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Definition of terms
Pulmonary hypertension is defined as a mean pulmonary artery pressure > 25 mm Hg at rest or 30 mm Hg with exercise.
The classification of pulmonary hypertension is linked to the anatomy or the aetiology. These diagnostic categories are for the convenience of deciding on treatment.
Primary pulmonary hypertension refers to pulmonary hypertension for which no cause can be identified. The term remains unchanged from the previous system of classification. Primary pulmonary hypertension is a form of pulmonary arterial hypertension which also includes collagen vascular disease*, congenital systemic to pulmonary shunts*, portal hypertension*, HIV infection*, exposure to various drugs or toxins*, and persistent pulmonary hypertension of the newborn*. Other types of pulmonary hypertension include pulmonary venous hypertension*, pulmonary hypertension associated with disorders of the respiratory system and/or hypoxaemia*, pulmonary hypertension caused by chronic thrombotic and/or embolic disease*, and miscellaneous causes*.
*These hitherto have been called secondary pulmonary hypertension, a term without value for diagnosis and decisions on treatment.
PRESENTATION AND INVESTIGATION
- The symptoms of pulmonary hypertension are relatively non-specific. Breathlessness is the most common.
- Where the diagnosis of pulmonary hypertension is suspected a transthoracic echocardiogram should be performed to screen for pulmonary hypertension.
- Referral of patients to a designated specialist centre should normally be made after an ECG, chest x ray, simple spirometry, and demonstration of pulmonary hypertension by echocardiography, but before cardiac catheterisation. Referral should not be delayed owing to the risk of early death from this condition.
- The diagnosis should be confirmed at right heart catheterisation.
- The aetiology of pulmonary hypertension should be sought in order to determine optimal treatment.
- Acute vasodilator testing should be undertaken at the time of cardiac catheterisation. The response to acute vasodilator testing accurately identifies patients who may respond to long term oral vasodilator treatment.
- All patients with primary or thromboembolic pulmonary hypertension should be treated with warfarin. Warfarin should be seriously considered in other types of pulmonary arterial hypertension where there are no contraindications.
- Vasodilator therapy with oral calcium antagonists may improve symptoms, haemodynamics, and survival in selected patients with pulmonary arterial hypertension who respond to an acute vasodilator test.
- Patients with severe pulmonary hypertension which is primary, familial or caused by anorectic agents, connective tissue diseases, shunts associated with congenital heart disease, portal hypertension, sarcoidosis, HIV or chronic thromboembolic disease (either inoperable or as a bridge to pulmonary thromboendarterectomy) should be considered for long term intravenous infusion of prostaglandins.
- Patients with chronic proximal pulmonary thromboembolic disease should be considered for pulmonary thromboendarterectomy.
- Lung or heart lung transplantation should be considered in selected patients with pulmonary hypertension with disease which is severely symptomatic and progressive despite optimal medical and/or surgical treatment.
- Controlled oxygen therapy may be indicated for those patients with sustained nocturnal hypoxaemia where arterial oxygen saturations are below an average of 90% on air and patients with chronic pulmonary disease associated with hypoxia and pulmonary hypertension.
- Atrial septostomy may be considered in severe pulmonary hypertension refractory to prostaglandin therapy particularly if it is associated with recurrent syncope.
- Women of child bearing age with pulmonary hypertension require contraceptive advice.
- Patients should receive a one-off pneumococcal immunisation and annual immunisation against influenza.
- Patients with pulmonary hypertension require life long monitoring in a specialist centre with the instigation of appropriate therapies as the disease evolves. Submaximal exercise testing is a useful objective assessment since exercise capacity and severity of pulmonary hypertension are correlated. Patients with pulmonary hypertension caused by hypoxia, chronic heart failure, and congenital heart disease will be followed up by other physicians and do not normally require monitoring in a specialist pulmonary hypertension centre.
Primary pulmonary hypertension (PPH) is a rapidly progressive disease with an incidence of 1–2 per million per annum.1 2 It causes disabling symptoms and untreated leads to early death.3 Other causes of pulmonary arterial hypertension may account for a further 1–2 cases per million per annum. Until 1981 when heart–lung transplantation was introduced there was no treatment. Challenged by the limited numbers of suitable donors, medical treatments have been sought, the most successful of which have now postponed the need for transplantation.
The purpose of this paper is to provide guidance for best practice in the management of adults and children following the practice in those centres which routinely investigate these patients. We present a description of the methods for achieving an accurate diagnosis and determining which patients should benefit from medical and surgical treatments. The recommendations were originally devised for patients with PPH, but …
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