Year : 2014  |  Volume : 17  |  Issue : 3  |  Page : 245--246

Aortopulmonary window and double outlet right ventricle: A rare combination


Sambhunath Das1, Kalpna Irpachi1, Rajat Kalra2, Balram Airan2,  
1 Department of Cardiac Anaesthesia, All India Institute of Medical Sciences, New Delhi, India
2 Department of Cardiothoracic and Vascular Surgery, All India Institute of Medical Sciences, New Delhi, India

Correspondence Address:
Sambhunath Das
Department of Cardiac Anaesthesia, Room No 7, 7th Floor, Cardiothoracic Centre, AIIMS, Ansari Nagar, New Delhi - 110 029
India




How to cite this article:
Das S, Irpachi K, Kalra R, Airan B. Aortopulmonary window and double outlet right ventricle: A rare combination.Ann Card Anaesth 2014;17:245-246


How to cite this URL:
Das S, Irpachi K, Kalra R, Airan B. Aortopulmonary window and double outlet right ventricle: A rare combination. Ann Card Anaesth [serial online] 2014 [cited 2019 Oct 18 ];17:245-246
Available from: http://www.annals.in/text.asp?2014/17/3/245/135887


Full Text

Aortopulmonary window combined with double outlet right ventricle (DORV) is a very rare congenital cardiac anomaly. We managed a 5-month-old child weighing 4.5 kg diagnosed as a case of aortopulmoary window (APW) combined with DORV with ostium secundum atrial septal defect (ASD), and bilateral confluent and adequate pulmonary arteries. The surgical correction was planned under general anesthesia. Transesophageal echocardiography (TEE; Philips pediatric S7-3t) in the upper esophageal position with slight anticlockwise rotation detected a communication between ascending aorta and main pulmonary artery [Figure 1]. The TEE images of APW with DORV are shown in [Figure 1] and [Figure 2]. Aortopulmonary window was arising approximately 1.0 cm above the aortic valve annulus and its diameter measured approximately 10 mm [Figure 3]. The approximate size of the pulmonary artery and aorta was 13.5 and 12 mm. Under cardiopulmonary bypass, the APW was dissected and transected. Both the openings on ascending aorta and pulmonary artery were closed by continuous suture. The DORV and ASD were corrected. Post cardiopulmonary bypass TEE examination revealed no residual ventricular septal defect (VSD), APW and left/right ventricular outflow tract obstruction [Figure 4] and [Figure 5]. The trachea was extubated after 12 h of mechanical ventilation. The subsequent course was uneventful.{Figure 1}{Figure 2}{Figure 3}{Figure 4}{Figure 5}

Aortopulmonary window is an uncommon anomaly, occurring in 1% of all patients with congenital heart disease. [1] Aortopulmonary window is a communication between the ascending aorta and the pulmonary trunk and/or the right pulmonary artery. It has similar hemodynamic features to a patent ductus arteriosus (PDA) or persistent truncus arteriosus, the anatomical difference from the latter being the presence of well-defined aortic and pulmonary valve apparatus. Undiagnosed children with APW and DORV may die due to heart failure and pulmonary hypertension. The most frequent associated cardiac defects are tetralogy of Fallot, VSD, PDA, single ventricle and absent aortic valve. [2] The combined presence of APW and DORV are reported with maternal phenylketonuria and Cornelia de Lange syndrome. [3],[4]

In congenital heart surgery, during intra- operative echocardiography an effort should be made to screen thoroughly the great arteries and their possible branches to find direct or indirect evidence of aortopulmonary connections or other abnormalities of branches, including their sizes.

References

1Soares AM, Atik E, Cortêz TM, Albuquerque AM, Castro CP, Barbero-Marcial M, et al. Aortopulmonary window. Clinical and surgical assessment of 18 cases. Arq Bras Cardiol 1999; 73:59-74.
2Sabati AA, Wong PC, Randolph L, Pruetz JD. Absent aortic valve associated with double outlet right ventricle and aortopulmonary window: Physiologic implications of a rare malformation in both the fetus and neonate. Congenit Heart Dis 2013 May 22. doi: 10.1111/chd. 12089.
3Cabano R, Lombardi G, Mannarino S, Cerbo RM, Manzoni P, Stronati M. A newborn with double-outlet right ventricle and aortopulmonary window associated with maternal phenylketonuria: A first case report. Acta Paediatr 2011;100:318.
4Akdeniz C, Odemis E, Erdem A, Celebi A. Double outlet right ventricle and aortopulmonary window in a patient with Cornelia de Lange syndrome: A novel association. Genet Couns 2009;20:161-6.