- Email: [email protected]
Natural history and management of Kommerell's diverticulum in a single tertiary referral center
From the Society for Clinical Vascular Surgery
Natural history and management of Kommerell’s diverticulum in a single tertiary referral center Young Erben, MD,a Adam J. Brownstein, BA,b Camilo A. Velasquez, MD,b Yupeng Li, PhD,c John A. Rizzo, PhD,b Hamid Mojibian, MD,d Maryam Tanweer, MD,b Mohammad A. Zafar, MBBS,b Bulat A. Ziganshin, MD, PhD,b and John A. Elefteriades, MD,b Jacksonville, Fla; New Haven, Conn; and Glassboro, NJ
ABSTRACT Objective: The Kommerell diverticulum (KD) is an extremely rare developmental abnormality of the aorta related to an aberrant subclavian artery (ASCA). The objective of our study was to review the natural history of KD and ASCA using our single-center experience in diagnosing and managing KD and ASCA. Methods: A retrospective review of the Yale radiological database from January 1999 to December 2016 was performed. Only patients with KD/ASCA and a computed tomography (CT) scan of the chest were selected for review. The primary goal was to examine the natural history of KD and ASCA and the secondary goals were to review the management and outcomes of those patients treated for KD and ASCA. Results: There were 75 patients with KD/ASCA identified, with a mean age of 63 6 19 years; 49 were female (65%). On CT scans, left- and right-sided aortas were present in 47 (63%) and 28 (37%) patients. A right ASCA or a left ASCA were present in 47 (63%) and 28 (37%) patients. Six patients were symptomatic on presentation. Symptoms included dysphagia, chest or back pain, and emboli to the fingers. The mean KD diameter was 21.8 6 6.0 mm and the distance to the opposite aortic wall (DAW) was 48.3 6 10.8 mm. Sixty-six patients were followed for a mean of 31.7 6 32.5 months. One patient ruptured without repair. Nine patients underwent operative intervention, including eight open and one endovascular repair. Complications from operative intervention included ischemic stroke with hemorrhagic transformation, deep vein thrombosis and pneumonia. The mean growth rate for KD and DAW was 1.45 6 0.39 mm/year and 2.29 6 0.47 mm/year, respectively. On multivariable regression analysis, hypertension was a predictor of growth of DAW (P ¼ .03). Conclusions: KD is uncommon and shows a female predominance. The diverticulum grows, albeit slowly (KD and DAW growth rates of 1.45 6 0.39 mm/year and 2.29 6 0.47 mm/year). Most patients are asymptomatic, but dysphagia, chest/ back pain, and distal emboli may occur. Rupture is rare. Symptomatic patients should be operated. Asymptomatic patients can be followed with serial CT scans. (J Vasc Surg 2019;-:1-8.) Keywords: Aberrant subclavian artery; Open and endovascular repair; Kommerell diverticulum
Bayford1 in 1787 described an autopsy case of “obstructed deglutition” caused by a right aberrant subclavian artery (ASCA) passing posterior to the esophagus. The term dysphagia lusoria was coined, and the aberrant ASCA termed “arteria lusoria,” by Arkin.2 However, it was not until 1936 that the first clinical diagnosis of dysphagia lusoria caused by an ASCA and an aortic diverticulum compressing the esophagus was made in a living subject by Dr Burkhard F. Kommerell.3 Kommerell diverticulum (KD) is a persistent remnant of the fourth primitive dorsal arch owing to failed regression.4 In the case of left aortic arch with KD, the
right dorsal aorta involutes proximal to the right subclavian artery and leaves the subclavian artery attached to the left descending aorta via the distal portion of the right dorsal aorta. In contrast, in patients with the right aortic arch with KD, the left dorsal arch diminishes between the subclavian and carotid arteries and allows the right subclavian to originate from the remnant right dorsal arch.5 Salomonowitz et al6 classified the KD according to pathogenesis into three types: (1) KD in left aortic arch with right ASCA, (2) KD in the right aortic arch with left ASCA, and (3) aortic diverticulum without ASCA.
From the Division of Vascular and Endovascular Surgery, Mayo Clinic, Jackson-
Correspondence: John A. Elefteriades, MD, Aortic Institute at Yale-New Haven,
villea; the Aortic Institute at Yale-New Haven Hospital, Yale School of Medici-
Yale University School of Medicine, 789 Howard Ave, Clinic BuildingeCB317,
ne,b and the Section of Vascular Interventional Radiology, Yale School of Medicine,d New Haven; and the Department of Political Science and Economics, Rowan University, Glassboro.c A.J.B. was supported by the Richard K. Gershon, M.D., Student Research Fellowship and the Yale University School of Medicine Medical Student Research Fellowship. Author conflict of interest: none.
New Haven, CT 06519 (e-mail: [email protected]). The editors and reviewers of this article have no relevant financial relationships to disclose per the JVS policy that requires reviewers to decline review of any manuscript for which they may have a conflict of interest. 0741-5214 Copyright Ó 2019 by the Society for Vascular Surgery. Published by Elsevier Inc. https://doi.org/10.1016/j.jvs.2019.08.260
Presented at the Forty-sixth Annual Symposium of the Society for Clinical Vascular Surgery, Las Vegas, Nev, March 17-21, 2018.
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There is a paucity of data available regarding the natural history and management of ASCA and KD in the adult population, which is mainly due to the rarity of this condition and the low incidence of symptoms, reported at 5%.7 Some groups recommend surgical repair in all encountered KD owing to a high rupture and dissection rate of up to 53%.8,9 To date, only Ota et al10 proposed a size criterion for repair of the KD, using the KD diameter itself and the distance to the opposite aortic wall (DAW) (Fig 1). The minimal size recommended for repair includes a KD of 30 mm and a DAW of 50 mm.10-12 The objective of our study is to review the natural history of KD and ASCA using our single-center experience diagnosing and managing KD and ASCA. Furthermore, we sought to review the operative management and outcomes of those patients undergoing surgical intervention for KD and ASCA.
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ARTICLE HIGHLIGHTS d
d
d
Type of Research: Retrospective cohort study Key Findings: In 75 patients with a Kommerell diverticulum (KD) and aberrant left subclavian artery, 65% were females; right-sided aorta and right aberrant subclavian artery were present in 63%. Only six patients were symptomatic. One of 66 KDs that were followed ruptured. Open repair was performed in eight of nine patients, with complications of ischemic stroke, deep vein thrombosis, and pneumonia. Hypertension predicted growth. Take Home Message: KD is uncommon and shows a female predominance. Most patients are asymptomatic, rupture is rare. Symptomatic patients should be operated. Asymptomatic patients can be followed with serial computed tomography scans.
