[Summary: International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes]. / Resumen: Consenso internacional para la nomenclatura y clasificación de la válvula aórtica bicúspide congénita y su aortopatía, con fines clínicos, quirúrgicos, intervencionistas y de investigación.

This consensus of nomenclature and classification for congenital bicuspid aortic valve and its aortopathy is evidence-based and intended for universal use by physicians (both pediatricians and adults), echocardiographers, advanced cardiovascular imaging specialists, interventional cardiologists, cardiovascular surgeons, pathologists, geneticists, and researchers spanning these areas of clinical and basic research. In addition, as long as new key and reference research is available, this international consensus may be subject to change based on evidence-based data1.

Este consenso de nomenclatura y clasificación para la válvula aórtica bicúspide congénita y su aortopatía está basado en la evidencia y destinado a ser utilizado universalmente por médicos (tanto pediatras como de adultos), médicos ecocardiografistas, especialistas en imágenes avanzadas cardiovasculares, cardiólogos intervencionistas, cirujanos cardiovasculares, patólogos, genetistas e investigadores que abarcan estas áreas de investigación clínica y básica. Siempre y cuando se disponga de nueva investigación clave y de referencia, este consenso internacional puede estar sujeto a cambios de acuerdo con datos basados en la evidencia1.

Systematic adjustment of root dimensions to cusp size in aortic valve repair: a computer simulation.

OBJECTIVES: Aortic valve repair requires the creation of a normal geometry of cusps and aortic root. Of the different dimensions, geometric cusp height is the most difficult to change while annular and sinotubular dimensions can be easily modified. The objective of this study was to investigate, by computer simulation, ideal combinations of annular and sinotubular junction size for a given geometric height. METHODS: Based on a literature review of anatomical data, a computational biomechanics model was generated for a tricuspid aortic valve. We aimed to determine the ideal relationships for the root dimensions, keeping geometric height constant and creating different combinations of the annular and sinotubular junction dimensions. Using this model, 125 virtual anatomies were created, with 25 different combinations of annulus and sinotubular junction. Effective height, coaptation height and mechanical cusp stress were calculated with the valves in closed configuration. RESULTS: Generally, within the analysed range of geometric heights, changes to the annular diameter yielded a stronger impact than sinotubular junction diameter changes for optimal valve configuration. The best results were obtained with the sinotubular junction being 2-4 mm larger than the annulus, leading to higher effective height, normal coaptation height and lower stress. Within the range tested, stenosis did not occur due to annular reduction. CONCLUSIONS: In tricuspid aortic valves, the geometric height can be used to predict ideal post-repair annular and sinotubular junction dimensions for optimal valve configuration. Such an ideal configuration is associated with reduced cusp stress.

Management of Adults With Anomalous Aortic Origin of the Coronary Arteries: State-of-the-Art Review.

As a result of increasing adoption of imaging screening, the number of adult patients with a diagnosis of anomalous aortic origin of the coronary arteries (AAOCA) has grown in recent years. Existing guidelines provide a framework for management and treatment, but patients with AAOCA present with a wide range of anomalies and symptoms that make general recommendations of limited applicability. In particular, a large spectrum of interventions can be used for treatment, and there is no consensus on the optimal approach to be used. In this paper, a multidisciplinary group of clinical and interventional cardiologists and cardiac surgeons performed a systematic review and critical evaluation of the available evidence on the interventional treatment of AAOCA in adult patients. Using a structured Delphi process, the group agreed on expert recommendations that are intended to complement existing clinical practice guidelines.

Management of Adults With Anomalous Aortic Origin of the Coronary Arteries: State-of-the-Art Review.

As a result of increasing adoption of imaging screening, the number of adult patients with a diagnosis of anomalous aortic origin of the coronary arteries (AAOCA) has grown in recent years. Existing guidelines provide a framework for management and treatment, but patients with AAOCA present with a wide range of anomalies and symptoms that make general recommendations of limited applicability. In particular, a large spectrum of interventions can be used for treatment, and there is no consensus on the optimal approach to be used. In this paper, a multidisciplinary group of clinical and interventional cardiologists and cardiac surgeons performed a systematic review and critical evaluation of the available evidence on the interventional treatment of AAOCA in adult patients. Using a structured Delphi process, the group agreed on expert recommendations that are intended to complement existing clinical practice guidelines.

