The impact of unilateral pulmonary artery stenosis on right ventricular to pulmonary arterial coupling in patients with transposition of the great arteries.

BACKGROUND: Unilateral pulmonary artery (PA) stenosis is common in the transposition of the great arteries (TGA) after arterial switch operation (ASO) but the effects on the right ventricle (RV) remain unclear. AIMS: To assess the effects of unilateral PA stenosis on RV afterload and function in pediatric patients with TGA-ASO. METHODS: In this retrospective study, eight TGA patients with unilateral PA stenosis underwent heart catheterization and cardiac magnetic resonance (CMR) imaging. RV pressures, RV afterload (arterial elastance [Ea]), PA compliance, RV contractility (end-systolic elastance [Ees]), RV-to-PA (RV-PA) coupling (Ees/Ea), and RV diastolic stiffness (end-diastolic elastance [Eed]) were analyzed and compared to normal values from the literature. RESULTS: In all TGA patients (mean age 12 ± 3 years), RV afterload (Ea) and RV pressures were increased whereas PA compliance was reduced. RV contractility (Ees) was decreased resulting in RV-PA uncoupling. RV diastolic stiffness (Eed) was increased. CMR-derived RV volumes, mass, and ejection fraction were preserved. CONCLUSION: Unilateral PA stenosis results in an increased RV afterload in TGA patients after ASO. RV remodeling and function remain within normal limits when analyzed by CMR but RV pressure-volume loop analysis shows impaired RV diastolic stiffness and RV contractility leading to RV-PA uncoupling.

Fetal transposition of the great arteries: 3D virtual and physical models from ultrasound datasets.

Transposition of the great arteries (TGA) is a cyanotic congenital heart disease characterized by ventriculoarterial discordance and atrioventricular concordance with the great arteries in a parallel relationship. Prenatal diagnosis of TGA has implications for postnatal outcomes, allowing for planned delivery and perinatal management. Three-dimensional virtual or physical models of fetal TGA allow better understanding of fetal cardiac anomalies by parents and interactive discussion among the multidisciplinary team (obstetricians, pediatricians, maternal-fetal specialists, pediatric cardiologists, and cardiovascular surgeons), as well as continuing medical education.

Fetal left and right ventricular strain parameters using speckle tracking in congenital heart diseases.

Assessment of fetal ventricular function is mostly subjective, and currently, for the objective assessment left ventricular shortening fraction is obtained. However, this by itself is not very reliable. Hence, more tools that can provide an objective assessment are needed to increase the confidence of functional assessment. Speckle tracking imaging can provide one such tool. In this study we sought to establish the normative value of global longitudinal and circumferential strain for our fetal patients and for two major forms of congenital heart diseases, namely atrioventricular canal defects (AVC) and uncorrected dextro-transposition of the great arteries (dTGA) to act as a benchmark. The study was completed via a single center retrospective analysis on 72 fetal echocardiograms (26 normal, 15 dTGA, and 31 AVC). Tomtec Arena™ echocardiography analysis software was used for analysis. In normal fetuses, mean left ventricular (LV) global longitudinal strain (GLS) was - 22.6% (95% CI -24, -21.1) and mean right ventricular (RV) GLS was - 22.1% (95% CI -23.6, -20.6). In AVC patients LV GLS was-26.6% (95% CI -28,-25.3) and mean RV GLS was - 26.5% (95% CI -27.9,-25.2). In dTGA patients LV GLS was - 22.9% (95% CI of -24.8, -21) and RV GLS was - 21.3% (95% CI was - 23.4, -20.8). There was good intra-rater reliability though poor to fair inter-rater reliability. Notwithstanding its current limitations, strain imaging can provide useful information that can increase confidence of cardiac functional assessment in fetal patients. However, to be reliable across the board, further automation and standardization is required.

Decreased clinical performance in TGA-ASO patients after RVOT interventions; a multicenter European collaboration.

