title: Improved survival in patients with inoperable chronic thromboembolic pulmonary hypertension.
abstract: Patients with inoperable chronic thromboembolic pulmonary hypertension (Inop-CTEPH) treated with conventional therapy have a poor survival. We compare the 3-year survival between those treated with conventional therapy and those treated with conventional therapy and a combination of novel drugs. We also evaluate the clinical course. A total of 34 Inop-CTEPH consecutive patients were evaluated from 1991 to 2009 including right heart catheterization (RHC) and perfusion lung scan (PLS): 7 underwent surgical treatment while 27 were confirmed inoperable. Of these 27 patients, 12 evaluated from 1991 to 2003 (Group 1) were treated with conventional therapy and 15 evaluated from 2004 to 2009 (Group 2) were treated with conventional and novel therapies. At baseline, no group difference emerged at RHC. Based on clinical course, novel drugs and oxygen supplementation were given to patients of Group 2. Seven of these who had worse clinical course repeated RHC and four of them also PLS during therapy. Those without repeat RHC had baseline pulmonary artery mean pressure and brain natriuretic peptide (NT-proBNP) lower and mixed venous saturation (SvO2) and exercise test higher (p = 0.022, 0.015, 0.044 and 0.003, respectively). During therapy, those with repeat RHC had total pulmonary vascular resistance reduced (p = 0.012), base excess increased (p = 0.002) and significant redistribution of pulmonary blood flow at PLS. At the 3-year follow-up, survival was 86% in Group 2 and 31% in Group 1 (p = 0.031). In Inop-CTEPH patients, the clinical course may help to select drugs and the level of oxygen supply that can improve hemodynamics, gas exchange and long-term survival.
authors.affiliation: Fondazione CNR/Regione Toscana "G. Monasterio", Via G. Moruzzi 1, 56124, Pisa, Italy. marini@ifc.cnr.it
authors.forename: Carlo
authors.initials: C
authors.lastname: Marini
authors.forename: Bruno
authors.initials: B
authors.lastname: Formichi
authors.forename: Carolina
authors.initials: C
authors.lastname: Bauleo
authors.forename: Claudio
authors.initials: C
authors.lastname: Michelassi
authors.forename: Renato
authors.initials: R
authors.lastname: Prediletto
authors.forename: Giosuè
authors.initials: G
authors.lastname: Catapano
authors.forename: Dario
authors.initials: D
authors.lastname: Genovesi
authors.forename: Simonetta
authors.initials: S
authors.lastname: Monti
authors.forename: Francesca
authors.initials: F
authors.lastname: Mannucci
authors.forename: Carlo
authors.initials: C
authors.lastname: Giuntini
mesh_terms: D000136:Acid-Base Equilibrium; D000368:Aged; D000959:Antihypertensive Agents / Q000627:therapeutic use; D001784:Blood Gas Analysis; D000077300:Bosentan; D002302:Cardiac Output; D002908:Chronic Disease; D004359:Drug Therapy, Combination; D005080:Exercise Test; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy*; D007555:Isoxazoles / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D010101:Oxygen Consumption; D010102:Oxygen Inhalation Therapy; D010879:Piperazines / Q000627:therapeutic use; D011687:Purines / Q000627:therapeutic use; D012720:Severity of Illness Index; D000068677:Sildenafil Citrate; D013449:Sulfonamides / Q000627:therapeutic use; D013450:Sulfones / Q000627:therapeutic use; D015996:Survival Rate; D013876:Thiophenes / Q000627:therapeutic use; D013923:Thromboembolism / Q000150:complications* / Q000175:diagnosis / Q000628:therapy*; D016896:Treatment Outcome; D014655:Vascular Resistance; D014665:Vasodilator Agents / Q000627:therapeutic use
chemical_list: D000959:Antihypertensive Agents; D007555:Isoxazoles; D010879:Piperazines; D011687:Purines; D013449:Sulfonamides; D013450:Sulfones; D013876:Thiophenes; D014665:Vasodilator Agents; D000068677:Sildenafil Citrate; C106276:sitaxsentan; D000077300:Bosentan