Publications by authors named "Iki Adachi"

159 Publications

Waveform-guided management of implantable ventricular assist device in a small child.

JTCVS Tech 2021 Aug 4;8:177-181. Epub 2021 Jun 4.

Congenital Heart Surgery, Michael E. DeBakey Department of Surgery, Texas Children's Hospital, Baylor College of Medicine, Houston, Tex.

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http://dx.doi.org/10.1016/j.xjtc.2021.05.023DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8350945PMC
August 2021

Commentary: Aortic homograft for Warden procedure: Insatiable curiosity.

JTCVS Tech 2020 Dec 22;4:274. Epub 2020 Oct 22.

Congenital Heart Surgery, Michael E. DeBakey Department of Surgery, Texas Children's Hospital, Baylor College of Medicine, Houston, Tex.

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http://dx.doi.org/10.1016/j.xjtc.2020.10.032DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8308262PMC
December 2020

Commentary: Evolution of pediatric living-donor lung transplant: Adaptation to unique environment.

JTCVS Tech 2020 Sep 9;3:317-318. Epub 2020 Jul 9.

Congenital Heart Surgery, Texas Children's Hospital, Houston, Tex.

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http://dx.doi.org/10.1016/j.xjtc.2020.07.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8304891PMC
September 2020

Atriopulmonary connection for mechanically assisted Fontan completion: Classic technique for modern strategy.

JTCVS Tech 2020 Sep 21;3:307-309. Epub 2020 Jun 21.

Pediatric Critical Care, Department of Pediatrics, Texas Children's Hospital and Baylor College of Medicine, Houston, Tex.

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http://dx.doi.org/10.1016/j.xjtc.2020.06.019DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8302996PMC
September 2020

Commentary: A unified chain of command for organized team performance.

JTCVS Tech 2020 Sep 15;3:298. Epub 2020 Aug 15.

Division of Congenital Heart Surgery, Michael E. DeBakey Department of Surgery, Texas Children's Hospital, Baylor College of Medicine, Houston, Tex.

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http://dx.doi.org/10.1016/j.xjtc.2020.08.030DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8304893PMC
September 2020

Mechanical Circulatory Support for the Failing Sub-Aortic Right Ventricle in Adults.

Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2021 ;24:2-9

Texas Children's Hospital Heart Center, Texas Children's Hospital and Department of Surgery, Baylor College of Medicine, Houston, Texas. Electronic address:

Patients with ccTGA or d-TGA managed via atrial switch (Mustard or Senning operations) have biventricular circulations with a sub-aortic right ventricle (2V-RV). Other than in a tiny percentage of ccTGA patients, premature heart failure (HF) is common, driven by chronic RV dilatation and dysfunction and/or tricuspid regurgitation. These patients are different from the general HF population in that they are younger, more heterogeneous, are predisposed to pulmonary hypertension and present unique and complex surgical challenges. Despite their young age, they experience disproportionately poor access to advanced therapies and are often disqualified for transplant by pulmonary hypertension, HLA sensitization, program risk-tolerance and psychosocial issues. Mechanical support of the subaortic RV with ventricular assist device (subaortic RVAD, also known as SVAD), although technically challenging, can be an effective alternative to palliative care and offers high likelihood of bridging patients to heart transplant candidacy. In addition, temporary trans-catheter SVAD Impella support has been advantageous for stabilization of decompensated 2V-RV patients or as bridge to durable SVAD support. Improved awareness of and access to specialist ACHD-HF teams offering mechanical support (and transplantation) for 2V-RV patients is increasingly urgent for this aging population, and will improve options and outcomes for these patients as HF emerges.
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http://dx.doi.org/10.1053/j.pcsu.2021.04.003DOI Listing
January 2021

Mechanical Support for The Failing Single Ventricle at Pre-Fontan Stage: Current State of The Field and Future Directions.

Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2021 ;24:10-18

Congenital Heart Surgery, Texas Children's Hospital, Department of Surgery, Baylor College of Medicine, Houston, Texas. Electronic address:

With the substantial growth of pediatric ventricular assist device (VAD) support, there has been an expansion of the target population towards more complex patients, including congenital heart disease (CHD) with single ventricle (SV) physiology. The outcomes of Stage I and Stage II SV-CHD patients on VAD support from the Pedimacs database are poor, with less than 50% survival on VAD by the 3-month mark in both. The primary objective of this article is to describe the current state of VAD support for the failing Stage I and II SV-CHD circulation, to provide insight into potential areas of outcome improvement. We reviewed the published literature in the form of database and registry reports as well as single-center studies to discuss the outcomes of Stage I and Stage II SV-CHD patients on VAD support. Registry-based studies suggest that VAD support for the failing Stage I and Stage II SV-CHD circulations is challenging. However, the more promising outcomes in several single-institutional reports for both Stage I and Stage II SV-VAD indicate that the grim picture from the databases does not reflect the best outcomes that are possible to be achieved, potentially at experienced centers with higher volumes. Areas of future study and potential improvement including timely initiation of VAD support in the cohort of patients expected to not be a candidate for standard SV palliations, pump selection and the benefits of continuous-flow devices, and the decision-making for setting up the optimum circulation for VAD support, be it Fontan completion if feasible or takedown to shunt physiology.
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http://dx.doi.org/10.1053/j.pcsu.2021.04.004DOI Listing
January 2021

Right Ventricle to Pulmonary Artery Conduit Size Is Associated with Conduit and Pulmonary Artery Reinterventions After Truncus Arteriosus Repair.

