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Biomedical subjects

B H Bulkley

Publications and source records attributed to B H Bulkley.

At least 127 records · Page 7Linked to original sources

Catenoid shape of the interventricular septum: possible cause of idiopathic hypertrophic subaortic stenosis.

Previous studies have shown that asymmetric septal hypertrophy (ASH) may occur in embryonic and malformed hearts and that muscle cell disarray is found in myocardium that contracts isometrically. To account for the characteristic ASH and septal muscle fiber disarray of idiopathic hypertrophic subaortic stenosis (IHSS), we postulated that a catenoid shape of the septum, i.e., net zero curvature, would have the mechanics required to produce IHSS. Accordingly, hearts from eight autopsied patients with IHSS were studied for curvature and thickness of free walls and septum, and they were compared to similar measurements in 80 other hearts. In all eight hearts with IHSS the septum was concave to the left in the transverse plane but convex to the left in the apex-to-base plane. Such a catenoid configuration of the septum was not observed in any of the other 80 hearts. The distinctive shape of the septum in IHSS would account for isometric contraction, since adjacent fiber tracts with opposite curvatures would develop maximum tension but would not have motion. Fiber disarray and local hypertrophy would result from such isometric contraction. Since ventricular configuration is acquired early in cardiogenesis, IHSS might therefore result from a genetic or embryonic determination of a catenoid septum.

Adolescent↗

Isolated drainage of the superior vena cava into the left atrium in a 52-year-old man: a rare congenital malformation in the adult presenting with cyanosis, polycythemia, and an unsuccessful lung scan.

This report describes a 52-year-old black male with the isolated finding of an anomalous superior vena cava draining into the left atrium. The patient presented with dizziness, mild cyanosis, polycythemia and normal cardiac and pulmonary findings. The first major diagnostic clue in this confusing clinical presentation was an unsuccessful lung perfusion scan in which intravenous tracer consistently bypassed the lungs. This appears to be the first adult presenting with this rare anomaly. This condition should be suspected if cyanosis, clubbing, and a "normal" cardiac examination coexist and if the more common pulmonary and hematological causes of this triad have been excluded.

Cyanosis↗

Cardiac sarcoid: a clinicopathologic study of 84 unselected patients with systemic sarcoidosis.

Although sarcoid may involve the myocardium, there is little information on its incidence or significance. We studied 84 consecutive autopsied patients with sarcoidosis. The patients ranged in age from 18--80 years (average 46 years) and 61% were women; 23 (27%) of them had myocardial granulomas. In eight (35%) these were clinically silent, and in 15 (65%) there was a history of heart failure and/or arrhythmias and conduction defects. Of the 23 patients, only four (17%) had grossly evident, widespread myocardial lesions: three of these four (75%) had documented arrhythmias. All four had sudden, unexpected death at an average age of 36 years; in only two had sarcoid been suspected during life. The other 19 patients (83%) had microscopically evident granulomatous involvement. Of these, eight (42%) had a thythm or conduction disturbance and three (16%) sudden death, although none of those who suffered sudden death had a recognized rhythm or conduction disturbance. Nine (15%) of those without cardiac sarcoidosis had a rhythm or conduction disturbance and eight (13%) suffered a sudden death. The results show that although myocardial involvement occurs in at least 25% of patients with sarcoid, it most often involves a small portion of myocardium and is clinically silent. Since some of the 61 patients in whom myocardial lesions were not identified may still have had small microscopic granulomas, the true incidence of myocardial sarcoid may be even greater than suggested here. Rhythm and conduction disturbances are more common in the cardiac sarcoid group, but the findings suggest that only the small subset of patients with severe, grossly evident myocardial sarcoid are at increased risk for sudden death.

Adult↗

Two-dimensional echocardiographic diagnosis of left atrial myxoma.

Two patients with left atrial myxomas detected with one-dimensional echocardiographic studies were evaluated before and after surgery with real-time phased-array two-dimensional echocardiographic studies. This latter technique provided relatively quantitative information regarding the size, shape, and mobility of the tumor and its effect on cardiac function. In case 1, the two-dimensional echocardiogram showed a relatively immobile tumor measuring 3 x 4.5 cm in diameter in the left atrium only. At surgery a 2 x 4.5-cm myxoma that was attached to the left atrium by a broad-based short stalk was removed. In case 2, the left atrial mass appeared to be 3 x 4 cm in diameter, with much movement during the cardiac cycle. At surgery a 3 x 4-cm left atrial myxoma that was attached to the interatrial septum by a long stalk was removed. In both cases, masses in other cardiac chambers were excluded, the mitral valves were normal, and left ventricular function was normal, all of which were confirmed at surgery and by postoperative echocardiograms. In one patient the information obtained by two-dimensional echocardiographic studies was believed to be sufficient to preempt the need for cardiac catheterization. These cases illutstrate that this new noninvasive technique may provide sufficient quantitative preoperative detail in patients with left atrial tumors to obivate the risk and expense of caridac catheterization.

