INDIANAPOLIS- The Pulmonary Hypertension Program, supported by the Divisions of Pulmonology and Cardiology within Pediatrics, at Riley Children’s Health, is the first and only program in Indiana that provides expert care for children with pulmonary hypertension as a primary diagnosis or as a result of heart or lung disease.
Pulmonary arterial hypertension (PAH) is high blood pressure in the blood vessels of the lungs and the right side of the heart. While PAH was once considered rapidly fatal, it can now be treated with many medications, which have been developed just within the past two decades. Although medical treatment has advanced, the median time for a PAH diagnosis is still 1.1 years, and statistically patients see three or more different physicians before they are properly diagnosed.
Co-Directors, Gregory Montgomery, MD, and Michael Johansen, DO, shared, “Our pediatric pulmonary hypertension program is a fully integrated partnership between the divisions of Pediatric Pulmonology and Pediatric Cardiology, guaranteeing a complete evaluation of each patient's unique cardiopulmonary physiology.” They continued, “The Riley PH Program is truly a situation where two physicians collaboratively sit face-to-face and discuss each patient in detail, challenging each other's assertions and creating the most effective diagnostic or treatment plan.”
Since 2011, The Pulmonary Hypertension Association’s Scientific Leadership Council, 28 global leaders in the field of pulmonary hypertension, have spearheaded the PHA-Accredited PH Care Centers (PHCC) initiative to establish a program for accreditation of centers with special expertise in pulmonary hypertension (PH), particularly pulmonary arterial hypertension (PAH), to raise the overall quality of care and outcomes in patients with this life-threatening disease.
Recently, the Pediatric Pulmonary Hypertension Program at Riley Children’s Hospital became a PHA - Accredited Pulmonary Hypertension Center of Comprehensive Care. Montgomery and Johansen said, “From the beginning, almost 10 years ago, we used the detailed guidelines for the PHA’s Pediatric Comprehensive Care Centers as a framework and guide as we carefully constructed this program over time. For us, receiving accreditation from the PHA is validation that we have built a program of national distinction that provides exceptional care for children with what is often a very concerning, even life-threatening diagnosis.”
Although there is no cure for pulmonary hypertension, the Riley Children’s Hospital team can manage conditions through adolescence and early adulthood using oral, inhaled, intravenous or subcutaneous medicines. Montgomery and Johansen shared, “There are a limited number of medications that are formally approved by the FDA for use in children with pulmonary hypertension. To address this, we continue to actively enroll children in several investigative clinical research trials that are looking at the effectiveness in children of PH medications already approved for adults.”
Montgomery and Johansen are in the early stages of organizing a larger project to better understand the development of pulmonary hypertension in children with a type of severe congenital heart disease that requires a complex surgery called a Fontan procedure. Their goal for this project is to better appreciate how pulmonary vascular remodeling occurs in some of these patients, allowing us to identify worrisome changes earlier and to develop more aggressive medication therapies either to treat or even prevent these changes.