Repair Of Double-outlet Right Ventricle Research Articles

Clinical Course of Residual Ventricular Septal Defects After Congenital Heart Disease Repair.

The clinical course of residual ventricular septal defects after congenital heart disease repair is not completely elucidated in the medical literature. This study assessed the incidence, size, and clinical course of residual defects.This single-center retrospective study included 132 patients who survived after ventricular septal defect patch closure (n = 107) and intracardiac repair of double-outlet right ventricle (n = 16) and tetralogy of Fallot (n = 9). Residual defect was evaluated on transthoracic echocardiogram upon hospital discharge and at outpatient clinic visits.The median age at surgery was 1.2 (0.3-13.9) years. In total, 45 (34.1%) patients presented with residual defects upon hospital discharge. The residual defects were within 2mm (n = 27), 2-3mm (n = 15), and > 3mm (n = 3), and the median size was 1.5 (0.5-3.8) mm. There was no late mortality during a median follow-up of 5.4years. Among 42 residual defects measuring < 3mm upon hospital discharge, 37 (82.2%) spontaneously closed. Further, five defects decreased in size (1.8 ± 0.6mm upon hospital discharge vs1.2 ± 0.8mm at the latest visits, p = 0.15). However, the size of three residual defects measuring > 3mm upon hospital discharge increased, and two patients required re-surgery for residual defect.Significant residual defect requiring reoperation was rare. In most cases, residual defects measuring < 3mm upon hospital discharge spontaneously closed within 5years, and the size of the other defects decreased.

Modeling Tool for Rapid Virtual Planning of the Intracardiac Baffle in Double-Outlet Right Ventricle

PurposeBiventricular repair of double-outlet right ventricle (DORV) necessitates the creation of a complex intracardiac baffle. Creation of the optimal baffle design and placement thereof can be challenging to conceptualize, even with 2-dimensional and 3-dimensional images. This report describes a recently developed methodology for creating virtual baffles to inform intraoperative repair. DescriptionA total of 3 heart models of DORV were created from cardiac magnetic resonance images. Baffles were created and visualized using custom software. EvaluationThis report demonstrates application of the tool to virtual planning of the baffle for repair of DORV in 3 cases. Models were examined by a multidisciplinary team, on screen and in virtual reality. Baffles could be rapidly created and revised to facilitate planning of the surgical procedure. ConclusionsVirtual modeling of the baffle pathway by using cardiac magnetic resonance, creation of physical templates for the baffle, and visualization in virtual reality are feasible and may be beneficial for preoperative planning of complex biventricular repairs in DORV. Further work is needed to demonstrate clinical benefit or improvement in outcomes.

Reoperations after the correction of partial abnormal venous return: a clinical case series

Rationale: The surgical procedure for partial abnormal pulmonary venous return (PAPV) implies the formation of a tunnel between the orifices of the right pulmonary veins and the left atrial cavity with the interatrial septum repair. To avoid any obstruction of the tunnel, a direct anastomosis between the superior vena cava (SVC) and the right atrial appendage is placed in a number of cases. However, the reparative procedure is potentially associated with such complications as SVC stenosis and/or pulmonary vein stenosis. Aim: To present our experience of reoperations for stenosis of systemic and pulmonary veins after primary PAPV repair. Materials and methods: From 2014 to 2018, seven patients (boys, 4) with SVC stenosis and/or with stenosis of the right pulmonary veins have been admitted to the FCHMT (Kaliningrad). Their mean bodyweight was 29.1 kg (± 12.5 kg), mean age 9.3 years (± 3.6 years). Three patients had an isolated SVC stenosis. In two patients, there was a combination of SVC stenosis and pulmonary vein obstruction (from stenosis to complete occlusion); in one patient the right pulmonary vein stenosis had been caused by thrombosis. SVC stenosis with subaortal obstruction after the repair of double-outlet right ventricle was diagnosed in one case. In six patients, various procedures for correction of SVC and/or pulmonary vein stenosis were performed. Only in one case the obstruction of systemic veins, confirmed by cardiac catheterization, turned out to be non-significant, making a reoperation unnecessary. Results: There were no deaths in this patient group. Endovascular procedures were performed in two patients. In one case, there was an unsuccessful attempt of transluminal balloon angioplasty for SVC stenosis, with subsequent Warden procedure. In another case, a 16-year old patient with SVC stenosis and high pulmonary hypertension was admitted after two previous surgeries had failed. We used a homograft as SVC prosthesis; however, after one year, a re-stenosis was identified. In this case, a SVC stent placement was successful. Due to sick sinus syndrome at one year after the Warden procedure and the repair of pulmonary veins by a homograft, an implantation of electric cardiac pacemaker was necessary in one patient. Conclusion: The curative repair of partial abnormal venous return is a well-established surgical technique with satisfactory shortand long-term results. A potential complication of the procedure is an obstruction of systemic and pulmonary veins. To prevent such complications during primary defect repair, as well as with reoperations for vein stenosis, the Warden procedure seems effective and highly reproducible technique associated with lower surgical risks.

