Clinical findings in right ventricular noncompaction in hypoplastic left heart syndrome

摘要: Background Noncompaction is a poorly understood form of cardiomyopathy that typically affects the left ventricle and may be associated with congenital heart disease. Right ventricular noncompaction (RVNC) occur when affected but rarely seen in isolation. RVNC have clinical significance affecting surgical long-term outcomes. We describe diagnosis course three patients at our institution. Methods We performed retrospective review diagnosed over 12-month period institution reviewed their imaging course. Results Three were identified. All had by echocardiography (echo) made on postnatal which degree trabeculation, noncompaction-to-compaction ratio myocardium. Patient A was neonate hypoplastic syndrome (HLHS) who underwent Norwood operation Sano modification. Her postoperative notable for low-normal RV function. She returned pericardial effusion warranting immediate pericardiocentesis. continued to effusions, medically managed. subsequently found an apical pseudoaneurysm, required resection. B HLHS function echo. medical management effusion. C also His elevated serum brain natriuretic peptide, treated digoxin. Conclusion RVNC rare limited known impact. One these very complication after pericardiocentesis (pseudoaneurysm) been related RVNC. Our understanding this disease process requires additional investigation, emphasizes importance appropriate allow timely follow-up counseling unique population.