Acute on Chronic Heart Failure Secondary to Left Ventricular Noncompaction

Abstract

Left ventricular noncompaction (LVNC) is a rare cardiomyopathy characterized by hypertrabeculations and intertrabecular recesses most often seen in the left ventricle (LV). The patient may be asymptomatic or present with heart failure, arrhythmia, and sudden death. We discuss a previously healthy 7-year-old male who presented to the Emergency Department (ED) multiple times over a three-week period. His complaints evolved over the course of his illness, initially presenting with fatigue and suicidal ideation, followed by diffuse abdominal pain. Prior to his ICU admission, he had been discharged from the ED twice, due to well appearance and reassuring lab findings. He returned to the ED a final time with severe venous congestion and cardiogenic shock with acute hepatic injury. Echocardiogram revealed LV apical hypertrabeculation with a severe dilated cardiomyopathy and biventricular failure along with a large thrombus in the left ventricular cavity. Congestive heart failure and anticoagulation therapy was initiated, and the patient went on to biventricular assist device (BiVAD) placement and cardiac transplant. Although LVNC is rare, pediatric heart failure does present to the general pediatrician and has high morbidity and mortality. The presenting symptoms can be obscure and pose a challenge to pediatricians. This case report and review will assist in familiarizing the general pediatrician with pediatric heart failure presentation, treatment, and course.

Non-Compacted Cardiomyopathy in Children and Adolescents: From the Challenge of Echocardiographic Diagnosis to Clinical Follow-Up

“…Corroborating the literature, the case series studied shows pediatric NCM heterogeneity from clinical presentation to diagnosis ranging from asymptomatic patients to ventricular arrhythmia and HF or in the context of genetic syndrome investigation 1,3,4,[13][14][15] . HF is the most common clinical presentation of NCM 1-4, 14,16,17 , so the recognition of the several HF signs and symptoms in children essential to its diagnosis 18 . HF was also the main clinical presentation in the described group of patients.…”