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  1. 1. C O N G E N I T A L C A R D I O L O G Y T O D A Y R E L I A B L E I N F O R M A T I O N I N C O N G E N I T A L C A R D I O L O G Y INTERNATIONAL EDITION WWW.CONGENITALCARDIOLOGYTODAY.COM VOL. 3, ISSUE 4 APRIL 2005 I NCORPOR ATING PED IATR IC C ARD IOL OGY T OD AY INSIDE THIS ISSUE C O N G E N I TA L H E A R T D I S E A S E I N T H E Congenital Heart Disease 1 DEVELOP ING WORLD in the Developing World by R. Krishna Kumar, MD By R. Krishna Kumar, MD when introduced. Over a period of time Highlights from 9 Cardiology 2005: 8th after introduction, the costs decline with Annual Post Graduate increasing consumption and competition Course in Pediatric The specialties of pediatric cardiology and Cardiovascular Disease among manufacturers. Coronary angio- by Gil Wernovsky, MD pediatric cardiac surgery have rapidly plasty is a good example. Since its intro- evolved in the last four decades, largely duction the cost of angioplasty hardware Screening for Sudden 12 Cardiac Death Using the spurred by the dedicated efforts of pio- has declined substantially. The cost re- Pre-participation Physical neering professionals together with major duction was made possible through an Exam technological advances. Today, they are exponential growth in consumption. The by Tiffany J. Riehle, MD and Robert C. Campbell, MD good examples of medical specialties that large size of the potential market spurred a largely depend on advanced training and number of manufacturers to enter the fray Respiratory Failure After 15 sophisticated modern technology to make and the competition forced them to focus Intrathoracic Surgery an impact on affected patients. The list of on reducing costs. by Aphrodite Tzifa, MD and achievements in the last 40 years is truly Shakeel Qureshi, MD The size of the market impressive. 1 Either cor- Congenital Cardiovascular 20 rection or some form of or the consumers of Interventional Study palliation is possible for “Unfortunately ... very few “ p e d i a t r i c car diac Consortium (CCISC) technology” is per- by Tom Forbes, MD the majority of congenital high-quality institutions heart diseases in most with comprehensive ceived as small. This advanced pediatric heart is because the num- DEPARTMENTS facilities to take care of centers. The results are ber of children af- May Conference Focus - children with congenital fected with congenital The 1st Annual Toronto 3 often dramatic in individ- ual patients. The spe- heart disease exist outside heart disease is rela- Symposium cialties continue to grow of the developed world.” tively small when Medical News 6 impressively with pro- compared to acquired June Conference Focus - gressive sophistication and advancement coronary artery dis- 8th International Workshop of technology. The industry that has de- ease. However, most manufacturers 11 in Congenital and Structural veloped in connection with the technology of “pediatric cardiac technology” have Heart Disease enthusiastically supports this progress and not been looking beyond industrialized Medical Conferences 16 works in close collaboration with pediatric nations, at least not until recently. The cardiac surgeons, pediatric cardiologists global burden of congenital heart dis- June Conference Focus - PEDIRHYTHM 2 19 and other providers of pediatric cardiac ease is likely to be several times that care. With the advent of globalization, this of the industrialized or developed na- new technology has become widely avail- tions simply because of a much larger able for patients all over the world. population. Unfortunately, however, CONGENITAL CARDIOLOGY TODAY very few high-quality institutions with 9008 Copenhaver Drive, Ste. M Potomac, MD 20854 USA While impressive strides have been made comprehensive facilities to take care of www.CongenitalCardiologyToday.com in the quality of care, the costs of care children with congenital heart disease © 2005 by Congenital Cardiology Today continue to be rather high. 2,3 Most inter- exist outside of the developed world. (ISSN 1554-7787 - print; ISSN 1554-049 ventional or surgical procedures are simply - online). Published monthly. All rights unaffordable to most families in the ab- It is not just the costs of consumables sence of some form of heath insurance. and equipment that make pediatric reserved. Statements or opinions ex- pressed in Congenital Cardiology Today reflect the views of the authors and are Unlike other specialties, over the years the heart care expensive. A number of not necessarily the views of Congenital Cardiology Today. situation has not changed significantly. other factors contribute. They include Most new technologies are expensive infrastructure costs and costs of em-
  2. 2. PAGE 2 APRIL 2005 INTERNATIONAL EDITION CONGENITAL CARDIOLOGY TODAY CONGENITAL CARDIOLOGY TODAY ploying skilled manpower. The incorporating Pedi atric Cardiology Today specialty consumes considerable material and human resources “Given the current health © 2005 by Congenital Cardiology Today (ISSN 1554-7787 - print; ISSN 1554-049 - online) just to be able to deliver “basic” care structure in most of Published monthly. All rights reserved. services. The manpower required the developing world, it is Publishing Management for taking care of a typical child not surprising that the Tony Carlson, Founder undergoing congenital heart sur- T o n yC @ C o ng e ni ta lC a r di ol og y To d ay .c om vast majority of children gery include: the pediatric cardiac Richard Koulbanis, Publisher & Editor surgeon, pediatric cardiologist, born with congenital R ic h a rd K @ Co n ge ni t al C a rd io lo gy T o day .c om pediatric anesthesiologist, pediat- heart disease receive John W. Moore, MD, MPH, Medical Editor & Medical Editorial Board ric intensivists (ideally pediatric no attention.” j wm o o r e @m e dn e t .uc la . ed u cardiac intensivists), pediatric Editorial Board perfusion technologists, pediatric cally. These definitions and clas- Zohair Al Halees, MD nurses and respiratory therapists. sifications are essentially broad The infrastructure includes a so- genera liza tion s ( see h ttp :// Mazeni Alwi, MD phisticated operation theater with hdr.undp.org/reports/global/2004). Felix Berger, MD current cardiopulmonary bypass Not only do the countries in the Fadi Bitar, MD equipment, a modern pediatric developing world differ markedly Philipp Bonhoeffer, MD intensive care facility, a cardiac in their health statistics from de- catheterization laboratory, and a veloped countries, but there is Anthony C. Chang, MD, MBA non-invasive imaging laboratory also marked variability between Bharat Dalvi, MD, MBBS, DM for echocardiography. countries of medium and low hu- Horacio Faella, MD man development. Further, there What is the Developing World? Yun-Ching Fu, MD is considerable variability even Felipe Heusser, MD The ter m “d evelop ing world” within countries. 