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Regionalization of care has been proposed to optimize outcomes in Congenital Cardiac Surgery (CCS). We hypothesized that hospital infrastructure/systems of care factors could also be considered in regionalization efforts.Observed-to-expected mortality (O/E ratio) and hospital volumes were obtained between 2015 and 2018 from public reporting data. Using a resource dependence framework we examined factors obtained from American Hospital Association, Children's Hospital Association and hospital websites. Linear regression models were estimated with volume only, then with hospital factors, stratified by procedural complexity. Robust regression models were re-estimated to assess the impact of outliers.We found wide variation in the volume of congenital cardiac surgeries performed (89-3,920) and in the surgical outcomes (O/E ratio range 0.3-3.1). Six outlier hospitals performed few high complexity cases with high mortality. Univariate analysis including all cases indicated that higher volume predicted lower O/E ratio (β=-0.020, SE=0.008, p=0.011). However, this effect was driven by the most complex cases. Models stratified by Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) category show that volume is only a significantly predictor in category 5 cases (β=-1.707, SE=0.663, p=0.012). Robust univariate regression accounting for outliers found no effect of volume on O/E ratio (β =.005, SE =.002, p=.975). Elimination of outliers through robust multivariate regression decreased the volumes/outcomes relationship and found a modest relationship between health plan ownership and outcomes.Systems of care factors should be considered in addition to volume when designing regionalization in CCS. Patient level datasets will better define these factors.

Patients with congenital heart disease (CHD) that have surgical repair with cardiopulmonary bypass (CPB) reflect a unique population with multiple pulmonary and systemic factors that may contribute to increased alveolar dead space and low cardiac output syndrome. This study aimed to assess and compare changes in the alveolar dead space fraction (AVDSf) in the immediate postoperative period with outcomes in children with CHD who underwent repair on CPB. A single-center retrospective review study of critically ill children with CHD, younger than 18 years of age admitted to the Pediatric Intensive Care Unit (PICU) after undergoing surgical repair on CPB and received invasive mechanical ventilation for at least 24 h. One hundred and two patients were included in the study. Over the first 24 h, mean AVDSf was significantly higher in patients who had longer hospital length of stay (LOS) (> 21 days) p = 0.02, and longer duration of invasive mechanical ventilation (DMV) (> 170 h) p = 0.01. Cross-sectional analyses at 23–24 h revealed that AVDSf > 0.25 predicts mortality and DMV (p = 0.03 and P = 0.02 respectively); however, it did not predict prolonged hospital LOS. For every 0.1 increase in the AVDSf, the odds of mortality, DMV, and hospital LOS increased by 4.9 [95% CI = 1.45–16.60, p = 0.002], 2.06 [95% CI = 1.14–3.71, p = 0.01], and 1.43[95% CI = 0.84–2.45, p = 0.184], respectively. The area under the ROC curve at 23–24 h for AVDSf was 0.868 to predict mortality as an outcome. AVDSf > 0.25 at 23–24 h postoperatively was an independent predictor of mortality with sensitivity and specificity of 83% and 80%, respectively and was superior to other commonly used surrogates of cardiac output. In the immediate postoperative period of pediatric patients with CHD, high AVDSf is associated with longer hospital length of stay and duration of invasive mechanical ventilation. Increased AVDSf values at 23–24 h postoperatively is associated with mortality in patients with CHD exposed to CPB. Supplementary Information The online version contains supplementary material available at 10.1007/s00246-021-02674-2.

Infants undergoing congenital heart surgery (CHS) with cardiopulmonary bypass (CPB) are at risk of acute kidney injury (AKI) and fluid overload. We hypothesized that placement of a passive peritoneal drain (PPD) can improve postoperative fluid output in such infants. We analyzed 115 consecutive patients, age birth to 60 days, admitted to the PICU after CHS with CPB between 2012 and 2018. Patients who needed postoperative ECMO were excluded. Linear and logistic regression models compared postoperative fluid balances, diuretics administration, AKI, vasoactive-inotropic scores (VIS), time intubated, and length of stay after adjusting for pre/operative predictors including STAT category, bypass time, age, weight, and open chest status. PPD patients had higher STAT category (p = 0.001), longer CPB times (p = 0.001), and higher VIS on POD 1–3 (p ≤ 0.005 daily). PPD patients also had higher AKI rates (p = 0.01) that did not reach significance in multivariable modeling. There were no postoperative deaths. Postoperative hours of intubation, hospital length of stay, and POD 1–5 fluid intake did not differ between groups. Over POD 1–5, PPD use accounted for 48.8 mL/kg increased fluid output (95% CI [2.2, 95.4], p = 0.043) and 3.41 mg/kg less furosemide administered (95% CI [1.69, 5.14], p

