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. 2016 Jul 1;10(7):TE01–TE10. doi: 10.7860/JCDR/2016/21443.8210

[Table/Fig-2]:

Tetralogy of Fallot.

TOF
Components VSD (usually perimembranous) RVOT obstruction (at infundibular, pulmonary valvular or combination) RVH Overriding of aorta
Incidence [13] 1-3 cases per 1000 live births. Most common cyanotic CHD.
Arises from single gene defect involving TBX1 gene (TBX1 gene encodes for a transcriptional factor integral to development of cardiac outflow tracts)
Association [13] Right aortic arch (25%). Abnormal coronary arteries (5%) LAD arising from RCA with prepulmonic course being most common. Complete AVSD (2%) Persistent left SVC
Syndromes CHARGE syndrome Di George syndrome Shprintzen (velo-cardio-facial) syndrome
Imaging features Plain Boot shaped heart (cor en Sabot)= concave MPA segment with an upturned apex Pulmonary oligemia Thymic atrophy Right aortic arch
Imaging features specific Echo defines all components of TOF MRI useful in post-surgical follow-up for RV volume assessment, pulmonary regurgitation quantification and to rule out residual VSD CT evaluation of pulmonary arteries, collaterals and coronary arteries
* McGoon Ratio and Nakata Index are used for quantification of degree of PA hypoplasia
Management [13] Palliative procedures:
• Modified Blalock-Taussig shunt most preferred; placement of Gore-Tex interposition graft between subclavian artery and ipsilateral pulmonary artery Definitive surgery: Total ICR (intracardiac repair) VSD patch closure + widening of RVOT (infundibular tissue resection) + pulmonary valvotomy Note: Presence of coronary anomalies (esp. LAD from RCA with RVOT crossing) is a contraindication for primary repair.