Pulmonary Stenosis

Valvular disorders can arise from the pulmonary valve Pulmonary valve A valve situated at the entrance to the pulmonary trunk from the right ventricle. Heart: Anatomy, located between the right ventricle (RV) and the pulmonary artery Pulmonary artery The short wide vessel arising from the conus arteriosus of the right ventricle and conveying unaerated blood to the lungs. Lungs: Anatomy (PA). Valvular disorders are diagnosed by echocardiography Echocardiography Ultrasonic recording of the size, motion, and composition of the heart and surrounding tissues. The standard approach is transthoracic. Tricuspid Valve Atresia (TVA). Pulmonary stenosis Stenosis Hypoplastic Left Heart Syndrome (HLHS) ( PS PS Invasive Mechanical Ventilation) is valvular narrowing causing RV outflow tract obstruction. Patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship are often asymptomatic unless they have other congenital Congenital Chorioretinitis cardiac anomalies or severe PS PS Invasive Mechanical Ventilation. Symptoms (exertional dyspnea Dyspnea Dyspnea is the subjective sensation of breathing discomfort. Dyspnea is a normal manifestation of heavy physical or psychological exertion, but also may be caused by underlying conditions (both pulmonary and extrapulmonary). Dyspnea, chest pain Pain An unpleasant sensation induced by noxious stimuli which are detected by nerve endings of nociceptive neurons. Pain: Types and Pathways, and syncope Syncope Syncope is a short-term loss of consciousness and loss of postural stability followed by spontaneous return of consciousness to the previous neurologic baseline without the need for resuscitation. The condition is caused by transient interruption of cerebral blood flow that may be benign or related to a underlying life-threatening condition. Syncope) are due to RV failure. Severe PS PS Invasive Mechanical Ventilation is treated surgically.

Last updated: Sep 29, 2022

Editorial responsibility: Stanley Oiseth, Lindsay Jones, Evelin Maza

Overview

Anatomy

Pulmonary (or pulmonic) valve:

  • Semilunar valve between the right ventricle (RV) and pulmonary artery Pulmonary artery The short wide vessel arising from the conus arteriosus of the right ventricle and conveying unaerated blood to the lungs. Lungs: Anatomy (PA)
  • Consists of 3 cusps: anterior, left, and right
  • Opens during systole Systole Period of contraction of the heart, especially of the heart ventricles. Cardiac Cycle as the RV pressure exceeds the pulmonary trunk Pulmonary Trunk Truncus Arteriosus pressure
  • Closes during diastole Diastole Post-systolic relaxation of the heart, especially the heart ventricles. Cardiac Cycle as the RV pressure drops ( S2 S2 Heart Sounds), preventing retrograde flow Flow Blood flows through the heart, arteries, capillaries, and veins in a closed, continuous circuit. Flow is the movement of volume per unit of time. Flow is affected by the pressure gradient and the resistance fluid encounters between 2 points. Vascular resistance is the opposition to flow, which is caused primarily by blood friction against vessel walls. Vascular Resistance, Flow, and Mean Arterial Pressure from the pulmonary trunk Pulmonary Trunk Truncus Arteriosus
Table: Overview of pulmonary valve Pulmonary valve A valve situated at the entrance to the pulmonary trunk from the right ventricle. Heart: Anatomy disorders
Pulmonary stenosis Stenosis Hypoplastic Left Heart Syndrome (HLHS) ( PS PS Invasive Mechanical Ventilation) Pulmonary regurgitation Pulmonary regurgitation Backflow of blood from the pulmonary artery into the right ventricle due to imperfect closure of the pulmonary valve. Pulmonary Regurgitation (PR)
Etiology Mostly congenital Congenital Chorioretinitis Mostly acquired
Murmur Systolic murmur, left upper sternal border (preceded by a systolic click that decreases with inspiration Inspiration Ventilation: Mechanics of Breathing) Diastolic murmur, left upper sternal border, increases with inspiration Inspiration Ventilation: Mechanics of Breathing
S2 S2 Heart Sounds Split S2 S2 Heart Sounds with soft and delayed P2 Split S2 S2 Heart Sounds with a loud P2
Echocardiographic findings Thick and domed leaflets, with increased systolic velocity across the valve, RVH Valvular abnormalities (depending on etiology), RV enlargement, and a regurgitant jet in the right ventricular outflow tract
RV: right ventricle
RVH: right ventricular hypertrophy Ventricular Hypertrophy Tetralogy of Fallot

