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1.
Zhonghua Fu Chan Ke Za Zhi ; 55(12): 830-836, 2020 Dec 25.
Artigo em Chinês | MEDLINE | ID: mdl-33355757

RESUMO

Objective: To investigate the prenatal diagnosis, integrated management and prognosis of fetal complete transposition of the great arteries (D-TGA) detected by ultrasonography. Methods: The prenatal diagnosis, integrated management and prognosis of 19 D-TGA fetuses found by ultrasound during pregnancy in Peking University People's Hospital from January 2014 to June 2019 were analyzed retrospectively. Results: The incidence of D-TGA was 0.12% (19/16 028) among fetuses diagnosed by ultrasound during 5 years. Among the 19 cases, there were 7 cases (7/19) of D-TGA alone, 7 cases (7/19) of D-TGA combined with ventricular septal defect (VSD), 5 cases (5/19) of D-TGA combined with other cardiac malformations; 2 cases (2/19) of D-TGA combined with extra cardiac malformations, and 1 case (1/19) of fetal growth restriction. Nuchal translucency (NT) thickening was found in 3 cases (3/19) at the first trimester of pregnancy. Among the 19 D-TGA fetuses found by ultrasound examination, 18 (18/19) had chromosome karyotype analysis of fetuses or newborns, and chromosomal abnormalities were found in 2 cases, all of which were terminated in the second trimester of pregnancy. The integrated management and multidisciplinary diagnosis and treatment of D-TGA fetuses during pregnancy and perinatal period were carried out. Nine cases (9/19) had induction in the second trimester of pregnancy, 10 cases (10/19) were delivered at term, and the gestational week of delivery was (38.3±0.7) weeks, among which 6 cases (6/10) were delivered by caesarean section due to obstetric factors, and 4 cases (4/10) were delivered by vaginal birth. The oxygen saturation was (69.2±11.3)% at birth and (77.8±6.7)% when transferred to the department of pediatrics. Except for one case lost to follow-up, the other 9 newborns received operation. The average operation time was (21.8±22.1) days after birth, 8 cases (8/9) completed one operation and 1 case (1/9) performed two operations. All of the 9 cases treated by surgery were followed up well. Conclusions: Prenatal diagnosis, individualized evaluation and integrated management during pregnancy and perinatal period should be carried out for the patients with fetal D-TGA detected by ultrasound. Fetal D-TGA is not an indication of cesarean section. The open of ductus arteriosus can be maintained with drugs when necessary after birth, and a good prognosis could be obtained through surgery.


Assuntos
Estudos Retrospectivos , Transposição dos Grandes Vasos , Ultrassonografia Pré-Natal/métodos , Artérias , Cesárea , Criança , Feminino , Feto , Humanos , Recém-Nascido , Assistência Perinatal , Gravidez , Resultado da Gravidez , Prognóstico , Transposição dos Grandes Vasos/diagnóstico por imagem , Transposição dos Grandes Vasos/cirurgia
2.
J Card Surg ; 35(11): 2927-2933, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-33111442

RESUMO

OBJECTIVE: The arterial switch operation (ASO) is the standard treatment for the transposition of the great arteries. The timely variation in the residual pressure gradient across the pulmonary arteries is ill-defined. This study is aimed to study the progressive changes in the pressure gradient across the pulmonary valve and pulmonary arteries after ASO. METHODS: All eligible patients for this study who underwent ASO between 2000 and 2019 were reviewed. Transthoracic echocardiography was used to estimate the peak pressure gradient across the pulmonary artery and its branches. The primary outcome was the total peak pressure gradient (TPG) which is the sum of peak pressure gradients across the main pulmonary artery and pulmonary artery branches. Furthermore, longitudinal data analyses with mixed-effect modeling were used to determine the independent predictors for the changes in the pressure gradient. RESULTS: Three hundred and nine patients were included in the study. Over a 17-year follow-up, the freedom from pulmonary stenosis reintervention was 95% (16 out of the 309 patients underwent reintervention = 5%). The longitudinal data analyses of serial 1844 echocardiographic studies for the included patients revealed that the TPG recorded in the first postoperative echocardiogram across pulmonary valve, right and left pulmonary artery branches was the most significant predictor for reintervention. CONCLUSION: The total peak gradient measured in the first postoperative echocardiogram is the most important predictor for reintervention. We propose that a total peak gradient in the first postoperative echocardiography of 55 mm Hg or more is a predictor for reintervention.


