The Impact of Bicuspid Aortic Valve Leaflet Fusion Morphology on the Ascending Aorta and on Outcomes of Aortic Valve Replacement.

BACKGROUND: Patients with bicuspid aortic valves (BAVs) tend to develop dilation of the ascending aorta. The aim of this study was to analyze the impact of leaflet fusion pattern on aortic root diameter and outcomes in patients undergoing surgery for BAV vs tricuspid aortic valve (TAV) disease. METHODS: This is a retrospective review of 90 patients with aortic valve disease (mean [SD] age, 51.5 [8.2] years) who underwent aortic valve replacement for BAV (n = 60) and TAV (n = 30). Fusion of right-left (R/L) coronary cusps was identified in 45 patients, whereas the remaining 15 patients had right-noncoronary (R/N) cusp fusion. Aortic diameter was measured at 4 levels, and Z values were computed. RESULTS: There were no significant differences between the BAV and TAV groups for age, weight, aortic insufficiency grade, or size of implanted prostheses. However, a higher preoperative peak gradient at the aortic valve was significantly associated with R/L fusion (P = .02). Preoperative Z values of ascending aorta and sinotubular junction diameter were significantly higher in patients with R/N fusion than with the R/L (P < .001 and P = .04, respectively) and TAV (P < .001 and P < .05, respectively) subgroups. During the follow-up period (mean [SD], 2.7 [1.8] years), 3 patients underwent a redo procedure. At the last follow-up, the sizes of ascending aorta were similar among all 3 patient groups. CONCLUSION: This study suggests that preoperative dilation of the ascending aorta is more common in patients with R/N fusion than in patients with R/L and TAV but is not significantly different between all groups in the early follow-up period. R/L fusion was associated with an increased risk of preoperative presence of aortic stenosis.

Right ventricular outflow tract landing zone perimeter / circularised diameter - new imaging standards in pulmonary valve replacement reporting.

BACKGROUND: Right ventricular outflow tract intervention spans transcatheter, surgical, or hybrid pulmonary valve replacement methodologies. Standardised pre-procedure workup includes cardiac MRI to identify an intended valve site (landing zone). Our institutional practice includes measurement of the right ventricular outflow tract perimeter (circumference) of this site in end-systole. Our primary aim was to compare patients by their perimeter values to the palliative interventions performed (transcatheter versus surgical/hybrid methodologies). METHODS: Retrospective review of patients undergoing pulmonary valve replacement from January 2017 to 2021. We performed perimeter measurements at the intended valve site on advanced imaging; the outcomes of interventions were outlined via descriptive and statistical analyses. RESULTS: A total of 37 patients underwent pulmonary valve replacement that met study criteria - 21 transcatheter, 7 surgical, and 9 hybrid. Median age at intervention was 26 years (range 8-70). The mean end-systolic perimeter of the transcatheter cohort was 88.9 ± 8.7 mm and in the surgical/hybrid cohort measured 106.6 ± 7.5 mm. For the transcatheter cohort, the median "circularised" diameter derived from the perimeter measurement (divided by π) was 27.7 mm (range 24.3-32.4). Notably, this correlated (r = 0.93, p < 0.01) with the median diameter of the narrowest region during actual transcatheter right ventricular outflow tract balloon sizing (lateral imaging) of 27.1 mm (range 23.2-30.1). CONCLUSIONS: Right ventricular outflow tract perimeter measurement to determine circularised diameter is useful in planning pulmonary valve replacement in terms of candidacy of transcatheter versus the need for a surgical/hybrid approach. The circularised diameter correlates with transcatheter right ventricular outflow tract balloon sizing.

Anomalous Aortic Origin of Right Coronary Artery: Outcomes of Surgical and Nonsurgical Treatment.

BACKGROUND: Anomalous aortic origin of right coronary artery (AAORCA) is a congenital heart lesion that may be associated with coronary ischemia and sudden death; however, the management of these patients remains controversial. The aim of this study was to analyze all patients with AAORCA managed at our center. METHODS: The medical records of patients with an isolated diagnosis of AAOCA were retrospectively reviewed, irrespective of symptoms, from 2007 to 2020. Follow-up was obtained by medical record review. AAORCA was diagnosed by echocardiogram and computed tomographic or magnetic resonance imaging studies in all patients. Treatment was based on anatomic, morphologic, and symptomatic features for patients older than 10 years with AAORCA. RESULTS: The review identified 86 patients with a median age of 16 years; of these, 26 (30%) were managed surgically and 60 (70%) are monitored nonsurgically. Surgical intervention included a "classic" unroofing in 10 (39%), neo-ostial creation in 7 (27%), modified unroofing with neo-ostial creation in 6 (23%), a "classic" unroofing with reimplantation in 2 (7%), and reimplantation only in 1 (4%). Surgical patients were significantly older (P = .01), described more chest pain symptoms (P = .004), had the presence of slitlike ostia (P = .03), and longer length of coronary artery narrowing (P = .0002). At follow-up (median, 3 years; range, 0-13 years), 100% of surgical patients underwent functional testing and had no evidence of ischemia. Postoperative evaluation included one or more of echocardiography, computed tomographic angiogram, magnetic resonance imaging, and exercise stress test. CONCLUSIONS: Our program uses a systematic approach for patients with AAORCA. With this paradigm, outcomes are excellent in the midterm, as validated with anatomic- and function-based testing.

