Effects of on-Table Extubation after Pediatric Cardiac Surgery.

Background: Enhanced recovery after surgery (ERAS) protocols are utilizing a multidisciplinary approach, reassessing physiology to improve clinical outcomes, reducing length of hospital stay (LOS) stay, resulting in cost reduction. Since its introduction in colorectal surgery. the concept has been utilized in various fields and benefits have been recognized also in adult cardiac surgery. However, ERAS concepts in pediatric cardiac surgery are not yet widely established. Therefore, the aim of the present study was to assess the effects of on-table extubation (OTE) after pediatric cardiac surgery compared to the standard approach of delayed extubation (DET) during intensive care treatment. Study Design and Methods: We performed a retrospective analysis of all pediatric cardiac surgery cases performed in children below the age of two years using cardiopulmonary bypass at our institution in 2021. Exclusion criteria were emergency and off pump surgeries as well as children already ventilated preoperatively. Results: OTE children were older (267.3 days vs. 126.7 days, p < 0.001), had a higher body weight (7.0 ± 1.6 kg vs. 4.9 ± 1.9 kg, p < 0.001), showed significantly reduced duration of ICU treatment (75.9 ± 56.8 h vs. 217.2 ± 211.4 h, p < 0.001) and LOS (11.1 ± 10.2 days vs. 20.1 ± 23.4 days; p = 0.001) compared to DET group. Furthermore, OTE children had significantly fewer catecholamine dependencies at 12-, 24-, 48-, and 72-h post-surgery, while DET children showed a significantly increased intrafluid shift relative to body weight (109.1 ± 82.0 mL/kg body weight vs. 63.0 ± 63.0 mL/kg body weight, p < 0.001). After propensity score matching considering age, weight, bypass duration, Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery Mortality (STATS)-Score, and the outcome variables, including duration of ICU treatment, catecholamine dependencies, and hospital LOS, findings significantly favored the OTE group. Conclusion: Our results suggest that on-table extubation after pediatric cardiac surgery is feasible and in our cohort was associated with a favorable postoperative course.

Image quality and radiation dose of dual source high pitch computed tomography in pediatric congenital heart disease.

To explore the image quality and radiation dose of dual source high-pitch cardiac computed tomography with tailored contrast injection protocols for pediatric congenital heart disease patients (CHD). In total, 27 infants with CHD (median age 109 days [IQR 6-199]) were retrospectively analyzed regarding dose length product (DLP) and effective dose (ED) after undergoing cardiothoracic CT imaging. Scan parameters were adjusted on a dual source/detector CT (DSCT) to minimize radiation dose while maintaining adequate quality. Image acquisition was performed at 70% of the R-R interval. Dose reducing measures included prospective electrocardiogram gating, utilizing slow injection velocities and foregoing bolus tracking during contrast injection. Image quality was assessed for artefacts, vessel definition, and noise on a 5-point scale (1 non-diagnostic, 5 excellent). Series were scored on a 0-to-3-point scale regarding answered clinical questions (0 non-diagnostic, 3 all clinical questions could be answered). The median DLP was 5.2 mGy*cm (IQR 3.5-7.8) leading to a median ED of 0.20 mSv (IQR 0.14-0.30). On average the acquired images scored 13.3 ± 2.1 (SD) out of a maximum 15 points with an intraclass correlation coefficient (ICC) of 0.94. All acquired series were able to fully answer all clinical questions scoring maximum points (ICC 1.0). Dual source high pitch CT protocols combined with custom contrast agent injection protocols in pediatric patients with CHD delivered sufficiently high diagnostic imaging quality combined with low submilisievert radiation doses. Prospective high pitch imaging is a reliable method for depiction of cardiac anatomy even in very young pediatric CHD patients with elevated heart rates.

Sedation versus General Anesthesia for Cardiac Catheterization in Infants: A Retrospective, Monocentric, Cohort Evaluation.

