RESUMO
CLINICAL DATA: A nine-month-old female infant diagnosed with transposition of the great arteries with symptoms of heart failure associated with cyanosis and difficulty in gaining weight was referred to our center with late diagnosis (at nine months of age). CHEST RADIOGRAPHY: Cardiomegaly; attenuated peripheral vascular markings.Electrocardiography: Sinus rhythm with biventricular overload and aberrantly conducted supraventricular extra systoles. ECHOCARDIOGRAPHY: Wide atrial septal defect, ventricular axis torsion with concordant atrioventricular connection and discordant ventriculoarterial connection. COMPUTED TOMOGRAPHY ANGIOGRAPHY: Concordant atrioventricular connection, right ventricle positioned superiorly and left ventricle positioned inferiorly; discordant ventriculoarterial connection with right ventricle connected to the aorta and left ventricle connected to pulmonary artery. DIAGNOSIS: Crisscross heart is a rare congenital heart defect, accounting for 0.1% of congenital heart diseases. It consists of the 90º rotation of ventricles' axis in relation to their normal position; therefore, ventricles are positioned in the superior-inferior direction rather than anterior-posterior. Most cases have associated cardiac anomalies, and in this case, it is associated with transposition of the great arteries. The complexity and rarity of its occurrence make diagnosis and surgical treatment challenging. OPERATION: Modified Senning procedure using the pericardial sac in the construction of a tunnel from pulmonary veins to the right atrium. Cardiopulmonary bypass time of 147 minutes with nine minutes of total circulatory arrest.
Assuntos
Coração Entrecruzado , Transposição dos Grandes Vasos , Humanos , Feminino , Transposição dos Grandes Vasos/cirurgia , Transposição dos Grandes Vasos/diagnóstico por imagem , Lactente , Coração Entrecruzado/cirurgia , Coração Entrecruzado/diagnóstico por imagem , Ecocardiografia , Resultado do Tratamento , Transposição das Grandes Artérias/métodos , Angiografia por Tomografia Computadorizada , EletrocardiografiaRESUMO
Congenitally correct transposition of the great arteries (cc-TGA) is an extremely rare congenital cardiac malposition. It can be detected antenatally by echocardiography. This case report describes a 58-year-old female patient who presented with tachycardia. The combination of cc-TGA and isolated levocardia is incidentally diagnosed by transthoracic echocardiography and cardiac magnetic resonance imaging.
Assuntos
Ecocardiografia , Levocardia , Transposição dos Grandes Vasos , Humanos , Feminino , Transposição dos Grandes Vasos/diagnóstico por imagem , Pessoa de Meia-Idade , Ecocardiografia/métodos , Levocardia/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodosAssuntos
Amniocentese , Hibridização Genômica Comparativa , Dupla Via de Saída do Ventrículo Direito , Transposição dos Grandes Vasos , Ultrassonografia Pré-Natal , Humanos , Feminino , Gravidez , Adulto , Dupla Via de Saída do Ventrículo Direito/diagnóstico por imagem , Dupla Via de Saída do Ventrículo Direito/embriologia , Transposição dos Grandes Vasos/diagnóstico por imagem , Síndrome de DiGeorge/genética , DNA/análise , CariótipoRESUMO
BACKGROUND: Arterial switch operation (ASO) is the standard surgical choice for D-transposition of great arteries (D-TGA). However, the implications of ASO on pulmonaries, coronaries, and aorta have not been adequately investigated. The current study evaluates arterial morphologic changes post-ASO at intermediate-term surveillance. METHODS: From May 2021 to May 2022, patients with D-TGA who underwent ASO for more than six months were recruited. Preoperative and operative data were collected. Patients were assessed using echocardiography (ECHO) and multislice CT angiography (MSCT) to evaluate pulmonary, coronary, and aortic arterial anatomy. RESULTS: Twenty patients were included with median age of 11 (10-23.25) days at ASO and 14 (7.25-32.75) months on last follow-up. Neo-aortic regurgitation was detected in 12(60%) and neo-pulmonary regurgitation in 3 (15%). Using ECHO, complete evaluation of pulmonary arteries (PAs) was not achieved in 35% and incomplete coronaries assessment in 40% of cases. No stenosis was detected in coronaries using MSCT, although coronary anomalies were found in 9/20 (45%). Dilated Aortic annulus was detected in 16/20 (80%), dilated aortic root in 18/20 (90%), and dilated sinotubular junction in 70%. Right PA stenosis was diagnosed in 10/20 (50%) and left PA(LPA) stenosis in 7/20 (35%). Although Z-score of PAs did not correlate with aortic data, LPA bending angle was positively correlated to neo-aortic root diameter and Z-score (rho = 0.65,p = 0.016; rho = 0.69,p = 0.01), respectively. CONCLUSION: Echocardiography alone is not a conclusive surveillance tool for detecting late post-ASO anatomic changes in D-TGA patients. Cardiac MSCT should be considered for comprehensive evaluation on the intermediate-term follow-up post-ASO to accurately track morphologic abnormalities in the aorta, pulmonary, and coronary arteries.
