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Scimitar syndrome is a rare congenital heart syndrome. It has a varied presentation and is therefore difficult to diagnose. Diagnosis requires a high index of suspicion and imaging modalities like computed tomography and magnetic resonance imaging are usually helpful. Treatment involves stabilizing the patient followed by surgical repair which can vary case by case. We report a rare case of an infant incidentally diagnosed with scimitar syndrome. This case highlights the unique occurrence of left pulmonary vein dilation and tortuosity and the progressive nature of pulmonary venous stenosis that could be associated with scimitar syndrome. It's worth acknowledging as a case because of its incidental finding, variable presentation, and rare involvement of the left pulmonary vasculature.

The Ross procedure has been considered in children as an optimal surgical procedure due to potential growth of the aortic annulus, lack of anticoagulation requirement, very low morbidity rate and excellent survival. Five-hundred-thirty-six (366 male, mean age 29.4 ± 11.1 years) underwent Ross procedure between 1990 and 2016 and had complete clinical and echocardiographic follow-up. Mean follow-up was 16.3 ± 4.9 years. Patients were divided in 2 groups according to age at surgery. Group 1 consisted of 320 (60%) patients less than 18 years old (223 male, mean age at surgery of 9.5 ± 5.6 years). Group 2 consisted of 216 (40%) patients older than 18 years of age (143 male, mean age at surgery of 26.3 ± 8.2 years). One-hundred-thirty (24%) patients had a redo procedure or surgery. Freedom from all re-operation and or percutaneous reintervention on either the aortic and pulmonary valves was 99% after 1 year, 94% after 5 years, 89% after 10 years, 83% after 15 years and 78% after 20 years. Freedom from redo surgery for AV 99% after 1 year, 94% after 5 years, 90% after 10 years, 81% after 15 years and 80% after 20 years. Freedom from redo surgery for PV was 100% after 1 year, 95% after 5 years, 89% after 10 years, 78% after 15 years and 76% after 20 years. The ideal candidate for Ross operation is a patient with congenital aetiology and an aortic root diameter ≤ 15 mm/m2. A pulmonary fresh preserved homograft seems to perform better on the long term.

OBJECTIVES: Survival of children having cardiac arrest refractory to conventional cardiopulmonary resuscitation (CPR) is very poor. We sought to examine current era outcomes of extracorporeal CPR (ECPR) support for refractory arrest following surgical correction of congenital heart disease. METHODS: Demographic, anatomical, clinical, surgical and support details of children requiring postoperative ECPR (2007–12) were included in multivariable logistic regression models to determine the factors associated with survival. RESULTS: Thirty-nine children, median age 44 days (4 days–10 years), required postoperative ECPR at a median interval of 1 day (up to 15 days) after surgery. Thirteen (33%) children had single-ventricle pathology; Risk Adjustment in Congenital Heart Surgery (RACHS)-1 categories were 2, 3, 4 and 6 in 6, 15, 13 and 5 patients, respectively. Median CPR duration was 34 (8–125) min, while median support duration was 4 (1–17) days. Seven (18%) patients underwent cardiac re-operation, 28 (72%) survived >24 h after support discontinuation and 16 (41%) survived. Survival rates in neonates, infants and older children were 53, 39 and 17% (P=0.13). Survival rates for single- vs two-ventricle pathology patients were 54 and 35%, (P=0.25) and 50, 47, 23 and 60% in RACHS-1 2, 3, 4 and 6 patients, respectively (P=0.37). Survivors had shorter CPR duration (25 vs 34 min, P=0.05), lower pre-arrest lactate (2.6 vs 4.6 mmol/l, P=0.05) and postextracorporeal membrane oxygenation (ECMO) peak lactate (15.4 vs 20.0 mmol/l, P

