Primary pulmonary hypertension

Published on 05/05/2015 by admin

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Last modified 05/05/2015

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34 Primary pulmonary hypertension

Instruction

Listen to this patient’s heart.

Salient features

History

• Dyspnoea (60–89%)

• Fatigue (19–73%)

• Syncope, near syncope or dizziness (13–88%)

• Oedema (3–37%)

• Palpitations (5–33%)

• Determine whether the patient is on oral contraceptives, fenfluramine or aminorex (N Engl J Med 1996;335:609–16).

• Determine whether the patient has habitually consumed plant products from Crotalaria species (particularly if from the Caribbean).

• Determine whether there is a family history: the chromosome locus 2q31-q32 has been identified in one familial cohort of primary pulmonary hypertension (Circulation 1997;95:2603–6).

• Determine whether the patient is positive for the human immunodeficiency virus (HIV) (HIV-associated pulmonary hypertension is associated with poor prognosis).

Examination

• Young woman

• Loud pulmonary second sound

• Early diastolic murmur of pulmonary regurgitation best heard on inspiration (Graham Steell murmur)

• Examine the chest for chronic lung disease

• Tell the examiner that you would like to:

• Investigate for a tight or occult mitral stenosis
• Perform a ventilation–perfusion (V/Q) scan to exclude pulmonary emboli.

Diagnosis

This patient has pulmonary hypertension (lesion) and should be investigated for an underlying cause; she is in cardiac failure (functional status) and belongs to WHO class III (see below).

Questions

What are the signs of pulmonary hypertension?

• Large ‘a’ waves in JVP

• Left parasternal heave

• Loud or palpable P2

• Ejection click in the pulmonary area

• Early diastolic murmur (Graham Steell murmur) caused by pulmonary regurgitation.

Advanced-level questions

What is clinical classification of pulmonary hypertension that guides therapy?

National Pulmonary Hypertension Centres of the UK and Ireland Physicians Committee classification (Thorax 2008 63(suppl ii):1–41):

• Pulmonary arterial hypertension: disease-targeted therapies (but caution in venoocclusive disease)

• Pulmonary hypertension with left heart disease: medical, interventional and surgical therapies for chronic heart failure, coronary artery disease, valve disease and pericardial disease

• Pulmonary hypertension associated with lung diseases and/or hypoxaemia: therapy to treat the primary lung disorder, oxygen, disease-targeted therapies when pulmonary hypertension out of proportion to lung disease

• Pulmonary hypertension caused by chronic thrombotic and/or embolic disease: pulmonary endarterectomy (PEA) for proximal disease; disease-targeted therapies for distal disease, significant residual post-PEA pulmonary hypertension or late redevelopment of symptomatic pulmonary hypertension post-PEA

• Miscellaneous: specific to individual diseases.

How would you investigate such a patient?

• Blood investigations include routine haematology and biochemistry, thyroid function, thrombophilia screen in chronic thromboembolic pulmonary hypertension; autoimmune screen (for anti-centromere antibody, anti-SCL70, U1-ribonucleoprotein (U1RNP), anti-phospholipid antibodies, hepatitis serology, serum ACE, HIV.

• Urine for human chorionic gonadotrophin.

• Chest radiograph shows enlarged main pulmonary arteries with reduced peripheral branches, enlargement of the RV.

• Pulmonary function testing includes arterial blood-gas study, 6 min walk test and nocturnal oxygen saturation monitoring.

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