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  • P E D I A T R I C 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 P E D I A T R I C C A R D I O L O G Y VOLUME 2, ISSUE 2 WWW.PEDIATRICCARDIOLOGYTODAY.COM FEBRUARY 2004 USE OF THE E XCIMER L ASER IN THE I NITIAL INSIDE THIS ISSUE M ANAGEMENT OF P ULMONARY V ALVE A TRESIA W ITH I NTACT V ENTRICULAR S EPTUM Use of the Excimer 1 mer laser catheter is described in order to Laser in the Initial By Michael C. Slack, MD produce a controlled discrete opening in the Management of imperforate membrane of an atretic pulmo- Pulmonary Valve nary valve as a method of performing suc- Atresia with Intact Introduction cessful balloon valvuloplasty in the initial Ventricular Septum palliative management of this uncommon by Michael C. Slack, MD Lasers (an acronym for Light Amplification condition. Additionally, we will present some by Stimulated Emission of Radiation) from of the basics of the excimer laser and infor- various light sources have been used in mation regarding the effective use of the The Paperless Office 6 medicine for over 30 years. In cardiovascu- recently available and FDA approved small by Warren H. Toews, MD diameter concentric “Extreme” laser cathe- ters. Congenital Heart Block 8 “Further refinements in the in Infants Born to Case Report Mothers With SLE: design of the excimer laser Maternal-Infant catheters, including signifi- A 36 week gestation male infant, birth Autoantibody Markers and Management cantly smaller catheter size Strategy for the coronary and periph- by Z. Zain, MBBS; A. A. Majid; A. Omar, eral vascular markets, have MBBS; C.S. Khuan, made this technology poten- MBBS; and tially useful to the pediatric J. Hassan, MBBS interventional cardiologist.” DEPARTMENTS lar medicine, in the 1980s, various lasers including those from Argon and YAG sources found limited use. The excimer laser (an Medical Conferences 5 acronym for excited dimer), was found useful in the angioplasty of clogged saphenous vein PCT FREE Drawing 5 pediatriccardiologytoday.com grafts.(1) More recently, the excimer laser has been refined for use in various cardio- Useful Websites 9 vascular indications such as coronary artery Associations and revascularization, treatment of In-stent Societies restenosis, thrombosed vessel revasculari- Figure 1. Lateral view of RVOT angiogram zation, peripheral vascular disease, trans- myocardial revascularization, and pace- showing the completely intact atretic pulmo- maker lead removal. Until recently, the use nary valve. Note: Visible rudimentary valve PEDIATRIC CARDIOLOGY TODAY of lasers in the management of congenital leaflets below the intact membrane. 9008 Copenhaver Drive, Ste. M heart disease was limited primarily to pace- Potomac, MD 20854 USA weight 2.8 kg, was evaluated at one day of www.PediatricCardiologyToday.com maker lead revision procedures. age for circumoral cyanosis and a heart mur- © 2004 by Pediatric Cardiology Today. Further refinements in the design of the exci- mur. His clinical evaluation, including a com- Published monthly. All rights reserved. mer laser catheters, including significantly plete transthoracic echocardiogram revealed Statements or opinions expressed in smaller catheter size for the coronary and pulmonary valve atresia with intact ventricu- Pediatric Cardiology Today reflect the peripheral vascular markets, have made this lar septum. The atretic pulmonary valve views of the authors and are not neces- technology potentially useful to the pediatric sarily the views of Pediatric Cardiology morphologically consisted of a completely Today. interventional cardiologist. In this report, the intact membrane without evidence of ante- use of the latest extremely small profile exci- grade flow by color Doppler. There was also
  • PAGE 2 FEBRUARY 2004 PEDIATRIC CARDIOLOGY TODAY Figure 2a. (see text for description) Figure 2b. (see text for description) Figure 2c. (see text for description) Figure 2d. (see text for description) Figure 2e. (see text for description) Figure 2f. (see text for description) a mild to moderate severe Ebstein’s laboratory for pulmonary valve perfora- tion and balloon valvuloplasty. Initial malformation of the tricuspid valve with diagnostic catheterization confirmed mild tricuspid regurgitation (TR). There the diagnosis and angiography in the was a stretched patent foramen ovale “Importantly, there right ventricular outflow tract (RVOT) and a large patent ductus arteriosus demonstrated a completely intact pul- (PDA). A continuous infusion of pros- are serious safety monary valve membrane (Figure 1). taglandin (PGE 1) was immediately concerns and potential The CVX-300 Excimer Laser System instituted. consequences from the (Spectranetics Corporation, Colorado Springs, CO), previously warmed up for At about a week of age, informed con- improper use of the laser 20 minutes, was then employed. A 6 sent was obtained and the infant was taken to the cardiac catheterization equipment...” French FR4 (renal) guide catheter (Boston Scientific Corp., Natick, MA) © Copyright 2004, Pediatric Cardiology Today. All rights reserved www.Ped iatricCard iolog y Today .com
