PMID- 19942562
OWN - NLM
STAT- MEDLINE
DCOM- 20100218
LR  - 20180815
IS  - 2241-5955 (Electronic)
IS  - 1109-9666 (Linking)
VI  - 50
IP  - 6
DP  - 2009 Nov-Dec
TI  - Pulmonary arterial hypertension: many years' experience and modern approach to a 
      malignant disease in a pulmonary hypertension centre.
PG  - 484-92
AB  - INTRODUCTION: The aim of this study was to record the results from a modern 
      diagnostic and therapeutic approach to patients with pulmonary arterial 
      hypertension. METHODS: We studied the clinical characteristics and the treatment 
      of 69 patients (50 women, 72.5%), aged 44 +/- 17 years, who were diagnosed with 
      pulmonary hypertension (World Health Organisation categories I, IV and V). The 
      patients' outcomes were recorded over 14 years' operation of our Pulmonary 
      Hypertension Unit. RESULTS: Twenty-seven patients (39.1%) suffered from 
      idiopathic pulmonary hypertension, 12 (17.4%) from thromboembolic obstructive 
      disease, 14 (20.3%) from congenital heart diseases, 11 (15.9%) from connective 
      tissue diseases, 3 (4.3%) from portal hypertension, 2 (2.9%) from sarcoidosis, 
      and 1 (1.4%) from pulmonary veno-occlusive disease. Six patients (8.7%) were in 
      New York Heart Association (NYHA) functional class I, 28 (40.6%) were in class 
      II, 30 (43.5%) were in class III, and 5 (7.2%) were in class IV. Thirty-four 
      patients completed a 6-minute walk test and covered a mean distance of 352 +/- 
      137 m. N-terminal pro-brain natriuretic peptide (NT-proBNP) levels were measured 
      in 18 patients, with a mean value of 1665 +/- 1935 pg/ml. A vasoreactivity test 
      in 41 patients had a positive response in 10 (24.4%). Twenty-six patients (37.7%) 
      were treated with combination therapy, whereas 16 (23.2%) either did not comply 
      with or were not given specific medication. The mortality over a mean follow-up 
      period of 5 +/- 4 years was 26%, significantly lower than that reported before 
      the introduction of new drugs. An advanced NYHA class was an independent 
      predictor of mortality (p=0.004), while elevated NT-proBNP levels were also 
      associated with poor survival (p=0.013). CONCLUSIONS: Pulmonary arterial 
      hypertension is a severe disease that leads to right heart failure and death. 
      Despite the latest advances, vigilance and continuous investigation are needed in 
      order to achieve a prompt diagnosis and the most suitable treatment.
FAU - Karyofillis, Panagiotis
AU  - Karyofillis P
AD  - 1st Department of Cardiology, Onassis Cardiac Surgery Centre, Athens, Greece. 
      pakar768@yahoo.gr
FAU - Manginas, Athanasios
AU  - Manginas A
FAU - Thomopoulou, Sofia
AU  - Thomopoulou S
FAU - Balanos, Dimitrios
AU  - Balanos D
FAU - Spargias, Konstantinos
AU  - Spargias K
FAU - Cokkinos, Dennis V
AU  - Cokkinos DV
LA  - eng
PT  - Journal Article
PL  - Netherlands
TA  - Hellenic J Cardiol
JT  - Hellenic journal of cardiology : HJC = Hellenike kardiologike epitheorese
JID - 101257381
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Aged
MH  - Child
MH  - Female
MH  - Humans
MH  - *Hypertension, Pulmonary/diagnosis/drug therapy/physiopathology
MH  - Male
MH  - Middle Aged
MH  - Young Adult
EDAT- 2009/11/28 06:00
MHDA- 2010/02/19 06:00
CRDT- 2009/11/28 06:00
PHST- 2009/11/28 06:00 [entrez]
PHST- 2009/11/28 06:00 [pubmed]
PHST- 2010/02/19 06:00 [medline]
PST - ppublish
SO  - Hellenic J Cardiol. 2009 Nov-Dec;50(6):484-92.