METHODS Patient cohort. The Yale radiology computed tomography (CT) database was searched with specific search terms including KD and/or ASCA for the period from January 1, 1999, to December 31, 2016. Patients were also identified via International Classification of Diseases, Ninth Edition, disease codes Q24.48 (anomalous congenital origin of the subclavian artery) and Q24.47 (persistent right aortic arch). Data collection included details of each patient’s clinical presentation, comorbidities, management, and follow-up as identified via retrospective chart review (Table I). Consistent with prior reports, symptomatic patients were defined as those with dysphagia, chest pain, dyspnea, and gastroesophageal reflux disease without other evident etiology for these symptoms.12 The primary goal was to review the natural history of KD and ASCA, including growth rate and risk of rupture, in those patients who were observed over time. The secondary goals were review of the management and outcomes of those patients treated surgically for KD and ASCA. Individual patient consent for study inclusion was not obtained or required, and this study received approval from the Yale Institutional Review Board (HIC# 1411014866) as a retrospective chart review. Imaging data and growth rate calculation. All CT scans of the chest were reanalyzed to collect data regarding orientation of the aorta (either right sided or left sided), laterality of the ASCA, KD diameter, and DAW, as well as to identify the presence of other arterial aneurysms. Anatomic measurements of KD and DAW were determined by measuring aneurysm size from outer wall to outer wall in coronal, axial, and oblique views depending on the orientation of the ASCA in relation to the KD and the descending aorta. The decision to obtain both axial and oblique views for the measurement of KD and DAW diameters was supported by the challenges that our centerline software faced in the presence of very
odd vascular shapes and poorly timed images, which can lead to mismeasurement of vascular structures. Observed growth rates were calculated for all patients with two or more CT scans as follows: (diameter at last CT scanediameter at initial CT scan)/time interval between CT scans. Additionally, estimated growth rates for KD and DAW were calculated with an instrumental variables approach. This method uses a variable (the time interval between imaging studies) that is measured accurately and correlates it with changes in diameter size, because patients with longer time intervals between CT scans should demonstrate greater true aneurysm growth.13 The estimates were obtained by means of regression analysis in which aneurysm growth followed an exponential path. This method of estimating growth has been described previously and has been shown to be more accurate than simple arithmetic calculations for both measuring aneurysm growth rates and estimating risk factors for aneurysm growth.14 Statistical analysis. Data are reported using means and standard deviations for continuous variables or frequencies for categorical variables. Multivariable regression analysis was used to evaluate the impact of comorbidities and aneurysm characteristics on growth rate. Statistical analyses were performed using R (R foundation for Statistical Computing, Vienna, Austria) and www.socscistatistics.com.
RESULTS Patients. Between January 1999 and December 2016, 75 patients were identified with KD and ASCA, who had undergone a CT scan of the chest at the Yale-New Haven Hospital. The mean age at presentation was 63 6 19 years (range, 17-95 years). The majority of patients were female (65%). There were 47 left-sided (63%) and 28 right-sided (37%) aortas. There were 47 right ASCA (63%) and 28
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Table I. Patient comorbidities and clinical presentation
Characteristic
Total patients (N ¼ 75), Mean 6 SD (Range) or No. (%)
Demographics Mean age at initial presentation, years Female
63 6 19 (17-95) 49 (65)
Comorbidities
Fig 1. Diagrammatic representation of a Kommerell diverticulum (KD) with the usual measurements to record growth on the KD including KD diameter (yellow arrow) and the distance to the opposite aortic wall (DAW; blue dashed arrow).
left ASCA (37%) (Fig 2). Information on a number of comorbidities (Table I) was collected including hypertension (55%), chronic obstructive pulmonary disease (37%), hypercholesterolemia (27%), history of smoking (43%), coronary artery disease (15%), diabetes mellitus (11%), and chronic renal insufficiency (8%). The majority of patients were asymptomatic at presentation (92%). Six patients presented with symptoms: dysphagia in four, chest or back pain in one, and emboli to the fingers in one. Nine patients underwent operative intervention, including the six symptomatic patients. The three other patients, who were asymptomatic at presentation, were operated on owing to size and/or growth rate. One presented with a KD of 35.4 mm and DAW of 42.6 mm, and two patients were followed for 5.2 6 2.7 years and demonstrated growth with a change in KD of 2.6 mm and 8.6 mm. Diameters. The mean diameter of KD was 21.8 6 6.0 mm and the mean DAW was 48.3 6 10.8 mm. The mean KD and DAW of the nine ASCA who underwent operative repair were 23.8 6 6.8 mm and 47.0 6 10.8 mm, respectively, whereas the mean KD and DAW diameter at presentation of the 50 ASCA who were observed was 21.5 6 5.9 mm and 48.5 6 10.9 mm, respectively. There were two concomitant dissections of the descending thoracic aorta noted. Follow-up and growth rate. An overview of the management of ASCA treated during our study period is shown in Fig 3. Of the 66 patients with ASCA who were observed, 45 patients had two or more CT scans that were on average 31.7 6 32.5 months apart
Hypertension
41 (52)
Chronic obstructive pulmonary disease
28 (37)
Hypercholesterolemia
20 (27)
Smoking history
20 (27)
Coronary artery disease
11 (15)
Diabetes mellitus
8 (11)
Chronic renal insufficiency
6 (8)
SD, Standard deviation.
(range, 1-150 months). One patient died from a ruptured dissection of the aorta without undergoing operative repair owing to frailty. Eighteen patients did not have radiographic follow-up. However, clinically they did not develop symptoms or undergo any aortic procedure or operation. Three deaths occurred in our cohort owing melanoma nonresponsive to chemotherapy, cardiac arrest owing to decompensated congestive heart failure, and an unknown cause. The estimated growth rate in the 45 patients followed with ASCA, calculated using the instrumental variables approach, was for KD 1.45 6 0.39 mm/year and for DAW 2.29 6 0.47 mm/year. The frequency distribution of these estimated growth rates is depicted in Fig 4 and it can be appreciated that the majority of KD grew very slowly. Furthermore, to evaluate the impact of comorbidities and aneurysm characteristics on growth rates, a multivariable regression analysis was conducted, which demonstrated hypertension to be the only significant variable associated with faster growth for the DAW diameter (P ¼ .03; odds ratio [OR], 1.022; 95% confidence interval, 1.002-1.042). The other nonsignificant variables analyzed included history of smoking, chronic obstructive pulmonary disease, and history of other aneurysms. Surgical treatment. Repairs included eight open and one hybrid repair. For aberrant right subclavian arteries, we generally divided the artery from the aorta via left thoracotomy, with an additional right carotid to subclavian bypassdeither before or after the division. For aberrant left subclavian artery, we generally performed the entire procedure through the left chestddividing the artery from the right side arch and revascularizing with a side arm graft end to end to the distal intrathoracic subclavian artery. The hybrid repair for aberrant left subclavian artery included a left common carotid artery to
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Fig 2. A, Axial and (B) coronal views of a computed tomography (CT) scan of the chest demonstrating a left-sided aortic arch with an aberrant right subclavian artery (arrow). C, Axial and (D) coronal right-sided aortic arch with an aberrant left subclavian artery (arrow).
left subclavian artery bypass followed by the endovascular placement of a 38- to 34-mm tapered aortic TX2 endograft (Cook Medical, Bloomington, Ind) to occlude the mouth of the KD.
