RESUMO
Tetralogy of Fallot (TOF) is the most common cyanotic heart defect. TOF consists of the combination of four anomalies (Fig. 35.1): (1) a large malalignment ventricular septal defect, (2) an obstruction of the right ventricular outflow tract (usually infundibular and valvular pulmonary stenosis with a small pulmonary valve annulus and supravalvular stenosis, (3) an aorta that "overrides" the ventricular septal defect, and (4) right ventricular hypertrophy. TOF represents 4-8% of congenital heart defects. Specific variations of TOF include all forms of pulmonary atresia with VSD and absent pulmonary valve syndrome. In addition, the left and right main pulmonary arteries may be stenotic or hypoplastic. In these cases, there may be major aortopulmonary collateral arteries (MAPCAs) which are vessels arising from the aorta or the subclavian arteries that supply segments of the pulmonary arterial tree. Additional variations include an ASD (Pentalogy of Fallot), a right aortic arch, and coronary abnormalities.
Assuntos
Dupla Via de Saída do Ventrículo Direito , Tetralogia de Fallot , Tetralogia de Fallot/diagnóstico por imagem , Humanos , Dupla Via de Saída do Ventrículo Direito/diagnóstico por imagem , Dupla Via de Saída do Ventrículo Direito/cirurgiaRESUMO
PURPOSE OF REVIEW: In the ever-evolving field of medical imaging, this review highlights significant advancements in preoperative and postoperative imaging for Tetralogy of Fallot (TOF) and double outlet right ventricle (DORV) over the past 18âmonths. RECENT FINDINGS: This review showcases innovations in echocardiography such as 3D speckle tracking echocardiography (3DSTE) for assessing right ventricle-pulmonary artery coupling (RVPAC) and Doppler velocity reconstruction (DoVeR) for intracardiac flow fields evaluation. Furthermore, advances in assessment of cardiovascular anatomy using computed tomography (CT) improve the integration of imaging in ablation procedures. Additionally, the inclusion of cardiac magnetic resonance (CMR) parameters as risk score predictors for morbidity, and mortality and for timing of pulmonary valve replacement (PVR) indicates its significance in clinical management. The utilization of 4D flow techniques for postoperative hemodynamic assessment promises new insights into pressure mapping. Lastly, emerging technologies such as 3D printing and 3D virtual reality are expected to improve image quality and surgical confidence in preoperative planning. SUMMARY: Developments in multimodality imaging in TOF and DORV are poised to shape the future of clinical practice in this field.
Assuntos
Dupla Via de Saída do Ventrículo Direito , Imagem Multimodal , Tetralogia de Fallot , Tetralogia de Fallot/cirurgia , Tetralogia de Fallot/diagnóstico por imagem , Humanos , Imagem Multimodal/métodos , Dupla Via de Saída do Ventrículo Direito/diagnóstico por imagem , Dupla Via de Saída do Ventrículo Direito/cirurgia , Tomografia Computadorizada por Raios X/métodosRESUMO
En bloc rotation of the outflow tracts or double root translocation offers an anatomic repair of transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction and closely related forms of double outlet right ventricle. The technical principle is to excise aortic and pulmonary root en bloc, rotate them as a whole by 180°, and reimplant them. The left ventricular outflow tract is enlarged with the patch closing the ventricular septal defect. In our experience, two thirds of the pulmonary valves could be preserved. Growth of the aortic and pulmonary root could be demonstrated in several studies performed by our group. It is still a complex and technically demanding procedure with long cardiopulmonary bypass periods and cross-clamp times. However, perioperative mortality and complications do not differ significantly from other forms of reconstruction. The reoperation rate is significantly lower. Presently, the best time to perform this operation seems to be after the newborn period within the first year of life.
