Dr. Massimo Chessa,
All cardiologists involved in the care of Grown Ups with Congenital Heart defects (GUCH), especially those not working in Specialised Centres must know that GUCH are seldom cured and usually have ongoing cardiac problems. They must help to identify the best location for procedures to take place, seek appropriate expertise from relevant non-cardiac subspecialties, and secure prenatal and post-partum management for GUCH patients wanting to get pregnant.
Advances in echocardiography, anaesthesia, intensive care, and cardiac surgery especially, have revolutionised the prognosis of infants and children with cardiac defects over the last 50 years such that an increasing number of patients now reach adolescence and adult life, including patients with complex defects. This triumph has led to a new population of patients: adults with congenital heart disease. Patients with acquired heart disease are different from GUCH patients in that the former will have most often noticed a distinct change in their symptoms at onset whereas GUCH patients, having lived with a lifelong cardiac condition, may not detect subtle changes in exercise capacity until they have become significant. Before a GUCH patient may even notice any dyspnoea or exercise limitation, valvular residua and ventricular dysfunction may have become severe and even irreversible (1). GUCH patients as well as their families and physicians must know that cardiac surgery is for the most part palliative rather than curative: lifelong follow-up at centres where expertise is available for dealing with their complex problem and co-morbidities is in order. Co-morbidies include renal failure, peripheral vascular disease, chronic obstructive pulmonary disease, deep venous thrombosis, and substance abuse, which are not uncommon among children. GUCH patients fare best when treated by teams with extensive experience in the management of congenital heart defects in infants and children as well as knowledge of the principles of conventional adult cardiac surgery (2). As GUCH patients are increasingly turning up at cardiologists’ practises for care, healthcare professionals need to increase their knowledge regarding the medical issues these patients face (3).
Arrhythmia is the major cause of morbidity and mortality and is one of the most important cause for hospitalisation in this patient population. Aetiology is multifactorial with electrical disturbances that are part of the defect - e.g. pre-excitation in Ebstein’s anomaly, a consequence of the operation e.g. atrial arrhythmias following Senning/Mustard repair for transposition of the great arteries, or the result of hemodynamic abnormalities during follow-up - e.g. ventricular tachycardia with pulmonary regurgitation after tetralogy of Fallot repair. Arrhythmia, which might be considered benign in patients with normal hearts, may lead to catastrophic hemodynamic decline and result life-threatening in patients with surgical residua. Supraventricular arrhythmia is more frequent than ventricular arrhythmia, and its incidence is growing along with longer follow-up. Sinus node dysfunction is most common following atrial surgery. Pharmacological treatment may be limited by hemodynamic side-effects, concomitant sinus node dysfunction and by a desire for pregnancy. Catheter ablation and surgical approaches have been increasingly applied, but success rates remain lower than those in structurally normal hearts.Pacing in these patients is often difficult due to limited, abnormal access to the heart as well as the abnormal cardiac anatomy itself. Implantable cardioverter defibrillator will be used with increasing frequency in patients with congenital heart defects who are considered at risk of sudden death. This patient population is at an increased need of therapeutic interventions. Transcatheter septal defects closure, balloon valvuloplasty, paravalvular leaks closure, stent implantation, and percutaneous valve implantation are frequently performed in the cath labs of units for adult with congenital heart defects. These procedures usually hold very good results. Embolisation of venous and arterial collaterals, fistula and patent ductus arteriosus are also, frequently needed. Haematological problems are most frequently encountered in the cyanotic patient. The hyper viscosity syndrome, iron deficiency, a reduced platelet count and abnormal platelet function together with clotting factor deficiencies, and are a cluster of the major concerns for these patients. Iron deficiency that may result from inapropriate treatment, must be avoided. Hyperuricaemia is common and may produce frank gout. Additionally, most, however not all, patients have a lifelong risk of endocarditis. Appropriate information and health patient education on this subject is mandatory. Antibiotics prophylaxis is recommended following surgical repair of most conditions. The portals of entry for infection are more numerous than generally appreciated and include acne, body piercing and tattooing. Needs are also increased for specialist advice in other disciplines:
Regarding management of contraception and potential pregnancy in this patient population, cardiologists must suggest specialised counseling. It is important for this counseling to begin before conception with consideration of the risks to both foetus and mother, and wih genetic counselling included. In addition to provision of care for complex medical and surgical problems, indicating GUCH patients where to find proper support for their many psychosocial problems is also in order (4).
1. Warnes CA. The adult with congenital heart disease: born to be bad? J Am Coll Cardiol. 2005 Jul 5;46(1):1-8. 2. Karamlou T, Diggs BS, Ungerleider RM, Welke KF. Adults or big kids: what is the ideal clinical environment for management of grown-up patients with congenital heart disease? Ann Thorac Surg. 2010 Aug;90(2):573-9. 3. Dearani JA, Connolly HM, Martinez R, Fontanet H, Webb GD. Caring for adults with congenital cardiac disease: successes and challenges for 2007 and beyond. Cardiol Young. 2007 Sep;17 Suppl 2:87-96. 4. Baumgarner H. et al. ESC guidelines for the management of grown-up congenital heart disease (new 2010 version) by The Task Force on the Management of Grown-up Congenital Heart disease of the European Society of cardiology (ESC) European Heart Journal.
Dr Massimo Chessa, MD, PhD, FSCAI Department of Paediatric Cardiology & Adult with Congenital Heart Disease IRCCS-Policlinico San Donato Via Morandi 30 20097 San Donato Milanese, MI - Italy Tel: +39-02-52774531 Fax: +39-02-52774459 Italian GUCH Association
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