|Year : 2023 | Volume
| Issue : 1 | Page : 62-64
Tetralogy of Fallot with Right Aortic Arch and Long-Segment Coarctation of Aorta
Anil Kumar Singhi1, Soumya Kanti Mohapatra1, Ejaz Ahmed Bari2
1 Department of Pediatric Cardiology, Medica Superspecialty Hospital, Kolkata, West Bengal, India
2 Department of Radiodiagnosis, Medica Super Specialty Hospital, Kolkata, West Bengal, India
|Date of Submission||24-May-2022|
|Date of Acceptance||10-Jul-2022|
|Date of Web Publication||03-Oct-2022|
Dr. Anil Kumar Singhi
Senior Consultant and Head, Department of Pediatric and Congenital Heart Disease, Medica Superspeciality Hospital, 127, Mukundapur, E.M Bypass, Kolkata - 700 099, West Bengal
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Singhi AK, Mohapatra SK, Bari EA. Tetralogy of Fallot with Right Aortic Arch and Long-Segment Coarctation of Aorta. J Indian Acad Echocardiogr Cardiovasc Imaging 2023;7:62-4
|How to cite this URL:|
Singhi AK, Mohapatra SK, Bari EA. Tetralogy of Fallot with Right Aortic Arch and Long-Segment Coarctation of Aorta. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2023 [cited 2023 May 29];7:62-4. Available from: https://jiaecho.org/text.asp?2023/7/1/62/357813
A 2-year-old girl presented with cyanosis and poor weight gain. Her oxygen saturation was 84%. Clinical evaluation revealed a pulse rate of 80/min and normal first heart sound with a single second heart sound. There was a long systolic murmur best heard at the second left intercostal space. Chest X-ray showed a cardiothoracic ratio of 0.5 with features of reduced pulmonary blood flow. Echocardiogram revealed a large conoventricular septal defect (bidirectional shunt) and severely narrowed right ventricular outflow tract. The pulmonary stenosis was at the infundibular, annular, and supravalvular levels with the peak gradient >100 mmHg. The branch pulmonary arteries were confluent but hypoplastic [Figure 1]a, [Figure 1]b, [Figure 1]c, [Figure 1]d, [Figure 1]e, [Figure 1]f and [Video 1]. The initial diagnosis was in favor of Tetralogy of Fallot (TOF). The aortic arch was right sided. The descending thoracic aorta showed significant flow acceleration. The echocardiographic window was suboptimal in the suprasternal and parasternal views. Subcostal imaging showed descending thoracic aorta with flow acceleration and diastolic tailing. The pulsed-wave Doppler at the level of the diaphragm showed nonpulsatile flow. Both left and right ventricles were significantly hypertrophied [Figure 2]a, [Figure 2]b, [Figure 2]c, [Figure 2]d, [Figure 2]e, [Figure 2]f and [Video 2]. Computed tomographic angiogram with three-dimensional reconstruction was done to delineate the anatomy. It showed right-sided aortic arch and long segment hypoplasia of descending thoracic aorta with coarctation. The branch pulmonary arteries were good sized [Figure 3]a, [Figure 3]b, [Figure 3]c, [Figure 3]d and [Video 3]. The anatomy was discussed in a combined meeting with the surgical team and the plan was to do the hybrid correction. Cardiac catheterization with stenting of the long-segment coarctation of aorta with Cook Formula stent (Cook Medical LLC, Bloomington, IN, USA), followed by intracardiac repair was decided. The Cook Formula stent was planned as it could be post dilated up to 20 mm, which represents adult descending aortic diameter. During the planning period for the repair, the coronavirus epidemic struck. Multiple family members of the child developed severe coronavirus disease, with some even succumbing to it. In view of this, the parents of the child requested to postpone the surgery, and eventually the child was lost to follow-up.
|Figure 1: Echocardiogram in (a) apical five chamber view and (b) parasternal long axis view showing large subaortic ventricular septal defect (arrow). The hypoplastic RVOT with flow acceleration (arrow) is seen in (c) subcostal short axis view and (d) parasternal short axis view (arrow). (e) The hypoplastic branch pulmonary arteries are seen in the short-axis view. (f) The continuous-wave Doppler interrogation of the RVOT shows a significant gradient. RVOT: Right ventricular outflow tract|
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|Figure 2: Echocardiogram in (a) suprasternal long-axis view and (b) subcostal short-axis view showing turbulence (arrow) in the aortic arch and descending thoracic aorta. Echocardiogram in (c) subcostal short-axis view and (f) parasternal short-axis view showing evidence of biventricular hypertrophy. (d) Pulsed-wave Doppler of the aorta at the level of the diaphragm showing nonpulsatile flow. (e) Continuous wave Doppler at the level of thoracic aorta showing significant aortic gradient with diastolic tailing|
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|Figure 3: CT angiogram in (a) lateral view showing long segment hypoplastic descending thoracic aorta (arrow) and (b) narrowed right ventricular outflow tract (star) and confluent branch pulmonary artery seen in the oblique view. Three-dimensional reconstruction of the CT angiogram showing severe long-segment coarctation of the aorta in (c) posterior and (d) oblique views. CT: Computed tomographic|
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[Additional file 1]
Video 1: Echocardiogram in (a) apical five-chamber view and (b) parasternal long-axis view showing large subaortic ventricular septal defect. The hypoplastic RVOT with flow acceleration is seen in (c) subcostal short-axis view and (d) parasternal short-axis view. (e) The hypoplastic branch pulmonary arteries are seen in the short-axis view. RVOT: Right ventricular outflow tract.
[Additional file 2]
Video 2: Echocardiogram in (a) suprasternal long-axis view and (b) subcostal short-axis view showing turbulence in the aortic arch and descending thoracic aorta. Echocardiogram in (c) subcostal short-axis view and (d) parasternal short-axis view showing evidence of biventricular hypertrophy.
[Additional file 3]
Video 3: (a-c) Three-dimensional reconstruction of computed tomographic angiogram in different views delineating the hypoplastic descending aorta and pulmonary artery anatomy.
A combination of TOF and obstruction of the left ventricular outflow tract is a very rare anomaly. This defies Rudolph's theory of flow-related development of great arteries in fetal life. Very few cases are reported in the literature. Prabhu et al. reported the presence of coarctation in 0.04% of patients with the tetralogy. The nature of the coarctation varies with the sidedness of the aortic arch. In the left-sided aortic arch, the coarctation is usually seen distal to the left subclavian artery. In TOF and right aortic arch, the coarctation is seen distal to the right common carotid artery as part of a more complex anomaly of the outflow tracts. Discrete narrowing of the arch is more commonly reported in TOF with the left aortic arch. Diffuse hypoplasia of the descending thoracic aorta in TOF and the right aortic arch as seen in the index patient is extremely rare. Preoperative diagnosis of this entity is very important to decide on the staged or simultaneous repair of the lesions. Initial clinical evaluation and detailed echocardiogram help to pick up the diagnosis. Computed tomographic angiography helps to delineate the anatomy and plan the intervention.,
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the parent has given consent for images and other clinical information to be reported in the journal. The parent understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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Prabhu S, Kasturi S, Mehra S, Tiwari R, Joshi A, John C, et al.
The aortic arch in tetralogy of Fallot: Types of branching and clinical implications. Cardiol Young 2020;30:1144-50.
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[Figure 1], [Figure 2], [Figure 3]