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@#Objective To explore growth pattern of neo-aortic root as well as development of neo-aortic regurgitation after arterial switch operation (ASO) for Taussig-Bing anomaly. Methods From 2002 to 2017, the patients who received ASO, and were discharged alive from Shanghai Children’s Medical Center and followed up for more than 3 years were retrospectively involved in this study. Results A total of 127 patients were enrolled. There were 98 (77.2%) males, the median age at ASO was 73.0 d and the average weight was 4.7 kg. Forty-five (35.4%) children were complicated with mild or mild-to-moderate pulmonary insufficiency (PI) before ASO. The average follow-up time was 7.0 years. During the follow-up, 14 (11.0%) children presented moderate or greater neo-aortic regurgitation (neo-AR). The diameter of neo-aortic annulus and sinus of Valsalva was beyond normal range during the entire follow-up. The average diameter of neo-aortic annulus was 18.0 mm at 5 years and 20.5 mm at 10 years. The average diameter of sinus of Valsalva was 25.9 mm at 5 years and 31.1 mm at 10 years. Neo-AR continued to develop over time. The diameter of children who developed moderate or greater neo-AR was constantly larger than that of children who did not (χ2=18.3, P<0.001). Preoperative mild or mild-to-moderate PI was an independent risk factor for the development of moderate or greater neo-AR during mid-to-long term follow-up (c-HR=3.46, P=0.03). Conclusion The diameters of neo-aortic annulus and sinus of Valsalva of Taussig-Bing children who receive ASO repair continue to expand without normalization. The dilation of annulus correlates with the development of neo-AR. PI before ASO repair increases the risk of neo-AR development.
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@#Objective To explore the effect of LeCompte maneuver on in-hospital mortality and mid-to-long term reintervention after single-stage arterial switch operation in children with side-by-side Taussig-Bing anomaly. Methods Clinical data of patients diagnosed with side-by-side Taussig-Bing anomaly and undergoing single-stage arterial switch operation in Shanghai Children’s Medical Center from 2006 to 2017 were retrospectively analyzed. Patients were divided into two groups based on whether LeCompte maneuver was performed: a LeCompte maneuver group and a non LeCompte maneuver group. The clinical data of two groups were compared. Results Finally 92 patients were collected. LeCompte maneuver was performed in 32 out of 92 patients with a median age of 65.0 days and an average weight of 4.3 kg, among whom 24 (75.0%) were male. Fifteen (46.9%) patients received concomitant aortic arch repair while 12 (37.5%) patients were associated with coronary artery malformation. LeCompte maneuver was not performed in 60 patients with a median age of 81.0 days and an average weight of 4.8 kg, among whom 45 (75.0%) were male. Twenty-two (36.7%) patients received concomitant aortic arch repair while 35 (58.3%) patients were associated with coronary artery malformation. The average cardiopulmonary bypass duration of the LeCompte maneuver group showed no statistical difference from the non LeCompte maneuver group (179.0±60.0 min vs. 203.0±74.0 min, P=0.093). The in-hospital mortality of the two groups were 6 (18.8%) and 7 (11.7%), respectively, which also showed no statistical difference (P=0.364). The median follow-up period was 4.1 (1.6, 7.5) years for 79 patients with 8 lost to follow-up, and no death was observed. Kaplan-Meier curve and log-rank test showed no statistical difference in overall mid-to-long term reintervention rate (P=0.850) as well as right ventricular outflow tract and pulmonary artery reintervention rate (P=0.240) with or without LeCompte maneuver. Conclusion Whether or not to perform LeCompte maneuver shows no statistical impact on in-hospital mortality and mid-to-long term reintervention rate of single-stage arterial switch operation for side-by-side Taussig-Bing anomaly.
