| Ventricular outflow tract obstruction | |
|---|---|
 | |
| Human heart | |
| Specialty | Cardiology |
| Causes | Congenital or acquired |
| Treatment | Surgery, medications, catheter based procedures (i.e. valvuloplasty) |
A ventricular outflow tract obstruction is a heart condition in which either the right or left ventricular outflow tract is blocked or obstructed. These obstructions represent a spectrum of disorders. Majority of these cases are congenital, but some are acquired throughout life. [1]
A right ventricular outflow tract obstruction (RVOTO) may be due to a defect in the pulmonic valve, the supravalvar region, the infundibulum, or the pulmonary artery. [2]
A left ventricular outflow tract obstruction (LVOTO) may be due to a defect in the aortic valve, or a defect located at the subvalvar or supravalvar level. [1] [3]
A ventricular outflow tract obstruction means there is a limitation in the blood flow out of either the right or left ventricles of the heart, depending on where the obstruction is. This can lead to cardiac hypertrophy, dilatation of the heart, and ultimately heart failure in some cases. [1] The right side of the heart is much smaller and weaker than the left side of the heart. It pumps de-oxygenated blood into the lungs. The left side of the heart is more muscular than the right side of the heart. It pumps oxygenated blood from the lungs into the aorta to perfuse the rest of the body. [4] When the heart has to pump against increased resistance, or afterload, as in the case of a ventricular obstruction, it compensates by growing in size. This adaptation is beneficial to pump blood past the obstruction, but eventually this hypertrophy can lead to other problems including arrhythmias, ischemia, and heart failure. [5]
In hypertrophic cardiomyopathy, there is disorganized production of cardiac myocytes leading to increased septal wall thickness and a pathologic motion of the mitral valve. The anterior mitral valve moves anteriorly during systole in these patients leading to dynamic LVOTO, and the increased mass of the septum leads to a physical narrowing for blood to pass through. Thus, both of these contribute to the left ventricular outflow tract obstruction seen in some cases of this disease. If severe enough, this condition requires prompt treatment, as these patients are at risk for lethal tachyarrythmias. [1]
Aortic valve stenosis is the most common cause of LVOTO. Aortic valve stenosis means the aortic valve has narrowed and is not opening freely. The aortic valve opens to allow blood to flow from the left ventricle to the aorta. Stenosis here leads to a narrowing of the passage for blood to flow out of the left ventricle, thus a LVOTO. More than 50% of patients with aortic valve stenosis have a congenital heart abnormality called a bicuspid aortic valve. The aortic valve is normally three leaflets but when it is bicuspid it is made of two. [6] This increases the risk for aortic stenosis due to increased stress on the leaflets, calcium deposition, turbulent blood flow, and scarring. If the stenosis is severe enough, surgical intervention may be necessary. [1]
A heart valve is a one-way valve that allows blood to flow in one direction through the chambers of the heart. Four valves are usually present in a mammalian heart and together they determine the pathway of blood flow through the heart. A heart valve opens or closes according to differential blood pressure on each side.
The aortic valve is a valve in the heart of humans and most other animals, located between the left ventricle and the aorta. It is one of the four valves of the heart and one of the two semilunar valves, the other being the pulmonary valve. The aortic valve normally has three cusps or leaflets, although in 1–2% of the population it is found to congenitally have two leaflets. The aortic valve is the last structure in the heart the blood travels through before stopping the flow through the systemic circulation.
Heart murmurs are unique heart sounds produced when blood flows across a heart valve or blood vessel. This occurs when turbulent blood flow creates a sound loud enough to hear with a stethoscope. Turbulent blood flow is not smooth. The sound differs from normal heart sounds by their characteristics. For example, heart murmurs may have a distinct pitch, duration and timing. The major way health care providers examine the heart on physical exam is heart auscultation; another clinical technique is palpation, which can detect by touch when such turbulence causes the vibrations called cardiac thrill. A murmur is a sign found during the cardiac exam. Murmurs are of various types and are important in the detection of cardiac and valvular pathologies.
Tetralogy of Fallot (TOF), formerly known as Steno-Fallot tetralogy, is a congenital heart defect characterized by four specific cardiac defects. Classically, the four defects are:
Hypertrophic cardiomyopathy is a condition in which the heart becomes thickened without an obvious cause. The parts of the heart most commonly affected are the interventricular septum and the ventricles. This results in the heart being less able to pump blood effectively and also may cause electrical conduction problems.
Bicuspid aortic valve is a form of heart disease in which two of the leaflets of the aortic valve fuse during development in the womb resulting in a two-leaflet (bicuspid) valve instead of the normal three-leaflet (tricuspid) valve. BAV is the most common cause of heart disease present at birth and affects approximately 1.3% of adults. Normally, the mitral valve is the only bicuspid valve and this is situated between the heart's left atrium and left ventricle. Heart valves play a crucial role in ensuring the unidirectional flow of blood from the atrium to the ventricles, or from the ventricle to the aorta or pulmonary trunk. BAV is normally inherited.