BACKGROUND: Children with congenital heart disease require repeated catheterization. Anesthetic management influences the procedure and may influence outcome; however, data and recommendations are lacking for infants. We studied the influence of sedation versus general anesthesia (GA) on adverse events during catheterization for children <2 years old. METHODS: We conducted a monocentric, retrospective study of all catheterization procedures (2008-2013). High-severity adverse event (HSAE) rates were compared using propensity-score-adjusted models, including pre- and intra-procedural variables. RESULTS: 803 cases (619 patients) (368 (46%) GA, 435 (54%) sedation) with a mean age of 6.9 ± 6.1 months were studied. The conversion rate (GA after sedation) was 18 (4%). Hospital stay was 4.9 ± 4.0 and 4.1 ± 2.5 (p = 0.01) after GA or sedation, respectively. HSAE occurred in 75 (20%) versus 40 (9%) (p < 0.01) in GA versus sedation procedures, respectively. Risk factors (multivariable analysis) were older patients (p = 0.05), smaller weights (p < 0.01), palliated status (OR 3.2 [1.2-8.9], p = 0.02), two-ventricle physiology (OR 7.3 [2.7-20.2], p < 0.01), cyanosis (OR 4.6 [2.2-9.8], p < 0.01), pulmonary hypertension (OR 5.6 [2.0-15.5], p < 0.01), interventional catheterization (OR 1.8 [1.1-3.2], p = 0.02) and procedure-type risk category 4 (OR 28.9 [1.8-455.1], p = 0.02). Sedation did not increase the events rate and decreased the requirement for hemodynamic support (OR 5.2 [2.2-12.0], p < 0.01). CONCLUSION: Sedation versus GA for cardiac catheterization in children <2 years old is safe and effective with regard to HSAE. Sedation also decreases the requirement for hemodynamic support. Paradoxical effects (older age and two-ventricle physiology) on risk have been found for this specific age cluster.

Performance of epimyocardial leads in patients with a single ventricle circulation.

BACKGROUND: Cardiac pacing can be challenging after a Fontan operation, and limited data exist regarding strategies to plan these epimyocardial systems while minimizing the number of surgical procedures. METHODS: A retrospective review of all our 47 patients (mean age 18 years, standard deviation 9 years) with a Fontan palliation who received an epimyocardial cardiac implantable electronic device (CIED) between 2002 and 2020 with regard to the stability of the epimyocardial lead parameters and the incidence of system revisions. RESULTS: Over the last 18 years, 84 implantations or revisions of the epimyocardial CIED in 47 Fontan patients were performed. Mean age at time of the first implantation was 9.4 (range 0.28-29.3) years. Follow-up period ranges from 0.11 to 18.2 (mean 7.7, standard deviation 4.2) years. A total of 123 pacing leads were implanted of which 99 are still active. From 2010 triple lead cardiac resynchronization devices were used in 17 patients to better cope with lead problems. The initial pacing threshold of the leads inactivated during this study period proved significantly higher (mean 1.66 V) than in the "all leads" group (mean 1.27 V, p = .0005) or the group of the still active leads (mean 1.17 V, p = .00004). CONCLUSIONS: When implanted with a low pacing threshold, the bipolar epimyocardial electrodes show stable and good long-term results in young patients with a Fontan circulation. Resynchronization pacing systems and the prospective implantation of reserve leads may help to reduce the rate of resternotomies and provide a flexible concept to deal with lead failure.

Aortic stenosis of the neonate: A single-center experience.

