Long-Term Outcomes Following the Ross Procedure in Neonates and Infants: A Multi-Institutional Analysis.

OBJECTIVES: For neonates and infants with aortic valve pathology, the Ross procedure has historically been associated with high rates of morbidity and mortality. Data regarding long-term durability are lacking. METHODS: The international, multi-institutional Ross Collaborative included six tertiary-care centers. Infants who received a Ross operation between 1996-2016 (allowing a minimum five years of follow-up) were retrospectively identified. Serial echocardiograms were examined to study evolution in neoaortic size and function. RESULTS: Primary diagnoses for the 133 patients (n=30 neonates) included isolated aortic stenosis (AS; 14%, n=19), Shone complex (14%, n=19), and AS+other (excluding Shone complex; n=95, 71%) including arch obstruction (n=55), left ventricular hypoplasia (n=9), and mitral disease (>moderate stenosis or regurgitation, n=31). At the time of Ross, median age was 96 (IQR 36-186) days and median weight was 4.4 (3.6-6.5) kg. In-hospital mortality occurred in 13/133 (10%) patients (4/30 [13%] neonates). Post-discharge mortality occurred in 10/120 (8%) patients at a median 298 days post-Ross. Post-Ross neoaortic dilatation occurred, peaking at 4-5 standard deviations above normal at 2-3 years before returning to near-baseline z-score at a median follow-up of 11.5 [6.4-17.4] years. Autograft/LVOT reintervention was required in 5/120 (4%) patients at a median 10.3 [4.1-12.8] years. Freedom from >moderate neoaortic regurgitation (AR) was 86% at 15 years. CONCLUSIONS: Neonates and infants experience excellent post-discharge survival and long-term freedom from autograft reintervention and AR following Ross. Neoaortic dilatation normalizes in this population in the long-term. Increased consideration should be given to Ross in neonates and infants with aortic valve disease.

Migration of the left atrial appendage closure device to the aorta.

Percutaneous closure of the left atrial appendage may be indicated in patients with contraindications to anticoagulation therapy, for example, after recurrent gastrointestinal bleeding. It is an effective and safe procedure but is not without complications. We present a patient who presented with severe aortic insufficiency due to migration of the left atrial appendage closure device, which required urgent cardiac surgery for its removal.

Asymptomatic, Incidental Quadricuspid Aortic Valve: A Case Report and Discussion of Management.

With its exceeding rarity, there is little research on the quadricuspid aortic valve (QAV) inherently to drive guideline-based management. This leaves physicians without evidence-based guidance on the management of such patients should they come across this finding on imaging or should they care for a symptomatic patient. This article describes the case of an incidentally identified QAV in a patient undergoing treatment for tuberculosis, which seemingly had bicuspid-appearing valve hemodynamics. Additionally, current literature is reviewed to describe classification, presentation, complications, and intervention, with additional exploration and commentary on the lack of guideline-based care.

Management of aortic valve insufficiency in patients with continuous-flow left ventricular assist device: a republication of the review published in Japanese Journal of Artificial Organs.

Since 2011, implantable ventricular assist devices have been a standard treatment for severe heart failure alongside heart transplantation in Japan. However, the limited availability of donors has led to a prolonged wait for transplants, now averaging 1719 days, intensifying the issue of aortic insufficiency in patients with continuous flow ventricular assist devices. These devices limit the opening of the aortic valve, leading to sustained closure and increased shear stress, which accelerates valve degradation. Risk factors for aortic insufficiency include having a smaller body surface area, being of advanced age, and the presence of mild aortic insufficiency prior to device implantation. In patients presenting with mild or moderate aortic regurgitation at the time of ventricular assist device implantation, interventions such as aortic valve repair or bioprosthetic valve replacement are performed with the aim of halting its progression. The choice of surgical procedure should be tailored to each patient's individual condition. The management of de novo aortic insufficiency in patients with continuous flow ventricular assist devices remains challenging, with no clear consensus on when to intervene. Interventions for significant aortic insufficiency typically consider the patient's symptoms and aortic insufficiency severity. De novo aortic insufficiency progression in continuous flow ventricular assist devices patients necessitates careful monitoring and intervention based on individual patient assessments and valve condition. This review was created based on a translation of the Japanese review written in the Japanese Journal of Artificial Organs in 2023 (Vol. 52, No. 1, pp. 77-80), with some modifications.