Objectives The Glenn bidirectional cavopulmonary connection (BCPC) is an established procedure in multistage palliation of various single ventricle (SV) anomalies. We aimed to report the current outcomes following BCPC and to examine risk factors affecting survival and progression to the next palliation stage. Methods Two hundred and twenty-seven consecutive children with variable SV pathologies underwent BCPC from 2002 to 2007. Competing risk analyses were performed to model events after BCPC (death and transition to Fontan) and subsequently after Fontan (death and cardiac reoperation) and to examine the associated risk factors for poor outcomes. Results There were 139 males (61%) with a median age of 7.6 months [interquartile range (IQR) 6.0-10.8] and median weight of 6.2 kg (IQR 5.2-7.4). Forty-three patients (19%) had primary BCPC and 184 (81%) had prior palliation: aortopulmonary shunt (APS) (n=83), Norwood (n=55), pulmonary artery (PA) band (n=48), atrial septectomy (n=25), PA reconstruction (n=14), anomalous pulmonary venous connection repair (n=7) and other (n=8). Predominant ventricle was left morphology (n=122, 54%), right morphology (n=95, 42%) and two equally developed ventricles (n=10, 4%). Twenty-six patients (12%) had bilateral superior vena cava. Concomitant surgery included atrioventricular valve repair (n=18), PA augmentation (n=80), percutaneous Fontan preparation (n=34) and other (n=24). Competing risk analysis showed that 5 years following BCPC, ∼17% have died, 76% have undergone Fontan and 7% were alive awaiting or not qualifying for Fontan. On multivariable analysis, risk factors for death prior to Fontan were pulmonary vascular resistance (PVR) index of >3 WU/M2 [hazard ratio (HR) 3.9, P=0.001], dominant right ventricle (HR 2.1, P=0.03) and prior palliation other than APS (HR 0.4, P=0.03). Competing risk analysis showed that 3 years following 172 Fontan operations, ∼10% have died, 6% have undergone further cardiac surgery and 84% were alive and free from reoperation. Overall, 8-year survival following BCPC was only 74%. Conclusions Despite established selection criteria and improved surgical technique and medical management, there is a continuous failure and attrition risk following BCPC. Outcomes are influenced by underlying cardiac anomaly; patients with dominant left ventricle (i.e. tricuspid atresia, double inlet left ventricle) having the best survival while those with dominant right ventricle (i.e. hypoplastic left heart syndrome, double outlet right ventricle with heterotaxy) having the worst survival. Increased PVR remains a significant factor affecting mortality.

Background We report early results of surgical preparation and subsequent percutaneous Fontan completion strategy for the treatment of single-ventricle defects. Methods Two hundred twenty-seven patients underwent bidirectional cavopulmonary connection (BDCPC) between 2002 and 2007. Thirty-four patients had lateral tunnel created at time of BDCPC, fenestrated with 10 to 14 mm openings with the cardiac superior vena cava end patched to maintain BDCPC physiology. At second stage, Fontan circulation was established by superior vena cava patch perforation, tunnel dilatation, and stenting plus fenestration device closure. Results Thirty-four patients underwent Fontan preparation with BDCPC. Median age was 7.7 months (5 to 51) and 29 patients (85%) had previous palliation. Mean bypass and ischemic times were 141 and 72 minutes, respectively. Median ventilation, intensive care, and hospital stay durations were 1, 5, and 10 days, respectively. There was one early death and two take-downs. Twenty-eight patients underwent Fontan procedure: surgical (n = 3), percutaneous (n = 25). None of the patients who underwent percutaneous Fontan completion required inotropes, chest tube insertion, or mechanical ventilation. Median intensive care and hospital stay durations were 1 and 6 days, respectively. There were no early mortalities after percutaneous Fontan but one late death and one surgical revision. Overall survival after BDCPC with Fontan preparation was 77%. Conclusions Despite longer bypass and ischemic times, Fontan preparation at time of BDCPC is feasible and associated with encouraging early outcomes. Percutaneous Fontan completion is associated with short recovery, low morbidity and excellent early dynamics, and echocardiographic and clinical outcomes. Further follow-up is needed to confirm those favorable results.

