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Despite decades of experience, aspects of the management of tetralogy of Fallot with pulmonary stenosis (TOF) remain controversial. Practitioners must consider newer, evolving treatment strategies with limited data to guide decision making. Therefore, the TOF Clinical Practice Standards Committee was commissioned by the American Association for Thoracic Surgery to provide a framework on this topic, focused on timing and types of interventions, management of high-risk patients, technical considerations during interventions, and best practices for assessment of outcomes of the interventions. In addition, the group was tasked with identifying pertinent research questions for future investigations. It is recognized that variability in institutional experience could influence the application of this framework to clinical practice. The TOF Clinical Practice Standards Committee is a multinational, multidisciplinary group of cardiologists and surgeons with expertise in TOF. With the assistance of a medical librarian, a citation search in PubMed, Embase, Scopus, and Web of Science was performed using key words related to TOF and its management; the search was restricted to the English language and the year 2000 or later. Articles pertaining to pulmonary atresia, absent pulmonary valve, atrioventricular septal defects, and adult patients with TOF were excluded, as well as nonprimary sources such as review articles. This yielded nearly 20,000 results, of which 163 were included. Greater consideration was given to more recent studies, larger studies, and those using comparison groups with randomization or propensity score matching. Expert consensus statements with class of recommendation and level of evidence were developed using a modified Delphi method, requiring 80% of the member votes with 75% agreement on each statement. In asymptomatic infants, complete surgical correction between age 3 and 6 months is reasonable to reduce the length of stay, rate of adverse events, and need for a transannular patch. In the majority of symptomatic neonates, both palliation and primary complete surgical correction are useful treatment options. It is reasonable to consider those with low birth weight or prematurity, small or discontinuous pulmonary arteries, chromosomal anomalies, other congenital anomalies, or other comorbidities such as intracranial hemorrhage, sepsis, or other end-organ compromise as high-risk patients. In these high-risk patients, palliation may be preferred; and, in patients with amenable anatomy, catheter-based procedures may prove favorable over surgical palliation. Ongoing research will provide further insight into the role of catheter-based interventions. For complete surgical correction, both transatrial and transventricular approaches are effective; however, the smallest possible ventriculotomy should be utilized. When possible, the pulmonary valve should be spared; and if unsalvageable, reconstruction can be considered. At the conclusion of the operation, adequate relief of the right ventricular outflow obstruction should be confirmed, and identification of a significant fixed anatomical obstruction should prompt further intervention. Given our current knowledge and the gaps identified, we propose several key questions to be answered by future research and potentially by a TOF registry: When to palliate or proceed with complete surgical correction, as well as the ideal type of palliation; the optimal surgical approach for complete repair for the best long-term preservation of right ventricular function; and the utility, efficacy, and durability of various pulmonary valve preservation and reconstruction techniques. [ABSTRACT FROM AUTHOR]

Objective To determine the safety and efficacy of bivalirudin as an anticoagulant for pediatric open heart surgery (OHS) and to determine its appropriate dosage for this purpose. Design Prospective, randomized controlled trial. Setting Tertiary care hospital. Participants Fifty acyanotic children aged 1-12 years undergoing OHS. Interventions The children were randomized to receive either 4 mg/kg of heparin (n = 25, group H) or 1 mg/kg of bivalirudin bolus followed by 2.5 mg/kg/h infusion (n = 25, group B) as the anticoagulant. The doses were adjusted to maintain activated clotting time (ACT) above 480 seconds. At the conclusion of surgery, protamine (1.3 mg/100 U of heparin) was administered to children in group H. Measurements and Main Results The children were comparable in both groups with regard to demographic characteristics. The mean age and weight were 51.5 months and 13.4 kg in group H, and 59.3 months and 13.4 kg in group B. The dose of anticoagulant required was 4.0 ± 0.2 mg/kg in group H and 1.7 ± 0.2 mg/kg followed by 3.0 ± 0.7 mg/kg/h infusion in group B (p < 0.001). One child in group H required an additional dose compared to 13 (54.2%) children in group B. Intraoperatively, the ACT achieved was higher in group H compared to group B (p < 0.05). The ACT returned to baseline value after protamine administration in group H, but it remained elevated for 2 hours after termination of cardiopulmonary bypass (CPB) in group B (p < 0.01). The ACT was higher in group B compared to group H for 6 hours after termination of CPB (p < 0.05). Heparin prolonged the onset of clotting, decreased the rate and strength of thrombus formation, and inhibited platelet function to a greater extent than bivalirudin on viscoelastic coagulation testing. The total duration of surgery was prolonged in group B. The postoperative chest tube drainage was similar in group B (4.9 mL/kg) as in group H (5.9 mL/kg) in spite of higher ACT. The transfusion requirements were similar. No adverse event occurred in any patient. Conclusion Bivalirudin is a safe and effective anticoagulant for pediatric OHS. Though it is not suitable as a routine anticoagulant for this purpose, it may be used as a heparin alternative in instances when heparin cannot be used. The dose required to maintain ACT for more than 480 seconds was 1.7 ± 0.2 mg/kg followed by 3.0 ± 0.7 mg/kg/h infusion. The ACT remained elevated for 2 hours after stopping the infusion. Bivalirudin did not increase postoperative bleeding and transfusion requirement. [ABSTRACT FROM AUTHOR]

