Pulmonary hypertension

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Classification according to ICD-10
I27 Other pulmonary heart diseases
I27.0 Primary pulmonary hypertension
I27.2 Other specified secondary pulmonary hypertension
ICD-10 online (WHO version 2019)

The pulmonary hypertension ( PH or PHT ) is a symptom of diseases caused by an increase in blood pressure in the pulmonary circulation and often a progressive increase of the vascular resistance in the pulmonary artery (with increased pulmonary artery pressure are marked). The patients with such an increase in pressure ( hypertension ) in the small circulation suffer from severely restricted physical performance, shortness of breath , circulatory disorders and fatigue . A secondary pulmonary hypertension caused by other diseases, is common. A primary pulmonary hypertension, however, is rare and has a poor prognosis. Pulmonary hypertension was first described by Ernst von Romberg in 1891 .

Symptoms

The symptoms , especially those of secondary pulmonary hypertension, can vary widely. The patients often suffer from reduced physical fitness, circulatory disorders including syncope , angina pectoris , Raynaud's syndrome , peripheral edema and fatigue. In connection with pulmonary arterial hypertension, a mostly right-sided pleural effusion can develop .

Pathophysiology

Pulmonary hypertension is present when the mean blood pressure in the pulmonary artery increases from normal (12–16  mmHg ) to more than 20 mmHg at rest. In the range of 30 to 40 mmHg, the first clinical symptoms can be observed, especially during exercise. In the range from 50 to 70 mmHg, the cardiac output decreases continuously. The pulmonary arterial blood pressure can chronically rise further to over 100 mmHg. The determination that pulmonary hypertension is present is the description of a measured value with the echocardiography or as an exact measurement with the right heart catheter . The pulmonary vascular resistance (PVR) describes the entirety of the resistance that the pulmonary vessels themselves or the flow obstacles in them offer to the blood flow through the lungs.

In acute pulmonary arterial hypertension, the pulmonary arteries constrict and the interior space in the vessel becomes smaller, but this process is reversible. Chronic pulmonary arterial hypertension leads to permanent thickening of the vascular muscles and progressive fibrosis with narrowing of the veins. The vessel walls are then no longer flexible and in this state regression is no longer possible (fixed pulmonary hypertension). As the pulmonary arteries are remodeled, less and less oxygen is absorbed through the lungs, while the increased pulmonary arterial resistance reduces cardiac output .

Classification and causes

In principle, there are three mechanisms that can lead to pulmonary hypertension: an obstruction of the outflow from the pulmonary veins, a narrowing of the pulmonary circulation and a strong increase in the lung flow through circulatory shunts before the onset of secondary vascular sclerosis.

The classification of pulmonary hypertension is based on the 2018 Nice Classification (6th World Conference). It replaces the Nice Classification from 2013. It should be noted that pulmonary hypertension is a common secondary pulmonary hypertension as a result of other diseases, such as B. chronic obstructive pulmonary disease (COPD) , heart failure , pulmonary embolism , pulmonary fibrosis , sarcoid , AIDS , ARDS , sickle cell anemia , scleroderma and congenital heart defects, can occur.

According to its etiology , pulmonary hypertension is divided into five classes:

1. Pulmonary artery hypertension (PAH)

1.1 Idiopathic pulmonary artery hypertension (IPAH)
1.2 Hereditary pulmonary arterial hypertension (HPAH), also known as familial pulmonary hypertension (fPAH)
1.2.1 BMPR2 malfunction (malfunction of a gene from chromosome 2 in humans)
1.2.2 ALK1, Endoglin (with or without hereditary hemorrhagic telangiectasia) - malfunction
1.2.3 Unknown
1.3 Drug- and Toxin-Induced PAH (DPAH)
1.4 Associated Pulmonary Artery Hypertension (APAH); with the following underlying diseases:
1.4.1 Collagenoses
1.4.2 HIV infection
1.4.3 portal hypertension
1.4.4 Congenital systemic-pulmonary shunts (including heart defects )
1.4.5 schistosomiasis
1.5 Pulmonary veno-occlusive disease PVOD and / or pulmonary capillary hemangiomatosis (PCH)
1.6 Persistent Pulmonary Artery Hypertension of the Newborn (PPHN)

