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Tricuspid Atresia

Tricuspid Atresia. Seoul National University Hospital Department of Thoracic & Cardiovascular Surgery. Tricuspid Atresia. 1. Definition Congenital cardiac malformation in which the right atrium fail to open into a ventricle through an A-V valve.

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Tricuspid Atresia

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  1. Tricuspid Atresia Seoul National University Hospital Department of Thoracic & Cardiovascular Surgery

  2. Tricuspid Atresia • 1. Definition • Congenital cardiac malformation in which the right • atrium fail to open into a ventricle through an A-V valve. • The atrial situs is almost solitus in association with a • ventricular D-loop, and right ventricle is hypoplastic. • 2. History • Kuhne : Recognized entity in 1906 • Bellet : Description of clinical feature in 1933 • Glenn : Glenn shunt in 1958 • Fontan : Successful repair in 1968 • Kreutzer : Modification in 1973

  3. Tricuspid Atresia • Pathophysiology • Atretic tricuspid valve, hypoplastic right ventricle, ASD, VSD and restricted pulmonary blood flow(70%) lead to right-to-left shunting and cyanosis. • In patients with unrestricted pulmonary blood flow (30%), pulmonary overcirculation and congestive heart failure develop. • Manifestations of left ventricular volume overload (e.g., ventricular dilation, mitral regurgitation, ventricular dysfunction) result from combined systemic and pulmonary venous return.

  4. Tricuspid Atresia • Hemodynamics • Deoxygenated blood • Oxygenated blood

  5. Tricuspid Atresia • Surgical Morphology • 1. Types of atresia • . Muscular : 75% • . Membranous • Fibrous diaphragm • Ebstein variant • A-V canal defect variant ; rarer variant • 2. Origin of great arteries • . Ventriculoarterial concordant : 60~70% • . Ventriculoarterial discordant : 30~40% • . DORV, DOLV, single outlet with truncus : rarely • 3. Others • . Abnormality of mitral valve & LV ; high • . Juxtaposition of atrial appendage in 11%

  6. Classification of Tricuspid Atresia • 1. TA with normal origin of great arteries (type I) • . ASD always (4% restrictive) • Uncommonly ostium primum defect • . Obstruction to pulmonary blood flow : 85% (atretic in 10%) • . LSVC (15%), LSVC to LA (1~5%) • 2. TA with transposition of great arteries (Type II) • . D-malposition, uncommonly L-malposition (Type III) • . ASD always (small in 50%) • . Lt. atrial juxtaposition (10%), CoA (30%), IAA (rare) • . Pulmonary blood flow • Usually large flow • Subpulmonic stenosis (20~30%, rarely atretic)

  7. Classification of TA Most frequent

  8. Clinical Features & Diagnosis • 1. Symptoms & signs • . Usually cyanosis, dyspnea, hypoxic spell depends on severity of PS • . Clubbing in childhood • . Excessive pulmonary flow with mild cyanosis & CHF in minority • . Harsh ejection systolic murmur in most patients • 2. Chest radiography • . Variable, depends on pulmonary blood flow • 3. Electrocardiography • . LAD, LVH, abnormal P-wave • 4. Echocardiography • 5. Cardiac catheterization & cineangiography

  9. 1. Incidence 1~3% of CHD 2. Survival 1) Normal origin of great arteries . With PS : 10% survival in one year Rapid narrowing of VSD : progressive cyanosis . Without PS : 10% survival in 10 years Congestive heart failure Narrowing of VSD : increasing cyanosis 2) Transposed great arteries . Without PS : almost dead in one year Tendency to close of VSD : subaortic stenosis . With PS : 50% survival in 2 years Narrowing of VSD is slower 3. Modes of death . Hypoxia, LCO in young . Volume overload & hypoxia : cardiomyopathy in old Natural History of TA

  10. TricuspidAtresia Muscular atresia

  11. TricuspidAtresia RV LV VSD

  12. TricuspidAtresia Membranous atresia

  13. Operative Techniques of TA • 1. General plan of Fontan operation • . Risk of thrombosis around catheter • . Coronary sinus to pulmonary venous chamber • . Pericardiopleural window • 2. Procedures • . Systemic-pulmonary artery shunting • . Pulmonary artery banding • . Bidirectional superior cavopulmonary shunt • . Hemi-Fontan operation • . Fontan operation by right atrial-pulmonary artery connection • . Fontan operation by total cavopulmonary connection • with or without incomplete atrial partition • . Total cavopulmonary connection with extracardiac conduit

  14. Repair for Systemic Obstruction Repair of hypoplastic aortic arch and pulmonary trunk band placement via thoracotomy

  15. Repair for Systemic Obstruction Construction of proximal pulmonary trunk to aortic anastomosis ( Damus-Kaye-Stansel operation ) for systemic outflow tract obstruction

  16. Repair for Systemic Obstruction Direct relief of subaortic obstruction in the univentricular heart with transposed great arteries ( VSD extension )

  17. Bidirectional Cavopulmonary Shunt

  18. Hemi-Fontan Operation

  19. Fontan Operation Lateral Tunnel

  20. Fontan Operation Extracardiac Conduit

  21. Results of Palliative Operation • 1. Systemic pulmonary artery shunting • 2. Pulmonary artery banding • 3. Glenn operation • Pulmonary A-V fistula • 4. Incomplete ( partial ) Fontan operation • BCPC ( SVC, IVC ) • Hemi - Fontan operation • Fontan operation with deliberately incomplete • atrial partitioning

  22. Results of Fontan Operation • 1. Survival ; Early & time-related survival • 2. Modes of death ; Early & Late • 3. Incremental risk factors for death • 4. Functional & hemodynamic status • 5. Cardiac rhythm • 6. Abnormality of pulmonary circulation • 7. Protein-losing enteropathy • 8. Thromboembolic complication • 9. Neurologic complications • 10. Desaturation after Fontan operation • 11. Reoperation

  23. Incremental Risk Factors for Death • 1. Acute ventricular decompression • 2. Late pulmonary & ventricular deterioration • 3. Age at operation • 4. Cardiac morphology • 5. Pulmonary artery size • 6. PA pressure & PVR • 7. Ventricular hypertrophy • 8. Atrial isomerism • 9. RA-PA connection technique

  24. Operative Indications for TA • 1. Indications for palliative procedure • . Shunt procedure • . Banding • 2. Indications for Fontan operation • Indicated in whom one ventricle is too small or dysplastic or • both, be unable to generate pulmonary or systemic blood flow • . Optimal Age : 18 ~ 30 months of age (early : 10~12 months) • . Hemi - Fontan or BCPC around 6 months of age • . PVR 4units more : contraindicated • . Ventricular end-diastolic pressure of greater than 15mmHg , • important A-V valve incompetence , or EF of less than 45% • raises a question of suitability

  25. Special Situations & Controversies • Types of operation for tricuspid atresia • Valved Fontan pathway • Persistent left superior vena cava with hemiazygos extension of inferior vena cava No longer Kawashima variant • Ideal Fontan technique Currently, extracardiac conduit or lateral tunnel Fontan variants are preferred

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