- 1. Department of Cardiac Surgery, Guangdong General Hospital, School of Medicine, South China University of Technology, Guangzhou, 510030, P.R.China;
- 2. Department of Cardiac Surgery, Guangdong Provincial Cardiovascular Institute, Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangdong General Hospital, Guangdong Academy of Medical Sciences, Guangzhou, 510100, P.R.China;
CHEN Jimei,
Email: jimei1965@gmail.com
Congenital heart disease refers to the structural or functional abnormality of the macrovascular in the heart or thoracic cavity caused by the failure of the formation of the heart and large blood vessels during the embryonic development or the abnormal closure of the heart or the closure of the channel after birth. In the past few years, a new and broader definition of structural heart disease has been gradually proposed. Structural heart disease narrowly refers to the pathological and physiological changes of the heart caused by abnormal anatomical structures in the heart, including congenital heart disease. A few decades ago, congenital heart disease was considered as a pediatric disease, because most patients with severe lesions rarely survive to adulthood. Due to recent advances in echocardiography, anesthesia, intensive care, percutaneous intervention, especially cardiac surgery in recent decades, the treatment and intervention strategies for congenital heart disease in children have been greatly improved, a fatal defect in childhood can now be successfully repaired or alleviated. Because of these successes, more than 90% of congenital heart disease patients are expected to survive to adulthood, which has led to emerge a new population: adult patients with congenital heart disease. Adult congenital heart disease patients are different from children. Pulmonary hypertension leads to right heart failure and eventually progresses to whole heart failure. The appearance of Eisenmenger syndrome leads to severe cyanosis and worsening of the disease. At present, the continuous development of mechanical assisted circulation support devices and heart or cardiopulmonary transplantation technology has increased the survival rate of end-stage adult congenital heart disease patients with heart failure. The high incidence of cardiovascular events in pregnant patients requires comprehensive multidisciplinary team care and early coordination planning for delivery, including early counseling for pregnancy-related risks, close monitoring of cardiac function and regular scan of fetal assessment. The prenatal and postpartum integrated diagnosis and treatment model and the development of intrauterine treatment technology reduce the incidence of congenital heart disease in adults from the source through fetal intervention. Other complications such as arrhythmia, infective endocarditis, cerebrovascular accidents, and other medical underlying metabolic diseases also challenge future diagnosis and treatment. The incidence and epidemiology of adult congenital heart disease, pulmonary hypertension and end-stage heart failure complications, as well as prenatal and postpartum integrated diagnosis and treatment and intrauterine treatment are summarized in this review.
Citation： TIAN Miao, ZHANG Yong, CHEN Jimei. Current status and future of adult congenital heart disease. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2019, 26(6): 590-600. doi: 10.7507/1007-4848.201810056 Copy
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- 4. Ntiloudi D, Giannakoulas G, Parcharidou D, et al. Adult congenital heart disease: A paradigm of epidemiological change. Int J Cardiol, 2016, 218: 269-274.
- 5. Baggen VJM, Venema E, Zivna R, et al. Development and validation of a risk prediction model in patients with adult congenital heart disease. Int J Cardiol, 2018.
- 6. Awerbach JD, Krasuski RA, Camitta MGW. Coronary disease and modifying cardiovascular risk in adult congenital heart disease patients: should general guidelines apply? Prog Cardiovasc Dis, 2018, 61(3-4): 300-307.
- 7. Bouma BJ, Mulder BJ. Changing landscape of congenital heart disease. Circ Res, 2017, 120(6): 908-922.
- 8. Warnes CA. The adult with congenital heart disease: born to be bad? J Am Coll Cardiol, 2005, 46(1): 1-8.
- 9. Erikssen G, Liestol K, Seem E, et al. Achievements in congenital heart defect surgery: a prospective, 40-year study of 7038 patients. Circulation, 2015, 131(4): 337-346.
- 10. Khairy P, Ionescu-Ittu R, Mackie AS, et al. Changing mortality in congenital heart disease. J Am Coll Cardiol, 2010, 56(14): 1149-1157.
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- 16. Verheugt CL, Uiterwaal CS, Grobbee DE, et al. Long-term prognosis of congenital heart defects: a systematic review. Int J Cardiol, 2008, 131(1): 25-32.
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- 18. Babu-Narayan SV, Diller GP, Gheta RR, et al. Clinical outcomes of surgical pulmonary valve replacement after repair of Tetralogy of Fallot and potential prognostic value of preoperative cardiopulmonary exercise testing. Circulation, 2014, 129(1): 18-27.
- 19. Bokenkamp R, Aguilar E, van der Palen RL, et al. Reoperation for right ventricular outflow tract obstruction after arterial switch operation for transposition of the great arteries and aortic arch obstruction. Eur J Cardiothorac Surg, 2016, 49(5): e91-e96.
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