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Diagnosis, treatment and outcomes of patients with aortopulmonary window
Ibrahim Halil Demir1, Abdullah Erdem1, Türkay Sarıtaş1
1Department of Pediatric Cardiology, Dr. Siyami Ersek Thoracic and Cardiovascular Surgery Training and Research Hospital, İstanbul, Turkey.
Insights
Aortopulmonary window (APW) is a rare heart defect. Surgical and transcatheter repairs show excellent outcomes, with most patients remaining asymptomatic post-treatment.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- Aortopulmonary window (APW) is an exceptionally rare congenital heart defect characterized by a communication between the ascending aorta and pulmonary artery.
- It is the rarest form of septal defects, often presenting diagnostic and therapeutic challenges.
Purpose of the Study:
- To present the clinical experience with the diagnosis and outcomes of patients with aortopulmonary window (APW).
Main Methods:
- A retrospective cohort study reviewed 13 patients diagnosed with APW between June 2003 and October 2011.
- Data collected included clinical features, echocardiographic and angiographic findings, surgical interventions, and follow-up outcomes.
Main Results:
- Eleven children underwent surgical correction, and one 12-month-old boy had a transcatheter repair for APW.
- Associated defects like VSD and interrupted aortic arch were noted in some patients.
- No mortality occurred due to procedures; most patients were asymptomatic at a median 40-month follow-up.
Conclusions:
- APW should be considered in infants with congestive heart failure and failure to thrive.
- Echocardiography is sufficient for diagnosing isolated APW in infants under 6 months.
- Cardiac catheterization is valuable for complex cases and for vasoreactivity testing and potential defect closure in older infants.
Background:
Aortopulmonary window (APW) is a communication between the ascending aorta and the pulmonary artery in the presence of two separate semilunar valves and is the rarest of septal defects.
Aims:
To present our experience with the diagnosis and outcome of APW cases.
Study Design:
Retrospective cohort study.
Methods:
Between June 2003 and October 2011, thirteen patients were diagnosed with APW. Clinical features of patients, findings of echocardiographic and angiographic examination, results of surgical intervention and follow-up were reviewed retrospectively.
Results:
Eleven children (10 days to 16 years), underwent surgical correction of APW. In a 12-month-old boy, the defect was repaired by the transcatheter approach. In addition to APW repair, closure of VSD was performed in 2 patients. APW were associated with interruption in two patients; one also had a complex pathology. None of the patients died due to complications of surgical or transcatheter procedures. After a median follow-up period of 40 months, the patients were asymptomatic and none of them required additional medication, except for the patient with complex pathology including an interrupted aortic arch, who underwent balloon angioplasty for recoarctation.
Conclusion:
In any infant with the findings of congestive heart failure and failure to thrive, APW must be kept in mind as a differential diagnosis. In isolated APW cases before 6 months of age, echocardiography is often sufficient for diagnosis. In complex cases, cardiac catheterisation is performed for the comprehensive evaluation of associated defects. After 6 months, cardiac catheterisation could be utilised to perform vasoreactivity testing and, if possible, to close the defect.
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