• Korean Journal of Radiology
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• v.24 no.10
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• pp.960-973
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• 2023

The bicuspid aortic valve (BAV) is the most common congenital cardiovascular malformation. Patients with BAV are at higher risk of other congenital cardiovascular malformations and valvular dysfunction, including aortic stenosis/regurgitation and infective endocarditis. BAV may also be related to aortic wall abnormalities such as aortic dilatation, aneurysm, and dissection. The morphology of the BAV varies with the presence and position of the raphe and is associated with the type of valvular dysfunction and aortopathy. Therefore, accurate diagnosis and effective treatment at an early stage are essential to prevent complications in patients with BAV. This pictorial essay highlights the characteristics of BAV and its related congenital cardiovascular malformations, valvular dysfunction, aortopathy, and other rare cardiac complications using multimodal imaging.

• Korean Journal of Radiology
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• v.22 no.6
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• pp.890-900
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• 2021

Objective: To identify the association between morphological and functional characteristics of the bicuspid aortic valve (BAV) and bicuspid aortopathy and to identify the determinants of aortic dilatation using transthoracic echocardiography (TTE) and cardiac computed tomography (CCT). Materials and Methods: This study included 312 subjects (mean [SD] age, 52.7 [14.3] years; 227 males [72.8%]) who underwent TTE and CCT. The BAVs were classified by anterior-posterior (BAV-AP) or right-left (BAV-RL) orientation of the cusps and divided according to the presence (raphe+) or absence of a raphe (raphe-) based on the CCT and intraoperative findings. The dimensions of the sinus of Valsalva and the proximal ascending aorta were measured by CCT. We assessed the determinants of aortic root and proximal ascending aortic dilatation (size index > 2.1 cm/m²) by Univariable and multivariable logistic regression analyses. Results: Of the 312 patients, BAV-AP was present in 188 patients (60.3%), and 185 patients (59.3%) were raphe+. Moderate-to-severe aortic stenosis (AS) was the most common hemodynamic abnormality (54.8%). The most common type of aortopathy was the combined dilated root and mid-ascending aortic phenotype (62.5%). On multivariable analysis, age and AS severity were significantly associated with aortic root dilatation (p < 0.05), and age, sex, and AS severity were significantly associated with ascending aortic dilatation (p < 0.05). However, the orientation of the cusps, presence of a raphe, and severity of aortic regurgitation were not associated with aortic root and ascending aortic dilatation. Conclusion: BAV morphological characteristics were not determinants of aortic dilatation. Age, sex, and AS severity were predictors of bicuspid aortopathy. Therefore, age, sex, and AS severity, rather than valve morphology, need to be considered when planning treatment for BAV patients.

• Journal of Chest Surgery
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• v.56 no.4
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• pp.255-261
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• 2023

Background: The surgical threshold for bicuspid aortic valve (BAV)-related aortopathy is a matter of debate due to its uncertain etiology and prognosis. This study investigated the prognosis of unrepaired BAV aortopathy in patients undergoing surgical aortic valve replacement (SAVR). Methods: We retrospectively analyzed data from 720 patients (age, 60.8±11.5 years; 246 women) who underwent SAVR for BAV disease without aortic repair between 2005 and 2020 at Asan Medical Center. The clinical endpoints were defined as occurrences of sudden death, aortic dissection or rupture, and elective aortic repair. To estimate postoperative changes in the dimensions of the unrepaired aorta, the individual annual aortic expansion rate was calculated. Multiple linear regression models were used to evaluate the risk of aortic expansion. Results: The mean ascending aortic diameter was 39.5±4.6 mm, and 299 patients (41.5%) had a baseline ascending aorta diameter >40 mm. During 70.0±68.3 months of follow-up, the mean annual aortic expansion rate was 0.39±1.96 mm/yr, no aortic dissection or rupture was observed, and sudden deaths were reported in 12 patients (0.34% per person-year). Linear regression analysis revealed no significant correlation between the baseline ascending aortic diameter and postoperative aortic expansion (R²=0.004, β=-0.84, p=0.082). Conclusion: In selected patients undergoing SAVR for a BAV (<55 mm), the risk of adverse aortic events was very low. As this observation contradicts current practice guidelines advocating for proactive aortic replacement in dilated ascending aortas measuring >45 mm, the study results need further validation by studies involving larger populations or randomized controlled trials.

• Journal of Chest Surgery
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• v.49 no.6
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• pp.443-450
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• 2016

Background: Although unique aortic pathology related to bicuspid aortic valve (BAV) has been previously reported, clinical implications of BAV to aortopathy risk have yet to be investigated. We looked for potential differences in matrix protein expressions in the aortic wall in BAV patients. Methods: Aorta specimens were obtained from 31 patients: BAV group (n=27), tricuspid aortic valve (TAV) group (n=4). The BAV group was categorized into three subgroups: left coronary sinus-right coronary sinus (R+L group; n=13, 42%), right coronary sinus-non-coronary sinus (R+N group; n=8, 26%), and anteroposterior (AP group; n=6, 19%). We analyzed the expression of endothelial nitric oxide synthase (eNOS), matrix metalloproteinase (MMP)-9, and tissue inhibitor of matrix metalloproteinase (TIMP)-2. Results: Based on the mean value of the control group, BAV group showed decreased expression of eNOS in 72.7% of patients, increased MMP-9 in 82.3%, and decreased TIMP in 79.2%. There was a higher tendency for aortopathy in the BAV group: eNOS $(BAV:TAV)=53%{\pm}7%:57%{\pm}11%$, MMP-9 $(BAV:TAV)=48%{\pm}10%:38%{\pm}1%$. The AP group showed lower expression of eNOS than the fusion (R+L, R+N) group did; $48%{\pm}5%$ vs. $55%{\pm}7%$ (p=0.081). Conclusion: Not all patients with BAV had expression of aortopathy; however, for patients who had a suspicious form of bicuspid valve, aortic wall biopsy could be valuable to signify the presence of aortopathy.

