Hybrid Stage I Palliation / Bilateral PAB

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Hybrid Stage I Palliation / Bilateral PAB Jeong-Jun Park Dept. of Thoracic & Cardiovascular Surgery Asan Medical Center, University of Ulsan

CASE 1 week old neonate with HLHS GA 38 weeks Birth weight 3.0Kg No definite risk factor

HLHS Norwood op. Hybrid procedure Norwood after bilat. PAB 2~4wks Bilat. PAB with continuous infusion of PGE1 Bilat. PAB + ductal stent Hybrid procedure : bridge to TPL Rapid 2 stage Norwood op Selective cerebral perfusion vs DHCA Arch repair: patch vs direct anastomosis BT shunt vs RV-PA conduit Modification of RV-conduit anastomosis Norwood + BCS TPL

Risk factors of Norwood operation Operative mortality: 30~40% approaching 10% Low birth wt Prematurity Significant TR / Restrictive IAS / Poor ventricular function Preoperative shock Renal failure Small asc. Aorta Extracardiac abnormalities Chromosomal abnormalities

Norwood operation Unstable in-parallel circulation of Norwood physiology (BT shunt) Surgical stress driven by CPB and systemic inflammatory response Hybrid palliation / Rapid 2 stage Norwood operation For high risk patients Shifting the major surgical stage to later in life

Hybrid approach for HLHS: intermediate results after learning curve Galantowicz et al Ann Thorac Surg 2008;85:2063-2071

Hybrid vs Norwood operation Equivalent but not superior stage 1 palliation survival Comparable 1 year survival to conventional Norwood palliation Comparable pre-stage II hemodynamics and pulmonary artery growth Preserved ventricular function in stage II palliation not randomized trial, different population, recently introduced

Predictors of RAAO after Hybrid palliation 29% of pts Aortic atresia Small aortic root size Larger angiographic angle btw PDA and isthmus Higher retrograde aortic arch velocities Nationwide Children s Hosp, Ohio, Pediatr Cardiol 2011

Rapid 2 stage Norwood operation : Modification of conduit anastomosis

Rapid 2 stage Norwood operation : Modification of conduit anastomosis

HLHS Increasing surgical and interventional options. Remarkable improvements in results, with ongoing evolution Case by case Consideration of hospital s experiences and results

Bilateral PA banding in high risk patients with HLHS Department of Cardiovascular Surgery Rapid Stage bilateral PA banding Progress of Fetal echocardiogram Progress of Maternal transfer Progress of Hybrid strategy Intact atrial septum Preoperative shock Low birth weight Bilateral PAB Norwood procedure : 2-4 weeks time Disadvantage of prolonged use of PGE1 Interstage high mortality after bil.pab Acceptable outcome of 1st stage palliation

Bilateral PA banding in high risk patients with HLHS Result Await Norwood 1 Low body weight (<2kg) 6(4) Indication of bilateral PAB 15 IAS 5(2) Low body weight + tiny PFO 2 CAVB 1 3 Early Death 1: High flow 2: PH 3 death after Norwood 1: Ischemic event after Norwood (On ECMO) 2: Severe PH after Norwood Norwood procedure 11 BDG 8 TCPC 2 Shock 1

Management of CHD Conventional therapy Surgery Catheter intervention Hybrid procedure combination of surgical & interventional techniques less invasive, less trauma to patient

Objectives of Stage 1 palliation for neonates with SV physiology Control PBF Unobstructed systemic output Unobstructed coronary flow Unrestricted atrial septum avoiding CPB, cardioplegic arrest circulatory arrest in neonatal period

Reverse BT shunt? Arch obstruction by ductal stent 10%(4/40 cases) Galantowicz 2008 data Potential of coronary steal More complicated postop course No longer use of reverse BT shunt

HLHS Marked hypoplasia or absence of the LV + Severe hypoplasia of the Asc. Aorta Ductus dependent systemic circulation Fatal lesion: without surgical intervention, 25% of cardiac death during the 1st wk of life Still high surgical and interstage mortality Anatomic subtype AA + MS 45% AA+ MA 41% AS + MS 13% AS + MA Significant TR 8~10% Dx: echo

HLHS Initial support Maintenance of ductal patency: PGE1 Qp/Qs balance Correction of metabolic acidosis

HLHS Priciples of 1st stage palliation Unobstructed systemic flow Unrestrictive ASD: Adequate mixing Reliable PBF General aims Preserve ventricular fxn by avoiding pressure and vol. overload Minimize PVR by avoiding excessive PBF or PVO Maintain optimal PA growth: adequate size and freedom from distortion

HLHS Classic Norwood

HLHS Classic Norwood

Hybrid procedure Beating heart/ short term CBP Extensive use of introperative imaging techniques (Fluoroscope/TEE/ICE) Devices cath. Lab equipments Hybrid procedure combination of surgical & interventional techniques less invasive, less trauma to patient

Hybrid Procedure GA/median sternotomy/ off CPB Bilateral PAB Ductal stenting MPA sheath with pursestring C-arm fluoroscoic guide complete cover PDA BAS at cath Lab 3.5 mm GTVG(3.0 mm for <2.5 kg) RT: SVC-AAo, Lt: LPA origin just prior to discharge mpg 8 mmhg by Echo Reverse BT shunt

PAB size PTFE graft BWT less than 2.5kg : 3.0mm PTFE graft more than 2.5kg : 3.5mm PTFE graft PAB flow velocity : 3.5-4.0m/sec SaO2 : 80-85% on room air cut into 5mm wide and opened, then this was re-sutured around through the pulmonary artery