ACC/AHA and ESC/EACTS Guidelines for the Management of Valvular Heart Diseases: JACC Guideline Comparison.

Valvular heart disease (VHD) is common and poses important challenges from the standpoints of diagnosis and therapeutic management. Clinical practice guidelines have been developed to help health care professionals to overcome these challenges and provide optimal management to patients with VHD. The American College of Cardiology, in collaboration with the American Heart Association, and the European Society of Cardiology, in collaboration with the European Association for Cardio-Thoracic Surgery, recently updated their guidelines on the management of VHD. Although these 2 sets of guidelines are generally concordant, there are some substantial differences between these guidelines, which may have significant implications for clinical practice. This review prepared on behalf of the EuroValve Consortium describes the consistencies and discrepancies between the guidelines and highlights the gaps in these guidelines and the future research perspectives to fill these gaps.

Aortic Annuloplasty: Focus on the Use of an External Teflon Ring.

Aortic annuloplasty has been clearly demonstrated to have a protective influence in aortic valve repair. Over the past 20 years, different annuloplasty concepts have been proposed by different groups. However, the most appropriate approach to enable long-term annular stability remains highly controversial. The aim of this article is to give a general overview of all types of aortic annuloplasty, particularly focusing on the use of an external Teflon ring, as proposed by our group. In this technique, external root dissection is performed in the same fashion as for reimplantation; the only difference is that it is necessary to go below the coronary ostia take-off. A series of pledgeted sutures (usually between 6 and 9 sutures) are placed at the level of the virtual basal ring. The external ring is made using a Teflon strip with a length of 8 to 9 cm, to reduce the annulus to a diameter of between 21 and 23 mm. The sub-annular sutures are then passed at the appropriate level through the Teflon strip and the strip is parachuted outside the aortic root base, passing under the coronary ostia. The two ends of the Teflon strip are tied at the level of the non-coronary sinus. Aortic annuloplasty is a crucial step to improve valve competence and stabilization. While several techniques offer good mid- to long-term results, annuloplasty with an external Teflon ring appears to be a simple and effective alternative to guarantee stable root diameters. Longer follow-up studies are needed to confirm the mid- to long-term results.

Durability of bioprosthetic aortic valve replacement in patients under the age of 60 years - 1-year follow-up from the prospective INDURE registry.

OBJECTIVES: We report 1-year safety and clinical outcomes in patients <60 years undergoing bioprosthetic surgical aortic valve intervention. METHODS: The INSPIRIS RESILIA Durability Registry is a prospective, multicentre registry to assess clinical outcomes of patients <60 years. Patients with planned SAVR with or without concomitant replacement of the ascending aorta and/or coronary bypass surgery were included. Time-related valve safety, haemodynamic performance and quality of life (QoL) at 1 year were assessed. RESULTS: A total of 421 patients were documented with a mean age of 53.5 years, 76.5% being male and 27.2% in NYHA class III/IV. Outcomes within 30 days included cardiovascular-related mortality (0.7%), time-related valve safety (VARC-2; 5.8%), thromboembolic events (1.7%), valve-related life-threatening bleeding (VARC-2; 4.3%) and permanent pacemaker implantation (3.8%). QoL was significantly increased at 6 months and sustained at 1 year. Freedom from all-cause mortality at 1 year was 98.3% (95% confidence interval 97.1; 99.6) and 81.8% were NYHA I versus 21.9% at baseline. No patient developed structural valve deterioration stage 3 (VARC-3). The mean aortic pressure gradient was 12.6 mmHg at 1 year and the effective orifice area was 1.9 cm2. CONCLUSIONS: The 1-year data from the INSPIRIS RESILIA valve demonstrate good safety and excellent haemodynamic performance as well as an early QoL improvement. CLINICAL TRIAL REGISTRATION: clinicaltrials.gov: NCT03666741.

