Pulmonary hypertension (PH) is an incurable and life-threatening disease that occurs when the pulmonary arteries, which are responsible for transporting the blood from the heart to the lungs, become narrowed and obstructed. Due to this defect in the pulmonary arteries, the right heart ventricle needs to work harder to properly pump the blood, making the organ enlarged and weakened. There are different types and causes for the disease — factors that influence diagnosis, treatment and prognosis.
When babies are born with the disease, it is called persistent pulmonary hypertension of the newborn (PPHN), or neonatal pulmonary hypertension. The disease is characterized by a failure in the normal circulatory transition that is inborn in babies and can result in hypoxemia and right-to-left intracardiac shunting of blood. There are signs and symptoms that can help identify the disease and start early treatment to prevent further complications.
Symptoms and Diagnosis of Pulmonary Hypertension in Newborns
Perinatal distress associated with pulmonary hypertension in newborns manifests itself through symptoms like asphyxia; tachypnea; respiratory distress; a loud, single second heart sound (S2) or a harsh systolic murmur (secondary to tricuspid regurgitation), low Apgar scores, meconium staining, cyanosis, poor cardiac function and perfusion, systemic hypotension, and symptoms of shock. However, the symptoms differ between patients and babies can suffer from the disease and not present any signs.
Patients usually have a history of marked lability in oxygenation and levels of hypoxemia disproportionate to the level of pulmonary disease. Contrary to pulmonary hypertension in adults, diagnosing babies does not depend on a specific pulmonary circulation pressure. Physicians can confirm PPHN diagnosis regardless of the arterial pressure, if the patient reveals right-to-left shunt and absence of congenital heart disease. In order to do so, patients are usually submitted to an echocardiography, as well as laboratory testing to evaluate arterial blood gas levels, complete blood count, coagulation studies, and both serum electrolytes (e.g., calcium) and glucose levels.
Pulmonary Hypertension in Newborns Treatment and Prognosis
According to the study “Neonatal Pulmonary Hypertension” published in 2010, the general management of PPHN is based on maintenance of normal temperature, electrolytes (particularly calcium), glucose, and intravascular volume. In addition, it is important to maximize the systemic hemodynamics with volume and cardiotonic therapy, such as dobutamine, dopamine, and milrinone, which can improve the cardiac transportation. Patients who are resistant to medication may need extracorporeal membrane oxygenation (ECMO) therapy.
“The use of surfactant therapy remains variable between centers. Single center trials have shown that surfactant improves oxygenation, reduces airleak, and reduces need for ECMO in infants with meconium aspiration,” said the researchers.
Prognosis and survival depend on the severity of the disease and the timing of intervention, but overall mortality ranges from 10 percent to 60 percent and is associated with the underlying disease. In addition, a quarter of the surviving patients still suffer developmental delay, hearing deficits, functional disabilities, or a combination of those.
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