Ventricular Septal Defect

What the Nurse Caring for a Patient with CHD Needs to Know

Courtney Petro, BSN, RN, CCRN

Registered Nurse, Cardiovascular ICU,
Lucile Packard Children's Hospital at Stanford

Melanie Sojka, MSN, RN, CPNP-AC/PC

Pediatric Nurse Practitioner, Cardiac & Thoracic Surgery,
University of Chicago Medicine, Comer Children’s Hospital

Grace Macek, MSN, RN, PNP-BC

Pediatric Nurse Practitioner, Cardiac & Thoracic Surgery,
University of Chicago Medicine, Comer Children’s Hospital

Jennifer Newcombe, MSN, CNS, CPNP-AC/PC

Nurse Practitioner, Pediatric Cardiothoracic Surgery,
Loma Linda University Children’s Hospital

Dorothy M Beke, RN, MS, CPNP-PC/AC

CICU Clinical Nurse Specialist and Cardiology Clinic NP,
Boston Children’s Hospital

Embryology
 One of most common congenital heart defects (CHD)
 Intraventricular septum divides right (RV) & left (LV) ventricles
o Consists of 3 separate septa
o Beginning in 5th week embryonic development
o Completely formed and closed by 7th-8th week embryonic development
 Septum results from:
o Growth of muscular portion upward from ventricular floor towards endocardial
cushions
o Growth of subendocardial tissue from right side of endocardial cushion
 Fuses with aorticopulmonary septum
 Fuses with muscular portion
 Causes of ventricular septal defect (VSD)
o Unclear
 Multifactorial
 Genetic/chromosomal syndromes (trisomy 13, 18, 21/ Holt-Orem,
Cornelia de Lang)
o Majority not associated with other defects or syndromes
o More common in premature or low-birthweight infants

Anatomy
 Results when interventricular septum fails to close (See illustration below for locations
and types of VSDs
 o May occur in any part of the septum
o May occur in more than one location

Outlet

Inlet Perimembranous

Muscular

Apical Muscular
Muscular

Illustrations reprinted from PedHeart Resource. www.HeartPassport.com.

