Moçambique, oficialmente designado como República de Moçambique, é um país localizado no sudeste do continente africano, banhado pelo Oceano Índico a leste e que faz fronteira com a Tanzânia ao norte; Maláui e Zâmbia a noroeste; Zimbábue a oeste e Essuatíni e África do Sul a sudoeste. A capital e maior cidade do país é Maputo, anteriormente chamada de Lourenço Marques, durante o domínio português.
Introdução
O que você precisa saber de cara
Síndrome de Shone é uma cardiopatia congênita rara caracterizada por múltiplas obstruções do lado esquerdo do coração, incluindo estenose supravalvar mitral, válvula mitral em "parafuso de arquiteto", coarctação da aorta e hipoplasia do arco aórtico. Apresenta-se com insuficiência cardíaca e cianose, necessitando de intervenção cirúrgica precoce.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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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
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 — Síndrome de Shone
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Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Pesquisa e ensaios clínicos
Nenhum ensaio clínico registrado para esta condição.
Publicações mais relevantes
A rare case of partial Shone complex in adulthood: coarctation-site methicillin-sensitive Staphylococcus aureus endocarditis and saccular aneurysm.
Shone complex is a rare congenital cardiovascular anomaly characterized by multiple left-sided obstructive lesions. Adult presentation is uncommon and may result in atypical clinical manifestations, particularly when complicated by infective endocarditis. Coarctation of the aorta-associated infective endocarditis is especially rare and carries significant clinical risk. We report a 25-year-old male with partial Shone complex consisting of a parachute mitral valve, mitral stenosis, and coarctation of the aorta. The patient presented with persistent high-grade fever and was diagnosed with methicillin-sensitive Staphylococcus aureus infective endocarditis based on positive blood cultures. Transthoracic and transesophageal echocardiography revealed obstructive left-sided lesions, mitral valve pathology, and moderate aortic regurgitation. Positron emission tomography demonstrated intense hypermetabolic activity at the coarctation site, consistent with active infection and confirming a saccular aneurysm. The patient underwent prolonged intravenous cefazolin therapy followed by surgical repair. The early postoperative course was stable; however, seven months later he re-presented with recurrent fever. Repeat imaging demonstrated graft infection, vegetation on the prosthetic material, and a perforated mitral leaflet. A second course of intravenous antibiotics (cefazolin and rifampicin) was administered, followed by long-term suppressive oral therapy. Subsequent follow-up showed persistent moderate-to-severe mitral regurgitation with stable postoperative anatomy. This case highlights a rare adult manifestation of partial Shone complex complicated by coarctation of the aorta-site methicillin-sensitive Staphylococcus aureus infection, saccular aneurysm formation, and recurrent graft-associated infective endocarditis. The clinical course underscores the importance of vigilant multimodality imaging, early multidisciplinary evaluation, and long-term follow-up in adults with complex CHD, particularly when infectious complications are present.
Never Too Late: Late Heart Block in Shone Complex: A Case Series.
Shone complex is a group of coexisting obstructive left-sided cardiac lesions with variable presentation and severity. There are limited data regarding long-term outcomes of patients with Shone complex given the rarity of the condition. Late-onset (outside the postoperative period) complete heart block (CHB) has not typically been associated with Shone and is not well described in the literature. In this case series, we retrospectively analyzed 3 clinical cases of patients with Shone complex, aged 31 to 59 years, who developed late heart block. The primary objective of the case series was to identify any associated characteristics among the three Shone complex patients who developed late CHB.
Unmasking Partial Shone Complex: A Late Diagnosis with Rare Associations.
• Supravalvular AS is a rare finding in Shone complex (SC). • Despite absence of MS, common complications of SC developed. • Frequent reevaluation via echocardiography is key in detection and management. [Figure: see text]
Customized Pulmonary Artery Stent for Management of Complex Coronary Artery Fistula.
