, Armed Forces India2025

The corrective surgery of transposition of great arteries with intact interventricular septum (TGA-IVS) is an arterial switch operation (ASO), and this requires a neonate with hemodynamic stability, absence of infection, and left ventricle (LV) prepared to take the afterload. ASO is carried out at 2-3 weeks of life at our center thus making stabilization procedures like balloon atrial septostomy (BAS) essential. We hypothesized that in neonates with TGA-IVS with restrictive interatrial communication requiring BAS, simultaneous stenting of the patent ductus arteriosus (PDA) will maintain an adequate left ventricular load so that the LV is prepared to undergo ASO. We present our experience of combined BAS with PDA stenting as a first-stage procedure prior to ASO and compare the data with those who did not undergo PDA stenting. A single-center, retrospective study of neonates with TGA-IVS with restrictive interatrial communication who underwent BAS with PDA stenting as stage 1 procedure was done. These neonates underwent ASO in stage 2. Echocardiographic assessment of the LV volumes, mass, geometry, and free wall thickness was performed prior to stage 1 and stage 2 and compared. After surgery and the postoperative clinical, hemodynamic and morbidity parameters were analyzed. Five neonates were included in the BAS with PDA stenting group and six patients were included in the non-PDA stenting group controls. Study neonates after stage I procedure had favorable LV geometry with significant improvement in LV mass from 36.7 ± 2.6 to 56.3 ± 3.1 g/m2 (p < 0.0001), posterior wall thickness in systole from 0.49 ± 0.08 mm to 0.54 + 0.08 mm (p = 0.002), LV end-diastolic volume from 14.7 + 7.4 ml to 24.2 + 8.6 ml (p = 0.001), good ejection fraction, and favorable LV geometry. A "limited" BAS was associated with consistent saturations above 84%. All patients underwent ASO subsequently with favorable outcomes to discharge. The control patients without a PDA stent had no significant changes in LV mass after BAS alone and had longer requirement of postoperative ventilation; one patient required extracorporeal membrane oxygenation. Combined BAS with PDA stenting is a safe and effective stage 1 procedure to pretrain the LV in TGA-IVS while simultaneously maintaining adequate saturations till ASO.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC159 artigos no totalmostrando 126

2026

Immediate Reversal of Hypoxemia With PFO Closure in Carcinoid Heart Disease and Platypnea-Orthodeoxia Syndrome.

JACC. Case reports
2026

The Role of Serial Fetal Echocardiography in Postnatal Surgical Decision-Making for Borderline Left Ventricle: A Case Report.

Pediatric reports
2025

Biventricular Interatrial Shunt With Desaturation After Atrial Septal Defect Repair.

JACC. Case reports
2025

A novel approach to preparation of neonates with transposition of great arteries with intact ventricular septum for successful arterial switch operation: Combined balloon atrial septostomy and patent ductus arteriosus stenting.

Medical journal, Armed Forces India
2025

A Rare Levoatrial Cardinal Vein with an Interatrial Communication.

Anatolian journal of cardiology
2025

A large atrial septal aneurysm presenting with severe arrhythmias: A case report.

International journal of surgery case reports
2025

Multimodal Imaging to Guide Stent Placement in a Patient After Fontan Palliation With Restrictive Interatrial Communication.

JACC. Case reports
2025

Restrictive Atrial Communication in Tricuspid Atresia With Minimal Duct-Dependent Pulmonary Circulation in a Newborn.

JACC. Case reports
2025

Left atrial pressure normalisation by graded radiofrequency atrial septostomy in heart failure with preserved ejection fraction: a single-arm pilot study.

Heart (British Cardiac Society)
2025

'One Stop' Therapy has a Satisfying Performance on AF Patients with Interatrial Communication: Evidence from Pooled Clinical Experience.

Reviews in cardiovascular medicine
2025

Hybrid Versus Percutaneous Left Atrial Decompression in Infants With Hypoplastic Left Heart Variants and an Intact or Highly Restrictive Atrial Septum: A Multicenter PICES Study.