Assuntos
Pressão Arterial , Transposição das Grandes Artérias/métodos , Artéria Pulmonar/fisiopatologia , Valva Pulmonar/fisiopatologia , Transposição dos Grandes Vasos/fisiopatologia , Transposição dos Grandes Vasos/cirurgia , Adolescente , Adulto , Criança , Pré-Escolar , Ecocardiografia , Feminino , Seguimentos , Humanos , Recém-Nascido , Estudos Longitudinais , Masculino , Complicações Pós-Operatórias/diagnóstico por imagem , Complicações Pós-Operatórias/fisiopatologia , Valor Preditivo dos Testes , Artéria Pulmonar/diagnóstico por imagem , Valva Pulmonar/diagnóstico por imagem , Estenose da Valva Pulmonar/diagnóstico por imagem , Estenose da Valva Pulmonar/fisiopatologia , Reoperação , Transposição dos Grandes Vasos/diagnóstico por imagem , Resultado do Tratamento , Adulto Jovem
3.
Georgian Med News ; (304-305): 85-90, 2020.
Artigo em Russo | MEDLINE | ID: mdl-32965255

RESUMO

Aortic arch hypoplasia is a congenital anomaly of the development of the aortic arch, characterized by hemodynamically significant narrowing of one or more segments of the aortic arch. The combination of simple transposition of the great arteries (TGA) and obstruction at the level of the aortic arch is not very common. However, when transposition is combined with the VSD, Taussig-Bing anomaly, this combination is more common. The degree of obstruction at the level of the aortic arch may vary from discrete coarctation of the aorta, tubular hypoplasia of the aortic arch, to interruption. Despite the improvement in the results of surgical treatment of this pathology in recent years, the question of the stage of treatment remains debatable.; The objective of the study was to analyze immediate and long-term results of the correction of transposition of the great arteries and aortic arch hypoplasia.; From 2010 to 2019 at the "Amosov National Amosov Institute of Cardiovascular Surgery of the National Academy of Medical Sciences of Ukraine" and "Scientific Center For Pediatric Cardiology and Cardiac Surgery of the Ministry of Health of Ukraine" 76 infants underwent repair of TGA and aortic arch hypoplasia. The study included only patients with two-ventrical physiology and subsequent two-ventrical repair. There were 49 (64%) male patients and 27 (36%) female patients. The mean age of patients was 1.1 ± 0.5 months, mean body weight was 3.7 ± 1.4 kg. The average body surface area was 0.23 ± 0.05 m². Patients were divided into two groups: group I involved 52 (68%) individuals - patients who underwent one-stage repair of TGA and aortic arch hypoplasia, group II included 24 (32%) patients with two-stage repair, consisting of aortic arch reconstruction and pulmonary banding at the first stage and correction of TGA at the second stage. Antegrade selective cerebral perfusion was performed in 24 (46%) patients during reconstruction of the aortic arch.; The in-hospital mortality rate was 5.3% (n= 4). In group I, in-hospital mortality was 3.8% (n=2), and was significantly lower compared to the group II - 8.3% (n=2), p<0.05. Eight patients (14.5%) had delayed sternal closure in the early postoperative period. The average long-term follow-up was 3.7±2.8 years (from one months to 9.1 years). One late death occurred in group II. Aortic arch restenosis developed in 11 (14.4%) patients: 7 (13.4%) patients in group I and 4 (16.6%) patients in group II. Right ventricle outflow tract and pulmonary artery obstruction occurred in 9 (11.8%) patients: in 2 (3.8%) patients from group I and in 7 (29.1%) patients from group II. In the long term follow-up there were no neurological complications and compression of the trachea.; Surgical treatment of TGA with aortic arch hypoplasia is effective in infants with good immediate and long-term results. The study indicates that one-stage repair has better immediate and long-term results, compared to two-stage treatment and may be the method of choice in the treatment of this complex pathology.


