[Evaluation of systolic intervals in Duchenne cardiomyopathy]. / Evaluation des intervalles systoliques dans la cardiomyopathie de Duchenne.

Patients with progressive muscular dystrophy rarely survive beyond the second or third decades: the commonest causes of death are respiratory failure and cardiac failure. An easily repeatable method for early recognition of cardiac dysfunction and its follow-up would therefore be valuable. We performed a polycardiographic study of systolic time intervals in 11 patients with dystrophy (each subject representing an age from 7 to 17 years) in a group comprising 10 of their mothers and in control groups matched for age and sex. The mean values of QS2, PEP and QS1 were significantly higher in patients compared to controls (QS2 : 543 +/- 26 msec compared to 519 +/- 19 msec; PEP : 142 +/- 21 msec compared to 123 +/- 18 msec, and QS1 : 105 +/- 22 msec compared to 75 +/- 12 msec) whilst mean ICT was shorter (36 +/- 11 msec, compared to 49 +/- 5 msec). Mean LVET was comparable in patients and control. These results indicate a reduction in compliance and an increase in end diastolic left ventricular tension. When the systolic time intervals were analysed in each child, an increase in PEP and reduction in LVET were characteristic of the more serious phases of the disease with cardiac failure. It is emphasized that early stages of the disease are characterised by restrictive changes resulting in a reduction in ICT whilst terminal stages with reduced cardiac output cause a reduction in LVET and an increase in PEP. No significant changes were found in the mothers of patients compared to the control group. These results indicate that systolic time intervals may be useful in following up patients with Duchenne's cardiomyopathy.

[Electro-vectorcardiographic study in Duchenne de Boulogne progressive muscular dystrophy]. / Etude électro-vectocardiographique dans la dystrophie musculaire progressive de Duchenne de Boulogne.

Electrocardiographic changes are well known in Duchenne's muscular dystrophy (DMD). Their cause is still controversial. The aim of this study was: to verify the genetic theory of these changes to improve our understanding of their pathogenesis, by studying the distribution and characteristics of the mother carriers; to determine whether electro-vectocardiography could be of value in detecting carriers of the disease. Fourteen patients aged from 7 to 17 years old with DMD underwent ECG and VCG. ECG changes were recorded in 13 of the 14 cases: increased amplitude of the R wave in the right precordial leads or deep Q waves (amplitude greater than or equal to 2 mm, duration less than 0,04 msec) in the peripheral or left precordial leads. The algebraic sum of R--S in V1 was greater than normal in 6 carriers. VCG criteria of posterior myocardial infarction were fulfilled in 11 patients. "Bites" were recorded in 4 patients and 8 carriers. Electrical changes were therefore found in both patients and carriers with equal significance: in the first group the main appearances were of pseudo-infarction, principally involving the posterior wall; in the second group, less specific changes, best recorded on VCG, suggesting foci of myocardial fibrosis were observed. We think that the "bites" recorded in 66 p. 100 of mothers, could be a useful parameter for detecting carrier subjects.

[Comparison of the efficacy of monotherapy with a beta-blocker, a diuretic, and ACE inhibitors in the control of blood pressure during stress]. / Confronto dell'efficacia delle monoterapie con beta-bloccante, diuretico, ACE-inibitore nel controllo dei valori pressori durante stress.

In order to compare the efficacy of beta-blocking, diuretics and ACE-inhibiting monotherapy in controlling the blood pressure increase to stress, a study was conducted on 30 subjects (10 treated with atenolol, 10 with hydrochlorothiazide/amiloride combination, 10 with enalapril) with mild or moderate essential hypertension whose resting blood pressures were normalised by therapy. In the 3 groups of subjects blood pressure values at rest, during mental stress, static and dynamic exercise did not significantly differ before antihypertensive therapy. Atenolol and enalapril significantly reduced systolic and diastolic pressure below pretreatment values throughout and immediately after each test, differing from diuretic therapy which did not show any significant reduction in diastolic rises at the peak of hand-grip or in both systolic and diastolic pressures at the highest work-loads during dynamic exercise. In the recovery period of the exercise cycle test diuretics also produced a later normalisation of diastolic pressure. In conclusion, beta-blockers and ACE-inhibitors seem to be more effective than diuretics in the control of the blood pressure response to stress in hypertensive patients, suggesting that these drugs are the first choice treatment of mild to moderate hypertension.

