Down syndrome electrocardiogram: Difference between revisions

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__NOTOC__
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{{Down syndrome}}
{{Down syndrome}}
{{CMG}}; {{AE}}  
{{CMG}}; {{AE}} {{SH}}


==Overview==
==Overview==
There are no ECG findings associated with Down syndrome however 40-60 percent of patients with Down syndrome suffer from congenital heart defects the most common being atrial septal defect, atrioventricular septal defect, ventricular septal defect, and patent ductus arteriosus. The ECG findings in Down syndrome are because of the aforementioned underlying congenital heart defects.  
There are no [[The electrocardiogram|ECG]] findings associated with Down syndrome however 40-60 percent of patients with Down syndrome suffer from [[congenital heart defects]] the most common being [[atrial septal defect]], [[atrioventricular septal defect]], [[ventricular septal defect]], and [[patent ductus arteriosus]]. The ECG findings in Down syndrome are of the aforementioned underlying [[congenital heart defects]].  


==Electrocardiogram==
==Electrocardiogram==
*There are no ECG findings associated with Down syndrome however 40-60 percent<ref name="RoizenMagyar2014">{{cite journal|last1=Roizen|first1=Nancy J.|last2=Magyar|first2=Caroline I.|last3=Kuschner|first3=Emily S.|last4=Sulkes|first4=Steven B.|last5=Druschel|first5=Charlotte|last6=van Wijngaarden|first6=Edwin|last7=Rodgers|first7=Lisa|last8=Diehl|first8=Alison|last9=Lowry|first9=Richard|last10=Hyman|first10=Susan L.|title=A Community Cross-Sectional Survey of Medical Problems in 440 Children with Down Syndrome in New York State|journal=The Journal of Pediatrics|volume=164|issue=4|year=2014|pages=871–875|issn=00223476|doi=10.1016/j.jpeds.2013.11.032}}</ref><ref name="pmid1829969">{{cite journal |vauthors=Tubman TR, Shields MD, Craig BG, Mulholland HC, Nevin NC |title=Congenital heart disease in Down's syndrome: two year prospective early screening study |journal=BMJ |volume=302 |issue=6790 |pages=1425–7 |date=June 1991 |pmid=1829969 |pmc=1670107 |doi= |url=}}</ref> of patients with Down syndrome suffer from congenital heart defects the most common being ventricular septal defect, atrial septal defect, atrioventricular septal defect, and patent ductus arteriosus. The ECG findings in Down syndrome are because of the aforementioned underlying congenital heart defects.  
*There are no ECG findings associated with Down syndrome however 40-60 percent<ref name="RoizenMagyar2014">{{cite journal|last1=Roizen|first1=Nancy J.|last2=Magyar|first2=Caroline I.|last3=Kuschner|first3=Emily S.|last4=Sulkes|first4=Steven B.|last5=Druschel|first5=Charlotte|last6=van Wijngaarden|first6=Edwin|last7=Rodgers|first7=Lisa|last8=Diehl|first8=Alison|last9=Lowry|first9=Richard|last10=Hyman|first10=Susan L.|title=A Community Cross-Sectional Survey of Medical Problems in 440 Children with Down Syndrome in New York State|journal=The Journal of Pediatrics|volume=164|issue=4|year=2014|pages=871–875|issn=00223476|doi=10.1016/j.jpeds.2013.11.032}}</ref><ref name="pmid1829969">{{cite journal |vauthors=Tubman TR, Shields MD, Craig BG, Mulholland HC, Nevin NC |title=Congenital heart disease in Down's syndrome: two year prospective early screening study |journal=BMJ |volume=302 |issue=6790 |pages=1425–7 |date=June 1991 |pmid=1829969 |pmc=1670107 |doi= |url=}}</ref> of patients with Down syndrome suffer from congenital heart defects the most common being [[atrial septal defect]], [[ventricular septal defect]], [[atrioventricular septal defect]], and [[patent ductus arteriosus]]. The ECG findings in Down syndrome are because of the aforementioned underlying [[congenital heart defects]].  
*The ECG findings suggestive of an underlying congenital heart defects are:<ref name="CaroConde2014">{{cite journal|last1=Caro|first1=Milagros|last2=Conde|first2=Diego|last3=Pérez-Riera|first3=Andrés R.|last4=de Almeida|first4=Adail P.|last5=Baranchuk|first5=Adrian|title=The electrocardiogram in Down syndrome|journal=Cardiology in the Young|volume=25|issue=01|year=2014|pages=8–14|issn=1047-9511|doi=10.1017/S1047951114000420}}</ref>
*The ECG findings suggestive of an underlying congenital heart defects are:<ref name="CaroConde2014">{{cite journal|last1=Caro|first1=Milagros|last2=Conde|first2=Diego|last3=Pérez-Riera|first3=Andrés R.|last4=de Almeida|first4=Adail P.|last5=Baranchuk|first5=Adrian|title=The electrocardiogram in Down syndrome|journal=Cardiology in the Young|volume=25|issue=01|year=2014|pages=8–14|issn=1047-9511|doi=10.1017/S1047951114000420}}</ref>
''' Small VSD '''


