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Collins GL, Hargis-Villanueva AE, Jayaraman MS, Lai K, Weidler EM, Griffiths PS, Rhee EK, Jorgensen S, Youssfi M, van Leeuwen K. A Prospective Management Strategy for Heterotaxy Syndrome with Intestinal Rotation Abnormalities: Imaging Does Not Predict Need for Surgery. J Pediatr Surg 2023; 58:1838-1842. [PMID: 36740478 DOI: 10.1016/j.jpedsurg.2023.01.002] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/11/2022] [Revised: 12/22/2022] [Accepted: 01/03/2023] [Indexed: 01/12/2023]
Abstract
PURPOSE Heterotaxy syndrome (HS) affects right-left anatomical development in 3% of children with congenital heart disease. Commonly, these patients have intestinal rotation abnormalities (IRA) that differ from typical malrotation. In this prospective study, we examine the development of a management pathway, imaging findings, and clinical course of patients with HS and IRA. METHODS After literature review, a multispecialty focus group designed a pathway for HS. Participants from a single institution were prospectively enrolled from 2016 to 2019. They underwent an abdominal ultrasound and UGI, however timing was variable based on symptoms. Symptomatic IRA was defined as feeding intolerance, bilious or non-bilious vomiting, bloating, severe reflux, and intermittent pain or abdominal distention. Screening for symptoms occurred every three months for the first two years and then annually. RESULTS 18 participants were followed for a median of 5.0 years. Three clinical categories were identified: 1) asymptomatic, not requiring intestinal surgery, 72.2%, 2) symptomatic with feeding intolerance or failure to thrive requiring gastrostomy placement and diagnostic laparoscopy with Ladd procedure, 16.7%, and 3) symptomatic requiring urgent exploration for suspicion of volvulus, 11.1%. Need for surgery did not necessarily correlate with US and/or UGI findings. CONCLUSIONS In children with HS and IRA, postnatal imaging did not inform the need for intestinal surgery. Although rare, volvulus or other forms of proximal obstruction can be anticipated, and symptomatic patients should be offered surgical intervention. A multidisciplinary care pathway for patients with HS and IRA can coordinate care and help counsel families on the likelihood of needing intestinal surgery for IRA. LEVEL OF EVIDENCE Level II. TYPE OF STUDY Prospective Cohort Study.
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Affiliation(s)
- Grant L Collins
- Division of Pediatric Surgery, Department of Surgery, Phoenix Children's Hospital, 1919 E Thomas Rd., Phoenix, AZ, 85016, USA
| | - Angela E Hargis-Villanueva
- Division of Pediatric Surgery, Department of Surgery, Phoenix Children's Hospital, 1919 E Thomas Rd., Phoenix, AZ, 85016, USA
| | - Meghna S Jayaraman
- Division of Pediatric Surgery, Department of Surgery, Phoenix Children's Hospital, 1919 E Thomas Rd., Phoenix, AZ, 85016, USA
| | - Krista Lai
- Division of Pediatric Surgery, Department of Surgery, Phoenix Children's Hospital, 1919 E Thomas Rd., Phoenix, AZ, 85016, USA
| | - Erica M Weidler
- Division of Pediatric Surgery, Department of Surgery, Phoenix Children's Hospital, 1919 E Thomas Rd., Phoenix, AZ, 85016, USA
| | - Pamela S Griffiths
- Division of Neonatology, Phoenix Children's Hospital, 1919 E Thomas Rd., Phoenix, AZ, 85016, USA
| | - Edward K Rhee
- Division of Cardiology, Phoenix Children's Hospital, 1919 E Thomas Rd., Phoenix, AZ, 85016, USA
| | - Scott Jorgensen
- Department of Radiology, Phoenix Children's Hospital, 1919 E Thomas Rd., Phoenix, AZ, 85016, USA
| | - Mostafa Youssfi
- Department of Radiology, Phoenix Children's Hospital, 1919 E Thomas Rd., Phoenix, AZ, 85016, USA
| | - Kathleen van Leeuwen
- Division of Pediatric Surgery, Department of Surgery, Phoenix Children's Hospital, 1919 E Thomas Rd., Phoenix, AZ, 85016, USA.
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Rao PS. Therapy of Patients with Cardiac Malposition. CHILDREN (BASEL, SWITZERLAND) 2023; 10:739. [PMID: 37189988 PMCID: PMC10137016 DOI: 10.3390/children10040739] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 03/10/2023] [Revised: 03/31/2023] [Accepted: 04/14/2023] [Indexed: 05/17/2023]
Abstract
Positional abnormalities per se do not require treatment, but in their place, the accompanying pulmonary pathology in dextroposition patients and pathophysiologic hemodynamic abnormalities resulting from multiple defects in patients with cardiac malposition should be the focus of treatment. At the time of the first presentation, treating the pathophysiologic aberrations caused by the defect complex, whether it is by improving the pulmonary blood flow or restricting it, is the first step. Some patients with simpler or single defects are amenable to surgical or transcatheter therapy and should be treated accordingly. Other associated defects should also be treated appropriately. Biventricular or univentricular repair dependent on the patient's cardiac structure should be planned. Complications in-between Fontan stages and after conclusion of Fontan surgery may occur and should be promptly diagnosed and addressed accordingly. Several other cardiac abnormalities unrelated to the initially identified heart defects may manifest in adulthood, and they should also be treated.