4 loosely encompasses a group of Ziyad M. Hijazi, MD, MPH With the advent of globalization middle- and low-income countries Ralf Holzer, MD there are important changes tak- classified by the World Bank ing place in the developing world. R. Krishna Kumar, MD, DM, MBBS largely on basis of the per-capita Selected, small urban pockets Gerald Ross Marx, MD gross national income (see http:// within the developing countries www.worldbank.org/data/ Tarek S. Momenah, MBBS, DCH have become increasingly affluent cou ntr ycla ss/coun tr ycl a ss.h tml) . Toshio Nakanishi, MD, PhD and often cannot be distinguished Time and experience have shown, from developed nations. The per Carlos A. C. Pedra, MD however, that social conditions capita income in these pockets is James C. Perry, MD and the general well being of peo- considerably higher and infra- ple may not necessarily improve Shakeel A. Qureshi, MD structure is quite sophisticated in when a country’s average income Andrew Redington, MD comparison with the rest of the level increases. Considerable dif- country. Essentially, these are Carlos E. Ruiz, MD, PhD ferences exist in health care de- islands of “development” that are Girish S. Shirali, MD, MBBS, FAAP, FACC livery and health indices among situated amidst an ocean of depri- these countries. The human de- Horst Sievert, MD vation and poverty. These afflu- velopment index (HDI) is a more Hideshi Tomita, MD ent pockets are looked upon as comprehensive index that takes Gil Wernovsky, MD prospective, lucrative markets for into account quality of life as the global industry and local en- Carlos Zabal, MD measured by life expectancy, in- trepreneurs. The health care fa- To Contact an Editorial Board Member fant mortality and literacy. Nations cilities in these areas are often Email to: NAME@CongenitalCardiologyToday.com are now ranked on basis of HDI ve r y so ph i sti ca ted , bu t car e Place the Board Member’s surname before the @. and this list is revised periodi- P I CS /EN TI CHS - 2 005 Pediatric Interventional Cardiac Symposium and Emerging New Technologies in Congenital Heart Surgery SEPTEMBER 15-18, 2005 At the Hilton Buenos Aires Buenos Aires, Argentina Immediately preceding the 4th World Congress Of Pediatric Cardiology and Cardiac Surgery www.picsymposium.com © Copyright 2005, Congenital Cardiology Today. All rights reserved www.Cong enitalCard iolog y Tod ay .com
  3. 3. CONGENITAL CARDIOLOGY TODAY INTERNATIONAL EDITION APRIL 2005 PAGE 3 comes at a price that is not at all the population, as health care is affordable to the average citizen becoming increasingly corpora- MAY CONFERENCE FOCUS of the country. There are sharp tized and driven by profit motives. paradoxes: with areas of extreme The situation is particularly frus- The 1st Annual Toronto poverty, malnutrition and ill health trating for patients and families Symposium: Contemporary without access to the very basic who are often unable to afford Questions In Congenital Heart health services in urban slums quality care that is available in the Disease 2005 si tu ated ad ja cen t to affluen t cities in which they live. May 29-31, 2005; Toronto, Canada neighborhoods with access to the www .sickkids.ca/cardiology Congenital Heart Disease in the very best in tertiary health care. Developing World This symposium from Sick Kids Hospi- These paradoxes are visible even tal in Toronto (a health care, teaching to the casual visitor to modern The majority of the world’s inhabi- and research facility dedicated exclu- metros of the developing world. tants live in developing countries. sively to children; affiliated with the Today, there are examples of pa- Although there are no reliable sta- University of Toronto) will aim to ad- tients from United Kingdom who tistics, the number of affected dress hot topics in the field of child and are now flying into selected cen- children in the developing world is adolescent cardiology. ters in Indian Metros (such as likely several times that of the de- Each lecture is framed to answer a Mumbai and Bangalore) for car- veloped world simply because of specific and important question in pedi- diac surgery that is offered at a the large populations. Given the atric cardiology. As such, the clinical considerably lower cost as com- current health care structure in management, evidence base, and pared to private centers in the most of the developing world, it is current research understanding of UK. Yet, the vast majority of those not surprising that the vast major- very specific issues will be addressed living in the less privileged sec- ity of children born with congenital in detail. tions of the same city cannot af- heart disease receive no atten- ford care in these centers. The tion. The situation can only This program is planned to bring to- gap between the “haves” and gether experts in the field of congenital change through establishment of heart disease, answer important and “have-nots” only widened over the regionalized centers of excellence provocative questions in the field of last few decades when developing within each country that focus on pediatric cardiology, develop a better nations have attempted to make affordable care for children with understanding of the topics and pro- the transition to emerging econo- congenital heart disease. The vide opportunity for discussion and mies. Quality tertiary health care population, geography and avail- audience participation. Major topics essentially can now only be af- able resources will determine the include: forded by a tiny fraction of the number of such centers. Simulta- population. Sadly, however, very neously, institution of a number of • Disease in the Normal Heart, little efforts are being made to other measures targeted at en- reach out to a larger fraction of couraging early diagnosis and • Heart Failure/Cardiomyopathy, Catheter Intervention, prompt referral of children with congenital heart disease is re- • Out-Patient Cardiology “Formulation of health quired. It is also necessary to policies in the developing obtain an estimate of the absolute • Surgical Outcomes world for congenital heart and relative magnitude of the problem of congenital heart dis- • Imaging, and Fetal Cardiology disease in a complex and rapidly changing health ease through appropriate surveys. There will be breakout sessions on Formulation of health policies in Inpatient Care, Questions Parents Ask care environment is likely the developing world for congeni- and How to Best Answer Them, and to remain a formidable tal heart disease in a complex and Genes and Development. There will challenge.” rapidly changing health care envi- also be debates on important topics. ronment is likely to remain a for- www.Conge nitalCardiology Tod ay .com © Copyright 2005, Congenital Cardiology Today. All rights reserved
  4. 4. PAGE 4 APRIL 2005 INTERNATIONAL EDITION CONGENITAL CARDIOLOGY TODAY midable challenge. Clearly in Broad Category Specific Issues much of the developing world, the Epidemiological Sig- Global disease burden of congenital heart disease focus should be on health care nificance delivery with an aim to reach out The burden in developing world to the greatest proportion of ef- Proportional infant and childhood mortality for CHD fected patients. For example, it may not be appropriate to perform Relative significance of CHD as compared to acquired multi-staged palliative operations heart disease such as rheumatic heart disease with a doubtful long-term survival Planning epidemiologic surveys in the developing world or neurodevelopmental outcome (such as in hypoplastic left heart Establishing a Center for Personnel Comprehensive Care of Material resources; infrastructure Patients with Congenital “In the coming issues of Heart Disease Support services and systems Congenital Cardiology Ensuring economic sustainability of the program Today, we hope to Overcoming philosophical hurdles (teamwork, multi- highlight these items specialty institution-based care etc., infection control, etc.) systematically using the experience of selected Resources Number of centers for comprehensive pediatric heart care in the developing world centers that have been established in the Proportion of patients with CHD are likely to receive care in these centers developing world in the last 10 years.” Health insurance for patients with CHD Institutional and governmental support for individual pa- tients syndrome) when there are many infants with relatively straightfor- Special Challenges of the Malnutrition ward conditions (such as ventricu- CHD Patient Population in lar septal defect) that need a sin- Infections: Neonatal sepsis; lung infections the Developing World gle corrective operation. Technol- Consequences of late presentation: ogy and human endeavor has to Pulmonary hypertension be appropriately directed towards this end. A list of action items that Neurological complications and major neuro- could be considered in order to developmental sequelae establish a system for care of Cardiac consequences: ventricular dysfunction, atrial children with congenital heart dis- enlargement and arrhythmias ease in the developing world is shown in the table. Managing CHD Patients in Prioritization with respect to lesions Developing Nations: In the coming issues of Congeni- Cost-effective strategies for cardiac catheterization and Practicalities tal Cardiology Today, we hope to interventions highlight these items systemati- Cost-effective strategies for congenital heart surgery and cally using the experience of se- intensive care lected centers that have been es- The challenge of infection control tablished in the developing world in the last 10 years. The cumula- Table 1. Issues involved in care of Patients with Congenital Heart Disease in Developing Countries. tive experience in terms of num- 28th Annual Scientific Sessions and Melvin P. Judkins Cardiac Imaging Symposium May 4-7, 2005, Ponte Vedra Beach, Florida The Society for Cardiovascular Angiography and Interventions www.scai.org © Copyright 2005, Congenital Cardiology Today. All rights reserved www.Cong enitalCard iolog y Tod ay .com