© Scientific Software Solutions, 2016. All rights reserved

 Abnormal communication along septum between right & left ventricles

o Disruption in fusion of 3 separate septa
o Size and anatomical location
 May be single or multiple defects
 Varies with 4 major locations
 Perimembranous (Membranous, infracristal, conoventricular
malalignment including tetralogy of Fallot and double outlet
defects) (Number 2 in above illustration)
o Located in upper portion of septum
o Most common - 70-80%
o Frequently close in first year of life – 30-50%
o Conoventricular defects do not spontaneously close
 Outlet (Supracristal, conal, subpulmonary, subarterial) (Number 1
in above illustration)
o Incomplete fusion along aortopulmonary septum with
endocardial cushions & muscular portion
o Located just beneath pulmonary valve
 o May involve prolapse of aortic valve leaflet
 Results to damage in aortic valve
 May result in aortic valve insufficiency
o Spontaneous closure uncommon
 Inlet or Canal VSDs (Number 6 in above illustration) ( Number 6
in above illustration)
o Lie beneath septal leaflet of TV
o May be referred to as an atrioventricular septal defect, but
does not involve either atrioventricular valve
o Will not spontaneously close
 Muscular or trabecular VSDs (Numbers 3,4, & 5 in above
illustration)
o Less common – 5-20%
o Completely surrounded by muscular tissue
o May appear as single defect on LV side and multiple
defects on RV side due to trabeculations (criss-crossing
fibrous and muscular tissue strands)
o May close spontaneously
o “Swiss cheese” septum
 Multiple defects
 Involve all septal regions
 Associated with other cardiac defects: Atrial Septal Defect (ASD), Patent Ductus
Arteriosus (PDA), Coarctation of the Aorta (CoAo), subvalvar Aortic Stenosis (AS), or
subpulmonic stenosis (PS)
 Multiple VSDs often present with: Tetralogy of Fallot (TOF), Double-Outlet Right
Ventricle (DORV)
 May be acquired in older patients from post-surgical leak, trauma, or myocardial
infarction
Physiology
 Abnormal blood flow across defect in ventricular septum
 Affected by:
o Size of defect
 Primary variable
 Impacts shunt and need for repair
 VSDs <25% of the aortic annulus diameter
o Small
o Minimal, if any, left-to-right shunting
o Potential for spontaneous closure based upon location
 VSDs 25%-75% of the aortic annulus diameter
o Little to moderate left to right shunting
o No pulmonary artery hypertension
o May be mild to moderate pulmonary overcirculation
o May have symptoms of congestive heart failure (CHF)
 Can be managed with medications
  May improve as the patient grows and the defect
starts to close
 VSDs >75% of the aortic annulus diameter
o No restriction to flow
o Moderate to large volume shunt
o Pulmonary overcirculation with CHF symptoms
 Increased pulmonary venous return
 LA and LV dilation
 LV hypertrophy
 Increased RV volume
 Increased pulmonary blood flow
 Increased pulmonary pressures
 May result in pulmonary artery hypertension
(PAH)(See Peds/Neo Problem Guideline on
Pulmonary Hypertension)
 Long standing PAH may result in
Eisenmenger’s Syndrome (See Adult
Problem Guideline on Eisenmenger’s
Syndrome)
o Most likely requires closure
o Resistance to flow
 Pulmonary vascular resistance (PVR)
 Newborn
o Pulmonary pressure > systemic pressures
o Rapidly fall with first few breaths after birth
o Reaches normal adult pressures within first 2 months of age
(Normal mean pressures < 25 mmHg after first few weeks
of life)
o Decrease accelerated by:
 Supplemental oxygen
 Pulmonary vasoactive medications (iNO, sildenafil)
 Decreased resistance increases flow across defect (left-to-right
shunt)
o Normal maturation of pulmonary vascular bed
 Usually occurs by 2 months of age
 RV pressure usually drops to ~ 1/3rd to ½ of LV
pressure by ~ 2 weeks; however in the presence of a
VSD, RV pressure may take longer to decrease.
o Allows for development of pulmonary overcirculation
 Systemic vascular resistance (SVR)
 Newborn
o Systemic pressures = pulmonary pressures
o Left sided lesions increase systemic resistance
 Coarctation of the aorta (CoA), aortic valve (AV)
stenosis
 Vasoconstriction
  Increased left-sided systolic pressure increases flow to RV (left-to-
right shunt)
Procedures/Interventions
 Indications for Intervention:
o Infants with greater than 2:1 shunt
 Large sized VSDs and significant CHF
 Medical treatment of CHF
 Diuretic therapy
 Digoxin/ angiotensin converting enzyme (ACE) inhibitors
 Increase caloric intake
 Goals: control symptoms and allow infant to grow
 Surgical closure
 Early closure (~ less than 3 months of age)
 Unable to manage CHF and provide for somatic growth
o Infants with shunts 1.5:1
 Moderate sized VSD
 May usually be followed for up to 5 years of age to maximize chance of
spontaneous closure
o Shunts less than 1.5:1
o Small VSD
o Require close follow-up
o Outlet VSDs
o May see prolapse of leaflet of AV and develop progressive aortic
regurgitation (AR)
o Repair before significant aortic regurgitation (AR) develops
 Surgical intervention - complete repair via open heart surgery (OHS)
o Patch closure
 Requires cardiopulmonary bypass & sternotomy
 Majority of membranous & inlet VSDs closed through the transatrial
approach
 Repair thru tricuspid valve (TV)
 May have obstructed visualization from valve leaflet
 Requires retraction and sometimes detachment of valve leaflet and
subsequent repair of TV
 Some defects require a right ventriculotomy or pulmonary artery
approach
 Closed with patch material (Dacron or Polytetrafluoroethylene [PTFE]
o Direct suture closure for very small defects
o Risks
 Injury to AV or conduction system
 Residual VSD
 Diminished RV function with ventriculotomy
o Intraoperative transesophageal echocardiography (TEE)
 Assess repair
 Rule out residual VSD
  Assess competence of TV and AV
 Assess ventricular function
 Surgical intervention – palliative
o Pulmonary Artery Band (PAB)
 Requires sternotomy; cardiopulmonary bypass not required
 Controls symptoms related to CHF
 Allows infants to grow and reach appropriate size for surgical repair
o Rarely indicated
 Exceptions: infants < 2.5 kg with multiple/complex defects and/or
intractable CHF
 Combined Surgical/ cardiac catheterization intervention (Hybrid intervention)
o Indications
 Patients too small for percutaneous catheter system
 Part of repair of complex lesion
 Visualization of VSD difficult
o Periventricular closure
 Requires sternotomy but avoids cardiopulmonary bypass
 Placement of percutaneous device directly through the right ventricular
(RV) free wall
 Cardiac catheterization intervention
o Percutaneous closure
o Avoids sternotomy & cardiopulmonary bypass
o Device is placed during cardiac catheterization
o Muscular VSDs may be amenable to device closure