Semin Thorac Cardiovasc Surg 2021 Jun 2. Epub 2021 Jun 2.

Division of Congenital Heart Surgery, Michael E. DeBakey Department of Surgery, Texas Children's Hospital, Baylor College of Medicine; Houston, Texas. Electronic address:

We studied conduit-related risk factors for mortality, conduit reintervention, conduit replacement, and pulmonary artery (PA) reinterventions after truncus repair. Patients who underwent truncus repair at our institution between 1995 and 2019 were studied. Cox proportional hazards modeling evaluated variables for association with mortality, time to conduit reintervention, time to conduit replacement, and time to PA reintervention. Truncus was repaired in 107 patients at median age of 17 days (IQR 9-45). Median follow-up time was 7 years. Aortic homografts were implanted in 57 (53%) patients, pulmonary homograft in 40 (37%), and bovine jugular conduit in 10 (9%). Median conduit size was 11 mm (IQR 10-12) and median conduit Z-score was 1.71 (IQR 1.08-2.34). At 5 years, there was 87% survival, 21% freedom from conduit reinterventions, 37% freedom from conduit replacements, and 55% freedom from PA reinterventions. Conduit size (HR 0.7, 95%CI 0.4-1.4, p=.41) and type (aortic homograft reference; bovine jugular vein graft HR 0.6, 95% CI 0.08-5.2, p=.69; pulmonary homograft HR 0.7, 95% CI 0.2-2.3, p=.58) were not associated with mortality. On multivariate analysis, the hazard for conduit reintervention, conduit replacement, and PA reintervention decreased with increasing conduit Z-score values of 1 to 2.5 (non-linear relationship, p<.01), with little additional reduction in hazard beyond this range. Implantation of a larger conduit within Z-score values of 1 and 2.5 is associated with a decreased hazard for conduit reintervention, conduit replacement, and PA reintervention after truncus repair. The type and size of the conduits did not impact mortality.
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http://dx.doi.org/10.1053/j.semtcvs.2021.05.013DOI Listing
June 2021

Impact of Major Residual Lesions on Outcomes After Surgery for Congenital Heart Disease.

J Am Coll Cardiol 2021 May;77(19):2382-2394

Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts, USA; Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, USA.

Background: Many factors affect outcomes after congenital cardiac surgery.

Objectives: The RLS (Residual Lesion Score) study explored the impact of severity of residual lesions on post-operative outcomes across operations of varying complexity.

Methods: In a prospective, multicenter, observational study, 17 sites enrolled 1,149 infants undergoing 5 common operations: tetralogy of Fallot repair (n = 250), complete atrioventricular septal defect repair (n = 249), arterial switch operation (n = 251), coarctation or interrupted arch with ventricular septal defect (VSD) repair (n = 150), and Norwood operation (n = 249). The RLS was assigned based on post-operative echocardiography and clinical events: RLS 1 (trivial or no residual lesions), RLS 2 (minor residual lesions), or RLS 3 (reintervention for or major residual lesions before discharge). The primary outcome was days alive and out of hospital within 30 post-operative days (60 for Norwood). Secondary outcomes assessed post-operative course, including major medical events and days in hospital.

Results: RLS 3 (vs. RLS 1) was an independent risk factor for fewer days alive and out of hospital (p ≤ 0.008) and longer post-operative hospital stay (p ≤ 0.02) for all 5 operations, and for all secondary outcomes after coarctation or interrupted arch with VSD repair and Norwood (p ≤ 0.03). Outcomes for RLS 1 versus 2 did not differ consistently. RLS alone explained 5% (tetralogy of Fallot repair) to 20% (Norwood) of variation in the primary outcome.

Conclusions: Adjusting for pre-operative factors, residual lesions after congenital cardiac surgery impacted in-hospital outcomes across operative complexity with greatest impact following complex operations. Minor residual lesions had minimal impact. These findings may provide guidance for surgeons when considering short-term risks and benefits of returning to bypass to repair residual lesions.
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http://dx.doi.org/10.1016/j.jacc.2021.03.304DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8245007PMC
May 2021

Commentary: The fate of pediatric cardiac palliations: Beyond the convergence.

J Thorac Cardiovasc Surg 2021 Apr 16. Epub 2021 Apr 16.

Congenital Heart Surgery, Michael E. DeBakey Department of Surgery, Texas Children's Hospital, Baylor College of Medicine, Houston, Tex. Electronic address:

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http://dx.doi.org/10.1016/j.jtcvs.2021.04.016DOI Listing
April 2021

Central venoarterial extracorporeal life support in pediatric refractory septic shock: a single center experience.

Perfusion 2021 Mar 15:2676591211001782. Epub 2021 Mar 15.

Division of Pediatric Critical Care Medicine, Department of Pediatrics, Texas Children's Hospital and Baylor College of Medicine, Houston, TX, USA.

Objective: Venoarterial extracorporeal membrane oxygenation (VA ECMO) is recognized as a potential support therapy for pediatric patients with refractory septic shock (RSS). This review aims to report our experience with central VA cannulation in pediatric patients with RSS, and to compare this with peripheral VA ECMO cannulations for this condition at our institution.

Design: Retrospective case series.

