Pulmonary Valvular Insufficiency
Congenital Pulmonary Valvular Insufficiency
Pulmonary valvular insufficiency (PI) is a condition characterized by retrograde blood flow from the pulmonary artery into the right ventricle during diastole due to incomplete closure or malformation of the pulmonary valve leaflets.
Key Points
- Isolated congenital PI is rare, occurring in approximately 0.1% of the general population
- More commonly associated with other cardiac anomalies or post-surgical states
- Can be graded as mild, moderate, or severe based on regurgitant fraction
- Natural history varies significantly based on underlying etiology and associated conditions
Anatomical Considerations
- Primary valve abnormalities:
- Congenital absence or hypoplasia of valve leaflets
- Abnormal leaflet morphology
- Dysplastic valve tissue
- Annular dilation
Hemodynamic Effects
- Right ventricular volume overload
- Compensatory RV dilation and hypertrophy
- Eventual RV dysfunction if severe and chronic
- Potential right heart failure in advanced cases
Associated Conditions
- Tetralogy of Fallot (post-repair)
- Pulmonary hypertension
- Carcinoid heart disease
- Marfan syndrome
- Endocarditis sequelae
Symptoms
- Often asymptomatic in mild cases
- Exercise intolerance
- Right-sided heart failure symptoms:
- Peripheral edema
- Hepatomegaly
- Ascites
- Fatigue
- Palpitations
- Syncope in severe cases
Physical Examination
- Characteristic early diastolic murmur:
- High-pitched, decrescendo
- Best heard at left upper sternal border
- Increases with inspiration (Carvallo's sign)
- Right ventricular heave
- Elevated jugular venous pressure
- Hepatojugular reflux
- Peripheral edema in advanced cases
Diagnostic Approach
Echocardiography
- Primary diagnostic tool
- Evaluates:
- Valve morphology and motion
- Regurgitant jet characteristics
- RV size and function
- Associated anomalies
- Quantitative measures:
- Regurgitant fraction
- Effective regurgitant orifice area
- RV volumes
Cardiac MRI
- Gold standard for RV assessment
- Provides:
- Accurate RV volumes
- Ejection fraction
- Regurgitant fraction
- Flow dynamics
Other Studies
- ECG: Right ventricular hypertrophy, right bundle branch block
- Chest X-ray: RV enlargement, pulmonary artery dilation
- Exercise testing: Functional capacity assessment
Treatment Approaches
Medical Management
- Regular monitoring in mild cases
- Heart failure therapy when indicated:
- Diuretics
- ACE inhibitors
- Beta-blockers
- Rhythm management if needed
- Endocarditis prophylaxis in select cases
Surgical Intervention
- Indications:
- Severe PI with symptoms
- RV dysfunction
- Progressive RV dilation
- Associated lesions requiring repair
- Options:
- Pulmonary valve replacement
- Valve repair in selected cases
- Transcatheter valve replacement in suitable candidates
Long-term Outcomes
- Generally good with mild isolated PI
- Variable with associated conditions
- Factors affecting prognosis:
- Severity of regurgitation
- RV function
- Associated anomalies
- Timing of intervention
Follow-up
- Regular cardiac evaluation
- Serial imaging studies
- Exercise testing as needed
- Pregnancy counseling in females of childbearing age
Disclaimer
The notes provided on Pediatime are generated from online resources and AI sources and have been carefully checked for accuracy. However, these notes are not intended to replace standard textbooks. They are designed to serve as a quick review and revision tool for medical students and professionals, and to aid in theory exam preparation. For comprehensive learning, please refer to recommended textbooks and guidelines.
