CHD Flashcards
What are pulmonary vascular rings?
Congenital vascular conditions where the trachea and esophagus are encircled by vascular structures
Rare, occurring in only 1% of congenital heart disease (CHD) cases.
What is the most common type of vascular ring?
Double aortic arch
Illustrated in figures 39.2 and 39.3.
What is the second most common type of vascular ring?
Right aortic arch in association with an aberrant left subclavian artery
Illustrated in figures 39.4 and 39.5.
What is a pulmonary artery sling?
A condition where the left pulmonary artery connects directly to the right pulmonary artery instead of the pulmonary trunk
Not a true vascular ring; illustrated in figures 39.6 and 39.7.
What are common respiratory signs and symptoms of pulmonary vascular rings?
- Noisy breathing
- Barking cough
- Wheezing
- Frequent upper respiratory tract infections
- Apneic episodes
- Intermittent cyanosis
What gastrointestinal symptoms are associated with pulmonary vascular rings?
- Sensation of food being stuck in the throat
- Slow eating
- Hyperextension during feedings
At what age do double aortic arches typically present?
Earlier in life, typically within the first month
When do right aortic arch and aberrant left subclavian artery symptoms usually appear?
Between 1 and 6 months of age
What is the significance of a pulmonary artery sling with complete tracheal rings?
It creates further narrowing of the airway and is associated with significant respiratory symptoms with high morbidity and mortality
What diagnostic tools are used for vascular rings?
- Chest x-ray
- Barium swallow
- Echocardiography
- CT scan with contrast
- MRI
- Bronchoscopy
What does a chest x-ray help to suggest in the diagnosis of vascular rings?
The position of the aortic knob and apparent tracheal narrowing
What is the typical surgical treatment for double aortic arches?
Resection of the smaller nondominant arch, often through a thoracotomy
How is a right aortic arch with an aberrant left subclavian artery treated?
Division of the associated ligamentum arteriosum and possibly resection of Kommerell’s diverticulum
What surgical intervention may be required for pulmonary vascular slings?
Translocating the anomalous left pulmonary artery to the main pulmonary artery
What is a significant postoperative risk after congenital heart surgery?
Pulmonary hypertension
What are some risk factors for postoperative pulmonary hypertensive crises?
- Congenital heart disease
- Total anomalous pulmonary venous return
- Truncus arteriosus
- Transposition of the great arteries (TGA)
- Ventricular septal defect (VSD) with late repair
- Complete atrioventricular canal
- Hypoplastic left heart syndrome
What is a critical consequence of increased pulmonary arterial pressure?
Right ventricular dilation and dysfunction
What can be monitored to evaluate decreased pulmonary blood flow?
Increased gradient between arterial pCO2 from a blood gas and end-tidal CO2 from capnography
What are the three pharmacologic mechanisms of pulmonary vasodilation?
- Nitric oxide/cyclic guanosine monophosphate (cGMP) pathway
- Prostacyclin (PGI2)/cyclic adenosine monophosphate (cAMP) pathway
- Endothelin-1 pathway
What is a selective pulmonary vasodilator with a rapid onset of action?
Inhaled nitric oxide
What is the common presentation of pulmonary vein stenosis?
- Tachypnea
- Failure to thrive
- Repeated lower respiratory infections
What is the preferred imaging approach for pulmonary vein stenosis?
Cardiac catheterization and angiography
What are common factors contributing to impaired pulmonary function after cardiac surgery?
- Postoperative atelectasis
- Pulmonary edema
- Pleural effusions
- Alterations in chest wall mechanics due to sternotomy
- Impaired respiratory effort due to postoperative pain
- Hemidiaphragmatic paresis or paralysis
