Coarctation of the aorta in a young female treated with left subclavian artery to descending thoracic aorta bypass: a case report.

Coarctation of the aorta is a rare congenital abnormality, with an incidence of 6-8% of all congenital heart problems. It is usually diagnosed in childhood during routine clinical examination and adults mostly present with hypertension. Various investigations like transthoracic echocardiography, computed tomography, and magnetic resonance angiography can help with diagnosis. Prognosis depends on age at presentation and the severity of coarctation. Treatment options available are open and endovascular repair. Extra-anatomical bypass is the preferred option in cases with unfavorable anatomy. Long term follow up is required post repair due to risk of restenosis and aneurysm formation. Here is a case in which a young female presented with hypertension, was diagnosed with coarctation of the aorta, and was treated a left subclavian artery to descending thoracic aorta bypass. Her postoperative course was uneventful and she had improvement in hypertension.

A coarctação da aorta é uma anomalia congênita rara, com incidência de 6-8% entre todos os problemas cardíacos congênitos. É geralmente diagnosticada na infância durante o exame clínico de rotina, e os adultos normalmente apresentam hipertensão. Diversas investigações, como ecocardiografia transtorácica, tomografia computadorizada e angiorressonância magnética, podem auxiliar no diagnóstico. O prognóstico depende da idade na apresentação e da gravidade da coarctação. As opções de tratamento disponíveis são o reparo aberto e o reparo endovascular. O bypass extra-anatômico é a opção preferida em caso de anatomia desfavorável. O acompanhamento de longo prazo é necessário após o reparo devido ao risco de reestenose e formação de aneurisma. Descrevemos o caso de uma jovem do sexo feminino que apresentou hipertensão, a qual foi diagnosticada como coarctação da aorta. Foi realizada derivação da artéria subclávia esquerda para a aorta torácica descendente. O pós-operatório transcorreu sem intercorrências, e a paciente apresentou melhora da hipertensão.

Coarctation of the aorta in a young female treated with left subclavian artery to descending thoracic aorta bypass: a case report / Coarctação de aorta em jovem tratada com artéria subclávia esquerda para bypass de aorta torácica descendente: relato de caso

Abstract Coarctation of the aorta is a rare congenital abnormality, with an incidence of 6-8% of all congenital heart problems. It is usually diagnosed in childhood during routine clinical examination and adults mostly present with hypertension. Various investigations like transthoracic echocardiography, computed tomography, and magnetic resonance angiography can help with diagnosis. Prognosis depends on age at presentation and the severity of coarctation. Treatment options available are open and endovascular repair. Extra-anatomical bypass is the preferred option in cases with unfavorable anatomy. Long term follow up is required post repair due to risk of restenosis and aneurysm formation. Here is a case in which a young female presented with hypertension, was diagnosed with coarctation of the aorta, and was treated a left subclavian artery to descending thoracic aorta bypass. Her postoperative course was uneventful and she had improvement in hypertension.

Resumo A coarctação da aorta é uma anomalia congênita rara, com incidência de 6-8% entre todos os problemas cardíacos congênitos. É geralmente diagnosticada na infância durante o exame clínico de rotina, e os adultos normalmente apresentam hipertensão. Diversas investigações, como ecocardiografia transtorácica, tomografia computadorizada e angiorressonância magnética, podem auxiliar no diagnóstico. O prognóstico depende da idade na apresentação e da gravidade da coarctação. As opções de tratamento disponíveis são o reparo aberto e o reparo endovascular. O bypass extra-anatômico é a opção preferida em caso de anatomia desfavorável. O acompanhamento de longo prazo é necessário após o reparo devido ao risco de reestenose e formação de aneurisma. Descrevemos o caso de uma jovem do sexo feminino que apresentou hipertensão, a qual foi diagnosticada como coarctação da aorta. Foi realizada derivação da artéria subclávia esquerda para a aorta torácica descendente. O pós-operatório transcorreu sem intercorrências, e a paciente apresentou melhora da hipertensão.

Midterm Follow-up of Fenestrated and Scalloped Physician-Modified Endovascular Grafts for Zone 2 TEVAR.

