Dutch survey of congenital coronary artery fistulas in adults: coronary artery-left ventricular multiple micro-fistulas multi-center observational survey in the Netherlands.

BACKGROUND

Congenital coronary artery-left ventricular multiple micro-fistulas (CA-LVMMFs) in adults are rare anomalies. They may cause angina pectoris and myocardial infarction in association with normal coronary arteries.

METHODS AND RESULTS

From the medical databases of a Dutch Survey of coronary artery fistulas in adult cardiology population (30,829 patients), we identified 20 patients with CA-LVMMFs out of 71 fistula-subjects between 1996 and 2003. Clinical files and individual coronary angiograms were reviewed and analysed. There were 13 females and 7 males with a mean age of 67.3 years (range 49-82). The main presenting symptoms were angina pectoris and dyspnea in 70% of the patients. The ECG showed pathologic changes in 75%. Exercise tolerance test and 201-thallium stress scintigraphy were positive for myocardial ischemia in 29% and 50%, respectively of the tested patients. In the absence of significant atherosclerotic coronary artery disease, ipsilateral to the fistulas, myocardial infarction was documented in 15% of the patients. Chest X-ray revealed cardiomegaly in 38% of the patients. Congestive heart failure was documented in 10% of the patients. Uni-, bi- and multilateral fistulas were present in 50%, 45% and 5%, respectively. The origin was the LCA in 71% and the RCA in 29% of the fistulas. The majority (97%) originated from the mid or distal segments of the coronary vessels. Among those patients, the coronary arterial tree had single, dual, and triple vessel disease in 25%, 15% and 5%, respectively. Angiographic anatomy precludes surgical intervention; they were all followed by conservative medical management.

CONCLUSIONS

Coronary artery-left ventricular multiple micro-fistulas are found more often in female patients. Furthermore, they originated from the distal segment of the coronary arteries. Coronary artery-left ventricular multiple micro-fistulas, in the presence of normal coronary arterial tree, may often lead to angina pectoris and coronary insufficiency.