Coexistence Of Interrupted Aortic Arch With Aortopulmonary Window Detected On MDCT In A 9 Year Old Male Child
A Joshi, S Gujare, S Merchant, A Sankhe
ap window, interrupted aortic arch, mdct
A Joshi, S Gujare, S Merchant, A Sankhe. Coexistence Of Interrupted Aortic Arch With Aortopulmonary Window Detected On MDCT In A 9 Year Old Male Child. The Internet Journal of Pediatrics and Neonatology. 2007 Volume 8 Number 1.
Interrupted aortic arch is a congenital malformation of the aortic arch that occurs in 3 per million live births
With advent of Multidetector Computed tomography (MDCT), utility of CT in evaluation of cardiac pathology has gained importance with supplementary role in the evaluation of patients with congenital heart disease
We report a case of 9 years old child with cyanosis, radio-femoral delay and claudication. Contrast CT chest was performed on MDCT that showed co-existence of Type A Interrupted aortic arch with large Aortopulmonary window.
A 9 year old male child presented with generalized cyanosis, radio-femoral delay and lower limb claudication. On 2D Echocardiography the patient was diagnosed as coarctation of aorta with changes of pulmonary hypertension and pulmonary regurgitation. The child was referred to the CT scan to confirm the same and to look for any ancillary finding. Plain and contrast CT scan of chest was performed on Multidetector CT scanner [Siemens Somatom MDCT] by injecting 100 cc of nonionic contrast at the rate of 2.5 ml/ sec with 1.25mm collimation, which were use to generate 1.25 mm axial CT scan slice. These axial slices were utilized for reconstructions in various planes.
The CT angiography revealed normal aortic root, normal origins of both the coronary arteries, with 2.5 cms of normal ascending aorta. A 3 cms sized large aorta pulmonary window was seen between the ascending aorta and main pulmonary artery, beyond which normal 1 cms of ascending aorta and proximal arch of aorta was seen. The three arch branches namely innominate artery, left common carotid and subclavian arteries originated normally from the arch, but in close proximity to each other. Beyond the origin of left subclavian artery the arch of aorta was interrupted. The descending aorta was seen in higher CT scan sections arising or rather forming from the distal portion of main pulmonary artery (MPA). Thus a diagnosis of a large AP window associated with interruption of arch of aorta was made.
Interrupted aortic arch and AP window individually are rare congenital cardiac anomalies and we present an even rarer condition in which both these pathologies showed co-existence.
The classification of arch interruptions 4 is based on the position of the interruption relative to the brachiocephalic vessels. It consists of three major types,
The associated anomalies seen with Interrupted aortic arch are DiGeorge syndrome (in Type B: 10-50%)5, associated abnormalities of the aortic arch (aberrant or isolated left or right subclavian arteries), intracardiac anomalies (96%)6,7 including VSD, patent ductus arteriosus, left ventricular outflow obstruction (subaortic stenosis or bicuspid aortic valve), truncus arteriosus, CHARGE syndrome (
When an APW occurs, the aortic and the pulmonary valves are usually normally formed.
The clinical presentation in AP window is related to its haemodynamics of a significant left to right shunt. In early life, patients present with signs and symptoms of congestive heart failure, failure to thrive and propensity for frequent chest infections 2. The extent and severity depend on the amount of left to right flow, which is in turn is dependent on the size of the defect. Elevation of pulmonary pressure results in development of pulmonary hypertension. Physical findings may include a widened pulse pressure, praecordial heave, and a systolic or continuous heart murmur.
Whereas the Interrupted aortic arch infants do well right after birth but develop symptoms of congestive heart failure such as rapid breathing, clammy sweating, and poor feeding during the first week of life 9 .If the condition is left untreated, 90% of the affected infants succumb at a median age of 4 days 10.
In this patient the 2D echo findings were possibly interpreted due to the MPA and descending aorta forming a narrow arch, thus simulating a coarctation.
The rarity in our case lies in the co-existence of two rare congenital cardiac anomalies i.e. Type A Interrupted aortic arch and Aortopulmonary window with a delayed age of presentation i.e. 9 years.
CT is a useful imaging modality for the morphologic evaluation of CHD. Reformatted images from MDCT can accurately and systematically delineate the normal and pathologic morphologic features of the cardiovascular structures.
Dr. Ashwini Sankhe, Address: 7b /304 Accolade CHS, Louiswadi, Hajuri dargah rd, Thane (west)-401604, State: Maharashtra, India Email id: firstname.lastname@example.org