GPU accelerated digital twins of the human heart open new routes for cardiovascular research.

The recruitment of patients for rare or complex cardiovascular diseases is a bottleneck for clinical trials and digital twins of the human heart have recently been proposed as a viable alternative. In this paper we present an unprecedented cardiovascular computer model which, relying on the latest GPU-acceleration technologies, replicates the full multi-physics dynamics of the human heart within a few hours per heartbeat. This opens the way to extensive simulation campaigns to study the response of synthetic cohorts of patients to cardiovascular disorders, novel prosthetic devices or surgical procedures. As a proof-of-concept we show the results obtained for left bundle branch block disorder and the subsequent cardiac resynchronization obtained by pacemaker implantation. The in-silico results closely match those obtained in clinical practice, confirming the reliability of the method. This innovative approach makes possible a systematic use of digital twins in cardiovascular research, thus reducing the need of real patients with their economical and ethical implications. This study is a major step towards in-silico clinical trials in the era of digital medicine.

Surgical approach to the suprarenal abdominal aorta.

AIM: The surgical approach to the pararenal aorta can be performed through a midline laparotomy or retroperitoneal approach. The current paper reports the techniques for the suprarenal aortic approach, through the review of technical literature on this topic. METHODS: Forty-six out of 82 technical papers regarding the surgical approach to the suprarenal aorta were reviewed, focusing on relevant technical details, such as the position of patient, type of incision, aortic approach and anatomical limitations. RESULTS: The left retroperitoneal abdominal approach offers numerous advantages, mainly observing some modifications of the original technique (9th intercostal space incision, short radial frenotomy, section of the inferior mesenteric artery). The traditional transperitoneal access, through a midline or bilateral subcostal incision with retroperitoneal medial visceral rotation, is best indicated when an unrestricted approach to the right iliac arteries is needed, but it can be more challenging in patients with "hostile abdomen", for which a retroperitoneal route is probably more appropriate. A more aggressive surgical approach through a 7th-9th space thoracolaparotomy, combined with semicircunferential frenotomy, should be strongly recommended to provide a safe suprarenal aortic aneurysm repair in high risk patients, who often require adjunctive procedures, such as selective visceral perfusion and left heart bypass. CONCLUSIONS: Many technical options can be used to approach the suprarenal aorta, but none can be "radicalized". The surgical strategy must be individualized according to the anatomo-clinical characteristics of the patient and aneurysm morphology as well. KEY WORDS: Abdominal aorta, Aortic aneurysm, Surgical approach.

Formal consensus study on surgery to replace the aortic valve in adults aged 18-60 years.

OBJECTIVE: There is uncertainty about surgical procedures for adult patients aged 18-60 years undergoing aortic valve replacement (AVR). Options include conventional AVR (mechanical, mAVR; tissue, tAVR), the pulmonary autograft (Ross) and aortic valve neocuspidisation (Ozaki). Transcatheter treatment may be an option for selected patients. We used formal consensus methodology to make recommendations about the suitability of each procedure. METHODS: A working group, supported by a patient advisory group, developed a list of clinical scenarios across seven domains (anatomy, presentation, cardiac/non-cardiac comorbidities, concurrent treatments, lifestyle, preferences). A consensus group of 12 clinicians rated the appropriateness of each surgical procedure for each scenario on a 9-point Likert scale on two separate occasions (before and after a 1-day meeting). RESULTS: There was a consensus that each procedure was appropriate (A) or inappropriate (I) for all clinical scenarios as follows: mAVR: total 76% (57% A, 19% I); tAVR: total 68% (68% A, 0% I); Ross: total 66% (39% A, 27% I); Ozaki: total 31% (3% A, 28% I). The remainder of percentages to 100% reflects the degree of uncertainty. There was a consensus that transcatheter aortic valve implantation is appropriate for 5 of 68 (7%) of all clinical scenarios (including frailty, prohibitive surgical risk and very limited life span). CONCLUSIONS: Evidence-based expert opinion emerging from a formal consensus process indicates that besides conventional AVR options, there is a high degree of certainty about the suitability of the Ross procedure in patients aged 18-60 years. Future clinical guidelines should include the option of the Ross procedure in aortic prosthetic valve selection.

