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TRICUSPID ATRESIA

Dr Anjum rashid
Pediatrics PRH
Tricuspid atresia
• Defined as congenital absence or agenesis of
the tricuspid valve
• Incidence : 0.06 per 1000 live births
• Prevalence:1-2% of congenital heart disease
• It is the 3rd most common cyanotic cardiac
condition.
Types
• The muscular variety -constitutes 89% of cases
• The membranous type (6.6%)
• The valvar type (1%)
• The Ebstein type (2.6%)
• The atrioventricular canal type is extremely
rare (0.2%)
Muscular type
Tricuspid Atresia
Pathophysiology:

• Absent communication
from RA to RV
• An interatrial
communication is
necessary for survival.
• The right atrium is
enlarged and
hypertrophied
• The RV is hypoplastic.
Pathophysiology:

• The VSD can be of varying


size.
• There may be pulmonary
stenosis of varying degrees.
• GA normally related
• GA transposed
Pathophysiology:

• The NB may depend on


the Ductus Arteriosus
for pulmonary blood
flow.
• The degree of cyanosis
and symptoms are
related to these multiple
factors.
Classification

• Proposed by Kuhne and later modified


• Type 1 : normally related great arteries (70 –
80%)
a. intact ventricular septum with pulmonary
atresia( 9%)
b. small ventricular septal defect and
pulmonary stenosis( 51%)
c. large ventricular septal defect without
pulmonary stenosis ( 9%)
Type 2:TA withTransposition of
great arteries (12 – PA
25%) Ao
a. ventricular septal defect with RA LA
pulmonary
atresia( 2%)
b. ventricular septal defect with RV
pulmonary
stenosis( 8%)
c. ventricular septal defect without
pulmonary stenosis(18%)
Clinical features
• Symptoms manifest early in life.
• Depend on the magnitude of pulmonary
blood flow.
• The 2 known presentations are decreased
pulmonary blood flow and increased
pulmonary blood flow
In decreased pulmonary blood flow
• Central cyanosis, tachypnea or hyperpnea, normal
pulses, and prominent a waves in the jugular venous
pulse.
– A quiet precordium and no thrills on palpation.
– Second heart sound is single
– A holosystolic type of murmur at the lower sternal
border, suggestive of VSD, is heard.
In decreased pulmonary blood flow
– In patients with pulmonary atresia, the
holosystolic murmur is not present, and a
continuous murmur of patent ductus arteriosus is
occasionally heard.
– Clinical signs of heart failure are not observed
In increased pulmonary blood flow
• Dyspnea, fatigue, difficulty feeding, and
perspiration.
• Failure to thrive and recurrent respiratory
tract infection
• Tachypnea, tachycardia, minimal cyanosis,
prominent neck venous pulsations and
hepatomegaly.
In increased pulmonary blood flow
– Increased and hyperdynamic precordial impulses.
– The second heart sound may be single or split,
and a third heart sound at the apex may be heard.
– Holosystolic murmur of VSD at the left lower
sternal border and middiastolic rumble at the
apex.
• Chronic cyanosis-clubbing, polycythemia,
stroke, brain abscess, coagulation
abnormalities, and hyperuricemia
• Infective endocarditis
• Atrial arrythmias
CHEST X-RAY
Tr. Atresia with TGA
ECG
ECHOCARDIOGRAM
• Cardiac catheterization
• Right atrial angiography
Management :FOR ↓ PBF.
• PGE1 infusion- 0.01-0.20 microgram/kg is
initiated for infants who depend on the PDA
for pulmonary blood flow; and the infant is
stabilized and readied for surgery.
• Rashkind Balloon atrial septostomy
• Surgical septectomy
• Modified Blalock-
taussig shunt -(Gore-
Tex
interposition graft
between the subclavian
artery and the ipsilateral
pulmonary artery)-
newborn period
• Bidirectional
Glenn(superior vena
cava–to–right
pulmonary artery
anastomosis) thereby
reducing left ventricular
volume load by 1/3.
This part of the blood
flow to the lungs is now
by passive flow -3-6
month age
↑ PBF
In patients with tricuspid atresia type II ,
pulmonary artery banding should be
performed following stabilization with
anticongestive measures.
Modofied Fontan operation
• Between ages 18 months and 3 years age.
Desaturated blood is directly channeled from
the IVC to the pulmonary arteries
• Lateral tunnel fontan (internal baffle)
• External conduit Fontan (homograft or goretex
tube)
Modofied Fontan operation
Modofied Fontan operation
Contraindications
Elevated pulmonary vascular resistance
Pulmonary artery hypoplasia
Left ventricular dysfunction
Mitral insufficiency
Abnormal sinus rhythm
Modofied Fontan operation
• Complications
• Fluid retention, pleural and peicardial effusions.
• Baffle obstruction SVC or IVC syndrome
• Vena cava or pulmonary artery
thromboembolism
• Protein loosing enteropathy
• Supraventricular arrhythmias
• Hepatic cirrhosis
Successful treatment option
• Heart transplantation
THANK
YOU