# Acute Rheumatic Fever Complicating Tetralogy of Fallot

Authors: Maneesha Bhaya; Rajesh Beniwal; Raja Babu Panwar
Journal: Scholarly Research Exchange
 (2008)

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## Abstract

This is a unique case of a patient aged 35 years developing rheumatic carditis secondary to acute rheumatic fever. The patient developed acute mitral regurgitation yet tolerated it relatively well because of coexistent Tetralogy of Fallot. The hemodynamics in this patient was significantly altered by the coexistence of these two conditions. This is the first case of its kind when acute rheumatic fever has been documented in a patient of Tetralogy of Fallot.


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## Body

## 1. Introduction

The  prevalence  of rheumatic heart disease (RHD) in India reportedly ranges from 0.5 to 0.67 per 1000 in recent times [1]. RHD in
patients with congenital heart defects is known to occur [2, 3]. Higher
incidence of RHD has been reported in patients with congenital heart disease as
compared to the general population [2, 4]. Tetralogy of Fallot (TOF) is the
commonest cyanotic congenital heart disease encountered in adults [5]. The
aortic and pulmonary valves in a patient of TOF have been reported to be
predisposed to infective endocarditis [6]. There have been reports of mitral
stenosis due to RHD coexisting with TOF [7, 8]. We present a unique case of
acute rheumatic fever with carditis complicating TOF.


## 2. Case Presentation

A thirty-year-old female was referred for evaluation of recently
worsened breathlessness. She had a history of mild breathlessness on exertion
since last ten years. Earlier on, she used to get breathless on strenuous
activity. Up to the past week, she had been unable to perform activities of daily routine. There was no history of fever, joint pain,
any involuntary movements, or any other complaint. General physical examination
revealed small-built individual with mild anemia and fluctuation and softening
of nail bed corresponding to grade I clubbing. The pulse was regular with a rate of 100
beats per minute.The blood pressure was 100/60 mmHg. The apex beat was palpable in the
sixth intercostal space, lateral to the mid-clavicular line. A soft first heart
sound and a single loud second heart sound were heard. A grade III ejection
systolic murmur was heard at the base, and a grade III pansystolic regurgitant murmur was heard at the apex, which radiated
to the axilla. The lungs were clear. There was no hepatic or splenic enlargement.On room air, the patient maintained oxygen saturation of 85%. Chest X-ray
revealed enlarged heart, pulmonary oligemia, and no evidence of pulmonary
venous hypertension. The coeur en sabot typical of TOF was not present.
Electrocardiogram revealed sinus rhythm, increased PR interval [0.24 seconds],
low voltage, and QRS axis of 90 degrees.An echocardiogram revealed a large malaligned ventricular septal defect (VSD)
with 50% aortic override (see Figure1). There was a net right to left shunt.
There was moderate grade, predominantly pulmonary valvular stenosis with peak
gradient across the pulmonary valve being 55 mmHg (see Figure 2). There was no
poststenotic dilatation of pulmonary trunk. The left and right pulmonary
arteries were confluent. There was moderate pericardial effusion without any
evidence of tamponade. The mitral valve was thickened. There was severe mitral
regurgitation with a posteriorly directed jet (see Figure 3). Mitral valve area
was 3.2cm2 as measured by planimetry. The tricuspid and aortic
valve leaflets were also thickened with moderate tricuspid regurgitation and
mild aortic regurgitation (see Figures 4, 5).Figure 1
Parasternal long axis view depicting nonrestrictive, subaortic VSD, thick mitral valve leaflets and pericardial effusion.Figure 2
Parasternal short axis view depicting moderate grade pulmonary stenosis with gradient across the valve as 55 mmHg.Figure 3
Parasternal long axis view depicting nonrestrictive VSD with moderate to severe mitral regurgitation.Figure 4
Apical four-chamber view depicting thick mitral and tricuspid valve and a rim of pericardial fluid.Figure 5
Parasternal SAX view depicting mild aortic regurgitation.A repeat echocardiogram after a few days showed a decrease in the amount
of pericardial fluid. The serum antistreptolysin O titer was elevated above the normal
reference range; C-reactive protein and erythrocyte sedimentation rate were
elevated, and hemoglobin was 12 gm/dl. Blood cultures were negative.The presence of malaligned nonrestrictive VSD with aortic override and
moderate pulmonary stenosis with net right to left shunt confirmed the
diagnosis of TOF although we did not find significant infundibular stenosis.Pericardial effusion with thickening of mitral, aortic, tricuspid
valves, and valvular insufficiency was the evidence of acute rheumatic
carditis.The final diagnosis of Tetralogy of Fallot and rheumatic carditis
secondary to acute rheumatic fever was made.


## 3. Conclusion

This patient fulfilled the Jones criteria of acute rheumatic fever.
Although the occurrence of acute rheumatic fever at this age is unusual, she
may be having a recurrence with the previous episode being unrecognized. An
alternative hypothesis is that TOF predisposed her to the development of acute
rheumatic carditis even at a later age.The occurrence of acute severe mitral regurgitation and mild aortic
regurgitation imposed volume overload on the left ventricle. This was
relatively well tolerated in this patient without the development of overt
pulmonary edema although there was slight elevation in pulmonary artery
pressure with a peak systolic pressure of 45 mmHg (ventricular pressure = 100 mmHg/gradient =
55 mmHg) possibly because of low pulmonary flow situation in a
patient of TOF. However, the occurrence of these lesions would substantially
complicate further management in this patient. The risk of developing infective
endocarditis would substantially increase. Given the multifactorial etiology of
both congenital and rheumatic heart disease, it is quite possible that
congenital heart disease predisposes an individual to the development of
rheumatic heart disease. The present case highlights this association. This is
the first reported case of documented acute rheumatic activity in a patient of
TOF with severe mitral regurgitation, moderate tricuspid regurgitation, mild
aortic regurgitation, and pericardial effusion. We need to think about primary prophylaxis against acute rheumatic fever
in patients with congenital heart disease especially in endemic areas.



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*Source: 541548-2008-11-03.xml*