  5. 5. CONGENITAL CARDIOLOGY TODAY INTERNATIONAL EDITION APRIL 2005 PAGE 5 Alto, CA, for critically reviewing the Broad Category Specific Issues manuscript. Inexpensive and Indigenous Occlusive Devices References Technology Mechanical heart valves 1. Gersony WM. Major advances in Disposables for cardiac catheterization, congenital heart pediatric cardiology in the 20th century: surgery and intensive care II. Ther apeut ics. J Pedi atr. 2001;139:328-33. Echocardiography machines Cardiac catheterization laboratories 2. Hewitson J, Brink J, Zilla P. The challenge of pediatric cardiac services Training Pediatric Cardiology in the developing world. Semin Thorac Pediatric Cardiac Surgery Cardiovasc Surg. 2002;14:340-5. Anesthesiology and intensive care 3. Kumar K. Congenital heart disease management in the developing world Improving Awareness Pediatricians and primary care physicians (letter to editor), Pediatric Cardiology Through Education Adult cardiologists involved in the care of patients with 2003, 24:311. CHD 4. Saran R, India’s best and worst Obstetricians, radiologists performing prenatal ultrasound states, India Today, 2004, Aug 11-16, General public Vol 29, 22-33. Formulating Health Policies Defining the relative priority for congenital heart disease For comments to this article, send email to: APRRKK@CongenitalCardiologyToday.com Regulatory bodies on medical education ~CCT~ Policies on health insurance coverage Support to institutions with pediatric heart programs Research and education grants Support for individual patients Collaboration with Centers Exchange programs for training in the Developed World Visits of teams from institutions in the developed world R. Krishna Kumar, MD, DM, FACC Chief Pediatric Cardiologist Ethical Issues Bridging the gap between what is possible and what is feasible Amrita Institute of Medical Sciences and Research Center Managing “complex” defects Kochi, 682026, Kerala, India Facilitating decision-making for families Phone: 91-484-2801234 Table 1—(continued). Issues involved in care of Patients with Congenital Heart Disease in Fax: 91-484-2802020 Developing Countries. rkris hnaku mar@a imsh osp ita l.org bers in these centers has been Acknowledgement substantial. The challenges of providing care to a unique popula- The author wishes to acknowledge You may qualify for a free subscription. tion with limited resources are Dr. Stephen Roth, Associate Pro- Send email with name, organization, many and worth sharing with the fessor, Department of Pediatric address, phone and email to: global community of providers of Cardiology, Lucille Packard Chil- Subs@CongenitalCardiologyToday.com pediatric cardiac care. dren’s Hospital at Stanford, Palo www.Conge nitalCardiology Tod ay .com © Copyright 2005, Congenital Cardiology Today. All rights reserved
  6. 6. PAGE 6 APRIL 2005 INTERNATIONAL EDITION CONGENITAL CARDIOLOGY TODAY M E D I C A L N E W S A N D I N F O R M AT I O N The Genetic Causes of One of the Most Common Types of either of these genes or two other related genes are impli- Congenital Heart Defects are Being Studied by cated in sporadic heart abnormalities." Academics at The University of Nottingham Learning more about how the genes work could eventually Professor David Brook in the University's Institute of Genetics lead to a better diagnosis of the condition. is working in collaboration with colleagues at Leicester's Professor Brook added: "Methods for DNA testing are improv- Glenfield Hospital on the British Heart Foundation funded pro- ing all the time. It could be that in the future doctors could test ject, which will explore the molecular science behind the con- just a single drop of blood to identify at birth whether a child ditions Atrial Septal Defect (ASD) and Ventricular Septal De- has this faulty gene and warrants further examination for hole fect (VSD) ― more commonly known as a 'hole in the heart' in the heart." and accounts for around half of all congenital heart defects. Professor Peter Weissberg, Medical Director of the BHF, con- Until recently, ASD was thought to be sporadic and diagnosis cluded, "We are delighted to have funded this research, which for many patients came out of the blue. However, the team of provides a molecular explanation for why the wall that sepa- researchers is studying patients ― both adults and children ― rates the left and right chambers of the heart (the atria) fails to who have been diagnosed with ASD and other congenital form properly. It is a good illustration of how detailed research heart disorders, and their families, to find out whether in some on a rare familial form of a condition can provide answers that cases it could be an inherited condition. may ultimately lead to treatments for a much larger popula- Funded with a grant of £1,079,803 from the British Heart tion. Since ASDs can be readily detected at an early age by Foundation (BHF), the five-year project will be looking for ultrasound imaging, this research marks the first step towards more evidence to support the researchers' theory that the developing drugs that might induce the closure of a detected heart defect may be due to faulty genes, which helps to regu- defect without the need to resort to surgery, something that late the development of the heart. the BHF is proud to be funding." The first breakthrough came when the researchers were The British Heart Foundation is a major funder and authority studying the rare genetically-inherited condition Holt Oram in cardiovascular research. For more information: Syndrome. Patients with the condition are born with limb de- www.nottingham.ac.uk/biology or www.bhf.org.uk fects, in some cases without any upper limbs. It is also char- acterized by a heart defect, most often a hole in the heart, either ASD or VSD. International Heart and Sports Medicine Experts Call for Cardiovascular Screening Programme for Athletes A gene termed TBX5 was found to be responsible for the con- dition, which led the researchers to question whether it may (Source: European Heart Journal) International heart and also be a factor in cases of ASD or VSD that doctors previ- sports medicine experts have called for a Europe-wide cardio- ously believed to be sporadic. vascular screening programme for all young athletes before they are allowed to take part in competitive athletics. In collaboration with researchers in Paris, the Nottingham group has now found that another gene called MYH6 is faulty The aim is to pick up potentially life-threatening problems that in members of a family with inherited ASD. This work, pub- put young athletes at risk and to cut the numbers collapsing lished in a recent issue of the journal Nature Genetics, also and dying while participating in competitive sport. shows