Aortopulmonary window
| Aortopulmonary window | |
|---|---|
| Other names | Aortopulmonary septal defect ECG, and imaging.[2] |
| Differential diagnosis | Truncus arteriosus, ventricular septal defect, and patent ductus arteriosus.[3] |
| Treatment | Heart surgery.[2] |
| Prognosis | 40% chance of death within the first year if left untreated.[4] |
| Frequency | 0.15-0.6% of all congenital heart malformations.[4] |
Aortopulmonary window (APW) is a faulty connection between the
Signs and symptoms
A
Findings among individuals with an isolated aortopulmonary window vary based on the size of the defect and the
When the defect is larger, pulmonary vascular resistance may continue to be elevated in the initial weeks or months of life, and there is a modest amount of pulmonary overcirculation. A rather faint basal systolic ejection murmur without a diastolic element and a loud single second heart sound develop due to mild overcirculation. As the pulmonary vascular resistance decreases throughout the first few months, the left-to-right shunting of blood into the lungs increases, and the systolic murmur becomes more intense and longer, eventually extending into diastole and becoming a continuous murmur.[2]
Complications
Because there is a pressure gradient from the aorta to the pulmonary artery throughout systole and diastole, a small aortopulmonary defect may produce a murmur similar to a patent ductus arteriosus.[2]
When a large defect is left unrepaired, Eisenmenger syndrome will develop with reversal of the shunt.[2]
Untreated cases with major malformations have a poor prognosis, with 40% dying within their first year of life.[4]
Diagnosis
Physical examination findings, ECG, and imaging are used to diagnose aortopulmonary window. An electrocardiogram reveals right ventricular hypertrophy or biventricular hypertrophy. Cardiomegaly, a big main pulmonary artery segment, and enhanced pulmonary vascular marking are all visible on a chest x-ray.[2]
Differential diagnosis
The appearance of aortopulmonary window has similarities to that of
Treatment
Corrective heart surgery, which is normally performed in the first year of life, is the definitive intervention for an aortopulmonary window. If the patient's symptoms don't allow for corrective surgery, medical therapy of
See also
- Aortic window
- Major aortopulmonary collateral artery, develops when native pulmonary circulation is underdeveloped
References
- ^ "Aortopulmonary window: MedlinePlus Medical Encyclopedia". medlineplus.gov. Retrieved 27 May 2019.
- ^ a b c d e f g h i j k l Beerman, Lee B. (April 4, 2023). "Aortopulmonary Window — Pediatrics". Merck Manuals Professional Edition. Retrieved October 4, 2023.
- ^ PMID 32809451. Retrieved October 4, 2023.
- ^ PMC 4115960.