Objective: We investigated the incidence and etiologies for unplanned hospital readmissions during the first year following congenital heart surgery (CHS) at our institution and the potential association of readmissions with longer term survival. Methods: We retrospectively reviewed 263 patients undergoing CHS at our institution from August 2011 to June 2015. Scheduled readmissions were excluded. Results: Seventy patients accrued a total of 120 readmissions (1.7 readmission/patient) within one year after surgery. The first readmission for 57% of the patients was within 30 days postdischarge. Twenty-two patients were first readmitted between 31 and 90 days postdischarge. Eight patients were first readmitted between 90 days and 1 year postdischarge. Median time-to-first readmission was 21 days. Median hospital length of stay at readmission was two days. Causes of 30-day readmissions included viral illness (25%), wound infections (15%), and cardiac causes (15%). Readmissions between 30 and 90 days included viral illness (27%), gastrointestinal (27%), and cardiac causes (9%). Age, STAT category, length of surgery, intubation, intensive care unit, and hospital stay were risk factors associated with readmissions based on logistic regression. Distance to hospital had a significant effect on readmissions ( P < .001). Patients with higher family income were less likely to be readmitted ( P < .001). There was no difference in survival between readmitted and non-readmitted patients ( P = .68). Conclusions: The first 90 days is a high-risk period for unplanned hospital readmissions after CHS. Complicated postoperative course, higher surgical complexity, and lower socioeconomic status are risk factors for unplanned readmissions the first 90 days after surgery. Efforts to improve the incidence or readmission after CHS should extend to the first 3 months after surgery and target these high-risk patient populations.

Pulmonary valve replacement (PVR) with right ventricular outflow tract (RVOT) reconstruction is a common congenital cardiac operation. Porcine submucosal intestinal-derived extracellular matrix (ECM) patches have been used for RVOT reconstruction. We present 2 adult patients with Tetralogy of Fallot who underwent PVR with RVOT reconstruction utilizing ECM. Both cases required reoperation due to patch dehiscence causing a large paravalvular leak. One patient also had a pseudoaneurysm associated with ECM dehiscence. There may be a propensity for ECM dehiscence in this application and, based on these cases, we recommend avoidance of ECM in RVOT reconstruction with PVR. PVR patients repaired with ECM should be monitored for this complication.

Objective We describe experience treating a patient with atrioventricular (AV) discordance with ventriculoarterial (VA) concordance, ventricular septal defect (VSD), and situs inversus. This is a rare congenital lesion in which closing the VSD would septate D-transposition physiology and performing an arterial switch and VSD closure would produce congenitally corrected transposition of the great arteries physiology. As such, it is the only lesion for which an isolated atrial switch (and VSD closure) remains the preferred correction. Case A term baby girl born with AV discordance/VA concordance, a large VSD, and situs inversus totalis was palliated with atrial septostomy on day of life 3 to allow for optimal mixing and pulmonary artery banding during the 6th week of life to control symptoms of pulmonary overcirculation and protect the pulmonary vasculature. At 8 months of age, she underwent complete repair with a Senning atrial switch procedure, VSD closure, and pulmonary artery debanding with pulmonary arterioplasty. Results The patient underwent corrective surgery with patch closure of the VSD, and the Senning atrial switch procedure resulting in a total anatomic correction. The patient was discharged on postoperative day 6 and is clinically doing well 12 months later. Follow-up transthoracic echocardiogram shows no pulmonary venous baffle obstruction, mild systemic venous baffle obstruction, and moderate pulmonary stenosis/pulmonary insufficiency. Conclusion The isolated atrial switch is rarely employed as its outcomes are inferior to the arterial switch operation in the setting of the dextro-transposition of the great arteries. However, it remains the procedure of choice for AV discordance with VA concordance as it leads to physiologically corrected biventricular circulation.

