Aortopulmonary Window: Classification, Associated Cardiac Anomalies, Treatment Options, and Clinical Outcome
Objective: Aortopulmonary window (APW) is an uncommon congenital cardiac abnormality marked by a septation defect between the ascending aorta and pulmonary artery. This study aimed to define the clinical characteristics, diagnostic features, treatment strategies, and follow-up outcomes of pediatric...
| Main Authors: | , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Galenos Publishing House
2023-08-01
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| Series: | Cam & Sakura Medical Journal |
| Subjects: | |
| Online Access: | https://jag.journalagent.com/z4/download_fulltext.asp?pdir=csmedj&un=CSM-00719 |
| _version_ | 1797633682931974144 |
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| author | Fatma Sevinç Şengül Perver Arslan Pelin Ayyıldız Erkut Öztürk İbrahim Cansaran Tanıdır Okan Yıldız Sertaç Haydin Alper Güzeltaş |
| author_facet | Fatma Sevinç Şengül Perver Arslan Pelin Ayyıldız Erkut Öztürk İbrahim Cansaran Tanıdır Okan Yıldız Sertaç Haydin Alper Güzeltaş |
| author_sort | Fatma Sevinç Şengül |
| collection | DOAJ |
| description | Objective: Aortopulmonary window (APW) is an uncommon congenital cardiac abnormality marked by a septation defect between the ascending aorta and pulmonary artery. This study aimed to define the clinical characteristics, diagnostic features, treatment strategies, and follow-up outcomes of pediatric patients diagnosed with APW.
Material and Methods: We retrospectively reviewed children diagnosed with APW from 2010 to 2023. Morphological APW typing of our patients was based on the classification that is settled by the Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database Committee. The patients' demographic data, symptoms at admission, transthoracic echocardiography, cardiac computed tomography, management modalities, and follow-up data were evaluated.
Results: Twenty-five children were diagnosed with APW over the study period. Thirteen patients were male (52%), and the median age at presentation of the patients was three months (8 days-7.5 years). Two patients were diagnosed with coronary fistula by echocardiography at the first admission and were diagnosed with APW after catheterization. APW was detected in one patient while being operated on for large ventricular septal defect. According to the STS classification, 32% (n=8) of the patients were type III, 32% (n=8) were type I, 16% (n=4) were intermediate type, 12% (n=3) were type II, and 4% (n=1) were APW with aortic interruption. Associated cardiovascular malformations were in 76% (n=19) of the patients. Fifteen patients (60%) underwent surgery. Transcatheter closure of APW was performed in four patients (16%).
Conclusion: Detection of the APW requires careful and systematic investigation. Transcatheter closure can be performed in selected cases where the defect is suitable. Although rare, this defect, which can cause severe left-right shunting, should be kept in mind as a cause of pulmonary hypertension and unexplained cardiac dilation and should be investigated in patients whose cause cannot be determined. |
| first_indexed | 2024-03-11T11:57:20Z |
| format | Article |
| id | doaj.art-f4dad783e0fe4a5bb83162610714c6b6 |
| institution | Directory Open Access Journal |
| issn | 2791-8823 |
| language | English |
| last_indexed | 2024-03-11T11:57:20Z |
| publishDate | 2023-08-01 |
| publisher | Galenos Publishing House |
| record_format | Article |
| series | Cam & Sakura Medical Journal |
| spelling | doaj.art-f4dad783e0fe4a5bb83162610714c6b62023-11-08T13:00:17ZengGalenos Publishing HouseCam & Sakura Medical Journal2791-88232023-08-0132465210.4274/csmedj.galenos.2023.2023-5-1CSM-00719Aortopulmonary Window: Classification, Associated Cardiac Anomalies, Treatment Options, and Clinical OutcomeFatma Sevinç Şengül0Perver Arslan1Pelin Ayyıldız2Erkut Öztürk3İbrahim Cansaran Tanıdır4Okan Yıldız5Sertaç Haydin6Alper Güzeltaş7University of Health Sciences Turkey, İstanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital, Clinic of Pediatric Cardiology, İstanbul, TurkeyUniversity of Health Sciences Turkey, İstanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital, Clinic of Pediatric Cardiology, İstanbul, TurkeyUniversity of Health Sciences Turkey, İstanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital, Clinic