Hospital Sultanah Aminah Johor Bahru Pediatric Cardiology Report

Hospital Sultanah Aminah Johor Bahru Pediatric Cardiology Report

Hospital Sultanah Aminah Johor Bahru Pediatric Cardiology Report 2006-­‐2012 Dr Mohd Nizam Mat Bah No 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 Chapters Introduction Organisation and staff In patient services Outpatient sevices Fetal echocardiography services Non invasive investigation Invasive intervention Congenital Cardiac Surgery Congenital Heart Disease Kawasaki disease Rheumatic Fever Paediatric Arrhythmia Training Research Selected Abstract Page 5 6 7 9 11 12 14 15 16 19 20 21 22 23 25 2
Abbreviations AET ALCAPA AMO AP window AR AS ASD AVNRT AVSD BAS BT shunt CAA ccTGA CHD DILV DORV ECG HLHS HOCM HSAJB ICU IJN KD LPA LV MAPCAS MPA MRI MS MV PA PAC PAIVS PAVSD PCCIS PDA PICU PS RF RPA SN SVT TAPVD TGA Atrial Ectopic Tachycardia Anomalous Left Coronary Artery From Pulmonary Artery Assistant Medical Officer Aortopulmonary Window Aortic regurgitation Aortic stenosis Atrial Septal Defect Atrioventricular Nodal reentry tachycardia Atrioventricular septal defect Balloon Atrial Septostomy Blalock Tausig Shunt Coronary Artery Abnormality Conegenitally corrected TGA Congenital Heart Disease Double Inlet Left Ventricla Double Outlet Right Ventricle Electrocardiogram Hypoplastic Left Heart Syndrome Hyperthrophy Obstructive Cardiomyopathy Hospital Sultanah Aminah Johor Bahru Intensive Care Unit Institut Jantung Negara Kawasaki disease Left Pulmonary Artery Left ventricle Major Aorta Pulmonary Collateral A Main Pulmonary Artrery Magnetic Resonance Imaging Mitral stenosis Mitral valve Pulmoanry Atresia Premature Atrial Contraction Pulmonary Atresia With Intact Septum Pulmonary Atresia With VSD Pediatric Cardiology Clinical Information System Patent Ductus Arteriosus Pediatric Intensive Care Unit Pulmonary Stenosis Rheumatic Fever Right Pulmonary Artery Staff nurse Supraventricular Tachycardia Total Anomalus Venous Drainage Transposition Great Arteries 3
TOF VSD VT WPW Tetralogy Of Fallot Ventricular Septal Defect Ventricular Tachycardia Wolf Parkison White 4
1. INTRODUCTION Pediatric Cardiology services in HSAJB started in January 2006. Prior to 2006, the activities were run by the pediatrician with an interest in pediatric cardiology and limited to diagnostic echocardiogram for inpatient and outpatient clinic. The no of patients seen in the clinic is shown in the figure 1. Most of these patients were referred to National Heart Institute (IJN) for further intervention or surgery. Table1: No of patients seen from 1996-­‐2005 Clinic 1996 1997 1998 Cardiology Clinic 1360 1128 1101 ECHO Clinic 228 266 452 1999 960 505 2000 966 525 2001 945 571 2002 1091 784 2003 1188 690 2004 1253 779 2005 1277 843 The main objectives were to provide comprehensive service include in patient and out patient pediatric cardiology services such as diagnostic, surgical and interventional treatment and follow up care for patients with acquired and congenital heart diseases. Despite lack of staff, we managed to build a unit that provides a quality and high standard of care for patients with acquired and congenital heart diseases. These include management of pre and post operative cardiac surgery as well as interventional cardiology. With close collaboration with Departments of Cardiology, Cardiothoracic Surgery and Anaesthesiology, and radiology, the activities have increases tremendously as shown in the timelines below: Timelines 1.
1996 – opening up of Cardiothoracic Centre in HSAJB 2.
1996-­‐2005-­‐running by general pediatrician 3.
2006: Reorganizations of pediatric cardiology clinic and activities 4.
Jul 2006: First Fetal echo service started 5.
Jul 2007: Visiting Clinic to Hospital Sultan Ismail 6.
Feb 2007-­‐First invasive Balloon atrial septostomy (BAS) in HSAJB 7.
Jun 2007: Adult congenital heart disease clinic 8.
Nov 2007: Holter and stress test 9.
Jan 2008-­‐ Paediatric Cardiology Clinic in Batu Pahat Hospital 10.
Jun 2008-­‐First PDA occlusion for pediatric patient in HSAJB 11.
Nov 2009: Cardiac CT angiogram for congenital heart disease 12.
Nov 2010: Paediatric cardiology Clinic in Muar Hospital 13.
