Comprehensive Functional Echocardiographic Assessment of Transposition of the Great Arteries: From Fetus to Newborn.

To improve the understanding of the pathophysiology of transposition of the great arteries with intact ventricular septum (TGA/IVS) and the cardiac remodeling occurring from fetal to neonatal life, we performed a morphometric and functional echocardiographic assessment in fetuses and newborns. This was a prospective case-control study performed in a tertiary referral center, which included fetuses and newborns with a diagnosis of TGA/IVS between 2011 and 2018. Morphometry and systolic and diastolic function parameters were compared with age and body surface-matched healthy controls. Twenty-one TGA/IVS patients were included during the study period and morphometric and functional echocardiographic data were recorded. TGA/IVS patients showed morphometric and functional changes of increased overall volume and output, predominantly in the aortic component from fetus to newborn, probably due to compensatory mechanisms secondary to brain hypoxia.

Idiopathic dilatation of the right atrium: a not so benign entity.

Idiopathic dilatation of the right atrium is an isolated enlargement of the right atrium in the absence of other cardiac lesions. This rare anomaly has a clinical spectrum ranging from asymptomatic to heart failure or even sudden death. It can be associated with atrial arrhythmias and thrombus formation. Antiplatelet therapy is prescribed in most cases reported in the literature, and reduction plasty is indicated when there is rapid growth of the right atrium, compression of adjacent structures, or refractory arrhythmias. We report four cases of idiopathic dilatation of the right atrium diagnosed during prenatal screening. We describe the intrauterine course and management in postnatal life until early childhood.

Cardiovascular adaptation to extrauterine life after intrauterine growth restriction.

Introduction The adaptive changes of the foetal heart in intrauterine growth restriction can persist postnatally. Data regarding its consequences for early circulatory adaptation to extrauterine life are scarce. The aim of this study was to assess cardiac morphometry and function in newborns with late-onset intrauterine growth restriction to test the hypothesis that intrauterine growth restriction causes cardiac shape and functional changes at birth. METHODS: A comprehensive echocardiographic study was performed in 25 neonates with intrauterine growth restriction and 25 adequate-for-gestational-age neonates. RESULTS: Compared with controls, neonates with intrauterine growth restriction had more globular ventricles, lower longitudinal tricuspid annular motion, and higher left stroke volume without differences in the heart rate. Neonates with intrauterine growth restriction also showed subclinical signs of diastolic dysfunction in the tissue Doppler imaging with lower values of early (e') diastolic annular peak velocities in the septal annulus. Finally, the Tei index in the tricuspid annulus was higher in the intrauterine growth restriction group. CONCLUSION: Neonates with history of intrauterine growth restriction showed cardiac remodelling and signs of systolic and diastolic dysfunction. Overall, there was a significant tendency to worse cardiac function results in the right heart. The adaptation to extrauterine life occurred with more globular hearts, higher stroke volumes but a similar heart rate compared to adequate-for-gestational-age neonates.

Accuracy of Fetal Echocardiography in the Differential Diagnosis between Truncus Arteriosus and Pulmonary Atresia with Ventricular Septal Defect.

OBJECTIVES: To report on the accuracy of fetal echocardiography in the distinction between truncus arteriosus communis (CAT) and pulmonary atresia with ventricular septal defect (PA-VSD) and to describe the association with extracardiac and chromosomal anomalies. METHODS: This was a retrospective study on 31 fetuses with a single arterial trunk overriding a VSD with a nonidentifiable right ventricle outflow tract with anterograde flow. Data on the type of cardiac defect, gestational age, characteristics of the arterial trunk valve, presence of additional vascular, chromosomal and extracardiac abnormalities and postnatal outcome were obtained. Misdiagnosed cases were reevaluated by four-dimensional spatiotemporal image correlation (4D-STIC) echocardiography. RESULTS: The overall diagnostic accuracy was 81% and increased to 93.5% with 4D-STIC. Chromosomal and extracardiac anomalies were detected in 40 and 27%, respectively. In the PA-VSD group, patent ductus arteriosus and major aortopulmonary collateral arteries (MAPCAs) were present in 70 and 50% of the cases, respectively, coexisting in 1 of 5 cases. MAPCAs were significantly associated with a right aortic arch and with a 22q11 microdeletion in 50% of cases. CONCLUSIONS: A prenatal distinction between CAT and PA-VSD can currently be achieved in most cases. MAPCAs should be actively searched for when PA-VSD is suspected, as they are associated with a higher risk of 22q11 microdeletion and potentially complicate postnatal treatment.