Truncating variants in the penultimate exon of TGFBR1 escaping nonsense-mediated mRNA decay cause Loeys-Dietz syndrome.

Pathogenic variants in TGFBR1 are a common cause of Loeys-Dietz syndrome (LDS) characterized by life-threatening aortic and arterial disease. Generally, these are missense changes in highly conserved amino acids in the serine-threonine kinase domain. Conversely, nonsense, frameshift, or specific missense changes in the ligand-binding extracellular domain cause multiple self-healing squamous epithelioma (MSSE) lacking the cardiovascular phenotype. Here, we report on two novel variants in the penultimate exon 8 of TGFBR1 were identified in 3 patients from two unrelated LDS families: both were predicted to cause frameshift and premature stop codons (Gln448Profs*15 and Cys446Asnfs*4) resulting in truncated TGFBR1 proteins lacking the last 43 and 56 amino acid residues, respectively. These were classified as variants of uncertain significance based on current criteria. Transcript expression analyses revealed both mutant alleles escaped nonsense-mediated mRNA decay. Functional characterization in patient's dermal fibroblasts showed paradoxically enhanced TGFß signaling, as observed for pathogenic missense TGFBR1 changes causative of LDS. In summary, we expanded the allelic repertoire of LDS-associated TGFBR1 variants to include truncating variants escaping nonsense-mediated mRNA decay. Our data highlight the importance of functional studies in variants interpretation for correct clinical diagnosis.

Aortic root anatomy after aortic valve reimplantation.

OBJECTIVE: During the last decade, special concerns have been raised about the anatomic relationships among the sinotubular junction, ventricular-aortic junction, and virtual basal ring to improve the results of root reconstruction. The aim of this study is to evaluate the in vivo anatomy of the aortic root after reimplantation with the Valsalva graft and the anatomic relationship between its components. METHODS: We analyzed 10 consecutive patients with tricuspid aortic valves who underwent reimplantation with the Valsalva graft between September and December 2019. Surgical clips were applied as markers at the level of proximal annular knots and at the distal reimplanted commissures on the neo-sinotubular junction. Electrocardiogram-gated computed tomography scan of the aortic root was performed. Coordinates of the markers were exported on a 3-dimensional modeling software, and the distances between the virtual basal ring and the Dacron graft basal landmarks were measured. RESULTS: The mean heights of Dacron graft basal landmarks from virtual basal ring were right-left commissure 7.1 ± 5.1 mm; right sinus 4.7 ± 4.1 mm; right-noncoronary commissure 2.8 ± 2.2 mm; noncoronary sinus 1.4 ± 1.6 mm; left-noncoronary commissure 2.2 ± 2.3 mm; and left sinus 2.0 ± 0.9 mm. The mean planar distances of basal Dacron graft landmarks from virtual basal ring (thickness) were right-left commissure 5.3 ± 3.1 mm; right sinus 2.8 ± 1.4 mm; right-noncoronary commissure 2.2 ± 1.5 mm; noncoronary sinus 1.5 ± 1.5 mm; left-noncoronary commissure 1.3 ± 1.0 mm; and left sinus 3.4 ± 2.5 mm. CONCLUSIONS: After reimplantation, despite a complete dissection of the root, slight asymmetry of graft proximal seating exists. The inner annuloplasty is on the virtual basal ring, and the proximal edge of the Dacron graft is on the ventricular-aortic junction at a slightly different thickness and height along the annular circumference. At the level of the right sinus and left/right commissure, the Dacron graft is higher than the virtual basal ring and the relative wall thickness is increased. The annular stabilization is unaffected.

