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.

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.

A model of ischemic mitral regurgitation in pigs with three-dimensional echocardiographic assessment.

BACKGROUND AND AIM OF THE STUDY: Ischemic mitral regurgitation (IMR), the incidence of which is increasing, results from annular and subvalvular remodeling after myocardial infarction (MI). Although a sheep model of IMR has been used extensively over the past two decades, the ventricular, coronary and leaflet anatomy in sheep is significantly different from that in humans. In contrast, pigs are more similar to humans with regard to these parameters, and therefore may serve as a better animal to test emerging new technologies designed to treat IMR. METHODS: Twenty-nine pigs (body weight 30-35 kg) underwent left thoracotomy and ligation of the mid main circumflex and distal right posterior descending coronary arteries to create a posterolateral MI. Of these pigs, 18 were used for acute data acquisition, while 11 surviving animals in the chronic group were assessed at eight weeks after MI. Real-time three-dimensional echocardiography was performed at baseline, and at 30 min and eight weeks after MI, to assess geometric changes in the mitral annulus, mitral leaflets and left ventricle. RESULTS: Compared to baseline, the MR grade was increased significantly at eight weeks (0.7 + 0.5 versus 2.0 +/- 1.2), together with a significant decrease in left ventricular ejection fraction (40.3 +/- 6.6% versus 25.8 +/- 7.7%). Significant increases were also noted at eight weeks in the commissural width (30.1 +/- 3.2 mm versus 35.1 +/- 2.9 mm) and septolateral diameter (25.0 +/- 2.0 mm versus 33.8 +/- 5.9 mm), with a resultant increase in mitral annular area (596 +/- 85 versus 931 +/- 181 mm3) and a decrease in the annular height to commissural width ratio (15.7 +/- 2.6% versus 13.7 +/- 1.9%). The mitral valve tenting volume was also increased significantly (1577 +/- 645 versus 2440 +/- 755 mm3). The distance between the papillary muscle tips at baseline and at eight weeks was increased significantly (23.9 +/- 2.5 versus 30.9 +/- 5.2 mm), as was the distance between the posterior papillary muscle tip and the posterior commissure (20.9 +/- 2.7 versus 24.1 +/- 2.8 mm). CONCLUSION: The surgical model described here reliably replicates the changes seen in humans with IMR. Hence, this model can be used for further studies of the pathophysiology of IMR, and of any novel interventions in this challenging clinical area.

Three-dimensional echocardiographic analysis of mitral annular dynamics: implication for annuloplasty selection.

BACKGROUND: Proponents of flexible annuloplasty rings have hypothesized that such devices maintain annular dynamics. This hypothesis is based on the supposition that annular motion is relatively normal in patients undergoing mitral valve repair. We hypothesized that mitral annular dynamics are impaired in ischemic mitral regurgitation and myxomatous mitral regurgitation. METHODS AND RESULTS: A Philips iE33 echocardiographic module and X7-2t probe were used to acquire full-volume real-time 3-dimensional transesophageal echocardiography loops in 11 normal subjects, 11 patients with ischemic mitral regurgitation and 11 patients with myxomatous mitral regurgitation. Image analysis was performed using Tomtec Image Arena, 4D-MV Assessment, 2.1 (Munich, Germany). A midsystolic frame was selected for the initiation of annular tracking using the semiautomated program. Continuous parameters were normalized in time to provide for uniform systolic and diastolic periods. Both ischemic mitral regurgitation (9.98 ± 155 cm(2)) and myxomatous mitral regurgitation annuli (13.29 ± 3.05 cm(2)) were larger in area than normal annuli (7.95 ± 1.40 cm(2)) at midsystole. In general, ischemic mitral regurgitation annuli were less dynamic than controls. In myxomatous mitral regurgitation, annular dynamics were also markedly abnormal with the mitral annulus dilating rapidly in early systole in response to rising ventricular pressure. CONCLUSIONS: In both ischemic mitral regurgitation and myxomatous mitral regurgitation, annular dynamics and anatomy are abnormal. Flexible annuloplasty devices used in mitral valve repair are, therefore, unlikely to result in either normal annular dynamics or normal anatomy.

Development of a semi-automated method for mitral valve modeling with medial axis representation using 3D ultrasound.

PURPOSE: Precise 3D modeling of the mitral valve has the potential to improve our understanding of valve morphology, particularly in the setting of mitral regurgitation (MR). Toward this goal, the authors have developed a user-initialized algorithm for reconstructing valve geometry from transesophageal 3D ultrasound (3D US) image data. METHODS: Semi-automated image analysis was performed on transesophageal 3D US images obtained from 14 subjects with MR ranging from trace to severe. Image analysis of the mitral valve at midsystole had two stages: user-initialized segmentation and 3D deformable modeling with continuous medial representation (cm-rep). Semi-automated segmentation began with user-identification of valve location in 2D projection images generated from 3D US data. The mitral leaflets were then automatically segmented in 3D using the level set method. Second, a bileaflet deformable medial model was fitted to the binary valve segmentation by Bayesian optimization. The resulting cm-rep provided a visual reconstruction of the mitral valve, from which localized measurements of valve morphology were automatically derived. The features extracted from the fitted cm-rep included annular area, annular circumference, annular height, intercommissural width, septolateral length, total tenting volume, and percent anterior tenting volume. These measurements were compared to those obtained by expert manual tracing. Regurgitant orifice area (ROA) measurements were compared to qualitative assessments of MR severity. The accuracy of valve shape representation with cm-rep was evaluated in terms of the Dice overlap between the fitted cm-rep and its target segmentation. RESULTS: The morphological features and anatomic ROA derived from semi-automated image analysis were consistent with manual tracing of 3D US image data and with qualitative assessments of MR severity made on clinical radiology. The fitted cm-reps accurately captured valve shape and demonstrated patient-specific differences in valve morphology among subjects with varying degrees of MR severity. Minimal variation in the Dice overlap and morphological measurements was observed when different cm-rep templates were used to initialize model fitting. CONCLUSIONS: This study demonstrates the use of deformable medial modeling for semi-automated 3D reconstruction of mitral valve geometry using transesophageal 3D US. The proposed algorithm provides a parametric geometrical representation of the mitral leaflets, which can be used to evaluate valve morphology in clinical ultrasound images.

