An unusual form of "haemodynamic vise" in supra cardiac totally anomalous pulmonary venous drainage.

We report an unusual variant of obstructed supra cardiac anomalous pulmonary venous drainage where the vertical vein is obstructed by a vice formed between the persistent arterial duct and the left pulmonary artery.

Homozygous variants in the GDF1 gene related to recurrent right isomerism and complex CHD in two Indian families.

Disorders of laterality are often associated with complex CHD. There is considerable debate about the appropriate terminology to describe these conditions. As our understanding of the genetic basis of these disorders improves, it is likely that terminology will be dictated by the genetic aetiology. The genetic basis of laterality disorders in the Indian population has not been studied. We report two families with autosomal recessive inheritance of isomerism and homozygous variants in the GDF1 gene in affected family members.

Tricuspid Valve Dysplasia at Fetal Autopsy.

Introduction: Tricuspid valve abnormalities detected in fetal life include Ebstein anomaly and tricuspid valve dysplasia. The differentiation between these 2 entities can sometimes be challenging in the 2nd trimester fetus. We report a case of tricuspid valve dysplasia diagnosed on fetal autopsy. Case Report: A primigravida was diagnosed at 22 weeks' gestation to have Ebstein anomaly with severe tricuspid regurgitation. There was intra-uterine fetal demise. On fetal autopsy, the tricuspid valve leaflets were not apically displaced and the leaflets were nodular with rolled up edges. This supported a diagnosis of tricuspid valve dysplasia. Conclusion: The difficulties in differentiating Ebstein anomaly from tricuspid valve dysplasia due to inherent limitations in fetal imaging can be resolved by fetal autopsy. Valvular dysplasia will not have apical displacement of the valve leaflets.

Heterotaxy - Res ipsos loquitur.

The essence of so-called heterotaxy is the potential disharmony between the arrangement of the bronchuses, abdominal organs, and the atrial appendages. Accurate description of the heart, however, can only be provided by specific description of these features, all of which are readily discernible in the clinical setting. We argue that, when accurate description of the atrial and visceral arrangement is provided, along with appropriate description of the intracardiac findings, no further accuracy is gained by suggesting that an individual heart is "heterotaxic".

2021 PACES expert consensus statement on the indications and management of cardiovascular implantable electronic devices in pediatric patients.

In view of the increasing complexity of both cardiovascular implantable electronic devices (CIEDs) and patients in the current era, practice guidelines, by necessity, have become increasingly specific. This document is an expert consensus statement that has been developed to update and further delineate indications and management of CIEDs in pediatric patients, defined as ≤21 years of age, and is intended to focus primarily on the indications for CIEDs in the setting of specific disease categories. The document also highlights variations between previously published adult and pediatric CIED recommendations and provides rationale for underlying important differences. The document addresses some of the deterrents to CIED access in low- and middle-income countries and strategies to circumvent them. The document sections were divided up and drafted by the writing committee members according to their expertise. The recommendations represent the consensus opinion of the entire writing committee, graded by class of recommendation and level of evidence. Several questions addressed in this document either do not lend themselves to clinical trials or are rare disease entities, and in these instances recommendations are based on consensus expert opinion. Furthermore, specific recommendations, even when supported by substantial data, do not replace the need for clinical judgment and patient-specific decision-making. The recommendations were opened for public comment to Pediatric and Congenital Electrophysiology Society (PACES) members and underwent external review by the scientific and clinical document committee of the Heart Rhythm Society (HRS), the science advisory and coordinating committee of the American Heart Association (AHA), the American College of Cardiology (ACC), and the Association for European Paediatric and Congenital Cardiology (AEPC). The document received endorsement by all the collaborators and the Asia Pacific Heart Rhythm Society (APHRS), the Indian Heart Rhythm Society (IHRS), and the Latin American Heart Rhythm Society (LAHRS). This document is expected to provide support for clinicians and patients to allow for appropriate CIED use, appropriate CIED management, and appropriate CIED follow-up in pediatric patients.

2021 PACES expert consensus statement on the indications and management of cardiovascular implantable electronic devices in pediatric patients: Executive summary.