Definition and Epidemiology

Definition

Pulmonary stenosis Stenosis Hypoplastic Left Heart Syndrome (HLHS) is the narrowing of the pulmonary valve Pulmonary valve A valve situated at the entrance to the pulmonary trunk from the right ventricle. Heart: Anatomy causing:

Pulmonary valve stenosis

Differentiation of the normal heart with a normal pulmonary valve Pulmonary valve A valve situated at the entrance to the pulmonary trunk from the right ventricle. Heart: Anatomy and a heart with pulmonary valve Pulmonary valve A valve situated at the entrance to the pulmonary trunk from the right ventricle. Heart: Anatomy stenosis Stenosis Hypoplastic Left Heart Syndrome (HLHS)

Image: “ Pulmonary valve Pulmonary valve A valve situated at the entrance to the pulmonary trunk from the right ventricle. Heart: Anatomy stenosis Stenosis Hypoplastic Left Heart Syndrome (HLHS)” by LadyofHats. License: Public domain.

Etiology and Classification

Etiology

  • Cases are often congenital Congenital Chorioretinitis:
    • Isolated valvular PS PS Invasive Mechanical Ventilation
    • Other congenital Congenital Chorioretinitis forms:
      • As part of Noonan syndrome Noonan syndrome A genetically heterogeneous, multifaceted disorder characterized by short stature, webbed neck, ptosis, skeletal malformations, hypertelorism, hormonal imbalance, cryptorchidism, multiple cardiac abnormalities (most commonly including pulmonary valve stenosis), and some degree of intellectual disability. The phenotype bears similarities to that of turner syndrome that occurs only in females and has its basis in a 45, X karyotype abnormality. Noonan syndrome occurs in both males and females with a normal karyotype (46, XX and 46, xy). Mutations in a several genes (ptpn11, kras, sos1, nf1 and raf1) have been associated the ns phenotype. Mutations in ptpn11 are the most common. Leopard syndrome, a disorder that has clinical features overlapping those of noonan syndrome, is also due to mutations in ptpn11. In addition, there is overlap with the syndrome called neurofibromatosis-noonan syndrome due to mutations in nf1. Hypogonadism
      • Tetralogy of Fallot Tetralogy of Fallot Tetralogy of Fallot is the most common cyanotic congenital heart disease. The disease is the confluence of 4 pathologic cardiac features: overriding aorta, ventricular septal defect, right ventricular outflow obstruction, and right ventricular hypertrophy. Tetralogy of Fallot (TOF):
      • Congenital rubella Congenital rubella Transplacental infection of the fetus with rubella usually in the first trimester of pregnancy, as a consequence of maternal infection, resulting in various developmental abnormalities in the newborn infant. They include cardiac and ocular lesions, deafness, microcephaly, mental retardation, and generalized growth retardation. Rubella Virus syndrome
  • Acquired PS PS Invasive Mechanical Ventilation:
    • Carcinoid syndrome Carcinoid syndrome A symptom complex associated with carcinoid tumor and characterized by attacks of severe flushing of the skin, diarrheal watery stools, bronchoconstriction, sudden drops in blood pressure, edema, and ascites. The carcinoid tumors are usually located in the gastrointestinal tract and metastasize to the liver. Symptoms are caused by tumor secretion of serotonin, prostaglandins, and other biologically active substances. Cardiac manifestations constitute carcinoid heart disease. Carcinoid Tumors and Syndrome
    • Rheumatic heart disease Rheumatic Heart Disease Cardiac manifestation of systemic rheumatological conditions, such as rheumatic fever. Rheumatic heart disease can involve any part the heart, most often the heart valves and the endocardium. Rheumatic Fever (rarely involves PS PS Invasive Mechanical Ventilation)
    • Previous cardiothoracic surgeries
Tetralogy of fallot

Comparison of the normal heart and a heart with TOF: pulmonary valve Pulmonary valve A valve situated at the entrance to the pulmonary trunk from the right ventricle. Heart: Anatomy stenosis Stenosis Hypoplastic Left Heart Syndrome (HLHS), overriding aorta Aorta The main trunk of the systemic arteries. Mediastinum and Great Vessels: Anatomy, RVH, VSD

Image: “ Tetralogy of Fallot Tetralogy of Fallot Tetralogy of Fallot is the most common cyanotic congenital heart disease. The disease is the confluence of 4 pathologic cardiac features: overriding aorta, ventricular septal defect, right ventricular outflow obstruction, and right ventricular hypertrophy. Tetralogy of Fallot” by LadyofHats. License: Public domain.

