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The objective of this study was to determine medium-term morbidity and mortality of patients who have undergone device closure of an extracardiac Fontan fenestration with an Amplatzer Vascular Plug II (AVPII) or Septal Occluder (ASO). A secondary objective was to compare medium-term morbidity and mortality between these patients and other fenestrated Fontan patients. A retrospective chart review was performed on patients who underwent an extracardiac fenestrated Fontan procedure between 1992 and 2015 at Cardinal Glennon Children's Medical Center. Procedural and follow-up data were obtained and compared between those who underwent fenestration closure and those who did not. Additional outcome measures included whether the fenestration had spontaneously closed, morbidity and mortality, oxygen saturations, and hemodynamics pre- and post-closure. Fifty-nine of 118 patients (50%) with a fenestrated Fontan underwent 60 device closures of the fenestration. Thirty-two (53%) of these were with the AVPII and 28 (47%) with the ASO. There was one device embolization. At a median follow-up of 3.9 years, five patients suffered morbidity, including 2 with arrhythmias, 1 with plastic bronchitis, 1 with protein losing enteropathy, and 1 with stroke. There were no cardiopulmonary deaths in this group. Twenty-three of 118 patients (19%) had spontaneous closure. There was no difference in morbidity and mortality between patients who underwent percutaneous fenestration closure and those who either had spontaneous closure or a persistently patent fenestration. Device closure of Fontan fenestrations is a safe and effective procedure with minimal morbidity and mortality comparable to other patients with fenestrations.

Background: Bidirectional Glenn shunt (BDG) failure carries high morbidity and mortality but the clinical factors associated with failure and the optimal management strategy are understudied. Methods: A total of 217 patients undergoing BDG at our institution between 1989 and 2020 were retrospectively reviewed and categorized as success or failure. Failure was defined as the need for reoperation (BDG takedown, reoperation for correction of cardiac defect, and/or transplantation) at any time postoperatively; operative mortality (death attributable to BDG malfunction occurring during the index hospitalization for BDG or within 30 days of discharge); or late mortality (death directly attributable to BDG malfunction occurring prior to Fontan or next-stage palliation). Univariate and binary logistic regression analyses were performed. Results: BDG failure occurred in 14 (6.5%) patients. Univariate predictors were: hypoplastic left heart syndrome ( P = .037), right ventricular (RV) dominance ( P = .010), greater pre-BDG pulmonary vascular resistance (PVR) ( P = .012), concomitant atrioventricular valve repair ( P = .020), prolonged pleural drainage ( P = .001), intensive care unit ( P

Heart transplantation for children with congenital heart disease is a challenging procedure that requires significant preoperative planning and occasionally some intraoperative ingenuity. Failed single ventricle palliation has become the most common indication for heart transplantation in the diagnostic group of congenital heart disease. Reconstructive procedures in addition to the transplant procedure itself are often necessary, particularly in patients with single ventricle anomalies. Situs inversus with dextrocardia is probably the most challenging diagnosis for transplantation.

Retroesophageal course of the left brachiocephalic vein is a rare variant seen in patients with congenital heart disease. However, this anomaly without associated cardiac or aortic abnormalities is nearly unheard of, with only one prior case described in the literature. We present an infant with anomalous retroesophageal left brachiocephalic vein that was an incidental finding on computed tomography (CT). We also briefly discuss its embryologic and clinical significance.

Unicuspid aortic valves (Sievers type 2 bicuspid) are characterized by major fusion and clefting of the right-left coronary commissure, and minor fusion of the right-noncoronary commissure. Repair has been difficult because of two fusions, variable relative sinus sizes, and peripheral leaflet deficiencies or tears after balloon valvuloplasty.Twenty unicuspid aortic valves patients underwent valve repair in nine institutions. Right-left major fusion and right-noncoronary minor fusion occurred in 17 of 20 (85%). Commissurotomy was performed on the minor fusion, and a bicuspid annuloplasty ring with circular base geometry and two 180-degree subcommissural posts was sutured beneath the annulus, equalizing the annular circumferences of the fused and nonfused cusps. The nonfused leaflet was plicated, and the cleft in the major fusion was closed linearly until leaflet effective heights and lengths became greater than 8 mm and equal, respectively.Average age (mean ± SD) was 22.3 ± 12.3 years (range, 13 to 58), 12 of 20 (60%) were symptomatic, 10 of 20 (50%) required aortic aneurysm resection. Pre-repair hemodynamic data included mean systolic valve gradient 25.8 ± 12.9 mm Hg, aortic insufficiency grade 2.9 ± 1.2, and annular diameter 24.7 ± 3.3 mm. No mortality or major complications occurred. Post-repair annular (ring) size was 20.5 ± 1.3 mm, mean gradient fell to 16.2 ± 5.9 mm Hg, and aortic insufficiency grade decreased to 0.1 ± 0.3 (P.001). At an average follow-up of 11 months (range, 1 to 22), all 20 patients were asymptomatic and had returned to full activity.Aortic ring annuloplasty reduced annular diameter effectively, recruiting more leaflet to midline coaptation. Minor fusion commissurotomy and annular remodeling to 180-degree commissures converted UAV repair to a simple and reproducible procedure.

