persistent fetal circulation syndrome

Persistent Fetal Circulation Syndrome (PFCS) Description

Persistent fetal circulation syndrome, also known as persistent pulmonary hypertension of the newborn (PPHN), is a condition that affects newborn babies. It occurs when the baby's circulation fails to transition from the antenatal pattern to the normal postnatal pattern after birth.

Key Features:

• Failure of Circulatory Transition: The systemic and pulmonary circulation fail to convert from the fetal circulation pattern to the normal adult circulation pattern.
• High Pulmonary Vascular Resistance: The pulmonary arteries remain constricted, leading to high blood pressure in the lungs.
• Right-to-Left Shunting: Blood flows from the right side of the heart to the left side through the foramen ovale and ductus arteriosus, causing hypoxemia (low oxygen levels).
• Impaired Circulatory Adaptation: The baby's circulation is unable to adapt to the changes that occur after birth.

Causes and Risk Factors:

• Prenatal stress
• Postnatal stress
• Anatomical differences
• Respiratory distress syndrome
• Overwhelming sepsis
• Meconium aspiration

Symptoms:

• Respiratory distress
• Cyanosis (blue discoloration of the skin)
• Apnea (pauses in breathing)
• Bradycardia (slow heart rate)

Diagnosis and Treatment:

• Diagnosis is based on clinical presentation, laboratory tests, and imaging studies.
• Treatment involves supportive care, such as oxygen therapy, mechanical ventilation, and medication to reduce pulmonary vascular resistance.

Prognosis:

• The prognosis for babies with PFCS depends on the severity of the condition and the effectiveness of treatment.
• With prompt and proper management, many babies can recover fully. However, some may experience long-term complications or even death.

References:

[1] [2] [3] [4] [5] [6] [7] [8] [9] [10] [11] [12] [13]

Common Signs and Symptoms of Persistent Fetal Circulation Syndrome

Persistent fetal circulation syndrome, also known as persistent pulmonary hypertension of the newborn (PPHN), is a condition that affects newborn babies. It occurs when the blood vessels in the lungs fail to open after birth, causing the heart to work against higher pressures.

• Rapid Breathing: One of the earliest signs of PFC is rapid breathing, also called tachypnea [7]. This can be accompanied by other respiratory distress symptoms such as flaring nostrils and grunting.
• Rapid Heart Rate: Infants with PFC often experience a rapid heart rate, which can be a sign that their heart is working harder to pump blood through the body [9].
• Respiratory Distress: Babies with PFC may exhibit signs of respiratory distress, including difficulty breathing and a bluish discoloration of the skin (cyanosis) [7][8].
• Tachycardia: In severe cases, PFC can lead to poor cardiac output and shock, which can cause tachycardia, ashen or gray color, capillary refill time > 2 seconds, and other signs of circulatory compromise [6].

These symptoms can occur in the first hours after birth and may be accompanied by a general appearance of illness. It's essential to seek medical attention immediately if you suspect your baby is experiencing any of these symptoms.

References: [6] - Signs & symptoms · Rapid breathing, also called tachypnea · Rapid heart rate · Respiratory distress, including signs such as flaring nostrils and grunting ... [7] - Signs & symptoms · Rapid breathing, also called tachypnea · Rapid heart rate · Respiratory distress, including signs such as flaring nostrils and grunting ... [8] - Persistent pulmonary hypertension of the newborn (PPHN) is a dangerous condition that may cause a baby to not get enough oxygen after birth. [9] - Infants experience a high mean arterial pulmonary artery pressure and a high afterload at the right ventricle. This means that the heart is working against ...

Diagnostic Tests for Persistent Fetal Circulation Syndrome

Persistent fetal circulation syndrome, also known as persistent pulmonary hypertension of the newborn (PPHN), can be diagnosed using various tests.

• Echocardiogram: This painless test uses sound waves to take a picture of the heart and blood vessels. It is a crucial diagnostic tool for PPHN, as it can show abnormal right ventricular dilatation and leftward deviation of the interventricular septum [5][7].
• Chest X-ray: A chest X-ray can show certain problems with the lungs or heart that may indicate PPHN. However, this test is not definitive on its own and should be used in conjunction with other diagnostic tests [6][8].
• Blood tests: Blood tests, including arterial blood gas analysis, can help diagnose PPHN by measuring oxygen levels in the blood and detecting abnormalities in blood chemistry [3][9].
• Pulse oximetry: Pulse oximetry is a noninvasive way to measure oxygen levels in the blood. It can be used to monitor oxygen saturation in infants suspected of having PPHN [1].

Other Diagnostic Tests

In addition to these tests, other diagnostic procedures may be used to rule out underlying disorders or structural abnormalities that could be contributing to PPHN. These include:

• Echocardiography: This test can help evaluate the direction of fetal shunts and diagnose PPHN [11].
• Genetic testing: Genetic testing may be performed to identify genetic mutations associated with PPHN, although this is not a primary diagnostic tool for the condition [4][14].

References

[1] Your infant’s healthcare provider may use various laboratory and imaging tests to diagnose PPHN, including pulse oximetry.

[3] Diagnosis. Physical examination of the infant is sufficient for the health care provider to diagnose gastroschisis. The mother may have shown signs indicating ...

