Publications by authors named "Michiaki Imamura"

115 Publications

Perioperative evaluation of neonatal aortic arch thrombosis.

J Card Surg 2021 Oct 26;36(10):3872-3873. Epub 2021 Jul 26.

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

Herein, we present a neonatal case of coarctation of the aorta, with aortic arch thrombus confirmed by echocardiography. We performed thrombus removal and aortic arch repair emergently. This critical condition necessitates quick preoperative evaluation with echocardiography. Moreover, postoperative evaluation using computed tomography is reasonable to assess an aortic arch configuration, and exclude the remnant thrombus.
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http://dx.doi.org/10.1111/jocs.15852DOI Listing
October 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

The American Association for Thoracic Surgery Congenital Cardiac Surgery Working Group 2021 consensus document on a comprehensive perioperative approach to enhanced recovery after pediatric cardiac surgery.

J Thorac Cardiovasc Surg 2021 Sep 1;162(3):931-954. Epub 2021 May 1.

Division of Cardiothoracic Anesthesiology, Department of Anesthesiology and Critical Care Medicine, The Children's Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Penn.

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http://dx.doi.org/10.1016/j.jtcvs.2021.04.072DOI Listing
September 2021

Heart transplantation in a case of scimitar syndrome.

Ann Thorac Surg 2021 Mar 29. Epub 2021 Mar 29.

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

We present a rare clinical scenario of a patient with tetralogy of Fallot, hypertrophic cardiomyopathy, and concomitant scimitar syndrome. We created a scimitar vein cuff from a recipient heart, performed its translocation, and subsequently performed heart transplantation. This ingenuity reduces the likelihood of scimitar vein obstruction.
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http://dx.doi.org/10.1016/j.athoracsur.2021.03.054DOI Listing
March 2021

Intrinsic coronary stenosis with ventricular dysfunction in Tetralogy of Fallot.

Ann Thorac Surg 2021 Mar 4. Epub 2021 Mar 4.

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

Intrinsic coronary anomalies in tetralogy of Fallot are rare manifestations that are difficult to diagnose, especially when accompanied with ventricular dysfunction in the postoperative period. We present a case of severe left ventricular dysfunction after repair of tetralogy of Fallot. Coronary angiography revealed intrinsic left main coronary stenosis. Therefore, we emergently performed patch augmentation of the left main coronary artery concomitantly with residual ventricular septal defect closure. While evaluating patients with tetralogy of Fallot, especially in the presence of ventricular dysfunction, intrinsic coronary anomalies should be kept in mind. Coronary angiography is effective in diagnosing coronary anomalies.
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http://dx.doi.org/10.1016/j.athoracsur.2021.02.047DOI 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

Commentary: Truncal valve intervention in children.

Authors:
Michiaki Imamura

J Thorac Cardiovasc Surg 2020 Nov 5. Epub 2020 Nov 5.

Division of Congenital Heart Surgery, Texas Children's Hospital, Houston, Tex. Electronic address:

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http://dx.doi.org/10.1016/j.jtcvs.2020.10.100DOI Listing
November 2020

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

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

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

Congenital Aortic Insufficiency From an Abnormal Left Aortic Cusp Results in Acute Coronary Syndrome.

World J Pediatr Congenit Heart Surg 2021 Jul 13;12(4):551-554. Epub 2020 Aug 13.

The Lillie Frank Abercrombie Section of Cardiology, 3984Texas Children's Hospital, Department of Pediatrics, Baylor College of Medicine, Houston, TX, USA.

Acute coronary syndrome (ACS) is a rare presentation in children with isolated congenital aortic insufficiency (AI). We report the case of a six-week-old previously well male who presented with an out-of-hospital cardiac arrest and was diagnosed with severe AI from a left aortic cusp anomaly resulting in ACS. The infant successfully underwent an emergent Ross operation.
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http://dx.doi.org/10.1177/2150135120947405DOI Listing
July 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

Case 3: Seizure, Facial Droop, and Altered Mental Status in a 9-year-old Girl.

Pediatr Rev 2020 06;41(6):300-302

Division of Cardiology, Department of Pediatrics, and.

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http://dx.doi.org/10.1542/pir.2018-0102DOI Listing
June 2020

Right Ventricle-Dependent Coronary Circulation: Location of Obstruction Is Associated With Survival.