Assuntos
Dupla Via de Saída do Ventrículo Direito , Comunicação Interventricular , Transposição dos Grandes Vasos , Recém-Nascido , Humanos , Lactente , Transposição dos Grandes Vasos/cirurgia , Transposição dos Grandes Vasos/complicações , Resultado do Tratamento , Rotação , Dupla Via de Saída do Ventrículo Direito/complicações , Dupla Via de Saída do Ventrículo Direito/cirurgia , Comunicação Interventricular/cirurgiaRESUMO
PURPOSE: Our aim in this study was to investigate the prenatal and postnatal prognosis of double outlet right ventricle (DORV) cases diagnosed prenatally by analyzing the outcomes based on the subtype. METHODS: This study is a retrospective chart review. Cases diagnosed with fetal DORV by prenatal ultrasound in the maternal-fetal medicine department of our hospital between 2014 and 2022 were included. Data on maternal characteristics, fetal echocardiographic features (type of DORV), pregnancy and neonatal outcomes (termination of pregnancy [TOP], intrauterine fetal death [IUD], neonatal death [NND], death in infancy (IND), survival) were collected and analyzed. RESULTS: Ninety-nine cases of prenatally diagnosed cases of DORV were included. The prenatal diagnosis was right in 97% of the liveborn fetuses. The cases were classified into subtypes, including transposition of great arteries (TGA), Fallot, ventricular septal defect (VSD), remote, and heterotaxy types. The cohort consisted of 32.3% TGA type, 19.1% fallot type, 11.1% VSD type, 2% remote type, and 35.3% heterotaxy type of DORV. An additional cardiac anomaly was observed in 87% and an extra-cardiac anomaly was observed in 54% of the cases. When we excluded the cases with heterotaxy type but without any chromosomal abnormality, additional genetic abnormalities were detected in 42% of the remaining cases. Outcome of pregnancy was livebirth in 68/99 (68.7%), IUFD in 5/99 (5.1%), and TOP in 26/99 (26.3%). Postnatal cardiac surgical repair was performed in 48 cases. Survival among livebirths was 39/68 (57.3%). Twenty-nine neonates or infants who had additional cardiac anomalies and/or genetic abnormalities died before any surgical intervention. The postoperative survival rate was 39/48 (81.2%). CONCLUSION: The prognosis in DORV depends on the anatomical subtype, the presence, and severity of associated anomalies. Survival increases in isolated cases without any additional structural or genetic anomalies.
Assuntos
Dupla Via de Saída do Ventrículo Direito , Cardiopatias Congênitas , Comunicação Interventricular , Transposição dos Grandes Vasos , Gravidez , Lactente , Recém-Nascido , Feminino , Humanos , Dupla Via de Saída do Ventrículo Direito/diagnóstico por imagem , Dupla Via de Saída do Ventrículo Direito/cirurgia , Estudos Retrospectivos , Cardiopatias Congênitas/diagnóstico por imagem , PrognósticoRESUMO
PURPOSE OF REVIEW: There has been much variability in the definition of double outlet right ventricle (DORV) spanning the last century. Historically, emphasis has been placed on the assignment of the great arteries to the right ventricle as a definition of DORV. In this review, we aim to underscore the importance of conal muscle, rather than rules surrounding assignment of great arteries to ventricles. We will be outlining the variability in patient anatomy that results from variations in conal muscle development in DORV, which may not fit perfectly into predefined constructs. This anatomic variability directly determines physiology and surgical repair options. RECENT FINDINGS: There is a growing appreciation of the utility of cross-sectional imaging in complex DORV, and the generation of patient-specific 3D models with virtual reality simulations for surgical planning. These models improve the prediction of candidacy for biventricular repair and allow the mapping of complex baffle pathways preoperatively. SUMMARY: DORV is not a disease entity in itself, but rather a vast spectrum of disorders associated with maldevelopment of conal muscle and often abnormal expansion of one the great vessels. Patient-specific 3D models will be crucial for improved surgical planning and patient outcomes.