Objectives: The Ross procedure is the aortic valve-replacement procedure of choice in children. Nonetheless, late autograft re-operation for dilatation and/or valve regurgitation is of concern. We examined whether preoperative haemodynamic manifestation (e.g., stenosis, regurgitation and mixed aortic valve disease) affected late re-operation risk. Methods: Medical records of 227 children who underwent the Ross procedure (1991—2004) were reviewed. Competing-risks methodology determined time-related prevalence and associated factors for two mutually exclusive end-states after the Ross procedure: (1) death prior to subsequent autograft re-operation and (2) autograft re-operation, with the remainder of patients being alive and free from subsequent autograft re-operation.Results:There were 162 male patients (71%) in this study. Median age at surgery was 12.1 years (range: 1 week—18 years). The haemodynamic aortic valve dysfunction was primarily stenosis (n = 40, 18%), primarily regurgitation (n = 109, 48%) and mixed disease (n = 78, 35%). Underlying pathology was rheumatic fever (n = 104, 46%), congenital heart disease (n = 113, 50%) and endocarditis (n = 8, 3%). Competing-risks analysis showed that, at 10 years following the Ross procedure, 5% of patients had died, 16% had undergone autograft re-operation with aortic valve replacement and 79% were alive and free from autograft reoperation. Ten-year freedom from autograft re-operation for patients with preoperative stenosis, regurgitation and mixed disease was 97%, 69% and 93%, respectively, (p < 0.001 for regurgitation vs others). Risk factors for increased risk of autograft re-operation were rheumatic fever (parameter estimates (PEs): 2.09 0.75, p = 0.006), and earlier year of surgery (PE: 0.20 0.06, p = 0.001). Ten-year freedom from homograft replacement was 81% and was not dependent on haemodynamic manifestation (PE: 0.16 0.38, p = 0.68). Significant factors for homograft replacement included fresh homografts (PE: 2.2 0.63, p = 0.01) and annular enlargement (PE: 1.11 0.3, p = 0.01). Ten-year freedom from cardiac re-operation other than auto-/homograft was 85%, higher in patients with preoperative aortic regurgitation (PE: 1.01 0.42, p = 0.02). Concomitant cardiac surgery was a significant factor for late cardiac re-operation other than auto-/homograft replacement (PE: 1.79 0.39, p < 0.001). Conclusions: The Ross procedure in children is associated with excellent survival. Late autograft re-operation may be required; however, it is more common in children with preoperative aortic regurgitation, especially those with rheumatic fever. Better patient selection in later era has mitigated the risk of autograft re-operation. Continued improved candidate selection, along with modifications in autograft implantation and root/sinotubular stabilisation techniques, may further decrease late autograft failure. # 2010 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

Objectives: Although the majority of infants with severe left ventricular outflow tract obstruction (LVOTO) can be managed with balloon or surgical aortic valvotomy, a more complex biventricular repair may be required in a subset of infants with multi-level obstruction, failure of or complication to prior intervention. In the presence of normal left ventricle size and inflow, the Ross procedure is applied in patients with/without ventricular septal defect (VSD), while the Yasui procedure is applied only in those with VSD. We report mid-term outcomes in a single institution. Methods: Thirty-five consecutive infants with severe LVOTO underwent complex biventricular repair using the Ross (n = 21) or Yasui (n = 14) procedure. Outcomes were studied using univariate and multivariable parametric models. Results:The Ross procedure was done at a median age of 88 days (8—353days), in 8/21(38%) neonates.As manyas 12/21 (57%) hadprior catheterand/orsurgicalintervention. Concomitant procedures included arch reconstruction (n = 4/21, 19%) and mitral valve repair (n = 6/21, 29%). In addition, 14/21 (67%) had annular enlargement (modified Ross—Konno). Haemodynamic manifestation was isolated obstruction (n = 10/21, 48%) or mixed obstruction/regurgitation (n = 11/21, 52%). Survival was 81% at 1 month, 70% at 1 year and 63% at 5 years. In multivariable regression models, factors associated with increased risk of mortality included neonatal surgery (p = 0.007), mitral valve repair (p = 0.02), longer cross-clamp time (p = 0.003), and postoperative extracorporeal membrane oxygenator (ECMO) (p = 0.004). Freedom from any cardiac re-operation was 86% at 5 years. The Yasui procedure was done at a median age of 36 days (7—207 days), 6/14 (43%) in neonates. As many as 13/14 procedures (93%) were primary and one procedure followed a prior single-stage Norwood palliation. A total of 10/14 (71%) had critical aortic stenosis and 4/14 (29%) had atresia. All patients had VSD, and 11/14 (79%) required concomitant arch reconstruction. Survival was 79% at 1 month and 5 years while 5-year freedom from re-operation was 57%. Conclusions: Complex biventricular repair can be performed in neonates and infants with severe LVOTO with mid-term survival that is equivalent to that published following balloon or surgical aortic valvotomy. Associated lesions are significant factors that influence outcome and proper patient selection may further improve survival. In neonates with concomitant arch obstruction and VSD, the Yasui operation may be associatedwithlowerearlymortalityrisk.Neonateswithconcomitantmitralvalvepathologymaybebetterservedwithsingleventriclepalliation strategy.

