Transcript 6.Case Study 09-03-2012

Case Study
A. Swartbooi
Diagnostic Radiology, UFS
9 March 2012
Patient Info
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46 yr old male pt
Known cardiac pt
Previous Mitral valve replacement
Complaint of dyspnoea and orthopnoea
Clinical Presentation
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Chronically ill (RVD on Rx)
Pulse & Temp N
BP 110/80
Blood results N
Imaging
CXR
Imaging
CXR
• Findings:
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Dextrocardia
Cardiomegaly
Post Sternotomy wires
Right gastric air bubble
Left sided liver
Left lower pleural disease
Imaging
Heart Sonar
• Findings
– Dextrocardia
– Global hypokinesia
– EF 32%
Diagnosis
• Dextrocardia with situs inversus
Investigations
• Further evaluation in pt with
abovementioned chest findings include:
– Abdominal ultrasound
• If inconclusive
– CT abdomen
– MRI
– Angiography
Imaging
• Critical structures for evaluation of situs
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Cardiac apex
Atria
Lungs (bi- or trilobed)
Subdiaphragmatic venous Drainage
Aorta relative to midline
Stomach position (? malrotation)
Liver and gallbladder
Spleen (presence, appearance, number)
Imaging modalities
• Chest x-ray
– Plain chest and abdominal x-rays are the first
imaging modalities
– Presence or absence of normal situs
• Evaluate heart
• Stomach air bubble
• Liver outline
Imaging modalities
• Ultrasound
– Evaluate intra-abdominal solid organs
• Spleen
• Liver
– Vasculature
• Relation of large vessels
Imaging modalities
• Transthoracic echocardiography
– Directly visualize cardiovascular anomalies
• CT
• MRI
• Angiocardiography (Less commonly used)
Introduction
• Situs anomalies are often confusing, in part
because of the overlapping features of some
anomalies
• NB to understand the terminology
• Situs refers to the position of the heart and
viscera relative to midline.
Terminology
• Situs Solitis
• Situs inversus
• Situs ambiguous/Heterotaxy
Situs Solitus
• Situs solitus
– Represents the normal position of the heart and
abdominal viscera, with the cardiac apex,
spleen, stomach, and aorta located on the left
and the liver and inferior vena cava (IVC)
located on the right
– Congenital heart disease occurs in less than 1%
of individuals with situs solitus
– In case of dextrocardia 95% chance of CHD
Situs Solitus
Situs Inversus
• Situs Inversus
– Indicates mirror-image location of the organs
compared to situs solitus
– NB to recognize
• May help avoid mishaps at surgery or other interventions,
particularly in the emergency setting
– Two major subcategories
• Situs inversus with dextrocardia
• Situs inversus with levocardia
Situs Inversus
Situs Inversus
• Situs inversus with dextrocardia
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Situs inversus totalis
More common
Heart and viscera relative to situs solitus
Cardiac apex, spleen, stomach, and aorta
located on the right and the liver and IVC
located on the left
– 3-5 % chance of CHD
Situs Inversus
Situs Inversus
• Situs inversus with levocardia
– Extremely rare
– Mirror-image location of the viscera relative to
situs solitus and a left-sided cardiac apex
– 95 % chance of CHD
Heterotaxy
• Heterotaxy syndrome
– Rare condition that occurs in approximately
0.8% of patients with congenital heart disease
– Abnormal arrangement of organs and vessels as
opposed to the orderly arrangement typical of
situs solitus and situs inversus
Heterotaxy
• Patients with heterotaxy syndrome have a high
probability of having severe complex
cardiovascular anomalies (50 – 100%)
• Characterized not by a single set of abnormalities
but by a spectrum of abnormalities
• Encompasses 2 groups of diseases
• Asplenia
• Polysplenia
Heterotaxy
• Situs ambiguous with asplenia
• aKa Right isomerism or bilateral right-sidedness
• Findings include
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Bilat Trilobed lungs (minor fissures bilat)
Eparterial bronchi
Bilat left atria
Central liver
Stomach position indeterminate
Absent spleen
IVC and Aorta on same side of vertebra **
Heterotaxy
Heterotaxy
• Situs ambiguous with asplenia
– 99%–100% prevalence of congenital heart disease
– Common cardiac anomalies
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Common atrioventricular canal
Univentricular heart
TGA
TAPVR
– More in males
– Death in 1st yr of life up to 80%
– Asplenia related to greater risk for sepsis
• Carefull search for spleen NB
Heterotaxy
• Asplenia
Heterotaxy
• Situs ambiguous with polysplenia
• aKa Left isomerism or bilateral left-sidedness
• Findings include
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Bilat bilobed lungs
Bilat pulm atria
Central liver
Stomach position indeterminate
Multiple spleens
IVC interuption with continuation of azygos system
• No single anomaly is pathognomonic
Heterotaxy
Heterotaxy
• Situs ambiguous with polysplenia
– Cardiac anomalies less common and less
complex (50-90%)
– Most common cardiac anomalies
• PAPVR
• ASD
• Atrioventricular canal
– More in females
Heterotaxy
• Polysplenia
Summary
Summary
• Situs anomalies are often detected incidentally, with ⇧ frequency, in
adults during imaging evaluation for unrelated conditions
• It is important for radiologists to become familiar with these
anomalies, the spectrum of their manifestations, and their significance
• Not all patients with heterotaxy present with the classic features of
either asplenia or polysplenia
Summary
• Nb to utilize an individualized approach in these patients reflecting the
unique anatomy
– eg. Heterotaxy syndrome (bilateral bilobed lungs, levocardia, left
sided malrotated stomach)
• A complete, correct anatomic diagnosis cannot only lead to earlier
intervention, but also provide some indication of prognosis
THANK
YOU
References
• RadioGraphics 2002; 22:1439–1456
• RadioGraphics 1999; 19:837-852
• American Journal of Cardiology
Volume 81, Issue 2, Pages 188-194 , 15 January 1998
• Dahnert. Radiology Review Manual, page 593-594
• Weisleder. Primer of Diagnostic Radiology, page 139-140