Reverse double switch operation for the borderline left ventricle.

OBJECTIVE(S): This study investigates outcomes of the reverse double switch operation (R-DSO) and ventricular switch, novel approaches for patients with D-looped borderline left hearts that utilize the morphologic right ventricle as the systemic ventricle and the hypoplastic left ventricle as the subpulmonary ventricle. METHODS: This retrospective review analyzed early outcomes of children who underwent R-DSO/ventricular switch at a single institution between 2015 and 2023. Our primary end points were right ventricular (RV) function and tricuspid regurgitation. Secondary outcomes included mortality, reoperation, and perioperative complications. RESULTS: Twenty-eight patients underwent either R-DSO (n = 19) or ventricular switch (n = 9). In 19 patients, a decompressing bidirectional cavopulmonary shunt was utilized, creating a reverse 1.5 switch. Median age at R-DSO/ventricular switch was 3.1 years (range, 9 months-12 years). At a median follow-up of 1.0 year (range, 38 days-7.2 years), no mortalities or heart transplants had occurred. Mild-moderate or greater RV dysfunction was detected in 4 patients, and mild-moderate or worse tricuspid regurgitation was also detected in 4 patients. Three patients required reoperations. Preoperative RV ejection fraction <55% was associated with higher incidence of postoperative RV dysfunction. CONCLUSIONS: The R-DSO/VS strategy is an alternative to single-ventricle palliation or anatomic biventricular repair in the borderline left heart. Concerns for RV dysfunction and tricuspid regurgitation mandate close monitoring. Patients with preoperative RV dysfunction may be at risk for postoperative RV dysfunction. Further studies with longer follow-up are needed to delineate outcomes in comparison to the Fontan pathway and identify optimal candidates for this novel strategy.

The cone repair allows right ventricle rehabilitation with excellent tricuspid valve function following the Starnes procedure.

BACKGROUND: We present a case series of right ventricle (RV) rehabilitation after the Starnes procedure in patients with Ebstein anomaly (EA), applying the Cone repair of the tricuspid valve (TV) to achieve 2-ventricle or 1.5-ventricle physiology. METHODS: This is a retrospective database analysis from 2 institutions in North America. We included all consecutive cases of Cone repair after the Starnes procedure. The data are expressed as median and interquartile range (IQR). RESULTS: Eleven patients underwent RV rehabilitation between 2019 and 2023 after initial Starnes palliation at a median age of 27 months (IQR, 20.5 months). All patients were critically ill before their Starnes procedure, and 4 were on extracorporeal membrane oxygenation. Before the Cone repair, the median preoperative regurgitant velocity at the Starnes patch was 1.65 m/s (IQR, 1.3 m/s). During the Cone procedure, 9 patients required a concomitant pulmonary valve repair, of whom 3 needed a transannular monocusp patch. Four patients were successfully rerouted to a 2-ventricle repair, and 7 patients with a previous Glenn achieved 1.5-ventricle circulation. There were no cases of heart block and no deaths. Seven patients had trivial, 3 patients had mild, and 1 patient had moderate tricuspid regurgitation (TR) at a median follow-up of 11 months (IQR, 21.5 months). There was no significant TV stenosis; all patients had good functional status at the last follow-up despite severe RV dysfunction in 1 patient. CONCLUSIONS: After the Starnes procedure, the Cone repair allowed RV rehabilitation, resulting in trivial or mild TR at a midterm follow-up. The Starnes procedure is a reproducible technique that no longer commits patients to lifetime single-ventricle physiology.

Hybrid palliation to promote growth of left ventricle and left ventricular outflow tract.