Types of stenosis Stenosis Hypoplastic Left Heart Syndrome (HLHS)

  • Valvular PS PS Invasive Mechanical Ventilation
    • Narrowed or thickened valve
    • Most common type
    • Associated with TOF, congenital rubella Congenital rubella Transplacental infection of the fetus with rubella usually in the first trimester of pregnancy, as a consequence of maternal infection, resulting in various developmental abnormalities in the newborn infant. They include cardiac and ocular lesions, deafness, microcephaly, mental retardation, and generalized growth retardation. Rubella Virus syndrome, Noonan syndrome Noonan syndrome A genetically heterogeneous, multifaceted disorder characterized by short stature, webbed neck, ptosis, skeletal malformations, hypertelorism, hormonal imbalance, cryptorchidism, multiple cardiac abnormalities (most commonly including pulmonary valve stenosis), and some degree of intellectual disability. The phenotype bears similarities to that of turner syndrome that occurs only in females and has its basis in a 45, X karyotype abnormality. Noonan syndrome occurs in both males and females with a normal karyotype (46, XX and 46, xy). Mutations in a several genes (ptpn11, kras, sos1, nf1 and raf1) have been associated the ns phenotype. Mutations in ptpn11 are the most common. Leopard syndrome, a disorder that has clinical features overlapping those of noonan syndrome, is also due to mutations in ptpn11. In addition, there is overlap with the syndrome called neurofibromatosis-noonan syndrome due to mutations in nf1. Hypogonadism, acquired PS PS Invasive Mechanical Ventilation from carcinoid syndrome Carcinoid syndrome A symptom complex associated with carcinoid tumor and characterized by attacks of severe flushing of the skin, diarrheal watery stools, bronchoconstriction, sudden drops in blood pressure, edema, and ascites. The carcinoid tumors are usually located in the gastrointestinal tract and metastasize to the liver. Symptoms are caused by tumor secretion of serotonin, prostaglandins, and other biologically active substances. Cardiac manifestations constitute carcinoid heart disease. Carcinoid Tumors and Syndrome
  • Subvalvular infundibular PS PS Invasive Mechanical Ventilation:
    • Fibromuscular narrowing of the infundibular region, with normal valve
    • Rare
    • Can be found with other congenital Congenital Chorioretinitis lesions (TOF, VSD)
  • Supravalvular (or peripheral) PS PS Invasive Mechanical Ventilation
    • Narrowing of the main PA or its branches, up to the periphery
    • Murmurs heard over the lungs Lungs Lungs are the main organs of the respiratory system. Lungs are paired viscera located in the thoracic cavity and are composed of spongy tissue. The primary function of the lungs is to oxygenate blood and eliminate CO2. Lungs: Anatomy
    • Mild form: nonpathologic, with murmur resolving by 6 months of age
    • Associated with congenital rubella Congenital rubella Transplacental infection of the fetus with rubella usually in the first trimester of pregnancy, as a consequence of maternal infection, resulting in various developmental abnormalities in the newborn infant. They include cardiac and ocular lesions, deafness, microcephaly, mental retardation, and generalized growth retardation. Rubella Virus syndrome or other anomalies (TOF, patent ductus arteriosus Ductus arteriosus A fetal blood vessel connecting the pulmonary artery with the descending aorta. Patent Ductus Arteriosus (PDA), VSD)

Pathophysiology and Clinical Presentation

Pathophysiology

  • PS PS Invasive Mechanical Ventilation causes sustained outflow tract obstruction, which increases right ventricular afterload Afterload Afterload is the resistance in the aorta that prevents blood from leaving the heart. Afterload represents the pressure the LV needs to overcome to eject blood into the aorta. Cardiac Mechanics
  • RV is less adaptive than the left ventricle, with early rises in pressures; so, with lower systolic pressures, RVH occurs.
  • Right atrial (RA) pressure increases as a result of elevated RV pressure.
  • Late in the disease: persistently increased RV strain → RV dysfunction occurs → eventual RV failure
  • Other complications: arrhythmia, infective endocarditis Infective endocarditis Infective endocarditis (IE) is caused by infection or inflammation of the inner lining of the heart (endocardium), most commonly affecting the heart valves. Endocarditis

