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.


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