Dilatable Pulmonary Artery Banding Palliation in Congenital Heart Disease.

BACKGROUND: Surgical pulmonary artery banding (PAB) has been limited in practice because of later requirement for surgical removal or adjustment. The aim of this study is to describe our experience creating a dilatable PAB via transcatheter balloon dilation (TCBD) in congenital heart disease (CHD) patients. METHODS: Retrospective chart review of adjustable PAB-outline anatomical variants palliated and patient outcomes. RESULTS: Sixteen patients underwent dilatable PAB-median age 52 days (range 4-215) and weight 3.12 kg (1.65-5.8). Seven (44%) of the patients were premature, 11 (69%) had ventricular septal defect(s) with pulmonary over-circulation, four (25%) atrioventricular septal defects, and four (25%) single ventricle physiology. Subsequent to the index procedure: five patients have undergone intracardiac complete repair, six patients remain well palliated with no additional intervention, and four single ventricles await their next palliation. One patient died from necrotizing enterocolitis (unrelated to PAB) and one patient required a pericardiocentesis postoperatively. Five patients underwent TCBD of the PAB without complication-Two had one TCBD, two had two TCBD, and another had three TCBD. The median change in saturation was 14% (complete range 6-22) and PAB diameter 1.7 mm (complete range 1.1-5.2). Median time from PAB to most recent outpatient follow-up was 868 days (interquartile range 190-1,079). CONCLUSIONS: Our institution has standardized a PAB technique that allows for transcatheter incremental increases in pulmonary blood flow over time. This methodology has proven safe and effective enough to supplant other institutional techniques of limiting pulmonary blood flow in most patients-allowing for interval growth or even serving as the definitive palliation.

Scavenging right atrial Bretschneider histidine-tryptophan-ketoglutarate cardioplegia: Impact on hyponatremia and seizures in pediatric cardiac surgery patients.

OBJECTIVE: Custodiol-HTK cardioplegia (Custodiol-HTK Koheler Chemie, GmbH, Bensheim, Germany) causes fluctuations in serum sodium levels, hyponatremia, and is associated with postoperative seizures. We investigated the influence of scavenging right atrial effluent during delivery on intraoperative serum sodium levels and postoperative seizure incidence in pediatric cardiac surgery patients. METHODS: A total of 204 patients younger than age 18 years undergoing congenital heart surgery between January 2016 and March 2018 were analyzed retrospectively. Serum sodium levels after administration of Custodiol-HTK cardioplegia were compared between the scavenge and nonscavenge groups and then in the propensity score-matched cohort (n = 96). Postoperative seizures were documented clinically and with electroencephalogram findings. Logistic regression models were used to identify the independent predictors of serum sodium level after aortic crossclamp. RESULTS: Of 204 patients, 156 (76.5%) were in the nonscavenge, and 48 (23.5%) in the scavenge groups. A serum sodium level <130 mEq/L after crossclamp and administration of Custodiol-HTK cardioplegia in the nonscavenge group were 70% versus 21% in the scavenge group (odds ratio, 8.8; 95% confidence interval, 4.1-18.3; P < .0001) in the entire cohort, and 77% versus 21% (odds ratio, 12.8; 95% confidence interval, 4.8-33.1; P < .0001) in the propensity score-matched cohort. Of 16 patients experiencing a postoperative seizure, 14 (87.5%) had a sodium level <130 mEq/L and 2 (12.5%) had a sodium level ≥130 mEq/L (odds ratio, 5.1; 95% confidence interval, 1.3-22.8; P = .021) after crossclamp. Postoperative seizures occurred in the nonscavenge group but not the scavenge group in the entire cohort (P = .02) and in the propensity score-matched patients (P = .041). Multivariable analysis of the entire cohort showed that scavenge intervention was an independent factor associated with significantly decreased risk of sodium level <130 mEq/L (odds ratio, 0.17; 95% confidence interval, 0.08-0.36; P = .000). CONCLUSIONS: Right atrial effluent scavenging was protective against fluctuations in serum sodium levels after crossclamp and Custodiol-HTK cardioplegia administration independently in both entire and matched cohort, and was also associated with decreased incidence of postoperative seizures.

