At our Institution we have 2 pts with isolated right ventricluar hypoplasia; both of them in haemodynamic balance and with sat 02 in the 90's.

If his saturation lowers with increasing age I think the best approach should be of

cavopulmonary anastomosis plus ASD surgical repair.

The problems might arise at older age when superior vena cava contribuition to cardiac output become lower but at this stage the closure of the ASD shoud have forced the RV to an appropriate preload

Dear Dr. Troise, my patient had a cavopulmonary anastomosis at one year of age, and since then he was fine. But now his SatO2 is becoming lower at rest, and as I have no experience with the disease, I am a little affraid of closing de ASD. Do you think right ventricle can grow up if I raise the preload by closing the ASD? I tried to find something in literature, but found only some cases and most of them with a bad prognosis. Thank you for your answer.

Sorry I didn't understaND VERY WELL THE HISTORY OF YOUR PATIENT; try to manage your pt as he would be a pulmonary atresia and intact ventrcicluar septum s/p perforation and BTshunt procedure with the ASD temporary occlusion and wait and see pressure in RA RV PA in order to analyze if RV promote a pressure step-up between the chamber above and below it. Please let me know

Yes, I send the patient to our scientific meeting and we decided to do exactly this. I thank you for your answer.

The ASD may be closed surgically with a fenestrated patch leaving a pop-off mechanism for RA to decompress in the event of sudden increase in RA pressure.

An alternative to surgical closure is implanting a fenestrated ASD occluder (techniques of fenestrating ASD devices are described in the literature and are fairly simple), after you confirmed that closure can be safely undertaken following temporary balloon occlusion). In my experience a saturation of 90 % in this situation is not too bad and is probably suggestive of potential "closability".

Yes, these too options may be used, but after a discussion with our team of surgery and hemodynamics, the our first option will be trying a fenestrating ASD ocluder. But Dr. Daniel, have you ever tried this on a patient like mine? There are not too many cases of isolated right ventricle hypoplasia in literature...

I personally saw 3 cases: one ended without any intervention necessary at all, one had ASD closure and one ended with 1 1/2 ventricle repair and closure of the ASD. This leaves all the options open. Personally, my trigger to add a bidirectional cavopulmonary anastomosis if pressures rise during the balloon test occlusion, would be rather low.

Though implanting a fenestrated ASD occluder is an alternative, i am not sure how long the fenestration persists. We have seen cases where we deployed a fenestrated ASD device (device patch fenestrated with a 14Fr dilator) in cases with high PA pressures or elderly patients with high LV diastolic pressures; but could not visualise residual flow on follow up echo's. Whereas surgical ASD fenestrations (in our experience) are almost always reliably seen (provided they are good sized and not just tiny). I am not sure what is everyone else's experience?

Indeed, the fate of the fenestration in the ASD occluder on the long term is uncertain, but the fenestration should only be used to allow the EV to adapt to the new hemodynamics. If the fenestration would be mandatory during longer term follow-up, a bidirectional cavopulmonary anastomosis is mandatory to achive the best hemodynamic result. That is the reason why I mentioned that my trigger for the combination of ASD closure and bidirectional cavopulmonary anastomosis in this particular disease is rather low.

This particular patient had a cavopulmonary anastomosis at 1 year of age, but now he is 9yo and has a large right to left shunt specially during efforts, he has no complains, but in an ergoespirometric test satO2 fell to 87% by the end of exam by the 8th minute. My big doubt is ... shoul I close or not ASD? ASD occluder with fenestration is a possibility, but I have no experience with it or with this disease in particular. Will this small right ventricle support inferior vena cava >> pulmonary artery flow?

87% at 8 minutes of exercise is not too bad actually. I would favour test occlusion with a balloon and if OK, close the ASD. If in doubt, use a fenestrated device. By the way, recent article of new fenestration method with ASD occluder and coronary stent.

Can you send me the reference please?

Cardiology in the YoungCardiology in the Young / Volume 23 / Issue 05 / October 2013, pp 692-696Copyright © Cambridge University Press 2012

DOI: http://dx.doi.org/10.1017/S1047951112001771 (About DOI), Published online: 23 October 2012New Content AlertsJournal Widget

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$5.99 / £3.99 / €4.49 Request Permissions Previous AbstractNext AbstractOriginal ArticlesAtrial septal defect closure with an Amplatzer septal occluder fenestrated with a coronary stent in a child with pulmonary arterial hypertensionArticle author queryskinner gj [PubMed] [Google Scholar]tulloh rm [PubMed] [Google Scholar]tometzki aj [PubMed] [Google Scholar]schulze-neick i [PubMed] [Google Scholar]morgan gj [PubMed] [Google Scholar]

Gregory J. Skinnera1, Robert M. Tulloha1, Andrew J. Tometzkia1, Ingram Schulze-Neicka2 and Gareth J. Morgana1 c1

a1 Department of Paediatric Cardiology, Bristol Children's Hospital, Bristol, London, United Kingdom

a2 Department of Paediatric Cardiology, Great Ormond Street Hospital, London, United Kingdom

Abstract

The presence of an atrial septal defect in pulmonary hypertension has benefits and detractions. Even in idiopathic pulmonary arterial hypertension, a significant left-to-right shunt at atrial level may increase the pulmonary arterial pressure and exacerbate the disease. However, it is well recognised that the presence of an atrial communication may be protective in subgroups with severe disease, allowing maintenance of cardiac output during times of increased pulmonary resistance. In the present paper, we present the case of a young boy with significant idiopathic pulmonary arterial hypertension and an atrial septal defect. We report our technique of septal occlusion using a device to decrease left-to-right shunting with concomitant stent insertion in that device to maintain the potential for right-to-left shunting during times of high pulmonary arterial pressure.

Thank you!