Acquired and progressive coronary arterial fistulae in patients with single-ventricle physiology and treated with pulmonary vasodilators.

BACKGROUND: Cardiac dysfunction, arrhythmia, and hepatic fibrosis are well-known complications after right heart bypass surgery in patients with single-ventricle physiology. However, little is known about coronary arterial fistulae, and only a few reports have been published. This study aimed to elucidate the clinical characteristics of these rare coronary arterial fistulae that developed as complications in cases of single-ventricle physiology after right heart bypass surgery. METHODS: We retrospectively investigated the clinical features and courses of patients who developed acquired and progressive coronary arterial fistulae after right heart bypass surgery in our hospital. RESULTS: We identified three cases of coronary arterial fistulae out of 21 patients who underwent right heart bypass surgery. All three cases underwent cardiac catheterisation for post-operative evaluation and were administered pulmonary vasodilators of phosphodiesterase type V inhibitors, antiplatelet, anticoagulation, and diuretics. Moreover, they had common clinical features such as right-dominant single ventricle and long-term exposure to chronic hypoxia. Serial angiograms revealed acquired and progressive coronary arterial fistulae. In addition, coronary arterial fistulae contributed to their symptoms of heart failure. CONCLUSION: Patients with chronic hypoxia and dominant right ventricle, who are treated with phosphodiesterase type V inhibitors, should be followed up after right heart bypass surgery to monitor the possible development of coronary arterial fistulae. Moreover, the indication for pulmonary vasodilators in single-ventricle physiology after right heart bypass surgery should be optimised to avoid adverse effects.

Fistulae ligation and left main artery ligation for a bilateral giant coronary arterial fistulae-related aneurysm.

Bilateral congenital coronary artery fistulae complicated with a giant coronary artery aneurysm is a very rare condition. A coronary artery aneurysm is a coronary artery dilatation that exceeds the diameter of normal adjacent segments or the diameter of the patient's largest coronary vessel by 1.5 times. The complications associated with a coronary artery aneurysm include thrombosis, embolization, rupture, vasospasm, congestive heart failure and infectious endocarditis. We report on a 63-year-old woman presenting with severe heart failure related to bilateral coronary artery fistulae. A giant coronary aneurysm was noted in the right coronary artery, and a tortuous coronary artery fistula was noted in the left coronary artery. Symptoms were relieved after surgical intervention for bilateral coronary artery fistulae.