49 

Ventricular Septal Defects

AS HOK MURALIDARAN, MD; IRVING SHEN, MD

Definition the aortic annulus. There may be a variable amount of muscular

rim at the superior and lateral borders. The defect can extend into
A ventricular septal defect (VSD) is an opening in the interven- the inlet, trabecular, or outlet portions of the interventricular
tricular septum resulting in direct communication between the septum. Extension of the defect to the base of the noncoronary
left and right ventricles. VSDs can be single or multiple. It is the leaflet of the aortic valve may cause aortic regurgitation (AR). The
most commonly diagnosed congenital heart anomaly, present in conal septum may be anteriorly malaligned as in tetralogy of Fallot,
20% of patients with congenital heart disease. causing right ventricular outflow tract obstruction, or, less com-
monly, it may be malaligned posteriorly, causing left ventricular
Embryology outflow tract obstruction.
Inlet defects are also called atrioventricular canal-type defects.
Traditionally the ventricular septum is thought to be derived mostly The posterior margin of the defect runs along the septal leaflet of
from three sources. The primary fold arising from the apex of the the tricuspid valve. The anterior leaflet of the mitral valve often
primitive ventricle, eventually becoming the trabecular septum, has a cleft. The defect extends superiorly to the membranous septum.
fuses with the inlet septum, which originates posteroinferiorly, Muscular defects are located anywhere in the muscular septum.
and with the infundibular or conal septum, which extends downward The margins are characteristically muscular. They are frequently
from the conal ridge. Some evidence, however, points to the inlet multiple. They may be anterior, midmuscular, apical, or in the
and the apical trabecular septa originating from the same source.1 inlet septum. The latter differs from the inlet or atrioventricular
The conal ridge fuses with the endocardial cushions to form the canal-type VSD in that it is separated from the tricuspid valve and
membranous portion of the interventricular septum. Fig. 49.1 membranous septum by muscle tissue. Infundibular or outlet
shows the various components contributing to the formation of muscular VSDs differ from subarterial VSDs because of the presence
the interventricular septum. of a rim of muscle separating the defect from the annuli of the
aortic and pulmonary valves.
Anatomy
Associated Anomalies
There are several schemes for classifying VSDs. The traditional
classification describes four types based on the location of the VSDs can be isolated lesions or part of a variety of major congenital
VSD: type 1 or supracristal/subarterial/conal, type 2 or malformations such as tetralogy of Fallot, double-outlet right
perimembranous/paramembranous, type 3 or inlet/atrioventricular ventricle, transposition of the great vessels, and truncus arteriosus.
canal, and type 4 or muscular2 (Fig. 49.2). A separate classification Fifty percent of patients with VSDs requiring repair have associated
of “malaligned” defects is often employed to describe VSDs that cardiovascular anomalies, most commonly patent ductus arteriosus,
are part of lesions like tetralogy of Fallot or interrupted aortic atrial septal defect (ASD), aortic coarctation, aortic stenosis, and
arch, in which the conal septum and the other components of the pulmonary stenosis.3
septum are fully formed but are displaced anterior or posterior
with respect to each other. Perimembranous defects constitute Pathophysiology and Natural History
80% of the defects with the other types being 5% to 10% each.
Subarterial defects are also known as outlet, conal septal, The dimension of the defect and pulmonary vascular resistance
supracristal, or subpulmonary VSDs. They are located beneath the (PVR) determine the blood flow across a VSD (shunt).4 Smaller
pulmonary valve, and their superior edge is a fibrous ridge between VSDs are said to be restrictive because there is a pressure gradient
the two semilunar valves. They can be associated with prolapse of across the defect with a greater left ventricular pressure than right
the right coronary leaflet of the aortic valve with associated regurgita- ventricular pressure. The left-to-right shunt depends primarily on
tion. This type of defect is more common in the Asian the pressure differences. Larger defects approximate the size of the
population. aortic annulus and are nonrestrictive with equal right and left
Perimembranous (paramembranous) defects are also known as ventricular pressures. In these cases the relative resistances in the
membranous or infracristal defects. They are located between the systemic and pulmonary vasculature determine the left-to-right
anterior and posterior divisions of the septal band and between shunting.
the conal and trabecular interventricular septum. The lateral border PVR is high in the immediate postnatal period and begins to
is formed by the tricuspid annulus; the superior border is usually decrease within the first 2 weeks of life.5 For babies with large

597
 598 PART V Defects

Pulmonary history study from the Belgian registry on adult congenital heart
channel Aortic channel
disease documented a 13% incidence of spontaneous closure of
perimembranous VSDs.13 The authors also noted a 3% incidence
of infective endocarditis, 3% incidence of progression to moderate
to severe aortic valve insufficiency, and a low rate of atrial arrhythmia
Conal septum and heart block in the unrepaired perimembranous VSD group.
Of note, the unrepaired patients had a significantly smaller VSD
and left-to-right shunt compared with the repaired group.

