Congenital Aortic Stenosis:

PEDIATRICS ◽  
1959 ◽  
Vol 23 (2) ◽  
pp. 347-347
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Surgical Techniques ◽  
Clinical Results ◽  
Anterior Chest Wall ◽  
Left Ventricular ◽  
Heart Catheterization ◽  
Congenital Aortic Stenosis ◽  
Direct Puncture ◽  
Aortic Obstruction

A group of 30 patients with congenital aortic stenosis, ranging in age from 4 to 39 years, has been extensively studied at the National Heart Institute. The majority of these patients revealed cardiomegaly and electrocardiographic evidence of left ventricular hypertrophy, and experienced symptoms which included dyspnea, angina and syncope. The severity and site of aortic obstruction (valvular or subvalvular) was evaluated by left heart catheterization. Measurements of left ventricular pressures were made either by the transbronchial route or by direct puncture of this chamber through the anterior chest wall. The aortic valve gradients, i.e., the differences between simultaneous peak systolic pressures in the left ventricle and aorta or peripheral artery, ranged from 15 to 172 mm Hg. It is currently the policy to recommend operation to patients with aortic stenosis who, regardless of symptoms, have aortic valve gradients in excess of 50 mm Hg. Present surgical techniques employing direct vision by means of hypothermia or extracorporeal circulation are discussed. Of the 18 patients operated upon, there are 15 survivors most of whom have had gratifying clinical results substantiated by post-operative hemodynamic studies.

scholarly journals Balloon Valvuloplasty for Congenital Aortic Stenosis: Experience at a Tertiary Center in a Developing Country

2021 ◽  
Vol 2021 ◽  
pp. 1-7
Author(s):  
Fatme A. Charafeddine ◽  
Haytham Bou Houssein ◽  
Nadine B. Kibbi ◽  
Issam M. El-Rassi ◽  
Anas M. Tabbakh ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Developing Country ◽  
Aortic Regurgitation ◽  
Developed Countries ◽  
Left Ventricular ◽  
Complication Rates ◽  
Congenital Aortic Stenosis ◽  
Tertiary Center

Background. Aortic valve stenosis accounts for 3–6% of congenital heart disease. Balloon aortic valvuloplasty (BAV) is the preferred therapeutic intervention in many centers. However, most of the reported data are from developed countries. Materials and Methods. We performed a retrospective single-center study involving consecutive eligible neonates and infants with congenital aortic stenosis admitted for percutaneous BAV between January 2005 and January 2016 to our tertiary center. We evaluated the short- and mid-term outcomes associated with the use of BAV as a treatment for congenital aortic stenosis (CAS) at a tertiary center in a developing country. Similarly, we compared these outcomes to those reported in developed countries. Results. During the study period, a total of thirty patients, newborns (n = 15) and infants/children (n = 15), underwent BAV. Left ventricular systolic dysfunction was present in 56% of the patients. Isolated AS was present in 19 patients (63%). Associated anomalies were present in 11 patients (37%): seven (21%) had coarctation of the aorta, two (6%) had restrictive ventricular septal defects, one had mild Ebstein anomaly, one had Shone’s syndrome, and one had cleft mitral valve. BAV was not associated with perioperative or immediate postoperative mortality. Immediately following the valvuloplasty, a more than mild aortic regurgitation was noted only in two patients (7%). A none-to-mild aortic regurgitation was noted in the remaining 93%. One patient died three months after the procedure. At a mean follow-up of 7 years, twenty patients (69%) had more than mild aortic regurgitation, and four patients (13%) required surgical intervention. Kaplan–Meier freedom from aortic valve reintervention was 97% at 1 year and 87% at 10 years of follow-up. Conclusion. Based on outcomes encountered at a tertiary center in a developing country, BAV is an effective and safe modality associated with low complication rates comparable to those reported in developed countries.

