Predictors of morbidity and mortality in contemporary Fontan patients: results from a multicenter study including cardiopulmonary exercise testing in 321 patients

Diller, Gerhard‐Paul; Giardini, Alessandro; Dimopoulos, Konstantinos; Gargiulo, Gaetano; Müller, Jan; Derrick, Graham; Giannakoulas, George; Khambadkone, Sachin; Lammers, Astrid E.; Picchio, Fernando Maria; Gatzoulis, Michael Α.; Hager, Alfred

doi:10.1093/eurheartj/ehq356

Cited by 310 publications

(241 citation statements)

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“…Atrial tachycardias are a leading source of morbidity and mortality in patients after Fontan-type procedures [6,7,11]. Among patients with RA-PA connection that was performed in the past, the proportion of patients with supraventricular arrhythmia at 20 years after the surgery may be as high as 50% [12,13].…”

Section: Discussionmentioning

confidence: 99%

“…Occurrence of atrial tachycardia (AT) in a patient after the Fontan procedure may result in a significant worsening of the clinical status, often leading to the development or worsening of heart failure, syncope, or even death (Fig. 1) [6,7]. Persistent arrhythmia with rapid ventricular response may lead to impaired function of the single ventricle, and systolic and diastolic dysfunction of the single ventricle may in turn result in the development or worsening of the atrioventricular valve regurgitation.…”

Section: Introductionmentioning

confidence: 99%

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Atrial tachycardia ablation in patients with a functional single ventricle after the Fontan surgery

et al. 2016

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A b s t r a c tBackground: Atrial tachyarrhythmias are a leading source of morbidity and mortality after Fontan-type procedures and antiarrhythmic drug therapy is often ineffective in these patients. Aim:To evaluate short-and long-term outcomes of radiofrequency current ablation for atrial tachycardia (AT) in patients after the Fontan procedure, and to report clinical, electrophysiological and electroanatomical characteristics of these arrhythmias. Methods:We retrospectively analysed data obtained in 8 patients (5 males, 3 females) after the Fontan procedure who underwent ablation for AT between 2002 and 2013. In order to compare the clinical impact of arrhythmia before and after ablation, we used the modified arrhythmia score, ranging from 0 (no arrhythmia activity) to 12 (very severe arrhythmia). In all patients, electroanatomical mapping using the CARTO system was performed, allowing semiquantification of low-voltage (< 0.5 mV) areas and scars.Results: Seven patients had an atriopulmonary connection and 1 patient had an extracardiac conduit. The mean patient age was 9.4 ± 3.1 years at the time of the Fontan procedure and 26.2 ± 4.6 years at the time of the first ablation. A total of 18 ablations were performed with no complications, 1 to 4 (median 2.5) procedures per patient. In patients who had more than 1 ablation, the mean time from the first to the last procedure was 34.8 months (range 1-64 months). In individual patients, 1 to 4 (median 2.5) different ATs were observed, with the mean tachycardia cycle length of 334 ± 95 ms. In 6 patients, low-voltage area (< 0.5 mV) comprised 25-50% of the right atrium, and in two others it comprised 10-25% and < 10% of the right atrium, respectively. Seven procedures were fully successful (ablation of all ATs), 7 were partially successful (ablation of only some AT, including clinical arrhythmia, but not of all ATs) and 4 were unsuccessful (failed ablation of clinical AT). The mean procedural, fluoroscopy and ablation times were 176 ± 54.6, 13.7 ± 5.7 and 21.7 ± 11.9 min, respectively. Freedom from arrhythmia during the mean follow-up of 58.6 ± 46 months (range 11-127 months) since the last procedure was obtained in 4 patients. The median arrhythmia score after the last ablation was significantly reduced compared to baseline (4.5 vs. 8; p < 0.05).Conclusions: Catheter ablation of AT in patients after the Fontan procedure is safe but its acute and long-term efficacy is limited. Due to complex and extensive substrate, along with complex anatomy, recurrences are frequent and patients may require repeat ablation procedures. Suppression of arrhythmia is associated with an improved clinical status of the patients.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Atrial tachycardia ablation in patients with a functional single ventricle after the Fontan surgery

et al. 2016

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“…Recent studies further report an annual decline of 1-3 % in maximal oxygen consumption starting at an adolescent age. The average peak consumption ranges from 19 to 28 ml/kg/min, or 50-60 % of the predicted consumption for age [10].…”

Section: Fontan Physiology Pvr and Exercisementioning

confidence: 99%

The Fontan Circulation: The Known, the Unknown and the Plausible

Louw

Gewillig

2013

Curr Pediatr Rep

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The Fontan circulation is used as palliative surgery in the functionally univentricular heart. Due to the advances made in prenatal diagnosis, cardiac surgery, technology, and monitoring in complex congenital heart disease, survival rates have improved significantly. For this group of patients, the physiology and pathology remain challenging and eluding, even more so in the long-term follow-up. In the last decade pulmonary vascular resistance has been shown to be of utmost importance regarding cardiac output both at rest and during exercise. The aim of this article is to review the most recently published data, namely new concepts and our current understanding regarding the physiology; performance during exercise; the value of imaging modalities; and chronic liver pathology in the Fontan circulation.

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“…(45,46) There will be difficulties encountered in EPS in patients with an extra-cardiac conduit Fontan, as there is no direct connection between the venous system and the atrial mass, however there are potential strategies. (47) …”

Section: Atrial Arrhythmiasmentioning

confidence: 99%

Management of the Fontan circulation in the adult patient

Clift

2017

SAHJ

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Predictors of morbidity and mortality in contemporary Fontan patients: results from a multicenter study including cardiopulmonary exercise testing in 321 patients

Cited by 310 publications

References 33 publications

Atrial tachycardia ablation in patients with a functional single ventricle after the Fontan surgery

Atrial tachycardia ablation in patients with a functional single ventricle after the Fontan surgery

The Fontan Circulation: The Known, the Unknown and the Plausible

Management of the Fontan circulation in the adult patient

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