  • PEDIATRIC CARDIOLOGY TODAY FEBRUARY 2004 PAGE 3 vanced into the main pulmonary artery (Figure 2b), down the PDA and into the “Until recently, the use of descending thoracic aorta (Figure 2c). lasers in the management The wire was snared and brought out of a 4 French arterial sheath (Figure of congenital heart disease 2c). A 6mm x 2 cm Symmetry balloon was limited primarily to (Boston Scientific Corp., Natick, MA) was then advanced over the BMW wire pacemaker lead revision and advanced across the valve using procedures.” forward pressure and firm pulling trac- tion from the arterial side of the wire loop (Figure 2d). Two inflations to 8 was advanced over a .035 angled atmospheres were performed (Figure Terumo “glide” wire (Boston Scientific 2e). The wire was then up-sized to a Figure 4. a-side view of the 0.9 Extreme Corp., Natick, MA) into the right ven- .035 wire and two additional inflations excimer laser catheter tip. Spectranetics, tricular outflow tract. Using both an- were performed using 8mm x 1.5 cm with permission giographic and transesophageal echo- Ultra Thin Diamond balloon (Boston cardiographic imaging, the FR4 guide Scientific Corp., Natick, MA) (Figure Initially the infant did well with pulse was manipulated into optimal position 2f). Anatomic separation of the valve oximetry averaging in the low to mid in the RVOT. A Point 9™ Extreme leaflets with good forward flow through 80s. Over the next week, with the PDA (over-the-wire) Excimer Laser catheter now closed, the infant had periods of (Spectranetics Corporation, Colorado significant desaturation. Echocardi- Springs, CO) was advanced over a ography demonstrated a velocity of 0.014 BMW coronary wire (Guidant 3.2m/s across the pulmonary valve with Corp., Santa Clara, CA) through the mild pulmonary insufficiency and 2+ prepositioned guide. The wire and TR. Additionally, significant right to left laser catheter were advanced taking shunting was noted at the atrial level. care to maintain the floppy tip of the Repeat balloon pulmonary valvu- wire beyond the tip of the laser cathe- loplasty was performed with a 10mm x ter at all times. The floppy tip of the 2 cm with reduction of the valve gradi- wire was allowed to form a “u” shaped ent from 34mmHg to 6mmHg. A follow- loop on the underside of the valve up angiogram demonstrated improved membrane while the laser catheter was valve leaflet excursion with excellent advanced and centered on the valve forward flow through the valve. Pulmo- (Figure 2a). Once positioning was con- nary insufficiency increased from mild firmed by TEE and angiographic road- map, the wire was retracted to the tip of the laser catheter and laser energy was applied (45 mJ/mm(2);15 kV;25 Hz). The laser catheter was noted to cross the valve within several seconds Figure 3. Lateral right ventricular an- and the wire was immediately ad- giogram following successful laser perfo- ration and balloon valvuloplasty with 6mm then 8mm diameter balloons. Note the “One drawback is the valve leaflets now separated and in the warm-up time of nearly open position (arrow). 20 minutes for the the valve was visible angiographically console requiring (Figure 3). No change in the degree of Figure 4b-detail of catheter tip showing advanced planning tricuspid regurgitation (2+) was seen the concentric ring of fiber bundles sur- however, negative washout from com- for use of the system.” rounding the wire lumen. Spectranetics, petitive PDA flow was noted. with permission. www.Ped iatricCard iolog y Today .com © Copyright 2004, Pediatric Cardiology Today. All rights reserved
  • PAGE 4 FEBRUARY 2004 PEDIATRIC CARDIOLOGY TODAY predominately left to right shunting. percutaneous revascularization in pa- tient with coronary artery occlusive and Discussion lower limb vascular disease. One Valve perforation in the management of drawback is the warm-up time of nearly intact membranous type of pulmonary 20 minutes for the console requiring valve atresia is a technique which has advanced planning for use of the sys- been in a state of continual albeit slow tem. Importantly, there are serious evolution. The combination of ex- safety concerns and potential conse- tremely low procedural volumes with no quences from the improper use of the specifically designed tools, have pro- laser equipment, therefore all potential vided the interventional cardiologist users must complete a formal training with the opportunity to be creative. course sponsored by Spectranetics Stiff-end wire perforation(2), radiofre- Corporation prior to clinical usage Figure 5. Hole lased in an ex vivo human quency ablation, catheter-assisted per- (contact your local salesperson to ar- tissue sample with the 0.9 mm Extreme range training). foration,(3)(4) and laser catheter- excimer laser catheter (33sec;80mJ/mm2 assisted perforation,(5)(6) have been & 60Hz). Note: well circumscribed discrete described. Reports of the use of exci- edges. Spectranetics. with permission. mer lasers in congenital interventional procedures have been few due in large The recommended CPT Code to 1.5+ and TR remained unchanged. part to the limited stearability and re- The infant improved markedly and was quirement for large sheaths. Recently, by Spectranetics is 35480 discharged from the hospital by three however, due to the introduction of the and a half weeks of age. At six month Extreme Point 9™ line of laser cathe- follow-up, his pulse oximetry is averag- ters from Spectranetics, the potential References utility of this versatile laser catheter has been realized.