Published Research

Recent Publications by Faculty and Fellows

• Nellis ME, Tucci M, Lacroix J, Spinella PC, Haque KD, Stock A, Steiner ME, Faustino EVS, Zantek ND, Davis PJ, Stanworth SJ, Cholette JM, Parker RI, Demaret P, Kneyber MCJ, Russell RT, Stricker PA, Vogel AM, Willems A, Josephson CD, Luban NLC, Loftis LL, Leteurtre S, Stocker CF, Goobie SM, Karam O. Bleeding Assessment Scale in Critically Ill Children (BASIC): Physician-Driven Diagnostic Criteria for Bleeding Severity. Crit Care Med. 2019 Dec;47(12):1766-1772. Epub 2019 Sep 25.
• Richter F, Hoffman GE, Manheimer KB, Patel N, Sharp AJ, McKean D, Morton SU, DePalma S, Gorham J, Kitaygorodksy A, Porter GA, Giardini A, Shen Y, Chung WK, Seidman JG, Seidman CE, Schadt EE, Gelb BD. ORE identifies extreme expression effects enriched for rare variants. Bioinformatics. 2019 Oct 15;35(20):3906-3912. Epub 2019 Mar 23.
• Blumberg N, Cholette JM, Cahill C, Pietropaoli AP, Winters S, Phipps R, Noronha SA, Heal JM, Kirkley SA, Fountaine TJ, Henrichs K, Masel D, Refaai MA. Transfusion medicine: A research agenda for the coming years. Transfus Apher Sci. 2019 Oct;58(5):698-700. Epub 2019 Aug 06.
• Swartz MF, Makhija P, Rubenstein J, Henrichs KF, Powers KS, Wang HY, Simon BV, Alfieris GM, Blumberg N, Cholette JM. Met-Hemoglobin Is a Biomarker for Poor Oxygen Delivery in Infants Following Surgical Palliation. World J Pediatr Congenit Heart Surg. 2019 Jul;10(4):485-491. Epub 2019 May 29.
• Hansen RL, Naimi I, Wang H, Atallah N, Smith F, Byrum C, Kveselis D, Leonard G, Devanagondi R, Egan M. Long-term outcomes up to 25 years following balloon pulmonary valvuloplasty: A multicenter study. Congenit Heart Dis. Epub 2019 Jun 28.
• Pietropaoli AP, Henrichs KF, Cholette JM, Spinelli SL, Phipps RP, Refaai MA, Blumberg N. Total plasma heme concentration increases after red blood cell transfusion and predicts mortality in critically ill medical patients. Transfusion. 2019 Jun;59(6):2007-2015. Epub 2019 Feb 27.
• Ait-Aissa K, Blaszak SC, Beutner G, Tsaih SW, Morgan G, Santos JH, Flister MJ, Joyce DL, Camara AKS, Gutterman DD, Donato AJ, Porter GA, Beyer AM. Mitochondrial Oxidative Phosphorylation defect in the Heart of Subjects with Coronary Artery Disease. Sci Rep. 2019 May 20; 9(1):7623.
• Seidlmayer LK, Gomez-Garcia MR, Shiba T, Porter GA, Pavlov EV, Bers DM, Dedkova EN. Dual role of inorganic polyphosphate in cardiac myocytes: The importance of polyP chain length for energy metabolism and mPTP activation. Arch Biochem Biophys. 2019 Feb 15;662:177-189. Epub 2018 Dec 17.
• Lo CW, Liu X, Gabriel GC, Martin LJ, Porter GA, Benson DW. Reply to 'Double-outlet right ventricle is not hypoplastic left heart syndrome'. Nat Genet. 2019 Feb;51(2):198-199.
• Thammineni K, Vinocur JM, Harvey B, Menk JS, Kelleman MS, Korakiti AM, Thomas AS, Moller JH, St Louis JD, Kochilas LK. Outcomes after surgical coronary artery revascularisation in children with congenital heart disease. Heart. 2018 Sep; 104(17):1417-1423. Epub 2018 Feb 22.
• Doctor A, Cholette JM, Remy KE, Argent A, Carson JL, Valentine SL, Bateman ST, Lacroix J. Recommendations on RBC Transfusion in General Critically Ill Children Based on Hemoglobin and/or Physiologic Thresholds From the Pediatric Critical Care Transfusion and Anemia Expertise Initiative. Pediatr Crit Care Med. 2018 Sep; 19(9S Suppl 1):S98-S113.
