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Differentiating PH: it’s not all PAH

This article provides an overview of the differentiation, diagnosis and treatment of PH, acknowledging the recent publication of the 2015 Guidelines for the diagnosis and treatment of this condition

Harm Jan Bogaard MD PhD FAHA Anton Vonk Noordegraaf MD PhD FCCP Department of Pulmonary Medicine Institute for Cardiovascular Research VU University Medical Center Amsterdam, The Netherlands

While most doctors encounter patients with high pressure in the systemic circulation on a regular basis, they will much less frequently diagnose a patient with pulmonary hypertension (PH). The most serious form of this condition, pulmonary arterial hypertension (PAH), was not described until the 1950s.1

In the past two

decades, new, effective therapies have become available and early treatment of PAH patients has improved their survival.2–5

This chapter provides an 8

overview of the diagnosis and treatment of PH, acknowledging the recent publication of the 2015 Guidelines for the diagnosis and treatment of PH.6 The normal physiology of the pulmonary circulation is characterised by low vascular resistance and low pressure in the pulmonary arteries of around 12mmHg. PH is defined as mean pulmonary artery pressure ≥ 25mmHg. Symptoms of PH are a consequence of a compromised function of the right ventricle of the heart and primarily consist of a decreased exercise tolerance. In later stages of the disease, increased venous pressure results in ascites and ankle oedema. Other symptoms of patients with PH are fatigue, shortness of breath, chest pain and palpitations. Because most symptoms are non-specific and the disease is rare, a final diagnosis of PH

Table 1: Diagnostic classification of pulmonary hypertension (PH), ESC/ERS guidelines 20156 Group

Examples 1. PAH

• Idiopathic • Hereditary

• Drug-induced (appetite suppressants; dasatinib, etc.) • Associated with congenital heart disease, systemic sclerosis, portopulmonary hypertension, HIV infection, etc

2. PH due to left heart failure and/ or valve disease

3. PH due to chronic hypoxic lung disease

4. CTEPH 5. Other

• Systolic heart failure • Diastolic heart failure • Valve disease


• Interstitial lung disease • Sleep apnoea

• Following an acute episode of venous thromboembolism (70% of all patients) • In 30% of cases, no history of acute venous thromboembolism

• Haematological disorders • Sarcoidosis • Gaucher’s disease

• Langerhans cell histiocytosis

Pulmonary arterial hypertension (PAH)-specific treatment consists of prostanoids, endothelin receptor antagonists, type 5 phosphodiesterase inhibitors and soluble guanylyl cyclase stimulators. COPD = chronic obstructive pulmonary disease; CTEPH = chronic thromboembolic PH.

Yes, when inoperable

No (with exceptions)

No No

Medical treatment


is, on average, only made three years after the onset of symptoms.7

In most

cases of PH, the disease is associated with an underlying condition. Currently, more than 40 causes of PH have been recognised and these are subdivided into five groups (Table 1). During the Fifth World Symposium on Pulmonary Hypertension (WSPH), held in Nice in February 2013, the existing classification of PH was largely kept unchanged.8

PH due to left heart disease The most common cause of PH is left heart failure, either with preserved or reduced ejection fraction (HFpEF or HFrEF, respectively). In this condition, an elevated left ventricular end-diastolic pressure is transmitted into the left

Left heart failure Ipc-PH and Cpc-PH can both be associated with very severe increases in pulmonary artery pressure, but there are no treatment options in addition to standard left heart failure therapy.9

When valve

abnormalities are present, surgical correction needs to be considered.

atrium and pulmonary circulation. This passive increase in pressure is frequently compounded by functional (vasoconstriction) and structural (remodelling) changes in the pulmonary vascular bed, from arterioles to venules. As such, the 2015 Guidelines recognise two forms of left heart disease-related hypertension: isolated post-capillary pulmonary hypertension (Ipc-PH) and combined post-capillary and pre- capillary pulmonary hypertension (Cpc-PH).6

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