1 year-old boy with congenital heart disease

 

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Doctor's Information

Name : Abbas
Family :Arjmand Shabestari
Affiliation : Radiology Department, Modarres Hospital, SBMU
Academic Degree: Associate Professor of Radiology
Email : This email address is being protected from spambots. You need JavaScript enabled to view it.
Resident : Ramin Pourghorban

 

Case Section

Cardiovascular

 

Patient's Information

Gender : Male
Age : 1

 

Clinical Summary

1 year-old boy with congenital heart disease

 

 

Imaging Findings

Contrast-enhanced CT scans show a large defect (double arrows in Figs. 9-10) between the ascending aorta and the main pulmonary artery, consistent with aorto-pulmonary window.

 

Differential Diagnosis

Aorto-Pulmonary Window, PDA, Truncus Arteriosus

 

Final Diagnosis

Aorto-Pulmonary Window

 

Discussion (Related Text)

Aortopulmonary window, or aortopulmonary septal defect, is a relatively rare cardiac malformation. Since first described by Elliotson just over 300 cases have been reported. It accounts for 0.2% to 0.6% of all cases of congenital heart disease. Nearly half of all patients have associated cardiac lesions, including aortic origin of the right pulmonary artery, type A interruption of the aortic arch, tetralogy of Fallot, and anomalous origin of the right or left coronary artery from the pulmonary artery and right aortic arch. More rarely, it is associated with ventricular septal defect, pulmonary or aortic atresia, d-transposition, and tricuspid atresia. Diagnosis is usually suggested by echocardiographic findings and confirmed with angiography. The chest roentgenogram is indicative of a large left-to-right shunt. A moderately enlarged heart with prominent pulmonary vascular markings is usually present. The main pulmonary artery segment is usually pronounced, as are the left atrial and left ventricular borders. The aortic knuckle usually is not prominent. The lung fields frequently show hyperinflation, and pulmonary edema may be present. Although the role of MR imaging in the diagnosis of aortopulmonary window has not been investigated extensively, a number of published reports indicate that MR imaging can fully depict the anatomic features of aortopulmonary window. Transverse and coronal spin-echo images can depict the abnormal connection between the aorta and the pulmonary trunk, and cine images can reveal a left-to-right shunt. MR images also may be useful for quantifying shunt volume.

 

References

1. Wang ZJ, Reddy GP, Gotway MB, Yeh B, Higgins CB. Cardiovascular shunts: MR imaging evaluation. Radiographics 2003;23:S181??S194.

2. Allen HD, Driscoll DJ, Shaddy RE, Feltes FF: Moss and Adams' Heart Disease in Infants, Children, and Adolescents: Including the Fetus and Young Adults, 7th ed. Philadelphia: Lippincott Williams & Wilkins, 2008, PP696-699.

 

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