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Hypertrophic cardiomyopathy is an inherited primary disorder of themyoc ardium characterised by clinical heterogeneity. The severity and rate of progression of hypertrophy is an important factor in prognosis, and is likely to be dependent on factors including age, the disease-causing gene mutation, environmental infl uences and genetic modifiers.To study the influence of age on progression of hyp ertrophy, 62 patients with hypertrophic cardiomyopathy followed up for a minimum of 2 years were studied to determine the changes in left ventricular hypertroph y based on transthoracic M-mode and 2D echocardiography. DNA studies were perfo rmed to determine the role of the angiotensin-converting enzyme (ACE) gene dele tion polymorphism in modulating progression of left ventricular hypertrophy.Sixt y two patients were followed-up over a period of 6.0±3.2 years (range 2-16 ye ars). Patient data were analysed in two age groups: group 1 (patients aged a >30 years at first echocardiogram) had an increase in left ventricular septal wall thickness from 23.8±8.9 to 28.8± 8.7 mm (p< 0.001), while group 2 (patients aged >30 years) had a smaller but s ignificant increase from 17.8±4.2 to 19.5±6.2 mm (p< 0.05). DNA analysis of th e ACE gene deletion polymorphism showed those with the deletion/deletion(D/D) ge notype had a greater progression of left ventricular hypertrophy compared to tho se carrying the other ACE genotypes (increase in hypertrophy: 6.2±3.3 vs. 1.7± 4.2 mm; p< 0.01, D/D vs. I/D genotype; 2.8±5.8 mm; p=ns, D/D vs. I/I genotype). This association was independent of age, body mass and resting blood pressure.P rogression of left ventricular hypertrophy is most evident in the first 3 decade s of life, but is also observed in older age groups. Presence of the ACE gene D/ D polymorphism may be an important marker to identify those individuals with hyp ertrophic cardiomyopathy who are likely to have more progressive disease, and th erefore at higher risk of adverse clinical outcomes.
Hypertrophic cardiomyopathy is an inherited primary disorder of themyoc ardium characterised by clinical heterogeneity. The severity and rate of progression of hypertrophy is an important factor in prognosis, and is likely to be dependent on factors including age, the disease-causing gene mutation, environmental infl uences and genetic modifiers. To study the influence of age on progression of hyp ertrophy, 62 patients with hypertrophic cardiomyopathy followed up for a minimum of 2 years were studied to determine the changes in left ventricular hypertrophic based on transthoracic M-mode and 2D echocardiography DNA studies were perfo rmed to determine the role of the angiotensin-converting enzyme (ACE) gene deletion polymorphism in modulating progression of left ventricular hypertrophy. Sixt y two patients were followed-up over a period of 6.0 ± 3.2 years (range 2 -16 ye ars). Patient data were analyzed in two age groups: group 1 (patients aged a> 30 years at first echocardiogram) had an increase in left ventricular septal wall thickness from 23.8 ± 8.9 to 28.8 ± 8.7 mm (p <0.001), while group 2 (patients aged> 30 years) had a smaller but s ignificant increase from 17.8 ± 4.2 to 19.5 ± 6.2 mm (p <0.05). DNA analysis of th e ACE gene deletion polymorphism showed those with the deletion / deletion (D / D) ge notype had a greater progression of left ventricular hypertrophy compared to tho se carrying the other ACE genotypes (increase in hypertrophy: 6.2 ± 3.3 vs. 1.7 ± 4.2 mm; p <0.01, D / D vs. I / D genotype; 2.8 ± 5.8 mm; p = ns, D / D vs. I / body mass and resting blood pressure. P rogression of left ventricular hypertrophy is most evident in the first 3 decade s of life, but also also observed in older age groups. Presence of the ACE gene D / D polymorphism may be an important marker to identify those individuals with hyp ertrophic cardiomyopathy who are likely to have more progressive disease, and th erefore at higher risk of adverse cl inical outcomes.