Symptoms

  • Commonly asymptomatic during mild-to-moderate disease (majority of patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship)
  • Severe PS PS Invasive Mechanical Ventilation:
    • The more severe the obstruction is, the earlier it is detected.
    • Fatigue Fatigue The state of weariness following a period of exertion, mental or physical, characterized by a decreased capacity for work and reduced efficiency to respond to stimuli. Fibromyalgia and exertional dyspnea Dyspnea Dyspnea is the subjective sensation of breathing discomfort. Dyspnea is a normal manifestation of heavy physical or psychological exertion, but also may be caused by underlying conditions (both pulmonary and extrapulmonary). Dyspnea 
    • Anginal chest pain Pain An unpleasant sensation induced by noxious stimuli which are detected by nerve endings of nociceptive neurons. Pain: Types and Pathways from oxygen supply-demand mismatch
    • Syncope Syncope Syncope is a short-term loss of consciousness and loss of postural stability followed by spontaneous return of consciousness to the previous neurologic baseline without the need for resuscitation. The condition is caused by transient interruption of cerebral blood flow that may be benign or related to a underlying life-threatening condition. Syncope especially when accompanied by provoking factors (e.g., atrial fibrillation Atrial fibrillation Atrial fibrillation (AF or Afib) is a supraventricular tachyarrhythmia and the most common kind of arrhythmia. It is caused by rapid, uncontrolled atrial contractions and uncoordinated ventricular responses. Atrial Fibrillation, infection)

Signs

  • Prominent a wave in jugular venous pulse (strong atrial contraction)
  • Heart sounds Heart sounds Heart sounds are brief, transient sounds produced by valve opening and closure and by movement of blood in the heart. They are divided into systolic and diastolic sounds. In most cases, only the first (S1) and second (S2) heart sounds are heard. These are high-frequency sounds and arise from aortic and pulmonary valve closure (S1), as well as mitral and tricuspid valve closure (S2). Heart Sounds:
  • Left parasternal lift (from RVH) in severe PS PS Invasive Mechanical Ventilation
  • Cyanosis Cyanosis A bluish or purplish discoloration of the skin and mucous membranes due to an increase in the amount of deoxygenated hemoglobin in the blood or a structural defect in the hemoglobin molecule. Pulmonary Examination in those with a right-to-left shunt (e.g., atrial septal defect Atrial Septal Defect Atrial septal defects (ASDs) are benign acyanotic congenital heart defects characterized by an opening in the interatrial septum that causes blood to flow from the left atrium (LA) to the right atrium (RA) (left-to-right shunt). Atrial Septal Defect (ASD))
  • Very late in the course: peripheral edema Edema Edema is a condition in which excess serous fluid accumulates in the body cavity or interstitial space of connective tissues. Edema is a symptom observed in several medical conditions. It can be categorized into 2 types, namely, peripheral (in the extremities) and internal (in an organ or body cavity). Edema, hepatomegaly, ascites Ascites Ascites is the pathologic accumulation of fluid within the peritoneal cavity that occurs due to an osmotic and/or hydrostatic pressure imbalance secondary to portal hypertension (cirrhosis, heart failure) or non-portal hypertension (hypoalbuminemia, malignancy, infection). Ascites