As the quality of surgical outcomes depend on many factors, the development of validated tools to assess the different aspects of complex multidisciplinary teams' performance is crucial. The Technical Performance Score (TPS) has only been validated to correlate with outcomes in large-volume surgical programs. Here we assess the utility of TPS in correlation to perioperative outcomes for complex congenital heart surgeries (CHS) performed in a small-to-medium-volume program. 673 patients underwent CHS from 4/2012 to 12/2017 at our institution. Of those, 122 were STAT 4 and STAT 5. TPS was determined for each STAT 4 and STAT 5 operation using discharge echocardiogram: 1 = optimal, 2 = adequate, 3 = inadequate. Patient outcomes were compared including mortality, length of stay, ventilation times, and adverse events. 69 patients (57%) were neonates, 32 (26%) were infants, 17 (14%) were children, 4 (3%) were adults. TPS class 1 was assigned to 85 (70%) operations, TPS class 2 was assigned to 25 (20%) operations, and TPS class 3 was assigned to 12 (10%) operations. TPS was associated with re-intubation, ICU length of stay, postoperative length of stay, and mortality. TPS did not correlate with unplanned 30-day readmissions, need for reoperation, and inotropic score. Technical performance score was associated with perioperative outcomes and is a useful tool to assess the adequacy of repair for high complexity CHS in a small-to-medium-volume surgical program. TPS should be a part of program review in congenital heart programs of all sizes to identify strategies that may reduce postoperative morbidity and potentially improve long-term outcomes.

BACKGROUND: Characterizing the flow characteristics of the Fontan circuit, and correlating these characteristics with the development of complications, is an important clinical challenge. Past work has analyzed the flow characteristics of Fontan circulation on a component-by-component basis. Four-dimensional (4D) flow magnetic resonance imaging (MRI) with radial projections allows for large volumetric coverage, and therefore can be used to analyze the flow through many co-dependent cardiovascular components in a single imaging session. PURPOSE: The purpose of this study is to describe flow characteristics across the entire Fontan circuit and to compare these with the flow characteristics in healthy volunteers. STUDY TYPE: Prospective SUBJECTS: Eleven (11) single ventricle patients with a Fontan connection and fifteen (15) healthy controls. SEQUENCE: Phase Contrast with Vastly undersampled Isotropic Projection Reconstruction (PC-VIPR) at a field strength of 3T. ASSESSMENT: Cavopulmonary and ventricular flow distributions, blood flow kinetic energy, vorticities, efficiency indices, and other flow parameters were analyzed using Ensight and Matlab. STATISTICAL TESTS: Results were compared across Fontan subjects, between respiratory phases, and between Fontan subjects and healthy volunteers using a Student’s t-test for unequal sample sizes and linear regression. RESULTS: Cava-specific pulmonary flow distributions of Fontan patients varied significantly between respiratory phases (p

Introduction Congenital single lung (CSL) is a rare condition, and symptomatic patients often present with respiratory distress or recurrent respiratory infection due to mediastinal shift causing vascular or airway compression. Aberrant right subclavian artery (ARSA) is another rare congenital anomality that can lead to tracheal or esophageal compressions. There is only one other case of concurrent presentation of CSL and ARSA reported, which presented unique challenge in surgical management of our patient. Here we present a step-wise, multidisciplinary approach to manage symptomatic CSL and ARSA. Case presentation An infant girl with a prenatal diagnosis of CSL developed worsening stridor and several episodes of respiratory illnesses at 11 months old. Cross-sectional imaging and bronchoscopic evaluation showed moderate to severe distal tracheomalacia with anterior and posterior tracheal compression resulting from severe mediastinal rotation secondary to right-sided CSL. It was determined that her tracheal compression was mainly caused by her aortic arch wrapping around the trachea, with possible additional posterior compression of the esophagus by the ARSA. She first underwent intrathoracic tissue expander placement, which resulted in immediate improvement of tracheal compression. Two days later, she developed symptoms of dysphagia lusoria due to increased posterior compression of her esophagus by the ARSA. She underwent transposition of ARSA to the right common carotid with immediate resolution of dysphagia lusoria. As the patient grew, additional saline was added to the tissue expander due to recurrence in compressive symptoms. Conclusions Concurrent presentation of CSL and ARSA is extremely rare. Asymptomatic CSL and ARSA do not require surgical interventions. However, if symptomatic, it is crucial to involve a multidisciplinary team for surgical planning and to take a step-wise approach as we were able to recognize and address both tracheomalacia and dysphagia lusoria in our patient promptly.

The Society of Thoracic Surgeons (STS) public reporting in congenital heart surgery has received considerable attention; however, it is unclear how pediatric cardiac providers use these data to guide surgical referrals.We surveyed members of the American Academy of Pediatrics Section on Cardiology and Cardiac Surgery and PediHeartNet members regarding use of STS public reporting.There were 155 respondents (90% cardiologist, 7% surgeons) from approximately 800 solicitations (∼19% response rate). While most (83%) felt that STS public reporting is important, 60% are unsure of its accuracy and only 37% find it useful in practice. Most (71%) believe STS public reporting leads to risk aversion. Overall, 92% answered that STS public reporting rarely or never overrides other factors determining referrals. Compared with smaller centers (300 cases/year), providers in larger centers were more likely to report that STS public reporting data never overrides other factors determining referrals (54% vs 32%, P = .03). Providers using STS public reporting to guide referrals (14% overall) trust the system's accuracy (P = .03) and believe it presents useful outcomes (P.01). There was no correlation between use of STS public reporting to guide referrals and practice size, type, location, time in practice, surgical center affiliation, or center volume.Providers believe that public reporting of outcomes is important; however, most do not use the data to guide surgical referrals. Understanding these limitations of the current STS public reporting may enable change and increased usefulness for providers.