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

ObjectiveBiventricular 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. MethodsThirty-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). ResultsOne 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. ConclusionsBiventricular 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.

Patient-specific simulations for planning treatment in congenital heart disease.

Patient-specific computational models have been extensively developed over the last decades and applied to investigate a wide range of cardiovascular problems. However, translation of these technologies into clinical applications, such as planning of medical procedures, has been limited to a few single case reports. Hence, the use of patient-specific models is still far from becoming a standard of care in clinical practice. The aim of this study is to describe our experience with a modelling framework that allows patient-specific simulations to be used for prediction of clinical outcomes. A cohort of 12 patients with congenital heart disease who were referred for percutaneous pulmonary valve implantation, stenting of aortic coarctation and surgical repair of double-outlet right ventricle was included in this study. Image data routinely acquired for clinical assessment were post-processed to set up patient-specific models and test device implantation and surgery. Finite-element and computational fluid dynamics analyses were run to assess feasibility of each intervention and provide some guidance. Results showed good agreement between simulations and clinical decision including feasibility, device choice and fluid-dynamic parameters. The promising results of this pilot study support translation of computer simulations as tools for personalization of cardiovascular treatments.

SURGEM: A solid modeling tool for planning and optimizing pediatric heart surgeries

Approximately 1% of children are born with a moderate to severe congenital heart defect, and half of them undergo one or more surgeries to fix it. SURGEM, a solid modeling environment, is used to improve surgical outcome by allowing the surgeon to design the geometry for several possible surgical options before the operation and to evaluate their relative merits using computational fluid simulation. We describe here the solid modeling and graphical user interface challenges that we have encountered while developing support for three surgeries: (1) repair of double-outlet right ventricle, which adds a graft wall within the cardiac chambers to split the solid model of the unique ventricle, (2) the Fontan procedure, which routes a graft tube to connect the inferior vena cava to the pulmonary arteries, and (3) stenosis repair, which adds a stent to expand a constricted artery. We describe several solutions that we have developed to address these challenges and to improve the performance, reliability, and usability of SURGEM for these tasks.

Assessment of the vortex flow in the post-stenotic dilatation above the pulmonary valve stenosis in an infant using echocardiography vector flow mapping

A 15-month-old male diagnosed with double outlet right ventricle (DORV) suffered from right ventricular failure 8 months after DORV repair. The motion of the anterior cusp of the pulmonary valve (PV) after the commissurotomy of the bucuspid valve was poor on echocardiography, and computed tomography showed post-stenotic dilatation ( Panels A and B ). We assessed the blood flow in the post-stenotic dilatation site of the main …

Concomitant congenital heart defect repair and Nuss procedure for pectus excavatum

The combination of complex congenital cardiac anomalies and pectus excavatum represents a technical challenge. Most concomitant repairs have been performed in adult patients. We report the uncomplicated concomitant repair of double-outlet right ventricle with absent pulmonary valve syndrome and the Nuss procedure for pectus excavatum in a 3-year-old child.

Combined obstructive hypertrophic cardiomyopathy and double outlet right ventricle in an infant with Down syndrome

