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Biventricular non-compaction cardiomyopathy and tricuspid hypoplasia in a novel non-POU domain-containing octamer-binding gene variant
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- Sanam Safi, Stephen P. Sanders, Melissa Zhao, Chrystalle Katte Carreon
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- Journal:
- Cardiology in the Young / Volume 32 / Issue 8 / August 2022
- Published online by Cambridge University Press:
- 12 January 2022, pp. 1333-1337
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A maternally inherited novel pathogenic non-POU domain-containing octamer-binding gene variant c.767G>T, p.R256I [NM_001145408], manifested in a male infant as dilated cardiomyopathy with severe left ventricular dysfunction and dilation, biventricular non-compaction, tricuspid hypoplasia, and hydrocephaly. To the best of our knowledge, no previous non-POU domain-containing octamer-binding gene variants with biventricular non-compaction have been associated with tricuspid valve hypoplasia. Hence, this case introduces a new pathogenic variant observed in the non-POU domain-containing octamer-binding gene and adds to the range of cardiac phenotypes identified in non-POU domain-containing octamer-binding gene variants.
Nomenclature for Pediatric and Congenital Cardiac Care: Unification of Clinical and Administrative Nomenclature – The 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Revision of the International Classification of Diseases (ICD-11)
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- Jeffrey P. Jacobs, Rodney C. G. Franklin, Marie J. Béland, Diane E. Spicer, Steven D. Colan, Henry L. Walters III, Frédérique Bailliard, Lucile Houyel, James D. St. Louis, Leo Lopez, Vera D. Aiello, J. William Gaynor, Otto N. Krogmann, Hiromi Kurosawa, Bohdan J. Maruszewski, Giovanni Stellin, Paul Morris Weinberg, Marshall Lewis Jacobs, Jeffrey R. Boris, Meryl S. Cohen, Allen D. Everett, Jorge M. Giroud, Kristine J. Guleserian, Marina L. Hughes, Amy L. Juraszek, Stephen P. Seslar, Charles W. Shepard, Shubhika Srivastava, Andrew C. Cook, Adrian Crucean, Lazaro E. Hernandez, Rohit S. Loomba, Lindsay S. Rogers, Stephen P. Sanders, Jill J. Savla, Elif Seda Selamet Tierney, Justin T. Tretter, Lianyi Wang, Martin J. Elliott, Constantine Mavroudis, Christo I. Tchervenkov
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- Journal:
- Cardiology in the Young / Volume 31 / Issue 7 / July 2021
- Published online by Cambridge University Press:
- 29 July 2021, pp. 1057-1188
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Substantial progress has been made in the standardization of nomenclature for paediatric and congenital cardiac care. In 1936, Maude Abbott published her Atlas of Congenital Cardiac Disease, which was the first formal attempt to classify congenital heart disease. The International Paediatric and Congenital Cardiac Code (IPCCC) is now utilized worldwide and has most recently become the paediatric and congenital cardiac component of the Eleventh Revision of the International Classification of Diseases (ICD-11). The most recent publication of the IPCCC was in 2017. This manuscript provides an updated 2021 version of the IPCCC.
The International Society for Nomenclature of Paediatric and Congenital Heart Disease (ISNPCHD), in collaboration with the World Health Organization (WHO), developed the paediatric and congenital cardiac nomenclature that is now within the eleventh version of the International Classification of Diseases (ICD-11). This unification of IPCCC and ICD-11 is the IPCCC ICD-11 Nomenclature and is the first time that the clinical nomenclature for paediatric and congenital cardiac care and the administrative nomenclature for paediatric and congenital cardiac care are harmonized. The resultant congenital cardiac component of ICD-11 was increased from 29 congenital cardiac codes in ICD-9 and 73 congenital cardiac codes in ICD-10 to 318 codes submitted by ISNPCHD through 2018 for incorporation into ICD-11. After these 318 terms were incorporated into ICD-11 in 2018, the WHO ICD-11 team added an additional 49 terms, some of which are acceptable legacy terms from ICD-10, while others provide greater granularity than the ISNPCHD thought was originally acceptable. Thus, the total number of paediatric and congenital cardiac terms in ICD-11 is 367. In this manuscript, we describe and review the terminology, hierarchy, and definitions of the IPCCC ICD-11 Nomenclature. This article, therefore, presents a global system of nomenclature for paediatric and congenital cardiac care that unifies clinical and administrative nomenclature.