Ann Thorac Surg 2020 05 30;109(5):1480-1487. Epub 2019 Sep 30.

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

Background: Pulmonary atresia with intact ventricular septum (PAIVS) with right ventricle-dependent coronary circulation (RVDCC) carries suboptimal outcomes primarily due to cardiac ischemia. We hypothesize clinical outcomes are affected by the level of coronary obstruction, a surrogate for vulnerable myocardium.

Methods: We conducted a single-institution retrospective analysis of all neonates with PAIVS with RVDCC from 1995 to 2017. RVDCC was defined as the presence of any coronary-cameral fistula with coronary obstruction proximal to the fistula and angiographic evidence of RV perfusion of the myocardium through the fistulous communication. Location of coronary obstruction was categorized as either proximal or distal segments, using the SYNTAX score criteria. Transplant-free survival was compared between patients with proximal and distal obstruction, then these groups were compared with patients without RVDCC.

Results: Of 103 neonates with PAIVS, 28 (27%) had RVDCC: 18 proximal (64%), 10 distal (36%). Median age at last follow-up for patients with RVDCC was 1.8 years (interquartile range, 0.3-8.1 years). All deaths (10 of 28, 36%) occurred at 6 months old or earlier. Proximal coronary artery obstruction was associated with decreased transplant-free survival relative to distal obstruction (hazard ratio = 3.63; 95% confidence interval, 1.01-13.00; P = .048). Transplant-free survival at 1 year was 33% and 70% in the proximal and distal obstruction groups, respectively. Compared with patients without RVDCC, patients with proximal obstruction had significantly lower transplant-free survival (P < .001), whereas patients with distal obstruction did not (P = .217).

Conclusions: The location of coronary artery obstruction affects clinical outcome and may represent a potential branch point in the management for PAIVS with RVDCC.
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http://dx.doi.org/10.1016/j.athoracsur.2019.08.066DOI Listing
May 2020

Reoperation after isolated subaortic membrane resection.

Cardiol Young 2019 Nov 26;29(11):1391-1396. Epub 2019 Sep 26.

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

Background: The resection of a subaortic membrane remains far from a curative operation. We sought to examine factors associated with reoperation and the degree of aortic valve regurgitation as a potential long-term source for reoperation.

Methods: All patients who underwent resection of an isolated subaortic membrane between 1995 and 2018 were included. Patients who underwent other procedures were excluded. Paired categorical data were compared using McNemar's test. Univariate time-to-event analyses were performed using Kaplan-Meier methods with log-rank tests for categorical variables and univariate Cox models for continuous variables.

Results: A total of 84 patients (median age 6.6, 31% females) underwent resection of isolated subaortic membrane. At a median follow-up of 9.3 years (interquartile range 0.6-22.5), 12 (14%) patients required one reoperation and 1 patient required two reoperations. Median time to first reoperation was 4.6 years. The degree of aortic valve regurgitation improved post-operatively from pre-operatively (p = 0.0007); however, the degree of aortic valve regurgitation worsened over the course of follow-up (p = 0.010) to equivalence with pre-operative aortic valve regurgitation (p = 0.18). Performance of a septal myectomy was associated with longer freedom from reoperation (p = 0.004).

Conclusions: In patients with isolated subaortic membranes, performance of a septal myectomy can minimise risk for reoperation. Patients should be serially monitored for degradation of the aortic valve, even if aortic regurgitation is not present post-operatively.
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http://dx.doi.org/10.1017/S1047951119002336DOI Listing
November 2019

Contemporary Midterm Outcomes in Pediatric Patients Undergoing Vascular Ring Repair.

Ann Thorac Surg 2020 02 14;109(2):566-572. Epub 2019 Aug 14.

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

Background: This single-institution study assessed the midterm outcomes of patients undergoing complete vascular ring (CVR) repair and the need for reintervention.

Methods: The study included all patients who underwent surgical repair of an isolated CVR from 1996 to 2018 at our institution. Patients who underwent concomitant intracardiac repair were excluded. Data analysis included demographics, type of anomaly, other congenital heart disease, clinical symptomatology, operative technique, perioperative outcomes, reoperation rates, and mortality.

