Florid pulmonary veno-occlusive disease.

A young woman presented with rapidly progressive dyspnea and clinical findings strongly suggestive of primary pulmonary hypertension or possible pulmonary embolism (or both). She died of acute right-sided heart failure. A diagnosis of pulmonary veno-occlusive disease was made at autopsy. Approximately 100 cases of this disease have been reported previously in the literature. We describe a patient with a particularly florid progression of this unusual disease. Death occurred within six weeks of the onset of symptoms.

Rupture of left sinus of Valsalva aneurysm into the pulmonary artery.

Congenital sinus of Valsalva aneurysm is an uncommon lesion that frequently presents after rupture in adult life. This report describes a patient with a left sinus of Valsalva aneurysm that ruptured into the main pulmonary artery, a previously unreported anatomic variant. Anatomic and clinical features of previously reported cases of ruptured sinus of Valsalva aneurysm are reviewed.

Congenital aortic stenosis: ten to 22 years after valvulotomy.

Between 1956 and 1967, 34 patients, aged 2 months to 40 years, underwent aortic valvulotomy under hypothermia for congenital aortic stenosis. There were two early and five late deaths. Twenty-seven patients were followed up for a mean of 15 years. Thirteen patients had no subsequent operation: 11 are asymptomatic, seven with mild aortic insufficiency. Ten patients have had aortic valve replacement (AVR), one revalvulotomy, three will require AVR. Three late deaths were sudden. The literature has been reviewed for data on mortality, endocarditis, aortic insufficiency, and reoperation. Operation improves longevity, but does not restore it to normal. Aortic valve replacement in children carries a poor prognosis, possibly reflecting severity of disease. The chances of reoperation after ten years are 20% to 40%. Valvulotomy must, therefore, be regarded as the first in a possibly lifelong series of operations.

Left stellectomy in the long QT syndrome.

Recent investigations have reaffirmed the role of autonomic innervation of the heart in the genesis of certain cardiac arrhythmias. The long QT syndrome (LQTS) has been described for years, but only recently has evidence of its link to autonomic imbalance been established. A case of LQTS is presented with intraoperative evidence at the time of left stellectomy of life-threatening arrhythmias triggered by stimulation of this neural body. Removal of the left stellate ganglion normalized the electrocardiographic (ECG) abnormalities and has rendered the patient asymptomatic since surgery. Left stellectomy may become definitive therapy for selected patients with the LQTS.

Systemic embolization of the disk occluder of the Wada-Cutter prosthetic valve. A late complication.

The results in 22 patients in the Denver area with Wada-Cutter prostheses were reviewed. There were 14 late deaths, with an average follow-up of six years. Four cases of valve malfunction not related to thrombosis were documented. There were three cases of embolization of the occluder. A survivor is reported. Clinical evaluation of the group suggested new mitral regurgitation to be a sensitive indicator of impending embolization of the occluder. Documentation of valve malfunction warrants valvular replacement.

Homograft and prosthetic aortic valve replacement: a comparative study.

Homograft aortic valve replacement was done in 103 patients and prosthetic aortic valve replacement in 106 between January 1962 and December 1973. Patients who received homograft and prosthetic valves were compared with respect to age, sex, preoperative functional impairment, infection, dyspnea, angina, hemodynamics, chest X-ray, electrocardiogram, associated operations, early and late mortality, and valve failure. Combined total mortality was 28% (12% operative, 8% first postoperative year, 8% late). Ten percent of valve required replacement. One year after operation, 70% of survivors were asymptomatic, 27% were improved, and 3% were unchanged or between homograft and prosthetic valve replacement. Valve-related failure and infections were more common after homograft aortic valve replacement. Emboli, hemorrhage, and hemolysis were commoner after prosthetic valve replacement. Fungal infections occurred in five homograft patients but in no patient with a prosthetic aortic valve. Severe properative symptoms or recent endocarditis was associated with greater mortality and valve failure in both the homograft and the prosthetic series. Increased mortality and failure was also seen in patients with either preoperative aortic regurgitation with high left ventricular end-diastolic pressure and low cardiac index, or aortic stenosis with cardiomegaly or roentgenographic evidence of congestive heart failure. Therefore, in two series of patients at equal risk, mortality and valve failure were similar for homograft and prosthetic aortic valve replacement.

Effects of ouabain on early ventricular relaxation in patients with acute myocardial infarction.

The effects of the administration of ouabain on the peak negative left ventriculat (LV) dP/dt were studied in 14 patients with acute myocardial infarction. Prior to administration of ouabain, negative LV dP/at was lower than previously reported for patients without coronary arterial disease. One hour after administration of ouabain, significant (P less than 0.05) increases in peak positive LV dP/dt and maximal velocity of contractile-element shortening and decreases in LV diastolic pressure were present. These changes were associated with small and insignificant increases in negative LV dP/dt. Early LV relaxation rate is impaired in patients with acute myocardial infarction; however, the reduction in LV diastolic pressures which occurs after administration of ouabain does not appear to be due to an increase in the rate of early LV relaxation but rather is probably related to improved contractility.

The heritable syndrome of prolonged Q-T interval, syncope, and sudden death. Electron microscopic observation.

A family with the heritable syndrome of prolonged Q-T interval is reported. Three members died suddenly. Six had a prolonged Q-T interval. One had syncopal episodes for four years but has had no syncope since therapy with propranolol was initiated two years ago. The inheritance appears to be autosomal dominant. There was no evidence of hearing defects. Histologic and electron microscopic studies were performed on cardiac tissue from one patient. This is the first report of electron microscopic studies, and the findings suggest a possible defect of calcium metabolism in the myofiber.

Echocardiography in aortic root dissection and dilatation.

Six patients with aortic root dissection proved by angiography, surgery or autopsy, and six patients with aortic root dilatation were studied by echocardiography. Echocardiography was diagnostic in five or six patients with dissection and suggestive in the sixth, disclosing anterior and posterior dissection in three, anterior dissection in one and posterior dissection in one. The recording of a double echo in the aorta was the diagnostic feature. Angiography was diagnostic in four of the six patients, yielded a false negative result in one and was not performed in one. Six patients with dilatation had an enlarged aortic root by echocardiography. Left ventricular size, stroke volume, ejection fraction, aortic regurgitant flow and velocity of circumferential fiber shortening were calculated in 11 patients. Echocardiography was extremely helpful in the diagnosis, management and follow-up in patients with aortic dissection or dilatation.