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the worst prognosis in congenital heart disease

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A question from last year's examination in neonatology ( MCQ ) : " Which congenital heart anomaly causes the highest mortality in first seven days of life?" any ideas? articles? hints where to find the correct answer? :confused:

What was the MCQ alternatives?

My guess is some kind of severe outflow malformation (those can present with dramatic clinical pictures, such as critical aortic or pulmonary stenosis/atresia) or hypoplastic left heart syndrome (although those latter infants can survive for some time, according to my experience).

I found this UK site (without Google, I'd know less...). http://www.heartstats.org/datapage.asp?id=3390

The spreadsheet below gives some hints, although it presents infant mortality rates.

http://www.heartstats.org/documents/download.asp?nodeid=3517&libraryversionid=818

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I would have responded hypoplastic left heart syndrome I guess

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According to the pediatric cardiology for practitioner, 4th edition

HLHS occurs in 1% of all congenital heart defects or 9% of such defects in newborns. and it is the most common cause of death from cardiac defects during the first month of life.

But D-TGA is the most common congenital cyanotic heart disease in newborns and the nost common cause of neonatal HF during the first week of life. Most of these cases are surgically corrected. even Without surgical correction death occurs in 90% of patients before they reach 6 months of age.

So i think the correct answer is HLHS

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Thank you very much :) Unfortunately I do not know the alternative answers. The question comes from my colleague who passed the exam several months ago - and could not remember them. This UK site is very helpful and I suppose the decision should be HLHS. What would I do without my NICU friends, chocolate and caffeine? ;)

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