Obstructive hypertrophic cardiomyopathy in type III glycogen-storage disease.

We present here a rare case of a patient affected by hypertrophic obstructive cardiomyopathy related to type III glycogenosis. In this patient the correct diagnosis could only be performed by endomyocardial biopsy.

Left ventricular concentric remodelling and carotid structural changes in essential hypertension.

AIM: Left ventricular concentric remodelling defines a modified left ventricular geometry in the presence of a normal left ventricular mass; it is an early and frequent adaptation in arterial hypertension. The present study was designed to evaluate the extent of carotid structural changes in essential hypertensives with left ventricular remodelling. PATIENTS AND METHODS: Two groups of hypertensive patients, who had never previously received anti-hypertensive treatment, 14 with left ventricular concentric remodelling (group I, relative wall thickness 0.48 +/- 0.02) and 48 with normal left ventricular geometry (group II, relative wall thickness 0.37 +/- 0.04) underwent clinical and laboratory examination, echocardiography, carotid artery ultrasonography and 24 h ambulatory blood pressure monitoring (ABPM). The left ventricular dimensions and mass were obtained according to the Penn convention. The intima-media thickness (IMT) of the posterior wall of both common carotid arteries was measured 5, 10 and 20 mm caudally to the bulb and the average value was used for analysis. RESULTS: In both groups age (group I 44 +/- 9 years; group II 40 +/- 9 years), body surface area (group I 1.85 +/- 0.2 m2; group II 1.80 +/- 0.2 m2), duration of hypertension (group I 4.4 +/- 4; group II 3.8 +/- 3.9 years), metabolic parameters and smoking habits were similar. Both clinic and 24 h ABPM values were higher in group I (clinic 157 +/- 12/102 +/- 5; 24 h ABPM 145 +/- 10/95 +/- 7 mmHg) than they were in group II (clinic 146 +/- 11/97 +/- 5; 24 h ABPM = 134 +/- 10/87 +/- 8 mmHg, P < 0.01). The left ventricular mass index (LVMI) and IMT were found to be slightly but significantly greater in group I than they were in group II (LVMI 106 +/- 7 versus 98 +/- 12 g/m2, P < 0.05; IMT 0.68 +/- 0.13 versus 0.61 +/- 0.10 mm, P < 0.05). A significant correlation was found between LVMI and common carotid IMT in the whole group of hypertensive patients (r = 0.43, P < 0.01). CONCLUSIONS: Our results indicate that left ventricular concentric remodelling does not represent the only early cardiovascular change in arterial hypertension but rather is associated often with carotid intima-media thickening.

[Cardiac and vascular morphology in patients with "white-coat hypertension": echocardiographic and ultrasonographic study]. / Caratteristiche strutturali cardiache e vascolari nei soggetti con "white coat hypertension": studio echocardiografico ed ultrasonografico.

BACKGROUND: The introduction of ambulatory blood pressure (ABPM) in the clinical practice has defined a new subgroup of hypertensive patients (pts) called "white coat" hypertensive pts. It has been reported that white coat hypertensive pts have less cardiac involvement than established hypertensive pts. This study was designed to examine the extent of cardiac and vascular involvement in pts with white coat hypertension and established hypertension. METHODS: Fifty-four previously never treated pts with mild or moderate hypertension underwent a 24h ABPM and an echocardiographic and vascular ultrasonographic study in order to assess left ventricular anatomical parameters and the intima-media thickness of common carotid arteries. Left ventricular dimensions and mass were obtained according to Penn convention. The intima-media thickness of the far wall of both common carotid arteries was measured at 5, 10 and 20 mm caudally to the flow-divider; the average values were considered. RESULTS: Twenty-eight pts (age 43 +/- 8 years) had an average daytime ABPM below 140/90 mm Hg ("white coat") and 26 pts (age 46 +/- 10) had a consistently elevated diastolic blood pressure. Both groups had similar office blood pressure (152 +/- 9/101 +/- 7 vs 155 +/- 10/103 +/- 6 mm Hg) body surface mass (1.79 +/- 0.23 vs 1.86 +/- 0.21 mq), sex (18M/20F vs 18M/8F), duration of hypertension, glycolipidic parameters and smoking habit. The daytime ABPM was (by definition) significantly higher in established hypertensive pts than in white coat hypertensive pts (145 +/- 11/97 +/- 4 vs 129 +/- 7/83 +/- 5 mm Hg, p < 0.001). Left ventricular mass index and intima-media thickness resulted significantly greater in established (119 +/- 1.7 g/m2; 0.70 +/- 0.11 mm) than in white coat hypertensive pts (99 +/- 16 g/m2; 0.60 +/- 0.1 mm, p < 0.002). The prevalence of left ventricular hypertrophy and cardiac remodeling was more frequent in established hypertensive pts (53%) compared to white coat hypertensive pts (7.7%). CONCLUSION: The results of our study confirm that structural changes of left ventricle in white coat hypertensive pts are more limited than in established hypertensive pts and for the first time show that in white coat hypertensive pts the involvement of the conductance vessels is significantly lower than in established hypertensive pts.

Cardiac and carotid structure in patients with established hypertension and white-coat hypertension.

AIM: The introduction of ambulatory blood pressure monitoring in the clinical practice has defined a new subgroup of hypertensive patients called white-coat hypertensives. It has been reported that white-coat hypertensives have less cardiac involvement than established hypertensive patients. This study was designed to examine the extent of cardiac and vascular involvement in patients with white-coat hypertension and established hypertension. PATIENTS AND METHODS: We studied 82 patients with mild essential hypertension, never previously treated, using 24-h ambulatory blood pressure monitoring and an echocardiographic and vascular ultrasonographic study. Left ventricular dimensions and mass were obtained according to the Penn convention. The intima-media thickness of the posterior wall of both common carotid arteries was measured 5, 10 and 20 mm caudally to the flow-divider and the average value was used for analysis. RESULTS: Of the 82 patients, 31 (mean +/- SD age 35 +/- 10 years) had average 24-h systolic/diastolic blood pressure values of below 132/85 mmHg (white-coat hypertensives) and 51 (aged 42 +/- 2 years) had a consistently elevated diastolic blood pressure. Both groups had similar body surface area (1.82 +/- 0.22 versus 1.81 +/- 0.22 m2), sex distribution (20 males and 11 females versus 32 males and 19 females), duration of hypertension, metabolic parameters and smoking habit. The 24-h ambulatory blood pressure monitoring values were, by definition, significantly higher in established hypertensives than in white-coat hypertensives (142 +/- 10/94 +/- 6 versus 127 +/- 6/79 +/- 4 mmHg, P<0.001). The left ventricular mass index and intima-media thickness were significantly higher in the established hypertensives (112 +/- 17 g/m2, 0.67 +/- 0.11 mm, respectively) than in the white-coat hypertensives (98 +/- 18 g/m2, 0.58 +/- 0.09 mm; P<0.001 for both). CONCLUSIONS: The prevalence of left ventricular hypertrophy and cardiac remodeling was significantly more frequent in established hypertensives (51%) compared to white-coat hypertensives (19%). These confirm that structural changes in the left ventricle in white-coat hypertensives are more limited than in established hypertensives and show that in white-coat hypertensives there is significantly less involvement of the conductance vessels than in established hypertensives.