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Divertículo cardíaco
ORPHA:1686CID-10 · Q24.8CID-11 · LA88.YDOENÇA RARA

O divertículo cardíaco congênito (DCC) é uma malformação congênita muito rara, caracterizada por um apêndice muscular emergindo do ápice do ventrículo esquerdo, raramente do ventrículo direito ou de ambas as câmaras, com manifestações clínicas que variam de assintomático a colapso hemodinâmico com risco de vida.

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Introdução

O que você precisa saber de cara

📋

O divertículo cardíaco congênito (DCC) é uma malformação congênita muito rara, caracterizada por um apêndice muscular emergindo do ápice do ventrículo esquerdo, raramente do ventrículo direito ou de ambas as câmaras, com manifestações clínicas que variam de assintomático a colapso hemodinâmico com risco de vida.

Publicações científicas
52 artigos
Último publicado: 2026 Mar

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
Infancy
+ neonatal
🏥
SUS: Cobertura mínimaScore: 15%
CID-10: Q24.8
🇧🇷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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Do básico ao detalhe, leia no seu ritmo

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Sinais e sintomas

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

Partes do corpo afetadas

❤️
Coração
21 sintomas
🫃
Digestivo
3 sintomas
🩸
Sangue
1 sintomas
🫁
Pulmão
1 sintomas

+ 16 sintomas em outras categorias

Características mais comuns

55%prev.
Eletrocardiograma anormal
Frequente (79-30%)
17%prev.
Diástase dos retos
Ocasional (29-5%)
17%prev.
Coartação da aorta
Ocasional (29-5%)
17%prev.
Onfalocele
Ocasional (29-5%)
17%prev.
Transposição das grandes artérias
Ocasional (29-5%)
17%prev.
Hipoplasia da artéria pulmonar
Ocasional (29-5%)
42sintomas
Frequente (1)
Ocasional (26)
Muito raro (15)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 42 características clínicas mais associadas, ordenadas por frequência.

Eletrocardiograma anormalAbnormal EKG
Frequente (79-30%)55%
Diástase dos retosDiastasis recti
Ocasional (29-5%)17%
Coartação da aortaCoarctation of aorta
Ocasional (29-5%)17%
OnfaloceleOmphalocele
Ocasional (29-5%)17%
Transposição das grandes artériasTransposition of the great arteries
Ocasional (29-5%)17%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2025
Total histórico52PubMed
Últimos 10 anos16publicações
Pico20233 papers
Linha do tempo
2025Hoje · 2026🧪 2000Primeiro ensaio clínico📈 2023Ano de pico
Publicações por ano (últimos 10 anos)

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Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
·Pré-clínico3
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 3 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Divertículo cardíaco

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

Pesquisa e ensaios clínicos

0 ensaios clínicos encontrados.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
17 papers (10 anos)
#1

Delayed Clinical Onset of Congenital Heart Disease in a Geriatric Macaw (Ara ararauna).

Journal of avian medicine and surgery2025 Dec

An approximately 33-year-old male blue and gold macaw (Ara ararauna) was presented for postmortem examination after dying without premonitory signs at the owner's home. The bird had a recent history of a grade II/VI left-sided systolic murmur, feather-destructive behavior, was overweight, and had bilateral cataracts. Gross and microscopic postmortem examinations revealed a subaortic interventricular septal saccular defect most compatible with a diverticulum intimately associated with a multifocally fibrotic right coronary aortic semilunar cusp, and its distended sinus. The resulting congestive heart disease was morphologically characterized by concentric myocardial hypertrophy of the left ventricle and cardiogenic pulmonary edema. Reports of cardiac congenital disease in birds include ventricular septal defects, congenital aneurysm, persistent truncus arteriosus, aortic hypoplasia, duplicitas cordis, multiplicatis cordis, and ectopia cordis. To the best of the authors' knowledge, this is the first documentation of a subaortic interventricular septal saccular defect in an avian species. This case adds to the knowledge of cardiovascular disease and raises awareness of delayed-onset clinical disease linked with congenital heart anomalies in birds.

#2

Survival after Right Ventricular Diverticulum Caused by Right Ventricular Rupture.