OBJECTIVES: In patients with borderline left hearts or a severe left ventricular outflow tract obstruction, hybrid palliation can be used to stabilize the patient and postpone biventricular repair (BVR). In this study, we analysed growth of left-sided structures and outcomes of these patients. METHODS: We conducted a retrospective cohort study including patients who received hybrid palliation between January 2010 and September 2023. Echo measurements were collected at hybrid palliation, BVR and last follow-up. Growth of left ventricular structures were analysed. RESULTS: In 38 patients, hybrid palliation was used to promote growth of left ventricular structures. In total, 15 patients received a Ross-Konno/Yasui procedure, while 23 patients received conventional BVR. In patients with a conventional BVR, a significant increase was found in left ventricular volume indexed by body surface area, Z-score of aortic valve and left ventricular outflow tract between hybrid palliation and BVR. Mitral valve Z-score did not increase significantly. After BVR until follow-up, only increase of the aortic valve Z-scores and left ventricular volume indexed by body surface area was found significant. Of all included patients (n = 38), additional surgical procedures were necessary in 8 patients during the interstage period and 15 patients after BVR. Additional catheter interventions were needed in 14 patients in the interstage period and 15 after BVR. Six patients died, with no mortality in the conventional BVR group. CONCLUSIONS: Hybrid palliation as part of a staged BVR is a safe and effective initial step and promotes the growth of left ventricular structures in patients with small left-sided heart structures. Close follow-up is mandatory because extra catheter or surgical interventions are frequently needed.

Ventricular Septation of the Double-Inlet Ventricle: Over Three Decades of Follow-Up.

There is renewed interest in septation of the double-inlet ventricle as an alternative to Fontan palliation. We examined our septation experience with over 30 years of follow-up. We retrospectively reviewed patients with double-inlet ventricle from 1990 to 2011. Patients with two adequate atrioventricular valves, a volume-overloaded ventricle, and no significant subaortic obstruction were septation candidates. Of 98 double-inlet ventricle patients, 9 (9.2%) underwent attempted septation via a one-stage (n = 2, 22.2%) or two-stage (n = 7, 77.8%) approach. Ages at primary septation were 7.5 and 20.2 months. In the staged group, median age at the first and second stage was 8.3 months [range 4.1-14.7] and 22.4 months [range 11.4-195.7], respectively. There were no operative mortalities. Median follow-up was 18.8 years [range 0.4-32.9] and 30-year transplant-free survival was 77.8% ± 13.9%. Both single stage patients are alive and in sinus rhythm; 1 underwent bilateral outflow tract obstruction repair 27 years later. Of 7 patients planned for two-stage septation, there was 1 interval mortality and 1 deferred the second stage. Five patients underwent the second stage; 1 required early reintervention for a residual neo-septal defect and 1 underwent right atrioventricular valve replacement 28 years later. Three patients required a pacemaker preoperatively (n = 1) or after partial septation (n = 2). At latest follow-up, 7 patients have normal biventricular function and no significant valvulopathy. All remain NYHA functional class I. Select double-inlet ventricles may be septated with excellent long-term outcomes. Reconsideration of this strategy is warranted to avoid the sequelae of Fontan circulation.

Contemporary management of borderline left ventricle.

Borderline left ventricle cases present considerable difficulties in determining the most effective surgical approaches. The evolution of approaches in the field has shifted from classical systemic pulmonary shunts to orthodox univentricular palliation and has subsequently seen the emergence of biventricular repair concepts. The concept of biventricular repair for borderline left heart conditions has developed through studies that aim to establish predictive scoring systems for identifying appropriate candidates. Despite continuous efforts, a definitive scoring system for guiding this decision is still difficult to find. There is a growing trend to provide neonatal patients with borderline ventricles with options other than univentricular palliation. Several centres have developed personalized strategies, including hybrid and staged ventricular recruitment approaches. These strategies provide sufficient time for personalized decision-making, taking into account the individual circumstances of each patient. This article presents an overview of the changing approaches to borderline left ventricular cases. It discusses the use of predictive scoring systems and emphasizes the advancements in staged strategies that improve the likelihood of successful biventricular repairs.

Staged Ventricular Septation of the Double-Inlet Ventricle: How-I-Do-It.

Ventricular septation of the double-inlet ventricle is a largely abandoned operation due to poor historical outcomes. However, there has been renewed interest in septation as an alternative to Fontan palliation given its long-term sequelae. As one of the few centers to revisit septation in the early 1990s, our institution has long-term data on a series of patients with a double-inlet ventricle who underwent biventricular repair. This manuscript is a summary of our approach to staged septation of the double-inlet ventricle, with a focus on patient selection criteria, surgical techniques, perioperative considerations on timing of interventions, and long-term results. We believe that septation of the double-inlet ventricle should be reconsidered in patients with suitable anatomy in light of the known complications of Fontan palliation.