Objective To determine effects of allopurinol administration on outcomes following intracardiac repair of tetralogy of Fallot (TOF). Materials and Methods Fifty patients undergoing TOF repair were randomized to 2 groups of 25 each: the allopurinol group (n = 25) and the placebo group (n = 25). Postoperatively, inotropic score, rhythm, duration of mechanical ventilation, cardiac output, intensive care unit (ICU) stay, and hospital stay were assessed. Plasma troponin-I, superoxide dismutase (SOD), interleukin (IL) 1-ß, IL-6, and malondialdehyde were measured serially. Results Inotropic score was lower in the allopurinol compared with placebo group (11.04 ± 5.70 vs 17.50 ± 7.83; P = .02). Duration of ICU and hospital stay was lower in the allopurinol group. Plasma levels of SOD preoperative were (2.87 ± 1.21 U/mL vs 4.5 ± 2.08 U/mL; P = .012), immediately following release of crossclamp (2.32 ± 0.98 U/mL vs 5.32 ± 2.81 U/mL; P < .001), and after termination of CPB (2.18 ± 1.0.78 U/mL vs 3.44 ± 1.99 U/mL; P = .003) between the placebo versus allopurinol group, respectively. Postoperative levels of IL1-ß and IL-6 were lower in the allopurinol group. Malondialdehyde levels following CPB were lower in the allopurinol group (11.80 ± 2.94 pg/mL in the placebo vs 9.16 ± 3.02 g/mL in the allopurinol group; P < .001). Conclusions Allopurinol administration in patients undergoing intracardiac repair of TOF is associated with reduced inotropic scores, duration of mechanical ventilation, ICU stay, and hospital stay and favorable biochemical markers of inflammation. Further studies in multiple setups are needed before recommending it as a routine practice. [ABSTRACT FROM AUTHOR]

Objective The present study evaluates the feasibility, safety, and efficacy of edge-to-edge repair for moderate secondary/functional mitral regurgitation in patients undergoing aortic valve/root interventions. Methods Sixteen patients underwent transaortic edge-to-edge mitral valve repair. Mitral regurgitation was 2+ in 8 patients and 3+ in 6 patients. Two patients in whom cardiac arrest developed preoperatively had severe (4+) mitral regurgitation. Patients underwent operation for severe aortic regurgitation ± aortic root lesions. The mean left ventricular systolic and diastolic diameters were 51.5 ± 12.8 mm and 70.7 ± 10.7 mm, respectively. Left ventricular ejection fraction ranged from 20% to 60%. Primary surgical procedure included Bentall's ± hemiarch replacement in 10 patients, aortic valve replacement in 5 patients, and noncoronary sinus replacement with aortic valve repair in 1 patient. Results Severity of mitral regurgitation decreased to trivial or zero in 13 patients, 1+ in 2 patients, and 2+ in 1 patient. There were no gradients across the mitral valve in 9 patients, less than 5 mm Hg in 6 patients, and 9 mm Hg in 1 patient. There was no operative mortality. Follow-up ranged from 2 weeks to 54 months. Echocardiography showed trivial or no mitral regurgitation in 12 patients, 1+ in 2 patients, and 2+ in 2 patients. None of the patients had significant mitral stenosis. The mean left ventricular systolic and diastolic diameters decreased to 40.5 ± 10.3 mm and 58.7 ± 11.6 mm, respectively. Ejection fraction also improved slightly (22%-65%). Conclusions Transaortic edge-to-edge mitral valve repair is a safe and effective technique to abolish secondary/functional mitral regurgitation. However, its impact on overall survival needs to be studied. [ABSTRACT FROM AUTHOR]

We conducted a prospective randomized trial to compare del Nido (DN) cardioplegia with conventional cold blood cardioplegia (St Thomas [STH]) in pediatric patients. We randomized 100 pediatric patients aged ≤12 years undergoing elective repair of ventricular septal defects and tetralogy of Fallot to the DN and the STH groups. In the DN group, a 20 mL/kg single dose was administered. In the STH group, a 30 mL/kg dose was administered, followed by repeated doses at 25- to 30-minute intervals. The primary outcome was cardiac index that was measured 4 times intra- and postoperatively. Troponin-I, interleukin-6, and tissue necrosis factor-alpha were measured. Myocardial biopsy was obtained to assess electron-microscopic ultrastructural changes. Cardiac indices were significantly higher in the DN group than in the STH group 2 hours after termination of cardiopulmonary bypass (P = 0.0006), after 6 hours (P = 0.0006), and after 24 hours (P ≤ 0.0001). On repeated measure regression analysis, the cardiac index was on an average 0.50 L/min/m2 higher in the DN group than in the STH group at any time point (P = 0.002). Duration of mechanical ventilation (P = 0.01), intensive care unit stay (P = 0.01), and hospital stay (P = 0.0007) was significantly lower in the DN group. Patients in the DN group exhibited lower troponin-I release 24 hours following cardiopulmonary bypass (P = 0.021). Electron microscopic studies showed more myofibrillar disarray in the STH group (P = 0.02). Use of long-acting DN cardioplegia solution was associated with better preservation of cardiac index, lesser troponin-I release, and decreased morbidity. Ultrastructural changes showed better preservation of myofibrillar architecture. [ABSTRACT FROM AUTHOR]

