Long-term outcome of bicuspid aortic valve repair using figure-of-8 hitch-up stitches.

Objective: To maximize successful repair of bicuspid aortic valves by adding figure-of-8 hitch-up stitches at commissures. Methods: From 2000 to 2022, bicuspid aortic valve repair was performed on 1112 patients at Cleveland Clinic, with 367 patients receiving figure-of-8 hitch-up stitches along with classical techniques, including Cabrol suture, cusp plication, raphe resection, and valve-sparing root replacement. Operative outcomes, repair durability, and survival were assessed in the figure-of-8 hitch-up stitches cohort, and outcomes were compared among 195 balancing-score-matched patient pairs who underwent bicuspid aortic valve repair with and without figure-of-8 hitch-up stitches. Results: Patients who underwent bicuspid aortic valve repair with figure-of-8 stitches had an operative mortality of 0.3% (1 of 367) and in-hospital reoperation for aortic valve dysfunction of 1.1% (4 of 367). At 10 years, prevalence of severe aortic regurgitation was 8.6%, mean gradient 24 mm Hg, freedom from aortic valve reoperation 75%, and survival 98%. In matched cohorts, operative mortality was similar (0.51% vs 0%; P > .9) as were morbidities, including in-hospital reoperation due to aortic valve dysfunction (1.0% vs 1.5%; P > .9). Comparable long-term outcomes were observed at 10 years (prevalence of severe aortic regurgitation of 8.7% vs 5.0% [P = .11], mean gradient 18 vs 17 mm Hg [P = .40]; freedom from aortic valve reoperation 80% vs 81% [P = .73]; and survival 99.5% vs 94.6% [P = .18]). Conclusions: Figure-of-8 hitch-up stitch is a safe bicuspid aortic valve repair technique. It increases the likelihood of a successful repair without increasing risk of cusp tear and achieves satisfactory long-term survival and durability when added to classical repair techniques.

Contemporary experience with the Commando procedure for anterior mitral anular calcification.

Objective: Anterior mitral anular calcification, particularly in radiation heart disease, and previous valve replacement with destroyed intervalvular fibrosa are challenging for prosthesis sizing and placement. The Commando procedure with intervalvular fibrosa reconstruction permits double-valve replacement in these challenging conditions. We referenced outcomes after Commando procedures to standard double-valve replacements. Methods: From January 2011 to January 2022, 129 Commando procedures and 1191 aortic and mitral double-valve replacements were performed at the Cleveland Clinic, excluding endocarditis. Reasons for the Commando were severe calcification after radiation (n = 67), without radiation (n = 43), and others (n = 19). Commando procedures were referenced to a subset of double-valve replacements using balancing-score methods (109 pairs). Results: Between balanced groups, Commando versus double-valve replacement had higher total calcium scores (median 6140 vs 2680 HU, P = .03). Hospital outcomes were similar, including operative mortality (12/11% vs 8/7.3%, P = .35) and reoperation for bleeding (9/8.3% vs 5/4.6%, P = .28). Survival and freedom from reoperation at 5 years were 54% versus 67% (P = .33) and 87% versus 100% (P = .04), respectively. Higher calcium score was associated with lower survival after double-valve replacement but not after the Commando. The Commando procedure had lower aortic valve mean gradients at 4 years (9.4 vs 11 mm Hg, P = .04). After Commando procedures for calcification, 5-year survival was 60% and 59% with and without radiation, respectively (P = .47). Conclusions: The Commando procedure with reconstruction of the intervalvular fibrosa destroyed by mitral anular calcification, radiation, or previous surgery demonstrates acceptable outcomes similar to standard double-valve replacement. More experience and long-term outcomes are required to refine patient selection for and application of the Commando approach.

The Ozaki Procedure: Standardized Protocol Adoption of a Complex Innovative Procedure.