*Restrictive VSD, Qρ/Qѕ < 1.5/1.0 Qρ/Qs is pressure gradient between pulmonary and systemic circulation: EKG is normal. A few patients will have an rsr' in V1.
'''Ventricular septal defect electrocardiogram'''
 
'''Small VSD '''
*Restrictive VSD, Qρ/Qѕ < 1.5/1.0 Qρ/Qs is pressure gradient between pulmonary and systemic circulation: EKG is normal.  
*A few patients will have an rsr' in V1.
''' Medium-sized VSD '''
''' Medium-sized VSD '''
*Left atrial overload - broad notched P wave
*Left atrial overload - broad notched P wave
*Left ventricular overload - Deep 'Q' wave, tall 'R' wave, tall 'T' wave in lead V5 and V6
*Left ventricular overload - Deep 'Q' wave, tall 'R' wave, tall 'T' wave in lead V5 and V6
*Atrial fibrillation can also be seen
*[[Atrial fibrillation]] can also be seen
''' Large VSD '''
''' Large VSD '''
*In adults or adolescence with a large VSD and pulmonary vascular obstructive disease, LVH is absent because volume overload of the LV is no longer present. Large VSD will produce [[right ventricular hypertrophy]] with [[right axis deviation]]. At this point there is either an rsR' pattern in the right precordial leads, or more  commonly, a tall monophasic R wave in the right precordial leads reflecting RVH. Also deep S waves in the lateral precordial leads and tall peaked P waves.
*In adults or adolescence with a large VSD and pulmonary vascular obstructive disease, [[Left ventricular hypertrophy|LVH]] is absent because volume overload of the [[Left ventricle|LV]] is no longer present. Large VSD will produce [[right ventricular hypertrophy]] with [[right axis deviation]]. At this point there is either an rsR' pattern in the right precordial leads, or more  commonly, a tall monophasic R wave in the right precordial leads reflecting RVH. Also deep S waves in the lateral precordial leads and tall peaked P waves.


'''Atrial septal defect'''
'''Atrial septal defect electrocardiogram'''
The [[ECG]] findings in atrial septal defect vary with the type of defect present.  
The [[ECG]] findings in atrial septal defect vary with the type of defect present.  


