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Exercise-induced pulmonary hypertension in hypertrophic cardiomyopathy: a combined cardiopulmonary exercise test—echocardiographic study

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Abstract

Pulmonary arterial hypertension (PAH), documented in a significant portion of hypertrophic cardiomyopathy (HCM) patients, has been shown to adversely impact prognosis. In most HCM patients congestive symptoms are consistently elicited by exercise, thus suggesting the need for a provocative test to assess cardiac hemodynamics during effort. Combining cardiopulmonary exercise test (CPET) with echocardiography, we aimed to evaluate the presence of exercise induced pulmonary arterial hypertension (EiPAH), its role in functional limitation and its prognostic significance in a cohort of patients with obstructive and non-obstructive HCM. Study population included 182 HCM patients evaluated combining CPET and stress echocardiography. Left-ventricular outflow tract (LVOT) velocities, trans-tricuspid gradient, and cardiopulmonary variables were continuously measured. Thirty-seven patients (20%) developed EiPAH, defined as systolic pulmonary arterial pressure (sPAP) > 40 mmHg during exercise. EiPAH was associated with lower exercise performance, larger left atrial volumes, higher LVOT gradient and higher VE/VCO2 slope. At multivariable analysis baseline sPAP (p < 0.0001) and baseline LVOT obstruction (p = 0.028) were significantly associated with EiPAH. Kaplan–Meier curve analysis showed EiPAH was a significant predictor of HCM-related morbidity (Hazard Ratio 6.21, 95% CI 1.47–26.19; p = 0.05; 4.21, 95% CI 1.94–9.12; p < 0.001 for the primary and the secondary endpoint respectively). EiPAH was present in about one fifth of HCM patients without evidence of elevated pulmonary pressures at rest and was associated with adverse clinical outcome. Diagnosing EiPAH by exercise echocardiography/CPET may help physicians to detect early stage of PAH thus allowing a closer clinical monitoring and individualized therapies.

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The authors have no source of funding to declare.

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Authors and Affiliations

  1. Cardiology Division, Cardiac Arrhythmia Center and Cardiomyopathies Unit, St. Camillo-Forlanini Hospital, Rome, Italy

    Federica Re, Andrea Avella & Elisabetta Zachara

  2. Cardiac Unit, G. da Saliceto Hospital, AUSL Piacenza and University of Parma, Parma, Italy

    Geza Halasz

  3. Division of Cardiology, VCU Health Pauley Heart Center, Virginia Commonwealth University, Richmond, VA, USA

    Francesco Moroni

  4. Cardiology Unit, San Giovanni di Dio Hospital, Via Torregalli 3, 50142, Florence, Italy

    Matteo Beltrami

  5. Department of Cardiology, Misericordia Hospital, Grosseto, Italy

    Pasquale Baratta

  6. Cardiomyopathy Unit, Careggi University Hospital, Florence, Italy

    Iacopo Olivotto

Authors
  1. Federica Re
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  2. Geza Halasz
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  3. Francesco Moroni
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  4. Matteo Beltrami
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  5. Pasquale Baratta
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  6. Andrea Avella
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  7. Elisabetta Zachara
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  8. Iacopo Olivotto
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Contributions

FR, MB and IO conceived the idea presented, wrote the manuscript and supervised the work. GH and FM contributed to the acquisition, analysis, and interpretation of data for the work. PB collected figures and tables and performed the literature research. AA and EZ critically revised the manuscript. All authors contributed to the editorial changes in the manuscript. All authors read and approved the final manuscript.

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Correspondence to Matteo Beltrami.

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This study had local institutional and ethics committee approval.

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All patients provided written informed consent.

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Re, F., Halasz, G., Moroni, F. et al. Exercise-induced pulmonary hypertension in hypertrophic cardiomyopathy: a combined cardiopulmonary exercise test—echocardiographic study. Int J Cardiovasc Imaging 38, 2345–2352 (2022). https://doi.org/10.1007/s10554-022-02671-3

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  • DOI: https://doi.org/10.1007/s10554-022-02671-3

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