Every year, 100,000 babies don't make it to their first birthday because of CHD.
Charlotte Christine Marra | Heart Warrior of the Week

Name: Charlotte Christine Marra

Defect: Hypoplastic Left Heart Syndrome (HLHS)

Birthday: September 18th, 2017

Described in a few words: Strong-willed, smart, funny

Charlotte’s story, as told by her Dad, Marc:

Charlotte was one of the lucky Heart Babies that was diagnosed in-utero, and pretty early on in the pregnancy too. Shortly after her birth we were brutally welcomed into the CHD life with surgeries, long hospital stays, and all the uncertainties and fears that come with that.

So far, our Charlotte has had two open-heart surgeries (the Glenn and Norwood), and multiple angioplasty procedures. She still has one more major open-heart surgery to go, the Fontan, which will complete the series of three surgeries that are commonly used to treat her particular heart defect. This will take place sometime in the next year or two, but as is the case with so many Heart Babies, it’s not a guarantee that it will be her last.

We spent the first 226 days of Charlotte’s life in the Cardiac Intensive Care Unit (CICU) of Nicklaus Children’s Hospital. Likely as a result of the dozens of medications she had to receive orally upon birth, she developed a severe oral aversion which resulted in her needing a feeding tube surgically implanted in her abdomen. While she is now doing much better feeding orally, her primary source of sustenance is received via feeding tube for the time being.  But despite her rough start to life, she lives a full life that is pretty restriction-free, which is miraculous considering all she has endured.

Everything about Charlotte inspires me. We are so blessed to have the team at Nicklaus Children’s Hospital involved in our journey. Without them, Charlotte would not be with us today.

Hypoplastic Left Heart Syndrome

Anatomy: Most of the structures of the left side of the heart are underdeveloped or missing altogether.  This includes the left atria, left ventricle, aorta and mitral valve. Basically, they are born with half a heart.

Complications: Blood is diverted to the right atrium through an atrial septal defect (hole in the heart). Because the left-sided structures of the heart are essentially missing, the right ventricle must collect both oxygen-rich blood from the lungs and oxygen-poor blood from the body. It is also responsible for pumping blood to both the lungs and the body. Since deoxygenated and oxygenated blood are mixing and being pumped to the body, HLHS babies are cyanotic (blue in color) and have low oxygen levels.

Symptoms: Rapid breathing or shortness of breath, rapid heartbeat or pounding heart, poor sucking and feeding abilities, cold extremities, blue color of the skin, lips and nailbeds (cyanosis), overall body weakness.

Treatment: HLHS is treated with a series of three open-heart surgeries called the Norwood, Glenn and Fontan procedures.  HLHS is also sometimes treated with a heart transplant.

Prevalence: HLHS accounts for approximately 2-3% of all congenital heart defects. It occurs in 2-3 out of 10,000 live births worldwide.

HLHS with Intact Atrial Septum

With partial or complete closure of the atrial septum, flow is diverted away from the left atrium and left ventricle. This has little effect while the child is still in utero, but at birth, elevation of blood pressure in the left atrium due to the intact atrial septum causes a shift in the distribution of blood flow away from the blood vessels of the lungs, resulting in dangerously low oxygen levels in the blood.

January 03, 2020 by Lani O'Gorman

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