Oct 2011: First cardiac MRI on Congenital Heart disease 5
2. Organisation and staff No Positions Name 1 Head Dr. Mond Nizam Mat Bah 2 Pediatric Cardiologist Dr Chan Tee Ling 3 Pediatric Cardiology Trainee Dr Mohd Amin Itam Dr Amelia Alias Dr Muhammad Tamim Jamil 4 Medical Officer: Dr Nizar Kandar Dr Tan Dr Silawati Dr Myat Su Dr Norfadzlin Dr Norhayati Dr Chow Huey Ling Dr Hanizam Dr Intan Raihani Dr Wong Dr Azirawati Dr Adleena Dr Kan 5 Cardiac technician AMO Mohd Afzan Mastuki AMO Mohd Sahrul fazli AMO Mohd Izuan AMO Hafizam SN Zihan 6 Assistant nurse An Salmiah 7 Attendant Mazelah Figure 2.1. The no of staff, position and year of services Year 2006-­‐ current 2007-­‐2009 2008-­‐2010 July 2010-­‐2012 Aug 2012-­‐2013 2006/2007 2006/2007 2008/2009 2008/2009 2008/2009 2009/2010 2009/2010 2009/2010 2009/2010 2012 2012 2012 2013 2007-­‐2009 200-­‐current 2010-­‐2012 2012-­‐ current 2010-­‐ current 2006-­‐current 2011-­‐current Pediatric Cardiology Unit is under the department of pediatric and Dr Mohd Nizam headed the unit since 2006. Currently, there is only only one pediatric cardiologist available as shown in Figure 2.1 and as a training centre, so far, 3 pediatric cardiology trainees had their training done in our unit since 2008. Dr Mohd Amin is cuurently works as pediatric cardiologist in Serdang hospital, and dr Amelia is still in IJN All senior medical officers in pediatric department were posted to pediatric cardiology unit for 4-­‐month duration. During this rotation, they were taught the basic pediatric cardiology as well as basic 2d-­‐echocardiography in CHD and acquired condition. As a new unit in 2006, only one cardiac technician was available in 2007 and currently there were 2 assistant medical officers and one staff nurse. The scopes of the job were varies from basic ECG application, Holter, stress test, echocardiography as well as cardiac catheterisation. 6
3. IN-­‐PATIENT SERVICES There is no dedicated ward for pediatric cardiology patients. All of them were admitted in general pediatric ward, Pediatric ICU, neonatal ICU or special care nursery depending their status and age group. The number of new cases admitted to pediatric ward has increased gradually from 168 to 482 in 2012. The distribution and trend of repeat and new cases from 2006 to 2012 is shown in Figure 3.1. 1200 No of patients 1000 800 600 400 200 0 Repeat 2006 108 2007 153 2008 217 2009 238 2010 296 2011 399 2012 439 New 168 228 231 260 503 629 482 Figure 3.1 No of inpatients from 2006-­‐2012 7
Figure 3.2 show the demographic data of 2501 patient who were admitted to pediatric ward since 2006. Almost two third (1556/2501) of admission were neonates. Meanwhile, an average of 50 cases of cyanotic CHD seen in pediatric and neonatal ward. Demographic Data Total % 2006 2007 2008 YEAR 2009 2010 2011 2012 Sex Female 1151 46.0 84 99 108 133 258 274 195 Male 1350 54.0 84 129 123 127 245 355 287 Race Malay 1646 65.8 113 140 151 177 331 396 338 Chinese 505 20.2 36 61 42 60 90 134 82 Indian 204 8.2 15 17 21 12 50 59 30 Non Malaysian 64 2.6 1 1 2 3 19 22 16 Bumiputera, Sarawak 32 1.3 2 2 5 2 6 7 8 Malaysian, other 28 1.1 1 7 10 5 2 1 2 Orang Asli 12 0.5 1 3 4 4 Bumiputera, Sabah 10 0.4 2 6 2 Age group Neonate 1556 62.2 92 140 137 162 339 407 279 1mth-­‐1yr 562 22.5 40 51 57 61 94 136 123 1-­‐4yr 187 7.5 21 20 14 18 31 36 47 5-­‐9yr 110 4.4 10 9 10 8 26 31 16 10-­‐14yr 71 2.8 5 6 11 8 9 16 16 15-­‐20yr 6 0.2 1 1 2 2 >20yr 9 0.4 1 1 1 2 3 1 Diagnosis Group Normal Heart 989 39.5 22 64 56 76 244 298 229 Left to right shunt 621 24.8 67 60 62 69 115 140 108 Cyanotic lesion 358 14.3 37 41 58 61 52 55 54 Acquired 178 7.1 16 29 13 13 31 39 37 Miscellaneous 149 6.0 13 17 21 11 28 39 20 Obstructive lesion 138 5.5 10 13 19 18 20 36 22 Arrhythmias 43 1.7 2 11 6 19 5 CHD, Other 17 0.7 2 1 2 5 3 4 Cardiac Tumour 8 0.3 1 1 1 2 3 Total 2501 100.0 168 228 231 260 503 629 482 Figure 3.2. The demographic data of 2501 of new patients admitted to pediatric and neonatal ward from 2006-­‐
2012 There were 8 neonatal cardiac rhabdomyomas admitted to NICU with an average 1 cases per year. All except one were diagnosed antenatally and all survived the acute event (see selected abstract) 8
4. OUTPATIENT SERVICES There is no dedicated echocardiography clinic. All patients, either new or repeat had their consultations and 2d-­‐echocardiography at the same setting during the clinic visit. The clinic schedule is on Monday, Wednesday and Thursday from 0830 – 1300hr. Pediatric Cardiology Clinical Information System (PCCIS), which was developed in 2006, were used in the cardiology clinic. All clinical data and echocardiography finding were entered into the system during each clinic visit. The 2d-­‐ECHO report were generated , and parents or patients were given a copy of echo report immediately after the consultations. Two clinics run concurrently at the same time with an average of 25 patients from 8.30am to 1.00pm. The total no of patients seen in pediatric cardiology clinic is shown in figure 4.1, This is handdle by 1 Pediatric cardiologist assisted by 1 pediatric cardiology trainee, 1 medical officer and 2 Assistant medical officer who were trained in echocardiography in CHD. Due to limited no. of pediatric cardiologist, there is no specific clinic for adult with CHD or Pulmonary hypertension clinic or Marfan Clinic. All were seen during the same clinic time. 3500 No of patient 3000 2500 2000 1500 1000 500 0 Repeat New 2006 587 2007 1083 2008 1464 2009 1696 2010 2140 2011 1962 2012 2511 398 609 511 486 637 524 788 Figure 4.1 Total No of out patients from 2006-­‐2012 Figure 4.2 show the demographic data of new patients attending the pediatric cardiology outpatient clinic. Majority of patients (33%) were in infant less than 1 year of age and 5% were more than 15 years of age. 9
