Nikita has reversed function of the ventricles; the result is the child does not get enough oxygen. Nikita requires two operations; a (classic) Blalock Taussig Shunt with an Atrial Septectomy to sever the septum so that blood can mix and have more oxygen. In about a year, after the child grows and thrives, he can have a more advanced repair. Dr. Raff’s explanation is clear, kind, and tactful, but straightforward. He tells the family something that I will hear him say often in the course of our stay. Namely, that heart surgery does not make the heart as it should be; operations only circumvent the defects and allow for proper, although not normal, function.

Sveta’s father asked how long recovery would take; six days in hospital. Sveta asks what causes this condition. Something happens in the course of fetal development, Dr. Raff explains; it is nothing the parents have done and they bear no responsibility for it. Nikita’s father must return to school, leaving Sveta alone at the hospital. Sveta couldn’t hold her baby for 3 days after his birth. At that time his murmur was noted and he went directly from a maternity hospital to children’s hospital where he was diagnosed with a defect. Now, like the other mothers, she will remain with her baby in hospital and will take care of him while he recovers.

The day of Nikita’s operation, someone poked their head in the door of our briefing room and shouted that he was in crisis! It was as if a vacuum cleaner had emptied the room of all the medical personnel including Doctors Raff and Pretzlaff and all the Samaran doctors and nurses. They were gone about a half hour: Dr. Pretzlaff decided to stay with Nikita all night. Jan Campo, RN stayed with him so that if Nikita needed nursing support, Dr. Pretzlaff would not need translation. Both returned to the hotel after midnight.

July 7, 2004 Nikita has to be re-intubated today. He is having a hard time breathing. Yesterday I noted that Sveta was hanging out with other moms and babies waiting for exams or operation. She holds babies, relieves mothers. She is quite tender, but still very restrained and without much affect. A little community seems to be forming on 3rd floor where the waiting families are “housed.” By the time we left, July 12, Sveta was coming to the ICU to feed Nikita. He was looking quite pearly and well, and when I commented she smiled.
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Kristina has Downs Syndrome. She neither sits nor walks. She can sit if someone props her up, but she tends to fall forward. Mother Natasha describes her as calm. Dr. Tacy calls her “lethargic.” Natasha says she babbles, but I didn’t hear it. She says it was a shock to have a Down’s Syndrome baby, but she says it with very little affect. In fact, she manifests very little affect in general and appears very depressed. Kristina’s heart problem was diagnosed very early, because Down Syndrome babies are prone to heart disease. Natasha was advised to go to Moscow, but the wait for treatment is very long. Samara is a state hospital, which means that Kristina will be treated virtually free of cost.

After Kristina is out of ICU, I find Natasha’s mother and father in the room. The grandma is holding Kristina and rocking her frantically. At some point mom and grandma put Kristina in her father’s arms. He looks even younger than Natasha, and seems terrified to hold baby Kristina. He is very awkward, holding her with her legs dangling down, as the two women try to get him to support her head properly.
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Anna is an orphan. Her caretaker, Faina, brought her here from the orphanage – a two hour ride by bus. The orphanage houses 80 sick children and only has six caregivers. The facility cannot afford to employ cleaners and maintenance workers, so the caregivers have to do all the cleaning. Faina claims that there is medical personnel at the orphanage but "If not for this hospital (Samara), Anna would perish."

Anna’s parents brought her to the Orphanage directly from the hospital where she was born, but they have never since come to see her. Anna is very tiny. To my eyes she looks like a child half her age, and indeed she weighs only 8.2 kg. She does not walk or speak, but Faina says that she sits, babbles, crawls, and sleeps well. I observed none of that behavior. She was either in Faina’s arms or sitting on the bed during the interview and she was totally unresponsive. She is the “blue-est” baby I have every seen. (due to poor blood flow) It broke my heart to see Baby Anna put her little hand around Faina’s neck, clinging to her, enveloped by her.

Anna was originally diagnosed with a Taussig Bing anomaly. However, Dr. Tacy did an echocardiogram which demonstrated that this is not the case. Rather, she has a double-outlet right ventricle with normally related great vessels. On July 7th, Anna underwent surgical repair of her DORV, via transannular patch repair. Postoperatively, Anna experienced severe diastolic dysfunction, affecting her left ventricle more than her right ventricle. She died 12 hours after the operation from elevation of her filling pressures and an inability to maintain adequate cardiac output. Both Dr. Raff and Dr. Pretzlaff were present when she died.
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Katya, Leonid and baby Karina live in a factory dormitory, which is a two-room complex; they and another family each have one room and share bath and kitchen. Mother Katya is very upbeat: “I haven’t had time to be scared.” She is less apprehensive and more genuinely optimistic about the outcome of the forthcoming operation than many of the other young mothers. Especially since she learned that American doctors would be presiding, which she only learned when she got to the hospital.

Katya says a prenatal ultrasound revealed that baby Karina had a heart defect. Karina constantly breathes fast, and has to take frequent breaks when breastfeeding. She sleeps through the night. She has only mild cyanosis and very modest growth. She has a double inlet LV, L-TGA . Now she will have a shunt and DKS. If her oxygen saturations remain above 70%, she should have a heart catheterization in April (in Samara), followed by Glenn operation in May of 2005.

Following the DKS and Aortopulmonary Shunt surgical procedures, Karina had to return to the OR because she was bleeding profusely from the incision. Dr. Raff had difficulty speculating where/why she was bleeding in such a way. When they opened her up again, they found that she was bleeding from the holes in the shunt suture, which was made of Russian materials. Doctors Raff, Pretzlaff and ICU RN Jan Campo remained until 1:30 a.m. to care for her. Baby Karina was saved because Heart to Heart OR nurse Gail Keyser donated her own blood in the OR. The hospital has a history of problems with the storage and delivery of blood. After this situation, the issue was thoroughly discussed with the Samara surgical staff and will be remedied. I saw the baby’s mother Katya in the corridor after Karina had been re-operated. She was sitting alone, withdrawn and crying. Baby Karina was fine by the time we departed July 12.
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Although Maksim’s parents had difficulty conceiving, an ultra-sound done in-utero revealed nothing irregular. But immediately after his birth, tests revealed Maksim was born with rectal atresia and a serious heart defect, transposition of the great arteries. The hospital in Neftgorod sent him to Samara Children’s Hospital. Irina is very happy that he will be treated by American doctors. But she wishes she had more technical understanding of baby’s condition.

Maksim’s father Anton asked for time off, but “not all of his superiors are very understanding.” He can’t risk offending his bosses because jobs are very hard to get even in the oil town of Neftgorod. Jobs are more available in Samara, but affordable housing is a big problem. Irina is the only mother I interviewed to ask me why I am asking her questions. At 25 she is also the oldest of the mothers of infants. The childbearing age for women is much lower in Russia than in the USA. She is more self-confident and more forthcoming than many of the other mothers--and more independent. Which helps when her husband must return to work. When we spoke about how she would be enduring the surgery ordeal alone for the most critical period, she waved her hand and said that she preferred it that way-- she says she prefers to worry on her own.

Maksim, whose operation I watched, was given a pulmonary band. This is palliative. It will allow him to “grow and thrive” and he can have a more complicated operation in a year or so, a full arterial switch. When to palliate versus when to attempt a full correction is a great teaching experience for Heart to Heart and the Russian medical staff.
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