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Mixed PH in the Real World- An Ill-defined Phenotype

patricia gresham

Vijay Balasubramanian

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Conference: 2017 PH Professional Network Symposium

Release Date: 10.06.2017

Presentation Type: Abstracts

File Download: 2017 PHPN Abstract 1025

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Abstract presented at the 2017 PH Professional Network Symposium held in Bethesda, MD on October 5-7, 2017

Purpose

Mixed PH in the Real World – An Ill-defined Phenotype
Patricia Gresham, Vijay Balasubramanian
Pulmonary Arterial Hypertension (PAH) is a devastating condition that leads to premature death if left undiagnosed or untreated. The Evian Classification and more recent revisions (J Am Coll Cardiol 2013;62(25 Suppl): D34-41) reclassified PH into 5 subgroups based upon etio-pathogenesis. Group I PH ( Pulmonary Arterial Hypertension, PAH) represents a growing list of entities, and outcomes are different for various phenotypes. In the “real world”, clinical characterization can often pose a challenge when there are mixed etiologies for PH in the same patient often referred to as “Mixed PH”. Proving primary Pulmonary vascular disease and initiation of pulmonary vasodilator therapy can be a very difficult decision. We wish to present a patient with “Mixed PH” in the real world, who demonstrated an impressive clinical response to Pulmonary vasodilator therapy despite very atypical hemodynamic characteristics.

Background

A 62-year-old HF presented to the hospital with a background of multiple hospitalizations for Dyspnea and recurrent edema. She had a history of CAD – Prior myocardial infarction along with 2 vessel disease, Valvular heart disease – Mitral regurgitation, Pacemaker insertion for Complete Heart Block. In addition, Pulmonary history included severe restrictive Lung disease (TLC 46%, DLCO 19%) & Prior large Right pleural effusion. Clinical and Hemodynamic Characteristics are summarized in the table below 

Methods

2012 Before TX 2015 Following
Left atrial Dim 4.5 4.5
LVEF 68% 65%
MR Mod to Sev Mild to Mod
mRAP 25 15
PAP 105/45 118/32
mPAP 65 60
PCWP 20 25
LVEDP 20
CO/CI 6.03/3.22 6.2/3.52
PVR 7.5 5.6
TPG/DPG 45/25 35/7
BNP 450 20
6MWT 207M 345M
Hospital Admissions
3 in 15 months 0
WHO FC IV II

Results

Pt had 3 hospitalizations prior to and none since initiation of Pulmonary vasodilator therapy.

Conclusion

This real world “Phenotype” is still not well-characterized by the available recognized tools and clearly may need to be studied further. Although, TPG & DPG are useful in clinical delineation, other biomarkers and clinical characteristics are needed to enable better definition of these unique phenotypes.