Bidirectional Cavo-pulmonary Anastomosis

Seoul National University Hospital

Department of Thoracic & Cardiovascular Surgery

Major Concerns of BCPC

1. Time of BCPC 3~6 months of age

2. Time of complete palliation 2~3 years of age

3. Pulmonary A-V malformation 1) Duration of BCPC (within 2~3 years)

2) Time of BCPC (age of less than 2months old)

3) Isomerism (left) and heterotaxy syndrome 4) Extra source of pulmonary blood flow

SVC Flow to Total Cardiac Output

1. At birth : 50% of total cardiac output

2. At 2 years : 59% of total cardiac output

3. At 6 years : 35% of total cardiac output

4. At adult : 1/3 of total cardiac output

Advantages of Bidirectional CP Shunt

1. Relief of volume load

2. Improve of ventricular & AV valve function

3. Avoidance of pulmonary arterial distortion

4. Simplification of eventual Fontan procedure

5. Prevention of pulmonary vascular disease

Advantages & Disadvantages of BCPS

1. Advantages 1) Increase effective PBF 2) Decrease cardiac volume overload 3) Maintain PA shape 4) Decrease PVOD 5) Correct the associated cardiac anomalies (PA distortion, AVV regurgitation. VSD extension, DKS, ASO, )

2. Disadvantages 1) Decrease PBF with aging 2) Retarded PA development 3) Pulmonary AV fistula 4) Venovenous collateral circulation 5) Abnormal distribution of PBF 6) Additional risks due to secondary operation

Ventricular Function after BCPC

1. Immediate decrease in the cavity size

2. Concomitant increase in wall thickness

3. Reduction in myocardial wall stress or

afterload reduction

Collaterals after BCPC

Risk factors for development

• Subnormal oxygen content

• Decreased or nearly absent pulsatility

• Decreased absolute volume & velocity of flow

• Lack of hepatic venous effluent

Inadequate Oxygenation after BCPC

1. Elevated PVR

2. Pulmonary venous obstruction

3. Progression of AV valve regurgitation

4. Progression of ventricular dysfunction

Improving Oxygenation after BCPC

1. Increase systemic cardiac output to increase t

he saturation of IVC

2. Ventilatory manipulation

3. Additional source of pulmonary blood flow (s

mall systemic to pulmonary artery shunt)

; The shunt will defeat one of the objectives

of the BCPS.

BCPC with Accessory Pulsatile Flow

• Surgeon must be aware of potential beneficial influence

on pulmonary vascular development and deleterious

impact by imposing a volume load on the ventricle.

* Volume load

* Pulmonary hypertension

* Morbidity & mortality

• Bidirectional Glenn allows single ventricle to adapt &

remodel in accordance with the reduced volume loading

with a consequently much reduced risk of mortality.

Auxilliary Pulmonary Flow on BCPC

1 Aim 1) Promoting pulmonary arterial growth

2) Preventing arteriovenous fistulas 2 Indication 1) PaO2 less than 30 mmHg 2) Older patients (beyond the toddler stage) 3) Antegrade flow by banding or tightening

3 Results 1) Increased arterial O2 saturation 2) Increased morbidity & mortality

Hemi-Fontan Operation

Hemi-Fontan OperationAlternative Method

Bidirectional Cavopulmonary Connection

BCPC

BCPC. Operative View

BCPC

Before BCPC. Operative View

• Diagnosis ; Tricuspid atresia, Ib , 3 months old

SVC

BCPC. Operative View

BCPC

BCPC. Operative View

BCPC

Pulmonary Arteriovenous Malformations

The role of angiogenesis in PAVM development

• The liver is known to produce precursors of angiogenesis inhibitors.

• Collagen XVIII and plasminogen are produced in large quantities by the liver and secreted into hepatic venous effluent where subsequent action by proteolytic enzymes cleaves these precursors into the potent angiogenesis inhibitors endostatin and angiostatin, respectively.

• Exclusion of these substances from the pulmonary arterial circulation after cavopulmonary anastomosis may result in vascular proliferation

Pulmonary Arteriovenous Malformations

• VEGF = vascular endothelial growth factor

Pulmonary Arteriovenous Malformation