Abstract 15785: The Natural History of Doppler-Derived Left Ventricular Outflow Tract Gradients in Patients With Congenital Valvar Aortic Stenosis Before and After Balloon Valvuloplasty

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Joseph D Kuebler ◽  
Jill Shivapour ◽  
Kimberlee Gauvreau ◽  
Steven D Colan ◽  
Doff B McElhinney ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Natural History ◽  
Aortic Stenosis ◽  
Surgical Intervention ◽  
Left Ventricular ◽  
Mixed Effect ◽  
Congenital Aortic Stenosis ◽  
Rate Of Progression ◽  
Before And After ◽  
History Of

Introduction: Congenital aortic stenosis (AS) has been reported to manifest a slow rate of progression in mild disease with a greater likelihood of progression in patients with moderate-severe disease. The natural history of the Doppler estimated peak gradient (DEPG) in patients after balloon aortic valvuloplasty (BAV) has not previously been studied on a large scale. Methods: A retrospective review was performed of 360 patients from 1984-2012 with AS providing a total of 2051 echocardiograms before and after BAV. Patients were excluded if they had an intervention within the first 30 days of life. The relationships between the AS DEPG and several predictors (age at time of initial echocardiogram, valve morphology, and history of intervention) were explored using linear mixed effect models. The DEPG slope was then calculated in patients who had at least 2 echocardiograms before and after balloon dilation using linear regression modeling. Results: The rate of increase in the DEPG for all patients with AS was 5.6 mmHg per 10 years of age (p<0.001). The DEPG increased over time regardless of age at presentation with the greatest mean increase in patients presenting from 10-14.9 years (n=59; 11.9 mmHg per 10 years; p<0.001). Patients who went on to have a BAV or surgical intervention on the aortic valve had a significantly higher rate of AS progression than the overall patient cohort (n=59; 18.0 mmHg/10 years and n=36; 13.1 mmHg/10 years). Patients with a unicommissural (n=39) aortic valve had a significantly higher rate of progression compared to those with a bicommissural (n=270) aortic valve (8.1 mmHg/10 years and 4.5 mm Hg/10 years; p<0.001). The median rate of progression in the post-BAV group was significantly lower than the median pre-BAV rate of progression (n=34; pre-BAV 3.97 (1.69-8.7) mmHg/year; post-BAV 0.40 (-1.80-3.88) mmHg/year; p<0.01). Conclusions: The DEPG of native valve congenital aortic stenosis shows a slow, linear rate of progression prior to intervention. The rate of progression is significantly higher in patients with a unicommissural aortic valve as well as those patients that go on to have a BAV and/or surgical intervention. The rate of the DEPG progression is significantly lower after BAV.

THE TELEMETERED EXERCISE ELECTROCARDIOGRAM IN CONGENITAL AORTIC STENOSIS

PEDIATRICS ◽  
1971 ◽  
Vol 47 (1) ◽  
pp. 31-39
Author(s):  
Katherine H. Halloran
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Aortic Valve Stenosis ◽  
Systolic Pressure ◽  
Left Ventricular ◽  
Normal Children ◽  
Congenital Aortic Stenosis ◽  
Electrocardiographic Leads ◽  
Response To Exercise ◽  
Exercise Electrocardiogram

Since children with aortic valve stenosis, who are at risk of syncope or sudden death, cannot be identified by the resting electrocardiogram or vectorcardiogram, the exercise electrocardiogram was evaluated and compared with the hemodynamic data obtained during cardiac catheterization. Telemetered exercise electrocardiograms were obtained in 31 children, ages 8 to 18 years, with aortic valve stenosis and in 25 normal children of comparable age. Electrocardiographic leads V1, V5, and V6 were obtained prior to, during, and following exercise on a variable resistance bicycle ergometer. Subjects pedalled until a heart rate of 170 per minute or greater was attained and maintained for at least 2 minutes. An increase in T-wave amplitude was observed in both control children and in those with aortic stenosis. No S-T segment abnormalities were noted in the normal children. Of the 16 patients with peak systolic left ventricular to aortic pressure gradients of less than 50 mm Hg, only one showed a segmental S-T depression. Of the 15 children with aortic valve gradients of 50 to 100 mm Hg, however, all except one showed an S-T segment depression in lead V5 of 2 mm or greater. No correlation between the resting electrocardiogram or the vectorcardiogram and the aortic valve gradient or left ventricular peak systolic pressure could be made. In addition, the abnormal S-T segment response to exercise could not be predicted from or correlated with the resting electrocardiogram. Since an ischemic S-T segment response to exercise was found uniformly in those with the higher gradients, this test appears to have a high degree of specificity in the clinical evaluation of these patients.