* It may be normal with an uncomplicated ASD and a small shunt.  
* It may be normal with an uncomplicated ASD and a small shunt.  
* Individuals with atrial septal defects may have a prolonged [[PR interval]] (a [[first degree heart block]]). The prolongation of the [[PR interval]] is probably due to the enlargement of the atria that is common in ASDs and the increased distance due to the defect itself.  
* Individuals with [[Atrial septal defect|atrial septal defects]] may have a prolonged [[PR interval]] (a [[first degree heart block]]). The prolongation of the [[PR interval]] is probably due to the enlargement of the atria that is common in ASDs and the increased distance due to the defect itself.  
* Incomplete and less frequently complete [[right bundle branch block]] ([[RBBB]]) is often present. [[Right ventricular hypertrophy]] ([[RVH]]) with strain suggests onset of [[pulmonary hypertension]] or associated [[pulmonic stenosis]].The QRS complex may be slightly prolonged and has a characteristic rSr' or rsR' pattern that is contributed to the disproportionate thickening of the right ventricular outflow tract (the last portion of the ventricle to depolarize).  
* Incomplete and less frequently complete [[right bundle branch block]] ([[RBBB]]) is often present.  
* [[Right ventricular hypertrophy]] ([[RVH]]) with strain suggests onset of [[pulmonary hypertension]] or associated [[pulmonic stenosis]].
* The QRS complex may be slightly prolonged and has a characteristic rSr' or rsR' pattern that is contributed to the disproportionate thickening of the right ventricular outflow tract (the last portion of the ventricle to depolarize).  
'''Lesion Specific Electrocardiogram Findings'''
'''Lesion Specific Electrocardiogram Findings'''
*[[Ostium secundum ASD]]- Patients with ostium secundum ASDs often develop [[atrial fibrillation]] or [[atrial flutter]], and this occurs with a higher incidence with increasing age and with [[pulmonary hypertension]]. 2 out of 3 patients with an ostium secundum ASD have [[right axis deviation]], incomplete [[right bundle-branch block]].
*[[Ostium secundum ASD]]- Patients with ostium secundum ASDs often develop [[atrial fibrillation]] or [[atrial flutter]], and this occurs with a higher incidence with increasing age and with [[pulmonary hypertension]]. 2 out of 3 patients with an ostium secundum ASD have [[right axis deviation]], incomplete [[right bundle-branch block]].
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* [[Sinus venosus ASD]] - Individuals with a sinus venosus ASD exhibit a left axis deviation of the [[P wave]] (not the [[QRS]] complex). It is often associated with low atrial and [[junctional rhythm|junctional rhythms]], abnormal P-wave axis.  
* [[Sinus venosus ASD]] - Individuals with a sinus venosus ASD exhibit a left axis deviation of the [[P wave]] (not the [[QRS]] complex). It is often associated with low atrial and [[junctional rhythm|junctional rhythms]], abnormal P-wave axis.  
* Familial ASD - [[Complete heart block]] may be present in association with familial ASD <ref name="pmid5437412">{{cite journal| author=Bizarro RO, Callahan JA, Feldt RH, Kurland LT, Gordon H, Brandenburg RO| title=Familial atrial septal defect with prolonged atrioventricular conduction. A syndrome showing the autosomal dominant pattern of inheritance. | journal=Circulation | year= 1970 | volume= 41 | issue= 4 | pages= 677-83 | pmid=5437412 | doi= | pmc= | url= }} </ref>.
* Familial ASD - [[Complete heart block]] may be present in association with familial ASD <ref name="pmid5437412">{{cite journal| author=Bizarro RO, Callahan JA, Feldt RH, Kurland LT, Gordon H, Brandenburg RO| title=Familial atrial septal defect with prolonged atrioventricular conduction. A syndrome showing the autosomal dominant pattern of inheritance. | journal=Circulation | year= 1970 | volume= 41 | issue= 4 | pages= 677-83 | pmid=5437412 | doi= | pmc= | url= }} </ref>.
'''Atrioventricular septal defect electrocardiogram'''
*Rhythm: normal sinus rhythm, PVCs 30%
*PR interval: 1° AVB >50%
*QRS axis: Moderate to extreme LAD; normal with atypical
*QRS Configuration: rSr´ or rsR´
*Atrial Enlargement: Possible LAE
*Ventricular hypertrophy: Uncommon in partial; BVH in complete; RVH with Eisenmenger
*Particularities: Inferoposteriorly displaced AVN
'''Patent ductus arteriosus electrocardiogram'''
*An [[electrocardiogram]] will appear differently depending on the severity of [[disease]] onset. In general, one can expect:
*Small PDA: the EKG is normal.
*Medium-sized PDA: there is [[LVH]], [[LA]] increase, [[prolonged PR interval]] and eventual [[atrial fibrillation]].
*Large-sized PDA: is similar to that of a [[VSD]] complicated by [[pulmonary hypertension]]. One can also expect:
**Evidence of [[LVH]] is decreased or absent because there is essentially normal volume work by the [[LV]].
**There is [[RVH]] instead with a large [[R wave]] in [[precordial leads|V1]]. No Rsr' like [[ASD]].
**Marked [[right axis deviation]] is common.
**Peaked [[RA]] [[p wave]]s are present


