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Echocardiographic predictors of poor outcome in infants with hypertrophic cardiomyopathy
K Suda1, T Kohl, J P Kovalchin
1Department of Pediatrics and Cardiovascular Research Institute, University of California, San Francisco, 94143-0214, USA.
Insights
In infants with hypertrophic cardiomyopathy (HC), increased left ventricular posterior wall thickness at birth predicts poor outcomes. Serial echocardiograms showing progressive thickening also indicate a higher mortality risk in these young patients.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Imaging
- Neonatal Medicine
Background:
- Hypertrophic cardiomyopathy (HC) is often diagnosed in infants using echocardiography.
- Echocardiographic predictors of outcome in infants with HC are not well-established.
- Early diagnosis and risk stratification are crucial for managing infantile HC.
Purpose of the Study:
- To identify echocardiographic predictors of poor outcome in infants diagnosed with hypertrophic cardiomyopathy (HC) before one year of age.
- To differentiate between infants with HC who survive and those who succumb within the first year of life.
- To correlate specific echocardiographic measurements with clinical outcomes in this pediatric population.
Main Methods:
- Retrospective review of medical records and echocardiograms for 19 infants (<1 year) with HC.
- Measurement of ventricular septum thickness, left ventricular (LV) posterior wall thickness, LV mass, LV volume, and ejection fraction.
- Comparison of echocardiographic parameters between infants who died within one year and those who survived.
Main Results:
- Nonsurvivors had a significantly larger Z value for LV posterior wall thickness (median 5.2 vs 1.3, p<0.04) at initial examination.
- Nonsurvivors exhibited progressive increases in ventricular septum and LV posterior wall thickness on serial echocardiograms.
- Survivors showed decreased LV posterior wall and septal thickness over time.
- Associated conditions in nonsurvivors included metabolic disease or Noonan syndrome; survivors had classic HC or maternal diabetes.
Conclusions:
- Early and progressive left ventricular posterior wall hypertrophy is a significant predictor of poor outcome in infants with HC.
- Echocardiographic assessment of LV posterior wall thickness is vital for risk stratification in neonates and infants with HC.
- These findings aid in understanding the natural history and prognosis of infantile hypertrophic cardiomyopathy.
Abstract:
Although hypertrophic cardiomyopathy (HC) is readily diagnosed by echocardiography in infants with isolated ventricular hypertrophy, echocardiographic predictors of outcome have not been described in this age group. To identify echocardiographic predictors of poor outcome of infants with HC, we reviewed medical records and echocardiograms of all 19 patients with HC aged <1 year seen at the University of California, San Francisco, from January 1981 through December 1995. Thickness of the ventricular septum and the left ventricular (LV) posterior wall, ventricular septum/LV posterior wall ratio, LV mass, LV volume, LV mass/volume ratio, LV ejection fraction, and right ventricular anterior wall thickness were measured. The median age at diagnosis was 1 day; 11 were boys and 8 were girls. Within 1 year after birth, 10 patients died: the other 9 patients have survived to > 12 months of age. At the initial postnatal examination, the Z value for LV posterior wall was larger (median, 5.2 vs 1.3, p <0.04) and, consequently, the ventricular septum/LV posterior wall ratio was lower (1.6 vs 2.3, p <0.04) in nonsurvivors than in survivors. In serial studies, the Z value for both the ventricular septum and the LV posterior wall thickness increased in nonsurvivors, but decreased in survivors. Nonsurvivors had intrinsic metabolic disease or Noonan syndrome. Survivors had classic HC or maternal diabetes. We conclude that, in infants with HC, early or progressive hypertrophy of the LV posterior wall is a predictor of poor outcome, regardless of associated disease.