2014
An Official American Thoracic Society Clinical Practice Guideline: Diagnosis, Risk Stratification, and Management of Pulmonary Hypertension of Sickle Cell Disease
Klings E, Machado R, Barst R, Morris C, Mubarak K, Gordeuk V, Kato G, Ataga K, Gibbs J, Castro O, Rosenzweig E, Sood N, Hsu L, Wilson K, Telen M, Decastro L, Krishnamurti L, Steinberg M, Badesch D, Gladwin M. An Official American Thoracic Society Clinical Practice Guideline: Diagnosis, Risk Stratification, and Management of Pulmonary Hypertension of Sickle Cell Disease. American Journal Of Respiratory And Critical Care Medicine 2014, 189: 727-740. PMID: 24628312, PMCID: PMC3983842, DOI: 10.1164/rccm.201401-0065st.Peer-Reviewed Original ResearchMeSH KeywordsAdultAnemia, Sickle CellAnticoagulantsAntihypertensive AgentsAntisickling AgentsCardiac CatheterizationDecision Support TechniquesEchocardiography, DopplerErythrocyte TransfusionHumansHydroxyureaHypertension, PulmonaryPhosphodiesterase 5 InhibitorsRisk AssessmentSeverity of Illness IndexConceptsRight heart catheterizationTricuspid regurgitant velocitySickle cell diseasePulmonary vascular resistancePulmonary hypertensionManagement of patientsEvidence-based recommendationsStrong recommendationsWedge pressureVascular resistanceRisk stratificationWeak recommendationCell diseaseMortality riskOfficial American Thoracic Society Clinical Practice GuidelineSerum N-terminal pro-brain natriuretic peptide (NT-proBNP) levelsN-terminal pro-brain natriuretic peptide levelsElevated pulmonary artery wedge pressureNormal pulmonary capillary wedge pressurePro-brain natriuretic peptide levelsElevated tricuspid regurgitant velocityLow pulmonary vascular resistanceNT-pro-BNP levelsPhosphodiesterase-5 inhibitor therapyPulmonary artery wedge pressure
2009
Safety and Efficacy of Sildenafil Therapy for Doppler-Defined Pulmonary Hypertension in Patients with Sickle Cell Disease: Preliminary Results of the Walk-PHaSST Clinical Trial.
Machado R, Barst R, Yovetich N, Hassell K, Goldsmith J, Woolson R, Gordeuk V, Gibbs S, Little J, Kato G, Schraufnagel D, Krishnamurti L, Girgis R, Morris C, Berman-Rosenzweig E, Badesch D, Waclawiw M, Gladwin M. Safety and Efficacy of Sildenafil Therapy for Doppler-Defined Pulmonary Hypertension in Patients with Sickle Cell Disease: Preliminary Results of the Walk-PHaSST Clinical Trial. Blood 2009, 114: 571. DOI: 10.1182/blood.v114.22.571.571.Peer-Reviewed Original ResearchSerious adverse eventsSickle cell diseaseRight heart catheterizationBrief Pain InventoryPulmonary hypertensionCell diseaseAdverse eventsArterial pressureMean pulmonary arterial pressureSix-minute walk distanceApparent safety issuesDose of sildenafilSecondary endpoint analysisWeeks of sildenafilPulmonary vascular resistancePulmonary arterial pressureTransthoracic Doppler echocardiographySystemic arterial pressurePremature study terminationSingle test doseSignificant differencesSignificant increaseSickle cell anemiaOral sildenafilSildenafil dosing