Background: The Ozaki procedure using autologous pericardium is an interesting but complex alternative for aortic valve replacement. We present a standardized approach to minimize the learning curve and confirm reproducibility. Methods: After careful preparation, from May 2015 to February 2021, an Ozaki procedure was performed on 46 patients age 51 ± 14 years. Seven had unicuspid (15%), 29 bicuspid (63%), and 10 tricuspid (22%) aortic valves, and 2 patients had endocarditis. Endpoints were operative learning curves, perioperative outcomes, intermediate-term valve hemodynamics, reintervention, health-related quality of life (MacNew Heart Disease Health-Related Quality of Life questionnaire), and mortality. Results: Cardiopulmonary bypass and aortic clamp times decreased from 145 to 125 â€‹minutes and 120 to 100 â€‹minutes, respectively, over the first 20 cases, reflecting the learning curve. There was no major perioperative morbidity or mortality. Median postoperative stay was 6.9 days. Aortic regurgitation was mild or less in all but 2 patients who developed moderate aortic regurgitation. Mean aortic valve gradient was 7.9 mmHg postoperatively, 9.2 mmHg by 6 months, and constant thereafter. Left ventricular ejection fraction was 58% preoperatively, 60% at 6 months, and remained stable thereafter. One patient developed infective endocarditis 7 months postoperatively, failed medical management, and underwent valve replacement at 14 months. Two-year survival was 96%, with 1 noncardiac death at 16 months. Health-related quality of life in mental, physical, and emotional domains was better than matched norms, global 6.2 vs. 5.0 (p < 0.0001). Conclusions: Using a well-prepared standardized approach, the Ozaki procedure is reproducible with a short learning curve, excellent hemodynamic performance, and good quality of life.

Outcomes of aortic root replacement with tricuspid aortic valve reimplantation in patients with residual aortic regurgitation.

OBJECTIVE: To characterize residual aortic regurgitation (AR), identify its risk factors, and evaluate outcomes following aortic root replacement with aortic valve reimplantation. METHODS: From 2002 to 2020, 756 patients with a tricuspid aortic valve underwent elective reimplantation for aortic root aneurysm. AR on transthoracic echocardiograms before hospital discharge was graded as mild or greater. Machine learning was used to identify risk factors for residual AR and subsequent aortic valve reoperation. RESULTS: Sixty-five patients (8.6%) had mild (58 [7.7%]) or moderate (7 [0.93%]) residual postoperative AR. They had more severe preoperative AR (38% vs 12%; P < .0001), thickened cusps (7.7% vs 2.2%; P = .008), aortic valve repair (38% vs 23%; P = .004), and multiple returns to cardiopulmonary bypass for additional repair (11% vs 3.3%; P = .003) than those without AR. Predictors of residual AR were severe preoperative AR, smaller aortic root graft, and concomitant cusp repair. At 10 years, patients with versus without residual AR had more moderate or severe AR (48% vs 7.0%; P < .0001) and freedom from reoperation was worse (89% vs 98%; P < .0001). Residual AR was a risk factor for early reoperation. Concomitant coronary bypass, lower body mass index, and lower ejection fraction were risk factors for late reoperation. Ten-year survival was similar among patients with and without residual AR (97% vs 93%; P = .43). CONCLUSIONS: Residual AR after elective reimplantation of a tricuspid aortic valve for aortic root aneurysm is uncommon. Patients with severe preoperative AR and those who undergo valve repair have higher risk for residual AR, which can progress and increase risk of aortic valve reoperation.

Survival and repair durability in patients undergoing concomitant aortic valve reimplantation and mitral valve repair.

Objective: The study objective was to determine repair durability and survival in patients with and without connective tissue disorders undergoing concomitant aortic valve reimplantation and mitral valve repair. Methods: From 2002 to 2019, 68 patients underwent concomitant aortic valve reimplantation and mitral valve repair, including 27 patients with Marfan syndrome (39.7%). Follow-up echocardiograms were analyzed using nonlinear multiphase mixed-effects cumulative logistic regression. The regurgitation grade over time was estimated by averaging patient-specific profiles. Survival and freedom from reoperation were estimated by the Kaplan-Meier method. Results: At 7 years, 11% of patients had aortic insufficiency greater than mild (severe in 2 patients). There was no difference in greater than mild aortic insufficiency between patients with or without Marfan syndrome (P = .37). Twenty percent of patients had progressed to mitral regurgitation greater than mild (severe in only 1 patient). The prevalence of recurrent mitral regurgitation was higher in those without Marfan syndrome, with greater than mild regurgitation increasing to 24% by 2 years and remaining constant thereafter (P = .04). Freedom from reoperation on the aortic valve or mitral valve was 83% at 10 years and did not differ between Marfan syndrome groups. There were no cases of perioperative mortality. Survival at 5 and 10 years was 94% and 87%, respectively, without a difference between those with and without Marfan syndrome. Conclusions: Patients can undergo a total repair strategy using combined aortic valve reimplantation and mitral valve repair procedures with a low risk of mortality and complications, with favorable freedom from both residual valve regurgitation and reoperation.