RA
Diagnosis
Membranous
Symptoms
Right
atrioventricular interventricular Infants with small defects are usually asymptomatic, and the
orifice septum
detection of a murmur results in their diagnosis. Moderate defects
RV result in a predisposition to pulmonary infection and varying degrees
of growth retardation during the first few years of life but without
Muscular
interventricular severe signs of CHF. Larger defects, particularly nonrestrictive
septum VSDs, produce signs and symptoms of CHF early in the first year
of life, including tachypnea, poor feeding, sweating, irritability,
Figure 49.1  Longitudinal section through the fetal right ventricle and
outflow tract displaying the components contributing to the formation of
failure to thrive, and poor weight gain. This symptom complex
the interventricular septum. RA, Right atrium; RV, right ventricle. results from the increased work of breathing and energy expenditure
associated with pulmonary overcirculation and poor peripheral
perfusion.
nonrestrictive VSDs the drop in PVR accentuates the left-to-right
shunt, leading to a large volume load in the pulmonary circulation, Physical Signs
increasing left atrial pressure, and causing left ventricular volume
overload. Congestive heart failure (CHF) develops with decreased Children with smaller defects may present with a harsh holosystolic
peripheral perfusion and increased work of breathing, associated murmur with no overt signs of CHF. Those patients with larger
with a drop in stroke volume and tissue oxygen delivery. This defects and shunts (pulmonary-to-systemic blood flow ratio [Qp:Qs];
results in activation of the renin-angiotensin system, which further see subsequent discussion) in excess of 2 : 1 may have signs of
increases systemic vasoconstriction and the left-to-right shunting, CHF, including poor growth, tachypnea, poor perfusion with
exacerbating the CHF.6-8 reduced peripheral pulses, a palpable thrill along the left sternal
If infants with nonrestrictive VSDs remain untreated, the border, and a harsh holosystolic murmur loudest over the fourth
pulmonary overcirculation can eventually lead to a fixed elevation intercostal space. There may be a diastolic murmur related to
of PVR and pulmonary vascular disease. These changes are not increased blood return to the left atrium. There is accentuation of
reversible after VSD closure. Eisenmenger complex develops when the second heart sound. Hepatomegaly and pulmonary congestion
PVR becomes greater than systemic resistance, reversing the shunt may be present. Resting oxygen saturation less than 90% before
and causing cyanosis. surgical repair is a sign of significantly elevated PVR and portends
Infants and children with smaller restrictive VSDs remain an increased risk of pulmonary hypertension and death after surgical
asymptomatic. Those with larger nonrestrictive VSDs develop closure.
increasingly severe CHF within the first several months of life.
This manifests as respiratory symptoms and failure to thrive. These Electrocardiography
infants have an appreciable mortality within the first year if left
untreated.9 Children with moderate shunting develop a gradual Typical findings on the electrocardiogram include left ventricular
increase in PVR over time, and as the PVR approaches systemic hypertrophy and left atrial enlargement reflected in bifid P waves
levels, the degree of shunting decreases with improvement in and prominent R and T waves in the inferior leads and V6, par-
symptoms. If untreated, such patients go on to develop Eisenmenger ticularly with larger defects. Findings of right ventricular hypertrophy
complex and die in the third or fourth decade of life. The develop- with an RSR′ pattern in lead V1 occur later in the disease process.
ment of Eisenmenger physiology increases the risk of death 10- to
12-fold and carries a 25-year survival of only 42%.10 Chest X-Ray Examination
Spontaneous closure of VSDs is well documented and is more
likely to occur within the first several months of life in patients Radiographic findings include cardiomegaly and an enlarged main
with smaller defects. Muscular defects are the most likely to close pulmonary artery shadow. Left atrial enlargement and prominence
by further septal muscular development. This is based mostly on of the pulmonary vasculature are also seen. These radiographic
autopsy studies in which most of the postmortem reports of findings are more notable with larger defects and may be absent
spontaneously closed defects are of the muscular type.11 Perimem- or more subtle with small VSDs.
branous VSDs have the next highest rate of spontaneous closure.
The mechanism of spontaneous closure of these defects frequently Echocardiography
involves the adherence of excess (aneurysmal) tricuspid valve tissue.
Before the description of this mechanism in 1970, septal aneurysms The type, size, number, and location of medium and large VSDs
were often presumed to be congenital in origin.12 A recent natural can be accurately defined by two-dimensional transthoracic
 CHAPTER 49  Ventricular Septal Defects 599

Supracristal
Membranous

A B

Muscular
Inlet

C D
Figure 49.2  Various types of ventricular septal defects viewed from within the right ventricle. (A) Supra-
cristal or subarterial. (B) Membranous, perimembranous, or paramembranous. (C) Inlet or atrioventricular
(AV) canal type. (D) Muscular or trabecular.

echocardiography. Figs. 49.3 to 49.6 demonstrate the four Cardiac Catheterization

common VSD types based on their location. Left atrial and
ventricular dimensions can be measured and may be important Cardiac catheterization may be indicated when echocardio-
in determining the management of a particular defect. Estimates graphic data are unsatisfactory, in patients with large defects
of pulmonary artery pressure can be made using Doppler imaging and significant pulmonary hypertension, or if there is doubt
of the velocity of a tricuspid regurgitant jet, if present. Other about the anatomy of associated lesions. Recent American Heart
associated cardiac anomalies and the relationship of the VSD Association and American Thoracic Society (AHA/ATS) guide-
to the surrounding structures and valves can also be delineated lines for pediatric pulmonary hypertension recommend cardiac
with this modality. In the majority of cases echocardiography catheterization to measure PVR index (PVRI) if early repair
provides sufficient information to proceed with closure of the has not been performed in the first 1 to 2 years of life for a
defect or to follow it expectantly. Transesophageal or epicardial significant VSD.14
echocardiography is particularly important for providing an Cardiac catheterization can demonstrate the location, size, and
intraoperative assessment of VSD closure. Small defects can be number of defects, along with associated cardiac anomalies. It
missed, especially if they are in the apex of the heart or in the vicin- facilitates direct measurement of left and right heart pressures and
ity of larger ones, and often reveal themselves after closure of the oxygen saturations (Fig. 49.7), from which quantification of the
bigger VSD. intracardiac shunt and PVR may be derived.
 600 PART V Defects

Figure 49.3  Transthoracic two-dimensional echocardiogram showing Figure 49.6  Four-chamber view of a midmuscular ventricular septal
the short-axis view of a supracristal ventricular septal defect (VSD). Note defect (VSD). LV, Left ventricle; RV, right ventricle.
the defect in the 1- to 2-o’clock position at the right ventricular outflow
tract. RA, Right atrium; RV, right ventricle.

Figure 49.4  Short-axis view in a transthoracic echocardiogram of a

perimembranous VSD. The defect is seen at the 10-o’clock position close
to the tricuspid valve, in contrast to the position of the supracristal defect
shown in Fig. 49.3. RV, Right ventricle; TV, tricuspid valve; VSD, ventricular
septal defect.

Figure 49.7  Pathophysiology of ventricular septal defect (VSD). Note

step-up in O2 saturation from the right atrium (66%) to the right ventricle
(86%), indicating left-to-right shunt at the ventricular level. Right ventricular
pressure (60/5 mm Hg) is elevated compared with the normal right ven-
tricular pressure but is less than the left ventricular pressure (90/5  mm  Hg),
indicating that the VSD is restrictive. The pulmonary artery pressure is
elevated as well. m, Mean pressure.