[4] by P Bendapudi · 2015 · Cited by 118 — The mainstay of diagnosis and to exclude structural abnormalities is echocardiography.

[5] Jun 11, 2024 — The diagnosis of PPHN is confirmed by echocardiography. The cardinal findings include abnormal right ventricular dilatation, leftward deviation ...

[6] How is PPHN in the newborn diagnosed? · Chest X-ray. This can show certain problems with the lungs or heart.

[7] Diagnosis · Echocardiogram (echo): This painless test uses sound waves to take a picture of the heart and blood vessels.

[8] Genetic Tests for Persistent Fetal Circulation Syndrome. No Genetic Tests ... Clinical studies linking genetic mutations to PPHN are limited.

[9] PROM—Premature rupture of membranes, CDH—Congenital diaphragmatic hernia, MAS—Meconium aspiration syndrome, PPHN—Persistent pulmonary hypertension of the newborn, LV—Left ventricle. ... The diagnosis is made on echocardiography by evaluating the direction of fetal shunts.

[11] Persistent pulmonary hypertension of the newborn (PPHN) is often secondary to parenchymal lung disease (such as meconium aspiration syndrome) or lung hypoplasia (with congenital diaphragmatic hernia) but can also be idiopathic. PPHN is characterized by elevated pulmonary vascular resistance, resulting in right-to-left shunting of blood and hypoxemia.

[12] The definition, pathophysiology, signs and symptoms, diagnostic and therapeutic procedures of the persistent fetal circulation syndrome are presented. A description of the clinical characteristics of 38 patients, admitted during a 3 years period, is included.

[13] Persistent fetal circulation syndrome Synonyms ... NIH makes no endorsements of tests or laboratories listed in the GTR. GTR is not a substitute for medical advice. Patients and consumers with specific questions about a genetic test should contact a health care provider or a genetics professional.

[14] Persistent pulmonary hypertension of the newborn (PPHN), previously referred to as persistent fetal circulation, is a syndrome of impaired circulatory adaptation at birth . The hallmark of PPHN physiology is sustained elevation of pulmonary vascular resistance (PVR) and persistent hypoxemia after birth ( 2 ).

Persistent pulmonary hypertension, also known as persistent fetal circulation syndrome, is a condition where a newborn's circulation reverts back to the circulation of a fetus. This can lead to high blood pressure in the lungs and potentially fatal consequences.

Treatment Options:

Several treatment options are available for managing persistent pulmonary hypertension:

• Lung Recruitment: Optimal mean airway pressure and surfactant therapy can help improve lung function.
• Vasodilator Drugs: Various vasodilator drugs have been used to treat infants with pulmonary hypertension, including:
  • Tolazoline: Responds in 25-50% of affected babies
  • Sildenafil: A promising treatment option due to its satisfactory efficacy and easy mode of administration [2]
  • Milrinone: Can be effective in managing pulmonary hypertension [7][8]
• Inhaled Iloprost: A stable analogue of prostacyclin that has been used to treat persistent pulmonary hypertension [6]
• Pulmonary Vasodilators: Other treatment options include nitrous oxide inhalation (iNO), milrinone, and glucocorticoids.

Management Strategies:

Effective management strategies for persistent pulmonary hypertension include:

• Inotropic Support: Dobutamine, dopamine, or milrinone can be used to support cardiac function [7][8]
• Surfactant Therapy: Can help improve lung function and reduce the need for mechanical ventilation
• Mechanical Ventilation: May be necessary in severe cases to support respiratory function

It's essential to note that each case of persistent pulmonary hypertension is unique, and treatment plans should be tailored to individual needs. Ongoing research and clinical trials are exploring new and more effective treatment options for this condition.

References:

[1] Context result 3 [2] Context result 2 [6] Context result 6 [7] Context result 6 [8] Context result 8

Persistent pulmonary hypertension of the newborn (PPHN), previously referred to as persistent fetal circulation, has several differential diagnoses that need to be considered for accurate diagnosis.

• Meconium Aspiration Syndrome: This condition can cause respiratory distress and hypoxemia in newborns, similar to PPHN. It is essential to rule out Meconium Aspiration Syndrome through clinical evaluation and diagnostic tests.
• Pediatric Metabolic Acidosis: Metabolic acidosis can lead to respiratory alkalosis as a compensatory mechanism, which may be mistaken for PPHN. However, metabolic acidosis has distinct laboratory findings that need to be considered in the differential diagnosis.
• Pediatric Pneumonia: Pneumonia can cause respiratory distress and hypoxemia, making it a potential differential diagnosis for PPHN. However, pneumonia typically presents with fever, cough, and other signs of infection.
• Pediatric Pulmonary Hypoplasia: This condition involves underdevelopment of the lungs, which can lead to respiratory distress and hypoxemia similar to PPHN.

It is crucial to consider these differential diagnoses when evaluating a newborn for PPHN. A thorough clinical evaluation, including physical examination and diagnostic tests such as echocardiography, is necessary to rule out other conditions and confirm the diagnosis of PPHN [8].

In addition to these differential diagnoses, structural heart disease needs to be excluded when considering PPHN as a likely diagnosis. Echocardiography is the definitive diagnostic tool for this purpose [8].