Overview
Pulmonary valve malformation causes narrowing of the pulmonary artery opening and obstruction of blood flow in mild asymptomatic disease, or with shortness of breath after activity, chest tightness, fainting, fatigue, and poor physical strength congenital valvular developmental anomalies are the main cause of the main cause of interventional and surgical treatments
Definition
Pulmonary valve stenosis is a rare congenital heart disease in which congenital developmental malformation of the pulmonary valve leads to narrowing of the pulmonary artery opening and obstruction of blood flow, and it accounts for about 8% to 10% of all congenital heart diseases.
The main manifestations are shortness of breath after activity, palpitation, chest tightness, easy fatigue, poor physical strength.
Mild stenosis or asymptomatic people can be followed up regularly. Moderate and severe stenosis is cured by interventional therapy or surgery, and the prognosis is generally better.
Classification
Typical pulmonary stenosis
Typical pulmonary valve stenosis is characterized by fusion of the leaflet junctions with each other, restricting valve opening and narrowing the orifice.
The leaflets are structurally intact, the annulus is normal, and the pulmonary trunk is dilated after stenosis.
Dysplastic pulmonary stenosis
The pulmonary valve leaflets are irregularly shaped and markedly thickened or nodular, with no interleaflet adhesions, inflexible opening and closing of the leaflets, dysplastic annulus, and an undilated or hypoplastic pulmonary trunk.
There is a family history of dysplastic pulmonary stenosis, and most cases of Noonan syndrome are associated with this lesion.
Morbidity
Pulmonary stenosis is highly prevalent, up to 25% of adult congenital heart disease [1].
The incidence of pulmonary valve stenosis in Chinese live births is 0.73 per 1,000 live births [2].
Etiology
Pathogenesis
The etiology of pulmonary valve stenosis is unknown is unclear.
Fetuses have a higher incidence of pulmonary stenosis if the mother had rubella virus infection in the first trimester of pregnancy.
In addition, patients with Noonan syndrome, trisomy 18, multiple freckles (Leopard syndrome), Watson syndrome, neurofibromas, and a number of other chromosomal aberrations and genetic syndromes often have pulmonary stenosis.
Pathogenesis
Pulmonary stenosis occurs as a result of congenital developmental abnormalities that result in interleaflet fusion, irregular leaflet morphology, and leaflet thickening.
This lesion, which obstructs blood flow from the right ventricle to the pulmonary vascular bed, increases right ventricular pressure, compensatory hypertrophy of the right ventricle, and eventual enlargement of the right heart leading to right heart failure [3].
Symptoms.
Symptoms of pulmonary stenosis depend mainly on the degree of stenosis and the degree of secondary right heart hypertrophy; patients with mild or even moderate stenosis may be asymptomatic for a long time.
Major Symptoms
Cyanosis, dyspnea, and tachypnea are typical of neonates presenting with severe pulmonary valve stenosis, and are exacerbated if arterial ductus arteriosus occurs.
Most patients with mild pulmonary stenosis have no obvious symptoms, and most often a heart murmur is detected on physical examination.
Patients with moderate stenosis may experience shortness of breath after activity, palpitations, chest tightness, easy fatigue, and poor stamina with age.
Patients with severe stenosis may have peripheral cyanosis, and patients often have chest pain, fainting or even sudden death due to strenuous activities.
Complications
Right heart failure
Jugular vein filling, hepatomegaly, lower limb edema, abdominal distension, nausea, vomiting, and loss of appetite may occur.
Infective endocarditis
Patients may have symptoms of fever, chest tightness, petechiae and hemorrhagic spots on the skin [4-5].
Consultation
Department of Medicine
Cardiovascular Medicine
Patients with heart murmurs found during physical examination or doctor’s visit, as well as those with symptoms such as shortness of breath after activity, chest tightness, palpitations, and fainting, need to be taken seriously and go to the Department of Cardiovascular Medicine for diagnosis and treatment.
Cardiovascular Surgery
If cardiovascular medicine diagnoses pulmonary stenosis and recommends surgical treatment, the cardiovascular surgery department should evaluate the indications for surgery.
Preparation
Consultation: Registration, Documentation, Frequently Asked Questions
Tips
Avoid over-exertion, emotional stress, and unauthorized use of medication that may affect the test results.
Preparation Checklist
Symptom list
Pay particular attention to the time of onset of symptoms and special manifestations.
Is there any physical discomfort such as shortness of breath, chest tightness, palpitations, etc. after activity?
When did the symptoms appear and how long did they last?
Are there factors that aggravate or relieve the symptoms?
Medical history checklist
Is there a past history of growth failure?
Is there a family history of congenital heart disease?
Was there any comorbidity and medication use during the mother’s pregnancy?
Was the baby born prematurely?
Checklist
Examination results in the last six months, which can be brought to the doctor’s office.
Imaging and other ancillary tests: chest X-ray, electrocardiogram, echocardiogram, right heart catheterization, cardiac magnetic resonance, and other results, etc.
Diagnosis
Diagnosis is based on
Medical history
There may be a family history of congenital heart disease.
There may be a history of genetic disease.
Clinical symptoms
Symptoms
Most patients with mild pulmonary stenosis have no obvious clinical symptoms. Those with moderate stenosis may have shortness of breath after activity, palpitations, chest tightness, fatigue, and poor energy. Patients with severe stenosis may have abdominal distension, lack of appetite, nausea, vomiting and other common manifestations of right heart failure, or even fainting and sudden death.
