Misdiagnosis of atrial septal defect in patients with hereditary telangiectasia (Osler-Weber-Rendu disease) and hepatic arteriovenous fistulas.

Two patients with hereditary telangiectasia (Osler-Weber-Rendu disease) and high-output congestive heart failure secondary to large hepatic arteriovenous malformations had preoperative heart catheterization and exploratory cardiotomy to correct presumed intracardiac left-to-right shunts at the atrial level. At operation, both patients had oxygen-enriched blood returning from the inferior vena cava. Subsequent hepatic angiography demonstrated large hepatic arteriovenous fistulas, and both patients underwent hepatic artery banding and ligation, with reduction of left-to-right shunting.

Atrial septal defect: echocardiographic observations. Studies in 120 patients.

Previous studies on small numbers of patients have indicated a high incidence of increased right ventricular dimension and abnormal ventricular septal motion in patients with atrial septal defect. However, recent evidence suggests that septal motion may be normal in as many as 46% of patients with atrial septal defect when observed at proper levels. We have analyzed the echocardiograms of 120 patients with sinus venosus or secundum atrial septal defect in an attempt to define sensitivity of the foregoing two echocardiographic abnormalities. Right ventricular dimension index was increased in 98% of patients. When both sides of the ventricular septum were analyzed, abnormal ventricular septal motion was observed in 87% of patients. It is important to observe both right and left septal echoes at all levels. Other measurements and observations were made when possible and are included in the study.

Cardiovascular complications of pheochromocytoma crisis.

Two patients with hypertensive crises due to pheochromocytoma presented with unusual features suggestive of cardiovascular disorders other than pheochromocytoma. These features included transient cortical blindness and other neurologic deficits, electrocardiographic changes indicative of transmural infarction and peripheral arterial spasm. In both patients the diagnosis of pheochromocytoma was made later; removal of the tumor was followed by ready reversal of the clinical and biochemical abnormalities. Management of such cases includes suspicion of the diagnosis in the presence of atypical features, early initiation of therapy with alpha and beta adrenergic blocking agents and a definitive surgical procedure before peripheral vascular changes become irreversible. When an apparent myocardial infarction occurs, the diagnosis of coronary artery disease cannot always be excluded, but rapid stabilization of the clinical and electrocardiographic changes after adrenergic blockade would favor the diagnosis of a catecholamine-induced myocarditis. The coexistence of coronary artery disease and uncontrollable arrhythmias presents an increased risk but, if pheochromocytoma is suspected, surgery may be necessary despite the increased risk.