Aortopulmonary Window and Associated Anomalies

Aortopulmonary Window andAssociated AnomaliesJames S. Tweddell, MDProfessor of Surgery and Pediatrics,Chair, Division of Cardiothoracic Surgery,Medical College of Wisconsin.S. Bert Litwin Chair of Cardiothoracic Surgery,Herma Heart Center,Children’s Hospital of Wisconsin.AATS, Toronto, Ontario, Canada, May 2, 2010

Disclosures•Financial disclosures–Consultant for Cubist Pharmaceuticals–Consultant for Shering­Plough Corporation

Aortopulmonary Window• The result of failure of fusion ofthe two opposing conotruncalridges that are responsible forseparating the truncus arteriosusinto the aorta and pulmonaryartery.• APW occurs between theascending aorta and the mainpulmonary artery and may befound just above the semilunarvalves or between the moredistal ascending aorta andmain pulmonary artery.

ClassificationMori, K et al British Heart Journal 1978

Classification•Perhaps more a continuum?Backer and Mavroudis EJCTS 2002

Classification –APW with IAA• Embryology of aortopulmonary windowincluding interrupted aortic archBerry, T et al American Journal Cardiology 1982

Classification –APW with IAAKonstantinov et al JTCVS 2006

Antenatal Diagnosis•First reported by Collinet et al 2002.–Simple aortopulmonary window may notbe identified by fetal echocardiographybecause equal pressure in the ascendingaorta and pulmonary root.•Antenatal diagnosis ofaortopulmonary window withinterrupted aortic arch first reported in2009 by Hayashi et al–Posterior deviation of the outlet septum isabsent.

Presentation•Similar to that of other patients withleft­to­right shunts, such as PDA or VSDand depends on the size of the defect.•congestive heart failure–tachypnea–diaphoresis–poor feeding–inadequate weight gain are common.with large defects, bidirectional shuntingcan produce systemic desaturation.

Presentation• Physical exam– tachypneic infant with accessory respiratorymuscle use.– Cardiac examination reveals an enlarged heart,and like patients with patent ductus arteriosus,the pulses are bounding.– A systolic murmur can be heard along the leftsternal border; however, unlike patients with apatent ductus arteriosus, a diastolic componentto the murmur is rare.– Patients with associated arch abnormalitiesfrequently present with shock coinciding withclosing of the ductus arteriosus.

Presentation• The diagnosis is routinelymade withechocardiography.• The location and size of thecommunication as well asassociated anomalies arecarefully identified.• Cardiac catheterization isoccasionally indicated for thepatient who presents afterearly infancy to assesspulmonary vascular resistanceGangana et al Arq Bras Cardiol 2007

Presentation•MRIGarver, K. A. et al. Circulation 1997

Presentation•Associated cardiac lesions >50% (otherthan ASD and PDA)–Arch anomalies; arch hypoplasia/coarct arch interruption one­third–VSD–RVOT anomalies; TOF, PS, PA/IVS, aorticorigin of RPA–LVOTO–Transposition–Coronary anomalies

Indications for intervention•Presence of an aortopulmonarywindow is an indication for treatment–Very small window could be followed andultimately closed using catheter basedtechniques•Small to moderate intermediate APWmay be suitable for device closure–Access in a small infant may be difficult•Repair is best accomplished using CPB

Preoperative management•Resuscitation ­ limiting excessive pulmonaryblood flow– requires intubation and mechanical– sedation ± neuromuscular blockade– hypercapnea and minimizing FIO 2 will increasethe pulmonary vascular resistance, decrease leftto­rightshunting, and improve systemic oxygendelivery.– Inotropic support may be required.• Prostaglandin infusion is necessary inpatients with Interrupted aortic arch

Su rg ic a l R e p a ir o f A P WYear Surgeon Method1948 Gross Ligation1953 Scott and Sabiston Division without cardiopulmonary bypass1957 Cooley et al Division with cardiopulmonary bypass1966 Putnam and Gross Transpulmonary patch closure1967­1968 Negre et alBircksJohansson et alAnterior sandwich patch closure1968 Wright et al Transaortic suture closure1969 Deverall et al Transaortic patch closure1987 Shatapathy et al Pulmonary artery flap closure1992 Matsuki et al Anterior pulmonary artery flap closureFrom Gaynor in Pediatric Cardiac Surgery

Operative technique – APW• Median sternotomy• Inspection– Extent of APW– Evidence ofanomalous coronaryarteries

Operative technique – APW• Preparation for CPB– Encircle branchpulmonary arteries– Arterial cannulation– Single venouscannulation unlessassociated intracardiacrepair is necessary• Once CPB initiatedbranch PAs are snared– Mild hypothermia– Antegrade cardioplegia

Operative technique – APW• Repair of APW canbe accomplishedusing an incisionthrough the aorta,pulmonary artery orthe aortopulmonarywindow

Operative technique – APW• Repair of APW bestaccomplishedthrough an incisionin the window itself– Incision initiated inthe superior aspect– Identify the positionof coronary originsbefore continuing– Single patch ofGore­Tex

Operative technique – APW• Single suture line• Anteriorly suture lineis used to completeclosure of aorta andpulmonary artery

Operative technique –APW with IAA• Aortopulmonary window with interrupted aortic archPDAType A IAAMPARPA origin from ascending aorta

Operative technique –APW with IAA

Operative technique –APW with IAA

Operative technique –APW with IAA

Results of repair• Outcome dependent on era of surgery andpresence of additional anomalies– APW with IAA associated with increased time relatedmortality, hazard ratio = 5.87, p = 0.009Bagtharia et al Cardiol Young 2004

Children’s Hospital of Wisconsin experience• Between 1983 and 2009 n = 24• Simple APW (n = 11)• Complex APW (n = 13)– IAA or coarctation of the aorta (n = 8),– VSD (n = 1),– PA/VSD and anomalous origin of the rightcoronary artery (n = 1),– PA/IVS and PAPVR (n = 1),– d­transposition of the great vessels (n = 1)– congenital absence of the left pulmonary arterywith pulmonary artery hypertension (n = 1).

Children’s Hospital of Wisconsin experience• Simple APW– No early or late mortality• Complex APW– Early mortality = 1 APW, pulmonary atresia andintact ventricular septum and PAPVR.– Late mortality = 1 Following lung transplantationin the patient with APW, absent left pulmonaryartery and pulmonary hypertension.– Morbidity –one patient underwent surgical repairof residual arch obstruction

Conclusion•In the current era results with APW areexcellent, mortality is rare and isassociated with concomitantanomalies•Long­term follow­up is indicated–Branch pulmonary artery stenosis–Recurrent arch obstruction

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