Demographic Data Total % 2006 2007 2008 Year 2009 2010 2011 2012 Race Malay 2780 70.3 294 430 344 339 452 374 547 Chinese 753 19.0 80 122 104 95 115 95 142 Indian 269 6.8 17 35 35 35 42 42 63 Malaysian, other 62 1.6 5 15 23 7 8 1 3 Bumiputera, Sarawak 44 1.1 5 4 8 10 5 12 Non Malaysian 25 0.6 2 2 1 2 8 10 Bumiputera, Sabah 14 0.4 1 5 8 Orang Asli 6 0.2 1 2 3 Sex Male 2082 52.7 218 308 272 234 328 282 440 Female 1871 47.3 180 301 239 252 309 242 348 Age Group Neonate 245 6.2 20 19 18 23 41 29 95 1mth-­‐1yr 1325 33.5 146 210 159 178 200 146 286 1-­‐4yr 774 19.6 91 126 96 85 137 100 139 5-­‐9yr 984 24.9 105 178 133 119 160 137 152 10-­‐14yr 425 10.8 30 66 57 52 75 70 75 15-­‐20yr 57 1.4 4 3 16 4 9 10 11 >20yr 143 3.6 2 7 32 25 15 32 30 Diagnosis group Normal Heart 2001 50.6 193 319 222 205 332 281 449 Left to right shunt 1127 28.5 130 166 154 163 171 141 202 Cyanotic 256 6.5 27 33 46 40 36 23 51 Obstructive lesion 240 6.1 26 32 37 35 37 35 38 Acquired 150 3.8 7 27 22 25 30 17 22 CHD, Other 66 1.7 6 14 10 9 12 7 8 Miscellaneous 53 1.3 1 9 10 4 14 6 9 Arrhythmias 53 1.3 7 8 8 5 5 12 8 Cardiac Tumour 7 0.2 1 1 2 2 1 Total 3953 100.0 398 609 511 486 637 524 788 Figure 4.2. The demographic data of 3953 of new patients attending Pediatric cardiology outpatient clinic Half of the patients (50.6%) had a normal echocardiogram. Most were patients with functional murmur, high risk infant, thallasaemia or oncology patient second largest group were patient with left to right shunt. Cyanotic CHD contributes 6.5% of all patients. Hospital Total 2006 2007 Muar Hospital Batu Pahat Total Figure 4.3. Total no of patients seen in distric visit 2008 YEAR 2009 2010 2011 2012 103 136 34 151 143 158 122 87 Figure 4.4 show the no. of patients seen in district hospital. Batu Pahat visting clinic was started in 2007 and Muar Hospital only in 2010. 10
5. FETAL ECHOCARDIOGRAPHY SERVICES Fetal echocardiography clinic was introduced in the late 2008. In the early years, only limited no of echocardiography can be done. However, the number has increased as shown in figure 5.1 with majority maternal diabetic (figure 5.2). The clinic day is only on Friday at 0830-­‐1215 hr. No of patient
140
120
100
80
60
40
20
0
New
Repeat
2008
2
2009
10
2010
12
2011
51
2012
123
1
10
5
9
15
Figure 5.1. The no of patients attending Fetal echocardiography clinic from 208-­‐2012 FETAL echo indication and echocardiography findings. Indication
Total
%
Echocardiography Findings
Normal Heart
CHD
Abnormal, Non
CHD
Undetermined
Diabetic Mother
175
57.8
134
8
6
Abnormal fetal Scan
70
23.1
19
40
7
CHD in previous Child
37
12.2
34
2
Others
13
4.3
12
1
Mother with CHD
8
2.6
7
Total
303 100.0
206
48
16
Figure 5.2 The indication and echocardiography findings in 198 fetal echocardiography clinic from 2008-­‐12 27
4
1
1
33
A total of 303 mothers had fetal echocardiogram. The indications were 175 (58%) maternal diabetic mother and 70 (23%) abnormal fetal scan. The echocardiograhy findings were normal heart (68%) and CHD (16%). Unfortunately due to poor echo window, 33 (10%) were undetermined. Of the 175 echocardiography in maternal diabetic, only 8 (4.6%) had CHD. These finding correlate with other studies in western country. In abnormal fetal scan detected by O&G specialist, 40/70 (57%) had CHD and 19/70 (27%) had a normal finding. 11
6. NON INVASIVE INVESTIGATIONS Variuos non invasive test were offered in pediatric cardiology unit. These include Holter test, cardiac CT, cardiac MRI and stress test as shown in figure 6.1 50 45 40 35 Holter Test 30 CT angio 25 MRI, Cardiac 20 Stress test 15 10 5 0 2009 2010 2011 2012 Figure 6.1 Type of non invasive investigations done in pediatric cardiology unit, HSAJB from 2009-­‐2012 Cardiac CT angiogram was available in HSAJB in 2009. However due to limited experience and slot given (only 2 cases per week), only 108 had CT angiogram. The indication as shown in figrure 6.2 with majority (28%) to delineate pulmonary artery anatomy, 16% to delineate Pulmonary vein and 14% for arch study Indications PA anatomy Study Pulmonary vein Study Arch Study Vascular ring study Pulmonary Hypertension Study MAPCAs Study Coronary Study PDA Study Collateral Study BT Shunt Study Pericardial effusion Aorta Study Total % 31 28.7 18 16.7 16 14.8 12 11.1 11 10.2 5 4.6 4 3.7 3 2.8 3 2.8 3 2.8 1 0.9 1 0.9 Total 108 100.0 Figure 6.2 . The indication for cardiac CT angiogram from 2009-­‐2013 Year 2009 2010 2011 2012 6 2 1 1 1 1 12 4 10 5 4 4 1 2 2 2 34 9 5 6 2 5 3 2 1 1 34 12 3 3 5 1 2 1 1 28 12
Holter services was only started in 2009 after the availability of holter machine. Since then the number of patient varies from 35-­‐40 per year. The main indications for Holter was SVT (20%) followed by chest pain (19%) and heart block (12%). Indication
SVT
Chest Pain
Heart Block
Palpitation
Bradycardia
Syncope
Prolong QT
Post Operative arrhythmias
PVC
Tachycardia
Arrhythmias
WPW
Atrial ectopic
Long QT Syndrome
Abnormal ECG
Screening
Chest Pain and Palpitation
VT
Chest discomfort
Cardiomyopathy
Atrial Flutter/Multiple AET
Atrial Ectopic tachycardia
Broad complex tachycardia
Neonatal Atrial Flutter
PAC
Pacemaker
Sinus bradycardia
HOCM
Total
Figure 6.3. The indication for Holter test N
%
2009
2010
2011
2012
31
29
18
10
10
9
6
6
5
4
4
3
3
3
2
2
2
2
2
2
1
1
1
1
1
1
1
1
161
20.5
19.2
11.9
6.6
6.6
6.0
4.0
4.0
3.3
2.6
2.6
2.0
2.0
2.0
1.3
1.3
1.3
1.3
1.3
1.3
0.7
0.7
0.7
0.7
0.7
0.7
0.7
0.7
106.6
6
5
4
3
5
9
4
5
4
1
2
15
7
3
1
13
6
3
4
5
6
4
1
2
1
3
1
3
2
2
1
1
3
3
1
1
1
1
1
1
1
1
1
2
2
1
1
1
1
1
1
1
1
1
36
1
37
39
49
13
7. INVASIVE INTERVENTION Figure 7 show type of invasive procedures since 2007. The first invasive intervention was balloon atrial septostomy (BAS) which was done at the bedside with echocardiography guidance in PICU in June 2007. Since then, the number of BAS range from 4-­‐10 per year. There was no major complication or mortality associated with the BAS since 2007. Meanwhile PDA ligation with device was started in 2008, and a total of 42 cases were done in 2008-­‐2009. Unfortunately due to lack of pediatric cardiologist and renovation of the cardiac catherisation laboratory, the services were stop temporarily in 2010 and 2011. Another 37 cases of PDA occlusion were done at the end of 2011 an 2012. Of 79 cases who had PDA occlusion , only one required conversion to pda ligation due to large PDA and device embolisation to descending aorta. No mortality or major complication were noted 35
