Unrestrictive Aortopulmonary Window

HomeCirculationVol. 133, No. 19Unrestrictive Aortopulmonary Window Free AccessResearch ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessResearch ArticlePDF/EPUBUnrestrictive Aortopulmonary WindowExtreme Presentation as Non-Eisenmenger in a 30-Year-Old Patient Patrick O. Myers, MD, Frédéric Lador, MD, PhD, Anne-Lise Hachulla, MD, Judith Bouchardy, MD, Stéphane Noble, MD, Marc Licker, MD, Jean-Claude Pache, MD, Dimitra Kalimanovaska-Ostric, MD, Milan Djukic, MD, Afksendiyos Kalangos, MD, PhD and Maurice Beghetti, MD Patrick O. MyersPatrick O. Myers From Divisions of Cardiovascular Surgery (P.O.M., A.K.), Pulmonary Hypertension Program (P.O.M., F.L., A.-L.H., S.N., M.L., M.B.), Pneumology (F.L.), Radiology (A.-L.H.), Cardiology (J.B., S.N.), Anesthesiology (M.L.), and Pathology (J.-C.P.), Geneva University Hospitals & Faculty of Medicine, Switzerland; Cardiology Clinic, Clinical Center of Serbia, Belgrade (D.-K.O.); University Children’s Hospital, Belgrade, Serbia (M.D.); and Unit of Pediatric Cardiology, Children’s University Hospital Geneva, Switzerland (M.B.). Search for more papers by this author , Frédéric LadorFrédéric Lador From Divisions of Cardiovascular Surgery (P.O.M., A.K.), Pulmonary Hypertension Program (P.O.M., F.L., A.-L.H., S.N., M.L., M.B.), Pneumology (F.L.), Radiology (A.-L.H.), Cardiology (J.B., S.N.), Anesthesiology (M.L.), and Pathology (J.-C.P.), Geneva University Hospitals & Faculty of Medicine, Switzerland; Cardiology Clinic, Clinical Center of Serbia, Belgrade (D.-K.O.); University Children’s Hospital, Belgrade, Serbia (M.D.); and Unit of Pediatric Cardiology, Children’s University Hospital Geneva, Switzerland (M.B.). Search for more papers by this author , Anne-Lise HachullaAnne-Lise Hachulla From Divisions of Cardiovascular Surgery (P.O.M., A.K.), Pulmonary Hypertension Program (P.O.M., F.L., A.-L.H., S.N., M.L., M.B.), Pneumology (F.L.), Radiology (A.-L.H.), Cardiology (J.B., S.N.), Anesthesiology (M.L.), and Pathology (J.-C.P.), Geneva University Hospitals & Faculty of Medicine, Switzerland; Cardiology Clinic, Clinical Center of Serbia, Belgrade (D.-K.O.); University Children’s Hospital, Belgrade, Serbia (M.D.); and Unit of Pediatric Cardiology, Children’s University Hospital Geneva, Switzerland (M.B.). Search for more papers by this author , Judith BouchardyJudith Bouchardy From Divisions of Cardiovascular Surgery (P.O.M., A.K.), Pulmonary Hypertension Program (P.O.M., F.L., A.-L.H., S.N., M.L., M.B.), Pneumology (F.L.), Radiology (A.-L.H.), Cardiology (J.B., S.N.), Anesthesiology (M.L.), and Pathology (J.-C.P.), Geneva University Hospitals & Faculty of Medicine, Switzerland; Cardiology Clinic, Clinical Center of Serbia, Belgrade (D.-K.O.); University Children’s Hospital, Belgrade, Serbia (M.D.); and Unit of Pediatric Cardiology, Children’s University Hospital Geneva, Switzerland (M.B.). Search for more papers by this author , Stéphane NobleStéphane Noble From Divisions of Cardiovascular Surgery (P.O.M., A.K.), Pulmonary Hypertension Program (P.O.M., F.L., A.-L.H., S.N., M.L., M.B.), Pneumology (F.L.), Radiology (A.-L.H.), Cardiology (J.B., S.N.), Anesthesiology (M.L.), and Pathology (J.-C.P.), Geneva University Hospitals & Faculty of Medicine, Switzerland; Cardiology Clinic, Clinical Center of Serbia, Belgrade (D.