Introduction: Left ventricle noncompactation represents a cardiac disease that is not fully elucidated. It is a rare, primary myocardial disease, genetically determined, that is characterised by a thin, compacted epicardial layer and an extensive non-compacted endocardial layer, with prominent trabeculation and deep recesses that communicate with the left ventricular cavity , probably due to an arrest of compaction during intrauterine life.
Case presentation: A 52 years old patient presents at the emergency department for rest dyspnea with orthopnea. He has complained about extreme fatigue and dyspnea at progressive efforts until minimum efforts for the last 2 months. From the family history we found out that the patient’s mother died at age 50 by cardio-vascular cause. We find ourselves in front of a patient who has been known with cardiovascular disease since 2012 when he presented with an episode of cardiogenic pulmonary edema thought to be caused by acute coronary syndrome (without medical papers). Afterwards, the patient was evaluated again in 2016 and the echocardiography result showed dilated heart cavities with severe systolic dysfunction (LVEF 15%), severe mitral regurgitation and structural changes that suspected left ventricle noncompactation.
Results: The cardiac MRI revealed severe left ventricle systolic dysfunction (LVEF 18%) with dilated cardiac cavities and questioned left ventricle noncompactation as primary cause. Coronary angiography – no obstructive disease. In 2016, a CRT-D is implanted with favorable clinical outcome until 2017 when the patient presents multiple episodes of acute decompensated heart failure that led to numerous hospitalizations.The pati-ent is admitted in our unit with NYHA IV heart failu-re. At echocardiography the severe left ventricle systolic dysfunction is maintained (LVEF 15%, dP/dT 900 mmHg/s, CI=1 L/min/m²), TAPSE 20 mm, LVTDVml/m², LVTSV 241 ml/m², severe mitral regurgitation with VC 18 mm, PASP 50 mmHg. A couple of days of positive inotrope therapy is needed afterwards the digitalis therapy is restarted. The patient has shortness of breath at minimal efforts with multiple episodes of paroxysmal dyspnea. Due to the fact that the symptoms persist under optimal tolerated medical treatment and resynchronization therapy the only future therapeutic options remain: MitraClip, LVAD, heart transplantation.
Conclusions: In left ventricle noncompatation cardiomyopathy, the mortality resembles the one of dilated cardiomyopathy, this data confirms the fact that the severe left ventricle systolic dysfunction remains the main cause of increase morbidity and mortality. The conventional therapy for advanced medically and re-synchronization refractory heart failure is heart trans-plantation but the ventricle assisted devices can represent optimal solutions for increase survival and a better quality of life.