Pulmonary hypertension (PAH) in rheumatic disease 4.17/5 (6)

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Pulmonary hypertension (PAH): Diameter of pulmonary artery (blue arrow) exceeds aortic diameter (red arrow): Rajaram S, Thorax, 2014. CC BY-NC 4.0


Pulmonary hypertension (PH) is a serious condition where blood from the heart experiences increased resistance to the blood vessels of the lungs (lung arteries). This causes the pressure to rise in the pulmonary arteria. Thus, the heart (right ventricle) must pump at increased strain. Eventually, heart failure may occur.

Disease Causes

  • Thickening of the artery walls (hyperplasia of intimate and hypertrophy of smooth muscle cells), often at Systemic sclerosis) (PAH SSc)
  • Blood clots (thrombosis in small and medium-sized lung arterioles), (CTEPH)
  • Heart failure (left ventricle), (PH-LHD)
  • Pulmonary disease (PH-ILD)


  • Echo cor (ultrasound of the heart): Here calculated systolic pulmonary artery pressure indirect The measurements are less accurate than in cardiac catheter examination (see below). Estimated pressure above 30-35mmHg is suspicious of PH
  • Cardiac catheter (Right part of the heart). Mean pressure (MAP) above 20 mmHg at rest or more than 30mmHg at load is pulmonary hypertension

Classification (WHO)


Pulmonary hypertension (PH) occurs without special reason (idiopathic) or associated with diseases, especially at Systemic sclerosis (7-16%), less frequently MCTD (4%) (reference: Gunnarsson R, 2013), SLE (1%), Sjøgren`s syndrome (2%) and Myositis. Also congenital or acquired heart disease, portal hypertension, HIV infection, intake of anorexia (slimming) or amphetamine (drug) and severe lung disease may be the cause of PH.


Heavy breathing (dyspnoea) and fainting (syncope) when stressed, fatigue / exhaustion, chest pain, cough, bloody cough (haemoptysis), hoarseness. Peripheral swelling (edema), enlarged liver (hepatomegaly).

Screening with ultrasound of the heart (yearly) in risk groups (systemic sclerosis) important. Early treatment improves prognosis.

Medical Examination

  • Screening (echo of the heart) of risk groups (annual at Systemic sclerosis)
  • Clinical: visible jugular vein pulse, left sided parasternal swelling, split 2. heart tone, systolic bilyd
  • Chest X-ray (central pulmonary arteries): The right ventricle / atrium is enlarged in the retrosternal space
  • CT examination of the thorax: The diameter of the pulmonary artery is greater than the aorta (see illustration at the top of the page)
  • Lung function tests (very low DLCO)
  • 6 min walking test (falling below 330-380 m may indicate unfavouable prognosis)
  • pro-BNP elevated and increasing in blood sample
  • Pulmonary angiography (X-ray examination) and scintigraphy (nuclear medicine) are considered if the cause of the disease is unclear (exclude thromboembolisms)
  • ECG (right hypertrophy = R / S more than 1 in V1), right branch block, β-pulmonary in II, arrhythmias)

Echo-doppler (Ultrasound heart)

  • Thickened wall in right ventricle and paradox deviation to the left in the systole
  • Functional tricuspid insufficiency
  • The pressure is calculated: Bernoulli equation: PAP systolic = (4 x tricuspid jet velocity squared) + RAP (v.cava blood pressure)
    •  Serious PH: systolic pressure more than 50mmHg
  • Estimated systolic pressure by ultrasonic Doppler is approx. 10 mmHg higher than mean pressure at right catheter (Catheter: mild PH at 35mmHg, 36-50mmHg at Doppler). Ejection fraction (EF) is normally more than 55% when there is no heart failure

Cardiac catheter

Right catheterization with acute vasodilation test (reversibility test)

  • Definition: Pulmonary Hypertension when the Medium Arterial Pressure = MAP is above 20 mmHg at rest (revised from 25 mmHg per 2019, reference: Simonneau, 2019). MAP is usually 10-20 mmHg
  • PCWP (Mean pulmonary wedge pressure) are registered simultaneously.
    • Normal value is 8-12 mmHg.
    • PCWP is more than 15mmHg suggesting left-side heart disease as a cause of increased pulmonary arterial pressure, but 5% has a false pos value. It is also recommended to measure left ventricular pressure and diastolic pressure (LVEDP)
  • If MAP is more than 25 and PCWP less than 15, it indicates pulmonary hypertension, as we can see in systemic sclerosis.

Lung function tests

  • Often there is reduced DLCO, but normal FVC.
  • Ratio FVC% / DLCO% is thus increased.
    • Ratio values ​​more than 2 increase the possibility that pulmonary hypertension is present.
    • If there are pulmonary symptoms and a FVC% / DLCO% ratio less than 1,4, interstitial pulmonary disease is more likely

ECG (right axis deviation)

DETECT calculator (indication for right-hearted catheter examination?)

