Official Diagnosis & Descriptions

 After reviewing Alyssa's medical records and meeting with a geneticist, we finally have what I believe is Alyssa's official diagnosis.

Hypoplastic Left Heart Syndrome  (diagnosed in-utero)
Aortic Valve Atresia and Mitral Valve Atresia (diagnosed in-utero w/ connection to HLHS)
Right Aortic Arch with an aberrant left subclavian artery (found during attempted Norwood and later officially diagnosed during cath procedure)
Complete vascular ring  (found during attempted Norwood and later officially diagnosed during cath procedure)
Large Atrial Septal Defect or ASD (Confirmed during echo done after cardiac arrest while performing CPR and putting her on ECMO)
Meckel's diverticulum (Discovered during exploratory surgery into her tummy after she went into cardiac arrest and they needed to look for internal bleeding before placing her on ECMO)
Heterotaxy -(Diagnosed by a geneticist after she passed away based on information found on her autopsy)


HLHS is fatal without surgical intervention; and even with the surgeries, her heart would never be whole.  There is no cure for HLHS, no fix that will make their heart function like that of a healthy heart.  But there is hope; hope that these beautiful babies with half a heart are able to live their life and feel the love and compassion they so deserve.  It may be a rough life and most certainly not that way any parent envisions their child to have to live.  But at least they are now able to live and be loved and show everyone just how strong they are.  1 out of every 10,000 babies will be born with HLHS.

Descriptin of HLHS and explanation of 3 staged surgeries starting with the Norwood
http://www.chop.edu/service/cardiac-center/heart-conditions/hypoplastic-left-heart-syndrome-hlhs.html

Description of Right Aortic Arch - Approximately 0.1% of population has a right sided aortic arch, and about half of these have an aberrant left subclavian artery which may arise either directly from the aorta or from the Kommerell’s diverticulum.1 Although an aberrant left subclavian artery may occur in isolation, it is the commonest anomaly associated with a right aortic arch. Any symptoms, which result from an aberrant left subclavian artery, are associated with compression of the esophagus or trachea and are most likely to occur if its origin is dilated.2, 3  Quoted from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3074843/

Description of Cardaic Catheterization and Echocardiogram - both of which Alyssa had (she had two in-utero fetal echos and two echos after birth, one before surgery and one after the attempted surgery)
http://www.chop.edu/service/cardiac-center/our-services/pediatric-cardiac-diagnosis-and-testing.html

Description of Atrial Septal Defect (ASD)
http://www.chop.edu/service/cardiac-center/heart-conditions/atrial-septal-defect-asd.html

Description of Meckel's Diverticulum
*Present in about 2% of all infants
http://www.chop.edu/healthinfo/meckels-diverticulum.html

Description of Heterotaxy
*Alyssa's form is known as polysplenia or left atrial isomerism
*Alyssa had 3 accessory spleens in addition to her normal spleen
*Heterotaxy is very rare.  For every 1,000,000 births, 4 of those babies will have heterotaxy.  Our sweet Alyssa really was 1 in a million.
http://www.heterotaxy.org/Pages/what_is_heterotaxy.aspx
http://www.chdbabies.com/2009/08/overview-of-heterotaxy.html

These are just the definitions of what we know effected our sweet baby girl.  Since our hospital never did any genetic testing on Alyssa, we will never know if she was a carrier of something that could be genetically linked to any of her defects.  It's just one of the many things that were sadly left unanswered in regards to our baby girl.