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Cardiac catheterization, angiocardiography and ventricular muscle biopsy were performed in forty patients with idiopathic cardiomyopathy and included 21 cases of hypertrophic cardiomyopathy and 19 cases of congestive cardiomyopathy. 1) Cardiac catheterization revealed normal cardiac index and stroke index in both types, although there was a slight tendency toward decrease in cases of CCM. HCM showed slightly elevated right ventricular end-diastolic pressure and left ventricular end-diastolic pressure with a high incidence of atrial kick. CCM showed an elevated mean pulmonary artery, mean pulmonary wedge and left ventricular end-diastolic pressure. 2) Angiocardiographic findings revealed that in HCM left ventricular end-diastolic volume as well as left ventricular end-systolic volume, ejection fraction, meanVcf and MNSER were within normal range, and left ventricular anterior wall thickness, left ventricular mass and shortening of short axis in systole were increased. In CCM left ventricular end-diastolic volume and end-systolic volume increased, and ejection fraction, meanVcf, MNSER were decreased. The left ventricular anterior wall thickness was normal, and the left ventricular mass was smaller compared to the volume. The shortening of long and short axes in systole was slight. Left ventricular asynergy and mitral regurgitation occurred frequently. Coronary cineangiograms revealed normal patterns in both types. 3) Histological findings revealed hypertrophy of myofibers, degenerative changes, i.e. scarcity of myofibrils, deformity of nucleus and vacuolization of myocardial fibers, and collagen proliferation in both types. 4) No definite relationship was seen between parameters of left ventricular function and the findings of biopsied left ventricular muscle except for increase in wall thickness which might be apparently due to hypertrophy of the myocardial fibers.
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PMID:Angiocardiograms and hemodynamics in idiopathic cardiomyopathy, with reference to histology of biopsied ventricular myocardium. 15 67

Coronary sinus blood flow (CSBF) was measured by the dye dilution method in 18 patients with idiopathic cardiomyopathy including 13 with hypertrophic type and 5 with congestive type, and the capacity for increasing CSBF in response to handgrip exercise was studied in 14 patients. CSBF at rest ranged from 41 to 236 ml/min/M2. Average CSBF of 114 +/- 12 ml/min/M2 (112 +/- 14 in hypertrophic type and 119 +/- 25 in congestive type) was significantly larger than control value of 77 +/- 6 presented previously (p less than 0.01). Handgrip exercise at 30% maximal effort for 3 min resulted in the increase in CSBF, averaging 23 +/- 8 ml/min/M2 (22 +/- 8%). However, in about one-half of cases, the percent increase in CSBF was much smaller than the percent increase in effort index which represents myocardial oxygen demand. The change of CSBF was not correlated with the changes of left ventricular systolic pressure, cardiac output, and stroke work. The diminished response of CSBF to isometric handgrip exercise was ascribed to the decreased coronary vascular reserve in this disorder.
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PMID:Effects of isometric handgrip exercise on coronary sinus blood flow in idiopathic cardiomyopathy. 91 53

Antiarrhythmic and hemodynamic effects of i.v. disopyramide phosphate (1.7 mg/kg b.wt. over 2 min) have been studied in nine patients, several in various degrees of cardiac decompensation, with sinus rhythm and persistent ventricular ectopic beats (VEBs). In one case with primary cardiomyopathy, with greater than 30 VEBs/min, disopyramide (DE) abolished the arrhythmia for 30 min, but precipitated brief dysponea. Other side-effects were tolerable and mainly attributable to anticholinergic effects of the drug. DE either abolished or significantly reduced the arrhythmia in all cases. For 30 min, only one patient showed VEBs, and in three patients no VEBs were seen for three hours. Changes in cardiac output and pulmonary artery (PAP) and central aortic pressures were measured in eight patients. Negative inotropic effects were indicated in seven by an increased diastolic PAP/stroke volume ratio and in seven by a decreased central aortic (dp/dt)max. Patients with high control values for diastolic PAP showed marked reductions in cardiac output, stroke volume and stroke work. In predicting myocardial depressant effects of DE, the control values for diastolic PAP seemed to be superior to central venous pressure, cardiac index and systolic time intervals. Mean arterial pressure measured 5 and 10 min after drug administration showed no significant change, indicating that vasoconstrictor reflexes were well preserved, and a pressure level significantly above the control value was reached from the 20th min. It is concluded that DE is potent in suppressing VEBs but exerts negative inotropic effects that may be of clinical importance. The optimal antiarrhythmic dose is probably lower than that used in the present study.
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PMID:Hemodynamic and electrocardiographic effects of disopyramide in patients with ventricular arrhythmia. 125 72

