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Pulmonic Stenosis - Causes, Complication, and Management

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Pulmonic stenosis is a valve abnormality that stiffens and restricts blood flow as low-air blood enters the lungs through the pulmonary artery, causing stenosis.

Medically reviewed by

Dr. Kaushal Bhavsar

Published At April 24, 2024
Reviewed AtApril 24, 2024

Introduction

Pulmonic stenosis is a defect of the pulmonic valve in which the valve becomes rigid, restricting the flow of blood through the valve. This condition is almost always present at birth, is not harmful, and is identified and treated in children. It could be cured. Pulmonic stenosis can also be found in adults, and it typically occurs in tandem with more severe forms of heart structural illness.

What Are the Clinical Manifestations Associated With Pulmonary Stenosis?

The manifestation of symptoms related to pulmonary stenosis depends upon the degree of severity of the stenotic condition. As the degree of stenosis in the pulmonary valve increases, the symptoms of this disorder grow more pronounced, and the physiological impact on the heart and body becomes more significant. A significant proportion of individuals afflicted with this particular illness experience just mild or no symptoms. Mild pulmonary stenosis typically exhibits minimal or no symptoms.

Typical manifestations of pulmonic stenosis encompass:

  • Chest Pain: Individuals may feel discomfort in the thoracic region and respiratory system.

  • Dyspnea: Breathing may be perceived as challenging, particularly in physical exertion.

  • Syncope: Individuals may experience a loss of awareness or sensations of dizziness.

  • Fatigue: Individuals may experience heightened susceptibility to exhaustion following physical exercise.

What Are the Causes of the Condition?

The etiology of pulmonary stenosis remains elusive, but it typically manifests prenatally due to aberrant development of the pulmonary valve within the intrauterine environment. This particular problem consists of congenital cardiac disease.

Individuals born with pulmonary valve stenosis require ongoing and continuous surveillance by a medical professional specializing in cardiology or adult congenital heart disease. While infrequent, people can acquire pulmonary stenosis later in life. Several risk factors are associated with non-congenital pulmonary stenosis.

  • Carcinoid syndrome is a pathological disorder resulting from the presence of carcinoid tumors within the gastrointestinal tract. These tumors secrete bioactive substances into the bloodstream, potentially leading to detrimental effects on cardiac valves.

  • Rheumatic fever, infrequent sequelae of streptococcal pharyngitis,

  • The administration of radiation therapy to the thoracic region, potentially as a consequence of cancer treatment near the thoracic area.

  • Rubella, also called German measles, has been identified as a medical ailment that may elevate the likelihood of developing pulmonary stenosis during pregnancy.

What Are the Potential Complications Associated With Pulmonary Valve Stenosis?

Individuals who are afflicted with heart valve disorders may experience health-related consequences beyond the potential manifestation of symptoms. The likelihood of experiencing consequences associated with pulmonary stenosis is elevated, particularly when the condition is not addressed or properly controlled. Individuals diagnosed with pulmonic stenosis may potentially have the following manifestations:

  • Endocarditis is a pathological condition characterized by inflammation of the endocardium, the inner lining of the heart chambers and valves. The etiology of this infection primarily involves bacterial pathogens, with fungal pathogens being infrequently implicated.

  • Ventricular hypertrophy is the pathological thickening of the right ventricle, resulting from the heart chamber's excessive workload in response to reduced blood supply.

  • Cardiac arrhythmia, also referred to as an irregular cardiac rhythm, arises due to aberrant electrical impulses governing the heart's contraction.

  • Pregnancy-related complications.

  • Heart failure, also known as congestive heart failure, is a medical condition characterized by the inability of the heart to pump blood adequately.

What Are the Management Strategies for Pulmonic Stenosis?

  • Patients with no symptoms and a peak Doppler gradient of less than 30 mm Hg may undergo follow-up assessments every five years using electrocardiography and Doppler echocardiography.

  • Patients who do not exhibit symptoms and have a peak Doppler gradient greater than 30 mm Hg may undergo follow-up examinations using Doppler echocardiography at intervals of two to five years.

  • Patients with no symptoms but a peak Doppler gradient greater than 60 mm Hg are advised to undergo balloon valvotomy.

  • Patients who exhibit symptoms and have a peak Doppler gradient greater than 50 mm Hg, together with a domed pulmonic valve, are advised to undergo balloon valvotomy.

  • Surgical intervention is typically advised in cases of severe valvular stenosis accompanied by severe pulmonary regurgitation, hypoplastic pulmonary annulus, subvalvular stenosis, or supravalvular stenosis. Regrettably, the efficacy of balloon valvotomy is comparatively lower in cases involving dysplastic valves than domed valves, hence rendering surgical intervention the preferable course of action. The performance of balloon valvotomy may be beneficial in cases where the peak Doppler gradient exceeds 60 mm Hg in asymptomatic individuals or surpasses 50 mm Hg in symptomatic individuals. The inclusion of surgery should be taken into account for individuals who are undergoing simultaneous cardiac surgical operations. Pulmonary artery balloon angioplasty (a medical procedure used to treat blockages in the pulmonary artery by using a balloon to widen the narrowed blood vessel) and the possibility of stent insertion represent a viable therapeutic approach for addressing both supravalvular and subvalvular pulmonic stenosis.

What Is the Prognosis of the Condition?

The majority of patients have a very favorable prognosis and will live normal lives, except those who develop severe stenosis during the newborn period.

  • Most patients who have surgical revision reach their maximum exercise capacity, and they only require reintervention after this stage of recovery. Re interventions are performed when there is severe pulmonary regurgitation, and if necessary, most supraventricular tachycardias (SVTs) (A supraventricular tachycardia is an elevated heart rate that originates above the two lower chambers of the heart. It occurs when the heart's typical electrical impulses are interrupted, resulting in an irregularly quick heartbeat.) disappear after the procedure.

  • Patients diagnosed with pulmonic stenosis administered with antibiotic treatment are only suggested in cases when a previous endocarditis infection (an inflammation of the interior lining of the valves and chambers of the heart that is potentially fatal) or a prosthetic valve installation has been documented.

  • Patients with moderate or severe gradients should avoid excessive exercise throughout pregnancy, especially during the second half. Finally, no activity constraints exist for athletes with minor stenosis and less than 50 mm Hg gradients.

Conclusion

After treatment, pulmonary stenosis can reoccur in some children with the condition. This can occur for various reasons, including scar tissue formation after a procedure or a valve replacement that does not expand as children develop. Therefore, some children may require multiple procedures to maintain the valve healthy.

Since pulmonary stenosis can be a lifelong condition, children with the defect must see a cardiologist regularly to ensure the narrowing is not worsening. Many children will not require specialized medical care, and those who do will typically be able to return to most of their regular activities after recovery. Before engaging in competitive sports or intense physical activity, children and adolescents with moderate or severe pulmonary stenosis should consult their cardiologist.

Dr. Kaushal Bhavsar
Dr. Kaushal Bhavsar

Pulmonology (Asthma Doctors)

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