75 aberrant subclavian arteries
Patients undergoing repair
Patients observed
9 patients
66 patients
KD diameter: 23.8±6.8mm
KD diameter: 21.5±5.9cm
DAW diameter: 47.0±10.8mm
DAW diameter: 48.5±10.9mm
Open 8 patients
Observation 45 patients Time: 31.7±32.5 mo.
Endovascular
No radiographic follow-up
1 patient
18 patients (3 deaths)
Fig 3. Management of patients with Kommerell diverticulum (KD) and aberrant subclavian artery (ASCA; n ¼ 75). DAW, Distance to the opposite aortic wall.
Postoperatively, seven patients recovered without complications and left the hospital after 5 days of convalescence. One patient had a slow but complete neurologic recovery owing to prolonged intubation. A second patient had a left occipital and bilateral cerebellar ischemic strokes. He subsequently developed cerebral edema and hydrocephalus needing craniectomy. Further, he developed a femoral vein thrombosis that required anticoagulation and with this, his ischemic stroke transformed into a hemorrhagic one. This same patient had a prolonged intubation of more than 1 week with a complicating pneumonia. He remained hospitalized for 61 days and was later discharged to a rehabilitation facility.
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Fig 4. Frequency distribution of observed growth rates for (A) Kommerell diverticulum (KD) and (B) distance to opposite aortic wall (DAW).
DISCUSSION Dearth of literature. We are not aware of any studies analyzing the natural history of patients with KD and ASCA. Owing to a dearth of knowledge in the literature regarding the management of this developmental anomaly, optimal treatment is unclear. It has even been recommended that all KD and ASCA should undergo repair to prevent rupture and/or dissection (Table II).8,12 Female preponderance. In our cohort, a higher number of female patients (65%) was identified, as previously described regarding the prevalence of KD and ASCA.12,16 Aberrant right subclavian artery preponderance. Furthermore, we have also observed that the majority of patients identified had a left-sided aorta with a right ASCA (63%) versus a right-sided aorta and a left ASCA (37%) consistent with the literature, which reports a right-/left-sided prevalence in the general population of 2.0% and 0.4%, respectively.12 Incidental identification of aberrant artery. The vast majority of patients with ASCA and concomitant KD were identified incidentally on CT scans done for reasons not related to ASCA. We had a single death in our cohort attributable to a ruptured descending thoracic aorta in a 76-year-old patient with ASCA and KD who did not undergo operative repair owing to extreme medical frailty. The patient had a right-sided aortic arch with a left ASCA with a proximal dissection in the descending thoracic aorta. The growth of the KD and DAW in this patient was noted to be 3.7 mm and 18.5 mm, respectively within a 2-year period. The last sizes recorded for this patient were 35.8 mm and 84.2 mm for KD and DAW, respectively.
Slow growth and benign natural history. We found that slow growth of KD (1.45 6 0.39 mm/year) and DAW (2.29 6 0.47 mm/year) parallels a relatively benign clinical course during our follow-up period of 31.7 6 32.5 months, which argues for observation of asymptomatic patients and leaving surgery for cases of rapid growth or presentation with large sizes suggested by our colleagues Ota et al,10 who recommend repair at a KD size of 30 mm and a DAW size of 50 mm. We used our instrumental variable estimates to show estimated growth rate is to minimize any potential measurement errors. The mean observed annual growth rate (from simple growth rate method) for KD would be 2.3 6 8.58 mm and DAW would be 3.64 6 9.99 mm. Our method of estimating growth has been previously described and has been shown to be more accurate than simple arithmetic calculations for both measuring aneurysm growth rates and estimating risk factors for aneurysm growth.14 Prognostic importance of hypertension. The single variable statistically promoting growth was found to be hypertension with a P value of .03, an OR of 1.022, and a 95% confidence interval of 1.002 to 1.042. (Although statistically significant, this OR is of small magnitude.) All other variables such as smoking, chronic obstructive pulmonary disease, and a history of other aneurysms did not play a role. Risk of operation. Two of our nine patients had significant complications. The largest reports to date included 22 patients (mainly treated medically) identified by Fisher et al15 in a review of a radiologic database; one operated patient died. Another 22 patients, all undergoing surgery, were reported by van Bogerijen et al,15,17 with substantial postoperative complications, including
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Table II. Studies reporting case series on Kommerell diverticulum (KD) in the literature No. of patients reported
Mean KD size, mm
Mean DAW size, mm
Endovascular repair/open repair, %
Author
Year
Cina et al8
2004 4 (surgical)
60
Not mentioned
0/100
1 Respiratory failure, 1 death
Right thoracotomy provides better exposure Left carotid to subclavian transposition before the thoracic portion of operation recommended
Fisher et al15
2005 22 (mostly medical)
Not Not mentioned mentioned
0/100
1 Death (surgical)
Early detection and surgical/endovascular management can be lifesaving
Ota et al10
2006 6 (surgical)
Not 53.3 mentioned
0/100
1 Mediastinitis
Symptomatic patients should be operated on (dysphagia, aneurysm) Multiple options for operative intervention, including in situ reconstruction and replacement of the descending aorta, total arch replacement, and ligation of ASCA
0/100
None
Symptomatic patients should be operated on (dysphagia, cough, wheezing, stridor, dyspnea on exertion) Precise preoperative imaging necessary before the operation
2 type II endoleaks requiring coil embolization, 1 stroke
Hybrid repairs for KD are safe and effective treatment option Selection should be based on anatomy conditions
0/100
1 death, 1 hemorrhage
Repair of a ruptured KD is linked with mortality Aberrant subclavian arteries and KD should be repaired under elective conditions
0/100
2 phrenic nerve injuries, 1 transient ptosis, 1 graft occlusion, 1 laryngeal nerve injury, 1 reintubation, 1 type A dissection, 1 chylothorax, 1 transient ischemic attack
High percentage of Kommerell diverticula showing medial degeneration suggests asymptomatic patients with enlarged diverticulum benefit from resection
Backer et al18
2012 20 (surgical) Not Not mentioned mentioned
Idress et al19
2014 10 (surgical) Not Not 100% hybrid mentioned mentioned
Hosoba et al20 2014 5 (surgical)
Kim et al16
Not 50 mentioned
2014 19 (surgical) 26.2
Not mentioned
Complications
Important findings/ recommendations
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Table II. Continued. No. of patients reported
Mean KD size, mm
Author
Year
Van Bogerijen et al17
2015 22 (surgical) 25.5 6 6.9
Mean DAW size, mm
Endovascular repair/open repair, %
45.9 6 13.6
41/59
Complications
Important findings/ recommendations
1 death, 1 stroke, Surgical repair of 1 spinal cord aberrant subclavian ischemia, 1 permanent arteries has renal failure, acceptable 1 tracheostomy complication rates Endovascular approach did not appear to affect the late outcomes, suggesting that the choice of treatment should be based on patient-specific anatomy and associated comorbidities
ASCA, Aberrant subclavian artery; DAW, distance to the opposite aortic wall.
