January

Examination of pathological features of the right atrioventricular groove in hearts with Ebstein’s anomaly, and correlation with arrhythmias

Examination of pathological features of the right atrioventricular groove in hearts with Ebstein's anomaly, and correlation with arrhythmias. Marcondes L, Sanders SP, Del Nido PJ, Walsh EP. Heart Rhythm. 2020 Jan 21. pii: S1547-5271(20)30026-6. doi: 10.1016/j.hrthm.2020.01.013. [Epub ahead of print] PMID: 31978592 Similar articles Select item 31972995   Take Home Points: Cardiac anatomy in Ebstein’s anomaly (EA) is frequently characterized by a prominent muscular ridge along the inferior AV annulus. The presence of this ridge in postmortem hearts correlates with a clinical history of supraventricular tachycardia. The presence of an inferior annular ridge has important implications for the pathogenesis of accessory pathways in EA and may affect catheter ablation outcomes. Commentary by Dr. Jeremy Moore (Los Angeles) Congenital and Pediatric Cardiac EP section editor: Marcondes and colleagues report on the pathological features of the right AV groove in hearts with EA and correlate it with clinical evidence of arrhythmia. The study points to a prominent inferior annular ridge in many gross specimens with EA and correlates this anatomical finding with the clinical presence of accessory pathways. The authors were able to identify thirty-three cardiac specimens with EA from their insitutional cardiac registry for morphometric analysis. At blinded gross examination, a prominent ridge was identified along the inferior AV annulus in nearly half of the specimens. In addition, when correlated with a clinical history of definite or suspected arrhythmia, the ridge was identified in 7 of 10 patients with arrhythmia versus only 3 of 14 patients without arrhythmia (70% vs 21%, p=0.03). Due to the quality of the specimens that were up to 60 years old in some cases, the results of microscopic examination in this study were limited. Although accessory muscular connections were suspected by gross examination in 17 cases, this could not be verified by light microscopy. Therefore, a direct correlation between anatomic accessory connections and the presence of a fibromuscular inferior ridge could not be established in the present investigation. As the authors point out however, in the modern era, intact specimens have become a relatively rare commodity as operative outcomes have improved and surgical repair along the true tricuspid annulus is more commonly pursued. Despite these limitations, the current study contributes greatly to our understanding of the relevant anatomy for catheter ablation of accessory connections in EA. Not only should the usual impedements to successful catheter ablation (i.e. multiple accessory connections, atrial enlargement, displaced AV system conduction, diseased atrial and ventricular myocardium) be expected, but the presence of a prominent annular ridge should be routinely anticipated. The present report also opens the door to future investigations of practical implications of the study findings. For instance, it is possible that the presence of an annular ridge may correlate with specific electrogram characteristics, broad insertions, or multiple accessory connections as often observed in EA. Specific strategies to improve outcomes, such as utilization of intracardiac echocardiography (as the authors suggest) or alternatively, use of pre-procedural advanced imaging with electroanatomical mapping data, could be routinely considered to facilitate technical success for EA. Finally, coronary angiography could be considered standard practice when the operator encounters an annular ridge eithe pre- or intra-procedurally to avoid collateral damage. Ultimately, this study may raise as many questions as it answers, but it represents a major step forward in our understanding of the anatomical correlates of electrophysiology in EA.  

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Routine Surveillance Catheterization is Useful in Guiding Management of Stable Fontan Patients. Patel ND, Sullivan PM, Sabati A, Hill A, Maedler-Kron C, Zhou S, Shillingford N, Williams R, Takao C, Badran S. Pediatr Cardiol. 2020 Jan 24. doi: 10.1007/s00246-020-02293-3. [Epub ahead of print] PMID: 31980851 Take Home Points: • Routine cardiac catheterization is a common part of Fontan surveillance algorithms. • Despite appearing to be clinically stable, a large proportion of Fontan patients in this cohort required transcatheter interventions; required initiation of pulmonary vasodilator therapy; and were identified to have hepatic fibrosis. Commentary from Dr. Konstantin Averin (Edmonton), catheterization section editor of Pediatric Cardiology Journal Watch: As survival of patients with single ventricle physiology palliated with a Fontan circulation improves it is becoming increasingly recognized that they are prone to developing significant end organ pathology. Various monitoring strategies have been proposed but all usually involve a surveillance catheterization 5-10 years after the Fontan surgery. The value of this assessment has not been fully explored. The authors sought to explore the utility of surveillance cardiac catheterization in stable Fontan patients at a single center. Sixty-three Fontan patients (mean age 14.6 ± 3.0 and mean time from Fontan surgery 11.3 ± 3.1 years) underwent cardiac catheterization over a 5-year period. The pre-catheterization echocardiograms (within a year) were unremarkable – 80% had normal ventricular function and less than mild AVV insufficiency. Baseline hemodynamics were mostly reassuring with 4 patients having a Fontan pressure > 15 mmHg and 19 having a PVRi > 2.0 iWu. Vasoreactivity testing with iNO was performed in 53 and half had a reduction in the PVRi of at least 20%. Fifty-seven patients underwent a trans-jugular liver biopsy at the time of procedure with more than half (32/57) demonstrating higher grades of congestive hepatic fibrosis. Forty percent of patients had an intervention at the time of the procedure: pulmonary artery stent (16), existing stent dilation (5), SVC angioplasty (4), Fontan stent (3), and others (7). There were no major complications. Despite the limitations of this work, the authors demonstrate that routine invasive assessment of Fontan patients can provide valuable information and allow for optimization of the Fontan circulation. Given the high morbidity experienced by this patient population it is important for providers to be pro-active in longitudinal Fontan assessment management as a stable Fontan is not necessarily a good Fontan

Routine Surveillance Catheterization is Useful in Guiding Management of Stable Fontan Patients. Patel ND, Sullivan PM, Sabati A, Hill A, Maedler-Kron C, Zhou S, Shillingford N, Williams R, Takao C, Badran S. Pediatr Cardiol. 2020 Jan 24. doi:...

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Imaging of the pulmonary vasculature in congenital heart disease without gadolinium contrast: Intraindividual comparison of a novel Compressed SENSE accelerated 3D modified REACT with 4D contrast-enhanced magnetic resonance angiography

Imaging of the pulmonary vasculature in congenital heart disease without gadolinium contrast: Intraindividual comparison of a novel Compressed SENSE accelerated 3D modified REACT with 4D contrast-enhanced magnetic resonance angiography. Pennig L, Wagner A, Weiss K,...

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