that MYH6 is regulated by TBX5, which provides an important link between the two genes in the origins of heart A European Society of Cardiology consensus report1 was published 2 February 2005 in Europe's leading cardiology defects. journal, the European Heart Journal, 2 recommends that every Professor Brook said, "In patients with Holt Oram Syndrome young athlete involved in organised sport has a rigorous their TBX5 doesn't work properly. It either doesn't tell the physical examination, a detailed investigation of their personal other genes that it controls what to do or there is not enough and family medical history and, most importantly, a 12-lead of it to make other 'down-stream- genes’ behave as they ECG. should. The report's writers believe that screening using ECG has the "We now know that MYH6 is an important downstream gene potential to cut sports-related cardiac deaths in Europe by and we believe that a certain proportion of sporadic ASDs and 50%-70% if it can be implemented in every country.3 VSDs may be due to an inherited predisposition. Thus we are testing patients for changes in their DNA to discover whether Lead author Dr. Domenico Corrado from the Departments of © Copyright 2005, Congenital Cardiology Today. All rights reserved www.Cong enitalCard iolog y Tod ay .com
  7. 7. CONGENITAL CARDIOLOGY TODAY INTERNATIONAL EDITION APRIL 2005 PAGE 7 Cardiology and Pathology at the University of Padova, Italy, petitors, he said, because baseline ECG signs of blood flow said, "We know very little about the risk of sudden death asso- (ischaemic) problems were scarce. However, his research ciated with exercise in young competitors, so the benefits ver- team had earlier reported that about a quarter of the young sus the hazards of sports activity pose a clinical dilemma. athletes who had died from coronary artery diseases had dis- However, we know from a study in the Veneto region of Italy played warning signs or ECG abnormalities during screening that adolescents and young adults involved in competitive that could raise suspicions of a cardiac disease. sport had a two and a half times higher risk of sudden death. The consensus group recommends that screening should start The young competitors who died suddenly were affected by around the age of 12 to 14 and be repeated at least every two silent cardiovascular diseases, predominantly cardio- years. It should involve complete personal and family history, a myopathies, premature coronary artery disease and congeni- physical examination that includes blood pressure measure- tal coronary anomalies." ment and a 12-lead ECG. It should be performed by a physi- He said it was not sport that directly caused the deaths, but cian with specific training who could reliably identify clinical rather that it triggered cardiac arrest in athletes with underly- symptoms and signs associated with cardiovascular diseases ing diseases predisposing them to life threatening ventricular responsible for exercise-related sudden death. Those who arrhythmias. tested positive according to set criteria should be referred for more extensive tests, and if that confirmed suspicions, barred The consensus group was also drawing on the results of It- from competition and training. aly's 25-year experience of systematic prescreening in reach- ing its conclusions. Italy has a mandatory eligibility test involv- "From all the evidence that we have from 25 years experience ing nearly six million young people every year and the test in Italy, we can state unequivocally that screening is war- leans heavily on the use of 12-lead ECG. In one 17-year study ranted," said Dr. Corrado. "It is ethically and clinically justifiable by the Center for Sports Medicine of Padova involving nearly to make every effort to recognize in good time the diseases that 34,000 athletes under 35, over 1,000 were disqualified from put these athletes and risk, and to reduce fatalities. competing on health grounds, 621 (1.8%) because the tests "Screening of large athletic populations will have significant revealed relevant cardiovascular abnormalities. socio-economic impact and its implementation across Europe Dr. Corrado said that in the USA young athletes had physical will depend on the different socio-economic and cultural back- examinations and personal and family history investigations, grounds as well as on the specific medical systems in place in but 12-lead ECG was done only at the doctor's discretion. The different countries. However, experience in Italy indicates that American Heart Association previously assumed that ECG the proposed screening design is made feasible because of the would not be cost-effective for screening because of low limited cost of 12-lead ECG in a mass screening setting. specificity.4 "The cost in Italy of screening, aside from equipment and train- "In fact, this is not the case," said Dr Corrado "The Italian ing, is around €30 including the ECG and is covered by the screening method has proven to be more sensitive than the athlete or his team, except for the under 18s for whom there is limited US protocol. ECG is abnormal in up to 95% of patients National Health System support. Although the protocol is at with hypertropic cardiomyopathy (HCM), which is the leading present difficult to implement in all European countries, we cause of sudden death in an athlete. ECG abnormalities have hope that the successful Italian experience will lead to its wide- also been documented in the majority of athletes who died spread adoption under European regulations." from other arrhythmogenic heart muscle diseases." References Comparisons between findings in Italy where ECG is used 1. The Consensus Statement is a report by the Study Group of and research in the USA showed a similar prevalence of HCM Sport Cardiology of the Working Group on Cardiac Rehabilita- in non-sport sudden cardiac death, but a significant difference tion and Exercise Physiology and the Working Group on Myo- – 2% versus 24% – in sports-related cardiovascular events. cardial and Pericardial Diseases of the European Society of "This suggests we have selectively reduced sports-related Cardiology. sudden death from HCM because our system, using ECG, 2. Cardiovascular pre-participation screening of young competi- identifies vulnerable young people," said Dr Corrado. tive athletes for the prevention of sudden death: proposal for a common European protocol. European Heart Journal. He said that a number of the conditions now being picked up doi:10.1093/eurheartj/ehi108. by ECG had only recently been discovered, so diagnosis was increasing. Researchers would shortly be examining the im- 3. There are no estimates of the prevalence of sports-related pact on mortality of the increased detection of potentially le- sudden cardiac death in European countries apart from Italy, thal problems. where sports-related sudden death occurs in approximately 2 per 100,000 athletes per year. Screening programmes on se- It was harder to detect premature hardening of the coronary lected groups of athletes are used in the majority of European arteries or abnormalities in the coronary artery in young com- countries, but the screening is never systematic as it is in Italy. www.Conge nitalCardiology Tod ay .com © Copyright 2005, Congenital Cardiology Today. All rights reserved
