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Background: Aortic cusp extension is a technique for aortic valve (AV) repairs in pediatric patients. The choice of the material used in this procedure may influence the time before reoperation is required. We aimed to assess post-operative and long-term outcomes of patients receiving either pericardial or synthetic repairs. Methods: We conducted a single center, retrospective study of pediatric patients undergoing aortic cusp extension valvuloplasty (N=38) with either autologous pericardium (n=30) or CorMatrix (n=8) between April 2009 and July 2016. Short and long-term postoperative outcomes were compared between the two groups. Freedom from reoperation was compared using Kaplan Meier analysis. Degree of aortic stenosis (AS) and aortic regurgitation (AR) were recorded at baseline, post-operatively, and at outpatient follow-up. Results: At five years after repair, freedom from reoperation was significantly lower in the CorMatrix group (12.5%) compared to the pericardium group (62.5%) (P = 0.01). For the entire cohort, there was a statistically significant decrease in the peak trans-valvar gradient between pre- and post-operative assessments with no significant change at outpatient follow-up. In the pericardium group, 28 (93%) had moderate to severe AR at baseline which improved to 11 (37%) post-operatively and increased to 21 (70%) at time of follow-up. In the biomaterial group, 8 (100%) had moderate to severe AR which improved to 3 (38%) post-operatively and increased to 7 (88%) at time of follow-up. Conclusion: In terms of durability, the traditional autologous pericardium may outperform the new CorMatrix for AV repairs using the cusp extension method.

Bioprosthetic aortic valve replacement (bAVR) in patients with congenital heart disease is challenging due to age, size and complexity. Our objective was to assess survival and identify predictors of re-operation. Data were retrospectively collected for 314 patients undergoing bAVR at 8 centers from 2000-2014. Kaplan-Meier estimation of time to re-operation and Cox regression were utilized. Average age was 45.2 years (IQR 17.8-71.1) and 30% were

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.

Many instances of coronary artery anomalies are documented in the literature; however, the detection and treatment of an asymmetric, large aortic cusp causing obstruction of a coronary ostium in a symptomatic infant remains unreported. We present a case of a 2-month-old infant with an enlarged right coronary cusp obstructing the left coronary ostium, requiring emergent repair by relocating the left coronary button and reconstructing the sinus of Valsalva with autologous pericardium. This procedure preserved native aortic valve function, and the child remains asymptomatic months after discharge

Loeys–Dietz syndrome is a connective tissue disorder known to cause aggressive aortopathy in paediatric patients, but it is extremely rare for cardiovascular events to present during infancy. We report the first successful aortic repair in a neonate with LDS presenting in extremis with an early onset, massive aortic aneurysm.

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.

Infants are a unique transplant population due to a suspected immunologic advantage, in addition to differences in size and physiology. Consequently, we expect infants to have significantly different diagnoses, comorbidities, and outcomes than pediatric transplant recipients. In this study, we compare patterns and trends in pediatric and infant heart transplantation during three decades. The United Network for Organ Sharing (UNOS) database was queried for transplants occurring between January 1990 and December 2018. Patients were categorized as pediatric (1-17) or infant (0-1). Congenital heart disease (CHD) primary diagnoses have increased from 37% to 42% in pediatric patients (p = 0.001) and decreased from 80% to 61% in infants during the 1990s and 2010s (p < 0.001). Those with CHD had worse outcomes in both age groups (p < 0.001). Infants who underwent ABO-incompatible transplants had similar survival as compared to those with compatible transplants (p = 0.18). Overall, infants had better long-term survival and long-term graft survival than pediatric patients; however, they had worse short-term survival (p < 0.001). Death due to rejection or graft failure was less likely in infants (p = 0.034). However, death from infection was over twice as common (p < 0.001). In summary, pediatric and infant heart transplant recipients differ in diagnoses, comorbidities, and outcomes, necessitating different care for these populations.

At present, aortic valvuloplasty is considered an effective procedure for treatment of aortic regurgitation in pediatric patients. It has encouraging mid- and long-term results. The improved outcome is primarily related to better understanding of the functional anatomy of the normal valve and the different factors that alter it. It is also related to the realization that outcome after valvuloplasty is dependent on comprehensive repair of all of the involved components of the aortic root. Refinement in preoperative diagnosis has helped identify these abnormal components and focus the surgical approach on the needed reconstruction. Although the technical aspects of valvuloplasty are well defined, suboptimal long-term results still occur in some cases because the patch material used for valve repair can become fibrotic or calcified. This review summarizes the surgical approach to and the management of the different abnormal root components in pediatric patients with significant aortic valve regurgitation.

ObjectiveTo evaluate differences in interstage growth of pulmonary arteries between use of polytetrafluoroethylene and femoral vein homograft as Sano shunt during stage-I Norwood palliation.MethodsA retrospective review of all patients who survived to the second stage following Norwood–Sano operation at two institutions was performed. Either polytetrafluoroethylene or the valved segment of femoral vein homograft was used for construction of the Sano shunt. The size of pulmonary arteries was compared at pre-Glenn catheterisation.ResultsA total of 48 neonates with the diagnosis of hypoplastic left heart syndrome or its variants comprised the study population. Femoral vein homograft of 5–6 mm diameter was used in 14 and polytetrafluoroethylene graft of 5 mm was used in 34 patients. The two groups were comparable in terms of preoperative demographics and age at time of pre-Glenn catheterisation (3.9±0.7 versus 3.4±0.8 months, p=0.06). Patients who received femoral vein homograft demonstrated a significantly higher pre-Glenn Nakata index [264 (130–460) versus 165 (108–234) mm2/m2, p=0.004]. The individual branch pulmonary arteries were significantly larger in the femoral vein group (right, 7.8±3.6 versus 5.0±1.2, p=0.014; left, 7.2±2.1 versus 5.6±1.9, p=0.02). There were no differences in cardiac index, Qp:Qs, ventricular end-diastolic pressure or systemic oxygen saturations.ConclusionsUtilisation of a valved segment of femoral vein homograft as right ventricle to pulmonary artery conduit during Norwood–Sano operation confers better interstage growth of the pulmonary arteries. Further studies are needed to evaluate the impact of femoral vein homograft on single ventricle function.