This chapter summarizes the anatomy, biology, pathophysiology, surgical treatment, and outcomes in patients with interrupted aortic arch and ventricular septal defect. Emphasis is placed on diagnostic assessment and surgical decision making in the presence of varying degrees of left ventricular outflow tract obstruction. The hybrid technique for high-risk patients with interrupted aortic arch with ventricular septal defect is also summarized. Finally, early and late postoperative complications and outcomes are included.

Giant aneurysm of the right atrial appendage is an exceedingly rare congenital abnormality. Only eight previous cases of right atrial appendage aneurysm (RAAA) present in neonates could be identified in the literature to date. The etiology and natural history of this condition remains poorly understood. Here we present the case of a giant RAAA diagnosed in utero at 20 weeks gestation and which enlarged steadily until birth. The condition was corrected surgically by aneurysmectomy at three weeks of age.

We describe a case of type A aortic dissection in a 25-year-old woman who had undergone a Ross procedure for aortic valve endocarditis 13 years previously. She was pregnant and noted to have significant enlargement of the aortic root during the latter portion of the third trimester of her pregnancy. An echocardiogram after delivery demonstrated new aortic valve insufficiency in addition to an aortic root diameter of 6.5 cm. A computed tomography angiogram demonstrated a type A aortic dissection involving only the very proximal portion of the ascending aorta. At operation, the dissection was found to be limited to the pulmonary autograft. This was repaired using a valve-sparing technique. Her postoperative course was uneventful, and the aortic valve has shown only trace insufficiency at 3 years of follow-up.

Retroesophageal course of the left brachiocephalic vein is a rare variant seen in patients with congenital heart disease. However, this anomaly without associated cardiac or aortic abnormalities is nearly unheard of, with only one prior case described in the literature. We present an infant with anomalous retroesophageal left brachiocephalic vein that was an incidental finding on computed tomography (CT). We also briefly discuss its embryologic and clinical significance.

Cardiac intervention remains controversial in patients with trisomy 13 and 18 and little is known about factors that may affect outcomes. The goal of this study was to evaluate preoperative factors and surgical approach with respect to outcomes in these patients. Patients with congenital heart disease and trisomy 13 or 18 presenting to our institution from 2004 through 2015 were retrospectively reviewed. Patients were grouped into complete intervention, palliated intervention, and non-intervention. Pre-intervention variables, timing and type of intervention, post-intervention outcomes, and survival were recorded and comparisons were made between the groups. Of 34 patients, 18 cardiac interventions were performed. Complete repair was performed in 11(61%) and palliation in 7(39%). Median age for complete repair was 9.2 vs. 1.7 months in palliated patients (p

The purpose of this study is to compare morbidity and mortality between fenestrated (F, 61 patients) and nonfenestrated (NF, 54 patients) extracardiac Fontan patients during two eras from July 1995 to December 2010: era 1(1995 to 2004) and era 2 (2005 to 2010).Variables evaluated included morphology, hemodynamics, chest tube volume and duration, intensive care and hospital stay, oxygen saturation, neurologic events, rhythm, and readmissions for chylous effusions. Follow-up in 114 hospital survivors was longer in the nonfenestrated cohort (F, 5.0 ± 3.3 years; NF, 7.1 ± 4.6 years; p0.005).Cohorts were similar in body size, morphology, and hemodynamics. Fenestration in hypoplastic left heart syndrome was appreciatively higher in era 2. Bypass time (F, 69 ± 27 minutes; NF, 57 ± 21 minutes) and conduit size (F, 18.8 mm; NF, 19.1 mm) were similar. There was 1 early nonfenestrated Fontan death (1 of 54; 2%) and 4 late deaths (F, 2 of 61, 5%; NF, 2 of 53, 4%; p = 0.86). Room air saturation was higher in NF patients (F, 89%; NF, 94%; p0.05). Total chest tube volume was similar, but fenestration was associated with greater chest tube drainage among hypoplastic left heart patients (HLHS, 5,582 ± 3,286 mL; non-HLHS, 3,405 ± 2,533 mL; p = 0.06; odds ratio; 2.0). Readmission to treat chylous effusions, loss of sinus rhythm, actuarial freedom from death, all neurologic events, pacemaker insertion, and Fontan takedown were similar in both cohorts.Fenestration was associated with lower discharge oxygen saturations, but late outcomes in fenestrated and nonfenestrated patients are equivalent.