Patient: Male, 2Final Diagnosis: Obstructive hypertrophic cardiomyopathySymptoms: Congestive heart failureMedication: —Clinical Procedure: Left ventricular septal myectomy • repair of congenital heart diseaseSpecialty: CardiologyObjective:Rare diseaseBackground:Hypertrophic cardiomyopathy (HCM) is uncommon in Down syndrome (DS). When combined with congenital heart disease (CHD) both morbidity and mortality may be greater compared to CHD alone. Whether HCM in DS patients is related to having trisomy 21 versus a second site mutation is unknown.Case Report:We report a case of severe HCM in an infant with DS in combination with double outlet right ventricle (DORV) who required surgery for relive of sub-aortic obstruction and congestive heart failure. We predicted that this infant would have a second site mutation involving either a sarcomeric protein or metabolic disorder as a cause for his HCM. Using current genetic and metabolic testing as well as histologic assessment of excised cardiac tissue we sought to further characterize the nature of the HCM. A successful resection of sub-aortic stenosis and DORV repair was performed. Genetic and metabolic testing was negative for gene defects and/or syndromes commonly associated with familial HCM. Excised cardiac tissue from the ventricular septum exhibited myocyte hypertrophy and sub-endocardial fibrosis but no sarcomeric disarray, myocyte fibrosis or glycogen storage. Metabolic testing for common forms of mitochondrial disease was negative. Post-operative echocardiograms show persistent, non-obstructive septal hypertrophy.Conclusions:Unlike prior reports, this child required a surgical intervention to relieve his sub-aortic obstruction. Thus, HCM in this population can be more serious that previously suspected. Although testing did not reveal the cause of his HCM, we still suggest screening for known causes of HSC until the etiology of the HCM in DS is well understood.

Catheter ablation of three macroreentrant atrial tachycardias after surgical repair of Double-Outlet Right Ventricle

A 54-year-old man with a surgically repaired double-outlet right ventricle (DORV) presented with palpitations and worsening right heart failure. His 12-lead ECG showed atrial tachycardia (AT) with an atrial cycle length (CL) of 300ms and an inverted saw-tooth F-wave pattern in the inferior leads II, III, and aVF typical of atrial flutter. Electrophysiological study and radiofrequency catheter ablation were performed. A total of 3 sustained ATs (AT1–AT3) were induced. Using the electroanatomical mapping system, CARTO3, and conventional mapping techniques, the ATs were identified as macroreentrant tachycardias circling around an incisional line on the free wall of the right atrium (AT1), the tricuspid annulus (AT2), and low voltage area in the lateral wall including the right septum (AT3). Accuracy of CARTO3 in three-dimensional reconstruction was sufficient to elucidate anatomical features (including catheter sites, incision, and low voltage areas) and macroreentrant circuits. However, conventional mapping techniques were also necessary to identify the mechanism of the tachycardias, and therefore to eliminate all of them successfully. This case demonstrates that the use of combined conventional and electroanatomical mapping techniques, such as CARTO3, can be helpful in identifying the critical isthmus for catheter ablation of macroreentrant AT in patients with surgically corrected congenital heart disease (CHD).

Surgical Management of the Neonate With Heterotaxy and Long-Term Outcomes of Heterotaxy

A review of the many challenges facing the neonate with heterotaxy has identified total anomalous pulmonary venous connection, atrioventricular valve abnormalities, pulmonary atresia, and arrhythmias including heart block as particular risk factors for the child who will pursue a single-ventricle pathway. Experience varies widely between different centers as to the percentage of patients who are suitable for biventricular repair, ranging from less than 20% to greater than 50%. Biventricular repair may only require simple baffling of anomalous systemic or pulmonary veins or may involve complex intraventricular baffle repair of double-outlet right ventricle with common atrioventricular valve. The long-term complications of heterotaxy include accelerated development of pulmonary arteriovenous malformations after the Kawashima procedure, high mortality and morbidity for the Fontan procedure (although improving results have been reported more recently), and the development of late arrhythmias. Extracardiac problems include a high risk of volvulus if malrotation is present, suggesting the need for an elective Ladd procedure. The presence of associated ciliary dyskinesia appears to be associated with an increased risk of postoperative morbidity, particularly ventilator dependence and other respiratory complications. The child with heterotaxy faces many challenges that are often underappreciated by both caregivers and families.

Double-Root Translocation for Double-Outlet Right Ventricle With Noncommitted Ventricular Septal Defect or Double-Outlet Right Ventricle With Subpulmonary Ventricular Septal Defect Associated With Pulmonary Stenosis: An Optimized Solution

