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Background: The long-term outcome of adults with repaired total anomalous pulmonary venous connection (TAPVC) is poorly documented. Therefore, the present study aims to provide current clinical data on adult survivors with repaired TAPVC focusing on arrhythmia. Methods: Clinical and imaging data (prevalence and type of arrhythmias, symptoms, surgical and medical treatment, echocardiographic and cardiac magnetic resonance haemodynamic parameters) were retrospectively collected from 8 European centres and compared between patients with and without arrhythmias. Results: Fifty-seven patients were included (age 20 [16-67] years [female 28, 49%]). At the last follow-up, that is, 21 (8-51) years after surgery, 79% and 93% of patients were free of symptoms and cardiac medication, respectively. The prevalence of late arrhythmias was 21%; 9 (16%) patients showed intra-atrial re-entrant tachycardia (IART) and 2 (4%) ventricular arrhythmias. Patients with IART were older (P = 0.018) and 4 (7%) required antiarrhythmic medication. Three patients (5%) underwent an electrophysiological study, and another 3 (5%) underwent pacemaker implantation within 36 months after surgical correction, which were removed in 2 patients after 7 years. Early postoperative arrhythmias (P = 0.005), right ventricular dilatation (P = 0.003), and valvulopathy (P = 0.009) were more often present in patients with late IART. Conclusions: Adult survivors after isolated-TAPVC repair presented a high prevalence of arrhythmias. Age, right ventricular dilatation, early arrhythmias, and valvular lesions are risk factors for IART. Long-term follow-up is important as some of these currently asymptomatic patients will probably develop arrhythmias in the future. Résumé: Contexte: Les résultats de santé à long terme chez les adultes ayant subi la correction d’un retour veineux pulmonaire anormal total (RVPAT) sont mal connus. Notre étude vise donc à recueillir des données cli-niques au sujet des patients ayant subi cette intervention et ayant survécu jusqu’à l’âge adulte, en particulier pour ce qui est des arythmies. Méthodologie: Les données d’observation clinique et d’imagerie (la prévalence et le type d’arythmies, les symptômes, les traitements chirurgicaux et médicaux, et les paramètres hémodynamiques obtenus par échographie et par résonance magnétique cardiaque) ont été recueillies de façon rétrospective dans huit centres européens et comparées selon que les patients présentaient ou non une arythmie. Résultats: Cinquante-sept patients ont été retenus (âge médian : 20 [16-67] ans; 28 [49 %] femmes). Au dernier suivi, soit 21 (8-51) ans après l’intervention chirurgicale, 79 % des patients ne présentaient pas de symptômes et 93 % des patients ne prenaient pas de médicaments pour des troubles cardiaques. La prévalence d’arythmies tardives s’élevait à 21 %; neuf patients (16 %) présentaient une tachycardie par réentrée intra-atriale (TRIA) et deux patients (4 %) présentaient des arythmies ventriculaires. Les patients qui présentaient une TRIA étaient plus âgés (P = 0,018) et quatre d’entre eux (7 %) devaient prendre des médicaments antiarythmiques. Trois patients (5 %) avaient subi des études électrophysiologiques et trois autres patients (5 %) avaient subi l'implantation d’un stimulateur cardiaque au cours des 36 mois suivant la correction chirurgicale; le stimulateur cardiaque a été retiré sept ans plus tard dans deux de ces cas. Les arythmies postopératoires précoces (P = 0,005), la dilation du ventricule droit (P = 0,003) et la valvulopathie (P = 0,009) étaient plus fréquentes chez les patients qui présentaient une TRIA tardive. Conclusions: La prévalence d’arythmies chez les patients survivant jusqu’à l’âge adulte après la correction isolée d’un RVPAT était élevée. L’âge, la dilation du ventricule droit, les arythmies précoces et les lésions valvulaires sont des facteurs de risque de TRIA. Il est important d’effectuer un suivi à long terme des patients ayant subi une RVPAT puisque certains d’entre eux, asymptomatiques pour le moment, présenteront sans doute des arythmies dans les années à venir.

Abstract Background Cardiovascular magnetic resonance (CMR) is considered the method of choice for evaluation of aortic root dilatation in congenital heart disease. Usually, a cross-sectional 2D cine stack is acquired perpendicular to the vessel’s axis. However, this method requires a considerable patient collaboration and precise planning of image planes. The present study compares a recently introduced 3D self-navigated free-breathing high-resolution whole heart CMR sequence (3D self nav) allowing a multiplanar retrospective reconstruction of the aortic root as an alternative to the 2D cine technique for determination of aortic root diameters. Methods A total of 6 cusp-commissure (CuCo) and cusp-cusp (CuCu) enddiastolic diameters were measured by two observers on 2D cine and 3D self nav cross-sectional planes of the aortic root acquired on a 1.5 T CMR scanner. Asymmetry of the aortic root was evaluated by the ratio of the minimal to the maximum 3D self nav CuCu diameter. CuCu diameters were compared to standard transthoracic echocardiographic (TTE) aortic root diameters. Results Sixty-five exams in 58 patients (32 ± 15 years) were included. Typically, 2D cine and 3D self nav spatial resolution was 1.1–1.52 × 4.5-7 mm and 0.9–1.153 mm, respectively. 3D self nav yielded larger maximum diameters than 2D cine: CuCo 37.2 ± 6.4 vs. 36.2 ± 7.0 mm (p = 0.006), CuCu 39.7 ± 6.3 vs. 38.5 ± 6.5 mm (p

A 38-year-old asymptomatic man was referred by his general practitioner for a 3/6 systolic heart murmur, which was detected during a routine consultation. Echocardiography revealed a parachute mitral valve associated with a parachute-like membrane, causing significant subaortic obstruction that was eventually surgically resected with an excellent postoperative outcome. (Level of Difficulty: Beginner.)

Introduction: The Ross procedure requires like the arterial switch operation (ASO) coronary artery reimplantation. There is no information on coronary artery stenosis at the site of the reimplantation in Ross patients as has been described for ASO patients. This study assessed therefore the prevalence of positive exercise tests (pETs) suggesting myocardial ischemia in Ross and ASO patients in comparison to tetralogy of Fallot patients (TOF), the latter not undergoing coronary artery reimplantation. The study evaluated further, whether a coronary artery complication was confirmed by a supplementary non- and/or invasive evaluation. Methods: Results of exercise tests, echocardiography, coronary computed tomography (CCT) and coronary angiography (CA) were retrospectively reviewed in 30 Ross, 14 ASO and 30 TOF patients. Results: Exercise capacity did not differ between groups. There was a trend to more frequent pETs in Ross patients (Ross vs. ASO vs. TOF, N, %): 8 (27) vs. 1 (7) vs. 2 (7) p = 0.062. CCT was performed in 30% Ross, 21% ASO, 3% TOF (p = 0.023); CA in 20% Ross, 21% ASO and 13% TOF patients (p = 0.727). None of these investigations revealed a coronary artery stenosis. Patients with pET showed a higher right ventricular outflow tract (RVOT) gradient (44 ± 30 vs. 22 ± 13 mmHg, p = 0.004) and more frequently a severe RVOT stenosis (27 vs. 0%, p