Differences between genetic dilated cardiomyopathy and myocarditis in children presenting with severe cardiac dysfunction.

Acute myocarditis is an inflammatory disease of the myocardium, and it can present as severe heart failure in children. Differential diagnosis with genetic cardiomyopathy can be difficult. The objective of this study is to identify patterns of clinical presentation and to assess invasive and non-invasive measures to differentiate patients with acute myocarditis from patients with dilated genetic cardiomyopathy. We performed a retrospective descriptive study of all paediatric patients (0-16 years old) that presented with new-onset heart failure with left ventricle ejection fraction < 35% in whom we performed an endomyocardial biopsy (EMB) during the period from April 2007 to December 2020. The patients were classified into two groups: Group 1 included 18 patients with myocarditis. Group 2 included 9 patients with genetic cardiomyopathy. Findings favouring a diagnosis of myocarditis included a fulminant or acute presentation (77.8% vs 33.3%, p = 0.01), higher degree of cardiac enzyme elevation (p = 0.011), lower left ventricular dimension z-score (2.2 vs 5.4, p = 0.03) increase of ventricular wall thickness (88.8% vs 33.3%, p = 0.03) and oedema in the EMB. Seven (77.8%) patients with genetic cardiomyopathy had inflammation in the endomyocardial biopsy fulfilling the diagnostic criteria of inflammatory cardiomyopathy.Conclusion: Differentiating patients with a myocarditis from those with genetic cardiomyopathy can be challenging, even performing an EMB. Some patients with genetic cardiomyopathy fulfil the diagnostic criteria of inflammatory cardiomyopathy. Using invasive and non-invasive measures may be useful to develop a predictive model to differentiate myocarditis from genetic cardiomyopathy. What is Known: • Acute myocarditis could present with cardiogenic shock in paediatric patients. • Parvovirus B19 is the main cause of myocarditis in this population. What is New: • Current diagnostic criteria for myocarditis have limited use in paediatric patients presenting with new-onset heart failure. • Some patients with a genetic cardiomyopathy and a new-onset heart failure fulfill the diagnostic criteria of inflammatory cardiomyopathy.

Graves' disease TSHR-stimulating antibodies (TSAbs) induce the activation of immature thymocytes: a clue to the riddle of TSAbs generation?

Graves' disease (GD) is an autoimmune thyroid disease defined by the production of stimulating autoantibodies to the thyroid-stimulating hormone receptor (TSHR) (TSAbs) that induce a sustained state of hyperthyroidism in patients. We previously demonstrated that TSHR, the target of this autoimmune response, is also a key susceptibility gene for GD, probably acting through thymic-dependent central tolerance. We also showed that TSHR is, unexpectedly, expressed in thymocytes. In this report, we confirm the expression of TSHR in thymocytes by protein immunoblotting and quantitative PCR, and show that expression is confined to maturing thymocytes. Using functional assays, we show that thymic TSHR is functional and that TSAbs can stimulate thymocytes through this receptor. This new activity of TSAbs on thymocytes may: 1) explain GD-associated thymic enlargement (hyperplasia), and 2) suggest the provocative hypothesis that the continuous stimulation of thymocytes by TSAbs could lead to a vicious cycle of iterative improvement of the affinity and stimulating capability of initially low-affinity antibacterial (e.g., Yersinia) Abs cross-reactive with TSHR, eventually leading to TSAbs. This may help to fill one of the gaps in our present understanding of unusual characteristics of TSAbs.

Usefulness of transaortic approach for a complex double-outlet right ventricle.

Surgical procedures for double-outlet right ventricle with ventricular septal defect are based on rerouting the blood flow of the left ventricle to the aorta through the ventricular septal defect (VSD) with an intraventricular baffle. The right atriotomy is the most common approach combined with a right ventriculotomy in some cases, particularly in pulmonary stenosis association. However, in complex cases, this standard operative strategy may not provide an adequate exposure. We describe the transaortic approach as an alternative procedure to repair a complex case of double-outlet right ventricle (DORV) with subaortic stenosis. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s12055-021-01261-7.

Case report: Removal of a subcutaneous implantable cardiac defibrillator in a pediatric patient with hypertrophic cardiomyopathy after a septal myectomy. Insights on current indications of type of ICD in children with hypertrophic cardiomyopathy and left ventricular tract obstruction.

Hypertrophic cardiomyopathy is a heart muscle disease with an annual incidence between 0.24 and 0.47/100000 in childhood. Sudden cardiac death is the most common cause of death in this population. Although some medical treatment can decrease the risk of sudden cardiac death, implantable cardioverter defibrillator continues to be the most reliable treatment. Different types of devices and programming strategies can be used in patients with hypertrophic cardiomyopathy depending on each center and specific patient condition. We report a pediatric patient affected with hypertrophic cardiomyopathy who had and ICD implantation in primary prevention. Four years later he developed symptomatic left ventricular outflow tract obstruction and a surgical septal myectomy was performed. After the myectomy the patient developed complete left bundle branch block on his 12 lead ECG, and unfortunately none of the S-ICD vectors were suitable after the myectomy and it had to be explanted and replaced for a new transvenous ICD.

