Publications by authors named "Jeffrey S Heinle"

110 Publications

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

Variables Prevalent Among Early Unplanned Readmissions in Infants Following Congenital Heart Surgery.

Pediatr Cardiol 2021 Aug 11;42(6):1449-1456. Epub 2021 May 11.

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

Medically complex children including infants undergoing cardiac surgery are at increased risk for hospital readmissions. Investigation of this population may reveal opportunities to optimize systems and coordination of care. A retrospective study of all infants undergoing cardiac surgery from 2015 through 2016 at a large tertiary institution who were readmitted within 1 year of discharge from cardiac surgical hospitalization was performed. Data specific to patient characteristics, surgical hospitalization, and readmission hospitalization are described. Unplanned readmissions within 1 year of hospital discharge were analyzed with Cox proportional hazard regression to identify factors associated with increased hazard for earlier unplanned readmission. Comparable to previous reports, 12% (78/658) of all surgical hospitalizations were associated with unplanned readmission within 30 days. Infectious etiology, followed by cardiac and gastrointestinal problems, was the most common reasons for unplanned 30-day readmission. Unplanned readmissions within 2 weeks of discharge were multifactorial and less commonly related to cardiac or surgical care. Primary nasogastric tube feeding at the time of discharge was the only significant risk factor for earlier unplanned readmission (p = 0.032) on multivariable analysis. Increased care coordination with particular attention to feeding and comorbidity management may be future targets to effectively mitigate readmissions and improve quality of care in this population.
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http://dx.doi.org/10.1007/s00246-021-02631-zDOI Listing
August 2021

Total anomalous pulmonary venous connection: Influence of heterotaxy and venous obstruction on outcomes.

J Thorac Cardiovasc Surg 2021 Apr 2. Epub 2021 Apr 2.

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

Background: Previous studies have demonstrated increased early mortality and pulmonary vein reintervention for patients with total anomalous pulmonary venous connection (TAPVC) and heterotaxy syndrome (HTX+) compared with patients with TAPVC without heterotaxy syndrome (HTX-). We aimed to evaluate the longitudinal risk of pulmonary vein reintervention and mortality in HTX + patients.

Methods: A retrospective review was performed to identify longitudinal interventions in patients with TAPVC seen at a single center from 1995 to 2019. The mean cumulative interventions were described for all patients using the Nelson-Aalen estimator. Survival with TAPVC was described using Kaplan-Meier estimates.

Results: A total of 336 patients were identified with TAPVC, of whom 118 (35%) had heterotaxy syndrome. Functional single ventricles were identified in 106 of these 118 HTX + patients (90%) and in 14 of 218 HTX- patients (6%) (P < .001). Obstructed TAPVC (OBS+) was present in 49 of 118 HTX + patients (42%) and in 87 of 218 HTX- patients (40%) (P = .89). The median duration of follow-up was 6.5 years. Five-year survival was 69% for HTX+/OBS + patients, 72% for HTX+/OBS- patients, 86% for HTX-/OBS + patients, and 95% for HTX-/OBS- patients (P < .0001, log-rank test). The mean number of pulmonary vein interventions at the median follow-up time was greater in the HTX+/OBS + patients compared with HTX+/OBS- patients (mean, 2.0 vs 1.1; P = .030), HTX-/OBS + patients (mean, 1.3; P = .033), and HTX-/OBS- patients (mean, 1.3; P = .029).

Conclusions: Among the 4 cohorts, HTX+ was associated with a higher rate of mortality, and HTX+/OBS+ was associated with a greater number of pulmonary vein interventions. This may be due in part to the high prevalence of single ventricle physiology in the HTX + cohort.
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http://dx.doi.org/10.1016/j.jtcvs.2021.03.058DOI Listing
April 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

Ultrasound-guided intraoperative trans-epicardial needle biopsy of an intracardiac tumor.

Ann Pediatr Cardiol 2020 Oct-Dec;13(4):346-348. Epub 2020 Jul 24.

Department of Pediatrics, Section of Pediatric Cardiology, Texas Children's Hospital, Houston, TX, USA.

