Multimodality Imaging in Ebstein Anomaly.

Ebstein anomaly is the most common form of tricuspid valve congenital anomalies. The tricuspid valve is abnormal with different degrees of displacement of the septal leaflet and abnormal rotation of the valve towards the right ventricular outflow tract. In severe forms, it results in significant tricuspid regurgitation and requires surgical repair. There is an increased interest in understanding the anatomy of the tricuspid valve in this lesion as the surgical repair has evolved with the invention and wide adoption of the cone operation. Multimodality imaging plays an important role in diagnosis, follow-up, surgical planning and post-operative care. This review provides anatomical tips for the cardiac imagers caring for patients with Ebstein anomaly and will help provide image-based personalized medicine.

A High-Fidelity Percutaneous Model Used to Demonstrate ECMO Cannulation.

Medical simulation provides a realistic environment for practitioners to experience a planned clinical event in a controlled educational setting. We established a simulation model composed of synthetic ballistic gelatin that provided an inexpensive high-fidelity model for our extracorporeal membrane oxygenation (ECMO) team members to develop, master, and maintain clinical skills necessary for percutaneous cervical or femoral cannulation. The simulation component includes a cervical torso or femoral percutaneous synthetic gelatin model that is attached to either a static fluid model or to the high-fidelity perfusion simulator. Either model can be accessed with ultrasound guidance, cannulated with appropriately sized cannula, and connected to an in situ ECMO circuit. This article explains how the model is made and connected to the simulator with the purpose of re-creating this high-fidelity experience at any institution.

The impact of COVID-19 on thoracic surgery residency programs in the US: A program director survey.

OBJECTIVE: The coronavirus disease 2019 (COVID-19) has altered how the current generation of thoracic surgery residents are being trained. The aim of this survey was to determine how thoracic surgery program directors (PDs) are adapting to educating residents during the COVID-19 pandemic. METHODS: Thoracic surgery PDs of integrated, traditional (2 or 3 year), and combined 4 + 3 general/thoracic surgery training programs in the United States were surveyed between 17th April and 1st May 2020 during the peak of the COVID-19 pandemic in much of the United States. The 15-question electronic survey queried program status, changes to the baseline surgical practice, changes to didactic education, deployment/scheduling of residents, and effect of the pandemic on case logs and preparedness for resident graduation. RESULTS: All 23 institutions responding had ceased elective procedures, and most had switched to telemedicine clinic visits. Online virtual didactic sessions were implemented by 91% of programs, with most (69.6%) observing same or increased attendance. PDs reported that 82.7% of residents were on a non-standard schedule, with most being deployed in a 1 to 2 week on, 1 to 2 week off block schedule. Case volumes were affected for both junior and graduating trainees, but a majority of PDs report that graduating residents will graduate on time without perceived negative effect on first career/fellowship position. CONCLUSIONS: The COVID-19 pandemic has radically changed the educational approach of thoracic surgery programs. PDs are adapting educational delivery to optimize training and safety during the pandemic. Long-term effects remain uncertain and require additional study.

A High-Fidelity Surgical Model and Perfusion Simulator Used to Demonstrate ECMO Cannulation, Initiation, and Stabilization.

Our high-fidelity simulation model provides a realistic example for health-care professionals to experience cannulation, initiation, and hemodynamic stabilization during extracorporeal membrane oxygenation (ECMO) therapy. This educational experience brings a variety of critical care specialties together, in a controlled simulation setting, to develop, master, and maintain clinical skills. This may include perfusionists, ECMO specialists, surgical technicians, registered nurses, physicians, and students. The simulation component includes a unique vascular access pad that is attached to either a static fluid model or to the Califia perfusion simulator system (Biomed Simulation, Inc., San Diego, CA). This collective high-fidelity simulation model can be surgically cannulated via a cutdown technique using an appropriately sized cannula and connected to an in situ ECMO circuit. This article explains the educational strategy, how the surgical pad is made, and the simulator connections so that any hospital can re-create this experience.

Charges and resource utilization for pediatric heart transplantation across a positive virtual and/or cytotoxicity crossmatch.

