CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 9/10, October/November 2013
e4
AFRICA
Case Report
Mini-sternotomy approach for aortic valve replacement
in a patient with osteogenesis imperfecta
GEORGIOS DIMITRAKAKIS, JITENDRAKUMAR RATHOD, ULRICH OTTO VON OPPELL,
AGAMEMNON PERICLEOUS, STEPHEN HUTCHISON
Abstract
Osteogenesis imperfecta (OI) is a heritable disorder of the
connective tissue. Cardiovascular involvement is rare, related
mainly to aortic valve regurgitation. Open-heart surgery in
these patients is associated with increased morbidity and
mortality rates as a result of tissue friability and bone brittle-
ness as well as platelet dysfunction.We present a patient with
OI who underwent successful aortic valve replacement with
a mini-sternotomy approach.
Keywords:
osteogenesis imperfecta, cardiac surgery, aortic
valve replacement, mini-sternotomy
Submitted 24/4/13, accepted 18/9/13
Cardiovasc J Afr
2013;
24
: e4–e7
DOI: 10.5830/CVJA-2013-070
Osteogenesis imperfecta (OI) is a heritable disorder of collagen
biosynthesis, which is transmitted in an autosomal dominant
fashion. OI is characterised mainly by osseous fragility and
even though its primary clinical manifestations include skeletal,
cutaneous, ocular, dental and otological abnormalities, serious
pathological conditions of the cardiovascular system have also
been reported sporadically.
The reported incidence is 1:20 000 to 1:50 000 patients
in the general population, with a predominance in females.
Cardiovascular involvement in OI is rare compared with that of
other connective tissue disorders. Morbidity and mortality rates
in OI patients who undergo cardiac surgery have been shown to
be high.
1
Case report
A 34-year-old female was referred to our department for surgical
intervention because of severe aortic valve regurgitation. The
patient had been diagnosed with OI during childhood. Her past
medical history included multiple limb fractures plus spinal
compression fractures and various tendon-lengthening operations
in childhood. She had also had a previous sternal fracture, which
had resulted in a pectus carinatum-type deformity as well as
kyphoscoliosis and she was wheelchair bound (Figs 1, 2). In
Department of Cardiothoracic Surgery, University Hospital
of Wales, Heath Park, Cardiff, United Kingdom
GEORGIOS DIMITRAKAKIS, MD, MSc,
JITENDRAKUMAR RATHOD, MSMch
ULRICH OTTO VON OPPELL, MBBCh, PhD
AGAMEMNON PERICLEOUS, BSc
STEPHEN HUTCHISON, MD
A
B
Fig. 1. A: pre-operative chest X-ray. B: postoperative
chest X-ray.