We describe the use of a novel interventional approach to the histopathologic diagnosis of a ventricular septal tumor using intraoperative ultrasound-guided trans-epicardial biopsy without the need for cardiopulmonary bypass in a 2-year-old child. This novel approach has not been previously reported. Multidisciplinary collaboration between cardiothoracic surgery, cardiology, cardiac imaging, and interventional radiology provided the ability to perform cardiac biopsy. This technique may be used in specific cases of cardiac tumors where tissue diagnosis is important, but surgical resection is deemed excessively risky or impossible.
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http://dx.doi.org/10.4103/apc.APC_45_20DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7727904PMC
July 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

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

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

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

Mandibular Distraction in Robin Sequence With Multi-Level Airway Disease: Always Contraindicated?

J Craniofac Surg 2020 Oct;31(7):1883-1887

Division of Plastic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine.

In patients with Pierre Robin sequence (PRS), understanding the mechanism of airway obstruction and the potential for concurrent airway lesions is important for determining appropriate intervention. When concomitant airway anomalies are present with PRS, mandibular distraction osteogenesis alone is often contraindicated and unsuccessful at alleviating the obstruction, mandating the need for tracheostomy. Herein, the authors present the second-reported case of PRS with concomitant complete tracheal rings and highlight the importance collaborative surgical efforts to avoid tracheostomy. Our patient was born with signs and symptoms of PRS and concomitant complete tracheal rings. At birth, he developed respiratory compromise that was resistant to invasive and noninvasive intervention and was transferred to our care. The severity of his airway compromise necessitated operative intervention. Due to the patient's complex medical condition, avoiding the long-term sequelae of a tracheostomy and performing mandibular distraction was favorable. However, due to his multi-level airway disease, a 2-staged approach was planned: sliding tracheoplasty to release the tracheal rings, followed by mandibular distraction to correct the micrognathia. The patient tolerated both procedures and was successfully distracted and eventually weaned into room air. When managing patients with PRS in the setting of severe respiratory distress, securing the airway and carefully evaluating for concomitant airway anomalies is crucial. Even in the setting of multi-level airway disease where mandibular distraction is typically contraindicated, by directly addressing each anatomic level of airway obstruction, the airway can be safely secured and the resultant consequences of tracheostomy can be avoided. Careful planning with a multidisciplinary team is essential.
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http://dx.doi.org/10.1097/SCS.0000000000006620DOI Listing
October 2020

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

Durability of the St. Jude Epic Supra Bioprosthetic Valve in the Pulmonary Position.

Semin Thorac Cardiovasc Surg 2021 Spring;33(1):184-191. Epub 2020 Jun 4.

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

Epic Supra valves have been used off-label in the pulmonary position. We aim to evaluate the durability of Epic valves in the pulmonary position. We performed a retrospective review of all Epic valves placed in the pulmonary position from October 2008 to May 2019. Time-to-event analysis was performed using Kaplan-Meier estimates to evaluate freedom from valve intervention, moderate pulmonary regurgitation, and peak velocity greater than 3.5 m/s. Valve dysfunction was a composite of all 3 end points. A total of 79 patients had Epic valves implanted in the pulmonary position. Median age was 18.5 years (15th-85th percentile 11.2-41.0). In total, 1 (1%) 19 mm valve, 4 (5%) 21 mm valves, 8 (10%) 23 mm valves, 23 (29%) 25 mm valves, and 43 (54%) 27 mm valves were implanted. There were no deaths or transplants. Median follow-up was 3.1 years (interquartile range 1.0-5.5). At 5 years, freedom from valve intervention was 95%, freedom from valve dysfunction was 68%, freedom from moderate pulmonary regurgitation was 73%, and freedom from peak velocity greater than 3.5 m/s was 82%. Epic Supra valves provide an acceptable valve replacement in the pulmonary position for children and adults. Longer follow-up is needed to determine valve durability through the entirety of the valve life expectancy.
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http://dx.doi.org/10.1053/j.semtcvs.2020.05.026DOI Listing
May 2021

Primary Aortic Root Replacement Outcomes and Risk Factors in Pediatric Patients.

