SESSION TITLE: Pulmonary Vascular Disease Cases I
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Monday, October 26, 2015 at 11:00 AM - 12:00 PM
INTRODUCTION: Pulmonary hypertension (PHTN) secondary to high cardiac output (CO) from AV fistula is an underappreciated but well described condition in patients with end-stage renal disease (WHO Group 5) undergoing hemodialysis (HD). It is usually difficult to diagnose as an isolated, major contributing factor to elevated pulmonary pressures.
CASE PRESENTATION: The patient is a 71 year old male, with NYHA FC III dyspnea, diabetes with ESRD on HD via AV fistula (2007), hypertension, PVD, paralyzed right hemidiaphragm, chronic anemia, 28 pack-year smoker, referred for PHTN . Consecutive TTEs showed progression of PASPs from baseline of 40 mmHg (2006, pre-fistula). TTE (2013) estimated PASP at 90 mm Hg, demonstrated severe RV dilatation, moderate RA enlargement, ventricular interdependence with normal LVEF and grade I-II diastolic dysfunction. RHC (2/2014) performed after HD showed RA15/13(10), RV 90/0(16), PA 96/31(58), PCWP 10/13(10), CO/CI (TD) 7.0/3.6, CO/CI (Fick) 4.86/2.5, PVR 7.4 wood units, TPG 48 . Additional evaluation revealed low probability V/Q scan, PFTs with mild restriction, moderate obstruction, DLCO 84%, normal LFT and TSH. To determine if high CO could be secondary to AV fistula rather than anemia alone, RHC was repeated pre and post occlusion of AV fistula. There was 22.5% drop in CO after occlusion of AV fistula for 5 minutes. To ameliorate high CO from fistula, surgical ligation of fistula was performed. Dyspnea improved for several months then recurred and symptoms progressed. Latest RHC (10/2014) demonstrated marked PHTN despite closure of AV fistula suggesting progression of intrinsic pulmonary vascular disease. A trial of PDE-5 inhibitor and ERA was initiated.
DISCUSSION: High CO state from AV fistula is a potential factor leading to PHTN in ESRD besides anemia, fluid overload, diastolic dysfunction (HFpEF), secondary hyperparathyroidism and increased Endothelin-1 levels. PHTN is an independent risk factor for mortality in ESRD. Measures to decrease fistula related PHTN including shunt reduction, fistula ligation, peritoneal dialysis, or renal transplantation could be considered.
CONCLUSIONS: A hyperdynamic circulatory state from AV fistula in ESRD should be considered in the differential of PHTN. Improvement in CO or pulmonary pressures after obliterating or bypassing AV fistula confirms this hypothesis. Early screening of PHTN by echocardiography may enable earlier intervention.
Reference #1: Yigla M, Nakhoul F, Sabag A etal: Pulmonary Hypertension in Patients with ESRD (CHEST 2003)
DISCLOSURE: The following authors have nothing to disclose: Abhinav Gupta, Mary Jo Farmer, Harrison Farber
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