Reconstruction of the Neopulmonary Root After Coronary Button Harvest for Arterial Switch Operation Using 2-ply Extracellular Matrix (Tyke): A Post-Implant Histology.

In children with Transposition of the Great Arteries (TGA), the pulmonary artery, and aorta are connected to the heart abnormally resulting in blue blood (deoxygenated) recirculating to the body and red blood (oxygenated) recirculating to the lungs. The arterial switch operation (ASO) is the standard of care for transposition of the great arteries (TGA), and given the low risk of early mortality and satisfactory long-term outcomes, focus is now on managing longer term complications such as neo-aortic root dilatation, and pulmonary artery stenosis. Since May 2016, we have used 2-ply extracellular matrix (ECM; Tyke) for reconstruction of the coronary button defects using a pantaloon patch. We present histology of implanted 2-ply ECM (Tyke) from a patient who went back to surgery for development of subaortic stenosis ~12 months after ASO.

The Destiny of Postoperative Residual Ventricular Septal Defects After Surgical Repair in Infants and Children.

PURPOSE: Residual ventricular septal defects (rVSDs) of small size are commonly seen on transesophageal echocardiography after surgical repair. This study aimed to determine the destiny of rVSD found on intraoperative echocardiogram. METHODS: Patients undergoing surgical repair of VSD as the primary procedure with available intraoperative and discharge echocardiograms between 2007 and 2017 were reviewed. Presence of an rVSD on intraoperative echo triggered review of discharge echo and of subsequent follow-up echocardiograms. RESULTS: One hundred four patients were analyzed. The mean age and weight for the entire cohort were 1.4 ± 2.9 years (median, 5.4 months; range, 29 days to 14 years) and 8.8 ± 9.9 kg (median, 5.1 kg; range, 2.7-58 kg), respectively. Sixty (57%) patients had rVSD at discharge, with mean size of residual VSD of 1.38 ± 0.92 mm (mode, 0.6; median, 2.2 mm; range, 0.5-3.9 mm). The mean follow-up time was 3.7 ± 3.1 years (range, 1 month to 9.3 years). Among those with rVSD at discharge, a residual shunt persisted in 73% at one-month follow-up. On follow-up at three years postdischarge, of the 60 patients with early rVSD, 6 had a persistent rVSD (10%) with a mean diameter of 3.0 ± 0.8 mm (range, 2.4-3.9 mm). CONCLUSIONS: Residual VSD after surgical repair is detected frequently on postoperative echocardiogram. The presence of rVSD was not associated with any preoperative, intraoperative, or postoperative factors. By three years of follow-up, only six patients continued to demonstrate rVSD with a mean diameter of 3 mm, suggesting that defects 3 mm or greater may be less likely to close spontaneously after three years.

Custodiol cardioplegia solution compared to cold blood cardioplegia in pediatric cardiac surgery: a single-institution experience.

OBJECTIVE: Custodiol is an intracellular, crystalloid cardioplegia solution that is a single-dose alternative to multi-dose cold blood cardioplegia; however, there is scarce data regarding its use in infants and children. The objective of this study was to compare its impact on myocardial function in infants. METHODS: Single-center retrospective review including 132 patients <12 months old undergoing biventricular repair. There were 106 patients who received single-dose Custodiol and 27 patients who received multi-dose blood cardioplegia. Demographic and echocardiographic data were compared between the two groups. RESULTS: Patients receiving Custodiol were slightly younger (100 ± 62 days) and lower weight (4.7 ± 1.3 kg) compared to 152 ± 86 days and 5.2 ± 1.3 kg for blood cardioplegia (p < 0.05). The Society of Thoracic Surgeons/European Association for Cardio-Thoracic Surgery Congenital Heart Surgery score was similar between both groups. Average cardiopulmonary bypass time was similar between both groups (Custodiol 93 ± 54 minutes vs. blood 81 ± 44 minutes, p = 0.46) as was aortic cross-clamp time (Custodiol 58 ± 33 minutes vs. cold blood 53 ± 33 minutes, p = 0.62). Pre-operative left ventricular ejection fraction was similar for blood 73 ± 8% versus Custodiol 70 ± 9%, p = 0.21. There was also no intergroup difference in left ventricular ejection fraction 24 hours post op (blood 64 ± 9% vs. Custodiol 65 ± 12%, p = 0.53) or at discharge (blood 66 ± 10% vs. Custodiol 66 ± 11%, p = 0.95). The pre-operative right ventricle function by fractional area change was also similar in blood cardioplegia (46 ± 13%) versus Custodiol (48 ± 9%, p = 0.38) and showed similar drops in parameters in the two groups 24 hours after surgery and at discharge. CONCLUSION: Single-dose Custodiol is as safe as blood cardioplegia for myocardial protection in congenital cardiac surgery for the cross-clamp times evaluated in this study. Evaluation at longer cross-clamp times would be helpful to determine if there is a greater benefit to single-dose Custodiol versus more repeated doses of blood cardioplegia for longer cross-clamp times.