• Cholette JM, Willems A, Valentine SL, Bateman ST, Schwartz SM. Recommendations on RBC Transfusion in Infants and Children With Acquired and Congenital Heart Disease From the Pediatric Critical Care Transfusion and Anemia Expertise Initiative. Pediatr Crit Care Med. 2018 Sep; 19(9S Suppl 1):S137-S148.
• Kuebler JD, Ghelani S, Williams DM, Nathan M, Marx G, Colan SD, Harrild DM. Normal Values and Growth-Related Changes of Left Ventricular Volumes, Stress, and Strain in Healthy Children Measured by 3-Dimensional Echocardiography. Am J Cardiol. 2018 Jul 15; 122(2):331-339. Epub 2018 Apr 24.
• Refaai MA, Conley GW, Henrichs KF, McRae H, Schmidt AE, Phipps RP, Spinelli SL, Masel D, Cholette JM, Pietropaoli A, Eaton MP, Blumberg N. Decreased Hemolysis and Improved Platelet Function in Blood Components Washed With Plasma-Lyte A Compared to 0.9% Sodium Chloride. Am J Clin Pathol. 2018 Jul 3; 150(2):146-153.
• Louis C, Swartz MF, Simon BV, Cholette JM, Atallah-Yunes N, Wang H, Gensini F, Alfieris GM. Modified Repair of Type I and II Truncus Arteriosus Limits Early Right Ventricular Outflow Tract Reoperation. Semin Thorac Cardiovasc Surg. 2018 Summer; 30(2):199-204. Epub 2018 Feb 08.
• Cholette JM, Noronha SA, Seghatchian J, Blumberg N. Indications for red cell transfusions in pediatric patients. Transfus Apher Sci. 2018 Jun; 57(3):342-346. Epub 2018 May 10.
• Spector LG, Menk JS, Knight JH, McCracken C, Thomas AS, Vinocur JM, Oster ME, St Louis JD, Moller JH, Kochilas L. Trends in Long-Term Mortality After Congenital Heart Surgery. J Am Coll Cardiol. 2018 May 29; 71(21):2434-2446.
• Yee M, Cohen ED, Domm W, Porter GA, McDavid A, O'Reilly MA. Neonatal hyperoxia depletes pulmonary vein cardiomyocytes in adult mice via mitochondrial oxidation. Am J Physiol Lung Cell Mol Physiol. 2018 May 1; 314(5):L846-L859. Epub 2018 Jan 18.
• Alanzalon RE, Burris JR, Vinocur JM. Brugada phenocopy associated with diabetic ketoacidosis in two pediatric patients. J Electrocardiol. 2018 Mar-Apr; 51(2):323-326. Epub 2017 Oct 28.
• Bounds RL, Kuebler JD, Cholette JM, Alfieris GM, Emani SM, Wittlieb-Weber CA. Left Main Coronary Artery Atresia in an Infant With Inclusion-Cell Disease." W J Pediatr Congenit Heart Surg. 2018 Mar; 9(2):246-250. Epub 2016 Sep 12.
• Roston TM, Yuchi Z, Kannankeril PJ, Hathaway J, Vinocur JM, Etheridge SP, Potts JE, Maginot KR, Salerno JC, Cohen MI, Hamilton RM, Pflaumer A, Mohammed S, Kimlicka L, Kanter RJ, LaPage MJ, Collins KK, Gebauer RA, Temple JD, Batra AS, Erickson C, Miszczak-Knecht M, Kubuš P, Bar-Cohen Y, Kantoch M, Thomas VC, Hessling G, Anderson C, Young ML, Choi SHJ, Cabrera Ortega M, Lau YR, Johnsrude CL, Fournier A, Van Petegem F, Sanatani S. The clinical and genetic spectrum of catecholaminergic polymorphic ventricular tachycardia: findings from an international multicentre registry. Europace. 2018 Mar; 20(3):541-547.
• Kuebler JD, Shivapour J, Yaroglu Kazanci S, Gauvreau K, Colan SD, McElhinney DB, Brown DW. Longitudinal Assessment of the Doppler-Estimated Maximum Gradient in Patients With Congenital Valvar Aortic Stenosis Pre- and Post-Balloon Valvuloplasty. Circ Cardiovasc Imaging. 2018 Mar; 11(3):e006708.
