Pulmonary Hypertension (PH)
What is pulmonary hypertension?
Pulmonary hypertension is a serious clinical condition that should not be confused with arterial hypertension. In a patient with pulmonary hypertension the pressure in the pulmonary circulation (lungs) is increased so the right side of the heart (right ventricle) needs to work harder in order to effectively push the blood from the heart to the lungs. Over the years, the right ventricle begins to tire and becomes insufficient, resulting in worsening symptoms and clinical deterioration, leading to what we call “right heart failure”.
In many cases, the cause of pulmonary hypertension is unknown and this type of pulmonary hypertension is then called “idiopathic pulmonary arterial hypertension” (IPAH), formerly known as “primary pulmonary hypertension” (PPH). Idiopathic pulmonary arterial hypertension (IPAH) is a rare disease that affects more women than men.
If the cause of pulmonary hypertension is known, it is called “secondary pulmonary hypertension”. Causes of secondary pulmonary hypertension are: Congenital heart disease, blood clots in the lungs (pulmonary embolism), congestive heart failure, heart valve dysfunction, HIV infection, autoimmune diseases such as scleroderma and rheumatoid arthritis, low levels of oxygen in the blood for long time (years), chronic obstructive pulmonary disease or pulmonary fibrosis from pharmaceutical drugs (for example, certain slimming drugs) and obstructive sleep apnea.
The first and main symptom of pulmonary hypertension is shortness of breath. Other symptoms include dizziness, blackouts, oedema (swelling) and retrosternal pain, especially during exercise.
Treatment of the disease can be either medical (using very specific medications like Bosentan, Iloprost,Sildenafil), surgical or a combination of both. Treatment should be administered after the patient has been properly diagnosed by specialist doctors, in a specialist center.
Pulmonary hypertension can be treated by surgery. Every patient with unexplained pulmonary hypertension should be assessed for the presence of blood clots in the lungs. This condition is known as “chronic thromboembolic pulmonary hypertension” (CTEPH) and is diagnosed using a ventilation/perfusion scan (also called V/Q scan) and a CT angiography. A patient who is found to have a positive test(s) should be referred to a specialist center, such as our clinic, where expert medical and surgical treatment can be carried out.
Pulmonary endarterectomy is a surgical operation which involves removing the old blood clots which obstruct normal blood flow to the lungs. During the surgery the patient is connected to an ‘extracorporeal circulation machine’. This machine allows blood to flow around the body but to bypass the heart and lungs. The surgeon is then able to open the pulmonary arteries within the lungs and remove clots from within the pulmonary arteries.
A successful pulmonary endarterectomy can lead to a significant improvement in symptoms and, as a result, patients may experience a clear improvement in quality of life and improve their odds of attaining a normal life expectancy. In some cases however, despite an otherwise successful pulmonary endarterectomy, patients may retain some form of pulmonary hypertension and require lifelong therapy with special medications.
All patients who undergo a pulmonary endarterectomy should take the drug Warfarin (Coumadin, Sintrom) for the rest of their lives. This is to prevent the reoccurrence of blood clots within the pulmonary arteries.
Links:These, like any other published guidelines & evidence, do not and should not override the individual responsibility of healthcare professionals to make appropriate decisions in the circumstances of the individual patients, in consultation with that patient, and where appropriate and necessary the patient’s guardian or carer.
- 2022 ESC/ERS Guidelines for the diagnosis and treatment ofpulmonary hypertension
- ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association: developed in collaboration with the American College of Chest Physicians, American Thoracic Society, Inc., and the Pulmonary Hypertension Association. McLaughlin VV, Archer SL, Badesch DB, Barst RJ, Farber HW, Lindner JR, Mathier MA, McGoon MD, Park MH, Rosenson RS, Rubin LJ, Tapson VF, Varga J, Harrington RA, Anderson JL, Bates ER, Bridges CR, Eisenberg MJ, Ferrari VA, Grines CL, Hlatky MA, Jacobs AK, Kaul S, Lichtenberg RC, Lindner JR, Moliterno DJ, Mukherjee D, Pohost GM, Rosenson RS, Schofield RS, Shubrooks SJ, Stein JH, Tracy CM, Weitz HH, Wesley DJ; ACCF/AHA. Circulation. 2009 Apr 28;119(16):2250-94. Epub 2009 Mar 30. Review. No abstract available. Erratum in: Circulation. 2009 Jul 14;120(2):e13.
- Chronic thromboembolic pulmonary hypertension (CTEPH): updated Recommendations of the Cologne Consensus Conference 2011. Wilkens H, Lang I, Behr J, Berghaus T, Grohe C, Guth S, Hoeper MM, Kramm T, Krόger U, Langer F, Rosenkranz S, Schδfers HJ, Schmidt M, Seyfarth HJ, Wahlers T, Worth H, Mayer E. Int J Cardiol. 2011 Dec;154 Suppl 1:S54-60.
- Surgical management and outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry. Mayer E, Jenkins D, Lindner J, D’Armini A, Kloek J, Meyns B, Ilkjaer LB, Klepetko W, Delcroix M, Lang I, Pepke-Zaba J, Simonneau G, Dartevelle P. J Thorac Cardiovasc Surg. 2011 Mar;141(3):702-10.
- Surgical and post-operative treatment of chronic thromboembolic pulmonary hypertension. Mayer E. Eur Respir Rev. 2010 Mar;19(115):64-7. Review.
- Improved survival among patients with Eisenmenger syndrome receiving advanced therapy for pulmonary arterial hypertension. Dimopoulos K, Inuzuka R, Goletto S, Giannakoulas G, Swan L, Wort SJ, Gatzoulis MA. Circulation. 2010 Jan 5;121(1):20-5. Epub 2009 Dec 21.