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.

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.

Longitudinal Evaluation of Right Ventricle Function after Right Ventricle-Pulmonary Artery Shunt vs.Blalock-Taussig Shunt

Background:Still little is known about the impact on right ventricle function of the 2 main approaches to Norwood palliation in Hypoplastic left heart syndrome,the right ventricle-pulmonary artery shunt(RVPAS)and the modified Blalock-Taussig shunt(mBTS).Methods:The cohort included 27 patients with Hypoplastic left heart syndrome(10 in the mBTS group,17 in the RVPAS group).Longitudinal strain,tricuspid annulus peak systolic excursion and fractional area change were evaluated before Norwood and in four different breakpoints in the steady-state after Norwood procedure(30-days,90-days,140-days and 200-days after Norwood).Results:No significant differences were found in all standard echocardiographic functional parameters between the two groups at any times.However,when we compared right ventricle function before and after Norwood procedure,longitudinal strain significantly improved in mBTS group compared to pre-surgical assessment(after 90-days:mBTS+27,35%±43,47 vs.RVPAS-8,20%±25,25,p=0,03;after 200-days:mBTS+10.19%±36.58 vs.RVPAS-21.64%±30.43,p=0.04).Conclusion:The mBTS group,which did not receive any ventriculotomy,showed a significant increase in right ventricle longitudinal strain during convalescence.These data support the use of longitudinal strain in Hypoplastic left heart syndrome evaluation and may suggest potential value in terms of cardiac mechanics in using mBTS,preserving the right ventricle integrity.