Results: CVR repair through open thoracotomy was performed in 148 patients (80 boys [54%]), median age, 1.04 years (interquartile range, 0.4-5.2 years), and median weight, 12.8 kg (interquartile range, 7.5-26.5 kg). The cohort included 72 patients with double aortic arch (DAA), 69 with right aortic arch (RAA) with aberrant left subclavian artery and left ligamentum arteriosum (LLA), 5 with RAA with left descending aorta and LLA, and 2 with RAA with mirror-image branching and LLA. There was 1 outpatient perioperative death (0.7%) 15 days postoperatively. Perioperative complications occurred in 20 patients (14%): 18 (12%) with chylothorax (3 required reintervention), 1 pneumothorax, and 1 vocal cord paresis. Two of 36 patients (5.5%) without primary diverticulum resection required reoperation and subclavian reimplantation at 3 and 4 years, and 1 patient required aortic translocation 9 years later for persistent symptoms.

Conclusions: Freedom from reoperation after CVR repair was 93% at 5 years and 86% at 10 years. A small proportion of patients who do not undergo diverticulum resection and aberrant left subclavian artery reimplantation at the time of CVR repair will require reintervention in the future.
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http://dx.doi.org/10.1016/j.athoracsur.2019.06.076DOI Listing
February 2020

Long-term outcomes after transplantation after support with a pulsatile pediatric ventricular assist device.

J Heart Lung Transplant 2019 04 26;38(4):449-455. Epub 2018 Oct 26.

Division of Congenital Heart Surgery, Baylor College of Medicine, Houston, TX, US.

Background: There has been increasing use of durable ventricular assist devices (VAD) in children as a bridge to transplantation (BTT). The Berlin Heart investigational device exemption (IDE) trial was the first pediatric VAD trial to demonstrate excellent survival outcomes as a BTT.

Objectives: Our aim was to compare the expanded post-transplant outcomes for children enrolled in the Berlin Heart IDE trial to a matched Pediatric Heart Transplant Study (PHTS) cohort not requiring mechanical circulatory support (MCS).

Setting: University Hospitals.

Methods: This was a retrospective review of linked PHTS and Berlin Heart IDE databases for pediatric (≤18 years) recipients transplanted from 2007-2011. Subjects with <5 years of follow up were excluded. VAD supported patients were matched 1:2 to non-VAD supported controls from the PHTS database.

Results: Among 109 Berlin Heart IDE study enrollees, 83 were merged with the PHTS database and matched to 166 non-MCS supported patients. There was no difference in diagnosis, status at listing, and age between groups with the expected difference in inotrope use in the non-MCS supported patients. Compared to their matched cohort, there was no statistical difference in 5-year patient survival between VAD and non-VAD patients (81% vs 88%; p = 0.09) nor was there a difference in freedom from rejection or infection.

Conclusions: This data suggests that children supported with a Berlin Heart VAD had similar survival, infection and rejection rates compared to those not requiring MCS support. Continued surveillance of the Berlin Heart IDE trial population post heart transplantation is warranted.
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http://dx.doi.org/10.1016/j.healun.2018.10.005DOI Listing
April 2019

Incidence of Immediate Extubation After Pediatric Cardiac Surgery and Predictors for Reintubation.

World J Pediatr Congenit Heart Surg 2018 Sep;9(5):529-536

1 Division of Pediatric and Congenital Cardiothoracic Surgery, Arkansas Children's Hospital, University of Arkansas for Medical Sciences, Little Rock, AR, USA.

Objectives: The objectives were to assess the incidence of immediate tracheal extubation in the operating room after pediatric cardiac surgery and to investigate predictors for subsequent reintubation.

Methods: This is a single institutional retrospective study including all patients who had a cardiac operation with cardiopulmonary bypass from 2011 to 2016. Patients who required preoperative ventilator support, postoperative open chest, or mechanical support were excluded. Predictors for reintubation after immediate extubation were analyzed only for patients with stage II palliation for single ventricle physiology.