Assuntos
Dupla Via de Saída do Ventrículo Direito , Humanos , Dupla Via de Saída do Ventrículo Direito/cirurgia , Ventrículos do Coração/anormalidades , Imageamento Tridimensional , Procedimentos Cirúrgicos Cardíacos/métodosRESUMO
BACKGROUND: Neo-aortic root dilatation and valve regurgitation are emerging problems late after arterial switch operation (ASO). We sought to evaluate the prevalence and outcomes of neo-aortic root or valve reoperation after ASO. METHODS: All patients with biventricular circulation who underwent an ASO between 1983 and 2015 were included at a single institution. RESULTS: In our cohort of 782 late ASO survivors, the median duration of follow-up was 18.1 years (interquartile range [IQR], 11.3-25.6 years). During follow-up, 47 patients (6.0%) underwent 60 reoperations on the neo-aortic valve/root. The first neo-aortic valve/root reoperation occurred at a median of 15.2 years (IQR, 7.8-18.4 years) after ASO. Operations included mechanical Bentall (31.9%; n = 15), aortic valve repair (25.5%; n = 12), mechanical aortic valve replacement (AVR) (21.3%; n = 10), valve-sparing root replacement (19.1%; n = 9), and the Ross procedure (2.1%; n = 1). There was 1 late death (2.1%). Multivariable predictors of neo-aortic valve/root reoperation were bicuspid valve (hazard ratio [HR], 4.8; 95% confidence interval [CI], 2.1-10.7; P < .001), Taussig-Bing anomaly (HR, 3.0; 95% CI, 1.2-7.4; P < .02), previous pulmonary artery band (HR, 2.8; 95% CI, 1.2-6.3; P < .01) and left ventricular outflow tract obstruction before ASO (HR, 2.4; 95% CI, 1.0-5.8; P < .04). Freedom from neo-aortic valve or root reoperation was 98.0% (95% CI, 96.7%-98.8%) at 10 years, 93.3% (95% CI, 90.8%-95.2%) at 20 years, and 88.5% (95% CI, 84.1%-91.8%) at 30 years after ASO. Among the 47 patients who underwent neo-aortic reoperation, freedom from AVR was 82.3% (95% CI, 67.7%-90.7%) at 10 years, 58.0% (95% CI, 41.8%-71.2%) at 20 years, and 43.2% (95% CI, 27.0%-58.3%) at 25 years after ASO. CONCLUSIONS: The need for neo-aortic valve or root reoperation surpasses 10% by 30 years post-ASO. Evolving understanding of the mechanisms of neo-aortic valve insufficiency and techniques of neo-aortic valve repair may decrease the need for AVR.
Assuntos
Insuficiência da Valva Aórtica , Transposição das Grandes Artérias , Dupla Via de Saída do Ventrículo Direito , Transposição dos Grandes Vasos , Humanos , Transposição das Grandes Artérias/efeitos adversos , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/cirurgia , Transposição dos Grandes Vasos/cirurgia , Estudos Retrospectivos , Dupla Via de Saída do Ventrículo Direito/cirurgia , Insuficiência da Valva Aórtica/diagnóstico por imagem , Insuficiência da Valva Aórtica/cirurgia , Insuficiência da Valva Aórtica/epidemiologia , Reoperação , Seguimentos , Resultado do TratamentoRESUMO
This study aimed to evaluate the anatomical reproducibility of a preoperative intracardiac 3D image (IC image) created using computed tomography, and to investigate its usefulness as a surgical decision-making tool. Between 2012 and 2022, ventricular septal defect (VSD) patients, and double outlet right ventricle (DORV) or transposition of the great arteries (TGA) with pulmonary stenosis (PS) patients who underwent cardiac surgery and had preoperative computed tomography were enrolled. SYNAPSE VINCENT® (Fujifilm) was used to create an IC image which was analyzed retrospectively. In 14 VSD patients, the diagnostic consistency rate in the Soto classification with intraoperative findings was 100% (14/14) for IC image versus 64% (9/14) for transthoracic echocardiography (P = 0.04). The defect size showed a higher correlation coefficient with IC image (0.837, P = 0.001) than with transthoracic echocardiography (0.567, P = 0.034). In 11 DORV/TGA with PS patients, the diagnostic consistency rate in the Lev classification was 100% (9/9) for IC image versus 77% (7/9) for transthoracic echocardiography (P = 0.47). The secondary interventricular foramen (SVF)/left ventricular outflow tract (LVOT) ratio by IC image was significantly smaller in the biventricular-repair group (median 0.71, IQR 0.67-1.06) than in the univentricular-repair group (median 1.79, IQR 1.53-2.42) (P = 0.006). An IC image is useful as a surgical decision-making tool for simple VSDs and complex congenital heart diseases such as DORV or TGA with pulmonary stenosis. The SVF/LVOT ratio determined from the IC image may be a useful indicator for avoiding LVOT obstruction.
