Tetralogy of Fallot Associated with Aberrant Right Subclavian Artery. Clinical Implications

Since the first description of Tetralogy of Fallot (ToF) in 1671 by Niels Stensen and in 1888 by Étienne-Louis Arthur Fallot, numerous papers have reported on this anomaly, along with its variants and concomitant cardiovascular anomalies. Aberrant right subclavian artery (ARSA) is the most common anomaly of the aortic arch. Different from the left aberrant subclavian artery, occurrence of ARSA in ToF-patients has only casuistically been reported so far. The present study reports on two ToF-patients with ARSA. It is important to note that knowledge of the coexistence of both anomalies has highly practical points during surgical or endovascular corrections of congenital heart defects (including ToF).

Heart Defects Congênital; Tetralogy of Fallot; Aorta, Thoracic; Esophageal, Obstruction; Subclavian, Artery

Authorship

Maciej Michałowski

National Institute of Cardiology – Department of Interventional Cardiology and Angiology, Warsaw – PolôniaNational Institute of Cardiology – Department of Interventional Cardiology and Angiology, Warsaw – PolôniaPolôniaPolôniaNational Institute of Cardiology – Department of Interventional Cardiology and Angiology, Warsaw – Polônia

Pawel Tyczynski

https://orcid.org/0000-0001-6769-0104

Magdalena Lipczynska

National Institute of Cardiology – Department of Congenital Heart Diseases, Warsaw – PolôniaNational Institute of Cardiology – Department of Congenital Heart Diseases, Warsaw – PolôniaPolôniaPolôniaNational Institute of Cardiology – Department of Congenital Heart Diseases, Warsaw – Polônia

Anna Wójcik

Piotr Hoffman

Adam Witkowski

Ilona Michałowska

National Institute of Cardiology – Department of Radiology, Warsaw – PolôniaNational Institute of Cardiology – Department of Radiology, Warsaw – PolôniaPolôniaPolôniaNational Institute of Cardiology – Department of Radiology, Warsaw – Polônia

Mailing Address: Pawel Tyczynski • National Institute of Cardiology – Department of Interventional Cardiology and Angiology – Alpejska 42 Street Warsaw 04628 – Poland E-mail: medykpol@wp.pl

Author Contributions

Conception and design of the research and Writing of the manuscript: Michałowski M, Tyczynski P; Acquisition of data: Tyczynski P, Lipczynska M, Wójcik A, Michałowska I; Critical revision of the manuscript for intellectual content: Hoffman P, Witkowski A, Michałowska I.

Potential Conflict of Interest

No potential conflict of interest relevant to this article was reported.

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Figures (2)

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Figure 1
Patient 1 A) Transthoracic echocardiography, parasternal short axis view. Significant pressure gradient across the pulmonary homograft; B) Cardiac computed tomography, axial plane. White arrow indicates left-sided aortic arch; C) Cardiac computed tomography. Visible calcifications of the pulmonary homograft at the level of the pulmonary valve (red arrow); D) Magnification of panel “B” with a focus on the right aberrant subclavian artery (white arrow) branching-off from the left-sided aortic arch.

Thumbnail

Figure 2
Patient 2 A) Transthoracic echocardiography, parasternal short axis view. Significant regurgitation across the pulmonary homograft; B) Transthoracic echocardiography, parasternal long axis view. Jet of the significant pulmonary regurgitation visible in the right ventricle outflow tract (white arrow); C) Cardiac computed tomography, axial plane. White arrow indicates left-sided aortic arch; D) Cardiac computed tomography. Visible calcifications of the pulmonary homograft at the level of the pulmonary valve (red arrow). E) Magnification of panel “D” with a focus on the right aberrant subclavian artery (white arrow) branching-off from the left-sided aortic arch.

Figure 1 Patient 1 A) Transthoracic echocardiography, parasternal short axis view. Significant pressure gradient across the pulmonary homograft; B) Cardiac computed tomography, axial plane. White arrow indicates left-sided aortic arch; C) Cardiac computed tomography. Visible calcifications of the pulmonary homograft at the level of the pulmonary valve (red arrow); D) Magnification of panel “B” with a focus on the right aberrant subclavian artery (white arrow) branching-off from the left-sided aortic arch.

Figure 2 Patient 2 A) Transthoracic echocardiography, parasternal short axis view. Significant regurgitation across the pulmonary homograft; B) Transthoracic echocardiography, parasternal long axis view. Jet of the significant pulmonary regurgitation visible in the right ventricle outflow tract (white arrow); C) Cardiac computed tomography, axial plane. White arrow indicates left-sided aortic arch; D) Cardiac computed tomography. Visible calcifications of the pulmonary homograft at the level of the pulmonary valve (red arrow). E) Magnification of panel “D” with a focus on the right aberrant subclavian artery (white arrow) branching-off from the left-sided aortic arch.

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Tetralogy of Fallot Associated with Aberrant Right Subclavian Artery. Clinical Implications

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[1] Mailing Address: Pawel Tyczynski • National Institute of Cardiology – Department of Interventional Cardiology and Angiology – Alpejska 42 Street Warsaw 04628 – Poland E-mail: medykpol@wp.pl