Setting: Pediatric and cardiac intensive care units in an academic pediatric hospital.

Patients: All patients 0-18 years old meeting criteria of RSS placed on VA ECMO between January 2011 and December 2018.

Interventions: None.

Measurements: Demographics, relevant clinical variables, ECMO run details, and outcomes were collected.

Results: Between 2011 and 2018, 14 children were placed on VA ECMO for RSS. Nine were cannulated centrally, with the rest placed on peripheral VA ECMO. Overall survival to hospital discharge was 57.1% (8/14), with 66.7% of the central cannulation cohort surviving versus 40% in the peripheral cannulation (p = 0.34). Median ECMO duration was 147.1 hours (IQR: 91.9-178.6 hours), with survivors having a median length of 147.1 (IQR: 138.5-185.7) versus non survivors 114.7 hours (IQR: 63.7-163.5), p = 0.48. Overall median ICU length of stay (LOS) was 19 days (IQR: 10.5-42.2). The median % maximum flow achieved on VA ECMO was higher in the central cannulation group at 179.6% (IQR: 154.4-188.1) versus the peripheral with 133.5% (98.1-149.1), p = 0.01. Functional status scale (FSS) was used to capture morbidity. All survivors had a mean increase in their FSS from baseline. In the centrally cannulated group, 50% (4/8) received mediastinal exploration, but none developed mediastinitis. In terms of blood product utilization, the central cannulation received more platelets compared to the peripherally cannulated group (median 15.6 vs 3.3 mL/kg/day, p = 0.03).

Conclusion: A central approach to VA ECMO cannulation is feasible and has potential for good patient outcomes in selected patients.
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http://dx.doi.org/10.1177/02676591211001782DOI Listing
March 2021

Smaller right pulmonary artery is associated with longer survival time without scimitar vein repair.

J Card Surg 2021 Apr 18;36(4):1352-1360. Epub 2021 Feb 18.

Heart Center, Baylor College of Medicine, Texas Children's Hospital, Houston, Texas, USA.

Introduction: The optimal management of scimitar syndrome remains incompletely defined. We (1) evaluated the impact of aortopulmonary collateral (APC) occlusion, (2) compared outcomes according to surgical approach for patients who underwent surgery, and (3) identified anatomic factors associated with longer survival time without scimitar vein repair.

Methods: We conducted a single center, retrospective study of 61 patients diagnosed with scimitar syndrome between 1995 and 2019. Right pulmonary artery to total pulmonary artery cross-sectional area (RPA:PA CSA) quantitatively assessed right pulmonary artery size. Anatomical features were analyzed for association with longer survival time without scimitar vein repair.

Results: Median follow-up time was 6 years (Q1-Q3, 2-12), with 96% 5-year survival. Twenty-three patients underwent APC occlusion, which significantly decreased symptoms of overcirculation (100%-46%; p = .001) and systolic pulmonary artery pressure (median, 34-29 mmHg; p = .004). Twenty-three patients underwent scimitar vein repair; 5-year freedom from scimitar vein stenosis was 90% among patients who underwent a reimplantation compared with 42% in patients with baffle repair (p = .1). Three patients underwent surgery before the first year of age, with lower 5-year freedom from scimitar vein stenosis (0% vs. 84%; p < .001). On multivariate analysis, a lower RPA:PA CSA was associated with longer survival time without scimitar vein repair (p = .003).

Conclusions: APC occlusion improves the clinical status of young and hemodynamically unstable patients. Repair at an early age is associated with an increased risk of scimitar vein stenosis. Scimitar vein repair might be avoided in patients with a smaller right pulmonary artery.
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http://dx.doi.org/10.1111/jocs.15405DOI Listing
April 2021

"Compassionate" Cases of the Jarvik 2015 Ventricular Assist Device.

ASAIO J 2021 09;67(9):1036-1043

Congenital Heart Surgery, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas.

The Jarvik 2015 Ventricular Assist Device (VAD) (Jarvik Inc, New York, NY) is the first and currently only continuous-flow VAD specifically designed for small children, and it is being evaluated in the so-called Pump for Kids, Infants, and Neonates (PumpKIN) trial. Due to the strict inclusion criteria of the trial, there have been a group of patients who failed to meet the criteria and therefore received the Jarvik 2015 VAD under the designation of "compassionate use." This is the same phenomenon seen previously during the Berlin Heart EXCOR trial. While we await the results of the PumpKIN trial, which will report the device performance in a strictly selected population, the compassionate use cases represent actual "real world" experiences. We describe herein our experience of two compassionate use cases. In particular, this report has a special emphasis on the power consumption and hemolysis and inflammatory lab profile of the Jarvik 2015 VAD as hemocompatibility was the primary focus of the developmental and the preclinical phases.
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http://dx.doi.org/10.1097/MAT.0000000000001351DOI Listing
September 2021

Coronary Artery Anomalies Are Associated With Increased Mortality After Truncus Arteriosus Repair.

Ann Thorac Surg 2020 Nov 18. Epub 2020 Nov 18.

Division of Congenital Heart Surgery, Texas Children's Hospital, Department of Surgery, Baylor College of Medicine, Houston, Texas. Electronic address:

Background: Truncus arteriosus is associated with coronary anomalies. We identified coronary artery lesions in patients undergoing repair of truncus arteriosus, defined the impact of lesions on mortality, and studied the effect of surgical intervention of coronary lesions.

