Subvalvular aortic stenosis is an abnormality of the subaortic region currently classified as a non-cyanotic congenital heart malformation that causes the partial obstruction of the left ventricle outflow tract. This condition has a progressive and recurrent character.
Presentation
Subvalvular aortic stenosis is more frequently seen in males [1] and may be asymptomatic during the early childhood. In this case, it is incidentally discovered during a heart murmur evaluation [2]. Symptoms tend to occur as the patient grows older [3] and the lesion becomes more severe. Initially, the patient comes with complaints that only install during physical efforts, such as dyspnea, dizziness, presyncope or angina with normal coronary arteries. In more advanced stages, veritable syncope is caused by less intense effort. Additional complaints include orthopnea and other heart failure manifestations. Sudden cardiac death may be the first sign of disease. Orthopnea may signify the presence of pulmonary venous hypertension, while syncope may be due to an arrhythmia, as well.
The stature and ponderal growth of affected children are normal. In cases with other heart malformations, like a ventricular septal defect, patent ductus arteriosus, pulmonic stenosis or aortic coarctation [4], the physical evaluation may fail to raise subvalvular aortic stenosis suspicion. Jugular inspection reveals a prominent "a" wave, caused by decreased compliance of the right ventricle. Arterial pulses are symmetrical and seldom decreased. Carotid palpation may prove the presence of an arterial thrill, while a precordial thrill may be felt upon palpation of this area. The apical impulse is strong. Auscultation highlights the existence of a narrow or paradoxical split second heart sound, associated with an ejection murmur, best heard in the middle left sternal border, radiating to the upper left border of the sternum, with a longer duration if the obstruction is more severe. The physician can differentiate this murmur from that of valvular aortic stenosis based on the absence of clicks. Other murmurs, like that of aortic or mitral regurgitations, sometimes coexist.
Entire Body System
- Congestive Heart Failure
Congenital mitral stenosis, subvalvular aortic stenosis, and congestive heart failure in a duck. The cardiac pathologies of VSD, PDA, subvalvular aortic stenosis and ASD have been reported in previous literature. [medical-dictionary.thefreedictionary.com]
Physical findings: Aortic stenosis is often diagnosed due to the presence of a heart murmur. Infants may have symptoms of congestive heart failure as described above as well as weak pulses. [mottchildren.org]
For example, the treatment for heartworm or hyperthyroidism is drastically different from the treatment for congestive heart failure. In some cases, especially with young dogs with mild murmurs, the murmur may even resolve on its own. [akc.org]
Heart failure occurs only occasionally in pediatric patients. When present early in life, heart failure usually results from associated congenital heart defects. [emedicine.medscape.com]
- Veterinarian
Knowing the type of murmur can help your veterinarian figure out what is causing the murmur. Heart Murmur Grade Another tool veterinarians use to help diagnose the cause of the murmur is grading. [akc.org]
A cardiologist, is more likely to auscultate a murmur than a regular veterinarian. [rottclub.ca]
Tests such as chest X-rays, echocardiography, and electrocardiography can be performed to evaluate the severity of the situation The condition is usually detected during puppy visits to the veterinarian by hearing a heart murmur during physical examination [en.wikipedia.org]
The condition is usually detected during puppy vaccine visits to the veterinarian by hearing a heart murmur during physical examination. A heart murmur is the abnormal sound of blood rushing through one of the heart valves. [web.archive.org]
- Anemia
In patients with aortic stenosis and GB, the main treatment is aortic valve replacement but the patients may be unfit to undergo surgery due to the complicating anemia. [pure.au.dk]
Murmurs can exist due to a large number of heart problems (infection, trauma, anemia, etc.; some are innocent, with no cardiac pathology. [en.wikipedia.org]
Heart murmurs are graded on a scale of 1 to 6, with one being very mild and six being very serious Murmurs can exist due to a large number of heart problems ( infection, trauma, anemia, etc.) and some murmurs are innocent, meaning that no cardiac pathology [rottclub.ca]
Other conditions that can cause systolic murmurs include: Anemia Heartworm disease Hyperthyroidism Cardiomyopathy Aortic valve insufficiency Mitral and tricuspid heart failure Systolic anterior mitral motion (SAM) Endocarditis of the mitral and tricuspid [akc.org]
Respiratoric
- Exertional Dyspnea
Among symptomatic patients, exertional dyspnea is the most common manifestation. (See Clinical Presentation.) [emedicine.medscape.com]
Cardiovascular
- Heart Disease