Echocardiography↗

Coronary artery embolism and myocardial infarction.

Although coronary artery embolism is a recognized entity, there is little morphologic information indicating it is a cause of myocardial infarction. We studied patients with coronary artery embolic infarcts, which comprised 13% of our autopsy-studied infarcts. Underlying diseases predisposing to coronary emboli included valvular heart disease (40%), myocardiopathy (29%), coronary atherosclerosis (16%), and chronic atrial fibrillation (24%). Mural thrombi were present in 18 (33%). Myocardial infaction, clinically diagnosed in 15 (27%) patients, caused death in 11 (20%). Most emboli involved the left coronary artery and lodged distally, causing infarcts that were usually transmural. Because of their distal location and recanalization, coronary emboli may be a cause of infarcts with angiographically normal coronaries. Thus, coronary emboli are not rare, may produce signs and symptoms indistinguishable from altherosclerotic coronary disease, and by lodging distally in coronary arteries that are usually previously normal, they most often cause small but transmural myocardial infarction.

Adult↗

Mitral-valve prolapse syndrome and recurrent ventricular tachyarrhythmias: a malignant variant refractory to conventional drug therapy.

Of 60 patients referred for management of drug-refractory ventricular tachyarrhythmias, 10 (17%) had mitral-valve prolapse. These 10 patients ranged in age from 19 to 70 years (mean, 47 years); seven were women. All 10 had recurrent ventricular tachycardia, while four had a history of ventricular fibrillation. Nine patients were refractory to propranolol in combination with one or more of the standard antiarrhythmic agents. All showed improvement with aprindine therapy. The results show that refractory malignant ventricular prolapse syndrome; patients with mitral-valve prolapse account for a rather high percentage of those patients referred with recurrent drug-refractory ventricular tachyarrhythmias; in patients with unexplained ventricular arrhythmias, mitral-valve prolapse should be considered; and aprindine may be effective for ventricular tachyarrhythmias associated with mitral-valve prolapse.

Adult↗

Effects of calcium administration during post-ischemic reperfusion on myocardial contractility, stiffness, edema, and ultrastructure.

Previous investigators have suggested that calcium may play a role in the pathogenesis of myocardial cell damage following ischemia and reperfusion. Twenty-six in-situ blood perfused isovolumic canine preparations were divided into four groups. Group I dogs were maintained normocalcemic during 45 min of reperfusion following 45 min of hypothermic (27 degrees C) ischemic arrest; Group II dogs received CaCl2 (7 mg/kg) after 15 min of reperfusion; Group III dogs received citrate solution (0.8 ml/kg citrate-phosphate-dextrose [CPD]) after 15 min of reperfusion; Group IV dogs received 7 mg/kg of CaCl2 at 5 min after receiving the same citrate dose as Group III after 15 min of reperfusion. In Group II hearts, calcium improved the left ventricular contractility (P < 0.05 vs Group I) without causing additional cellular or subcellular injury. Calcium also appeared to increase myocardial stiffness (alpha(n)) compared to Group I hearts (P < 0.01). In Group III hearts, citrate reduced contractility (P < 0.01 vs Group I) and increased myocardial edema (P < 0.005 vs Group I) without any apparent improvement in cellular or subcellular preservation. In Group IV hearts, calcium reversed the depression of contractility caused by citrate, resulted in no additional morphologic injury, increased myocardial stiffness compared to Group I or Group III (P < 0.005), and minimized myocardial edema (P < 0.005 vs Group I or III). These results suggest that calcium administered after 15 min of reperfusion improves the depression of contractility that follows hypothermic ischemic arrest without causing additional myocardial damage.

Animals↗

Pathology of coronary artery bypass graft surgery.

Coronary artery bypass graft surgery has been available and widely successful for the symptomatic treatment of ischemic heart disease. Despite its widespread use, there is little information available on the pathological consequences of this procedure on the human heart. In this article, morphological consequences of coronary artery bypass graft surgery is reviewed. Intimal changes occurring within the vein graft itself consist predominately of fibrous initimal proliferation, which in some patients may progress to from an occlusive plaque. Most occlusions, however, occur at the coronary artery bypass graft anastomosis site and the mechanisms of occlusion include compression of the vascular lumen, thrombosis, and dissection of the coronary artery. Most graft failure occurs in the setting of too small a native coronary artery lumen. The myocardium is also at risk for alterations as a result of the bypass operation. Contraction band or reperfusion necrosis is the type of injury most commonly seen, and it appears to occur most often in the distribution of patent grafts. Accelerated atherosclerosis in vein grafts and the myocardial injury associated with revascularization require further detailed morphological studies, but these are important areas for pathological exploration since they bear on important and yet unanswered questions about coronary bypass surgery: can it in the long run perserve myocardium and prolong life?

Adult↗

Cardiovascular manifestations of Pseudoxanthoma elasticum.