2. Pulmonary hypertension in diseases of the left half of the heart

2.1 Systolic dysfunction
2.2 Diastolic dysfunction
2.3 Heart valve diseases ( mitral or aortic valve defects )
2.4 congenital / acquired left heart influence / outflow tract obstructions and congenital cardiomyopathies

3. Pulmonary hypertension in lung disease or hypoxia

3.1 Chronic obstructive pulmonary disease (COPD)
3.2 interstitial lung disease
3.3 other restrictive and obstructive mixed pulmonary diseases
3.4 Sleep apnea syndrome
3.5 Alveolar hypoventilation
3.6 Chronic altitude sickness
3.7 Systemic malformations

4. Pulmonary hypertension due to pulmonary artery obstruction

4.1 CTEPH
4.2 Other pulmonary artery obstructions

5. Pulmonary hypertension with unclear or multifactorial mechanisms

5.1 Haematological diseases: chronic haemolytic anemia, myeloproliferative diseases
5.2 Systemic and metabolic disorders: sarcoidosis , pulmonary Langerhans cell histiocytosis , neurofibromatosis , glycogen storage disease , Gaucher disease
5.3 Other diseases: chronic renal insufficiency with or without hemodialysis, fibrosing mediastinitis
5.4 Complex congenital heart diseases

Pulmonary artery hypertension (PAH) (1.)

In contrast to secondary pulmonary hypertension , primary or idiopathic pulmonary arterial hypertension ( incidence 4: 1,000,000 / year) does not represent a complication of another underlying disease. Often no cause can be found for it. The prognosis for PAH is rather poor.

  • Idiopathic pulmonary hypertension (1.1)

The causes of primary (or idiopathic ) pulmonary hypertension, however, are not exactly known. An increased release of blood vessel -contracting factors such. B. endothelin and thromboxane , and a reduced production of relaxing factors such. B. nitric oxide and prostacyclin are discussed as causes of increased tone of the pulmonary arteries. The increased serotonin levels in patients with primary pulmonary hypertension are considered to be a cause of the serotonin-mediated remodeling of the vascular muscles. Proinflammatory cytokines can also play an important role in this process .

  • Hereditary Pulmonary Artery Hypertension (HPAH) and BMPR2 - Malfunction ( 1.2 and 1.3 )

At the genomic level, a mutation of the bone morphogenic protein receptor 2 (BMPR2) is considered to be a possible cause for the familial accumulation of this disease. A mutation of activin-receptor-like-kinase 1 (ALK1) and the polymorphism of the promoter of the serotonin transporter (SERT) are also discussed as possible causes of primary pulmonary hypertension. Somatic genetic changes were also found in the endothelial cells of patients with pulmonary arterial hypertension.

  • Drug- and toxin-induced pulmonary artery hypertension (1.6)

A form associated with idiopathic pulmonary hypertension can be triggered in predisposed persons by drugs , especially some appetite suppressants . The respective appetite suppressants fenfluramine , dexfenfluramine and aminorex have meanwhile been withdrawn from the market due to this side effect. Activation of serotonin receptors (5-HT 2B type) and an associated change (remodeling) of pulmonary blood vessels are discussed as the cause of the pulmonary hypertension triggered by these drugs .

  • Associated Pulmonary Artery Hypertension (APAH); for other underlying diseases (1.7)

Pulmonary hypertension in heart failure (2.)

Pulmonary hypertension is most common in heart disease, especially in widespread left heart failure (contraction failure of the left ventricle). Isolated right heart failure is rather rare. Over a million people in Germany are affected by this heart disease, and a significant proportion develop pulmonary hypertension, at least a milder form.

  • Pulmonary hypertension in acquired heart defects (2.3)

Almost all patients with symptomatic mitral valve insufficiency develop PH during the course of their disease. Over 60% of patients with symptomatic aortic valve insufficiency also develop PH. If the heart is pumping weak, for example due to coronary artery disease or after a heart attack, PH often develops. Treatment of PH in the operating room and in the intensive care unit may be necessary in the context of the operative cardiac surgical care of the diseases mentioned. There are various options available in intensive care medicine : inhalative nitrogen monoxide (NO), levosimendan , milrinone , iloprost nebulization.

Pulmonary hypertension associated with lung disease or hypoxia (3.)

They are mostly mild forms of PH and they respond well to treatment with CPAP ventilation .