Intracardiac Three-Dimensional Image as Surgical Decision-Making Tool of Congenital Heart Disease.

This study aimed to evaluate the anatomical reproducibility of a preoperative intracardiac 3D image (IC image) created using computed tomography, and to investigate its usefulness as a surgical decision-making tool. Between 2012 and 2022, ventricular septal defect (VSD) patients, and double outlet right ventricle (DORV) or transposition of the great arteries (TGA) with pulmonary stenosis (PS) patients who underwent cardiac surgery and had preoperative computed tomography were enrolled. SYNAPSE VINCENT® (Fujifilm) was used to create an IC image which was analyzed retrospectively. In 14 VSD patients, the diagnostic consistency rate in the Soto classification with intraoperative findings was 100% (14/14) for IC image versus 64% (9/14) for transthoracic echocardiography (P = 0.04). The defect size showed a higher correlation coefficient with IC image (0.837, P = 0.001) than with transthoracic echocardiography (0.567, P = 0.034). In 11 DORV/TGA with PS patients, the diagnostic consistency rate in the Lev classification was 100% (9/9) for IC image versus 77% (7/9) for transthoracic echocardiography (P = 0.47). The secondary interventricular foramen (SVF)/left ventricular outflow tract (LVOT) ratio by IC image was significantly smaller in the biventricular-repair group (median 0.71, IQR 0.67-1.06) than in the univentricular-repair group (median 1.79, IQR 1.53-2.42) (P = 0.006). An IC image is useful as a surgical decision-making tool for simple VSDs and complex congenital heart diseases such as DORV or TGA with pulmonary stenosis. The SVF/LVOT ratio determined from the IC image may be a useful indicator for avoiding LVOT obstruction.

Aortic root translocation (Nikaidoh) procedure for complex transposition of the great arteries with left ventricular outflow tract obstruction.

Background: Several surgical techniques have been developed for the management of complex transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction (TGA/VSD/LVOTO). Aortic root translocation, or the Nikaidoh operation, offers the most anatomic biventricular repair in these patients. However, the Nikaidoh operation commonly has been limited to patients with "typical" anatomy, including a conoventricular VSD and usual coronary anatomy. We sought to describe a single surgeon's experience with aortic root translocation for complex TGA/VSD/LVOTO. Methods: We present a series of 12 patients with complex anatomy who underwent the Nikaidoh operation over the last 13 years. Results: We report good mid- to long-term results, excellent performance of the reconstructed left ventricular outflow tract, aortic valve competence, and no coronary insufficiency. Conclusions: Our experience suggests that the Nikaidoh operation is a valid option even for patients with complex TGA/VSD/LVOTO.

The Yasui operation: A single institutional experience over 30 years.

Objective: The Yasui operation was introduced in 1987 for patients with 2 adequate ventricles, a ventricular septal defect, and aortic atresia or interrupted aortic arch. Despite promising early outcomes, left ventricular outflow tract obstruction (LVOTO) remains a long-term concern. The purpose of this study is to report our institutional experience with the Yasui operation. Methods: We retrospectively reviewed all patients undergoing the Yasui operation between 1989 and 2021. Results are reported as median with interquartile range (IQR). Results: Twenty-five patients underwent a Yasui operation (19 primary), at 11 days (IQR, 7-218 days) of life and weight of 3 kg (IQR, 2.8-4.1 days). Fundamental diagnosis was ventricular septal defect/interrupted aortic arch in 11 patients and ventricular septal defect/aortic atresia in 14. Follow-up was 96% (24 out of 25) at 5 years (IQR, 1.4-14.7) with 92% survival. Freedom from LVOTO reoperation was 91% at late follow-up with 2 patients requiring baffle revision at 6 and 9 years. Latest echocardiogram showed 100% of patients had normal biventricular function and 87% (20 out of 23) less than mild LVOTO at 5 years (IQR, 2.3-14.9). Diagnosis, aortic valve morphology, and material used were not predictors of LVOTO. Freedom from right ventricle-to-pulmonary artery conduit reoperation was 48% at a median of 5 years (IQR, 1.4-14.7). Conduit type was not a predictor of reintervention. Conclusions: The Yasui operation can be performed with low morbidity and mortality in patients with 2 acceptable-size ventricles and aortic atresia or interrupted aortic arch with severe LVOTO. Despite some burden of reoperation, midterm reoperation for LVOTO is not common and ventricular function is preserved.

