SURGICAL MANAGEMENT OF D-TGA VSD LVOTO

Transcription

1 SURGICAL MANAGEMENT OF D-TGA VSD LVOTO ABDULRAOOF ALSAEEDI FRCSI, FRCSED, MBA. TGA, VSD, LVOTO Left ventricular outflow tract obstruction (LVOTO) is present at birth in perhaps 20 33% of infants with transposition of the great arteries (TGA), and is more commonly associated with ventricular septal defect (VSD). Sarris GE et al. J Thorac Cardiovasc Surg 2006; 132: Shribastava Set al Circulation 1976; 54: Wernovsky G et al. J Am Coll Cardiol 1990; 16:

2 Types of LVOTO Dynamic LVOTO was defined as LVOTO without LVOT abnormalities requiring resection. Fixed LVOTO was defined as LVOTO with LVOT abnormalities that could or could not be resected. Fixed LVOTO Fixed LVOTO defined as pulmonary valve (PV) z-score -2.0 or LVOT gradient >20 mm Hg in the presence of anatomic subvalvar stenosis. 2

3 TGA, VSD, LVOTO Neonatal ASO LVOTO resection Non-Neonatal intervention Delayed ASO Aortic Translocation Rastelli OP, REV Single Ventricle palliation. 3

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5 Case no days, 3 Kg Male TGA, PS, SubPS MR, Single coronary Case no. 2 2 Months Male 4.8 Kg DORV, TGA, Sever PS and subps. 5

6 Case no.3 One year 7.5 KG DORV, TGA, PS and SubPS, Restrictive VSD, disconnected LPA, S/P RBTS. TGA, VSD, LVOTO Feasibility of the option. (anatomy and surgical experience). Long term results of different options. 6

7 Anatomical Factors Affecting surgical decision VSD (position, size, boundaries) Cause of LVOTO (cause and severity) Relation of GA Coronary anatomy. Anatomical Factors Thirty-three specimens with TGA LVOTO. LVOTO resection and pulmonary valvotomy frequently permits an ASO. M. Hazekamp et al. / European Journal of Cardio-thoracic Surgery 31 (2007) Inlet VSD, impossibility of VSD enlargement, distant aorta and small right ventricle make the Nikaidoh procedure the best option. Mitral anomalies preventing LVOTO relief can make biventricular repair impossible. 7

8 VSD in TGA Subpulmonary( perimembranous subpulmonary VSDs, muscular subpulmonary VSDs). Doubly committed. Subaortic (perimembranous and muscular/anterior subaortic). Inlet VSD. Remote VSDs. Specimen : view from the opened LV. The VSD extends into the inlet septum. Specimen : view from the opened RV. A LVto-aorta tunnel will obstruct the tricuspid valve. ARastelli operation is therefore not possible. A Nikaidoh rocedure is the only way to obtain a biventricular repair. (A) (B) TGA, VSD TV: tricuspid valve. MV: mitral valve; PV: pulmonary valve. 8

9 TGA, VSD View from the opened RV. A probe is positioned in the muscular subpulmonary VSD. A LV-to-aorta tunnel (Rastelli procedure) will result in important volume loss of the RV. For that reason a Nikaidoh procedure was considered to be the best option. TV: tricuspid valve. View from the opened RV. A probe is positioned in the muscular subpulmonary VSD. A LV-to-aorta tunnel (Rastelli procedure) will result in important volume loss of the RV. For that reason a Nikaidoh procedure was considered to be the best option. TV: tricuspid valve. TGA, VSD 9

10 Levels of LVOTO in TGA 1) Bicuspid pulmonary valve or pulmonary valve dysplasia. 2)Posterior deviation of the infundibular septum. 3) Fibromuscular ridge. 4) Outlet accessory tissue from the membranous septum or mitral apparatus. Cause of LVOTO view from the opened LV. A probe is positioned in the pulmonary artery and extends into the VSD and LV. The VSD is perimembranous and subpulmonary. The mitral valve has a cleft anterior leaflet and straddles into the RV. This prevents septation, thus making biventricular repair impossible. MV: mitral valve. 10

11 Cause of LVOTO PV How small PV annulus that prevent patient from receiving the standard operation of TGA i.e ASO?. Aortic Z value of PV LVOT Z value LVOTO complexity score Park et al 11

12 Park et al 12

13 TGA, VSD, LVOTO Patients with a pulmonary valve z-score >-3.5, LVOT z-score >-5, and complexity score <2.4 underwent successful neonatal ASO. The LVOTO complexity score could be a useful tool for neonatal decision-making. Y. Kotani et al The Hospital for Sick Children, Toronto, ON, Canada ASO in TGA VSD LVOTO Actuarial freedom from a recurrent left ventricular outflow tract obstruction in (A) all patients and in (B) patients with preoperative pulmonary valve z-score of 1.7 or less (green) vs with a z-score exceeding 1.7 (blue). KALFA ET AL Ann Thorac Surg 2013;95:

