Post-cardiotomy Rescue Extracorporeal Cardiopulmonary Resuscitation in Neonates with Single Ventricle After Intractable Cardiac Arrest: Attrition After Hospital Discharge and Predictors of Outcome.

Extracorporeal cardiopulmonary resuscitation (ECPR) in children with cardiac arrest refractory to conventional cardiopulmonary resuscitation (CPR) has been reported with encouraging results. We reviewed outcomes of neonates with functional single ventricle (FSV) surviving post-cardiotomy ECPR after hospital discharge. Fifty-eight patients who required post-cardiotomy extracorporeal membrane oxygenation (ECMO) since the introduction of our ECPR protocol (January 2007-December 2011) were identified. Forty-one were neonates. Survival analysis was conducted. Of 41 neonates receiving post-cardiotomy ECMO, 32 had FSV. Twenty-one had ECPR. Fourteen underwent Norwood operation (NO) for hypoplastic left heart syndrome (HLHS). Seven had non-HLHS FSV. Four (of 7) underwent modified NO/DKS with systemic-to-pulmonary shunt (SPS), 2 SPS only and 1 SPS with anomalous pulmonary venous connection repair. Mean age was 6.8 ± 2.1 days. ECMO median duration was 7 days [interquartile range (IQR25-75: 4-18)]. Survival to ECMO discontinuation was 72% (15 of 21 patients) and at hospital discharge 62% (13 of 21 patients). The most common cause of late attrition was cardiac. At last follow-up (median: 22 months; IQR25-75: 3-36), 47% of patients were alive. Duration of ECMO and failure of lactate clearance within 24 h from ECMO deployment determined late survival after hospital discharge (p < 0.05). Rescue post-cardiotomy ECMO support in neonates with FSV carries significant late attrition. ECMO duration and failure in lactate clearance after deployment are associated with unfavorable outcome. Emphasis on CPR quality, refinement of management directives early during ECMO and aggressive early identification of patients requiring heart transplantation might improve late survival.

Surgical Correction of Aberrant Right Coronary Anomalies Stranding an Aortic Commissure with and Without Unroofing.

The technique for successful surgical correction of an anomalous origin of the right coronary artery from the opposite aortic cusp with an aberrant course between the aorta and pulmonary artery is illustrated in a symptomatic 62-year-old woman. The intramural course of the right coronary artery traversed the tip of the commissure between the anterior and posterior leaflets, and its repair entailed unroofing of the intramural segment from inside the aortic intima. This technique required resuspension of the overlying commissure to maintain optimal aortic valve leaflet coaptation and prevent aortic insufficiency. Modifications of this technique have been utilized by us whenever the intramural segment traversed behind the commissure. In these cases, partial or subtotal unroofing of the intramural segment was performed to preserve the integrity of the intima behind the overlying commissure. More recently, we have performed the surgical correction by probing the intramural segment within the aortic wall to its most anterior location and then performing a wide anterior unroofing in the aortic intima, and marsupializing the aortic and coronary intima to avoid dissection or intimal flap development. We favor utilizing these techniques of anatomic correction of the anomalous coronary to other techniques involving coronary artery bypass grafting of the anomalous coronary, especially in adult patients, as unroofing provides more lasting results.

Transposition of Great Arteries with Complex Coronary Artery Variants: Time-Related Events Following Arterial Switch Operation.

Coronary artery anatomy represents a challenging and, often, determining predictor of outcome in an arterial switch operation (ASO). Impact of specific coronary artery variants, such as single, intramural and inverted, on time-related events following ASO, is, yet, to be determined. We sought to compare early and late outcomes within the group of nonstandard coronary artery variants. Patients who underwent ASO from January 1995 to October 2010 were reviewed. Patients with coronary artery variants other than L1Cx1R2 ("standard" by Leiden classification) were included. Patients with single, intramural and inverted coronary artery variants incorporated in group A. All other nonstandard coronary variants incorporated in group B. Demographics, perioperative variables, early and late outcomes were assessed. Of the 123 ASO, 24 patients (19.5%) with nonstandard coronary variant were studied. Thirteen were in group A and 11 in group B. There were two early deaths (1 in group A and 1 in group B) (p > 0.05). There is one death early after hospital discharge (group A). Mean follow-up was 59.4 ± 55.1 months. There was no structural coronary artery failure after hospital discharge following ASO. Freedom from any reintervention at 8 years was (78.3 ± 9.6%) (p 0.55) with no late neo-aortic or mitral valve intervention. ASO with single, intramural or inverted coronary artery course carries no added longitudinal risk for structural or flow impairment within the group of nonstandard coronary artery variants. There is an early hazard period with no late survival attrition. Aortic arch repair as part of staged strategy prior to ASO might influence early and late outcome.

