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Biomedical subjects

A Simcha

Publications and source records attributed to A Simcha.

At least 19 recordsLinked to original sources

Prenatal diagnosis of tetralogy of Fallot with absence of the leaflets of the pulmonic valve.

Tetralogy of Fallot with absence of the leaflets of the pulmonic valve is relatively rare. We describe the prenatal diagnosis of this malformation in two fetuses. The association between the agenesis of the arterial duct and absence of the valvar leaflets is discussed. The diagnosis of the syndrome in the presence of dilated and pulsatile pulmonic arteries is easy to make. It should allow early counselling and planning of early care after delivery.

Abnormalities, Multiple↗

Intracardiac causes of superior vena cava obstruction.

Most causes of superior vena cava (SVC) obstruction are extracardiac. In rare instances, an intracardiac process may obstruct the venous return from the SVC. This is illustrated by a report of a patient with a congenital coronary artery fistula to the right atrium obstructing the SVC return. We propose a classification and clinical approach to the differential diagnosis of SVC obstruction.

Child, Preschool↗

An unusual presentation of right coronary artery fistula.

A four year old girl with infective endocarditis had unexplained facial swelling. Cross sectional echocardiography showed that a large right coronary artery fistula to the right atrium was obstructing the distal superior vena cava. The diagnosis was confirmed by cardiac catheterisation and at operation. The child was symptom free one year after operation.

Child, Preschool↗

Severe hydrops fetalis due to congenital supraventricular tachycardia.

Intrauterine supraventricular tachycardia is one of the main causes of non-immunological intrauterine hydrops fetalis. Without early diagnosis and treatment it may terminate in fetal death or delivery of a baby with severe hydrops and extreme heart failure. With the improvement in non-invasive imaging techniques in prenatal medicine, this condition can be diagnosed early and treated successfully. A case is described of such a newborn diagnosed in utero and treated before and after delivery, and the different therapeutic approaches to congenital supraventricular tachycardia are discussed.

Adult↗

Symptomatic sinus bradycardia in infants with structurally normal hearts.

Sixteen infants, ages 6 weeks to 23 months, were evaluated because of syncopal attacks (n = 6), apneic episodes (n = 3), or a combination of syncope, apnea, or "convulsions" (n = 7). Comprehensive metabolic and neurologic investigations yielded negative results in 15 infants; in one, an abnormal EEG was recorded. Severe sinus bradycardia, complete sinus arrest, junctional escape rhythm, or ventricular escape rhythm was found in four patients, and permanent multiprogrammable pacemakers were implanted. Follow-up for 6 to 24 months with ventricular demand pacing showed complete cessation of attacks in two infants and marked improvement in two.

Apnea↗

Echocardiographic diagnosis of pulmonary atresia with intact ventricular septum.

The echocardiographic features of pulmonary valvar atresia with intact ventricular septum are described. The pulmonary valve fails to open during systole: there is a large pre-systolic 'a' wave dip, but the pulmonary valve membrane then returns to its closed diastolic position where it remains for the remainder of the cardiac cycle. The echocardiogram also provides valuable information about right ventricular size, the tricuspid valve, and aorta-mitral and aorta-septal relations.

Cardiomegaly↗

Familial Ebstein's anomaly.

A family is described in which both a father and son are affected with Ebstein's anomaly, while several other family members manifest different cardiac malformations. Five additional instances of familial Ebstein's anomaly were found in the literature and compared with our family. Inspection of possible modes of inheritance in this group of families suggests that Ebstein's anomaly is probably inherited as a polygenic character with a threshold phenomenon.

Adult↗