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Biomedical subjects

J T Lie

Publications and source records attributed to J T Lie.

At least 199 records · Page 11Linked to original sources

Triple-barrel aorta: dissection of a healed aortic dissection.

An unusual case of a triple-barrel aorta in a 51-year-old woman is described. The patient first had a spontaneous type I dissection of the aorta and acute aortic insufficiency, for which she underwent aortic valve replacement and Dacron graft replacement of the ascending aorta. She remained asymptomatic for five years with a healed aortic dissection (double-barrel aorta) distal to the graft. She then underwent a second operation for repair and poppet replacement of the malfunctioned prosthesis. Postoperative cardiac failure necessitated the use of a counterpulsation intra-aortic balloon catheter, which entered and dissected the wall of the false lumen, thus creating a triple-lumen aorta.

Aortic Dissection↗

Hypertrophic cardiomyopathy in infants: clinical features and natural history.

The clinical and morphologic features of hypertrophic cardiomyopathy in 20 patients recognized as having cardiac disease in the first year of life are described. Fourteen of these 20 infants were initially suspected of having heart disease solely because a heart murmur was identified. However, the infants showed a variety of clinical findings, including signs of marked congestive heart failure (in the presence of non-dilated ventricular cavities and normal or increased left ventricular contractility) and substantial cardiac enlargement on chest radiograph. Other findings were markedly different from those usually present in older children and adults with hypertrophic cardiomyopathy (e.g., right ventricular hypertrophy on the ECG and cyanosis). Consequently, in 14 infants, the initial clinical diagnosis was congenital cardiac malformation other than hypertrophic cardiomyopathy. Twelve of the 14 infants who underwent left-heart catheterization showed substantial obstruction to left ventricular outflow (peak systolic pressure gradient greater than or equal to 35 mm Hg). However, unlike older patients with hypertrophic cardiomyopathy, infants with this condition commonly had marked obstruction to right ventricular outflow (35-106 mm Hg) (nine patients); in six patients, the magnitude of obstruction to right ventricular outflow was at least as great as that to left ventricular outflow. Asymmetric hypertrophy of the ventricular septum relative to the left ventricular free wall was present in the 16 patients who had echocardiographic or necropsy examination. Ventricular septal thickening was substantial in patients studied both before and after 6 months of age (mean 16 mm), indicating that in patients with hypertrophic cardiomyopathy, marked left ventricular hypertrophy may be present early in life and is probably congenital. The clinical course was variable in these patients, but the onset of marked congestive heart failure in the first year of life appeared to be an unfavorable prognostic sign; nine of the 11 infants with congestive heart failure died within the first year of life. In infants with hypertrophic cardiomyopathy, unlike older children and adults with this condition, sudden death was less common (two patients) than death due to progressive congestive heart failure.

Angiocardiography↗

Selenium deficiency and fatal cardiomyopathy in a patient on home parenteral nutrition.

An adult patient with chronic idiopathic intestinal pseudo-obstruction maintained on home parenteral nutrition for 6 consecutive years died from cardiomyopathy and ventricular fibrillation. Postmortem examination of the heart revealed widespread myocytolysis and replacement fibrosis similar to that seen in the selenium deficient cardiomyopathy in China (Keshan disease) and animal models. Selenium deficiency in this patient was documented with extremely low concentrations of selenium and decreased activity of the selenoprotein, glutathione peroxidase, in blood, heart, liver, and skeletal muscle. Reports of selenium deficient diets causing myocardial damage in humans and animals and the findings in our patient strongly suggest that his fatal cardiomyopathy was caused by selenium deficiency.

Adult↗

Thoracic aortic aneurysms: a population-based study.

Thoracic aortic aneurysms were detected in 72 residents (44 women and 28 men) in a stable midwestern community over a 30-year period, for an age- and sex-adjusted incidence of 5.9 new aneurysms per 100,000 person-years. The incidence was equal in both sexes and decreased slightly over the 30 years. Ages ranged from 47 to 93 years (median 65 years for men and 77 years for women). The ascending aorta was involved in 37 patients, the aortic arch in 8, and the descending aorta in 27. Pathologic examination was performed in 51 patients. The cause was aortic dissection in 27 patients (53%), atherosclerosis in 15 (29%), aortitis in 4 (8%), cystic medial necrosis in 3 (6%), and syphilis in 2 (4%). All autopsied patients had pathologic evidence of significant hypertension. Eleven patients (25%) had concomitant abdominal aortic aneurysms. Rupture occurred in 53 patients (74%) and 50 died. Thirty-seven of these patients had no prior diagnosis of aneurysm. The median interval between diagnosis and rupture in the 16 remaining patients was 2 years (range 1 month to 16 years). Ninety-five percent of aortic dissections ruptured and 51% of nondissecting aneurysms ruptured. The actuarial 5-year survival for all 72 patients was 13%; for patients with aortic dissection, 7% and for patients without dissection, 19.2%.

