Paeds SAQs · cardiology
Hypoplastic left heart syndrome — formative SAQs
Two formative SAQs on hypoplastic left heart syndrome: the neonate who collapses on day three of life with uniformly weak pulses and metabolic acidosis, testing the prostaglandin-E1-first rule and the ductal-dependent mechanism; and the interstage infant between the Norwood and the Glenn, testing the interstage mortality window, the home-monitoring strategy and the response to deterioration.
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SAQ 1 — The day-three collapse (10 marks, 15 minutes)
A three-day-old term infant who was feeding well presents to the emergency department in shock. The brachial and femoral pulses are both weak, the skin is grey and mottled, and the blood gas shows a pH of 7.06 with a lactate of 10 mmol per litre. The heart sounds reveal a single second sound. The working diagnosis is hypoplastic left heart syndrome. [2]
a) State the immediate pharmacological management and the principle that governs its timing. (4 marks) [2]
b) Explain the pathophysiological mechanism that links the timing of the collapse to the postnatal age. (3 marks) [3]
c) Describe the bedside and auscultatory findings that distinguish this lesion from coarctation of the aorta. (3 marks) [2]
References5ShowHide
- [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
- [3]Rychik J Hypoplastic left heart syndrome: can we change the rules of the game? Circulation, 2014.PMID 25052402
- [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
- [6]Rudd NA; Ghanayem NS; Hill GD; et al Interstage Home Monitoring for Infants With Single Ventricle Heart Disease: Education and Management. J Am Heart Assoc, 2020.PMID 32777961