Three-dimensional printing (3Dp) has a broad array of medical applications and has been applied extensively in congenital heart disease given the variety and complexity of lesions encountered. 3Dps are unique when compared to other imaging modalities in that they are theoretically equally interpretable by radiologists, cardiologists and surgeons. The literature regarding 3Dp in congenital heart disease is recent and burgeoning. This review, categorized by application, should provide the reader with a comprehensive, albeit not complete, overview of 3Dp in congenital heart disease during the last decade.

OBJECTIVE To determine the consequences of an early catheter-based intervention on pulmonary artery (PA) growth and right ventricular (RV) myocardial function in an animal model of branch PA stenosis. BACKGROUND Acute results and safety profiles of deliberate stent fracture within the pulmonary vasculature have been demonstrated. The long-term impact of early stent intervention and deliberate stent fracture on PA growth and myocardial function is not understood. METHODS Implantation of small diameter stents was performed in a pig model of left PA stenosis at 6 weeks (10 kg) followed by dilations at 10 (35 kg) and 18 weeks (65 kg) with intent to fracture and implant large diameter stents. Hemodynamics, RV contractility, and 2D/3D angiography were performed with each intervention. The heart and pulmonary vasculature were histologically assessed. RESULTS Stent fracture occurred in 9/12 and implantation of large diameter stents was successful in 10/12 animals with no PA aneurysms or dissections. The final stented PA segment and distal left PA branch origins equaled the corresponding PA diameters of sham controls. Growth of left PA immediately beyond the stent was limited and there was diffuse fibro-intimal proliferation within the distal left and right PA. RV contractility was diminished in the intervention group and the response to dobutamine occurred uniquely via increases in heart rate. CONCLUSIONS Early stent intervention in this surgically created PA stenosis model was associated with improved growth of the distal PA vasculature but additional investigation of PA vessel physiology and impact on the developing heart are needed.

Pulmonary embolism is a rare but potentially fatal complication in neonates with congenital heart disease. The authors report a case of pulmonary embolism in the immediate postoperative period following modified Norwood procedure with Sano shunt. In addition, this report discusses the initial evaluation and available anticoagulation options to treat pulmonary embolism in children with congenital heart disease.

BACKGROUND Dressing open sternal wounds after cardiac surgery can be challenging. In cases where extracorporeal membrane oxygenation (ECMO) is required, the sternum is left open with cannulae traversing the wound, making it difficult to create a water-tight seal and maintain sterility. Patients with a deep sternal infection may also be left with an open sternum between the time of debridement and reconstruction; in such cases, stabilizing the dressing in an ambulatory patient can pose a challenge. METHODS Two cases are reviewed, each highlighting the solutions to the problems mentioned above. RESULTS In case 1, a patient with an open sternum and central ECMO, we describe construction of a dressing that achieves air seal and blood seal, and helps maintain the cannulae in a safe, stable position. In case 2, an ambulatory patient, we describe a modification of a recently published "Two Bridge Technique" that provides a stable dressing in three dimensions. CONCLUSION Performance of specific techniques and attention to detail in complex situations can make a big difference in open sternum patients treated with temporary, vacuum-assisted dressings. Achieving vacuum and fluid seal is important for sterility, integrity of adjacent skin, and caregiver safety.

Summary Here, we describe the NeoThy humanized mouse model created using non-fetal human tissue sources, cryopreserved neonatal thymus and umbilical cord blood hematopoietic stem cells (HSCs). Conventional humanized mouse models are made by engrafting human fetal thymus and HSCs into immunocompromised mice. These mice harbor functional human T cells that have matured in the presence of human self-peptides and human leukocyte antigen molecules. Neonatal thymus tissue is more abundant and developmentally mature and allows for creation of up to ∼50-fold more mice per donor compared with fetal tissue models. The NeoThy has equivalent frequencies of engrafted human immune cells compared with fetal tissue humanized mice and exhibits T cell function in assays of ex vivo cell proliferation, interferon γ secretion, and in vivo graft infiltration. The NeoThy model may provide significant advantages for induced pluripotent stem cell immunogenicity studies, while bypassing the requirement for fetal tissue., Highlights • Neonatal tissue is a viable alternative to fetal for mouse humanization • Over 1,000 NeoThy mice can be made from one neonatal thymus donor • The NeoThy enables robust pre-clinical immunogenicity studies of iPSC therapies, Corresponding author William Burlingham and colleagues created a humanized mouse model called the NeoThy. The NeoThy uses human neonatal, rather than fetal, tissue sources for generating a human immune system within immunocompromised mouse hosts. NeoThy mice are an attractive alternative to conventional humanized mouse models, as they enable robust and reproducible iPSC immunogenicity experiments in vivo.