OBJECTIVES: Because data for neonates are limited, optimal management of critical aortic stenosis remains controversial (balloon valvotomy [BV] or open valvoplasty [OV]). In a center with balanced experience in both methods, we hypothesized that OV can provide a better individualized approach than blunt BV and better serve long-term outcomes. METHODS: A retrospective review of data and follow-up (survival, freedom from operation/replacement) of all neonates, suitable for biventricular repair, undergoing aortic valve procedure (1989-2015), was performed. RESULTS: One hundred three patients were concomitantly treated (BV [n = 51], OV [n = 52). Median age was 8 days, median aortic annulus Z-score was -1.3 for BV (range, -3.9 to 2.0) and OV (-3.9 to 3.2) groups. Operative mortality after BV or OV was 8% (n = 4) and 4% (n = 2), respectively. With a 13-year median follow-up, 10-year freedom from operation was 36% and 66% after BV or OV, respectively. Valve replacement was ultimately required in 32 patients (n = 20 [39%] in the BV group; n = 12 [23%] in the OV group) within a 5.9-year median time. After OV, tricuspid arrangement of the repaired aortic valve provided a 10-year freedom from operation and replacement of 87% and 95%, respectively. In multivariate analysis, associated left heart malformations, BV, nontricuspid geometry, and inadequate post procedural result were predictive of operation and replacement. CONCLUSIONS: In neonates with critical aortic stenosis, both methods (BV and OV) offer excellent survival benefit. OV significantly minimizes the need for operation, whereas BV did not postpone age of replacement. Clearly superior results are achieved with OV when a post repair tricuspid arrangement is obtained.

Contemporary results of aortic valve repair for congenital disease: lessons for management and staged strategy.

OBJECTIVES: Any aortic valve (AoV) operation in children (repair, Ross or mechanical replacement) is a palliation and reinterventions are frequent. AoV repair is a temporary solution primarily aimed at allowing the patient to grow to an age when more definitive solutions are available. We retrospectively analysed AoV repair effectiveness across the whole age spectrum of children, excluding neonates and AoV disease secondary to congenital heart disease. METHODS: From 2003 to 2015, 193 consecutive patients were included. The mean age was 9.2 ± 6.9 years (22% <1 year); 86 (45%) had a preceding balloon valvuloplasty. The indications for the procedure were stenotic (n = 123; 64%), regurgitant (n = 63; 33%) or combined (n = 7; 4%) disease. The procedures performed were commissurotomy shaving (n = 74; 38%), leaflet replacement (n = 78; 40%), leaflet extension (n = 21; 11%) and neocommissure creation (n = 21; 11%). Post-repair geometry was tricuspid in 137 (71%) patients. RESULTS: The 10-year survival rate was 97.1%. Freedom from reoperation and replacement at 7 years was, respectively, 57% (95% confidence interval, 47-66) and 68% (95% confidence interval, 59-76). In multivariate analysis, balloon dilatation before 6 months, the absence of a developed commissure, a non-tricuspid post-repair geometry and cross-clamp duration were predictors for reoperation and replacement. After a mean follow-up period of 5.1 ± 3.0 years, 145 (75%) patients had a preserved native valve, with undisturbed valve function (peak gradient <40 mmHg, regurgitation ≤mild) in 113 (58%). CONCLUSIONS: Aortic valve repair in children is safe and effective in delaying the timing for more definitive solution. Surgical strategy should be individualized according to the age of the patient. Avoidance of early balloon dilatation and aiming for a tricuspid post-repair arrangement may improve outcomes.

Promising Outcome of Anatomic Correction of Corrected Transposition of the Great Arteries.