Does the pattern of bicuspid aortic valve leaflet fusion determine the success of the Ross procedure?

BACKGROUND: In addition to being associated with aortopathy, a bicuspid aortic valve (BAV) has been posed to be a risk factor for the dilation of the pulmonary autograft in the aortic position. The aim of this study is to assess the association between the subtype of native aortic valve leaflet fusion (right and noncoronary leaflets [R/N] vs right and left leaflets [R/L]) and autograft dilation and valve dysfunction after the Ross procedure. METHODS: We performed a retrospective review of 43 patients with BAV who underwent a Ross procedure in our center from 1993 to 2013. Serial transthoracic echocardiography was used to measure changes in autograft and ascending aortic diameter over time. The aortic diameter was measured at four levels, and Z values were computed. Aortic dilation was defined as a Z value greater than 3. RESULTS: The mean age at the time of the Ross procedure was 13.5 ± 9.2 years. R/L was the most prevalent native aortic valve subtype (R/L, n = 26, 61% vs R/N, n = 17, 39%). PreRoss procedure, aortic dilation was more frequent in patients with R/N fusion (P = .02), whereas the initial aortic valve gradient and grade of aortic insufficiency (AI) did not differ between the subgroups. At follow-up, (mean = 9.6 ± 4.3 years) dilation of the autograft and ascending aorta was seen more often in patients with R/N leaflet fusion (P = .03). Conversely, the prevalence of more than moderate AI was significantly higher in patients with R/L leaflet fusion (P = .03). There was no significant difference between groups among numbers of late reintervention on the aortic valve or root (P = .75); however the type of intervention varied by morphologic subtype. Patients with R/L fusion underwent more aortic valve replacements (AVRs) while patients with R/N fusion underwent more valve-sparing aortic root replacements. CONCLUSIONS: After Ross procedure, both groups of patients were likely to have a combination of dilation of the aortic root and the tubular portion of the ascending aorta at follow-up. Patients with R/L fusion were more likely to have a prevalence of root dilation, while patients with R/N fusion were more likely to have tubular ascending aorta dilation. The R/L phenotype is associated with a slightly more rapid dilation at follow-up and is more likely to have postoperative autograft insufficiency. This information may serve to guide patient and procedure selection for AVR.

Septal Leaflet Versus Chordal Detachment in Closure of Hard-to-Expose Ventricular Septal Defects.

BACKGROUND: Different techniques have been used for exposure of ventricular septal defect (VSD) margins when there is crowding of the VSD anatomy by tricuspid valve subvalvar apparatus. This study compared surgical outcomes for the two techniques of tricuspid valve leaflet detachment and the rarely described tricuspid valve chordal detachment for hard-to-expose VSDs. METHODS: Patients undergoing transatrial VSD repair were identified from our institutional database. Follow-up echocardiography and patient data were obtained from medical records. Between January 2005 and August 2016, 130 isolated conoventricular VSDs were repaired. Among these, 26 patients underwent leaflet detachment, 15 underwent chordal detachment, and 89 underwent regular VSD repair (reference group). RESULTS: The groups did not differ significantly in age, weight, postoperative length of stay, genetic/syndromic abnormalities, time to extubation, and left and right ventricular systolic function. The cardiopulmonary bypass and cross-clamp time were significantly longer in the leaflet detachment group than in the reference group (118 ± 28 vs 102 ± 32 minutes [p = 0.02] and 73 ± 20 vs 61 ± 23 minutes [p = 0.01], respectively). Echocardiographic follow-up was available for 87 patients at a mean of 2.6 years (range, 1 month to 11 years). Tricuspid regurgitation was rated as none or trivial in 66 (76%), mild in 20 (23%), and moderate in 1 reference group patient. There was no difference in presence of residual VSD or degree of tricuspid regurgitation among the three groups. There was no reoperation for tricuspid regurgitation. CONCLUSIONS: Tricuspid valve leaflet and chordal detachment techniques provide an equally viable and safe alternative to closure of hard-to-expose VSDs while maintaining appropriate tricuspid valve function. Their use in our series did not lead to increased tricuspid valve dysfunction at early-to-midterm echocardiographic assessment.

Recurrent Laryngeal Nerve Injury and Swallowing Dysfunction in Neonatal Aortic Arch Repair.

