Pulmonary valve stenosis (PVS) is a

heart valve disorder Valvular heart disease is any cardiovascular disease process involving one or more of the four Heart valve, valves of the heart (the aortic valve, aortic and mitral valves on the left side of heart and the pulmonary valve, pulmonic and tricuspid ...

. Blood going from the

heart The heart is a muscular Organ (biology), organ in most animals. This organ pumps blood through the blood vessels of the circulatory system. The pumped blood carries oxygen and nutrients to the body, while carrying metabolic waste such as ca ...

to the

lungs The lungs are the primary organs of the respiratory system in humans and most other animals, including some snails and a small number of fish. In mammals and most other vertebrates, two lungs are located near the backbone on either si ...

goes through the

pulmonary valve The pulmonary valve (sometimes referred to as the pulmonic valve) is a valve of the heart that lies between the right ventricle and the pulmonary artery and has three cusps. It is one of the four valves of the heart and one of the two semilunar v ...

, 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. While the most common cause of pulmonary valve stenosis is

congenital heart disease A congenital heart defect (CHD), also known as a congenital heart anomaly 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 ...

, it may also be due to a malignant

carcinoid A carcinoid (also carcinoid tumor) is a slow-growing type of neuroendocrine tumor originating in the cells of the neuroendocrine system. In some cases, metastasis may occur. Carcinoid tumors of the midgut (jejunum, ileum, appendix, and cecum) ...

tumor. Both stenosis of the pulmonary artery and pulmonary valve stenosis are forms of pulmonic stenosis (nonvalvular and valvular, respectively) but pulmonary valve stenosis accounts for 80% of pulmonic stenosis. PVS was the key finding that led

Jacqueline Noonan Jacqueline Anne Noonan (October 28, 1928 – July 23, 2020) was an American pediatric cardiologist best known for her characterization of a genetic disorder now called Noonan syndrome. She was also the original describer of hypoplastic left hear ...

to identify the syndrome now called

Noonan syndrome Noonan syndrome (NS) is a genetic disorder that may present with mildly unusual facial features, short height, congenital heart disease, bleeding problems, and skeletal malformations. Facial features include widely spaced eyes, light-colored ...

Symptoms and signs

Among some of the symptoms consistent with pulmonary valve stenosis are the following: *

Heart murmur 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 di ...

Cyanosis Cyanosis is the change of body tissue color to a bluish-purple hue as a result of having decreased amounts of oxygen bound to the hemoglobin in the red blood cells of the capillary bed. Body tissues that show cyanosis are usually in locations ...

Dyspnea Shortness of breath (SOB), also medically known as dyspnea (in AmE) or dyspnoea (in BrE), is an uncomfortable feeling of not being able to breathe well enough. The American Thoracic Society defines it as "a subjective experience of breathing di ...

Dizziness Dizziness is an imprecise term that can refer to a sense of disorientation in space, vertigo, or lightheadedness. It can also refer to disequilibrium or a non-specific feeling, such as giddiness or foolishness. Dizziness is a common medical c ...

* Upper

thorax The thorax or chest is a part of the anatomy of humans, mammals, and other tetrapod animals located between the neck and the abdomen. In insects, crustaceans, and the extinct trilobites, the thorax is one of the three main divisions of the c ...

pain * Developmental disorders

Cause

In regards to the cause of pulmonary valve stenosis a very high percentage are congenital, the

right ventricular A ventricle is one of two large chambers toward the bottom of the heart that collect and expel blood towards the peripheral beds within the body and lungs. The blood pumped by a ventricle is supplied by an atrium, an adjacent chamber in the upper ...

flow is hindered (or obstructed by this). The cause in turn is divided into:

valvular A valve is a device or natural object that regulates, directs or controls the flow of a fluid (gases, liquids, fluidized solids, or slurries) by opening, closing, or partially obstructing various passageways. Valves are technically fitting ...

, external and intrinsic (when it is acquired).

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. The

left ventricle A ventricle is one of two large chambers toward the bottom of the heart that collect and expel blood towards the peripheral beds within the body and lungs. The blood pumped by a ventricle is supplied by an atrium, an adjacent chamber in the uppe ...

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.

Diagnosis

The diagnosis of pulmonary valve stenosis can be made using stethoscopic

auscultation Auscultation (based on the Latin verb ''auscultare'' "to listen") is listening to the internal sounds of the body, usually using a stethoscope. Auscultation is performed for the purposes of examining the circulatory and respiratory systems (he ...

of the heart, which can reveal a systolic ejection murmur that is best heard at the second left intercostal space. Transthoracic or transesophageal echocardiography can provide a more accurate diagnosis.

Obstetric ultrasonography Obstetric ultrasonography, or prenatal ultrasound, is the use of medical ultrasonography in pregnancy, in which sound waves are used to create real-time visual images of the developing embryo or fetus in the uterus (womb). The procedure is a stan ...

can be useful for the ''

in utero ''In Utero'' is the third and final studio album by American rock band Nirvana. It was released on September 21, 1993, by DGC Records. After breaking into the mainstream with their second album, ''Nevermind'' (1991), Nirvana hired Steve Albin ...

'' diagnosis of pulmonary valve stenosis and other congenital cardiovascular defects such as

Tetralogy of Fallot 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: *pulmonary stenosis, which is narrowing of the exit from the r ...

. Other conditions to consider in the

differential diagnosis In healthcare, a differential diagnosis (abbreviated DDx) is a method of analysis of a patient's history and physical examination to arrive at the correct diagnosis. It involves distinguishing a particular disease or condition from others that p ...

of pulmonic valvular stenosis include

infundibular An infundibulum (Latin for ''funnel''; plural, ''infundibula'') is a funnel-shaped cavity or organ. Anatomy * Brain: the pituitary stalk, also known as the ''infundibulum'' and ''infundibular stalk'', is the connection between the hypothalamus a ...

stenosis and pulmonary artery stenosis.

Treatment

In terms of treatment for pulmonary valve stenosis,

valve replacement Valve replacement surgery is the replacement of one or more of the heart valves with either an artificial heart valve or a bioprosthesis (homograft from human tissue or xenograft e.g. from pig). It is an alternative to valve repair. __TOC__ Proc ...

or surgical repair (depending upon whether the stenosis is in the valve or vessel) may be indicated. If the valve stenosis is of

congenital A birth defect, also known as a congenital disorder, is an abnormal condition that is present at birth regardless of its cause. Birth defects may result in disabilities that may be physical, intellectual, or developmental. The disabilities can ...

origin,

balloon valvuloplasty Heart valve repair is a cardiac surgery procedure, carried out to repair one or more faulty heart valves. In some valvular heart diseases repair where possible is preferable to valve replacement. A mechanical heart valve is a replacement valve ...

is another option, depending on the case. Valves made from animal or

human tissue In biology, tissue is a biological organizational level between cells and a complete organ. A tissue is an ensemble of similar cells and their extracellular matrix from the same origin that together carry out a specific function. Organs are th ...

(are used for valve replacement), in adults metal valves can be used.

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.

References

External links

Overview
at

American Heart Association The American Heart Association (AHA) is a nonprofit organization in the United States that funds cardiovascular medical research, educates consumers on healthy living and fosters appropriate cardiac care in an effort to reduce disability and deat ...

{{Congenital malformations and deformations of circulatory system Valvular heart disease Articles containing video clips