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 Table of Contents  
Year : 2021  |  Volume : 7  |  Issue : 1  |  Page : 60-62

An unusual case report of two cases with coexisting type I aortopulmonary window with tetralogy of fallot with pulmonary atresia

Department of Cardiothoracic and Vascular Surgery, Cardiothoracic and Neurosciences Centre, All India Institute of Medical Sciences, New Delhi, India

Date of Submission18-Sep-2020
Date of Decision17-Mar-2021
Date of Acceptance17-Mar-2021
Date of Web Publication24-Apr-2021

Correspondence Address:
Palleti Rajashekar
Department of Cardiothoracic and Vascular Surgery, Cardiothoracic and Neurosciences Centre, CTVS Office, 7th Floor, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110 029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jpcs.jpcs_92_20

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Aortopulmonary window is an uncommon truncal anomaly. Its coexistence with tetralogy of Fallot (TOF) with pulmonary atresia is rare. This rare complex association is always a surgical challenge due to lack of an established surgical approach. The association with TOF makes the interventional approach unlikely. In the two cases summarized below, we have addressed the investigation strategy reaching the final diagnosis. The surgical interventions and our decision-making in the final adopted technique have been highlighted. The relevant surgical outcome with postoperative follow-up has been discussed. The rarity of this association without a standard management algorithm mandates the relevance of this presentation.

Keywords: Pericardium, pulmonary atresia, pulmonary valve, ventricular septal defect

How to cite this article:
Vaswani P, Rajashekar P, Devagourou V. An unusual case report of two cases with coexisting type I aortopulmonary window with tetralogy of fallot with pulmonary atresia. J Pract Cardiovasc Sci 2021;7:60-2

How to cite this URL:
Vaswani P, Rajashekar P, Devagourou V. An unusual case report of two cases with coexisting type I aortopulmonary window with tetralogy of fallot with pulmonary atresia. J Pract Cardiovasc Sci [serial online] 2021 [cited 2023 May 28];7:60-2. Available from: https://www.j-pcs.org/text.asp?2021/7/1/60/314485

  Introduction Top

An aortopulmonary window (APW) is an abnormal communication between the main pulmonary artery (MPA) and the ascending aorta in the presence of two separate semilunar valves.[1] It can occur as an isolated condition or in association with other cardiac anomalies. Its coexistence with tetralogy of Fallot (TOF) with pulmonary atresia (PA) is rare.[2] Only a few sporadic case reports are available with this description. We would like to share our experience with two such cases with successful surgical outcome.

  Case Description Top

Case 1

A 2-year-old female child weighing 8 kg presented with symptoms of recurrent lower respiratory tract infections (LRTIs) and failure to thrive with saturation of 96% on room air. Clinical examination revealed tachycardia and tachypnea with chest retractions. Chest X-ray showed normal size heart with pulmonary plethora. On evaluation by transthoracic-two-dimensional echocardiography (2D echo), a diagnosis of TOF with PA with large type I APW associated with severe pulmonary arterial hypertension (PAH) was made. Standard median sternotomy was performed. The branch pulmonary arteries were dissected and looped before systemic heparinization [Figure 1]. Cardiopulmonary bypass (CPB) was established with aortobicaval cannulation, and pulmonary arteries were snugged to avoid runoff. After aortic cross-clamp, and usual myocardial protection by antegrade cold blood cardioplegia and systemic hypothermia, APW was repaired by transwindow approach using polytetrafluoroethylene (PTFE) patch by sandwich technique [Figure 2]. The right ventricular outflow tract (RVOT) was opened in the infundibular portion, and resection of septal and parietal bands was performed. Large, single, malaligned subaortic ventricular septal defect (VSD) was closed by a Dacron patch from the same incision. On beating heart, while rewarming, bicuspid pulmonary valve was reconstructed using 0.1-mm thick PTFE membrane, and RVOT augmentation was performed using PTFE patch [Figure 3].[3] The child was gradually weaned off CPB with 5 µg/kg/min of dobutamine and 0.5 µg/kg/min of milrinone. The child had uneventful postoperative period, and the 2D echo at the time of discharge revealed no residual VSD or RVOT gradient with moderate pulmonary regurgitation. On 1-year follow-up, the child has gained weight, healthy, and playful.

Case 2
Figure 1: Surgical anatomy.

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Figure 2: Sandwich polytetrafluoroethylene patch closure of aortopulmonary window.

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Figure 3: Right ventricular outflow tract reconstruction with polytetrafluoroethylene patch.

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An 11-month-old, 5.3 kg male child presented with a history of recurrent LRTI and congestive heart failure with saturation of 95% on room air. Chest X-ray revealed a boot-shaped heart with bilateral plethoric lung fields. Two-dimensional echo revealed truncus arteriosus type 1 with large subtruncal VSD with severe PAH. Contrast-enhanced computerized tomography (CECT) confirmed the presence of TOF with short-segment PA with APW type 1 [Figure 4]. It also revealed a high origin of right coronary artery coursing anterior to APW. The branch pulmonary arteries were good sized. The CPB strategy and myocardial protection were similar to Case 1. In this case, both the branched pulmonary arteries were mobilized till the first prebranching portions. The APW was repaired through the pulmonary artery with a PTFE patch. Later, VSD was closed with a Dacron patch through the right atrial approach. RVOT was opened just below the atretic pulmonary artery, and infundibular muscle bundle resection was performed. The confluence was opened further toward the left pulmonary artery to reconstruct wider and adequate-sized RVOT. The pulmonary confluence was brought down to the ventriculotomy to establish the continuity of RVOT posteriorly. Later, the RVOT augmentation was performed using nonfixed autologous pericardium. Weaning of CPB and inotropic strategy was similar to Case 1. The postoperative course was uneventful, and the 2D echo at the time of discharge showed no residual VSD or RVOT gradient with free pulmonary regurgitation. The child is doing well with improved 2D echo findings on follow-up at 6 months.
Figure 4: Contrast-enhanced computerized tomography anatomy – high origin of the right coronary artery.

