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Outpatient clinics for adults with congenital heart disease
  2. A J PAGE
  1. Department of Cardiology
  2. Norfolk and Norwich Health Care Trust
  3. Brunswick Road, Norwich, Norfolk NR1 3SR, UK

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Editor,—We agree with the views expressed by Gatzoulis et al concerning the need for dedicated clinics for adults with congenital heart disease.1 We started such a clinic in 1993. Initially this was once a month, becoming semimonthly in 1995 and weekly in 1999. The clinic is in a district general hospital serving a population of 650 000, and the initial numbers may have been present because of the early work of Bill Oliver in establishing catheter facilities on this site and the link with Great Ormond Street visiting cardiologists. One of us also runs a paediatric clinic and the other has experience in adult congenital heart disease.

Forty three per cent of our 260 patients are male, and 25% of the patients have septal defects (both atrial and ventricular). As uncomplicated, closed defects are not followed up all these patients have additional lesions or residual sequelae of the original repair.

  • 12% have had a tetralogy of Fallot repaired

  • 11.5% have bicuspid aortic valves

  • 8.7% have had a coarctation repaired

  • 11% have cyanotic heart disease (5% with the Eisenmenger reaction)

  • 5% have left ventricular outflow tract abnormalities

  • 5% have transposition of the great arteries—all of whom have had the Mustard repair

  • 12.1% of patients have Marfan’s syndrome (although patients with Marfan’s are also seen in general clinics).

The remaining 9.9% have various condition from complex congenital heart disease including tricuspid atresia with Fontan operation (1.3%), single ventricle with total cavopulmonary connection (TCPC) (1%), complex pulmonary atresia with right ventricular outflow tract reconstruction (2.6%), septated double outlet ventricles (2.6%) to abnormal AV valves (mitral clefts and Ebstein’s anomaly) (2.1%), corrected transposition, pulmonary valve and pulmonary branch stenoses.

  • 33.6% have had no previous interventions

  • 58.5% have had a single surgical repair of which 10.9% have had one or more reoperations

  • 7.9% have had palliative procedures only.

There have been a few patients with complex cyanotic heart disease who we have referred for their first operative intervention in their 20s, including TCPC in a patient with single ventricle, and right ventricular outflow tract reconstruction in a patient with complex pulmonary atresia.

  • 2.1% have had catheter interventions such as dilatation of aortopulmonary collaterals, coil obliteration of residual shunts, etc

  • 2.1% have had pacemaker implantations.

One patient has already been transplanted (atrial septal defect and restrictive cardiomyopathy), three are waiting (two heart–lung and one heart transplant), and one died on the waiting list.

We use transoesophageal echocardiography and magnetic resonance imaging in selected patients in addition to the more routine use of echocardiography, ECGs, Holter monitoring, and exercise testing. Very complicated cases are usually sent to a tertiary centre for catheterisation, with whom we have a close liaison; indeed in some cases the patient care is shared and discussion of difficult problems in other cases is helpful. The patients do seem to value a dedicated clinic in their local hospital, particularly when given the time to discuss contraception, employment, insurance, housing, inheritance, and pregnancy.

New patients transferred from the paediatric service are also introduced to the GUCH (grown up congenital heart) association, which has newsletters, helplines, and area meetings. We try to maintain an emergency slot for patients if they become worried about symptoms. “At risk” pregnancies are also supervised within the clinic, with close liaison with obstetricians and anaesthetists. Fetal echocardiography is performed at 20 weeks. We would concur with Gatzoulis et al that structured transitional requirements for these patients must be introduced so that they are not lost to follow up when the leave the paediatric service and, as we hope we have shown, a dedicated clinic within a region does fulfil a need, a point purchasers may care to note.