Assisted reproductive technologies are associated with cardiovascular remodeling in utero that persists postnatally.

BACKGROUND: Assisted reproductive technologies (ARTs) have been shown to be associated with general vascular dysfunction in late childhood. However, it is unknown whether cardiac remodeling is also present and if these changes already manifest in prenatal life. Our aim was to assess fetal and infant (6 months of age) cardiovascular function in ART pregnancies. METHODS AND RESULTS: This prospective cohort study included 100 fetuses conceived by ART and 100 control pregnancies. ART fetuses showed signs of cardiovascular remodeling, including a more globular heart with thicker myocardial walls, decreased longitudinal function (tricuspid ring displacement in controls: median, 6.5 mm [interquartile range, 6.1-7.1 mm]; tricuspid ring displacement in ART: 5.5 mm [interquartile range, 5.1-6.1]; P<0.001), impaired relaxation, and dilated atria (atrial area in controls, 1.46 cm(2) [interquartile range, 1.2-1.5 cm(2)]; atrial area in ART, 1.6 cm(2) [interquartile range, 1.3-1.8 cm(2)]; P<0.001). Additionally, ART infants showed persistence of most cardiac changes and a significant increase in blood pressure and aortic intima-media thickness (systolic blood pressure in controls, 74 mm Hg [interquartile range, 67-83 mm Hg]; systolic blood pressure in ART, 83 mm Hg [interquartile range, 75-94 mm Hg]; P<0.001; aortic intima-media thickness in controls, 0.52 mm [interquartile range, 0.45-0.56 mm]; aortic intima-media thickness in ART, 0.64 mm [interquartile range, 0.62-0.67]; P<0.001). We could not demonstrate that our findings were directly caused by ART because of their association with various confounding factors, including intrauterine growth restriction or factors related to the cause of infertility. CONCLUSIONS: Children conceived by ART manifest cardiac and vascular remodeling that is present in fetal life and persists in postnatal life, suggesting opportunities for early detection and potential intervention. The underlying mechanisms and the effect of potential confounders such as growth restriction or prematurity remain to be elucidated.

Isolated atrioventricular block in the fetus: a retrospective, multinational, multicenter study of 175 patients.

BACKGROUND: Isolated complete atrioventricular block in the fetus is a rare but potentially lethal condition in which the effect of steroid treatment on outcome is unclear. The objective of this work was to study risk factors associated with death and the influence of steroid treatment on outcome. METHODS AND RESULTS: We studied 175 fetuses diagnosed with second- or third-degree atrioventricular block (2000-2007) retrospectively in a multinational, multicenter setting. In 80% of 162 pregnancies with documented antibody status, atrioventricular block was associated with maternal anti-Ro/SSA antibodies. Sixty-seven cases (38%) were treated with fluorinated corticosteroids for a median of 10 weeks (1-21 weeks). Ninety-one percent were alive at birth, and survival in the neonatal period was 93%, similar in steroid-treated and untreated fetuses, regardless of degree of block and/or presence of anti-Ro/SSA. Variables associated with death were gestational age < 20 weeks, ventricular rate ≤ 50 bpm, fetal hydrops, and impaired left ventricular function at diagnosis. The presence of ≥ 1 of these variables was associated with a 10-fold increase in mortality before birth and a 6-fold increase in the neonatal period independently of treatment. Except for a lower gestational age at diagnosis in treated than untreated (23.4 ± 2.9 versus 24.9 ± 4.9 weeks; P=0.02), risk factors were distributed equally between treatment groups. Two-thirds of survivors had a pacemaker by 1 year of age; 8 children developed cardiomyopathy. CONCLUSIONS: Risk factors associated with a poor outcome were gestation < 20 weeks, ventricular rate ≤ 50 bpm, hydrops, and impaired left ventricular function. No significant effect of treatment with fluorinated corticosteroids was seen.

