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==Causes==
==Causes==
The cause of APW has not been identified.
 
* The cause of APW has not been identified.
* It may be associated with some genetic disease such as [[DiGeorge syndrome]](choromosome 22q11 deletion)<ref name="AurigemmaDixon2018">{{cite journal|last1=Aurigemma|first1=David|last2=Dixon|first2=Chandler|last3=Tucker|first3=Suzanne|last4=Davis|first4=Christopher|last5=Silverman|first5=Norman|title=Aortopulmonary window in tetralogy of Fallot with absent conal septum|journal=Echocardiography|volume=36|issue=2|year=2018|pages=411–414|issn=0742-2822|doi=10.1111/echo.14243}}</ref>


==Differentiating Aortopulmonary Window from other Diseases==
==Differentiating Aortopulmonary Window from other Diseases==
Line 26: Line 28:


==Epidemiology and Demographics==
==Epidemiology and Demographics==
The incidence/prevalence of [disease name] is approximately [number range] per 100,000 individuals worldwide.
OR
In [year], the incidence/prevalence of [disease name] was estimated to be [number range] cases per 100,000 individuals worldwide.
OR
In [year], the incidence of [disease name] is approximately [number range] per 100,000 individuals with a case-fatality rate of [number range]%.
Patients of all age groups may develop [disease name].
OR
The incidence of [disease name] increases with age; the median age at diagnosis is [#] years.
OR
[Disease name] commonly affects individuals younger than/older than [number of years] years of age.
OR
[Chronic disease name] is usually first diagnosed among [age group].
OR
[Acute disease name] commonly affects [age group].
There is no racial predilection to [disease name].
OR
[Disease name] usually affects individuals of the [race 1] race. [Race 2] individuals are less likely to develop [disease name].
[Disease name] affects men and women equally.


OR
* Since APW is a very rare congenital heart defect, there is very little known about epidemiology of the disease.
 
[Gender 1] are more commonly affected by [disease name] than [gender 2]. The [gender 1] to [gender 2] ratio is approximately [number > 1] to 1.
 
 
 
The majority of [disease name] cases are reported in [geographical region].
 
OR
 
[Disease name] is a common/rare disease that tends to affect [patient population 1] and [patient population 2].


==Risk Factors==
==Risk Factors==
There are no established risk factors for [disease name].
There are no established risk factors for aortopulmonary window.
 
OR
 
The most potent risk factor in the development of [disease name] is [risk factor 1]. Other risk factors include [risk factor 2], [risk factor 3], and [risk factor 4].
 
OR
 
Common risk factors in the development of [disease name] include [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].
 
OR
 
Common risk factors in the development of [disease name] may be occupational, environmental, genetic, and viral.


==Screening==
==Screening==
There is insufficient evidence to recommend routine screening for [disease/malignancy].
There is insufficient evidence to recommend routine screening for aortopulmonary window.
 
OR
 
According to the [guideline name], screening for [disease name] is not recommended.
 
OR
 
According to the [guideline name], screening for [disease name] by [test 1] is recommended every [duration] among patients with [condition 1], [condition 2], and [condition 3].


==Natural History, Complications, and Prognosis==
==Natural History, Complications, and Prognosis==
If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].


OR
* Clinical features of APW have been described differently due to their classification.
 
* Manifestation of the disease is not specific, but majority of patients have a large left to right shunt.
Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
* patients with small defects may be asymptomatic.
 
* If left untreated, large APW may cause symptoms of pulmonary hypertension and congestive heart failure such as [[tachypnea]], [[diaphoresis]], [[failure to thrive]] and recurrent respiratory difficulties.
OR
* pericardium is often hyperdynamic and a mitral valve rumble, causes continues heart murmur.
 
