Pulmonary hypertension (PH)
Pulmonary hypertension is raised blood pressure in the pulmonary arteries, the vessels that carry blood from the heart to the lungs.
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Medicines commonly prescribed for Pulmonary hypertension (PH)
For informational purposes only. Always consult a doctor before using any medicine.
Dosage form: TABLET, 100 mg of sildenafilActive substance: sildenafilManufacturer: Krka D.D. Novo MestoPrescription requiredDosage form: TABLET, 50 mg/tabletActive substance: sildenafilManufacturer: Kern Pharma S.L.Prescription requiredDosage form: TABLET, 10 mgActive substance: ambrisentanManufacturer: Glaxosmithkline (Ireland) LimitedPrescription required
Pulmonary hypertension is raised blood pressure in the pulmonary arteries, the vessels that carry blood from the heart to the lungs. It has nothing to do with the blood pressure measured on your arm: a cuff will not detect it and a routine check-up will not show it. The walls of these vessels thicken and stiffen, the channel narrows, and the right side of the heart has to work against steadily rising resistance. It copes for years and then begins to fail — and that is where the danger of this condition lies.
Why the diagnosis arrives so late
The chief problem with pulmonary hypertension is not that it is serious but that nobody looks for it. Two or three years often pass between the first complaints and the correct diagnosis, and throughout that time the person carries a label: asthma, bronchitis, excess weight, being out of condition, or nerves.
The reason is that the only early sign — breathlessness on exertion — belongs to dozens of far commoner conditions. What sets pulmonary hypertension apart is not the type of breathlessness but its history: it builds over months and without flare-ups. Not in attacks, as in asthma, and not tied to a cold, but steadily and relentlessly: first the hill is hard, then your own staircase, then walking on the flat. Inhalers do not help, and that is an important clue which is usually overlooked.
Other complaints appear alongside it:
- tiredness out of all proportion to what has been done;
- dizziness and darkening vision during exertion;
- pain or pressure behind the breastbone on effort;
- an awareness of the heartbeat;
- swelling of the ankles and lower legs and, later, a swollen abdomen from fluid;
- a dry cough, a bluish tinge to the lips and nails, prominent neck veins.
Fainting during exertion deserves a separate mention. Losing consciousness while running, climbing stairs, bending down or straining is not low blood pressure and not a stuffy room. It means the heart cannot increase its output when asked to, and in pulmonary hypertension this is regarded as one of the most worrying signs. It calls for investigation within days, not at some point.
When you cannot wait for an appointment
Call an ambulance — in Spain, Italy, Portugal, Poland and Ukraine the single European number is 112 — or go straight to an emergency department if:
- there has been a faint, or someone nearly lost consciousness;
- severe chest pain comes on and does not settle within a few minutes;
- breathlessness worsens sharply over hours, or appears at rest;
- the lips and fingertips turn blue and the skin is cold and clammy;
- the heartbeat is fast and irregular with dizziness or chest pain.
These signs occur both in known pulmonary hypertension and in conditions that imitate it: a pulmonary embolism, a heart attack, an abnormal heart rhythm. This is not something to work out at home.
Separately, but also urgently — the same day, not next week: swelling that is growing quickly, a two-kilogram weight gain over a few days, and a waistband that has suddenly become tight. These indicate that the right side of the heart is starting to lose the battle.
Why the pressure in the lung vessels rises
Pulmonary hypertension is not one disease but the shared endpoint of several different processes, and which one a person has determines the treatment entirely. Five broad groups are recognised.
- Pulmonary arterial hypertension, where the smallest branches of the pulmonary arteries are affected and their walls thicken. It may be inherited, idiopathic — meaning no cause is found — or linked to connective tissue disease (above all scleroderma), congenital heart defects, HIV, portal hypertension from liver disease, and certain medicines and recreational drugs.
- Disease of the left side of the heart: valve problems, or a weakened or stiff left ventricle. Blood backs up before the lungs and the pressure rises passively. This is the commonest group.
- Lung disease and lack of oxygen: chronic obstructive pulmonary disease, pulmonary fibrosis, severe sleep apnoea, long-term life at high altitude. Low oxygen in itself makes the lung vessels constrict.
- The aftermath of a clot: emboli that never dissolve turn into scar tissue and block the vessels permanently. This is chronic thromboembolic pulmonary hypertension, covered separately below.
- Other causes: sarcoidosis, vasculitis, thyroid disease, sickle cell disease and thalassaemia, chronic kidney disease, and compression of the vessels by a tumour.
The question of cause is therefore not academic. The answer decides whether a valve is treated, whether oxygen is prescribed, whether surgery on the pulmonary arteries is on the table, or whether specific drugs are used — drugs that in some of these groups do not help but cause harm.
How the diagnosis is reached
The path starts at an ordinary appointment: the doctor listens to the heart and lungs, looks at the neck veins and any swelling, and arranges an ECG, a chest X-ray, blood tests and lung function tests.
Next comes an echocardiogram, and its role is worth understanding. An echocardiogram does not measure the pressure in the pulmonary artery; it estimates it indirectly from the flow across the tricuspid valve, and it errs in both directions, overestimating in some people and missing the condition in others. It answers the question of whether to keep looking, not whether the disease is present. Pulmonary hypertension is neither diagnosed nor ruled out on a scan alone.
