Introdução
O que você precisa saber de cara
Esta é uma lista de doenças que começam com a letra "P".
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Entender a doença
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
Linha do tempo da pesquisa
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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
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Agenesia parcial do pericárdio congênita
Centros de Referência SUS
24 centros habilitados pelo SUS para Agenesia parcial do pericárdio congênita
Centros para Agenesia parcial do pericárdio congênita
Detalhes dos centros
Hospital Universitário Prof. Edgard Santos (HUPES)
R. Dr. Augusto Viana, s/n - Canela, Salvador - BA, 40110-060 · CNES 0003808
Serviço de Referência
Hospital Infantil Albert Sabin
R. Tertuliano Sales, 544 - Vila União, Fortaleza - CE, 60410-794 · CNES 2407876
Serviço de Referência
Hospital de Apoio de Brasília (HAB)
AENW 3 Lote A Setor Noroeste - Plano Piloto, Brasília - DF, 70684-831 · CNES 0010456
Serviço de Referência
Hospital Estadual Infantil e Maternidade Alzir Bernardino Alves (HIABA)
Av. Min. Salgado Filho, 918 - Soteco, Vila Velha - ES, 29106-010 · CNES 6631207
Serviço de Referência
Hospital das Clínicas da UFG
Rua 235 QD. 68 Lote Área, Nº 285, s/nº - Setor Leste Universitário, Goiânia - GO, 74605-050 · CNES 2338424
Serviço de Referência
Hospital Universitário da UFJF
R. Catulo Breviglieri, Bairro - s/n - Santa Catarina, Juiz de Fora - MG, 36036-110 · CNES 2297442
Atenção Especializada
Hospital das Clínicas da UFMG
Av. Prof. Alfredo Balena, 110 - Santa Efigênia, Belo Horizonte - MG, 30130-100 · CNES 2280167
Serviço de Referência
Hospital Universitário Julio Müller (HUJM)
R. Luis Philippe Pereira Leite, s/n - Alvorada, Cuiabá - MT, 78048-902 · CNES 2726092
Atenção Especializada
Hospital Universitário João de Barros Barreto
R. dos Mundurucus, 4487 - Guamá, Belém - PA, 66073-000 · CNES 2337878
Serviço de Referência
Hospital Universitário Lauro Wanderley (HULW)
R. Tabeliao Estanislau Eloy, 585 - Castelo Branco, João Pessoa - PB, 58050-585 · CNES 0002470
Atenção Especializada
Instituto de Medicina Integral Prof. Fernando Figueira (IMIP)
R. dos Coelhos, 300 - Boa Vista, Recife - PE, 50070-902 · CNES 0000647
Serviço de Referência
Hospital Pequeno Príncipe
R. Des. Motta, 1070 - Água Verde, Curitiba - PR, 80250-060 · CNES 3143805
Serviço de Referência
Hospital Universitário Regional de Maringá (HUM)
Av. Mandacaru, 1590 - Parque das Laranjeiras, Maringá - PR, 87083-240 · CNES 2216108
Atenção Especializada
Hospital de Clínicas da UFPR
R. Gen. Carneiro, 181 - Alto da Glória, Curitiba - PR, 80060-900 · CNES 2364980
Serviço de Referência
Hospital Universitário Pedro Ernesto (HUPE-UERJ)
Blvd. 28 de Setembro, 77 - Vila Isabel, Rio de Janeiro - RJ, 20551-030 · CNES 2280221
Serviço de Referência
Instituto Nacional de Saúde da Mulher, da Criança e do Adolescente Fernandes Figueira (IFF/Fiocruz)
Av. Rui Barbosa, 716 - Flamengo, Rio de Janeiro - RJ, 22250-020 · CNES 2269988
Serviço de Referência
Hospital São Lucas da PUCRS
Av. Ipiranga, 6690 - Jardim Botânico, Porto Alegre - RS, 90610-000 · CNES 2232928
Serviço de Referência
Hospital de Clínicas de Porto Alegre (HCPA)
Rua Ramiro Barcelos, 2350 Bloco A - Av. Protásio Alves, 211 - Bloco B e C - Santa Cecília, Porto Alegre - RS, 90035-903 · CNES 2237601
Serviço de Referência
Hospital Universitário da UFSC (HU-UFSC)
R. Profa. Maria Flora Pausewang - Trindade, Florianópolis - SC, 88036-800 · CNES 2560356
Serviço de Referência
Hospital das Clínicas da FMUSP
R. Dr. Ovídio Pires de Campos, 225 - Cerqueira César, São Paulo - SP, 05403-010 · CNES 2077485
Serviço de Referência
Hospital de Base de São José do Rio Preto
Av. Brg. Faria Lima, 5544 - Vila Sao Jose, São José do Rio Preto - SP, 15090-000 · CNES 2079798
Atenção Especializada
Hospital de Clínicas da UNICAMP
R. Vital Brasil, 251 - Cidade Universitária, Campinas - SP, 13083-888 · CNES 2748223
Serviço de Referência
Hospital de Clínicas de Ribeirão Preto (HCRP-USP)
R. Ten. Catão Roxo, 3900 - Vila Monte Alegre, Ribeirão Preto - SP, 14015-010 · CNES 2082187
Serviço de Referência
UNIFESP / Hospital São Paulo
R. Napoleão de Barros, 715 - Vila Clementino, São Paulo - SP, 04024-002 · CNES 2688689
Serviço de Referência
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
Nenhum ensaio clínico registrado para esta condição.