Assuntos
Coartação Aórtica/cirurgia , Transposição dos Grandes Vasos/cirurgia , Aorta Torácica/diagnóstico por imagem , Aorta Torácica/cirurgia , Criança , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Masculino , Resultado do Tratamento , Ucrânia
4.
J Cardiothorac Surg ; 15(1): 136, 2020 Jun 11.
Artigo em Inglês | MEDLINE | ID: mdl-32527284

RESUMO

Transposition of the great arteries (TGA) and interruption of the aortic arch (IAA) are uncommon congenital heart diseases. The association between TGA and IAA is rare. The aim of this study is to present a case with combined TGA and IAA, who underwent the primary repair and review the literature with similar cases. The one-month-old patient was admitted with tachypnea and cyanosis. Delayed diagnosis was caused due to the absence of prenatal examination. Echocardiography and computed tomography angiography confirmed TGA with anterior-posterior-oriented great arteries, wide patent ductus arteriosus, type B IAA, ventricular septal defect (VSD) and pulmonary arterial hypertension. The patient underwent a single-stage primary surgical repair process leading to VSD closure, reconstruction of the aortic arch and arterial switch operation in October 2019. The patient is doing well at a 3-month follow-up post-surgery. The echocardiogram suggests a normal systolic function of the ventricles and trivial regurgitation for both aortic and pulmonary valves. CONCLUSIONS: The single-stage repair with VSD closure, reconstruction of aortic arch and arterial switch operation might be an applicable approach for most of the patients with combined TGA and IAA. Long term follow-up is required as a high re-intervention rate for recurrent coarctation, supravalvular aortic stenosis, neoaortic valve regurgitation, obstruction of the right heart system and coronary stenosis has been reported.


Assuntos
Aorta Torácica/anormalidades , Aorta Torácica/cirurgia , Permeabilidade do Canal Arterial/cirurgia , Comunicação Interventricular/cirurgia , Transposição dos Grandes Vasos/cirurgia , Transposição das Grandes Artérias , Humanos , Lactente , Masculino , Hipertensão Arterial Pulmonar/complicações , Valva Pulmonar
5.
Asian Cardiovasc Thorac Ann ; 28(6): 333-335, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32551840

RESUMO

The introduction of the arterial switch operation has improved the surgical outcome of transposition of the great arteries. However, coronary anomalies such as intramural coronary arteries, single coronary artery, or coronary arteries originating from a single arterial sinus have been reported as independent risk factors for early mortality and late morbidity after an arterial switch operation. We performed an arterial switch operation using a unique technique for translocation of the coronary arteries originating from a single left-side arterial sinus, to prevent coronary artery distortion and subsequent coronary malperfusion.


Assuntos
Transposição das Grandes Artérias , Seio Coronário/cirurgia , Anomalias dos Vasos Coronários/cirurgia , Transposição dos Grandes Vasos/cirurgia , Seio Coronário/anormalidades , Seio Coronário/diagnóstico por imagem , Anomalias dos Vasos Coronários/complicações , Anomalias dos Vasos Coronários/diagnóstico por imagem , Comunicação Interventricular/complicações , Comunicação Interventricular/diagnóstico por imagem , Comunicação Interventricular/cirurgia , Humanos , Lactente , Transposição dos Grandes Vasos/complicações , Transposição dos Grandes Vasos/diagnóstico por imagem , Resultado do Tratamento
6.
Am J Cardiol ; 129: 87-94, 2020 08 15.
Artigo em Inglês | MEDLINE | ID: mdl-32593432