The optimal surgical treatment of patients with transposition of the great arteries, ventricular septal defect, and pulmonary stenosis is controversial. Although the Rastelli operation has been standard surgical management of this lesion, aortic root translocation with right ventricular outflow tract (RVOT) reconstruction (Nikaidoh) and the pulmonary artery translocation (Lecompte) or REV (réparation a l'étage ventriculaire) are surgical alternatives more recently introduced to treat this complex lesion. This report reviews our 20-year experience with the Rastelli procedure and attempts to compare our outcomes with those recently published using the Nikaidoh and REV procedures.Between 1988 and 2008, 40 patients (median age, 4 years; range, 9 months to 17 years) underwent Rastelli operation at our institutions. The RVOT was obstructed in 32 and atretic in 8. Follow-up was available for all but one patient (mean follow-up, 8.6±5.6 years). The RVOT was reconstructed with homograft (n=25), bovine jugular vein (n=8), nonvalved Dacron tube (n=5), or a porcine valved conduit (n=2). Two patients required a pacemaker.There were no early, but three late deaths and one heart transplantation 12 years postoperative the Rastelli operation. Kaplan-Meier survival was 93% at 5, 10, and 20 years. Univariate risk factors for death or transplantation included surgery before 1998 (p=0.03) and concomitant noncardiac anomalies (p=0.001). Sixteen patients (40%) had reoperation for right ventricular-pulmonary artery conduit stenosis (mean, 7.8±3.8 years) without mortality. Freedom from conduit replacement was 86%, 74%, 63%, and 59% at 5, 10, 15, and 20 years, respectively. Multivariate analysis revealed that the risk factors of conduit replacement were younger age at operation (p=0.001) and surgery before 1998 (p0.001). Two patients (5%) required reoperation for left ventricular outflow tract obstruction. At follow-up, there were no sudden unexplained deaths, and New York Heart Association functional class is I or II.The Rastelli procedure is a low-risk operation with regard to early and late mortality and reoperation for left ventricular outflow tract obstruction. Conduit change operations will be required in most patients regardless of the technique of repair, but currently can be performed with low morbidity and mortality. These midterm outcomes after the Rastelli operation should serve as a basis for comparison with surgical alternatives more recently introduced for transposition of the great arteries and ventricular septal defect with RVOT obstruction.

Our goal is to demonstrate the utility of the calculus of moving surfaces (CMS) in boundary variation problems. We discuss the relative advantages of the CMS compared to the alternative approach of interior variations. We illustrate the technique by calculating the two leading terms of a power series for the Laplace eigenvalues on an ellipse with semi-axes 1 + a and 1 + b, where a and b are small. We compare the CMS estimates with those obtained by the conventional finite element method with Richardson extrapolation. The comparison confirms the cubic rate of convergence for the CMS estimates.

Goel N, Huddleston CB, Fiore AC. A novel mutation of the MYH7 gene in a patient with hypertrophic cardiomyopathy. Turk J Pediatr 2018; 60: 315-318. Hypertrophic cardiomyopathy (HCM) is a genetic disorder characterized by asymmetric cardiac hypertrophy due to inherited mutations in genes that encode sarcomeric proteins. MYH7, which encodes β-myosin heavy chain, is among the most commonly mutated genes in patients affected by HCM. We aimed to identify the specific mutation responsible for HCM in a six-month old Caucasian patient. NextGen DNA sequencing revealed a novel p.Ala1328Thr (A1328T) mutation of MYH7 in the affected patient as well as his asymptomatic father and asymptomatic brother. The clinical details of this mutation are described for the first time in this report. The genetic variant affects a residue that is highly conserved across species. Theoretical analysis suggests that A1328T is very likely deleterious to β-myosin heavy chain protein structure and function. Furthermore, this novel mutation was not observed with any significant frequency in approximately 6,500 healthy individuals of European and African American ancestry in the NHLBI Exome Sequencing Project, underlining the potential pathogenicity of this variant.

Objective: We review our experience with relief of subaortic obstruction in univentricular hearts following pulmonary artery banding (PAB) with double barrel Damus—Kaye Stansel procedure (DKS) and subsequent staged palliation to Fontan. The purpose was to determine if PAB alters semilunar valve function after the double barrel DKS procedure and if this staged approach negatively influences the achievement of Fontan palliation. Methods: From January 1990 to March 2006, 27 patients underwent PAB (mean 22 days, range 1—150 days; 3.4 kg) and coarctation as corrected simultaneously in 18 (18/27) 66%. These 27 patients subsequently had PA debanding and double barrel DKS connection at a mean age of 10.2 months (range 0.3—58 months). Pulmonary flow was established with a bidirectional Glenn in 14 patients; modified Blalock in 6, Glenn with modified Blalock in 5 and completion Fontan in 2 patients. Results: There were six early deaths (22%) following DKS: four patients receiving DKS with systemic shunt and two receiving bidirectional Glenn and systemic shunt. Patients receiving DKS with bidirectional Glenn shunt had a significantly lower mortality than patients who had a DKS with systemic shunt alone or in combination with a Glenn (p < 0.03). Single ventricle to aortic gradient was reduced from 27.5 18 mmHg to 3.4 2 mmHg following double barrel DKS procedure (p < 0.001). Aortic and pulmonary insufficiency was trace to mild in all patients. Nineteen of 21 survivors (90%) have completed Fontan with no early and three late deaths. Two patients are completion Fontan candidates. Conclusions: PAB (coarctation repair) with interval double barrel DKS is effective palliation for univentricular heart and excessive pulmonary blood flow. PAB does not create significant pulmonary insufficiency and subsequent DKS effectively relieves single ventricle to aortic gradient. Optimal second stagepulmonary bloodflow is usuallyestablished with a bidirectionalGlenn. The need for a Blalock shunt or a Glenn plus a Blalock is associated with increased mortality. # 2008 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

