Key takeaways
- The early signs in children are quiet. Parents often notice poor feeding, slow weight gain, fast breathing, or a bluish tinge before anything else.
- In India, most childhood pulmonary hypertension is linked to a heart defect present from birth (congenital heart disease).
- Newborns can have a short-term form called PPHN, which is treated in a special care unit and often settles.
- Timing matters. Many heart defects can be closed with good results if treated early, before the lung arteries are damaged for good.
- Diagnosis starts with a painless echocardiogram, and is confirmed with a cardiac catheter test.
- With the right treatment, many children do well. Early referral to a paediatric heart specialist gives the best chance.
Children do not describe their symptoms the way adults do. A breathless adult will say so. A baby simply feeds poorly, tires, and falls behind on the growth chart. This is why pediatric pulmonary hypertension is often spotted late, and why the signs parents notice first matter so much.
This guide covers the full picture: the signs at each age, the causes, how it is diagnosed, how it is treated, and what the outlook looks like. If your child already has a diagnosis, you can read about pulmonary hypertension treatment and specialist care.
What is pulmonary hypertension in children?
Pulmonary hypertension in children is high blood pressure in the arteries that carry blood to the lungs. The right side of the heart then has to work much harder, and over time it tires. In children, the cause is usually a heart defect present from birth, which makes it different from the adult form.
The lung arteries are normally soft and open. In pulmonary hypertension they become narrow or stiff, so the heart must push harder to move blood through the lungs. Over months and years, this strain affects how a child grows, feeds, and plays.
The causes in children are not the same as in adults. In adults, pulmonary hypertension is usually linked to long-standing heart or lung disease. In children, the leading causes are congenital heart disease, an unknown cause called idiopathic PAH, and problems with lung development (pediatric PH review, 2024). Because the mix is different, children need care from a specialist who treats them specifically.
The signs parents notice first
In babies, the first signs are usually feeding trouble, sweating during feeds, fast breathing, poor weight gain, and a bluish tinge around the lips. In older children, the signs show up during play, as tiring quickly, blue spells, or fainting on exertion.
Because young children cannot explain what they feel, parents and carers are often the first to spot that something is wrong. The signs differ by age.
Signs of high lung pressure in babies
The clearest clue in a baby is that feeding becomes hard work. Watch for:
- Feeding difficulties, especially tiring or pausing often during feeds
- Sweating during feeds, sometimes with a damp head or neck
- Faster breathing than usual, even at rest
- Poor weight gain, or weight falling behind on the growth chart (sometimes called failure to thrive)
- A bluish tinge around the lips, tongue, or nails (cyanosis)
Signs in toddlers and older children
Older children can move around, so the signs appear during activity:
- Getting tired or breathless quickly during play
- Blue spells, where the lips or face turn dusky during crying or effort
- Fainting or near-fainting during exertion
- Complaints of chest pain or a racing heartbeat
- Being unusually quiet, or sitting out games other children manage
A single sign on its own is rarely a worry. But fainting during activity, or any blue spell, needs prompt medical review. These are not signs to watch and wait on.
Pulmonary hypertension in newborns (PPHN)
Pulmonary hypertension in newborns is called persistent pulmonary hypertension of the newborn, or PPHN. It happens when a baby’s lung circulation does not switch over normally after birth, so lung pressure stays high. It usually appears within hours of birth and is treated in a neonatal unit.
Before birth, a baby’s lungs are not used for breathing, so blood largely bypasses them. At birth, the lung arteries should open up and relax. In PPHN, they stay tight, so the baby cannot take in enough oxygen (Merck Manual, PPHN).
The main PPHN symptoms are fast breathing, grunting or chest pulling-in, and severe blue colouring that does not improve much even when oxygen is given. It is more common in babies born at term or past their due date.
Treatment is given in a neonatal intensive care unit. It may include oxygen, a calm environment, support with breathing, and a gas called inhaled nitric oxide that helps the lung arteries relax. In severe cases, a heart-lung bypass machine (ECMO) is used. Many babies recover well with timely care, though PPHN can be serious, so it is always treated as urgent.
