D. Luke Glancy

Heart in malignant lymphoma (Hodgkin's disease, lymphosarcoma, reticulum cell sarcoma and mycosis fungoides): A study of 196 autopsy cases∗

Abstract Of 196 patients with malignant lymphoma studied at autopsy, 48 (24 per cent) had lymphoma involving the heart. In 27 subjects the cardiac lymphoma was observed on gross examination, and in the other 21 it was found only on study of histologic sections. Lymphoma in the heart occurred most frequently in mycosis fungoides (33 per cent) and least frequently in Hodgkins disease (16 per cent). Cardiac lymphoma was found in approximately 25 per cent of patients with lymphosarcoma, reticulum cell sarcoma and undifferentiated or mixed cell malignant lymphoma. The cardiac metastases when observed grossly were usually firm white focal nodules located most frequently in the pericardium but also often in the walls of the chambers. In 9 patients the cardiac lymphoma extended through the wall of a cardiac chamber or septum. The incidence of dyspnea, chest pain, effusions into body cavities, precordial murmurs, ventricular gallops, edema and electrocardiographic disturbances was similar in patients with and without cardiac lymphoma, and these clinical findings most often were the result of mediastinal, pleural or pulmonary lymphoma, anemia, hypoalbuminemia, or an underlying cardiac condition. Signs or symptoms of cardiac dysfunction could be attributed to lymphomatous involvement of the heart in only 5 of the 48 patients with cardiac lymphoma. Three of them had mycosis fungoides, and 2, Hodgkins disease.

The heart in malignant melanoma. A study of 70 autopsy cases.

Abstract Cardiac metastases were found in 45 of 70 patients (64 per cent) dying of metastatic melanoma. Of the 70 patients, 11 had significant cardiac dysfunction clinically. Tumor implants in the heart did not appear to cause dysfunction unless cardiac involvement by tumor was extensive. Many previously proposed criteria for clinically determining the presence of cardiac metastases were found to be unreliable.

Cardiac Surgery for Grown-Up Congenital Heart Patients: Survey of 307 Consecutive Operations from 1991 to 1994

The cardiac surgery performed from 1991 to 1994 in a unit dedicated specifically for grown-up congenital heart (GUCH) patients was reviewed to determine the frequency of various procedures, incidence of first and reoperations, early mortality, and its determinants. The 295 patients, aged 16 to 77 years (31 +/- 13), had 307 operations. First operations (n = 128, 42%) were most commonly for closure of atrial septal defect (n = 40), aortic valve replacement (n = 31) or repair of aortic coarctation (n = 14). Reoperations were more frequent (n = 179, 58%) and divided among first corrective repair (n = 49), reoperation after corrective repair (n = 115), and further palliation (n = 15). First corrective surgery was mainly for aortic valve disease (n = 17), Fallot (n = 7), and lesions needing a Fontan procedure (n = 5). Reoperations after corrective repair were needed for aortic valve disease (n = 43), right-sided conduit (n = 30), or recoarctation (n = 11). Early mortality was influenced by presence of central cyanosis (9 of 49, 18% in cyanotic patients; 12 of 258, 5% in acyanotic; p <0.001), increased number of previous operations (0 = 4%, 1 = 7%, 2 = 11%, >2 = 13%; p = 0.003), and increasing age of patients. Cyanotic patients had more serious postoperative complications: pleural and pericardial effusions, severe bleeding, renal insufficiency, and sepsis, and their hospital stay was longer compared with acyanotic patients (20 +/- 17 vs 11 +/- 8 days; p <0.001). In GUCH patients, reoperations cause the largest demand on cardiac surgical services. Increased survival of patients with complex cardiovascular malformations brings difficult challenges not only to cardiologists but also to cardiovascular surgeons. There is a need to provide continued highly specialized care. Resources, patients, and funding should be concentrated in a few designated centers.

Hemodynamic Accompaniments of Angina A Comparison During Angina Induced by Exercise and by Atrial Pacing

The hemodynamic responses of nine patients with severe coronary artery disease were studied during the precipitation of angina by both supine exercise and increasing rates of atrial pacing. Tension-time index and the first derivative of left ventricular pressure pulse (LV dp/dt) at the onset of angina were significantly higher (P<0.01) in each patient when angina was induced by exercise than when angina was provoked by atrial pacing. Heart rate, in contrast, was significantly greater (P<0.05) when angina was precipitated by atrial pacing. Left ventricular end-diastolic pressure (LVEDP) was abnormally elevated in each patient when angina occurred during supine exercise, whereas LVEDP was normal in all patients at the onset of angina provoked by atrial pacing. On the basis of these results it appears that the hemodynamic accompaniments of angina depend to a large extent on the particular circumstances leading to the development of angina. Tension-time index, LV dp/dt, and heart rate are major determinants of myocardial oxygen consumption, and the interrelationships between the determinants of myocardial oxygen consumption are complicated. Thus, changes in any one of these determinants after a therapeutic intervention must be viewed in relation to possible changes in the others.

