Noncommitted Ventricular Septal Defect Research Articles

3D modeling and printing for complex biventricular repair of double outlet right ventricle.

Double outlet right ventricle (DORV) describes a group of congenital heart defects where pulmonary artery and aorta originate completely or predominantly from the right ventricle. The individual anatomy of DORV patients varies widely with multiple subtypes classified. Although the majority of morphologies is suitable for biventricular repair (BVR), complex DORV anatomy can render univentricular palliation (UVP) the only option. Thus, patient-specific decision-making is critical for optimal surgical treatment planning. The evolution of image processing and rapid prototyping techniques facilitate the generation of detailed virtual and physical 3D models of the patient-specific anatomy which can support this important decision process within the Heart Team. The individual cardiovascular anatomy of nine patients with complex DORV, in whom surgical decision-making was not straightforward, was reconstructed from either computed tomography or magnetic resonance imaging data. 3D reconstructions were used to characterize the morphologic details of DORV, such as size and location of the ventricular septal defect (VSD), atrioventricular valve size, ventricular volumes, relationship between the great arteries and their spatial relation to the VSD, outflow tract obstructions, coronary artery anatomy, etc. Additionally, physical models were generated. Virtual and physical models were used in the preoperative assessment to determine surgical treatment strategy, either BVR vs. UVP. Median age at operation was 13.2 months (IQR: 9.6-24.0). The DORV transposition subtype was present in six patients, three patients had a DORV-ventricular septal defect subtype. Patient-specific reconstruction was feasible for all patients despite heterogeneous image quality. Complex BVR was feasible in 5/9 patients (55%). Reasons for unsuitability for BVR were AV valve chordae interfering with potential intraventricular baffle creation, ventricular hypoplasia and non-committed VSD morphology. Evaluation in particular of qualitative data from 3D models was considered to support comprehension of complex anatomy. Image-based 3D reconstruction of patient-specific intracardiac anatomy provides valuable additional information supporting decision-making processes and surgical planning in complex cardiac malformations. Further prospective studies are required to fully appreciate the benefits of 3D technology.

Transposition of great arteries with left outflow tract obstruction and non-committed VSD: surgical management and late results.

Transposition of the great arteries with ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO) is a rare malformation. Our objective was to report on management and results of the cohort with non-committed VSD from a national registry for congenital heart disease. Multicentre data were screened in the German National Registry for Congenital Heart Defects (Berlin, Germany) for repairs of transposition of the great arteries-VSD-LVOTO. A subgroup of patients with a remote/non-committed VSD was identified. End points included survival, reoperation and a composite of reoperations for LVOTO-/VSD- or baffle-related problem. N = 47 patients were identified treated in 14 different national centres between 1984 and 2020. The mean age was 14 (standard deviation 9) months, ranging from 7 days to 9.5 years. Nine patients (19%) were treated as neonates, 21 (45%) as infants and 17 children (36%) beyond the age of 1 year. Survival was >90% (80-100%) at 20 years. Freedom from any reoperation was 30% (10-50%) at 20 years. Freedom from the composite end point was 72% (50-90%) at 20 years. Patients after Rastelli underwent more reoperations compared to those without intraventricular baffle (freedom from reoperation 14% vs 50%, P = 0.1). The rates of the composite end point were similar when comparing Rastelli to other techniques (63% vs 83%, P = 0.32). The Rastelli operation yields robust results in the setting of non-committed VSD. Late results after neonatal arterial switch operation are outstanding. If LVOTO is not resectable and neonatal arterial switch operation suboptimal, interim palliation does not negatively impact outcome, patients can be safely delayed to beyond 1 year of age.

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.

