Publications by authors named "Mohammed Al-Aklabi"

24 Publications

  • Page 1 of 1

Surgical Repair of a Transannular Rupture During Transfemoral Transcatheter Aortic Valve Replacement.

Clin Med Insights Case Rep 2021 6;14:11795476211038126. Epub 2021 Aug 6.

Division of Cardiac Surgery, Department of Surgery, University of Alberta, Edmonton, AB, Canada.

Annular rupture is a rare but life-threatening complication of transcatheter aortic valve replacement (TAVR). Mortality rates are high if immediate intervention, most often necessitating surgical repair, is not performed. Herein, we describe an 87-year-old man who, after deployment of TAVR, experienced acute decompensation and required urgent conversion to a midline sternotomy to repair an aortic annular rupture. This case demonstrates an example of a rare but severe complication of TAVR. This report provides an in-depth description of the surgical approach to repair an aortic annular rupture and demonstrates the utility of performing minimally invasive procedures inside a hybrid operating room.
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http://dx.doi.org/10.1177/11795476211038126DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8361553PMC
August 2021

Paracorporeal Support in Pediatric Patients: The Role of the Patient-Device Interaction.

Ann Thorac Surg 2021 Jul 28. Epub 2021 Jul 28.

Department of Pediatric Cardiology University of Alberta, Edmonton AB, Canada; Division of Pediatric Cardiology, Stollery Children's Hospital, Edmonton AB, Canada. Electronic address:

Background: Ventricular assist devices (VADs) are important in the treatment of pediatric heart failure. While paracorporeal pulsatile (PP) devices have historically been used, there has been increased use of paracorporeal continuous (PC) devices. We sought to compare the outcomes of children supported with a PP, PC, or combination of devices.

Methods: Retrospective review (2005-19) of patients <19 years of age from a single center, who received a PC, PP or combination of devices. Patient characteristics were compared between device strategies and Kaplan-Meier survival analysis was performed.

Results: Sixty-six patients were included (62% male, 62% non-congenital heart disease, median age 0.9 years (IQR 0.2, 4.9), median weight 8.5 kg (IQR 4.3, 17.7). PC devices were used in 45% of patients, PP in 35% and a combination in 20%. Patients on PC devices had a lower median weight (p=.02), a higher proportion of CHD (p=.02) and more patients requiring pre-VAD dialysis (p=.01). There was no difference in pre-VAD ECMO (p=.15) use. There was a difference in survival between the three device strategies (p=.02) CONCLUSIONS: Differences in survival was evident, with those on PC support having worse outcomes. Transition from PC to a PP devices was associated with a survival advantage. These findings may be driven by differences in patient characteristics across device strategies. Further studies are required to confirm these findings and to better understand the interaction between patient characteristics and device options.
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http://dx.doi.org/10.1016/j.athoracsur.2021.06.062DOI Listing
July 2021

Berlin Heart EXCOR and ACTION post-approval surveillance study report.

J Heart Lung Transplant 2021 04 19;40(4):251-259. Epub 2021 Jan 19.

Lucile Packard Children's Hospital Stanford, Stanford Children's Health, Palo Alto, California.

Background: The Berlin Heart EXCOR Pediatric (EXCOR) ventricular assist device (VAD) was introduced in North America nearly 2 decades ago. The EXCOR was approved under Humanitarian Device Exemption status in 2011 and received post-market approval (PMA) in 2017 from Food and Drug Administration. Since the initial approval, the field of pediatric mechanical circulatory support has changed, specifically with regard to available devices, anticoagulation strategies, and the types of patients supported. This report summarizes the outcomes of patients supported with EXCOR from the Advanced Cardiac Therapies Improving Outcomes Network (ACTION) registry. These data were part of the PMA surveillance study (PSS) required by the Food and Drug Administration.

Methods: ACTION is a learning collaborative of over 40 pediatric heart failure programs worldwide, which collects data for all VAD implantations as one of its initiatives. All patients in North America with EXCOR implants reported to ACTION from 2018 to 2020 (n = 72) who had met an outcome were included in the EXCOR PSS group. This was compared with a historical, previously reported Berlin Heart EXCOR study group (Berlin Heart study [BHS] group, n = 320, 2007‒2014).

Results: Patients in the PSS group were younger, were smaller in weight/body surface area, were more likely to have congenital heart disease, and were less likely to receive a bi-VAD than those in the BHS group. Patients in the PSS group were less likely to be in Interagency Registry for Mechanically Assisted Circulatory Support Profile 1 and were supported for a longer duration. The primary anticoagulation therapy for 92% of patients in the PSS group was bivalirudin. Success, defined as being transplanted, being weaned for recovery, or being alive on a device at 180 days after implantation, was 86% in the PSS group compared with 76% in the BHS group. Incidence of stroke was reduced by 44% and the frequency of pump exchange by 40% in the PSS group compared with those in the BHS group. Similarly, all other adverse events, including major bleeding, were reduced in the PSS group.

Conclusions: The PSS data, collected through ACTION, highlight the improvement in outcomes for patients supported with EXCOR compared with the outcomes in a historical cohort. These findings may be the result of changes in patient care practices over time and collaborative learning.
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http://dx.doi.org/10.1016/j.healun.2021.01.010DOI Listing
April 2021

Health-related quality of life after pediatric heart transplantation in early childhood.

Pediatr Transplant 2020 11 1;24(7):e13822. Epub 2020 Sep 1.

