Arterial switch operation for Taussig-Bing anomaly with aortic arch obstruction.

INTRODUCTION: The short-term survival rate after single-stage correction of Taussig-Bing anomaly with aortic arch obstruction remains favorable. However, some cases are encountered occasionally in which single-stage correction was not performed during the neonatal period. Accordingly, we evaluated the midterm outcomes of different surgical strategies. METHODS: Seven patients who underwent an arterial switch operation and intraventricular rerouting as definitive surgery between 2007 and 2017 were investigated. Of these 7 patients, 3 had undergone previous pulmonary artery banding and aortic arch reconstruction. RESULTS: The median body weight at definitive surgery was 3.3 kg (range 2.9-8.3 kg). At definitive surgery, the arrest time for single-stage correction (162.3 ± 21.7 min) was significantly shorter than that of staged repair (206.3 ± 5.1 min, p = 0.020). There was no hospital or late death. One patient in both strategy groups underwent aortic reintervention 54 months and 7.1 months after the definitive operation. Neoaortic valve (perinatal pulmonary valve) diameter decreased significantly from the perinatal valve diameter following definitive surgery (median +4.94z and +2.12z, respectively, p = 0.016) but there was no significant difference in the neopulmonary valve (perinatal aortic valve) diameter. Both single-stage correction and staged repair patients showed a similar trend. At the last follow-up, no patient had greater than mild neoaortic or neopulmonary valve regurgitation. CONCLUSION: The surgical outcomes of both single-stage correction and staged correction for Taussig-Bing anomaly with aortic arch obstruction are excellent. Both strategies produce similar changes in the diameter and regurgitation grade of the neoaortic and neopulmonary valves.

Personalized Perioperative Multi-scale, Multi-physics Heart Simulation of Double Outlet Right Ventricle.

For treatment of complex congenital heart disease, computer simulation using a three-dimensional heart model may help to improve outcomes by enabling detailed preoperative evaluations. However, no highly integrated model that accurately reproduces a patient's pathophysiology, which is required for this simulation has been reported. We modelled a case of complex congenital heart disease, double outlet right ventricle with ventricular septal defect and atrial septal defect. From preoperative computed tomography images, finite element meshes of the heart and torso were created, and cell model of cardiac electrophysiology and sarcomere dynamics was implemented. The parameter values of the heart model were adjusted to reproduce the patient's electrocardiogram and haemodynamics recorded preoperatively. Two options of in silico surgery were performed using this heart model, and the resulting changes in performance were examined. Preoperative and postoperative simulations showed good agreement with clinical records including haemodynamics and measured oxyhaemoglobin saturations. The use of a detailed sarcomere model also enabled comparison of energetic efficiency between the two surgical options. A novel in silico model of congenital heart disease that integrates molecular models of cardiac function successfully reproduces the observed pathophysiology. The simulation of postoperative state by in silico surgeries can help guide clinical decision-making.

Biventricular repair of double-outlet right ventricle with noncommitted ventricular septal defect using intraventricular conduit.

OBJECTIVE: Biventricular repair of double-outlet right ventricle with noncommitted ventricular septal defect is preferred, but previously developed surgical procedures are complicated and associated with high mortality and morbidity. We developed a technique using an intraventricular conduit to connect the ventricular septal defect and the aorta in this anomaly in patients aged more than 2 years. METHODS: Thirty-one patients (age 2-23 years; median, 5.4) with double-outlet right ventricle with noncommitted ventricular septal defect underwent biventricular repair with intraventricular conduit. A 16-mm or 19-mm polytetrafluoroethylene (Gore-Tex; WL Gore & Associates, Flagstaff, Ariz) vascular prosthesis was used to construct the intraventricular conduit rerouting the ventricular septal defect to the aorta, with enlargement of the ventricular septal defect and resecting the hypertrophic muscular bands in the bilateral conus when necessary. Follow-up was made in all patients with a median duration of 93 months (range, 8-140 months). RESULTS: One patient died during hospitalization and 1 patient died at 8 months after operation, making the mortality 6.5%. The peak pressure gradient across the left ventricular outflow tract was less than 30 mm Hg in all patients but 1 (3.3%). In the last patient, it increased from 16 mm Hg early after operation to 50 mm Hg at 7 years follow-up. The peak pressure gradient across the right ventricular outflow tract ranged from 6 to 30 mm Hg in all patients. One patient had moderate mitral regurgitation with New York Heart Association class II. One patient had preoperative severe pulmonary arterial hypertension (mean pressure, 50 mm Hg) and was treated with bosentan. Other patients were in New York Heart Association class I. CONCLUSIONS: Biventricular repair with intraventricular conduit is a relatively simple and safe procedure for patients aged more than 2 years with double-outlet right ventricle with noncommitted ventricular septal defect, with excellent early and midterm outcomes.

