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1. Survival After Heart Transplant Listing for Infants on Mechanical Circulatory Support

Author

Jennifer Conway, Ryan Cantor, Devin Koehl, Robert Spicer, Dipankar Gupta, Michael McCulloch, Alfred Asante‐Korang, Dean T. Eulrich, James K. Kirklin, and Elfriede Pahl

Subjects
congenital heart disease, extracorporeal membrane oxygenation, mortality, pediatric, transplantation, ventricular assist device, Diseases of the circulatory (Cardiovascular) system, RC666-701
Abstract

Background Infants with heart failure remain at significant risk for wait list mortality, despite mechanical circulatory support (MCS). It is unclear if the outcomes are influenced by modality of support or underlying diagnosis. We sought to compare the outcomes of infants

Published
2020
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2. Cardiac electrical system involvement in Alström syndrome: uncommon causes of dilated cardiomyopathies

Author

Richard J. Czosek, Paula Goldenberg, Erin M. Miller, Robert Spicer, Jeffrey A. Towbin, and Stephanie M. Ware

Subjects
Alström syndrome, arrhythmia, cardiomyopathy., Diseases of the circulatory (Cardiovascular) system, RC666-701
Abstract

Alström syndrome is a rare autosomal recessive disorder with dilated cardiomyopathy in 60% of patients. Despite the frequency of cardiac involvement in Alström syndrome, conduction system abnormalities or arrhythmias have not been characterized previously. We report two siblings with Alström syndrome with conduction system involvement with left bundle branch block on electrocardiogram (ECG). One patient had first degree atrioventricular block in addition to bundle branch block and underwent pacemaker implantation. This same patient developed intra-atrial reentry tachycardia requiring anti-arrhythmic medication and eventual trans-catheter ablation. The second patient developed atrial and ventricular arrhythmias and underwent placement of a bi-ventricular defibrillator. These findings suggest that cardiac conduction system involvement and clinical arrhythmia may be significant yet under-recognized complications in patients with Alström syndrome. Patients should be routinely screened with ECG and Holter monitoring in addition to echocardiographic assessment and a cardiologist experienced with cardiomyopathy should be an integral part of the care team.

Published
2012
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4. Late Eocene-early Oligocene paleoenvironmental changes recorded at Lühe, Yunnan, southwestern China

Author

Vittoria Lauretano, Caitlyn Witkowski, Alex Farnsworth, Shufeng Li, Shihu Li, Jan Peter Mayser, B. David A. Naafs, Robert Spicer, Tao Su, He Tang, Zhekun Zhou, Paul Valdes, and Richard Pancost

Abstract

During the late Eocene to the early Oligocene, marine records document a globally congruent record of declining carbon dioxide concentrations, Antarctic icesheet growth, and associated reorganisation of the global climate system. In contrast, the few existing terrestrial records demonstrate high heterogeneity of environmental change and are difficult to reconcile with those of the oceanic realm. Global drivers for climatic change are particularly difficult to disentangle from regional ones, especially those caused by the complex tectonic evolution of the Tibetan region and its influence on the Asian monsoon system and vegetation. Here, we reconstruct the climatic and environmental history from the late Eocene into the early Oligocene at Lühe Basin, Yunnan, China, a key sedimentary repository along the SE margin of the Tibetan Plateau and an important region for assessing Asian monsoon changes. We investigate a 340-m long section via a multi-proxy approach and climate model simulations. The organic geochemical proxies, via n-alkanes, terpenoids, and hopanes, suggest that thermally immature sediments were deposited in a terrestrial flood plain basin that was primarily occupied by gymnosperms and angiosperms. Branched glycerol diakyl glycerol tetraethers indicate relatively stable temperatures (ca. 10 °C) throughout the section, including across the Eocene-Oligocene boundary. This temperature, cooler than the modern-day average for this site (ca. 15 °C), suggests that this area has not undergone significant uplift since the Oligocene. To further contextualize our data, we tested a suite of climate model simulations with varying pCO2, paleogeography, and Tibetan topography across the Eocene-Oligocene boundary. This data-model comparison suggests that a response to regional factors might explain the absence of a pronounced cooling at Lühe across the Eocene-Oligocene boundary, supporting the emerging picture that the global expression of the EOT in terrestrial environments is more complex than indicated by the marine record.