Surgical Experience with Doty's Repair for Supravalvular Aortic Stenosis

1996 ◽  
Vol 4 (1) ◽  
pp. 29-32
Author(s):  
Balram Airan ◽  
Sunil K Kaushal ◽  
Anil Bhan ◽  
Panangipalli Venugopal
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Coronary Sinus ◽  
Innominate Artery ◽  
Left Ventricular ◽  
Supravalvular Aortic Stenosis ◽  
Coarctation Of Aorta ◽  
Valve Function ◽  
Aortic Obstruction ◽  
The Right

The surgical treatment of severe supravalvular aortic stenosis by conventional, asymmetrical, one point patch aortoplasty across the narrowed area has been associated with a substantial incidence of residual stenosis and reoperations. Almost symmetrical relief of such stenosis was achieved with Doty's extended aortoplasty in 5 patients in whom left ventricular aortic gradient was reduced from 60–170 nun Hg to 0–25 mm Hg. This technique of inserting an inverted V-shaped gusset across the stenosed segment into the right coronary sinus and non-coronary sinus, restores the aortic root geometry, provides more predictable relief of aortic obstruction and also improves aortic valve function. Associated procedures involved relief of coarctation of aorta and innominate artery stenosis in 1 case and repair of aortic valve and mitral valve in another case.

Sudden Death in Young Patients With Congenital Aortic Stenosis

PEDIATRICS ◽  
1974 ◽  
Vol 53 (4) ◽  
pp. 481-489
Author(s):  
Eugenie F. Doyle ◽  
Palaniappan Arumugham ◽  
Emilia Lara ◽  
Monika R. Rutkowski ◽  
Brian Kiely
Keyword(s):  
Sudden Death ◽  
Aortic Stenosis ◽  
Young Patients ◽  
Left Ventricular ◽  
Exertional Dyspnea ◽  
Congenital Aortic Stenosis ◽  
Left Ventricular Strain ◽  
Strain Pattern ◽  
Aortic Obstruction ◽  
Almost All

Four patients with congenital valvular and subvalvular aortic stenosis who died suddenly are presented in detail. Each had evidence of severe obstruction and left ventricular "strain" pattern on the electrocardiogram. Symptoms were present in three cases. Survey of the literature was done with particular reference to the usefulness of standard scalar electrocardiography and patient symptomatology in estimating the severity and the progression of the aortic obstruction. Of those who died suddenly with isolated aortic stenosis, 70% had left ventricular strain pattern, 21% had left ventricular hypertrophy alone, and 9% had an apparently normal electrocardiogram. Symptoms of exertional dyspnea, syncope, chest pain, and fatigue were present in almost all cases. Sudden death in the absence of severe ECG abnormalities and/or symptoms is very uncommon.

scholarly journals Prospective validation of a non-invasive method to estimate myocardial work in aortic stenosis

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
A Hubert ◽  
KP Owashi ◽  
V Le Rolle ◽  
A Hernandez ◽  
E Galli ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Left Ventricular Pressure ◽  
Left Ventricular ◽  
Ventricular Pressure ◽  
Heart Catheterization ◽  
Aortic Valve Area ◽  
Non Invasive ◽  
Funding Sources ◽  
Invasive Method