There is growing acceptance of transplantation across a positive crossmatch for highly allosensitized pediatric HT candidates. While survival may be similar to patients transplanted across a negative crossmatch, costs are unknown. Among 60 HT recipients at our center from 5/07 to 6/12, we analyzed hospital charges and length of stay from the day of HT to discharge and through the first year after transplant. Median age at HT was 6.2 years (15 days-20.5 years). Charges in the first year post-HT were greater for crossmatch-positive patients ($907 678 vs $549 754; P = .017), with a trend toward higher charges for the HT hospitalization ($537 640 vs $407 374; P = .07). Plasmapheresis was more common in crossmatch-positive patients during the HT hospitalization (80% vs 4%, P < .001). In the first year after HT, crossmatch-positive patients had a greater number of endomyocardial biopsies (10 vs 7.5, P = .03) and episodes of treated rejection (2 vs 0, P = .004). Pediatric HT across a positive crossmatch is associated with higher first-year costs, including increased use of plasmapheresis and care around an increased number of rejections. These novel data will help inform decision and policymaking regarding care practices for the growing population of highly sensitized pediatric HT candidates.

Brain Dysplasia Associated with Ciliary Dysfunction in Infants with Congenital Heart Disease.

OBJECTIVE: To test for associations between abnormal respiratory ciliary motion (CM) and brain abnormalities in infants with congenital heart disease (CHD) STUDY DESIGN: We recruited 35 infants with CHD preoperatively and performed nasal tissue biopsy to assess respiratory CM by videomicroscopy. Cranial ultrasound scan and brain magnetic resonance imaging were obtained pre- and/or postoperatively and systematically reviewed for brain abnormalities. Segmentation was used to quantitate cerebrospinal fluid and regional brain volumes. Perinatal and perioperative clinical variables were collected. RESULTS: A total of 10 (28.5%) patients with CHD had abnormal CM. Abnormal CM was not associated with brain injury but was correlated with increased extraaxial cerebrospinal fluid volume (P < .001), delayed brain maturation (P < .05), and a spectrum of subtle dysplasia including the hippocampus (P < .0078) and olfactory bulb (P < .034). Abnormal CM was associated with higher composite dysplasia score (P < .001), and both were correlated with elevated preoperative serum lactate (P < .001). CONCLUSIONS: Abnormal respiratory CM in infants with CHD is associated with a spectrum of brain dysplasia. These findings suggest that ciliary defects may play a role in brain dysplasia in patients with CHD and have the potential to prognosticate neurodevelopmental risks.

Perioperative mortality is the Achilles heel for cardiac transplantation in adults with congenital heart disease: Evidence from analysis of the UNOS registry.

BACKGROUND: Adults with congenital heart disease may present with end-stage heart failure necessitating orthotopic heart transplant (OHT). We sought to review the United Network for Organ Sharing (UNOS) experience with this unique cohort focusing on surgical outcomes and survival. METHODS: From the UNOS registry, 737 adult congenital heart disease recipients (ACHDR) out of 26,993 OHT patients (2.7%) who underwent OHT were studied to analyze early and late outcomes and compared to non-congenital recipients (NCR) over a 15-year period (2000-2014). RESULTS: More ACHDR underwent OHT in the recent-era (3.1%; 2010-2014) as compared to the initial-era (2.5%; 2000-2004; p = 0.03). ACHDR were more likely female (40% vs. 24%; p < 0.01), younger (35 vs. 53 years; p < 0.01), less likely to have ventricular assist devices (4.2% vs. 19.3%; p < 0.01), more likely to have class-II panel-reactive antibody >10%; p < 0.01, and were listed for a longer time (249 vs. 181 days; p < 0.01). When compared to the NCR in the same period, the ACHDR cohort had longer postoperative length of stay (27 vs. 20 days; p < 0.01), higher operative mortality (11.5% vs. 4.6% p < 0.001), higher incidence of primary graft dysfunction (4.3% vs. 2.6%; p < 0.01), and higher need for dialysis (20% vs. 9%; p < 0.01). Primary graft dysfunction is the most common cause of death in (5.8%) ACHDR. Although short-term survival is poorer, long-term survival of ACHDR was found to be equivalent or better than NCR in long term. CONCLUSIONS: Perioperative morbidity and mortality adversely affects short-term survival in ACHDR. ACHDR who survive the first post-transplant year have equivalent or better long-term survival than NCR.

