A proposed pathogenesis of cor triatriatum: impingement of the left superior vena cava on the developing left atrium.
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Adult↗
Biomedical subjects
B H Bulkley
Publications and source records attributed to B H Bulkley.
Correlation of age and heart weight with tortuosity and caliber of normal human coronary arteries.
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Adult↗
A clinicopathologic study of prosthetic valve endocarditis in 22 patients: morphologic basis for diagnosis and therapy.
Although endocarditis is a frequently lethal complication of prosthetic valve replacement, there is little pathological information on which to base diagnosis and treatment. We have studied the clinical and pathological features of 22 patients with prosthetic valve endocarditis seen at The Johns Hopkins Hospital over the past 17 years. Five patients developed endocarditis within two months of operation and 17 between two and 48 months (average 12) after operation. Patients dying early had a more fulminant course and their endocarditis was less often recognized during life. Late deaths, tending to present with fever and bacteremia unmasked by postoperative problems, were more readily recognized. Mitral and aortic prosthetic endocarditis generally led to a different type of prosthetic valve dysfunction: nine of 14 aortic valve prostheses with endocarditis developed incompetence and one other stenosis; five of seven patients with mitral valve prostheses developed stenosis and one, a homograft, developed incompetence. Prosthetic valve dysfunction led to death in 10 patients (45 per cent) and embolic events in five (23 per cent), including four cerebrovascular accidents. Ring infection, often believed to be universally present, and a contraindication to surgery, was found only in 50 per cent of these patients. In four patients (18 per cent) the endocarditis was sterilized by antibiotics but death occurred from valve dysfunction or emboli. These findings suggest that early surgical intervention combined with antibiotics has a chance of providing effective therapy for prosthetic valve endocarditis.
Adolescent↗
In vitro computed tomography of the human heart.
In vitro computed tomography (CT) was performed on nine human hearts. Mean attenuation coefficients from a 2.5 X 2.5-cm area of the left ventricular wall were determined from a 160 X 160 computer printout before and after formalin fixation in five hearts. These values averaged 27.1 and 24.7 units, respectively (p less than 0.05). The absorption characteristics of normal human myocardium do not differ from those observed in animals and the potential of CT application to ischemic heart disease can be assessed by this mehtodology applied to postmortem hearts of patients with myocardial infarction of varying duration.
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Accelerated "atherosclerosis". A morphologic study of 97 saphenous vein coronary artery bypass grafts.
Intimal thickening of uncertain cause has been noted in saphenous vein bypass grafts. To study the development of these changes, 97 vein grafts in 55 autopsied patients were examined by angiography and histology. Significant alterations in the grafts were confined to the intima and included thin layers of circumferential thrombus, concentric fibrous plaque and occlusive thrombus with organization. Circumferential nonocclusive intimal thrombus was present in 36 (73%) postoperative patent grafts. Concentric fibrous plaque, found in all twelve patent late grafts, showed changes characteristic of atheromata, including smooth muscle and foam cells. Intimal fibroelastosis was absent in the grafts, indicating that concentric plaque is not a simple response to increased pressure, or an "arterialization" of the vein. The findings suggest that early appearing concentric intimal thrombus is converted into fibrous plaque, and that this early thrombus is a factor in the development of acclereated "atherosclerosis" in saphenous vein bypass grafts.
Adult↗
Thallium 201 imaging and gated cardiac blood pool scans in patients with ischemic and idiopathic congestive cardiomyopathy. A clinical and pathologic study.
In ischemic cardiomyopathy (CM) fibrosis replaces large segments of myocardium, but in idiopathic congestive CM the myocardium contains only small foci of fibrosis or is morphologically normal. As coronary disease and myocardial infarction may be clinically silent, it is not always possible to distinguish ischemic from idiopathic congestive CM during life without cardiac catheterization. To determine whether noninvasive methods, thallium 201 myocardial (Tl) imaging and technetium 99m gated cardiac blood pool scans (GCBPS), could separate the entities, we evaluated radioisotope images of the heart in 13 patients with ischemic, and eight patients with idiopathic congestive CM, and 14 patients with normal hearts. Diagnosis was setablished by cardiac catherterization and/or autopsy in each of the 35 patients. The 14 normals could be readily distinguished from CM, and ischemic could be distinguished from idiopathic dilated CM in 20 of 21 patients. All patients with myocardiopathy showed hypokinetic and dilated left ventricles, but right ventricular dilatation was evident mainly in those with idiopathic CM. Tl images in the ischemic type had defects of greater than 40% of image circumference which corresponded to segmental wall motion abnormalities on GCBPS, whereas those with the idiopathic congestive form were homogeneous or had defects of less than 20% of image circumference. Autopsy studies in 7 of 35 patients correlated Tl defects of greater than 20% of circumference with transmural myocardial fibrosis.
