In the first intrauterine surgery of its kind, researchers successfully repaired a cerebrovascular malformation. Cerebrovascular malformations can often lead to heart failure, severe brain damage, or death shortly after birth.
A team from Boston Children’s Hospital and Brigham and Women’s Hospital used ultrasound guidance to repair a Galen’s malformed vein, which causes excessively high blood flow and neurological and cardiac complications.
The operation was performed on a fetus at 34 weeks of gestation with amazing results. The baby girl, who was confirmed in utero to be at high risk of suffering serious complications of the malformation since birth, did not require any medications or postnatal surgery to treat heart failure.
Postnatal repeat echocardiography showed marked improvement in cardiac output, and brain MRI showed no brain damage and a normal neurological examination.
“This is incredibly exciting. The hope is that this baby and others with this condition who will undergo intrauterine surgery in the future will lead normal lives,” said principal investigator Darren B. Orbach. medical doctor commented theheart.org| Medscape Cardiology.
“We were thrilled to find that there was none of the rapid decline that usually occurs after birth. At six weeks, the infant was drug-free, eating normally and gaining weight. I am happy to report that I am doing very well until I get home, and there are no signs of adverse effects on my brain,” he added.
Orbach, co-director of the Center for Cerebrovascular Surgery and Intervention at Boston Children’s Hospital, and colleagues describe this first case report of an intrauterine vein in Galen malformation repair in a research letter published online today in the journal. increase. stroke.
Galen malformation vein
Galen’s vein malformation, which occurs in about 1 in every 60,000 live births, Orbach said, is caused by the arterial system going directly to the venous system rather than the capillaries needed to slow blood flow and oxygenate the environment. I explained that it was a connected cerebrovascular anomaly. brain tissue.
“The arterial and venous systems are fundamentally very different. The arterial system has high pressure and high flow, whereas the venous system has low pressure and low flow. They should not be directly connected,” he pointed out.
The galenic vein is the most extreme version of such an anomaly. It develops early in pregnancy, is associated with a large increase in blood flow through the brain, and can grow over time to more than double the body’s total cardiac output.
The placenta is thought to be protective because most babies do not have overt physiological problems in the womb, but abnormally high blood flow can cause great stress on the heart, which can lead to crisis after birth. There is a possibility
Babies usually present with heart failure as the first major symptom immediately after birth, says Orbach. You’re trying to pump blood into the malformation and then quickly back to the heart, but that blood isn’t doing anything, a useful feature.
“These newborns can be very sick. They need multiple medications to support their cardiovascular system, and they need to have procedures done to reduce blood flow,” he explained. .
Brain injury is also a common problem. “Cerebral circulation is highly abnormal. Blood is shunted through the malformation rather than circulating through potentially ischemic brain tissue,” commented Orbach.
“Sick babies have a very high mortality rate (up to 90%) without specialist care. %, there is a high mortality rate among surviving babies, who are at risk of neurological and cognitive impairment,” he added.
Current treatment for babies born with this condition includes transarterial embolization. This allows catheters to be inserted into the arterial system to occlude the malformation using a variety of techniques.
But Orbach points out that some babies are born sick enough to receive postnatal intervention. “Heart failure and brain damage are very serious and cannot be reversed by anything and usually these babies do not survive. It’s trying to help a baby who can’t. It’s the current postpartum approach,” he said.
The first phase of this study attempted to identify these high-risk babies in utero. On her fetal MRI, the researchers found that her one specific measurement of the sinus that drains the main malformation was a good predictor of how the baby would deliver. Pay the fare after birth. Babies predicted to do poorly on this test are candidates for fetal surgery.
Techniques used for postnatal interventions are technically too difficult to perform in utero. “We therefore developed an alternative approach for intrauterine surgery in which a needle is guided under ultrasound guidance into a receiving vein within the malformation and the vein is filled with a metal coil to dramatically reduce blood flow. did,” he explained Orbach.
This surgery was performed on this first patient on March 15 and is reported in the current article. The surgery was part of a planned clinical trial involving a total of 20 patients.
“The immediate goal is to see if we can transform a fetus, who is at very high risk of postnatal illness, into a baby who can do well in the NICU and then go home for elective care in the first few months of life. Orbach pointed out, “It’s important to continue to show efficacy and safety in other patients, so the study continues,” he added.
Orbach said the results for this first case are very encouraging. “Each step was exciting — the technical success of the procedure and the [blood] The flow is reduced with ultrasound during the procedure. A fetal echocardiogram the next day showed a dramatic decrease in abnormal cardiac output, and an MRI scan of her fetus also showed the malformation to have already shrunk. ”
The baby was born prematurely 2 days after the procedure due to membrane rupture with a birth weight of 1.9 kg (4.2 lb). She did not require cardiovascular support or her postnatal embolization.
“Until the baby was born, we waited with bated breath to see how she would turn out clinically. Now at home and on oxygen therapy for the first few weeks, “Right now her neurological condition is completely intact and she basically looks like any other baby.” ‘ commented Orbach.
It is not yet known if infants need additional procedures. “We will follow her closely and determine if further treatment is needed based on whether the deformity is growing,” Orbach said. Occasional secondary problems, such as seizures, are also evaluated.
Although other fetal surgeries are now routinely performed, this is believed to be the first intrauterine surgery targeting the cerebral vasculature.
“There were a lot of uncertainties,” says Orbach. “We didn’t even know if our instruments could be seen by ultrasound.” rice field. This was key to gaining his FDA approval of the study.
If the study shows success in other patients, the technology could be expanded to other centers. We definitely need a capable fetal surgery and neurointervention team, but we are not the only center with these capabilities, yes other teams in specialized children’s hospitals around the world can do this too. I hope we can,” he added.
Commenting on this initial case report in an American Heart Association press release, Colin Derdeyn, MD, neurointerventional radiologist at the University of Iowa College of Health Care, who performs venous embolization for Galen’s malformation in neonates, said: said like this. Physiological events of childbirth can lead to life-threatening heart failure. ”
Derdeyn, former chairman of the American Heart Association’s Stroke Council, warned that one successful case isn’t enough experience to conclude that the risks of this surgery are worth the benefits.
However, he said, “The positive changes in hemodynamics they observed in utero and postnatally—decreased flow, decreased size of draining veins, reversal of abnormal reflux in the aorta—are really encouraging.” It’s the most exciting and surprising aspect of the case report,” he said. “This is pioneering work being done in a very careful and responsible way.”
This study was funded by a grant from the Sage Schermerhorn Chair for Image-Guided Therapy.
strokePublished online May 4, 2023.full text
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