When faced with unexplained shortness of breath, don’t forget about pulmonary hypertension

Pulmonary hypertension (PH) is a rare and potentially fatal condition caused by high blood pressure in the pulmonary arteries. Underdiagnosed, the majority of patients in Canada must wait more than two years for proper diagnosis.Typically, pulmonary hypertension manifests itself as dyspnea and fatigue. Since these symptoms are common to many heart and lung diseases, PH often flies under the radar of health professionals. “We regularly see young PH patients diagnosed with asthma, for example. Or their symptoms are attributed to obesity or anxiety,” noted Jamie Myrah, executive director of the Pulmonary Hypertension Association of Canada (PHA Canada). Treatment urgencyYet time is running out for these patients, since the average life expectancy of an untreated PH sufferer is less than three years. A recent report highlights the many challenges of access to care for PH patients across Canada.“When you've looked for common causes and are faced with unexplained shortness of breath, don't forget to consider pulmonary hypertension,” said cardiologist Dr. David Langleben, director of the division of cardiology at the Jewish General Hospital in Montreal. Patients suspected of having PH should undergo an echocardiogram, as this enables mPAP to be estimated using a non-invasive method. Unfortunately, patients face long delays in accessing the test—almost a year.In Quebec, if the echo indicates PH, the patient is referred to one of the province's two specialized pulmonary hypertension clinics, located at the Institut universitaire de cardiologie et de pneumologie de Québec (IUCPQ) in Quebec City and the Jewish General. "At present, this is sufficient to meet demand,” said Dr. Langleben. A stable patient is seen in two to three weeks. If they're unstable, we see them within 24 to 48 hours.”Pulmonary hypertension falls into five groups, depending on the underlying cause of the condition. It is often caused by narrowing and closure of certain pulmonary arteries. Many medical conditions increase the risk, including COPD, scleroderma, lupus, HIV, liver disease and pulmonary embolism. Some people are also more likely to develop the condition, particularly women, or if a family member carries a genetic mutation causing PH. Other risk factors include obesity, pregnancy, drug abuse and certain medications. The link between dasatinib, amphetamine-like appetite suppressants or methamphetamine and the onset of pulmonary arterial hypertension has been demonstrated, according to the British Journal of Pharmacology, with other medications likely linked. New antihypertensiveAt present, the drugs available to PH patients slow disease progression, reduce symptoms and improve survival, but do not cure the disease. All eyes are on sotatercept-csrk, already approved in the U.S. and currently under review by Health Canada. In clinical trials, this antihypertensive significantly increased the functional capacity of subjects suffering from pulmonary arterial hypertension (PAH), a type of PH characterized by high blood pressure in the smallest pulmonary arteries.Dr. Langleben is very optimistic for this new player on the market. “It gives amazing results. I've had 20 years of tears in my clinic, but this year, for the first time, I had tears of joy. I can't wait to be able to offer more of it to my patients,” he said.

When faced with unexplained shortness of breath, don’t forget about pulmonary hypertension

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