Diagnosis and treatment of myocarditis and inflammatory cardiomyopathy. Consensus document of the SEC-Working Group on Myocarditis.

Myocarditis is defined as myocardial inflammation and its etiology is highly diverse, including infectious agents, drugs, and autoimmune diseases. The clinical presentation also varies widely, extending beyond the classic clinical picture of acute chest pain, and includes cases of cardiomyopathy of unknown cause whose etiology may be inflammatory. Because certain patients may benefit from targeted treatments, the search for the etiology should begin when myocarditis is first suspected. There remain several areas of uncertainty in the diagnosis and treatment of this disease. Consequently, this consensus document aims to provide clear recommendations for its diagnosis and treatment. Hence, a diagnostic algorithm is proposed, specifying when non-invasive diagnosis with cardiac MR is appropriate vs a noninvasive approach with endomyocardial biopsy. In addition, more novel aspects are discussed, such as when to suspect an underlying genetic etiology. The recommendations cover the management of myocarditis and inflammatory cardiomyopathy, both for general complications and specific clinical entities.

Papel de la ecocardiografía speckle-tracking (rastreo de marcas) y la resonancia magnética cardíaca-feature tracking en la circulación de Fontan. Diferencias entre los ventrículos derecho e izquierdo / Speckle-tracking echocardiography and MCR-feature tracking in Fontan circulation. Differences between right and left ventricles

Resumen Introducción: En los pacientes con DCPT, la disfunción ventricular es inevitable, y más temprana en VU derechos. La deformación miocárdica por STE y RMC-FT parece promisoria. Objetivo: Analizar la función ventricular mediante STE y RMC-FT en pacientes con DCPT, en comparación con RMC convencional según la morfología del VU y la posible implicación en su diagnóstico temprano. Método: Se recogieron medidas del strain longitudinal y circunferencial por STE y RMC-FT, volúmenes ventriculares y FE por RMC en 64 pacientes con DCPT. Resultados: La morfología ventricular no se relacionó con disfunción por RMC. Los VU derechos tuvieron valores por STE y RMC-FT disminuidos respecto de los VU izquierdos, con FE similares. Existe correlación entre STE y RMC-FT, no equivalentes, con buena factibilidad y reproducibilidad. Conclusiones: La RMC-FT y el STE son técnicas útiles en el diagnóstico temprano y la vigilancia de la función ventricular en VU derechos con FE preservada.

Abstract Introduction: In patients with TCPC, the development of ventricular dysfunction is inevitable and is more precocious in SRVs. Myocardial deformation by STE and CMR-FT is promising. Objective: To analize ventricular function in patients with TCPC using STE and CMR-FT compared with conventional cMRI, depending on SV morphology, to determine their role in early diagnosis of ventricular dysfunction. Method: Sixty-four patients with TCPC were included. Longitudinal and circumferential strain by STE and CMR-FT and ventricular volume and EF were obtained. Results: Dysfunction analyzed by cMRI showed no association with ventricular morphology. SRVs had lower values in STE and CMR-FT compared with SLVs, with similar EF. While not equivalent, correlation was observed between the STE and the CMR-FT values, demonstrating good feasibility and reproducibility. Conclusion: The strain data in CMR-FT and STE could be useful for diagnosis and monitoring of ventricular function and as markers of early SRV dysfunction with preserved EF.

Prenatal Diagnosis of Aortic Coarctation: Prediction Algorithm according to Gestational Age.

OBJECTIVES: The aim of the study was first to quantify the diagnostic accuracy of predictive anatomical factors of aortic coarctation (CoA) and second to design a postnatal CoA probability algorithm according to gestational age (GA) in prenatal period. METHODS: Global and according to GA diagnostic performance of cardiac anatomical variables using the ROC curve were evaluated in a retrospective cohort of fetuses with suspicion of CoA (2004-2020). A serial testing strategy to predict postnatal CoA by fetal echocardiography was designed. RESULTS: 114 fetuses were included. Isthmus-to-ductal (I/D) ratio provided the best discrimination between healthy fetuses and those with CoA (AUC 0.91, 95% CI: 0.86-0.96, I/D < 0.74 sensitivity 96.3%, I/D < 0.6, specificity 92.5%) with good classification capacity in both the second and third trimesters of gestation. Isthmus z-score and pulmonary/aortic valve ratio increased accuracy in fetuses >28 and tricuspid/mitral valve ratio (TV/MV) in fetuses ≤28 weeks. Study of I/D plus TV/MV ratio in fetuses ≤28 and I/D ratio plus isthmus z-scores in fetuses >28 weeks allowed to correctly classify 91.8% of fetuses as high or low probability of postnatal CoA. CONCLUSIONS: Diagnostic discrimination of anatomic predictive factors for CoA varies according to GA. Specific algorithms according to GA increase accuracy in CoA's prenatal prediction.