-K.O.); University Children’s Hospital, Belgrade, Serbia (M.D.); and Unit of Pediatric Cardiology, Children’s University Hospital Geneva, Switzerland (M.B.). Search for more papers by this author , Marc LickerMarc Licker From Divisions of Cardiovascular Surgery (P.O.M., A.K.), Pulmonary Hypertension Program (P.O.M., F.L., A.-L.H., S.N., M.L., M.B.), Pneumology (F.L.), Radiology (A.-L.H.), Cardiology (J.B., S.N.), Anesthesiology (M.L.), and Pathology (J.-C.P.), Geneva University Hospitals & Faculty of Medicine, Switzerland; Cardiology Clinic, Clinical Center of Serbia, Belgrade (D.-K.O.); University Children’s Hospital, Belgrade, Serbia (M.D.); and Unit of Pediatric Cardiology, Children’s University Hospital Geneva, Switzerland (M.B.). Search for more papers by this author , Jean-Claude PacheJean-Claude Pache From Divisions of Cardiovascular Surgery (P.O.M., A.K.), Pulmonary Hypertension Program (P.O.M., F.L., A.-L.H., S.N., M.L., M.B.), Pneumology (F.L.), Radiology (A.-L.H.), Cardiology (J.B., S.N.), Anesthesiology (M.L.), and Pathology (J.-C.P.), Geneva University Hospitals & Faculty of Medicine, Switzerland; Cardiology Clinic, Clinical Center of Serbia, Belgrade (D.-K.O.); University Children’s Hospital, Belgrade, Serbia (M.D.); and Unit of Pediatric Cardiology, Children’s University Hospital Geneva, Switzerland (M.B.). Search for more papers by this author , Dimitra Kalimanovaska-OstricDimitra Kalimanovaska-Ostric From Divisions of Cardiovascular Surgery (P.O.M., A.K.), Pulmonary Hypertension Program (P.O.M., F.L., A.-L.H., S.N., M.L., M.B.), Pneumology (F.L.), Radiology (A.-L.H.), Cardiology (J.B., S.N.), Anesthesiology (M.L.), and Pathology (J.-C.P.), Geneva University Hospitals & Faculty of Medicine, Switzerland; Cardiology Clinic, Clinical Center of Serbia, Belgrade (D.-K.O.); University Children’s Hospital, Belgrade, Serbia (M.D.); and Unit of Pediatric Cardiology, Children’s University Hospital Geneva, Switzerland (M.B.). Search for more papers by this author , Milan DjukicMilan Djukic From Divisions of Cardiovascular Surgery (P.O.M., A.K.), Pulmonary Hypertension Program (P.O.M., F.L., A.-L.H., S.N., M.L., M.B.), Pneumology (F.L.), Radiology (A.-L.H.), Cardiology (J.B., S.N.), Anesthesiology (M.L.), and Pathology (J.-C.P.), Geneva University Hospitals & Faculty of Medicine, Switzerland; Cardiology Clinic, Clinical Center of Serbia, Belgrade (D.-K.O.); University Children’s Hospital, Belgrade, Serbia (M.D.); and Unit of Pediatric Cardiology, Children’s University Hospital Geneva, Switzerland (M.B.). Search for more papers by this author , Afksendiyos KalangosAfksendiyos Kalangos From Divisions of Cardiovascular Surgery (P.O.M., A.K.), Pulmonary Hypertension Program (P.O.M., F.L., A.-L.H., S.N., M.L., M.B.), Pneumology (F.L.), Radiology (A.-L.H.), Cardiology (J.B., S.N.), Anesthesiology (M.L.), and Pathology (J.-C.P.), Geneva University Hospitals & Faculty of Medicine, Switzerland; Cardiology Clinic, Clinical Center of Serbia, Belgrade (D.-K.O.); University Children’s Hospital, Belgrade, Serbia (M.D.); and Unit of Pediatric Cardiology, Children’s University Hospital Geneva, Switzerland (M.B.). Search for more papers by this author and Maurice BeghettiMaurice Beghetti From Divisions of Cardiovascular Surgery (P.O.M., A.K.), Pulmonary Hypertension Program (P.O.M., F.L., A.-L.H., S.N., M.L., M.B.), Pneumology (F.L.), Radiology (A.-L.H.), Cardiology (J.B., S.N.), Anesthesiology (M.L.), and Pathology (J.-C.P.), Geneva University Hospitals & Faculty of Medicine, Switzerland; Cardiology Clinic, Clinical Center of Serbia, Belgrade (D.