BACKGROUND: Anatomic correction of corrected transposition of the great arteries with associated lesions, utilizing the morphologic left ventricle as a systemic pumping chamber, is the preferred method in many centers. The purpose of this study was to analyze functional outcome after anatomic correction. METHODS: Between Jan 1997 and May 2016, 63 patients with corrected transposition of the great arteries and associated lesions underwent anatomic correction. Forty-two patients (67%) underwent palliation before correction, including 14 patients (22%) who required training of systemic ventricle. The double switch procedure was performed in 37 patients; 25 patients underwent the Senning-Rastelli operation, and 1 patient underwent the Senning-Nikaidoh procedure. The median age at correction was 1.6 ± 3.7(SD) years (range, 0.2 to 17.8 years). RESULTS: The survival and freedom from any event was 95% and 71%, respectively, at 15-year follow-up. The combined freedom from death, failure of systemic ventricle, or heart transplant was 93% at 15-year follow-up regardless of procedure type. Sinus rhythm was present in 49 patients, with 14 patients requiring pacemaker (22%)-8 preoperatively, 4 early postoperatively, and 2 late postoperatively. Neurological development is normal in all patients. Fifty-four percent of the patients are not on medication. CONCLUSIONS: Anatomic correction of corrected transposition of the great arteries is a safe procedure that provides encouraging survival and functional benefits. Ninety-three percent preservation of morphological left ventricle function in 15 years of follow-up supports the concept of anatomic correction. Longer follow-up is needed to confirm superiority of this approach over other management strategies.

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.

Long-term outcomes of the arterial switch operation for transposition of the great arteries and ventricular septal defect and/or aortic arch obstruction.

OBJECTIVES: Long-term outcomes after the arterial switch operation (ASO) for complex transposition of the great arteries (TGA) should be clarified. METHODS: A retrospective study was conducted in patients operated on between 1982 and 1998. Overall 220 postoperative survivors, 79.1% with a ventricular septal defect, 13.2% with multiple ventricular septal defects, and 29.1% with aortic arch obstruction, were followed for 17 years (0-28 years). RESULTS: The conditional survival rate was 96.7% [95% confidence interval (CI): 94.4-99.1] at 25 years. Late sudden death occurred in 2 asymptomatic patients. The cumulative incidence rate of death or reinterventions was 3.8% (95% CI: 2.9-4.8) at 25 years, with age at ASO <10 days and aortic regurgitation at discharge identified as independent risk factors. The cumulative incidence rate of neoaortic regurgitation was 41.6% (95% CI: 20.5-62.8) at 25 years with an aorto-pulmonary diameter mismatch at the time of the ASO, age at ASO <10 days and aortic regurgitation at discharge identified as independent risk factors. At the last follow-up, 53 patients (24.1%) had neoaortic root dilatation with an aortic sinus z-score ≥3 and 6 of them had a Bentall operation at a median delay of 14.1 years since the ASO. The only independent factors for neoaortic root dilatation were male sex and an aorto-pulmonary diameter mismatch at the time of the ASO. CONCLUSIONS: Despite a continual rate of reinterventions, long-term survival and cardiovascular outcome are excellent after ASO for complex TGA. Dilatation of the neoaortic root and neoaortic regurgitation may be observed with time and 2 late sudden deaths occurred, justifying a close follow-up in all patients.

Subaortic Area at Risk for Development of Obstruction After Surgical Repair of Atrioventricular Septal Defect: Myth or Reality?

BACKGROUND: Morphology of the left ventricular outflow tract (LVOT) in atrioventricular septal defects (AVSDs) has been reported to be at risk for development of obstruction. The purpose of the present study was to identify the incidence, the risk factors, and the surgical outcomes of subaortic stenosis in repaired AVSDs. METHODS: Records of 427 consecutive patients who underwent anatomical repair for all types of AVSDs from January 2000 to December 2012 were reviewed. Outcomes, independent risk factors, reoperation, and death were analyzed. RESULTS: In a median follow-up of five years (range: 17 months-11.8 years), eight patients required nine reoperations for subaortic stenosis. Study group (n = 11) included three additional patients for whom repair was performed in a different institution. Median delay for reoperation was 11.9 years (range: 1.3-19.4 years). Surgical relief of subaortic stenosis was obtained by means of the enlargement of the LVOT: resection of fibrous structures in all reoperated patients and associated with septal myectomy in four. Two patients required a modified Konno procedure. Five reoperations were associated with left atrioventricular valve repair or replacement. Statistical analysis didn't reveal any morphologic or demographic risk factors. No early or late death occurred. CONCLUSION: The development of subaortic stenosis after repair of AVSD remains a rare complication in midterm follow-up (incidence: 1.9%). The performance of preventive gesture during repair seemed to be unjustified. The surgical management of this reoperation consists of a simple and safe procedure.