Assuntos
Transposição das Grandes Artérias , Ecocardiografia , Tomografia Computadorizada Multidetectores , Artéria Pulmonar , Transposição dos Grandes Vasos , Humanos , Masculino , Feminino , Transposição dos Grandes Vasos/diagnóstico por imagem , Transposição dos Grandes Vasos/cirurgia , Lactente , Recém-Nascido , Transposição das Grandes Artérias/efeitos adversos , Artéria Pulmonar/diagnóstico por imagem , Pré-Escolar , Vasos Coronários/diagnóstico por imagem , Aorta/diagnóstico por imagem , Estudos Retrospectivos , Angiografia por Tomografia Computadorizada , SeguimentosRESUMO
The great artery relationship in transposition of great arteries (TGA) is based on the degree of resorption and growth of subsemilunar conus. In transposition of great arteries, the spatial relationship of the great arteries can be variable. Commonly, the aorta will be related right and anterior to pulmonary artery (D-TGA). Less commonly, the aorta can be related left and anterior (SDL-TGA), side-by-side, and antero-posterior to pulmonary artery. Four fetuses with concordant atrioventricular and discordant ventriculoarterial connection with various great arteries relationship were presented in this imaging essay.
Assuntos
Transposição dos Grandes Vasos , Ultrassonografia Pré-Natal , Humanos , Transposição dos Grandes Vasos/diagnóstico por imagem , Transposição dos Grandes Vasos/embriologia , Ultrassonografia Pré-Natal/métodos , Feminino , Gravidez , Coração Fetal/diagnóstico por imagem , Ecocardiografia Quadridimensional/métodosRESUMO
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.
Assuntos
Coração Fetal , Valor Preditivo dos Testes , Transposição dos Grandes Vasos , Ultrassonografia Pré-Natal , Transposição dos Grandes Vasos/diagnóstico por imagem , Transposição dos Grandes Vasos/cirurgia , Transposição dos Grandes Vasos/fisiopatologia , Humanos , Gravidez , Coração Fetal/diagnóstico por imagem , Coração Fetal/fisiopatologia , Feminino , Imageamento Tridimensional , Modelos Cardiovasculares , Idade Gestacional , Modelagem Computacional Específica para o Paciente , Prognóstico , Interpretação de Imagem Assistida por ComputadorRESUMO
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.
Assuntos
Coração Fetal , Idade Gestacional , Valor Preditivo dos Testes , Ultrassonografia Pré-Natal , Função Ventricular Esquerda , Função Ventricular Direita , Humanos , Estudos Retrospectivos , Feminino , Ultrassonografia Pré-Natal/métodos , Gravidez , Reprodutibilidade dos Testes , Coração Fetal/diagnóstico por imagem , Coração Fetal/fisiopatologia , Transposição dos Grandes Vasos/diagnóstico por imagem , Transposição dos Grandes Vasos/fisiopatologia , Fenômenos Biomecânicos , Contração Miocárdica , Interpretação de Imagem Assistida por Computador , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/fisiopatologia , Defeitos dos Septos Cardíacos/diagnóstico por imagem , Defeitos dos Septos Cardíacos/fisiopatologia , Variações Dependentes do ObservadorRESUMO
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.