Coronary artery fistulas (CAFs) are rare anomalies that can cause ischemia, arrhythmias, and sudden cardiac death. Conventional closure techniques often fail in anatomically complex cases. We present the first-in-human use of a customized, partially covered G-Armor pulmonary artery (PA) stent to manage a left main-to-right pulmonary artery fistula in a patient with Shone complex. The intervention was planned using a three-dimensionally printed model, allowing precise stent customization to avoid obstruction of key pulmonary branches. This case shows the feasibility of using customized endovascular devices to address complex CAFs in high-risk patients. The intervention improved antegrade coronary perfusion and clinical outcomes. To the best of our knowledge, this is the first reported case of a customized partially covered PA stent for CAF management. In anatomically complex and high-risk CAFs, customized stenting offers a viable solution when conventional therapies fail.
A Rare Case of Shone Complex With a Persistent Left Superior Vena Cava and Absent Right Superior Vena Cava.
Shone complex is a rare and underrecognized congenital heart disease involving four obstructive lesions of the left heart. This report presents a rare case of incomplete Shone complex, along with other associated anomalies, requires an extensive clinical workup for accurate diagnosis and appropriate surgical planning.
Publicações recentes
A rare case of partial Shone complex in adulthood: coarctation-site methicillin-sensitive Staphylococcus aureus endocarditis and saccular aneurysm.
Unmasking Partial Shone Complex: A Late Diagnosis with Rare Associations.
Customized Pulmonary Artery Stent for Management of Complex Coronary Artery Fistula.
A Rare Case of Shone Complex With a Persistent Left Superior Vena Cava and Absent Right Superior Vena Cava.
Never Too Late: Late Heart Block in Shone Complex: A Case Series.
📚 EuropePMC35 artigos no totalmostrando 45
A rare case of partial Shone complex in adulthood: coarctation-site methicillin-sensitive Staphylococcus aureus endocarditis and saccular aneurysm.
Cardiology in the youngUnmasking Partial Shone Complex: A Late Diagnosis with Rare Associations.
CASE (Philadelphia, Pa.)Customized Pulmonary Artery Stent for Management of Complex Coronary Artery Fistula.
JACC. Case reportsA Rare Case of Shone Complex With a Persistent Left Superior Vena Cava and Absent Right Superior Vena Cava.
CureusNever Too Late: Late Heart Block in Shone Complex: A Case Series.
Pediatric cardiologyDon't Give up on the Left Ventricle! Surgical Strategies for Recruitment of the Borderline Left Heart.
Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annualSevere Reversible Pulmonary Hypertension in a Patient with Shone Complex Presenting for Heart Transplantation.
Journal of cardiothoracic and vascular anesthesiaA clinical perspective on surgical and diagnostic strategies for neonatal partial shone complex: Insights from a case report.
International journal of surgery case reportsThe landscape of congenital heart disease treated with the Ross procedure.
The Journal of thoracic and cardiovascular surgeryLong-term outcomes following the Ross procedure in neonates and infants: A multi-institutional analysis.
The Journal of thoracic and cardiovascular surgeryArrhythmic Risk in Shone Complex: Lumping the Heterogeneity Together.
JACC. AdvancesAtrial and Ventricular Arrhythmia in Adults With Shone Complex: A Single-Center Cohort Study.
JACC. AdvancesEchocardiography to the Rescue in Adult-Onset Partial Shone Complex.
CASE (Philadelphia, Pa.)The Road to Heart Transplant in a Patient With Cardiomyopathy, Shone Complex, and Severe Pulmonary Hypertension.
JACC. Case reportsNon-syndromic Parachute Mitral Valve "When the Valve Dives in": Case Report and Review of the Literature.
CureusAn Undiagnosed Shone Complex in a 52-Year-Old Female: A Case Report.
Journal of investigative medicine high impact case reportsA Rare Case of Intramitral Valve Ring in the Setting of Shone Complex Diagnosed by Echocardiography.
Journal of cardiovascular echographySurgical Considerations in Shone Complex.
