Early assessment of cardiomyopathy in Duchenne patients by means of longitudinal strain echocardiography.

INTRODUCTION: The diagnosis of Duchenne-linked cardiomyopathy may be challenging. Conventional echocardiographic measurements typically show deterioration beyond the second decade. Global longitudinal strain has been proposed as an earlier marker than left ventricular ejection fraction. MATERIAL AND METHODS: A prospective, observational, cross-sectional, case-control study was carried out. Both Duchenne patients and control subjects underwent transthoracic echocardiogram in order to assess left ventricle function. Bayesian linear regression was the main tool for inference. Age effects were parameterised by means of a spline function because of its simplicity to characterise continuous variables and smooth contributions. The posterior distribution of the marginal age effects was used to assess the earliest age of deterioration of each marker. RESULTS: Sixteen Duchenne patients and twenty-two healthy male subjects were enrolled. On overage, cardiac function measures were found for ejection fraction and longitudinal strain among different groups. Age effects on global longitudinal strain are more reliably found at ages of 6 and above, while ejection fraction starts to deteriorate at an older age. Progressive left ventricular dysfunction in Duchenne patients is one of the key issues and starts at an early age with subtle symptoms. CONCLUSION: This cross-sectional study provides supporting evidence that global longitudinal strain is an earlier marker of disease progression than ejection fraction in Duchenne patients.

Right ventricle speckle tracking in bronchopulmonary dysplasia: one-year follow-up.

BACKGROUND: Bronchopulmonary dysplasia (BPD) is still a major concern in preterm infants and adequate screening methods for secondary right ventricle (RV) failure are needed. Early detection could be aided by taking measurements of RV deformation using speckle tracking echocardiography. A prospective longitudinal study was carried out over 28 months at a tertiary care pediatric cardiology center. Preterm infants < 32 weeks gestational age (GA) were eligible for the study. Enrolled patients were separated in two groups: NO-BPD or BPD. At three timepoints over the first year of life, echocardiogram measurements were performed. Right ventricle strain was studied using speckle tracking analysis and compared to conventional function parameters. RESULTS: Fifty patients were enrolled in the study, 22 in the NO-BPD group and 28 in the BPD group. RV strain showed no statistical differences between groups. However, the BPD group showed worse RV function than the NO-BPD group, using speckle tracking analysis and other conventional parameters. During the study follow-up, an improvement trend is shown in RV strain. CONCLUSIONS: RV longitudinal strain and strain rate derived by speckle tracking is feasible in preterm infants. Although there seems to be a good correlation between RV strain and BPD severity, the results of this study were not conclusive. More studies should be carried out to investigate the optimum echocardiographic screening model of RV dysfunction in BPD patients.

Atrial flutter without structural heart disease in pediatrics: a retrospective review of cases in the Hospital Infantil Miguel Servet, Zaragoza, Spain.

BACKGROUND: Atrial flutter is a rare condition in pediatrics that usually occurs as a late complication after surgery for congenital heart diseases, although it can also appear in structurally normal hearts. CLINICAL CASES: We conducted a retrospective study of cases of atrial flutter with no structural heart disease diagnosed in a pediatric population (between 0 and 15 years of age) during 2015-2021 in a tertiary hospital. A total of seven cases were diagnosed, with a clear predominance of males (6/7). Of the seven patients, five debuted in the perinatal period: two were diagnosed at 20 and 36 hours of life, and three, prenatally. Among these perinatal cases, more than half (3/5) were preterm. The treatment was electrical cardioversion. The evolution was satisfactory in these cases, and there were no tachycardias in their subsequent development. In contrast, when the debut occurred at a later age (5-7 years), it was associated with channelopathy (Brugada syndrome and catecholaminergic polymorphic ventricular tachycardia), and electrical ablation of the ectopic focus was required due to poor response to pharmacological treatment. CONCLUSIONS: This study confirms the low incidence of this pathology in pediatrics and the benignity and good prognosis of neonatal flutter in most cases. The prognosis worsens when atrial flutter is diagnosed in older children, and the probability of concomitant associated heart disease increases.

