• Jürgen Hörer.
• Department of Pediatric Cardiology and Congenital Heart Disease, Hôpital Marie Lannelongue, Université Paris-Sud, Le Plessis Robinson, France.
• Cardiovasc Diagn Ther. 2018 Dec 1; 8 (6): 754-764.

AbstractToday, more than two thirds of patients with congenital heart disease (CHD) are adults. Cardiac surgery plays an essential role in restoring and maintaining cardiac function, aside from evolving medical treatment and catheter-based interventions. The aim of the present publication was to describe the spectrum of operations performed on adults with CHD (ACHD) by reviewing current literature. Currently, surgery for ACHD is predominantly valve surgery, since valvular pathologies are often either a part of the basic heart defect or develop as sequelae of corrective or palliative surgery. Surgical techniques for valve repair, established in patients with acquired heart disease (non-ACHD), can often be transferred to ACHD. New valve substitutes may help to reduce the number of redo operations. Most of valve operations yield good results in terms of survival and quality of life, with the precondition that the ventricular function is preserved. Heart failure due to end-stage CHD is the most frequent cause of mortality in ACHD. However, surgical treatment by means of mechanical circulatory support (MCS) is still uncommon and the mortality exceeds the one following other operations in ACHD. Currently, different devices are used and new technical developments are in progress. However, there still is no ideal assist device available. Therefore, heart transplantation remains the only valid option for end-stage CHD. Despite higher early mortality following heart transplantation in ACHD compared to non-ACHD, the long-term survival compares favorably to non-ACHD. There is room for improvement by refining the indications, the time of listing, and the perioperative care of ACHD transplant patients. Sudden death is the second most frequent cause of mortality in ACHD. Ventricular tachycardia is the most frequent cause of sudden death followed by coronary artery anomaly. Due to the increasing awareness of physicians and the improved imaging techniques, coronary artery anomalies are coming more into the focus of cardiac surgeons. However, the reported experience is limited and it is currently difficult to provide a standardized and generally applicable recommendation for the indication and the adequate surgical technique. With the increasing age and complexity of ACHD, treatment of rhythm disturbances by surgical ablation, pacemaker or implantable cardioverter defibrillator (ICD) implantation and resynchronisation gains importance. A risk score specifically designed for surgery in ACHD is among the newest developments in predicting the outcome of surgical treatment of ACHD. This evidence-based score, derived from and validated with data from the Society of Thoracic Surgeons Congenital Heart Surgery Database, enables comparison of risk-adjusted performance of the whole spectrum of procedures performed in ACHD and helps in understanding the differences in surgical outcomes. The score is thus a powerful tool for quality control and quality improvement. In conclusion, new developments in surgery for ACHD are currently made with regard to valve surgery, which comprises more than half of all operations in ACHD and in treatment of end-stage CHD, which still yields high mortality and morbidity.

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