Twenty-five years' experience with isolated bicuspid aortic valve repair: impact of commissural orientation.

OBJECTIVES: Repair of the bicuspid aortic valve (BAV) has evolved in the past 25 years. The aim of this study was to review and analyse the long-term durability of isolated BAV repair with particular focus on commissural orientation (CO). METHODS: All patients who underwent BAV repair for severe aortic regurgitation between October 1998 and December 2022 were included. The study group consists of all patients operated after 2009, i.e. since CO modification. The control group includes patients who were operated before 2009. CO was classified as symmetric, asymmetric and very asymmetric. RESULTS: Overall, 594 adult patients (93% male; mean age 42 years) were included. At 15 years, survival was 94.8% [standard deviation (SD): 2.2]; freedom from reoperation was 86.8% (SD: 2.3). Freedom from aortic insufficiency ≥II was 70.8% (SD: 4.7) at 15 years. Modification of CO by sinus plication was performed in 200 (33.7%) instances. Using competing risks analysis, the absence of effective height measurement (P = 0.018), very asymmetric CO (P = 0.028), the presence of calcification (P < 0.001), the use of pericardial patch (P < 0.001), the use of subcommissural sutures (P < 0.001) and preoperative endocarditis (P = 0.005) were identified as independent predictors for reoperation. Follow-up was 97% complete (4228 patient-years); mean follow-up was 7 years (SD: 5). CONCLUSIONS: Isolated BAV repair leads to good survival and durability in all morphologic types if cusp repair is guided by effective height, suture annuloplasty is performed, and CO is modified using sinus plication in asymmetrical valves. Very asymmetrical valves may should be treated with a lower threshold for replacement.

Contemporary Valvular Mechanisms of Aortic Regurgitation in Tricuspid Aortic Valves: Importance in Repair Versus Replacement Strategy.

BACKGROUND: This study was performed to determine cusp causes of aortic regurgitation in patients with tricuspid aortic valves without significant aortic dilatation and define cusp pathologies amenable to surgical repair (aortic valve repair [AVr]) versus aortic valve replacement. METHODS AND RESULTS: We retrospectively reviewed surgical reports of consecutive adults with tricuspid aortic valves undergoing surgery for clinically significant aortic regurgitation within a prospective registry from January 2005 to September 2019. Valvular mechanisms were determined by systematic in vivo intraoperative quantification methods. Of 516 patients, 287 (56%) underwent repair (AVr; mean±SD age, 59.9±12.4 years; 81% men) and 229 (44%) underwent replacement (aortic valve replacement; mean±SD age, 62.8±13.8 years [P=0.01 compared to AVr]; 67% men). A single valvular mechanism was present in 454 patients (88%), with cusp prolapse (46%), retraction (24%), and perforation (18%) being the most common. Prolapse involved the right cusp in 86% of cases and was more frequent in men (P<0.001). Two-dimensional transesophageal echocardiography accuracy for predicting mechanisms was 73% to 82% for the right cusp, 55% to 61% for the noncoronary cusp, and 0% for the left-coronary cusp. Cusp prolapse, younger age, and larger patient size were associated with successful AVr (all P<0.03), whereas retraction, perforation, older age, and concomitant mitral repair were associated with aortic valve replacement (all P<0.03). CONCLUSIONS: Right cusp prolapse is the most frequent single valvular mechanism in patients with tricuspid aortic valve aortic regurgitation, followed by cusp retraction and perforation. The accuracy of 2-dimensional transesophageal echocardiography is limited for left and noncoronary cusp mechanistic assessment. Prolapse is associated with successful AVr, whereas retraction and perforation are associated with aortic valve replacement. With systematic intraoperative quantification methods and current surgical techniques, more than half of tricuspid aortic valve aortic regurgitation cases may be successfully repaired.

Forensic, legal, and clinical aspects of deaths associated with implanted cardiac devices.

