Prolapsing atrioventricular valve in partial atrioventricular defect.
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Biomedical subjects
Publications and source records attributed to B F Waller.
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Certain clinical and morphologic cardiac observations are described in 18 necropsy patients, aged 33 to 58 years (mean 45 years) (14 women), with chronic hypercalcemia (11.6 to 34.4 mg/dl [19.4]) from one to nine years (mean five years). Primary hyperparathyroidism was present in nine patients and secondary hyperparathyroidism in the other nine (of renal origin in seven). Cardiac valve anular and coronary arterial calcific deposits were present in 10 patients (Group I) including four (mean age 51 years) with considerable narrowing of two or three of the four major epicardial coronary arteries. None of the other eight patients (Group II) had cardiac valve anular or cuspal calcific deposits; only two had coronary calcific deposits, small in each, and none had significant coronary luminal narrowing. Calcium was in the media ("medial calcinosis"), with or without intimal deposition, of the coronary arteries in five patients. Comparison of the patients in Group I to those in Group II disclosed similar mean ages, durations of hypercalcemia and serum calcium levels, but significantly (p less than 0.05) higher mean total serum cholesterol levels (216 versus 163 mg/dl) and heart weights (426 versus 320 g). This study demonstrates that chronic hypercalcemia is associated with accelerated deposition of calcium in the cardiac anuli and valvular cusps, in the media and intima of the coronary arteries and in individual myocardial fibers (dystrophic calcification), and that coronary intimal calcification may be associated with or produce luminal narrowing, especially in patients with serum total cholesterol levels over 200 mg/dl. Thus, chronic hypercalcemia may be viewed as a "risk factor" to accelerated coronary atherosclerosis.
Certain clinical and necropsy findings are described in 16 young (aged 15 to 33 years) patients who received greater than 3,500 rads to the heart five to 144 months before death. All 16 had some radiation-induced damage to the heart: 15 had thickened pericardia (five of whom had evidence of cardiac tamponade); eight had increased interstitial myocardial fibrosis, particularly in the right ventricle; 12 had fibrous thickening of the mural endocardium and 13 of the valvular endocardium. Except for valvular thickening, the changes were more frequent in the right side of the heart than in the left, presumably because of higher radiation doses to the anterior surface of the heart. In six of the 16 study patients and in one of 10 control subjects, one or more major epicardial coronary arteries were narrowed from 76 to 100 percent in cross-sectional area by atherosclerotic plaque; one patient had a healed myocardial infarct at necropsy and one died suddenly. In 10 patients and in the 10 control subjects, the four major epicardial coronary arteries were examined quantitatively: 6 percent of the 469 five millimeter segments of coronary artery from the patients were narrowed from 76 to 100 percent (controls = 0.2 percent, p = 0.06) and 22 percent were narrowed from 51 to 75 percent (controls = 12 percent). The proximal portion of the arteries in the patients had significantly more narrowing than the distal portions. The arterial plaques in the patients were largely composed of fibrous tissue; the media were frequently replaced by fibrous tissue, and the adventitia were often densely thickened by fibrous tissue. In five patients, there was focal thickening (with or without luminal narrowing) of the intramural coronary arteries. Thus, radiation to the heart may produce a wide spectrum of functional and anatomic changes but particularly damage to the pericardia and the underlying epicardial coronary arteries.
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A patient is described with clinical features of massive pulmonary embolism and a normal pulmonary angiogram. At necropsy, a large thrombus obstructed right ventricular outflow. A right atrial or right ventricular angiogram is suggested in patients suspected of having pulmonary embolism when a pulmonary angiogram shows the pulmonary trunk and main right and left pulmonary arteries to be normal.
Transmural myocardial infarction in the absence of epicardial coronary arterial narrowing is fairly common at necropsy in patients with hypertrophic cardiomyopathy. Although difficult to diagnose clinically in patients with hypertrophic cardiomyopathy, acute myocardial infarction occasionally has a typical presentation, as in the patient presented in this report. More often, however, transmural acute myocardial infarction in patients with hypertrophic cardiomyopathy is not associated with classic signs and symptoms, but leads to production of, or worsening of, congestive cardiac failure which thereafter usually rapidly progresses. Thus, transmural myocardial infarction is a major complication of hypertrophic cardiomyopathy.