The members of ISNPCHD realize that the nomenclature published in this manuscript will continue to evolve. The version of the IPCCC that was published in 2017 has evolved and changed, and it is now replaced by this 2021 version. In the future, ISNPCHD will again publish updated versions of IPCCC, as IPCCC continues to evolve.
A Pediatric Cardiology Fellowship Boot Camp improves trainee confidence*
- Catherine K. Allan, Paul Tannous, Elizabeth DeWitt, Michael Farias, Laura Mansfield, Christina Ronai, David Schidlow, Stephen P. Sanders, James E. Lock, Jane W. Newburger, David W. Brown
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- Journal:
- Cardiology in the Young / Volume 26 / Issue 8 / December 2016
- Published online by Cambridge University Press:
- 02 February 2017, pp. 1514-1521
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Introduction
New paediatric cardiology trainees are required to rapidly assimilate knowledge and gain clinical skills to which they have limited or no exposure during residency. The Pediatric Cardiology Fellowship Boot Camp (PCBC) at Boston Children’s Hospital was designed to provide incoming fellows with an intensive exposure to congenital cardiac pathology and a broad overview of major areas of paediatric cardiology practice.
MethodsThe PCBC curriculum was designed by core faculty in cardiac pathology, echocardiography, electrophysiology, interventional cardiology, exercise physiology, and cardiac intensive care. Individual faculty contributed learning objectives, which were refined by fellowship directors and used to build a programme of didactics, hands-on/simulation-based activities, and self-guided learning opportunities.
ResultsA total of 16 incoming fellows participated in the 4-week boot camp, with no concurrent clinical responsibilities, over 2 years. On the basis of pre- and post-PCBC surveys, 80% of trainees strongly agreed that they felt more prepared for clinical responsibilities, and a similar percentage felt that PCBC should be offered to future incoming fellows. Fellows showed significant increase in their confidence in all specific knowledge and skills related to the learning objectives. Fellows rated hands-on learning experiences and simulation-based exercises most highly.
ConclusionsWe describe a novel 4-week-long boot camp designed to expose incoming paediatric cardiology fellows to the broad spectrum of knowledge and skills required for the practice of paediatric cardiology. The experience increased trainee confidence and sense of preparedness to begin fellowship-related responsibilities. Given that highly interactive activities were rated most highly, boot camps in paediatric cardiology should strongly emphasise these elements.
Contributors
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- By Robert S. Anderson, (Mary) Colleen Bhalla, Michelle Blanda, Christopher Carpenter, Chris Chauhan, Paul L. DeSandre, Maura Dickinson, Jonathan A. Edlow, Dany Elsayegh, Kara Iskyan Geren, Peter J. Gruber, Jin H. Han, Marianne Haughey, Teresita M. Hogan, Ula Hwang, Lindsay Jin, Michael P. Jones, Joseph H. Kahn, Keli M. Kwok, Denise Law, Megan M. Leo, Stephen Y. Liang, Judith A. Linden, Brendan G. Magauran Jr, Joseph P. Martinez, Amal Mattu, Karen M. May, Aileen McCabe, Kerry K. McCabe, Jolion McGreevy, Ron Medzon, Ravi K. Murthy, Aneesh T. Narang, Lauren M. Nentwich, David E. Newman-Toker, Jonathan S. Olshaker, Joseph R. Pare, Thomas Perera, Joanna Piechniczek-Buczek, Jesse M. Pines, Timothy Platts-Mills, Suzanne Michelle Rhodes, Lynne Rosenberg, Mark Rosenberg, Todd C. Rothenhaus, Kristine Samson, Arthur B. Sanders, Jeffrey I. Schneider, Rishi Sikka, Kirk A. Stiffler, Morsal R. Tahouni, Mary E. Tanski, Abel Wakai, Scott T. Wilber, Deborah R. Wong
- Edited by Joseph H. Kahn, Brendan G. Magauran, Jr, Jonathan S. Olshaker
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- Book:
- Geriatric Emergency Medicine
- Published online:
- 05 January 2014
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- 16 January 2014, pp vii-x
