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Case Presentation
Thoracic and Cardiovascular surgery department, SMC
2007313075 Son Eui Young
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Chief Complaint
 M / 2개월 11일
 Abnormal echocardiography result
- Onset : 2012-04-03
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Present illness
 2개월 남아, 31+0wk, 1.69kg, syphilis mother's baby, C/sec d/t PPROM
and breech presentation, Apgar score 2/2으로 출생
 출산 후 시행한 TTE 상 VSD 및 ASD 소견 관찰
 이에 대해 further evaluation 및 management 위하여 본원 NICU 전원
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Other Histories
 Development: normal
 Surgical history: none
 Current medication: none
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Review of System
 특이사항 없음
 Abdominal pain (-)
 Anorexia/Nausea/Vomiting(-/-/-)
 General Weakness(-)
 Constipation/Diarrhea(-/-)
 Fever/Chill/Night sweat(-/-/-)
 Melena/hematochezia/ hematemesis (-/-/-)
 Headache/Dizziness(-/-)
 Dysuria/Frequent voiding(-/-)
 Visual disturbance/ocular pain(-/-)
 Red urine/Foamy urine(-/-)
 Sore throat/Rhinorrhea/Sneezing(-/-/-)
 Arthralgia/Myalgia (-/-)
 Cough/Sputum (-/-/-)
 Morning stiffness(-/-/-)
 Dyspnea/Hemoptysis (-/-)
 Easily bruisilibility (-)
 Chest pain (-)
 Palpitation (-)
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Physical Exam
 Vital sign
 Chest
- BP 110/70, HR 94, RR 16, BT 36.3℃
- Symmetric expansion
- SpO2 94%
- chest wall retraction (+) : subcostal
 General appearance: Acute ill-looking
- Vesicular Breath sound s wheezing
 Mental state: alert & well oriented
- Lower Left sternal border, pansystolic grade 3
- GCS (E 4, V 5, M 6)
 Head and Neck
- Pinkish Conjunctiva/ unicteric Sclera
- Isocoric Pupil size, prompt light reflex at both eyes
- Murmur (+)
- Subclavicular node / Axillary node ( - / - )
 Abdomen
- Liver palpation( - )
- Spleen palpation ( - )
- Nystagmus ( - )
- Otoscopy (정상)
- oral ulcer ( - ), tongue dehydration ( - )
- Tonsilar hypertrophy( - ), Pharyngeal injection( - )
 Skin
- Rash/Purpura/erythema (- / - / - )
- Cervical LNE( -);
- Thyroid enlargement ( - )
- Carotid bruit Rt/Lt ( - / - )
- Paranasal tenderness ( - )
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Echocardiogram
 2012-04-30, #18
 1) Large PMOE VSD (9.6mm) with left-to-right dominant
bidirectional shung
 2) Large secundum ASD (8.8 x 7.4 mm) with left-to-right
shunt
 3) dilated RA & RV
 4) no PDA, no CoA
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Initial Lab
 CBC
8180 - 9.1 - 278k
 CRP
0.03
 T-bil/AST/ALT/ALP
0.8/23/11/▲399
 BUN/Cr
8.5/0.19
 e’
141 - ▲5.3 - 103
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Problem List
 #1. Preterm AGA
 #2. Low birth weight infant
 #3. Syphilis mother's baby
 #4. Abnormal echocardiographic finding including VSD
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Assessment
 #1, #2, #4
- VSD (SA, small to moderate)
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Plan
 VSD closure with bovine pericardium
 ASD primary closure
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Operation
 Operation
- VSD closure with bovine pericardium
- ASD primary closure
 수술 후 진단명
- VSD (PMOE, large)
- ASD (secundum, large)
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Hospital Course
 POD #1
- vital sign stable
- pain tolerable
- wound clear
- no immediate complication
 POD #5
- extubation done
 POD #12
- C-line remove
- 퇴원 고려
 POD #14 (2012-06-12)
- Vital sign stable
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Disease Review
Ventricular Septal Defect
2007313075 Son Eui Young
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Introduction
 1 in 1000 live births
 선천성 심질환 중 가장 흔하다. (단독으로는 25%)
 50% associated with other congenital malformations
 First described by Roger in 1879, hence small VSDs are also known
as the ‘maladie de Roger’.