-K.O.); University Children’s Hospital, Belgrade, Serbia (M.D.); and Unit of Pediatric Cardiology, Children’s University Hospital Geneva, Switzerland (M.B.). Search for more papers by this author Originally published10 May 2016https://doi.org/10.1161/CIRCULATIONAHA.115.020819Circulation. 2016;133:1907–1910A 30-year-old woman was referred for management of an aortopulmonary window. Her history was notable for chronic atrial fibrillation treated by amiodarone and Coumadin anticoagulation. Peripheral oxygen saturation was 95%. A chest x-ray film showed cardiomegaly, bilateral prominent hilar vascularization, and interstitial edema (Figure 1). The echocardiogram showed a large, 15-mm aortopulmonary (AP) window, ≈25 mm above the aortic valve annulus between the ascending aorta and main pulmonary artery (type I) with an unrestrictive, exclusively left-right shunt and a peak gradient of 30 mm Hg (Figure 2). There was no aortic coarctation or interruption of the aortic arch, and the main pulmonary artery (3.3 cm) was dilated to a size equal to the aortic root, with dilated branch pulmonary arteries (2.3 cm each). The left atrium was severely dilated (45 cm2), as was the left ventricle (end-diastolic diameter, 7.5 cm; indexed, 5.2 cm/m2). There was moderate mitral regurgitation (regurgitation volume, 26.6 mL; proximal isovelocity surface area convergence radius, 6.8 mm) attributable to annular dilatation. The biventricular systolic function was normal. The right ventricular systolic pressure was estimated at 63.3 mm Hg by the gradient of the tricuspid regurgitation jet. Cardiac MRI confirmed these findings (Figure 3). Cardiac catheterization showed a pulmonary systolic pressure 40% of systemic pressure (44/20 mm Hg; mean, 32), pulmonary capillary wedge pressure of 20 mm Hg, cardiac index of 3.04 mL·min–1·m–2, pulmonary vascular resistance (PVR) of 1.79 Woods units (WU)/m2, and PVR:systemic vascular resistance (SVR) ratio of 0.21. After acute vasodilator testing with inhaled nitric oxide, the PVR decreased to 1.54 WU/m2 and a PVR:SVR ratio of 0.11. The hemodynamics met criteria suggestive of operability.1 Test occlusion with a 12-mm Tyshak balloon was not successful, and the patient was brought to the operating room for surgical repair and lung biopsy. The AP window was closed with a 14-mm circular GoreTex patch under cardiopulmonary bypass and cardioplegic arrest. The patient was weaned uneventfully from cardiopulmonary bypass without systemic vasopressors, pulmonary vasodilators, or inotropes. A biopsy was taken from the anterior segment of the superior lobe of the right lung. The patient was extubated hours after the operation. Her postoperative course was uneventful. The discharge echocardiogram showed no residual shunt, decrease of left ventricular (end-diastolic diameter, 6.1 cm; indexed, 4.2 cm/m2) and left atrial (36.5 cm2) dilatation, mild mitral regurgitation, and regression of the pulmonary artery dilatation (main, 2.3 cm; branch arteries, 1.4 cm). There was moderate biventricular systolic dysfunction. There was no significant tricuspid regurgitation, prohibiting estimation of the right ventricular pressure. Cardiac catheterization 20 days after surgical repair showed a pulmonary artery pressure of 68/27 mm Hg (mean, 41), pulmonary capillary wedge pressure of 11 mm Hg, cardiac index of 2.5 L·min–1·m–2, PVR of 5.5 WU/m2 with a PVR:SVR ratio of 0.34, and normal end-diastolic left ventricular pressure (6 mm Hg). After pulmonary vasodilation with inhaled nitric oxide, the PVR decreased to 2.89 