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Cyanosis in late teens
  1. S G Williams,
  2. D J Wright,
  3. U M Sivananthan
  1. Institute for Cardiovascular Research, Yorkshire Heart Centre, Jubilee Building, Leeds General Infirmary, Leeds LS1 3EX, UK
  1. Dr Williamssimongwilliams{at}hotmail.com

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An 18 year old youth presented with a three year history of decreasing exercise tolerance and cyanosis. Physical examination revealed clubbing and cyanosis, with an estimated oxygen saturation of 76% (on air). There were no other abnormalities on cardiovascular or respiratory examination. Arterial blood gas estimation revealed a Po 2 of 5.85 kPa breathing air, increasing only to 6.8 kPa after 10 minutes on 100% oxygen. Haemoglobin was 22 g/dl. ECG, chest x ray, and conventional echocardiography were normal.

A transoesophageal echocardiogram is shown in fig1.

Figure 1

Transoesophageal echocardiogram. Ao, aorta; LA, left atrium; MPA, main pulmonary artery; RPA, right pulmonary artery.

Questions

(1)
What is the most likely cause of the cyanosis?
(2)
What does the transoesophageal echocardiogram show?
(3)
What treatment options are available for this patient?

View Abstract

Footnotes

  • Answers on p 56.

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