Figure 49.5  Four-chamber view of an inlet ventricular septal defect

(VSD), displaying its posterior location at the level of the tricuspid and
mitral valves. LV, Left ventricle; MV, mitral valve; RV, right ventricle; TV,
tricuspid valve.
 CHAPTER 49  Ventricular Septal Defects 601

The shunt fraction is the ratio of pulmonary blood flow (Qp) develops. Simultaneous aortic valvuloplasty should be considered
to systemic blood flow (Qs). It is calculated according to the fol- if the AR has progressed beyond a moderate degree. One study
lowing formula: has shown that lesser degrees of AR remained stable after simple
Q p:Q s = [( AO2 − MVO2 ) (PVO2 − PaO2 )] defect closure, and more severe regurgitation was associated with
a significant need for reintervention despite aortic valvuloplasty
where AO2 is the aortic oxygen saturation, MVO2 is the mixed at the time of VSD repair.18 A recent study on adults, however,
venous oxygen saturation, PaO2 is the pulmonary arterial saturation, showed that patients older than 40 with unrepaired supracristal
and PVO2 is the pulmonary venous saturation.14 VSDs have a lower risk of AR progression than younger patients,
One can calculate the PVR using the formula: questioning the need for routine prophylactic repair in this specific
population.19
( Mean PAP − PCWP or LAP) × 80 Some patients with pressure-restrictive VSD by Doppler criteria
PVR =
Cardiac Output can still develop progressive left heart dilation due to the “volume
unrestrictive” nature of the defect. Although such patients are
where PAP is the pulmonary artery pressure, PCWP is the pul- referred for surgical closure, this practice has been questioned by
monary capillary wedge pressure, and LAP is the left atrial some based on observations that the left heart dilation often regresses
pressure. spontaneously over time.20
Surgical repair of the VSD is considered safe for PVRI less than Children with small VSDs and left-to-right shunts less than
6 Wood units (WU) × m2 (WU • m2) or if the ratio of pulmonary 1.5 : 1 generally have no symptoms. Although they are at risk of
to systemic vascular resistance (PVR/SVR) is less than 0.3.14 The bacterial endocarditis, close follow-up and prophylactic antibiotic
AHA/ATS guidelines recommend testing for vascular reactivity therapy may be considered as an alternative to surgical repair.21 A
during catheterization using inhaled nitric oxide and oxygen to assess report from the Swedish registry for congenital heart disease noted
for reversibility of pulmonary hypertension for PVRI that is greater a 20- to 30-fold higher incidence of infective endocarditis among
than 6 WU • m2 and for PVR/SVR that is 0.3 or higher. Repair adults with small unrepaired VSDs compared with the general
can be beneficial if the vascular bed is reactive but is contraindicated population.22 Some would hence recommend surgical closure of
if it is not. In one study, for example, patients with a PVRI of less small VSDs after an episode of VSD-associated infective endocar-
than 8 WU • m2 had a greater than 90% survival after surgery, ditis. Preemptive surgical closure for such small VSDs is
whereas patients with a PVRI of greater than 8 WU • m2 had a controversial.
survival of less than 45%.15 Heart-lung transplantation or lung A special situation in the neonatal period occurs when an infant
transplantation with VSD closure is the only surgical option for is diagnosed with an aortic coarctation and a concomitant VSD.
this group of patients.16 The optimal management strategy for neonates with this combina-
With the availability of newer agents for managing pulmonary tion of lesions is controversial. A two-stage approach, involving
hypertension, it is not unreasonable to treat patients with single coarctation repair with or without pulmonary artery banding via
or multiple pulmonary vasodilators for a period of time and reassess a left thoracotomy, followed by VSD closure and removal of the
the hemodynamics with cardiac catheterization to revisit candidacy band 6 to 12 months later, has been demonstrated to be safe and
for surgical repair. effective.23,24 A single-stage approach of simultaneous VSD and
coarctation repair via a median sternotomy can be performed with
Indications for Surgical Repair comparably low morbidity and mortality.25 A single-stage, two-
incision approach is another alternative whereby the coarctation
Infants with a large defect and significant CHF in whom spontane- repair is performed via a thoracotomy followed immediately by
ous closure is unlikely are candidates for early closure, regardless the VSD repair via sternotomy.26 It is also a reasonable strategy to
of the patient’s size. A trial of diuretic therapy with or without repair the coarctation through a left thoracotomy and leave the
the addition of digoxin may control the symptoms of CHF and pulmonary artery unbanded. If the infant remains in severe CHF,
allow the infant to grow. The addition of an angiotensin-converting even after “unloading” of the systemic output with coarctation
enzyme (ACE) inhibitor to reduce SVR may be helpful in selected repair, the VSD can be closed through a sternotomy with a short
cases. In these cases, surgical repair is typically performed between period of cardiopulmonary bypass (CPB).
3 and 6 months of age. If medical therapy fails, surgical repair Pulmonary artery banding is rarely indicated for the treatment
should be undertaken promptly and can be done safely even in of VSD except in some infants with multiple or complex defects
neonates. and/or contraindications for being placed on CPB (sepsis, intra-
Children with moderate-sized defects and shunts greater than cranial hemorrhage). Banding in this situation controls the heart
1.5 : 1 generally have mild to moderate elevations of pulmonary failure and allows the resolution of comorbid conditions before
artery pressure and resistance. They can be followed until they are surgical VSD closure and pulmonary artery debanding.
up to 5 years of age to maximize the chance of spontaneous closure.
Failing the latter, surgical repair may be performed. Operative Management
Patients with subarterial or supracristal VSDs can develop
progressive AR due to prolapse of the adjacent aortic valve leaflet With the patient in supine position a median sternotomy is
caused by Venturi forces associated with left-to-right flow across performed and the thymus subtotally resected to facilitate exposure.
the defect.17 The risk of aortic valve prolapse increases with increasing The pericardium is opened and suspended. Ascending aortic and
defect size. Surgical closure of these defects is usually recommended bicaval venous cannulation are performed after heparinization.
because the rate of spontaneous closure is low and the risk of Purse-string sutures should all be elongated and narrow. CPB is
developing aortic valve insufficiency is common. These defects established, and, depending on the anticipated complexity of the
should be repaired as soon as there is echocardiographic evidence procedure, cooling anywhere from 34°C to 30°C is begun. The
or physical findings of AR and definitely before significant AR left heart can be decompressed by placing a vent through the left
 602 PART V Defects

Ant. TV leaflet

P.

