Aortopulmonary Window: Difference between revisions
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* 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> | * 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== | ||
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* pericardium is often hyperdynamic and a mitral valve rumble, causes continues heart murmur. | * 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. | * Bounding pulses happen due to decreased diastolic blood pressure secondary to aortic flow reversal in diastole. | ||
<ref name="Aortopulmonary Septal Defect">{{Cite journal| author = [[Mark A.. Law]] & [[Kunal Mahajan]]|title = Aortopulmonary Septal Defect|year = 2020|month = January|pmid = 28723032}}</ref> | <ref name="Aortopulmonary Septal Defect">{{Cite journal| author = [[Mark A.. Law]] & [[Kunal Mahajan]]|title = Aortopulmonary Septal Defect|year = 2020|month = January|pmid = 28723032}}</ref> | ||
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===Laboratory Findings=== | ===Laboratory Findings=== | ||
There are no specific diagnostic laboratory findings associated with aortopulmonary window.. | |||
There are no diagnostic laboratory findings associated with | |||
===Electrocardiogram=== | ===Electrocardiogram=== | ||
* [[Tachycardia]] | |||
* Increased right and left sided voltage | |||
===X-ray=== | ===X-ray=== | ||
* Cardiomegaly | |||
* Increased pulmonary vascular markings | |||
===Echocardiography or Ultrasound=== | ===Echocardiography or Ultrasound=== | ||
* Gold standard foe diagnosis of aortopulmonary window. | |||
* Since the connection is usually without significant restriction, color-Doppler echocardiography will not demonstrate high velocity jet. | |||
===CT scan=== | ===CT scan=== | ||
Revision as of 20:08, 5 April 2020
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.[7]
Diagnosis
Diagnostic Study of Choice
The diagnosis of aortopulmonary window is made by echocardigraphy after suspicion of large left to right shunt. [8]
History and Symptoms
- tachypnea
- diaphoresis
- failure to thrive
- recurrent respiratory difficulties
Physical Examination
- continuous murmur
- If late eisenmenger's syndrome:
Laboratory Findings
There are no specific diagnostic laboratory findings associated with aortopulmonary window..
Electrocardiogram
- Tachycardia
- Increased right and left sided voltage
X-ray
- Cardiomegaly
- Increased pulmonary vascular markings
Echocardiography or Ultrasound
- Gold standard foe diagnosis of aortopulmonary window.
- Since the connection is usually without significant restriction, color-Doppler echocardiography will not demonstrate high velocity jet.
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
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ Mark A.. Law & Kunal Mahajan (2020). "Aortopulmonary Septal Defect". PMID 28723032. Unknown parameter
|month=ignored (help) - ↑ 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.
- ↑ 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.