Six-Year Outcome after Valve Replacement and Resynchronization Therapy in TGA Patient

Patients with complete transposition of the great arteries(TGA)treated by the Senning procedure have a higher risk of developing heart failure due to:a)additional work load of the systemic(morphologic right)ventricle(sRV),b)arrhythmias,mainly caused by surgical implications at the atria as well as c)worsening of systemic tricuspid regurgitation.We present a unique case of a female patient who developed all these complications,who was successfully treated and was able to carry out a twin pregnancy.This breakthrough approach was based on:1.detecting reversibility potential of myocardial systolic dysfunction in a severe valvular lesion combined with continuous systemic afterload settings and permanent tachyarrhythmia,and 2.prevention of subsequently iatrogenic worsening of systemic ventricular function due to permanent pacing.Surgical replacement of systemic tricuspid valve(sTV)and cardiac resynchronization device(CRT)implantation after nodal ablation resulted in recovering of the systolic function and a positive remodeling of the sRV.The reversal of a further decline in systolic function was achieved by permanent arrhythmia control,synchronous pacing with epicardial leads of CRT,sTV replacement as well as echocardiographic monitoring during pregnancy to determine the right time for delivery.Two years after delivery,the patient remains in NYHA Class I.