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Biomedical subjects

F L Gobel

Publications and source records attributed to F L Gobel.

At least 19 recordsLinked to original sources

Cardiac amyloidosis in hereditary neuropathic amyloidosis diagnosed by endomyocardial biopsy.

A 57 year old male first developed progressive neuropathy of the lower extremities and then similar involvement in the upper extremities. Two years later he developed dyspnea and then complete atrioventricular block requiring the use of a permanent cardiac pacemaker. An older brother had an almost identical clinical course, including the installation of a permanent pacemaker. Both died of left ventricular failure. An endomyocardial biopsy revealed extensive amyloidosis and fibrosis of the myocardium. This is the first report of a myocardial biopsy showing amyloid on electron microscopy.

Amyloidosis

The rate-pressure product as an index of myocardial oxygen consumption during exercise in patients with angina pectoris.

In order to evaluate hemodynamic predictors of myocardial oxygen consumption (MVO2), 27 normotensive men with angina pectoris were studied at rest and during a steady state at sympton-tolerated maximal exercise (STME). Myocardial blood flow (MBF) was measured by the nitrous oxide method using gas chromatography. MBF increased by 71% from a resting value of 57.4 +/- 10.2 to 98.3 +/- 15.6 ml/100 g LV/min (P less than 0.001) during STME while MVO2 increased by 81% from a resting value of 6.7 +/- 1.3 to 12.1 +/- 2.8 ml O2/100 g LV/min (P less than 0.001). MVO2 correlated well with heart rate (HR) (r = 0.79), with HR x blood pressure (BP) (r = 0.83), and, adding end-diastolic pressure and peak LV dp/dt as independent variables, slightly improved this correlation (r = .86). Including the ejection period (tension-time index) did not improve the correlation (r = 0.80). Thus, HR and HR x BP, both easily measured hemodynamic variables, are good predictors of MVO2 during exercise in normotensive patients with ischemic heart disease. Including variables reflecting the contractile state of the heart and ventricular volume may further improve the predictability.

Adult

Hypereosinophilic syndrome with biventricular involvement.

In a 45-year-old man with hypereosinophilic syndrome, cardiac disease, mainly endocardial thickening and extensive mural thrombosis of both ventricles, was confirmed at autopsy. Early in the course of the disease, right ventricular endocardial biopsy had demonstrated the basic process. Restriction in filling and contraction of the right ventricle were demonstrated by functional studies. By echocardiographic study, progressive reduction in size of the right ventricular cavity and premature opening of the pulmonary valve were demonstrated, while this method was less adequate in identifying the process in the left ventricle.

Autopsy

The significance and prognosis of chronic bifascicular block.

The clinical course of 303 patients with bifascicular block was reviewed. Initially, 62% of the total group had evidence of heart disease. The highest frequency of heart disease was in patients with left bundle-branch block and first-degree atrioventricular block (78% or 22/28), while the lowest was in those with right bundle-branch block and left axis deviation (56% or 55/98). During the subsequent clinical course the occurrence of cardiovascular morbidity was greatest in patients who had left bundle-branch block and heart disease (55% or 49/89). Complete heart block occurred in only 11 patients. The highest incidence of complete heart block occurred in right bundle-branch block and left axis deviation when associated with heart disease, but the annual rate was only 4%/yr. Mortality was highest in those with left bundle-branch block, first-degree atrioventricular block, and left axis deviation (43%/yr). A one-year mortality of 65% (11/17) was noted for patients who had bifascicular block prior to the time of acute myocardial infarction.

Bundle-Branch Block

Exercising pulmonary blood flow in mitral stenosis with anomalous pulmonary venous connection.

Exercising pulmonary blood flow was estimated from indicator-dilution curves in a patient with the infrequent combination of rheumatic mitral stenosis and anomalous pulmonary venous connection from the left lung. During supine exercise against progressively increasing external workloads, the proportion of flow to each lung remained nearly constant. The pulmonary vascular resistance was highest in the left lung, which emptied into the low-pressure systemic vein. Although right and left atrial pressures differed markedly during supine exercise, the proportion of pulmonary blood flow shunted to the systemic venous sytem remained essentially unchanged.

Adult

Multifocal atrial tachycardia.

Multifocal atrial tachycardia (MAT) was observed in 41 patients, 35 of whom were in acute respiratory distress. A hospital mortality of 37% reflected the seriousness of their underlying conditions. In no patient was the arrhythmia the primary cause of death. The MAT preceded and/or followed atrial fibrillation (AF) or atrial flutter (Af) in 19 patients (46%). The MAT simulated AF in several cases in which P-waves were inconspicuous. Our cases suggest that MAT is not a manifestation of digitalis intoxication. Quinidine sulfate, procainamide hydrochloride, lidocaine, and phenytoin (diphenythydantoin) did not affect the rhythm. Digitalis was usually not effective in slowing the ventricular response in patients with MAT. Propranolol hydrochloride was given to seven patients and was effective in slowing the atrial rate in all cases. An attempt at electrocardioversion was unsuccessful in one patient.

Aged

Chest pain as a predictor of coronary artery disease in patients with obstructive aortic valve disease.

