Longitudinal changes in fetal head biometry and fetoplacental circulation in fetuses with congenital heart defects.

INTRODUCTION: Objectives were to analyze changes in fetal cephalic biometry and fetoplacental circulation throughout pregnancy in fetuses with congenital heart defects. MATERIAL AND METHODS: Prospective study conducted on three university tertiary referral hospitals. Fetuses with the diagnosis of isolated congenital heart defects attending between 2014 and 2018 were included. Congenital heart defects were divided into two groups according to the oxygen supply to the central nervous system: group I (expected low placental blood content and low oxygen delivery to the brain) and group II (expected intermediate and high placental blood content). Fetal biometry and Doppler parameters were collected between 25-30 weeks of gestation and 34-40 weeks of gestation and transformed into Z scores. The results were compared with healthy controls. Finally, general linear modeling was performed to analyze repeated measurements. RESULTS: In all, 71 fetuses met the inclusion criteria. Fetuses with congenital heart defects had significantly smaller head (biparietal diameter [p < 0.001], head circumference [p = 0.001]) and abdominal circumference (p < 0.001), and lower estimated fetal weight (p < 0.001) than controls. When analyzing according to congenital heart defects type, head size was significantly smaller in group I compared with group II (p = 0.04). Regarding Doppler parameters, fetuses with congenital heart defects showed higher umbilical artery pulsatility index (p < 0.001) and lower cerebroplacental ratio (p = 0.044) than controls. In group I, umbilical artery pulsatility index was above the 95th centile in 15.4% of fetuses compared with 6.7% in group II and 1.9% in controls (p < 0.001); moreover, middle cerebral artery pulsatility index was below the 5th centile in 5.4% of group I fetuses compared with 0% in group II and 1.2% in controls (p = 0.011). General linear model for two measurements showed significant longitudinal changes in biometric parameters. Growth rate of fetal head through pregnancy (head circumference Z score) was lower in fetuses with congenital heart defects compared with controls (p = 0.043). In group I, the head circumference Z score longitudinal decrease was significantly higher than in group II (p < 0.001). CONCLUSIONS: Fetuses with congenital heart defects are at risk of smaller head size and Doppler changes. Growth rate of fetal head throughout pregnancy is also affected. These findings are mainly associated with cardiac defects with expected low oxygen supply to the central nervous system.

Evaluación de doble arco aórtico por ecografía y tomografía computarizada / Evaluation of double aortic arch by ultrasound and computed tomography

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Pulmonary hypertension in bronchopulmonary dysplasia: clinical findings, cardiovascular anomalies and outcomes.

OBJECTIVE: Pulmonary hypertension (PH) worsens the prognosis of bronchopulmonary dysplasia (BPD). The following items have not been fully established for PH in BPD: clinical characterization, incidence of cardiovascular anomalies (CVAs), response to PH treatment, and outcome. STUDY DESIGN: A review of clinical records, computed tomography (CT) images and catheterization data of 36 patients with PH-BPD referred to our PH Unit (March 2006 to December 2011) was performed. Twenty-nine patients without major congenital heart defects and with complete follow-up data were included. RESULTS: The diagnosis of PH was made at a median age of 4.5 months (IQR 2.4-7.8), with an echocardiography estimated median right ventricular pressure/systemic pressure ratio of 70% (IQR 60-80%). CT scanning was performed in 21 patients and catheterization in 14 patients. CVAs were found in 19 patients (65.5%): aortopulmonary collaterals (n = 9), pulmonary vein stenosis (n = 7), ASD (n = 4), and PDA (n = 9). Hemodynamic data: PVRI 4.3 UW m(2) (2.7-7); PVRI/SVRI 0.44 (0.32-0.8); and transpulmonary gradient 28 mmHg (19-40). At a median follow-up of 35 months (IQR 21-91), 6 patients had undergone shunts closure, 22 received specific PH drugs, 3 spontaneously improved of their PH, and 8 (26%) had died. CONCLUSION: PH in BPD is not always a transient condition; it can be diagnosed at later stages and can have a protracted course. The incidence of associated CVAs is high. Prompt diagnosis, detection, and treatment of CVAs, and specific drug therapy can improve the outcome in these patients, although the mortality rate remains high.