30
25
20
15
10
5
0
2007
2008
2009
2010
2011
2012
PDA occlusion-device
Balloon Atrial Septostomy
Pericardiocentesis_Percutaneous
Transvenous pacing_temporary
Figure 7. The no. of invasive intervetion done in Pediatric cardiology from 2007-­‐2012 Other major intervention such balloon valvuloplasty in neonate or more high risk procedures were not done in HSAJB due to lack of human resources and surgical back up. 14
8. Congenital Cardiac Surgery 100
90
80
70
60
50
40
30
20
10
0
2006
2007
IJN
2008
HSAJB
2009
2010
Others, Overseas
2011
2012
Others, Local
Figure 8.1. The no of congenital heart surgery and place of surgery from 2006-­‐2012 An average of 150 patients had CHD surgery from 2006-­‐2012 at different cardiac centre as shown in figure 8.1 with IJN as the main centre range from 60-­‐90 cases per year. In 2012, a total of 142 patients had congenital heart surgery, which is slightly lower compared with previous year in 2011 (172). Of these 142, 38 (27%) were done in HSAJB and 66% in IJN. In HSAJB, the majority of surgery were PDA ligation in small infant or premature baby with large PDA as shown in figure 8.2 . ProcedureName PDA Ligation ASD Closure VSD closure Pericardiocentesis, surgical VSD closure + PDA ligation PDA Ligation-­‐redo VSD closure + Aortic valve replacement PDA Ligation and amplatzer removal ASD closure + PDA Ligation ASD closure + MV repair Total Total 163 83 28 4 2 2 1 1 1 1 286 2006 20 9 1 30 2007 19 14 33 2008 23 11 1 35 2009 19 13 4 36 2010 24 11 2 2 2 1 42 2011 2012 38 20 14 11 15 6 3 1 1 1 72 38 Figure 8.2 name of procedures according to year Of the 163 PDA ligations, there was only 1 mortality (not cardiac related) and 2 had re-­‐
surgery within 24hr due significant residual and LPA ligation. Meanwhile, of the 83 ASD closure, no mortality were observed and 1 had cardiac tamponade due to large pericardial effusiion after one month surgery. 15
9. Congenital Heart disease (CHD) A total of 2073 CHD detected in children born in state of Johor from 2006-­‐2012 with prevalence of 5.5 per 1000 livebirth (figure 9.1). Figure 9.2 show the distribution of CHD and timing of diagnosis of specific diagnosis of CHD. Three common lesions were VSD, PDA and PS with rate of 34%, 19% and 10% respectively. Meanwhile TOF and TGA were 2 common cyanotic CHD seen in this cohort of children with rate of 5.8% and 2.9% respectively. This distribution is similar as other studies. 2011 2012 Total No of Live birth 52049 53137 53130 51928 54575 54082 No of CHD detected 244 270 280 306 326 339 No of cases/1000 livebirth 4.7 5.1 5.3 5.9 6.0 6.3 Figure 9.1 The no of live birth, CHD and prevalence per 1000 livebirth in state of Johor 55519 308 5.5 374420 2073 5.5 2006 2007 2008 Birth year 2009 2010 Diagnosis N % < 1 mth 1-­‐3 mth Timing of diagnosis 6-­‐
3-­‐6mth 12mth 1-­‐4yr Ventricular septal defect 711 34.3 283 176 117 77 52 Patent ductus asrteriosus 392 18.9 187 76 34 41 52 Pulmonary stenosis 204 9.8 59 65 32 29 19 Tetralogy of fallot 121 5.8 56 18 15 15 17 Atrial septal defect 112 5.4 32 22 16 18 21 Transposition great arteries 61 2.9 51 7 2 1 AVSD 52 2.5 28 6 8 4 6 Coarctation 43 2.1 27 7 4 3 1 Heterotaxia syndrome 36 1.7 22 5 8 1 PAVSD 35 1.7 28 4 2 1 PAIVS 32 1.5 28 1 2 1 DORV 31 1.5 16 7 4 2 2 Tricuspid atresia 30 1.4 19 6 2 2 1 Hypoplastic left heart 25 1.2 22 3 TAPVD 22 1.1 12 7 3 PAVSD, MAPCAs 20 1.0 9 5 1 2 3 Ebsteins anomaly 19 0.9 15 2 1 1 Mitral atresia 18 0.9 13 3 2 Unbalance AV canal 15 0.7 9 6 Truncus arteriosus 14 0.7 12 2 Duoble Inlet LV 14 0.7 13 1 Aortic Stenosis 13 0.6 4 3 5 1 Interupted Arch 11 0.5 9 2 Vascular rings and slings 8 0.4 4 2 2 ccTGA 8 0.4 7 1 Pulmonary Artery abnormality 6 0.3 2 2 1 1 Coronary Artery Fistula 5 0.2 3 1 1 Cor Triatriatum 4 0.2 1 1 2 ALCAPA 3 0.1 1 2 AP window 2 0.1 1 1 Other 6 0.3 2 1 1 2 Total 2073 100.0 973 439 257 209 183 Figure 9.2. The distribution of CHD and timing of diagnosis among 2073 children born in 2006-­‐2012 5-­‐9yr 6 2 3 1 12 16
Timing of diagnosis of CHD Of the 2073 CHD, 1878 (91%) were diagnosed within first year of life with majority (973) detected during neonatal period as shown in figure 9.2. The trend of diagnosis is shown in figure 9.3 with significant increase in neonatal diagnosis from 2009 onwards 250
200
150
100
50
0
2006
2007
< 1 mth
2008
1-3 mth
2009
2010
3-6mth
2011
6-12mth
2012
1-4yr
5-9yr
Figure 9.3. Trend of timing of diagnosis of CHD in children born in Johor State from 2006-­‐2012 Immediate outcome of CHD Figure 9.4 show the immediate outcome of 2073 children with CHD detected in Johor. Of the 2073 CHD, 28% required no intervention and 16% lesions close spontaneously. Meanwhile, 32% already had surgery or intervention. Another 16% still awaiting surgery. Unfortunately due to various factors, 8% were treated conservatively. The overall mortality rate is 12.2% Management N % Alive Corrective surgery or intervention done 660 31.8 599 No surgical or intervention is needed 572 27.6 509 Lesion close or resolve spontaneously 344 16.6 341 Awaiting Surgery or intervention 332 16.0 190 Conservative Management 165 8.0 53 Total 2073 100.0 1692 Figure 9.4 Immediate outcome of CHD in Johor born children from 2006-­‐2012. Final Outcome Died Default fup 58 8 1 92 94 253 3 55 2 50 18 128 Of 660 who had surgery or intervention, 58 (9%) died either within 30 days of surgery or after 30 days of surgery. Of those 332 who were waiting for surgery or intervention, 92 (28%) died for various reasons and 50 (15%) were lost to follow up. 17
Figure 9.5. The severity of CHD among 2073 Johorean children with CHD
Diagnosis
Total
VSD
PDA
PS
TOF
ASD
TGA
AVSD
Coarctation
Heterotaxia syndrome