D-Transposition of the Great Arteries with Ventricular Septal Defect and Left Ventricular Outflow Tract Obstruction (D-TGA/VSD/LVOTO): A Survey of Perceptions, Preferences, and Experience.

Several procedures have been introduced to manage this complex congenital heart malformation. It is not clear that any one approach is best for all patients. Decision-making for a given patient may be influenced by multiple patient factors. There is little doubt that surgeon and institutional experience and preference also play a role. We have focused our survey on three procedures, i.e., the Rastelli, Nikaidoh (aortic root translocation), and réparation à l'étageventriculaire (REV) procedures. The questionnaire was directed to all Congenital Heart Surgeon Society member surgeons. A total of 61 clinical practitioners responded to our survey. Of them, 45 (73.8 %) were staff congenital heart surgeons, 11 (18 %) were staff pediatric cardiologists, 1 (1.6 %) was a staff adult congenital cardiologist, 1 (1.6 %) was a fellow pediatric cardiologist, 2 (3.3 %) were fellow or resident congenital heart surgeons, and 1 (1.6 %) was a nurse practitioner. Most respondents believe that the Rastelli procedure should be considered the first option (n = 28, 45.9 %), while 24 (39.3 %) respondents believe that the Nikaidoh procedure should be considered the first option. Asked "To which surgeon do you prefer to refer your patient" (one who will perform Rastelli, Nikaidoh, or REV), all staff cardiologists (n = 12, 100 %) responded "doesn't matter." This survey revealed a wide diversity among clinical practitioners in terms of management of patients with TGA/VSD/LVOTO and preferred surgical approaches. A multi-institutional study of surgical management of TGA/VSD/LVOTO may help to define principles for optimal matching of procedures to patients.

Cerebral regional oxygen saturation and serum neuromarkers for the prediction of adverse neurologic outcome in pediatric cardiac surgery.

BACKGROUND: The aim of this study is to determine the utility of non-invasive bedside neuromonitoring, including cerebral regional oxygen saturation (rSO2) measured by near-infrared spectroscopy and serum biomarkers, in identifying children at risk from adverse neurological outcome after heart surgery. METHODS: Prospective observational study including 39 consecutive children undergoing heart surgery with cardiopulmonary bypass (CPB) and normal neurologic exam prior to surgery. Cerebral rSO2 was measured at baseline (prior to surgery) and then continuously during surgery and for the first 16 h post-operatively. Neuromarkers [neuron-specific enolase (NSE), S100ß, glial fibrillary acidic protein (GFAP), and brain-derived neurotrophic factor (BDNF)] were measured in serum at baseline, immediately after CPB and at 16 h post-operatively. Adverse neurological outcome was defined as an abnormal pediatric cerebral performance category (PCPC) scale score at 12 months after surgery. RESULTS: Sixteen children (41 %) had an abnormal PCPC scale score at the 12-month evaluation after surgery. In children with unfavorable neurological outcomes, mean cerebral rSO2 values were lower and the area-under-the-curve below a threshold of 40 and 20% below baseline were also increased. No significant differences were found in serum neuromarkers between groups at the time points that were assessed. CONCLUSIONS: Bedside determination of cerebral rSO2 may have some utility in identifying children at risk for adverse neurological outcome after heart surgery in children. Additional studies that are sufficiently powered to control for the many covariates in this patient population will be required to fully interrogate this important question. The role of serum neuromarkers in the immediate post-operative period do not appear to be helpful in this question, though more thorough interrogation of delayed periods may ultimately demonstrate some utility in answering this question.

Optimizing Surgical Selection for Transposition With Left Ventricular Outflow Tract Obstruction.