Biventricular repair using subvalvular techniques for unbalanced atrioventricular septal defect.

We report the case of a 19-month-old girl with a right-dominant unbalanced atrioventricular septal defect and severe right-sided atrioventricular valve regurgitation who underwent biventricular repair using basal chordae resection, artificial chordae reconstruction and a left-sided atrioventricular valvuloplasty. At 14-month postoperative follow-up, the patient had minimal heart failure, gained weight and adapted to biventricular circulation.

The so-called "one-and-a-half" ventricular repair: where are we after 40 years?

OBJECTIVES: The indications, timing, and results of the so-called "one-and-a-half ventricle repair", as a surgical alternative to the creation of the Fontan circulation, or high-risk biventricular repair, currently remain nebulous. We aimed to clarify these issues. METHODS: We reviewed a total of 201 investigations, assessing selection of candidates, the need for atrial septal fenestration, the fate of an unligated azygos vein and free pulmonary regurgitation, the concerns regarding reverse pulsatile flow in the superior caval vein, the growth potential and function of the subpulmonary ventricle, and the role of the superior cavopulmonary connections as an interstage procedure prior to biventricular repair, or as a salvage procedure. We also assessed subsequent eligibility for conversion to biventricular repair and long-term functional results. RESULTS: Reported operative mortalities ranged from 3% to 20%, depending on the era of surgical repair with 7% risk of complications due to a pulsatile superior caval vein, up to one-third incidence of supraventricular arrhythmias, and a small risk of anastomotic takedown of the superior cavopulmonary connection. Actuarial survival was between 80% and 90% at 10 years, with two-thirds of patients in good shape after 20 years. We found no reported instances of plastic bronchitis, protein-losing enteropathy, or hepatic cirrhosis. CONCLUSIONS: The so-called "one-and-a-half ventricular repair", which is better described as production of one-and-a-half circulations can be performed as a definitive palliative procedure with an acceptable risk similar to that of conversion to the Fontan circulation. The operation reduces the surgical risk for biventricular repair and reverses the Fontan paradox.

Fate of transposition of the great arteries with pulmonary stenosis after double-root translocation, Rastelli, and Réparation à l'Etage Ventriculaire.

OBJECTIVES: For transposition of the great arteries with unrestricted ventricular septal defect and pulmonary stenosis, double-root translocation is reported to reconstruct ideal double artery roots with growth potential. However, prospective long-term studies describing the long-term outcomes are still scarce. Therefore, the aim was to assess development of double artery roots, hemodynamics, and freedom from death and heart failure 17 years after double-root translocation, Rastelli, and Réparation à l'Etage Ventriculaire procedures. METHODS: In this prospective population-based study, 266 patients with transposition of the great arteries/ventricular septal defect/pulmonary stenosis (from July 2004 to August 2021) were consecutively included before surgery. All patients were divided into 3 groups based on the type of operation: double-root translocation (174), Rastelli (68), and Réparation à l'Etage Ventriculaire (24), who accepted postoperative evaluations annually. Generalized linear mixed model analysis was performed to determine growth potential of artery roots. RESULTS: Longitudinal repeated computed tomography measurements show the pulmonary root has significantly increased diameter (0.62 [0.03] mm/y, P < .001) over time and an adequate Z-score (-0.18) at the last follow-up only in the double-root translocation group. The pressure gradients of double outflow tracts in the double-root translocation group were the least among 3 groups. The probabilities of freedom from death/heart failure at the 15th year were 73.1%, 59.3%, and 60.9% in the double-root translocation, Rastelli, and Réparation à l'Etage Ventriculaire groups, respectively (double-root translocation vs Rastelli, P = .026; double-root translocation vs Réparation à l'Etage Ventriculaire, P = .009; Rastelli vs Réparation à l'Etage Ventriculaire, P = .449). CONCLUSIONS: By reconstructing ideal double artery roots, double-root translocation can provide postoperative long-term excellent hemodynamics and minimal death and heart failure for patients with transposition of the great arteries/ventricular septal defect/pulmonary stenosis.