S.

Cor. sinus
AV node
Membranous VSD B
A
Figure 49.8  Ventricular septal defect (VSD) closure via the transatrial approach. (A) A right atriotomy has
been performed, and the VSD is seen across the tricuspid valve (TV). Note the position of the atrioven-
tricular node (AV node) and the conduction system that runs along the posterior and inferior border of
the VSD. (B) A Dacron or PTFE patch is used to close the defect. A portion of the TV suspensory apparatus
is being retracted. P., Posterior leaflet; PTFE, polytetrafluoroethylene; S., septal leaflet of the tricuspid
valve.

atrial appendage or, more commonly, the right superior pulmonary in the superior vena cava and pulmonary artery can be made, and
vein. Cardioplegia is administered in antegrade fashion after cross- the Qp:Qs can be estimated. A decision can then be made to go
clamping the aorta and redosed at regular intervals if necessary. back on bypass to repair the residual defect if the Qp:Qs remains
For neonates, another option is to use single venous cannulation significant (≥1.5 : 1). Rarely, if it is felt that further attempts at
through the right atrial appendage and perform the repair under repair may be unsuccessful, a pulmonary artery band can be placed
hypothermic circulatory arrest at a rectal temperature of 18°C. to restrict the pulmonary blood flow with plans to repair the
An oblique right atrial incision is made after the caval snares residual defects in the future, allowing time for spontaneous
are secured. Retractors are positioned after placement of stay sutures. regression of the defects and somatic growth.
The anatomy is inspected through the tricuspid valve. VSDs are For infants with moderate to severe pulmonary hypertension,
ordinarily closed with a patch (usually polytetrafluoroethylene placement of a pulmonary artery catheter, left atrial line, and even
[PTFE], Dacron, bovine, or glutaraldehyde-treated autologous a peritoneal dialysis catheter should be considered to aid in
pericardium) unless they are quite small. Patch material can be postoperative management. Inhaled nitric oxide can also be helpful
sewn into place with either interrupted sutures or a continuous in the postoperative management of this subgroup of patients.
technique. Particular care is required with the superior sutures to The vast majority of membranous and inlet VSDs are closed
avoid injury to the aortic valve; inferiorly, sutures are placed away via the transatrial approach (Fig. 49.8) but can be closed through
from the margins of the VSD to avoid the conduction tissue. a ventriculotomy in selected cases. Supracristal defects must be
Occasionally exposure of the defect may be compromised by the closed through either a ventriculotomy or an incision in the
tricuspid valve or supporting apparatus. In these cases it may be pulmonary artery, with sutures anchored to the pulmonary valve
helpful to partially detach the septal leaflet of the tricuspid valve annulus.
to facilitate exposure of the lateral and inferior borders. The septal Muscular VSDs, especially multiple/complex defects remain a
leaflet is then repaired with a running polypropylene suture. surgical challenge. Whereas inlet and midmuscular defects can be
After repair of the VSD is complete, the tricuspid valve is tested repaired through the tricuspid valve, anterior defects are sometimes
for competence and repaired as necessary. An ASD or patent foramen approached via a right ventriculotomy and apical VSDs through
ovale, if present, is closed. The right atrium is closed, and after an apical left ventriculotomy. To enhance exposure of these defects,
deairing the heart the cross-clamp is removed. When deairing and transection of crossing muscle bundles or even the moderator band
rewarming are complete, the patient is separated from CPB. has been suggested.27 Other described techniques include placing
Ultrafiltration can be undertaken either during CPB or after an “oversized patch” on the left ventricular side of the defect and
cessation of CPB but before decannulation and protamine admin- the “sandwich” technique, in which sutures are passed along the
istration. Ideally, intraoperative transesophageal or epicardial inferior rim of an anterior muscular VSD and brought out of the
echocardiography is used to rule out a residual VSD. Information epicardial surface of the right ventricle and tied down, hence
about tricuspid and aortic valve competence and ventricular function obliterating the defect.28 A cardioscope, introduced through the
is also obtained using this modality. If there is a question about aortic root, can aid in visualization of the muscular defects, permit-
the significance of a residual VSD, direct measurement of pulmonary ting direct inspection of the left side of the ventricular septum
artery pressure and simultaneous measurement of oxygen saturations and illuminating the defects from the right ventricular aspect.
 CHAPTER 49  Ventricular Septal Defects 603