BACKGROUND: Studies that have assessed the Rastelli and Nikaidoh operations for transposition of the great arteries (TGA) with obstructed left ventricular outflow tract obstruction (LVOTO) have not fully evaluated the anatomic drivers that may contribute to surgical selection. We present our procedural selection process for optimizing outcomes of complex TGA in the modern era. METHODS: This is a single-center, retrospective study that included pediatric patients who underwent either a Nikaidoh or Rastelli operation for the treatment of TGA-LVOTO, congenitally corrected TGA-LVOTO, or double-outlet right ventricle TGA type-LVOTO from June 2004 to June 2021. RESULTS: There were 34 patients stratified by Nikaidoh (n = 16) or Rastelli (n = 18) operation. The incidence of all postoperative complications and mortality was low, and the incidence of complications between the groups was similar. Patients were more likely to have undergone a Nikaidoh than a Rastelli if they had a pulmonary annulus >5 mm (87.5% vs 11.1%), anteriorly/posteriorly oriented great vessels (88% vs 8%), remote (80% vs 11%) or restrictive (75% vs 6%) ventricular septal defect, and right ventricular hypoplasia (50% vs 0%; all, P < .05). The resulting rates of reoperation were similar between the groups (44.0% vs 37.5%; P = .24) and largely composed of conduit replacements in the Rastelli patients and valvular repairs or replacements in the Nikaidoh group. Rates of catheter-based interventions were also similar. CONCLUSIONS: These findings suggest that for the optimal treatment of conotruncal anomalies with discordant ventriculoarterial connections, procedural selection should be based on pathoanatomic criteria that can ensure patients undergo the operation most suited to their anatomy.

Long-term Outcome After Repair of Transposition of the Great Arteries With Aortic Arch Obstruction.

BACKGROUND: This study compares the long-term outcomes of patients after repair of transposition of the great arteries (TGA) with and without aortic arch obstruction (AAO). METHODS: This is a single-institution, retrospective study between October 2004 and February 2023. Patients who underwent arterial switch operation and aortic arch repair (ASO-AAR group) with patch augmentation were compared with those without AAO (ASO group). The primary end point was survival; freedom from reintervention was a secondary end point. RESULTS: We identified 176 patients, 31 in the ASO-AAR group and 145 in the ASO group. The median follow-up period was 10.3 years. There were no differences between the ASO-AAR group and the ASO group in early deaths (3.2% vs 0.7%) and late deaths (3.2% vs 2.8%), or 15-year survival rates (92.6% vs 96.2%). Surgical and catheter-based reinterventions were higher in the ASO-AAR group, involving the pulmonary arteries (41.9% vs 4.8%, P < .001), aortic arch (16.1% vs 0.7%, P < .001), and residual ventricular septal defects (11.4% vs 0%, P = .05). The ASO-AAR group showed a higher prevalence of double-outlet right ventricle TGA-type (61.3% vs 4.1%, P < .001) and a lower aortopulmonary index (0.67 vs 1.01, P < .001). CONCLUSIONS: Patients undergoing surgical repair of TGA and AAO achieved excellent survival rates, comparable to patients with simple transposition. A higher rate of surgical and catheter-based reinterventions was observed in patients with arch obstruction and/or a low aortopulmonary index. AAR with patch augmentation proved to be an effective surgical technique with a low incidence of aortic reinterventions.

The Cone repair Allows Right Ventricle Rehabilitation with Excellent Tricuspid Valve Function Following the Starnes Procedure.

OBJECTIVES: We present a case series of Right Ventricle (RV) rehabilitation after Starnes in patients with Ebstein anomaly, applying the Cone repair of the tricuspid valve (TV) to achieve biventricular or 1.5-ventricle physiology. METHODS: This is a retrospective database analysis from two institutions in North America. We included all consecutive cases of Cone repair after the Starnes procedure. The data are expressed as median and interquartile range (IQR). RESULTS: Eleven patients underwent RV rehabilitation between 2019 and 2023 after initial Starnes palliation with a median age of 27 months (IQR 20.5). All were critically ill before their Starnes procedure; four were on extracorporeal membrane oxygenation. Before the Cone repair, the median pre-operative regurgitant velocity at the Starnes patch was 1.65 m/s (IQR 1.3). During the Cone procedure, nine patients required a concomitant PV repair, of which three required a transannular monocusp patch. Four patients were successfully rerouted to a two-ventricle repair, and seven patients with a previous Glenn achieved 1.5 ventricle circulation. There was no heart block and no deaths. Seven patients had trivial regurgitation in a median follow-up of eleven months (IQR 21.5), three mild and one moderate tricuspid regurgitation. There was no significant TV stenosis; all patients had good functional status at the last follow-up despite severe RV dysfunction in one patient. CONCLUSIONS: After the Starnes procedure, the Cone repair allowed RV rehabilitation, resulting in trivial or mild TV regurgitation sustained in midterm follow-up. The Starnes procedure is a reproducible technique that no longer commits patients to lifetime single ventricle physiology.

