Classifying Cardiac Anomalies in Right and Left Isomerism:Concordant and Discordant Patterns

Aims:Evidence is emerging that,in the setting of isomerism,the atrial and bronchial arrangement are not always concordant,nor are these patterns always harmonious with the arrangement of the abdominal organs.We aimed to evaluate the concordance between these features in a cohort of patients with cardiac malformations in the setting of known isomerism,seeking to determine whether it was feasible to assess complexity on this basis,in this regard taking note of the potential value of bronchial as opposed to appendage morphology.Methods and Results:We studied 78 patients known to have isomerism of the bronchuses,43 with right and 35 with left isomerism.Appendage anatomy could be determined in 49 cases(63%),all but one of these being concordant with bronchial anatomy.When assessing abdominal features,in only 59 cases(76%)was splenic morphology in keeping with the thoracic findings.As expected,right isomerism was associated with greater complexity of cardiac malformations,with an odds ratio of 6.53,with confidence intervals from 2.2–19.3(p<0.001).The odds were slightly decreased with thoraco-abdominal disharmony,when lesions shown to carry higher risk were then found in the setting of left isomerism.Conclusion:Harmony is excellent between bronchial and appendage isomerism,but less so with the arrangement of the abdominal organs.Right isomerism in our cohort,was indicative of a sixfold increase in intracardiac complexity.When discordance was found between the systems,however,the cardiac anomalies were less typical of the anticipated findings for right vs.left isomerism of the appendages.

3D Non-Fluoroscopic Cryoablation of Right-Sided Accessory Pathways in Children:Monocentric Study and Literature Review

Background:Cryoablation of accessory pathways(APs)is effective and very safe in children,as previously reported by our group.The aim of this retrospective study was to evaluate the current efficacy of 3D non-fluoroscopic cryoablation of right sided APs in children,comparing results obtained with the Ensite VelocityTM and the more recent Ensite PrecisionTM 3D mapping systems.Methods and Results:From January 2016 to December 2019,102 pediatric patients[mean age 12.5±2.8,62 males(61%of total cohort)]with right APs underwent 3D non-fluoroscopic transcatheter cryoablation at our Institution.Fifteen(14.7%)patients had previously undergone catheter ablation.Acute procedural success rate was 95.1%(n=97).No significant differences were detected in acute success rates achieved with Ensite Velocity^(TM)or Ensite PrecisionTM systems nor between manifest(94%)and concealed APs(100%).No permanent complications occurred.During follow-up(428±286 days,median 396 days[interquartile range 179-713]),19 patients(19.6%)had recurrences.Recurrences were more frequent for parahissian/anterior APs compared to midseptal/posterior and lateral APs(p=0.043).Recurrences were not related to the Ensite system used.A redo ablation procedure was attempted in 13 cases,11 cryoablation and 2 radiofrequency ablations:the former was successful in 10 cases out of 11(90.9%).Conclusion:3D cryoablation of right-sided APs is associated with a very high acute success rate with limited use of fluoroscopy,resulting in great benefit to the children.Recurrence rates are not high and patients can be retreated with cryo-energy with higher success rates.

Life Threatening Broad QRS Tachycardia in an Infant with Conduction Disorder and SCN5A Mutation

We present the case of an infant admitted to our department for a rapid broad complex tachycardia and cardiovascular collapse.The patient was submitted to genetic testing because of a conduction defect at baseline ECG and family history of gene mutation.A new SCN5A gene mutation variant was found leading to diagnosis of sodium-channel dysfunction arrhythmia.

139例心外Fontan术后患者最初15年的临床结局

Objectives: We sought to evaluate the mid-term outcome of hospital survivors with extracardiac Fontan circulation. Background: Few data exist about the mid-term and long-term results of the extracardiac Fontan operation. Methods: From November 1988 to November 2003, 221 patients underwent an extracardiac Fontan procedure as primary(9 patients) or secondary(212 patients) palliation, at a mean age of 72.2 months(range 13.1 to 131.3 months). A total of 165 of 193 early survivors underwent programmed noninvasive follow-up evaluations and at least one cardiac catheterization. Results: The overall survival, including operative deaths, was 85%at 15 years. Freedom from late failure among hospital survivors is 92%at 15 years. A total of 127 of 165 survivors(77%) were in New York Heart Association functional class I. The incidence of late major problems was 24%(42 major problems in 36 of 165 patients): 19 patients had arrhythmias(11%), 5 patients had obstruction of the extracardiac conduit(3%) and 6 of the left pulmonary artery(3.5%), and 5 patients experienced ventricular failure(3%), leading to heart transplantation in 3 patients. Protein-losing enteropathy was found in two patients(1%). The incidence of late re-interventions was 12.7%(21 of 165 patients, including 15 epicardial pacemaker implantations). Four patients died(2.3%), two after heart transplantation. Conclusions: After 15 years of follow-up, the overall survival, the functional status, and the cardiopulmonary performance of survivors of the extracardiac Fontan procedure compare favorably with other series of patients who underwent the lateral tunnel approach. The incidence of late deaths, obstructions of the cavopulmonary pathway, re-interventions, and arrhythmias is lower than that reported late after other Fontan-type operations.