Acquired anomalies of the coronary arteries after arterial switch operation. Usefulness of coronary computed tomography angiography and impact on follow-up.

INTRODUCTION: Mortality and morbidity in patients with transposition of the great arteries after an arterial switch operation depends mainly on the status of coronary perfusion. Coronary computed tomography angiography (CCTA) provides accurate information on coronary morphology, however its use in these patients is not yet routine procedure. OBJECTIVE: We sought to assess its accuracy to identify acquired coronary anomalies in this population, compared to conventional angiography in a subset of patients, and assess its impact on postoperative management. METHODS: Retrospective analysis of clinical data on transposition of the great arteries in patients who underwent CCTA between January 2013 and September 2017. RESULTS: Between January 2013 and September 2017, 18 patients underwent CCTA. Seven patients (39%) disclosed iatrogenic coronary lesions (stenosis 1; kinking 2, occlusion 1; filiform coronary 3). The exam was performed in 78% of patients due to suggestion of myocardial ischemia (symptoms or altered exams). Only 16% needed to undergo additional exams, and in four patients the CCTA result modified therapeutic management. Conventional coronary angiography was also performed in 10 patients (55%), and in three cases, the results were discordant with underestimation or non-identification of coronary lesions on conventional angiography. The medium radiation dose used was 2.4 mSv and no complications after CT were reported. CONCLUSION: CCTA accurately identified iatrogenic postoperative coronary lesions and it has proven to be superior to conventional angiography in this population. It should be performed routinely in this group of patients, even in the absence of symptoms.

Acquired anomalies of the coronary arteries after arterial switch operation. Usefulness of coronary computed tomography angiography and impact on follow-up.

INTRODUCTION: Mortality and morbidity in patients with transposition of the great arteries after an arterial switch operation depends mainly on the status of coronary perfusion. Coronary computed tomography angiography (CCTA) provides accurate information on coronary morphology, however its use in these patients is not yet routine procedure. OBJECTIVE: We sought to assess its accuracy to identify acquired coronary anomalies in this population, compared to conventional angiography in a subset of patients, and assess its impact on postoperative management. METHODS: Retrospective analysis of clinical data on transposition of the great arteries in patients who underwent CCTA between January 2013 and September 2017. RESULTS: Between January 2013 and September 2017, 18 patients underwent CCTA. Seven patients (39%) disclosed iatrogenic coronary lesions (stenosis 1; kinking 2, occlusion 1; filiform coronary 3). The exam was performed in 78% of patients due to suggestion of myocardial ischemia (symptoms or altered exams). Only 16% needed to undergo additional exams, and in four patients the CCTA result modified therapeutic management. Conventional coronary angiography was also performed in 10 patients (55%), and in three cases, the results were discordant with underestimation or non-identification of coronary lesions on conventional angiography. The medium radiation dose used was 2.4 mSv and no complications after CT were reported. CONCLUSION: CCTA accurately identified iatrogenic postoperative coronary lesions and it has proven to be superior to conventional angiography in this population. It should be performed routinely in this group of patients, even in the absence of symptoms.

Progresión y predictores de insuficiencia valvular neoaórtica y dilatación de la raíz neoaórtica en adultos jóvenes tras cirugía de switch arterial / Time course and predictors for neoaortic root dilatation and neoaortic valve regurgitation during adult life after arterial switch operation

Introducción y objetivos Hay pocos datos sobre la evolución en adultos de la dilatación de la raíz neoaórtica (RAO) y la insuficiencia valvular neoaórtica (IA) tras la cirugía de switch arterial (SA) en la transposición de grandes arterias. Métodos Análisis retrospectivo de 152 pacientes con transposición de grandes arterias, mayores de 15 años, intervenidos mediante SA y seguidos durante 4,9±3,3 años en 2 centros de referencia. Se analizaron los cambios de diámetro de la RAO ajustados a superficie corporal y la progresión a grado moderado/grave de la IA con ecocardiografías seriadas. Se realizó un modelo de regresión de Cox para identificar factores predictores de progresión de la IA. Resultados Inicialmente, 4 pacientes (2,6%) presentaban IA grave (3 habían precisado cirugía valvular) y 9 (5,9%) moderada. La RAO basal media era 20,05±2,4mm/m2, y al final del seguimiento, 20,73±2,8mm/m2 (p<0,001), con un crecimiento medio de 0,14 (IC95%, 0,07-0,2) mm/m2/año. La IA progresó en 20 (13,5%) y 6 (4%) fueron intervenidos. La progresión de IA se asoció con válvula bicúspide, IA inicial, dilatación de la RAO inicial y crecimiento de la RAO. La válvula bicúspide (HR=3,3; IC95%, 1,1-15,2; p=0,037), la IA inicial (HR=5,9; IC95%, 1,6-59,2; p=0,006) y el crecimiento de la RAO (HR=4,1; IC95%, 2-13,5; p=0,023) resultaron predictores independientes. Conclusiones La dilatación de la RAO y la IA progresan en el adulto joven intervenido mediante SA. La válvula bicúspide, la IA basal y el crecimiento de la RAO son predictores de progresión de IA. (AU)

