O R I G I N A L A R T I C L E S Indian J Pediatr 1993; 60 : 777-782

Clinical Course of Isolated Ventricular Septal Defect : An Indian Experience

Anita Saxena, Rajendra Tandon and Savitri Shrivastava

Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi

Abstract. To define the clinical course of ventricular septal defect, 410 consecutive patients with =solated ventricular septal defect were evaluated over a period of 13 years. Their age ranged from 12 days to 24 years at the time of first visit to the hospital. Patients with less than 2 years follow-up period were excluded. One hundred and fifty seven patients were one year of age or less. The left to right shunt size remained the same in 52.4% of cases. In 34.4% the shunt size decreased, with complete closure of ventricular septal defect in 8.8%. Closure of ventricular septal defect was ob- served even in patients who had initially presented with large left to right flow, and congestive heart failure in infancy. Right ventricular outflow tract obstruction developed in 8.5% of patients usually between 2 and 10 years of age. Murmur of aortic regurgitation appeared in 8.g% on follow-up. In- fective endocarditis developed in 6 cases. The unfortunate complication of Eisemenger's complex was seen in 3 patients; they had not returned for follow up for a long period of time.

Hence, our data show that the left to right shunt across the ventricular septal defect decreases in about one-third of patients. However, a regular follow up is essential to prevent development of Eisenmenger's complex and for early detection of other complications like aortic regurgitation and right ventricutar outflow tract obstruction. (Indian J Pediatr 1993; 60 ; 777-782)

Key words : VentricuIar sept al defect; Pulmonary stenosis, Aortic regurgitation; Infective endocarditis

Ventricular septal defect is one of the commonest congenital cardiac lesions at birth, accounting for 2/1000 live births and 0.9/1000 school age children. 1 However, it is less commonly diagnosed during adult life. The change in frequency is related to the natural history of the anomaly. Spontaneous closure of ventricular septal defect, physiological transformation into tetralogy of Fallot or Eisenmenger's complex, and development of aortic regurgitation or surgical intervention are some of the changes that may occur in patients born with isolated ventricular

Reprint requests: Dr. Anita Saxena, Associate Professor, Cardiothoracic Centre, All India Institute of Medical Sciences, Ansari Nagar, New Delhi-110 029.

septal defect. The present study was undertaken to evaluate the clinical course of patients with ventricular septal defect during a follow up period of two to 13 years.

MATERIAL AND METHODS

All patients with an isolated ventricular septal defect attending the cardiac clinic were included in the study. Patients pre- senting with Eisenrnenger's complex or associated with lesions like aortic stenosis, pulmonary stenosis or aortic regurgitation at the initial visit were excluded. Patients who had a follow-up period of less than two years, or had undergone any cardiac surgical intervention were excluded from

778 THE INDIAN JOURNAL OF PEDIATRICS 1993; Vol. 60. No 6

the study. There were 410 patients, ranging in age

from 12 days to 24 years at the time of first visit between the years 1973 to 1983. Of the 410 cases, 270 were male and 140 were fe- male patients. Each patient was assessed clinically by at least two pediatric cardiolo- gists. The thoracic roentgenogram and the electrocardiogram was available in every case. Of the 410 cases, 118 cases were sub- jected to cardiac catheterization and angio- cardiography as a part of pre-operative work up. At each visit to the cardiac clinic, patients were carefully screened for any change in physical findings and for evi- dence of infective endocarditis. The tho- racic roentgenogram and the electrocardio- gram were re-evaluated during the follow- up visits periodically.

The patients were divided into two groups. Group I included 204 patients with a left to right shunt ratio of more than 2 : 1

as determined by the presence of a widely split second sound, cardiomegaly and dia- stolic flow murmur at the apex. Group II consisted of 206 cases where the pulmo- nary to systemic flow ratio was assessed to be less than 2 : 1 without pulmonary arte- rial hypertension or pulmonic stenosis. This was further supported by a normal heart size on chest X-ray and a normal electrocardiogram. Each group was further divided into two subgroups depending on the age at initial presentation (group a _~ 1 year and group b > I year).

RESULTS

Follow up over 2-13 years is summarized in Table 1. In 215 of 410 patients (52.4%), the shunt size remained the same. However, in 141 cases (34.4%) the shunt size decreased with complete closure of the ventricular septal defect in 36 (8.8%). In

TABLE 1. Clinical Profile and Follow-up Data

Follow-up Group I Group II (shunt 2 : 1) (shunt 2 : 1)

(n=204) (n=206)

Ia Ib IIa IIb (age -<1 year (age > 1 year) (age _~ 1 year) (age > 1 year)

(n=110) (n=94) (n=47) (n=159)

Shunt size Same 36 51 22 106 Smaller 44 29 7 25 Closed 8 2 12 14

RVOT Obstruction 17 7 5 6 Aortic regurgitation (AR) 2 4 1 5 Infective endocarditis (IE) 1 1 0 4

Eisenmenger complex 2 1 0 0

One patient each in group Ib and Ilb had both IE and AR, and one patient in group IIb had AR and RVOT obstruction. RVOT-Right ventricular outflow tract.

