Phillip K. Fulkerson

The Spectrum of Cardiac Defects in the Ehlers-Danlos Syndrome, Types I and III

Nineteen patients with types I and III Ehlers-Danlos syndrome were hospitalized at our institution between 1973 and 1978. Chest roentgenogram, electrocardiogram, and echocardiogram were done; 11 patients underwent cardiac catheterization. Thirty-five cardiac or great vessel abnormalities were detected. Fifteen patients had nitral valve prolapse; six also had tricuspid valve prolapse. Dilatation of the aortic root or extasia of the sinuses of Valsalva, or both, occurred in six patients. Dilatation of the pulmonary artery and annulus caused pulmonary regurgitation in one patient. Congenital heart defects included bicuspid aortic valve (two), pulmonary valvular stenosis (one), ventricular septal defect (two), and an atrial septal defect (one). The apparent high prevalence of cardiovascular abnormalities in hospitalized patients with types I and III Ehlers-Danlos syndrome necessitates a careful cardiovascular evaluation. Conversely, Ehlers-Danlos syndrome types I and III should be excluded in patients with mitral or tricuspid valve prolapse, great vessel dilatation, and congenital heart defects.

Cardiac function in Duchenne's muscular dystrophy: Results of 10-year follow-up study and noninvasive tests

The purposes of this study were to: (1) evaluate the progression of cardiac involvement in Duchennes muscular dystrophy using systolic time intervals (PEP/LVET); (2) determine if the degree of cardiac involvement bears a relation to the severity of skeletal muscle disease; and (3) describe the M-mode and two-dimensional echocardiographic findings. In 1970, systolic time intervals were studied in 16 patients. During the 10-year interim, two patients were lost to follow-up study, and five patients died. Nine remaining patients were re-studied in 1980 with M-mode and two-dimensional echocardiography as well as systolic time intervals. The PEP/LVET value of these nine patients increased from 0.37 +/- 0.05 (mean +/- SD) in 1970 to 0.47 +/- 0.07 (p less than 0.005) in 1980. Three patients remained ambulatory, and their PEP/LVET value (0.41 +/- 0.04) was significantly better than that of the nonambulatory patients (0.50 +/- 0.07, p less than 0.05). The M-mode echocardiography percentage diameter change was also worse in the nonambulatory group (21 +/- 4 percent versus 34 +/- 7 percent, p less than 0.02). The five patients who were nonambulatory in 1970 died in the intervening 10 years. This study demonstrated that the heart disease of Duchennes muscular dystrophy is progressive and that the severity of skeletal muscle disease is probably associated with the degree of cardiac dysfunction.

Relationship of angiographic and echographic dimensions in chronic left ventricular dilatation

Recently discrepancies have been reported between echocardiographic (Echo) % delta D and angiocardiographic (Angio) ejection fraction (EF), particularly in valvular regurgitation. One hundred and twelve patients with varying degrees of LV dilatation were studied in the right anterior oblique position with M-mode Echo. None had localized contraction abnormalities. There were 20 normals, 33 with primary myocardial disease, and 59 with mitral and/or aortic regurgitation. Echo end-diastolic diameter (EDD) and end-systolic diameter (ESD) were consistently smaller than Angio calculated EDD and ESD, and the difference was magnified at larger EDDs (p less than 0.01). The result is a relatively poor correlation of Echo % delta D and Angio EF (r = 0.69), compared to r = 0.98 for Angio % delta D and EF, both from Angio visualization. However, if valvular disease patients are excluded, the correlation improves to 0.82. The mechanisms for these disparities include increased sphericity as the ventricle dilates, and in the case of valvular disease where the EF is better for any degree of dilatation, the echo measurement errors for EDD and ESD are different.

Effect of Propranolol on Postexercise Left Ventricular Ejection Time Index

The net delta left ventricular ejection time index 4 minutes after exercise is prolonged in many patients with coronary artery disease. This prolongation is thought to be due to the lack of response of the ischemic myocardium to adrenergic stimulation and has been proposed as a measure of myocardial ischemia. In this study, the effect of beta adrenergic blockade on net delta left ventricular ejection time was studied in nine normal subjects (Group A) and in eight patients with stable angina and coronary artery disease (Group B). In Group A, a treadmill exercise test was performed for 10 minutes before and after administration of propranolol, 160 mg daily, for 2 days. The postexercise net delta left ventricular ejection time was significantly greater after propranolol (mean +/- standard error of the mean 12 +/- 4 versus 35 +/- 4 ms, p less than 0.01). In group B a maximal treadmill exercise test was performed before and after therapy with propranolol. Only patients with a normal net delta left ventricular ejection time before propranolol were selected. The net delta left ventricular ejection time again increased significantly after propranolol (11.5 +/- 4 versus 35.3 +/- 5 ms p less than 0.01). It is concluded that prolongation of postexercise net delta left ventricular ejection time cannot be used to diagnose ischemia in patients who are receiving propranolol therapy. Our data support the hypothesis that prolongation of net delta left ventricular ejection time after exercise is caused by an impaired myocardial response to catecholamines, whether due to ischemia or effective beta adrenergic blockade.

A paucity of chronic electrocardiographic changeswith adriamycin therapy

Serial electrocardiograms (ECGs) of 49 patients receiving adriamycin were analyzed for the development of persistent changes. The ECG changes were compared with those of a control group of 20 patients receiving other chemotherapeutic drugs, which were comparable to the additional chemotherapy received by the adriamycin patients. The only chronic ECG changes noted with adriamycin over control were the loss of P wave amplitude in the greater than 500 mg/m2 dose subgroup and the clockwise rotation of the precordial QRS in the 250-500 mg/m2 dose subgroup. In contrast, systolic time intervals demonstrated a gradual diminution in left ventricular function at increasing doses of adriamycin. The electrocardiogram itself appears to be of limited value in the assessment of cardiac toxicity with adriamycin therapy.