Congenital aortic valve stenosis patients could rapidly develop heart failure symptoms, such as tachypnea, deficient feeding, unsatisfactory ponderal curve, and decreased urine output. These become worse every day, as the patency of ductus arteriosus decreases. If the valvular stenosis is not critical, children remain asymptomatic for a period of time and the suspicion is raised by clinical examination performed for other conditions. It is estimated that less than 15% of patients are symptomatic before they reach the age of one year [1] [2]. However, congenital aortic valve stenosis patients may have certain complaints, such as asthenia, excessive sleeping, decreased exercise tolerance, presyncope, syncope, dyspnea, and chest pain. Sudden death during strenuous activities may be the first manifestation of congenital aortic valve stenosis.

In severely ill newborns, a murmur may not be audible or may be low grade and not accompanied by a thrill because of the low cardiac output. Clicks are also absent, because of the decreased mobility of the valve. In these patients, presentation resembles a sepsis case. Affected patients have symmetrically decreased or absent peripheral pulses and low peripheral perfusion, they are tachycardic, tachypneic, and dyspneic. If the stenosis is not critical, the patient is likely to be sent to a consultation with a pediatric cardiologist because an intense systolic murmur is heard.

Congenital aortic valve stenosis may be accompanied by aortic regurgitation. In this case, a balloon dilatation should not be performed. In cases where regurgitation is absent, this procedure may alleviate symptoms [3], but the valve may become stenotic again after a while or regurgitation may follow dilatation [4] [5].

In older children, the clinical diagnosis is easier to establish and is based on findings like a harsh systolic crescendo-descrescendo ejection murmur that peaks in mid-systole in the aortic auscultation area, which is more intense and longer if the valve is severely stenotic. A suprasternal thrill is often found. The murmur radiates to the neck and is better heard in a forward leaning, squatting or left decubitus position. The aortic component of the second heart sound is more or less delayed, depending on the severity of the obstruction, sometimes leading to a paradoxically split second heart sound. Left ventricular hypertrophy is suggested by the presence of the fourth heart sound or an increased apical impulse.

• Fever

  Rheumatic fever may damage more than one heart valve, and in more than one way. A damaged heart valve may not open fully or close fully — or both. While rheumatic fever is rare in the United States, some older adults had rheumatic fever as children. [mayoclinic.com]

  Among them are: Rheumatic fever : Strep throat can turn into rheumatic fever if it’s not properly treated. Rheumatic fever, which was once much more common in childhood than it is today, can damage your aortic valve. [webmd.com]

  Another cause is rheumatic fever. This condition can develop after strep throat or scarlet fever. Valve problems do not develop for 5 to 10 years or longer after rheumatic fever occurs. Rheumatic fever is becoming rarer in the United States. [nlm.nih.gov]

  Rheumatic Fever: Prior to the advent of antibiotics, many people developed rheumatic fever as a child or young adult after an episode of strep throat or scarlet fever. [sharecare.com]

• Fatigue

  Eventually the overworked left ventricle can fatigue, causing it to fail in its efforts to pump blood to the body, a condition called heart failure. [secondscount.org]

  Heart failure signs and symptoms include fatigue, shortness of breath, and swollen ankles and feet. When to see a doctor If you have a heart murmur, your doctor may recommend that you visit a cardiologist. [mayoclinic.com]

  This means that not enough blood can flow through it, which can lead to a variety of issues, including fatigue, blood clots, and heart failure. Mitral valve stenosis is usually a congenital heart defect meaning it is present at the time of birth. [stlouischildrens.org]

  Signs and symptoms of severe defects in newborns include Rapid breathing Cyanosis - a bluish tint to the skin, lips, and fingernails Fatigue Poor blood circulation Many congenital heart defects cause few or no signs and symptoms. [icdlist.com]

  Occasionally older children with long standing, significant aortic valve stenosis may develop symptoms with exercise such as fatigue or even fainting. Diagnosis of aortic valve stenosis can be made in a number of different ways. [pediatricheartspecialists.com]

• Congestive Heart Failure

  Sutter offers the widest array of heart-related services in Northern California, including expert diagnosis and treatment for heart attacks, congenital cardiac defects, congestive heart failure, valve conditions, heart arrhythmias, and circulation problems [checksutterfirst.org]

  It is a rough, low-pitched sound that is loudest at the base of the heart and most commonly heard in the second right intercostal space. If congestive heart failure leads to a fall in cardiac output, the murmur will be quieter. [patient.info]

  When the heart begins to fail, symptoms of congestive heart failure can develop including fatigue, weakness, shortness of breath with exercise or at night, and swelling in the ankles. [healthcentral.com]

