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Title:

Surgery for Congenital Heart Diseases

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... not indicated Congenital Aortic Stenosis The various forms of ... LV hypoplasia) and obstructive types are supravalvular, valvular, subvalvular ... – PowerPoint PPT presentation

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Date added: 27 September 2019
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Title: Surgery for Congenital Heart Diseases


1
Surgery for Congenital Heart Diseases
  • By Dr. Shkar R. Saeed

2
Etiologic Basis of Congenital Heart Diseases
  • 1. Primary genetic factor (10)
  • 1) Chromosomal 5-10
  • 2) Single mutant gene 3
  • Recessive
  • Dominant
  • 2. Genetic-environmental interaction (90)
  • 1) Multifactorial inheritance
    majority
  • 2) Risks to offspring of an affected parent
  • 3) Environmental contribution
  • Drugs
  • Infections
  • Maternal conditions

3
Potential Cardiovascular Teratogens
  • 1. Drugs
  • Alcohol
  • Amphetamines
  • Anticonvulsants
  • Chemotherapy
  • Sex hormone
  • Thalidomide
  • Retinoic acid
  • 2. Infections
  • Rubella
  • Coxsakie virus
  • 3. Maternal conditions
  • Old age
  • Diabetes
  • Lupus
  • Phenylketonuria
  • 4. Others

4
Maternal Risk Factors
  • Factors Malformation
  • Advanced age Trisomy 21
  • Maternal CHD Various
  • Diabetes mellitus VSD, TGA,
    cardiomyopathy
  • SLE Heart
    block
  • Phenylketonuria TOF, VSD, COA, HLHS
  • Viruses
    Teratogenic, myocarditis
  • (cytomegalovirus,
  • herpes, coxsacki B,
  • parvovirus)

5
Maternal Drug Exposures
  • Drug Malformation
  • Diphenylhydantoin PS, AS
  • Trimetadione VSD, TOF, TGA, HLHS
  • Thalidomode TOF, Truncus arteriosus
  • Lithium Ebstein anomalies
  • Alcohol VSD, ASD, PDA, TOF
  • Amphetamine VSD, ASD, PDA, TGA
  • Birth control pills VSD, TOF, TGA

6
classification of Congenital Heart Diseases
  • 1. Lt to Rt Shunt ( 53 )
  • PDA 17
  • ASD 16.5
  • VSD 13
  • AVSD 3.5
  • 2. Rt to Lt Shunt (11 )
  • TOF 4.5
  • TA 3
  • PAVSD 2.5
  • PAIVS 0.5
  • 3. Admixture Lesion ( 15 )
  • TGA 5
  • Univ. Ht. 5
  • DORV lt 2
  • Truncus 0.8
  • Corrected TGA lt 0.5
  • 4. Obstructive Lesion ( 15 )
  • Coarctation 9.5
  • PS 2
  • MS etc. 1.5
  • LVOTO 1.3
  • HLHS 0.9
  • IAA 0.6
  • 5. Valvular Lesion
  • Ebstein lt 1
  • AR lt 0.5
  • MR lt 0.5
  • 6. Miscellaneous
  • Arrhythmia 5
  • Vascular ring 0.5

7
Evaluation of CHD by History Taking
  • 1. Infants
  • 1) Murmur
  • 2) Symptoms of CHF
  • poor feeding,
  • low weight gain,
  • tachypnea, tachycardia,
  • sweating, anxiety,
  • irritability, frequent URI
  • 3) Symptoms of hypoxemia
  • cyanosis, hypoxic spell
  • 2. Children
  • 1) Murmur
  • 2) Symptoms of CHF
  • exercise intolerance,
  • dyspnea on exertion,
  • frequent URI,
  • palpitation
  • 3) Syncope, chest pain
  • 4) Symptoms of Hypoxemia
  • cyanosis,
  • hypoxic spell,clubbing

8
Investigations
  • 1.CXR
  • Integral part of evaluation
  • TOF(boot shaped heart)
  • TGA(egg on side)
  • Dilated PA (PHTN)
  • 2.Echo(TTE,TEE)
  • 3. MRI
  • 4. Cardiac catheterization(exact anatomy, PA
    pressure messurements,Qp/QA messure, occluders
    and dilators

