Pulmonary arterial hypertension


Pulmonary arterial hypertension (PAH) is a Rare Disease that causes an increase in the pulmonary vascular resistance and an increase in blood pressure in the pulmonary circulation. The reduced blood flow through the lungs is accompanied by an insufficient supply of oxygen to the body. As a consequence, patients suffer from lethargy, shortness of breath and a drop in physical performance. If PAH is left untreated for two or three years after first diagnosis has been made, it will lead to life-threatening heart failure. Early diagnosis and commencement of proper treatment are important for the progress of the disease and the prognosis.

  • Erweiteter Inhalt für eingeloggte Fachkreis-Mitglieder.
    Öffentlicher Inhalt. Für erweiterten Fachkreis-Inhalt bitte anmelden.
    Cause & Symptoms
  • Erweiteter Inhalt für eingeloggte Fachkreis-Mitglieder.
    Öffentlicher Inhalt. Für erweiterten Fachkreis-Inhalt bitte anmelden.
    Diagnosis & Therapy
  • Erweiteter Inhalt für eingeloggte Fachkreis-Mitglieder.
    Öffentlicher Inhalt. Für erweiterten Fachkreis-Inhalt bitte anmelden.
    Everyday life

Diagnosis

If a patient is suspected of having pulmonary hypertension (due to poor physical performance and impaired breathing), they will undergo a comprehensive physical examination and their medical history will be analyzed. Aside from the family medical history, it is also very important to provide details of any previous drug prescriptions and possible substance abuse. 

Other possible tests:

  • ECG (electrocardiogram)
  • Heart ultrasound – an echocardiography is a highly informative examination and does cause the patient any stress
  • CXR (chest X-ray)
  • Laboratory tests such as haemogram (blood count), coagulation parameters, liver findings (diagnostic), any existing antibodies, HIV test (if an appropriate risk profile exists)
  • Blood gas analysis
  • Lung function test
  • High-resolution computed tomography (HRCT)
  • Ventilation/perfusion scan
  • Angiography
  • Cardiac catheter examination

Treatment

The treatment of pulmonary arterial hypertension has been the subject of intensive research over the past few years. This has improved the prognosis and quality of life of patients afflicted by pulmonary arterial hypertension.

Currently, the primary goal of therapeutic methods is to improve the blood flow in the small (pulmonary) circulation. Improving the blood flow relieves pressure on the right side of the heart, increases the absorption of oxygen and improves physical performance.

It is very important that patients who suffer from PAH have their disorder treated at a center that specializes in PAH.

Drug treatment

This treatment is based on medication that expand the blood vessels: 

  • Prostacyclin derivatives (intravenous: epoprostenol; subcutaneous and intravenous: treprostinil; inhalative: iloprost)
    Treprostinil is usually administered subcutaneously using a micropump.
    In case of local intolerance to an otherwise effective subcutaneous therapy, treprostinil may also be administered via a special implantable pump.
  • Endothelin receptor antagonists, selective phosphodiesterase 5 inhibitors and Riociguat a soluble guanylate cyclase (sGC) are drugs for oral administration.

In addition anticoagulant drugs, glycosides, and diuretic agents are also used.

Long-term oxygen therapy

If it is necessary to improve the patient’s oxygen supply, a nasal oxygen tube should be used to complement the drug treatment.

Surgical measures

Depending on the cause and the severity of the pulmonary arterial hypertension, surgical measures such as atrioseptostomy or a lung transplant may also be considered.