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Diagnostik der pulmonalen Hypertonie

Was kann der Pneumologe beitragen?

Diagnostic workup of pulmonary hypertension

What can pneumonology add?

  • Leitthema
  • Published:
Der Pneumologe Aims and scope

Zusammenfassung

Die pulmonale Hypertonie (PH) ist durch eine im Rechtsherzkatheter gemessene Erhöhung des pulmonal-arteriellen Mitteldrucks auf ≥25 mmHg in Ruhe charakterisiert und hat untherapiert eine schlechte Prognose. Der Begriff umfasst die verschiedenen Formen eines Lungengefäßhochdrucks, die entweder ohne bekannte Ursache auftreten oder sich im Rahmen einer anderen Erkrankung manifestieren können. Eine genaue diagnostische Klassifikation in eine von 5 Gruppen ist unbedingt notwendig, da sich für jede Gruppe unterschiedliche Therapieansätze ergeben. Erste Anzeichen einer PH sind unspezifisch und machen die Frühdiagnose schwierig. Zur Diagnosestellung wird in den aktuellen europäischen Leitlinien ein neuer Algorithmus empfohlen. Bei Verdacht wird nichtinvasiv durch Echokardiographie das Vorliegen der PH untersucht und anschließend geprüft, ob eine Linksherzerkrankung oder eine Lungenerkrankung als Ursache der PH vorliegen. Können diese ausgeschlossen werden oder bestehen Hinweise auf eine „Out-of-proportion-PH“, die nicht allein durch die Grunderkrankung erklärbar ist, erfolgt die systematische Abklärung mit Untersuchung der Ätiologie sowie der funktionalen und hämodynamischen Einschränkungen.

Abstract

Chronic pulmonary hypertension (PH) is defined as a permanently increased mean pulmonary artery pressure ≥25 mmHg as assessed by right heart catheterisation and is associated with a poor prognosis without therapy. Depending on pathological, pathophysiological and therapeutic characteristics, clinical conditions with PH are classified into five groups. An exact diagnostic classification is necessary for application of the current treatment options for the different forms of PH. The first symptoms of PH are non-specific; therefore an early diagnosis is difficult. The current guidelines propose a new diagnostic algorithm to improve the precise diagnostic workup. In any case of suspected PH transthoracic echocardiography should be performed. If signs of PH are found, underlying left heart disease and lung diseases need to be searched for. In the case of exclusion or presence of “out of proportion PH”, which is not explained by underlying disease, a systematic evaluation is needed to clarify the specific aetiology and clinical group of PH and to quantify the functional and haemodynamic impairment.

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Abbreviations

ALK-1:

Activin-receptor-like Kinase

ANA:

Antinukleäre Antikörper

BMPR2:

“Bone morphogenetic protein receptor 2”

CHD:

Angeborene Herzfehler („congenital heart disease“)

KMR:

Kardiale Kernspintomographie

CTD:

“Connective tissue disease”

CTEPH:

Chronische thromboembolische PH

HHT:

Hereditäre hämorrhagische Telangiektasie

HRCT:

„High-resolution computed tomography“

PCH:

Pulmonal-kapilläre Hämangiomatose

PVOD:

Pulmonale venookklusive Erkrankung

RHK:

Rechtsherzkatheter

TEE:

Transösophageale Echokardiographie

TTE:

Transthorakale Echokardiographie

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  1. Medizinische Klinik V, Universitätsklinikum des Saarlandes, Kirrberger Str. 1, 66421, Homburg / Saar, Deutschland

    H. Wilkens

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Wilkens, H. Diagnostik der pulmonalen Hypertonie. Pneumologe 7, 174–186 (2010). https://doi.org/10.1007/s10405-009-0378-1

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