Division of Pediatric Cardiology, Department of Pediatrics, University of Alberta, Edmonton, AB, Canada.

Background: There is limited information about HRQL after pediatric heart transplantation at a young age.

Methods: Prospective follow-up study of children who received a heart transplant at age ≤4 years. HRQL was assessed using the PedsQL 4.0 at age 4.5 years. This cohort was compared with healthy children, children with CHD, and with chronic conditions. Peri-operative factors associated with HRQL were also explored.

Results: Of 66 eligible patients, 15 (23%) died prior to the HRQL assessment and 2 (3%) were lost to follow-up, leaving 49 patients. Indication for transplantation was CHD in 27 (55%) and CMP in 22 (45%). Median age (IQR) at transplant was 9 (5-31) months. HRQL was significantly lower in transplanted children compared to population norms (65.3 vs 87.3, P < .0001), children with chronic conditions (65.3 vs 76.1, P = .001), and children with CHD (65.3 vs 81.1, P < .0001). Transplanted children with CHD had lower HRQL than those with a prior diagnosis of CMP (59.5 vs 72.5, P-value = .020). Higher creatinine pretransplant and higher lactate post-operatively were associated with lower HRQL.

Conclusion: Children after heart transplant had significantly lower HRQL, as reported by their parents, than the normative population, children with chronic conditions, and children with CHD.
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http://dx.doi.org/10.1111/petr.13822DOI Listing
November 2020

Discharge and Readmission to the Pediatric Cardiac ICU in Pediatric Patients With Durable Ventricular Assist Devices.

Pediatr Crit Care Med 2020 09;21(9):e810-e818

Department of Pediatric Cardiology, University of Alberta, Edmonton, AB, Canada.

Objectives: Pediatric patients implanted with a durable ventricular assist device are initially managed in the pediatric cardiac ICU but are eligible for discharge to the ward. Our objectives were to characterize discharge and readmission of ventricular assist device patients to the pediatric cardiac ICU, identify risk factors for readmission, and determine whether discharge or readmission is associated with mortality.

Design: Retrospective study.

Setting: Stollery Children's Hospital.

Patients: Patients implanted with a durable ventricular assist device at less than 18 years old between 2005 and 2016.

Interventions: None.

Measurements And Main Results: There were 44 patients who underwent ventricular assist device implantation at a median age of 3.7 years (interquartile range, 0.6-9.0 yr), with the most common etiology being noncongenital heart disease (76.7%). Median time of total ventricular assist device support was 110.0 days (interquartile range, 42.3-212.3 d) with the median index pediatric cardiac ICU stay being 34.0 days (interquartile range, 19.8-81.0 d). Thirty patients (68.0%) were discharged to the ward with 18 (60.0%) having at least one readmission. The median time to first readmission was 18.0 days (interquartile range, 14.8-109.8 d) with a median of two readmissions per patient (interquartile range, 1.0-3.0). The most common reason for readmission was pump thrombosis (30.4%), followed by neurologic dysfunction (23.9%). There were no statistically significant pre- or post-implant factors associated with readmission, and readmission was not associated with mortality (p = 0.600). Univariate Kaplan-Meier survival analysis indicated that use of pre-implant extracorporeal membrane oxygenation, post-implant continuous renal replacement therapy, as well as failure to be discharged from the index pediatric cardiac ICU stay were associated with mortality.

Conclusions: Readmissions to the pediatric cardiac ICU occurred in 60.0% of pediatric patients on durable ventricular assist devices with the first readmission occurring within a month of discharge from the index pediatric cardiac ICU stay. While readmission was not associated with mortality, lack of discharge from index pediatric cardiac ICU stay was likely due to a worse pre-implant clinical status.
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http://dx.doi.org/10.1097/PCC.0000000000002456DOI Listing
September 2020

End-Stage Liver Disease Models and Outcomes in Pediatric Patients Supported With Short-Term Continuous-Flow Ventricular Assist Devices.

ASAIO J 2020 08;66(8):933-938

Division of Pediatric Cardiology, University of Alberta, Alberta, Canada.

Short-term continuous-flow ventricular assist devices (STCF-VADs) are increasingly being utilized in pediatrics. End-stage liver disease (ELD) models have been associated with outcomes in adult patients on mechanical circulatory support. We sought to determine the relationship between outcomes in children on STCF-VADs and three ELD models: model for end-stage liver disease-excluding international normalized ratio (MELD-XI; all) and MELD-XI (> 1 year), PELD, and a novel score, PedMELD-XI. All patients (< 19 years) supported with STCF-VADs, between June 2009 and December 2016 were included. The MELD-XI, PELD, and PedMELD-XI scores were calculated and their association with adverse events and a composite measure of death, major bleeding, and neurologic dysfunction was analyzed. Of 32 patients, median age was 0.57 years (interquartile range [IQR], 0.10-4.43), median weight was 7.15 kg (IQR, 3.68-16.53), 53.1% had congenital heart disease, and 53.1% were male. In total, 78.1% patients experienced an adverse event (78.1% a major bleed, 25.0% neurologic dysfunction, and 15.6% death). The median MELD-XI score was 11.17 (IQR, 9.44-30.01), MELD-XI (>1 year) 9.44 (IQR, 9.44-24.33), PELD 6.00 (IQR, 4.00-13.75), and PedMELD-XI -14.91 (IQR, -18.85 to -12.25). A higher MELD-XI for all ages (13.80 vs. 9.44, p = 0.037) and less negative PedMELD-XI (-14.16 vs. -19.34, p = 0.028) scores were significantly associated with bleeding and the composite outcome. PedMELD-XI was significantly associated with death (-12.87 vs. -16.84, p = 0.041) while a trend was seen for increased MELD-XI in all ages being associated with death (31.52 vs. 10.11, p = 0.051). Last, there was no association with the models and neurologic events. MELD-XI and PedMELD-XI were significantly associated with major bleeding and the composite endpoints with PedMELD-XI also being associated with death. These results suggest that ELD models can be used to predict outcomes in this specific patient population, however, further analysis in a larger population is required.
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http://dx.doi.org/10.1097/MAT.0000000000001078DOI Listing
August 2020

Tricuspid Intervention Following Pulmonary Valve Replacement in Adults With Congenital Heart Disease.

