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==Overview==
==Overview==
[[Eisenmenger's syndrome]] should be differentiated from [[idiopathic pulmonary hypertension]], [[pulmonary infarction]], [[respiratory failure]], [[tricuspid atresia]], [[persistent truncus arteriosus]], and other [[Congenital heart disease|congenital heart diseases]].
==Differential Diagnosis of causes of Eisenmenger's Syndrome==
==Differential Diagnosis of causes of Eisenmenger's Syndrome==
A number of congenital heart defects can cause Eisenmenger's syndrome, including [[Atrial septal defect|atrial septal defect,]] [[patent ductus arteriosus]], and [[ventricular septal defect]].
[[Eisenmenger's syndrome]] should be differentiated from [[idiopathic pulmonary hypertension]], [[pulmonary infarction]], [[respiratory failure]], [[tricuspid atresia]], [[persistent truncus arteriosus]], and other [[Congenital heart disease|congenital heart diseases]]<ref name="pmid29939577">{{cite journal| author=| title=StatPearls | journal= | year= 2020 | volume=  | issue=  | pages=  | pmid=29939577 | doi= | pmc= | url= }}</ref><ref name="pmid19541542">{{cite journal| author=Moons P, Canobbio MM, Budts W| title=Eisenmenger syndrome: A clinical review. | journal=Eur J Cardiovasc Nurs | year= 2009 | volume= 8 | issue= 4 | pages= 237-45 | pmid=19541542 | doi=10.1016/j.ejcnurse.2009.05.004 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19541542  }}</ref><ref name="pmid9556469">{{cite journal| author=Vongpatanasin W, Brickner ME, Hillis LD, Lange RA| title=The Eisenmenger syndrome in adults. | journal=Ann Intern Med | year= 1998 | volume= 128 | issue= 9 | pages= 745-55 | pmid=9556469 | doi=10.7326/0003-4819-128-9-199805010-00008 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9556469  }}</ref><ref name="pmid12411974">{{cite journal| author=Berman EB, Barst RJ| title=Eisenmenger's syndrome: current management. | journal=Prog Cardiovasc Dis | year= 2002 | volume= 45 | issue= 2 | pages= 129-38 | pmid=12411974 | doi=10.1053/pcad.2002.127492 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12411974  }}</ref>.  
{| class="wikitable"
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! align="center" style="background:#4479BA; color: #FFFFFF;" + |Echocardiography
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Echocardiography
|-
|-
|Idiopathic [[Pulmonary hypertension|Pulmonary Hypertension]]
|[[Pulmonary hypertension|Idiopathic Pulmonary Hypertension]]
|Unknown, but possible reasons may include
 
* [[Connective tissue disease]]
* [[Cirrhosis|Liver cirrhosis]]
* [[Anorexia|Anorex]]<nowiki/>ic and alpha-adrenergic stimulants [<nowiki/>[[cocaine]]/[[amphetamine]]]
* [[HIV AIDS|HIV infection]]
|
|
|<nowiki/><br />
* <nowiki/>[[Dyspnea]]
|<nowiki/><br />
 
|<nowiki/><br />
* Recurrent [[syncope]]
* [[Fatigue]]
* [[Chest pain]]
* [[Cough]]
* [[Hemoptysis]]
|
|
* <nowiki/>+ve [[Antinuclear antibodies|ANA]] assay
* <nowiki/>+ve [[Anti-neutrophil cytoplasmic antibody|ANCA]]
* <nowiki/>Anti-topoisomerase antibody
* <nowiki/>[[Thyrotropin]]: screen thyroid disorders
* <nowiki/>Elevated [[Brain natriuretic peptide|BNP]] & N-terminal BNP.<br />
|
* <nowiki/>[[Right axis deviation]]
* <nowiki/>[[Right ventricular hypertrophy]]
* <nowiki/>[[ST depression]]
* <nowiki/>[[T-wave inversion|T-wave inversion<br />]]
|
* Flattening of the [[Interventricular septum|intraventricular septum]] during systole & diastole
*[[Right ventricular hypertrophy]]
* Reduced right ventricular function
*[[Tricuspid regurgitation]]
|-
|-
|[[Respiratory failure|Respiratory Failure]]
|[[Respiratory failure|Respiratory Failure]]
|
|
* Respiratory: [[Chronic obstructive pulmonary disease|COPD]], [[Pneumonia]], [[Pulmonary embolism]], [[Pulmonary hypertension]]
* Nervous: [[Guillain-Barré syndrome]], [[muscular dystrophy]], and [[myasthenia gravis]]
* Cardiac: [[Congenital heart disease|Congenital heart diseases]]
|
|
* [[Cyanosis]]
* [[Dyspnea]]
* [[Tachypnea]]
* [[Tachycardia]]
* [[Confusion]]
* [[Asterixis]]
|
|
|
* [[Arterial blood gas|Arterial blood gases]] (ABG) may detect [[hypoxemia]], hypercapnea, and [[acidosis]]
|
* [[Pulmonary function tests]]
|[[Electrocardiography]] may be used to evaluate the underlying cardiac causes of [[respiratory failure]].
|[[Echocardiography]] may be used to evaluate the underlying cardiac causes of [[respiratory failure]].
 