Midterm Outcomes After Aortic Valve Neocuspidization (Ozaki Procedure) in Adults.

BACKGROUND: Trileaflet aortic valve neocuspidization (AVN) using autologous pericardium (Ozaki procedure) is an emerging surgical treatment option for aortic valve diseases. Although excellent results have been reported from Japan, data pertaining to its use in the United States are sparse. METHODS: All adult patients who underwent AVN (AVN group) or surgical aortic valve replacement (SAVR) with a bioprosthetic valve (SAVR group) between 2015 and 2022 were identified. Propensity score matching was used to adjust the baseline characteristics between the 2 groups. RESULTS: A total of 101 patients underwent AVN, and 1816 patients underwent SAVR with a bioprosthetic valve. None in the AVN group required conversion to SAVR. Before matching, mean age in the AVN group was 68.5 ± 8.8 years, and 56 patients (55.4%) underwent concomitant procedures. Preoperatively, 3 (3%) had endocarditis. Bicuspid valve was observed in 38 (38.4%). None died at 30 days in the AVN group. The median follow-up duration was 3.2 years. After propensity score matching, the expected survival and freedom from at least moderate aortic regurgitation at 5 years was 91.7% ± 3.1% and 97.6% ± 1.7%, respectively. Propensity score matching yielded 77 patients in each group. The Kaplan-Meier curve demonstrated equivalent survival at 5 years between the 2 groups (P = .95). Additionally, freedom from at least moderate aortic regurgitation was comparable at 5 years (P = .23). CONCLUSIONS: AVN can be safely performed for a variety of aortic valve diseases, with or without concomitant operations. AVN demonstrated similar midterm outcomes compared with SAVR with a bioprosthetic valve in the United States adult population.

Coronary transfer for complex looping and intramural variants in the arterial switch procedure.

Achieving a safe and accurate coronary transfer in the context of the precise geometry of the realigned great arterial connections is the essence of the arterial switch operation for transposition. Because the incidence of variant coronary patterns is not low, one needs to be familiar with techniques to transfer all types of coronary patterns when performing the arterial switch operation. Both closed and open techniques for coronary transfer have been widely adopted for the arterial switch operation. We routinely use the open technique for coronary transfer combined with a liberal use of trapdoor incisions. We demonstrate that this technique, with appropriate technical modifications, serves as a template for achieving accurate coronary transfer even in the context of complex looping and intramural variants.

Predictors of Mediastinal Exploration While on Extracorporeal Membrane Oxygenation After Pediatric Cardiac Surgery.

Cardiac surgical patients requiring extracorporeal membrane oxygenation (ECMO) are at increased risk for hemorrhage due to necessary anticoagulation, in-situ cannulas, and disturbed hemostasis. We performed a retrospective, cross-sectional study of patients 0-18 years old in our cardiac intensive care unit (CICU) cannulated to ECMO within 48 h of cardiopulmonary bypass. The 69 patients included in the study were divided into three analysis groups based on serial chest tube output per hour: no bleeding (NB) on admission to the CICU (21/69, 30%), bleeding stopped (BS) with medical management (26/69, 38%), bleeding requiring emergent mediastinal exploration (BME) (22/69, 32%). The NB group had a more favorable coagulation profile upon admission to the CICU (PTT 53 s NB, 105 s BS, 83 s BME p < 0.001, ACT 169 s NB, 225 s BS, 211 s BME, p =0.013). Only chest tube output during the first three postcannulation hours remained associated with the need for mediastinal exploration by multivariable analysis. An average chest-tube output of 11.6 mL/kg/h during the first three hours had the highest percentage of patients classified correctly (84%) for requiring mediastinal exploration during their ECMO run (sensitivity 91%, specificity 81%).

