II. Epidemiology

  1. Incidence: 15-25% of Congenital Heart Disease
    1. Most common Congenital Heart Disease cause
    2. Most common CHD found in chromosomal abnormalities
  2. Gender
    1. More common in males

III. Pathophysiology

  1. Defect in interventricular septal wall
    1. Most often located in membranous ventricular septum
  2. Physiology of VSD changes with time after birth
    1. Newborn initially has a right to left shunt due to increased pulmonary pressures
    2. By 6 months of age, pulmonary pressures decrease, and shunt is left to right
    3. Longterm large VSD leads to Eisenmenger Syndrome with right to left shunt
      1. Results from chronic pulmonary overload with Pulmonary Hypertension

IV. Signs and symptoms

  1. Severity of symptoms related to:
    1. Defect size
    2. Pulmonary vascular resistance
    3. Associated cardiac lesions
  2. Small to moderate VSD
    1. Normal P2 component of the Second Heart Sound
    2. Pansystolic harsh murmur
      1. Grade II-VI of VI
      2. Located at lower left sternal border
  3. Large VSD with significant shunt
    1. Includes moderate VSD findings
    2. Mid-diastolic flow rumble at apex
    3. Congestive Heart Failure signs and symptoms in a pink child (not cyanotic)
  4. Marked Pulmonary Hypertension
    1. Right Ventricular lift
    2. Loud P2 component of the Second Heart Sound
    3. Short systolic ejection murmur at left sternal border

V. Imaging (large defect findings)

  1. Chest XRay
    1. Cardiomegaly
    2. Increased pulmonary vasculature
    3. Lungs appear dark in Pulmonary Hypertension (contrast with white of Pulmonary Edema)
  2. Echocardiogram
    1. Defines position and size of defect

VI. Management: Medical

  1. Small Ventricular Septal Defect
    1. No surgical repair indicated
    2. SBE Prophylaxis
  2. Congestive Heart Failure
    1. See Pediatric Congestive Heart Failure
    2. Supplemental Oxygen
      1. May worsen Pulmonary Hypertension (increasing left to right shunt)
    3. Positive Pressure Ventilation (BiPAP)
    4. Diuretics (e.g. Furosemide 1 mg/kg IV)
    5. Consider Nitroglycerin in flash Pulmonary Edema (Cardiogenic Shock)
      1. However, may worsen left to right shunt
    6. Consider inotropes (Dobutamine or Milrinone)
      1. Indicated in Cardiogenic Shock with Pulmonary Edema (cold and wet)
      2. Consider Epinephrine if hypotensive
    7. Consider Digoxin

VII. Management: Surgical repair indications

  1. Growth failure refractory to medical therapy
  2. Pulmonary Hypertension
  3. Pulmonary to systemic flow ratio > 2:1

VIII. Course

  1. Spontaneous closure age <6 months: 30-40% related to:
    1. Membranous and muscular defects
    2. Smaller defects

IX. Complications: Without repair

X. Complications: With repair

  1. Conduction defect: transient Right Bundle Branch Block

XI. References

  1. Claudius and Strobel (2024) EM:Rap, 9/9/2024
  2. Cyran (1998) PREP review lecture, October, Phoenix
  3. Merenstein (1994) Pediatrics, Lange
  4. Saenz (1999) Am Fam Physician 59(7):1857-66 [PubMed]

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Related Studies

Ontology: Ventricular Septal Defects (C0018818)

