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about the efforts and treatments required to keep his 11-year-old son healthy.
The heart illustrations below were provided by Dr. Shunji Sano
who performed the Cone Procedure and other the repairs at UCSF’s Benioff Children’s Hospital.

Michael Tomlin, an IT professional for almost three decades, has been a Manager and Agile practitioner at TrinityTG since 2015.  He and his wife, Ella, have three children, including their 11-year-old middle son, Will. But Will suffers from Epstein’s Anomaly, a congenital heart defect of the tricuspid valve.

The tricuspid valve is located between the right atrium and the right ventricle. In a normal valve, blood flows through the valve, then the valve closes completely to ensure the deoxygenated blood flows to the lungs for cleaning. When this valve doesn’t close properly, not all the blood gets replenished by the lungs and the right atrium tends to enlarge over time.

A surgical treatment known as the “Cone Procedure” has been completed twice to repair Will’s tricuspid valve. First at the Mayo Clinic in February, 2013 by Dr. Dearani. The second six years later by Dr. Sano at the UCSF Benioff Children’s Hospital.

Technically, doctors talk in terms of the amount of regurgitation occurring in the tricuspid valve. During pre-op Will’s heart was characterized as having moderate to severe regurgitation.

This pre-op illustration indicates there were holes and gaps in the tricuspid valve still present after original repair back in 2013.

After the most recent eight to ten-hour surgery, which included three hours on a bypass machine, Will’s tricuspid valve now has only a trace amount of regurgitation. The holes and gaps in Will’s heart are now closed.

An additional complication of the regurgitation issue is that the heart tends to enlarge over time. The enlarged and damaged area has been described as a stretched-out balloon that is no longer able to squeeze efficiently.

This damaged area (indicated by the horizontal hash marks) was folded over and sutured to effectively reduce the overall size of Will’s heart.  This offers the additional benefit of providing extra space in the chest cavity for the lungs and other organs.

To reduce the workload on the heart during the original 2013 surgery at the Mayo Clinic, Will also had a Bi-Directional Glenn Procedure. In this treatment, the superior vena cava is re-routed to flow to the lungs, thereby skipping the heart.

In Will’s most recent 2019 surgery, a band was added to reduce the overall blood flow resulting in a reduction of the backflow pressures by roughly half. Flow pressures are now within normal ranges.

Backflow pressure resulted in his enduring a puffy face and neck as well as a tendency to have headaches. If this banding proves to be a problem, the band can be “popped-off” using a relatively easy catheterization procedure.

Long term benefits of the Cone Procedure compared to other options include a more anatomically correct repair that does not include any artificial elements or tissues that could be rejected by the body.

Our immediate goal was to increase Will’s exercise capacity and overall quality of life. In November 2018, during an exercise capacity test, he was rated at 43% compared to other children with congenital heart defects.

This second open-heart surgery by all accounts was considered very successful. He was released in early March and returned to school just a few weeks after coming home.

Over the course of his career, Dr. Shunji Sano has performed more than 7,000 pediatric cardiac surgeries. We are incredibly thankful to have such skilled experts in our area to aid in our son’s recovery!

Some links for additional reading:

https://www.ucsfbenioffchildrens.org/shunji.sano

http://www.chp.edu/our-services/heart/cardiothoracic-surgery/da-silva-center/cone-procedure

http://www.severinbrenny.com/bidirectional_glenn_operation.html