[Feasibility of cardiopulmonary rehabilitation in patients with idiopathic pulmonary arterial hypertension treated with intravenous prostacyclin infusion therapy].
OBJECTIVE
To evaluate cardiopulmonary rehabilitation in patients with idiopathic pulmonary arterial hypertension who had severe heart failure.


METHODS
The subjects comprised 11 men and 13 women aged 5 to 37 years old with idiopathic pulmonary arterial hypertension, who received cardiopulmonary rehabilitation following the start of continuous intravenous prostacyclin administration between January 1999 and September 2003. Fifteen patients were categorized in New York Heart Association (NYHA) functional class-III and 9 were class-IV on admission. Patients received cardiopulmonary rehabilitation consisting of breathing exercise, training of upper extremity muscles, gait training, bicycle ergometer training, and treadmill walking for 30 to 60 min per day, 5 days a week. Cardiothoracic ratio, NYHA class, heart rate, pulse oximeter saturation, plasma levels of human atrial natriuretic peptide and brain natriuretic peptide, tricuspid regurgitation, and right ventricular myocardial index (RV Tei index) were evaluated by echocardiography, lower extremity muscle strength, ambulation ability, Barthel index, and 6-minute walking distance at the beginning and end of cardiopulmonary rehabilitation.


RESULTS
The average period of cardiopulmonary rehabilitation was 6.7 weeks. There was no deterioration in cardiothoracic ratio, human atrial natriuretic peptide, brain natriuretic peptide levels, tricuspid regurgitation, RV Tei index and pulse oximeter saturation. The results also showed decreased heart rate at rest (p = 0.007), and improved NYHA class (p = 0.010), lower extremity strength (p < 0.001), ambulation ability (p < 0.001), Barthel index (p < 0.001), and 6-minute walking distance (p = 0.001).


CONCLUSIONS
Cardiopulmonary rehabilitation is safe and effective for idiopathic pulmonary arterial hypertension patients in NYHA class-III and IV during intravenous prostacyclin infusion without deterioration of cardiac functions, despite the conventional contraindication.
Uchi
Masayuki
M
Department of Physical Medicine and Rehabilitation, School of Medicine, Faculty of Medicine, Toho University, Tokyo. masayuki@med.toho-u.ac.jp
Saji
Tsutomu
T
Harada
Takashi
T
D000203:Activities of Daily Living; D000293:Adolescent; D000328:Adult; D000959:Antihypertensive Agents / Q000008:administration & dosage*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D011464:Epoprostenol / Q000008:administration & dosage*; D017079:Exercise Tolerance; D005260:Female; D006333:Heart Failure / Q000150:complications; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000188:drug therapy* / Q000534:rehabilitation*; D007262:Infusions, Intravenous; D008297:Male
D000959:Antihypertensive Agents; D011464:Epoprostenol