Introduction: Idiopathic dilatation of the right atrium (IDRA) is a rare abnormality usually detected by chance at any time between antenatal and adult life. It is defined as isolated enlargement of the right atrium in the absence of other cardiac lesions causing right atrial dilatation. IDRA can be associated with atrial arrhythmia and systemic embolism. The clinical presentation shows high variability ranging from the lack of any symptoms up to cardiac failure. Methods/Results: We describe 2 children with antenatally diagnosed IDRA, the intrauterine course in 1 case, the postnatal management and its long-term follow-up. There has been no need for surgical intervention so far because of the lack of arrhythmias and no further progression of right atrial diameters. Thrombus formation in the right atrium, which is a potential risk for pulmonary embolism, led us to initiate anticoagulation in our cases to prevent such complications. Furthermore, we suggest one possible pathomechanism of congenital right atrial dilatation. Conclusion: Optimal management of severe IDRA depends on the individual case. Long-term follow-up of these patients is necessary to monitor a possible further progression of right atrial size and occurrence of arrhythmias. As a possible pathomechanism, a functional partial anomalous pulmonary venous insertion may imitate a structural abnormal pulmonary vein connection in some idiopathic cases of congenital right atrial dilatation. / Dieser Beitrag ist mit Zustimmung des Rechteinhabers aufgrund einer (DFG-geförderten) Allianz- bzw. Nationallizenz frei zugänglich.

Introduction: Idiopathic dilatation of the right atrium (IDRA) is a rare abnormality usually detected by chance at any time between antenatal and adult life. It is defined as isolated enlargement of the right atrium in the absence of other cardiac lesions causing right atrial dilatation. IDRA can be associated with atrial arrhythmia and systemic embolism. The clinical presentation shows high variability ranging from the lack of any symptoms up to cardiac failure. Methods/Results: We describe 2 children with antenatally diagnosed IDRA, the intrauterine course in 1 case, the postnatal management and its long-term follow-up. There has been no need for surgical intervention so far because of the lack of arrhythmias and no further progression of right atrial diameters. Thrombus formation in the right atrium, which is a potential risk for pulmonary embolism, led us to initiate anticoagulation in our cases to prevent such complications. Furthermore, we suggest one possible pathomechanism of congenital right atrial dilatation. Conclusion: Optimal management of severe IDRA depends on the individual case. Long-term follow-up of these patients is necessary to monitor a possible further progression of right atrial size and occurrence of arrhythmias. As a possible pathomechanism, a functional partial anomalous pulmonary venous insertion may imitate a structural abnormal pulmonary vein connection in some idiopathic cases of congenital right atrial dilatation. / Dieser Beitrag ist mit Zustimmung des Rechteinhabers aufgrund einer (DFG-geförderten) Allianz- bzw. Nationallizenz frei zugänglich.

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Background: Right ventricular (RV) function is a known predictor of adverse events in heart failure and following acute myocardial infarction (AMI). While right atrial (RA) involvement is well characterized in pulmonary arterial hypertension, its relative contributions to adverse events following AMI especially in patients with heart failure and congestion need further evaluation. Methods: In this cardiovascular magnetic resonance (CMR)-substudy of AIDA STEMI and TATORT NSTEMI, 1235 AMI patients underwent CMR after primary percutaneous coronary intervention (PCI) in 15 centers across Germany (n = 795 with ST-elevation myocardial infarction and 440 with non-ST-elevation MI). Right atrial (RA) performance was evaluated using CMR myocardial feature tracking (CMR-FT) for the assessment of RA reservoir (total strain εs), conduit (passive strain εe), booster pump function (active strain εa), and associated strain rates (SR) in a blinded core-laboratory. The primary endpoint was the occurrence of major adverse cardiac events (MACE) 12 months post AMI. Results: RA reservoir (εs p = 0.061, SRs p = 0.049) and conduit functions (εe p = 0.006, SRe p = 0.030) were impaired in patients with MACE as opposed to RA booster pump (εa p = 0.579, SRa p = 0.118) and RA volume index (p = 0.866). RA conduit function was associated with the clinical onset of heart failure and MACE independently of RV systolic function and atrial fibrillation (AF) (multivariable analysis hazard ratio 0.95, 95% confidence interval 0.92 to 0.99, p = 0.009), while RV systolic function and AF were not independent prognosticators. Furthermore, RA conduit strain identified low- and high-risk groups within patients with reduced RV systolic function (p = 0.019 on log rank testing). Conclusions: RA impairment is a distinct feature and independent risk factor in patients following AMI and can be easily assessed using CMR-FT-derived quantification of RA strain.