Introduction and objectives There are limited data on the long-term development of neoaortic root dilatation (NRD) and neoaortic valve regurgitation (AR) after arterial switch operation (ASO) for transposition of the great arteries during adult life. Methods We performed a retrospective longitudinal analysis of 152 patients older than 15 years who underwent ASO for transposition of the great arteries and who were followed-up for 4.9±3.3 years in 2 referral centers. Sequential changes in body surface-adjusted aortic root dimensions and progression to moderate/severe AR were determined in patients with 2 or more echocardiographic examinations. Risk factors for dilatation were tested by Cox regression to identify predictors of AR progression. Results At baseline, moderate AR was present in 9 patients (5.9%) and severe AR in 4 (2.6%), of whom 3 had required aortic valve surgery. Initially, the median neoaortic root dimension was 20.05±2.4mm/m2, which increased significantly to 20.73±2.8mm/m2 (P <.001) at the end of follow-up. The mean change over time was 0.14mm/m2/y (95%CI, 0.07-0.2). Progressive AR was observed in 20 patients (13.5%) and 6 patients (4%) required aortic valve surgery. Progressive AR was associated with bicuspid valve, AR at baseline, NRD at baseline, and neoaortic root enlargement. Independent predictors were bicuspid valve (HR, 3.3; 95%CI, 1.1-15.2; P=.037), AR at baseline (HR, 5.9; 95%CI, 1.6-59.2; P=.006) and increase in NRD (HR, 4.1 95%CI, 2-13.5; P=.023). Conclusions In adult life, NRD and AR progress over time after ASO. Predictors of progressive AR are bicuspid valve, AR at baseline, and increase in NRD. (AU)

Time course and predictors for neoaortic root dilatation and neoaortic valve regurgitation during adult life after arterial switch operation.

INTRODUCTION AND OBJECTIVES: There are limited data on the long-term development of neoaortic root dilatation (NRD) and neoaortic valve regurgitation (AR) after arterial switch operation (ASO) for transposition of the great arteries during adult life. METHODS: We performed a retrospective longitudinal analysis of 152 patients older than 15 years who underwent ASO for transposition of the great arteries and who were followed-up for 4.9±3.3 years in 2 referral centers. Sequential changes in body surface-adjusted aortic root dimensions and progression to moderate/severe AR were determined in patients with 2 or more echocardiographic examinations. Risk factors for dilatation were tested by Cox regression to identify predictors of AR progression. RESULTS: At baseline, moderate AR was present in 9 patients (5.9%) and severe AR in 4 (2.6%), of whom 3 had required aortic valve surgery. Initially, the median neoaortic root dimension was 20.05±2.4mm/m2, which increased significantly to 20.73±2.8mm/m2 (P <.001) at the end of follow-up. The mean change over time was 0.14mm/m2/y (95%CI, 0.07-0.2). Progressive AR was observed in 20 patients (13.5%) and 6 patients (4%) required aortic valve surgery. Progressive AR was associated with bicuspid valve, AR at baseline, NRD at baseline, and neoaortic root enlargement. Independent predictors were bicuspid valve (HR, 3.3; 95%CI, 1.1-15.2; P=.037), AR at baseline (HR, 5.9; 95%CI, 1.6-59.2; P=.006) and increase in NRD (HR, 4.1 95%CI, 2-13.5; P=.023). CONCLUSIONS: In adult life, NRD and AR progress over time after ASO. Predictors of progressive AR are bicuspid valve, AR at baseline, and increase in NRD.

Influences on the Functional Behavior of Great Arteries during Orthostasis / Gravidade Influencia o Comportamento Funcional das Grandes Artérias Durante a Ortostase

Abstract Background: Arterial compliance reduction has been associated with aging and hypertension in supine position. However, the dynamic effects of orthostatism on aortic distensibility has not been defined. Objective: We sought to determine the orthostatic influence and the interference of age, blood pressure (BP) and heart rate (HR) on the great arteries during gravitational stress. Methods: Ninety-three healthy volunteers (age 42 ± 16 years). Carotid-femoral pulse wave velocity (PWV) assumed as aortic stiffness was assessed in supine position (basal phase), during tilt test (TT) (orthostatic phase) and after return to supine position (recovery phase). Simultaneously with PWV acquisition, measures of BP and HR rate were recorded. Results: PWV during TT increased significantly compared to the basal and recovery phases (11.7 ± 2.5 m/s vs. 10.1 ± 2.3 m/s and 9.5 ± 2.0 m/s). Systolic BP (r = 0.55, r = 0.46 and r = 0.39) and age (r = 0.59, r = 0.63 and r = 0.39) correlated with PWV in all phases. The significance level for all tests was established as α = 0.05. Conclusion: We conclude that there is a permanent increase in PWV during orthostatic position that was returned to basal level at the recovery phase. This dynamic pattern of PWV response, during postural changes, can be explained by an increase in hydrostatic pressure at the level of abdominal aorta which with smaller radius and an increased elastic modulus, propagates the pulse in a faster way. Considering that it could increase central pulse reflection during the orthostatic position, we speculate that this mechanism may play a role in the overall adaptation of humans to gravitational stress.

