Pulmonary atresia with intact ventricular septum

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Definition: 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) 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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