Background Data regarding the risk of aortic dissection in patients with bicuspid aortic valve and large ascending aortic diameter are limited, and appropriate timing of prophylactic ascending aortic replacement lacks consensus. Thus our objectives were to determine the risk of aortic dissection based on initial cross-sectional imaging data and clinical variables and to isolate predictors of aortic intervention in those initially prescribed serial surveillance imaging. Methods From January 1995 to January 2014, 1,181 patients with bicuspid aortic valve underwent cross-sectional computed tomography (CT) or magnetic resonance imaging (MRI) to ascertain sinus or tubular ascending aortic diameter greater than or equal to 4.7 cm. Random Forest classification was used to identify risk factors for aortic dissection, and among patients undergoing surveillance, time-related analysis was used to identify risk factors for aortic intervention. Results Prevalence of type A dissection that was detected by imaging or was found at operation or on follow-up was 5.3% (n = 63). Probability of type A dissection increased gradually at a sinus diameter of 5.0 cm—from 4.1% to 13% at 7.2 cm—and then increased steeply at an ascending aortic diameter of 5.3 cm—from 3.8% to 35% at 8.4 cm—corresponding to a cross-sectional area to height ratio of 10 cm2/m for sinuses of Valsalva and 13 cm2/m for the tubular ascending aorta. Cross-sectional area to height ratio was the best predictor of type A dissection (area under the curve [AUC] = 0.73). Conclusions Early prophylactic ascending aortic replacement in patients with bicuspid aortic valve should be considered at high-volume aortic centers to reduce the high risk of preventable type A dissection in those with aortas larger than approximately 5.0 cm or with a cross-sectional area to height ratio greater than approximately 10 cm2/m.

Three distinct phenotypes of bicuspid valve-associated aortopathy were identified using machine-learning methodology. Patient characteristics and valvular dysfunction vary by phenotype, suggesting that the location of aortic pathology may be related to the underlying pathophysiology of this disease.

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Background. Constrictive pericarditis is a rare, but increasingly recognized long-term postoperative complication of lung transplantation. Heightened clinical suspicion, improved diagnostic imaging, and effective surgical treatment of the disease have led to progressive awareness of the pathology. We present our institutional experience with constrictive pericarditis after lung transplant in an effort to investigate the cause and natural history of the disease. Methods. From October 2005 to October 2018, 1234 patients underwent orthotopic lung transplantation at Duke University Hospital. An institutional database was queried to identify incident patients and determine baseline clinical data. At a median of 11.2 months (interquartile range = 4.6–28.6 mo), 10 patients (0.8%) developed constrictive pericarditis. Simple descriptive statistics were used to describe cohort characteristics and identify variables associated with constrictive pericarditis after lung transplantation. Results. The indication for transplantation at index operation was idiopathic pulmonary fibrosis in 8 of 10 patients (1.2% of the 760 restrictive lung disease patients transplanted in the same time period). All 10 patients presented with worsening dyspnea and pleural effusions. Right heart catheterization confirmed constrictive physiology in all cases. Eight patients underwent pericardiectomy with improvement in cardiovascular hemodynamics and resolution of symptoms with no 30-day mortality. Conclusions. Diagnosis of constrictive pericarditis should be considered in patients with new-onset heart failure symptoms or recurrent pleural effusions within 2 years of lung transplantation. Idiopathic pulmonary fibrosis may be associated with increased risk for constrictive pericarditis. Pericardiectomy is a safe and effective treatment for posttransplant constrictive pericarditis.

Background Unicuspid aortic valve is an important subset of bicuspid aortic valve, and knowledge regarding its aortopathy pattern and surgical outcomes is limited. Our objectives were to characterize unicuspid aortic valve patients, associated aortopathy, and surgical outcomes. Methods From January 1990 to May 2013, 149 adult unicuspid aortic valve patients underwent aortic valve replacement or repair for aortic stenosis (n = 13), regurgitation (n = 13), or both (n = 123), and in 91 (61%) the aortic valve operation was combined with aortic repair. Data were obtained from the Cardiovascular Information Registry and medical record review. Three-dimensional imaging analysis was performed from preoperative computed tomography and magnetic resonance imaging scans. The Kaplan-Meier method was used for survival analysis. Results Patients had a mean maximum aortic diameter of 44 ± 8 mm and variably involved the aortic root, ascending, or arch, or both. Patients with valve operations alone were more likely to be hypertensive (p = 0.01) and to have severe aortic stenosis (p = 0.07) than those who underwent concurrent aortic operations. There were no operative deaths, strokes, or myocardial infarctions. Patients undergoing aortic repair had better long-term survival. Estimated survival at 1, 5, and 10 years was 100%, 100%, and 100% after combined operations and was 100%, 88%, and 88% after valve operations alone (p = 0.01). Conclusions Patients with a dysfunctional unicuspid aortic valve frequently present with an ascending aneurysm that requires repair. Combined aortic valve operations and aortic repair was associated with significantly better long-term survival than a valve operation alone. Further study of this association may direct decisions about timing of surgical intervention.

Objective Staged elephant trunk (ET) repair is a commonly performed procedure for extensive aortic disease. A significant proportion of patients with predominantly proximal aortic pathology often have in addition a moderately dilated descending aorta (<5 cm) that can progress over time. Objectives were to characterize patients, determine completion rate after prophylactic stage 1 ET, and assess outcomes. Methods From 1992 to 2012, a total of 572 patients underwent stage 1 ET for degenerative aneurysm and dissection at Cleveland Clinic. Prophylactic stage 1 ET was performed in 117 (20.5%) who had predominantly proximal disease (5.5 ± 1 cm) with moderate dilation of the descending aorta (4 ± 0.6 cm). Aortic pathology included: aneurysm (n = 56 [48%]); chronic dissection (n = 41 [35%]); pseudoaneurysm (n = 9 [7.7%]); penetrating ulcer (n = 9 [7.7%]); and intramural hematoma (n = 2 [1.7%]). Other diagnoses included connective tissue disorder (12 [10%]); aortitis (20 [17%]); bicuspid aortic valve (9 [7.6%]); and previous type A dissection repair (27 [23%]). Results Operative mortality was 0.8% (1 of 117). This patient suffered postoperative myocardial infarction and mesenteric ischemia, resulting in sepsis and death. Other complications included: stroke (n = 7 [6%]); tracheostomy (n = 6 [5%]); renal dialysis (n = 4 [3.3%]); and reoperation for bleeding (n = 7 [6%]). The mean follow-up time was 4 ± 3 years. Fifty-three (45%) patients completed the stage 2 ET (open: 20 [38%]; endovascular: 33 [62%]) at a median interval of 6 months (9 days–10 years). The mean descending diameter increased from 4.1 ± 0.6 cm to 5 ± 1 cm at the time of stage 2 completion. In 11 patients, stage 2 was performed for acute aortic events. Estimated survival at 1, 5, and 8 years was 94%, 88%, and 74%, respectively. Conclusions Prophylactic ET for moderately dilated descending aorta is an effective strategy for staged repair, especially in patients with chronic dissection, connective tissue disorder, and aortitis. In addition, this approach can be beneficial for emergency treatment of late distal aortic complications.