Pulmonary Hypertension

Abstract

Background: About 1% of adults suffer from pulmonary hypertension (PH). The various types of PH differ widely with respect to their incidence, clinical significance, and treatment.

Methods: Selective review of the literature in association with a consensus conference.

Results: Pulmonary hypertension is divided into five major categories. Those that are of particular clinical relevance are pulmonary arterial hypertension, chronic thromboembolic pulmonary hypertension, and pulmonary hypertension due to left heart and lung diseases. Ten drugs from five different substance classes are now available for the treatment of PH and are often given in combination. The treatment strategy is determined by risk stratification based on the severity of disease, along with the clinical phenotype and possible accompanying illnesses. The preferred treatment for chronic thromboembolic pulmonary hypertension is surgical pulmonary endarterectomy; inoperable patients are treated with drugs and endovascular interventions. PH due to left heart and lung diseases generally calls for specific treatment of pulmonary hypertension only if there is severe right-heart strain.

Conclusion: The diagnosis and treatment of severe forms of PH, in particular, pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension, are complex and are best carried out in close cooperation between the local physician and specialized centers.

Figure 1

The principal forms of pulmonary hypertension (modified from [1, 2]) COPD, Chronic obstructive lung disease; CTEPH, chronic thromboembolic pulmonary hypertension; HIV, human immunodeficiency virus; ILD, interstitial lung disease; PAH, pulmonary arterial hypertension; PVOD, pulmonary veno-occlusive disease

Figure 2

Initial diagnostic procedure in suspected pulmonary hypertension BNP, Brain natriuretic peptide; CT, computed tomography; CTEPH, chronic thromboembolic pulmonary hypertension; ECG, electrocardiography; PAH, pulmonary arterial hypertension; PH, pulmonary hypertension; LA, left atrium; NT-proBNP, N-terminal fragment of pro-brain natriuretic peptide; RV, right ventricle; V/Q, ventilation/perfusion

Figure 3

Treatment algorithm for pulmonary arterial hypertension based on the European guidelines and the Cologne Consensus Conference PAH, pulmonary hypertension *¹ The phenotype determines whether PAH is classified as typical or atypical; age alone is not a sufficient criterion, but the older the patient, the greater the likelihood of comorbidity and risk factors for cardiopulmonary disease (hypertonia, coronary heart disease, diabetes mellitus, obesity, etc.). *² Initiation immediately, or within 3 months of diagnosis, of combination treatment with endothelin receptor antagonists plus phosphodiesterase-5 inhibitors or stimulators of soluble guanylate cyclase. *³ Initiation immediately, or within 3 months of diagnosis, of combination treatment with endothelin receptor antagonists plus phosphodiesterase-5 inhibitors or stimulators of soluble guanylate cyclase plus a prostacyclin derivative. *⁴ In these often elderly patients, who present with cardiac and/or pulmonary comorbidity, the efficacy and tolerance of drugs for PAH have been less extensively investigated; as this is particularly true fo combination treatments, starting with monotherapy is recommended. *⁵ Individual adjustment of treatment: in “typical” PAH, if it seems appropriate, further escalation of the combination treatment to include prostacyclin derivatives; consider SC/IV prostacyclin; consider switch from phosphodiesterase-5 inhibitor to sGC stimulator; in “atypical” PAH,decide case by case; moreover, optimize supportive treatment for all patients, including rehabilitation measures.

Figure 4

Procedure in patients with chronic thromboembolic pulmonary hypertension (modified from [27, 40]) *¹ Clinical examination and echocardiography are usually sufficient *² Currently only riociguat is licensed for the treatment of these patients *³ Lung transplantation is seldom necessary in patients with CTEPH