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Affiliation(s)
- P Syamasundar Rao
- Children's Heart Institute, Children's Memorial Hermann Hospital, McGovern Medical School, University of Texas-Houston, 6410 Fannin Street, UTPB Suite # 425, Houston, TX 77030, USA
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Splenic Torsion in Heterotaxy Syndrome with Left Isomerism: A Case Report and Literature Review. Diagnostics (Basel) 2022; 12:diagnostics12122920. [PMID: 36552927 PMCID: PMC9776906 DOI: 10.3390/diagnostics12122920] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/12/2022] [Revised: 11/07/2022] [Accepted: 11/22/2022] [Indexed: 11/25/2022] Open
Abstract
Splenic torsion is an unusual condition that results in congenital abnormality, especially in the visceral abnormal arrangement. We report the case of an 8.5-year-old boy with features in the right upper quadrant. Radiological investigations revealed heterotaxy syndrome with polysplenia and a hypodense tumor in the right upper quadrant adjacent to several spleens. We initially treated it as an intra-abdominal tumor. Laparoscopy was performed to check the tumor condition and revealed a congestive tumor located in the abdomen of the right upper quadrant below the central liver, which was suspected to be a torsion spleen without attaching ligaments. Laparoscopic splenectomy was successfully carried out without complications. The pathological report shows splenic tissue with hemorrhagic infarction. Physicians should be vigilant of the differential diagnosis of the acute abdomen in adolescents.
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Kwiatkowski DM, Ball MK, Savorgnan FJ, Allan CK, Dearani JA, Roth MD, Roth RZ, Sexson KS, Tweddell JS, Williams PK, Zender JE, Levy VY. Neonatal Congenital Heart Disease Surgical Readiness and Timing. Pediatrics 2022; 150:189888. [PMID: 36317977 DOI: 10.1542/peds.2022-056415d] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 08/29/2022] [Indexed: 11/06/2022] Open
Affiliation(s)
- David M Kwiatkowski
- Department of Pediatrics, Texas Tech University Health Sciences Center, Lubbock, Texas
| | - Molly K Ball
- Department of Pediatrics, Nationwide Children's Hospital, Columbus, Ohio
| | - Fabio J Savorgnan
- Department of Pediatrics, UT Southwestern, Children's Health, Dallas, Texas
| | - Catherine K Allan
- Department of Cardiology, Harvard Medical School, Boston Children's Hospital, Boston, Massachusetts
| | - Joseph A Dearani
- Department of Cardiovascular Surgery, Mayo College of Medicine, Mayo Clinic, Rochester, Minnesota
| | | | | | - Kristen S Sexson
- Department of Pediatrics, UT Southwestern, Children's Health, Dallas, Texas
| | - James S Tweddell
- Department of Surgery, University of Cincinnati, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio
| | - Patricia K Williams
- Department of Pediatrics, University of Oklahoma College of Medicine, Oklahoma City, Oklahoma
| | - Jill E Zender
- Department of Pediatrics, UT Southwestern, Children's Health, Dallas, Texas
| | - Victor Y Levy
- Department of Pediatrics, Texas Tech University Health Sciences Center, Lubbock, Texas
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5
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Saba TG, Geddes GC, Ware SM, Schidlow DN, Del Nido PJ, Rubalcava NS, Gadepalli SK, Stillwell T, Griffiths A, Bennett Murphy LM, Barber AT, Leigh MW, Sabin N, Shapiro AJ. A multi-disciplinary, comprehensive approach to management of children with heterotaxy. Orphanet J Rare Dis 2022; 17:351. [PMID: 36085154 PMCID: PMC9463860 DOI: 10.1186/s13023-022-02515-2] [Citation(s) in RCA: 11] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/08/2022] [Accepted: 09/04/2022] [Indexed: 11/10/2022] Open
Abstract
Heterotaxy (HTX) is a rare condition of abnormal thoraco-abdominal organ arrangement across the left-right axis of the body. The pathogenesis of HTX includes a derangement of the complex signaling at the left-right organizer early in embryogenesis involving motile and non-motile cilia. It can be inherited as a single-gene disorder, a phenotypic feature of a known genetic syndrome or without any clear genetic etiology. Most patients with HTX have complex cardiovascular malformations requiring surgical intervention. Surgical risks are relatively high due to several serious comorbidities often seen in patients with HTX. Asplenia or functional hyposplenism significantly increase the risk for sepsis and therefore require antimicrobial prophylaxis and immediate medical attention with fever. Intestinal rotation abnormalities are common among patients with HTX, although volvulus is rare and surgical correction carries substantial risk. While routine screening for intestinal malrotation is not recommended, providers and families should promptly address symptoms concerning for volvulus and biliary atresia, another serious morbidity more common among patients with HTX. Many patients with HTX have chronic lung disease and should be screened for primary ciliary dyskinesia, a condition of respiratory cilia impairment leading to bronchiectasis. Mental health and neurodevelopmental conditions need to be carefully considered among this population of patients living with a substantial medical burden. Optimal care of children with HTX requires a cohesive team of primary care providers and experienced subspecialists collaborating to provide compassionate, standardized and evidence-based care. In this statement, subspecialty experts experienced in HTX care and research collaborated to provide expert- and evidence-based suggestions addressing the numerous medical issues affecting children living with HTX.
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Affiliation(s)
- Thomas G Saba
- Department of Pediatrics, Pulmonary Division, University of Michigan Medical School, 1500 E. Medical Center Drive, Ann Arbor, MI, USA.
