PMC8280289_01
Family pedigree. Black arrow, proband; square, male; circle, female; black, heart septal defects; white, not affected; diagonal stripe, death; red lines, non-compaction cardiomyopathy; blue lines, sudden death; green lines, atrioventricular block.
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F1
The proband's niece (III.2 in Figure 1) had a history of surgical patch closure of ASD and perimembranous ventricular septal defect (VSD). The proband's brother (II.2 in Figure 1) had a history of surgically repaired ASD and nodal rhythm.
(A) Contrast echocardiography showing left ventricular non-compaction. (B) Hypertrabeculated left ventricle in one slice of the short axis view by coronary computed tomography angiography.
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F2
The echocardiogram showed a 40% left ventricular ejection fraction, and coronary heart disease was ruled out with coronary computed tomography angiography (CCTA) and catheterization. An echocardiogram with contrast was performed as his device was not compatible with cardiac magnetic resonance (CMR). This test showed a hypertrabeculated myocardium of the left ventricle, also present in the previous CCTA performed (Figure 2) and compatible with left ventricular non-compaction (LVNC) with a ratio of non-compacted/compacted myocardium of 2.2.
PMC8280289_02
Family pedigree. Black arrow, proband; square, male; circle, female; black, heart septal defects; white, not affected; diagonal stripe, death; red lines, non-compaction cardiomyopathy; blue lines, sudden death; green lines, atrioventricular block.
fcvm-08-691203-g0001.jpg
PMC8280289_02_fcvm-08-691203-g0001.jpg
F1
The proband's niece (III.2 in Figure 1) had a history of surgical patch closure of ASD and perimembranous ventricular septal defect (VSD). The proband's brother (II.2 in Figure 1) had a history of surgically repaired ASD and nodal rhythm.
Cine cardiac magnetic resonance images in the short axis revealing non-compaction cardiomyopathy.
fcvm-08-691203-g0003.jpg
PMC8280289_02_fcvm-08-691203-g0003.jpg
F3
The CMR confirmed a non-compacted myocardium in three segments, compatible with LVNC (Figure 3).