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The extraordinary advances and dramatic successes of pediatric cardiology and surgery over the past 50 years have witnessed an increase in children born with congenital heart disease (CHD) who survive into adulthood. This success in survival has created a new population of patients of adults with congenital heart disease (ACHD). The number of ACHD will very likely continue to increase in the near future.1 The 32nd Bethesda Conference had estimated that there were approximately 800,000 adults with CHD in the United States in the year 20002; in the United Kingdom alone there are currently approximately 250,000 ACHD patients.3

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Since about 0.8 percent of live births are complicated by cardiovascular malformations (disregarding the two most common congenital cardiac anomalies: functionally normal congenital bicuspid aortic valve and mitral valve prolapse), approximately 2800 adults per million population will have some form of CHD, with half of them having moderate- or high-complexity defects.2 These patients are at significant risk of early mortality from complications of future treatment including reoperation, especially if undertaken in facilities or by physicians not resourced or trained in their care. At present, there is shortage of facilities dedicated to the care of adults with CHD.4 Care givers and ACHD should have been taught in adolescence about their condition, their future outlook, and the possibility of further surgery and complications, as appropriate. They should be advised about their responsibility in ensuring self-care and professional surveillance. Copies of operative reports should accompany patients being transferred for adult care, along with other key documents from pediatric medical records. Recommendations on transfer of care, organization of ACHD service network, infrastructural requirements, involvement of multidisciplinary teams and their training/certification have been dealt with in detail in the ESC5 and ACC/AHA6 taskforce guidelines. A well-working network of specialist centers with general adult care is of paramount importance since, although complex defects can be easily recognized and assigned to high-level care, even simple defects may still require specialist care under certain circumstances [i.e., atrial septal defects (ASD) in the setting of pulmonary hypertension (PAH)].

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The subsequent sections of this chapter deal with clinical management of ACHD patients, with special emphasis on issues unique to each lesion, in line with the recently published ESC-20107 and ACC/AHA-20086 taskforce guidelines.

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Clinical Evaluation

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A detailed history from an ACHD patient aims at analyzing present and past symptoms including any significant intercurrent event. Changes in lifestyle should be recorded. Clinical examination during initial and follow-up visits should lay special emphasis on any changes in oxygen saturations, auscultatory findings, blood pressure measurements or signs of heart failure development. A 12-lead electrocardiography (ECG) and pulse oximetry should be part of the examination. Chest x-ray is performed if the clinical situation demands, and provides useful information on changes in heart size, configuration, and pulmonary vascularity.

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Noninvasive investigative modalities like echocardiography, cardiac MRI (CMR) are being increasingly used ...

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