Hypoplastic left heart syndrome

Common Name(s)

Hypoplastic left heart syndrome

Hypoplastic left heart syndrome (HLHS) is a problem with the heartÕs structure that is present at birth (congenital). It occurs when parts of the left side of the heart (mitral valve, left ventricle, aortic valve, and aorta) do not develop completely. The underdeveloped left side of the heart is unable to provide enough blood flow to the body, which decreases the oxygen-rich blood supply. Babies with HLHS might look normal at birth, but will develop symptoms of HLHS within a few days. These symptoms might include: poor feeding, problems breathing, pounding heart, weak pulse, and ashen or bluish skin color. The cause of HLHs is presently unknown.
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Hypoplastic left heart syndrome" for support, advocacy or research.

Saving Little Hearts

Saving Little Hearts is dedicated to helping children with congenital heart defects and their families by providing emotional assistance and educational information primarily through the distribution of Care Packages.

Last Updated: 15 Mar 2013

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How do you compare to others with this condition?

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Hypoplastic left heart syndrome" for support, advocacy or research.

Saving Little Hearts

Saving Little Hearts is dedicated to helping children with congenital heart defects and their families by providing emotional assistance and educational information primarily through the distribution of Care Packages.

http://www.savinglittlehearts.com

Last Updated: 15 Mar 2013

View Details

 

General Support Organizations

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Hypoplastic left heart syndrome" returned 124 free, full-text research articles on human participants. First 3 results:

Use of computational fluid dynamics to estimate hemodynamic effects of respiration on hypoplastic left heart syndrome surgery: total cavopulmonary connection treatments.
 

Author(s): Jinlong Liu, Yi Qian, Qi Sun, Jinfen Liu, Mitsuo Umezu

Journal:

 

Total cavopulmonary connection (TCPC), a typical kind of Fontan procedure, is commonly used in the treatment of a functional single ventricle. The palliative cardiothoracic procedure is performed by connecting the superior vena cava and the inferior vena cava to the pulmonary arteries. ...

Last Updated: 3 Jan 2014

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Cardiac troponin I, cardiac troponin-specific autoantibodies and natriuretic peptides in children with hypoplastic left heart syndrome.
 

Author(s): Anneli Eerola, Tuija Poutanen, Tanja Savukoski, Kim Pettersson, Heikki Sairanen, Eero Jokinen, Jaana Pihkala

Journal: Interact Cardiovasc Thorac Surg. 2014 Jan;18(1):80-5.

 

To evaluate serum levels of cardiac troponin I (cTnI), autoantibodies against cardiac troponin (cTnAAbs) and natriuretic peptides during the treatment protocol in children with hypoplastic left heart syndrome (HLHS).

Last Updated: 19 Dec 2013

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Surgical palliation strategy does not affect interstage ventricular dysfunction or atrioventricular valve regurgitation in children with hypoplastic left heart syndrome and variants.
 

Author(s): Devin Chetan, Yasuhiro Kotani, Frederic Jacques, Jeffrey A Poynter, Lee N Benson, Kyong-Jin Lee, Rajiv R Chaturvedi, Mark K Friedberg, Glen S Van Arsdell, Christopher A Caldarone, Osami Honjo

Journal: Circulation. 2013 Sep;128(11 Suppl 1):S205-12.

 

All 3 palliation strategies, Norwood, Sano, and Hybrid, currently used for hypoplastic left heart syndrome pose a risk of myocardial injury at different times and through different mechanisms. We sought to compare these strategies to understand longitudinal differences in interstage ...

Last Updated: 13 Sep 2013

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Hypoplastic left heart syndrome" returned 11 free, full-text review articles on human participants. First 3 results:

Nutrition algorithms for infants with hypoplastic left heart syndrome; birth through the first interstage period.
 

Author(s): Julie Slicker, David A Hehir, Megan Horsley, Jessica Monczka, Kenan W Stern, Brandis Roman, Elena C Ocampo, Liz Flanagan, Erin Keenan, Linda M Lambert, Denise Davis, Marcy Lamonica, Nancy Rollison, Haleh Heydarian, Jeffrey B Anderson,

Journal: Congenit Heart Dis. ;8(2):89-102.

 

Failure to thrive is common in infants with hypoplastic left heart syndrome and its variants and those with poor growth may be at risk for worse surgical and neurodevelopmental outcomes. The etiology of growth failure in this population is multifactorial and complex, but may be impacted ...

Last Updated: 4 Apr 2013

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Hypoplastic left heart syndrome: current considerations and expectations.
 

Author(s): Jeffrey A Feinstein, D Woodrow Benson, Anne M Dubin, Meryl S Cohen, Dawn M Maxey, William T Mahle, Elfriede Pahl, Juan Villafañe, Ami B Bhatt, Lynn F Peng, Beth Ann Johnson, Alison L Marsden, Curt J Daniels, Nancy A Rudd, Christopher A Caldarone, Kathleen A Mussatto, David L Morales, D Dunbar Ivy, J William Gaynor, James S Tweddell, Barbara J Deal, Anke K Furck, Geoffrey L Rosenthal, Richard G Ohye, Nancy S Ghanayem, John P Cheatham, Wayne Tworetzky, Gerard R Martin

Journal: J. Am. Coll. Cardiol.. 2012 Jan;59(1 Suppl):S1-42.

 

In the recent era, no congenital heart defect has undergone a more dramatic change in diagnostic approach, management, and outcomes than hypoplastic left heart syndrome (HLHS). During this time, survival to the age of 5 years (including Fontan) has ranged from 50% to 69%, but current ...

Last Updated: 23 Dec 2011

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Neurodevelopment and quality of life for children with hypoplastic left heart syndrome: current knowns and unknowns.
 

Author(s): Caren S Goldberg, Kathleen Mussatto, Daniel Licht, Gil Wernovsky

Journal: Cardiol Young. 2011 Dec;21 Suppl 2():88-92.

 

The aim of this review is to describe the current state of knowledge related to neurodevelopmental outcomes and quality of life for children with hypoplastic left heart syndrome and to explore future questions to be answered for this group of children.

Last Updated: 13 Dec 2011

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in GeneReviews.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

Fetal Intervention for Aortic Stenosis and Evolving Hypoplastic Left Heart Syndrome
 

Status: Recruiting

Condition Summary: Aortic Stenosis; Hypoplastic Left Heart Syndrome

 

Last Updated: 21 Oct 2014

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A Prospective Study of Patients With Hypoplastic Left Heart Syndrome (HLHS) Following Stage II Surgical Palliation
 

Status: Recruiting

Condition Summary: Hypoplastic Left Heart Syndrome

 

Last Updated: 29 Aug 2014

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Safety Study of Autologous Umbilical Cord Blood Cells for Treatment of Hypoplastic Left Heart Syndrome
 

Status: Recruiting

Condition Summary: Hypoplastic Left Heart Syndrome

 

Last Updated: 29 Aug 2014

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