Circulation. Cardiovascular interventions
2025

Unusual presentation of anomalous origin of the right pulmonary artery from the ascending aorta: case report.

The Egyptian heart journal : (EHJ) : official bulletin of the Egyptian Society of Cardiology
2025

The impact of a residual atrial communication in patients undergoing complete repair for tetralogy of Fallot: A propensity score-matched analysis.

The Journal of thoracic and cardiovascular surgery
2023

Feasibility, Safety, and Efficacy of the Atrial Flow Regulator in Pediatric Patients: A Single-Center Experience.

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

Interatrial Communications: Prevalence and Subtypes in 12,385 Newborns-a Copenhagen Baby Heart Study.

Pediatric cardiology
2024

Virtual reality modelling for planning of percutaneous first step palliation in a newborn with heterotaxy syndrome.

Cardiology in the young
2024

Atrial Septal Defect vs Ventricular Septal Defect: Getting the Right Mix in Transposition of the Great Arteries.

Cureus
2024

Predicting Long-Term Childhood Survival of Newborns with Congenital Heart Defects: A Population-Based, Prospective Cohort Study (EPICARD).

Journal of clinical medicine
2024

Sinus venosus atrial septal defect presenting with platypnea-orthodeoxia syndrome in the setting of COVID-19 infection.

Cardiology in the young
2023

Oral manifestation in a pediatric patient with pentasomy x. A case report.

Revista cientifica odontologica (Universidad Cientifica del Sur)
2024

Endoleak in covered CP stent causes procedural failure during transcatheter closure of sinus venosus defects.

Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography &amp; Interventions
2024

Surgical Repair of a Coronary Sinus Atrial Septal Defect in an Elderly Patient.

Annals of thoracic surgery short reports
2023

A rare unreported complication following transcatheter correction of sinus venosus defect.

Annals of pediatric cardiology
2023

Outcomes After Hybrid Palliation for Infants With Critical Left Heart Obstruction.

Journal of the American College of Cardiology
2023

Platypnea-Orthodeoxia Syndrome: Two Case Reports.

Cureus
2023

Congenital anomalous drainage of the inferior vena cava and hepatic veins into the left atrium associated with long QT syndrome: Multimodality imaging, pitfalls, and caveats.

Echocardiography (Mount Kisco, N.Y.)
2023

A clinical case of successful palliative endovascular treatment of a patient with a single ventricle, mitral valve atresia, an intact atrial septum and persistent cardinal vein.

The Egyptian heart journal : (EHJ) : official bulletin of the Egyptian Society of Cardiology
2023

Hybrid palliation versus nonhybrid management for a multi-institutional cohort of infants with critical left heart obstruction.

The Journal of thoracic and cardiovascular surgery
2023

Atrial Septal Defect Associated With Right Bundle Branch Block Masking Brugada Syndrome.

JACC. Case reports
2023

Unusual Association of Diamond-Blackfan Anemia and Severe Sinus Bradycardia in a Six-Month-Old White Infant: A Case Report and Literature Review.

Medicina (Kaunas, Lithuania)
2022

Patent Foramen Ovale-Induced Platypnea-Orthodeoxia Syndrome: A Case Report and Literature Review.

Cureus
2023

Harlequin effect and central veno-arterial extracorporeal life support.

Cardiology in the young
2023

A novel algorithm for classification of interatrial communications within the oval fossa in the newborn.

Cardiology in the young
2023

Device-based percutaneous treatments to decompress the left atrium in heart failure with preserved ejection fraction.

Heart failure reviews
2022

Is combined use of radiofrequency ablation and balloon dilation the future of interatrial communications?

Expert review of cardiovascular therapy
2022

The First Successful Transcatheter Closure of an Inferior Sinus Venosus Defect with Anomalous Drainage of the Right Lower Pulmonary Vein Using Bare and Covered Stents: A Single-Case Report.

Case reports in cardiology
2022

Percutaneous transhepatic and transseptal dilatation of a surgically implanted Melody® Valve in the mitral position in a 2-year-old child.