Publicações mais relevantes
Left Ventricular Pericardial Strangulation: A Diagnostic Challenge in Acute Coronary Syndrome.
Partial congenital pericardial defects are exceptionally uncommon and typically asymptomatic. In rare circumstances, cardiac herniation through the defect may precipitate mechanical strangulation with extrinsic coronary artery compression and myocardial infarction (MI). We describe a young man who presented with ST-elevation MI attributed to left ventricular (LV) herniation through a previously unrecognized pericardial defect. Despite a typical clinical presentation of MI, the highly atypical angiographic and echocardiographic findings required further investigation. Cardiac magnetic resonance was crucial for excluding alternative causes of LV wall thickening and coronary artery compression, such as tumors or infiltrative diseases, and contributed to identifying the pericardial defect. Although rare, partial pericardial defects should be considered in the differential diagnosis of acute coronary syndrome, especially in young patients with atypical presentations or findings.
A missed diagnosis: a case of partial pericardial defect.
Congenital pericardial defects (CPDs) are rare congenital abnormalities characterized by the complete or partial absence of the pericardium. They are often asymptomatic and discovered incidentally through imaging. Some individuals can experience non-specific symptoms, whilst others can have serious complications. The gold standard for diagnosing pericardial defects is cardiac MRI. Management is case-dependent and usually reserved for partial defects. Here, we present a case of a 57-year-old male who presented with recurrent chest pain and was found to have partial pericardial defect, a diagnosis missed on prior imaging, and discuss the diagnosis and management.
Outcomes of the Modified Warden Procedure for Partial Anomalous Pulmonary Venous Connection Drainage to a High Location in the Superior Vena Cava.
To report the safety and effectiveness of a modified Warden procedure. Twenty-six (26) patients underwent modified Warden surgery at our centre from September 2017 to September 2021. In all patients, the superior vena cava (SVC) was reconstructed by turning the atrial wall in the main body of the right atrium upwards and applying fresh autologous pericardial patches. There were 13 male patients and 13 female patients, and the median age of the patients was 7.0 (range, 0.3-47.0) years. The median follow-up time after surgery was 47.0 (range, 32.0-80.0) months. The aortic cross-clamp time was 108.50±34.72 minutes, the cardiopulmonary bypass time was 154.81±41.65 minutes, the median postoperative mechanical ventilation time was 8.5 (range, 0.0-91.0) hours, the median intensive care unit stay was 43.5 (range, 15.0-352.0) hours, and the median length of postoperative hospital stay was 7.0 (range, 6.0-19.0) days. All patients were safely and successfully discharged from the hospital. No deaths or sinus node dysfunction occurred during the follow-up period. The mean gradient of the reconstructed SVC and/or the right pulmonary vein (PV) increased in eight patients and decreased to less than 1 mmHg in six patients. One (1) adult patient had already undergone two vascular interventions, and one child had persistent stenosis but did not undergo reoperation at the time of the study. In addition, two patients were subjected to pericardial windowing. A modified Warden technique using a right atrial flap and autologous pericardium effectively corrected the high-plane connection between the PVs and SVC. This technique effectively avoids sinus node dysfunction; however, a short-term increase in the differential pressure of the re-established SVC or PV after the procedure is acceptable, and reintervention is required only in rare cases.