Management of hypoplastic left heart syndrome with intact atrial septum: a two-centre experience.

UNLABELLED: Introduction Hypoplastic left heart syndrome with an intact atrial septum is a poor predictor of outcomes. Prenatal assessment of pulmonary venous Doppler and emergent postnatal cardiac intervention may be associated with better outcomes. Materials and methods A retrospective review of all hypoplastic left heart syndrome patients in two centres over a 5-year period was performed. Group 1 included patients with adequate inter-atrial communication. Group 2 included patients with prenatal diagnosis with an intact atrial septum who had immediate transcatheter intervention. Group 3 included patients with intact atrial septum who were not prenatally diagnosed and underwent either delayed intervention or no intervention before stage 1 palliation. Primary outcome was survival up to stage 2 palliation. RESULTS: The incidence of hypoplastic left heart syndrome with a restrictive atrial communication was 11.2% (n=19 of 170). Overall survival to stage 2 or heart transplantation was 85% and 67% for Groups 1 and 2, respectively (n=129/151, n=8/12; p=0.03), and 0% (n=0/7) for Group 3. Survival benefits were observed between Groups 2 and 3 (p<0.001). Foetal pulmonary vein Doppler reverse/forward velocity time integral ratio of ⩾18% (sensitivity, 0.99, 95% CI, 0.58-1; specificity, 0.99, 95% CI, 0.96-1) was predictive of the need for emergent left atrial decompression. CONCLUSION: Using a multidisciplinary approach and foetal pulmonary vein Doppler, time-saving measures can be instituted by delivering prenatally diagnosed neonates with hypoplastic left heart syndrome with intact atrial septum close to the cardiac catheterisation suite where left atrial decompression can be performed quickly and safely that may improve survival.

Single-center outcome analysis comparing reintervention rates of surgical arterioplasty with stenting for branch pulmonary artery stenosis in a pediatric population.

Although catheter-based intervention is generally accepted as the treatment of choice for branch pulmonary artery (PA) stenosis, there are no data directly comparing both the need for reintervention and time to reintervention in patients undergoing transcatheter stenting versus surgical arterioplasty. We compared children who underwent surgical branch pulmonary arterioplasty and branch PA stent placement between January 2008 and May 2012 at a single tertiary center. Need for reintervention and mean time to reintervention were assessed using chi-square and independent sample Student t test. Thirty-seven patients were included (surgery n = 18, stent n = 19). Mean weight at initial intervention was 11.3 ± 8.8 kg for surgical and 20.1 ± 15.5 kg for stent (p = 0.041). Intervention was performed on the left PA in 17 patients, the right PA in 12 patients, and both PAs in 8 patients. Five patients had undergone previous intervention. On mean follow-up of 807 ± 415 days, 50% (9 of 18) of the surgery cohort and 5.3% (1 of 19) of the stent cohort required reintervention (p = 0.002). In all but one case reintervention was catheter-based. Mean time to reintervention for the surgery cohort was 272 ± 162 days and for the single stent cohort it was 150 days. When comparable age and weight groups were analyzed, reintervention was still more common in the surgery cohort (p = 0.007). Children undergoing surgical branch pulmonary arterioplasty are more likely to require reintervention than those undergoing stent placement.

Fate of ventricular and valve performance following early bidirectional Glenn procedure after Norwood operation controlled for hypoplastic left heart syndome anatomic subtype.