Diagnosis

  • Transthoracic echocardiography Echocardiography Ultrasonic recording of the size, motion, and composition of the heart and surrounding tissues. The standard approach is transthoracic. Tricuspid Valve Atresia (TVA) ( TTE TTE Imaging of the Heart and Great Vessels) with Doppler Doppler Ultrasonography applying the doppler effect, with frequency-shifted ultrasound reflections produced by moving targets (usually red blood cells) in the bloodstream along the ultrasound axis in direct proportion to the velocity of movement of the targets, to determine both direction and velocity of blood flow. Ultrasound (Sonography)
    • 2-dimensional 2-Dimensional Imaging of the Heart and Great Vessels (2D) and Doppler Doppler Ultrasonography applying the doppler effect, with frequency-shifted ultrasound reflections produced by moving targets (usually red blood cells) in the bloodstream along the ultrasound axis in direct proportion to the velocity of movement of the targets, to determine both direction and velocity of blood flow. Ultrasound (Sonography) echocardiogram Echocardiogram Transposition of the Great Vessels confirms the diagnosis.
    • Findings: 
      • Pulmonary valve Pulmonary valve A valve situated at the entrance to the pulmonary trunk from the right ventricle. Heart: Anatomy thickening, doming of the valves
      • RVH, narrowed RV outflow tract
      • Post-stenotic dilation of the PA
      • Associated cardiac defects
      • Increased transvalvular gradient Transvalvular gradient Tricuspid Stenosis between the RV and the PA
    • Grading Grading Methods which attempt to express in replicable terms the level of cell differentiation in neoplasms as increasing anaplasia correlates with the aggressiveness of the neoplasm. Grading, Staging, and Metastasis:
      • Mild PS PS Invasive Mechanical Ventilation: peak Doppler Doppler Ultrasonography applying the doppler effect, with frequency-shifted ultrasound reflections produced by moving targets (usually red blood cells) in the bloodstream along the ultrasound axis in direct proportion to the velocity of movement of the targets, to determine both direction and velocity of blood flow. Ultrasound (Sonography) gradient < 30 mm MM Multiple myeloma (MM) is a malignant condition of plasma cells (activated B lymphocytes) primarily seen in the elderly. Monoclonal proliferation of plasma cells results in cytokine-driven osteoclastic activity and excessive secretion of IgG antibodies. Multiple Myeloma Hg
      • Moderate PS PS Invasive Mechanical Ventilation: peak Doppler Doppler Ultrasonography applying the doppler effect, with frequency-shifted ultrasound reflections produced by moving targets (usually red blood cells) in the bloodstream along the ultrasound axis in direct proportion to the velocity of movement of the targets, to determine both direction and velocity of blood flow. Ultrasound (Sonography) gradient of 30–50 mm MM Multiple myeloma (MM) is a malignant condition of plasma cells (activated B lymphocytes) primarily seen in the elderly. Monoclonal proliferation of plasma cells results in cytokine-driven osteoclastic activity and excessive secretion of IgG antibodies. Multiple Myeloma Hg
      • Severe PS PS Invasive Mechanical Ventilation: peak Doppler Doppler Ultrasonography applying the doppler effect, with frequency-shifted ultrasound reflections produced by moving targets (usually red blood cells) in the bloodstream along the ultrasound axis in direct proportion to the velocity of movement of the targets, to determine both direction and velocity of blood flow. Ultrasound (Sonography) gradient across the pulmonary valve Pulmonary valve A valve situated at the entrance to the pulmonary trunk from the right ventricle. Heart: Anatomy of > 50 mm MM Multiple myeloma (MM) is a malignant condition of plasma cells (activated B lymphocytes) primarily seen in the elderly. Monoclonal proliferation of plasma cells results in cytokine-driven osteoclastic activity and excessive secretion of IgG antibodies. Multiple Myeloma Hg