Indices Predicting Long-term Survival after Valve Replacement in Patients with Aortic Regurgitation and Patients with Aortic Stenosis

The long-term results of aortic valve replacement were reviewed in all 88 patients with isolated aortic regurgitation and all 103 patients with isolated aortic stenosis who were operated upon at the National Heart and Lung Institute from 1963 to 1971. Survival curves were compared to determine whether any of 30 preoperative clinical and hemodynamic findings correlated with long-term survival. The indices that were of predictive value in patients with aortic regurgitation were found to be different from those in aortic stenosis. Symptoms, cardiac index, and cardiothoracic ratio did not influence survival in patients with aortic regurgitation. In these patients, survival was inversely correlated with the level of left ventricular end-diastolic pressure (LVEDP): six-year survival was 74% in patients with LVEDP ≤ 10 mm Hg, 41% with LVEDP 11-20 (P < .05), and 30% with LVEDP > 20 (P < .01). Survival also was lower in patients with aortic regurgitation who had elevated pulmonary arterial and left atrial pressures, and in patients with electrocardiographic evidence of severe left ventricular hypertrophy (LVH). Using an LVH point score method (Romhilt-Estes), 56% of patients with a score ≤ 6 survived six years; 29% with a score > 6 survived (P < .02). Survival in aortic stenosis did not relate to any of the above, but did correlate with preoperative functional class. Five-year survival was 70% in class II, but only 40% in class III-IV (P < .02). Moreover, cardiothoracic ratio in patients with aortic stenosis correlated with survival in an unexpected way. Eleven of 31 patients with cardiothoracic ratio ≤ .45 had sudden unexplained death postoperatively, compared to only six of 72 patients with cardiothoracic ratio > .45 (P < .01). This difference did not correlate with postoperative hemodynamic measurements, including magnitude of the transprosthetic gradient. We conclude that certain preoperative indices are of value in predicting long-term prognosis after valve replacement for aortic regurgitation and for aortic stenosis, but that the specific predictive indices for the two groups differ.

Coronary Arterial-Right Heart Fistulae Long-Term Observations in Seven Patients

Long-term follow-up is described of seven patients with fistulae between a coronary artery and the right atrium or right ventricle. Left-to-right shunt flow ranged from minimal to 2.2:1. Of six patients followed 3½ to 17 years (average 10) without operation, five demonstrated symptomatic, electrocardiographic, hemodynamic, and angiographic stability. In the sixth patient, a second angiographic study, performed 15 years after the first one, showed the right coronary artery to be occluded proximal to its fistulous communication with the right ventricle, and a left-to-right shunt could no longer be detected. Four of the seven patients underwent operative closure of a fistulous opening into the right atrium, and all four have been restudied postoperatively. Right heart pressures and the degree of dilatation of the involved coronary artery were essentially unchanged following operation. One patient, who had a moderate-sized shunt preoperatively, noted alleviation of her fatigue and demonstrated electrocardiographic improvement. Analysis of flow dynamics did not suggest that the shunt predisposed to shear-induced intimal damage of the dilated feeding coronary artery, but did suggest such changes might occur in the narrow fistulous communication. We conclude that little anatomic and functional change occurs in patients with coronary artery fistulae and small-to-moderate shunts over rather prolonged medical follow-up periods, and that operative closure does not reduce the size of the dilated proximal coronary artery. Since it is unclear whether the abnormality predisposes to premature coronary atherosclerosis, a better understanding of the natural history of the disease is necessary before the precise role of operation in patients with small-to-moderate shunts can be defined.