| | - Gabrielle C Geddes
- Department of Medical and Molecular Genetics, Indiana University School of Medicine, Indianapolis, IN, USA
| | - Stephanie M Ware
- Department of Medical and Molecular Genetics, Indiana University School of Medicine, Indianapolis, IN, USA
| | - David N Schidlow
- Department of Cardiology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA
| | - Pedro J Del Nido
- Department of Cardiac Surgery, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA
| | - Nathan S Rubalcava
- Department of Surgery, Section of Pediatric Surgery, University of Michigan Medical School, Ann Arbor, MI, USA
| | - Samir K Gadepalli
- Department of Surgery, Section of Pediatric Surgery, University of Michigan Medical School, Ann Arbor, MI, USA
| | - Terri Stillwell
- Department of Pediatrics, Infectious Disease Division, University of Michigan Medical School, Ann Arbor, MI, USA
| | - Anne Griffiths
- Department of Pediatrics, Pulmonary/Critical Care Division, Children's Minnesota and Children's Respiratory and Critical Care Specialists, Minneapolis, MN, USA
| | - Laura M Bennett Murphy
- Department of Pediatrics, Division of Pediatric Psychiatry and Behavioral Health, University of Utah, Primary Children's Hospital, Salt Lake City, UT, USA
| | - Andrew T Barber
- Department of Pediatrics, Division of Pulmonology, University of North Carolina School of Medicine, Chapel Hill, NC, USA
| | - Margaret W Leigh
- Department of Pediatrics, Division of Pulmonology, University of North Carolina School of Medicine, Chapel Hill, NC, USA
| | - Necia Sabin
- Heterotaxy Connection, Eagle Mountain, UT, USA
| | - Adam J Shapiro
- Department of Pediatrics, McGill University Health Centre Research Institute, Montreal, QC, Canada
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Roychaudhuri S, Grewal G, Vijayashankar SS, Lavoie P, Maheshwari A. Necrotizing Enterocolitis Associated with Congenital Heart Disease-A Review Article. NEWBORN (CLARKSVILLE, MD.) 2022; 1:170-176. [PMID: 36864827 PMCID: PMC9976612 DOI: 10.5005/jp-journals-11002-0016] [Citation(s) in RCA: 7] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Indexed: 11/23/2022]
Abstract
Necrotizing enterocolitis (NEC) is a relatively rare but devastating entity associated classically with the preterm cohort in the neonatal intensive care unit. Preterm and term babies with congenital heart disease are at risk of a number of comorbidities because of the hemodynamic derangements due to a structurally abnormal heart and the corrective procedures adopted. Necrotizing enterocolitis is one of the dreaded complications associated with this cohort and impacts the course of these babies in the hospital in a major way. A large majority of term babies with NEC are in the backdrop of a significant congenital cardiac lesion. This review article summarizes the literature and elaborates this entity including its specific features, risk factors associated with its causality, histopathology and related aspects of hemodynamics, and feeding in this vulnerable population. It also provides insight into modifiable risk factors and early markers of detection of gut necrosis to facilitate prevention and early detection. It highlights the subtle but definite difference in outcome variables to help physicians enable the parents of babies with heart disease to develop a better understanding of the entity and its expected course while counseling.
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Affiliation(s)
- Sriya Roychaudhuri
- Department of Pediatrics, Division of Neonatology, BC Women’s Hospital, Vancouver, Canada
| | - Gurpreet Grewal
- Department of Neonatal-Perinatal Medicine BC Women’s Hospital and Health Centre, British Columbia, Vancouver, Canada
| | | | - Pascal Lavoie
- Department of Neonatal-Perinatal Medicine BC Women’s Hospital and Health Centre, British Columbia, Vancouver, Canada
| | - Akhil Maheshwari
- Department of Neonatal-Perinatal Medicine, Global Newborn Society, Baltimore, Maryland, United States of America
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Mathis L, Shafer B, Crethers D, Polimenakos AC. Standardized Approach to Intervention for Intestinal Malrotation in Single Ventricle Patients with Heterotaxy Syndrome: Impact on Interstage Attrition and Time to Superior Cavopulmonary Connection. Pediatr Cardiol 2019; 40:1224-1230. [PMID: 31240371 DOI: 10.1007/s00246-019-02136-w] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/21/2019] [Accepted: 06/13/2019] [Indexed: 01/19/2023]
Abstract
Heterotaxy syndrome (HS) is a significant determinant of outcome in single ventricle (SV) physiology. Attrition rate and time-related events associated with intestinal malrotation (IM) are, yet, to be determined. We sought to evaluate hospital and interstage outcomes in relation with operative intervention for IM (IMO). Twelve SV/HS patients, who underwent IMO, from January 2004 to December 2016, were studied. Early shunt failure, time to superior cavopulmonary connection (SCPC) and interstage attrition were assessed. Since September 2014, based on a comprehensive standardized protocol, IMO was performed at the time of hospitalization for stage-I palliation (S1P) irrespective of clinical manifestations. Patients were assigned to Group A (n = 8): expectant /symptoms-driven versus Group B (n = 4): protocol-driven. At S1P 7 had systemic-to-pulmonary shunt (SPS), 1 SPS with anomalous pulmonary venous return (APVR) repair (Group A) compared to 2 SPS, 1 SPS with APVR repair and 1 Norwood operation (Group B). Median duration from S1P to IMO was 82 days (range 57-336; Group A) compared to 14 days (range 11-31; Group B); p < 0.05. Median age at IMO was 87 days (range 8-345) [Group A: 99 days (range 68-345) vs Group B: 25 days (range 8-39)] (p < 0.05). Early SPS failure occurred in 25% (2 of 8) for Group A compared to none in Group B (p < 0.05). Hospital mortality following IMO was 25% [Group A: 37.5% (3 of 8) vs Group B: 0; p < 0.05]. Interstage survival was 67% [Group A: 50% (4 of 8) vs Group B: 100%; p < 0.05]. Time to SCPC following S1P was 186 days (range 169-218) for Group A compared to 118 days (range 97-161) (Group B); p < 0.05. Operative intervention for IM in SV/HS is associated with significant interstage attrition and might impact the time to SCPC. SPS is at risk for early failure after IMO. A comprehensive standardized concept can mitigate detrimental implications.
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Affiliation(s)
- Lauren Mathis
- Division of Pediatric and Congenital Cardiothoracic Surgery, Children's Hospital of Georgia, 1120 15th Street BAA 8300, Augusta, GA, 30912, USA
| | - Brendan Shafer
- Division of Pediatric and Congenital Cardiothoracic Surgery, Children's Hospital of Georgia, 1120 15th Street BAA 8300, Augusta, GA, 30912, USA
| | | | - Anastasios C Polimenakos
- Division of Pediatric and Congenital Cardiothoracic Surgery, Children's Hospital of Georgia, 1120 15th Street BAA 8300, Augusta, GA, 30912, USA.