Methods: A retrospective review identified 107 patients with truncus repair (1995-2019). Coronary lesions were categorized as ostial stenosis, intramural, juxtacommissural origin, and single coronary. Survival analysis characterized survival after truncus repair and studied the association of coronary lesions and mortality.

Results: Among 107 patients with truncus repair 34 patients had at least 1 coronary lesion. Median follow-up time was 7 years, with 85% 5-year survival. Coronary lesions including ostial stenosis, intramurality, and juxtacommissural origin were associated with increased mortality, whereas single coronaries did not impact survival. Eleven patients had 1 coronary lesion and 6 patients with 2 coronary lesions had similar (80% and 83%, respectively) 5-year survival. Eight patients with 3 coronary lesions had 24% 5-year survival (P = .0003). Among patients with 1 or 2 lesions, surgical intervention on the coronary lesions tended to be associated with longer 5-year survival (100% vs 62%, respectively; P = .06). All patients with 3 lesions underwent coronary artery intervention, with 24% 5-year survival.

Conclusions: Impact of coronary lesions on mortality after truncus repair increases with the number of lesions. Coronary artery intervention may be associated with improved time-related survival among patients with 1 or 2 lesions. Patients with the most complex anomalies (3 lesions) have poor survival and warrant ongoing study of repair techniques.
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http://dx.doi.org/10.1016/j.athoracsur.2020.08.082DOI Listing
November 2020

The intraoperative use of recombinant activated factor VII in arterial switch operations.

Cardiol Young 2021 Mar 19;31(3):386-390. Epub 2020 Nov 19.

Texas Children's Hospital, Houston, TX, USA.

Background: The rate of bleeding complications following arterial switch operation is too low to independently justify a prospective randomised study for benefit from recombinant factor VIIa. We aimed to evaluate factor VIIa in a pilot study.

Methods: We performed a retrospective cohort study of patients undergoing arterial switch operation from 2012 to 2017. Nearest-neighbour propensity score matching on age, gender, weight, and associated cardiac defects was used to match 27 controls not receiving recombinant factor VIIa to 30 patients receiving recombinant factor VIIa. Fisher's exact test was performed to compare categorical variables. Wilcoxon's rank-sum test was used to compare continuous variables between cohorts.

Results: Post-operative thrombotic complications were not associated with factor VIIa administration (Odds Ratio (OR) 0.28, 95% CI 0.005-3.77, p = 0.336), nor was factor VIIa administration associated with any re-explorations for bleeding. No intraoperative transfusion volumes were different between the recombinant factor VIIa cohort and controls. Post-operative prothrombin time (10.8 [10.3-12.3] versus 15.9 [15.1-17.2], p < 0.001) and international normalised ratio (0.8 [0.73-0.90] versus 1.3 [1.2-1.4], p < 0.001]) were lower in recombinant factor VIIa cohort relative to controls.

Conclusions: In spite of a higher post-bypass packed red blood cell transfusion requirement, patients receiving recombinant factor VIIa had a similar incidence of bleeding post-operatively. With no difference in thrombotic complications, and with improved post-operative laboratory haemostasis, a prospective randomised study is warranted to evaluate recombinant factor VIIa.
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http://dx.doi.org/10.1017/S1047951120004072DOI Listing
March 2021

Hospital outcomes for pediatric heart transplant recipients undergoing tracheostomy: A multi-institutional analysis.

Pediatr Transplant 2021 May 11;25(3):e13904. Epub 2020 Nov 11.

Division of Pediatric Cardiology, Department of Pediatrics/Primary Children's Hospital Heart Center, University of Utah, Salt Lake City, UT, USA.

Tracheostomy is associated with increased mortality and resource utilization in children with CHD. However, the prevalence and hospital outcomes of tracheostomy in children with HTx are not known. We describe the prevalence and compare the post-HTx hospital outcomes of pediatric patients with Pre-TT and Post-TT to those without tracheostomy. A multi-institutional retrospective cohort study was performed using the Pediatric Health Information System database. Hospital mortality, mediastinitis, LOS, and costs were compared among patients with Pre-TT, Post-TT, and no tracheostomy. Pre-TT was identified in 29 (1.1%) and Post-TT was identified in 41 (1.6%) of 2603 index HTx hospitalizations. Patients with Pre-TT were younger and more likely to have CHD, a non-cardiac birth defect, or an airway anomaly compared to those without Pre-TT. Pre-TT was not independently associated with increased post-HTx in-hospital mortality. Age at HTx < 1 year, CHD, and Post-TT were associated with increased in-hospital mortality. Pre-TT that occurred during the HTx hospitalization and Post-TT were associated with increased resource utilization. Tracheostomy was not associated with mediastinitis.
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http://dx.doi.org/10.1111/petr.13904DOI Listing
May 2021

Commentary: Ventricular assist device support in an early infancy with single ventricle: Transformation from salvage to elective.

J Thorac Cardiovasc Surg 2021 08 17;162(2):416-417. Epub 2020 Oct 17.

Congenital Heart Surgery, Michael E. DeBakey Department of Surgery, Texas Children's Hospital, Baylor College of Medicine, Houston, Tex. Electronic address:

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http://dx.doi.org/10.1016/j.jtcvs.2020.10.045DOI Listing
August 2021

Midterm outcomes of pulmonary artery sling repair with and without tracheoplasty.

