Exercise-induced Pulmonary Hypertension in Patients With Sickle-cell Anemia
Exercise-induced Pulmonary Hypertension in Patients With Sickle-cell Anemia
Pulmonary hypertension (PH) at rest is a risk factor for death in patients with sickle-cell
 anemia (SCA). Exercise echocardiography (EE) can detect latent PH. We sought to investigate
 the occurrence of exercise-induced PH in patients with SCA and normal pulmonary pressure (PP)
 at rest, and its relationship with clinical and echocardiographic variables.Forty-four
 patients with SCA and normal PP at rest were studied and divided into two groups: exhibiting
 normal PP after treadmill EE (TRV≤2.7m/s) (G1), and exhibiting exercise-induced PH
 (TRV>2.7m/s) (G2). TRV cutoff points at rest and during exercise were based on data from
 healthy control subjects, matched for age, sex, and body surface area. Data obtained from EE
 were correlated with clinical, echocardiographic and ergometric variables.Exercise-induced PH
 occurred in 57% of the sample (G2), significantly higher than those of G1. Exercise-induced
 PH was related to higher levels of creatinine (p<0.05), increased left atrial volume (p<0.05)
 and right ventricular diastolic area (p<0.05), larger E/Em waves ratio derived from spectral
 and tissue Doppler (p<0.05), and higher TRV at rest (p<0.005).We concluded that patients with
 SCA and normal PP at rest may exhibit exercise-induced PH, which was related to renal
 function, increased cardiac chambers, abnormal indices of diastolic function and baseline TRV
 levels.
Pulmonary hypertension (PH) at rest is a risk factor for death in patients with sickle-cell
 anemia (SCA). Exercise echocardiography (EE) can detect latent PH. We sought to investigate
 the occurrence of exercise-induced PH in patients with SCA and normal pulmonary pressure (PP)
 at rest, and its relationship with clinical and echocardiographic variables.Forty-four
 patients (22 men, mean age 25 y.o.) with SCA and normal PP at rest (tricuspid regurgitant jet
 flow velocity [TRV] <2.5 m/s) were studied and divided into two groups: exhibiting normal PP
 after treadmill EE (TRV≤2.7m/s) (G1), and exhibiting exercise-induced PH (TRV>2.7m/s) (G2).
 TRV cutoff points at rest and during exercise were based on data from healthy control
 subjects, matched for age, sex, and body surface area. Data obtained from EE were correlated
 with clinical, echocardiographic and ergometric variables.Exercise-induced PH occurred in 57%
 of the sample (G2), with mean TRV level of 3.4±0.4 m/s (range 2.8 - 4.5m/s), significantly
 higher than those of G1 (2.5±0.3 m/s, p<0.001). Exercise-induced PH was related to higher
 levels of creatinine (p<0.05), increased left atrial volume (p<0.05) and right ventricular
 diastolic area (p<0.05), larger E/Em waves ratio derived from spectral and tissue Doppler
 (p<0.05), and higher TRV at rest (p<0.005).We concluded that patients with SCA and normal PP
 at rest may exhibit exercise-induced PH, which was related to renal function, increased
 cardiac chambers, abnormal indices of diastolic function and baseline TRV levels. The
 clinical meaning of these findings requires clarification in future studies.
Inclusion Criteria:

 -  patients with Sickle Cell Anemia homozygous to hemoglobin S

 -  preserved physical capacity

 -  steady state of the disease

 Exclusion Criteria:

 -  Pulmonary Hypertension at rest

 -  Recent sickling crisis(<2 months)

 -  High Blood Pressure

 -  Atrial fibrillation

 -  Chronic obstructive pulmonary disease

 -  Last blood transfusion > 3 months before

 -  Signs of congestive heart failure or significant valve disease

 -  Normal global and regional systolic function on echocardiography