BACKGROUND: In patients with transposition of the great arteries and an arterial switch operation (TGA-ASO) right ventricular outflow tract (RVOT) obstruction is a common complication requiring one or more RVOT interventions. OBJECTIVES: We aimed to assess cardiopulmonary exercise capacity and right ventricular function in patients stratified for type of RVOT intervention. METHODS: TGA-ASO patients (≥16 years) were stratified by type of RVOT intervention. The following outcome parameters were included: predicted (%) peak oxygen uptake (peak VO2), tricuspid annular plane systolic excursion (TAPSE), tricuspid Lateral Annular Systolic Velocity (TV S'), right ventricle (RV)-arterial coupling (defined as TAPSE/RV systolic pressure ratio), and N-terminal proBNP (NT-proBNP). RESULTS: 447 TGA patients with a mean age of 25.0 (interquartile range (IQR) 21-29) years were included. Patients without previous RVOT intervention (n = 338, 76%) had a significantly higher predicted peak VO2 (78.0 ± 17.4%) compared to patients with single approach catheter-based RVOT intervention (73.7 ± 12.7%), single approach surgical RVOT intervention (73.8 ± 28.1%), and patients with multiple approach RVOT intervention (66.2 ± 14.0%, p = 0.021). RV-arterial coupling was found to be significantly lower in patients with prior catheter-based and/or surgical RVOT intervention compared to patients without any RVOT intervention (p = 0.029). CONCLUSIONS: TGA patients after a successful arterial switch repair have a decreased exercise capacity. A considerable amount of TGA patients with either catheter or surgical RVOT intervention perform significantly worse compared to patients without RVOT interventions.

Increased aortic pressures and pulsatile afterload components promote concentric left ventricular remodeling in adults with transposition of the great arteries and arterial switch operation.

BACKGROUND: Functional abnormalities of the ascending aorta (AA) have been mainly reported in young patients who underwent arterial switch operation (ASO) for transposition of the great arteries (TGA). OBJECTIVES: To compare systolic, diastolic brachial and central blood pressures (bSBP, bDBP, cSBP, cDBP), aortic biomechanical parameters, and left ventricular (LV) afterload criteria in adult ASO patients with healthy controls and to assess their relationships with LV remodeling and aortic size. MATERIALS AND METHODS: Forty-one prospectively enrolled patients (16.8 to 35.8 years) and 41 age- and sex-matched healthy volunteers underwent cardiac MRI to assess LV remodeling with simultaneous brachial BP estimation. After MRI, carotid-femoral tonometry was performed to measure pulse wave velocity (cfPWV), cSBP and cDBP for further calculation of pulse pressure (cPP), AA distensibility (AAD), and AA and LV elastance (AAE, LVE). RESULTS: bSBP, bDBP, cSBP,cDBP and cPP were all significantly higher in ASO group than in controls: cSBP (116.5 ± 13.8 vs 106.1 ± 12.0, p < 0.001), cDBP (72.5 ± 6.9 vs 67.1 ± 9.4, p = 0.002), cPP (44.0 ± 12.1 vs 39.1 ± 8.9, p = 0.003) and not related to aortic size. AAD were decreased in ASO patients vs controls (4.70 ± 2.72 vs 6.69 ± 2.16, p < 0.001). LV mass was correlated with bSBP, cSBP, cPP (ρ = 0.48; p < 0.001), while concentric LV remodeling was correlated with AAE (ρ = 0.60, p < 0.001) and LVE (ρ = 0.32, p = 0.04), but not with distensibility. CONCLUSION: Even without reaching arterial hypertension, aortic sBP and PP are increased in the adult TGA population after ASO, altering the pulsatile components of afterload and contributing to LV concentric remodeling.

