Persistent Fetal Circulation Syndrome
Disease Details
Family Health Simplified
- Description
- Persistent fetal circulation syndrome (PFCS) is a neonatal condition where the newborn's circulatory system does not adapt to breathing outside the womb, causing high blood pressure in the lungs and insufficient oxygenation of the blood.
- Type
- Persistent fetal circulation syndrome, also known as persistent pulmonary hypertension of the newborn (PPHN), is not typically categorized by a specific genetic transmission. It is a condition that arises due to the failure of normal circulatory transition after birth, leading to high blood pressure in the lungs and inadequate oxygenation. The majority of cases are not inherited in a Mendelian fashion, though there may be some genetic predispositions and associations with certain conditions.
- Signs And Symptoms
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Persistent Fetal Circulation Syndrome, also known as Persistent Pulmonary Hypertension of the Newborn (PPHN), is a serious condition that affects newborns. The signs and symptoms can include:
- Rapid breathing (tachypnea)
- Grunting while breathing
- Flaring of the nostrils
- Retractions (pulling in of the chest wall with each breath)
- Cyanosis (a bluish tint to the skin, lips, and fingernails)
- Low oxygen levels despite oxygen therapy
- Hypotension (low blood pressure)
- Poor feeding
- Lethargy or fatigue
This condition requires immediate medical attention and treatment in a neonatal intensive care unit (NICU). - Prognosis
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Persistent Fetal Circulation Syndrome (PFCS), also known as Persistent Pulmonary Hypertension of the Newborn (PPHN), has varying prognoses depending on the severity of the condition and timeliness of treatment.
Prognosis:
- Mild to Moderate Cases: With prompt and appropriate medical intervention, many infants recover without long-term complications. Treatments often include supplemental oxygen, mechanical ventilation, and pulmonary vasodilators.
- Severe Cases: May require more intensive interventions such as extracorporeal membrane oxygenation (ECMO). These cases have a higher risk of complications, and some infants may experience long-term health issues, including chronic lung disease, developmental delays, or neurological deficits.
Nan: Not applicable in this context as it typically refers to "nanotechnology" or "Not a Number," which is irrelevant to PFCS. - Onset
- Persistent fetal circulation syndrome, also known as persistent pulmonary hypertension of the newborn (PPHN), typically presents in full-term or late preterm infants shortly after birth. The onset is usually within the first 24 hours of life.
- Prevalence
- Persistent fetal circulation syndrome, also known as persistent pulmonary hypertension of the newborn (PPHN), does not have a well-defined prevalence as it can vary widely based on population and diagnostic criteria. However, it is estimated to occur in about 1 to 2 per 1,000 live births.
- Epidemiology
- It occurs in 1–2 infants per 1000 live births. It is more common in males and in areas with higher altitudes. Additionally, two percent of infants with respiratory distress syndrome develop PPHN.
- Intractability
- Persistent fetal circulation syndrome, also known as persistent pulmonary hypertension of the newborn (PPHN), is not inherently intractable. With timely and appropriate medical interventions, including oxygen therapy, mechanical ventilation, and medications like inhaled nitric oxide, the condition can often be managed successfully. However, severe cases may present significant treatment challenges and potential complications, requiring advanced care and sometimes long-term follow-up.
- Disease Severity
- Persistent fetal circulation syndrome (PFCS), also known as persistent pulmonary hypertension of the newborn (PPHN), is a serious condition that affects newborns. The disease severity can range from mild to severe, and it may be life-threatening if not promptly diagnosed and treated. The condition requires immediate medical intervention to manage the high blood pressure in the lungs and ensure adequate oxygenation of the baby's blood. The severity of PFCS is largely dependent on the degree of pulmonary hypertension and the infant's overall health.
- Healthcare Professionals
- Disease Ontology ID - DOID:13042
- Pathophysiology
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Typically, a fetus experiences pulmonary hypertension in utero since it is relying on the placenta for oxygen rather than its lungs. When the fetus is born, it is no longer attached to the placenta and must use the lungs to receive oxygen. To facilitate this change from fetus to newborn, the baby must change from a state of high PVR to low PVR, allowing for increased blood flow to circulate throughout the body. This inability of the newborn to adapt to these changes is caused by various processes, such as:
Normal vascular anatomy with functional vasoconstriction: This has a good prognosis, as it is reversible. Causes include hypoxia, meconium aspiration, and respiratory distress syndrome. Left untreated, this can lead to hypoxic respiratory failure (HRF).
Decreased diameter of pulmonary vessels with hypertrophy of vessel walls: This has a poor prognosis, as it is a fixed abnormality. Causes include post-term pregnancy, placental insufficiency, and NSAID use by the mother.
Decreased size of pulmonary vascular bed: This has a poor prognosis, as it is a fixed abnormality. It is caused by space occupying lesions such as pleural effusions and diaphragmatic hernias.