Saddle-Shaped Annuloplasty Improves Leaflet Coaptation in Repair for Ischemic Mitral Regurgitation.

BACKGROUND: Current repair results for ischemic mitral regurgitation (IMR) with undersized annuloplasty rings are characterized by high IMR recurrence rates. Current annuloplasty rings treat annular dilatation, but they do little to improve (and may actually exacerbate) leaflet tethering. New saddle-shaped annuloplasty rings have been shown to maintain or restore a more physiologic annular and leaflet geometry and function. Using a porcine IMR model, we sought to demonstrate the influence of annuloplasty ring shape on leaflet coaptation. METHODS: Eight weeks after posterolateral infarct, eight pigs with grade 2+ or higher IMR were randomized to undergo either a 28-mm flat ring annuloplasty (n = 4) or a 28-mm saddle-shaped ring annuloplasty (n = 4). Real-time three-dimensional echocardiography and a customized image analysis protocol allowed three-dimensional assessment of leaflet coaptation before and after annuloplasty. RESULTS: Total leaflet coaptation area was significantly higher after saddle-shaped ring annuloplasty (109.6 ± 26.9 mm(2)) compared with flat ring annuloplasty (46.2 ± 7.7 mm(2), p <0.01). After annuloplasty, total coaptation area decreased by 87.5 mm(2) (or 65%) in the flat annuloplasty group (p = 0.01), whereas total coaptation area increased by 22.2 mm(2) (or 25%) in the saddle-shaped annuloplasty group (p = 0.28). CONCLUSIONS: This study shows that the use of undersized saddle-shaped annuloplasty rings in mitral valve repair for IMR improves leaflet coaptation, whereas the use of undersized flat annuloplasty rings worsens leaflet coaptation. Because one of Carpentier's fundamental principles of mitral valve repair (durability) is to create a large surface of coaptation, saddle-shaped annuloplasty may increase repair durability.

A novel contribution towards coherent and reproducible intravalvular measurement of the aortic annulus by multidetector computed tomography ahead of transcatheter aortic valve implantation.

BACKGROUND: As current multidetector computed tomography (MDCT) measurements underestimate the size of the aortic annulus ahead of transcatheter aortic valve implantation (TAVI), a strategy of approximate annulus area oversizing has been adopted recently. AIMS: To measure the aortic annulus using a novel complementary intravalvular MDCT slice. METHODS: Fifty-five patients with severe aortic stenosis were selected for MDCT ahead of and 1 month after CoreValve(®) TAVI. Two MDCT slices were analysed and compared: the current standard virtual basal ring (VBR) at the nadir of the aortic cusps; and a novel slice, defined as the basal (lowest) complete commissural coaptation (BCCC) plane. RESULTS: BCCC is an intravalvular plane lying 5.2±0.8 mm above the VBR. The BCCC annulus is almost circular, unlike the VBR (mean eccentricity index 0.09±0.04 vs 0.3±0.1, respectively). The mean BCCC annulus diameter was 26.6±2.3 mm, 16% larger than that of the VBR (23.9±2.2 mm; P<0.001). The BCCC annulus area proved coherent with the orifice area measured after TAVI on the projection of the same slice (i.e. systematically equal to or greater than the latter [mean difference, +2.3±1.4 mm]), in contrast to the wider scatter found for the VBR (-1.3±2.0 mm). Once the sclerotic calcified valves have been pushed back by the implant, the aortic orifice after TAVI will inevitably be equal to or less than the diameter of the virtually unvalved annulus before TAVI. CONCLUSION: Based on the present results, we recommend including a BCCC slice to complete aortic annulus sizing, in order to optimize implant calibration.