Ann Thorac Surg 2020 07 3;110(1):189-197. Epub 2020 Apr 3.

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

Background: The study sought to identify the optimal aortic root replacement (ARR) strategy for pediatric patients, and a single-center experience was analyzed.

Methods: Retrospective review of patients undergoing ARR from 1995 to 2018 was performed. Patients were stratified by surgical strategy (pulmonary autograft [Ross procedure], aortic homograft, mechanical valve conduit [Bentall procedure], or porcine xenograft [Freestyle bioprosthesis]) and aortic annulus size.

Results: ARR was performed in 206 patients with a median follow-up of 5.0 (interquartile range, 1.4-11.4) years. Root replacements included Ross procedure (n = 98), homograft (n = 83), Bentall procedure (n = 18), and Freestyle bioprosthesis (n = 7). Overall survival was 92%, and freedom from reoperation or death was 81%. Reoperation-free survival was superior in the Ross group when compared with other groups. Because surgical options differ based on the size of the aortic annulus, the analysis was arbitrarily stratified. When the aortic annulus diameter was greater than 19 mm (n = 74), procedures included Ross procedure (n = 23), homograft (n = 29), Bentall procedure (n = 17), and Freestyle bioprosthesis (n = 5). Reoperation-free survival at median follow-up (5 years) was 86%, 58%, 100%, and 100%, respectively. The Bentall procedure offered the longest freedom from reoperation. In the subset with aortic annulus diameter less than 19 mm and a pulmonary valve suitable for a Ross procedure, patients underwent the Ross procedure (n = 75) or homograft ARR (n = 36). At median follow-up (3.8 years), reoperation-free survival was longer after the Ross procedure than after homograft ARR (88% vs 46%; P < .001).

Conclusions: In patients with a large aortic annulus, a Bentall ARR offers the longest reoperation-free survival. For patients with small aortic roots, a Ross procedure provides better a reoperation-free survival than does homograft ARR.
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http://dx.doi.org/10.1016/j.athoracsur.2020.02.060DOI Listing
July 2020

Twenty-Three-Year Experience With the Arterial Switch Operation: Expectations and Long-Term Outcomes.

Semin Thorac Cardiovasc Surg 2020 Summer;32(2):292-299. Epub 2020 Jan 17.

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

We aimed to describe the short- and long-term outcomes of patients after an arterial switch operation (ASO) at a single institution during a 23-year period. A retrospective chart review of all patients <18 months of age who underwent an ASO between January 1995 and March 2018 at Texas Children's Hospital, Houston, TX was performed. Primary endpoints include mortality and reintervention. Perioperative mortality was defined as mortality occurring in-hospital and/or <30 days after surgery. Survival and freedom-from-reintervention were analyzed using Kaplan-Meier method, log-rank tests, and Cox regression models. The cohort included 394 patients. Diagnoses included 204 patients (52%) with intact ventricular septum, 137 (35%) with a ventricular septal defect, 17 (4%) with a ventricular septal defect and left ventricular outflow tract obstruction (LVOTO), and 36 (9%) with Taussig-Bing anomaly. Median age at surgery was 8 days (range: 1 day to 17 months) and median weight was 3.4 (range: 0.8-12.0) kg. Overall perioperative mortality was 1.3% (n = 5), 0.3% (n = 1) since 1999. Overall survival at 5, 10, and 15 years was 98.2%, 97.8%, and 97.8%, respectively. Perioperative morality was associated with prematurity (P = 0.012), <2.5 kg (P< 0.001), and longer circulatory arrest (P = 0.024) after univariate analysis. Reintervention was associated with a longer cross-clamp time (P < 0.001), <2.5 kg (P = 0.009), LVOTO resection (P = 0.047), and genetic syndrome (P= 0.011) after multivariable analysis. Current ASO expectations should include a perioperative mortality risk of <1% and good long-term survival. Reinterventions are more frequent in patients <2.5 kg, concomitant LVOTO resection, a genetic syndrome, and longer cross-clamp time.
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http://dx.doi.org/10.1053/j.semtcvs.2020.01.004DOI Listing
September 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

Outcomes Associated With Unplanned Interstage Cardiac Interventions After Norwood Palliation.

