Kincaid, Owings W.

Five patients have had the common features of pulmonary atresia or severe stenosis associated with a septal defect and a coronary artery-to-pulmonary artery (CA-PA) fistula. Four had pulmonary valvular atresia, and one had severe pulmonary stenosis. In all five, the CA-PA fistula contributed the principal component of the pulmonary blood flow. Cyanosis, continuous murmur, right ventricular hypertrophy, and decreased or normal pulmonary vascularity were frequent clinical manifestations, but angiocardiography was required to establish the diagnosis. The fistula was a side-to-side communication between the left coronary artery and the main pulmonary artery in each of the five patients. The dilated proximal coronary artery connecting the aortic root with the pulmonary artery gave a unique angiocardiographic appearance distinguishing it from truncus arteriosus or aorticopulmonary window. It is suggested that this unique angiocardiographic appearance be called an "aorticopulmonary tunnel." The surgical repair of this anomaly is closely related to that for severe tetralogy of Fallot and should involve a comparable risk and achieve a comparable result.

Sixteen patients with diffuse hypertrophic subaortic stenosis have been treated surgically. Thirteen of 14 patients undergoing excision of hypertrophied muscle from the left ventricular outflow tract via left ventriculotomy or aortotomy have survived. Operation has provided excellent symptomatic relief, associated with eradication or significant reduction of the systolic pressure gradient between the left ventricle and the aorta while the patient is at rest. Operation is recommended for those patients with this disease who are experiencing significant and limiting symptoms related to obstruction of the left ventricular outflow tract.

Eighteen cases of stenosis of the branches of the pulmonary artery, seen at the Mayo Clinic, have been reviewed and subdivided into two main types: Type I, stenosis of the main branches of the pulmonary artery, 11 cases, and type II, stenosis of multiple peripheral branches of the pulmonary arteries, seven cases. Of special interest were two cases in which thrombosis occurred as a complication and one case in which surgical relief of multiple areas of stenosis in the peripheral branches of the pulmonary arteries was accomplished. Unexplained pulmonary hypertension necessitates further investigation, which includes cardiac catheterization for measurement of intracardiac pressure and frequently selective angiocardiography. Main branch or peripheral pulmonary artery stenosis should be suspected when a continuous murmur is heard or when poststenotic dilatations of the pulmonary artery branches or areas of decreased pulmonary vasculature can be perceived in the roentgenogram. Angiocardiography is the only technic that permits detailed anatomic evaluation of the entire pulmonary vasculature, especially in the cases associated with severe infundibular or valvular pulmonic stenosis. Stenosis of the branches of the pulmonary artery must be taken into account, when surgical repair of associated cardiovascular defects is considered. These lesions also might complicate anastomotic procedures involving the pulmonary artery in cyanotic patients.

The clinical features and follow-up data on 107 patients who had a clinical diagnosis of aneurysm of the thoracic aorta made at the Mayo Clinic in the period 1945 through 1955 were reviewed. The ratio of male to female was 2.8 to 1, and the average age at the time of diagnosis was 59.3 years. Fusiform aneurysms were four times as frequent as saccular aneurysms; the most common location of the former was the descending thoracic aorta and of the latter the ascending thoracic aorta. Most of the aneurysms (73 per cent) were probably arteriosclerotic in origin. Symptoms, usually pain, and physical signs were present in a minority of patients and indicated a large aneurysm with a poor prognosis. Diastolic hypertension was present in almost half of the patients at the time of diagnosis and had an adverse effect on the patient's survival. Associated arteriosclerotic cardiovascular disease was present in 40 (37 per cent) of the patients at the time of diagnosis; some of these patients had multiple associated cardiovascular lesions. The presence of associated coronary, cerebral, or other peripheral arterial occlusive or aneurysmal disease had the most deleterious effect on survival. The prognosis for patients with large aneurysms was poorer than for those with small aneurysms. Information as to the cause of death was available in 59 (83 per cent) of the 71 patients known to be dead at the time of follow-up. Approximately a third of the deaths were due to rupture of the thoracic aortic aneurysm, and approximately a half, to associated cardiovascular disease, particularly that due to arteriosclerosis. It is believed that this study of patients with untreated aneurysms of the thoracic aorta may be used as a guide to the selection of patients with aneurysm for surgical treatment and may serve as a basis for evaluating the long-term results of such treatment.

Eighteen patients with the combination of ventricular septal defect and aortic insufficiency were studied. Nine also had infundibular pulmonary stenosis. Seventeen were treated by open-heart operations. The physical findings were those of a typical ventricular defect murmur and thrill together with an aortic insufficiency blow and a wide pulse pressure. A systolic murmur at the upper left sternal border with thrill is strongly suggestive of the additional lesion of infundibular pulmonary stenosis, but the presence or absence of infundibular pulmonary stenosis was indicated most accurately at cardiac catheterization and on inspection at operation. It was not of sufficient severity for signs of additional right ventricular hypertrophy to appear on the electrocardiogram. Retrograde aortography serves to demonstrate the severity of the aortic regurgitation. The problem of the surgical correction of these lesions will be the subject of a separate communication.