Biventricular repair of double-outlet right ventricle (DORV) with noncommitted ventricular septal defect (VSD) or subpulmonary VSD, associated with pulmonary stenosis, remains controversial. The usual technique, Rastelli or réparation à l'étage ventriculaire (REV) procedure, may not meet a perfect biventricular outflow tract reconstruction in terms of hemodynamic performance and long-term outcome. Here we present an early result of an alternative solution for these anomalies by double-root translocation technique. Between August 2006 and August 2009, a total of 10 consecutive patients underwent a double-root translocation procedure, at a median age of 48 +/- 55 months (range, 1 to 16 years). The VSD was repaired with a Dacron patch, and VSD enlargement was done in 3 patients. The aortic translocation was done with (n = 4) or without (n = 6) coronary reimplantation. The neopulmonary artery was reconstructed with a monocusp bovine jugular vein patch (n = 8) or a homograft patch (n = 2). The mean follow-up interval was 21.9 +/- 11 months (range, 2 to 36). Biventricular outflow tract function was assessed by echocardiography. There were no early or late deaths, and no required reoperations. Two patients required early support by extracorporeal membrane oxygenation. Postoperative echocardiography showed satisfactory hemodynamic effect of the reconstructed biventricular outflow tract and ventricular function. One patient had trivial aortic regurgitation and 4 patients had trivial or mild pulmonary insufficiency in follow-up. The early results showed an optimized solution for DORV with noncommitted VSD or DORV with subpulmonary VSD, associated with pulmonary stenosis. Long-term benefits need to be evaluated with a larger number of patients and longer follow-up.

Implications of incising the ventricular septum in double outlet right ventricle and in the Ross–Konno operation☆

Incision into the ventricular septum in complex biventricular repair is controversial, and has been blamed for impairing left ventricular function. This retrospective study evaluates the risk of a ventricular septal incision in patients undergoing double outlet right ventricle (DORV) repair and Ross-Konno procedure. From January 2003 to September 2007, 11 patients with DORV had a ventricular septum (VS) incision and 12 DORV patients did not. Sixteen patients had a Ross-Konno, and 16 had an isolated Ross procedure. The ventricular septal incision was made to match at least the diameter of a normal aortic annulus. In DORV, the VSD was enlarged superiorly and to the left. In the Ross-Konno, the aortic annulus was enlarged towards the septum posteriorly and to the left. The median follow-up for the study is 19 months (1 month-4 years). For DORV, there were no significant differences in discharge mortality (p=0.22), late mortality (p=0.48), or late mortality plus heart transplant (p=0.093). Although patients with DORV and VSD enlargement have a more complex postoperative course, there were no differences in ECMO use (p=0.093), occurrence of permanent AV block (p=0.55), left ventricular ejection fraction (LVEF) (p=0.40), or shortening fraction (LVSF) (p=0.50). Similarly, for the Ross-Konno there were no significant differences in discharge mortality (p=0.30), late mortality (p=NS), LVEF (p=0.90) and LVSF (p=0.52) compared to the Ross, even though the Ross-Konno patients were significantly younger (p<0.0001). Making a ventricular septal incision in DORV repair and in the Ross-Konno operation does not increase mortality and does not impair the LV function. The restriction of the VSD remains an important issue in the management of complex DORV. These encouraging results need to be confirmed by larger series.

The course of pregnancy and delivery in a patient after a double-outlet right ventricle Fontan repair and the influence of this procedure on her general condition: Report of a case

The course of pregnancy and delivery in a patient after Fontan repair of double-outlet right ventricle is herein presented. She uneventfully delivered a healthy infant in good general condition at 38 weeks of gestation through a caesarean section, at 24 years after cardiosurgery. Although the patient demonstrated a good hemodynamic state after the operation and conceived while being clinically stable, a worsening of her general condition was observed. After a 4-year follow-up it was clear that pregnancy and delivery had a harmful effect on her cardiovascular function.

Pentalogy of Cantrell with a double-outlet right ventricle: 3.5-year follow-up in a prenatally diagnosed patient

Pentalogy of Cantrell is a rare congenital defect associated with five ventral midline anomalies and high mortality. An obstetric sonogram revealed a fetus with a body wall defect suggesting a diagnosis of this condition. Soon after birth, the infant underwent a closure of the upper abdominal wall defect followed by the successful repair of double-outlet right ventricle and pulmonary valve stenosis at the age of 5 months. The patient is currently alive and well 3.5 years after surgery. It is concluded that a deliberate therapeutic strategy based on the intrauterine diagnosis may alter the natural history of this devastating disorder.