WU/m2 with a PVR:SVR ratio of 0.12 and pulmonary capillary wedge pressure of 14 mm Hg (Figure 4). The patient was placed on sildenafil 20 mg 3 times per day. The 6-minute walk test showed a distance 64% of predicted, with a transcutaneous saturation decreasing from 98% to 91%. The lung biopsy (Figure 5) showed muscularization of the media of the intralobar and centrolobar arteries, with no intimal reaction, compatible with Heath and Edwards grade I pulmonary hypertension.2Download figureDownload PowerPointFigure 1. Preoperative anteroposterior chest x-ray.Download figureDownload PowerPointFigure 2. Preoperative echocardiogram. A, Four-chamber view showing left atrial and ventricular dilatation and moderate mitral regurgitation. B, Long-axis view at the level of the aortopulmonary window (*).Download figureDownload PowerPointFigure 3. Preoperative cardiac MRI. Coronal reconstruction at the level of the aortopulmonary window (arrow), showing the defect and (A) left-right shunt in contrast phase (B).Download figureDownload PowerPointFigure 4. Vasodilator testing 20 days after surgical repair. Pulmonary artery pressure at baseline (A) and after inhaled nitric oxide administration (B).Download figureDownload PowerPointFigure 5. Lung biopsy histopathology. A small pulmonary artery demonstrates hypertrophy of the media (hematoxylin and eosin stain, original magnification ×400).At follow-up 13 months after repair and after discontinuing sildenafil, the patient denied any symptoms and had resumed full-time work. Her echocardiogram showed regression of left ventricular dilatation (end-diastolic diameter, 5.9 cm), mild systolic left ventricular dysfunction (ejection fraction, 50%), moderate mitral regurgitation, and estimated right ventricular systolic pressure of 35 mm Hg. Cardiac catheterization showed a pulmonary artery pressure of 31/8 mm Hg (mean, 18 mm Hg), a mean pulmonary capillary wedge pressure of 8 mm Hg, pulmonary vascular resistance of 1.6 WU/m2, with a PVR:SVR ratio of 0.03.An AP window, a failure of septation between the ascending aorta and the pulmonary artery during fetal life because of nonfusion of the opposing conotruncal ridges responsible for separating the truncus arteriosus into the aorta and pulmonary artery, is a rare congenital cardiac anomaly.3 Patients usually present in the first week of life, because the lesion is usually large and nonrestrictive.4 Late or extremely late presentation is unusual, but has been reported in adults at the stage of cyanosis and Eisenmenger syndrome.5 We report the rare presentation of an AP window with an unrestrictive left-right shunt in a patient at 30 years of age, still at the stage of reactive pulmonary hypertension and who underwent successful surgical repair. Despite this positive short-term outcome, with an excellent functional result and normalization of hemodynamics just beyond 1 year from surgery, the long-term outcome remains to be seen. Not all patients follow the predicted development of irreversible pulmonary vascular lesion. This patient is of particular interest because AP windows are thought to develop rapid pulmonary vascular disease. Understanding why some patients seem to be protected from the development of pulmonary vascular disease would be of extreme importance for the treatment of pulmonary hypertension.DisclosuresDr Beghetti has served as consultant, steering committee and/or advisory board member for Actelion Pharmaceuticals Ltd., Bayer-Healthcare, Eli