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Objective To review the surgical outcomes of single-stage arterial switch with aortic arch reconstruction for Taussig-Bing anomaly with aortic arch obstruction in a single institution of china.Methods The clinical data of 26 consecutive patients with Taussig-Bing anomaly or TGA and aortic arch obstruction undergoing single-stage arterial switch with aortic arch reconstruction in Guangzhou Women and Children's Medical Center from November 2009 to December 2015 were retrospectively reviewed,and the risk factors for re-intervention were analyzed.Results There were three in-hospital deaths,and the earlyterm survival rate was 88.5％ (23/26).Follow-up data were available for all patients who survived the operation(range 24-103 months).There were two late deaths.Survival estimates for the entire cohort following surgery were 84％ (95％ CI:70％-98％) and 77％ (95％ CI:58％-96％) at 1 year and 5 years,respectively.Echocardiology showed three cases of aortic regurgitation,two cases of supra-aortic stenosis,one case of recoarctation,seven cases of right heart outflow tract obstruction,and two cases of moderate pulmonary regurgitation.Six patients required a re-intervention during the follow-up period with no mortality.Freedom from re-intervention were 90％ (95％ CI:78％-99％) and 59％ (95％ CI:32％-86％) at 1 year and 5 years,respectively.Freedom from re-intervention for right-sided outflow tract obstruction were 94％ (95％ CI:84％-99％)and 66％ (95％CI:37％-95％) at 1 year and 5 years,respectively.All survivors remained in good condition(New York Heart association functional class Ⅰ or Ⅱ).Conclusion Single-stage arterial switch with aortic arch reconstruction for Taussig-Bing anomaly with aortic arch obstruction had favorable early-and mid-term outcomes.The incidence of right heart outflow tract obstruction is still higher.The optimal operative procedure should be chosen according to the long-term follow-up.
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La anomalía de Taussig-Bing es una cardiopatía congénita cianosante caracterizada por la dextrotransposición de grandes vasos. Esta produce una doble salida arterial desde el ventrículo derecho, asociado a una comunicación interventricular. Este cuadro puede generar una hipertensión pulmonar secundaria al aumento de las resistencias vasculares y un flujo reverso cardiaco, conocido como síndrome de Eisenmenger. Normalmente, se presenta antes de la pubertad, aunque en ocasiones, puede debutar en la vida adulta, progresando durante dicha etapa. Clínicamente, se caracteriza por rasgos crónicos, como las acropaquias, la disnea, la sensación de cansancio o la cianosis. El diagnóstico de este tipo de cardiopatías se basa en la clínica y en las pruebas de imagen, preferentemente en el estudio ecocardiográfico fetal o durante la edad pediátrica. El tratamiento de elección es la corrección quirúrgica de las malformaciones cardiacas, siendo preferente la rectificación de la salida de la aorta y el cierre de la comunicación interventricular. El pronóstico depende del grado de hipertensión pulmonar, del momento del diagnóstico y de la corrección quirúrgica precoz. A edades tempranas se obtiene un mejor resultado, aunque las tasas de mortalidad alcanzan 50 % en algunos casos, incluso tras una corrección quirúrgica óptima. La gestación no está recomendada en pacientes que padecen dicha patología, la cual se ha contraindicado, según algunos estudios, en ausencia de tratamiento adecuado. Así pues, describimos un caso en el que una gestante con una anomalía Taussig-Bing sufre una atonía uterina y un posterior paro cardiorrespiratoria tras el parto, realizado mediante cesárea electiva, tras la que la paciente falleció(AU)
The Taussig-Bing anomaly is a congenital cyanosis characterized by the dextrotransposition of large vessels. It produces a double arterial exit from the right ventricle, associated with an interventricular communication. This may lead to pulmonary hypertension secondary to increased vascular resistance and a cardiac reverse flow, known as Eisenmenger syndrome. Generally, it occurs before puberty, although occasionally, it can debut in adults, progressing during that stage. It is clinically characterized by chronic features, such as acropachies, dyspnea, tiredness or cyanosis. The diagnosis of this type of heart disease is based on clinical exam and imaging tests, if at all possible in the fetal echocardiographic