Annals of pediatric cardiology
2022

Transcatheter Palliation of a Young Infant With Obstructed Supracardiac Total Anomalous Pulmonary Venous Connection.

Cureus
2022

Intraoperative Collateral Drainage Evaluation Before Superior Caval Interruption in Partial Anomalous Pulmonary Venous Connection Repair.

Innovations (Philadelphia, Pa.)
2022

Evolving Approach in Hypoplastic Left Heart Syndrome With Restrictive and Intact Septum.

World journal for pediatric &amp; congenital heart surgery
2022

Lutembacher Syndrome with Sinus Venosus-Type Interatrial Communication: An Educational Presentation.

Brazilian journal of cardiovascular surgery
2022

Modern advances regarding interatrial communication in congenital heart defects.

Journal of cardiac surgery
2021

Ebstein Anomaly and Right Aortic Arch in Patient with Charge Syndrome.

Medicina (Kaunas, Lithuania)
2021

A rare type of interatrial communication: levoatrial cardinal vein.

European heart journal. Cardiovascular Imaging
2022

Does interatrial communication affect post-operative course of children undergoing tetralogy of Fallot repair? Single centre retrospective cohort study: propensity score matching.

Cardiology in the young
2021

Incidental diagnosis of a large cardiac thrombus swinging through an interatrial communication in a COVID-19 patient: Case report and literature review.

Annals of medicine and surgery (2012)
2021

Platypnoea-orthodeoxia syndrome due to a right-to-left interatrial shunt following pneumonectomy.

BMJ case reports
2021

Maternal hyperoxygenation in congenital heart disease.

Translational pediatrics
2021

Surgical Management of Three Dogs with an Interatrial Communication and Atrioventricular Valve Abnormalities.

CASE (Philadelphia, Pa.)
2021

Coronary Artery Fistula: Association between Pathway Patterns, Clinical Features and Congenital Heart Disease.

Arquivos brasileiros de cardiologia
2021

Cost-Effectiveness Analysis of Percutaneous Patent Foramen Ovale Closure Preventing Secondary Ischemic Stroke in Japan.

Journal of stroke and cerebrovascular diseases : the official journal of National Stroke Association
2021

Reliability of Fetal Echocardiography in Predicting Postnatal Critical Hypoxia in Patients with Transposition of Great Arteries and Intact Ventricular Septum.

Pediatric cardiology
2021

TARP syndrome associated with renal malformation and optic nerve atrophy.

BMJ case reports
2021

A case report of an elderly male with isolated coronary sinus atrial septal defect.

European heart journal. Case reports
2021

Novel TNIP2 and TRAF2 Variants Are Implicated in the Pathogenesis of Pulmonary Arterial Hypertension.

Frontiers in medicine
2021

Transcatheter creation of a pulmonary artery to left atrial fenestration in a failing Fontan circulation using the Atrial Flow Regulator (AFR).

Cardiology in the young
2021

Late survival in Ellis-van Creveld syndrome with common single atrium.

BMJ case reports
2021

Does persistent (patent) foramen ovale closure reduce the risk of recurrent decompression sickness in scuba divers?

Diving and hyperbaric medicine
2021

Patent Foramen Ovale Closure among Patients with Hypercoagulable States Maintained on Antithrombotic Therapy.

Cardiology
2021

Newly-found channels in the interatrial septum of the heart by dissection, histologic evaluation, and three-dimensional microcomputed tomography.

PloS one
2021

Safety of Transseptal Puncture for Access to the Left Atrium in Infants and Children.

Pediatric cardiology
2021

Para-aortic channel in congenital heart disease: computed tomography and transoesophageal echography depiction of an undescribed type of interatrial communication.

European heart journal
2022

Posture Dependent Hypoxia Following Lobectomy: The Achilles Tendon of the Lung Surgeon?

Journal of investigative surgery : the official journal of the Academy of Surgical Research
2020

A rare case of total anomalous pulmonary venous connection with intact interatrial septum.

