Surgical Strategies for TOF Repair Yong Jin Kim M. D Surgical Strategies for TOF Repair Yong Jin Kim M.D. Seoul National University Children’s Hospital 1

Tetralogy of Fallot Definition Characterized by underdevelopment of right ventricular infundibulum with anterior & leftward displacement of infundibular ( conal, outlet ) septum & parietal extension. This displacement of infundibular septum is associated with RV outflow stenosis & large VSD. 99’ SNUCH TS

Tetralogy of Fallot Definition Classification 99’ SNUCH TS A congenital cardiac anomaly characterized by underdevelopment of the RV infundibulum with anterior & leftward displacement of the infundibular septum & parietal extension . This displacement of the infundibular septum is associated with RV outflow stenosis & large VSD . Classification Simple TOF TOF with AV canal TOF with absent pulmonary valve syndrome TOF and pulmonary atresia with well formed PDA TOF and pulmonary atresia with MAPCAs 99’ SNUCH TS

Backgrounds I 1. 1945 Blalock & Taussig 2. 1948 Sellors & Brock Subclavian - pulmonary artery anastomosis 2. 1948 Sellors & Brock Closed pulmonary valvotomy & infundibulotomy 3. 1954 Lillehei & Varco First successful repair using cross-circulation 99’ SNUCH TS

Backgrounds II 4. 1955 Kirklin 5. 1957 Warden and Lillehei First successful repair using pump oxygenator 5. 1957 Warden and Lillehei Patch enlargement of the infundibulum 6. 1959 Kirklin Transannular patching 7. 1963 Hudspeth Transatrial approach 99’ SNUCH TS

Backgrounds III 8. 1965 Rastelli 9. 1966 Ross Right ventricular-pulmonary artery conduit 9. 1966 Ross Valved extracardiac conduit 10. 1969 Barratt-Boyes & Neutze One-stage repair 99’ SNUCH TS

Surgical Strategies 1. Around 3 months with symptoms Early total correction 2. 1 - 2 months with severe symptoms Palliative shunt or early total correction 3. Asymptomatic and uncomplicated Definitive repair at 6 - 24 months 99’ SNUCH TS

Surgical Indications I 1. Diagnosis is generally an indication for repair 2. Urgency : Symptpms at presentation Associated lesions 3. Trend toward open correction in early infancy 99’ SNUCH TS

Surgical Indications II 1. Below 3 months with severe symptoms Early total correction 2. 1 - 2 months with severe symptoms Palliative shunt or early total correction 3. Asymptomatic & uncomplicated Definitive repair at 6 - 24 months 99’ SNUCH TS

Early Total Correction Advantages : Avoid risk & complication of palliative shunt Early correction of RVH Prevention of LV volume overload Early correction of chronic hypoxemia 99’ SNUCH TS

Palliation Disadvantages Advantages : PA distortion - complicating & increasing risk of subsequent complete repair Advantages : Lower mortality & RVOTO recurrence Rick factors of mortality : PA distortion from previous shunts More than one palliation 99’ SNUCH TS

Indications of Palliative Procedure 1. Anomalous coronary artery crossing RVOT 2. Extremely small pulmonary arteries 3. Unrelenting "tet" spells for several hours 4. Significant & severe associated lesions 99’ SNUCH TS

Mortality for Risk Factors Presence of multiple VSDs Down's syndrome Large aortopulmonary collaterals Complete AV canal defects Early age at presentation 99’ SNUCH TS

Total Correction The goals of operation 1. VSD closure 2. Relief of RVOT obstruction 3. Relief of pulmonary artery stenoses 99’ SNUCH TS

Transventricular Approach Vertical extension across annulus to relieve PS Division in parietal extension of infundibular septum to expose VSD Not to much resect muscle in infants 99’ SNUCH TS

Transventricular Approach 99’ SNUCH TS

Transventricular Approach amputation amputation a TV ant. leaflet TV septal leaflet Transection TV post. leaflet 99’ SNUCH TS

Transventricular Approach Hypoplastic PV AV VSD a a TV Condunction bundle 99’ SNUCH TS

Transventricular Approach 99’ SNUCH TS

Transventricular Approach 99’ SNUCH TS

Transatrial Approach By retracting TV leaflet or incising TV Relief of RVOT obstruction Preserving long-term RV function Limiting ventricular dysrhythmias Access to atrial septum - ASD closure 99’ SNUCH TS

Transatrial Approach 99’ SNUCH TS

Transatrial Approach 99’ SNUCH TS

Transatrial Approach 99’ SNUCH TS

Transatrial Approach 99’ SNUCH TS

Transatrial Approach Infundibular septum 99’ SNUCH TS

Limited Ventriculotomy Patch enlargement in the infundibulum for hypoplasia of infundibular septum Muscle resection is not always required Leave a small ASD in infants 99’ SNUCH TS

Infundibular Patch Infundibular patch 99’ SNUCH TS

Methods of RVOT Reconstruction Long ventriculotomy : Long-term outcome ↓ Transatrial approach : In some , small ventriculotomy is necessary for the patch of hypoplastic infundibulum Limited ventriculotomy : Less than the half length Preserve late right ventricular function Adequate enlargement of hypoplastic RVOT 99’ SNUCH TS