Background The Rastelli procedure is the standard surgical treatment of d-transposition of great arteries (d-TGA), ventricular septal defect (VSD), and pulmonary stenosis. Late morbidity is significant due to recurrent left ventricular outflow obstruction (LVOTO), early conduit obstruction, and arrhythmias, with troublesome late mortality. To avoid recurrent LVOTO, we routinely enlarge the VSD and resect the infundibular septum before LV baffling to the aorta. We examined the efficacy of this approach in mitigating recurrent LVOTO risk. Methods Late echocardiographic and time-related clinical results of patients undergoing the Rastelli procedure were examined. Demographics and operative variables affecting outcomes were analyzed. Results The Rastelli cohort comprised 36 patients with d-TGA, VSD, and pulmonary stenosis. Median age at operation was 2.4 years (range, 0.3 to 8.3 years). Pulmonary stenosis was present in 31 and atresia in 5. Twenty-two patients had undergone a previous aortopulmonary shunt, and 6 had an atrial septectomy. No operative or late deaths occurred. Time-related freedom from permanent pacemaker implantation, recurrent LVOTO on echocardiogram, and conduit replacement at 10 years was 82%, 100%, and 49%, respectively. Systolic function was normal in all but 3 patients and 92% were in New York Heart Association functional class I and II. None of the patients had late arrhythmias or required heart transplantation. Conclusions Early and midterm survival after the Rastelli procedure is satisfactory. Aggressive resection of the infundibular septum to enlarge the VSD has mitigated the risk of LVOTO recurrence. Late conduit obstruction remains an important source of morbidity and frequently requires reintervention.

BACKGROUND AND OBJECTIVES: The Blalock–Taussig (BT) shunt is regarded a safe and effective means increasing pulmonary blood flow for cyanotic heart conditions. The evaluation of shunt size for postoperative hemodynamics and until second-stage palliation remains difficult. Our objective is to compare the effect of different shunt sizes on short- and long-term outcomes after a BT shunt surgery. DESIGN AND SETTINGS: This is a retrospective review in a tertiary care hospital. PATIENTS: The records of all patients with a modified BT shunt between January 2007 and January 2010 were reviewed. METHODS: Patients were divided into 2 groups: Group A with a BT shunt of 3.5 mm and Group B with a BT shunt of 4 mm, and their body weight was less than 4 kg. Groups were compared for the short-term outcome, which includes: intensive care unit course, intensive care unit (ICU) and hospital stay, mortality during the same admission, and the size of branch pulmonary arteries (PAs) before second surgery. RESULTS: A total of 29 (42%) patients were in Group A and 40 (58%) in Group B. There was no significant difference in the mean weight between both groups; P value .06. There was no significant difference between the 2 groups in regard to diastolic blood pressure and lactate levels by the end of the first 48 hours after surgey. Group A required longer duration of inotropes and more days of ventilation with P value .03 and .001, respectively. The mean (standard deviation) ICU and hospital stay were 10.0 (8.9) days and 17.0 (11.4) days, respectively, for Group A and 12 (8.9) days and 15 (12.9) days, respectively, for Group B with P value .7 and P value .6, respectively. Yet Group B had a better branch PA size and required lesser intervention for branch PAs in comparison to Group A. CONCLUSION: These data suggest that a smaller shunt size may have a trend toward higher morbidity. A bigger shunt size does not necessairly lead to stealing phenomena and its consequnces, and can be performed with a low risk leading to a better growth of branch PAs.

Background: Improved survival with postoperative extracorporeal membrane oxygenation (ECMO) has expanded its application to children with single ventricle (SV) anomalies. We examine current-era outcomes of postoperative ECMO with special focus on patients with SV. Methods: Demographic, anatomic, surgical, and support details of 100 consecutive children requiring postoperative ECMO (2007-2012) were included into multivariable regression models to identify factors affecting survival. Results: Median age was 73 days (4 days-16.2 years), 31 patients had SV physiology. The ECMO indication was failure to wean cardiopulmonary bypass (34%) and postoperative low cardiac output (66%) including 37% having extracorporeal cardiopulmonary resuscitation (ECPR). Median ECMO duration was four days (1-21). The ECMO decannulation and survival to hospital discharge were 62% and 37%. In SV group, decannulation and survival rates were 55% and 32%. The SV-ECMO outcomes were best in ECPR subgroup (54%), following shunt (57%) or Norwood (46%) and worst following Glenn, Fontan, or total anomalous pulmonary venous connection repair (0% survival). On multivariable analysis, factors affecting odds of survival were performing angiogram (odds ratio [OR]: 15.28, confidence interval [CI]: 2.34-99.89, P = .004), prolonged ECMO duration (OR: 0.64, CI: 0.47-0.88 per day, P = .005), leaving cannulation snares (OR: 28.41, CI: 2.65-304.70, P = .006), higher HCO3 (OR: 1.19, CI: 1.04-1.36, P = .01), renal failure requiring hemodialysis (OR: 0.21, CI: 0.06-0.76, P = .02), bleeding requiring re-exploration (OR: 0.21, CI: 0.06-0.75, P = .02), ECPR in patients with SV (OR: 11.84, CI: 1.11-126.07, P = .04), delayed lactate normalization (OR: 0.95, CI: 0.90-0.99 per hour, P = .02), and elevated liver enzymes (OR: 0.97, CI: 0.95-1.00 per 10 unit/L, P = .05). Conclusions: The ECMO is valuable in patients with SV however results depend on anatomy, procedure, and support indication. Persistent markers of poor perfusion, end-organ injury, and prolonged ECMO duration are associated with mortality. Those factors could be modified by early ECMO application before organ damage, meticulous homeostasis to ensure adequate perfusion, early diagnosis, and reoperation on residual lesions to expedite weaning.