Indian journal of thoracic and cardiovascular surgery
2020

Outcomes of Atrioseptostomy with Stenting in Patients with Pulmonary Arterial Hypertension from a Large Single-Institution Cohort.

Diagnostics (Basel, Switzerland)
2020

Identification and Morphogenesis of Vestibular Atrial Septal Defects.

Journal of cardiovascular development and disease
2020

Percutaneous closure of an atrial septal defect in a patient with Ebstein anomaly and right-to-left shunt.

Revista portuguesa de cardiologia
2020

Veno-venous Extracorporeal Membrane Oxygenation for Right Ventricular Failure with Atrial Septostomy After Corrective Repair of Tetralogy of Fallot.

International heart journal
2020

Migration and Surgical Retrieval of an Amplatzer Septal Occluder into Abdominal Aorta.

Annals of vascular surgery
2020

Atrial septum stenting in a foetus with hypoplastic left heart syndrome and restrictive foramen ovale: an alternative to emergency atrioseptectomy in the newborn-a case report.

European heart journal. Case reports
2020

Graded balloon atrial septostomy for palliation of congenital pulmonary hypertension in a dog: A case report.

Journal of veterinary internal medicine
2019

Tetralogy of Fallot With Interatrial Communication of the Inferior Sinus Venosus Type: A Rare Association Causing Post-Operative Desaturation.

World journal for pediatric &amp; congenital heart surgery
2019

[Physiological and paraphysiological echocardiographic findings in neonatal and pediatric age].

Giornale italiano di cardiologia (2006)
2020

Prevalence and Outcomes of Balloon Atrial Septostomy in Neonates With Transposition of Great Arteries.

Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies
2019

Atrial septal defects, supravalvular aortic stenosis and syndromes predisposing to aneurysm of large vessels.

Acta bio-medica : Atenei Parmensis
2019

Use of virtual reality for pre-surgical planning in separation of conjoined twins: A case report.

Proceedings of the Institution of Mechanical Engineers. Part H, Journal of engineering in medicine
2019

Third time lucky: challenging secundum atrial septal defect.

Cardiology in the young
2020

The morphogenesis and associated anomalous pulmonary venous drainage in sinus venosus defect.

Pediatrics and neonatology
2019

Three-dimensional echocardiographic imaging of a double-barrel interatrial communication.

Turk Kardiyoloji Dernegi arsivi : Turk Kardiyoloji Derneginin yayin organidir
2020

Pulmonary haemodynamic effects of interatrial shunt in heart failure with preserved ejection fraction: a preclinical study.

EuroIntervention : journal of EuroPCR in collaboration with the Working Group on Interventional Cardiology of the European Society of Cardiology
2019

Sinus Venosus Interatrial Communication, Anomalous Pulmonary Venous Return, Pulmonary Artery Aneurysm With Left Main Compression: Complex Case With Complex Management.

The Canadian journal of cardiology
2019

Unroofed coronary sinus: An unusual interatrial communication and a rare childhood entity.

Annals of pediatric cardiology
2018

Current management aspects in adult congenital heart disease: non-surgical closure of patent foramen ovale.

Cardiovascular diagnosis and therapy
2019

Innovative 2-Step Management Strategy Utilizing EXIT Procedure for a Fetus With Hypoplastic Left Heart Syndrome and Intact Atrial Septum.

Mayo Clinic proceedings
2019

Closure of foramen ovale triggered by injury to tunnel surfaces of septum primum and secundum.

Journal of interventional cardiac electrophysiology : an international journal of arrhythmias and pacing
2019

Total Anomalous Pulmonary Venous Connection With an Intact Interatrial Septum.

The Annals of thoracic surgery
2018

Platypnea-Orthodeoxia: A Case of Unexplained Hypoxia.

WMJ : official publication of the State Medical Society of Wisconsin
2018

Successful Transcatheter Atrial Septal Defect Closure Prior to Coronary Artery Bypass Grafting Using Anti-Congestive Therapies and Intraaortic Balloon Pumping in a Patient with Severe Ischemic Cardiomyopathy and Triple-Vessel Coronary Artery Disease.