Prenatal diagnosis and management challenges of Pentalogy of Cantrell at term in a resource-constrained setting: A rare case report.
Pentalogy of Cantrell (POC) is a rare and often fatal congenital malformation characterized by a midline developmental defect involving five anatomical structures: the lower sternum, anterior diaphragm, diaphragmatic pericardium, abdominal wall, and heart. This condition presents significant diagnostic and therapeutic challenges, particularly in resource-limited settings where advanced imaging and surgical interventions are limited. Early diagnosis, supportive care, and strategic surgical planning with a multidisciplinary team are all key components in managing patients with POC. Here we present a case of a 32-year-old female (Gravidity 2 Parity 1 Living 1) presented to our specialty hospital in Northern Tanzania at 31 weeks and 3 days of gestational age for regular antenatal visit clinic with a complain of mild lower abdominal pain for 1 week. Obstetric ultrasound revealed a single fetus with an estimated gestational age of 31 weeks and 3 days and a 3.3 cm anterior chest wall defect with partial cardiac herniation, along with herniation of the liver, bowel, kidney, and urinary bladder with thoracolumbar kyphotic deformity was also seen. The radiological findings were consistent with limb body wall complex syndrome and POC. A multidisciplinary board team of obstetrician, pediatricians, surgeons and radiologist discussed the patient and planned for medical termination of the pregnancy. The patient was discharged home after receiving a psychological care. Psychological support was provided, and the patient remained emotionally stable during the follow-up. This case highlights the antenatal diagnostic and postnatal management challenges of POC in resource-constrained settings. Patients diagnosed with this condition should receive thorough antenatal counseling regarding the associated risks of morbidity and mortality. Currently, there are no established guidelines or studies defining the optimal mode of delivery for these cases. In case of severe malformations and or confirmed chromosomal abnormalities, and where legally permissible, pregnancy termination may be considered.
Congenital partial pericardial defect affecting the right ventricle in a dog.
A 1.8-year-old, 7.7-kg male mixed-breed dog was examined before castration surgery. Thoracic radiographs showed a prominent bulging of the cardiac silhouette. Echocardiography revealed a partial absence of the bright pericardial signal in the right ventricular outflow tract. Fluoroscopy revealed a bulging pulsating sac anterior to the right ventricular outflow tract. On contrast-enhanced computed tomography, a balloon-shaped right ventricular lumen protruding in the cranial direction was seen on the cranial side of the right ventricle near the pulmonary infundibulum. Accordingly, right ventricular herniation due to a partial pericardial defect was diagnosed. This report describes cardiac computed tomography in dogs with right ventricular pericardial defects; our findings highlight the usefulness of fluoroscopic examination in diagnosing pericardial defects.
Publicações recentes
Left Ventricular Pericardial Strangulation: A Diagnostic Challenge in Acute Coronary Syndrome.
Congenital partial pericardial defect affecting the right ventricle in a dog.
A missed diagnosis: a case of partial pericardial defect.
Congenital unilateral pericardial agenesis presenting as an isolated chest pain in an adolescent: a case report and comprehensive review.
📚 EuropePMCmostrando 31
Left Ventricular Pericardial Strangulation: A Diagnostic Challenge in Acute Coronary Syndrome.
Methodist DeBakey cardiovascular journalPrenatal diagnosis and management challenges of Pentalogy of Cantrell at term in a resource-constrained setting: A rare case report.
International journal of surgery case reportsCongenital partial pericardial defect affecting the right ventricle in a dog.
Journal of veterinary cardiology : the official journal of the European Society of Veterinary CardiologyA missed diagnosis: a case of partial pericardial defect.
Clinical research in cardiology : official journal of the German Cardiac SocietyCongenital unilateral pericardial agenesis presenting as an isolated chest pain in an adolescent: a case report and comprehensive review.
Journal of cardiothoracic surgeryHybrid Coronary Revascularization in Congenital Pericardial Absence: A Case Report.