Definition (MSHCZE) Vývojová porucha v jakékoli části MEZIKOMOROVÉ PŘEPÁŽKY má za následek nesprávné propojení mezi dvěma dolními srdečními komorami. Klasifikace ventrikulárních septálních defektů je založena na lokalizaci tohoto vadného propojení – jako např. vada srdeční membrány, vada na vtoku či výtoku do/ze srdce, vada v oblasti centrální nebo boční svalové stěny nebo hrotový svalový defekt.
Definition (NCI) The presence of a defect (opening) in the septum that separates the two ventricles of the heart. It can be congenital or acquired.
Definition (NCI_CDISC) The presence of a defect (opening) in the septum that separates the two ventricles of the heart. The VSD can be congenital or acquired.
Definition (MSH) Developmental abnormalities in any portion of the VENTRICULAR SEPTUM resulting in abnormal communications between the two lower chambers of the heart. Classification of ventricular septal defects is based on location of the communication, such as perimembranous, inlet, outlet (infundibular), central muscular, marginal muscular, or apical muscular defect.
Concepts Anatomical Abnormality (T190)
MSH D006345
ICD9 745.4
ICD10 Q21.0
SnomedCT 253549006, 30288003, 270547008, 156914003, 204314001, 204310005, 204309000
English Heart Septal Defects, Ventricular, Defect, Ventricular Septal, Septal Defect, Ventricular, Septal Defects, Ventricular, VENTRICULAR SEPTAL DEFECT, VSD - Ventricular sept defect, Ventricular septal defect unsp, Ventricular septal defect, unspecified, Defect, Intraventricular Septal, Defects, Intraventricular Septal, Septal Defect, Intraventricular, Intraventricular Septal Defects, Septal Defects, Intraventricular, Ventricular Septal Defect, Intraventricular Septal Defect, ventricular septal defect, ventricular septal defect (diagnosis), VSD (ventricular septal defect), Ventricular sept defect, Heart Septal Defects, Ventricular [Disease/Finding], Defect;ventricular septal, ventricular septal defects, Ventricular Septal Defect (VSD), vsd, interventricular septal defect, Ventricular septal abnormality (disorder), Ventricular septal abnormality, Ventricular septal defect (VSD), Ventriculoseptal defect, Ventricular septal defects, Ventricular septal def., Ventricular septal defect, unspecified (disorder), Septal def.-ventricular, Ventricular septal defect NOS (disorder), Interventricular septal defect (disorder), ventricular septal abnormality (diagnosis), Ventricular septal defect, Interventricular septal defect, VSD - Ventricular septal defect, Ventricular septal defect (disorder), closure; incomplete, septum, interventricular, closure; incomplete, septum, ventricular, defect; interventricular septal, defect; septum, ventricular, defect; ventricular septal, heart; anomaly, septum, ventricular, incomplete; closure, septum, interventricular, incomplete; closure, septum, ventricular, interventricular septal; defect, septum; defect, ventricular, anomaly; heart, septum, ventricular, ventricular; septal defect, Ventricular Septal Defects, VSD, Ventricular septal defect NOS, Congenital ventricular septal defect, Defect interventricular septum, Septum interventricular patent
French COMMUNICATION INTERVENTRICULAIRE, Cloison iinterventriculaire perméable, Communication interventriculaire SAI, CV, Anomalie congénitale du septum interventriculaire, CIV (Communication InterVentriculaire), Communication interventriculaire, Anomalies du septum interventriculaire, Communications inter-ventriculaires, Communications interventriculaires
Dutch ventriculair septumdefect, opening in het interventriculaire tussenschot, ventrikelklepdefect, congenitaal, ventriculair septumdefect NAO, VSD, open interventriculair septum, anomalie; hart, septum, ventriculair, defect; interventriculair septum, defect; septum, ventriculair, defect; ventrikelseptum, hart; anomalie, septum, ventriculair, interventriculair septum; defect, onvolledig; sluiting, septum, interventriculare, onvolledig; sluiting, septum, ventriculair, septum; defect, ventriculair, sluiting; onvolledig, septum, interventriculare, sluiting; onvolledig, septum, ventriculair, ventrikel; septumdefect, Defect, ventrikelseptum-, Defecten, ventrikelseptum-, Hartseptumdefect, ventriculair, Ventriculair hartseptumdefect, Ventrikelseptumdefect, Ventrikelseptumdefecten, septumdefect, ventrikel-, septumdefecten, ventrikel-
Italian Difetto del setto interventricolare, Difetto congenito del setto interventricolare, Setto interventricolare pervio, Difetto del setto intraventricolare, Difetto del setto interventricolare NAS, Difetti del setto interventricolare, Difetto del setto ventricolare, Difetti del setto ventricolare, Difetti del setto cardiaco ventricolare
Portuguese Defeito do septo interventricular, Defeito do septo ventricular, Defeito congénito do septo interventricular, Defeito do septo interventricular NE, COMUNICACAO INTER-VENTRICULAR, Comunicação Interventricular, Malformações Congênitas do Septo Interventricular, Malformação do Septo Interventricular, Defeitos do Septo Interventricular, Malformações do Septo Interventricular, Defeitos do Septo Ventricular
Spanish Defecto del tabique interventricular, Defecto congénito del tabique ventricular, Tabique interventricular persistente, Defecto septal ventricular NEOM, Defectos del Tabique Interventricular, Defectos del Tabique Ventricular, SEPTO VENTRICULAR, DEFECTO, defecto del tabique interventricular, defecto del tabique interventricular (trastorno), defecto septal interventricular (trastorno), defecto septal interventricular, anomalía del tabique interventricular, anomalía del tabique ventricular, anomalía del tabique interventricular (trastorno), anomalía del tabique ventricular (trastorno), Ventricular septal abnormality, defecto del tabique ventricular, no especificado, defecto del tabique ventricular, SAI (trastorno), defecto del tabique ventricular, no especificado (trastorno), defecto del tabique ventricular, SAI, Malformación del Tabique Interventricular, Defectos del Septo Intraventricular, Defectos Septales Ventriculares, Malformaciones Congénitas del Tabique Interventricular, Malformaciones del Tabique Interventricular, Defectos del Septum Interventricular, CIV, defecto del tabique ventricular
German Septum interventrikulaer offen, kongenitaler Ventrikelseptumdefekt, interventrikulaerer Septumdefekt, Ventrikelseptumdefekt NNB, VSD, VENTRIKELSEPTUMDEFEKT, Herzkammerseptumdefekte, Kammerseptumdefekte, Ventrikelseptumdefekte, Septumdefekte, Ventrikel-, Herzseptumdefekte, ventrikuläre, Ventrikelseptumdefekt
Japanese 心室中隔欠損症NOS, 先天性心室中隔欠損症, 心室中隔開存症, センテンセイシンシツチュウカクケッソンショウ, シンシツチュウカクケッソンショウ, シンシツチュウカクカイゾンショウ, シンシツチュウカクケッソンショウNOS, VSD, VSD, 心室中隔欠損, 中隔欠損-心室, 心室中隔欠損症
Swedish Ventrikelseptumdefekter
Finnish Kammioväliseinän aukot
Russian MEZHZHELUDOCHKOVOI PEREGORODKI DEFEKTY, МЕЖЖЕЛУДОЧКОВОЙ ПЕРЕГОРОДКИ ДЕФЕКТЫ
Czech Defekt komorového septa, VSD, Otevřené mezikomorové septum, Defekt komorového septa NOS, Vrozený defekt komorového septa, Defekt mezikomorového septa, defekty septa komor, srdce - defekty komorového septa, srdce - defekty septa komor, defekty komorového septa
Korean 심실 중격 결손증
Croatian SRCE, DEFEKTI VENTRIKULARNOG SEPTUMA
Polish Ubytek przegrody międzykomorowej, VSD
Hungarian Ventricularis septum defectus k.m.n., Veleszületett kamrai septum defectus, Ventricularis septum defectus, Interventricularis septum-defectus, Septum interventricularis nyitott
Norwegian Ventrikkelseptumdefekter