Bloodless Atrial Septal Defect Repair (ASD) in 8 Month-Old
From Noblood
| 
| Please feel free to participate in the development of this article by editing, discussing it or posting a comment on the Forum. |
Part I, The Case Report
At two days of age, an echocardiogram subsequently confirmed the presence of a large secundum-type atrial septal defect (ASD) and moderate size patent ductus arteriosus (PDA). She was begun on breast feedings, but did not nurse well. Reassessment at six weeks of age indicated resolution of the PDA, but some right ventricular volume overload. She did develop some bloody stools and colic at about four month of age. She was seen in consultation by pediatric gastroenterology. Nutrivene D supplements were also begun.
When the PDA closed, she began to nurse much better, but soon developed significant sensitivities to foods in the maternal diet. Surgeons at Mt. Sinai in New York advised somewhat urgent closure of the ASD but were not highly confident about totally bloodless management.
The family obtained a second opinion at Nationwide Children’s Hospital in Columbus, Ohio. After reviewing the echocardiograms, their cardiology team felt that surgery could safely be postponed until a later age, and their surgeon, Dr. Mark Galantowitz, was highly confident about a bloodless hospital course. Due to a family history of pulmonary hypertension and other considerations, surgical closure was scheduled for 11/19/08.
This was a busy time. Although routine vaccines had been deferred due to the colitis (which created some additional issues in the pediatric practice), she was started on Synagis (a passive vaccine for RSV).
She was also begun on erythropoietin (EPO). There were managed care approval challenges with both the Synagis and the EPO. There was some disagreement as to the optimal dosing. She ended up receiving 5,000 units (approx 600 /kg) weekly subcutaneously, starting one month pre-op. She also received supplemental vitamin C (100 mg per day) and iron (50 mg per day). The Nutrivene supplied the needed folic acid. The new black box warnings about thrombosis actually raised the concern of overshooting. A target pre-op hematocrit of 50 was set. Her baseline hct was 37.4. 10 days after the first weekly dose, it rose to 43.5. Twenty days after starting, it had risen to 47.3.
She was admitted to the hospital early in the morning on the day of surgery. She was put under general anesthesia, cooled to 34C, and placed on total cardiopulmonary bypass using a bloodless prime. The right atrium was opened and the septel defect was identified and stitched closed. She was placed in Trendelenberg, de-aired, and the aortic crossclamp was then removed. A mediastinal chest tube was placed. She was rewarmed, and weaned off bypass. Post-closure, the right ventricle appeared full, so direct pressure measurements were made, which did show some evidence of pulmonary hypertension. Milrinone was begun. Post-bypass ultrafiltration was performed, protamine given, the cannula removed, and sternum closed. Bypass time was 37 minutes, crossclamp time was 16 minutes.
Post-op, there was one brief episode of desaturation. Her hematocrit was 36. She was discharged on post-op day three on Lasix, Zantac, and Motrin. At follow up, she had some mild facial eczema, but had a notable increase in appetite, weight, and activity level.
Part II, The Interview of Parents David & Robin Haut
Parents: It really began before Kenzie was even born. Because Robin was considered a high-risk pregnancy, we were very careful to make sure Kenzie would be born in a facility that respected our wishes about non-blood medical management. We wanted to make sure that both Robin and Kenzie would be in an environment where blood transfusions were not an issue. We chose Englewood Hospital for Kenzie’s birth. Because Kenzie was born with Down syndrome, she had a thorough cardiology check when she was in the neonatal ICU (she developed respiratory distress after birth and was in intensive care for her first week of life).
At that time the cardiologists found an ASD (atrial septal defect) and a PDA (patent ductus arteriosus). They informed us that Kenzie would be monitored over the course of several months. Possibly, the two defects would close and no surgery would be needed. However, after the first check up at 4 months of age, it became clear that while the PDA had closed, the ASD was getting larger. It would be monitored for a few more months until finally when Kenzie was 9 months old, our cardiologist at Mt. Sinai recommended that Kenzie have the surgery soon, before winter.
We called the local HLC (Hospital Liaison Committee of Jehovah’s Witnesses) after Kenzie was born and well before surgery so we could gather names of surgeons in case Kenzie did indeed need the surgery. We also began asking different friends for referrals. We were impressed with the number of contacts we received of families who had gone through something similar with their babies and who would welcome hearing from us. We kept hearing very good things about the children’s hospital in Columbus, Ohio.
NoBlood: When exactly did you contact your local HLC?
Parents: Several times. First, right after learning of the pregnancy, due to the high-risk nature. Then again, after learning of the DS diagnosis. And yet a third time, when the recommendation for surgery was made.
NoBlood: Were they able to help?
Parents: Among other things, they gave us contact information for the HLC in Columbus, Ohio. You see, Dr. Mark Galantowitz at Nationwide Children’s Hospital in Columbus, Ohio, had come highly recommended by the Bloodless Center in Englewood, NJ, where Kenzie had been born.
NoBlood: You live near New York City, which has a number of bloodless programs, but you eventually chose a hospital hundreds of miles away. Why?
Parents: Our cardiologist was connected with Mt. Sinai. We felt that either Mt. Sinai or Columbia Presbyterian in New York City would have the best surgeons in the area for Kenzie, and we thought for that reason that they would be much less likely to use blood during her surgery. However, when we met with the pediatric heart surgeon at Mt. Sinai, he stated very clearly that he would absolutely use blood if he felt it was necessary, and he believed that the likelihood of using blood for Kenzie’s surgery was 50/50. We were very grateful that he was honest with us regarding his technique and the possibility of using blood.
