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Report of the Fifth European meeting on Adult Congenital Heart Disease, 10-11 April 2014, Amsterdam, the Netherlands

The scientific programme of this meeting was endorsed by the WG on GUCH.

First speakers were Christian Schreiber and David Barron about M Ebstein: repair or replace? Christian showed very good results for repair from the surgical group in Munich. Indeed better then after replacement of the valve, where re-operation was necessary in 65% of the patients after 20 years. Especially the Cone repair is promising and currently first choice. However, long-term outcome has to be awaited. The learning curve of this repair technique is steep and probably especially at young age the method of choice. 

David showed that at this moment replacement is most widely used in Europe.  He showed that no differences were found in long-term results for repair versus replacement.  Indeed repair is no good option if there is severe displacement of the valve, or severe regurgitation exists with multiple jets,  or when the RV function or LV function is reduced. In these situations it is better to go for a short procedure:  replacement of the valve. Repair should be first choice at young age, good clinical condition, or small RV.  In addition David argued in favor of early surgical intervention. This was a topic of vivid discussion as not all agreed with him.

In the second session Victor Tsang and John Deanfield discussed the timing of TV surgery in ccTGA and TGA after Senning/Mustard. John showed that in TGA patients nothing is wrong with the intrinsic myocardial performance but that preload is probably the most important factor for outcome. He highlighted that TGA patients with VSD did worse compared to TGA patients without VSD. In addition his opinion was that tricuspid regurgitation is the result of RV dysfunction and not the cause. Losartan has no effect in Mustard patients. In ccTGA the RV is often abnormal (Ebstein-like) and therefore you cannot put ccTGA in one group with Mustard/Senning patients. The concept of septal shift by inducing PS is elegant but implies additional interventions. In a series of the Mayo Clinic of early surgery for tricuspid regurgitation in ccTGA no clear effect on the natural history could be found. When an operation is done, often valve replacement is necessary, so surgery has not to be done too early.Victor told us that RV ejection fraction is the most important factor, also for the results after surgery. If not done too late, systemic ventricular ejection fraction not deteriorate after valve replacement, so a clear case exists for early surgery to prevent irreversible damage to the RV. Nice discussions focused on the underlying cause of tricuspid regurigtation. Is it functional or a morphological problem? Early banding of the PS could be a good choice.

Peter Verhamme discussed the role of NOACs in cardiology. He nicely explained the different indications for anticoagulation, varying from prophylactic treatment for VTE to treatment in mechanical valves. For atrial fibrillation NOACs are proven to be as effective with less bleeding complications. Especially less cerebral bleeds, however more GE bleeding complications were encountered.  Recently also for VTE this was found. Is there a role for longer treatment after 6 months? Yes, recently a trial was published showing superiority for the NOACs over placebo with only 2% recurrence of VTE in the NOAC group versus 10% in the placebo group (Agnelli NEJM 2013).
However, in mechanical valve patients the NOACs seem to do worse and are not effective enough.

Gruschen Veldtman discussed the role of NOACs in Fontan patients. He first showed the factors that make the Fontan circulation prone to thrombosis. The huge RA, abnormal coagulation and endothelial disruption all play a role. TEE is much more effective in finding clots than TTE. In a TEE study, 22% of Fontan patients have a clot, which was detected with TTE in only a fifth of the cases. No clear differences are found between ASA and warfarin in the prevention of clots and in both treatment regimens still recurrent thrombosis occurs, so no effective treatment is currently available. Perhaps there is indeed a role for NOACs and Gruschen suggests a RCT.

Rob de Winter talked about devices and anticoagulation, showing that no good data is available on this topic. In general cardiology indeed large RCTs have been performed, but these are completely lacking in the field of congenital heart disease. Currently everybody treats patients after ASD device closure with dual antiplatelet therapy, but that is not based on large studies, but merely on expert opinion. Residual leakage after ASD shunt closure or the size of the shunt were not found to predict thrombosis. Three large RCTs were published in NEJM on PFO closure, showing no advantage of closing the PFO for recurrent stroke. Therefore, we can now stop closing PFOs on a regular basis. Of course there can be an individual case of a young patient with no other risk factors and residual paroxysmal embolism is the most likely cause so that it might be considered. The Cristal AF study showed that patients after cryptogenic stroke often had AF, discovered with an implantable loop recorder, which was not recognized before.