9
To Be Corrected in Neonate
  • Critical AS
  • Hypoplastic left heart syndrome
  • Interrupted aortic arch
  • Symptomatic COA
  • TGA
  • Truncus Arteriosus
  • Other symptomatic complex heart diseases

10
To Be Corrected in Infancy
  • Cardiac anomalies with pulmonary outflow
  • tract obstruction
  • Critical PS
  • Tricuspid atresia
  • TGA
  • TOF
  • PA with or without VSD
  • Corrected TGA

11
Surgical Indications and Optimal Timing of
Operation
12
Palliative Surgery
  • Systemic pulmonary artery shunt
  • Blalock-Taussig shunt
  • Cavopulmonary shunt (BCPS)
  • Hemifontan Fontan procedures
  • RVOT reconstruction
  • Valvotomy
  • Patch widening
  • Valved conduit
  • Pulmonary artery banding
  • Atrial septectomy

13
SystemicPulmonary Artery Shunt ( QpltQs
i.e cyanosis)
  • Systemicpulmonary artery shunt is indicated due
    to age, size, anatomy or
    other conditions when
  • Complex anomaly with severe cyanosis,
    irritability, hypoxic episode
  • Critically ill neonates or infants due to
    decreased pulmonary flow
  • Facilitating growth of hypoplastic pulmonary
    artery

14
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15
Pulmonary Artery Banding QpgtQs i.e more Pulm.
flow
  • Pulmonary artery banding is indicated to decrease
  • pulmonary blood flow protect vascular
    disease when
  • Control of congestive heart failure
  • Complex or multiple VSD (/- coarctation)
  • CPB medically contraindicated
  • Protection of pulmonary vascular bed

16
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17
Atrial Septectomy
  • For the increasing of effective pulmonary
  • flow and systemic oxygen saturation
  • Indication of atrial septectomy
  • TGA
  • Tricuspid atresia
  • Pulmonary atresia
  • MV and LV hypoplasia
  • Decreasing tendency of indication due to early
    total correction or intervention

18
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19
Reparative Surgery
  • Non-open heart surgery
  • Open heart surgery
  • Palliative procedure
  • Corrective procedure
  • Anatomic correction
  • Physiologic correction

20
Non-open Heart Surgery
  • Corrective procedure
  • PDA
  • COA
  • Vascular ring and sling
  • Coronary artery anomalies
  • Stenotic valvular diseases
  • Instrumental dilatation

21
Patent Ductus Arteriosus
  • Open communication usually between upper
    descending Aorta and proximal portion of LPA
  • Significant PDA indicated after 1st month
  • Prophylactic closure 6-12 months
  • Symptoms of heart failure or failure to thrive
    indicated at any time
  • Severe pulmonary vascular disease contraindicated

22
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24
Coarctation of the Aorta
  • Congenital narrowing of upper thoracic aorta
    adjacent
  • to the ductus arteriosus
  • Operation is indicated when
  • Reduction of luminal diameter gt 50
  • Upper body HT gt 150mmHg in young infant
  • With CHF at any age
  • COA with VSD
  • COA with other important intracardiac defects
  • One stage repair

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28
Open Heart Surgery
29
ASD with or without PAPVR
  • A hole of variable size in the atrial septum and
    is
  • most common cardiac malformation with various
  • location of defect, fossa ovalis, posterior,
    ostium,
  • primum, coronary sinus, subcaval (sinus venosus)
  • Uncomplicated ASD or of PAPVC with RV volume
  • overload (Qp/Qsgt1.5 or 2.0) is an indication of
    surgery.
  • Optimal age under 5 years but recently 1-2
    years to avoid RV volume overload

30
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32
Total Anomalous Pulmonary Venous Connection
  • These are no direct connection between any
  • pulmonary vein and the LA. But rather, all the
  • pulmonary veins connect to the RA or one of its
  • tributaries
  • Diagnosis is an indication of operation
  • Immediate repair with Diagnosis in any ill
    neonate
  • Preoperative preparation is not needed
  • Repair should be done nearly always before 6
    months
  • Diagnosis at 6-12 months prompt repair is
    indicated

33
Ventricular Septal Defect
  • A hole (or multiple holes) between Lt Rt
    ventricle
  • Symptomatic large VSD an indication of
    operation
  • Before 3 months indicated in large VSDs with CHF
    or respiratory symptoms
  • Moderate sized VSDs (Qp/Qs lt 3.0) with few
    symptoms observation during infancy
  • Small VSDs (Qp/Qs lt 1.5)
  • not indicated, risk of bacterial endocarditis
  • Subarterial type early repair is indicated
    before childhood

