Therapeutic Targets
- To turn patients into low-risk patients through therapy, i.e., improve prognosis
- Curative therapy for pulmonary therapy does not exist.
Therapy recommendations
- Treatment of the underlying disease
- Reduction of pressure in the pulmonary circulation:
- Initial therapy or main therapy depending on the degree of heart failure (NYHA): endothelin receptor antagonists (ERA), PDE-5 inhibitors, prostacyclin analogs, selective prostacyclin IP receptor agonists; note:
- Therapy should be administered at specialized centers
- If responders in vasoreactivity testing (test out in right heart catheterization; up to 15% responders), then calcium antagonists high dose in WHO functional class I-III.
- Vasoreactivity test negative: oral combination therapy in:
- Clinical deterioration or failure to achieve treatment goals after 3-6 months.
- Low and intermediate risk (WHO classes II and III) as an option initial oral combination therapy.
- high risk (WHO class IV) initial 3-drug combination therapy:
- Endothelin receptor antagonist (ERA) + PDE-5 inhibitor (or sGC stimulator) + prostacyclin analog (i.v.).
- Follow-up by spiroergometry, 6-min walk distance.
- In the case of right heart decompensation, if necessary, intensive therapy with inhaled / i.v. prostacyclin analogs.
- Adjuvant or supportive (“supportive”) therapy for pulmonary hypertension may include:
- Oxygen at a pO2 < 60 mmHg (target saturation ≥ 92%), oxygen administration (also as long-term therapy) in chronic obstructive pulmonary disease (COPD) (I-C).
- Diuretics (dehydrating drugs) v.a. in right heart failure (right heart weakness) (I-C)
- Iron supplementation in iron deficiency anemia (anemia due to iron deficiency) (IIb-C.
- Cardiac glycosides in concurrent atrial fibrillation (VHF) with tachycardic conduction.
- Anticoagulation/oral anticoagulation (OAK; anticoagulant) – may be considered in sporadic and hereditary (inherited) forms of PAH (level of evidence IIB-C)).
- Influenza and pneumococcal vaccinations (I-C).
- Supervised physical training in specialized facilities (IIa-B).
- Initial therapy or main therapy depending on the degree of heart failure (NYHA): endothelin receptor antagonists (ERA), PDE-5 inhibitors, prostacyclin analogs, selective prostacyclin IP receptor agonists; note:
- Lung transplantation (LUTX; last therapeutic option) when conservative treatment options fail. Notice: If an adequate therapeutic outcome is not achieved despite multiple combination therapies, the patient should present promptly to a transplant center.
- Chronic thromboembolic pulmonary hypertension/ pulmonary hypertension (CTEPH): lifelong anticoagulation (anticoagulation); primary surgery (see below “Surgical therapy”: pulmonary endarterectomy), if this is not possible or does not lead to the desired success → therapy with riociguat.
- See also under “Further therapy”.
Note
- In left heart or lung disease and pulmonary hypertension, the use of pulmonary arterial hypertension medications is indicated only in exceptional cases.
Initial therapy
| NYHA II | NYHA III | NYHA IV | Grade of recommendation |
|
|
IA | |
|
|
IB | |
|
|
IIaC | |
|
IIbB |
NYHA classification – see under heart failure (heart failure)/classification.
* Combination therapy in patients whose disease is inadequately controlled by the use of an endothelin receptor antagonist, and/or in combination with a PDE-5 inhibitor, or as monotherapy.
* * According to an AMC communication, sildenafil use in pregnancy for placental insufficiency resulted in the following: 19 babies out of a total of 93 women died: 11 of the babies who died suffered from lung disease, specifically pulmonary hypertension. Information on the study “The Dutch STRIDER” (Sildenafil TheRapy In Dismal Prognosis Early-onset Fetal Growth Restriction): https://clinicaltrials.gov/ct2/show/NCT02277132.
Note: Endothelin receptor antagonists (ERAs) have an affinity for cytochrome P450 (CYP) isoenzyme 3A4; interactions: see below.
