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Congenital heart disease (CHD) affects about 1% of live births. Among them, about 20% will undergo one or more surgical or percutaneous maneuvers on the right ventricle outflow tract or pulmonary valve. Transcatheter pulmonary valve implantation is a recently available less invasive alternative to surgery for treatment of right ventricular outflow tract dysfunction. Thus, residual dysfunction can be treated early and with a lower risk profile. This narrative review aimed to describe the state of the art of percutaneous pulmonary valve implantation. [ABSTRACT FROM AUTHOR]

This document has been developed to provide a guide for basic and advanced reporting in paediatric echocardiography. Furthermore, it aims to help clinicians in the interpretation of echocardiographic measurements and functional data for estimating the severity of disease in different paediatric age groups. The following topics will be reviewed and discussed in the present document: (i) the general principle in constructing a paediatric echocardiographic report, (ii) the basic elements to be included, and (iii) the potential and limitation of currently employed tools used for disease severity quantification during paediatric reporting. A guide for the interpretation of Z-scores will be provided. Use and interpretation of parameters employed for quantification of ventricular systolic function will be discussed. Difficulties in the adoption of adult parameters for the study of diastolic function and valve defects at different ages and pressure and loading conditions will be outlined, with pitfalls for the assessment listed. A guide for careful use of prediction scores for complex congenital heart disease will be provided. Examples of basic and advanced (disease-specific) formats for reporting in paediatric echocardiography will be provided. This document should serve as a comprehensive guide to (i) structure a comprehensive paediatric echocardiographic report; (ii) identify the basic morphological details, measures, and functional parameters to be included during echocardiographic reporting; and (iii) correctly interpret measurements and functional data for estimating disease severity. [ABSTRACT FROM AUTHOR]

The European Heart Journal—Cardiovascular Imaging with its over 10 years existence is an established leading multi-modality cardiovascular imaging journal. Pertinent publications including original research, how-to papers, reviews, consensus documents, and in our journal from 2022 have been highlighted in two reports. Part I focuses on cardiomyopathies, heart failure, valvular heart disease, and congenital heart disease and related emerging techniques and technologies. [ABSTRACT FROM AUTHOR]

The article discusses the European Paediatric Cardiomyopathy and Myocarditis Registry, which aims to improve understanding and management of childhood-onset cardiomyopathies (CMPs). The registry provides valuable information about the complex nature of CMP and highlights the need for early diagnosis, genetic assessment, and tailored therapeutic approaches. The study also identifies the prevalence of hereditary and genetic disorders in children with CMP and emphasizes the importance of identifying rare disease phenocopies for targeted therapies. However, the study acknowledges limitations in registry studies and the need for further research to overcome obstacles to early diagnosis. Overall, the registry's insights have the potential to improve outcomes and well-being for affected children and their families. [Extracted from the article]

Children with congenital and acquired heart disease may be exposed to relatively high lifetime cumulative doses of ionizing radiation from necessary medical invasive and non-invasive imaging procedures. Although these imaging procedures are all essential to the care of these complex paediatric population and have contributed to meaningfully improved outcomes in these patients, exposure to ionizing radiation is associated with potential risks, including an increased lifetime attributable risk of cancer. The goal of this manuscript is to provide a comprehensive review of radiation dose management and cardiac computed tomography performance in the paediatric population with congenital and acquired heart disease, to encourage informed imaging to achieve indication-appropriate study quality at the lowest achievable dose. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES The role of an additional ventricle in patients with a functional single ventricle undergoing the Fontan operation has been debated due to conflicting data. Our goal was to report our experience with Fontan circulation for complex congenital heart disease, with a focus on the influence that an additional ventricular chamber may have on early and long-term clinical outcomes. METHODS We performed a retrospective clinical study including all patients undergoing the Fontan procedure between 1978 and 2019. Clinical data were retrieved from our institutional database. A 'biventricular' Fontan (BVF) was defined as that performed in a patient with single ventricle anomaly where an additional diminutive ventricular cavity was present at echocardiographic evaluation. RESULTS A total of 210 consecutive patients with functional single ventricle were included. Among these, 46 had BVF (21.9%). Early complications occurred in 42 patients (20.0%; 11 in BVF vs 31 in univentricular Fontan; P  = 0.53) There were 18 early deaths (8.6%) with no difference between the groups. At a median follow-up of 12.7 years (interquartile range 5.4–20.7), there were no significant differences in late mortality, whereas cardiac rhythm disturbances resulted more frequently in univentricular Fontan (P  = 0.018). Statistical analysis showed an equal distribution of BVF across time (P  = 0.620), and there were no significant differences in terms of early and late survival (P  = 0.53 and P  = 0.72, respectively) or morbidity (P  = 0.45 and P  = 0.80, respectively). CONCLUSIONS A secondary ventricle in Fontan circulation is not significantly related to any clinical disadvantage in terms of survival or onset of complications. However, the immediate postoperative course may be influenced negatively by the presence of an additional secondary ventricle. [ABSTRACT FROM AUTHOR]

The right ventricle ( RV) is of lesser importance in acquired heart disease, but its role is of increasing importance in congenital heart disease ( CHD). Despite major progress being made, precise measurements of the RV are challenging because of its peculiar anatomical structure that is not adaptable to any planar geometrical assumption. This is particularly true in adult patients with CHD where the RV shape eludes any standardization, it may assume various morphologies, and its modality of contraction depends on previous surgical treatment and/or pathophysiological conditions. However, reliable and repeatable quantification of RV dimensions and function for these patients are essential to provide appropriate timing for intervention to optimize outcomes. In this population, echocardiographic evaluation should not be limited to an observational and subjective functional assessment of the RV but must provide quantitative values repeatable and clinically reliable to help the decision-making process. The aim of this review was to discuss the echocardiographic approach to the RV in adult patients with CHD in general and in specific cases of pressure or volume overload. [ABSTRACT FROM AUTHOR]