A congenital heart defect (CHD), also known as a congenital heart anomaly, congenital cardiovascular malformation, and congenital heart disease, is a defect in the structure of the heart or great vessels that is present at birth. A congenital heart defect is classed as a cardiovascular disease. Signs and symptoms depend on the specific type of defect. Symptoms can vary from none to life-threatening. When present, symptoms are variable and may include rapid breathing, bluish skin (cyanosis), poor weight gain, and feeling tired. CHD does not cause chest pain. Most congenital heart defects are not associated with other diseases. A complication of CHD is heart failure.
The Rastelli procedure is an open heart surgical procedure developed by Italian physician and cardiac surgery researcher, Giancarlo Rastelli, in 1967 at the Mayo Clinic, and involves using a pulmonary or aortic homograft conduit to relieve pulmonary obstruction in double outlet right ventricle with pulmonary stenosis.
Hypoplastic left heart syndrome (HLHS) is a rare congenital heart defect in which the left side of the heart is severely underdeveloped and incapable of supporting the systemic circulation. It is estimated to account for 2-3% of all congenital heart disease. Early signs and symptoms include poor feeding, cyanosis, and diminished pulse in the extremities. The etiology is believed to be multifactorial resulting from a combination of genetic mutations and defects resulting in altered blood flow in the heart.
A transthoracic echocardiogram (TTE) is the most common type of echocardiogram, which is a still or moving image of the internal parts of the heart using ultrasound. In this case, the probe is placed on the chest or abdomen of the subject to get various views of the heart. It is used as a non-invasive assessment of the overall health of the heart, including a patient's heart valves and degree of heart muscle contraction. The images are displayed on a monitor for real-time viewing and then recorded.
Ventricular hypertrophy (VH) is thickening of the walls of a ventricle of the heart. Although left ventricular hypertrophy (LVH) is more common, right ventricular hypertrophy (RVH), as well as concurrent hypertrophy of both ventricles can also occur.
Valvular heart disease is any cardiovascular disease process involving one or more of the four valves of the heart. These conditions occur largely as a consequence of aging, but may also be the result of congenital (inborn) abnormalities or specific disease or physiologic processes including rheumatic heart disease and pregnancy.
Pulsus bisferiens, also known as biphasic pulse, is an aortic waveform with two peaks per cardiac cycle, a small one followed by a strong and broad one. It is a sign of problems with the aorta, including aortic stenosis and aortic regurgitation, as well as hypertrophic cardiomyopathy causing subaortic stenosis.
A right-to-left shunt is a cardiac shunt which allows blood to flow from the right heart to the left heart. This terminology is used both for the abnormal state in humans and for normal physiological shunts in reptiles.
The following outline is provided as an overview of and topical guide to cardiology, the branch of medicine dealing with disorders of the human heart. The field includes medical diagnosis and treatment of congenital heart defects, coronary artery disease, heart failure, valvular heart disease and electrophysiology. Physicians who specialize in cardiology are called cardiologists.
Hypoplastic right heart syndrome is a congenital heart defect in which the structures on the right side of the heart, particularly the right ventricle, are underdeveloped. This defect causes inadequate blood flow to the lungs, and thus a cyanotic infant.
A plot of a system's pressure versus volume has long been used to measure the work done by the system and its efficiency. This analysis can be applied to heat engines and pumps, including the heart. A considerable amount of information on cardiac performance can be determined from the pressure vs. volume plot. A number of methods have been determined for measuring PV-loop values experimentally.
Apicoaortic Conduit (AAC), also known as Aortic Valve Bypass (AVB), is a cardiothoracic surgical procedure that alleviates symptoms caused by blood flow obstruction from the left ventricle of the heart. Left ventricular outflow tract obstruction (LVOTO) is caused by narrowing of the aortic valve (aortic stenosis) and other valve disorders. AAC, or AVB, relieves the obstruction to blood flow by adding a bioprosthetic valve to the circulatory system to decrease the load on the aortic valve. When an apicoaortic conduit is implanted, blood continues to flow from the heart through the aortic valve. In addition, blood flow bypasses the native valve and exits the heart through the implanted valved conduit. The procedure is effective at relieving excessive pressure gradient across the natural valve. High pressure gradient across the aortic valve can be congenital or acquired. A reduction in pressure gradient results in relief of symptoms.
The Yasui procedure is a pediatric heart operation used to bypass the left ventricular outflow tract (LVOT) that combines the aortic repair of the Norwood procedure and a shunt similar to that used in the Rastelli procedure in a single operation. It is used to repair defects that result in the physiology of hypoplastic left heart syndrome even though both ventricles are functioning normally. These defects are common in DiGeorge syndrome and include interrupted aortic arch and LVOT obstruction (IAA/LVOTO); aortic atresia-severe stenosis with ventricular septal defect (AA/VSD); and aortic atresia with interrupted aortic arch and aortopulmonary window. This procedure allows the surgeon to keep the left ventricle connected to the systemic circulation while using the pulmonary valve as its outflow valve, by connecting them through the ventricular septal defect. The Yasui procedure includes a modified Damus–Kaye–Stansel procedure to connect the aortic and pulmonary roots, allowing the coronary arteries to remain perfused. It was first described in 1987.
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