Noncompaction Phenotype, But What is Cause and is the Diagnosis Correct?

Özet

Objectives: The study aims to reveal different groups of left ventricular (LV) noncompaction (LVNC) by evaluating patients who have undergone cardiac magnetic resonance imaging for the confirmation of diagnosis and also to examine detailed features of cardiac magnetic resonance imaging. Noncompaction cardiomyopathy is a rare myocardial pathology characterized by increased trabeculation. Diagnosis requires echocardiography-and cardiac magnetic resonance-based quantitative indexes measuring ratios of noncompacted and compacted layers of the left ventricle. Although LVNC can be seen as an isolated entity, it may also be a feature of several cardiac and noncardiac disorders. Materials and Methods: Our study is a retrospective cohort study. Cardiac magnetic resonance imaging performed between the years of 2006 and 2018 in our radiology department were analyzed with the keyword “noncompaction”. A total of 64 imaging were examined regarding diagnostic criteria, hypertrabeculated areas, LV ejection fraction values and comorbidities. Mortality and cardiac operation outcomes were also investigated. Results: In our study population, 38 (%59) of 64 patients were men. The mean age was 41 years in an age range of 18- 73 years. Hypertrabeculations were prominently seen in the left ventricle lateral wall followed by the apex and inferior wall. Noncompacted and compacted myocardial layers thickness ratio was measured and averaged around 2.9 (2.2 - 4) at the site of maximal wall thickness. Forty-eight reports had also data about LV ejection fraction values and the mean value was 35 (17-69). Regarding comorbidity, coronary artery disease, primary valve disease, connective tissue disease, duchenne muscular dystrophy, neurofibromatosis type 1, peripartum cardiomyopathy, and congenital heart anomalies were observed. Totally 38 patients were diagnosed with noncompaction cardiomyopathy. Also, biventricular noncompaction was observed in three patients and isolated right ventricle noncompaction was observed in one patient.Regarding cardiac operation endpoints, it was observed that cardiac resynchronization therapy-defibrillator implantation was performed in six patients, LV assist device implantation in five patients, and heart transplantation in three patients. Four patients were exitus concerning mortality. Conclusion: The LVNC remains subject to controversy due to lack of consensus on its etiology, pathophysiology, diagnosis and management. There is a requirement for a consensus about diagnostic imaging modalities, and accompanying conditions about noncompaction.