Myocardial Infarction in Young Women with Normal Coronary Arteriograms

Two women, ages 34 and 36 years, suffered an acute transmural anterior myocardial infarction accompanied by typical ECG and serum enzyme changes. At the time of selective cinecoronary arteriography 29 and 57 months later, however, neither had demonstrable narrowing of any coronary artery. Both have persistent ECG changes of an anterior infarct and markedly diminished contractions of a large segment of the anterior wall and apex on left ventriculogram. Neither before nor after infarction has either patient experienced angina pectoris. Both patients are premenopausal, and neither of them has diabetes, hypertension, obesity, valvular heart disease, a lipoprotein abnormality, or a family history of premature coronary arterial disease. Both smoke cigarettes. At the time of infarction one patient was taking an oral contraceptive and the other was 11-days postpartum. The pathogenesis of the myocardial infarcts in these patients is unknown. Embolization or in situ thrombosis of a previously normal anterior descending coronary artery with subsequent clot lysis would explain the infarcts, the normal coronary arteriograms, and the absence of prior or subsequent angina.

Deterioration of Myocardial Function Following Aorto-Coronary Bypass Operation

Twenty-two patients underwent cardiac catheterization before and an average of five months after aorto-coronary bypass operation (ACBO). Two groups were examined: 10 patients with all grafts patent, and 12 patients with one or more grafts occluded. All patients improved symptomatically, regardless of graft patency. However, in the occluded group, left ventricular end-diastolic pressure (LVEDP) increased (4.4 ± 2.2 mm Hg, P < 0.05), stroke volume index fell (9.8 ± 3.1 ml/m2, P < 0.05), ejection fraction decreased (10 ± 4%, P < 0.05), and left ventricular stroke work index fell (12 ± 3 g-m/m2, P < 0.01).Qualitative analysis of segmental left ventricular contractility was performed. Of 28 segments supplied by patent grafts, six improved and nine deteriorated. Of 22 segments supplied by occluded grafts, none improved and eight deteriorated. Frequently no angiographically demonstrable basis for the segmental deterioration was evident.We concluded that while ACBO may appreciably benefit severely symptomatic patients, our results do not substantiate the claim that ACBO should be recommended when the primary surgical goal is preservation or enhancement of myocardial function.

Mitral restenosis: An uncommon cause of recurrent symptoms following mitral commissurotomy

Abstract Of 226 patients undergoing mitral commissurotomy, 163 had both preoperative and early postoperative cardiac catheterizations. Forty-five of these 163 patients had a late postoperative catheterization because of persistent or recurrent symptoms. Restenosis of the mitral valve after successful commissurotomy was found in 5 of the 45 patients. Commoner causes of residual or recurrent symptoms were residual mitral stenosis (16 patients), residual stenosis and operatively induced mitral regurgitation (15 patients) and cardiac abnormalities unrelated to the mitral valve (9 patients). Thus, mitral restenosis has been documented by left heart catheterization for the first time, but restenosis has been found to be an uncommon cause of symptomatic deterioration after mitral commissurotomy.

Juxtaductal aortic coarctation. Analysis of 84 patients studied hemodynamically, angiographically, and morphologically after age 1 year.

Although many studies of juxtaductal coarctation of the aorta have been reported, none has correlated clinical, hemodynamic, angiographic, anatomic, and operative findings. Of 84 patients (62 male and 22 female; age range, 1 to 49 years [mean 17]), all had murmurs; 76 had absent, diminished, or delayed femoral pulsations; 50 had cuff systolic blood pressures in the arm greater than 140 mm Hg, and 30 had diastolic pressures greater than 90 mm Hg. The average pressure gradients (mm Hg) by direct measurements above and below the coarctation in 35 patients were peak systolic, 45; mean, 17; and diastolic, 5. Rib notching, visible in chest roentgenograms in 43 patients, correlated directly with age and inversely with the diameter of the coarctation. Moderate or marked cardiomegaly by radiograph was present in only 1 of 48 patients with isolated coarctation and in 17 of 36 with associated cardiovascular malformations. Electrocardiograms were abnormal in more than two thirds of patients with associated anomalies, but were normal in more than three fourths of those with isolated coarctation. In 70 excised, serially sectioned coarctations the aortic lumens were completely occluded in 4 patients, up to 0.5 mm in internal diameter in 22 patients, from 0.6 to 2 mm in 26 patients, from 2.1 to 5 mm in 14, and greater than 5 mm in 4, and correlated directly with lumens measured angiographically. The most significant anatomic factor causing the coarctation was invagination of the media from the posterior aortic wall, but intimal proliferation (jet lesion) at and immediately distal to the invagination contributed to the narrowing. Three (each with associated anomalies) of 70 patients died early after coarctation repair. Systolic or diastolic blood pressures decreased early postoperatively in 58 (87%) of 67 surviving patients, and both pressures decreased in 42 (63%). Late postoperatively (mean follow-up, 4.7 years), the systolic blood pressure remained elevated in 25% of patients.