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Tetralogy of Fallot with Restrictive Ventricular Septal Defect

Article  in  The Indian Journal of Pediatrics · November 2017

DOI: 10.1007/s12098-017-2503-1

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Ashish Tiwari Manojkumar K Rohit

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Indian J Pediatr (May 2018) 85(5):400
DOI 10.1007/s12098-017-2503-1

SCIENTIFIC LETTER

Tetralogy of Fallot with Restrictive Ventricular Septal Defect

Ashish Tiwari 1 & Savita Bhunwal 2 & Manojkumar Rohit 1

Received: 11 July 2017 / Accepted: 20 September 2017 / Published online: 20 November 2017
# Dr. K C Chaudhuri Foundation 2017

To the Editor: Tetralogy of Fallot (TOF) is the most common

congenital cyanotic heart disease after first year of life.
Ventricular septal defect is usually unrestricted and
malaligned. Main purpose of this communication is to present
an uncommon case of TOF with heart failure due to restrictive
VSD caused by ventricular septum hypertrophy.
A 6-y-old boy was referred to us with features of right heart
failure, complaints of cyanosis since 6 mo of age and exertional
dyspnea since 5 y of age. Child was managed initially with bed
rest, humidified oxygen and low dose decongestive measures.
Transthoracic echocardiography was diagnostic of TOF (tetral-
ogy of fallot) with a small restrictive perimembranous VSD Fig. 1 Echocardiography showing restrictive VSD
(ventricular septal defect) with hypertrophy of ventricular sep-
tum causing restriction with mild aortic regurgitation (Fig. 1).
After initial stabilization, patient was operated and discharged Table 1 Causes of CCF in TOF
successfully on post-operative day 12.
1. Anemia
In TOF, VSD is usually nonrestrictive. TOF with restrictive 2. Large aortopulmonary collaterals
VSD is a rare entity and is one of the rarest cause of congestive 3. Oversize BT (Blalock-Taussig) shunt surgery
4. Restrictive VSD with suprasystemic RV pressures
cardiac failure in TOF. Cause for the restriction of VSD can be 5. Infective endocarditis
6. Incidental myocarditis
accessory tricuspid valve tissue, tricuspid valve vegetation, pro- 7. Aortic regurgitation
8. Systemic hypertension
lapsed aortic valve and membranous flap and very rarely, caused 9. Coronary artery disease
by hypertrophied ventricular septum. Very limited literature is
available on septal hypertrophy as the cause for restriction of VSD Ventricular septal defect; RV Right ventricular
VSD [1, 2]. One such case was described by Flanagan et al. in Compliance with Ethical Standards
1988 where restriction of VSD was caused by hypertrophied
ventricular septum in one pediatric patient [1]. Table 1 summa- Conflict of Interest None.
rizes the causes of heart failure in TOF. In conclusion, TOF is
rarely associated with restrictive VSD and it is a cause for Source of Funding None.
suprasystemic right ventricular pressure and subsequent right
heart failure.
References
* Ashish Tiwari
aashishtiwari@live.com 1. Flanagan MF, Foran RB, VanPraagh R, Jonas R, Sanders SP. Tetralogy
of Fallot with obstruction of the ventricular septal defect: spectrum of
echocardiographic findings. J Am Coll Cardiol. 1988;11:386–95.
1 2. Faggian G, Frescura C, Thiene G, Bortolotti U, Mazzucco A,
Department of Cardiology, Advance Cardiac Center, PGIMER,
Anderson RH. Accessory tricuspid valve tissue causing obstruction
Chandigarh 160012, India
of the ventricular septal defect tetralogy of Fallot. Br Heart J.
2
Department of Pediatrics, Advance Pediatric Center, PGIMER, 1983;49:324–7.
Chandigarh, India

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