Transient upper limb ischaemia during veno-arterial extracorporeal membrane oxygenation in a child.

Neck cannulation is the most common cannulation strategy performed to provide veno-arterial extracorporeal membrane oxygenation support in paediatric patients, especially in small children. Upper limb ischaemia is a rare complication of neck cannulation and is likely caused by arterial cannula malposition. We describe a case of right arm ischaemia caused by extrinsic compression of the right subclavian artery by the venous drainage cannula inserted through the right internal jugular vein. Upper limb hypoperfusion was resolved immediately after changing the venous drainage cannula from the right jugular vein to the right femoral vein.

Pediatric Tricuspid Valve Replacement With Transcatheter Bioprosthetic Valve: An Alternative Option in High-Risk Patients.

Transcatheter valve can be an alternative option of pediatric valve replacement in high-risk patients. We present 2 cases of Edwards Sapien 3 implantation in tricuspid position.

Myocardial protection in the arterial switch operation: Custodiol versus cold blood cardioplegia.

OBJECTIVES: The optimal myocardial protective solution in the neonatal arterial switch operation remains controversial. The aim of this study was to demonstrate that Bretschneider's histidine-tryptophan-ketoglutarate crystalloid solution (Custodiol) offers protection at least similar to that of cold blood cardioplegia. METHODS: Patients who underwent the neonatal arterial switch operation with Custodiol between January 2016 and December 2018 (n = 23) were compared with an historical cohort from August 2010 to December 2015 in which cold blood cardioplegia was used (n = 41). A linear mixed-effect model for repeated measures was performed to test the recovery of myocardial function based on inotropic and vasoactive inotropic scores, cardiac enzyme release and left ventricular ejection fraction. RESULTS: Patients in the cold blood cardioplegia group had higher inotropic scores in the first 24 h (0 h, P = 0.001 and 24 h, P = 0.006) and higher vasoactive inotropic scores in the first 72 h (0 h, 24 h and 48 h, P < 0.001; 72 h, P = 0.012). Cardiac troponin-I concentrations were higher in the cold blood cardioplegia group at postoperative hours 1-72 (1 h, 6 h, 12 h and 24 h, P < 0.001; 48 h, P = 0.001 and 72 h, P = 0.003). Creatinine-kinase-MB concentrations were higher in the cold blood cardioplegia group at postoperative hours 1-24 (1 h, 6 h and 12 h, P < 0.001; 24 h, P = 0.042). The left ventricular ejection fraction was higher in the Custodiol group just after the operation (P = 0.005), at 24 h (P = 0.001) and on the first day without inotropic support (P = 0.011). CONCLUSIONS: Neonatal myocardium protected with Custodiol during the arterial switch operation presented optimal ventricular function recovery with less inotropic support and less myocardial damage compared with cold blood cardioplegia.

Late Dissection of a Contegra Conduit: A Rare Complication.

Surgical palliation of many types of congenital heart defects requires the use of a conduit between the right ventricle and the pulmonary artery. Dissections of these conduits are very infrequent. We report a case of a ten-year-old girl who developed acute right heart failure related to a Contegra conduit dissection. She underwent a new conduit replacement on an emergency basis.

Selective visceral perfusion improves renal flow and hepatic function in neonatal aortic arch repair.

OBJECTIVES: The aortic arch repair in the neonatal period is a complex procedure with significant morbidity. We define a useful double-perfusion technique and its effect on the function of abdominal organs in the postoperative course. METHODS: Nine patients with double perfusion (Group 1) were compared with 14 patients with antegrade cerebral perfusion (Group 2). The objective was to discern the incidence of postoperative acute kidney injury and impaired hepatic function, as well as tissue perfusion and myocardial function parameters. Mechanical ventilation time, postoperative length of stay and 30-day mortality were measured. We excluded patients with extracorporeal membrane oxygenation, early mortality (<72 h) and preoperative renal or hepatic insufficiency. RESULTS: Nine (39%) patients developed postoperative acute kidney injury, with 22% (n = 2) in Group 1 and 50% (n = 7) in Group 2 (P = 0.183). A higher urine output was observed during the first 24 h for Group 1 (P = 0.032). Eleven patients developed impaired hepatic function in the immediate postoperative period: 2 (18.2%) in Group 1 and 9 (81.8%) in Group 2 (P = 0.04). The international normalized ratio (P = 0.006-0.031) and prothrombin time (P = 0.007-P = 0.016) were significantly lower in the double-perfusion group during the first 72 h. Significant difference was observed in lactate levels in the first 72 h (P = 0.001-0.009). There was no postoperative mortality in either group. CONCLUSIONS: Selective visceral perfusion is a safe procedure that provides a better urine output, hepatic function and tissue perfusion. This technique allows for the repair of complex aortic arch anomalies in neonates without deep hypothermic circulatory arrest.

Corrección de defectos cardiacos congénitos con la matriz extracelular CorMatrix en pacientes pediátricos. ¿Es realmente seguro? / Correction of congenital cardiac defects with CorMatrix extracellular matrix in pediatric patients: is it really safe?

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