Prenatal Diagnosis of Congenital Absence of Aortic Valve: A Report of Two Cases with Different Outcomes and a Literature Review

2014 ◽  
Vol 38 (4) ◽  
pp. 307-314 ◽  
Author(s):  
Takashi Murakami ◽  
Hitoshi Horigome ◽  
Junko Shiono ◽  
Takumi Ishiodori ◽  
Yukiko Ban ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Left Ventricle ◽  
Aortic Regurgitation ◽  
Fetal Echocardiography ◽  
Left Ventricular ◽  
Congenital Absence ◽  
Mitral Insufficiency ◽  
Intrauterine Death ◽  
Foramen Ovale ◽  
Mitral Atresia

Congenital absence of aortic valve (AAV) is a rare cardiac anomaly associated with high mortality. We present 2 prenatally diagnosed cases of AAV. In both cases, fetal echocardiography showed no aortic valve tissue and free aortic regurgitation. At 24 weeks' gestation, case 1 showed a hypoplastic hypocontractile left ventricle and mitral atresia, but did not develop hydrops and was born at term. Bilateral pulmonary arterial banding was performed with continuous infusion of prostaglandin E1 at 5 days of age followed by Norwood and bidirectional Glenn procedures at 3 months of age. The hypoplastic non-compliant left ventricle and mitral atresia might have limited aortic regurgitation. Case 2 showed hydrops at 23 weeks' gestation. An enlarged hypocontractile left ventricle and massive mitral insufficiency were noted. The regurgitant flow was directed to the right atrium through a foramen ovale. This peculiar hemodynamic established a malignant circuit which was arbitrarily called ‘inverse circulatory shunt'. Intrauterine death occurred at 28 weeks' gestation. An autopsy showed absent aortic valve leaflets but three tiny membranous remnants. Hemodynamic evaluation by fetal echocardiography should include the documentation of the presence of and assessment of the grade of aortic regurgitation, mitral regurgitation and blood flow through a foramen ovale as well as left ventricular function.

scholarly journals Left ventricle reverse remodeling in chronic aortic regurgitation patients with dilated ventricle after aortic valve replacement

2022 ◽  
Vol 17 (1) ◽  
Author(s):  
Ming-Kui Zhang ◽  
Li-Na Li ◽  
Hui Xue ◽  
Xiu-Jie Tang ◽  
He Sun ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Left Ventricle ◽  
Aortic Valve Replacement ◽  
Aortic Regurgitation ◽  
Left Ventricular ◽  
Reverse Remodeling ◽  
Left Ventricle Remodeling ◽  
Lv Remodeling ◽  
Chronic Aortic Regurgitation

Abstract Background Aortic valve replacement (AVR) for chronic aortic regurgitation (AR) with a severe dilated left ventricle and dysfunction leads to left ventricle remodeling. But there are rarely reports on the left ventricle reverse remodeling (LVRR) after AVR. This study aimed to investigate the LVRR and outcomes in chronic AR patients with severe dilated left ventricle and dysfunction after AVR. Methods We retrospectively analyzed the clinical datum of chronic aortic regurgitation patients who underwent isolated AVR. The LVRR was defined as an increase in left ventricular ejection fraction (LVEF) at least 10 points or a follow-up LVEF ≥ 50%, and a decrease in the indexed left ventricular end-diastolic diameter of at least 10%, or an indexed left ventricular end-diastolic diameter ≤ 33 mm/m2. The changes in echocardiographic parameters after AVR, survival analysis, the predictors of major adverse cardiac events (MACE), the association between LVRR and MACE were analyzed. Results Sixty-nine patients with severe dilated left ventricle and dysfunction underwent isolated AVR. LV remodeling in 54 patients and no LV remodeling in 15 patients at 6–12 months follow-up. The preoperative left ventricular dimensions and volumes were larger, and the EF was lower in the LV no remodeling group than those in the LV remodeling group (all p < 0.05). The adverse LVRR was the predictor for MACE at follow-up. The mean follow-up period was 47.29 months (range 6 to 173 months). The rate of freedom from MACE was 94.44% at 5 years and 92.59% at 10 years in the remodeling group, 60% at 5 years, and 46.67% at 10 years in the no remodeling group. Conclusions The left ventricle remodeling after AVR was the important predictor for MACE. LV no remodeling may not be associated with benefits from AVR for chronic aortic regurgitation patients with severe dilated LV and dysfunction.

