Eduard Nemecek

Contemporary Treatment of Amyloid Heart Disease

The amyloidoses represent a group of diseases characterized by extracellular deposition of abnormal protein, amyloid, which is formed by insoluble extracellular fibrils in β-pleated sheets. Although cardiac involvement may occur in all types of amyloidoses, clinically relevant amyloid cardiomyopathy is a typical feature of AL amyloidosis and transthyretin-related amyloidoses. Congestive heart failure represents the commonest manifestation of amyloid heart disease. Noninvasive imaging techniques, especially echocardiography and cardiac magnetic resonance, play a major role in the diagnosis of amyloid cardiomyopathy; however, histological confirmation and exact typing of amyloid deposits is necessary whether in extracardiac location or directly in the myocardium. Early diagnosis of amyloid heart disease is of utmost importance as the presence and especially the severity of cardiac involvement generally drives the prognosis of affected subjects and plays a major role in determining the intensity of specific treatment, namely in AL amyloidosis. The management of patients with amyloid heart disease is complex. Loop diuretics together with aldosterone antagonists represent the basis for influencing signs of congestion. In AL amyloidosis, high-dose chemotherapy followed by autologous stem cell transplantation is generally considered to be a front-line treatment option, if the disease is diagnosed at its early stage. The combination of mephalan with dexamethasone has been the standard therapy for severely affected individuals; however, the combinations with several novel agents including immunomodulatory drugs and bortezomibe have been tested in clinical trials with promising results. New therapeutic substances with the potential to slow or even stop the progression of transthyretin-related amyloidosis are also extensively studied.

Absence of Borrelia burgdorferi in the myocardium of subjects with normal left ventricular systolic function: a study using PCR and electron microscopy

BACKGROUND Several studies have demonstrated the presence of the Borrelia burgdorferi (Bb) genome in the myocardium of patients with dilated cardiomyopathy (DCM). To further support a causal relationship between the presence of Bb in the heart muscle and the development of DCM, demonstration of the absence of Bb in the myocardium of subjects with normal left ventricular (LV) systolic function is needed. AIM To determine the prevalence of Bb by polymerase chain reaction (PCR) and electron microscopy (EM) in individuals with normal LV systolic function and no history suggestive of myocarditis. METHODS We investigated 50 patients (67 ± 9 years, 15 women) with normal LV ejection fraction (EF) ≥ 50% undergoing cardiac surgery. During surgery, four samples from the right atrial appendage were obtained and subsequently examined by PCR and EM for the presence of Bb, and by immunohistochemistry to detect inflammatory cells. Serological testing of antibodies against Bb was also performed. RESULTS Neither PCR nor EM detected Bb in any of the subjects. Immunohistological examination revealed myocardial inflammation in 2 individuals (4%). Serological analysis by enzyme-linked immunosorbent assay demonstrated IgM antibodies against Bb in 4% and IgG antibodies in 12% of the study cohort; Western blot revealed IgM as well as IgG positivity in 14% of patients. CONCLUSIONS The absence of Bb in the myocardium of individuals who undergo cardiac surgery and have normal LV systolic function supports the idea of Bb pathogenicity in the development of DCM.

New Therapeutic Aspects of Inflammatory Cardiomyopathy

Inflammatory cardiomyopathy is a term used for left ventricular systolic dysfunction associated with myocarditis. In order to establish definitely the diagnosis, an endomyocardial biopsy (EMB) must be performed. Based on the results of EMB analysis, specific treatment may be added to conventional heart failure therapy. Immunosuppressive therapy in patients with non-viral inflammatory cardiomyopathy has been shown to be effective, and there is also some evidence that antiviral or antimicrobial therapy may be beneficial.

Monitoring Cardiac Function During Idebenone Therapy in Friedreich's Ataxia

Friedreichs ataxia (FA) is associated with progressive cardiac hypertrophy resulting from a genetic abnormality in the frataxin gene. Cardiac involvement is the most common cause of death (59%) in FA patients. Cardiac related death occurs at a significantly younger age than non-cardiac related death. Idebenone is a short-chain quinone analogue with a potent free-radical scavenger action. This drug has the potential to preserve and even improve mitochondrial function.Studies on Idebenone treatment showed rather conflicting results on FA cardiomyopathy. The present article reviews the clinical features of FA cardiomyopathy, imaging techniques used to diagnose, follow and monitor therapy which aimed to revert FA cardiomyopathy.

Needle fragment embolism into the right ventricle: a rare cause of chest pain case report and literature review

SummaryChest pain in young adults is usually self-limited and of benign etiology. However, rare causes of chest pain must be considered in patients for whom initial diagnostic tests are negative, particularly if unusual risk factors are identified. The authors present a rare case describing a 27-year-old male intravenous drug user who developed transient chest pain most likely secondary to pericardial irritation caused by a needle fragment that embolized from a peripheral vein to his right ventricle. The current literature on intracardiac needles and similar foreign bodies is discussed, providing insight to the epidemiology, complications, and treatment of such patients.

Herpes simplex virus-induced cardiomyopathy successfully treated with acyclovir

ZusammenfassungDie entzündliche dilatative Kardiomyopathie stellt eine erworbene Form der dilatativen Kardiomyopathie dar, wobei Virusinfektionen die häufigste Ursache darstellen. Im Gegensatz zu anderen kardiotropen Viren, wird das Herpes simplex Virus (HSV) nur selten in Biopsien des Myokards von Patienten mit dilatativer Kardiomyopathie gefunden. Wir berichten über einen Fall einer erfolgreichen Behandlung mit Azyclovir einer durch HSV induzierten Kardiomyopathie.SummaryInflammatory dilated cardiomyopathy (DCMi) represents an acquired form of dilated cardiomyopathy. Viral infection is the most common cause of DCMi. In contrast with other cardiotropic viruses, herpes simplex virus (HSV) is a very rare finding in endomyocardial biopsies of patients with dilated cardiomyopathy. We report a case of HSV-induced cardiomyopathy successfully treated with acyclovir.