* Left ventricular dilatation
* Regional wall motion abnormalities
*[[Mitral regurgitation]]
|-
|-
|Pulmonary Infarction
|[[Lung infarction|Pulmonary Infarction]]
|
|
|
* Cardiac diseases
* Inherited [[coagulation]] disorders
* [[Sickle-cell disease|Sickle cell anemia]]
* [[Neoplasm]]
* [[Dehydration]]
* [[Surgery]]
*
|
|
* [[Tachypnea]]
* [[Dyspnea]]
* [[Pleuritic chest pain]]
* [[Cough]]
* [[Hemoptysis]]
* [[Syncope]]
* [[Sweating]]
|
|
* ABG: [[Hypoxemia]], hypercapnea, and [[metabolic acidosis]]
* Elevated [[D Dimer|D-Dimer]] levels
* [[Complete blood count|CBC]]: High [[White blood cells|WBC]]<nowiki/>s
* [[Hypercoagulation]] panel
|
|
* [[Sinus tachycardia]]
* [[T wave inversion|T-wave inversion]]
* [[Right axis deviation]]
* [[Right bundle branch block]]
* [[P pulmonale]]
|The main diagnostic studies are
* CT [[pulmonary angiography]]
* V[[Ventilation/perfusion scan|entilation/perfusion]] scanning.
* However, [[echocardiography]] may diagnose underlying cardiac conditions.
|-
|-
|Tetralogy of Fallot
|[[Tetralogy of Fallot]]
|
|Multifactorial
|
 
* [[Polymorphism (biology)|Genetic polymorphisms]]
* Maternal [[rubella]]
* Poor prenatal nutrition
* Maternal [[alcohol]] use
* Maternal [[diabetes]]
|
|
* [[Cyanosis]] on exertion
* [[Exertional dyspnea]]
* [[Palpitation|Palpitations]]
* [[Fatigue]]
|
|
* CBC: [[Anemia]] or [[polycythemia]].
* [[Coagulation]] profile.
* Arterial blood gas: Low [[oxygen saturation]] and [[acidosis]]
|
|
*[[Right ventricular hypertrophy]]
*[[Right bundle branch block]]
*[[Tachycardia]]
* Rate of [[QRS complex|QRS]] change predicts [[Ventricular arrhythmias|ventricular arrhythmia]]
|[[Echocardiography]] may show:
* Residual [[Ventricular septal defect|VSD]] or [[Atrial septal defect|ASD]]
* RV outflow tract obstruction
* Abnormal valvular anatomy
|-
|-
|Total Anomalous Pulmonary Venous Connection
|[[Total anomalous pulmonary venous connection|Total Anomalous Pulmonary Venous Connection]]
|
|
Multifactorial
*[[Polymorphism (biology)|Genetic polymorphisms]]
* Maternal [[rubella]]
* Poor prenatal nutrition
* Maternal [[alcohol]] use
* Maternal [[diabetes]]
|
|
* [[Tachypnea]]
* [[Palpitation|Palpitations]]
* [[Cyanosis]]
* [[Failure to thrive]]
|
|
* Arterial blood gas: Low [[oxygen saturation]] and [[acidosis]]
* [[Coagulation]] profile.
|
|
* [[Right ventricular hypertrophy]] with a qR pattern
|
|
*[[Right ventricular hypertrophy]]
*Right ventricular loading
*Paradoxical septal motion
*
|-
|-
|Tricuspid Atresia
|[[Tricuspid atresia|Tricuspid Atresia]]
|
|Multifactorial
|
 
*[[Polymorphism (biology)|Genetic polymorphisms]]
* Maternal [[rubella]]
* Poor prenatal nutrition
* Maternal [[alcohol]] use
* Maternal [[diabetes]]
|
|
* Respiratory difficulties  as nasal flaring or muscle retractions
* [[Cyanosis]]
* Growth retradation
|
|
* [[Arterial blood gas|Arterial blood gases]]
* [[Complete blood count|CBC]]: [[Polycythemia]]
* [[Coagulation]] profile
|
|
* Tall [[P wave|P waves]] indicate atrial enlargement.
* First-degree [[atrioventricular block]].
* Frontal plane [[QRS complex|QRS]] axis may be leftward.
|Echocardiography may show
* Defect size
* Pulmonary [[blood flow]]
*[[Ventricular function]]
* Valve abnormalities
|-
|-
|Ventricular Septal Defect
|[[Ventricular septal defect|Ventricular Septal Defect]]
|
|Multifactorial
 