(7) 1. Litvack F, Grundfest WS, Golden- berg T, Laudenslager J, Forrester JS. The excimer laser uses light in the ul- Percutaneous excimer laser angio- traviolet wavelength (308 nm) pro- plasty of aortocoronary saphenous vein duced as an excited dimer from xenon grafts. J Am Coll Cardiol 1989;14:803- chloride gas. The resulting laser light 808. ablates tissue by delivering extremely short bursts of very high light energy 2. Kuhn MA, Mulla NF, Dyar D, that result in disruption of the carbon to Cephus C, Larsen RL. Valve perfora- carbon double bonds, a process called tion and pulmonary valvuloplasty in an “photo molecular dissociation.” The infant with tetralogy of Fallot and pul- depth of the lesion can be manipulated monary atresia. Cathet Cardiovasc but is usually no more than 100µ deep Diag 1997;40:403-406. per laser application. Ninety-five per- cent of the particles formed from tissue 3. Rosenthal E, Qureshi SA, Chan KC, ablation are < 5 microns in size. The Martin RP, Skehan DJ, Jordan SC, catheters consist of fiber optic bundles Tynan M. Radiofrequency-assisted balloon dilatation in patients with pul- surrounding a central wire lumen run- monary valve atresia and intact ven- ning the entire length of the catheter (Figure 4). In vitro testing confirms that tricular septum. Br Heart J the catheter creates an extremely re- 1993;69:347-351. Figure 6. From: Litvack F, Eigler NL, For- producible and discrete lesion in tissue 4. Wright SB, Radtke WA, Gillette PC. rester JS. Excimer Laser Coronary Angio- (Figure 5). We have found the Extreme Percutaneous radiofrequency val- plasty. Science & Medicine, 1996;3(1):42- Point 9™ laser catheter very user votomy using a standard 5-Fr electrode 51; with permission. friendly and extremely trackable catheter for pulmonary atresia in neo- ing 91% with significantly improved (Figure 6). The CVX-300 Generation 4 nates. Am J Cardiol 1996;73:1370- right ventricular growth. There is a Excimer Laser System from Spectra- 1372. small residual atrial septal defect with netics Corporation is FDA approved for © Copyright 2004, Pediatric Cardiology Today. All rights reserved www.Ped iatricCard iolog y Today .com
  • PEDIATRIC CARDIOLOGY TODAY FEBRUARY 2004 PAGE 5 5. Qureshi SA, Rosenthal E, Tynan M, Sign up for a FREE Anjos R, Baker EJ. Transcatheter la- subscription to MEDICAL CONFERENCES ser-assisted balloon pulmonary valve dilatation in pulmonic valve atresia. Pediatric Cardiology Today Am J Cardiol 1991;67:428-431. ACC '04 (Annual Scientific Session) 6. Redington AN, Cullens S, Rigby and Automatically be Entered March 7-10, 2004, New Orleans, LA MC. Laser or radiofrequency pulmo- in a Drawing for a FREE www.acc.org nary valvotomy in neonates with pul- PedHeart Primer on CD monary valve atresia and intact ven- Electrophysiology in the Young tricular septum: Description of a new Visit and Adults: A New Frontier method avoiding arterial catheteriza- PediatricCardiologyToday.com March 23-25, Riyadh, Saudi Arabia tion. Cardiol Young 1992;2:387-390. www.kfshrc.edu.sa 7. Weber HS. Initial and late results 29th Annual Scientific Meeting - after catheter intervention for neonatal critical pulmonary valve stenosis and 2004 SIR (Society of Interventional atresia with intact ventricular septum: Radiology) "The World of A technique in continual evolution. PedHeart Primer (formerly The New Heart Intervention" Cathet Car diov asc I nt er v en t Journal) is an innovative and easy to follow March 25-30, 2004, Phoenix, AZ 2002;56:394-399. introduction to important CHD topics. Ideal for www.sirweb.org parent and medical personnel education. Nu- For comments to this article, send email to: merous animations, vivid color diagrams, and 39th Annual Meeting - Association FEBMS@PediatricCardiologyToday.com clear, comprehensible text, plus a beautiful new interface. for European Paediatric Cardiology ~PCT~ May 19-22, 2004 - Munich, Germany PedHeart Primer covers: www.i-plan.de/aepc ~ Single Ventricle ~ Atrial Septal Defect 14th World Congress in Cardiac ~ Ventricular Septal Defect ~ Surgical Procedures Electrophysiology and Cardiac ~ Transposition of the Great Arteries Techniques ~ Tetralogy of Fallot June 16-19, 2004, Nice, France ~ Electrophysiology www.cardiostim.fr ~ Fetal Circulation ~ Normal Anatomy 81st CPS Annual Conference ~ Bios of pediatric cardiology pioneers (Canadian Paediatric Society) Michael C. Slack, MD, FAAP, FACC, Drawing Prize is the Courtesy of…. June 16-20, 2004, Montreal, Canada FSCAI www.cps.ca/english/index.htm mslack@cnmc.org Director, Diagnostic & Interventional Society of Nuclear Medicine 51st Cardiac Catheterization Annual Meeting Department of Cardiology June 19-23, 2004, Philadelphia, PA Children’s National Medical Center Visit Scientific Software Solutions website to see interactive.snm.org more about this innovative software: Assistant Professor of Pediatrics The George Washington University w w w . s ci s o f t i