Publications by authors named "Ziyad M Binsalamah"

30 Publications

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Corrigendum to "Congenitally Corrected Transposition of the Great Arteries: Anatomic, Physiologic Repair, and Palliation" [Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann 2019;22:32-42].

Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2021 ;24:95-96

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

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http://dx.doi.org/10.1053/j.pcsu.2021.05.001DOI Listing
January 2021

Anomalous Aortic Origin of the Coronary Arteries - State of the Art Management and Surgical Techniques.

Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2021 ;24:85-94

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

Anomalous aortic origin of a coronary artery (AAOCA) can be associated with myocardial ischemia and sudden cardiac arrest. We describe and compare the management and surgical techniques for patients with AAOCA. Patients presenting to the Coronary Artery Anomalies Program are evaluated and managed following a standardized approach. Our approach and data were compared to other single-center and multi-institutional data and results. Patients with AAOCA present as an incidental finding approximately 50% of the time. Advanced axial imaging is essential to define the anatomic characteristics of this lesion. Preoperative and postoperative assessment of myocardial perfusion with provocative testing is feasible and contributes to risk stratification. The surgical techniques for AAOCA repair include coronary unroofing, transection and reimplantation, and neo-ostium creation, among others. In general, surgical repair of AAOCA can mitigate the risk of ischemia with low mortality. The specific morbidities and complications of each different technique should be considered during the surgical planning. Surgical repair of AAOCA can mitigate the risk of ischemia with a low associated mortality but with clinically relevant morbidities. Long-term follow-up is necessary to accurately balance the risks of repaired and unrepaired AAOCA.
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http://dx.doi.org/10.1053/j.pcsu.2021.03.004DOI Listing
January 2021

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

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

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

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

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

Outcomes in anomalous aortic origin of a coronary artery after surgical reimplantation.

J Thorac Cardiovasc Surg 2021 Jan 5. Epub 2021 Jan 5.

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

Objective: Anomalous aortic origin of a coronary artery (AAOCA) can be associated with myocardial ischemia and sudden cardiac arrest. We compared outcomes data of patients who underwent transection and reimplantation (TAR) and patients who underwent an unroofing.

Methods: Patients who presented to the Coronary Artery Anomalies Program were evaluated and managed following a standardized approach. Anatomy was determined using computed tomography angiography, myocardial perfusion using advanced stress imaging, and surgical intervention according to anatomic features.

Results: Sixty-one patients underwent surgical repair of AAOCA between 2012 and 2019: 16 (26%) patients underwent TAR of the anomalous coronary without an aortic button and 45 (74%) patients underwent coronary unroofing. Compared with patients who underwent an unroofing, patients who underwent TAR had similar intramural length (5 mm with interquartile range of 4-7.7 vs 6 mm with interquartile range of 5-7; P = .6). One patient with an anomalous right coronary underwent coronary artery bypass grafting after TAR because of persistent postoperative ischemic changes. One patient with unroofing of an anomalous left coronary artery presented with recurrent aborted sudden cardiac death and underwent subsequent TAR, without further events. At last follow-up, 15 of 16 patients (94%) who underwent TAR and 42 of 45 (93%) patients who underwent an unroofing were released to unrestricted exercise activities.

Conclusions: Coronary artery TAR is a useful surgical alternative for AAOCA when there is a course below the commissure, when unroofing does not relocate the ostium to the appropriate sinus, or when unroofing results in compression by the intercoronary pillar.
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http://dx.doi.org/10.1016/j.jtcvs.2020.12.100DOI Listing
January 2021

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Sickle Cell-Related Complications in Patients Undergoing Cardiopulmonary Bypass.

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

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

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

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

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

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

Predictors of Transplant-Free Survival After the Norwood Procedure.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Replacement of Left Ventricular Assist Device Inflow Cannula Using a Right Angle Atrial Cannula.

Ann Thorac Surg 2020 05 23;109(5):e389. Epub 2020 Jan 23.

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

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http://dx.doi.org/10.1016/j.athoracsur.2019.11.060DOI Listing
May 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

Congenitally Corrected Transposition of the Great Arteries: Anatomic, Physiologic Repair, and Palliation.

Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2019 ;22:32-42

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

Congenitally corrected transposition of the great arteries (ccTGA) is a lesion that rarely occurs in isolation. The presenting physiology of ccTGA is predominantly secondary to the concurrent cardiac lesions; however, as the child ages, unrepaired ccTGA results in progressive failure of the morphologic right ventricle under the strain of maintaining a systemic pressure. Repair of ccTGA was initially focused on rectification of the underlying physiologic aberrations, but in recent years, the focus of repair has shifted toward anatomic correction to avoid failure of the morphologic right ventricle. This anatomic repair is commonly associated with improved long-term mortality at the cost of increased short-term mortality. Key preoperative considerations such as morphologic left ventricular pressure, tricuspid valve competency, and out flow tract obstructions can assist in determining the optimal repair for individual patients. An alternative, single ventricle, pathway has been proposed for any patient without optimal preoperative anatomy to improve long-term survival. Adjunctive repair options including pulmonary artery banding and one-and-a-half ventricle repairs have also been proposed to augment the survival curves.
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http://dx.doi.org/10.1053/j.pcsu.2019.02.008DOI Listing
December 2019

Cervical left aortic arch with distal tortuosity causing coarctation and aneurysmal formation in a child.

J Card Surg 2018 Aug 26;33(8):466-468. Epub 2018 Jun 26.

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

We present a case of a 10-year-old female with a cervical aortic arch and distal transverse arch tortuosity causing coarctation and aneurysmal formation and describe the surgical repair of this rare congenital anomaly.
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http://dx.doi.org/10.1111/jocs.13750DOI Listing
August 2018

Type A interrupted aortic arch and type III aortopulmonary window with anomalous origin of the right pulmonary artery from the aorta.

J Card Surg 2018 Jun 10;33(6):344-347. Epub 2018 May 10.

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

Interruption of the aortic arch, aortopulmonary window, and anomalous origin of the right pulmonary artery from the ascending aorta are very rare congenital anomalies. It is even rarer to have all three anomalies in the same setting. We present a case of a newborn who was diagnosed with these lesions and describe the primary repair of these anomalies.
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http://dx.doi.org/10.1111/jocs.13717DOI Listing
June 2018

Circumflex aorta with double aortic arch in an infant.

J Card Surg 2018 May 17;33(5):292-295. Epub 2018 Apr 17.

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

Circumflex aorta and double aortic arch are two forms of rare vascular rings. We present a case of an infant who was diagnosed with circumflex aorta and double aortic arch, and describe the surgical management of this rare anomaly.
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http://dx.doi.org/10.1111/jocs.13604DOI Listing
May 2018

A pulmonary artery sling with a vascular ring in a toddler: an uncommon combination.

Cardiol Young 2018 May 1;28(5):783-785. Epub 2018 Mar 1.

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

Pulmonary artery slings and vascular rings are very rare congenital anomalies. It is even rarer to have both anomalies in the same setting. We present a case of a toddler who was diagnosed with a left pulmonary artery sling and a vascular ring as part of the screening process for the VACTERL association - co-occurrence of vertebral, anorectal, cardiac, tracheoesophageal, renal, and limb malformations. He underwent a successful surgical repair via median sternotomy and on cardiopulmonary bypass with an uneventful postoperative course.
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http://dx.doi.org/10.1017/S1047951118000148DOI Listing
May 2018

Acute humoral rejection in an infant without risk factors after heart transplantation.

J Card Surg 2018 Feb 4;33(2):126-128. Epub 2018 Feb 4.

Congenital Heart Surgery and Pediatric Cardiology, Texas Children's Hospital-Baylor College of Medicine, Houston, Texas.

Graft rejection is the most common factor that limits graft survival after transplantation. During infancy, the humoral immune system is partially suppressed and humoral rejection of a cardiac allograft has not been reported in the absence of risk factors such as prior transplantation, blood transfusions, ventricular assist devices, and elevation of panel reactive antibodies. We present a case of an infant with dilated cardiomyopathy who developed multiple episodes of acute humoral rejection after heart transplantation in the absence of risk factors.
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http://dx.doi.org/10.1111/jocs.13532DOI Listing
February 2018

Anomalous origin of the left coronary artery from the right pulmonary artery in a univentricular heart.

