Cardiac magnetic resonance imaging
Cardiac magnetic resonance imaging was performed on 25 patients. CMR measured mean LVEF was 35.3±10.5, mean LVEDd was 58.8±8.0 mm, and mean LVESd was 50.4 ±10.4 mm. Seven (28%) patients LVEDd was in the normal range. Despite the decreased LVEF and enlarged left ventricle, the mean volumes, cardiac output, and cardiac index were preserved.
Three patients exhibited late gadolinium enhancement (LGE) patterns. Sub-endocardial and mid-wall uptake pattern types were detected, one patient with subendocardial LGE experienced sudden cardiac death in a HF medication titration course, and the second patient experienced ventricular tachycardia with appropriate ICD shock. The third patient with mid-wall LGE pattern type needed heart transplantation surgery.
All patients demonstrated trabeculation at least in one left ventricular segment, and trabeculations were mostly detected in lateral and inferior segments. The mean non-compacted to compacted (NC/C) ratio was 2.73±0.88, and 18 (75%) patients met the Petersen non-compaction cardiomyopathy criteria. Patients were also compared regarding NC/C ratio; patients with NC/C ratio lower than 2.3 had lower LVEDV(i) and LVESV(i) (124.9±35.4, 86.4±7.5, p=0.003; 86.8±34.6, 52.6±7.6, p=0.006), respectively.
Seven patients underwent a second CMR to evaluate the left ventricle volumes and cardiac function. Four patients with NC/C ratio over 2.3 had a non-compaction cardiomyopathy phenotype at the second CMR examination. In all seven patients who underwent CMR, LVEF increased; the mean change was 13.85±12.5 (p=0.0035) (Table 3).
Survival data were available for all patients. The median follow-up time was 2129 (IQR [911-2634]) days. Ten (27%) patients experienced primary end-point; five (13.5%) patients experienced death, two (5.4%) patients underwent left ventricular assisted device implantation, and three (8.1%) patients experienced heart transplantation surgery. The primary end-point occurrence median time was 786 (IQR [167-1498]) days-(Figure 2).
The primary end-point free one-year survival was 88.9% (event rate 11.1%), and five-year survival was 75.7% (event rate 24.3%) (Figure 3).