A 21-year-old patient with familial hypercholesterolemia presented with angina caused by ostial stenosis of the left internal mammary artery and severe calcific aortic stenosis with small aortic root 9 years after coronary revascularization. The ostium of the left internal mammary artery was enlarged using a saphenous vein patch through a left supraclavicular incision, which improved left ventricular function. Successful aortic valve replacement with posterior aortic root enlargement was subsequently performed. The surgical management of this condition is discussed briefly. [ABSTRACT FROM AUTHOR]

Background The standard surgical treatment of coarctation of the aorta is through a left posterolateral thoracotomy. However, when a concomitant cardiac procedure is required or the conventional approach is not possible or is hazardous, extraanatomic bypass to the supraceliac abdominal aorta may be advantageous. We discuss our technique and report the long-term results. Methods Between January 1986 and January 2015, 25 patients (16 males, 9 females) underwent extraanatomic bypass to the supraceliac abdominal aorta for various lesions of the arch and the descending thoracic aorta. Extraanatomic bypass to the supraceliac abdominal aorta was performed for patients in whom balloon dilatation was not feasible due to associated arch hypoplasia (n = 9), long-segment thoracic aorta narrowing due to nonspecific aortoarteritis (n = 3), or isolated long-segment coarctation of the aorta (n = 3). Patients who needed concomitant cardiac procedures, such as aortic valve replacement (n = 4), ascending aortic aneurysm repair (n = 2), or coronary artery bypass grafting (n = 1), and in whom balloon dilatation had failed, also underwent extraanatomic bypass to the supraceliac abdominal aorta. Extraanatomic bypass was also performed in 3 patients with recurrent coarctation after surgical repair and in whom balloon dilation was not feasible or unsuccessful. Results There were no early or late deaths. The peak-to-peak gradients between the upper limb and the lower limb decreased from 59.3 ± 16.3 mm Hg to 2.0 ± 2.8 mm Hg ( p < 0.0001). The mean follow-up was 96.6 ± 92.6 months (range, 1 to 240 months; median, 54 months). Doppler interrogation of the lower limb arterial system after a mean follow-up of 86.4 ± 85.2 months showed an unobstructed flow pattern. The ankle-brachial pressure index improved from a preoperative value of 0.60 ± 0.07 to 1.04 ± 0.11 ( p < 0.0001). Systolic blood pressure decreased significantly compared with preoperative values (153.9 ± 18.9 vs 122.8 ± 10.2, p < 0.0001). Three patients continued to receive antihypertensive medication due to persistent mild hypertension. Conclusions Extraanatomic bypass to the supraceliac abdominal aorta provides effective palliation for complex aortic obstructions. It is easy and quick to perform, avoids fatal complications, and is well tolerated in all age groups. [ABSTRACT FROM AUTHOR]

Objective: The purpose of the present study was to study the midterm hemodynamic outcomes of unidirectional valved patch closure of ventricular septal defects (VSDs) in patients with VSD and pulmonary arterial hypertension (PAH). Methods: From January 2006 to January 2012, 20 patients with VSD with PAH and a pulmonary vascular resistance index >8 Wood units underwent VSD closure with a unidirectional valved patch using the technique previously described by us. Of these, 13 patients agreed to follow-up cardiac catheterization and were studied at a mean follow-up of 34.7 ± 18.6 months (range, 2-56). The mean age of these 13 patients was 8.5 ± 4.4 years (range, 2-19; median, 9), and the mean preoperative systemic saturation was 94.1% ± 3.4% (range, 87-99; median, 95.0) The mean preoperative pulmonary artery systolic pressure was 96.2 ± 13.6 mm Hg (range, 75-115; median, 103.0), and the mean preoperative pulmonary vascular resistance index was 10.0 ± 2.1 Wood units (range, 8.0-15.1; median, 9.3). Results: At follow-up cardiac catheterization, the mean systemic saturation had increased to 98.92%. The pulmonary vascular resistance index had decreased significantly to 5.8 ± 2.1 Wood units (P = .02). A significant decrease was seen in the pulmonary artery systolic, diastolic, and mean pressures (P = .000), and none of the patients had severe PAH. No patients died, and all patients were in New York Heart Association class I. Conclusions: Unidirectional valved patch closure of VSD is a promising technique for patients with a large VSD and severe PAH. It had a favorable effect on the immediate, early, and midterm clinical outcomes and hemodynamic parameters. [ABSTRACT FROM AUTHOR]

In the present report, we discuss a patient who developed persistent hypoxemia after an attempt at Fontan completion failed. As a bail-out procedure, a left common carotid artery to left internal jugular vein shunt was constructed, after which the hypoxemia was relieved. [Copyright &y& Elsevier]

Objective: The objective of this study was to assess the effectiveness of 0.5% ropivacaine used for parasternal intercostal blocks for postoperative analgesia in pediatric patients undergoing cardiac surgery. Design: A randomized, controlled, prospective, double-blind study. Setting: A tertiary care teaching hospital. Participants: Thirty children scheduled for cardiac surgery with a median sternotomy. Interventions: A 0.5% ropivacaine injection with 5 doses of 0.5 to 2.0 mL on each side in the 2nd to 6th parasternal intercostal space with a total dose of ropivacaine below 5 mg/kg or the same volume of saline before sternal wound closure. Measurements and Main Results: The time to extubation was significantly lower in patients administered the parasternal blocks with ropivacaine than in the control group; the mean values were 2.66 hours and 5.31 hours, respectively (p < 0.001). The pain scores were lower in the ropivacaine group compared with the saline group; mean values were 2.20 for the ropivacaine group and 4.83 for the saline group on a scale of 10. The cumulative fentanyl dose requirement over a 24-hour period was higher in the saline group than the ropivacaine group (p < 0.001). Conclusions: Parasternal blocks with ropivacaine appear to be a simple, safe, and useful technique of supplementation of postoperative analgesia in pediatric patients undergoing cardiac surgery with a median sternotomy. [ABSTRACT FROM AUTHOR]