Endocarditis infecciosa en paciente con transposición de grandes arterias con reparación de Rastelli / Infective endocarditis in patient with transposition of the great arteries with Rastelli repair

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Características y evolución de la transposición de grandes vasos en el periodo neonatal / Characteristics and outcomes of transposition of great arteries in the neonatal period

Introducción y objetivos. La transposición de grandes vasos es una cardiopatía congénita frecuente, con alta supervivencia y escaso número de secuelas, especialmente si se interviene precozmente, en el periodo neonatal. Nuestro objetivo es describir las características y la evolución de los pacientes afectados y determinar posibles factores pronósticos de morbimortalidad precoz y a largo plazo. Métodos. Estudio retrospectivo mediante revisión de historias clínicas y base de datos de los pacientes con transposición de grandes vasos ingresados entre los años 2000 y 2011. Resultados. Ingresaron 136 pacientes afectados de transposición de grandes vasos, de los que 119 se sometieron a cirugía correctora durante el periodo neonatal. Los pacientes se dividieron en tres grupos: grupo I , 81 transposición de grandes vasos simple; grupo II , 24 con comunicación interventricular concomitante, y grupo III , 31 transposiciones «complejas». La supervivencia postoperatoria fue de 96,7% (115 de 119 pacientes), aunque ningún paciente del grupo I falleció. La duración de la cirugía, la intubación y el uso posquirúrgico de inotrópicos y el tiempo de ingreso fueron mayores para los pacientes de los grupos II y III . Tras una media de 6 años de seguimiento, el 90,4% (123 de 136) de los pacientes estaban vivos. La lesión residual más frecuente en el seguimiento fue la estenosis supravalvular pulmonar en 33 de 113 pacientes seguidos. Conclusiones. En nuestro estudio, la supervivencia en el conjunto de pacientes con transposición de grandes vasos, y especialmente los sometidos a switch arterial, es alta. Los pacientes con formas más complejas tienen más complicaciones hospitalarias, pero no tras el seguimiento (AU)

Introduction and objectives. Transposition of the great arteries is a prevalent congenital heart defect with a high survival rate and a good long-term outcome, especially if managed with early surgical intervention during the neonatal period. In this study, our main objective was to describe patient characteristics and outcomes and to identify possible predictors of early and long-term morbidity and mortality. Methods. Retrospective analysis through review of clinical and surgical charts of patients with transposition of the great arteries admitted to the service of neonatology during 2000-2011. Results. The study included 136 patients; 119 of them had undergone corrective surgery during the neonatal period. Patients were divided into 3 groups: group I, 81 cases of isolated transposition; group II, 24 cases with ventricular septal defect; and group III, 31 with "complex" transposition of the great arteries. The overall postoperative survival was 96.7% (115 of 119 patients); no patients from group I died after surgery. Duration of surgery, intubation, inotropic treatment, and length of stay were higher in patients in groups II and III. The overall survival rate after an average of 6 years of follow-up was 90.4% 123 of 136 patients, with no deaths after discharge in group I. The most frequent residual defect during cardiac follow-up was supravalvular pulmonary stenosis, in 33 of 113 patients that had follow-up data. Conclusions. In our study, the survival rate was high in patients with transposition of great arteries and especially in those undergoing arterial switch. The number of subsequent residual heart defects was low (AU)

Evaluación de las alteraciones de la perfusión miocárdica y función ventricular derecha con SPECT y ecocardiografía en la transposición corregida de las grandes arterias / Evaluation of myocardial perfusion and right ventricular function alterations by SPECT and echocardiography after great arteries corrected transposition

Antecedentes: La transposición corregida de grandes arterias (TCGA) es una cardiopatía congénita rara, asociada a disfunción ventricular derecha. El objetivo del presente trabajo fue evaluar por técnicas de medicina nuclear como la ventriculografía en equilibrio y SPECT sincronizado utilizando Tc-99m sestamibi reposo-esfuerzo, la función y perfusión del ventrículo derecho en pacientes adultos con TCGA no operados y comparar los resultados con el ecocardiograma convencional (ETT) y de contraste (EC). Material y métodos: Se estudiaron 13 pacientes a quienes se les realizó ventriculografía con radioisótopos para evaluar la función ventricular derecha y SPECT sincronizado para estudiar la perfusión y función del ventrículo derecho. Los resultados obtenidos se compararon con el ETT y EC. Resultados: La fracción de expulsión ventricular derecha calculada por ventriculografía tuvo correlación de 0.67 (p = 0.059) con la fracción de acortamiento de áreas y de 0.84 (p = 0.01) para el método de descenso del anillo tricuspídeo. Conclusiones: El EC y SPECT sincronizado son métodos de gran utilidad en el estudio de la perfusión miocárdica del ventrículo derecho en pacientes en el que dicho ventrículo se comporta como sistémico. El índice de concordancia diagnóstica entre estas dos técnicas obtenido fue de 0.76 (p = 0.005) para identificar defectos fijos y 0.68 (p = 0.009) para defectos reversibles.

