Current review
Congenital foramen of the left pericardium

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Abstract

Congenital foramen of the left parietal pericardium is uncommon. The condition has the potential to cause angina pectoris, myocardial infarction, or even death. Forty-three confirmed cases have been retrieved from the English language literature, and a case report of “life-threatening” herniation of the heart is here added. The diagnosis, made at a mean age of 20 years (range 2 to 48), was five times more common in men. In 5 fatal cases, the heart had become incarcerated. In the remainder of cases, one-third were asymptomatic, and two-thirds suffered a chest complaint that prompted diagnosis. Chest discomfort, dyspnea, and syncope were the most common symptoms. The most common finding at surgery, which 34 patients underwent, was a foramen at the base of the heart through which the left atrial appendage had herniated. In eight instances, the rim of the defect lay upon and compressed the coronary circulation. Measures to remedy the disorder have included a variety of operations, some to enlarge the defect, others to close it, amputation of the atrial appendage, and, in two cases, myocardial revascularization. Surgery is appropriate in the majority of symptomatic patients and in all who are at risk for ventricular herniation.

Section snippets

Case report

On January 19, 1978, during a light snow, P.M., age 31, was roused from sleep by pain which she said, “felt like I was hit in the chest by a heart attack.” She was unable to lie flat, preferring to sit upright. Within a few moments it became more painful while spreading to the upper left chest and arm. Breathing had no effect, but moving her left arm made it worse. She went to the emergency room of the community’s hospital, where she received injections of morphine. After 4 1/2 hours of steady

Material and methods

The accounts of the foramen type of defect of the left parietal pericardium that are the basis of this review are only confirmed∗ cases reported after the 1938 review of Southworth and Stevenson [3]. This article does not represent all such reports, but only English language papers found with a MEDLINE search, citations from earlier papers, plus the case herein recorded. Defects confined to the right side of the pericardium do occur, and herniation of the right atrium has been reported, but

Results

Forty-four accounts (5 fatal, 39 nonfatal) of the foramen type of defect were retrieved. There was a fivefold male preponderance (37 to 7). Age at diagnosis ranged from 2 to 48 years, with a mean age of 20. In the 5 fatal cases, only 1 survived the 7th year. Otherwise, the ages clustered in the second and third decades, with almost two-thirds between 11 and 29 years.

Clinical features

Males predominate. Many individuals are symptom free, and about one in three defects are suspected only after a chest film is made for unrelated reasons. In those investigated for a complaint probably due to the disorder, (eg, chest discomfort, dyspnea, syncope), the most common by far is chest discomfort. Occasionally arm movement, especially the left, breathing, or turning from side to side affects the discomfort, but otherwise the character is such that it sheds little light as to cause.

Acknowledgements

The author thanks Freeman T. Bennett, MD, for helpful suggestions and critical review of this manuscript.

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