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Unlabelled: Atrioventricular block is classified as congenital if diagnosed in utero, at birth, or within the first month of life. The pathophysiological process is believed to be due to immune-mediated injury of the conduction system, which occurs as a result of transplacental passage of maternal anti-SSA/Ro-SSB/La antibodies. Childhood atrioventricular block is therefore diagnosed between the first month and the 18th year of life. Genetic variants in multiple genes have been described to date in the pathogenesis of inherited progressive cardiac conduction disorders. Indications and techniques of cardiac pacing have also evolved to allow safe permanent cardiac pacing in almost all patients, including those with structural heart abnormalities.Conclusion: Early diagnosis and appropriate management are critical in many cases in order to prevent sudden death, and this review critically assesses our current understanding of the pathogenetic mechanisms, clinical course, and optimal management of congenital and childhood AV block.What Is Known: • Prevalence of congenital heart block of 1 per 15,000 to 20,000 live births. AV block is defined as congenital if diagnosed in utero, at birth, or within the first month of life, whereas childhood AV block is diagnosed between the first month and the 18th year of life. As a result of several different etiologies, congenital and childhood atrioventricular block may occur in an entirely structurally normal heart or in association with concomitant congenital heart disease. Cardiac pacing is indicated in symptomatic patients and has several prophylactic indications in asymptomatic patients to prevent sudden death. • Autoimmune, congenital AV block is associated with a high neonatal mortality rate and development of dilated cardiomyopathy in 5 to 30 % cases. What is New: • Several genes including SCN5A have been implicated in autosomal dominant forms of familial progressive cardiac conduction disorders. • Leadless pacemaker technology and gene therapy for biological pacing are promising research fields. In utero percutaneous pacing appears to be at high risk and needs further development before it can be adopted into routine clinical practice. Cardiac resynchronization therapy is of proven value in case of pacing-induced cardiomyopathy. [ABSTRACT FROM AUTHOR]

The onset of right ventricular dysfunction in patients presenting with congenital heart disease is associated with a dismal long-term outcome and often represents a therapeutic dead end. Our study had several objectives: (1) to analyse the anatomical, functional, histological and cellular characteristics of an animal model of repaired tetralogy of Fallot with right ventricular dysfunction (2) to test the new electrical treatment known as cardiac contractility modulation in this animal model. Seven sheep underwent a first surgery at the age of three weeks aiming to mimic the characteristics of a repaired tetralogy of Fallot. Five controls were sham-operated. Experimental studies were performed 12 months after the initial operation. The hemodynamic, echocardiographic, and mitochondrial function studies were carried out before and after cardiac contractility modulation in closed- and open-chest conditions. In this animal model of right ventricular dysfunction, short-term cardiac contractility modulation was associated with a significant improvement in (a) right ventricular function, as evidenced by a significant increase in right ventricular dP/dt ( p < 0.05) (b) left ventricular function evidenced by the increase in left ventricular dP/dt max ( p < 0.05) (c) in mitochondrial function ( p < 0.05). In this animal model of chronic right ventricular dysfunction, cardiac contractility modulation significantly improved acute cardiac hemodynamic and mitochondrial functions of both ventricles and may represent a promising option in patients with right heart failure. [ABSTRACT FROM AUTHOR]

In this article we report the first case of Q fever endocarditis in a 13 years old child with a percutaneous pulmonary Melody valve. The patient had a new onset of Melody valve dysfunction associated with the combination of hepatosplenomegaly and pancytopenia but was afebrile. Although blood cultures were negative, we have further investigated in the direction of infective endocarditis by performing PCR detection and the serology of C. burnetii which were positive. A combination antibiotic therapy with doxycycline and hydroxychloroquine was started with good clinical evolution. Our case emphasizes the fact that any Melody valvular dysfuntion should be considered as a potential infective endocarditis despite the absence of typical bacterial features. [ABSTRACT FROM AUTHOR]

Coronary artery imaging in children is challenging, with high demands both on temporal and spatial resolution due to high heart rates and smaller anatomy. Although invasive conventional coronary angiography remains the benchmark technique, over the past 10 years, CT and MRI have emerged in the field of coronary imaging. The choice of hardware is important. For CT, the minimum requirement is a 64-channel scanner. The temporal resolution of the scanner is most important for optimising image quality and minimising radiation dose. Manufacturers have developed several modes of electrocardiographic (ECG) triggering to facilitate dose reduction. Recent technical advances have opened new possibilities in MRI coronary imaging. As a non-ionising radiation technique, MRI is of great interest in paediatric imaging. It is currently recommended in centres with appropriate expertise for the screening of patients with suspected congenital coronary anomalies. However, MRI is still not feasible in infants. This review describes and discusses the technical requirements and the pros and cons of all three techniques. [ABSTRACT FROM AUTHOR]

This report describes the case of a 6-month-old girl with a large cardiac fibroma in the right ventricle. Ventricular tachycardia associated with the fibroma was successfully treated with amiodarone. At the age of 3 years, surgical resection was indicated because of right ventricular outflow tract obstruction caused by progression of the tumor. The fibroma was successfully resected, and further follow-up evaluation was uneventful. [ABSTRACT FROM AUTHOR]

The article presents several photographs illustrating the degradation of the myofilaments in the spider cells.