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The Boot-shaped Heart Sign
Thorsang Chayovan
Outline
 Overview of the
boot-shaped heart
sign
 Tetralogy of Fallot
 Overview
 Radiology
 Prognosis and
Treatment
Haider, Ehsan A. "The Boot-shaped Heart Sign." Radiology.rsna.org. RSNA, 1 Jan. 2008. Web. 23 July 2012.
<http://radiology.rsna.org/content/246/1/328.full>.
The Boot-shaped Heart Sign
 Frontal chest
radiograph
 The shape of a
wooden boot
 Tetralogy of Fallot
Haider, Ehsan A. "The Boot-shaped Heart Sign." Radiology.rsna.org. RSNA, 1 Jan. 2008. Web. 23 July 2012.
<http://radiology.rsna.org/content/246/1/328.full>.
Haider, Ehsan A. "The Boot-shaped Heart Sign." Radiology.rsna.org. RSNA, 1 Jan. 2008. Web. 23 July 2012.
<http://radiology.rsna.org/content/246/1/328.full>.
Haider, Ehsan A. "The Boot-shaped Heart Sign." Radiology.rsna.org. RSNA, 1 Jan. 2008. Web. 23 July 2012.
<http://radiology.rsna.org/content/246/1/328.full>.
Tetralogy of Fallot
 One of the most common cyanotic congenital
heart conditions (75%)
 Accounts for 10% of all congenital heart
diseases
 Has estimated prevalence of 1 in 2,000 birth
Congenital Heart Diseases
Congenital
Heart Diseases
Acyanotic Cyanotic
Congenital Heart Diseases
Acyanotic
 Simple and single lesion
 Mechanism
 Left-to-right shunt
 Obstructive lesion
Cyanotic
 Multiple and probable
complex lesions
 Capillary reduced
hemoglobin > 5 gm%
(Oxygen saturation < 85%)
 Mechanism
 Right-to-left shunt
 Common mixing
 Parallel circulations
Tetralogy of Fallot
 Eccentric septation of the truncus arteriosus
 Conotruncal septum is displaced anteriorly
 The infundibular septum fails to fuse with the top of
the interventricular septum  high VSD
 The anterior part of aortic valve (superior border of
the VSD) overrides the ventricular septum 
overiding aorta
 The right ventricular outflow tract obstruction or
Pulmonary stenosis
 Right ventricular hypertrophyright ventricle at
systemic pressurelater in life
Embryogenesis of the heart
Witmer, Lawrence M. "Developmental Anatomy of the Heart and the Embryological Basis for Cardiac Defects." Ohio University, Athens.23 July 2012. Lecture.
Tetralogy of Fallot
 Obstruction of the
right ventricular
outflow tract
 High VSD
 Overriding aorta
 Right ventricle
hypertrophy
+ Atrial septal defect 
Pentalogy of
Fallot
Cukukoglu, Sardar M. "Cyanotic Diseases." Escardio.org. N.p., 2010.
Web. 23 July 2012.
<http://www.escardio.org/congresses/euroecho2010/Documents/teach
ing-course-slides/euroecho2010-cyaotic-disease-Kucukoglu-591.pdf>.
Tetralogy of Fallot
 Cyanosis
 Severe cases  at birth  severe PS
 Mild cases  much later  mild PS
 Squatting
 Dyspnea
 Failure to thrive
Tetralogy of Fallot
 Degree of pulmonary stenosis determines the
clinical picture
 If mild, the clinical picture of VSD is shown instead
 However the muscular element of pulmonary
stenosis is nearly always progressive
 Eventually reversing the left to right shunt to be
permanently right to left before the first birthday
Radiology in Tetralogy of Fallot
 The Boot-shaped
Heart sign
coeur en sabot
 The UPPER part of the
Boot: flat / concave
pulmonary trunk
 The TOE--upward
pointing cardiac apex:
concentric right
ventricular
hypertrophy
Accentuated by large lung volume,
small thymus, and lordotic
projection
Radiology in Tetralogy of Fallot
 Heart size
 The heart is usually not enlarged at birth
Unless there is a large left-to-right shunt
 Could be enlarged in later life from biventricular
hypoxic failure
 Pulmonary vasculaturedecreased
 Aortic arch
 Always enlarged
 25% has right-sided aortic arch
 might be associated with absent left pulmonary artery
 resulting in less pulmonary vasculature in the left lung
Upturned cardiac apex sign
 Concentric right ventricular hypertrophy
 However not seen in severe pulmonic stenosis
with intact interventricular septum
(RV systolic pressure >>> systemic systolic pressure)
 Maybe related to right ventricular hypertrophy
with altered development of the left ventricle
and abnormal horizontal ventricular septum
 Only encountered in congenital heart lesions and
not in acquired right ventricular hypertrophy
Gallard, Frank. "Tetralogy of Fallot." Radiopaedia.org. N.p., 5 Jan. 2010. Web. 23 July 2012. <http://radiopaedia.org/images/156636>.