n c . c o m / e d u c a t . p h p International Society for Minimally Medical Center Invasive Cardiac Surgery (ISMICS) The Center for Heart, Lung, and Kidney Annual Scientific Meeting Disease at the Children’s National The drawing for two PedHeart Primers will June 23-29, London, UK Medical Center be held by Pediatric Cardiology Today on www.ismics.org 3/24/04 of those qualified individuals visiting w w w . Pe d i a t r i c C a r d i o l o g y T od ay . c om The 2004 "Specialty Review in and signing up for a free subscription to Pediatric Read back issues of Pediatric Cardiology" Course Cardiology Today between 1/15/04—3/14/04. Pediatric Cardiology Today. Winners will be notified by email and listed in the July 12-15, 2004, Chicago, IL www.PediatricCardiologyToday.com April 2004 edition of Pediatric Cardiology Today. www.uic.edu/depts/ci/pcard04 www.Ped iatricCard iolog y Today .com © Copyright 2004, Pediatric Cardiology Today. All rights reserved
  • PAGE 6 FEBRUARY 2004 PEDIATRIC CARDIOLOGY TODAY THE PAPERLESS OFFICE By Warren H. Toews, MD concerns about patient confidentiality, word protected. Our office employees fragility of the Internet connection, and have access only to those parts of the the fate of the records if the ASP goes record that are necessary for their jobs. Compared to the airline and banking out of business. Perhaps the biggest disadvantage is that most of these sys- Currently, in our practice, access is industries, healthcare lags dramatically immediate, typically requiring seconds behind in information management. tems were made for primary care prac- tices, and require quite a lot of adapta- to pull up any of our nearly 10,000 pa- And yet, few of us would dispute the tient records on the computer. Files necessity for readily accessible, com- tion for cardiology practices. are available from either of our two plete medical records in the hospital There are also “stand alone” systems. metro-Denver offices, as well as from and in our offices. In addition, the eco- We chose to use such a Cardiology- our homes (great when on call), and nomic pressures of practice today re- specific product with the file server from any of our seven remote outreach quire greater efficiencies. located in our office. It is much faster sites (via the Internet). Phone calls Fewer than 10% of physicians in the than most Internet connections, has from other physicians or from patients U.S. currently use any form of Elec- the necessary firewalls, and the data is are easily and quickly dealt with. We tronic Medical Record (EMR) genera- appropriately backed up. can search for records by any demo- tion or storage, and fewer than 2% of graphic identifier, by referring physi- After looking at a number of products in practices are completely paperless. cian, by diagnosis, etc. Old habits die hard. AllMeds for Cardiology has a series of The most commonly listed reason for “Fewer than 10% of templates for HPI, PMFSH, ROS, and using an EMR is access to patient re- physicians in the US Physical Exam. Each template has a cords. Traditionally, charts are kept in “drop-down” menu so that the entire currently use any form template can, if desired, be filled out by file cabinets or rooms. They require time to pull, and finding charts can oc- of Electronic Medical mouse clicks. I typically use few of casionally be challenging. Other rea- Record (EMR) generation those, often preferring to use one of a sons to use an EMR include decreased series of standard notes and stored or storage…” outlines I have composed. For exam- dictation time and transcription ex- pense, improved documentation and ple, I have notes for new patients re- E & M Coding, better HIPAA compli- 2000, we chose AllMeds for Cardiol- ferred for murmurs, one for babies and ance, better handling of prescriptions, ogy, one of the specialty-specific prod- one for older children. I simply pull up ucts from AllMeds*. Although originally the note, use my keyboard to make any and easier communication with refer- designed for adult cardiology, it suits necessary changes, and then enter it in ring physicians. our needs quite well, and is easily the appropriate place. I do the same EMR’s come in 3 basic varieties. The modified for our purposes through the for follow-up patients. The record auto- most basic type is a “virtual transcrip- use of standard notes and stored out- matically enters the reason for follow- tionist”, typically using either a menu- lines. up (i.e., diagnosis), and I use a stan- driven keyboard or voice-activated data dard follow-up note that includes the entry system, still requiring that the Our system is a searchable database, date of the last visit, modifying it if nec- report be manually printed, mailed, so that any data entered in any previ- essary for changes in the history. After ous patient visit can be “pulled forward” the first follow-up visit, if the symptom copied and filed. to the current entry, saving time, and status is unchanged, all I need do is For paperless offices, some kind of making for more complete records. We change the date of the previous visit data storage system is required. There only make appropriate changes in the and make any changes in the history. are Application Service Providers data. Our system will perform an E & Prior to implementing the AllMeds (ASP) that maintain records on their M Coding check automatically, allowing EMR, reports required a minimum of 2 website, and physicians enter data us to be sure that our billing is appro- working days to process--from dictation over the Internet. Although these sys- priate. Since all of the records are to mailing. Now, we generate reports tems are fairly inexpensive, there are electronically signed off, they cannot from communications templates in our be altered at a later time. It is pass- © Copyright 2004, Pediatric Cardiology Today. All rights reserved www.Ped iatricCard iolog y Today .com