PAVSD
PAIVS
DORV
Tricuspid atresia
HLHS
TAPVD
PAVSD, MAPCAs
Ebsteins anomaly
Mitral atresia
Unbalance AV canal
Truncus arteriosus
DILV
Aortic Stenosis
Interupted Arch
Vascular rings and slings
ccTGA
Pulmonary Artery abnormality
Coronary Artery Fistula
Cor Triatriatum
ALCAPA
AP window
Mitral Stenosis
Single ventricle, other
Hemitruncus
AV Discordant, VA Concordant
Partial Anomalous Venous Connection
Coronary Artery Anomalies,others
Total
711
392
204
121
112
61
52
43
36
35
32
31
30
25
22
20
19
18
15
14
14
13
11
8
8
6
5
4
3
2
1
1
1
1
1
1
2073
%
Total
CHD
34.3
18.9
9.8
5.8
5.4
2.9
2.5
2.1
1.7
1.7
1.5
1.5
1.4
1.2
1.1
1.0
0.9
0.9
0.7
0.7
0.7
0.6
0.5
0.4
0.4
0.3
0.2
0.2
0.1
0.1
0.0
0.0
0.0
0.0
0.0
0.0
100.0
Severity
Mild
482
217
165
Moderate
79
86
11
85
26
1
2
4
1
1
1
5
3
1
1
4
1
3
1
Severe
150
89
28
121
1
61
47
40
36
35
32
30
30
25
22
20
18
18
15
14
14
5
11
6
8
1
2
4
3
1
1
1
1
1
1
1
964
218
891
The severity of CHD is shown in the figure above Of 2073, 891 (43%) were severe and 964 (46%) were mild lesions. 18
10. Kawasaki Disease (KD) A total of 251 KD were detected with 218 (87%) were complete KD, and 13% incomplete or atypical KD (figure 10.2). Figure 10.1 show the trend of KD over 8 years period. There is slight increase in KD from 2010 onwards with 48 KD in 2012. Of the 251 KD, 20 (7.9%) have some degree of coronary abnormality with majority (13/20) as small aneurysm. However, majority regress with 7 small aneurysm (3-­‐5mm) and 1 giant CAA remain the same as shown in figure 10.3 40
35
30
25
20
15
10
5
0
2005
2006
2007
2008
Complete
2009
Atypical
2010
2011
2012
Incomplete
Figure 10.1. Trend of KD from 2005-­‐2012 Type Complete Atypical Incomplete Total Normal 218 17 16 207 12 12 Coronary Dilatation Small Medium 7 3 3 Giant 4 1 1 1 Total 251 231 13 6 Figure 10.2 Type of KD and coronary artery involvement in 251 children 1 CAA at diagnosis Total Normal Small (3-­‐5mm) Medium (5-­‐8mm) Giant (>8mm) Total 231 13 6 1 251 Normal 228 12 3 243 CAA at latest review Small Medium 3 1 3 7 Giant 1 1 19
Figure 10.3. The immediate outcome of coronary artery abnormality (CAA) in 251 children 11. Rheumatic fever (RF) 10
8
6
4
2
0
2006
2007
RF, MR
2008
RF, No valve
2009
RF, MR and AR
2010
RF, AR
2011
2012
RF, MR and MS
Figure 11.1 Trend of rheumatic fever and valvular involvement from 2006-­‐2012 A total of 69 RF was diagnosed from 2006 to 2012, ranging from 4 cases per year (2012) to 18 cases per year (2010). Of these 69, 25 (36.2%) were severe lesion with mitral regurgitation (73%) as a common lesion as shown in figure 11.2 Severity RF
Total
Valvular involvement
MR only
MR and AR
Mild
35
13
4
Severe
25
16
8
Moderate
9
1
7
1
Total
69
16
3
36
13
Figure 11.2 The severity of RF and valvular involvement in 69 patients with Rheumatic fever No
16
AR only
2
MR and MS
1
1
20
12. Pediatric Arrhythmias Pediatric arrhythmias is uncommon condition and usually due to supraventricular tachycardia (SVT) rather than ventricular tachycardia (VT). In The year 2012, only 12 patients were noted to have arrhythmias compared to 32 cases in 2011. 25
20
15
10
5
0
2006
2007
2008
2009
2010
Dysarrhythmias_supraventricular
Heart Block
Dysarrhythmias_ventricular
Long QT syndrome
2011
2012
Figure 12.1.Trend of Pediatric Arrhythmias from 2006-­‐2012 On average, 15 cases pediatric arrhythmias were detected every year with sudden increase in 2011. Of the 96 patients with pediatric arrhythmias, 58 (60%) were due to SVT as shown in figure 12.2 Diagnosis group Detail Dysarrhythmias_supraventricular Dysarrhythmias_ventricular Heart Block Atrial Ectopic Tachycardia Supraventricular Tachycardia AVNRT Atrial Ectopic Tachycardia, Multiple Atrial Flutter Junctional Rhythm PAC Wolff-­‐Parkinson-­‐White syndrome LV, VT PVC, non neonatal PVC, benign neonatal Heart block, complete Heart block, first degree Heart block, second degree Heart block, second degree, Mobitz 1 Sinus bradycardia Long QT syndrome Long QT syndrome Total Figure 12.2 The detail of arrhythmias in 96 children with arrhythmias Total % 20 19 4 3 3 1 2 6 2 8 2 10 5 2 1 1 20.8 19.8 4.2 3.1 3.1 1.0 2.1 6.3 2.1 8.3 2.1 10.4 5.2 2.1 1.0 1.0 7 7.3 96 100.0 21
13. TRAINING Early detection of CHD with 2d-­‐echocardiogram require good clinical skill among medical staff. As there is only one pediatric cardiologist available, involvement of pediatrician , medical officer and assistant medical officer is needed to reduced the burden. No Year Level No of workshops 1 2009 Hospital 12 sessions (2 hour per sessions) From January –December 2009 2 2011 Hospital 12 sessions (2 hr per sessions) from April to June 2011 3 2011 Southern 2 days region from 13 -­‐ 14/10/2011 4 2012 Hospital 12 sessions (2 hr per sessions) from May 2012 to July 2012 Figure 13.1 The training in 2d-­‐echocardiography from 2009-­‐2011 Total hours 24 hrs 24 hrs 15 hrs 24 hrs In the early stage 2007-­‐2008, one dedicated AMO and two medical officer were trained in echocardiography in CHD. In subsequent year, echo workshops were done on monthly basis as shown above. A total 18 medical officers, assistant medical ofifcer and pediatrcian were trained in echocardiography as shown in Figure 13.2. No Name 1 Dr Mohd Amin Itam 2 Dr Amelia Alias Postion Pediatric cardiologist Pediatric cardiologist Hospital Year HSAJB 2008-­‐2009 HSAJB 2010-­‐2012 3 Dr Fazlida Fauzi Pediatrician HSAJB 2010/2011 4 Dr Nizar Kandar Medical officer HSAJB 2006/2007 5 Dr Tan Medical officer HSAJB 2006/2007 6 Dr Silawati Medical officer HSAJB 2008/2009 7 Dr Myat Su Medical officer HSAJB 2008/2009 8 Dr Norfadzlin Medical officer HSAJB 2008/2009 9 Dr Norhayati Medical officer HSAJB 2009/2010 10 Dr Chow Huey Ling Medical officer HSAJB 2009/2010 11 Dr Hanizam Medical officer HSAJB 2009/2010 12 Dr Intan Raihani Medical officer HSAJB 2009/2010 13 PPP Mohd afzan Mastuki AMO HSAJB 2006/2007 14 PPP Sahrul AMO HSAJB 2008/2009 15 PPP Mohd Izuan AMO HSAJB 2010/2011 16 SN Roziyana Paijang SN Batu Pahat Hospital 2010 17 PPP Hazly Mahadi AMO Muar Hospital 2011 18 PPP Rohaizad AMO Batu Pahat Hospital 2011 Figure 13.2 . Name of medical officers, assistant medical ofifcer and pediatrcian trained in Pediatric 2D-­‐