  8. 8. PAGE 8 APRIL 2005 INTERNATIONAL EDITION CONGENITAL CARDIOLOGY TODAY 4. Cardiovascular preparticipation screening of competitive cells, which could augment existing surgical procedures. If athletes. Circulation 1996;94:850-856. the cells maintain pacemaker function when placed in the intact heart, they might serve as biological pacemakers for infants born with heart block, which could also be valu- UCSD Team Discovers Specialized, Rare Heart Stem able." Cells In Newborns, With Potential for Replacing Damaged Tissue After the isl1+ cells were found in newborn rats, the UCSD team used sophisticated genetic methods to tag the pro- The first evidence of cardiac progenitor cells - rare, special- genitor cells in living embryonic tissue and in the newborn ized stem cells located in the newborn heart of rats, mice heart of mice. With these techniques, they were able to and humans - has been shown by researchers at the Uni- show that the isl1+ progenitor cells were spontaneously versity of California, San Diego (UCSD) School of Medi- able to form cardiac muscle tissue. cine. The cells are capable of differentiation into fully ma- ture heart tissue. "Furthermore, the cardiac muscle cells formed were totally mature and had the complete array of function that one Called isl1+ cells, these cardiac progenitor cells are stem would expect in completely differentiated heart tissue," said cells that have been programmed to form heart muscle the study's co-first author Jason Lam, Ph.D. candidate in during fetal growth. Until this new discovery, the cells were the IMM. The cells exhibited contractility, pumping ability, thought to be absent after birth. However, the UCSD team the correct electrical physiology and normal heart structure. discovered a small number of the specialized stem cells In addition, the progenitor cells coupled with neighboring remained embedded in a region of the newborn heart cardiac muscle cells with resulting normal electrical heart called the atrium. They also determined that the cells could beats. be expanded into millions of progenitor cells by growing them on a layer of neighboring heart cells called fibro- "Another important discovery was the ability to expand the blasts. few cells found in a newborn heart, into millions of cells in lab culture dishes," Laugwitz said. "This implies that the Published in the 10 February 2005 issue of the journal Na- isl1+ cells potentially could be harvested from an individ- ture, the research identified the isl1+ progenitor cells in the ual's heart tissue, multiplied in a laboratory setting, then re- tissue of newborn rats and mice, and then in heart tissue implanted into the patient. Furthermore, the developmental taken from five newborn human babies undergoing surgery lineage marker which identifies undifferentiated cardiogenic for congenital heart defects. precursors suggests the feasibility of isolating isl1+ cardiac Study author Sylvia Evans, PhD, a member of the UCSD progenitors from mouse and human embryonic stem cell Institute of Molecular Medicine (IMM) and professor of systems during cardiogenesis." pharmacology, and co-first author Alessandra Moretti, PhD, "We think that these cells normally play an important role in IMM member, explained that the cells are programmed to the remodeling of the heart after birth, when the newborn become spontaneously beating cardiac muscle cells simply heart no longer relies upon the mother's circulation and by exposure to other neighboring heart cells. oxygenation," Chien said. "We believe the isl1+ progenitor Since these rare cardiac progenitor cells are found in re- cells are left over from fetal development so that they can gions of the atrium that are normally discarded during rou- insure the closure of any existing small heart defects and tine cardiac surgery, the discovery raises the possibility the formation of a completely mature heart in newborns." that an individual could receive their own cardiac stem cells The UCSD team noted in the Nature paper that the next to correct a wide spectrum of pediatric cardiac diseases, research steps with the isl1+ cells will be cellular transplan- according to co-first authors Moretti and Karl-Ludwig tation in living animals to study their role in endogenous Laugwitz, MD, a Heisenberg-Scholar of the German Re- repair after cardiac injury. search Foundation. In addition to Chien, Evans, Lauagwitz, Moretti and Lam, "Conceptually, these cells could provide a cell-therapy study authors included Peter Gruber, MD, PhD, Children's based approach to pediatric cardiac disease, which is new Hospital of Philadelphia; Yinhong Chen, MD, PhD, Sarah for cardiology," said the study's senior author, Kenneth Woodard, BS, Lizhu Lin, PhD, and Chen-Leng Cai, PhD, Chien, MD, PhD, director of the UCSD Institute of Molecu- UCSD Institute of Molecular Medicine; Min Min Lu, PhD, lar Medicine. "Traditionally, pediatric cardiologists and car- Department of Medicine, University of Pennsylvania; and diac surgeons have relied on mechanical devices, human Michael Reth, PhD, Max-Planck Institut fur Immunbiologie, and synthetic tissue grafts, and artificial and animal derived Universitat Freiburg, Freiburg, Germany. The research was valves to surgically repair heart defects. While progenitor supported by the National Institutes of Health, the Fonda- cells won't grow a whole new heart, our research has tion Leducq, and the German Research Foundation. For shown that they can spontaneously become cells from spe- more information: h tt p :/ / h ea l t h. u c s d . ed u / n ews /. cific parts of the heart by simple co-exposure to other heart © Copyright 2005, Congenital Cardiology Today. All rights reserved www.Cong enitalCard iolog y Tod ay .com
  9. 9. CONGENITAL CARDIOLOGY TODAY NTERNATIONAL EDITION APRIL 2005 PAGE 9 H I G H L I G H T S FRO M C A R D I O LO G Y 2 0 0 5 : 8 T H A N N UA L P O S T G R A D UAT E C O U R S E I N P E D I AT R I C C A R D I OVA S C U L A R D I S E A S E By Gil Wernovsky, MD Tworetzky (see also the December 2006 in Scottsdale, Arizona (February issue of Pediatric Cardiology Today). 8th-12th, 2006). Later on the opening day, Dr. Ed Walsh On February 17th, a series of plenary Between February 16th and 20th, (Boston) updated the audience on risk lectures were held to review important 2005, The Cardiac Center at The Chil- stratification and current experience topics in genetics (Dr. Betsy dren's Hospital of Philadelphia hosted with ICD therapy for young patients Goldmuntz, Philadelphia), heart failure Cardiology 2005: 8th Annual Post with hypertrophic cardiomyopathy, Ka- (Dr. Robert Shaddy, Salt Lake City), Graduate Course in Pediatric Cardio- tie Do d d s , RN, MSN, PNP coagulation (Dr. David Jobes, Philadel- vascular Disease at Disney’s Yacht (Philadelphia) discussed the current phia), cardiac intensive care (Dr. Sarah and Beach Club Resorts in Orlando, roles and future directions in Florida. advanced practice nursing, Just over 500 professionals in pediatric and Dr. Neil Wilson (London) and adult congenital cardiovascular reviewed the exciting work disease attended the meeting from 45 on percutaneous replace- states and 15 countries, including at- ment of the pulmonary valve. tendees from Japan, Taiwan, Australia, Finally, a series of controver- North and South America, Scandinavia sial topics were presented on and Europe. the future roles of cardiac MRI (Dr. Tal Geva, Boston), At the opening sessions, Professor 3D echo (Dr. Girish Shirali, Robert Anderson (London, England) Charleston), interventional and Peter Gruber, MD, PhD catheterization (Dr. Evan (Philadelphia) discussed “Conception Zahn, Miami) and surgical and Formation”, in which Professor solutions to interventional Anderson used common congenital ‘headaches’ (Dr. Thomas Figure 1. Dr. Edward Bove (left), Dr. Scott Bradley (center) heart examples to review the evidence Spray, Philadelphia). The and Dr. Robert Anderson (right) following the Pro-Con De- based approach to cardiac morphol- evening was capped off with bate. Dr. Bove was also the 6th Annual C. Walton Lillehei ogy. Dr. Gruber, who is both a pediat- Lecturer. a special networking and ric cardiac surgeon and developmental informational session for residents and Tabbutt, Philadelphia) and implantable biologist, spoke about new molecular fellows entering a career in pediatric cardiac support (Dr. Elizabeth Blume, concepts on how the heart forms. cardiovascular medicine. Over 30 Boston). A mini-symposium on issues These opening talks were followed by a young