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Pulmonary Vascular Disease |

Time to Recognition of Pediatric Pulmonary Arterial Hypertension — Factors Identified From the REVEAL Registry

Shirleen Loloyan*, MD; Robyn Barst, MD; Lynette Brown, MD; D. Dunbar Ivy, MD; Erika Rosenzweig, MD; Ginny Lai, MPH; C. Gregory Elliott, MD; Jacqueline Szmuszkovicz, MD; Ronald Oudiz, MD
Author and Funding Information

Children's Hospital Los Angeles, Keck School of Medicine/University of Southern California, Los Angeles, CA


Chest. 2012;142(4_MeetingAbstracts):824A. doi:10.1378/chest.1385734
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Abstract

SESSION TYPE: DVT/PE/Pulmonary Hypertension Posters I

PRESENTED ON: Wednesday, October 24, 2012 at 01:30 PM - 02:30 PM

PURPOSE: Pulmonary artery hypertension (PAH) presents with a broad spectrum of clinical features in both children and adults. Disease recognition is often delayed (defined as >12 months from symptom onset to being told they have PAH, initiating PAH-specific therapy, or undergoing a diagnostic right heart catheterization [RHC]) as symptoms are often ascribed to more common diseases like asthma. We sought to identify factors associated with time to PAH recognition using data from the pediatric population enrolled in the Registry to EValuate Early And Long-term Pulmonary Arterial Hypertension Disease Management (REVEAL).

METHODS: Of the 3515 PAH patients enrolled in REVEAL between March 2006 and October 2011, 207 children met inclusion criteria at diagnostic RHC: <19 yrs: mean pulmonary arterial pressure >25 mm Hg at rest; pulmonary vascular resistance >3 Wood units; pulmonary capillary wedge pressure ≤15 mm Hg. Patients with missing data were excluded.

RESULTS: Of the 207 patients included, 72.5% (n=150) had a time to disease recognition of ≤1 year from symptom onset, while the remaining 27.5% (n=57) had >1 yr delay. An adjusted logistic regression model indicated that time to PAH recognition >1 yr was associated with female gender (adjusted OR 2.38; CI 1.18-4.80; P=0.015), absence of peripheral edema (adjusted OR 0.0 P=0.003), and absence of acute vasodilator response (adjusted OR 0.38; CI 0.14-1.01; P=0.054).

CONCLUSIONS: Despite progress in PAH treatment, time to disease recognition has not significantly improved. Unlike adult patients (>2 years delay), we found female gender, absence of peripheral edema, and absence of acute vasodilator response to be associated with delayed diagnosis in pediatric patients. Reasons for these associations are unclear.

CLINICAL IMPLICATIONS: PAH is a rapidly progressive disease with significant morbidity and mortality. Identifying factors that may contribute to delay in recognition should lead to earlier initiation of treatment with anticipated improved quality of life and outcomes.

DISCLOSURE: Robyn Barst: Consultant fee, speaker bureau, advisory committee, etc.: Consultant for Actelion and member of REVEAL Steering Committee which is supported by Actelion

Lynette Brown: Grant monies (from industry related sources): Receives grant support from Actelion

D. Dunbar Ivy: Consultant fee, speaker bureau, advisory committee, etc.: Consultant for Actelion

Erika Rosenzweig: Consultant fee, speaker bureau, advisory committee, etc.: Consultant for Actelion

Ginny Lai: Other: Employee of ICON Late Phase & Outcomes Research, a company which receives research funding from Actelion and other pharmaceutical companies

C. Gregory Elliott: Grant monies (from industry related sources): Receives grant support from Actelion, Consultant fee, speaker bureau, advisory committee, etc.: Member of REVEAL Steering Committee which is supported by Actelion

Ronald Oudiz: Consultant fee, speaker bureau, advisory committee, etc.: Consultant for Actelion

The following authors have nothing to disclose: Shirleen Loloyan, Jacqueline Szmuszkovicz

No Product/Research Disclosure Information

Children's Hospital Los Angeles, Keck School of Medicine/University of Southern California, Los Angeles, CA

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