Actuarial Analysis↗

Reduction of thrombosis in canine coronary bypass vein grafts with dipyridamole and aspirin.

The potential benefit of platelet inhibitor drugs in reducing early thrombosis of coronary arterial vein bypass grafts was assessed in dogs. There were 26 control dogs and 24 dogs treated with dipyridamole, 55 mg/day plus aspirin, 325 mg/day. The dogs in both groups were killed at 2 hours and 1, 2, 3, 7 and 14 days after operation. The grafts were perfused with fixative in vivo, harvested and examined with light microscopy. Severe alterations of the graft wall were observed in the dogs in both groups. The grafts in the control group had a high incidence rate of thrombosis, which occurred early after the operation; those in the treated group had a significantly reduced incidence of thrombosis (p = 0.025). Our study indicates that a combined regimen of dipyridamole and aspirin is effective in reducing early graft thrombosis in dogs.

Animals↗

Pulmonary hypertension in polymyositis.

Pulmonary hypertension is an uncommon but lethal complication of polymyositis. A 69-year-old woman is described who had a 20-month history of polymyositis and cardiac failure and in whom severe pulmonary hypertension was noted terminally when she was first seen at our clinic. She died within 24 hours of admission, and autopsy revealed severe plexogenic pulmonary vascular disease.

Aged↗

Prevalence of clinically unsuspected pheochromocytoma. Review of a 50-year autopsy series.

Pheochromocytoma is surgically curable, and lethal complications often ensue when the diagnosis is not made. We review 54 autopsy-proven cases of pheochromocytoma seen at the Mayo Clinic over the 50-year period 1928-1977. There were 31 females and 23 males, and the patients' ages ranged from 40 hours to 92 years (mean, 53 years). Pheochromocytoma was multiple in 10 patients (19%), extra-adrenal in 5 (9%), and malignant in 6 (11%). In 13 (24%) pheochromocytoma had been correctly diagnosed in life--after the investigation of hypertension in 8 patients, incidentally at laparotomy for unrelated conditions in 4 patients, and in association with the multiple endocrine neoplasia syndrome in 1 patient. In the group of 41 patients (76%) in whom pheochromocytoma had not been suspected clinically, hypertension also was a common finding (22 patients, 54%). Symptoms such as headache, sweating, and palpitations were nonspecific, but all occurred more often among the group of patients in whom the diagnosis had been made. For both groups, hypertensive or hypotensive or hypotensive crisis precipitated by surgery for unrelated conditions was a common cause of death. Prevention of such deaths requires a high degree of clinical alertness and biochemical screening tests for pheochromocytoma in patients with labile or accelerated hypertension.

Adolescent↗

Scanning electron microscopy of intracardiac myxoma.

Scanning electron microscopy was utilized to study the surface morphology of intracardiac myxomas and to compare it with that of an atrial thrombus. The thrombus was only partially endothelialized, whereas myxomas were almost always completely covered by endothelial cells. Endothelium-lined crevices and clefts were a striking feature of the myxoma, and they were not seen in the atrial thrombus. In areas where the surface endothelium was denuded, fibrin and platelet aggregates were noted in both the myxoma and the thrombus.

Heart Atria↗

False aneurysm of the left coronary artery.

The case of a 65-year-old white man with a large false aneurysm of the left coronary artery resulting from spontaneous rupture of the circumflex coronary artery is described. An abnormal chest roentgenogram resulted in initial evaluation, and a coronary aneurysm was then suspected on the basis of two-dimensional echocardiography. Cardiac catheterization demonstrated a large, saccular false aneurysm arising from the left coronary artery and diffuse aneurysmal disease of the right coronary artery. The patient underwent surgical resection of the aneurysm and saphenous vein aorta-coronary bypass grafting but failed to survive the postoperative period. Pathological examination documented histologic proof of a false aneurysm. The optimal treatment for coronary artery aneurysms is unknown but must be based on symptomatic associated arteriosclerotic heart disease and the risks of complications related to the aneurysm, including the risk of rupture with formation of a false aneurysm as documented in this case.

