Pulmonary atresia with intact ventricular septum
Definition:Â atretic pulmonary valve leads to a hypertrophic and hypoplastic RV cavity. Pulmonary blood flow depends on PDA. Incidence 3:10.000
Physiology:
Decreased pulmonary blood flow (tet-spells) due to RVOT obstruction and increased PVR or/and congestive cardiac failure.
Diagnosis:
ECHO, angiography for coronary anatomy and to rule out ventriculo-coronary fistula.
Management preoperatively:
- PGE1 infusion (20ng/kg/min) to maintain PDA patency
- For persistent hypoperfusion with restrictive intra-atrial communication: ballon atrial septostomy
- Aim for SpO2 75 – 85%
Preoperative preparation:
ECG, CXR, CUS, FBE, clotting, UECs, PRBC (4), FFP (2), platelets (2), cryoprecipitate (2).
Methylprednisolone 10mg/kg 12hrs and 6hrs pre surgery.
Surgery:
Dependent on the anatomical associations, degree of right ventricular hypoplasia and development of pulmonary arteries (Z-score of tricuspid valve):
- In selected patients: balloon valvotomy (would require ongoing PGE1 infusion as the RV needs time for remodelling)
- Univentricular approach (Z-score <-4): systemic-pulmonary shunt (BT shunt, Glenn shunt, Fontan circulation)
- Biventricular repair (Z-score >-2)Â TOF
- Partial biventricular repair (1.5 ventricle repair) (Glenn Shunt)
Postoperative management:
As per BT shunt, Glenn shunt or Fontan circulation protocol
Specific problems:
- High inotropic support and/or severe cyanosis may be indicators for inappropriate RV size and function
- To prevent shunt thrombosis commence heparin 10U/kg/hr once no major bleeding
Outcome:
Long term survival: 86%
References
[1] Critical Heart Disease in Infants and Children; 2nd ed, Nichols et al: Pulmonary Atresia with intact ventricular septum
[2] Cardiol Young. 2005 Oct;15(5):447-68: Freedom et al: The significance of ventriculo-coronary arterial connections in the setting of pulmonary atresia with an intact ventricular septum
[3] Ann Thorac Surg. 2013 Feb 28. Mainwaring et al: Hemodynamic Assessment After Complete Repair of Pulmonary Atresia With Major Aortopulmonary Collaterals.
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