J Am Coll Cardiol 2020 03;75(9):1033-1043

Montreal Heart Institute, Université de Montréal, Montreal, Quebec, Canada; Centre hospitalier universitaire Sainte-Justine, Université de Montréal, Montreal, Quebec, Canada.

Background: Tricuspid regurgitation (TR) is common among adults with corrected tetralogy of Fallot (TOF) or pulmonary stenosis (PS) referred for pulmonary valve replacement (PVR). Yet, combined valve surgery remains controversial.

Objectives: This study sought to evaluate the impact of concomitant tricuspid valve intervention (TVI) on post-operative TR, length of hospital stay, and on a composite endpoint consisting of 7 early adverse events (death, reintervention, cardiac electronic device implantation, infection, thromboembolic event, hemodialysis, and readmission).

Methods: The national Canadian cohort enrolled 542 patients with TOF or PS and mild to severe TR who underwent isolated PVR (66.8%) or PVR+TVI (33.2%). Outcomes were abstracted from charts and compared between groups using multivariable logistic and negative binomial regression.

Results: Median age at reintervention was 35.3 years. Regardless of surgery type, TR decreased by at least 1 echocardiographic grade in 35.4%, 66.9%, and 92.8% of patients with pre-operative mild, moderate, and severe insufficiency. In multivariable analyses, PVR+TVI was associated with an additional 2.3-fold reduction in TR grade (odds ratio [OR]: 0.44; 95% confidence interval [CI]: 0.25 to 0.77) without an increase in early adverse events (OR: 0.85; 95% CI: 0.46 to 1.57) or hospitalization time (incidence rate ratio: 1.17; 95% CI: 0.93 to 1.46). Pre-operative TR severity and presence of transvalvular leads independently predicted post-operative TR. In contrast, early adverse events were strongly associated with atrial tachyarrhythmia, extracardiac arteriopathy, and a high body mass index.

Conclusions: In patients with TOF or PS and significant TR, concomitant TVI is safe and results in better early tricuspid valve competence than isolated PVR.
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http://dx.doi.org/10.1016/j.jacc.2019.12.053DOI Listing
March 2020

Management of Critical Coarctation of Aorta in a Premature Neonate With Low Birth Weight.

Ann Thorac Surg 2020 09 8;110(3):e225-e226. Epub 2020 Feb 8.

Division of Pediatric Cardiac Surgery, Stollery Children's Hospital, Mazankowski Alberta Heart Institute, University of Alberta, Edmonton, Alberta, Canada.

Premature neonates with low birth weight have always posed a huge management dilemma, even for teams with great expertise. In this case report, we discuss a premature neonate born with extremely low birth weight diagnosed with critical coarctation of aorta and the challenges faced in stabilizing the neonate. As per our literature review, this is the smallest neonate, weighing only 680 g, to have undergone successful repair of coarctation of aorta through posterolateral thoracotomy. Identifying the ideal timing for surgery in premature neonates with low birth weight is most crucial to minimize morbidity and mortality.
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http://dx.doi.org/10.1016/j.athoracsur.2019.12.057DOI Listing
September 2020

Cryopreservation timing is a critical process parameter in a thymic regulatory T-cell therapy manufacturing protocol.

Cytotherapy 2019 12 3;21(12):1216-1233. Epub 2019 Dec 3.

School of Biomedical Engineering, University of British Columbia, Vancouver, BC, Canada; BC Children's Hospital Research Institute, Vancouver, BC, Canada; Department of Surgery, University of British Columbia, Vancouver, BC, Canada. Electronic address:

Regulatory T cells (Tregs) are a promising therapy for several immune-mediated conditions but manufacturing a homogeneous and consistent product, especially one that includes cryopreservation, has been challenging. Discarded pediatric thymuses are an excellent source of therapeutic Tregs with advantages including cell quantity, homogeneity and stability. Here we report systematic testing of activation reagents, cell culture media, restimulation timing and cryopreservation to develop a Good Manufacturing Practice (GMP)-compatible method to expand and cryopreserve Tregs. By comparing activation reagents, including soluble antibody tetramers, antibody-conjugated beads and artificial antigen-presenting cells (aAPCs) and different media, we found that the combination of Dynabeads Treg Xpander and ImmunoCult-XF medium preserved FOXP3 expression and suppressive function and resulted in expansion that was comparable with a single stimulation with aAPCs. Cryopreservation tests revealed a critical timing effect: only cells cryopreserved 1-3 days, but not >3 days, after restimulation maintained high viability and FOXP3 expression upon thawing. Restimulation timing was a less critical process parameter than the time between restimulation and cryopreservation. This systematic testing of key variables provides increased certainty regarding methods for in vitro expansion and cryopreservation of Tregs. The ability to cryopreserve expanded Tregs will have broad-ranging applications including enabling centralized manufacturing and long-term storage of cell products.
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http://dx.doi.org/10.1016/j.jcyt.2019.10.011DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7474528PMC
December 2019

Predicting Post-Fontan Length of Stay: The Limits of Measured Variables.

