Chronic thromboembolic pulmonary hypertension

Classification according to ICD-10
I27.2	Other specified secondary pulmonary hypertension
I26.9	Pulmonary embolism without indication of an acute cor pulmonale
ICD-10 online (WHO version 2019)

The chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by the repeated Einschwemmung of thrombi in the pulmonary arteries, so pulmonary embolism . These then completely or partially clog and constrict the vessels and transform them into connective tissue (remodeling) . Pulmonary hypertension often develops with a poor prognosis. CTEPH is rare and is also not recognized in more than half of the cases. The prevalence is around 2%. The causes of the disease have only been partially clarified.

Risk factors

Risk factors are haematological diseases, shunts, thyroid diseases, tumor diseases, pacemakers, chronic inflammation. A connection with acute pulmonary embolism is questionable.

diagnosis

Clinically, weakness, shortness of breath and syncope are usually in the foreground. An echocardiography , a possible right heart load in the resting ECG, and an evaluation of the NT-proBNP can help. CT angiography and ventilation perfusion scintigraphy are very meaningful, in otherwise healthy patients as the sole perfusion scintigraphy . MRI procedures offer further possibilities. The hemodynamic parameters are then examined using a right heart catheter .

therapy

The curative option is pulmonary endarterectomy (PEA), which may be possible in about a third of patients. Balloon angioplasty may be considered depending on the situation . Additional measures include the treatment of a possible underlying disease as well as drug options for pulmonary hypertension, anticoagulation and riociguat . Oxygen administration and professionally guided physical training are recommended as support.

Individual evidence

↑ Henning Gall, Marius M. Hoeper u. a .: An epidemiological analysis of the burden of chronic thromboembolic pulmonary hypertension in the USA, Europe and Japan. In: European Respiratory Review. 26, 2017, p. 160121, doi : 10.1183 / 16000617.0121-2016 .
↑ M. Riedel, V. Stanek, J. Widimsky, I. Prerovsky: Long-term follow-up of patients with pulmonary thromboembolism. Late prognosis and evolution of hemodynamic and respiratory data. In: Chest. Volume 81, Number 2, February 1982, pp. 151-158, PMID 7056079 .
↑ MM Hoeper, MM Madani, N. Nakanishi, B. Meyer, S. Cebotari, LJ Rubin: Chronic thromboembolic pulmonary hypertension. In: The Lancet. Respiratory medicine. Volume 2, Number 7, July 2014, pp. 573-582, doi : 10.1016 / S2213-2600 (14) 70089-X , PMID 24898750 (review).

[1] Henning Gall, Marius M. Hoeper u. a .: An epidemiological analysis of the burden of chronic thromboembolic pulmonary hypertension in the USA, Europe and Japan. In: European Respiratory Review. 26, 2017, p. 160121, doi : 10.1183 / 16000617.0121-2016 .

[2] M. Riedel, V. Stanek, J. Widimsky, I. Prerovsky: Long-term follow-up of patients with pulmonary thromboembolism. Late prognosis and evolution of hemodynamic and respiratory data. In: Chest. Volume 81, Number 2, February 1982, pp. 151-158, PMID 7056079 .

[3] MM Hoeper, MM Madani, N. Nakanishi, B. Meyer, S. Cebotari, LJ Rubin: Chronic thromboembolic pulmonary hypertension. In: The Lancet. Respiratory medicine. Volume 2, Number 7, July 2014, pp. 573-582, doi : 10.1016 / S2213-2600 (14) 70089-X , PMID 24898750 (review).