- Department of Surgery, Medical College of Georgia, Augusta, GA, USA.
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8
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Arcus C, Sennaiyan U, Trivedi A, Alahakoon TI. Antenatal ultrasound diagnosis of small bowel non-rotation in complex left isomerism: a case report. Int J Surg Case Rep 2019; 56:32-36. [PMID: 30822676 PMCID: PMC6393701 DOI: 10.1016/j.ijscr.2018.11.069] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/06/2018] [Revised: 11/26/2018] [Accepted: 11/30/2018] [Indexed: 11/18/2022] Open
Abstract
A rare case of mixed isomerism and antenatally diagnosed non-rotation of bowel is reported. Features of bowel non-rotation should be sought antenatally in cases of isomerism. Mixed isomerism and postnatal sequelae should be considered when right and left sided pathology coexist.z Presentation of case A multiparous expectant mother was referred to our tertiary unit at 23 weeks with a complex fetal cardiac anomaly in the context of suspected heterotaxy syndrome. The cardiac findings were consistent with isomerism: the fetal cardiac position was levocardia with a single functioning double outlet ventricle and AV valve, pulmonary stenosis, and interrupted inferior vena cava (IVC) with azygous continuation. The fetal abdominal situs was also altered, with the stomach to the right, and the hepatobiliary system midline to left. The spleen was not identified antenatally or postnatally. At 36 weeks, ultrasound revealed an abnormal bowel pattern with small bowel loops on the right side of the abdomen and large bowel on the left, suggesting a diagnosis of non- rotation. The infant was delivered vaginally at 39 weeks. The cardiac diagnosis and non-rotation of the small bowel were confirmed by postnatal echocardiography and contrast fluoroscopy. Discussion Heterotaxy syndrome is traditionally classified into right or left isomerism depending on how and where the organs are anatomically arranged. The case presented here demonstrates mixed laterality and prenatal ultrasound features of non-rotation. Conclusion It is important to be informed of the embryological variants of isomerism and actively seek antenatal evidence of bowel non-rotation in such cases.
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Affiliation(s)
- Charles Arcus
- Westmead Institute for Maternal and Fetal Medicine, Westmead Hospital, Sydney, NSW, 2145, Australia; The University of Sydney, Sydney Medical School, Sydney, NSW, Australia.
| | - Usha Sennaiyan
- Westmead Institute for Maternal and Fetal Medicine, Westmead Hospital, Sydney, NSW, 2145, Australia
| | - Amit Trivedi
- Grace Centre for Newborn Care, Westmead Children's Hospital, Sydney, NSW, 2145, Australia.
| | - Thushari I Alahakoon
- Westmead Institute for Maternal and Fetal Medicine, Westmead Hospital, Sydney, NSW, 2145, Australia; The University of Sydney, Sydney Medical School, Sydney, NSW, Australia.
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Abstract
More children with congenital heart disease are surviving and require noncardiac surgery. A high-yield summary of congenital heart anatomy and pathophysiology is presented to contextualize these patients for surgeons. Preoperative planning including risk stratification, anesthetic management and timing of elective surgery are discussed. Specific intraoperative considerations for particular cases, such as the use of laparoscopy and thoracic surgery, are reviewed. Postoperative outcomes and recommended management required to mitigate complications are summarized.
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Affiliation(s)
| | - J Craig Egan
- Department of Surgery, Phoenix Children's Hospital, Phoenix, AZ, USA
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Landisch RM, Loomba RS, Salazar JH, Buelow MW, Frommelt M, Anderson RH, Wagner AJ. Is isomerism a risk factor for intestinal volvulus? J Pediatr Surg 2018; 53:1118-1122. [PMID: 29605269 DOI: 10.1016/j.jpedsurg.2018.02.071] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/21/2018] [Accepted: 02/27/2018] [Indexed: 11/24/2022]
Abstract
INTRODUCTION Isomerism, or heterotaxy syndrome, affects many organ systems anatomically and functionally. Intestinal malrotation is common in patients with isomerism. Despite a low reported risk of volvulus, some physicians perform routine screening and prophylactic Ladd procedures on asymptomatic patients with isomerism who are found to have intestinal malrotation. The primary aim of this study was to determine if isomerism is an independent risk factor for volvulus. METHODS Kid's Inpatient Database data from 1997 to 2012 was utilized for this study. Characteristics of admissions with and without isomerism were compared with a particular focus on intestinal malrotation, volvulus, and Ladd procedure. A logistic regression was conducted to determine independent risk factors for volvulus with respect to isomerism. RESULTS 15,962,403 inpatient admissions were included in the analysis, of which 7970 (0.05%) patients had isomerism, and 6 patients (0.1%) developed volvulus. Isomerism was associated with a 52-fold increase in the odds of intestinal malrotation by univariate analysis. Of 251 with isomerism and intestinal malrotation, only 2.4% experienced volvulus. Logistic regression demonstrated that isomerism was not an independent risk factor for volvulus. CONCLUSION Isomerism is associated with an increased risk of intestinal malrotation but is not an independent risk factor for volvulus. TYPE OF STUDY Prognosis study. LEVEL OF EVIDENCE Level III.
