Fainting, chest pain on exertion, or breathlessness at rest need urgent assessment today. Fainting in pulmonary hypertension is a serious warning sign. Air travel can also be unsafe with severe disease — ask before booking.
The pressure in the arteries carrying blood to the lungs rises, and the right side of the heart must work far harder to push against it. Eventually it fails.
For our patients this condition matters most because of what it decides. It determines whether a hole in the heart can still be closed, whether a narrowed mitral valve can still be treated, and whether an operation would help or cause harm.
Blood is pumped by the right ventricle through the lungs at a much lower pressure than the left ventricle generates for the body. When that lung circulation becomes stiff or obstructed, pressure rises, and the right ventricle — never designed for high pressure — thickens, dilates and eventually fails.
The causes divide into groups, and the distinction matters enormously. In the countries our patients travel from, two are especially common. Long-standing rheumatic mitral stenosis raises pressure backwards through the lungs; relieve the valve and the pressure often falls. And unrepaired holes in the heart send far too much blood through the lungs for years, until the lung arteries thicken permanently.
That second process leads to Eisenmenger syndrome. Once the lung arteries have remodelled to the point where their pressure exceeds the body's, blood flow through the hole reverses, the patient becomes blue, and closing the defect at that stage removes the pressure-relief valve and is actively harmful. It is irreversible, and it is the reason we press families not to postpone assessment of an unrepaired defect.
Pulmonary arterial hypertension can also occur without any of this — idiopathic, inherited, or associated with connective tissue disease, HIV or schistosomiasis, the last of which is relevant in parts of Africa and the Middle East.
For a patient with an unrepaired hole in the heart, or with long-standing rheumatic mitral stenosis, the level and reversibility of pulmonary pressure is the question on which everything turns. If the lung arteries are still responsive, closing the defect or relieving the valve is worthwhile and pressures usually fall. If they have remodelled permanently, closing the defect removes the escape route and can precipitate right heart failure. Echocardiography estimates the pressure but cannot answer the question properly — that requires right heart catheterisation with testing of how the circulation responds to a vasodilator. Any plan to close a shunt in an adult without that study is incomplete.
Echocardiography raises the suspicion; catheterisation confirms it and decides operability.
Echocardiography estimates pulmonary pressure indirectly and can be substantially wrong in either direction. Where the question is whether a defect can still be closed or a valve still treated, right heart catheterisation with vasodilator testing is required. Send the echocardiogram report with the estimated pressure, any catheterisation data if performed, your oxygen saturations, and details of any underlying defect and how long it has been known about.
Treating the cause comes first wherever a cause can be treated.
Relieving rheumatic mitral stenosis, closing a shunt while it is still safe to do so, treating left heart failure, removing chronic clot from the lung arteries, or treating sleep apnoea. Where the cause is treatable, pressures frequently fall substantially and this is by far the most effective intervention available.
Drugs that relax the lung arteries, used mainly in pulmonary arterial hypertension rather than in pressure caused by left heart disease, where they can be harmful. They improve symptoms and exercise capacity and, in combination, improve outcomes. They are expensive and require specialist supervision and monitoring.
Diuretics for fluid, oxygen where saturations are low, anticoagulation in selected groups, treatment of anaemia and iron deficiency, and careful management of pregnancy risk — pregnancy is dangerous in significant pulmonary hypertension and contraception advice is essential.
In established Eisenmenger syndrome, closing the defect is harmful rather than beneficial, and the correct management is medical treatment, careful supportive care and avoidance of situations that destabilise the circulation. We will say this plainly where it applies, rather than arrange a journey for an operation that cannot be performed.
Pressure caused by left heart disease is treated by fixing the left heart. Pulmonary arterial hypertension is treated with vasodilator drugs. Giving the wrong one causes harm.
Vasodilator testing during catheterisation answers this, and it determines whether closing a shunt or relieving a valve is still worthwhile.
How well the right ventricle is coping determines symptoms, prognosis and the safety of any procedure.
If catheterisation shows irreversible pulmonary vascular disease, we will tell you that surgery would not help and would likely cause harm. That answer is more useful than a quotation.
Severe pulmonary hypertension can make air travel unsafe, and pregnancy dangerous. Ask us before booking a flight and tell us immediately if you are pregnant.
Photographs taken on your phone are fine. Reports in Arabic, Russian or Bengali are fine — we translate them ourselves.
That depends entirely on the pulmonary pressure and whether it is still reversible. Where the lung arteries remain responsive, closure is worthwhile and pressures usually fall. Where they have remodelled permanently — Eisenmenger syndrome — closure removes the pressure-relief route and would cause harm. Right heart catheterisation with vasodilator testing gives the answer, and an echocardiogram alone does not.
The end result of years of excessive blood flow through the lungs from an unrepaired hole in the heart. The lung arteries thicken until their pressure exceeds the body's, flow through the hole reverses, and the patient becomes blue. It is irreversible, and it is why unrepaired defects should be assessed early rather than left.
For suspecting it, yes. For deciding an operation, no. Echocardiography estimates pressure indirectly and can be substantially wrong in both directions. Right heart catheterisation measures pressure and resistance directly and tests reversibility, and it is required before committing to closing a shunt in an adult.
Yes, including several pulmonary vasodilators at considerably lower cost than in Europe or North America. They are appropriate for pulmonary arterial hypertension confirmed on catheterisation. They can be harmful in pulmonary hypertension caused by left heart disease, which is why establishing the group first matters so much.
With mild disease, generally yes. With severe pulmonary hypertension, cabin altitude reduces available oxygen and can be genuinely dangerous, and assessment for in-flight oxygen should happen before booking rather than at the airport. Tell us your saturations and symptoms and we will advise honestly, including if the answer is not to travel.
Pregnancy carries a high risk in significant pulmonary hypertension and is generally advised against, because the circulatory changes of pregnancy and delivery are poorly tolerated. Effective contraception advice is an essential part of care for women of childbearing age with this condition, and it is frequently not discussed.
Send the echocardiogram with the estimated pulmonary pressure and any catheterisation data. For an unrepaired defect, that catheter study is what decides whether closure is still possible.
Your reports go directly to our medical team. We do not share your records with hospitals until you tell us to.
WhatsApp +91 83035 86344 · Phone +91 83035 86344 · Email tibhind@gmail.com
Coordinators available 9:00–20:00 IST. We speak Arabic, English, Russian and Bengali.