Persistent Pulmonary Hypertension of the Newborn

Signs and Symptoms

The following signs and symptoms may indicate that a baby has persistent pulmonary hypertension of the newborn (PPHN)

• Rapid breathing, also called tachypnea
• Rapid heart rate
• Respiratory distress, including signs such as flaring nostrils and grunting
• Cyanosis, a condition in which the baby's skin has a bluish tint, even while they are receiving extra oxygen to breathe
• Heart murmur, where a baby has an extra or abnormal heart sound
• Low oxygen levels -- a baby with PPHN may continue to have low oxygen levels in their blood, even while receiving 100 percent oxygen

Diagnosis

Various imaging and laboratory tests can help determine if a baby has persistent pulmonary hypertension of the newborn (PPHN). These may include:

• Chest X-rays may be recommended and can determine if the baby has lung disease or an enlarged heart.

• Echocardiogram, which is an ultrasound of the heart, can show whether the baby has heart or lung disease and evaluate blood flow in those organs.

• Ultrasound of the head may be used to look for bleeding in the brain.

• Arterial blood gas (ABG) determines how well oxygen is being delivered to the body.

• Complete blood count (CBC) measures the number of oxygen-carrying red blood cells, white blood cells and platelets.

• Serum electrolyte tests evaluate the balance of minerals in the blood.

• Lumbar puncture, also called a spinal tap, and blood tests can help determine whether an infection is present.

• Pulse oximetry, which measures oxygen levels in the blood, can help doctors monitor whether the baby's tissues are receiving an adequate amount of oxygen.

Treatment

The main goal of treatment for persistent pulmonary hypertension of the newborn (PPHN) is to increase oxygen levels to the baby's organs to avoid serious health problems. Treatment may include a wide range of mechanical ventilation and respiratory therapy options, including high frequency oscillatory ventilation and inhaled nitric oxide.

• Oxygen -- 100 percent supplemental oxygen may be given to your baby through a mask or plastic hood.

• Assisted Ventilation -- During this procedure, a tube is inserted into your baby's windpipe and a ventilator takes over your baby's breathing and oxygen is given.

• Nitric Oxide -- Research has shown that this gas is effective in treating PPHN because it relaxes contracted lung blood vessels and improves blood flow to the lungs. It is given through the ventilator.

• High Frequency Oscillatory Ventilation -- This type of ventilation may improve the oxygen level in the blood if other types of ventilation are not effective.

Extracorporeal Membrane Oxygenation

In addition, an extracorporeal membrane oxygenation (ECMO) machine may be used for patients who are experiencing serious heart or lung failure. It delivers oxygen to the brain and body as temporary support while the PPHN resolves. ECMO is similar to a heart-lung bypass machine, which takes over your baby's heart and lung functions with an external pump and oxygenator. Blood is drained from the patient to an artificial lung, where oxygen is added and carbon dioxide is removed, and then the blood is pumped back into your child.

At UCSF Children's Hospital, we treat over 20 patients each year with ECMO and our success outcomes are among the highest in the country. Our team of experts is specially trained in ECMO and includes a neonatologist, surgeon, respiratory therapists and nurses with special training in the management of ECMO.