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Pulmonary Hypertension Care Centers


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Murali Chakinala, MD
Division of Pulmonary & Critical Care
Washington University
St. Louis, MO, USA
chakinalam@wustl.edu




Our understanding and management of pulmonary arterial hypertension (PAH) has advanced tremendously over the last 30 years. Numerous vasomodulating therapies have been developed, and their widespread use has been associated with longer survival and improved quality of life. Even though PAH remains a rare disease with challenging therapies, the delivery of health-care in PAH has been transformed from experts at tertiary care centers to a broad spectrum of providers with varying degrees of expertise, leading to non-uniformity of care. Concomitantly, PAH-specific therapy has been applied to an increasingly diverse population of patients with PH. As a result, early access to expert centers and assurances of optimal patient-care have become relevant concerns.

Recent publications have shed light on these challenges. In the RePHerral Study from 3 large university-based tertiary care referral centers in the US, 98 of 140 referred patients had been assigned a diagnosis of PAH before referral, but 32 (33%) were subsequently determined to be misdiagnosed. Forty-two patients were started on PAH-specific medications prior to referral, and 24 of these therapies were contrary to published guidelines. Fifty-nine patients had not had a pre-referral right heart catheterization [1]. Additional literature suggests that patients followed outside of a referral center (compared with the individuals already under the referral center's care) are treated with oral therapies longer, are more compromised and more likely to need urgent initiation of parenteral prostanoids, and have lower survival rates even after prostanoids are initiated [2]. Evidence from the REVEAL registry demonstrates that a substantial number of patients in functional class III or IV within 6 months of death had not received parenteral prostanoid at the time of death, suggesting under-utilization of the most potent and effective class of therapies [3]. These reports intimate the perception of inaccurate diagnosis of PAH, untimely referral to expert centers, and inappropriate utilization of advanced therapies.

Two years ago, the PHA and its Scientific Leadership Council (SLC) spawned an initiative to address these challenges facing the PH community and created the Pulmonary Hypertension Comprehensive Care (PHCC) Committee. The initiative's mission statement is to establish a program of accredited centers with expertise in pulmonary hypertension that aspires to improve overall quality of care and ultimately improve outcomes of patients with pulmonary hypertension, particularly pulmonary arterial hypertension, a rare and life-threatening disease.

The PHCC Committee studied and patterned the program after the successful Cystic Fibrosis Foundation Accredited Care Centers. The over-arching objectives of the PHCC program are to improve overall care and long-term patient outcomes, which can be accomplished through several interlocking components:

The design for two types of Centers (i.e. Centers of Comprehensive Care and Regional Clinical Programs) is a central feature of the program that should maximize the eventual number of PHCCs across the country and enhance access to expert care. Both designations will be promoted as PHA-accredited PHCCs and will have to meet their respective criteria through the same application process and evaluation method. Both will have to broadly satisfy several categories of criteria, including Center Director, Center Coordinator, Program Staff/Support Services, Facilities, and Research (CCC only) [visit www.phassociation.org/phcarecenters]. Although inclusivity is emphasized, the criteria and the accreditation program still must adhere to standards for selecting PHCCs, so that the designation represents a tangible achievement and conveys meaningful information to relevant stakeholders.

The PHCC initiative has generated tremendous interest. At the cusp of launching this much needed grass-roots program, it is vital to appreciate the enormity of the project and its potential consequences without becoming paralyzed by fear and uncertainty. For the sake of our patients, the PH community needs to find the courage and perseverance to forge ahead!

Disclosure Statement: the author reports the following disclosures:


References:

  1. Deano RC, Glassner-Kolmin C, Rubenfire M, et al. Referral of Patients With Pulmonary Hypertension Diagnoses to Tertiary Pulmonary Hypertension Centers: The Multicenter RePHerral Study. JAMA internal medicine 2013:1-7
  2. Badagliacca R, Pezzuto B, Poscia R, et al. Prognostic factors in severe pulmonary hypertension patients who need parenteral prostanoid therapy: the impact of late referral. J Heart Lung Transplant 2012; 31:364-372
  3. Farber HW, Miller DP, Meltzer LA, et al. Treatment of patients with pulmonary arterial hypertension at the time of death or deterioration to functional class IV: insights from the REVEAL Registry. The Journal of heart and lung transplantation: the official publication of the International Society for Heart Transplantation 2013; 32:1114-1122



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