Congenitally corrected transposition of the great arteries (ccTGA) can occur in isolation, or in combination with other structural cardiac anomalies, most commonly ventricular septal defect, pulmonary stenosis and tricuspid valve disease. Clinical recognition can be challenging, so echocardiography is often the means by which definitive diagnosis is made. The tricuspid valve and right ventricle are on the systemic arterial side of the ccTGA circulation, and are therefore subject to progressive functional deterioration. The natural history of ccTGA is also greatly influenced by the nature and severity of accompanying lesions, some of which require surgical repair. The traditional surgical approach (physiologic repair) of ccTGA attempts at restoring normal physiology by repairing the associated lesions. It fails to address the most serious anatomic abnormality, mainly ventriculoarterial discordance, and results in less than optimal long-term outcomes. Anatomic repair was introduced to incorporate the left ventricle into the systemic circulation. The excellent short-term and intermediate results of the double switch operation and its modifications make it the procedure of choice for the treatment of ccTGA. Occasionally, the anatomic peculiarities of ccTGA do not allow straightforward biventricular repair, and univentricular palliation is a reasonable option. Regardless of the approach selected, late cardiovascular complications are relatively common, so ongoing outpatient surveillance is required. This chapter provides a comprehensive overview of the pathology, pathophysiology and surgical management of ccTGA.

Background Surgical repair of superior sinus venosus atrial septal defect associated with partial anomalous pulmonary venous connection is usually performed with either a two-patch technique or the Warden procedure. However, both techniques can lead to superior vena cava or pulmonary vein stenosis, as well as sinus node dysfunction. To prevent these complications, we have utilized a modified two-patch technique. Methods Between January 2000 and December 2015, 44 patients underwent modified two-patch repair of superior sinus venosus atrial septal defect associated with partial anomalous pulmonary venous connection at our institution. The technique utilizes the right atrial appendage to patch the superior vena cava and avoid superior vena cava obstruction. Patients' data were collected from reviewing hospital charts and clinic records. Results Mean age was 10.3 years (range, 0.68 to 61 years). There was no hospital mortality. Six patients (13.6%) had sinus node dysfunction after surgery. All of them recovered their normal sinus node function at a mean follow-up period of 45.7 months. No superior vena cava or pulmonary vein stenosis was observed at long-term follow-up. Conclusions A modified two-patch technique is a valid alternative to other methods of superior sinus venosus atrial septal defect and partial anomalous pulmonary venous connection repair, and prevents late systemic/pulmonary venous pathway obstruction while maintaining sinus rhythm.

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.

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.

Objective/Background Historically, the sole option for patients with a dysfunctional native right ventricular outflow tract (RVOT) requiring re-establishment of pulmonary competence has been surgical PVR. We sought to compare early outcomes of hybrid pulmonary valve replacement (PVR) combining surgical plication of the main pulmonary artery followed by transcatheter PVR, with a contemporary cohort of surgical PVR patients. Methods Retrospective chart analysis of all patients with a dilated native RVOT eligible for surgical PVR over 36 months was performed. The cohorts included patients with previous tetralogy of Fallot repair (n = 14), and previous intervention for congenital abnormality of the pulmonary valve (n = 7). Results Twenty-one patients with a dysfunctional native RVOT met criteria for PVR; 8 using the hybrid procedure (group 1: age, 31.5 +/- 17.4 years) and 13 with cardiopulmonary bypass (CPB) (group 2: age, 31 +/- 18.4 years). Valve delivery was successful in all patients with no procedural mortality. Group 1 had a lesser requirement for blood products (P =< 0.001) and a trend toward shorter hospital stay and higher post-operative hemoglobin. No patients in group 1 received inotropic support post-operatively compared to 54% of patients in group 2. Mean follow-up was 3.4 months for group 1 and 13.6 months for group 2 with the average peak gradient across the RVOT of 20.1 and 15.1 mm Hg respectively (P = 0.12), all with no more than mild PI. Conclusions Transcatheter hybrid PVR following RVOT plication provides a reasonable alternative to surgical PVR particularly in higher risk cohorts, reducing possible longer-term consequences of repeated runs of CPB. © 2016 Wiley Periodicals, Inc.

To assess the difference in time to and predictors of reintervention according to valve type in surgical bioprosthetic pulmonary valve replacement (PVR) in patients younger than 30 years of age from multiple centers.Data were retrospectively collected for 1278 patients30 years of age undergoing PVR at 8 centers between 1996 and 2015.Mean age at PVR was 19.3 ± 12.8 years, with 719 (56.3%) patients ≤18 years of age. Diagnosis was tetralogy of Fallot in 626 patients (50.5%) and 165 (12.9%) had previous PVR. Median follow-up was 3.9 years (interquartile range, 1.2, 6.4). Multiple valve types were used, most commonly CE PERIMOUNT, 488 (38.2%), CE Magna/Magna Ease, 361 (28.2%), and Sorin Mitroflow 322 (25.2). Reintervention occurred in 12.7% and was most commonly due to pulmonary stenosis (68.8%), with most reinterventions occurring in children (85.2%) and with smaller valve sizes (P .001) Among adults aged 18 to 30 years, younger age was not a significant risk factor for reintervention. Surgical indication of isolated pulmonary regurgitation was associated with a lower risk of reintervention (P .001). Overall, 1-, 3-, 5-, and 10-year freedom from reintervention rates were 99%, 97%, 92%, and 65%. The only independent risk factors for reintervention after controlling for age and valve size were lack of a concomitant tricuspid valve procedure (P = .02) and valve type (P .001); Sorin and St Jude valves were associated with similar time to reintervention, and deteriorated more rapidly than other valve types.In this large multicenter study, 8% of patients have undergone reintervention by 5 years. Importantly, independent of age and valve size, reintervention rates vary by valve type.

Aortic valvuloplasty is being used with increasing frequency due to lack of an ideal valve substitute. Improved understanding of the anatomy and function of the normal and abnormal aortic valves has expanded the indications of the procedure to a multitude of conditions in both adults and children.