The American journal of case reportsOutcomes of the Modified Warden Procedure for Partial Anomalous Pulmonary Venous Connection Drainage to a High Location in the Superior Vena Cava.
Heart, lung & circulation[Analysis of Prenatal Ultrasound Manifestations in 15 Cases of Cantrell Syndrome].
Sichuan da xue xue bao. Yi xue ban = Journal of Sichuan University. Medical science editionThe Heart Has its Reasons Which Reason Knows Not: A Curious Case of Chest Pain.
The Journal of emergency medicinePartial Pericardial Agenesis.
Methodist DeBakey cardiovascular journalModification of the Warden Procedure for Surgical Repair of Partial Anomalous Pulmonary Venous Connection.
World journal for pediatric & congenital heart surgeryHypogenetic right lung with partial anomalous pulmonary venous return and accessory diaphragm: a case of "scimitar lung".
Folia morphologicaReconstruction of Donor Anomalous Pulmonary Vein During Lung Transplantation.
The Annals of thoracic surgeryA case of congenital partial absence of the left pericardium presenting with atypical chest pain.
Turk Kardiyoloji Dernegi arsivi : Turk Kardiyoloji Derneginin yayin organidirCongenital Partial Absence of Pericardium in an Elderly Patient.
CureusSuccessful Surgical Separation of Thoraco-Omphalopagus Symmetrical Conjoined Twins in Iran: Two Case Reports.
Iranian journal of medical sciencesCase series, contemporary review and imaging guided diagnostic and management approach of congenital pericardial defects.
Open heartCongenital absence of pericardium: two cases and a comprehensive review of the literature.
BJR case reportsSuperior Vena Cava Flap to Reroute Partial Anomalous Pulmonary Venous Connection.
World journal for pediatric & congenital heart surgeryPartial Congenital Absence of The Pericardium: A Case Report.
Brazilian journal of cardiovascular surgeryPartial Pericardial Agenesis Mimicking Arrhythmogenic Right Ventricular Cardiomyopathy.
Clinical journal of sport medicine : official journal of the Canadian Academy of Sport MedicineCantrell Syndrome-A Rare Complex Congenital Anomaly: A Case Report and Literature Review.
Frontiers in pediatricsPartial Hammock Valve: Surgical Repair and Long-Term Follow-Up in 23 Patients.
The Annals of thoracic surgerySternal malformations and anesthetic management.
Paediatric anaesthesiaCongenital Diaphragmatic Hernia with Liver Herniation into the Pericardial Sac in a 30-Week Gestation Infant.
Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology SocietyCongenital Absence of the Pericardium.
Progress in cardiovascular diseases[Partial pericardium defect with a cardiac heart diverticulum and extensive intrauterine hypoxic myocardial lesions].
Der PathologeIsolated Partial Congenital Absence of the Pericardium: A Familial Presentation.
The Canadian journal of cardiologySymptomatic Partial Congenital Absence of the Pericardium Revealed Using Cardiac Magnetic Resonance.
The Canadian journal of cardiologyCongenital defects of the pericardium: a review.
European heart journal. Cardiovascular ImagingCongenital pericardial defect: a case of right pericardial partial absence with normal parietal pleura [corrected].
Japanese journal of radiologyAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
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Comunidades
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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.
- Left Ventricular Pericardial Strangulation: A Diagnostic Challenge in Acute Coronary Syndrome.
- A missed diagnosis: a case of partial pericardial defect.Clinical research in cardiology : official journal of the German Cardiac Society· 2026· PMID 40338327mais citado
- Outcomes of the Modified Warden Procedure for Partial Anomalous Pulmonary Venous Connection Drainage to a High Location in the Superior Vena Cava.
- Prenatal diagnosis and management challenges of Pentalogy of Cantrell at term in a resource-constrained setting: A rare case report.
- Congenital partial pericardial defect affecting the right ventricle in a dog.Journal of veterinary cardiology : the official journal of the European Society of Veterinary Cardiology· 2025· PMID 40840406mais citado
- Pericardial agenesis.
- Congenital unilateral pericardial agenesis presenting as an isolated chest pain in an adolescent: a case report and comprehensive review.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:99130(Orphanet)
- MONDO:0020455(MONDO)
- GARD:19667(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q55789395(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