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- By Ioannis P. Androulakis, Djillali Annane, Gérard Audibert, Lisa L. Barnes, Paolo Bartolomeo, Walter S. Bartynski, David A. Bennett, Nicolas Bruder, Nathan E. Brummel, Steve E. Calvano, Alain Cariou, F. Chretien, Jan Claassen, Colm Cunningham, Souhayl Dahmani, Robert Dantzer, Dimitry S. Davydow, Sanjay V. Desai, E. Wesley Ely, Frédéric Faugeras, Karen J. Ferguson, Brandon Foreman, Sadanand M. Gaikwad, Rebecca F. Gottesman, Maura A. Grega, Richard D. Griffiths, Marion Griton, Stefan D. Gurney, Hebah M. Hefzy, Michael T. Heneka, Dustin M. Hipp, Ramona O. Hopkins, Christopher G. Hughes, James C. Jackson, Christina Jones, Peter W. Kaplan, Keith W. Kelley, Raymond C. Koehler, Matthew A. Koenig, Jan Pieter Konsman, Felix Kork, John P. Kress, Stephen F. Lowry, Alawi Luetz, David Luis, Alasdair M. J. MacLullich, Guy M. McKhann, Jean Mantz, Panteleimon D. Mavroudis, Mervyn Maze, Bruno Mégarbane, Lionel Naccache, Dale M. Needham, Pratik P. Pandharipande, Jean-Francois Payen, V. Hugh Perry, Margaret Pisani, C. Rauturier, Benjamin Rohaut, Jennifer Ryan, Robert D. Sanders, Jeremy D. Scheff, Frederic Sedel, Ola A. Selnes, Tarek Sharshar, Martin Siegemund, Yoanna Skrobik, Jamie W. Sleigh, Romain Sonneville, Claudia D. Spies, Luzius A. Steiner, Robert D. Stevens, Raoul Sutter, Fabio Silvio Taccone, Richard E. Temes, Willem A. van Gool, Christel C. Vanbesien, F. Verdonk, Odile Viltart, Julia Wendon, Catherine N. Widmann, Robert S. Wilson
- Edited by Robert D. Stevens, Tarek Sharshar, E. Wesley Ely, Vanderbilt University, Tennessee
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- Brain Disorders in Critical Illness
- Published online:
- 05 October 2013
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- 19 September 2013, pp viii-xii
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Ventricular mechanics in patients with aortic valve disease: longitudinal, radial, and circumferential components
- Benedetta Leonardi, Renee Margossian, Stephen P. Sanders, Marcello Chinali, Steven D. Colan
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- Journal:
- Cardiology in the Young / Volume 24 / Issue 1 / February 2014
- Published online by Cambridge University Press:
- 07 February 2013, pp. 105-112
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Background
Reduced long-axis shortening despite enhanced global function has been reported in aortic stenosis. We sought to improve the understanding of this phenomenon using multi-dimensional strain analysis in conjunction with the evaluation of left ventricular rotation and twist – ventricular torsion – using tissue Doppler techniques.
MethodsA total of 57 patients with variable severity of aortic stenosis, aortic regurgitation, or mixed aortic valve disease, subdivided into six groups, were studied. Ventricular morphology was assessed using long-axis/short-axis and mass/volume ratios, afterload using end-systolic meridional wall stress, and global performance using ejection fraction. The circumferential and longitudinal strain was measured from two-dimensional images, and left ventricular rotation and twist were estimated as the difference in rotation between the base and apex of the ventricle.
ResultsAortic stenosis was associated with higher mass/volume, ejection fraction, circumferential strain and left ventricular rotation and twist, significantly lower end-systolic wall stress, and a trend towards lower longitudinal strain compared with normal. Myocardial mechanics in aortic regurgitation were normal despite ventricular dilation. Mixed aortic valve disease showed findings similar to aortic stenosis. Left ventricular rotation and twist correlated with midwall circumferential strain (r = 0.62 and p < 0.0001), endocardial circumferential strain (r = 0.61 and p < 0.0001), and end-systolic wall stress (r = 0.48 and p < 0.0001), but not with longitudinal strain (r = 0.18 and p > 0.05).
ConclusionsMyocardial mechanics are normal in patients with aortic regurgitation, independent of abnormalities in cardiac geometry. Conversely, in aortic stenosis and mixed aortic valve disease, significant alterations in the patterns of fibre shortening are found. The effects of stenosis on cardiac function seem to dominate the effect of ventricular remodelling.
The risk of having additional obstructive lesions in neonatal coarctation of the aorta
- Jami C. Levine, Stephen P. Sanders, Steven D. Colan, Richard A. Jonas, Philip J. Spevak
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- Cardiology in the Young / Volume 11 / Issue 1 / January 2001
- Published online by Cambridge University Press:
- 01 July 2011, pp. 44-53
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Infants with coarctation of the aorta may have obstructions at other sites within the left heart which are not always apparent on the initial echocardiogram. The magnitude of the risk of having the additional obstructions is not well described, with few reliable quantitative criterions for identifying patients at the highest risk. We determined the frequency of additional, late appearing, stenotic lesions within the left heart, and the predictive morphologic features on the initial cross-sectional echocardiogram.