Congenital Heart Surgery Training Experiences and Perceptions Among Cardiothoracic Surgery Residents.

Interest and core training in congenital heart surgery (CHS) has not been characterized among current cardiothoracic surgical trainees. This study aimed to evaluate perceptions, interest, exposure, and experience among current trainees. A 22 question survey was distributed to all cardiothoracic surgical trainees in ACGME-accredited thoracic surgery residencies. Questions included demographics, exposure to CHS during, perceptions of, participation in and quality assessment of CHS training. There were 106 responses (20.1% response rate) of which 31 (29.0%) were female and 87 (81.3%) were cardiothoracic track. While 69 (64.5%) reported having an interest in CHS at some point during training, only 24 (22.4%) were actively pursuing CHS. All but 7 (6.5%) residents reported having easy access to congenital mentorship, with 35 (32.7%) actively participating in CHS research. Three months was the median duration of congenital rotations. Residents reported less operative participation on CHS compared to adult cardiac surgery. Several residents noted the need for earlier exposure and increased technical/operative experience as areas in need of improvement. The most cited primary influences to pursue CHS included: mentorship, breadth of pathology, and technical nature of the specialty. Lack of consistent job availability and length of additional training were reported as negative influences. Cardiothoracic residents report adequate exposure to obtain case requirements and knowledge for board examinations in CHS but highly variable operative involvement. Mentorship and early exposure remain important for those interested in CHS, while additional training time and limited job availability remain hurdles to CHS.

Anatomic Position and Durability of Polytetrafluoroethylene Conduit ≥18 mm: Single-Center Experience.

BACKGROUND: Conduit longevity after right ventricular outflow tract (RVOT) reconstruction is determined by the interaction of different factors. We evaluated the relationship between conduit anatomic position and long-term durability among ≥18 mm polytetrafluoroethylene (PTFE) conduits. METHODS: A single-institution RVOT reconstructions using a PTFE conduit ≥18 mm were identified. Catheter-based interventions or the need for conduit replacement were comparatively assessed between orthotopic vs heterotopic conduit position. Time to the first reintervention, censored by death, was compared between the groups. RESULTS: A total of 102 conduits were implanted in 99 patients, with a median age of 13.2 years (interquartile range [IQR] 8.9-17.8 years), median weight of 47 kg (IQR, 29-67 kg), and body surface area of 1.4 m2 (IQR, 1-1.7 m2). Overall, 50.9% (n = 52) of conduits were placed in an orthotopic position after the Ross procedure in congenital aortic valve abnormalities (80% [n = 36]). Tetrology of Fallot in 39% (n = 18), followed by truncus arteriosus with 33% (n = 15), were the most common in the heterotopic position. Trileaflet configuration was similar (67% vs 69%; P = .32) between the groups. Survival free from reintervention was 91% (95% CI, 79-97) and 88% (95% CI, 71-95) in the orthotopic and the heterotopic group, respectively, at 5 years, without differences in the Kaplan Meier curves (log-rank >.05). CONCLUSIONS: RVOT reconstruction with PTFE conduits ≥ 8 mm showed >90% conduit survival free from replacement in our cohort at 5 years. The anatomic position of the PTFE conduit does not seem to impact intermediate durability.

Pulmonary Homograft vs Handmade Polytetrafluoroethylene-Valved Conduits After the Ross Procedure.