death, stroke, spinal cord ischemia, permanent renal failure, and tracheostomy affecting an overall 23% of their patients. These are serious operations, and careful case selection and preoperative planning are essential. Limitations. The major limitation of this study is the small sample size, as expected for an extremely rare anatomic developmental abnormality. Second, our treatments were predominantly open surgical approaches. In the present era, endovascular approaches can also be applied to address this patient population. Furthermore, five of the patients, who underwent surgical repair were younger than 55 years of age and we believe that providing the patients with the most durable surgical treatment at this juncture in time seemed reasonable. Third, 18 of the patients did not have radiologic followup owing to the fact that our query was based on the radiology CT database. As a result, many of the patients were originally scanned for other reasons (ie, rule out pulmonary embolism, possible lung nodule, and chest pain, among other reasons) and these individuals do not routinely undergo repeat CT scans that would have helped us delineate the growth rate of their KDs and ASCAs. Last, because there was a single rupture at a large diverticulum size, it is difficult to make conclusions about predicting or preventing rupture. Our growth rate determination was made from a small sample of patients with this rare condition; therefore, a degree of skepticism is warranted. Of note, this is the first pooled dataset from a very heterogeneous practice group composed of vascular surgeons, cardiac surgeons, interventional radiologists and interventional cardiologists. Nonetheless, we are not aware of a larger series of ASCA with KD investigated to date.
CONCLUSIONS Our single-center study is able to demonstrate that indeed growth over time of KD and ASCA are extremely rare. We have been able to determine that the growth of the diverticulum to be 1.45 6 0.39 mm/year and of the DAW to be 2.29 6 0.47 mm/year. Observation of asymptomatic KD and ASCA is appropriate. We agree with the current size criteria for repair (KD of 30 mm and DAW of 50 mm). Symptomatic patients (dysphagia, pain emboli) warrant surgical intervention regardless of size.
AUTHOR CONTRIBUTIONS Conception and design: YE, AB, BZ, JE Analysis and interpretation: YE, AB, YL, JR Data collection: YE, AB, CV, HM, MT, MZ Writing the article: YE, AB Critical revision of the article: YE, AB, CV, YL, JR, HM, MT, MZ, BZ, JE Final approval of the article: YE, AB, CV, YL, JR, HM, MT, MZ, BZ, JE Statistical analysis: YL, JR Obtained funding: Not applicable Overall responsibility: JE YE and AB contributed equally to this article and share co-first authorship.
REFERENCES 1. Bayford D. An account of a singular case of obstructed deglutition. Mem Med Society of London 1794;2:275-86. 2. Arkin A. Totale persistenz des rechten aortenbogens im Roentgenbild. Wien Arch fuer innere Medizin 1926;12:385. 3. Kommerell B. Verlagerung des oesophagus durch eine abnorm verlaufenden arteria subclavia dextra (arteria lusoria). Fortsch Geb Roentgenstrahlen 1936;54:590-5. 4. Edwards JE. Anomalies of the derivatives of the aortic arch system. Med Clin North Am 1948;32:925-49.
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5. Brown DL, Chapman WC, Edwards WH, Coltharp WH, Stoney WS. Dysphagia lusoria: aberrant right subclavian artery with a Kommerell’s diverticulum. Am Surg 1993;59:582-6. 6. Salomonowitz E, Edwards JE, Hunter DW, CastanedaZuniga WR, Lund G, Cragg AH, et al. The three types of aortic diverticula. AJR Am J Roentgenol 1984;142:673-9. 7. Molz G, Burri B. Aberrant subclavian artery (arteria lusoria): sex differences in the prevalence of various forms of the malformation. Evaluation of 1378 observations. Virchows Arch A Pathol Anat Histol 1978;380:303-15. 8. Cina CS, Althani H, Pasenau J, Abouzahr L. Kommerell’s diverticulum and right-sided aortic arch: a cohort study and review of the literature. J Vasc Surg 2004;39:131-9. 9. Austin EH, Wolfe WG. Aneurysm of aberrant subclavian artery with a review of the literature. J Vasc Surg 1985;2:571-7. 10. Ota T, Okada K, Takanashi S, Yamamoto S, Okita Y. Surgical treatment for Kommerell’s diverticulum. J Thorac Cardiovasc Surg 2006;131:574-8. 11. Idrees J, Keshavamurthy S, Subramanian S, Clair DG, Svensson LG, Roselli EE. Hybrid repair of Kommerell diverticulum. J Thorac Cardiovasc Surg 2014;147:973-6. 12. Tanaka A, Milner R, Ota T. Kommerell’s diverticulum in the current era: a comprehensive review. Gen Thorac Cardiovasc Surg 2015;63:245-59. 13. Rizzo JA, Chen J, Fang H, Ziganshin BA, Elefteriades JA. Statistical challenges in identifying risk factors for aortic disease. Aorta (Stamford) 2014;2:45-55.
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14. Rizzo JA, Coady MA, Elefteriades JA. Procedures for estimating growth rates in thoracic aortic aneurysms. J Clin Epidemiol 1998;51:747-54. 15. Fisher RG, Whigham CJ, Trinh C. Diverticula of Kommerell and aberrant subclavian arteries complicated by aneurysms. Cardiovasc Intervent Radiol 2005;28:553-60. 16. Kim KM, Cambria RP, Isselbacher EM, Baker JN, LaMuraglia GM, Stone JR, et al. Contemporary surgical approaches and outcomes in adults with Kommerell diverticulum. Ann Thorac Surg 2014;98:1347-54. 17. van Bogerijen GH, Patel HJ, Eliason JL, Criado E, Williams DM, Knepper J, et al. Evolution in the management of aberrant subclavian arteries and related Kommerell diverticulum. Ann Thorac Surg 2015;100:47-53. 18. Backer CL, Russell HM, Wurlitzer KC, Rastatter JC, Rigsby CK. Primary resection of Kommerell diverticulum and left subclavian artery transfer. Ann Thorac Surg 2012;94:1612-7. 19. Idrees J, Keshavamurthy S, Subramanian S, Clair DG, Svensson LG, Roselli EE. Hybrid repair of Kommerell diverticulum. J Thorac Cardiovasc Surg 2014;147:973-6. 20. Hosoba S, Suzuki T, Asai T, Nota H, Kuroyanagi S, Kinoshita T, et al. Surgical repair of Kommerell’s diverticulum and an aberrant subclavian artery. Surg Today 2014;44:247-51.
