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584 been mild. The patient’s intellectual development is normal. At the time of DCM diagnosis, the patient was metabolically stable and did not require diet modification. Because of the predisposition to hypotension, only angiotensin-converting enzyme inhibitor was administered (ramipril, 1 × 1.25 mg/d). A month later, transthoracic echocardiography revealed left ventricular ejection fraction of 35%, but the patient remained asymptomatic. Although the patient did not meet standard criteria for implantation of an implantable cardioverter -defibrillator (ICD), we considered information provided in case reports on sudden cardiac death in patients with PA and prolonged QTc, and decided to implant the ICD as a primary prevention of sudden cardiac death. Because of the progressing cardiomyopathy and reports describing the reversal of cardiac abnormalities after liver transplantation in patients with PA,3 the case was evaluated by a multidisciplinary team and the patient was referred for liver transplantation. Cardiomyopathy (mainly DCM) affects 9% to 23% of patients with PA, and prolonged QTc interval is observed in 70%. These conditions are associated with increased mortality and develop regardless of the metabolic control of the disease.1 The pathomechanism of cardiac involvement in PA remains unclear. It is probably caused Propionic acidemia (PA) is a rare (1 in 100 000– –150 000 persons) inborn error of the metabolism classified as an organic acidemia, inherited in an autosomal recessive pattern and caused by a deficiency of propionyl -coenzyme A carboxylase (PCC). Inhibited or reduced activity of PCC results in the accumulation of propionyl-coenzyme A and its metabolites: methylcitrate, 3-hydroxypropionate, triglyglycine, and propionyl glycine, leading to hypoglycemia, hyperammonemia, and hyperglycinemia.1 Clinical presentation of PA includes life -threatening episodes of metabolic decompensation, but also neurological, hematological, gastroenterological, and cardiac manifestations with cardiomyopathies and arrhythmias.2 Treatment of PA includes metabolic compensation, diet modification, carnitine supplementation, and enteral antibiotic therapy to reduce the burden of propionic acid–producing gut bacteria.1 We report a case of a 20 -year -old man diagnosed with PA at the age of 7 days. Since the age of 16 years, he had experienced asymptomatic prolonged corrected QT (QTc, 490–504 ms), infrequent ventricular extrasystoles, and episodes of junctional rhythm lasting a few seconds (Figure 1). At the age of 20 years, he was diagnosed with dilated cardiomyopathy (DCM) with left ventricular ejection fraction of 40%. Since early infancy, the patient was treated by diet modification. To date, the course of the disease has Correspondence to: Małgorzata Peregud­ ­Pogorzelska, MD, PhD, Department of Cardiology, Pomeranian Medical university, al. Powstańców Wielkopolskich 72, 70­111 Szczecin, Poland, phone: +48 91 466 13 77, email: m1peregud@gmail.com Received: March 4, 2019. Revision accepted: March 25, 2019. Published online: May 24, 2019. Kardiol Pol. 2019; 77 (5): 584­588 doi:10.33963/KP.14852 Copyright by Polskie Towarzystwo Kardiologiczne, Warszawa 2019 C L I N I C A L V I G N E T T E

Rare indication for cardioverter‑defibrillator implantation: propionic acidemia complicated by dilated cardiomyopathy and prolonged QT interval