CCAS Meeting Reviews

CCAS 2019 Annual Meeting Review:
Morning Sessions I and II

By Annelisa Cossu, MD
Riley Children’s Hospital

The 2019 CCAS Annual Meeting took place in downtown Houston, TX at the Marriott Marquis on March 14, 2019.  Program Chair Kirsten C. Odegard, MD (Harvard University/Boston Children’s Hospital, Boston) and Co-chairs James P. Spaeth, MD (Cincinnati Children’s Hospital, Cincinnati) and Susan C. Nicolson, MD (Children’s Hospital of Philadelphia) prepared a well thought-out and educationally meaningful meeting spanning a wide variety of topics in congenital heart disease.

The meeting featured the Arthur Keats Lecture, which was supported by the Dolly D. Hansen, MD Educational Foundation and was given by James A. DiNardo, MD (Boston Children’s Hospital).  The recently added round table discussions (RTDs) were back by popular demand at this year’s meeting in addition to the early morning Problem Based Learning Discussion (PBLD) sessions.

Despite Boeing 737 MAX groundings, a bomb cyclone in Colorado and prolonged travel plans, members started the early day with lively PBLDs and RTDs.  This year’s PBLD topics included three case-based discussions on:

• Dental rehabilitation in the patient with Trisomy 18 and Eisenmenger Syndrome by Faith Ross, MD and Michael Richards, MD (Seattle Children’s Hospital, Seattle)
• Emergent non-cardiac surgery in the patient with a Ventricular Assist Device (VAD) by Pablo Motta, MD and Erica P. Lin, MD (Cincinnati Children’s Hospital Medical Center, Cincinnati)
• Emergency surgery in the failing Fontan patient with ventricular dysfunction by Stephanie N. Grant, MD (Children’s Healthcare of Atlanta, Atlanta) and Annette Y. Schure, MD (Boston Children’s Hospital, Boston)

The three round table discussions included the following controversial topics:

• Neuroprotective strategies in pediatric cardiac surgery by Dean B. Andropoulos, MD (Texas Children’s Hospital, Houston) and Ken Brady, MD (Ann and Robert H. Lurie Children’s Hospital of Chicago, Chicago)
• A marathon of diagnostic studies and procedures under one anesthetic and the risks versus the benefits by Lisa Montenegro MD (Children’s Hospital of Philadelphia, Philadelphia) and Karen Bender, MD (Nemours Children’s Hospital, Orlando)
• How to deal with a bad outcome by Emad B. Mossad, MD and Ashraf Resheidat, MD (Texas Children’s Hospital, Houston)

Outgoing CCAS President Nina Guzzetta, MD welcomed all members to the 2019 CCAS Annual Meeting. She thanked all of the meeting sponsors in particular Silver Exhibitor Casmed and Bronze Exhibitor Berlin Heart, Inc and Staq Pharma.  Dr. Guzzetta thanked outgoing members of the Board of Directors Jeremy Geiduschek, MD and Emad Mossad, MD, who is the Immediate Past-President.  Finally, Dr. Guzzetta shared her appreciation, privilege, and warm sentiments as the outgoing CCAS President and graciously welcomed Wanda Miller-Hance, MD as the incoming CCAS President.

Wanda Miller-Hance, MD also welcomed members to the 2019 CCAS Annual Meeting and announced the new members of the Board of Directors, Kelly Chilson, MD (St. Louis Children’s Hospital, St. Louis) and Katherine Taylor, MD (The Hospital for Sick Children, Toronto).  Newly elected officers include Luis M. Zabala, MD (Children’s Medical Center, Dallas) as the Secretary/Treasurer and Mark Twite, MD as the Vice President (Children’s Hospital of Colorado, Aurora).  This year’s Program Chair, Kirsten Odegard, MD, then provided an outline of the educational program for the meeting.

Session I.  The Quest for Quality and Excellence

Dr. Odegard introduced the first session of the day that focused on the quest for quality and excellence in pediatric cardiac centers.

Mr. Doron Weber, author of Immortal Bird: A Family Memoir, shared the story of his son Damon who was born with Hypoplastic Left Heart Syndrome and palliated to a Fontan at the age of four.  Mr. Weber read excerpts from the memoir that told of how Damon was diagnosed with protein-losing enteropathy (PLE) and the difficulty of accepting the diagnosis as a parent for a variety of reasons. 

