Management of advanced adult congenital heart disease
SA Heart Journal
As surgical techniques continue to develop, for example as seen with the Norwood procedure for hypoplastic left heart syndrome, the number of patients with the most complex forms of CHD will continue to increase along with their associated long term complications. Our ability to manage these patients appropriately, therefore, must also develop alongside. The purpose of this review is to examine the management of these patients once they have developed advanced disease, including drug therapy,
... vices, transplant and end of life care. In particular, the unique problems posed by patients with a systemic right ventricle (RV) and univentricular circulations are discussed. DRUG THERAPY IN HEART FAILURE In patients with complex congenital heart disease, either palliated or unoperated, the development of ventricular dysfunction and clinical heart failure are common and as discussed above, is a common cause of morbidity and mortality in this population. (7) Causes of ventricular dysfunction include longstanding volume or pressure overload, cyanosis or surgical insult. It is usually a chronic ABSTRACT ADVANCED ACHD INTRODUCTION Due to advances in cardiac surgery and paediatric cardiology, increasing numbers of children born with congenital heart disease (CHD) are surviving into adulthood. (1-3) As a result there are now more adults than children living with CHD. (2,4,5), Furthermore, an increasing number of adults are surviving with the more severe forms of CHD with Marelli, et al. reporting the prevalence of severe CHD in adults increasing by 85% between 1985-2000 compared to just 22% in the paediatric population. (4) A recent report by Tutarel, et al. from the UK also recently reported an increase of 6-7 fold in patients with moderate to severe defects since 2000. (6) Only the very simple lesions are truly "cured" by surgical or interventional procedures with the majority of patients remaining at long term risk of complications including arrhythmias, ventricular failure and premature death. (5) Despite this, mortality has shifted away from infancy to adulthood with an increasing age of death reported. (7) Whilst the age of death is increasing, this population still has an excess mortality compared to their healthy counterparts. (3, 8) The commonest cause of death in adults with CHD is heart failure, accounting for up to 40% of all deaths with sudden cardiac death also being a major cause. (3, (8) (9) (10) Although the majority of deaths are cardiac, related to the underlying CHD, non-cardiac mortality was also two fold that expected. (3) Furthermore, as this population continues to age, they will also be at risk of significant medical comorbidities. (6, 11) There are increasing numbers of adults with congenital heart disease. These patients remain at lifelong risk of complications including heart failure, arrhythmias and premature death. This review examines the management of those patients with advanced disease, with particular reference to patients with either a systemic right ventricle or a univentricular circulation. Drugs used to treat left ventricular dysfunction in acquired heart disease have been shown to have little benefi t in this setting. There are, however, promising results from small trials looking at selective pulmonary vasodilators in patients with a previous Fontan operation. Whilst there is evidence of a benefi t with implantable cardiac defi brillators and cardiac resynchronisation, there remains a lack of clear guidelines as to which patients will benefi t from these invasive therapies. Cardiac transplantation in these patients is associated with an increased early mortality but the long term outcome is similar to those with acquired heart failure. Transplantation however, is limited by both the patient's suitability and the availability of a matched organ. End of life care should be discussed with all patients with advanced disease and ideally this should be done early on and in parallel to other therapies.