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  Discussion Top

APW is a rare truncal anomaly with an incidence of 0.2% of live births.[2] Jacobs et al. classified into four types based on relation to the ascending aorta, namely type I is proximal, type II is distal, type III is a total defect involving the majority of the ascending aorta, and type IV is the intermediate defect.[4] The association of other coexisting anomalies of conotruncal origin with APW can be explained by abnormalities in fusion of aorticopulmonary and conotruncal septum.[1],[5] The presence of APW with TOF usually masks the cyanosis, and these kids mostly present with signs of congestive heart failure as was seen in both cases highlighting the fact that clinical examination is not definitive in these co-associations.[6] Similar difficulties are proposed at a cardiac catheter-based study.[5],[6]

Two-dimensional echo is a reliable diagnostic modality, however, in this unusual co-existence, it may miss the PA component entirely due to increased pulmonary blood flow.[7] Echo with obstetric ultrasound can also detect anomalies in utero, therefore, its importance cannot be undermined.[8],[9] Indirect evidences of AP window such as dilated cardiac outlets with pulmonary regurgitation may aid further.[8],[9] CECT scan, however, can be used to demonstrate accurate clinicopathological anatomy in the cases of diagnostic dilemma. Although the transcatheter interventions are available for the management of APW, it becomes obsolete in the presence of intracardiac anomalies. The management of such complex combinations is difficult due to different pathophysiological manifestations than they present as of individual entity. The possibility of early development of irreversible PAH warrants an early repair of such entities preferably in infancy.[6],[10]

In our first case, APW was opened directly because of good-sized MPA and normal coronary anatomy. However, in the second case, the presence of high origin of right coronary artery in close proximity with the APW with only short segment PA, enabled the repair via pulmonary artery. APW was closed in both cases with PTFE patch as its smooth design prevents hemolysis and results in less fibrosis.[2]

The management of PA usually requires a conduit, which itself invites multiple re-interventions due to the growth of the child as has been performed in previous reports.[8],[10] The transannular patch in the place of conduit would definitely delay the re-intervention in these small kids, unlike in simple TOF, these patients have PAH, preoperatively. Hence, it is preferable to avoid conduit which does not have growth potential.[2],[3]

In cases of TOF-PA associated with APW, the branch pulmonary arteries are usually good sized as they are under constant systemic pressures.[1] Hence, the APW is conveniently approached through transpulmonary route.[2] This can be extended for RVOT reconstruction after infundibular resection as in our Case 2. Although this route has an association with need for late re-interventions in pulmonary arteries, its preference cannot be disregarded, especially so in association with TOF and also in cases with variant coronary anatomy.

  Conclusion Top

APW with TOF with PA is a rare association. Therefore, no standard surgical approach is available for this rare coexistence. Preoperative assessment of pathological anatomy and proper planning and execution of surgical repair is the key for good surgical outcome.[2] The avoidance of conduit in infants always provides a longer freedom from re-intervention with good quality of life.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patients' guardians have given consent for children's images and other clinical information to be reported in the journal. The patients' guardians understand that the patients' names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Anderson RH, Cook A, Brown NA, Henderson DJ, Chaudhry B, Mohun T. Development of the outflow tracts with reference to aortopulmonary windows and aortoventricular tunnels. Cardiol Young 2010;20 Suppl 3:92-9.  Back to cited text no. 1
Backer CL, Mavroudis C. Surgical management of aortopulmonary window: A 40-year experience. Eur J Cardiothorac Surg 2002;21:773-9.  Back to cited text no. 2
Nunn GR, Bennetts J, Onikul E. Durability of hand-sewn valves in the right ventricular outlet. J Thorac Cardiovasc Surg 2008;136:290-6.  Back to cited text no. 3
Jacobs JP, Jacobs ML, Mavroudis C, Backer CL, Lacour-Gayet FG, Tchervenkov CI, et al. Nomenclature and databases for the surgical treatment of congenital cardiac disease – An updated primer and an analysis of opportunities for improvement. Cardiol Young 2008;18 Suppl 2:38-62.  Back to cited text no. 4
Pérez-Martinez V, Burgueros M, Quero M, Pérez Leon J, Hafer G. Aorticopulmonary window associated with tetralogy of Fallot. Report of one case and review of the literature. Angiology 1976;27:526-34.  Back to cited text no. 5
Kothari SS, Rajani M, Shrivastava S. Tetralogy of Fallot with aortopulmonary window. Int J Cardiol 1988;18:105-8.  Back to cited text no. 6
Awasthy N, Shrivastav S, Iyer KS. Aortopulmonary window with pulmonary atresia: A very rare association. Pediatr Cardiol 2013;34:1052-4.  Back to cited text no. 7
Kuehn A, Oberhoffer R, Vogt M, Lange R, Hess J. Aortopulmonary window with ventricular septal defect and pulmonary atresia: Prenatal diagnosis and successful early surgical correction. Ultrasound Obstet Gynecol 2004;24:793-6.  Back to cited text no. 8
Alborino D, Guccione P, Di Donato R, Marino B. Aortopulmonary window coexisting with tetralogy of Fallot. J Cardiovasc Surg (Torino) 2001;42:197-9.  Back to cited text no. 9
Kostolny M Jr., Schreiber C, Hess J, Lange R. Successful primary correction of tetralogy of Fallot with pulmonary atresia and aortopulmonary window in a 2,220-g neonate with a valved bovine jugular vein conduit. Herz 2004;29:710-12.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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