VSD PA MAPCAS presenting late with hypoplastic pulmonary vasculature. Reply to letter to Editor: “Lesson learnt about right ventricle to pulmonary artery shunt for older children and young adults with ventricular septal defect, pulmonary atresia, and hypoplastic pulmonary arteries”

Dear Sir,

I thank Drs Patel and Gajjar for their observations [1] on our review article entitled ‘Ventricular septal defect with pulmonary atresia: approaches, results, prognosticators and current status’ [2] published in the journal earlier this year.

In their letter entitled ‘Lesson learnt about right ventricle to pulmonary artery shunt for older children and young adults with ventricular septal defect, pulmonary atresia, and hypoplastic pulmonary arteries’ [1], they have highlighted the experience of their group with the palliative non-valved right ventricle (RV) to pulmonary artery (PA) graft, with or without unifocalization of major aorto-pulmonary collateral arteries (MAPCAs), but without ventricular septal defect (VSD) closure in late presenters with small central PAs and have described the salutary effects on growth of the pulmonary arterial tree that they have documented on follow-up.

In our article, we too have described this approach, shown diagrammatically in Fig. 3 in the manuscript [2].

Our own approach is in agreement with Dr Patel’s. We too prefer the RV-PA direct connection in patients with VSD-pulmonary atresia where the central pulmonary vasculature is small and inadequate to handle complete septation in one stage, in preference to the systemic to PA shunt, which is prone to problems with low diastolic pressure, pulmonary overflow, and nonuniform PA growth. Our preference is for a palliative transannular patch, when possible, since the tube graft is more prone to stenosis, and has gone to complete repair in several patients despite the first stage being done in late presenters.

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