RESUMO

Several studies have reported aortic dilation and increased stiffness of the ascending aorta in patients after repair of congenital heart disease (CHD), which may be a predominant cardiovascular risk. However, the clinical significance has not been described in detail. In this retrospective study, 175 repaired patients with complex CHD achieving biventricular circulation and age-matched 39 control subjects were reviewed (median age: 14.9 and 15.7 years, respectively). We measured the diameters of the ascending aorta and descending aorta from catheterization angiograms to yield Z-scores and stiffness indexes (ß) using diameter fluctuations corresponding to pulsatile pressures. Clinical profile, peak oxygen uptake during the cardiopulmonary exercise test, and incidence of unscheduled hospitalization during follow-up was also reviewed. Compared with controls, patients with complex CHD, except for those with aortic coarctation, exhibited significant dilation and increased stiffness of the aortic root and ascending aorta, but not of the descending aorta. In this CHD population (n = 147, including 112 conotruncal anomalies), exercise capacities correlated independently with the diameter Z-score and stiffness index of the ascending aorta along with the history of repetitive thoracotomies, reduced forced vital capacity, and right ventricular hypertension. During a follow-up period (median 15.6 years), either dilation (Z-score >3.5) or increased stiffness (ß >6.0) of the ascending aorta stratified morbidity, but no synergistic impact was detected. In conclusion, in repaired patients with complex CHD, a stiffened and dilated ascending aorta was frequently found, exerting significant adverse impacts on diminished exercise capacity and morbidity.


Assuntos
Aorta/fisiopatologia , Doenças da Aorta/fisiopatologia , Tolerância ao Exercício/fisiologia , Cardiopatias Congênitas/fisiopatologia , Rigidez Vascular/fisiologia , Adolescente , Coartação Aórtica/fisiopatologia , Coartação Aórtica/cirurgia , Insuficiência da Valva Aórtica/fisiopatologia , Estenose da Valva Aórtica/fisiopatologia , Transposição das Grandes Artérias , Procedimentos Cirúrgicos Cardíacos , Estudos de Casos e Controles , Criança , Dilatação Patológica/fisiopatologia , Dupla Via de Saída do Ventrículo Direito/fisiopatologia , Dupla Via de Saída do Ventrículo Direito/cirurgia , Feminino , Cardiopatias Congênitas/cirurgia , Humanos , Masculino , Estudos Retrospectivos , Tetralogia de Fallot/fisiopatologia , Tetralogia de Fallot/cirurgia , Transposição dos Grandes Vasos/fisiopatologia , Transposição dos Grandes Vasos/cirurgia , Persistência do Tronco Arterial/fisiopatologia , Persistência do Tronco Arterial/cirurgia
7.
J Card Surg ; 35(7): 1664-1668, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32383229

RESUMO

INTRODUCTION: In adult congenital patients with transposition of the great arteries originally treated with the Mustard (atrial switch) procedure, the most common reason for re-intervention is baffle stenosis. This may be exacerbated by permanent transvenous pacemaker lead placement across the baffle. CASE REPORT: A 47-year-old female status post Mustard procedure performed at 15 months old presented with a high-grade stenosis of the superior vena cava (SVC) baffle from the SVC to the left atrium, with a nonfunctional permanent pacemaker lead passing through the baffle. A mechanical rotating dilator sheath was used for attempted lead extraction, relieving the baffle stenosis almost completely as a secondary effect, before the placement of a 10 × 27 mm Visipro balloon-expandable stent in the SVC baffle. CONCLUSIONS: Use of the mechanical rotating dilator sheath is an evolving treatment strategy in adult congenital heart disease to minimize the risk of bleeding, trauma to surrounding structures, and death. Its ability to fully alleviate baffle stenosis even when full lead extraction is not feasible or is associated with significant procedural risk, further demonstrates its expanded role in this patient population. A multidisciplinary approach and great diligence must be employed to avoid potential complications.


Assuntos
Transposição das Grandes Artérias/métodos , Remoção de Dispositivo/instrumentação , Marca-Passo Artificial/efeitos adversos , Complicações Pós-Operatórias/cirurgia , Transposição dos Grandes Vasos/cirurgia , Veia Cava Superior/patologia , Veia Cava Superior/cirurgia , Transposição das Grandes Artérias/efeitos adversos , Constrição Patológica/etiologia , Constrição Patológica/cirurgia , Remoção de Dispositivo/métodos , Feminino , Humanos , Pessoa de Meia-Idade , Complicações Pós-Operatórias/etiologia , Stents Metálicos Autoexpansíveis , Resultado do Tratamento
8.
Int Heart J ; 61(3): 616-619, 2020 May 30.
Artigo em Inglês | MEDLINE | ID: mdl-32418970

RESUMO

We experienced a 33-year-old patient with D-looped transposition of the great arteries (D-TGA) and a history of Senning operation who was referred to our institute with cardiogenic shock and subsequently underwent urgent paracorporeal ventricular assist device (VAD) implantation, which was a first in Japan, that was eventually converted to a durable VAD. Central venous pressure was maintained relatively high to obtain VAD filling and recover end-organ dysfunction, given the migration of the inflow cannula due to rich trabeculae carneae of the anatomical right ventricle (systemic ventricle in this case).


