Diseases of the Aortic Valve and Ascending Aorta: An Overview of Current Knowledge on Pathogenesis, Diagnosis and Treatment

A special issue of Journal of Cardiovascular Development and Disease (ISSN 2308-3425). This special issue belongs to the section "Acquired Cardiovascular Disease".

Deadline for manuscript submissions: closed (15 May 2024) | Viewed by 13716

Special Issue Editors


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Guest Editor
Department of Cardiothoracic Surgery, Weill Cornell Medicine–New York Presbyterian Medical Center, New York, NY 10065, USA
Interests: aortic surgery; thoracoabdominal aneurysm; supra-aortic trunks; end-organ failure; spinal cord protection

Special Issue Information

Dear Colleagues,

In this Special Issue, we aim to focus our attention on the pathology of the aortic valve and ascending aorta with particular emphasis on valve stenosis, infective endocarditis and acute dissection.

Considering aortic valve stenosis, the crucial points of investigation are represented by the evaluation of the stage of disease, disease prevention, timing of aortic valve replacement and optimal treatment option.

Considering infective endocarditis, besides variations in etiology,  management is still highly debated, as some prefer to take advantage of a two-week “cool down” period with antibiotic treatment. Thus, the publication of peer-reviewed clinical and research articles, generating useful immunological and pathogenetic knowledge, are considered the most important tool to improve consensus and, consequently, to guide appropriate diagnostic and clinical choices.

Acute aortic syndrome is caused by a life-threatening disease process involving the aortic wall, and most patients present with a Type A acute aortic dissection which requires emergent surgery. Extensive aortic replacement of the aortic root and aortic arch is constantly debated. It is mandatory to decrease long-term distal aortic events and the risk of perioperative mortality. In this Special Issue, we intend to investigate the outcomes that depend on surgical choices (conservative vs extended surgical strategy), with particular attention directed towards either the replacement or no replacement of the aortic arch.

Dr. Francesco Nappi
Dr. Ivancarmine Gambardella
Guest Editors

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Keywords

  • aortic valve stenosis
  • aortic dissection
  • connective disorder of aortic valve
  • thoracic aorta
  • infective endocarditis
  • pathogenesis
  • treatment

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Published Papers (7 papers)

