Clinical outcome and survival in 30 pulmonary hypertension patients with high severity indices and advanced functional class

BACKGROUND: Pulmonary hypertension (PH) is an idiopathic or secondary disorder associated with many systemic illnesses. Long-term survival in PH depends on the severity and functional class. Several new drugs are now available to treat PH, but their impact on clinical outcome and survival are not well established. OBJECTIVES: Evaluate severity parameters and the impact of current recommended therapy on survival in PH. DESIGN: Cross-sectional. SETTINGS: Tertiary care center. PATIENTS AND METHODS: The study included adult patients who had undergone right heart catheterization since 2012 and were diagnosed with pulmonary hypertension. Survival was recorded after 6 years, at the end of the study. Nine severity variables for PH were assessed including right ventricular size by echocardiogram and pulmonary artery diameter (PA diameter) and the ratio of pulmonary artery diameter to ascending aorta diameter (PA/Ao ratio) by CT. MAIN OUTCOME MEASURES: Evaluation of severity parameters. SAMPLE SIZE: 30 patients. RESULTS: Twenty-five patients were positive for 8/9 severity parameters. Eight of 30 (26.6%) patients died. In nonsurvivors, right ventricular size was increased by 25% (P=.427), pulmonary vascular resistance increased by 29.4% in nonsurvivors (P=.302), the 6-minute walk distance decreased by 21% (P=.875), median brain natriuretic peptide increased by 96% (P=.890), median GGT and alkaline phosphatase were 3 times higher in nonsurvivors (P=.893 and P=.047, respectively) and PA/Ao was nonsignificantly decreased in nonsurvivors (P=.373), Survival was decreased by a median of 2.3 years in nonsurvivors. CONCLUSION: Our study identified a subgroup of PH patients with NYHA functional class III and above with worsening severity indicators who were labeled as a high-risk group. These patients showed continuous deterioration in their clinical status despite escalation of therapy with current guidelines. We recommend these high-risk group patients be referred for early lung transplantation. LIMITATIONS: Low sample size and only a single center. Needs confirmation with a larger multicenter trial. CONFLICT OF INTEREST: None.