The COACH program focuses on: Adults with congenital heart disease Pulmonary hypertension Cardiovascular connective tissue disorders Pregnancy in women with heart disease Transition of adolescents with congenital heart disease into adult congenital heart [wexnermedical.osu.edu]
[…] of Adults With Congenital Heart Disease). [wikidoc.org]
Subvalvular aortic stenosis (SAS) is one of the common adult congenital heart diseases, with a prevalence of 6.5%. It is usually diagnosed in the first decade of life. Echocardiography is the test of choice to diagnose SAS. [ncbi.nlm.nih.gov]
Task Force 1: congenital heart disease. J Am Coll Cardiol 1994, 24 :867–873. [link.springer.com]
- Heart Murmur
Murmurs can exist due to a large number of heart problems (infection, trauma, anemia, etc.; some are innocent, with no cardiac pathology. [en.wikipedia.org]
Innocent Murmurs in Dogs Heart murmurs sound serious, and often are, but sometimes a heart murmur is classified as an innocent heart murmur. These heart murmurs are not serious and often resolve with time. [akc.org]
Heart Disease Rottweilers are a breed which has a high incidence of an abnormal heart sound, or murmur, caused by Subaortic stenosis (SAS). [rottclub.ca]
The condition is usually detected during puppy vaccine visits to the veterinarian by hearing a heart murmur during physical examination. A heart murmur is the abnormal sound of blood rushing through one of the heart valves. [web.archive.org]
[…] if there is a history of a heart murmur in other close family members. [chop.edu]
- Heart Failure
SOURCES: American Heart Association: "Heart Failure." Heart Failure Society of America. Texas Heart Institute. National Heart Lung and Blood Institute. SOURCES: American Heart Association: "Heart Failure." Heart Failure Society of America. [webmd.com]
The gradual worsening of heart failure took place parallel to the increase in severity of mitral regurgitation in a short period. The patient underwent successful mitral valve replacement with myectomy. [ncbi.nlm.nih.gov]
Puppies and adult dogs diagnosed with subaortic stenosis can suffer from heart failure and sudden death. [en.wikipedia.org]
In more severe cases in babies, symptoms include: Rapid breathing Rapid heart rate Inability to eat Heart failure In more severe cases in older children, symptoms include: Fatigue Fainting Chest pain Why choose Ohio State for subvalvular aortic stenosis [wexnermedical.osu.edu]
Congenital mitral stenosis, subvalvular aortic stenosis, and congestive heart failure in a duck. The cardiac pathologies of VSD, PDA, subvalvular aortic stenosis and ASD have been reported in previous literature. [medical-dictionary.thefreedictionary.com]
- Systolic Murmur
Your veterinarian will investigate these diseases based on the type of murmur. Causes of Systolic Murmurs in Dogs Most heart murmurs are systolic. [akc.org]
- Continuous Murmur
Causes of Continuous Murmurs in Dogs Continuous murmurs are most commonly caused by PDA, but aortic regurgitation caused by a ventricular septal defect, and aortic stenosis with aortic regurgitation, can also cause a continuous murmur. [akc.org]
Workup
Given that pure subvalvular aortic stenosis is a non-cyanotic congenital heart malformation, blood workup is usually noncontributory. The electrocardiogram depicts the degree of underlying left ventricular hypertrophy in most patients. The strain pattern, as well precordial deep Q waves are rarely seen.
Echocardiography is the diagnostic method of choice. This imaging is able to describe the shape of the left ventricular outflow tract, the degree of obstruction, the existence of associated congenital abnormalities, like aortic coarctation or patent ductus arteriosus [5] and disease consequences [6]. The color doppler probe placed in the left ventricular outflow tract reveals a turbulent flow [7], the first obstruction indicator. The M-mode cursor put in the same area demonstrates the presence of early closure and flutter of the aortic valve leaflets. It is important to characterize the length and position of the lesions and their relationship with the mitral and aortic valves and this is done using parasternal, apical and subcostal views. The gravity of the disease is assessed by continuous doppler wave interrogation, based on mean pressure gradient across the left ventricle outflow tract. However, this is not a reliable method in tunnel-like or multiple lesions. In these cases, a cardiac catheterization with pullback pressure measurement is needed.
The echocardiography should be performed several times in order to observe disease progression, to characterize left and right ventricular filling and function and the impact of the condition on the aortic valve in terms of regurgitation [8] [9]. In cases where the acoustic window is poor, diagnosis is aided by transesophageal echocardiography. This is most useful in severe, symptomatic patients that are going to be referred for surgery [10]. Supplementary information, like the severity of mitral regurgitation, the existence of ventricular septal defects or the exact anatomy of the left ventricle is offered by a left ventriculogram.