Assuntos
Coração Auxiliar , Implantação de Prótese , Choque Cardiogênico/cirurgia , Transposição dos Grandes Vasos , Adulto , Transposição das Grandes Artérias , Feminino , Humanos , Transposição dos Grandes Vasos/cirurgia
10.
Ann Thorac Surg ; 110(4): e327-e329, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32224240

RESUMO

Conjoined twins are rare congenital malformations known to be associated with cardiac abnormalities. Management of transposition of the great arteries with an intact ventricular septum in this subgroup of patients is challenging, especially in the presence of multiple comorbidities. Prevention of left ventricular involution until the patient is stable for an arterial switch operation is a real challenge. We report a case of early ductal stenting to keep the left ventricle well trained in a separated conjoined twin who eventually underwent an arterial switch operation.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Doenças em Gêmeos , Stents , Transposição dos Grandes Vasos/cirurgia , Gêmeos Unidos , Função Ventricular Esquerda/fisiologia , Septo Interventricular/cirurgia , Pré-Escolar , Humanos , Imageamento Tridimensional , Tomografia Computadorizada por Raios X , Transposição dos Grandes Vasos/diagnóstico , Septo Interventricular/diagnóstico por imagem
11.
Ann Thorac Surg ; 110(4): e279-e280, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32311344

RESUMO

Systemic right ventricle failure often develops in patients with dextro-transposition of the great arteries who have undergone atrial-level switch procedures. An adult with dextro-transposition of the great arteries with Mustard repair in childhood presented with end-stage heart failure due to systemic right ventricle failure, Mustard baffle leak, and unrepaired partial anomalous pulmonary venous return. She underwent device closure of the baffle leak by catheterization, followed by baffle revision and concomitant systemic ventricular assist device placement by adult and pediatric heart surgeons. This case demonstrates the importance of multidisciplinary collaboration between pediatric and adult heart failure specialists in caring for complex adult congenital heart patients.


Assuntos
Transposição das Grandes Artérias , Insuficiência Cardíaca/cirurgia , Coração Auxiliar , Complicações Pós-Operatórias/cirurgia , Transposição dos Grandes Vasos/cirurgia , Adulto , Feminino , Humanos , Reoperação
12.
J Thorac Cardiovasc Surg ; 160(1): 191-199.e1, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32222408

RESUMO

OBJECTIVE: We sought to determine the influence of coronary artery anatomy on mortality in more than 1000 children undergoing the arterial switch operation. METHODS: All patients who underwent an arterial switch operation were identified from 2 hospital databases and reviewed retrospectively. Coronary anatomy was recorded from operative reports using the Leiden classification. RESULTS: An arterial switch operation was performed in 1033 children between 1983 and 2013. Coronary anatomy was normal in 697 patients (67%). The most common type of anomalous coronary anatomy was the circumflex coronary artery arising from sinus 2 (in 152 patients [15%]). Forty-seven patients (4.5%) had all coronary arteries arising from a single sinus. Of these 47 patients, 34 patients (3.3%) had a true single coronary artery. Fifty-two patients (5.0%) had an intramural coronary artery. Overall early mortality was 3.3% (34 out of 1033 patients) over the 30-year period. Early mortality was 3.0% (21 out of 697) for patients with normal coronary anatomy and 3.9% (13 out of 336) for any type of anomalous coronary anatomy. Early mortality was 3.3% (5 out of 152) for patients with the circumflex coronary artery arising from sinus 2, 6.4% (3 out of 47) for patients with all coronary arteries arising from a single sinus, and 5.9% (2 out of 34) for patients with a true single coronary artery. Early mortality for patients with intramural coronaries was 1.9% (1 out of 52). No coronary pattern was found to be a risk factor for mortality. CONCLUSIONS: Patients with anomalous coronary artery anatomy had higher rates of early mortality after the arterial switch operation but this was not statistically significant. Coronary artery reoperations were rare.