• Biomolecules & Therapeutics
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• v.28 no.1
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• pp.98-103
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• 2020

Marfan syndrome (MFS), a connective tissue disorder caused by mutations in the fibrillin-1 (Fbn1) gene, has vascular manifestations including aortic aneurysm, dissection, and rupture. Its vascular pathogenesis is assumed to be attributed to increased transforming growth factor β (TGFβ) signaling and blockade of excessive TGFβ signaling has been thought to prevent dissection and aneurysm formation. Here, we investigated whether galunisertib, a potent small-molecule inhibitor of TGFβ receptor I (TβRI), attenuates aneurysmal disease in a murine model of MFS (Fbn1^C1039G/+) and compared the impact of galuninsertib on the MFS-related vascular pathogenesis with that of losartan, a prophylactic agent routinely used for patients with MFS. Fbn1^C1039G/+ mice were administered galunisertib or losartan for 8 weeks, and their ascending aortas were assessed for histopathological changes and phosphorylation of Smad2 and extracellular signal-regulated kinase 1/2 (Erk1/2). Mice treated with galunisertib or losartan barely exhibited phosphorylated Smad2, suggesting that both drugs effectively blocked overactivated canonical TGFβ signaling in Fbn1^C1039G/+ mice. However, galunisertib treatment did not attenuate disrupted medial wall architecture and only partially decreased Erk1/2 phosphorylation, whereas losartan significantly inhibited MFS-associated aortopathy and markedly decreased Erk1/2 phosphorylation in Fbn1^C1039G/+ mice. These data unexpectedly revealed that galunisertib, a TβRI inhibitor, showed no benefits in aneurysmal disease in MFS mice although it completely blocked Smad2 phosphorylation. The significant losartan-induced inhibition of both aortic vascular pathogenesis and Smad2 phosphorylation implied that canonical TGFβ signaling might not prominently drive aneurysmal diseases in MFS mice.

• Journal of Chest Surgery
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• v.52 no.5
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• pp.335-341
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• 2019

Background: The endovascular approach to aortic disease treatment has been increasingly utilized in the past 2 decades. This study aimed to determine the long-term results of using the Seal thoracic stent graft. Methods: We retrospectively reviewed the outcomes of patients who underwent thoracic endovascular aortic repair or a hybrid procedure using the Seal thoracic stent graft (S&G Biotech, Seongnam, Korea) from January 2008 to July 2018 at a single institution. We investigated in-hospital mortality and the incidence of postoperative complications. We also investigated the mid-term survival rate and incidence of aorta-related complications. Results: Among 72 patients with stent grafts, 15 patients underwent the hybrid procedure and 21 underwent emergency surgery. The mean follow-up period was $37.86{\pm}30.73$ months (range, 0-124 months). Five patients (6.9%) died within 30 days. Two patients developed cerebrovascular accidents. Spinal cord injury occurred in 2 patients. Postoperative renal failure, postoperative extracorporeal membrane oxygenation support, and pneumonia were reported in 3, 1, and 6 patients, respectively. Stent-related aortic complications were observed in 5 patients (6.8%). The 1- and 5-year survival and freedom from stent-induced aortic event rates were 81.5% and 58.7%, and 97.0% and 89.1%, respectively. Conclusion: The use of the Seal thoracic stent graft yielded good mid-term results. Further studies are needed to examine the long-term outcomes of this device.

• Journal of Chest Surgery
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• v.45 no.5
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• pp.295-300
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• 2012

Background: With growing attention to the aortopathy associated with aortic valve diseases, the number of candidates for accompanying ascending aorta and/or root replacement is increasing among the patients who require aortic valve replacement (AVR). However, such procedures have been considered more risky than AVR alone. This study aimed to compare the surgical outcome of isolated AVR and AVR combined with aortic procedures. Materials and Methods: A total of 86 patients who underwent elective AVR between 2004 and June 2010 were divided into two groups: complex AVR (n=50, AVR with ascending aorta replacement in 24 and the Bentall procedure in 26) and simple AVR (n=36). Preoperative characteristics, surgical data, intra- and postoperative allogenic blood transfusion requirement, the postoperative clinical course, and major complications were retrospectively reviewed and compared. Results: The preoperative mean logistic European System for Cardiac Operative Risk Evaluation (%) did not differ between the groups: $11.0{\pm}7.8%$ in the complex AVR group and $12.3{\pm}8.0%$ in the simple AVR group. Although complex AVR required longer cardiopulmonary bypass ($152.4{\pm}52.6$ minutes vs. $109.7{\pm}22.7$ minutes, p=0.001), the quantity of allogenic blood products did not differ ($13.4{\pm}14.7$ units vs. $13.9{\pm}11.2$ units). There was no mortality, mechanical circulatory support, stroke, or renal failure requiring hemodialysis/filtration. No difference was found in the incidence of bleeding (40% vs. 33.3%) which was defined as red blood cell transfusion ${\geq}5$ units, reoperation, or intentional delayed closure. The incidence of mediastinitis (2.0% vs. 0%), ventilator ${\geq}24$ hours (4.0% vs. 2.8%), atrial fibrillation (18.0% vs. 25.0%), mean intensive care unit stay (34.5 hours vs. 38.8 hours), and median hospital stay (8 days vs. 7 days) did not differ, either. Conclusion: AVR combined with additional aortic or root replacement showed an excellent outcome and recovery course equivalent to that after isolated AVR.