Throughout her life, Estee had been told that she would be unable to sustain a pregnancy because of her congenital heart condition. But in September 2022, she gave birth to a healthy baby girl with the help of her team of multidisciplinary specialists at the Mothers Center at NewYork-Presbyterian and Columbia.
Estee has been a NewYork-Presbyterian and Columbia patient since birth. At 20 weeks gestation, her mother had a fetal echocardiogram, which showed a complex malformation of Estee’s heart. “She had a univentricular heart,” says Robert J. Sommer, MD, a cardiologist and Director of Invasive Adult Congenital Heart Disease. “In her first few years of life, we had to perform three complex surgeries to correct her circulation and redirect the blood flow.”
Her third surgery – a Fontan operation – connected the major veins directly to the lungs, bypassing the heart altogether. While the surgery helped her heart function better, as she grew older it came with a host of complications: chronic liver disease, duodenal varices, arrythmias, and thrombocytopenia. Eventually, she required a pacemaker.
Because of all her comorbidities, Estee had always been cautioned against pregnancy. “Generally, patients with Fontans who don’t have other issues do very well with pregnancy,” says Dr. Sommer. “However, for patients like Estee who have such a complex medical history, there are a lot of potential risks that come with pregnancy, such as miscarriage, stroke, pump dysfunction, bleeding, and fetal growth restriction.”
Generally, patients with Fontans who don’t have other issues do very well with pregnancy. However, for patients like Estee who have such a complex medical history, there are a lot of potential risks that come with pregnancy, such as miscarriage, stroke, pump dysfunction, bleeding, and fetal growth restriction.
— Dr. Robert J. Sommer
“Pregnancy places a high strain on the woman’s body,” he adds. “The heart has to increase its workload by about 40% to compensate for the extra blood that it has to pump to the baby.”
But recognizing how important having a family was to Estee, Dr. Sommer connected her with Stephanie Purisch, MD, a maternal-fetal medicine (MFM) specialist and co-director of the Mothers Center Heart Program at NewYork-Presbyterian and Columbia.
“I was comfortable with her moving forward with pregnancy from a cardiac perspective; however, she needed to be seen by Dr. Purisch and the team at the Mothers Center to assess her other risks. They are the true experts on caring for heart patients during pregnancy, “he says. “Not everybody should be taking care of patients like Estee, but that’s why there are places like NewYork-Presbyterian and the Mothers Center to take care of these high risk, complex cases.”
The Path to Motherhood
Estee began her preconception counseling in August of 2021. “That’s when we assess what a patient’s underlying heart disease is and get an understanding of their functional status, any past cardiac complications, surgeries, medications, and how well they’ll tolerate pregnancy,” says Dr. Purisch.
Not everybody should be taking care of patients like Estee, but that’s why there are places like NewYork-Presbyterian and the Mothers Center to take care of these high risk, complex cases.
— Dr. Robert J. Sommer
A big component of the preconception counseling was making sure that Estee understood her risks. “We discussed what specific maternal cardiac risks a pregnancy would have, what obstetric risks there would be, and what the implications would be for her potential offspring,” says Dr. Purisch. “We also discussed how pregnancy would impact the long-term trajectory of her cardiovascular disease.”
We discussed what specific maternal cardiac risks a pregnancy would have, what obstetric risks there would be, and what the implications would be for her potential offspring. We also discussed how pregnancy would impact the long-term trajectory of her cardiovascular disease.
— Dr. Stephanie Purisch
At a minimum, the preconception evaluation and risk assessment involves:
- An electrocardiogram
- An echocardiogram
- Assessment of the New York Heart Association (NYHA) functional classification either by history or exercise testing
- Review of medications
- Detailed history of cardiac and other medical and surgical history
- Family history and genetic assessment
Dr. Purisch used two cardiovascular risk assessment tools: the modified World Health Organization (WHO) risk classification – which estimates maternal cardiac risk based on diagnosis, and the CARPREG II risk score, which incorporates information about the patient’s diagnosis as well as echocardiogram findings, history of cardiac events, and social determinants of care.
