Recurrent coronary artery spasm in the billowing mitral leaflet syndrome (primary mitral valve prolapse). A case report and review of the literature.

A 46-year-old White man had a 2-year history of frequent atypical chest pain associated with palpitations and presyncope. Clinical examination revealed an intermittent mid-systolic non-ejection click followed by a blowing mid-to-late systolic murmur indicative of the billowing mitral leaflet syndrome (BMLS) (primary mitral valve prolapse (MVP); Barlow's syndrome). This diagnosis was confirmed on cardiac catheterization, left ventricular cine angiography showing mild mitral insufficiency. M-mode and cross-sectional echocardiography documented the intermittent non-ejection click and mid-systolic apical murmur. Ambulatory Holter monitoring showed symptomatic inferolateral myocardial ischaemia, and maximal stress-testing elicited asymptomatic ischaemia in the same zone. Selective coronary angiography delineated a normal left coronary artery and an insignificant fixed obstructive lesion in the second part of the dominant right coronary artery (RCA). Provocation with ergometrine (ergonovine) maleate gave rise to severe coronary vasospasm superimposed on the insignificant lesion in the RCA. This spasm provoked myocardial ischaemia resulting in symptomatic ventricular fibrillation which was successfully reversed. The patient's symptoms have been fairly well controlled by nitrates and nifedipine. As far as I am aware this is the first documentation of coronary vasospasm in the BMLS. In this syndrome coronary artery spasm has often been postulated to be responsible for acute myocardial infarction with a normal appearance of the coronary arteries on angiography. This mechanism has also been incriminated in the genesis of ventricular arrhythmias in cases of primary MVP. These various contentious and important issues are reviewed.