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Prenatal diagnosis of aortopulmonary window associated with aberrant subclavian artery

Published online by Cambridge University Press:  16 March 2017

Adetola F. Louis-Jacques ,
Sarah G. Običan ,
Thieu Nguyen  and
Anthony Odibo
Adetola F. Louis-Jacques*
Affiliation:
Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, University of South Florida Morsani College of Medicine, 2 Tampa General Circle, Tampa, FL 33606, United States of America
Sarah G. Običan
Affiliation:
Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, University of South Florida Morsani College of Medicine, 2 Tampa General Circle, Tampa, FL 33606, United States of America
Thieu Nguyen
Affiliation:
Division of Pediatric Cardiology, Department of Cardiology, All Children’s Heart Institute, 601 5th Street, Saint Petersburg, FL 33701, United States of America
Anthony Odibo
Affiliation:
Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, University of South Florida Morsani College of Medicine, 2 Tampa General Circle, Tampa, FL 33606, United States of America
*
Correspondence to: A. F. Louis-Jacques, Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, University of South Florida Morsani College of Medicine, 2 Tampa General Circle, Tampa, FL 33606, United States of America. Tel: 813 259 0828; Fax: 813 250 2203; E-mail: alouisjacques@health.usf.edu
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Abstract

Aortopulmonary window is a rare cardiac developmental anomaly characterised by a communication between the ascending aorta and the pulmonary artery. Aortopulmonary window may be isolated or associated with cardiac defects such as ventricular septal defect, atrial septal defect, interrupted aortic arch, and tetralogy of Fallot. We report a case of aortopulmonary window associated with aberrant subclavian artery based on fetal two-dimensional echocardiogram. The mother was referred for fetal echocardiography because of multiple fetal anomalies. Prenatal echocardiography at 30 weeks of gestation revealed a defect between the main and right pulmonary arteries and the ascending aorta (type III). The patient was born at 38 weeks of gestation via caesarean delivery, and was admitted to the neonatal intensive care unit because of respiratory failure and multiple congenital anomalies. Postnatal echocardiogram and cardiac MRI confirmed the prenatal findings. In addition, this patient had severe Dandy–Walker malformation and renal anomalies with poor prognosis. The family decided to withdraw respiratory care support on day of life 4, and the neonate passed away shortly after.

Keywords

Aortopulmonary windowaortopulmonary septal defectsCHD
Type
Case Report
Information
Cardiology in the Young , Volume 27 , Issue 7 , September 2017 , pp. 1441 - 1443
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Copyright
© Cambridge University Press 2017 

Case description

A 26-year-old woman – gravida 3, para 1, abortion 1 – with history notable for bipolar disorder and marijuana use underwent an anatomy scan at 28 weeks of gestation. The anatomy ultrasound revealed bilateral urinary tract dilation of low risk and severe bilateral ventriculomegaly. A fetal echocardiogram was performed at 30 weeks of gestation because of multiple fetal anomalies. The fetal heart showed situs solitus and levocardia, with normal venoatrial, atrioventricular, and ventriculoarterial connections. A 6×8-mm aortopulmonary window was visualised (Figs 1–3). Color and pulsed Doppler examinations confirmed unrestrictive flow across the defect. There was also evidence of an aberrant right subclavian artery on color flow mapping.

Figure 1 Fetal echocardiogram at the time of diagnosis at 30 weeks of gestation. ( a ) Normal four chamber. ( b ) Aortic arch. ( c ) Left ventricular outflow tract. ( d ) Right ventricular outflow tract in the short-axis view demonstrating the aortopulmonary window. AO=aorta; LA=left atrium; LV=left ventricle; PA=pulmonary artery; RA=right atrium; RV=right ventricle; W=window.

Figure 2 Fetal echocardiogram demonstrating the aortopulmonary window in the long axis. AO=aorta; LPA=left pulmonary artery; MPA=main pulmonary artery; RPA=right pulmonary artery; W=window.

Figure 3 Postnatal echocardiogram demonstrating the aortopulmonary window in the short axis with and without color flow. AO=aorta; AoV=aortic valve; bpm=beats per minute; MPA=main pulmonary artery; PV=pulmonary vein; RA=right atrium; RPA=right pulmonary artery; RV=right ventricle; W=window.

Delivery was by repeat caesarean delivery at 38 weeks of gestation due to severe hydrocephalus. A male infant was born with complications, weighing 5 kg with a length of 52.5 cm. Extracardiac malformations included severe hydrocephalus – Dandy–Walker malformation – vertebral anomalies, and bilateral urinary tract dilation of the kidneys. Postnatal chromosome analysis revealed a normal karyotype, but the microarray reported a pathogenic terminal gain in 7p and terminal loss in 6p. The postnatal echocardiogram and cardiac MRI confirmed prenatal diagnosis of type III aortopulmonary window and aberrant right subclavian artery.