Submitted Feb 11, 2019; accepted Aug 18, 2019.
Natural history and management of Kommerell’s diverticulum in a single tertiary referral center Young Erben, MD,a Adam J. Brownstein, BA,b Camilo A. Velasquez, MD,b Yupeng Li, PhD,c John A. Rizzo, PhD,b Hamid Mojibian, MD,d Maryam Tanweer, MD,b Mohammad A. Zafar, MBBS,b Bulat A. Ziganshin, MD, PhD,b and John A. Elefteriades, MD,b Jacksonville, Fla; New Haven, Conn; and Glassboro, NJ
ABSTRACT Objective: The Kommerell diverticulum (KD) is an extremely rare developmental abnormality of the aorta related to an aberrant subclavian artery (ASCA). The objective of our study was to review the natural history of KD and ASCA using our single-center experience in diagnosing and managing KD and ASCA. Methods: A retrospective review of the Yale radiological database from January 1999 to December 2016 was performed. Only patients with KD/ASCA and a computed tomography (CT) scan of the chest were selected for review. The primary goal was to examine the natural history of KD and ASCA and the secondary goals were to review the management and outcomes of those patients treated for KD and ASCA. Results: There were 75 patients with KD/ASCA identified, with a mean age of 63 6 19 years; 49 were female (65%). On CT scans, left- and right-sided aortas were present in 47 (63%) and 28 (37%) patients. A right ASCA or a left ASCA were present in 47 (63%) and 28 (37%) patients. Six patients were symptomatic on presentation. Symptoms included dysphagia, chest or back pain, and emboli to the fingers. The mean KD diameter was 21.8 6 6.0 mm and the distance to the opposite aortic wall (DAW) was 48.3 6 10.8 mm. Sixty-six patients were followed for a mean of 31.7 6 32.5 months. One patient ruptured without repair. Nine patients underwent operative intervention, including eight open and one endovascular repair. Complications from operative intervention included ischemic stroke with hemorrhagic transformation, deep vein thrombosis and pneumonia. The mean growth rate for KD and DAW was 1.45 6 0.39 mm/year and 2.29 6 0.47 mm/year, respectively. On multivariable regression analysis, hypertension was a predictor of growth of DAW (P ¼ .03). Conclusions: KD is uncommon and shows a female predominance. The diverticulum grows, albeit slowly (KD and DAW growth rates of 1.45 6 0.39 mm/year and 2.29 6 0.47 mm/year). Most patients are asymptomatic, but dysphagia, chest/ back pain, and distal emboli may occur. Rupture is rare. Symptomatic patients should be operated. Asymptomatic patients can be followed with serial CT scans. (J Vasc Surg 2019;-:1-8.) Keywords: Aberrant subclavian artery; Open and endovascular repair; Kommerell diverticulum
Bayford1 in 1787 described an autopsy case of “obstructed deglutition” caused by a right aberrant subclavian artery (ASCA) passing posterior to the esophagus. The term dysphagia lusoria was coined, and the aberrant ASCA termed “arteria lusoria,” by Arkin.2 However, it was not until 1936 that the first clinical diagnosis of dysphagia lusoria caused by an ASCA and an aortic diverticulum compressing the esophagus was made in a living subject by Dr Burkhard F. Kommerell.3 Kommerell diverticulum (KD) is a persistent remnant of the fourth primitive dorsal arch owing to failed regression.4 In the case of left aortic arch with KD, the
right dorsal aorta involutes proximal to the right subclavian artery and leaves the subclavian artery attached to the left descending aorta via the distal portion of the right dorsal aorta. In contrast, in patients with the right aortic arch with KD, the left dorsal arch diminishes between the subclavian and carotid arteries and allows the right subclavian to originate from the remnant right dorsal arch.5 Salomonowitz et al6 classified the KD according to pathogenesis into three types: (1) KD in left aortic arch with right ASCA, (2) KD in the right aortic arch with left ASCA, and (3) aortic diverticulum without ASCA.
From the Division of Vascular and Endovascular Surgery, Mayo Clinic, Jackson-
Correspondence: John A. Elefteriades, MD, Aortic Institute at Yale-New Haven,
villea; the Aortic Institute at Yale-New Haven Hospital, Yale School of Medici-
Yale University School of Medicine, 789 Howard Ave, Clinic BuildingeCB317,
ne,b and the Section of Vascular Interventional Radiology, Yale School of Medicine,d New Haven; and the Department of Political Science and Economics, Rowan University, Glassboro.c A.J.B. was supported by the Richard K. Gershon, M.D., Student Research Fellowship and the Yale University School of Medicine Medical Student Research Fellowship. Author conflict of interest: none.
New Haven, CT 06519 (e-mail: [email protected]). The editors and reviewers of this article have no relevant financial relationships to disclose per the JVS policy that requires reviewers to decline review of any manuscript for which they may have a conflict of interest. 0741-5214 Copyright Ó 2019 by the Society for Vascular Surgery. Published by Elsevier Inc. https://doi.org/10.1016/j.jvs.2019.08.260
Presented at the Forty-sixth Annual Symposium of the Society for Clinical Vascular Surgery, Las Vegas, Nev, March 17-21, 2018.
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There is a paucity of data available regarding the natural history and management of ASCA and KD in the adult population, which is mainly due to the rarity of this condition and the low incidence of symptoms, reported at 5%.7 Some groups recommend surgical repair in all encountered KD owing to a high rupture and dissection rate of up to 53%.8,9 To date, only Ota et al10 proposed a size criterion for repair of the KD, using the KD diameter itself and the distance to the opposite aortic wall (DAW) (Fig 1). The minimal size recommended for repair includes a KD of 30 mm and a DAW of 50 mm.10-12 The objective of our study is to review the natural history of KD and ASCA using our single-center experience diagnosing and managing KD and ASCA. Furthermore, we sought to review the operative management and outcomes of those patients undergoing surgical intervention for KD and ASCA.
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ARTICLE HIGHLIGHTS d
d
d
Type of Research: Retrospective cohort study Key Findings: In 75 patients with a Kommerell diverticulum (KD) and aberrant left subclavian artery, 65% were females; right-sided aorta and right aberrant subclavian artery were present in 63%. Only six patients were symptomatic. One of 66 KDs that were followed ruptured. Open repair was performed in eight of nine patients, with complications of ischemic stroke, deep vein thrombosis, and pneumonia. Hypertension predicted growth. Take Home Message: KD is uncommon and shows a female predominance. Most patients are asymptomatic, rupture is rare. Symptomatic patients should be operated. Asymptomatic patients can be followed with serial computed tomography scans.