of Pediatric Cardiology, İstanbul, TurkeyUniversity of Health Sciences Turkey, Başakşehir Çam and Sakura City Hospital, Clinic of Pediatric Cardiology, İstanbul, TurkeyUniversity of Health Sciences Turkey, Başakşehir Çam and Sakura City Hospital, Clinic of Pediatric Cardiology, İstanbul, TurkeyUniversity of Health Sciences Turkey, İstanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital, Clinic of Pediatric Cardiovascular Surgery, İstanbul, TurkeyUniversity of Health Sciences Turkey, İstanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital, Clinic of Pediatric Cardiovascular Surgery, İstanbul, TurkeyUniversity of Health Sciences Turkey, İstanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital, Clinic of Pediatric Cardiology, İstanbul, TurkeyObjective: Aortopulmonary window (APW) is an uncommon congenital cardiac abnormality marked by a septation defect between the ascending aorta and pulmonary artery. This study aimed to define the clinical characteristics, diagnostic features, treatment strategies, and follow-up outcomes of pediatric patients diagnosed with APW. Material and Methods: We retrospectively reviewed children diagnosed with APW from 2010 to 2023. Morphological APW typing of our patients was based on the classification that is settled by the Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database Committee. The patients' demographic data, symptoms at admission, transthoracic echocardiography, cardiac computed tomography, management modalities, and follow-up data were evaluated. Results: Twenty-five children were diagnosed with APW over the study period. Thirteen patients were male (52%), and the median age at presentation of the patients was three months (8 days-7.5 years). Two patients were diagnosed with coronary fistula by echocardiography at the first admission and were diagnosed with APW after catheterization. APW was detected in one patient while being operated on for large ventricular septal defect. According to the STS classification, 32% (n=8) of the patients were type III, 32% (n=8) were type I, 16% (n=4) were intermediate type, 12% (n=3) were type II, and 4% (n=1) were APW with aortic interruption. Associated cardiovascular malformations were in 76% (n=19) of the patients. Fifteen patients (60%) underwent surgery. Transcatheter closure of APW was performed in four patients (16%). Conclusion: Detection of the APW requires careful and systematic investigation. Transcatheter closure can be performed in selected cases where the defect is suitable. Although rare, this defect, which can cause severe left-right shunting, should be kept in mind as a cause of pulmonary hypertension and unexplained cardiac dilation and should be investigated in patients whose cause cannot be determined.https://jag.journalagent.com/z4/download_fulltext.asp?pdir=csmedj&un=CSM-00719aortopulmonary windowechocardiographypulmonary hypertensionsurgical treatmenttranscatheter treatment |
| spellingShingle | Fatma Sevinç Şengül Perver Arslan Pelin Ayyıldız Erkut Öztürk İbrahim Cansaran Tanıdır Okan Yıldız Sertaç Haydin Alper Güzeltaş Aortopulmonary Window: Classification, Associated Cardiac Anomalies, Treatment Options, and Clinical Outcome Cam & Sakura Medical Journal aortopulmonary window echocardiography pulmonary hypertension surgical treatment transcatheter treatment |
| title | Aortopulmonary Window: Classification, Associated Cardiac Anomalies, Treatment Options, and Clinical Outcome |
| title_full | Aortopulmonary Window: Classification, Associated Cardiac Anomalies, Treatment Options, and Clinical Outcome |
| title_fullStr | Aortopulmonary Window: Classification, Associated Cardiac Anomalies, Treatment Options, and Clinical Outcome |
| title_full_unstemmed | Aortopulmonary Window: Classification, Associated Cardiac Anomalies, Treatment Options, and Clinical Outcome |
| title_short | Aortopulmonary Window: Classification, Associated Cardiac Anomalies, Treatment Options, and Clinical Outcome |
| title_sort | aortopulmonary window classification associated cardiac anomalies treatment options and clinical outcome |
| topic | aortopulmonary window echocardiography pulmonary hypertension surgical treatment transcatheter treatment |
| url | https://jag.journalagent.com/z4/download_fulltext.asp?pdir=csmedj&un=CSM-00719 |
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