Increasing surgeon experience and cumulative institutional experience drive decreasing hospital mortality after reoperative cardiac surgery.

OBJECTIVE: The study objective was to identify the effects of surgeon experience and age, in the context of cumulative institutional experience, on risk-adjusted hospital mortality after cardiac reoperations. METHODS: From 1951 to 2020, 36 surgeons performed 160,338 cardiac operations, including 32,871 reoperations. Hospital death was modeled using a novel tree-bagged, generalized varying-coefficient method with 6 variables reflecting cumulative surgeon and institutional experience up to each cardiac operation: (1) number of total and (2) reoperative cardiac operations performed by a surgeon, (3) cumulative institutional number of total and (4) reoperative cardiac operations, (5) year of surgery, and (6) surgeon age at each operation. These were adjusted for 46 patient characteristics and surgical components. RESULTS: There were 1470 hospital deaths after cardiac reoperations (4.5%). At the institutional level, hospital death decreased exponentially and became less variable, leveling at 1.2% after approximately 14,000 cardiac reoperations. For all surgeons as a group, hospital death decreased rapidly over the first 750 reoperations and then gradually decreased with increasing experience to less than 1% after approximately 4000 reoperations. Surgeon age up to 75 years was associated with ever-decreasing hospital death. CONCLUSIONS: Surgeon age and experience have been implicated in adverse surgical outcomes, particularly after complex cardiac operations, with young surgeons being novices and older surgeons having declining ability. However, at Cleveland Clinic, outcomes of cardiac reoperations improved with increasing primary surgeon experience, without any suggestion to mid-70s of an age cutoff. Patients were protected by the cumulative background of institutional experience that created a culture of safety and teamwork that mitigated adverse events after cardiac surgery.

A dynamic Norwood mortality estimation: Characterizing individual, updated, predicted mortality trajectories after the Norwood operation.

Objective: Post-Norwood mortality remains high and unpredictable. Current models for mortality do not incorporate interstage events. We sought to determine the association of time-related interstage events, along with (pre)operative characteristics, with death post-Norwood and subsequently predict individual mortality. Methods: From the Congenital Heart Surgeons' Society Critical Left Heart Obstruction cohort, 360 neonates underwent Norwood operations from 2005 to 2016. Risk of death post-Norwood was modeled using a novel application of parametric hazard analysis, in which baseline and operative characteristics and time-related adverse events, procedures, and repeated weight and arterial oxygen saturation measurements were considered. Individual predicted mortality trajectories that dynamically update (increase or decrease) over time were derived and plotted. Results: After the Norwood, 282 patients (78%) progressed to stage 2 palliation, 60 patients (17%) died, 5 patients (1%) underwent heart transplantation, and 13 patients (4%) were alive without transitioning to another end point. In total, 3052 postoperative events occurred and 963 measures of weight and oxygen saturation were obtained. Risk factors for death included resuscitated cardiac arrest, moderate or greater atrioventricular valve regurgitation, intracranial hemorrhage/stroke, sepsis, lower longitudinal oxygen saturation, readmission, smaller baseline aortic diameter, smaller baseline mitral valve z-score, and lower longitudinal weight. Each patient's predicted mortality trajectory varied as risk factors occurred over time. Groups with qualitatively similar mortality trajectories were noted. Conclusions: Risk of death post-Norwood is dynamic and most frequently associated with time-related postoperative events and measures, rather than baseline characteristics. Dynamic predicted mortality trajectories for individuals and their visualization represent a paradigm shift from population-derived insights to precision medicine at the patient level.

Effect of ascending aorta replacement on the long-term outcomes of bicuspid aortic valve repair.