Pediatr Cardiol 2019 Aug 22;40(6):1208-1216. Epub 2019 Jun 22.

Department of Pediatrics, University of Alberta, Edmonton, Canada.

Post-operative length of stay (LOS) is an important metric for both healthcare providers and patients and their families. Predicting LOS is a challenge as it is sensitive to multitudinous patient and system factors. All subjects undergoing a Fontan from 1996-2016 who survived to hospital discharge were included. Details about the pre-operative status, operative conduct, and post-operative course of each patient were obtained. The association between patient characteristics and post-Fontan LOS were determined using stepwise multivariable regression models. Of 320 subjects who underwent a Fontan, 314 (98.1%) survived to hospital discharge. Median age at Fontan was 3.3 years (IQR 2.8, 4.0) and the most common underlying diagnosis was hypoplastic left heart syndrome (106, 33.8%). Median post Fontan LOS was 11 days (IQR 8, 17). Univariable risk factors for longer LOS included number of previous surgeries, post-Glenn LOS, cardiopulmonary bypass time, post-operative chylothorax, and failure to extubate in the operating room (all p < 0.05). In multivariable models, number of previous operations, extubation in the operating room, and postoperative complications predicted LOS (R = 0.5185 for full model). The proportion of patients discharged on week days (14.7-18.8% per day) was significantly higher than the proportion discharged on weekend days (5.1-9.9% per weekend day). Pre-operative variables have limited use in predicting post-Fontan length of stay. The most important predictors of post-operative LOS are extubation in the operating room and the occurrence of post-operative complications. However, a significant proportion of variability in LOS was not explained by available measurable variables.
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http://dx.doi.org/10.1007/s00246-019-02134-yDOI Listing
August 2019

Pediatric extracorporeal membrane oxygenation (ECMO): a guide for radiologists.

Pediatr Radiol 2018 09 6;48(10):1488-1502. Epub 2018 Aug 6.

Department of Radiology and Diagnostic Imaging, University of Alberta, 8440-112 St., Edmonton, Alberta, T6G 2B7, Canada.

Extracorporeal membrane oxygenation (ECMO) is a life-saving treatment for pediatric patients with respiratory and/or cardiac failure. The ECMO circuit oxygenates and sometimes pumps the blood, effectively replacing lung and/or heart function temporarily. ECMO patients are clinically very complex not only because of their underlying, life-threatening pathology, but also because of the many physiological parameters that must be monitored and adjusted to maintain adequate tissue perfusion and oxygenation. Drainage and reinfusion cannulae connecting the patient to the ECMO circuit are visible on radiograph. These cannulae have different functions, different configurations, different radiographic appearances, and different positions that should be familiar to the interpreting pediatric radiologist. The primary complications of ECMO include hemorrhage, thrombosis and ischemia, as well as equipment failure and cannula malpositioning, all of which may be detected on imaging. In this pictorial essay, we discuss the basics of ECMO function and clinical management, ECMO cannula features and configurations, and the many complications of ECMO from an imaging perspective. Our goal is to educate pediatric radiologists about ECMO imaging, equipping them to properly interpret these studies and to become a useful consultant in ECMO patient care.
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http://dx.doi.org/10.1007/s00247-018-4211-zDOI Listing
September 2018

Cardiac Surgery-Associated Kidney Injury in Children and Renal Oximetry.

Pediatr Crit Care Med 2018 09;19(9):839-845

Division of Pediatric Nephrology, Department of Pediatrics, Stollery Children's Hospital, University of Alberta, Edmonton, AB, Canada.

Objectives: Cardiac surgery-associated acute kidney injury is common in children and associates with negative outcomes. Novel interventions to reduce cardiac surgery-associated acute kidney injury require knowledge of its pathophysiology. States of altered perfusion, oxygen delivery, and energy consumption occur during cardiopulmonary bypass and could protect against or contribute to renal cellular injury and recovery. Near-infrared spectroscopy is noninvasive technology for monitoring regional blood flow and tissue oxygenation. This study evaluated the relationship between renal regional oxygen saturation and cardiac surgery-associated acute kidney injury, using near-infrared spectroscopy monitoring before, during, and after cardiopulmonary bypass in children.

Design: Prospective cohort study.

Setting: Single-center, tertiary care pediatric hospital (Stollery Children's Hospital, Edmonton, AB, Canada).

Patients: Children less than or equal to 10 kg undergoing congenital heart disease repair with cardiopulmonary bypass. Heart transplant, preoperative dialysis, sepsis, extracorporeal life support, congenital renal disease, and preoperative nephrotoxins were exclusions.

Interventions: Renal regional near-infrared spectroscopy monitoring before, during, and after cardiopulmonary bypass.