BACKGROUND: We evaluated the incidence, clinical effect, and recovery rate of vocal cord dysfunction (VCD) and swallowing dysfunction in neonates undergoing aortic arch repair. METHODS: We retrospectively evaluated 101 neonates who underwent aortic arch reconstruction from 2008 to 2015. Direct flexible laryngoscopy was performed in 89 patients before initiation of postoperative oral feeding after Norwood (n = 63) and non-Norwood (n = 26) arch reconstruction. We defined VCD as immobility of vocal cords or their lack of coaptation and poor mobility. RESULTS: The incidence of VCD after aortic arch repair was 48% (n = 43). There was no significant difference between the VCD and non-VCD groups in postoperative length of stay, extubation failure, cardiopulmonary bypass, cross-clamp, selective cerebral perfusion time, operative death, and The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) Congenital Heart Surgery Mortality Categories. Placement of gastrostomy (p = 0.03) and documented aspiration (p = 0.01) were significantly more common in VCD patients. The incidence of VCD was 41% (n = 26) after Norwood and 65% (n = 17) after non-Norwood repairs (p = 0.06). Gastrostomy was required in 44 Norwood patients vs 9 non-Norwood patients (p = 0.004). Median length of stay was similar in Norwood patients with or without VCD (p = .28) but was significantly longer in non-Norwood patients with VCD vs those without (p = 0.002). At follow-up direct flexible laryngoscopy, VCD recovery was 74% (14 of 19) in the Norwood group and 86% (12 of 14) in the non-Norwood group. CONCLUSIONS: The incidence of VCD and swallowing dysfunction in neonates undergoing aortic arch reconstruction is high. Patients with VCD have a significantly higher incidence of gastrostomy placement and aspiration. In the Norwood population, length of stay is not associated with presence or absence of VCD. More than 70% of patients in each group who had direct flexible laryngoscopy follow-up recovered vocal cord function.

The Impact of Roller Pump vs. Centrifugal Pump on Homologous Blood Transfusion in Pediatric Cardiac Surgery.

Centrifugal pumps are considered to be less destructive to blood elements (1) when compared to roller pumps. However, their large prime volumes render them unsuitable as arterial pumps in heart lung machine (HLM) circuitry for children. In November of 2014, the circuit at Arnold Palmer Hospital, a Biomedicus BP-50 with kinetic assist venous drainage (KAVD) and 1/4″ tubing was converted to a roller pump in the arterial position with gravity drainage. Vacuum-assisted venous drainage (VAVD) was mounted on the HLM as a backup, but not used. Tubing was changed to 3/16″ in the arterial line in patients <13 kg. A retrospective study with a total of 140 patients compared patients placed on cardiopulmonary bypass (CPB) with Biomedicus centrifugal pumps and KAVD (Centrifugal Group, n = 40) to those placed on CPB with roller pumps and gravity drainage (Roller Group, n = 100). Patients requiring extra-corporeal membrane oxygenation (ECMO)/cardio-pulmonary support (CPS) or undergoing a hybrid procedure were excluded. Re-operation or circulatory arrest patients were not excluded. Prime volumes decreased by 57% from 456 ± 34 mL in the Centrifugal Group to 197 ± 34 mL in the Roller Group (p < .001). There was a corresponding increase in hematocrit (HCT) of blood primes and also on CPB. Intraoperative homologous blood transfusions also decreased 55% from 422 mL in the Centrifugal Group to 231 mL in the Roller Group (p < .001). The Society of Thoracic Surgeons--European Association for Cardio-Thoracic Surgery (STAT) categorized intubation times and hospital length of stay (LOS) for all infants showed a trend toward reduction, but was not statistically significant. Overall mortality was 5% utilizing the centrifugal configuration and 0% in the roller pump cohort. We demonstrated that the transition to roller pumps in the arterial position of the HLM considerably reduced our priming volume and formed a basis for a comprehensive blood conservation program. By maintaining higher HCTs on CPB, we were able to reduce intraoperative homologous blood transfusions.

Comparing del Nido and Conventional Cardioplegia in Infants and Neonates in Congenital Heart Surgery.

