Study of Pulmonary Hypertension and Left ventricular diastolic dysfunction in Patients of Sickle Cell Anaemia

Background: Sickle Cell Disease(SCD) is a complex disorder with multi-organ complications. Cardiovascular abnormalities are well-recognized complication in SCD with cardiac enlargement and systolic murmurs being the most common findings. Multiple pathologic and clinical studies have shown that patients with pulmonary hypertension and diastolic left ventricular dysfunction represent a particularly high-risk subgroup. These cardiopulmonary complications contribute to a markedly low functional capacity and associated high risk of both sudden death and severe multi-organ dysfunction in SCD patients. The aim of study was to assess prevalence of Pulmonary hypertension and Left ventricular diastolic dysfunction by using 2D Echocardiography in the patients of sickle cell anemia. Material and Methods: This study was conducted in the Department of Medicine, Pt. Jawaharlal Nehru Memorial Medical College and Dr. B.R. A.M. Hospital, Raipur (C.G.) among the patients presenting in O.P.D. and admitted in medicine ward from Dec. 2013 to Sep. 2015. A total of 100 patients were taken fulfilling the inclusion criteria for the study. Detailed clinical history was recorded and all patients underwent complete clinical examination. Hb electrophoresis and 2D Echocardiography of all patients were done. Results: In our study out of 100 patients, 33 patients (33%) were male while 67 patients (67%) were female. 70 subjects were having sickle cell trait out of which 18 patients were male and 52 were female, whereas 30 subjects were having sickle cell disease out of which 15 patients were male and 15 were female. Mean Hb level was 8.41 ± 1.74 gm%. In our study population maximum Hb was 12 gm% whereas minimum Hb was 4 gm%. Diastolic dysfunction was present in 50% of the total cases (100) among them 45.45% were males, 52.23% were females. Diastolic dysfunction was almost equally prevalent among female compared to male (p=0.51). Mean age of women with diastolic dysfunction was 29.37 ± 9.89 yrs, whereas mean age of men with diastolic dysfunction was 29.82 ± 10.24 yrs. Statistically there was no association was found between age and LVDD prevalence ( p=0.99). In our study we found prevalence of LVDD in studied genotypes i.e. AS and SS to be almost similar (P = 0.62, non-significant). Our study showed that diastolic dysfunction is more closely associated with moderate (Hb level 7-10 gm%) and severe anemia (Hb level 4-7gm%) than mild anemia (Hb level >10%). Statistically it was found to be significant (p=0.006). Mean Hb level of patients with diastolic dysfunction was 7.88 ± 1.81 gm%, whereas mean Hb level of patients without diastolic dysfunction was 8.94 ± 1.49 gm% (P<0.01, significant). Pulmonary Hypertension (PH) was present in 24 cases (24%) of the study population, among them 5 were males and 19 were females. There was no significant difference of prevalence of PH between both genders. (p=0.14). Out of 100 patients 12 patients (12%) had both LVDD and PH. Conclusion: The findings in our study indicate that in patients of sickle cell anemia, left ventricular diastolic function is impaired much before development of systolic dysfunction. Lower Hb levels in patients of sickle cell anemia is associated with cardiopulmonary complications. No significant correlation was found in respect to prevalence of PH and LVDD with age or gender. More so presence of both PH and LVDD significantly contributes to a higher mortality in patients of sickle cell anemia. Therefore by early detection we can start early treatment and can retard the progression of these cardiopulmonary abnormalities and its future consequences. Doppler Echocardiography is a simple, non-invasive, easily available technique that identifies sickle cell subjects who have asymptomatic pulmonary hypertension and/or left ventricular diastolic dysfunction much before abnormalities detected on ECG or by clinical examination. Therefore echocardiography becomes an indispensable tool for assessment of cardiac function in SCD patients.