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

Successful Pulmonary Vasodilator Therapy in a Patient With Group 5 Pulmonary Hypertension in Association With Neurofibromatosis FREE TO VIEW

J Saadi Imam, MD; Scott Helgeson, MD; Mohamad Zetir, MD; Charles Burger, MD
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Mayo Clinic Florida, Jacksonville, FL

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

Chest. 2016;150(4_S):1227A. doi:10.1016/j.chest.2016.08.1336
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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: Pulmonary hypertension (PH) in association with neurofibromatosis is relegated to group 5 diagnostic group by the 5th World Symposium on PH. Currently, there are no FDA-approved treatments for group 5 PH. Nonetheless, the PH is due to elevated pulmonary vascular resistance (PVR) and may respond to pulmonary vasodilator therapy.

CASE PRESENTATION: A 45-year-old woman with neurofibromatosis presented with a 4 year history of worsening exertional dyspnea equivalent to WHO functional class (FC) IV. Chest CT prior to evaluation ruled out pulmonary embolism but showed diffuse bilateral ground-glass infiltrates and a prominent main pulmonary artery (PA) measuring 4cm. She had been given the presumptive diagnosis of interstitial pulmonary fibrosis but had not improved on steroids. Arterial blood gas confirmed hypoxemia on room air with PaO2 56mmHg. Pulmonary function testing showed mild restriction with total lung capacity 74% and diffusing capacity 52% predicted. Repeat chest CT revealed low lung volumes, mild centrilobular emphysema, and marked enlargement of the main PA (4.4cm) but no diffuse interstitial lung disease. Echocardiography showed moderate right atrial and severe right ventricular (RV) enlargement, and severe decrease in RV systolic function. Right heart catheterization (RHC) demonstrated right atrial pressure (RAP) 20mmHg, mean PA pressure (MPAP) 54mmHg, pulmonary capillary wedge pressure 9mmHg and cardiac output (CO) 1.6L/min. The calculated PVR was greater than 28 Woods units. There was no step-up in O2 saturation or significant response to inhaled nitric oxide. Due to financial limitations, patient was reluctant to initiate infusion prostanoid therapy; therefore, sildenafil and bosentan were started. Six month follow-up showed persistently elevated brain natriuretic peptide level and reduced 6 minute walk distance with severe RV enlargement and dysfunction by echocardiography. The patient was admitted for RHC and initiation of continuous epoprostenol infusion, which offered significant improvement in MPAP, CO and PVR on 10ng/kg/min epoprostenol: MPAP decreased from 75 to 49mmHg, CO increased from 5 to 9L/min and RAP decreased from 24 to 18mmHg. Upon follow-up, patient was switched from sildenafil to tadalafil. When she developed pancytopenia from myelodysplastic syndrome, bosentan was discontinued. Currently, she is FC II on epoprostenol 50ng/kg/min and tadalafil 40mg daily.

DISCUSSION: PH symptoms relate to progressive worsening of RV function. There are currently no approved PH medications for patients with group 5 disease. However, patients meeting group 5 criteria with associated conditions such as neurofibromatosis may benefit from pulmonary vasodilator therapy as illustrated here.

CONCLUSIONS: Further research and discussion is needed to assess the appropriate use of group 1 PH medications in patients with group 5 disease.

Reference #1: Galiè N, Simonneau G. The Fifth World Symposium on Pulmonary Hypertension. J Am Coll Cardiol. 2013;62(25_S).

DISCLOSURE: The following authors have nothing to disclose: J Saadi Imam, Scott Helgeson, Mohamad Zetir, Charles Burger

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