11.07.2015 Views

Greg Calligaro Definition and classification of pulmonary hypertension

Greg Calligaro Definition and classification of pulmonary hypertension

Greg Calligaro Definition and classification of pulmonary hypertension

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Group 1: PAH associated with haemolysis• Sickle cell disease (SCD)most important sub-group• Prevalence <strong>of</strong> PH in patientswith sickle cell diseasereported on echo studies as±30%• Much lower on RHC: ±5%• Free Hb scavenges NO –induces state <strong>of</strong> resistanceto NO bioactivity• Also associated withplexiform lesionsGladwin MT, NEJM, 2004.

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