BACKGROUND: The aim of this study was to evaluate outcome measures after the use of del Nido (dN) cardioplegia compared with conventional multidose high-potassium (non-dN) cardioplegia in neonates and infants. METHODS: We retrospectively analyzed data in patients, aged younger than 1 year, undergoing cardiopulmonary bypass (CPB) from January 2012 to August 2015. We changed our cardioplegia protocol from non-dN to dN administered in a single or infrequently dosed strategy in September 2013. The outcomes of the dN group (n = 107) are compared with the non-dN group (n = 118). We analyzed variables for demographic, intraoperative, early postoperative, and discharge variables. RESULTS: The two groups were similar in age, weight, height, CPB, and cross-clamp time; preoperative and postoperative echocardiographic systolic functions; first 24-hour postoperative urine output and inotropic score; length of stay; and mortality rate. The Society of Thoracic Surgeons/European Association for Cardio-Thoracic Surgery Congenital Heart Surgery (STAT) mortality category was significantly higher in the dN group (p = 0.03). The cardioplegia dosing interval was lower for the non-dN group (p < 0.001). The volume and doses of cardioplegia per patient were significantly higher in the non-dN group (p < 0.001). In a subanalysis, when the Norwood patients were excluded from both groups, the overall STAT mortality category difference was no longer significant. The demographic, early postoperative, and discharge variables still showed no significant difference when the two groups were compared. CONCLUSIONS: Similar outcomes can be achieved with less frequent interruption of the operation and lower volume of cardioplegia when using dN cardioplegia solution compared with conventional cardioplegia. The dN cardioplegia with extended ischemic interval can be used as an alternative strategy in the neonatal and infant population during cardiac operations.

Performance of SynerGraft Decellularized Pulmonary Allografts Compared With Standard Cryopreserved Allografts: Results From Multiinstitutional Data.

BACKGROUND: Structural deterioration of allografts over time is believed to be at least partly related to an immune response mounted against human leukocyte antigen specific to the transplanted tissue. SynerGraft (SG) processing (CryoLife, Kennesaw, GA) is a technology that decellularizes an allograft leaving only connective tissue, therefore, reducing immunogenicity and potentially increasing durability of the implant. METHODS: We performed a retrospective review of 163 SG patients and 124 standard allograft controls from 3 medical centers. Patient demographics were tabulated, and conduit stenosis and insufficiency were measured by echocardiography. RESULTS: There were 28 deaths (15 of 163 [9%] SG patients vs 13 of 124 [11%] standard patients; p = 0.72), but no deaths were attributed to structural failure of the conduit. The actuarial survival for SG vs standard cohorts was not different at 5 and 10 years. Among the 274 hospital survivors, 17% SG vs 42% standard had evidence for significant conduit dysfunction at the most recent follow-up or before conduit replacement. Freedom from conduit dysfunction was significantly worse at 10 years in the standard group (58%) than in the SG group (83%, p < 0.001). CONCLUSIONS: This study represents a multiinstitutional retrospective comparison of SG and standard cryopreserved allografts used in right ventricular outflow tract reconstruction in a broad range of patient ages. Our results demonstrate that at an intermediate-term to long-term follow-up, conduit dysfunction and pulmonary insufficiency and stenosis are higher among patients receiving standard allografts. We postulate that the improved durability of SG is related to decreased immunogenicity of the SG technology.

The Association Between Aortic Valve Leaflet Morphology and Patterns of Aortic Dilation in Patients With Bicuspid Aortic Valves.

BACKGROUND: Dilation of the aorta is a frequent complication in patients with bicuspid aortic valves. The aim of this study was to determine the relationship between the subtype of leaflet fusion, right and noncoronary leaflet (R/N) fusion versus right and left leaflet (R/L) fusion, and the patterns of aortic dilation and valve dysfunction in young patients with bicuspid aortic valves. METHODS: We performed a retrospective review of 642 patients who presented with bicuspid aortic valves between 1994 and 2014. Of these patients, 210 (33%) had aortic dilation (z score >3) by echocardiogram. For each patient, the most recent study or the last study before intervention was reviewed. RESULTS: Median patient age was 15 years (range, 0 to 40 years) with patients with R/N fusion being younger. The most prevalent subtype was R/N fusion (R/N, n = 114, 54% versus R/L, n = 96, 46%). Dilation of the ascending aorta was seen more often in patients with R/N fusion (R/N, 88% versus R/L, 68%; p = 0.004), whereas the prevalence of dilation of the sinuses of Valsalva was significantly higher in patients with R/L fusion (R/L, 46% versus R/N, 20%; p = 0.01). The magnitude of dilation differed as well. The z value of the sinuses of Valsalva was significantly higher in patients with R/L fusion (R/L, 2.03 versus R/N, 1.2; p = 0.003), whereas the z values of the ascending aorta and sinotubular junction were similar between the groups. Patients with R/N fusion were more likely to have aortic stenosis, and within the R/N group, patients with aortic insufficiency had a greater degree of ascending aorta dilation (p = 0.04). CONCLUSIONS: Our study suggests that in young patients with bicuspid aortic valves and aortic dilation, aortic valve morphology may be associated with the patterns of aortic dilation and valve dysfunction. Patients with R/N fusion were more likely to have ascending aorta dilation, whereas patients with R/L fusion were more likely to have dilation of the aortic root. In addition, patients with R/N fusion presented at a younger age and were more likely to have aortic stenosis. Recognition of these differences may eventually be helpful for patient counseling and the planning of follow-up.

