Late Development of Pulmonary Arterial Hypertension After Arterial Switch for Transposition of the Great Arteries

Persistent Pulmonary Hypertension in a Neonate With Transposition of Great Arteries and Intact Ventricular Septum

“…The combination of TGA/IVS with PPH early in life is very rare and can be fatal. [1][2][3][4][5][8][9][10][11][12][13] The hallmark of the disease is persistent hypoxemia, despite unrestrictive interatrial communication and ductal patency requiring maximum mechanical and circulatory support and pulmonary vasodilators to improve intracardiac mixing.…”

“…There are some reports of progressive worsening of pulmonary vascular disease months or years after corrective surgery. 5,9 This raises the question of whether these patients may have reduced mortality and morbidity risk on long-term pulmonary vasodilators with close follow-up. We chose not to treat this patient with pulmonary vasodilator after discontinuation of iNO and to follow closely with serial echocardiogram and clinic visits.…”

Late-onset pulmonary arterial hypertension after successful early arterial switch surgery for simple transposition of the great arteries

“…(3,10) Carroll WD, et al reported a favourable response to monotherapy with sildenafil in a 7 year-old boy lasting up to at least 9 months. (11) However, Chan, et al reported little, to no, response to combination therapy of sildenafil and bosentan in a 16-year-old male with systemic pulmonary pressures.…”

“…To the best of our knowledge 4 such cases have been published. (3,(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20) …”