Left coronary artery arteriovenous malformation presenting as a diastolic murmur with exercise intolerance in a child with a suspected familial vascular malformation syndrome.
OBJECTIVE
Intracardiac arteriovenous malformations are rare and may be associated with sudden death in adults. This case report describes an intracardiac left coronary arteriovenous malformation in a 7-year-old boy with a suspected familial cutaneous vascular malformation syndrome. The patient presented with a diastolic murmur, exercise intolerance, chest pain, and a left ventricular mass.


METHODS
The left ventricular mass was initially identified by echocardiography. Subsequently, a computed tomography scan revealed the vascular nature of the lesion. We hypothesized that the lesion represented either an arteriovenous malformation (AVM) or a hemangioma. These lesions are thought to cause coronary steal and myocardial dysfunction. Skin biopsies of the patient's cutaneous lesions revealed capillary hyperplasia, which was not consistent with either hemangioma or AVM. Thus, a surgical biopsy and partial resection of the mass was performed.


RESULTS
The surgical pathology of the cardiac mass was consistent with an AVM. Within 6 months following partial resection of the mass, the patient unexpectedly developed a left ventricular pseudoaneurysm at the resection site and required re-operation. Although a portion of the mass remains, both the patient's chest pain and exercise tolerance have improved subjectively.


CONCLUSIONS
Patients with cutaneous vascular malformations and diastolic murmurs, as well as cardiac symptoms, should undergo echocardiography or alternative imaging modalities to screen for treatable pathological myocardial vascular malformations.
Schroeder
Valerie A
VA
Division of Pediatric Cardiology, University of Kentucky, Lexington, KY 40536, USA. vschroeder1@hotmail.com
Lee
Eun Y
EY
Douglas
William I
WI
Cottrill
Carole M
CM
D017541:Aneurysm, False / Q000601:surgery; D001165:Arteriovenous Malformations / Q000150:complications* / Q000175:diagnosis / Q000473:pathology / Q000601:surgery; D002637:Chest Pain / Q000209:etiology; D002648:Child; D003330:Coronary Vessel Anomalies / Q000150:complications* / Q000175:diagnosis / Q000473:pathology / Q000601:surgery; D003971:Diastole; D004452:Echocardiography; D017079:Exercise Tolerance; D006322:Heart Aneurysm / Q000601:surgery; D006337:Heart Murmurs / Q000209:etiology* / Q000503:physiopathology; D006352:Heart Ventricles; D006801:Humans; D008297:Male; D011183:Postoperative Complications; D012086:Reoperation; D012867:Skin / Q000098:blood supply*; D014057:Tomography, X-Ray Computed; D054079:Vascular Malformations / Q000235:genetics*