DETECT calculator can be used to estimate the need for investigation with

1) Echocardiography based on results of

  • Lung function tests (FVC% of expected / DLCO% of expected)
  • ECG (right-hand deviation)
  • Pro-BNP
  • Serum urate / uric acid
  • Anti-centromer / CENP antibody (expected in systemic sclerosis, limited form)
  • Teleangiectasia (at clinical examination)

2) To estimate indication of right-sided cardiac catheterization, results are added from echocardiography

  • Echocardiography: The area of ​​the right atrium (in square centimeter) and the rate of tricuspid valve backflow (TR speed im / s) are risk factors that are measured and included in the DETECT calculator

Incorrect diagnosis? (Similar diseases / differential diagnoses)


Most people use Marevan (Warfarin), but bleeding risk, for example, by systemic sclerosis may be contraindication

Endothelin antagonists:

  • Bosentan «Cipla» (Tracleer, Stayveer). Endothelin A + B inhibits
  • Bosentan / Tracleer / Stayveer: (62,5mg x 2 for 4 weeks, then 125 mg x2), by NYHA class II-III. Effect on 6-min walking test, O2 uptake, delays deterioration. Long-term effect. Known security profile. Bosentan ("Accord") written on H prescription and is covered by the health enterprise
  • interactions:
    • The dose of Marevan must be increased
    • Fluconazole (Diflucan) (anti-mycotics) significantly increases bosentan plasma levels
    • Do not give cyclosporin (Sandimmun) or glibenclamide (against diabetes)
    • Controls; liver enzymes: discontinue if 2-3x upper reference range. check hemoglobin
  • Ambrisentan (ET-A antagonist): Volibris, Endothelin A (ETA) -selective receptor antagonist, 5-10mg x1 in NYHA II-III. Side effects: Headache, edema, anemia (reactive?), Abdominal pain. Volibris is prescribed on H-prescription
  • Marcitentan (Opsumit) (Endothelin antagonists A + B inhibitors), 10mg x 1. As of 2019, Opsumit is more expensive than Volibris, but Volibris should not be given for pulmonary fibrosis (which is often present in systemic sclerosis and PAH). Opsumit is written on H-prescription
  • Check: Liver enzymes monthly, Hgb. Interactions: Marevan-neutral

PD-5 Inhibitors / Nitrogen Oxides

  • Sildenafil (20-80mg / d): Mysildecard, Orisild 20mg x 3 / Revatio / Viagra 25mg, / By NYHA II-III. Selective inhibitor CGMP-specific phosphodiesterase type 5 (PDE5). Orisild ("Orion") is written on H respet and is covered by the health enterprise
  • Tadalafil (Adcirca) 20mg or 40mg can be given once a day, but significantly more expensive (as of 2019) than Orisild
  • Riociguat, guanylate cyclase agonist

Prostacyclin analogues

  • Selexipag (Uptravi)
    • Tablets, selective IP agonist. Individual dose titration
  • Beriprost
  • iloprost
  • epoprostenol

Prescription on H-prescription (covered by the patient's health enterprise)

  • Bosentan «Accord»
  • Orisild «Orion»
  • Volibris
  • Opsumit

Norwegian HELFO is applied for approval on «blue prescription» in each case, paragraph 3a

Other treatment

  • Continuous iv prostacyclin (NYHA III-IV). (Epoprostenol = Flolan has shown survival gains. Dose: 15-50 ng / kg / min)
  • Prostaglandin antagonists (e.g., lioprost /Ilomedin). iv, sc (Treprostinil = Remodulin iv, sc. 20-60ng / kg / min), inhaled (iloprost / Ventavis), po: Beraprost (not shown effect> 3 months, not approved in Norway). NYHA III-IV
  • Combination Therapy
  • Balloon blockage of arteries
  • Lung transplantation
  • Calcium Antagonists (Nifedipine /Adalat): Less than 5% has response over time

Systemic sclerosis and PAH

  • 7-16% of patients with PAH (Limited more often than diffuse form). Disease mechanism (pulmonary hypertension in systemic sclerosis):
  • Pulmonary vasospasm (“pulmonary Raynaud's ”) -> endothelial proliferation, myofibroblast, smooth muscle - >> Pulmonary fibrosis
  • Endothelial receptor antagonist Volibris (ambrisentan) against PAH should not be used in pulmonary fibrosis. Opsumit (macitentane) is preferred in such cases.

Medical prognosis

Serious. Best prognosis for early diagnosis and treatment.


Pulmonary hypertension, BINDEVEVSSYKDOMMER.no

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