  Life expectancy with onset of symptoms is approximately 3 years, and only 1year in those after the onset of congestive heart failure. Surgical in- hospital mortality rate is approximately 3%. [cthsurgery.com]

• Weight Gain

  In most cases, the symptoms that are most identifiable are rapid breathing, slow growth/weight gain or a soft heart murmur. [stlouischildrens.org]

  Nutrition Some babies with tricuspid atresia become tired while feeding and do not eat enough to gain weight. To make sure babies have a healthy weight gain, a special high-calorie formula might be prescribed. [cdc.gov]

• Regurgitation

  Substantial increases in aortic regurgitation (greater than three of five grades) occurred in three of eight unicommissural and one of 50 bicommissural valves. [ncbi.nlm.nih.gov]

• Nausea

  Case Report Patient A.M., male, 76 years old, sought medical attention complaining of chest tightness, without irradiation, associated with nausea and sweating, usually starting with minor stresses. [web.archive.org]

  Vasopressin Isolated in 1951, 16 the nonapeptide vasopressin or “antidiuretic hormone” is stored primarily in granules in the posterior pituitary gland and is released after increased plasma osmolality or hypotension, as well as pain, nausea, and hypoxia [doi.org]

• Heart Disease

  […] defect (Medical Encyclopedia) [ Read More ] Heart Valve Diseases Also called: Valvular heart disease Your heart has four valves. [icdlist.com]

  Aortic stenosis is the most common heart valve disease in the elderly. It is associated with congenital bicuspid aortic valve and previous rheumatic heart disease, but is also often caused by calcification of a normal valve. [sciencedaily.com]

  Citation US Cardiology, 2007;4(1):91-3 Pages The prevalence of heart disease in pregnant women is estimated to be between 0.1 and 4%; although the prevalence has not changed for decades, the relative contribution of different types of heart disease varies [uscjournal.com]

  Pulmonary atresia is a form of heart disease in which the pulmonary valve does not form properly. It is present from birth (congenital heart disease). [medlineplus.gov]

  Why do we need new guidelines on valvular heart disease? 2.2. Content of these guidelines 2.3. New format of the guidelines 2.4 How to use these guidelines 3. [escardio.org]

• Chest Pain

  Clinical presentations range from a neonatal severe presentation often associated with sudden cardiac death, to a slowly progressive stenosis that presents later with cardiac murmur, chest pain, dizziness, and loss of consciousness with exercise-induced [orpha.net]

  Chest pain in patients with aortic stenosis is the same as chest pain (angina) experienced by patients with coronary artery disease. [medicinenet.com]

  Patients feel shortness of breath, dizziness, and chest pain. © 2018 Intermountain Healthcare. All rights reserved. The content presented here is for your information only. [intermountainhealthcare.org]

• Heart Murmur

  The main sign of heart valve disease is an unusual heartbeat sound called a heart murmur. Your doctor can hear a heart murmur with a stethoscope. But many people have heart murmurs without having a problem. [icdlist.com]

  A patient with aortic valve stenosis usually comes to attention due to the presence of a heart murmur. This simply refers to the sound that blood is making as it flows past the narrowed valve. [pediatricheartspecialists.com]

  Many times, a doctor will first notice your child has a heart murmur. Heart murmurs can be detected with a stethoscope during a routine physical examination or with an electrocardiogram (EKG or ECG). [stlouischildrens.org]

  […] if there is a history of a heart murmur in other close family members. [chop.edu]

  A child with this type will have an easily detected and identified heart murmur. [childrenshospital.org]

• Systolic Murmur

  In older children, the clinical diagnosis is easier to establish and is based on findings like a harsh systolic crescendo-descrescendo ejection murmur that peaks in mid-systole in the aortic auscultation area, which is more intense and longer if the valve [symptoma.com]

  Cardiovascular examination revealed ejective systolic murmur at aortic focus +++/6+, irradiating to the suprasternal notch. Pulmonary examination with crackling rales at the bases. Peripheral edema ++/4+, and good peripheral perfusion. [web.archive.org]

  (or ejection systolic) murmur that is heard on praecordial auscultation 1,2. [radiopaedia.org]

  The typical murmur of AS is a crescendo-decrescendo systolic ejection murmur shortly after the first heart sound that ends just before the second heart sound. [patient.info]

• Pulsus Parvus et Tardus

  Symptoms typically present on exertion, unless AS is severe Dyspnea Angina pectoris Dizziness and syncope Small blood pressure amplitude, decreased pulse pressure Cardiac exam (see cardiovascular examination ) Delayed and weak pulse ( Pulsus parvus et [amboss.com]

