Study of pulmonary hypertension as a complication of sickle cell disease
DOI:
https://doi.org/10.18203/2349-3933.ijam20200084Keywords:
Complication, Electrophoresis, Pulmonary hypertension, Prognosis, Sickle cell anaemia, Sickle cell diseaseAbstract
Background: Sickle cell anaemia is the most common inherited hematological abnormality across human race, particularly prevalent in pockets of central India and pulmonary hypertension (PH) supposedly worsens prognosis. Data from central India is lacking though. Present study aims to study incidence of pulmonary hypertension as a complication in patients of sickle cell disease.
Methods: Patients aged more than 12 years diagnosed to have sickle cell anaemia on hemoglobin electrophoresis during the study period (total- 94; 54 SS and 40 AS) were enrolled as cases. Sixty four age/sex matched healthy close relatives of the cases with ‘AA’ Hb electrophoresis pattern doubled up as controls. Participants were thoroughly assessed with complete history, examination, haematological and biochemical tests, X-ray chest, ECG, pulmonary function tests and 2D-echocardiography and Doppler studies and data analysed.
Results: Pulmonary hypertension was observed in a total of five SS cases (5/54, 9.26%). No participant from the AS group or the control group developed pulmonary hypertension. It was more common in females (3/23, 13%) than males (2/26, 7.7%). The mean age of the SS cases with PH (32±5.15 years) was found to be significantly higher than that of the SS cases without PH (24.2±6.21 years) (p<0.01).
Conclusions: The incidence of PH in sickle cell disease was relatively lower as compared to western countries and was found to affect females and older individuals more. Larger community-based studies are recommended for corroboration.
References
Wang WC. Sickle cell anemia and other sickling syndromes. In: JP Greer, eds., Wintrobe's Clinical Hematology, 12th Ed. Philadelphia; Lippincott Williams and Wilkins:1038-1082.
Kar BC, Devi S, Dash KC, Das M. The Sickle cell gene is widespread in India. Trans Roy Soc Trop Med and Hyg. 1987;81(2):273-5.
Rao VR. Genetics and epidemiology of sickle cell anemia in India. Indian J Med Sci. 1988;42:218-22.
Shukla RN, Solanki BR. Sickle Cell Trait in Central India. Lancet. 1958;1(7015):297-8.
Yater WM, Hansmann GH. Sickle cell anemia: a new cause of cor pulmonale. Am J Med Sci. 1936;191:474-84.
Abedeji MO, Cespedes J, Allen K, Subramony C, Hughson MD. Pulmonary thrombotic arteriopathy in patients with sickle cell disease. Arch. Pathol Lab Med. 2001 Nov;125(11):1436-41.
Powars D, Weidman J, Odom-Maryon T, Niland J, Johnson C. Sickle cell chronic lung disease: Prior morbidity and the risk of pulmonary failure. Med. 1988;67:166-76.
Sutton LL, Castro O, Cross DJ, Spencer JE, Lewis JF. Pulmonary Hypertension in sickle cell disease. Am J Cardiol. 1994;74:626-8.
Abubakr SE, Girgis R, Swerdlow P. Pulmonary Hypertension in sickle cell disease. Am J Respir Crit Care Med. 1999;160:A144.
Castro O, Hoque M, Brown BD. Pulmonary Hypertension in sickle cell disease: cardiac catheterization results and survival. Blood. 2003 Feb 15;101(4):1257-61.
Braunwald E. Heart Disease. 6th edition. Philadelphia: Saunders; 2001:100,1908,1919-1921, 1924,1925.
Schamroth Leo. An introduction to electrocardiography. 6th edition. Blackwell Science ltd; 1988:83-86.
Anderson G, Reid L, Simon G. The radiographic appearances in primary and in thromboembolic pulmonary hypertension. Clini Radiol. 24:113:1973.
Kar BC. Sickle cell disease in India. J Assocof Physic India. 1991,39:954-60.
Kar BC, Satapathy R, Kulozik A, Kulozik M, Sirr S, Sergeant B, et al. Sickle cell disease in Orissa state, India. Lancet. 1986,2:1198-201.
Kaur M, Das GP, Verma IC. Sickle cell trait and disease among tribal communities in Orissa, Madhya Pradesh and Kerala. Ind J Med Res. 1997. 105:111-6.
Abhyankar D, Zanwar S, Mundada A, Oak S, Vora A, Khandait V, et al. Arterial blood gas analysis in acute chest syndrome and vaso-occlusive of sickle cell disease. Ind J Hematol Blood Transfusion. 2000 June;18(2):25-8.
Collins FS, Orringer EP. Pulmonary hypertension and cor pulmonale in the sickle haemoglobinopathies. Am J Med. 1982;73:814-21.