Cardiol Young 2021 Jan 13;31(1):52-59. Epub 2020 Oct 13.

Division of Congenital Heart Surgery, Texas Children's Hospital, Department of Surgery, Baylor College of Medicine, Houston, TX, USA.

Objective: Review a single-centre experience with pulmonary artery sling repair and evaluate risk factors for re-intervention.

Methods: Patients with surgically repaired pulmonary artery sling at a single institution between 1996 and 2018 were retrospectively reviewed. A univariate Cox regression analysis was used to evaluate variables for association with freedom from re-intervention.

Results: Eighteen patients had pulmonary artery sling repair. At operation, median age and weight were 6.9 months (interquartile range 4.1-18.1) and 9.5 kg (interquartile range 6.5-14.5), respectively. A median hospital length of stay was 12 days (interquartile range 5.8-55.3). Twelve patients (67%) had complete tracheal rings, of whom six (50%) underwent tracheoplasty (five concurrently with pulmonary artery sling repair). Airway re-intervention was required in five (83%) of the six patients who underwent tracheoplasty. One patient had intraoperative diagnosis and repair of pulmonary artery sling during unrelated lesion repair and required tracheoplasty 24 days post-operatively. One patient died 55 days after pulmonary artery sling repair and tracheoplasty following multiple arrests and re-interventions. Median post-operative follow-up for surviving patients was 6.3 years (interquartile range 11 months-13 years), at which time freedom from re-intervention was 61%. When controlling for patient and tracheal size, initial tracheoplasty was associated with decreased freedom from re-intervention (hazard ratio 21.9, 95% confidence interval 1.7-284.3, p = 0.018).

Conclusions: In patients with pulmonary artery sling, tracheoplasty is associated with decreased freedom from re-intervention. In select patients with pulmonary artery sling and complete tracheal rings, conservative management without tracheoplasty is feasible. Further study is necessary to delineate objective indications for tracheoplasty.
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http://dx.doi.org/10.1017/S1047951120003212DOI Listing
January 2021

Fourth Annual Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs) Report.

Ann Thorac Surg 2020 12 8;110(6):1819-1831. Epub 2020 Oct 8.

Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.

Background: The Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs), originally a National Institutes of Health-sponsored U.S. database, provides a platform to understand the population of children supported with ventricular assist devices (VADs) during this time of increasing numbers, new devices, expanding indications, and improved outcomes.

Methods: Between September 19, 2012, and December 31, 2019, 44 hospitals implanted 1031 devices in 856 patients under 19 years of age.

Results: Overall, diagnosis was cardiomyopathy in 497 (58%) patients, congenital heart disease (CHD) in 216 (25%), myocarditis in 85 (10%), and other in 58 (7%). Positive outcome (alive on device or bridge to transplantation and recovery) occurred in 82% at 6 months. The patient cohort for implantable continuous (IC) pumps (n = 365) (age 13.2 ± 3.9 years, 18% Interagency Registry for Mechanically Assisted Circulatory Support [Intermacs] profile 1, 23% intubated at implantation, 16% with CHD) was significantly different from the paracorporeal continuous (PC) pump cohort (n = 212) (age 3.6 ± 4.9 years, 46% Intermacs profile 1, 81% intubated, 42% CHD) and the paracorporeal pulsatile (PP) pump cohort (n = 230) (age 2.7 ± 3.5 years, 31% Intermacs profile 1, 76% intubated, 26% CHD). Consistent with their cohort composition, positive outcomes at 6 months based on device type were the following: IC, 92%; PC, 68%; and PP, 81%. The incidence of cerebrovascular accidents in the IC, PC, and PP cohorts was 7%, 14%, and 15%, respectively.

Conclusions: IC VADs, the most common VAD type placed in children, are associated with improved outcomes compared with PP and PC devices, though PP and PC devices are limited to supporting our most challenging patients. Noteworthy, the incidence of cerebrovascular accidents for pediatric VADs has significantly decreased and is now 11% overall. This report demonstrates again that although often attributed to age, size, or device type, much of the burden in mortality and adverse events is correlated to the patient's overall state at VAD implantation.
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http://dx.doi.org/10.1016/j.athoracsur.2020.09.003DOI Listing
December 2020

Surgical Techniques in Management of Supravalvular Aortic Stenosis in Children.

Ann Thorac Surg 2021 06 16;111(6):2021-2027. Epub 2020 Sep 16.

Division of Congenital Heart Surgery, Texas Children's Hospital, Houston, Texas; Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas. Electronic address:

Background: Multiple techniques exist for the repair of supravalvular aortic stenosis (SVAS), but given the lesion's rarity, analyses comparing the efficacy of each repair have been limited.

Methods: A retrospective review of all children at a single institution who underwent repair of SVAS from June 1995 to May 2019 was performed. Anatomic and physiologic measurements across time points were compared between 2 predominant surgical techniques. Time-to-event outcomes were compared using the log-rank test.

Results: SVAS was repaired in 89 patients, by using a single-patch in 31 (35%) and the Doty repair in 58 (65%). Median age at operation was 2.5 years (interquartile range [IQR], 1.0 to 6.8 years), with median follow-up of 5.8 years (IQR, 1.8 to 10.7 years). Reoperation was required in 8 (9%) patients at a median of 1.5 years postoperatively (IQR, 0.3 to 4.8 years). There was 1 death after multiple reinterventions. The change from the preoperative to the postoperative sinotubular junction z-score was greater for patients after Doty repair (median change +2.5; IQR, 1.5, 4.1) than for patients after single-patch repair (median change +0.8; IQR, -0.1, 2.1; P = .001). Freedom from reoperation was longer for patients after Doty repair than after the single-patch technique (P = .008).