We contacted the bloodless department at Englewood Hospital (where Kenzie was born, and all of her follow up appointments were held) and we were given the referral for Dr. Mark Galantowitz at Nationwide Children’s Hospital in Columbus, Ohio. After requesting that her records be sent to him, we had telephone consults with Dr. Galantowitz, who told us that in all the years he has done pediatric cardiac surgery he has never used blood during surgery on a heart defect such as the one Kenzie had. After that initial consultation and further research into the hospital and the type of surgery Kenzie would need, we decided to go to Columbus for her surgery.
NoBlood: Can you describe the weeks right before surgery? You must have had a lot going on.
Parents: An understatement! By midsummer, our cardiologist, Dr. Srivastava, felt that Kenzie's surgery had become more urgent and urged us to schedule it by October because she was at risk for complications with respiratory illnesses during the winter season. A telephone conference with our pediatrician at that appointment set things in motion to prepare Kenzie for the surgery; he would manage her care for Synagis (the vaccine to prevent RSV), EPO, iron, and monitor the results of her blood work. Referrals were made for a surgeon, and appointments to meet and discuss bloodless surgery were scheduled.
It did not go smoothly. We experienced delays with our insurance provider in obtaining both the Synagis and EPO. Once Kenzie began treatment with EPO and iron, she developed eczema. Kenzie also had an ongoing issue with colitis, which required appointments with a gastrointestinal specialist. Diet concerns and restrictions were required for her colitis caused by a whole laundry list of intolerances/allergies including dairy, wheat, soy, corn, pineapple, and a number of other foods.
We were concerned about the effect surgery would have on her already compromised immune system. We had also delayed Kenzie's schedule for vaccinations because of this problem. We felt that if she was so reactive to foods, how would she react to vaccinations? At the same time that we were preparing for surgery, individuals in the pediatric practice had isolated our case and were threatening to discharge Kenzie as a patient immediately if Kenzie did not receive a vaccination! Additional pediatric appointments were necessary to plead our case with the physician who was the practice manager.
In spite of the tremendous stress, there was no time for us to sit back and take a laid-back attitude. Our priorities were mapped out for us: getting Kenzie ready for surgery (getting her blood count to 50), finding a surgeon, maintaining her health (including doing our best to help her colitis heal), and keeping her pediatrician!
NoBlood: Explain a little more on the decision to proceed with surgery. Was there some urgency?
Parents: It was not too difficult for us to see the need for Kenzie’s surgery. Even though technically she would not have symptoms that were obvious to us, it seemed that she was short of breath at times, and a little weak. Another very serious concern for us was the likelihood of her developing pulmonary hypertension over time because of her heart defect. You see, pulmonary hypertension already runs in our family, so the possibility that our little baby could develop it was heartbreaking to us.
But our decision to go ahead with the surgery was challenged when Dr. Galantowitz, after consulting with his cardiology team in Columbus, felt that Kenzie could wait until next year for the surgery. If she were a little larger, she would be a candidate for the catheter device that closes the heart defect, allowing for the possibility of no surgery at all.
We were really unsure of which path to take. We prayed very fervently for wisdom to make a decision in Kenzie’s best interests and in accord with the dictates of our consciences. We asked Dr. Galantowitz to do the surgery this year, based on our observations of our baby and the very real concern to us of pulmonary hypertension developing in the meantime.
As it turned out, shortly after the procedure, Dr. Galantowitz informed us that he measured Kenzie’s lung pressure during surgery, and it was higher than would be expected. She indeed had a propensity for pulmonary hypertension. His comment to us was that the decision to have the surgery this year was sound. “Mother knew best,” was how he put it. We were grateful to him for his skill and his honesty.
NoBlood: How was the matter of consent for surgery & treatment handled at Nationwide?
Parents: In the most supportive manner, we were given every indication that no blood would be used, and we would be advised of the situation first, and that there was no reason to expect any cause to develop. Seeing the consent document was very assuring that we were on the same wave length with the staff on our request for bloodless care and surgery.
NoBlood: Had you heard of NoBlood.org, and is there anything else you would like to add?
Parents: No, we hadn’t heard of NoBlood, but our pediatrician apparently was aware. There is not enough that can be said for the support that we received on our trip to Ohio for surgery. The Hospital took care of rooming for us and our visiting families and friends! The local HLC was at the hospital every day to help us and in contact with us by phone throughout the day. The PVG (Patient Visitation Group) brothers stayed at the hospital with us and helped us during the surgery day around the clock almost.
The local congregation arranged for visits and we always had at least 10 visitors each day with us and our family. The waiting rooms were full! The congregation also offered to help us with rooming and food during our stay. We learned that in Columbus one to two heart surgeries are performed each week on Witnesses and the local brothers are really very busy and happy to support those coming.
NoBlood: Any final advice for another family that might face a comparable situation?
Parents: Always contact and work in cooperation with your Hospital Liaison Committee. If possible, connect with other parents who have had similar experiences and find out where the best treatment is currently being given.
NoBlood: And that might be where NoBlood could come in handy! Thanks to both you, David & Robin. Best wishes.
Moderator Note
This article is moderated by Joseph Malak, MD FAAP[1]. Did you enjoy reading it? Would you like to see more case reports like this posted? Was the format okay? Go ahead and click the discussion tab above and share your thoughts.
If you have questions or comments about the actual case such as how it was managed or the decisions made, the best place to post those thoughts would be in the thread[2] that was started in the NoBlood Forum.
