Right ventricular outflow tract landing zone perimeter / circularised diameter - new imaging standards in pulmonary valve replacement reporting.

Background: Right ventricular outflow tract intervention spans transcatheter, surgical, or hybrid pulmonary valve replacement methodologies. Standardised pre-procedure workup includes cardiac MRI to identify an intended valve site (landing zone). Our institutional practice includes measurement of the right ventricular outflow tract perimeter (circumference) of this site in end-systole.

Nomenclature for Pediatric and Congenital Cardiac Care: Unification of Clinical and Administrative Nomenclature - The 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Revision of the International Classification of Diseases (ICD-11).

Substantial progress has been made in the standardization of nomenclature for paediatric and congenital cardiac care. In 1936, Maude Abbott published her Atlas of Congenital Cardiac Disease, which was the first formal attempt to classify congenital heart disease. The International Paediatric and Congenital Cardiac Code (IPCCC) is now utilized worldwide and has most recently become the paediatric and congenital cardiac component of the Eleventh Revision of the International Classification of Diseases (ICD-11).

Nomenclature for Pediatric and Congenital Cardiac Care: Unification of Clinical and Administrative Nomenclature - The 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Revision of the International Classification of Diseases (ICD-11).

Substantial progress has been made in the standardization of nomenclature for paediatric and congenital cardiac care. In 1936, Maude Abbott published her Atlas of Congenital Cardiac Disease, which was the first formal attempt to classify congenital heart disease. The International Paediatric and Congenital Cardiac Code () is now utilized worldwide and has most recently become the paediatric and congenital cardiac component of the Eleventh Revision of the International Classification of Diseases (ICD-11).

Novel Loss of Function in the Gene: Rare Cause of End-Stage Heart Failure.

The authors present a case of mitochondrial cardiomyopathy due to a novel mutation of gene that led to progressive heart failure. The cardiac magnetic resonance image findings of diffusely elevated relaxation time and increase in extracellular volume in the myocardium without early or late gadolinium enhancement may suggest mitochondrial cardiomyopathy. The authors emphasized the multidisciplinary team approach in the care of patients with mitochondrial cardiomyopathies.

Global Left Ventricular Relaxation: A Useful Echocardiographic Marker of Heart Transplant Rejection and Recovery in Children.

Background: Tissue Doppler velocities are impaired after heart transplantation and further diminished in acute rejection.

Methods: Left ventricular relaxation index (LVRI) was calculated as the sum of E' of the left ventricular lateral, septal and posterior walls divided by left ventricular posterior wall (LVPW) thinning (LVRI = E' lateral + E' septal + E' posterior/[systolic LVPW - diastolic LVPW/systolic LVPW]). On the basis of a prior study, LVRI > 0.

Myocardial stress perfusion magnetic resonance in children with hypertrophic cardiomyopathy.

Background: Microvascular dysfunction in hypertrophic cardiomyopathy has been associated with poor clinical outcome. Several studies have demonstrated a reduced perfusion reserve proportional to the magnitude of the hypertrophy. We investigated the utility of stress perfusion cardiac MRI to detect microvascular dysfunction in children with hypertrophic cardiomyopathy.

Synthetic hematocrit derived from the longitudinal relaxation of blood can lead to clinically significant errors in measurement of extracellular volume fraction in pediatric and young adult patients.

Background: Extracellular volume fraction (ECV) is altered in pathological cardiac remodeling and predicts death and arrhythmia. ECV can be quantified using cardiovascular magnetic resonance (CMR) T1 mapping but calculation requires a measured hematocrit (Hct). The longitudinal relaxation of blood has been used in adults to generate a synthetic Hct (estimate of true Hct) but has not been validated in pediatric populations.

Global left ventricular relaxation: A novel tissue Doppler index of acute rejection in pediatric heart transplantation.

Background: Serial invasive cardiac catheterization with endomyocardial biopsies (EMBs) is the current standard of reference for evaluation after orthotopic heart transplant (OHTx). We developed a novel, non-invasive echocardiographic index of global left ventricular relaxation (LVRI) and assessed its sensitivity, specificity and predictive value for detecting rejection compared with EMB results in a prospective study conducted from September 2012 through May 2014.

Methods: LVRI was calculated as the sum of diastolic tissue Doppler imaging (TDI) velocities (E') of the left ventricular lateral, septal and posterior walls divided by the percentage of left ventricular posterior wall (LVPW) thinning by M-mode.

The Right Subclavian Artery Arising as the First Branch of a Left-Sided Aortic Arch.

We describe an unusual pattern of branching of a left-sided aortic arch in which the first branch is the right subclavian artery, followed by the common carotid arteries arising from a common trunk. The patient was born with transposition (concordant atrioventricular and discordant ventriculoarterial connections) and had 18q23 deletion. We discuss the implication of these anatomic findings in the light of inferences currently made by echocardiographers when the first branch of the aortic arch fails to bifurcate.

Crossed pulmonary arteries with hypoplasia of the transverse aortic arch.

Background: The entity of crossed pulmonary arteries was first described by Jue, Lockman, and Edwards in 1966, in a patient with trisomy 18. Since then, several series have been described, both in terms of the isolated anatomic variant, or its association with other intracardiac or extracardiac anomalies. We describe a rare association that has previously not been reported.

Takotsubo cardiomyopathy: how much do we know of this syndrome in children and young adults?

Since Takotsubo cardiomyopathy was first described by Sato in 1990, multiple cases have been reported, but only few in children, among whom this type of cardiomyopathy is to some extent underappreciated. A series of children with this syndrome were therefore reviewed, drawing comparison with cases reported by others. The review addresses the current challenges in diagnosis, presentation, triggers, clinical course, management, and possible pathogenic mechanisms of the entity.

Atrioventricular septal defect with an imperforate right-sided component of the common atrioventricular valve: anatomic and embryologic considerations.

We describe an atypical case of an atrioventricular septal defect with a common atrioventricular junction in which the right-sided component of the common atrioventricular valve was imperforate, producing tricuspid atresia with a severely hypoplastic right ventricle and an ostium primum defect. We discuss the implications of the anatomic findings with regard to concepts of cardiac development, drawing a comparison with similar cases previously reported.

Takotsubo cardiomyopathy: an unusual cardiomyopathy at an unusual age.

We describe an atypical presentation of stress-induced cardiomyopathy - Takotsubo cardiomyopathy - in a 16-month-old boy previously diagnosed with cyclic vomiting and episodic hypertension. He developed features of cardiac failure and his echocardiogram showed left ventricular wall motion abnormality accompanied with elevated cardiac enzymes. Cardiac catheterisation showed no coronary arterial abnormality.