Congenital, Hereditary, and Neonatal Diseases and Abnormalities Commons^™

Sequential Segmental Analysis., Robert H. Anderson, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

No abstract provided.

Design And Rationale Of A Randomized Trial Comparing The Blalock-Taussig And Right Ventricle-Pulmonary Artery Shunts In The Norwood Procedure., Richard G. Ohye, J William Gaynor, Nancy S. Ghanayem, Caren S. Goldberg, Peter C. Laussen, Peter C. Frommelt, Jane W. Newburger, Gail D. Pearson, Sarah Tabbutt, Gil Wernovsky, Lisa M. Wruck, Andrew M. Atz, Steve D. Colan, James Jaggers, Brian W. Mccrindle, Ashwin Prakash, Michael D. Puchalski, Lynn A. Sleeper, Mario P. Stylianou, Lynn Mahony, Pediatric Heart Network Investigators, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVE: The initial palliative procedure for patients born with hypoplastic left heart syndrome and related single right ventricle anomalies, the Norwood procedure, remains among the highest risk procedures in congenital heart surgery. The classic Norwood procedure provides pulmonary blood flow with a modified Blalock-Taussig shunt. Improved outcomes have been reported in a few small, nonrandomized studies of a modification of the Norwood procedure that uses a right ventricle-pulmonary artery shunt to provide pulmonary blood flow. Other nonrandomized studies have shown no differences between the two techniques.

METHODS: The Pediatric Heart Network designed a randomized clinical trial to compare outcomes for …

Functional Status, Heart Rate, And Rhythm Abnormalities In 521 Fontan Patients 6 To 18 Years Of Age., Andrew D. Blaufox, Lynn A. Sleeper, David J. Bradley, Roger E. Breitbart, Allan Hordof, Ronald J. Kanter, Elizabeth A. Stephenson, Mario Stylianou, Victoria L. Vetter, J Philip Saul, Pediatric Heart Network Investigators

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: Our objective was to determine the relationship between functional outcome and abnormalities of heart rate and rhythm after the Fontan operation.

METHODS: The National Heart, Lung, and Blood Institute Pediatric Heart Network conducted a cross-sectional analysis of patients who had undergone a Fontan procedure at the 7 network centers. Analysis was based on 521 patients with an electrocardiogram (n = 509) and/or bicycle exercise test (n = 404). The Child Health Questionnaire parent report and the oxygen consumption at the anaerobic threshold were used as markers of functional outcome.

RESULTS: Various Fontan procedures had been performed: intracardiac lateral tunnel …

Current Significance Of Meconium Plug Syndrome., Scott J. Keckler, Shawn D. St Peter, Troy L. Spilde, Kuojen Tsao, Daniel J. Ostlie, G W. Holcomb Iii, Charles L. Snyder

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: The significance of meconium plug syndrome is dependent on the underlying diagnosis. The incidence of pathologic finding, particularly Hirschsprung's disease, contributing to the presence of these plugs, has been debated. However, there are little recent data in the literature. Therefore, we reviewed our experience with meconium plugs as a cause of abdominal distension to evaluate the associated conditions and incidence of Hirschsprung's disease.

METHODS: We reviewed the records of newborns with meconium plugs found in the distal colon on contrast enema from 1994 to 2007. Demographics, radiologic findings, histologic findings, operative findings, and clinical courses were reviewed.

RESULTS: During …

Three Dimensional Echocardiography In Congenital Heart Defects., Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

Three dimensional echocardiography (3DE) is a new, rapidly evolving modality for cardiac imaging. Important technological advances have heralded an era where practical 3DE scanning is becoming a mainstream modality. We review the modes of 3DE that can be used. The literature has been reviewed for articles that examine the applicability of 3DE to congenital heart defects to visualize anatomy in a spectrum of defects ranging from atrioventricular septal defects to mitral valve abnormalities and Ebstein's anomaly. The use of 3DE color flow to obtain echocardiographic angiograms is illustrated. The state of the science in quantitating right and left ventricular volumetrics …

Survival After Bidirectional Cavopulmonary Anastomosis: Analysis Of Preoperative Risk Factors., Mark A. Scheurer, Elizabeth G Hill, Nagavardhan Vasuki, Scott Maurer, Eric M. Graham, Varsha Bandisode, Girish S. Shirali, Andrew M. Atz, Scott M. Bradley

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVE: Prognostic factors for survival after bidirectional cavopulmonary anastomosis for functionally single ventricle are not well defined. We analyzed preoperative hemodynamic and echocardiographic data to determine risk factors for death or transplantation at least 1 year after bidirectional cavopulmonary anastomosis.

METHODS: Data for all patients who underwent bidirectional cavopulmonary anastomosis before 5 years of age at our institution from September 1995 through June 2005 were analyzed. Available preoperative echocardiograms and catheterizations were reviewed. Survivors were compared with those who died or underwent transplantation. Bivariable associations between demographic and clinical risk factors and survival status (alive without transplantation vs dead or …

Incidence And Outcome Of Cardiopulmonary Resuscitation In Patients With Shunted Single Ventricle: Advantage Of Right Ventricle To Pulmonary Artery Shunt., Eric M. Graham, Geoffrey A. Forbus, Scott M. Bradley, Girish S. Shirali, Andrew M. Atz

Manuscripts, Articles, Book Chapters and Other Papers

No abstract provided.

Cardiac Transplant Following Failed Fontan Or Glenn Procedures., Naveen L. Pereira, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

No abstract provided.