Resumo Fundamento: A redução da complacência arterial tem sido associada ao envelhecimento e à hipertensão na postura supina. Entretanto, os efeitos dinâmicos do ortostatismo na distensibilidade aórtica não foram definidos. Objetivo: Determinar a influência ortostática e a interferência da idade, pressão arterial (PA) e frequência cardíaca (FC) sobre as grandes artérias durante o estresse gravitacional. Métodos: Noventa e três voluntários saudáveis (idade de 42 ± 16 anos). A velocidade da onda de pulso carotídeo-femoral (VOP), assumida como rigidez aórtica, foi avaliada na posição supina (fase basal) durante o teste de inclinação (TT) (fase ortostática) e após o retorno à posição supina (fase de recuperação). Simultaneamente à aquisição da PWV, registrou-se as medidas de PA e FC. Resultados: A VOP durante o TT aumentou significativamente em comparação com as fases basal e de recuperação (11,7 ± 2,5 m/s vs. 10,1 ± 2,3 m/se 9,5 ± 2,0 m/s). PA sistólica (r = 0,55, r = 0,46 e r = 0,39) e idade (r = 0,59, r = 0,63 e r = 0,39) correlacionaram-se com a VOP em todas as fases. O nível de significância para todos os testes foi estabelecido como = 0,05. Conclusão: Observou-se um aumento permanente da VOP durante a postura ortostática, que retornou ao nível basal na fase de recuperação. Esse padrão dinâmico de resposta da VOP, durante as alterações posturais, pode ser explicado pelo aumento da pressão hidrostática no nível da aorta abdominal que, com raio menor e aumento do módulo de elasticidade, propaga o pulso de maneira mais rápida. Considerando-se que poderia aumentar a reflexão do pulso central durante a posição ortostática, podemos especular que esse mecanismo pode desempenhar um papel na adaptação global do humano ao estresse gravitacional.

Pulmonary Arterial Hypertension and Neonatal Arterial Switch Surgery for Correction of Transposition of the Great Arteries.

INTRODUCTION AND OBJECTIVES: There are few reports of the appearance of pulmonary arterial hypertension following arterial switch surgery in the neonatal period to correct transposition of the great arteries. We assessed the frequency and clinical pattern of this complication in our series of patients. METHODS: Our database was reviewed to select patients with transposition of the great arteries corrected by neonatal arterial switch at our hospital and who developed pulmonary hypertension over time. RESULTS: We identified 2 (1.3%) patients with transposition of the great arteries successfully repaired in the first week of life who later experienced pulmonary arterial hypertension. The first patient was a 7-year-old girl diagnosed with severe pulmonary hypertension at age 8 months who did not respond to medical treatment and required lung transplantation. The anatomic pathology findings were consistent with severe pulmonary arterial hypertension. The second patient was a 24-month-old boy diagnosed with severe pulmonary hypertension at age 13 months who did not respond to medical therapy. CONCLUSIONS: Pulmonary hypertension is a rare but very severe complication that should be investigated in all patients with transposition of the great arteries who have undergone neonatal arterial switch, in order to start early aggressive therapy for affected patients, given the poor therapeutic response and poor prognosis involved.

Characteristics and outcomes of transposition of great arteries in the neonatal period.

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.

Estudio caso-control de uso de complejo protrombínico en cirugía de switch arterial / Case-control study of use of complex protrombinico in surgery of arterial switch

Objetivo: El objetivo del siguiente trabajo fue evaluar si existió diferencia de reposición de hematocomponentes intra y postoperatoria, de sangrado y de reintervención en las primeras 24 horas, en pacientes intervenidos de switch arterial, según uso o no de complejo protombínico. Material y métodos: Se describieron las variables en estudio por grupo o para toda la población. Se utilizaron el test de Fischer y t de Student para comparar los grupos, con una significacion estadística alfa=0.05. Resultados: No hubo diferencias en cuento a sexo, edad, peso, tiempo de circulación extracorpórea, ni clampeo aórtico entre los dos grupos. La saturación al ingreso en los pacientes que se usó complejo fue menor que los del grupo control (p=0.044). Hubo mayor número de pacientes tratados con plasma intraoperatorio en los que no se administró complejo (p=0.046) y mayor volúmen de resposición con crioprecipitados también en el grupo sin complejo (p=0.02). Los pacientes que salieron con tórax abierto tuvieron mayor reposición de sangre desplasmatizada (p=0.041) y plaquetas (p=0.029) independientemente de su pertenencia al grupo con o sin complejo. No hubo diferencias con respecto al sangrado por los drenajes. Hubo solo un caso con reintervención por sangrado en el grupo control. Conclusiones: Hubo una tendencia de reposición mayor con plasma y crioprecipitados en el intraoperatorio en los pacientes que no se usó complejo protombínico, con significación estadística.