"Cyanotic Heart Diseases." Learningradiology.com. N.p., n.d. Web. 23 July 2012. <www.learningradiology.com/.../cyanoticheartdz.pdf>.
MRI
 Exquisite anatomical and functional information
 Detailed assessment of the pulmonary artery is
particularly important
 Repair of the cardiac defects without addressing
pulmonary artery hypoplasia / stenosis has a poor
outcome
 The main pulmonary artery or right pulmonary
artery/ascending aorta < 0.3
 Primary repair would be unsuccessful
 Bridging shunt operation may be of benefit
 Assessment of coronary artery origin
 essential to surgical planning
Prognosis
 Depends on timing
 Best outcome seen in patients repaired before
the age of 5
 90% of untreated tetralogy patients succumb
by the age of 10 years
 Residual right ventricular dysfunction is
common
 Up to 10% of patients require re-operation within
20 years
Treatment
 Primary repair
 Preferred
usually performed at the time of diagnosis
 Palliative shunt
 typically in pulmonary arterial hypoplasia
 Post-surgical complications
 conduction dysfunction
 valvular dysfunction
Reference
 Haider, Ehsan A. "The Boot-shaped Heart Sign." Radiology.rsna.org. RSNA,
1 Jan. 2008. Web. 23 July 2012.
<http://radiology.rsna.org/content/246/1/328.full>.
 Grainger, Ronald G. Grainger & Alison's Diagnostic Radiology. 4th ed. Vol.
1. N.p.: Churchill Livingstone, 2003. Print.
 Cukukoglu, Sardar M. "Cyanotic Diseases." Escardio.org. N.p., 2010. Web.
23 July 2012.
<http://www.escardio.org/congresses/euroecho2010/Documents/teachin
g-course-slides/euroecho2010-cyaotic-disease-Kucukoglu-591.pdf>.
 Gallard, Frank. "Tetralogy of Fallot." Radiopaedia.org. N.p., 5 Jan. 2010.
Web. 23 July 2012. <http://radiopaedia.org/images/156636>.
 "Cyanotic Heart Diseases." Learningradiology.com. N.p., n.d. Web. 23 July
2012. <www.learningradiology.com/.../cyanoticheartdz.pdf>.
 Witmer, Lawrence M. "Developmental Anatomy of the Heart and the
Embryological Basis for Cardiac Defects." Ohio University, Athens. 23 July
2012. Lecture.

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The boot shaped heart sign

  • 1. The Boot-shaped Heart Sign Thorsang Chayovan
  • 2. Outline Overview of the boot-shaped heart sign Tetralogy of Fallot Overview Radiology Prognosis and Treatment Haider, Ehsan A. "The Boot-shaped Heart Sign." Radiology.rsna.org. RSNA, 1 Jan. 2008. Web. 23 July 2012. <http://radiology.rsna.org/content/246/1/328.full>.
  • 3. The Boot-shaped Heart Sign Frontal chest radiograph The shape of a wooden boot Tetralogy of Fallot Haider, Ehsan A. "The Boot-shaped Heart Sign." Radiology.rsna.org. RSNA, 1 Jan. 2008. Web. 23 July 2012. <http://radiology.rsna.org/content/246/1/328.full>.
  • 4. Haider, Ehsan A. "The Boot-shaped Heart Sign." Radiology.rsna.org. RSNA, 1 Jan. 2008. Web. 23 July 2012. <http://radiology.rsna.org/content/246/1/328.full>. Haider, Ehsan A. "The Boot-shaped Heart Sign." Radiology.rsna.org. RSNA, 1 Jan. 2008. Web. 23 July 2012. <http://radiology.rsna.org/content/246/1/328.full>.
  • 5. Tetralogy of Fallot One of the most common cyanotic congenital heart conditions (75%) Accounts for 10% of all congenital heart diseases Has estimated prevalence of 1 in 2,000 birth
  • 6. Congenital Heart Diseases Congenital Heart Diseases Acyanotic Cyanotic
  • 7. Congenital Heart Diseases Acyanotic Simple and single lesion Mechanism Left-to-right shunt Obstructive lesion Cyanotic Multiple and probable complex lesions Capillary reduced hemoglobin > 5 gm% (Oxygen saturation < 85%) Mechanism Right-to-left shunt Common mixing Parallel circulations
  • 8. Tetralogy of Fallot Eccentric septation of the truncus arteriosus Conotruncal septum is displaced anteriorly The infundibular septum fails to fuse with the top of the interventricular septum high VSD The anterior part of aortic valve (superior border of the VSD) overrides the ventricular septum overiding aorta The right ventricular outflow tract obstruction or Pulmonary stenosis Right ventricular hypertrophyright ventricle at systemic pressurelater in life
  • 9. Embryogenesis of the heart Witmer, Lawrence M. "Developmental Anatomy of the Heart and the Embryological Basis for Cardiac Defects." Ohio University, Athens.23 July 2012. Lecture.
