TRICUSPID ATRESIA

AMRUTHA R

• Atresia means closed or absent. • Tricuspid Atresia results in a number of

problems: • ♥ The tricuspid valve is closed or absent. • ♥ The right ventricle is small. • ♥ The pulmonary artery is narrow. • ♥ The pulmonary valve may be blocked.

Definition

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

• Incidence : 0.06 per 1000 live births

• Prevalence :in clinical series of congenital heart disease is 1- 2.4 %.

To survive your baby will need either

• ASD OR VSD

• PDA

HISTORY

• First reported by Kreysig in 1817.• Clinical features reported by Bellet and

Stewart in 1933.• Also by Taussig and Brown in 1936.

Embryology

Formation of the atrioventricular cardiac valves:

Days 34 to 36 • 1.Formed from endocardial cushion tissue • 2. Tricuspid valve, papillary muscles and

chordate tendineae formed largely from the conus septum.

Causes

• Multifactorial inheritance hypothesis

TYPES

MUSCULARCOMMON

89%

DIMPLING OR FIBROUS THICKENING

MEMBRANEOUS6.6%

SEPTUM FORM THE FLOOR OF RA

VALVAR1%

FUSED CUSPS

EBSTEINS ANOMALY

• The atrioventricular canal type is extremely rare (0.2%). -a leaflet of the common atrioventricular valve seals off the only entrance into the right ventricle.

PATHOPHYSIOLOGY

• ATRESIA OF TRICUSPID VALVE

• No communication between RA AND RV

• RV id underdeveloped.• Systemic venous blood received by RA

• Enters LA through PFO or ASD

• Mixing of systemic and pulmonary blood

• Enters LV

• Blood enters RV through VSD

• From RV blood enters Pulm trunk

• Blood enters pulm trunk via PDA

• Increased pulmonary blood flow

• LA and LV hypertrophy

CHF

WITH TGA

LUNGS

• The clinical features of tricuspid atresia largely depend on the quantity of pulmonary blood flow

DECREASED PULM FLOW 90%severe cyanosis, hypoxemia, and acidosisLV apical impulseWaves in jugular venous pulse

• pulmonary oligemia • may have central cyanosis,• tachypnea or hyperpnea,

INCREASED PULM FLOW

• Diff to diagnose• may not appear cyanotic but may present

with signs of heart failure later in infancy• pulmonary plethora present with

symptoms of dyspnea, fatigue, difficulty feeding, and perspiration, which are suggestive of congestive heart failure.

• Cyanosis is minimal

Other features

• holosystolic type of murmur at the lower sternal border, suggestive of VSD,

• Problems related to chronic cyanosis, such as

• clubbing, • polycythemia, relative anemia, • stroke, brain abscess, • coagulation abnormalities,

INVESTIGATIONS

• History and PE• Pulse oximetry• ABG• Hb and hematocrit

Reduced pulm flow

INCREASED BLOOD FLOW

Large ventricular cavitySmall RV

EECHOCARDIOGRAPHY

angiography

MEDICAL

• an intravenous infusion of PGE1• 0.03-0.1 mcg/kg/min to open the ductus

arteriosus• anticongestive therapy with digoxin,

diuretics

Rashkind balloon atrial septostomy.

• PALLIATION FOR DECREASED PULMONARY BLOOD FLOW

• Systemic to pulmonary artery shunt: increases pulmonary blood flow through

surgically created left to right shunt at the great vessel level

Classic Blalock-Taussig shunt

end to side anastomosis

Rarely perf

Modified Blalock-Taussig shunt

Gortex interposition graft

Central shunt

Gortex interposition graft between aorta and main pulmonary artery Reprinted

Potts

Direct anastomosis descending aorta to left pulmonary artery

Direct anastomosis ascending aorta to right pulmonary artery

• Palliation for increased pulmonary blood flow

• Control amount of pulmonary blood flow to prevent CHF and pulmonary vascular disease from pulmonary

overcirculation

Pulmonary artery band

• Palliation for tricuspid atresia

Hemifontan/Bidirectional Glenn

• Surgical options for Fontan operation • a. Lateral tunnel: Gortex graft placed

inside RA to direct IVC flow through RA/SVC junction and into MPA

• b. Extracardiac (As Illustrated): Gortex or Dacron circumferential tube graft from IVC to MPA

complications:

• Arrhythmia:• ablation, pacemaker, ICD, medications,

conversion to lateral tunnel • B. Ventricular dysfunction: • rhythm and transplant

• C. Atrioventricular valve regurgitation (AVVR): Valve repair/replacement

• D. Fontan pathway obstruction: reoperation for relief of conduit stenosis

bronchitis:

• G. Thromboembolic events: • anticoagulation varies from center to

center but minimally life long aspirin (ASA