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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
• 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

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

• The VSD can be of varying

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

• The NB may depend on

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

• Proposed by Kuhne and later modified

• Type 1 : normally related great arteries (70 –
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
atresia( 2%)
b. ventricular septal defect with RV
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
– 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
• Failure to thrive and recurrent respiratory
tract infection
• Tachypnea, tachycardia, minimal cyanosis,
prominent neck venous pulsations and
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
• Chronic cyanosis-clubbing, polycythemia,
stroke, brain abscess, coagulation
abnormalities, and hyperuricemia
• Infective endocarditis
• Atrial arrythmias
Tr. Atresia with TGA
• 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-
interposition graft
between the subclavian
artery and the ipsilateral
pulmonary artery)-
newborn period
• Bidirectional
Glenn(superior vena
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
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
Modofied Fontan operation
Modofied Fontan operation
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
• Protein loosing enteropathy
• Supraventricular arrhythmias
• Hepatic cirrhosis
Successful treatment option
• Heart transplantation