  • Transesophageal echocardiography Transesophageal echocardiography Ultrasonic recording of the size, motion, and composition of the heart and surrounding tissues using a transducer placed in the esophagus. Imaging of the Heart and Great Vessels ( TEE TEE Ultrasonic recording of the size, motion, and composition of the heart and surrounding tissues using a transducer placed in the esophagus. Imaging of the Heart and Great Vessels):
  • Electrocardiogram Electrocardiogram An electrocardiogram (ECG) is a graphic representation of the electrical activity of the heart plotted against time. Adhesive electrodes are affixed to the skin surface allowing measurement of cardiac impulses from many angles. The ECG provides 3-dimensional information about the conduction system of the heart, the myocardium, and other cardiac structures. Electrocardiogram (ECG) ( ECG ECG An electrocardiogram (ECG) is a graphic representation of the electrical activity of the heart plotted against time. Adhesive electrodes are affixed to the skin surface allowing measurement of cardiac impulses from many angles. The ECG provides 3-dimensional information about the conduction system of the heart, the myocardium, and other cardiac structures. Electrocardiogram (ECG)): 
    • Right axis deviation, RVH, right atrial enlargement
    • Right bundle branch block Bundle branch block A form of heart block in which the electrical stimulation of heart ventricles is interrupted at either one of the branches of bundle of His thus preventing the simultaneous depolarization of the two ventricles. Bundle Branch and Fascicular Blocks
  • Chest X-ray Chest X-ray X-ray visualization of the chest and organs of the thoracic cavity. It is not restricted to visualization of the lungs. Pulmonary Function Tests
    • Frontal Frontal The bone that forms the frontal aspect of the skull. Its flat part forms the forehead, articulating inferiorly with the nasal bone and the cheek bone on each side of the face. Skull: Anatomy view: 
      • Prominent PA from post-stenotic dilation
      • Lifting of the cardiac apex off the diaphragm Diaphragm The diaphragm is a large, dome-shaped muscle that separates the thoracic cavity from the abdominal cavity. The diaphragm consists of muscle fibers and a large central tendon, which is divided into right and left parts. As the primary muscle of inspiration, the diaphragm contributes 75% of the total inspiratory muscle force. Diaphragm: Anatomy
      • Prominent right heart border Right Heart Border Imaging of the Heart and Great Vessels from RA enlargement
    • Lateral view: filling of the retrosternal space by the enlarged RV
  • Cardiac magnetic resonance Cardiac magnetic resonance Aortic Regurgitation imaging:
    • An option if TTE TTE Imaging of the Heart and Great Vessels is suboptimal
    • Evaluates severity of the valve abnormalities and branch PA flow Flow Blood flows through the heart, arteries, capillaries, and veins in a closed, continuous circuit. Flow is the movement of volume per unit of time. Flow is affected by the pressure gradient and the resistance fluid encounters between 2 points. Vascular resistance is the opposition to flow, which is caused primarily by blood friction against vessel walls. Vascular Resistance, Flow, and Mean Arterial Pressure
    • Delineates anatomy of the PA and right heart
  • Cardiac catheterization Cardiac Catheterization Procedures in which placement of cardiac catheters is performed for therapeutic or diagnostic procedures. Cardiac Surgery and pulmonary angiography Angiography Radiography of blood vessels after injection of a contrast medium. Cardiac Surgery:

