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Paeds Topicscardiology

Paeds · cardiology

Ventricular septal defect

Also known as Ventricular septal defect · VSD · Interventricular communication · Perimembranous VSD · Maladie de Roger (small VSD)

Fellowship guide to the commonest congenital heart defect: the anatomic and haemodynamic classification of a ventricular septal defect, the left-to-right shunt physiology, the natural history from spontaneous closure to Eisenmenger syndrome, the murmur and heart-failure presentations, echocardiographic sizing, and the surgical, device and medical management across infancy and adolescence.

high10 referencesUpdated 13 July 202620 min readVerification in progress

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Target exams

RACP DWERACP DCEMRCPCH TheoryMRCPCH Clinical

Red flags

  • A previously well infant who becomes increasingly breathless, sweaty on feeds and fails to thrive at four to eight weeks is in pulmonary overcirculation from a moderate-to-large VSD as pulmonary vascular resistance falls — assess urgently with echocardiography and start heart-failure therapy
  • Cyanosis, clubbing or a right-to-left shunt in an older child or adult with a VSD signals Eisenmenger syndrome — the pulmonary vascular disease is now fixed, and closing the defect is harmful and potentially fatal
  • Aortic regurgitation developing in a child with a perimembranous or outlet VSD means aortic cusp prolapse and is an indication to close the defect, even if the shunt is small
  • Surgical closure carries a risk of complete heart block from injury to the atrioventricular node; a new bradycardia after VSD repair needs urgent assessment

Life stages

fetalneonateinfanttoddlerpreschoolschool-ageadolescentyoung-adult-transition

Care settings

preventive-medical-homeoutpatientwarded-acutenicupicu

Clinical exam formats

written-onlyracp-dce-short-casemrcpch-short-clinicalracp-dce-long-case

Board mappings

  • Ventricular septal defect
  • Ventricular septal defect and left-to-right shunt physiology
  • Ventricular septal defect: murmur recognition, natural history, and management
  • Short case: systolic murmur in an infant (VSD)
  • Long case: the child with a repaired VSD and follow-up
  • Ventricular septal defect: anatomy, haemodynamics, and management
  • Cardiac: systolic murmur in an infant
  • Congenital heart disease: ventricular septal defect
  • Patient Care: congenital heart disease, including ventricular septal defect
  • Medical Expert: ventricular septal defect
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  • Short-answer question1
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Target exams

RACP DWERACP DCEMRCPCH TheoryMRCPCH Clinical

Red flags

  • A previously well infant who becomes increasingly breathless, sweaty on feeds and fails to thrive at four to eight weeks is in pulmonary overcirculation from a moderate-to-large VSD as pulmonary vascular resistance falls — assess urgently with echocardiography and start heart-failure therapy
  • Cyanosis, clubbing or a right-to-left shunt in an older child or adult with a VSD signals Eisenmenger syndrome — the pulmonary vascular disease is now fixed, and closing the defect is harmful and potentially fatal
  • Aortic regurgitation developing in a child with a perimembranous or outlet VSD means aortic cusp prolapse and is an indication to close the defect, even if the shunt is small
  • Surgical closure carries a risk of complete heart block from injury to the atrioventricular node; a new bradycardia after VSD repair needs urgent assessment

Life stages

fetalneonateinfanttoddlerpreschoolschool-ageadolescentyoung-adult-transition

Care settings

preventive-medical-homeoutpatientwarded-acutenicupicu

Clinical exam formats

written-onlyracp-dce-short-casemrcpch-short-clinicalracp-dce-long-case

Board mappings

  • Ventricular septal defect
  • Ventricular septal defect and left-to-right shunt physiology
  • Ventricular septal defect: murmur recognition, natural history, and management
  • Short case: systolic murmur in an infant (VSD)
  • Long case: the child with a repaired VSD and follow-up
  • Ventricular septal defect: anatomy, haemodynamics, and management
  • Cardiac: systolic murmur in an infant
  • Congenital heart disease: ventricular septal defect
  • Patient Care: congenital heart disease, including ventricular septal defect
  • Medical Expert: ventricular septal defect