Adult↗
Hypercontractile cardiac states simulating hypertrophic cardiomyopathy.
Hypertrophic cardiomyopathy (HCM) or idiopathic hypertrophic subaortic stenosis (IHSS) has been defined as an autosomal dominant myocardial disease characterized by specific physical findings, echocardiographic features, asymmetric septal hypertrophy and disordered myocardial architecture. Echocardiographic and scintiphotographic studies failed to reveal evidence of asymmetric septal hypertrophy in four patients with systolic anterior movement of the mitral valve and the typical ausculatory and peripheral pulse abnormalities characteristic of HCM. Postmortem examimination in one patient demonstrated mild concentric left ventricular hypertrophy and a normal arrangement of myocardial muscle fibers. These observations in four patients demonstrate that both systolic anterior movement of the mitral valve and the physical findings characteristic of HCM are not specific for the autosomal dominant myocardial disease characterized by asymmetric septal hypertrophy and abnormal septal histology.
Adult↗
Correlation of coronary arteriograms and left ventriculograms with postmortem studies.
To assess the accuracy of angiographic determinations of disease of coronary arteries and left ventricular myocardium we compared clinical with postmortem coronary arteriograms and left ventriculograms with myocardial pathology in 28 patients, all of whom died postoperatively and within three months of angiography; 19 had ischemic heart disease, four valvular heart disease, and five both. Comparison of pre and postmortem lumenal occlusion in 315 epicardial coronary segments, excluding those operated upon, showed greater than 50% narrowing discrepancies in 21 (7%). Significant coronary artery lesions were overestimated in six and underestimated in 15. Of the six overestimations, three appeared to be due to coronary spasm; of the 15 underestimations, 12 were due to overlapping images; six discrepancies were unexplained. Comparison of wall motion in 140 ventriculogram segments with myocardial pathology, excluding any post-study or perioperative injury, showed good correlation of reduced motion with 48 (34%) infarcted and 10 (7%) aneurysmal segments. However, 58 (41%) other segments had poor or absent ventriculogram motion, with structurally normal myocardium and patent coronary artery supply; 19 were on infarct margins and 39 in dilated or hypertrophied hearts. Thus, premortem coronary arteriographic occlusions generally indicate atherosclerotic narrowing; but decreased or absent segmental wall motion frequently does not indicate a myocardial lesion. It may be attributable to ischemia in the distribution of a critically narrowed coronary artery or it could be due to abnormal ventricular topography.
Adult↗
Asymmetric septal hypertrophy and myocardial fiber disarray. Features of normal, developing, and malformed hearts.
The specificity and significance of the asymmetric septal hypertrophy (ASH) and myocardial fiber disarray of idiopathic hypertrophic subaortic stenosis (IHSS) is uncertain. To examine this we studied 215 hearts, including normal embryos, fetuses, children, and adults; and hearts with congenital and acquired disease. Disproportionate septal thickening was present in all embryos and in some abnormal hearts, particularly those with severe right ventricular hypertrophy due to congenital malformations. Some myocardial fiber disarray was present in all hearts at the junctions of interventricular septum and ventricular free wall. In hearts with semilunar valve atresia with intact ventricular septums, and in the infundibulum of some with tetralogy of Fallot, however, extensive fiber disarray was present. Thus, ASH occurs in the normal developing heart and in some malformed hearts with RVH; marked muscle fiber disarray may occur in certain congenital lesions with abnormal systolic contraction. Neither morphologic finding independently or in combination is pathognomonic of idiopathic hypertrophic subaortic stenosis.
Adult↗
The use of 201thallium for myocardial perfusion imaging in sarcoid heart disease.
Cardiac dysfunction due to systemic sarcoidosis is most of ten due to severe restrictive pulmonary diseases. Although the diagnosis is frequently missed during life, direct granulomatous infiltration of the myocardium may occur with systemic sarcoidosis and, when present in the heart, is a major cause of death. To explore the possible use of radioactive 201thallium, a new agent for myocardial imaging, for improved clinical recognition of sarcoid heart disease, myocardial perfusion imaging with 201Tl was performed in six normal volunteers and in five patients with documented systemic sarcoidosis and clinically apparent cardiac dysfunction. Two of the patients with sarcoidosis had severe restrictive pulmonary disease. Their myocardial perfusion scans revealed relatively uniform uptake of 201Tl by the left ventricle, similar to that found in the normal volunteers; however, the right ventricular uptake of 201Tl and right ventricular thickness in these two patients with sarcoidosis was greater than normal. The other three patients with sarcoidosis had unexplained congestive heart failure, mitral regurgitation, or arrhythmias. Myocardial perfusion imaging in these patients revealed normal right ventricular uptake of radioactivity but segmental defects in the left ventricle compatible with an infiltrative disease of the myocardium. Segmental myocardial infiltration by sarcoid was confirmed by autopsy in one of these patients and at operation in another. Thus, myocardial imaging with 201Tl may provide a noninvasive technique for the improved clinical recognition of primary myocardial sarcoid and for distinguishing it from cardiac dysfunction secondary to pulmonary disease.