OBJECTIVES: Children with various single ventricle anomalies are at risk of developing systemic ventricular outflow tract obstruction (SVOTO) following volume unloading with cavopulmonary connection (CPC). We aim to evaluate the value of Damus–Kaye–Stansel (DKS) anastomosis at the time of CPC in eliminating late SVOTO risk. METHODS: Retrospective review of single ventricle patients who underwent DKS concurrent with CPC between 1997 and 2012 was performed. Clinical, echocardiographic and angiographic outcomes were analysed. RESULTS: Thirty-six children with single ventricle underwent DKS at the time of Glenn bidirectional CPC (n= 29) or Fontan total CPC (n= 7). The underlying anatomy was double inlet left ventricle (n= 18), double outlet right ventricle (n= 8), unbalanced atrioventricular septal defect (n= 4) and other (n= 6). Prior palliation included pulmonary artery band (n= 35), coarctation/arch repair (n= 11) and atrial septectomy (n= 8). Median age at the time of DKS was 8.9 months (range 3.6 months–9.1 years) and the median weight was 6.7 kg (range 5–27 kg). At the time of DKS, 17 patients (47%) had no SVOT gradient and 19 (53%) had SVOT gradient (mean 23.4 ± 18.7 mmHg). Overall survival was 89 and 83% at 1 month and 5 years, respectively. None of the deaths were related to SVOTO or DKS complications. When present, SVOT gradient decreased from 23.4 ± 18.7 mmHg preoperatively to 0 after DKS (P< 0.001). At the last follow-up, none of the patients developed any SVOT gradient; 78% of them had zero or trivial aortic/neoaortic valve regurgitation while 22% had mild regurgitation. None of the patients had evidence of compression of the left pulmonary artery or bronchus. Eighty-one percent of patients have reached or are suitable candidates awaiting final palliative surgery. CONCLUSIONS: DKS can be safely performed in conjunction with CPC without added mortality risk. It is very effective in mitigating SVOTO risk, with sustainable good semilunar valves function. Our data support an aggressive approach to performing DKS concurrent with CPC in children with single ventricle pathologies at risk of developing SVOTO.

BACKGROUND: Right ventricular pacing has been the treatment of choice in patients with congenital complete atrioventricular block (CAVB). However, the effect of chronic right ventricular pacing on left ventricular function in young patients is still controversial. AIM: The aim of the study was to assess the change in left ventricular systolic function in young patients (age ≤20 years) paced for isolated CAVB and to identify possible predictors of left ventricular systolic dysfunction. METHODS: We studied 55 young patients who underwent permanent right ventricular pacemaker implantation for CAVB in the absence of significant structural heart disease. We excluded patients affected by any condition known to affect left ventricular function. Echocardiographic data prior to and after pacemaker implantation were obtained. RESULTS: The mean age at the time of pacemaker implantation was 20 months, range 2.3-72 months. The mean duration of follow-up was 94.86 (range: 2-268 months). Chronic right ventricular pacing affected left ventricular shortening fraction (LVSF) significantly (pre = 37.8 ± 7.8 vs. post = 32.8 ± 5.5%, P = 0.0036). In 14 patients (25.4%), LVSF decreased by at least 7% (group A). The only parameter studied able to significantly discriminate the two groups was a better baseline LVSF in group A (baseline LVSF: 42.1 ± 5.2 vs. 32.2 ± 2.2%, P = 0.019; cut-off value >39%, P

