Ventricular Septal defect

Dr. Md. Rezwanul Hoque

MBBS,MS,FCPS, FRCSG, FRCSEd

Associate Professor Department of Cardiac Surgery BSMMU, Dhaka, Bangladesh

Definition
A ventricular septal defect (VSD) is a hole or a defect in the septum that divides the 2 lower chambers of the heart, resulting in communication between the ventricular cavities. A VSD may occur as a primary anomaly, with or without additional major associated cardiac defects. It may also occur as a single component of a wide variety of intracardiac anomalies, including Tetralogy of Fallot, complete atrioventricular (AV) canal defects, Transposition of great arteries, and corrected transpositions.
http://emedicine.medscape.com/article/892980-overview

VSD- Incidence & Prevalence

Second most common cardiac malformation after Bicuspid aortic valve. 2-6/ 1000 live birth. Isolated VSD in 20% of congenital cardiac anomalies Coexists with other in 5% cases Denotes a defect in IVS composed of muscular & membranous segment

IVS
The 4 parts of the interventricular septum :
Inlet septum Smooth-walled and extends from the septal attachments of the tricuspid valve to the distal attachments of the tricuspid tensor apparatus; it is also called the AV canal septum Trabecular septum Coarse trabeculations, also known as the muscular septum or the ventricular sinus septum Outlet (infundibular) septum Smooth-walled, separated from the trabeculated portion of the RV by the septal band of the trabecula septomarginalis; also known as the parietal band or the distal conal septum, and defects in this area may be termed conal septal defects Membranous septum The smallest part of the interventricular septum, lies between the anterior and the septal tricuspid leaflets and below the right and the noncoronary cusps of the aortic valve
http://emedicine.medscape.com/article/892980-overview

Anatomic positions of the defects: Outlet defect (a) Papillary muscle of the conus (b) Perimembranous defect (c); Marginal muscular defects (d); Central muscular defects (e); Inlet defect (f); Apical muscular defects (g).

Types of VSD

Various types of ventricular septal defects (VSDs) from the right ventricular aspect. A = Doubly committed subarterial B = Perimembranous C = Inlet or atrioventricular canal type D = Muscular ventricular septal defect.

Position of VSD as seen from right atrium

VSD-types
Perimembranous (infracristal, conoventricular) VSD-80%

Lie in the LV outflow tract just below the aortic valve. Occur in the membranous septum Defects may extend to the adjacent muscular portion of the septum and they are subclassified as perimembranous inlet, perimembranous outlet, or perimembranous muscular. Perimembranous VSDs are associated with pouches or aneurysms of the septal leaflet of the tricuspid valve, which can partially or completely close the defect. Because of lower position of tricuspid valve compared to mitral valve, an LV-to-RA shunt may be associated with this defect.

LV-RA communication may occur either through the atrioventricular membranous septum (A), or more commonly through a defect in the interventricular septum and a defect in the septal tricuspid valve (B).

Supracristal (conal septal, infundibular, subpulmonic, subarterial, subarterial doubly committed, outlet)-5-8%

VSDs account for 5-8% of isolated VSDs in the United States but 30% of such defects in Japan. These defects lie beneath the pulmonic valve and communicate with the RV outflow tract above the supraventricular crest and are associated with aortic regurgitation secondary to the prolapse of the right aortic cusp.

Muscular VSDs (trabecular)-5-20%

Entirely bounded by the muscular septum and are often multiple. The term Swiss-cheese septum has been used to describe multiple muscular VSDs. Other sub classifications depend on the location and include central muscular or midmuscular, apical, and marginal (when the defect is along the RV-septal junction). Any single defect observed from the LV aspect may have several openings on the RV aspect.

