TY - JOUR T1 - Heartbeat: New insights in congenital heart disease JF - Heart JO - Heart SP - 1225 LP - 1226 DO - 10.1136/heartjnl-2017-312089 VL - 103 IS - 16 AU - Catherine M Otto Y1 - 2017/08/01 UR - http://heart.bmj.com/content/103/16/1225.abstract N2 - Overall survival of patients with transposition of the great arteries (TGA) now is about 97% at 25 years because of the success of the arterial switch procedure, starting in the 1980's. However, patients require ongoing monitoring, medical therapy and, in some cases, reoperation due to late sequelae such as aortic sinus dilation, aortic valve dysfunction, right ventricular outflow obstruction or arrhythmias.1 Pulmonary arterial hypertension (PAH) is a rare, but highly morbid, associated condition in TGA patients after an arterial switch procedure. In a series of 25 children with PAH after a TGA arterial switch repair, only 58% survived 5 years or longer despite PAH-targeted medical therapy in 19 patients, lung transplantation in four patients and the addition of a Potts shunt in two patients.2 Prognosis was equally poor in those with early onset PAH (within weeks to months) and those with late-onset PAH. (figure 1)Figure 1 Potts shunt- and transplant-free survival of children with first PAH detection within and more than 1 year after ASO. (A) Survival from ASO. Survival of 1, 3, 5 and 10 years was 100%, 74%, 59% and 40% for the 18 children with first PAH detection within 1 year after ASO and … ER -