Physical signs
Grade III-IV loud and rough jet-blowing systolic murmur can be heard at the second rib of the left sternal border, which is transmitted to the left neck or left subclavian region, and systolic tremor can be detected at the loudest point of the murmur, as well as weakening and splitting of the second heart sound of the pulmonary valve area.
In severe stenosis, a pronounced lifting-like beat is seen in the precordial region.
There is bilateral lower extremity edema, jugular venous filling, positive hepatic jugular venous regurgitation, and hepatomegaly.
Electrocardiogram
It is used to check for arrhythmias and to assess right heart load.
The electrocardiogram may be normal in patients with mild stenosis, but in patients with severe stenosis, increased right heart load may be manifested as rightward deviation of the electrical axis, right ventricular hypertrophy, enlargement of the right atrium, and incomplete right bundle branch block may also be seen.
Imaging
Chest X-ray
It can show the size, shape, position and contour of the heart and the surrounding blood vessels.
It can determine the severity of the disease. In mild and moderate stenosis, the size of the heart shadow may be normal, while in severe stenosis, the right ventricle is enlarged and the main pulmonary artery is widened in most patients.
Echocardiography
Observation of the heart, valve anatomy, and preliminary estimation of the trans-pulmonary valve pressure difference.
It is highly accurate in the diagnosis of pulmonary valve stenosis and can clarify the site and degree of stenosis. Patients with pulmonary valve stenosis can see thickening of the pulmonary valve, enhanced echogenicity or restricted opening, accelerated perivalvular blood flow, and dilatation of the pulmonary arteries.
Right heart catheterization
It provides insight into the hemodynamic changes in the heart and shows the location and severity of the stenosis.
Right heart catheterization is the “gold standard” for diagnosing pulmonary stenosis, determining indications for surgery and assessing cardiac function.
Cardiac MRI
It shows the anatomy of the right ventricular outflow tract, pulmonary artery and its branches, and assesses ventricular volume.
It can clarify the exact location of stenosis and estimate the flow rate of pulmonary valve regurgitation, which is of great significance to the staging of pulmonary valve stenosis and the evaluation of its condition.
The test is contraindicated in patients with implanted pacemakers, pregnant women, and patients with metallic substances in the tissues surrounding the heart [6-7].
Differential diagnosis
Atrial septal defect, ventricular septal defect
Similarity: In mild cases, there may be no obvious symptoms or mild symptoms, and there may be symptoms such as fatigue, shortness of breath after activity, chest tightness, etc., and heart murmur can be heard on auscultation.
Differences: Distinction is mainly based on echocardiography, which may show a blood shunt between the right and left atria or ventricles.
Tetralogy of Fallot
Similarities: Anatomically, both have narrowing of the pulmonary arteries, resulting in obstruction of the right ventricular outflow tract.
Differences: Cyanosis, squatting, growth retardation, ventricular septal defect and aortic dissection on echocardiography and right heart catheterization are common symptoms.
Treatment
Aim of treatment: to relieve right ventricular outflow tract obstruction, reduce right heart pressure load and prevent heart failure.
Treatment principle: interventional and surgical treatments are the mainstay. Mild stenosis does not require surgery. Those with moderate stenosis or above, obvious clinical symptoms, electrocardiogram suggesting right ventricular hypertrophy, and pressure step difference between right ventricle and pulmonary artery >50mmHg should be operated on an elective basis. Severe stenosis with syncope or secondary right ventricular outflow tract stenosis should be operated as early as possible.
Interventional therapy
Percutaneous balloon pulmonary valvuloplasty is the treatment of choice for pulmonary stenosis.
The results are comparable to those of surgery, without the need for a thoracotomy and with fewer complications.
Cautions: some patients with unsatisfactory dilatation may have complications such as pulmonary valve closure insufficiency and tricuspid valve damage, and still require interventional therapy of valve replacement or undergo surgical treatment [8].
Surgical treatment
The mainstay is pulmonary valve junction dissection.
Surgical treatment should be adopted for those who have unsuccessful balloon dilatation or those who are not suitable for balloon dilatation, such as the combination of funnel stenosis and severe tricuspid regurgitation.
Postoperative cardiotonic drugs and moderate activity are required as prescribed. Postoperative immunity is low, avoid getting cold and flu. Regular checkups should be performed to detect complications such as perivalvular leakage. Postoperative echocardiography should be reviewed regularly to monitor cardiac function [9-10].
Prognosis
Cure
Patients who are not treated with interventional or surgical procedures generally survive into adulthood. Mild stenosis or asymptomatic patients can be followed up regularly.
Severe stenosis of the pulmonary valve, if left untreated, can lead to right heart failure and death.
Symptoms of pulmonary stenosis may be reduced or disappear after intervention or surgery, and long-term survival after surgery is similar to that of the normal population.
Hazards
Severe stenosis carries the risk of heart failure and sudden death.
Daily
Daily Management
Dietary management
Regular diet and attention to nutrition.
Promote a diet high in protein, low in animal fat and high in vitamins.
Life Management
Take rest and get enough sleep.
Strenuous exercise is not recommended.
Quit smoking and drinking.
Disease monitoring
If new symptoms appear or existing symptoms such as panic, chest tightness and shortness of breath worsen, you need to go to the hospital.
After the operation, follow up regularly according to the doctor’s requirements, and conduct echocardiography regularly.
Prevention
The cause of pulmonary stenosis is not known and there is no effective preventive measure for the time being.
Mothers should avoid viral infections in the early stages of pregnancy and undergo regular labor and delivery examinations.
Regular medical checkups should be performed and timely treatment should be provided when the disease is detected.