Citations

Fourteen hearts with tricuspid atresia were reviewed at autopsy. The study included examples of normally related great arteries and of transposition of the great arteries; instances of pulmonary stenosis were present in both groups. In these examples of tricuspid atresia the size of the right ventricle depended on the size of the ventricular septal defect. The ventricular septal defect and the right ventricle usually were smaller in proportion to the size of the heart in specimens with normally related great arteries. In the absence of transposition of the great arteries, the ventricular septal defect was found to be the site of obstruction to pulmonary flow, and its size in relation to the size of the aorta determined the size of the pulmonary artery. Morphologic abnormalities exist in the region in which the proximal portions of the major atrioventricular conduction system (A-V node, bundle of His, origin of bundle branches) normally are located; thus, anatomic abnormalities in the course of the atrioventricular conduction tissue could be expected in tricuspid atresia. Based on histologic evidence, severe elevation of pulmonary vascular resistance, in general, does not occur in infancy in tricuspid atresia.

A total of 696 patients with atrial septal defect (ASD) of either the secundum (90.5%) or the sinus venosus (9.5%) type were operated on at the Mayo Clinic from 1954 through 1965. Pulmonary hypertension (PHT, defined as pulmonary artery systolic pressure of 60 mm Hg or greater) or chronic congestive cardiac failure (CCF) (or both) was associated with 80% of the hospital deaths. For the years 1958 through 1965 (531 cases), the hospital mortality for these three groups was 10% (three of 30), 14% (one of seven), and 18% (two of 11), respectively; only two (0.4%) of the remaining 483 patients died, and both were more than 45 years old (among a total of 93 patients more than 45 years old). There have been no operative deaths among those less than 45 years of age and without PHT or CCF (390 cases) in the years 1958 through 1965. No operative deaths occurred in the years 1963 through 1965. Of 16 patients 60 years or older (1954 through 1965), one has died. A 6- to 12-year follow-up was possible on 397 patients operated on from 1954 through 1960. In the absence of PHT or CCF, 330 survived operation. Among the 53 patients more than 45 years old, four died later (not definitely related to closure of ASD), three were not well, 32 were in excellent health, and 14 were lost to follow-up in 1966. Among the 277 patients less than 45 years old, 10 died later (all but one probably unrelated to closure of ASD), three were unwell, 186 were in excellent health, and 78 were lost to follow-up in 1966. Forty-two patients with PHT survived operation in the period from 1954 through 1960. Ten had pulmonary resistance of more than 640 dynes sec cm -5 ; four of these were well, two had died (with PHT), two had severe PHT, one was in CCF, and one was moderately disabled. Thirty-two had pulmonary resistances of 640 dynes sec cm -5 or less; three of these had died (unrelated to closure of ASD), five were moderately disabled, 16 were in excellent health, and eight were lost to follow-up in 1966. Eighteen patients with CCF survived operation in the period from 1954 through 1960. All had had grade IV disability (New York Heart Association classification). There were three late deaths (one due to severe pulmonary vascular disease and two unrelated to closure of ASD), one patient had grade IV disability, five had grades I to II disability, eight were in excellent health, and one was lost to follow-up in 1966. Paroxysmal arrhythmia occurred in 75 of 397 patients (19%) operated on from 1954 through 1960. In 53 patients it was present preoperatively and persisted postoperatively in 15 of them (28%); in an additional 22 it appeared for the first time after closure of the ASD.

The status of 58 patients, 3 to 57 years of age, with ventricular septal defect and a marked increase in pulmonary vascular resistance (pulmonary obstructive disease) was determined 5 years or longer after diagnostic catheterization. Forty-six (80%) were alive 5 years after that study. The probability of living 5 years was 95% for patients aged 10 through 19 years and 56% for those 20 years of age or older. The dissimilarity of actuarial curves between these two groups is consistent with the hypothesis that pulmonary vascular obstructive disease develops under age 20. Calculated pulmonary resistance increased with age of patient on admission to the study, suggesting its progressive nature. Hemoptysis was rare under age 20, and its presence over this age indicated a poor prognosis. The most common causes of death were "sudden" or "unknown." Most surviving patients were gainfully employed or attending school without severe symptoms.

Left ventricular (LV) performance was studied during mild exercise (100 kg-m/min for 3 minutes) in 19 cases of coronary artery disease (CAD). Each patient suffered from a severe anginal syndrome. None had ventricular aneurysm, cardiomegaly, or clinical heart failure. Mean resting values follow: LV end-diastolic pressure (LVEDP), 19 ± 7 mm Hg; LV systolic pressure (LVSP), 150 ± 16 mm Hg; stroke volume index (SVI), 48 ± 10 ml/beat/m 2 ; and LV stroke work index (LVSWI), 81 ± 19 g-m/m 2 . During exercise LVEDP rose to 30 ± 8 mm Hg ( P ≤0.001) and LVS to 160 ± 20 (not significant [NS]); SVI diminished to 46 ± 11 (NS) and LVSWI, to 76 ± 24 (NS). Patients with decreased or asynchronous areas of LV myocardium identified on cine left ventriculograms had higher mean LVEDPs during exercise than did those with normal ventriculograms (34 ± 10 versus 26 ± 4 mm Hg; P ≤0.05). The difference between responses to exercise of patients who experienced angina during the study and of those who did not was NS. Administration of nitroglycerin reduced the increase in LVEDP during exercise. An abnormality in LV performance-a significant rise in LVEDP without a significant increase in SVI or LVSWI-was observed in 13 of 19 cases of CAD.