INTRODUCCIÓN: El flutter o aleteo auricular es una patología poco frecuente en pediatría que suele presentarse como complicación tardía tras la cirugía de cardiopatías congénitas, aunque también puede aparecer en corazones estructuralmente normales. CASOS CLÍNICOS: Se llevó a cabo un estudio retrospectivo de los casos de flutter auricular sin cardiopatía estructural diagnosticados en una población pediátrica (entre 0 y 15 años de edad) durante el periodo 2015-2021 en un hospital terciario. En total fueron diagnosticados siete casos, con un claro predominio de varones (6/7). De los siete, cinco debutaron en periodo perinatal: dos fueron diagnosticados a las 20 y 36 horas de vida y tres de ellos, prenatalmente. Entre estos casos perinatales, más de la mitad (3/5) fueron pretérmino. El tratamiento fue la cardioversión eléctrica. La evolución fue satisfactoria en estos casos, y no se presentaron taquicardias en su evolución posterior. Por el contrario, cuando el debut se produjo en edades posteriores (5-7 años), se asoció con canalopatía (síndrome de Brugada y taquicardia ventricular polimorfa catecolaminérgica) que requirió de una ablación eléctrica del foco ectópico por escasa respuesta al tratamiento farmacológico. CONCLUSIONES: En este trabajo se confirma la baja incidencia de esta patología en pediatría, además de la benignidad y el buen pronóstico de flutter neonatal en la mayoría de casos. Cuando el diagnóstico se realiza en niños mayores, el pronóstico empeora, y aumenta la probabilidad de presentar de forma concomitante cardiopatías asociadas.

Atrial flutter without structural heart disease in pediatrics: a retrospective review of cases in the Hospital Infantil Miguel Servet, Zaragoza, Spain / Flutter auricular sin cardiopatía estructural en pediatría: una revisión retrospectiva de casos en el Hospital Infantil Miguel Servet, Zaragoza, España

Abstract Background: Atrial flutter is a rare condition in pediatrics that usually occurs as a late complication after surgery for congenital heart diseases, although it can also appear in structurally normal hearts. Clinical cases: We conducted a retrospective study of cases of atrial flutter with no structural heart disease diagnosed in a pediatric population (between 0 and 15 years of age) during 2015-2021 in a tertiary hospital. A total of seven cases were diagnosed, with a clear predominance of males (6/7). Of the seven patients, five debuted in the perinatal period: two were diagnosed at 20 and 36 hours of life, and three, prenatally. Among these perinatal cases, more than half (3/5) were preterm. The treatment was electrical cardioversion. The evolution was satisfactory in these cases, and there were no tachycardias in their subsequent development. In contrast, when the debut occurred at a later age (5-7 years), it was associated with channelopathy (Brugada syndrome and catecholaminergic polymorphic ventricular tachycardia), and electrical ablation of the ectopic focus was required due to poor response to pharmacological treatment. Conclusions: This study confirms the low incidence of this pathology in pediatrics and the benignity and good prognosis of neonatal flutter in most cases. The prognosis worsens when atrial flutter is diagnosed in older children, and the probability of concomitant associated heart disease increases.

Resumen Introducción: El flutter o aleteo auricular es una patología poco frecuente en pediatría que suele presentarse como complicación tardía tras la cirugía de cardiopatías congénitas, aunque también puede aparecer en corazones estructuralmente normales. Casos clínicos: Se llevó a cabo un estudio retrospectivo de los casos de flutter auricular sin cardiopatía estructural diagnosticados en una población pediátrica (entre 0 y 15 años de edad) durante el periodo 2015-2021 en un hospital terciario. En total fueron diagnosticados siete casos, con un claro predominio de varones (6/7). De los siete, cinco debutaron en periodo perinatal: dos fueron diagnosticados a las 20 y 36 horas de vida y tres de ellos, prenatalmente. Entre estos casos perinatales, más de la mitad (3/5) fueron pretérmino. El tratamiento fue la cardioversión eléctrica. La evolución fue satisfactoria en estos casos, y no se presentaron taquicardias en su evolución posterior. Por el contrario, cuando el debut se produjo en edades posteriores (5-7 años), se asoció con canalopatía (síndrome de Brugada y taquicardia ventricular polimorfa catecolaminérgica) que requirió de una ablación eléctrica del foco ectópico por escasa respuesta al tratamiento farmacológico. Conclusiones: En este trabajo se confirma la baja incidencia de esta patología en pediatría, además de la benignidad y el buen pronóstico de flutter neonatal en la mayoría de casos. Cuando el diagnóstico se realiza en niños mayores, el pronóstico empeora, y aumenta la probabilidad de presentar de forma concomitante cardiopatías asociadas.

Alteraciones electrocardiográficas tras electrocución / Electrocardiographic alterations after electrocution

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Isolated fetal pericardial effusion follow-up: Should we worry?

