Recent updates modify definition of pulmonary hypertension

The understanding of pulmonary hypertension (PH) continues to slowly evolve over time. At the 6^th World Symposium on Pulmonary Hypertension in 2018, the threshold to diagnose PH was lowered to mPAP ≥ 20 mmHg. Most recently, the 2022 guidelines issued by the European Society of Cardiology and European Respiratory Society added to this new definition by lowering the threshold for pulmonary vascular resistance (PVR) to >2 instead of >3.

“Pulmonary hypertension is a rare disease that is inherently progressive in nature. Untreated PH results in progressive right ventricular failure, and, ultimately, death,” says Chetan Naik, MD, MS, medical director of the pulmonary hypertension program and transplant pulmonologist on the medical staff at Baylor University Medical Center at Dallas (Baylor Dallas), part of Baylor Scott & White Health. “This latest change to the PVR number is another step in increasing our ability to diagnose PH early so that patients may be regularly screened or started on effective therapy before the disease progresses.”

Patients who should be screened early and regularly include those at risk for PH due to familial disease or those who have co-morbid conditions that predispose them to PH. PH should also always be in the differential diagnosis of unexplained dyspnea. Because echocardiography remains an important screening tool – it is noninvasive and easily accessible – Baylor Dallas has instituted a new PH-specific limited protocol echocardiogram to identify additional signs of PH in at-risk patients.

“I can’t emphasize enough how important early detection of PH is,” says Dr. Naik. “While vasodilators are the backbone of therapy for PH, we have at least 10 medications that are efficacious against the disease. All are designed to lower blood pressure within the lungs and help the right ventricle recover. In addition, Baylor Dallas is the site of several clinical trials of next-generation PH medications that target the underlying pathophysiological mechanisms of the disease, and many of our patients are candidates for these trials.

Because PH can develop as a primary disease of the pulmonary arteries or can complicate diseases affecting the heart, lungs and certain systemic disorders, 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 & palliative care services.

The Baylor Dallas pulmonary hypertension program was established more than 12 years ago and follows more than 600 patients in outpatient clinics at Baylor Dallas and Baylor Scott & White All Saints Medical Center – Fort Worth. While lung transplant is rarely indicated for PH as a result of medication efficacy, Baylor Dallas has a robust program lung transplantation for patients with advanced PH who are refractory to medical therapy.