Transcript 婦產部院際部際聯合討論會 April, 11, 2007

婦產.小兒.心臟外科.聯合討論會
May, 25, 2007
07:20
Opening
洪正修主任
07:20-07:35
Case Presentation (mother)
黃本湘醫師
Image studies (sonography)
陳志堯醫師
Case Presentation (fetus)
陳浚銘醫師
07:35-07:50
07:50-08:10
Review of Articles
- Genetics of TGA
張家銘醫師
- Review of TGA
陳浚銘醫師
Open Discussion
婦產部
小兒部
心臟外科
08:10
Closing Remarks
洪正修主任
婦產.小兒.心臟外科.聯合討論會
報告：黃本湘 醫師
指導：洪正修 主任
General data
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Age: 31 year-old, G2 P0 AA1
L.M.P.: 2006-06-20
E.D.C.: 2007-03-28
Prenatal condition: referred from LMD of preg. 24
weeks
Pre-pregnancy body weight: 54kg
Menarche: 13 years-old
Menstrual D/I: 3-4/28-30 days, dysmenorrhea (-)
Oral pills or IUD: denied
BMI: 20.1
General data
General data
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Marital status: married
Previous pregnancy history
No.1, 27 y/o, AA < 5 weeks
Personal history
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Systemic disease: denied
Surgery history: fibrocyst, right breast s/p
operation in 1993
Smoking / Alcoholism: denied
Allergy: denied
General data
Family history
Father is a victim of HBV carrier with liver
Cirrhosis
Traveling history in recent half year
Italia and Singapore
Clinical course
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The prenatal condition was followed up at
LMD till pregnancy 24 weeks while fetal
cardiac anomaly was noted.
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2006-12-07: visited CV OPD Dr. Jiang
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2006-12-11: referred to OBS OPD Chief
Hong.
Clinical course
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Survey for GDM:
50 gm OGT: 174 gm/dl
100 gm OGT: 75/164/140/124 gm/dl
HbA1c: 4.5
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VDRL: negative
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Rubella IgG: positive
Clinical course
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The amniocentesis on 2006-12-11
- chromosome karyotypes:46,XY,9qh+ with
normal variant
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The sonography on 2006-12-15:
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singleton, AGA, male, internal os: 13mm,
polyhydramnios(126 x 70mm), r/o TGA and
TV defect
Hospital course
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The patient was ever admitted for tocolysis and antibiotics
treatment due to preterm contraction and urinary tract
infection (urine routine: pyuria: 2+) from 2006-12-15 to
2007-01-02.
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The sonographic studying (with color doppler) on 2007-0201, 2007-03-01, and 2007-03-08 showed AGA, with normal
flow pattern, r/o TGA and TV defect.
The sonographic studying (with color doppler) on 2007-0315 showed r/o TGA with minimal TR and pericardial
effusion 1.7mm, reverse flow of DV.
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Hospital course
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The Cesarean section was arranged on 200703-21 due to the pregnancy 39 weeks fetus
with transposition of great artery and
pericardial effusion.
09:21, Boy, BL: 50 cm, BW: 3108 gm,
Apgar score: 6 at 1' and 9 at 5'
Hospital course
Apgar score while performing baby care
心跳
呼吸
肌肉
張力
刺激
反射
膚色
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△
○
○
0
1
△
2
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△○
△○
△- 1 minute, ○- 5 minutes
Hospital course
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The boy baby was transferred to NICU for
further management due to progressive
cyanosis and bradycardia at DR.
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The condition after the C-section was fair
and the patient was discharged on 2007-0327.
OBS SONOGRAPHY
General history
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病歷號：34785xx0
姓名：倪xx之男
住院日期：20070321
住院號：11121xx8
身份性別：M
生日：20070321
Chief complaint
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D-TGV was diagnosed by fetal
echocardiography when GA 24 wks
Present illness
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Mother didn't have any major systemic disease
before
Denied any problem during pregnancy
Regular fetal sonography revealed D-TGA when
GA 24 wks
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C/S was performed at 09:21 on 3/21
Bradycardia, cyanosis after delivery
Ambu-bagging
 Vital signs stable
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Transfer to NICU
Brief history
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Birth history
G2P1A1
 GA:39weeks
 BBW:3108gm
 A/P:6->9, by C/S
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Family history
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Grandmother: lymphoma
Physical examination
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身高:50cm(75 percentile), 體重:3.108kg(50 percentile)
Vital signs: T:35.2℃, P:140/min, R:54/min, BP:56/18mmHg
GENERAL
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HEAD
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no nasal flaring
THROAT & MOUTH
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Eardrum: injected(-)
NOSE
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grossly normal, no icteric sclera
EARS
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grossly normal, no trauma wound
EYES
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General appearance: moderate-developed (+) ,well-nourished (+)
injected throat(-)
NECK
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supple, no lymphadenopathy, no jugular vein engorgement, no meningeal sign
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SKIN
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CHEST & LUNGS
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no tenderness, no pits
EXTREMITIES
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Inspection: no distention, no striae
Percussion: no shifting dullness, mild tympanic sounds
Palpation: no rebound pain
BACK & SPINE
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regular heart beat, no murmur
ABDOMEN
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symmetric expansion, no chest deformity, bilateral clear breathing sound
HEART
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intact, pink
freely movable, no pitting edema, no clubbing finger
NEUROLOGI
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sucking reflex(+), Moro reflex(+), grasp reflex(+)
Hospital course
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EKG
