Rare association of tetralogy of Fallot with absent pulmonary valve syndrome with anomalous origin of right pulmonary artery from ascending aorta

Description 

A 2-month-old boy presented to the paediatric cardiology department with cyanosis and feeding difficulties. Chest radiograph revealed cardiomegaly, right-sided aortic knuckle and dilated pulmonary artery segment along with mild indentation on the lower trachea and diffusely narrowed left main bronchus (figure 1A). A diagnosis of tetralogy of Fallot (TOF) with absent pulmonary valve syndrome was made on transthoracic echocardiogram; however, origin of right pulmonary artery (RPA) was not well visualised. The patient further underwent CT angiography (CTA) to delineate the cardiac as well as any extracardiac abnormalities.

Figure 1

Frontal chest radiograph (A) reveals cardiomegaly, right-sided aortic knuckle and dilated pulmonary artery segment along with mild indentation on the lower trachea and diffusely narrowed left main bronchus. Sagittal oblique maximum intensity projection (B) shows presence of features of tetralogy of Fallot, that is, right ventricular hypertrophy, perimembranous ventricular septal defect and pulmonary annular stenosis....

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