JBR–BTR, 2014, 97: 356-357.

AORTOPULMONARY WINDOW: A RARE CONGENITAL DISEASE

T.J. Costa1, N. Damry1, C. Jacquemart 2, C. Christophe1

We report the case of a 3,5 month-old infant presented at cardiology consultation of our institution for investigation of a heart murmur associated with failure to thrive and respiratory distress. Echocardiogram showed dilatation of left heart cavities, patent foramen ovale, small muscular ventricular septal defect and . Angio-CT was performed on a 64-slice CT to better characterize the congenital heart disease. Aortopulmonary ­window was diagnosed and surgically corrected. Patient evolution was good with discharge 10 days after surgery. The authors review the literature and stress the importance of angio-CT in pre-operative evaluation.

Key-word: Infants, cardiovascular system.

Introduction

A 3.5-month-old infant presented at cardiology consultation for explo- ration of a heart murmur and failure to thrive. The patient was examined * the first time when he was 3-month- old and presented with dyspnea and tachypnea with infra-costal retrac- tion. Physical examination in our institution revealed a clinical picture that included poor general health, a systolic-diastolic murmur 4/6 and respiratory distress. ECG showed sinus rhythm and signs of biventric- A B ular hypertrophy. Echocardiogram detected dilatation of left heart cavi- ties with left ventricle hyper-contrac- tility, a patent foramen ovale, a small muscular ventricular septal defect and pulmonary hypertension. An angio-CT was performed for pre-operative evaluation of congeni- * tal heart disease. The exam was realized in a 64-slice CT with a non ECG-synchronized spiral acquisition (tube voltage: 80 kV; tube current: 63 mAs), after manual non-ionic contrast injection of 2-ml/kg-body weight (IOMERON 400). MIP reconstructions images were C D obtained in the axial (Fig. 1A, B, C) and coronal (D, E) planes, showing a large communication (window) between ascending aorta and pul- monary artery in proximity to the semilunar valves. VRT reconstruc- tion image (F) shows the very anteri- * * or position of the defect between the arterial trunks. Based on the radio- logical findings, the diagnosis of an aortopulmonary window type I was made. The large defect was localized anteriorly in the proximal part of the aortopulmonary septum in the pres- ence of two independent semilunar valves. Coronary arteries origin was E F Fig. 1. — MIP reconstruction images in axial (A, B, C) and coronal planes (D, E) and VRT reconstruction image (F). In this case, the aortopulmonary window defect From: 1. Department of Radiology, 2. Department of Cardiology, Hôpital Universitaire occurs in an anterior and proximal Des Enfants Reine Fabiola, Brussels, Belgium. position (*). Coronary arteries origin is Address for correspondence: Dr T.J. Costa, M.D., Avenue Ilya Prigogine, 15/10B, normal. B: left coronary artery (arrow), 1180 Brussels, Belgium. E-mail: [email protected] C: right coronary artery (arrow).

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normal, as showed in images B and septum adjacent to the right pulmo- were prevented by sildenafil admin- C. Vascular pulmonary accentuation nary artery; type III (total) defect is a istration during early hospitalisation. (not show in images) was also pres- combination of types I and II , involv- ent and interpreted as signs of pul- ing the entire length of the pulmo- Conclusion monary arterial hypertension. nary trunk, from immediately above the semilunar valves to the level of This case shows that angio-CT is a Discussion pulmonary bifurcation and the prox- valuable additional imaging method imal portion of the right pulmonary in the evaluation of this congenital Aortopulmonary window was first artery (4). Based on the recent utili- heart disease, allowing a clear visu- described by Eliotson in 1830, as a zation of transcatheter devices to alization of the defect location as communication between the ascend- close the defect, Ho et al. (5), added well as coronary arteries origin. ing aorta and the pulmonary trunk in and additional type to this classifica- Indeed it enables a better planning the presence of separated aortic and tion – type IV for intermediate defect of surgical approach. pulmonary valves. Robert E. Gross, with adequate superior and inferior The patient was surgically treated MD, at Boston Children’s Hospital, rims. and could be discharged 10 days reported the first successful surgical Aortopulmonary window produces after surgery with no signs of pulmo- repair in 1952 (1). a large and usually unrestricted left- nary hypertension. It is an uncommon entity, repre- to-right shunt that worsens as pul- senting approximately 0.1% of all monary vascular resistance falls congenital heart diseases (2). during the newborn period. Con­ References It may occur as an isolated lesion gestive heart failure and low cardiac and/or associated with minor and output can rapidly follow. These 1. Gross R.E.: Surgical closure of an simple repair anomalies (patent duc- patients are particularly susceptible aortic septal defect. Circulation, 1952, tus arteriosus, , to Eisenmenger’s syndrome at an 5: 858-863. patent foramen ovale) or as part of a early age because of combined sys- 2. Jacobs J.P., Quintessenza J.A., larger complex of lesions such as tolic and diastolic run-off into the Gaynor J.W., Burke R.P., Mavroudis C.: Congenital Heart , pulmonary atre- pulmonary circulation. Surgery Nomenclature and Database sia, aortic arch interruption, aortic Besides clinical suspicion of con- Project: aortopulmonary window. arch hypoplasia with or without genital heart disease and frequently Ann Thorac Surg, 2000, 69: S44- coarctation, and anomalous origin of ECG findings of biventricular hyper- S49. coronary arteries. Fifty per cent of trophy, echocardiography can sus- 3. Barnes M.E., Mitchell M.E., patients usually have no other heart pect or do the diagnosis by showing Tweddell J.S.: Aortopulmonary win- defects. the “T sign” in the presence of two dow. Semin Thorac Cardiovasc Surg The defect between the great ves- normal semilunar valves, downward Pediatr Card Surg Annu, 2011, 14: sels results from failure of the two flow through the right side of main 67-74. embryonic conotruncal ridges to pulmonary artery and diastolic flow 4. Mori K., Ando M., Takao A., Ishikawa S., Imai Y.: Distal type of fuse. It is separate from truncus arte- reversal in both aortic arch and Aortopulmonary window: report of riosus because it occurs in the pres- descending aorta. 4 cases. British Heart Journal, 1978, ence of essentially normal aortic and However angio-CT gives addition- 40: 681-689. pulmonary valves. The “window” al anatomic information such as the 5. Ho S.Y., Gerlis L.M., Anderson C., usually begins just above the sinus- precise location of the defect as well Devine W.A., Smith A.: The morphol- es of Valsalva and then extends into as associated coronary origin anom- ogy of aortopulmonary window with a variable distance distally into the alies. In our case DLP was 24 mGy. regard to their classification and mor- arch (3). cm, CTDI vol. 1.30 mGy and calculat- phogenesis. Cardiol Young, 1994, 4: Aortopulmonary window can be ed effective dose was 2.0 mSv. 146-155. classified into 3 types, according to The patient was surgically treated 6. Corno A.F., Festa P.: Aortopulmonary window. In: Congenital Heart Defects Mori’s classification: type I (proxi- and post-surgical evolution was good Decision Making for Cardiac Surgery, mal) defects occur in the proximal with just a transitory episode of Vol. 3 CT-Scan and MRI. Edited by part of aortopulmonary septum; myocardial dysfunction at Intensive Gasser A., Steinkopff Verlag Springer. type II (distal) defects occur in the Care Unit treated by dobutamine. Printed by Stürtz GmbH, Würzburg, distal part of the aortopulmonary Pulmonary hypertension episodes 2009, pp 169-172.

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