Congenital absence of the pericardium

Jump to navigation Jump to search
Congenital absence of the pericardium

WikiDoc Resources for Congenital absence of the pericardium

Articles

Most recent articles on Congenital absence of the pericardium

Most cited articles on Congenital absence of the pericardium

Review articles on Congenital absence of the pericardium

Articles on Congenital absence of the pericardium in N Eng J Med, Lancet, BMJ

Media

Powerpoint slides on Congenital absence of the pericardium

Images of Congenital absence of the pericardium

Photos of Congenital absence of the pericardium

Podcasts & MP3s on Congenital absence of the pericardium

Videos on Congenital absence of the pericardium

Evidence Based Medicine

Cochrane Collaboration on Congenital absence of the pericardium

Bandolier on Congenital absence of the pericardium

TRIP on Congenital absence of the pericardium

Clinical Trials

Ongoing Trials on Congenital absence of the pericardium at Clinical Trials.gov

Trial results on Congenital absence of the pericardium

Clinical Trials on Congenital absence of the pericardium at Google

Guidelines / Policies / Govt

US National Guidelines Clearinghouse on Congenital absence of the pericardium

NICE Guidance on Congenital absence of the pericardium

NHS PRODIGY Guidance

FDA on Congenital absence of the pericardium

CDC on Congenital absence of the pericardium

Books

Books on Congenital absence of the pericardium

News

Congenital absence of the pericardium in the news

Be alerted to news on Congenital absence of the pericardium

News trends on Congenital absence of the pericardium

Commentary

Blogs on Congenital absence of the pericardium

Definitions

Definitions of Congenital absence of the pericardium

Patient Resources / Community

Patient resources on Congenital absence of the pericardium

Discussion groups on Congenital absence of the pericardium

Patient Handouts on Congenital absence of the pericardium

Directions to Hospitals Treating Congenital absence of the pericardium

Risk calculators and risk factors for Congenital absence of the pericardium

Healthcare Provider Resources

Symptoms of Congenital absence of the pericardium

Causes & Risk Factors for Congenital absence of the pericardium

Diagnostic studies for Congenital absence of the pericardium

Treatment of Congenital absence of the pericardium

Continuing Medical Education (CME)

CME Programs on Congenital absence of the pericardium

International

Congenital absence of the pericardium en Espanol

Congenital absence of the pericardium en Francais

Business

Congenital absence of the pericardium in the Marketplace

Patents on Congenital absence of the pericardium

Experimental / Informatics

List of terms related to Congenital absence of the pericardium

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]


Overview

Congenital absence of the pericardium or congenital defects of the pericardium (1/10,000 autopsies) comprise partial left (70%), right (17%), or total bilateral (extremely rare) pericardial absence. About 30% of patients have additional congenital abnormalities. Most patients with a total absence of pericardium are asymptomatic. However, homolateral cardiac displacement and augmented heart mobility impose an increased risk for traumatic aortic type A dissection.

Associated congenital abnormalities

Pathopysiology

Normally, the aortopulmonary window is covered by pericardium and contains some fat. Most pericardial defects are partial and occur on the left side. Left-sided absence of the pericardium allows interposition of lung tissue between the aorta and the main segment of the pulmonary artery. Occasionally, there is bulging of the left atrial appendage through the defect. The heart usually rotates toward the left.

Diagnosis

Patients who have a pericardial defect without associated congenital abnormalities are often asymptomatic. The chest x-ray is typical, but the diagnosis is confirmed by echocardiography and computed tomography (CT) / magnetic resonance imaging (MRI).

Plain film

CT

  • Interposition of lung tissue between the aorta and the main segment of the pulmonary artery

Treatment

Excision of the atrial appendage and surgical pericardioplasty (Dacron, Gore-tex, or bovine pericardium) is indicated for imminent strangulation.

Complications

Partial left side defects can be complicated by cardiac strangulation caused by herniation of the left atrial appendage, atrium, or left ventricle through the defect (chest pain, shortness of breath, syncope, or sudden death).

References

See Also

External Links


Template:WikiDoc Sources