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Figure 2: Indicators of pulmonary hypertension on computed tomography angiography. A: Pulmonary artery (PA) dilatation, as shown by a diameter exceeding the diameter of the ascending aorta (AO). B: Right ventricular hypertrophy (RVH).

Figure 3: Computed tomography (A) and conventional (B) pulmonary angiogram of a patient with chronic thromboembolic pulmonary hypertension. The arrows show near complete occlusions of the right middle and right lower lobe arteries. Figures reproduced with kind permission from Diagnosis and treatment of pulmonary hypertension. Vonk Noordegraaf A, Boonstra A, Konings TC, Marques KM, Bogaard HJ. Ned Tijdschr Geneeskd 2014;158:A7315.

factor, idiopathic PAH is diagnosed, a condition that most frequently occurs in young women but can also develop in all age groups and males (30% of all patients).21

Before 1997, PAH was

untreatable, with a median survival after diagnosis of about three years. Currently, more than 12 drugs are available for the treatment of PAH and these drugs do improve survival.4,5 Because PAH is a rare disease requiring an elaborate diagnostic approach, including right heart catheterisation and because PAH treatment is complex, European guidelines advocate diagnosis and treatment of patients in expert centres.7,22

Symptoms and signs

Despite recent improvements in PAH treatment, the prognosis of this group of relatively young patients remains poor. Timely referral for lung transplantation is an essential aspect of the management of this disease.


Diagnostic algorithm To prevent unnecessary diagnostic procedures, a diagnostic algorithm for patients with suspected PH is incorporated into the European guidelines for the diagnosis and treatment of PH, which is outlined in Figure 1. Awareness of the possibility of a diagnosis of PH is an important first step to assure timely treatment. The first clue towards a diagnosis of PH is usually found in a patient’s symptoms or signs during physical examination.

An unexplained decrease in exercise tolerance is often the first symptom of the presence of PH. Unfortunately, this symptom is neither sensitive nor specific and frequently the diagnosis of PH is only suspected after symptoms of more severe PH and right heart failure develop, such as palpitations, chest pain, peripheral oedema and syncope. A physical examination may reveal signs of PH, for example, a right parasternal heave, a loud second heart tone and a systolic murmur at the fourth left intercostal space due to tricuspid insufficiency.

Coincidental findings of diagnostic tests It is not uncommon for a diagnosis of PH to be unsuspected until a diagnostic test ordered for other purposes provides specific clues, such as right ventricular strain on the electrocardiogram, cardiac enlargement or pulmonary vessel dilatation on the chest X-ray or CT scan (see Figure 2), or signs of right ventricular pressure overload on the echocardiogram. The latter diagnostic modality allows an estimation of the right ventricular systolic pressure or may provide other hints at a diagnosis of

Screening of groups at increased risk Early recognition of symptoms increases the likelihood of an early diagnosis of PH in patients at risk of this condition. Systematical screening for PH adds to this approach in patients with systemic sclerosis, in which population the prevalence of PAH is about 9%.23,24 Capillaroscopy may be an additional diagnostic modality that allows early recognition of PAH in this patient group.25

Alternatively, the suspicion of PH arises with incidental findings on diagnostic tests or during specific screening efforts.

PH, such as right ventricular hypertrophy and dilatation and paradoxical movement of the interventricular septum towards the left ventricle.

Whether systematic screening is also of value in other populations at risk (for example, healthy carriers of BMPR-II mutations, who have a 30% lifetime risk of PAH development) remains to be determined.

When echocardiography confirms a diagnostic suspicion of PH, the next step is to confirm or exclude the presence of left heart disease and/or chronic lung disease. While pulmonary function testing and arterial blood gas analysis are essential to evaluate the existence of chronic lung disease, echocardiography may provide important clues towards a diagnosis of systolic or diastolic left heart failure or valve abnormalities. Left heart failure can further be suggested by the medical history or by

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