Living a Full Life With Half of a Heart

Graphical abstract

S.dV.I. was born with tricuspid atresia, a cardiac defect characterized by incomplete development of the tricuspid valve and, consequently, of the right side of her heart. Accordingly, she underwent Fontan palliation, which ensured redirection of the systemic venous flow toward the lungs.

At the age of 23 years, she developed decompensated heart failure caused by Fontan conduit stenosis, which was treated with cardiac catheterization and stent implant (Figure 1). However, she developed multiple complications, including renal failure requiring intermittent renal replacement therapy, protein-losing enteropathy, iatrogenic diabetes, and infections. She had been hospitalized almost continuously for >1 year at the time of this interview, and her doctors did not have a plan to discharge her home (Figure 2).

Figure 1.

Echocardiographic/Angiographic Images Demonstrating the Patient Anatomy and Physiology

(A) Suprasternal echocardiographic view demonstrating aliasing (asterisks) at the distal end of the Fontan conduit. (B) Pulsed-wave Doppler interrogation confirming accelerated blood flow. (C) Angiogram demonstrating significant stenosis (asterisk) of the Fontan conduit. (D) Angiogram after stenting placement in the conduit. IVC = inferior vena cava; LPA = left pulmonary artery; RPA = right pulmonary artery; SVC = superior vena cava.

Figure 2.

Pictures of S.dV.I. in 3 Different Phases of Her Life, Reflecting Disease Progression

Interview

“My name is Ilaria. I am a 24-year-old student at the School of Journalism of Naples in southern Italy. I suffer from a congenital heart disease, and one could say that I have only a ‘half heart.’ I was diagnosed with my condition when I was 8 months old at a center specialized in congenital heart disease. By the age of 5 years, I had already undergone 3 open chest cardiac surgeries. I had to stay under strict follow-up during my entire childhood at the sole center in the southern Italy providing care for congenital heart disease patients.”

What was your life like before the first manifestation of your medical problem?

“As I was diagnosed with tricuspid atresia as a child, I can’t say what my life was like before the disease, but I can certainly describe my life after that. I had a normal childhood and adolescence. My parents and my doctors have always explained my conditions to me, and it felt normal to me. Of course, I had some limitations: I couldn’t exercise, and I had to avoid any strenuous activities. I also needed to remember to take my medications, but this has never been a problem to me. I used to have a normal life. I used to hang with my friends, I used to work and to travel. I used to do multiple jobs at the same time while also attending university. The job that I enjoyed the most was preparing the music playlists for a local radio. I was proud of my scar, and my heart has never kept me from reaching my goals.”

How was the course of the disease?

“Suddenly, in November 2020, I started to feel breathless and to suffer from palpitations, and my legs became swollen. I was urgently admitted for decompensated heart failure. My ankles and belly were so swollen and filled with liquid that I couldn’t even walk. I underwent a cardiac catheterization to dilate the Fontan conduit. However, soon after the procedure, I presented again with acute heart failure. From that moment, my condition has rapidly deteriorated, and I developed multiple complications, among them an internal bleeding requiring an urgent operation to remove the left ovary. After surgery, I remained in the intensive care unit for 20 days for acute renal failure requiring renal replacement therapy. Because of all those complications, my immune system became weak. In the last months I have had several dangerous infections, and I got COVID-19 pneumonia twice.”

Could you describe a critical episode?

“One of the most critical and traumatizing episodes in my story is the postoperative period in the intensive care unit. I woke up naked with a urinary catheter and diaper, and I had only a blanket to cover my body. I couldn’t move, and I was forced to stay in a bed. I was attached to several machines, I had many tubes in my body, and I felt dizzy because of the drugs I was given. I could breathe only with an oxygen mask. I couldn’t move my legs and arms and I was fed with intravenous nutrition. I spent 20 days alone, in this big, cold room with very bright lights 24 hours a day. Every morning, I was carefully washed by the nurses. This was the worst part, as I felt very embarrassed and humiliated. I was only 23, and it was for me inconceivable that I had to be washed by someone else. I did not feel like a woman anymore; rather, I felt I was a powerless object. The only way to escape that terrifying feeling and forget my condition was to sleep. When I was awake, I cried all the time wondering when that nightmare was going to end. I had to adjust to a new condition in which I need to rely on others for help in my everyday activities. I felt I had lost control over my body. I started suffering from anxiety and panic attacks, which I never had before: I felt my heart racing, and I felt like I was going to pass out. I also had muscular twitching that I couldn’t control. After 1 year from that intervention, I haven’t left the hospital yet, but those 20 days in the intensive care unit were shocking. I am still upset by bright lights, and I prefer darkness.”