Spanish registry of percutaneous VSD closure with NitOcclud Lê VSD Coil device: lessons learned after more than a hundred implants.

INTRODUCTION AND OBJECTIVES: The NitOcclud Lê VSD Coil was specifically designed for transcatheter occlusion of ventricular septal defects (VSD) and became available for this purpose in August 2010. Our objective was to describe the Spanish experience of this technique and analyze its reliability and short- to mid-term efficacy. METHODS: National multicenter observational study, which retrospectively recruited all patients (of any age) with VSD (of any location or type) who underwent percutaneous NitOcclud occlusion, using an intention-to-treat analysis, until January 2019. RESULTS: A total of 117 attempts were made to implant at least 1 NitOcclud in 116 patients in 13 institutions. The median [range] age and weight was 8.6 [0.4-69] years and 27 [5.8-97] kg, respectively. In 99 patients (85%), the VSD was an isolated congenital defect. The location was perimembranous in 95 (81%), and 74 (63%) of them were aneurysmatic. The mean fluoroscopy time was 34 [11.4-124] minutes. Of the 117 attempts, 104 were successful (89%) with a follow-up of 31.4 [0.6-59] months. At the last review, final complete occlusion of the defect without residual shunt or with only a minimal shunt was achieved in 92.3% (no shunt, n=73; trivial shunt, n=23). Four patients required a second procedure for residual shunt occlusion. Two devices had to be surgically explanted due to severe hemolysis. There were no deaths or other major complications. CONCLUSIONS: The NitOcclud device can be used successfully for a wide anatomical spectrum of VSD. The main issue is residual shunt, but its incidence decreases over time. The incidence of hemolysis was very low and no permanent changes were detected in atrioventricular conduction.

Valores de referencia (Z-score) de diámetros de porción de entrada y trabecular del ventrículo derecho: primera descripción en población pediátrica española sana / Reference values (Z-score) of inlet and trabecular portion diameters of the right ventricle: First description in Spanish healthy children

INTRODUCCIÓN Y OBJETIVOS: La cuantificación del tamaño del ventrículo derecho (VD) es crucial en determinadas cardiopatías congénitas. Para su evaluación se recomiendan distintas técnicas de imagen, siendo más accesible la medición de diámetros ventriculares mediante ecocardiografía. En pediatría la normalización de parámetros ecocardiográficos es compleja, escasa y heterogénea. El objetivo de este estudio consistió en establecer Z-score de diámetros de VD fiables y reproducibles capaces de predecir valores de referencia en población pediátrica sana española. MÉTODOS: Estudio multicéntrico prospectivo de 661 pacientes sanos (edades 0-18 años, 43,5% mujeres). Los diámetros ecocardiográficos del VD se relacionaron con variables biométricas mediante distintas ecuaciones de regresión. Para su estandarización se analizaron factores de confusión (sexo, edad y variabilidad inter/intraobservador), heterocedasticidad y residuos (test Saphiro-Wilk y Breusch-Pagan). RESULTADOS: Se obtuvieron curvas de normalidad (Z-score) para cada diámetro del VD que permitieron predecir el valor medio de cada diámetro en función de la edad, peso, talla y distintas superficies corporales. La superficie corporal según fórmula de Haycock ofreció un excelente ajuste para los distintos diámetros basal, medial y longitudinal (R2 0,81; 0,82; 0,9). Los factores de confusión no aportaron cambios significativos, por lo que no fueron incluidos en los modelos finales (variabilidad inter- e intraobservador, CCI > 0,9). CONCLUSIONES: Se brindan valores de referencia de diámetros VD de población pediátrica sana. Las curvas de Z-score ofrecidas cubren una importante carencia en cardiología pediátrica y son aplicables a todos los grupos de edad para evaluar el tamaño del VD, de gran interés clínico en la práctica diaria

