The understanding of pulmonary hypertension (PH) continues to evolve. The threshold to diagnose PH was lowered to mPAP ≥ 20 mmHg in the 6th World Symposium on PH in 2018. The therapeutic approach has also changed over time, favoring aggressive use of multiple pulmonary vasodilators in combination upfront and escalation of therapy based frequent assessment of disease progression. The changes to diagnostic criteria and management underscore the need to identify the disease early and to treat it aggressively.
“We previously used a mean pulmonary artery pressure of 25 to define PH. But delays in diagnosis and initiation of treatment can lead to progressive right heart failure and a poor prognosis,” says Chetan Naik, MD, MS, Director of the Pulmonary Hypertension Program and transplant pulmonologist on the medical staff at Baylor University Medical Center, part of Baylor Scott & White Health. “Management of PH is based on the physician’s ability to predict disease progression in the short-term using risk-stratification tools. It is important to start treatment as soon as possible. At least 10 effective medications have been introduced within the last decade, and all are designed to lower blood pressure within the lungs and help the right ventricle recover.”
Because PH can develop as a primary disease of the pulmonary arteries or can complicate diseases affecting the heart, lungs and certain systemic disorders, Dr. Naik emphasizes that management of the disease requires a multidisciplinary approach. The Baylor Dallas pulmonary hypertension program is designed to facilitate collaboration among advanced lung disease specialists, advanced heart failure specialists, transplant pulmonologists and thoracic surgeons. These physicians work closely with allied services, including genetic counselors, physiotherapists, dieticians and palliative care services. Baylor Dallas is in the process of seeking accreditation as a comprehensive care center for PH from the Pulmonary Hypertension Association.
“Patients with PH require close and frequent monitoring and risk assessment,” Dr. Naik says. “We have an excellent program at Baylor Dallas for pulmonary hypertension with all available testing modalities. Routinely used testing includes right heart catheterization, echocardiography, radiology, including cardiac MRI, cardiopulmonary exercise testing and pulmonary function testing. We can also offer patients who are candidates participation in ongoing clinical trials in PH.” The Baylor Dallas pulmonary hypertension program was established more than 10 years ago and follows more than 500 patients in the outpatient clinics at Baylor Dallas and Baylor Scott & White All Saints Medical Center – Fort Worth. An inpatient service is available for patients who need advanced vasodilator therapy. While lung transplant is rarely indicated for PH as a result of medication efficacy, Baylor Dallas has a robust program for lung transplantation for patients with advanced PH who are refractory to medical therapy.