Pulmonary Hypertension

What is Pulmonary Hypertension?

Pulmonary Hypertension (PH) is a disease in which the pressures in the arteries to the lungs are elevated along with the resistance to blood flow. If untreated or resistant to treatment, the right side of the heart can no longer compensate to pump the blood effectively against this high resistance.  This can lead to progressive right heart failure and early death. If the PH occurs without other causes it is called Idiopathic Pulmonary Hypertension or it may be secondary to other conditions such as congenital heart or chronic lung disease.

Symptoms

The most common symptoms in children with pulmonary hypertension are breathing problems which may worsen with exercise or activity.

Specific symptoms may include:

• Chest Pain
• Feeling short of breath, weak or dizzy when active
• Fainting
• Being more tired than normal
• Bluish color to the lips, hands and feet (cyanosis)
• Swelling in the ankles

Children affected by PH include those with certain congenital heart defects both before and after surgery or those with pulmonary vein stenosis, chronic lung disease and connective tissue disorders such as lupus.  Other conditions associated with PH include Eisenmenger's Syndrome, portal hypertension, HIV infection, sleep apnea, blood clotting disorders, small vessel disease such as sickle cell anemia, or those suffering from end-stage heart disease.

Pulmonary Hypertension Center at DMC Children’s Hospital of Michigan

The Pulmonary Hypertension Center is believed to be one of only two pediatric pulmonary hypertension programs in the state.  Specialists treat children with a wide range of conditions that can cause pulmonary hypertension. Typically, patients are seen every three months.  The clinic sees patients from newborn to adults including patients with congenital heart disease.

Screening for PH includes a thorough history and physical examination along with diagnostic testing including an Electrocardiogram (EKG) and Echocardiography, as well as a six-minute walk test for older children. Some will require a heart catheterization for direct measurements of pulmonary hemodynamics and for guidance in mediation selection and dosage.

Depending upon the patient's underlying cause of PH and the severity of illness, the goals of the Pulmonary Hypertension Center range from reversing the PH to making children feel better and experience improved quality of life, to palliative care for the severest forms of the condition.

Pulmonary Hypertension Treatment Options

Traditionally, pulmonary hypertension carries a very poor prognosis. But the good news there have been many advances in newer therapies and medications that have improved both survival and quality of life.

Treatment options can include oxygen therapy and medications to help the heart pump better or relax the muscles in the walls of the blood vessels.  Other beneficial treatments may include oral or inhaled medicines and infusion therapy to deliver appropriate medicine continuously through their vein. Children may also require surgery such as a lung transplant or atrial septostomy, a surgical procedure that is intended to improve communications between the right and left atria and to relieve pressure on the right side of the heart.

The staff of the Pulmonary Hypertension Center actively participate in research studies that assist in the standard of care patients receive.

For further information on the Pulmonary Hypertension Center and other clinical services at DMC Children’s Hospital of Michigan, call (313) 745-KIDS or toll-free at (888) 362-2500