  • PEDIATRIC CARDIOLOGY TODAY FEBRUARY 2004 PAGE 7 system and fax them to the referring physicians, often directly from the Preliminary EMR Checklist Emergency Medical Records exam room, before the patient leaves. I also provide the parents with a copy of my full report as they leave the of- Access to records Selected List of EMR Companies fice. This has proved invaluable when Compile records *AllMeds patients have to visit an ER when on www.allmeds.com vacation, etc. Legible records Amicore, Inc. I use stored outlines for HPI, Past, Searchable database Family and Social History, ROS and www.amicore.com Physical Exam. These populate the Prescription writing ComChart EMR template with negatives and normals, www.comchart.com Cost savings and I just alter those that are incorrect. After the first visit, I only enter DocuMed changes. Many practices employ a we needed to upgrade some of our www.documed.com scribe, usually a PA, MA, or RN, who hardware. We have experienced a performs the computer data entry while e-MDs small savings compared to using tran- the physician takes the history and www.e-mds.com scription after factoring in costs associ- does the exam. Since the scribe works ated with startup and expansion. A with the same physician every day, he GEMMS larger practice may experience a www.gemmsnet.com or she knows what the physician wants greater financial benefit. in the report. In some practices, this MedInfomatix technique has increased patient AllMeds has a good product. During www.medinformatix.com throughput by up to 30%. the three years of our usage they have demonstrated a commitment to support MEDCOM Our prescription-writing module not and service. Furthermore, the company only checks for allergies and alerts us www.emirj.com has been open to suggestions for soft- to possible drug interactions, but also ware changes. AllMeds for Cardiology NextGen EMR prints the prescriptions, or faxes them has helped improve the quality of our www.nextgen.com based on a built-in pharmacy database. patient records, improved workflow, There are never questions about hand- and most importantly, given us immedi- PowerMed EMR writing. We also can enter any number ate and complete access to our patient www.powermed.com of Patient Information Sheets that we records. can access, display to the patient, and SynaMed print immediately. Any printed data For comments to this article, send email to: www.synamed.com received from other sources is simply FEBWT@PediatricCardiologyToday.com scanned in to the patient file. Our pre- TCAOS ~PCT~ www.tcaos.com vious file clerk is now a scanning clerk. Transcription previously cost our 3- EMR Information Resources physician practice about $4500/mo. Comparatively, our cost for AllMeds’ EMR Comparisons EMR is about $3,000 per month. This www.elmr-electronic-medical-records- includes the software license, support, emr.com training and maintenance, along with a Healthcare Informatics Online (see 3-year equipment lease. After the first year, we have paid about $1000/month Spotlights) for software support and upgrades. We Warren H. Toews, MD, FAAP, FACC www.healthcare-informatics.com incurred some extra expenses with the whtoews@aol.com CTS Software Selection recent opening of our second office in Western Cardiology Associates www.ctsguides.com/medical- Denver. The office required quite a bit Division for Fetal, Pediatric, and Adult method.asp of IT work to get high speed access to Congenital Heart Disease the server from the second office, and www.Ped iatricCard iolog y Today .com © Copyright 2004, Pediatric Cardiology Today. All rights reserved
  • PAGE 8 FEBRUARY 2004 PEDIATRIC CARDIOLOGY TODAY C O N G E N I T A L H E A R T B L O C K I N I N FA N T S B O R N T O M O T H E R S W I T H S L E : M A T E R N A L - I N FA N T A U T O A N T I B O D Y M A R K E R S A N D M A N A G E M E N T S T R AT E G Y By Z. Zain, MBBS; A. A. Majid; A. Omar, of maternal-infant lupus autoantibody ings were recorded to determine atrio- MBBS; C. S. Khuan, MBBS; and markers. ventricular dissociation and estimate left J. Hassan, MBBS ventricular contractility. Patients and Methods Introduction All pregnancies were seen up till term All study infants were born in the single gestation and delivery was planned with The association between maternal sys- centre in which the study was the cardiologist and cardiac surgeon on temic lupus erythromatosus (SLE) and reported. All infants were identified dur- standby. adverse fetal outcome due to the