Adult↗
Echocardiographic recognition of silent aortic root dilatation in Marfan's syndrome.
Echocardiography has proven a useful and sensitive means to determine noninvasively the cardiac dimensions. This report describes the echocardiographic detection of progressive dilatation of the aortic root over an 18-month period, which led to death in a 33-year-old man with Marfan's syndrome; however, at no time was the aortic dilatation evident on chest x-ray films. This patient illustrates the sensitivity of the echocardiogram in detecting and assessing the severity of disease of the aortic root not readily assessed by other noninvasive techniques and the value of serial measurements of aortic dimensions in patients with proven or suspected dilatation of the aortic root.
Adult↗
Mechanisms of occlusion of saphenous vein--coronary artery "jump" grafts.
Seven patients with 12 end-to-side and eight side-to-side saphenous vein graft-to-coronary artery anastomoses were studied at autopsy. Postmortem coronary arteriograms and serial histologic sections of the anastomoses were performed. Occlusive changes occurring in the coronary arteries tended to be at the ends of the arteriotomy and could be attributed to reduction of arterial circumference from its eversion and utilization in the anastomotic connection, or from thrombosis along the suture line or from both. The mechanisms of arterial occlusion were the same in side-to-side and end-to-side anastomoses. In addition to the occlusive changes at the anastomosis, "jump" grafts with two anastomoses were susceptible to rolling or kinking of the graft, apparently as a result of torsion from placement of the side-to-side anastomosis.
Aged↗
Occlusive changes at the coronary artery--bypass graft anastomosis. Morphologic study of 95 grafts.
Ninety-five bypass graft anastomoses in 52 patients dying up to 4 years after direct coronary revascularization were studied at autopsy by angiograms and serial histologic sectioning of the graft-artery anastomosis. When new coronary occlusions and narrowings occurred, they were adjacent to either the proximal or distal ends of the anastomosis and were due to compression or loss of circumference of the arterial lumen (40 per cent), thrombus formation (40 per cent), mural dissection of the coronary wall (8 per cent) or the combination of compression and thrombosis (12 per cent). Small coronary artery diameter, local atheromas, and extension of the arteriotomy into a branch vessel were significant factors predisposing to occlusive changes. The findings emphasize the importance of careful artery selection for bypass, the need to avoid local vascular disease and branch-points, and the technical difficulties encountered in the presence of local vascular lesions or small coronary arteries.
Adult↗
Correlation of myocardial contraction band necrosis and vascular patency. A study of coronary artery bypass graft anastomoses at branch points.
Eleven patients who died within 1 week of operation and in whom saphenous vein bypass graft anastomoses were located at or extended across coronary artery branch points were studied at autopsy. New surgically introduced narrowings of greater than 75% in one branch of the 13 anastomoses studied were more frequent (six of eight) when the arteriotomy extended into a branch artery than when the arteriotomy ended proximal to the flow divider of the branch point (zero to five). Obstruction was most frequently caused by suture compression of the arterial lumen. In five of the six anastomoses, where one branch contained a significant new narrowing and the other did not, a striking difference in the severity of myocardial contraction band necrosis in the distribution of the two arteries was found. In each such case the severe necrosis was in the distribution of the patent branch artery and the obstructed branch had slight or trivial injury. The results are interpreted as showing that myocardium that has the potential for developing contraction band necrosis may not develop it if the reflow phase is suppressed.
Adult↗
A clinicopathological study of post-thoracotomy purulent pericarditis. A continuing problem of diagnosis and therapy.
Cardiac infections after operations are infrequent but, when present, are often fatal. The 14 autopsied patients in whom purulent pericarditis developed after thoracic operations over an 88 year period at The Johns Hopkins Hospital were studied. Purulent pericarditis developed after cardiac operations in 10 and after pulmonary resections in 4. In 12 of the 14 cases the pericardial sac had been opened. Associated postoperative infection, present in 13 patients, included mediastinitis in 7 and empyema in 3. Staphylococcus was the infection organism in half of the patients. Associated cardiac infection, including endocarditis, myocardial abscess, and graft infection, was present in 5 (36 per cent) patients. Death occurred within 2 months of operation in 11 (79 per cent) patients; it was due to infection in 9, cardiac tamponade in 4, and arrhythmias and heart failure in one. The diagnosis of purulent pericarditis was made before death in only 5 (36 per cent) cases, in part owing to masking of the usual signs of pericarditis in the postoperative patient. Since the introduction of antibiotics, the over-all incidence of purulent pericarditis has decreased. However, pericardial infection after thoracotomy has increased tenfold, and patients undergoing cardiac operations in particular provide a new and increasing population at risk for this disease.
