Valvular and Congenital Heart DiseaseCongenital left ventricular aneurysm: Clinical, imaging, pathologic, and surgical findings in seven new cases☆
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.
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]