trans- ing fetal ultrasound screening for preg- placental transfer of autoantibodies has nant mothers with SLE between August Infants born were seen immediately by been widely reported. Congenital heart block is one of the manifestations of neo- No Maternal Maternal Baby Type of Heart Block natal lupus syndrome and is a recog- ANA anti-Ro/ anti- La anti-Ro/ anti- La nized cause of morbidity and mortality in infants born to mothers with SLE. 1 Positive Pos / Pos Pos / Pos Complete Heart Block Autoantibodies to SSA/Ro and SSB/La ribonucleoproteins have been demon- 2 Positive Pos / Neg Pos / Pos Complete Heart Block strated almost universally in the maternal circulation when isolated congenital heart block is identified [1]. These are soluble 3 Positive Neg / Neg Neg / Neg Complete Heart Block nuclear (SSA/Ro) and soluble cytoplas- mic (SSB/La) autoantibodies which are 4 Positive Pos / Pos Pos / Pos Complete Heart Block directed against cellular ribonucleopro- tein complexes predominantly found in 5 Positive Pos / Pos Pos / Pos Complete Heart Block patients with sicca syndrome or SLE and is readily identified by the double immu- nodiffuson method [2]. 6 Positive Pos / Pos Pos / Pos Complete Heart Block The presence of these autoantibodies is 7 Positive Pos / Pos Pos / Pos Complete Heart Block strongly associated with neonatal lupus, a disorder considered as a model of pas- sively acquired autoimmunity [3]. Com- 8 Positive Pos / Pos Pos / Pos Junctional Heart Block plete heart block which is a potentially severe and permanent manifestation of Table 1. Maternal-infant lupus autoantibody profile neonatal lupus appearing after the first trimester of pregnancy is an example of 1999 till March 2002. the cardiologists for cardiac assessment. this model. The mechanism of disease is Blood samples were obtained for ANA Connective tissue disease screening dependent on the placental transport of and specific nuclear antigens. including lupus autoantibodies in mothers maternal antibodies (Anti-SSA/SSB) ca- were examined during antenatal check Results pable of causing specific myocardial in- and the extracted nuclear antigen (ENA) flammation that permanently damages screen was performed for positive serum. There were eight patients in this study. the conduction system of the developing There were seven girls and one boy. All fetal heart [4]. Fetal echocardiography was performed patients were diagnosed at the mean periodically and standard views were gestational age of 28 weeks. Fetal echo- We describe the management and out- obtained to identify structure and intra- cardiogram showed normal intra-cardiac come of congenital heart block in infants cardiac connections. Fetal M-mode trac- structures in all patients with bradycardia, of mothers with SLE and the association © Copyright 2004, Pediatric Cardiology Today. All rights reserved www.Ped iatricCard iolog y Today .com
  • PEDIATRIC CARDIOLOGY TODAY FEBRUARY 2004 PAGE 9 dilated and moderate to poorly contract- The implantable pacemakers used in all ing ventricles in seven patients. Fetal patients were from St Jude’s Medical USEFUL WEBSITES cardiac M-mode recorded complete AV (Models Microny™ K SR, Microny™ SR+ Associations and Societies dissociation in these seven patients with or Microny™ II SR+) all of which are mild to moderate degree of pericardial designed to be used in small patients effusion. No treatment in-utero was who needs high base rate pacing HighWire - Library of the Sciences given. (12.8grams, 5.9cc and 6mm thin). and Medicine highwire.stanford.edu In one patient, the fetal bradycardia was One patient with complete AV block had detected during routine ultrasound on the complete spontaneous recovery to sinus Heart Foundation of South Africa mother and investigations revealed that rhythm without treatment. She remains www.heartfoundation.co.za she was positive for SLE autoantibodies well on follow-up. Two patients died. One including anti-Ro/SSA despite not being patient with a fixed junctional block (rate The Hypertrophic Cardiomyopathy having active disease. Only one of the 60 per minute with isoprenaline) died of Association (HCMA) mothers was in active disease at the time hematological complications related to www.4hcm.org/home.php of delivery and she and the baby suc- SLE soon after birth. Another patient died cumbed to haematological complications after pacemaker insertion due to respira- Indian Society of Vascular and of SLE. tory complications at the age of 2 Interventional Radiology (ISVIR) months. www.isvir.org The mean gestational age was 36 ± 4 weeks. Six were delivered by Caesarean Lupus Autoantibody Profile International Academy of section, two vaginally. Mean birth weight Cardiology was 2.65 ± 0.4 Kg. All patients had sur- All mothers (n = 8, 100%), had ANA posi- tive. Seven (87.5%) of the babies had www.cardiologyonline.com face electrocardiogram recording and evidence of transplacental transfer of transthoracic echocardiogram performed. The International Society for lupus autoantibodies in which 6 ( 75%) Seven patients (87.5%) had complete AV mother-baby pairs were positive for the Minimally Invasive Cardiac Surgery block and one had a slow fixed junctional anti-SSA/Ro