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Research

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14 pages, 3518 KiB  
Article
The Role of NOTCH Pathway Genes in the Inherited Susceptibility to Aortic Stenosis
by Olga Irtyuga, Rostislav Skitchenko, Mary Babakekhyan, Dmitrii Usoltsev, Svetlana Tarnovskaya, Anna Malashicheva, Yulya Fomicheva, Oksana Rotar, Olga Moiseeva, Ulyana Shadrina, Mykyta Artomov, Anna Kostareva and Evgeny Shlyakhto
J. Cardiovasc. Dev. Dis. 2024, 11(7), 226; https://doi.org/10.3390/jcdd11070226 - 17 Jul 2024
Viewed by 926
Abstract
The NOTCH-signaling pathway is responsible for intercellular interactions and cell fate commitment. Recently, NOTCH pathway genes were demonstrated to play an important role in aortic valve development, leading to an increased calcified aortic valve disease (CAVD) later in life. Here, we further investigate [...] Read more.
The NOTCH-signaling pathway is responsible for intercellular interactions and cell fate commitment. Recently, NOTCH pathway genes were demonstrated to play an important role in aortic valve development, leading to an increased calcified aortic valve disease (CAVD) later in life. Here, we further investigate the association between genetic variants in the NOTCH pathway genes and aortic stenosis in a case–control study of 90 CAVD cases and 4723 controls using target panel sequencing of full-length 20 genes from a NOTCH-related pathway (DVL2, DTX2, MFNG, NUMBL, LFNG, DVL1, DTX4, APH1A, DTX1, APH1B, NOTCH1, ADAM17, DVL3, NCSTN, DTX3L, ILK, RFNG, DTX3, NOTCH4, PSENEN). We identified a common intronic variant in NOTCH1, protecting against CAVD development (rs3812603), as well as several rare and unique new variants in NOTCH-pathway genes (DTX4, NOTCH1, DTX1, DVL2, NOTCH1, DTX3L, DVL3), with a prominent effect of the protein structure and function. Full article
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14 pages, 1987 KiB  
Article
Minimally Invasive and Full Sternotomy Aortic Valve Replacements Lead to Comparable Long-Term Outcomes in Elderly Higher-Risk Patients: A Propensity-Matched Comparison
by Jan Hlavicka, Larissa Gettwart, Julian Landgraf, Razan Salem, Florian Hecker, Enis Salihi, Arnaud Van Linden, Thomas Walther and Tomas Holubec
J. Cardiovasc. Dev. Dis. 2024, 11(4), 112; https://doi.org/10.3390/jcdd11040112 - 31 Mar 2024
Viewed by 1583
Abstract
Background: Minimally invasive aortic valve replacement (AVR) via upper ministernotomy (MiniAVR) is a standard alternative to full sternotomy access. Minimally invasive cardiac surgery has been proven to provide a number of benefits to patients. The aim of this study was to compare the [...] Read more.
Background: Minimally invasive aortic valve replacement (AVR) via upper ministernotomy (MiniAVR) is a standard alternative to full sternotomy access. Minimally invasive cardiac surgery has been proven to provide a number of benefits to patients. The aim of this study was to compare the short- and long-term outcomes after MiniAVR versus conventional AVR via full sternotomy (FS) using a biological prosthesis in an elderly higher-risk population. Methods: Between January 2006 and July 2009, 918 consecutive patients received AVR ± additional procedures with different prostheses at our center. Amongst them, 441 received isolated AVR using a biological prosthesis (median age of 74.5; range: 52–93 years; 50% females) and formed the study population (EuroSCORE II: 3.62 ± 5.5, range: 0.7–42). In total, 137 (31.1%) of the operations were carried out through FS, and 304 (68.9%) were carried out via MiniAVR. Follow-up was complete in 96% of the cases (median of 7.6 years, 6610 patient-years). Propensity score matching (PSM) resulted in two groups of 68 patients with very similar baseline profiles. The primary endpoints were long-term survival, freedom from reoperation, and endocarditis, and the secondary endpoints were early major adverse cardiac and cerebrovascular events (MACCEs). Results: FS led to shorter cardio-pulmonary bypass and aortic cross-clamp durations: 90 (47–194) vs. 100 (46–246) min (p = 0.039) and 57 (33–156) vs. 69 (32–118) min (p = 0.006), respectively. Perioperative stroke occurred in three patients (4.4%; FS) vs. one patient (1.5%; MiniAVR) (p = 0.506). The 30-day mortality was similar in both groups (2.9%, p = 1.000). Survival at 1, 5, and 10 years was 94.1 ± 3% (FS and MiniAVR), 80.3 ± 5% vs. 75.7 ± 5%, and 45.3 ± 6% vs. 43.8 ± 6%, respectively (p = 0.767). There were two (2.9%) reoperations in each group and two thrombo-embolic events (2.9%) vs. one (1.5%) thrombo-embolic event in the MiniAVR and FS groups, respectively (p = 0.596). Conclusions: In comparison to FS, MiniAVR provided similar short- and long-term outcomes in a higher-risk elderly population receiving biological prostheses. In particular, long-term survival, freedom from reoperation, and the incidence of endocarditis were comparable. These results clearly advocate for the routine use of MiniAVR as a standard procedure for AVR, even in a high-risk population. Full article
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14 pages, 2999 KiB  
Article
Potential Clinical Usefulness of Post-Valvular Contrast Densities to Determine the Severity of Aortic Valve Stenosis Using Computed Tomography
by Agnes Orsolya Racz, Gabor Tamas Szabo, Tamas Papp, Benjamin Csippa, Daniel Gyurki, Bertalan Kracsko, Zsolt Koszegi and Rudolf Kolozsvari
J. Cardiovasc. Dev. Dis. 2023, 10(10), 412; https://doi.org/10.3390/jcdd10100412 - 30 Sep 2023
Viewed by 1470
Abstract
Background: Different methods are established for the changes in aortic valve stenosis with cardiac computed tomography angiography (CCTA), but the effect of the grade of stenosis on contrast densities around the valve has not been investigated. Aims/methods: Using the information from flow dynamics [...] Read more.
