Cardiac resynchronization therapy in a patient with left ventricular non-compaction cardiomyopathy: A case report

Abstract

Introduction: Left ventricular non-compaction cardiomyopathy (LVNC) is a rare and specific type of congenital cardiomyopathy with an incidence rate between 0.05% and 0.24%. Dilated cardiomyopathy (DCM) and ischemic cardiomyopathy have poor prognosis. Cardiac resynchronization therapy (CRT) improves cardiac function and long-term prognosis in patients with heart failure with left bundle branch block, but its efficacy on LVNC is uncertain. Case Report: This paper reported a case of a 57-year-old male with LVNC who was hospitalized for complaining of palpitations and shortness of breath on exertion and then diagnosed as heart failure. Electrocardiogram revealed a sinus rhythm with a QRS duration of 164 ms, as well as left bundle branch block morphology. Chest X-ray showed marked cardiomegaly and mild pulmonary congestion, and ultrasound echocardiography showed abnormal echo of left ventricular myocardium. The myocardium was cavernous sinus-like, forming sinusoidal cavities in varying sizes, communicating with left ventricular cavity, with the ratio of non-compacted to compacted myocardium of 3:1. The patient featured a large left ventricular end-diastolic diameter of 91 mm, and the function was severely impaired with an ejection fraction (EF) of 31%. After diuretic treatment, improvement of myocardial remodeling, heart strengthening, vasodilation, etc., he recovered gradually and was discharged 20 days later. However, the patient was admitted to hospital again due to shortness of breath within one month without obvious inducement. Considering the poor effect of drug therapy, we recommended that the patient undergo cardiac resynchronization therapy, he recovered and was discharged seven days after surgery. During the 6-month follow-up period, his left ventricle (LV) function and clinical symptoms showed significant and sustained improvement. B-type natriuretic peptide (BNP) levels decreased from 4836.79 to 1253.18 pg/mL. QRS duration (164 ms) on ECG significantly decreased after CRT. Conclusion: We reported the management of a rare case of congenital LVNC with recurrent heart failure and frequent premature ventricular contractions. Patients with LVNC have clear indications and poor pharmacological response. Early cardiac resynchronization treatment might benefit them.