The aim of the present study is to provide hemodynamic and cardiopulmonary functions of sickle cell disease patients at a university hospital.
Patients with Sickle Cell Disease and tricuspid regurgitant jet velocity (TRJV) of ≥ 2.5 m/sec on a routine 2 D echocardiography were included in the analysis. Right heart catheterizations, pulmonary function testing, and exercise data was available for further analysis.
24 (13 male and 11 female) patients met the inclusion criteria with TRJV ≥ 2.5 m/s and mean age of 36.7 ± 11 years. 21 patients had diagnosis of HbSS, 2 HbSC and 1 HbS/beta-thalasemia. 11 patients were diagnosed with acute chest syndrome in the past 5 years. Echocardiogram showed a mean ejection fraction of 58 ± 5 and mean TRJV of 2.89 ± 0.47 m/s. Seven (35%) of these patients had mean pulmonary artery pressure mPAP > 25mmHg. Out of these 7 patients only 3 (10%) had PCWP ≤ 15 mmHg. When compared with patients without pulmonary hypertension, patients with pulmonary hypertension had higher TRJV (3.2 ± 0.73 VS 2.73 ± 0.15), and higher cardiac output (10.38 ± 2.98 VS 7.85 ± 1.96 L/m). All the patients pulmonary hypertension had restrictive lung physiology (TLC < 80 % predicted) vs 8 (61 %) of the patients without pulmonary hypertension. DLCO was significantly reduced in all patients (mean 20.67 ± 5.73).
The prevalence of pulmonary hypertension in sickle cell disease patients with TRJV ≥ 2.5 m/sec is 35%. One third had pulmonary arterial hypertension, and 2/3 had pulmonary venous hypertension. Almost all patients with pulmonary hypertension had TRJV > 2.7 m/sec. Restrictive lung physiology and history of acute chest syndrome is common among patients who suffer from pulmonary hypertension.
The prevalence of pulmonary hypertension in sickle cell disease patients is significant. Further emphasis on screening and evaluation is warranted.
Mohammad Al-Ali, No Financial Disclosure Information; No Product/Research Disclosure Information