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Pulmonary Vascular Disease |

Incremental Burden of Disease in Patients Diagnosed With Pulmonary Arterial Hypertension Receiving Monotherapy and Combination Vasodilator Therapy

Richard Channick*, MD; Mark Small, BS; James Piercy, MS; James Pike, MA; Jie Zhang, PhD; Annamaria Cerulli, MPH
Author and Funding Information

Massachusetts General Hospital, Boston, MA


Chest. 2012;142(4_MeetingAbstracts):825A. doi:10.1378/chest.1386169
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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 arterial hypertension (PAH) is a severely debilitating disease with high mortality. To achieve treatment goals, patients are increasingly treated with PAH-specific combination therapy, including endothelin receptor antagonists (ERA), phosphodiesterase 5-inhibitors (PDE5) and prostanoids (PGI2). However, limited data exists on the residual unmet need for patients treated with one or multiple PAH therapies. This analysis aimed to assess the burden of disease for patients with PAH treated with (a) monotherapy, (b) combination therapy excluding intravenous (IV) PGI2, and (c) combination therapy including IV PGI2.

METHODS: Data were drawn from the Adelphi PAH Disease Specific Programme, a cross sectional study of consulting patients undertaken in the US, Germany, Italy and the UK in 2010. Statistical tests were performed to detect significant differences in the burden of disease between groups. Outcomes included demographics, clinical characteristics, healthcare resource utilization, and quality of life measured by the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR).

RESULTS: 446 patients with a PAH diagnosis confirmed by right heart catheterisation were analysed (a=337, b= 88, c=21). Mean age was 52.2 years and 61.7% were female. The majority of patients were classified as WHO Functional Class (FC) II and III (a=46.7% [FC II], b=59.1%, c=50.0%) and the mean time since diagnosis was a=13.2, b=23.7, c=37.2 months (p<0.0001). Pulmonary vascular resistance was a=620.0, b=803.8 and c=981.7 dynes (p<0.0001) and 6 minute walk test was a=363.5, b=303.0, and c=261.1metres (p<0.001). Mean number of PAH-related hospitalizations in the past year were a=0.5, b=0.7 and c=2.2 (p<0.001). CAMPHOR symptom scores were a=10.1, b=12.9 and c=17.0 (p<0.001), functioning scores were a=8.4, b=10.7 and c=18.1 (p<0.0001) and quality of life scores were a=7.9, b=8.4 and c=12.2 (p=0.1232). Modified Medical Research Council dyspnoea scale was a=1.6, b=2.1 and c=2.7 (p<0.0001).

CONCLUSIONS: Patients with PAH receiving combination therapy including IV PGI2 suffer from greater disease burden compared with those on monotherapy or combination therapy excluding IV PGI2.

CLINICAL IMPLICATIONS: The association of escalating treatment with PAH disease severity suggests an ongoing treatment gap.

DISCLOSURE: Richard Channick: Consultant fee, speaker bureau, advisory committee, etc.: Richard Channick has consulted for and/or received research grants from companies that produce treatments for pulmonary hypertension, including Actelion Pharmaceuticals, Bayer, Novartis, and United Therapeutics

Mark Small: Employee: Mark Small is an employee of Adelphi Real World, who conducted the research. Mark Small does not have any personal financial relationship with Novartis

James Piercy: Employee: James Piercy is an employee of Adelphi Real World, who conducted the research. James Piercy does not have any personal financial relationship with Novartis

James Pike: Employee: James Pike is an employee of Adelphi Real World, who conducted the research. James Pike does not have any personal financial relationship with Novartis

Jie Zhang: Employee: Jie Zhang is an employee of Novartis Pharma AG, who funded this study

Annamaria Cerulli: Employee: Annamaria Cerulli is an employee of Novartis Pharma AG, who funded this study

No Product/Research Disclosure Information

Massachusetts General Hospital, Boston, MA

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