We report a 14-month-old child with persistent truncus arteriosus and crossed pulmonary arteries. The potential advantage of crossed pulmonary artery arrangement in achieving surgical correction is discussed. [ABSTRACT FROM AUTHOR]

The present report describes the technical aspects of the atrial switch operation in the setting of dextrocardia, bilateral superior vena cavae, left atrial isomerism, and unroofed coronary sinus. Augmentation of the right atrial wall using bovine pericardium and in situ pericardial technique for construction of the pulmonary venous baffle ensured unobstructed systemic and pulmonary venous pathways. [Copyright &y& Elsevier]

Objective: Anatomic repair is being actively evaluated as the preferred option for congenitally corrected transposition of the great arteries. We present our 13-year experience with this approach. Methods: Between May 1994 and September 2007, 68 patients with congenitally corrected transposition of the great arteries underwent anatomic repair. Thirty-one patients (group 1, mean age of 94.8 ± 42.3 months) underwent a combined Rastelli and atrial switch operation. Thirty-seven patients (group 2, mean age of 36.1 ± 46.9 months) underwent an arterial switch operation and atrial rerouting. Eight patients in group 2 had an intact ventricular septum. Results: Group 1 had 5 early deaths (17%) but no late deaths. Three patients underwent conduit revision at a mean follow-up of 62 months. Group 2 had 5 early deaths (13.5%). There were 4 late reoperations (2 pulmonary baffle revisions, 1 mitral valve replacement, and 1 permanent pacemaker implantation) and 4 late deaths (1 secondary to progressive left ventricular dysfunction, 2 secondary to uncontrolled atrial tachyarrhythmia, and 1 secondary to pulmonary hypertension and right ventricular failure). In group 2, 4 patients have a left ventricular ejection fraction less than 40%, 5 patients have moderate aortic incompetence, 5 patients have symptomatic tricuspid incompetence, 1 patient has tricuspid stenosis, 1 patient has superior cava obstruction, and 3 patients are receiving antiarrhythmic therapy. Conclusion: The occurrence of left ventricular dysfunction indicate that anatomic repair in the arterial switch group is still fraught with imperfections. The Rastelli group required conduit revisions but has otherwise performed well. [Copyright &y& Elsevier]

Background: The association of tetralogy of Fallot with total anomalous pulmonary venous drainage (TAPVD) is rare. We report our experience with this condition and review the literature. Methods: Between January 1997 and May 2008, 6 patients (aged 3 months to 5 years; median weight, 10 kg) with combined tetralogy of Fallot with TAPVD underwent complete primary repair at All India Institute of Medical Sciences, New Delhi, India. Their records were retrospectively reviewed. Results: A correct preoperative diagnosis was available in 5 patients by echocardiography and cardiac catheterization. Four patients had supracardiac TAPVD, and 1 each had TAPVD to the coronary sinus and right atrium. There were no early or late deaths. Median follow-up was 37.5 months ± 46.8 months (range, 2 to 112). All patients are in New York Heart Association class I. Follow-up echocardiograms have revealed no significant abnormalities and have documented normal biventricular function and pulmonary artery pressures. One patient underwent a 24-hour Holter examination at 68 months of follow-up for investigation of a new-onset 2:1 atrioventricular block, for which a permanent pacemaker implantation is planned. Conclusions: When diagnosed accurately, complete primary repair is possible in patients with tetralogy of Fallot and TAPVD and the outcomes are satisfactory with planned surgery. [Copyright &y& Elsevier]

Background: Conventional pulmonary artery banding (CPAB) is associated with high morbidity and mortality. We studied the changes in outcome with the use of an adjustable pulmonary artery band (APAB). Methods: Between June 2001 and June 2006, 147 patients underwent PAB: 91 underwent CPAB and 56 underwent APAB. Results: The clinical profile of patients was similar in both groups. Inotropic drugs were used in 91 (100%) patients in the CPAB group and in 12 (21%) in the APAB group (p < 0.001). Early band related reoperation was required in 17 patients in the CPAB group compared with 2 patients in the APAB group (p = 0.014). There were 21 (23%) early deaths in CPAB group compared with 1 (1.8%) in the APAB group (p < 0.001). There was no difference in the intensive care unit stay, hospital stay, and final band gradients in the two groups. On a mean follow-up of 22.8 ± 18.6 months (range, 4 to 72 months), there was PA distortion in 6 patients and band-migration in 4 patients in the CPAB group. These were not observed in the APAB group. Conclusions: Similar band gradients were achieved with the use of conventional or adjustable PAB. However, the use of this simple and inexpensive technique of APAB was associated with a significant reduction in the early band-related deaths, need for early multiple reoperations, and early adverse acute events, thus making it a safer alternative to CPAB, more so in unstable patients. [Copyright &y& Elsevier]