Specific considerations
 Pre-operative considerations
o Age
 Consider size and location of defect
 Premature neonates
 Consider size of defect relative to body size to determine timing of
surgical correction
 Neonates
 Due to elevated PVR development of CHF rare in the first few
weeks of life (See resistance to flow section)
 Do not administer supplemental oxygen unless oxygen saturations
persistently < 75%
 Infants and children
 May be managed medically
 Require surgical correction
o CHF unresponsive to medical management,
o Growth failure
o Outlet and inlet defects
o Health status
 Recent/frequent viral respiratory infections
 Supportive management until cultures negative
  Surgery delayed until patients are symptom free to minimize post-
bypass pulmonary complications
 Failure to thrive
 Consider nasogastric, high caloric feeding
 Attempt to achieve positive caloric balance
 Postoperative (See Peds/Neo Problem Guidelines for Postoperative Management)
o Most common open heart surgery for CHD
o Ventilation
 Patients beyond infancy may be extubated in OR
 Neonates and infants may require ventilator support and aggressive
diuresis before extubation
 Patients with respiratory viral infections preoperatively may require
extended intubation
 Patients with pre-operative respiratory syncytial virus (RSV)
should have negative cultures and be asymptomatic prior to
surgery to minimize post-operative complications
 Patients with pre-operative PAH will require pulmonary vasodilators
(iNO, IV pulmonary vasodilators) (See Peds/Neo Problem Guidelines for
Pulmonary Hypertension)
o Inotropic support
 Majority with minimal inotropic support
 Repair of VSD with complex lesions or PAH
 May also consider pulmonary vasodilators
o Monitor for the following complications:
 Arrhythmias (See Peds/Neo Problem Guidelines for Arrhythmia
Management)
 Most patients who have cardiopulmonary bypass surgery have
temporary epicardial wires placed in OR
 Complete Heart Block
o Typically transient 24-48 hours
o May be permanent and require placement of a permanent
pacemaker, usually after 7-10 days
 Supraventricular Tachycardia (SVT) or Junctional Ectopic
Tachycardia (JET)
 Residual VSD
 Common to have some residual leaks around patch which often
eventually close with endothelialization
 Assessment
o Operating room
 Analysis of RA and PA saturations
 Echocardiography [Transesophageal (TEE) or
Transthoracic (TTE)]
o Intensive care unit
 Desaturation
 Increased pulmonary pressures
 Decreased systemic pressures
  VSD patch dehiscence with low cardiac output
 Pulmonary hypertensive crisis
 Patients with elevated PVR preoperatively or long-standing
pulmonary over-circulation (See Ped/Neo Problem Guidelines for
Pulmonary Hypertension)
o Monitor PA pressures if PA line available
o Follow pulmonary hypertension precautions
 Avoid noxious stimulation
 Fastidious pulmonary toilet (pre-medicate prior to
suctioning)
 Hyperventilation
 Oxygenation
 Strict acid / base control
 Inhaled nitric oxide - potent pulmonary vasodilator
 Sedation/paralysis
 Post-catheter device monitoring:
o Bleeding at puncture site
o Arrhythmias (Complete Heart Block)
 Device may put pressure on the septum close to the left and right bundle
branches
o Valvar regurgitation
 Risk of “trapping” the aortic, tricuspid, or mitral valve leaflets in the
device
o Device embolization

Long-Term Problems/Complications
 Structural Complications
o Residual VSD
o AI secondary to aortic cusp prolapse
o Supravalvar pulmonic stenosis after prior placement of PAB
o Subaortic membrane (rare)
o Right ventricle muscle bundle hypertrophy (rare)
 Arrhythmias ( See Peds/Neo Problem Guidelines for Arrhythmia Management)
o Transient post-operative heart block
 At risk of developing complete heart block
 May require pacemaker placement
o Ventricular arrhythmias
 Monitor with periodic electrocardiograms (ECG), Holter monitor
 Increased with ventriculotomy
 Heart Block requiring pacemaker placement (see Peds/Neo Problems Guidelines for
Pacemakers)
o Pacemaker interrogation every 3-6 months
o Generator changes ~ every 6-10 years
o Lead malfunction or fracture
o Cardiovascular implantable electronic device (CIED) infection
Routine Cardiology Care for Surgical and Catheter Intervention
 Routine follow-up interval
o Every 1-2 years by a cardiologist trained in Congenital Heart Disease (CHD) until
18 years of age
o Adults
 No residual VSD, no associated lesions and normal pulmonary pressure
 Does not require continued follow-up at a regional ACHD center
 Small residual VSD
 Follow up visit every 3 to 5 years at an Adult CHD (ACHD)
regional center
 Device closure of a VSD
 Follow up visit every 1 to 2 years at an ACHD center
 Depends on the location of the VSD
 Cardiac studies as indicated by assessment/symptoms
o Transient post-operative heart block, at least a yearly ECG
o Echocardiogram
 Endocarditis prophylaxis recommendations (AHA, 2015)
o Six months post-surgical repair/device placement
o Longer if a residual defect is present

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Illustrations reprinted from PedHeart Resource. www.HeartPassport.com. © Scientific Software

Solutions, 2016. All rights reserved.

2/2016