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Treatment options in adult congenital heart disease

ESC Congress 2010

Congenital Heart Disease in Children and Adults

The session on treatment options in adult congenital heart disease, organized by the Working Group on Grown-up Congenital Heart Disease, focussed on clinically relevant questions.

SA Qureshi (London, GB) discussed “when are you too old for atrial septal defect closure”. Data in the literature show that atrial septal defect closure at older age (above 60 years) improves symptoms, increases quality of live, induces positive remodelling of the right ventricle and decreases pulmonary arterial pressures. Because of these findings, atrial septal defect closure might indeed be indicated at older age. However, the effect on the development of atrial fibrillation after closure is unclear and closure is preferred to be percutaneous where applicable.

P Trigo Trindade (Zurich, CH), the new chairman of the Working Group, gave an excellent overview about the treatment of Ebstein anomaly: atrial septal defect closure, tricuspid valvuloplasty or conservative? Ebstein anomaly is not a benign disease and needs careful follow-up. Natural history is not uneventful (progressive valve dysfunction, atrial arrhythmias), so that not infrequently, interventions are needed. Depending on the right ventricular function, signs of right heart failure, cyanosis through an interatrial communication, the type of structural and/or electrophysiological intervention are chosen. In case of a right to left shunt and good tricuspid valve function, only closure of the interatrial defect might be indicated; when severe valvular dysfunction is present, surgical valve repair or replacement is needed. In this way, P Trigo Trindade referred to the recently presented and published guidelines of Grown-up Congenital Heart Diseases, where the choice of therapeutic approach is more elucidated.

PJ Holm (Lund, SE) focussed on medical therapy in adult congenital heart disease. A main problem in several patients is the function of a systemic right ventricle. A substantial number of congenital heart disease patients die because of heart failure (26%). To prevent heart failure, treatment to reduce afterload, diuretics and betablockers are frequently started. Although symptomatic patients seem to improve with this therapy, no large randomized trials are conducted to answer the question of whether they would influence outcome. The effect of ACE-inhibitors or sartans on the systemic right ventricular function remains unclear, and in some patients betablockers are even contra-indicated. A case of a patient with a systemic right ventricle and successful resynchronisation therapy on top of medical treatment was presented. Unfortunately, several patients still die suddenly (19%), even when in NYHA class I, so that the issue rose for arrhythmic prophylaxis.

The last speaker, K Dimopoulos (London, GB), highlighted the importance of iron repletion, especially in patients with secondary erythrocytosis. Low iron reserves might lead to the same symptoms as those of hyperviscocity, are related to the occurrence of stroke, and by repletion, patients feel better, have better exercise capacity and increased functionality. However, the debate is still going on as to what the minimal and/or maximal iron reserves have to be, and what the dose of substitution should be. He stressed that phlebotomies in secondary erythrocytosis need to be discouraged in order to avoid the risk of iron depletion. He finally mentioned that low iron reserves might enhance hypoxic vasoconstrictor responses.

I appreciated this as an excellent session with a lot of attendees. The speakers focussed their talks on clinically relevant issues, and that was highly appreciated by the audience. Take home messages were clear, and the new guidelines will help to implement all this in daily practice. I would encourage the working group to continue with sessions such as this.




Treatment options in adult congenital heart disease
The content of this article reflects the personal opinion of the author/s and is not necessarily the official position of the European Society of Cardiology.