Does the Homograft for RVOT Reconstruction in Ross: Patients Fare Better than for Non-Ross Patients? A Single-Center Experience.

BACKGROUND AND AIM OF THE STUDY: In patients undergoing the Ross procedure, the right ventricular outflow tract (RVOT) conduit is inserted in an orthotopic position rather than in the more heterotopic position used in the repair of complex congenital RVOT obstruction. The study aim was to compare the authors' institutional mid-term experience of large-sized homografts (>19 mm) in patients with Ross and non-Ross RVOT reconstructions. METHODS: The outcome was reviewed of all homografts implanted for Ross (n = 72) or non-Ross (n = 64) RVOT reconstruction at a single center between 1993 and 2012. Echocardiographic data were reviewed to evaluate valve performance. Homograft dysfunction was defined as RVOT obstruction with peak echo-Doppler gradient >40 mmHg and/or grade >III/IV conduit valve regurgitation. Homograft failure was defined as the need for conduit replacement or catheter or surgical reintervention. RESULTS: The age, body weight, conduit diameter and previous surgery were significantly higher in patients with Ross compared to the non-Ross group (p = 0.002, 0.003 and <0.001, respectively). The mean follow up duration was similar in both groups. There were 35 re-interventions (Ross, n = 17; non-Ross, n = 18). The data acquired showed actuarial survival, freedom from conduit dysfunction and conduit failure to be similar in both cohorts. Freedom from any type of reoperation was worse for the Ross group (58%) than for the non-Ross group (72%) (p = 0.05). CONCLUSION: During the first 15 years after Ross or non-Ross pulmonary homograft implantation, the survival rate, freedom from failure and dysfunction, and RVOT gradient were statistically similar. Freedom from any type of reoperation was significantly higher in the non-Ross group, however.

Outcomes of tricuspid valve repair in children with hypoplastic left heart syndrome.

OBJECTIVES: Tricuspid valve regurgitation (TR) is a common finding in children with hypoplastic left heart syndrome (HLHS) undergoing staged surgical reconstruction and can result from either abnormal valve morphology or incomplete leaflet coaptation due to annular dilatation. The purpose of this study was to determine the incidence of severe TR and to evaluate the effect of surgically treated tricuspid valve (TV) disease on overall survival. METHODS: Between December 1988 and December 2012, 86 patients with HLHS underwent a Norwood procedure (mean age/weight, 11.1 days/3.5 kg). Thirty-five percent had associated congenital cardiac, structural, and genetic anomalies. Subsequently, 65 patients underwent a bidirectional Glenn procedure or a hemi-Fontan (stage II) and 42 patients a modified Fontan procedure (stage III). The mean follow-up was 6.6 years. All patients were evaluated preoperatively and postoperatively by Doppler echocardiography to determine the degree of TR. RESULTS: At the time of discharge following the Norwood procedure, 64% (n = 47) of the patients had none/trivial, 32% (n = 24) had mild, 3% had moderate (n = 2), and 1% (n = 1) had severe TR on their post-Norwood Doppler echocardiograms. Among 74 Norwood survivors, 11 children (15%) with severe TR underwent TV repair at a median interval after the Norwood procedure of nine months (range, 2-70). There were one surgical and one late death, giving an overall mortality of 18%. The overall survival rate between TR and non-TR repair groups was not significantly different (p = 0.18). Four patients (36%) underwent reoperation for persistent TR. CONCLUSIONS: Severe TR as a risk factor for mortality in children undergoing palliative procedures for HLHS can be diminished by successful TV procedures. Survival at intermediate follow-up of patients with successful TV procedures that eliminate severe TR and patients without a history of severe TR are similar. Further follow-up is required to determine the impact of surgically treated TR on long-term survival and the durability of surgical techniques.

Failed autograft after the ross procedure in children: management and outcome.

BACKGROUND: Autograft dilatation (AD) and aortic insufficiency (AI) after the Ross procedure are the most common causes of late autograft failure. The purpose of this study was to examine the results of valve-sparing root replacement (modified David) and composite root replacement. METHODS: We performed a retrospective review of all children (n=78) undergoing a Ross procedure at our Center from 1993 to 2011. RESULTS: Median follow-up was 10 years (1to 18 years). Freedom from autograft reoperation was 94% at 5 years, and 65% at 15 years. Freedom from greater than 2+ autograft AI was 93% at 5 years and 76% at 15 years. Autograft reoperation was necessary in 22 patients, at a median interval of 8.7 years after the original procedure. Indications for reoperation were AI with autograft dilatation in 15 patients, AI without dilatation in 2 patients, and AD without AI in 5 patients. Surgical procedures used at reoperation included valve-sparing root replacement in 14 patients, root replacement either mechanical or biologic valved conduit in 6 patients, and valve replacement in 2 patients. At a mean follow-up of 5.8 years after reoperation, 4 patients from the valve-sparing group underwent second reoperation (valve replacement). Freedom from second autograft reoperation was 71% for patients after a valve sparing procedure and 100% for patients after an aortic valve or root replacement (Bentall procedure) at 5 years. CONCLUSIONS: Autograft valve-sparing root replacement and composite aortic root replacement are effective treatments for aortic root dilation and AI after the Ross procedure. The potential of late autograft insufficiency after valve-sparing root replacement warrants annual follow-up.

