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TRICUSPID ATRESIA. Dr Bijilesh u Senior Resident, Dept. of Cardiology, Medical College, Calicut . References. - Perloff ‘s text book of congenital heart diseases - Moss and Adams ‘s text book of congenital cardiology

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tricuspid atresia

TRICUSPID ATRESIA

Dr Bijilesh u

Senior Resident,

Dept. of Cardiology,

Medical College, Calicut

slide2

References

- Perloff ‘s text book of congenital heart diseases

- Moss and Adams ‘s text book of congenital cardiology

- Rudolph text book of pediatric cardiology

- Freedom’s natural and modified natural history of cong.heart diseases

- Andreson text book of congenital heart disease

- Figenbaum’ text book of echocardiography

- Hurst’s heart disease

- Braunwald’ heart diseases

- Langman’s Embryology

slide3

Defined as congenital absence or agenesis of the tricuspid valve, with no direct communication between the right atrium and right ventricle

slide4

Incidence : 0.06 per 1000 live births

  • Prevalence : 1- 3% of CHD
  • (Report of New England Regional
  • Infant Cardiac Program – 1980)
history
HISTORY
  • First reported by Kreysig in 1817
  • Clinical features reported by Bellet and Stewart in 1933
  • Taussig and Brown in 1936
embryology
EMBRYOLOGY
  • Early embryogenesis - process of expansion of inlet portion of right ventricle coincides with development of AV valves
  • Failure of this process - atresia of tricuspidvalve & absent inlet portion of right ventricle
  • Embryological insult occurring later in gestation - Less common variety - with well formed but fused leaflets
  • If valve fusion incomplete - tricuspid stenosis
slide7

ANATOMY

Muscular

Membranous

Atrioventricular

slide8

Most common type

  • – muscular (89%)
  • Dimple or a localized fibrous thickening in the floor of RA at expected site of tricuspid valve.
slide9

Membranous type - membranous septum forms floor of the RA at the expected location of TV

  • May be associated with
  • absent pulmonary valve leaflets
slide10

Atrioventricular canal type

    • Extremely rare (0.2%)
    • Leaflet of the common AV valve
    • seals off the only entrance into RV
slide11

MORPHOLOGICAL

CONSIDERATIONS

ra asd
RA & ASD
  • The right atrium is enlarged and hypertrophied.
  • Interatrial communication is necessary for survival
  • Stretched patent foramen ovale - ¾ cases
  • True ASD less common - ostiumsecundum type
slide13

Rarely patent foramen ovale is obstructive and may form an aneurysm of fossaovalis

  • Sometimes large enough to produce mitral inflow obstruction
la lv
LA & LV
  • Left atrium may be enlarged, especially when pulmonary blood flow is increased
  • Mitral valve is morphologically normal - rarely incompetent
  • LV is enlarged and hypertrophied
right ventricle
Right ventricle

Size of the RV varies – depends on size of VSD

With a large VSD or TGA - RV larger

When VSD is small - only the conus is present

Small and hypoplastic

Inflow or sinus portion absent

Trabecular portion & outflow or conus region often well developed

vsd in tricuspid atresia
VSD in Tricuspid Atresia
  • Associated VSD - 90% of individuals during infancy
  • Usually perimembranous
  • Can be muscular /malalignment types
  • Restrictive VSD’S cause
  • subpulmonic obstruction in pts with NRGA
  • subaortic obstruction in pts with TGA
slide17

At birth VSD is usually restrictive- permitting adequate but not excessive PBF

  • 40% of these defects close spontaneously/ decrease in size - acquired pulmonary atresia
  • Majority of defects close in the first yr of life
slide18

Classification- KUHNE

  • Type 1 Normally related great arteries
  • Type 2 D-transposition of great arteries
  • Type 3 L- Transposition of great arteries
slide19

Type 1

  • Normally related great arteries (70 – 80%)
  • a. Intact IVS with pulmonary atresia( 9%)
  • b. Small VSD and pulmonary stenosis( 51%)
  • c. Large VSD without pulmonary stenosis ( 9%)
type ii
TypeII

D-transposition of great arteries (12 – 25%)

a. VSD with pulmonary atresia( 2%)

b. VSD with pulmonary stenosis( 8%)

c. VSD without pulmonary stenosis(18%)

type 3
Type3
  • L- Transposition or malposition of great arteries (3-6%)
  • Associated with complex lesions
  • Truncusarteriosus
  • Endocardial cushion defect
additional cardiovascular abnormalities 20
Additional cardiovascular abnormalities- 20%
  • Coarctation of aorta – 8%
  • Persistent left SVC
  • Juxtaposition of atrial appendages
  • -50% of TA with TGA
  • Right aortic arch
  • Abnormalities of mitral apparatus- cleft in AML,MVP ,direct attachment of leaflets to papillary muscles
physiology ta
PHYSIOLOGY- TA
  • Obligatory rt to left shunt at atrial level
  • LA receives both the entire systemic and pulmonary venous return
  • Entire mixture flows into LV - sole pumping chamber
slide26