A 14-month-old boy with a structurally normal heart presented with signs of cardiac tamponade caused by purulent pericarditis. During his hospital stay, mitral and tricuspid valve endocarditis developed, and a ventricular septal abscess expanded despite appropriate, prolonged antibiotic therapy for methicillin-resistant Staphylococcus aureus. The day before scheduled surgical correction, the abscess ruptured, creating a septal aneurysm. Surgical intervention resulted in an excellent outcome. Throughout the patient's 67-day hospitalization, the use of echocardiography was crucial in monitoring and diagnosis.In addition to reporting this case, we discuss our diagnostic and treatment considerations. To our knowledge, this is only the 4th report of S. aureus bacterial pancarditis with myocardial abscess.

Apical aortic conduits were used in children with complex left ventricular outflow tract obstruction at our institution before we adopted the Ross and Ross-Konno procedures. The early results with apical aortic conduits were quite good when we used a porcine xenograft conduit and were less satisfactory when we converted to an aortic homograft valve as the conduit valve. This report summarizes our clinical experience with apical aortic conduits in children with an emphasis on hemodynamic results, reoperation, and long-term follow-up.Records of 28 patients (age range, 2 weeks to 19 years) who underwent insertion of apical aortic conduit between September 1979 and June 1993 were reviewed. All patients had complex multilevel left ventricular outflow tract obstruction. All were symptomatic, and 22 (79%) had had one or two previous aortic valvotomies or left ventricular outflow tract operations, or both.Hospital mortality was 11% (3 of 28). Twenty-five children survived the perioperative period and improved, and 21 have had one or more cardiac catheterization from 6 to 18 months (mean, 1.2 years) after the initial operation. Reduction or resolution of resting mean left ventricular-to-aortic peak gradient in the early postoperative period from 81.8 +/- 24.0 to 15.4 +/- 8.9 mm Hg was demonstrated (p0.001). Overall 25-year survival was 57%. Fourteen surviving patients (56%) have undergone subsequent procedures (n = 18) from 5 months to 16 years postoperatively (mean, 6.9 years) because they developed a recurrent left ventricular-to-aortic gradient of 58 +/- 28 mm Hg (p0.002). One patient underwent heart transplantation. All other late survivors have normal left ventricular function as determined by serial echocardiography.Apical aortic conduit is effective in relieving complex left ventricular outflow tract obstruction and improving left ventricular performance with acceptable short-term and midterm results, but late complications caused primarily by conduit tissue valve dysfunction are frequent in children. Since the early 1990s, the apical aortic conduit procedure has been largely replaced with the Ross or Ross-Konno procedure in our pediatric practice.

A 9-year-old boy was found to have ruptured sinus of Valsalva aneurysm (RSVA) and aortic coarctation. Following relief of aortic coarctation by balloon angioplasty, transcatheter coil occlusion of the RSVA was performed successfully under transesophageal echocardiographic and fluoroscopic monitoring; bioptome-assisted delivery of 0.052″ Gianturco coil was undertaken via a 7 Fr sheath stabilized by an 0.035″ guidewire passing through the RSVA and the sheath. This report details the technique of occlusion. Cathet Cardiovasc Intervent 2003;58:130–134. © 2003 Wiley-Liss, Inc.

Nonvalve transannular patch repair of right ventricular outflow tract obstructive (RVOTO) defects results in pulmonary insufficiency, which can contribute to early postoperative right ventricular dysfunction. In both animal and clinical studies, monocusp valves provide perioperative RVOT competence and improved right ventricular functional characteristics. In these reports, monocusp leaflet construction with 0.1-mm polytetrafluoroethylene (PTFE) appeared equal, or superior, to biologic monocusp valves. Construction of the polytetrafluoroethylene monocusp valve is an inexpensive and straightforward way to create a competent RVOT in a variety of RVOTO anomalies. Based on our clinical experience, it effectively prevents short-term and significantly reduces midterm pulmonary insufficiency without evidence of stenosis.

Background: Tracheomalacia with anterior great vessel compression is a common disorder in infants and children, which can lead to life-threatening airway occlusion. In this study, a large number of patients underwent anterior aortopexy to provide a more normal distal airway. Methods Thirty-two infants and children with tracheomalacia associated with esophageal atresia-tracheoesophageal fistula (18), vascular ring (8), abnormal innominate artery takeoff (4), and primary (2) were evaluated with bronchoscopy, magnetic resonance imaging, and pulmonary functions. Aortopexy was accomplished through left thoracotomy and suture fixation of the aorta and innominate artery to the posterior sternum. Results Intraoperative bronchoscopy showed marked improvement in airway caliber and rigidity, and no patients had further obstructive episodes. Forced expiratory volume improved from 52% ± 4% of predicted to 82% ± 3%. Conclusions Aortopexy is a simple procedure for the treatment of distal tracheomalacia that is immediately effective and provides permanent relief of obstructive episodes.