Echocardiographic Classification and Surgical Approaches to Double-Outlet Right Ventricle for Great Arteries Arising Almost Exclusively from the Right Ventricle.

Selecting an appropriate surgical approach for double-outlet right ventricle (DORV), a complex congenital cardiac malformation with many anatomic variations, is difficult. Therefore, we determined the feasibility of using an echocardiographic classification system, which describes the anatomic variations in more precise terms than the current system does, to determine whether it could help direct surgical plans. Our system includes 8 DORV subtypes, categorized according to 3 factors: the relative positions of the great arteries (normal or abnormal), the relationship between the great arteries and the ventricular septal defect (committed or noncommitted), and the presence or absence of right ventricular outflow tract obstruction (RVOTO). Surgical approaches in 407 patients were based on their DORV subtype, as determined by echocardiography. We found that the optimal surgical management of patients classified as normal/committed/no RVOTO, normal/committed/RVOTO, and abnormal/committed/no RVOTO was, respectively, like that for patients with large ventricular septal defects, tetralogy of Fallot, and transposition of the great arteries without RVOTO. Patients with abnormal/committed/RVOTO anatomy and those with abnormal/noncommitted/RVOTO anatomy underwent intraventricular repair and double-root translocation. For patients with other types of DORV, choosing the appropriate surgical approach and biventricular repair techniques was more complex. We think that our classification system accurately groups DORV patients and enables surgeons to select the best approach for each patient's cardiac anatomy.

Double-outlet right ventricle revisited.

OBJECTIVES: Double-outlet right ventricle is a form of ventriculoarterial connection. The definition formulated by the International Society for Nomenclature of Paediatric and Congenital Heart Disease is based on hearts with both arterial trunks supported in their greater part by a morphologically right ventricle. Bilateral infundibula and ventricular septal defects are highly debated criteria. This study examines the anatomic controversies surrounding double-outlet right ventricle. We show that hearts with double-outlet right ventricle can have atrioventricular-to-arterial valvular continuity. We emphasize the difference between the interventricular communication and the zone of deficient ventricular septation. METHODS: The hearts examined were from the University of Florida in Gainesville; Johns Hopkins All Children's Hospital, St Petersburg, Fla; and Lurie Children's Hospital, Chicago, Ill. Each specimen had at least 75% of both arterial roots supported by the morphologically right ventricle, with a total of 100 hearts examined. The morphologic method was used to assess anatomic features, including arterial-atrioventricular valvular continuity, subarterial infundibular musculature, and the location of the hole between the ventricles. RESULTS: Most hearts had fibrous continuity between one of the arterial valves and an atrioventricular valve, with bilateral infundibula in 23%, and intact ventricular septum in 5%. CONCLUSIONS: Bilateral infundibula are not a defining feature of double-outlet right ventricle, representing only 23% of the specimens in our sample. The interventricular communication can have a posteroinferior muscular rim or extend to become perimembranous (58%). Double-outlet right ventricle can exist with an intact ventricular septum.

Doubly committed subarterial ventricular septal defect: A risk factor for aortic distortion in patients considered for percutaneous pulmonary valve implantation?

Percutaneous pulmonary valve implantation has gradually become the first line strategy for re-intervention for right ventricular outflow tract dysfunction during long-term follow-up after congenital cardiac surgery in many centers. We describe a case of a patient with double outlet right ventricle (Fallot's type) with a doubly committed subarterial ventricular septal defect, where the unique anatomy precluded percutaneous pulmonary valve implantation. © 2016 Wiley Periodicals, Inc.

Surgical outcomes of 380 patients with double outlet right ventricle who underwent biventricular repair.