*[[Polymorphism (biology)|Genetic polymorphisms]]
* Poor prenatal nutrition
* Maternal [[alcohol]] use
* Maternal [[diabetes]]
|
|
* Asymptomatic: small [[Ventricular septal defect|VSD]]
* Excessive [[sweating]]
* [[Fatigue]] during feeding
* [[Failure to thrive]]
|
|
* High [[troponin]] I and amino-terminal [[procollagen]] type III peptide
* High [[Brain natriuretic peptide|BNP]] and pro-BNP levels
|
|
* Moderate [[Ventricular septal defect|VSD]]: [[Left ventricular hypertrophy]]
* Large [[Ventricular septal defect|VSD]]: [[Right ventricular hypertrophy]]
* Biphasic [[P wave]]: Left atrial dilatation
|
|
[[Echocardiography]] may show
* Defect size
* Pulmonary [[blood flow]]
* [[Ventricular function]]
* Valve abnormalities
|-
|-
|Transposition of the Great Arteries
|[[Transposition of the great vessels|Transposition of the Great Arteries]]
|
|Multifactorial
|
 
*[[Polymorphism (biology)|Genetic polymorphisms]]
* Poor prenatal nutrition
* Maternal [[alcohol]] use
* Maternal [[diabetes]]
|
|
* Prominent [[cyanosis]] within hours of birth
*[[Congestive heart failure]]
*
|
|
*[[Arterial blood gas|Arterial blood gases]]: [[Hypoxemia]]
* Hyperoxia test
|
|
* [[Right ventricular hypertrophy]]
* [[Right axis deviation]]
* Varying degrees of [[Atrioventricular block|AV block]]
* [[Q waves]]
|[[Echocardiography]] may show:
* Relationship between [[great vessels]]
* Associated anatomic lesions
* [[Coronary artery]] origin and branches
|-
|-
|Truncus Arteriosus
|[[Persistent truncus arteriosus]]
|
|
|
* [[Genetic disorders]]
* [[Teratogen|Teratogens]] ([[Virus|viruses]], metabolic imbalance, and industrial agents)
|
|
* [[Cyanosis]]
* [[Growth failure]]
* [[Fatigue]]
* Poor feeding
* [[Tachypnea]]
* [[Dyspnea]]
|
|
* [[Arterial blood gas]] for [[acidosis]] and [[oxygen saturation]]
* [[Hypocalcemia]] if associated with Digeorge syndrome
|
|
* [[Right axis deviation]]
* Biventricular hypertrophy
|[[Echocardiography]] may show
* Large truncal artery overriding the [[Ventricular septal defect|VSD]]
* Pulmonary [[blood flow]]
* [[Tricuspid regurgitation]]
|}
|}


Latest revision as of 04:18, 27 January 2020

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Abdelrahman Ibrahim Abushouk, MD[2]

Overview

Eisenmenger's syndrome should be differentiated from idiopathic pulmonary hypertension, pulmonary infarction, respiratory failure, tricuspid atresia, persistent truncus arteriosus, and other congenital heart diseases.

Differential Diagnosis of causes of Eisenmenger's Syndrome

Eisenmenger's syndrome should be differentiated from idiopathic pulmonary hypertension, pulmonary infarction, respiratory failure, tricuspid atresia, persistent truncus arteriosus, and other congenital heart diseases[1][2][3][4].

Disorders Etiology Clinical Presentation Laboratory Findings Electrocardiogram Echocardiography
Idiopathic Pulmonary Hypertension Unknown, but possible reasons may include
  • +ve ANA assay
  • +ve ANCA
  • Anti-topoisomerase antibody
  • Thyrotropin: screen thyroid disorders
  • Elevated BNP & N-terminal BNP.
Respiratory Failure Electrocardiography may be used to evaluate the underlying cardiac causes of respiratory failure. Echocardiography may be used to evaluate the underlying cardiac causes of respiratory failure.
Pulmonary Infarction
The main diagnostic studies are
Tetralogy of Fallot Multifactorial Echocardiography may show:
  • Residual VSD or ASD
  • RV outflow tract obstruction
  • Abnormal valvular anatomy
Total Anomalous Pulmonary Venous Connection

Multifactorial

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
Ventricular Septal Defect Multifactorial

Echocardiography may show

Transposition of the Great Arteries Multifactorial Echocardiography may show:
Persistent truncus arteriosus Echocardiography may show

References

  1. ↑ "StatPearls". 2020. PMID 29939577.
  2. ↑ Moons P, Canobbio MM, Budts W (2009). "Eisenmenger syndrome: A clinical review". Eur J Cardiovasc Nurs. 8 (4): 237–45. doi:10.1016/j.ejcnurse.2009.05.004. PMID 19541542.
  3. ↑ Vongpatanasin W, Brickner ME, Hillis LD, Lange RA (1998). "The Eisenmenger syndrome in adults". Ann Intern Med. 128 (9): 745–55. doi:10.7326/0003-4819-128-9-199805010-00008. PMID 9556469.
  4. ↑ Berman EB, Barst RJ (2002). "Eisenmenger's syndrome: current management". Prog Cardiovasc Dis. 45 (2): 129–38. doi:10.1053/pcad.2002.127492. PMID 12411974.


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