Results of Hemodynamic Correction in Patients with Double Outlet Right Ventricle

Aim. To analyze results of hemodynamic correction in surgical treatment of double outlet right ventricle (DORV).
 Маterials and methods. For the period from January 1996 to September 2017, 31 (6.03 % of total number of patients with DORV) patients underwent hemodynamic correction of DORV. The age of the patients ranged from 1 to 19 years (71.2 ± 50.5 months on the average). The weight of the patients ranged from 9 to 41 kg (19.6 ± 11.3 kg on the average). Of these, 19 (61.3 %) were male patients and 12 (38.7 %) were female patients. The overwhelming majority (25 (80.6 %)) of the patients were diagnosed with transposition-type DORV. The anatomy of DORV with non-committed ventricular septal defect was observed in 5 (16.1 %) patients. In one patient (3.1 %), the anatomy of DORV (in the form of tetralogy of Fallot) was combined with tricuspid valve atresia.
 Results. The main reasons of hemodynamic correction in 16 (51.6 %) cases was LV hypoplasia. In 2 cases it was combined with tricuspid valve (TV) straddling, and in 2 cases it was an integral part of the unbalanced form of complete atrio-ventricular communication (AVC). In one case (3.1 %), the unbalanced form of complete AVC was combined with a mixed form of the common ventricle. The mixed form of the common ventricle was the reason of hemodynamic correction in 9 (29 %) patients. In 2 (6.2 %) cases, hemodynamic correction was performed due to the anatomy of the RV hypoplasia. In the remaining 2 patients, anatomy of the common ventricle was not diagnosed, but a combination of other concomitant defects was a contraindication to biventricular correction. Palliative operations (Blalock-Taussig shunt, BTS) as the first stage of correction were performed in 16 (51.6 %) patients. In 2 patients with LV outflow tract obstruction, systemic-pulmonary anastomosis was applied in combination with plastic repair of the great vessel roots using the proprietary technique for elimination of the left ventricular outflow tract (LVOT) stenosis. Bidirectional cavopulmonary anastomosis (BCPA) was applied in 29 (93.5 %) cases. Of these, 4 (13.8 %) patients subsequently underwent total cavopulmonary anastomosis (TCPA) procedure. Two patients with good hemodynamic parameters underwent TCPA without prior palliative procedures.
 Conclusion. Palliative surgery as the first stage to hemodynamic correction is accompanied by significant improvement in hemodynamic parameters of patients. Application of BCPA as the second stage of hemodynamic correction provides good results and is required to prepare the patient for TCPA. The long-term period is characterized by improvement in the quality of life in patients with complex DORV. In the long-term period, 85.2 % of patients are classified as NYHA FC I.

Application of three-dimensional printing technology in preoperative planning of double outlet right ventricle

Objective To assess the clinic value of three-dimensional(3D) printing models in operation scheme of double outlet right ventricle(DORV). Methods From September 2015 to December 2016, the imaging data of 29 patients (13 males and 16 females) with DORV were acquired using Dual Source CT.And then the cardiac models were generated using 3D printing technology.The cardiac models were used in diagnosing the type of DORV and guiding the surgery scheme.The 3D printed models were compared with two-dimensional imaging in diagnosis and surgical scheme of DORV patients. Results Both the two-dimensional imaging and 3D printed models were effective in the diagnosis and typing of DORV.According to 3D printing models, 28 cases were consistent with the real operations, and 1 case was inconsistent.According to the two-dimensional imaging data, 20 cases of surgical strategies were consistent with the real operations and 9 cases were inconsistent.For patients with DORV with non-committed ventricular septal defect (NC-VSD), 3D printing models were more accurate in the designing of surgical strategies. Conclusions 3D printing models can display 3D anatomical structures and it is helpful in the diagnosis and making preoperative planning for DORV especially for DORV with NC-VSD, which provides a new method for the assessment of DORV. Key words: Congenital heart disease; Double outlet right ventricle; Three-dimensional printing technology; Cardiac model