Posterior (canal-type, endocardial cushiontype, AV septum type, inlet, juxtatricuspid)-8-10%

VSDs lie posterior to the septal leaflet of the tricuspid valve. Although the locations of posterior VSDs are similar to those of VSDs observed with AV septal defects, they are not associated with defects of the AV valves.

http://emedicine.medscape.com/article/892980-overview

Malalignment of inlet septum produces mitral or tricuspid valve override or straddle, malalignment of outlet septum may be on the right or left of trabecular septum, e.g. TOF, DORV, TA, TGA it is on the left.

Clinical significance of VSD depends on

Size Qp/Qs ratio determined by SVR versus PVR If Qp/Qs<1.75:1 no appreciable cardiac chamber enlargement occurs, PVR when Qp/Qs>2:1 cardiomegaly appreciable A left-to-right shunt at the ventricular level has 3 hemodynamic consequences: Increased LV volume load Excessive pulmonary blood flow Reduced systemic cardiac output

VSD- Size and Significance

Size Small, restrictive Moderate restrictive Large nonrestrictive

Area <0.5cm2

Qp /Qs <1.5:1 1.5-2.5:1

PSP/ASP <0.3 >0.3

Hemodynamic change LVP high, RVP low Gradient high LA, LV dilatation, PVR, arrhythmia LVP & RVP equalized, LV volume overload, PVR

LR shunt Low Moderate shunt Shunt depends on SVR/PVR

>1.0cm2

>0.3

Natural history
Wide spectrum, ranging from spontaneous closure to congestive heart failure (CHF) to death in early infancy. Spontaneous closure frequently occurs in children, usually occurs by age 2 years. Closure is uncommon after age 4 years. Closure is most frequently observed in muscular defects (80%), followed by perimembranous defects (35-40%). Outlet VSDs have a low incidence of spontaneous closure, and inlet VSDs do not close. Closure may occur by means of hypertrophy of the septum, formation of fibrous tissue, subaortic tags, apposition of the septal leaflet of the tricuspid valve, or (in rare cases) prolapse of a leaflet of the aortic valve. When perimembranous VSDs close because of development of fibrous tissue or the apposition of the tricuspid valve, an aneurysm of the interventricular septum may appear. http://emedicine.medscape.com/article/892980-overview

VSD in Neonate Initially high PVR limits LR shunt, after few weeks normal involution of small pulmonary arterioles LR shunt increases, clinical symptoms become apparent. VSD in Infant After the neonatal high PVR normalizes features of CCF appears Harsh systolic murmur at lower left sternal border points to VSD though the initial CXR and ECG might be normal VSD in Children Asymptomatic in small defect Cardiomegaly with forceful LV impulse, systolic thrill and murmur along lower left sternal border, normally split S2,presence of S3, rumbling apical diastolic murmur, midsystolic ejection murmur at PA, cardiomegaly on CXR, ECHO, CT, LVH/ BVH in ECG, Catheter study shows step up, Qp/ Qs ratio, LR shunt, degree of PVR

VSD in adult
Small VSDs are associated with a relatively high risk of endocarditis but otherwise patients enjoy a normal life expectancy. Atrial arrhythmias may occur.

Moderate VSDs are unusual in the adult but may occur when a prolapsing aortic valve cusp partially obstructs the defect. They are associated with the development of left heart dilation and shunt-related pulmonary hypertension (which often reverses with correction of the defect), and resultant congestive heart failure and atrial fibrillation, as well as the risk of endocarditis. Large VSDs without pulmonary hypertension exist in adults only when associated with obstruction to right ventricular outflow and are rare. All such patients are at risk for endocarditis. Some are cyanotic because of more severe right ventricular outflow tract obstruction at the infundibular or valvular level. VSD patients with Eisenmenger syndrome are at continuous risk of mortality and morbidity. Poor prognostic features are felt to be atrial flutter/fibrillation, syncope, heart failure, hemoptysis and aneurysmal dilation of proximal hypertensive pulmonary arteries which may rupture even with laminated thrombus in such dilated arteries. Five percent of VSDs develop aortic valve regurgitation. Patients with doubly-committed sub-arterial VSDs are more likely to develop aortic regurgitation from progressive prolapse of the aortic valve cusps than those with a perimembranous VSD. Consensus Conference Report on Adult Congenital Heart Disease (ACHD),, Canadian Cardiovascular Society (CCS) -2001

Physiological Classification of Isolated VSD in Adults

Restrictive:
RV pressure < LV pressure in the absence of right ventricular outflow tract obstruction.