* Bounding pulses happen due to decreased diastolic blood pressure secondary to aortic flow reversal in diastole.
Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.
* If the diagnosis is not made until late in infancy or during childhood. APW may lead to [[Eisenmenger's syndrome|eisenmenger syndrome.]]<ref name="MyersLador2016">{{cite journal|last1=Myers|first1=Patrick O.|last2=Lador|first2=Frédéric|last3=Hachulla|first3=Anne-Lise|last4=Bouchardy|first4=Judith|last5=Noble|first5=Stéphane|last6=Licker|first6=Marc|last7=Pache|first7=Jean-Claude|last8=Kalimanovaska-Ostric|first8=Dimitra|last9=Djukic|first9=Milan|last10=Kalangos|first10=Afksendiyos|last11=Beghetti|first11=Maurice|title=Unrestrictive Aortopulmonary Window|journal=Circulation|volume=133|issue=19|year=2016|pages=1907–1910|issn=0009-7322|doi=10.1161/CIRCULATIONAHA.115.020819}}</ref>


==Diagnosis==
==Diagnosis==
===Diagnostic Study of Choice===
===Diagnostic Study of Choice===
The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met: [criterion 1], [criterion 2], [criterion 3], and [criterion 4].
The diagnosis of aortopulmonary window is made by echocardigraphy after suspicion of large left to right shunt. <ref name="ZhaoYang2019">{{cite journal|last1=Zhao|first1=Dong|last2=Yang|first2=Keming|last3=Li|first3=Shoujun|last4=Yan|first4=Jun|last5=Hua|first5=Zhongdong|last6=Fang|first6=Nengxin|last7=Su|first7=Wenjun|last8=Lv|first8=Xiaodong|last9=Yu|first9=Bing|title=Outcomes of different rehabilitative procedures in patients with pulmonary atresia, ventricular septal defect and major aortopulmonary collateral arteries|journal=European Journal of Cardio-Thoracic Surgery|volume=55|issue=5|year=2019|pages=837–844|issn=1010-7940|doi=10.1093/ejcts/ezy375}}</ref>
 
OR
 
The diagnosis of [disease name] is based on the [criteria name] criteria, which include [criterion 1], [criterion 2], and [criterion 3].
 
OR
 
The diagnosis of [disease name] is based on the [definition name] definition, which includes [criterion 1], [criterion 2], and [criterion 3].
 
OR
 
There are no established criteria for the diagnosis of [disease name].


===History and Symptoms===
===History and Symptoms===
The majority of patients with [disease name] are asymptomatic.


OR
* [[tachypnea]]
 
* [[diaphoresis]]
The hallmark of [disease name] is [finding]. A positive history of [finding 1] and [finding 2] is suggestive of [disease name]. The most common symptoms of [disease name] include [symptom 1], [symptom 2], and [symptom 3]. Common symptoms of [disease] include [symptom 1], [symptom 2], and [symptom 3]. Less common symptoms of [disease name] include [symptom 1], [symptom 2], and [symptom 3].
* [[failure to thrive]]  
* recurrent respiratory difficulties


===Physical Examination===
===Physical Examination===
Patients with [disease name] usually appear [general appearance]. Physical examination of patients with [disease name] is usually remarkable for [finding 1], [finding 2], and [finding 3].
OR
Common physical examination findings of [disease name] include [finding 1], [finding 2], and [finding 3].
OR
The presence of [finding(s)] on physical examination is diagnostic of [disease name].
OR


The presence of [finding(s)] on physical examination is highly suggestive of [disease name].
* continuous murmur
* If late [[Eisenmenger's syndrome|eisenmenger]]'s syndrome:
** [[cyanosis]]
** [[clubbing]]  


===Laboratory Findings===
===Laboratory Findings===

Revision as of 14:53, 4 April 2020

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

Synonyms and keywords:

Overview

Historical Perspective

Aortopulmonary window first described by Elliotson as a defect which causes communication between proximal aorta and main pulmonary artery. [1]

Classification

There are different ways of classifying aortopulmonary window. proximal defects are the most common between proximal aorta and sinus of valsalva. Distal types are located in the upper portion of the ascending before the aortic branches. Total defects are large and involve the majority of the ascending aorta between valsalva and aortic branches.[2]

Pathophysiology

  • Aortopulmonary (APW) window is a rare condition with presence of a communication between the ascending aorta and pulmonary artery with two separate semilunar valves.[3]
  • APW can present as an isolated phenomena but more over is assosiated with other congenital heart defects such as interrupted aortic arch, transposition of the great arteries and tetralogy of fallot.[4]

Causes

  • The cause of APW has not been identified.
  • It may be associated with some genetic disease such as DiGeorge syndrome(choromosome 22q11 deletion)[5]

Differentiating Aortopulmonary Window from other Diseases

Aortopulmonary Window must be differentiated from other diseases or conditions that cause continuous heart murmur. for further information click Here.

