What Is Persistent Pulmonary Hypertension of the Newborn?
In the womb, the pathway of your baby's blood circulation is different than it is after birth.
In the uterus, a baby's circulation bypasses the lungs. The lungs are not needed to exchange oxygen because the placenta (the organ that nourishes and protects your developing baby) supplies the baby with oxygen through the umbilical cord. The pulmonary artery - which, after birth, will carry blood from the heart to the lungs - instead sends blood directly back to the heart through a fetal blood vessel called the ductus arteriosus.
Normally, when a baby is born and begins to breathe air, his circulatory system quickly adapts to the outside world. The pressure in the lungs changes as air enters and inflates the lungs. As a result, the ductus arteriosus, which previously supplied the fetal heart with blood, permanently closes. Blood returning to the heart from the body can now be pumped into the lungs, where oxygen and carbon dioxide are exchanged. The blood is then returned to the heart and pumped back out to the body in an oxygen-rich state.
In a baby with PPHN, however, the fetal circulatory system doesn't "switch over." The ductus arteriosus remains open, and the baby's blood flow continues to bypass the lungs. Even though the baby is breathing, oxygen in the breathed air will not reach the bloodstream. Because the blood returning from the body is unable to enter the lungs properly - and instead flows through the still- open ductus arteriosus - it returns to the heart in an oxygen-poor state. This condition is known as persistent fetal circulation, or PFC. "The baby's circulation has not made the normal transition from fetal circulation to normal newborn circulation, because pressure in the lungs is increased and this causes distress," says Neal Cohn, MD, a pediatrician. Depending on the degree of PPHN causing the persistent fetal circulation, the oxygen in the air your baby breathes into his lungs is not adequately picked up and carried by the blood to other areas of the body that need it (such as the brain, kidneys, liver, and other organs). These organs soon become stressed from lack of oxygen.
PPHN sometimes develops as the result of another event during delivery or from a disease or congenital condition affecting the newborn (usually one that either directly affects the lungs or oxygen supply to the baby before or during birth). Often, however, PPHN occurs as an isolated condition, and its cause is not known. It is usually seen soon after birth, often within 12 hours after birth. PPHN occurs in approximately one in 700 births.
What Causes PPHN?
In an otherwise healthy newborn, the cause of PPHN is usually unknown. Some researchers believe that stress while the baby is in the uterus (associated with certain pregnancy complications, such as maternal diabetes, high blood pressure or anemia, or delivery after 40 weeks) may increase the risk of developing PPHN.
PPHN may occur with certain diseases or congenital conditions of the infant that affect the lungs in some way. Meconium aspiration syndrome, anemia, severe pneumonia, infection, hypoglycemia (low blood sugar), and birth asphyxia (when the baby is deprived of oxygen during a complicated delivery) have all been associated with PPHN.
These conditions may cause the pressure in the blood vessels leading to the lungs to increase to the point where the baby's blood continues to bypass the lungs after birth, resulting in PFC. These conditions are often temporary and reversible, with intensive care and time for the lungs and body to heal. Certain congenital conditions that result in immature or incomplete lung development (such as diaphragmatic hernia) may also be associated with PPHN.
Signs and Symptoms>
The following signs and symptoms may indicate a baby has PPHN:
- rapid breathing (also called tachypnea)
- rapid heart rate
- respiratory distress, including signs such as flaring nostrils and grunting
- cyanosis (when the skin has a bluish tinge), even while the baby is receiving extra oxygen to breathe
Sometimes when examining a baby with PPHN, the doctor will hear a heart murmur (an extra or abnormal heart sound). With PPHN, a baby may also continue to have low oxygen levels in the blood while receiving 100% oxygen. |