Pulmonary valve stenosis

Last updated
Pulmonary valve stenosis
Other namesValvular pulmonary stenosis [1]
Pulmonary valve stenosis.svg
Specialty Cardiology
Symptoms Cyanosis, dizziness [2]
CausesCongenital (most often) [3]
Diagnostic method Echocardiogram, Ultrasound [4]
TreatmentValve replacement or surgical repair

Pulmonary valve stenosis (PVS) is a heart valve disorder. Blood going from the heart to the lungs goes through the pulmonary valve, whose purpose is to prevent blood from flowing back to the heart. In pulmonary valve stenosis this opening is too narrow, leading to a reduction of flow of blood to the lungs. [1] [5]

Contents

While the most common cause of pulmonary valve stenosis is congenital heart disease, it may also be due to a malignant carcinoid tumor. Both stenosis of the pulmonary artery and pulmonary valve stenosis are forms of pulmonic stenosis (nonvalvular and valvular, respectively) [6] but pulmonary valve stenosis accounts for 80% of pulmonic stenosis. PVS was the key finding that led Jacqueline Noonan to identify the syndrome now called Noonan syndrome.

Symptoms and signs

Cyanosis Cynosis.JPG
Cyanosis

Among some of the symptoms consistent with pulmonary valve stenosis are the following: [2]

Cause

In regards to the cause of pulmonary valve stenosis a very high percentage are congenital, the right ventricular flow is hindered (or obstructed by this). The cause in turn is divided into: valvular, external and intrinsic (when it is acquired). [3]

Pathophysiology

The pathophysiology of pulmonary valve stenosis consists of the valve leaflets becoming too thick (therefore not separate one from another), which can cause high pulmonary pressure, and pulmonary hypertension. This however, does not mean the cause is always congenital. [7]

The left ventricle can be changed physically, these changes are a direct result of right ventricular hypertrophy. Once the obstruction is subdued, it (the left ventricle) can return to normal. [8]

Diagnosis

Pulmonary valve stenosis on an echocardiogram

The diagnosis of pulmonary valve stenosis can be made using stethoscopic auscultation of the heart, which can reveal a systolic ejection murmur that is best heard at the second left intercostal space. [9] Transthoracic or transesophageal echocardiography can provide a more accurate diagnosis. Obstetric ultrasonography can be useful for the in utero diagnosis of pulmonary valve stenosis and other congenital cardiovascular defects such as Tetralogy of Fallot.

Other conditions to consider in the differential diagnosis of pulmonic valvular stenosis include infundibular stenosis and pulmonary artery stenosis. [2]

Treatment

In terms of treatment for pulmonary valve stenosis, valve replacement or surgical repair (depending upon whether the stenosis is in the valve or vessel) may be indicated. If the valve stenosis is of congenital origin, balloon valvuloplasty is another option, depending on the case. Valves made from animal or human tissue (are used for valve replacement), in adults metal valves can be used. [10] [11]

Epidemiology

The epidemiology of pulmonary valve stenosis can be summed up by the congenital aspect which is the majority of cases, in broad terms PVS is rare in the general population. [4]

Related Research Articles

<span class="mw-page-title-main">Aortic stenosis</span> Narrowing of the exit of the hearts left ventricle

Aortic stenosis is the narrowing of the exit of the left ventricle of the heart, such that problems result. It may occur at the aortic valve as well as above and below this level. It typically gets worse over time. Symptoms often come on gradually with a decreased ability to exercise often occurring first. If heart failure, loss of consciousness, or heart related chest pain occur due to AS the outcomes are worse. Loss of consciousness typically occurs with standing or exercising. Signs of heart failure include shortness of breath especially when lying down, at night, or with exercise, and swelling of the legs. Thickening of the valve without narrowing is known as aortic sclerosis.

<span class="mw-page-title-main">Heart valve</span> A flap of tissue that prevent backflow of blood around the heart

A heart valve is a biological 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.

<span class="mw-page-title-main">Heart sounds</span> Noise generated by the beating heart

Heart sounds are the noises generated by the beating heart and the resultant flow of blood through it. Specifically, the sounds reflect the turbulence created when the heart valves snap shut. In cardiac auscultation, an examiner may use a stethoscope to listen for these unique and distinct sounds that provide important auditory data regarding the condition of the heart.

<span class="mw-page-title-main">Aortic valve</span> Valve in the human heart between the left ventricle and the aorta

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.

<span class="mw-page-title-main">Tetralogy of Fallot</span> Type of congenital heart defect

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:

<span class="mw-page-title-main">Afterload</span> Pressure in the wall of the left ventricle during ejection

Afterload is the pressure that the heart must work against to eject blood during systole. Afterload is proportional to the average arterial pressure. As aortic and pulmonary pressures increase, the afterload increases on the left and right ventricles respectively. Afterload changes to adapt to the continually changing demands on an animal's cardiovascular system. Afterload is proportional to mean systolic blood pressure and is measured in millimeters of mercury.

<span class="mw-page-title-main">Mitral stenosis</span> Heart disease with narrowing of valve

Mitral stenosis is a valvular heart disease characterized by the narrowing of the opening of the mitral valve of the heart. It is almost always caused by rheumatic valvular heart disease. Normally, the mitral valve is about 5 cm2 during diastole. Any decrease in area below 2 cm2 causes mitral stenosis. Early diagnosis of mitral stenosis in pregnancy is very important as the heart cannot tolerate increased cardiac output demand as in the case of exercise and pregnancy. Atrial fibrillation is a common complication of resulting left atrial enlargement, which can lead to systemic thromboembolic complications such as stroke.