The purpose of this study is to evaluate clinical outcomes of neonates who underwent a Norwood operation as a first step of a planned biventricular repair and the impact of associated risk factors.A retrospective cohort study was performed on all neonates (n = 44) undergoing the Norwood operation as the first stage of a biventricular (Norwood-Rastelli) repair from January 2000 to December 2012 at a single center. Multivariable analysis was performed to identify predictors of survival.Stage one mortality was 9%. The interstage survival for nonsyndromic and syndromic patients was 100% versus 46%, respectively (p0.001). Twenty-four patients (55%) underwent biventricular completion repair with no mortality. Freedom from reintervention after biventricular completion was 53% at 6 years. The overall survival for nonsyndromic patients versus syndromic patients was 86% versus 43%, respectively (p = 0.01). Genetic syndromes and prematurity were significant predictors of interstage mortality on multivariable analysis.Staged biventricular repair for patients with complex left ventricular outflow tract obstruction, ventricular septal defect, and aortic arch obstruction can be achieved with excellent outcomes for neonates without genetic syndromes. The staged approach is associated with longer time to reintervention after the biventricular completion.

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.

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.

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. © 2013 Wiley Periodicals, Inc.

This chapter describes the pertinent pathoanatomy of D-transposition of the great arteries, L-transposition of the great arteries (congenitally corrected transposition), as well as the malpositions of the great arteries associated with double-outlet right ventricle. It also comprises the surgical options to correct and palliate these complex defects. This includes correction of further complicating features like VSD and ventricular outflow tract obstructions and anomalies of the coronary arteries. The classic operations dealt with are carrying the names of Senning, Mustard, Rastelli, Nikaidoh, and Ilbawi. Special treatment options include palliative features like pulmonary artery banding and aortopulmonary shunts, both as mere palliations and as measures for retraining anatomical left ventricles.

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.

Background The use of right ventricle-to-pulmonary artery shunt (RV-PA) is thought to improve postoperative hemodynamics in hypoplastic left heart syndrome (HLHS). Proximal shunt obstruction often prompts early reintervention. Description of our technique and analysis of outcomes after Norwood operation using RV-PA shunt (NRVPA) was undertaken. Methods Between January 2005 and June 2009, 100 consecutive neonates with HLHS underwent stage-1 palliation (S1P). Sixty-five had NRVPA. From 56 survivors, 52 (86. 2%) reached pre-Glenn cardiac catheterization. The RV-PA shunt was constructed using nonringed polytetrafluoroethylene with reversed beveling and hood augmentation of its anterior wall. The PA size, Nakata index, pulmonary to systemic blood flow ratio, and any pre-Glenn intervention were studied. The RV function and NRVPA diastolic reversal flow were assessed. Results Mean age and weight at S1P were 6. 6 ± 3. 5 days and 3. 21 ± 0. 57 kg, respectively. No shunt reversal flow to RV was noted the first 48 hours after S1P by echocardiogram. No patient had shunt revision after hospital discharge from S1P. Mean age and weight at stage-2 palliation were 14. 9 ± 5. 1weeks and 5. 4 ± 2. 1 kg, respectively. No patient had proximal shunt stenosis. Six patients with distal shunt stenosis and associated central PA hypoplasia (32% ± 17% PA diameter reduction), 3 with left PA and 2 with right PA) stenoses had balloon angioplasty. The pulmonary to systemic blood flow ratio between patients requiring pre–stage-2 palliation intervention and patients without intervention was 1. 05 ± 0. 3 versus 1. 13 ± 0. 37 ( p = 0. 09). Right PA and left PA growth was balanced (right PA/left PA 0. 99 ± 0. 3; right PA 26. 8 ± 11. 5 mm 2 versus left PA 27. 2 ± 12. 3 mm 2 ; p = 0. 1). Freedom from significant RV dysfunction for S1P survivors at 3, 6, 12, 36, and 48 months was 94.4% ± 3.1%, 88.7% ± 4.4%, 86.7% ± 4.7%, 83.9% ± 5.3%, and 79.7 ± 6.7%, respectively. Conclusions Norwood RV-PA promotes balanced branch PA growth. The RV-to-PA shunt with proximal valvelike hood modification significantly reduces proximal anastomotic stenosis and need for early shunt revision. Limited early shunt diastolic reversal flow into the RV and subsequent early unloading of RV during BDG might preserve ventricular performance.

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.

Blind pouch formation of the pulmonary artery (PA) in patients having undergone a Fontan operation can present a serious risk for thromboembolic events. Either primary or secondary closure of this stump is necessary to reduce this risk. Unfortunately, secondary closure is oftentimes difficult due to the size and anatomy of the presenting patient. We describe the insertion of a muscular ventricular septal defect (VSD) device via a perventricular approach for successful closure of a pulmonary stump in a 3-year old, 16-kg child. To our knowledge, this is the first report of a perventricular approach for successful closure of a PA stump. © 2014 Wiley Periodicals, Inc.