METHODS Patient cohort. The Yale radiology computed tomography (CT) database was searched with specific search terms including KD and/or ASCA for the period from January 1, 1999, to December 31, 2016. Patients were also identified via International Classification of Diseases, Ninth Edition, disease codes Q24.48 (anomalous congenital origin of the subclavian artery) and Q24.47 (persistent right aortic arch). Data collection included details of each patient’s clinical presentation, comorbidities, management, and follow-up as identified via retrospective chart review (Table I). Consistent with prior reports, symptomatic patients were defined as those with dysphagia, chest pain, dyspnea, and gastroesophageal reflux disease without other evident etiology for these symptoms.12 The primary goal was to review the natural history of KD and ASCA, including growth rate and risk of rupture, in those patients who were observed over time. The secondary goals were review of the management and outcomes of those patients treated surgically for KD and ASCA. Individual patient consent for study inclusion was not obtained or required, and this study received approval from the Yale Institutional Review Board (HIC# 1411014866) as a retrospective chart review. Imaging data and growth rate calculation. All CT scans of the chest were reanalyzed to collect data regarding orientation of the aorta (either right sided or left sided), laterality of the ASCA, KD diameter, and DAW, as well as to identify the presence of other arterial aneurysms. Anatomic measurements of KD and DAW were determined by measuring aneurysm size from outer wall to outer wall in coronal, axial, and oblique views depending on the orientation of the ASCA in relation to the KD and the descending aorta. The decision to obtain both axial and oblique views for the measurement of KD and DAW diameters was supported by the challenges that our centerline software faced in the presence of very
odd vascular shapes and poorly timed images, which can lead to mismeasurement of vascular structures. Observed growth rates were calculated for all patients with two or more CT scans as follows: (diameter at last CT scanediameter at initial CT scan)/time interval between CT scans. Additionally, estimated growth rates for KD and DAW were calculated with an instrumental variables approach. This method uses a variable (the time interval between imaging studies) that is measured accurately and correlates it with changes in diameter size, because patients with longer time intervals between CT scans should demonstrate greater true aneurysm growth.13 The estimates were obtained by means of regression analysis in which aneurysm growth followed an exponential path. This method of estimating growth has been described previously and has been shown to be more accurate than simple arithmetic calculations for both measuring aneurysm growth rates and estimating risk factors for aneurysm growth.14 Statistical analysis. Data are reported using means and standard deviations for continuous variables or frequencies for categorical variables. Multivariable regression analysis was used to evaluate the impact of comorbidities and aneurysm characteristics on growth rate. Statistical analyses were performed using R (R foundation for Statistical Computing, Vienna, Austria) and www.socscistatistics.com.
RESULTS Patients. Between January 1999 and December 2016, 75 patients were identified with KD and ASCA, who had undergone a CT scan of the chest at the Yale-New Haven Hospital. The mean age at presentation was 63 6 19 years (range, 17-95 years). The majority of patients were female (65%). There were 47 left-sided (63%) and 28 right-sided (37%) aortas. There were 47 right ASCA (63%) and 28
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Table I. Patient comorbidities and clinical presentation
Characteristic
Total patients (N ¼ 75), Mean 6 SD (Range) or No. (%)
Demographics Mean age at initial presentation, years Female
63 6 19 (17-95) 49 (65)
Comorbidities
Fig 1. Diagrammatic representation of a Kommerell diverticulum (KD) with the usual measurements to record growth on the KD including KD diameter (yellow arrow) and the distance to the opposite aortic wall (DAW; blue dashed arrow).
left ASCA (37%) (Fig 2). Information on a number of comorbidities (Table I) was collected including hypertension (55%), chronic obstructive pulmonary disease (37%), hypercholesterolemia (27%), history of smoking (43%), coronary artery disease (15%), diabetes mellitus (11%), and chronic renal insufficiency (8%). The majority of patients were asymptomatic at presentation (92%). Six patients presented with symptoms: dysphagia in four, chest or back pain in one, and emboli to the fingers in one. Nine patients underwent operative intervention, including the six symptomatic patients. The three other patients, who were asymptomatic at presentation, were operated on owing to size and/or growth rate. One presented with a KD of 35.4 mm and DAW of 42.6 mm, and two patients were followed for 5.2 6 2.7 years and demonstrated growth with a change in KD of 2.6 mm and 8.6 mm. Diameters. The mean diameter of KD was 21.8 6 6.0 mm and the mean DAW was 48.3 6 10.8 mm. The mean KD and DAW of the nine ASCA who underwent operative repair were 23.8 6 6.8 mm and 47.0 6 10.8 mm, respectively, whereas the mean KD and DAW diameter at presentation of the 50 ASCA who were observed was 21.5 6 5.9 mm and 48.5 6 10.9 mm, respectively. There were two concomitant dissections of the descending thoracic aorta noted. Follow-up and growth rate. An overview of the management of ASCA treated during our study period is shown in Fig 3. Of the 66 patients with ASCA who were observed, 45 patients had two or more CT scans that were on average 31.7 6 32.5 months apart
Hypertension
41 (52)
Chronic obstructive pulmonary disease
28 (37)
Hypercholesterolemia
20 (27)
Smoking history
20 (27)
Coronary artery disease
11 (15)
Diabetes mellitus
8 (11)
Chronic renal insufficiency
6 (8)
SD, Standard deviation.
(range, 1-150 months). One patient died from a ruptured dissection of the aorta without undergoing operative repair owing to frailty. Eighteen patients did not have radiographic follow-up. However, clinically they did not develop symptoms or undergo any aortic procedure or operation. Three deaths occurred in our cohort owing melanoma nonresponsive to chemotherapy, cardiac arrest owing to decompensated congestive heart failure, and an unknown cause. The estimated growth rate in the 45 patients followed with ASCA, calculated using the instrumental variables approach, was for KD 1.45 6 0.39 mm/year and for DAW 2.29 6 0.47 mm/year. The frequency distribution of these estimated growth rates is depicted in Fig 4 and it can be appreciated that the majority of KD grew very slowly. Furthermore, to evaluate the impact of comorbidities and aneurysm characteristics on growth rates, a multivariable regression analysis was conducted, which demonstrated hypertension to be the only significant variable associated with faster growth for the DAW diameter (P ¼ .03; odds ratio [OR], 1.022; 95% confidence interval, 1.002-1.042). The other nonsignificant variables analyzed included history of smoking, chronic obstructive pulmonary disease, and history of other aneurysms. Surgical treatment. Repairs included eight open and one hybrid repair. For aberrant right subclavian arteries, we generally divided the artery from the aorta via left thoracotomy, with an additional right carotid to subclavian bypassdeither before or after the division. For aberrant left subclavian artery, we generally performed the entire procedure through the left chestddividing the artery from the right side arch and revascularizing with a side arm graft end to end to the distal intrathoracic subclavian artery. The hybrid repair for aberrant left subclavian artery included a left common carotid artery to
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Fig 2. A, Axial and (B) coronal views of a computed tomography (CT) scan of the chest demonstrating a left-sided aortic arch with an aberrant right subclavian artery (arrow). C, Axial and (D) coronal right-sided aortic arch with an aberrant left subclavian artery (arrow).
left subclavian artery bypass followed by the endovascular placement of a 38- to 34-mm tapered aortic TX2 endograft (Cook Medical, Bloomington, Ind) to occlude the mouth of the KD.
