cardiovascular-agents and Cardiomyopathy--Restrictive

When amyloidosis affects the heart, a devastating and progressive process can lead to congestive heart failure, arrhythmias, conduction abnormalities, angina, and death. The signs and symptoms of cardiac amyloidosis are generally dominated by diastolic heart failure resulting from restrictive cardiomyopathy. Amyloid infiltration of the heart initially causes mild diastolic dysfunction, but late disease produces a thickened heart wall with a firm and rubbery consistency, which worsens cardiac relaxation and diastolic compliance. Patients usually complain of progressive dyspnea from congestive heart failure, chest discomfort secondary to microvascular involvement, and weight loss, which might be a manifestation of cardiac cachexia. Echocardiographic findings include nondilated ventricles with concentric left ventricular thickening, right ventricular thickening, prominent valves, dilated atria, and thickening of the interatrial septum. Recent advances in our understanding of the pathophysiology of amyloid have allowed the various types to be differentiated, which has led to targeted therapy for each unique pathophysiologic process.

Topics: Amyloidosis; Anti-Infective Agents; Biomarkers; Cardiac Catheterization; Cardiomyopathy, Restrictive; Cardiovascular Agents; Drugs, Investigational; Echocardiography; Electrocardiography; Heart Diseases; Heart Failure; Heart Transplantation; Humans; Liver Transplantation; Magnetic Resonance Imaging; Stem Cell Transplantation; Treatment Outcome

A cardiac localization is one of the most severe manifestations of sarcoidosis and may cause sudden death (ventricular tachycardia or atrial ventricular block III) or restrictive cardiomyopathy. Lesions are most frequently observed in the interventricular septum and the free left wall. Granulomatous infiltation can provoke nonspecific clinical, electric and echocardiographic signs, which, associated with regressive dipyridamol uptake on tomoscintigraphy, are suggestive of cardiac sarcoidosis. The diagnosis of cardiac sarcoidosis is based on the presence of systemic sarcoidosis, histological evidence of granuloma and the lack of another cause of cardiomyopathy. Corticosteroid therapy is indicated, associated with specific cardiologic treatments.

Topics: Anti-Inflammatory Agents; Biopsy; Cardiomyopathies; Cardiomyopathy, Restrictive; Cardiovascular Agents; Death, Sudden, Cardiac; Dipyridamole; Echocardiography; Electrocardiography; Heart Block; Humans; Immunosuppressive Agents; Magnetic Resonance Imaging; Sarcoidosis; Steroids; Tachycardia, Ventricular; Vasodilator Agents

The management of patients with hypertrophic cardiomyopathy (HCM) and refractory symptoms due to massive hypertrophy and severe diastolic dysfunction represents a real challenge for the clinical cardiologist. Such patients often require novel therapeutic approaches, both invasive and pharmacological, involving multidisciplinary teamwork; however, the implementation of potentially viable treatment options is hindered by lack of disease-specific evidence. We report the case of a young woman with severe HCM and restrictive physiology, who underwent extensive myectomy via the transaortic and transapical approach, followed by biventricular pacing for cardiac resynchronization, with significant but incomplete symptomatic improvement. The subsequent introduction of ranolazine, based on promising preclinical data, has led to an excellent final result. An ongoing randomized clinical trial is currently testing the efficacy of ranolazine in symptomatic HCM.

Topics: Acetanilides; Adult; Cardiac Pacing, Artificial; Cardiac Surgical Procedures; Cardiomyopathy, Hypertrophic; Cardiomyopathy, Restrictive; Cardiovascular Agents; Catheter Ablation; Enzyme Inhibitors; Female; Humans; Pacemaker, Artificial; Piperazines; Ranolazine; Severity of Illness Index; Treatment Outcome

Topics: Acetanilides; Cardiomyopathy, Hypertrophic; Cardiomyopathy, Restrictive; Cardiovascular Agents; Enzyme Inhibitors; Female; Humans; Piperazines

Medically refractory heart failure may be present in children with cardiomyopathy (CMP) or complex congenital heart disease (CHD). In adults, the surgical management of this condition is either heart transplantation or the Batista operation. From March 1995 to January 2000, a total of 6 children, aged from 1 to 16 years, with medically refractory heart failure associated with CMP or complex CHD underwent cardiac transplantation and one of them also had the Batista operation as a bridge to transplantation. One of the 6 patients died of intractable sepsis 17 days after the operation, but the other 5 were discharged with satisfactory hemodynamics. Immunosuppressive agents, including azathioprine, cyclosporin or FK-506, were given. One patient experienced moderate acute rejection, but it was controlled by FK-506, OKT-3 and solumedrol. However, another suffered from lymphoproliferative disease 8 months after transplant, but it was controlled by intravenous immunoglubulin, alpha-interferon and acyclovir. Cardiac function during serial follow-up (range, 1 month to 5 years) revealed normal systolic and diastolic function and none received any anticongestive medications. Almost all patients received an oversized donor heart. The left ventricle (LV) mass was remodeled, initially as an decrease and later as an increase. The patient who underwent the Batista operation was discharged 1 month after the operation with an increased LV ejection fraction (from 10% to 22%). She was successfully bridged to heart transplantation 7 months after the Batista operation. The results of cardiac transplantation in growing children are satisfactory and remain the mainstay of surgical treatment for medically refractory heart failure in these patients. However, with a shortage of donor hearts, the Batista operation may be adopted as a bridge to heart transplant with a fair response.

Topics: Adolescent; Cardiomyopathy, Dilated; Cardiomyopathy, Restrictive; Cardiovascular Agents; Child; Child, Preschool; Drug Resistance; Female; Follow-Up Studies; Graft Rejection; Heart Defects, Congenital; Heart Failure; Heart Function Tests; Heart Transplantation; Heart Ventricles; Humans; Immunosuppressive Agents; Male; Organ Size; Postoperative Complications; Severity of Illness Index; Survival Rate; Treatment Outcome; Ventricular Remodeling