Background There is limited data regarding the management of children with HLHS (hypoplastic left heart syndrome) and completion Fontan (CF) at or prior to 2 years of age. A study was undertaken to investigate intermediate outcomes. Methods From August 1999 to December 2008, 52 HLHS survivors underwent extracardiac-conduit CF (29 prior to [group A] versus 23 after 25 months of age [group B]). Mean weight and median follow-up was 9.9 ± 1.3 kg and 79 months (2 to 112) for group A versus 12.1 ± 2.2 kg and 87 months (1 to 97) for group B, respectively. Polytetrafluoroethylene conduits were used. Perioperative outcome variables were studied. Results There was no hospital mortality and 1 late death (group B). In group A, 16 CF had 20-mm conduit (vs 18 mm in 13). Thirteen group B patients received 18-mm conduit (vs 20 mm in 10). No CF was taken down. Eight patients in each group required fenestration ( p = not significant [NS]). Cardiopulmonary bypass was 78 ± 37.3 minutes in group A versus 77 ± 33.9 minutes in group B ( p = NS). Mean pulmonary artery size, McGoon ratio, and transpulmonary gradient in group A versus group B were 7.94 ± 0.59 mm versus 7.87 ± 0.58 ( p = NS), 1.79 ± 0.2 versus 1.77 ± 0.19 ( p = NS), and 4.1 ± 1.1 mm Hg versus 3.5 ± 0.9 ( p = NS), respectively. Aspirin and warfarin were used postoperatively. Mean hospital length of stay, intensive care unit length of stay, and pleural drainage duration between groups A and B were the following: 10.9 ± 5.8 days versus 12.7 ± 6.1 ( p = NS); 5.6 ± 2.6 days versus 6.7 ± 2.9 ( p = NS); and 7.6 ± 3.8 days versus 8.7 ± 4.1 ( p = NS), respectively. Between groups, no difference in ventilatory support time, arrhythmia, sinus-atrioventricular node dysfunction, protein-losing-enteropathy, and thromboembolic events were noted. Conclusions In HLHS patients, extracardiac conduit CF can be performed with good intermediate results at or prior to 2 years of age. Earlier unloading of a univentricular heart by means of CF in patients with collateral accessory flow between systemic and pulmonary circulation might be advantageous for future ventricular function preservation. In addition, relief from early cyanosis might alleviate deleterious effects from a prolonged cyanotic state. Fenestration is less often required without effect in pleural drainage duration. Age at CF had no effect in preoperative pulmonary artery growth and conduit size selection.

BACKGROUND Unroofing of anomalous coronary artery originating from the opposite sinus of Valsalva has become the procedure of choice for this congenital lesion, with surgery performed in children as young as two years old. An increasing number of this anomaly is diagnosed in infancy with no clear indication whether surgical repair should be done in this age group. This paper reviews our experience with this anomaly, and focuses on its surgical management in infants. METHODS Between April 2002 and February 2007, eight patients underwent surgical repair of anomalous coronary artery arising from the opposite sinus of Valsalva and coursing between the aorta and pulmonary artery. Patients' age varied from two months to 28 years with a mean of 11.7 +/- 11.1 years. SURGICAL TECHNIQUE Surgical repair involved unroofing the intramural segment of the anomalous coronary artery using cardiopulmonary bypass. RESULTS Two patients were younger than one year (Group A), and six patients were older than one year (group B). The mean intensive care unit stay was 2.5 +/- 0.7 days for Group A and 2.8 +/- 1.9 for Group B. The mean hospital stay was 4 +/- 1.4 days for Group A and 4.3 +/- 2.4 days for Group B. There was no mortality and no complications. The mean follow-up period is 14 +/- 15.7 months with a range of one to 39 months. At the time of the last follow-up, all patients were asymptomatic in New York Heart Association class I and follow-up echocardiography on six of eight patients showed wide open coronary ostium. CONCLUSION Unroofing the anomalous coronary artery arising from the opposite sinus of valsalva can be done in infants with minimal morbidity and mortality. Longer follow-up is needed to assess long-term results.

Several major advances in the field of neonatal cardiac surgery have improved the outcome of these severely ill patients. Among the preoperative advances are improvement in the management of patients with single ventricle equivalents, prenatal diagnosis, and interventional procedures. Intraoperative advances include better understanding of the inflammatory process caused by cardiopulmonary bypass and ways to manage it, newer techniques for treating hypoplastic left heart syndrome and aortic valve disease, and primary repair of complex lesions. Postoperative refinements in managing pulmonary hypertension, low cardiac output state, and ventricular assist devices have decreased surgical mortality and morbidity. Although his primary interest has been adult cardiac surgery, Dr. Geha has contributed directly and indirectly to these advances.

The hybrid approach to management of hypoplastic left heart syndrome (HLHS) was developed as an alternative to neonatal Norwood surgery, providing a less invasive initial palliation for HLHS. We describe our experience in extending the concept of the hybrid procedure to palliate neonates with anatomically compromised systemic arterial blood flow in a variety of congenital cardiac anomalies and supporting its application as first-line palliation in centers developing their HLHS programs. Retrospective review of patients undergoing therapy for HLHS at a single institution from June 2008 to December 2014 was performed. Subject demographics, clinical and procedural data, along with follow-up, were collected. Thirteen patients had initial hybrid palliation for HLHS during the time frame indicated at a median age of 8 days (range 1-29 days) and median weight of 3.4 kg (range 2.4-4.6 kg). Diagnoses included typical HLHS (n = 6), right-dominant unbalanced atrioventricular septal defect with arch hypoplasia (n = 4), double outlet right ventricle [subpulmonic VSD (n = 1) and intact ventricular septum (n = 1)] with hypoplastic transverse aortic arch and borderline left ventricular dimensions. Standard approach with bilateral pulmonary artery banding and ductal stenting was carried out in all thirteen patients. Two patients required two ductal stents at the time of index procedure. There were no intraprocedural complications. Median intubation length post-procedure was 4 days (range 1-74 days). Median hospital stay post-procedure was 47 days (range 15-270 days). The overall mortality rate on follow-up through comprehensive stage 2 over the 6-year experience was 38 % (5 out of 13). Of note, the mortality rate was significantly lower in the latter 3 years of the study period when the procedure was adopted as a primary palliation for HLHS (14 % or 1 out of 7) compared to the initial 3-year period when it was reserved for higher risk cohorts (67 % or 4 out of 6). Median time to subsequent surgery was 3 months (range 1-4 months). One patient required further ductal stenting on follow-up and developed subsequently airway compression. On median follow-up of 24 months, two patients required pulmonary artery arterioplasty. The hybrid procedure may be used for palliation for a variety of cardiac lesions to avoid high-risk surgery in the neonatal period. This approach may be also an alternative in centers performing lower number of Norwood surgery, which has been associated with higher mortality.

IntroductionHypoplastic 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 methodsA 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.ResultsThe 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 (pConclusionUsing 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.

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.