Background: Double valve replacement is associated with reduced long-term survival. This study investigates aortic valve replacement with mitral valve repair as an alternative to double valve replacement in patients with rheumatic heart disease (RHD). Methods: Between January 1995 and December 2005, 369 patients with RHD underwent combined aortic and mitral valve procedures. In 76 patients (20.6%), mitral valve repair with aortic valve replacement (group 1) was done. The remaining 293 patients (79.4%) underwent double valve replacement (group 2). A total of 351 patients (95%)—73 (96%) in group 1 and 278 (94.8%) in group 2—were 50 years of age or younger. Results: There was no difference in early mortality in the groups (4 in group 1 versus 25 in group 2, p = 0.35). The median follow-up was 60 months (range, 6 to 132 months) and 96% complete in group 1 and 92% in group 2. Actuarial survival was 90.5% ± 3.4% in group 1 and 81.60% ± 2.4% in group 2 at 60 months (p = 0.07). Event-free survival at 60 months was 78.3% ± 5.1% in group 1 and 48.4 % ± 3.2% in group 2 (p < 0.001). Reoperation-free survival was 92.5% ± 0.4% in group 1 and 99.5% ± 0.05% in group 2 (p = 0.014). Conclusions: Mitral valve repair with aortic valve replacement provides significantly better event-free survival than double valve replacement without a better actuarial survival. Reoperation rates are higher in the mitral valve repair and aortic valve replacement group, whereas thromboembolic complications were more in the double valve replacement group. Better event-free survival in patients undergoing mitral valve repair and aortic valve replacement still argues in favor of repair of the mitral valve whenever possible. [Copyright &y& Elsevier]

Background: We used the right atrial free wall as a patch to close atrial septal defects (ASD) and report its results. Methods: Between July 1998 and April 2006, 87 patients (mean age, 21.9 ± 13.9 years; range, 7 months to 54 years), underwent closure of ASD with an autologous right atrial free wall patch. The underlying diagnosis were very large secundum ASD in 51 patients, sinus venosus defect in 15, primum ASD in 5, large defect resulting from excision of a left atrial myxoma in 12, complete atrioventricular canal defect in 1, total anomalous pulmonary venous return with ASD in 2, and Ebstein anomaly with a large ASD in 1. Associated surgical procedures were mitral valve repair in 18 patients, repair of total or partial anomalous pulmonary venous drainage in 17, mitral valve replacement in 1, and tricuspid valve repair for Ebstein anomaly in 1. Results: There were two early deaths. One patient with primum defect and preoperative congestive heart failure died 3 weeks postoperatively from refractory ventricular fibrillation. Another patient died from persistent congestive heart failure after undergoing reoperation for residual mitral regurgitation. The remaining patients were discharged after 4 to 9 days. No flow was detected across the septal patch on predischarge echocardiography. At a mean follow up of 53.4 ± 26.7 months (range, 1 to 103 months), all patients except 1 are in sinus rhythm. One patient underwent reoperation for failed mitral valve repair after 1 month. At reoperation, the patch was intact with normal texture and without any suture dehiscence. Histopathologic examination of the explanted patch revealed viable endothelium and subendothelial muscle on both the surfaces of the patch. Results of Holter monitoring in 9 patients were normal. Electrophysiologic studies in 2 patients recorded normal atrial potentials from the site of the patch. No patch shrinkage, calcification, or thromboembolic complications were noted. Conclusions: The autologous, free, right atrial wall can be safely used as a patch for ASD closure and offers several advantages. [Copyright &y& Elsevier]

Background: The aim of this study was to analyze the feasibility and early results of transatrial total correction of tetralogy of Fallot (TOF). Methods: Of the 860 patients undergoing total correction for TOF between January 2000 and July 2005, 334 patients were considered morphologically suitable for transatrial total correction. The ventricular septal defect (VSD) closure, infundibular resection, and pulmonary valvotomy were performed through the right atrium without a right ventriculotomy. Age ranged from 6 months to 40 years (median, 2.8 years), and weight ranged from 5.5 to 70 kg (median, 14 kg). Results: Peroperatively, 34 patients required right ventriculotomy and transannular patch; hence, they were excluded from the study. In addition, pulmonary arteriotomy was required in 71 patients (22.9%). There were 4 hospital deaths. There were 4 early reoperations (residual/additional VSD in 3 and tricuspid regurgitation in 1). Two patients had complete heart block requiring permanent pacemaker. Echocardiography at discharge showed a peak right ventricular outflow tract gradient of 20 ± 5.2 mm Hg. Mean follow-up was 26.8 ± 4.2 months (range, 1 to 52 months). The right ventricular outflow tract gradients reduced to 13 ± 4.2 mm Hg after a mean interval of 18.8 ± 5.2 months. Follow-up New York Heart Association class was I in 240 cases (82%), II in 49 (16%), and III in 7 (2%). There were no late deaths or reoperations. Conclusions: Transatrial total correction of TOF can be accomplished in selected patients with good early results. In 300 cases (90%), the feasibility of transatrial total correction could be predicted accurately. [Copyright &y& Elsevier]

Objective: Pulmonary artery banding is associated with a high morbidity and mortality. We describe a new technique of adjustable pulmonary artery banding to prevent these problems. Methods: Between December 2003 and May 2005, 32 patients aged 18 days to 2 years (mean age, 2.5 ± 0.5 months) and weighing 2.1 to 6.3 kg (mean, 3.6 ± 1.3 kg) underwent adjustable pulmonary artery banding. Results: All patients survived the operation. There were 2 deaths, one caused by meningitis and another caused by aspiration pneumonitis. Satisfactory band gradients were achieved between 3 and 10 days (7.2 ± 2.6 days) in 3 to 6 sittings. Mean follow-up was 7.5 ± 3.8 months (1-16 months). One patient required reoperation for unsatisfactory band gradient 2 weeks after discharge. There were no late deaths. Follow-up computed tomographic angiograms (n = 4) demonstrated proper band placement and ruled out distortion of the pulmonary arteries. Four patients underwent uneventful definitive operations after an interval of 7 to 13 months. Conclusion: This technique of percutaneously adjustable pulmonary artery banding is simple and inexpensive and allows easy band adjustments without the need for multiple reoperations. [Copyright &y& Elsevier]