Hybrid Palliation for Hypoplastic Borderline Left Ventricle: One More Chance to Biventricular Repair.

Treatment options for hypoplastic borderline left ventricle (LV) are critically dependent on the development of the LV itself and include different types of univentricular palliation or biventricular repair performed at birth. Since hybrid palliation allows deferring major surgery to 4-6 months, in borderline cases, the decision can be postponed until the LV has expressed its growth potential. We aimed to evaluate anatomic modifications of borderline LV after hybrid palliation. We retrospectively reviewed data from 45 consecutive patients with hypoplastic LV who underwent hybrid palliation at birth between 2011 and 2015. Sixteen patients (mean weight 3.15 Kg) exhibited borderline LV and were considered for potential LV growth. After 5 months, five patients underwent univentricular palliation (Group 1), eight biventricular repairs (Group 2) and three died before surgery. Echocardiograms of Groups 1 and 2 were reviewed, comparing LV structures at birth and after 5 months. Although, at birth, all LV measurements were far below the normal limits, after 5 months, LV mass in Group 2 was almost normal, while in Group 1, no growth was evident. However, aortic root diameter and long axis ratio were significantly higher in Group 2 already at birth. Hybrid palliation can be positively considered as a "bridge-to-decision" for borderline LV. Echocardiography plays a key role in monitoring the growth of borderline LV.

Staged ventricular recruitment and biventricular conversion following single-ventricle palliation in unbalanced atrioventricular canal defects.

Objective: Restoration of biventricular circulation is an alternative management strategy in unbalanced atrioventricular canal defects (uAVCDs), especially in patients with risk factors for single-ventricle palliation (SVP) failure. When ventricular volume is inadequate for biventricular circulation, recruitment procedures may accommodate its growth. In this study, we review our uAVCD experience with biventricular conversion (BIVC) after prior SVP. Methods: This is a single-institution, retrospective cohort study of uAVCD patients who underwent BIVC after SVP, with staged recruitment (staged) or primary BIVC (direct) between 2003 to 2018. Mortality, unplanned reinterventions, imaging, and catheterization data were analyzed. Results: Sixty-five patients underwent BIVC from SVP (17 stage 1, 42 bidirectional Glenn, and 6 Fontan). Decision for conversion was based on poor SVP candidacy (n = 43) or 2 adequately sized ventricles (n = 22). Of the 65 patients, 20 patients underwent recruitment before conversion. The staged group had more severe ventricular hypoplasia than the direct group, reflected in prestaging end-diastolic volume z scores (-4.0 vs -2.6; P < .01), which significantly improved after recruitment (-4.0 to -1.8; P < .01). Median follow-up time was 1.0 years. Survival and recatheterizations were similar between both groups (hazard ratio, 0.9; 95% CI, 0.2-3.7; P = .95 and hazard ratio, 1.9; 95% CI, 0.9-4.1; P = .09), but more reoperations occurred with staged approach (hazard ratio, 3.1; 95% CI, 1.3-7.1; P = .01). Conclusions: Biventricular conversion from SVP is an alternative strategy to manage uAVCD, particularly when risk factors for SVP failure are present. Severe forms of uAVCDs can be converted with staged BIVC with acceptable mortality, albeit increased reinterventions, when primary BIVC is not possible.

Biventricular repair in an infant with transposition of the great arteries and straddling of the tricuspid valve.

Biventricular repair is challenging in patients with transposition of the great arteries and straddling of the atrioventricular valves. Biventricular repair is the preferred option because of its anatomical and physiological advantages. However, in cases where biventricular repair carries operative risks that are too high or cases with unsuitable intracardiac anatomy, univentricular heart repair may have to be chosen. We report a five-month-old male patient with transposition of the great arteries, an inlet ventricular septal defect and anomalous coronary anatomy who had previously undergone a pulmonary banding operation and balloon atrial septostomy. Successful biventricular repair was performed while the patient had straddling of the tricuspid valve.