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Affiliation(s)
- Rachel M Landisch
- Children's Hospital of Wisconsin/Medical College of Wisconsin, Division of Pediatric Surgery, Milwaukee, WI.
| | - Rohit S Loomba
- Children's Hospital of Wisconsin/Medical College of Wisconsin, Division of Pediatric Cardiology, Milwaukee, WI
| | - Jose H Salazar
- Children's Hospital of Wisconsin/Medical College of Wisconsin, Division of Pediatric Surgery, Milwaukee, WI
| | - Matthew W Buelow
- Children's Hospital of Wisconsin/Medical College of Wisconsin, Division of Pediatric Cardiology, Milwaukee, WI
| | - Michele Frommelt
- Children's Hospital of Wisconsin/Medical College of Wisconsin, Division of Pediatric Cardiology, Milwaukee, WI
| | - Robert H Anderson
- Institute of Genetics, Newcastle University, Division of Pediatric Cardiology, Newcastle Upon Tyne, United Kingdom
| | - Amy J Wagner
- Children's Hospital of Wisconsin/Medical College of Wisconsin, Division of Pediatric Surgery, Milwaukee, WI
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Putnam LR, Anderson KT, Tsao K, Kao LS, Lugo JA, Lally KP, Kawaguchi AL. The impact of cardiac risk factors on short-term outcomes for children undergoing a Ladd procedure. J Pediatr Surg 2017; 52:390-394. [PMID: 27894758 DOI: 10.1016/j.jpedsurg.2016.09.064] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/15/2016] [Revised: 09/29/2016] [Accepted: 09/29/2016] [Indexed: 10/20/2022]
Abstract
BACKGROUND/PURPOSE The purpose of this study was to describe the outcomes of children with and without congenital heart disease who undergo a Ladd procedure. METHODS The 2012-2014 National Surgical Quality Improvement Program Pediatric (NSQIP-P) data were queried for patients undergoing a Ladd procedure. Utilizing NSQIP-P definitions, patients were categorized into four cardiac risk groups (none, minor, major, severe) based on severity of cardiac anomalies, previous cardiac procedure(s), and ongoing cardiac dysfunction. Ladd procedures were elective/non-elective. Outcomes included length of stay, adverse events, and mortality. RESULTS 878 patients underwent Ladd procedures. 633 (72%) patients had no cardiac risk factors and 84 (10%), 109 (12%), and 52 (6%) had minor, major, and severe cardiac risk factors, respectively. Children with congenital heart disease experienced increased morbidity and mortality and longer hospital stays (all p<0.05). Elective Ladd procedures were associated with similar morbidity but shorter length of stay and lower mortality than non-elective procedures. Older age at time of operation was associated with fewer adverse events. CONCLUSIONS Although overall mortality remains low, children with higher risk cardiac disease experience increased morbidity and mortality when undergoing a Ladd procedure. Older age at the time of the Ladd procedure was associated with improved outcomes in children.
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Affiliation(s)
- Luke R Putnam
- Center for Surgical Trials and Evidence-based Practice, McGovern Medical School at The University of Texas Health Science Center at Houston, 6431 Fannin Street, MSB 5.248, Houston, TX 77030, USA; Department of Pediatric Surgery, McGovern Medical School at The University of Texas Health Science Center at Houston, 6431 Fannin Street, MSB 5.258, Houston, TX 77030, USA; Children's Memorial Hermann Hospital, 6411 Fannin Street, Houston, TX 77030, USA
| | - Kathryn T Anderson
- Center for Surgical Trials and Evidence-based Practice, McGovern Medical School at The University of Texas Health Science Center at Houston, 6431 Fannin Street, MSB 5.248, Houston, TX 77030, USA; Department of Pediatric Surgery, McGovern Medical School at The University of Texas Health Science Center at Houston, 6431 Fannin Street, MSB 5.258, Houston, TX 77030, USA; Children's Memorial Hermann Hospital, 6411 Fannin Street, Houston, TX 77030, USA
| | - KuoJen Tsao
- Center for Surgical Trials and Evidence-based Practice, McGovern Medical School at The University of Texas Health Science Center at Houston, 6431 Fannin Street, MSB 5.248, Houston, TX 77030, USA; Department of Pediatric Surgery, McGovern Medical School at The University of Texas Health Science Center at Houston, 6431 Fannin Street, MSB 5.258, Houston, TX 77030, USA; Children's Memorial Hermann Hospital, 6411 Fannin Street, Houston, TX 77030, USA
| | - Lillian S Kao
- Center for Surgical Trials and Evidence-based Practice, McGovern Medical School at The University of Texas Health Science Center at Houston, 6431 Fannin Street, MSB 5.248, Houston, TX 77030, USA; Department of General Surgery, McGovern Medical School at The University of Texas Health Science Center at Houston, 6431 Fannin Street, MSB 5.252, Houston, TX 77030, USA
| | - Jane A Lugo
- Children's Memorial Hermann Hospital, 6411 Fannin Street, Houston, TX 77030, USA
| | - Kevin P Lally
- Center for Surgical Trials and Evidence-based Practice, McGovern Medical School at The University of Texas Health Science Center at Houston, 6431 Fannin Street, MSB 5.248, Houston, TX 77030, USA; Department of Pediatric Surgery, McGovern Medical School at The University of Texas Health Science Center at Houston, 6431 Fannin Street, MSB 5.258, Houston, TX 77030, USA; Children's Memorial Hermann Hospital, 6411 Fannin Street, Houston, TX 77030, USA
| | - Akemi L Kawaguchi
- Center for Surgical Trials and Evidence-based Practice, McGovern Medical School at The University of Texas Health Science Center at Houston, 6431 Fannin Street, MSB 5.248, Houston, TX 77030, USA; Department of Pediatric Surgery, McGovern Medical School at The University of Texas Health Science Center at Houston, 6431 Fannin Street, MSB 5.258, Houston, TX 77030, USA; Children's Memorial Hermann Hospital, 6411 Fannin Street, Houston, TX 77030, USA.