Overview & Definition

A ventricular septal defect (VSD) is a congenital communication between the two ventricles through the interventricular septum. Because the left ventricle generates higher pressures than the right, blood shunts from left to right across the defect. The result is extra blood flowing into the pulmonary circulation, and the child's whole clinical course is set by how much blood crosses that gap. [1]

The VSD is the commonest congenital heart lesion present at birth, accounting for roughly thirty to forty percent of all structural heart disease. It sits at one end of a spectrum so wide that it runs from a tiny muscular defect that closes itself within weeks to a large non-restrictive defect that floods the lungs, causes infant heart failure, and over years can cause irreversible pulmonary vascular disease. [1] [2]

What is a VSD and why does its size decide everything?

A ventricular septal defect is a hole between the two ventricles that produces a left-to-right shunt. Its anatomy (where it sits on the septum) and its size (how much it restricts the shunt) together determine everything that follows: a small restrictive VSD is often silent and closes itself, a moderate VSD dilates the left heart and causes symptoms, and a large non-restrictive VSD risks fixed pulmonary hypertension (Eisenmenger syndrome) if not closed in time. [1] [9]

The heart-failure window at four to eight weeks

A moderate-to-large VSD is often silent at birth because the neonatal pulmonary vascular resistance is still high. As that resistance falls over the first six to eight weeks, the left-to-right shunt increases and the infant develops tachypnoea, poor feeding, sweating with feeds and failure to thrive. Any infant presenting in this window with these signs needs urgent echocardiography and heart-failure therapy. [1]

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References10ShowHide
  1. [1]Hoffman JI; Kaplan S The incidence of congenital heart disease. J Am Coll Cardiol, 2002.PMID 12084585
  2. [2]van der Linde D; Konings EE; Slager MA; Witsenburg M; Helbing WA; Takkenberg JJ; Roos-Hesselink JW Birth prevalence of congenital heart disease worldwide: a systematic review and meta-analysis. J Am Coll Cardiol, 2011.PMID 22078432
  3. [3]Du ZD; Roguin N; Wu XJ Spontaneous closure of muscular ventricular septal defect identified by echocardiography in neonates. Cardiol Young, 1998.PMID 9855105
  4. [4]Butera G; Carminati M; Chessa M; Piazza L; Abella R; Biondi-Zoccai GG; Negura DG; Magro F; Pantaleo A; Amin Z Transcatheter closure of perimembranous ventricular septal defects: early and long-term results. J Am Coll Cardiol, 2007.PMID 17868812
  5. [5]Carminati M; Butera G; Chessa M; Drago M; Negura D; Piazza L Transcatheter closure of congenital ventricular septal defect with Amplatzer septal occluders. Am J Cardiol, 2005.PMID 16399093
  6. [6]Galiè N; Beghetti M; Gatzoulis MA; Granton J; Berger RM; Lauer A; Chiossi E; Landzberg M; Bosentan Randomized Trial of Endothelin Antagonist Therapy-5 (BREATHE-5) Investigators Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Circulation, 2006.PMID 16801459
  7. [7]Gatzoulis MA; Beghetti M; Galiè N; Granton J; Berger RM; Lauer A; Chiossi E; Landzberg M 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
  8. [8]Wilson WR; Gewitz M; Lockhart PB; Bolger AF; DeSimone DC; Koenig MJ; Sadhu Lohr J; Miro JM; Padera RF; Prendergast BD; Sexton DJ; Spelman DW; Tleyjeh IM; Van der Meer JTM; Westermann CJL; Whited W; Wojda TR; American Heart Association Prevention of Viridans Group Streptococcal Infective Endocarditis: A Scientific Statement From the American Heart Association. Circulation, 2021.PMID 33853363
  9. [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
  10. [10]Meberg A; Hals J; Thaulow E Congenital heart defects--chromosomal anomalies, syndromes and extracardiac malformations. Acta Paediatr, 2007.PMID 17590185

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