Purpose: To investigate the midterm outcomes of scalloped or fenestrated physician-modified endovascular grafts (PMEGs) for zone 2 thoracic endovascular aortic repairs (TEVAR). Materials and Methods: Between November 2013 and May 2019, 54 consecutive patients (mean age 63 years; 41 men) were treated with thoracic PMEGs modified with 7 scallops or 47 fenestrations for the left subclavian artery (LSA). Indications for aortic repair were acute complicated type B aortic dissection (17, 31%), degenerative aneurysm (13, 24%), acute traumatic rupture of the aortic isthmus (9, 16%), post chronic dissection aneurysmal evolution (8, 15%), penetrating aortic ulcer (3, 6%), intramural hematoma (2, 4%), and floating thrombus (2, 4%). Results: Technical success was 94%; 3 (6%) LSAs were unintentionally covered. An intraoperative type Ia endoleak was treated during the index procedure. One (2%) patient suffered spinal cord ischemia, with irreversible bilateral paraplegia. Three (6%) patients experience postoperative minor strokes with full neurological recovery. Four (7%) patients died in the perioperative period; 2 (2%) were due to aneurysm rupture. Mean follow-up was 26±16 months; 15 (28%) patients had at least 3 years of follow-up. Two (4%) type II endoleaks were identified and successfully treated (4% reintervention rate); no other endoleaks were identified. All the LSAs remained clinically and radiologically patent. There were no conversions to open repair, ruptures, retrograde dissection, stent fracture, migrations, or other aortic complications. Conclusion: Scalloped or single-fenestrated PMEGs for the LSA appear to be durable and safe in the midterm. Combined with low periprocedural morbidity and mortality, these results suggest that this approach can be considered as an off-label alternative to extend proximal seal to zone 2 for TEVAR. Further studies with a larger number of patients and long-term outcomes are needed to fully validate this approach.

Arteria subclavia aberrante izquierda, hallazgo infrecuente / Aberrant left subclavian artery, an uncommon variation

Objetivo: La arteria subclavia aberrante (ASA) o arteria lusoria, es una de las malformaciones más comunes del arco aórtico; en especial su variante derecha. Por otro lado, la arteria subclavia aberrante izquierda es más rara. Presentamos un caso infrecuente de un paciente con síndrome deleción 22q11.2 y ASA izquierda con síntomas en extremidad superior izquierda. La ASA izquierda es una condición muy poco frecuente y hay escasa información sobre su tratamiento quirúrgico. La mayoría de las veces, el ASA es asintomática; especialmente en adultos. Si presenta clínica, sus síntomas son disfagia, tos, disnea, claudicación de la extremidad superior. La presencia de sintomatología es una indicación quirúrgica. El tratamiento quirúrgico de una ASA es discutido. Éste va a depender de la anatomía, comorbilidades y experiencia del cirujano.

Objective: The Aberrant Right Subclavian Artery (ARSA) or Lusoria Artery is one of the most common aortic arch malformations. Aberrant Left Subclavian Artery (ALSA) on the other hand, is a much rarer condition. We present an uncommon case of ALSA in a patient with 22q11.2 deletion syndrome with upper limb symptoms and review the treatment options. ALSA is an exceedingly rare condition and information on its surgical treatment is scarce. In most cases the presence of an ALSA is asymptomatic, especially in adults. Some of the most typical symptoms are dysphagia (dysphagia lusoria), cough, dyspnea, claudication of the upper limb extremity. When symptomatic, the patient has indication of surgery. The treatment of an ALSA is still debatable and it depends on the anatomy, comorbidities and surgeon´s experience.

Arco aórtico derecho con subclavia izquierda aberrante y divertículo de Kommerell. Una causa de anillo vascular / Right-side aortic arch with aberrant left subclavian artery and Kommerell's diverticulum. A cause of vascular ring

Resumen: El arco aórtico derecho puede estar asociado a subclavia izquierda aberrante, en algunos casos esta se origina de una dilatación aneurismática que se conoce como divertículo de Kommerell. Se presentan 2 casos de anillo vascular formado por un arco aórtico derecho, subclavia izquierda anómala con divertículo de Kommerell y persistencia del conducto arterioso izquierdo con una revisión de la literatura acerca del desarrollo embriológico y los métodos de imagen que ayudan al diagnóstico de esta rara anomalía vascular.

Abstract: The right-side aortic arch may be associated with aberrant left subclavian artery, in some cases this artery originates from an aneurismal dilation of the aorta called Kommerell's diverticulum. A report is presented on 2 cases of vascular ring formed by a right-side aortic arch, anomalous left subclavian artery, Kommerell's diverticulum and left patent ductus arteriosus. A review the literature was also performed as regards the embryological development and the imaging methods used to help in the diagnosis of this rare vascular anomaly.

[Right-side aortic arch with aberrant left subclavian artery and Kommerell's diverticulum. A cause of vascular ring]. / Arco aórtico derecho con subclavia izquierda aberrante y divertículo de Kommerell. Una causa de anillo vascular.

The right-side aortic arch may be associated with aberrant left subclavian artery, in some cases this artery originates from an aneurismal dilation of the aorta called Kommerell's diverticulum. A report is presented on 2 cases of vascular ring formed by a right-side aortic arch, anomalous left subclavian artery, Kommerell's diverticulum and left patent ductus arteriosus. A review the literature was also performed as regards the embryological development and the imaging methods used to help in the diagnosis of this rare vascular anomaly.