Assuntos
Transposição das Grandes Artérias , Cateterismo Cardíaco , Artéria Pulmonar , Estenose de Artéria Pulmonar , Transposição dos Grandes Vasos , Função Ventricular Direita , Adolescente , Criança , Feminino , Humanos , Masculino , Transposição das Grandes Artérias/efeitos adversos , Complacência (Medida de Distensibilidade) , Contração Miocárdica , Artéria Pulmonar/fisiopatologia , Artéria Pulmonar/diagnóstico por imagem , Estudos Retrospectivos , Estenose de Artéria Pulmonar/fisiopatologia , Estenose de Artéria Pulmonar/diagnóstico por imagem , Estenose de Artéria Pulmonar/etiologia , Volume Sistólico , Transposição dos Grandes Vasos/fisiopatologia , Transposição dos Grandes Vasos/cirurgia , Transposição dos Grandes Vasos/complicações , Transposição dos Grandes Vasos/diagnóstico por imagem , Resultado do Tratamento , Rigidez Vascular , Disfunção Ventricular Direita/fisiopatologia , Disfunção Ventricular Direita/etiologia , Disfunção Ventricular Direita/diagnóstico por imagem , Pressão VentricularRESUMO
Congenitally corrected transposition of the great arteries (ccTGA) is a rare malformation with diverse morphology. We assessed features of fetuses with ccTGA and evaluated neonatal and pediatric outcomes. This was a retrospective review of fetuses with ccTGA at Birmingham Women's and Children's Hospital born from 2005 to 2019. Of thirty-six fetuses identified, six had unavailable prenatal data, one was postnatally diagnosed with isomerism and 29 fetuses were evaluated. ccTGA without associated cardiac lesions was found in 28% (8/29), ccTGA with significant VSD in 31% (9/29), ccTGA with pulmonary obstruction in 24% (7/29) and ccTGA with complex anomalies in 17% (5/29). Tricuspid regurgitation (TR) was observed in 17% (5/29) and heart block (HB) in 10% (3/29) prenatally. Six, that is 21% underwent genetic testing of which one was abnormal. Five extra-cardiac anomalies were reported prenatally and postnatally. Pregnancy was discontinued in five, of which two had moderate TR. There were thirty-one liveborn. Coarctation of the aorta was found in five postnatally but not suspected prenatally. In one, pulmonary stenosis was underestimated; otherwise, prenatal morphology was confirmed. Cardiac interventions were performed in 77% (24/31) liveborn with 39% (12/31) undergoing neonatal intervention. Overall, 6/31 liveborn died including all three with prenatal heart block and one with TR. Estimated survival for all liveborn at 1, 5 and 10 years was 87% (95% CI 76-100%), 83% (95% CI 72-98%) and 80% (95% CI 66-96%) respectively. Accurate prenatal diagnosis of ccTGA is critical for counseling. Early outcomes are favorable with 77% of liveborn undergoing surgery. Fetuses with prenatal diagnosis of complex associated abnormalities, HB and TR appear to do less well.
Assuntos
Transposição das Grandes Artérias Corrigida Congenitamente , Ultrassonografia Pré-Natal , Humanos , Feminino , Estudos Retrospectivos , Gravidez , Recém-Nascido , Transposição dos Grandes Vasos/diagnóstico , Transposição dos Grandes Vasos/diagnóstico por imagem , Ecocardiografia , Diagnóstico Pré-Natal/métodos , MasculinoRESUMO
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.
