Facial resemblance to a patient in whom supravalvular aortic stenosis was discovered and successfully relieved at operation has led to the correct diagnosis of supravalvular stenosis in three other patients. All four patients are mentally subnormal. The presence of supravalvular aortic stenosis in mentally retarded patients with the unusual facial features here detailed may constitute a syndrome that has not previously been described.

Supravalvular aortic stenosis.

Normal ECG values were determined using computer-assisted measurement of the ECGs of 2,141 white children aged 0 to 16 years divided into 12 age groups.

Normal ecg standards for infants and children

Patent ductus arteriosus (PDA) developed in 17 of 111 premature infants (birth weight 1750 g or less) born during a four-year period (15.3 per cent incidence). During that time we treated 29 such infants, 17 born at our own institution and 12 similar infants transferred from other hospitals. Sixteen of the 29 had cardiac catheterization, 10 had operative closure of the PDA, and 24 survived. When the PDA became evident, 23 infants had no pulmonary disease, were recovering from the idiopathic respiratory-distress syndrome (IRDS) or had chronic lung disease; all survived. In six infants with severe IRDS, onset of PDA was associated with a worsening of the pulmonary status; only one survived. We recommend cardiac catheterization and operative closure of the PDA in neonates when heart failure cannot be controlled medically. The prognosis is good in infants in whom onset of PDA is not associated with progressively worsening IRDS.

Patent ducts arteriosus in premature infants. Incidence, relation to pulmonary disease and management.

Congenital fistulas are the most common of the coronary arterial malformations and with the widespread use of selective coronary arteriography are being recognized with increasing frequency. Twenty-eight patients with congenital coronary fistulas have been evaluated at the Duke University Medical Center between 1960 and 1981. An additional 258 patients have previously been reported in the literature, making a total of 286 available for review. The right coronary artery is most commonly involved, and the fistulous communication is most often to the right ventricle, right atrium or pulmonary artery. Slightly more than half of the patients with coronary fistulas are symptomatic at the time the diagnosis is made. Surgical correction is strongly recommended to prevent the development of congestive heart failure, angina, subacute bacterial endocarditis, myocardial infarction, and pulmonary hypertension, as well as coronary aneurysm formation, with subsequent rupture or embolization. There were no operative or late deaths in the patients who underwent operations. Moreover, there have been no recurrent fistulas during a mean follow-up period of ten years. The risks of operative correction appear to be considerably less than the potential for development of serious and potentially fatal complications, even in asymptomatic patients.

Surgical Management of Congenital Coronary Artery Fistulas

Background—T-wave inversion on a 12-lead ECG is usually dismissed in young people as normal persistence of the juvenile pattern of repolarization. However, T-wave inversion is a common ECG abnormality of cardiomyopathies such as hypertrophic cardiomyopathy and arrhythmogenic right ventricular cardiomyopathy, which are leading causes of sudden cardiac death in athletes. We prospectively assessed the prevalence, age relation, and underlying cardiomyopathy of T-wave inversion in children undergoing preparticipation screening. Methods and Results—The study population included 2765 consecutive Italian children (1914 male participants; mean age, 13.9±2.2 years; range 8–18 years) undergoing preparticipation screening including an ECG. Of 229 children (8%) who underwent further evaluation because of positive findings at initial preparticipation screening, 33 (1.2%) were diagnosed with cardiovascular disease. T-wave inversion was recorded in 158 children (5.7%) and was localized in the right precordial leads in 13...

Prevalence of Cardiomyopathy in Italian Asymptomatic Children With Electrocardiographic T-Wave Inversion at Preparticipation Screening

The importance of patent ductus arteriosus in infants.

The importance of positive T waves in the right precordial electrocardiogram during the first year of life.

The electrocardiogram in uncomplicated coarctation of the aorta in infants reflects the pattern of the fetal circulation and serves as a useful prognostic guide. When the fetal ductus inserts proximally to the region of coarctation and closes at birth, right ventricular enlargement is present at birth but is retrogressive. Furthermore, in this situation collateral circulation has begun to develop before birth so that the left ventricle can compensate on assumption of the postnatal circulation, and the prognosis is good. When the insertion of the fetal ductus is distal to the coarctation, progressive, and frequently fatal, left ventricular enlargement and failure develop shortly after birth. Modifications of these electrocardiographic patterns in the production of right bundle branch block are also discussed and illustrated.

The Genesis and Importance of the Electrocardiogram in Coarctation of the Aorta

Triatrial Heart with Persistent Ostium Primum and Cleft Mitral Valve

The determination of the circulation time from the ventricle to the face using fluorescein and ultraviolet light eliminates all of the variable factors from the venous side of the circulation and gives evidence specifically of the presence or absence of an intraventricular right to left shunt. The most important clinical application is the differentiation of tetralogy of Fallot from pulmonary valvular stenosis with an intact ventricular septum, the first requiring a shunting type of operation and the second a pulmonary valvulotomy.

Robert F. Ziegler

Papers

The importance of patent ductus arteriosus in infants.

The importance of positive T waves in the right precordial electrocardiogram during the first year of life.

The Genesis and Importance of the Electrocardiogram in Coarctation of the Aorta

Triatrial Heart with Persistent Ostium Primum and Cleft Mitral Valve

Circulation Time Determinations from the Right Ventricle