Two years of multidisciplinary diagnostic and therapeutic experience in patients with pulmonary arterial hypertension

OBJECTIVES
Information is limited on the prognosis of patients with pulmonary arterial hypertension (PAH) in Turkey. We evaluated our multidisciplinary diagnostic and therapeutic experience in PAH patients.

STUDY DESIGN
The study included 51 patients (32 women, 19 men; mean age 45.4±9.7 years) who were prospectively monitored during a two-year period by the PAH Working Group in our hospital. The diagnoses were as follows: idiopathic/familial PAH (n=9); PAH associated with connective tissue disease (n=16), congenital heart disease (n=11), and with pulmonary veno-occlusive disease (n=1); chronic thromboembolic pulmonary hypertension (n=10), and other causes (n=4). The patients were assessed every three months with clinical examination, six-minute walk test, transthoracic echocardiography, and BNP levels.

RESULTS
The mean pulmonary artery pressure was 54.7±18.8 mmHg. Functional capacity was NYHA class II in nine patients (17.7%), class III in 28 patients (54.9%), and class IV in 14 patients (27.5%). Thirty-seven patients (72.6%) received treatment with specific pharmacological agents, in whom 19 patients (51.4%) required modifications during treatment. Nine patients (17.7%) benefited from treatment with decreases of at least one NYHA class, whereas NYHA class remained unchanged in 25 patients (49%). Seventeen patients (33.3%) exhibited clinical deterioration, of whom 11 died with an overall mortality of 21.6%. Patients who died were all in NYHA class III or IV and significantly differed from those who survived with respect to mean pulmonary artery pressure (72.5±18.7 mmHg vs. 49.8±21.2 mmHg), BNP level at the time of diagnosis (293.8±88.3 pg/ml vs. 141.6±62.1 pg/ml), and six-minute walk distance (123.8±41.3 m vs. 200.7±52.1 m) (p<0.05).

CONCLUSION
Despite relative improvements in the end points over the last two decades, PAH is detected late in the course of the disease, resulting in severe functional and hemodynamic problems in the majority of patients.