Purpose This study compares cerebral protection using no cerebroplegia and using antegrade cerebroplegia with variable flow rates during deep hypothermic circulatory arrest (DHCA). Methods Twenty healthy neonatal piglets (2.5-3.8 kg) underwent 60 minutes of DHCA. No cerebroplegia was used in group 1 (n = 5). Cold (16°C) antegrade cerebral perfusate was administered through the innominate artery at 10 mL/kg per minute in group 2 (n = 5), at 25 mL/kg per minute in group 3 (n = 5), and at 50 mL/kg per minute in group 4 (n = 5). Venous samples for lactate, pyruvate, S-100B protein, and creatine kinase BB (CKBB) were drawn from the jugular vein before and after discontinuation of cardiopulmonary bypass—lactate at 5 minutes postbypass, pyruvate at 5 minutes postbypass, S-100B protein at 30 minutes postbypass, and CKBB at 6 hours postbypass. Piglets were killed 6 hours postbypass and their brains were harvested for histological/immunologic studies. Extent of damage was assessed using a semiquantitative score of 0 to 4 based on a validated method. Results Evidence for significant apoptosis and necrosis was apparent in all groups. The mean H&E score was 2.2 for group 1, 2.3 for group 2, 2.5 for group 3, and 2.3 for group 4. The mean terminal deoxynucleotidyl transferase–mediated dUTP-biotin nick end labeling score was 1.0 for group 1, 1.2 for group 2, 1.7 for group 3, and 0.8 for group 4. Pathological changes were not greater in the piglets that did not have antegrade cerebral perfusion. Serum lactate, pyruvate, S-100B protein, and CKBB did not distinguish between perfusion strategies. Conclusions In neonates, unmodified antegrade cerebral perfusion at flow rates of 10, 25, and 50 mL/kg per minute during DHCA does not provide additional protection of the brain as determined by histology, immunology, serum lactate, pyruvate, S-100B protein, and CKBB.

Cardiac hypertrophy is an early landmark during the clinical course of heart failure, and is an important risk factor for subsequent morbidity and mortality. The hypertrophy response to different types of cardiac overload is distinguished both at the molecular and cellular levels. These changes have been extensively characterized for pressure load hypertrophy; however, similar information for volume load hypertrophy is still needed. This study was undertaken to improve the existing method of producing experimental cardiac volume load. Previous investigators have employed surgical aorto-caval shunt (ACS) as a model for volume load hypertrophy (VO) in rats. The procedure is relatively simple and involves glue to seal the aortic hole after ACS. However, it has several limitations mostly related to the use of glue e.g. poor visualization due to hardening of tissues, imperfect sealing of the puncture site and glue seeping through the aortic hole resulting in shunt occlusion. We have modified the procedure using aortic adventitial suture instead of glue and 18G angiocatheter instead of 16G needle, which eliminated the technical difficulties from the former method. The ACS was visually confirmed at sacrifice, and the VO demonstrated by time-related changes in the heart weight/body weight ratio which increased from 78% at 4 weeks to 87% at 10 weeks and increased liver/body weight ratio by 22% at 10 weeks of post aorto-caval shunt. Cardiac expression of atrial natriuretic peptide (ANF) also demonstrated time-related increase in ANF mRNA (+275% increase at 4 weeks, p < 0.05, and +370% increase at 10 weeks, p < 0.001). This modified technique of aorto-caval shunt offers simpler, reproducible and consistent model for VO hypertrophy in rats.

Objectives: This study was undertaken to compare conventional cardioplegic strategies with a new approach that uses a modified nonpotassium maintenance solution between cardioplegia doses in stressed neonatal hearts. Methods: Thirty-five neonatal piglets underwent 60 minutes of ventilator hypoxia (inspired oxygen fraction 8%-10%) followed by 20 minutes of ischemia on cardiopulmonary bypass. In 10 animals bypass was discontinued without further ischemia (stress control group). The other 25 received a warm blood cardioplegic induction and were separated into 5 groups. In 5 animals cardiopulmonary bypass was discontinued without further ischemia (cardioplegia control group); the remaining 20 underwent an additional 70 minutes of cold blood cardioplegic arrest. Five received only intermittent cardioplegia every 20 minutes, whereas 15 also received cold blood maintenance infusions between cardioplegic doses (integrated strategy). In 5 of these animals the blood was unmodified, whereas in 10 a modified nonpotassium “cardioplegia-like” solution was delivered either antegradely (n = 5) or retrogradely (n = 5). Myocardial function was assessed by pressure-volume loops (expressed as percentage of control); vascular function was assessed by coronary vascular resistance. Results: All piglets that underwent hypoxic ischemic stress alone (controls) died. Warm induction alone (cardioplegic controls) partially repaired the stress injury. Intermittent cardioplegia preserved the depressed systolic function (end-systolic elastance 40% vs 39%), increased diastolic stiffness (255% vs 239%), reduced adenosine triphosphate (10.6 vs 12.2 μg/g tissue), and elevated coronary vascular resistance at levels identical to warm induction alone; infusing unmodified blood between cardioplegia doses (standard integrated) improved results slightly. In contrast, infusion of a cold modified solution (antegrade or retrograde) between cardioplegia doses (modified integrated) completely restored systolic function (end-systolic elastance 100% and 97%, P

The traditional surgical approach (physiologic repair) of congenitally corrected transposition of the great arteries (ccTGA) attempts at restoring normal physiology by repairing the associated lesions. It fails to address the most serious anatomic abnormality, mainly ventriculoarterial discordance, and results in less than optimal long-term outcomes. Anatomic repair was introduced to incorporate the left ventricle into the systemic circulation. The excellent short-term and intermediate results of the double switch operation and its modifications make it the procedure of choice for the treatment of ccTGA.