How has your life changed?

“I can’t really say how deeply my life has changed, because I have been hospitalized ever since. It seems that any time I am doing better and I might finally have the opportunity of going back home, something happens that complicates my condition. I have lived the last year closed in a hospital room facing always new complications. It’s really hard, especially because my relatives were not allowed to visit me because of COVID-19 measures, and so I had to fight for my health all on my own. It has not been easy: after a while in my loneliness, I started to feel exhausted not only physically, but also from a psychological perspective. I sometimes found myself thinking of having reached the bottom and that it was time to give up. I thought, “Why me? Why has all of this happened to me?” and “Will it ever finish?”, but there are no answers to those questions, and the only thing I can do is fight with everything I have got, hoping to finally go home.”

How does the disease affect your life and family?

“This past year had a huge impact on me and my family: I haven’t seen my father, brother, and sister in 8 months. My mother instead comes and goes from the hospital to spend some time with me. She is the strong one in the family; she has never once given up; she is facing the disease with me without ever letting me know when she was worried about my health. As for my social life, in the last year with my prolonged hospitalization I lost connection with many people. My phone is my sole connection to the outside world. I keep in touch with my closer friends through social media, but I haven’t seen them in more than a year now. I was able to attend my best friend’s graduation celebration through videocall from the hospital.

“My disease has deeply influenced my possibility to work. Finding a job suitable for my disease is challenging. Last year I was going to graduate, but it was not possible because of my prolonged hospitalization. Until the age of 18, I used to receive a disability check and I did not have to pay for my medical treatments, which are extremely expensive. The aid I received allowed me to survive and pay for everything I needed. However, when I turned 18, the Italian public committee for the allocation of funds deemed me not needy of those aids and revoked my benefits. During the last years, I received absolutely no economic aid, and I had to pay for every single test I have had done outside the hospital and for all the expensive medications I need. When I get discharged from the hospital, I will have to pay a private nurse to administer my intravenous medications at home and for my physical therapy, which I need to start walking again.”

What else could be done to help people with your disease?

“I feel quite lucky to live close to a center specialized in the care of congenital heart disease. I am followed by doctors who are trained in this type of disease and know how to deal with my special needs. Unfortunately, to my knowledge, there are very few specialized centers in my country. Some patients may not be so lucky and not receive adequate treatment.

“Living with a congenital heart disease is not easy, especially because there is no adequate information on the disease. I used to know as little as possible about my condition before I got worse. I believe that promotion of public knowledge of those diseases may be helpful to prevent disease progression through implementation of routine visits. Public health policy should include economic aids to help families of patients with similar conditions.”

What are your hopes and wishes for your future?

“Hopes and wishes for the future? I can’t heal; it is simply impossible. I have a congenital heart disease, which means I was born and I certainly will die like that, and the missing part of my heart will never appear magically. I can only hope to get better, that my renal function will improve, my immune system will recover, and I can stop all the injections. I wish to get out of this hospital and go back home to stay with my family and friends. I dream of going back to university and graduating and becoming a journalist and finally doing the job of my dreams. I wish I could be again the girl that I used to be, the girl who loves traveling and having fun, who loves life so much that not even a second can be wasted.”

Because of multiorgan involvement, the patient was not deemed a candidate for heart transplantation.

A few months after this interview, S.dV.I. was finally discharged home for few days to be with her relatives and friends. She was admitted back to the ICU of our center with severe fluid overload and died few days later.

She will always be in the hearts of the doctors and nurses who treated her. We all learned a lot from her.

Funding Support and Author Disclosures

The authors have reported that they have no relationships relevant to the contents of this paper to disclose.