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Exercise‐Induced Pulmonary Hypertension in Scleroderma Patients: A Common Finding but with Elusive Pathophysiology

2012; Wiley; Volume: 30; Issue: 4 Linguagem: Inglês

10.1111/echo.12063

1540-8175

Rui Baptista, Sara Serra, Rui Martins, Maria João Salvador, Graça Castro, Manuel Gomes, Lèlita Santos, Pedro Monteiro, José António Pereira da Silva, Mariano Pêgo,

Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis

Background: The etiology of exercise‐induced pulmonary hypertension (ex PH ) in systemic sclerosis ( SS c) remains a complex task, as both left ventricle ( LV ) diastolic dysfunction and pulmonary vascular disease can contribute to its development. We determined the incidence of ex PH in SS c and examined the association between pulmonary artery systolic pressure ( PASP ) and tissue D oppler‐derived indexes of pulmonary capillary wedge pressure ( PCWP ). Methods: Thirty‐eight patients with SS c were studied, using a cycloergometer protocol; 10 were excluded due to resting PH or absence of tricuspid regurgitation ( TR ); TR and mitral E‐wave velocities, LV outflow tract time‐velocity integral and LV septal E′‐wave were measured before and in peak exercise to calculate cardiac output ( CO ), PCWP and pulmonary vascular resistance ( PVR ). Results: Mean age of diagnosis was 57.9 ± 8.9 years. At a mean workload of 64 ± 29 Watts, 48% of patients increased PASP ≥ 50 mmHg. PCWP , assessed by the E/e′ ratio, did not change significantly during exercise (10.2 ± 3.1–10.0 ± 5.1; P = NS ). Only 3 patients had elevations of the E/e′ ratio ≥ 13 during exercise; 2 of them had an exercise PASP ≥ 50 mmHg, yielding a proportion of ex PH due to elevated LV filling pressures of 2/11 (18%). Patients with ex PH had lower DL CO and had more frequently the diffuse SS c. Conclusion: The elevation of PASP during exercise in most patients of this cohort seems to be related to a reduced pulmonary vascular reserve, and not to an increase in PCWP . Further studies are warranted to determine the therapeutic, as well as prognostic implications of these findings.