Mr. Weber explained his disbelief because Damon, with his bright red hair, was full of vitality for eight years after his Fontan operation and also because medical providers never mentioned PLE as a complication of the Fontan circulation.  We learned from the excerpts that Damon had a Fontan revision which temporarily improved his symptoms of PLE, but that he was eventually listed for a heart transplantation. Mr. Weber shared his frustration and bewilderment at not receiving enough information about heart transplantation.  Furthermore, we learned that Damon’s transplant status is changed from Status 2 to Status 1B due to Mr. Weber’s question of Damon’s age as a factor. Consequently, Damon had been listed with the incorrect status for several weeks. 

The most poignant moment shared by Mr. Weber illustrated the deep bond and love between him and his son when Damon at age 16 deposited himself in his father’s lap while sitting on the front porch of their home in a touching overture of regression.  He embraced his son tightly and they held hands - the deepest conversation between the two without words.  Through this touching address, Mr. Weber’s overwhelming and heartfelt message to the group was to prioritize patient centered care over your personal and professional goals.

The next lecture in the session was the Arthur Keats Lecture given by James A. DiNardo, MD. Arthur Keats, MD was the Chief of Cardiovascular Anesthesiology at the Texas Heart Institute, St. Luke’s Episcopal Hospital, and Texas Children’s Hospital from 1974 to 2005.  Dr. Keats made contributions related to the development of anesthetic techniques for neonates with congenital cardiac disease and the emerging field of cardiopulmonary support for open heart surgery in the 1950s.  During his early career in 1955, he was appointed Professor and Chairman of the Department of Anesthesiology at Baylor College of Medicine.  He remained Chair of the department for 19 years.

In the course of his career, he published at least 130 papers related to the field of anesthesiology. He also accumulated many honors and awards including the Texas Heart Institute Medal and was named a Distinguished Physician of this institution. (Tex Heart Inst J. 2007; 34(4): 402–403). This lecture was given to recognize the contribution of the speaker to the field of pediatric cardiac anesthesiology in honor of Dr. Keats’ career.

Dr. DiNardo discussed building a heart center of excellence. He began with the sentiments that there must be a shared philosophy or ethos across multiple disciplines in the institution, dedication to stellar patient care, careful consideration of innovation, commitment to mentoring trainees, and engendering mutual respect and trust among colleagues. 

Intrinsic to the development of a center of excellence is a dedicated executive committee working together toward a common goal.  The executive committee should be comprised of the chief of cardiac surgery, chief of cardiology, chief of cardiac anesthesiology, and the vice president of nursing for cardiovascular surgery.  The establishment of a separate and financially independent division of pediatric cardiac anesthesia with an adequate number of anesthesiologists who are supported with appropriate time and resources to advance their careers is of great importance, as well. 

At Boston Children’s Hospital, the cardiac anesthesiologists have a separate call schedule from the main operating room anesthesiologists and an independent academic schedule consisting of journal club, morbidity and mortality conference and a lecture series.  Dr. DiNardo shared the structure of the division of Pediatric Cardiac Anesthesiology at Boston Children’s Hospital in terms of anesthetizing locations and staffing ratios.  There is a dedicated preoperative clinic for patients undergoing cardiac surgery that is financially supported by cardiac surgery and cardiology.  The preoperative clinic is staffed by nurse practitioners who are trained by anesthesiologists to perform the pre-anesthetic evaluation.

Dr. DiNardo touched upon the specific IT innovations now used regularly at Boston Children’s Hospital, including two computerized programs called Heart Center Scene and PACE.  Heart Center Scene allows users to view the OR/cardiac catheterization lab schedule and a summary of each patient’s cardiac history, echocardiogram data, and cardiac catheterization data. PACE is another program that tracks the progress of pre-anesthetic evaluations and consent for each patient scheduled for surgeries and procedures in the cardiac catheterization laboratory, electrophysiology laboratory, and imaging.  PACE also contains cardiac anesthesia consults for congenital cardiac patients presenting for non-cardiac surgery.

Dr. DiNardo ended his lecture by discussing the importance of mentoring faculty both academically and administratively. He stressed the importance of facilitating the development of productive and durable connections between individuals that lead to independence and credibility. He also emphasized the significance of equitable distribution of clinical exposure, delegation of division responsibilities, and embracing conflict as an opportunity to solve problems and to allow junior faculty to mature.  He also warned against efforts to standardize care in a system involving many individual practitioners. Dr. DiNardo’s final thoughts revolved around his role as chief and warned that a division chief should never be more in love with having the job than doing the job. He also stated that a division chief should never be the only person who can solve a problem as that is a sign of failed mentorship.

Questions for Session I:

For Mr. Weber: What can we do better as anesthesiologists?

Put yourself in the parents’ shoes. Say to yourself, “What if I were you?”
Mr. Weber went on to say that his son had awareness during a surgery and remembers hearing things.  He asked how anesthesiologists dose medications.

