• Christian Gerges, Antoine Beurnier, Xavier Jaïs, Philippe Hervé, LauEdmund M TEMTDepartment of Respiratory Medicine, Royal Prince Alfred Hospital, Camperdown, Australia., Barbara Girerd, Sven Günther, Amir Bouchachi, Mitja Jevnikar, Athénaïs Boucly, Harm Jan Bogaard, Gérald Simonneau, Olivier Sitbon, Laurent Savale, Denis Chemla, Marc Humbert, and David Montani.
• Division of Cardiology, Department of Internal Medicine II, Medical University of Vienna, Vienna, Austria; School of Medicine, Université Paris-Saclay, Le Kremlin-Bicêtre, Paris, France; Service de Pneumologie et Soins Intensifs Respiratoires, AP-HP, Hôpital Bicêtre, Le Kremlin-Bicêtre, Paris, France.
• Chest. 2024 Jul 24.

BackgroundExercise hemodynamics are recommended for early detection of pulmonary arterial hypertension (PAH) and have been suggested to be predictive of future development of PAH in high-risk populations such as BMPR2 mutation carriers. However, the optimal exercise hemodynamic screening parameter remains to be determined. Recent data suggest that pulmonary vascular distensibility coefficient (α) may serve as a useful parameter for early detection of PAH.Research QuestionWhat is the value of exercise hemodynamics, including α, for predicting the occurrence of PAH during long-term follow-up in BMPR2 mutation carriers?Study Design And MethodsFifty-two asymptomatic BMPR2 mutation carriers who underwent symptom-limited exercise hemodynamic assessment were followed up for a median of 10 years. The impact of hemodynamics at rest and exercise, presence of exercise pulmonary hypertension, and α on occurrence of PAH during long-term follow-up were assessed.ResultsDuring long-term follow-up, five patients demonstrated PAH. Patients who demonstrated PAH showed a significantly lower α (0.8 ± 0.4%/mm Hg) than patients without PAH (1.8 ± 0.8%/mm Hg; P = .008). The only hemodynamic parameter that predicted the occurrence of PAH during long-term follow-up at regression analysis was α. Receiver operating characteristic analysis showed that α ≤ 1.5%/mm Hg predicted PAH occurrence with a specificity of 75% and sensitivity of 100%.InterpretationBefore development of PAH in BMPR2 mutation carriers, α is reduced markedly and may serve as a useful parameter in the setting of early disease detection. Given the low event rate, caution is warranted in interpreting these results, highlighting the need for validation studies.Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.

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