Miltiadis A. Stefadouros

Potentially lethal arrhythmias and their management in hypertrophic cardiomyopathy

The prevalence of potentially lethal arrhythmias (PLA) in groups of patients with hypertrophic cardiomyopathy has been assessed, but the rate at which they develop (their incidence) during long-term follow-up has not been reported. Therefore, conduction system disease (CSD) (sick sinus syndrome and His-ventricular disease), ventricular couplets and ventricular tachycardia (VT) detected by routine electrocardiograms, periodic 24-hour Holter monitoring and periodic exercise stress testing were studied in 50 patients treated with large doses of beta-adrenergic blocking drugs who were followed for 2 to 14 years (mean 5.9). Sixteen PLAs detected at the beginning of observation were excluded from actuarial analysis for new PLAs . Twenty-one patients had 24 new PLAs (7 with CSD, 1 patient with sustained supraventricular tachycardia, 6 with ventricular couplets and 10 with VT); only 43% of these PLAs were heralded by new symptoms. In 6 patients, the arrhythmia caused symptoms and was identified by a routine electrocardiogram. The 3 patients with His-ventricular disease presented with syncope and required electrophysiologic confirmation of this diagnosis. In only 1 patient was a PLA (ventricular couplets) detected only by exercise testing. All other ventricular arrhythmias were detected by Holter monitoring. The incidence of CSD in 47 patients free of this condition at entry was 5% at 5 years and 33% at 10 years. The incidence of ventricular couplets or VT in 39 patients free of these at entry was 26% at 5 years and 75% at 10 years, and the incidence of VT only was 18% at 5 years and 40% at 10 years.(ABSTRACT TRUNCATED AT 250 WORDS)

The impact of mitral valve morphology on the long-term outcome of mitral balloon valvuloplasty.

Background: The effect of mitral valve morphology (MVM) on the long‐term results of mitral balloon valvuloplasty (MBV) is not well established. The aim of the study was to evaluate the impact of MVM on long‐term outcome of MBV. Methods : Five hundred and eighteen consecutive patients (mean age, 31 ± 11 years) who underwent successful MBV were followed up for 0.5–16.5 (mean, 6 ± 4.5) years. Patients were divided into two groups according to their mitral echo score (MES) before MBV: group A (n = 340; MES ≤ 8) and group B (n = 178; MES > 8). Results: We report the immediate and long‐term clinical and echocardiographic results of the above‐mentioned 518 consecutive patients. The mitral valve area was significantly larger in group A than in group B, both immediately after MBV (2.0 ± 0.3 vs. 1.82 ± 0.3 cm2, respectively; P < 0.0001) and also at the last follow‐up (1.8 ± 0.33 vs. 1.5 ± 0.33 cm2, respectively; P < 0.0001). Restenosis occurred in 38/340 (11%) in group A vs. 73/178 (41%) in group B (P < 0.0001). Actuarial freedom from restenosis at 5, 10, 15 years were 92 ± 2%, 85 ± 3%, 65 ± 6% for group A vs. 72 ± 4%, 44 ± 5%, 9 ± 6% for group B (P < 0.001). Event‐free survival rates at 5, 10, 15 years for group A were 93 ± 1%, 88 ± 2%, 66 ± 6% vs. 82 ± 3%, 59± 6%, 8 ± 7% for group B (P < 0.0001). Stepwise Cox multivariate regression analysis identified MES, preprocedure functional class, and postprocedure mitral valve area ≤ 1.8 cm2, as predictors of restenosis (P < 0.0001, P = 0.014, P = 0.0015, respectively); MES and age as predictors of event‐free survival (P < 0.0001, P < 0.0001, respectively). Conclusion: MBV has excellent long‐term results in patients with favorable MVM. Favourable MVM is a strong predictor of good long‐term outcome.

Paradoxic response of the murmur of idiopathic hypertrophic subaortic stenosis to the Valsalva maneuver

A case of documented severe idiopathic hypertrophic subaortic stenosis in which the Valsalva maneuver resulted in paradoxic attenuation of the systolic ejection murmur is presented. Such a response should not be considered incompatible with the diagnosis of this disease.

Hypercalcemia and idiopathic hypertrophic subaortic stenosis.

Excerpt Although the primary cause of idiopathic hypertrophic subaortic stenosis is not known, it has been suggested that this cardiomyopathy may be induced by continuous excessive positive inotrop...

The effect of upright tilt on the volume of the failing human left ventricle

The effect of a passive change from supine to 25-degree head-up tilted position on left ventricular volume was studied by echocardiography and other noninvasive techniques in 18 normal subjects, 6 patients with compensated LV volume overloading, and 12 patients with LV failure. In normal subjects and patients with compensated LV volume overloading, 10 minutes of head-up tilt resulted in a significant decrease in the echocardiographic LV internal dimension equivalent to a decrease in the calculated LV end-diastolic volume of 27 and 16 per cent, respectively. In contrast, no change in LV end-diastolic dimension and volume was noted during tilt in the patients with LV failure. The response of heart rate, blood pressure, and LV ejection fraction to this intervention was insignificant in all groups. These data indicate that volume preload is unresponsive to postural changes in patients with LV failure but not in normal subjects or in those with compensated LV volume overloading. It is suggested that the effect of posture on LV volume and output is primarily determined by the absence or presence of LV failure and the consequences of it on the peripheral circulation.

