Primary pulmonary hypertension typically shows up in middle age. Secondary pulmonary hypertension associated with diseases like scleroderma is also usually diagnosed in adults. But pulmonary hypertension can also occur in infants and children.

There are a variety of causes. In infants, a condition called persistent pulmonary hypertension of the newborn is found in one out of 700 births. In such cases, a baby's circulatory and respiratory systems fail to make the normal transition at birth. In the womb, the baby's lungs aren't needed to get oxygen; the placenta is the fetal organ or gas exchange via the umbilical cord. In utero, a fetus's pulmonary artery sends blood back to the heart and bypasses the lungs. At birth, the pulmonary artery normally begins pumping blood into the lungs so the baby can begin breathing. When this switch-over fails to happen, the result can be persistent pulmonary hypertension of the newborn, or PPHN. This condition is associated with other respiratory diagnoses like pneumonia or meconium in the majority of infants.

Pulmonary hypertension can occur in infants and young children for other reasons as well. A variety of congenital defects in the structure of the heart or its valves can cause elevated blood pressure in the pulmonary arteries, for instance. In rare cases, infants and young children develop primary pulmonary hypertension, the form of the disease that is not linked to any underlying disease or defect. As with the adult-onset version, researchers don't fully understand what causes primary pulmonary hypertension in children. An inherited gene has been linked to some cases of PPH. Researchers suspect that patients with the condition have cells in the lining of their artery walls that are unusually sensitive to certain factors.

Whatever the cause, pulmonary hypertension in children poses special challenges. In the young, the disease often progresses more rapidly than in middle-aged patients. And while many of the drugs that are proving to be effective in controlling symptoms in adults also work well in children, few controlled studies have been done.

The symptoms of abnormal pulmonary blood pressure in infants and children are similar to those in adults. They include:

•Lethargy

•Irregular heartbeat

•Labored breathing

•Sweating

•Nausea or vomiting

•Poor appetite

•Poor growth

•Shortness of breath when exercising

•Dizziness or fainting spells

•Chest pain

•A bluish tinge to the skin from lack of oxygen (cyanosis)

A physical examination by a doctor may turn up other signs of pulmonary hypertension. These include unusual heartbeat sounds sometimes associated with the disease, subtle swelling in the extremities, or an enlarged liver.

Many of the same tests employed to diagnose pulmonary hypertension in adults are used with infants and children when doctors strongly suspect pulmonary hypertension. These include chest X-ray, EKG, arterial blood gas, echocardiograms, and cardiac catheterization. Doctors often perform tests to determine if the cause of PH in children is sleep apnea, a condition caused when breathing is interrupted during sleep.

Infants suffering from persistent pulmonary hypertension of the newborn (PPHN) are placed in a neonatal intensive care unit so that they can be monitored and supplied with supplemental oxygen that widens the pulmonary arteries. In some cases, the problem resolves on its own. Other drugs that widen the pulmonary artery, such as nitric oxide, have been shown to be helpful in newborns. In serious cases, surgery may be necessary. Serious treatment is available for several of the associated lung diseases.

Children diagnosed with pulmonary hypertension are treated with many of the same drugs used to treat adults. These include calcium channel blockers, prostacyclins, endothelin-1 inhibitors, and blood-thinning agents. Children with PH sometimes require supplemental oxygen either during the day or while they sleep.

Children with pulmonary hypertension must be followed closely by medical specialists. Because the condition is uncommon, that may require travel to a large medical center that specializes in the disease.

Along with treatment for the condition itself, it is important to keep children as healthy as possible to avoid unnecessary strain on the heart and lungs. Doctors recommend an annual influenza vaccination, as well as preventive treatment with palivizumab, which helps protect against respiratory syncytial virus (RSV), a virus hazardous to certain high-risk infants and young children. All respiratory illnesses should be treated quickly and completely. Fevers should also be reduced because they can weaken children and make them more susceptible to breathing difficulties. Depending on the severity of the illness, some doctors recommend keeping supplemental oxygen equipment at home for use in an emergency.

Many but not all children with pulmonary hypertension have to limit their physical activity. A safe level of activity is usually determined by graded exercise testing, which monitors breathing and oxygen consumption. If you have a child with PH, talk to your doctor about the kinds of physical activity that are safe and appropriate.

-- Peter Jaret is a contributing editor for Health magazine and a winner of the American Medical Association's award for medical reporting. His work has appeared in National Geographic, Newsweek, Hippocrates, and many other national magazines. He is also the author of In Self-Defense (Harcourt Brace Jovanovich), Active Living Every Day, and Heart Healthy for Life.

References

First published October 28, 2003
Last updated October 30, 2007
Copyright © 2003 Consumer Health Interactive

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