Conclusions: The Doty repair provides longer freedom from reoperation after supravalvular aortic stenosis repair compared with a single-patch technique, likely through a greater increase in the sinotubular junction at the time of initial operation.
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http://dx.doi.org/10.1016/j.athoracsur.2020.06.118DOI Listing
June 2021

Mechanical Mitral Valve Replacements in the Pediatric Population.

Ann Thorac Surg 2021 08 31;112(2):626-631. Epub 2020 Aug 31.

Division of Congenital Heart Surgery, Texas Children's Hospital, Houston, Texas; Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas. Electronic address:

Background: We evaluated the range of prosthetic size-to-weight ratio to optimize valve survival in small children.

Methods: A single-institution retrospective review of mechanical mitral valve replacements from 1995 to 2019 was performed. Prosthetic valve size-to-weight ratio was calculated as the prosthetic valve diameter divided by the patient's operative weight in children less than or equal to 35 kg. Patient death or reoperation on the valve was analyzed by size-to-weight ratio. Identifying a U-shaped distribution of events, patients were stratified as being in the nadir of the distribution or on the edges.

Results: Mechanical mitral valve replacements were performed in 56 (75%) children weighing less than or equal to 35 kg. Median follow-up time was 3.7 (interquartile range, 0.46-12) years. Median size-to-weight ratio was 1.5 (interquartile range, 1.0-2.0). A second replacement was required in 15 (27%) patients. Death occurred in 6 (11%) patients, including 3 after reoperation. The nadir of U-shaped distribution of events by size-to-weight ratio was bounded by a ratio from 1 to 2, which included 29 (52%) patients. A size-to-weight ratio from 1 to 2 provided optimal outcomes regardless of patient age. Reoperation-free survival at 5 years was 96% for patients with a ratio from 1 to 2 and 46% for patients with a ratio less than 1 or greater than 2. Patients with size-to-weight ratio 1 to 2 had longer reoperation-free survival than patients with a ratio less than 1 or greater than 2 (P < .001).

Conclusions: Regardless of patient age, in patients less than or equal to 35 kg, optimal reoperation-free survival after prosthetic mitral valve replacement can be obtained by placing a prosthetic valve whose diameter is between 1 and 2 times the patient's weight in kilograms.
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http://dx.doi.org/10.1016/j.athoracsur.2020.06.068DOI Listing
August 2021

Sickle Cell-Related Complications in Patients Undergoing Cardiopulmonary Bypass.

World J Pediatr Congenit Heart Surg 2020 09;11(5):565-571

Texas Center for Pediatric and Congenital Heart Disease, 441903University of Texas Dell Medical School/Dell Children's Medical Center, Austin, TX, USA.

Background: We aimed to describe our experience with patients with sickle cell trait (SCT) and undergoing surgery on cardiopulmonary bypass (CPB).

Methods: Data on all patients with SCT or sickle-α thalassemia who underwent surgery on CPB were collected (1996-2017).

Results: Overall, 46 patients were included, 37 (80%) had SCT and 9 (20%) had sickle-α thalassemia. A total of 4 (9%) developed a potential sickle cell-related complication. Patients with sickle cell-related complications were significantly older (median 14 years vs 14 months, = .037) and heavier (median 54 kg vs 9 kg, = .041). Complications occurred, although without statistical significance, in patients who underwent longer median CPB times (249 minutes vs 137 minutes, = .069), lower median temperature (31.7 °C vs 33.3 °C, = .094), and a higher percentage underwent deep hypothermic circulatory arrest (50% vs 7%, = .053). A total of 30 (65%) patients underwent exchange transfusion (ET) pre-bypass. Patients who underwent ET were significantly older (median 4 years vs 7 months, = .003) and heavier (median 16 kg vs 6 kg, = .015) than patients who did not undergo ET. The incidence of complications was comparable between patients who underwent ET (10%) and those who did not (6%).

Conclusions: In this retrospective, single-center study, it has been shown that cardiac surgery requiring CPB in patients with SCT and sickle-α thalassemia had a low risk for sickle cell-associated complications. In this cohort of patients, older age, longer CPB times, lower median temperature, and the utilization of deep hypothermic circulatory arrest appear to play an important role in the development of complications.
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http://dx.doi.org/10.1177/2150135120926991DOI Listing
September 2020

Predictors of Transplant-Free Survival After the Norwood Procedure.

Ann Thorac Surg 2021 08 17;112(2):638-644. Epub 2020 Aug 17.

Division of Congenital Heart Surgery, Department of Surgery, Texas Children's Hospital/Baylor College of Medicine, Houston, Texas. Electronic address:

Background: Birth weight, preterm delivery, and size for gestational age are surrogate markers for development that are commonly used in congenital heart surgery. Understanding the associations of these variables with patient outcomes is of great importance.

