Landmark lecture: Perloff lecture: Tribute to Professor Joseph Kayle Perloff and lessons learned from him: aortopathy in adults with CHD

2017 ◽  
Vol 27 (10) ◽  
pp. 1959-1965 ◽  
Author(s):  
Koichiro Niwa
Keyword(s):  
Aortic Valve ◽  
Aortic Root ◽  
Coarctation Of The Aorta ◽  
Left Ventricular ◽  
Lessons Learned ◽  
Aortic Dilatation ◽  
Persistent Truncus Arteriosus ◽  
Artery Flow ◽  
Age Range ◽  
Left Heart Syndrome

AbstractMarfan syndrome, bicuspid aortic valve, and/or coarctation of the aorta are associated with medial abnormalities of the ascending aortic or para-coarctation aorta. Medial abnormalities in the ascending aorta are prevalent in other type of patients with a variety of CHDs such as single ventricle, persistent truncus arteriosus, transposition of the great arteries, hypoplastic left heart syndrome, and tetralogy of Fallot, encompassing a wide age range and may predispose to dilatation, aneurysm, and rapture necessitating aortic valve and root surgery. These CHDs exhibit ongoing dilatation of the aortic root and reduced aortic elasticity and increased aortic stiffness that may relate to intrinsic properties of the aortic root. These aortic dilatation and increased stiffness can induce aortic aneurysm, rapture of the aorta, and aortic regurgitation, but also provoke left ventricular hypertrophy, reduced coronary artery flow, and left ventricular failure. Therefore, a new clinical entity can be used to call this association of aortic pathophysiological abnormality, aortic dilation, and aorto-left ventricular interaction – “aortopathy”.

scholarly journals Bicuspid Aortic Valve Alters Aortic Protein Expression Profile in Neonatal Coarctation Patients

2019 ◽  
Vol 8 (4) ◽  
pp. 517
Author(s):  
Katie L. Skeffington ◽  
Andrew R. Bond ◽  
Safa Abdul-Ghani ◽  
Dominga Iacobazzi ◽  
Sok-Leng Kang ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Coarctation Of The Aorta ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Aortic Tissue ◽  
Hemodynamic Changes ◽  
Left Ventricular Outflow ◽  
Age Range ◽  
First Time ◽  
Formalin Fixed

Coarctation of the aorta is a form of left ventricular outflow tract obstruction in paediatric patients that can be presented with either bicuspid (BAV) or normal tricuspid (TAV) aortic valve. The congenital BAV is associated with hemodynamic changes and can therefore trigger different molecular remodelling in the coarctation area. This study investigated the proteomic and phosphoproteomic changes associated with BAV for the first time in neonatal coarctation patients. Aortic tissue was collected just proximal to the coarctation site from 23 neonates (BAV; n = 10, TAV; n = 13) that were matched for age (age range 4–22 days). Tissue from half of the patients was frozen and used for proteomic and phosphoproteomic analysis whilst the remaining tissue was formalin fixed and used for analysis of elastin content using Elastic Van-Gieson (EVG) staining. A total of 1796 protein and 75 phosphoprotein accession numbers were detected, of which 34 proteins and one phosphoprotein (SSH3) were differentially expressed in BAV patients compared to TAV patients. Ingenuity Pathway Analysis identified the formation of elastin fibres as a significantly enriched function (p = 1.12 × 10−4) due to the upregulation of EMILIN-1 and the downregulation of TNXB. Analysis of paraffin sections stained with EVG demonstrated increased elastin content in BAV patients. The proteomic/phosphoproteomic analysis also suggested changes in inositol signalling pathways and reduced expression of the antioxidant SOD3. This work demonstrates for the first time that coarcted aortic tissue in neonatal BAV patients has an altered proteome/phosphoproteome consistent with observed structural vascular changes when compared to TAV patients.

Unicommissural aortic valve in neonates and its association with other congenital heart disease

1995 ◽  
Vol 5 (2) ◽  
pp. 132-139 ◽  
Author(s):  
Ana C. Durán ◽  
Luciano Daliento ◽  
Carla Frescura ◽  
Giovanni Stellin ◽  
Valentín Sans-Coma ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Coarctation Of The Aorta ◽  
Left Ventricular ◽  
Etiologic Factor ◽  
Fetal Life ◽  
Aortic Valves ◽  
Flow Through ◽  
And Gender ◽  
University Of Padua ◽  
Congenital Aortic Valvar Stenosis