OBJECTIVE: The study objective was to determine the effect of sinutubular junction stabilization on long-term outcomes of bicuspid aortic valve repair. METHODS: From January 1998 to January 2020, 419 patients underwent bicuspid aortic valve repair with ascending aorta replacement and 421 without (bicuspid aortic valve repair alone). Propensity score matching (97 pairs) was used to compare outcomes. RESULTS: Before matching, prevalence of severe aortic regurgitation at 10 years was 5.4% after bicuspid aortic valve repair + ascending aorta replacement and 10% after bicuspid aortic valve repair alone; aortic valve gradient was 20 mm Hg after bicuspid aortic valve repair + ascending aorta replacement and 19 mm Hg after bicuspid aortic valve repair alone. Ten-year freedom from reoperation overall was 79% after bicuspid aortic valve repair + ascending aorta replacement and 75% after bicuspid aortic valve repair alone; freedom from late aortic regurgitation was 93% after bicuspid aortic valve repair + ascending aorta replacement and 92% after bicuspid aortic valve repair alone; and freedom from aortic stenosis was 87% after bicuspid aortic valve repair + ascending aorta replacement and 93% after bicuspid aortic valve repair alone. Ten-year survival was 95% after bicuspid aortic valve repair + ascending aorta replacement and 96% after bicuspid aortic valve repair alone. After matching, prevalence of severe aortic regurgitation at 10 years was 11% after bicuspid aortic valve repair + ascending aorta replacement and 9.1% after bicuspid aortic valve repair alone (P = .33); aortic valve gradient was 16 mm Hg after bicuspid aortic valve repair + ascending aorta replacement and 25 mm Hg after bicuspid aortic valve repair alone (P < .0001). Ten-year freedom from reoperation was 85% after bicuspid aortic valve repair + ascending aorta replacement and 72% after bicuspid aortic valve repair alone (P = .08) overall. Ten-year freedom from reoperation for late aortic regurgitation was 88% after bicuspid aortic valve repair + ascending aorta replacement and 86% after bicuspid aortic valve repair alone (P = .65). Freedom from aortic stenosis was 97% after bicuspid aortic valve repair + ascending aorta replacement and 91% after bicuspid aortic valve repair alone (P = .03). Ten-year survival was 96% after bicuspid aortic valve repair + ascending aorta replacement and 96% after bicuspid aortic valve repair alone (P = .16). CONCLUSIONS: Bicuspid aortic valve repair with or without ascending aorta replacement is associated with good short- and long-term outcomes. Bicuspid aortic valve repair + ascending aorta replacement has a minimal effect on long-term repair durability. Sinutubular junction stabilization should not be performed for the sole purpose of long-term repair durability.

Aortic valve reimplantation in patients with connective tissue disorders: Are the leaflets durable?

OBJECTIVE: The durability of reimplanted myxomatous aortic valves in root replacements for patients with connective tissue disorders (CTD) is unclear; therefore, we sought to evaluate the long-term resilience of these repairs. METHODS: From January 1980 to January 2020, 214 patients with CTD and 645 without CTD underwent primary, elective aortic valve reimplantation operations at Cleveland Clinic. The CTD cohort included 164 (77%) with Marfan, 23 (11%) with Loeys-Dietz, and 7 (3.3%) with Ehlers-Danlos CTD. We accounted for differing patient characteristics between the groups by propensity score matching to compare outcomes, yielding 96 matched pairs. Longitudinal echocardiographic measures were compared using nonlinear mixed effects models. RESULTS: In the CTD cohort, there were no operative mortalities (30-day or in-hospital), 1 (0.47%) stroke, and 1 (0.47%) early in-hospital reoperation for valve dysfunction. Ten-year prevalence of no aortic regurgitation was 86%, mild 11%, and moderate 3%. Ten-year freedom from reoperation was 97%. In propensity matched cohorts, there were no significant differences in in-hospital outcomes, longitudinal aortic regurgitation and mean gradient, risk of reoperation on the aortic valve, or risk of late death. CONCLUSIONS: Aortic valve reimplantation is a durable operation in patients with CTD and root aneurysms. These patients do not experience early degeneration of their reimplanted aortic valves.