Objectives In children with aortic valve disease associated with annular hypoplasia or complex multilevel left ventricular outflow tract obstruction, the Ross procedure, combined with a modified Konno-type aortoventriculoplasty, is advocated. We aim to examine the fate of the neoaortic apparatus and assess neoaortic valve function after the modified Ross–Konno procedure. Methods Forty-three patients, with a median age of 6 years, underwent the modified Ross–Konno procedure with a myectomy but without the use of a ventricular septal patch. Serial postoperative echocardiograms (n = 187) were analyzed, and regression models adjusted for repeated measures were used to model the longitudinal growth of the neoaortic annulus and root. Results There were 2 operative deaths (5%) and 1 late mortality. At 8 years, survival was 93% and freedom from autograft, homograft, and all-cause reoperation was 100%, 81%, and 72%, respectively. The median postprocedure diameter and z score were 14 mm (7-21 mm) and +1.3 (−3.0 to +6.1) for the neoaortic annulus and 21 mm (9-30 mm) and +1.6 (−1.3 to +4.1) for the neoaortic root, respectively. Serial echocardiograms showed a progressive increase in annular (+0.56 mm/year, P 001) and root (+0.89 mm/year, P 001) diameters but little change in annular (−0.07/year, P = .08) and root (−0.002/year, P = .96) z scores. Autograft regurgitation developed in 9 patients; however, the degree and progression of regurgitation over time were not significant ( P = .22). Conclusions After the modified Ross–Konno procedure, the neoaortic annulus and root increased in size proportionately to somatic growth. Autograft regurgitation, usually mild and stable, developed in few patients, and none required autograft reoperation. Our findings support the use of the modified Ross–Konno as the procedure of choice in children with aortic valve disease and complex left ventricular outflow tract obstruction.

OBJECTIVES: The Ross procedure is the preferred aortic valve replacement (AVR) choice in small children. Nonetheless, it is a complicated surgery and there are concerns that subsequent cardiac reoperations are exceptionally complex and associated with high morbidity and mortality. We examine the surgical spectrum and report outcomes of cardiac reoperations in patients who had undergone the Ross procedure during childhood. METHODS: Records of 227 consecutive children (

programs. Because of the pediatric cardiology and cardiac surgery advances, the number of children with CHD surviving into adulthood continues to increase. The complexity of these patients on the adulthood often exceeds the expertise of the health professionals that deal with them. Therefore, North American and European task forces have been developing recommendations for the management of these individuals. According to the World Health Organization (2006) the epidemiology of CHD in Saudi Arabia has not been determined. However, this group of patients has a peculiar anatomy and complications that will have a significant impact on their lives. As most early interventions have not been curative and approximately 50% of them might face further surgery and heart failure, as health professionals, we need to support the education of CHD patients; moreover we need to discuss lifestyle issues such as exercise, pregnancy, career planning, etc. These issues are often more important to patients and families than details about their disease. ACHD teams provide education to these patients and encourage them to create their own health files in order to make them proactive about their health. These teams have to establish a life-long partnership, particularly with young adults who are highly motivated and have excellent survival profile. Only through educated professionals and educated patients we will enable every CHD patient to enjoy their full potential life.

Objective: We aim to report time-related outcomes following mitral valve replacement (MVR) in children and to identify factors affecting outcomes. Methods: Clinical records from 307 children who underwent MVR between 1985 and 2004 were reviewed. Competing-risks methodology determined time-related prevalence of three mutually exclusive end-states: death, mitral reoperation and survival without subsequent MVR, and their associated risk factors. Results: Mean age was 11.4 5.6 years including 36 (12%) patients

