Case 1: Young woman with dyspnoea

A 23 year old lady presented with progressive worsening of shortness of breath on exertion and fatigue. Initially, she was able to walk three blocks but now she is struggling to walk one block and is confined to her own house. At presentation, she has a dry cough and has no fevers, night sweats or weight loss or ankle swelling.

She has no recent travel history, does not take illicit drugs and is a non-smoker. She had not come into contact with anyone with infection, and does not have any family history of asthma, hayfever or cancer. She has no exposure to pets and has no allergies.

On examination, she appeared to be in slight respiratory distress. Normal blood pressure and pulse with reduced oxygen saturation that was corrected with 2L oxygen. Cardiovascular examination revealed L parasternal heave, and a loud second heart sound with a positive Kussmaul's sign. Lung fields were clear.

Blood tests were unremarkable. Chest radiograph (illustrated) showed a prominent R pulmonary artery segment with tapering of distal vessels on the left. ECG showed a right bundle branch block with right axis deviation and atrial enlargement. Spirometry tests were normal that excluded significant lung parenchymal disease.

What are the likely diagnoses?



















http://www.med.yale.edu/intmed/cardio/imaging/cases/hypertension/index.html


In this scenario, the young age of the patient and the clinical presentation suggested the following disorders:

• Pulmonary embolism - always need to exclude this as a cause of acute dyspnoea. A CTPA scan performed subsequently excluded emboli in the major vessels.
• Pulmonary hypertension - this is probably the most likely given the clinical findings. Pulmonary arterial pressure can be measured with R heart catheterisation. The normal pressure in the pulmonary artery is around 12-15 mmHg, and hypertension when the pressure exceeds 25mm Hg.

According to the WHO, pulmonary hypertension has been classified as follows:

• Pulmonary arterial hypertension
• Pulmonary venous hypertension (ie L heart failure)
• Hypertension secondary to hypoxaemia
• Thromboembolic hypertension (ie PE)
• Miscellaneous hypertensive disorders

The treatment of pulmonary arterial hypertension is usually with vasodilators, such as nitric oxide, calcium channel blockers, sildenafil (aka Viagra), prostanoids and the endothelin-receptor antagonist bosenta. One must note that there is a rare variant where these treatments can potentially worsen the condition (pulmonary veno-occlusive disease). Failing all these, the only other potential option is double lung transplant.