Discussion

Aortopulmonary window is one of the more rare forms of CHD comprising up to 0.2% of all live birth cardiac defects.Reference Kuehn, Oberhoffer, Vogt, Lange and Hess 1 , Reference Alborino, Guccione, Di Donato and Marino 2 Aortopulmonary window is defined as an opening or a communication between the ascending aorta and the pulmonary artery in the presence of two anatomically separate semilunar – aortic and pulmonary – valves. The Society of Thoracic Surgeons Congenital Heart Surgery Database Committee for aortopulmonary window recommended a classification system for aortopulmonary window (Table 1).Reference Backer and Mavroudis 3 With fetal echocardiography, early detection is possible; however, without heightened suspicion, an aortopulmonary window may be missed, especially in fetuses with complex cardiac malformations and ever increasing maternal obesity.Reference Alborino, Guccione, Di Donato and Marino 2 Aortopulmonary window is associated with concomitant CHD in 25–35% of the time.Reference Kuehn, Oberhoffer, Vogt, Lange and Hess 1 , Reference Alborino, Guccione, Di Donato and Marino 2 The most common of these include tetralogy of Fallot, ventricular septal defects, and aortic arch abnormalities,Reference Kuehn, Oberhoffer, Vogt, Lange and Hess 1 but atrial septal defects, patent ductus arteriosus, and coronary artery anomalies have also been noted.Reference Kose, Ucar, Emet, Akpinar and Yalin 4 Early fetal diagnosis and treatment is critical in order to improve neonatal outcomes. This helps avoid congestive heart failure, which can occur as early as the 1st week of neonatal life and development of irreversible pulmonary hypertension.Reference van Son, Puga and Dandzlson 5 Surgical repair is recommended as soon as diagnosis of aortopulmonary window is established.Reference Backer and Mavroudis 3 As with all CHD, survival rates depend on concomitant defects and genetics and overall are reported to be close to 90%.Reference Kuehn, Oberhoffer, Vogt, Lange and Hess 1

Table 1 Aortopulmonary window classification system*.

* Adapted from Backer and MavroudisReference Backer and Mavroudis 3

Conclusion

Visualising the aortopulmonary septum during a fetal echocardiographic examination is essential to allow for diagnosis of a very rare but critical fetal diagnosis. Prenatal diagnosis of aortopulmonary window is important because early corrective surgery in the neonatal period prevents congestive heart failure and pulmonary vascular obstructive disease.

Acknowledgement

None.

Financial Support

This research received no specific grant from any funding agency, commercial or not-for-profit sectors.

Conflicts of Interest

None.

References

1. 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: 793–796.CrossRefGoogle ScholarPubMed
2. Alborino, D, Guccione, P, Di Donato, R, Marino, B. Aortopulmonary window coexisting with tetralogy of fallot. J Cardiovasc Surg 2001; 42: 197–199.Google ScholarPubMed
3. Backer, CL, Mavroudis, C. Surgical management of aortopulmonary window: a 40-year experience. Eur J Cardiothorac Surg 2002; 21: 773–779.CrossRefGoogle ScholarPubMed
4. Kose, M, Ucar, S, Emet, S, Akpinar, TS, Yalin, K. A case of aortopulmonary window: asymptomatic until the first pregnancy. Case Rep Cardiol 2015; 2015.Google Scholar
5. van Son, JA, Puga, FJ, Dandzlson, GK, et al. Aortopulmonary window: factors associated with early and late success after surgical treatment. Mayo Clin Proc 1993; 68: 128–133.CrossRefGoogle ScholarPubMed
Figure 0

Figure 1 Fetal echocardiogram at the time of diagnosis at 30 weeks of gestation. (a) Normal four chamber. (b) Aortic arch. (c) Left ventricular outflow tract. (d) Right ventricular outflow tract in the short-axis view demonstrating the aortopulmonary window. AO=aorta; LA=left atrium; LV=left ventricle; PA=pulmonary artery; RA=right atrium; RV=right ventricle; W=window.

Figure 1

Figure 2 Fetal echocardiogram demonstrating the aortopulmonary window in the long axis. AO=aorta; LPA=left pulmonary artery; MPA=main pulmonary artery; RPA=right pulmonary artery; W=window.

Figure 2

Figure 3 Postnatal echocardiogram demonstrating the aortopulmonary window in the short axis with and without color flow. AO=aorta; AoV=aortic valve; bpm=beats per minute; MPA=main pulmonary artery; PV=pulmonary vein; RA=right atrium; RPA=right pulmonary artery; RV=right ventricle; W=window.

Figure 3

Table 1 Aortopulmonary window classification system*.