Hypoplastic left heart syndrome - Revision history

Danbot: Add MedicationDose entry for PGE1

2026-03-20T21:21:21Z

Add MedicationDose entry for PGE1

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 ==Disposition==
 *Admit
 ==See Also==

2024-11-06T17:59:30Z

← Revisión anterior		Revisión del 17:59 6 nov 2024
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	==Background==		==Background==
			[[File:Hlhs.jpeg\|thumb\|Heart with hypoplastic left heart syndrome]]
	*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% 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% 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]]~~

	===Physiology===		===Physiology===

2019-09-15T00:51:09Z

← Revisión anterior		Revisión del 00:51 15 sep 2019
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	[[File:HLHS.jpg\|thumb\|Infant with cyanosis due to hypoplastic left heart syndrome.]]		[[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 ~~hypotension, acidosis, and respiratory~~ distress		*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==
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	*[[Chest x-ray]]		*[[Chest x-ray]]
	**Cardiomegaly, increased pulmonary vasculature		**Cardiomegaly, increased pulmonary vasculature
	*Echocardiography		*[[Echocardiography]]

	==Management==		==Management==

2019-07-03T05:33:33Z

Clinical Features

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	==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 hypotension, acidosis, and respiratory distress		*As the PDA begins to close and pulmonary vascular resistance decreases, may develop hypotension, acidosis, and respiratory distress

2019-07-03T05:31:25Z

Evaluation

← Revisión anterior		Revisión del 05:31 3 jul 2019
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	==Evaluation==		==Evaluation==
	*~~Echocardiography~~		*[[ECG]]
			**Right axis deviation, RV hypertrophy
	*[[Chest x-ray]]		*[[Chest x-ray]]
	**Cardiomegaly, increased pulmonary vasculature		**Cardiomegaly, increased pulmonary vasculature
	*~~[[ECG]]~~		*Echocardiography
	**Right axis deviation, RV hypertrophy

	==Management==		==Management==

2019-06-10T00:31:26Z

Management

← Revisión anterior		Revisión del 00:31 10 jun 2019
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	***SpO2 ranges in mid 70s-80s		***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 ~~rating~~ from low to high 90s		***Preload dependent with SpO2 ranging from low to high 90s

	==Disposition==		==Disposition==

2019-06-10T00:30:46Z

Management

← Revisión anterior		Revisión del 00:30 10 jun 2019
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	*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 rating from low to high 90s

	==Disposition==		==Disposition==

2017-11-10T20:47:19Z

Text replacement - " percent" to "%"

← Revisión anterior		Revisión del 20:47 10 nov 2017
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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% 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]]

2017-07-24T01:12:54Z

Text replacement - "* " to "*"

← Revisión anterior		Revisión del 01:12 24 jul 2017
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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

2017-06-27T17:37:34Z

Background

← Revisión anterior		Revisión del 17:37 27 jun 2017
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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 ~~CHD~~ <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>

	[[File:Hlhs.jpeg\|thumb]]		[[File:Hlhs.jpeg\|thumb]]