Critical Congenital Heart Diseases in Preterm Neonates: Is Early Cardiac Surgery Quite Reasonable?

Prematurity is a recognized risk factor for morbidity and mortality following cardiac surgery. Postoperative and long-term outcomes after cardiac surgery performed in the preterm period are poorly described. The aim of this study was to analyze a population of preterm neonates operated on for critical congenital heart disease (CHD) before 37 weeks of gestational age (wGA) with special attention given to early and late mortality and morbidity. Between 2000 and 2013, 28 preterm neonates (median gestational age (GA) 34.3 weeks) underwent cardiopulmonary bypass (CPB) surgery for critical CHD before 37 wGA; records were retrospectively reviewed. All patients except three with single ventricle physiology had a single-stage anatomic repair. Overall mortality was 43 % (95 % CI 25-62). Risk factors for death were birth weight (p = 0.032) and weight at surgery (p = 0.037), independently of GA, preoperative status, CPB and aortic clamp time. Seven patients, including those with univentricular hearts, died during the postoperative period, and five in the first year after surgery. Median follow-up was 5.9 years (range 1 month-12.8 years). Kaplan-Meier survival rate was 75 % (95 % CI 59-91) at 1 month, and 57 % (95 % CI 39-75) at 1 and 5 years. Eight patients required reoperations after a delay of 2.8 ± 1.3 months; eight had bronchopulmonary dysplasia. At the end of follow-up, nine patients were asymptomatic. One-stage biventricular repair for critical CHD on preterm neonates was feasible. Mortality remained high but acceptable, mainly confined to the first postoperative year and related to small weight. Despite reoperations, long-term clinical status was good in most survivors. Further long-term prospective investigations are necessary to evaluate neurodevelopmental outcomes.

Repeat mitral valve repair for haemolysis in children.

BACKGROUND: Severe haemolysis is a rare complication after mitral valve repair in congenital heart disease. AIM: We describe four children with severe mitral regurgitation who underwent valve repair and subsequently developed profound haemolytic anaemia. METHODS: Clinical, echocardiographic and surgical data were collected retrospectively from a surgical centre in France during a 5-year period. RESULTS: Two patients had atrioventricular septal defects, one patient had congenital mitral dysplasia and one had anomalous left coronary artery from the pulmonary artery with mitral regurgitation. Haemolysis was diagnosed 20 to 75 days after surgery, as a result of clinical and biological examination; it was severe, and blood transfusion support was necessary in all cases. Haemolysis was always associated with eccentric mitral regurgitation with a variable degree of severity (from low to severe). After exclusion of other haemolysis aetiology, redo mitral repair surgery was performed successfully in all cases. The haemolysis was considered to be mechanical in origin, caused by regurgitation of blood through the residual mitral regurgitation and stitches. CONCLUSION: Severe haemolysis is a rare complication that can occur after mitral valve repair in congenital heart disease. All patients underwent successful redo repair and had resolution of haemolysis after surgery. Even if redo surgery is required, iterative mitral repair is possible.

Atrio-ventricular valve regurgitation in univentricular hearts: outcomes after repair†.