Assuntos
Transposição das Grandes Artérias , Transposição dos Grandes Vasos , Remodelação Ventricular , Humanos , Transposição dos Grandes Vasos/cirurgia , Transposição dos Grandes Vasos/fisiopatologia , Transposição dos Grandes Vasos/diagnóstico por imagem , Masculino , Feminino , Adulto , Remodelação Ventricular/fisiologia , Adulto Jovem , Estudos Prospectivos , Adolescente , Pressão Arterial/fisiologia , Aorta/diagnóstico por imagem , Aorta/fisiopatologia , Análise de Onda de Pulso , Imagem Cinética por Ressonância Magnética/métodos , Fluxo Pulsátil/fisiologiaRESUMO
AIM: To examine whether antenatal diagnosis modifies relationships between neonatal brain volumes and 18-month neurodevelopmental outcomes in children with transposition of the great arteries (TGA). METHOD: In a retrospective cohort of 139 children with TGA (77 antenatally diagnosed), we obtained total brain volumes (TBVs) on pre- (n = 102) and postoperative (n = 112) magnetic resonance imaging. Eighteen-month neurodevelopmental outcomes were assessed using the Bayley Scales of Infant and Toddler Development, Third Edition. Generalized estimating equations with interaction terms were used to determine whether antenatal diagnosis modified associations between TBVs and neurodevelopmental outcomes accounting for postmenstrual age at scan, brain injury, and ventricular septal defect. RESULTS: Infants with postnatal diagnosis had more preoperative hypotension (35% vs 14%, p = 0.004). The interactions between antenatal diagnosis and TBVs were significantly related to cognitive (p = 0.003) outcomes. Specifically, smaller TBVs were associated with lower cognitive scores in infants diagnosed postnatally; this association was attenuated in those diagnosed antenatally. INTERPRETATION: Antenatal diagnosis modifies associations between neonatal brain volume and 18-month cognitive outcome in infants with TGA. These findings suggest that antenatal diagnosis may be neuroprotective, possibly through improved preoperative clinical status. These data highlight the need to improve antenatal diagnosis rates. WHAT THIS PAPER ADDS: Antenatal diagnosis of transposition of the great arteries modified relationships between neonatal brain volume and neurodevelopment. Smaller brain volumes related to poorer cognitive scores with postnatal diagnosis only. There was more preoperative hypotension in the postnatal diagnosis group.
Assuntos
Encéfalo , Imageamento por Ressonância Magnética , Diagnóstico Pré-Natal , Transposição dos Grandes Vasos , Humanos , Transposição dos Grandes Vasos/diagnóstico por imagem , Feminino , Estudos Retrospectivos , Encéfalo/diagnóstico por imagem , Encéfalo/crescimento & desenvolvimento , Encéfalo/patologia , Masculino , Lactente , Recém-Nascido , Transtornos do Neurodesenvolvimento/diagnóstico por imagem , Transtornos do Neurodesenvolvimento/etiologia , Transtornos do Neurodesenvolvimento/diagnóstico , Desenvolvimento Infantil/fisiologia , GravidezRESUMO
A comprehensive and structured imaging approach in the evaluation of the systemic right ventricle (sRV) in patients with complete transposition of the great arteries (TGA) after atrial switch procedure and congenitally corrected transposition of the great arteries (ccTGA) is a key for their optimal lifelong surveillance. Despite the improvements in cardiovascular imaging of adults with congenital heart disease (ACHD), the imaging of sRV remains an ongoing challenge due to its complex morphology and the difficulty in applying the existing knowledge for the systemic left ventricle. While cardiac magnetic resonance (CMR) is considered the gold standard imaging method, echocardiographic evaluation is primarily preferred in everyday clinical setting. Although qualitative assessment of its systolic function is primarily used, the introduction of advanced echocardiographic techniques, such as speckle tracking echocardiography (STE) and three-dimensional echocardiography (3DE), has provided new insights into the optimal assessment of the sRV. Standardized quantitative parameters remain to be elucidated, and morphometric and mechanistic studies are warranted to validate reference ranges for the sRV. This review highlights the challenges in the optimal evaluation of sRV and summarizes the available imaging tools. HIGHLIGHTS: CMR is the gold standard imaging method of sRV. Qualitative assessment of the systolic function of sRV is primarily used. Advanced echocardiographic techniques (STE and 3DE) provide optimal sRV assessment. Reference ranges for the sRV indices are warranted to be validated.
