tricuspid atresia in pediatrics
DESCRIPTION
tricuspid atresiaTRANSCRIPT
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TRICUSPID ATRESIA
AMRUTHA R
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• 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.
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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 %.
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To survive your baby will need either
• ASD OR VSD
• PDA
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HISTORY
• First reported by Kreysig in 1817.• Clinical features reported by Bellet and
Stewart in 1933.• Also by Taussig and Brown in 1936.
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Embryology
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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.
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Causes
• Multifactorial inheritance hypothesis
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TYPES
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MUSCULARCOMMON
89%
DIMPLING OR FIBROUS THICKENING
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MEMBRANEOUS6.6%
SEPTUM FORM THE FLOOR OF RA
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VALVAR1%
FUSED CUSPS
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EBSTEINS ANOMALY
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• 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.
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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
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• Mixing of systemic and pulmonary blood
• Enters LV
• Blood enters RV through VSD
• From RV blood enters Pulm trunk
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• Blood enters pulm trunk via PDA
• Increased pulmonary blood flow
• LA and LV hypertrophy
CHF
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WITH TGA
LUNGS
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• The clinical features of tricuspid atresia largely depend on the quantity of pulmonary blood flow
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DECREASED PULM FLOW 90%severe cyanosis, hypoxemia, and acidosisLV apical impulseWaves in jugular venous pulse
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• pulmonary oligemia • may have central cyanosis,• tachypnea or hyperpnea,
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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
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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,
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INVESTIGATIONS
• History and PE• Pulse oximetry• ABG• Hb and hematocrit
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Reduced pulm flow
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INCREASED BLOOD FLOW
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Large ventricular cavitySmall RV
EECHOCARDIOGRAPHY
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angiography
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MEDICAL
• an intravenous infusion of PGE1• 0.03-0.1 mcg/kg/min to open the ductus
arteriosus• anticongestive therapy with digoxin,
diuretics
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Rashkind balloon atrial septostomy.
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• 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
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Classic Blalock-Taussig shunt
end to side anastomosis
Rarely perf
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Modified Blalock-Taussig shunt
Gortex interposition graft
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Central shunt
Gortex interposition graft between aorta and main pulmonary artery Reprinted
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Potts
Direct anastomosis descending aorta to left pulmonary artery
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Direct anastomosis ascending aorta to right pulmonary artery
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• Palliation for increased pulmonary blood flow
• Control amount of pulmonary blood flow to prevent CHF and pulmonary vascular disease from pulmonary
overcirculation
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Pulmonary artery band
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• Palliation for tricuspid atresia
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Hemifontan/Bidirectional Glenn
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• 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
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complications:
• Arrhythmia:• ablation, pacemaker, ICD, medications,
conversion to lateral tunnel • B. Ventricular dysfunction: • rhythm and transplant
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• C. Atrioventricular valve regurgitation (AVVR): Valve repair/replacement
• D. Fontan pathway obstruction: reoperation for relief of conduit stenosis
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bronchitis:
• G. Thromboembolic events: • anticoagulation varies from center to
center but minimally life long aspirin (ASA