Characterization of Favorable Right Ventricular Dimensions for Optimal Reverse Remodeling Following Pulmonary Valve Replacement.

We sought to couple current cardiac magnetic resonance (CMR) thresholds of right ventricular (RV) size and function with longitudinal trajectories of RV recovery, after pulmonary valve replacement (PVR). We aimed to identify optimal timing of PVR and couple CMR-based metrics with contemporaneous echocardiographic metrics. From June 2002 to January 2019, 174 patients with severe pulmonary regurgitation and peak RV outflow tract gradient <30 mm Hg underwent PVR at Cleveland Clinic. Mean age was 35 ± 16 years and 60 (34%) had concomitant tricuspid valve surgery. RV end diastolic area index (RVEDAi) and function metrics were measured by offline image review on preoperative and 794 postoperative echocardiograms. Contemporaneous RV end diastolic volume index (RVEDVi) was assessed on CMR and correlated to RVEDAi. Multiphase nonlinear mixed-effects models were used to analyze the longitudinal change in RV size and function after PVR. RVEDAi was correlated with RVEDVi (P < 0.0001, r = 0.59). RVEDAi decreased slowly over 10 years following PVR. An inflection point at 24 cm2/m2 was noted at 1 year post-PVR and was associated with failure of RV reverse remodeling and RVEDVi ≥150 mL/m2. Compared to patients with preoperative RVEDVi ≥150 mL/m2, patients with RVEDVi <150 mL/m2 had accelerated recovery of longitudinal trajectories of RV size and function metrics on echocardiograms. Reverse remodeling of RV following PVR is an ongoing process. Current accepted threshold values for PVR are associated with greatest RV recovery, suggesting that earlier PVR is warranted. Echocardiography can potentially be utilized in lieu of CMR for surveillance and interventional triage.

Aortic Valve Reconstruction With Autologous Pericardium Versus a Bioprosthesis: The Ozaki Procedure in Perspective.

Background We assessed the Ozaki procedure, aortic valve reconstruction using autologous pericardium, with respect to its learning curve, hemodynamic performance, and durability compared with a stented bioprosthesis. Methods and Results From January 2007 to January 2016, 776 patients underwent an Ozaki procedure at Toho University Ohashi Medical Center. Learning curves, aortic regurgitation (AR), and peak gradient, assessed by serial echocardiograms, valve rereplacement, and survival were investigated. Valve performance and durability were compared with 627 1:1 propensity-matched patients receiving stented bovine pericardial valves implanted from 1982 to 2011 at Cleveland Clinic. Learning curves were observed for aortic clamp and cardiopulmonary bypass times, AR prevalence, and early mortality. Decreased aortic clamp time was observed over the first 300 cases. New surgeons performing parts of the procedure after case 400 resulted in a slight increase in aortic clamp and cardiopulmonary bypass times. Among matched patients, the Ozaki cohort had more AR than the PERIMOUNT cohort (severe AR at 1 and 6 years, 0.58% and 3.6% versus 0.45% and 1.0%, respectively; P[trend]=0.006), although with a steep learning curve. Peak gradient showed the opposite trend: 14 and 17 mm Hg for Ozaki and 24 and 28 mm Hg for PERIMOUNT at these times (P[trend]<0.001). Freedom from rereplacement was similar (P=0.491). Survival of the Ozaki cohort was 85% at 6 years. Conclusions Patients undergoing the Ozaki procedure had lower gradients but more recurrent AR than those receiving PERIMOUNT bioprostheses. Although recurrent AR is concerning, results confirm low risk and good midterm performance of the Ozaki procedure, supporting its continued use.

The decreasing risk of reoperative aortic valve replacement: Implications for valve choice and transcatheter therapy.

