In pulmonary hypertension (PH) – colloquially called pulmonary hypertension – (thesaurus synonyms: Pulmonary artery sclerosis; primary pulmonary hypertension; pulmonary arterial hypertension (PAH); pulmonary artery sclerosis; pulmonary arteriosclerosis; pulmonary hypertension; pulmonary hypertension associated with chronic thromboembolism; pulmonary idiopathic hypertension; pulmonary hypertension; ICD-10 I27.-) is an increase in pressure in the pulmonary arterial system. This increase in pressure results in elevated blood pressure. Definition of pulmonary arterial hypertension (measured by right heart catheterization):
- Pulmonary arterial mean pressure ≥ 25 mmHg* , pulmonary arterial wedge pressure (PAWP; occlusion pressure) ≤ 15 mmHg, and pulmonary vascular resistance (PVR) > 240 dyn × s × cm-5 (or with a cutoff > 3 Wood units).
- If the pulmonary arterial mean pressure at rest is between 21-24 mmHg, the patient is said to have latent pulmonary hypertension.
Children: mPAP > 20 mmHg in children > 3 months at sea level. Hemodynamic criteria are used to differentiate a precapillary from a postcapillary form of PH (see classification below). Many different conditions can cause pulmonary hypertension. The following forms of pulmonary hypertension (PH) can be distinguished:
- Primary pulmonary hypertension; this is also referred to as idiopathic pulmonary arterial hypertension (iPAH) b
- Secondary pulmonary hypertension, i.e., is a consequence of another underlying disease.
- Pulmonary arterial hypertension (PAH).
- Chronic thromboembolic pulmonary hypertension (CTEPH); persistent obstruction (occlusion) of the pulmonary circulation as a result of inadequate recanalization of the pulmonary circulation after pulmonary (“lung-related”) thromboembolism (occlusion of a blood vessel by a dislodged blood clot)
- Other forms of pulmonary hypertension with different pathomechanisms.
Primary pulmonary hypertension is many times less common than secondary pulmonary hypertension. Sex ratio: males to females is 1: 2 (primary pulmonary hypertension). Peak incidence: The peak incidence of pulmonary hypertension is in middle age. Primary pulmonary hypertension occurs predominantly between 20 and 30 years of age in women and between 30 and 40 years of age in men. Prevalence: The 2-year prevalence of chronic thromboembolic pulmonary hypertension (CTEPH) is approximately 1-4%. The incidence (frequency of new cases) for primary pulmonary hypertension is approximately 1-2 cases per 1,000,000 population per year (in Germany). The incidence for pulmonary arterial hypertension is about 3-10 new cases per 1,000,000 population per year.The incidence for chronic thromboembolic pulmonary hypertension (CTEPH) in patients after pulmonary embolism was 3.8%. Course and prognosis:In the setting of secondary pulmonary hypertension, treatment of the underlying disease is the primary concern. Progression (progression) of pulmonary hypertension can be slowed. In the early stages of pulmonary hypertension, the disease causes few, if any, symptoms. It is only in the advanced stages that symptoms such as reduced physical performance, dyspnea on exertion (shortness of breath on exertion) or peripheral edema (water retention in the legs) occur. From blood pressure values in the pulmonary artery of 50-70 mmHg, the consequence of untreated pulmonary hypertension is right heart failure (right heart weakness). In severe cases, transplantation (organ transplantation) of the heart and lungs is the last therapeutic option.The diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH) is made on average with a delay of more than 1.5 years. Patients present with the following symptoms: Exertional dyspnea (shortness of breath on exertion), chest pain (chest pain), fatigue, edema (water retention), or syncope (brief loss of consciousness). Untreated, these patients have a median life expectancy of less than three years. however, there are now several effective treatment options (surgical excision of thrombotic material, i.e., pulmonary endarterectomy using the heart–lung machine; a new treatment option is pulmonary balloon angioplasty (pulmonary artery balloon angioplasty, BPA)). The 5-year survival rate depends on the level of mean pulmonary artery pressure (mPAP).If the pulmonary arterial mean pressure is > 30 mmHg, the 5-year survival rate is approximately 30%, and only 10% for values > 50 mmHG. Untreated, the average life expectancy from diagnosis is three years. Caveat (Warning! ): patients with pulmonary hypertension should avoid a Valsalva pressor (synonym: Valsalva maneuver) because of the risk of orthostatic hypotension (drop in blood pressure associated with standing up) and syncope (brief loss of consciousness).