Results: Nine hundred nine qualifying operations were identified. Immediate extubation was performed in 590 (64.9%) operations. A multivariable logistic regression model showed that the identities of anesthesiologist ( P = .0003), year of the operation performed ( P < .001), cardiopulmonary bypass time ( P < .001), and type of operations ( P < .001) were significantly associated with immediate extubation. Reintubation was significantly less frequent in patients with immediate extubation compared to those without (6.1% vs 15.0%; P < .001). A subgroup analysis for stage II palliation showed that reintubation after immediate extubation was significant for younger age (0.42 vs 0.54 years, P = .044), lower Po/Fio and Po at the last blood gas analysis (66 vs 98 mm Hg, P = .032 and 39 vs 47 mm Hg, P = .008), and higher inotropic score (2 vs 0, P = .034). A multivariable logistic regression model showed that only inotropic score was significantly associated with reintubation ( P = .018).

Conclusions: Immediate extubation in the operating room after pediatric cardiac surgery can be performed in most patients. Inotropic score is a predictor for reintubation in stage II palliation.
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http://dx.doi.org/10.1177/2150135118779010DOI Listing
September 2018

An Empirically Derived Pediatric Cardiac Inotrope Score Associated With Pediatric Heart Surgery.

Semin Thorac Cardiovasc Surg 2018 31;30(1):62-68. Epub 2018 Jan 31.

Department of Mathematical Sciences, University of Arkansas, Fayetteville, Arkansas.

We aimed to empirically derive an inotrope score to predict real-time outcomes using the doses of inotropes after pediatric cardiac surgery. The outcomes evaluated included in-hospital mortality, prolonged hospital length of stay, and composite poor outcome (mortality or prolonged hospital length of stay). The study population included patients <18 years of age undergoing heart operations (with or without cardiopulmonary bypass) of varying complexity. To create this novel pediatric cardiac inotrope score (PCIS), we collected the data on the highest doses of 4 commonly used inotropes (epinephrine, norepinephrine, dopamine, and milrinone) in the first 24 hours after heart operation. We employed a hierarchical framework by representing discrete probability models with continuous latent variables that depended on the dosage of drugs for a particular patient. We used Bayesian conditional probit regression to model the effects of the inotropes on the mean of the latent variables. We then used Markov chain Monte Carlo simulations for simulating posterior samples to create a score function for each of the study outcomes. The training dataset utilized 1030 patients to make the scientific model. An online calculator for the tool can be accessed at https://soipredictiontool.shinyapps.io/InotropeScoreApp. The newly proposed empiric PCIS demonstrated a high degree of discrimination for predicting study outcomes in children undergoing heart operations. The newly proposed empiric PCIS provides a novel measure to predict real-time outcomes using the doses of inotropes among children undergoing heart operations of varying complexity.
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http://dx.doi.org/10.1053/j.semtcvs.2018.01.003DOI Listing
October 2018

Experience Using Kaolin-Impregnated Sponge to Minimize Perioperative Bleeding in Norwood Operation.

World J Pediatr Congenit Heart Surg 2017 07;8(4):475-479

1 Division of Pediatric and Congenital Cardiothoracic Surgery, Department of Pediatrics, Arkansas Children's Hospital, University of Arkansas for Medical Sciences, Little Rock, AR, USA.

Purpose: A kaolin-impregnated hemostatic sponge (QuikClot) is reported to reduce intraoperative blood loss in trauma and noncardiac surgery. The purpose of this study was to assess if this sponge was effective for hemostasis during Norwood operation.

Description: We conducted a retrospective review of patients undergoing Norwood operation in infancy between 2011 and 2016 at our institution.

Evaluation: Of 31 identified Norwood operations, a kaolin-impregnated sponge was used intraoperatively in 15 (48%) patients. The preoperative profiles and cardiopulmonary bypass status were similar between the operations with or without kaolin-impregnated sponge. The comparison on each operative outcome between operations with or without kaolin-impregnated sponge showed that the intraoperative platelets, cryoprecipitate, and factor VII dosage were significantly less in the operations with kaolin-impregnated sponge (55 mL, 10 mL, 0 µg/kg vs 72 mL, 15 mL, 45 µg/kg; P = .03, .021, .019), as well as the incidence of perioperative bleeding complications (second cardiopulmonary bypass for hemostasis or postoperative mediastinal exploration, 0% vs 31%, P = .043). A logistic regression model showed that the nonuse of kaolin-impregnated sponge and longer aortic cross clamp time were associated with perioperative bleeding complication in univariable model ( P = .02 and .005).