The purpose of this study was to assess the diagnostic capabilities of transesophageal echocardiography (TEE) compared to completion angiography for detection of residual post-operative pulmonary artery lesions. This is a retrospective review of 19 consecutive surgical cases involving the pulmonary arteries that had post-operative TEE and completion angiography from 2014 to 2017. The echocardiograms were reviewed by 2 blinded examiners and categorized as adequate or inadequate visualization of the surgical repair. Based on TEE images, the surgical repair was graded as no revision necessary, residual lesion present requiring revision, or unable to assess. TEE was compared to completion angiography to determine the ability of each method to detect residual pulmonary artery lesions. Fifty-three percent of TEE imaging was graded as inadequate. Based on TEE, surgical revision was indicated in 2 of 19 cases. Completion angiography documented 4 additional residual lesions resulting in surgical revision in 6 of 19 patients. TEE sensitivity for detecting residual pulmonary artery lesions was 40%. One Glenn patient with adequate image quality and repair by TEE had moderate left pulmonary artery stenosis by completion angiography. All other discrepancies occurred in patients with inadequate TEE imaging. No patient with pulmonary artery abnormalities had hemodynamic instability or excessive desaturations. Completion angiography-related complications included three transient arrhythmias with no increased incidence of acute kidney injury. Completion angiography may be more effective than TEE at detecting post-operative pulmonary artery lesions even in patients not manifesting clinical symptoms. Documentation of residual lesions with completion angiography allows immediate surgical revision potentially limiting necessity for future interventions.

Persistent ductus arteriosus is the postnatal patency of the ductus arteriosus seen primarily in premature infants. The molecular mechanisms are incompletely understood. PDA results in volume overload and can progress to heart failure and occasionally irreversible pulmonary hypertension. A PDA in an older infant, a child, or an adult is usually addressed with transcatheter closure. In the small premature infant for whom pharmacologic closure is unsuccessful or contraindicated, surgical intervention is sometimes necessary, but only after thorough evaluation and establishment of a clear hemodynamic indication for closure.

Improvements in surgical technique and perioperative care have resulted in increased long-term survival for patients with congenital heart disease. As these patients begin to reach their later years, clinicians are challenged with determining optimal management of noncardiac diseases in this complex patient population, including surgically treatable malignancies. We present a case of esophageal cancer in a patient with previously repaired tetralogy of Fallot and right-sided aortic arch, treated with neoadjuvant therapy followed by laparoscopic and left thoracoscopic esophagectomy.

The objective of this study was to evaluate the effect of structured post-discharge telephone follow-up (TFU) on the time to the first postoperative clinic visit and early unplanned hospital readmissions in patients after congenital heart surgery. Structured phone calls delivered by senior surgical practitioners were made 1-4 days post-discharge. Demographics and clinical outcomes of pediatric patients receiving a TFU from 2012 to 2014 were assessed. In total, 196 phone calls were made in 165 patients. Thirty-four health problems were identified in 32 (19 %) patients (15 infants, 9 children, 8 neonates). Sixty-nine percent (n = 22) of the patients with problems identified at TFU were males. Fifty-three percent (n = 17) of the patients with problems identified at TFU were RACHS 2. Most of the problems (53 %) were identified in middle-class economic families. Gastrointestinal and incision site complications were the most common problems identified. Eighteen (56 %) patients had adjustments of medications. Six (19 %) TFUs resulted in earlier than scheduled post-op clinic visits. Overall incidence of unplanned readmissions was 16 % and similar between the patients that had problems during TFU (13 %) and patients that did not have problems identified at TFU (18 %; p = 0.50). Our study demonstrates the potential impact of the timely and structured post-discharge TFU in identifying post-discharge complications and modifying discharge instructions. When TFU identifies a problem, the interventions may be successful in preventing readmissions before first postoperative clinic visit. TFUs may be helpful, as a supplement standard of care with specific focus on patients