Determinación de valores normalizados del desplazamiento sistólico del plano del anillo tricuspídeo (TAPSE) en 405 niños y adolescentes españoles / Determination of normalized values of the tricuspid annular plane systolic excursion (TAPSE) in 405 spanish children and adolescents

Introducción y objetivos. El desplazamiento sistólico del plano del anillo tricuspídeo (siglas en inglés, TAPSE) es una medida ecocardiográfica que permite evaluar adecuadamente la función sistólica del ventrículo derecho y se emplea habitualmente en adultos. No obstante, hay poca información sobre los valores de referencia de TAPSE en niños. Nuestro objetivo fue determinar valores de referencia de TAPSE en niños españoles y evaluar su relación con la edad y la superficie corporal. Métodos. Incluimos prospectivamente a 405 sujetos (neonatos hasta 18 años, el 53% varones) remitidos para evaluación de soplo cardiaco. Excluimos a los sujetos con cardiopatía o cualquier otra enfermedad. Recogimos la medida por modo M de TAPSE y variables antropométricas. Analizamos la reproducibilidad intraobservador e interobservadores de las mediciones. Resultados. El TAPSE medio fue 17,09±5,09cm, sin diferencias significativas entre sexos. Se demostró adecuado un modelo de regresión curvilínea, con valores incrementales proporcionales a los estratos de edad, talla, peso, índice de masa corporal y superficie corporal. La superficie corporal presentó una importante correlación positiva con los valores de TAPSE (r=0,81), mientras que la frecuencia mantuvo una correlación negativa (r=−0,74). El análisis multivariable confirmó estas correlaciones, así como las interacciones entre variables (edad, talla, peso, superficie corporal). Aportamos gráficas con las estimaciones poblacionales normales para TAPSE ajustadas por edad y superficie corporal. Conclusiones. Presentamos valores de referencia de TAPSE para población pediátrica española. La medida del TAPSE fue reproducible y se relacionó directamente con la edad y la superficie corporal. Estos valores de referencia podrían guiar la toma clínica de decisiones (AU)

Introduction and objectives. Tricuspid annular plane systolic excursion (TAPSE) is an echocardiographic measure that allows us to assess right ventricular systolic function. TAPSE measurement is common in adults but reference values for children are scarce. Our objective was to establish reference values for TAPSE in Spanish children and to determine the relationship of these values with age and body surface. Methods. This prospective study included 405 patients (from newborn to age 18 years, 53% male) referred for assessment of cardiac murmurs. Patients with confirmed cardiac or any other disease were excluded. We collected TAPSE measurements by M-mode echocardiography and recorded anthropometric variables. We analyzed the intra- and interobserver reproducibility of these measurements. Results. Mean TAPSE values were 17.09±5.09cm with nonsignificant differences between sexes. A curvilinear regression model proved appropriate, with values increasing in proportion to age group, height, weight, body mass index, and body surface. Body surface showed a strong positive correlation with TAPSE values (r=0.81), whereas frequency had a negative correlation (r=–0.74). Multivariate analysis confirmed these correlations and the interactions between variables (age, height, weight, body surface). Graphs of estimated normal population-based TAPSE values adjusted by age and body surface are provided. Conclusions. We present reference values for TAPSE in Spanish children and adolescents. The TAPSE measurement was reproducible and associated directly with age and body surface. These reference values could guide decision making in daily clinical practice (AU)

Determination of normalized values of the tricuspid annular plane systolic excursion (TAPSE) in 405 Spanish children and adolescents.

INTRODUCTION AND OBJECTIVES: Tricuspid annular plane systolic excursion (TAPSE) is an echocardiographic measure that allows us to assess right ventricular systolic function. TAPSE measurement is common in adults but reference values for children are scarce. Our objective was to establish reference values for TAPSE in Spanish children and to determine the relationship of these values with age and body surface. METHODS: This prospective study included 405 patients (from newborn to age 18 years, 53% male) referred for assessment of cardiac murmurs. Patients with confirmed cardiac or any other disease were excluded. We collected TAPSE measurements by M-mode echocardiography and recorded anthropometric variables. We analyzed the intra- and interobserver reproducibility of these measurements. RESULTS: Mean TAPSE values were 17.09 ± 5.09 cm with nonsignificant differences between sexes. A curvilinear regression model proved appropriate, with values increasing in proportion to age group, height, weight, body mass index, and body surface. Body surface showed a strong positive correlation with TAPSE values (r = 0.81), whereas frequency had a negative correlation (r = -0.74). Multivariate analysis confirmed these correlations and the interactions between variables (age, height, weight, body surface). Graphs of estimated normal population-based TAPSE values adjusted by age and body surface are provided. CONCLUSIONS: We present reference values for TAPSE in Spanish children and adolescents. The TAPSE measurement was reproducible and associated directly with age and body surface. These reference values could guide decision making in daily clinical practice.