and anti-SSB/La (Table 1). (ISMICS) rhythm. The mean intrinsic heart rate Two babies were strongly positive for www.ismics.org was 46 ± 13 beats per minute. ANA (> 1:1000), 3 had in addition The Mid-America Interventional anti-sm/RNP, anti-cardiolipin 7 and anti- The mean left ventricular internal dia- Jo1 positive respectively. Radiological Society stolic diameter (LVIDD) was 2.3 ± 0.6 cm www.mirs.org (mean z-score = 1.9 ± 0.4) and the mean Period of follow-up is between 1-3 years. left ventricular ejection fraction was 36 ± All patients with pacemakers are thriving The National Association of 7%. Other findings were pericardial effu- well with satisfactory normal left ventricu- Children's Hospitals and Related sion in the same seven patients (with lar dimensions and function on echocar- Institutions (NACHRI) complete AV block) and valvular pulmo- diogram. None of the patients are on any www.childrenshospitals.net nary stenosis in one patient. cardiac medications. The outcome of patients in this study is shown in figure 1. National Center for Early Six (75%) of the patients underwent ur- Defibrillation gent thoracotomy for epicardial wire in- Discussion http://198.65.239.253 sertion and temporary ventricular pacing within 12 hours of delivery. Two new- Neonatal lupus is characterized by cuta- National Electrical Manufacturers borns had immediate general anesthesia neous lesions, heart block or both [2]. The type of in-utero heart blocks reported Association (NEMA) and epicardial wire insertion performed www.nema.org with the umbilical cord unclamped and range from partial to complete atrioven- tricular block and is due to fibrosis of the National Medical Association placenta not separated until the wires conduction system. Ante-natal detection were secured and external pacemaker (NMA) connected. Patients were then managed has allowed peripartum management www.nmanet.org in the hospital and permanent pace- including use of beta-agonists and ster- maker insertion was performed elec- oids but the mainstay of treatment re- NASPE-Heart Rhythm Society tively. The median time of permanent mains the insertion of single chamber www.naspe.org pacemaker implantation (n=6) was 30 ventricular pacemaker. days (range 18-180 days). www.Ped iatricCard iolog y Today .com © Copyright 2004, Pediatric Cardiology Today. All rights reserved
  • PAGE 10 FEBRUARY 2004 PEDIATRIC CARDIOLOGY TODAY Mothers with SLE are at risk to bear chil- AV block (n=6) had an external tempo- number of babies with similar history dren with congenital heart block. The rary pacemaker with epicardial wires in- were not satisfactory. Transvenous pace- overall risk of giving birth to an infant with serted to the right ventricle by thora- maker wire was inserted either by the congenital heart block, among women cotomy soon after birth. Cardiac decom- femoral vein or umbilical vein route while with probable or definite SLE was 1:60 pensation was determined clinically as awaiting permanent pacemaker insertion but when the woman had anti-SSA/Ro, the presence of signs of poor cardiac which was complicated by infection at the the risk was 1:20 [5]. output. Two newborns had their proce- insertion site or recurrent dislodgment of dure done on a side table to the mothers the lead wire. Patients weighing or had achieved weights of 3.2 Kg and above were sched- uled for permanent pacemaker insertion. n=8 Pacemaker implantation was done in a subcutaneous pocket created in the sub- costal region in all patients and epicardial leads secured to the epicardium adjacent to the right ventricle and allowing excess wire to allow for growth and movement. Junctional Heart Block We found this strategy of staged ap- Complete Heart Block proach to have good outcome albeit n=7 n= 1 (Died) longer stay for the patient in hospital. The experience with the existing patients in this study has allowed us to organize a multi-disciplinary approach in the man- agement of these infants beginning from Paced, n=6 Recovered ante-natal period in which pregnant mothers are counseled by the rheuma- (Died = 1) n=1 tologists and obstetricians. Upon detec- tion of any cardiac abnormalities particu- larly heart block, the pediatric cardiolo- Figure 1. Outcome of patients with congenital heart block gists and cardiac surgeons are then in- volved in the planning of the post-natal with the umbilical cord unclamped with management of these infants. In this study, all infants were detected the placenta intact in the uterus as soon during routine ultrasound check for as the baby was delivered by caesarean Perinatal treatment has not been proven women with SLE who were pregnant. section. The patent flow across the um- to be useful to improve heart rate or ven- These patients were then carefully fol- bilical vessels provided adequate volume tricular contractility except for experi- lowed up till term gestation with pre- and stimulation and both procedures ences in small numbers of patients [14- existing management plans in order for were completed without any complica- 17] and therefore not practiced in our the infant with congenital heart block tions. center. Steroids are used, however, to except for one. In one patient, the mother