Ann Thorac Surg 2019 11 7;108(5):1423-1429. Epub 2019 Aug 7.

Division of Critical Care, Department of Pediatrics, Michael E. DeBakey Department of Surgery, Texas Children's Hospital and Baylor College of Medicine, Houston, Texas.

Background: Anatomic lesions are a common cause of decompensation during the interstage period after Norwood stage 1 palliation (S1P). This study describes the risk factors for and outcomes after unplanned surgical and catheter-based interstage cardiac interventions.

Methods: Participants in the National Pediatric Cardiology Quality Improvement Collaborative registry discharged from the hospital after S1P between 2008 and 2016 were studied. Variables at S1P, interstage, and at stage 2 palliation (S2P) hospitalizations were examined. Multivariable logistic regression was used to compare those who had an unplanned interstage intervention to those who did not.

Results: Of 1994 participants from 60 programs, 343 (17.1%) had at least 1 unplanned interstage intervention. Aortic valve dilation before S1P, longer S1P cardiopulmonary bypass time, pulmonary artery stent placement between S1P and discharge, aortic arch obstruction on the S1P discharge echocardiogram, and lower weight at S1P discharge were independently associated with receiving an unplanned interstage intervention. Interstage mortality between groups was similar at 6%, as was interstage duration. Participants undergoing unplanned interstage interventions were more likely to undergo heart transplant before S2P or deemed to be unsuitable for S2P (7.3% vs 2.7%, P < .001).

Conclusions: Unplanned interstage interventions after S1P did not increase interstage mortality, but participants with an unplanned intervention were less likely to progress to S2P. Residual anatomic lesions are risk factors for unplanned interstage interventions. For those with progressive ventricular dysfunction in the presence of arch obstruction by echocardiogram, aortic arch reintervention is warranted.
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http://dx.doi.org/10.1016/j.athoracsur.2019.06.041DOI Listing
November 2019

Contemporary Outcomes of the Fontan Operation: A Large Single-Institution Cohort.

Ann Thorac Surg 2019 11 4;108(5):1439-1446. Epub 2019 Jul 4.

Adult Congenital Heart Disease Program, Texas Children's Hospital/Baylor College of Medicine, Houston, Texas. Electronic address:

Background: The Fontan procedure is the accepted standard for single-ventricle palliation. The goal of this study was to determine short- and midterm outcomes of patients undergoing a Fontan operation at a single institution and to identify contemporary risk factors for acute and chronic failure.

Methods: All patients undergoing a Fontan operation between 1995 and 2016 were included. Failure was defined as death, transplantation, Fontan takedown or revision, fenestration creation or enlargement, plastic bronchitis, protein-losing enteropathy, or major perioperative reintervention. Multivariable logistic and Cox regression models were used to identify risk factors for acute (perioperative) and chronic failure (after hospital discharge or 30 days postoperatively, or both).

Results: The cohort included 610 patients. Median age at surgery was 4 years. Median follow-up was 6.8 years. Trends showed increasing use of extracardiac conduits, nonfenestrated Fontan, and extubation in the operating room. Perioperative mortality was 0.5% (n = 3). Transplant-free survival at 5, 10, and 15 years was 97%, 94%, and 92%, respectively; freedom from failure was 91%, 89%, and 87%, respectively. Extubation in the operating room was associated with lower risk of acute failure (odds ratio, 0.30; 95% confidence interval [CI], 0.11 to 0.87). Independent risk factors for chronic failure included genetic syndrome (hazard ratio [HR], 2.54; 95% CI, 1.11 to 5.83), ventricular dysfunction (HR, 3.86; 95% CI, 1.81 to 8.24), cardiopulmonary bypass time in 30-minute intervals (HR, 1.242; 95% CI, 1.100 to 1.402), and persistent pleural effusions (HR, 4.26; 95% CI, 2.25 to 8.07). Moderate or severe atrioventricular valve regurgitation (HR, 2.61; 95% CI, 1.13 to 6.02) and cardiopulmonary bypass time (HR, 1.22; 95% CI, 1.03 to 1.45) were associated with reduced long-term transplant-free survival.