Published
2021
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5. Modelling the Interaction between Tibet and Climate and Biosphere during the Cenozoic

Author

Paul Valdes, Alex Farnsworth, Tao Su, Robert Spicer, Lin Ding, Shufeng Li, Zhekun Zhou, and Shihu Li

Abstract

The Cenozoic uplift history of Tibet and its impact on the Asian monsoon and vegetation is complex. The building of the Tibetan Plateau is not a simple story of the rise of a single geological entity driven by the relentless northward passage of India as depicted in numerous modelling exercises, but was a complex process involving a succession of collisions of several Gondwanan terranes with Asia. The talk will review our current understanding of the uplift history of Tibet and show new climate model simulations of how Tibet has influenced climate, vegetation and biodiversity in the region. We make use of isotope-enabled Earth System models, as well as high resolution models to show that the complex history of Tibet has important consequences for understanding the evolution of both the summer and winter Asian monsoon. We show that post-Oligocene growth of north and north-eastern Tibet is crucial for the evolution of vegetation and biodiversity in the region by altering the strength of the winter monsoon system over Asia.

Published
2020
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6. Contributors

Author

Jon S. Abramson, Mark J. Abzug, John J. Aiken, H. Hesham A-kader, Cezmi A. Akdis, Harold Alderman, Ramin Alemzadeh, Evaline A. Alessandrini, Omar Ali, Namasivayam Ambalavanan, Karl E. Anderson, Peter M. Anderson, Kelly K. Anthony, Alia Y. Antoon, Stacy P. Ardoin, Carola A.S. Arndt, Stephen S. Arnon, Stephen C. Aronoff, David M. Asher, Barbara L. Asselin, Joann L. Ater, Dan Atkins, Erika F. Augustine, Marilyn Augustyn, Ellis D. Avner, Parvin H. Azimi, Carlos A. Bacino, Robert N. Baldassano, Christina Bales, William F. Balistreri, Robert S. Baltimore, Manisha Balwani, Shahida Baqar, Christine E. Barron, Dorsey M. Bass, Mark L. Batshaw, Richard E. Behrman, Michael J. Bell, John W. Belmont, Daniel K. Benjamin, Michael J. Bennett, Daniel Bernstein, Jatinder Bhatia, Zulfiqar Ahmed Bhutta, Leslie G. Biesecker, James Birmingham, Samra S. Blanchard, Ronald Blanton, Archie Bleyer, C.D.R. Lynelle M. Boamah, Steven R. Boas, Thomas F. Boat, Walter Bockting, Mark Boguniewicz, Daniel J. Bonthius, Laurence A. Boxer, Amanda M. Brandow, David Branski, David T. Breault, Rebecca H. Buckley, Cynthia Etzler Budek, E. Stephen Buescher, Gale R. Burstein, Amaya Lopez Bustinduy, Mitchell S. Cairo, Bruce M. Camitta, Angela Jean Peck Campbell, Rebecca G. Carey, Waldemar A. Carlo, Robert B. Carrigan, Mary T. Caserta, Ellen Gould Chadwick, Lisa J. Chamberlain, Jennifer I. Chapman, Ira M. Cheifetz, Wassim Chemaitilly, Sharon F. Chen, Yuan-Tsong Chen, Russell W. Chesney, Jennifer A. Chiriboga, Robert D. Christensen, Andrew Chu, Michael J. Chusid, Theodore J. Cieslak, Jeff A. Clark, Thomas G. Cleary, John David Clemens, Joanna S. Cohen, Mitchell B. Cohen, Pinchas Cohen, Michael Cohen-Wolkowiez, Robert A. Colbert, F. Sessions Cole, Joanna C.M. Cole, John L. Colombo, Amber R. Cooper, Ronina A. Covar, Barbara Cromer, James E. Crowe, Natoshia Raishevich Cunningham, Steven J. Czinn, Toni Darville, Robert S. Daum, Richard S. Davidson, H. Dele Davies, Peter S. Dayan, Michael R. DeBaun, Guenet H. Degaffe, David R. DeMaso, Mark R. Denison, Arlene E. Dent, Nirupama K. DeSilva, Robert J. Desnick, Gabrielle deVeber, Esi Morgan DeWitt, Chetan Anil Dhamne, Anil Dhawan, Harry Dietz, Lydia J. Donoghue, Patricia A. Donohoue, Mary K. Donovan, John P. Dormans, Daniel