Impact of hybrid procedure on pulmonary arterial dimensions and right ventricular load after biventricular repair.

BACKGROUND: Hybrid procedure with ductal stenting and bilateral pulmonary banding offers a temporary approach in high-risk neonates with complex congenital heart defects aiming biventricular repair. This procedure may also have negative impact concerning post-banding pulmonary stenosis resulting in right ventricular pressure load. METHODS: Between 2010 and 2021 we identified 5 patients with interrupted aortic arch and complex congenital heart defect who underwent hybrid procedure and staged biventricular repair ("hybrid-group"). Other 7 cases with interrupted aortic arch were corrected in the neonatal phase without hybrid procedure ("nonhybrid-group"). Detailed intra- and extracardiac features and surgical procedures were documented as well as pulmonary interventions during follow up. Pulmonary vessel size was assessed by diameter of left and right pulmonary artery in absolute and indexed values. RV pressure was evaluated invasively via catheterization. RESULTS: Survival in cases with hybrid procedure and staged biventricular repair was 91% for a follow-up time of 40.7 months (95% CI 26-55 months) and 100% in the non-hybrid-group. Postoperative results concerning left ventricular function showed normal LV dimensions and systolic function without relevant stenosis on distal aortic arch. Hybrid procedure was associated with impaired local pulmonary arterial diameter after debanding resulting in increased right ventricular pressure and need for interventions (number intervention per patient: hybrid group 1.7 ± 0.95, non-hybrid group 0.17 ± 0.41; P 0.003). CONCLUSIONS: Hybrid procedure in high-risk cases with interrupted aortic arch and staged biventricular repair shows good postoperative results with low perioperative mortality and normal left ventricular function. Due to potential risk of relevant pulmonary stenosis and right ventricular pressure load, follow up examinations must not only focus on left but also on the right heart.

Hybrid First-stage Palliation and Other Strategies to Achieve Biventricular Repair in High-Risk Neonates With Complex Heart Anomalies and Aortic Arch Obstruction.

Medical and surgical advances have allowed single-stage total repair in neonates born with complex congenital heart anomalies and aortic arch obstruction. Nonetheless, total repair might be too complex or high risk in certain neonates with demographic, clinical or morphologic risk factors. Alternative management strategies might offer these neonates better outcomes with superior anatomic repair, shorter hospitalization, reduced morbidity, and improved survival. Alternative initial surgical strategies might include aortic arch repair and pulmonary artery band with or without cardiopulmonary bypass, extracardiac repair only and pulmonary artery band, Norwood operation, and hybrid first-stage palliation; all deferring complex biventricular intra-cardiac repair to later stage. The strategy choice should be personalized to each patient, taking into consideration the morphologic and clinical state, and the existent goals of care.

Conduction mapping during complex congenital heart surgery: Creating a predictive model of conduction anatomy.

OBJECTIVES: The study objectives were to report on a growing experience of conduction system mapping during complex congenital heart surgery and create a predictive model of conduction anatomy. METHODS: Patients undergoing complex cardiac repair with conduction mapping were studied. Intraoperative mapping used a multielectrode catheter to collect His bundle electrograms in the open, decompressed, beating heart. Patient anatomy, operative details, His bundle location, and postoperative conduction status were analyzed. By using classification and regression tree analysis, a predictive model of conduction location was created. RESULTS: A total of 109 patients underwent mapping. Median age and weight were 1.8 years (range, 0.2-14.9) and 10.8 kg (range, 3.5-50.4), respectively. Conduction was identified in 96% (105/109). Median mapping time was 6 minutes (range, 2-33). Anatomy included atrioventricular canal defect, double outlet right ventricle, complex transposition of the great arteries, and multiple ventricular septal defects. By classification and regression tree analysis, ventricular looping and visceroatrial situs were the greatest discriminators of conduction location. A total of 94 of 105 patients (89.5%) were free of complete heart block. Only 1 patient (2.9%) with heterotaxy syndrome developed complete heart block. CONCLUSIONS: The precise anatomic location of the conduction system in patients with complex congenital heart defects can be difficult for the surgeon to accurately predict. Intraoperative conduction mapping enables localization of the His bundle and adds to our understanding of the anatomic factors associated with conduction location. Predictive modeling of conduction anatomy may build on what is already known about the conduction system and help surgeons to better anticipate conduction location preoperatively and intraoperatively.