P5987Novel model of intensity graded murine wire-induced aortic valve stenosis mimics distinct stages of human aortic valve pathology

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
S T Niepmann ◽  
E Steffen ◽  
A Zietzer ◽  
M Adam ◽  
J Nordsiek ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Left Ventricle ◽  
Ejection Fraction ◽  
Flow Velocity ◽  
Disease Progression ◽  
Aortic Regurgitation ◽  
Aortic Valve Stenosis ◽  
Left Ventricular ◽  
Valve Disease ◽  
Severe Injury

Abstract Background Aortic valve stenosis (AS) is the most common valve disease requiring therapeutic intervention. Even though the incidence of AS has been continuously rising and AS is associated with significant morbidity and mortality, to date, no medical treatments have been identified that can modify disease progression. In fact, only invasive interventional or surgical replacement of severely diseased valves is recommended. This unmet medical need is likely attributed to the lack of a clear understanding of the molecular mechanism driving disease development. To investigate the pathophysiology leading to AS, reliable and reproducible animal models that mimic human pathophysiology are needed. Hypothesis Induction of a graded wire-induced aortic valve stenosis model in mice is feasible to reflect and study pathophysiological mechanisms underlying the progression of aortic valve stenosis. Methods We have tested and expanded the protocols of a novel wire-injury induced aortic valve stenosis mouse model. A spring coronary guide wire or a bare metal wire was used to apply shear stress to the aortic valve cusps with increasing intensity with ultrasound-guided monitoring in male 10 to 12-week-old C57Bl/6j mice. These protocols allowed the induction of distinct models with soft, moderate and intense wire injury. Functional analysis including maximum flow velocity (Vmax), ejection fraction, fractional shortening, left ventricular volumes, diameters and wall thickness were assessed by echocardiography before, one and four weeks after induction of aortic valve stenosis. Immunohistological analysis were performed after eight weeks (hematoxylin and eosin, von-Kossa staining, anti-CD68-staining). Results Upon moderate or severe injury, AS developed with a significant increase in aortic valve peak blood flow velocity. While moderate injury promoted solitary AS, severe-injury induced mixed aortic valve disease with concomitant mild to moderate aortic regurgitation. Only 5% of the mice who received a moderate injury displayed a mild aortic regurgitation. In the group of mice with intense injury 50% of the mice had a mild and 18,75% had a moderate aortic insufficiency. The changes in aortic valve function were reflected by dilation and hypertrophy of the left ventricle, as well as a decreased left ventricular ejection fraction after intense injury, while moderate injury did not show significant dilation of the left ventricle. Histological analysis revealed the three classic hallmarks of human disease with aortic valve thickening, increased macrophage infiltration and calcification eight weeks after injury. Conclusion Hereby, we demonstrate that the induction of a graded wire induced aortic valve stenosis model in mice mimicking relevant pathophysiological mechanisms is feasible to study disease progression. We extended existing protocols to induce moderate stenosis allowing to solely study aortic valve stenosis without relevant aortic valve regurgitation. Acknowledgement/Funding S.N. was funded by Else-Kröner-Fresenius-Foundation of the Medical Faculty of the University of Bonn

Presence of adipose tissue in aortic valves from aortic regurgitation patients: pathophysiological role and clinical implications

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
A Sadaba Cipriain ◽  
A.M Navarro Echeverria ◽  
C.R Tiraplegui Garjon ◽  
A Garcia De La Pena Urtasun ◽  
V Arrieta Paniagua ◽  
...  
Keyword(s):  
Adipose Tissue ◽  
Aortic Valve ◽  
Aortic Regurgitation ◽  
Systolic Function ◽  
Statistical Significance ◽  
Left Ventricular Systolic Function ◽  
Left Ventricular ◽  
Funding Source ◽  
Matrix Remodeling ◽  
Aortic Valves