INTRODUCTION AND OBJECTIVES: Fetal pericardial effusion appears in different pathologies such as hydrops fetalis, heart structural or rhythm alterations, however, it can be observed in isolation but an increase in its incidence has been observed in relation to the presence of severe pathologies. METHODS: Analysis of all cases of IFPE detected in Aragon and assessed in a cardiological consultation for prenatal diagnosis of a tertiary hospital collected over ten years, as well as the evolution of the patients to the present. RESULTS: A sample of 38 fetuses was obtained from 37 pregnant women diagnosed with DPFA with spontaneous resolution in 86.8%. Two abortions (voluntary interruptions after prenatal diagnosis of 22q13 deletion and primary infection by cytomegalovirus) and one spontaneous fetal death were recorded. Pathological alterations were observed in 10/38 newborns: two patients with metabolic disease, two patients with chromosomopathies, one patient with pulmonary hypoplasia and unilateral hydronephrosis, one patient with hypertrophic cardiomyopathy, and four patients studied for alterations in psychomotor development and/or congenital ophthalmological or hearing disorders. The overall morbidity rate was 34.2% and death rate 15.7%. The detection of other ultrasound alterations and the alteration in the first trimester screening were significantly associated with the presence of pathology. CONCLUSIONS: IFPE has been classically associated with a good prognosis, although it is sometimes related to clinical entities with high morbidity and mortality: more than a third of the patients in our sample are affected. An exhaustive pre and postnatal follow-up of these cases is recommended in order to perform an early intervention.

Seguimiento del derrame pericárdico fetal aislado: ¿debemos preocuparnos? / Isolated fetal pericardial effusion follow-up: Should we worry?

Introducción y objetivos: El derrame pericárdico fetal aparece en diferentes enfermedades como hidropesía fetal, alteraciones estructurales o del ritmo cardiaco, aunque puede observarse de manera aislada. Se ha observado un incremento de su incidencia con relación a la presencia de enfermedades graves. Métodos: Análisis de la totalidad de casos de derrame pericárdico fetal aislado (DPFA) detectados en Aragón y valorados en consulta cardiológica de diagnóstico prenatal de un hospital terciario recogidos durante 10años, así como la evolución de los pacientes hasta la actualidad. Resultados: Se obtuvo una muestra de 38 fetos en 37 gestantes diagnosticados de DPFA con resolución espontánea en el 86,8%. Se registraron 2abortos (interrupciones voluntarias tras diagnóstico prenatal de deleción 22q13 y de primoinfección por citomegalovirus) y una muerte fetal espontánea. Se objetivaron alteraciones patológicas en 10/38 recién nacidos: 2pacientes con metabolopatía, 2pacientes con cromosomopatía, un paciente con hipoplasia pulmonar e hidronefrosis unilateral, un paciente con miocardiopatía hipertrófica y 4pacientes estudiados por alteraciones del desarrollo psicomotor o alteraciones congénitas oftalmológicas o auditivas. La tasa de morbilidad fue del 34,2% y de fallecimiento del 15,7%. La detección de otras alteraciones ecográficas y la alteración en el cribado del primer trimestre se asociaron de forma significativa con la presencia de patología. Conclusiones: El DPFA se ha asociado clásicamente a buen pronóstico, aunque en ocasiones se relaciona con entidades clínicas con elevada morbimortalidad: más de un tercio de los pacientes en nuestra muestra. Se recomienda un seguimiento estrecho pre y posnatal de estos casos para poder realizar una intervención precoz. (AU)

Introduction and objectives: Fetal pericardial effusion appears in different pathologies such as hydrops fetalis, heart structural or rhythm alterations, however, it can be observed in isolation but an increase in its incidence has been observed in relation to the presence of severe pathologies. Methods: Analysis of all cases of IFPE detected in Aragon and assessed in a cardiological consultation for prenatal diagnosis of a tertiary hospital collected over 10years, as well as the evolution of the patients to the present. Results: A sample of 38 fetuses was obtained from 37 pregnant women diagnosed with DPFA with spontaneous resolution in 86.8%. Two abortions (voluntary interruptions after prenatal diagnosis of 22q13 deletion and primary infection by cytomegalovirus) and one spontaneous fetal death were recorded. Pathological alterations were observed in 10/38 newborns: 2patients with metabolic disease, 2patients with chromosomopathies, one patient with pulmonary hypoplasia and unilateral hydronephrosis, one patient with hypertrophic cardiomyopathy, and 4patients studied for alterations in psychomotor development and/or congenital ophthalmological or hearing disorders. The overall morbidity rate was 34.2% and death rate 15.7%. The detection of other ultrasound alterations and the alteration in the first trimester screening were significantly associated with the presence of pathology. Conclusions: IFPE has been classically associated with a good prognosis, although it is sometimes related to clinical entities with high morbidity and mortality: more than a third of the patients in our sample are affected. An exhaustive pre- and posnatal follow-up of these cases is recommended in order to perform an early intervention. (AU)

[Isolated fetal pericardial effusion follow-up: Should we worry?] / Seguimiento del derrame pericárdico fetal aislado: ¿debemos preocuparnos?