Background The purpose of this study is to determine the outcome and performance of bovine pericardial valves in the pulmonary position. Methods This is a retrospective review of all patients with congenital heart disease who had pulmonary valve replacement using a bovine pericardial valve from 2002 to 2009 at a single institution. Results There were 73 consecutive patients, with a median age of 17.3 years (range, 2.1 to 64.4). Their diagnosis was tetralogy of Fallot (n = 47), pulmonary stenosis (n = 11), or other (n = 15). Sixty-nine patients had 91 previous surgical procedures. The mean time from last surgery was 19.9 ± 11.6 years. Forty-three patients had concomitant surgical procedures. There were no perioperative deaths. Clinical follow-up was available in 68 patients (93%). There were no late deaths, and all patients were in New York Heart Association functional class I during a median follow-up period of 2.6 years (range, 0.2 to 8.0). One patient had endocarditis necessitating valve removal 2 years after surgery. Freedom from pulmonary valve reoperation was 100%, 97.7%, and 97.7% at 1, 3, and 5 years, respectively (95% confidence interval: 93.2% to 100%). Mean pulmonary valve gradient at follow-up was 19 ± 14 mm Hg. Degree of pulmonary insufficiency was less than moderate in 62 patients, moderate in 4, and more than moderate in 2. Freedom from moderate-severe or severe pulmonary insufficiency was 97.7%, 89.1%, and 89.1% at 1, 3, and 5 years, respectively (5-year 95% confidence interval: 77.0% to 100%). Conclusions Pulmonary valve replacement using a bovine pericardial valve can be accomplished with low perioperative morbidity and favorable midterm outcomes. Further follow-up is necessary to evaluate the long-term performance of bovine pericardial valves in the pulmonary position.

Background Papillary muscle rupture in the fetus and neonate is a rare event that leads to severe mitral or tricuspid insufficiency and is associated with high perinatal mortality. We undertook surgical repair of this lesion in the neonatal period and report on our midterm results. Methods Three neonates with tricuspid insufficiency and 1 infant with mitral insufficiency, all due to papillary muscle or chordal rupture, underwent surgical repair with artificial chordal replacement and a modification of the de Vega annuloplasty technique that allowed external adjustment of the annulus size under transesophageal echocardiographic guidance after separation from cardiopulmonary bypass. Results All patients recovered well from the operation. There have been no late deaths and no valve-related complications. On discharge, all 3 patients had evidence of trace to mild atrioventricular valve regurgitation. At a median follow-up of 33 months (range, 7 to 50; 123 patient-months), all 4 patients are growing normally. Three patients have had no change in the degree of tricuspid or mitral regurgitation. One patient required reoperation at 54 months postoperatively for acute mitral insufficiency secondary to separation of an artificial chorda from the ventricular wall. Conclusions Surgical repair of critical neonatal tricuspid and mitral insufficiency associated with papillary muscle or chordal rupture is feasible and can result in good early and midterm results. Our modification of the De Vega annuloplasty technique with the ability to externally adjust the size of the annulus under echocardiographic guidance may improve the accuracy of the repair in the neonate.

Background A coronary artery arising from the wrong aortic sinus, with or without a proximal course between the aorta and the pulmonary artery, may predispose to myocardial ischemia, myocardial infarction, or sudden death. Methods We repaired this lesion in 5 symptomatic patients, all of whom had experienced exercise-related chest pain, dyspnea, or syncopal episodes. All patients underwent patch angioplasty of the proximal anomalous coronary artery with glutaraldehyde-treated autologous pericardium. When the coronary artery coursed between the aorta and the main pulmonary artery, the main pulmonary artery was translocated toward the left pulmonary artery to create additional space and prevent compression. Results All patients recovered well and to date have demonstrated no evidence of recurrent myocardial ischemia. Conclusions The combination of coronary artery angioplasty and translocation of the pulmonary artery seems to effectively address all the mechanisms that can generate ischemia.

