Abstract
Pulmonary hypertension is defined as a syndrome characterized by an increase in mean pulmonary artery pressure > 20 mm Hg. It is most commonly found in diseases of the heart or lungs.
More rarely, pulmonary hypertension results from primary pulmonary vascular involvement (particularly pulmonary arterial hypertension, when pulmonary arterioles are affected, and chronic thromboembolic pulmonary hypertension associated with incomplete reperfusion after acute pulmonary embolism). The treatment of pulmonary arterial hypertension is dominated by pharmacotherapy.
Patients with a positive vasodilation test are indicated for treatment with high-dose calcium channel blockers. In the case of a negative test (the majority of patients), pharmacotherapy with prostanoids and prostacyclin receptor agonists, endothelin receptor antagonists, phosphodiesterase 5 inhibitors and soluble guanylate cyclase stimulators is indicated (initial combination therapy in patients without cardiopulmonary comorbidities, initial monotherapy in patients with cardiopulmonary comorbidities, repeated reevaluation during follow-up and adequate modification of treatment).
In patients with pulmonary hypertension associated with cardiopulmonary disease, special attention should be focused on patients with a severe precapillary component or severe pulmonary hypertension (pulmonary vascular resistance > 5 Wood units). In these patients who clinically manifest significant hypoxemia, repeated episodes of right-sided cardiac decompensation and échocardiographie findings of a dilated and dysfunctional right ventricle, a possible concomitant cause of pre-capiiiary pulmonary hypertension should be excluded.
These patients should be discussed with specialized pulmonary hypertension centers with the question of individualized therapeutic intervention. In chronic thromboembolic pulmonary hypertension, lifelong anticoagulation therapy is indicated.
Further treatment is decided by operability. In operable patients, pulmonary endarterectomy is indicated.
In inoperable patients and in patients with residual pulmonary hypertension after pulmonary endarterectomy, balloon pulmonary angioplasty and/or drug therapy is indicated.