Pulmonary Hypertension: Early Diagnosis Tricky, But Essential
A 10-year-old girl was living an active life – school, activities, friends -- until a syncopal episode while visiting the zoo took her to the emergency room.
Emergency Department staff at Cincinnati Children’s Hospital Medical Center discovered that she had been experiencing troubling symptoms for some time. Over the previous 10 months, the young girl had become increasingly short of breath, tired easily and had experienced multiple pre-syncopal episodes. Her physical exam and serial tests revealed severe pulmonary hypertension.
After Russel Hirsch, MD, director of the Pulmonary Hypertension service, led a thorough evaluation that included echocardiograms and cardiac catheterization, the young girl began pulmonary vasodilator therapy. Six months later, she was asymptomatic, had no further syncope, and was able to return to school.
In the early stages of pulmonary hypertension, the changes in the lung’s blood vessels that cause increased resistance may be reversible, but in later stages they become permanent, with muscular thickening and fibrosis. These changes cause an elevation of the right ventricular pressure which, if not relieved, can result in right ventricular failure, arrhythmias and death.
The disease may have no known cause, or may be secondary to other diseases (such as congenital heart disease or collagen vascular diseases). The disorder has often progressed to late stage by the time it is diagnosed. The long-term prognosis depends on what causes the disease, its severity at the time of diagnosis, and response to therapy.
While there is no cure, there are treatments that have been found to relieve many of the symptoms, and that can slow or arrest progression of the disease. These treatments are available through the Pulmonary Hypertension Clinic.
For referrals, contact Michelle Bagby, RN, MSN, CNP at 513-803-0695 or michelle.bagby@cchmc.org.