OBJECTIVE: Increasing use of bioprostheses for surgical aortic valve replacement (SAVR) in younger patients, together with wider use of transcatheter aortic valve replacement, necessitates understanding risks associated with surgical valve reintervention. Therefore, we sought to identify risks of reoperative SAVR compared with those of primary isolated SAVR. METHODS: From January 1980 to July 2017, 7037 patients underwent nonemergency isolated SAVR, with 753 reoperations and 6284 primary isolated operations. These 2 groups were propensity score-matched on 46 preoperative variables, yielding 581 patient pairs for comparing outcomes. RESULTS: Among propensity score-matched patients, aortic clamp time (median 63 vs 52 minutes; P < .0001), cardiopulmonary bypass time (median 88 vs 67 minutes; P < .0001), and postoperative stay (median 7.1 vs 6.9 days; P = .003) were longer for reoperative SAVR than primary isolated SAVR. Hospital mortality after reoperative SAVR decreased from 3.4% in 1985 to 1.3% in 2011, similar to that of primary isolated SAVR. Occurrence of stroke, deep sternal wound infection, and new renal dialysis was similar. Blood transfusion (67% vs 36%; P < .0001) and reoperations for bleeding/tamponade (6.4% vs 3.1%; P = .009) were more common after reoperative SAVR. Survival at 1, 5, 10, and 20 years was 94%, 82%, 64%, and 33% after reoperative SAVR and 95%, 86%, 72%, and 46% after elective primary isolated SAVR. CONCLUSIONS: Risk of mortality and morbidity after reoperative SAVR has declined and is now similar to that of primary isolated SAVR. Decisions regarding prosthesis choice and SAVR versus transcatheter aortic valve replacement should be made in the context of lifelong disease management rather than avoidance of reoperation.

Characterizing the anatomic spectrum, surgical treatment, and long-term clinical outcomes for patients with Shone's syndrome.

OBJECTIVE: Shone's syndrome (SS) has a varied anatomic spectrum without consensus on need and timing for mitral valve intervention (MVI). We sought to (1) characterize the anatomic spectrum and treatment pathways; (2) describe long-term outcomes and their determinants; and (3) define the impact of MVI timing on survival. METHODS: In total, 121 patients with SS who underwent operation at Cleveland Clinic between 1956 and 2021 were reviewed. Multivariable parametric hazard analyses including time-varying covariables, and modulated renewal to account for repeated events, were performed. End points included time-related survival and reintervention. RESULTS: Median follow-up was 9.9 years. Mitral stenosis (MS) (98%), coarctation (80%), and aortic stenosis (70%) predominated. The most common combination was MS + aortic stenosis + coarctation (26%). Median initial mean mitral and aortic gradients were 3.6 (15th/85th percentiles: 2.0/6.8) and 9.0 (2.1/46) mm Hg, respectively. Median initial surgery age was 0.041 (0.011/3.2) years. Initial surgeries included coarctation repair (43%), arch repair (18%), and staged biventricular repair (18%). Overall survival was 92% at 20 years. Freedom from reoperation was 66% and 24% at 1 and 20 years. Patients with no MVI or initial MVI (N = 7) tended to be associated with better early survival compared with those with MVI at subsequent operation (N = 29) (P = .06). Risk factors for early reintervention included initial Norwood operation, with younger age and arch hypoplasia increasing later reintervention. CONCLUSIONS: Despite excellent long-term survival, reoperation in SS is frequent and occurs most commonly on left ventricular outflow tract and mitral valve. Although MS is present in most, few require MVI. Delaying MVI may compromise early survival.

Aortic valve cusp repair does not affect durability of modified aortic valve reimplantation for tricuspid aortic valves.

Objective: During aortic valve reimplantation, cusp repair may be needed to produce a competent valve. We investigated whether the need for aortic valve cusp repair affects aortic valve reimplantation durability. Methods: Patients with tricuspid aortic valves who underwent aortic valve reimplantation from January 2002 to January 2020 at a single center were retrospectively analyzed. Propensity matching was used to compare outcomes between patients who did and did not require aortic valve cusp repair. Results: Cusp repair was performed in 181 of 756 patients (24%). Patients who required cusp repair were more often male, were older, had more aortic valve regurgitation, and less often had connective tissue disease. Patients who underwent cusp repair had longer aortic clamp time (124 ± 43 minutes vs 107 ± 36 minutes, P = .001). In-hospital outcomes were similar between groups and with no operative deaths. A total of 98.3% of patients with cusp repair and 99.3% of patients without cusp repair had mild or less aortic regurgitation at discharge. The median follow-up was 3.9 and 3.2 years for the cusp repair and no cusp repair groups, respectively. At 10 years, estimated prevalence of moderate or more aortic regurgitation was 12% for patients with cusp repair and 7.0% for patients without cusp repair (P = .30). Mean aortic valve gradients were 6.2 mm Hg and 8.0 mm Hg, respectively (P = .01). Ten-year freedom from reoperation was 99% versus 99% (P = .64) in the matched cohort and 97% versus 97%, respectively (P = .30), in the unmatched cohort. Survival at 10 years was 98% after cusp repair and 93% without cusp repair (P = .05). Conclusions: Aortic valve reimplantation for patients with tricuspid aortic valves has excellent long-term results. Need for aortic valve cusp repair does not affect long-term outcomes and should not deter surgeons from performing valve-sparing surgery.