An athletic 15-year-old girl with aberrant left coronary artery from the right coronary sinus, presented with syncope during exercise. Trans-thoracic echocardiography was the only imaging technique that clearly demonstrated her anomaly. The results of magnetic resonance and selective coronary angiographic imaging were inconclusive. Surgical intervention was successfully performed on the basis of the echocardiographic diagnosis.

Background . Major leg wound complications after coronary artery bypass graft procedures are infrequent and few are reported in the literature. We present our experience in treating 23 patients with major leg wound complications after coronary revascularization procedures. Methods . A retrospective review of 3,525 bypass procedures with saphenous vein grafts performed over a 10-year period was conducted. Ten potential risk factors for those who developed major leg wound complications were analyzed and compared with the entire cohort of patients undergoing similar bypass procedures during the same period. Results . Lower extremity wound complications occurred in 145 patients (4.1%), 23 of whom (0.65%) required additional surgical interventions (62 total). There were 32 wound debridements, 8 skin grafts, 11 vascular procedures, 5 amputations, 3 fasciotomies, 2 free tissue transfers, and 1 fasciocutaneous flap. Of ten variables evaluated by multivariate analysis, female gender, peripheral vascular disease, and postoperative intraaortic balloon pump use were identified as significant independent predictors of major leg wound complications ( p Conclusions . The causes of major leg wound complications after saphenous vein harvest for coronary artery bypass graft procedures are multifactorial. To minimize these complications, we recommend vascular evaluations before saphenous vein harvest, attention to proper surgical technique, and careful harvest site selection.

Background . Ventricular assist devices (VADs) have gained wider acceptance due to refinements in patient selection and management and device availability. Methods . To evaluate early and late results, we reviewed our first 111 patients with the Thoratec VAD. Results . Forty-four patients were supported for myocardial recovery. The mean age in the recovery group was 51.9 years. There were 18 left VADs (LVADs), 17 biventricular VADs (BVADs), and nine right VADs (RVADs). Complications included bleeding in 20 patients (45%) and device-related infection in 1 patient (2%). Nineteen were weaned from the VAD, with 12 survivors. Sixty-seven patients were supported as a bridge to cardiac transplantation. The mean age was 41.5 years. There were 39 LVADs and 28 BVADs. Complications included bleeding in 21 patients (31%) and device-related infection in 12 (18%). Three patients were weaned and 39 patients were transplanted from the assist device, for a total of 42 bridge survivors. Device-related thromboembolism occurred in 9 patients (8.1%), 7 of whom were bridge to transplantation. The duration of VAD support ranged from 0.1 to 27 days (mean 4.5 days) in the recovery group and 0.2 to 184 days (mean 40.7 days) in the bridge to transplantation group. The 10-year actuarial survival was 16% for the recovery group, 22% for the bridge group, and 33% for transplanted patients. Conclusions . Despite advances, VAD support remains associated with significant morbidity and operative mortality.

To better define the merits of the bileaflet and tilting-disc valves, we prospectively randomized 102 patients whose age, 57 years; range, 11 to 85 years) to receive either the St. Jude (n = 55) or the Medtronic-Hall (n = 47) mitral valve prosthesis between September 1986 and May 1991. The two groups were not different with respect to preoperative New York Heart Association class, incidence of mitral stenosis and insufficiency, angina score, extent of coronary artery disease, ventricular function, completeness of revascularization, or cross-clamp or bypass time. The hospital mortality (14.5% versus 10.6%, St. Jude versus Medtronic-Hall) and late mortality (7.3% versus 2.1%) were not significantly different. Follow-up was complete in 84 of 89 hospital survivors (94%) with a mean of 26 months (range, 1 to 60 months). The linearized rates of valve-related events and the 3-year actuarial survival demonstrated no significant differences between both cohorts. Comparison of the clinical outcome and echocardiographic parameters obtained at the time of follow-up demonstrated no significant differences between the two prostheses. These data indicate that the Medtronic-Hall and St. Jude mitral prostheses are similar with respect to their rates of valve-related complications and hemodynamic profiles. This study suggests that there is no difference between the St. Jude and Medtronic-Hall prostheses with regard to early clinical performance or hemodynamic results and therefore does not support the preferential selection of either prosthesis.

