Autosomal dominant polycystic kidney disease (ADPKD) can determine end stage renal disease (ESRD) which require haemodialysis. Surgical creation of arteriovenous fistula (AVF) has been associated with development of left ventricular (LV) dilatation and high output heart failure (HF) and right ventricle (RV) dysfunction at long term follow-up. Databases show an association between ADPKD and idiopathic dilated cardiomyopathy, suggesting that PKD mutations contribute to development of heart failure.
36 ys old female patient affected by ADPKD who required in 2018 bilateral nephrectomy, due to a relapse of severe urinary tract bleeding, and then put in thrice-weekly hemodialysis. Before the procedure in 2016 an echocardiogram showed normal biventricular function, with valvular defects. An AVF, between left humeral artery and the perforator vein of the elbow, was employed as vascular access. 6 months later she was referred to our center for atypical chest pain and palpitations. A transthoracic echocardiogram showed a diffuse hypokinesia with severe left ventricular dysfunction and dilation (EF 25%, VTDi 90ml/mq), a cardiac output of 6,5 l/min, moderate mitral insufficiency, hypokinetic RV, pulmonary artery systolic pressure (PASP) of 56mmHg, severe biatrial dilatation. No signs of congestion. She was admitted to our service. The coronary angiogram was normal, ruling out an underlying ischemic cardiomyopathy. Therapy with b-blocker and ACE-I was started. Doppler ultrasonography of the AVF was performed and showed an high output fistula. Two weeks later at Hospital discharge with medical therapy only, EF improved to 43%. Five months later, the AVF was ligated, after the creation of another more distal one. Echocardiographic re-evaluation one month after the intervention showed a normalization of left ventricular function and volumes ( EF 55%, VDTi 54ml/mq)and of PASP. In 2020 she underwent renaltransplant without complications. The patient is nowadays stable only on beta-blocker for some PVCs, with ongoing annual follow-up.
Even if dilated cardiomyopathy and HF in patients affected by APDKD can be caused by genetic predisposition, in our case the main factor was a high output AVF, as showed by complete reversion of left ventricular dysfunction after its ligation.