To clarify the association between chest pain and significant coronary artery disease in patients who have aortic valve disease, 76 consecutive candidates for aortic valve replacement were evaluated prospectively with use of a historical questionnaire and coronary arteriography. Of the 76 patients, 19 (25 percent) had no chest pain, 21 (28 percent) had chest pain that was not typical of angina pectoris and 36 (47 percent) had chest pain typical of anigina pectoris. In 18 of 19 patients the absence of chest pain correlated with the absence of coronary artery disease. The single patient without chest pain who had coronary artery disease had evidence of an inferior myocardial infarction in the electrocardiogram. Thus, absence of chest pain and the absence of electrocardiographic evidence of infarction predicted the absence of coronary disease in all cases. The presence of chest pain did not predict the presence of coronary artery disease, but the more typical the pain of angina pectoris the more likely were patients to have significant coronary artery disease. Of the 21 patients with atypical chest pain, 6 (29 percent) had coronary artery disease, but of the 36 patients with typical angina pectoris 23 (64 percent) had significant coronary artery disease. In addition, when patients with chest pain not typical of angina pectoris also had coronary artery disease, the diseased vessels usually supplied smaller areas of the left ventricle than when the pain was typical of angina pectoris. In 21 of 23 patients (91 percent) with typical angina pectoris and significant coronary artery disease, lesions were present in the left coronary artery. There was no systolic pressure gradient across the aortic valve that excluded the presence of coronary artery disease, although all patients with a calculated aortic valve area of less than 0.4 cm2 were free of coronary artery disease. Patients with severe left ventricular dysfunction were more likely to have normal coronary arteries.

Adult

Bradycardia with mitral valve prolapse: a potential mechanism of sudden death.

Eleven members of a family with a high prevalence of mitral valve prolapse were investigated. Seven had documented sinus bradycardia, and five had mitral valve prolapse. Three patients with both mitral valve prolapse and bradycardia had recurrent syncope reproduced by simple head-up tilting, and in one patient this resulted in asystole. The hemodynamic response to isoproterenol and phenylephrine administration were normal. Supine plasma norepinephrine levels were normal in all three and increased appropriately in two of three patients after tilting. Atrial pacing studies documented marked prolongation of atrial-His intervals and inability to maintain 1:1 atrioventricular conduction when paced at a rate of 120/min. These findings were reversed by atropine. This family shows a close correlation between mitral valve prolapse and potentially lethal bradycardia. Excessive vagal tone is believed to be responsible for both bradycardia and sinus arrest, which in two patients was prevented by permanent demand pacing.

Adolescent

Hemodynamic changes during ventricular pacing in patients with complete heart block and aortic and mitral valvular heart disease.

Increasing the heart rate to near normal in patients with complete heart block (CHB) and slow ventricular rates may lead to greater improvement in ventricular function than when the heart rate is increased from normal to more rapid heart rates. Improvement in ventricular function is usually manifested by a decrease in left ventricular end-diastolic pressure (LVEDP) and volume and by an increase in contractility. In patients with both CHB and valvular heart disease improvement in ventricular function during pacing may be modified by the nature of the valvular disease. Hemodynamic data from six patients with both valvular heart disease and CHB were compared with those from ten patients with CHB and normal cardiac valves. Hemodynamic studies were performed at slow or idioventricular rates and again after increasing the heart rate to more nearly normal levels by ventricular pacing. When obstruction to left ventricular inflow (mitral stenosis) co-existed with CHB, increasing the heart rate resulted in a reduction of an elevated LVEDP to normal. This resulted in only a small increase in left atrial pressure in spite of a striking increase in the mean left atrial-ventricular gradient. When obstruction to left ventricular outflow prevailed (aortic stenosis), improvement in cardiac function was manifested mainly by a decrease in LVEDP and was accompanied by a decrease in left ventricular stroke work. When a large regurgitant volume (aortic insufficiency) was added to a ventricle which has enlarged subsequent to CHB, there was striking elevation in ventricular filling pressures which returned to more nearly normal levels when the heart rate was increased. This was accompanied by a reduction in regurgitant stroke volume in the patient in whom it was measured. Thus, an increase in heart rate may be especially beneficial to those patients with CHB who also have valvular lesions which contribute to an increase in LVEDP and end-diastolic volume. Careful hemodynamic evaluation is helpful in determining appropriate therapy in these patients.

Aged

Coronary artery surgery for recurrent ventricular arrhythmias in patients with variant angina.

Two patients with Prinzmetal's variant angina had recurrent episodes of resting chest pain, ST segment elevation, QRS widening, ventricular tachycardia, and ventricular fibrillation. These eqisodes were unresponsive to medical therapy including lidocaine, procaine amide, and quinidine sulfate. Coronary arteriography revealed severe obstructive coronary artery disease, involving more than one coronary artery, in both patients. Aorticocoronary saphenous vein grafts were utilized to bypass significant disease in each patient. In one patient blood flow through the grafts was measured at 90 and 65 ml per minute, respectively, at operation and patent grafts were demonstrated six months postoperatively. Neither patient has had recurrence of chest pain or evidence of ventricular tachycardia at one year or 2 1/2 years postoperatively. Postoperative resting and maximal exercise ECG's are normal. Coronary artery surgery may be an effective method of therapy for ischemic ventricular tachycardia when medical therapy fails.

Adult

Bacterial endocarditis in idiopathic hypertrophic subaortic stenosis.

Bacterial endocarditis complicating idiopathic hypertrophic subaortic stenosis (IHSS) is uncommon but endocarditis may be the first clinical manifestation of IHSS. In this report of such a case, the aortic and the mitral valves were the sites of the bacterial infection. Many chordae tendineae to the mitral valve were ruptured from the extension of the infectious process. The endothelial lesions, which served as the seat for the bacterial infection on the anterior mitral leaflet, likely resulted from its abutting action against the septal prominence. Damage to the aortic valve leaflet may have resulted from abnormal valve motion caused by IHSS and created an environment conducive to endocarditis. This patient developed aortic insufficiency during the course of bacterial endocarditis, suggesting that the occasional association of aortic insufficiency in patients with IHSS may be secondary to healed endocarditis of the aortic valve.

Adult