echocardiography 22
14. RESEARCH No 1 2 3 4 5 6 7 8 9 9 Tittle /Author Detection of Congenital Heart Disease By School Health Programme In In Johor State, Malaysia. Silawati Abd Rashid, Amelia Alias, Mat Bah MN Transient Myeloproliferative Disorder and Large Pericardial Effusion In Neonates: A Case Report. Gopinathan U, Amelia Alias, Mat Bah MN, Acute heart failure in children: Congenital or Acquired ? Mohd Tamim MJ, Mat bah MN Wide complex tachycardia: Ventricular or supraventricular in origin? Mat Bah MN Double Artic Arch: A Rare Causes Of Neonatal Stridor. Amelia Alia, Mat Bah MN A High Frequency Oscillatory Ventilation, Inhaled Nitrous Oxide and Pleurectomy in Severe Perinatal Tuberculosis: A Case Report. Noor Azlina Mustapha, Nisah Abdullah, Mat Bah MN. Abdominal Aortic Thrombosis: A Rare Cause Of Severe Left Ventricular Systolic Dysfunction In NeonatE. Mat Bah MN, Nisah Abdullah , Amelia Alias Fetal And Neonatal Cardiac Tumour: A 5 Year Experience In Hospital Sultanah Aminah Johor Bahru. Amelia Alias, Mat bah MN Coronary Artery Abnormality in Kawasaki disease children treated nd
with IVIG (2 Place for Young investigator Award” ). Amelia Alias, Mat Bah MN Spectrum of CHD in HSAJB. Intan Raihani, Amelia Alias, Mat Bah MN Level (national/international) th
14 Asia Pacific Pediatric Congress 2012 Kuching Sarawak Year Type 2012 Poster 19th Annual Congress Of The Perinatal Society Of Malaysia,2012 2012 Poster Pediatric Cardiology Update, IJN 2012 NHAM-­‐MPCS meeting 2012 2012 Best interesting/challenging case presentaion Best interesting/challenging case th
18 Asean Congress of Cardiology, Cebu, Philiphine 2011 Poster 19th Annual Congress Of The Perinatal Society Of Malaysia,2012 2012 Poster 1 8th Annual Congress of the Perinatal Society of Malaysia, 17th-­‐20th 2011,Penang 2011 Poster 18th Annual Congress of
the Perinatal Society of
Malaysia, 17th-20th
2011,Penang 2011 Poster 32th Malaysian Paediatric Association Congress, 15-­‐
th
17 October 2010, Hilton K 2010 YIA-­‐Oral presentations 2010 Oral presentations 32th Malaysian Paediatric
Association Congress, 1517th October 2010, Hilton
KL 23
10 The outcome and risk factors of surgical closure of ventricular septal defect in young children. Mat Bah MN 6th Johor Scientific Meeting,
Johor Bahru, 21st to 23rd
August 2007 2007 Oral presentations 11 Presentation of Congenital heart Disease. Mohd Nizar Kandar, Mat Bah MN 6th Johor Scientific Meeting, Johor Bahru, 21st to 23rd August 2007 2007 Oral Presentation 24
FETAL AND NEONATAL CARDIAC TUMOUR: A 5 YEAR EXPERIENCE IN HOSPITAL SULTANAH AMINAH JOHOR BAHRU Amelia Alias, Mat Bah MN Paediatric cardiology unit, Paediatric Department ,Hospital Sultanah Aminah Johor Bahru Advances in fetal ultrasonography have improved on the early detection of cardiac tumours. We are reporting a 5 year experience in managing 5 cases of primary cardiac tumours diagnosed in-­‐utero and subsequently confirmed after delivery. The gestation upon diagnosis in these patients was in between 32 to 36 weeks period of amenorrhea. One cardiac tumour was located in the right atrium and required surgical removal to relieve right inflow obstruction. Histopathological findings of this mass confirmed myxoma. 2 neonates have cardiac tumours located in the right ventricle and two others in the left ventricle. Of these 4 tumours, 2 caused significant obstruction to the right ventricle outflow tract. One required surgical removal and the other required prostaglandin infusion (to maintain pulmonary circulation). Spontaneous regression of the tumour occurred in the other three. Tuberous sclerosis was subsequently diagnosed at birth in 2 neonates who has cardiac tumour and subependymal tuber. The other two with cardiac rhabdomyoma are still free of other features of Tuberous sclerosis. None of these infants has known family history of Tuberous sclerosis. In summary, neonatal primary cardiac tumour is uncommon .Tuberous sclerosis is the most common cause. Early detection during pregnancy is feasible with advances in ultrasonography and fetal echocardiography .Thus, help in the future plan for the neonatal management. Prostaglandin infusion in cardiac rhabdomyomas may help in maintaining the pulmonary circulation in tumours with outflow obstruction while waiting for tumour regression. Surgical removal is indicated if the were obstruction of both cardiac inflow and outflow. ABDOMINAL AORTIC THROMBOSIS: A RARE CAUSE OF SEVERE LEFT VENTRICULAR SYSTOLIC DYSFUNCTION IN NEONATE. Nisah Abdullah, Amelia A, Mat Bah MN Department of Paediatric Hospital Sultanah Aminah Johor Baharu Dilated left ventricle (LV) with severe LV systolic dysfunction can be due to either acquired or congenital heart condition such as myocarditis, cardiomyopathy, anomalous origin of left coronary artery and obstruction in the left ventricular outflow tract (LVOT). We describe a rare cause of dilated LV with systolic dysfunction secondary to abdominal aortic thrombosis. A term infant, 2.15kg was ventilated at birth for 6 days for pneumonia and sepsis. An umbilical artery catheter was inserted at day 1 of life and was removed completely due to blockage at day 3. There was no maternal history gestational diabetes. At day 9 of life, persistent respiratory distress with a soft cardiac murmur was detected. A Chest X-­‐ray showed a globular heart with a normal lung field. A 2D-­‐Echocardiography showed dilated left ventricle (LV) with poor systolic function with an ejection fraction of 30%. The coronary artery is normal in origin and there is no LVOT obstruction. However, a large thrombus was detected in the abdominal aorta. There was no clinical sign of arterial obstruction of his lower limbs. Coagulation study was normal. A loading dose of heparin infusion was started immediately and subsequent echocardiography at 10hr after infusion showed improvement of the cardiac contractility and LV size. Heparin infusion was continued and a total disappearance of the thrombosis was evident after 26days of anticoagulation therapy. In summary, abdominal aortic thrombosis is uncommon condition and may present as dilated LV with systolic dysfunction. Prompt diagnosis and treatment is warranted to prevent mortality. 25
