Shyamal K. Sanyal

Moraxella endocarditis following balloon angioplasty of aortic coarctation

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Right atrial myxoma in infancy and childhood

Abstract The case of a 7 month old infant with antemortem diagnosis and successful surgical removal of a right atrial myxoma has been presented. The clinical manifestations may be vague and generalized, embolic or primarily obstructive in nature. The presence of anemia, leukocytosis, changing heart murmur and intermittent pyrexia may suggest subacute bacterial endocarditis. Angiocardiography provides a definite diagnosis. Once the diagnosis is established, surgical removal is indicated.

Superiorly oriented electrocardiographic axis in infants with the rubella syndrome

Abstract An unusual superiorly oriented mean electrical axis has been described in 12 of a series of 23 infants with congenital heart disease associated with the rubella syndrome. The majority of infants had a patent ductus arteriosus or peripheral pulmonic stenosis. The electrical axis did not correlate with the anatomic diagnosis of ventricular hypertrophy, and the possibility that this axis may represent a conduction disturbance is discussed.

CONGENITAL INSUFFICIENCY OF THE PULMONARY VALVE.

Congenital insufficiency of the pulmonary valve, whether an isolated entity or associated with other intracardiac defects, is rare. The clinical picture varies from one of total absence of symptoms to one in which signs of congestive cardiac failure occur during the neonatal period. One case is presented with 3 normal cusps and a lack of valvular tissue posteriorly through which regurgitation occurred. Such an anatomic variation has not been recorded previously. The characteristic clinical features are discussed as well as roentgenographic findings, electrocardiographic tracings, and cardiac catheterization data which may be of help in making the diagnosis.

Jervell and Lange-Nielsen syndrome in a father and daughter from a large highly inbred family: a 16-year follow-up of 59 living members.

OBJECTIVE To report the autosomal dominant inheritance of the Jervell and Lange-Nielsen syndrome in a highly inbred family, the initiation of Torsades de Pointes, and the natural history of the syndrome based on a 16-year follow-up of the kindred. METHOD A family tree was constructed that included 66 blood relatives from three successive generations. Electrocardiograms were obtained from 59 living members including the proband, four members from a nuclear family, and 54 from the extended family. Evoked response audiometry was recorded for the proband and the nuclear family. All 59 family members were followed up regularly for 16 years. RESULTS A total of 24 living members were affected--QTc: 480-680 ms. The proband had long QTc, bilateral high-tone sensorineural deafness, recurrent syncope, and Torsades de Pointes. The asymptomatic father had long QTc and unilateral high-tone sensorineural deafness that involved specifically the left ear. One asymptomatic sibling of the proband had long QTc and normal hearing. The mother and another sibling were asymptomatic; QTc and hearing were normal in both. A total of 21 affected members from the extended family had only long QTc, and all were asymptomatic. There were three congenitally deaf first cousins who had recurrent syncope and adrenergic-triggered sudden death. In all, seven of 10 parents had consanguineous marriage to a first cousin. Each affected offspring had at least one affected parent. The severely symptomatic proband who received only β-blocker therapy and the 23 affected members without antiadrenergic therapy, all remained asymptomatic throughout the 16-year follow-up period. CONCLUSION Jervell and Lange-Nielsen syndrome was inherited as autosomal dominant in this kindred. The majority of the affected members had a mild phenotype. The severity of auditory and cardiac phenotypes corresponded.

Differential cyanosis in congenital heart disease

Three cases are described to illustrate various clinical patterns of differential cyanosis.The prerequisite conditions responsible for the various types of differential cyanosis are persistent ductus arteriosus and severe pulmonary arterial hypertension. The hemodynamic pathways and other factors affecting several types of differential cyanosis are discussed.