  • Alveolar hypoventilation and chronic altitude sickness (3.5 and 3.6)
  • Pulmonary hypertension in congenital heart defects (inherited malformations) (3.7)

Congenital heart defects can lead to the development of this clinical picture: at the chamber level, the ventricular septal defect (ventricular septal defect, VSD; especially a large ventricular septal defect), an atrio-ventricular septal defect (AVSD / AV canal), or a transposition of the large arteries (TGA) with a single ventricle Double outlet right ventricle (DORV). At the atrial level, the atrial septal defect (ASD, especially a large atrial septal defect), the malfunction of one or more pulmonary veins can cause the disease. At the aorto-pulmonary level, an (open) ductus arteriosus Botalli (PDA), an aorto-pulmonary window or a common arterial trunk can be the cause. In the case of congenital heart defects, an increase in pressure in the pulmonary vasculature can lead to a shunt reversal. The lung pressure is then higher than the pressure in the body's circulation, which leads to the so-called Eisenmenger reaction in the lungs , from which the symptoms of Eisenmenger syndrome can develop.

Pulmonary hypertension due to pulmonary artery obstruction (4.)

  • CTEPH (4.1)
  • Other pulmonary artery obstructions (4.2)

Pulmonary hypertension with unclear or multifactorial mechanisms (5.)

Diagnosis of pulmonary hypertension

The most meaningful, direct measurement of pulmonary arterial blood pressure and PAWP takes place during a right heart catheter examination with the help of the Swan-Ganz catheter . A mean pulmonary artery pressure (mPAP) of> 20 mmHg is a PH. An important non-invasive alternative is the determination and assessment of pulmonary hypertension with the help of echocardiography , in which tricuspid regurgitation is usually present. This is often done via systolic pulmonary artery pressure (sPAP). In 2010 there were indications that measuring the mPAP is more advantageous. An Echo study on almost 250 patients showed that a mean PAP of 25.5 mmHg can detect PH with very good sensitivity and specificity or diagnostic reliability. In addition, that can X-ray image of the thorax of pulmonary hypertension indicate. A possible concomitant heart failure can be made plausible in the blood test by an increased BNP . A biopsy of the lung tissue is only carried out if there is a suspicion of independent, possibly causative lung diseases. In addition to the anamnesis, the " 6-minute walk test ", which determines the distance the patient can cover in six minutes, provides information about the initial situation and the success of the therapy. The basic rule of diagnosis is to determine the type of PH and whether there is a treatable underlying disease.

Therapy - as of 2017

Treatment requires a precise determination of which PH it is. If an underlying disease leading to PH can be treated well, pulmonary hypertension can also be favorably influenced. Left ventricular failure, for example, can often be treated properly as an underlying disease, and children with congenital heart defects are operated on as early as possible so that pulmonary hypertension cannot develop. Oxygen therapy ( LTOT ) is an option among general measures . Iron deficiency should be corrected, but bloodletting is almost never indicated. Diuretics can be used, sometimes anticoagulation is necessary. Expertly guided physical training is recommended. The patient's renunciation of nicotine and a reduction in excess weight to normal weight is actually essential.

Special drug therapy for pulmonary arterial hypertension is difficult. Some drugs have been approved for the treatment of pulmonary arterial hypertension, in some cases with restrictions. Before prescribing these medications, the attending physician should seek advice from an expert center. Sometimes a right heart catheter has to be used to find out whether the patient is a “responder”. Calcium channel blockers can also be given in cases with a positive vasoreactivity test (a significant decrease in pulmonary artery pressure in the acute test with nitric oxide ) .

Depending on the severity of the disease or NYHA recommend international guidelines the differentiated use of  endothelin receptor antagonists , PDE-5 inhibitors , Riociguat , prostacyclin - analogues and in particular cases, the lung transplant . After a lung transplant for interstitial lung disease ( ILD ), the median survival time is about five years.