Assuntos
Transposição das Grandes Artérias/mortalidade , Anomalias dos Vasos Coronários/mortalidade , Vasos Coronários , Anomalias dos Vasos Coronários/complicações , Vasos Coronários/anatomia & histologia , Vasos Coronários/patologia , Humanos , Recém-Nascido , Estudos Retrospectivos , Transposição dos Grandes Vasos/complicações , Transposição dos Grandes Vasos/cirurgia
13.
J Card Surg ; 35(5): 1085-1089, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32207192

RESUMO

Hypoplastic ascending aorta and interrupted aortic arch (IAA) are rarely associated with dextro-transposition of the great arteries (D-TGA). Severe hypoplastic ascending aorta may preclude coronary artery transfer making arterial switch operation problematic. We report a case of D-TGA with a large subpulmonic ventricular septal defect, IAA, and hypoplastic ascending aorta that underwent successful biventricular surgical repair without coronary artery transfer.


Assuntos
Aorta/cirurgia , Procedimentos Cirúrgicos Cardiovasculares/métodos , Transposição dos Grandes Vasos/cirurgia , Anormalidades Múltiplas , Aorta/patologia , Aorta Torácica/anormalidades , Aorta Torácica/cirurgia , Transposição das Grandes Artérias , Vasos Coronários/cirurgia , Feminino , Comunicação Interventricular/cirurgia , Humanos , Recém-Nascido
15.
Pediatr Cardiol ; 41(4): 747-754, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32006085

RESUMO

We tested the hypothesis that left ventricular (LV) myocardial stiffness is altered in patients with transposition of great arteries (TGA) after arterial switch operation (ASO) and explored its associations with myocardial calibrated integrated backscatter (cIB) and LV myocardial deformation. Thirty-one patients and twenty-two age-matched controls were studied. LV myocardial stiffness was assessed by diastolic wall strain (DWS) and stiffness indices including (E/e)/LV end-diastolic dimension, (E/LV global longitudinal early diastolic strain rate)/LV end-diastolic volume, and (E/LV global circumferential early diastolic strain rate)/LV end-diastolic volume, where E and e are early diastolic transmitral and mitral annular velocities, respectively. LV myocardial cIB and longitudinal and circumferential myocardial deformation were determined by conventional and speckle tracking echocardiography. Patients had significantly lower DWS, higher stiffness indices, and greater myocardial cIB than controls (all p < 0.05). The LV longitudinal and circumferential systolic strain and systolic and diastolic strain rates were significantly lower in patients than controls (all p < 0.05). Greater average myocardial cIB was associated with lower DWS (r = - 0.44, p = 0.002). Worse DWS and LV stiffness indices were found to correlate with lower mitral annular systolic velocity, mitral annular late diastolic velocity, and LV longitudinal late diastolic strain rate (all p < 0.05). LV longitudinal and circumferential systolic strain and strain rate were also found to correlate with DWS (all p < 0.05). In conclusion, LV myocardial stiffening occurs in adolescents and young adults with TGA after ASO and is associated with impairment of ventricular systolic and diastolic myocardial deformation and myocardial fibrosis.


Assuntos
Transposição das Grandes Artérias/efeitos adversos , Ventrículos do Coração/fisiopatologia , Transposição dos Grandes Vasos/cirurgia , Disfunção Ventricular Esquerda/etiologia , Adolescente , Criança , Ecocardiografia/métodos , Feminino , Fibrose , Humanos , Masculino , Miocárdio/patologia , Estudos Retrospectivos , Disfunção Ventricular Esquerda/patologia , Adulto Jovem
16.
Semin Thorac Cardiovasc Surg ; 32(2): 292-299, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31958553