Conclusions: Use of kaolin-impregnated hemostatic sponge was associated with reduced blood product use and perioperative bleeding complications in Norwood operation at a single institution.
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http://dx.doi.org/10.1177/2150135117713698DOI Listing
July 2017

A ferromagnetic surgical system reduces phrenic nerve injury in redo congenital cardiac surgery.

Interact Cardiovasc Thorac Surg 2017 05;24(5):802-803

Division of Pediatric and Congenital Cardiothoracic Surgery, Arkansas Children's Hospital, University of Arkansas for Medical Sciences, Little Rock, AR, USA.

A ferromagnetic surgical system (FMwand®) is a new type of dissection device expected to reduce the risk of adjacent tissue damage. We reviewed 426 congenital cardiac operations with cardiopulmonary bypass through redo sternotomy to assess if this device prevented phrenic nerve injury. The ferromagnetic surgical system was used in 203 operations (47.7%) with regular electrocautery and scissors. The preoperative and operative details were similar between the operations with or without the ferromagnetic surgical system. The incidence of phrenic nerve injury was significantly lower with the ferromagnetic surgical system (0% vs 2.7%, P = 0.031). A logistic regression model showed that the use of the ferromagnetic surgical system was significantly associated with reduced odds of phrenic nerve injury (P < 0.001).
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http://dx.doi.org/10.1093/icvts/ivw444DOI Listing
May 2017

Improved Survival While Waiting and Risk Factors for Death in Pediatric Patients Listed for Cardiac Transplantation.

Pediatr Cardiol 2017 Jan 1;38(1):77-85. Epub 2016 Nov 1.

Division of Pediatric Cardiology, Children's Medical Center, University of Texas Southwestern Medical Center, Dallas, TX, USA.

Our aim is to determine (a) the effect of changes in pre-transplant management and era of listing on survival of children listed for HTx and (b) risk factors for death while waiting. This retrospective study included all children listed between 1/1993 and 12/2009 at our center. Survival was determined using survival analysis and competing outcomes modeling. There were 254 listed patients of whom 144 (57%) had congenital heart disease, 208 (82%) were status 1, 52 used ECMO (20%), and 28 used ventricular assist device support (VAD) (11%) beginning in 2005. Overall mortality while waiting was 17% at 6 months, and 69% underwent transplant. Seven of 95 patients (7%) died waiting after 2004 compared to 36 of 159 (23%) before. ECMO and earlier year of listing were significant risk factors (p < 0.001) for wait-list mortality, whereas mortality was significantly lower (p = 0.002) after availability of VADs. Race, gender, blood type, and congenital diagnosis were not significant risk factors for death. Survival in pediatric patients listed for HTx has improved significantly in the current era at our institution. The availability of pediatric VADs has had a significant impact on survival while waiting in children listed for transplantation.
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http://dx.doi.org/10.1007/s00246-016-1486-4DOI Listing
January 2017

Single center experience of aortic bypass graft for aortic arch obstruction in children.

Heart Vessels 2017 Jan 27;32(1):76-82. Epub 2016 Apr 27.

Division of Pediatric and Congenital Cardiothoracic Surgery, Arkansas Children's Hospital, University of Arkansas for Medical Sciences, 1 Children's Way, Slot 677, Little Rock, AR, 72202, USA.

The purpose of this study is to access the outcomes of aortic bypass graft placement in children. This is a retrospective review of all children having aortic bypass graft placement for aortic arch obstruction for the first time between 1982 and 2013 at a single institution. The actuarial survival and the freedom from aortic arch reoperation were calculated and compared between the groups. Seventy consecutive children underwent aortic bypass graft placements. The median age and body weight at the operation were 14 days and 3.6 kg. There were 7 early deaths, 6 late deaths, and 7 heart transplants during the median follow-up of 10.8 years (0.0-31.5 years). The actuarial transplant free survival was 64.7 % at 20 years and the freedom from aortic arch reoperation was 50.5 % at 10 years. Between the children younger than 1 year old and older than 1 year old, there were significant differences in actuarial transplant free survival (56.4 vs. 100 % at 15 years, p = 0.0042) and in the freedom from aortic arch reoperation (18.7 vs. 100 % at 10 years, p < 0.001). The children who received aortic bypass graft larger than 16 mm in size had no aortic arch reoperation at 15 years. The aortic bypass graft placement for aortic arch obstruction can be done with low mortality and morbidity for children who can receive bypass graft larger than 16 mm in size. However, it should be avoided for the neonates and infants except selected situations.
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http://dx.doi.org/10.1007/s00380-016-0842-xDOI Listing
January 2017

The Midterm Outcomes of Bioprosthetic Pulmonary Valve Replacement in Children.