==References==
==References==

Latest revision as of 06:31, 21 March 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Dildar Hussain, MBBS [2]

Overview

There are no ECG findings associated with Down syndrome however 40-60 percent of patients with Down syndrome suffer from congenital heart defects the most common being atrial septal defect, atrioventricular septal defect, ventricular septal defect, and patent ductus arteriosus. The ECG findings in Down syndrome are of the aforementioned underlying congenital heart defects.

Electrocardiogram

Ventricular septal defect electrocardiogram

Small VSD

  • Restrictive VSD, Qρ/Qѕ < 1.5/1.0 Qρ/Qs is pressure gradient between pulmonary and systemic circulation: EKG is normal.
  • A few patients will have an rsr' in V1.

Medium-sized VSD

  • Left atrial overload - broad notched P wave
  • Left ventricular overload - Deep 'Q' wave, tall 'R' wave, tall 'T' wave in lead V5 and V6
  • Atrial fibrillation can also be seen

Large VSD

  • In adults or adolescence with a large VSD and pulmonary vascular obstructive disease, LVH is absent because volume overload of the LV is no longer present. Large VSD will produce right ventricular hypertrophy with right axis deviation. At this point there is either an rsR' pattern in the right precordial leads, or more commonly, a tall monophasic R wave in the right precordial leads reflecting RVH. Also deep S waves in the lateral precordial leads and tall peaked P waves.

Atrial septal defect electrocardiogram The ECG findings in atrial septal defect vary with the type of defect present.

Lesion Specific Electrocardiogram Findings

Atrioventricular septal defect electrocardiogram

  • Rhythm: normal sinus rhythm, PVCs 30%
  • PR interval: 1° AVB >50%
  • QRS axis: Moderate to extreme LAD; normal with atypical
  • QRS Configuration: rSr´ or rsR´
  • Atrial Enlargement: Possible LAE
  • Ventricular hypertrophy: Uncommon in partial; BVH in complete; RVH with Eisenmenger
  • Particularities: Inferoposteriorly displaced AVN

Patent ductus arteriosus electrocardiogram

References

  1. Roizen, Nancy J.; Magyar, Caroline I.; Kuschner, Emily S.; Sulkes, Steven B.; Druschel, Charlotte; van Wijngaarden, Edwin; Rodgers, Lisa; Diehl, Alison; Lowry, Richard; Hyman, Susan L. (2014). "A Community Cross-Sectional Survey of Medical Problems in 440 Children with Down Syndrome in New York State". The Journal of Pediatrics. 164 (4): 871–875. doi:10.1016/j.jpeds.2013.11.032. ISSN 0022-3476.
  2. Tubman TR, Shields MD, Craig BG, Mulholland HC, Nevin NC (June 1991). "Congenital heart disease in Down's syndrome: two year prospective early screening study". BMJ. 302 (6790): 1425–7. PMC 1670107. PMID 1829969.
  3. Caro, Milagros; Conde, Diego; Pérez-Riera, Andrés R.; de Almeida, Adail P.; Baranchuk, Adrian (2014). "The electrocardiogram in Down syndrome". Cardiology in the Young. 25 (01): 8–14. doi:10.1017/S1047951114000420. ISSN 1047-9511.
  4. Clark E, Kugler J (1982). "Preoperative secundum atrial septal defect with coexisting sinus node and atrioventricular node dysfunction". Circulation. 65 (5): 976–80. PMID 7074763.
  5. Bizarro RO, Callahan JA, Feldt RH, Kurland LT, Gordon H, Brandenburg RO (1970). "Familial atrial septal defect with prolonged atrioventricular conduction. A syndrome showing the autosomal dominant pattern of inheritance". Circulation. 41 (4): 677–83. PMID 5437412.

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