Dr. DiNardo answered that many anesthetic medications can cause hemodynamic instability, requiring their doses to be scaled back.  He also mentioned monitoring devices for awareness. Dr. Odegard referenced MAC versus GA and that potentially Damon could remember things from MAC.  She also mentioned that giving increased doses of some anesthetics can increase the risk of cardiac arrest.

Mr. Weber also asked if there are any new treatments for protein losing enteropathy (PLE).  Dr. Odegard mentioned that she is not sure if there are new treatments for PLE but that many complications arise from the Fontan operation.  She added that a biventricular repair is now attempted more frequently at Boston Children’s Hospital. Dr. DiNardo also mentioned research at CHOP studying abnormalities in lymphatic drainage and potential benefits of altering the pattern of drainage.

For Dr. DiNardo: How do we balance teaching with efficiency in an environment with reduced manpower? 

Dr. DiNardo answered that allowing fellows to learn independently is not useful in this day and age and that mentoring is imperative. He suggested reducing case numbers as a solution and touched upon the importance of work-life balance.

Session II. Innovation in Congenital Cardiac Anesthesia

James P. Spaeth, MD (Cincinnati Children’s Hospital, Cincinnati) moderated the session on innovation in congenital cardiac anesthesia. 

David M. Axelrod, MD (Stanford University School of Medicine, Stanford) started the session with a presentation on the use of virtual reality to create a virtual three-dimensional model of the heart called the Stanford Virtual Heart.  The technology is supported by The Betty Irene Moore Children’s Heart Center at Stanford and Oculus VR and was created by Lighthaus, Inc.  Dr. Axelrod explained that Stanford Virtual Heart is being used to educate students, patients and parents.  In the clinical setting, a virtual reality headset allows parents to have an immersive, interactive experience while using the Stanford Virtual Heart to facilitate an understanding of their child’s congenital heart defect and the surgery used to repair the defect.  Research has shown that physician assistant students at Stanford had improved test scores in a subject they had already been taught after using virtual reality for further education.  Furthermore, the physician assistant students desired to use virtual reality for learning. Preliminary studies are now underway to model blood flow patterns in the vessels originating from or surrounding the heart.

James D. McCully, PhD (Boston Children’s Hospital, Boston) followed with an innovative presentation on mitochondrial rescue with autologous mitochondrial transplantation. Dr. McCully began by explaining ischemia reperfusion injury secondary to cardiopulmonary bypass.  He explained that myocardial ischemia reperfusion injury significantly damages mitochondrial structure and function which then severely compromises functional recovery and cellular viability.

Most pharmacologic agents intended to target mediators of ischemia reperfusion injury have had limited efficacy.  Dr. McCully has pioneered the novel therapy of mitochondrial transplantation.  The therapy uses viable, functional mitochondria isolated from autologous skeletal muscle to reverse the damaging effects of ischemia reperfusion injury.  The efficacy of mitochondrial transfer has already been validated in a series of in vitro and in vivo heart models using ischemia reperfusion protocols.  The transplanted mitochondria immediately increase ATP levels and upregulate proteomic pathways for energy production and cell respiration. 

There is also reduced cardiomyocyte apoptosis.  The mitochondria have been shown to maintain viability for up to 28 days and have no arrhythmogenic, immune or autoimmune response.  The mitochondria can be isolated in a matter of 20-30 minutes and transplanted immediately.  The mitochondria are delivered directly by injection with a tuberculin syringe or by vascular infusion. 

This technique has been used in pediatric patients with ischemia reperfusion injury who required extracorporeal membrane oxygenation (ECMO) support for more than 72 hours. Recovery of myocardial function was seen within 24-28 hours after mitochondrial transplantation. Mitochondrial transplantation has also been shown to prolong cold ischemia time in a murine heart transplantation model. Further studies are currently underway in kidney and lung transplant models.

Questions for Session II:

To Dr. Axelrod: How readily available is this technology for fellows and families?

There are a number of set ups in the hospital and the virtual reality modules are very portable.

To Dr. McCully: What is the ideal timing for mitochondrial injection?

It is better to do it early on or immediately after surgery because this can lead to 100% recovery.  If the surgery is predicted to be very long and complicated, this can be done immediately after surgery in the operating room. Mitochondrial transplantation can also be done prophylactically.

To Dr. McCully: Would you do this for patients with mitochondrial myopathy?

Yes I would, but there are no animal models of mitochondrial myopathy to prove efficacy. Mitochondrial transplantation has been done in cells lacking mitochondria.  Twenty-four hours after transplantation, ATP production was increased and mitochondrial DNA was detected in cells.