Aims: N-terminal pro brain natriuretic peptide (NT-proBNP) is an important biomarker in congestive heart failure. This has also been confirmed in congenital heart disease. However, its clinical value in patients with different types of Fontan circulation remains questionable. Methods and results: We prospectively analysed 124 patients with various types of Fontan surgery between October 2006 and February 2011. We included 49 patients with older Fontan modification [atriopulmonary connection (APC) and atrioventricular connection (AVC)] and 75 patients with total cavopulmonary connection (TCPC). The NT-proBNP levels of patients with APC/AVC were significantly higher than in patients with TCPC (P < 0.001), even after accounting for sex, age, ventricular function, atrioventricular regurgitation, ventricular morphology, and arrhythmia (P = 0.035). Levels of NT-proBNP positively correlated with atrioventricular valve regurgitation (r = 0.29, P = 0.013) and ventricular dysfunction (r = 0.23, P = 0.052) only in patients with TCPC, but not in patients with APC or AVC (r = 0.01, P = 0.509 and r = 0.10, P = 0.493, respectively). Conclusion: Levels of NT-pro BNP are related to the type of Fontan circulation. The older types (APC/AVC) that involve more atrial tissue in the systemic venous pathway show higher NT-proBNP levels independently of their cardiac status. Their NT-proBNP levels should be interpreted with care.

Anomalous origin of coronary arteries from the pulmonary artery (ACAPA) are rare but clinically significant condition with high mortality if left untreated. Even more rarely, ACAPA is associated with other congenital heart defects. From 1974 to 2024, 120 patients with anomalous coronary arteries connected to the pulmonary artery were retrospectively analyzed. Medical records including surgical operative notes and angiography protocols were screened for any other cardiac and extracardiac defects. Anomalous left coronary artery connected to the pulmonary artery (ALCAPA) was present in 103 patients, anomalous right coronary artery connected to the pulmonary artery (ARCAPA) in 6, anomalous circumflex coronary artery connected to the pulmonary artery (ACXPA) in 7, anomalous left anterior descending coronary artery connected to the pulmonary artery (ALADPA) in 2, and anomalous single coronary artery connected to the pulmonary artery (ASCAPA) in 2 patients. Anomalous origin of the coronary artery from the pulmonary arteries was associated with other congenital heart defects in 16 patients (13%) and with extracardiac anomalies in 10 patients (8%). Most associated cardiac anomalies were left-sided obstructive defects or shunt-lesions. Patients with ACAPA and associated cardiac defects had poorer perioperative survival. A precise diagnosis of coronary anatomy is crucial for preoperative planning and the success of the surgery of patients with congenital heart defects. In particular, for patients with a challenging postoperative course, an anomalous coronary artery originating from the pulmonary artery should be considered.