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Biomedical subjects

P M Elliott

Publications and source records attributed to P M Elliott.

At least 37 records · Page 2Linked to original sources
PubMed2001

Relation between myocyte disarray and outcome in hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy (HC) is associated with an increased risk of sudden cardiac death or death from heart failure. Little is known of the pathologic substrate for risk of premature death in this disease. We therefore set out to correlate the pathologic findings with the mode of death and risk profile in 75 patients with HC. Hearts with HC were obtained after death or transplantation. The clinical details were correlated with the macroscopic findings and the percent fibrosis, disarray, and small-vessel disease across 19 sections of each heart. Thirty-nine patients died suddenly, 28 had end-stage heart failure, and 8 died of other causes. Myocyte disarray correlated positively with evidence of ischemia (r = 0.5, p <0.0001), and was greater in patients who died before age 21 years (mean disarray 33% vs 18%, p <0.0001) and in those with an abnormal vascular response to exercise (mean disarray and 30% vs 19%, p = 0.04). Myocardial fibrosis was greater in patients who died in heart failure (mean percent fibrosis was 2.8% versus 0.9%, p = 0.003), and in patients with nonsustained ventricular tachycardia or a high risk fractionation study (4.9% vs 2.7%, p = 0.04, and 6.84% vs 2.8%, p = 0.03, respectively). In conclusion, young patients who die with HC have greater disarray than their older counterparts. In contrast, myocardial fibrosis is the substrate for premature deaths from heart failure and is associated with an increased risk of a primary ventricular arrhythmia.

Adolescent↗
PubMed2001

Relation between severity of left-ventricular hypertrophy and prognosis in patients with hypertrophic cardiomyopathy.

BACKGROUND: A previous study suggested that severe left-ventricular hypertrophy (maximum wall thickness > or = 30 mm) in patients with hypertrophic cardiomyopathy is associated with a risk of sudden cardiac death sufficient to warrant consideration for implantation of a cardioverter defibrillator (ICD). However, the prognostic significance of left-ventricular hypertrophy in relation to other clinical risk factors is poorly characterised. METHODS: We studied 630 patients consecutively referred to one hospital in London, UK (mean age 37 years [SD 16]; 382 male; mean follow-up 59 months). Patients underwent two-dimensional and doppler echocardiography, upright exercise testing, and Holter monitoring. FINDINGS: 39 patients died suddenly or had an appropriate ICD discharge; nine died from progressive heart failure; 11 from other cardiovascular causes and 23 from non-cardiac causes. There was a trend towards higher probability of sudden death or ICD discharge with increasing wall thickness (p=0.029, relative risk per 5 mm increment 1.31 [95% CI 1.03-1.66]). Of the 39 patients who died suddenly or had an ICD discharge, ten had a wall thickness of 30 mm or more. Patients with wall thickness of 30 mm or more had higher probability of sudden death or ICD discharge than patients with wall thickness less than 30 mm (p=0.049, 2.07 [1.00-4.25]. When considered together, the number of additional risk factors (one to three) was a better predictor of risk of sudden death or ICD discharge than wall thickness (p=0.0001, relative risk per additional factor 2.00 [1.43-2.79] vs p=0.058, 1.26 per 5 mm increment [0.99-1.60]). There was no relation between the pattern of hypertrophy and survival. INTERPRETATION: The risk of sudden death associated with a wall thickness of 30 mm or more in patients without other risk factors is insufficient to justify aggressive prophylactic therapy. Most sudden deaths occurred in patients with wall thickness less than 30 mm, so the presence of mild hypertrophy cannot be used to reassure patients that they are at low risk.

Adult↗
PubMed2001

The role of pharmacologic treatment to prevent sudden death in the implantable cardioverter defibrillator era.

The prevention of sudden death remains a key aspect of clinical management in patients with hypertrophic cardiomyopathy (HCM). For many years, amiodarone has been widely used to treat and prevent symptomatic atrial arrhythmias in patients with HCM, but its role in the prevention of potentially fatal ventricular arrhythmia has remained controversial. The publication of several observational studies examining the efficacy of implantable cardioverter defibrillators (ICDs) in patients with the disease has stimulated a re-evaluation of the drug's place in the management of HCM. This article reviews the literature on amiodarone therapy in HCM and discusses the indications for its use in the ICD era.

Amiodarone↗
PubMed2001

Is QT dispersion associated with sudden cardiac death in patients with hypertrophic cardiomyopathy?