Valve replacement in children is problematic, and associated with high anticoagulation-related complications and increased reoperation requirements. Multiple valve replacement may further increase morbidity and worsen outcome. The results are reported of combined aortic valve replacement (AVR) and mitral valve replacement (MVR) in children.The medical records of children who underwent simultaneous AVR and MVR between 1984 and 2004 were reviewed, and the short-term and long-term results and variables affecting outcomes explored. The mean duration of follow up was 9.7 +/- 6.6 years.A total of 84 patients (62 males, 22 females; mean age 15.0 +/- 2.2 years) was identified. The underlying pathology was mainly rheumatic (94%) and endocarditis (4%). Among the patients, 21 (25%) had undergone a prior cardiac surgery. The implanted valves were either mechanical (n = 71) or bioprosthetic (n = 13). The average aortic and mitral valve sizes were 22 mm and 29 mm, respectively. In total, 21 patients had concomitant cardiac surgery, most commonly tricuspid valve repair (n = 18). The mean cardiopulmonary bypass time and ischemic time were 142 +/- 47 min and 107 +/- 33 min, respectively. Survival at 30 days and at one year was 96% and 94%, respectively. The overall 15-year survival was 78% (bioprosthesis 92% versus mechanical 76%; p = 0.4). The 15-year freedom from cardiac reoperation was 59%, and 68% and 75% for mitral and aortic reoperation, respectively. Significant risk factors for reoperation were the use of a bioprosthetic valve (p = 0.003) and female gender (p = 0.03). Freedom rates from endocarditis, thromboembolic and bleeding complications at 15 years were 90%, 92%, and 96%, respectively. Among survivors, 95% were in NYHA class I/II.Children with rheumatic fever and endocarditis may require simultaneous AVR and MVR. Although the operative mortality is acceptable, patients continue to have constant attrition with time, especially those who have received mechanical prostheses. The risk of cardiac reoperation requirement is high in all patients. Despite the greater need for reoperation, bioprosthetic valves could be offered to selected patients, such as females and those who are non-compliant with anticoagulation regimens.

Hypoplastic left heart syndrome (HLHS) is characterized by left ventricular and ascending aorta hypoplasia. The treatment of this condition usually involves three surgical stages beginning with the Norwood Stage I operation and culminating in the Fontan-Kreutzer procedure with an intermediate cavopulmonary shunt. We will illustrate our current surgical approach for the Norwood Stage I reconstructive procedure.

The HemoDynamics Systems enabler is a new cardiac assist pump that can expel blood from the left ventricle and provide pulsatile flow in the aorta. We evaluated the efficacy of the 18 Fr enabler. The enabler was inserted from the left ventricular apex into the ascending aorta in eight sheep. Heart failure (mild, moderate, and severe) was induced by microsphere injection into the coronary arteries to reduce cardiac output by 10-30%, 31-50%, and more than 50% from baseline, respectively. The enabler was activated, and its flow was increased to approximately 2.0 L/min. Hemodynamic variables were recorded before and after activation. In moderate heart failure, cardiac output and mean aortic pressure increased from 2.3 +/- 0.6 L/min and 59 +/- 12 mm Hg before assist to 2.8 +/- 0.6 L/min and 70 +/- 8 mm Hg at 30 minutes after activation, respectively (p < 0.01). Left atrial pressure decreased from 17 +/- 3 to 13 +/- 4 mm Hg (p < 0.05). Similar findings were observed in mild and severe heart failure. Despite its small diameter, the enabler significantly improved the hemodynamics of failing hearts and may potentially serve as a means of peripheral left ventricular support. Further study is warranted.

Objectives Delayed first-stage palliation of children with hypoplastic left heart syndrome and related pathologies can be associated with poor outcomes because of development of progressive pulmonary vascular disease and volume load effects on the systemic ventricle and atrioventricular valve. We examine the current era's survival in this subgroup. Methods Fifty-five infants older than 2 weeks underwent the Norwood operation (2003–2007). Separate competing risk analyses were performed to model outcomes (death and transition to the next stage) after the Norwood operation and after bidirectional cavopulmonary connection. Results Median age was 32 days (range, 15–118 days). Forty-seven percent had hypoplastic left heart syndrome, and 53% had other complex univentricular variants. Mean ascending aortic size was 4.4 ± 1.9 mm, 10% had impaired ventricular function, 11% had moderate atrioventricular valve regurgitation, and 32% had restrictive pulmonary venous return. Pulmonary blood flow was established through an aortopulmonary shunt (n = 30) or Sano shunt (n = 25). After the Norwood operation, patients required longer ventilation and more oxygen and nitric oxide and had higher inotropic scores compared with those undergoing the traditional management protocol. Competing risks analysis showed that 2 years after the Norwood operation, 39% had died, and 57% underwent bidirectional cavopulmonary connection. Four years after bidirectional cavopulmonary connection, 15% had died, and 85% underwent the Fontan operation. Overall 3-year survival after the Norwood operation was 53%. Factors associated with mortality were age, lower weight at the time of the Norwood operation, impaired ventricular function, longer circulatory arrest, and lower pre–bidirectional cavopulmonary connection saturation. Conclusions Children older than 2 weeks undergoing the Norwood operation frequently require postoperative pulmonary vasodilatation and high inotropic support. A significant hazard of death persists through all steps of multistage palliation. Increased pulmonary vascular resistance and volume load effects, such as systemic ventricular impairment and atrioventricular valve regurgitation, are commonly evident in patients in whom treatment fails or who do not qualify to proceed to the next stage of palliation. Those patients should be closely monitored for timely referral for heart transplantation when indicated.

