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Great advances in medicine, in particular surgical and interventional advances, for the treatment of cyanotic congenital heart disease (CCHD) and early detection of large septal defects, have turned cyanotic children into acyanotic survivors and revolutionised survival of these patients.w1–6 Persistence of cyanosis is the exception in patients with unrepaired or palliated CCHD (eg, tetralogy of Fallot, transposition complexes, double outlet right ventricle, univentricular hearts) or undetected septal defects with Eisenmenger physiology in western countries or in immigrants from countries where surgical repair of congenital heart disease (CHD) is not available or not affordable.
Understanding the underlying anatomy, pathology and pathophysiology is fundamental for the optimal management of patients with CCHD to avoid errors and mistakes. Care for children with CCHD is the domain of paediatric cardiologists, who have to be fully integrated into a multidisciplinary team of experts caring for these cyanotic patients after their graduation from paediatric cardiology and after proper transition and transfer to adult care.w7 w8
This article addresses challenges in the management of cyanotic patients surviving into adulthood. Longevity of patients with CCHD is better than assumed, and survival into adulthood is not uncommon.1–3 w9 w10 The complication rate is usually low during the first 30 years, but complications and the attrition rate start in the third or fourth decades of their life. Complications and death can be triggered by inappropriate therapeutic measures. The most common mistakes and avoidable complications are based on poor appreciation of the complex pathophysiology of this heterogeneous population with a multisystem disorder, on misconceptions, and on translation of practice guidelines from patients with other disease to patients with CCHD. It is also essential to not miss therapeutic opportunities to improve the patient's symptoms and quality of life and to improve outcomes. It can be a challenge to find the …
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