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Paeds Vivascardiology

Paeds Vivas · cardiology

Ventricular septal defect — branching viva

Branching viva from the recognition of the VSD murmur through the anatomic and haemodynamic classification, the four-to-eight-week heart-failure physiology, the device-versus-surgery decision, and the Eisenmenger management with family and pregnancy counselling.

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Target exams

RACP General PaediatricsRACP DCEMRCPCH ClinicalRCPSC Pediatrics

Target exams

RACP General PaediatricsRACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
You are the paediatric registrar in clinic. The consultant asks you to talk through four children: a well six-week-old with a loud murmur found at the check, a three-month-old in overcirculation failure, a two-year-old with a perimembranous VSD and new aortic regurgitation, and a sixteen-year-old lost to follow-up who now has Eisenmenger physiology.

Station opening

Examiner: "Define the ventricular septal defect and explain why its clinical course is set by its size." [1]

Strong candidate (must-hit)

  • Defines the VSD as a congenital communication between the ventricles producing a left-to-right shunt because the left ventricle pumps at higher pressure; explains that the clinical course is set by shunt size, which depends on the defect size and the pulmonary vascular resistance, running from a tiny defect that closes to a large defect that floods the lungs, causes heart failure, and over years risks Eisenmenger syndrome. [1]

Weak candidate

  • "A VSD is a hole in the heart that some children are born with." [1]

Branch A — The well six-week-old with a loud murmur

Examiner: "A thriving six-week-old has a loud harsh holosystolic murmur at the lower left sternal border with a thrill, found at the routine check. What is the likely diagnosis, why is the child well, and what do you do next?" [1]

Strong

  • Diagnoses a small restrictive ventricular septal defect (maladie de Roger); explains the paradox that a small defect produces a high-velocity turbulent jet and therefore a loud harsh murmur while the child remains well because the shunt is small; orders echocardiography to confirm and size; outlines surveillance echocardiography every one to two years with reassurance, citing the high spontaneous-closure rate of small muscular defects (Du and Roguin). [1] [3]

Weak

  • "Reassure the parents it is an innocent murmur and discharge." [1]

Branch B — The three-month-old in overcirculation failure

Examiner: "A three-month-old has a pansystolic murmur, an apical mid-diastolic rumble, tachypnoea, sweating with feeds and failure to thrive. Explain the pathophysiology, the significance of the rumble, and your immediate management with doses." [1]

Strong

  • Explains that the moderate-to-large VSD has declared itself as pulmonary vascular resistance fell over the first weeks, producing a left-heart volume load and failure; the apical flow rumble is the sign of a large shunt dilating the left atrium and increased flow across the mitral valve; immediate management is a bridge with a loop diuretic (furosemide 1 to 2 mg/kg/day), an angiotensin-converting-enzyme inhibitor (captopril or enalapril) to reduce afterload and shunt, and increased caloric density of feeds, with oxygen used cautiously because it lowers pulmonary resistance and can increase the shunt; arranges urgent echocardiography and refers for a closure plan within the first year. [1]

Weak

  • "Give high-flow oxygen and a fluid bolus and see if the baby improves." [1]

Branch C — The perimembranous VSD with new aortic regurgitation

Examiner: "A two-year-old with a known perimembranous VSD and a small shunt now has an early diastolic murmur of aortic regurgitation. What has happened, and what is the management?" [4] [9]

Strong

  • Identifies aortic cusp prolapse through the perimembranous defect producing progressive aortic regurgitation; explains that this is an indication to close the defect to protect the aortic valve even though the shunt itself is small; discusses surgical patch closure as the standard for perimembranous defects with aortic involvement, with device closure reserved for selected perimembranous defects with favourable rims (Butera), weighing the small risks of block and aortic regurgitation. [4] [9]

Weak

  • "Leave it; the shunt is small so it does not need closure." [4]

Branch D — The adolescent lost to follow-up (Eisenmenger)

Examiner: "A sixteen-year-old with a large unrepaired VSD, lost to follow-up, presents with exertional cyanosis, clubbing, a soft murmur and haemoptysis. What is the diagnosis, why must the defect not be closed, and what is the management?" [6]

Strong

  • Diagnoses Eisenmenger syndrome: pulmonary vascular resistance has exceeded systemic and the shunt has reversed to right-to-left, softening the murmur; explains that closure is contraindicated because the VSD now acts as a pressure pop-off for the right ventricle and removing it can precipitate fatal right-heart failure; outlines management as pulmonary arterial hypertension with bosentan, citing the BREATHE-5 trial (Galiè) and the open-label extension (Gatzoulis); counsels against pregnancy given the high maternal mortality; ensures air and particulate filters for any intravenous access; and transitions to lifelong adult congenital heart disease care. [6] [7]

Weak

  • "Refer for urgent surgical closure of the defect." [6]

Close

Examiner: "Summarise your approach to the child with a ventricular septal defect in one sentence." [1] [9]

Strong

  • "The VSD is the commonest congenital heart defect, a left-to-right shunt whose course is set by its size: I classify it anatomically and haemodynamically, confirm and size it with echocardiography, manage the small defect with surveillance and the moderate-large symptomatic defect with a medical bridge followed by surgical or device closure in infancy, never close an Eisenmenger defect but treat it with bosentan and lifelong adult congenital care, and counsel the family that the whole strategy of repair is to prevent fixed pulmonary vascular disease." [1] [9]

References

  1. [1]Hoffman JI; Kaplan S The incidence of congenital heart disease. J Am Coll Cardiol, 2002.PMID 12084585
  2. [3]Du ZD; Roguin N; Wu XJ Spontaneous closure of muscular ventricular septal defect identified by echocardiography in neonates. Cardiol Young, 1998.PMID 9855105
  3. [4]Butera G; Carminati M; Chessa M; et al Transcatheter closure of perimembranous ventricular septal defects: early and long-term results. J Am Coll Cardiol, 2007.PMID 17868812
  4. [6]Galiè N; Beghetti M; Gatzoulis MA; Granton J; et al Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Circulation, 2006.PMID 16801459
  5. [7]Gatzoulis MA; Beghetti M; Galiè N; Granton J; et al Longer-term bosentan therapy improves functional capacity in Eisenmenger syndrome: results of the BREATHE-5 open-label extension study. Int J Cardiol, 2008.PMID 17658633
  6. [8]Wilson WR; Gewitz M; Lockhart PB; Bolger AF; et al Prevention of Viridans Group Streptococcal Infective Endocarditis: AHA Scientific Statement. Circulation, 2021.PMID 33853363
  7. [9]Baumgartner H; De Backer J The ESC Clinical Practice Guidelines for the Management of Adult Congenital Heart Disease. Eur Heart J, 2020.PMID 33128054