  • 10. Tetralogy of Fallot Obstruction of the right ventricular outflow tract High VSD Overriding aorta Right ventricle hypertrophy + Atrial septal defect Pentalogy of Fallot Cukukoglu, Sardar M. "Cyanotic Diseases." Escardio.org. N.p., 2010. Web. 23 July 2012. <http://www.escardio.org/congresses/euroecho2010/Documents/teach ing-course-slides/euroecho2010-cyaotic-disease-Kucukoglu-591.pdf>.
  • 11. Tetralogy of Fallot Cyanosis Severe cases at birth severe PS Mild cases much later mild PS Squatting Dyspnea Failure to thrive
  • 12. Tetralogy of Fallot Degree of pulmonary stenosis determines the clinical picture If mild, the clinical picture of VSD is shown instead However the muscular element of pulmonary stenosis is nearly always progressive Eventually reversing the left to right shunt to be permanently right to left before the first birthday
  • 13. Radiology in Tetralogy of Fallot The Boot-shaped Heart sign coeur en sabot The UPPER part of the Boot: flat / concave pulmonary trunk The TOE--upward pointing cardiac apex: concentric right ventricular hypertrophy Accentuated by large lung volume, small thymus, and lordotic projection
  • 14. Radiology in Tetralogy of Fallot Heart size The heart is usually not enlarged at birth Unless there is a large left-to-right shunt Could be enlarged in later life from biventricular hypoxic failure Pulmonary vasculaturedecreased Aortic arch Always enlarged 25% has right-sided aortic arch might be associated with absent left pulmonary artery resulting in less pulmonary vasculature in the left lung
  • 15. Upturned cardiac apex sign Concentric right ventricular hypertrophy However not seen in severe pulmonic stenosis with intact interventricular septum (RV systolic pressure >>> systemic systolic pressure) Maybe related to right ventricular hypertrophy with altered development of the left ventricle and abnormal horizontal ventricular septum Only encountered in congenital heart lesions and not in acquired right ventricular hypertrophy
  • 16. Gallard, Frank. "Tetralogy of Fallot." Radiopaedia.org. N.p., 5 Jan. 2010. Web. 23 July 2012. <http://radiopaedia.org/images/156636>.
  • 17. "Cyanotic Heart Diseases." Learningradiology.com. N.p., n.d. Web. 23 July 2012. <www.learningradiology.com/.../cyanoticheartdz.pdf>.
  • 18. MRI Exquisite anatomical and functional information Detailed assessment of the pulmonary artery is particularly important Repair of the cardiac defects without addressing pulmonary artery hypoplasia / stenosis has a poor outcome The main pulmonary artery or right pulmonary artery/ascending aorta < 0.3 Primary repair would be unsuccessful Bridging shunt operation may be of benefit Assessment of coronary artery origin essential to surgical planning
  • 19. Prognosis Depends on timing Best outcome seen in patients repaired before the age of 5 90% of untreated tetralogy patients succumb by the age of 10 years Residual right ventricular dysfunction is common Up to 10% of patients require re-operation within 20 years
  • 20. Treatment Primary repair Preferred usually performed at the time of diagnosis Palliative shunt typically in pulmonary arterial hypoplasia Post-surgical complications conduction dysfunction valvular dysfunction
  • 21. Reference Haider, Ehsan A. "The Boot-shaped Heart Sign." Radiology.rsna.org. RSNA, 1 Jan. 2008. Web. 23 July 2012. <http://radiology.rsna.org/content/246/1/328.full>. Grainger, Ronald G. Grainger & Alison's Diagnostic Radiology. 4th ed. Vol. 1. N.p.: Churchill Livingstone, 2003. Print. Cukukoglu, Sardar M. "Cyanotic Diseases." Escardio.org. N.p., 2010. Web. 23 July 2012. <http://www.escardio.org/congresses/euroecho2010/Documents/teachin g-course-slides/euroecho2010-cyaotic-disease-Kucukoglu-591.pdf>. Gallard, Frank. "Tetralogy of Fallot." Radiopaedia.org. N.p., 5 Jan. 2010. Web. 23 July 2012. <http://radiopaedia.org/images/156636>. "Cyanotic Heart Diseases." Learningradiology.com. N.p., n.d. Web. 23 July 2012. <www.learningradiology.com/.../cyanoticheartdz.pdf>. Witmer, Lawrence M. "Developmental Anatomy of the Heart and the Embryological Basis for Cardiac Defects." Ohio University, Athens. 23 July 2012. Lecture.