International heart journal2025 May 31

Ventricular diverticula can be divided into congenital and acquired ventricular diverticulum. Cardiac rupture is a rare occurrence of cardiac injury, but the mortality rate is very high. Some cardiac ruptures may form an acquired ventricular diverticulum. We report the case of a 57-year-old man who presented with a right ventricular diverticulum caused by traumatic ventricle rupture.A 57-year-old man was admitted with chest pain and dyspnea after falling from a high place. His condition gradually stabilized after emergency surgery and rescue treatment. One week later, the patient suddenly presented with swelling of the right upper limb. Venous thrombosis in the right upper extremity was detected by vascular color ultrasound. Further pulmonary artery computed tomography angiography (CTA) revealed an upper right pulmonary artery embolism and a diverticulum in the right ventricular wall; no obvious diverticulum or pericardial effusion was found by echocardiography. After a thorough discussion with the cardiac surgeon, anticoagulant therapy was administered and his condition was closely monitored. After 10 days, pulmonary CTA showed that the pulmonary thrombus had disappeared, but the right ventricular diverticulum was more prominent than before; therefore, anticoagulant therapy was stopped, although hemostatic drugs were not administered. The right ventricular diverticulum gradually shrank and healed.Asymptomatic cardiac diverticula may resolve spontaneously with conservative treatment. We learned from this case that the diagnostic value of cardiac CTA or pulmonary artery CTA for occult heart injury might be superior to that of cardiac ultrasound.

#3

A case report of dyspnea that revealed a congenital left cardiac diverticulum of discovery in adulthood with a fatal course.

International journal of surgery case reports2025 Apr

Congenital cardiac diverticula are rare malformations involving the myocardium, the endocardium, and occasionally the pericardium. They have variable presentations and are often incidental findings. A 61-year-old man with no significant history presented worsening exertional dyspnea. Examination revealed obesity but was otherwise unremarkable. Initial tests were normal, but chest radiography showed cardiomegaly and ECG indicated diffuse microvoltage. Echocardiography revealed a large left ventricular diverticulum (7 × 8 cm) with a wide neck (4 cm) and severe dysfunction (EF 30 %). Coronary angiography confirmed normal coronary arteries. Despite intensive care, his condition deteriorated and required mechanical ventilation and vasoactive support. He underwent emergency surgery but succumbed intraoperatively. Although rare, congenital cardiac diverticula can cause severe complications, including heart failure and sudden death. Echocardiography is the key to diagnosis, with CT or MRI providing further characterization. Surgery is the preferred treatment for symptomatic cases, while asymptomatic patients require close follow-up. This case highlights the need for early diagnosis and timely intervention in congenital cardiac diverticula to prevent fatal outcomes.

#4

Comprehensive preoperative evaluation of left ventricular outflow tract diverticulum: Report of a rare case.

Asian journal of surgery2024 Oct
#5

A case series of double-chambered left ventricle detected by cardiovascular magnetic resonance.

European heart journal. Case reports2023 May

Double-chambered left ventricle (DCLV) is a rare congenital condition, and few case reports are mentioned in literature. Entity, clinical course, and prognosis remain unclear. Cardiovascular magnetic resonance (CMR) is often used for characterization of various congenital heart diseases and can be particularly useful for imaging rare phenomena. Three cases of DCLV were detected by CMR within 2 years in our CMR centre with and without associated congenital heart disease or hypertrabecularization. The patients did not suffer from cardiac symptoms despite the presence of premature ventricular complexes in one patient. Diagnosis of DCLV was made based on a first CMR study that was performed in adulthood, although some anatomical suspicion was already raised by previous echocardiography. Double-chambered left ventricle, synonymous with the terminus 'cor triventriculare sinistrum', has been previously perceived as a rare phenomenon compared with double-chambered right ventricle. It has to be distinguished from ventricular aneurysm or cardiac diverticulum and is characterized by an additional contractile septum with normal wall structure that divides the LV cavum into two (rather) same-sized chambers. The prognosis seems to be benign, since there is no restriction in functionality and no increased thrombogenicity until adulthood. Consequently, there is (presumably) no need for a tailored therapy-at least in the cases present here. Accordingly, we recommend follow-up CMR examinations for progress monitoring and recognize CMR's significant role for diagnosis and follow-up of cardiac abnormalities in orphan diseases. Due to its broader availability, we expect further cases of DLVC in the future.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC25 artigos no totalmostrando 16

2025

Delayed Clinical Onset of Congenital Heart Disease in a Geriatric Macaw (Ara ararauna).

