Hypoplastic left heart syndrome: Difference between revisions
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==Background== | |||
*A [[congenital heart disease|congenital heart defect]] in which the left heart is severely underdeveloped | |||
*Accounts for 2 to 3% 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> | |||
[[File:Hlhs.jpeg|thumb]] | [[File:Hlhs.jpeg|thumb]] | ||
===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 | ||
==Clinical Features== | ==Clinical Features== | ||
[[File:HLHS.jpg|thumb|Infant with cyanosis due to hypoplastic left heart syndrome.]] | |||
*Asymptomatic at birth because of adequate systemic perfusion through a PDA and initially high pulmonary vascular resistance | *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 | *As the PDA begins to close and pulmonary vascular resistance decreases, may develop: | ||
*Symptoms can rapidly progress from cyanosis, increased respiratory distress, and poor feeding to heart failure and cardiogenic shock | **[[Pediatric shock|Hypotension]] | ||
**[[Acidosis]] | |||
**[[shortness of breath (peds)|Respiratory distress]] | |||
*Symptoms can rapidly progress from cyanosis, increased respiratory distress, and poor feeding to [[heart failure]] and [[cardiogenic shock]] | |||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
| Line 19: | Line 24: | ||
==Evaluation== | ==Evaluation== | ||
* | *[[ECG]] | ||
**Right axis deviation, RV hypertrophy | |||
*[[Chest x-ray]] | *[[Chest x-ray]] | ||
**Cardiomegaly, increased pulmonary vasculature | **Cardiomegaly, increased pulmonary vasculature | ||
*[[ | *[[Echocardiography]] | ||
==Management== | ==Management== | ||
| Line 33: | Line 38: | ||
*Staged surgical repair | *Staged surgical repair | ||
**First stage (Norwood procedure) performed in neonates | **First stage (Norwood procedure) performed in neonates | ||
***SpO2 ranges in mid 70s-80s | |||
**Second stage (bidirectional Glenn procedure) performed at 3-6 months | **Second stage (bidirectional Glenn procedure) performed at 3-6 months | ||
***SpO2 ranges in mid 70s-80s | |||
**Third stage (Fontan procedure) performed at 18-30 months | **Third stage (Fontan procedure) performed at 18-30 months | ||
***Preload dependent with SpO2 ranging from low to high 90s | |||
==Disposition== | ==Disposition== | ||
Latest revision as of 00:51, 15 September 2019
Background
- A congenital heart defect in which the left heart is severely underdeveloped
- Accounts for 2 to 3% 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:
- 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
- ECG
- Right axis deviation, RV hypertrophy
- Chest x-ray
- Cardiomegaly, increased pulmonary vasculature
- Echocardiography
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
- SpO2 ranges in mid 70s-80s
- Second stage (bidirectional Glenn procedure) performed at 3-6 months
- SpO2 ranges in mid 70s-80s
- Third stage (Fontan procedure) performed at 18-30 months
- Preload dependent with SpO2 ranging from low to high 90s
- First stage (Norwood procedure) performed in neonates
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