COVID-19 complicates vascular problems in woman with Alagille

Case suggests such infections may increase risk of blood clots in patients

Marisa Wexler, MS avatar

by Marisa Wexler, MS |

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A 34-year-old woman with Alagille syndrome and vascular problems — who had been implanted with a stent for her narrowed pulmonary arteries — experienced a blood clot that blocked her arteries after contracting COVID-19, a case study reports.

A stent is a small, expandable metal mesh tube that can be inserted in narrowed or blocked blood vessels to keep them open. As with this patient, they can be used in the pulmonary arteries, which carry blood from the heart to the lungs.

This case, according to the researchers, suggests that COVID-19 may increase the risk of blood clotting complications in Alagille patients with heart defects and stents. Further, per the team, preventive anti-clotting treatment may be warranted for such patients.

“Based on our experience treating this patient, we suggest that COVID-19 infection places patients with [stents in blood vessels] and known pulmonary vessel malformations at high risk for stent [blood clots] and secondary pulmonary hypertension,” or high blood pressure in the lung arteries, the researchers wrote.

Such blood clots, known as stent thromboses, are “a devastating phenomenon and should be treated urgently and aggressively,” the team wrote.

The case study, “Pulmonary artery stent thrombosis and symptomatic pulmonary hypertension following COVID-19 infection in Alagille patient: A case report,” was published in the World Journal of Clinical Cases.

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Woman had experienced vascular problems since childhood

Alagille syndrome is a genetic disorder that can cause abnormalities in the development of many different organs throughout the body, including the liver, heart, and kidneys. Some patients experience problems with the pulmonary arteries, and in certain cases, a stent may be surgically placed to help normalize blood flow.

This report from Israel detailed the case of a woman with Alagille and a history of heart problems who developed vascular complications after a COVID-19 infection.

As a child, she had been found to have several heart defects, including a narrowing of her left pulmonary artery. She underwent several surgeries to repair these defects, one of which was the implantation of a stent in her left pulmonary artery.

The woman had also shown consistently high levels of liver enzymes, a sign of liver damage, and chronic kidney disease.

Prior to getting COVID-19, the pulmonary artery issues were well-controlled; the woman didn’t have any breathing problems, worked full-time as a kindergarten teacher, and danced in her free time.

Her case of COVID-19 was quite mild, per the report, not causing any substantial breathing problems and not requiring medical treatment. But about a month after her COVID-19 diagnosis, she sought treatment at a hospital emergency department for acute shortness of breath and chest pain.

A series of imaging tests showed no lung abnormalities, but revealed a blood clot in the stent in her left pulmonary artery — a condition known as stent thrombosis. She was given anticoagulants, which prevent clotting, and was eventually able to be discharged from the hospital while continuing on these medications.

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Patient developed hypertension; treatment failed to resolve symptoms

After four months, however, the woman was still experiencing shortness of breath and needed supplemental oxygen when sleeping or exerting herself.

Further testing later revealed that the woman had developed pulmonary hypertension (PH), a disease marked by high blood pressure in the pulmonary arteries. She was initially given the PH treatments Revatio (sildenafil) and Opsumit (macitentan), but her breathing problems got worse and these were discontinued.

The woman was then given a course of steroids aiming to reduce lung inflammation, but this had no effect on her symptoms. Surgeries aiming to reopen the stent were also unsuccessful.

As of the latest follow-up, the woman’s condition is stable, but she still needs supplemental oxygen, and the researchers reported that she is “far from her [previous] level of functioning.”

We suggest these patients be considered for preventive … anticoagulation therapy. Once a [blocked] stent is diagnosed, prompt and aggressive treatment should be pursued to prevent an adverse disease course.

Because COVID-19 is associated with an increased risk of thrombosis, or blockage of blood vessels due to a blood clot, the researchers hypothesized that COVID-19 set the stage for a clot to form in the woman’s stent.

Based on this case, they suggested that special care may be warranted for people with stents who contract COVID-19, even if the initial infection doesn’t seem serious.

“We suggest these patients be considered for preventive … anticoagulation therapy. Once a [blocked] stent is diagnosed, prompt and aggressive treatment should be pursued to prevent an adverse disease course,” the team concluded.