Warm cardioplegic induction improves the ischemically "stressed" adult heart. However, it is rarely used in infants, despite the fact that many newborn hearts are stressed by other factors such as hypoxia. The need for amino acids as well as their mechanism of action has also not been studied.We first assessed the role of cardioplegic induction temperature in 10 nonhypoxic neonatal piglets undergoing 70 minutes of multidose blood cardioplegic arrest. Five piglets (group 1) received a cold (4 degrees C) induction, and 5 (group 2) a warm (37 degrees C) induction. Twenty-six other piglets underwent ventilator hypoxia (fraction of inspired oxygen, 8% to 10%) for 60 minutes before cardiopulmonary bypass (stress). Six piglets (group 3) then underwent 70 minutes of cardiopulmonary bypass without ischemia (hypoxia controls), and 20 underwent 70 minutes of cardioplegic arrest. Five of these (group 4) received cold cardioplegic induction, and 15 received warm induction; in 5 of these (group 5), the warm cardioplegic solution contained amino acids, in 5 others (group 6), it was unsupplemented, and in the remaining 5 (group 7), nitroglycerin was added to determine the role of vasodilation. Myocardial function was assessed by pressure-volume loops (expressed as a percent of control), and coronary vascular resistance was measured with cardioplegic infusions.In nonhypoxic (normal) piglets, cold (group 1) and warm (group 2) induction completely preserved systolic function (end-systolic elastance, 100% versus 104%) and preload recruitable stroke work (100% versus 102%), with minimal increase in diastolic compliance (162% versus 156%). Hypoxia-reoxygenation alone (group 3) depressed systolic function (end-systolic elastance, 51%+/-2%) and preload recruitable stroke work (54%+/-3%), and raised diastolic stiffness (260%+/-15%). The detrimental effects of reoxygenation persisted (unchanged from reoxygenation alone) with cold induction (group 4) or warm induction without amino acids (groups 6 and 7). In contrast, warm induction with amino acids (group 5) restored systolic function (end-systolic elastance, 105%+/-3%; p0.001 versus groups 3, 4, 6, and 7) and preload recruitable stroke work (103%+/-2%; p0.001 versus groups 3, 4, 6, and 7), and decreased diastolic stiffness (154%+/-7%; p0.001 versus groups 3, 4, 6, and 7). However, there was no difference in myocardial oxygen consumption in hypoxic hearts receiving a warm induction (6.9 versus 6.5 versus 7.3 mL/g per 5 minutes) (groups 5, 6, 7), and coronary vascular resistance was lowest with nitroglycerin (group 7).Cardioplegic induction can be given either warm or cold in nonhypoxic neonatal hearts. In contrast, only warm induction with amino acids repairs the hypoxic injury, but the primary mechanism of action is not related to increased metabolic activity or vasodilation.

Background . Coarctation repair in neonates or small infants, using a subclavian patch, has a relatively high risk of restenosis, especially if complicated by the presence of a short subclavian artery or long coarctation segment. We introduce a technical modification that facilitates the use of a subclavian flap, and decreases the restenosis rate in this subgroup of patients. It consists of a side-to-side transverse aortic anastomosis at the level of the coarctation, which widens the coarctation segment, shortens the isthmus, and pulls the distal end of the aortotomy proximally, allowing a tension-free subclavian flap aortoplasty. Methods . Fifty-three consecutive neonates or infants less than 18 weeks old, with complex coarctation, underwent repair using this technique. Mean age was 26 ± 3 days and 36 patients (68%) were less than 28 days old. Weights ranged from 1.4 to 6.4 kg (mean 3.4 ± 0.2 kg), and 26 patients had other cardiac anomalies. Preoperative gradient by Doppler measurement ranged from 25 to 90 mm Hg (mean 49 ± 2 mm Hg). Results . Mean aortic cross-clamp time was 27 ± 1 minutes (range 19 to 34 minutes). There were no deaths or surgical complications. Follow-up echocardiogram 4 to 52 months postoperatively (mean 25 ± 2 months) demonstrated no significant pressure gradient (less than 20 mm Hg) in 51 of 53 patients (96%), and a significant gradient in 2 patients (4%), which was subsequently corrected with balloon angioplasty. Conclusions . The technical modification described shortens the isthmus, and thus allows for a longer aortotomy distal to the area of coarctation resulting in a tension-free repair especially in patients with a short subclavian artery. It also widens the area of coarctation, and as a result leads to a lower early recoarctation rate in this high-risk group. With increasing emphasis on the need for a longer aortotomy to prevent restenosis, this modification will have increasing application, especially in the neonatal population.

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

OBJECTIVE To evaluate the different surgical options in patients with recoarctation and minimal collaterals. METHODS Thirty-three cases operated on between January 1980 and January 1995 were reviewed. Initial repair was end-to-end anastomosis in 16 patients, subclavian artery aortoplasty in 10, synthetic patch aortoplasty in 4 and bypass conduit in 3 patients. Age at reoperation was 7.5 +/- 5.2 years (1-17 years). Pressure gradient was 20-48 Torr (33 +/- 9). Upper extremity resting or exercise systemic hypertension was present in all. In 18 patients recoarctation was repaired using subclavian artery aortoplasty (n = 15) or synthetic patch aortoplasty (n = 3); alone in 9, with temporary heparinized bypass in 2, or in addition to placement of ascending aorta to descending aorta conduit as a permanent bypass through a left thoracotomy in 9. In 13 patients a conduit was interposed between ascending aorta and descending aorta through a right thoracotomy. In one patient recoarctation segment was patched on cardiopulmonary bypass through a midsternotomy. RESULTS There was no mortality or complications. All patients had no echocardiographic pressure gradients across recoarctation on 5 +/- 3.4 years follow-up. Persistent systemic hypertension following recoarctation repair was present in 3/8 patients (37%) operated on at age greater than 10 years, but has been resolved in all 25 patients less than 10 years of age (P = 0.02). CONCLUSIONS Use of ascending aorta to descending aorta conduit, either alone through a right thoracotomy, or as permanent bypass in combination with patching the recoarctation through a left thoracotomy provides safe and excellent relief of obstruction.

Valve repair in children and young adults is an attractive alternative to replacement or Ross procedure. In the past, the various valvuloplasty techniques have addressed isolated valve pathology. Recently, however, a holistic approach involving repair of all aortic root structures has evolved and promised to be an excellent alternative to valve replacement. It provides good long-term results and avoids all the drawback of prosthetic valve insertion in this age group. Development of ideal patch material needed for valve repair and better understanding of the anatomy and function of the aortic root may further improve the short- and long-term outcomes of aortic valvuloplasty.

