TUGAS BACA

Natural History VSD

  Patients may remain asymptomatic throughout their lives with small or moderate defect.
 The defect may close or decrease in size. The clinical evidence of decrease in size is associated
by relief of symptoms, decrease cardiac size in roentgenography and regression of LVH in
ECG.
 During infancy or childhood about 5 percent of patients of moderate to large VSD may
develop RV infundibular stenosis resembling TOF in later life, which is known as Gasul’s
phenomenon.
 CHF occurs with large defect, common in infancy.
 In some cases the VSD size may increase as the child grows, leading to worsening of
hemodynamic parameters.

Clinical Diagnosis of CHD - M Satpathy

  Pulmonary hypertension and pulmonary vascular disease may develop leading to
Eisenmenger’s complex.
 Aortic regurgitation may occur between 2 to 10 years of age in about 5 percent of cases
particularly in a setting of high VSD (outlet type), due to improper support of one or more of
the aortic cusps (usually right coronary cusp).
 Some patients of large VSD develop different types of arrhythmias in course of time.
 Infective endocarditis is one of the principal hazards and life threatening complication for all
types of VSD.

Clinical Diagnosis of CHD - M Satpathy

  The natural history of the patient with VSD has a wide spectrum, ranging from spontaneous
closure to CHF to death in early infancy. The natural history is in uenced by its position, size,
number of defects and association of other malformations. Small defects remain asymptomatic
but are predisposed to endocarditis and AR. Large defects often develop LV failure, PH
(Eisenmenger syndrome) and eventually RV failure. Thus, in the natural history of VSDs there
may be:
1. Spontaneous diminution in size or closure.
2. Development of right ventricular outflow tract obstruction (Gasul’s effect).
3. Development of AR.
4. Development of left ventricular out ow tract obstruction.
5. Development of pulmonary vascular obstructive disease.
6. Infective endocarditis.
A Comprehensive Approach to Congenital Heart Diseases, IB Vijayalakshmi
 SPONTANEOUS CLOSURE OF VSD

 The incidence of spontaneous closure in perimembranous and muscular VSDs is high, while it
is low in outlet defects and inlet defects do not close.
 Studies have documented that the spontaneous closure within the rst year is signi cantly higher
for muscular than for perimembranous defects.
 In patients with restrictive VSDs followed up from birth, there is a higher incidence of
spontaneous closure (50-75 percent).
 The incidence of spontaneous closure in moderate and large VSDs is only 5 to 10 percent.

A Comprehensive Approach to Congenital Heart Diseases, IB Vijayalakshmi

  Small perimembranous VSD can close by various methods:

1. The adherence of the septal leaflet of TV to the IVS causing an aneurysm-like pouch. This
can partially or completely close the defect, but this is at the cost of causing tricuspid
regurgitation (TR).
2. The in growth of fibrous tissue with endocardial proliferation causing septal aneurysm
3. Prolapse of the aortic cusp especially the noncoronary or the right coronary cusp, through
the defect can close the VSD at the cost of causing AR.
4. Growth and hypertrophy of the muscular portion of the septum around the defect.
5. The vegetation caused by bacterial endocarditis on the RV side of the VSD, but this is at the
cost of infection

A Comprehensive Approach to Congenital Heart Diseases, IB Vijayalakshmi

 RIGHT VENTRICULAR OUTFLOW
OBSTRUCTION
 Right ventricular outflow obstruction occurs secondary to VSD in 3 to 7 percent.
 Gasul et al. were the rst to suggest that large VSD can over a variable period develop
hypertrophy of the crista supraventricularis leading to signi cant infundibular obstruction.
 There may be no clinical evidence of the infundibular stenosis in many infants, but it can be
documented on catheterization and on echocardiography.

A Comprehensive Approach to Congenital Heart Diseases, IB Vijayalakshmi

 AORTIC REGURGITATION

 The incidence of aortic cuspal prolapse in outlet VSDs has been shown to be as high as 73%.
 In perimembranous VSDs, aortic cuspal prolapse has been shown to be 14% with progression
to AR in 6%.
 In early systole, blood is ejected from the LV and is also shunted through the VSD. The
anatomically unsupported coronary cusp and aortic sinus are driven into the RV due to the
Venturi effect. The Venturi effect is caused by the high velocity jet passing through the small
VSD causing negative pressure.
 In diastole the intra-aortic pressure forces the aortic valve lea et to close, but the unsupported
cusp (right or noncoronary) is pushed down into the left ventricular out ow tract away from
the opposed coronary cusp, resulting in AR

A Comprehensive Approach to Congenital Heart Diseases, IB Vijayalakshmi

 PULMONARY VASCULAR OBSTRUCTIVE
DISEASE
 Pulmonary vascular obstructive disease may develop in 10 percent of the large VSDs.
 In patients with pulmonary artery systolic pressure >50 percent of the systemic arterial systolic
pressure, there is signi cant risk for the development of pulmonary vascular changes.
 If untreated these large or non-restrictive VSDs will have a progressive rise in pulmonary
artery pressure and a fall in left to right shunting. In turn, eventually this leads to higher PVR
and to Eisenmenger syndrome.

A Comprehensive Approach to Congenital Heart Diseases, IB Vijayalakshmi

 INFECTIVE ENDOCARDITIS

 Infective endocarditis (IE) is an uncommon risk occurring in <1 to 3 percent of patients with
VSD.
 A small perimembranous VSD that does not close spontaneously is generally associated with a
good prognosis, but is at risk for development of IE.
 The vegetation is usually located on the septal tricuspid leaflet at the site of impact of the jet.

A Comprehensive Approach to Congenital Heart Diseases, IB Vijayalakshmi

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