AbstractThe morphology of the aortic valve was studied in 1,022 heart specimens belonging to the collection of the Institute of Pathological Anatomy, University of Padua. Twenty specimens were found to have a unicommissural aortic valve, characterized by the presence of a single leaflet with only one functional commissure; however, the presence of two raphes enabled the recognition of a basically three-sinus arrangement. Age and gender were known in 19 cases: 14 male and five female, mean age nine days. In 19 cases, the unicommissural valve was dysplastic and resulted in a severe congenital aortic valvar stenosis. Only in two hearts was the unicommissural nature of the aortic valve an isolated finding; among the remaining 18, left ventricular fibroelastosis in 11, malformation of the mitral valve in 11, hypoplasia of the left ventricle in eight, ventricular septal defect in four, mitral atresia in three, and subaortic fibrous diaphragm in one. Furthermore, seven (35%) of these 20 unicommissural aortic valves were associated with coarctation of the aorta. Statistical analysis shows that this association is not a random event. Our findings support the hypothesis that the unicommissural aortic valve originates from the early fusion of the three mesenchymal valvar cushions or leaflet primordia. Although the present data do not exclude the possibility that reduction of the blood flow through the aorta during fetal life may play a role in the formation of the unicommissural aortic valve, they rather point in the direction that another etiologic factor, such as an anomalous migration of neural crest cells, may be responsible for the fusion of the valvar cushions.

scholarly journals Effect of Eccentric Calcification of an Aortic Valve on the Implant Depth of a Venus-A Prosthesis During Transcatheter Aortic Valve Replacement: A Retrospective Study

2021 ◽  
Vol 12 ◽  
Author(s):  
Lanlan Li ◽  
Yang Liu ◽  
Ping Jin ◽  
Jiayou Tang ◽  
Linhe Lu ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Aortic Valve Replacement ◽  
Valve Replacement ◽  
Aortic Root ◽  
Transcatheter Aortic Valve Replacement ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Release Process ◽  
Standard Position ◽  
Transcatheter Aortic Valve

ObjectOur goal was to assess the implant depth of a Venus-A prosthesis during transcatheter aortic valve replacement (TAVR) when the areas of eccentric calcification were distributed in different sections of the aortic valve.MethodsA total of 53 patients with eccentric calcification of the aortic valve who underwent TAVR with a Venus-A prosthesis from January 2018 to November 2019 were retrospectively analyzed. The patients were divided into three groups (A, B, and C) according to the location of the eccentric calcification, which was determined by preprocedural computerized tomography angiography (CTA) images. The prosthesis release process and position were evaluated by contrast aortography during TAVR, and the differences in valve implant depths were compared among the three groups. The effects of different aortic root structures and procedural strategies on prosthesis implant depth were analyzed.ResultsEleven patients had eccentric calcification in region A; 19 patients, in region B; and 23 patients, in region C. The patients with eccentric calcification in region B had a higher risk of prosthesis migration (10.5% upward and 21.1% downward), and the position of the prosthesis after TAVR in group B was the deepest among the three groups. When eccentric calcification was located in region A or C, the prosthesis was released at the standard position with more stability, and the location of the prosthesis was less deep after TAVR (region A: 4.12 ± 3.4 mm; region B: 10.2 ± 5.3 mm; region C: 8.4 ± 4.0 mm; region A vs. region B, P = 0.0004; region C vs. region B; and P = 0.0360). In addition, the left ventricular outflow tract (LVOT) (P = 0.0213) and aortic root angulation (P = 0.0263) also had a significant effect on implant depth in the aortic root structure of the patients. The prosthesis size was 28.3 ± 2.4 in the deep implant group and 26.4 ± 2.0 in the appropriate implant group (P = 0.0068).ConclusionThe implant depth of the Venus-A prosthesis is closely related to the distribution of eccentric calcification in the aortic valve during TAVR. Surgeons should adjust the surgical strategy according to aortic root morphology to prevent prosthesis migration.

scholarly journals An infrequent association: Left ventricular non compaction with bicuspid aortic valve and coarctation of the aorta.