Coronary artery disease in adults with anomalous aortic origin of a coronary artery.

Objectives: This study sought to characterize coronary artery disease (CAD) among adults diagnosed with an anomalous aortic origin of a coronary artery (AAOCA). We hypothesized that coronaries with anomalous origins have more severe CAD stenosis than coronaries with normal origins. Methods: This single-center study of 763 adults with AAOCA consisted of 620 patients from our cardiac catheterization database (1958-2009) and 273 patients from electronic medical records query (2010-2021). Within left main, anterior descending, circumflex, and right coronary arteries, the CAD stenosis severity, assessed by invasive or computer tomography angiography, was modeled with coronary-level variables (presence of an anomalous origin) and patient-level variables (age, sex, comorbidities, and which of the four coronaries was anomalous). Results: Of the 763 patients, 472 (60%) had obstructive CAD, of whom, 142/472 (30%) had obstructive CAD only in the anomalous coronary. Multivariable modeling showed similar CAD stenosis severity between coronaries with anomalous versus normal origins (P = .8). Compared with AAOCA of other coronaries, the anomalous circumflex was diagnosed at older ages (59.7 ± 11.1 vs 54.3 ± 15.8 years, P < .0001) and was associated with increased stenosis in all coronaries (odds ratio, 2.7; 95% confidence interval, 2.2-3.4, P < .0001). Conclusions: Among adults diagnosed with AAOCA, the anomalous origin did not appear to increase the severity of CAD within the anomalous coronary. In contrast to the circumflex, AAOCA of the other vessels may contribute a greater ischemic burden when they present symptomatically at younger ages with less CAD. Future research should investigate the interaction between AAOCA, CAD, and ischemic risk to guide interventions.

Comparison of Outcomes of Patients Undergoing Reimplantation versus Bentall Root Procedure.

BACKGROUND: A bioprosthesis- or mechanical-prosthesis-containing polyester graft (composite graft) is standard surgical management for aortic root aneurysms (Bentall procedure), but particularly in the young patient in whom a bioprosthesis is likely to deteriorate and a mechanical prosthesis mandates life-long anticoagulation, valve-sparing procedures have been devised. One such procedure involves reimplantation of the native aortic valve in the polyester graft. With focus on selecting the optimum procedure for young relatively asymptomatic patients, we compared outcomes of reimplantation of the aortic valve versus the Bentall procedure and identified factors influencing outcomes. METHODS: From January 2000 to January 2017, 643 adults age ≤ 70 with tricuspid aortic valves underwent elective aortic root replacement with either reimplantation (n = 448/70%) or a composite valve graft (Bentall) procedure (n = 195/30%). Outcomes were compared in 100 propensity-matched pairs. RESULTS: Patients with fewer symptoms, less aortic regurgitation (AR), higher left ventricular ejection fraction, and smaller cross-sectional aortic area/height ratio had a higher likelihood of valve repair with reimplantation (all p < 0.02) versus receiving a Bentall procedure. Operative mortality was 0.16% (reimplantation, 1/448, 0.22%; Bentall 0/195, 0%). After reimplantation, 8-year freedom from severe AR was 95% and 10-year freedom from reintervention was 98%. Ten-year survival was 95%. Higher preoperative AR grade (p < 0.0001) but not larger root diameter (p = 0.3) was associated with higher grade of late regurgitation after a reimplantation procedure. Among propensity-matched patients, reimplantation compared with a Bentall was associated with similar 10-year survival (89% vs. 94%), but more late AR (8-year freedom from severe AR: 93% vs. 99.9%) and greater early reduction in, but similar late, left ventricular mass (104 vs. 105 g•m-2 at 8 years). CONCLUSION: Excellent aortic valve reimplantation results versus Bentall lead us to recommend reimplantation more often in patients who present with even moderately severe or severe AR and significantly enlarged aortic roots.