Outcomes of the modified norwood procedure: hypoplastic left heart syndrome versus other single-ventricle malformations.

Recent studies have suggested that survival after the modified Norwood procedure is influenced by anatomy and is worse for patients with hypoplastic left heart syndrome (HLHS) than for patients with other forms of functional single ventricle. This study aimed to compare the outcomes of the modified Norwood procedure for these two groups at a single center. A retrospective chart review compared modified Norwood procedure outcomes between children with HLHS (n = 40) and children with other single-ventricle malformations (n = 19) from January 2002 to August 2012. The HLHS patients had a lower mean age (10.0 ± 13.2 days) at the time of operation than the non-HLHS patients (16.5 ± 18.5 days). The difference was not significant. The number of patients with a Sano modification was significantly higher in the HLHS group (HLHS 85 % vs non-HLHS 32 %; p < 0.001). The follow-up data did not differ between the groups (HLHS 3.6 ± 3.2 years vs non-HLHS 4.8 ± 3.3 years, nonsignificant difference). The overall operative survival was 85 %: 83 % for the patients with HLHS and 89 % for the patients with non-HLHS (nonsignificant difference). The Kaplan-Meier transplant-free survival estimate at 10 years was 73 % and did not differ between the HLHS patients (67 %) and the non-HLHS patients (84 %, nonsignificant difference). The timing of stages 2 and 3 palliation (bidirectional Glenn and external conduit Fontan) did not differ significantly (HLHS, 7.0 ± 2.8 months and 3.0 ± 0.5 years vs non-HLHS 6.1 ± 1.7 months and 2.8 ± 0.8 years). The two groups were similar with respect to the need for post-Norwood extracorporeal membrane oxygenation support, aortic arch reinterventions, atrioventricular valve repair, pulmonary artery reconstruction, pacemaker implantation, and progression to bidirectional Glenn and Fontan completion. The modified Norwood reconstruction provides good palliation for HLHS as well as other forms of functional single-ventricle anatomy. Survival, postoperative morbidity, and progression to Glenn and Fontan are not significantly different.

Outcomes of double inlet left ventricle and similar morphologies: a single center comparison of initial pulmonary artery banding versus a norwood-type reconstruction.

OBJECTIVE: Patients with double inlet left ventricle (DILV)/transposition and similar morphologies have their systemic outflow traverse a bulboventricular foramen (BVF), which has a propensity to narrow over time. The aim of this study is to evaluate the outcomes of initial pulmonary artery banding (PAB) compared with the Norwood-type reconstruction in neonates. METHODS: A retrospective study of children with DILV and similar morphologies presenting between 1982 and 2012. The outcomes of initial PAB (n=17) are compared with a Norwood-type reconstruction (n=20). The two groups were similar with respect to age, gender, weight, noncardiac syndromes, and Fontan completion. Mean follow-up was longer in PAB patients (13.3 ± 9.8 years) versus Norwood (4.5 ± 3.0 years, p=0.001). RESULTS: Survival was 75% at eight years in the Norwood group versus 71% in the PAB group (p=0.76). Mortality in the Norwood group was higher before 2002 (p=0.06). The age of patients who underwent a bidirectional Glenn shunt was significantly higher for PAB group (PAB, 9.1 ± 1.4 months vs. Norwood, 6.1 ± 1.6 months; p<0.001). Freedom from any type of reintervention (systemic outflow obstruction or coarctation) was similar (Norwood, 83% vs. PAB, 71%; p=0.62). Freedom from heart block with a pacemaker insertion was significantly better for Norwood patients (Norwood, 89% vs. PAB, 41%; p=0.001). CONCLUSIONS: The Norwood-type reconstruction provides good palliation in this subgroup of patients and avoids the need for subsequent intracardiac operations, maintaining an unobstructed systemic outflow tract and avoiding the risk of heart block. Survival does not differ depending on the type of procedure. Patients with PAB show comparable satisfactory early and long-term results, with an increasing reoperation risk and heart block remaining a major concern.