Cardiovascular programming in children born small for gestational age and relationship with prenatal signs of severity.

OBJECTIVE: The objective of the study was to evaluate cardiovascular function in children who were small-for-gestational-age (SGA) fetuses. STUDY DESIGN: This was a prospective study including 100 controls and 50 children diagnosed in utero as SGA after 34 weeks subdivided into the following categories: SGA and intrauterine growth restriction (IUGR) according to the absence or presence, respectively, of weight centile less than 3 or abnormal cerebroplacental Doppler. Postnatal cardiovascular outcome was evaluated at 3-6 years of age by echocardiography, blood pressure, and carotid ultrasound. RESULTS: Both SGA and IUGR presented in childhood more globular hearts, reduced longitudinal motion, and impaired relaxation with an increase in radial function. Both groups showed increased blood pressure and carotid intima-media thickness. There was a linear tendency to worse cardiovascular results in IUGR as compared with SGA. CONCLUSION: Fetal cardiovascular programming occurs in SGA, regardless of Doppler and weight centile. These findings challenge the concept of constitutionally small and warrant further investigation to identify predictors of cardiovascular outcome in SGA.

Fetal growth restriction results in remodeled and less efficient hearts in children.

BACKGROUND: Fetal growth restriction (FGR) affects 5% to 10% of newborns and is associated with increased cardiovascular mortality in adulthood. The most commonly accepted hypothesis is that fetal metabolic programming leads secondarily to diseases associated with cardiovascular disease, such as obesity, diabetes mellitus, and hypertension. Our main objective was to evaluate the alternative hypothesis that FGR induces primary cardiac changes that persist into childhood. METHODS AND RESULTS: Within a cohort of fetuses with growth restriction identified in fetal life and followed up into childhood, we randomly selected 80 subjects with FGR and compared them with 120 normally grown fetuses, matched for gender, birth date, and gestational age at birth. Cardiovascular assessment was performed in childhood (mean age of 5 years). Compared with control subjects, children with FGR had a different cardiac shape, with increased transversal diameters and more globular cardiac ventricles. Although left ejection fraction was similar among the study groups, stroke volume was reduced significantly, which was compensated for by an increased heart rate to maintain output in severe FGR. This was associated with subclinical longitudinal systolic dysfunction (decreased myocardial peak velocities) and diastolic changes (increased E/E' ratio and E deceleration time). Children with FGR also had higher blood pressure and increased intima-media thickness. For all parameters evaluated, there was a linear increase with the severity of growth restriction. CONCLUSIONS: These findings suggest that FGR induces primary cardiac and vascular changes that could explain the increased predisposition to cardiovascular disease in adult life. If these results are confirmed, the impact of strategies with beneficial effects on cardiac remodeling should be explored in children with FGR.

Defibrillation threshold decrease with the supradiaphragmatic extracardiac implantable cardioverter-defibrillator implantation technique.

Despite advances in implantable cardioverter-defibrillator (ICD) technology, the optimal ICD implantation technique for pediatric patients has not yet been established. One increasingly used option is totally extracardiac implantation. However, concern exists about the high defibrillation threshold (DFT) at the moment of implantation or during follow-up. We report the case of a 3-year-old boy with repetitive syncopal idiopathic ventricular tachycardia episodes treated with ICD implantation using the extracardiac technique. Changing device position from abdominal to a supradiaphragmatic, solved unsafe elevated discharge impedance and DFT during follow-up.

[Ductus arteriosus patency with stenting in critical pulmonary stenosis and pulmonary atresia with intact interventricular septum]. / Permeabilidad del conducto arterioso con stent en la estenosis pulmonar crítica y atresia pulmonar con septo interventricular intacto.