BACKGROUND: This study aimed to longitudinally compare expanded polytetrafluoroethylene (ePTFE)-valved conduits vs pulmonary homograft (PH) conduits after right ventricular outflow tract reconstruction in the Ross procedure. METHODS: Patients undergoing a Ross procedure from June 2004 to December 2021 were identified. Echocardiographic data, catheter-based interventions, or conduit replacements, as well as time to first reintervention or replacement, were comparatively assessed between handmade ePTFE-valved conduits and PH conduits. RESULTS: A total of 90 patients were identified. The median age and weight were 13.8 years (interquartile range [IQR], 8.08-17.80 years) and 48.3 kg (IQR, 26.8-68.7 kg), respectively. There were 66% (n = 60) ePTFE-valved conduits and 33% (n = 30) PHs. The median size was 22 mm (IQR, 18-24 mm) for ePTFE-valved conduits and 25 mm (IQR, 23-26 mm) for PH conduits (P < .001). Conduit type had no differential effect in the gradient evolution or the odds of presenting with severe regurgitation in the last follow-up echocardiogram. Of the 26 first reinterventions, 81% were catheter-based interventions, without statistically significant differences between the groups (69% PH vs 83% ePTFE). The overall surgical conduit replacement rate was 15% (n = 14), and it was higher in the homograft group (30% vs 8%; P = .008). However, conduit type was not associated with an increased hazard for reintervention or reoperation after adjusting for covariates. CONCLUSIONS: Right ventricular outflow tract reconstruction using handmade ePTFE-valved conduits after a Ross procedure provides encouraging midterm results, without a differential effect in hemodynamic performance or valve function compared with PH conduits. These results are reassuring about the use of handmade valved conduits in pediatric and young adult patients. Longer follow-up of tricuspid conduits will complement valve competency assessment.

Lobar displacement following pediatric heart-lung transplantation: case report and review of literature.

Lobar displacement (LD) after heart-lung transplantation (HLT) has been reported in adults, but there are no reported pediatric cases. Its occurrence may cause vascular compromise of the displaced lung segment leading to necrosis, infection and bronchiectasis, as well as compression of contralateral lobes. We report two cases of LD in children following HLT, treated differently and with different outcomes. Assessment of pulmonary perfusion and weighing the risk of surgical repair may be considered for optimal patient management of this condition.

Activated partial thromboplastin time is a better trending tool in pediatric extracorporeal membrane oxygenation.

OBJECTIVES: To determine whether activated partial thromboplastin times are a better heparin management tool than activated clotting times in pediatric extracorporeal membrane oxygenation. DESIGN: A single-center retrospective analysis of perfusion and patient records. SETTING: Academic pediatric tertiary care center. PATIENTS: Pediatric patients (<21 yrs old) requiring extracorporeal membrane oxygenation support initiated at Children's Hospital of Pittsburgh. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Point-of-care activated clotting time and activated partial thromboplastin time values, clinical laboratory activated partial thromboplastin time values, weight-normalized heparin administration (units/kg/hr), and reported outcomes were collected for pediatric patients treated for cardiac and/or respiratory failure with extracorporeal membrane oxygenation. Spearman's ranked correlations were performed for each coagulation test compared to heparin dosage. The Bland-Altman test was used to determine the validity of the point-of-care activated partial thromboplastin time. Hazard analysis was conducted for outcomes and complications for patients whose heparin management was based on the clinical laboratory activated partial thromboplastin time or the activated clotting time. Only the clinical laboratory activated partial thromboplastin time showed a correlation (ρ = 0.40 vs. ρ = -0.04 for activated clotting time) with the heparin administration (units/kg/hr). Point-of-care activated partial thromboplastin time and activated partial thromboplastin time values correlated well (ρ = 0.76), with <5% of samples showing a difference outside 2 SDs, but differences in their absolute values (Δactivated partial thromboplastin time = 100 secs) preclude them from being interchangeable measures. Furthermore, despite no effective change in the mean activated clotting time, cardiac patients showed a significantly improved correlation to heparin dose for all coagulation tests (e.g., point-of-care activated partial thromboplastin time ρ = 0.60). Management of patients with the clinical laboratory activated partial thromboplastin time did not significantly affect patient survival rates but did significantly reduce bleeding complications and significantly increased clotting in the extracorporeal membrane oxygenation circuit. A hazard analysis demonstrated that bleeding complications were associated with an increased risk of mortality, whereas clotting complications in the extracorporeal membrane oxygenation circuit were not. CONCLUSIONS: The activated clotting time is not an accurate monitoring tool for heparin management in pediatricextracorporeal membrane oxygenation. The point-of-care activated partial thromboplastin time correlates well with the clinical laboratory activated partial thromboplastin time but cannot be substituted for the clinical laboratory activated partial thromboplastin time values. Management of pediatric extracorporeal membrane oxygenation patients with the clinical laboratory activated partial thromboplastin time reduced bleeding complications which are associated with increases in mortality.