Asploro Journal of Biomedical and Clinical Case Reports
ISSN: 2582-0370
Article Type: Case Report
DOI: 10.36502/2023/ASJBCCR.6285
Asp Biomed Clin Case Rep. 2023 Feb 13;6(1):23-29
A Rare, Relapsing Kommerell Diverticulum – A Case Report
Alejandro Bautista-Pérez-Gavilán1,2, Denya Sánchez-Quintero2, Ana Gilabert-García2, José Emiliano Ríos-Méndez2, Santiago Saenz-Ancira1, Carlos Alberto Villa-Ramirez1,2, Andrea Ibarra-Moreno1, Mariell Rodríguez-Salazar1, Sofía Molina Castillo1, Jorge Luis Bermudez-Gonzalez1,2, Nilda Espinola-Zavaleta1,3, Leonardo Proaño Bernal1, Erick Alexanderson-Rosas1,2iD*
1Department of Nuclear Cardiology, Instituto Nacional de Cardiología Ignacio Chávez, Mexico City, Mexico
2School of Medicine, Universidad Nacional Autónoma de México, Mexico City, Mexico
3Department of Echocardiography, Centro Médico ABC, I.A.P., Mexico City, Mexico
Corresponding Author: Erick Alexanderson-Rosas ORCID iD
Address: National Institute of Cardiology Ignacio Chávez, Juan Badiano N°1, Colonia Sección XVI, Tlalpan, P.C. 14080, Mexico City, Mexico.
Received date: 19 January 2023; Accepted date: 06 February 2023; Published date: 13 February 2023
Citation: Bautista-Pérez-Gavilán A, Sánchez-Quintero D, Gilabert-García A, Ríos-Méndez JE, Saenz-Ancira S, Villa-Ramirez CA, Ibarra-Moreno A, Rodríguez-Salazar M, Castillo SM, Bermudez-Gonzalez JL, Espinola-Zavaleta N, Bernal LP, Alexanderson-Rosas E. A Rare, Relapsing Kommerell Diverticulum – A Case Report. Asp Biomed Clin Case Rep. 2023 Feb 13;6(1):23-29.
Copyright © 2023 Bautista-Pérez-Gavilán A, Sánchez-Quintero D, Gilabert-García A, Ríos-Méndez JE, Saenz-Ancira S, Villa-Ramirez CA, Ibarra-Moreno A, Rodríguez-Salazar M, Castillo SM, Bermudez-Gonzalez JL, Espinola-Zavaleta N, Bernal LP, Alexanderson-Rosas E. This is an open-access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium provided the original work is properly cited.
Keywords: Kommerell Diverticulum, Congenital Vascular Disease, Aortic Regurgitation, Vascular Surgery, Aortitis
Abstract
Kommerell diverticulums are an uncommon congenital vascular disease involving an aberrant origin of the right or left subclavian artery and a dilation of its root. Here, we present the case of a 44-year-old woman with a relapse of a surgically repaired aberrant subclavian artery with a Kommerell diverticulum.
Past Medical History
The patient had a history of hypothyroidism, asthma, migraine and poorly controlled hypertension. She had been admitted to the emergency room two years prior due to piercing precordial pain with an intensity of 10/10 AVS that irradiated all the way to the interscapular region. An aortic regurgitation murmur II/IV in intensity was heard. An angiotomography was performed, revealing an aberrant right subclavian artery with a KD along with signs of imminent rupture. The defects were surgically repaired and the patient remained asymptomatic during the following eighteen months.
History of Presentation
The patient was admitted to the emergency department due to acute onset intense chest pain. She had a history of a surgically-corrected aberrant right subclavian artery associated with a Kommerell diverticulum (KD). There were no complications during or after initial intervention, and she had been asymptomatic for eighteen months until that day. She was admitted to our center’s emergency department for further evaluation.
Differential Diagnosis
Due to the patient’s clinical manifestations and past medical history, there was a strong suspicion of a relapsing KD. Other diagnoses that could possibly explain the acute and intense chest pain referred by the patient such as angina or acute myocardial infarction were ruled out after performing transthoracic echocardiography (TTE); which revealed severe aortic regurgitation (Fig-1a and Fig-1b). Follow-up with transesophageal echocardiography (TEE) also showed severe aortic insufficiency (Carpenter type 1) with important dilation of the aortic ring (Fig-1c).
Fig-1: 2D TTE with continuous Doppler and 3D TEE with color
Investigations
A magnetic resonance imaging study (MRI) was performed, demonstrating a newly formed fusiform aneurysm at the location of the previous KD measuring 59 mm in diameter (Fig-2). The study also showed early gadolinium enhancement, suggesting acute inflammation in the ascending aorta. A Positron Emission Tomography (PET) study with 18-fluorodeoxyglucose (18-FDG) was performed, exhibiting abnormally high metabolic activity in the wall of the ascending aorta, thus confirming the diagnoses of aortitis and a relapsing KD (Fig-3). As a complementary study, an aortogram was performed, showing clear evidence of the relapsing KD in the proximal segment of the aberrant right subclavian artery (Fig-4).
Fig-2: Cardiac MRI study where the recurrent Kommerel’s diverticulum was first seen
Fig-3: PET Study with 18-FDG
Fig-4: Aortogram
Management
Due to the patient’s clinical presentation and current findings, surgical intervention was performed. Exclusion of the aneurysm, three-staged revascularization of the supra-aortic vascular branches, and aortic valve replacement with a bioprosthetic valve were carried out successfully and the patient recovered during the following months.