Alternatively, a right-angle clamp introduced through an aortotomy of brain natriuretic peptide are elevated in the postoperative period
to probe the septum from the left side can be helpful in identifying compared with their preoperative values and seem to continue to
the defects. Multiple apical defects can be effectively repaired with rise for at least 3 days following repair.37
the septal obliteration technique, in which the defects are excluded Patients who have long-standing pulmonary overcirculation or
from the right ventricular cavity with a pericardial patch.29 A simpler preoperative evidence of elevated PVR are prone to pulmonary
technique involves primary closure of the muscular defects after hypertensive crises early in their postoperative recovery. A pulmonary
identifying each VSD with a silk suture passed through the defect artery line may be helpful in these patients. A typical pulmonary
from the right ventricle and fished out of the left heart via an hypertension protocol to maintain pulmonary vasodilation involves
incision in the interatrial septum.30 full sedation and paralysis, hyperventilation, strict acid-base control,
The intraoperative placement of devices designed for transcatheter and fastidious pulmonary toilet. Use of inhaled nitric oxide and
closure of ASDs or VSDs is another option for muscular defects.31 inhaled prostacyclin analogues is highly recommended in addition
Periventricular device deployment without the use of CPB has to conventional measures mentioned earlier with transition to oral
been described by multiple groups—initially in muscular VSDs pulmonary vasodilatory agents if the pulmonary artery pressures
but more recently also in perimembranous and supracristal remain elevated.14
defects.32-35 The periventricular approach is usually through a median Early postoperative dysrhythmias occur in up to one-third of
sternotomy. After placement of a purse-string suture on the right patients.38 Supraventricular and junctional ectopic tachycardias
ventricle, a guide wire is introduced through the purse-string suture are usually accompanied by marked deterioration in cardiac output
across the VSD under transesophageal echocardiography guidance. and must be aggressively treated. Cooling the patient to 33°C or
A sheath that is introduced into the right ventricle over the guide 34°C is effective with simple topical cooling. A peritoneal dialysis
wire is used to deploy the closure device across the defect. Extreme catheter, if present, may also be used for this purpose. These
care must be taken to avoid a transmural injury to the left ventricular interventions require sedation and frequently neuromuscular
free wall by the guide wire or the introducer. blockade. Amiodarone, procainamide, dexmedetomidine, or a
combination of these agents can be used to treat these tachycardias
Postoperative Care should the response to cooling be insufficient.
Complete heart block is another complication of VSD closure.
Extubation in the operating room for older uncomplicated patients It is often transient in nature, due to surgical trauma or edema
is appropriate. Neonates and infants may require ventilatory support associated with suture placement adjacent to the conduction tissue.
and aggressive diuresis for 24 to 72 hours before safe extubation. Heart block usually resolves in 24 to 48 hours. Disruption of the
If postoperative inotropic support is required, diuresis may not be conduction pathway results in permanent heart block. A contem-
effective in the initial 24 hours after surgery. porary series quoted a 5% incidence of combined temporary and
On the patient’s admission to the intensive care unit a baseline permanent heart block and a 2% incidence of complete heart
measurement of hemodynamic parameters is performed. The heart block requiring a pacemaker implantation.39 All patients undergoing
rate and rhythm, arterial blood pressure, central venous pressure, VSD repair should have temporary atrial and ventricular epicardial
and core and peripheral temperatures are monitored. Left atrial pacing wires placed at the time of surgery. These temporary wires
or pulmonary artery pressures may also be monitored in complicated are useful in both the diagnosis and treatment of dysrhythmias
cases or in patients who are at risk for or have exhibited signs of during the early postoperative period. If there is postoperative
pulmonary hypertension. Admission hematocrit, serum chemistries, complete heart block, a transvenous or epicardial permanent
serum lactate level, arterial blood gas values, and coagulation pacemaker system is required if sinus rhythm is not restored within
parameters are checked. In addition to an electrocardiogram, chest 7 to 10 days.
radiography to check support equipment position and the pleural
spaces is performed. Outcome
Estimates of cardiac output are made on the basis of peripheral
perfusion and temperature, strength of pedal pulses, urinary output, Mortality rates for isolated VSD closure are less than 1%. For
serum lactate, and acid-base status. Some programs also use near- closure of multiple VSDs the rate may be as high as 7%.40 The
infrared spectroscopy routinely or selectively to monitor the incidence of residual shunting due to patch dehiscence is very
adequacy of tissue oxygenation. Volume replacement may be small. On the contrary, it is common to have some degree of
required for low filling pressures. For elevated filling pressures residual shunting from small residual defects around the patch,
(>10 mm Hg) and low cardiac output, inotropic support is required. with an incidence reported to be approximately 33%.41 The same
A vasodilating agent is used to treat inadequate cardiac output study noticed that two-thirds of these residual defects closed by
associated with an elevated mean arterial blood pressure. Milrinone the time of hospital discharge. Fig. 49.9 shows a residual patch
works well in this situation. The majority of patients undergoing leak on echocardiography with color Doppler imaging. Morbidity
straightforward VSD closure require minimal if any inotropic rates in infants seem to be higher with decreasing operative weight.
support postoperatively and can be extubated fairly promptly. Most In patients with postoperative right bundle branch block, there is
benefit from diuresis instituted within 12 hours of surgery and a tendency toward left ventricular dilation in the long term, which
continued until after the institution of enteral feedings. In patients confirms the need for long-term follow-up of these patients.42
with a large VSD and significant left-to-right shunt, it is not Long-term survival is excellent after successful VSD repair.
uncommon to find depressed left ventricular function by echo-
cardiography in the immediate postoperative period, especially if Interventional Therapy
their repair was delayed.36 It is felt that an acute change in loading
conditions—decreased preload from closure of the defect and an Percutaneous transcatheter closure of VSDs with devices is less
increased afterload from excluding the relatively low-resistance well established than device closure of ASDs. This technology
pulmonary circulation—contributes to this finding. Plasma levels has most commonly been used in the treatment of muscular
 604 PART V Defects

patient size because most patients with hemodynamically significant

VSDs present with failure to thrive during infancy. Currently in
the United States, percutaneous closure of perimembranous VSDs
in the cardiac catheterization laboratory is not a recommended or
commonly performed procedure.
A staged approach to treating infants with multiple VSDs
involving initial pulmonary artery banding followed by delayed
catheter-based closure of the muscular VSDs that have failed to
close spontaneously in the intervening period may prove effective
in the management of these challenging patients. As discussed
earlier, a combined approach in the operating room could also be
effective. As the technology improves, the indications for these
procedures will expand. Patient follow-up will be important because
the long-term outcomes of these procedures are still being accrued.