The diagnosis is confirmed by right heart catheterisation: a fine catheter is passed through a vein in the neck, arm or groin up to the pulmonary artery, and the pressure is measured directly. Cardiac output and wedge pressure are recorded at the same time, which separates pulmonary arterial hypertension from congestion caused by the left side of the heart — two situations treated very differently. Where pulmonary arterial hypertension is suspected, vasoreactivity testing is carried out during the same procedure.
One further test should be done in every case: a ventilation-perfusion scan. Its purpose is to avoid missing the thromboembolic form, the only one that can be cured by an operation. CT scanning misses this form, so it cannot take the place of the perfusion scan. To these are added a six-minute walk test and a blood test for natriuretic peptide, which measure severity and give a baseline against which treatment is judged.
Why treatment belongs in a specialist centre
This is a condition where where you are treated matters as much as how. The specific drugs for pulmonary arterial hypertension are selected, combined and adjusted on the basis of repeated measurements; some require continuous intravenous or subcutaneous infusion, some carry serious contraindications, and choosing the wrong group can make a person worse. Those decisions are made in centres that do this work constantly, not in a general clinic.
General treatment includes diuretics for fluid retention, oxygen when blood levels are low, anticoagulants in the thromboembolic form, and treatment of whatever underlying disease started it all.
The specific drugs act on three vessel-widening pathways: endothelin receptor antagonists, phosphodiesterase 5 inhibitors and soluble guanylate cyclase stimulators, along with prostacyclin-based treatments ranging from tablets to continuous infusion in severe cases. They are given in combination and only once the form of the disease has been established.
Calcium channel blockers need a warning of their own. They are sometimes listed as a routine treatment for pulmonary hypertension, and that is a dangerous simplification. They suit only a small minority of people with pulmonary arterial hypertension — those whose vasoreactivity test during catheterisation was positive. Outside that narrow group, and especially in left-sided heart disease, they can cause a sharp deterioration. They must not be prescribed on the strength of the diagnosis alone.
The form that can be cured
Chronic thromboembolic pulmonary hypertension stands apart: it is the one variant where the disease can be removed rather than merely held in check. It develops in a proportion of people who have had a pulmonary embolism, sometimes months later and sometimes years, and often after an episode that went entirely unnoticed at the time.
The main treatment is pulmonary endarterectomy, an operation in which the scarred clot material is stripped out of the pulmonary arteries. It is demanding and performed in only a few centres, but in suitable patients it brings lasting improvement, sometimes with the pressure returning to normal. Where the position of the blockages rules out surgery, balloon pulmonary angioplasty is used — the narrowed segments are widened with a balloon on a catheter, usually across several sessions — together with drug treatment. Anticoagulants are taken for life in this form.
The practical conclusion is simple: if breathlessness is still there three to six months after a pulmonary embolism, say so and ask for an echocardiogram rather than putting it all down to weakness after an illness.
Living with the diagnosis
- Movement is possible and necessary, within limits agreed with your doctor. Steady aerobic activity and supervised rehabilitation help; lifting weights, straining and pushing on until you are breathless or dizzy do not.
- Pregnancy in pulmonary arterial hypertension is life-threatening and in most cases advised against, so reliable contraception is discussed as soon as the diagnosis is made. Combined hormonal methods do not suit everyone and interact with some of the drugs, so the choice is made with a doctor. If pregnancy does occur, seek advice immediately.
- Influenza and pneumococcal vaccination are worthwhile: an ordinary chest infection is harder to weather with this condition.
- Be careful with altitude and flying. Mountains above two thousand metres and cabin depressurisation are not for these patients; before a long journey it is worth discussing oxygen in flight.
- Many over-the-counter products are not harmless here. Decongestant drops and cold remedies, slimming products, some painkillers and any nitrate taken alongside a phosphodiesterase inhibitor can cause blood pressure to plummet. Ask before you buy.
- Weigh yourself daily: a rapid gain is the earliest sign of fluid building up.
Online consultation
An online consultation is most valuable exactly where this condition tends to get lost: at the stage when breathlessness is already limiting life and nobody has explained it. The doctor will go through how the breathlessness developed, what it relates to, whether inhalers helped and whether there have been faints on exertion, and will advise which tests to ask for — starting with an echocardiogram.
It is also worth booking if the diagnosis is already known: to make sense of your drug regimen and its interactions, to interpret follow-up results, to discuss how much exertion is safe, travel, pregnancy and contraception, and to prepare for planned surgery, since anaesthesia in pulmonary hypertension calls for particular care.
Have your hospital letters, echocardiogram and catheterisation reports, a list of medicines with doses and, if you have one, your six-minute walk test result to hand. If anything in your account points to an emergency, you will be directed there straight away.
This material is for information only and does not replace medical advice.
Consult with a doctor about Pulmonary hypertension (PH)
Discuss your symptoms and possible next steps with a doctor online.
Online doctors for Pulmonary hypertension (PH)
Discuss your symptoms and possible next steps for Pulmonary hypertension (PH) with a doctor online.