TRANSIENT MYELOPROLIFERATIVE DISORDER AND LARGE PERICARDIAL EFFUSION IN NEONATES: A CASE REPORT Mat Bah MN, Gopinathan U, Amelia Alias, Department Of Pediatrics, Hospital Sultanah Aminah, Johor Bahru Pericardial effusion in neonate is an uncommon condition and may be due to infection, malignancy, post cardiac surgery, post central line or umbilical venous catherisation. We describe two neonates with large pericardial effusion associated Down syndrome and transient myeloproliferative disorder. Both presented with severe respiratory distress requiring ventilatory support and chest x-­‐ray showed massive cardiomegaly. Large pericardial effusions were confirmed with 2D-­‐
echocardiogram. One neonate requires transcutaneous pericardial drainage for severe cardiac tamponade. Steroid therapy was started in both cases and responded well to the treatment. Even though pericardial effusion is rare in neonate, early intervention is requires to avoid mortality. If pericardial effusion associated with Down syndrome and transient abnormal myelopoesis, steroids treatment is helpful. DETECTION OF CONGENITAL HEART DISEASE BY SCHOOL HEALTH PROGRAMME IN IN JOHOR STATE, MALAYSIA Silawati Abd Rashid, Amelia Alias, Mat Bah MN Pediatric Department, Hospital Sultanah Aminah, Johor Bahru, Johor , Malaysia OBJECTIVE: School Health Programme (SHP) in Malaysia was establish to promote health in children. It also serves to detect potential morbidity such as congenital heart disease (CHD) and malnutrition. A study yet to be done to analyse the efficacy of this programme in detecting CHD. We studied the usefullness of this programme in detecting CHD and the predictors for CHD. METHODS: This is a retrospective review of all school children refered by SHP to Pediatric Cardiology Clinic for cardiac assessment from 1st January 2009 to 31st December 2011. All patients have full cardiac assessment including ECG, CXR and 2d-­‐echocardiography. Data was extracted from Pediatric Cardiology Clinical Information System. RESULTS: A total of 2855 patients were refered for cardiac assessment with 167 (5.8%) from SHP. Of these 167, 161 (96.4%) were refered for cardiac murmur. However our assessment showed 54% have functional murmur and 19.4% have no murmur. There were 1173 CHD during the study period with 32 (2.7%) came from SHP [30 acyanotic and 2 cyanotic lesions]. Four cases of significant arrythmias were also detected from SHP. Univariate analysis showed that presence of pathological murmur, abnormal ECG and CXR are significant predictors for CHD. Binary logistic regression showed abnormal ECG as an only significant predictor for CHD OR 0.09 95% CI [0.02-­‐0.34], p< 0.001. CONCLUSION: School Health Programme detects 2.7% of newly diagnosed CHD. Significant number were referred for functional murmur. Abnormal ECG findings is a significant predictor for CHD in patients referred by SHP. Keywords: School Health Programme, Congenital Heart Disease 26
SPECTRUM OF CONGENITAL HEART DISEASE IN HOSPITAL SULTANAH AMINAH JOHOR BAHRU 1
2
1
1
Amelia Alias, Chan TL, Mohd Amin Itam, Mat Bah MN 1
Paediatric Cardiology Unit, Paediatric Dept., Hospital Sultanah Aminah Johor Bahru, 2
Gleneagles Hospital, Kuala Lumpur OBJECTIVE: Congenital heart disease (CHD) is the commonest form of congenital malformation. Studies have reported right sided heart lesion is more common than left heart lesion in Asian population. None of these studies, however, came from Malaysia. Our study is looking into the spectrum of CHD in our center. st
METHODS: This is an observational cross sectional study of all newly diagnoses CHD from 1 January 2006 to th
30 July 2010. All data were entered into Pediatric Cardiology Clinical Information System. The diagnosis of CHD was made with 2D-­‐Echocardiogram and coded with International Pediatric Congenital Cardiac Code (IPCCC). RESULTS: A total of 3525 patients were referred for cardiac assessment with 1517 (43%) were CHD patients. Of these 1517, 54.2% were female, 6.7% premature infant and 75.6% less than one year of age. There were 61.6% left to right shunt, 23.3% cyanotic (86 TOF) and 14.8% obstructive lesion (34 Coarctation). Using IPCCC, there were 45.7% septal defect with 498 VSD, 21.9% right heart lesion with 168 PS, 19.6% thoracic arteries and vein with 245 PDA, 4.7% single ventricle with 23 isomerism, 3.3% transposition with 38 D-­‐TGA and only 2.1% left heart lesion with 15 HLHS. There were 192 (12.7%) duct dependent lesion with 143 (74.5%) duct dependent pulmonary circulation. Six hundred and sixty one (43.5%) lesions were considered severe lesion with 123 were large VSD. CONCLUSIONS: The spectrum of CHD seen in our unit is comparable with previously reported studies. Our study also supports that right heart lesion are more common than left heart lesion in Asian population. CORONARY ARTERY ABNORMALITY IN COMPLETE KAWASAKI DISEASE CHILDREN TREATED WITH IMMUNOGLOBULIN 1
2
1
1
Amelia Alias, Chan TL, Mohd Amin Itam, Mat Bah MN 1
2
Paediatric Cardiology Unit, Paediatric Dept., Hospital Sultanah Aminah Johor Bahru, Gleneagles Hospital, Kuala Lumpur OBJECTIVE: Intravenous immunoglobulin (IVIG) therapy in Kawasaki disease (KD) has been shown to reduced coronary artery aneurysm to 5%. However, we still observed significant number of coronary aneurysm post IVIG in our centre. The objectives are to study the prevalence of coronary artery abnormality (CAA) and associated risk factors. st
th