Twenty-year experience of aortic valve reimplantation using the Valsalva graft.

OBJECTIVES: Over the past 20 years, valve-sparing aortic root replacement has aroused increasing interest because of a progressive attitude towards the preservation of natural tissue. Aortic reimplantation is the most used technique to spare the valve, allowing simultaneously aortic root replacement and aortic annular stabilization. The reimplantation into a graft with sinuses guarantees an optimal anatomic and functional reconstruction with established good results at 15 years. The aim of this study is to report the world longest follow-up (up to 20 years) of aortic valve reimplantation using the Valsalva graft. METHODS: From February 2000 to December 2021, 265 consecutive patients with aortic root aneurysm received aortic valve reimplantation using the Valsalva graft. From 2018, leaflet plication with the routine use of calliper was performed. For each patient, we performed both intraoperative and post-procedural transoesophageal echocardiography. All patients were followed with clinical assessment and echocardiography. The mean duration of follow-up was 85 ± 63 months. RESULTS: The study cohort had a median age of 55 ± 18 and 87.2% were male. The aortic root aneurysm was associated to bicuspid aortic valve in 18.9% of patients and to Marfan syndrome in 10.6% of cases. 55.9% had an aortic regurgitation ≥ 2+. Overall survival at 15 was 87.6 ± 3.4. Freedom from cardiac death was stable at 99.6 ± 0.4 at 5, 10 and 15 years. Freedom from recurrent AR ≥3+ and freedom from reoperation remained stable at 10 and 15 years at 92.2 ± 2.1 and 95.9 ± 1.6, respectively. There was a minimal incidence of infective endocarditis (0.8%), thromboembolism (2.2%) and haemorrhage (2.0%). Six out of 7 patients requiring reoperation had surgery in the first period of our experience (last in 2004). Early suboptimal results had a negative effect on residual aortic regurgitation. Moreover, we hypothesized that the routine use of calliper may have contributed to a further improvement of the outcome, even if these data need to be confirmed by a longer follow-up. CONCLUSIONS: The first long-term follow-up after aortic valve reimplantation using the Valsalva graft demonstrated excellent results. These long-term results gradually improved with learning curve, remaining stable during the second decade of observation. The systematic use of calliper may have contributed to a further improvement of the outcome.

Mitral repair of myxomatous valves with simple annuloplasty: a follow-up up to 12 years.

OBJECTIVES: Diffuse myxomatous mitral valve degeneration (DMD) represents a challenge in the reparative mitral valve surgery. A subgroup of patients with symmetrical DMD can be effectively treated with a simple band-annuloplasty with good early and mid-term results. Here, we evaluate the long-term outcomes in terms of freedom from reoperation, recurrence of moderate or severe mitral regurgitation (MR) and overall survival. METHODS: Between April 2006 and December 2020, patients with DMD causing severe MR and the echocardiographic features of symmetrical bileaflet prolapse, central regurgitant jet(s), annular dilation and no chordal ruptures were treated using a simple annuloplasty with a semi-rigid band. These patients were prospectively collected and retrospectively analysed. RESULTS: Seventy-five patients were enrolled. The mean clinical follow-up time was 104 [standard deviation (SD): 43] months, and echocardiographic follow-up time was 95 (SD: 43) months. The mean age was 54 (SD: 15) years, and 56% were females. Long-term overall survival was 98.2% [standard error (SE): 1.8], 93.7% (SE: 4.7) and 93.7% (SE: 4.7) at 4, 8 and 12 years, respectively. The freedom from reoperation was 100% at 4 and 8 years and 94.1% (SE: 5.7) at 12 years. The freedom from recurrent moderate or severe MR was 98.3% (SE: 1.7), 98.3% (SE: 1.7) and 92.8% (SE: 5.5) at 4, 8 and 12 years, respectively. CONCLUSIONS: Mitral repair with the simple band-annuloplasty for the treatment of MR due to symmetrical DMD seems to be stable and effective in the long term.