Persistent Fetal Circulation Syndrome (Persistent Pulmonary Hypertension In The Neonate,PPHN)

Persistent Fetal Circulation Syndrome
Body Parts: Whole Body
Medical Subjects: Children's Health Respiratory System Heart

What Is The Persistent Fetal Circulation Syndrome?

Persistent Pulmonary Hypertension in the Neonate (PPHN), also known as persistent fetal circulation syndrome, is related to pulmonary vascular dysplasia and is a relatively rare neonatal disease, which mainly occurs in full-term infants, overdue infants or premature infants with gestational age over 34 weeks.

The disease is very dangerous and the mortality rate is high. The surviving children may have sequelae.

After the child is born, the blood circulation system will undergo major changes in the first place, upgrading from the "fetal version" to the "adult version". Due to various reasons, if the blood circulation system fails to be upgraded in time, the blood circulation system of the "fetal version" lasts outside the mother's abdomen, and the child will suffer a series of abnormalities, the most obvious of which is pulmonary hypertension.


What Is The Cause Of Persistent Fetal Circulation Syndrome?

Pulmonary vascular anomalies, intrauterine infections, or mothers taking medications that affect pulmonary vascular development during pregnancy can lead to untimely changes in the fetal circulation after birth, leading to persistent pulmonary hypertension in the newborn.

Blood circulation is different before and after birth. Oxygen supply to the fetus before birth mainly comes from the placenta, and after birth mainly comes from the lungs. Before birth, pulmonary vascular resistance is bigger, very little blood circulation. Under normal circumstances, after birth, neonatal pulmonary vasodilation, blood circulation increases, causing a series of changes in blood circulation, blood circulation from the fetus, excessive to the normal blood circulation in the human body.

If pulmonary vascular dysplasia, developmental abnormalities, or infection and other factors lead to pulmonary blood vessels can not be timely expansion, blood circulation can not be timely conversion, leading to neonatal oxygen deficiency, a series of symptoms.


What Are The Symptoms Of Persistent Fetal Circulation Syndrome?

How long can persistent fetal circulation syndrome appear abnormal? Anything unusual?

Some prenatal abnormalities will occur, such as abnormal fetal heart, amniotic fluid abnormalities. At birth, the fingernails and skin of the newborn are contaminated with meconium. For the newborn with abnormality before or at birth, the medical staff will give him/her oxygen inhalation and tracheal intubation during delivery. Some are admitted to neonatal intensive care units immediately after birth.

Almost all children, within 24 hours after birth will appear shortness of breath, skin, blue lips color, etc.

If the newborn is around the parents, the parents should pay attention to observe the newborn's breathing, skin, lip color, abnormal should promptly inform the medical staff.


How To Check For Persistent Fetal Circulation Syndrome?

Echocardiography is the most important examination used to observe whether the neonatal heart structure is normal, and whether there are abnormalities in pulmonary artery morphology and pressure.

In addition, newborns generally need chest X-ray, ECG, blood tests, etc.


How To Prevent Persistent Fetal Circulation Syndrome?

Some studies have shown that the use of non-steroidal anti-inflammatory drugs and antidepressants (selective serotonin receptor inhibitors) during pregnancy may increase the risk of persistent pulmonary hypertension in neonates. It is suggested that avoiding contact with these two drugs during pregnancy and monitoring fetal movement and fetal heart rate during pregnancy may prevent the occurrence of persistent pulmonary hypertension in neonates.


How To Treat Persistent Fetal Circulation Syndrome?

Persistent Pulmonary Hypertension in the Neonate needs to be treated in a neonatal intensive care unit (NICU). The treatment time varies from one week to several months according to the severity of the disease. What the parents can do is to try their best to cooperate with the medical staff in the treatment.

Possible treatments include the following:

  • Oxygen support: Most children need to use a ventilator for assisted ventilation.
  • Vasoactive drugs: Improving circulation and reducing pulmonary artery pressure.
  • Extracorporeal membrane oxygenation (ECMO): It can be simply understood as artificial lung, which can replace the function of the lung, exchange gas outside the body and maintain feeding, thus greatly improving the survival rate of children.
  • In addition, the child may be treated with fluid replacement, acidosis correction, and anti-infection therapy.

What is the treatment effect of persistent fetal circulation syndrome?

The effect of treatment is related to the etiology of children. If it is pulmonary vascular agenesis, children often have other developmental abnormalities. The treatment effect is often poor and the mortality rate is high.

If it is abnormal thickening of the pulmonary vascular wall or persistent pulmonary hypertension caused by poor pulmonary vascular adaptation, the therapeutic effect is relatively good in the case of timely treatment. In particular, the application of extracorporeal membrane oxygenation technology has greatly improved the survival rate and the life quality of children after treatment.

What sequela can you have after treating persistent fetal circulation syndrome?

Possible sequelae include growth retardation, dyskinesia, and hearing impairment. The risk of these sequelae is higher in critically ill children, and the majority of surviving children can grow and live like other children.

Children diagnosed with persistent pulmonary hypertension in neonates should be evaluated for growth and development every six months after birth, observing for abnormalities. If there are abnormalities, active rehabilitation therapy can also bring the intellectual development of children to a level as close to normal as possible.

In addition, for severe patients or patients receiving ECMO or iNO therapy, a neurodevelopmental evaluation should be performed every 6–12 months within 2 years of birth and a hearing test should be performed prior to discharge and at a corrected age of 18–24 months.


What Should Patients With Persistent Fetal Circulation Syndrome Pay Attention To In Life?

If the first child is diagnosed with persistent fetal circulation syndrome, is the second child also affected?

There is no evidence to suggest that the child's persistent pulmonary hypertension is related to the parents' genes. So there is basically no need to worry about having another child and getting sick.

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