 First VSD closed under direct vision by Lillehei in 1955
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Anatomic Classification of VSD
 Perimembranous: 80%
 Subarterial: 14%
 Muscular VSD : 10%
Figure 117–4 Classification of ventricular septal defects (VSDs):
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Commonly Associated Defects
 Patent ductus arteriosus (PDA) :25%
- Diagnostic difficulties:preop.echo, TEE etc
- should be ligated or clipped
 Coarctation or Aorta : 10%
- Augmented L-R shunt
 left ventricular outflow tract obstruction(Congenital valvar or
subvalvar aortic stenosis): 4%
 large atrial septal defects (ASDs), right ventricular outflow tract
obstruction, vascular ring, and persistent left superior vena cava.
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Pathophysiology
 Shunt Direction and Magnitude
- depend on the size of the defect and the pressure gradient
- Qp/Qs
 Small size
- RV의 수축기압을 의미있게 증가시키지 못하는 작은 결손
- 폐동맥혈류 증가가 심하지 않음 (Qp/Qs < 1.75)
- 폐동맥고혈압 없음
 Large size : 결손 크기가 aortic valve ring 보다 클 때
- 폐혈관저항 증가 (Qp/Qs > 2)
- 우심실과 좌심실간의 압력차가 소실되거나 역전되어 L-R shunt와 R-L shunt 가
같이 생김
- 청색증, 객혈, 심부전증 등이 나타나 사망할 수 있음
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Complication
 Growth failure
 Congestive heart failure (left heart
failure)
 Pulmonary vascular disease :as
Eisenmenger syndrome or
complex
 Severe illness with viral or
bacterial pneumonia
 Infective endocarditis
 Acquired left ventricular outflow
tract obstruction
 Aneurysm of the ventricular
septum
 Paradoxical emboli
 Sudden death
 Heart block secondary to
intracardiac repair
 Aortic regurgitation
 Impaired left ventricular function in
some patients
 Stenosis in the right ventricular
outflow tract
 Increase in weight following VSD
closure
 Discrete fibrous subaortic stenosis
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Diagnostic work-up
 Symptoms
- tachypnea, growth failure, profuse sweating during feeding, a bulging
precordium, a pansystolic murmur, an enlarged liver, and thready pulses
 the physical examination, chest radiograph, and electrocardiogram
(ECG)
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VSD Natural Course
Spontaneous closure or decrease of size
 Perimembranous or muscular type
 Aggressive medical management
cf. Malalignment type, SA, MO, Endocardial cushion type
 Usually within 6-12 Mo of age
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VSD Natural Course
Eisenmenger or Severe PHT
 Usually after 1 yr of age
 Contraindications for op d/t PHT
- PVR > 8-10 Wood unit
- No response to pulmonary vasodilators
such as O2 or nitric oxide
- Mainly R-L shunt or no L-R shunt through VSD
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VSD Natural Course
AV Deformity
 Mainly subarterial type
Some of PM type
MO VSD
 Increased incidence of infective endocarditis if there is AR
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VSD Natural Course
Subaortic stenosis
 Usually discrete membrane type
- Occasionally tunnel type
 Op indications
- AV deformity
- PG > 20-30 mm Hg
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Treatment of VSD : Indications
 Approximately 30% of infants
- surgery within the first year of life
 Significant shunt
- Medically uncontrolled CHF
- PA pressure > 1/2 of systemic artery pressure
- Shunt amount; Qp/Qs > 1.5 or 1.7
 Other problems
- DCRV, Subaortic stenosis, AV deformity
 Consider natural course, patient’s age
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Treatment of VSD : depends on size
 Large VSD
< 3 months – CHF, failure to thrive
 Elective repair at 6-12 months (PVR < 8.0 units)
 Small VSD (Qp:Qs<2:1)
Endocarditis
Cardiac Enlargement
Any Aortic Incompetence
 Subarterial (supracristal) VSD - any size, operate early
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