ObjectivesThe transannular patch used to relieve right ventricular outflow tract obstruction in children with tetralogy of Fallot may result in pulmonary insufficiency. We hypothesized that pulmonary valve cusp augmentation with pericardium would decrease pulmonary insufficiency and improve the early outcome for transatrial–transpulmonary tetralogy of Fallot repair requiring transannular patch.MethodsSince November 2001, 41 patients with tetralogy of Fallot and 2 patients with isolated pulmonary valve stenosis had relief of right ventricular outflow tract obstruction with either a transannular patch plus pulmonary valve cusp augmentation (n = 18) or a transannular patch alone (n = 25). Data were retrospectively collected.ResultsThe median age (5.3 vs 3.2 months; P = .09) and weight (6.4 vs 5.2 kg; P = .3) were similar for the cusp augmentation and transannular patch groups, respectively. The diameter of the pulmonary valve annulus (6.4 vs 6.0 mm; P = .57) and the McGoon index (1.47 vs 1.69, P = .75) were also similar. The mean aortic clamp time (48 ± 18 minutes vs 52 ± 19 minutes; P = .46) and median cardiopulmonary bypass time (89 vs 91 minutes; P = .9) did not differ. One patient with a transannular patch died of multiorgan system failure. Patients with a pulmonary valve cusp augmentation had a shorter duration of intubation (1 vs 3 days; P < .001) and intensive care unit stay (2 vs 8 days; P < .001). Thirteen patients with a transannular patch and 1 patient with a pulmonary valve cusp augmentation required inotropic support for more than 72 hours (P = .001). Discharge echocardiograms demonstrated moderate or severe pulmonary insufficiency in 5 patients with a pulmonary valve cusp augmentation and in 21 patients with a transannular patch (P < .001). At 7.5 months, 3 patients (17%) with a pulmonary valve cusp augmentation had progression of pulmonary insufficiency.ConclusionsAugmentation of a pulmonary valve cusp reduces the incidence of clinically significant postoperative pulmonary insufficiency. This technique may improve the early outcome for children with tetralogy of Fallot requiring a transannular patch.

We sought to determine whether descending thoracic aortic occlusion (DTAOC) induced ischemia results in adrenal dysfunction.Eight pigs underwent DTAOC for 45 min. Six control pigs underwent a sham procedure. Serum cortisol and adrenocorticotropic hormone (ACTH) were measured at baseline, at the end of DTAOC, 30 and 60 min after restoration of flow, and 24 hours later. Statistical analysis was performed using repeated measures ANOVA and t-test.In the study group, cortisol levels decreased during DTAOC (p=0.048) and 30 min after flow restoration (p=0.004). In the control group there was no change in serum cortisol levels. In the study group the drop in serum cortisol was associated with an increase in ACTH levels during DTAOC (p=0.040) and 30 minutes after flow restoration (p=0.070). The increase in ACTH was also significant when compared to the controls during DTAOC (p=0.030) and 30 min after blood flow restoration (p=0.040).There is a transient period of adrenal dysfunction associated with DTAOC that results in stimulation of the pituitary-adrenal axis.

We present a newborn with heterotaxy features, multiple congenital anomalies, truncus arteriosus with long segment tracheal stenosis, and a left pulmonary artery sling. The patient had complete neonatal repair with slide tracheoplasty and repair of the left pulmonary artery sling with anterior translocation of the pulmonary artery. The truncus was repaired with a transventricular ventricular septal defect closure with a patch and right ventricle to pulmonary artery conduit. Complete repair of complex cardiac neonatal lesions with critical tracheal stenosis is feasible and should be the strategy of choice in these complex patients.

ObjectiveDiscrete morphologic, enzymatic and functional changes in skeletal muscle mitochondria have been demonstrated in patients with peripheral arterial disease (PAD). We examined mitochondrial respiration in the gastrocnemius muscle of nine patients (10 legs) with advanced PAD and in nine control patients (nine legs) without evidence of PAD.MethodsMitochondrial respiratory rates were determined with a Clark electrode in an oxygraph cell containing saponin-skinned muscle bundles. Muscle samples were obtained from the anteromedial aspect of the gastrocnemius muscle, at a level 10 cm distal to the tibial tuberosity. Mitochondria respiratory rate, calculated as nanoatoms of oxygen consumed per minute per milligram of noncollagen protein, were measured at baseline (V0), after addition of substrates (malate and glutamate; (VSUB), after addition of adenosine diphosphate (ADP) (VADP), and finally, after adenine nucleotide translocase inhibition with atractyloside (VAT). The acceptor control ratio, a sensitive indicator of overall mitochondrial function, was calculated as the ratio of the respiratory rate after the addition of ADP to the respiratory rate after adenine nucleotide translocase inhibition with atractyloside (VADP/ VAT).ResultsRespiratory rate in muscle mitochondria from patients with PAD were not significantly different from control values at baseline (0.31 ± 0.06 vs 0.55 ± 0.12; P = .09), but Vsub was significantly lower in patients with PAD compared with control subjects (0.43 ± 0.07 vs 0.89 ± 0.20; P < .05), as was VADP (0.69 ± 0.13 vs 1.24 ± 0.20; P < .05). Respiratory rates after atractyloside inhibition in patients with PAD were no different from those in control patients (0.47 ± 0.07 vs 0.45 ± P = .08). Compared with control values, mitochondria from patients with PAD had a significantly lower acceptor control ratio (1.41 ± 0.10 vs 2.90 ± 0.20; P < .001).ConclusionMitochondrial respiratory activity is abnormal in lower extremity skeletal muscle in patients with PAD. When considered in concert with the ultrastructural and enzymatic abnormalities previously documented in mitochondria of chronically ischemic muscle, these data support the concept of defective mitochondrial function as a pathophysiologic component of PAD.