OBJECTIVES: The study objective was to report the outcomes of biventricular repair in patients with double outlet right ventricle. METHODS: Patients with double outlet right ventricle who underwent biventricular repair at Fuwai Hospital from January 2005 to December 2012 were included. Patients were excluded if double outlet right ventricle was combined with atrioventricular septal defect, heterotaxy syndrome, atrioventricular discordance, or univentricular physiology. RESULTS: A total of 380 consecutive patients with a mean age of 1.9 ± 2.1 years (range, 1 month to 6 years) were included. Varied types of biventricular repair were customized individually. Follow-up was 90.4% complete, and the mean follow-up time was 3.4 ± 3.9 years. There were 17 (4.5%) early deaths and 7 (2.1%) late deaths. Preoperative pulmonary hypertension was the only risk factor for early mortality. Postoperative significant left ventricular outflow tract obstruction was present in 9 survivors. Patients with noncommitted ventricular septal defect had a longer crossclamp time, longer cardiopulmonary bypass time, and higher incidence of postdischarge left ventricular outflow tract obstruction. There were 4 reoperations, all of which were caused by subaortic left ventricular outflow tract obstruction. All of the pressure gradients were decreased to less than 20 mm Hg after the modified Konno procedure with an uneventful postoperative course. CONCLUSIONS: Optimal results of varied types of biventricular repair for double outlet right ventricle have been acquired. Although noncommitted ventricular septal defect is technically difficult, the outcomes of patients are favorable. Late-onset left ventricular outflow tract obstruction is the main reason for reoperation but can be successfully relieved by the modified Konno procedure.

Pulmonary root translocation in malposition of great arteries repair allows right ventricular outflow tract growth.

OBJECTIVE: Optimal surgical treatment of patients with transposition of the great arteries (TGA), ventricular septal defect (VSD), and pulmonary stenosis (PS) remains a matter of debate. This study evaluated the clinical outcome and right ventricle outflow tract performance in the long-term follow-up of patients subjected to pulmonary root translocation (PRT) as part of their surgical repair. METHODS: From April 1994 to December 2010, we operated on 44 consecutive patients (median age, 11 months). All had malposition of the great arteries as follows: TGA with VSD and PS (n = 33); double-outlet right ventricle with subpulmonary VSD (n = 7); double-outlet right ventricle with atrioventricular septal defect (n = 1); and congenitally corrected TGA with VSD and PS (n = 3). The surgical technique consisted of PRT from the left ventricle to the right ventricle after construction of an intraventricular tunnel that diverted blood flow from the left ventricle to the aorta. RESULTS: The mean follow-up time was 72 ± 52.1 months. There were 3 (6.8%) early deaths and 1 (2.3%) late death. Kaplan-Meier survival was 92.8% and reintervention-free survival was 82.9% at 12 years. Repeat echocardiographic data showed nonlinear growth of the pulmonary root and good performance of the valve at 10 years. Only 4 patients required reinterventions owing to right ventricular outflow tract problems. CONCLUSIONS: PRT is a good surgical alternative for treatment of patients with TGA complexes, VSD, and PS, with acceptable operative risk, high long-term survivals, and few reinterventions. Most patients had adequate pulmonary root growth and performance.

Arterial switch for transposition of the great vessels and Taussig-Bing anomaly after six months of age.

BACKGROUND: Indications and outcomes of the arterial switch operation in children who are older than 1 month of age and have transposition of the great arteries plus ventricular septal defect or Taussig-Bing anomaly were studied. METHODS: Arterial switch operation was performed in 68 children between January 2000 and December 2008. Thirty infants (1 to 6 months old) had transposition of the great arteries plus ventricular septal defect or Taussig-Bing anomaly (group A), and 38 children older than 6 months of age had transposition of the great arteries plus ventricular septal defect or Taussig-Bing anomaly (group B). The preoperative pulmonary artery pressure in group B was significantly higher than that in group A (46.5 +/- 16.3 mm Hg and 31.3 +/- 8.6 mm Hg, respectively; p < 0.05). Arterial switch operation was performed under general anesthesia, hypothermia (18 degrees to 22 degrees C), and low-flow (50 mL x kg(-1) x min(-1)) extracorporeal bypass circulation. Concomitant cardiac anomalies were treated during the same surgical session. RESULTS: Average extracorporeal circulation time, aortic clamping time, postoperative overall hospitalization, and intensive care unit duration were not significantly different between the two groups (p > 0.05). The operative mortality rate in group A was 10.0% (3 of 30 patients) and in group B, 7.9% (3 of 38 patients; p > 0.05). Follow-up examinations in the surviving 62 patients after a mean of 13.5 +/- 7.9 months (range, 0.5 to 56 months) showed significantly improved cardiac function without any long-term complications, and no cases of death occurred during this period. CONCLUSIONS: Arterial switch operation shows satisfactory operative outcome of transposition of the great arteries plus ventricular septal defect or Taussig-Bing anomaly in children older than the age of 6 months with moderate-to-severe pulmonary hypertension.