Background: Different methods are established for the changes in aortic valve stenosis with cardiac computed tomography angiography (CCTA), but the effect of the grade of stenosis on contrast densities around the valve has not been investigated. Aims/methods: Using the information from flow dynamics in cases of increased velocity through narrowed lumen, the hypothesis was formed that flow changes can alter the contrast densities in stenotic post-valvular regions, and the density changes might correlate with the grade of stenosis. Forty patients with severe aortic stenosis and fifteen with a normal aortic valve were enrolled. With echocardiography, the peak/mean transvalvular gradients, peak transvalvular velocity, and aortic valve opening area were obtained. With CCTA, densities 4–5 mm above the aortic valve; at the junction of the left, right, and noncoronary cusp to the annulus; at the middle level of the left, right, and noncoronary sinuses of Valsalva in the center and the lateral points; at the sinotubular junction; and 4 cm from the sinotubular junction at the midline were measured. First, a comparison of the densities between the normal and stenotic valve was performed, and then possible correlations between echocardiography and CCTA values were investigated in the stenotic group. Results: In all CCTA regions, significantly lower-density values were detected among stenotic valve patients compared to the normal aortic valve population. Additionally, in both groups, higher densities were measured in the peri-jet regions than in the lateral ones. Furthermore, a good correlation was found between the aortic valve opening area and the densities in almost all perivalvular areas. With regard to the densities at the junction of the non-coronary leaflet to the fibrotic annulus and at the most lateral point of the right sinus of Valsalva, a high level of correlation was found between all echocardiography and CCTA parameters. Lastly, with receiver operating characteristic curve measurements, area under the curve values were between 0.857 and 0.930. Conclusion: Certain CCTA density values, especially 4–5mm above the valve opening, can serve as auxiliary information to echocardiography when the severity of aortic valve stenosis is unclear. Full article
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15 pages, 2053 KiB  
Article
Sex-Based Difference in Aortic Dissection Outcomes: A Multicenter Study
by Francesco Nappi, Sandra Petiot, Antonio Salsano, Sanjeet Singh Avtaar Singh, Joelle Berger, Marisa Kostantinou, Severine Bonnet, Ivancarmine Gambardella, Fausto Biancari, Almothana Almazil, Francesco Santini, Rim Chaara and Antonio Fiore
J. Cardiovasc. Dev. Dis. 2023, 10(4), 147; https://doi.org/10.3390/jcdd10040147 - 30 Mar 2023
Cited by 10 | Viewed by 2010
Abstract
Background: Type A Acute Aortic Dissection (TAAAD) repair is a surgical emergency associated with high morbidity and mortality. Registry data have noted several sex-specific differences in presentation with TAAAD which may account for the differences in men and women undergoing surgery for this [...] Read more.
Background: Type A Acute Aortic Dissection (TAAAD) repair is a surgical emergency associated with high morbidity and mortality. Registry data have noted several sex-specific differences in presentation with TAAAD which may account for the differences in men and women undergoing surgery for this condition. Methods: A retrospective review of data from three departments of cardiac surgery (Centre Cardiologique du Nord, Henri-Mondor University Hospital, San Martino University Hospital, Genoa) between January 2005 and 31 December 2021 was conducted. Confounders were adjusted using doubly robust regression models, a combination of regression models with inverse probability treatment weighting by propensity score. Results: 633 patients were included in the study, of which 192 (30.3%) were women. Women were significantly older with reduced haemoglobin levels and pre-operative estimated glomerular filtration rate compared to men. Male patients were more likely to undergo aortic root replacement and partial or total arch repair. Operative mortality (OR 0.745, 95% CI: 0.491–1.130) and early postoperative neurological complication results were comparable between the groups. The adjusted survival curves using IPTW by propensity score confirmed the absence of a significant impact of gender on long-term survival (HR 0.883, 95% CI 0.561–1.198). In a subgroup analysis of women, preoperative levels of arterial lactate (OR 1.468, 95% CI: 1.133–1.901) and mesenteric ischemia after surgery (OR 32.742, 95% CI: 3.361–319.017) were significantly associated with increased operative mortality. Conclusions: The advancing age of female patients alongside raised preoperative level of arterial lactate may account for the increasing preponderance among surgeons to perform more conservative surgery compared to their younger male counterparts although postoperative survival was similar between the groups. Full article
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15 pages, 1229 KiB  
Article
Impact of Mitral Regurgitation Recurrence on Mitral Valve Repair for Secondary Ischemic Mitral Regurgitation
by Antonio Salsano, Antonio Nenna, Nicolas Molinari, Sanjeet Singh Avtaar Singh, Cristiano Spadaccio, Francesco Santini, Massimo Chello, Antonio Fiore and Francesco Nappi
J. Cardiovasc. Dev. Dis. 2023, 10(3), 124; https://doi.org/10.3390/jcdd10030124 - 15 Mar 2023
Cited by 3 | Viewed by 2006
Abstract
Objectives. The current guidelines still do not include specific recommendations on the use of subvalvular repair (SV-r) for treatment of ischemic mitral regurgitation (IMR). Therefore, the objective of our study was to evaluate the clinical impact of mitral regurgitation (MR) recurrence and ventricular [...] Read more.
Objectives. The current guidelines still do not include specific recommendations on the use of subvalvular repair (SV-r) for treatment of ischemic mitral regurgitation (IMR). Therefore, the objective of our study was to evaluate the clinical impact of mitral regurgitation (MR) recurrence and ventricular remodeling on long-term outcomes after SV-r combined with restrictive annuloplasty (RA-r). Methods. We performed a subanalysis of the papillary muscle approximation trial, studying 96 patients with severe IMR and coronary artery disease undergoing restrictive annuloplasty alongside subvalvular repair (SV-r + RA-r group) or restrictive annuloplasty alone (RA-r group). We analyzed treatment failure differences, the influence of residual MR, left ventricular remodeling, and clinical outcomes. The primary endpoint was treatment failure (composite of death; reoperation; or recurrence of moderate, moderate-to-severe, or severe MR) within 5 years of follow-up after the procedure. Results. A total of 45 patients showed failure of the treatment within 5 years, of which 16 patients underwent SV-r + RA-r (35.6%) and 29 underwent RA-r (64.4%, p = 0.006). Patients with significant residual MR presented with a higher rate of all-cause mortality at 5 years compared with trivial MR (HR 9.09, 95% CI 2.08–33.33, p = 0.003). MR progression occurred earlier in the RA-r group, as 20 patients in the RA-r group vs. 6 in SV-r + RA-r group had a significant MR 2 years after surgery (p = 0.002). Conclusions. RA-r remains a surgical mitral repair technique with an increased risk of failure and mortality at 5 years compared with SV-r. The rates of recurrent MR are higher, and recurrence occurs earlier, with RA-r alone compared to SV-r. The addition of the subvalvular repair increases the durability of the repair, thus extending all of the benefits of preventing MR recurrence. Full article
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Review