RESUMO

We aimed to describe the short- and long-term outcomes of patients after an arterial switch operation (ASO) at a single institution during a 23-year period. A retrospective chart review of all patients <18 months of age who underwent an ASO between January 1995 and March 2018 at Texas Children's Hospital, Houston, TX was performed. Primary endpoints include mortality and reintervention. Perioperative mortality was defined as mortality occurring in-hospital and/or <30 days after surgery. Survival and freedom-from-reintervention were analyzed using Kaplan-Meier method, log-rank tests, and Cox regression models. The cohort included 394 patients. Diagnoses included 204 patients (52%) with intact ventricular septum, 137 (35%) with a ventricular septal defect, 17 (4%) with a ventricular septal defect and left ventricular outflow tract obstruction (LVOTO), and 36 (9%) with Taussig-Bing anomaly. Median age at surgery was 8 days (range: 1 day to 17 months) and median weight was 3.4 (range: 0.8-12.0) kg. Overall perioperative mortality was 1.3% (n = 5), 0.3% (n = 1) since 1999. Overall survival at 5, 10, and 15 years was 98.2%, 97.8%, and 97.8%, respectively. Perioperative morality was associated with prematurity (P = 0.012), <2.5 kg (P< 0.001), and longer circulatory arrest (P = 0.024) after univariate analysis. Reintervention was associated with a longer cross-clamp time (P < 0.001), <2.5 kg (P = 0.009), LVOTO resection (P = 0.047), and genetic syndrome (P= 0.011) after multivariable analysis. Current ASO expectations should include a perioperative mortality risk of <1% and good long-term survival. Reinterventions are more frequent in patients <2.5 kg, concomitant LVOTO resection, a genetic syndrome, and longer cross-clamp time.


Assuntos
Transposição das Grandes Artérias , Transposição dos Grandes Vasos/cirurgia , Transposição das Grandes Artérias/efeitos adversos , Transposição das Grandes Artérias/mortalidade , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Complicações Pós-Operatórias/mortalidade , Complicações Pós-Operatórias/terapia , Retratamento , Estudos Retrospectivos , Fatores de Risco , Texas , Fatores de Tempo , Transposição dos Grandes Vasos/diagnóstico por imagem , Transposição dos Grandes Vasos/mortalidade , Transposição dos Grandes Vasos/fisiopatologia , Resultado do Tratamento
17.
J Thorac Cardiovasc Surg ; 160(1): 229-240.e1, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-31973896

RESUMO

BACKGROUND: Hands-on surgical simulation has been sought to address training limitations within congenital heart surgery (CHS). However, there is a need for objective assessment methods to measure surgeons' performance to justify its global adoption. This study aimed to validate a procedure-specific assessment tool for the simulation of the arterial switch operation on 3D-printed models and to evaluate the consistency of scoring among evaluators with different levels of experience in CHS. METHODS: Five "expert" and 5 "junior" surgeons performed the arterial switch procedure on 3D-printed models with transposition of the great arteries during 2 hands-on surgical training courses. Their performance was retrospectively assessed by 9 evaluators with varying experience in CHS (staff surgeons, resident surgeons, and non-MD raters). Assessments were done using 2 assessment tools: the Hands-On Surgical Training-Congenital Heart Surgery (HOST-CHS) assessment tool and the global rating scale (GRS). RESULTS: The HOST-CHS tool showed a higher interrater and intrarater reliability compared with the GRS. Total scores for expert surgeons were highly consistent across all evaluators. Non-MD raters' total scores for junior surgeons were slightly higher than those of residents and staff evaluators. All grades of evaluator were able to discriminate between junior and expert surgeons. CONCLUSIONS: This study demonstrates the development and validation of an objective, procedure-specific assessment tool for the arterial switch operation with consistency among evaluators with different experience. There is now a platform for quantifying and accurately evaluating performance, which will be highly beneficial in training and developing the next generation of congenital heart surgeons.