The Norwood operation (NO) with a right ventricle (RV)-to-pulmonary artery (PA) shunt (NRVPA) is reportedly associated with early hemodynamic advantage. Shunt strategy has been implicated in ventricular function. Outcomes after NRVPA compared with classic procedure as part of a strategy involving early bidirectional Glenn (BDG) procedure were analyzed with reference to RV, tricuspid, and neoaortic valve performance. Between January 2005 and December 2010, 128 neonates with hypoplastic left heart syndrome (HLHS) underwent NO. Controlled for aortic/mitral stenosis (AS-MS) subtype, 28 patients underwent NRVPA (group A), and 26 patients had classic procedure (group B). The patients with a non-HLHS single-ventricle anatomy and those who had undergone a hybrid approach for HLHS were excluded from the study. The mean age at NO was 6.8 ± 3.5 days in group A and 6.9 ± 3.6 days in group B. Transthoracic echocardiographic evaluation (TTE) after NO (TTE-1) at the midinterval between NO and BDG (TTE-2), before BDG (TTE-3), before Fontan (TTE-4), and at the last follow-up evaluation (TTE-5) was undertaken. Cardiac catheterization was used to assess hemodynamic parameters before the Glenn and Fontan procedures. The operative, interstage, and pre-Fontan survival rates for AS-MS after NO were respectively 88.1 % (90.3 % in group A vs. 84.7 % in group B; p = 0.08), 82.5 % (82.7 % in group A vs. 81.8 % in group B; p = 0.9), and 80.7 % (79.5 % in group A vs. 81.8 % in group B; p = 0.9). The median follow-up period was 39.6 months (interquartile range 2.7-4.9 months). The RV global function, mid- and longitudinal indexed dimensions, fractionated area change before BDG (TTE-1, TTE-2, TTE-3) and after BDG (TTE-4, TTE-5), and right ventricular end-diastolic pressure did not differ statistically between the groups (p > 0.05). No statistically significant difference in tricuspid or neoaortic intervention was found between the groups (p > 0.05). Controlled for the AS-MS HLHS subtype, shunt strategy showed no midterm survival or hemodynamic (ventricular or valve) impact. At midterm, the follow-up need for neoaortic or tricuspid valve surgical intervention was not affected by shunt selection. The structural ventricular adaptation after reversal of shunt physiology was irrespective of shunt strategy.

Perventricular Melody valve implantation in a 12 kg child.

Transcatheter pulmonic valve implantation has emerged as a safe and effective alternative to valve surgery in patients with surgically corrected congenital heart disease. In cases where the transcatheter approach has failed, or was thought to be technically challenging, a hybrid approach to pulmonic valve implantation has been described. This approach involves a small subxyphoid incision made by the cardiac surgeon and implantation of the pulmonic valve through the appropriate delivery sheath through this incision. Here we describe a case of a successful implantation of a Melody valve in a 12 kg child with tetralogy of Fallot. To our knowledge this is the smallest child to have undergone this procedure reported.

Tetralogy of Fallot with atrioventricular septal defect: surgical strategies for repair and midterm outcome of pulmonary valve-sparing approach.

Repair for tetralogy of Fallot (TOF) with complete atrioventricular septal defect (CAVSD) has been reported with good early and intermediate outcomes. Morbidity, however, remains significantly high. To date, repair of CAVSD/TOF using a pulmonary valve-sparing technique (PVS) and freedom from valve reoperation are not well defined. A study was undertaken to investigate outcomes. This study was conducted in as a retrospective investigation. Between January 1988 and December 2008, 13 consecutive patients with CAVSD/TOF were identified, and their records were reviewed retrospectively. Of these 13 patients, 9 had Rastelli type C CAVSD. Trisomy 21 was present in 9 cases (69 %; 7 with type C). Five patients had received a systemic-to-pulmonary shunt (SPS) before complete repair at a mean age 1.7 ± 0.6 months. All the patients survived until complete repair. At complete CAVSD/TOF repair, AVSD was corrected with a two-patch technique in all patients. For eight patients (61.5 %), PVS was used. The remaining five patients had transannular patch (TAP) repair. The mean age at complete repair was 6.3 ± 2.4 months. At complete repair, the mean cardiopulmonary bypass time was 173.5 ± 30.6 min, and the cross-clamp time was 134.7 ± 28.8 min. There was one hospitalization and no late deaths. The median follow-up period was 9.2 years [interquartile range (IQR), 4.7-13.3 years]. The actuarial survival was 90.0 ± 9.5 % at 1 year, 90 ± 9.5 % at 5 years, and 90 ± 9.5 % at 8 years. Of the 12 survivors, 6 had some reintervention during the follow-up period. Within the first 11 years after complete repair, two patients underwent left atrioventricular (AV) valve repair, and one patient had right AV valve repair. Two patients had residual VSD closure. Four patients underwent the first right ventricular outflow tract (RVOT) reintervention for critical insufficiency or stenosis at a mean interval of 6 ± 21) months. One patient had a second RVOT reoperation. Findings showed that CAVSD/TOF with PVS was related to significantly higher freedom from RVOT reintervention (100 % at 1, 5, and 8 years compared with 80 ± 17.9 % at 1 year, 60 ± 21.9 % at 5 years, and 40 ± 21.9 % at 8 years for CAVSD/TOF using TAP; P < 0.05). No patient who underwent PVS had left ventricular outflow tract obstruction requiring reoperation. Overall freedom from any reintervention was 90.9 ± 8.6 % at 1 year, 71.6 ± 14.0 % at 5 years, and 53.7 ± 8.7 % at 8 years in this group of patients. Correction of TOF with CAVSD can be performed at low risk with favorable intermediate-term survival and satisfactory freedom from reoperation. Use of TAP can be avoided in almost two thirds of patients and may influence freedom from early RVOT reintervention.