Measurements And Main Results: Outcome measure was cardiac surgery-associated acute kidney injury (defined according to Kidney Disease: Improving Global Outcomes criteria). Regional oxygen saturation was measured continuously using near-infrared spectroscopy (INVOS 5100C Cerebral/Somatic Oximeter; Medronic, Troy, MI) from time of anesthesia to time of transfer to intensive care. Cardiac surgery-associated acute kidney injury occurred in 65%. Lower baseline (precardiopulmonary bypass) regional oxygen saturation was associated with decreased risk of cardiac surgery-associated acute kidney injury (p = 0.01); children with baseline regional oxygen saturation in the highest tertile were 7.14 times more likely to get cardiac surgery- associated acute kidney injury (vs lowest tertile). Area under the curve for ability of baseline regional oxygen saturation to predict cardiac surgery-associated acute kidney injury was 0.73 (95% CI, 0.60-0.85). Children with lower baseline glomerular filtration rate had lower mean renal regional oxygen saturation.

Conclusions: Findings demonstrate that preoperative oxygen supply/demand balance is an important predictor of cardiac surgery-associated acute kidney injury, suggesting lower preoperative (and intraoperative) renal blood flow may be protective. There is not yet a definite link between remote ischemic preconditioning and prevention of cardiac surgery-associated acute kidney injury; however, renal protective effects of sublethal ischemia should continue to be explored.
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http://dx.doi.org/10.1097/PCC.0000000000001656DOI Listing
September 2018

Tricuspid Valve Repair in Infancy Using Neochordae: Three-Dimensional Echocardiographic Imaging.

World J Pediatr Congenit Heart Surg 2017 11;8(6):740-742

3 Department of Surgery, University of Alberta, Edmonton, Alberta, Canada.

Tricuspid regurgitation (TR) in infancy poses a surgical challenge. Both two- and three-dimensional echocardiography (3DE) can provide detailed information about the mechanism(s) of valve failure and insights into valve adaptation during follow-up. We report two patients who underwent tricuspid valve repair using Gore-Tex neochordae, repairs which were facilitated by and assessed with 3DE. Both infants had less than mild residual TR and no valve tethering at hospital discharge. Furthermore, follow-up 3DEs have helped to confirm valve competence, lack of tethering, and growth of the valve and valve apparatus.
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http://dx.doi.org/10.1177/2150135117736286DOI Listing
November 2017

Hypoplastic Left Heart Syndrome Is Not a Predictor of Worse Intermediate Mortality Post Fontan.

Ann Thorac Surg 2017 Dec 31;104(6):2037-2044. Epub 2017 Oct 31.

Department of Surgery, University of Alberta, Edmonton, Canada.

Background: An increasing proportion of those living with single ventricle physiology have hypoplastic left heart syndrome (HLHS). Our objective was to assess the association between HLHS and outcomes post Fontan operation.

Methods: All pediatric patients who underwent a Fontan procedure at the University of Alberta between 1996 and 2016 were included. Follow-up clinical data collected included early and late surgical or catheter reintervention, echocardiography, and long-term transplant-free survival. Characteristics were compared between those with and without HLHS, and the association between outcomes and HLHS were assessed.

Results: A total of 320 children (median age 3.3 years, interquartile range 2.8 to 3.9 years; 121 [43.4%] female) underwent a Fontan procedure over the course of the study. Nearly one third of subjects had HLHS (107, 33.4%). Patients with HLHS were more likely to have abnormal ventricular function (19.6% versus 7.0%, p = 0.003) and worse than mild atrioventricular valve (AVV) regurgitation (23.4 versus 9.2%, p = 0.001) preoperatively. HLHS was not predictive of in-hospital Fontan failure (odds ratio 0.82, 95% CI 0.28, 2.39), late reintervention (hazard ratio [HR] 1.08, 95% CI 0.66, 1.76), or transplant-free survival (HR 1.58, 95% CI 0.72, 3.44). Subjects with HLHS were more likely to have more than mild AVV regurgitation (31.6% versus 13.3%, p = 0.028) and abnormal ventricular function (29.8% versus 10.7%, p < 0.0001) at late follow-up.

Conclusions: Patients with HLHS who survive to the Fontan procedure do no worse with the operation than those with other anatomy. Given worse late ventricular function and AVV regurgitation, equivalent survival may not persist throughout a patient's life course.
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http://dx.doi.org/10.1016/j.athoracsur.2017.08.032DOI Listing
December 2017

Post-operative Outcomes in Children Undergoing Fontan Palliation in a Regionalized Surgical System.

Pediatr Cardiol 2017 Dec 22;38(8):1654-1662. Epub 2017 Aug 22.

Department of Surgery, University of Alberta, Edmonton, AB, Canada.

Evidence suggests that outcomes in pediatric cardiac surgery are improved by consolidating care into centers of excellence. Our objective was to determine if outcomes are equivalent in patients across a large regional referral base, or if patients from centers without on-site surgery are at a disadvantage. Since 1996, all pediatric cardiac surgery has been offered at one of two centers within the region assessed, with the majority being performed at Stollery Children's Hospital. All patients who underwent a Fontan between 1996 and 2016 were included. Follow-up data including length of stay (LOS), repeat surgical interventions, and transplant-free survival were acquired for each patient. The association between post-operative outcomes and home center was assessed using Kaplan-Meier survival analysis and Cox proportional Hazards models. 320 children (median age 3.3 years, IQR 2.8-4.0) were included; 120 (37.5%) had the surgical center as their home center. Cardiac anatomy was hypoplastic left heart syndrome in 107 (33.4%) subjects. Median LOS was 11 days (IQR, 8-17), and there were 8 in-hospital deaths. There were 17 deaths and 11 transplants over the course of follow-up. Five-year transplant-free survival was 92.5%. There was no difference in hospital re-intervention, late re-intervention, or survival by referral center (all p > 0.05). In multivariable analysis, home center was not predictive of either LOS (R  = -0.40, p = 0.87) or transplant-free survival (1.52, 95%CI 0.66, 3.54). In children with complex congenital heart disease, a regionalized surgical care model achieves good outcomes, which do not differ according to a patient's home base.
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http://dx.doi.org/10.1007/s00246-017-1710-xDOI Listing
December 2017

Neonatal Tracheal and Intracardiac Repair in a High-Risk Premature Infant Requiring Preoperative ECMO Transport.