Relief of RVOT Obstruction PT Pul. valve Ao 99’ SNUCH TS

Relief of RVOT Obstruction Dacron Pericardium 99’ SNUCH TS

Transannular Patch Pulmonary annular Z-value > - 2 : postrepair RV/LV pressure ratios (< 0.7) < - 3 : transannular patch Hegar dilator : assess annulus size Patch : autopericardium, Dacron, Gore-Tex 99’ SNUCH TS

Transannular Patch RV dysfunction requiring reoperation for PI Not employed unless necessary for RVOT Limit PI to preserve long-term RV dynamics Monocusp valve for short-term Homograft for the long-term 99’ SNUCH TS

Transannular Patch Transannular patch Tied Transannular patch 99’ SNUCH TS

Pulmonary Artery Stenoses Obstruction in main PA branch Previous shunt Tissue from ductus arteriosus Spectrum of anatomy of defect Angioplastic technique Patch to bifurcation & LPA 99’ SNUCH TS

Relief of Pulmonary Artery Stenoses Distal aspect of transannular patch Blunt and not tapered Obstruction in MPA Distal stenosis in PA Stent at operation Balloon angioplasty later 99’ SNUCH TS

Relief of Pulmonary Artery Stenoses LPA Pericardium Dacron patch 99’ SNUCH TS

Relief of Pulmonary Artery Stenoses LPA Pericardial patch Dacron patch PT RPA Ao 99’ SNUCH TS

Anomalous Left Coronary Artery Crossing the RVOT (I) Transverse incision in infundibulum & separate incision in the MPA - patching of pulmonary artery, valvotomy Dissecting with patch beneath coronary artery - RV distension causing coronary ischemia by stretching 99’ SNUCH TS

Anomalous Left Coronary Artery Crossing the RVOT (II) Systemic-pulmonary artery shunt followed by RV-PA conduit Complete repair with homograft in infancy 99’ SNUCH TS

TOF and Pulmonary Atresia Surgical strategies - Initial ductal stabilization with PG - Shunt or total correction - 5mm RMBT in full-term baby last up to 18 months or 2 years - In LPA coarctation, early complete repair within a few months or 4mm LMBT 99’ SNUCH TS

TOF & Pulmonary Atresia Assessment of repair quality pRV/LV pressure ratios Postrepair RV/LV ratio above 0.7 Unfavorable outcome Early repair is advantageous before spells 99’ SNUCH TS

TOF and Pulmonary Atresia Morphology Differentiating features from TOF 1. No blood from RV to PA 2. Pulmonary artery anomalies 3. Aortopulmonary collaterals 99’ SNUCH TS

TOF and Pulmonary Atresia Definite repair 1. Closure of VSD 2. Continuity between RV & PA 3. Occlusion of collaterals & shunts 99’ SNUCH TS

TOF and Pulmonary Atresia Preparation for definitive repair 1. Maximize the pulmonary artery The size & distribution 2. Maintain the adequate PBF 3. Avoid the excessive PBF 99’ SNUCH TS

TOF and Pulmonary Atresia Selection for final repair 1. Central combined Rt & Lt PA area at least 50-75% of predicted normal 2. Distribution of unobstructed confluent PAs equivalent to at least one whole lung 3. Presence of a predominant Lt to Rt shunt without restrictive RV-PA connection 99’ SNUCH TS

TOF with Complete AV Canal General principle of complete repair : At a time when heart is volume loaded - hazard relate to operative length & difficulty in dividing single AV valve : Shunt when cyanosis & later complete repair until 12-24 months 99’ SNUCH TS

TOF with Complete AV Canal CHF due to AV regurgitation & not high PBF complete repair Heart failure with poor PBF simply repair of AV valve combined with shunt CHF because of inadequate RVOTO complete repair at 3 to 4 months Inadequate shunt & no longer volume loaded not wait 99’ SNUCH TS

TOF with Complete AV Canal 99’ SNUCH TS

TOF with Absent PV Syndrome Definition (I) 1. Ringlike and stenotic malformation rather than absence of PV with failure of development 2. Hugely dilated or aneurysmal central PA 3. Tightly stenotic pulmonary annulus with free PI against high PVR in utero 99’ SNUCH TS

TOF with Absent PV Syndrome Definition (II) 1. Abnormal tufted segmental PA branching 2. Branching arteries : spread peripherally with little change in size entwing and compressing associated bronchi 3. Bronchi : deficient or defective cartilage formation, abnormal broncho-alveolar multiplication 99’ SNUCH TS

TOF with Absent PV Syndrome Aim : Alleviate bronchial compression Prevent right-sided heart failure Palliative procedures : not successful Surgery : In a one stage procedure VSD closure Pulmonary artery plication Insertion of RV-PA homograft 99’ SNUCH TS

TOF with Absent PV Syndrome Timing - related to symptom presentation Neonate : urgent repair Infants : deferred selectively RVOT reconstruction Transannular patch - not wise ( PI, RV failure) Insertion of a valved conduit - valved > monocusp Aortic or pulmonary homograft - larger homograft 99’ SNUCH TS

TOF with Absent PV Syndrome Operative techniques VSD closure Insertion of homograft - in infants for increased PVR - severe intrapulmonary stenoses Reduction pulmonary angioplasty 99’ SNUCH TS

TOF with Absent PV Syndrome 99’ SNUCH TS

TOF with Absent PV Syndrome 99’ SNUCH TS