Malgré des progrès significatifs dans le diagnostic précoce, on constate ces dernières années une nette tendance à l'augmentation du nombre de patients atteints de carcinome à cellules rénales (CCR) présentant une complication spécifique sous la forme d'un thrombus tumoral (TT) s'étendant le long de la lumière de la veine cave inférieure (VCI) au-dessus du niveau du diaphragme, jusqu'à l'atrium droit. Le seul traitement efficace du CCR avec TT reste la chirurgie. La chirurgie conventionnelle comprend la néphrectomie radicale avec thrombectomie, nécessitant des techniques de pontage cardio-pulmonaire et l'arrêt de la circulation sanguine, ce qui implique toujours des complications graves. Pour les éviter, diverses options d'accès chirurgical à la partie supradiaphragmatique de la VCI et à l'atrium droit ont été proposées exclusivement par le côté abdominal, en utilisant diverses options de diaphragmotomie et de péricardotomie avec isolement de la VCI dans la cavité péricardique. L'un des points les plus importants dans le traitement chirurgical du CCR avec TT supradiaphragmatique, est le contrôle de l'extrémité distale du TT. Cette étape dépend largement de l'accès choisi. Dans ce travail, afin d'optimiser les techniques chirurgicales pour les patients atteints de TT supradiaphragmatique, nous avons comparé la faisabilité et la sécurité des différents accès chirurgicaux transdiaphragmatiques. Nous avons également proposé un accès transdiaphragmatique transpéricardique combiné comprenant des diaphragmotomies en forme de T et circulaires, combinées à des péricardotomies longitudinales et circulaires. Nous avons utilisé quatre corps préservés avec la méthode Thiel pour réaliser les simulations chirurgicales. La néphrectomie radicale avec thrombectomie a été réalisée par deux experts de manière indépendante. Quatre approches chirurgicales de la VCI supradiaphragmatique ont été réalisées consécutivement. Nous avons utilisé des simulateurs de TT de différents diamètres et densités pour étudier la palpation de l'apex du TT et son déplacement. La faisabilité et la sécurité de chaque accès et de chaque procédure chirurgicale ont été évaluées à l'aide du test du Chi carré, avec correction des comparaisons multiples (Bonferroni). Notre travail a confirmé la faisabilité et la sécurité des approches transdiaphragmatiques. Les accès transpéricardiques sont préférables dans les cas où les TT sont plus élevés. Ils constituent une alternative adéquate à la chirurgie conventionnelle en cas de TT flottant supradiaphragmatique, jusqu’à l’atrium droit. / Despite significant progress in early diagnosis, there has been a clear trend in recent years towards an increasing number of renal cell carcinoma (RCC) patients with a specific complication in the form of a tumor thrombus (TT) extending along the lumen of the inferior vena cava (IVC) above the level of the diaphragm into the right atrium. The only effective treatment for RCC with TT remains surgery. Conventional surgery includes radical nephrectomy with thrombectomy, requiring cardiopulmonary bypass techniques and cessation of blood flow, which always involves serious complications. To avoid them, various options for surgical access to the supradiaphragmatic part of the IVC and the right atrium have been proposed exclusively from the abdominal side, using various options of diaphragmotomy and pericardotomy with isolation of the IVC in the pericardial cavity. One of the most important issues in the surgical treatment of RCC with supradiaphragmatic TT is the control of the distal end of the TT. This step depends largely on the access chosen. In this work, to optimize surgical techniques for patients with supradiaphragmatic TT, we compared the feasibility and safety of different transdiaphragmatic surgical accesses. We also proposed a combined transdiaphragmatic transpericardial access including T-shaped and circular diaphragmotomies combined with longitudinal and circular pericardotomies. We used four bodies preserved with the Thiel method to perform the surgical simulations. Radical nephrectomy with thrombectomy was performed by two experts independently. Four supradiaphragmatic ICV surgical approaches were performed consecutively. We used TT simulators of different diameters and densities to study palpation of the TT apex and its displacement. The feasibility and safety of each access and surgical procedure were evaluated using the Chi-square test with correction for multiple comparisons (Bonferroni). Our work confirmed the feasibility and safety of transdiaphragmatic approaches. Transpericardial accesses are preferable in cases with higher TT. They are an adequate alternative to conventional surgery in cases of supradiaphragmatic floating TT, up to the right atrium.