Veterinary medicine

The following are used:

forecast

The prognosis varies depending on the cause and severity of the PH. A possible death happens through a mostly acute onset of right cardiac decompensation ( right heart failure ) with malignant cardiac arrhythmias .

literature

  • German Society for Cardiology - Working group pulmonary hypertension: Diagnosis and therapy of chronic pulmonary hypertension . In: Clinical Research in Cardiology . tape 96 , no. 5 , 2007, p. 301–330 ( leitlinien.dgk.org [PDF]).
  • Ventzislav Petkov, D. Doberer: Pulmonary Hypertension. In: Journal of Hypertension. Volume 7, Number 3, 2003, pp. 7-15. online ( Memento from September 28, 2007 in the Internet Archive )
  • Ina Michel-Behnke among others: Pulmonary hypertension in childhood and adolescence: Possibilities of long-term treatment . In: Deutsches Ärzteblatt . tape 94 , no. 14 , April 4, 1997, pp. A-917-923 ( aerzteblatt.de [PDF]).
  • Nazzareno Galiè, Alessandra Manes, Luca Negro, Massimiliano Palazzini, Maria Letizia Bacchi-Reggiani, Angelo Branzi and others: A meta-analysis of randomized controlled trials in pulmonary arterial hypertension . In: Eur Heart J . tape 30 , no. 4 , 2009, p. 394-403 , PMC 2642921 (free full text).
  • Nazzareno Galie, Gerald Simonneau et al .: The Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT) . In: Eur Heart J . tape 30 , no. 20 , 2009, p. 2493-2537 ( eurheartj.oxfordjournals.org [PDF]).
  • S2k guideline pulmonary arterial hypertension (PAH) in children and adolescents of the German Society for Pediatric Cardiology (DGPK). In: AWMF online (as of 2013)

Web links

Individual evidence

  1. Berthold Jany, Tobias Welte: Pleural effusion in adults - causes, diagnosis and therapy. In: Deutsches Ärzteblatt Volume 116, No. 21, (May) 2019, pp. 377–385, here: p. 380.
  2. ^ Herbert Reindell , Helmut Klepzig: Diseases of the heart and the vessels. In: Ludwig Heilmeyer (ed.): Textbook of internal medicine. Springer-Verlag, Berlin / Göttingen / Heidelberg 1955; 2nd edition ibid. 1961, pp. 450-598, here: p. 579 ( functional disorders and diseases that lead to an increase in pressure in the small circulation ) and 584 f. ( The hypertension in the small circulation ).
  3. Gérald Simonneau, David Montani, David S. Celermajer, Christopher P. Denton, Michael A. Gatzoulis, Michael Krowka, Paul G. Williams, Rogerio Souza: Haemodynamic definitions and updated clinical classification of pulmonary hypertension in European Respiratory Journal 2018; DOI: 10.1183 / 13993003.01913-2018
  4. Nazzareno Galiè, Marc Humbert u. a .: 2015 ESC / ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. In: European Heart Journal , Volume 37, 2015, p. 67, doi : 10.1093 / eurheartj / ehv317 .
  5. HM Hoeper et al. : Pulmonary hypertension. In: Deutsches Ärzteblatt International 114, 2017, pp. 73–84. doi: 10.3238 / arztebl.2017.0073
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  7. M. Humbert, RC Trembath: Genetics of pulmonary hypertension: from bench to bedside. In: Eur Respir J. 20 (3), September 2002, pp. 741-749. PMID 12358355
  8. ^ B. Preckel, S. Eberl, J. Fräßdorf, MW Hollmann: Management of patients with pulmonary hypertension. In: The anesthesiologist . 61, 2012, pp. 574-587, doi: 10.1007 / s00101-012-2040-2 .
  9. Gerd Herold and colleagues: Internal Medicine 2020. Self-published, Cologne 2020, ISBN 978-3-9814660-9-6 , p. 408.
  10. Denis Chemla et al .: Systolic and Mean Pulmonary Artery Pressures - Are They Interchangeable in Patients With Pulmonary Hypertension? In: Chest . tape 147 , no. 4 , 2015, p. 943 - 950 , doi : 10.1378 / chest.14-1755 (English, full text available).
  11. F. Er, S. Ederer, AM Nia, E. Caglayan, KM Dahlem, N. Semmo, N. Gassanov: Accuracy of Doppler-echocardiographic mean pulmonary artery pressure for diagnosis of pulmonary hypertension. In: PloS one. Volume 5, number 12, 2010, p. E15670, doi: 10.1371 / journal.pone.0015670 . PMID 21179417 , PMC 3003692 (free full text).
  12. N. Galie include: Comparative analysis of clinical trials and evidence-based treatment algorithm in pulmonary arterial hypertension. In: J Am Coll Cardiol , 43 (12 Suppl S), Jun 16, 2004, pp. 81S-88S. PMID 15194183 .
  13. AW Brown: Lung Transplantation in IIP , Review, in: Respirology (2016) 21, 1173–1184.