Figure 49.9  Two-dimensional echocardiogram with color Doppler

Selected References
revealing a residual defect at the border of a patch following closure of a
A complete list of references is available at ExpertConsult.com.
perimembranous defect. LA, Left atrium; LV, left ventricle; RA, right atrium;
RV, right ventricle.
1. Lamers WH, Wessels A, Verbeek FJ, et al. New findings concerning
ventricular septation in the human heart. Implications for maldevelop-
ment. Circulation. 1992;86(4):1194–1205.
VSDs with acceptable results.43 The proximity of vital structures 2. Jacobs JP, Burke RP, Quintessenza JA, Mavroudis C. Congenital
such as the aortic valve and the septal leaflet of the tricuspid heart surgery nomenclature and database project: ventricular septal
valve to the paramembranous VSD makes device closure of this defect. Ann Thorac Surg. 2000;69(suppl 4):S25–S35.
12. Misra KP, Hildner FJ, Cohen LS, Narula OS, Samet P. Aneurysm of
defect more challenging, although newer modifications have been the membranous ventricular septum. A mechanism for spontaneous
developed. closure of ventricular septal defect. N Engl J Med. 1970;283(2):58–61.
The only US Food and Drug Administration–approved phase 13. Gabriels C, De Backer J, Pasquet A, et al. Long-term outcome of
I clinical trial for device closure of hemodynamically significant patients with perimembranous ventricular septal defect: results from
perimembranous VSDs was reported in 2006.44 This multicenter the Belgian registry on adult congenital heart disease. Cardiology.
study used the Amplatzer Membranous VSD Occluder (AGA 2017;136(3):147–155.
Medical Corp., Golden Valley, MN) in 32 of 35 patients in whom 14. Abman SH, Hansmann G, Archer SL, et al. Pediatric pulmonary
it was attempted. The median age was 7.7 years, and patients hypertension: guidelines from the American Heart Association and
under 8 kg were excluded from the study. Although the rate of American Thoracic Society. Circulation. 2015;132(21):2037–2099.
complete closure by echocardiography was 96% by 6 months, 2 19. Egbe AC, Poterucha JT, Dearani JA, Warnes CA. Supracristal
ventricular septal defect in adults: Is it time for a paradigm shift?
patients required permanent pacemaker implantation at 3 and 16 Int J Cardiol. 2015;198:9–14.
months post procedure. New AR developed in 53% of patients 20. Kleinman CS, Tabibian M, Starc TJ, Hsu DT, Gersony WM.
within 24 hours of the procedure, deceasing to 39% by 6 months. Spontaneous regression of left ventricular dilation in children with
Of the 35 patients, 4 were referred to surgery for either device restrictive ventricular septal defects. J Pediatr. 2007;150(6):583–
placement–related significant AR, inadequate device positioning, 586.
or difficulty with device retrieval causing entanglement in the 22. Berglund E, Johansson B, Dellborg M, et al. High incidence of
tricuspid valve chordae. A total of 6 out of 35 (17%) patients infective endocarditis in adults with congenital ventricular septal
eventually needed a surgical intervention either for pacemaker defect. Heart. 2016.
placement or for VSD closure. 26. Kanter KR, Mahle WT, Kogon BE, Kirshbom PM. What is the
A later multi-institutional study narrowed the device closure optimal management of infants with coarctation and ventricular
septal defect? Ann Thorac Surg. 2007;84(2):612–618, discussion 618.
to a subpopulation of membranous defects that had an associated 27. Seddio F, Reddy VM, McElhinney DB, Tworetzky W, Silverman NH,
aneurysm.45 The investigators used the Amplatzer Duct Occluder Hanley FL. Multiple ventricular septal defects: how and when should
I device, positioning it within the aneurysm and hence away from they be repaired? J Thorac Cardiovasc Surg. 1999;117(1):134–139,
the aortic valve and from the crest of the ventricular septum, which discussion 139–140.
correlated with a reduced incidence of new AR and heart block. 28. Kitagawa T, Durham LA 3rd, Mosca RS, Bove EL. Techniques and
The authors selected patients with a “wind-sock” appearance of results in the management of multiple ventricular septal defects.
the aneurysm, with very specific inclusion criteria based on the J Thorac Cardiovasc Surg. 1998;115(4):848–856.
geometry of the aneurysm, the details of which are beyond the 30. Talwar S, Bhoje A, Airan B. A simple technique for closing mul-
scope of this chapter. The device was successfully placed in 19 of tiple muscular and apical ventricular septal defects. J Card Surg.
21 selected patients, whose weights were all above 8 kg with a 2015;30(9):731–734.
32. Bacha EA, Cao QL, Starr JP, Waight D, Ebeid MR, Hijazi ZM.
median age of 5 years. Perventricular device closure of muscular ventricular septal defects
A recent meta-analysis of published studies comparing device on the beating heart: technique and results. J Thorac Cardiovasc Surg.
closure with surgical closure of perimembranous VSDs showed 2003;126(6):1718–1723.
comparable rates of successful closure and short-term complication 33. Omelchenko A, Gorbatykh Y, Voitov A, Zaitsev G, Bogachev-
rates.46 The mean age in the device group was 12.2 years, whereas Prokophiev A, Karaskov A. Perventricular device closure of ventricular
that of the surgical cohort was 5.5 years. An important current septal defects: results in patients less than 1 year of age. Interact
limitation to the application of percutaneously placed devices is Cardiovasc Thorac Surg. 2016;22(1):53–56.
 CHAPTER 49  Ventricular Septal Defects 605

