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.

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.

Pediatrics Spanish Registry of Percutaneous Melody Pulmonary Valve Implantation in Patients Younger Than 18 Years.

INTRODUCTION AND OBJECTIVES: A decade has passed since the first Spanish percutaneous pulmonary Melody valve implant (PPVI) in March 2007. Our objective was to analyze its results in terms of valvular function and possible mid-term follow-up complications. METHODS: Spanish retrospective descriptive multicenter analysis of Melody PPVI in patients < 18 years from the first implant in March 2007 until January 1, 2016. RESULTS: Nine centers were recruited with a total of 81 PPVI in 77 pediatric patients, whose median age and weight were 13.3 years (interquartile range [IQR], 9.9-15.4) and 46kg (IQR, 27-63). The predominant cardiac malformation was tetralogy of Fallot (n = 27). Most of the valves were implanted on conduits, especially bovine xenografts (n = 31). The incidence of intraprocedure and acute complications was 6% and 8%, respectively (there were no periprocedural deaths). The median follow-up time was 2.4 years (IQR, 1.1-4.9). Infective endocarditis (IE) was diagnosed in 4 patients (5.6%), of which 3 required surgical valve explant. During follow-up, the EI-related mortality rate was 1.3%. At 5 years of follow-up, 80% ± 6.9% and 83% ± 6.1% of the patients were free from reintervention and pulmonary valve replacement. CONCLUSIONS: Melody PPVI was safe and effective in pediatric patients with good short- and mid-term follow-up hemodynamic results. The incidence of IE during follow-up was relatively low but was still the main complication.

Extended Szabo (anchor-wire) technique concept for stent implantation in congenital heart lesions.

We report the usefulness of the Szabo (anchor-wire) technique and two modifications of such based on the same concept for stent implantation in congenital heart lesions. The modifications of the original technique are related to the localization of the cell of the stent through which the anchor wire, which stops the stent advancement, is introduced: proximal in the original technique and central or distal in the reported modifications. These techniques were performed in six patients: in two to maintain permeability of the ductus arteriosus, in three to achieve a fenestration of the interatrial septum, and in one to implant a stent in a right ventricle-to-pulmonary artery conduit close to the bifurcation. We describe the technique as well as the most important difficulties and complications encountered. The Szabo or anchor-wire technique concept is a new tool for stent implantation that can provide more accurate stent positioning compared with conventional angiographically guided implantation in different congenital heart defects. As with any new tool, this technique demands a learning curve and knowledge of potential complications.

Percutaneous embolization of vascular fistulas using coils or Amplatzer vascular plugs.

INTRODUCTION AND OBJECTIVES: A great variety of different types of vascular fistula are referred to cardiac catheterization laboratories for diagnosis and percutaneous occlusion. In addition, a wide range of devices is available for treating them percutaneously. The objectives of this study were to assess the usefulness and difficulty of treating vascular fistulas percutaneously using controlled-release coils or Amplatzer vascular plugs and to report on the complications and overall outcomes observed with these two devices. METHODS: Retrospective review of percutaneous embolizations performed from January 2004 through June 2008. RESULTS: In total, 51 vascular fistulas in 30 patients aged from 6 days to 28 years (mean, 8.4 years) underwent successful embolization. The underlying diagnoses were: 27 venous collaterals in 16 patients after the Glenn procedure, four surgical (i.e. Blalock-Taussig) fistulas, 11 pulmonary arteriovenous fistulas in three patients, two aortopulmonary collateral arteries in two patients, one venous collateral in a patient who underwent the Fontan procedure, one aortopulmonary artery fistula in a patient with Scimitar syndrome, one coronary arteriovenous fistula, three systemic arteriovenous fistulas in a newborn, and one fistula from the left atrium to the superior vena cava after the repair of anomalous pulmonary venous return. The lesions were treated percutaneously using 34 vascular plugs and 19 coils. CONCLUSIONS: Vascular fistulas can be occluded percutaneously with good RESULTS: Small fistulas can be closed using coils, while vascular plugs are preferable for large lesions. Both devices are highly effective as occluders and no particular difficulty or significant complication was observed.

[Pregnancy and congenital heart disease]. / Embarazo y cardiopatías congénitas.

INTRODUCTION AND OBJECTIVES: Since the creation of the Adult Congenital Heart Disease Units and of the High Obstetric Risk Units, there has been increasing interest in hemodynamic and obstetric outcomes in pregnant woman with congenital heart disease. METHODS: Retrospective descriptive study of 56 women with congenital heart disease aged (mean [range]) 25 (18-40) years, who experienced a total of 84 pregnancies between January 1992 and August 2006. The women were divided into three pregnancy risk groups: A, low-risk; B, moderate-risk, and C, high-risk. RESULTS: The incidence of complications during pregnancy was 1.6%, 15%, and 20% in groups A, B, and C, respectively; the incidence during the puerperium was 2%, 23%, and 50%, respectively; and maternal mortality was 0%, 7.6%, and 25%, respectively. Overall, 69 children were born, and the prematurity rates in the three groups were 11%, 15%, and 100%, respectively. The following risk factors were studied: pulmonary hypertension, cyanosis, arrhythmia, left ventricular outflow tract obstruction, right ventricular dilatation, systemic right ventricle, and anticoagulation therapy. The risk factor most significantly associated with maternal or fetal morbidity or mortality was found to be pulmonary hypertension. CONCLUSIONS: Risk stratification in pregnant women with congenital heart disease provides prognostic information that can help multidisciplinary teams to target care to achieve the best results.