Guidelines for the implantation of cardiac implantable electronic devices (CIEDs) have evolved since publication of the initial ACC/AHA pacemaker guidelines in 1984 [1]. CIEDs have evolved to include novel forms of cardiac pacing, the development of implantable cardioverter defibrillators (ICDs) and the introduction of devices for long term monitoring of heart rhythm and other physiologic parameters. In view of the increasing complexity of both devices and patients, practice guidelines, by necessity, have become increasingly specific. In 2018, the ACC/AHA/HRS published Guidelines on the Evaluation and Management of Patients with Bradycardia and Cardiac Conduction Delay [2], which were specific recommendations for patients >18 years of age. This age-specific threshold was established in view of the differing indications for CIEDs in young patients as well as size-specific technology factors. Therefore, the following document was developed to update and further delineate indications for the use and management of CIEDs in pediatric patients, defined as ≤21 years of age, with recognition that there is often overlap in the care of patents between 18 and 21 years of age. This document is an abbreviated expert consensus statement (ECS) intended to focus primarily on the indications for CIEDs in the setting of specific disease/diagnostic categories. This document will also provide guidance regarding the management of lead systems and follow-up evaluation for pediatric patients with CIEDs. The recommendations are presented in an abbreviated modular format, with each section including the complete table of recommendations along with a brief synopsis of supportive text and select references to provide some context for the recommendations. This document is not intended to provide an exhaustive discussion of the basis for each of the recommendations, which are further addressed in the comprehensive PACES-CIED document [3], with further data easily accessible in electronic searches or textbooks.

2021 PACES expert consensus statement on the indications and management of cardiovascular implantable electronic devices in pediatric patients.

In view of the increasing complexity of both cardiovascular implantable electronic devices (CIEDs) and patients in the current era, practice guidelines, by necessity, have become increasingly specific. This document is an expert consensus statement that has been developed to update and further delineate indications and management of CIEDs in pediatric patients, defined as ≤21 years of age, and is intended to focus primarily on the indications for CIEDs in the setting of specific disease categories. The document also highlights variations between previously published adult and pediatric CIED recommendations and provides rationale for underlying important differences. The document addresses some of the deterrents to CIED access in low- and middle-income countries and strategies to circumvent them. The document sections were divided up and drafted by the writing committee members according to their expertise. The recommendations represent the consensus opinion of the entire writing committee, graded by class of recommendation and level of evidence. Several questions addressed in this document either do not lend themselves to clinical trials or are rare disease entities, and in these instances recommendations are based on consensus expert opinion. Furthermore, specific recommendations, even when supported by substantial data, do not replace the need for clinical judgment and patient-specific decision-making. The recommendations were opened for public comment to Pediatric and Congenital Electrophysiology Society (PACES) members and underwent external review by the scientific and clinical document committee of the Heart Rhythm Society (HRS), the science advisory and coordinating committee of the American Heart Association (AHA), the American College of Cardiology (ACC), and the Association for European Paediatric and Congenital Cardiology (AEPC). The document received endorsement by all the collaborators and the Asia Pacific Heart Rhythm Society (APHRS), the Indian Heart Rhythm Society (IHRS), and the Latin American Heart Rhythm Society (LAHRS). This document is expected to provide support for clinicians and patients to allow for appropriate CIED use, appropriate CIED management, and appropriate CIED follow-up in pediatric patients.

Massive parallel sequencing of dried umbilical cord remnants.

Genetic diagnosis depends on having available tissue to test. This can be important for many reasons, such as related to familial diagnosis in the case of another pregnancy. When blood or DNA samples from affected family members are not available, accurate prenatal diagnosis may be much more difficult and hence additional effort may be needed to obtain a genetic diagnosis in such families. We report two families with suspected monogenic disorders where attempts were made to establish the genetic etiology in deceased offspring using dried umbilical cord remnants which had been preserved by the family.

Catheter-based palliation for infants with tetralogy of Fallot.

BACKGROUND: The optimal management of symptomatic tetralogy of Fallot in neonates and younger infants with unfavourable anatomy is unclear and is further constrained by resource limitations in low and middle income countries. METHODS: Retrospective medical record review of infants with tetralogy of Fallot undergoing corrective or palliative procedures between January 2016 and June 2019. RESULTS: The study included 120 infants; of whom 83 underwent primary complete repair, four underwent surgical palliation, and 33 underwent catheter-based palliation, including balloon pulmonary valvuloplasty (n = 18), right ventricular outflow tract stenting (n = 14), and stenting of the patent arterial duct (n = 1). Infants undergoing catheter-based procedures were younger in age (median 32 days; inter-quartile range (IQR) 7-144 versus 210 days; IQR 158-250), with lower baseline saturation (65 ± 12% versus 87 ± 7%) and had smaller pulmonary artery z-scores compared to the complete repair cohort. Follow-up was available for 31/33 (94%) infants (median 7 months [IQR 4-11]) who underwent trans-catheter palliation; 12 underwent complete repair, 10 are well, awaiting repair, eight required further palliation (catheter: 6; surgical: 2), and one died post-discharge from non-cardiac causes. CONCLUSION: Catheter-based palliation is a safe and effective alternative in infants with tetralogy of Fallot who are at high risk for primary surgical repair.

