Hemodynamic Mechanisms of Exercise-Induced Pulmonary Hypertension in Patients with Lymphangioleiomyomatosis: The Role of Exercise Stress Echocardiography

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Abstract

Background: The pathogenesis of pulmonary hypertension (PH) in lymphangioleiomyomatosis (LAM) has not yet been completely clarified. The aim of this study was to conduct a noninvasive evaluation of the main hemodynamic mechanisms of exercise-induced PH in patients with LAM, assessed using exercise stress echocardiography. Methods: Fifteen patients with LAM (mean age, 47 ± 13 years; all women) without resting PH were enrolled in a prospective single-center study and compared with 15 healthy female control subjects (mean age, 45.2 ± 8 years; P =.65). A complete echocardiographic study with Doppler tissue imaging was performed at baseline and during semisupine symptom-limited exercise testing to evaluate (1) left ventricular systolic and diastolic function, (2) right ventricular contractile function, (3) estimated pulmonary capillary wedge pressure, (4) estimated systolic and mean pulmonary artery pressure, and (5) estimated pulmonary vascular resistance. Results: Compared with healthy control subjects, patients with LAM during exercise showed echocardiographic signs of right ventricular overload and right ventricular systolic dysfunction and significant increases in mean pulmonary artery pressure (14.4 ± 6.5 vs 4.2 ± 3.1 mm Hg, P <.0001), pulmonary vascular resistance (+68.3 ± 42.1 vs −0.1 ± 18.3 dyne-sec/cm5, P <.0001), and, unexpectedly, pulmonary capillary wedge pressure (+8.3 ± 5.3 vs −0.5 ± 1.3 mm Hg, P <.0001). Conclusions: Exercise-induced PH in patients with LAM could be related not only to hypoxic pulmonary vascular vasoconstriction during exercise (precapillary PH) but also to a significant exercise-induced increase in estimated pulmonary capillary wedge pressure, probably secondary to diastolic dysfunction (postcapillary PH).

Original languageEnglish
Pages (from-to)888-901
Number of pages14
JournalJournal of the American Society of Echocardiography
Volume31
Issue number8
DOIs
Publication statusPublished - Aug 1 2018

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Lymphangioleiomyomatosis
Stress Echocardiography
Pulmonary Hypertension
Hemodynamics
Exercise
Pulmonary Wedge Pressure
Vascular Resistance
Pulmonary Artery
Right Ventricular Dysfunction
Pressure
Right Ventricular Function
Vasoconstriction
Blood Vessels
Healthy Volunteers
Lung

Keywords

  • Cystic lung diseases
  • Diastolic dysfunction
  • Exercise stress echocardiography
  • Exercise-induced pulmonary hypertension

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Cardiology and Cardiovascular Medicine

Cite this

@article{f0785c631b1044649be9499dfdf68f67,
title = "Hemodynamic Mechanisms of Exercise-Induced Pulmonary Hypertension in Patients with Lymphangioleiomyomatosis: The Role of Exercise Stress Echocardiography",
abstract = "Background: The pathogenesis of pulmonary hypertension (PH) in lymphangioleiomyomatosis (LAM) has not yet been completely clarified. The aim of this study was to conduct a noninvasive evaluation of the main hemodynamic mechanisms of exercise-induced PH in patients with LAM, assessed using exercise stress echocardiography. Methods: Fifteen patients with LAM (mean age, 47 ± 13 years; all women) without resting PH were enrolled in a prospective single-center study and compared with 15 healthy female control subjects (mean age, 45.2 ± 8 years; P =.65). A complete echocardiographic study with Doppler tissue imaging was performed at baseline and during semisupine symptom-limited exercise testing to evaluate (1) left ventricular systolic and diastolic function, (2) right ventricular contractile function, (3) estimated pulmonary capillary wedge pressure, (4) estimated systolic and mean pulmonary artery pressure, and (5) estimated pulmonary vascular resistance. Results: Compared with healthy control subjects, patients with LAM during exercise showed echocardiographic signs of right ventricular overload and right ventricular systolic dysfunction and significant increases in mean pulmonary artery pressure (14.4 ± 6.5 vs 4.2 ± 3.1 mm Hg, P <.0001), pulmonary vascular resistance (+68.3 ± 42.1 vs −0.1 ± 18.3 dyne-sec/cm5, P <.0001), and, unexpectedly, pulmonary capillary wedge pressure (+8.3 ± 5.3 vs −0.5 ± 1.3 mm Hg, P <.0001). Conclusions: Exercise-induced PH in patients with LAM could be related not only to hypoxic pulmonary vascular vasoconstriction during exercise (precapillary PH) but also to a significant exercise-induced increase in estimated pulmonary capillary wedge pressure, probably secondary to diastolic dysfunction (postcapillary PH).",
keywords = "Cystic lung diseases, Diastolic dysfunction, Exercise stress echocardiography, Exercise-induced pulmonary hypertension",
author = "Andrea Sonaglioni and Massimo Baravelli and Roberto Cassandro and Olga Torre and Davide Elia and Claudio Anz{\`a} and Sergio Harari",
year = "2018",
month = "8",
day = "1",
doi = "10.1016/j.echo.2018.02.004",
language = "English",
volume = "31",
pages = "888--901",
journal = "Journal of the American Society of Echocardiography",
issn = "0894-7317",
publisher = "Mosby Inc.",
number = "8",