Seminars in cardiothoracic and vascular anesthesiaSingle coronary artery and coronary artery-pulmonary artery fistula in a variation of the Shone complex.
Pediatric radiologyShone Complex: A Case Report of Congenital Heart Disease Detected Using Point-of-care Ultrasound.
Clinical practice and cases in emergency medicineDouble-chambered left ventricle in a patient with incomplete Shone complex.
Revista portuguesa de cardiologia : orgao oficial da Sociedade Portuguesa de Cardiologia = Portuguese journal of cardiology : an official journal of the Portuguese Society of CardiologyComparison of early-stage stenosis and regurgitation results among patients who underwent aortic valve repair.
Cardiology in the youngRoss procedure in neonates and infants: A valuable operation with defined limits.
The Journal of thoracic and cardiovascular surgeryOutcomes of aortic valve repair in children resulting in bicuspid anatomy: Is there a need for tricuspidization?
The Journal of thoracic and cardiovascular surgeryShone syndrome revealed by treatment-resistant hypertension.
Annals of medicine and surgery (2012)The Appearance of an Underrecognized Congenital Heart Disease in a Patient With COVID-19 Pneumonia.
Journal of investigative medicine high impact case reportsThe clinical presentation and outcome of aortic coarctation associated with left ventricular inflow and outflow tract lesion in adult patients: Shone syndrome and beyond.
International journal of cardiologyMitral valve replacement in infants and younger children.
Scientific reportsAn unusual case of Shone's complex in an adult depicted on computed tomography angiography.
Journal of cardiac surgeryRare Variant of Shone Complex.
Circulation. Cardiovascular imagingComplex congenital heart diseases among children presenting for cardiac surgery in a tertiary health facility in Enugu; South-East Nigeria. A rising trend.
Nigerian journal of clinical practiceHemodynamics in Adults With the Shone Complex.
The American journal of cardiologyPediatric Cardiology Specialist's Opinions Toward the Acceptability of Comfort Care for Congenital Heart Disease.
Pediatric cardiologyManagement of Severe Coarctation of the Aorta During Pregnancy.
JACC. Case reportsHeart failure with reduced and preserved ejection fraction in adult congenital heart disease.
Heart failure reviewsRole of perioperative echocardiography in repair of incomplete shone complex: A case series.
Annals of cardiac anaesthesiaIncomplete Form of Shone Complex in an Adult Congenital Heart Disease Patient.
The Korean journal of thoracic and cardiovascular surgeryComputed tomography imaging characteristics of shone syndrome.
Radiology case reportsContribution of rare inherited and de novo variants in 2,871 congenital heart disease probands.
Nature geneticsCongenital heart defects in molecularly proven Kabuki syndrome patients.
American journal of medical genetics. Part AEvolving Understanding of Shone Complex Through the Lifespan: What's in an Eponym?
The Canadian journal of cardiologyShone Complex: An Under-recognized Congenital Heart Disease With Substantial Morbidity in Adulthood.
The Canadian journal of cardiologyRole of intraoperative transesophageal echocardiography in pediatric cardiac surgery.
Journal of the Saudi Heart AssociationDouble orifice mitral valve and bicuspid aortic valve: pieces of the same single puzzle?
Case reports in cardiologyRight Ventricular Enlargement In Utero: Is It Coarctation?
Pediatric cardiologyAssociações
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.
- A rare case of partial Shone complex in adulthood: coarctation-site methicillin-sensitive Staphylococcus aureus endocarditis and saccular aneurysm.
- Never Too Late: Late Heart Block in Shone Complex: A Case Series.
- Unmasking Partial Shone Complex: A Late Diagnosis with Rare Associations.
- Customized Pulmonary Artery Stent for Management of Complex Coronary Artery Fistula.
- A Rare Case of Shone Complex With a Persistent Left Superior Vena Cava and Absent Right Superior Vena Cava.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:99063(Orphanet)
- MONDO:0020404(MONDO)
- GARD:19630(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q2279783(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