INTRODUCTION AND OBJECTIVES: Right ventricle (RV) measurements are crucial for certain congenital heart diseases and various cardiovascular conditions. Echocardiographic RV diameters are especially useful for its assessment. Paediatric echocardiographic data standardisation in normal subjects is complex, scarce, and heterogeneous. The aim of this study was to establish reliable and reproducible echocardiographic reference values (Z-score) of RV diameters in a healthy Spanish paediatric cohort. METHODS: A multicentre study was conducted on 661 healthy subjects (age range 0-18 years, 43.5% female). Several regression models were tested to examine the relationship between RV diameters and biometric variables. Heteroscedasticity and residual associations (Shapiro-Wilk and Breusch-Pagan tests) and confounding factors (gender, age, inter/intraobserver agreement) were considered for an unbiased standardisation. RESULTS: Structured Z-scores were computed for each RV diameter. Predicted mean value for each diameter was determined according to age, weight, height, and different body surface area. The Haycock formula provided the best fit for basal, midcavity, and longitudinal diameters (R2 0.81; 0.82; 0.9). Confounders were not significant, and therefore not included in final models (inter/intraobserver agreement > 0.9). CONCLUSIONS: This study reports reference values for echocardiographic RV diameters from a Spanish healthy paediatric cohort using a rigorous statistical design. These Z-scores partly cover a gap in current paediatric cardiology and represent a relevant diagnostic tool for clinical practice, as well as a useful guide to decision making at any paediatric stage

¿Coartación de aorta en el recién nacido? No siempre es lo que parece: a propósito de un caso / Coarctation of aorta in a neonate? It is not always what it seems: an illustrative case

La coartación de aorta en neonatos se puede manifestar como insuficiencia cardíaca según el grado de obstrucción. Hay situaciones que pueden simular una coartación de aorta en los recién nacidos. Limitarse a la imagen típica de muesca aórtica en la ecocardiografía para diagnosticar una coartación de aorta puede inducir a error y demorar el diagnóstico adecuado. Se presenta el caso de un recién nacido con insuficiencia cardíaca con diagnóstico inicial de coartación de aorta y, posteriormente, de malformación arteriovenosa cerebral. Se debe considerar la malformación arteriovenosa en el diagnóstico diferencial de un recién nacido con insuficiencia cardíaca.

Coarctation of the aorta in neonates can manifest as heart failure when there is a certain degree of obstruction. There are some situations that can simulate a coarctation of the aorta in newborns. Diagnosis of coarctation of the aorta based solely on the typical aortic arch image on echocardiography can be misleading and delay an accurate diagnosis. We describe an unusual case of a newborn with heart failure who was initially diagnosed with coarctation of the aorta and then with cerebral arteriovenous malformation. We must consider the cerebral arteriovenous malformation in the differential diagnosis of a newborn with heart failure.

[Coarctation of aorta in a neonate? It is not always what it seems: an illustrative case]. / ¿Coartación de aorta en el recién nacido? No siempre es lo que parece: a propósito de un caso.

Coarctation of the aorta in neonates can manifest as heart failure when there is a certain degree of obstruction. There are some situations that can simulate a coarctation of the aorta in newborns. Diagnosis of coarctation of the aorta based solely on the typical aortic arch image on echocardiography can be misleading and delay an accurate diagnosis. We describe an unusual case of a newborn with heart failure who was initially diagnosed with coarctation of the aorta and then with cerebral arteriovenous malformation. We must consider the cerebral arteriovenous malformation in the differential diagnosis of a newborn with heart failure.

La coartación de aorta en neonatos se puede manifestar como insuficiencia cardíaca según el grado de obstrucción. Hay situaciones que pueden simular una coartación de aorta en los recién nacidos. Limitarse a la imagen típica de muesca aórtica en la ecocardiografía para diagnosticar una coartación de aorta puede inducir a error y demorar el diagnóstico adecuado. Se presenta el caso de un recién nacido con insuficiencia cardíaca con diagnóstico inicial de coartación de aorta y, posteriormente, de malformación arteriovenosa cerebral. Se debe considerar la malformación arteriovenosa en el diagnóstico diferencial de un recién nacido con insuficiencia cardíaca.

