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Pulmonary hypertension: diagnosis and treatment in Bangalore

Raised pressure in the lung arteries has five quite different causes, and almost everything about treatment depends on which one a patient has. Dr. Manjunath M Negigoudara assesses and treats pulmonary hypertension at KIMS Hospital, Electronic City, Bengaluru, and has published Indian data on pulmonary hypertension in fibrotic lung disease in the European Respiratory Journal.

Medically reviewed Written and reviewed by Dr. Manjunath M Negigoudara, Transplant Pulmonologist, KIMS Hospital, Electronic City, Bengaluru. KMC 116911. Last reviewed · 4 peer-reviewed sources

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What is pulmonary hypertension?

The right side of the heart pumps blood through the lungs at a pressure far lower than the left side uses for the rest of the body. When that pressure rises, the right ventricle has to work against resistance it was never built for. It copes for a while by thickening, then it begins to dilate, and eventually it fails. Most of what we call the symptoms of pulmonary hypertension are really the symptoms of a right ventricle under strain.

The 2022 ESC/ERS guidelines define pulmonary hypertension as a mean pulmonary artery pressure above 20 mmHg measured directly at catheterisation. The threshold was lowered from 25 mmHg in 2022, which brought a group of patients with milder elevation into the diagnosis, and those patients need identifying because their outcomes are worse than people with normal pressures.

The delay is the problem

Pulmonary hypertension is characteristically diagnosed late, because its first symptom is exertional breathlessness and that gets attributed to asthma, weight, anaemia or being out of shape. Unexplained breathlessness that has been investigated without an answer, particularly with blackouts on exertion, deserves an echocardiogram specifically looking at the right heart.

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What are the five groups of pulmonary hypertension?

The five groups of pulmonary hypertension and the treatment for each: group 1 pulmonary arterial hypertension, group 2 left heart disease, group 3 lung disease and hypoxia, group 4 chronic thromboembolic disease which is potentially curable, and group 5 multifactorial
The five groups and what each is treated with. Group 4 is the only potentially curable form, and a V/Q scan is what finds it.

This is the part patients are rarely told clearly, and it is the most consequential thing on this page. Pulmonary hypertension is classified into five groups by cause. The expensive targeted drugs are proven in group 1. In some of the other groups they do not help, and in group 3 they can actively make oxygen levels worse.

Group Cause What treatment looks like
1 Pulmonary arterial hypertension Disease of the small lung arteries themselves. Idiopathic, heritable, connective tissue disease, congenital heart disease, drug induced. Targeted combination therapy, escalated by risk category. The group where modern drugs have transformed survival.
2 Left heart disease The commonest cause overall. Pressure backs up from a failing or stiff left ventricle or a diseased valve. Treat the heart. Targeted pulmonary arterial hypertension drugs are not indicated and may cause harm.
3 Lung disease and hypoxia Interstitial lung disease, COPD, combined fibrosis and emphysema, sleep-disordered breathing, high altitude. Treat the lung disease, correct hypoxia. Inhaled treprostinil has evidence specifically in interstitial lung disease. Marks a worse trajectory and argues for earlier transplant referral.
4 Chronic thromboembolic Old pulmonary emboli that organised instead of clearing. Potentially curable. Pulmonary endarterectomy surgery or balloon pulmonary angioplasty, with lifelong anticoagulation.
5 Multifactorial Sarcoidosis, haematological disorders, chronic kidney disease, metabolic disease. Directed at the underlying condition. Managed case by case.

Which type of pulmonary hypertension is curable?

Group 4, chronic thromboembolic pulmonary hypertension, is the only potentially curable form, and it is regularly missed. The reason is that the test which finds it is not the test most people get. A CT pulmonary angiogram can look unremarkable in chronic thromboembolic disease. A ventilation-perfusion scan is far more sensitive for it, and the ESC/ERS guidelines state that every patient with unexplained pulmonary hypertension should have one.

Many patients with this condition never had a diagnosed clot. The original event was silent, or was treated as a chest infection. If you have been told you have pulmonary hypertension and have not had a V/Q scan, that is worth asking about directly, because the difference between a curable and a lifelong illness turns on it.

How is pulmonary hypertension diagnosed?

Echocardiogram as the screening test

An echocardiogram estimates pulmonary pressure from the speed of a regurgitant jet across the tricuspid valve. It is indirect, it can be technically difficult, and it both over-calls and under-calls the diagnosis. Its real value is in assessing right ventricular size and function, and in looking for left-sided heart disease that would place the patient in group 2. A raised estimated pressure on echocardiography is a reason to investigate, not a diagnosis.

