Despite rapidly deteriorating health, she would not undergo the treatment until the expected birth of her first grandchild.
“Whatever fate had in store, I was determined to see my beautiful granddaughter’s face. I simply would not take any chance of missing that. Once she arrived, I became even more determined to share a long life with her and the rest of my family. I’m grateful to the doctors at AGH for giving me that chance,” said Pastorius, 59, of Lyndora, PA
Pastorius suffers from chronic thromboembolic pulmonary hypertension (CTEPH), a condition in which large blood clots and scar tissue obstruct the network of pulmonary arteries that carry blood from the heart to the lungs, ultimately resulting in high pressure and heart failure.
CTEPH is a progressive disease that requires surgical intervention to offer patients any chance of an extended life, said Raymond Benza, MD, medical director of AGH’s Advanced Heart Failure, Transplantation, Pulmonary Hypertension and Mechanical Circulatory Support Program.
Fortunately for Pastorius and thanks to the efforts of Dr. Benza and AGH cardiothoracic surgeon Robert Moraca, MD, the hospital is now one of just a handful of U.S. medical centers that has acquired the advanced expertise to perform a sophisticated procedure for CTEPH called pulmonary thrromboendarterectomy (PTE).
Pastorius became the third patient to undergo the new procedure at AGH in July.
Prior to launching the new AGH program, Drs. Moraca, Benza and critical care physician Subbarao Elapavaluru, MD, spent six months observing and performing several PTE procedures at the University of Alabama in Birmingham under the guidance of David McGiffin, MD, one of the nation’s most experienced and prominent PTE specialists. Dr. Benza co-founded the CTEPH program at UAB with Dr. McGriffin prior to joining AGH.
Dr. McGiffin also traveled to Pittsburgh to participate in Pastorius’ surgery.
“Our surgical team completed an extraordinary amount of training in this sophisticated procedure. That, coupled with Dr. Benza’s experience in the field, ensured that from the very outset we could perform PTE with the same outcomes as the few specialized centers in the country,” said Dr. Moraca, who serves as Director of Cardiothoracic Surgical Research at AGH and Chief of the hospital’s section of Thoracic Aortic and Arrhythmia Surgery.
PTE is an eight to 10 hour long operation that involves opening the chest to expose the pulmonary arteries, connecting patients to a heart-lung bypass machine and cooling the body.
According to Dr. Elapavaluru, the bypass machine mimics the actions of the heart and lungs, providing oxygen to the blood and pumping it throughout the body. He said lowering the body temperature to around 18 degrees Centigrade (64 degrees Fahrenheit) reduces the body’s need for oxygen, which allows the surgical team to halt the bypass machine periodically to create a bloodless surgical field.
While no blood is being pumped through the arteries, the surgeon opens the vessels and carefully removes the scar tissue blocking the arteries. The on-off process of the heart-lung bypass is repeated until all of the scar tissue is removed and blood flow is restored to the lungs.
Pastorius was discharged from AGH six days following PTE surgery with normal pulmonary pressures, and eight weeks later, her breathing and quality of life has improved considerably.
At a recent follow up visit with Dr. Benza, she said feels like she is getting stronger every day.
“It’s really remarkable the difference this has made in my life. Before the surgery, I was tired and run down to the point where even the simplest of tasks were challenging. I’m breathing so much easier now and I’m looking forward to chasing around after my granddaughter!” Pastorius said.
CTEPH is often difficult to diagnose because its primary symptom in the early stages, shortness of breath, can signify a variety of conditions. As the degree of the pulmonary blockage progresses, however, additional debilitating symptoms may appear due to stress placed on the right side of the heart as it struggles to push blood through the blocked area and oxygenation of the blood is diminished. Those symptoms may include: difficulty breathing, water retention in the limbs (edema) due to the heart’s reduced ability to move fluid through the body’s blood vessels, chest pain, light-headedness and fainting (syncope).
Physicians diagnose the condition using a series of imaging tools, including echocardiography (sound wave pictures of the heart) and pulmonary angiography – or X-ray imaging of the pulmonary arteries.
Dr. Benza said for those who are candidates, the promise of a successful PTE surgery is substantial.
“With most patients who undergo the procedure, the pressure in the pulmonary arteries drops back to normal, shortness of breath dissipates and the dangerous right heart dysfunction that typically occurs as a result of the disease tends to resolve completely,” he said. “And most importantly, these results appear to persist over time in most patients.”
Studies show that five-year survival following PTE is approximately 75% in a patient group whose life expectancy without surgery is often just a few years.
“Developing expertise in PTE surgery is an important accomplishment for the Cardiovascular Institute that offers hope to patients from the greater Pittsburgh region who suffer from this very difficult and often fatal pulmonary vascular disease,” said Srinivas Murali, MD, Medical Director of the West Penn Allegheny Health System Cardiovascular Institute and Director of the System’s Division of Cardiovascular Medicine.
“The goal of the Cardiovascular Institute is to provide a complete spectrum of advanced surgical and medical care for complex cardiopulmonary diseases and it is extremely gratifying to add this important new surgical technique to our capabilities.”
For more information, contact:
The West Penn Allegheny Health System