Cardiol Young 2017 Nov 27;27(9):1853-1856. Epub 2017 Jun 27.

1Michael E. DeBakey Department of Surgery,Division of Congenital Heart Surgery,Baylor College of Medicine,Houston,Texas,United States of America.

Anomalous origin of the left coronary artery from the right pulmonary artery in single ventricles is a very rare congenital anomaly. Failure to recognise it preoperatively may lead to adverse outcomes, including death. We report the case of a neonate with a univentricular heart in the form of a double-outlet right ventricle, mitral atresia with discrete coarctation of the aorta, and an incidental intraoperative finding of an anomalous origin of the left coronary artery from the right pulmonary artery. The patient underwent a successful repair with an uneventful postoperative course.
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http://dx.doi.org/10.1017/S1047951117001317DOI Listing
November 2017

Cor triatriatum sinister with an intact interatrial septum and a decompressing vein in a toddler.

Cardiol Young 2017 Aug 6;27(6):1221-1224. Epub 2017 Mar 6.

1Division of Congenital Heart Surgery,Texas Children's Hospital,Houston,TX,United States of America.

Cor triatriatum sinister is a very rare cardiac anomaly that may lead to pulmonary hypertension, right ventricular dilation, and eventually right heart failure. We report a case of a toddler who presented with respiratory distress and cardiomegaly and was found to have cor triatriatum sinister with a restrictive communication, decompressing vertical vein, pulmonary hypertension, severe tricuspid regurgitation, and severe right ventricular dysfunction. She underwent a successful surgical repair, with normalisation of right ventricular function and pulmonary artery pressure.
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http://dx.doi.org/10.1017/S1047951117000130DOI Listing
August 2017

Aortic arch advancement for type A interrupted aortic arch with persistent fifth aortic arch type B.

Cardiol Young 2017 Jul 6;27(5):1018-1021. Epub 2017 Mar 6.

Michael E. DeBakey Department of Surgery,Division of Congenital Heart Surgery,Baylor College of Medicine,Houston,Texas,United States of America.

Persistence of the fifth aortic arch is a very rare anomaly, but is clinically relevant when it is associated with coarctation. We report a case of a neonate with type A interrupted aortic arch and severe coarctation of a persistent fifth aortic arch, which was discovered after repair of a left congenital diaphragmatic hernia. The combination of anomalies was discovered intra-operatively following left thoracotomy, and was treated with aortic arch advancement. The postoperative course was uneventful.
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http://dx.doi.org/10.1017/S1047951117000051DOI Listing
July 2017

Mid-term outcome and angiographic follow-up of endarterectomy of the left anterior descending artery in patients undergoing coronary artery bypass surgery.

J Card Surg 2014 Jan 17;29(1):1-7. Epub 2013 Oct 17.

Department of Surgery, Division of Cardiac Surgery, Royal Victoria Hospital, Montreal, Quebec, Canada.

Background And Aim: With the advancement of percutaneous coronary interventions (PCIs), more patients with diffuse coronary artery disease are referred for coronary artery bypass graft (CABG) surgery. The use of coronary endarterectomy may be useful in such cases. We reviewed our experience with left anterior descending artery endarterectomy as an adjunct to conventional CABG.

Methods: Between June 2005 and 2011, 58 consecutive patients underwent left anterior descending artery endarterectomy as an adjunct to CABG. These were matched to 58 cases based on age, gender, and Parsonnet score. All data were collected prospectively in a departmental database. Postoperative complications and in-hospital mortality were analyzed. Survival curves were produced.

Results: There was one death in the endarterectomy group (1.7%) from liver failure. There was no significant difference in postoperative complications (especially perioperative myocardial infarction) between the two groups with similar hospital mortality. Computed tomography (CT) angiography was performed in 24 patients with endarterectomy (41%), which showed 100% patency of the left internal mammary artery graft to the left anterior descending artery. Survival and freedom from intervention at a mean follow-up of 4.2 years were similar.

Conclusions: In patients with diffuse disease, the use of endarterectomy is a safe technique with no increase in short-term morbidity or mortality. Mid-term results are similar to nonendarterectomized patients. This technique is useful in patients with diffuse coronary artery disease.
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http://dx.doi.org/10.1111/jocs.12230DOI Listing
January 2014
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