75 aberrant subclavian arteries
Patients undergoing repair
Patients observed
9 patients
66 patients
KD diameter: 23.8±6.8mm
KD diameter: 21.5±5.9cm
DAW diameter: 47.0±10.8mm
DAW diameter: 48.5±10.9mm
Open 8 patients
Observation 45 patients Time: 31.7±32.5 mo.
Endovascular
No radiographic follow-up
1 patient
18 patients (3 deaths)
Fig 3. Management of patients with Kommerell diverticulum (KD) and aberrant subclavian artery (ASCA; n ¼ 75). DAW, Distance to the opposite aortic wall.
Postoperatively, seven patients recovered without complications and left the hospital after 5 days of convalescence. One patient had a slow but complete neurologic recovery owing to prolonged intubation. A second patient had a left occipital and bilateral cerebellar ischemic strokes. He subsequently developed cerebral edema and hydrocephalus needing craniectomy. Further, he developed a femoral vein thrombosis that required anticoagulation and with this, his ischemic stroke transformed into a hemorrhagic one. This same patient had a prolonged intubation of more than 1 week with a complicating pneumonia. He remained hospitalized for 61 days and was later discharged to a rehabilitation facility.
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Fig 4. Frequency distribution of observed growth rates for (A) Kommerell diverticulum (KD) and (B) distance to opposite aortic wall (DAW).
DISCUSSION Dearth of literature. We are not aware of any studies analyzing the natural history of patients with KD and ASCA. Owing to a dearth of knowledge in the literature regarding the management of this developmental anomaly, optimal treatment is unclear. It has even been recommended that all KD and ASCA should undergo repair to prevent rupture and/or dissection (Table II).8,12 Female preponderance. In our cohort, a higher number of female patients (65%) was identified, as previously described regarding the prevalence of KD and ASCA.12,16 Aberrant right subclavian artery preponderance. Furthermore, we have also observed that the majority of patients identified had a left-sided aorta with a right ASCA (63%) versus a right-sided aorta and a left ASCA (37%) consistent with the literature, which reports a right-/left-sided prevalence in the general population of 2.0% and 0.4%, respectively.12 Incidental identification of aberrant artery. The vast majority of patients with ASCA and concomitant KD were identified incidentally on CT scans done for reasons not related to ASCA. We had a single death in our cohort attributable to a ruptured descending thoracic aorta in a 76-year-old patient with ASCA and KD who did not undergo operative repair owing to extreme medical frailty. The patient had a right-sided aortic arch with a left ASCA with a proximal dissection in the descending thoracic aorta. The growth of the KD and DAW in this patient was noted to be 3.7 mm and 18.5 mm, respectively within a 2-year period. The last sizes recorded for this patient were 35.8 mm and 84.2 mm for KD and DAW, respectively.
Slow growth and benign natural history. We found that slow growth of KD (1.45 6 0.39 mm/year) and DAW (2.29 6 0.47 mm/year) parallels a relatively benign clinical course during our follow-up period of 31.7 6 32.5 months, which argues for observation of asymptomatic patients and leaving surgery for cases of rapid growth or presentation with large sizes suggested by our colleagues Ota et al,10 who recommend repair at a KD size of 30 mm and a DAW size of 50 mm. We used our instrumental variable estimates to show estimated growth rate is to minimize any potential measurement errors. The mean observed annual growth rate (from simple growth rate method) for KD would be 2.3 6 8.58 mm and DAW would be 3.64 6 9.99 mm. Our method of estimating growth has been previously described and has been shown to be more accurate than simple arithmetic calculations for both measuring aneurysm growth rates and estimating risk factors for aneurysm growth.14 Prognostic importance of hypertension. The single variable statistically promoting growth was found to be hypertension with a P value of .03, an OR of 1.022, and a 95% confidence interval of 1.002 to 1.042. (Although statistically significant, this OR is of small magnitude.) All other variables such as smoking, chronic obstructive pulmonary disease, and a history of other aneurysms did not play a role. Risk of operation. Two of our nine patients had significant complications. The largest reports to date included 22 patients (mainly treated medically) identified by Fisher et al15 in a review of a radiologic database; one operated patient died. Another 22 patients, all undergoing surgery, were reported by van Bogerijen et al,15,17 with substantial postoperative complications, including
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Table II. Studies reporting case series on Kommerell diverticulum (KD) in the literature No. of patients reported
Mean KD size, mm
Mean DAW size, mm
Endovascular repair/open repair, %
Author
Year
Cina et al8
2004 4 (surgical)
60
Not mentioned
0/100
1 Respiratory failure, 1 death
Right thoracotomy provides better exposure Left carotid to subclavian transposition before the thoracic portion of operation recommended
Fisher et al15
2005 22 (mostly medical)
Not Not mentioned mentioned
0/100
1 Death (surgical)
Early detection and surgical/endovascular management can be lifesaving
Ota et al10
2006 6 (surgical)
Not 53.3 mentioned
0/100
1 Mediastinitis
Symptomatic patients should be operated on (dysphagia, aneurysm) Multiple options for operative intervention, including in situ reconstruction and replacement of the descending aorta, total arch replacement, and ligation of ASCA
0/100
None
Symptomatic patients should be operated on (dysphagia, cough, wheezing, stridor, dyspnea on exertion) Precise preoperative imaging necessary before the operation
2 type II endoleaks requiring coil embolization, 1 stroke
Hybrid repairs for KD are safe and effective treatment option Selection should be based on anatomy conditions
0/100
1 death, 1 hemorrhage
Repair of a ruptured KD is linked with mortality Aberrant subclavian arteries and KD should be repaired under elective conditions
0/100
2 phrenic nerve injuries, 1 transient ptosis, 1 graft occlusion, 1 laryngeal nerve injury, 1 reintubation, 1 type A dissection, 1 chylothorax, 1 transient ischemic attack
High percentage of Kommerell diverticula showing medial degeneration suggests asymptomatic patients with enlarged diverticulum benefit from resection
Backer et al18
2012 20 (surgical) Not Not mentioned mentioned
Idress et al19
2014 10 (surgical) Not Not 100% hybrid mentioned mentioned
Hosoba et al20 2014 5 (surgical)
Kim et al16
Not 50 mentioned
2014 19 (surgical) 26.2
Not mentioned
Complications
Important findings/ recommendations
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Table II. Continued. No. of patients reported
Mean KD size, mm
Author
Year
Van Bogerijen et al17
2015 22 (surgical) 25.5 6 6.9
Mean DAW size, mm
Endovascular repair/open repair, %
45.9 6 13.6
41/59
Complications
Important findings/ recommendations
1 death, 1 stroke, Surgical repair of 1 spinal cord aberrant subclavian ischemia, 1 permanent arteries has renal failure, acceptable 1 tracheostomy complication rates Endovascular approach did not appear to affect the late outcomes, suggesting that the choice of treatment should be based on patient-specific anatomy and associated comorbidities
ASCA, Aberrant subclavian artery; DAW, distance to the opposite aortic wall.