14 Bicuspid Pulmonary Valve Angeli et al. ASO is a safe option for TGA associated with a wellfunctioning bicuspid pulmonary valve with low morbidity and mortality. Prevalence of AR was not particularly high. Even though ARD was frequent. E. Angeli et al. / European Journal of Cardio-thoracic Surgery (2011) Relation of GA Aorta right anterior to the pulmonary artery Aorta side by side to the pulmonary artery Aorta directly anterior Aorta right posterior Aorta left anterior to the pulmonary artery (with associated dextrocardia). 14

15 Coronary Anatomy coronary artery patterns that prevent Aortic Translocation: Single coronary RL-2Cx Abnormal LAD course Another Reason not to do Nikaidoh procedure When the VSD is perimembranous, subaortic, anterior, and large. The aortic valve was big and the LVOT very small. Moving the aorta over a very limited distance would not have meant any improvement in the position of the tunnel LV-aorta. 15

16 Single Ventricle Option View from the opened LV. A probe is positioned in the pulmonary artery and extends into the VSD and LV. The VSD is perimembranous and subpulmonary. The mitral valve has a cleft anterior leaflet and straddles into the RV. This prevents septation, thus making biventricular repair impossible. When Both Nikaidoh and Rastelli procedure are possible, what to choose? 16

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18 The aortic translocation (Nikaidoh) procedure Freedom from death. One of 19 patients died in the cohort, for an actuarial freedom from death of 95%. No late deaths have occurred The aortic translocation (Nikaidoh) procedure Freedom from LVOT and RVOT reintervention. RVOT reinterventions have been required at rates similar to other procedures using valved conduits or valveless methods to reconstruct the RVOT and are currently 64% free of reintervention. The Nikaidoh procedure has been 100% free of reintervention on the LVOT. 18

19 The aortic translocation (Nikaidoh) procedure Summary of longitudinal follow-up of Nikaidoh, 20 Rastelli (W. G. Williams, MD, personal communication, 2006), 4,5 and REV procedures.3 Survival after the Rastelli procedure has been strik ingly similar (50%-60%) at 3 experienced centers. Reports on the midterm follow-up (>10 years) on the Lecompte procedure have been limited, reported as 79% at 15 years. The Nikaidoh procedure has been free of late mortality 19

20 TGA, VSD, LVOTO Boston children Hospital Emani et al (Circulation. 2009;120[suppl 1]:S53 S58.) Emani et al (Circulation. 2009;120[suppl 1]:S53 S58.) Progression of PV z-scores (measured by echocardiogram) in the postnatal period in patients undergoing delayed definitive repair (A) or primary neonatal repair (B). Horizontal lines of delayed definitive repair note median values. 20

21 Emani et al (Circulation. 2009;120[suppl 1]:S53 S58.) The aortic translocation (Nikaidoh) procedure:initial experience of 7 cases at PSCC Seven patients underwent a Nikaidoh procedure at a median age of 3.3. The median follow-up was 18 months (0.3-4 years). All patients survived. Right ventricular outflow tract reoperations were required in 2 patients (28.6%). No reoperations have been performed on the left ventricular outflow tract or aortic valve. No patient had any left ventricular outflow tract obstruction or aortic insufficiency more than mild 21

22 TGA, VSD, LVOTO Neonatal ASO PV Z-value >-2, resectable subps, Non-Neonatal intervention Delayed ASO PV Z-value >-2, resectable subps Aortic Translocation PV Z-value <-2, usual coronay pattern, Rastelli OP, REV PV value <-2, unusual coronary anatomy, VSD can be tunneled to Aorta Single Ventricle palliation. When all other options are not suitable.. Case no days, 3 Kg Male TGA, PS, SubPS MR, Single coronary 22

23 Case no.1 20 days, 3 Kg Male TGA, PS, SubPS MR, Single coronary. ASO, resection of SubPS Pulmonary Valvotomy MVRepair Case no. 2 2 Months Male 4.8 Kg DORV, TGA, Sever PS and subps. 23

24 Case no. 2 2 Months Male 4.8 Kg DORV, TGA, Sever PS and subps. Aortic Translocation. Case no. 2 2 Months Male 4.8 Kg DORV, TGA, Sever PS and subps. Aortic Translocation. 24

25 Case no.3 One year 7.5 KG DORV, TGA, PS and SubPS, Restrictive VSD, disconnected LPA, S/P RBTS. Case no.3 One year 7.5 KG DORV, TGA, PS and SubPS, Restrictive VSD, disconnected LPA, S/P RBTS. Rastelli OP 25

26 Conclusion LVOTO is present in significant percent 25-33% of patients with TGA VSD. When Aortic Z-value of PV > -2 and the cause of obstruction is resectable then neonatal arterial switch offer the best available options. Conclusion When PV z value <-2. then Aortic translocation offer a better choice when compare to Rastelli or REV procedures. Unusual coronary pattern and extremely small LVOT preclude Aortic translocation There are cases when single ventricle palliation is the only available option. 26

27 THANK YOU SHOKRAN 27