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{{CMG}}; '''Associate Editor-In-Chief:''' [[User:KeriShafer|Keri Shafer, M.D.]] [mailto:kshafer@bidmc.harvard.edu] [[Priyamvada Singh|Priyamvada Singh, MBBS]] [[mailto:psingh@perfuse.org]]; '''Assistant Editor-In-Chief:''' [[Kristin Feeney|Kristin Feeney, B.S.]] [[mailto:kfeeney@perfuse.org]]
{{CMG}}; '''Associate Editor-In-Chief:''' {{Sara.Zand}} [[User:KeriShafer|Keri Shafer, M.D.]] [mailto:kshafer@bidmc.harvard.edu] [[Priyamvada Singh|Priyamvada Singh, MBBS]] [mailto:psingh13579@gmail.com]; '''Assistant Editor-In-Chief:''' [[Kristin Feeney|Kristin Feeney, B.S.]] [mailto:kfeeney@elon.edu]
==Overview==
Patients with [[tricuspid atresia]] should be differentiated from other [[cardiac]] causes of [[cyanosis]] and [[lung]] olygemia include:[[Tetralogy of Fallot]], [[Truncus arteriosus]], [[Total anomalous pulmonary venous connection]], [[Pulmonary atresia]], [[Tricuspid stenosis]].
== Differentiating tricuspid atresia from other Diseases ==
 
 


==Overview==
Patients with [[tricuspid atresia]] should be differentiated from other [[cardiac]] causes of [[cyanosis]] and [[lung]] olygemia include:


==Differentiating Tricuspid atresia from other Diseases==
Patients with tricuspid atresia should be differentiated from other cardiac and non-cardiac causes of cyanosis-


Cardiac causes (starts with 't'):
* [[Tetralogy of Fallot]]
* [[Tetralogy of Fallot]]
* [[Truncus arteriosus]]
* [[Total anomalous pulmonary venous connection]]
* [[Total anomalous pulmonary venous connection]]
* Other tricuspid valve abnormalities like [[tricuspid regurgitaton]], [[tricuspid stenosis]]
* [[Pulmonary atresia]],  
* [[Tricuspid stenosis]]
 
Table below compares [[Congenital heart disease cyanotic|cyanotic heart]] diseases:<ref name="pmid19144126">{{cite journal |vauthors=Bailliard F, Anderson RH |title=Tetralogy of Fallot |journal=Orphanet J Rare Dis |volume=4 |issue= |pages=2 |date=January 2009 |pmid=19144126 |pmc=2651859 |doi=10.1186/1750-1172-4-2 |url=}}</ref><ref name="pmid30065477">{{cite journal |vauthors=Kao CC, Hsieh CC, Cheng PJ, Chiang CH, Huang SY |title=Total Anomalous Pulmonary Venous Connection: From Embryology to a Prenatal Ultrasound Diagnostic Update |journal=J Med Ultrasound |volume=25 |issue=3 |pages=130–137 |date=2017 |pmid=30065477 |pmc=6029298 |doi=10.1016/j.jmu.2017.08.002 |url=}}</ref><ref name="pmid13059216">{{cite journal |vauthors=ASTLEY R, OLDHAM JS, PARSONS C |title=Congenital tricuspid atresia |journal=Br Heart J |volume=15 |issue=3 |pages=287–97 |date=July 1953 |pmid=13059216 |pmc=479498 |doi=10.1136/hrt.15.3.287 |url=}}</ref><ref name="pmid24400257">{{cite journal |vauthors=Unolt M, Putotto C, Silvestri LM, Marino D, Scarabotti A, Valerio Massaccesi, Caiaro A, Versacci P, Marino B |title=Transposition of great arteries: new insights into the pathogenesis |journal=Front Pediatr |volume=1 |issue= |pages=11 |date=June 2013 |pmid=24400257 |pmc=3860888 |doi=10.3389/fped.2013.00011 |url=}}</ref>
{| class="wikitable"
|+
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Disorders
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Etiology
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Clinical Presentation
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Laboratory Findings
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Electrocardiogram Findings
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Echocardiography Findings
! align="center" style="background:#4479BA; color: #FFFFFF;" + |X-Ray Findings
|-
! style="padding: 5px 5px; background: #DCDCDC; " align="left"|'''[[Tetralogy of Fallot]]'''
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |Multifactorial
 