Factors determining prognosis in 100 patients with recent onset of congestive heart failure (CHF) were evaluated. The 1 year, 3 year, 5 year, and 10 year survival rates in the entire CHF group were 78.5%, 59.8%, 50.4% and 14.7%, respectively. No correlations between age, sex, heart rate and cardiothoracic ratio, and the cumulative survival rate were found. The prognosis of patients with CHF due to underlying coronary artery disease or primary cardiomyopathy was poor compared with that of patients with other types of heart disease. Patients whose NYHA classification was class III or VI had a significantly lower survival rate than those in class II. Patients with lower left ventricular stroke work and consecutive ventricular premature depolarization also had a significantly lower survival rate. These results suggest that functional status, underlying heart disease, left ventricular stroke work, and the presence of ventricular tachycardia provide important information regarding the long-term prognosis in patients with congestive heart failure.
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PMID:Long-term prognosis of patients with congestive heart failure. 233 33

Despite success in adults, heart transplantation (HT) is still considered by many as only desperation therapy for children with end-stage heart disease. Thus, of 30 pediatric patients undergoing HT at our center, only seven (23%) patients had not developed pulmonary hypertension with increased pulmonary vascular resistance (PH) or hemodynamic decompensation (HD) requiring inotropic support at the time of transplantation. We have retrospectively reviewed the effect of preoperative PH, HD, and seven other potential risk factors on survival of our pediatric heart transplant recipients. All 30 patients, aged 5 days to 18 years, had New York Heart Association class III or IV symptoms. Twenty had idiopathic cardiomyopathy, nine had congenital lesions, and one infant had a large left ventricular tumor. A univariate and multivariate Cox proportional-hazards analysis was performed examining the effects of nine variables on survival after transplantation: PH, HD, age, need for hospitalization, congenital heart disease, need for surgical pulmonary artery reconstruction, prior stroke, history of cardiac arrest(s), and mechanical ventilator dependence. One-year actuarial survival for the entire series was 66% and was 100% for the seven patients with neither PH nor HD. None of the nine potential risk factors was a statistically significant predictor of risk, yet the combined presence of PH and HD represented a highly significant predictor of mortality (relative risk, 4.08: 1; p less than 0.002). One-year actuarial survival of the 10 patients with this combination was 30% versus 84% of those without the combination.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Optimal timing of pediatric heart transplantation. 280 8

The hemodynamic effects of combined administration of ibopamine (Ib) (150 mg orally) with nitroprusside (NP) (50-150 micrograms/min intravenously) were compared with those of NP alone in 17 patients with severe congestive heart failure due to coronary artery disease (7 patients) or idiopathic cardiomyopathy (10 patients). Hemodynamic measurements were obtained using a Swan-Ganz thermodilution catheter and a bedside thermodilution cardiac output computer. Nitroprusside alone produced a significant decrease (-12.4%) in mean arterial pressure, mean pulmonary arterial pressure (-28.3%), and systemic vascular resistance (-22.6%), and a significant increase in stroke volume index (23.1%). The administration of combined NP and Ib produced a further significant increase of stroke volume index (20.1%) with a concomitant and significant reduction of systemic vascular resistance (-19.4%); heart rate, mean systemic and pulmonary arterial pressures did not change significantly from the values observed with NP alone. Moreover, stroke work index, although not significantly modified with the vasodilator alone, was significantly increased over control values with NP + Ib association. Although NP alone induced similar effects in both the ischemic and idiopathic cardiomyopathies, the association of Ib gave a more favorable, though not significant, hemodynamic response in the subjects with primitive cardiomyopathy than in the ischemic ones. Thus, the association of Ib to NP therapy, in patients with congestive heart failure, further increases stroke volume index and stroke work index with a concomitant reduction of systemic vascular resistance, without any significant change in mean systemic and pulmonary arterial pressures, or heart rate. These results point out the possibility of associating Ib with other orally active vasodilators in the chronic treatment of congestive heart failure.
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PMID:Hemodynamic advantage of combined administration of oral ibopamine and nitroprusside in patients with ischemic and idiopathic congestive cardiomyopathy. 402 36

The hemodynamic effects of a new cardioselective beta agonist, prenalterol, were evaluated in 12 patients with moderate or severe impairment of left ventricular function due to coronary heart disease or primary cardiomyopathy. In doses up to 7 mg the drug led to a substantial increase of left ventricular pressure rise (+55%) and mean circumferential fiber shortening (+59%) and a decrease of left ventricular end-diastolic pressure (-52%), mean pulmonary artery pressure (-24%) and pulmonary vascular resistance (-37%) indicating augmented myocardial contractility and reduced left ventricular preload. Cardiac output was increased only in 4 of 12 patients, heart rate, left ventricular systolic and mean right atrial pressures and the pressure-rate product as an index for myocardial oxygen demand remained essentially unchanged. The same is true for stroke index, stroke work index, total peripheral resistance, left ventricular end-diastolic and end-systolic volume and ejection fraction. The positive inotropic effect was achieved with good tolerance and without arrhythmogenic or other side effects. Prenalterol may be especially useful in patients with low sympathetic activity and hypotension. In patients with diffuse congestive cardiomyopathy, high sympathetic activity, pronounced peripheral vasoconstriction and normal blood pressure, vasodilator therapy alone or in combination with prenalterol should be considered.
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PMID:Hemodynamic assessment of prenalterol: a cardioselective beta agonist in patients with imparied left ventricular function. 612 57