Functional obstruction of pulmonary blood flow: This has a good prognosis if it is reversible. Causes include polycythemia and hyperfibrinogenemia. - Carrier Status
- Persistent Fetal Circulation Syndrome, also known as Persistent Pulmonary Hypertension of the Newborn (PPHN), is not an inherited condition and does not involve carrier status. It is a serious medical condition that occurs when a newborn's circulatory system does not adapt to breathing outside the womb, leading to high blood pressure in the lungs and the inability to get enough oxygen into the bloodstream. The focus of treatment is on managing the newborn's oxygenation and pulmonary blood pressure.
- Mechanism
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Persistent fetal circulation syndrome (PFCS), also known as persistent pulmonary hypertension of the newborn (PPHN), is a condition where a newborn's circulation continues to follow the fetal circulatory pattern, leading to inadequate oxygenation.
**Mechanism:**
During fetal life, the lungs are filled with fluid, and the blood bypasses the lungs via the ductus arteriosus and foramen ovale, directed instead to the rest of the body. Upon birth, the first breath should initiate the closure of these shunts, and the pulmonary vessels should dilate, allowing blood to be oxygenated in the lungs. In PFCS/PPHN, this transition fails, causing high pulmonary vascular resistance and resultant right-to-left shunting of blood, leading to hypoxemia.
**Molecular Mechanisms:**
- **Endothelial dysfunction:** An imbalance between vasoconstrictors such as endothelin-1 and vasodilators such as nitric oxide (NO) and prostacyclin may contribute to sustained high pulmonary vascular resistance.
- **Pulmonary artery remodeling:** Structural changes and thickening of the pulmonary artery walls can impede normal blood flow.
- **Impaired NO-cGMP signaling:** Nitric oxide (NO) is critical for vasodilation. Disruption in its production or the signaling pathway involving cyclic guanosine monophosphate (cGMP) can inhibit relaxation of pulmonary vessels.
- **Inflammatory mediators:** Elevated levels of inflammatory cytokines and growth factors may contribute to vessel constriction and remodeling.
- **Hypoxia-induced factors:** Chronic hypoxia can lead to increased production of hypoxia-inducible factors (HIFs), promoting vasoconstriction and vascular remodeling.
Understanding these mechanisms is essential for developing targeted therapies to manage PPHN effectively. - Treatment
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Treatment aims to increase the amount of oxygen in the blood and reverse any causes of hypoxia as well as gain adequate perfusion.Common treatments include:
Oxygen therapy
Mechanical ventilation
Pulmonary vasodilators
Nitrous Oxide Inhalation (iNO)
Sildenafil
Milrinone
GlucocorticoidsThe therapies available to manage PPHN include high frequency ventilation, surfactant instillation, pulmonary vasodilators, and extracorporeal membrane oxygenation.iNO is the preferred medication for PPHN due to its ability to more selectively cause pulmonary vasodilation in comparison to intravenous vasodilators. While this medication decreases the need for extracorporeal membrane oxygenation or extracorporeal life support, it has not been shown to reduce mortality. Intravenous sildenafil has been shown to have similar efficacy and is becoming more commonly used as treatment for PPHN.Assessment of the efficacy of these treatments includes chest radiographs and arterial blood gases. Signs of inefficacious treatments include prolonged capillary filling time, low pulse volume, low blood pressure, and sustained metabolic acidosis.In addition to treating the direct effects of this condition, other management strategies are implemented concurrently to stabilize the newborn.These include, but are not limited to nutritional support, reduction of stressful environment, gentle sedation, monitoring/treating acidosis and establishing normal systemic blood pressure.Challenges in developing countries:
PPHN has been seen more frequently in developing countries or resource-poor areas, though it occurs across the globe. Treating this condition often involves large interdisciplinary teams, which is not always possible in developing countries. In low-resource environments, it is recommended to focus on five main bundles of management:
Increasing oxygen supply
Decreasing oxygen demand
Facilitating gas exchange
Inducing pulmonary vasodilation
Fixing metabolic disturbances - Compassionate Use Treatment
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Persistent Fetal Circulation Syndrome (PFCS), also known as Persistent Pulmonary Hypertension of the Newborn (PPHN), is a serious condition that affects newborns. For treatment, the primary approach typically includes supportive measures like mechanical ventilation and the administration of oxygen.
**Compassionate Use Treatment:**
- In very severe cases where conventional treatments fail, compassionate use treatments may include extracorporeal membrane oxygenation (ECMO). ECMO is a form of advanced life support used to provide adequate oxygenation while allowing the lungs to heal.
**Off-label or Experimental Treatments:**
- Sildenafil, a phosphodiesterase-5 inhibitor typically used for erectile dysfunction or pulmonary hypertension in adults, has been used off-label to improve pulmonary blood flow in infants with PPHN.
- Inhaled nitric oxide (iNO), while not experimental anymore, was considered innovative when first used for PPHN treatment. It helps relax pulmonary vessels and improve oxygenation.
- Milrinone, a phosphodiesterase-3 inhibitor, can be utilized off-label for its vasodilatory effects on the pulmonary artery.
