• Pulmonary vascular disease
• Secondary pulmonary hypertension
• Congenital heart disease
• Transposition of the great arteries
• Epidemiology

Pulmonary hypertension (PH) represents a severe problem affecting patients' wellbeing and is considered to occur in approximately 5.7% of patients with congenital heart disease (CHD) following corrective surgery.1 Particularly prone to the development of PH are patients with post-tricuspid shunt lesions, with an even higher incidence.

Because surgical strategy for transposition of the great arteries (TGA) has changed from atrial switch to anatomical correction, survival rates after arterial switch operation (ASO) have become excellent, with an early mortality of only 1% in the recent era2 and survival rates at 25 years of approximately 97%.3 However, morbidity and late sequelae remain a problem, leading to a freedom from reoperation of 82% at 23 years.2 Problems such as aortic root dilatation, aortic stenosis/recoarctation, outflow tract obstruction, pulmonary or supravalvular pulmonary stenosis and arrhythmia may occur, especially in longer term follow-up.2–4 Given the nature of the operation with reimplantation of the coronary arteries at neonatal age, particular attention is paid to the integrity of the coronary artery perfusion. Despite initial concerns, the incidence of relevant, symptomatic coronary artery disease is reported to be relatively low, although the reported prevalence varies between 2.1% and 11.3%.4 5

In this journal, Zijlstra et al …