Methods: This study included all patients with hypoplastic left heart syndrome who underwent a Norwood procedure at a single institution from 1995 to 2018. Low birth weight was defined as weight less than 2.5 kg, and preterm delivery occurred at less than 37 weeks' gestation. Overall and conditional analyses were performed to evaluate for association with outcomes after the Norwood procedure. Secondary analyses evaluated the association of development measures with postoperative length of stay and ventilator duration.

Results: In total, 303 neonates (60% male) underwent the Norwood procedure and were followed for a median of 3.9 years (interquartile range, 0.5 to 10.4 years). Median birth weight was 3.1 kg (interquartile range, 2.8 to 3.4 kg). Patients with low birth weight had decreased transplant-free survival compared with patients with a normal birth weight (hazard ratio, 1.7; 95% confidence interval, 1.03 to 2.82; P = .039). When conditioning on survival to second-stage palliation, patients born small for gestational age had decreased transplant-free survival compared with patients born at appropriate size for gestational age (hazard ratio, 2.8; 95% confidence interval, 1.31 to 6.09; P = .008). Patients delivered preterm had a longer hospital length of stay (median, 55 days vs 31 days; P = .02) and more ventilator days compared with patients delivered at term (median, 7 days vs 4 days; P = .004).

Conclusions: Various developmental markers have differing prognostic importance for patients undergoing the Norwood procedure. Understanding these differences can help guide preoperative decision making and patient selection.
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http://dx.doi.org/10.1016/j.athoracsur.2020.06.024DOI Listing
August 2021

Younger Age at Operation Is Associated With Reinterventions After the Warden Procedure.

Ann Thorac Surg 2021 06 24;111(6):2059-2065. Epub 2020 Jul 24.

Division of Congenital Heart Surgery, Department of Surgery, Texas Children's Hospital/Baylor College of Medicine, Houston, Texas.

Background: Multiple techniques are available for repair of supracardiac partial anomalous pulmonary venous return (PAPVR); however, most series fail to compare the techniques in contemporary cohorts. This study aimed to describe outcomes of the Warden procedure with a single-patch repair cohort to serve as a control.

Methods: A retrospective cohort analysis of all patients at a single institution (Texas Children's Hospital, Houston, TX) included patients undergoing either the Warden procedure or single-patch repair from 1996 to 2019 for PAPVR. Reintervention was defined as any catheter or surgical procedure on the superior vena cava (SVC) or pulmonary veins. Subgroup analysis was performed within the Warden cohort to evaluate for association between an SVC patch and reintervention-free survival.

Results: In total, 158 patients (122 in the Warden group and 36 in the single-patch group) were identified. The median age at operation was younger for patients in the Warden cohort (5.4 years; interquartile range, 3.3 to 10.2 years) compared with patients in the single-patch cohort (13.3 years; interquartile range, 6.5 to 18.7 years; P < .001). One patient in each cohort died. One patient required reoperation after the Warden procedure for dehiscence of the intracardiac patch. Ten patients required transcatheter reinterventions. Reintervention-free survival was not different between patients in the Warden cohort and patients in the single-patch cohort (P = .54) or within the Warden cohort in patients with an SVC patch (P = .27). When controlling for repair type, older age at repair was associated with longer reintervention-free survival (hazard ratio, 0.81; 95% confidence interval, 0.71 to 0.93; P = .002).

Conclusions: The Warden procedure is a viable option for younger patients requiring supracardiac PAPVR repair, although these younger patients are likely at greatest risk for reintervention regardless of surgical technique.
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http://dx.doi.org/10.1016/j.athoracsur.2020.05.143DOI Listing
June 2021

Recurrent Pulmonary Artery Interventions Following the Norwood Procedure Are Not Associated With Conduit Type.

Semin Thorac Cardiovasc Surg 2021 Spring;33(1):195-201. Epub 2020 Jun 5.

Department of Surgery, Division of Congenital Heart Surgery, Baylor College of Medicine and Texas Children's Hospital, Houston, Texas. Electronic address:

Given pulmonary artery interventions following the Norwood procedure can recur, the average number of occurrences per patient over time is likely more informative than the crude percentage of patients who required an intervention. Pulmonary artery intervention was defined as any surgical or catheter-based procedure after the Norwood procedure. The number of pulmonary artery interventions for patients with hypoplastic left heart syndrome were compared between patients with modified Blalock-Taussig Shunts (MBTS) and right ventricle-to-pulmonary artery conduits (RVPA) at a single institution from 2011 to 2018. The comparison was replicated using data from the Single Ventricle Reconstruction Trial (SVR), a nonoverlapping dataset. The mean number of pulmonary artery interventions per patient over time (mean cumulative function, MCF) is described using Nelson-Aalen estimates and compared using the pseudo-score test. The number of patients requiring intervention was compared using the chi-square test. Using our institutional dataset, the Norwood operation was performed on 117 patients (59 MBTS, 58 RVPA). In total, 73 patients had a pulmonary artery intervention, including 32 of 58 (55%) after MBTS and 41 of 59 (69%) after RVPA (P= 0.11). The MCF did not vary between cohorts (P = 0.55). Using the SVR trial dataset, 140 of 549 patients required pulmonary artery intervention, including 55 (21%) after MBTS and 85 (30%) after RVPA (P = 0.0090). The MCF did not vary between cohorts (P = 0.067). Although more patients with RVPA than MBTS require pulmonary artery interventions after the Norwood procedure, the MCFs are not different, which may be of greater importance to patients and families.
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http://dx.doi.org/10.1053/j.semtcvs.2020.05.028DOI Listing
May 2021

Commentary: Pediatric myocardial recovery with a ventricular assist device: "Chance favors the prepared mind".