Coronary artery-to-pulmonary artery fistula in a case of pulmonary atresia with ventricular septal defect.

Pulmonary atresia with ventricular septal defect (PA-VSD) can be associated with varying pulmonary artery connections. The origin of pulmonary blood flow can vary greatly among patients, and some case reports have described the presence of left coronary artery-to-pulmonary artery fistula. Two cases of patients found to have coronary artery-to-pulmonary artery fistula in the settings of PA-VSD are reported.

Hypoplastic left heart syndrome and aortic atresia-mitral stenosis variant: role of myocardial protection strategy and impact of ventriculo-coronary connections after stage I palliation.

Aortic atresia-mitral stenosis (AA-MS) has been implicated as a determinant of outcome after Stage-1 palliation (S1P) in hypoplastic left heart syndrome (HLHS).Studies evaluating the association of AA-MS with ventriculo-coronary connections (VCC) and mortality report conflicting results. The significance of VCC, myocardial protection, and shunt strategy after S1P has yet to be determined. Between January 2005 and July 2009, 100 neonates with HLHS underwent S1P. Mitral and aortic valves and presence of VCC were assessed. Antegrade continuous cold blood cardioplegia was administered throughout the vast extent of the neo-aortic reconstruction. A right ventricle-to-pulmonary shunt was used for an ascending aortic diameter of 0.6 mm/kg or less. Survival analysis was performed to determine predictors and assess impact of AA-MS and VCC on hospital and interstage mortality. Twenty-seven (of 100) patients had AA-MS. The mean age and weight at S1P were 6.5 ± 2.8 days and 3.09 ± 0.47 kg, respectively. VCC were found in 56% of AA-MS. Twenty-two had Norwood-Sano, 3 had classic Norwood, and 2 had hybrid S1P. VCC were associated with AA-MS, endocardial fibroelastosis, and ascending aortic size <2 mm (P < 0.05) but not higher mortality (P = ns). Operative and interstage survival for AA-MS after S1P was 85.2 and 71%, respectively (not statistically different compared to all other subtypes; P = ns). Actuarial survival after S1P at 1, 3, 6, 12, and 36 months was 92.9 ± 4.9, 78.6 ± 7.8, 75 ± 8.2, 71.3 ± 8.3, and 71.3 ± 8.3%, respectively. Intact atrial septum and post-S1P renal dysfunction (P < 0.05) were independent predictors of hospital and interstage mortality. In patients with HLHS, AA-MS carries no survival disadvantage after S1P during the hospital and interstage period regardless of VCC. Intact atrial septum and post-S1P renal dysfunction predict early and interstage mortality. Myocardial protection and shunt strategy might influence the outcome in this HLHS variant.

Post-Fontan pulmonary artery growth in patients with a bidirectional cavopulmonary shunt with additional antegrade pulsatile blood flow.