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12
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Loomba RS, Nijhawan K, Anderson R. Impact of Era, Type of Isomerism, and Ventricular Morphology on Survival in Heterotaxy: Implications for Therapeutic Management. World J Pediatr Congenit Heart Surg 2016; 7:54-62. [PMID: 26714995 DOI: 10.1177/2150135115601831] [Citation(s) in RCA: 24] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/06/2023]
Abstract
BACKGROUND Heterotaxy has been demonstrated to reduce survival. There are several different subgroups of patients, however, and no single study has had a large number of patients and analyzed survival across the different subgroups such as patients born in different eras, patients with right and left isomerism, and patients with biventricular or functionally univentricular hearts. This study pools previously reported data from Kaplan-Meier curves and performs such subgroup analysis. METHODS A systematic review of the literature was performed to identify studies reporting survival of patients with the so-called "heterotaxy" by means of Kaplan-Meier survival curves. Data were extracted from these survival curves and then pooled together. A polynomial regression was then used to generate a pooled survival curve. This was done for all patients, those born in a more recent era, those with right and left isomerism, and those with biventricular or functionally univentricular hearts. RESULTS Those born in the more recent era (after 2000) had increased survival compared to the overall cohort. Those with left isomerism tended to have a survival benefit compared to those with right isomerism until about 16 years of age, beyond which those with right isomerism developed a survival benefit. Those with biventricular hearts had a survival benefit compared to those with left isomerism. CONCLUSION Survival in the so-called heterotaxy syndrome is based on several factors, which include era of birth, sidedness of isomerism, and whether the heart is biventricular or functionally univentricular.
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Affiliation(s)
- Rohit S Loomba
- Division of Cardiology, Children's Hospital of Wisconsin/Medical College of Wisconsin, Milwaukee, WI, USA
| | - Karan Nijhawan
- Department of Medicine, Rush University Medical Center, Chicago, IL, USA
| | - Robert Anderson
- Institute of Genetic Medicine, Newcastle University, United Kingdom
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Piggott KD, George G, Fakioglu H, Blanco C, Narasimhulu SS, Pourmoghadam K, Munroe H, Decampli W. Single institution experience with the Ladd’s procedure in patients with heterotaxy and stage I palliated single-ventricle. World J Clin Pediatr 2016; 5:319-324. [PMID: 27610349 PMCID: PMC4978626 DOI: 10.5409/wjcp.v5.i3.319] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/20/2016] [Revised: 04/01/2016] [Accepted: 06/03/2016] [Indexed: 02/06/2023] Open
Abstract
AIM: To investigate and describe our current institutional management protocol for single-ventricle patients who must undergo a Ladd’s procedure.
METHODS: We retrospectively reviewed the charts of all patients from January 2005 to March 2014 who were diagnosed with heterotaxy syndrome and an associated intestinal rotation anomaly who carried a cardiac diagnosis of functional single ventricle and were status post stage I palliation. A total of 8 patients with a history of stage I single-ventricle palliation underwent Ladd’s procedure during this time period. We reviewed each patients chart to determine if significant intraoperative or post-operative morbidity or mortality occurred. We also described our protocolized management of these patients in the cardiac intensive care unit, which included pre-operative labs, echocardiography, milrinone infusion, as well as protocolized fluid administration and anticoagulation regimines. We also reviewed the literature to determine the reported morbidity and mortality associated with the Ladd’s procedure in this particular cardiac physiology and if other institutions have reported protocolized care of these patients.
RESULTS: A total of 8 patients were identified to have heterotaxy with an intestinal rotation anomaly and single-ventricle heart disease that was status post single ventricle palliation. Six of these patients were palliated with a Blaylock-Taussig shunt, one of whom underwent a Norwood procedure. The two other patients were palliated with a stent, which was placed in the ductus arteriosus. These eight patients all underwent elective Ladd’s procedure at the time of gastrostomy tube placement. Per our protocol, all patients remained on aspirin prior to surgery and had no period where they were without anticoagulation. All patients remained on milrinone during and after the procedure and received fluid administration upon arrival to the cardiac intensive care unit to account for losses. All 8 patients experienced no intraoperative or post-operative complications. All patients survived to discharge. One patient presented to the emergency room two months after discharge in cardiac arrest and died due to bowel obstruction and perforation.
CONCLUSION: Protocolized intensive care management may have contributed to favorable outcomes following Ladd’s procedure at our institution.
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Tan YW, Khalil A, Kakade M, Carvalho JS, Bradley S, Cleeve S, Giuliani S. Screening and Treatment of Intestinal Rotational Abnormalities in Heterotaxy: A Systematic Review and Meta-Analysis. J Pediatr 2016; 171:153-62.e1-3. [PMID: 26868865 DOI: 10.1016/j.jpeds.2015.12.074] [Citation(s) in RCA: 18] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/02/2015] [Revised: 11/20/2015] [Accepted: 12/28/2015] [Indexed: 10/22/2022]
Abstract
OBJECTIVE To assess the role of screening and prophylactic surgery for intestinal rotational abnormalities (IRAs) in asymptomatic patients with heterotaxy. STUDY DESIGN PubMed, Embase, and Cinahl were searched electronically to determine the overall incidence of IRAs in heterotaxy; the detection rate of IRAs associated with screening; the incidence of midgut volvulus in patients without screening; and the incidence of morbidity and mortality after prophylactic and emergency Ladd procedures. Relevant data were computed with a meta-analysis of proportions. Between-study heterogeneity was assessed with the I(2) statistic. RESULTS From 276 papers identified, 24 studies with a total of 1433 patients with heterotaxy were included for systematic review. No randomized study was identified. True incidence of IRA in heterotaxy could not be ascertained through meta-analysis. In patients who underwent screening, the incidence of IRA was 58%. Acute midgut volvulus occurred in 5.8% of those who did not undergo screening. Postoperative mortality after Ladd procedure mainly was associated with cardiac insufficiency, and overall it was significantly greater in the emergency group compared with the prophylactic group (18% vs 5.6%). The complication rate also was greater in case of emergency vs prophylactic abdominal surgery (27% vs 16%); adhesional small bowel obstruction was the most common complication overall (6%). CONCLUSION The screen-detected incidences of IRA and acute midgut volvulus were significantly greater in heterotaxy than the normal population. Prophylactic Ladd procedure was associated with less morbidity and mortality compared with emergency surgery. A long-term prospective randomized trial is needed to define the indication for screening and prophylactic treatment of IRA in heterotaxy.