A. Doyle, Jefferson Doyle, Stephen C. Dreskin, Denis S. Drummond, Howard Dubowitz, J. Stephen Dumler, Janet Duncan, Paula M. Duncan, LauraLe Dyner, Michael G. Earing, Elizabeth A. Edgerton, Marie Egan, Jack S. Elder, Sara B. Eleoff, Dianne S. Elfenbein, Stephen C. Eppes, Michele Burns Ewald, Jessica K. Fairley, Susan Feigelman, Marianne E. Felice, Eric I. Felner, Edward Fels, Thomas Ferkol, Jonathan D. Finder, Kristin N. Fiorino, David M. Fleece, Patricia M. Flynn, Joel A. Forman, Michael M. Frank, Melvin H. Freedman, Melissa Frei-Jones, Jared E. Friedman, Sheila Gahagan, Paula Gardiner, Luigi Garibaldi, Gregory M. Gauthier, Abraham Gedalia, Matthew J. Gelmini, Michael A. Gerber, K. Michael Gibson, Mark Gibson, Francis Gigliotti, Walter S. Gilliam, Janet R. Gilsdorf, Charles M. Ginsburg, Frances P. Glascoe, Donald A. Goldmann, Denise M. Goodman, Marc H. Gorelick, Gary J. Gosselin, Jane M. Gould, Olivier Goulet, Dan M. Granoff, Michael Green, Thomas P. Green, Larry A. Greenbaum, Marie Michelle Grino, Andrew B. Grossman, David C. Grossman, Alfredo Guarino, Lisa R. Hackney, Gabriel G. Haddad, Joseph Haddad, Joseph F. Hagan, Scott B. Halstead, Margaret R. Hammerschlag, Aaron Hamvas, James C. Harris, Mary E. Hartman, David B. Haslam, Fern R. Hauck, Gregory F. Hayden, Jacqueline T. Hecht, Sabrina M. Heidemann, J. Owen Hendley, Fred M. Henretig, Gloria P. Heresi, Andrew D. Hershey, Cynthia E. Herzog, Jessica Hochberg, Lauren D. Holinger, Jeffrey D. Hord, B. David Horn, William A. Horton, Harish S. Hosalkar, Hidekazu Hosono, Peter J. Hotez, Michelle S. Howenstine, Heather G. Huddleston, Vicki Huff, Denise Hug, Winston W. Huh, Carl E. Hunt, Anna Klaudia Hunter, Patricia Ibeziako, Richard F. Jacobs, Peter Jensen, Hal B. Jenson, Chandy C. John, Michael V. Johnston, Richard B. Johnston, Bridgette L. Jones, James F. Jones, Marsha Joselow, Anupama Kalaskar, Linda Kaljee, Deepak Kamat, Alvina R. Kansra, Sheldon L. Kaplan, Emily R. Katz, James W. Kazura, Virginia Keane, Gregory L. Kearns, Desmond P. Kelly, Judith Kelsen, Kathi J. Kemper, Melissa Kennedy, Eitan Kerem, Joseph E. Kerschner, Seema Khan, Young-Jee Kim, Charles H. King, Stephen L. Kinsman, Adam Kirton, Priya S. Kishnani, Nora T. Kizer, Martin B. Kleiman, Bruce L. Klein, Bruce S. Klein, Michael D. Klein, Robert M. Kliegman, William C. Koch, Patrick M. Kochanek, Eric Kodish, Stephan A. Kohlhoff, Elliot J. Krane, Peter J. Krause, Richard E. Kreipe, Steven E. Krug, John F. Kuttesch, Jennifer M. Kwon, Catherine S. Lachenauer, Stephan Ladisch, Stephen LaFranchi, Oren Lakser, Marc B. Lande, Philip J. Landrigan, Gregory L. Landry, Wendy G. Lane, Philip S. LaRussa, Brendan Lee, Chul Lee, K. Jane Lee, J. Steven Leeder, Rebecca K. Lehman, Michael J. Lentze, Norma B. Lerner, Steven Lestrud, Donald Y.M. Leung, Chris A. Liacouras, Susanne Liewer, Andrew H. Liu, Stanley F. Lo, Franco Locatelli, Sarah S. Long, Anna Lena Lopez, Steven V. Lossef, Jennifer A. Lowry, Kerith Lucco, G. Reid Lyon, Prashant V. Mahajan, Akhil Maheshwari, Joseph A. Majzoub, Asim Maqbool, Ashley M. Maranich, Mona Marin, Joan C. Marini, Morri Markowitz, Kevin P. Marks, Stacene R. Maroushek, Wilbert H. Mason, Christopher Mastropietro, Kimberlee M. Matalon, Reuben K. Matalon, Robert Mazor, Susanna A. McColley, Margaret M. McGovern, Heather S. McLean, Rima McLeod, Peter C. Melby, Joseph John Melvin, Diane F. Merritt, Ethan A. Mezoff, Marian G. Michaels, Alexander G. Miethke, Mohamad A. Mikati, Henry Milgrom, E. Kathryn Miller, Jonathan W. Mink, Grant A. Mitchell, Robert R. Montgomery, Joseph G. Morelli, Anna-Barbara Moscicki, Hugo W. Moser, Kathryn D. Moyer, James R. Murphy, Timothy