TETRALOGY OF FALLOT AND RIGHT VENTRICULAR OUTFLOW TRACT

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TETRALOGY OF FALLOT AND RIGHT VENTRICULAR OUTFLOW TRACT OBSTRUCTION KSC MALEFAHLO

TETRALOGY OF FALLOT AND RIGHT VENTRICULAR OUTFLOW TRACT OBSTRUCTION KSC MALEFAHLO

 • "Dr. Blalock, I stand in awe and admiration of your surgical skill

• "Dr. Blalock, I stand in awe and admiration of your surgical skill but the truly great day will be when you build a ductus for the child dying of anoxemia and not when you tie off a ductus for a child with a little too much blood going this lungs. "Dr. Blalock gave a Sigh and replied, "When that day comes, this will seem like child's play. "

Outline Anatomical Features Associated Anomalies and Fallot Variants Physiology and Clinical Presentation Natural History

Outline Anatomical Features Associated Anomalies and Fallot Variants Physiology and Clinical Presentation Natural History Diagnosis Surgical Treatment • Modified Blalock-Taussig shunt • Technique of complete repair Post-operative Care and Results Anatomical Subsets • Absent pulmonary valve syndrome • With atrioventricular septal defect • With anomalous course of coronary artery

Anatomical features • DEFINITION – Due to antero-cephalad deviation of outlet septum leading to:

Anatomical features • DEFINITION – Due to antero-cephalad deviation of outlet septum leading to: » Unrestricted malaligned large subaortic VSD; » RVOTO at any level; » Consequent RVH and » An overriding aorta(<50%) – Additional features include muscular VSD, anomalous coronaries, PDA, MAPCAs(esp with pulm. atresia) and RSAarch Pentalogy of Fallot has an ASD or PFO

 • D. Mayer and C. Mullins, Congenital Heart Disease, A Diagrammatic Atlas. New

• D. Mayer and C. Mullins, Congenital Heart Disease, A Diagrammatic Atlas. New York: Alan R. Liss, Inc, 1988. p. 5.

ASSOCIATED ANOMALIES • PERSISTENT PATENCY OF THE FORAMEN OVALE AND ASD • ADDITIONAL MUSCULAR

ASSOCIATED ANOMALIES • PERSISTENT PATENCY OF THE FORAMEN OVALE AND ASD • ADDITIONAL MUSCULAR VSD • 11% HAVE PERSISTENT LEFT SVC • ABERRANT ORIGIN OF THE LEFT CORONARY 5 TO 15%. LAD ARISES FROM RCA • 20 TO 25% HAVE A RIGHT SIDED AORTIC ARCH • <5% HAVE MAPCAs

ANATOMIC VARIANTS • TET PULMONARY ATRESIA WITH OR WITHOUT MAPCAs • TET WITH ABSENT

ANATOMIC VARIANTS • TET PULMONARY ATRESIA WITH OR WITHOUT MAPCAs • TET WITH ABSENT PULMONARY VALVE • TET WITH DOUBLE OUTLET RIGHT VENTRICLE • TET WITH ATRIOVENTRICULAR SEPTAL DEFECT

PHYSIOLOGY AND CLINICAL PRESENTATION • PRESENTATION VARIES DEPENDING ON THE SEVERITY OF RVOTO •

PHYSIOLOGY AND CLINICAL PRESENTATION • PRESENTATION VARIES DEPENDING ON THE SEVERITY OF RVOTO • MILD OR NO CYANOSIS WILL PRESENT LATER WHEN RVOTO WORSENS • CLUBBING IS SEEN IN LONG STANDING CYANOSIS • TET-SPELLS ARE EPISODES OF CYANOTIC HYPOXAEMIC ATTACKS. TREATMENT CONSISTS OF INCREASING LV AFTERLOAD. Fyler DC (1992) Tetralogy of Fallot. In: Keane JF, Dyler DC, Lock JE, Nadas’ (eds) Pediatric cardiology. Hanley & Belfus, Philadelphia, pp 471 -491

NATURAL HISTORY • VARIABLE AND DEPENDS ON THE SEVERITY OF RVOTO • 25% WITH

NATURAL HISTORY • VARIABLE AND DEPENDS ON THE SEVERITY OF RVOTO • 25% WITH SEVERE RVOTO DIE IN THE FIRST YEAR IF NOT SURGICALLY TREATED • IF NOT TREATED 40% DIE BY AGE 3 YEARS, 70% BY AGE 10 YEARS AND 95% BY AGE OF 40 YEARS. • MAJOR CAUSES OF DEATH IF UNTREATED INCLUDES HYPOXAEMIC SPELLS AT 62%, CVI AT 17% AND BRAIN ABSCESSES AT 13% • HIGHEST RISK OF DEATH IS IN THE FIRST YEAR AND THEN IT REMAINS CONSTANT UNTIL AGE 25 YEARS AND THEN INCREASES. Loh TF, Ang YH, Wong YK et al (1973) Fallot’s tetralogy-natural history. Singap Med J 14: 169 -171

DIAGNOSIS • PHYSICAL EXAM • CXR AND ECG

DIAGNOSIS • PHYSICAL EXAM • CXR AND ECG

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• http: //www. google. co. za/url? sa=i&rct=j&q=&esrc=s&source=images&cd=&cad=rja&uact=8&ved=&url=http%3 A%2 F%2 Fwww. nairaland. com%2 F 1235070%2 Fmedicalrecall&ei=84 u. RVdmt. A 4 KL 7 Aap 7 o. Xw. DQ&bvm=bv. 96783405, d. ZGU&psig=AFQj. CNFTp 8 JE 2 OYMktwu. U 8 i. QAj. P_DT 2 A 1 w&ust=1435688307586797

