J.Russell Green

Fibromyxosarcoma of the pulmonary artery: Associated with syncope, intractable heart failure, polycythemia and thrombocytopenia

Abstract This case represents the third reported case of a fibromyxosarcoma of the pulmonary artery. The diagnosis of pulmonary artery obstruction may be confused with primary pulmonary hypertension, atrial myxoma and mitral valve obstruction. The rapid development of intractable right heart failure, syncopal attacks, soft to absent pulmonic sound and fluoroscopic evidence of differential pulmonary artery pulsations without left atrial enlargement suggest the correct diagnosis. Cardiac catheterization and angiography are important adjuncts to the diagnosis. A syndrome comprised of intractable right ventricular failure, recurrent syncope, polycythemia and thrombocytopenia is suggested as being secondary to obstruction of the pulmonary artery.

Familial Ebstein's anomaly of the tricuspid valve

Abstract Ebsteins anomaly of the tricuspid valve has not been reported previously in two members of the same family. A 6-year-old boy and his 29-year-old maternal uncle are described, both of whom had the Ebstein type of tricuspid valve documented by cardiac catheterization. A review of the literature suggests that when multiple cases of congenital heart disease occur in a family in which one individual has Ebsteins malformation of the tricuspid valve, another member may have the same anomaly; or even more likely, an analagous anomaly of the left-sided atrioventricular valve with or without congenitally corrected transposition of the great vessels.

Congenital heart disease in an adult with the Ellis-van Creveld syndrome.

Excerpt McIntosh (1) in 1933 described a patient who presented with ectodermal dysplasia, polydactyly, and chondrodysplasia. In 1940 Ellis and van Creveld (2) described 2 more patients with similar...

Idiopathic dilatation of the pulmonary artery

Abstract Further delineation of the natural history of patients having idiopathic dilatation of the pulmonary artery was accomplished by studying 15 patients for periods varying from two to six years. The clinical, laboratory and catheterization data of these patients are presented. Criteria for the diagnosis of idiopathic dilatation of the pulmonary artery included (1) the absence of intracardiac shunts or acquired disease of the cardiovascular or pulmonary systems; (2) a right ventricular systolic pressure of 30 mm. Hg or less; and (3) a systolic pressure difference across the pulmonic valve of 15 mm. Hg or less during stress. Twelve of 15 (80%) of the patients had associated pulmonic insufficiency. In 4 pulmonic valvular insufficiency developed during the period of observation; 3 of these patients had elevated right and left end-diastolic pressures at the time of recatheterization which could not be explained on the basis of rhythm, heart size, or myocardial function. All patients had a normal response to stress, with appropriate increases in stroke work. It is believed that these elevated end-diastolic pressures may reflect a change in ventricular compliance or may represent values which may occur normally at the upper end of “normal” end-diastolic pressures. The data presented indicate the need for serial long term follow-up studies of patients with this anomaly.

A Pathological Basis for Sudden Death in Eight Generations.

Excerpt The sudden death of a 16-year-old boy and his 14-year-old sister led to the establishment of a family pedigree encompassing eight generations with historical details on 127 members, and to ...