Non-Compacted Myocardium (Ncm) with Pre-Exitacion Syndrome: Report of Two Cases

Abstract

Nineteen (19) years old Colombian male,who consulted the cardiology service, for a 7 years history of Grade II NHYA dyspnea, syncope in the last 4 years, and more recently intermittent chest palpitations, accompanied by atypical chest pain.The patient underwent surgical closure of patent arterious ductus at 10 years of age, and ablation of a right septal posterior pathway associated with supraventricular tachycardia (SVT) with. WPW syndrome, at 16 years of age. There is a history of allergy to dipyrone and metoclopropamide. Physical examination and EKGOn admission blood pressure was 110/60mm/Hg, heart rate 136 beats per minuteand respiratory rate 18 breaths per minute. No other abnormalities were found. FBC, renal function tests, blood glucose, coagulation and electrolytes were within normal limits. Natriuretic peptide was 154 pg/ml (normal <100 pg/ml). The EKG documented atrial fibrillation witha high ventricular response to 140 beats per minute. Diagnosis and managementA transesophageal echocardiogram ruledout intracavitary thrombi. The left chambers were dilated with slow flow in the left atrium. The walls of the left ventricle had normal thickness with severe overall decreased contractility, and the endocardium was thickened and hyper refringent at the middle and basal thirds. The apex was also thickened with increased number of trabeculae and obliteration of the cavity. Left ventricular function was 20-25%. A a bicuspid aortic valve,with mildregurgitation was found, the right atrium was severely dilated and the right ventricle was normal in size but its walls were thickened and severely hypo kinetic.Pulmonary arterysystolic pressure was 46 mmHg, with moderate tricuspid regurgitation. No pericardial effusion was seen, and the aorta was normal with atrial septal integrity. Heart MRI revealed an altered left ventricular morphological pattern characterized by the presence of multiple trabeculae forming a reticular pattern, especially affecting the distal half of the ventricle. The interventricular septum bulged from left to right, and there was mild hypokinesia of the middle third of the interventricular septum, mainly towards itsanterior half. No areas of abnormal uptakewere found in the ventricular walls. There was no pericardial effusion and no infiltration of the myocardium or areas of scarring. The findings were compatible with non-compacted myocardium (Figure 1 & 2). An electrophysiological study ruled out the presence of an accessory hidden pathway. Due to the presence of an atypical slow atrial flutter fast degenerating into atrial fibrillation, no ablation was performed. AbstractNon-compaction cardiomyopathy (spongiform cardiomyopathy) is a primary disorder of the ventricular muscle of unknown cause, which can occur at any age, often between 20 to 40 years of age. It affects mainly men, and can be asymptomatic or present with symptoms of heart failure, arrhythmias or embolic events. Color Doppler echocardiography and nuclear magnetic resonance (cMRI) are the most commonly used diagnostic methods. No s...