As the population ages, the prevalence of heart failure and individuals wearing an implanted cardiac device is increasing. The combination of different underlying pathophysiologies and (the combination of) implanted cardiac devices can become a challenge with regard to the determination of cause and manner of death in such individuals. Additionally, heart disease is frequently associated with mental disease, ranging from anxiety and depression to suicidality and suicide (attempts). At the same time, the correct diagnosis of cause and manner of death is the basis for quality assurance, further therapeutic advances, legal safety, and suicide prevention. By that, an interdisciplinary field between legal medicine, clinicians, and law enforcement opens up. In this field, the different participants can simultaneously benefit from and need each other. For example, legal medicine experts need investigatory results and clinical expertise for the interpretation of readout data of implanted cardiac devices in order to correctly determine the cause of death. A correctly determined cause of death can assist law enforcement and help clinicians to further improve various therapeutic approaches based on correct mortality data collection. In addition, it is the basis for identification of suicides of device carriers, allowing psychological and psychiatric experts to better understand the burden of mental disease in this particular cohort. Against this interdisciplinary background, this manuscript summarizes information about psychiatric comorbidities and suicidality while being on a device. Thereby, basic information on complications and malfunctions of implanted cardiac devices, device-associated deaths with particular emphasis on device manipulation is displayed as basic information needed for correct determination of the cause of death. Also, legal and ethical issues in this field are outlined. The final result is a proposal of an interdisciplinary assessment workflow for a conjoint approach to improve the diagnosis of deaths associated with implanted cardiac devices. It will allow for a differentiation between an individual who died with or due to the device.

Valve-sparing aortic root replacement-for all patients?

BACKGROUND: Valve-sparing root replacement (VSRR) has been associated with good survival and low rates of valve-related complications (VRCs). Whether these advantages are present irrespective of patient comorbidity or age is unclear. The aim of this study was to analyze survival and frequency of VRCs in relation to patient comorbidity and age. METHODS: Between October 1995 and December 2021, 1156 patients with a bicuspid or tricuspid aortic valve were treated by root remodeling. The mean patient age was 53.3 ± 14 years, and 973 (84%) were male. The mean duration of follow-up was 6.7 ± 5.5 years (median, 5.9 years), and follow-up was 95% complete (7746 patient-years). We analyzed the population according to comorbidity and age at surgery. A discriminating cutoff for the effect of age was determined using receiver operating characteristic curve (ROC) analysis. RESULTS: Mean survival at 15 years was 74.7 ± 2.5%. Deceased patients were older (mean, 65.3 ± 12 years vs 51.6 ± 14.1 years; P < .001) at the time of surgery and had more comorbidities (coronary artery disease [CAD], 28.4% vs 9.8%; P < .001). The sole significant adjusted predictor was age (P < .001). By ROC analysis (area under the curve, 0.780), the optimal cutoff for age was 61 years. Survival was 87.1 ± 2.8% at 15 years in patients age <61 years, compared to 55.3 ± 4.3% in patients age >61 years (P < .0001). Using competing risk analysis, VRC-free survival at 15 years was 66.8% at 15 years, including 76.7% in patients age <61 years and 52.4% in those age >61 years (P < .0001). CONCLUSIONS: VSRR is associated with a low incidence of VRC and excellent durability. Survival is decreased in the presence of comorbidities, mainly CAD, and patient age >61 years. Despite lower survival, freedom from VRC is good.

Remodeling of the aortic root-a 28-year journey.

Background: Root remodeling is one form of valve-preserving root replacement for aortic regurgitation and root aneurysm. The objective of this review was to summarize our experience with root remodeling encompassing 28 years. Methods: We performed root remodeling in 1,189 patients (76% male, mean age 53±14 years) between October 1995 and September 2022. The original valve morphology was unicuspid in 33 (2%), bicuspid in 472 (40%) and tricuspid in 684 (58%) patients. Fifty-four patients (5%) had Marfan's syndrome. Objective measurement of valve configuration was performed in 804 (77%) and an external suture annuloplasty was added in 524 patients (44%). Cusp repair was performed in 1,047 (88%) patients, most commonly for prolapse (n=972; 82%). Mean follow-up was 6.7±5.5 years [1 month to 28 years]. Follow-up was 95% complete (7,700 patient-years). Results: Survival was 71% at 20 years; freedom from cardiac death was 80%. Freedom from aortic regurgitation ≥2 was 77% at 15 years. Freedom from reoperation was 89% and was higher in tricuspid aortic valves (94%) compared to bicuspid (84%) and unicuspid valves (P<0.001). Since the introduction of effective height measurement, freedom from reoperation has remained stable at 15 years (91%). With the addition of a suture annuloplasty, freedom from reoperation was 94% at 12 years. The difference with or without annuloplasty (91%) was not significant (P=0.949). Conclusions: Root remodeling is a viable option in valve-preserving root replacement. Concomitant cusp prolapse is frequent and can be corrected reproducibly by intraoperative measurement of effective height. The long-term benefit of an annuloplasty still needs to be defined.