DIAGNOSIS • LAB STUDIES – POLYCYTHEMIA WITH CYANOSIS – STUDIES FOR DELETION ON LONG

DIAGNOSIS • LAB STUDIES – POLYCYTHEMIA WITH CYANOSIS – STUDIES FOR DELETION ON LONG ARM OF CHROMOSOME 22. ACRONYM CATCH-22 • ECHOCARDIOGRAPHY – – – DEFINITIVE DIAGNOSTIC MODALITY NUMBER AND LOCATION OF VSD’s ANATOMY AND PULMONARY ARTERIES CORONARY ARTERY PATTERN PRENATAL DIAGNOSIS ACCURATE • CATHETERIZATION – HIGHLY ACCURATE AND INVASIVE – WHEN SUSPECTING MAPCAs – INTERVENTION

SURGICAL TREATMENT TET ASYMPTOMATIC >4 MONTHS & >4 KG <1 MONTH REPAIR 1 –

SURGICAL TREATMENT TET ASYMPTOMATIC >4 MONTHS & >4 KG <1 MONTH REPAIR 1 – 6 MONTHS SHUNT >6 MONTHS >4 KG REPAIR <4 KG SHUNT

Surgical repair • Historical perspective – augmenting blood flow to the lungs, Blalock and

Surgical repair • Historical perspective – augmenting blood flow to the lungs, Blalock and Taussig shunt. – Lillehei et al, using controlled cross-circulation, performed first Tet correction in 1954 – When to repair depends in part on the degee of cyanosis.

Surgical repair • Palliation – Blalock-Taussig shunt or modification (subclavian artery-to-pulmonary artery shunt). –

Surgical repair • Palliation – Blalock-Taussig shunt or modification (subclavian artery-to-pulmonary artery shunt). – Waterston shunt (ascending aorta-to-right pulmonary artery). – Potts shunt (descending aorta-to-left pulmonary artery). – Central interposition tube graft. – Infundibular resection (Brock procedure) or pulmonary valvotomy. – RV-to-PA conduit without VSD closure or with fenestrated closure

Surgical repair • Technique ( first series late 1960’s) – VSD repair via right

Surgical repair • Technique ( first series late 1960’s) – VSD repair via right ventriculotomy – Resection of the RVOT muscle bundles and pulmonary valve • Long term results – Aortic and pulmonary valve insufficiency – RV dilatation – Reinterventions

Surgical repair Recent era from 1970 to present • Complete repair in younger age

Surgical repair Recent era from 1970 to present • Complete repair in younger age including neonates Barratt-Boyes BG, Neutze JM (1973) Primary repair of tetralogy of Fallot in infancy using profound hypothermia with circulatory arrest and limited cardiopulmonary bypass: a comparison with conventional two stage management. Ann Surg 178: 406– 411

Surgical repair Neonatal surgery: Mc. Millan noted that primary VSD closure had high incidence

Surgical repair Neonatal surgery: Mc. Millan noted that primary VSD closure had high incidence of heart block and death. Bonchek and Starr concluded that early repair was advantageous with less mortality and prevention of worsening fibrosis with RVOT Bonchek LI, Starr A, Sunderland CO et al (1973) Natural history of tetralogy of Fallot in infancy. Circulation 48: 392– 397

Surgical repair Neonatal surgery: Shunt first then complete repair? Two-stage repair has high mortality(

Surgical repair Neonatal surgery: Shunt first then complete repair? Two-stage repair has high mortality( 20% early and 6% late with shunts). Primary repair 8% Barratt-Boyes BG, Neutze JM (1973) Primary repair of tetralogy of Fallot in infancy using profound hypothermia with circulatory arrest and limited cardiopulmonary bypass: a comparison with conventional two stage management. Ann Surg 178: 406– 411

Surgical repair Transannular patch technique: • Small PV annulus patients have improved relief of

Surgical repair Transannular patch technique: • Small PV annulus patients have improved relief of RVOT • Results in regurgitation of PV with RV volume load • Long term RV myocardial dysfunction and ventricular arrhythmias Blackstone EH, Kirklin JW, Pacifico AD. Decision-making in the repair of tetralogy of Fallot based on intraoperative measurements of the pulmonary arterial outflow tract. J Thorac Cardiovasc Surg. 1979; 77: 526 -532.

Surgical repair Transatrial–transpulmonary repair: • To deal with complications related to the use of

Surgical repair Transatrial–transpulmonary repair: • To deal with complications related to the use of the TA Patch technique • Pulmonary valve sparing technique • Avoid resection of the RV muscle Right ventricular to Pulmonary artery conduit • Tiny pulmonary arteries • Aberrant origin of coronay arteries • Type of conduit used is surgeon dependent

Future • Tissue engineered valves and valved conduits • Percutaneous valves • Genetic manipulation

Future • Tissue engineered valves and valved conduits • Percutaneous valves • Genetic manipulation as a therapeutic tool

Conclusion Initially an incurable condition, then a condition for palliation, now it is a

Conclusion Initially an incurable condition, then a condition for palliation, now it is a curable condition!

References 1. 2. Morales DL, Zafar F, Fraser CD Jr. Tetralogy of Fallot repair:

References 1. 2. Morales DL, Zafar F, Fraser CD Jr. Tetralogy of Fallot repair: the right ventricle infundibulum sparing (RVIS) strategy. Semin Thorac Cardiovasc Surg Pediatr. Card Surg Annu. 2009: 54 -58. Cebotari S, Lichtenberg A, Tudorache I et al (2006) Clinical application of tissue engineered human heart valves using autologous progenitor cells. Circulation 114(Suppl 1): 132– 137