Exercise Capacity in Patients with Pulmonary Atresia with Intact Ventricular Septum: Does the Type of Surgical Repair Matter?

Two standard surgical palliative options for neonates born with pulmonary atresia and intact ventricular septum (PA/IVS) include uni-or biventricular repair. Whenever feasible, the biventricular repair is considered to have better exercise capacity (XC) and outcomes. However, there is a paucity of data comparing objective XC between these two surgical techniques. Our aim was to compare XC, including longitudinal changes in patients with PA/IVS following uni-biventricular repair. We performed a single-center retrospective study of survivors with repaired PA/IVS who underwent comprehensive treadmill cardiopulmonary exercise testing. Initial and latest exercise parameters were compared for longitudinal analysis. Demographic and exercise parameters were collated. Peak oxygen uptake (VO2 in ml/kg/min), an indicator of maximal aerobic capacity, peak heart rate, and other measures of spirometry performed at the same time were collected. Recorded parameters included, (a) Percentage of predicted VO2 (% VO2) normalized for age, weight, height, and gender, (b) % oxygen (O2) pulse, (c) anaerobic threshold (AT), (d) Chronotropic index (CI), (e) % Breathing reserve, (f) Forced vital capacity (FVC), (g) % Forced Expiratory volume in 1 s (FEV1), (h) Maximum voluntary ventilation (MVV), and (i) VE/VCO2. Appropriate statistical tests were performed, and a p value < 0.05 was considered significant. A total of 35 patients (43% male, 57% univentricular repair) were included, with a mean (SD) age of 20.1(7.5) years. Patients with univentricular palliation demonstrated significantly impaired peak heart rate, chronotropic index (0.50 ± 0.2 vs. 0.90 ± 0.1, p = 0.02), VE/VCO2 (35.4 ± 5.0 vs. 30.2 ± 2.8, p = 0.001), and %FVC (78.3 ± 8.3 vs. 88.6 ± 15.1, p = 0.02). There was a trend towards reduction in % VO2 in the Fontan patients though it was statistically similar between the groups (68.4 ± 21.4 vs. 81.2 ± 18.9, p = 0.07). Longitudinal data were available for 11 patients in each group, and there was no longitudinal decline in their exercise parameters over similar intermediate follow-up duration [6.8 (UV) vs. 5.3 (BV) years]. We conclude that young survivors with PA/IVS with prior univentricular palliation demonstrated an objective impairment in their chronotropic parameters compared with the biventricular repair. However, this did not translate into a significant difference in their exercise capacity. There was no longitudinal decline in exercise capacity or other parameters over intermediate follow-up.

Long-term results of biventricular correction for patients with double outlet right ventricle.

OBJECTIVES: The purpose of this study was to report outcomes of double outlet right ventricle biventricular repair at a single institution in developing countries and to investigate post-operatively determined risk factors for mortality and late intervention. METHODS: Patients with double outlet right ventricle who underwent biventricular repair in our centre from January 2009 to December 2021 were included. RESULTS: A total of 96 patients (male/female = 61/35) with biventricular repair were included. According to its specific anatomical type, the appropriate individual surgical plan was selected. Multivariate analysis indicated that prolonged cardiopulmonary bypass time(OR = 1.044; p = 0.012) and pre-operative moderate or above pulmonary hypertension(OR = 24.558; p = 0.023) were independent risk factors for early mortality. Univariate analysis showed that different anatomical types and different surgical methods had similar late intervention and late mortality. Concomitant coarctation of the aorta(OR = 40; p = 0.020) and concomitant ventricular septal defect enlargement(OR = 26.667; p = 0.005) were independent risk factors for late intervention by multivariate analysis. CONCLUSION: Selection of appropriate surgical techniques based on different anatomical types often results in similar late outcomes. For patients with concomitant ventricular septal defect enlargement during the operation, it is necessary to fully expand and avoid damage to the conduction bundle. We should timely intervention in patients with coarctation of the aorta and pay attention to the occurrence of left ventricular outflow tract obstruction during follow-up.