The ideal temperature for blood cardioplegia administration remains controversial.Fifty-two patients who required elective myocardial revascularization were prospectively randomized to receive intermittent antegrade tepid (29 degrees C; group T, 25 patients) or cold (4 degrees C; group C, 27 patients) blood cardioplegia.The two cohorts were similar with respect to all preoperative and intraoperative variables. The mean septal temperature was higher in group T (T, 29.6 degrees +/- 1.1 degrees C versus 17.5 degrees +/- 3.0 degrees C; p0.0001). After reperfusion, group T exhibited significantly greater lactate and acid release despite similar levels of oxygen extraction (p0.05). The creatine kinase-MB isoenzyme release was significantly lower in group T (764 +/- 89 versus 1,120 +/- 141 U x h/L; p0.04). Hearts protected with tepid cardioplegia demonstrated significantly increased ejection fraction with volume loading, improvement in left ventricular function at 12 hours, and decreased need for postoperative inotropic support (p0.05). The frequency of ventricular defibrillation after cross-clamp removal was lower in this cohort (p0.05). There were no hospital deaths, and both groups had similar postoperative courses.Intermittent antegrade tepid blood cardioplegia is a safe and efficacious method of myocardial protection and demonstrates advantages when compared with cold blood cardioplegia in elective myocardial revascularization.

This report describes the technique to fenestrate the extracardiac Fontan conduit without cardiopulmonary bypass in a patient with levocardia and atrial situs inversus.

Objective: The ideal prosthesis for aortic valve replacement in patients with small annuli remains controversial and has yet to be identified. The purpose of this report is to compare the St. Jude (SJ) Medical and Medtronic Hall (MH) valves for aortic valve replacement in the small aortic root. Methods: From 1986 to 1994 we prospectively randomized 456 patients to receive either the SJ or the MH valve. From this population, 80 patients (SJ, 42 patients; MH 38 patients) had a 19 or 21 mm aortic prosthesis inserted without annulus enlarging procedure. Results: Follow-up was complete in all 80 patients for 270 patient years (mean 40.5 months). Analysis showed that the SJ and MH groups were similar with respect to age, gender, body surface area, valve area, NYHA class, ventricular function, prosthesis size, frequency of revascularization, bypass and global ischemic time. There were two operative deaths (1 SJ, 1 MH). Clinical performance and Dobutamine stress transesophageal doppler echocardiography could not demonstrate a significant advantage of one prosthesis over the other in this population. The change in aortic valve gradient, and left ventricular mass index measured preoperatively and within 12 months postoperatively were not different in both cohorts. Conclusion: The study could not detect a difference in the performance of the SJ and MH heart valves for aortic valve replacement in patients with small aortic annuli.

Left ventricle to aortic conduits were used for the treatment of complex left ventricular outflow tract obstruction in the pediatric population in the mid-1970s. Although this technique has been largely replaced by the Ross-Konno procedure, many patients still have functioning apicoaortic conduits in place today. Few clinical reports or case series exist in pediatric cohorts documenting the natural history or potential long-term complications of this prosthesis. In this report, we describe our experience managing a patient with Shone’s syndrome and an apical aortic porcine-valved conduit remnant that became infected 17 years postconduit valve excision for valvular insufficiency.

Objective Two different strategies have emerged in the initial palliation for hypoplastic left heart syndrome, the conventional Norwood operation and the so-called hybrid procedure. We have used each of these at our center. The purpose of the present study was to compare the outcomes of both procedures. Methods From 2007 to 2012, 40 patients presented to the Cardinal Glennon Children's Medical Center with hypoplastic left heart syndrome or 1 of its variants. Of the 40 patients, 24 underwent a hybrid procedure and 16 a Norwood procedure for initial palliation. The medical records, echocardiograms, and cardiac catheterization data were retrospectively reviewed. Standard statistical analysis was performed. Results The patients who underwent the hybrid procedure weighed less than those who underwent the Norwood procedure. Overall unadjusted survival was better in the Norwood group, although this did not reach statistical significance. Overall hospital resource usage was similar in both cohorts, taking into account both first and second palliation stages. Conclusions In our review, we found no statistically significant difference in survival or resource usage between those patients undergoing the Norwood procedure and those undergoing a hybrid procedure as initial palliation for hypoplastic left heart syndrome.

Heart transplantation in children with intellectual disability (ID) is an issue of debate due to the shortage of available donor organs. We sought to perform the first large-scale retrospective cohort study describing the prevalence and outcomes of heart transplantation in this population. The United Network of Organ Sharing database was queried from 2008 to 2015 for pediatric patients (age

Purpose: The purpose of this study was to assess the incidence of and predictors for vascular complications in patients who required perioperative intraaortic balloon pump (IABP) support. Methods: Data from 580 patients collected with a retrospective review were statistically analyzed with 25 perioperative parameters, and significant variables were evaluated with multivariate analysis. These data were also statistically compared with data from a 1983 study from our institution. Results: Vascular complications occurred in 72 patients (12.4%). The three aortic perforations were fatal. Ipsilateral leg ischemia occurred in 69 patients. Of these, ischemia was resolved in 82% of patients by IABP removal (21), thrombectomy (21), vascular repair (13), fasciotomy (2), or without intervention (2). Six patients died with the intraaortic balloon in place. Four patients required amputation for ischemia, but all survived. Conclusions: Vascular complications were not predictive of operative death (p = 0.26). Risk analyses with 25 perioperative parameters revealed that history of peripheral vascular disease, female sex, history of smoking, and postoperative insertion were independent predictors of vascular complications. However, most risk for vascular complications cannot be explained by these factors because of a low R 2 value. Compared with the results of our 1983 study, the incidence of IABP-related complications has not changed, but the severity of complications has decreased significantly, and IABP-induced death has decreased significantly. (J VASC SURG 1994;19:81-9.)

