Congenital Pulmonary Venous Hypertension
Introduction
Congenital Pulmonary Venous Hypertension (CPVH) is a rare but severe condition characterized by abnormally elevated pressure in the pulmonary venous system, typically resulting from developmental abnormalities of the pulmonary veins or left atrium.
Key Pathophysiological Features
- Obstruction to pulmonary venous drainage leads to increased pulmonary venous pressure
- Progressive development of pulmonary arterial hypertension
- Right ventricular hypertrophy and eventual failure
- Associated with conditions like Total Anomalous Pulmonary Venous Return (TAPVR) with obstruction
Anatomical Classifications
- Localized Stenosis: Individual pulmonary vein narrowing
- Diffuse Hypoplasia: Multiple vein involvement
- External Compression: Due to surrounding cardiac structures
- Cor Triatriatum: Membrane division of left atrium
Clinical Presentation
Early Infancy Signs
- Tachypnea and respiratory distress
- Poor feeding and failure to thrive
- Recurrent pulmonary infections
- Cyanosis may be present
Physical Examination
- Cardiac Examination:
- Loud P2 component of second heart sound
- Right ventricular heave
- Systolic murmur of tricuspid regurgitation
- Respiratory Examination:
- Tachypnea with intercostal retractions
- Fine rales on auscultation
- Possible wheeze
Progressive Symptoms
- Development of right heart failure
- Hepatomegaly and peripheral edema
- Exercise intolerance in older children
- Hemoptysis in severe cases
Diagnostic Approach
Initial Evaluation
- Chest X-ray Findings:
- Pulmonary venous congestion
- Interstitial edema
- Cardiomegaly with right heart enlargement
- ECG Changes:
- Right axis deviation
- Right ventricular hypertrophy
- Right atrial enlargement
Advanced Imaging
- Echocardiography:
- Evaluation of pulmonary venous anatomy
- Assessment of right ventricular function
- Estimation of pulmonary pressures
- Detection of associated cardiac anomalies
- CT/MR Angiography:
- Detailed pulmonary venous anatomy
- Course and caliber of veins
- Identification of stenotic segments
- Cardiac Catheterization:
- Direct pressure measurements
- Pulmonary vein gradients
- Pulmonary vascular resistance
Management Strategies
Medical Management
- Initial Stabilization:
- Diuretics for congestion control
- Oxygen therapy as needed
- Management of right heart failure
- Pulmonary Hypertension Therapy:
- Phosphodiesterase inhibitors
- Endothelin receptor antagonists
- Prostacyclin analogues in severe cases
Surgical Intervention
- Surgical Options:
- Sutureless repair techniques
- Pulmonary vein stenting
- Atrial septectomy when needed
- Timing Considerations:
- Early intervention preferred
- Risk of rapid progression
- Higher success rates with earlier surgery
Long-term Follow-up
- Monitoring:
- Regular echocardiographic assessment
- Pulmonary function testing
- Growth and development tracking
- Complications Watch:
- Restenosis
- Progressive pulmonary hypertension
- Right heart failure
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.
