Pulmonary Vascular Disease |

Evolution to Pulmonary Arterial Hypertension in Patients With Collagen Vascular Disease With Borderline Elevations in Mean Pulmonary Artery Pressure (Pre-PAH Group) FREE TO VIEW

Sonu Sahni; Sameer Verma; Alexander Blood; Anup Singh; Nina Kohn; Arunabh Talwar
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North Shore-LIJ Health System, Manhasset, NY

Chest. 2014;146(4_MeetingAbstracts):864A. doi:10.1378/chest.1995115
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SESSION TITLE: Pulmonary Hypertension

SESSION TYPE: Original Investigation Slide

PRESENTED ON: Tuesday, October 28, 2014 at 02:45 PM - 04:15 PM

PURPOSE: Pulmonary arterial hypertension (PAH) is defined as mean pulmonary artery pressures (mPAP) > 25 mmHg on right heart catheterization (RHC) and pulmonary capillary wedge pressure (PCWP) < 15mmHg. There is a group of patients with borderline elevation of mPAP (20 to 24 mmHg) which can be labeled as a Pre-PAH group and many of these can subsequently develop PAH. The purpose of this study was to evaluate evolution to PAH in a well defined Pre-PAH group based on RHC.

METHODS: 33 consecutive patients with Pre-PAH on RHC were managed over 13 years (2000-2013) at our Advanced Lung Center. 12/33 (36.4%) patients had collagen vascular disease (CVD); and a retrospective chart methodology was applied on this cohort. Patient data of PFT, echocardiogram and 6 minute walk distance (6MWD) was collected to evaluate for progression of PAH. Repeat RHC was done as per clinical judgment of physician.

RESULTS: 33 patients [14 males, 21 females; mean age of 63.7 years] had mPAP of 20 to 24 mmHg. Of the CVD patients, 10 (83.3%) were at functional class II at initial evaluation. Carbon monoxide diffusion capacity ranged between 29% and 79% of predicted. Mean 6MWD was 344.96 + 67.7m. Repeat RHC showed that 4/12 (33.3%) patients developed definitive PAH and 1 (8.3%) patient develop pulmonary hypertension (WHO Group II). Average time between initial and diagnostic RHC was 624.4 ± 254.9 days. Of the remaining patients, 4/12 (33.3%) died due to worsening co-morbidities, 1/12 (8.3%) was lost to follow up and 2/12 (16.7%) have remained stable on echocardiogram and PFTs.

CONCLUSIONS: Patients with Pre-PAH, especially those with CVD are at high risk of developing PAH and hence, must be monitored closely by a pulmonary hypertension specialist.

CLINICAL IMPLICATIONS: Further prospective studies should be conducted to determine the rate of evolution to PAH in this high risk CVD group.

DISCLOSURE: The following authors have nothing to disclose: Sonu Sahni, Sameer Verma, Alexander Blood, Anup Singh, Nina Kohn, Arunabh Talwar

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