Pulmonary Vascular Disease: Pulmonary Vascular Disease |

Cardio-Renal-Like-Syndrome in Pulmonary Arterial Hypertension While on Epoprostenol FREE TO VIEW

Judette Polynice, MD; Robert Walter, MD; Robert Holladay, MD
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University Health Shreveport, Shreveport, LA

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;149(4_S):A498. doi:10.1016/j.chest.2016.02.520
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SESSION TITLE: Pulmonary Vascular Disease

SESSION TYPE: Case Report Poster

PRESENTED ON: Sunday, April 17, 2016 at 11:45 AM - 12:45 PM

INTRODUCTION: Prostacyclin therapy remains the drug of choice for progressive pulmonary arterial hypertension (PAH) with New York Heart Association class IV patients. Persons presenting with worsening kidney function are often viewed as experiencing disease progression. This is an unusual case of a patient who developed worsening renal failure with up-titration of intravenous (IV) epoprostenol despite significant improvement of the hemodynamic markers and his functional class.

CASE PRESENTATION: A 56-year-old African American male diagnosed with iPAH who declined after several years on oral phosphodiesterase-5-inhibitor despite adding an endothelin-receptor-antagonist. Invasive hemodynamics demonstrated a mean pulmonary arterial pressure (mPAP) of 51, pulmonary vascular resistance (PVR) of 7 Woods units, and cardiac index (CI) 2. The renal panel was remarkable for a creatinine of 2.2 mg/dl and B natriuretic peptide (BNP) of 8,000. He had hemodynamic evidence of right ventricular failure, he was initiated on IV epoprostenol, titrated in response to his clinical status over several weeks to 22 ng/kg/min. His functional class and 6-minute-walk-test have improved, his BNP and creatinine level continued to rise to 90,000 and 3.9 respectively. He experienced a 20-pound weight gain. Invasive hemodynamics showed improvements, mPAP of 40, PVR of 4 Woods units, and CI of 5. He had renal failure with a sodium-avid state the fractional excretion of sodium (FENA) was < 1%. IV epropostenol was slowly tapered to 10ng/kg/min, CI fell (table1), renal function improved.

DISCUSSION: Pre-renal type of acute renal failure (ARF) is usually seen in iPAH NYHA class VI patients with Cor-Pulmonale. IV epoprostenol, when administered, improves hemodynamics such as CI, PVR renal perfusion and thereby increase GFR and decrease creatinine. For reasons that remain obscure our patient experienced ARF on optimal therapy. One hypothesis could be the vasodilatory effects of IV epoprostenol created a shunt, similar to hepatorenal syndrome (HRS). In this setting, arterial hypotension is the key factor, which even if it does not reach shock values, causes simultaneous renal vasoconstriction and renal hypoperfusion with decreased glomerular filtration. “Thus, cardiac output is the relevant factor, it may be low, normal or high, but is relatively insufficient to prevent a severe reduction of effective circulating volume due to the splanchnic arterial vasodilatation in patients with HRS”[2].

CONCLUSIONS: Pre-renal ARF in PAH tends to represent a marker undertreated or untreated disease. A shunt similar to HRS has not been described in PAH patient who are optimized with IV epoprostenol.

Reference #1: Ayoola O. Akinbamowo, MD, Daniel J. Salzberg, MD, Matthew R. Weir, MD Renal Consequences of Prostaglandin Inhibition in Heart Failure J Clin Invest. 2004 July 1; 114 (1): 5-14.

Reference #2: Marcela Kopacova, MD, PhD, Hepatorenal syndrome World J Gastroenterol 2012;18(36):4978-4984.

DISCLOSURE: The following authors have nothing to disclose: Judette Polynice, Robert Walter, Robert Holladay

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