Ivabradine in Post-operative Junctional Ectopic Tachycardia (JET): Breaking New Ground.

Junctional ectopic tachycardia (JET) is the commonest tachyarrhythmia in the early post-operative period in children undergoing open-heart surgery. It frequently leads to hemodynamic instability and needs to be managed aggressively. Amiodarone is the first-line agent along with non-pharmacological interventions. We report our initial experience with the use of Ivabradine in post-operative JET. A retrospective case records review of children with post-operative JET during the period from June 2018 to May 2019 was performed. Eight patients with post-operative JET were treated with Ivabradine during this period. The first patient was initially treated with Amiodarone. All eight patients responded to Ivabradine. The initial response was rate control permitting overdrive pacing. One patient had recurrence of JET 10 h after Ivabradine and after return to sinus rhythm. Amiodarone was administered along with the second dose of Ivabradine resulting in remission to sinus rhythm. Ivabradine appears to be an effective alternative to Amiodarone in children with post-operative JET based on our initial clinical experience.

Kearns Sayre Syndrome: Looking beyond A-V conduction.

A 15-year-old boy was diagnosed with Kayne Sayre Syndrome. He presented with pigmentary retinopathy, progressive ophthalmoplegia and complete heart block. He received a transvenous dual chamber pacemaker. Two years later he died suddenly while at home. This case highlights the importance of recognizing mechanisms other than heart block as a cause of sudden death in a patient with KSS.

Atrioventricular Septal Defect With Tetralogy of Fallot and Exclusive Shunting at the Ventricular Level.

Atrioventricular septal defect (AVSD) with shunting restricted to the ventricular level is a rare form of AVSD. To our knowledge, this shunting pattern has not been reported in AVSD with tetralogy of Fallot. We report a child with this unusual combination who underwent a successful single-stage repair at two years of age.

Three-dimensional spatiotemporal imaging correlation in the diagnosis of isolated infracardiac total anomalous pulmonary venous connection in fetal life.

Total anomalous pulmonary venous connection (TAPVC) is a critical congenital heart disease which is often missed on prenatal echocardiography because of the decreased pulmonary blood flow in fetal life. Improvement in technology has resulted in increasing prenatal diagnosis of this condition. We report a foetus with infra cardiac TAPVC in whom prenatal diagnosis was facilitated by the use of STIC technology.

A Rare Entity: Double Outlet of Both Ventricles.

Double outlet of both ventricles is an anomaly wherein both ventricles equally share the arterial trunks. A majority of the literature describes a variant of this disease with a muscular outlet septum which is perpendicular to the plane of the ventricular septum although a variant with a fibrous muscular septum can also be present. The condition may be associated with obstruction of either outflow tract, which may complicate repair. We report a child with double outlet of both ventricles and a fibrous outlet septum with unobstructed outflows. The child underwent a successful surgical repair using a two-patch technique.

The utility of speckle-tracking echocardiography in early and midterm follow-up after anomalous origin of the left coronary artery from the pulmonary artery repair.

Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a reversible cause of left ventricular (LV) dysfunction in infants. The LV function is expected to improve serially and return to normal by 1 year after surgical repair. The pattern of improvement in LV function has not been serially analyzed after ALCAPA repair. We report our preliminary experience with serial assessment of LV function in infants undergoing ALCAPA repair utilizing speckle tracking echocardiography.

Ivabradine monotherapy in congenital junctional ectopic tachycardia.

Congenital JET (junctional ectopic tachycardia) is a rare and often difficult to treat tachyarrhythmia in young infants. The addition of Ivabradine to standard Congenital JET therapy has been shown to improve arrhythmia control. However, Ivabradine has not been reported as a single drug in the control of congenital JET. We report a pre-term neonate in whom Ivabradine monotherapy was successful in treating congenital JET.