We present 3 patients who underwent HT for ARVC, which rarely occurs in children.
2 girls (aged 13-15 years) underwent HT with a history of ventricular tachycardia requiring cardiopulmonary resuscitation and symptomatic biventricular pump failure within 6 months. Echocardiography revealed that the RVDD, LVDD and LVEF was 42mm, 42mm and 15% in one girl, 30mm, 60mm and 15% in another girl. One of them had received ECMO for 14 days and underwent HT after 10 days weaning off ECMO. A 13-year-old boy was admitted to our hospital due to chest tightness. After anti-heart failure therapy, echocardiography revealed that the enlarged RVDD reduced from 35 to 28mm and severe tricuspid regurgitation (TR) (IV/IV) improved to moderate TR (III/IV). No palpitation or arrhythmias evidence with 24-hour Holter recordings presented at that time. In 7 days after tricuspid valvuloplasty (TVP), echocardiography showed RVDD 17mm and mild TR (I/IV). However, 3 years later he developed gradually severely RVF complicated with definite protein-losing enteropathy (PLE) and required frequent admissions for intravenous diuretic therapy. Echocardiography revealed RVDD 40mm and severe TR (IV/IV). After 46 months of TVP, the boy underwent HT and had a difficult post-transplant course with RVF. ECMO weaned off on post-operative Day 7 and PLE was resolved until post-operative Day 20. All 3 patients are alive now for 12, 36, 50 months.
These children with end-stage ARVC had relative short nature history. So it is important for them to be diagnosed and referred to HT assessment as early as possible.