Transcatheter aortic valve implantation for pure aortic insufficiency in a patient following David-type aortic valve-sparing root replacement after aortic dissection-A successful off-label procedure.

Transcatheter aortic valve implantation (TAVI) has traditionally been indicated for the treatment of aortic stenosis. However, in this case report, we describe a successful TAVI procedure in a 46-year-old male patient who had previously undergone David aortic valve-sparing aortic root replacement for type 1 aortic dissection. The patient presented with aortic valve insufficiency 4 years after the initial surgery and was subsequently treated with a 34 mm Medtronic CoreValve Evolut R prosthesis via TAVI. This case highlights the feasibility of TAVI as a viable treatment option for postoperative aortic valve insufficiency in patients with prior ascending aortic or aortic arch surgery.

Treatment options for isolated aortic valve insufficiency: a review.

Aortic insufficiency (AI) is a valvular disease with increasing prevalence in older patients. The modern era provides numerous options for the management of AI which is explored here. Traditional interventions included aortic valve replacement with either mechanical or bioprosthetic aortic valves. While the former is known for its durability, it has grown out of favor due to the potential complications of anticoagulation. The preference for bioprosthetic valves is thus on the rise, especially with the advancements of transcatheter technology and the use of valve-in-valve therapy. Surgical options are also no longer limited to replacement but include complex techniques such as those required for aortic valve repair, Ozaki neocuspidization, Ross procedure and valve-sparring aortic root repair. Transcatheter options for the management of AI are not commercially available currently; however, preliminary data from ad-hoc trials, showed promising results and potential use of transcatheter technology in a variety of patients with pure AI.

Simultaneous management of aortic and mitral regurgitation through one-stage transcatheter aortic valve replacement and transcatheter edge-to-edge repair: case report.

This case report presents a 72-year-old male patient who presented with exertional dyspnea for over 10 years, which had progressively worsened over the past 4 months. Transthoracic echocardiography revealed severe aortic and mitral regurgitation, with a left ventricular ejection fraction of 37% and a left ventricular end-diastolic diameter of 64 mm. Despite receiving long-term optimal medical management, there was no improvement in symptoms or severity of valvular regurgitation. Given the relatively high surgical risk associated with double valve replacement in this elderly patient and his preference for minimally invasive procedures, a one-stage transapical aortic valve replacement and transcatheter mitral valve repair using the edge-to-edge technique were planned. The patient was discharged 8 days post-procedure without any complications. At 1-month follow-up, the patient's New York Heart Association (NYHA) functional class had improved to grade II.

Aortic regurgitation is associated with African American and Asian race, smoking, renal disease, and numerous autoimmune diseases in addition to traditional cardiovascular risk factors but has lower risk with alcohol intake.

