Piccolo: Heart umbrellas with a big impact

"After the procedure, Jonah was a different child, the improvement was immediate. He turned 180 degrees in the right direction," says his mother Ania. On June 22 of this year, the boy underwent an effective procedure in the cardiac catheterization laboratory at the Heart and Diabetes Center NRW (HDZ NRW) in Bad Oeynhausen when he was just six months old.

Prof. Stephan Schubert, Director of Pediatric Cardiology there, closed Jonah's open ductus arteriosus with a small nitinol umbrella, a "piccolo" umbrella.

The ductus arteriosus is a small vessel that connects the aorta with the pulmonary artery in the unborn baby. In the womb, blood is pumped from the right ventricle into the aorta via the ductus, bypassing the lungs. After birth, when the lungs develop and the newborn begins to breathe, the duct closes spontaneously within hours to days after birth. If it fails to do so, it is referred to as a persistent ductus arteriosus botalli (PDA).

Jonah and his twin brother were also affected by an open ductus when they were born prematurely in December 2022. The patent ductus is one of the most common congenital heart defects in premature babies and accounts for between five and nine percent of all congenital heart defects.

While the open ductus did not cause any health problems for Jonah's brother, the opposite was true for Jonah. "Jonah was in the hospital for 130 days after his birth because, on the one hand, he was extremely premature and, on the other, he suffered from a rare, serious underlying condition. We were allowed to go home at the end of April, but after about six weeks, I noticed that his condition was gradually deteriorating.

He was cold-sweaty, had a high respiratory rate and was unstable despite oxygen supply - Jonah is tracheostomized. His breathing seemed labored, and his heart rate was too high. Fortunately, I am a doctor myself, an oral surgeon and not a neonatologist, but I can recognize when someone seems abnormal. I called the clinic and described the symptoms and was told to call '112' immediately," says his mother.

Jonah was admitted to the intensive care unit at Dortmund Hospital. His mother was certain that there was more to it than the elevated inflammation markers found there. Together, they decided that Jonah should also undergo another pediatric cardiology check-up. Among other things, it turned out that his ductus had now become hemodynamically relevant and his left heart was significantly enlarged. He was immediately given medication - diuretics and beta blockers to prevent the other organs from being affected - and the pediatric cardiologist promptly contacted the Heart and Diabetes Center NRW (HDZ NRW) in Bad Oeynhausen.

"We were admitted there on June 21, and the procedure took place a day later. The team in Bad Oeynhausen was perfectly organized. We felt very well informed by Prof. Schubert; he even took us to the playground so that our youngest could play there while he recorded the procedure on a piece of paper," Jonah's mother recalls.

"Jonah and his family had already had a difficult journey when they came to us due to his premature birth - he was born at 1300 grams - and his serious underlying illness. His lung pressure was high, Jonah was not thriving, and he had signs of heart failure. The ductus had to be closed quickly," says Prof. Schubert.

"Even though ductus closure itself is a straightforward technique, as it is a frequent intervention, we have to work very precisely. How do you get the device into the desired position? Which device do you choose so as not to provoke difficulties elsewhere? The smaller the child and the larger the duct, the more difficult the procedure will be. In Jonah's case, who, unlike most of our smaller patients, already weighed five kilograms at the time of the procedure, the challenge was the complexity of his underlying disease," explains Prof. Schubert.

Using a minimally invasive approach and mainly under ultrasound and minimal X-ray control, Prof. Schubert guided the narrow catheter through a groin vein to the small children's heart, where the nitinol sheath unfolds in the ductus and closes the open connection.

This cardiac catheterization procedure, which is less invasive than major heart surgery, has only been possible for children weighing 700 grams or more since 2019. It saves young patients from having to open their chest and, according to Prof. Schubert's estimates, is performed by eight to ten heart centers in Germany. Previously, the weight limit was six kilograms, which meant that the procedure would not have been an option for Jonah at the time.

"For Jonah's PDA closure with the Amplatzer Duct Occluder (ADO) II AS 5/6 mm - "Piccolo", Abbott company - we chose the largest "Piccolo" umbrella, as the duct was very large. However, the procedure went smoothly," says Schubert. He and his team have already successfully performed more than 40 procedures of this type on children weighing less than 3000 grams in recent years.

"The plug heals, the symptoms disappear. If the child was very stressed by the duct, like Jonah, you can see the improvement very quickly. A switch flips immediately," explains Schubert.

Jonah's mother happily confirms this: "Before the operation, Jonah was just a sick child who looked miserable and breathed badly. He made his first attempts to swallow after the operation, although the doctors had previously told us that he would never be able to swallow. The operation is the best thing that could have happened to us. What this clinic has achieved..."

Jonah had spent half his life in the hospital up until then. "But now we've arrived home reasonably well. Everyday life remains a challenge due to Jonah's underlying illness, simply because of the intensive medical care required. But we've become braver, we dare to go out with Jonah and accept invitations again," says his mother.

Fortunately, the topic of the heart no longer plays a major role in Jonah's life thanks to the placement of the "Piccolo" umbrella. "Jonah no longer needs any medication; we just have to go for regular check-ups," reports his mother.