World J Pediatr Congenit Heart Surg 2019 05 20;10(3):380-383. Epub 2017 Aug 20.

1 Division of Cardiac Surgery, Department of Surgery, University of Alberta, Edmonton, Alberta, Canada.

Congenital tracheal stenosis is an uncommon malformation that portends a poor outcome in children who are symptomatic in the neonatal period. Over time, the management of significant tracheal disease has been consolidated at high-volume centers, and increasingly complex patients have undergone surgical repair. We present a premature newborn boy who was diagnosed with critical multi-level airway and cardiac disease who decompensated at a remote site, requiring extracorporeal membrane oxygenation support for transport. He underwent a complete repair including a slide tracheoplasty and was successfully discharged home, with no residual stenosis at follow-up.
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http://dx.doi.org/10.1177/2150135117696490DOI Listing
May 2019

Neurologic, Neurocognitive, and Functional Outcomes in Children Under 6 Years Treated with the Berlin Heart Excor Ventricular Assist Device.

ASAIO J 2017 Mar/Apr;63(2):207-215

From the *Division of Pediatric Neurology, Department of Pediatrics, University of Alberta, Edmonton, Canada; †Division of Developmental Pediatrics, Department of Pediatrics, University of Alberta, Edmonton, Canada; ‡Pediatric Rehabilitation, Glenrose Rehabilitation Hospital, Edmonton, Canada; §Division of Pediatric Critical Care, Department of Pediatrics, University of Alberta and Stollery Children's Hospital, Edmonton, Canada; ¶Division of Pediatric Cardiology, Department of Pediatrics, University of Alberta and Stollery Children's Hospital, Edmonton, Canada; and ‖Division of Cardiac Surgery, Department of Surgery, University of Alberta, Edmonton, Canada.

The objective of this study is to describe the neurologic, neurocognitive, and functional outcomes of children aged under 6 years supported on the Berlin Heart EXCOR ventricular assist device (VAD) followed in the Complex Pediatric Therapies Follow-up Program (CPTFP). Sixteen patients were prospectively followed through this longitudinal, developmental program. The patients were evaluated with neurologic physical examination. Intelligence quotients (IQ) and functional outcome scores (ABAS-II scores) were obtained. Neuroimaging reports from before, during, and after VAD implantation were retrospectively reviewed for reported brain injury (BI). Twelve patients (75%) had neuroimaging documented BI at some point in their life (i.e., before, during, or after VAD support). Five patients (31%) had neuroimaging evidence of acute BI incurred while on the VAD. The high overall number of patients with neuroimaging documented BI at any point in their life illustrates that the risk for BI also exists outside the window of VAD support. Patients with abnormal neurologic physical examination at follow-up had lower IQ and ABAS-II scores compared with patients with normal neurologic physical examination (mean full-scale IQ 66.9 vs. 95.0, p = 0.001; mean ABAS-II 66.3 vs. 94.2, p < 0.001).
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http://dx.doi.org/10.1097/MAT.0000000000000462DOI Listing
October 2017

Carotid Artery Dissection Following Neck Cannulation for Extracorporeal Life Support.

World J Pediatr Congenit Heart Surg 2017 05 3;8(3):414-416. Epub 2016 May 3.

4 Department of Cardiac Surgery, Stollery Children's Hospital, Edmonton, Alberta, Canada.

A term neonate was cannulated for venoarterial extracorporeal life support (ECLS) via the right neck for non-postoperative junctional ectopic tachycardia. Initial echocardiogram demonstrated an echogenic strand in the transverse arch. Computed tomography angiogram confirmed arterial dissection of the right common carotid artery that extended into the proximal transverse arch. Dissection flap was repaired at the time of ECLS decannulation without cardiopulmonary bypass. Follow-up computed tomography angiogram revealed a segment of narrowing of approximately 50% of the right common carotid artery without false lumen or aneurysm.
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http://dx.doi.org/10.1177/2150135116639542DOI Listing
May 2017

Supporting pediatric patients with short-term continuous-flow devices.

J Heart Lung Transplant 2016 05 8;35(5):603-9. Epub 2016 Feb 8.

Division of Pediatric Cardiac Surgery, Stollery Children's Hospital, Edmonton, Alberta, Canada.

Background: Short-term continuous-flow ventricular assist devices (STCF-VADs) are increasingly being used in the pediatric population. However, little is known about the outcomes in patients supported with these devices.

Methods: All pediatric patients supported with a STCF-VAD, including the Thoratec PediMag or CentriMag, or the Maquet RotaFlow, between January 2005 and May 2014, were included in this retrospective single-center study.

