A boot shaped heartBMJ 2006; 333 doi: https://doi.org/10.1136/sbmj.0611414 (Published 01 November 2006) Cite this as: BMJ 2006;333:0611414
- Najindra Maharjan, final year medical student1,
- Subarna Mani Acharya, assistant professor2
- 1Institute of Medicine, Nepal
- 2Department of Medicine, Institute of Medicine, Nepal
An 18 month old female child presented with a history of dyspnoea on exertion. She often gets cyanosed after feeding or crying and sometimes becomes apnoeic. On examination, there was cyanosis and digital clubbing. Auscultation of the heart showed a loud ejection systolic murmur in the pulmonary area. Her chest x ray film is shown in fig 1.
(1) What does the chest x ray film show?
(2) Can you think of a likely diagnosis?
(3) What other investigations can confirm the diagnosis?
(4) What complications could the patient develop?
(5) How would you manage the patient?
(1) The chest x ray film shows a normal sized heart with upturned apex suggestive of right ventricular hypertrophy. The absence of the main pulmonary artery segment gives the cardiac shadow a characteristic appearance often termed as “coeur en sabot” or boot shaped heart.
(2) The most likely diagnosis is tetralogy of Fallot, a congenital condition of the heart, characterised by four features:
Ventricular septal defect, communication between the two ventricles