1. We examined the effects of eprosartan, an AT(1) receptor antagonist, on the progression of left ventricular (LV) dysfunction and remodelling in dogs with heart failure (HF) produced by intracoronary microembolizations (LV ejection fraction, EF 30 to 40%). 2. Dogs were randomized to 3 months of oral therapy with low-dose eprosartan (600 mg once daily, n=8), high-dose eprosartan (1200 mg once daily, n=8), or placebo (n=8). 3. In the placebo group, LV end-diastolic (EDV) and end-systolic (ESV) volumes increased after 3 months (68+/-7 vs 82+/-9 ml, P

Purpose: The causative role of consumptive coagulopathy in the development of bleeding complications after supraceliac (SC) aortic cross-clamping (AXC) has been challenged by recent reports that ascribe this coagulopathy to primary fibrinolysis. This theory is made on the basis of evidence that tissue plasminogen activator (t-PA) antigen (Ag) levels increase after SC AXC. However, t-PA Ag levels reflect both active and inactive (bound to serum t-PA inhibitors) forms of serum t-PA, and elevations confirm the presence of fibrinolysis only in conjunction with an increase in t-PA activity. Methods: To investigate the etiology of this coagulopathy, we submitted eight pigs to SC AXC and six pigs to infrarenal (IR) AXC for 30 minutes. Blood was drawn from the portal vein, the hepatic vein, and the carotid artery before AXC, just before unclamping, and 5, 30, and 60 minutes after unclamping. Prothrombin time (PT), partial thromboplastin time (PTT), fibrinogen (FBG), platelets (PLT), thrombin-antithrombin complexes (TAT), t-PA Ag, t-PA activity, plasminogen activator inhibitor-1 (PAI-1), and α2-antiplasmin (AP) activities were measured. Statistical analysis was performed by using repeated measures analysis of variance and t tests Results: The PT did not differ between the two groups at any point. After unclamping, in the SC group there was a drop in PLT levels (P =.005), a decrease in FBG levels (P

We examined the effects of long-term monotherapy with the beta-blocker, metoprolol controlled release/extended release (CR/XL), on the progression of LV dysfunction as well as on global and cellular remodeling in dogs with heart failure (HF). Chronic HF was produced by intracoronary microembolizations that were discontinued when LV ejection fraction (EF) was between 30% and 40%. Dogs were randomized to 3 months oral monotherapy with metoprolol CR/XL (100 mg once daily, n = 7) or no therapy at all (control, n = 7). In control dogs, EF decreased from 38 ± 1% to 31 ± 2% (p = 0.002), and LV end-systolic volume (ESV) and LV end-diastolic volume (EDV) increased (37 ± 2 vs 45 ± 2 ml, p = 0.001; 59 ± 3 vs 65 ± 3 ml, p = 0.001; respectively) during the 3 month follow-up period. In dogs treated with metoprolol CR/XL, EF increased after 3 months from 36 ± 1% to 43 ± 1% (p = 0.001), and ESV decreased (42 ± 2 vs 38 ± 2 ml, p = 0.003), whereas EDV remained unchanged. Compared to controls, treatment with metoprolol CR/XL showed 46% reduction in replacement fibrosis, 54% reduction in interstitial fibrosis and 20% reduction in myocyte cross-sectional area, a measure of myocyte hypertrophy. These findings indicate that metoprolol CR/XL improves LV function and attenuates progressive global and cellular LV remodeling in dogs with HF. The benefits are fully attributable to β-blockade alone as no other adjunctive therapy was used.

Sixty-five consecutive patients undergoing nonemergent repair of an abdominal aortic aneurysm (AAA) originating above the visceral and/or renal arteries were studied to determine operative results and identify factors influencing outcome of proximal AAA repair. Factors associated with postoperative morbidity were analyzed using multivariate analysis. There were no postoperative deaths, paraplegia/paraparesis, or symptomatic visceral ischemia. Proximal AAA repair can be accomplished with acceptable mortality. If renal artery bypass or reimplantation is anticipated, cold renal perfusion may protect against renal dysfunction. Postoperative pulmonary dysfunction can be reduced by avoiding radial division of the diaphragm.