Right heart catheterisation as the standard

This is the only test that measures pressure directly. It provides mean pulmonary artery pressure, pulmonary artery wedge pressure and pulmonary vascular resistance, and that combination is what separates group 1 from group 2 and establishes severity. No patient should be committed to long-term targeted therapy on the basis of an echocardiogram alone.

Finding the cause

Alongside catheterisation, the workup includes a V/Q scan to exclude chronic thromboembolic disease, high-resolution CT of the chest for lung disease, full pulmonary function testing with DLCO, an autoimmune panel, an overnight sleep study where sleep-disordered breathing is suspected, a six-minute walk test, and blood natriuretic peptide levels for risk assessment and monitoring.

Pulmonary hypertension in fibrotic lung disease

This is the group Dr. Manjunath has published on. His 2023 paper in the European Respiratory Journal, Pulmonary hypertension in patients with Progressive Fibrosing Interstitial Lung Disease (PF-ILD) referred for Lung Transplantation, a retrospective study from India, examined exactly this population in an Indian transplant referral setting. Details are on the publications page.

The clinical message is consistent. When pulmonary hypertension appears in someone with fibrotic lung disease, it marks a change in trajectory. Breathlessness becomes disproportionate to the extent of scarring on the scan, oxygen requirements climb, walk distance falls, and the prognosis worsens. Its presence is one of the clearest arguments for bringing forward a transplant assessment rather than continuing to observe.

It also means the treatment approach differs from group 1. The one exception with trial evidence is inhaled treprostinil, which improved exercise capacity in the INCREASE trial in pulmonary hypertension associated with interstitial lung disease. Otherwise the instinct to reach for pulmonary arterial hypertension drugs has to be resisted, because in diseased lung they can increase blood flow to areas that are not ventilating and drop oxygen saturation further. Related reading: interstitial lung disease and pulmonary fibrosis and IPF.

How is pulmonary hypertension treated?

For group 1 pulmonary arterial hypertension, treatment is risk-based and usually begins with combination therapy rather than a single drug, escalating to a prostacyclin agent when a patient remains at high risk. Patients are re-assessed at intervals using walk distance, natriuretic peptide levels, echocardiographic right ventricular function and functional class, and therapy is stepped up when they are not in a low-risk category.

Alongside drug treatment: supervised exercise training improves capacity, supplemental oxygen is used where saturation falls, anticoagulation is decided case by case except in chronic thromboembolic disease where it is lifelong, pregnancy carries very high risk and needs explicit counselling, and vaccination against influenza and pneumococcus matters more than in the general population.

When does pulmonary hypertension need a lung transplant?

For pulmonary arterial hypertension, referral is indicated when a patient stays in a high-risk category despite appropriate combination therapy including a prostacyclin agent, or when right heart failure is progressing, per the ISHLT 2021 consensus. Pulmonary arterial hypertension patients can deteriorate quickly, so referral is made while the patient is still able to complete an assessment.

For pulmonary hypertension complicating lung disease, the presence of pulmonary hypertension is itself the trigger to bring forward assessment. Where right heart failure is severe and a patient is deteriorating while waiting, ECMO can be used as a bridge. Read about lung transplantation and what it costs in Bangalore.

Consulting at KIMS Electronic City

Bring your echocardiogram report, any catheterisation report, HRCT images, pulmonary function tests with DLCO, V/Q scan if done, recent blood work and a full medication list. KIMS Hospital, Survey No. 37 and 38, PES University EC Campus, Hosur Road, Konappana Agrahara, Bengaluru 560100. Monday to Saturday, 9 AM to 5 PM, with evening and video consultations by prior appointment. Call or WhatsApp +91 79937 41199.

For patients from Bangalore, across India, and abroad

Bengaluru and Electronic City

The hospital is on Hosur Road opposite the PES University EC Campus, about 15 minutes from Silk Board via the elevated expressway. Patients attend from HSR Layout, Koramangala, Sarjapur Road, BTM Layout, Jayanagar, JP Nagar, Bannerghatta Road, Whitefield and Marathahalli, and from Bommasandra, Anekal, Chandapura and Attibele.

Karnataka and the rest of India

Referrals come from Mysuru, Mangaluru, Hubballi-Dharwad, Belagavi, Kalaburagi, Shivamogga, Tumakuru, Davangere and Ballari, and from Chennai, Coimbatore, Madurai, Salem, Hyderabad, Warangal, Vijayawada, Visakhapatnam, Tirupati, Kochi, Thiruvananthapuram, Kozhikode, Goa, Mumbai, Pune, Nagpur, Ahmedabad, Delhi NCR, Kolkata, Bhubaneswar, Guwahati, Lucknow, Patna, Ranchi and Raipur. Because pulmonary hypertension workup involves several tests, send your existing reports on WhatsApp first so the visit can be planned and duplicate testing avoided. Kempegowda International Airport is about 45 km away.

