Navigation Links
Risk factors help predict outcomes for children with rare heart condition
Date:9/3/2013

A long-term study of children with a complex heart condition called hypertrophic cardiomyopathy (HCM) found that the risk of death or need for immediate listing for heart transplantation was greatest for those who developed this disease as infants with congestive heart failure and for children who also had selective inborn errors of metabolism, a group of rare genetic disorders in which one or more of the body's key metabolic processes are disrupted.

The findings will be published online Tuesday in The Lancet to coincide with a presentation at the European Society of Cardiology Congress 2013 meeting in Amsterdam. The National Heart, Lung, and Blood Institute (NHLBI) and the Children's Cardiomyopathy Foundation supported this research.

Hypertrophic cardiomyopathy, a type of pediatric cardiomyopathy (diseases of the heart muscle) with varied causes and outcomes, is characterized by increased thickness (hypertrophy) of the heart wall.

HCM is rare, with fewer than one out of 100,000 children (ages birth to 18 years old) diagnosed annually in the United States. The condition is more frequently diagnosed in infants (under the age of one year), with 2 to 3 out of every 100,000 identified each year.

Researchers from the University of Miami Miller School of Medicine, along with colleagues from other institutions, sought to better understand how risk factors at the time of HCM diagnosis are predictors of death or the need to be added to a heart transplantation waiting list. They spent 19 years amassing data on more than 1,000 affected children at 98 pediatric cardiology centers in the U.S. and Canada through the NHLBI-funded Pediatric Cardiomyopathy Registry.

Researchers found that children with HCM caused by inborn errors of metabolism had the lowest rate of transplant-free survival within two years after diagnosis (43 percent). At two years after diagnosis, the rate of transplant-free survival was 55 percent for children with a mixed version of the disease that included thickening of the heart walls and enlargement and weakening of the heart and 62 percent for those with a restrictive form of the disease characterized by both a thickening and hardening of the heart walls. Children diagnosed after age one year with hypertrophic cardiomyopathy of unknown cause had the most favorable outcome, a 97 percent rate of transplant-free survival.

Infants and children who had two or more of the following risk factors at HCM diagnosis (depending on the cause of HCM) lower weight or body size, female gender, diagnosis during infancy, congestive heart failure, and abnormal heart function had an increased risk of poor outcomes (death or need for immediate heart transplantation).

These findings are expected to assist clinicians in identifying which children are likely to have the worst outcomes (death or need for immediate heart transplantation) within two years of diagnosis, thereby enabling medical teams, genetic counselors, and family planners to jointly and quickly determine the best treatment approach.


'/>"/>

Contact: NHLBI Communications Office
nhlbi_news@nhlbi.nih.gov
301-496-4236
NIH/National Heart, Lung and Blood Institute
Source:Eurekalert

Related biology news :

1. Environmental factors in Tiny Tims near fatal illness
2. Soy-based S-equol supplement reduces metabolic syndrome risk factors
3. Impaired recovery of Atlantic cod -- forage fish or other factors?
4. Longer sleep times may counteract genetic factors related to weight gain
5. Time, place and how wood is used are factors in carbon emissions from deforestation
6. Some factors that impact islet transplantation explored in Cell Transplantation papers
7. Some factors that impact islet transplantation explored in Cell Transplantation papers
8. True blood: SLU scientist investigates clotting factors
9. Johns Hopkins researchers link 2 biological risk factors for schizophrenia
10. Forest killer plant study explores rapid environmental change factors
11. Social factors trump genetic forces in forging friendships, CU-led study finds
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:12/22/2016)... SuperCom (NASDAQ:   SPCB ... e-Government, Public Safety, HealthCare, and Finance sectors announced today that Leaders ... to implement and deploy a community-based supportive services program to reduce ... , further expanding its presence in the state. ... This new program, which is expected ...
(Date:12/16/2016)... DUBLIN , Dec 16, 2016 Research ... Access System Market - Global Forecast to 2021" report to ... ... projected to grow at a CAGR of 14.06% from 2016 to ... 2016, and is projected to reach 854.8 Million by 2021. The ...
(Date:12/15/2016)... 2016 Advancements in biometrics will ... and wellbeing (HWW), and security of vehicles ... passenger vehicles begin to feature fingerprint recognition, ... beat monitoring, brain wave monitoring, stress detection, ... pulse detection. These will be driven by ...
Breaking Biology News(10 mins):
(Date:1/19/2017)... Jan 19, 2017 Research and Markets ... has announced the addition of the ... - Forecast to 2025" report to their offering. ... The report provides a detailed analysis on current and future market ... 2025, using estimated market values as the base numbers ...
(Date:1/19/2017)... AquaBounty Technologies, Inc. (AIM: ABTU; NASDAQ: AQB), ... and a majority-owned subsidiary of Intrexon Corporation (NYSE: ... listing of its common shares on the NASDAQ Stock ... "AquaBounty,s listing on NASDAQ represents an important milestone ... the U.S. markets as we advance plans for commercial ...
(Date:1/19/2017)... NEW YORK , Jan. 18, 2017 /PRNewswire/ ... expected to reach USD 92.9 billion by 2025, ... Research, Inc. Pharmaceutical industry has been adaptive of ... functions as early as 2002. Among the services ... the forerunners. For instance, Johnson & Johnson was ...
(Date:1/18/2017)... , January 18, 2017 According to a new market ... Neuroscience, Cytology, Infectious Disease), & End User (Molecular Diagnostic Laboratories, Academic and Research ... to reach USD 739.9 Million by 2021 from USD 557.1 Million in 2016, ... ... MarketsandMarkets Logo ...
Breaking Biology Technology: