A Letter from Dylan's Surgeon

I'm not feeling to hot right now..... both physically and mentally. :(   I was back at the hospital yesterday in Triage due to a lot of cramping and some pressure....... I managed to skip all of the IV's and needles and all the other fun stuff and was only there for a few hours and then got to come home.  I went to bed early last night trying to get some sleep in because I haven't been sleeping well, even with the Ambien that my doctor gave me.... I got a few good hours of sleep in, but was awake at 2am.  I logged into Facebook on my phone just to see what I missed while I was sleeping and to check in for updates from a fellow Heart Mom whose little baby boy Travis had his 4th open heart surgery yesterday. :(   They live in the area and Travis is seen at Rady's Childrens, where Dylan is going to be, and both Travis and Dylan have the same surgeon.  I wish that I wasn't on bed rest, so that I could go visit and support you Nicole. I'm thinking about you and Travis every day and I'm always checking for updates <3

I wish that I didn't check my email........  inside my email was a letter from Dylan's surgeon Dr. Lamberti.  He had sent me a copy of the letter that he sent to my Perinatologist, Dr. Daneshmand, about the meeting he had with Joe and I two weeks ago.  He was just filling Dr. D in on what we discussed and his thoughts and expertise about what is going on and how to handle things.  I don't know why the letter effected me so much last night, It's not like there was anything in there, that Dr. Lamberti didn't tell us, or discuss with us, I guess it was just hard to actually see it all in writing because I've been struggling with all of the information since our meeting two weeks ago.  I'm having a hard time keeping strong.  I don't know if I can do it.  Everybody keeps telling me to try and not worry so much right now until Dylan is here, because sometimes things look worse than they are, and it's harder to get a clear picture and everything while Dylan is inside me, that they will know EXACTLY what we are dealing with once Dylan is here, and they can do a full work up and Echo on him being outside of me instead of trying to get a clear picture while inside.  I get it............ I really do....... I know that there is nothing I can do about it right now......... actually nothing I can really do once he's born either......and that we don't know whats going to happen, and things might look better once he's here....... but my heart still hurts, I still cry every day, I still wish I could take away the pain I know he's going to go through, I still wish I could protect my precious baby boy.  But I can't. I can't do anything, but sit here in bed and cry.  Maybe I just need more time to be sad, before I can go back to being strong. Maybe I just need more time to cry for my baby boy before I have to be strong and not show him my fears. Maybe I'll be able to be the mommy and the fighter that he needs me to be when the time comes.... just maybe I'll be able to do that.

Dylan has another Non Stress Test today at the hospital that I need to get ready for, so I'll leave you with the letter that we got from Dr. Lamberti.


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On February 9, 2011, I met with Krystal Clausen and Joe Perry to discuss their unborn child.  Krystal is 29 years old.  This is her first pregnancy.  She is currently on bed rest for contractions which began in the 24^th week of pregnancy.  She has been in and out of the hospital for the treatment of her premature contractions.  This male infant is due on March 31, 2011.  Fetal echocardiography has identified a rather severe form of hypoplastic left heart syndrome.  Mother is taking nifedipine for her preterm labor. 

I reviewed the fetal echocardiogram reports in detail.  I utilized a heart model and white board diagram to review the findings for Mother and Dad.  I described normal anatomy and then I described the special findings in hypoplastic left heart syndrome.  According to the most recent fetal echocardiogram, the patient has aortic valve atresia with severe hypoplasia of the mitral valve.  The ascending aorta is described as severely hypoplastic.  The atrial communication is mildly restrictive.  I discussed the implications of these findings within the context of our usual treatment for hypoplastic left heart syndrome. 

In general, there are four therapies which can be offered to a baby born with this diagnosis.  Cardiac transplantation has been suggested as a solution for this complex cardiac anomaly.  However, cardiac transplantation is  an unattractive therapeutic option simply because of the lack of donor hearts that are available to us at the present time.  In recent years, the Norwood procedure has become the standard of care for a baby born with hypoplastic left heart syndrome. The Norwood procedure was developed about 30 years ago at Boston Children’s Hospital.  In the Norwood operation, the right ventricle is utilized as the systemic ventricle.  The main pulmonary artery is connected to the aorta and the distal, narrow aorta is patched open.  In the early days, a shunt from the innominate artery to the pulmonary artery was utilized to provide pulmonary blood flow.  In recent years, we have favored the Sano/Norwood operation wherein pulmonary blood flow is provided by a conduit from the right ventricle to the pulmonary arteries.  In either approach, the Norwood component is referred to as a stage I procedure.  The Stage I operation is generally required during the first week or ten days of life.  A second operation is necessary at four to six months of age when we connect the superior vena cava to the pulmonary artery (bidirectional Glenn).  Finally, at about three years of age we connect the inferior vena cava to the pulmonary artery.  The third stage operation is known as the Fontan.  After the Fontan procedure, all blood flows in the normal pathways; however, there is no right ventricle or pumping chamber for the pulmonary circulation.

The operative risk for the stage I operation varies depending upon the anatomy and physiology of an individual baby.  In a term infant with good anatomy, the operative risk can be as low as 10%.  On the other hand, when there is an obstructed atrial septum and the baby is born prematurely, the risk is increased.  In addition, a very small aorta will increase the risk somewhat.  Baby Clausen appears to have an unfavorable anatomic configuration to his aorta.  We are hopeful that there will be some growth and development of the ascending aorta during the next month of pregnancy. 

In very high risk babies or in babies that are in poor condition soon after birth, the Hybrid approach has been utilized.  In the Hybrid approach, the pulmonary arteries are banded and the ductus arteriosus is stented or maintained open with prostaglandin.  The Hybrid approach can be used for a few days, a few weeks or as a replacement for the traditional stage I operation.  The Hybrid operation may be a short term solution in babies born with aortic atresia.  The presence of an obstructed atrial septum can result in poor respiratory function soon after delivery.  If baby Clausen’s atrial septal obstruction progresses during the next several weeks, we will need to be prepared for an emergency cardiac catheterization soon after birth.  On the other hand, if the atrial obstruction remains relatively mild, then the baby can be stabilized.   The fourth option for management of the baby is utilized when the prognosis is grave.  If a baby with unfavorable anatomy is born in very poor condition, supportive care can be rendered.  If the baby does not turn the corner, then surgical treatment will be futile. 

The parents have done some reading and research regarding the diagnosis.  I have counseled them that the internet is loaded with stories regarding babies born with hypoplastic left heart syndrome.  Some patients have a dramatically successful course, others are quite ill for a prolonged period of time following surgery.  Our goal in the management of hypoplastic left heart syndrome is to utilize an approach which provides the best chance for long term survival coupled with the optimal neurodevelopmental outcome.  Dr. Fripp is continuing to follow Krystal quite closely.  He will be performing another fetal echocardiogram in early March.  If the atrial septal defect is more obstructive at the next echocardiogram, then our approach to delivery must be adjusted since the baby is likely to be quite sick soon after he is born. 

Thank you very much for allowing me to participate in the counseling of this couple anticipating the birth of their first child together.  I am available to meet with them again should the need arise.  If you have any questions regarding my analysis, please do not hesitate to call upon me at any time.

Time spent in consultation: 30 minutes

Kindest personal regards.

Sincerely yours,

John J. Lamberti, M.D.

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