INTRODUCTION AND OBJECTIVES: Fetal pericardial effusion appears in different pathologies such as hydrops fetalis, heart structural or rhythm alterations, however, it can be observed in isolation but an increase in its incidence has been observed in relation to the presence of severe pathologies. METHODS: Analysis of all cases of IFPE detected in Aragon and assessed in a cardiological consultation for prenatal diagnosis of a tertiary hospital collected over 10years, as well as the evolution of the patients to the present. RESULTS: A sample of 38 fetuses was obtained from 37 pregnant women diagnosed with DPFA with spontaneous resolution in 86.8%. Two abortions (voluntary interruptions after prenatal diagnosis of 22q13 deletion and primary infection by cytomegalovirus) and one spontaneous fetal death were recorded. Pathological alterations were observed in 10/38 newborns: 2patients with metabolic disease, 2patients with chromosomopathies, one patient with pulmonary hypoplasia and unilateral hydronephrosis, one patient with hypertrophic cardiomyopathy, and 4patients studied for alterations in psychomotor development and/or congenital ophthalmological or hearing disorders. The overall morbidity rate was 34.2% and death rate 15.7%. The detection of other ultrasound alterations and the alteration in the first trimester screening were significantly associated with the presence of pathology. CONCLUSIONS: IFPE has been classically associated with a good prognosis, although it is sometimes related to clinical entities with high morbidity and mortality: more than a third of the patients in our sample are affected. An exhaustive pre- and posnatal follow-up of these cases is recommended in order to perform an early intervention.

Flutter auricular neonatal: presentación de tres casos / Neonatal atrial flutter: Presentation of three cases

Resumen Introducción: El flutter auricular es un tipo poco frecuente de arritmia fetal y neonatal. A pesar de que puede conducir a graves morbilidades, como hidrops fetal o incluso el fallecimiento, el diagnóstico y tratamiento precoz confieren un buen pronóstico a la mayoría de los casos. Pacientes y métodos: Se presentan tres casos de flutter auricular, dos de inicio en periodo fetal y uno en periodo neonatal, y se revisa la literatura en relación con las características clínicas, diagnósticas y terapéuticas del flutter auricular fetal y neonatal. Resultados y discusión: En el flutter auricular fetal la terapia materna con fármacos antiarrítmicos es el tratamiento más empleado durante la gestación. El tratamiento postnatal más utilizado es la cardioversión eléctrica sincronizada. El flutter auricular no suele asociar cardiopatía estructural; la recidiva neonatal es poco habitual y normalmente no precisa la administración de tratamiento profiláctico.

Abstract Introduction: Atrial flutter is a rare type of fetal and neonatal arrhythmia. Although it can lead to serious morbidities such as fetal hydrops or even death, diagnosis and early treatment confer a good prognosis in most cases. Patients and methods: Three cases of atrial flutter are presented, two of which start in the fetal period and one in the neonatal period. The literature is reviewed in relation to the clinical, diagnostic and therapeutic characteristics of fetal and neonatal atrial flutter. Results and discussion: In fetal atrial flutter maternal therapy with antiarrhythmic drugs is the most used treatment during pregnancy. The most used postnatal treatment is synchronized electrical cardioversion. Atrial flutter does not usually associate structural heart disease, neonatal recurrence is uncommon and usually does not require prophylactic treatment.

Early factors related to mortality in children treated with bi-level noninvasive ventilation and CPAP.

OBJECTIVE: To describe and analyze the characteristics and the early risk factors for mortality of noninvasive ventilation (NIV) in critically ill children. STUDY DESIGN: A multicenter, prospective, observational 2-year study carried out with critically ill patients (1 month - 18 years of age) who needed NIV. Clinical data and NIV parameters during the first 12 h of admission were collected. A multilevel mixed-effects logistic regression was performed to identify mortality risk factors. RESULTS: A total of 781 patients (44.2 ± 57.7 months) were studied (57.8% male). Of them, 53.7% had an underlying condition, and 47.1% needed NIV for lower airway respiratory pathologies. Bi-level NIV was the initial support in 78.2% of the patients. Continuous positive airway pressure (CPAP) was used more in younger patients (33.7%) than in older ones (9.7%; p < .001). About 16.7% had to be intubated and 6.2% died. The risk factors for mortality were immunodeficiency (odds ratio [OR] = 11.79; 95% confidence interval [CI] = 2.95-47.13); cerebral palsy (OR = 5.86; 95% CI = 1.94-17.65); presence of apneas on admission (OR = 5.57; 95% CI = 2.13-14.58); tachypnea 6 h after NIV onset (OR = 2.59; 95% CI = 1.30-6.94); and NIV failure (OR = 6.54; 95% CI = 2.79-15.34). CONCLUSION: NIV is used with great variability in types of support. Younger children receive CPAP more frequently than older children. Immunodeficiency, cerebral palsy, apneas on admission, tachypnea 6 h after NIV onset, and NIV failure are the early factors associated with mortality.