Assuntos
Cardiopatias Congênitas , Transposição dos Grandes Vasos , Adulto , Humanos , Transposição dos Grandes Vasos/diagnóstico por imagem , Transposição dos Grandes Vasos/cirurgia , Ventrículos do Coração/diagnóstico por imagem , Cardiopatias Congênitas/diagnóstico por imagem , Transposição das Grandes Artérias Corrigida Congenitamente , Imagem MultimodalRESUMO
Our study aimed to elucidate the role of different shunts and provide novel insights into optimal treatment approaches for complete transposition of the great arteries (TGA), which is characterized by unique and complicated circulatory dynamics. We constructed a computational cardiovascular TGA model and manipulated cardiovascular parameters, such as atrial septal defect (ASD) and patent ductus arteriosus (PDA) sizes, to quantify their effects on oxygenation and hemodynamics. In addition, ASD flow patterns were investigated as innovative indications for balloon atrial septostomy (BAS). Our model of TGA with an intact ventricular septum (TGA-IVS) showed that a large ASD can achieve sufficient mixing for survival without PDA, and the presence of PDA is detrimental to oxygen delivery. A treatment strategy for TGA-IVS that enlarges the ASD as much as possible by BAS and PDA closure would be desirable. In TGA with a ventricular septal defect (TGA-VSD), the VSD allows for higher oxygenation and reduces the detrimental effects of PDA on systemic circulation. In TGA-VSD, both strategies of enlarging the ASD by BAS with a closed PDA and adjusting the PDA in response to pulmonary vascular resistance (PVR) reduction without BAS may be effective. The simulated ASD flow patterns showed that the sharp peak left-to-right flow pattern in systole (σ-wave) reflected the hemodynamically significant ASD size, independent of PDA, VSD, and PVR. The ASD flow pattern visualized by Doppler echocardiography provides clinical insights into the significance of an ASD and indications for BAS, which are not readily apparent through morphological assessment.NEW & NOTEWORTHY Complete transposition of the great arteries (TGA) represents complex and unique circulation that is dependent on blood mixing through multiple interacting shunts. Consequently, the role of each shunt and the treatment strategy remain unclear. We developed a mathematical model of TGA circulation, revealing the significant influence of atrial septal defect (ASD) on oxygenation and hemodynamics. The blood flow pattern through the ASD reflects its hemodynamic impact and helps determine treatment strategies.
Assuntos
Comunicação Interatrial , Comunicação Interventricular , Transposição dos Grandes Vasos , Humanos , Transposição dos Grandes Vasos/diagnóstico por imagem , Transposição dos Grandes Vasos/cirurgia , Hemodinâmica , ArtériasRESUMO
BACKGROUND: No study has focused on left atrial (LA) function assessed by echocardiography in adult patients with simple D-TGA after arterial switch operation (ASO). We aimed to describe LA strain parameters in these patients. METHODS: A prospective cohort study including 42 adult patients with simple D-TGA after ASO and 33 aged-matched controls. Phasic LA and LV global longitudinal strain (GLS) were obtained by transthoracic 2D-speckle tracking echocardiography (STE). Volumetric and functional analysis of LA and LV were also evaluated by 2D and 3D analysis. A multivariable model was performed to investigate the variables that best differentiate patients with D-TGA from healthy controls. RESULTS: LA strain parameters in D-TGA patients were within the normal range described for healthy subjects. However, the three LA strain parameters (Reservoir, Conduit, and Contraction) were lower in patients (LASr: 31.13 ± 7.67 vs. 49.71 ± 8.38; LAS cd: -22.91 ± 5.69 vs. -34.55 ± 6.54; LASct: -8.14 ± 4.93 vs. -15.15 ± 6.07, p < .001 for all three comparisons). LA volumes were similar between patients and controls. LV-GLS remained significantly lower in the D-TGA group than in controls (-17.29 ± 2.68 vs. -21.98 ± 1.84, p < .001). D-TGA patients had evidence of worse LV ejection fraction measured by the Teichholz method (63.38 ± 8.23 vs. 69.28 ± 5.92, p = .001) and 3D analysis (57.97% ± 4.16 vs. 60.67 ± 3.39, p = .011) and diastolic dysfunction as compared to healthy controls. LV-GLS and conduit LAS were the variables best differentiating patients with D-TGA from healthy controls. CONCLUSIONS: LA strain is impaired in young adults with simple D-TGA late after the ASO, probably in agreement with some degree of LV dysfunction previously described.
