Project description:Persistent fifth aortic arch (PFAA) is a rare congenital cardiovascular anomaly of the aortic arch, which can occur independently but is frequently associated with other cardiovascular malformations including patent ductus arteriosus (PDA), interrupted aortic arch (IAA), pulmonary atresia, tetralogy of Fallot, and transposition of the great vessels. PFAA can be classified into three different types according to different abnormal vascular connections (type A, B and C). We report an infant diagnosed with PFAA along with interrupted fourth aortic arch (type B) and PDA. Several surgical methods have been delivered to patients diagnosed with PFAA. In our case, the correction of coarctation PFAA and the ligation of PDA were performed without cardiopulmonary bypass through left lateral thoracotomy. We removed the coarctation part of the PFAA, and then performed the end-to-end anastomosis between the fifth aortic arch and the descending aorta. Despite the stenosis at the site of anastomosis was observed 10 months after the operation, the patient resulted in good recovery by once balloon dilation procedure. Thus, we recommend it is more appropriable to select this procedure in the surgical treatment of PFAA for those patients whose fourth aortic arches were interrupted with fifth aortic arches well developed. Our experience can provide a beneficial reference for future cases.
Project description:BackgroundPersistent fifth aortic arch (PFAA) is a rare anomaly often associated with aortic coarctation or interruption, and various surgical techniques for this anomaly have been reported. Herein, we show a case of an infant with PFAA and severe aortic coarctation.Case presentationA 41-day-old female infant was admitted for sustained fever. Initially, the patient was diagnosed with bacterial meningitis, and echocardiography showed PFAA with severe aortic coarctation. Because the patient presented progressive oliguria and metabolic acidosis, she was transferred for emergency cardiac surgical intervention. The aortic arch was reconstructed using end-to-side anastomosis between the fifth aortic arch and the descending aorta without any artificial conduit or patching material.ConclusionsPFAA with aortic coarctation can be repaired by various surgical methods. Among them, our surgical approach is easy and effective, has growth potential, and an additional surgery is not needed.
Project description:Aortic pseudocoarctation is a rare congenital anomaly characterized by elongation and deformity of the aortic arch and is known to be associated with aneurysmal formation. Several studies unite to say it leads to a surgical sanction as soon as symptomatic or associated with aneurysms of the aortic arch. Our patient is a 12 years old boy, followed since birth for a little tight pseudocoarctation with a cervical aortic arch and transverse aortic arch hypoplasia. Close clinical and paraclinical monitoring including angioscans, showed the gradual enlargement of the superior mediastinum, in relation with the appearance of three aneurysms of the aortic arch. The intervention, performed by sternotomy, has consisted of the resection of the aneurysmal area and the interposition of a Dacron tube to repair the aortic arch and the reimplantation of the left subclavian artery into the left carotid artery. The postoperative course was uneventful. Management of pseudocoarctation associated with cervical aortic arch and aneurysms remains surgical. Close monitoring of patients with pseudocorctation, seems to be essential to avoid fatal complications such as aneurysmal rupture.
Project description:Persistent 5th aortic arch is a rare cardiac anomaly that is usually surgically corrected during infancy or early childhood if it is associated with coarctation of the aorta. Here, we report an adult with coarctation of the 5th aortic arch who was successfully treated by stent implantation. An asymptomatic 32-year-old woman presented with hypertension and a significant arm-leg difference in pressure. On suspicion of coarctation of the aorta, a chest computed tomography was performed, leading to a diagnosis of an interrupted 4th aortic arch with coarctation of a persistent 5th aortic arch. Percutaneous catheter intervention using a PALMAZ large stent dilated to 12 mm resulted in a minimal peak-to-peak pressure gradient. The patient was discharged home after a 2-day monitoring without hypertension and arm-leg blood pressure difference. She remained normotensive with a patent aortic arch on echocardiography performed 10 months after treatment. As for simple coarctation of the aorta, stent implantation was feasible and effective in an adult patient with coarctation of the 5th aortic arch.
Project description:We report stenting of coarctation of an angulated right circumflex aortic arch (RCAA) using four Cheatham Platinum stents in a child with Goldenhar syndrome. Difficulties in measuring the accurate length of the curved and narrowed transverse aortic arch marked discrepancy between the luminal diameters of the long narrow transverse arch and wide descending thoracic aorta, increased displacement force caused by the 90° bend between the two parts resulted in repeated stent migrations. We discuss the tips to avoid distal stent migration in the setting of an angulated RCAA.