death, stroke, spinal cord ischemia, permanent renal failure, and tracheostomy affecting an overall 23% of their patients. These are serious operations, and careful case selection and preoperative planning are essential. Limitations. The major limitation of this study is the small sample size, as expected for an extremely rare anatomic developmental abnormality. Second, our treatments were predominantly open surgical approaches. In the present era, endovascular approaches can also be applied to address this patient population. Furthermore, five of the patients, who underwent surgical repair were younger than 55 years of age and we believe that providing the patients with the most durable surgical treatment at this juncture in time seemed reasonable. Third, 18 of the patients did not have radiologic followup owing to the fact that our query was based on the radiology CT database. As a result, many of the patients were originally scanned for other reasons (ie, rule out pulmonary embolism, possible lung nodule, and chest pain, among other reasons) and these individuals do not routinely undergo repeat CT scans that would have helped us delineate the growth rate of their KDs and ASCAs. Last, because there was a single rupture at a large diverticulum size, it is difficult to make conclusions about predicting or preventing rupture. Our growth rate determination was made from a small sample of patients with this rare condition; therefore, a degree of skepticism is warranted. Of note, this is the first pooled dataset from a very heterogeneous practice group composed of vascular surgeons, cardiac surgeons, interventional radiologists and interventional cardiologists. Nonetheless, we are not aware of a larger series of ASCA with KD investigated to date.
CONCLUSIONS Our single-center study is able to demonstrate that indeed growth over time of KD and ASCA are extremely rare. We have been able to determine that the growth of the diverticulum to be 1.45 6 0.39 mm/year and of the DAW to be 2.29 6 0.47 mm/year. Observation of asymptomatic KD and ASCA is appropriate. We agree with the current size criteria for repair (KD of 30 mm and DAW of 50 mm). Symptomatic patients (dysphagia, pain emboli) warrant surgical intervention regardless of size.
AUTHOR CONTRIBUTIONS Conception and design: YE, AB, BZ, JE Analysis and interpretation: YE, AB, YL, JR Data collection: YE, AB, CV, HM, MT, MZ Writing the article: YE, AB Critical revision of the article: YE, AB, CV, YL, JR, HM, MT, MZ, BZ, JE Final approval of the article: YE, AB, CV, YL, JR, HM, MT, MZ, BZ, JE Statistical analysis: YL, JR Obtained funding: Not applicable Overall responsibility: JE YE and AB contributed equally to this article and share co-first authorship.
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5. Brown DL, Chapman WC, Edwards WH, Coltharp WH, Stoney WS. Dysphagia lusoria: aberrant right subclavian artery with a Kommerell’s diverticulum. Am Surg 1993;59:582-6. 6. Salomonowitz E, Edwards JE, Hunter DW, CastanedaZuniga WR, Lund G, Cragg AH, et al. The three types of aortic diverticula. AJR Am J Roentgenol 1984;142:673-9. 7. Molz G, Burri B. Aberrant subclavian artery (arteria lusoria): sex differences in the prevalence of various forms of the malformation. Evaluation of 1378 observations. Virchows Arch A Pathol Anat Histol 1978;380:303-15. 8. Cina CS, Althani H, Pasenau J, Abouzahr L. Kommerell’s diverticulum and right-sided aortic arch: a cohort study and review of the literature. J Vasc Surg 2004;39:131-9. 9. Austin EH, Wolfe WG. Aneurysm of aberrant subclavian artery with a review of the literature. J Vasc Surg 1985;2:571-7. 10. Ota T, Okada K, Takanashi S, Yamamoto S, Okita Y. Surgical treatment for Kommerell’s diverticulum. J Thorac Cardiovasc Surg 2006;131:574-8. 11. Idrees J, Keshavamurthy S, Subramanian S, Clair DG, Svensson LG, Roselli EE. Hybrid repair of Kommerell diverticulum. J Thorac Cardiovasc Surg 2014;147:973-6. 12. Tanaka A, Milner R, Ota T. Kommerell’s diverticulum in the current era: a comprehensive review. Gen Thorac Cardiovasc Surg 2015;63:245-59. 13. Rizzo JA, Chen J, Fang H, Ziganshin BA, Elefteriades JA. Statistical challenges in identifying risk factors for aortic disease. Aorta (Stamford) 2014;2:45-55.
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14. Rizzo JA, Coady MA, Elefteriades JA. Procedures for estimating growth rates in thoracic aortic aneurysms. J Clin Epidemiol 1998;51:747-54. 15. Fisher RG, Whigham CJ, Trinh C. Diverticula of Kommerell and aberrant subclavian arteries complicated by aneurysms. Cardiovasc Intervent Radiol 2005;28:553-60. 16. Kim KM, Cambria RP, Isselbacher EM, Baker JN, LaMuraglia GM, Stone JR, et al. Contemporary surgical approaches and outcomes in adults with Kommerell diverticulum. Ann Thorac Surg 2014;98:1347-54. 17. van Bogerijen GH, Patel HJ, Eliason JL, Criado E, Williams DM, Knepper J, et al. Evolution in the management of aberrant subclavian arteries and related Kommerell diverticulum. Ann Thorac Surg 2015;100:47-53. 18. Backer CL, Russell HM, Wurlitzer KC, Rastatter JC, Rigsby CK. Primary resection of Kommerell diverticulum and left subclavian artery transfer. Ann Thorac Surg 2012;94:1612-7. 19. Idrees J, Keshavamurthy S, Subramanian S, Clair DG, Svensson LG, Roselli EE. Hybrid repair of Kommerell diverticulum. J Thorac Cardiovasc Surg 2014;147:973-6. 20. Hosoba S, Suzuki T, Asai T, Nota H, Kuroyanagi S, Kinoshita T, et al. Surgical repair of Kommerell’s diverticulum and an aberrant subclavian artery. Surg Today 2014;44:247-51.
Submitted Feb 11, 2019; accepted Aug 18, 2019.