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 ::  Abstract
 ::  Introduction
 ::  Material And Method
 ::  Case reports
 ::  Discussion
 ::  Acknowledgement
 ::  References
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Year : 1979  |  Volume : 25  |  Issue : 2  |  Page : 117-120

Isolated aortic stenosis-development of pulmonary hypertension in childhood

Department of Cardiology, K.E.M Hospital, Parel, Bombay-400012, India

Correspondence Address:
J J Dalal
Department of Cardiology, K.E.M Hospital, Parel, Bombay-400012
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Source of Support: None, Conflict of Interest: None

PMID: 501672

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 :: Abstract 

Pulmonary hypertension is uncommon in children with isolat­ed congenital aortic stenosis, and even when present is usually mild. It is primarily due to transmission of the elevated left ventricular end-diastolic pressure through the pulmonary capil­lary circulation and may be then further elevated by reflex vaso­constriction. In some cases the stretching of a patent foremen ovate secondary to elevated left atrial pressure; may lead to a significant left to right shunt which further enhances pulmonary hypertension. This report discusses two cases of isolated aortic stenosis developing pulmonary hypertension in childhood.

How to cite this article:
Dalal J J, Kulkarni H L, Jain A P, Nair K G. Isolated aortic stenosis-development of pulmonary hypertension in childhood. J Postgrad Med 1979;25:117-20

How to cite this URL:
Dalal J J, Kulkarni H L, Jain A P, Nair K G. Isolated aortic stenosis-development of pulmonary hypertension in childhood. J Postgrad Med [serial online] 1979 [cited 2023 Oct 3];25:117-20. Available from:

 :: Introduction Top

There are various causes of left ventri­cular outflow tract obstruction such as valvular and supravalvular aortic stenosis, and fixed and dynamic forms of subvalvular stenosis. [8] Aortic stenosis forms 3% of all congenital heart diseases. [3] Congenital valvular aortic stenosis may be classified as unicuspid, bicuspid, tricuspid, undifferentiated and membrane type, the anatomical structure usually correlating with the age, and commonly bicuspid in these below 15 years. [8]

Pulmonary hypertension secondary to resistance to left ventricular filling may be due to many factors such as systolic hypertension, left ventricular outflow obstructions, coronary artery disease and altered compliance as in patients with cardiomyopathy. [1] Though left ventri­cular failure can raise the pulmonary artery pressure as high as 90 mm Hg. [11] this is uncommon, the rise usually being mild to moderate. This is a report of two children with significant pulmonary hypertension secondary to isolated val­vular aortic stenosis.

 :: Material And Method Top

Two children with severe aortic stenosis and pulmonary hypertension were studied. Electrocardiographic and roentgenographic study was performed in both cases.

Echocardiograms were recorded using a Unirad diagnostic Ultrasound Unit model 902. Recordings were made on a photography film 120 size using a Graflex 197 A, Camera.

Cardiac catheterization data was re­corded with a Sanborn model 760 on a three channel recorder.

 :: Case reports Top

Case 1

A.P., a 5 year old boy presented with a history of grade II dyspnoea and exertional syncope of two years' duration. There was no history of angina or congestive cardiac failure. His physi­cal and mental milestones were normal. There was no history of rheumatic fever.

Examination revealed an averagely built boy with normal facies and a regular pulse rate of 100/min. The pulse upstroke was slow and a carotid thrill was present. His B.P. was 100'70 mm. Hg., in both the upper limbs. There was no cyanosis or clubbing. The apex was located in the 5th left intercostal space just outside the midclavicular line. Auscultation revealed a nor­mal splitting of the second heart sound with a slight accentuation of the pulmonic component. An aortic ejection click followed by a grade 5/6 ejection systolic murmur were present in the aortic area.