20 Multimodality 4D imaging and modelling for complex double outlet right ventricles

Double outlet right ventricle can have multilevel complexity some with normal situs, levocardia and non-committed ventricular septal defect (DORV-NCVSD), and some with Dextrocardia, or abnormal situs, borderline right or left ventricles and juxtaposed atrial appendages. Decision-making can be extremely difficult due to the complex 3-dimensional spatial relationships of the atrial communication, the ventricular septal defect (VSD), the semilunar valves and the atrioventricular valves; along with the size of the ventricular cavities. Understanding of these relationships can determine whether the patient is suitable for a biventricular repair or not. Recently, advances in 3D printing have been made allowing models to be made from cross-sectional imaging (usually CT or MRI images). These can be very useful; however, they are still subject to the limitations of the chosen imaging modality. We present a case series of 4 cases of complex DORVs; 2 with NCVSD, and 2 with Dextrocardia borderline ventricles and Juxtaposed atrial appendages where we used a combination of 4D echocardiographic, CT and angiographic information fed into the materialise software to create as detailed as possible picture of all the anatomic features. This enabled detailed surgical plans to be made. Anatomical details at the time of surgery were entirely consistent with the information given by all of the various imaging modalities, allowing confidence in the planning process. Conclusion These cases demonstrate the value of using a variety of imaging modalities for complex DORV cases, ensuring that important details are not missed.

Impact of anatomic characteristics and initial biventricular surgical strategy on outcomes in various forms of double-outlet right ventricle

ObjectivesSurgical management of various forms of double-outlet right ventricle uses a variety of approaches depending on the underlying anatomic form. In this study, we sought to determine the risk factors of mortality and reoperation in those with double-outlet right ventricle undergoing biventricular repair, according to anatomic characteristics and initial surgical strategy. MethodsBetween 1992 and 2013, 433 patients were included in the study. Double-outlet right ventricle was classified as double-outlet right ventricle with subaortic ventricular septal defect associated with subpulmonary obstruction in 33% of patients (n = 141), with subaortic ventricular septal defect without subpulmonary obstruction in 30% of patients (n = 130), with subpulmonary ventricular septal defect in 32% of patients (n = 139), and with noncommitted ventricular septal defect in 5% of patients (n = 23). Three types of repairs were performed: (1) intraventricular baffle repair, n = 149 (34%); (2) intraventricular baffle repair with right ventricular outflow tract reconstruction, n = 163 (38%); and (3) intraventricular baffle repair with arterial switch operation, n = 121 (28%). ResultsThirty-day overall mortality was 7.4%. Early reoperation was needed in 6% of the cases. Early mortality was higher in the intraventricular baffle repair with arterial switch operation group (P = .01). Survival at 10 years was 86.2%, and freedom from reoperation at 10 years was 61.4%. At last follow-up (median, 5.7 years; 95% confidence interval, 4.5-6.6), mortality and reoperation rates were similar in the different surgical strategy groups. Late reoperation and late mortality were significantly higher in the double-outlet right ventricle with noncommitted ventricular septal defect group (P < .01). In multivariate analyses, risk factors for reoperation were concomitant surgical procedures (P = .03) and duration of cardiopulmonary bypass (P < .01). Risk factors for mortality were restrictive ventricular septal defect (P = .01), mitral cleft (P < .01), and associated coronary artery anomalies (P = .01). ConclusionsThose with the anatomic type of double-outlet right ventricle with noncommitted ventricular septal defect were at higher risk for reoperation and mortality. Intraventricular baffle repair with arterial switch operation was the surgical strategy in patients at higher risk of early death. Initial surgical strategy did not influence the late outcomes.

Double-Outlet Right Ventricle With Noncommitted Ventricular Septal Defect and 2 Adequate Ventricles: Is Anatomical Repair Advantageous?