Abstract Introduction Adipose tissue is a common constituent of the heart and it is located, without great clinical relevance, frequently in the pericardium. The presence of adipose tissue in the aortic valve is rare, with unknown significance on valve structural properties and function. Aortic regurgitation (AR) is the third most prevalent valve disease, although it is uncommon to find it in isolation. Myxoid degeneration may be the cause or result of AR, although the pathophysiology remains poorly understood. Purpose To describe and characterize the presence of adipose tissue in the aortic valves from a cohort of AR patients. Methods 116 patients undergoing aortic valve replacement due to severe AR were enrolled. We classified them in two groups according to the histological results showing presence or absence of adipose tissue in the aortic valves. In the valve tissue molecular analysis were performed by RT-PCR, Western Blot and ELISA to analyze markers of adipocytes (leptin, adiponectin, resistin), inflammation (Rantes, interleukin-6, interleukin-1β), extracellular matrix remodeling (metalloproteinases-1, -2 and -9), proteoglycans (aggrecan, hyaluronan, lumican, syndecan-1, decorin) and fibrosis (collagens, fibronectin). Results Adipose tissue was found in 63% of the aortic valves analyzed. Baseline characteristics (age, hypertension, dyslipidemia, diabetes, smoking, left ventricular telediastolic diameter, left ventricular systolic function, ascending aorta) were similar in patients presenting valve adipose tissue as compared with patients without valve adipose tissue. Valves containing adipocytes exhibited a higher leptin content (p&lt;0.001), fibronectin (p&lt;0.01), decorin (p&lt;0,0001), hyaluronan (p=0.03), aggrecan (p=0.04) and metalloproteinase 1 (p=0.03). Interestingly, the presence of adipocytes in the valve was positively correlated with valve thickness measured by echocardiogram (Pearson chi2 statistical significance = 26.3345 p&lt;0.001). Conclusion To our knowledge, this is the first study that describes the presence of adipose cells in aortic valves from a cohort of AR patients. Aortic valves containing adipocytes were thicker and exhibited significant higher levels of proteoglycans, suggesting that adipocytes could contribute to the myxomatous degeneration process. Our results propose that the valve adipose tissue could play a role in the pathophysiology of AR. Funding Acknowledgement Type of funding source: Public hospital(s). Main funding source(s): Gobierno de Navarra

Echocardiographic assessment of the aortic valve and left ventricular outflow tract

2005 ◽  
Vol 15 (S1) ◽  
pp. 27-36 ◽  
Author(s):  
Alfred Asante-Korang ◽  
Robert H. Anderson
Keyword(s):  
Aortic Valve ◽  
Left Ventricle ◽  
Left Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Outflow Tract ◽  
Left Heart ◽  
Transesophageal Echocardiogram ◽  
Left Ventricular Outflow ◽  
Echocardiographic Assessment

The previous reviews in this section of our Supplement1,2 have summarized the anatomic components of the ventriculo-arterial junctions, and then assessed the echocardiographic approach to the ventriculo-arterial junction or junctions as seen in the morphologically right ventricle. In this complementary review, we discuss the echocardiographic assessment of the comparable components found in the morphologically left ventricle, specifically the outflow tract and the arterial root. We will address the echocardiographic anatomy of the aortic valvar complex, and we will review the causes of congenital arterial valvar stenosis, using the aortic valve as our example. We will also review the various lesions that, in the outflow of the morphologically left ventricle, can produce subvalvar and supravalvar stenosis. We will then consider the salient features of the left ventricular outflow tract in patients with discordant ventriculo-arterial connections, and double outlet ventricles. To conclude the review, we will briefly address some rarer anomalies that involve the left ventricular outflow tract, showing how the transesophageal echocardiogram is used to assist the surgeon preparing for repair. The essence of the approach will be to consider the malformations as seen at valvar, subvalvar, or supravalvar levels,1 but we should not lose sight of the fact that aortic coarctation or interruption, hypoplasia of the left heart, and malformations of the mitral valve are all part of the spectrum of lesions associated with obstruction to the left ventricular outflow tract. These additional malformations, however, are beyond the scope of this review.