We evaluated the hemodynamic effects of nifedipine in 10 symptomatic patients with chronic refractory heart failure due to idiopathic cardiomyopathy. Nifedipine significantly increased cardiac index (from 1.80 +/- 0.4 to 3 +/- 0.6 L/min/m2), stroke volume index (from 21 +/- 6 to 33 +/- 8 ml/beat/m2), and stroke work index (from 17.9 +/- 7 to 25.5 +/- 7 g-m/m2). The drugs also produced a significant decrease in left ventricular filling pressure (from 24.6 +/- 3 to 19 +/- 2 mm Hg), mean blood pressure (from 86 +/- 9 to 74 +/- 5 mm Hg), mean pulmonary arterial pressure (from 31.9 +/- 5 to 25.6 +/- 3 mm Hg), total systemic vascular resistance (from 2,104 +/- 329 to 1,088 +/- 249 dyn/s/cm-5), and pulmonary vascular resistance (from 200 +/- 71 to 107 +/- 50 dyn/s/cm-5). Heart rate remained unchanged. In all patients maintained on nifedipine therapy, repeat hemodynamic studies at 2 months revealed sustained effects, and all patients had symptomatic improvement of at least one New York Heart Association (NYHA) functional class. Long-term treatment was well tolerated. Forty-eight hours after discontinuation of nifedipine administration the hemodynamic benefits were lost. We conclude that nifedipine may be of value for long-term ambulatory therapy of severe chronic heart failure.
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PMID:Oral nifedipine in the long-term management of severe chronic heart failure. 618 18

To evaluate the hemodynamic effects of the beta-adrenergic receptor agonist, terbutaline sulfate, when given by inhalation in ventricular dysfunction, 0.5 mg of the agent was given by nebulizer to 13 patients with congestive heart failure (nine coronary heart disease and four with idiopathic cardiomyopathy). Data were obtained before and 10 and 30 minutes post inhalation, by right heart catheterization and by gated equilibrium radionuclide ventriculography. All patients responded with increased cardiac output (3.5 to 4.3 L/min, p less than 0.01) and stroke volume (40 to 49 ml, p less than 0.01) without change in heart rate. Decreases occurred in peripheral vascular resistance (1924 to 1443 dsc-5, p less than 0.01), left ventricular filling pressure (21 to 15 mm Hg, p less than 0.01), and systemic arterial oxygen tension (81 to 72 mm Hg, p less than 0.05). both left and right ventricular ejection fractions rose (0.24 to 0.38 and 0.36 to 0.51, both p less than 0.01) with concomitant declines in biventricular end-diastolic volumes. All variables indicated changed rapidly at 10 minutes post inhalation and returned to control levels by 30 minutes after the agent. Thus moderate inhaled doses of terbutaline produce prompt, potent, and transient salutary hemodynamic effects due to its peripheral vasodilator and cardiotonic properties, without untoward arrhythmogenic or anginal provoking influences in the present study.
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PMID:Hemodynamic effects of inhaled terbutaline in congestive heart failure patients without lung disease: beneficial cardiotonic and vasodilator beta-agonist properties evaluated by ventricular catheterization and radionuclide angiography. 722 95

Circulatory responses to the upright tilt were studied in 20 normal subjects and 27 cardiac patients with ischemic heart disease or idiopathic cardiomyopathy. In normal subjects, the upright tilt caused obvious increases in heart rate and diastolic pressure, a slight decrease in systolic pressure and marked decreases in cardiac output and stroke volume. The circulatory changes during the tilt were less pronounced in the cardiac patients as compared with the normal subjects. The reductions of cardiac output and stroke volume and the increase in total peripheral resistance were all significantly diminished. A paradoxical increase in cardiac output during the tilt, an observation hitherto not well recognized, was observed in 5 cases with low cardiac index during the control period. Although several possibilities can be considered for the explanation of the diminished, sometimes paradoxical, circulatory responses to the tilt in cardiac patients, the improvement of the function of the diseased heart by preload reduction was proposed as an important factor. There was a significant negative correlation between the per cent changes of cardiac output and the per cent changes of PEP/LVET. It was suggested that the measurements of systolic time intervals during the tilt might be useful for evaluating the severity of the hemodynamic derangement in cardiac patients.
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PMID:Alterations of circulatory responses to upright tilt in cardiac patients. 730 26


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