Situs ambiguo asociado a cardiopatía e interrupción de la vena cava inferior con continuidad de la ácigos / Situs ambiguus associated with congenital heart disease and interruption of the inferior vena, cava and with azygos continuation

Entre la lateralidad habitual (situs solitus) y laimagen completa en espejo (situs inversus) seencuentra el situs ambiguo o heterotaxia. Sus dosmodalidades principales son el isomerismoizquierdo (con poliesplenia) o el derecho (conasplenia). La heterotaxia implica alteraciones en lamovilidad ciliar que dificultan la migración deórganos embrionarios. Presenta malposición deórganos toracoabdominales, cardiopatías complejasy otras malformaciones. Presentamos un caso dediagnóstico ecográfico prenatal de isomerismoizquierdo, hígado a la izquierda y asplenia,asociado a cardiopatía e interrupción de la venacava inferior con continuidad de la ácigos. Lalateralidad de los órganos fetales debe ser partedel examen ultrasonográfico rutinario, por lasfrecuentes malformaciones asociadas a laheterotaxia

There is a spectrum of heterotaxic syndromesbetween normal organ distribution (situs solitus)and congenital conditions in which major organsare mirrored from their normal position (situsinversus). The two main modalities are leftisomerism (with polysplenia) and right isomerism(with asplenia). Heterotaxic defects involve ciliarydyskinesia, hampering migration of embryonicorgans and leading to malposition of thoracic andabdominal organs, complex cardiac defects, andother malformations. We present a case of prenatalechographic diagnosis of levocardia, with left-sidedliver, asplenia, congenital heart disease, andinterruption of the inferior vena cava with azygoscontinuation. Because of the malformationsfrequently associated with heterotaxy, the positionof fetal organs should form part of routineultrasonographic examination (AU)

[Interventional catheterization after the Norwood procedure]. / Cateterismo intervencionista tras cirugía tipo Norwood.

INTRODUCTION AND OBJECTIVES: To carry out a retrospective analysis of the indications for, and the results and complications of interventional catheterization after the Norwood procedure. METHODS: Between February 1993 and December 2006, 25 interventional catheterizations were performed in 14 patients who had undergone the Norwood procedure, prior to the Glenn or Fontan procedure. RESULTS: Nine angioplasties were carried out for recoarctation in seven of the 14 patients (2 patients developed restenosis after their first angioplasty). Detachment of the left pulmonary artery occurred either immediately or during follow-up in 3 patients who underwent the classical Norwood procedure. Overall, 10 pulmonary artery angioplasties were required in 7 patients. Three patients needed embolization: one of venous collaterals (using coils), one of the left superior vena cava (using an Amplatzer duct occluder), and one of a left Blalock-Taussig shunt (using an Amplatzer duct occluder). Two patients required a cavopulmonary (Glenn) anastomosis, and another underwent fibrinolysis for thrombosis of the superior vena cava and pulmonary artery. Other findings, which were not treated percutaneously, included: stenosis of the supra-aortic trunk (n=6), femoral artery stenosis (n=2), femoral vein thrombosis (n=5), and subclavian vein thrombosis (n=1). The following complications were recorded: arterial ischemia (n=2), cardiac arrest or bradycardia (n=4), and transient atrioventricular block (n=1). CONCLUSIONS: Following stage I of the Norwood procedure, the angiographic and hemodynamic assessments needed for the diagnosis and treatment of pulmonary artery or aortic arch stenosis must be carried out promptly. Although treating recoarctation by angioplasty can be effective, restenosis frequently occurs. With the Sano procedure, detachment of the left pulmonary artery, but not pulmonary artery stenosis, can be avoided. After stage II, the presence of venovenous collaterals must be ruled out, because they frequently require embolization. In these patients, interventional catheterization is associated with a higher incidence of complications than in other groups.