PHYSIOLOGY

TAWITHNRGA

  • Pulm artery blood flow is usually reduced
  • Restrictive VSD - zone of subpulmonicstenosis.
  • LV overload is curtailed but
  • at the cost of cyanosis
  • 90% of cases
slide27

TAWITHNRGA

PHYSIOLOGY

  • When VSD is non restrictive and pulmonary vascular resistance is low
  • PBF and LV volume over load - excessive
  • Cyanosis is mild
ta with tga
TA withTGA

PHYSIOLOGY

  • VSD is almost always non-restrictive and PS usually absent
  • Low PVR > abundant pulmonary arterial blood flow
  • Minimal cyanosis,marked LV volume overload
  • With restrictive vsd or infundibular narrowing
  • →diminished syst circulation
  • →metabolic acidosis and shock
sex predilection
SEXPREDILECTION
  • TA with NRGA - Equal frequency in males & females
  • TA with TGA
  • - male preponderance
  • - no male preponderance with
  • juxtaposition of atrial appendages
genetics
GENETICS
  • Specific genetic causes - remain to be determined in humans
  • FOG2 gene may be involved
  • Validated only in animal studies
  • 22q11 deletion
  • Familial recurrence is low
  • Recurrence in siblings is only about 1%
natural history
NATURALHISTORY

TA with NRGA with an intact IVS

  • Few infants survive beyond 6 months without surgical palliation
  • Intense hypoxia and death ensue
  • unless ductus is patent
  • unless adequate systemic to PA collaterals
  • present
ta with nrga small vsd
TA with NRGA & SMALL VSD

NATURALHISTORY

  • VSD closes spontaneously or become excessively obstructive - majority die by one year
  • Rarely a favorable balance achieved b/w VSD & PBF permitting survival from 2nd to 5th decades
ta with nrga large vsd
TA with NRGA & LARGE VSD.
  • NATURALHISTORY
  • Excessive PBF > vol. overload of LV and CCF
  • Patients usually do not fare well
  • Some have lived to ages 4 to 6 years
  • Long survivals reported between ages 32 and 45 yrs - in exceptional cases
tr atresia with tga
TR.ATRESIAWITHTGA
  • NATURALHISTORY
  • Same poor longevity patterns hold for TA with TGA and large VSD
  • Exceptional survivals to mid-late teens reported
  • TA with TGA with subaorticstenosis
  • ( restrictive VSD) - ominous combination
overall survival in infants with ta
Overall survival in infants with TA
  • 1 year- 72%.
  • 5 years- 52%.
  • 10 years- 46% Franklin et al 1972 -1987, 237 patients
slide36

Survival of patients presenting in infancy with tricuspid

atresia to the Toronto Hospital for Sick Children, Tame et al , 101 patients, 1970 - 1984

Probability of surviving for 1 year was 64% and to 8 years was 55%

The overall surgical mortality for the palliative procedures was 35.8%

physical examination appearance
Physicalexamination-appearance
  • Dysmorphicfacies
  • - Cat-eye syndrome
  • - congenital coloboma
  • JVP
  • - a wave amplitude increase due to restrictive
  • interatrial communication.
  • - Y descent is slow
precordium
Precordium
  • LV impulse without an RV impulse in a cyanotic patient
  • Gentle RV impulse - TA with non restrictive VSD and a well developed RV
  • Palpable thrill if VSD is restrictive
auscultation
AUSCULTATION
  • First heart sound is single
  • Second usually single - soft pulmonic component - occasionally present
  • TA with NRGA
  • - prominent murmur of restrictive VSD
  • – holosystolic maximal at mid to lower LSB
ta with tga increased pbf
TA with TGA & increased PBF

AUSCULTATION

  • Holosystolic murmur – across VSD
  • S2 – single & loud
  • S3
  • MDM
pulmonary vascular resistance high
Pulmonary vascular resistance – high
  • AUSCULTATION
  • VSD murmur vanishes
slide42