Twenty-five years of root remodelling for root aneurysm and tricuspid aortic valve.

OBJECTIVES: The aim of this retrospective study was to assess the long-term results of root remodelling with tricuspid aortic valves and the effects of concomitant cusp repair and annuloplasty. METHODS: Between October 1995 and December 2021, 684 patients with root aneurysm and regurgitant tricuspid valves were treated by root remodelling. The mean age was 56.5 [standard deviation (SD): 14] years, and 538 (77.6%) were male. Relevant aortic regurgitation was present in 68.3%. Concomitant procedures were performed in 374 patients. The long-term results were analysed. The mean follow-up of 7.2 (SD: 5.3) years (median 6.6 years); it was 95% complete (4934.4 patient-years). RESULTS: Cusp prolapse was repaired in 83%, and an annuloplasty was added in 353 instances (51.6%). Hospital mortality was 2.3%, and survival was 81.7% (SD: 1.2) and 55.7% (SD: 5.8) at 10 and 20 years; age and measurement of effective height were independent predictors for death. Freedom from Aortic insufficiency (AI) II was 90.5 (SD: 1.9) at 10 years and 76.7 (SD: 4.5) at 20 years. Cusp repair of all cusps showed a lower freedom from recurrent AI ≥II at 10 years (P < 0.001). Suture annuloplasty showed a lower freedom from recurrent AI II at 10 years (P = 0.07). Freedom from reoperation was 95.5 (SD: 1.1) and 92.8 (SD: 2.8) at 10 and 20 years. The addition of an annuloplasty showed no difference (P = 0.236). Cusp repair had no effect on valve durability (P = 0.390). CONCLUSIONS: Root remodelling leads to good long-term stability. The addition of cusp repair improves the valve stability over time. The addition of suture annuloplasty improves early valve competency; it showed no effect on freedom from reoperation up to 10 years.

The Ross procedure versus repair for treatment of a unicuspid aortic valve in adults†.

OBJECTIVES: Aortic stenosis or regurgitation in patients with a unicuspid valve morphology requires interventions early in life. We have performed either primary valve repair or the Ross procedure. The goal of this study was to compare the midterm results of repair and pulmonary autograft replacement. METHODS: Between December 1998 and April 2022, a total of 345 patients (77% male; mean age 34 ± 9.7 years) underwent treatment of a unicuspid aortic valve. Patients were excluded if they were <18 years (n = 84) or >54 years (n = 3) at the time of the operation. The remaining cohort was divided into 2 groups: 167 (64%) patients underwent valve repair; 91 (36%) patients underwent pulmonary autograft replacement.The indications for surgery were aortic regurgitation (n = 104), aortic stenosis (n = 45), combined disease (n = 103) and endocarditis (n = 6). Fifty-one patients had root dilatation (>43 mm) with aortic regurgitation (repair n = 23; Ross n = 28). Mean follow-up was 5.9 years (SD: 5 years) [range 0.1-22.3 years]. RESULTS: There were 1 early and 3 late deaths; 47 patients required reintervention. Survival at 10 years was 95% in the Ross group and 97% after valve repair (P = 0.769). Freedom from reintervention at 10 years was 98% in the Ross group and 80% after valve repair (P = 0.012). A receiver operating characteristics curve analysis showed a trend towards better durability in patients < 26 years. CONCLUSIONS: The ideal treatment of the unicuspid aortic valve remains debatable. Repair of a unicuspid valve can be considered a bridge to pulmonary autograft replacement, at least in younger patients. The appropriate times to replace and to repair require further investigation.

