Thirteen new patients and 174 patients previously reported with coronary arteriovenous fistula (CAVF) were reviewed to delineate the course and management of CAVF and to clarify the role of surgical ligation in the young asymptomatic patient. Patients were grouped according to age: 99 patients (four new and 95 reported) were less than 20 years old and 88 (nine new and 79 reported) were greater than or equal to 20 years old. Of those under 20 years of age, 19% had preoperative symptoms or CAVF-related complications, including congestive heart failure (CHF) in 6%, subacute bacterial endocarditis in 3% and death in one patient. Seventy-six patients younger than 20 years old had CAVF ligation with only one significant complication. In contrast, 63% of the older group and all of our nine older patients had preoperative symptoms or complications, including CHF in 19%, SBE in 4%, myocardial infarction (MI) in 9%, death in 14% and fistula rupture in one patient. Of the 43 ligated older patients, 23% had surgical complications, including MI in three and death in three. Mean pulmonic-to-systemic flow in the entire group was 1.6:1 and did not differ significantly in those with or without symptoms or complications. One of our patients and one previously reported had spontaneous CAVF closure. In summary, early elective ligation of CAVF is indicated in all patients because of the high incidence of late symptoms and complications and the increased morbidity and mortality associated with ligation in older patients.

Congenital coronary arteriovenous fistula. Report of 13 patients, review of the literature and delineation of management.

Bilateral coronary artery-pulmonary artery fistulas fistulas that originate from both coronary arteries are an uncommon subgroup of coronary fistulas and may have a distinct embryologic origin. We present five original cases and review the nine previously reported cases of this anomaly.

Bilateral coronary artery--pulmonary artery fistulas. Report of five cases and review of the literature.

The first stage of a repair of pulmonary atresia with intact ventricular septum (type I) was attempted in a 2-day-old infant. At surgery, decompression of the hypertensive small right ventricle was followed by a sudden loss of myocardial contractility and death. Postmortem examination revealed a fistula with a large orifice in the right ventricular infundibulum that communicated directly with the left main coronary artery. Severe hypertensive changes indicative of abnormally high perfusion pressure were noted in the distal left coronary artery branches. The clinical course suggests that the effect of relieving right ventricular outflow obstruction was a reduction of left main coronary artery blood flow, resulting in fatal intraoperative myocardial ischemia. This unusual case draws attention to the anomalous ventriculocoronary communications often present in pulmonary atresia and their potential for limiting a successful surgical repair.

Pulmonary atresia with intact ventricular septum and ventriculocoronary communications: surgical significance.

Ten women with primary pulmonary hypertension had resting hemodynamic measurements taken before and after the nonparenteral administration of various vasodilators and prostaglandin inhibitors. Only sublingual isoproterenol, alone or combined with sublingual isosorbide dinitrate, effected a substantial (greater than 20%) drop in pulmonary vascular resistance; this decrease was accompanied by little change in pulmonary artery pressure. Isosorbide dinitrate was the only drug that elicited any reduction in pulmonary artery pressure; pulmonary vascular resistance decreased modestly. The oral administration of diazoxide, hydralazine, phentolamine, and tolazoline elicited little change in pulmonary artery pressure or resistance. Except for tolazoline, all these agents significantly decreased systemic blood pressure and resistance. Prostaglandin inhibition by indomethacin (acute and chronic dosing) increased pulmonary and systemic vascular resistances and reduced cardiac output. Aspirin combined with dipyridamole elicited no changes. The vasodilators and prostaglandin inhibitors studied evoked little improvement in resting pulmonary hemodynamic abnormalities in primary pulmonary hypertension.

Vasodilators and Prostaglandin Inhibitors in Primary Pulmonary Hypertension

The early and late results of surgical treatment in 23 patients with congenital coronary artery fistula (CCAF) are presented. One patient died for reasons unrelated to the fistula during corrective surgery for an associated complex cyanotic abnormality. The patients with isolated CCAF and preoperative symptoms improved significantly after surgical closure of the CCAF (p less than 0.05). We conclude that all symptomatic patients with CCAF should be treated with surgical closure of the fistula.

https://thorax.bmj.com/content/thoraxjnl/42/5/369.full.pdf

Early and late results of surgical treatment of congenital coronary artery fistula

Possible relationship between pulmonary hypertension and prostaglandins

Anomalies of the systemic venous system.

Congenital coronary artery cardiac chamber fistula.

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Possible relationship between pulmonary hypertension and prostaglandins

Anomalies of the systemic venous system.

Congenital coronary artery cardiac chamber fistula.