Congenital coronary artery fistulas complicated with pulmonary hypertension: Analysis of 211 cases.

AIM: To compare the behavior of pulmonary hypertension (PHT) associated with coronary artery fistulas (CAFs) between the Asian and Caucasian subjects.

METHODS: CAFs may be complicated with PHT secondary to left-to-right shunt. Literature review limited to the English language. A total of 211 reviewed patients were collected. Of those, 111 were of Asian and 100 were of Caucasian ethnic origin. The mean age of the Asian and the Caucasian groups of patients were 48.9 (range 19-83) and 49.9 years (range 16-85), respectively. In both groups, right heart catheterization was the most commonly (95%) used method for determining pulmonary artery pressure.

RESULTS: From all of the reviewed subjects, PHT was found in 49 patients (23%), of which 15 were Asian and 34 were Caucasian. In 75% of PHT subjects, mild to moderate PHT was reported and 76% of the fistulas had a vascular mode of termination. Treatment was surgical in 61%, followed by percutaneous therapeutic embolization (27%) and finally conservative medical management in 12% of PHT subjects. PHT was associated with a slight female gender predominance. The majority demonstrated mild to moderate PHT. PHT was reported more frequent in the Caucasian compared with the Asian ethnicity group. The majority of fistulas in patients with PHT had a vascular mode of termination. The results of this review are intended to be indicative and require cautious interpretation.

CONCLUSION: The likelihood for a CAF patient to develop PHT is presented when possessing the following features, with a Caucasian female having a fistula with a vascular mode of termination.