Assuntos
Transposição das Grandes Artérias/educação , Avaliação Educacional/métodos , Treinamento por Simulação/métodos , Cirurgiões/educação , Transposição dos Grandes Vasos/cirurgia , Humanos , Impressão Tridimensional , Reprodutibilidade dos Testes , Estudos Retrospectivos
18.
Int J Cardiol ; 299: 116-122, 2020 01 15.
Artigo em Inglês | MEDLINE | ID: mdl-31405586

RESUMO

BACKGROUND: To assess the relationship between heart rate response and exercise tolerance in adults with systemic right ventricle (sRV) after atrial switch repair for Transposition of the Great Artery (TGA) in addition to other physiological parameters. METHODS: All patients with a sRV after atrial switch repair for TGA followed in our institution between June 2015 and April 2018 who underwent cardiopulmonary exercise testing (CPET) were analyzed. Cardiac imaging performed within a six-month time period of the CPET were also collected. Chronotropic incompetence was defined as the inability to achieve 80% of age-predicted maximal heart rate reserve (HRR) and <62% on a beta-blocker regimen. Patient characteristics were assessed according to tertiles of the percentage of predicted pVO2 (%ppVO2). RESULTS: We studied 70 patients (mean of age 32.4 ±â€¯7.6 years old, 51 males). Mean peak oxygen uptake was 21.5 ±â€¯5.8 mL/kg/min, corresponding to a %ppVO2 of 57 ±â€¯14.1% while mean VE/VCO2 slope was 37.1 ±â€¯8.2. There was a trend toward more exaggerated hyperventilation in patient with lower pVO2. Mean age-adjusted HRR was 68.5 ±â€¯19%. Chronotropic incompetence was observed in 65.7% and was correlated with %ppVO2 (r = 0.482; p < 0.001) as physical training evaluated with Ventilatory Anaerobic threshold (r = 0.571; p < 0.001), while no difference was found based on respiratory parameters. No echocardiographic or Magnetic Resonance Imaging parameters assessing sRV systolic function at rest were correlated with %ppVO2. CONCLUSIONS: Exercise limitation is related to the inability to increase cardiac output during exercise and is notably due to the degree of chronotropic incompetence.


Assuntos
Transposição das Grandes Artérias/tendências , Teste de Esforço/métodos , Tolerância ao Exercício/fisiologia , Frequência Cardíaca/fisiologia , Transposição dos Grandes Vasos/diagnóstico por imagem , Transposição dos Grandes Vasos/cirurgia , Adulto , Feminino , Humanos , Imagem Cinética por Ressonância Magnética/métodos , Masculino , Valor Preditivo dos Testes , Estudos Retrospectivos , Transposição dos Grandes Vasos/fisiopatologia
19.
Clin J Gastroenterol ; 13(1): 73-78, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31273674

RESUMO

A 27-year-old woman was admitted to our hospital due to a liver tumor. She had been born late at 41 weeks of gestation and had heterotaxy syndrome, polysplenia, and complete transposition of the great arteries. She underwent percutaneous balloon angioplasty at 5 years of age and the Fontan procedure at 6 years of age. At 25 years of age, computed tomography detected liver congestion. Her alpha-fetoprotein level increased from 13 to 2098 ng/dL (L3 fraction 1.8%) at 27 years of age. Gadolinium ethoxybenzyl diethylenetriamine pentaacetic acid enhanced magnetic resonance imaging showed a 22-mm liver tumor in the second liver segment. The liver tumor was enhanced in the arterial phase and washed-out in the hepatobiliary phase; the patient was, therefore, diagnosed with hepatocellular carcinoma. Radiofrequency ablation and surgery were not indicated due to the proximity of the tumor to the inferior vena cava. Therefore, proton beam therapy was selected as conservative therapy, and a dose of 74 Gray equivalents in 37 fractions was administered at the University of Tsukuba Hospital. There were no severe adverse events and she survived for 38 months after treatment without recurrence.


Assuntos
Carcinoma Hepatocelular/radioterapia , Técnica de Fontan/efeitos adversos , Síndrome de Heterotaxia/cirurgia , Neoplasias Hepáticas/radioterapia , Complicações Pós-Operatórias/radioterapia , Transposição dos Grandes Vasos/cirurgia , Adulto , Carcinoma Hepatocelular/diagnóstico por imagem , Carcinoma Hepatocelular/etiologia , Feminino , Humanos , Hepatopatias/etiologia , Neoplasias Hepáticas/diagnóstico por imagem , Neoplasias Hepáticas/etiologia , Imagem por Ressonância Magnética , Complicações Pós-Operatórias/etiologia , Terapia com Prótons , Tomografia Computadorizada por Raios X
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