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83 of these 141 patients, the initial left to right shunt was assessed to be more than 2

1. Spontaneous closure of ventricular septal defect also occurred in five patients who had presented with large left to right flow and congestive heart failure in infancy. Right ventricular outflow tract obstruction developed in 35 cases (8.5%), and in none of these a right aortic arch could be identified on chest x-ray film. Aortic incompetence developed in 12 cases (2.9%). In two of these, aortic regurgitation followed an episode of infective endocarditis over a bicuspid nonobstructive aortic valve. Including these two patients, infective endocarditis was seen in 6 cases. Three cases who developed the unfortunate complication of El~evrnenger complex, had not returned for follow-up after their initial visit for long oeriod of time (5 to 11 years), and when presented they had already undergone Eisenmenger reaction.

Analysing the data further (Table 2), it was seen that out of 36 patients in whom ventricular septal defect closed spontane- ously, 19 closed by five years of age and closure was observed only in one patient

beyond 10 years. Right ventricular outflow obstruction developed in only one patient before the age of one year, the majority de- veloped it between two to ten years. Simi- larly none of the patients developed aortic regurgitation below three years of age, while eight out of 12 cases developed aor- tic regurgitation five years of age. Cardiac catheterization and angiocardiogram was done in seven cases with aortic regurgita- tion. A subpulmonlc ventricular septal de- fect was found in two of the seven cases, in the remaining five cases the ventricular septal defect was perimembranous. Aortic valve prolapse was the cause of aortic re- gurgitation in two patients.

D I S C U S S I O N

Ventricular septal defect is of considerable practical significance as it is tile commonest intracardiac malformation if bicuspid aortic valve and mitral valve prolapse are excluded. 2~ There are many studies on the natural history of ventricular septal defect, especially in infancy and early childhood. In our study, 61.7% of patients were over one year of age at the time of first presentation.

TABLE2. Course of Ventricular Septal Defect in Relation to Age

Age Closure RVOT AR Eisenmenger IE (yr) (n=36) obstruction (n=12) reaction (n=6)

(n=35) (n=3)

< 1 4 1 - -

1-3 10 15 - 1 4-5 5 8 2 -

6-7 4 5 4 1 8-10 11 2 4 1 1 > 10 2 4 2 2 3

Abbreviations as in Table 1.

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Decrease in Size of Left to Right Shunt

The spontaneous decrease in the size of the ventricular septal defect with or without complete closure is a ve ry important as- pect of its natural history. However, the frequency reported in literature has varied depending upon the age of population studied, follow-up period and the type of ventricular septal defect. The various fig- ures reported are 33%, 1 26%, 3 35%, 4 and 21%. 5 The incidence of isolated ventricular septal defect is very low in adult autopsy series, again conforming to the fact that majority of ventricular septal defects either come to notice in early life or close by adult life. In our study, the shunt size de- creased in 34.6% of cases with complete closure in 8.8%. This low incidence of spontaneous closure may be related to the higher age at initial presentation in our study, as chances of closure are maximum in early life.

Another interesting feature of ventricu- lar septal defect closure is that even large ventricular septal defects are known to close completely. In our data, 10 out of 34 (29.4%) cases with VSD closure had an ini- tial shunt size of more than 2 : 1, and five of these had presented in infancy with con- gestive heart failure. This has been re- ported earlier in a series, 6 where 20% of moderate sized ventricular septal defect, who presented in congestive heart failure, ultimately progressed to closure in first few years of life.

Right Ventricular Outflow Tract Obstruc- tion

The incidence of right ventricular outflow tract obstruction has varied from 3-7% in large series, s,7 It is rare in childhood. We found right ventricular outflow tract

1993; Vol. 60. No. 6

obstruction developing in 35 (8.5%) of our ventricular septal defect cases during follow-up, and in 25 cases it was sufficiently severe to reverse shunt, producing cyanosis. According to Verghese et al, s development of right ventricular hyper t rophy in electro- cardiogram and right aortic arch in chest skiagram are clues to the possible development of right ventricular outflow tract obstruction. In our experience, change in character of second heart sound along with development of right ventricular hypertrophy in ECG were indicative of development of right ventricular outflow tract obstruction. In none of the cases, was there evidence of right sided aortic arch on chest x-ray.

Aortic Regurgitation

Deformities of the aortic valve are present in almost 25% of patients having retrograde aortograms, in association with ventricular septal defect, but in most instances the degree of change is trivial. 6 The incidence of development of aortic regurgitation in the literature has been reported to vary from 5% - 6.6%. s'9'1~ According to Corone et al, 5 aortic regurgitation was never found below two years of age, rare between two and four years and was most often seen in children between five and nine years. Halloran t~ also corroborated these findings. In our series, aortic regurgitation developed in 2.9% of ventricular septal defect cases on follow-up, all of whom were over three years of age. In keeping with previous reports in the literature, we also found that in two cases, aortic regurgitation developed secondary to infective endocarditis.

