Eighteen-year-old man complained of dyspnea on exercise in 2011 at his13 years old, and was diagnosed as left ventricular noncompaction (LVNC) and pulmonary hypertension (PAWP 38 mmHg, mPAP 56 mmHg, PVR 3.4 W.U.) in 2013. After his optical medical therapy, he was frequently admitted due to heart failure (HF) and non-sustained ventricular tachycardia. He received implantable cardioverter defibrillator (VVI-ICD) and was registered as a candidate of heart transplantation in Japan in 2014. In the following year, he underwent implantation of a left ventricular assist device (Heart Mate II). After his surgery, tailored medications on his pulmonary arterial hypertension were administered, and his pulmonary circulation was improved (PAWP 19 mmHg, mPAP 28 mmHg, PVR 2.4 W.U.). However, in spite of adjustment of not only medications but pump rotation speed tricuspid, regurgitation (TR) became worse and right HF was still remained. In 2016, we added an atrial lead and changed his pacing mode from VVI to AAI pacing to improve TR, LV dysfunction, and dyssynchrony of his both ventricles, and then, TR reduced and no history of his admission due to HF has been significantly observed. From our experience, physiological pacing would be effective to manage right HF in patients with LVNC and LVAD.
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