Journal of avian medicine and surgery
2025

Survival after Right Ventricular Diverticulum Caused by Right Ventricular Rupture.

International heart journal
2025

A case report of dyspnea that revealed a congenital left cardiac diverticulum of discovery in adulthood with a fatal course.

International journal of surgery case reports
2024

Comprehensive preoperative evaluation of left ventricular outflow tract diverticulum: Report of a rare case.

Asian journal of surgery
2023

A case series of double-chambered left ventricle detected by cardiovascular magnetic resonance.

European heart journal. Case reports
2023

A right ventricular diverticulum with a very special cardiac anomaly.

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
2023

Prenatal and postnatal echocardiographic imaging of a rare congenital cardiac diverticulum.

Prenatal diagnosis
2022

Left Ventricular Diverticulum Mimicking Ventricular Septal Defect During Acute Coronary Syndrome: A Case Report.

Cureus
2022

A complete case of Cantrell's Pentalogy with isolated left ventricular diverticulum.

Cardiology in the young
2021

[Ventricular diverticulum in Cantrell´s pentalogy: rare but not isolated entities].

Archivos peruanos de cardiologia y cirugia cardiovascular
2021

Prenatal diagnosis of fetal right ventricular diverticulum with massive pericardial effusion in one of monochorionic diamniotic twins: a case report with a favorable outcome following in utero pericardiocentesis.

The Journal of international medical research
2018

Multimodality Imaging and Clinical Significance of Congenital Ventricular Outpouchings: Recesses, Diverticula, Aneurysms, Clefts, and Crypts.

Journal of cardiovascular echography
2017

Corrigendum to "Prenatal Diagnosis of Cardiac Diverticulum with Pericardial Effusion in the First Trimester of Pregnancy with Resolution after Early Pericardiocentesis".

Case reports in obstetrics and gynecology
2017

Sudden perinatal death due to rupture of congenital cardiac diverticulum. Pathological findings and medico-legal investigations in malpractice charge.

Legal medicine (Tokyo, Japan)
2016

Isolated congenital cardiac diverticulum originating from the left ventricular apex: Report of a pediatric case.

Annals of pediatric cardiology
2015

Prenatal Diagnosis of Cardiac Diverticulum with Pericardial Effusion in the First Trimester of Pregnancy with Resolution after Early Pericardiocentesis.

Case reports in obstetrics and gynecology
Ver todos os 25 no EuropePMC

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Comunidades

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Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

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. Delayed Clinical Onset of Congenital Heart Disease in a Geriatric Macaw (Ara ararauna).
    Journal of avian medicine and surgery· 2025· PMID 41420804mais citado
  2. Survival after Right Ventricular Diverticulum Caused by Right Ventricular Rupture.
    International heart journal· 2025· PMID 40368765mais citado
  3. A case report of dyspnea that revealed a congenital left cardiac diverticulum of discovery in adulthood with a fatal course.
    International journal of surgery case reports· 2025· PMID 40157060mais citado
  4. Comprehensive preoperative evaluation of left ventricular outflow tract diverticulum: Report of a rare case.
    Asian journal of surgery· 2024· PMID 39097451mais citado
  5. A case series of double-chambered left ventricle detected by cardiovascular magnetic resonance.
    European heart journal. Case reports· 2023· PMID 37153815mais citado
  6. A Benign Outpouching Masquerading as a Scar: A Case of an Incidentally Diagnosed Cardiac Diverticulum.
    Cureus· 2026· PMID 41952935recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:1686(Orphanet)
  2. MONDO:0015677(MONDO)
  3. GARD:1094(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q55785643(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

Divertículo cardíaco
Compêndio · Raras BR

Divertículo cardíaco

ORPHA:1686 · MONDO:0015677
Prevalência
Unknown
Herança
Not applicable
CID-10
Q24.8 · Outras malformações congênitas especificadas do coração
CID-11
Início
Infancy, Neonatal
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C4020965
EuropePMC
Wikidata
Papers 10a
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