Autograft reoperations after the Ross procedure.

OBJECTIVES: After a Ross procedure, autograft failure can occur. At reoperation, repair of the autograft preserves the advantages of the Ross procedure. The aim of this retrospective study was to assess mid-term results after reoperation of a failed autograft. METHODS: Between 1997 and 2022, 30 consecutive patients (83% male; age 41 ± 11 years) underwent autograft reintervention between 60 days and 24 years (median 10 years) after a Ross procedure. The initial technique varied, full-root replacement (n = 25) being the most frequent. The indication for reoperation was isolated autograft regurgitation (n = 7), root dilatation (>43 mm) with (n = 17) or without (n = 2) autograft regurgitation, mixed dysfunction (n = 2) and endocarditis (n = 2). In 4 instances, the valve was replaced by valve (n = 1) or combined valve and root replacement (n = 3). Valve-sparing procedures consisted of isolated valve repair (n = 7) or root replacement (n = 19), and tubular aortic replacement. Cusp repair was performed in all but 2. Mean follow-up was 5.4 ± 6 years (35 days to 24 years). RESULTS: Mean cross-clamp and perfusion times were 74 ± 26 and 132 ± 64 min. There were 2 perioperative deaths (7%; both valve replacement) and 2 patients died late (32 days to 1.2 years postoperatively). Freedom from cardiac death at 10 years was 96% after valve repair and 50% after replacement. Two patients required reoperation (1.68 and 16 years) following repair. One underwent valve replacement for cusp perforation, the other, root remodelling for dilatation. Freedom from autograft reintervention at 15 years was 95%. CONCLUSIONS: Autograft reoperations after the Ross procedure can be performed as valve-sparing operations in the majority of cases. With valve-sparing, long-term survival and freedom from reoperation are excellent.

Twenty-five years' experience with root remodeling and bicuspid aortic valve repair.

Background: Bicuspid aortic valves may be associated with ascending aortic aneurysm, or develop severe aortic regurgitation with variable aortic dilatation. If aortic dilatation involves the root, valve-preserving root replacement is a treatment option, and we prefer root remodeling for this purpose. The objective of this study is to review our experience encompassing 25 years. Methods: Between November 1995 and August 2021, 472 patients (429 male; age 9-80 years; mean 48±13 years) were treated by bicuspid aortic valve repair and root remodeling. Aortic regurgitation was present in 322 cases. The primary indication for surgery was aortic regurgitation (n=317), aortic aneurysm (n=143) or acute type A aortic dissection (n=12). In 271 instances, a suture annuloplasty was added. Cusp calcification was present beyond the raphe in 80 cases, and a pericardial patch was used for partial cusp replacement in 44 cases. Follow-up was 92.8% complete with a mean of 71±68 months (median 61 months). Results: Hospital mortality was 0.4% and survival at 20 years was 76.9%. Reoperation was necessary for recurrent aortic regurgitation in 26 patients; nine patients underwent reoperation for stenosis. The overall freedom of reoperation was 90.5% after ten years and 76.6% after 20 years. Annuloplasty was associated with a higher proportion of competent aortic valves at discharge (P=0.001), and had no effect on ten-year freedom from reoperation. The use of a pericardial patch for cusp repair was a predictor for reoperation (P=0.003). The presence of cusp calcification was a predictor for the development of aortic stenosis and reoperation (P=0.032). Conclusions: Bicuspid aortic valve repair combined with root remodeling leads to excellent ten- and 20-year results. Cusp calcification and partial cusp replacement are associated with an increased probability of valve failure requiring reoperation.

Ross Operation With Autologous External Autograft Stabilization-Long-term Results.

