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.

Arrhythmic Risk in Paediatric Patients Undergoing Surgical Repair for Pulmonary Atresia with Intact Ventricular Septum

Introduction:While previous studies only focused on the arrhythmic risk associated with specific correction strategies,this study evaluates this risk in a large cohort of paediatric patients with all phenotypes of PA-IVS after surgical repair.Methods:In this single centre observational cohort study,we retrospectively evaluated 165 patients with a diagnosis of PA-IVS and we excluded those with an exclusively percutaneous treatment,patients lost or with insufficient follow-up and those affected by other arrhythmic syndromes.Surgical history and clinical outcomes were reviewed.Results:86 patients were included in the study(54 male[62.8%],mean age 16.4±6.1 years),with median follow-up from definitive repair of 12.8 years(6.4–18.9 years).They underwent three different final repairs:23 patients(26.7%)univentricular palliation,43(50%)biventricular correction,and 20(23.3%)one and a half ventricle correction.Thirteen patients(15%)developed arrhythmia:6 patients(all the subgroups)sinus node disfunction(SND);2(biventricular repair)premature ventricular complexes;2(one and a half ventricle repair)non-sustained ventricular tachycardia;1(biventricular repair)intra-atrial re-entrant tachycardia;1(one and a half ventricle repair)supraventricular tachyarrhythmia;1(biventricular repair)atrial fibrillation.Three patients with SND needed a pacemaker implantation.Only Fontan circulation showed an association with SND,while the other two groups heterogeneous types of arrhythmias.Conclusions:The low arrhythmic risk is related to surgical repair,it does not appear to be associated with native cardiomyopathy,and it appears to increase with length of follow up.Continuous follow-up in specialized centres is necessary to make an early diagnosis and to manage the potential haemodynamic impact at medium-long term.

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.