Dancing in the Rain
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A Trisomy 18 Journey

Life is not about waiting for the storm to pass. It's about learning to dance in the rain. -- Vivian Greene

Thursday, February 9, 2012

Julia's Heart

February 7-14 is officially designated as CHD Awareness Week.  CHD, or congenital heart defects, are the most common birth defects.  Heart defects are commonly associated with the three most common trisomies (Trisomy 21,18, and 13) but often occur without a related syndrome. When I was pregnant with Julia, a heart defect was found at our 21 week ultrasound and was the first indicator that something was wrong.  Later ultrasounds and echocardiograms revealed several more abnormalities.  Julia's particular heart defect, or series of heart defects, was rare and would be considered life-threatening even without a chromosomal disorder. I thought I would try to explain her various heart issues since it is CHD Awareness Week.

Definitions* of Julia's Various Heart Defects: 
VSD: A Ventricular Septal Defect (VSD) is a hole in the wall separating the two lower chambers of the heart. If the hole is small, open heart surgery is not always required. The hole may close on its own or may be patched through catheterization. If the hole is large, open heart surgery is required and is usually performed in the first year of life. This is the most common congenital heart defect. 

ASD: An Atrial Septal Defect (ASD) is a hole in the wall separating the two upper chambers of the heart.

Coarctation of the aorta: A narrowing of the major artery (the aorta) that carries blood to the body.

PDAA Patent Ductus Arteriosis is an unclosed hole in the aorta. This hole usually closes soon after birth.  Julia's remained open throughout her life and was a blessing in that it lessened the effects of the coarctation.

Overriding aorta: The major artery (aorta) is located usually located over the left ventricle to carry fully oxygenated blood to the body. With an overriding aorta, the aorta is located partially over the right ventricle instead of over the left ventricle. This means the blood leaving the heart will come partially from both chambers and not be fully oxygenated. In Julia's case, this helped compensate for her hypoplastic left ventricle. 

Hypoplastic left ventricle: An underdeveloped left lower chamber of the heart. This was Julia's most severe defect.  Because of her large VSD and PDA and overriding aorta, Julia's right ventricle managed to do the work of both ventricles. The surgeries required to address this defect are complex and difficult even in a child with normal chromosomes. The single ventricle repair surgeries have not been performed in children with trisomy 18.
*From www.americanheart.org 

With all of this going on plus the unknown effects of trisomy 18 on Julia's respiratory system at birth, it is easy to see why her docs thought she would live only a few days.  Somehow, though, her heart managed to find its own balance. Her oxygen saturation levels were lower than what is normal, but her body did not feel a lack of oxygen (no ischemia on multiple electrocardiograms). Over time, her heart worked so hard that it caused the pressure in her lungs to increase.  This combination made each of her colds/respiratory viruses so difficult for her.  She fought so hard.

We are so thankful that her beautiful little complicated heart gave us a whole year with her. 


Amy said...

Jenny, I love hearing stories about your sweet Julia. What a beautiful little sweetheart!

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