BACKGROUND: We have proposed an external stabilization technique to minimize autograft dilatation after the Ross operation. This study analyzed autograft function and root dimensions after root replacement with and without external root support. METHODS: Between October 1995 and February 2021, 185 adult patients (73% male; age, 38 ± 9 years) underwent a Ross operation as full-root replacement, 136 with support and 49 without support. Autograft function and root dimensions were determined echocardiographically. Median follow-up was 3.4 years (interquartile range, 1.13-14.74 years) and was 95% complete. Survival and freedom from reoperation were calculated. Changes in autograft root dimensions were analyzed using mixed-effect models. RESULTS: Survival (95%) and freedom from autograft reoperation (94%) at 15 years were higher with than without support (P = .003 or P = .004). In the first 5 years, patients with support showed an indexed root size progression of 0.712 mm/(y ∙ m) (P = .003) compared with 1.554 mm/(y ∙ m) (P = .001) without. Progression rates were higher for patients without stabilization (P = .045). After 5 years, progression rates were similar in both groups at 0.248 mm/(year ∙ m) (P < .001) but with persistent a difference between the groups. CONCLUSIONS: The Ross procedure as full-root replacement is associated with a relevant rate of autograft dilatation and reoperation. The use of external root stabilization may reduce autograft dilatation and is associated with improved survival and durability.

Ross operation after failure of aortic valve repair.

BACKGROUND: Repair failure remains one of the most important complications of aortic valve reconstruction. Young patients might benefit from a Ross procedure in such a scenario, provided it can be performed safely and with adequate durability. The aim of this study was to assess the safety and clinical outcomes of a Ross operation following a failed repair. METHODS: Between 1996 and 2019, 80 patients (male, 76%; mean age, 31±13 years) underwent a Ross procedure after a median of 6.6 (1.7-15.9) years following an initial aortic valve repair. The previous valve repair was performed for unicuspid (53%), bicuspid (39%), tricuspid (7%), and quadricuspid morphology (1%). Median follow-up after the Ross operation was 2.8 (0.964-13.25) years, mean 5±5 years (92% complete). RESULTS: Median cardiopulmonary bypass and cross-clamp times were 144 [106-154] minutes and 98 [79-113] minutes, respectively. Thirty-two patients (40%) required a concomitant procedure, most commonly, an ascending aortic replacement (n=23). There were no peri-operative deaths, myocardial infarctions, or neurological complications. There was one late death from a non-cardiac cause. At 10 years, overall survival was 99%±1%, similar to that of an age- and gender-matched population. Nine patients required re-intervention after their Ross procedure (five on the autograft and four on the pulmonary conduit). The autograft re-interventions were valve-sparing procedures in all patients. The cumulative incidence of re-intervention on the autograft at 8 years was 5.1%±3.1%. CONCLUSIONS: The stepwise strategy of an initial valve repair followed by Ross operation represents a safe and valid option for failed aortic valve repair. It is associated with low peri-operative morbidity. Mid-term survival is excellent, similar to that of a matched general population. The probability of re-intervention after the Ross procedure appears similar to that of a primary Ross operation, deeming it a warranted consideration in cases of failed aortic valve repair.

Which Aortic Valve Can Be Surgically Reconstructed?

PURPOSE OF REVIEW: Preservation or repair of the aortic valve has evolved dynamically in the past 20 years. It leads to a high freedom from valve-related complications if an adequate valve durability can be achieved; it may possibly also improve survival. To date, little structured information is available about which valves can be repaired and which should better be replaced. RECENT FINDINGS: For surgical decision-making, the size of the aortic root is important and the anatomy of the aortic valve must be considered. In the presence of root aneurysm, most tricuspid and bicuspid aortic valves can be preserved. In aortic regurgitation and normal aortic dimensions, the majority of tricuspid and bicuspid aortic valves can be repaired with good long-term durability. In bicuspid aortic valves, the morphologic characteristics must be taken into consideration. Unicuspid and quadricuspid aortic valves can be repaired in selected cases. Generally, cusp calcification is a sign of a poor substrate for repair; the same is true for cusp retraction and cusp destruction due to active endocarditis. They are associated with limited valve durability. Using current concepts, many non-calcified aortic valves can be repaired. Modern imaging, in particular three-dimensional transesophageal echocardiography (TEE), should be able to define repairable aortic valves with a high probability.