Miocardiopatia não compactada: uma visão atual

Abstract

Isolated non-compaction cardiomyopathy is a rare disease that is likely to develop in the embryonic period. It is caused by the intrauterine arrest of the myocardial compaction process in the beginning of the fetal development. It is characterized by prominent myocardial trabeculations and deep intertrabecular recesses, as well as the thickening of the myocardium into two distinct layers (compacted and not compacted). Even though this disease is said to be prevalent in the pediatric population or together with congenital heart disease, one can understand that this disease occurs in isolation, because the diagnosis is becoming more common in adult patients that have no other heart disease. The clinical manifestations vary greatly, because they range from absence of symptoms to congestive heart failure, arrhythmias and systemic thromboembolism. Echocardiography is the most widely used diagnostic procedure, but the little knowledge about this disease, its similarity to other myocardial diseases and the limitation of the echocardiographic technique used delay the diagnosis. The purpose of this review is to show that that other imaging techniques, such as MRI, CT and left ventriculography have emerged as diagnostic alternatives.

Pediatric Cardiomyopathies: causes, epidemiology, Clinical course, Preventive Strategies and Therapies

“…LV noncompaction is thought to result from the arrest of normal endomyocardial embryogenesis during weeks 5 and 8 [92,93]. Although the cause of this arrest is unknown, LV noncompaction has been associated with other congenital heart defects.…”

Isolated Ventricular Noncompaction Cardiomyopathy Presenting as Recurrent Syncope

“…Symptomatic patients with impaired ejection fraction should be refrained from participating in competitive sports. AICD, cardiac resynchronization therapy, and evaluation for heart transplant should be considered in patients with NYHA class III-IV heart failure and permanent atrial fibrillation or with bundle branch block [23]. Considering NYHA class III symptoms, low ejection fraction, and recurrent syncope, we proceeded with AICD implantation in our patient.…”

Sildenafil for Noncompaction Cardiomyopathy Treatment in a Child: Case Report

“…It might result from a failure in compaction of the ventricular myocardium between weeks 5 and 8 of the embryonic life, leading to the persistence of myocardial trabeculations and deep recesses, which communicate with the ventricular cavity, generating myocardial thickening in two distinct layers (compacted and noncompacted). Initially reported in the pediatric population or with a congenital cardiopathy, it also affects adults with no other heart disease 2,3 .…”

“…However, the literature has reported a genetic aspect emphasizing the relationship of the disease with different genes, such as the mutation of gene G4.5 in families with severe infantile noncompaction cardiomyopathy, mutations P121L, CYPHER/ZASP, E101K, and a locus containing the disease gene in chromosome 11p15 3 .…”

“…The major complications of patients with noncompaction cardiomyopathy are pulmonary thromboembolism, arrhythmias and CHF 3 . In addition to the usual treatment of CHF, new classes of drugs, such as the phosphodiesterase type 5 (PDE-5) inhibitors, have been studied 4 .…”