We report our experience with stenting of the ductus arteriosus in three neonates. Two patients had pulmonary atresia with an intact interventricular septum and one had critical pulmonary stenosis. Radiofrequency ablation was used to open the atretic pulmonary valve in two patients. In all three patients implanting a stent avoided the need for surgical creation of a Blalock-Taussig shunt. In two patients the procedure was scheduled as elective surgery, and in one it was done as an emergency procedure. Ductus arteriosus stenting is an alternative to palliative cardiac surgery.

[Percutaneous closure of ventricular septal defect with an Amplatzer device]. / Cierre percutáneo de la comunicación interventricular con dispositivo Amplatzer.

We present a series of 4 patients in whom a ventricular septal defect (VSD) was closed with an Amplatzer muscular VSD device during cardiac catheterization. In one patient with type I truncus arteriosus and subarterial VSD, closure of a wide apical defect was done to allow further surgical correction while avoiding left ventricular ventriculotomy. The second patient had congenitally corrected transposition of the great arteries with complete A-V block and a pacemaker implanted from birth. Our intention was unload the systemic anatomical right ventricle. The third patient had had 3 heart surgeries to correct a double outlet right ventricle. She had a residual ventricular septal defect which was large enough to cause heart failure. The last patient had an isolated muscular ventricular septal defect. Percutaneous closure was successful in all patients, and there were no complications. Percutaneous closure of the defect may be used as a primary procedure, before surgery, or as a complementary procedure after surgery.

[Surgical treatment of anomalous left pulmonary artery]. / Tratamiento quirúrgico del origen anómalo de la arteria pulmonar izquierda.

We report our experience with the surgical treatment of anomalous origin of the left pulmonary artery in eight children between 2004 and 2009. The congenital heart disease most frequently associated with this condition was patent ductus arteriosus. Surgery was carried out with extracorporeal circulation in five children, and without, in three. The anomalous pulmonary artery was divided and translocated to the main pulmonary artery. One patient died soon after surgery because of hemodynamic instability and another died later because of respiratory complications. The other patients progressed satisfactorily during follow-up: the reimplanted artery remained patent in all cases and respiratory symptoms improved. However, one patient required endoscopic treatment.

[Cardiopulmonary stress testing in children who have had congenital heart disease surgery. Physical exercise recommendations during school hours]. / Prueba de esfuerzo con función cardiopulmonar en niños operados de cardiopatía congénita. Recomendaciones de ejercicio físico en el ámbito escolar.

INTRODUCTION AND OBJECTIVES: To analyze and discover if stress testing with exhaled gases in children who have had congenital heart surgery is useful so we could make physical exercise recommendations according to heart disease, type of surgery performed, present hemodynamic state and level of exercise practiced. METHODS: Prospective study of 108 children, who performed stress testing with exhaled gases, electrocardiogram monitoring and blood pressure. A questionnaire was used to obtain variables concerning heart disease, surgery, present functional condition and level of exercise practiced. Exercise recommendations were given after stress testing, and after a year 35 patients answered a questionnaire. RESULTS: There were significant differences between lesion severity and heart rate at rest and during effort, systolic pressure at rest and during effort, oxygen uptake, oxygen pulse, carbon dioxide production and test duration. A relationship was observed between level of weekly exercise and greater oxygen uptake and test duration, but this was not observed with the underlying heart disease. We observed that best performance occurred with fast repairing for 59 children with cyanotic heart disease. Increased exercise level was recommended for 48 children. CONCLUSIONS: The cardiopulmonary function study allows us to examine the physical performance of children who have had congenital heart surgery and provides us with important data so that we can recommend better physical exercise planning.

Complex aortic coarctation and PHACE syndrome.

In PHACE syndrome, the acronym PHACE stands for the association of posterior fossa malformations, cervicofacial hemangiomas, arterial anomalies, coarctation and eye anomalies. We report our findings in four patients with this syndrome, in whom it was characterized by complex aortic coarctation that required not only preoperative echocardiographic investigation, but also the use of techniques such as magnetic resonance imaging and angiography. Surgical treatment was also complex. Prognosis in this condition depends primarily on cardiovascular and cerebral artery complications associated with the syndrome.