  Clinical examination classically reveals a narrow pulse pressure, a slow-rising peripheral pulse, pulsus parvus et tardus (weak and delayed pulse) in the carotid pulse, a pressure loaded ('heaving') apex beat, and a crescendo-decrescendo mid-systolic [radiopaedia.org]

  Signs Examination of the pulse in significant AS reveals a slow-rising, flat character called pulsus parvus et tardus. [ 7 ] Blood pressure will show a narrow pulse pressure (difference between systolic and diastolic pressures). [patient.info]

  The phenomenon known as pulsus parvus et tardus refers to a weak ( parvus ) and delayed ( tardus ) carotid upstroke. [healio.com]

• Flushing

  If skin contact occurs, immediately flush the affected area with water; in the event of contact with eyes, seek immediate medical attention. [newheartvalve.com]

  It may also be accompanied by the characteristic "Dresden china" appearance of pallor with a light flush. Angina in setting of heart failure also increases the risk of death. [en.wikipedia.org]

• Dizziness

  Clinical presentations range from a neonatal severe presentation often associated with sudden cardiac death, to a slowly progressive stenosis that presents later with cardiac murmur, chest pain, dizziness, and loss of consciousness with exercise-induced [orpha.net]

  Patients feel shortness of breath, dizziness, and chest pain. © 2018 Intermountain Healthcare. All rights reserved. The content presented here is for your information only. [intermountainhealthcare.org]

  Greater degrees of aortic stenosis can result in symptoms such as shortness of breath on exertion, chest pain, dizziness, or fainting. [cumc.columbia.edu]

  In some children chest pain, unusual tiring, dizziness or fainting may occur. Otherwise, most children with aortic stenosis have no symptoms, and special tests may be needed to determine the severity of the problem. [heart.org]

  ( Thus, the first step in management of the patient with severe AS is to ensure the patient actually is asymptomatic based on a detailed discussion including any changes in levels of physical activity and directed questions about exertional dyspnea, dizziness [acc.org]

Workup should include a thoracic radiography, that may reveal the presence of cardiomegaly and pulmonary venous congestion in critical congenital aortic valve stenosis. The ascending aorta may be dilated. The diagnosis is established by echocardiography. Two-dimensional echo will characterize valve anatomy as well as the number and mobility of leaflets. Moreover, it will measure the size of the valvular ring. It is also important to describe the presence of associated abnormalities and characterize the left ventricular function. Continuous and pulsed Doppler echocardiography will determine the transvalvular maximum and mean gradient, thus establishing disease severity and need for effort restriction [6], assuming that left ventricular function is not compromised. Associated severe aortic regurgitation will lead to an overestimation of gradients by this imaging method [7]. It is important while measuring gradients to pay an attention to the angle between the jet flow and ultrasound beam (they should be parallel) [8]. Color Doppler will reveal a turbulent flow across the valve. Transesophageal echocardiography is rarely necessary and only applicable to adolescents and adults. Exercise testing is used for risk stratification and an assessment regarding the surgical intervention. Three-dimensional echocardiography, if available, is a useful tool in congenital aortic valve stenosis [9], allowing precise valve and volumes measurement and characterization of subvalvular membranes, if present [10].

Cardiac catheterization is performed before a balloon valvuloplasty and offers information about the peak systolic gradient and left ventricular filling pressures.

The electrocardiogram could reveal left ventricular hypertrophy, left bundle branch block, ST segment depression, and negative T waves [11]. Diffuse T-wave and ST-segment changes suggest severe stenosis [12].

Holter monitoring may demonstrate the presence of ventricular arrhythmias. Magnetic resonance imaging is used in patients with poor acoustic windows and is also useful in acknowledging the presence of endocardial fibroelastosis [13] [14].

• Left Axis Deviation

  References: [3] [7] [4] Diagnostics ECG Nonspecific for AS Signs of left ventricular hypertrophy (e.g., left axis deviation, positive Sokolow-Lyon index ) Chest x-ray Findings of left ventricular hypertrophy, such as left ventricular enlargement and rounded [amboss.com]

• Left Ventricular Hypertrophy

  Left ventricular hypertrophy is suggested by the presence of the fourth heart sound or an increased apical impulse. [symptoma.com]

  Echocardiography reveals atresia or dysplasia of the aortic valve most commonly associated with a bicuspid morphology, restricted left ventricular outflow, and left ventricular hypertrophy. [orpha.net]

  References: [3] [7] [4] Diagnostics ECG Nonspecific for AS Signs of left ventricular hypertrophy (e.g., left axis deviation, positive Sokolow-Lyon index ) Chest x-ray Findings of left ventricular hypertrophy, such as left ventricular enlargement and rounded [amboss.com]

  A S4 heart sound is also often present due to the severe concentric left ventricular hypertrophy that develops in aortic stenosis. [healio.com]

