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.

Fetal Bradyarrhythmias:Etiopathogenesis,Diagnosis and Treatment:Between Literature Review and Experience of a Tertiary Center

Fetal arrhythmias reach up around 10%of the total third-level perinatal cardiology references.Sustained bradycardia is defined as a baseline fetal heart rate(FHR)of less than 110 bpm sustained for at least 10 min.The overall incidence of malignant fetal bradyarrhythmias,such as complete atrioventricular block(AVB)and channellopathies,is relatively rare,1:5000 pregnancies,but represents a serious emergency for the gynecologist,neonatologists,and pediatric cardiologists.Fetal complete AVB is strongly associated with maternal connective tissue disease,but it can be also associated with congenital heart disease and usually with a poorer prognosis with high risk of fetal hydrops and abortion.Currently,the treatment of severe fetal bradyarrhythmias is principally pharmacological and aims to increase the FHR,besides an early resolution of underlying causes,when possible,and a promptly management of fetal heart failure.Intrauterine electrostimulation nowadays is an experimental pioneering method,reserved for limited selected cases.

Arrhythmias in Common Arterial Trunk(CAT):Uncommon Atrial Tachycardia in CAT with Anomalous Pulmonary Venous Connection and Re-entry Atrial Tachycardia in CAT with HIV Seropositive Mother

We show a brief report of two common arterial trunk cases(CAT)with different arrhythmias and discuss anatomy,clinical and diagnostic management.The burden of volume and pressure overload of this cardiac malformation may predispose to different types of arrhythmia before and after surgical repair.Because of labile hemodynamic state in this group of patients,prompt diagnosis of any arrhythmia is mandatory as the devastating factor on prognosis.The first patient with a diagnosis of CAT Type II Collett and Edwards(CE)had a particular history with HIV seropositive mother assuming antiretroviral therapy during pregnancy,who presented hyperbilirubinemia and liver dysfunction at birth,and re-entry atrial tachycardia after repair.The second patient had CAT Type I CE with a partial anomalous venous connection of left superior pulmonary vein and uncommon type of atrial tachycardia with dual AV nodal physiology.

Management of“Wall to Wall Heart”in a Transient Neonatal Tricuspid Regurgitation

We present a case of a one-day-old newborn,without prenatal diagnosis,referred to our cardiologic intensive care unit in critical condition presenting sub-cyanosis and peripheral oxygen saturation of 80%.Echocardiography diagnosis was tricuspid valve dysplasia with severe regurgitation,functional pulmonary valve atresia with intact ventricular septum and reversal flow in the large patent ductus arteriosus(PDA).Chest X-ray showed severe cardiomegaly and wall to wall heart.Prostaglandin E1 infusion was started once after birth.After few days,clinical conditions progressively worsened because of right heart failure;a first pharmacological approach to close PDA failed and surgery ligation of PDA was necessary to restore anterograde pulmonary flow and heart size.