BACKGROUND: Great arteries corrected transposition is a rare congenital heart disease associated with right ventricle dysfunction. The objective of this study was to assess by equilibrium ventriculography (EV) and gated SPECT with Tc-99m sestamibi rest-stress protocol, right ventricle function and perfusion in adult patients with CTGA without previous surgery, and to compare the results with the standard transthoracic cross-sectional (TTE) and color coded Doppler echocardiography (CE) techniques. METHODS: We studied 13 patients, who underwent ventriculography to assess right ventricle function and gated SPECT to evaluate myocardial perfusion and right ventricle function. The data obtained were compared with TTE and CE. RESULTS: Right ventricle ejection fraction was measured using nuclear medicine and showed a Pearson correlation coefficient of 0.67 (p = 0.059) with the shortening area fraction and 0.84 (p = 0.01) with the tricuspid ring descendent method. CONCLUSIONS: Gated SPECT and Echo contrast are extremely valuable methods in the assessment of myocardial perfusion of the right ventricle in this group of patients. The diagnostic agreement index of both studies was 0.76 (p = 0.005) to identify non-reversible defects and 0.68 (p = 0.009) for reversible defects.

Cardiopatías congénitas en el adulto. Aportaciones de la tomografía computarizada multidetector / Congenital heart disease in adults: the contribution of multidetector CT

Las cardiopatías congénitas (CC) constituyen actualmente una enfermedad no infrecuente en el adulto. Generalmente el paciente está diagnosticado previamente y el estudio se solicita como seguimiento o por complicaciones de la anomalía o de su corrección quirúrgica. Clásicamente, estos pacientes se estudiaban con ecocardiografía y cateterismo cardíaco, pero el abordaje ha cambiado con la tomografía computarizada multidetector (TCMD) y la resonancia magnética, ya que permiten una excelente demostración anatómica de las anomalías cardíacas y de su corrección quirúrgica. Pretendemos resaltar la importancia de la TCMD como método complementario en el estudio de las CC descubiertas en el adulto o en las corregidas quirúrgicamente en la infancia y seguidas posteriormente. Cuando existen anomalías vasculares extracardíacas o tras la realización de cirugía paliativa, la TCMD muestra detalles anatómicos difíciles o imposibles de valorar con ecocardiografía. También resaltamos la frecuente asociación de hipertensión pulmonar con CC que pueden debutar en la edad adulta (AU)

Congenital heart disease is relatively common among adults. Patients’ conditions have generally been diagnosed previously and imaging tests are requested for follow-up or for complications of the anomaly or of its surgical correction. Classically, these patients were studied with echocardiography and cardiac catheterization, but multidetector CT and magnetic resonance imaging have changed the approach because these techniques show the anatomy of heart defects and their correction very clearly. We emphasize the importance of multidetector CT as a complementary technique for the study of congenital heart disease that is newly discovered in adults or for the follow-up of congenital heart disease that was surgically corrected during childhood. When vascular anomalies are present outside the heart or after palliative surgery, multidetector CT shows anatomical details that are difficult or impossible to see with echocardiography. We also emphasize the frequent association between pulmonary hypertension and congenital heart disease that can debut in adults (AU)

Valoración ecocardiográfica de la relación comunicación interauricular/septum interauricular durante el cateterismo en niños con transposición completa de los grandes vasos / Echocardiographic evaluation of atrial septal defect/interatrial septum ratio during catheterization in infants with complete transposition of the great arteries