BACKGROUND: Patients with antegrade pulmonary blood flow after a bidirectional cavopulmonary shunt (Glenn) may have better pulmonary artery growth. This study evaluated pulmonary artery growth in patients with and without prior additional pulsatile antegrade flow in a Glenn shunt at midterm follow-up after a Fontan procedure. METHODS: We reviewed 212 patients who had single-ventricle palliation in a 10-year period;103 (33 in pulsatile group 1 and 70 in nonpulsatile group 2) were selected for analysis. Data on demographics, procedures, perioperative course, and midterm follow-up after the Fontan procedure were compared. Echocardiography data were collected. Pulmonary artery sizes measured at cardiac catheterization and follow-up echocardiograms were used to calculate the Nakata index. RESULTS: Perioperative details were comparable in both groups, mean pulmonary artery pressure and systemic oxygen saturations were higher in group 1 compared to group 2. Venovenous collaterals were increased in group 1. There was a significant difference in the pre-Fontan and follow-up Nakata index between groups. There was a significant increase in the Nakata index in group 1 between the pre-Glenn and pre-Fontan assessments as well as the Nakata index between the pre-Fontan and midterm follow-up. There was no significant change in the Nakata index in group 2 between assessments. CONCLUSIONS: A pulsatile Glenn shunt is associated with better pulmonary artery growth which continues long after the additional pulsatile flow is eliminated. It is possible that the effects of anterograde pulmonary blood flow on pulmonary artery growth in early life continue long after the Fontan completion.

Technical Modifications That Might Improve Long-term Outcomes of the Ross Procedure in Children.

BACKGROUND: Failure of the pulmonary valve autograft (PVA) after the Ross procedure (RP) has discouraged its widespread use and has led to modifications or alternatives to the procedure. We sought to analyze whether certain technical modifications could improve results of the RP in children. METHODS: Sixty-nine patients (median age, 12 years; range, 0.25-17.9) underwent the RP between January 1996 and December 2018. A concomitant Konno procedure was performed on 20 of 69 patients (29%). Prior interventions included balloon valvuloplasty in 30 (44%) and/or surgical valvuloplasty in 39 (57%). Technical modifications included using the native aortic root for external annuloplasty, implanting the autograft using uniplanar horizontal sutures through the aortic wall, normalizing the sinotubular junction, and wrapping the native root remnant around the PVA. RESULTS: Operative mortality was 1 of 69 patients (1.5%), with no late deaths. No patient had neoaortic valvar stenosis and 7 of 68 (10%) had mild regurgitation on discharge echocardiogram. At latest follow-up (median, 9.4 years; range, 0.4-21.3) there was no significant change in Z scores of annulus, sinus, or sinotubular junction diameters when compared with those at discharge. Three patients (4.4%) required late autograft replacement, 2 PVA repair, and 2 resection of a pseudoaneurysm. Actuarial freedom from PVA replacement was 87% at 20 years. Freedom from right ventricular outflow tract catheter reintervention or reoperation was 83% and 80%, respectively. CONCLUSIONS: Technical modifications of the RP used in this cohort might successfully prolong the life of the PVA without compromising its growth, an important advantage in pediatric patients.

Ventriculocoronary artery connections with the hypoplastic left heart: a 4-year prospective study: incidence, echocardiographic and clinical features.

Ventriculocoronary connections (VCCs), also called sinusoids, occur with hypoplastic left heart (HLH). Previous reports are limited to case reports, pathologic series, and surgical series with limited detail, which may underestimate the incidence and overestimate the severity of VCCs in HLH. A study was conducted to determine the incidence VCCs in HLH, their effect on survival, and their echocardiographic and clinical features. The echocardiograms and medical records of 100 consecutive neonatal HLH cases were analyzed. All had an aortic and a mitral valve diameter and a left ventricular (LV) volume less than Z-3. For palliation, Norwood, Sano, or hybrid procedures were used, and if the patient was alive, subsequent bidirectional Glenn and extracardiac Fontan procedures were applied. Cases were classified as manifesting mitral and aortic atresia (MAAA), mitral and aortic stenosis (MSAS), or mitral stenosis and aortic atresia (MSAA). All other diagnoses or any case with additional cardiac anomalies were excluded from the study. Overall, VCCs were found in 15% of the cases. They occurred in 56% of the MSAA subtype cases and were not statistically associated with a high mortality rate. However, in one case, large and multiple VCCs definitely caused or contributed to early death. All VCCs had a transmyocardial course, a turbulent color-Doppler flow, and a dominant usually retrograde systolic coronary artery flow pattern. The VCCs were associated (p < 0.05) with MSAA, endocardial fibroelastosis, and ascending aortic size less than 2 mm. As shown by the findings, 15% of the HLH patients had MSAA with VCCs. Unless the VCCs were large or extensive, they did not contribute to mortality. Detailed echocardiographic analysis of VCCs in HLH was feasible. Recent reports emphasize more severe cases.