- Bosentan, an endothelin-receptor antagonist used for pulmonary arterial hypertension in older patients, has been explored off-label in neonatal use but requires careful monitoring due to potential side effects. - Lifestyle Recommendations
-
Persistent fetal circulation syndrome, also known as persistent pulmonary hypertension of the newborn (PPHN), primarily affects newborns. It is a serious condition where a newborn's circulation system doesn't adapt to breathing outside the womb, causing high blood pressure in the lungs and limited oxygen flow.
Because PPHN is a neonatal condition treated in a medical setting, lifestyle recommendations for the newborn aren't typically applicable. However, some steps that parents and caregivers can take to support the health of a newborn with PPHN include:
1. **Follow Medical Advice Strictly**: Adhere to all treatment protocols and follow-up care as prescribed by the healthcare team.
2. **Breastfeeding or Appropriate Nutrition**: Ensure the baby receives proper nutrition. For those who are able, breastfeeding is encouraged, as it supports overall health and immunity.
3. **Avoiding Infections**: Keep the baby away from sick individuals and practice good hygiene to reduce the risk of infections, which can complicate recovery.
4. **Smoke-Free Environment**: Ensure the baby is in a smoke-free environment, as exposure to smoke can impair lung function and overall health.
5. **Regular Monitoring**: Attend all scheduled pediatrician appointments for regular monitoring and early detection of any complications.
6. **Healthy Home Environment**: Maintain a clean and safe environment to support the infant's health and well-being.
These steps can assist in the overall care and recovery of a newborn with PPHN. However, the condition itself requires specialized medical intervention. - Medication
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Persistent fetal circulation syndrome, also known as persistent pulmonary hypertension of the newborn (PPHN), is a serious condition that requires timely medical intervention. Medications used to manage PPHN include:
1. Inhaled Nitric Oxide: A vasodilator that helps to relax blood vessels in the lungs, improving oxygenation.
2. Sildenafil: A phosphodiesterase inhibitor that can help lower pulmonary blood pressure.
3. Milrinone: An inotrope and vasodilator that improves heart function and reduces pulmonary vascular resistance.
4. Prostacyclin Analogues (e.g., Epoprostenol): Used to dilate pulmonary vessels.
5. Surfactant Therapy: Administered in cases where lung function is compromised.
6. Antimicrobials: Administered if an infection is suspected as the underlying cause.
Management usually takes place in a neonatal intensive care unit (NICU), and treatment is individualized based on the infant's specific needs. - Repurposable Drugs
-
Persistent Fetal Circulation Syndrome (PFCS), also known as Persistent Pulmonary Hypertension of the Newborn (PPHN), is a serious condition that affects newborns. It involves high blood pressure in the lungs and may require treatment strategies that use repurposable drugs to improve outcomes. Some potential repurposable drugs include:
1. **Sildenafil**: Originally used for erectile dysfunction and pulmonary hypertension, sildenafil can help reduce pulmonary vascular resistance.
2. **Milrinone**: Typically used for heart failure, milrinone can improve cardiac output and reduce pulmonary pressure.
3. **Bosentan**: This endothelin receptor antagonist, used for pulmonary arterial hypertension, can help decrease pulmonary artery pressure.
4. **Inhaled Nitric Oxide (iNO)**: Though not a repurposed drug, it is a standard therapy for PPHN due to its selective pulmonary vasodilatory effects.
These treatments aim to improve oxygenation and reduce the need for more invasive measures. Always consult a healthcare professional for treatment plans tailored to individual needs. - Metabolites
- Persistent Fetal Circulation Syndrome (PFCS), also known as Persistent Pulmonary Hypertension of the Newborn (PPHN), does not have a specific set of "metabolites" typically identified for diagnostic or monitoring purposes. The condition is generally diagnosed based on clinical signs and symptoms, as well as specific tests like echocardiograms and blood gas analyses. Diagnostics focus on circulatory function rather than metabolic byproducts.
- Nutraceuticals
- Persistent fetal circulation syndrome, also known as persistent pulmonary hypertension of the newborn (PPHN), primarily requires medical treatment including oxygen therapy, mechanical ventilation, and medications like nitric oxide, sildenafil, or prostacyclin. Nutraceuticals are not currently established as an effective treatment for PPHN. Always consult with a healthcare professional for appropriate management and treatment options.
- Peptides
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Persistent Fetal Circulation Syndrome (PFCS), also known as Persistent Pulmonary Hypertension of the Newborn (PPHN), is a serious condition that affects the circulatory system of newborns. This condition involves high blood pressure in the lungs, which can restrict blood flow and reduce oxygen delivery to the body.
Peptides may be relevant in this context as therapeutic agents or biomarkers. For example, Endothelin-1 is a peptide that can cause vasoconstriction, potentially contributing to the pathological high blood pressure in the lungs. Additionally, vasoactive medications like sildenafil, a phosphodiesterase inhibitor, although not a peptide, can influence peptide signaling pathways to help relax pulmonary blood vessels.
"NAN" could refer to something as simple as 'not applicable' or explain an acronym or technical term not directly immediately available or pertinent. If you meant another specific term by "nan," please provide further clarification.