Assuntos
Dupla Via de Saída do Ventrículo Direito , Cardiopatias Congênitas , Comunicação Interventricular , Estenose da Valva Pulmonar , Transposição dos Grandes Vasos , Humanos , Lactente , Transposição dos Grandes Vasos/cirurgia , Estudos Retrospectivos , Imageamento Tridimensional , Reprodutibilidade dos Testes , Resultado do Tratamento , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/cirurgia , Dupla Via de Saída do Ventrículo Direito/cirurgia , Comunicação Interventricular/diagnóstico por imagem , Comunicação Interventricular/cirurgia , Estenose da Valva Pulmonar/diagnóstico por imagem , Estenose da Valva Pulmonar/cirurgiaRESUMO
BACKGROUND: Published data on the outcomes of the arterial switch operation in the developing world are limited. We sought to identify the midterm outcomes of and risk factors for mortality and morbidity in patients undergoing the arterial switch operation at a high-volume center in Vietnam. METHODS: A retrospective review was conducted of 608 patients who underwent an arterial switch operation for the treatment of transposition of the great arteries (TGA; n = 509) or double-outlet right ventricle TGA (DORV-TGA; n = 99) at a single center from 2010 to 2021. RESULTS: Overall early mortality was 5.9%, and late mortality was 1%. Multivariate Cox regression showed weight at operation of <3 kg (P < .001), prolonged aortic cross-clamp time (P < .001), and need for secondary aortic cross-clamp (P = .011) as risk factors for overall mortality. The actuarial 10-year survival rate was 93%. The overall cardiac reoperation rate was significantly higher in patients with DORV-TGA (hazard ratio [HR], 16.43; 95% CI, 4.70-57.43; P < .001). The rate of freedom from cardiac reoperation at 10 years was 94%. Risk factors for outflow tract reoperation were patients with DORV-TGA (HR, 23.28; 95% CI, 2.86-189.66; P = .003), single coronary artery (HR, 4.1; 95% CI, 1.16-14.46; P = .028), and mild aortic valve regurgitation observed in 3.7% of patients. CONCLUSIONS: The outcomes of the arterial switch operation in a low- and middle-income country were satisfactory. Nevertheless, the complexity of the procedure and lower weight of patients are still challenges. Patients with DORV-TGA and single coronary artery need to be closely followed up.
Assuntos
Transposição das Grandes Artérias , Dupla Via de Saída do Ventrículo Direito , Transposição dos Grandes Vasos , Humanos , Lactente , Transposição das Grandes Artérias/métodos , Transposição dos Grandes Vasos/cirurgia , Dupla Via de Saída do Ventrículo Direito/cirurgia , Reoperação , Fatores de Risco , Estudos Retrospectivos , Resultado do Tratamento , SeguimentosRESUMO
OBJECTIVES: The goal of this retrospective multicentre study was to present late surgical outcomes of the treatment of children with double outlet right ventricle (DORV) coming from emerging countries. METHODS: The Mécénat Chirurgie Cardiaque brings to France for surgery selected children with simple and complex congenital diseases, including DORV. The patients are operated on in 9 hospitals that specialize in paediatric cardiac surgery. Data are collected from the Mécénat Chirurgie Cardiaque comprehensive database, with a strict postoperative follow-up. The patients included only those who had biventricular repair of DORV with 2 viable ventricles. According to the classification of the Eleventh Revision of the International Classification of Diseases, DORV was defined as a congenital cardiovascular malformation in which both great arteries arise entirely or predominantly from the morphologically right ventricle. RESULTS: From January 1996 to January 2022, a total of 81 consecutive DORV biventricular repair operations were performed. There were 6 subtypes of DORV divided into 2 groups: DORV-committed ventricular septal defect (VSD): DORV-VSD (n = 25), DORV-Fallot (n = 34), DORV-transposition of the great arteries (n = 5); and DORV-non-committed (nc) VSD: DORV-ncVSD-no pulmonary stenosis (PS) (n = 7), DORV-ncVSD-PS (n = 5) and DORV-atrioventricular septal defect (AVSD)-PS (n = 5). Four Fontan patients were excluded. Three patients were lost to follow-up (3.4%). The overall perioperative mortality was 7.4% ± 2.6%, 6/81 (95% confidence interval: 2.8%-15.4%) ranging from 0% in DORV-AVSD-PS to 14% for DORV-ncVSD-no PS. The overall 10-year survival was 86%. The early mortality of DORV-ncVSD at 5.9% ± 2.4% (1/17) was similar to that of DORV-committed VSD at 7.8% ± 2.7% (5/64) (P = 0.79). There was a trend towards an optimal outcome for the arterial switch operation and the DORV-AVSD-PS repair. VSD enlargement was significantly more frequent in DORV-ncVSD at 42% (5/12) (P = 0.001). There were low numbers in the complex groups. The number of Fontan cases was noticeably low. The aorta located entirely on the right ventricle represents the fundamental anomaly and the surgical challenge of DORV. CONCLUSIONS: Overall survival at 10 years was 86%. This study shows a trend towards satisfactory early and late outcomes in BVR of simple DORV with committed VSD, compared to complex DORV with ncVSD.