Non-restrictive:
Equal right and left ventricular pressures in the absence of right ventricular outflow tract obstruction.

VSD- symptoms

Depends on size, PB Flow & Pressure Small- asymptomatic, detected during routine examination Moderately restrictive- Dyspnoea on exertion, failure to thrive, feeding difficulties, profuse perspiration, rec. pulm. Infection, cardiac failure Large nonrestrictive- Eisenmenger syndrome

VSD- signs

Loud, harsh, blowing holosystolic murmur over lower left sternal border(3rd/ 4th ICS) Displaced cardiac apex S3 sound, p2 Apical diastolic murmur Pulmonary systolic murmur Cardiac failure

VSD- Investigation
CXR-normal to gross Cardiomegaly, pulmonary plethora, Pulm. Edema, effusion, Eisenmenger physiology ECG- Normal broad notched P (lt. atrial ), deep Q, tall R, tall T(LVH)RVH, RADBVH, notched or peaked P Echocardiography- Site, size, number, shunt direction, gradient, PASP, associated anomaly, chamber enlargement, extrapericadial pulm. Vasculature Cardiac cath- confirmation, step up in oximetry, chamber pressure & PVR, Shunt direction & calculation & reversibility, Qp/Qs, associated lesion Cardiac CT- Cardiomegaly, LA, Vascular engorgement MRI- morphologic information on ECG-gated spin-echo & cine MRI. Ventricular volumes, mass, function, shunt, valvular function, pressure gradient by cine MRI- supplementary to echo. Coronary angiography in patients at risk of coronary artery disease or in patients over the age of 40 years if a surgical repair is planned. Open lung biopsy should only be considered when the reversibility of the pulmonary hypertension is uncertain from the hemodynamic data. It is potentially hazardous and should be done only at centres with substantial relevant experience in CHD

VSD- CXR

Cardiomegaly and distinctly increased vasculature strongly suggesting a left-toright shunt. There is also a suggestion of a left superior vena cava which, in turn, may suggest an atrial septal defect -- or it may be partial anomalous venous return to the left superior vena cava.

VSD- Echocardiography

VSD- catheter study

A - Aorta, LV - Left Ventricle, RVOT - Right Ventricular Outflow Tract, Catheter in Left Ventricle, LPA - Left Pulmonary Artery

VSD- complication

Failure to thrive Rec. respiratory infection Heart failure Aortic regurgitation in supracristal VSD Infective endocarditis High PVR with reversal of shunt, Eisenmenger physiology Arrhythmia, subaortic stenosis

VSD- intervention
Very small VSD- follow up, surgical closure not recommended, protection against IE advised Device closure of VSDs may be performed in the setting of isolated trabecular muscular VSDs but are still considered an experimental procedure for perimembranous VSDs Surgical closure under CPB PA banding with later definitive surgery in complicated cases Pulmonary artery banding is reserved for patients with unique circumstances. A small infant with multiple trabecular muscular VSDs may have a better result from definitive surgery after he or she has grown. In addition, some VSDs disappear with time and growth. Certain surgeons have advocated pulmonary artery banding for low birth-weight infants. Others have recommended the same approach as that used for term newborns.
Consensus Conference Report on Adult Congenital Heart Disease (ACHD),,Canadian Cardiovascular Society (CCS) -2001

Device closure
Percutaneous transcatheter device occlusion of membranous VSDs. may be associated with further complications, including conduction anomalies and valve dysfunction. Either Amplatzer Membranous VSD Occluder or clamshell double umbrella device may be used.
A multicentre study of 430 patients demonstrated successful percutaneous VSD closure in 410 (95%) of cases. Complete heart block occurred in 16 patients (4%), aortic regurgitation in 14 patients (2 required surgery), and tricuspid regurgitation in 27 patients (none required surgery). Carminati M et al. Transcatheter closure of congenital ventricular septal defects: results of the European Registry. Eur Heart J. Oct 2007;28(19):2361-8.