Results: Twenty-seven patients (15 girls [56%]) underwent 33 STCF-VAD runs in 28 separate hospital admissions. The STCF-VAD was implanted 1 time in 23 patients (85%), 2 times in 2 patients (7%), and 3 times in 2 patients (7%). Implantation occurred most commonly in the context of congenital heart disease in 14 runs (42.2%), cardiomyopathy in 11 (33%), and after transplant in 6 (18%). The median age at implantation was 1.7 (interquartile range [IQR] 0.1, 4.1) years, and median weight was 8.9 kg (IQR 3.7, 18 kg). Patients were supported for a median duration of 12 days (IQR 6, 23 days) per run; the longest duration was 75 days. Before implantation, 15 runs (45%) were supported by extracorporeal membrane oxygenation (ECMO). After implantation, an oxygenator was required in 20 runs (61%) and continuous renal replacement therapy in 21 (64%). Overall, 7 runs (21%) resulted in weaning for recovery, 14 (42%) converted to a long-term VAD, 4 (12%) resulted in direct transplantation, 3 (9%) were converted to ECMO, and 5 (15%) runs resulted in death on the device or within 1 month after decannulation. The most common complication was bleeding requiring reoperation in 24% of runs. In addition, 18% of runs were associated with neurologic events and 15% with a culture-positive infection. Hospital discharge occurred in 19 of 28 STCF-VAD admissions (67%). In follow-up, with a median duration of 9.2 months (IQR 2.3, 38.3 months), 17 patients (63%) survived.

Conclusions: STCF-VADs can successfully bridge most pediatric patients to recovery, long-term device, or transplant, with an acceptable complication profile. Although these devices are designed for short-term support, longer support is possible and may serve as an alternative approach to patients not suitable for the current long-term devices.
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http://dx.doi.org/10.1016/j.healun.2016.01.1224DOI Listing
May 2016

The development of a congenital heart programme quality dashboard to promote transparent reporting of outcomes.

Cardiol Young 2015 Dec;25(8):1579-83

1Pediatric Cardiac Critical Care Unit,Division of Pediatric Critical Care,Stollery Children's Hospital,Edmonton,Canada.

In 2001, the Institute of Medicine identified healthcare transparency as a necessity for re-designing a quality healthcare system; however, despite widespread calls for publicly available transparent data, the goal remains elusive. The transparent reporting of outcome data and the results of congenital heart surgery is critical to inform patients and families who have both the wish and the ability to choose where care is provided. Indeed, in an era where data and means of communication of data have never been easier, the paucity of transparent data reporting is paradoxical. We describe the development of a quality dashboard used to inform staff, patients, and families about the outcomes of congenital heart surgery at the Stollery Children's Hospital.
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http://dx.doi.org/10.1017/S1047951115002085DOI Listing
December 2015

Chronic kidney disease in congenital heart disease patients: a narrative review of evidence.

Can J Kidney Health Dis 2015 11;2:27. Epub 2015 Aug 11.

Division of Pediatric Critical Care, Department of Pediatrics, 4-548 Edmonton Clinic Health Academy, 11405 - 87 Avenue, Edmonton, AB T6G 1C9 Canada.

Purpose Of Review: Patients with congenital heart disease have a number of risk factors for the development of chronic kidney disease (CKD). It is well known that CKD has a large negative impact on health outcomes. It is important therefore to consider that patients with congenital heart disease represent a population in whom long-term primary and secondary prevention strategies to reduce CKD occurrence and progression could be instituted and significantly change outcomes. There are currently no clear guidelines for clinicians in terms of renal assessment in the long-term follow up of patients with congenital heart disease. Consolidation of knowledge is critical for generating such guidelines, and hence is the purpose of this view. This review will summarize current knowledge related to CKD in patients with congenital heart disease, to highlight important work that has been done to date and set the stage for further investigation, development of prevention strategies, and re-evaluation of appropriate renal follow-up in patients with congenital heart disease.

Sources Of Information: The literature search was conducted using PubMed and Google Scholar.

Findings: Current epidemiological evidence suggests that CKD occurs in patients with congenital heart disease at a higher frequency than the general population and is detectable early in follow-up (i.e. during childhood). Best evidence suggests that approximately 30 to 50 % of adult patients with congenital heart disease have significantly impaired renal function. The risk of CKD is higher with cyanotic congenital heart disease but it is also present with non-cyanotic congenital heart disease. Although significant knowledge gaps exist, the sum of the data suggests that patients with congenital heart disease should be followed from an early age for the development of CKD.

Implications: There is an opportunity to mitigate CKD progression and negative renal outcomes by instituting interventions such as stringent blood pressure control and reduction of proteinuria. There is a need to invest time, thought and money to fill existing knowledge gaps to improve health outcomes in this population. This review should serve as an impetus for generation of follow-up guidelines of kidney health evaluation in patients with congenital heart disease.
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http://dx.doi.org/10.1186/s40697-015-0063-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4531493PMC
August 2015

An increased incidence of conduit endocarditis in patients receiving bovine jugular vein grafts compared to cryopreserved homograft for right ventricular outflow reconstruction.

Ann Thorac Surg 2015 Jan 18;99(1):140-6. Epub 2014 Nov 18.

Division of Pediatric Cardiac Surgery, Stollery Children's Hospital, Mazankowski Alberta Heart Institute, University of Alberta, Edmonton, Alberta, Canada.

Background: We compared the outcome of patients receiving bovine jugular vein grafts versus cryopreserved homografts for right ventricular outflow tract reconstruction.

Methods: Between 2000 and 2012, 379 conduits (244 bovine jugular vein grafts, 135 homografts) were implanted in 298 patients (median age 50 months) with a median follow-up of 3.4 years.