• Blumberg N, Cholette JM, Pietropaoli AP, Phipps R, Spinelli SL, Eaton MP, Noronha SA, Seghatchian J, Heal JM, Refaai MA. 0.9% NaCl (Normal Saline) - Perhaps not so normal after all? Transfus Apher Sci. 2018 Feb; 57(1):127-131. Epub 2018 Feb 21.
• Ghelani SJ, Brown DW, Kuebler JD, Perrin D, Shakti D, Williams DN, Marx GR, Colan SD, Geva T, Harrild DM. Left Atrial Volumes and Strain in Healthy Children Measured by Three-Dimensional Echocardiography: Normal Values and Maturational Changes. J Am Soc Echocardiogr. 2018 Feb; 31(2):187-193. Epub 2017 Dec 06.
• Schmidt AE, Gore E, Henrichs KF, Conley G, Dorsey C, Bjugstad KB, Refaai MA, Blumberg N, Cholette JM. Oxidation Reduction Potential (ORP) is Predictive of Complications Following Pediatric Cardiac Surgery. Pediatr Cardiol. 2018 Feb; 39(2):299:306. Epub 2017 Oct 31.
• St Louis JD, McCracken CE, Turk EM, Hancock HS, Menk JS, Harvey BA, Vinocur JM, Oster ME, Moller JH, Spector LG, Kochilas LK. Long-Term Transplant-Free Survival After Repair of Total Anomalous Pulmonary Venous Connection.  Ann Thorac Surg. 2018 Jan; 105(1):186-192. Epub 2017 Aug 25.
• Stolla M, Henrichs K, Cholette JM, Pietropaoli AP, Phipps RP, Spinelli SL, Blumberg N. Haem is associated with thrombosis in neonates and infants undergoing cardiac surgery for congenital heart disease. Vox Sang. 2018 Jan; 113(1):72-75. Epub 2017 Oct 16.
• Johnson JN, Barrett CS, Franklin WH, Graham EM, Halnon NJ, Hattendorf BA, Krawczeski CD, McGovern JJ, O'Connor MJ, Schultz AH, Vinocur JM, Chowdhury D, Anderson JB. Development of quality metrics for ambulatory pediatric cardiology: Infection prevention. Congenit Heart Dis. 2017 Dec; 12(6):756-761. Epub 2017 Jul 25.
• Beutner G, Alanzalon RE, Porter GA. Cyclophilin D regulates the dynamic assembly of mitochondrial ATP synthase into synthasomes. Sci Rep. 2017 Nov 3; 7(1):14488.
• Jin SC, Homsy J, Zaidi S, Lu Q, Morton S, DePalma SR, Zeng X, Qi H, Chang W, Sierant MC, Hung WC, Haider S, Zhang J, Knight J, Bjornson RD, Castaldi C, Tikhonoa IR, Bilguvar K, Mane SM, Sanders SJ, Mital S, Russell MW, Gaynor JW, Deanfield J, Giardini A, Porter GA, et al. Contribution of rare inherited and de novo variants in 2,871 congenital heart disease probands. Nature Genetics. 2017 Nov; 49(11):1593-1601. Epub 2017 Oct 09.
• Devanagondi R, Suntharos P, Boyle GJ, Prieto LR. Protein Losing Enteropathy After Cardiac Transplantation Successfully Treated by Stent Implantation. World J Pediatr Congenit Heart Surg. 2017 Nov; 8(6):754:757. Epub 2016 Aug 22.
• Blumberg N, Cholette JM, Schmidt AE, Phipps RP, Spinelli SL, Heal JM, Pietropaoli AP, Refaai MA, Sime PJ. Management of Platelet Disorders and Platelet Transfusions in ICU Patients. Transfus Med Rev. 2017 Oct;31(4):252-257. Epub 2017 May 04
• Patel N, Sollinger C, D'Angio CT, Vinocur JM, Ackerman KG, Katzman PJ. Congenital Diaphragmatic Hernia with Liver Herniation into the Pericardial Sac in a 30-Week Gestation Infant. Pediatr Dev Pathol. 2017 Sep-Oct;20(5):421-425. Epub 2017 Jan 25.
• Liu X, Yagi H, Saeed S, Bais AS, Gabriel GC, Chen Z, Peterson KA, Li Y, Schwartz MC, Reynolds WT, Saydmohammed M, Gibbs B, Wu Y, Devine W, Chatterjee B, Klena NT, Kostka D, de Mesy Bentley KL, Ganapathiraju MK, Dexheimer P, Leatherbury L, Khalifa O, Bhagat A, Zahid M, Pu W, Watkins S, Grossfeld P, Murray SA, Porter GA, Tsang M, Martin LJ, Woodrow Benson D, Aronow BJ, Lo CW. The complex genetics of hypoplastic left heart syndrome. Nat Genet. 2017 Jul; 49(7):1152-1159. Epub 2017 May 22.