BACKGROUND: Aortic regurgitation (AR) is associated with increasing age, rheumatic heart disease, and a bicuspid aortic valve, but its association with other comorbidities and race is less known. The purpose of this study was to investigate any association between AR and comorbid conditions in older adults above 40. METHOD: The large Nationwide Inpatient Sample database was utilized for our study using uni- and multivariate analysis. Data were extracted from available ICD-10 codes for the years of 2016-2020. RESULTS: The NIS data included 112,982,565 patients. A total of 660,730 were found to have AR. AR was found to be associated with male gender (OR 1.15, CI 1.14-1.16, P < 0.001), smoking (OR 1.04, CI 1.02-1.05, P < 0.001), hypertension (OR 1.65, CI 1.62-1.68, P < 0.001), hyperlipidemia (OR 1.36, CI 1.34-1.37, P < 0.001), chronic kidney disease (OR 1.22, CI 1.21-1.24, P < 0.001), antiphospholipid antibody syndrome (OR 1.56, CI 1.33-1.83, P < 0.001), rheumatoid arthritis (OR 1.1, CI 1.06-1.14, P < 0.001), scleroderma (OR 1.49, CI 1.31-1.7, P < 0.001), systemic connective tissue disorders (OR 1.32, CI 1.25-1.4, P < 0.001), Raynaud's syndrome (OR 1.62, CI 1.47-1.77, P < 0.001), and systemic lupus erythematosus (OR 1.44, CI 1.34-1.54, P < 0.001) in add to known bicuspid aortic valve. CONCLUSION: Using a very large database, we found new associations between AR and many comorbid conditions, including many inflammatory and chronic degenerative diseases in addition to the known risk factors.

Bicuspid Aortic Valve Endocarditis Caused by Streptococcus sanguinis: A Case Report.

Antibiotic prophylaxis prior to dental work in bicuspid aortic valve (BAV) patients is currently a matter of debate. The American Dental Association does not require those with native BAV to receive antibiotic prophylaxis prior to dental work as BAV is considered an "intermediate" risk for infective endocarditis (IE). We present the case of a 63-year-old male, with a medical history of BAV, who acquired Streptococcus sanguinis IE after a routine dental cleaning four months prior to initial onset of symptoms. He exhibited new-onset and severe aortic regurgitation at presentation, requiring urgent aortic valve replacement to restore valve function. BAV patients are at high risk of IE, emphasizing the need for prophylactic antibiotics in dental cleaning as well as invasive dental procedures in those with BAV.

Clover stitch technique for aortic valve in mitral valve repair.

In the case of mitral repair with severe aortic regurgitation, aortotomy and selective cardioplegia are necessary for myocardiac protection. In this situation, the saline test for mitral valve repair cannot be accomplished due to incomplete left ventricular filing. In patients undergoing mitral valve repair concomitant with severe aortic valve insufficiency, after cardiac stand still was achieved by selective cardioplegia. Each center of the aortic leaflet, termed the node of Arantius, was stitch up using a 5-0 polypropylene suture, forming a clover leaflet shape. This stitch inhibits aortic valve opening and reduces saline leakage thorough aortic valve. We have termed this procedure as the "Clover Stitch Technique". Upon completion of this technique, mitral valve repair can be undertaken via a right-side left atrial incision. This technique enables accurate evaluation of mitral valve morphology or the extent of regurgitation, repeatedly without complicated manipulations during and after mitral valve repair.

Aortic insufficiency following balloon aortic valvuloplasty does not impact long-term autograft durability after the Ross procedure.

OBJECTIVE: Preoperative aortic insufficiency (AI) is associated with inferior autograft durability after the Ross procedure. However, many patients with aortic stenosis (AS) undergo balloon aortic valvuloplasty (BAV) early and present with longstanding AI before Ross. We studied how BAV and subsequent valvular pathology impacts autograft durability. METHODS: Patients undergoing the Ross operation from 1993 to 2020 were identified. Those who underwent BAV before Ross were compared with patients who did not undergo BAV and underwent Ross for predominant AI (AI group) or AS (AS group). Those who underwent previous open surgical aortic valve intervention were excluded. Primary outcome of interest was autograft failure, defined as a composite of autograft reintervention or severe insufficiency. RESULTS: A total of 198 patients were included. Seventy-nine (39.9%) underwent BAV and subsequently underwent the Ross for predominant AI (45.6%) or AS (54.4%). Of patients who did not undergo BAV, 66 (33.3%) presented with predominant AI and 53 (26.8%) with AS. Freedom from autograft failure at 15 years was 90%, 92%, and 62% in BAV, AS, and AI groups, respectively. The AI group was at significantly increased risk of long-term autograft failure (hazard ratio, 5.6; P = .01), whereas the AS and BAV groups had similar, low risk (hazard ratio, 1.1; P = .91). Autograft durability was similar among patients who received BAV and presented with AS or AI before the Ross (P = .84). CONCLUSIONS: BAV before the Ross procedure is common in patients with AS. These patients have excellent long-term autograft durability regardless of preoperative valvular pathology and should strongly be considered for the Ross operation.