International patients

Patients are seen from Bangladesh, Nepal, Sri Lanka, the Maldives and Bhutan, from Oman, the UAE, Saudi Arabia, Iraq, Yemen, Kuwait, Bahrain and Qatar, from Kenya, Tanzania, Ethiopia, Uganda, Rwanda, Sudan, Nigeria and Ghana, and from Myanmar, Indonesia and Malaysia. The pathway is remote review over WhatsApp, a written opinion that can support a medical visa application, a three to four day evaluation visit including catheterisation where indicated, and video follow-up from home.

Related reading

References

The clinical statements on this page are drawn from the peer-reviewed literature and international guidelines listed below. Each link opens the source on PubMed.

  1. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. European Heart Journal, 2022. PMID 36017548.
  2. Inhaled Treprostinil in Pulmonary Hypertension Due to Interstitial Lung Disease. New England Journal of Medicine, 2021. PMID 33440084.
  3. Ventilation-perfusion scintigraphy is more sensitive than multidetector CTPA in detecting chronic thromboembolic pulmonary disease as a treatable cause of pulmonary hypertension. Journal of Nuclear Medicine, 2007. PMID 17475953.
  4. Consensus document for the selection of lung transplant candidates: An update from the International Society for Heart and Lung Transplantation. Journal of Heart and Lung Transplantation, 2021. PMID 34419372.

Frequently asked questions

Which doctor treats pulmonary hypertension in Bangalore?
Pulmonary hypertension sits between cardiology and pulmonology, and the group a patient falls into decides which specialty leads. Dr. Manjunath M Negigoudara assesses and treats pulmonary hypertension at KIMS Hospital, Electronic City, Bengaluru, with a particular focus on pulmonary hypertension complicating lung disease. He published Indian data on this exact group in the European Respiratory Journal in 2023. WhatsApp +91 88670 59997, Monday to Saturday, 9 AM to 5 PM.
What are the symptoms of pulmonary hypertension?
Breathlessness on exertion is almost always first and is the reason the diagnosis is so often delayed, since it is easily attributed to asthma, weight or being unfit. Other features are fatigue, light-headedness or blackouts on exertion, chest tightness, palpitations, and swelling of the ankles and abdomen once the right side of the heart begins to struggle. Blacking out on exertion is a red flag that warrants urgent assessment.
Can pulmonary hypertension be cured?
One form can. Chronic thromboembolic pulmonary hypertension, caused by old blood clots that never cleared, is potentially curable by pulmonary endarterectomy surgery or balloon pulmonary angioplasty. It is the single most important diagnosis not to miss, and the test that finds it is a V/Q scan. Other forms are not curable but are treatable, and for pulmonary arterial hypertension modern combination therapy has changed outcomes substantially.
Is an echocardiogram enough to diagnose pulmonary hypertension?
No. An echocardiogram is the screening test and it estimates pressure indirectly, which means it both over-calls and under-calls the diagnosis. Right heart catheterisation is the only test that measures pressure directly and it remains the diagnostic standard. It also measures pulmonary vascular resistance and wedge pressure, which is what separates the treatment groups from one another.
What is the difference between pulmonary hypertension and pulmonary arterial hypertension?
Pulmonary hypertension is the broad finding of raised pressure in the lung arteries, from any of five distinct causes. Pulmonary arterial hypertension, group 1, is one specific disease of the small lung arteries themselves. The distinction matters enormously because the expensive targeted drugs are proven in group 1 and can be harmful in some of the other groups.
Does pulmonary hypertension in lung fibrosis get treated with the same drugs?
Generally no, and this is one of the most common errors in practice. When pulmonary hypertension is caused by lung disease, it falls into group 3, where the standard pulmonary arterial hypertension drugs have not shown benefit and can worsen oxygen levels by increasing blood flow to poorly ventilated lung. The exception is inhaled treprostinil, which has evidence in pulmonary hypertension associated with interstitial lung disease. The priority in group 3 remains treating the underlying lung disease and correcting hypoxia.
When does pulmonary hypertension need a lung transplant?
For pulmonary arterial hypertension, referral is indicated when a patient remains in a high-risk category despite appropriate combination therapy including a prostacyclin agent, or when right heart failure is progressing. For pulmonary hypertension complicating lung disease, its presence is itself an argument for earlier transplant referral, because it consistently marks a worse trajectory. See who needs a lung transplant.
Can I have my case reviewed remotely before travelling?
Yes. WhatsApp your echocardiogram report, any right heart catheterisation report, HRCT images, pulmonary function tests with DLCO, V/Q scan if performed, and current medication list to +91 88670 59997. A written opinion covers which group your pulmonary hypertension belongs to, whether the workup is complete, whether treatment matches the group, and whether transplant assessment is warranted. See the second opinion page.

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