[Coexistence of renovascular hypertension and pheochromocytoma. Description of 2 clinical cases]. / Coesistenza di ipertensione nefrovascolare e feocromocitoma. Descrizione di due casi clinici.

The two unusual clinical cases described here illustrate the importance of correct application of clinical methodology. Two patients, a 55-year-old man and a 50-year-old woman, presented with severe hypertension due to the coexistence of renal artery stenosis and pheochromocytoma. Their symptoms were indicative of renovascular hypertension which was verified by the finding of extremely elevated plasma renin activity and angiographic detection of critical renal artery stenosis. Further consideration of specific clinical findings led to the suspicion of coexisting pathologies: the detection of elevated plasma catecholamine levels and abdominal computed tomography and iodobenzylguanidine imaging confirmed the presence of pheochromocytoma. The first patient repeatedly refused surgical treatment and died after 9 months; the second patient recovered after undergoing combined nephrectomy and tumor removal. These cases underscore the importance of a carefully planned sequence of studies in patients presenting with uncommon or equivocal clinical manifestations, particularly when conclusive diagnosis is essential to successful treatment.

[Prospective evaluation of the captopril test in diagnosing renal artery stenosis in hypertensive patients]. / Valutazione prospettica del test al captopril nella diagnosi di stenosi di arteria renale in soggetti ipertesi.

In order to verify the utility of the captopril test (CT) in diagnosing renal artery stenosis we performed a prospective study in 94 consecutive patients (40 females, 54 males, mean age 52.4 +/- 12.3 years) suspected of having renovascular hypertension and with a serum level of creatinine < 2 mg/dl. Antihypertensive drugs were withdrawn one week before the CT or, if this was considered unsafe, patients were treated with nifedipine or diltiazem (53 subjects; 56.4%). We used renal angiography and the Muller criteria to interpret the CT. Our results were as follows: sensitivity, 92%; specificity, 96%; positive predictive value, 88%; and negative predictive value, 97%. In our study a simplified criterion for positive CT-postcaptopril plasma renin activity > 10 ng/mL/h-provided a similar diagnostic value. We conclude that the captopril test is a useful screening test for the detection of renal artery stenosis in selected hypertensive patients and that it can also be reliably performed in patients who are taking calcium antagonists.

[Severe hypopotassemia and rhabdomyolysis in a normotensive subject after use of a nasal spray containing 9-alpha-fluoroprednisolone]. / Severa ipopotassiemia e rabdomiolisi da uso di spray nasale contenente 9-alfa-fluorprednisolone in soggetto normoteso.

We report the case of a 16-year-old boy who was admitted to our hospital for acute urinary retention, flaccid paralysis of the legs and arms and rhabdomyolysis due to a nasal spray containing 9-alpha-fluoroprednisolone. In our normotensive subject, rhabdomyolysis occurred after vigorous physical activity performed before the administration. The case emphasizes the need to avoid the use of 9-alpha-fluoroprednisolone considering its intense mineralocorticoid activity.

[P wave analysis for the electrocardiographic diagnosis of left ventricular hypertrophy. A study of a population with arterial hypertension (author's transl)]. / Analasi dell'onda P per la diagnosi elettrocardiografica di ipertrofia ventricolare sinistra. Studio di una popolazione di ipertesi.

The electrocardiogram of 100 normal subjects and 216 patients with arterial hypertension in different stages have been analysed. P wave especially has been taken into consideration: it is often abnormal in the presence of left ventricular hypertrophy (LVI). The point-score system elaborated by Romhilt and Estes allows, if only QRS-T is considered, to diagnose LVI in 32 of the hypertensive patients (15%) and in 41 cases (19%) if the increase of P wave terminal forces is emphasized. Nevertheless this P wave characteristic does not appear more useful than others (axis less than or equal to 40 degrees; duration greater than or equal to 0.12 sec; presence of notch). The best results are achieved by considering the coexistence of two or more abnormalities of the P wave. Thus LVI is diagnosed in 52 (24%) of our cases, without false positive.

Beta-blocker versus diuretic for control of the blood pressure response to stress in hypertensive patients.