Management

  • Monitoring:
    • Asymptomatic, with peak Doppler Doppler Ultrasonography applying the doppler effect, with frequency-shifted ultrasound reflections produced by moving targets (usually red blood cells) in the bloodstream along the ultrasound axis in direct proportion to the velocity of movement of the targets, to determine both direction and velocity of blood flow. Ultrasound (Sonography) gradient < 30 mm MM Multiple myeloma (MM) is a malignant condition of plasma cells (activated B lymphocytes) primarily seen in the elderly. Monoclonal proliferation of plasma cells results in cytokine-driven osteoclastic activity and excessive secretion of IgG antibodies. Multiple Myeloma Hg: physical examination, Doppler Doppler Ultrasonography applying the doppler effect, with frequency-shifted ultrasound reflections produced by moving targets (usually red blood cells) in the bloodstream along the ultrasound axis in direct proportion to the velocity of movement of the targets, to determine both direction and velocity of blood flow. Ultrasound (Sonography) echocardiography Echocardiography Ultrasonic recording of the size, motion, and composition of the heart and surrounding tissues. The standard approach is transthoracic. Tricuspid Valve Atresia (TVA), ECG ECG An electrocardiogram (ECG) is a graphic representation of the electrical activity of the heart plotted against time. Adhesive electrodes are affixed to the skin surface allowing measurement of cardiac impulses from many angles. The ECG provides 3-dimensional information about the conduction system of the heart, the myocardium, and other cardiac structures. Electrocardiogram (ECG) every 5 years 
    • Asymptomatic, with peak Doppler Doppler Ultrasonography applying the doppler effect, with frequency-shifted ultrasound reflections produced by moving targets (usually red blood cells) in the bloodstream along the ultrasound axis in direct proportion to the velocity of movement of the targets, to determine both direction and velocity of blood flow. Ultrasound (Sonography) gradient > 30 mm MM Multiple myeloma (MM) is a malignant condition of plasma cells (activated B lymphocytes) primarily seen in the elderly. Monoclonal proliferation of plasma cells results in cytokine-driven osteoclastic activity and excessive secretion of IgG antibodies. Multiple Myeloma Hg: Doppler Doppler Ultrasonography applying the doppler effect, with frequency-shifted ultrasound reflections produced by moving targets (usually red blood cells) in the bloodstream along the ultrasound axis in direct proportion to the velocity of movement of the targets, to determine both direction and velocity of blood flow. Ultrasound (Sonography) echocardiography Echocardiography Ultrasonic recording of the size, motion, and composition of the heart and surrounding tissues. The standard approach is transthoracic. Tricuspid Valve Atresia (TVA) every 2–5 years
  • Diuretics Diuretics Agents that promote the excretion of urine through their effects on kidney function. Heart Failure and Angina Medication: for symptoms of RV overload
  • Percutaneous balloon valvotomy Balloon valvotomy Percutaneous procedure to separate and stretch the valve leaflets to increase the valve opening. Tricuspid Stenosis:
    • Symptomatic patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship with a domed valve and a peak gradient > 50 mm MM Multiple myeloma (MM) is a malignant condition of plasma cells (activated B lymphocytes) primarily seen in the elderly. Monoclonal proliferation of plasma cells results in cytokine-driven osteoclastic activity and excessive secretion of IgG antibodies. Multiple Myeloma Hg (or a mean Mean Mean is the sum of all measurements in a data set divided by the number of measurements in that data set. Measures of Central Tendency and Dispersion gradient > 30 mm MM Multiple myeloma (MM) is a malignant condition of plasma cells (activated B lymphocytes) primarily seen in the elderly. Monoclonal proliferation of plasma cells results in cytokine-driven osteoclastic activity and excessive secretion of IgG antibodies. Multiple Myeloma Hg)
    • Asymptomatic patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship with a peak gradient > 60 mm MM Multiple myeloma (MM) is a malignant condition of plasma cells (activated B lymphocytes) primarily seen in the elderly. Monoclonal proliferation of plasma cells results in cytokine-driven osteoclastic activity and excessive secretion of IgG antibodies. Multiple Myeloma Hg (or a mean Mean Mean is the sum of all measurements in a data set divided by the number of measurements in that data set. Measures of Central Tendency and Dispersion gradient > 40 mm MM Multiple myeloma (MM) is a malignant condition of plasma cells (activated B lymphocytes) primarily seen in the elderly. Monoclonal proliferation of plasma cells results in cytokine-driven osteoclastic activity and excessive secretion of IgG antibodies. Multiple Myeloma Hg)
  • Surgical valvotomy for severe PS PS Invasive Mechanical Ventilation associated with:
    • Hypoplastic pulmonary annulus
    • Severe pulmonary regurgitation Pulmonary regurgitation Backflow of blood from the pulmonary artery into the right ventricle due to imperfect closure of the pulmonary valve. Pulmonary Regurgitation
    • Subvalvular PS PS Invasive Mechanical Ventilation
    • Most dysplastic valves
    • Severe tricuspid regurgitation Tricuspid regurgitation Tricuspid regurgitation (TR) is a valvular defect that allows backflow of blood from the right ventricle to the right atrium during systole. Tricuspid regurgitation can develop through a number of cardiac conditions that cause dilation of the right ventricle and tricuspid annulus. A blowing holosystolic murmur is best heard at the left lower sternal border. Tricuspid Regurgitation
    • Surgical cardiac condition requiring operative intervention