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.

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.

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.

A novel approach to in vivo mitral valve stress analysis.

Three-dimensional (3-D) echocardiography allows the generation of anatomically correct and time-resolved geometric mitral valve (MV) models. However, as imaged in vivo, the MV assumes its systolic geometric configuration only when loaded. Customarily, finite element analysis (FEA) is used to predict material stress and strain fields rendered by applying a load on an initially unloaded model. Therefore, this study endeavors to provide a framework for the application of in vivo MV geometry and FEA to MV physiology, pathophysiology, and surgical repair. We hypothesize that in vivo MV geometry can be reasonably used as a surrogate for the unloaded valve in computational (FEA) simulations, yielding reasonable and meaningful stress and strain magnitudes and distributions. Three experiments were undertaken to demonstrate that the MV leaflets are relatively nondeformed during systolic loading: 1) leaflet strain in vivo was measured using sonomicrometry in an ovine model, 2) hybrid models of normal human MVs as constructed using transesophageal real-time 3-D echocardiography (rt-3DE) were repeatedly loaded using FEA, and 3) serial rt-3DE images of normal human MVs were used to construct models at end diastole and end isovolumic contraction to detect any deformation during isovolumic contraction. The average linear strain associated with isovolumic contraction was 0.02 ± 0.01, measured in vivo with sonomicrometry. Repeated loading of the hybrid normal human MV demonstrated little change in stress or geometry: peak von Mises stress changed by <4% at all locations on the anterior and posterior leaflets. Finally, the in vivo human MV deformed minimally during isovolumic contraction, as measured by the mean absolute difference calculated over the surfaces of both leaflets between serial MV models: 0.53 ± 0.19 mm. FEA modeling of MV models derived from in vivo high-resolution truly 3-D imaging is reasonable and useful for stress prediction in MV pathologies and repairs.

Congenital native interruption of aortic arch in an adult: extra-anatomic approach by right-side thoracotomy.

We outline the case of an 18-year-old male patient with a congenital nonoperated interruption of the aortic arch. A right thoracotomy without cardiopulmonary bypass facilitated repair through an extra-anatomic bypass between the ascending and the supradiaphragmatic descending aorta. Results for the immediate and 2-year radiologic and clinical check-up were satisfactory. The most common complications in anatomic correction are stroke under selective cerebral perfusion, risk of paraplegia, and hemorrhage. We present a new technique for repair of interruption of the aortic arch in adults that avoids the need for extended dissection of the aorta and a partial occlusion clamp during anastomosis and allows for cerebral and medullar perfusion.

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.

Redo aortic root surgery for failure of an aortic homograft is a major technical challenge.

OBJECTIVE: Aortic homografts offer many advantages over prosthetic valves. However, homograft dysfunction due to degeneration or infection may lead to reoperation. Aortic valve replacement in patients who have undergone previous aortic root replacement with an aortic homograft remains a technical challenge. To assess reoperation events a retrospective review was conducted. MATERIALS AND METHODS: From January 2000 to October 2006, 20 consecutive patients (38.8+/-14.9 years old) underwent repeat surgery for aortic homograft failure. RESULTS: Reoperation was performed 7.2+/-3.5 years after implantation of the aortic homograft as a root. Indication was homograft degeneration (n=18 [90%]) and endocarditis (n=2 [10%]). In patients with major homograft wall calcifications or endocarditis, nine aortic root reconstructions were performed (Bentall procedure n=7; homograft implantation n=2). Each homograft was dissected with electrical cauterization and removed 'en-bloc' sparing the coronary buttons. In case of flexible homograft wall, stented prostheses (mechanical n=10, bioprosthesis n=1) were implanted along the homograft annulus. Additional procedures consisted of mitral valve replacements (n=8), tricuspid repairs (n=4), Konno procedure (n=1) and coronary bypass (n=5). Perioperative complications occurred in seven (35%) patients: sternal re-entry accident (n=2); reoperations for mediastinitis (n=1) or bleeding (n=2); renal insufficiency (n=1); total heart block (n=1). No association was found between operative procedures and postoperative complications (Fisher's exact test). Two patients (10%) died from multiorgan failure in the early postoperative period. In total, 94.4% of the survivors remained free from reoperation at 74 months. CONCLUSION: Reoperation on patients with an aortic homograft as a root presents a relatively high perioperative morbidity. The surgical strategy depends on the degree of homograft wall calcification.

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.

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.

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.

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.