Background: This study was designed to compare the outcomes after total versus partial pericardiectomy clinically, echocardiographically, and hemodynamically. Methods: Three hundred ninety-five patients undergoing pericardiectomy for constrictive pericarditis between January 1985 and December 2004 were studied. Age was 10 months to 71 years (mean, 25.1 ± 13.4 years). Three hundred thirty-eight patients (85.6%) underwent total pericardiectomy (group I), and 57 patients (14.4%) underwent partial pericardiectomy (group II). Results: Operative and late mortality rates were 7.6% and 4.9%, respectively. Preoperative high right atrial pressure, hyperbilirubinemia, renal dysfunction, atrial fibrillation, pericardial calcification, thoracotomy approach, and partial pericardiectomy were significant risk factors for death. The risk of death was 4.5 times higher (95% confidence interval: 2.05 to 9.75) in patients undergoing partial pericardiectomy. At a mean follow-up of 17.9 ± 0.3 years (95% confidence interval: 17.3 to 18.6), actuarial survival was 83.8% ± 0.04% in group I and 73.9% ± 0.06% in group II (p = 0.004). At their last follow-up, 96.3% survivors of group I and 79.1% survivors of group II were in New York Heart Association class I/II (p < 0.001). Conclusions: Total pericardiectomy is associated with lower perioperative and late mortality, and confers significant long-term advantage by providing superior hemodynamics that appear to be independent of the etiology of constrictive pericarditis. [Copyright &y& Elsevier]

Background: The study was designed to assess the long-term results of one and one-half ventricular repair on systemic and pulmonary circulation, right ventricular growth and function, and the prevalence of arrhythmias. Methods: Eighty-four patients undergoing one and one-half ventricular repair between January 1990 and December 2003 were studied. Age was 4 to 504 months (mean, 47.9 ± 57.3 months). Sixty-nine survivors underwent serial echocardiography, radionuclide studies, cardiac magnetic resonance imaging, and cardiac catheterization. Results: Operative and late mortality were 10.7% and 8%, respectively. Perioperative and postoperative supraventricular arrhythmias were observed in 14.3% and 15.9% of patients, respectively. Risk factors for supraventricular arrhythmias included systemic ventricular dysfunction, heterotaxy syndrome, and Ebstein’s anomaly. Mean late postoperative superior vena caval pressure was 14.2 ± 1.52 mm Hg and right atrial pressure was 6.6 ± 0.74 mm Hg. At a median follow-up of 87 months, actuarial survival was 81.9% ± 0.04%, and 89.8% were in New York Heart Association class I or II. Serial cine–magnetic resonance imaging demonstrated significant growth of tricuspid valve and right ventricular cavity in 45% of patients. Conclusions: One and one-half ventricular repair can be performed with an acceptable risk. The operation maintains a low pressure in the inferior vena caval tributaries, and reverses the Fontan paradox. Patients with tripartite right ventricles demonstrated a tendency toward enlargement of the pulmonary ventricular chamber commensurable with somatic growth. [Copyright &y& Elsevier]

Background: The aim of our study is to assess the results of aortic valve replacement with the aortic homograft. Methods: From January 1994 through September 2003, 154 patients with aortic valve disease (rheumatic = 118, nonrheumatic = 36), and a mean age of 28.8 ± 18.2 years, underwent aortic valve replacement with an aortic homograft by the scalloped subcoronary (n = 110) or root replacement (n = 38) technique, or as a valved homograft conduit (n = 6). Associated procedures included mitral valve repair (n=30), open mitral commissurotomy (n = 22), tricuspid valve repair (n = 8), coronary artery bypass grafting (n = 6), and atrial septal defect closure (n = 1). Results: Early mortality was 7.8% (12 patients). Mean follow-up was 62 ± 33.4 months (4 to 127 months; median, 68.5 months). One hundred and twenty-four survivors (87.3%) had no or trivial to mild aortic regurgitation. A total of six patients required reoperation for homograft dysfunction alone (n = 4), infective endocarditis (n = 1), or failure of mitral valve repair (n = 1). There were four late deaths. Actuarial and reoperation-free survival at the median follow-up were 92.2 ± 2.2% and 95.8 ± 1.9%, respectively. Freedom from significant aortic stenosis or regurgitation was 86.1 ± 3.2%. Conclusions: Aortic valve replacement with an aortic homograft can be performed with acceptable early and late mortality and provides satisfactory midterm results. We did not note any difference in homograft dysfunction and reoperation with the use of either scalloped subcoronary or root replacement technique. [Copyright &y& Elsevier]