Assuntos
Transposição das Grandes Artérias , Transposição dos Grandes Vasos , Disfunção Ventricular Esquerda , Adulto Jovem , Humanos , Idoso , Transposição dos Grandes Vasos/diagnóstico por imagem , Transposição dos Grandes Vasos/cirurgia , Estudos Prospectivos , Átrios do Coração/diagnóstico por imagem , Artérias , Função Ventricular EsquerdaRESUMO
OBJECTIVE: Restrictive foramen ovale (FO) in dextro-transposition of the great arteries (d-TGA) with intact ventricular septum may lead to severe life-threatening hypoxia within the first hours of life, making urgent balloon atrial septostomy (BAS) inevitable. Reliable prenatal prediction of restrictive FO is crucial in these cases. However, current prenatal echocardiographic markers show low predictive value, and prenatal prediction often fails with fatal consequences for a subset of newborns. In this study, we described our experience and aimed to identify reliable predictive markers for BAS. METHODS: We included 45 fetuses with isolated d-TGA that were diagnosed and delivered between 2010 and 2022 in two large German tertiary referral centers. Inclusion criteria were the availability of former prenatal ultrasound reports, of stored echocardiographic videos and still images, which had to be obtained within the last 14 days prior to delivery and that were of sufficient quality for retrospective re-analysis. Cardiac parameters were retrospectively assessed and their predictive value was evaluated. RESULTS: Among the 45 included fetuses with d-TGA, 22 neonates had restrictive FO postnatally and required urgent BAS within the first 24 h of life. In contrast, 23 neonates had normal FO anatomy, but 4 of them unexpectedly showed inadequate interatrial mixing despite their normal FO anatomy, rapidly developed hypoxia and also required urgent BAS ('bad mixer'). Overall, 26 (58%) neonates required urgent BAS, whereas 19 (42%) achieved good O2 saturation and did not undergo urgent BAS. In the former prenatal ultrasound reports, restrictive FO with subsequent urgent BAS was correctly predicted in 11 of 22 cases (50% sensitivity), whereas a normal FO anatomy was correctly predicted in 19 of 23 cases (83% specificity). After current re-analysis of the stored videos and images, we identified three highly significant markers for restrictive FO: a FO diameter < 7 mm (p < 0.01), a fixed (p = 0.035) and a hypermobile (p = 0.014) FO flap. The maximum systolic flow velocities in the pulmonary veins were also significantly increased in restrictive FO (p = 0.021), but no cut-off value to reliably predict restrictive FO could be identified. If the above markers are applied, all 22 cases with restrictive FO and all 23 cases with normal FO anatomy could correctly be predicted (100% positive predictive value). Correct prediction of urgent BAS also succeeded in all 22 cases with restrictive FO (100% PPV), but naturally failed in 4 of the 23 cases with correctly predicted normal FO ('bad mixer') (82.6% negative predictive value). CONCLUSION: Precise assessment of FO size and FO flap motility allows a reliable prenatal prediction of both restrictive and normal FO anatomy postnatally. Prediction of likelihood of urgent BAS also succeeds reliably in all fetuses with restrictive FO, but identification of the small subset of fetuses that also requires urgent BAS despite their normal FO anatomy fails, because the ability of sufficient postnatal interatrial mixing cannot be predicted prenatally. Therefore, all fetuses with prenatally diagnosed d-TGA should always be delivered in a tertiary center with cardiac catheter stand-by, allowing BAS within the first 24 h after birth, regardless of their predicted FO anatomy.
Assuntos
Forame Oval , Transposição dos Grandes Vasos , Gravidez , Feminino , Recém-Nascido , Humanos , Forame Oval/diagnóstico por imagem , Forame Oval/cirurgia , Transposição dos Grandes Vasos/diagnóstico por imagem , Transposição dos Grandes Vasos/cirurgia , Estudos Retrospectivos , Ultrassonografia Pré-Natal/métodos , Feto , Artérias , HipóxiaRESUMO
BACKGROUND: Neo-aortic root dilatation can lead to significant late morbidity after the arterial switch operation (ASO) for dextro-transposition of the great arteries (d-TGA). OBJECTIVES: We sought to examine the growth of the neo-aortic root in d-TGA. METHODS: A single-center, retrospective cohort study of patients who underwent the ASO between July 1, 1981 and September 30, 2022 was performed. Morphology was categorized as dextro-transposition of the great arteries with intact ventricular septum (d-TGA-IVS), dextro-transposition of the great arteries with ventricular septal defect (d-TGA-VSD), and double-outlet right ventricle-transposition of the great arteries type (DORV-TGA). Echocardiographically determined diameters and derived z scores were measured at the annulus, sinus of Valsalva, and sinotubular junction immediately before the ASO and throughout follow-up. Trends in root dimensions over time were assessed using linear mixed-effects models. The association between intrinsic morphology and the composite of moderate-severe aortic regurgitation (AR) and neo-aortic valve or root intervention was evaluated with univariable and multivariable Cox proportional hazards models. RESULTS: Of 1,359 patients who underwent the ASO, 593 (44%), 666 (49%), and 100 (7%) patients had d-TGA-IVS, d-TGA-VSD, and DORV-TGA, respectively. Each patient underwent a median of 5 echocardiograms (Q1-Q3: 3-10 echocardiograms) over a median follow-up of 8.6 years (range: 0.1-39.3 years). At 30 years, patients with DORV-TGA demonstrated greater annular (P < 0.001), sinus of Valsalva (P = 0.039), and sinotubular junction (P = 0.041) dilatation relative to patients with d-TGA-IVS. On multivariable analysis, intrinsic anatomy, older age at ASO, at least mild AR at baseline, and high-risk root dilatation were associated with moderate-severe AR and neo-aortic valve or root intervention at late follow-up (all P < 0.05). CONCLUSIONS: Longitudinal surveillance of the neo-aortic root is warranted long after the ASO.