Conclusions: Contemporary midterm outcomes for Fontan patients are reassuring. Lifelong follow-up is mandatory to determine long-term outcomes and need for additional surgery as patients reach adulthood.
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http://dx.doi.org/10.1016/j.athoracsur.2019.05.039DOI Listing
November 2019

Characteristics and Outcomes of Pediatric Patients Supported With Ventricular Assist Device-A Multi-Institutional Analysis.

Pediatr Crit Care Med 2019 08;20(8):744-752

Section of Critical Care Medicine, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, Houston, TX.

Objectives: The use of ventricular assist devices for pediatric patients with heart failure is increasing, but is associated with significant morbidity and mortality. Our objectives were to describe the admission outcomes and resource utilization of pediatric patients supported with ventricular assist devices, utilizing a multicenter database.

Data Sources: Pediatric Health Information System database (comprising 49 nonprofit children's hospitals).

Study Selection: Retrospective cohort analysis of the database from January 2006 to September 2015 for all admissions less than or equal to 21 years old with ventricular assist device implantation.

Data Extraction: The primary outcome was hospital mortality. The secondary outcomes were hospital length of stay and adjusted cost.

Data Synthesis: We analyzed 744 ventricular assist device implantations (740 patients), 422 (57%) males, and 363 (49%) non-Hispanic white. Median age at admission was 5.9 years (interquartile range, 0.9-13.5 yr), and median length of stay was 69 days (interquartile range, 36-122 d). The overall hospital mortality was 188 (25%), whereas 395 (53%) were transplanted and 141 (19%) were discharged on ventricular assist device. Extracorporeal membrane oxygenation was used, in addition to ventricular assist device, in 340 (46%). The majority of ventricular assist device implantations (453, 61%) were from 2011 to 2015 (compared to 2006-2010). More patients discharged on ventricular assist device from 2011 to 2015 (23% vs 13% in 2006-2010; p = 0.001). There was no difference in median age, mortality, length of stay, or adjusted costs between these time periods. On multivariable analysis, underlying congenital heart disease, renal failure, liver congestion, sepsis, cerebrovascular accident, and extracorporeal membrane oxygenation were associated with hospital mortality. Sepsis and ventricular assist device replacement/repair were associated with higher adjusted cost and longer length of stay.

Conclusions: The pediatric ventricular assist device experience continues to grow, with a significant increase in the number of patients undergoing ventricular assist device implantation and a higher proportion being discharged from hospital on ventricular assist device support in recent years. Underlying congenital heart disease, renal failure, sepsis, cerebrovascular accident, and extracorporeal membrane oxygenation are significantly associated with hospital mortality.
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http://dx.doi.org/10.1097/PCC.0000000000001966DOI Listing
August 2019

The miniaturized pediatric continuous-flow device: A successful bridge to heart transplant.

J Heart Lung Transplant 2019 07 29;38(7):789-793. Epub 2019 Apr 29.

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

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http://dx.doi.org/10.1016/j.healun.2019.04.004DOI Listing
July 2019

Contemporary results after repair of partial and transitional atrioventricular septal defects.

J Thorac Cardiovasc Surg 2019 03 24;157(3):1117-1127.e4. Epub 2018 Nov 24.

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

Objective: The exact incidence and risk factors for reoperation in partial and transitional atrioventricular septal defects are unclear. The goal of this study was to assess risk factors for left atrioventricular valve and left ventricular outflow tract reoperation in partial and transitional atrioventricular septal defects.

Methods: All patients undergoing partial and transitional atrioventricular septal defects repair between 1995 and 2017 were reviewed. Patients were classified as infants (<1 year), toddlers (1-3 years), children (3-17 years), and adults (≥18 years). Survival and reoperation were assessed using log-rank test and Cox models for univariate and multivariable analyses, respectively.

