Adult congenital heart disease

Over the last 20 years, the face of congenital heart disease has changed beyond all recognition. Early death in childhood has been replaced by late death in adulthood. With increasing longevity, long-term sequelae of congenital disease corrected in early life are being recognized. In a sizeable proportion the consequences include ventricular dysfunction and heart failure (HF). The inclusion of congenital heart disease as an important aetiology of adult HF is a demonstration of changing demographics. Indeed, there are now more adults with congenital heart lesions than children. It is estimated that there are 185 000 adults with significant congenital heart disease in the United Kingdom. Many of the longterm consequences of congenital heart disease were not appreciated in the early era of paediatric cardiac surgery. Many patients (such as those with repaired coarctation of the aorta) were discharged, resulting in only a minority of the patients now being under specialist cardiology follow-up. When an adult patient presents with HF, it is important to exclude a pre-existing congenital aetiology. It is difficult to define HF in adults with congenital heart disease. Early definitions (‘A clinical syndrome caused by an abnormality of the heart and recognized by a characteristic pattern of haemodynamic, renal, neural and hormonal responses’, Poole-Wilson,1985) could encompass every congenital cardiac lesion. Even the modern European Society of Cardiology definition (‘Symptoms of HF at rest or exercise and objective evidence of cardiac dysfunction ± response to treatment’, ESC Task Force 2005) captures a very wide and divergent range of congenital lesions. For the purpose of this chapter we will concentrate on disorders where ventricular function is the predominant lesion.