– Interventions that are simple and straightforward for other patients – such as therapeutic phlebotomy, bronchoscopy, anticoagulation, administration of IV antibiotics – can quickly have catastrophic results in patients with Eisenmenger syndrome, Carole A. Warnes, MD, cautioned at the Annual Cardiovascular Conference at Snowmass sponsored by the American College of Cardiology.

Dr. Carole A. Warnes is a professor of medicine at the Mayo Clinic in Rochester, Minn., and director of the Snowmass conference. Bruce Jancin/MDedge News

Dr. Carole A. Warnes

This is why the 2018 ACC/American Heart Association guidelines on the management of patients with adult congenital heart disease recommend as a Class I indication that most patients with ACHD, including all those with Eisenmenger syndrome, be managed in collaboration with a cardiologist at a specialized ACHD center (Circulation. 2018 Aug 16. doi: 10.1161/CIR.0000000000000603), noted Dr. Warnes, professor of medicine at the Mayo Clinic in Rochester, Minn., and director of the Snowmass conference.

“There are lots of mistakes in diagnosis and management because so many physicians are unfamiliar with the kinds of problems these patients face,” the cardiologist said.

She cited as a real-world example a patient with Eisenmenger syndrome admitted for pneumonia. Twenty minutes after being placed on intravenous antibiotics she had a stroke. Why?

“She didn’t have an air filter on her IV line. Any air going into a cyanotic’s blood stream will go to the head and give them a stroke. Something that is simple – routine for other patients – may kill a patient with cyanotic heart disease,” Dr. Warnes said.

Another illustrative case: a patient with Eisenmenger syndrome presents with hemoptysis, an infiltrate in her right lung, a hemoglobin of 19.8 g/dL, and anemia. Should she undergo therapeutic phlebotomy? How about urgent bronchoscopy?

No and no, Dr. Warnes emphasized.

“You do not phlebotomize cyanotic patients unless they have symptoms of hyperviscosity, meaning terrible headache or poor concentration, along with a hemoglobin greater than 20 g/dL. They need that high hemoglobin. They may be blue, and you need those red cells to carry the oxygen around. Otherwise you may make them worse. And if you give them iron for their anemia, you will increase their risk of stroke,” she explained.

Hemoptysis in patients with Eisenmenger syndrome is a life-threatening warning sign. By the time Eisenmenger syndrome patients reach age 40 years, nearly all of them have experienced episodes of hemoptysis. And more and more patients with the syndrome are moving well beyond that milestone and surviving into their 60s and beyond.

“Many Eisenmenger syndrome patients are not dying when they’re 20 or 30. We can get them to a good old age,” according to Dr. Warnes, who founded the ACHD center at the Mayo Clinic.

Most of the time the cause of hemoptysis in patients with Eisenmenger syndrome is an intrapulmonary hemorrhage. Anticoagulation can turn that hemorrhage catastrophic. So can bronchoscopy. Indeed, the cause of death in roughly 30% of patients with this form of congenital heart disease is catastrophic hemoptysis.

“You bar the door from your friendly pulmonologist who wants to bronchoscope these patients. Bronchoscopy never does any good unless the patient is so hypoxic that you need to suck the blood out of their lungs,” Dr. Warnes emphasized.

Anticoagulation is ordinarily to be avoided in patients with Eisenmenger syndrome because of their bleeding risk, even when pulmonary thrombosis is suspected, she added.

So, to summarize: These patients basically need stabilizing, with no anticoagulation, no bronchoscopy, and no phlebotomy, she said.

Another danger zone for patients with Eisenmenger syndrome is pregnancy. The condition is associated with a 30% maternal mortality rate.

Dr. Warnes noted that, even at the Mayo Clinic, which has had a pioneering ACHD center for decades, awareness of key elements of management of affected patients is low among nonspecialists.

“As I’ve done my hospital services in the last couple of months, I sort of felt I was on patrol, snatching these patients from problems,” she recalled.

Dr. Warnes reported having no financial conflicts regarding her presentation.