Neglected Pulmonary Arterial Hypertension in Sickle Cell Anaemia during Prenatal Care

  • Minoodokht Bavarsad Karimi Department of Gynecology, Iran University of Medical Sciences, Tehran, Iran


Pregnancy, pulmonary hypertension, sickle cell anaemia, prenatal care


Pulmonary arterial hypertension (PAH) is one of the main complications of sickle cell disease (SCD) and imparts significant risk during pregnancy. Here, we report the outcome of undetected PAH in a pregnant woman with SCD. The patient presented with severe progressive dyspnoea with echocardiographic findings of high pressure in the pulmonary artery in the 37th week of pregnancy. Despite an emergency caesarean section, both mother and neonate died. Regular cardiovascular check-up is essential for SCD patients and careful prenatal care should include cardiovascular evaluation. PAH during pregnancy is associated with high mortality and morbidity. As there is no proof that new advanced therapies decrease the risks, early diagnosis in pregnant patients with underlying disease, like sickle cell anaemia, is essential and termination of pregnancy should be considered.



  • Rees DC, Williams TN, Gladwin MT. Sickle-cell disease. Lancet 2010;376(9757):2018–2031.

  • Boulet SL, Okoroh EM, Azonobi I, Grant A, Hooper WC. Sickle cell disease in pregnancy: maternal complications in a Medicaid-enrolled population. Matern Child Health J 2013;17(2):200–207.

  • Chakravarty EF, Khanna D, Chung L. Pregnancy outcomes in systemic sclerosis, primary pulmonary hypertension, and sickle cell disease. Obstet Gynecol 2008;111(4):927.

  • Dauphin-McKenzie N, Gilles JM, Jacques E, Harrington T. Sickle cell anemia in the female patient. Obstet Gynecol Surv 2006;61(5):343–352.

  • Fort AT, Morrison JC, Berreras L, Diggs LW, Fish SA. Counseling the patient with sickle cell disease about reproduction: pregnancy outcome does not justify the maternal risk! Am J Obstet Gynecol 1971;111(3):324–327.

  • Hassell K. Pregnancy and sickle cell disease. Hematol Oncol Clin N Am 2005;19(5):903–916.

  • Lin E, Rodgers G, Gladwin M. Hemolytic anemia-associated pulmonary hypertension in sickle cell disease. Curr Hematol Rep 2005;4(2):117–125.

  • Jison ML, Gladwin MT. Hemolytic anemia-associated pulmonary hypertension of sickle cell disease and the nitric oxide/arginine pathway. Am J Respir Crit Care Med 2003;168(1):3–4.

  • Van Enk A, Visschers G, Jansen W, Statius van Eps L. Maternal death due to sickle cell chronic lung disease. Br J Obstet Gynaecol 1992;99(2):162–163.

  • Cunningham F, Leveno K, Bloom S, Spong CY, Dashe J. Williams Obstetrics, 24e. New York: McGraw-Hill Education; 2014.

  • Veille J-C, Hanson R. Left ventricular systolic and diastolic function in pregnant patients with sickle cell disease. Am J Obstet Gynecol 1994;170(1):107–110.

  • Dunn L, Greer R, Flenady V, Kumar S. Sildenafil in pregnancy: a systematic review of maternal tolerance and obstetric and perinatal outcomes. Fetal Diagn Ther 2017;41(2):81–88.
  • Published: 2020-04-08

    Issue: Vol 7 No 6 (view)

    Section: Articles

    How to cite:
    Karimi MB. Neglected Pulmonary Arterial Hypertension in Sickle Cell Anaemia during Prenatal Care. EJCRIM 2020;7 doi:10.12890/2020_001532.