JTCVS Tech 2021 Feb 11;5:93-94. Epub 2020 May 11.

Congenital Heart Surgery, Michael E. DeBakey Department of Surgery, Texas Children's Hospital, Baylor College of Medicine, Houston, Tex.

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http://dx.doi.org/10.1016/j.xjtc.2020.04.024DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8299964PMC
February 2021

Pulmonary Artery Banding for Children With Dilated Cardiomyopathy: US Experience.

Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2020 ;23:69-76

Baylor College of Medicine and Texas Children's Hospital, Houston, Texas. Electronic address:

Pulmonary artery band placement is a recently described therapeutic strategy for dilated cardiomyopathy with preserved right ventricular function, originally reported from Germany.1 We present the results of the multicenter retrospective study of pulmonary artery band experience in the United States, with comparison to the German experience. Five centers contributed a total 14 patients (median age 5 months, interquartile range 3.5-10). Mechanical ventilation was required in 9/12 (75%) patients and inotropes were used in 13/14 (93%) patients preoperatively. Ultimately, 4 (29%) patients experienced cardiac recovery, 8 (57%) were bridged to cardiac transplantation (6 with ventricular assist device placement), and 2 (14%) died. Although both the US and Germany series demonstrated high prevalence of achieving patients' individual target (either cardiac recovery or transplant), the mode of success was different (recovery rate: <1/3 in the United States and >2/3 in Germany). Lower recovery rate may be a reflection of sicker preoperative status, and thereby a more advanced stage of heart failure (preoperative intubation: >2/3 in the United States vs <1/3 in Germany). Further studies would be warranted to gain more insight into patient selection as well as optimal timing for the intervention.
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http://dx.doi.org/10.1053/j.pcsu.2020.03.002DOI Listing
March 2021

Current status of pediatric mechanical circulatory support.

Curr Opin Organ Transplant 2020 06;25(3):231-236

Division of Congenital Heart Surgery, Michael E. DeBakey Department of Surgery, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas, USA.

Purpose Of Review: The field of pediatric mechanical circulatory support has experienced exponential evolution in recent decades. With favorable complication profiles, implantable continuous-flow ventricular assist devices (VADs) have become a standard option in children, as has been seen in the adult counterpart. Nevertheless, there still exists room for further advances, not just for survival, but throughout the whole trajectory of treatment courses. With reviewing the current state of pediatric VAD support, including existing challenges, we aim to highlight the targets clinicians should focus on for further improvement of pediatric VAD support.

Recent Findings: The field of pediatric VAD has been steadily growing, as evidenced by an increasing number of total VAD implants, particularly with continuous-flow VAD. Currently, HeartWare HVAD (Medtronic Inc., Mounds View, MN) is the most widely used continuous-flow VAD in children with excellent performance. However, only half of the children with HVAD are discharged home, which is drastically different from adult patients, suggesting that the pediatric field is still in the process of maturation. Additionally, outcomes of VAD support for complex congenital heart defect, particularly single ventricle physiology, remain suboptimal, despite an increasing number of such patients.

Summary: With the ongoing advancement, the field of pediatric VAD support is undergoing a rapid maturation process. This will eventually lead to further paradigm changes, including the use of VAD as permanent therapy.
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http://dx.doi.org/10.1097/MOT.0000000000000761DOI Listing
June 2020

Left ventricular unloading during extracorporeal membrane oxygenation - Impella versus atrial septal defect: A simulation study.

Int J Artif Organs 2020 Oct 22;43(10):663-670. Epub 2020 Feb 22.

Department of Pediatric Cardiology and Cardiac Surgery, Pediatric Hospital Bambino Gesù, Rome, Italy.

Background: Atrial septal defect and Impella have been proposed for left ventricular unloading in venoarterial extracorporeal membrane oxygenation patients. This work aims at evaluating the haemodynamic changes in venoarterial extracorporeal membrane oxygenation patients after Impella implantation or atrial septal defect realization by a simulation study.

Methods: A lumped parameter model of the cardiovascular system was adapted to this study. Atrial septal defect was modelled as a resistance between the two atria. Venoarterial extracorporeal membrane oxygenation and Impella were modelled starting from their pressure-flow characteristics. The baseline condition of a patient undergoing venoarterial extracorporeal membrane oxygenation was reproduced starting from haemodynamic and echocardiographic data. The effects of different atrial septal defect size, Impella and venoarterial extracorporeal membrane oxygenation support were simulated.

Results: Impella caused an increment of mean arterial pressure up to 67%, a decrement in mean pulmonary arterial pressure up to 8%, a decrement in left ventricular end systolic volume up to 11% with a reduction up to 97% of left ventricular cardiac output. Atrial septal defect reduces left atrial pressure (19%), increases right atrial pressure (22%), increases mean arterial pressure (18%), decreases left ventricular end systolic volume (11%), increases right ventricular volume (33%) and decreases left ventricular cardiac output (55%).

Conclusion: Impella has a higher capability in left ventricular unloading during venoarterial extracorporeal membrane oxygenation in comparison to atrial septal defect with a lower right ventricular overload.
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http://dx.doi.org/10.1177/0391398820906840DOI Listing
October 2020
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