Minimally invasive transthoracic device closure of perimembranous VSDs has been performed with an inferior sternotomy, with a 16-gauge trocar, guidewire, sheath, and loading sheath being employed under transesophageal echocardiographic guidance without cardiopulmonary bypass.
Xing Q et al. Minimally invasive perventricular device closure of perimembranous ventricular septal defect without cardiopulmonary bypass: Multicentre experience and mid-term follow-up. J Thorac Cardiovasc Surg. Apr 2 2010

The following situations warrant operative closure:

The presence of a "significant" VSD [symptomatic; Qp/Qs of 2/1; pulmonary artery systolic pressure > 50 mmHg]; deteriorating ventricular function due to volume (LV) or pressure (RV) overload). Significant right ventricular outflow tract obstruction (cath gradient or mean echo gradient greater than 50 mmHg). A perimembranous or doubly committed VSD with more than mild aortic incompetence. In the presence of severe pulmonary hypertension (PAP > 2/3 SABP or pulmonary arteriolar resistance greater than 2/3 systemic arteriolar resistance), there must be a net left-to-right shunt of at least 1.5:1; or evidence of pulmonary artery reactivity when challenged with a pulmonary vasodilator (e.g. oxygen, nitric oxide and/or prostaglandins); or lung biopsy evidence that pulmonary arterial changes are potentially reversible (Heath Edwards grade II-III or less).

Timing of surgery
For symptomatic patients or patients with larger ventricular septal defects (VSDs) or elevated PVR, early surgical closure is indicated. The ideal time to intervene is when the likelihood of spontaneous VSD closure is lowest and the risk of irreversible pulmonary vascular disease and ventricular dysfunction will be minimized. In subarterial VSDs, the risk of irreversible aortic valve damage caused by cusp prolapse leads to earlier intervention. With perimembranous and muscular defects, surgery may be reasonably delayed up to 1 year or more if the infant is thriving and the pulmonary artery pressure is known to be near normal.

Surgical/Interventional Outcomes
Successful closure is associated with excellent survival if ventricular function is normal. Elevated pulmonary artery pressures preoperatively may progress, regress or remain unchanged postoperatively.

Contraindication for surgery

Irreversible pulmonary vasculature changes with no reactivity to oxygen or nitric oxide.

Post operative arrhythmias Atrial fibrillation may occur, especially if there has been longstanding volume overload of the left heart, or if other reasons for left atrial dilation are present. Late ventricular arrhythmias and sudden death are a potential risk especially in patients repaired late in life. Complete heart block may also occur after surgical repair. Pregnancy Pregnancy is well tolerated in women with small or moderate VSD and in women with repaired VSDs.

Pregnancy is contraindicated in Eisenmenger syndrome because of high maternal (up to 50%) and foetal (up to 60%) mortality.

VSD with associated lesion

Treatment of double-outlet right ventricle with subpulmonary VSD

Intraventricular repair with rerouting arterial switch operation.

Treatment of VSD and transposition of the great arteries

Treatment is complicated. The Rastelli procedure, Nikaidoh procedure involves complete transfer of aortic root.

Treatment of VSD-associated aortic arch obstruction

Aortic arch reconstruction and VSD repair.

Treatment of VSD with aortic regurgitation

VSD with a large, hemodynamically significant left-to-right shunt, repair of the VSD is indicated, but aortic regurgitation is repaired only if at least moderate aortic regurgitation exists. In the presence of moderate or severe aortic regurgitation, valvuloplasty is preferred to valve replacement.