Cateterismo intervencionista tras cirugía tipo Norwood / Interventional catheterization after the Norwood procedure

Introducción y objetivos. Análisis retrospectivo de las indicaciones, los resultados y las complicaciones del cateterismo terapéutico en pacientes tras cirugía tipo Norwood. Métodos. Entre febrero de 1993 y diciembre de 2006 se realizaron 25 procedimientos intervencionistas en 14 pacientes con cirugía tipo Norwood, previo al Glenn o Fontan. Resultados. En 7 de los 14 pacientes se realizaron 9 angioplastias de recoartación (2 presentaron nuevamente reestenosis tras la primera angioplastia). En 3 pacientes con Norwood clásico se encontró inicialmente o evolutivamente desconexión de arteria pulmonar izquierda; 7 pacientes precisaron 10 angioplastias de arterias pulmonares; 3 pacientes precisaron embolizaciones: de colaterales venosas (n = 1, con coils), de vena cava superior izquierda (n = 1, con oclusor Amplatzer para ductus) y de fístula Blalock-Taussig izquierdo (n = 1, con oclusor Amplatzer para ductus). En 2 casos se realizó angioplastia del Glenn y en otro, fibrinolisis de trombosis de vena cava superior y arteria pulmonar. Otros hallazgos, sobre los que no se realizaron técnicas intervencionistas, fueron: estenosis de troncos supraaórticos (n = 6), estenosis en las arterias femorales (n = 2), trombosis venosa femoral (n = 5) o de vena subclavia (n = 1). Como complicaciones se registraron: isquemia arterial (n = 2), parada cardiaca o bradicardia (n = 4) y bloqueo auriculoventricular transitorio (n = 1). Conclusiones. Tras la cirugía tipo Norwood debe realizarse una valoración angiohemodinámica precoz para detectar y tratar estenosis de arterias pulmonares y/o del arco aórtico. Aunque la angioplastia de la recoartación puede ser efectiva, la reestenosis es frecuente. La técnica de Sano evita la desconexión de la arteria pulmonar izquierda, pero no el desarrollo de estenosis de arterias pulmonares. Tras el estadio II, debe estudiarse si hay colaterales venovenosas, pues éstas con frecuencia precisan embolización. El cateterismo terapéutico en estos pacientes comporta una incidencia de complicaciones mayor que en otros grupos de pacientes (AU)

Introduction and objectives. To carry out a retrospective analysis of the indications for, and the results and complications of interventional catheterization after the Norwood procedure. Methods. Between February 1993 and December 2006, 25 interventional catheterizations were performed in 14 patients who had undergone the Norwood procedure, prior to the Glenn or Fontan procedure. Results. Nine angioplasties were carried out for recoarctation in seven of the 14 patients (2 patients developed restenosis after their first angioplasty). Detachment of the left pulmonary artery occurred either immediately or during follow-up in 3 patients who underwent the classical Norwood procedure. Overall, 10 pulmonary artery angioplasties were required in 7 patients. Three patients needed embolization: one of venous collaterals (using coils), one of the left superior vena cava (using an Amplatzer duct occluder), and one of a left Blalock-Taussig shunt (using an Amplatzer duct occluder). Two patients required a cavopulmonary (Glenn) anastomosis, and another underwent fibrinolysis for thrombosis of the superior vena cava and pulmonary artery. Other findings, which were not treated percutaneously, included: stenosis of the supra-aortic trunk (n=6), femoral artery stenosis (n=2), femoral vein thrombosis (n=5), and subclavian vein thrombosis (n=1). The following complications were recorded: arterial ischemia (n=2), cardiac arrest or bradycardia (n=4), and transient atrioventricular block (n=1). Conclusions. Following stage I of the Norwood procedure, the angiographic and hemodynamic assessments needed for the diagnosis and treatment of pulmonary artery or aortic arch stenosis must be carried out promptly. Although treating recoarctation by angioplasty can be effective, restenosis frequently occurs. With the Sano procedure, detachment of the left pulmonary artery, but not pulmonary artery stenosis, can be avoided. After stage II, the presence of venovenous collaterals must be ruled out, because they frequently require embolization. In these patients, interventional catheterization is associated with a higher incidence of complications than in other groups (AU)