Results: Overall, 265 patients underwent partial and transitional atrioventricular septal defects repair (partial: 177 [67%]). Median age was 2 years. The cohort included 73 infants (28%), 85 toddlers (32%), 94 children (35%), and 13 adults (5%). Trisomy 21 was present in 76 patients (29%), and in 216 patients (83%), the zone of apposition was completely closed. Perioperative mortality was 0.8%. Complete heart block did not develop in any patients. Ten-year survival and freedom from reoperation were 98% and 81%, respectively. On multivariable analysis, trisomy 21 (hazard ratio [HR], 0.16) and older age compared with infants (toddlers: HR, 0.35; children: HR, 0.25) were protective for any reoperation, whereas heterotaxy (HR, 3.43) was a risk factor. For left atrioventricular valve reoperation, toddlers (HR, 0.35), children (HR, 0.25), and trisomy 21 (HR, 0.16) remained protective, whereas left atrioventricular valve anomaly was a risk factor (HR, 2.61). Likewise, for left ventricular outflow tract reoperation, toddlers (HR, 0.24) and children (HR, 0.06) were protective.

Conclusions: Mortality after partial and transitional atrioventricular septal defects repair is minimal, yet reoperation for left atrioventricular valve disease and left ventricular outflow tract obstruction remains significant. Patients requiring repair during infancy are at higher risk of reoperation.
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http://dx.doi.org/10.1016/j.jtcvs.2018.10.154DOI Listing
March 2019

Ventricular Assist Device Support: Single Pediatric Institution Experience Over Two Decades.

Ann Thorac Surg 2019 03 6;107(3):829-836. Epub 2018 Oct 6.

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

Background: We reviewed our single institutional experience with pediatric ventricular assist device (VAD) support over the last 2 decades, with an aim to improve our current management and gain an insight into the future direction.

Methods: A retrospective review was conducted on all patients that had undergone VAD support between 1996 and 2017. Outcomes were analyzed based on the type of VADs, whether temporary or durable devices. Primary end points were positive outcomes, including bridge-to-transplantation, bridge-to-recovery, alive on device, and bridge-to-bridge to another VAD, or negative outcomes, including death during VAD support or in-hospital death after bridge-to-recovery. The Pediatric Interagency Registry for Mechanical Circulatory Support definition was used to classify adverse events.

Results: Overall, 201 VADs were implanted in 159 patients, with 82 (41%) and 119 (59%) being temporary and durable support, respectively. There has been a trend toward an increasing annual implant volume both with temporary and durable VADs. Positive outcomes were achieved in 80% (66 of 82) of those with temporary support, with bridge-to-recovery (53% [35 of 66]) and bridge-to-bridge to another VAD (38% [25 of 66]) being the predominant outcomes. Of those on durable support, 84% (100 of 119) achieved positive outcomes, with bridge-to-transplant (66% [78 of 119]) being the leading destination. The most notable change during the study period was the introduction of implantable continuous-flow VADs, resulting in outpatient management becoming a routine practice. No patients were discharged on VAD support before 2004, but 85% were discharged on VADs with discharge capability after 2013.

Conclusions: The present study has demonstrated the evolutional changes of pediatric VAD support and their effect on clinical outcomes over the last 2 decades.
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http://dx.doi.org/10.1016/j.athoracsur.2018.08.018DOI Listing
March 2019

Pulsatile Glenn as long-term palliation for single ventricle physiology patients.

Congenit Heart Dis 2018 Nov 2;13(6):927-934. Epub 2018 Oct 2.

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

Objective: There are limited studies analyzing pulsatile Glenn as a long-term palliation strategy for single ventricle patients. This study sought to determine their outcomes at a single institution.

Design: A retrospective review was performed.

Setting: Study performed at a single pediatric hospital.

Patients: All single ventricle patients who underwent pulsatile Glenn from 1995 to 2016 were included.