Background: The purpose of this study was to define the prevalence of specific sequelae after extracardiac Fontan operation. Methods: Sixty-five consecutive patients undergoing extracardiac Fontan operation were studied for mortality, Fontan failure, systemic ventricular function, supraventricular arrhythmias, thromboembolism, and growth potential. Age was 3 to 31 years (mean ± standard deviation, 9.4 ± 1.8; median, 7 years). The conduits were constructed of polytetrafluoroethylene (n = 50), and “viable” in situ pericardium (n = 15). The patients underwent serial echocardiogram, dynamic radionuclide studies, and cardiac catheterization. Results: Operative mortality was 3%, and the incidence of conduit thrombosis was 4.6%. There was paradoxic filling of the right lung after femoral injection of the radiotracer in all cases of conduit obstruction. Perioperative and late postoperative supraventricular arrhythmias were observed in 9.2% and 4.7% of patients, respectively. Risk factors for supraventricular arrhythmias included systemic ventricular dysfunction (p = 0.000), heterotaxy syndrome (p = 0.008), systemic venous anomalies (p = 0.015), and previous bidirectional Glenn operation (p = 0.017). At a mean follow-up of 77 ± 2 months (range, 8 to 79 months), there were no late deaths (actuarial survival at 79 months, 96.9% ± 0.02%). Serial echocardiograms demonstrated evidence of growth of the viable tunnels. Postoperatively, there was transient depression of ejection fraction in all patients (p = 0.000). Conclusions: Supraventricular arrhythmias after extracardiac Fontan are more common in patients with heterotaxy syndrome, bilateral superior venae cavae, systemic ventricular dysfunction, and those undergoing completion Fontan operation. The viable tunnel may emerge as an optimal alternative by virtue of reduction of supraventricular arrhythmias, elimination of the need for anticoagulation, and addressing the issue of growth potential in selected patients. [Copyright &y& Elsevier]

Background: The purpose of this study is to assess the mid-term results of aortic valve replacement with the pulmonary autograft. Methods: From October 1993 through September 2003, 153 patients with aortic valve disease (81 rheumatic and 72 non-rheumatic), with a mean age of 28 ± 14.2 years underwent the Ross procedure with root replacement technique and right ventricular outflow tract reconstruction using a homograft. Associated procedures included mitral valve repair (n = 19), open mitral commissurotomy (n = 15), tricuspid valve repair (n = 2), homograft mitral valve replacement (n = 2), and subaortic membrane resection (n = 1). Results: Early mortality was 6.5% (10 patients). Mean follow-up was 77 ± 42 months (range, 7 to 132 months; median, 90 months). One hundred, twenty-one survivors (84.6%) had no significant aortic regurgitation. Reoperation was required in 10 patients for autograft dysfunction alone (n = 3), infective endocarditis (n = 2), autograft dysfunction with failed mitral valve repair (n = 3), and failed mitral valve repair alone (n = 2). No reoperations were required for the pulmonary homograft. There were 8 late deaths. Actuarial and reoperation-free survival at 90 months were 91.% ± 3.5%, 95.3% ± 2.7%, in non-rheumatics and 86.1 ± 3.9%, 90.5 ± 3.7% in rheumatics, respectively. Freedom from significant aortic stenosis or regurgitation was 91.5 ± 2.8% in non-rheumatics and 80.6 ± 4.8% in rheumatics. Event-free survival was 86.2 ± 4.9% in non-rheumatics and only 68.9 ± 5.3% in rheumatics. Conclusions: The Ross procedure is not recommended for young patients (&#60; 30 years) with rheumatic heart disease. It provides satisfactory hemodynamic and clinical results in properly selected patients. Important autograft dilatation was not observed in our patients. [Copyright &y& Elsevier]

Background: The aim of this study is to assess the long-term results of aortic valve repair in patients with rheumatic aortic valve disease. Methods: From April 1991 through December 2003, 61 patients with rheumatic aortic valve disease underwent aortic valve repair. Mean age was 23.7 ± 9.3 years (range, 6 to 53 years). Thirty-nine (63.9%) patients were in New York Heart Association functional class III. Reparative procedures included cuspal thinning (n = 59), commissurotomy (n = 45), subcommissural annuloplasty (n = 24), commissural plication (n = 12), perforation closure using pericardium (n = 2), and decalcification of cusps (n = 2). Associated procedures included mitral valve repair (n = 36) and tricuspid valve repair with mitral valve repair (n = 5). Results: Early mortality was 4.9% (3 patients). Mean follow-up was 93.8 ± 46.4 months (range, 6 to 160 months, median, 103 months). Forty-six survivors (65%) had no or trivial or mild aortic regurgitation. Four patients required reoperation for valve dysfunction. There were no late deaths. Actuarial and reoperation-free survival, at 160 months, was 95.2% ± 2.8% and 85.4% ± 6.7%, respectively. Freedom from significant aortic stenosis or regurgitation was 52.4% ± 16.9%. Conclusions: Aortic valve repair in patients with rheumatic aortic valve disease is feasible and yields gratifying long-term results. [Copyright &y& Elsevier]