This report describes a case of partial anomalous pulmonary venous return in a young white girl for whom transcatheter intervention successfully replaced surgical correction. This intervention was feasible because the left lung had dual pulmonary venous drainage.

From March 1990 through January 1992, 108 patients undergoing myocardial revascularization had one (91) or both (17) inferior epigastric arteries (IEA) used for myocardial revascularization. The internal thoracic artery (ITA) was used bilaterally in 87 patients. Of the 373 distal anastomoses, 210 (56%) were with the ITA, 130 (35%) with the IEA, and 12 (3%) with the gastroepiploic artery. Nineteen patients (18%) received 21 saphenous veins. When compared with the ITA, the IEA demonstrated a longer harvest time (36.5 vs 29.6 min, P0.0001), a shorter usable length (11.9 cm vs 16.5 cm, P0.001), and similar flow (49.7 cc/min vs 48.7 cc/min, P = NS). The operative mortality was 2.8%. Two deaths resulted from low cardiac output and the one remaining death from complications of a cerebral vascular accident. The most common major complication was respiratory insufficiency, which occurred in 11 patients (12%). There were two sternal infections (2%), and two abdominal wound infections (2%), none of which were fatal. The IEA is an acceptable additional arterial conduit. It can be safely employed with one or both ITAs. Short-term and long-term patency must be established before preferential use of this conduit is advised.

Background This report reviews our 40-year experience with pediatric mitral valve replacement (MVR) with respect to mortality, valve-related morbidity, and reoperation risk factors. Methods From 1970 to 2010, 97 patients have undergone a total of 136 MVRs. Median age was 8 years (2 weeks to 18 years), 41 patients (42%) were less than 5 years, and 16 were infants (17%). Etiology was congenital in 65 patients (67%), rheumatic in 27 (28%), and endocarditis in 5 (5%). Regurgitation was the predominant lesion in 67 patients (69%), stenosis in 23 (24%), and mixed in 7 (7%) patients. Mechanical valves (ball, n=11; or bileaflet disc, n=66) and xenografts (porcine, n=14; bovine, n=2) were used in 93 initial MVR patients. Since 2002, 5 children have undergone Ross MVR with a pulmonary autograft in 3 and an aortic homograft in 2. Results Hospital mortality was 6% (6 of 97). There were 23 late deaths and 5 patients have required cardiac transplantation. Thirty-five year actuarial survival was 71%. Age less than 2 years, MVR prior to 1980, atrioventricular septal defect, univentricular heart, and additional left side obstructions were significant predictors of death. Mean follow-up was 12.8 ± 10.1 years (range, 2 months to 38 years). Seventeen patients with mechanical valves experienced systemic emboli in 9 (10%), valve thrombosis in 5 (6%), and bleeding requiring transfusion in 3 (3%) patients. Thirty-two patients required reoperations (35%) from 3 months to 14 years (mean, 6.5 ± 4.4 years) after initial MVR. Actuarial freedom from reoperation at 35 years was 63%. Variables associated with mitral re-replacement were younger age, small weight, valve diameter less than 23 mm, MVR prior to 1980, and type of implanted valves (xenograft, single-leaflet disk, ball-caged, or human valves). Conclusions Pediatric MVR can be performed with low initial mortality but should be reserved for medical and reconstruction failure because reoperation, valve-related complications, and late mortality are high. Bileaflet prostheses larger than 23 mm have the lowest reoperation risk. Ross MVR may offer select patients a durable tissue valve without lifelong anticoagulation and its associated complications.

Background We retrospectively reviewed the 12-year (1999 to 2010) clinical and echocardiographic performance of 232 bovine jugular vein conduits for extracardiac right ventricular outflow tract reconstruction in non-Ross patients. Methods The bovine jugular vein conduit cohorts, group 1 (12 to 14 mm), group 2 (16 to 18 mm), and group 3 (20 to 22 mm), had mean follow-up of 48 ± 30 months. Graft dysfunction is defined as right ventricular outflow tract obstruction with peak echo Doppler gradient greater than 40 mm Hg or grade 3/4 valve regurgitation. Graft failure is the need for conduit replacement or transcatheter or surgical reintervention. Results Early mortality (4 of 232; 2%) and late mortality (8 of 228; 3.5%) were not conduit related. Twenty-four conduits (10%) were explanted. Mean implant Z score was significantly lower for group 1 (1.7 ± 0.08 versus group 2, 2.7 ± 0.6, or group 3, 2.5 ± 1.5; p = 0.001). Ten-year actuarial survival (group 1, 84% versus 2, 100%, and 3, 99%; p = 0.001) and freedom from conduit dysfunction (group 1, 64%; group 2, 92%; and group 3, 90%) and failure (group 1, 75%; 2, 82%; and 3, 91%; p = 0.002) were significantly better for groups 2 and 3. Conclusions Bovine jugular vein is an excellent immediate substitute for right ventricular outflow tract reconstruction, with early durability superior to that of pulmonary homografts reported at similar follow-up. Conduits larger than 14 mm have improved performance. Longer follow-up will define the structural integrity and efficacy of this prosthesis.

