title: Eisenmenger syndrome in the adult--experience with new drugs for the treatment of pulmonary hypertension.
abstract: BACKGROUND
  The last few years have seen significant progress in the treatment of advanced pulmonary arterial hypertension (PAH). The efficacy of new drugs has been proved mainly in idiopathic PAH or PAH associated with connective tissue diseases. As the pathophysiologic patterns are similar, it is reasonable to use these drugs also in Eisenmenger syndrome or in other congenital heart defects with PAH related to initial high pulmonary flow.
  
  
  OBJECTIVE
  To evaluate our early experience with new drugs for PAH in patients with Eisenmenger syndrome.
  
  
  METHODS
  A retrospective study of five patients, aged 28 to 51 years (39.6 +/- 9.94), four female, with Eisenmenger syndrome due to atrial septal defect (n = 2), patent ductus arteriosus (n = 2) or ventricular septal defect (n = 1), who began therapy with iloprost (n = 4, later associated with sildenafil in one patient) and bosentan (n = 1), between April 2001 and May 2003. The existence of severe and fixed PAH, with predominant right-to-left shunt, was confirmed by hemodynamic study in all cases. The patients were evaluated by clinical examination, Doppler echocardiography and the six-minute walk test before treatment and throughout follow-up (9 to 34 months, 19.8 +/- 9.04). Before treatment two patients were in NYHA class III and three in class III with periods in class IV. By Doppler echocardiography the right ventricle-right atrium (RV-RA) gradient was 74 to 111 mmHg (90.6 +/- 15.73) and the Tei index was 0.53 to 2.05 (1.13 +/- 0.62). In the six-minute walk test the distance was 214 to 500 meters (296.8 +/- 115.27).
  
  
  RESULTS
  All patients improved clinically, though three are still in class III. One patient is in class II and one patient died. At the latest evaluation the RV-RA gradient was 60 to 112 mmHg (84.8 +/- 19.11) and the Tei index was 0.5 to 1.33 (0.85 +/- 80.32). In the six-minute walk test a net increase in the distance covered was evident: 376 to 520 meters (420 +/- 57.89). The treatment was well tolerated in all cases, without serious adverse effects.
  
  
  CONCLUSIONS
  Though the number of patients was small, our initial experience with the new specific drugs for PAH in Eisenmenger syndrome showed promising results, with clinical and functional improvement and without adverse effects.
authors.affiliation: Serviço de Cardiologia, Hospital de Santa Marta, Lisboa, Portugal. afag@netcabo.pt
authors.forename: Ana Figueiredo
authors.initials: AF
authors.lastname: Agapito
authors.forename: Lídia
authors.initials: L
authors.lastname: Sousa
authors.forename: José Alberto
authors.initials: JA
authors.lastname: Oliveira
authors.forename: Joana
authors.initials: J
authors.lastname: Feliciano
authors.forename: Duarte
authors.initials: D
authors.lastname: Cacela
authors.forename: Jorge
authors.initials: J
authors.lastname: Quininha
mesh_terms: D000328:Adult; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D000077300:Bosentan; D004541:Eisenmenger Complex / Q000188:drug therapy*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy*; D016285:Iloprost / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D013449:Sulfonamides / Q000627:therapeutic use*; D014665:Vasodilator Agents / Q000627:therapeutic use*
chemical_list: D000959:Antihypertensive Agents; D013449:Sulfonamides; D014665:Vasodilator Agents; D016285:Iloprost; D000077300:Bosentan