HLHS/HRHS Surgical Repair

HLHS Characteristics

• Aortic atresia & mitral atresia

• No valves on left side w/ hypoplastic LV

• Universally fatal unless operated on

Why is the neonate dependent on the DA for systemic output at birth?

• Systemic venous return & pulmonary venous return mix in the RA

• RVOT sends blood via the PA to the bilateral branch PAs AND sends blood via the PDA to the body (including the Ao which supplies the coronary arteries)

Stage 1 HLHS repair

• Norwood Procedure

• Source of pulmonary BF

• Allows RV to pump to both the lungs & the body

Goal 1 of Norwood Procedure & how to achieve it

• Relief of systemic (aortic) obstruction

• Achieved by Aortic Arch reconstruction

Goal 2 of Norwood Procedure & how to achieve it

• Adequate pulmonary BF

• Achieved by Systemic-PA shunt or RV-PA shunt

Goal 3 of Norwood Procedure & how to achieve it

• Allow adequate pulmonary drainage

• Achieved by Atrial septectomy

Goal 4 of Norwood Procedure & how to achieve it

• Maintain optimal PA growth

• Achieved by Adequate shunt,& avoid distortion

Goal 5 of Norwood Procedure & how to achieve it

• Preserve TV function

• Achieved by Avoiding vol overload of the RV

Goal 6 of Norwood Procedure & how to achieve it

• Optimize ventricular function

• Achieved by Avoiding pressure or vol overload of the RV

Norwood Procedure techniques

• BT shunt (RSA to RPA)

• Sano/Rastelli Shunt (RV to RPA)

Other option for Stage 1 HLHS repair

• Hybrid Procedure

• Same goals as a Norwood

Components of a Hybrid Procedure

• Bilateral PA bands

• Balloon atrial septostomy/placement of atrial stent

• PDA stent vs PGE1 infusions

Stage 2 repair for HLHS

• Bi-Directional Glenn/Hemi-Fontan

• Performed at 4-6 months old

• SVC to RPA shunt & remove BT or Sano shunt

Relative contraindications for Stage 2

• <6 weeks old

• MPAP > 30-40 mmHg

• PVR > 4 Woods units/m²

• Pulmonary venous obstruction

• Severe atrioventricular valve regurgitation or RV dysfunction

Stage 3 for HLHS repair

• Fontan Procedure

• IVC to RPA shunt

• Lateral Tunnel (intracardiac) or extracardiac

Purpose of the Fontan procedure

Direct all venous blood to the lungs Passively

Indications for MCS in cardiac failure (<15kg)

• Post-cardiotomy ventricular dysfunction

• Medically-refractory HF (cardiomyopathy or congenital heart dz)

Goal for MCS in cardiac failure (<15kg)

Bridge to recovery vs Bridge to transplantation

MCS options in cardiac failure (<15kg)

• VA ECMO

• Pulsatile paracorporeal device (Berlin heart)

• Continuous paracorporeal device (PediMag or RotaFlow)

MCS aims in cardiac failure (<15kg)

• ↑ tissue & organ perfusion

• ↑ QOL

• ↑ waitlist survival

Berlin Heart Characteristics

• Pneumatically driven

• Only FDA approved VAD in children <3kg

• Available in LVAD, RVAD, BiVAD, SVAD models

Maquet Rotaflow characteristics

• Mag-lev, continuous flow

• Temporary

• Up to 10 L/min of flow

Abbot PediMag characteristics

• Mag-Lev, continuous flow

• Temporary

• for children <20kg

• Up to 1.5 L/min of flow

Why use continuous flow devices?

• Better ventricular unloading than VA ECMO

• Better able to extend temporary support for longer periods of time than VA ECMO