METHODS: A retrospective study performed on all children with KD from 1 January 2005 to 30 July 2010. Japanese Ministry of Health criteria were used to classify coronary arteries abnormality. Children with incomplete or atypical KD were excluded. Data were extracted from Pediatric Cardiology Clinical Information System RESULTS: A total of 126 KD were diagnosed during the study period with 69% were male and 52.4% were Chinese. The median age of diagnosis was 1.4yr [Q1, 0.6yr Q3, 2.3yr]. Of these 126, 118 (93.7%) received IVIG within 10 days of illness. Ten patients (7.9%) required more than one dose of IVIG. CAA were noted in 28 (22.2%) of patients with 21 ectasia, 4 small fusiform, one small saccular and 2 medium fusiform aneurysm. Of these 28, 22 who had IVIG within 10 days of illness (18 ectasia, 3 small and one medium coronary aneurysm). Significant risk factors for CAA were older children (2.3 v s 1.7yr, p=0.03), presentation after 10days of illness (p=0.006) and required more than 2gm/kg of IVIG (p=0.04). CONCLUSIONS: The prevalence of CAA in complete KD treated with IVIG was 22.2% with 5.5% significant aneurysm. Risk factors for coronary abnormality were older children, late presentation and require more than 2gm/kg of IVIG 27
PREVALENCE OF LATE DIAGNOSIS OF DUCT DEPENDENT AND CYANOTIC CONGENITAL HEART DISEASE IN HOSPITAL SULTANAH AMINAH JOHOR BAHRU 1
2
1
1
Amelia Alias, Chan TL, Mohd Amin Itam, Mat Bah MN, Paediatric Cardiology Unit, Paediatric Dept., Hospital Sultanah Aminah Johor Bahru 2
Gleneagles Hospital, Kuala Lumpur OBJECTIVE: Late diagnosis of duct dependent and cyanotic congenital heart disease (CHD) may have significant impact on the outcome. However, there is no study in Malaysia looking at this issue. Therefore we studied the prevalence of late diagnosis of this lesion in our centre. st
METHODS: This is an observational cross sectional study of all newly diagnosed CHD seen from 1 January th
2006 to 30 July 2010. The diagnosis of CHD was made with 2D-­‐Echocardiogram. All data were entered into Pediatric Cardiology Clinical Information System. Non Malaysian Children were excluded. RESULTS: A total of 1517 CHD patients were seen during the study period, with median age of diagnosis of 90days [Q1 13days Q3 350days]. There were 192 (12.7%) duct dependent lesions with 74.5% were duct dependent pulmonary circulation. The median age of diagnosis of duct dependent lesion were 4.5days [Q1 1day, Q3 23days]. Meanwhile there were 352 (23.2%) cyanotic lesion with 86 (24.4%) were Tetralogy of Fallot. The median age of diagnosis of cyanotic CHD were 12days [Q1 1day, Q3 93days]. There were 39 (11%) cyanotic CHD diagnosed after 1 year of life with 20 Tetralogy of Fallot. Nineteen (9.8%) duct dependent were diagnosed after 3 month of life (3 each for severe coarctation, PAVSD and severe pulmonary stenosis, 2 each for isomerism, mitral atresia, tricuspid atresia, PAIVS, DORV with pulmonary atresia) CONCLUSION: There were significant numbers of late diagnosis of cyanotic and duct dependent lesion with prevalence of 11% and 9.8% respectively. Further study is needed to understand the reason behind this. 1
SHOCK AND SEVERE ABDOMINAL DISTENSION IN INFANT: A LESSON LEARNT MB Mohd Nizam, YJ Wong Department of Paediatrics, Hospital Sultanah Aminah Johor Bahru Kawasaki disease (KD) is one of the most common vasculitides in childhood seen in our day to day practice. It is diagnosed when they fulfill the typical diagnostic criteria for Kawasaki disease. A 2 year old boy presented with fever for 5 days associated with severe abdominal distension with haemodynamic compromise required fluid resuscitation. Abdominal X-­‐ray showed markedly dilated loop of bowel suggestive of intestinal obstruction. He was treated as septicemia shock with intestinal obstruction and was kept nil by mouth and IV antibiotics. As he had features of Kawasaki disease; rashes on the trunk and limbs, erythematous and edematous hands and feet, cracked red lips, non purulent bilateral conjunctivitis and cervical lymphadenopathy, differential diagnosis of Kawasaki was made. However, as his fever settled within 48hr of antibiotic, and marked improvement of his clinical status, IVIG was not given. Furthermore, 2d-­‐
echocardiogram showed “normal” coronary arteries. At 16 days of illness, his bilateral conjunctivitis remains, and platelet was rising. A repeat ECHO showed uniformly dilated LMCA and LAD. A diagnosis of atypical Kawasaki was made and treated accordingly. Retrospective reviewed of his early echo showed features of early changes of coronary abnormality. A 2d-­‐
echocardiography at 2years post Kawasaki showed a regression of the aneurysm. Conclusions: KD may present with shock and abdominal distension. In the presence of other features of KD, and early changes of coronary abnormalities on 2d-­‐echocardiography, IVIG should be given to avoid coronary aneurysm. Keywords: Kawasaki disease; abdominal distension; septicemia shock; 2d-­‐echocardiography; coronaryaneurysm 28
WIDE COMPLEX TACHYCARDIA: VENTRICULAR OR SUPRAVENTRICULAR IN ORIGIN? Dr Mohd Nizam Mat Bah Pediatric Department, Hospital Sultanah Aminah Johor Bahru Wide complex tachycardia is commonly due to ventricular tachycardia. However it can be due to supraventricular tachycardia with aberrant conduction. Differentiation and management of these 2 condition is difficult due to it rarity in pediatric population We described a 2 month old boy who presented with heart failure and hemodynamic compromised. His initially ECG showed broad complex tachycardia and was given multiple synchronized cardio version without any success. The tachycardia was only control with Amiodarone and propranolol. Despite 2 antiarrhytmias agents, he still had frequent broad complex tachycardia and only control after the introduction of sotolol. In conclusion, wide complex tachycardia is not always due to ventricular tachycardia. A-­‐ 12 lead ECG recording is essential in differentiating other causes of wide complex tachycardia and help in further management 29