 The heart team at Our Lady of Lourdes Medical Center last month performed a unique valve replacement procedure on a 25-year-old pregnant woman, the first known case of its kind.
Arthur Martella, MD, chief of cardiothoracic surgery, and Ibrahim Moussa, DO, FACC, FSCAI, RPVI, medical director of the Structural Heart Program, used an Edwards Sapien valve to replace Elenilza Souza’s faulty mitral valve prosthesis.
The Edwards Sapien valve is commonly used in transcatheter aortic valve replacement (TAVR) and is also FDA approved for transcatheter mitral valve-in-valve replacement (TMVR). TMVR is a therapeutic option for patients with contraindications, are at high-risk for surgical treatment, or have a failed surgical bioprosthesis.
“Ms. Souza was having heart failure symptoms for the past six months. She was diagnosed with a failed mitral valve bioprosthesis and very shortly thereafter became pregnant. This procedure was essential for the heart to keep up with the demand of her body and her growing fetus. She would not be able to carry her baby to term or survive the stress of delivering a baby without correcting her valve disease,” said Dr. Moussa. “There would be an increased chance of mortality for her and her baby.”
A Childhood Illness
Souza, a native of Brazil, suffered rheumatic fever as a child. As she got older, she began to develop mitral stenosis—a narrowing of the mitral valve that regulates blood flow from the left atrium to the left ventricle—that left her gasping for breath at the slightest exertion. At age 18, she received a bioprosthetic valve made from heart tissue of a cow, which relieved her condition.
Souza moved to the United States to work as an au pair, taking care of two children. She started going to the gym.
“I worked out so I could be in good shape, to keep moving with the kids,” Souza told the Philadelphia Inquirer.
But by the time she married last December, the symptoms of her mitral valve stenosis began to return—a sign that her replacement valve was failing. Symptoms of mitral valve stenosis include shortness of breath, fatigue, swollen feet or legs, heart palpitations, dizziness and chest discomfort.
Charting a Course
Souza received initial treatment from Lourdes cardiologist Jayesh Khatiwala, MD, who referred her to Dr. Moussa. Initially, the team planned to use a mechanical valve, which would surgically replace her bioprosthetic valve. However, about three weeks before the procedure, Souza informed her doctors that she was nine weeks pregnant.
“People who receive a mechanical valve need to take Coumadin, a blood thinner, for the rest of their lives,” said Dr. Moussa. “However, Coumadin carries potential serious risks to the fetus, including birth defects. After consultations with Lourdes maternal/fetal physicians and other members of our multidisciplinary team, we decided to use an Edwards Sapien valve, with which she can use a blood thinner that does not pose the same risks.”
They also couldn’t delay doing the procedure, as Souza’s body would be producing more blood with every month to cope with the growing demands of pregnancy. Without a new valve to handle the increased flow, the lives of the fetus and mother would be at risk, said Dr. Martella.
“By the time you get to that third trimester, she would be in heart failure,” he said.
Switching to Plan B
Dr. Moussa and Dr. Martella planned extensively for the April 16 procedure, meeting multiple times with colleagues in pharmacy, anesthesia, maternal fetal medicine and cardiology.
“I wanted everybody to think about the worst-case scenario,” said Dr. Moussa. “We had her ready for Plan A and Plan B and Plan C in case things didn’t work out.”
Dr. Martella and Dr. Moussa planned to insert the new valve through a catheter inserted in Souza’s femoral vein. After reaching the heart, they would need to poke a hole through the atrial septum, a wall between the heart’s two upper chambers.
But once in the operating room, they found that because of Souza’s earlier open-heart surgery, her septum was tough and scarred. They switched to a transapical approach, which calls for the valve to be inserted through a small incision in the chest between the ribs.
Once inside the heart, the new valve was then expanded and placed inside the old one. It pushed aside the leaflets of the old valve and began to work immediately.
The procedure took six hours. She was discharged after several days in the hospital. She is now doing well and is expecting the baby in October.
“It’s incredible what they did,” Souza said.
“Now we feel confident that we gave her and her unborn child a fighting chance,” said Dr. Moussa. “We cannot find any evidence of another mitral valve-in-valve replacement in a 15-week pregnant woman, so this was a unique case. I’m pleased that through the advanced care provided at Lourdes, we were able to help this patient and will be following her through her pregnancy.” |