physicians attended this recep- related to patient safety, with sentinel mini-symposium on fetal cardiovascular tion, organized by Dr. Alan Friedman events described by Dr. Peter Laussen disease which featured Drs. Larry Rho- (Yale), Dr. Beth Ann Johnson (Boston) and Dr. James Jaggers des, Jack Rychik, Meryl Cohen and Gil (Milwaukee) and Dr. Geoffrey Bird (Chapel Hill) followed. Later in the Wernovsky (all from Philadelphia) and (Philadelphia). Topics included work- day, breakout sessions were held in Dr. Wayne Tworetzky (Boston). Up-to life balance, academic vs. private prac- electrophysiology, heart failure and -date results on fetal cardiovascular tice cardiology, salaries, benefits and transplantation, echocardiography, physiology during non-cardiac fetal long term career planning….presented cardiovascular nursing, ECMO and surgery were presented by Dr. Rychik, by many of the course faculty. This pediatric perfusion. The highlight of the as well as the exciting information on session will be repeated at Cardiology day was a spirited debate between Dr. fetal cardiovascular intervention by Dr. www.Conge nitalCardiology Tod ay .com © Copyright 2005, Congenital Cardiology Today. All rights reserved
  10. 10. PAGE 10 APRIL 2005 INTERNATIONAL EDITION CONGENITAL CARDIOLOGY TODAY Edward Bove (Ann Arbor) and his for- for the Surgical Management of AV mer trainee, Dr. Scott Bradley Discordant Hearts: Can Two Wrongs (Charleston). The session was moder- Make a Right?” followed by featured ated by Professor Robert Anderson, nursing lecturer Elisabeth C. Smith, and debated the topic: “Center Volume RGN, RSCN (London), speaking on is the Best Indicator of Surgical Re- Contemporary and Collateral Nurse sults.” Dr. Bradley, who took the “con” Leadership in the United Kingdom”. position, informally “won” the debate Finally, Dr. Norman Silverman gave the based upon a poll of the audience par- 4th Annual William Rashkind Memorial ticipants. Lecture on “Ebstein's Anomaly: From the Fetus to the Adult.” Friday, February 18th featured a morn- ing of breakout sessions on interven- The day was rounded out with mini- tional catheterization, cardiovascular symposia on pediatric perfusion, car- nursing, rare cardiovascular diseases, diovascular nursing, and a comprehen- cardiac surgery and anesthesia, outpa- sive review of pulmonary atresia with tient cardiology, and work-hour restric- Figure 2. Dr. Roberto Canessa held the audi- intact ventricular septum. The session ence spellbound during his presentation of tions. Larger sessions were held after featured anatomical review by Dr. Human Groups in Crisis Situations. lunch on “Essential Principles of Inpa- Anderson, imaging by Dr. Friedman, tient Care” and “Data Management, perioperative care by Dr. Wernovsky cial situations. The groups learned Outcomes and Access to Cardiovascu- and Dr . Ch i t r a Ravi shankar quite a bit about the variability in man- lar Services: Past, Present and Fu- (Philadelphia) and diagnostic and inter- agement patterns, and the data gener- ture”. For the first time, a wireless au- ventional catheterization by Dr. ated will be published in a supplement dience response system was used both Jacqueline Kreutzer (Philadelphia). A of Cardiology in the Young late in in the morning for the cardiovascular superb summary of the surgical man- 2005. Dr. Ungerleider then gave in- nursing session, and during general agement options was followed by a sightful comments about team compo- thoughtful review of the literature by sition and factors that build harmony in “Just over 500 Dr. James Tweddell (Milwaukee), and pediatric cardiovascular programs. At emphasized the current limitations in professionals in pediatric the end of the day, Dr. Roberto evidence-based surgical decision mak- and adult congenital Canessa (Montevideo, Uruguay) pre- ing. sented his experience of crashing in cardiovascular disease the Andes in 1972, and his epic strug- attended the meeting from gle to survive over 2 months (which 45 states and 15 countries, was highlighted in the movie: Alive!). including attendees from It was a compelling and moving experi- ence for all 500+ people in attendance. Japan, Taiwan, Australia, Ironically, on the same day that Dr. North and South America, Canessa presented this experience in Scandinavia and Europe.” Orlando, an American hiker in the Andes found the wallet of one of Dr. sessions, moderated by Dr. Ross Canessa’s teammates that had been Ungerleider (Portland). During this buried for over 32 years! session, 4 different viewpoints on the management of hypoplastic left heart Saturday, February 19th featured the syndrome were presented, and difficult named lectures that have been a part Figure 3. Rashkind Lecturer Dr. Norman questions were generated about the of this meeting since shortly after its Silverman (Stanford, left) and featured differences in management options inception. Dr. Edward Bove gave the Nursing Lecturer Elisabeth C. Smith, RGN, presented to families based upon so- Lillehei lecture on “Current Strategies RSCN (London, right). Do You Want to Recruit a Pediatric Cardiologist? Advertise in the only monthly publication totally dedicated to pediatric and congenital cardiology. For more information: Recruit@CongenitalCardiologyToday.com © Copyright 2005, Congenital Cardiology Today. All rights reserved www.Cong enitalCard iolog y Tod ay .com
  11. 11. CONGENITAL CARDIOLOGY TODAY INTERNATIONAL EDITION APRIL 2005 PAGE 11 Dr. Mitch Cohen (Phoenix), Dr. Paul Stephens and Dr. Bernard Clark JUNE CONGRESS FOCUS (Philadelphia), Dr. James Quintensenza and Dr. Michael Parpard, CCP (Tampa), Dr. Elizabeth Blume and Susan M. 8th International Workshop in Fernandes, MHP, PA-C (Boston), and Congenital and Structural Heart Marion McRae RN, MScN, CCRN, CCN Disease (C) (Toronto). The session also in- with Live Case Demonstrations cluded a presentation on administrative and Hands-on Workshops considerations in the delivery of ser- June 16-18, 2005; vices to adults in pediatric hospitals by Frankfurt, Germany Dr. Steven Altschuler (President and w w w. c h d - wor k s h o p . o r g Chief Executive Officer of The The course director is Horst Children's Hospital of Philadelphia). Sievert, MD, PhD of the Cardiovas- cular Center Frankfurt, Frankfurt, Figure 4. Dr. Edward Bove (right) receives the Cardiology 2006 will be held February 8- Germany with co-directors Neil W il- 6th Annual Lillehei Award from Dr. Thomas 12 at the Hyatt Gainey Ranch in Scotts- son, MD of the Royal Hospital for Spray (left), Executive Director of The Cardiac dale, Arizona, and will feature three Center at The Children’s Hospital of Sick Children in Glasgow, UK and broad themes: (1) State of the art man- Philadelphia Shakeel A. Qureshi, MD, Paediatric agement of the neonate and infant with Cardiology of Guy's Hospital in Lon- complex congenital heart disease, (2) Throughout the course, 40 posters of don, UK. There will be a number of Clinical trials in pediatric cardiovascular well-known guest and local faculty. original research were on display, rep- disease, and (3) Ethical issues in the Over the three days there will be resenting work from 28 different cardio- delivery of care to infants with cardiovas- ten major sessions plus step-by- vascular programs, and covering the cular disease. See www.chop.edu/ step live sessions including: disciplines of perioperative care, car- cardiology2006 for details. diovascular nursing, perfusion, imag- • Imaging ing, catheterization, surgery and many For comments to this article, send email to: others. Following a vote by the faculty, APRGW@CongenitalCardiologyToday.com • ASD Closure Dr. Sarah Tabbutt was awarded the ~CCT~ 2nd Annual Outstanding Investigator • Obstruction and Occlusions Award for her work on a contemporary • Step-by-Step and Tips and comparison of shunt type in the surgi- Tricks in PFO and LAA clo- cal reconstruction of hypoplastic left sures heart