Initial results and medium term follow up of PAIVS patients with moderate right ventricular hypoplasia following radiofrequency valvotomy and PDA stenting

  • Dr Kok Kuan Choo, National Heart Institute, Malaysia
  • Dr Mazeni Alwi, National Heart Institute, Malaysia
  • Dr Hasri Samion, National Heart Institute, Malaysia
  • Dr Haifa Abdul Latiff, National Heart Institute, Malaysia
  • Dr Geetha Kandavello, National Heart Institute, Malaysia

    • Objectives: To assess the initial results and medium-term outcome of pulmonary atresia-intact ventricular septum (PAIVS) patients with moderate right ventricular (RV) hypoplasia after radiofrequency valvotomy (RFV) with concomitant PDA stenting.

    Methods: Between January 2001 and June 2008, 33 PAIVS patients with moderate RV hypoplasia underwent RFV and concomitant PDA stenting. RV size was assessed by two-dimensional echocardiography and RV angiography. The finding of ‘moderate RV hypoplasia’ and decision for concomitant PDA stenting was determined by 2 independent observers.

    Results: RFV and concomitant PDA stenting were performed successfully in 33 patients. At time of procedure, the median age was 10 days, median weight 3.1kg, mean z-score of tricuspid annulus -3.9+/-2.5, mean tricuspid/mitral ratio 0.6+/-0.18. There was no procedure-related mortality. There were 2 early deaths from low cardiac output syndrome and 1 late death. Mean duration of follow up was 4.3 years. Of the 30 survivors, 9 (30%) were on follow up less than one year. 9 (30%) had 1˝ ventricular circulation and received bidirectional cavopulmonary shunt.12 (40%) had complete biventricular circulation, with 6 requiring no further reintervention whereas 2 required ASD closure, 2 RV outflow tract reconstruction, one PDA restenting and one balloon dilatation of RVOT. The freedom from reintervention was 70%, 50% and 40% at 1 year, 2 years and 3 years respectively.

    Conclusions: Primary treatment with RFV and concomitant PDA stenting for PAIVS with moderate RV hypoplasia provides good initial palliation that obviates the need for early surgical intervention. However, a significant proportion eventually required 1˝ ventricular repair.