  Echocardiogram may also show left ventricular hypertrophy, thickened and immobile aortic valve and dilated aortic root. However, it may appear deceptively normal in acute cases. [en.wikipedia.org]

Children with mild aortic stenosis rarely require treatment. However, it is important to note that aortic stenosis may be progressive, and that children with mild disease may eventually require treatment later in life. [cincinnatichildrens.org]

GOAL: To evaluate the impact of the Ross operation, recently (1997) introduced in our unit, for the treatment of patients with congenital aortic valve stenosis. [ncbi.nlm.nih.gov]

More information Aortic Stenosis About.com: Explains what aortic stenosis is and looks at when it should be treated and what options there are. eMedicine Health Consumer health resource center providing information on causes, symptoms, and treatment option [curlie.org]

This review summarizes laboratory and recent clinical investigations concerning AS and current best treatment. [doi.org]

Mortality was 16.7% but patients presenting with critical aortic stenosis had a much worse prognosis. [ncbi.nlm.nih.gov]

Without surgery, the prognosis is extremely poor, with a 3-year survival rate Circ J 2011; 75: 11-19) [doi.org]

MSCT may be useful in quantifying the valve area and coronary calcification, which aids in assessing prognosis. [patient.info]

Classification and etiology There are numerous other predisposing factors and causes of aortic stenosis. [radiopaedia.org]

Below we discuss the most common etiologies that lead to aortic stenosis. Age-related Degenerative Calcific Aortic Stenosis Age related hemodynamic wear-and-tear of the aortic valve is the most common etiology of aortic stenosis. [pathwaymedicine.org]

This article reviews the etiology, pathophysiology, diagnosis, and management of valvular AS in adults. [doi.org]

[…] pressure gradient Valve area Symptoms normal 2-4 mmHg 3-4 cm 2 Mild < 25 mm Hg > 1.5 cm 2 asymptomatic Moderate 25–40 mm Hg 1–1.5 cm 2 mostly asymptomatic Severe > 40 mm Hg < 1.0 cm 2 possibly asymptomatic Very severe > 70 mm Hg < 0.6 cm 2 symptomatic By etiology [amboss.com]

Etiology – Aortic Stenosis The most common cause of aortic stenosis in a person over age 70 years is calcification of a normal trileaflet aortic valve; this process is sometimes referred to as “senile degeneration.” [healio.com]

Epidemiology Most common valvular heart disease in industrialized countries Prevalence : Increases with age May reach up to 12.4% among individuals ≥ 75 years References: [1] [2] Epidemiological data refers to the US, unless otherwise specified. [amboss.com]

Although epidemiologic studies cannot directly infer causality, our results suggest the existence of a familial component to the disease. [sciencedaily.com]

Morphological, Epidemiological, and Hereditary Aspects of Congenital Aortic Stenosis Congenital obstruction of the LVOT may involve the subvalvular, valvular, or supravalvular part of the outflow tract complex. [uscjournal.com]

Sudden death in aortic stenosis : epidemiology Only 2 studies have reported the occurrence of sudden death in truly asymptomatic patients. [escardio.org]

Epidemiology [ 1 ] AS has become the most frequent type of valvular heart disease in Europe and North America. [patient.info]

This study reports the case of Heyde's syndrome and discusses the pathophysiology bases and potential therapies for this syndrome. [web.archive.org]

Particular emphasis will be placed on pathophysiology and on the concept of afterload mismatch and preload reserve in which left ventricular function is proportionately matched to level of left ventricular pressure, mainly because such studies carry important [doi.org]

Pathophysiology of congenital AS The mechanism underlying the increase in severity of AS in children is similar to that seen in adults. [intechopen.com]

Pathophysiology A systolic pressure gradient between the pumping chamber and its downstream artery is the primary hemodynamic abnormality of stenosis regardless of whether the obstruction is right- or left-sided. [pedsinreview.aappublications.org]

Stepwise dilatation of the stenosis does not prevent this complication. The Inoue balloon catheter offers technical advantages. [ncbi.nlm.nih.gov]

Some possible ways to prevent aortic valve stenosis include: Taking steps to prevent rheumatic fever. [mayoclinic.com]

Medications There is encouraging information suggesting that cholesterol-lowering medications, such as statins, may prevent aortic valve stenosis or slow its progress in certain individuals. [web.archive.org]

Valve between the left ventricle and the aorta that prevents blood from flowing from the aorta back into the heart. [curlie.org]

When the heart relaxes, the valve closes, preventing backflow of blood into the heart. Aortic valve stenosis refers to a condition in which the valve is abnormally narrowed. [pediatricheartspecialists.com]

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