Adolescent↗
The cardiac conduction system in progressive systemic sclerosis. Clinical and pathologic features of 35 patients.
Progressive systemic sclerosis may be associated with focal myocardial fibrosis. Electrocardiographic abnormalities including conduction block are common in progressive systemic sclerosis but whether they are due to direct destruction of the specialized conduction tissue of the heart is uncertain. The conduction systems of 35 patients with progressive systemic sclerosis were studied. Of these 35 patients, 17 (50 per cent) had myocardial fibrosis of the type seen in progressive systemic sclerosis. In 10 of the 17, it was severe. Sinus node fibrosis was present in 13 patients and was nearly as frequent in those with as in those without the progressive systemic sclerosis myocardial lesion. Overlying pericarditis may have contributed to the fibrotic changes within the sinoatrial nodes in seven of the 13 patients. The atrioventricular node and main His bundles were normal. However, fibrotic changes were found in the proximal bundle systems in six patients. In three of the six, severe myocardial progressive systemic sclerosis was present, two had focal fibrous atrophy of the left bundle, and one had complete interruption of the right bundle. In only the latter patient was this reflected in the electrocardiogram which showed a right bundle branch block. Three patients without progressive systemic sclerosis myocardial lesions also had fibrous atrophy of a portion of the proximal left bundle branch, and in one the electrocardiogram showed an isolated left anterior hemiblock. Thus, morphologic abnormalities within the conduction system in our patients are difficult to attribute to progressive systemic sclerosis per se. Furthermore, although conduction abnormalities were more frequent in patients with myocardial disease, specific conduction system disease was not the cause in most patients. As has been noted in ischemic heart disease, the conduction system appears to be relatively spared from the myocardial changes of progressive systemic sclerosis, and the high incidence of conduction disturbances in this condition may be a consequence, rather, of damage to working myocardium.
Adolescent↗
Myocardial lesions of progressive systemic sclerosis. A cause of cardiac dysfunction.
The nature, prevalence, functional significance, and indeed existence of myocardial disease in progressive systemic sclerosis (PSS) has been debated. In this study the clinical and pathological features of 52 autopsied patients were analyzed in an attempt to resolve these questions. A distinctive focal myocardial lesion ranging from contraction band necrosis to replacement fibrosis throughout both ventricular walls was present in 23 patients who had widely patent extramural coronary arteries. There were no morphologic abnormalities of the intramyocardial coronary arteries to account for these lesions. Comparing those patients having severe (13), mild (10), or no (24) PSS myocardial lesions, and patent extramural coronary arteries, there were no major differences in age, sex, frequency and severity of pulmonary, renal or hypertensive disease which could account for the myocardial necrosis and fibrosis. The three groups did differ, however, with regard to clinical cardiac abnormalities: ventricular arrhythmias and conduction disturbances were six and two times as frequent, respectively, in those with severe myocardial PSS compared to the other two groups. A pattern of primary myocardial disease with intractable congestive heart failure resulted from severe myocardial PSS in four patients, angina pectoris with normal coronary arteries was associated with the severe myocardial lesion in three patients, and sudden death in five. The occurrence of contraction band necrosis suggests that the myocardial damage in PSS might be due to intermittent vascular spasm of the type recognized in the digits and possibly kidneys and lungs, i.e., an intramyocardial Raynaud's phenomenon. The findings in our patients clearly show that myocardial progressive systemic sclerosis is a distinct entity with relatively frequent occurrence which may lead to arrhythmias, congestive heart failure, angina pectoris with normal coronary arteries and sudden death.
Adolescent↗
Atherosclerotic narrowing of the left main coronary artery. A necropsy analysis of 152 patients with fatal coronary heart disease and varying degrees of left main narrowing.
Histologic sections of the left main (LM) and the other three major coronary arteries were studied in 152 patients. The lumen of the LM in 35 patients was greater than 75% narrowed; in thirty, 50-75%; and in 87, less than 50% narrowed. The patients with greater than 75% narrowing were younger. Angina pectoris and hyperlipoproteinemia, specifically type II, were more common (P less than 0.02) and acute transmural and healed subendocardial myocardial infarcts were less frequent (P less than 0.05) in the patients with greater 75% LM narrowing than in those with less than 50% narrowing. Of the three other major coronary arteries, the average number narrowed in the patients with greater than 75% LM narrowing was 2.9; in those with 50-75% LM narrowing 2.7, and in those with less than 50% LM narrowing, 2.4. Of the 35 patients with greater than 75% LM narrowing, 33 had greater than 75% luminal narrowing of each of the other three major coronary arteries. Narrowing of the LM, therefore, indicates severe narrowing of usually all major coronary arteries.
Adult↗