Background The Sano modification of the Norwood operation is a well-established first step palliation for hypoplastic left heart syndrome (HLHS). Theoretically, the first point of resistance to pulmonary flow should be in the proximal Sano, generating high Doppler flow velocity. Paradoxically, however, some patients have low gradients in the proximal Sano conduit. The objective of this study was to determine the hemodynamic and anatomic significance of low proximal Sano Doppler flow velocity and its clinical implications. Methods Doppler-derived peak gradients in the proximal Sano conduits were measured in HLHS patients after Norwood-Sano surgery over a 4-year period and confirmed by cardiac catheterization within 2 to 4 weeks. Clinical outcomes of patients with proximal Sano gradients of 30 mm Hg or less (group 1) were compared with patients whose gradient was greater than 30 mm Hg (group 2). Results Of the 53 patients, 21 (40%) belonged to group 1. Patients in group 1 had smaller ostial right and left pulmonary artery (PA) diameter (3.2 ± 1.2 mm versus 4.5 ± 1.8 mm, p = 0.03; and 3.4 ± 1.2 mm versus 5.6 ± 2.1 mm, p = 0.01) when compared with patients in group 2. Patients (7 of 10) who needed either balloon angioplasty of a distal Sano or proximal branch PA were from group 1 ( p = 0.01). Patients in group 1 had higher rates of unintended PA interventions (33% versus 9%) and complications (48% versus 16%) compared with group 2. Conclusions Low peak Doppler flow velocity in the proximal Sano correlates with the presence of either distal Sano stenosis or proximal branch PA stenosis. These patients require close follow-up in the interstage period and may need earlier intervention.

We sought to determine the international experience with the quadripolar diaphragm pacer system and to test two hypotheses: the incidence of pacer complications would be (1) increased among pediatric as compared to adult patients; and (2) highest among active pediatric patients with idiopathic congenital central hypoventilation syndrome (CCHS). Data were collected via a questionnaire coupled with the Atrotech Registry data for a total of 64 patients (35 children and 29 adults) from 14 countries. Thoracic implantation of electrodes and bilateral pacer use each occurred in 94% of all subjects. Tetraplegic (vs pediatric CCHS) patients were more typically paced 24 hours/day (P = 0.001). Pacing duration averaged 2.0 +/- 1.0 years among children and 2.2 +/- 1.1 years among adults. Infections occurred among 2.9% of surgical procedures, all in pediatric CCHS patients (vs pediatric tetraplegic patients, P = 0.01). The incidence of mechanical trauma was 3.8%, without significant differences among patient groups. The incidence of presumed electrode and receiver failure were 3.1% and 5.9%, respectively, with internal component failure greater among pediatric CCHS than pediatric tetraplegic patients (P < 0.01). Intermittent or absent function of 0-4 electrode combinations occurred among 19% of all patients, with increased frequency among pediatric CCHS than pediatric tetraplegic patients (P < 0.03). Complication-free successful pacing occurred in 60% of pediatric and 52% of adult patients. In all, 94% of the pediatric and 86% of the adult patients paced successfully after the necessary intervention. Although pacer complications were not increased among pediatric as compared to adult patients, the incidence of complications was highest among the active pediatric patients with CCHS. Longitudinal study of these patients will provide invaluable information for modification and improvement of the quadripolar system.

Background DiGeorge anomaly is characterized by hypoplasia or atresia of the thymus and parathyroid glands resulting in T cell–mediated immune deficiency, hypocalcemic hypoparathyroidism, and conotruncal cardiac defects. It usually is associated with deletions of chromosomal region 22q11. We hypothesized that the stimulated (secretory reserve) but not the constitutive secretion of parathyroid hormone would be reduced in normocalcemic children with conotruncal cardiac defects but no overt immune deficiency and would be related to the presence of a deletion in the DiGeorge chromosomal region of 22q11. Methods and Results Blood-ionized calcium and serum-intact parathyroid hormone were measured at baseline and seven more times during hypocalcemia induced during cardiopulmonary bypass in 22 patients and 10 control subjects with an atrial septal defect. Chromosomal deletions were detected by fluorescent in situ hybridization and DNA dosage analysis. There were no differences in basal calcium and parathyroid hormone levels between patients and control subjects. All had increased parathyroid hormone in response to hypocalcemia; despite lower calcium levels, parathyroid hormone levels were lower in patients. The parathyroid hormone secretory reserve in 14 of 22 patients was reduced compared with control subjects; 4 of the 14 had deletions. Conclusions A significant number of children with conotruncal cardiac defects have normocalcemia and a normal constitutive level of parathyroid hormone but deficient parathyroid hormone secretory reserve; about 30% also have 22q11 deletions. Such children may be at risk for the later development of hypocalcemic hypoparathyroidism.

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.

Although ligation of the carotid artery following extracorporeal membrane oxygenation (ECMO) procedures has not been associated with any apparent acute neurologic changes, its long-term sequelae on cerebral function are still unknown. The authors have adopted a policy of repairing all carotid arteries following ECMO. Between April 1990 and June 1993, 34 consecutive patients underwent carotid artery reconstruction. Indications for ECMO were respiratory distress syndrome in 18, meconium aspiration in 8, pneumonia in 6, myocardial failure in 1, and sickle cell anemia in 1. All patients were neonates (ages seventy-two ± sixty-three hours), except 5 (2, three months; 1, two years; and 2, four years old).Duration of ECMO was (mean ± SD, 156 ± 68 hours). Arterial cannulation was performed after extended mobilization of the carotid artery by using a Biomedicus (8- 10F) cannula. Following decannulation the traumatized segment was resected and the artery repaired by means of multiple interrupted nonabsorbable 7-0 sutures. No heparin was given. There were no complications related to repair.Follow-up is available on all patients (7.6 ±4.1 months). Detailed neurologic evalu ation and ultrasound and Doppler studies of the carotid artery were performed on all patients at discharge, at two months, at six months, and at one year following ECMO. All carotid arteries were patent with good flow, except one. Transient communicating hydro cephalus developed in 1 patient but responded readily to medical treatment. The rest have normal-for-age neurologic evaluation. The authors' experience suggests that carotid reconstruction following ECMO is feasible, safe, and probably advantageous in the long term. Further follow-up is necessary before this technique can be applied to all infants undergoing ECMO procedure.