Assuntos
Procedimentos Cirúrgicos Cardíacos , Dupla Via de Saída do Ventrículo Direito , Defeitos dos Septos Cardíacos , Estenose da Valva Pulmonar , Transposição dos Grandes Vasos , Criança , Humanos , Lactente , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Dupla Via de Saída do Ventrículo Direito/cirurgia , Transposição dos Grandes Vasos/cirurgia , Resultado do Tratamento , Estudos RetrospectivosRESUMO
OBJECTIVES: The purpose of this study is to describe the long-term results of the 'réparation à l'étage ventriculaire' (REV) technique for double-outlet right ventricle and transposition of the great arteries (TGA) with pulmonary stenosis (PS). METHODS: Between 1980 and 2021, 157 patients underwent a REV procedure (median age and weight: 20.8 months and 7.7 kg). The most frequent anatomical presentation was the association between TGA, ventricular septal defect and PS (n = 116, 73.9%). RESULTS: Sixty-seven patients (42.7%) underwent a Rashkind procedure, and 67 patients (42.7%) a prior surgical palliation (including 62 systemic-to-pulmonary artery shunts). Resection of the conal septum and/or ventricular septal defect enlargement was performed in 109 patients (69.4%). Thirteen patients (8.3%) died, including 4 during the first postoperative month and 2 after heart transplant. Overall survival at 40 years was 89.3%. Thirty-seven patients (23.6%) required 68 reinterventions on the right ventricular outflow tract (RVOT), including 49 reoperations, with a median delay of 9 years after the REV (8 months to 27 years). Twenty patients (12.7%) underwent RVOT valvulation (16 surgical and 4 interventional). Freedom from RVOT reintervention and reoperation at 40 years were 60.3% and 62.6%, respectively. Four patients (2.5%) required reoperation for left ventricular outflow tract obstruction, with a median delay of 4.8 years. CONCLUSIONS: The REV procedure is a good alternative for TGA and double-outlet right ventricle with PS patients. Only a quarter of the patients required redo surgery on the RVOT. Reoperations for left ventricular outflow tract obstruction are scarce.
Assuntos
Procedimentos Cirúrgicos Cardíacos , Dupla Via de Saída do Ventrículo Direito , Comunicação Interventricular , Estenose da Valva Pulmonar , Transposição dos Grandes Vasos , Obstrução da Via de Saída Ventricular Esquerda , Humanos , Lactente , Transposição dos Grandes Vasos/cirurgia , Dupla Via de Saída do Ventrículo Direito/cirurgia , Procedimentos Cirúrgicos Cardíacos/métodos , Resultado do Tratamento , Estenose da Valva Pulmonar/cirurgia , Comunicação Interventricular/cirurgia , ArtériasRESUMO
Although complex congenital heart disease (CHD) patients usually present in childhood, it is not rare to see adults well past middle age. These patients undergo continuous pathophysiological changes in their heart and blood vessels, making anesthetic management more challenging if surgery is required. Herein, we report a case in which understanding the anatomy and pathophysiology helped optimally manage a patient with a double outlet right ventricle (DORV) who underwent plating and fixation for a hangman's fracture in the prone position.
Assuntos
Dupla Via de Saída do Ventrículo Direito , Cardiopatias Congênitas , Adulto , Pessoa de Meia-Idade , Humanos , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/cirurgia , Coração , Dupla Via de Saída do Ventrículo Direito/complicações , Dupla Via de Saída do Ventrículo Direito/diagnóstico por imagem , Dupla Via de Saída do Ventrículo Direito/cirurgia , Cianose , Vértebras Cervicais/cirurgiaRESUMO
This is a case of a 2.7-year-old girl with trisomy 21 and double outlet right ventricle who underwent epicardial pacemaker system placement for a surgical atrioventricular block and achieved atrioventricular conduction recovery immediately after residual ventricular septal defect closure. Although ventricular pacing ratio was 100% before re-operation, it declined to approximately 25% on the 6th post-operative day and was <1% 3 years after re-operation.