The management of double-outlet right ventricle associated with anatomically noncommitted ventricular septal defect constitutes a surgical challenge. The limits for, and the specific outcomes after anatomical vs univentricular repair still remain to be established. Between 1993 and 2011, 36 consecutive patients presenting with double-outlet right ventricle or noncommitted ventricular septal defect (21 inlet, 10 muscular, and 5 central perimembranous) and 2 adequately sized ventricles underwent surgical repair at 2 centers. Right ventricular outflow tract obstruction was present in 18 of 36 patients (50%). A total of 21 patients had undergone previous palliative procedures. Anatomical repair (group I) by means of intraventricular baffle construction was performed in 24 (associated right ventricular outflow tract reconstruction in 12 and arterial switch in 5) at a median age of 10.5 months. Ventricular septal defect was surgically enlarged in 12 (50%) patients. The remaining 12 patients underwent univentricular repair (group II). There were 4 hospital deaths (11%), all in group I (P = 0.30 vs group II). A total of 8 of 20 patients in group I survivors underwent 13 reoperations after a median delay of 24 months: subaortic stenosis was the main cause for reoperation in 6 of 8 patients. There was 1 late death in group I and 2 late deaths in group II. The median follow-up was 5.6 years (95% CI: 0.2-9.8). The 10- year actuarial survival rate and freedom from reoperation were 74.7 ± 5% and 58 ± 5% in group I and 71 ± 7% and 70 ± 7% in group II, respectively. At the last visit, all survivors were in New York Heart Association class I-II. Univariate analysis showed that atrioventricular septal defect and isolated mitral cleft were associated with death (P = 0.04) and need for reoperation (P = 0.038). In conclusion, anatomical repair, associated with substantial rates of mortality and need for reoperation, should be considered with caution. Associated atrioventricular septal defect and isolated mitral cleft were the only risk factors for mortality and reoperation.

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

The study objective was to report the outcomes of biventricular repair in patients with double outlet right ventricle. 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. 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. 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.

Complexity stratification of the arterial switch operation: a second learning curve

The arterial switch operation has become a safe operation in many centres. The complexity of the procedure has evolved over the last two decades. Several anatomical features can hardly be considered complex today, namely, normal coronary anatomy, circumflex coming off the right coronary artery, eccentric ostium, and early take-off of an infundibular artery. In addition, as peri-operative mortality becomes very low, the outcomes need to be evaluated on the peri-operative morbidity, late reoperations, and late deaths. The arterial switch operation remains complex in around 20% of the cases, where one or several complexity factors are associated. The complexity of the coronary arteries is a major factor. According to a classification essentially based on the course of the coronary arteries, complex coronaries include: double-looping coronaries, anterior-looping coronaries, intramural coronaries, and single coronary ostium. The most challenging coronary pattern remains the association of a single ostium with intramural course. Other features are equally complex: severe malalignment of the commissures, aortic arch obstruction, multiple ventricular septal defect, Taussig-Bing with subaortic obstruction, double-outlet right ventricle non-committed ventricular septal defect, transposition of the great arteries-intact ventricular septum >3 weeks, transposition of the great arteries-ventricular septal defect with high lung resistances and weight <2.5 kg. Owing to the fact that the risks of arterial switch operation vary according to the experience of the centres, we defined the arterial switch operation complexity based on a subjective approach as proposed by the Aristotle comprehensive score. The recent introduction of a morbidity score will allow to stratify more accurately the outcomes when the peri-operative mortality is very low or nil. The complexity of the coronary patterns tends to be well controlled today. It remains that rare coronary failures and aortic root dilation will occur in the long term, requiring a close follow-up of the most complex patients. Successfully achieving complex arterial switch operation implies a second learning curve.

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.

Invited Commentary

This article from the Fuwai Hospital of Beijing is a report of a new operation: the double root translocation applied to double-outlet right ventricle with noncommitted ventricular septal defect (DORV-nc-VSD) and Taussig-Bing; both with pulmonary stenosis (PS) [1]. The aortic translocation is considered today by many centers as the optimal technique for repair of transposition of the great arteries, ventricular septal defect and pulmonary stenosis (TGA-VSD-PS). The double root translocation technique removes en bloc the two aortopulmonary roots and the conal septum and is emerging as an interesting new technique.

