A treatable cause of shortness of breathBMJ 2013; 346 doi: http://dx.doi.org/10.1136/bmj.f1937 (Published 15 April 2013) Cite this as: BMJ 2013;346:f1937
- Stella Nikolaou, core surgical trainee 2,
- David P Jenkins, consultant surgeon
- 1Papworth Hospital, Cambridge CB23 3RE
- Correspondence to: S Nikolaou
An 81 year old man was referred to a respiratory doctor with a two year history of worsening shortness of breath on exertion. He also described presyncopal symptoms caused by exertion and one episode of collapse after walking up a hill.
His medical history included benign prostatic hypertrophy and encephalitis. He was a non-smoker, drank little alcohol, and had previously led an active life.
On examination he was afebrile, his blood pressure was 134/84 mm Hg, his heart rate was 80 beats/min in sinus rhythm, and his oxygen saturation on room air was 95%. His jugular venous pressure was not raised. He had a loud P2 component of the second heart sound and no ankle oedema. His lung fields were clear. Electrocardiography showed ST depression and T wave inversion anterolaterally. Chest radiography showed enlarged hila.
An echocardiogram showed normal left ventricular function with a dilated right ventricle and pulmonary artery systolic pressure of 63-68 mm Hg. Computed tomography pulmonary angiography showed a dilated right ventricle, dilation of the pulmonary trunk, multiple webs in pulmonary artery branches, and thrombus layering along the right main pulmonary and right upper lobe pulmonary artery. He was referred to a regional specialist centre to confirm the diagnosis and decide further management.
1 What is the diagnosis?
2 What investigations would you perform in this patient?
3 How should the patient be managed?
4 What are the options for long term management?
1 What is the diagnosis?
This patient has chronic thromboembolic pulmonary hypertension (CTEPH).
Echocardiography is often the first investigation to suggest pulmonary hypertension. It may show dilation of the right ventricle, raised right ventricular systolic pressure, and tricuspid regurgitation. In our patient, computed tomography pulmonary angiography showed a dilated right ventricle, dilation of the pulmonary trunk, multiple webs in pulmonary artery branches, and thrombus layering along the right main …
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