Outcome Measures: Pulsatile Glenn failure was defined as takedown, transplant, or death. Further palliation was defined as Fontan, 1.5, or biventricular repair. Risk factors were assessed by Cox multivariable competing risk analyses.

Results: Seventy-eight patients underwent pulsatile Glenn at age 9 months (interquartile range, 5-14). In total, 28% had heterotaxy, 18% had a genetic syndrome, and 24% had an abnormal inferior vena cava. There were 3 (4%) perioperative mortalities. Further palliation was performed in 41 (53%) patients with a median time-to-palliation of 4 years (interquartile range, 3-5). Pulsatile Glenn failure occurred in 10 (13%) patients with 8 total mortalities. Five- and 10-year transplant-free survival were 91% and 84%, respectively. At a median follow-up of 6 years (interquartile range, 2-8), 27 patients (35%) remained with PG (age 7 years [interquartile range, 3-11], oxygen saturation 83% ± 4%). Preoperative moderate-severe atrioventricular valve regurgitation (AVVR) (hazard ratio 7.77; 95% confidence interval 1.80-33.43; P =.005) and higher pulmonary vascular resistance (hazard ratio 2.59; 95% confidence interval 1.08-6.15; P =.031) were predictors of pulsatile Glenn failure after adjusting for covariates. Reaching further palliation was less likely in patients with preoperative moderate-severe AVVR (hazard ratio 0.22, 95% confidence interval 0.08-0.59; P =.002).

Conclusion: Pulsatile Glenn can be an effective tool to be used in challenging circumstances, these patients can have a favorable long-term prognosis without reducing their suitability for further palliation.
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http://dx.doi.org/10.1111/chd.12664DOI Listing
November 2018

Cortisol Response in Children After Second Cardiopulmonary Bypass.

Pediatr Cardiol 2019 Jan 27;40(1):47-52. Epub 2018 Aug 27.

Department of Pediatrics, Section of Critical Care, Texas Children's Hospital, Baylor College of Medicine, 6621 Fannin St. Suite W6006, Houston, TX, 77030, USA.

A surge in cortisol levels is seen after surgery with cardiopulmonary bypass (CPB). Based on evidence of attenuation of the cortisol response to repeated stress in other settings, we hypothesized that the magnitude of cortisol increase in children after a second exposure to CPB would be reduced. Serial cortisol levels were measured at three time points after each CPB: immediately (day 0), on the first morning (day 1), and second morning (day 2). Forty-six children underwent two surgeries with CPB during the study period. The mean age (standard deviation) at first and second surgery was 3.5 (6.3) months and 10.4 (9.9) months, respectively. Cortisol levels at the first surgery were 109 (105) µg/dl, 29 (62) µg/dl, and 17 (12) µg/dl on day 0, 1, and 2, respectively; similarly at second surgery, it was 61 (57) µg/dl on day 0 to 20 (16) µg/dl and 11 (10) µg/dl on day 1 and 2, respectively. After log-transformation and adjusting for time interval between surgeries, cortisol levels at the second surgery were lower by 42% on day 0 (p = 0.02), and 46% lower on day 2 (p = 0.02). A second exposure to CPB in children with congenital heart disease is associated with an attenuated cortisol release.
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http://dx.doi.org/10.1007/s00246-018-1959-8DOI Listing
January 2019

Transposition of the great arteries: When echocardiography does not match the clinical picture.

J Clin Ultrasound 2018 Nov 30;46(9):617-622. Epub 2018 Aug 30.

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

Aortopulmonary window (APW) is a rare form of congenital heart disease seen in isolation or with complex cardiac lesions. APW has been associated with other cardiac defects such as interrupted aortic arch and Tetralogy of Fallot, but few cases have been reported of APW associated with transposition of the great arteries (TGA). In a newborn with TGA and intact ventricular septum, diagnosis of APW requires a high index of suspicion. This article reviews the literature on TGA with APW and illustrates the importance of additional evaluation in neonates with TGA when oxygen saturation and PaO2 do not match predicted clinical values.
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http://dx.doi.org/10.1002/jcu.22615DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6710829PMC
November 2018
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