Attrition in patients with single ventricle and trisomy 21: outcomes after a total cavopulmonary connection.

OBJECTIVES: Data are limited regarding the management of children with trisomy 21 (T21) syndrome and a functional single ventricle (FSV). We evaluated patients with T21 and a FSV who had a total cavopulmonary connection (TCPC). METHODS: From September 1999 to August 2012, 139 patients with a FSV underwent a TCPC. Sixty-five had unbalanced atrioventricular septal defect. Thirteen had T21. Three (of 13) had heterotaxy syndrome. The mean age at the Fontan operation was 27.6 ± 12.1 months. RESULTS: The initial procedure was pulmonary artery banding in 9 patients, systemic-to-pulmonary shunt in 2 and Damus-Kaye-Stansel/Norwood procedure in 2. Median follow-up was 69 months (interquartile range 25-75, 21-99). There was 1 death after a Damus-Kaye-Stansel/Norwood procedure and one interstage death after a bidirectional Glenn procedure. Nine (of 11) survivors underwent a Fontan operation. A fenestrated Fontan procedure was the predominate operation in 78%. One patient was deemed unsuitable for a Fontan operation. There was 1 takedown and 1 late death after the Fontan operation. Heterotaxy syndrome did not affect outcome ( P > 0.05). There was no statistical difference in the pre-Fontan McGoon ratio, hospital length of stay, duration of pleural drainage and Fontan-related adverse events between patients with a dominant right ventricle and those with a left ( P > 0.05). CONCLUSIONS: A TCPC in patients with T21 and an FSV is associated with reproducible, satisfactory outcomes. An assisted-Glenn procedure with pulsatile pulmonary blood flow and a fenestrated Fontan may be associated with attenuated perioperative morbidity and late attrition.

Relationship of Normal Aortic Valve Cusp Dimensions: A Tool to Optimize Cusp Reconstruction Valvuloplasty.

Outcomes of surgical aortic valvuloplasty hinge primarily on optimal leaflet design. Imprecise valve reconstruction can result in insufficiency or stenosis. Predicting postrepair leaflet parameters from pre- or intraoperative readily measurable valve dimensions could result in improved reconstruction. This study analyzes the relationships between different parameters of the normal aortic valve to establish a method of deriving the optimal dimensions of the reconstructed leaflet following valvuloplasty. Morphologic analysis of the normal aortic valve was performed on 50 autopsy specimens. The following parameters were measured: circumference at ventriculoaortic junction, intercommissural circumferential distance (IC), commissural height, leaflet free-margin length (L1-length of the free margin of each leaflet), cusp attachment length (L2-cusp attachment length at the ventriculoaortic junction), and leaflet height (A-ie, the vertical or cephalocaudal distance along the convex surface of the leaflet from the midpoint of the leaflet free margin to the midpoint of the cusp attachment). Univariate linear regression analysis was used to test the interdependence of leaflet dimensions. The leaflet free-margin length L1 correlated strongly with the corresponding IC: r = 0.74, 0.81, and 0.79 for noncoronary, right coronary, and left coronary leaflets, respectively; P = 0.000. Leaflet height A and cusp attachment length L2 also correlated well with the corresponding IC: A-IC, r = 0.56, 0.74, and 0.66; and L2-IC, r = 0.78, 0.85, and 0.80 for noncoronary, right coronary, and left coronary cusps respectively; P = 0.000. Parameters required to construct an aortic valve leaflet can be reliably derived from the IC, which can be easily measured perioperatively. Using this as a basis for leaflet reconstruction might optimize the leaflet graft design process, compensate for pathologic variations in aortic annulus size and commissural height, and could result in better coaptation of the valve leaflets.