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NSR, RAD
CXR, 3/21
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Echocardiography
Simple D-TGA, no VSD
 No PS or AS
 PDA & ASD vs PFO /c bidirectional shunt
 r/o single intramural coronary artery (RCA from
main LCA)
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WBC
: 16.3 X 1000/UL
RBC
: 4.42 M/UL
HGB
: 15.5 G/DL
HCT
: 46.5 %
MCV
: 105 FL
MCH
: 35.0 PG
MCHC
: 33.3 G/DL
RDW-SD : 13.8 FL
PLATELET : 282 X 1000/UL
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Na
K
Bun
Cr
ALT
AST
142
4.4
7
0.7
6
24
MMOL/L
MMOL/L
MG/DL
MG/DL
U/L
U/L
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Impression
Simple D-TGA
 PDA & ASD vs PFO /c bidirectional shunt
 r/o single intramural coronary artery
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Plan
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PGE1(20ug/amp)
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0.03ug/kg/min
Arrange cath
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Cyanosis, mild poor activity, 1250, 3/21
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VBG:
PH: ( 7.150 ->7.169)
 PO2: ( 36.0 ->29.5 ) mmHg
 PCO2: ( 52.8 ->59.7 ) mmHg
 HCO3: ( 18.0 ->21.2) mmol/L
 BE:
( -11.1 ->-8.1) mmol/L
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s/p N-P /c IMV
 Sod. Bicarbonate, 3+3cc
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Keep PGE1 1.5md/min(0.03ug/kg/min)
N-P with IMV->N-P->room air, 3/25
Cath, 3/27
Simple D-TGA
 PDA & ASD
 Single coronary artery, r/o intramural ca
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Plan
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Consult CVS for arterial switch (Jatene procedure)
Op day, 96/4/2
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Operative findings
D-TGA + intact ventricular septum
 large ASD + large PDA
 two coronary arteries
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1L + 2RCx combination
Operative procedure
Arterial switch operation
 ASD repair
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Follow up echocardiography
TGA s/p Jatene operation
 Mild PS, TR
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MBD on 96/4/17 due to stable condition
Review of Articles
TRANSPOSITION OF
THE GREAT
ARTERIES
Cytogenetic Lab. 張家銘
Frequency of cardiovascular
malformations in first-degree
relatives of probands with TGA
Overall recurrence risk in sibs = 0.82% ( 5 in
612)
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Becker et al. (1996)
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levo-TGA (19) 1/50 sibs
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dextro-TGA (168) 1/369 sibs and 1 father
had levo-TGA
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complex TGA (65) 4 /143 sibs and 2/143
parents
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asplenia with TGA (19) 1/50 sibs,
mutation CFC1 gene mutation
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7-year-old girl with D-TGA and mental retardation
tandem duplication of the exon 4 splice donor site of the
CFC1 gene
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Goldmuntz et al. (2002)
THRAP2
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t(12,17)(q24.1;q21),
breakpoint on
chromosome 12
thyroid hormone
receptor–associated
protein
early embryonic
development in
various organisms
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97 D-TGA for mutations in THRAP2
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6 intronic polymorphisms
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6 silent mutations
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4 missense mutations; disease causing,
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glu251gly (608771.0001)
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arg1872his (608771.0002)
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asp2023gly (608771.0003)
Muncke et al. (2003)
Discussion
報告者：陳浚銘 醫師
指導者：黃碧桃 醫師
Background
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First described over 2 centuries ago
surgical atrial septectomy in the 1950s
balloon atrial septostomy in the 1960s
physiological repair (atrial switch operation)
anatomic repair (arterial switch operation).
survival rate > 90%
What is TGA?
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Abnormal development of the fetal heart during
the first 8 weeks of pregnancy
Aorta arises from the right ventricle and the
pulmonary artery arises from the left ventricle
anatomic classifications of TGA
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dextro-TGA [d-TGA] :
60% of the patients, the aorta is anterior and to the
right of the pulmonary artery
 (arteries change)
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levo-TGA [l-TGA] :
the aorta may be anterior and to the left of the
pulmonary artery
 (ventricles change, circulation is normal)
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One third of patients with TGA, the coronary
artery anatomy is abnormal
left circumflex coronary arising from the right coronary
artery (22%)
 single right coronary artery (9.5%)
 single left coronary artery (3%)
 inverted origin of the coronary arteries (3%)
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Symptoms & signs
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newborn cyanotic
first hours : 50% TGA
 first days : 90% TGA
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rapid breathing
rapid heart rate
heart murmur
cool, clammy skin
Treatment
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Prostaglandin E1 :
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keep the ductus arteriosus from closing
Cardiac catheterization
as a diagnostic procedure, and evaluate the defect(s) and
the amount of blood that is mixing.
 Rashkind balloon atrial septostomy
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Surgical treatment
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Arterial Switch Operation
(Jatene procedure)
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first week or two of life
Surgical treatment
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Senning or Mustard procedure
creates a tunnel (a baffle) between the atria
redirects blood flow
Prognosis
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dependent on the associated anatomic
malformations and the conduction system
abnormalities
overall survival rate following arterial switch
operation is 90%
Thanks for your
attention!!