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A boot shaped heart

BMJ 2006; 333 doi: https://doi.org/10.1136/sbmj.0611414 (Published 01 November 2006) Cite this as: BMJ 2006;333:0611414
  1. Najindra Maharjan, final year medical student1,
  2. Subarna Mani Acharya, assistant professor2
  1. 1Institute of Medicine, Nepal
  2. 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.

Questions

  • (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?

Answers

  • (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

    • Pulmonary …

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