Project description:Isolation of the right subclavian artery (RSCA) is rare, and this finding in association with d-transposition of the great arteries (d-TGA) is extremely unusual. We present a case of an isolated RSCA in a newborn with d-TGA in whom the clinical presentation was diagnostic. We discuss the imaging modalities used to confirm the diagnosis, the embryological basis of the finding, and the surgical repair.
Project description:Graphical abstract Highlights • Isolated RSCA from the PA is a rare finding in d-TGA.• Atypical differential cyanosis in d-TGA may indicate abnormal aortic branching.• Fetal imaging can provide evidence of an isolated RSCA.
Project description:Right aortic arch with Kommerell's diverticulum is a very rare situation. Surgical treatment is recommended for symptomatic patients or asymptomatic patients with a large diverticulum. However planning the strategy of operation is difficult without a 3D imaging. We report a case of a 57-year-old man with right aortic arch, Kommerell's diverticulum, and aberrant left subclavian artery. After a 3D-CT imaging, the patient underwent descending aortic replacement without reconstruction of aberrant left subclavian artery. After operation, there was no signs or symptoms of ischemia of the left arm. If the reconstruction of the aberrant subclavian artery was too difficult, closing its orifice is an acceptable decision. It has been found advantageous because of a decrease blood loss and a shorter cardiopulmonary bypass duration. If an ischemia of the arm is noticed, additional reconstruction will have to be considered. 3D-CT imaging was very useful to have a proper orientation and plan for the operative strategy.
Project description:BackgroundThe coexistence of a right-sided aortic arch (RAA), an aberrant left subclavian artery (ALSA), and a patent ductus arteriosus (PDA) is a rarely seen vascular ring anomaly. There is currently no general guideline consensus on the management and follow-up of this congenital defect, posing a challenge to the clinicians. At this point, the heart team plays a critical role in the management of the disease.Case summaryIn the present case, a 25-year-old male patient was presented to the outpatient clinic with dyspnoea and fatigue. A transthoracic echocardiography revealed PDA with a left-to-right shunt. To evaluate the anatomy thoroughly, a thoracic computed tomographic angiography was performed and showed PDA accompanying ALSA and RAA. The patient was evaluated by the Heart Team, and a percutaneous closure of PDA was recommended due to signs of left ventricular volume overload. The closure was successfully performed with Amplatzer vascular plug II. At follow-up, the patient was free of symptoms.DiscussionClinicians should be aware of the potential concomitant lesions during the diagnostic work-up. In selected patients, percutaneous closure of PDA may be the first-line therapy in experienced centres.
Project description:We report the rare case of a right aortic arch associated with agenesis of the left internal carotid artery. A 75-year-old woman with a medical history of tetralogy of Fallot presented with dizziness. Magnetic resonance angiography revealed agenesis of the left internal carotid artery in addition to a previously diagnosed right aortic arch. The left common carotid artery was present, but it was thin. Computed tomography showed the absence of the left carotid canal. The left anterior cerebral artery was fed via the anterior communicating artery. The left middle cerebral artery was fed via a thickened posterior communicating artery originating from the left posterior cerebral artery. Although a right aortic arch and agenesis of the internal carotid artery are both very rare, association of the two conditions may occur. Both anomalies depend on the abnormal regression of the dorsal aorta during embryonic development. In such a situation, the presence of other anomalies in the cardiac or central nervous system should be taken into consideration.
Project description:The frozen elephant trunk (FET) technique for total aortic arch replacement extends repair into the proximal portion of the descending thoracic aorta. Several techniques and modifications of total arch replacement have been described in the literature, and many of these iterations are related to facilitating the distal anastomosis while preserving flow to the left subclavian artery (LSCA), as well as maintaining posterior circulation of the brain via the vertebral artery, by reducing the circulatory arrest time during reconstruction. Because of the LSCA's posterior and deep anatomic location in the chest, particularly in obese patients, this revascularization is often challenging; additional concerns regarding LSCA revascularization include patients with large aortic arch aneurysms, those with dissected or calcified arteries, and reoperation. A careful plan for reconstruction is necessary. Whether revascularization is performed preoperative, intraoperative, or postoperatively, every effort should be made to include the left subclavian artery as part of the operational approach. Revascularization techniques include reimplantation as part of the island patch or direct anastomosis, stenting, bypass, transposition or a hybrid approach. The importance of maintaining circulation of the LSCA cannot be overstated. Preserving flow to the spinal cord via collaterals minimizes the risk of cord injury during FET procedure. In patients with a patent left internal mammary artery bypass, left arm arteriovenous fistula for hemodialysis, dominant circulation, or direct aortic origin of the left vertebral artery, revascularization is necessary as well. In the case of initial sacrifice, arm claudication or steal syndrome usually dictates delayed extra-anatomic revascularization in the postoperative period.
Project description:We report a 6-month-old female infant with deletion of chromosome 22q11.2 (DiGeorge/VFS TUPLE 1), normal atrial arrangement with concordant atrioventricular connection, pulmonary atresia, large subaortic ventricular septal defect, diminutive native pulmonary arteries, a characteristic weird-shape right aortic arch with arch-on-arch appearance and figure of 8 configuration. We presented the cardiac computed tomographic angiographic and cardiac angiographic features. Using Autodesk 3ds Max 2018 software, we explained and illustrated the speculative embryologic etiology of this bizarre aortic archanomaly with the extensive abnormal remodeling of the left brachiocephalic artery, based on a "five-embryonic aortic arches" concept. As to the best of the authors' knowledge, this is the first report of a genetically confirmed case of DiGeorge syndrome and an exceedingly rare type of right aortic arch anomaly with embryologic explanation according to the "five-embryonic-aortic-arches" concept. It seems that the constellation of pulmonary atresia, bizarreshaped right aortic arch due to abnormal development of the aortic sac, and abnormal remodeling of the left brachiocephalic artery may be strongly suggestive of DiGeorge syndrome.