To compare the antihypertensive effects of beta-blockers and diuretics on the blood pressure increase to stress, a randomized single-blind crossover study was performed in 27 patients with mild or moderate hypertension. At the initial examination and after two subsequent periods of therapy with 100 mg atenolol or with a combination of 50 mg hydrochlorothiazide and 5 mg amiloride hydrochloride, once a day, blood pressure and heart rate were measured at rest, during mental arithmetic, sustained handgrip and cycloergometric test. Both treatment significantly decreased supine and standing systolic and diastolic pressure at rest, during and immediately after mental stress and isometric exercise, with the reduction of diastolic pressure significantly greater after atenolol. During dynamic exercise, systolic and diastolic pressures were significantly decreased by diuretics at the lowest work-load only, whereas beta-blocker caused significant and greater blood pressure reductions throughout the exercise. The combination of two classes of drug normalized resting blood pressure in 8 of 9 subjects in which the monotherapy had failed to obtain values less than 140/90 mmHg and gave a better control of systolic and diastolic pressures throughout all the stress tests. It is concluded that atenolol is more effective than diuretics during stress, suggesting that beta-blocking drugs are the first choice treatment for mild to moderate hypertension and that when the antihypertensive effect of a single agent is insufficient, a combination of beta-blockers and diuretics is also effective during stress.

[Presence of bites in VCGrams of a high coronary risk population (author's transl)]. / Presenza di bites nel vettocardiogramma di una popolazione ad alto rischio coronarico.

The association between the presence of areas of myocardial fibrosis and the appearance of unexpected DEVIATIONS OF THE QRS loop-outline on the vectorcardiographic tracing (bites) has been reported. In order to re-evaluate the correlation between the presence of bites and the possible existence of scars we have studied 511 patients. On the basis of clinical data, laboratory data, rest and exercise electrocardiograms, the subjects have been divided into four groups; 195 normal subjects; 267 with ischemic heart disease, 16 with arterial hypertension and 33 with diabetes mellitus (the last two groups as representative of subjects with higher risk for myocardial ischemia). Bites in at least one plane were detected in 9.7% of normal subjects, 27.8% of ischemic patients, 56.3% of hypertensive patients and 18.2% of diabetics (less than 0.001). The genesis of bites and reliability of the diagnostic criteria are discussed. With more restrictive criteria the frequency in the normal subjects falls to 1%, while in the other three groups it remains much higher (10.1%-12.5%-6.1%).

Usefulness of stress testing for the evaluation of hypertensive heart disease in young hypertensive subjects.

To investigate the usefulness of stress testing for the evaluation of hypertensive heart disease, 40 subjects, 28 men and 12 women (mean age 30.8 +/- 6.2 years), with mild or moderate hypertension, without ST segment or T wave abnormalities in their resting ECG, were examined. 13 patients (32.5%) showed exercise-induced ST segment depression. The heart rate at rest was significantly higher in the patients with a positive response; 6 of the 7 subjects with electrocardiographic signs of left ventricular hypertrophy (summed SV1 + maximum R V5/V6 voltage of 45 mm or more) had a positive exercise electrocardiographic test. There were no significant differences between positive and negative cases in age, sex, systolic and diastolic blood pressure, or the double product (heart rate X systolic pressure) at rest or during exercise. After resting blood pressure values had been significantly decreased by giving methyldopa with or without diuretics for at least 6 months, there were a regression of left ventricular hypertrophy in the resting ECG and an impressive reduction in the prevalence of exercise-positive responses (to 17.5%). In the 7 patients with positive exercise electrocardiographic tests even after antihypertensive treatment, no significant reduction in blood pressure values during exercise was obtained.

Prognostic value of exercise EKG testing in asymptomatic normotensive subjects. A prospective matched study.

We prospectively studied 135 asymptomatic normotensive subjects with exercise-induced ST ischemic depression of 1 mm or more and compared them with 379 controls. At least two controls with negative responses on the exercise electrocardiographic (EKG) test were selected for each case and were matched for age, sex, work, community, and coronary-risk-factors index. The end points considered were the following coronary events: angina pectoris, myocardial infarction, and sudden death. After a median follow-up period of 6.0 years for the cases and 6.4 years for the controls, the relative risk was 5.55 (95 per cent confidence limits, 2.75 to 11.22). Coronary events occurred significantly earlier in the cases than in the controls. Our data also suggest that the exercise EKG response is a particularly good prognostic indicator for myocardial infarction. In addition, our analysis has confirmed the predictive roles of age, smoking, blood pressure, and the coronary-risk-factors index and suggests that the exercise EKG response is an additional independent risk indicator for coronary events.