We identified all patients with coarctation of the aorta diagnosed by 3 months of age, excluding those with complex cardiac disease or definite additional stenotic lesions at presentation, leaving 101 patients for study. At follow-up, 31 stenotic lesions were diagnosed in 23 patients, 15 of whom had at least 1 intervention. Mitral stenosis was diagnosed in 11 patients, aortic stenosis in 10, subaortic stenosis in 8, and supravalvar aortic stenosis in 2. The probability for freedom from obstructive lesions was 81% at 1 year, 74% at 3 years, and 70% at 5 years. Echocardiographic predictors of mitral stenosis included smaller mitral valvar annuluses, presence of a mean transmitral gradient between 2.5 and 5.0 mmHg, and elongation of the area of intervalvar fibrous continuity. Predictors of aortic stenosis were smaller mitral valvar annuluses, an initial aortic valvar gradient between 15 and 20 mmHg, and obliteration of the commissure between the right and non-coronary leaflets of the aortic valve. Predictors of subaortic stenosis were smaller mitral valvar annuluses and elongation of the area of intervalvar fibrous continuity. Patients with Z-scores for the diameter of the mitral valve of less than −1 were at the highest risk for manifesting obstructive lesions at any level.
Associated stenoses in the left heart are common in the setting of aortic coarctation. When Doppler data is equivocal, features of the cross-sectional echocardiogram can identify the sub-group of infants at increased risk.
Contributors
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- By Waiel Almoustadi, Brian J. Anderson, David B. Auyong, Michael Avidan, Michael J. Avram, Roland J. Bainton, Jeffrey R. Balser, Juliana Barr, W. Scott Beattie, Manfred Blobner, T. Andrew Bowdle, Walter A. Boyle, Eugene B. Campbell, Laura F. Cavallone, Mario Cibelli, C. Michael Crowder, Ola Dale, M. Frances Davies, Mark Dershwitz, George Despotis, Clifford S. Deutschman, Brian S. Donahue, Marcel E. Durieux, Thomas J. Ebert, Talmage D. Egan, Helge Eilers, E. Wesley Ely, Charles W. Emala, Alex S. Evers, Heidrun Fink, Pierre Foëx, Stuart A. Forman, Helen F. Galley, Josephine M. Garcia-Ferrer, Robert W. Gereau, Tony Gin, David Glick, B. Joseph Guglielmo, Dhanesh K. Gupta, Howard B. Gutstein, Robert G. Hahn, Greg B. Hammer, Brian P. Head, Helen Higham, Laureen Hill, Kirk Hogan, Charles W. Hogue, Christopher G. Hughes, Eric Jacobsohn, Roger A. Johns, Dean R. Jones, Max Kelz, Evan D. Kharasch, Ellen W. King, W. Andrew Kofke, Tom C. Krejcie, Richard M. Langford, H. T. Lee, Isobel Lever, Jerrold H. Levy, J. Lance Lichtor, Larry Lindenbaum, Hung Pin Liu, Geoff Lockwood, Alex Macario, Conan MacDougall, M. B. MacIver, Aman Mahajan, Nándor Marczin, J. A. Jeevendra Martyn, George A. Mashour, Mervyn Maze, Thomas McDowell, Stuart McGrane, Berend Mets, Patrick Meybohm, Charles F. Minto, Jonathan Moss, Mohamed Naguib, Istvan Nagy, Nick Oliver, Paul S. Pagel, Pratik P. Pandharipande, Piyush Patel, Andrew J. Patterson, Robert A. Pearce, Ronald G. Pearl, Misha Perouansky, Kristof Racz, Chinniampalayam Rajamohan, Nilesh Randive, Imre Redai, Stephen Robinson, Richard W. Rosenquist, Carl E. Rosow, Uwe Rudolph, Francis V. Salinas, Robert D. Sanders, Sunita Sastry, Michael Schäfer, Jens Scholz, Thomas W. Schnider, Mark A. Schumacher, John W. Sear, Frédérique S. Servin, Jeffrey H. Silverstein, Tom De Smet, Martin Smith, Joe Henry Steinbach, Markus Steinfath, David F. Stowe, Gary R. Strichartz, Michel M. R. F. Struys, Isao Tsuneyoshi, Robert A. Veselis, Arthur Wallace, Robert P. Walt, David C. Warltier, Nigel R. Webster, Jeanine Wiener-Kronish, Troy Wildes, Paul Wischmeyer, Ling-Gang Wu, Stephen Yang
- Edited by Alex S. Evers, Washington University School of Medicine, St Louis, Mervyn Maze, University of California, San Francisco, Evan D. Kharasch, Washington University School of Medicine, St Louis
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- Book:
- Anesthetic Pharmacology
- Published online:
- 11 April 2011
- Print publication:
- 10 March 2011, pp viii-xiv