Epidemiology and Demographics

  • Since APW is a very rare congenital heart defect, there is very little known about epidemiology of the disease.

Risk Factors

There are no established risk factors for aortopulmonary window.

Screening

There is insufficient evidence to recommend routine screening for aortopulmonary window.

Natural History, Complications, and Prognosis

  • Clinical features of APW have been described differently due to their classification.
  • Manifestation of the disease is not specific, but majority of patients have a large left to right shunt.
  • patients with small defects may be asymptomatic.
  • If left untreated, large APW may cause symptoms of pulmonary hypertension and congestive heart failure such as tachypnea, diaphoresis, failure to thrive and recurrent respiratory difficulties.
  • pericardium is often hyperdynamic and a mitral valve rumble, causes continues heart murmur.
  • Bounding pulses happen due to decreased diastolic blood pressure secondary to aortic flow reversal in diastole.
  • If the diagnosis is not made until late in infancy or during childhood. APW may lead to eisenmenger syndrome.[6]

Diagnosis

Diagnostic Study of Choice

The diagnosis of aortopulmonary window is made by echocardigraphy after suspicion of large left to right shunt. [7]

History and Symptoms

Physical Examination

Laboratory Findings

An elevated/reduced concentration of serum/blood/urinary/CSF/other [lab test] is diagnostic of [disease name].

OR

Laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

OR

[Test] is usually normal among patients with [disease name].

OR

Some patients with [disease name] may have elevated/reduced concentration of [test], which is usually suggestive of [progression/complication].

OR

There are no diagnostic laboratory findings associated with [disease name].

Electrocardiogram

There are no ECG findings associated with [disease name].

OR

An ECG may be helpful in the diagnosis of [disease name]. Findings on an ECG suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

X-ray

There are no x-ray findings associated with [disease name].

OR

An x-ray may be helpful in the diagnosis of [disease name]. Findings on an x-ray suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no x-ray findings associated with [disease name]. However, an x-ray may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

Echocardiography or Ultrasound

There are no echocardiography/ultrasound findings associated with [disease name].

OR

Echocardiography/ultrasound may be helpful in the diagnosis of [disease name]. Findings on an echocardiography/ultrasound suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no echocardiography/ultrasound findings associated with [disease name]. However, an echocardiography/ultrasound may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

CT scan

There are no CT scan findings associated with [disease name].

OR

[Location] CT scan may be helpful in the diagnosis of [disease name]. Findings on CT scan suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no CT scan findings associated with [disease name]. However, a CT scan may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

MRI

There are no MRI findings associated with [disease name].

OR

[Location] MRI may be helpful in the diagnosis of [disease name]. Findings on MRI suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no MRI findings associated with [disease name]. However, a MRI may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

Other Imaging Findings

There are no other imaging findings associated with [disease name].

OR

[Imaging modality] may be helpful in the diagnosis of [disease name]. Findings on an [imaging modality] suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

Other Diagnostic Studies

There are no other diagnostic studies associated with [disease name].

OR

[Diagnostic study] may be helpful in the diagnosis of [disease name]. Findings suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

Other diagnostic studies for [disease name] include [diagnostic study 1], which demonstrates [finding 1], [finding 2], and [finding 3], and [diagnostic study 2], which demonstrates [finding 1], [finding 2], and [finding 3].

Treatment

Medical Therapy

There is no treatment for [disease name]; the mainstay of therapy is supportive care.

OR

Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].

OR

The majority of cases of [disease name] are self-limited and require only supportive care.

OR

[Disease name] is a medical emergency and requires prompt treatment.

OR

The mainstay of treatment for [disease name] is [therapy].