Hypertrophy
- Left Ventricular Hypertrophy
When last seen, 90% of the patients were asymptomatic, left ventricular hypertrophy on ECG had disappeared in 63%. No patient had atrioventricular block. [ncbi.nlm.nih.gov]
The electrocardiogram depicts the degree of underlying left ventricular hypertrophy in most patients. The strain pattern, as well precordial deep Q waves are rarely seen. Echocardiography is the diagnostic method of choice. [symptoma.com]
There is mild concentric left ventricular hypertrophy (LVH) due to increased left ventricular systolic pressure. [cardiacmri.com]
Treatment
To our knowledge, this case report is the first to describe how this new drug combination therapy is an effective treatment of GB from angiodysplasias and can be used to bridge to surgical or endovascular treatment. [pure.au.dk]
Surgical correction is the best treatment modality, and the prognosis is usually excellent. [ncbi.nlm.nih.gov]
Prognosis
Surgical correction is the best treatment modality, and the prognosis is usually excellent. [ncbi.nlm.nih.gov]
What Is the Prognosis for a Dog With a Heart Murmur? Heart murmurs can be serious, but they are not a cause for panic. Many of the causes of heart murmurs are treatable, and in some cases, may resolve on their own. [akc.org]
The health status of the puppy and the advice of the veterinarian may warrant investigative procedures in order to give a prognosis. [rottclub.ca]
Prognosis Although natural history studies have not delineated the annual mortality rate, 2-10% of sudden deaths are reported in untreated individuals with severe left ventricular outflow tract (LVOT) obstruction, including subvalvar aortic stenosis [emedicine.medscape.com]
Etiology
Etiology The etiology of subvalvar aortic stenosis (SAS) still is not fully understood. [emedicine.medscape.com]
Epidemiology
It can be classified as fixed or dynamic type depending on the dynamics of the obstruction. [1] Epidemiology and Demographics Aortic subvalvular stenosis is the second most common form of congenital left ventricular outflow tract obstruction and occurs [wikidoc.org]
However, the Newfoundland dog has been shown to experience an increased incidence of subaortic membranes, which may in part be secondary to inbreeding and is consistent with an autosomal inheritance. [10] Epidemiology The approximate incidence of congenital [emedicine.medscape.com]
Pathophysiology
In this review, we describe the pathophysiology, diagnosis, prognosis, and management of SAS with a focus on different pathophysiologic mechanisms, diagnostic approach, and prognosis of the disease by reviewing the current literature. © 2018 Wiley Periodicals [ncbi.nlm.nih.gov]
HOCM (a.k.a. idiopathic hypertrophic subaortic stenosis or IHSS ) is not present at birth and is not considered a congenital lesion. [2] Pathophysiology There are several varieties of congenital aortic subvalvular stenosis (or subaortic stenosis): [1] [wikidoc.org]
(See Pathophysiology and Treatment and Management.) Go to Aortic Stenosis for more complete information on this topic. [emedicine.medscape.com]
Prevention
Consequently, it is reasonable to consider the role of alternative therapies which may help prevent recurrence in selected cases. [ncbi.nlm.nih.gov]
: Prevention of bacterial endocarditis: recommendations by American Heart Association. JAMA 1997, 77 :1794–1801. The most updated American Heart Association guidelines for bacterial endocarditis prophylaxis. CrossRef Google Scholar 17. Elkins RC. [link.springer.com]
Repair for discrete membranous stenosis is done to prevent damage to the aortic valve and to preserve left ventricular function. [mottchildren.org]
The aortic valve is located between the left ventricle and the aorta and prevents blood from leaking back into the heart between beats. A normal aortic valve is made up of three thin leaflets. [chop.edu]
References
- Kitchiner D, Jackson M, Malaiya N, et al. Incidence and prognosis of obstruction of the left ventricular outflow tract in Liverpool (1960-91): a study of 313 patients. Br Heart J. 1994;71:588.
- De Vries AG, Hess J, Witsemburg M, et al. Management of fixed subaortic stenosis- a retrospective study of 57 cases. J Am Coll Cardiol. 1992;19:1013-7.
- Bezold LI, Smith O, Kelly K, et al. Development and validation of an echocardiographic model for predicting progression of discrete subaortic stenosis in children. Am J Cardiol. 1998;81:314–20.
- Choi JY, Sullivan ID. Fixed subaortic stenosis: anatomical spectrum and nature of progression. Br Heart J. 1991;65:280.
- Lopes R, Lourenco P, Goncalves A, et al. The natural history of congenital subaortic stenosis. Congenit Heart Dis. 2011;6:417–23.
- De Agustin J, Gomez de Diego J, Marcos-Alberca P, et al. Combined subaortic membrane and aortic valve stenosis: additive value of three-dimensional echocardiography. Eur Heart J Cardiovasc Imaging. 2014;15(4):388.
- Piacentini G, Marino B, Digilio M. Familial recurrence of discrete membranous subaortic stenosis. J Thorac Cardiovasc Surg. 2007;134:818–9.
- Warnes C, Williams R, Bashore TM, et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiol. 2008;52:e143–263.
- Valeske K, Huber C, Mueller M, et al. The dilemma of subaortic stenosis: A single-center experience of 15 years with a review of the literature. Thorac Cardiovasc Surg. 2011;59:293–7.
- Barkhordarian R, Uemura H, Rigby ML, et al. A retrospective review in 50 patients with subaortic stenosis and intact ventricular septum: 5-year surgical experience. Interact Cardiovasc Thorac Surg. 2007;6:35–8.