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TY - JOUR

T1 - Hemodynamic Mechanisms of Exercise-Induced Pulmonary Hypertension in Patients with Lymphangioleiomyomatosis

T2 - The Role of Exercise Stress Echocardiography

AU - Sonaglioni, Andrea

AU - Baravelli, Massimo

AU - Cassandro, Roberto

AU - Torre, Olga

AU - Elia, Davide

AU - Anzà, Claudio

AU - Harari, Sergio

PY - 2018/8/1

Y1 - 2018/8/1

N2 - Background: The pathogenesis of pulmonary hypertension (PH) in lymphangioleiomyomatosis (LAM) has not yet been completely clarified. The aim of this study was to conduct a noninvasive evaluation of the main hemodynamic mechanisms of exercise-induced PH in patients with LAM, assessed using exercise stress echocardiography. Methods: Fifteen patients with LAM (mean age, 47 ± 13 years; all women) without resting PH were enrolled in a prospective single-center study and compared with 15 healthy female control subjects (mean age, 45.2 ± 8 years; P =.65). A complete echocardiographic study with Doppler tissue imaging was performed at baseline and during semisupine symptom-limited exercise testing to evaluate (1) left ventricular systolic and diastolic function, (2) right ventricular contractile function, (3) estimated pulmonary capillary wedge pressure, (4) estimated systolic and mean pulmonary artery pressure, and (5) estimated pulmonary vascular resistance. Results: Compared with healthy control subjects, patients with LAM during exercise showed echocardiographic signs of right ventricular overload and right ventricular systolic dysfunction and significant increases in mean pulmonary artery pressure (14.4 ± 6.5 vs 4.2 ± 3.1 mm Hg, P <.0001), pulmonary vascular resistance (+68.3 ± 42.1 vs −0.1 ± 18.3 dyne-sec/cm5, P <.0001), and, unexpectedly, pulmonary capillary wedge pressure (+8.3 ± 5.3 vs −0.5 ± 1.3 mm Hg, P <.0001). Conclusions: Exercise-induced PH in patients with LAM could be related not only to hypoxic pulmonary vascular vasoconstriction during exercise (precapillary PH) but also to a significant exercise-induced increase in estimated pulmonary capillary wedge pressure, probably secondary to diastolic dysfunction (postcapillary PH).

AB - Background: The pathogenesis of pulmonary hypertension (PH) in lymphangioleiomyomatosis (LAM) has not yet been completely clarified. The aim of this study was to conduct a noninvasive evaluation of the main hemodynamic mechanisms of exercise-induced PH in patients with LAM, assessed using exercise stress echocardiography. Methods: Fifteen patients with LAM (mean age, 47 ± 13 years; all women) without resting PH were enrolled in a prospective single-center study and compared with 15 healthy female control subjects (mean age, 45.2 ± 8 years; P =.65). A complete echocardiographic study with Doppler tissue imaging was performed at baseline and during semisupine symptom-limited exercise testing to evaluate (1) left ventricular systolic and diastolic function, (2) right ventricular contractile function, (3) estimated pulmonary capillary wedge pressure, (4) estimated systolic and mean pulmonary artery pressure, and (5) estimated pulmonary vascular resistance. Results: Compared with healthy control subjects, patients with LAM during exercise showed echocardiographic signs of right ventricular overload and right ventricular systolic dysfunction and significant increases in mean pulmonary artery pressure (14.4 ± 6.5 vs 4.2 ± 3.1 mm Hg, P <.0001), pulmonary vascular resistance (+68.3 ± 42.1 vs −0.1 ± 18.3 dyne-sec/cm5, P <.0001), and, unexpectedly, pulmonary capillary wedge pressure (+8.3 ± 5.3 vs −0.5 ± 1.3 mm Hg, P <.0001). Conclusions: Exercise-induced PH in patients with LAM could be related not only to hypoxic pulmonary vascular vasoconstriction during exercise (precapillary PH) but also to a significant exercise-induced increase in estimated pulmonary capillary wedge pressure, probably secondary to diastolic dysfunction (postcapillary PH).

KW - Cystic lung diseases

KW - Diastolic dysfunction

KW - Exercise stress echocardiography

KW - Exercise-induced pulmonary hypertension

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U2 - 10.1016/j.echo.2018.02.004

DO - 10.1016/j.echo.2018.02.004

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