Mark Hazekamp showed that the Contegra valve has very limited calcifications of the leaflets and that the valve survival at 10 years is excellent (freedom of replacement 90%) in the large study by Brown and collegues. However, others found worse outcome. The maximal diameter of 22 mm is a limitation. This versatile valve is user friendly.

Ronny Gustafsson showed his results with the vascutek prosthesis and presented promising results. By asking the audience, about 50% of the attendees chose a pulmonary homograft as the RV-PA conduit of choice.

Ad Bogers showed his results with a large population of patients with pulmonary homografts. He showed excellent survival of 88% at 15 years follow-up and freedom of reoperation of 81% at 15 years. Predictors of re-operation were young age and aortic allograft.

Helmut Baumgartner showed the results of the percutaneous PVR. He explained that it is essential to have a good result immediately and advised to try and reach a low gradient (25mmHg) directly after the procedure.  The coronaries remain important to visualize for prevention of coronary lesions. Endocarditis remains a problem and it is not well known how often this occurs. It is reported to occur at an incidence of 0,9% per year, but it may be higher.

New products are under development making placement of a percutaneous valve in a native outflow tract possible. No clear advantage of early valve intervention on survival or incidence of arrhythmias has been observed so far.

Barbara Mulder completed the first day with the Jane Somerville Lecture. She highlighted the enormous contribution that Jane has made to the field and clearly explained why a man needs to be more like a woman!

The second day of the meeting focused on adults with an arterial switch procedure for complete transposition of the great arteries. Folkert Meijboom summarized the imaging modalities currently used for documenting the coronary artery anatomy after surgical translocation of these vessels from the previously aortic to the “neo-aortic” trunk. The majority of GUCH centers in Europe use CT angiography to assess the coronary artery course and eventual lesions. Pascal Vouhé shared the extensive surgical experience of his center in Paris (> 1200 cases) with the audience and discussed treatment modalities for early and late coronary events after an arterial switch procedure. Helmut Baumgartner and Victor Tsang went on to talk about thresholds for surgical re-intervention in patients with progressive “neo-aortic root” dilatation. In the absence of additional risk factors for dissection (like neo-aortic regurgitation and rapid aortic root dilatation), an aortic diameter of 55-60 mm seemed to be the most widely accepted aortic diameter for re-intervention, which usually was a Bentall procedure.

Tjark Ebels discussed surgical strategies for re-interventions after an AVSD repair, most of the time for atrio-ventricular valve regurgitation. He stressed the importance of having the anatomy in mind when apporaching the atrioventricular valve in AVSD patients. Erwin Oechslin summarized the rationale for a “treat and repair” approach in selected patients with pulmonary hypertension due to an atrial shunt. Werner Budts presented additional insights regarding predictors of outcome, the rationale for combination therapy with selective pulmonary vasodilators, and the approach to segmental pulmonary hypertension. The first part of the afternoon was devoted to new developments in cardiac devices for GUCH patients. Potential indications and the Pro’s and Con’s of a subcutaneously implanted ICD were presented by Dr. Reinoud Knops from the University of Leiden. They have experience with these devices. One of the less obvious advantages of a subcutaneously implanted device is the longlivety of the leads. Lead fractures, an important limitations of endovascular leads, are not or only very rarely encountered in subcutaneously implanted devices. Cardiac resynchronization therapy was another electrophysiological topic addressed in the session. Dr. Jan Janousek pointed out the importance of a major electrical activation delay as an underlying reason for a QRS prolongation in GUCH patients. With this in mind, CRT therapy may be considered not only for failure of a systemic left ventricle, but also for a failing systemic right ventricle or a failing subpulmonic right ventricle. The role of mechanical assist devices in pediatric heart failure and adult congenital heart disease and new developments in stent design in order to reduce the need for re-do surgery were additional topics presented by Michael Huebler and Massimo Chessa in this interesting afternoon session.

The meeting ended with a nightmare case of a pregnant woman with severe pulmonary hypertension presented by Dr. Gruschen Veldtman and a glance of the future by Gary Webb on how the use of the internet, worldwide connections and modern electronical devices may affect the care of adult congenital heart disease patients worldwide.