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Many, perhaps most, general manuscripts relating to adults with congenital heart disease (CHD) are introduced by a standard mantra: Congenital heart defects are common and because of dramatic surgical advances, and later medical and catheter contributions, survival to adulthood is now the norm even for infants born with the most severe forms of CHD who otherwise would have died within days to months.
There is no doubt that outcomes have improved for people born with CHD. We are, however, increasingly appreciating the longer-term consequences of this triumph, the burgeoning number of adult patients with CHD. It has become painfully evident that too many patients suffer premature disability and death. In the current report, Dr Poterucha et al present a case series of 24 patients with unrepaired single ventricle physiology seen at the Mayo Clinic, all of whom had survived past 30 years of age. (see page 216) Most of these patients had double inlet left ventricle and either pulmonary stenosis or pulmonary vascular disease. In the current era, the chief importance of extended survival in unrepaired single ventricle CHD is the disconcerting questions it raises about our current approach to such patients. Has the paradigm shift towards earlier comprehensive intervention left an identifiable group of patients worse off than if we had done nothing at all? If so, should we be reconsidering our initial approach to a substantial proportion of patients born with a single ventricle?
Dangerous ideas are born of necessary creativity in challenging circumstances. In this case, the challenging circumstance, and the real reason for acute interest in unusual survival with untreated single ventricle physiology is the increasing concern about the long-term outcomes of the Fontan procedure. In addition to anticipated cardiac complications and almost universally reduced aerobic capacity, there is a wide array of end-organ manifestations related to …
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