Left ventricular noncompaction with pulmonary capillary hemangiomatosis-like lesions: case report

Abstract Left ventricular noncompaction (LVNC) is a cardiomyopathy characterized by prominent left ventricular trabeculae and deep intertrabecular recesses. Pulmonary capillary hemangiomatosis (PCH) is a rare disease that causes uncontrollable proliferation of pulmonary capillaries. We experienced a 52-year-old man who was diagnosed with LVNC about 8 years previously who subsequently died of heart failure. The major autopsy findings were enlargement of the heart with prominent trabeculations and deep intertrabecular recesses in the apical and middle regions of the left ventricular wall. The mean ratio of noncompacted to compacted layers was 2.4. In the lung, thickened alveolar walls with numerous pulmonary capillaries were evident, findings very similar to PCH. PCH-like lesions and LVNC may have coexisted coincidentally, and both, or either of them, may have contributed to the development of his pulmonary hypertension.