Exercise-induced Pulmonary Hypertension in Patients With Sickle-cell Anemia

Exercise-induced Pulmonary Hypertension in Patients With Sickle-cell Anemia: an Echocardiographic Study.

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.

Study Overview

• Status: Completed
• Conditions: Sickle Cell Anemia; Pulmonary Hypertension

Detailed Description

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.

• Study Type: Observational
• Enrollment (Actual): 44

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 17 years and older (Child, Adult, Older Adult)
• Accepts Healthy Volunteers: Yes
• Genders Eligible for Study: All

• Sampling Method: Probability Sample

Study Population

patients with SCA, homozygous to hemoglobin S with preserved physical capacity, followed at the Hematology and Blood Transfusion Division of the Federal University of Sao Paulo (UNIFESP) or at the Hematology and Oncology Division of Santa Casa de Misericordia de Sao Paulo Medical School.

Description

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

Study Plan

How is the study designed?

Number of groups / cohorts

2

Cohorts and Interventions

Group / Cohort
Patients without pulmonary hypertension; Patients with sickle cell with normal response of pulmonary artery pressure to exercise
Exercise-induced pulmonary hypertension; Patients with sickle-cell anemia with exercise-induced pulmonary hypertension.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Time Frame
Exercise-induced pulmonary hypertension	until one minute after exercise

Collaborators and Investigators

• Sponsor: Federal University of São Paulo
• Investigators: Study Chair: Orlando C Filho, Federal University of São Paulo

Study record dates

Study Major Dates

• Study Start: June 1, 2006
• Primary Completion (Actual): December 1, 2006
• Study Completion (Actual): November 1, 2007

Study Registration Dates

• First Submitted: July 28, 2009
• First Submitted That Met QC Criteria: July 28, 2009
• First Posted (Estimate): July 29, 2009

Study Record Updates

• Last Update Posted (Estimate): July 29, 2009
• Last Update Submitted That Met QC Criteria: July 28, 2009
• Last Verified: July 1, 2009

More Information

Terms related to this study

• Keywords: Pulmonary Hypertension; Echocardiography; Sickle Cell Anemia; Exercise echocardiography
• Additional Relevant MeSH Terms: Cardiovascular Diseases; Vascular Diseases; Respiratory Tract Diseases; Lung Diseases; Hematologic Diseases; Genetic Diseases, Inborn; Anemia, Hemolytic, Congenital; Anemia, Hemolytic; Hemoglobinopathies; Hypertension; Anemia; Anemia, Sickle Cell; Hypertension, Pulmonary
• Other Study ID Numbers: HPSC01