Influence of preoperative aortic regurgitation on long-term autograft durability and dilatation in children and adolescents undergoing the Ross procedure.

OBJECTIVE: Primary aortic insufficiency (AI) is a risk factor for autograft reintervention in adults undergoing the Ross procedure. We sought to examine the influence of preoperative AI on autograft durability in children and adolescents. METHODS: From 1993 to 2020, 125 consecutive patients between ages 1 and 18 underwent a Ross procedure. The autograft was implanted using a full-root technique in 123 (98.4%) and included in a polyethelene terephthalate graft in 2 (1.6%). Patients with aortic stenosis (aortic stenosis group) (n = 85) were retrospectively compared with those with AI or mixed disease (AI group) (n = 40). Median length of follow-up was 8.2 years (interquartile range, 3.3-15.4 years). The primary end point was the incidence of severe AI or autograft reintervention. Secondary end points included changes in autograft dimensions analyzed using mixed-effect models. RESULTS: The incidence of severe AI or autograft reintervention was 39.0% ± 13.0% in the AI group and 8.8% ± 4.4% in the aortic stenosis group at 15 years (P = .02). Annulus z scores increased in both aortic stenosis and AI groups over time (P < .001). However, the annulus dilated at a faster rate in the AI group (absolute difference, 3.8 ± 2.0 vs 2.5 ± 1.7; P = .03). Sinus of Valsalva z scores increased in both groups as well (P < .001), but at similar rates over time (P = .11). CONCLUSIONS: Children and adolescents with AI undergoing the Ross procedure have higher rates of autograft failure. Patients with preoperative AI have more pronounced dilatation at the annulus. Akin to adults, a surgical aortic annulus stabilization technique that modulates growth is needed in children.

Safety and efficacy of aortic valvuloplasty for de novo aortic insufficiency in patients with a left-ventricular assist device.

OBJECTIVES: Progression of aortic insufficiency during left-ventricular assist device (LVAD) support is a crucial topic. One treatment option is aortic valvuloplasty (AVP); however, there is controversy regarding its safety and efficacy. We investigated the safety and efficacy of AVP using the coaptation stitch method (Park's stitch) performed for de novo aortic insufficiency. METHODS: Between 2013 and 2020, 175 consecutive patients underwent LVAD implantation, of which 7 patients [men, 2 (28.6%); median age, 55 years] underwent late-stage AVP. Two patients underwent AVP within 2 weeks, and the remaining six patients underwent AVP 3, 19, 24, 28, 42, and 49 months, respectively, after LVAD implantation. RESULTS: Preoperatively, the degree of aortic insufficiency was moderate in 6 (85.7%) patients and severe in 1 (14.3%) patient. AVP was technically successful in 6 (85.7%) patients, while one case of failed plasty was subsequently treated with bioprosthetic valve replacement. A 1-year post-AVP right heart catheterization study revealed a median pulmonary artery wedge pressure of 10.0 mmHg. No deaths or heart failure admissions occurred during the follow-up (median, 38.0 months). There was no aortic insufficiency in 2 (28.6%) patients; however, trivial AI was observed in 3 (42.8%) patients, and mild AI was observed in 1 (14.3%) patient 2 years postoperatively. However, at the 3-year follow-up, two patients developed an increase in AI grade from trivial to mild. CONCLUSIONS: AVP using Park's stitch was safe. It is critical to carefully observe the aortic valve during AVP surgery to ensure that AVP is appropriate.

Prognostic impact of the E/e' ratio in patients with chronic severe aortic regurgitation undergoing aortic valve replacement.