Aneurysm↗

Cholesterol pericarditis terminating as constrictive calcific pericarditis. Follow-up study of patient with 40 year history of disease.

This is a follow-up report of a patient with idiopathic cholesterol pericarditis (previously described in this Journal in 1961). The patient had had recurrent pericardial effusions since 1939, and died with calcific constrictive pericarditis some 40 years later. In the interim, the patient had an extended period (17 years) of symptomatic relief after pericardiotomy in 1958.

Cholesterol↗

Status of the coronary arteries at necropsy in diabetes mellitus with onset after age 30 years. Analysis of 229 diabetic patients with and without clinical evidence of coronary heart disease and comparison to 183 control subjects.

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.

Adult↗

Heterotopic epithelial replacement (so-called "mesothelioma") of the atrioventricular node, congenital heart block, and sudden death.

Heterotopic epithelial replacement (so-called "mesothelioma") of the atrioventricular node is a rare cause of congenital heart block and sudden death; only about 50 cases have been reported in the literature in predominantly female patients ranging in age from infancy to 86 years. The lesion is virtually impossible to diagnose clinically and can be recognized at postmortem only by histologic examination of the atrioventricular conduction tissue. A unique case is reported since the patient, a young, asymptomatic woman, was chosen by chance as the subject of an electrophysiologic study of bradycardia 6 years before her sudden death. The morphologic (light- and electron-microscopic) findings of the atrioventricular node studied at autopsy were supportive of heterotopic epithelial origin of the lesion. The disputed histogenesis is briefly discussed.

Adolescent↗

Classification of ventricular septal defects.

A classification with clinical significance is proposed for ventricular septal defect based on the study of 220 hearts with defects of the ventricular septum. All had atrioventricular and ventriculoarterial concordance with normal relations of cardiac structure. For the purpose of classification, the ventricular septum was considered as possessing muscular and membranous portions, the muscular septum itself being divided into inlet, trabecular, and outlet (or infundibular) components. Defects were observed in the area of the membranous septum, termed perimembranous defects; within the muscular septum, termed muscular defects; or in the area of septum subjacent to the arterial valves, termed subarterial infundibular defects. Perimembranous defects were found extending either into the inlet, trabecular, or infundibular septa. Muscular defects were found in or between the inlet septum, trabecular septum, or infundibular septum. Review of the angiograms showed that the classification was easy to use in the catheterisation laboratory, and our observations suggest that the precision thus obtained has considerable surgical significance.

Heart Septal Defects, Ventricular↗

Histopathological specificity of hypertrophic obstructive cardiomyopathy. Myocardial fibre disarray and myocardial fibrosis.

The topography and specificity of fibre disarray and fibrosis in hypertrophic obstructive cardiomyopathy were determined in a histological study comprising 40 necropsy hearts--10 with hypertrophic cardiomyopathy, 10 with congestive cardiomyopathy, 10 with aortic valve stenosis, and 10 normal hearts. Seven standard regional sections were sampled from each heart and graded "double-blind" (tissue location and disease entity) for severity and extent of fibre dissarray and four distinct types of myocardial fibrosis. Statistical comparison of the severity and distribution of indices of fibre disarray and fibrosis within each group and between the normal and the disease groups showed that fibre disarray and fibrosis were qualitatively non-specific for hypertrophic cardiomyopathy. However, when fibre disarray was quantified (1) it was significantly increased in hypertrophic cardiomyopathy and allowed separation of hearts with hypertrophic cardiomyopathy from normal hearts and from those with congestive cardiomyopathy and aortic stenosis, (2) it did not vary significantly among sections of the left ventricle (that is, between the septum and the free wall) in hypertrophic cardiomyopathy, (3) it was closely associated with plexiform fibrosis, and (4) it varied independently of wall and septal thickness. Though the histogenesis of fibre disarray is unknown, it probably represents an exaggeration of a non-specific common pathway for many diverse pathophysiological processes.

Adolescent↗