Homozygous familial hypercholesterolemia is an
autosomal dominant disorder which is associated with
accelerated atherosclerosis and the development of
multiple tendon xanthomas.[
1] The primary challenge
is LDL receptor disorder in the liver that is why liver
transplantation is indicated, if medical therapy fails
to lower the blood lipid levels. Patients with HFH
carries out a high risk to develop CAD usually in the second or third decades of life; however, very early
onset of CAD in childhood has been also reported
in the literature.[
1] The youngest child reported for
CABG with familial hypercholesterolemia was a
seven-year-old boy[
2] However, our case is nominated
to be the youngest patient for CABG surgery in the
literature.
Recently, the use of arterial conduits for coronary
problems in infants and children has been widely
adopted with excellent long-term patency and growth
in children.[3,4] Variations in the adaptation to growth
of children have been shown between the arterial
and venous grafts with thoracic artery growing in a
proportion to somatic growth, while saphenous vein
grafts tend to course in a more linear way without any
increase in the length or diameter.[5-7] In our case, the
choice of LIMA depended on its growth potential.
However, the small size of the thorax, LIMA and
coronary arteries makes it difficult to position the
thorax, to harvest LIMA, and to anastomose it to a
small coronary artery. For multiple coronary artery
anastomoses, the right internal mammary artery can
be also used either as T-graft or as separately.
Very high blood LDL levels should be controlled in
patients with HFH via drugs, plasmapheresis, or liver
transplantation.[9] In patients refractory to medical
treatments, transplantation is the most effective and
most durable way to control LDL levels and its
complications,[10] as the problem is disorder of LDL
receptors at the liver. A new liver with a normal
working LDL receptor allows the patient to manipulate
the blood LDL level.
The first description of the living-related liver
transplantation procedure procured from a living
donor (the mother) and transplanted in a child dates
back to 1988.[8,9] Living-related liver transplants soon
came to account for a substantial number of pediatric
cases performed in many centers throughout the
world, and the only possibility for liver transplants in
countries, where cadaveric organ procurement was not
allowed until a few years ago.
In our case, the absence of the lateral wall
ischemia of the myocardium gave us the courage
not to do bypass to the circumflex artery, since we
did not prefer using neither a venous graft nor a
right internal mammary artery graft at such a young
case for the stretching potential of these grafts as
the child grows up. On the other hand, there were
soft atheromatous plaques at the aorta which may cause distally embolism. We cannulated aorta, as it
would be difficulty to do bypass surgery with offpump
techniques in a child; however, we avoided an
additional manipulation with proximal anastomosis
which we did not consider that the patient needed it,
due to the lack of the lateral wall ischemia.
In conclusion, coronary revascularization in
childhood is a rare procedure. The patients with
homozygous familial hypercholesterolemia must be
followed closely for the development of coronary
atherosclerosis particularly in patients scheduled for
liver transplantation. The surgical results for cardiac
revascularization are usually satisfactory in ischemic
patients. However, long-term survival of the patients
depends on strictly controlling lipid levels, due to the
ongoing nature of the disease. Our case is the youngest
patient with homozygous familial hypercholesterolemia
undergoing coronary artery bypass grafting procedure
with the longest follow-up.
Declaration of conflicting interests
The authors declared no conflicts of interest with respect
to the authorship and/or publication of this article.
Funding
The authors received no financial support for the research
and/or authorship of this article.