Restoring the Heart’s Natural Flow: Kristof’s Journey to Health

Kristof’s journey began long before his first breath. He is from Slovenia. At 20 weeks of pregnancy, his parents learned that their baby had a serious heart condition. During the routine scan, several heart defects were identified, including congenitally corrected transposition of the great arteries (ccTGA), coarctation of the aorta, a perimembranous ventricular septal defect (VSD), and a secundum atrial septal defect (ASD).

His parents, Tadeja and Dejan, explained that all of these diagnoses were confirmed again at birth.

Congenitally corrected transposition of the great arteries is a rare condition in which the heart’s lower chambers and the major arteries leaving the heart are reversed. Although blood continues to flow in the correct direction, the heart is not structured to function efficiently over time. In Kristof’s case, the condition was further complicated by a narrowing of the aorta, the main artery carrying blood from the heart to the body, as well as two holes in the walls dividing the heart chambers.

On the third day after birth, Kristof underwent his first operation to repair the coarctation of the aorta. Six weeks later, a pulmonary artery band was placed to help regulate blood flow to the lungs until he was strong enough for further surgery.

Fortunately, the diagnosis was made during pregnancy, so treatment could be started before any obvious symptoms appeared. In the first weeks after birth, his biggest problem was feeding. Fortunately, this also soon resolved.

Kristof remained stable for the next few years with the help of medication and careful monitoring. 

We took great care of Kristof and avoided crowds of people, so as not to expose him to infections. On the recommendation of the doctors, he did not attend kindergarten, so he was at home with his mother the whole time.

Kristof was three years old when he travelled to Great Ormond Street Hospital (GOSH) in London for surgery. Due to the rarity and complexity of his heart condition, cardiologists at the Paediatric Clinic of Ljubljana referred the family to GOSH for specialist treatment.

At GOSH, Kristof’s care was led by consultant cardiothoracic surgeon Mr Martin Kostolny. Kristof underwent a double switch operation, a highly complex procedure designed to restore normal blood flow through the heart. As part of the surgery, the hole between the heart’s pumping chambers was closed with a patch.

Mr Kostolny explained, “In double switch the upper chambers of the heart are cut open in a very specific way and resutured back together in order to divert blood from the systemic veins into the structurally right pumping chamber and the blood from the lung veins into the left pumping chamber. This part of the operation is called the Senning operation.”

The operation also involved an arterial switch, in which the two main arteries leaving the heart are detached and reconnected in their correct positions. Mr Kostolny added, “The next step is the arterial switch operation where the two main vessels coming out of the heart are divided and then switched. In addition the two arteries feeding the heart muscle are moved to their new position on the opposite vessel.”

A double switch operation restores the normal connections between the heart’s pumping chambers and the circulation to the lungs and body. It is technically one of the most demanding procedures in congenital heart surgery, which is why experience and outcome data from large patient groups remain limited. However, all available evidence suggests this approach offers the best long-term outcome for patients like Kristof, who present with congenitally corrected transposition of the great arteries.

Mr Martin Kostolny, Consultant Cardiothoracic Surgeon 

Kristof’s family felt reassured during their time at GOSH. “We all felt very comfortable and relaxed at GOSH. All the medical staff are very friendly and professional. GOSH remains in our fond memories.”

One of their clearest memories was the gentle way Kristof was taken into surgery. “We have a fond memory of how Kristof was prepared for anaesthesia. The nurse blew soap bubbles for him and while he was having fun, the anaesthetist set up his channel and arranged everything necessary. Everything happened completely without fear and stress. While playing with the bubbles, he fell asleep and was ready for the operation.”

The hardest part was the waiting during the surgery.

Kristof's Family

Mr Kostolny described the recovery process: “He was transferred to cardiac intensive care after surgery and was weaned from mechanical ventilation on the first postoperative day. His postoperative course was uncomplicated. He was discharged on the 9th postoperative day.”

Kristof’s parents were closely involved throughout. “We were able to be present for all the check-ups and also after he stayed in the hospital, so we could support him the whole time. Before the surgery, we explained to him in a nice way what to expect. He accepted it well and cooperated well during the recovery.”

Mr Kostolny reflected on Kristof’s progress: “I was impressed by the speed of Kristof’s recovery, especially given how complex the operation was “He had an excellent recovery from surgery, and his parents report very good activity levels since returning to Slovenia.”

Looking ahead, the plan is for Kristof to remain under observation. Mr Kostolny said, “He will continue follow-up with his local cardiology team. It is expected that he will not require any intervention for at least 5–10 years. Difficult to prognosticate long term for this type of heart lesion no matter how successful surgery is.”

Today, Kristof is thriving. “Kristof feels very well and is already very lively. With the surgery that was performed at GOSH, we can look to his future with great hope. This surgery allows him to live a normal life.”

Tadeja and Dejan shared their heartfelt thanks: “We can recommend GOSH to all parents. All the staff are very friendly and professional, the hospital has very nice playrooms and beautiful rooms with a pleasant atmosphere, spiritual care is provided and all visitors are made to feel comfortable.”

Behind every complex operation is not just a skilled team, but a determined family. Kristof’s story is a reminder of what can be achieved when care begins early, expertise is shared across borders, and children are given the chance to thrive.

Kristoff with family back home in Slovenia