An (excessively) large Heart
Our journey with our son's heart disease
When our son was six weeks old, he was diagnosed with heart disease. Pulmonary arterial banding surgery was our hope to avoid a transplant.
We were overjoyed when we held our second child perfectly healthy in our arms after a miscarriage. Now we could start our life as a family of four. Nobody expected what awaited us 6 weeks later.
He was born naturally on February 2nd, 2021. He was a happy baby and as a second mom I felt pretty confident dealing with newborns. After about a month, however, he began to cry more and more frequently and could not be calmed down. At some point I noticed the quickened breathing and remembered a reel that I had seen on Instagram a few weeks earlier. A mother drew attention to the warning signs and symptoms of heart disease in babies. I made an appointment with our pediatrician in the hope of reassurance and the all-clear. Instead, I got a referral to the emergency room that said: “Tachypnea respiratory rate 110/min, follow-up examination requested.”
A few routine examinations followed until more and more doctors were called in to auscultate (listen to) the heart. Worried faces and an unbearable silence in the room. Finally a statement: “I hear a prominent third heartbeat.” I had no idea what that meant. We were sent to the outpatient clinic for an ultrasound. There, a young intern doctor sang his heart out but said nothing about the black and white images. “One moment, please.” He got up and came back with a senior doctor. He looked and looked and looked.
At this point I would like to say that we were in the middle of the pandemic and everyone was wearing a mask. So I couldn't see the doctors' full facial expressions. But when this senior doctor looked at me, my blood froze in my veins. His look told me what I didn't want to hear before he said it. “Your son has a serious heart condition.” My ears began to ring, my eyes became rigid and I could only hear his voice as if from far away. "We're taking you up the floor to the ICU." I tried to get up and carry my baby there, but I was put in a wheelchair with my son in my arms and so we were pushed into the PICU.
Between PICU and IMC
Once in the Pediatric Intensive Care Unit, he was taken from me and within minutes he had more tubes, cables and sensors on his little body than I could count. In tears, I dialed my husband's number and then all the dams burst. The nursing manager pushed me into the anteroom and said they would come get me as soon as he had all the necessary lines. After half an hour a nurse came and took me to a room on the next ward. A little later I was allowed to see him. The nurse in charge gave it to me in my arms, I didn't even know where to touch it, there were cables everywhere. I was allowed to breastfeed him and then I had to leave again because the Pediatric Intensive Care Unit has visiting rules. But I didn't yet know how strict they were and how much trouble I got into with the staff because of it.
The next few days passed like a trance. During the day I sat next to his bed during visiting hours and at night I paced up and down the room, constantly expecting a call from a nurse. The doctors worked at full speed and carried out numerous examinations. Even at this time, the diagnosis of “dilated cardiomyopathy” was often made. I couldn't even pronounce it or remember it at first. My brain was in survival mode.
When he was somewhat stable, they moved us to the IMC (Intermediate Care Unit). The IMC is the link between the intensive care ward with its comprehensive therapeutic and intensive care options and the normal ward, where close monitoring of the patient is not possible due to the lower staffing levels. Here I was allowed to sleep on a fold-out bed next to him. After a few days the decision was made to have a cardiac catheter examination. This is a minimally invasive medical examination of the heart using a catheter that is inserted through venous or arterial veins in the groin. In Paulo's case, the coronary arteries were targeted.
Troubles seldom come alone
Since the procedure took place under anesthesia, he was then returned to the intensive care unit. When I was finally allowed to see him, I was almost shocked. He was bloated and white as a sheet, had a high flow of oxygen under his nose, and an orderly was giving him a bottle. Before I could say anything, a sensitive doctor took me aside and calmly explained to me that they now had to take stock of the food gain and that I would therefore no longer be able to breastfeed for a while. From now on it was time to pump and Paulo only received 70 ml every 4 hours. This was calculated - that was the recommended amount for his size and weight.
After the cardiac catheter, the word transplant kept coming up. In general, there would be no reliable prognosis, the disease would be rare and every child would react differently. In addition to dilated cardiomyopathy, he also had a left bundle branch block and thus a diagnosis like only a handful of other children in the world.
We were transferred back to the IMC, where the next disaster soon began. The plan was to prepare for a transfer to Vienna, where one of the two children's heart centers in Austria is located. Pulmonary artery banding should be done there. One day before departure, he suddenly got a fever and then everything happened very quickly. His condition worsened by the hour and he was taken back to the intensive care unit. There, sepsis was diagnosed, caused by a germ that had penetrated into the right atrium of the heart via the CVC (central venous access) in his neck. The coming hours were one of the worst of our lives. He repeatedly had attacks where he would become so upset at the slightest noise or touch that he would become unconscious and stop breathing. Due to his poor condition, he was unable to keep food down and the risk of necrotizing enterocolitis increased.
„A heart that is too big also means more love.“
Luckily he recovered within a few days and we were able to be transferred to Vienna. There we were taken straight to the intensive care unit. My little baby was now two months old and lying in a small room full of beeping monitors, flashing medication syringes and countless tubes. I wasn't allowed to take him out of the bed. At least here you were allowed to stay with him except for the handovers and rounds. But we still got a room in the teddy house so that my husband and I could take turns and I could also spend some time with my daughter.
After a few days we were transferred to the normal ward, unfortunately the planned operation was not to take place - he did not meet all the criteria. We were supposed to live here for almost two months. During this time, he was stabilized and slowly transitioned to oral medication.
After 51 days, we were finally allowed to go home, packed with numerous medications, some scars and many memoriers. At that time, our little baby was receiving multiple oral injections three times a day. These include beta blockers, ACE inhibitors and diuretics, as well as sodium chloride and electrolytes. We enjoyed this time at home and in what felt like complete freedom.
When a miracle happened.
We settled into life at home, attended the weekly check-ups in the clinic and learned every day from life with a chronically seriously ill child. I read through all kinds of forums, joined Facebook groups and tried to network with like-minded people. Then one day this wonderful mother from Croatia wrote to me. At first I wasn't sure what she wanted from me - her manner was very direct and she only ever talked about this PAB (pulmonary artery banding). We had already completely ignored this topic and I couldn't even get involved in it.
At some point she put me in touch with a professor in Germany with the recommendation to go to him for a second opinion. He would be the best in the field and would know who would be considered for banding. If you don't venture, you don't win - so I wrote the professor mentioned an email and a few days later we got a completely unexpected answer: “I read your story! (…) So, if you want, come to Frankfurt. Maybe I can arrange for you to stay here to plan the operation!”
And we did. From now on our little son was in the protective hands of this professor. There are not many doctors in this world whose dedication to their profession is so palpable.
The open heart surgery was carried out in Giessen in September 2021. After just a few weeks we saw the first positive changes and after a year the cardiac output had increased from 30% to 53%. Our little fighter mastered everyday life with 4 medications (Bisoprolol, Lisinopril, Spironolactone and ThromboASS).
The further Course
The first partial debanding had to be done in July 2023 because the right ventricle was under too much pressure and therefore hypertrophied. After this procedure, he felt very well again and in January 2024 he surprised us with an ejection performance of 65% after an MRI check! Normally a child's ejection fraction is between 60-70%, so it is completely within the normal range. We are incredibly grateful for the opportunities we were able to seize, for the special people we met along the way and for our extraordinarily strong and courageous boy. A transplant is no longer on the cards at the moment, but rather behind the house in the neighboring garden. He may need a pacemaker when he's a teenager, but I'm sure he will continue to show us how strong he is.
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