F. Murphy, Thomas S. Murray, Mindo J. Natale, William A. Neal, Jayne Ness, Kathleen A. Neville, Mary A. Nevin, Jane W. Newburger, Peter E. Newburger, Linda S. Nield, Zehava Noah, Lawrence M. Nogee, Robert L. Norris, Stephen K. Obaro, Makram Obeid, Theresa J. Ochoa, Katherine A. O'Donnell, Robin K. Ohls, Jean-Marie Okwo-Bele, Keith T. Oldham, Scott E. Olitsky, John Olsson, Susan R. Orenstein, Walter A. Orenstein, Judith A. Owens, Charles H. Packman, Michael J. Painter, Priya Pais, Cynthia G. Pan, Vijay Pannikar, Diane E. Pappas, Anjali Parish, John S. Parks, Laura A. Parks, Maria Jevitz Patterson, Pallavi P. Patwari, Timothy R. Peters, Larry K. Pickering, Misha L. Pless, Laura S. Plummer, Craig C. Porter, Dwight A. Powell, David T. Price, Charles G. Prober, Linda Quan, Elisabeth H. Quint, C. Egla Rabinovich, Leslie J. Raffini, Denia Ramirez-Montealegre, Giuseppe Raviola, Ann M. Reed, Harold L. Rekate, Megan E. Reller, Gary Remafedi, Jorge D. Reyes, Geoffrey Rezvani, Iraj Rezvani, A. Kim Ritchey, Frederick P. Rivara, Angela Byun Robinson, Luise E. Rogg, Genie E. Roosevelt, David R. Rosenberg, Melissa Beth Rosenberg, David S. Rosenblatt, Cindy Ganis Roskind, Mary M. Rotar, Ranna A. Rozenfeld, Sarah Zieber Rush, Colleen A. Ryan, H.P.S. Sachdev, Ramesh C. Sachdeva, Mustafa Sahin, Robert A. Salata, Denise A. Salerno, Edsel Maurice T. Salvana, Hugh A. Sampson, Thomas J. Sandora, Tracy Sandritter, Wudbhav N. Sankar, Ajit Ashok Sarnaik, Ashok P. Sarnaik, Harvey B. Sarnat, Minnie M. Sarwal, Mary Saunders, Laura E. Schanberg, Mark R. Schleiss, Nina F. Schor, Bill J. Schroeder, Robert L. Schum, Gordon E. Schutze, Daryl A. Scott, J. Paul Scott, Theodore C. Sectish, George B. Segel, Kriti Sehgal, Ernest G. Seidman, Janet R. Serwint, Dheeraj Shah, Raanan Shamir, Bruce K. Shapiro, Richard J. Shaw, Bennett A. Shaywitz, Sally E. Shaywitz, Meera Shekar, Elena Shephard, Philip M. Sherman, Benjamin L. Shneider, Scott H. Sicherer, Richard Sills, Mark D. Simms, Eric A.F. Simões, Thomas L. Slovis, P. Brian Smith, Mary Beth F. Son, Laura Stout Sosinsky, Joseph D. Spahn, Mark A. Sperling, Robert Spicer, David A. Spiegel, Helen Spoudeas, Jürgen Spranger, Rajasree Sreedharan, Raman Sreedharan, Shawn J. Stafford, Margaret M. Stager, Sergio Stagno, Virginia A. Stallings, Lawrence R. Stanberry, Charles A. Stanley, Bonita F. Stanton, Jeffrey R. Starke, Merrill Stass-Isern, Barbara W. Stechenberg, Leonard D. Stein, William J. Steinbach, Nicolas Stettler, Barbara J. Stoll, Gregory A. Storch, Ronald G. Strauss, Frederick J. Suchy, Karen Summar, Moira Szilagyi, Norman Tinanoff, James K. Todd, Lucy S. Tompkins, Richard L. Tower, Riccardo Troncone, Amanda A. Trott, David G. Tubergen, David A. Turner, Ronald B. Turner, Christina Ullrich, George F. Van Hare, Jakko van Ingen, Heather A. Van Mater, Dick van Soolingen, Scott K. Van Why, Pankhuree Vandana, Douglas Vanderbilt, Jon A. Vanderhoof, Andrea Velardi, Elliott Vichinsky, Linda A. Waggoner-Fountain, Steven G. Waguespack, David M. Walker, Heather J. Walter, Stephanie Ware, Kimberly Danieli Watts, Ian M. Waxman, Debra E. Weese-Mayer, Kathryn Weise, Martin E. Weisse, Lawrence Wells, Jessica Wen, Steven L. Werlin, Michael R. Wessels, Ralph F. Wetmore, Randall C. Wetzel, Isaiah D. Wexler, Perrin C. White, John V. Williams, Rodney E. Willoughby, Samantha L. Wilson, Glenna B. Winnie, Paul H. Wise, Laila Woc-Colburn, Joanne Wolfe, Cynthia J. Wong, Laura L. Worth, Joseph L. Wright, Peter F. Wright, Terry W. Wright, Eveline Y. Wu, Anthony Wynshaw-Boris, Nada Yazigi, Ram Yogev, Marc Yudkoff, Peter E. Zage, Anita K.M. Zaidi, Lonnie K. Zeltzer, Maija H. Zile, Peter Zimmer, and Barry Zuckerman