Rol del ecocardiograma en el estudio de la hipertensión arterial pulmonar / Role of echocardiography in the study of pulmonary arterial hypertension

La presencia de una presión arterial pulmonar media >25 mmHg en reposo y >30 mmHg en ejercicio, con una presión en cuña igual o menor de 15 mmHg y una resistencia vascular pulmonar >3 mmHg /L/ min (Uds. Wood) condiciona una entidad clínica heterogenea denominada hipertensión arterial pulmonar que tiene un amplio rango de causas o enfermedades que llevan a cambios estructurales de las pequeñas arterias pulmonares ocasionando un aumento progresivo de la presión arterial pulmonar y de la resistensia vascular pulmonar, produciendo finalmente una sobrecarga del ventrículo derecho, insuficiencia cardíaca y muerte. Es una entidad clínica con síntomas muy inespecíficos en sus etapas más tempranas y se necesita un buen criterio clínico para llegar a su diagnóstico más rápidamente. En este sentido es que adquiere valor el uso de la ecocardiografía, método que permitirá o solo cuantificar los valores de presión arterial pulmonar, sino también determinar la causa del problema, una adecuada evaluación anatómica y funcional del lecho pulmonar y de las cavidades derechas del corazón, predecir el pronostico de estos pacientes y vigilar el efecto terapéutico de tratamientos muy específicos al ayudar a detectar estados preclínicos de la enfermedad. Revisamos la utilidad y el papel del ecocardiograma en el estudio de la hipertensión arterial pulmonar.

The presence of Medium Arterial Pulmonary Pressure >25 mmHg at rest or >30mmHg during exercise associated with a wedge pulmonary pressure equal or less than 15mmHg, and a Pulmonary Vascular Resistance > 3 mmHg/L/min (Wood units) entails a heterogeneous clinical entity known as Pulmonary Arterial Hipertension which has a very wide spectrum of causes or diseases which produce estructural changes in the walls of the small pulmonary arteries causing a progressive increase of the arterial pulmonary pressure as well as the pulmonary vascular resistence, ultimately producing right ventricular overload, heart failure and death. It is a clinical entitywith very unspecific symptoms at the early stages. Wich makes the physician´s good clinical criteria needed to diagnose itsooner. In this way comes to great value the use of echocardiography, method that would allow not only to measure the arterial pulmonary pressure but would help to carify the cause of the disease, getting an adequate anatomical and funtional evaluation of the pulmonary bed and right heart chambers, predict the outcome in these patients by detecting pre-clinic stages of the disease. We reviewed the utility and role of the Echocardiogram in the study of Pulmonary Arterial Hypertension.

[Surgical treatment of Taussig-Bing anomaly in children in the first year of life].

The history of surgical treatment, clinical features, and main variants of correction of Taussig-Bing anomaly in children during the first year of life is briefly reviewed including original data obtained in the Department of Urgent Surgery of newborn and first-year infants, A. N. Bakulev Research Centre of Cardiovascular Surgery for the period of 2000-2006.

Redefining the impact of oxygen and hyperventilation after the Norwood procedure.

OBJECTIVE: Postoperative management after the Norwood procedure is aimed at optimizing systemic oxygen delivery and mixed venous oxygen saturation. High levels of fraction of inspired oxygen and hyperventilation may increase pulmonary blood flow at the expense of systemic flow. This study determines the effects of these interventions on mixed venous saturation and systemic oxygen delivery in postoperative neonates. METHODS: We prospectively studied the effects of 100% fraction of inspired oxygen and hyperventilation in 14 neonates (median age 8 days) 1 to 3 days after the Norwood procedure, while they were sedated, paralyzed, and mechanically ventilated. After establishment of baseline conditions (fraction of inspired oxygen = 29% +/- 2%, normal ventilation), patients were exposed to each of the 2 interventions in random order. Mixed venous saturation was measured through a transthoracic line in the superior vena cava. Oxygen excess factor (Omega = systemic oxygen delivery/oxygen consumption) was used as an indicator of systemic oxygen delivery. RESULTS: High levels of fraction of inspired oxygen produced significant increases from baseline in systemic saturation (90% +/- 1% vs 80% +/- 1%, P <.01), mixed venous saturation (54% +/- 3% vs 44% +/- 2%, P <.01), and oxygen excess factor (2.6% +/- 0.2% vs 2.3 +/- 0.2%, P <.01), but there was no change in arteriovenous saturation difference or blood pressure. Hyperventilation resulted in no changes in systemic or mixed venous saturation, arteriovenous saturation difference, oxygen excess factor, or blood pressure. CONCLUSIONS: High levels of fraction of inspired oxygen can improve mixed venous oxygen saturation and systemic oxygen delivery after the Norwood procedure. Hyperventilation does not change either mixed venous saturation or oxygen delivery. Management protocols aimed at minimizing the fraction of inspired oxygen and carefully controlling ventilation may not be warranted.