Results: Freedom from reoperation at 1, 5, and 7 years was 96.3%, 79.3%, and 64.2% after bovine jugular vein graft and 94.6%, 75.7%, and 68.6% after homograft insertion (p = 0.086). There were 24 cases of endocarditis, 23 associated with bovine jugular vein grafts (9.4%) and 1 associated with a homograft (0.7%; p < 0.001) at median follow-up of 44 months (range, 15 days to 10 years) after conduit implantation. After endocarditis, 15 of 24 conduits were replaced. Three patients had recurrent endocarditis in the revised conduit. Multivariate logistic regression analysis showed age less than 3 years and endocarditis to be significant risk factors associated with conduit replacement. Age more than 3 years and bovine jugular vein grafts were significant risk factors for graft endocarditis. Patients more than 3 years of age at bovine jugular vein graft implantation had significantly lower freedom from reoperation (p = 0.01).

Conclusions: Compared with homograft conduits, the use of bovine jugular vein grafts for right ventricular outflow tract reconstruction was associated with a significantly higher incidence of bacterial endocarditis and conduit deterioration in older children at our institution. That may influence decision making regarding conduit choice for right ventricular outflow tract reconstruction. Patients and practitioners should be aware of the late risks of bacterial endocarditis after bovine jugular vein graft implantation.
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http://dx.doi.org/10.1016/j.athoracsur.2014.08.034DOI Listing
January 2015

β(3)-Adrenoceptor Antagonist SR59230A Attenuates the Imbalance of Systemic and Myocardial Oxygen Transport Induced by Dopamine in Newborn Lambs.

Clin Med Insights Cardiol 2012 16;6:45-51. Epub 2012 Feb 16.

Department of Surgery, University of Alberta, Edmonton, Alberta.

Background: In neonates, the increase in O(2)-delivery (DO(2)) by dopamine is offset by a greater increase in O(2)-consumption (VO(2)). This has been attributed to β(3)-adrenergic receptors in neonatal brown fat tissue. β(3) receptors in the heart have negative inotropic properties. We evaluated the effects of SR59230A, a β(3)-antagonist, on the balance of systemic and myocardial O(2)-transport in newborn lambs treated with dopamine.

Methods: Lambs (2-5 days old, n = 12) were anesthetized and mechanically ventilated. Heart rate (HR) and rectal temperature were monitored. VO(2) was measured by respiratory mass spectrometry and cardiac output (CO) by a pulmonary artery transonic flowmeter. Arterial, jugular bulb venous and coronary sinus blood gases and lactate were measured to calculate DO(2), O(2) extraction ratio (ERO(2)), myocardial O(2) and lactate extraction ratios (mERO(2), mERlac). After baseline measurements, lambs were randomized to receive SR59230A at 5 mg/kg iv (SRG) or placebo. Both groups received incremental doses of a dopamine infusion (0-5-10-15-20 mcg/kg/min) every 15 min. Measurements were repeated at the end of each dose.

Results: After SR59230A infusion, CO and HR trended to decrease (P = 0.06), but no significant changes occurred in other parameters. Over the incremental doses of dopamine, temperature increased in both groups (P < 0.0001) but to a lesser degree in SRG (P = 0.004). CO and HR increased (P = 0.005 and 0.04) and similarly in both groups (P > 0.1). DO(2) trended to a small increase (P = 0.08). VO(2) increased in both groups (P < 0.0001) but to a lesser degree in SRG (P < 0.0001). As a result, ERO(2) increased in both groups (P < 0.0001), but to a lesser degree in SRG (P < 0.0001). mERO(2) was lower in SRG (P = 0.01) with a faster increase (P < 0.0001). mERlac was higher in SRG (P = 0.06) with a faster decrease (P = 0.04).

Conclusion: Although SR59230A tends to induce an initial drop in CO, it significantly attenuates the rise in VO(2) and hence the imbalance of systemic and myocardial O(2) transport induced by dopamine at higher doses. Studies are warranted to examine the effect of SR59230A in cases of cardiac dysfunction and increased VO(2), observed after cardiac surgery.
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http://dx.doi.org/10.4137/CMC.S8654DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3303209PMC
August 2012

Successful bilateral lung transplantation in a patient with end-stage lung disease and positive novel influenza virus (H1N1).

J Heart Lung Transplant 2010 Aug 11;29(8):898-9. Epub 2010 Jun 11.

Division of Cardiac Surgery, Heart and Lung Transplantation, University of Alberta, Edmonton, Alberta, Alberta, Canada.

In 2009, a pandemic novel influenza virus (H1N1) outbreak was declared by the World Health Organization and resulted in significant worldwide illness. This report describes a 50-year-old male with end-stage lung disease secondary to alpha(1)-anti-trypsin deficiency and chronic obstructive pulmonary disease. He was admitted for potential bilateral lung transplantation when suitable organs became available. Incidentally, he was found to have some non-specific symptoms, including malaise and myalgias. These findings were attributed to killed-virus H1N1 vaccine given 48 hours earlier. However, as a safety measure, a nasopharyngeal swab was taken, and anti-viral therapy with oseltamivir (Tamiflu) was started empirically. He underwent bilateral lung transplantation on the same day of admission. In the immediate post-operative period his nasopharyngeal swab came back positive for H1N1 influenza virus. Then, post-operatively, two consecutive bronchoalveolar lavage samples from the transplanted lungs were found to be positive for H1N1 virus. He received three-weeks of antiviral treatment post-operatively and he had uneventful procedure with favorable outcome.
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http://dx.doi.org/10.1016/j.healun.2010.04.017DOI Listing
August 2010
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