114 – Heterotaxy, AVSD, i-IVC, l-SVC

(See Normal Heart Image for comparison)
Click image to see download options from Flickr:

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(Click to see this heart after the NORWOOD or after the KAWASHIMA or after the Fontan)

-Aorta Arch hypoplastia (hypoplastia) [hypo=under, plasia=formation or development] the main vessel carrying oxygen-rich blood from the heart to the body is underdeveloped and too narrow.

Atrioventricular Septal Defect (AVSD) [septum=wall between the chambers of the heart, atriums=top chambers of the heart, ventricles=lower chambers of the heart]

-left-Superior Vena Cava (b-SVC) [superior=upper, vena cava=large vein] The upper vena cava (which brings oxygen-poor blood back from the body to the heart) is on the left side of the body. Normally it is on the right side of the body

-Interrupted Inferior Vena Cava (i-IVC) [interrupted=a break, inferior=lower, vena=vein, cava=space]  The main vein from the lower half of the body doesn’t lead into the heart as it should.  Instead, oxygen-depleted blood returns to the heart following a unique path:

  1. Hemiazygos continuation- [The Hemiazygos vein travels behind the heart near the spine.  It connects the upper and lower Vena Cava outside of the heart and sometimes acts as an alternate route for oxygen-depleted blood when the normal path is closed.] In this heart, it connects at the…
  2. Superior Vena Cava [superior=higher, vena=vein, cava=space] – the main vein from the upper half of the body leading into the heart that brings oxygen-depleted blood into the heart.

-Patent Ductus Arteriosus (PDA) [patent=open, ductus=duct, arteriosus=artery] an extra passageway between the pulmonary artery (carrying oxygen-poor blood from the heart to the lungs) and the aorta (carrying oxygen-rich blood from the heart to the body).  The ductus arteriosus is open in a fetus.  This allows extra bloodflow in the forming baby while it’s getting oxygen from Mom instead of its own lungs.  The PDA normally closes around 10 days after birth.

Also (not pictured):

-Heterotaxy [hetero=different, taxy=forming]:abnormal position of the organs inside the body. Look here for more information: http://www.chop.edu/service/cardiac-center/heart-conditions/heterotaxy-syndrome.html

with left Atrial Isomerism – refers to the arrangement of the organs in the body.

  • two left atria (instead of a left atrium and a right atrium)
  • Midline liver – the liver runs down the middle of the body instead of off to one side.)
  • bi-lobed lungs  – both lungs have two lobes on each side instead (as normal).

(Click here for an image showing the different types of atrial isomerism associated with heterotaxy:  http://www.pnas.org/content/108/7/2915/F1.expansion.html)

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One Thought to “114 – Heterotaxy, AVSD, i-IVC, l-SVC”

  1. […] to see this heart AT BIRTH or BEFORE the Kawashima [Norwood]  or after the […]

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