ObjectivesIn children with aortic valve disease associated with annular hypoplasia or complex multi-level left ventricular outflow tract obstruction (LVOTO), the Ross procedure, combined with a modified Konno-type aortoventriculoplasty is advocated. We aim to examine the fate of the neoaortic apparatus and assess neoaortic valve function following the modified Ross-Konno procedure.MethodsForty-three patients, median age 6 years, underwent modified Ross- Konno with myectomy but without ventricular septal patch utilization. Serial postoperative echocardiograms (n=187) were analyzed and regression models adjusted for repeated measures were used to model longitudinal growth of neoaortic annulus and root.ResultsThere were 2 operative deaths (5%) and 1 late mortality. At 8 years, survival was 93% and freedom from autograft, homograft and all-cause reoperation was 100%, 81%, and 72%, respectively. Median post-procedure diameter and Z-score were 14mm (7–21mm), and 1.25 (−3 to +6.1) for neoaortic annulus and 21mm (9–30mm) and 1.55 (−1.3 to +4.1) for neoaortic root. Serial echocardiograms showed progressive increase in annular (+0.56mm/year, p

Objective We aimed to identify characteristics differentiating children undergoing aortic valve replacement by using mechanical prostheses versus the Ross procedure and to compare survival and the need for aortic valve reoperation after each procedure. Methods From 1983 to 2004, 346 children underwent aortic valve replacement (215 underwent the Ross procedure and 131 underwent placement of a mechanical prosthesis). Factors associated with procedure choice were used to construct a propensity score for use as a covariate in regression models to adjust for potential confounding by indication. Results Patients undergoing the Ross procedure were younger, more likely to have a congenital cause, and less likely to have a rheumatic or connective tissue cause. They had a lower frequency of regurgitation, required more annular enlargement, and had less concomitant cardiac surgery. Competing-risk analysis showed that 16 years after aortic valve replacement, 20% of patients had died without subsequent aortic valve replacement, 25% underwent second aortic valve replacement, and 55% remained alive without further replacement. After propensity adjustment, factors associated with early-phase death included mechanical valves and a nonrheumatic cause. Mechanical valves were also associated with constant-phase mortality. Repeated aortic valve replacement was associated with the Ross procedure and a rheumatic cause. Both factors were also associated with all-cause cardiac reoperation. In children receiving mechanical prostheses, younger age and smaller valve size were significant risk factors for death. Freedom from homograft replacement after the Ross procedure was 82% at 16 years of follow-up. Conclusion Results from this study showed good outcomes and an acceptable complication rate with both valve choices. Given the significantly increased risk of early and late death in younger children receiving smaller mechanical valves, the Ross procedure confers survival advantage in this age group at the expense of increased reoperation risk, especially in patients with a rheumatic cause.

Introduction Dilated cardiomyopathy in children is a serious disease with significant burden and poor outcome. Ventricular desynchrony can lead to dysfunction and dilated cardiomyopathy (DCM). For such patients, cardiac resynchronization therapy (CRT) is a proven form of therapy however; the role of CRT in children is not well studied. Objective We aim to describe our experience with CRT in children with poor ventricular function. Methods We retrospectively reviewed all pediatric pts who underwent CRT in our institute. Data collected include: demographics, CRT indication, pre and post CRT ventricular assessment using echocardiogram, and follow up clinical evaluation. Results Between 09/05 and 12/08, 19 pediatric pts median age 4 years (8months to 16 years) underwent CRT for DCM. The etiology of d DCM was chronic RV pacing with and without congenital heart disease (CHD) in 15 pts, myocarditis in 2, and post repair of CHD 2. The median duration of chronic RV pacing prior to CRT was 4yrs (1-11 yrs) and all developed DCM. One pt had transvenous approach while all other pts had epicardial leads for the left ventricular and atrium. All devices were CRT-P. After 22 months of median follow up (1-46 months), 13 pts improved clinically with or without associated LV remolding. Theremaining 6 pts did not respond to CRT. Conclusion CRT is a promising mode of therapy for a subgroup of pediatric patients with poor ventricular function. More studies are needed to identify those who will benefit the most from this mode of therapy.