OBJECTIVES: The aim was to describe the early and mid-term outcome after atrio-ventricular valve (AVV) repair in patients with univentricular hearts (UVHs) and to identify risk factors for AVV reoperation and death. METHODS: This study is a retrospective review of patients undergoing valve repair for AVV regurgitation at any stage of univentricular palliation from 1998 to 2014. Patient- and procedure-related variables were analysed. RESULTS: A total of 31 consecutive patients underwent 38 procedures for ≥ moderate AVV regurgitation at a median age of 3.6 years. Thirty-two percent of patients had a common AVV, 26% had two AVVs, 22% had a dominant tricuspid valve and 19% had a dominant mitral valve. All patients underwent valve repair as a first procedure without early mortality. At discharge, patients preserved their ventricular function (fractional shortening <30%: preoperative 16% vs postoperative 22.5%, NS). In 19% (n = 6) of patients, the procedure was considered as failed because of significant residual regurgitation. There were three late deaths [median delay: 1 year (range 0.7-13.6)] and three heart transplantations. Six patients underwent seven AVV reoperations [median delay: 2 years (range 0.2-7.6)]. Longer intensive care stay (P = 0.022), longer total postoperative hospital stay (P = 0.039), higher total number of surgeries (P = 0.039), lower body mass index (P = 0.042) and higher preoperative mean pulmonary pressure (P = 0.047) were univariate risk factors for death/transplantation. Failed first AVV repair (P = 0.01), higher total number of surgeries (P = 0.026), lower body mass index (P = 0.031), male gender (P = 0.031) and need for valve repair before bidirectional cavopulmonary connection (P = 0.036) were univariate risk factors for AVV reoperation. In multivariate analysis, no univariate risk factor reached statistical significance. Freedom from death/transplantation was 84% (CI 95%: 70%-98%) at 5 and 10 years. Survival free from AVV reoperation was 72% (CI 95%: 52%-92%) at 5 years and 62% at 10 years (CI 95%: 36%-88%). Mean follow-up of survivors was 4.7 years (SD ± 4.3; range 0.2-15.6). At last visit, 96% of survivors were in NYHA Class I-II. Ninety-two percent had a ≤ mild residual regurgitation. CONCLUSIONS: In patients with a UVH and ≥ moderate AVV regurgitation, AVV repair is feasible without postoperative deterioration of their ventricular function. Nevertheless, these patients remain at increased risk for death/transplantation and AVV reoperation.

Late outcomes after arterial switch operation for Taussig-Bing anomaly.

OBJECTIVE: To assess the long-term results of the arterial switch operation (ASO) for Taussig-Bing Anomaly (TBA) and identify risk factors affecting outcomes. METHODS: Retrospective review and late follow-up was performed for all TBA patients from 1997 to 2010 (follow-up >3 years). Selection criteria included the absence of mitro-pulmonary continuity. RESULTS: Sixty-nine children underwent ASO at a median age of 24 days (interquartile range [IR] 11-125), with concomitant repair of aortic arch obstruction in 26 (37.7%). Complex coronary anatomy (n = 38; 55.0%) was common. Nine (13.0%) patients had staged repair. Hospital mortality was 5.8% (95% confidence interval [CI], 1.6%-14.2%; n = 4). Median follow-up was 11.2 years (IR 7.2-13.8). Subsequent mortality was confined to the first postoperative year (n = 5, 86% [95% CI, 78%-95%]), 1-, and 10-year survival). Overall mortality was related to coronary pattern (Yacoub types C and E vs A and D, multivariate, hazard ratio [HR] 12.2 [95% CI, 1.2-122.1], P = .03). At latest follow-up, 96% of the survivors are asymptomatic, with normal ventricular function. Cumulative incidence of reintervention at 10 years was 53% (95% CI, 28%-77%). Concomitant aortic arch obstruction was a predictor of reintervention (multivariate, HR 2.9 [95% CI, 1.1-7.4], P = .03). No mortality occurred upon reinterventions. CONCLUSIONS: In the largest series to date of ASO for TBA, mortality is confined to the first postoperative year, and related to coronary artery pattern. Beyond the first year, needed reinterventions are frequent, but with sustained functional status and no mortality over >10 years follow-up. Aortic arch obstruction is the main predictor for reintervention. Despite a significant rate of early events, favorable long-term outcomes argue for use of the ASO in TBA patients.

Aortopulmonary window and the interrupted aortic arch: midterm results with use of the single-patch technique.

