Persistent pulmonary hypertension of the newborn

Persistent Pulmonary Hypertension of the Newborn
[[File:|250px|]]
Synonyms	PPHN
Pronounce	N/A
Specialty	N/A
Symptoms	Cyanosis, tachypnea, respiratory distress
Complications	Hypoxemia, organ dysfunction
Onset	Neonatal period
Duration	Variable
Types	N/A
Causes	Failure of normal circulatory transition
Risks	Meconium aspiration syndrome, congenital diaphragmatic hernia, sepsis
Diagnosis	Echocardiography, clinical presentation
Differential diagnosis	N/A
Prevention	N/A
Treatment	Oxygen therapy, inhaled nitric oxide, extracorporeal membrane oxygenation
Medication	N/A
Prognosis	Variable
Frequency	1-2 per 1000 live births
Deaths	N/A

Persistent Pulmonary Hypertension of the Newborn (PPHN) is a serious medical condition that affects newborn infants. It is characterized by the failure of the normal circulatory transition that occurs after birth, leading to high blood pressure in the lungs and insufficient oxygenation of the blood.

Pathophysiology

In a healthy newborn, the transition from fetal to neonatal circulation involves a decrease in pulmonary vascular resistance and an increase in systemic vascular resistance. This allows blood to flow through the lungs for oxygenation. In PPHN, this transition is impaired, resulting in persistent high pulmonary vascular resistance and right-to-left shunting of blood through fetal circulatory pathways such as the ductus arteriosus and foramen ovale.

Causes

PPHN can be associated with several conditions, including:

• Meconium aspiration syndrome
• Congenital diaphragmatic hernia
• Neonatal sepsis
• Birth asphyxia
• Pulmonary hypoplasia

Clinical Presentation

Newborns with PPHN typically present with:

• Cyanosis (bluish discoloration of the skin due to low oxygen levels)
• Tachypnea (rapid breathing)
• Respiratory distress
• Low oxygen saturation despite adequate ventilation

Diagnosis

The diagnosis of PPHN is primarily clinical, supported by:

• Echocardiography: to assess pulmonary artery pressure and detect right-to-left shunting
• Chest X-ray: to rule out other causes of respiratory distress
• Blood gas analysis: to evaluate oxygenation status

Treatment

Management of PPHN involves:

• Oxygen therapy: to maintain adequate oxygenation
• Inhaled nitric oxide: a selective pulmonary vasodilator
• Extracorporeal membrane oxygenation (ECMO): for severe cases unresponsive to conventional therapy
• Surfactant therapy: in cases associated with respiratory distress syndrome

Prognosis

The prognosis of PPHN varies depending on the underlying cause and the severity of the condition. With advances in treatment, many infants recover fully, although some may experience long-term complications such as neurodevelopmental delay.

Also see

• Neonatal respiratory distress syndrome
• Meconium aspiration syndrome
• Congenital diaphragmatic hernia
• Neonatal sepsis