[Non-compaction cardiomyopathy]. / Non-Compaction-Kardiomyopathie.

HISTORY AND ADMISSION FINDINGS: A 49-year-old man with loss of performance, ventricular ectopy and left bundle branch block was referred for diagnostic workup. He had dysmorphic skeletal features with shortening and muscular atrophy of arm and leg and syndactylia. INVESTIGATIONS: Echocardiography revealed peculiar hypertrabeculation of left ventricular myocardium which was confirmed by cardiac magnetic resonance imaging. Therefore a diagnosis of non-compaction cardiomyopathy was made. Holter monitoring showed non-sustained ventricular tachycardia, coronary heart disease was excluded by coronary angiography. TREATMENT AUND COURSE: The patient received optimal heart failure drug therapy. Because of malignant ventricular ectopy a biventricular ICD system (CRT-D system) was implanted. Signs and symptoms of heart failure have been stable for four years (NYHA-class II). Repeated sustained ventricular tachycardia were terminated by ICD overstimulation. A history of inadequate ICD shocks was due to incompliance of the patient regarding beta blocker use. CONCLUSION: Non-compaction cardiomyopathy is a rare but characteristic cause of heart failure which is associated with potential malignant arryhthmias. Diagnosis is based on echocardiographic workup with typical features. However, because of its low prevalence and awareness deficits in the medical community, non-compaction cardiomyopathy probably is diagnosed too late and not frequently enough.

[Recurrent familial cardiac myxomas and lentiginosis. Second recurrence in a 41-year-old female patient]. / Rezidivierende familiäre kardiale Myxome und Lentiginose. Zweites Myxomrezidiv bei einer 41-jährigen Patientin.

The case of a 41-year-old woman with recurrent cardiac myxomas and widespread lentiginosis is reported. The diagnosis of a Carney complex was established 7 and 25 years, respectively, after first manifestation of the cardiac myxomas in both the patient and her brother. This peculiar hereditary disease is commonly associated with multiple neoplasms and an endocrine overactivity, requiring a thorough examination of the patients and their relatives to detect additional typical manifestations.

[Differential therapy of heart failure. Which drug for which patient?]. / Differenzialtherapie der Herzinsuffizienz. Welche Substanz für welchen Patienten?

The implementation of guidelines for medical therapy of heart failure may be problematic for the following reasons: 1. Elderly patients and women were underrepresented in large clinical trials which may limit their therapeutic impact in these patients. 2. Therapeutic decisions are influenced by co-morbidities like renal failure, obstructive airway disease (COLD, Asthma), stroke, and diabetes mellitus. We therefore discuss the differential therapy of heart failure in view of particular patient subgroups.

Massive pulmonary embolism with large floating thrombus in the truncus of the pulmonary artery.

A conservative strategy with anticoagulation led to spontaneous dissolution of a large floating thrombus (7.0x0.5 cm) in the truncus of the pulmonary artery in a 51-year-old woman with massive pulmonary embolism (pulmonary emboli in both lungs down to the level of the segmental arteries). Interventional therapy such as thrombolysis or pulmonary thrombectomy was not considered to be appropriate for this patient because of the risk of disrupture and embolization of parts of this large central thrombus. We believe that in certain cases with massive pulmonary embolism and large floating central thrombi a conservative strategy with anticoagulation may be appropriate. Such cases may be observed more often in the future using the technique of spiral computed tomographic angiography.

Energetic stimulation of the heart.

The management of patients with acute heart failure includes frequently the use of positive inotropic agents in order to increase cardiac output and hence to maintain adequate tissue perfusion. The concept of energetic stimulation of the heart was invented in order to avoid deleterious effects of conventional inotropes, i.e. catecholamines and phosphodiesterase-inhibitors, on myocardial oxygen consumption and energy reserves and to circumvent potential dangerous side effects of the drugs. Metabolic support with glucose-insulin-potassium has proven efficious of improving cardiac performance and prognosis in the setting of the ischemic and postischemic heart. The glycolytic intermediate pyruvate increases contractile performance in isolated animal and human myocardium and improves short-time hemodynamics in patients with congestive heart failure, which is discussed in detail in the present article. Energetic stimulation with pyruvate could therefore be a new promising approach to the treatment of acute heart failure, if conventional therapy fails.

Pyruvate potentiates inotropic effects of isoproterenol and Ca(2+) in rabbit cardiac muscle preparations.

Catecholamines and elevated extracellular Ca(2+) concentration ([Ca(2+)](o)) augment contractile force by increased Ca(2+) influx and subsequent increased sarcoplasmic reticulum (SR) Ca(2+) release. We tested the hypothesis that pyruvate potentiates Ca(2+) release and inotropic response to isoproterenol and elevated [Ca(2+)](o), since this might be of potential importance in a clinical setting to circumvent deleterious effects on energy demand during application of catecholamines. Therefore, we investigated isometrically contracting myocardial preparations from rabbit hearts at 37 degrees C, pH 7.4, and a stimulation frequency of 1 Hz. At a [Ca(2+)](o) of 1.25 mM, pyruvate (10 mM) alone increased developed force (F(dev)) from 1.89 +/- 0.42 to 3.62 +/- 0.62 (SE) mN/mm(2) (n = 8, P < 0.05) and isoproterenol (10(-6) M) alone increased F(dev) from 2.06 +/- 0. 55 to 25.11 +/- 2.1 mN/mm(2) (P < 0.05), whereas the combination of isoproterenol and pyruvate increased F(dev) overproportionally from 1.89 +/- 0.42 to 33.31 +/- 3.18 mN/mm(2) (P < 0.05). In a separate series of experiments, we assessed SR Ca(2+) content by means of rapid cooling contractures and observed that, despite no further increase in F(dev) by increasing [Ca(2+)](o) from 8 to 16 mM, 10 mM pyruvate could still increase F(dev) from 26.4 +/- 6.8 to 29.7 +/- 7. 1 mN/mm(2) (P < 0.05, n = 9) as well as the Ca(2+) load of the SR. The results show that the positive inotropic effects of pyruvate potentiate the inotropic effects of isoproterenol or Ca(2+), because in the presence of pyruvate, Ca(2+) and isoproterenol induced larger increases in inotropy than can be calculated by mere addition of the individual effects.

