Pulmonary arterial hypertension in patients with adult congenital heart disease have high mortality rates. The data reveals that one-third of such patients require emergency admission to hospitals and almost one-fourth of them died. The authors of the study, while emphasizing the requirement of this study wrote, “While risk factors for a worse prognosis are well described in younger cohorts, data regarding older PAH-ACHD patients are scarce. Therefore, the aim of this study is to examine the clinical course of patients with PAH-ACHD over the age of 40 years, and to identify possible risk factors for a worse prognosis.”

All the patients of 40 years and above with Pulmonary arterial hypertension associated with adult congenital heart disease who were under active follow-up between the period January 2005 to December 2018 were included in this study. The data, such as demography, medical history, and surgical history were obtained from the hospital records. All-cause mortality was the primary endpoint of the study.

There was a total of 65 patients included in the study. The mean age was 45.19 ± 6.75 years. 67.7% of females were included. 46 patients had shunt lesions, while 12 patients had pulmonary arterial hypertension that was associated with complex congenital heart defects. 7 patients had segmental pulmonary hypertension that was due to major aortopulmonary collaterals. 13 patients were suffering from Down Syndrome. 16 patients died during the median follow-up period of 4.2 years. The univariate analysis indicates that creatinine, NT-proBNP (log), and prior history of ventricular arrhythmias were all-cause mortality predictors. However, multivariate analysis reveals that creatinine and NT-proBNP were the independent predictors of all-cause mortality.

Source

Maurer SJ, Stöckemann K, Pujol C, Hörer J, Ewert P, Tutarel O. Pulmonary Arterial Hypertension Associated with Congenital Heart Disease in Adults over the Age of 40 Years. J Clin Med. 2020 Dec 17;9(12):4071. doi: 10.3390/jcm9124071. PMID: 33348628; P