34. Hongxin L, Wenbin G, Liang F, Zhang HZ, Zhu M, Zhang WL. 42. Veeram Reddy SR, Du W, Zilberman MV. Left ventricular mechanical
Perventricular device closure of a doubly committed juxtaarterial synchrony and global systolic function in pediatric patients late
ventricular septal defect through a left parasternal approach: midterm after ventricular septal defect patch closure: a three-dimensional
follow-up results. J Cardiothorac Surg. 2015;10:175. echocardiographic study. Congenit Heart Dis. 2009;4(6):454–458.
35. Zhang S, Zhu D, An Q, Tang H, Li D, Lin K. Minimally invasive 43. Holzer R, Balzer D, Cao QL, Lock K, Hijazi ZM. Amplatzer muscular
periventricular device closure of doubly committed sub-arterial ventricular septal defect I. Device closure of muscular ventricular
ventricular septal defects: single center long-term follow-up results. septal defects using the Amplatzer muscular ventricular septal defect
J Cardiothorac Surg. 2015;10:119. occluder: immediate and mid-term results of a U.S. registry. J Am
36. Pacileo G, Pisacane C, Russo MG, et al. Left ventricular mechanics Coll Cardiol. 2004;43(7):1257–1263.
after closure of ventricular septal defect: influence of size of the defect 44. Fu YC, Bass J, Amin Z, et al. Transcatheter closure of perimembranous
and age at surgical repair. Cardiol Young. 1998;8(3):320–328. ventricular septal defects using the new Amplatzer membranous
37. Mainwaring RD, Parise C, Wright SB, Juris AL, Achtel RA, Fallah VSD occluder: results of the U.S. phase I trial. J Am Coll Cardiol.
H. Brain natriuretic peptide levels before and after ventricular septal 2006;47(2):319–325.
defect repair. Ann Thorac Surg. 2007;84(6):2066–2069. 45. El Said HG, Bratincsak A, Gordon BM, Moore JW. Closure of
39. Anderson BR, Stevens KN, Nicolson SC, et al. Contemporary perimembranous ventricular septal defects with aneurysmal tissue
outcomes of surgical ventricular septal defect closure. J Thorac using the Amplazter Duct Occluder I: lessons learned and medium
Cardiovasc Surg. 2013;145(3):641–647. term follow up. Catheter Cardiovasc Interv. 2012;80(6):895–903.
41. Preminger TJ, Sanders SP, van der Velde ME, Castaneda AR, 46. Saurav A, Kaushik M, Mahesh Alla V, et al. Comparison of percu-
Lock JE. “Intramural” residual interventricular defects after repair taneous device closure versus surgical closure of peri-membranous
of conotruncal malformations. Circulation. 1994;89(1):236– ventricular septal defects: A systematic review and meta-analysis.
242. Catheter Cardiovasc Interv. 2015;86(6):1048–1056.
 CHAPTER 49  Ventricular Septal Defects 605.e1

