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 language | English |
|---|---|
| Pages (from-to) | 888-901 |
| Number of pages | 14 |
| Journal | Journal of the American Society of Echocardiography |
| Volume | 31 |
| Issue number | 8 |
| DOIs | |
| Publication status | Published - Aug 1 2018 |
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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
Hemodynamic Mechanisms of Exercise-Induced Pulmonary Hypertension in Patients with Lymphangioleiomyomatosis : The Role of Exercise Stress Echocardiography. / Sonaglioni, Andrea; Baravelli, Massimo; Cassandro, Roberto; Torre, Olga; Elia, Davide; Anzà, Claudio; Harari, Sergio.
In: Journal of the American Society of Echocardiography, Vol. 31, No. 8, 01.08.2018, p. 888-901.Research output: Contribution to journal › Article
}
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
UR - http://www.scopus.com/inward/record.url?scp=85044522102&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85044522102&partnerID=8YFLogxK
U2 - 10.1016/j.echo.2018.02.004
DO - 10.1016/j.echo.2018.02.004
M3 - Article
AN - SCOPUS:85044522102
VL - 31
SP - 888
EP - 901
JO - Journal of the American Society of Echocardiography
JF - Journal of the American Society of Echocardiography
SN - 0894-7317
IS - 8
ER -