Left Ventricular Non-Compaction Cardiomyopathy-Still More Questions than Answers

Figure 1. Transthoracic echocardiographic examination, patient with LVNC. (A) Parasternal short axis, 2D, diastole, right with color doppler. (B) Parasternal short axis, 2D, systole, right with color doppler. (C) Apical 4-chamber view, 2D, diastole. Left—note excessive trabeculation of the left ventricle. The white arrow shows the deep trabecular recess between the trabeculation. Right—healthy patient with a normal left ventricle. (D) Zoomed view of the same patient, apex, and apical segments of the lateral wall. Note excessive trabeculation of the left ventricle. The white arrow shows the deep trabecular recess between the trabeculation. (E) Zoomed view with color doppler of the same patient, apex, and apical segments of the lateral wall. Note excessive trabeculation of the left ventricle filled with blood as seen with color doppler (arrows). (F) Contrast echocardiography (SonoVue), modified apical 4 chamber view, end-diastole, patient with LVNC. Note the deep trabecular recess between trabeculation of the left ventricle filled with contrasted blood.

Figure 2. CMR examination. 25 years old male. Basal, midventricular, and apical cine steady-state free precession images in short-axis orientation during end-diastole and end-systole. Note differentiation between non-compacted from compacted myocardium.

Figure 3. CMR examination: (A) Three-chamber view, diastole; (B) three-chamber view, systole; (C) four-chamber view, diastole; (D) four-chamber view, systole. Cine steady-state free precession images at end-diastole and end-systole. Note an increased number of trabeculations along the LV lateral wall and LV apex (arrows). (E) Long-axis right ventricular view, cine steady-state free precession image of the right ventricle showing prominent hypertrabeculation (arrowhead).

Figure 4. CMR examination: Thrombus in the apex of the left ventricle in a patient with LVNC (A,B) (arrow). (C) Short-axis view, late-enhanced sequence (PSIR). Extensive intramyocardial LGE in the septum (arrowheads).

Figure 5. CMR examination, short-axis cine bSSFP image of the mid-cavitary LV. Prominent hypertrabeculation in a 20-year-old male with a history of ADPKD and LVNC phenotype. Measurement of the whole myocardial mass: (A) Determination of epicardial contours and endocardial contours at the NC myocardium border for measurement of whole myocardial mass. (B) Determination of endocardial contours at the border of the NC myocardium and non-trabeculated cavity for measurement of non-compacted myocardium mass. bSSFP—balanced steady-state free precession; LV— left ventricle. ADPKD—autosomal dominant polycystic kidney disease.

Figure 6. An Electrocardiogram of the patient from Figure 1C–E shows regular sinus rhythm 75/min. PQ 190 ms, QRS 100 ms, QT 460 ms, QTc 514 ms. Note notching of the R-wave of the QRS in lead I, II, III, aVF, and V4 (arrow) as well as prolongation of QTc.

Figure 7. A pathway for risk stratification and follow-up of patients with hypertrabeculation phenotype.