Haemodynamic effects of intracoronary pyruvate in patients with congestive heart failure: an open study.

BACKGROUND: Pyruvate, as an intermediate in the Krebs cycle, is an important source of energy for myocardium and improves contractility of normal, hypoxic, and postischaemic animal myocardium. We investigated the effect of intracoronary pyruvate in patients with congestive heart failure. METHODS: Haemodynamic measurements were done in eight patients with dilated cardiomyopathy after two 15 min infusions of pyruvate into the left main coronary artery and after saline washout of pyruvate. FINDINGS: There were no significant differences between the two pyruvate concentrations. Application of pyruvate resulted in a 23% increase in cardiac index (p<0.05), a 38% increase in stroke-volume index (p<0.05), and a 36% decrease in pulmonary capillary wedge pressure (p<0.05). Heart rate decreased significantly by 11%. Mean aortic pressure and systemic vascular resistance did not change. Most of the effects of pyruvate were reversed 15 min after the infusion stopped. INTERPRETATION: Pyruvate has the profile of a favourable inotropic substance. Other modes of administration need to be studied.

Cardiac and hemodynamic effects of the sinus node inhibitor tedisamil dihydrochloride in patients with congestive heart failure due to dilated cardiomyopathy.

Clinical and experimental investigations have demonstrated an inverse relation between heart rate and myocardial performance in patients with congestive heart failure. Accordingly, this study was designed to investigate the hemodynamic effect of the novel bradycardic compound tedisamil in patients with heart failure. We hypothesized that tedisamil would reduce heart rate and thereby improve hemodynamic parameters of failing hearts with an inverse force-frequency relation. Tedisamil was administered intravenously in nine patients with dilated cardiomyopathy (NYHA II-III). Hemodynamic measurements by right heart catheterization were carried out at time points -30, 10, 20 min, 1, 2, 4, and 6 h. Tedisamil decreased heart rate significantly from 84 +/- 6 beats/min to 73 +/- 4 beats/min (at 10 min; p < 0.05). Stroke volume index remained unchanged, and cardiac index tended to decrease transiently. Mean blood pressure increased from 98 +/- 5 to 104 +/- 6 mm Hg (p < 0.05) because of an increase in systemic vascular resistance from 1,619 +/- 145 to 2,079 +/- 198 dyn x s x cm(-5) (at 20 min; p < 0.05). Diastolic pulmonary pressure and pulmonary vascular resistance showed similar changes. Pulmonary capillary wedge pressure increased from 12 +/- 3 to 16 +/- 4 mm Hg (at 20 min; p < 0.05). Although tedisamil resulted in a significant heart-rate reduction, this was not associated with an improvement of hemodynamics. This may be due to increased afterload of the left and right ventricle. In these patients, tedisamil increased vascular resistance, which is unwanted in the treatment of congestive heart failure.

Influence of the force-frequency relationship on haemodynamics and left ventricular function in patients with non-failing hearts and in patients with dilated cardiomyopathy.

In isolated human myocardium it was shown that a positive force-frequency relationship occurs in non-failing myocardium; however, the force-frequency relationship was found to be inverse in myocardium from failing human hearts. In order to investigate the clinical relevance of these experimental findings, the influence of heart rate changes on haemodynamics and left ventricular function was studied in eight patients without heart failure and in nine with failing dilated cardiomyopathy (NYHA II-III). Right ventricular pacing was performed at a rate slightly above sinus rate and at 100, 120 and 140 beats.min-1. Haemodynamic parameters were obtained by right heart catheterization and by high-fidelity left ventricular pressure measurements. Left ventricular angiography was performed at basal pacing rate and at 100 and 140 beats.min-1. With increasing heart rate, cardiac index increased in patients with normal left ventricular function from 2.9 +/- 0.2 to 3.5 +/- 0.21.min-1.m-2 (P < 0.01) and decreased continuously in patients with dilated cardiomyopathy from 2.6 +/- 0.1 to 2.2 +/- 0.1 l.min-1. m-2 (P < 0.05). With increasing heart rate, the maximum rate of left ventricular pressure rise increased in non-failing hearts from 1388 +/- 86 to 1671 +/- 88 mmHg.s-1 (P < 0.01) and did not change in failing hearts. Ejection fraction decreased from 27 +/- 3% to 19 +/- 2% in patients with dilated cardiomyopathy (P < 0.05) when the pacing rate was changed from 84 +/- 2 beats.min-1 to 140 beats.min-1, which was associated with a significant increase in end-systolic volume without significant changes in end-diastolic volume.(ABSTRACT TRUNCATED AT 250 WORDS)