• Beutner G, Porter GA. Analyzing Supercomplexes of the Mitochondrial Electron Transport Chain with Native Electrophoresis, In-gel Assays, and Electroelution. J Vis Exp. 2017 Jun 1;(124).
• Lingan JV, Alanzalon RE, Porter GA. Preventing permeability transition pore opening increases mitochondrial maturation, myocyte differentiation and cardiac function in the neonatal mouse heart. Pediatr Res. 2017 Jun; 81(6):932-941. Epub 2017 Jan 31.
• Smith JD, Vinocur JM. Atypical long QT syndrome phenotype in heterozygous/homozygous KCNQ1 Ala590Thr. HeartRhythm Case Rep. 2017 Apr; 3(4):219-223. Epub 2017 Jan 27.
• Kaza AK, Wamala I, Friehs I, Kuebler JD, Rathod RH, Berra I, Ericsson M, Yao R, Thedsanamoorthy JK, Zurakowski D, Levitsky S, Del Nido PJ, Cowan DB, McCully JD. Myocardial rescue with autologous mitochondrial transplantation in a porcine model of ischemia/reperfusion. J Thorac Cardiovasc Surg. 2017 Apr; 153(4):934-943. Epub 2016 Nov 15.
• Devanagondi R, Peck D, Sagi J, Donohue J, Yu S, Pasquali SK, Armstrong AK. Long-Term Outcomes of Balloon Valvuloplasty for Isolated Pulmonary Valve Stenosis. Pediatr Cardiol. 2017 Feb; 38(2):247-254. Epub 2016 Nov 08.
• Mnatsakanyan N, Beutner G, Porter GA, Alavian KN, Jonas EA. Physiological roles of the mitochondrial permeability transition pore. J Bioenerg Biomembr. 2017 Feb; 49(1):13-25; Epub 2016 Feb 11.
• Roche ET, Horvath MA, Wamala I, Alazmani A, Song SE, Whyte W, Machaidze Z, Payne CJ, Weaver JC, Fishbein G, Kuebler J, Vasilyev NV, Mooney DJ, Pigula FA, Walsh CJ. Soft robotic sleeve supports heart function. Sci Transl Med. 2017 Jan 18; 9(373).
• Simon BV, Swartz MF, Egan M, Cholette JM, Gensini F, Alfieris GM. Use of a Dacron Annular Sparing Versus Limited Transannular Patch With Nominal Pulmonary Annular Expansion in Infants With Tetralogy of Fallot. Ann Thorac Surg. 2017 Jan;103(1):186-192. Epub 2016 Jul 29.
• Cholette JM, Swartz MF, Rubenstein J, Henrichs KF, Wang H, Powers KS, Daugherty LE, Alfieris GM, Gensini F, Blumberg N. Outcomes Using a Conservative Versus Liberal Red Blood Cell Transfusion Strategy in Infants Requiring Cardiac Operation. Ann Thorac Surg. 2017 Jan;103(1):206-214. Epub 2016 Aug 03.
• Muszynski JA, Spinella PC, Cholette JM, Acker JP, Hall MW, Juffermans NP, Kelly DP, Blumberg N, Nicol K, Liedel J, Doctor A, Remy KE, Tucci M, Lacroix J, Norris PJ. Transfusion-related immunomodulation: review of the literature and implications for pediatric critical illness. Transfusion. 2017 Jan;57(1):195-206. Epub 2016 Oct 2.
• Schubmehl HB, Swartz MF, Atallah-Yunes N, Wittlieb-Weber C, Pratt RE, Alfieris GM. Sustained Improvement in Right Ventricular Chamber Dimensions 10 Years Following Xenograft Pulmonary Valve Replacement. World J Pediatr Congenit Heart Surg. 2017 Jan;8(1):39-47.
• Beutner G, Kambiz AN, Jonas EA, Porter GA. The Mitochondrial Permeability Transition Pore and ATP Synthase. Handb Exp Pharmacol. 2017 240:21-46.
