The diagnosis of cor triatriatum sinistrum in children: a continuing dilemma.

Cor triatriatum (CT) is a rare congenital cardiac anomaly. The salient clinical, roentgenographic, electrocardiographic, echocardiographic, and hemodynamic findings are presented in two asymptomatic children and one with nonspecific dyspnea on exertion. Two male children had a classical form of cor triatriatum with normal physical and inconsequential roentgenographic and electrocardiographic findings. One of the male patients had surgery for a large atrial septal defect ostium secundum (ASD 2 degrees) and pulmonary hypertension in infancy. The female patient had CT with a communicating accessory chamber to right atrium and a rare patent foramen ovale. Her clinical findings confirmed an atrial level shunt. All patients had excision of the fibromuscular membrane from the right and left atrial transseptal approach with excellent results and with no recurrence during 2 to 4 year follow-up. We report the dilemma encountered in the clinical diagnosis of CT in children and the pivotal role played by echocardiography in the diagnosis of this anomaly.

Cardiovascular anomalies in Marfan's syndrome: the role of echocardiography and beta-blockers.

Six patients with Marfan's syndrome were included in a study to determine the effect of beta-blockers on progressive aortic dilation. Patients' histories and results of physical examination, chest radiogram, electrocardiogram, and echocardiogram are reported. Patients ranged in age from 5 years 8 months to 14 years; one was black, five were white, and four were male. The study included a brother and sister whose mother had had Marfan's syndrome and died at age 32. All six patients had aortic dilatation documented by echocardiography; two also had mitral valve prolapse. Auscultatory findings were unrevealing, but patients showed easy fatigability. During the prospective, randomized study, half of the patients were given beta-blockers; the other half formed a control group. Findings at short to midterm follow-up suggest that beta-blockers are beneficial in arresting progressive dilatation of the aorta.

Intracardiac fibroma in an asymptomatic infant.

Primary tumors of the heart are rare in infancy and childhood. A one-year-old, asymptomatic, male infant with unimpressive physical findings in whom an echocardiogram demonstrated a large, encapsulated, solid fibroma filling the right ventricle without obstruction of either the inflow or outflow tract is presented. Successful complete surgical excision of the tumor was accomplished. The histological examination was compatible with a benign fibroma. The infant has remained asymptomatic postoperatively with subsidence of the heart murmur. He has not shown any evidence of recurrence of the tumor during a follow-up period of 29 months. This represents an unusual case of a huge right ventricular fibroma and only the fifth youngest patient, to our knowledge, to undergo successful surgical removal.

Cardiac aspect of Cooley's anemia.

The short- and long-term effect of multiple versus regularly spaced blood transfusions on cardiac size, electrocardiographic alterations, heart sounds, murmurs, and carotid pulse were studied in 18 children with Cooley's anemia. These parameters were evaluated before transfusions, one week after multiple blood transfusions (when hemoglobin levels had reached normal), and during the following year, when conventionally spaced transfusions were given. Results showed that enlarged hearts may regress to normal size after multiple transfusions. Chamber hypertrophies and second heart sound were inconstant, and the average of all mean QRS and T vectors was not altered significantly after transfusions. There was good correlation between hemoglobin levels and tachycardia, intensity of first heart sound, heart murmurs, and ejection systolic clicks. Upstroke time of carotid pulse increased with restoration of normal hemoglobin levels. It is concluded that patients maintained at a higher hemoglobin level are generally in better cardiovascular status.