Autopsies were performed in three cases of pseudoxanthoma elasticum (PXE) to evaluate the cardiovascular changes. The endocardial lesion characterized by intimal fibroelastotic thickening and disorganization, fragmentation, and calcification of elastic fibers in the deeper endocardial layers is unique histologically. Severe atherosclerosis was present in all cases and resembled that encountered routinely. Fragmentation and degeneration of the elastic laminae of muscular arteries was followed by vascular calcification that could not be distinguished morphologically from Mönckeberg's arteriosclerosis. All three cases showed striking initimal fibroelastotic thickening, particularly in intrarenal arteries, resembling that seen in hypertension, although only one of the subjects was hypertensive. It would appear that the metabolic defect in PXE predisposes to the premature onset and accelerated development of commonly encountered vascular aging processes, and that the endocardial lesion is the only specific cardiovascular manifestation of the disease.

Aged↗

Isometric cardiac contraction. a possible cause of the disorganized myocardial pattern of idiopathic hypertrophic subaortic stenosis.

The bizarre septal architecture of idiopathic hypertrophic subaortic stenosis (IHSS) may be secondary to a small systolic cavity with late systolic isometric contraction. We examined ventricular muscle for IHSS-type muscle-fiber disarray in infant hearts in which isometric contraction would occur during development-namely, pulmonary or aortic-valve atresia with intact ventricular septum and normal atrio-ventricular valves. Fifteen patients with aortic atresia and 10 with pulmonary atresia were compared to 25 normal controls of matched age and heart weight. Aortic atresia showed disorganization of muscle-fiber alignment of left ventricle, particularly septum, and intramural coronary-artery changes virtually identical to IHSS. Pulmonic atresia had similar right ventricular disarray and vessel changes, again most marked in the septum. Thus, cardiac muscle-cell disorientation similar to IHSS occurs in infant ventricles with outflow-tract obstruction. This IHSS-type myocardial-fiber disarray may result from altered wall stresses related to isometric systolic contraction.

Adult↗

Rupture of the papillary muscle of the tricuspid valve. A complication of cardiopulmonary resuscitation and a rare cause of tricuspid insufficiency.

Rupture of a papillary muscle is a rare occurrence. Two patients are described in whom rupture of a papillary muscle of the tricuspid valve developed after external cardiac massage during cardiopulmonary resuscitation. One of these patients survived briefly with clinical evidence of triscupid regurgitation immediately after resuscitation. Although tricuspid valve papillary muscle rupture has been described as a complication of bacterial endocarditis, chest trauma and myocardial infarction, it is a generally unrecognized complication of external cardia massage. Findings in the two patients reported here suggest that patients with a dilated right ventricle may be more susceptible to this rare complication of cardiopulmonary resuscitation.

Adult↗

The conduction system in cardiac amyloidosis. Clinical and pathologic features of 23 patients.

Cardiac amyliodosis is frequently associated with major electrocardiographic conduction disturbances; but that these disturbances are due to infiltrative destruction of the conduction system by amyloid is unclear. We studied the conduction systems in 23 autopsy patients with cardiac amyloidosis (group 1) (mild in seven, moderate in five and severe in 11), 21 (91 per cent) of whom had had abnormalities of conduction or rhythm during life. For comparison, we examined the conduction system in 23 control subjects matched in age and heart weight (group 2). Of the 23 patients in group 1, only three had extensive amyloidosis of the conduction system; in all three, electrocardiograms showed first degree atrioventricular block and left anterior hemiblock. A more common morphologic abnormality of the conduction system was severe sinoatrial node fibrosis present in seven (30 per cent) patients, and idiopathic atrophy and fibrosis of the bundle branches present in six (26 per cent) patients. None of the patients in group 2 had severe sinoatrial node fibrosis, but two (9 per cent) had idiopathic atrophy and fibrosis of the left bundle branch. Marked fibrosis of the sinus node was more frequent in patients with severe or moderate amyloid, but fibrosis of the bundle branch did not appear to be related to the amount of amyloid elsewhere in the heart. Varying degrees of atrioventricular and bundle branch block were also present in six patients with no morphologic abnormalities of the conduction system. Thus, conduction and rhythm disturbances are frequent in cardiac amyloidosis, but direct amyloid infiltration of the specialized conduction tissue of the heart does not account for the majority of these disturbances. Whether the increased incidence of fibrosis of the conduction system in group 1 compared to that in group 2 relates to the infiltrative myocardiopathy is uncertain.

Adult↗

Clinical pathologic conference. Purulent pericarditis with asymmetric cardiac tamponade: a cause of death months after coronary artery bypass surgery.

This relatively young man with a host of medical problems including polycystic kidneys. chronic renal failure, long-standing hypertension, and premature atherosclerosis, died of cardiovascular disease; not, as might be expected, from his severe coronary artery disease but rather from purulent pericarditis. The latter was an unusual and unexpected consequence of the entire complex of his illnesses and because of its confinement to the posterior pericardium by postoperative adhesions produced an asymmetric cardiac tamponade.

Autopsy↗