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Hypertrophic cardiomyopathy presenting before 2 years of age in 13 patients
Insights
Early diagnosis and treatment of pediatric hypertrophic cardiomyopathy (HCM) led to favorable outcomes in young patients. Most children with HCM became asymptomatic with timely interventions, showing the importance of early detection.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Genetics
Background:
- Hypertrophic cardiomyopathy (HCM) is a significant cause of heart murmur in infants.
- Early presentation by age two necessitates prompt evaluation and management strategies.
Observation:
- Thirteen infants with HCM presented with heart murmurs; 4 had family histories, and 2 experienced congestive heart failure.
- Electrocardiograms revealed abnormalities in 12 patients, including ventricular hypertrophy and arrhythmias.
- Cardiac imaging confirmed asymmetric septal hypertrophy (ASH) in all, with left ventricular outflow tract (LVOT) obstruction in 3.
Findings:
- Patients received varied treatments: no therapy (6), propranolol (6), or left ventricular myomectomy (3).
- Over a mean follow-up of 6.1 years, no mortality occurred.
- Ten patients achieved or maintained an asymptomatic status.
Implications:
- Early diagnosis of pediatric HCM is crucial for timely intervention.
- Prompt treatment, including medical management and surgical options, can lead to favorable long-term clinical outcomes.
- Aggressive management strategies improve the prognosis for infants diagnosed with hypertrophic cardiomyopathy.
Abstract:
Thirteen patients with hypertrophic cardiomyopathy (HCM) who presented by 2 years of age were evaluated. All had been referred because of a heart murmur. Four had positive family histories for HCM and 2 had congestive heart failure. Cardiothoracic ratios ranged from 0.43 to 0.70 (mean 0.56). In 12 cases, electrocardiograms showed abnormal Q waves, ventricular hypertrophy, or aberrant rhythms. Resting peak systolic pressure differences ranged from 0 to 92 mm Hg (mean 21.1) across the right ventricular outflow tract, and from 0 to 112 mm Hg (mean 36) across the left ventricular outflow tract (LVOT). Cardiac angiography showed evidence of asymmetric septal hypertrophy (ASH) in all patients, LVOT obstruction in 3, and aortic and mitral insufficiency in 1. Six patients received no therapy, 6 were treated with propranolol, and 3 were treated with left ventricular myomectomy. During follow-up (mean 6.1 years), no patient died and 10 became or remained asymptomatic. The apparently favorable clinical course observed in these patients during this period of follow-up may be related to early treatment, made possible by the early diagnosis.