What causes pulmonary hypertension in children?
The most common cause of pulmonary hypertension in children is congenital heart disease, especially holes or abnormal connections called shunts. Other causes include an unknown trigger (idiopathic PAH), problems with lung development such as in premature babies, and newborn PPHN.
Congenital heart disease is by far the leading cause in India. Many babies are born with a hole or an abnormal connection between the heart’s chambers or vessels, known as a shunt lesion. Common examples are a hole between the lower chambers (ventricular septal defect), a hole between the upper chambers (atrial septal defect), and an open vessel that should have closed after birth (patent ductus arteriosus). These let extra blood flow into the lungs. Over time, that extra flow and pressure can damage the lung arteries and cause pulmonary hypertension.
The Indian data is clear on how common this is. In the PULMOEAST study from eastern India, congenital heart disease was the single biggest cause of pulmonary hypertension, with shunt lesions present in nearly 59% of patients. A paediatric pulmonary hypertension registry near Mumbai found the same pattern, with about 81% of 100 children having pulmonary hypertension linked to congenital heart disease.
The other main causes are:
| Cause | What it is |
|---|---|
| Congenital heart disease | Holes and shunts present from birth that send extra blood to the lungs |
| Idiopathic PAH | High lung pressure with no identifiable cause, which tends to be more serious |
| Developmental lung disease | Underdeveloped lungs and vessels, often in babies born very early (for example, bronchopulmonary dysplasia) |
| PPHN | The short-term newborn form described above |
| Inherited PAH | A rare form caused by a gene change that can run in families |
Why early treatment matters so much
When a heart defect is closed early, the lung arteries often recover and the child can do very well. If it is left too long, the damage can become permanent, the shunt can reverse, and surgery is no longer safe. This is why early referral changes outcomes.
In a child with a large shunt, blood floods the lungs at high pressure. For a while, closing the hole can fix the problem completely. Early closure of the shunt is the best way to prevent lasting lung-artery damage. Many children with large defects can be effectively cured if they are operated early in life.
If the defect is left too long, the lung arteries stiffen, the pressure keeps rising, and eventually blood starts flowing the wrong way through the hole. This advanced stage is called Eisenmenger syndrome. At that point the child turns blue more easily, and closing the defect is no longer safe. The window for a straightforward repair has closed.
This is not a rare, distant risk in India. In the Mumbai paediatric registry, nearly half of the children had already reached Eisenmenger syndrome by the time they were seen, which points to how often these children are referred late. The message for parents is simple: a known heart defect should never sit on a long “watch and see” plan without a specialist’s advice.
How pulmonary hypertension in children is diagnosed
Diagnosis starts with an echocardiogram, a painless scan that estimates lung pressure. If it suggests pulmonary hypertension, the specialist usually confirms it with a cardiac catheter test, which measures the pressure directly and shows whether a defect can still be safely closed.
An echocardiogram uses sound waves to look at the heart and estimate the pressure in the lung arteries. It is safe for babies and children and is the usual first step. You can read what an echo report means in our guide to echocardiography for children with suspected PH.
If the echo points to raised pressure, the specialist often advises a cardiac catheterisation. A thin tube is passed into the heart to measure the lung pressure and resistance directly. In children with a shunt, this test is important because it shows whether the defect can still be closed safely. The team may also do tests to find the exact cause, such as blood tests and scans. The aim at every step is the same: confirm the diagnosis, find the cause, and decide what can be treated.
How pediatric pulmonary hypertension is treated
Pediatric pulmonary hypertension treatment has two parts: treating the cause, and easing the pressure in the lungs. Where a heart defect can be safely closed, that is often the main fix. Alongside this, children may need medicines that relax the lung arteries, oxygen, and in severe cases, specialist procedures.
