Hypoplastic left heart syndrome: Difference between revisions
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==Background== | ==Background== | ||
* A [[congenital heart disease|congenital heart defect]] in which the left heart is severely underdeveloped | *A [[congenital heart disease|congenital heart defect]] in which the left heart is severely underdeveloped | ||
*Accounts for 2 to 3 percent of all [[congenital heart disease]] <ref>Gordon BM. Decreasing number of deaths of infants with hypoplastic left heart syndrome. J Pediatr. 2008;153(3):354-8.</ref> | *Accounts for 2 to 3 percent of all [[congenital heart disease]] <ref>Gordon BM. Decreasing number of deaths of infants with hypoplastic left heart syndrome. J Pediatr. 2008;153(3):354-8.</ref> | ||
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===Physiology=== | ===Physiology=== | ||
*With a diminutive LV, the RV must perfuse both pulmonary and systemic circulations | *With a diminutive LV, the RV must perfuse both pulmonary and systemic circulations | ||
* Survival is dependent on: | *Survival is dependent on: | ||
**[[PDA]] (for systemic perfusion from RV to the aorta) | **[[PDA]] (for systemic perfusion from RV to the aorta) | ||
**Nonrestrictive [[ASD]] to ensure adequate mixing of oxygenated and deoxygenated blood | **Nonrestrictive [[ASD]] to ensure adequate mixing of oxygenated and deoxygenated blood | ||
Revision as of 01:12, 24 July 2017
Background
- A congenital heart defect in which the left heart is severely underdeveloped
- Accounts for 2 to 3 percent of all congenital heart disease [1]
Physiology
- With a diminutive LV, the RV must perfuse both pulmonary and systemic circulations
- Survival is dependent on:
Clinical Features
- Asymptomatic at birth because of adequate systemic perfusion through a PDA and initially high pulmonary vascular resistance
- As the PDA begins to close and pulmonary vascular resistance decreases, may develop hypotension, acidosis, and respiratory distress
- Symptoms can rapidly progress from cyanosis, increased respiratory distress, and poor feeding to heart failure and cardiogenic shock
Differential Diagnosis
Congenital Heart Disease Types
- Cyanotic
- Acyanotic
- AV canal defect
- Atrial septal defect (ASD)
- Ventricular septal defect (VSD)
- Cor triatriatum
- Patent ductus arteriosus (PDA)
- Pulmonary/aortic stenosis
- Coarctation of the aorta
- Differentiation by pulmonary vascularity on CXR[2]
- Increased pulmonary vascularity
- Decreased pulmonary vascularity
- Tetralogy of fallot
- Rare heart diseases with pulmonic stenosis
Evaluation
- Echocardiography
- Chest x-ray
- Cardiomegaly, increased pulmonary vasculature
- ECG
- Right axis deviation, RV hypertrophy
Management
- Stabilize cardiopulmonary function prior to surgery
- Maintain PDA to provide sufficient mixing of oxygenated and deoxygenated blood, and adequate systemic perfusion
- Prostaglandin E1
- Start infusion at 0.05 mcg/kg/min IV and titrate up to 0.1 mcg/kg/min, monitoring for hypotension and apnea
- Side Effects: Hypotension, Bradycardia, Seizures and Apnea
- Prostaglandin E1
- Staged surgical repair
- First stage (Norwood procedure) performed in neonates
- Second stage (bidirectional Glenn procedure) performed at 3-6 months
- Third stage (Fontan procedure) performed at 18-30 months
Disposition
- Admit
See Also
External Links
References
- ↑ Gordon BM. Decreasing number of deaths of infants with hypoplastic left heart syndrome. J Pediatr. 2008;153(3):354-8.
- ↑ Knipe K et al. Cyanotic congenital heart diseases. Radiopaedia. http://radiopaedia.org/articles/cyanotic-congenital-heart-disease