34
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36
Atrioventricular Septal Defect
  • Abnormalities of atrioventricular valve form
    function and interatrial interventricular
    communication from maldevelopment of the
    endocardial cushions
  • Presence of AVSD indicated with Diagnosis
  • Partial AVSD 1-2 years of age except CHF or
    growth failure
  • Complete AVSD with good condition 3-6 months
  • Development of pulmonary vascular obstructive
    disease not indicated

37
Congenital Aortic Stenosis
  • The various forms of LVOTO occur in combination
    with other cardiac lesions (IAA, COA, MV
    anomalies, LV hypoplasia) and obstructive types
    are supravalvular, valvular, subvalvular,
    intraventricular
  • Critical AS in neonates urgent (severe CHF, LV
    dilatation, hypertrophy)
  • Infants and children
  • Pressure gradient gt 75mmHg
  • Symtoms of angina, syncope, exercise
    intolerance, LVH,
  • pressure gradient gt 50mmHg
  • Pressure gradient over 40mmHg in subvalvular
    lesion
  • to prevent progression

38
Ebsteins anomaly
  • A congenital defect of tricuspid valve in which
    the origin of septal and posterior leaflets or
    both are displaced downward into the right
    ventricle and the leaflets are variably deformed
  • Symptomatic Ebsteins anomaly is an indication
  • Valve repair and ASD closure
  • with important TR, moderate and severe
    cyanosis
  • WPW syndromes
  • ablation of accessory conduction pathway

39
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40
Pulmonary Stenosis
  • A form of RV outflow obstruction in which
    stenosis is usually valvar or both valvar
    subvalvularor only subvalvular
  • Critical PS in neonate indicated with Dx
  • Percutaneous balloon valvotomy
  • Valvotomy with CPB
  • Transannular RVOT patch widening
  • PS in infants and children indicated with
    Symptoms Pr gradient over 50mmHg
  • Surgical treatment is not indicated with mild
    stenosis

41
Tetralogy of Fallot
  • Characterized by underdevelopment of RV outflow
  • Diagnosis is an indication of operation
  • Symptomatic complicated in early life
  • Early total correction or
  • Shunt (1-2 months) and total correction (1
    year)
  • Asymptomatic uncomplicated
  • Total correction at 3-24 months
  • Multiple VSDs, LAD from RCA
  • Initial shunt and total correction

42
Double Outlet Right Ventricle
  • A congenital cardiac anomalies which both great
    arteries rise wholly or in large part from the
    RV. It is, then, a type of ventriculoarterial
    connection.
  • Dx is an indication of operation
  • Simple DORV with subaortic VSD repair by 6 mo
  • with PS --- repair like TOF
  • DORV with subpulmonic VSD (Taussig-Bing heart)
  • arterial switch operation within 1 mo

43
Transposition of Great Arteries
  • A cardiac anomaly in which the Aorta arises
    entirely or in large part from the RV, and PA
    from LV (atrioventricular concordant connection
    and ventriculoarterial discordant connection)
  • Simple TGA in neonate
  • arterial switch operation within 1 months
  • Simple TGA beyond 30 days
  • atrial switch operation (Mustard, Senning)
  • TGA with VSD
  • arterial switch operation as early

44
Tricuspid Atresia
  • A cardiac anomaly in which RV fails to open into
    a
  • ventricle through a AV valve. There is thus
    a
  • univentricular AV connection
  • PVR is an important indicator
  • gt 4 unit -- contraindication
  • 2-4 unit -- BCPS
  • lt 2 unit -- Fontan operation
  • Symptomatic in early life
  • early shunt or PAB
  • BCPS or hemi-Fontan at 6-12 months
  • Fontan at 12-24 months
  • Asymptomatic
  • Fontan candidate 12-30 months

45
Congenitally Corrected TGA
  • A cardiac anomaly with ventriculoarterial
    discordant connection atrioventricular
    discordant connection. The circulatory pathways
    are therefore in series
  • The presence of CCTGA per se is not an
    indication.
  • With VSD indications for VSD
  • With VSD PS indications for TOF
  • With complete heart block pacing
  • Double Switch operation anatomic correction
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