Long-term (up to 18 years) clinical and echocardiographic results of mitral balloon valvuloplasty in children in comparison with adult population.

AIMS The purpose of this study was to assess the safety, efficacy, and long-term results (up to 18 years) of mitral balloon valvuloplasty (MBV) in children in comparison to adults. METHODS 57 children age < or = 18 years (group A) and 474 adult patients (group B) who underwent successful MBV and were followed up for a mean 8.5 +/- 4.8 (range 1.5-18) years were analyzed. RESULTS Patients in group A had a lower mitral echocardiographic score (echo score), 7.6 +/- 1.3 vs. 8.1 +/- 1 (P = 0.0005); smaller Doppler mitral valve area (MVA), 0.82 +/- 0.16 cm(2) vs. 0.92 +/- 0.17 cm(2) (P < 0.0001); and higher mitral valve gradient, 15.2 +/- 2.3 mmHg vs. 14.3 +/- 2.1 mmHg (P = 0.0003), than group B. Immediately after MBV, group A had larger MVA whether measured by catheter, 1.99 +/- 0.57 cm(2) vs. 1.8 +/- 0.52 cm(2) (P < 0.001), or by Doppler, 2.0 +/- 0.27 cm(2) vs. 1.97 +/- 0.28 cm(2) (P < 0.01), and similar complication rates, compared to group B. After a mean follow-up of 8.5 +/- 4.8 (range 1.5-18 years), restenosis in group A was 26% vs. 31% for group B (P = 0.41). Echo score > 8 (P = 0.046) was a predictor of restenosis in children and echo score > 8 (P < 0.0001) and previous surgery (P = 0.043) were predictors of restenosis in adults. Actuarial freedom from restenosis at 10, 15, and 18 years for groups A and B were 78%+/- 7%, 64%+/- 9%, and 18%+/- 14% and 77%+/- 2%, 43%+/- 4%, and 17%+/- 4%, respectively (P = 0.26). Event-free survival rates at 10, 15, and 18 years were 87%+/- 6%, 62%+/- 1%, and 20%+/- 2% versus 87%+/- 1%, 51%+/- 4%, and 20%+/- 5% for groups A and B, respectively (P = 0.51). Postprocedure MVA < 2.0 cm(2) (P = 0.043) and previous surgery (P = 0.03) were identified as predictors of events in children. Echo score > 8 (P < 0.0001) and prevalvuloplasty AF (P = 0.03) were identified as predictors of events in adults. CONCLUSION MBV is safe and effective in children with rheumatic MS. It provides better immediate results than in adults and excellent long-term results that are comparable to those seen in adults.

Internally recorded systolic time intervals in hypertrophic subaortic stenosis

Abstract The cardiac catheterization records of 20 patients with documented hypertrophic subaortic stenosis were analyzed to evaluate the utility of systolic time intervals in quantitating left ventricular outflow tract obstruction in this disease. The preejection period, ejection time and instantaneous peak outflow pressure gradient were measured from simultaneous recordings of the electrocardiogram and left ventricular and central aortic pressure signals, and the preejection period/ejection time (PEP/ET) ratio was computed. Most patients had a significant spontaneous or induced (with isoproterenol or postextrasystolic potentiation) variation in peak outflow gradient (range 0 to 186, average 85 mm Hg) with a high linear correlation between the peak outflow gradient and the systolic time intervals. For the group, peak outflow gradient correlated best with PEP/ET ratio ( r = −0.82, no. = 183) and less well with preejection period ( r = −0.62), ejection time ( r = 0.56) or their rate-corrected values ( r = −0.71 and r = 0.60, respectively). The PEP/ET ratio for beats recorded at the time of cardiac output measurement correlated with instantaneous mean outflow pressure gradient ( r = −0.88, no. = 28, P r = 0.72, no. = 28, P

Value of ventricular premature complex (VPC) morphology in the diagnosis of hypertrophic cardiomyopathy

Experimental and clinical studies have established the value of ventricular premature complexes (VPC) with a QR pattern in the diagnosis of occult antecedent myocardial infarction. However, the clinical significance of a QR pattern VPC in patients with hypertrophic cardiomyopathy has not been previously evaluated. In order to study this, we examined the ECGs and 24-hour Holter monitor records of 45 consecutive patients with hypertrophic cardiomyopathy and 106 consecutive patients with various other diseases. A QR pattern VPC in the precordial leads with a Q greater than or equal to 40 ms and a Q/R ratio greater than or equal to 0.20 had a specificity of 97%, a sensitivity of 29% and a predictive value of 80% in the diagnosis of hypertrophic cardiomyopathy. We conclude that a QR pattern VPC may be of value in the eclectic diagnosis of hypertrophic cardiomyopathy and that it is not specific for antecedent myocardial infarction as previously reported.

Determination of systolic time intervals from the first derivative of the indirect brachial arterial pulse.

Technical difficulties occasionally preclude adequate recording of the indirect carotid pulsations needed for measuring the systolic time intervals by the conventional method. Accordingly, in 81 subjects with a wide spectrum of heart diseases, an alternative method was tested based on substituting the first time derivative of the indirect brachial arterial pulse for the carotid pulse. The observed differences between systolic time intervals measured by the two methods were small enough, and their correlation coefficients high enough, to inspire confidence in the use of the proposed method as an alternative way for determining systolic time intervals.