Wide variation in morphology of double-outlet left ventricle allows numerous surgical alternatives that require sorting out to develop a more organized approach. There is a high association between tricuspid abnormalities and right ventricular hypoplasia with double-outlet left ventricle that calls for either Fontan-type procedure or biventricular repair. With pulmonic stenosis, biventricular repair has been accomplished using right-sided conduits. When pulmonic stenosis is mild or absent, repair techniques without conduits depend on the commitment of the ventricular septal defect (VSD). With subaortic VSD and mild pulmonic valvar stenosis, we successfully performed translocation of the main pulmonary artery and valve to the right ventricle on 2 patients (ages 32 and 8 months). Both patients are doing well 2 years and 1 year postoperatively. Others have successfully connected the right ventricle to the pulmonary artery with intraventricular baffle by enlarging a subaortic VSD or when the VSD is either subpulmonic or doubly committed. With subaortic VSD, although it has not been reported, biventricular repair can also be accomplished using a right ventricle-to-aorta baffle combined by either atrial or arterial switch. We believe that a simplified management plan can be formed in double outlet left ventricle based on the size of the right ventricle, presence of pulmonic stenosis, and commitment of the VSD. Whenever possible, translocation of the main pulmonary artery and valve or intraventricular repair should be accomplished in double-outlet right ventricle minimizing the use of right-sided conduits and reoperation.

ObjectiveTo compare the results of bidirectional Glenn when performed with or without pulsatile pulmonary blood flow in a cohort of patients with a single ventricle.MethodsRecords of 212 patients undergoing staged single ventricle palliation during a 10-year period were retrospectively reviewed. Of those, 103 (33 in pulsatile group A and 70 in nonpulsatile group B) were selected.ResultsDemographics and pre- and intraoperative variables were comparable for both groups. There was no difference in oxygen saturations immediately after the bidirectional Glenn in the 2 groups. The duration and output of chest tube drainage, incidence of chylothorax, and total length of stay was higher in group A. There was no difference in the number of diuretics or oxygen requirement upon discharge between groups. Pre-Glenn measurements showed a mean McGoon ratio in group A of 1.5 (1.46-1.57) and in group B of 1.59 (1.53-1.7) (P = .11); however, there was a significant difference in the ratio between groups at pre-Fontan measurements: group A, 1.76 (1.73-1.79) and group B, 1.6 (1.53-1.66) (P

Over a 13-year period, 20 infants and children underwent transsternal approach for repair of coarctation and associated cardiac defects. Fifteen patients (75%) were operated on in the last 6 years. Thirteen patients (group 1) had intracardiac shunts and 7 (group 2), intracardiac obstruction of valvular insufficiency. Group 1 had a mean age of 0.8 ± 1.9 years versus 4 ± 3 years for group 2 ( p = 0.05). There were 12 patients (92%), 7 months old or less in group 1. Aortic arch hypoplasia was present in 6 patients in group 1. A large patent ductus arteriosus was present in 5 of these 6 patients versus no patent ductus arteriosus in patients without aortic arch hypoplasia ( p = 0.006). The mean pulmonary blood flow to systemic blood flow ratio in group 1 was 3.8 ± 2 and the mean right ventricular to left ventricular ratio, 0.8 ± 0.2. The coarctation repair fell mostly into three types: side patch aortoplasty (8), ductal tissue excision and patch aortoplasty of the concavity of the aortic arch (6), and subclavian aortoplasty (4). There was one early death (5%) which was due to sepsis in a newborn. Another newborn who had subclavian aortoplasty needed a left carotid artery—descending aorta bypass conduit early because of aortic arch hypoplasia. All patients were followed to 12 years (mean follow-up, 4.3 ± 3.5 years). There were no late deaths. Two patients had recurrent coarctation, 1 after an end-to-end repair and the other because of incomplete arch enlargement after a side patch aortoplasty. We conclude that a transsternal approach, which minimizes incisions and reoperations, can be safely accomplished in infants and children to repair coarctation and associated cardiac defects. Although most coarctation repair techniques could be performed, ductal tissue excision with patch aortoplasty of the concavity of the arch appears simple and provides an excellent option for complete relief of obstruction, unhampered aortic growth, and probably reduced aneurysm formation.

Recurrent significant aortic valvular stenosis or regurgitation, or both, after balloon or open valvotomy in pediatric patients often necessitates aortic valve replacement. In an attempt to preserve the aortic valve, we performed extended aortic valvuloplasty in 21 children with recurrent aortic valve stenosis or regurgitation from January 1989 to March 1993. Previous related procedures were one open aortic valvotomy or more (n =15), balloon valvotomy (n = 4), balloon valvotomy after surgical valvotomy (n =1), and repair of iatrogenic valve tear (n =1). Mean age at the time of the extended aortic valvuloplasty was 6 ± 3.4 years. Mean pressure gradient across the aortic valve was 56 ± 12 torr. Regurgitation was moderate (grade 2 to 3) in nine and severe (grade 4) in 12 patients. Extended aortic valvuloplasty techniques consisted of thinning of valve leaflets (n =15), augmentation of scarred and retracted leaflets with autologous pericardium (n =11), resuspension of the augmented leaflet (n =14), release of the rudimentary commissure from the aortic wall (n = 5), extension of the valvotomy incision into the aortic wall on both sides of the commissure (n = 20), patch repair of the sinus of Valsalva perforation (n =1), reapproximation of tears (n = 5), and narrowing of the ventriculoaortic junction (n = 2). No operative deaths occurred. The postoperative mean pressure gradient, assessed by most recent Doppler echocardiography or cardiac catheterization at a follow-up of 18 ± 6 months, was 19 ± 6 torr (p