Heath & Edwards classification of hypertensive pulmonary vascular disease Grade

1 2 3

Pathology
Medial hypertrophy without intimal proliferation Medial hypertrophy with cellular intimal reaction Medial hypertrophy, intimal fibrosis, early generalized vascular dilatation Generalized vascular dilatation, area of vascular occlusion by intimal fibrosis, plexiform lesion Cavernous & angimatoid dilatation lesions

Reversibility
+ + -

Necrotizing arteritis

PVR and surgery

Typically, children without VSD have a PVR of 2 Wood units (2 resistance units or 2 units), indexed for body surface area. If the PVR is greater than 2 units but less than 4 units, pulmonary vascular disease is not present. Patients with a large VSD and a PVR greater than 4 units but less than 8 units have some degree of PVR. If the PVR drops with administration of supplemental oxygen, surgery should be performed. Most of these children do not have elevated pulmonary artery pressure at rest after surgery. However, they do have an increased risk of elevated pulmonary artery pressure during exercise, suggesting an abnormal pulmonary vascular bed. If catheterization reveals a PVR greater than 8 units, vasodilator testing is indicated. If the PVR drops to less than 8 units in response to oxygen or inhaled nitric oxide, surgery should be performed. Some children from this group develop progressive pulmonary vascular obstructive disease. If pulmonary hypertension persists or develops, appropriate pulmonary vasodilator therapy lessens symptoms and prolongs life. In most cases, if the PVR remains above 8 units, even with vasodilator testing, pulmonary vascular disease is severe and progressive. Surgery does not prolong life or improve health in this group and is therefore not indicated.

Surgical technique
CPB with bicaval cannulation, aortic cannulation, root cardioplegic arrested heart. Transatrial approach mostly used Alternative transpulmonary approach for conal VSD, transventricular approach for muscular VSD, transaortic approach for VSD with AR. PTFE/ Dacron patch closure with interrupted/ continuous suturing is preferred, direct closure rarely done.

Outcome
Residual left-to-right shunt from incomplete VSD closure may result from insufficient intraoperative exposure or suture disruption with patch dehiscence. Significant residual shunting is most commonly observed in muscular defects (particularly multiple defects) in which trabeculations decrease visualization of the full extent of the VSD(s). Residual shunting with Qp:Qs greater than 1.5:1 occurs in 2% of patients or fewer and should prompt reoperation. The overall surgical mortality rate for patients with isolated VSD is less than 1%, and the mortality rate for low-risk candidates is miniscule. Risk factors for mortality include severe associated noncardiac anomalies, multiple VSDs, and major associated cardiac anomalies.
Stulak JM et al. Reoperations after repair of partial atrioventricular septal defect: a 45year single-center experience. Ann Thorac Surg. May 2010;89(5):1352-9.

Prognosis
Long-term results of ventricular septal defect (VSD) repair are favourable. In the absence of pulmonary vascular disease, infants who undergo VSD repair within the first 1-2 years of life are considered cured and demonstrate improved physical development (growth and weight gain), as well as normal longterm ventricular function. Most long-term survivors are asymptomatic and lead normal lives. Exercise tolerance may be diminished. If congestive heart failure and cardiomegaly are well established and repair has been undertaken late in life, postoperative symptoms, including exercise intolerance, are more common. Premature late death is rare (< 2.5%) in patients with low preoperative pulmonary vascular resistance. Patients with preoperative pulmonary vascular disease may develop severe, life-threatening pulmonary hypertension.

Patients with the following problems benefit from periodic evaluation by an ACHD cardiologist:

Patch leaks or residual VSDs (which seldom require reoperation). Elevated pulmonary vascular resistance at the time of surgery. Aortic valve surgery. Late repair of moderate or large defects. Significant atrial or ventricular arrhythmias. Associated cardiac lesions (e.g. right ventricular outflow tract obstruction or aortic regurgitation). Endocarditis prophylaxis is recommended for 6 months following VSD closure or for life if any residual defect persists.

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