Semin Thorac Cardiovasc Surg 2015 12;27(3):310-8. Epub 2015 Aug 12.

Division of Pediatric and Congenital Cardiothoracic Surgery, Arkansas Children׳s Hospital, University of Arkansas for Medical Sciences, Little Rock, Arkansas.

The purpose of this study was to assess the outcomes of bioprosthetic pulmonary valve replacement (PVR) in children. This is a retrospective review of all bioprosthetic PVR in children (≤ 20-year old) between 1992 and 2013 at a single institution. Most outcomes studied included pulmonary valve reintervention and bioprosthetic valve function. A total of 136 bioprosthetic PVRs were identified for 123 patients. The median age and body weight at the time of operation were 13.2 years and 48.4 kg. There were 1 early death and 3 late deaths during the median follow-up of 7.2 years (0-22.0 years). The actuarial transplant-free survival was 97.6% at 10 years. There were 43 bioprosthesis reinterventions with 29 reoperations and 14 catheter-based interventions. The freedom from bioprosthesis reintervention was 89.6% and 55.0% at 5 and 10 years, respectively. Echocardiographic bioprosthesis dysfunction (≥ moderate bioprosthesis insufficiency, ≥ 50 mmHg peak gradient through bioprosthesis, or bioprosthesis endocarditis with vegetation) was found in 57 bioprostheses. The freedom from bioprosthesis dysfunction was 74.0% and 32.8% at 5 and 10 years, respectively. Results from the Cox proportional hazards models showed that age had significant association with freedom from bioprosthesis reintervention and freedom from bioprosthesis dysfunction (P < 0.001 and P = 0.03), whereas bioprosthesis type had nonsignificant association with freedom from bioprosthesis dysfunction (P = 0.068). Bioprosthetic PVR in children had excellent early outcomes but rapidly deteriorating midterm outcomes. Careful and close follow-up are necessary for children with bioprosthesis in the pulmonary position.
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http://dx.doi.org/10.1053/j.semtcvs.2015.07.010DOI Listing
October 2016

Valved Polytetrafluoroethylene Conduits for Right Ventricular Outflow Tract Reconstruction.

Ann Thorac Surg 2015 Jul 21;100(1):129-37; discussion 137. Epub 2015 May 21.

Division of Pediatric and Congenital Cardiothoracic Surgery, Arkansas Children's Hospital, University of Arkansas for Medical Sciences, Little Rock, Arkansas.

Background: The purposes of this study were to review our early outcomes using valved expanded polytetrafluoroethylene (ePTFE) conduits, with or without bulging sinus structure, for right ventricular outflow tract reconstruction and to examine the mechanical properties of the ePTFE material after bulging sinuses were created.

Methods: A retrospective review was performed of all patients who received the valved ePTFE conduit between 2008 and 2014 at a single institution. The surface morphologies and the mechanical strengths of the ePTFE conduit with bulging sinuses examined by scanning electron microscopy and unidirectional pull test were compared with those of the original ePTFE material.

Results: There were 120 operations with the valved ePTFE conduit (60 with bulging sinuses). The patients median age and weight were 6.9 years and 23.7 kg. The conduits were a median size of 22 mm. At 5 years, freedom from conduit reoperation was 92.7% (95% confidence interval, 82.7% to 97.0%), and freedom from severe conduit insufficiency or more than a 50 mm Hg gradient was 74.8% (95% confidence interval, 60.8% to 84.4%). No significant differences in the surface morphologies were observed by the scanning electron microscopy or in the maximum tolerated loads obtained by the pull test between the original ePTFE material and the ePTFE with bulging sinuses (121 and 122 N in longitudinal direction and 115 and 121 N in circumferential direction; p = 0.88 and p = 0.68).

Conclusions: The valved ePTFE conduits demonstrated excellent early clinical outcomes. The mechanical property examinations showed no obvious difference after bulging sinuses were created on the ePTFE material.
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http://dx.doi.org/10.1016/j.athoracsur.2015.02.114DOI Listing
July 2015
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