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20 pages, 4961 KiB  
Review
Diagnostic Challenges in Aortic Stenosis
by André González-García, Pablo Pazos-López, Francisco Eugenio Calvo-Iglesias, Tatiana Mallely Matajira-Chía, Raquel Bilbao-Quesada, Elisa Blanco-González, Carina González-Ríos, María Castiñeira-Busto, Manuel Barreiro-Pérez and Andrés Íñiguez-Romo
J. Cardiovasc. Dev. Dis. 2024, 11(6), 162; https://doi.org/10.3390/jcdd11060162 - 23 May 2024
Viewed by 3329
Abstract
Aortic stenosis (AS) is the most prevalent degenerative valvular disease in western countries. Transthoracic echocardiography (TTE) is considered, nowadays, to be the main imaging technique for the work-up of AS due to high availability, safety, low cost, and excellent capacity to evaluate aortic [...] Read more.
Aortic stenosis (AS) is the most prevalent degenerative valvular disease in western countries. Transthoracic echocardiography (TTE) is considered, nowadays, to be the main imaging technique for the work-up of AS due to high availability, safety, low cost, and excellent capacity to evaluate aortic valve (AV) morphology and function. Despite the diagnosis of AS being considered straightforward for a very long time, based on high gradients and reduced aortic valve area (AVA), many patients with AS represent a real dilemma for cardiologist. On the one hand, the acoustic window may be inadequate and the TTE limited in some cases. On the other hand, a growing body of evidence shows that patients with low gradients (due to systolic dysfunction, concentric hypertrophy or coexistence of another valve disease such as mitral stenosis or regurgitation) may develop severe AS (low-flow low-gradient severe AS) with a similar or even worse prognosis. The use of complementary imaging techniques such as transesophageal echocardiography (TEE), multidetector computed tomography (MDTC), or cardiac magnetic resonance (CMR) plays a key role in such scenarios. The aim of this review is to summarize the diagnostic challenges associated with patients with AS and the advantages of a comprehensive multimodality cardiac imaging (MCI) approach to reach a precise grading of the disease, a crucial factor to warrant an adequate management of patients. Full article
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13 pages, 364 KiB  
Review
Referral of Patients for Surgical Aortic Valve Replacement before and after Introduction of the Transcatheter Aortic Valve Implantation—Changing Patterns of Preoperative Characteristics and Volume and Postoperative Outcome
by Wilhelm Mistiaen
J. Cardiovasc. Dev. Dis. 2023, 10(5), 223; https://doi.org/10.3390/jcdd10050223 - 22 May 2023
Cited by 2 | Viewed by 1403
Abstract
Transcatheter aortic valve implantation (TAVI) was first presented in 2002 as a case report. Randomized controlled trials showed that TAVI could serve as an alternative for surgical aortic valve replacement (SAVR) in high-risk patients. While the indications for TAVI have expanded into low-risk [...] Read more.
Transcatheter aortic valve implantation (TAVI) was first presented in 2002 as a case report. Randomized controlled trials showed that TAVI could serve as an alternative for surgical aortic valve replacement (SAVR) in high-risk patients. While the indications for TAVI have expanded into low-risk groups, favorable results of SAVR in elderly showed an increase in application of surgical treatment in this age category. This review aims to explore the effect of the introduction of TAVI in the referral for SAVR with respect to volume, patient profile, early outcome, and use of mechanical heart valves. Results show that the volume of SAVR has increased in several cardiac centers. In a small minority of series, age and risk score of the referred patients also increased. In most of the series, early mortality rate reduced. These findings, however are not universal. Different management policies could be responsible for this observation. Moreover, some patients in whom aortic valve replacement in whatever form is indicated still do not receive adequate treatment. This can be due to several reasons. Heart teams consisting of interventional cardiologists and cardiac surgeons should become a universal approach in order to minimize the number of untreated patients. Full article
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