2020 ◽  
Vol 4 (1) ◽  
Author(s):  
Aureo Campos-Tello ◽  
Giancarlo A Valle ◽  
Felix A Revilla ◽  
Sara Rami­rez-Flores ◽  
Ãngel CuevaParra ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Coarctation Of The Aorta ◽  
Left Ventricular

In the Loop—Left Ventricular Pressures

2011 ◽  
pp. 42-47
Author(s):  
James R. Munis
Keyword(s):  
Heart Rate ◽  
Cardiac Output ◽  
Aortic Valve ◽  
Left Ventricle ◽  
Stroke Volume ◽  
Cardiovascular System ◽  
Aortic Root ◽  
Left Ventricular ◽  
Root Pressure ◽  
The Third

We've already looked at 2 types of pressure that affect physiology (atmospheric and hydrostatic pressure). Now let's consider the third: vascular pressures that result from mechanical events in the cardiovascular system. As you already know, cardiac output can be defined as the product of heart rate times stroke volume. Heart rate is self-explanatory. Stroke volume is determined by 3 factors—preload, afterload, and inotropy—and these determinants are in turn dependent on how the left ventricle handles pressure. In a pressure-volume loop, ‘afterload’ is represented by the pressure at the end of isovolumic contraction—just when the aortic valve opens (because the ventricular pressure is now higher than aortic root pressure). These loops not only are straightforward but are easier to construct just by thinking them through, rather than by memorization.

P6496Endovascular reconstruction from aortic valve to aortic arch by one-piece valved-fenestrated-bifurcated endografting in animal experiments

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
Z P Jing ◽  
J X Feng ◽  
X H Bao ◽  
T Li ◽  
Y Zhao ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Aortic Arch ◽  
Aortic Root ◽  
Animal Experiments ◽  
Proximal Part ◽  
Minimal Invasive ◽  
Left Ventricular ◽  
Technical Success Rate

Abstract Aims The possibility of endovascular reconstruction of aortic valve, sinus of Valsalva, and ascending aorta by a minimal-invasive single endograft has not been proven in vivo. Combining our own long-term experiences from transcatheter aortic valve replacement (TAVR) and Thoracic Endovascular Repair (TEVAR) for ascending and arch dissection, we designed the special endo-graft: a novel one-piece valved-fenestrated-bifurcated endografting, and tried to endovascularly reconstruct the area from Left ventricular outflow tract to aortic arch in animal experiments. Methods and results For 20 healthy adult female pigs weighed between 62.3±2.2 kilograms, we did aortic compute tomography angiography (CTA) examinations and measured morphologic parameters of aortic root. Then we accordingly customized the valved-fenestrated-bifurcated endograft. The endograft was delivered through transapical access and endovascularly reconstructed the segment from aortic valve to proximal part of aortic arch. The overall technical success rate was 95% because of one case of delivery system failure. Instant transesophageal echography (TEE) and aortic CTA confirmed ideal position of the endograft, satisfactory function of aortic valve, and the patency of coronary arteries in all subjects. During follow-up, 12 subjects were sacrificed according to the plan and seven were followed up for 8.1±3.6 months. There was one unplanned death of cardiac infection (unplanned mortality: 5.3%). Follow-up re-examinations (aortic CTA, cardiac ultrasound, and electrocardiogram) found no adverse events. Among 12 sacrificed subjects, there was no evidence of fenestrations alignment lost and no myocardial ischemia according to the pathological analysis. Conclusion The novel one-piece valved-fenestrated-bifurcated endografting might be feasible for minimal-invasive reconstruction of aortic root in animal models, thus provided a prospect to simultaneously treat pathologies involving aortic valve and aortic root in endovascular way.

Bicuspid Aortic Valves Are Associated With Increased Turbulence Compared to Pure Aortic Stenosis: An In Vitro Study

2012 ◽  
Author(s):  
Neelakantan Saikrishnan ◽  
Nicole C. Milligan ◽  
Ajit P. Yoganathan
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Genetic Defect ◽  
In Vitro Study ◽  
Coarctation Of The Aorta ◽  
Vitro Study ◽  
Cardiac Anomaly ◽  
Aortic Dilatation ◽  
Congenital Cardiac Anomaly

In about 1–2% of all live births, the human aortic valve only consists of two anomalous leaflets and is known as the bicuspid aortic valve (BAV). BAVs are the most common congenital cardiac anomaly, and are associated with significant valvular dysfunction, including calcific aortic stenosis (AS) and aortic regurgitation (AR), as well as aortic wall abnormalities including coarctation of the aorta, ascending aortic dilatation and aneurysms [1]. Many studies have proposed a common underlying genetic defect in progression of complications with BAVs [2]. However, other recent studies have also suggested that the altered hemodynamic environment associated with BAVs could also be responsible for accelerated disease progression in these patients [3, 4]. A recent in vitro study showed elevated levels of turbulence associated with BAVs, and indicated that fluid flow patterns in the aortic sinuses are also affected due to the altered valve morphology [5]. The present work seeks to compare the levels of turbulence in BAVs to pure trileaflet aortic stenosis models.

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