<span class="mw-page-title-main">Aortic regurgitation</span> Medical condition

Aortic regurgitation (AR), also known as aortic insufficiency (AI), is the leaking of the aortic valve of the heart that causes blood to flow in the reverse direction during ventricular diastole, from the aorta into the left ventricle. As a consequence, the cardiac muscle is forced to work harder than normal.

<span class="mw-page-title-main">Congenital heart defect</span> Defect in the structure of the heart that is present at birth

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.

<span class="mw-page-title-main">Mitral regurgitation</span> Form of valvular heart disease

Mitral regurgitation(MR), also known as mitral insufficiency or mitral incompetence, is a form of valvular heart disease in which the mitral valve is insufficient and does not close properly when the heart pumps out blood. It is the abnormal leaking of blood backwards – regurgitation from the left ventricle, through the mitral valve, into the left atrium, when the left ventricle contracts. Mitral regurgitation is the most common form of valvular heart disease.

<span class="mw-page-title-main">Valvular heart disease</span> Disease in the valves of the heart

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.

Pulmonic stenosis, is a dynamic or fixed obstruction of flow from the right ventricle of the heart to the pulmonary artery. It is usually first diagnosed in childhood.

<span class="mw-page-title-main">Right ventricular hypertrophy</span> Medical condition

Right ventricular hypertrophy (RVH) is a condition defined by an abnormal enlargement of the cardiac muscle surrounding the right ventricle. The right ventricle is one of the four chambers of the heart. It is located towards the lower-end of the heart and it receives blood from the right atrium and pumps blood into the lungs.

Regurgitation is blood flow in the opposite direction from normal, as the backward flowing of blood into the heart or between heart chambers. It is the circulatory equivalent of backflow in engineered systems. It is sometimes called reflux.

<span class="mw-page-title-main">Tricuspid regurgitation</span> Type of valvular heart disease

Tricuspid regurgitation (TR), also called tricuspid insufficiency, is a type of valvular heart disease in which the tricuspid valve of the heart, located between the right atrium and right ventricle, does not close completely when the right ventricle contracts (systole). TR allows the blood to flow backwards from the right ventricle to the right atrium, which increases the volume and pressure of the blood both in the right atrium and the right ventricle, which may increase central venous volume and pressure if the backward flow is sufficiently severe.

<span class="mw-page-title-main">Lutembacher's syndrome</span> Medical condition

Lutembacher's syndrome is a very rare form of congenital heart disease that affects one of the chambers of the heart as well as a valve. It is commonly known as both congenital atrial septal defect (ASD) and acquired mitral stenosis (MS). Congenital atrial septal defect refers to a hole being in the septum or wall that separates the two atria; this condition is usually seen in fetuses and infants. Mitral stenosis refers to mitral valve leaflets sticking to each other making the opening for blood to pass from the atrium to the ventricles very small. With the valve being so small, blood has difficulty passing from the left atrium into the left ventricle. Septal defects that may occur with Lutembacher's syndrome include: Ostium primum atrial septal defect or ostium secundum which is more prevalent.

<span class="mw-page-title-main">Pulmonary regurgitation</span> Medical condition

Pulmonaryregurgitation is a condition in which the pulmonary valve is incompetent and allows backflow from the pulmonary artery to the right ventricle of the heart during diastole. While a small amount of backflow may occur ordinarily, it is usually only shown on an echocardiogram and is harmless. More pronounced regurgitation that is noticed through a routine physical examination is a medical sign of disease and warrants further investigation. If it is secondary to pulmonary hypertension it is referred to as a Graham Steell murmur.

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.

<span class="mw-page-title-main">Ventricular outflow tract obstruction</span> Medical condition

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.

References

  1. 1 2 "Pulmonary valve stenosis: MedlinePlus Medical Encyclopedia". www.nlm.nih.gov. Retrieved 2015-11-18.
  2. 1 2 3 "Pulmonic Valvular Stenosis Clinical Presentation: History, Physical, Causes". emedicine.medscape.com. Retrieved 2015-11-18.
  3. 1 2 Wang, Andrew; Bashore, Thomas M. (2010-01-14). Valvular Heart Disease. Springer Science & Business Media. p. 266. ISBN   9781597454117.
  4. 1 2 "Pulmonary Valve Disease. About Pulmonary valve disease | Patient". Patient. Retrieved 2015-11-18.
  5. Choices, NHS. "Congenital heart disease - Types - NHS Choices". www.nhs.uk. Retrieved 2015-11-18.
  6. Ren, XM; et al. (2014-12-23), "Pulmonic stenosis", Medscape.
  7. Levine, Shel; Coyne, Brian J.; Colvin, Lisa Cooper (2015-02-13). Clinical Exercise Electrocardiography. Jones & Bartlett Publishers. p. 14. ISBN   9781284034202.
  8. "Valvar Pulmonary Stenosis: Background, Pathophysiology, Epidemiology". 2018-06-07.{{cite journal}}: Cite journal requires |journal= (help)
  9. Armstrong, Guy P. (2021). "Pulmonic Stenosis". Merck Manual Professional Version . Merck & Co. Archived from the original on 2015-05-02. Retrieved 24 April 2022.
  10. Choices, NHS. "Congenital heart disease - Treatment - NHS Choices". www.nhs.uk. Retrieved 2015-11-18.
  11. "Balloon dilatation of pulmonary valve stenosis | Guidance and guidelines | NICE". www.nice.org.uk. Retrieved 2015-11-18.

Further reading