A patient is described in whom carcinoma of the lung caused compression and obstruction of the left and right main pulmonary arteries and auscultatory features of peripheral pulmonary arterial stenosis.
In 32 necropsy patients who died within 30 days of an aortocoronary bypass operation performed for relief of angina pectoris, the lumens in 42 (95 percent) of 44 nonbypassed and in 52 (100 percent) or 52 bypassed arteries were narrowed 76 to 100 percent in cross-sectional area by atherosclerotic plaque. Of 616 five mm segments of the 44 nonbypassed arteries examined histologically, 292 (47 percent) were narrowed 76 to 100 percent in cross-sectional area by atherosclerotic plaque; of 728 segments examined in the 52 bypassed arteries, 375 (52 percent) were similarly narrowed. Thirty-two (73 percent) of the 44 nonbypassed coronary arteries (in 23 patients) had been judged to be narrowed 50 percent or less in diameter on preoperative coronary angiography, but at necropsy 31 (97 percent) of these arteries were narrowed 76 to 100 percent in cross-sectional area and the other artery was narrowed 51 to 75 percent. Thus, significant amounts of atherosclerotic plaque tend to be present at necropsy in all three major coronary systems of patients with angina pectoris who die early after an aortocoronary bypass operation.
Clinical and morphologic findings are described in two patients with congenital hypoplasia of portions of both right and left ventricular free walls in the absence of associated coronary or valvular heart disease. One, a 61 year old man who had never had clinical evidence of cardiac dysfunction, died suddenly and unexpectedly. The second, a 55 year old woman, died of progressive, eventually intractable congestive heart failure of 29 months' duration. Although at least 22 necropsy patients have previously been reported to have "parchment-like" thinning of portions of the right ventricular free wall, only one patient has previously been described with such thinning of portions of both right and left ventricular free walls. The spectrum of right or right and left ventricular wall congenital hypoplasia is a broad one, with nearly half of described patients dying of congestive heart failure in the 1st year of life and the other half reaching adulthood with or without manifestations of cardiac dysfunction.
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Clinical and morphologic observations were made in 229 necropsy patients with diabetes mellitus (DM) with onset of diabetes mellitus after 30 years of age--65 without (DM-CHD) and 164 with (DM + CHD) clinical evidence of coronary heart disease (CHD). These observations were compared to those in 183 age-sex-matched nondiabetic control subjects who died from a fatal coronary event (CHD-DM). The average number of three major (right, left anterior descending, left circumflex) coronary arteries per patient narrowed > 75 percent in cross-sectional area by atherosclerotic plaques was identical in the 229 diabetic patients (DM-CHD and DM + CHD) and in the control subjects (CHD-DM), namely, 2.5/3.0. This similarity in the amount of coronary arterial narrowing was present irrespective of the age at onset (after 30 years) or duration of diabetes mellitus. The DM + CHD patients had more severe narrowing of the three major coronary arteries than did the DM-CHD patients (p < 0.01). The amount of severe narrowing in the proximal halves of each of these three arteries was similar to that in the distal halves. The amount of severe (> 75 percent in cross-sectional area) narrowing of the left main coronary artery was greater in the patients with diabetes mellitus than in the nondiabetic controls: 13 percent versus 6 percent (p < 0.01). The type of treatment received by the patients with diabetes mellitus or their adherence to the therapeutic program as measured by the level of random fasting blood sugar did not alter the amount of severe coronary narrowing observed at necropsy.
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Certain clinical and morphologic features are described in three patients who developed severe aortic regurgitation after aortic valve replacement. The regurgitation, fatal in two of the three patients, resulted from long sutures or their knots or both overhanging the metallic ring of the prosthesis preventing complete seating of the occluder. In one patient, simply cutting the overhanging suture eliminated the aortic regurgitation. The problem of suture overhang appears more likely to occur when aortic valve replacement is carried out in patients with relatively small aortic roots. Before closing the aortotomy after aortic valve replacement, it is advisable to move the occluder back and forth to make sure its movement, and specifically, its ability to seat completely, is not limited by sutures which are too long.
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