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Surgical Strategies for TOF Repair Yong Jin Kim M.D. Seoul National University Childrens Hospital.

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Presentation on theme: "Surgical Strategies for TOF Repair Yong Jin Kim M.D. Seoul National University Childrens Hospital."— Presentation transcript:

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

2 99 SNUCH TS Tetralogy of Fallot Definition Definition Characterized by underdevelopment of right ventricular Characterized by underdevelopment of right ventricular infundibulum with anterior & leftward displacement of infundibulum with anterior & leftward displacement of infundibular ( conal, outlet ) septum & parietal extension. infundibular ( conal, outlet ) septum & parietal extension. This displacement of infundibular septum is associated This displacement of infundibular septum is associated with RV outflow stenosis & large VSD. with RV outflow stenosis & large VSD.

3 99 SNUCH TS Tetralogy of Fallot Definition 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

4 99 SNUCH TS Backgrounds I Blalock & Taussig Subclavian - pulmonary artery anastomosis Sellors & Brock Closed pulmonary valvotomy & infundibulotomy Lillehei & Varco First successful repair using cross-circulation

5 99 SNUCH TS Backgrounds II Kirklin First successful repair using pump oxygenator Warden and Lillehei Patch enlargement of the infundibulum Kirklin Transannular patching Hudspeth Transatrial approach

6 99 SNUCH TS Backgrounds III Rastelli Right ventricular-pulmonary artery conduit Ross Valved extracardiac conduit Barratt-Boyes & Neutze One-stage repair

7 99 SNUCH TS Surgical Strategies 1. Around 3 months with symptoms Early total correction Early total correction months with severe symptoms Palliative shunt or early total correction Palliative shunt or early total correction 3. Asymptomatic and uncomplicated Definitive repair at months Definitive repair at months

8 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

9 99 SNUCH TS Surgical Indications II 1. Below 3 months with severe symptoms Early total correction months with severe symptoms Palliative shunt or early total correction 3. Asymptomatic & uncomplicated Definitive repair at months

10 99 SNUCH TS Early Total Correction o o Advantages : Avoid risk & complication of palliative shunt Early correction of RVH Prevention of LV volume overload Early correction of chronic hypoxemia

11 99 SNUCH TS Palliation o Disadvantages : PA distortion - complicating & increasing risk : PA distortion - complicating & increasing risk of subsequent complete repair of subsequent complete repair o Advantages : Lower mortality & RVOTO recurrence : Lower mortality & RVOTO recurrence o Rick factors of mortality : PA distortion from previous shunts More than one palliation More than one palliation

12 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

13 99 SNUCH TS Mortality for Risk Factors o o Presence of multiple VSDs o o Down's syndrome o o Large aortopulmonary collaterals o o Complete AV canal defects o o Early age at presentation

14 99 SNUCH TS Total Correction o o The goals of operation 1. VSD closure 2. Relief of RVOT obstruction 3. Relief of pulmonary artery stenoses

15 99 SNUCH TS Transventricular Approach o o Vertical extension across annulus to relieve PS o o Division in parietal extension of infundibular septum to expose VSD o o Not to much resect muscle in infants

16 99 SNUCH TS Transventricular Approach

17 99 SNUCH TS amputation TV ant. leaflet TV post. leaflet TV septal leaflet Transection a Transventricular Approach

18 99 SNUCH TS AV VSD a Condunction bundle Hypoplastic PV A TV a Transventricular Approach

19 99 SNUCH TS Transventricular Approach

20 99 SNUCH TS Transventricular Approach

21 99 SNUCH TS Transatrial Approach o o By retracting TV leaflet or incising TV o o Relief of RVOT obstruction Preserving long-term RV function Limiting ventricular dysrhythmias o o Access to atrial septum - ASD closure

22 99 SNUCH TS Transatrial Approach

23 99 SNUCH TS Transatrial Approach

24 99 SNUCH TS Transatrial Approach

25 99 SNUCH TS Transatrial Approach

26 99 SNUCH TS Infundibular septum Transatrial Approach

27 99 SNUCH TS Limited Ventriculotomy o o Patch enlargement in the infundibulum for hypoplasia of infundibular septum o o Muscle resection is not always required o o Leave a small ASD in infants

28 99 SNUCH TS Infundibular patch Infundibular Patch

29 99 SNUCH TS Methods of RVOT Reconstruction o o Long ventriculotomy : Long-term outcome o o Transatrial approach : In some, small ventriculotomy is necessary for the patch of hypoplastic infundibulum o o Limited ventriculotomy : Less than the half length Preserve late right ventricular function Adequate enlargement of hypoplastic RVOT

30 99 SNUCH TS PT Ao Pul. valve Relief of RVOT Obstruction

31 99 SNUCH TS Pericardium Dacron Relief of RVOT Obstruction

32 99 SNUCH TS Transannular Patch o o Pulmonary annular Z-value > - 2 : postrepair RV/LV pressure ratios (< 0.7) < - 3 : transannular patch o o Hegar dilator : assess annulus size o o Patch : autopericardium, Dacron, Gore-Tex

