Pulmonary hypertension ups mortality risk even after repair of cardiac defect

Pulmonary hypertension (PH) occurs rarely after correction of cardiac defects, but is nevertheless a significant mortality risk factor, a new study reports.

The retrospective study included 825 patients (median age, 23 years) with closed, isolated simple cardiac defects, such as atrial septal defect, ventricular septal defect and persistent atrial ducts. The primary study outcome was the risk of PH. Participants with genetic defects were excluded from the analysis.

Over a median follow-up of 15.9 years from defect closure, 25 participants developed PH, resulting in an incidence rate of 3.0 percent. These patients tended to be older and were more likely to have undergone defect closure later in life.

Age emerged as an important mediating factor. The prevalence of PH was 0.7 percent in participants <20 years of age. This shot up to 8.8 percent and 11.9 percent by the fifth and sixth decades of life, respectively. This was confirmed by logistic regression analysis, which showed that age at follow-up was a significant risk factor (odds ratio per additional year, 1.07, 95 percent confidence interval [CI], 1.02–1.11; p=0.003).

Fourteen patients died over the course of the study and the mortality rate was higher in the PH group (20 percent vs 1 percent). Age- and gender-adjusted Cox analysis verified the impact of PH on death risk (hazard ratio per year, 1.015, 95 percent CI, 1.01–1.072; p=0.003).

“Physicians should be aware of the increased risk of PH and its association with heart failure symptoms and increased mortality in this setting. A low threshold for specialized workup is recommended especially in the elderly to ascertain the diagnosis of PH, which has important prognostic and clinical implications,” researchers said.