Non-compact cardiomyopathy or ominous trabeculation
█ Case report
Kurczina Anita1, Kracskó Bertalan1, Balogh László1, Rácz Ágnes1, Vágó Hajnalka2, Clemens Marcell1, Csanádi Zoltán1, Borbély Attila1
1Debreceni Egyetem, Klinikai Központ, Kardiológiai és Szívsebészeti Klinika, Debrecen
2Semmelweis Egyetem, Városmajori Szív- és Érgyógyászati Klinika, Budapest
The 32-year-old male patient was hospitalized because of severe (NYHA class III-IV) heart failure (HF) symptoms in October 2018. Transthoracic echocardiography (TTE) showed extremely enlarged heart chambers, largely depressed left ventricular (LV) systolic function (ejection fraction, EF 18%) and severe functional mitral regurgitation. Cardiac MRI confirmed the suspected non-compact cardiomyopathy (NCCMP). Vitamin K antagonist was started. Next to the slow up-titration of the evidence-based HF therapy, a continuous improvement in the patient’s functional status could be observed. The patient was discharged with a wearable cardioverter defibrillator (WCD). Next to the optimized drug therapy echocardiography revealed a significant improvement in the EF (30%) during follow-up, however, LV dimensions remained unchanged. Based on the results, placement of an implantable cardioverter defibrillator (ICD) for primary prevention was performed. Heart transplantation will be considered in case of worsening in the functional status or by occurrence of therapy refractory ventricular arrhythmias.
The morphological feature of NCCMP is a spongy ventricular myocardium with abnormal trabeculation (compact and non-compact myocardial layers) usually most evident in the left ventricular apex. However, etiology of NCCMP is still uncertain: it remains unclear, whether it is a separate entity or a phenotypic appearance of other cardiac diseases. Its clinical presentation may occur with severe HF, sudden cardiac death or thromboembolic events. The diagnosis of NCCMP is based on cardiac imaging (TTE/TEE/MR).