Clinical Relevance

  • TOF: cyanotic congenital Congenital Chorioretinitis heart disease characterized by the tetrad of RVH, an overriding aorta Aorta The main trunk of the systemic arteries. Mediastinum and Great Vessels: Anatomy, pulmonary valve Pulmonary valve A valve situated at the entrance to the pulmonary trunk from the right ventricle. Heart: Anatomy stenosis Stenosis Hypoplastic Left Heart Syndrome (HLHS), and a ventricular septal defect Ventricular Septal Defect Tetralogy of Fallot. Surgically corrected TOF is a common cause for subsequent pulmonary regurgitation Pulmonary regurgitation Backflow of blood from the pulmonary artery into the right ventricle due to imperfect closure of the pulmonary valve. Pulmonary Regurgitation.
  • Pulmonary atresia Atresia Hypoplastic Left Heart Syndrome (HLHS): a rare congenital Congenital Chorioretinitis cyanotic heart disease characterized by the failed formation of the pulmonary valve Pulmonary valve A valve situated at the entrance to the pulmonary trunk from the right ventricle. Heart: Anatomy, leading to subsequent RV hypoplasia Hypoplasia Hypoplastic Left Heart Syndrome (HLHS). Pulmonary atresia Atresia Hypoplastic Left Heart Syndrome (HLHS) may occur as part of the most severe form of TOF (with VSD), where the entire RV outflow enters the aorta Aorta The main trunk of the systemic arteries. Mediastinum and Great Vessels: Anatomy.
  • Carcinoid syndrome Carcinoid syndrome A symptom complex associated with carcinoid tumor and characterized by attacks of severe flushing of the skin, diarrheal watery stools, bronchoconstriction, sudden drops in blood pressure, edema, and ascites. The carcinoid tumors are usually located in the gastrointestinal tract and metastasize to the liver. Symptoms are caused by tumor secretion of serotonin, prostaglandins, and other biologically active substances. Cardiac manifestations constitute carcinoid heart disease. Carcinoid Tumors and Syndrome: small, slow-growing neuroendocrine tumors Neuroendocrine tumors Tumors whose cells possess secretory granules and originate from the neuroectoderm, i.e., the cells of the ectoblast or epiblast that program the neuroendocrine system. Common properties across most neuroendocrine tumors include ectopic hormone production (often via apud cells), the presence of tumor-associated antigens, and isozyme composition. Gastrinoma. The tumors are associated with unregulated vasoactive hormone production, inducing the development of fibrous Fibrous Fibrocystic Change endocardial plaques and leading to pulmonary and tricuspid valve Tricuspid valve The valve consisting of three cusps situated between the right atrium and right ventricle of the heart. Heart: Anatomy abnormalities.
  • Rheumatic fever Fever Fever is defined as a measured body temperature of at least 38°C (100.4°F). Fever is caused by circulating endogenous and/or exogenous pyrogens that increase levels of prostaglandin E2 in the hypothalamus. Fever is commonly associated with chills, rigors, sweating, and flushing of the skin. Fever: a late complication of untreated streptococcal pharyngeal infection characterized by valvular abnormalities, most commonly of the left heart. While rare, the pulmonary valve Pulmonary valve A valve situated at the entrance to the pulmonary trunk from the right ventricle. Heart: Anatomy can be affected concurrently with the mitral valve Mitral valve The valve between the left atrium and left ventricle of the heart. Heart: Anatomy.

References

  1. Hoit, B.D. (2017). Tricuspid & pulmonic valve disease. Crawford M.H. (Ed.), Current, Diagnosis & Treatment: Cardiology, 5th ed. McGraw-Hill.
  2. Jone, P., Kim, J.S., Alvensleben, J., & Burkett, D. (2020). Cardiovascular diseases. In Hay Jr., W.W., Levin, M.J., Abzug, M.J., & Bunik, M (Eds.), Current Diagnosis & Treatment: Pediatrics, 25th ed. McGraw-Hill.
  3. Loewenthal, M., & O’Connor, R. (2016). Pulmonic valvular stenosis. Medscape. https://emedicine.medscape.com/article/759890-overview
  4. O’Gara P.T., & Loscalzo, J. (2018). Pulmonic valve disease. In Jameson, J., Fauci, A.S., Kasper, D.L., Hauser, S.L., Longo, D.L., & Loscalzo, J. (Eds.) Harrison’s Principles of Internal Medicine, 20th ed. McGraw-Hill.
  5. Stout, K., Connolly, H., & Yeon, S. (2018). Natural history and treatment of pulmonic stenosis in adults. UpToDate. Retrieved from Nov 3, 2020, from https://www.uptodate.com/contents/natural-history-and-treatment-of-pulmonic-stenosis-in-adults
  6. Sundjaja, J., & Bordoni, B. (2019) Anatomy, Thorax, Heart, Pulmonic valve. https://www.ncbi.nlm.nih.gov/books/NBK547706/
  7. Ren, X., & Lange, R. (2017) Pulmonic Stenosis. Medscape. https://emedicine.medscape.com/article/157737-overview

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