The chest X-ray showed a normal cardio­thoracic ratio with no evidence of post-stenotic aortic dilatation. There was evidence of pulmo­nary venous congestion. His ECG showed an axis of + 70° with biventricular hypertrophy and T wave inversions in V5 and V6. His vector­cardiogram showed a frontal plane QRS axis of + 80° with a clockwise loop. The horizontal plane showed a type C loop. Echocardiography revealed a normal mitral valve motion. The left ventricular cavity size was normal but there was a marked thickening of the left ventricular posterior wall. The aortic valve closure line was eccentric with full opening of the leaflets and no evidence of calcification.

Cardiac catheterization data are depicted in [Table 1]. The transventricular gradient. recordings are shown in [Figure 1] (See on page 11GB). Aortic root angiography revealed a deformed aortic valve with no evidence of aortic incompetence (See [Figure 2] on page 116B).

At surgery a deformed bicuspid severely stenosed aortic valve was found. The opening was enlarged by splitting the function for a small distance along the commissural line.

Case 2

K.B., a 12 year old boy was admitted with a history of exertional dyspnoea, angina and exertional palpitations of 3 years' duration. There was no history of syncope or congestive cardiac failure. There was no past history of rheumatic fever.

On examination, the patient was small built with a pulse rate of 90/min, and a blood pres­sure of 90/60 mm.Hg, in both the upper limbs. There was no cyanosis or clubbing. Cardiovas­cular examination revealed the apex beat in the 6th left intercostal space in the anterior axillary line. A mild right ventricular heave and a basal systolic thrill were present. On ausculta­tion, the second sound was normally split with an accentuation of the pulmonary component. An aortic ejection click followed by a grade 4/6 ejection systolic murmur were present in the aortic area and conducted to the carotids.

His X-ray showed a cardiac enlargement (cardiothoracic ratio 65%) with the presence of pulmonary venous congestion. His ECG showed an axis of + 90° with evidence of biventri­cular hypertrophy (See [Figure 3] on page 116B) . The echocardiogram showed a significant en­largement of the left ventricle (systolic dimen­sion - 4.0 cm., diastolic dimension = 5.1 cm.). The aortic valve was not calcified and the closure line was central. Cardiac catheteriza­tion data are depicted in [Table 1].

At surgery a severely Stenosed tricuspid aortic valve was present. The obstruction was relieved by slitting open two of the three com­missural fusions.

 :: Discussion Top

The presence of isolated aortic valvular stenosis in childhood favours the aetio­logical factor to be congenital rather than rheumatic. [9] The fact that many of these valves are structurally abnormal further stresses this possibility. Echocardio­graphy has been helpful in non-invasive­ly detecting these abnormalities, [6] as in case 2, where the eccentric aortic valve closure line suggested a bicuspid valve. This echocardiographic feature is not diagnostic, [7] but in the present case the bicuspidity was confirmed at surgery.

Braunwald, [2] in a study of hundred cases of congenital aortic stenosis found a 16% incidence of pulmonary - hyperten­sion and in only four of these was the pulmonary hypertension more than mild, leading him to conclude that significant pulmonary hypertension in isolated aortic stenosis signified very severe obstruction or an associated intracardiac shunt.

The pulmonary hypertension in patients with isolated aortic stenosis is due to retrograde transmission of the elevated pulmonary venous pressure across the capillary bed and reflects the elevated left ventricular end-diaistolic pressure. [1],[4] In both cases in the present study, cardiac catheterization has re­vealed s: vere critical aortic stenosis [5] and marked elevation of left ventricular end­diastolic pressures. The raised left ven­tricular end-diastolic pressure generally results from left ventricular failure, but may also be due to reduced ventricular compliance [4] Because of the small differ­ence in the pressures between the pul­monary artery and the left atrium which normally averages only 6 to 9 mm Hg., an elevation of the left atrial pressure causes an equal or slightly greater in­crease of pulmonary artery pressure. The occasional further rise of pulmonary artery pressure, as present in both the cases in this study, is suggestive of a re­flex vasoconstrictive element and results. in an increase in the gradient between the pulmonary arterial and the left atrial pressures. [12] In constrictive pericarditis, and restrictive cardiomyopathies, there is transmission of the elevated pulmonary venous pressure, but rarely a vasocon­strictive element and the gradient across the capillary bed is normal or only modestly increased. [4] In children with severe aortic stenosis, the elevated left atrial pressure stretches the patent foramen ovale and results in a left to right shunt which may be as large as 3.5 to 1. [10] This results in further enhancement of pulmonary arterial pressure. The over­all rise of pulmonary artery pressure in patients with aortic stenosis is therefore due to direct transmission of the elevated pulmonary venous pressure, a left to right atrial shunt and hypoxie pulmo­nary vasoconstriction secondary to pul­monary congestion.