Optimizing evaluation in pediatric and young adult patients with Marfan syndrome: Novel longitudinal metrics to track growth of aortic structures.

OBJECTIVE: Surveillance metrics in pediatric and young adult Marfan syndrome (pMFS) are challenging. We evaluated the utility of aortic root cross-sectional area/height index (CSA/Ht) on echocardiogram among pMFS patients as a risk stratification and surgical triage metric. METHODS: Genotype or phenotype positive pMFS patients aged 25 years or younger seen at our center from 2001 to 2020 were identified. Time-related transition to surgery was modeled using parametric methods. Predictive utility of CSA/Ht compared with aortic root diameter (ARd) and root Z score (ARz) were modeled using nonlinear multivariable parametric and nonparametric longitudinal regression models. RESULTS: Seventy-nine patients (43% female) presented at median age of 5.8 years (15th-85th percentile, 0.75-17 years) with median follow-up of 4.4 years (range, 0-18.5 years). Baseline echocardiography data were: CSA/Ht, 3.9 ± 1.4 cm2/m; ARd, 2.4 ± 0.89 cm; and ARz, 2.4 ± 1.7. CSA/Ht tracked ARd better compared with ARz (r = 0.91 vs 0.24). Eighteen patients underwent surgery. Surgical procedures included at least 2 components in 17 (aortic, mitral, tricuspid, aortic root, and arch procedures) and isolated mitral valve procedures in 1 patient. Time-related transition to surgery showed a prominent early phase to 1 year post presentation, followed by a slowly increasing late phase. CSA/Ht had a more linear correlation versus ARz during periods of rapid somatic growth in surgical patients. Surgical repair occurred at CSA/Ht between 5 and 7 cm2/m. CONCLUSIONS: CSA/Ht tracked ARd well over time. CSA/Ht between 5 and 7 cm2/m might be a promising metric for surgical triage in pMFS patients. CSA/Ht surgical threshold values in pMFS patients occurred at lower than current accepted "surgical" threshold values for CSA/Ht in adult Marfan syndrome patients.

Adjunctive endovascular balloon fracture fenestration for chronic aortic dissection.

OBJECTIVE: Positive remodeling after thoracic endovascular aortic repair (TEVAR) for chronic thoracic aortic dissection is variable due to incomplete distal seal and retrograde false lumen perfusion. We assessed the outcomes of adjunctive balloon fracture fenestration (BFF) during TEVAR in patients with chronic aortic dissection complicated by negative remodeling. METHODS: From June 2013 to January 2016, 49 patients with chronic aortic dissection complicated by aneurysm due to negative remodeling underwent TEVAR with BFF. Contrast-enhanced computed tomography was performed before discharge, at 3 to 6 months, and annually. RESULTS: Intraoperatively, endovascular stent graft expansion was achieved in all patients. There was 1 hospital death due to visceral malperfusion related to acute-on-chronic dissection noted before planned BFF. There were no occurrences of paraplegia, 3 patients had stroke, and 3 had acute renal failure. Survival at 1 year was 91%. Late reintervention for incomplete false lumen exclusion was required in 16 patients and freedom from reintervention was 75% at 1 year. Thirty-six patients (73.5%) had complete false lumen thrombosis through the treated segment. True lumen area increased following TEVAR with BFF and continued to incrementally expand with subsequent aortic remodeling at 1-year follow-up. Thirteen patients had positive remodeling, defined as thrombosis of false lumen, ≥10% decrease in aortic dimension, and ≥10% increase in true lumen diameter. Patients with positive remodeling had an average decrease of 11 mm in maximal aortic diameter at final follow-up. CONCLUSIONS: BFF of chronic dissection membrane is a beneficial adjunct to TEVAR during short-term follow-up and may promote positive aortic remodeling and is worthy of further study.