study or during the pediatric age. The surgical correction of cardiac malformations is the treatment of choice, modifying the aortic exit and closing of ventricular septal defect. The prognosis depends on the degree of pulmonary hypertension, the time of diagnosis, and the early surgical correction. Better outcome is obtained at early ages, although mortality rates reach 50% in some cases, even after optimal surgical correction. Pregnancy is not recommended in patients suffering from this disease. Some studies contraindicate pregnancy in absence of proper treatment. Thus, we describe a case of a pregnant woman with a Taussig-Bing anomaly, who suffered uterine atony and a subsequent cardiorespiratory arrest after delivery. Elective cesarean section was performed. This patient died(AU)
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Humanos , Feminino , Gravidez , Adulto , Dupla Via de Saída do Ventrículo Direito/complicações , Dupla Via de Saída do Ventrículo Direito/mortalidade , Morte Materna , Cesárea/mortalidade , Complexo de Eisenmenger/mortalidadeRESUMO
A 52 day-old male infant who had Taussig-Bing anomaly with coarctation of the aorta underwent initial palliative Damus-Kaye-Stansel (DKS) procedure including arch reconstruction because of suspected intramural coronary artery, size discrepancy of great arteries, potential subaortic stenosis, refractory pneumonia, and severe congestive heart failure. Total repair was done 44 months later, which was composed of VSD patch closure, DKS take-down, and arterial switch procedure. We report a successful case of DKS take-down and arterial switch operation for the reuse of native aortic and pulmonary valves rather than Rastelli-type procedure in a patient with Taussig-Bing anomaly having palliative DKS procedure.
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Humanos , Lactente , Masculino , Coartação Aórtica , Artérias , Constrição Patológica , Vasos Coronários , Dupla Via de Saída do Ventrículo Direito , Cardiopatias Congênitas , Insuficiência Cardíaca , Pneumonia , Valva PulmonarRESUMO
BACKGROUND: Excellent clinical results of the arterial switch operation and the limited availablity of the intraventricular rerouting has recently made an arterial switch operation to become the therapeutic method of choice for the repair of double-outlet right ventricle (DORV) with subpulmonary ventricular septal defect (VSD). The early and midterm outcomes of arterial switch operation for this anomaly were evaluated. MATERIAL AND METHOD: Between August 1994 and July 2002, 13 patients underwent an arterial switch operation for the correction of double-outlet right ventricle with subpulmonary VSD at Dong-A university hospital.. The 50% rule was used to define DORV. Median age and mean body weight were 27 days (range, 3~120 days) and 3.8+/-0.7 kg (range, 2.92~5.3 kg) respectively. Aortic arch anomalies were associated in 6 cases (46.2%), which were all repaired through one-stage operation. The relationship of the great arteries were side-by-side in 8 cases (61.5%) and anteroposterior in 5 (38.5%). Coronary artery patterns were 1LCx-2R in 6 cases, retropulmonary left coronary artery (LCA) in 6, and intramural LCA in 1 respectively. The enlargement of VSD was required in 1 patient and the patch enlargement of right ventricular outflow tract was performed in another one patient. The Lecompte maneuver was used in all but 3 patients with a side by side relationship of the great arteries. RESULT: Overall postoperative hospital mortality was 23.1% (3/13). All operative deaths were occurred in the patients with aortic arch anomalies. There was one late death related to the postoperative complication of the central nerve system during the mean follow-up of 41.3+/-30.7 months. Pulmonary valvar stenosis (>30 mmHg of pressure gradient) developed in 1 patient (10%) and left pulmonary artery stenosis in 2 (20%), among them, one required reoperation 52 months after repair. There was an asymptomatic patient with moderate aortic regurgitation. 5-year survival rate including operative deaths was 68.3%. CONCLUSION: Although the operative mortality is high in the patients with aortic arch anomaly, the arterial switch operation for DORV with supbpulmonary VSD can be performed with low operative mortality and low reoperation rate in the patients without arch anomaly. The arterial switch operation can be considered a good option for this complex anomaly.
