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Chest pain in Marfan syndrome
  1. Kaushik Sanyal1,
  2. Kanagasabesan Sabanathan2
  1. 1
    Norfolk and Norwich University Hospital Foundation Trust, Department of Critical Care and Anaesthesia, Colney Lane, Norwich NR4 7UY, UK
  2. 2
    Norfolk and Norwich University Hospital Foundation Trust, Department of Medicine, Colney Lane, Norwich, NR4 7UY, UK
  1. Kaushik Sanyal, ksanyal01{at}doctors.org.uk

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A fit, 45-year-old tall man attended the Emergency Department with crushing chest pain for the last 3 h. After swimming, he had developed sudden chest and back pain. He showed physical features of Marfan syndrome such as an increased arm span to height ratio greater than 1.05; additionally, arms and legs may be unusually long in proportion to the torso and arachnodactyly. There was a decrescendo diastolic murmur from aortic regurgitation. There was also an ejection click at the apex followed by a holosystolic high-pitched murmur from mitral prolapse and regurgitation. Electrocardiogram (ECG) was essentially normal with sinus rhythm of 68 beats/min and troponin <0.03 ng/ml.

This abrupt onset of thoracic pain with associated fatigue, pallor and paresthesia of the extremities helped in deciding on an urgent CT scan to rule out any aortic dissection (fig 1). CT of the chest was performed and showed type A aortic dissection of ascending aorta of 6.3 cm. A labetolol infusion was initiated and he underwent emergency surgery with St Jude aortic valve replacement and repair of the dissection.

Figure 1

CT scan showing dissecting aneurysm in a patient with Marfan syndrome experiencing chest pain.

The patient made a good postoperative recovery.

Aortic dissection is a dangerous cardiovascular emergency demanding prompt diagnosis and aggressive management. This abrupt excruciating pain often mimics myocardial ischaemia. Aortic distension, stretch and disruption are responsible for unpleasant chest sensations in the Marfans. Effective management requires high index of suspicion and immediate follow-up investigation. The mortality risk in younger patients is less1 than older ones. Sudden death still occurs, however elective resection of more than 5.5 cm leads to excellent survival.

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Footnotes

  • Competing interests: None.

  • Patient consent: Patient/guardian consent was obtained for publication.

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