ArticleHeart (British Cardiac Society)1997
A regional study of presentation and outcome of hypertrophic cardiomyopathy in infants.
Article in Heart (British Cardiac Society), 1997. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
5 citing papers in PubMed, 35 citations in OpenAlex.
- Efficacy of beta-blocker therapy in symptomatic athletes with exercise-induced intra-ventricular gradients.Cardiovascular ultrasound · 2010Trial
- Long-term outcomes of childhood onset Noonan compared to sarcomere hypertrophic cardiomyopathy.Cardiovascular diagnosis and therapy · 2019Article
- Beta-Blockers in Pediatric Hypertrophic Cardiomyopathies.Reviews on recent clinical trials · 2014Article
- A novel mutation in the PTPN11 gene in a patient with Noonan syndrome and rapidly progressive hypertrophic cardiomyopathy.European journal of pediatrics · 2005Article
- Article
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Authors and funding
5 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectiveTo describe regional incidence, presentation, and outcome of idiopathic (familial) and Noonan syndrome related infant hypertrophic cardiomyopathy (HCM) between 1969 and 1994.
designCase series.
settingRegional cardiac referral unit of the South West Region of England and south Wales, population approximately four million. PATIENTS: 21 cases of idiopathic (or familial) HCM, and eight infants with Noonan syndrome.
main outcome measuresSurvival and persistence or resolution of symptoms or cardiac hypertrophy.
resultsIncidence: eight cases between 1969 and 1982 (idiopathic 6, Noonan 2), 21 cases between 1982 to 1994 (idiopathic 15, Noonan 6). Mode of presentation: cardiac failure, 17 (59%); murmur, 9 (30%); cyanosis, 2 (7%); family history, 1 (7%). Age at presentation: 0-7 days, 16 (55%); 8 days-4 months, 9 (31%); 5-12 months, 4 (14%). OUTCOME: five deaths (17%), all < 1 year, all from progressive cardiac failure (idiopathic 3, Noonan 2). Four of these five had not received beta blockade. Among the 24 survivors (follow up 1.3-23.2 years, median 5.5 years) hypertrophy had resolved in nine (38%) (idiopathic 8, Noonan 1), was mild and asymptomatic in seven (29%), and was symptomatic or severe in eight (33%). All 10 infants presenting with septal thickness > 1.3 cm have persistent cardiac hypertrophy.
conclusionsMortality in infant HCM is much lower than previously reported and resolution is more frequent. This may reflect increased detection of less severe forms in addition to the success of aggressive medical management including beta blockade.
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