LOS ANGELES (FRONTLINE MEDICAL NEWS) – Dyspnea in pulmonary hypertension is caused by mitral valve disease until proven otherwise, according to Paul Forfia, MD, director of pulmonary hypertension, right heart failure, and pulmonary thromboendarterectomy at Temple University, Philadelphia.

Although mitral valve disease is a well-recognized cause of pulmonary hypertension, its significance is often underestimated in practice.

“Whether the valve is regurgitant or stenotic makes absolutely no difference. When you delay” repair or replacement, “the patient keeps getting sicker,” he said. In time, “everyone is standing around wringing their hands going, ‘Oh my god, what are we going to do? Are you serious? Fix the valve.’ We see this type of patient a couple times a month,” Dr. Forfia said at the American College of Chest Physicians annual meeting.

“I have seen lifesaving mitral valve surgery put off for many years in patients with pulmonary hypertension, when all they needed was to have their valve fixed,” he said.

A few things could explain the problem. Prevention of rheumatic fever has made mitral stenosis far less common than in the past, so cardiologists may not be as good at diagnosing it. The increased attention on pulmonary hypertension in recent years may also have eclipsed the importance of underlying mitral valve disease and the need to address it, said Dr. Forfia.

Whatever the case, pulmonologists who want the valve fixed often end up playing patient ping pong with cardiologists who want the hypertension controlled beforehand, but “if I treat the pulmonary circulation first, all I am going to do is unmask the left heart failure. There will be no functional improvement whatsoever,” Dr. Forfia said.

Surgery is the best solution as long as patients are well enough to recover. “With pulmonary hypertension in the setting of severe mitral valve regurgitation or stenosis, whether the pulmonary hypertension is related to passive left heart congestion or associated with pulmonary arteriopathy, the only sensible option is to correct the underlying valvular abnormality,” he said. The surgery should be done at an institution capable of managing postop pulmonary arteriopathy, if present.

The ping pong solution is to send patients to an expert pulmonology center; the mitral valve problem will be spotted right away.

“There is no pulmonary pressure cutoff that should prohibit surgery” in patients able to recover. “There is no such thing as a pulmonary artery pressure too high to be explained by mitral valve disease. The pulmonary pressure can be as high as it wants to be. You will get nowhere by thinking the pressure is too high to address the valve,” Dr. Forfia said.

Often “you hear, ‘I’m afraid the person is going to die on the table.’ I always say ‘if the patient is not going to die on the table, they are going to die in their living room of progressive heart failure because you [didn’t] fix their valve. I have never had a patient with pulmonary hypertension not separate from cardiopulmonary bypass. It’s a myth,” he said.

When there’s a “question if the dyspnea is coming from the mitral valve, we routinely use exercise right heart catheterization to probe the situation. We have a recumbent bike in the cath lab. You’ll often provoke significant left heart congestion with a low workload. It’s very revealing to the significance of mitral valve disease,” he said.

Aortic valve disease is also missed in pulmonary hypertension. “It’s not [a] similar” problem; “it’s the same” problem, Dr. Forfia said.

Dr. Forfia is a consultant for Bayer, Actelion, and United Therapeutics.


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