OR   The optimal therapy for [malignancy name] depends on the stage at diagnosis.

OR

[Therapy] is recommended among all patients who develop [disease name].

OR

Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].

OR

Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].

OR

Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].

OR

Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].

Surgery

Surgical intervention is not recommended for the management of [disease name].

OR

Surgery is not the first-line treatment option for patients with [disease name]. Surgery is usually reserved for patients with either [indication 1], [indication 2], and [indication 3]

OR

The mainstay of treatment for [disease name] is medical therapy. Surgery is usually reserved for patients with either [indication 1], [indication 2], and/or [indication 3].

OR

The feasibility of surgery depends on the stage of [malignancy] at diagnosis.

OR

Surgery is the mainstay of treatment for [disease or malignancy].

Primary Prevention

There are no established measures for the primary prevention of [disease name].

OR

There are no available vaccines against [disease name].

OR

Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

OR

[Vaccine name] vaccine is recommended for [patient population] to prevent [disease name]. Other primary prevention strategies include [strategy 1], [strategy 2], and [strategy 3].

Secondary Prevention

There are no established measures for the secondary prevention of [disease name].

OR

Effective measures for the secondary prevention of [disease name] include [strategy 1], [strategy 2], and [strategy 3].

References

  1. ↑ Kouchoukos, Nicholas (2013). Kirklin/Barratt-Boyes cardiac surgery : morphology, diagnostic criteria, natural history, techniques, results, and indications. Philadelphia: Elsevier/Saunders. ISBN 978-1-4160-6391-9.
  2. ↑ Tkebuchava, T (1997). "Congenital aortopulmonary window: diagnosis, surgical technique and long-term results". European Journal of Cardio-Thoracic Surgery. 11 (2): 293–297. doi:10.1016/S1010-7940(96)01048-2. ISSN 1010-7940.
  3. ↑ Demir, Ibrahim Halil; Erdem, Abdullah; Saritas, Turkay; Demir, Fadli; Erol, Nurdan; Yucel, Ilker Kemal; Aydemir, Numan Ali; Celebi, Ahmet (2013). "Diagnosis, Treatment and Outcomes of Patients with Aortopulmonary Window". Balkan Medical Journal. 30 (2): 191–196. doi:10.5152/balkanmedj.2013.6995. ISSN 2146-3123.
  4. ↑ McElhinney, Doff B; Reddy, V.Mohan; Tworetzky, Wayne; Silverman, Norman H; Hanley, Frank L (1998). "Early and Late Results After Repair of Aortopulmonary Septal Defect and Associated Anomalies in Infants < 6 Months of Age". The American Journal of Cardiology. 81 (2): 195–201. doi:10.1016/S0002-9149(97)00881-3. ISSN 0002-9149.
  5. ↑ Aurigemma, David; Dixon, Chandler; Tucker, Suzanne; Davis, Christopher; Silverman, Norman (2018). "Aortopulmonary window in tetralogy of Fallot with absent conal septum". Echocardiography. 36 (2): 411–414. doi:10.1111/echo.14243. ISSN 0742-2822.
  6. ↑ Myers, Patrick O.; Lador, Frédéric; Hachulla, Anne-Lise; Bouchardy, Judith; Noble, Stéphane; Licker, Marc; Pache, Jean-Claude; Kalimanovaska-Ostric, Dimitra; Djukic, Milan; Kalangos, Afksendiyos; Beghetti, Maurice (2016). "Unrestrictive Aortopulmonary Window". Circulation. 133 (19): 1907–1910. doi:10.1161/CIRCULATIONAHA.115.020819. ISSN 0009-7322.
  7. ↑ Zhao, Dong; Yang, Keming; Li, Shoujun; Yan, Jun; Hua, Zhongdong; Fang, Nengxin; Su, Wenjun; Lv, Xiaodong; Yu, Bing (2019). "Outcomes of different rehabilitative procedures in patients with pulmonary atresia, ventricular septal defect and major aortopulmonary collateral arteries". European Journal of Cardio-Thoracic Surgery. 55 (5): 837–844. doi:10.1093/ejcts/ezy375. ISSN 1010-7940.


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