Improved Results of Aortic Arch Reconstruction in the Norwood Procedure.

BACKGROUND: Despite several surgical modifications, the reported incidence of neoaortic coarctation after stage 1 palliation (S1P) of hypoplastic left heart syndrome remains high. A modification of aortic arch reconstruction that may decrease the incidence of neoaortic coarctation after S1P is described. METHODS: Between January 2008 and May 2013, 114 patients with hypoplastic left heart syndrome underwent S1P. The hospital records of the 101 surviving patients (89%) were reviewed. Demographic data and perioperative variables for S1P were collected. A modified technique for aortic arch reconstruction that contains a bovine pericardial patch tailored and sutured in a specific way was used in 51 patients (group MT), whereas the traditional technique was used in 50 patients (group TT). Clinical echocardiographic and cardiac catheterization data were analyzed to determine the incidence and location of neoaortic coarctation. RESULTS: There were no significant differences in the baseline characteristics, the intraoperative course, or hospital mortality between the two groups of subjects. Duration of ventilation, intensive care unit stay, and hospitalization were significantly shorter in group MT. The mean follow-up was 2.02 ± 0.63 years and 3.98 ± 0.66 years in each group, respectively. Coarctation developed in 4 of 51 patients (7.8%) in group MT compared with 10/50 patients (20%) in group TT. Multivariable logistic regression analysis showed that the estimated odds of coarctation for group MT were 0.41 (95% confidence interval: 0.04, 4.32) times the estimated odds of coarctation for group TT. CONCLUSIONS: The described technique may decrease the incidence of recoarctation after S1P by minimizing aortic arch and descending aorta distortion and providing ample enlargement of the aorta at its narrowest diameter.

Experience with an alternative technique for the management of anomalous left coronary artery from the pulmonary artery.

BACKGROUND: Several operative approaches are utilized for the management of anomalous origin of the left coronary artery from the pulmonary artery, each with some limitation. The long-term results of a technique that facilitates direct and tension-free implantation of the anomalous artery to the aorta in all patients are described. METHODS: From January 1, 1992 through August 30, 2000, 10 consecutive patients with anomalous left coronary artery underwent operation using this technique. It consists of isolating an anterior and posterior transverse segment of pulmonary artery in continuity with the origin of the anomalous coronary artery. The two segments are folded with the orifice of the coronary as its fulcrum, and the edges sutured together to form an extension tube of pulmonary artery tissue. This lengthens the coronary artery and allows direct aortic implantation (posterior to the pulmonary artery) without tension. The pulmonary artery is reconstructed with autologous pericardium. RESULTS: Patient age ranged from 3 weeks to 3 years old (median 8 weeks), with 80% of patients less than 11 weeks old. Median weight was 4.6 kg (3.7 to 23 kg). The left ventricle was dilated with an end-diastolic diameter z-value of +1 to +3, and the shortening fraction was markedly reduced to 16% +/- 6% (7% to 28%), with 8 of 10 patients having a shortening fraction less than 20%. Mitral regurgitation was severe in 5 patients, moderate in 2 patients, and all patients were in congestive heart failure. After repair there were no hospital deaths. Inotropic support was needed in all patients, but none required mechanical assistance. At a follow-up of 4.3 +/- 2.5 years (0.5 to 8.5 years), 9 patients are asymptomatic and 1 patient has intermittent chest pain. All patients (10/10) have echocardiographic documented patency of the reimplanted coronary artery, as well as marked improvement in the left ventricular shortening fraction (37% +/- 5%; p > 0.05 versus preoperative) and decrease in the end-diastolic diameter z-value (-1 to +1; p > 0.05 versus preoperative). Mitral regurgitation was absent in 4 patients, mild in 4 patients, and moderate in 2 patients. severe in 1 patient. Four patients have evidence of mild supravalvar pulmonary stenosis (15 to 32 mm Hg), 1992. CONCLUSIONS: This technique allows a tension-free direct aortic connection in all cases, has a low rate of coronary artery occlusion, and avoids significant pulmonary artery distortion or stenosis, making it an excellent alternative for the surgical management of anomalous origin of the coronary artery.

Pericardial tissue valves and Gore-Tex conduits as an alternative for right ventricular outflow tract replacement in children.