* [[Polymorphism (biology)|Genetic polymorphisms]]
* Maternal [[rubella]]
* Poor prenatal nutrition
* Maternal [[alcohol]] use
* Maternal [[diabetes]]
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* [[Cyanosis]] on exertion
* [[Exertional dyspnea]]
* [[Palpitation|Palpitations]]
* [[Fatigue]]
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* CBC: [[Anemia]] or [[polycythemia]].
* [[Coagulation]] profile.
* Arterial blood gas: Low [[oxygen saturation]] and [[acidosis]]
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
*[[Right ventricular hypertrophy]]
*[[Right bundle branch block]]
*[[Tachycardia]]
* Rate of [[QRS complex|QRS]] change predicts [[Ventricular arrhythmias|ventricular arrhythmia]]
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
[[Echocardiography]] may show:
 
* Residual [[Ventricular septal defect|VSD]] or [[Atrial septal defect|ASD]]
* RV outflow tract obstruction
* Abnormal valvular anatomy
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* The '''boot-shaped''' [[heart]] appearance
* Normal [[heart]] size
* [[Pulmonary]] vascular marking may be normal or decreased
|-
! style="padding: 5px 5px; background: #DCDCDC; " align="left"|'''[[Total anomalous pulmonary venous connection|Total Anomalous Pulmonary Venous Connection]]'''


Other less common causes are- [[pulmonary atresia]], [[hypoplastic left heart syndrome]], [[anomalous systemic venous connection]].
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |Multifactorial


*[[Polymorphism (biology)|Genetic polymorphisms]]
* Maternal [[rubella]]
* Poor prenatal nutrition
* Maternal [[alcohol]] use
* Maternal [[diabetes]]
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* [[Tachypnea]]
* [[Palpitation|Palpitations]]
* [[Cyanosis]]
* [[Failure to thrive]]
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* Arterial blood gas: Low [[oxygen saturation]] and [[acidosis]]
* [[Coagulation]] profile.
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* [[Right ventricular hypertrophy]] with a qR pattern
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
*[[Right ventricular hypertrophy]]
*Right ventricular loading
*Paradoxical septal motion
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* Prominence of the [[pulmonary]] arteries
* Mild enlargement of [[heart]]
* The classic '''snowman''' sign is seen in supracardiac subtype
|-
! style="padding: 5px 5px; background: #DCDCDC; " align="left"|'''[[Tricuspid atresia|Tricuspid Atresia]]'''
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |Multifactorial


Non-cardiac causes:
*[[Polymorphism (biology)|Genetic polymorphisms]]
* Pulmonary diseases - Structural abnormalities of the lung, V/P (ventilation-perfusion mismatch), airway obstruction, pneumothorax, and hypoventilation.
* Maternal [[rubella]]
* Abnormal hemoglobin like methemoglobin, polycythemia
* Poor prenatal nutrition
* Peripheral cyanosis for e.g. sepsis, hypoglycemia, dehydration, and hypoadrenalism.
* Maternal [[alcohol]] use
* Maternal [[diabetes]]
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* Respiratory difficulties  as nasal flaring or muscle retractions
* [[Cyanosis]]
* Growth retradation
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* [[Arterial blood gas|Arterial blood gases]]
* [[Complete blood count|CBC]]: [[Polycythemia]]
* [[Coagulation]] profile
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* Tall [[P wave|P waves]] indicate atrial enlargement.


* First-degree [[atrioventricular block]].
* Frontal plane [[QRS complex|QRS]] axis may be leftward.
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |Echocardiography may show
* Defect size
* Pulmonary [[blood flow]]
*[[Ventricular function]]
* Valve abnormalities
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* Smooth convexity of right [[heart]] border is absent
* Lower part of right [[heart]] border is limited to the [[spine]]
* Prominency of the [[pulmonary artery]] depends on their [[blood]] flow
* [[Cardiac]] size may be normal or enlarged
|-
! style="padding: 5px 5px; background: #DCDCDC; " align="left"|'''[[Transposition of the great vessels|Transposition of the Great Arteries]]'''
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
Multifactorial
*[[Polymorphism (biology)|Genetic polymorphisms]]
* Poor prenatal nutrition
* Maternal [[alcohol]] use
* Maternal [[diabetes]]
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* Prominent [[cyanosis]] within hours of birth
*[[Congestive heart failure]]
*
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
*[[Arterial blood gas|Arterial blood gases]]: [[Hypoxemia]]
* Hyperoxia test
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* [[Right ventricular hypertrophy]]
* [[Right axis deviation]]
* Varying degrees of [[Atrioventricular block|AV block]]
* [[Q waves]]
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
[[Echocardiography]] may show:
* Relationship between [[great vessels]]
* Associated anatomic lesions
* [[Coronary artery]] origin and branches
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* The classic '''egg on string''' appearance
* [[Pulmonary]] vascular marking may be normal or increased
|}


