Hypoplastic left heart syndrome

Hypoplastic left heart syndrome
Other namesCyanotic heart disease - hypoplastic left heart[1]
Illustration of heart with hypoplastic left heart syndrome
SpecialtyCardiology 

Hypoplastic left heart syndrome (HLHS) is a rare congenital heart defect in which the left side of the heart is severely underdeveloped and incapable of supporting the systemic circulation.[2] It is estimated to account for 2-3% of all congenital heart disease.[3] Early signs and symptoms include poor feeding, cyanosis, and diminished pulse in the extremities.[4] The etiology is believed to be multifactorial resulting from a combination of genetic mutations and defects resulting in altered blood flow in the heart.[5][6][7] Several structures can be affected including the left ventricle, aorta, aortic valve, or mitral valve all resulting in decreased systemic blood flow.[8]

Diagnosis can occur prenatally via ultrasound or shortly after birth via echocardiography.[5] Initial management is geared to maintaining patency of the ductus arteriosus - a connection between the pulmonary artery and the aorta that closes shortly after birth.[9] Thereafter, a patient subsequently undergoes a three-stage palliative repair over the next few years of life.[10] The Norwood procedure is typically done within a few days of birth.[10] The Glenn procedure is typically performed at three to six months of age.[10] Finally the Fontan procedure is done sometime between the age of two and five years of age.[10]

If left untreated, patients with HLHS die within the first weeks of life while 70% of those that undergo three-staged palliative surgery reach adulthood. After surgery, children with HLHS typically experience neurodevelopmental as well as motor delay and are at an increased risk of heart failure as adults.[11][12]

  1. ^ "Hypoplastic left heart syndrome: MedlinePlus Medical Encyclopedia". medlineplus.gov. Retrieved 28 May 2019.
  2. ^ Tchervenkov, C. I; Jacobs, J. P; Weinberg, P. M; Aiello, V. D; Béland, M. J; Colan, S. D; Elliott, M. J; Franklin, R. C; Gaynor, J. W; Krogmann, O. N; Kurosawa, H; Maruszewski, B; Stellin, G (2006). "The nomenclature, definition and classification of hypoplastic left heart syndrome". Cardiology in the Young. 16 (4): 339–368. doi:10.1017/s1047951106000291. PMID 16839428.
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