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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 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 Marital status: married Previous pregnancy history No.1, 27 y/o, AA < 5 weeks Personal history 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 The prenatal condition was followed up at LMD till pregnancy 24 weeks while fetal cardiac anomaly was noted. 2006-12-07: visited CV OPD Dr. Jiang 2006-12-11: referred to OBS OPD Chief Hong. Clinical course Survey for GDM: 50 gm OGT: 174 gm/dl 100 gm OGT: 75/164/140/124 gm/dl HbA1c: 4.5 VDRL: negative Rubella IgG: positive Clinical course The amniocentesis on 2006-12-11 - chromosome karyotypes:46,XY,9qh+ with normal variant The sonography on 2006-12-15: - singleton, AGA, male, internal os: 13mm, polyhydramnios(126 x 70mm), r/o TGA and TV defect Hospital course 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. 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. Hospital course 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 △ △ ○ ○ 0 1 △ 2 ○ △○ △○ △- 1 minute, ○- 5 minutes Hospital course The boy baby was transferred to NICU for further management due to progressive cyanosis and bradycardia at DR. The condition after the C-section was fair and the patient was discharged on 2007-0327. OBS SONOGRAPHY General history 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 D-TGV was diagnosed by fetal echocardiography when GA 24 wks Present illness Mother didn't have any major systemic disease before Denied any problem during pregnancy Regular fetal sonography revealed D-TGA when GA 24 wks C/S was performed at 09:21 on 3/21 Bradycardia, cyanosis after delivery Ambu-bagging Vital signs stable Transfer to NICU Brief history Birth history G2P1A1 GA:39weeks BBW:3108gm A/P:6->9, by C/S Family history Grandmother: lymphoma Physical examination 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 HEAD no nasal flaring THROAT & MOUTH Eardrum: injected(-) NOSE grossly normal, no icteric sclera EARS grossly normal, no trauma wound EYES General appearance: moderate-developed (+) ,well-nourished (+) injected throat(-) NECK supple, no lymphadenopathy, no jugular vein engorgement, no meningeal sign SKIN CHEST & LUNGS no tenderness, no pits EXTREMITIES Inspection: no distention, no striae Percussion: no shifting dullness, mild tympanic sounds Palpation: no rebound pain BACK & SPINE regular heart beat, no murmur ABDOMEN symmetric expansion, no chest deformity, bilateral clear breathing sound HEART intact, pink freely movable, no pitting edema, no clubbing finger NEUROLOGI sucking reflex(+), Moro reflex(+), grasp reflex(+) Hospital course EKG NSR, RAD CXR, 3/21 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) 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 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 Impression Simple D-TGA PDA & ASD vs PFO /c bidirectional shunt r/o single intramural coronary artery Plan PGE1(20ug/amp) 0.03ug/kg/min Arrange cath Cyanosis, mild poor activity, 1250, 3/21 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 s/p N-P /c IMV Sod. Bicarbonate, 3+3cc 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 Plan Consult CVS for arterial switch (Jatene procedure) Op day, 96/4/2 Operative findings D-TGA + intact ventricular septum large ASD + large PDA two coronary arteries 1L + 2RCx combination Operative procedure Arterial switch operation ASD repair Follow up echocardiography TGA s/p Jatene operation Mild PS, TR 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) Becker et al. (1996) levo-TGA (19) 1/50 sibs dextro-TGA (168) 1/369 sibs and 1 father had levo-TGA complex TGA (65) 4 /143 sibs and 2/143 parents asplenia with TGA (19) 1/50 sibs, mutation CFC1 gene mutation 7-year-old girl with D-TGA and mental retardation tandem duplication of the exon 4 splice donor site of the CFC1 gene Goldmuntz et al. (2002) THRAP2 t(12,17)(q24.1;q21), breakpoint on chromosome 12 thyroid hormone receptor–associated protein early embryonic development in various organisms 97 D-TGA for mutations in THRAP2 6 intronic polymorphisms 6 silent mutations 4 missense mutations; disease causing, glu251gly (608771.0001) arg1872his (608771.0002) 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 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? 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 dextro-TGA [d-TGA] : 60% of the patients, the aorta is anterior and to the right of the pulmonary artery (arteries change) levo-TGA [l-TGA] : the aorta may be anterior and to the left of the pulmonary artery (ventricles change, circulation is normal) 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%) Symptoms & signs newborn cyanotic first hours : 50% TGA first days : 90% TGA rapid breathing rapid heart rate heart murmur cool, clammy skin Treatment Prostaglandin E1 : 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 Surgical treatment Arterial Switch Operation (Jatene procedure) first week or two of life Surgical treatment Senning or Mustard procedure creates a tunnel (a baffle) between the atria redirects blood flow Prognosis dependent on the associated anatomic malformations and the conduction system abnormalities overall survival rate following arterial switch operation is 90% Thanks for your attention!!