OBJECTIVES: The study objective was to evaluate the clinical implication of left ventricular diastolic dysfunction in patients with chronic severe aortic regurgitation undergoing aortic valve replacement. METHODS: We reviewed the medical records of 323 patients (age, 56.3 ± 14.1 years; 111 female) who underwent aortic valve replacement for chronic severe aortic regurgitation between 2005 and 2019. Left ventricular diastolic dysfunction was assessed by the ratio of peak left ventricular inflow velocity over mitral annular velocity (E/e'). The study end point was the composite of death and heart failure requiring hospital admission. RESULTS: The E/e' ratio was significantly correlated with age, left atrial dimension, left ventricular end-diastolic volume, mitral regurgitation grade, and tricuspid regurgitation grade (all P < .001). During follow-up (1748.3 patient-years), death and heart failure occurred in 36 patients (2.06/patient-year) and 9 patients (0.53/patient-year), respectively. In multivariable analysis, E/e' ratio (per 5 increment, hazard ratio, 1.32; 95% confidence interval, 1.02-1.71; P = .03), age (hazard ratio, 1.06; 95% confidence interval, 1.03-1.10; P < .001), and left ventricular ejection fraction (hazard ratio, 0.94; 95% confidence interval, 0.90-0.98; P = .002) were independent predictors of death and heart failure. The 5-year heart failure-free survival was 94.9% ± 1.7% in patients with E/e' less than 15% and 84.2% ± 4.2% in patients with E/e' 15 or greater (P < .001). CONCLUSIONS: The E/e' ratio was significantly associated with adverse outcomes in patients with chronic severe aortic regurgitation undergoing aortic valve replacement and may be useful as a prognostic marker in such patients.

The Impact of Left Ventricular Assist Device Outflow Graft Positioning on Aortic Hemodynamics: Improving Flow Dynamics to Mitigate Aortic Insufficiency.

Heart failure is a global health concern with significant implications for healthcare systems. Left ventricular assist devices (LVADs) provide mechanical support for patients with severe heart failure. However, the placement of the LVAD outflow graft within the aorta has substantial implications for hemodynamics and can lead to aortic insufficiency during long-term support. This study employs computational fluid dynamics (CFD) simulations to investigate the impact of different LVAD outflow graft locations on aortic hemodynamics. The introduction of valve morphology within the aorta geometry allows for a more detailed analysis of hemodynamics at the aortic root. The results demonstrate that the formation of vortex rings and subsequent vortices during the high-velocity jet flow from the graft interacted with the aortic wall. Time-averaged wall shear stress (TAWSS) and oscillatory shear index (OSI) indicate that modification of the outflow graft location changes mechanical states within the aortic wall and aortic valve. Among the studied geometric factors, both the height and inclination angle of the LVAD outflow graft are important in controlling retrograde flow to the aortic root, while the azimuthal angle primarily determines the rotational direction of blood flow in the aortic arch. Thus, precise positioning of the LVAD outflow graft emerges as a critical factor in optimizing patient outcomes by improving the hemodynamic environment.

Severe aortic insufficiency-induced cardiogenic shock treated with left atrial VA-ECMO and emergent valve-in-valve TAVR.

Conventional venoarterial extracorporeal membrane oxygenation (VA-ECMO) places a functional afterload burden on the left ventricle. In the setting of acute severe aortic insufficiency-induced cardiogenic shock, the utility of VA-ECMO in combination with a failing valve may result in catastrophic haemodynamic consequences. This challenge is compounded when the culprit is a failing surgical bioprosthetic valve. We present a case of severe rapid-onset bioprosthetic aortic insufficiency-induced cardiogenic shock successfully resuscitated with left atrial VA-ECMO promptly followed by emergent percutaneous valve-in-valve transaortic valve replacement. We discuss the logistics, implications, and associated haemodynamic manifestations in utilizing this strategy for such disease processes.