==References==
==References==
{{reflist|2}}
{{reflist|2}}
[[Category:Cardiovascular system]]
[[Category:Cardiovascular system]]
[[Category:Cardiology]]
[[Category:Cardiology]]
[[Category:Congenital heart disease]]
[[Category:Congenital heart disease]]
[[Category:Pediatrics]]
[[Category:Pediatrics]]
 
[[category:Disease]]
[[Category:Cardiovascular diseases]]
[[Category:Up-To-Date cardiology]]
[[Category:Up-To-Date]]
[[Category:Needs overview]]
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Latest revision as of 15:52, 8 November 2020

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Sara Zand, M.D.[2] Keri Shafer, M.D. [3] Priyamvada Singh, MBBS [4]; Assistant Editor-In-Chief: Kristin Feeney, B.S. [5]

Overview

Patients with tricuspid atresia should be differentiated from other cardiac causes of cyanosis and lung olygemia include:Tetralogy of Fallot, Truncus arteriosus, Total anomalous pulmonary venous connection, Pulmonary atresia, Tricuspid stenosis.

Differentiating tricuspid atresia from other Diseases

Patients with tricuspid atresia should be differentiated from other cardiac causes of cyanosis and lung olygemia include:


Table below compares cyanotic heart diseases:[1][2][3][4]

Disorders Etiology Clinical Presentation Laboratory Findings Electrocardiogram Findings Echocardiography Findings X-Ray Findings
Tetralogy of Fallot Multifactorial

Echocardiography may show:

  • Residual VSD or ASD
  • RV outflow tract obstruction
  • Abnormal valvular anatomy
  • The boot-shaped heart appearance
  • Normal heart size
  • Pulmonary vascular marking may be normal or decreased
Total Anomalous Pulmonary Venous Connection Multifactorial
  • Prominence of the pulmonary arteries
  • Mild enlargement of heart
  • The classic snowman sign is seen in supracardiac subtype
Tricuspid Atresia Multifactorial
  • Respiratory difficulties as nasal flaring or muscle retractions
  • Cyanosis
  • Growth retradation
  • Tall P waves indicate atrial enlargement.
  • Frontal plane QRS axis may be leftward.
Echocardiography may show
Transposition of the Great Arteries

Multifactorial

Echocardiography may show:

  • The classic egg on string appearance
  • Pulmonary vascular marking may be normal or increased

References

  1. Bailliard F, Anderson RH (January 2009). "Tetralogy of Fallot". Orphanet J Rare Dis. 4: 2. doi:10.1186/1750-1172-4-2. PMC 2651859. PMID 19144126.
  2. Kao CC, Hsieh CC, Cheng PJ, Chiang CH, Huang SY (2017). "Total Anomalous Pulmonary Venous Connection: From Embryology to a Prenatal Ultrasound Diagnostic Update". J Med Ultrasound. 25 (3): 130–137. doi:10.1016/j.jmu.2017.08.002. PMC 6029298. PMID 30065477.
  3. ASTLEY R, OLDHAM JS, PARSONS C (July 1953). "Congenital tricuspid atresia". Br Heart J. 15 (3): 287–97. doi:10.1136/hrt.15.3.287. PMC 479498. PMID 13059216.
  4. Unolt M, Putotto C, Silvestri LM, Marino D, Scarabotti A, Valerio M, Caiaro A, Versacci P, Marino B (June 2013). "Transposition of great arteries: new insights into the pathogenesis". Front Pediatr. 1: 11. doi:10.3389/fped.2013.00011. PMC 3860888. PMID 24400257. Vancouver style error: initials (help)

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