Valvular and Congenital Heart Disease
Congenital left ventricular aneurysm: Clinical, imaging, pathologic, and surgical findings in seven new cases

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Abstract

Background Congenital left ventricular aneurysm is a poorly understood and potentially lethal entity. Methods and Results In a clinicopathologic study of 7 new cases, the major presenting features in 6 patients were congestive heart failure in 4, ventricular arrhythmias in a 32-week fetus, and multiple congenital anomalies in a fetus with trisomy 13. Accurate diagnosis was achieved in all 3 living patients by echocardiography, angiocardiography, and magnetic resonance imaging. The aneurysm was predominantly apical in 3 and involved most of the left ventricular free wall in 4. Of the 3 living patients, medical management alone sufficed in 2. The third, a newborn boy, underwent a new and successful aneurysm-exclusion left ventriculoplasty. The mitral valve was abnormal in all 4 autopsied cases, the papillary muscles being short, thin, or absent. The aneurysm was thinner and its area was larger than that of the nonaneurysmal left ventricle in all necropsied patients. Conclusions Congenital left ventricular aneurysm appears to be a developmental anomaly, an idiopathic dysplasia of left ventricular endocardium and myocardium. No evidence of a viral etiology was found. Some neonates can be managed medically, but others require urgent surgical intervention. A new surgical operation is presented, a functional left ventricular aneurysmectomy that minimizes intraoperative and postoperative blood loss and that preserves the coronary arteries. (Am Heart J 2001;141:491-9.)

Section snippets

Methods

The hospital records were reviewed with special emphasis on clinical findings, electrocardiograms, chest x-ray films, echocardiograms, angiocardiograms, and magnetic resonance imaging (in 2 cases). We also searched the records of the 3216 cases of congenital heart disease retained in the Cardiac Registry (the cardiac pathology laboratory) of the Children's Hospital in Boston and re-examined the 4 postmortem heart specimens of congenital left ventricular aneurysm (frequency = 0.12%).

Specimens

Results

In the interests of clarity and brevity, the findings in 7 cases of congenital left ventricular aneurysm are presented mainly in Tables I and II and Figures 1 to 9.

. Case 1, fetal 2-dimensional echocardiogram at 32 weeks' gestation, inferior view, showing large apical aneurysm (An) connecting via a broad mouth with the nonaneurysmal left ventricle (LV). The other chambers—right ventricle (RV), right atrium (RA), and left atrium (LA) —are unremarkable.

. A, Case 1, levophase of pulmonary

What is it?

Congenital left ventricular aneurysm appears to be an idiopathic endomyocardial dysplasia. The morphologic features of the internal left ventricular free wall, apex, septal surface, and papillary muscles are very different from normal (Fig. 5, Fig. 6, Fig. 7, Fig. 8). Consequently, congenital left ventricular aneurysm is considered to be an idiopathic developmental anomaly of the left ventricular endocardium and myocardium.

Evidence was not found to support the possibility of myocardial ischemia

Acknowledgements

We thank Neil Bowles, PhD, Xanthi Kourcouli, MD, and Jeffrey A. Towbin, MD, Director of the Pediatric Molecular Cardiology Laboratory, Baylor College of Medicine, Houston, Tex, who kindly performed the polymerase chain reaction investigation of our necropsied cases. We also thank Bill and Emily McIntosh for photography and artwork and Gloria Gaskill for secretarial assistance.

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    Reprint requests: Richard Van Praagh, MD, Cardiac Registry, Bader 138, Children's Hospital, 300 Longwood Ave, Boston, MA 02115. E-mail: [email protected]

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