Lilly, GlaxoSmithKline, and Pfizer. The other authors report no conflicts.FootnotesCorrespondence to Patrick O. Myers, MD, Division of Cardiovascular Surgery, Geneva University Hospitals & Faculty of Medicine, 4 rue Gabrielle-Perret-Gentil, 1211 Geneva, Switzerland. E-mail [email protected]References1. Myers PO, Tissot C, Beghetti M. Assessment of operability of patients with pulmonary arterial hypertension associated with congenital heart disease.Circ J. 2014; 78:4–11.CrossrefMedlineGoogle Scholar2. Heath D, Edwards JE. The pathology of hypertensive pulmonary vascular disease; a description of six grades of structural changes in the pulmonary arteries with special reference to congenital cardiac septal defects.Circulation. 1958; 18(4 pt 1):533–547.LinkGoogle Scholar3. Naimo PS, Yong MS, d’Udekem Y, Brizard CP, Kelly A, Weintraub R, Konstantinov IE. Outcomes of aortopulmonary window repair in children: 33 years of experience.Ann Thorac Surg. 2014; 98:1674–1679. doi: 10.1016/j.athoracsur.2014.06.043.CrossrefMedlineGoogle Scholar4. Barnes ME, Mitchell ME, Tweddell JS. Aortopulmonary window.Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2011; 14:67–74. doi: 10.1053/j.pcsu.2011.01.017.CrossrefMedlineGoogle Scholar5. Bobylev D, Sommer W, Avsar M, Horke A, Haverich A, Warnecke G. Aortopulmonary window: a rare untreated adult case.Heart Lung Circ. 2014; 23:e235–e236. doi: 10.1016/j.hlc.2014.06.006.CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited By Das D, Dutta N, Das P, Sharma M, Koley R, Das S, Chattopadhyay A, Ghosh S and Narayan P (2022) Late presentation of aortopulmonary window: a contemporary series, Indian Journal of Thoracic and Cardiovascular Surgery, 10.1007/s12055-021-01306-x, 38:4, (403-407), Online publication date: 1-Jul-2022. Rana A, Abbey P, Anand R and Gupta A (2021) Hidden in plain sight: A rare abnormality detected on computed tomography pulmonary angiography, Lung India, 10.4103/lungindia.lungindia_842_20, 38:5, (491), . Zografos P, Protopapas E, Hakim N, Alexopoulos C and Sarris G (2019) Remarkably Still Repairable Large Aortopulmonary Window in an Adult Patient, World Journal for Pediatric and Congenital Heart Surgery, 10.1177/2150135119878703, 11:1, (117-119), Online publication date: 1-Jan-2020. El Dick J, El‐Rassi I, Tayeh C, Bitar F and Arabi M (2019) Aortopulmonary window in adults: A rare entity leading to Eisenmenger syndrome, Echocardiography, 10.1111/echo.14368, 36:6, (1173-1178), Online publication date: 1-Jun-2019. Micheletti A (2019) Congenital Heart Disease Classification, Epidemiology, Diagnosis, Treatment, and Outcome Congenital Heart Disease, 10.1007/978-3-319-78423-6_1, (1-67), . Myers P and Beghetti M (2016) Response by Myers and Beghetti to Letter Regarding Article, “Unrestrictive Aortopulmonary Window: Extreme Presentation as Non-Eisenmenger in a 30-Year-Old Patient”, Circulation, 134:15, (e330-e331), Online publication date: 11-Oct-2016.Murakami T, Horibata Y and Niwa K (2016) Letter by Murakami et al Regarding Article, “Unrestrictive Aortopulmonary Window: Extreme Presentation as Non-Eisenmenger in a 30-Year-Old Patient”, Circulation, 134:15, (e328-e329), Online publication date: 11-Oct-2016. May 10, 2016Vol 133, Issue 19 Advertisement Article InformationMetrics © 2016 American Heart Association, Inc.https://doi.org/10.1161/CIRCULATIONAHA.115.020819PMID: 27166350 Originally publishedMay 10, 2016 PDF download Advertisement SubjectsCardiovascular SurgeryCongenital Heart DiseasePulmonary Hypertension