Discussion
KDs are uncommon congenital vascular abnormalities characterized by a dilation of the proximal segment of an aberrant right or left subclavian artery [1]. This patient had an aberrant right subclavian artery, which occurs when the right portion of a double aortic arch involutes into the segment between the right common carotid artery and the right subclavian artery. This results in the right subclavian artery arising as the fourth and last great branch of an otherwise normal aorta and subsequently passing to the right side of the body; behind and in close relation to the esophagus and trachea (Fig-5). We now know that even if 80% of the cases are retroesophageal, some of them can be located between the esophagus and the trachea, or even anterior to the trachea [2]. Additionally, around half of the patients with this condition have a higher risk for the development of aortic aneurysms, ruptures or dissections than normal individuals [3].
Fig-5: Aberrant right subclavian origin with a KD
Until nowadays, KDs were believed to be the result of a failure of regression of the primitive fourth dorsal arch that gives way to an abnormal remnant of this embryonic structure [4]. Nonetheless, there is now an ongoing debate, for in new literature, KDs are described as degenerative aneurysmal changes in the root of the aberrant right subclavian artery and not a congenital anomaly [5]. This condition was first described in 1936 by Burckrad Kommerell, a German radiologist that identified the anomaly after performing an esophagogram to a patient that exhibited a compressive and pulsatile mass posterior to the esophagus [1].
This condition has a prevalence of approximately 0.7-2% when found with an aberrant right subclavian artery, and of 0.04-0.4% when related to an aberrant left subclavian artery [6], the first type being the fourth most common congenital anomaly of the aortic arch [7]. Moreover, around 40% of the patients with an aberrant right subclavian artery present with a KD [8]. Most patients with KDs are asymptomatic, however, some of them (approximately 5%) can present a wide variety of clinical manifestations such as chest pain, coughing, dysphagia, exertional dyspnea, a difference in blood pressure between both upper limbs, and ischemia of the upper limb of the affected side due to occlusive complications [9]. In the most severe of scenarios, a spontaneous rupture or dissection of the diverticulum may occur, affecting around 6-19% of the cases with a 50% mortality rate. Another relevant but very rare complication is the formation of an esophageal or tracheal fistula, generated by either constant compression of the aberrant subclavian artery or procedures such as placement of endotracheal or nasogastric tubes. Nonetheless, the most common iatrogenic complication with these patients is puncture or laceration of the aberrant subclavian artery during central venous catheterization [2]. Finally, aortitis is also a very unusual complication of this pathology, but it can lead to potentially adverse situations, just as the recurrence of the aneurysm after previous surgical correction [10], as exemplified in the present case.
Currently, the best tools available for the diagnosis of KDs are MRI and computed tomography (CT), since they have the ability to accurately observe and characterize vascular anomalies, their relationship to nearby organs or structures, and possible coexisting malformations [11]. Additionally, incidental findings in other diagnostic modalities such as chest x-ray or esophagogram in conjunction with symptoms compatible with esophageal compression should lead to the diagnostic suspicion of a KD [12].
There are currently no specific guidelines regarding the treatment of this condition. Nonetheless, surgical interventions should be considered in symptomatic patients or in those with a diverticulum diameter bigger than 30 mm [13]. Surgical approach should be determined by the anatomic features of the KD, clinical presentation, comorbidities, past medical history and hemodynamic state to ensure an optimal treatment. Treatment must address the aneurysm as well as revascularization of the upper extremity [14].
Surgical repair is performed with the patient in supine position, with the right hemithorax elevated. The great blood vessels and the distal part of the aberrant left subclavian artery are exposed through a median sternotomy. Cardiopulmonary bypass is performed through the femoral artery and/or cannulation of the ascending aorta and bicaval drainage with pulmonary artery ventilation. Then, reconstruction of the aortic arch is performed in the following order: right subclavian artery, right common carotid artery and finally the left common carotid artery. After reconstruction, thoracotomy is performed in the 3rd, 4th or 5th intercostal space through which the diverticulum is exposed. After cross-clamping, the descending aorta is transected and anastomosed to the distal end of the graft. Finally, the graft is anastomosed with the ascending aorta [15].
Open surgical repair is associated with high morbidity as a major thoracic surgery. The procedure involves resection of the diverticulum and revascularization of the aberrant subclavian artery, which may be performed through a thoracotomy or median sternotomy [16].
Revascularization with the aortic arch can be performed through a sternotomy or extra-anatomically through a supraclavicular approach. Hybrid repair may be performed using an aortic endograft and extra-anatomic bypass in order to reduce morbidity associated with thoracotomy or sternotomy and aortic cross-clamping [17].
Many surgical approaches for repair of a KD have been described. We have briefly summarized open, hybrid and endovascular techniques in Table-1.
Table-1: Brief summary of the characteristics of the surgical techniques used to repair KDs
Follow-Up
After surgery, the patient was subsequently admitted to the intensive care unit for three days, where she was intubated and under close surveillance. She improved steadily during the following weeks, prompting hospital discharge. She remained asymptomatic and recovered steadily during the following months.
Conclusions
KDs are uncommon congenital heart diseases involving the aortic arch and should be suspected in patients with symptoms compatible with esophageal compression and/or chest pain. Multimodal approach to this condition is vital to ensure the best outcomes for patients.
Conflict of Interest
The authors have read and approved the final version of the manuscript. The authors have no conflicts of interest to declare.
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