Based on the assessments, Estee was thought to have at least a 40% risk for maternal cardiac events in pregnancy; and her associated health issues – such as her duodenal varices and thrombocytopenia- posed additional risks to her health which were harder to quantify. Ultimately, in partnership with her care team, she decided that she wanted to pursue pregnancy. She conceived naturally and, in March 2022, at eight weeks pregnant, she began her prenatal care with Dr. Purisch.
A Multidisciplinary Approach to Prenatal Care
Dr. Purisch assembled “Team Estee,” made up of specialists across NewYork-Presbyterian and Columbia’s cardiology, hepatology, hematology, psychiatry, pulmonology, and obstetrics anesthesia departments, including:
- Dr. Sommer
- Cardiac electrophysiologist Angelo B. Biviano, MD, MPH
- Hepatologist Lorna M. Dove, MD
- Hematologist Imo J. Akpan, MD
- Obstetrics anesthesiologist Kyra Bernstein, MD
Mothers Center patients all have different teams based on their specific medical concerns. What remains constant is the frequency at which they are seen by their MFM specialist. “Patients see me at least once a month in early pregnancy and that goes to every two weeks in the third trimester, and then weekly as they approach their due date from 36 weeks and beyond,” says Dr. Purisch. Visits, however, can be more frequent based on the patient’s condition. “For Estee, I was assessing her cardiac condition at every visit so that if I thought she needed to see Dr. Sommer or one of her other providers, I could make sure that connection happened. She probably saw me every other week for most of her pregnancy and I had check-ins with our team on a regular basis.”
Having thrombocytopenia put Estee at risk for bleeding during the pregnancy, which was Dr. Akpan’s main concern. At the same time, pregnancy and the Fontan physiology placed Estee at risk for clotting. Dr. Akpan managed Estee’s competing risks by keeping her on a regimen of baby aspirin and a prophylactic anticoagulant to prevent blood clots, while monitoring her labs and symptoms closely throughout. “She had some gum bleeding here and there and a little bit of bruising, but she did not have any severe bleeding complication during her pregnancy,” says Dr. Akpan. “We stayed on top of it.”
Estee’s pregnancy was progressed with some of the complications, as anticipated, but were identified early and managed appropriately by her team. She had an endoscopy early on to assess the status of her duodenal varices and to make sure they weren’t progressing. She also experienced some arrhythmias, which were managed by medication. At 26 weeks, she encountered her biggest complication: fetal growth restriction.
Fetal growth restriction is common in any patient with congenital heart disease but particularly with patients who have had Fontan surgery. At baseline, patients like Estee have lower cardiac output and lower oxygen levels. This was something that we spoke about at our initial visit. I told her, ‘I want you to be prepared that this baby will most likely be small and born preterm.’
— Dr. Stephanie Purisch
“Fetal growth restriction is common in any patient with congenital heart disease but particularly with patients who have had Fontan surgery,” says Dr. Purisch. “At baseline, patients like Estee have lower cardiac output and lower oxygen levels. This was something that we spoke about at our initial visit. I told her, ‘I want you to be prepared that this baby will most likely be small and born preterm.’”
Managing a Complicated Delivery
Estee was induced in the late preterm period, at 36 weeks and six days. Delivery can be complicated for mothers with congenital heart disease, as the physiology of labor puts an immense amount of stress on the heart. Even so, a vaginal delivery is still preferred.
“One of the things that we’ve learned over the years in taking care of patients who are pregnant with congenital heart disease is that vaginal delivery is pretty well tolerated,” says Dr. Sommer. “People who have heart failure symptoms and get short of breath with exercise can still exercise for a short period of time. That’s what labor and delivery is — it’s short, intense bursts of activity intervened with periods of rest. Cesarean section is less tolerated because there are big fluid shifts and a greater risk of bleeding.”
One of the things that we’ve learned over the years in taking care of patients who are pregnant with congenital heart disease is that vaginal delivery is pretty well tolerated. People who have heart failure symptoms and get short of breath with exercise can still exercise for a short period of time. That’s what labor and delivery is — it’s short, intense bursts of activity intervened with periods of rest. Cesarean section is less tolerated because there are big fluid shifts and a greater risk of bleeding.
