Pulmonary hypertension (PH) is a rare condition in the general public, but more common in patients with congenital heart disease (CHD). Some cardiologists specializing in CHD may identify PH but lack sufficient experience in treating the condition. Conversely, some pulmonologists who treat PH may not identify CHD as the underlying cause. These knowledge gaps can have an adverse impact on CHD patients who develop this very serious lung problem.
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Cleveland Clinic PH and CHD specialists sought to bridge this gap and improve patient care by combining their expertise in a Congenital Pulmonary Hypertension Clinic.
“For PH patients with CHD, multidisciplinary, coordinated care can make an enormous difference in quality and length of life,” says Cleveland Clinic pulmonologist and PH expert Neal Chaisson, MD, who established the clinic with adult CHD specialist Joanna Ghobrial, MD, MS, and other colleagues.
“PH is a significant threat to survival and quality of life. Even when it doesn’t kill, it can still affect a patient’s functional capacity to a significant degree,” he says.
Patients who visit the Congenital Pulmonary Hypertension Clinic meet with Dr. Chaisson and Dr. Ghobrial together. Following a careful history and physical examination, the physicians may arrange for the patient to meet with a dietitian, respiratory therapist, electrophysiologist, cardiac surgeon, behavioral health specialist or palliative medicine specialist during the same appointment.
“Our goal is to learn everything we can about your conditions and create a personalized, comprehensive plan of care for you to follow,” says Dr. Chaisson.
When CHD is the issue
A subset of patients referred to Dr. Chaisson ultimately are found to have CHD. Seeing these patients in the Congenital Pulmonary Hypertension Clinic allows them to begin appropriate treatment without delay.
“In some patients, this requires initiation of cardiac medications or proceeding with diagnostic catheterization or transcatheter interventions or open heart surgery, depending on the congenital defect,” says Dr. Ghobrial.
Furthermore, heart, lung or combined heart and lung transplantation may be required in a subset of patients. “Together, Dr. Ghobrial and I determine how best to treat the patient safely in hopes of creating a situation that is favorable for transplantation,” says Dr. Chaisson.
The complexities of PH and CHD mean that initial diagnoses are not always correct.
“It can be hard to tease out what is causing symptoms when a patient has complex underlying disease,” says Dr. Chaisson. “This is where two sets of eyes, and the experience of diverse specialists, can be very helpful.”
One patient with a congenital anomaly came to the Congenital Pulmonary Hypertension Clinic after developing dyspnea when running. He was initially told this was due to asthma and exposure to fumes during military service. Another physician thought it might be due to PH. To clear up the confusion, he sought another opinion in Cleveland. After several tests and discussions, the team ruled out both PH and asthma and identified a pulmonary vein obstruction as the source of his symptoms. Dr. Ghobrial subsequently stented the obstructed vessel, and the patient’s dyspnea disappeared.
In another case, Dr. Ghobrial suspected one of her patients with complex CHD also had PH. They met with Dr. Chaisson, who started the patient on medications. When she failed to improve, additional testing revealed new, intermittent atrial fibrillation. When this was corrected, the patient’s functional capacity tripled.
“In both cases, Dr. Chaisson and I saw these patients in a coordinated, timely manner and worked through their issues together,” says Dr. Ghobrial.
Partners in care
It is not necessary to have an accurate diagnosis before referring a patient to the Congenital Pulmonary Hypertension Clinic. CHD patients may be referred to rule out PH or receive help in identifying why current therapy is failing.
“Feel free to contact us. We will be happy to help you decide whether the referral will be appropriate and if we think we can help,” says Dr. Chaisson.
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