Published
2011
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7. Invasive fungal infections in pediatric heart transplant recipients: incidence, risk factors, and outcomes

Author

Theoklis E, Zaoutis, Steven, Webber, David C, Naftel, Mary Anne, Chrisant, Beth, Kaufman, F B, Pearce, Robert, Spicer, and Anne I, Dipchand

Subjects
Heart Failure, Male, Risk, Adolescent, Incidence, Candidiasis, Infant, Pediatrics, Treatment Outcome, Mycoses, Risk Factors, Child, Preschool, Multivariate Analysis, Aspergillosis, Heart Transplantation, Humans, Female, Child, Follow-Up Studies
Abstract

There are limited data on the incidence or risk factors for IFI in pediatric heart transplant recipients. The purpose of this study was to describe the incidence and types of IFI, to determine risk factors for outcomes of IFI, and to assist in decision-making concerning the need for prophylactic strategies in pediatric heart transplant recipients. Data from a multi-institutional registry of 1854 patients transplanted between 01/93 and 12/04 were analyzed to determine risk factors and outcomes of children with IFI post-heart transplantation. One hundred and thirty-nine episodes of IFI occurred in 123 patients and made up 6.8% of the total number of post-transplant infections. IFI was most commonly attributed to yeast (66.2%), followed by mold (15.8%) and Pneumocystis jiroveci (13%). Ninety percent of the yeast infections were caused by Candida spp., and Aspergillus spp. was causative in 82% of the mold infections. There was a significantly increased risk of fungal infection associated with pretransplant invasive procedures (e.g., ECMO, prior surgery, VAD, mechanical ventilation) with an incremental risk with increasing numbers of invasive procedures (early phase 0 vs. 1, RR 1.3; 0 vs. 3, RR 2.3; p0.001). In multivariate analysis, previous surgery (p=0.05) and mechanical support at transplantation (p=0.01) remained significant. Forty-nine percent of recipients with IFI died, all within six months post-transplant. Invasive fungal infections are uncommon in pediatric heart transplant recipients. Risk and mortality are highest in the first six months post-transplant especially in patients with previous surgery and those requiring mechanical support. Prophylactic strategies for high-risk patients should be considered and warrants further study.