Background: Subaortic membrane (SAM) is a form of fixed subaortic obstruction in which a fibrous membrane is located below the aortic valve. Aim: To determine the role of surgical treatment for patients with a discrete SAM. Patients and Methods: The hospital records of 45 patients (age range: 2–23 years; median 8 years) undergoing surgery for SAM between 1990 and 1998 at the All India Institute of Medical Sciences, New Delhi, India, were analysed. Preoperative echocardiographically calculated gradients across the left ventricular outflow tract ranged from 50 to 154 mmHg (mean: 86.5 ± 33.2 mmHg). Nine patients had trivial aortic regurgitation (AR), 10 had mild AR and five had moderate–severe AR. The left ventricular ejection fraction (LVEF) ranged from 20 to 68% (mean 48 ± 15%). Nineteen patients had significant left ventricular dysfunction (LVEF <50%). Transaortic resection of SAM was done in all patients along with excision of a wedge-shaped segment of septal muscle underlying the membrane. Results: There were no early or late postoperative deaths. On follow up (up to 113 months), only four patients had gradients >30 mmHg. LVEF improved to 45–70% (mean 58 ± 7.7%). AR reduced to mild in four patients and trivial in four patients, and did not progress further. Conclusion: Resection of SAM carries long-term benefits. Routine septal myectomy appears to be associated with a low risk of recurrence. (Heart, Lung and Circulation 2001; 10: 130–135). [ABSTRACT FROM AUTHOR]

De novo noninfective thrombus formation in the ascending aorta is rare. We report two cases of ascending aortic thrombus, their surgical management, and cardiopulmonary bypass strategy. [ABSTRACT FROM AUTHOR]

Compression of the right pulmonary artery is unusual. We describe a patient with a double-outlet right ventricle, a ventricular septal defect, and pulmonary stenosis in whom the right pulmonary artery was compressed by a right-sided aortic arch. The condition was successfully managed during surgical correction. [Copyright &y& Elsevier]

An interatrial communication is essential for adequate mixing and survival in cases of total anomalous pulmonary venous connection. We report a 5-month-old infant with total anomalous pulmonary venous connection (cardiac type) without an interatrial communication and a large ventricular septal defect. [ABSTRACT FROM AUTHOR]

Abstract Objectives We conducted a prospective randomized controlled trial to compare del Nido (DN) and histidine-tryptophan-ketoglutarate (HTK) cardioplegia solution in pediatric patients undergoing intracardiac tetralogy of Fallot repair. Methods One hundred consecutive patients 12 years of age or younger, undergoing intracardiac repair of tetralogy of Fallot were randomized into DN (n = 50) and HTK (n = 50) groups. Cardioplegia strategy consisted of a single dose of DN (20 mL/kg) or HTK (6 mL/kg/min for 6 minutes). Primary outcome was cardiac index (CI). Secondary outcomes were ventricular arrhythmias post cross-clamp release, time to peripheral rewarming, duration of mechanical ventilation, inotropic score, intensive care unit and hospital stay, and serum levels of troponin-I, interleukin-6, and tumor necrosis factor-α. Ultrastructural changes in the myocardium were assessed. Results CI was significantly higher in the DN group compared with the HTK group at 6 (P =.005) and 24 hours (P <.001) after surgery. It was on an average 0.44 L/min/m2 higher in the DN group at any time point (P =.004). Time for complete cessation of electrical activity was longer in the HTK group (P =.01) and more patients in the HTK group had ventricular arrhythmias post cross-clamp release (P =.03). Duration of mechanical ventilation (P =.006), intensive care unit stay (P =.05), and hospital stay (P <.001) were lower in the DN group. Patients in the DN group had lower troponin I levels 24 hours after cardiopulmonary bypass (P <.001). Electron microscopic studies showed more myocardial edema (P =.02) and myofibrillar disarray (P =.04) in the HTK group along with lower glycogen stores (P =.04). DN cardioplegia was more cost-effective than HTK cardioplegia (P <.001). Conclusions DN cardioplegia was associated with better preservation of CI, less duration of mechanical ventilation, shorter intensive care unit and hospital stays, lower inotropic scores, and less release of troponin-I. Electron microscopy showed less myocardial edema and better preservation of the myofibrillar architecture and glycogen stores in the DN group. [ABSTRACT FROM AUTHOR]

Background Decreases in serum total thyroxin and total triiodothyronine occurs after cardiopulmonary bypass, and is reflected as poor immediate outcome. We studied effects of oral thyroxin supplementation in infants who underwent open-heart surgery. Methods In this prospective study, 100 patients were randomized into 2 groups: 50 in the thyroxin group (TH) and 50 in the placebo group (PL). Patients in the TH group received oral thyroxin (5 μg/kg) 12 hours before surgery and once daily for the remainder of their intensive care unit (ICU) stay. Data on intraoperative and postoperative variables were recorded. Cardiac index (CI) was measured. Perioperative serum thyroid hormone levels and serum interleukin-6 and tumor necrosis factor-α were measured. Secondary analysis was performed by dividing patients into simple and complex subcategories. Results Results of the primary analysis indicated a higher CI in the TH compared with the PL. In the complex category, the mean duration of mechanical ventilation was 3.85 ± 0.93 and 4.66 ± 1.55 days in the TH and PL, respectively ( P = .001). Mean ICU stay was 6.79 ± 2.26 and 8.33 ± 3.09 days ( P = .03), and mean hospital stay was 15.70 ± 4.77 and 18.90 ± 4.48 days ( P = .01) in the TH and PL, respectively. There were no significant differences between the TH and the PL in the simple category. CI was higher in the TH at all time points ( P = .004). The average therapeutic intervention scoring system scores for the first 2 days were higher in the PL in the complex category. Conclusions Oral thyroxin supplementation improves the CI and reduces the inotropic requirement. In addition, it reduces the duration of mechanical ventilation, ICU and hospital stay, and therapeutic intervention scoring system in infants after surgery for complex congenital heart defects. [ABSTRACT FROM AUTHOR]