Paeds Vivas · cardiology
Truncus arteriosus and single-ventricle physiology — branching viva
Branching viva from the four-week-old cyanotic infant who has developed heart failure with bounding pulses and a single second sound, through the truncus bedside diagnosis, the falling-pulmonary-resistance mechanism, the complete neonatal repair and the 22q11.2 testing; then on to the staged Fontan pathway, the interstage infant, and the long-term Fontan survivor whose lifelong surveillance is set by the passive circulation.
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Branch one — the cyanotic infant in heart failure
The examiner opens with a four-week-old who was mildly dusky from birth and now has tachypnoea, sweating on feeds, bounding pulses, a wide pulse pressure and a single second heart sound. Take the candidate through the bedside reasoning to truncus arteriosus, the falling-pulmonary-vascular-resistance mechanism that turns cyanosis into heart failure, and the complete neonatal repair. Probe why every such infant needs 22q11.2 testing. [3] [5]
References5ShowHide
- [1]Naimo PS; Konstantinov IE Surgery for Truncus Arteriosus: Contemporary Practice. Ann Thorac Surg, 2021.PMID 32828754
- [3]Thompson LD; McElhinney DB; Reddy M; et al Neonatal repair of truncus arteriosus: continuing improvement in outcomes. Ann Thorac Surg, 2001.PMID 11515872
- [5]Goldmuntz E 22q11.2 deletion syndrome and congenital heart disease. Am J Med Genet C Semin Med Genet, 2020.PMID 32049433
- [10]Rychik J; Atz AM; Celermajer DS; et al Evaluation and Management of the Child and Adult With Fontan Circulation: A Scientific Statement From the American Heart Association. Circulation, 2019.PMID 31256636
- [12]Rudd NA; Ghanayem NS; Hill GD; et al Interstage Home Monitoring for Infants With Single Ventricle Heart Disease: Education and Management: A Scientific Statement From the American Heart Association. J Am Heart Assoc, 2020.PMID 32777961