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Rare association of tetralogy of Fallot with absent pulmonary valve syndrome with anomalous origin of right pulmonary artery from ascending aorta
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  1. Niraj Nirmal Pandey,
  2. Arun Sharma,
  3. Sanjeev Kumar
  1. Department of Cardiovascular Radiology and Endovascular Interventions, All India Institute of Medical Sciences, New Delhi, Delhi, India
  1. Correspondence to Dr Arun Sharma, drarungautam{at}gmail.com

http://dx.doi.org/10.1136/bcr-2018-227008

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    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. Pulmonary valve leaflets are not seen with aneurysmal dilatation of the main and left pulmonary artery (LPA). Coronal oblique maximum intensity projection (C) shows right pulmonary artery (RPA) arising from the mid-ascending aorta. Sagittal multiplanar reconstruction (D) reveals collapse (*) of …

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