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

An Unusual Cause of Dyspnea in Hereditary Hemorrhagic Telangiectasia FREE TO VIEW

Kai Chin Poh, MBBS; Keng Leong Tan, RCP
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Singhealth, Singapore, Singapore

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

Chest. 2016;150(4_S):1209A. doi:10.1016/j.chest.2016.08.1318
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SESSION TITLE: Student/Resident Case Report Poster - Pulmonary Vascular Disease I

SESSION TYPE: Student/Resident Case Report Poster

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

INTRODUCTION: Pulmonary hypertension (PH) is an underdiagnosed complication of hereditary hemorrhagic telangiectasia (HHT), which is associated with increased mortality1,2.

CASE PRESENTATION: A 50-year old female non-smoker presented with shortness of breath for the past 1 year. Pertinent past history included HHT with pulmonary arteriovenous malformation (AVM), status post coil embolization of her pulmonary AVM 3 years ago. Computed tomography imaging of the thorax did not show new AVM or recanalization of previously embolized AVM. Pulmonary function tests were normal. Cardiopulmonary exercise test showed reduced exercise capacity, anaerobic threshold, oxygen pulse, VE/MVV and increased VE/CO2, with no ventilatory abnormality observed. She was evaluated with transthoracic echocardiogram which showed a pulmonary artery systolic pressure of 64mmHg. Transesophageal echocardiogram showed normal valvular function and intact interatrial and interventricular septum. Diagnostic right heart catheterization study demonstrated pulmonary capillary wedge pressure of 8mm Hg, cardiac output of 4.1L/min and mean pulmonary arterial pressure of 64mmHg. There was no step up in oxygenation from the inferior venous cava to the pulmonary artery to suggest intra-cardiac shunt. Pulmonary vascular resistance was markedly elevated at 11 Woods, suggesting pulmonary arterial hypertension (PAH) as the cause of PH.

DISCUSSION: PH occurs in HHT due to different mechanisms; high flow, pulmonary venous hypertension (PVH) and PAH1. AVM may result in a hyper dynamic circulation and high cardiac output, leading to increased blood flow and pressure in the pulmonary artery. PAH is increasingly recognized as a cause of PH in patients with HHT2. Right heart catheterization is essential for accurate diagnosis and management of PH in HHT. Pulmonary AVM causing PH should be considered for embolization. Vasodilator therapy may improve symptoms in PAH but is not a recognized therapy in PVH3.

CONCLUSIONS: PH should be suspected in patients with HHT presenting with unexplained dyspnea. It is important to discern between the different causes of PH, as treatment options are different.

Reference #1: Lyle MA, Fenstad ER, McGoon MD, et al. Pulmonary Hypertension in the setting of Hereditary Hemorrhagic Telangiectasia. Chest. Jul 23 2015. 149(2): 362-371

Reference #2: Sopena B, Perez-Rodriguez MT, Portela D, Rivera A, Freire M, Martinez-Vazquez C. High prevalence of pulmonary hypertension in patients with hereditary hemorrhagic telangiectasia. European Journal of Internal Medicine. Apr 2013;24(3):e30-34.

Reference #3: Rich S, Rabinovitch M. Diagnosis and treatment of secondary (non-category 1) pulmonary hypertension. Circulation. Nov 18 2008;118(21):2190-2199.

DISCLOSURE: The following authors have nothing to disclose: Kai Chin Poh, Keng Leong Tan

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