Pulmonary hypertension

Pulmonary hypertension is characterized by a high blood pressure in the lungs and encompasses a group of progressive diseases.

Symptoms

Symptoms include shortness of breath during exertion, fatigue and tightness in the chest. While the main symptom is shortness of breath during exertion, other symptoms include tiredness, tightness in the chest, heart rhythm disorders and, in advanced disease, cardiovascular collapse with sudden loss of consciousness (syncope).

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Causes

Pulmonary hypertension (PH) is divided into five different groups depending on the underlying disease. These include the two intrinsic pulmonary circulation diseases: pulmonary arterial hypertension (PAH; WHO Group 1), and chronic thromboembolic hypertension (CTEPH; WHO Group 4).

PAH can develop with no apparent cause or in association with connective tissue diseases of the liver, heart and other diseases. CTEPH is a thromboembolic disease which may occur as a result of acute pulmonary embolism with varying latency periods of up to decades; however, it can also appear without prior pulmonary embolism. Furthermore, PH can occur in conjunction with heart failure (WHO Group 2), lung disease (WHO Group 3) or due to other unclear mechanisms (WHO Group 5). They all have increased mean pulmonary arterial pressure (mean PAP >25 mmHg) measured using right heart catheterization. Depending on the end-expiratory measured pulmonary arterial wedge pressure, PH is defined as precapillary (PAWP <15 mmHg) or postcapillary PH (PAWP >15mmHg), the latter indicating Group 2 PH with left heart disease.

Types of treatment

Without treatment, patients with PH have a rather limited prognosis. Only certain patients with CTEPH can be cured by surgical pulmonary endarterectomy. For many patients with PH, treatment of the underlying disease is crucial to improve their PH and its related symptoms. This includes quitting smoking, oxygen and inhalation therapy for PH in lung disease and the consistent treatment of heart failure in PH with left heart disease. Various drug therapies have been developed in recent years for PAH Group 1 which have shown efficacy in randomized trials and improve the prognosis of patients. Drug therapies have also been recently approved for inoperable CTEPH patients.

Collaboration

At the University Hospital Zurich, the conditions are optimal for the highly specialized treatment of patients with PH. Specialists at the Department of Pulmonology are clinically and scientifically involved with this disease in close collaboration with cardiologists, rheumatologists, thoracic surgeons, intensive care physicians, radiologists, anesthesiologists and professionals from other disciplines.

Consultation Pulmonary Hypertension
Swiss Society for Pulmonary Hypertension
Pulmonary Hypertension Patients' Association
Current research on Pulmonary Hypertension at USZ

Last update: 17.10.2017 | Person responsible:
Professor Malcolm Kohler, MD