diagnosed using tool in guideline Hypertension Personal Medical History N-terminal Pro-brain Natriuretic Peptide Test Physical Examination Pulmonary Hypertension Pulmonary Arterial Hypertension Simonneau et al 2013 J Am Coll Cardiol; Ataga et al. Few but definitions not specific enough Multifactorial, including chronic thromboembolism, splenectomy, high cardiac output, left-heart disease, and hyperviscosity; the role of an inactivation of nitric oxide by free plasma hemoglobin due to chronic hemolysis is controversial. Pulmonary hypertension can be secondary to known cardiac, pulmonary, or hepatic disease. If no cause is identifiable, the term primary pulmonary hypertension is used. Increased Blood Pressure in Blood Vessels of Lungs An elevation of mean pulmonary arterial systolic pressure (mPASP) (>20 mmHg at rest or >30 mmHg with exercise). The 6th World Symposium on PH reduced it from >25mmHg to >20mmHg for at rest, but did not adjust the definition for PH with exercise. Br J Haematol. 2006 Jul;134(1):109-15. Recommendations for the Management of Sickle Cell Disease in South Africa National Guideline for the Control and Management of Sickle Cell Disease Management of Sickle Cell Disease - The United Republic of Tanzania Standards for Clinical Care of Adults with Sickle Cell Disease in the UK Sickle Cell Disease: The Clinical Care Guidelines of the Sickle Cell Unit Consensus Statement on the Care of Patients with Sickle Cell Disease in Canada Sickle Cell Disease in Children, Standards and Guidelines for Clinic Care Evidence-Based Management of Sickle Cell Disease Management of Children with Sickle Cell Disease in Europe: Current Situation and Future Perspectives ENERCA Clinical Recommendations for Disease Management and Prevention of Complications of Sickle Cell Disease in Children