Mid-Term Results of Pulmonary Valve-Sparing Repair for Tetralogy of Fallot With Pulmonary Stenosis.

Objective: The transannular patch remains the most common procedure performed for patients with Tetralogy of Fallot (TOF) with pulmonary stenosis. Pulmonary regurgitation has a negative impact on early and late outcomes. To address this issue pulmonary valve-sparing repair (PVSR) has been developed.

Short-Term Results With Ozaki Valved Conduit-A Simple Solution for Patients Needing Right Ventricle to Pulmonary Artery Conduit in a Low-Resource Setting.

Background: The repair of certain types of complex congenital cardiac defects may require a right ventricle-pulmonary artery (RV-PA) conduit. Using the Ozaki Aortic valve neocuspidization (AVNeo)technique, a valved RV-PA conduit was constructed with an Ozaki valve inside a Dacron graft. This study aims to evaluate the short-term outcome of the Ozaki valved RV-PA conduit.

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.

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.

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.

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.

Visualization of the arterial valve morphology and number of leaflets in a fetus with common arterial trunk by spatiotemporal image correlation imaging.

Spatiotemporal imaging correlation (STIC) technology has been employed to visualize the fetal heart for close to two decades, but the additional value of the technology remains debatable. The value of the technology in identifying the morphology of the cardiac valves is being recognized. We report a 21-week gestational age fetus with common arterial trunk where STIC imaging enabled us to identify a bicuspid arterial valve.

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.

Familial left ventricular noncompaction cardiomyopathy due to a novel mutation in the MYH 7 gene.

Left Ventricular Non Compaction (LVNC) is considered a unique cardiomyopathy according to the American Heart Association guidelines. The genetic ethology of LVNC in children is not completely understood although upto 41% of LVNC are thought to be genetic. We report a family with LVNC due to a novel mutation in the MYH 7 gene.

Septal defect with polyvalvular involvement: A cardiac imaging hallmark of Trisomy 18.

Congenital Heart Diseases occur in close to 90% of children with Trisomy 18. A ventricular septal defect along with abnormalities of more than one cardiac valve is considered to be an imaging hallmark of Trisomy 18. We present echocardiographic images of an infant with Trisomy 18 who had a large ventricular septal defect and abnormalities of all cardiac valves.

Further clarification on the variants of double-outlet right atrium.

Uniatrial but biventricular atrioventricular connection is a rare congenital cardiac abnormality where the left atria-ventricular connection is absent and the right atrio-ventricular connection straddles the crest of the muscular ventricular septum. This anomaly has been referred to as double outlet right atrium and the atrio-ventricular valve as a common atrioventricular valve in the past. In the absence of a primary atrial septal defect, the atrio-ventricular junction is not a common junction and the valve cannot hence be described as a common trio-ventricular valve.

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.

2021 PACES Expert Consensus Statement on the Indications and Management of Cardiovascular Implantable Electronic Devices in Pediatric Patients: Developed in collaboration with and endorsed by the Heart Rhythm Society (HRS), the American College of Cardiology (ACC), the American Heart Association (AHA), and the Association for European Paediatric and Congenital Cardiology (AEPC) Endorsed by the Asia Pacific Heart Rhythm Society (APHRS), the Indian Heart Rhythm Society (IHRS), and the Latin American Heart Rhythm Society (LAHRS).

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.

Peak left atrial longitudinal strain: A potential diagnostic entity in children with multi-inflammatory syndrome in children.

The multi-inflammatory syndrome in children is a poorly understood febrile illness potentially linked to an immune response to COVID-19 infection. The disease is characterized by fever and elevated acute-phase reactants. A number of children with clinical and laboratory evidence of cardiovascular involvement have normal echocardiograms by conventional assessment.

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.