Repair of Atrioventricular Canal With Double-Outlet Right Ventricle, Transposition, or Truncus Arteriosus

Atrioventricular canal and conotruncal anomalies are a heterogeneous group of lesions presenting unique challenges for surgical repair. These are the establishment of unobstructed pathways from left ventricle (LV) to aorta and from right ventricle (RV) to pulmonary artery, closure of the inlet ventricular septal defect (VSD) and atrial septal defect (ASD) ostium primum, and the avoidance of significant left and right atrioventricular valve (AV) regurgitation. Repair of complete atrioventricular canal (CAVC) with tetralogy of Fallot (TOF) has been most commonly achieved, either using a single-patch or a 2-patch technique. In patients with CAVC with double-outlet right ventricle (DORV) with subaortic VSD extension, the 2-patch repair is not unlike that of CAVC with TOF. However, biventricular repair is most challenging in patients with CAVC and complete origin of the aorta from the RV, as in CAVC with DORV and noncommitted VSD and those with CAVC with transposition of the great arteries (TGA) and LVOTO. The technique of VSD translocation allows anatomic biventricular repair for these particularly challenging patients. The arterial switch operation with CAVC repair can be used for patients with CAVC with DORV with subpulmonary VSD extension and CAVC with TGA without left ventricular outflow tract obstruction. Biventricular repair is achievable in most patients with balanced complete atrioventricular canal and conotruncal anomaly. The extreme heterogeneity of CAVC with conotruncal anomalies requires a highly individual approach that is tailored to the specific constellation of lesions in each patient.

Determinants of repair type, reintervention, and mortality in 393 children with double-outlet right ventricle

We sought to define the prevalence of definitive end-states and their determinants in children with double-outlet right ventricle. We performed a clinical record review of 393 children with double-outlet right ventricle presenting to our institution from 1980 to 2000. Double-outlet right ventricle classification was as follows: subaortic ventricular septal defect with or without pulmonary stenosis in 47%, subpulmonic ventricular septal defect in 23%, noncommitted ventricular septal defect in 26%, and doubly committed ventricular septal defect in 4%. Hypoplastic ventricles were present in 39%, pulmonary stenosis was present in 65%, and aortic arch obstruction was present in 24%. Biventricular repair was performed in 194 patients (55%) at a median age of 10 months (range: birth to 14.0 years), and the Fontan operation (n = 182; 23%) was performed at a median age of 3.7 years (range: 6 months to 14.9 years). Results improved over time (P < .001). Factors discriminating among end-states included younger patient age at presentation (P < .001), lower weight (P < .001), and adequacy of left-sided heart structures, especially the size of the left ventricle (P < .001), aortic arch (P < .001), and mitral valve (P = .004). For complex double-outlet right ventricle, Rastelli-type repair increased early reintervention risk (P = .04) and late post-repair mortality (P = .02), whereas the arterial switch operation increased early post-repair mortality (P = .02) with a benefit of improved late post-repair survival. Biventricular repair, especially Rastelli-type reconstruction, is associated with higher late mortality and reintervention than is Fontan repair. The wisdom of extending biventricular repair to borderline anatomic candidates with hypoplastic left-sided structures or a nonsubaortic ventricular septal defect is questionable.

Biventricular repair in double outlet right ventricle: surgical results based on the STS-EACTS International Nomenclature classification☆