Assuntos
Bloqueio Atrioventricular , Dupla Via de Saída do Ventrículo Direito , Comunicação Interventricular , Feminino , Humanos , Pré-Escolar , Comunicação Interventricular/cirurgia , Dupla Via de Saída do Ventrículo Direito/cirurgia , Bloqueio Atrioventricular/terapia , Bloqueio Atrioventricular/cirurgia , Ventrículos do Coração , ReoperaçãoRESUMO
Criss-cross heart is an extreamly rare anomaly characterized by abnormal rotation of the heart on its long axis. Almost always there are associated cardiac anomalies such as pulmonary stenosis, ventricular septal defect (VSD) and ventriculoarterial connection discord, and most cases are candidates for Fontan procedure due to hypoplasia of right ventricle or straddling atrioventricular valve. We report a case of arterial switch operation for criss-cross heart with muscular ventricular septal defect. The patient was diagnosed with criss-cross heart, double outlet right ventricle, subpulmonary VSD, muscular VSD and patent ductus arteriosus (PDA). PDA ligation and pulmonary artery banding (PAB) was performed in the neonatal period, and an arterial switch operation (ASO) was planed at 6 months of age. Preoperative angiography showed nearly normal right ventricular volume and echocardiography showed normal subvalvular structures of atrioventricular valves. ASO, intraventricular rerouting and muscular VSD closure by sandwitch technique were successfully performed.
Assuntos
Transposição das Grandes Artérias , Coração Entrecruzado , Dupla Via de Saída do Ventrículo Direito , Permeabilidade do Canal Arterial , Comunicação Interventricular , Recém-Nascido , Humanos , Comunicação Interventricular/complicações , Comunicação Interventricular/diagnóstico por imagem , Comunicação Interventricular/cirurgia , Coração , Dupla Via de Saída do Ventrículo Direito/complicações , Dupla Via de Saída do Ventrículo Direito/diagnóstico por imagem , Dupla Via de Saída do Ventrículo Direito/cirurgiaRESUMO
OBJECTIVES: Taussig-Bing anomaly (TBA) and transposition of the great arteries (TGA) with hypoplastic or interrupted aortic arch (AA) are rare anomalies. Various operative techniques and a high incidence of reinterventions are described. The aim of this retrospective single-centre study was to evaluate operative data, mortality and reintervention rate with special regard to the AA. METHODS: At the Children's Heart Center Linz, 50 patients with the above-mentioned diagnosis have been corrected by a simultaneous repair between 2001 and 2022. Thirty-seven children had TBA, 13 had TGA and 5 of them had an interrupted AA. The median age at operation was 7 [interquartile range (IQR) 5-9] days, weight 3.38 (IQR 2.9-3.8) kg and follow-up 9.3 (IQR 3.1-14.5) years. The AA reconstruction was performed without patch material in 49 cases. RESULTS: There was 1 in-hospital mortality in a TBA patient and 1 late mortality (7 years later, neuroblastoma). 14/49 patients needed at least 1 reoperation (28.6%, all TBA) and 3 further patients had catheter reintervention or radiofrequency ablation only (6.1%, 2 TBA). Seventy-five percent of these procedures affected the right heart/pulmonary arteries; there was 1 re-coarctation repair. CONCLUSIONS: The simultaneous correction of TBA and TGA with AA obstruction or interruption is a safe operation with very low mortality. The AA reconstruction with minimized use of patch material resulted in a low restenosis rate.
Assuntos
Coartação Aórtica , Transposição das Grandes Artérias , Dupla Via de Saída do Ventrículo Direito , Transposição dos Grandes Vasos , Criança , Humanos , Lactente , Recém-Nascido , Transposição das Grandes Artérias/efeitos adversos , Aorta Torácica/cirurgia , Seguimentos , Estudos Retrospectivos , Resultado do Tratamento , Dupla Via de Saída do Ventrículo Direito/diagnóstico , Dupla Via de Saída do Ventrículo Direito/cirurgia , Coartação Aórtica/cirurgia , ReoperaçãoRESUMO
BACKGROUND: This study investigated the outcomes and factors associated with reintervention or development of significant pulmonary stenosis (PS) after repair of transposition of the great arteries (TGA) or Taussig-Bing anomaly (TBA) with aortic arch obstruction. METHODS: A total of 51 patients with TGA or TBA who underwent an arterial switch operation and aortic arch reconstruction between 2004 and 2020 were included. The outcomes of interest were all-cause death, including heart transplantation, all-cause reintervention, right-sided reintervention, and development of significant PS. RESULTS: The median age and body weight at repair were 9 days and 3.2 kg, respectively. Forty-nine patients (96.1%) underwent 1-stage repair. A total of 28 patients (54.9%) had TBA, and 8 patients (15.7%) had interrupted aortic arch. There were 5 early deaths (9.8%) and 2 late deaths during a median follow-up duration of 59 months. The transplant-free survival rate 10 years after repair was 82.6%. A total of 21 reinterventions were required in 10 patients. The significant PS-free survival rate 10 years after repair was 68.8%. In univariable analysis, a higher ratio of the diameter of the main pulmonary artery to the ascending aorta was associated with all-cause reintervention (P = .007) and right-sided reintervention (P = .002). A smaller aortic annulus z-score at the pulmonary position was associated with the development of significant PS (P = .049). CONCLUSIONS: The rates of overall mortality and reintervention after repair were not negligible. A higher degree of size discrepancy between the 2 great arteries was associated with all-cause or right-sided reintervention. A smaller aortic annulus z-score at the pulmonary position was associated with the development of significant PS.