Assuntos
Insuficiência da Valva Aórtica , Comunicação Interventricular , Transposição dos Grandes Vasos , Humanos , Lactente , Transposição dos Grandes Vasos/diagnóstico por imagem , Transposição dos Grandes Vasos/cirurgia , Estudos Retrospectivos , Aorta Torácica , Seguimentos , Prognóstico , Insuficiência da Valva Aórtica/diagnóstico por imagem , Insuficiência da Valva Aórtica/cirurgia , Insuficiência da Valva Aórtica/epidemiologia , Dilatação Patológica , Resultado do TratamentoAssuntos
Aneurisma da Aorta Torácica , Aneurisma da Raiz da Aorta , Síndrome de Marfan , Transposição dos Grandes Vasos , Humanos , Síndrome de Marfan/complicações , Transposição dos Grandes Vasos/complicações , Transposição dos Grandes Vasos/diagnóstico por imagem , Transposição dos Grandes Vasos/cirurgia , ArtériasAssuntos
Doenças das Valvas Cardíacas , Transposição dos Grandes Vasos , Humanos , Transposição das Grandes Artérias Corrigida Congenitamente , Valva Mitral/diagnóstico por imagem , Valva Mitral/cirurgia , Transposição dos Grandes Vasos/complicações , Transposição dos Grandes Vasos/diagnóstico por imagem , Transposição dos Grandes Vasos/cirurgiaRESUMO
BACKGROUND: Balloon atrial septostomy (BAS) is an emergent and essential cardiac intervention to enhance intercirculatory mixing at atrial level in deoxygenated patients diagnosed with transposition of the great arteries (TGA) and restrictive foramen ovale. The recent recall of several BAS catheters and the changes in the European legal framework for medical devices (MDR 2017/745), has led to an overall scarcity of BAS catheters and raised questions about the use, safety, and experience of the remaining NuMED Z-5 BAS catheter. AIMS: To evaluate and describe the practice and safety of the Z-5 BAS catheter, and to compare it to the performance of other BAS catheters. METHODS: A retrospective single-center cohort encompassing all BAS procedures performed with the Z-5 BAS catheter in TGA patients between 1999 and 2022. RESULTS: A total of 182 BAS procedures were performed in 179 TGA-newborns at Day 1 (IQR 0-5) days after birth, with median weight of 3.4 (IQR 1.2-5.7) kg. The need for BAS was urgent in 90% of patients. The percentage of BAS procedures performed at bedside increased over time from 9.8% (before 2010) to 67% (2017-2022). Major complication rate was 2.2%, consisting of cerebral infarction (1.6%) and hypovolemic shock (0.5%). The rate of minor complications was 9.3%, including temporary periprocedural AV-block (3.8%), femoral vein thrombosis (2.7%), transient intracardiac thrombus (0.5%), and atrial flutter (2.2%). BAS procedures performed at bedside and in the cardiac catheterization laboratory had similar complication rates. CONCLUSIONS: BAS using the Z-5 BAS catheter is both feasible and safe at bedside and at the cardiac catheterization laboratory with minimal major complications.