AUSCULTATION

  • TA with TGA
  • - coexisting pulmonic or subpulmonicstenosis
  • - midsystolic murmur – loudness and length vary
  • inversely with degree of obstruction
slide43
ECG
  • Tall peaked right atrial P waves - Himalayan P waves
  • LV hypertrophy
  • Absence of RV forces in precordial leads
  • QRS axis
  • - left and superior - type 1
  • - LAD or normal - type 2
chest x ray ta with nrga and small vsd
CHEST X-RAY- TA WITH NRGA AND SMALL VSD
  • Pulmonary vascularity reduced
  • Pulmonary artery segment – inconspicuous
  • Heart size – normal
  • Right cardiac border
  • superior convexity
  • - enlarged RA
  • Inferior part
  • flat or receding
  • - absence of RV
cxr ta with tga no obstruction
CXR - TA with TGA - no obstruction
  • Lungs – plethoric
  • LV, LA, RA – enlarged
  • Right cardiac border
  • no hump-shaped contour
  • – RV is relatively well developed
cxr ta with tga and ps
CXR - TA with TGA andPS
  • Pulmonary blood flow is normal or reduced
  • Prominent RA
  • Convex LV
  • Narrow vascular pedicle
echocardiogram
ECHOCARDIOGRAM

Presence of an imperforate linear echo density at the location of normal TV

  • Presence and size of the interatrial communication
  • Presence and size of a VSD
  • Relationship of the aorta and pulmonary artery
slide49

Size of the RV and pulmonary arteries

  • Presence and severity of infundibular or pulmonary stenosis
  • Presence and size of the ductusarteriosus
  • Presence of aortic isthmus narrowing or coarctation
  • Degree of mitral regurgitation
  • Left ventricular function
cardiac catheterization
CARDIACCATHETERIZATION
  • Limited role at present
  • Newborn
  • Define sources of pulmonary blood flow
  • Associated anomalies not clearly defined by echo
  • TA with TGA - Obstruction at VSD or infundibulum
  • Therapeutic role for balloon atrialseptostomy
slide51

CARDIACCATHETERIZATION

  • Prior to Fontan
  • - Pulm.Vascular resistance
  • - Pulmonary artery size
  • - Pulmonary artery distortion - by previous surgery
  • Older patients without definitive palliation
  • - detect collaterals from aorta - lungs
hemodynamic data
HEMODYNAMICDATA
  • Right atrial pressure is slightly higher than LAP
  • Prominent ‘a ‘wave in the right atrium
  • -interatrial communication is restrictive
  • LV systolic and EDP – normal
  • LVEDP may increase with large VSD as PVR drops and LV volume overload ensues
slide53

Oxygen saturation (Sao2)

  • in systemic venous return
  • - lower than normal
  • - diminished Sao2 in
  • systemic arterial blood
  • Sao2 of pulmonary venous
  • return - normal
  • LA and LV saturations
  • - diminished
  • - obligatory R-L shunt
initial medical management
INITIALMEDICALMANAGEMENT

PGE1

  • Maintain patency of the ductus before cardiac catheterization or planned surgery
  • Given as an infusion
  • .025 – 0.1 mg/kg/mt
  • Potential for apnea
  • Fever , siezure, hypotension
slide55

Balloon atrialseptostomy may be done as part of initial catheterization to improve the RA-LA shunt

for pbf
FOR↓PBF
  • PBF - increased by surgical creation of an aortopulmonary shunt
  • Blalock & Taussig - Subclavian artery - ipsilateral PA
  • Potts - Descending aorta – LPA
  • Waterston-Cooley - Ascending aorta – RPA
slide59

Modified Blalock-Taussig shunt

  • - Gore-Tex interposition graft
  • - Subclavian artery - PA
  • Central aortopulmonary fenestration
  • - Gore-Tex shunt
slide60

Glenn shunt

  • Superior vena cava – RPA
  • End-to- end
  • Improves PBF and Sa o2
  • No risk of pulmonary HTN
  • Pulmonary AV malformations later
slide61
↑PBF
  • Pulmonary artery banding
  • - In patients with
  • tricuspid atresia type II
corrective surgery
CORRECTIVESURGERY
  • Fontan and Kreutzer
  • - physiologically corrective operation
  • - complete separation of the systemic and
  • pulmonary circuits
choussat criteria
CHOUSSAT CRITERIA
  • Age at operation – 4 and 15 yrs
  • - not strictly followed nowadays
  • Normal sinus rhythm
  • Normal systemic venous connections
  • Normal right atrial size
  • Normal pulmonary arterial mean pressure
  • - > = 15 mm Hg
slide64

Low pulmonary vasc resistance

  • - 4 woods units/m2
  • Adequate sized PA with diameter > 75% of aorta
  • Normal LVEF (>60%)
  • Absence of MR
  • Absence of complicating factors from previous surgeries