Intended for healthcare professionals


Hypoplastic left heart syndrome

BMJ 1997; 314 doi: (Published 11 January 1997) Cite this as: BMJ 1997;314:87

Terminal care is not the only option

  1. Sean W O'Kelly, Director of pediatric cardiac anaesthesiologya,
  2. Edward L Bove, Professor of surgeryb
  1. a Section of Pediatric Anesthesiology, The University of Michigan, Mott Children's Hospital, F3900, Box 0211, Michigan 48109-0211, USA
  2. b Pediatric Cardiovascular Surgery Section of Thoracic Surgery, The University of Michigan, Mott Children's Hospital, F7900, Box 0223, Michigan 48109-0223 USA

    Hypoplastic left heart syndrome is not an uncommon condition and accounts for about 10% of critical congenital heart disease in infants. Each year in Britain about 200 children will be born with this condition, which, unlike some other congenital cardiac lesions, usually occurs without serious associated non-cardiac malformations. Hypoplastic left heart syndrome in its most common form consists of an underdeveloped left ventricle, mitral valve, and aortic root. Initially, systemic blood flow is maintained by the patent ductus arteriosus, and death occurs soon after this closes. Most children born with hypoplastic left heart syndrome in Britain will be managed with terminal supportive care, very few will present to cardiac surgeons (M Elliott, personal communication). Newborn infants with other complex congenital cardiac malformations–such as pulmonary atresia with intact ventricular septum–can usually expect corrective or palliative surgery with a reasonable likelihood of a satisfactory outcome. Why then are children with hypoplastic left heart syndrome still singled out for terminal care? Is there any evidence that surgery for these children is a reasonable proposition?

    It is over 15 years since Norwood described a surgical technique for repair of hypoplastic left heart syndrome.1 During this time modifications have resulted in three staged surgical procedures that ultimately allow the right ventricle to act as the systemic chamber and produce separation of the pulmonary and systemic blood flow by the performance of a Fontan procedure. Although there has been interest in the surgical palliation of hypoplastic left heart syndrome in the United States for some time, this has not yet been the case in Britain. With the publication last year of the results of surgical palliation at the Birmingham Children's Hospital,2 interest may be growing, although many paediatric cardiac centres may still need to be convinced of the value of this course of management.

    In several centres in the United States surgical palliation for hypoplastic left heart syndrome has been justified both in terms of survival and quality of life.3 4 5 The terminal care approach has been effectively challenged, with parents and physicians coming to expect a more positive outlook. It is important to stress that–with increasing experience and modification of surgical, anaesthetic, and intensive care techniques–survival figures have improved considerably over time. In our own centre, five year actuarial survival for 127 neonates with classic hypoplastic left heart syndrome–born without complicating factors such as severe pulmonary venous obstruction or important congenital non-cardiac conditions and who undergo first stage repair at less than one month of age–is currently in the order of 70%.6 7 The oldest survivors are now between 10 and 12 years of age. These children remain active and attend school. Although difficult to quantify, there is no reason to suppose that the quality of life for these children differs substantially from that of children born with other forms of single ventricle who undergo surgical palliation.

    Surgical palliation is not the only management option for hypoplastic left heart syndrome. Some centres perform heart transplantation for these children. Results of transplantation are comparable to surgical palliation in terms of survival.8 9 However, when mortality after transplantation is combined with the mortality of children waiting for a donor heart the figures are less encouraging. With the shortage of suitable donors, it seems unlikely that transplantation will result in overall superior survival rates for these children.

    Survival figures are not the only criteria on which the value of surgical intervention is judged. Questions concerning the prevalence of residual morbidity in this surgical population remain. Although no special problems have been identified in this group of patients, long term cardiopulmonary and neurodevelopmental outcomes have not yet been adequately established, given the ages of the oldest survivors. However, if, as we suspect, long term outcomes are at least as good as those associated with other complex cardiac lesions, should there be any question that surgical palliation of hypoplastic left heart syndrome is an equally appropriate course of action? Realistically it might be relevant to ask whether paediatric cardiac centres have the resources and willingness to initiate an intensive therapeutic programme, especially when the initial period of such a programme may involve setbacks and disappointments. Once surgical palliation is initiated, parents and children are committed to multiple procedures and invasive diagnostic studies. This requires a substantial degree of emotional and physical stamina; some families will cope better than others. Yet for those who have seen the success of surgical palliation for hypoplastic left heart syndrome and witnessed the joy of parents, as well as the continued wellbeing of their children, it is difficult to avoid enthusiasm for this approach and impossible to ignore the outcome.

    Hypoplastic left heart syndrome presents a considerable challenge to all those involved in the management of congenital cardiac malformations; but in the light of continued improvement of surgical palliation–with outcomes as good as, if not better than, those of other congenital cardiac lesions–it may be an appropriate time for the medical community to rethink its approach to children born with this condition. Parents and doctors should be aware of the possibilities of treatment. Simply letting these children die from their malformation may no longer be an option that is easily justified.


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