The absence of any atrial shunting in both our cases showed that the high pul­monary artery pressure was predominent­ly based on the transmission of the high left ventricular end-diastolic pressure and reflex vasoconstriction.

 :: Acknowledgement Top

We are thankful to Dr. C. K. Desh­pande, Dean, K.E.M. hospital for allow­ing us to publish this material.

 :: References Top

1.Blount, S. G. and Grover, R. F.: Pul­monary hypertension. In "The Heart,Arteries and Veins", Edited by Hurst W. J., 3rd Ed., McGraw Hill Inc., New York and London, 1974, p. 1248.  Back to cited text no. 1    
2.Braunwald, E., Goldblatt, A., Aygen, M. M., Rockoff, S. D. and Morrow, A. G.: Congenital aortic stenosis. I. Clinical and hemodynamic findings in 100 patients. II. Surgical treatment and the results of operation, Circulation, 27: 426-462, 1963.  Back to cited text no. 2    
3.Campbell, M. and Krauntze, R.: Con­genital aortic valvular stenosis, Brit. Heart J., 15: 179-194, 1953.  Back to cited text no. 3    
4.Fowler, N.: Pulmonary hypertension. Chapter 40, In, "Cardiac Diagnosis and Treatment, 2nd Ed., Harper and Row, Maryland, New York, San Francisco and London, 1976, p. 792.  Back to cited text no. 4    
5.Friedman, W. F. and Papelbaum, S. J.: Indications for hemodynamic evaluation and surgery in congenital aortic stenosis, Paed. Clin. N. Amer., 18: 1207-1223, 1971.  Back to cited text no. 5    
6.Nanda, N. C., Gramiak, R., Manning, J., Mahoney, E. B., Lipchick, E. C. and Deweese, J. A.: Echocardiographic re­cognition of the congenital bicuspid aortic valve, Circulation, 49: 870-875, 1974.  Back to cited text no. 6    
7.Radford, D. J., Bloom, K. R., Izukawa,T., Moes, C. A. F. and Rowe, R. D.: Echocardiographic assessment of bicuspid aortic valve-Angiographic and patho­logical correlates, Circulation, 53: 80, 85, 1976.  Back to cited text no. 7    
8.Roberts, W. C.: Left ventricular outflow tract obstruction and aortic regurgitation. Chapter 7, In "The Heart" Edited by Edward, J. C., Lev, 1% , t, and Abell, M. R., The Williams and Wilkins Company, Baltimore, 1974, p. 110.  Back to cited text no. 8    
9.Roberts, W. C.: Anatomically isolated ao tic valvular disease, the case against it being of rheumatic etiology, Amer. J. Med., 49: 151-159, 1970.  Back to cited text no. 9    
10.Rudolf, A. M.: Aortic stenosis, Chapter 9, In "The Congenital Disease of the Heart", Year Book Medical Publisher, Chicago, 1974, p. 296.  Back to cited text no. 10    
11.Selzer, A. and McCaughey, D. J.: Hemo­dynamic patterns in chronic cardiac failure, Amer. J. Med., 28: 337-346, 1960.  Back to cited text no. 11    
12.Si'ove, E. D., Travernor, W. D. and Berry, C. C.: Reactive pulmonary arterial hypertension after pulmonary venous con­striction in the calf, Cardiovasc. Res., 6:36-44, 1972.  Back to cited text no. 12    


  [Figure 1], [Figure 2], [Figure 3]

  [Table 1]


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