The Contegra conduit in the right ventricular outflow tract induces supravalvular stenosis

ObjectiveWe sought to evaluate the incidence and nature of pulmonary stenosis after implantation of the bovine jugular vein graft (Contegra; Medtronic, Inc, Minneapolis, Minn) in the right ventricular outflow tract.MethodsBetween May 2000 and September 2002, 58 Contegra conduits (8-22 mm) were implanted during primary (n = 27) or redo operations (n = 31) in 57 patients, with ages ranging from 2 days to 48 years (mean, 9 years). Indications were truncus arteriosus (n = 16), tetralogy of Fallot (n = 28), pulmonary replacement in the Ross operation (n = 10), and Rastelli-type repair for double-outlet right ventricle (n = 4). Echocardiography was prospectively performed by a fixed team of investigators during follow-up (mean, 22.7 ± 10 months). A peak gradient of greater than 50 mm Hg was considered severe stenosis.ResultsTwo patients died from Staphylococcus aureus–induced septicemia and enterococcal endocarditis after 12 days and 12 weeks, respectively. One patient died of heart failure caused by endocardial fibroelastosis after 1 year. Freedom from severe stenosis at the distal anastomosis was 91% ± 3% at 3 months, 68% ± 6% at 12 months, and 49% ± 8% at 24 months. The risk of development of stenosis does not change over time. Younger age and its derivatives (graft size and indication) are significantly related to the occurrence of severe stenosis (P < .0001). Seventeen (29%) conduits required an endovascular intervention (balloon dilatation or stent). Seven (12%) conduits were explanted (endocarditis, 2; stenosis, 5). Histologic analysis of the explanted conduits showed excessive proliferation of neointima at the level of the distal anastomosis. Valve regurgitation was observed in 9 (16%) conduits and was always secondary to dilatation in the presence of severe distal stenosis.ConclusionThe Contegra conduit induces a neointimal proliferation at the level of the pulmonary anastomosis. This leads to a high incidence of severe stenosis at intermediate-term follow-up. We sought to evaluate the incidence and nature of pulmonary stenosis after implantation of the bovine jugular vein graft (Contegra; Medtronic, Inc, Minneapolis, Minn) in the right ventricular outflow tract. Between May 2000 and September 2002, 58 Contegra conduits (8-22 mm) were implanted during primary (n = 27) or redo operations (n = 31) in 57 patients, with ages ranging from 2 days to 48 years (mean, 9 years). Indications were truncus arteriosus (n = 16), tetralogy of Fallot (n = 28), pulmonary replacement in the Ross operation (n = 10), and Rastelli-type repair for double-outlet right ventricle (n = 4). Echocardiography was prospectively performed by a fixed team of investigators during follow-up (mean, 22.7 ± 10 months). A peak gradient of greater than 50 mm Hg was considered severe stenosis. Two patients died from Staphylococcus aureus–induced septicemia and enterococcal endocarditis after 12 days and 12 weeks, respectively. One patient died of heart failure caused by endocardial fibroelastosis after 1 year. Freedom from severe stenosis at the distal anastomosis was 91% ± 3% at 3 months, 68% ± 6% at 12 months, and 49% ± 8% at 24 months. The risk of development of stenosis does not change over time. Younger age and its derivatives (graft size and indication) are significantly related to the occurrence of severe stenosis (P < .0001). Seventeen (29%) conduits required an endovascular intervention (balloon dilatation or stent). Seven (12%) conduits were explanted (endocarditis, 2; stenosis, 5). Histologic analysis of the explanted conduits showed excessive proliferation of neointima at the level of the distal anastomosis. Valve regurgitation was observed in 9 (16%) conduits and was always secondary to dilatation in the presence of severe distal stenosis. The Contegra conduit induces a neointimal proliferation at the level of the pulmonary anastomosis. This leads to a high incidence of severe stenosis at intermediate-term follow-up.

The Contegra conduit in the right ventricular outflow tract induces supravalvular stenosis