Project description:The aortic arch repair is one of the most complex surgeries and carries a high risk of complications as well as mortality. Since 1975, when the arch repair was first done by Randall B. Griepp using hypothermic circulatory arrest, many new technologies were introduced. But even with the use of antegrade and retrograde perfusion techniques and improvement of surgical techniques and grafts, the rate of mortality, cerebral, spinal, and visceral damage was much higher as compared to any other cardiac surgeries. With further developments aimed at less invasive approaches, thoracic endovascular aortic repair (TEVAR) along with de-branching of supra-aortic vessels or the frozen elephant trunk was introduced. Here, in this article, we review the myriad of approaches to the aortic arch and have come to a conclusion that while traditional open surgery is considered as the gold standard for treatment of extensive aortic arch pathologies, one school of thought suggests hybrid techniques such as the frozen elephant trunk and aortic arch vessel de-branching as more appropriate procedures for high-risk patients, where co-morbidities may contraindicate cardiopulmonary bypass and longer operative times required for traditional repair. No randomized trials are present to compare between open and hybrid or endovascular procedure in normal or high-risk patients. The meta-analysis of most of the studies defines open surgery as the gold standard for arch pathology because the hybrid procedures did not provide any proven survival benefits or decrease in stroke rate and spinal ischemia when compared to open surgery in early, mid, or long-term results.
Project description:BackgroundThoracic aortic arch aneurysms (TAAs) can be a life-threatening condition due to the potential risk of rupture. Treatment is recommended when the risk of rupture is greater than the risk of surgical complications. Depending on the cause, size and growth rate of the TAA, treatment may vary from close observation to emergency surgery. Aneurysms of the thoracic aorta can be managed by a number of surgical techniques. Open surgical repair (OSR) of aneurysms involves either partial or total replacement of the aorta, which is dependent on the extent of the diseased segment of the aorta. During OSR, the aneurysm is replaced with a synthetic graft. Hybrid repair (HR) involves a combination of open surgery with endovascular aortic stent graft placement. Hybrid repair requires varying degrees of invasiveness, depending on the number of supra-aortic branches that require debranching. The hybrid technique that combines supra-aortic vascular debranching with stent grafting of the aortic arch has been introduced as a therapeutic alternative. However, the short- and long-term outcomes of HR remain unclear, due to technical difficulties and complications as a result of the angulation of the aortic arch as well as handling of the arch during surgery.ObjectivesTo assess the effectiveness and safety of HR versus conventional OSR for the treatment of TAAs.Search methodsThe Cochrane Vascular Information Specialist searched the Cochrane Vascular Specialised Register, CENTRAL, MEDLINE, Embase, CINAHL and AMED databases and World Health Organization International Clinical Trials Registry Platform and ClinicalTrials.gov trials registers to 22 March 2021. We also searched references of relevant articles retrieved from the electronic search for additional citations.Selection criteriaWe considered for inclusion in the review all published and unpublished randomised controlled trials (RCTs) and controlled clinical trials (CCTs) comparing HR to OSR for TAAs.Data collection and analysisTwo review authors independently screened all titles and abstracts obtained from the literature search to identify those that met the inclusion criteria. We retrieved the full text of studies deemed as potentially relevant by at least one review author. The same review authors screened the full-text articles independently for inclusion or exclusion.Main resultsNo RCTs or CCTs met the inclusion criteria for this review.Authors' conclusionsDue to the lack of RCTs or CCTs, we were unable to determine the safety and effectiveness of HR compared to OSR in people with TAAs, and we are unable to provide high-certainty evidence on the optimal surgical intervention for this cohort of patients. High-quality RCTs or CCTs are necessary, addressing the objective of this review.
Project description:The double-barreled aorta connecting the ascending aorta and descending aorta caudal to the normal fourth aortic arch has fascinated the interests of cardiac morphologists for over a century. This condition is commonly associated with coarctation. While the controversies surrounding the embryology of the double-barreled aorta have settled down, we present a case-based illustration of the technical aspects of coarctation stenting peculiar to this condition.