BACKGROUND: An aortopulmonary window (APW) associated with an interrupted aortic arch (IAA) can be associated with significant rates of perioperative mortality and recurrent arch obstruction. We assessed the outcomes associated with the use of a single pericardial patch technique for primary repair. METHODS: Between 2002 and 2011, 9 neonates and 2 infants with APW and IAA underwent single-stage repair, under a hypothermic (28°C) continuous cardiopulmonary bypass with antegrade selective cerebral perfusion. A single autologous pericardial patch (glutaraldehyde-fixed) was used both to augment the IAA end-to-side anastomosis and to close the APW by use of the "sandwich" technique. RESULTS: The IAA was type A in 6 patients and type B in 5 patients. The APW morphology was type I in 6 patients, type II in 4 patients, and type III in 1 patient. The median age and weight at operation were 11 days (range, 6 to 180 days) and 2.6 kg (range, 2.2 to 6.5 kg), respectively. The mean cardiopulmonary bypass and aortic cross-clamp times were 108.6 ± 27.5 minutes and 49.3 ± 13.4 minutes, respectively. One patient required additional closure of a ventricular septal defect. Delayed sternal closure was performed in 8 patients. The mean follow-up time was 6 ± 3 years. There were no early and no late deaths. Postoperative morbidity consisted of one postoperative stroke with no late sequelae. There were no reoperations. The last follow-up visits confirmed the absence of recurrent aortic arch obstruction and pulmonary artery branch stenosis in all patients. CONCLUSIONS: Primary anatomic repair of APW associated with IAA can be safely performed. The efficiency of the single-patch technique was confirmed by the restoration of normal functional anatomy of the great arteries and aortic arch during follow-up.

The modified Fontan procedure with use of extracardiac conduit in adults: analysis of 32 consecutive patients.

BACKGROUND: Uncommonly, adults with functionally univentricular hearts are becoming candidates for a Fontan procedure. The purpose of this study was to evaluate the course of patients undergoing the modified Fontan procedure with an extracardiac conduit in recent years. METHODS: Between January 2003 and December 2013, 32 adult patients (17 female and 15 male) underwent total cavopulmonary connection (TCPC) with extracardiac conduit. The median age at procedure was 24.5 years (interquartile range [IQR] 20 to 33 years). The diagnoses included double-inlet left ventricle (DILV) in 10 patients (31.2%), tricuspid atresia in 8 patients (25%), double-outlet right ventricle in 4 patients (12.5%), heterotaxia in 4 patients (12.5%), and mitral atresia in 2 patients (6.2%). Seventy-eight percent of patients had undergone at least one prior palliative procedure; the most common procedures were Blalock-Taussig shunt (16 patients), superior cavopulmonary shunt (12 patients), and pulmonary artery banding (6 patients). All patients underwent cardiac catheterization preoperatively. Aortic cross-clamping was necessary in 15 patients for intracardiac procedures. Fenestration was required in 9 patients (28%). Four concomitant intraoperative cryoablation procedures were performed. RESULTS: There was no hospital mortality. One patient (3.1%) died 6 months after undergoing TCPC. Morbidities included prolonged pleural effusion lasting more than 7 days in 20 patients (62.5%), atrial arrhythmias in 4 patients (12.5%), and permanent pacemakers in 3 patients (9.3%). The median follow-up time was 33 months (interquartile range [IQR], 10.5 to 50 months). Actuarial survival was 91.83% (95% confidence limits, 71.07 to 97.89) at 1 year and 5 years. Ninety-two percent of patients were in New York Heart Association class I or II at follow-up. The median postoperative oxygen saturation was 95% (IQR, 93% to 95.5%). Cardiac arrhythmia occurred in 4 patients. Systolic ventricular function improved during follow-up for all patients except 1 patient, who underwent cardiac transplantation 7 months after the TCPC. CONCLUSIONS: The modified Fontan procedure with use of an extracardiac conduit can be performed in adults with encouraging early and midterm results. The majority of late survivors had improved quality of life. The incidence of late death, reoperation, arrhythmias, and thromboembolic events was low during follow-up.