Challenges in the care of adult patients with congenital heart defects

his article will endeavour to accomplish four things: to point out some of the more common challenges in the assessment and management of adult patients with congenital heart defects (CHD); to review in general which adult patients with CHD do not need to be seen in specialised adult CHD centres; to review which patients should be seen in such specialised centres; and to review the resources which should thereby be available to them in the expert centres. c TIPS IN THE MANAGEMENT OF ADULT PATIENTS WITH CHD Atrial septal defectsEven the humble atrial septal defect (ASD) has its complexities.First, not all ASDs need to be closed.As a rule, an ASD worth closing should be at least 10 mm in diameter and be associated with clear right ventricular dilation.There is a consensus view that all ASDs meeting these criteria should be closed unless there are reasons not to do so. 1 Moreover, the ASD should be closed when diagnosed, and preferably before the age of 25 years if the goal of treatment is to avoid premature mortality and morbidity.2 The dilemma as to whether to recommend ASD closure in the asymptomatic patient has eased greatly now that device closure of ASDs can be done so successfully and easily.That said, one needs to have access to a skilled interventionalist and modern devices to make this promise relevant.Estimation of the size, the number, and type(s) of atrial defects can be difficult at times.While transthoracic echocardiography (TTE) will usually confirm the diagnosis of an ASD, it will seldom identify whether multiple ASDs are present, an issue that must be evaluated by transoesophageal echocardiography (TOE) before considering device closure of the defect.Anomalous pulmonary venous drainage (APVD) must also be excluded before proceeding to device closure.While most isolated ASDs are of the secundum type, it is important to recognise if a primum (∼7%) or sinus venosus defect (∼8%) is present, since device closure is inappropriate, and since referral to a congenital heart surgeon should be considered.The presence of associated anomalous pulmonary venous drainage should be looked for in all ASDs, usually with TOE or magnetic resonance imaging (MRI) to ensure that all four pulmonary veins drain into the left atrium.The surgeon or the interventional cardiologist should have this information before the procedure.If anomalous pulmonary venous drainage is present, device closure is not appropriate, and referral to a congenital heart surgeon should be considered.All patient candidates for transvenous pacing should be evaluated for an ASD or possibly even a patent foramen ovale, since they are at increased risk of strokes and systemic embolic events through the embolisation of pacing lead thrombi. Ventricular septal defectsWhile small ventricular septal defects (VSDs) may be seen in practice, it is important to look for associations that may require repair.First, check that there is no more than mild associated aortic regurgitation.In some patients outlet VSDs 3 occur which can lead to aortic cusp prolapse and clinically important aortic regurgitation.Such patients should be evaluated as possible surgical candidates.Other patients with what is believed to be small VSDs may also have important right ventricular outflow tract obstruction causing right ventricular hypertrophy, symptoms, and atrial flutter.They too are surgical candidates.Other patients believed to have small VSDs 4 may dilate the left heart chambers in follow up, which in turn may lead to atrial fibrillation and congestive heart failure.A periodic TTE and awareness of this issue should avoid this happening. Patent ductus arteriosusIsolated patent ductus arteriosus (PDA) occurs in four forms:

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