Paeds Vivas · cardiology
Hypoplastic left heart syndrome — branching viva
Branching viva from the neonate who collapses on day three of life with uniformly weak pulses, through the prostaglandin-E1-first resuscitation rule, the ductal-dependent mechanism, the echocardiographic confirmation and the staged palliation, to the interstage infant and the Fontan survivor whose lifelong surveillance is set by the single-ventricle circulation.
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Branch one — the neonatal collapse
The examiner opens with a three-day-old term infant in shock. Take the candidate through the immediate assessment, the prostaglandin-E1-first rule, the bedside distinction from coarctation, and the echocardiographic anatomy of atretic mitral and aortic valves with a hypoplastic ascending aorta. Probe what happens if the atrial septum is restrictive. [2]
References4ShowHide
- [1]Ohye RG; Sleeper LA; Mahony L; et al Comparison of shunt types in the Norwood procedure for single-ventricle lesions. N Engl J Med, 2010.PMID 20505177
- [2]Feinstein JA; Benson DW; Dubin AM; et al Hypoplastic left heart syndrome: current considerations and expectations. J Am Coll Cardiol, 2012.PMID 22192720
- [4]Ghanayem NS; Allen KR; Tabbutt S; et al Interstage mortality after the Norwood procedure: Results of the multicenter Single Ventricle Reconstruction trial. J Thorac Cardiovasc Surg, 2012.PMID 22795436
- [9]Goldberg CS; Trachtenberg FL; Krawczeski PA; et al Longitudinal Follow-Up of Children With HLHS and Association Between Norwood Shunt Type and Outcomes. Circulation, 2023.PMID 37795623