Abstract Funding Acknowledgements Type of funding sources: Public hospital(s). Main funding source(s): ANR - Maestro project Background Stratification of aortic stenosis patients remains challenging and robust indices are required. Myocardial work assessment is a new afterload independent alternative to evaluate left ventricular function. Although, this method was developed in patients with normal aortic valve. We previously developed an integrated cardiovascular system simulated by a computational model to estimate non-invasively myocardial work in aortic stenosis patients* (figure 1A). In the present study, we tested our model in a prospective population of AS patients. Method and results 9 patients with severe AS (aortic valve area &lt; 1cm2) were included. A complete trans-thoracic echocardiography with a non-invasive blood pressure by brachial artery cuff were realized immediately before a left heart catheterization to have an invasive left ventricular pressure. Myocardial work is then calculated with non-invasive and invasive LV pressure combined to LV strain curves. For constructive and wasted work, root mean squared between invasive and estimated measures were respectively r2 = 0.92 and r2 = 0.94 (figure 1B) Conclusion The proposed model is efficient to estimate non-invasively myocardial work indices in AS-patients. These afterload independent indices could permit in future to better stratify this population.  *Owashi KP, Hubert A and al. Model-based estimation of left ventricular pressure and myocardial work in aortic stenosis. PlosOne 2020. Mar 3;15(3):e0229609 Abstract Figure 1

scholarly journals Long-term outcome of congenital aortic valve stenosis: predictors of reintervention

2014 ◽  
Vol 25 (5) ◽  
pp. 893-902 ◽  
Author(s):  
Léa Hochstrasser ◽  
Patrick Ruchat ◽  
Nicole Sekarski ◽  
Michel Hurni ◽  
Ludwig K. von Segesser
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Posterior Wall ◽  
Left Ventricular ◽  
Post Intervention ◽  
Term Outcome ◽  
Congenital Aortic Stenosis ◽  
Long Term Outcome ◽  
Posterior Wall Thickness

AbstractObjectives:To evaluate long-term outcome of initial aortic valve intervention in a paediatric population with congenital aortic stenosis, and to determine risk factors associated with reintervention.Patients and methods:From 1985 to 2009, 77 patients with congenital aortic stenosis and a mean age of 5.8±5.6 years at diagnosis were followed up in our institution for 14.8±9.1 years.Results:First intervention was successful with 86% of patients having a residual peak aortic gradient <50 mmHg, and the proportion of patients with grade >1 regurgitation increased by 7%. Long-term survival after the first procedure was excellent, with 91% survival at 25 years. At a mean interval of 7.6±5.3 years, 30 patients required a reintervention (39%), mainly because of a recurrent aortic stenosis. Freedom from reintervention was 97, 89, 75, 53, and 42% at 1, 10, 15, 20, and 25 years, respectively. Predictors of reintervention were residual peak aortic gradient (p=0.0001), aortic regurgitation post-intervention >1 (p=0.02), prior balloon aortic valvuloplasty (p=0.04), and increased left ventricular posterior wall thickness (p=0.1).Conclusions:Aortic valve intervention is a safe and effective procedure for congenital aortic stenosis with excellent survival results. However, rate of reintervention is high and influenced by increased left ventricular posterior wall thickness pre-intervention, prior balloon valvuloplasty, higher residual peak systolic valve gradient, and more than mild regurgitation post-intervention. The study highlights that long-term follow-up is recommended for these patients.

The Ross-Konno Procedure as Reoperative Treatment in a Young Adult with Congenital Aortic Stenosis

2012 ◽  
Vol 15 (4) ◽  
pp. 182
Author(s):  
Fotios A. Mitropoulos ◽  
Meletios A. Kanakis ◽  
Sotiria C. Apostolopoulou ◽  
Spyridon Rammos ◽  
Constantine E. Anagnostopoulos
Keyword(s):  
Young Adult ◽  
Aortic Valve ◽  
Aortic Stenosis ◽  
Aortic Valve Replacement ◽  
Severe Aortic Stenosis ◽  
Young Patients ◽  
Uneventful Recovery ◽  
Ross Procedure ◽  
Congenital Aortic Stenosis ◽  
Biological Prostheses

<p>Mechanical and biological prostheses are valid options when aortic valve replacement is necessary. The Ross procedure is also an alternative solution, especially for young patients.</p><p>We describe the case of a young patient with congenital aortic stenosis and bicuspid aortic valve who presented with dyspnea on exertion. An open commissurotomy was performed, and within 8 months the patient developed recurrent symptoms of severe aortic stenosis. He underwent redo sternotomy and a Ross-Konno procedure with an uneventful recovery.</p>

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