References with severe pulmonary hypertension. Chest. muscular ventricular septal defects: the septal
1989;96(1):31–39. obliteration technique. Ann Thorac Surg.
1. Lamers WH, Wessels A, Verbeek FJ, et al. 16. Haworth SG. Pulmonary vascular disease 2000;70(1):106–110.
New findings concerning ventricular in ventricular septal defect: structural and 30. Talwar S, Bhoje A, Airan B. A simple
septation in the human heart. Implica- functional correlations in lung biopsies from technique for closing multiple muscular
tions for maldevelopment. Circulation. 85 patients, with outcome of intracardiac and apical ventricular septal defects. J Card
1992;86(4):1194–1205. repair. J Pathol. 1987;152(3):157–168. Surg. 2015;30(9):731–734.
2. Jacobs JP, Burke RP, Quintessenza JA, 17. Tohyama K, Satomi G, Momma K. Aortic 31. Okubo M, Benson LN, Nykanen D, et al.
Mavroudis C. Congenital Heart Surgery valve prolapse and aortic regurgitation associ- Outcomes of intraoperative device closure
Nomenclature and Database Project: ated with subpulmonic ventricular septal of muscular ventricular septal defects. Ann
ventricular septal defect. Ann Thorac Surg. defect. Am J Cardiol. 1997;79(9):1285–1289. Thorac Surg. 2001;72(2):416–423.
2000;69(suppl 4):S25–S35. 18. Cheung YF, Chiu CS, Yung TC, Chau AK. 32. Bacha EA, Cao QL, Starr JP, Waight D, Ebeid
3. E A. Ventricular septal defect. In: Baue Impact of preoperative aortic cusp prolapse MR, Hijazi ZM. Perventricular device closure
AEGA, Hammond GL, et al, eds. Glenn’s on long-term outcome after surgical closure of muscular ventricular septal defects on the
Thoracic and Cardiovascular Surgery. Norwalk of subarterial ventricular septal defect. Ann beating heart: technique and results. J Thorac
CT: Appleton & Lange; 1991:1007–1016. Thorac Surg. 2002;73(2):622–627. Cardiovasc Surg. 2003;126(6):1718–1723.
4. Lucas RV Jr, Adams P Jr, Anderson RC, 19. Egbe AC, Poterucha JT, Dearani JA, Warnes 33. Omelchenko A, Gorbatykh Y, Voitov A,
Meyne NG, Lillehei CW, Varco RL. The CA. Supracristal ventricular septal defect in Zaitsev G, Bogachev-Prokophiev A, Karaskov
natural history of isolated ventricular septal adults: Is it time for a paradigm shift? Int J A. Perventricular device closure of ventricular
defect. A serial physiologic study. Circulation. Cardiol. 2015;198:9–14. septal defects: results in patients less than 1
1961;24:1372–1387. 20. Kleinman CS, Tabibian M, Starc TJ, Hsu year of age. Interact Cardiovasc Thorac Surg.
5. Kirklin JWB-BB. Ventricular septal defect. In: DT, Gersony WM. Spontaneous regression 2016;22(1):53–56.
Kirklin JWB-BB, ed. Cardiac Surgery. New of left ventricular dilation in children with 34. Hongxin L, Wenbin G, Liang F, Zhang HZ,
York: Churchill Livingstone; 1993:751–764. restrictive ventricular septal defects. J Pediatr. Zhu M, Zhang WL. Perventricular device
6. Boucek MM, Chang R, Synhorst DP. Renin- 2007;150(6):583–586. closure of a doubly committed juxtaarte-
angiotensin II response to the hemodynamic 21. Gersony WM, Hayes CJ. Bacterial endocar- rial ventricular septal defect through a left
pathology of ovines with ventricular septal ditis in patients with pulmonary stenosis, parasternal approach: midterm follow-up
defect. Circ Res. 1989;64(3):524–531. aortic stenosis, or ventricular septal defect. results. J Cardiothorac Surg. 2015;10:175.
7. Gidding SS, Bessel M. Hemodynamic Circulation. 1977;56(suppl 1):I84–I87. 35. Zhang S, Zhu D, An Q, Tang H, Li D, Lin
correlates of clinical severity in isolated 22. Berglund E, Johansson B, Dellborg M, et al. K. Minimally invasive perventricular device
ventricular septal defect. Pediatr Cardiol. High incidence of infective endocarditis in closure of doubly committed sub-arterial
1993;14(3):135–139. adults with congenital ventricular septal ventricular septal defects: single center long-
8. Scammell AM, Diver MJ. Plasma renin activ- defect. Heart. 2016. term follow-up results. J Cardiothorac Surg.
ity in infants with congenital heart disease. 23. Isomatsu Y, Imai Y, Shin’oka T, Aoki M, 2015;10:119.
Arch Dis Child. 1987;62(11):1136–1138. Sato K. Coarctation of the aorta and ven- 36. Pacileo G, Pisacane C, Russo MG, et al.
9. Rein JG, Freed MD, Norwood WI, Cas- tricular septal defect: should we perform a Left ventricular mechanics after closure of
taneda AR. Early and late results of closure single-stage repair? J Thorac Cardiovasc Surg. ventricular septal defect: influence of size of
of ventricular septal defect in infancy. Ann 2001;122(3):524–528. the defect and age at surgical repair. Cardiol
Thorac Surg. 1977;24(1):19–27. 24. Alsoufi B, Cai S, Coles JG, Williams WG, Young. 1998;8(3):320–328.
10. Kidd L, Driscoll DJ, Gersony WM, et al. Van Arsdell GS, Caldarone CA. Outcomes 37. Mainwaring RD, Parise C, Wright SB, Juris
Second natural history study of congenital of different surgical strategies in the treat- AL, Achtel RA, Fallah H. Brain natriuretic
heart defects. Results of treatment of patients ment of neonates with aortic coarctation peptide levels before and after ventricular
with ventricular septal defects. Circulation. and associated ventricular septal defects. septal defect repair. Ann Thorac Surg.
1993;87(suppl 2):I38–I51. Ann Thorac Surg. 2007;84(4):1331–1336, 2007;84(6):2066–2069.
11. Suzuki H. Spontaneous closure of ven- discussion 1336-1337. 38. Pfammatter JP, Wagner B, Berdat P, et al.
tricular septal defects. Anatomic evidence 25. Gaynor JW, Wernovsky G, Rychik J, Rome Procedural factors associated with early
in six adult patients. Am J Clin Pathol. JJ, DeCampli WM, Spray TL. Outcome fol- postoperative arrhythmias after repair of
1969;52(4):391–402. lowing single-stage repair of coarctation with congenital heart defects. J Thorac Cardiovasc
12. Misra KP, Hildner FJ, Cohen LS, Narula ventricular septal defect. Eur J Cardiothorac Surg. 2002;123(2):258–262.
OS, Samet P. Aneurysm of the membranous Surg. 2000;18(1):62–67. 39. Anderson BR, Stevens KN, Nicolson SC,
ventricular septum. A mechanism for spon- 26. Kanter KR, Mahle WT, Kogon BE, et al. Contemporary outcomes of surgical
taneous closure of ventricular septal defect. Kirshbom PM. What is the optimal man- ventricular septal defect closure. J Thorac
N Engl J Med. 1970;283(2):58–61. agement of infants with coarctation and Cardiovasc Surg. 2013;145(3):641–647.
13. Gabriels C, De Backer J, Pasquet A, ventricular septal defect? Ann Thorac Surg. 40. Serraf A, Lacour-Gayet F, Bruniaux J, et al.
et al. Long-term outcome of patients 2007;84(2):612–618, discussion 618. Surgical management of isolated multiple
with perimembranous ventricular septal 27. Seddio F, Reddy VM, McElhinney DB, ventricular septal defects. Logical approach
defect: results from the belgian registry on Tworetzky W, Silverman NH, Hanley FL. in 130 cases. J Thorac Cardiovasc Surg.
adult congenital heart disease. Cardiology. Multiple ventricular septal defects: how 1992;103(3):437–442, discussion 443.
2017;136(3):147–155. and when should they be repaired? J Thorac 41. Preminger TJ, Sanders SP, van der Velde
14. Abman SH, Hansmann G, Archer SL, et al. Cardiovasc Surg. 1999;117(1):134–139, ME, Castaneda AR, Lock JE. Intramural”
Pediatric pulmonary hypertension: guidelines discussion 139-140. residual interventricular defects after repair
from the american heart association and 28. Kitagawa T, Durham LA 3rd, Mosca of conotruncal malformations. Circulation.
american thoracic society. Circulation. RS, Bove EL. Techniques and results in 1994;89(1):236–242.
2015;132(21):2037–2099. the management of multiple ventricular 42. Veeram Reddy SR, Du W, Zilberman MV.
15. Yamaki S, Mohri H, Haneda K, Endo septal defects. J Thorac Cardiovasc Surg. Left ventricular mechanical synchrony
M, Akimoto H. Indications for surgery 1998;115(4):848–856. and global systolic function in pediatric
based on lung biopsy in cases of ventricular 29. Black MD, Shukla V, Rao V, Smallhorn JF, patients late after ventricular septal
septal defect and/or patent ductus arteriosus Freedom RM. Repair of isolated multiple defect patch closure: a three-dimensional
 605.e2 PART V Defects

echocardiographic study. Congenit Heart 44. Fu YC, Bass J, Amin Z, et al. Transcatheter learned and medium term follow up. Catheter
Dis. 2009;4(6):454–458. closure of perimembranous ventricular Cardiovasc Interv. 2012;80(6):895–903.
43. Holzer R, Balzer D, Cao QL, Lock K, septal defects using the new Amplatzer 46. Saurav A, Kaushik M, Mahesh Alla V, et al.
Hijazi ZM, Amplatzer Muscular Ven- membranous VSD occluder: results of Comparison of percutaneous device closure
tricular Septal Defect I. Device closure of the U.S. phase I trial. J Am Coll Cardiol. versus surgical closure of peri-membranous
muscular ventricular septal defects using 2006;47(2):319–325. ventricular septal defects: a systematic review
the Amplatzer muscular ventricular septal 45. El Said HG, Bratincsak A, Gordon BM, and meta-analysis. Catheter Cardiovasc Interv.
defect occluder: immediate and mid-term Moore JW. Closure of perimembranous ven- 2015;86(6):1048–1056.
results of a U.S. registry. J Am Coll Cardiol. tricular septal defects with aneurysmal tissue
2004;43(7):1257–1263. using the Amplazter Duct Occluder I: lessons