Percutaneous treatment of atrial septal defects, muscular ventricular septal defects and patent ductus arteriosus in infants under one year of age.

Amplatzer devices are used for the percutaneous closure of ostium secundum atrial septal defects, muscular ventricular septal defects and patent ductus arteriosus. However, very little experience has been gained in using these devices in infants under 1 year of age. Between January 2001 and January 2008, 22 symptomatic infants aged under 1 year underwent percutaneous treatment: three had an ostium secundum atrial septal defect, 15 had patent ductus arteriosus, and four had a muscular ventricular septal defect. All the procedures were completed successfully. No immediate or medium-term complications were observed. Closure of these types of defect using an Amplatzer device in infants under 1 year of age, who would otherwise require surgery, is a safe and effective procedure.

Tratamiento quirúrgico del origen anómalo de la arteria pulmonar izquierda / Surgical treatment of anomalous left pulmonary artery

Presentamos nuestra experiencia en el tratamiento quirúrgico del origen anómalo de la arteria pulmonar izquierda en 8 niños, intervenidos entre 2004 y 2009. La cardiopatía asociada más frecuentemente fue la persistencia del conducto arterioso. A 5 se realizó cirugía con circulación extracorpórea y a 3, sin ella. Se realizó división de la arteria pulmonar anómala y translocación al tronco de la arteria pulmonar. Hubo un fallecimiento de forma temprana por inestabilidad hemodinámica y una muerte más tardía por complicaciones respiratorias. La evolución de los pacientes en seguimiento ha sido satisfactoria con permeabilidad de la arteria reimplantada en todos los casos y mejoría de la clínica respiratoria; uno de ellos precisó de técnicas endoscópicas (AU)

We report our experience with the surgical treatment of anomalous origin of the left pulmonary artery in eight children between 2004 and 2009. The congenital heart disease most frequently associated with this condition was patent ductus arteriosus. Surgery was carried out with extracorporeal circulation in five children, and without, in three. The anomalous pulmonary artery was divided and translocated to the main pulmonary artery. One patient died soon after surgery because of hemodynamic instability and another died later because of respiratory complications. The other patients progressed satisfactorily during follow-up: the reimplanted artery remained patent in all cases and respiratory symptoms improved. However, one patient required endoscopic treatment (AU)

Prueba de esfuerzo con función cardiopulmonar en niños operados de cardiopatía congénita. Recomendaciones de ejercicio físico en el ámbito escolar / Cardiopulmonary stress testing in children who have had congenital heart disease surgery. Physical exercise recommendations during school hours

Introducción y objetivos. El objetivo es analizar la utilidad de la prueba de esfuerzo con determinación de gases espirados en niños operados de cardiopatía congénita en edad escolar, para valorar la recomendación de ejercicio físico en relación con la cardiopatía de base, el tipo de cirugía realizada, la situación hemodinámica actual y el nivel de ejercicio habitual. Métodos. Estudio prospectivo de 108 niños que realizaron una prueba de esfuerzo con análisis de gases, monitorizando electrocardiograma y presión arterial. Se recogieron por cuestionario variables sobre cardiopatía de base, cirugía practicada, estado funcional actual y nivel de ejercicio habitual. Se emitió una recomendación de ejercicio después de la ergometría, y al año se controló por cuestionario a 35 de los pacientes. Resultados. Se observaron diferencias significativas entre la gravedad actual de la lesión y la frecuencia cardiaca de reposo y esfuerzo, la presión arterial sistólica en reposo y en esfuerzo, el consumo de oxígeno, el pulso de oxígeno, la producción de dióxido de carbono y la duración de la prueba. Se detectó relación entre el nivel de ejercicio semanal y mayores consumo de oxígeno y duración de la ergometría, pero no con la cardiopatía subyacente. En los 59 niños con lesiones cianóticas, se observó que la mejor capacidad funcional se correspondía con reparación de la lesión más precoz y mejor. Se pudo recomendar un incremento del nivel de ejercicio a 48 niños. Conclusiones. La prueba ergoespirométrica permite explorar la capacidad funcional de los niños operados de cardiopatía congénita y aporta datos importantes para una mejor planificación del ejercicio físico aconsejable (AU)

