Reduced regional systolic function is not confined to the noncompacted segments in noncompaction cardiomyopathy

Abstract

Background Isolated ventricular noncompaction (IVNC) is a relatively rare genetic primary cardiomyopathy. The aim of the present study was to investigate with regional real-time three-dimensional echocardiographic analysis whether there is a difference between the contribution of noncompacted and compacted left ventricular (LV) segments to global LV dysfunction in patients with IVNC. Methods The study comprised 289 segments of 17 patients with stringent diagnostic criteria for IVNC. Their results were compared to 153 segments of 9 control subjects. The systolic performance of compacted and noncompacted LV segments was assessed using the wall motion score during 2D echocardiography. The 3D images were acquired with a RT3DE system with X4 matrix-array transducer and were used for the regional volume measurements. Results Wall motion score index was markedly abnormal in the compacted LV segments of IVNC patients but significantly less abnormal compared to the noncompacted segments (2.21 ± 0.63 vs. 2.01 ± 0.74, p < 0.05). No relationship was found between the number of noncompacted segments per patient and LV ejection fraction or end-diastolic volume. In the IVNC patients, noncompacted and compacted LV segments had comparable increased 3D regional volumes and reduced systolic function. Conclusions These results suggest that systolic LV dysfunction observed in IVNC is not confined to noncompacted LV segments.