The STS-EACTS International Nomenclature for Congenital Heart Surgery (CHS) defines four anatomic subtypes of double outlet right ventricle (DORV) based on the relationship of the ventricular septal defect (VSD) with the great vessels and the presence of right ventricular outflow tract obstruction (RVOTO). We reviewed our experience with DORV patients and two ventricles that underwent repair, applying this nomenclature. Between January 2000 and January 2005, 50 patients with DORV and two viable ventricles underwent surgical intervention: 44 patients had biventricular repair, 3 had 1.5 ventricular repair, 2 underwent a Fontan, and 1 died prior to corrective surgery. Median age at repair was 9.1 months (range: 4D-4Y). Eighteen patients (36%) were DORV-Fallot (including 5 with AVSD and heterotaxy), 9 (18%) were DORV-TGA (Taussig-Bing), 12 (24%) were DORV-VSD, and 11 (22%) were DORV non-committed VSD. Corrective surgery included 35 repairs with a VSD-aorta baffle+/-RVOTO procedure and 12 arterial switches with a VSD-PA baffle (9 Taussig-Bing and 3 DORV-ncVSD). Associated procedures included 13 VSD enlargements, 8 subaortic resections, 9 arch repairs, 5 AVSD repairs, and 7 others. There were three deaths in the 50 patients studied (overall mortality of 6%). Excluding one patient that died prior to corrective surgery and the two patients palliated with a Fontan procedure, the actual surgical mortality for a corrective repair was 4.3% (2/47 patients). Two surgical deaths occurred following, respectively, one repair of a Taussig-Bing with an interrupted arch and a Swiss cheese VSD and one repair of ncVSD-type with pulmonary atresia that had undergone a previous cavo-pulmonary anastamosis. No late deaths occurred. Two late reoperations included a heart transplant in a DORV-Fallot patient with Swiss cheese VSD and subaortic resection in a DORV-ncVSD patient. Angioplasties were needed for PA stenosis (n=2) and aortic arch obstruction (n=2). Four patients had LV to aorta baffle gradients between 10 and 20 mmHg. All patients were of NYHA class I/II. The STS-EACTS International Nomenclature provides more uniform analysis of outcomes with respect to acceptable surgical risk and mortality. Biventricular repair can be safely achieved on selected DORV, including DORV-ncVSD and DORV with AVSD and heterotaxy lesions traditionally indicated for a single ventricle palliative approach.

Resternotomy for a Retrosternal Cardiac Pseudoaneurysm in a 1.5-Year- Old Child: A Case Report

Cardiac pseudoaneurysm is a contained rupture of the myocardium that can occur after cardiac surgery, chest trauma, and endocarditis. The wall of the pseudoaneurysm consists of fibrous tissue and lacks the structural elements found in a normal cardiac wall, and it is contained by the pericardial adhesions or the epicardial wall. Early surgery is recommended even for asymptomatic patients due to the propensity for rupture and fatal outcome. We report our experience with the surgical approach of a child with a cardiac pseudoaneurysm who had undergone a biventricular repair of a double outlet right ventricle with non-committed ventricular septal defect in the form of intraventricular tunneling.

Successful extensive enlargement of a non-committed ventricular septal defect in double outlet right ventricle

We performed an arterial switch operation in a patient with double outlet right ventricle with non-committed ventricular septal defect, and abnormal insertion of the tension apparatus of the tricuspid valve which produced moderate tricuspid regurgitation. This required extensive enlargement of the ventricular septal defect between the attachments of the cords of the tricuspid valve so as to create the interventricular rerouting that made possible the arterial switch operation. Postoperatively, we produced a straight, unobstructed, left ventricular outflow tract, improved the extent of tricuspid regurgitation, and achieved low right atrial pressures. Enlargement of the interventricular communication can set the scene for biventricular repair in this particular subset of patients with both arterial trunks arising from the morphologically right ventricle.

Biventricular repair of complete atrioventricular canal, double-outlet right ventricle and common atrium using a modified double switch technique. A valid alternative to univentricular procedure

An eleven month-old child underwent a successful modified double switch operation for total correction of complete atrioventricular canal, double outlet right ventricle, noncommitted ventricular septal defect, pulmonary stenosis, common atrium and anomalous left superior vena cava to an unroofed coronary sinus. We describe the technique of modified double switch operation, utilizing an atrial switch combined with a Rastelli type reconstruction between the venous ventricle and the pulmonary artery.