Introduction and objectives. To analyze and discover if stress testing with exhaled gases in children who have had congenital heart surgery is useful so we could make physical exercise recommendations according to heart disease, type of surgery performed, present hemodynamic state and level of exercise practiced. Methods. Prospective study of 108 children, who performed stress testing with exhaled gases, electrocardiogram monitoring and blood pressure. A questionnaire was used to obtain variables concerning heart disease, surgery, present functional condition and level of exercise practiced. Exercise recommendations were given after stress testing, and after a year 35 patients answered a questionnaire. Results. There were significant differences between lesion severity and heart rate at rest and during effort, systolic pressure at rest and during effort, oxygen uptake, oxygen pulse, carbon dioxide production and test duration. A relationship was observed between level of weekly exercise and greater oxygen uptake and test duration, but this was not observed with the underlying heart disease. We observed that best performance occurred with fast repairing for 59 children with cyanotic heart disease. Increased exercise level was recommended for 48 children. Conclusions. The cardiopulmonary function study allows us to examine the physical performance of children who have had congenital heart surgery and provides us with important data so that we can recommend better physical exercise planning (AU)

Coartación aórtica compleja y síndrome de PHACE / Complex aortic coarctation and PHACE syndrome

La asociación de malformaciones de la fosa posterior, hemangiomas cervicofaciales y vasculares, coartación y alteraciones oculares es conocida con el acrónimo PHACE. Presentamos un análisis de 4 casos de este síndrome, caracterizados por coartación aórtica compleja que necesita no sólo estudio ecocardiográfico preoperatorio, sino además técnicas como resonancia magnética y angiografía. El tratamiento quirúrgico es también complejo. El pronóstico está dado por las complicaciones cardiovasculares y arteriales cerebrales propias del síndrome (AU)

In PHACE syndrome, the acronym PHACE stands for the association of posterior fossa malformations, cervicofacial hemangiomas, arterial anomalies, coarctation and eye anomalies. We report our findings in four patients with this syndrome, in whom it was characterized by complex aortic coarctation that required not only preoperative echocardiographic investigation, but also the use of techniques such as magnetic resonance imaging and angiography. Surgical treatment was also complex. Prognosis in this condition depends primarily on cardiovascular and cerebral artery complications associated with the syndrome (AU)

Tratamiento percutáneo de la comunicación interauricular, la comunicación interventricular muscular y el ductus arterioso permeable en niños menores de un año / Percutaneous treatment of atrial septal defects, muscular ventricular septal fefects and patent ductus arteriosus in infants under one year of age

Los dispositivos Amplatzer se utilizan en el tratamiento percutáneo de la comunicación interauricular ostium secundum (CIA-OS), el ductus arterioso persistente (PDA) y la comunicación interventricular muscular (CIV-m). Hay muy poca experiencia con estos dispositivos en niños menores de 1 año. Entre enero de 2001 y enero de 2008 se trató a 22 niños menores de 1 año sintomáticos; 3 tenían CIA-OS; 15, PDA y 4, CIV-m. Todos los procedimientos fueron exitosos. No observamos complicaciones inmediatas o a mediano plazo. El cierre percutáneo de estos defectos con dispositivos Amplatzer es una técnica eficaz y segura en niños menores de 1 año sintomáticos que de otro modo requerirían cirugía (AU)

Amplatzer devices are used for the percutaneous closure of ostium secundum atrial septal defects, muscular ventricular septal defects, and patent ductus arteriosus. However, very little experience has been gained in using these devices in infants under 1 year of age. Between January 2001 and January 2008, 22 symptomatic infants aged under 1 year underwent percutaneous treatment: three had an ostium secundum atrial septal defect, 15 had patent ductus arteriosus, and 4 had a muscular ventricular septal defect. All the procedures were completed successfully. No immediate or medium-term complications were observed. Closure of these types of defect using an Amplatzer device in infants under 1 year of age, who would otherwise require surgery, is a safe and effective procedure (AU)