• Parzen JS, Swartz MF, Cholette JM, Smith F, Gensini F, Alfieris GM. Continuous Cardiopulmonary Bypass During the Repair of Total Anomalous Pulmonary Venous Return. World J Pediatr Congenit Heart Surg. 2016 Nov;7(6):750-752.
• Spector LG, Menk JS, Vinocur JM, Oster ME, Harvey BA, St Louis JD, Moller J, Kochilas LK. In-Hospital Vital Status and Heart Transplants After Intervention for Congenital Heart Disease in the Pediatric Cardiac Care Consortium: Completeness of Ascertainment Using the National Death Index and United Network for Organ Sharing Datasets. J Am Heart Assoc. 2016 Aug 9;5(8).
• DeMichele JC, Vajaria N, Wang H, Sweeney DM, Powers KS, Cholette JM. Cuffed endotracheal tubes in neonates and infants undergoing cardiac surgery are not associated with airway complications. J Clin Anesth. 2016 Sep; 33:422-7. Epub 2016 Jun 04.
• Devanagondi R, Latson L, Bradley-Skelton S, Prieto L. Results of coil closure of patent ductus arteriosus using a tapered tip catheter for enhanced control. Catheter Cardiovasc Interv. 2016 Aug; 88(2):233-8. Epub 2016 Jan 23.
• Al-Haddad BJ, Menk JS, Kochilas L, Vinocur JM. Factors Affecting Length of Postoperative Hospitalization for Pediatric Cardiac Operations in a Large North American Registry (1982-2007). Pediatr Cardiol. 2016 Jun; 37(5):884-91. Epub 2016 Mar 10.
• Joynt MR, Lu JC, Bocks ML, Crowley DC. Ruptured aneurysm of a major aortopulmonary collateral. Eur Heart J. 2016 Jun 7; 37(22):1777. 2016 Jun 7; 37(22):1777. Epub 2015 Jul 18.
• Seltzer L, Swartz MF, Kwon J, Burchfiel J, Cholette JM, Wang H, Sweeney D, Adams HR, Meagher C, Angona R, Guillet R, Alfieris GM. Neurodevelopmental outcomes after neonatal cardiac surgery: Role of cortical isoelectric activity. J Thorac Cardiovasc Surg. 2016 Apr; 151(4):1137-42. Epub 2015 Oct 27.
• Patel N, Sollinger C, D'Angio CT, Vinocur JM, Ackerman KG, Katzman PJ. Case Report: Congenital diaphragmatic hernia with liver herniation into the pericardium in a 30-week gestation infant. Pediatr Dev Pathol. 2016 Mar 28; Epub 2016 Mar 28.
• Kuebler JD, Chen MH, Alexander ME, Rhodes J. Exercise Performance in Patients with D-Loop Transposition of the Great Arteries After Arterial Switch Operation: Long-Term Outcomes and Longitudinal Assessment. Pediatr Cardiol. 2016 Feb; 37(2):283-9. Epub 2015 Oct 06.
• Al-Haddad BJ, Menk JS, Kochilas L, Vinocur JM. Factors Affecting Length of Postoperative Hospitalization for Pediatric Cardiac Operations in a Large North American Registry (1982-2007). Pediatr Cardiol. Epub 2016 Mar 10.
• Maruyama EO, Lin H, Chiu SY, Yu HM, Porter GA, Hsu W. Extraembryonic but not embryonic SUMO-specific protease 2 is required for heart development. Sci Rep. 2016 6:20999. Epub 2016 Feb 17.
• Joynt MR, Yu S, Dorfman AL, Ghadimi Mahani M, Agarwal PP, Lu JC. Differential Impact of Pulmonary Regurgitation on Patients With Surgically Repaired Pulmonary Stenosis Versus Tetralogy of Fallot. Am J Cardiol. 2016 Jan 15; 117(2):289-94. Epub 2015 Nov 05.
• Yang YP, Lotta L, Beutner G, Li X, Schor NF. Induction of Expression of p75 Neurotrophin Receptor Intracellular Domain Does Not Induce Expression or Enhance Activity of Mitochondrial Complex II. Oxid Med Cell Longev. 2016 2016:8752821. Epub 2015 Nov 10.
• Wittlieb-Weber CA, Haude KM, Fong CT, Vinocur JM. A novel GJA1 mutation causing familial oculodentodigital dysplasia with dilated cardiomyopathy and arrhythmia. HeartRhythm Case Reports. 2016. Vol 2: 32-35. Epub 2015 Oct 9.