Hope for Patients with Rare Form of Pulmonary Hypertension
By Brenda Lange
Chronic thromboembolic pulmonary hypertension, or CTEPH, is rare, occurring in about 3% of patients who experience an acute pulmonary embolism. And though most cases can be treated using an innovative surgery called pulmonary thromboendarterectomy, which clears the pulmonary arteries of chronic blockages, the surgery is complex and is performed only by surgeons experienced in the procedure.
About 300 surgeries—approximately 20 per year—have been performed at Columbia’s Pulmonary Hypertension Comprehensive Care Center, one of the few centers in the eastern United States that offer the procedure. The program, established in 2009, is now led by cardiothoracic surgeon Koji Takeda, MD, PhD, surgical director of heart transplant and mechanical circulatory support for the Division of Cardiothoracic Surgery.
“Pulmonary thromboendarterectomy is the only curative therapy for CTEPH,” says Dr. Takeda, associate professor of surgery. These patients “are very sick” and typically have serious shortness of breath often requiring oxygen. Additional symptoms—fatigue, chest pain, cough, and edema—can make an accurate diagnosis difficult because of their similarities to symptoms of other disorders, including heart failure.
Diagnostic tests include an echocardiogram, pulmonary angiogram, and examinations by a pulmonary hypertension specialist and interventional cardiologists. This multidisciplinary approach allows for accurate planning and avoids delays in treatment.
Patients who have certain comorbidities or are too weak may not be appropriate candidates for the surgery, which takes up to six to eight hours to perform and requires deep hypothermia circulatory arrest. During the procedure, the surgical team uses special tools to gently loosen the clots, bit by bit, and remove them. Clots may extend from the main arteries into the smaller arteries of the entire lung.
“Age is not a comorbidity,” says Dr. Takeda, adding that he has operated on an 80-year-old woman who had a good outcome. However, patients with a higher body mass index, low preoperative hemoglobin levels, low ejection fraction, and low creatinine are more likely to develop acute kidney issues after the procedure, resulting in longer hospital stays and higher mortality rates.
The procedure is considered high-risk but is getting safer, says Dr. Takeda, and survival rates have improved. The one-year survival rate is now 92%, and the five-year rate is 88%
Columbia’s Pulmonary Hypertension Comprehensive Care Center, one of the largest in the country, offers advanced diagnostics and targeted medical treatments for children and adults with all types of pulmonary hypertension. Contact the center at (212) 305-4436.