We report the use of the hybrid procedure (bilateral pulmonary artery banding, ductal stenting followed by balloon atrial septostomy) in the first week of life for the treatment of two neonates with large left ventricular fibromas resulting in univentricular physiology. At 6 months of age, second-stage palliation was accomplished and the 2 patients are growing well with normal developmental milestones and no atrial or ventricular ectopy at 30 months. Hybrid strategy offers a less invasive, initial intervention and the options of typical second-stage univentricular palliation, conversion to biventricular physiology in cases of tumor regression, planned surgical resection, or transplantation.

The intraaortic balloon pump is usually the first mechanical device inserted for perioperative cardiac failure; however, little current information is available regarding short- and long-term effectiveness. From January 1983 through November 1990, 6856 adult patients underwent cardiac surgical procedures, 580 of whom (8.5%) had an intraaortic balloon inserted preoperatively (107 patients), intraoperatively (419 patients), or postoperatively (54 patients). There were 374 men and 206 women with a mean age of 63.9 years (range 19 to 88). Operations included 376 coronary artery bypass grafts, 100 mitral valve replacements (with or without bypass grafting), 70 aortic valve replacements (with or without bypass grafting), 15 double valve replacements (with or without bypass grafting), and 32 other procedures. There were 72 (12.4%) complications related to the balloon pump, of which 42 necessitated surgical intervention including thrombectomy (21), vascular repair (13), fasciotomy (2), aortic repair (1), and amputation (4). Operative mortality for patients supported by the balloon pump was 44%. Multivariate stepwise analysis of 27 parameters revealed six independent predictors of mortality: preoperative New York Heart Association class, transthoracic intraaortic balloon insertion (both p < 0.0001), preoperative administration of intravenous nitroglycerin, age, female gender, and preoperative balloon insertion (p < 0.001). Balloon-related complications were not predictive of death. Of the 326 hospital survivors, only 34 were lost to follow-up. There were 75 late deaths, the cause of which was cardiac in 41 (55%), noncardiac in 20 (27%), and unknown in 14 (19%). Actuarial survivals at 1, 5, and 9 years are 51%, 42%, and 33%. Of the 217 hospital survivors still alive and contacted, 81% were in class I (114) or II (60). These data demonstrate (1) operative mortality for patients requiring an intraaortic balloon in the perioperative period remains high, (2) perioperative risk factors can be identified, (3) complications related to the balloon pump do not affect survival, (4) operative survivors can achieve prolonged survival with excellent functional results, and (5) consideration for alternative methods of circulatory support is justified.

Chylothorax is a potentially life-threatening disorder that has profound respiratory, nutritional, and immunologic consequences. Treatment for this problem is controversial, and the results have been variable. From 1985 to 1990, 29 patients (16 males, 13 females; mean age: 20.1 years; range: 5 days to 76.8 years) were diagnosed as having chylothorax (18 right, 6 left, 5 bilateral). Etiologies included surgical trauma (26) and nonsurgical trauma (3). Initial conservative treatment consisted of tube thoracostomy drainage (mean duration: 13.3 days; range: 1 to 62 days; mean total volume: 4,030 mL) and dietary modification (low-fat diet, total parenteral nutrition). This resulted in resolution of the chylothorax in 23 patients (79% success), although 2 patients died of unrelated causes while hospitalized (myocardial infarction and cardiopulmonary arrest). Five adult patients and one infant (21%) required ligation of the thoracic duct, with resolution of the chylothorax in all six (100% success). Despite successful duct closure, one infant died of respiratory failure unrelated to the operation, and one adult died as the result of a cerebrovascular accident 6 weeks postoperatively, yielding an operative mortality of 33% and an overall mortality of 14% (4 of 29). Our experience demonstrates that initial treatment of chylothorax with thoracostomy drainage and dietary modification is successful in the majority of patients and is not associated with high morbidity or mortality rates. Surgical intervention for chylothoraces that fail to respond to initial conservative measures will be required in a minority of patients but appears to be associated with a higher risk of complications.

The role of retrograde coronary sinus cardioplegia in patients undergoing aortic valve replacement for aortic stenosis alone or in combination with myocardial revascularization has not been fully defined. Sixty-three patients undergoing elective aortic valve replacement received cold potassium blood cardioplegic solution via either the aortic root (36 patients) or the coronary sinus (27 patients). The patients were similar with respect to age, degree of aortic stenosis, ventricular function, severity of coronary artery disease, crossclamp time, completeness of revascularization, and mean volume and temperature of the infusion solution. The mean septal temperature and the release of myocardium-specific isoenzyme in the first 2 hours after crossclamp removal was higher in the retrograde group (p horac C ardiovasc S urg 1992;104:130–8)