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Related Concept Videos

Rheumatic Heart Disease I: Introduction01:23

Rheumatic Heart Disease I: Introduction

Rheumatic heart disease or RHD is a chronic condition that results from rheumatic fever, causing permanent damage to the heart valves.Etiology and Risk FactorsIt primarily arises from rheumatic fever, an inflammatory disease that can develop after untreated or inadequately treated group A streptococcal (GAS) pharyngitis. Streptococcus spreads through direct contact with oral or respiratory secretions. While the bacteria are the causative agents, factors like malnutrition, overcrowding, poor...
Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies01:22

Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies

The key clinical manifestations of Rheumatic heart disease (RHD) include several distinct cardiac symptoms.Carditis, a hallmark of acute rheumatic fever, involves inflammation of the heart's endocardium, myocardium, and pericardium. Chronic RHD often results from recurrent episodes of carditis. Its symptoms include the following:Murmurs are caused by valvular damage, especially to the mitral and aortic valves. Mitral stenosis or regurgitation is common, with characteristic heart murmurs...
Cardiomyopathy IV: Restrictive Cardiomyopathy01:29

Cardiomyopathy IV: Restrictive Cardiomyopathy

Restrictive cardiomyopathy (RCM) is a rare heart muscle disease characterized by impaired ventricular filling due to stiffened ventricular walls, leading to significant diastolic dysfunction.EtiologyRestrictive cardiomyopathy can arise from both inherited and acquired diseases, many of which are systemic. It is categorized into four main types: infiltrative, storage, non-infiltrative, and endomyocardial diseases.Infiltrative diseases, such as amyloidosis, lead to RCM by depositing amyloid...
Aneurysm I: Introduction01:30

Aneurysm I: Introduction

An aortic aneurysm is a localized outpouching or dilation at a weak point in the artery wall. It may involve different parts of the aorta, such as the abdominal aorta, aortic arch, or thoracic aorta.Etiological factorsSeveral disorders are associated with aortic aneurysms.Congenital causes, such as primary connective tissue disorders like Marfan syndrome, impact the integrity and strength of connective tissues, notably affecting the aorta. Marfan syndrome is a genetic disorder that specifically...
Aneurysm II: Clinical Manifestations and Diagnostic Studies01:21

Aneurysm II: Clinical Manifestations and Diagnostic Studies

Thoracic, aortic arch and abdominal aneurysms are significant vascular conditions that can present with various clinical manifestations and lead to serious complications. Understanding these manifestations and the appropriate diagnostic studies is essential for effective management and treatment.Thoracic Aortic AneurysmsThoracic aortic aneurysms often remain asymptomatic until they reach a size that impinges on adjacent structures. They typically cause deep, diffuse chest pain that radiates to...

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Murine Surgical Model of Topical Elastase Induced Descending Thoracic Aortic Aneurysm
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Pinhole Descending Aortic Rupture with Systemic Sclerosis and Dermatomyositis: A Case Report.

Norihiro Ando1, Ryuichi Nabeshima1, Taiki Ito1

  • 1Department of Cardiovascular Surgery, KKR Sapporo Medical Center, Sapporo, Hokkaido, Japan.

Annals of Vascular Diseases
|December 27, 2024
PubMed
Summary

This case report details a rare instance of thoracic descending aortic perforation in a patient with overlap syndrome (systemic sclerosis and dermatomyositis). The patient successfully underwent endovascular repair for this unusual vascular pathology.

Keywords:
overlap syndromepinholethoracic aortic rupture

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Area of Science:

  • Cardiovascular Medicine
  • Rheumatology
  • Vascular Surgery

Background:

  • Overlap syndrome, a concurrent manifestation of systemic sclerosis and dermatomyositis, presents complex diagnostic challenges.
  • Thoracic descending aortic perforation is a critical vascular emergency with high morbidity and mortality.
  • The co-occurrence of these conditions and aortic perforation is unprecedented in medical literature.

Observation:

  • A 75-year-old woman presented with acute symptoms including chest tightness, back pain, and dyspnea.
  • Computed tomography imaging identified a small rupture, or pinhole perforation, in the descending thoracic aorta.
  • The patient's clinical presentation suggested a rare vascular complication associated with her autoimmune condition.

Findings:

  • The patient was diagnosed with thoracic descending aortic perforation secondary to overlap syndrome.
  • A minimally invasive thoracic endovascular aortic repair (TEVAR) was performed.
  • Surgical intervention included placement of an aortic stent graft and left chest drainage.

Implications:

  • This case highlights the potential for rare vascular complications in patients with connective tissue diseases.
  • Endovascular repair offers a viable treatment option for thoracic aortic perforation in complex patient profiles.
  • Further research is warranted to understand the pathomechanisms linking autoimmune overlap syndromes and aortic pathologies.