Sponsor Content

This Hard-to-Detect Lung Condition Can Be Fatal During Pregnancy. Philly Doctors Found a Life-Saving Approach in the Heart

While being among the most beautiful experiences in life, pregnancy is one of the most emotionally intense and complex events a person can go through, psychologically, emotionally, and physically. When life-threatening complications are also part of the experience, this major life event can become the most stressful journey imaginable. 

For people with pulmonary arterial hypertension (PAH), this is a reality, and far too often, the worst occurs. The mortality rate for pregnant individuals with PAH can be between 30 and 50 percent. The risk is so high that, if people do discover that they have the condition prior to pregnancy (and many do not), they are advised to avoid ever becoming pregnant due to the condition’s life-threatening nature. 

And still, across the U.S., cardiac-related mortality in pregnant people is far too common. 

“The United States, surprisingly, has a relatively high risk of maternal mortality, even as a first-world country with advanced health care,” says Dr. Anjali Vaidya, co-director of the Pulmonary Hypertension, Right Heart Failure and CTEPH Program at Temple Health. “And the majority of that comes from cardiac risk of mortality.”

“We do have to be able to treat these patients because often they will not know they have the condition until they’re pregnant,” says Dr. Laura Hart, a maternal fetal medicine physician and obstetrician who works with Vaidya at Temple Health.

One glimmer of hope, at least in regards to the stark problem of PAH, is that, for the last decade, Paul Forfia, MD, Vaidya, and their team have produced a shocking result from treating the condition. In a recently published study, the team reported that they have developed an approach that has allowed them to maintain a zero-percent mortality rate in pregnant individuals with PAH. 

“Even in the early years, it was clear that this was a unique, valuable contribution to the field of pulmonary hypertension because all the reported outcomes in the literature to date had been so poor,” Vaidya says.

As word of the results have spread, it’s prompted calls and referrals from across the U.S. from other hospitals. According to Vaidya, it’s one step in a larger process of changing how we think about cardiac events in pregnant people and beyond. 

A Problem in the Heart

Kavitha Krishna knows how critical this innovation can be. After suffering from shortness of breath, she was diagnosed with pulmonary hypertension and told by her doctors that she wouldn’t be able to have children. But after a Google search for experts on the condition and subsequently getting another opinion from Forfia at Temple Health, she learned her condition could not only be treatable, but the possibility of children might even remain open. 

“I had been to many doctors and they had told me it was not possible at all,” Krishna says. “Dr. Forfia said he would treat me first, and then see.”

Forfia treated Krishna with pulmonary hypertension medical therapy, and ultimately recommended she have surgery to close a congenital hole in her heart. He continued to optimize her medical regimen and stabilized her condition–so successfully that he shifted her medication regimen to one that could support Krishna if she were to become pregnant. 

“I began to think it might be safe for me to carry out a pregnancy,” Krishna said. “I had full faith in him.”

The Temple team was able to have such success because of a deep understanding of the problem and how to address it. When someone has pulmonary hypertension, the focus can often be on the health of the lung (pulmonary means that it affects the lungs and respiratory system). 

But frequently, the actual cause of death in pulmonary hypertension is a resultant stress on the heart that leads to right-sided heart failure. The right side of the heart is structurally more vulnerable to the stress of pregnancy than the left side. The seismic changes that happen in the body during pregnancy also put a large amount of stress on the heart, and that’s what makes the addition of PAH so dangerous in pregnant individuals. 

Unfortunately, the resulting, potentially lethal cardiac events can be sudden and alarming, even for physicians, and often occur around the time of birth or soon after.

“It can be very clinically dramatic, acute, rapid, and challenging if it happens,” Vaidya says. “And that cardiac event is most predictable and preventable if you understand the impact of pregnancy on PAH and how to personalize a patient’s care to minimize their risk.” Trying to gauge the patient’s cardiac condition, however, can be difficult during pregnancy, as traditional methods depend on recognizing symptoms such as shortness of breath, decreased physical performance, and leg swelling, which already commonly occur during a healthy pregnancy.

Treatment options are also more limited during pregnancy.

“When we treat PAH, there’s a whole host of medications that we have available to use, but in pregnancy, a significant portion of them are not available because of the negative effects and risks for the fetus,” Vaidya says. 

The medications that cardiologists do have available in the context of pregnancy require a lot of skill to use–they may need to be infused continuously at carefully measured rates, and if not used precisely, can lead to significant side effects or adverse effects. A physician and patient may find themselves in a very high risk clinical outcome for the patient and baby. 

A New Kind of Expertise

But a fresh perspective can lead to a breakthrough. 