The morphologic nature of noncommitted ventricular septal defects in specimens with double-outlet right ventricle

Background: Lev's contribution to the understanding of the morphology of hearts with double-outlet right ventricle and the surgical feasibility for correction is important and remains in current use. However, the term noncommitted ventricular septal defect remains enigmatic. The aim of this study was to elucidate the morphologic nature of the noncommitted ventricular septal defect in view of its surgical implications. Methods: We examined 67 specimens with double-outlet right ventricle, focusing on the relationship of the ventricular septal defect to the semilunar orifices. Results: The defect was subaortic, subpulmonary, or doubly committed in 55 specimens. In a further 8 specimens, the defect opened into the outlet portion of the right ventricle, but the distance between the ventricular septal defect and the semilunar orifice was extensive, either because of extreme dextroposition of the aorta or a broad ventriculoinfundibular fold, which, in some cases, was associated with a long-outlet septum. A truly noncommitted ventricular septal defect was found in the inlet in the remaining 4 specimens. An atrioventricular septal defect without extension to the outlet was present in 3 cases, and a ventricular septal defect limited to the inlet was found in another case. The ventriculoinfundibular fold, part of the outlet septum and septal band or septomarginal trabeculation, had fused to form a crestlike structure. The septomarginal trabeculation is a useful landmark in the right ventricle to differentiate the inlet from the outlet in different forms of double-outlet right ventricle. Conclusion: We do not suggest to discard the Lev terminology but rather to differentiate the noncommitted ventricular septal defect into 2 types: the truly noncommitted defect of the inlet type and the not-directly-committed defect, which does open into the outlet portion of the right ventricle. The implication for the surgeon is 2-fold. The tricuspid valve or right part of the atrioventricular valve is interposed between the noncommitted ventricular septal defect and the semilunar orifice. The not-directly-committed defect opens into the outlet portion of the right ventricle but is not directly subaortic or subpulmonary.J Thorac Cardiovasc Surg 2002;124:984-90

Biventricular repair of double outlet right ventricle with non-committed ventricular septal defect (VSD) by VSD rerouting to the pulmonary artery and arterial switch.

Biventricular repair of double outlet right ventricle non-committed ventricular septal defect (DORVncVSD) is usually achieved by a VSD rerouting to the aorta. This technique can be limited by the presence of tricuspid chordae and by the pulmonary artery to tricuspid valve distance. Furthermore, there is an important risk of late subaortic obstruction related to the long patch required that creates a potential akinetic septal area. Presented here is another technique; by VSD rerouting to the pulmonary infundibulum and arterial switch. Ten patients, with DORVncVSD, underwent a VSD rerouting to the pulmonary infundibulum followed by arterial switch. Seven had a previous pulmonary artery banding and one a moderate infundibular stenosis. The median age at surgery was 16 months (range 3 weeks to 4.5 years). All patients had a bilateral infundibulum, with a large persistent subaortic conus, D malposition of the aorta, side-by-side vessels and double loop coronary patterns. The VSD was perimembranous with inlet or trabecular extension. Subaortic obstruction was constant. The VSD was severely distant from both the aortic and the pulmonary annulus. The operation was conducted through a combined approach. The VSD was constantly enlarged superiorly. The almost permanent subaortic obstruction was released. The VSD was always found quite close to the pulmonary infundibular ostium. The arterial switch technique was adapted to the complex coronary anatomy. There was one non-cardiac death. At a mean follow-up of 20 months, all nine survivors are in NYHA class I, in sinus rhythm, and have no subaortic gradient greater than 15 mm. This technique of VSD rerouting to the pulmonary artery and arterial switch limits greatly the size of the rerouting patch, respects the tricuspid chordae and is independent of the pulmonary artery-tricuspid valve distance. In this early series of biventricular repair of DORVncVSD, the VSDs were always found close to the pulmonary artery, allowing this new type of repair.