expedite lung maturity if necessary. had clinical features of SLE including Permanent subcutaneous implantable hematological disease and died post- pacemakers (single chamber) with bipolar Permanent pacemaker insertion is the partum secondary to disseminated in- steroid eluding epicardial leads were definitive treatment for babies with con- travascular coagulation (DIVC). The baby electively inserted in six patients. The genital complete AV block and the ideal had a fixed junctional type of heart block optimal time for permanent pacemaker situation would be to be able to manage of 60 beats per minute despite intrave- insertion was determined by the weight of them through perinatal period and nous infusion of isoprenaline and suc- the baby and this was achieved by estab- achieve a safe delivery. cumbed rapidly due to severe hemato- lishing adequate nutrition and treating Miniaturized permanent pacemakers in logical abnormalities. No pacemaker concurrent infections. this current era has allowed early inser- insertion was attempted. tion and eliminated the issue of the in- Previous management experience in our All babies with decompensated complete institution before this study for a small fant’s size and weight for selected cen- ters. The Barth Syndrome Foundation P.O. Box 974, Perry, FL 32348 Phone: 850.223.1128 info@barthsyndrome.org http://www.barthsyndrome.org Symptoms: Cardiomyopathy, Neutropenia, Muscle Weakness, Exercise Intolerance, Growth Retardation © Copyright 2004, Pediatric Cardiology Today. All rights reserved www.Ped iatricCard iolog y Today .com
  • PEDIATRIC CARDIOLOGY TODAY FEBRUARY 2004 PAGE 11 response to biochemical defined anti- Chow Sook Khuan, MBBS (Mal), MRCP gens of the SSA/Ro-SSB/La system in (UK), M. Med (Mal), AM “Congenital heart block is neonates. J Clin Invest 1989 Associate Professor Aug;84(2):627-34. Consultant Rheumatologist one of the manifestations Division of Rheumatology 6. Ramsey-Goldman R, Hom D, Deng Department of Medicine of neonatal lupus JS, Ziegler GC, Kahl LE, Steen VD, La- University of Malaya syndrome and is a Porte RE, Medsger TA Jr., Anti-SSA anti- chowsk@um.edu.my bodies and fetal outcome in maternal recognized cause of systemic lupus erythematosus. Arthritis morbidity and mortality Rheum 1986 Oct;29(10):1269-73. Jamiyah Hassan, MBBS (Mal), MRCOG Associate Professor, in infants born to ~See author for additional references~ Consultant Obstetrician mothers with SLE.” For comments to this article, send email to: Department of Obstetrics and Gynaecology FEBZZ@PediatricCardiologyToday.com University of Malaya drjay@um.edu.my We have adopted the staged approach ~PCT~ for pacemaker insertion in our infants in view of our limitations and this strategy has shown to have good outcome. Pacemaker Information Conclusion Selected Pacemaker Companies Congenital heart block is a rare but seri- ous fetal adverse disease due to mater- St. Jude Medical, Inc. nal-fetal transmission of anti-SSA/Ro in (+1-651) 483-2000 pregnant mothers with SLE. Anticipation Principal Author: www.sjm.com of this condition and early detection by fetal echocardiography allows an organ- Zarin Zain, MBBS (Malaya) Medtronic Master Medicine (Paeds) (+1-763) 514-4000 or 574-4000 ized multi-disciplinary management with Cert.Cardiology (RCH, Melbourne) a staged approach which promises favor- Lecturer and Cardiologist www.medtronic.com able results. Division of Cardiology BIOTRONIK GmbH & Co. Department of Paediatrics References Faculty of Medicine (+49 30) 6 89 05 –0 1. Tseng CE, Caldwell K, Feit S, Chan University of Malaya www.biotronik.com EK, Buyon JP., Subclass distribution of zarin@um.edu.my Guidant Corporation maternal and neonatal anti-Ro(SSA) and (+1-317) 971-2000 La(SSB) antibodies in congenital heart Aljafri Abdul Majid www.guidant.com block, J Rheumatol. 1996 May; Professor in Surgery 23(5):925-32. Consultant Cardiothoracic Surgeon Selected Resources 2. Reed BR, Lee LA, Harmon C, Wolfe Division of Cardiothoracic Surgery Department of Surgery American Heart Association R, Wiggins J, Peebles C, Weston WL. University of Malaya www.americanheart.org Autoantibodies to SS-A/Ro in infants with aljafri@ummc.edu.my congenital heart block. J Pediatr 1983 American College of Cardiology Dec; 103(6):889-91. www.acc.org 3. Mavragani CP, Dafni UG, Tzioufas Asma Omar, MBBS, MRCP, FRCP Professor in Paediatrics theheart.org AG, Moutsopoulos HM. Pregnancy out- Consultant Paediatric Cardiologist www.theheart.org come and anti-Ro/SSA in autoimmune diseases: a retrospective cohort study. Division of Cardiothoracic Surgery Department of Surgery U.S. Food & Drug Administration Br J Rheumatol. 1998 Jul; 37(7):740-5. University of Malaya (FDA) 5. Buyon JP et al: Acquired congenital asmaomar@um.edu.my www.fda.gov heart block. Pattern of maternal antibody American College of Cardiology Annual Scientific Session 2004 March 7-10, 2004, New Orleans, LA (800) 253-4636, X 694 or (301) 897-5400 w ww . acc.o rg/ 2004an n _meetin g/ ho me/h o me. h tm Crossing Borders in Cardiovasc ular Medicine www.Ped iatricCard iolog y Today .com © Copyright 2004, Pediatric Cardiology Today. All rights reserved
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