— Dr. Robert J. Sommer
Throughout her labor, delivery and early postpartum period, Estee was monitored in NewYork-Presbyterian and Columbia’s critical care obstetrics unit (CCOB), which is where patients who need telemetry and/or invasive monitoring during labor and delivery are cared for. During the final stages of Estee’s labor, she became hypoxic and required supplemental oxygen and a forceps-assisted delivery. While the forceps helped alleviate extra burden on her heart, the delivery was further complicated by a postpartum hemorrhage. “I think she was particularly sensitive to blood loss, more than someone without cardiac disease,” says Dr. Purisch. “She was not able to tolerate even mild anemia. She required a significant transfusion.”
Estee received four units of red cells, two units of fresh frozen plasma, and one unit of cryoprecipitate. By the following day, she still required supportive oxygen but not blood pressure support. “By postpartum day one, she was off oxygen,” says Dr. Purisch. “She still needed close monitoring and diuretics, but she was definitely on her road to recovery.”
Estee remained in the hospital longer than typical cardiac obstetric patients due to her coexisting conditions. “We were monitoring her labs, as her liver function tests went up,” says Dr. Purisch. “We were making sure she did not have preeclampsia. She had a brief episode of ventricular tachycardia and palpitations that resolved, and we kept her on prolonged telemetry to make sure she did not have more arrhythmias.”
She was released on postpartum day six to recover at home. “Once she left the hospital, she did very, very well,” says Dr. Purisch. “We did a video visit at one-to-two weeks postpartum, which is standard. She came for her postpartum visit at six weeks.”
Estee with her daughter nearly two years after her birth.
Nearly two years after her pregnancy, Estee is weighing whether she wants to pursue another one.
“Although her cardiac status is fairly similar to how it was before, there’s been a little bit of progression with her liver disease with some new esophageal varices,” says Dr. Purisch. “If Estee understands the risks and decides that she wants to move forward, Team Estee will reassemble, and we’ll do what we have to do to get her through the pregnancy.”
Although her cardiac status is fairly similar to how it was before, there’s been a little bit of progression with her liver disease with some new esophageal varices. If Estee understands the risks and decides that she wants to move forward, Team Estee will reassemble, and we’ll do what we have to do to get her through the pregnancy.
— Dr. Stephanie Purisch
The Mothers Center’s Difference
The multidisciplinary care model used at the Mothers Center is truly unique, as each case involves individualized planning and collaboration across the medical center. “Everyone in our maternal-fetal medicine division comes together with the specialists from other disciplines at our multidisciplinary meetings,” Dr. Purisch says. “It’s essential for patient care, and important for trainee education because fellows have a key role in our conferences. This model ensures that delivery plans and outcomes are the best possible.”
Dr. Sommer adds that not all institutions are equipped to treat such medically complex pregnancies. “At NewYork-Presbyterian, many women with complex congenital heart issues can be mothers, it just requires that they have a comprehensive assessment and a team of doctors, like Estee had, who are comfortable in their management,” he says.
We aren’t afraid to take on tough cases because we interact with our colleagues, we discuss these cases as a multidisciplinary team, and we come up with a plan that is unique to the patient. We have patients who will go to other places and people will say no to working with them, so they come to us. We’re willing to work with you and see what happens because we collaborate very well to provide high quality and personalized care
— Dr. Imo J. Akpan
“We aren’t afraid to take on tough cases because we interact with our colleagues, we discuss these cases as a multidisciplinary team, and we come up with a plan that is unique to the patient,” Dr. Akpan adds. “We have patients who will go to other places and people will say no to working with them, so they come to us. We’re willing to work with you and see what happens because we collaborate very well to provide high quality and personalized care.”
Pregnancy is a very exciting time, but when you have complicated medical problems, it can be incredibly stressful. We really support our patients so that we – the providers - can worry about the medical issues, and they can try to have as normal of a pregnancy experience as possible
— Dr. Stephanie Purisch
“At the Mothers Center, we really individualize our patient care and bring all our expertise together to take the best care of our patients,” says Dr. Purisch. “Pregnancy is a very exciting time, but when you have complicated medical problems, it can be incredibly stressful. We really support our patients so that we – the providers - can worry about the medical issues, and they can try to have as normal of a pregnancy experience as possible.”