Forfia and Vaidya knew they were bringing a new perspective to the field nearly a decade ago. They had a unique background for physicians working in pulmonary hypertension, with experience as advanced heart failure and transplant cardiologists. 

“When you come from that approach, it’s very natural to be focused on cardiac performance,” Vaidya says. 

Forfia and Vaidya developed an approach to treating pulmonary hypertension that involved ongoing assessment and optimization of the performance of the right side of the heart, which is most prone to failure from pulmonary hypertension. That practice allowed them to take a proactive approach and administer complex yet individualized treatment before and during pregnancy, to mitigate the risk of a negative outcome.

Forfia founded the Pulmonary Hypertension, Right Heart Failure, and CTEPH program at Temple Health, and recruited Vaidya shortly thereafter to join the team. Their subsequent success in treating hypertension has contributed to a gradual evolution in the field at large. 

“There has been more recognition in recent years or so that attention to the right heart should be given more emphasis. That change has come in part from our program,” Vaidya says. 

Applying that approach to pregnancy, however, is more complex, as it requires implementing that approach within the complicated biological changes of pregnancy. That’s why Forfia and Vaidya approached the maternal fetal medicine (MFM) and obstetrical anesthesia physicians at Temple Health to form a multidisciplinary team that could give pregnant people with PAH their best chance at life and a successful pregnancy.

“Our approach to pulmonary hypertension care has to be done with an added attention to detail and skill because it is more complex to do during pregnancy,” Vaidya says. “And it has to be done in a multidisciplinary approach.” 

The Support Team

Forfia and Vaidya work throughout the pregnancy to optimize the function of the heart, right from the initial diagnosis or start of pregnancy, in close collaboration with expertise from the MFM team.

I think one of the great things about our group is that we discuss the patient’s case right at the start, review the prognosis of pregnancy as a team, before recommending a course of action,” Hart says. “For patients that were just diagnosed during the time of their pregnancy, that conversation about how to proceed is much more difficult for them than someone who’s been optimized before pregnancy. But our shared knowledge helps them understand  treatment options and their pregnancy, before there are any surprises.”

That collaboration continues right through to birth and beyond.

“One of us is always present at the delivery to assist in monitoring and mobilization of advanced cardiac resources if warranted, while MFM and OB anesthesia teams deliver care with swift expertise,” Vaidya says. After the baby is delivered and obstetrics work is completed, the patient is moved to the cardiac care unit for additional monitoring post-pregnancy, while maintaining close contact with the newborn infant. 

“With having our cardiology colleagues at the bedside throughout the labor and delivery process, we avoid excessive invasive testing,” Hart says. “When we weigh the risks and benefits of invasive cardiac monitoring during the labor and delivery process, the risks absolutely outweigh any benefit. Therefore, unlike other centers, we do not routinely use invasive cardiac monitoring.”

Temple’s approach enabled Krishna to have two healthy children. Pictured is her son with Dr. Forfia and Frances Rogers.

For patients like Krishna, the result is life-changing. She was able to have not only one child free of complications, but two.

“Having one child felt like a miracle, but after my first child was born, we were in an appointment and Dr. Forfia asked, ‘Are you planning for a second’?” Krishna says. “It felt like a dream come true.” 

The approach has been so successful that Forfia, Vaidya, Dr. Estefania Oliveros, Hart, MFM physician Dr. Wadia Mulla and obstetric anesthesiologist Dr. Dianna Feinstein published the results of their work to increase understanding of the importance of an individualized approach to management with an emphasis on right heart performance. They also wanted to publicize the fact that, if a patient’s life is in danger from pulmonary arterial hypertension in combination with pregnancy, they may have the ability to help. 

Their collaboration has also inspired the development of a large multidisciplinary program, Cardiac Obstetrics. As with their current approach to pulmonary arterial hypertension, the team has never lost a patient. Led by Oliveros in the Pulmonary Hypertension, Right Heart Failure, and CTEPH Program, it extends Temple Health’s capabilities to address a broader range of cardiac conditions that increase the risk of pregnancy. 

In addition to helping  more patients, Forfia and Vaidya are training more physicians with the expertise to manage these kinds of cases on their own, through Temple’s pulmonary hypertension fellowship training program founded and directed by Vaidya. As the field has gradually started to evolve through recognition of their team’s work, she hopes that the next generation of physicians and patients will see a transformation.

“Dr. Forfia and I have become known across the country for pulmonary hypertension treatment. But it isn’t good for sick patients if we’re some of the only physicians with that expertise,” Vaidya says. “So I will know we’ve been successful if, by the time Dr. Forfia and I retire, people are still able to look to the Pulmonary Hypertension, Right Heart Failure and CTEPH Program for help. Because there will be a new generation of physicians there that are able to treat this condition successfully.”