Assuntos
Transposição das Grandes Artérias , Dupla Via de Saída do Ventrículo Direito , Estenose da Valva Pulmonar , Transposição dos Grandes Vasos , Humanos , Lactente , Transposição dos Grandes Vasos/cirurgia , Aorta Torácica/cirurgia , Seguimentos , Resultado do Tratamento , Estudos Retrospectivos , Dupla Via de Saída do Ventrículo Direito/cirurgia , Estenose da Valva Pulmonar/cirurgia , ReoperaçãoRESUMO
OBJECTIVES: The study objectives were to report on a growing experience of conduction system mapping during complex congenital heart surgery and create a predictive model of conduction anatomy. METHODS: Patients undergoing complex cardiac repair with conduction mapping were studied. Intraoperative mapping used a multielectrode catheter to collect His bundle electrograms in the open, decompressed, beating heart. Patient anatomy, operative details, His bundle location, and postoperative conduction status were analyzed. By using classification and regression tree analysis, a predictive model of conduction location was created. RESULTS: A total of 109 patients underwent mapping. Median age and weight were 1.8 years (range, 0.2-14.9) and 10.8 kg (range, 3.5-50.4), respectively. Conduction was identified in 96% (105/109). Median mapping time was 6 minutes (range, 2-33). Anatomy included atrioventricular canal defect, double outlet right ventricle, complex transposition of the great arteries, and multiple ventricular septal defects. By classification and regression tree analysis, ventricular looping and visceroatrial situs were the greatest discriminators of conduction location. A total of 94 of 105 patients (89.5%) were free of complete heart block. Only 1 patient (2.9%) with heterotaxy syndrome developed complete heart block. CONCLUSIONS: The precise anatomic location of the conduction system in patients with complex congenital heart defects can be difficult for the surgeon to accurately predict. Intraoperative conduction mapping enables localization of the His bundle and adds to our understanding of the anatomic factors associated with conduction location. Predictive modeling of conduction anatomy may build on what is already known about the conduction system and help surgeons to better anticipate conduction location preoperatively and intraoperatively.
Assuntos
Dupla Via de Saída do Ventrículo Direito , Cardiopatias Congênitas , Síndrome de Heterotaxia , Transposição dos Grandes Vasos , Humanos , Transposição dos Grandes Vasos/cirurgia , Dupla Via de Saída do Ventrículo Direito/cirurgia , Coração , Cardiopatias Congênitas/cirurgia , Bloqueio CardíacoRESUMO
Hearts with double outlet ventricles and concordant atrioventricular connections account for about 1%-3% of all cases of congenital heart disease. We review hearts with two ventricles and concordant atrioventricular connections with double outlet right ventricle (DORV), double outlet left ventricle (DOLV) and double outlet both ventricles (DOBV) from the morphological and clinical imaging perspectives. These hearts are a heterogeneous group of congenital cardiac malformations with a wide range of pathophysiologies that require an individualised surgical approach based on a precise understanding of the complex cardiovascular anatomy. Owing to their differing temporal, spatial and contrast resolutions, we propose that multimodality imaging provides optimal characterisation of various intracardiac morphological features of double outlet hearts. This approach aids clinical diagnosis for optimising treatment options across these malformations.
