User:Mr. Ibrahem/Hypoplastic left heart syndrome
| Hypoplastic left heart syndrome | |
|---|---|
| Other names | Cyanotic heart disease - hypoplastic left heart[1] |
 | |
| Illustration of heart suffering from hypoplastic left heart syndrome | |
| Specialty | Cardiac surgery |
| Symptoms | Shortness of breath, bluish skin[2] |
| Usual onset | Present at birth[3] |
| Causes | Usually unknown[2] |
| Diagnostic method | Ultrasound[3] |
| Differential diagnosis | Coarctation of the aorta, aortic stenosis, Shone's syndrome[3] |
| Treatment | Prostaglandin E1, surgery, comfort care[3] |
| Prognosis | High risk of disability or death[3] |
| Frequency | 1 in 5,000 newborns[3] |
Hypoplastic left heart syndrome (HLHS) is a congenital heart defect in which the left side of the heart is severely underdeveloped.[3] It may affect the left ventricle, aorta, aortic valve, and mitral valve.[3] While the baby may appear normal in the first few days of life, they than rapidly develop trouble breathing.[2] Other symptoms may include bluish skin with oxygen saturations between 75 and 85%.[3]
The cause is generally unknown.[2] It is associated with a number of genetic syndromes including Turner, DiGeorge, and Down.[3] Diagnosis is generally by prenatal ultrasound.[3]
Initial treatment is with prostaglandin E1 or comfort care.[3] A procedure to make a hole in the atrial septum may also be required.[3] This may be followed by a set of three surgeries.[3] ECMO may be used to bridge the baby until surgery.[3] Without treatment children die within the first week of life.[3] With treatment about 66% live to at least 5 years of age.[3]
HLHS is rare, affecting about 1 in 5,000 newborns.[3] It makes up about 3% of cases of congenital heart disease.[3] It was first described in 1952 by Lev.[4] Among cases diagnosed before birth 12% to 48% of parents elect to abort the pregnancy.[3]
References[edit]
- ^ "Hypoplastic left heart syndrome: MedlinePlus Medical Encyclopedia". medlineplus.gov. Retrieved 28 May 2019.
- ^ a b c d "Congenital Heart Defects - Facts about Hypoplastic Left Heart Syndrome". Centers for Disease Control and Prevention. 19 November 2019. Retrieved 6 February 2021.
- ^ a b c d e f g h i j k l m n o p q r s t Kritzmire, SM; Cossu, AE (January 2020). "Hypoplastic Left Heart Syndrome". PMID 32119463.
{{cite journal}}: Cite journal requires|journal=(help) - ^ Rychik, Jack; Wernovsky, Gil (2003). Hypoplastic Left Heart Syndrome. Springer Science & Business Media. p. 39. ISBN 978-1-4020-7319-9.