syndrome. The meeting ended with a terrific mini- • LAA Closure for Prevention of symposium on “The Lost Generation: Cardioembolic Stroke Challenges Facing Teenagers and • Ventricular Septal Defects Gil Wernovsky, MD, FACC, FAAP Young Adults with CHD (and their pro- Staff Cardiologist, Cardiac Intensive viders)”, chaired by Dr. Gary Webb • Complications – Prevention and Care Unit (Philadelphia). The session included Management Director, Program Development; topics such as exercise physiology, the psychological impact of pacemakers The Cardiac Center • And more... Professor of Pediatrics at The and other implantable devices and In addition there will be Hands-on Children's Hospital of Philadelphia long-term outcomes in patients with workshop in the catheterization lab University of Pennsylvania School transposition of the great arteries, at the CardioVascular Center of Medicine tetralogy of Fallot, and various forms of Frankfurt. single ventricle. Presenters included WERNOVSKY@email.chop.edu Get A FREE GOOGLE GMAIL FROM CONGENITAL CARDIOLOGY TODAY Congenital Cardiology Today is offering 500 free GMAIL accounts to subscribers compliments of Google. To get your own account send an email to: rkoulbanis@gmail.com. Include your name, title, organization, address, phone, and email. This offer is only available to the first 500 requests from qualified medical professionals. www.Conge nitalCardiology Tod ay .com © Copyright 2005, Congenital Cardiology Today. All rights reserved
  12. 12. PAGE 12 APRIL 2005 INTERNATIONAL EDITION CONGENITAL CARDIOLOGY TODAY S C R E E N I N G F O R S U D D E N C A R D I A C D E AT H U S I N G T H E P R E - PA R T I C I PA T I O N P H Y S I C A L E X A M By Tiffany J. Riehle, MD and Robert standard PPE is done thoroughly and A local news report of a young, ap- C. Campbell, MD consistently, by qualified individuals, parently healthy child’s death can it should be an effective tool in identi- devastate a community and fill the fying important life-threatening car- pediatrician’s office with worried A pre-participation physical exam diovascular conditions. families. Nearly half of patients who (PPE) is an important, often over- Structural/Functional looked medical tool. Since it is one of the few healthcare contacts a child Hypertrophic Cardiomyopathy (HCM) ♥ or adolescent may have, it should be Coronary Artery Anomalies used as a screening tool to identify conditions that may compromise the Aortic Rupture / Marfan ♥ athlete’s performance or health. 1 Dilated Cardiomyopathy (DCM) ♥ The pediatrician’s role is not only to appropriately clear or restrict young Myocarditis, Endocarditis athletes, but also to identify potential Left Ventricular Outflow Tract Obstruction risk factors for sudden cardiac death in the family. Mitral Valve Prolapse (MVP) Unfortunately, there is variability in Coronary Artery Atherosclerotic Disease ♥ PPE form content and personnel per- Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) ♥ forming the pre-participation exam. In the US, only 40% of states ade- Post-operative Congenital Heart Disease quately screen high school athletes for sudden cardiac death (SCD) and Electrical only 17% of high schools use forms that have all the elements of a car- Long QT Syndrome (LQTS) ♥ diac exam recommended by the AHA2. The 3rd Edition of the PPE Short QT Syndrome ♥ monograph, published in November Wolff-Parkinson-White Syndrome (WPW) 2004 by Mc-Graw Hill, can be pur- chased from any medical website or Brugada Syndrome ♥ bookstore. It was co-authored or Congenital Heart Block sponsored by several organizations: the American Academy of Pediatrics, Catecholaminergic Ventricular Tachycardia ♥ the American Academy of Family Physicians, the American College of Other Sports Medicine, the American Medi- Primary Pulmonary Hypertension ♥ cal Society for Sports Medicine, the American Orthopaedic Society for Drugs, Stimulants Sports Medicine, and the American Commotio Cordis Osteopathic Academy of Sports Medicine1. It is easy to use, inex- ♥ indicate familial or genetic causes pensive, and comprehensive. If a Table 1. Causes of Sudden Cardiac Death. 28th Annual Scientific Sessions and Melvin P. Judkins Cardiac Imaging Symposium May 4-7, 2005, Ponte Vedra Beach, Florida The Society for Cardiovascular Angiography and Interventions www.scai.org © Copyright 2005, Congenital Cardiology Today. All rights reserved www.Cong enitalCard iolog y Tod ay .com
  13. 13. CONGENITAL CARDIOLOGY TODAY INTERNATIONAL EDITION APRIL 2005 PAGE 13 succumb to SCD are asymptomatic or had unappreciated symptoms. The Patient History first sign of a life-threatening disease Have you ever passed out or nearly passed out DURING exercise? is often sudden death. Thus, care Have you ever passed out or nearly passed out AFTER exercise? providers should screen for SCD risk factors at any opportunity, not just at Have you ever had discomfort, pain, or pressure in your chest DURING times of heightened awareness. exercise? Does your heart race or skip beats DURING exercise? SCD occurs infrequently, with a likely underestimated range from 1:100,000 Has a doctor ever told you that you have high blood pressure, high – 1:200,000 athletes each year. 2,3 cholesterol, a heart murmur, or a heart infection? Although rare, 85% of SCD is caused Has a doctor ever ordered a test for your heart (for example, ECG, by primary cardiac diseases. Of echocardiogram)? those, 36% are due to hypertrophic cardiomyopathy and 19% due to “Tell Me About Any Family Members Who. . . “ coronary anomalies, usually left main coronary artery arising for the right Died for no apparent reason (SIDS, car accidents, drowning) sinus of Valsalva. 4 Table 1 lists sev- Has a heart problem eral other important causes of SCD, Died of heart problems or of sudden death before age 50 including structural, functional, and electrical conditions. All of these Has had syncope (fainting) or pre-syncope (nearly fainted) conditions may ultimately lead to ven- Has had unexplained seizures tricular fibrillation and sudden death. Has had significant arrhythmias or a pacemaker As indicated, many of these causes are genetic or familial. Since many Has any of the following genetic disorders: have no discernable physical exam Hypertrophic Cardiomyopathy (HCM) findings, close attention to patient Dilated Cardiomyopathy (DCM) and family history is the only way to Marfan Syndrome raise suspicion of risk for SCD. Ehrlers-Danlos Syndrome A thorough family history, including Arrhythmogenic Right Ventricular Cardiomyopathy source documentation, can be obtained at any time and updated periodically. Early coronary artery disease Often, parents or children will not vol- Coronary Artery Anatomical Anomalies unteer information unless specifically Brugada Syndrome asked. Nearly all Americans believe family health knowledge is important Long QT Syndrome (LQTS) but only 1/3 actively organize a family Short QT Syndrome history. 5 The Surgeon General Primary Pulmonary Hypertension launched the Family History Initiative in November 2004; a computerized tool, found at www.hhs.gov/familyhistory/, Physical Exam: aids in organizing a family history, es- Abnormal blood pressure or pulses pecially during family gatherings where Heart murmur multiple family members are present. Arrhythmias Questions 5-16 of the History Form and May be NORMAL!! Table 4 in the 3rd Edition of the stan- dard PPE monograph relate to cardio- Table 2. Warning Signs for Sudden Cardiac Death. www.Conge nitalCardiology Tod ay .com © Copyright 2005, Congenital Cardiology Today. All rights reserved