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Humanos , Aorta Torácica , Insuficiência da Valva Aórtica , Artérias , Peso Corporal , Constrição Patológica , Vasos Coronários , Dupla Via de Saída do Ventrículo Direito , Seguimentos , Defeitos dos Septos Cardíacos , Comunicação Interventricular , Mortalidade Hospitalar , Mortalidade , Complicações Pós-Operatórias , Artéria Pulmonar , Reoperação , Taxa de SobrevidaRESUMO
The patient was a 30-day-old female infant with symptoms of severe dyspnea and cyanosis, when she was admitted to the ER. The echocardiography revealed DORV with subpulmonary VSD, and the diagnosis of Taussig-Bing anomaly was made. Two days after admission, an urgent operation was performed. The operation consisted of intraventricular tunnel repair and arterial switch operation. She was discharged, and after checking her chest X-ray through OPD, there was no interval change of cardiomegaly. She was then re-admitted, and the angiography revealed coactation of aorta. We performed a resection and end-to-end anastomosis of aorta. She is currently in good condition 11 months postoperatively.
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Feminino , Humanos , Lactente , Angiografia , Aorta , Coartação Aórtica , Cardiomegalia , Cianose , Diagnóstico , Dupla Via de Saída do Ventrículo Direito , Dispneia , Ecocardiografia , TóraxRESUMO
We report a case of the successful anatomical correction of the Taussig-Bing anomaly associated with the interrupted aortic arch and intramural left coronary artery for an 38 day-old infant. Aortic arch and neoaortic reconstructions were conducted without any prosthetic or pericardial patch. Intramural left coronary was separated from right one after partial detachment of aortic commissure and both coronary artery buttons were transferred separately to the proximal main pulmonary artery(neo-aorta). Delayed sternal closure was done 3 days after the operation and hospital discharge was delayed for a month because of postoperative pneumonia. Now he is 5 months old and free of symptoms and cardiac drugs.
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Humanos , Lactente , Aorta Torácica , Vasos Coronários , Dupla Via de Saída do Ventrículo Direito , PneumoniaRESUMO
Taussig-Bing anomaly is infrequently associated with interrupted aortic arch and size discrepancy of great arteries makes it difficult to undergo arch recons- truction and arterial switch operation. A 20-day old male infant was admitted with the diagnosis of Taussig-Bing anomaly with type B interrupted aortic arch. Multi-organ failure, due to the diminution of ductal flow, was stabilized after 3 weeks of prostaglandin E1 and controlled ventilatory support. The surgical correction consisted of VSD closure, arterial swtich and extended aortic arch reconstruction. The marked disparity between the hypoplastic ascending aorta and the dilated main pulmonary artery was overcome by constructing distal neoaorta using both native ascending and descending aortic tissue. The patient was extubated on postoperative 2nd day. Postoperative catheterization showed no left ventricular outflow obstruction, no intracardiac shunt, and no incompetence of neoaortic valve.
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Humanos , Lactente , Masculino , Alprostadil , Aorta , Aorta Torácica , Artérias , Cateterismo , Catéteres , Diagnóstico , Dupla Via de Saída do Ventrículo Direito , Artéria Pulmonar , Obstrução do Fluxo Ventricular ExternoRESUMO
Objective Aortic arch obstruction is a commonly associated malformation in the patients with Taussig-Bing anoma- ly.Herein,we reported our outcomes of single stage corrections in patients with the Taussig-Bing anomaly associated with aortic arch obstruction.Methods Between May,2000 and Dec,2006,12 patients with Taussig-Bing anomaly associated with aortic arch obstr- uction (5 patients associated with interrupted aortic arch) underwent arterial switch operation with baffling of the left ventricle to neo- aorta.The corrections of aortic arch obstruction included extended resection combined with end to end anastomosis to aortic arch or end to side anastomosis to ascending aorta.Results The hospital mortality rate was 25% (3/12).The ventilating time and ICU stay were (7.4?2.1) days and (11.7?4.6) days,respectively.No reoperation because of residual anomalies was required.Conclusion Tanssig-Bing anomaly,especially associated with aortic arch obstruction,is different from transposition of great artery.The opti- mized operative indications,techniques and the managements of aortic arch obstruction are discussed in the article.