[Reference values (Z-score) of inlet and trabecular portion diameters of the right ventricle: First description in Spanish healthy children]. / Valores de referencia (Z-score) de diámetros de porción de entrada y trabecular del ventrículo derecho: primera descripción en población pediátrica española sana.

INTRODUCTION AND OBJECTIVES: Right ventricle (RV) measurements are crucial for certain congenital heart diseases and various cardiovascular conditions. Echocardiographic RV diameters are especially useful for its assessment. Paediatric echocardiographic data standardisation in normal subjects is complex, scarce, and heterogeneous. The aim of this study was to establish reliable and reproducible echocardiographic reference values (Z-score) of RV diameters in a healthy Spanish paediatric cohort. METHODS: A multicentre study was conducted on 661 healthy subjects (age range 0-18 years, 43.5% female). Several regression models were tested to examine the relationship between RV diameters and biometric variables. Heteroscedasticity and residual associations (Shapiro-Wilk and Breusch-Pagan tests) and confounding factors (gender, age, inter/intraobserver agreement) were considered for an unbiased standardisation. RESULTS: Structured Z-scores were computed for each RV diameter. Predicted mean value for each diameter was determined according to age, weight, height, and different body surface area. The Haycock formula provided the best fit for basal, midcavity, and longitudinal diameters (R2 0.81; 0.82; 0.9). Confounders were not significant, and therefore not included in final models (inter/intraobserver agreement > 0.9). CONCLUSIONS: This study reports reference values for echocardiographic RV diameters from a Spanish healthy paediatric cohort using a rigorous statistical design. These Z-scores partly cover a gap in current paediatric cardiology and represent a relevant diagnostic tool for clinical practice, as well as a useful guide to decision making at any paediatric stage.

Cor triatriatum dexter incompleto: un invitado incómodo en el cierre percutáneo de los defectos interauriculares / Incomplete cor triatriatum dexter: an unsettling guest in the percutaneous closure of atrial septal defects

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Sudden cardiac arrest as first symptom of a benign cardiac tumor growth / Muerte súbita como primer síntoma de crecimiento de tumor cardíaco benigno

Abstract Primary cardiac tumors are rare, especially in the pediatric age. Most of them are benign in the sense they are not invasive. However, benign tumors maintain the potential for serious illness related to significant hemodynamic compromise or life-threatening dysrhythmias. We present the case of an infant with an initial diagnosis of cardiac rhabdomyoma who suffered ventricular arrhythmia and cardiac arrest. He suffered irreversible severe neurologic sequelae, due to his prolonged cardiopulmonary arrest and was finally diagnosed of cardiac fibroma. Good arrhythmia control was obtained after an extensive partial surgical resection of the tumor. This case highlights the importance of arrhythmia burden in this condition. A correct diagnosis based essentially in different imaging modalities and closer clinical and rhythm follow up could have avoided this ominous event.

Resumen Los tumores cardíacos primarios son raros, especialmente en la edad pediátrica. La mayoría de ellos son benignos, en el sentido de que no son invasivos. Sin embargo, los tumores benignos tienen el potencial para producir enfermedades graves que pueden causar compromiso hemodinámico significativo o arritmias potencialmente letales. Se presenta el caso de un niño con un diagnóstico inicial de rabdomioma cardíaco, quien sufrió una arritmia ventricular y una parada cardíaca. Tuvo secuelas neurológicas severas irreversibles debido al tiempo prolongado en parada cardiorrespiratoria y se le diagnosticó finalmente fibroma cardíaco. Se obtuvo un buen control de las arritmias después de una extensa resección parcial del tumor. Este caso pretende subrayar la importancia del riesgo de aparición de arritmias en esta situación. El diagnóstico correcto basado fundamentalmente en el uso de distintas modalidades de imagen y el seguimiento clínico y arritmológico, podrían haber evitado este desenlace fatal.

Strain 2D en miocardiopatía dilatada / 2D strain in dilated cardiomyopathy

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