Intraventricular flow visualization in different heart failure stages with blood pump support in a mock circulatory loop

2021 ◽  
pp. 039139882110214
Author(s):  
Guang-Mao Liu ◽  
Fu-Qing Jiang ◽  
Jiang-Ping Song ◽  
Sheng-Shou Hu
Keyword(s):  
Heart Failure ◽  
Standard Deviation ◽  
Aortic Valve ◽  
Left Ventricle ◽  
Left Ventricular ◽  
Blood Pump ◽  
Blood Damage ◽  
Intraventricular Flow ◽  
Pump Inlet ◽  
Strong Vorticity

The intraventricular blood flow changed by blood pump flow dynamics may correlate with thrombosis and ventricular suction. The flow velocity, distribution of streamlines, vorticity, and standard deviation of velocity inside a left ventricle failing to different extents throughout the cardiac cycle when supported by an axial blood pump were measured by particle image velocimetry (PIV) in this study. The results show slower and static flow velocities existed in the central region of the left ventricle near the mitral valve and aortic valve and that were not sensitive to left ventricular (LV) failure degree or LV pressure. Strong vorticity located near the inner LV wall around the LV apex and the blood pump inlet was not sensitive to LV failure degree or LV pressure. Higher standard deviation of the blood velocity at the blood pump inlet decreased with increasing LV failure degree, whereas the standard deviation of the velocity near the atrium increased with increasing intraventricular pressure. The experimental results demonstrated that the risk of thrombosis inside the failing left ventricle is not related to heart failure degree. The “washout” performance of the strong vorticity near the inner LV wall could reduce the thrombotic potential inside the left ventricle and was not related to heart failure degree. The vorticity near the aortic valve was sensitive to LV failure degree but not to LV pressure. We concluded that the risk of blood damage caused by adverse flow inside the left ventricle decreased with increasing LV pressure.

Prenatal diagnosis of congenital absence of aortic valve associated with restrictive foramen ovale : Hemodynamic features and clinical outcome

2018 ◽  
Vol 47 (2) ◽  
pp. 104-106
Author(s):  
Takashi Murakami ◽  
Lisheng Lin ◽  
Takumi Ishiodori ◽  
Syusuke Takeuchi ◽  
Junko Shiono ◽  
...  
Keyword(s):  
Prenatal Diagnosis ◽  
Aortic Valve ◽  
Clinical Outcome ◽  
Congenital Absence ◽  
Foramen Ovale ◽  
Restrictive Foramen Ovale

Aortic regurgitation

2015 ◽  
pp. 141-147
Author(s):  
Raphael Rosenhek
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Aortic Valve ◽  
Aortic Regurgitation ◽  
Cardiac Computed Tomography ◽  
Transoesophageal Echocardiography ◽  
Left Ventricular ◽  
Disease Aetiology ◽  
Echocardiographic Parameters ◽  
And Function

The workup of patients with aortic regurgitation is routinely based on echocardiography and includes a detailed morphologic assessment of the aortic valve with the determination of disease aetiology. The quantification of aortic regurgitation is based on an integration of qualitative and quantitative parameters. Haemodynamic consequences of aortic valve disease on left ventricular size, hypertrophy, and function, as well as potentially coexisting valve lesions, are assessed. Predictors of outcome and indications for surgery are substantially defined by echocardiographic parameters. Cardiac magnetic resonance has become an important complementary technique, both for the quantification of regurgitant severity and for the assessment of ventricular function. While the proximal parts of the ascending aorta are routinely visualized by transthoracic echocardiography, transoesophageal echocardiography (TOE) and in particular cardiac magnetic resonance (CMR) and cardiac computed tomography (CT) allow a more comprehensive assessment of the thoracic aorta.

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