Published
2010

8. Abstract 2558: Outcomes of Children with Cardiomyopathy Listed for Heart Transplant: A Multi-Institutional Study

Author

Anne I Dipchand, David C Naftel, Brian Feingold, Robert Spicer, Delphine Yung, Beth Kaufman, Tina Allain-Rooney, and Daphne Hsu

Subjects
Physiology (medical), Cardiology and Cardiovascular Medicine
Abstract

BACKGROUND: Dilated (DCM), restrictive (RCM), and hypertrophic (HCM) cardiomyopathies (CM) in children have widely varying clinical courses and therapeutic options. For all types, heart transplant (HTx) offers a chance for long-term survival, but clinical outcomes following listing and predictors to guide in the optimal timing of listing have not been well defined. METHODS AND RESULTS: A multi-institutional, prospective, event-driven registry of pts ] Survival was particularly poor in RCM and HCM pts who were ] Late (10 yr) survival post-HTx was also higher in DCM pts (82%) compared with RCM (76%) and HCM pts (65%, p=0.02). CONCLUSION: Children with CM awaiting HTx have a low waitlist mortality and good survival post-HTx with DCM pts demonstrating a consistent, modest survival advantage from listing. The relatively poor survival in select groups of RCM and HCM pts suggests the need for further study to better understand the causes and timing of death, timing of listing, and influence of patient characteristics on survival.s

Published
2007
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10. The Cryopreserved Homograft Valve in the Pulmonary Position: Early Results and Technical Considerations

Author

JOHN J. LAMBERTI, WILLIAM W. ANGELL, J. DEANE WALDMAN, TODD M. GREHL, LILY GEORGE, JAMES W. MATHEWSON, STANLEY E. KIRKPATRICK, ROBERT SPICER, and DEBORAH THOMSON

Subjects
Adult, Heart Defects, Congenital, Reoperation, Pulmonary and Respiratory Medicine, medicine.medical_specialty, Adolescent, Pulmonary insufficiency, Persistent truncus arteriosus, California, Postoperative Complications, Internal medicine, medicine, Humans, Transplantation, Homologous, Ventricular outflow tract, cardiovascular diseases, Cardiac Surgical Procedures, Child, Survival rate, Contraindication, Aged, Cryopreservation, Pulmonary Valve, business.industry, Infant, Newborn, Infant, Middle Aged, Hospitals, Pediatric, medicine.disease, Pulmonary hypertension, Surgery, Survival Rate, Transplantation, surgical procedures, operative, medicine.anatomical_structure, Child, Preschool, Pulmonary valve, cardiovascular system, Cardiology, Cardiology and Cardiovascular Medicine, business, Follow-Up Studies
Abstract

Since September, 1985, 20 patients have undergone implantation of a homograft valve in the pulmonary position (16 pulmonary, 4 aortic). There were 11 primary operations and 9 reoperations. In 7 of 11 primary operations the homograft valve was utilized as a composite conduit with a short Dacron extension. In four of five reoperations for a failed porcine valved conduit, a composite homograft conduit was used. Four patients underwent implantation of a free homograft in a previously repaired right ventricular outflow tract (RVOT). Age ranged from 15 days to 22 years. There was one operative death (5%), a seven-week-old infant with truncus arteriosus. Long-term follow-up ranges from 1 to 30 months. Clinical performance has been satisfactory in 18 of 19 patients. One patient undergoing free implantation of a pulmonary valve in the RVOT required replacement at 18 months with a porcine valve. In this patient, pulmonary insufficiency was caused by distortion of the annulus secondary to dilatation and pulmonary hypertension. Nine of 18 survivors do not require medication. Eleven of 18 have trivial to mild pulmonary insufficiency murmurs without symptomatology. The homograft valve is extremely useful in reconstruction of the right heart, however, early insufficiency murmurs have been noted. Distortion of the valve annulus may contribute to the early onset of a benign insufficiency murmur. Residual distal obstruction or pulmonary hypertension may be a contraindication to the use of a free homograft in the orthotopic position.

Published
1988
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