It was amazing that Dr. Horn not only suspected I had PVOD, but also paid such close attention to how my body responded to the various medications and made choices that resulted in prolonging my life.
Chloé: Breathing Easy Again
Sometimes the person who saves your life is the one who made your original diagnosis, even if it was seven years before the double-lung transplant that restored your future. That was the case for Chloé Temtchine, 38, whose diagnosis at NewYork-Presbyterian/Weill Cornell Medical Center in 2013 led to the treatment that stabilized her illness and put her on the path to health.
As a professional singer, being able to breathe easily was essential for Chloé’s well-being and professional success. At age 25, however, during a recording session in New York City, she felt a sharp pain in her right shoulder that radiated to her lungs. She began experiencing an array of symptoms that stumped physicians at many elite medical centers: shortness of breath, chest pain, dry cough, body aches, racing heartbeat, fatigue, and water retention. Their diagnoses over the next five years were frustrating: pulmonary embolism, autoimmune disease, even a psychiatric disorder.
She took a turn for the worse in March 2013 and ended up in the emergency room at New York-Presbyterian/Weill Cornell. "I had trouble breathing and could barely walk five steps," she recalled.
Doctors were concerned about the pressure in her pulmonary arteries, which carry blood from the heart to the lungs. Her pulmonary artery systolic pressure escalated to 180 (it is normally under 35). She had pulmonary arterial hypertension (PAH) — a serious and chronic condition where the pulmonary arteries become muscularized, thicker, abnormal, and narrow — and the heart has to work harder to pump the blood through. Over time, the heart weakens and cannot do its job, and heart failure develops.
That’s exactly what happened to Chloé, who had gained an additional 10 pounds due to the excess fluid that collected in her body due to heart failure. Watching Chloé’s condition become increasingly dire despite the treatments they gave her, heart failure expert Evelyn Horn, MD, Director of Heart Failure and Pulmonary Hypertension at the Perkin Heart Failure Center at NewYork-Presbyterian/Weill Cornell, knew there might be more to the story.
It turns out she was right, and her experience likely saved Chloé’s life. Some people with PAH have pulmonary veno-occlusive disease (PVOD), a buildup of abnormal fibrous tissue in the small veins in the lungs, narrowing the vessels and impairing blood flow. It may sound like a subtle distinction from PAH, but it is an important one: the drugs normally used to treat PAH could, if used at the same doses in someone with PVOD, be fatal.
With that knowledge in hand, doctors gave Chloé lower doses of PAH medications that began to improve her symptoms. "It was amazing that Dr. Horn not only suspected I had PVOD, but also paid such close attention to how my body responded to the various medications and made choices that resulted in prolonging my life." (The PVOD diagnosis was confirmed seven years later when her lungs were biopsied after a double-lung transplant.)
Chloé remained in the hospital for five days on oxygen therapy, learning she would need it for the rest of her life and that her best bet was eventually a lung transplant. "At first, I was concerned about being able to sing while on oxygen therapy," she noted, "but then when I understood that my life was at stake, those concerns quickly became less important." With additional treatment and the right medications, Chloé was able to go home, where she began following a healthy diet and using portable oxygen 24/7. Aware of the risk of organ rejection from a lung transplant, she wanted to hold onto her own lungs for as long as possible.
Over the years, she began to get better and was able to continue her performing career. "Dr. Horn was so pleased that my health was improving," Chloé noted. "And I started to get excited about life again!" She was back to writing songs and released three albums. Her pulmonary arterial systolic pressure gradually declined to 32 and she moved from New York City to Los Angeles to continue pursuing her career.
In July 2020, she tackled a new hurdle as her heart rate shot up to 175 while working out on a treadmill. She was retaining fluid again and the jugular veins in her neck made her feel as though she was being strangled 24/7. Chloé went to a Los Angeles hospital emergency room, was given medication, and while there, had a heart attack. Her doctors put her into a medically induced coma. She was then transferred to another hospital on ECMO (extracorporeal membrane oxygenation, a way to artificially oxygenate the blood), remaining on it for 21 days while she waited for a lung transplant. A matched set of donor lungs became available and she was able to have a double-lung transplant on August 5, 2020, giving her a new lease on life.
Along the way, Chloé and her parents stayed in touch with Dr. Horn, consulting her for advice as they made treatment decisions. Chloé is still regaining her strength and has started singing again, writing music, and performing virtually. Through projects called Super Brave Kids and The Smile Tour, she enjoys interviewing and performing for pediatric patients with PAH and other chronic illnesses who are in hospitals across the United States, as well as for their families, friends, and hospital staff. "My goals are to instill a sense of hope in those who may have lost it, to generate public awareness of PAH — with the hope that there may one day be a cure — and to bring awareness to the importance of organ donation," she noted.
She is grateful that her journey to wellness began at NewYork-Presbyterian/Weill Cornell. "If I hadn’t met Dr. Horn in 2013, I might very well not be here today," Chloé continued. "I can’t tell you how grateful I am to be alive and how incredible it is to feel well again after not feeling well for so long."