Treatment is always built around the child and the cause. The main approaches are:
| Approach | What it does |
|---|---|
| Treating the cause | Closing a shunt, where it is still safe to do so, can greatly improve or resolve the pressure |
| PH medicines | Drugs such as sildenafil and tadalafil, or bosentan and similar medicines, help relax and open the lung arteries |
| Oxygen | Supports children whose oxygen levels drop, used as the specialist advises |
| Procedures for severe disease | For end-stage cases, options such as a Potts shunt or, rarely, lung transplant may be considered |
| Newborn care (PPHN) | Oxygen, inhaled nitric oxide, and breathing support in a neonatal unit |
For children with a shunt who are close to the limit of safe closure, specialists sometimes use a “treat and repair” approach. Here, PH medicines are started first to improve the lung arteries, and closure is reconsidered later. Decisions like these are made only after a cardiac catheter test and careful specialist review.
One honest point matters. For some forms, especially idiopathic PAH, pulmonary hypertension cannot be fully cured, but it can be managed well, and modern medicines have improved how children live with it. For many congenital cases caught early, the outlook is far better.
The outlook for children with pulmonary hypertension
The outlook depends on the cause and how early it is treated. Children whose heart defect is closed early often do very well. Idiopathic cases are more serious but are helped by modern treatment. Across the board, early diagnosis gives the best chance of a good result.
A child who is treated before the lung arteries are badly damaged has the best outlook, and in many congenital cases the problem can be resolved. A child referred late, after Eisenmenger syndrome has set in, faces a harder road, though treatment can still improve comfort and quality of life.
This is why parents should act on early signs rather than wait. The difference between an early referral and a late one can be the difference between a treatable defect and a lifelong condition.
When should you see a paediatric cardiologist?
See a paediatric cardiologist if your child shows signs of high lung pressure, or if a known heart defect is causing symptoms. Early referral is a safety step, not a sign of bad news. Most children referred early turn out to have a treatable problem.
Reasons to seek a referral include:
- A heart murmur with poor feeding or slow weight gain
- Breathlessness, blue spells, or fainting during activity
- A known congenital heart defect that seems to be getting worse
- An echocardiogram that suggests raised lung pressure
Parents can also ask for a referral themselves. If something about your child’s breathing, feeding, or energy worries you, it is reasonable to request a paediatric cardiology opinion. For adults with similar symptoms, see our guide to pulmonary hypertension symptoms in adults.
Getting your child the right care early
Childhood pulmonary hypertension is easy to miss, because the early signs look like everyday feeding or tiredness issues. Yet it is also a condition where early action can change everything. Caught in time, many heart defects can be repaired with excellent results.
Dr Prashant Bobhate is a paediatric cardiologist who focuses on pulmonary hypertension in children, from newborns with PPHN to older children with congenital heart disease. He offers full assessment, including echocardiography and cardiac catheterisation, and plans treatment around each child’s cause and stage. If your child shows any of the signs in this guide, or has a heart defect that worries you, book a consultation for a clear, expert opinion.
References
- Pulmonary hypertension in pediatrics: from clinical suspicion to management. European Journal of Pediatrics, 2024. https://pmc.ncbi.nlm.nih.gov/articles/PMC11982064/
- Persistent Pulmonary Hypertension of the Newborn (PPHN). MSD/Merck Manual, Professional. https://www.merckmanuals.com/professional/pediatrics/respiratory-problems-in-neonates/persistent-pulmonary-hypertension-of-the-newborn-pphn
- Singhi AK, et al. PULMOEAST: A Comprehensive Analysis of Pulmonary Hypertension in Eastern India. Cureus, 2023. https://pubmed.ncbi.nlm.nih.gov/38205444/
- Pediatric pulmonary hypertension registry: Single-center experience from India. Annals of Pediatric Cardiology, 2024. https://pubmed.ncbi.nlm.nih.gov/42112068/
- Pulmonary Hypertension in Congenital Shunts. Revista Española de Cardiología. https://www.revespcardiol.org/en-pulmonary-hypertension-in-congenital-shu-articulo-S1885585710702322