  14. 14. PAGE 14 APRIL 2005 INTERNATIONAL EDITION CONGENITAL CARDIOLOGY TODAY impact of a standard, properly con- athletes: clinical, demographic, and ducted PPE on SCD. Although im- pathological profiles. JAMA 1996;276 “In the US, only 40% of (3):199-204. perfect, the AHA considers it the best states adequately screen screening tool available for large 5. Yoon PW, Scheuner MT, Gwinn M, high school athletes for population screening. 2 et al. Awareness of Family Health His- sudden cardiac death Since the prevalence of SCD in the tory as a risk factor for disease – general population is low, screening United States, 2004, MM. (SCD) and only 17% of high schools use forms for SCD is difficult. Routine EKG and For comments to this article, send email to: echocardiograms as screening tools APRTJR@CongenitalCardiologyToday.com that have all the elements are not cost-effective and can lead to ~CCT~ of a cardiac exam misdiagnoses. 2 Many causes of SCD recommended by the evolve over time and patients may be AHA2.” silent carriers; thus, a negative test may not clear a child for life. A stan- dard PPE format can be applied inex- vascular problems. 1 This information pensively for screening at any age; is summarized in Table 2 of this arti- positive findings on family or patient cle. Insight may be gained from history or exam then warrant referral open-ended questions regarding SCD for a more targeted comprehensive warning signs and causes beginning cardiac evaluation. with “tell me about anyone who. . .” Principal Author: High-risk patients include those with A clear, comprehensive PPE exists Tiffany J. Riehle, MD, MSE syncope or chest pain during or after and is the current standard of care. Pediatric Cardiology Fellow exercise, or anyone with certain ge- We advocate the proper use of the Sibley Heart Center at Children’s netic disorders. The AHA recom- 3rd Edition PPE monograph as an Healthcare of Atlanta mends further cardiovascular evalua- excellent, inexpensive first line Atlanta, GA 30329 tion for anyone with a suspected or screening for cardiac causes of SCD detected cardiac disease. Further- in children of all ages. No opportu- riehlet@kidsheart.com more, any unexplained deaths in the nity to screen for families at risk for family should prompt comprehensive SCD should be overlooked. SCD screening for all first-degree relatives. Just one index patient References who had SCD, or who has warning 1. Wappes JR (editor) Pre-participation signs, may uncover an entire family Physical Evaluation 3rd Edition Min- at risk for SCD. Thus, taking extra neapolis McGraw-Hill Healthcare Infor- time and care to perform family and mation, 2005. patient history intake, especially as 2. Seto CK, Pre-participation cardio- Co-Author: part of the PPE, may save many lives. vascular screening. Clinics in Sports Robert C. Campbell, MD, FAAP, Medicine Jan 2003;22(1):23-35. FACC, CMO, In a large retrospective study of SCD Sibley Heart Center at Children’s in athletes, Maron et al. found that 3. Maron BJ, Gohman TE, Aeppli D. Prevalence of sudden cardiac death Healthcare of Atlanta only 3% of the 115 athletes who had Director, Sibley Heart Center undergone a pre-participation exam during competitive sports activities in Minnesota high school athletes. J Am Cardiology Division Director of were suspected of cardiovascular Coll Cardiol 1998;32:1881-4. Cardiology, Dept. of Pediatrics disease. 4 However, the forms or per- Emory University School of Medicine sonnel were not standard. There has 4. Maron BJ, Shirani J, Poliac LC, et been no prospective evaluation of the al: Sudden death in young competitive Ask the Editorial Board Would you like to ask the Editorial Board’s opinion about a procedure, technology or product? Email your question to: ASK@CongenitalCardiologyToday.com Selected questions and answers may be published in upcoming issues. Names will be withheld upon request. © Copyright 2005, Congenital Cardiology Today. All rights reserved www.Cong enitalCard iolog y Tod ay .com
  15. 15. CONGENITAL CARDIOLOGY TODAY INTERNATIONAL EDITION APRIL 2005 PAGE 15 R E S P I R ATO RY F A I LU R E A F T E R I N T R AT H O R A C I C S U R G E RY b) Ventilation perfusion mismatch By Aphrodite Tzifa, MD and Shakeel area of alveoli may reduce efficacy of Qureshi, MD gas exchange. This may include dis- Ventilation and perfusion are not dis- ease of the lung interstitial space, tributed homogeneously throughout alveolar wall or alveolar space such the lung as the gravity effect causes Introduction as pulmonary edema or hemorrhage. increase of both ventilation and per- Involvement of the alveolar air fusion from the top of the lung to the Respiratory failure can be defined as spaces can be focal, and confined to bottom. However, blood flow in- the inability to maintain adequate gas one or two lobes of the lung as in creases more rapidly so that the V/Q exchange to match metabolic require- pneumonic consolidation/atelectasis ratio is inhomogeneous across the ments. It can be characterized by or at the extreme end of the spectrum lung. Optimal gas exchange occurs abnormalities in oxygenation (hypoxic may involve four quadrant lung dis- when ventilation to perfusion match- respiratory failure) or abnormal car- ease as in ARDS. The incidence of ing occurs in every single alveoli. bon dioxide clearance (hypercapneic the latter, following cardiac surgery, respiratory failure). Categorization of In the postoperative patient V/Q mis- respiratory failure into these two match may be due to pulmonary, pul- groups is arbitrary and obtained from “The cause of blood gas monary vascular or cardiac origin. the arterial blood gases. The cause abnormalities following Pulmonary causes of blood gas abnormalities following intrathoracic surgery (ITS) may vary intrathoracic surgery (ITS) During V/Q mismatch areas of the and may be influenced by pre- may vary and may be lung with low V/Q ratios contribute existing lung disease or arise in the influenced by pre-existing blood with an abnormally low PO 2 to perioperative period. lung disease or arise in the pulmonary venous and systemic arterial blood and impair the removal Hypoxic Respiratory Failure the perioperative period.” of carbon dioxide. The diagnosis of hypoxic respiratory Lobar atelectasis is a frequent cause failure is arbitrarily defined in terms is low (0.4%) but carries a high mor- of hypoxia following ITS. In ventilated of arterial blood gas abnormalities tality (15%). Cardiogenic pulmonary patients it most often affects the right when PaO2 <60 mmHg or oxygen edema may resemble ARDS but is upper lobe and usually improves saturations below 90%. Hypoxia may associated with a high pulmonary spontaneously. Risk factors for be caused by loss of alveolar surface wedge pressure (>18 mmHg). Car- atelectasis include use of high in- area (e.g. pulmonary edema, ARDS) diac conditions commonly associated spi r ed o xyg en co nc ent r at i on or abnormalities in ventilation/ with pulmonary edema in the post- (resorption atelectasis), underlying perfusion such as pulmonary embo- operative period include residual lung disease (bronchiectasis, tra- lism and atelectasis. These two VSDs, residual mitral valve stenosis cheobronchomalacia) and anatomical mechanisms frequently co-exist. In or regurgitation, or conditions associ- lesions which obstruct the lumen of addition patients undergoing ITS may ated with excessive pulmonary blood the airways such as vascular rings, have underlying cardiac disease flow such as large aortopulmonary dilated pulmonary arteries and which can lead to hypoxia secondary shunts and inadequate PA banding. enlarged left atrium. to intracardiac or intrapulmonary right Treatment of cardiogenic pulmonary to left shunts. edema involves use of diuretics, ino- Nosocomial pneumonia is a common tropic support and correction of the complication in the patients who have a) Loss of alveolar surface area underlying cardiac anomaly. undergone intrathoracic surgery and Conditions which reduce the surface already have somewhat compromised The Fourth World Congress of Pediatric Cardiology and Cardiac Surgery Buenos Aires, Argentina September 18-22, 2005 www.pccs.com.ar www.Conge nitalCardiology Tod ay .com © Copyright 2005, Congenital Cardiology Today. All rights reserved