Prevalence, risk factors and mortality of pulmonary hypertension defined by right heart catheterization in patients with sickle cell disease

Abstract

Evaluation of: Parent F, Bachir D, Inamo J et al. A hemodynamic study of pulmonary hypertension in sickle cell disease. N. Engl. J. Med. 365(1), 44–53 (2011); Fonseca GH, Souza R, Salemi VM, Jardim CV, Gualandro SF. Pulmonary hypertension diagnosed by right heart catheterization in sickle cell disease. Eur. Respir. J. doi:10.1183/09031936.00134410 (2011) (Epub ahead of print).

Three new large screening studies of patients with sickle cell disease for pulmonary hypertension have now been published. Two of these studies included comprehensive right heart catheterization of all patients with an elevation in the estimated pulmonary artery systolic pressure. Together, these studies show that pulmonary hypertension, defined by a mean pulmonary artery pressure greater than or equal to 25 mmHg, occurs in approximately 10% of all adult sickle cell disease patients. Pulmonary hypertension in these studies is associated with hemolytic anemia, renal insufficiency, leg ulcers, increasing age, low exercise capacity and high levels of N-terminal pro-brain natriuretic peptide, but not with the rates of vaso-occlusive crisis or the acute chest syndrome, confirming prior epidemiological studies in adult sickle cell disease patients. The presence of pulmonary hypertension diagnosed by echocardiography or right heart catheterization indicates a significant and independent risk factor for prospective mortality.

Keywords::

• hemoglobin
• hemolytic anemia
• nitric oxide
• pulmonary hypertension
• right heart catheterization
• sickle cell disease
• sudden death

Financial & competing interests disclosure

MT Gladwin receives research support from NIH grants R01HL098032, RO1HL096973 and PO1HL103455, the Institute for Transfusion Medicine and the Hemophilia Center of Western Pennsylvania. The author has no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

No writing assistance was utilized in the production of this manuscript.