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Affiliation(s)
- Yew-Wei Tan
- Department of Pediatric and Neonatal Surgery, St. George's University Hospitals National Health Service Foundation Trust, London, United Kingdom
| | - Asma Khalil
- Fetal Medicine Unit, Division of Developmental Science, St George's University of London, London, United Kingdom
| | - Madhavi Kakade
- Department of Pediatric and Neonatal Surgery, King's College Hospital NHS Foundation Trust, London, United Kingdom
| | - Julene S Carvalho
- Fetal Medicine Unit, Division of Developmental Science, St George's University of London, London, United Kingdom; Department of Pediatric Cardiology, Royal Brompton and Harefield NHS Foundation Trust, London, United Kingdom
| | - Sarah Bradley
- Department of Pediatric and Neonatal Surgery, St. George's University Hospitals National Health Service Foundation Trust, London, United Kingdom
| | - Stewart Cleeve
- Department of Pediatric and Neonatal Surgery, Royal London Hospital, London, United Kingdom
| | - Stefano Giuliani
- Department of Pediatric and Neonatal Surgery, St. George's University Hospitals National Health Service Foundation Trust, London, United Kingdom.
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15
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Landisch R, Abdel-Hafeez AH, Massoumi R, Christensen M, Shillingford A, Wagner AJ. Observation versus prophylactic Ladd procedure for asymptomatic intestinal rotational abnormalities in heterotaxy syndrome: A systematic review. J Pediatr Surg 2015; 50:1971-4. [PMID: 26358665 DOI: 10.1016/j.jpedsurg.2015.08.002] [Citation(s) in RCA: 28] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/15/2015] [Revised: 07/27/2015] [Accepted: 08/02/2015] [Indexed: 11/28/2022]
Abstract
BACKGROUND The management of intestinal rotational abnormalities (IRA) in heterotaxy syndrome (HS) remains inconsistent. Because of the high incidence of malrotation in HS, screening of asymptomatic patients is standard of care in some institutions. The Ladd procedure is the treatment for malrotation, and has been reported to have high complication rates in HS patients. METHODS We performed a systematic review of publications describing IRA in pediatric HS patients from January 1993 to present. The incidence of volvulus on surgical exploration was determined. Perioperative and long-term outcomes were analyzed to determine complication and mortality rates. RESULTS Eleven retrospective studies describing 649 HS patients were identified. Of all patients with HS, 27% (176/649) underwent Ladd procedure. Only 1.2% (8/649) of HS patients included had volvulus. Postoperative complications occurred in 25 patients (14%), including a 10% incidence of small bowel obstruction. Perioperative and overall mortality rates after Ladd procedure were 3% and 21%, respectively. Six studies described mesenteric width, reporting 43% to have narrow mesentery. CONCLUSION The Ladd procedure is not without significant morbidity and mortality in heterotaxy patients. Further prospective studies should investigate predictors of mesenteric width to spare the unnecessary morbidity of surgery in patients who are at low risk for volvulus.
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Affiliation(s)
- Rachel Landisch
- Department of Surgery, Medical College of Wisconsin, Milwaukee, WI 53226.
| | | | - Roxanne Massoumi
- Division of Pediatric Surgery, Medical College of Wisconsin, Milwaukee, WI 53226.
| | - Melissa Christensen
- Division of Pediatric Surgery, Medical College of Wisconsin, Milwaukee, WI 53226.
| | - Amanda Shillingford
- Division of Pediatric Cardiology, Medical College of Wisconsin, Milwaukee, WI 53226.
| | - Amy J Wagner
- Division of Pediatric Surgery, Medical College of Wisconsin, Milwaukee, WI 53226.
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Graziano K, Islam S, Dasgupta R, Lopez ME, Austin M, Chen LE, Goldin A, Downard CD, Renaud E, Abdullah F. Asymptomatic malrotation: Diagnosis and surgical management: An American Pediatric Surgical Association outcomes and evidence based practice committee systematic review. J Pediatr Surg 2015. [PMID: 26205079 DOI: 10.1016/j.jpedsurg.2015.06.019] [Citation(s) in RCA: 74] [Impact Index Per Article: 7.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 10/23/2022]
Abstract
OBJECTIVE Patients with malrotation, or an intestinal rotation abnormality (IRA), can experience serious adverse events. Increasingly, asymptomatic patients are being diagnosed with malrotation incidentally. Patients with symptomatic malrotation require surgery in an urgent or semiurgent manner to address their symptoms. The treatment of asymptomatic or incidentally discovered malrotation remains controversial. METHODS Data were compiled from a broad search of Medline, Cochrane, Embase and Web of Science from January 1980 through January 2013 for five questions regarding asymptomatic malrotation. RESULTS There is minimal evidence to support screening asymptomatic patients. Consideration may be given to operate on asymptomatic patients who are younger in age, while observation may be appropriate in the older patient. If reliably diagnosed, atypical malrotation with a broad-based mesentery and malposition of the duodenum can be observed. Regarding diagnostic imaging, the standard of care for diagnosis remains the upper gastrointestinal contrast study (UGI), ultrasound may be useful for screening. A laparoscopic approach is safe for diagnosis and treatment of rotational abnormalities. Laparoscopy can aid in determining whether a patient has true malrotation with a narrow mesenteric stalk, has nonrotation and minimal risk for volvulus, or has atypical anatomy with malposition of the duodenum. It is reasonable to delay Ladd procedures until after palliation on patients with severe congenital heart disease. Observation can be considered with extensive education for family and caregivers and close clinical follow-up. CONCLUSIONS There is a lack of quality data to guide the management of patients with asymptomatic malrotation. Multicenter and prospective data should be collected to better assess the risk profile for this complex group of patients. A multidisciplinary approach involving surgery, cardiology, critical care and the patient's caregivers can help guide a watchful waiting management plan in individual cases.
