Cardiovascular Report
May 19, 2015

It could have been a case straight out of House, the TV drama known for depicting astoundingly complex if often far-fetched clinical scenarios.

In January 2014, 37-year-old Peggy Chung, four months pregnant and previously healthy, went to her local emergency room with persistent fever, cough and shortness of breath. Within hours, she developed severe hypotension and hypoxemia and was transferred to the hospital’s ICU with acute respiratory distress. Chung was then airlifted to The Johns Hopkins Hospital, where additional workup revealed flu infection and diffuse bleeding in her lungs.

Critical care specialists led by ARDS expert and intensive care unit director Roy Browerinitiated respiratory support and treatment with antivirals, but Chung’s fever was unrelenting, her pulmonary bleeding worsened and one of her lungs collapsed. Clinicians then initiated steroid treatment and ordered a CT scan.

What the scan showed was a true zebra case. Chung had multiple bilateral cysts on her lungs, a clue that led to further testing and the presumptive diagnosis of lymphangioleiomyomatosis (LAM), a rare lung disease with a predilection for women of childbearing age that’s characterized by the abnormal proliferation of smooth-muscle cells in the lungs or other organs. Yet, figuring out the diagnosis was only half the battle.

“This patient was in fulminant lung failure with severe airway bleeding, and conventional techniques were failing badly,” says cardiac surgeon Ashish Shah, who oversaw Chung’s care together with Brower and maternal fetal medicine fellow Arthur Jason Vaught. “We knew that extracorporeal life support — ECMO — was her only chance.”

Although Chung was stabilized on ECMO, her airway bleeding continued. The clinicians resorted to spraying the blood-clotting protein recombinant Factor VII directly into the patient’s airway, which stopped the bleeding.

But as if the case couldn’t get any more complicated, Chung’s blood pressure began to rise, steadily and relentlessly. Within days, she developed proteinuria, the hallmark of preeclampsia.

Waiting any longer could kill both mom and baby, so at just 24 weeks into her pregnancy and 11 days after going on ECMO, Chung was wheeled into the OR for a cesarean delivery. She gave birth to a 650-gram baby girl. A lung biopsy on Chung was done at the same time and confirmed the LAM diagnosis.

Following delivery, Chung’s condition improved rapidly. Within three days she was taken off ECMO, and two days later she was breathing on her own.

The newborn, however, faced her own set of challenges: a patent ductus arteriosus, which pediatric cardiac surgeon Luca Vricella closed a month after her birth; a bout with mild necrotizing enterocolitis, promptly nipped with antibiotics; and retinopathy of prematurity that regressed with photocoagulation therapy.

Believed to be only the second instance of a live delivery on ECMO, the case highlights a cascade of nearly catastrophic complications, but also how the orchestration of care among specialists from cardiac surgery, pulmonary critical care, obstetrics and neonatology helped both mom and baby overcome seemingly unbeatable odds.

Or as Shah puts it, “This case powerfully illustrates the intersection of high-tech medicine, Oslerian attention to detail and, above all, the good judgment of dedicated and smart clinicians.” 

To watch cardiac surgeon Ashish Shah and maternal fetal medicine fellow Arthur Jason Vaught talk about how they and colleagues managed this patient’s care, watch here.