Congenital heart malformations associated with disproportionate ventricular septal thickening.
Why the study?
Is disproportionate ventricular septal thickening in patients with congenital heart malformations a manifestation of genetically determined asymmetric septal hypertrophy (ASH)?
Population
8 patients with disproportionate septal thickening and associated congenital cardiac malformations, plus…
Design
Case_series
Authors
Discussion
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Member takes
Implication
Disproportionate septal thickening in congenital malformations warrants caution against assuming genetic ASH; case reports challenge this link and leave open distinct etiologies.
Structured PICO
Is disproportionate ventricular septal thickening in patients with congenital heart malformations a manifestation of genetically determined asymmetric septal hypertrophy (ASH)?
P
Population
8 patients with disproportionate septal thickening (ventricular septal to posterobasal left ventricular free wall thickness ratios of 1.5 to 2.5) and associated congenital cardiac malformations (parachute deformity of the mitral valve, complete interruption of the aortic arch, or ventricular septal defect), plus first-degree relatives of 6 of the patients.
I
Intervention
Cardiac pathologic observations and echocardiography of first-degree relatives
O
Outcome
Arrangement of cardiac muscle cells in the disproportionately thickened ventricular septum and echocardiographic findings in first-degree relativessurrogate
Disproportionate ventricular septal thickening in congenital heart malformations is not necessarily a manifestation of genetically determined asymmetric septal hypertrophy.
Cite This Study
Maron et al. (1975) studied this question.