title: Challenges in pulmonary hypertension: managing the unexpected.
abstract: The diverse challenges associated with diagnosis and management of patients with pulmonary hypertension are illustrated in this case-based review. Case 1 describes a patient diagnosed with pulmonary arterial hypertension (PAH) with right heart failure and active systemic lupus erythematosus who was effectively treated with an up-front triple combination of PAH therapies and immunosuppressive therapy. In case 2, a diagnosis of pulmonary veno-occlusive disease was reached after a combined approach of clinical suspicion, physical examination, and invasive and noninvasive tests. Cautious PAH therapy and high-dose diuretics provided clinical benefit in this patient and served as a bridge to lung transplantation. These cases highlight the need for ongoing follow-up of patients with PAH, comprising frequent assessment of treatment success and continued diagnostic evaluation.
authors.affiliation: Dept of Respiratory Medicine, Hannover Medical School, Hannover, Germany Olsson.Karen@mh-hannover.de.
authors.forename: Karen M
authors.initials: KM
authors.lastname: Olsson
authors.affiliation: Dept of Specialised, Experimental, and Diagnostic Medicine, University of Bologna, Bologna, Italy.
authors.forename: Massimiliano
authors.initials: M
authors.lastname: Palazzini
mesh_terms: D000328:Adult; D000925:Anticoagulants / Q000627:therapeutic use; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D006328:Cardiac Catheterization; D004232:Diuretics / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D004452:Echocardiography; D005260:Female; D006333:Heart Failure / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D016040:Lung Transplantation; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy* / Q000503:physiopathology; D008875:Middle Aged; D010102:Oxygen Inhalation Therapy; D011668:Pulmonary Veno-Occlusive Disease / Q000150:complications / Q000175:diagnosis / Q000628:therapy*; D012307:Risk Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D000925:Anticoagulants; D000959:Antihypertensive Agents; D004232:Diuretics; D007166:Immunosuppressive Agents