BACKGROUND: There is still no perfect conduit for reconstruction of the right ventricular outflow tract (RVOT) in children. Homografts are not always available in the appropriate size, and degenerate in a few years. This study evaluates the pericardial valve with Gore-Tex conduit as an alternative for RVOT construction. METHODS: From January 1, 1993, to September 30, 1999, a pericardial tissue valve was inserted in all patients undergoing RVOT reconstruction or pulmonary valve replacement (PVR) who were large enough to accommodate a tissue valve. In patients without a native main pulmonary artery, a new technique was used to construct an RV-PA conduit out of a flat sheet of Gore-Tex, as Dacron frequently leads to stenosis. Data were collected by retrospective review, follow-up echocardiograms, and assessment by a single cardiologist. RESULTS: There were 48 patients, 22 undergoing a PVR alone and 26 a RV-PA valved Gore-Tex conduit. Diagnosis included tetralogy of Fallot (n = 25); truncus arteriosis (n = 9); ventricular septal defect with PA (n = 5); DORV (n = 4); D-TGA with PS (n = 2); and 1 each IAA with sub AS, VSD with PI, and PS s/p Ross procedure. Patient age ranged from 3 to 33 years and 98% were reoperations. The valve sizes ranged from 19 to 33 mm and the median hospital length of stay was 4 days. There were 2 (4.2%) perioperative and 1 (2.1%) late deaths, none related to the valve or Gore-Tex conduit. At a follow-up of 15 to 86 months (mean 43 +/- 16 months), all remaining 45 patients are New York Heart Association class I, all valves are functional, and no patient has required valve or conduit replacement or revision; more importantly, echocardiogram revealed no significant valve or conduit stenosis (mean gradient 16 +/- 8 mm Hg) and no evidence of regurgitation or structural degeneration. CONCLUSIONS: A pericardial tissue valve and Gore-Tex conduit provides a reliable alternative for RVOT reconstruction in pediatric patients. It is readily available, molds in the limited retrosternal space, and has outstanding intermediate results with no evidence of failure or deterioration up to 7 years after insertion.

Intermediate results of the anatomic repair for congenitally corrected transposition.

BACKGROUND: Anatomic repair of congenitally corrected transposition of the great arteries has several advantages over the traditional approach but lacks long-term evaluation. METHODS: The data on 12 patients who had the procedure between January 1989 and June 2000 were retrospectively reviewed. Associated lesions included ventricular septal defect in 12 patients, pulmonary stenosis in 10 patients, and moderate to severe tricuspid valve regurgitation in 4 patients. Mean age at operation was 9+/-3.6 months. All patients had venous switch Mustard procedure. Tunneling of the morphologic left ventricle through the ventricular septal defect to the aorta with insertion of right ventricular to pulmonary artery conduit was performed in 10 patients, and arterial switch operation in 2. Concomitant tricuspid valvuloplasty was done in 2 patients and ventricular septal defect enlargement in 1. RESULTS: There was one hospital death (9%) in the patient who needed ventricular septal defect enlargement. Complications included atrioventricular block requiring pacemaker insertion in 1 patient (9%) and superior vena caval obstruction in 1 patient (9%). Follow-up is available on all patients 0.5 to 10 years (mean, 7.6+/-3.1 years). All patients are asymptomatic. Exercise test results on the three oldest patients were normal. Bradytachyarrhythmias developed in 4 patients (36%). Right ventricular to pulmonary artery conduit replacement was needed in 5 patients 2.2 to 7.1 years (mean 5.2+/-3.6 years) postoperatively. Mild to moderate tricuspid valve regurgitation persisted in 2 patients. Systemic left ventricular fractional shortening was 36% to 47% (mean, 39%+/-4.6%), and ejection fraction was 49% to 70% (mean, 60.8%+/-7.9%). CONCLUSIONS: The double switch operation can be performed safely with minimal intermediate and long-term complications.

Pericardial leaflet extension for aortic valve repair: Techniques and late results.

A modified technique to repair aortic valve is described. The technique consists of tailored extension of the old valve leaflets with gluteraldehyde treated autologous pericardium. The technique has excellent intermediate results and is an alternative to valve replacement in pediatric patients. Copyright 1999 by W.B. Saunders Company