International heart journal
2018

Stable Interatrial Communication Combining Balloon Atrial Septostomy and Radiofrequency Catheter Ablation.

Journal of the American College of Cardiology
2018

Creation of a restrictive atrial left-to-right shunt: a novel treatment for heart failure.

Heart failure reviews
2018

Right heart dilatation in a fetus with an abnormal foramen ovale valve: an indicator of interatrial communication restriction.

Medical ultrasonography
2018

[Surgical treatment of macroglossia in Beckwith-Wiedemann syndrome: case report].

Archivos argentinos de pediatria
2018

Genetic Syndromes Associated with Congenital Cardiac Defects and Ophthalmologic Changes - Systematization for Diagnosis in the Clinical Practice.

Arquivos brasileiros de cardiologia
2018

Agitated saline contrast echocardiography reveals a systemic-to-pulmonary venous shunt.

Echocardiography (Mount Kisco, N.Y.)
2019

Intracardiac echocardiography for percutaneous patent foramen ovale and atrial septal defect occlusion.

Herz
2018

Usefulness of cutting balloon angioplasty for the treatment of congenital heart defects.

Cardiology journal
2017

Feasibility of multiplane microtransoesophageal echocardiographic guidance in structural heart disease transcatheter interventions in adults.

Netherlands heart journal : monthly journal of the Netherlands Society of Cardiology and the Netherlands Heart Foundation
2017

Interatrial shunt devices for heart failure with normal ejection fraction: a technology update.

Medical devices (Auckland, N.Z.)
2017

Epidemiology of Myocardial Infarction Caused by Presumed Paradoxical Embolism via a Patent Foramen Ovale.

Circulation journal : official journal of the Japanese Circulation Society
2017

Intracardiac versus transesophageal echocardiography to guide transcatheter closure of interatrial communications: Nationwide trend and comparative analysis.

Journal of interventional cardiology
2017

Surgical Strategy Toward Biventricular Repair for Severe Ebstein Anomaly in Neonates and Infancy.

The Annals of thoracic surgery
2017

Relationship between interatrial communication, ductus arteriosus, and pulmonary flow patterns in fetuses with transposition of the great arteries: prediction of neonatal desaturation.

Cardiology in the young
2017

Is transnasal TEE imaging a viable alternative to conventional TEE during structural cardiac interventions to avoid general anaesthesia? A pilot comparison study of image quality.

Echo research and practice
2017

Ebstein Anomaly With QRS Fragmentation on Electrocardiogram.

Journal of investigative medicine high impact case reports
2017

Progressive dyspnea and signs of right heart dysfunction.

Echocardiography (Mount Kisco, N.Y.)
2017

'End-stage' heart failure therapy: potential lessons from congenital heart disease: from pulmonary artery banding and interatrial communication to parallel circulation.

Heart (British Cardiac Society)
2017

Development of the atrial septum in relation to postnatal anatomy and interatrial communications.

Heart (British Cardiac Society)
2016

Sinus Venosus Atrial Septal Defect Complicated by Eisenmenger Syndrome and the Role of Vasodilator Therapy.

Case reports in cardiology
2017

Anatomical considerations for the management of a left-sided superior caval vein draining to the roof of the left atrium.

Cardiology in the young
2016

Percutaneous Intervention to Treat Platypnea-Orthodeoxia Syndrome: The Toronto Experience.

JACC. Cardiovascular interventions
2016

Minimally Invasive Periareolar Approach to Unroofed Coronary Sinus Atrial Septal Defect Repair.

The Annals of thoracic surgery
2016

Is oral contraceptive-induced headache dependent on patent foramen ovale? Clinical dynamics, evidence-based hypothesis and possible patient-oriented management.

Medical hypotheses
2016

Evaluation of 60 cases of systemic-pulmonary shunt with cardiopulmonary bypass.

General thoracic and cardiovascular surgery
2016

Left ventricular regression after balloon atrial septostomy in d-transposition of the great arteries.

