Answer for BIR CoW 30 March 2025
Aorto pulmonary septal defect- TYPE III with pulmonary hypertension and mediastinal collaterals.
Findings
Cardiomegaly .Evidence of large defect measuring~ 3.2x4cm, seen connecting the ascending aorta and Main pulmonary trunk, defect seen 1.9 cm distal to aortic annulus. Aortic valve and pulmonary valve separately visualized. Main pulmonary artery dilated, measures 34 mm . Right atrium appears enlarged. Right & left ventricle hypertrophy seen. Multiple tiny tortuous mediastinal collaterals arising from descending thoracic aorta.
Discussion
Aortopulmonary septal defect (APSD) is a congenital anomaly where there is an abnormal communication between the proximal aorta and the pulmonary trunk in the presence of separate aortic and pulmonary valves. It is a rare anomaly occur in <1% of all cardiac malformations . 50-70% of all patients with APSD are associated with other cardiac malformations.
PATHOLOGY: Aortopulmonary septal defect results when there is a failure of fusion of the two opposing conotruncal ridges that are responsible for separating the truncus arteriosus into the aorta and pulmonary artery. The defect occurs between the ascending aorta and the main pulmonary artery and may be found just above the semilunar valves or between the more distal ascending aorta and main pulmonary artery.
CLASSIFICATION: This classification separates APSD into four types 3: type I: proximal APSD located just above the sinus of Valsalva, a few millimeters above the semilunar valve type II: distal APSD located in the uppermost portion of the ascending aorta type III: total defect involving the entire aortopulmonary septum or ascending aorta type IV: intermediate defect
IMAGING FEATURES:
X RAY: Cardiomegaly with pulmonary plethora.
CT ANGIOGRAPHY: Shows direct visualization of the defect and associated anomalous anatomy.
COMPLICATIONS: Pulmonary Hypertension. Eisenmenger syndrome. Congestive cardiac failure.
REFERENCES:
Samánek M, Vorísková M. Congenital heart disease among 815,569 children born between 1980 and 1990 and their 15-year survival: A prospective Bohemia survival study. Pediatr Cardiol. 1999;20:411–17. doi: 10.1007/s002469900502. [DOI] [PubMed] [Google Scholar] 2.Law MA, Mahajan K. StatPearls [Internet] Treasure Island (FL): StatPearls Publishing; 2023. Jan 21, 2023. Aortopulmonary Septal Defect. [Google Scholar]
Findings
Cardiomegaly .Evidence of large defect measuring~ 3.2x4cm, seen connecting the ascending aorta and Main pulmonary trunk, defect seen 1.9 cm distal to aortic annulus. Aortic valve and pulmonary valve separately visualized. Main pulmonary artery dilated, measures 34 mm . Right atrium appears enlarged. Right & left ventricle hypertrophy seen. Multiple tiny tortuous mediastinal collaterals arising from descending thoracic aorta.
Discussion
Aortopulmonary septal defect (APSD) is a congenital anomaly where there is an abnormal communication between the proximal aorta and the pulmonary trunk in the presence of separate aortic and pulmonary valves. It is a rare anomaly occur in <1% of all cardiac malformations . 50-70% of all patients with APSD are associated with other cardiac malformations.
PATHOLOGY: Aortopulmonary septal defect results when there is a failure of fusion of the two opposing conotruncal ridges that are responsible for separating the truncus arteriosus into the aorta and pulmonary artery. The defect occurs between the ascending aorta and the main pulmonary artery and may be found just above the semilunar valves or between the more distal ascending aorta and main pulmonary artery.
CLASSIFICATION: This classification separates APSD into four types 3: type I: proximal APSD located just above the sinus of Valsalva, a few millimeters above the semilunar valve type II: distal APSD located in the uppermost portion of the ascending aorta type III: total defect involving the entire aortopulmonary septum or ascending aorta type IV: intermediate defect
IMAGING FEATURES:
X RAY: Cardiomegaly with pulmonary plethora.
CT ANGIOGRAPHY: Shows direct visualization of the defect and associated anomalous anatomy.
COMPLICATIONS: Pulmonary Hypertension. Eisenmenger syndrome. Congestive cardiac failure.
REFERENCES:
Samánek M, Vorísková M. Congenital heart disease among 815,569 children born between 1980 and 1990 and their 15-year survival: A prospective Bohemia survival study. Pediatr Cardiol. 1999;20:411–17. doi: 10.1007/s002469900502. [DOI] [PubMed] [Google Scholar] 2.Law MA, Mahajan K. StatPearls [Internet] Treasure Island (FL): StatPearls Publishing; 2023. Jan 21, 2023. Aortopulmonary Septal Defect. [Google Scholar]
Note:
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!