We sought to evaluate the incidence and nature of pulmonary stenosis after implantation of the bovine jugular vein graft (Contegra; Medtronic, Inc, Minneapolis, Minn) in the right ventricular outflow tract. Between May 2000 and September 2002, 58 Contegra conduits (8-22 mm) were implanted during primary (n = 27) or redo operations (n = 31) in 57 patients, with ages ranging from 2 days to 48 years (mean, 9 years). Indications were truncus arteriosus (n = 16), tetralogy of Fallot (n = 28), pulmonary replacement in the Ross operation (n = 10), and Rastelli-type repair for double-outlet right ventricle (n = 4). Echocardiography was prospectively performed by a fixed team of investigators during follow-up (mean, 22.7 +/- 10 months). A peak gradient of greater than 50 mm Hg was considered severe stenosis. Two patients died from Staphylococcus aureus -induced septicemia and enterococcal endocarditis after 12 days and 12 weeks, respectively. One patient died of heart failure caused by endocardial fibroelastosis after 1 year. Freedom from severe stenosis at the distal anastomosis was 91% +/- 3% at 3 months, 68% +/- 6% at 12 months, and 49% +/- 8% at 24 months. The risk of development of stenosis does not change over time. Younger age and its derivatives (graft size and indication) are significantly related to the occurrence of severe stenosis ( P < .0001). Seventeen (29%) conduits required an endovascular intervention (balloon dilatation or stent). Seven (12%) conduits were explanted (endocarditis, 2; stenosis, 5). Histologic analysis of the explanted conduits showed excessive proliferation of neointima at the level of the distal anastomosis. Valve regurgitation was observed in 9 (16%) conduits and was always secondary to dilatation in the presence of severe distal stenosis. The Contegra conduit induces a neointimal proliferation at the level of the pulmonary anastomosis. This leads to a high incidence of severe stenosis at intermediate-term follow-up.

Results of arterial switch operation for primary total correction of the Taussig-Bing anomaly

Background This study evaluates the results of the arterial switch operation for early total repair of double-outlet right ventricle with subpulmonary ventricular septal defect (the Taussig-Bing heart). Methods From 1986 through April 2003, 27 patients with Taussig-Bing anomaly underwent arterial switch operation. Twenty patients were neonates (n = 11) or infants younger than 3 months (n = 9). Obstruction of aortic arch (n = 19) or subaortic right ventricular outflow tract obstruction (n = 20) and unusual coronary artery patterns (n = 19) were common. Total correction as a single procedure was performed in 21 patients. Events are depicted by Kaplan-Meier curves. Results There was 1 patient hospital death at 2 months after repair. One patient died late that was not cardiac related. Survival was 92% ± 6% at 8 months and remained constant thereafter. Four patients underwent reoperation (1 for residual aortic arch obstruction and 3 for subvalvular and valvular pulmonary stenosis). Freedom from reoperation decreased to stabilize at 83% ± 8% after 2 years. The risk to have right ventricular outflow tract obstruction develop was 33% ± 10% at 1 year, increasing slowly and leveling out at 57% ± 12% at year 5 and thereafter. Statistical analysis revealed no significant risk factor for death or need for reoperation. Conclusions The Taussig-Bing anomaly should be corrected in the neonatal period or in early infancy by arterial switch operation, closure of the ventricular septal defect, and simultaneous correction of associated cardiovascular anomalies as a one-stage procedure. Right ventricular outflow tract obstruction often complicates the postoperative course and is the main cause for reintervention.

Definitive repair of double-outlet right ventricle with subaortic ventricular septal defect and pulmonary atresia in adulthood

Definitive surgical repair of double-outlet right ventricle (DORV) is usually done early in life (≤6 months), with 10-year survival rates as high as 90%.1,2 Corrective surgery later in life (after an early palliative operation) is not usually an option due to the presence of significant pulmonary hypertension and right ventricular dysfunction. Two reported patients with repair after the age of 19 died within 36 months.3,4 We present a young man with 2 previous palliative operations, who at 21 years of age, underwent repair of DORV with a subaortic ventricular septal defect and pulmonary atresia.

Use of an Antithrombin Agent, Argatroban, in Two Patients with Extracorporeal Membrane Oxygenation after Pediatric Open Heart Surgery. Argatroban in Two Pediatric Cardiac ECMO.

Background: Argatroban, a selective and competitive antithrombin agent, produces antithrombotic effects without aggravating bleeding tendency. We used argatroban as an anticoagulant during extracorporeal membrane oxygenation (ECMO) in two pediatric patients with low output syndrome following open heart surgery.Patients and Results: Patient 1; A 1-year-old boy underwent a radical operation for repair of a common atrioventricular canal. Following surgery, ECMO was successfully performed with argatroban (1.5 to 7.0μg/kg/min) for 4 days. Patient 2; A 3-year-old girl underwent intraventricular baffle repair for double-outlet right ventricle. ECMO was started with heparin, but it was replaced with argatroban (5.0μg/kg/min) on day 2 because of massive gastrointestinal bleeding. This treatment resulted in a gradual decrease in bleeding. ECMO could be carried out without further bleeding for another 3 days.Conclusions: Our clinical findings suggest that argatroban may be a useful anticoagulant for ECMO.