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Pulmonary atresia with intact ventricular septum: surgical management based on right ventricular infundibulum
A Pawade1, A Capuani, D J Penny
1Victorian Paediatric Cardiac Surgical Unit, Royal Children's Hospital, Melbourne, Australia.
Insights
Management of pulmonary atresia with intact ventricular septum (PA.IVS) in infants is controversial. Echocardiography-based management focusing on right ventricular infundibulum development improves outcomes for PA.IVS patients.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Cardiac Surgery
Background:
- Pulmonary atresia with intact ventricular septum (PA.IVS) presents complex management challenges.
- Previous surgical approaches based on right ventricular (RV) geometry yielded suboptimal results.
- Optimal surgical strategies for PA.IVS remain a subject of debate.
Purpose of the Study:
- To evaluate an echocardiography-based management strategy for infants with PA.IVS.
- To correlate RV infundibulum development with surgical outcomes and long-term survival.
- To refine treatment protocols for PA.IVS based on anatomical assessment.
Main Methods:
- Retrospective analysis of 48 neonates with PA.IVS managed between 1980 and 1992.
- Echocardiographic assessment of RV infundibulum development guided initial palliation and subsequent repair strategies.
- Surgical interventions included pulmonary valvotomy, PTFE shunts, RV muscle excision, biventricular repair, and Fontan procedures.
Main Results:
- Patients with a well-formed infundibulum (n=31) had a 60% probability of biventricular repair by 40 months, with 93% survival at 8 months.
- Patients without a well-formed infundibulum (n=17) were managed with a Fontan procedure in mind, showing a 75% survival at 40 months.
- Overall survival probability at 104 months was 77%.
Conclusions:
- Echocardiographic assessment of RV infundibulum development is crucial for tailoring PA.IVS management.
- This strategy improves the likelihood of biventricular repair and long-term survival in select PA.IVS patients.
- Tailored surgical approaches based on RV anatomy enhance outcomes for complex congenital heart defects like PA.IVS.
Abstract:
The optimal management of infants with pulmonary atresia with intact ventricular septum (PA.IVS) remains a controversy. Attempts have been made to base the surgical approach on various geometrical or morphological characteristics of the right ventricle (RV). However, the overall results remain poor when compared to other complex congenital heart defects. Forty-eight neonates with PA.IVS were admitted to our unit between 1980 and 1992. The management plan has evolved to be based entirely on the echocardiographic assessment of the state of development of the infundibulum of the RV. In neonates with a well-formed infundibulum (n = 31), the initial palliation consisted mainly of pulmonary valvotomy (without cardiopulmonary bypass) and PTFE shunt from the left subclavian artery to the main pulmonary artery. There was one death from initial palliation in this subgroup. If necessary, the RV cavity was later enlarged by excision of the hypertrophic muscle of both the trabecular and infundibular portions, before finally attempting biventricular repair. The actuarial probability of achieving a biventricular repair at 40 months of age was 60% (95% CL = 39.5% to 71.3%). Thirteen patients have undergone biventricular repairs with one late death over a total follow-up of 1,720 patient months. In one patient, the RV failed to grow satisfactorily, necessitating a Fontan procedure. Seventeen patients without a well-formed infundibulum were approached with a Fontan procedure in mind. The initial palliation in these patients consisted of a modified Blalock-Taussig shunt only. Ten have undergone a Fontan procedure so far and five are awaiting such repairs. In this group there were four operative deaths: two after initial palliation, and two after Fontan procedures. In patients with a well-developed infundibulum, the actuarial survival probability was 93% (95% CL = 74% to 98%) at 8 months with no further late deaths over 120 months follow-up, whereas in patients without a well-formed infundibulum it was 75% at 40 months (95% CL = 46% to 89%). The overall survival probability at 104 months was 77% (95% CL = 51% to 90%).