Biventricular repair for double-outlet right ventricle. Results and long-term follow-up.

BACKGROUND: The purpose of the present study was to define the optimal management and to identify the risk factors for death and repeat operation in patients with double-outlet right ventricle. METHODS AND RESULTS: From 1985 through 1996, 154 consecutive patients underwent biventricular repair for double-outlet right ventricle. The presence of bilateral infundibular structures was the major inclusion criteria (142 patients). According to the relationship of the ventricular septal defect (VSD) to the great arteries, there were 86 patients with a subaortic VSD (56%), 45 patients with a subpulmonary VSD (29%), 18 patients with a noncommitted VSD (12%), and 5 patients with a doubly committed VSD (3%). Sixty-five patients (42%) had undergone previous palliative procedures. At repair, the median age was 10 months, and the median weight was 6.5 kg. Two main types of repair were used: intraventricular baffle repair (n = 115) and arterial switch operation with VSD-to-pulmonary artery baffle (n = 39). There were 14 hospital deaths (9%; 70% confidence limit [CL], 7% to 12%). The only significant risk factor for early death was the presence of congenital mitral valve anomalies (P = 0.02). Twenty-eight patients (18%) required 39 repeat operations. The repeat operation rate was higher in patients with associated VSD enlargement at baffle construction (n = 29; 19%) (P = 0.01). There were 6 late deaths (4%; 70% CL, 2% to 7%). Patients presenting with pulmonary stenosis constituted a low-risk group for global death (P = 0.008). The median follow-up was 52 months. Ten-year actuarial survival and survival with freedom from repeat operation rates were 86% and 62% (70% CL, 83% to 89% and 54% to 70%), respectively. CONCLUSIONS: Long-term survival with good quality of life can be achieved after either 1- or 2-stage repair of this complex anomaly.

Conversion of modified Fontan procedure to lateral atrial tunnel cavopulmonary anastomosis.

After modified Fontan procedures with atriopulmonary anastomoses or right atrium-right ventricle conduits, some patients have progressive exercise intolerance, effusions, arrhythmias, or protein-losing enteropathy. Theoretic advantages of a lateral atrial tunnel cavopulmonary anastomosis and published clinical results suggest that conversion of other Fontan procedures to the lateral atrial tunnel may afford clinical improvement for some patients. Eight patients (8 to 25 years old) with tricuspid atresia (n =4), double-inlet left ventricle (n = 3), and double-outlet right ventricle (n=1) underwent conversion to a lateral tunnel procedure between December 1990 and November 1994. An arbitrary clinical score was assigned before the lateral tunnel procedure and at follow-up. Before conversion, patients had decreased exercise tolerance (n = 8), arrhythmias (n = 6), effusions (n = 4), and protein-losing enteropathy (n = 8). At catheterization, all had a low cardiac index (1.9 +/- 0.7 L x min(-1) x M(-2), five had elevated pulmonary vascular resistance (>3 Wood units), and three had right pulmonary venous return obstruction by compression of an enlarged right atrium. Fenestrated lateral tunnel construction was undertaken 7.3 +/- 3.6 years after atriopulmonary anastomosis, with one early death related to low cardiac output. After the lateral tunnel procedure, two patients had no clinical improvement (no change in clinical score) but five patients had either marked or partial improvement. The right pulmonary vein compression present in three patients was resolved after conversion. The mean clinical scores improved from 4.5 +/- 1 to 3.0 +/- 2 (p < 0.04). In conclusion, conversion to a lateral tunnel procedure led to clinical improvement in five of eight patients at short-term follow-up and may be particularly indicated for patients with giant right atria or pulmonary vein compression who have symptoms. Pulmonary vein compression should be looked for in patients after modified Fontan procedures and can be relieved by conversion to the lateral tunnel procedure.