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).