Pulmonary Vascular Disease |

Pulmonary Arterial Hypertension in Pediatric Patients With Chronic Lung Disease: Insights From REVEAL FREE TO VIEW

Delphine Yung*, MD; George Mallory, MD; Lori Parsons, BS; Robyn Barst, MD
Author and Funding Information

University of Washington, Seattle Children's Hospital, Seattle, WA

Chest. 2012;142(4_MeetingAbstracts):812A. doi:10.1378/chest.1386299
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SESSION TYPE: Pulmonary Hypertension II

PRESENTED ON: Monday, October 22, 2012 at 04:00 PM - 05:30 PM

PURPOSE: Chronic lung disease (CLD) and asthma are not infrequent in children. Because the pulmonary vasculature is intricately linked to pulmonary growth and development during childhood, such comorbidities could worsen pulmonary arterial hypertension (PAH) severity and affect outcome.

METHODS: Patients aged ≤18 years at diagnosis, enrolled from March 2006-December 2009, in the Registry to EValuate Early And Long-term PAH Disease Management (REVEAL), a US-based, multicenter, observational registry, were included in the analysis. Patients (n=194) were grouped into the following 4 subgroups: CLD (n=54, 28%); ground glass abnormalities (GGA) on CT with normal pulmonary function tests (PFTs; n=14, 7%); asthma by history with normal PFTs (n=25, 13%); and no CLD (n=101, 52%). CLD was defined as: 1) reported lung dysplasia; 2) obstructive sleep apnea; or 3) abnormal PFTs with an abnormal chest CT, associated connective tissue disease, or a history of asthma. Characteristics analyzed included functional class, 6-minute walk distance (6MWD), hemodynamic parameters, PFTs, and treatment patterns. Kaplan-Meier survival estimates were calculated 3 years after enrollment.

RESULTS: Significant differences included: 1) lower 6MWD (±SD) in CLD patients versus patients without CLD (377±125m vs 461±131m, P=0.001) and 2) higher mean right atrial pressures (±SD) and pulmonary capillary wedge pressures (±SD) in patients with GGA versus patients without CLD (8.9±5.3 mmHg vs 6.4±2.8 mmHg, P=0.006; and 10.6±3.3 mmHg vs 8.5±3.2 mmHg, P=0.022, respectively). Survival estimates (±SD) at 3 years post-enrollment were numerically lower in patients with CLD (81%±6%, P=0.11) and in patients with asthma (78%±9%, P=0.45) versus patients without CLD (87%±3%).

CONCLUSIONS: Although there were no significant differences in 3-year survival estimates for patients with CLD or for patients with asthma versus patients without CLD, the tendency toward worse survival in patients with CLD would be of interest if it were replicated in other studies.

CLINICAL IMPLICATIONS: Evidence of lung disease is common in this large cohort of pediatric PAH patients without a primary diagnosis of lung disease, but differences in survival have not yet been found.

DISCLOSURE: Delphine Yung: Grant monies (from industry related sources): Dr Yung has a contract grant with Actelion

George Mallory: Grant monies (from industry related sources): Dr Mallory's section receives research funds for the TOPP and REVEAL Registries, funded by Actelion

Lori Parsons: Other: Lori Parsons is an employee of ICON Late Phase & Outcomes Research, a company that receives research funding from Actelion and other pharmaceutical companies

Robyn Barst: Consultant fee, speaker bureau, advisory committee, etc.: Dr Barst serves as a consultant for Actelion and other pharmaceutical s companies. She is also a member of the REVEAL Steering Committee which is supported by Actelion

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University of Washington, Seattle Children's Hospital, Seattle, WA




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