1993; Vol. 60. No. 6

Infective Endocardit is

Six of our 410 cases (1.4%) were compli- cated by infective endocarditis, their age ranged from three years to 28 years. The :ncidence reported in literature has varied from 1.3 to 2.4 for 1000 patients years, s,n,12 The absence of infective endocarditis un- der age of two years is explained by the presence of few teeth of primary dentition, and the reduced risk of the oral cavity (which is the commonest portal of entry) acting as the portal of entry.

Pulmonary Vascular Obstructive Disease

The relatively low incidence of Eisen- menger complex in this series, as well as d,ose reported in the literature relate to the fact that most of patients with large ven- tr;cular septal defect and pulmonary arte- rial hypertension have been operated at a fairly young age. We found that all the three cases who developed Eisenmenger complex had not come for follow-up for a long intervening period. Hence, in the natural history of ventricutar septal defect, the incidence of Eisenmenger will be very different if cases with large ventricular septal defect were allowed to evolve for long periods without any intervention.

A regular follow up of patients with ventricular septal defect is mandatory to prevent deve lopmen t of Elsenmenger complex, and for ear ly detect ion of development of aortic regurgitation or right ventricular tract obstruction. In our experience, left to r ight shunt size decreased in about one third of cases with complete closure in some of these. Infective endocardi t i s is a potential complication even in patients with small ventricular septal defect.

"[HE hNDIAN JOURNAL OF PEDIATRICS 781

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1. Hoffman JIE. Natural history of congeni- tal heart disease. Problems in its assess- ment with special reference to ventricular ~eptal defects. Circulation 1968; 37 : 97- 125.

2. Anderson RH, Macartney FJ, Shineboume et al. Ventricular septal defect. In :

Paediatric Cardiology. London : Churchill Livingstone, 1987 : 615.

3. Keith JD. Prevalence, incidence and epi- demi~iogy. In : Keith JD, Rowe RD, Vlad P, eds. Heart Disease in Infancy and Child- hood. New York : Macmillan. 1978 : 4-352.

4. Mitchell SC, Korones SB, Berendes h'W. Congenital heart disease in 56,109 births. Incidence and natural history. Circulation 1971; 43 : 323-332.

5. Corone P, Doyon F, Gaudeau S e t al. Natural history of ventricular septal defect. A study involving 790 cases. Circulation 1977; 55 : 908-915.

6. Rowe RD, Freedom RM, Fowler RS et al. Natural and unnatural history of congenital heart disease-ventricular septal defect. In : Godman MJ, ed. Paediatric Cardiology, Vol. 4. London : Churchill Livingstone, 1981 : 386 -396.

7. Weidrnan WH, Blount Jr SG, DuShane JW et al. Clinical course in ventricular septal defect. Circulation 1977; 56 (Supplement I) : 56-69.

8. Verghese PJ, Allen JR, Rosenquist GC et al. Natural history of ventricular septal defect with right sided aortic arch. Br Heart J 1970; 32 : 537-546.

9. Plauth WH, Braunwald E, Rockoff SD et al. Ventricular septal defect and aortic re- gurgitation. Clinical, hemodynamic and surgical considerations. Am J Med 1965; 39 : 552-567.

10. Halloran KH, Talner NS, Browne MJ. A study of ventricular septal defect associ- ated with aortic insufficiency. Am Heart J 1965; 69 : 320-326.

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1i. Shah P, Singh WSA, Rose V et al. Inci- dence of bacterial endocarditis in ven- tricular septal defects. Circulation 1966; 34 : 127-131.

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12. Keith JD, Rose V, Collins Ge t al. Ventricu- lar septal defect. Incidence, morbidity and mortality in various age groups. Br Heart J 1971; 33 (supplement) : 81-87.

MALNUTRITION: THE INVISIBLE COMPROMISE

Malnutrition is shocking in both scale and severity; a stealthy accomplice of poverty, it stunts the mental and physical growth of one in three children in the developing world.

Only 1 or 2% of the world's children exhibit visible signs of malnutrition. But an estimated 190 million children under age five are chronically malnourished, locked early into a pattern of ill health and poor development.

Specific problems such as low birth weight and specific practices such as bottle-feeding contribute heavily to malnutrition. Its principal cause, however, is the constellation of diseases, especially diarrhoea, that strives in poor communities lacking clean water and sanitation. Chronic illness drains nutrients from the body and its cells.

Malnutrition strikes hardest in the last trimester of pregnancy and during the first 12 months after birth.

The most severe effects of stuntingt are concentrated before a child's first birthday. Even if nutrition improves thereafter, the child is likely to suffer from below-normal growth, affecting physical and mental development and compromising the future of children and their nations.

Abstracted from :

UNICEF, The State of the World's Children 1994; 16.