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Affiliation(s)
- Kathleen Graziano
- Division of Pediatric General Surgery, Phoenix Children's Hospital, Phoenix, AZ.
| | - Saleem Islam
- Division of Pediatric Surgery, University of Florida, Gainesville, FL
| | - Roshni Dasgupta
- Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital, Cincinnati, OH
| | - Monica E Lopez
- Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX
| | - Mary Austin
- Department of Surgical Oncology, University of Texas M.D. Anderson Cancer Center; Department of Pediatric Surgery, University of Texas Medical School, Houston, TX
| | - Li Ern Chen
- Department of Surgery, Baylor University Medical Center, Dallas, TX
| | - Adam Goldin
- Division of Pediatric General and Thoracic Surgery, Seattle Children's Hospital; Department of Surgery, University of Washington, Seattle, WA
| | - Cynthia D Downard
- Division of Pediatric Surgery, Hiram C. Polk, Jr, MD; Department of Surgery, University of Louisville, Louisville, KY
| | - Elizabeth Renaud
- Division of Pediatric Surgery, Department of Surgery, Albany Medical Center, Albany, NY
| | - Fizan Abdullah
- Department of Surgery, Johns Hopkins University School of Medicine, Baltimore, MD
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Heterotaxy syndrome infants are at risk for early shunt failure after Ladd procedure. Ann Thorac Surg 2015; 99:918-25. [PMID: 25595831 DOI: 10.1016/j.athoracsur.2014.09.077] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/23/2014] [Revised: 09/19/2014] [Accepted: 09/23/2014] [Indexed: 02/06/2023]
Abstract
BACKGROUND Cardiac-specific risks and complications after a Ladd procedure in patients with heterotaxy syndrome (HS) and intestinal rotational anomalies (IRA) are unknown. We sought to (1) describe rates of hospital mortality and early systemic-to-pulmonary (S-P) artery shunt failure after the Ladd procedure in patients with HS and (2) explore risk factors associated with early shunt failure in patients with HS with single ventricle (SV). METHODS This retrospective study included all Ladd procedures performed from January 1999 to December 2012 in patients with HS at a single center. Risk factors investigated for early S-P artery shunt failure included birth weight, gestational age, sex, age at and timing of Ladd procedure relative to cardiac operations, and shunt type. RESULTS Ladd procedure was performed on 54 infants with HS and congenital heart disease. Hospital mortality for the entire cohort was 5.6% (3 of 54 patients). Early shunt failure occurred in 19% (4 of 21) of HS infants with SV. Mean preoperative blood urea nitrogen (BUN) levels were higher in HS infants with early shunt failure (20 versus 12.5 mg/dL; p = 0.054). CONCLUSIONS Patients with SV and HS with S-P artery shunts are at risk for early shunt failure after a Ladd procedure. A higher mean preoperative BUN level is noted in patients with HS and early shunt failure. Careful risk-benefit analysis is indicated before recommending routine elective Ladd procedures in patients with HS.
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18
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Sebastian VA, Brenes J, Murthy R, Veeram Reddy S, Dimas VV, Nugent A, Zellers T, Huang R, Guleserian KJ, Forbess JM. Management and outcomes of heterotaxy syndrome associated with pulmonary atresia or pulmonary stenosis. Ann Thorac Surg 2014; 98:159-66. [PMID: 24793683 DOI: 10.1016/j.athoracsur.2014.02.076] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/15/2013] [Revised: 02/13/2014] [Accepted: 02/20/2014] [Indexed: 11/28/2022]
Abstract
BACKGROUND Historic outcomes of patients with heterotaxy and pulmonary atresia or pulmonary stenosis (PA/PS) have been poor and in the current era are incompletely described. We reviewed our management of these patients and associated risk factors for death. METHODS We retrospectively reviewed the records of all patients with heterotaxy and PA/PS treated in our institution from January 1, 2002, to August 31, 2012. Death data were also confirmed with the Social Security Death Index. The log-rank test was done to assess six risk factors for death. RESULTS We identified 42 patients with heterotaxy and PA/PS. Median age at the first operation was 6.5 days, and median follow-up was 3.5 years. Death data were complete for all patients. Overall mortality was 19% (8 of 42). The 30-day, 1-year, and 5-year mortality estimation was 4.76%, 12.3%, and 19.1% respectively, as determined by the Kaplan-Meier method. The log-rank test showed total anomalous pulmonary venous return (TAPVR) (p<0.05) and obstructed TAPVR requiring an operation at less than 30 days (p=0.001) were significant risk factors for death. CONCLUSIONS In the current era, surgical treatment of heterotaxy and PA/PS can result in good outcomes. Associated TAPVR and obstructed TAPVR requiring neonatal correction were noted to be risk factors for death.
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Affiliation(s)
- Vinod A Sebastian
- Division of Pediatric Cardiothoracic Surgery, University of Texas Southwestern Medical Center, Dallas, Texas.
| | - Javier Brenes
- Division of Pediatric Cardiothoracic Surgery, University of Texas Southwestern Medical Center, Dallas, Texas
| | - Raghav Murthy
- Department of Cardiovascular and Thoracic Surgery, University of Texas Southwestern Medical Center, Dallas, Texas
| | - Surendranath Veeram Reddy
- Division of Pediatric Cardiology, University of Texas Southwestern Medical Center and Children's Medical Center, Dallas, Texas
| | - V Vivian Dimas
- Division of Pediatric Cardiology, University of Texas Southwestern Medical Center and Children's Medical Center, Dallas, Texas
| | - Alan Nugent
- Division of Pediatric Cardiology, University of Texas Southwestern Medical Center and Children's Medical Center, Dallas, Texas
| | - Thomas Zellers
- Division of Pediatric Cardiology, University of Texas Southwestern Medical Center and Children's Medical Center, Dallas, Texas
| | - Rong Huang
- Research Department, Children's Medical Center Dallas, Dallas, Texas
| | - Kristine J Guleserian
- Division of Pediatric Cardiothoracic Surgery, University of Texas Southwestern Medical Center, Dallas, Texas
| | - Joseph M Forbess
- Division of Pediatric Cardiothoracic Surgery, University of Texas Southwestern Medical Center, Dallas, Texas
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