Assuntos
Dupla Via de Saída do Ventrículo Direito , Cardiopatias Congênitas , Humanos , Dupla Via de Saída do Ventrículo Direito/diagnóstico , Dupla Via de Saída do Ventrículo Direito/cirurgia , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/cirurgia , Ventrículos do Coração , Ecocardiografia , Imagem MultimodalRESUMO
OBJECTIVES: The purpose of this study was to report outcomes of double outlet right ventricle biventricular repair at a single institution in developing countries and to investigate post-operatively determined risk factors for mortality and late intervention. METHODS: Patients with double outlet right ventricle who underwent biventricular repair in our centre from January 2009 to December 2021 were included. RESULTS: A total of 96 patients (male/female = 61/35) with biventricular repair were included. According to its specific anatomical type, the appropriate individual surgical plan was selected. Multivariate analysis indicated that prolonged cardiopulmonary bypass time(OR = 1.044; p = 0.012) and pre-operative moderate or above pulmonary hypertension(OR = 24.558; p = 0.023) were independent risk factors for early mortality. Univariate analysis showed that different anatomical types and different surgical methods had similar late intervention and late mortality. Concomitant coarctation of the aorta(OR = 40; p = 0.020) and concomitant ventricular septal defect enlargement(OR = 26.667; p = 0.005) were independent risk factors for late intervention by multivariate analysis. CONCLUSION: Selection of appropriate surgical techniques based on different anatomical types often results in similar late outcomes. For patients with concomitant ventricular septal defect enlargement during the operation, it is necessary to fully expand and avoid damage to the conduction bundle. We should timely intervention in patients with coarctation of the aorta and pay attention to the occurrence of left ventricular outflow tract obstruction during follow-up.
Assuntos
Coartação Aórtica , Procedimentos Cirúrgicos Cardíacos , Dupla Via de Saída do Ventrículo Direito , Comunicação Interventricular , Humanos , Masculino , Feminino , Lactente , Dupla Via de Saída do Ventrículo Direito/cirurgia , Coartação Aórtica/complicações , Procedimentos Cirúrgicos Cardíacos/métodos , Comunicação Interventricular/cirurgia , Aorta/cirurgia , Resultado do TratamentoRESUMO
We report an unusual case of a 2-month-old baby with a diagnosis of common arterial trunk and double outlet right ventricle with a remote type ventricular septal defect. Taking into consideration the physiologic moment and anatomic findings of the patient, we planned and successfully performed a bidirectional Glenn procedure as its first palliative procedure.
Assuntos
Dupla Via de Saída do Ventrículo Direito , Técnica de Fontan , Cardiopatias Congênitas , Persistência do Tronco Arterial , Coração Univentricular , Lactente , Humanos , Técnica de Fontan/métodos , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/cirurgia , Dupla Via de Saída do Ventrículo Direito/diagnóstico por imagem , Dupla Via de Saída do Ventrículo Direito/cirurgia , Cuidados Paliativos/métodos , Ventrículos do Coração/cirurgia , Resultado do TratamentoRESUMO
OBJECTIVE: To report the long-term outcomes of the arterial switch operation for transposition of the great arteries. METHODS: The data of 35 patients who underwent an arterial switch operation between April 2006 and May 2021 were retrospectively examined; five had double-outlet right ventricles, three had side-by-side transposition, and 27 had d-transposition. The punch-out technique was the basic method for coronary transplantation; however, if coronary artery kinking after additional peeling was a concern, the trap-door technique was used. In aortic reconstruction, the caliber difference between the neoascending aorta and the native pulmonary artery was fixed using the pulmonary artery flap. In pulmonary reconstruction, the pulmonary artery was bilaterally exposed over the second branch. RESULTS: The median age at operation and follow-up duration were 10.0 (8.0-12.5) days and 72.9 (40.7-139.5) months, respectively. Thirty-four patients survived, and one was lost to follow-up ([coronary transplantation]: right coronary artery, punch-out = 31, trap-door = 2; left coronary artery, punch-out = 28, trap-door = 7; [aortic regurgitation]: no-slight = 27, mild = 6, moderate = 1; [pulmonary regurgitation]: no-slight = 22, mild = 11, moderate = 1). Slight coronary bending occurred in two patients; ischemia was not observed. Catheter stent placement was performed in one of three patients with stenosis at the pulmonary artery bifurcation. Catheter balloon dilation was performed in one patient with supravalvular pulmonary stenosis. There were no cases of coronary, aortic, or pulmonary artery reoperations. The 15-year cumulative reintervention avoidance rate was 91.0%. CONCLUSIONS: There were no deaths or reoperations during the long-term follow-up period, and arterial switch operation outcomes at our hospital were satisfactory.