En 22 niños de 1 a 60 días (-x:22) con transposición completa de grandes vasos se valoró con ecocardiograma bidimensional en el postcateterismo inmediato la relación entre el tamaño de la comunicación interauricular (CIA) y la longitud del septum interauricular (SIA). En todos se comprobó incremento de la saturación, mayor del 80% del valor inicial, en ventrículo derecho y disminución franca del gradiente interauricular post-balón inmediato. Catorce niños con una relación CIA/SIA mayor de 0,20 (-x:0,24) tuvieron buena evolución clínica y fueron operados electivamente luego de los 6 meses con una relación prequirúrgica media de 0,16. Una relación menor de 0,12 (-x:0,07) se encontró en los 6 niños que requirieron un segundo cateterismo 11 a 33 días (-x:21) luego del primer procedimiento, con una relación media de 0,05. En conclusión; la relación CIA/SIA tendió a disminuir en función del tiempo, la evolución clínica tuvo relación directa con la relación CIA/SIA inicial y no hubo correlación entre saturación y gradiente interauricular inicial y evolución clínica. Pensamos que el cateterismo terapéutico debe realizarse con control ecocardiográfico no sólo para disminuir los riesgos sino para lograr una relación CIA/SIA mayor de 0,20 previniendo la necesidad de un segundo cateterismo terapéutico o de una cirugía temprana con mayor riesgo

Transposição dos grandes vasos da base: análise de 32 casos de necropsias pediátricas e revisão da literatura / Great vessels transposition: analyse 32 pediatric cases of autopsis and literature reviews

Objetivos: analisar aspectos epidemiologicos e anatomopatologicos de casos de transposição dos grandes vasos da base em necropsias perinatais e pediatricas.Métodos: avaliados 5.837 necropsias realizadas entre 1960 e 1995, 32 apresentavam TGVB.Dados como sexo, idade, sintomatologia, alterações anatomopatológicas associadas e procedimentos cirurgicos realizados foram analisados. Resultados: a relação masculino:feminino foi de 1,13:1. A idade variou desde natimortos até dez anos, com uma sobrevida média de 434 dias. a TGVB com septo sem persistência de canal arterial aconteceu em sete caos (21,87por cento). Ocorreu comunicação interatrial (CIA) ou interventricular (CIV) isolada em 25 por cento dos casos. A Associação de CIA e CIV foi a anormalidade mais encontrada. Conclusões: a TGVB tem sido citada como sendo predominantemente masculina. Neste estudo não se relacionou ao sexo. Houve tendência estatistica de a TGVB levar a óbito intra-uterino mais do que outras cardiopatias

Transposición corregida de las grandes arterias en pacientes mayores de 65 años / Corrected transposition of the great arteries in the elderly

Describimos tres enfermos con transpocisión corregida de las grandes arterias, mayores de 65 años. El primero con una sobrevida de 80 años tenía situs inversus atrial, estenosis valvular pulmonar calcificada, pequeña comunicación interventricular y discreta regurgitación tricuspídea. El segundo era portador de situs solitus atrial, con comunicación interatrial e interventricular amplias. El último también tenía situs solitus atrial y disctera regurgitación tricuspídea, diagnosticada por ecocardiografía y angiocardiografía. La evolución clínica de uno de estos enfermos mostró que los defectos septales evolucionan al igual que en los enfermos portadores de conexiones atrioventriculares y ventriculoarteriales concordantes, son pacientes que desarrollan hipertensión arterial pulmonar y posteriormente insuficiencia cardíaca secundaria. El primer sujeto de esta serie tenía como lesión significativa una estenosis pulmonar valvular de cierta importancia, que se calcificó y cuya asociación con cardiopatía isquémica tuvo un papel importante en la génesis de la insuficiencia cardíaca. El tercer sujeto, para fines prácticos sin defectos asociados, tuvo como complicación una enfermedad vascular cerebral. Ninguno desarrolló bloqueo atrioventricular, a pesar de la larga evolución. Estos tres casos ejemplifican una evolución natural poco habitual de la transpiración corregida de las grandes arterias