33 99 SNUCH TS Transannular Patch o o RV dysfunction requiring reoperation for PI o o Not employed unless necessary for RVOT o o Limit PI to preserve long-term RV dynamics Monocusp valve for short-term Homograft for the long-term

34 99 SNUCH TS Transannular patch Tied Transannular patch Transannular Patch

35 99 SNUCH TS P ulmonary Artery Stenoses o o Obstruction in main PA branch Previous shunt Tissue from ductus arteriosus Spectrum of anatomy of defect o o Angioplastic technique Patch to bifurcation & LPA

36 99 SNUCH TS Relief of Pulmonary Artery Stenoses o o Distal aspect of transannular patch Blunt and not tapered o o Obstruction in MPA o o Distal stenosis in PA Stent at operation Balloon angioplasty later

37 99 SNUCH TS LPA Pericardium Dacron patch Relief of Pulmonary Artery Stenoses

38 99 SNUCH TS LPA RPA PT Ao Pericardial patch Dacron patch Relief of Pulmonary Artery Stenoses

39 99 SNUCH TS Anomalous Left Coronary Artery Crossing the RVOT (I) o o Transverse incision in infundibulum & separate incision in the MPA - patching of pulmonary artery, valvotomy o o Dissecting with patch beneath coronary artery - RV distension causing coronary ischemia by stretching

40 99 SNUCH TS Anomalous Left Coronary Artery Crossing the RVOT (II) o o Systemic-pulmonary artery shunt followed by RV-PA conduit o o Complete repair with homograft in infancy

41 99 SNUCH TS TOF and Pulmonary Atresia o o 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

42 99 SNUCH TS TOF & Pulmonary Atresia Assessment of repair quality Assessment of repair quality pRV/LV pressure ratios pRV/LV pressure ratios Postrepair RV/LV ratio above 0.7 Postrepair RV/LV ratio above 0.7 Unfavorable outcome Unfavorable outcome Early repair is advantageous before spells Early repair is advantageous before spells

43 99 SNUCH TS TOF and Pulmonary Atresia Morphology Morphology Differentiating features from TOF Differentiating features from TOF 1. No blood from RV to PA 1. No blood from RV to PA 2. Pulmonary artery anomalies 2. Pulmonary artery anomalies 3. Aortopulmonary collaterals 3. Aortopulmonary collaterals

44 99 SNUCH TS TOF and Pulmonary Atresia Definite repair Definite repair 1. Closure of VSD 1. Closure of VSD 2. Continuity between RV & PA 2. Continuity between RV & PA 3. Occlusion of collaterals & shunts 3. Occlusion of collaterals & shunts

45 99 SNUCH TS TOF and Pulmonary Atresia Preparation for definitive repair Preparation for definitive repair 1. Maximize the pulmonary artery 1. Maximize the pulmonary artery The size & distribution The size & distribution 2. Maintain the adequate PBF 2. Maintain the adequate PBF 3. Avoid the excessive PBF 3. Avoid the excessive PBF

46 99 SNUCH TS TOF and Pulmonary Atresia Selection for final repair Selection for final repair 1. Central combined Rt & Lt PA area at least 50-75% of predicted normal 50-75% of predicted normal 2. Distribution of unobstructed confluent PAs equivalent to at least one whole lung equivalent to at least one whole lung 3. Presence of a predominant Lt to Rt shunt without restrictive RV-PA connection without restrictive RV-PA connection

47 99 SNUCH TS TOF with Complete AV Canal o o 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 : Shunt when cyanosis & later complete repair until months until months

48 99 SNUCH TS TOF with Complete AV Canal o o CHF due to AV regurgitation & not high PBF complete repair o o Heart failure with poor PBF simply repair of AV valve combined with shunt o o CHF because of inadequate RVOTO complete repair at 3 to 4 months o Inadequate shunt & no longer volume loaded not wait not wait

49 99 SNUCH TS TOF with Complete AV Canal

50 99 SNUCH TS TOF with Absent PV Syndrome o o 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

51 99 SNUCH TS TOF with Absent PV Syndrome o o 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

52 99 SNUCH TS TOF with Absent PV Syndrome o o Aim : Alleviate bronchial compression Prevent right-sided heart failure o o Palliative procedures : not successful o o Surgery : In a one stage procedure VSD closure Pulmonary artery plication Insertion of RV-PA homograft

53 99 SNUCH TS TOF with Absent PV Syndrome o o Timing - r elated to symptom presentation Neonate : urgent repair Infants : deferred selectively o o RVOT reconstruction Transannular patch - not wise ( PI, RV failure) Insertion of a valved conduit - valved > monocusp Aortic or pulmonary homograft - larger homograft

54 99 SNUCH TS TOF with Absent PV Syndrome o o Operative techniques VSD closure Insertion of homograft - in infants for increased PVR - severe intrapulmonary stenoses Reduction pulmonary angioplasty Reduction pulmonary angioplasty

55 99 SNUCH TS TOF with Absent PV Syndrome

56 99 SNUCH TS TOF with Absent PV Syndrome


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