Prevalence of congenital cardiovascular malformations in children of human immunodeficiency virus-infected women

Coronary artery anomalies were found in 1,686 patients (1.3% incidence) undergoing coronary arteriography at the Cleveland Clinic Foundation from 1960 to 1988. Of the 1,686 patients, 1,461 (87%) had anomalies of origin and distribution, and 225 (13%) had coronary artery fistulae. Most coronary anomalies did not result in signs, symptoms, or complications, and usually were discovered as incidental findings at the time of catheterization. Eighty-one percent were "benign" anomalies: 1) separate origin of the left anterior descending and circumflex from the left sinus of Valsalva; 2) ectopic origin of the circumflex from the right sinus of Valsalva; 3) ectopic coronary origin from the posterior sinus of Valsalva; 4) anomalous coronary origin from the ascending aorta; 5) absent circumflex; 6) intercoronary communications; and 7) small coronary artery fistulae. Other anomalies may be associated with potentially serious sequelae such as angina pectoris, myocardial infarction, syncope, cardiac arrhythmias, congestive heart failure, or sudden death. Potentially serious anomalies include: 1) ectopic coronary origin from the pulmonary artery; 2) ectopic coronary origin from the opposite aortic sinus; 3) single coronary artery; and 4) large coronary fistulae. Coronary artery anomalies require accurate recognition, and at times, surgical correction.

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.

This study compares the value of transthoracic (TTE) and transesophageal (TEE) color Doppler and contrast echocardiography for detecting a patent foramen ovale (PFO). A total of 238 patients were studied: 74 patients with a history of otherwise unexplained ischemic stroke, transient cerebral ischemic attacks or peripheral embolic events (group A), 48 with a history of similar episodes explained by other cardiac abnormalities (group B), and 116 with no embolic events (group C). A PFO was detected by contrast TEE in 50 of 238 patients (21%) compared with 45 patients (19%) by color Doppler TTE. In a subgroup of 198 patients, TEE results could be compared with TTE findings. No patient had a PFO identified by color Doppler TTE. Contrast TTE detected a PFO in 15 patients (8%) compared with contrast TEE which demonstrated a PFO in 44 of 198 patients (22%) (p less than 0.001). Prevalence of PFO by TEE was 22, 21 and 22% in groups A, B and C, respectively. A PFO was present in 50% of patients aged less than 40 years and otherwise unexplained ischemic stroke; this percentage was higher (p less than 0.05) than corresponding values found in all other groups. Thus, contrast and color Doppler TEE are significantly superior to TTE for detecting PFO. The prevalence of PFO is significantly increased in young adults with otherwise unexplained ischemic stroke.