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- By Charles E. Argoff, Gerard A. Banez, Samantha Boris-Karpel, Barbara K. Bruce, Alexandra S. Bullough, Annmarie Cano, Victor T. Chang, Elizabeth A. Clark, Daniel J. Clauw, June L. Dahl, Tam K. Dao, Amber M. Davis, Courtney L. Dixon, Michael H. Ebert, Robin M. Gallagher, Gerald W. Grass, Carmen R. Green, Jay Gunkelman, Bradford D. Hare, Jennifer A. Haythornthwaite, Jaclyn Heller Issner, W. Michael Hooten, Mark P. Jensen, Mark E. Jones, Robert D. Kerns, Raphael J. Leo, Morris Maizels, Mary E. Murawski, Brooke Myers-Sorger, Akiko Okifuji, Renata Okonkwo, John D. Otis, Stacy C. Parenteau, Laura E. Pence, Donald B. Penzien, Donna B. Pincus, Ellyn Poltrock Stein, Wendy J. Quinton, Jeanetta C. Rains, M. Carrington Reid, Thomas J. Romano, Jeffrey D. Rome, Robert L. Ruff, Suzanne S. Ruff, Steven H. Sanders, Ingra Schellenberg, John J. Sellinger, Howard S. Smith, Brenda Stoelb, Jon Streltzer, Mark D. Sullivan, Kimberly S. Swanson, Gabriel Tan, Stephen Thielke, Beverly E. Thorn, Cynthia O. Townsend, Dennis C. Turk, Stephanie C. Wallio, Lawrence J. Weinberger, David A. Williams, Hilary Wilson
- Edited by Michael H. Ebert, Yale University, Connecticut, Robert D. Kerns, Yale University, Connecticut
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- Book:
- Behavioral and Psychopharmacologic Pain Management
- Published online:
- 10 January 2011
- Print publication:
- 25 November 2010, pp ix-xii
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Anomalous origin of one pulmonary artery from the ascending aorta
- Vladimiro L. Vida, Stephen P. Sanders, Tomaso Bottio, Nicola Maschietto, Maurizio Rubino, Ornella Milanesi, Giovanni Stellin
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- Journal:
- Cardiology in the Young / Volume 15 / Issue 2 / March 2005
- Published online by Cambridge University Press:
- 22 April 2005, pp. 176-181
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We describe the surgical repair in three infants presenting with one pulmonary artery arising from the ascending aorta, the other artery arising normally from the right ventricle via the pulmonary trunk. Repair consisted of reimplantation of the anomalous pulmonary artery to the pulmonary trunk, in association with repair of associated intracardiac malformations. All patients survived the surgical procedures, and were discharged in stable clinical condition. Subsequently, two of the three patients developed stenosis at the surgical anastomosis relatively early after the initial procedure, and underwent reoperation. Although survival after operation is now expected for this malformation, reports of late results are lacking. Larger numbers of operations are needed before we can reach definitive conclusions.
The origin of one branch pulmonary artery from the ascending aorta in the presence of a pulmonary valve and main pulmonary artery is a very rare congenital cardiac anomaly. Patients and methods: Between January 1995 and June 2003, 3 infant girls presented with the origin of one branch artery from the ascending aorta, while the other pulmonary artery originated from the pulmonary trunk which was in continuity with the right ventricular outflow tract. The pulmonary artery that arose from the right ventricle was left in 2 and right in 1 patient. Results: At the age 13, 48 and 62 days respectively, the patients underwent surgical repair consisting with reimplantation of the anomalous pulmonary artery branch to the pulmonary trunk in association with repair of intracardiac malformations. There were no hospital deaths. Postoperative complications included: prologed intubation in two patients (10 and 16 days), low output syndrome in 1 patient, cardiac tamponade in 1 patient and seizures in 1 patient. All patients were discharged in good clinical condition. There have been no late deaths. Subsequently, two of the three patients developed stenosis at the surgical anastomosis relatively early after the initial procedure, and after unsuccessuful balloon dilation, underwent surgical reoperation. Conclusions: Although operative survival is now possible for this malformation, reports of late results are lacking. Two of the three patients developed stenosis at the surgical anastomosis relatively early after surgery. Larger numbers of operations are necessaries to reach definitive conclusions.