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
2016

Fetal cardiac interventions: clinical and experimental research.

Postepy w kardiologii interwencyjnej = Advances in interventional cardiology
2017

Understanding the spectrum of sinus venosus interatrial communications.

Cardiology in the young
2016

The comparative role of echocardiography and MRI for identifying critical lesions in patients with single-ventricle physiology, before bidirectional cavopulmonary connection.

Cardiology in the young
2016

Thrombus-in-Transit Following Watchman Device Implantation.

Echocardiography (Mount Kisco, N.Y.)
2016

Minimally invasive repair of fenestrated atrial septal aneurysm.

Journal of visualized surgery
2016

An Unusual Type of Totally Anomalous Pulmonary Venous Connection to the Superior Cavoatrial Junction.

World journal for pediatric &amp; congenital heart surgery
2016

Impact of Interatrial Communication on Left Ventricle Performance in Patients with Significant Post-tricuspid Shunt.

Pediatric cardiology
2015

Implantable atrial flow regulator for severe, irreversible pulmonary arterial hypertension.

EuroIntervention : journal of EuroPCR in collaboration with the Working Group on Interventional Cardiology of the European Society of Cardiology
2015

Transient cerebral ischemia in an elderly patient with patent foramen ovale and atrial septal aneurysm.

Clinical interventions in aging
2015

Impact of novel balloon catheter on static balloon atrial septostomy with double balloon technique in infants with congenital heart disease.

Circulation journal : official journal of the Japanese Circulation Society
2016

Inferior and right-sided juxtaposition of the left atrial appendage with an unexpected type of inter-atrial communication.

Cardiology in the young
2015

Delayed perforation of a transcatheter patent foramen ovale occluder 10 years after implantation.

The Annals of thoracic surgery
Ver todos os 159 no EuropePMC

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Doenças relacionadas

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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. Immediate Reversal of Hypoxemia With PFO Closure in Carcinoid Heart Disease and Platypnea-Orthodeoxia Syndrome.
    JACC. Case reports· 2026· PMID 41817485mais citado
  2. The Role of Serial Fetal Echocardiography in Postnatal Surgical Decision-Making for Borderline Left Ventricle: A Case Report.
    Pediatric reports· 2026· PMID 41718430mais citado
  3. Hybrid Versus Percutaneous Left Atrial Decompression in Infants With Hypoplastic Left Heart Variants and an Intact or Highly Restrictive Atrial Septum: A Multicenter PICES Study.
    Circulation. Cardiovascular interventions· 2025· PMID 40100953mais citado
  4. Biventricular Interatrial Shunt With Desaturation After Atrial Septal Defect Repair.
    JACC. Case reports· 2025· PMID 41109997mais citado
  5. A novel approach to preparation of neonates with transposition of great arteries with intact ventricular septum for successful arterial switch operation: Combined balloon atrial septostomy and patent ductus arteriosus stenting.
    Medical journal, Armed Forces India· 2025· PMID 41048640mais citado
  6. The role of interatrial stenting in the management of CHD.
    Cardiol Young· 2026· PMID 41914268recente
  7. Atrioventricular Canal Defects.
    · 2026· PMID 32491443recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:1478(Orphanet)
  2. MONDO:0006664(MONDO)
  3. GARD:15018(GARD (NIH))
  4. Variantes catalogadas(ClinVar)
  5. Busca completa no PubMed(PubMed)
  6. Artigo Wikipedia(Wikipedia)
  7. Q757749(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

Comunicação interatrial
Compêndio · Raras BR

Comunicação interatrial

ORPHA:1478 · MONDO:0006664
Prevalência
1-5 / 10 000
Herança
Autosomal dominant, Not applicable
CID-10
Q21.1 · Comunicação interatrial
CID-11
Início
Adolescent, Adult, Childhood, Elderly
Prevalência
0.0 (Europe)
MedGen
UMLS
C0018817
EuropePMC
Wikidata
Wikipedia
Papers 10a
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