Pulmonary Vascular Disease: Student/Resident Case Report Poster - Pulmonary Vascular Disease II |

A Case of Profound Hemodynamic Response to Combination of Inhaled Nitric Oxide and Riociguat in Severe PAH FREE TO VIEW

Srikanth Yandrapalli, MD; Ataul Qureshi, MD; Zeeshan Solangi, MD; Pratik Mondal, MD; Venkat Lakshmi Kishan Vuddanda, MD; Chhaya Aggarwal, MD; Gregg Lanier, MD
Author and Funding Information

Department of Medicine, New York Medical College at Westchester Medical Center, Valhalla, NY

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

Chest. 2016;150(4_S):1217A. doi:10.1016/j.chest.2016.08.1326
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SESSION TITLE: Student/Resident Case Report Poster - Pulmonary Vascular Disease II

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Riociguat is approved for the treatment of adults with chronic thromboembolic pulmonary hypertension (CTEPH) and pulmonary arterial hypertension (PAH). It is marketed with a warning against co-administration with nitric oxide donors in any form due to a risk of hypotension, although the supporting data is limited.

CASE PRESENTATION: A 33-year-old female with idiopathic PAH on subcutaneous treprostinil 50 ng/kg/min and macitentan 10 mg daily presented with worsening dyspnea and syncope. Suspecting absorption failure, treprostinil was changed to intravenous route. Right heart catheterization showed right atrial pressure of 2 mmHg, severely elevated pulmonary artery (PA) pressure of 97/38 mmHg, mean PA pressure (mPAP) of 59 mmHg, pulmonary capillary wedge pressure of 4 mmHg, Fick cardiac output of 3.83 L/min, and pulmonary vascular resistance(PVR) of 14.36 Wood units. During the procedure she responded favorably to 40 ppm inhaled nitric oxide (iNO), with a decrease in mPAP to 44 mmHg and PVR to 8.79 Wood units. In the setting of clinical right heart failure, iNO was continued at 40 ppm. PDE5 inhibitors could not be used as she had a prior allergic reaction to tadalafil. Riociguat 0.5mg was started and iNO stopped simultaneously. However, mPAP increased to 65 mmHg and iNO was restarted. Over the next 4 days, riociguat was titrated up to 2.5mg every 8 hours, iNO was gradually weaned off based on mPAP, and treprostinil increased to 60 ng/kg/min. During this period, hemodynamics improved considerably with the lowest recorded mPAP of 35 mmHg. By day 4, improvement was noted in mPAP to 43 mmHg, Fick cardiac output to 6.46 L/min, and PVR to 9 Wood units. Mean arterial pressure remained stable around her baseline of 70 mmHg without any hypotensive episodes. Her symptoms improved significantly and she was weaned off nasal oxygen.

DISCUSSION: Riociguat, iNO, and PDE5 inhibitors act via potentiation of the soluble guanylate cyclase pathway. Co-administration of these drugs is contraindicated due to a risk of hypotension, although the supporting data is limited. The safe and additive effect of iNO and sildenafil on postoperative hemodynamics in cardiac surgery patients with severe pulmonary hypertension was previously described. In this case of severe PAH, short term iNO was safely and successfully used in combination with riociguat with favorable synergistic effects on PA pressure and PVR without systemic hypotension.

CONCLUSIONS: To our best knowledge, this is the first reported case of safe co-administration of riociguat with iNO. Utility of riociguat added to short term iNO therapy in decompensated patients with severe PAH and in post-operative pulmonary thromboendarterectomy patients with CTEPH needs to be validated in larger trials.

Reference #1: Matamis D et al. Inhaled NO and sildenafil combination in cardiac surgery patients with out-of-proportion pulmonary hypertension: acute effects on postoperative gas exchange and hemodynamics. Circ Heart Fail. 2012;5(1):47-53

DISCLOSURE: Gregg Lanier: Consultant fee, speaker bureau, advisory committee, etc.: speakers bureau for Gilead, Actelion, United therapeutics, and Bayer. The following authors have nothing to disclose: Srikanth Yandrapalli, Ataul Qureshi, Zeeshan Solangi, Pratik Mondal, Venkat Lakshmi Kishan Vuddanda, Chhaya Aggarwal

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