Pulmonary hypertension (PH) is a rare, serious disease characterized by high blood pressure and damaged pulmonary arteries. These vessels, which are responsible for carrying the blood from the right heart ventricle to the lungs, become narrowed and thickened as result of the disease. Therefore, the normal blood flow becomes obstructed, and the heart needs to work harder to properly pump the blood, increasing the risk of enlargement and weakening of the organ, as well as right heart failure.
PH affects the lungs, heart and overall health, and can be caused by very different factors. While pulmonary hypertension can be a disease that develops independently, it can also be associated with any condition that disturbs normal blood flow and oxygen delivery. While research usually focuses on its occurrence among older people, it is in fact more common among children and can be developed by children at all ages.
Characteristics Of Pulmonary Hypertension in Children
Development of Pulmonary Hypertension by Newborns
Persistent pulmonary hypertension of the newborn (PPHN) or neonatal pulmonary hypertension are the terms given when babies are born with the disease. It is characterized by a failure in the normal circulatory transition that is inborn in infants and can result in hypoxemia and right-to-left intracardiac shunting of blood. Perinatal distress associated with pulmonary hypertension in newborns manifests itself through symptoms like asphyxia, tachypnea, respiratory distress, 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, symptoms of shock.
Development of Pulmonary Hypertension During Childhood
Pediatric pulmonary hypertension is similar to idiopathic pulmonary arterial hypertension in adults, which is when the cause is unknown and etiology is unclear. Children at all ages can be diagnosed with it when they reveal a mean pulmonary artery pressure greater than 25 mm Hg at rest with normal pulmonary capillary wedge pressure, in the absence of associated causes of pulmonary hypertension. It is thought that exercise hemodynamic abnormalities are also related to disease, which involves significant morbidity and mortality.
Diagnosis Of Pulmonary Hypertension in Children
Children who suffer from pulmonary hypertension usually present symptoms consistent with low cardiac output, including poor appetite, poor growth, nausea, vomiting, lethargy, sweating, tachypnea and tachycardia. Patients with patent foramen ovale may also suffer cyanosis and patients with no atrial level pop-off may also suffer syncope. As children get older, they may present symptoms similar to adult patients, such as exertional dyspnea and chest pain. As the disease progresses, patients may develop pulmonary hypertensive crises, arrhythmias, syncope and sudden cardiac death.
Since this is a rare disease, and the symptoms are consistent with other lung conditions, the diagnosis is often difficult or delayed. To confirm the diagnosis, physicians usually request medical and family histories, as well as numerous exams, including blood tests, coagulation studies, exams for collagen-vascular disease, and imaging tests. Physicians may also seek signs of accentuated pulmonic component of the second heart sound, a right ventricular heave with or without chest wall distortion, and tricuspid regurgitation.
Treatment Of Pulmonary Hypertension in Children
There is currently no cure for pulmonary hypertension, which compromises the growth, development and life expectancy of pediatric patients. There are, however, treatments that can improve the disease, including warfarin (Coumadin), digoxin, and vasodilators, such as nifedipine, intravenous prostacyclin, or both. For children whose disease is resistant to these treatments, lung transplantation is an option as well. The study “The management of pulmonary hypertension in children” focused on the evolution and challenges of treating children with pulmonary hypertension.
“The management of the condition has changed dramatically in the past 5 years with the introduction of new medicines. However, diagnosis, investigation and choice of therapy remain a challenge. (…) New medicines are being developed which, rather than focussing on dilating a diseased pulmonary vascular bed, aim to structurally remodel the pulmonary vasculature towards normal,” explained the author.
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