Association Of Viral Genome With Graft Loss In Children After Cardiac Transplantation., Girish S. Shirali, J Ni, R E. Chinnock, J K. Johnston, G L. Rosenthal, N E. Bowles, J A. Towbin

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: The survival of recipients of cardiac allografts is limited by rejection, lymphoproliferative disease, and coronary vasculopathy. The purpose of this study in children who had received heart transplants was to evaluate the cardiac allografts for myocardial viral infections and to determine whether the presence of viral genome in the myocardium correlates with rejection, coronary vasculopathy, or graft loss.

METHODS: We enrolled heart-transplant recipients 1 day to 18 years old who were undergoing evaluation for possible rejection and coronary vasculopathy. Endomyocardial-biopsy specimens were evaluated for evidence of rejection with the use of standard criteria and were analyzed for the presence …

Recognition Of Left Atrial Aneurysm By Fetal Echocardiography., R R. Fountain-Dommer, H B. Wiles, C O. Shuler, S M. Bradley, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

No abstract provided.

Posttransplant Recoarctation Of The Aorta: A Twelve Year Experience., Girish S. Shirali, C E. Cephus, M A. Kuhn, K K. Ogata, L K. Vander Dussen, R E. Chinnock, N F. Mulla, J K. Johnston, L L. Bailey, S R. Gundry, A J. Razzouk, R L. Larsen

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: This study was undertaken to investigate the incidence of posttransplant recoarctation of the aorta, delineate the mode of presentation, identify risk factors that predict recoarctation and examine the results of intervention for posttransplant recoarctation.

BACKGROUND: Patients with aortic arch hypoplasia require extended arch reconstruction at transplant, with an inherent possibility of subsequent recoarctation of the aorta.

METHODS: This was a retrospective review of all children (ageyears) who underwent cardiac transplantation over a 10-year period. Collected data included pretransplant diagnosis, details of the transplant procedure and posttransplant data including development of recoarctation of the aorta, interventions for recoarctation and the …

Dobutamine Stress Echocardiography For Assessing Coronary Artery Disease After Transplantation In Children., R L. Larsen, P M. Applegate, D A. Dyar, P A. Ribeiro, S D. Fritzsche, N F. Mulla, Girish S. Shirali, M A. Kuhn, R E. Chinnock, P M. Shah

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: The purpose of this study was to determine the feasibility, safety and diagnostic accuracy of dobutamine stress echocardiography (DSE) for evaluating posttransplant coronary artery disease (TxCAD) in children, and to determine the frequency of selected cardiac events after normal or abnormal DSE.

BACKGROUND: Posttransplant coronary artery disease is the most common cause of graft loss (late death or retransplantation) after cardiac transplantation (CTx) in children. Coronary angiography, routinely performed to screen for TxCAD, is an invasive procedure with limited sensitivity. The efficacy of DSE for detecting atherosclerotic coronary artery disease is established, but is unknown in children after CTx. …

The Optimal Fontan Connection: A Growing Extracardiac Lateral Tunnel With Pedicled Pericardium., S R. Gundry, A J. Razzouk, M J. Del Rio, Girish S. Shirali, L L. Bailey

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVE: The concept of a lateral tunnel for the Fontan operation is now widely accepted. Most lateral tunnels are constructed intraatrially with the use of aortic crossclamping. Construction of extracardiac lateral tunnels with the use of homografts or other nonviable tubes eliminates aortic crossclamping but lacks growth potential in length or width. The native pericardium, which is "sealed" posteriorly along the pulmonary artery, atrium, and inferior vena cava, could be turned down onto the right atrium to form a viable extracardiac lateral tunnel.

METHODS: We designed and successfully constructed extracardiac lateral tunnels using viable autologous pericardium, pedicled on its lateral …

Ventricular Remodeling Following Infant-Pediatric Cardiac Transplantation. Does Age At Transplantation Or Size Disparity Matter?, Girish S. Shirali, F Lombano, W L. Beeson, D A. Dyar, N F. Mulla, A Khan, J K. Johnston, R E. Chinnock, S R. Gundry, A J. Razzouk

Manuscripts, Articles, Book Chapters and Other Papers

Early left ventricular (LV) remodeling following pediatric cardiac transplantation has not been described. To identify patterns and determinants of change in left ventricular mass and volume posttransplant, we studied 125 consecutive children who underwent cardiac transplantation between January 1, 1989 and July 31, 1993. Two-dimensional imaging-directed M-mode echocardiograms were studied weekly until 26 weeks post-transplant. LV mass and volume (indexed to BSA1.5) were measured. LV mass index increased until 3 weeks post-transplant, and then decreased. The mean decrement in LV mass index after 8 weeks post-transplant (relative to baseline) was significantly larger in patients with donor-recipient weight ratio > 1.5 compared …

Conjoined Twins - A Review Of Literature With Two Case Studies, Naaeem Uz-Zafar Khan, Nadeem Ullah Khan, Khalid Matin, Adnan Zaidi

Department of Emergency Medicine

Two cases of conjoined twins have been reported. A review of literature is given.

The Great Potato Debate, James D. Hudson

Honors Theses

Anencephaly and spina bifida cystica are two of the most common severe congenital malformations consistently observed in human populations. Both represent defects in the neural tube and share a significant number of similar epidemiological associations among them, occupational class, geography, sex ratio, maternal age, year and season of the year. In anencephaly most of the brain and upper skull never form usually resulting in death within a few hours of birth. Spina bifida cystica is a malformation of the spinal cord often causing paralysis, vulnerability to infection, and early death. In 1972, Dr. J.H. Renwick presented a controversial hypothesis which …