QT dispersion is significantly greater in patients with hypertrophic cardiomyopathy (HCM) than that in healthy subjects. Few data exist regarding the prognostic value of QT dispersion in HCM. In this study, we retrospectively investigated the association between QT dispersion and sudden cardiac death in 46 patients with HCM (mean 33.1 +/- 15.5 years, 32 men). The case group consisted of 23 HCM patients who died suddenly, and the control group consisted of 23 HCM patients who survived uneventfully during follow-up. Study patients were pair-matched for age, gender, and maximum left ventricular wall thickness. QT dispersion (maximum minus minimum QT interval) was manually measured on early 12-lead ECGs using a digitizing board. An in-house program was used for calculating QT interval, QT dispersion, JT interval, and JT dispersion (maximum minus minimum J point to T end interval). Patients in the case group tended to have shorter RR intervals than those in the control group (777 +/- 171 vs 856 +/- 192 ms, P = 0.08). Maximum corrected QT and JT intervals did not discriminate the case group from controls (489 +/- 29 vs 479 +/- 27 ms, P = NS; 375 +/- 36 vs 366 +/- 22 ms, P = NS, respectively). Greater QT dispersion and JT dispersion were found in the case group compared with controls (74 +/- 28 vs 59 +/- 21 ms, P = 0.02 and 76 +/- 32 vs 59 +/- 26 ms, P = 0.03, respectively). The measurements of maximum QT, JT, and T peak to T end intervals, precordial QT and JT dispersion, and T peak and T end dispersion were all comparable between the two groups (P = NS for all). No systematic changes in ECG measurements were found from late ECGs of the case group compared to those from early ECGs (P = NS). No correlation between maximum left ventricular wall thickness and QT dispersion, JT dispersion, maximum QTc or JTc intervals was observed (r < 0.29, P > 0.05 for all). Our results show that increased QT dispersion and JT dispersion is weakly associated with sudden cardiac death in the selected patients with HCM.

Adolescent↗
PubMed2000

Natural history of hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy is a disease of the cardiac sarcomere and is the most common inherited cardiovascular disorder affecting up to 1 in 500 people in the general population. The disease is typified by variable clinical penetrance and heterogeneous clinical expression, resulting in a wide range of clinical manifestations. Most patients have few if any symptoms and a relatively benign clinical course. A minority are at risk of serious complications including ventricular arrhythmia, sudden death, thromboembolism, congestive cardiac failure, heart block, and infective endocarditis. This article reviews the natural history of the disease, with particular emphasis on lessons learned from recent genetic studies.

Cardiomyopathy, Hypertrophic↗
PubMed2000

Pacing in hypertrophic cardiomyopathy.

Dual chamber pacing has been proposed as an alternative to surgery in the management of hypertrophic cardiomyopathy. Reports have documented hemodynamic and symptomatic benefit from dual chamber pacing, raising the question of whether or not all patients with drug-refractory symptoms should undergo a trial of pacing before consideration of surgery. The enthusiasm for pacing in hypertrophic cardiomyopathy has generated a number of investigations addressing this issue, including several recently concluded clinical trials. This article reviews the recent experience with dual chamber pacing in hypertrophic cardiomyopathy.

Cardiac Pacing, Artificial↗
PubMed2000

Utility of metabolic exercise testing in distinguishing hypertrophic cardiomyopathy from physiologic left ventricular hypertrophy in athletes.

OBJECTIVES: This study evaluated the role of metabolic (cardiopulmonary gas exchange) exercise testing in differentiating physiologic LVH in athletes from HCM. BACKGROUND: Regular intensive training may cause mild increases in left ventricular wall thickness (LVWT). Although the degree of left ventricular hypertrophy (LVH) is typically less than that seen in hypertrophic cardiomyopathy (HCM), genetic studies have shown that a substantial minority of patients with HCM have an LVWT in the same range. The differentiation of physiologic and pathologic LVH in this "gray zone" can be problematic using echocardiography and electrocardiography alone. METHODS: Eight athletic men with genetically proven HCM and mild LVH (13.9 +/- 1.1 mm) and eight elite male athletes matched for age, size and LVWT (13.4 +/- 0.9 mm) underwent symptom limited metabolic exercise stress testing. Peak oxygen consumption (pVO2), anaerobic threshold, oxygen pulse and respiratory exchange ratios were measured in both groups and compared with those observed in 12 elite and 12 recreational age- and size-matched athletes without LVH. RESULTS: Elite athletes with LVH had significantly greater pVO2 (66.2 +/- 4.1 ml/kg/min vs. 34.3 +/- 4.1 ml/kg/min; p < 0.0001), anaerobic threshold (61.6 +/- 1.8% of the predicted maximum VO2 vs. 41.4 +/- 4.9% of the predicted maximum VO2; p < 0.001) and oxygen pulse (27.1 +/- 3.2 ml/beat vs. 14.3 +/- 1.8 ml/beat; p < 0.0001) than individuals with HCM. A pVO2 >50 ml/kg/min or >20% above the predicted maximum VO2 differentiated athlete's heart from HCM. CONCLUSIONS: Metabolic exercise testing facilitates the differentiation between physiologic LVH and HCM in individuals in the "gray zone."

Adolescent↗
PubMed2000

Sudden death in hypertrophic cardiomyopathy: identification of high risk patients.

OBJECTIVES: We sought to identify patients with hypertrophic cardiomyopathy (HCM) at high risk of sudden death (SD). BACKGROUND: Relatively low mortality rates in HCM make conventional analysis of multiple clinical risk markers for SD problematic. This study used a referral center registry to investigate a smaller number of generally accepted noninvasive risk markers. METHODS: We studied 368 patients (14 to 65 years old, 239 males) with HCM. There were five variables: nonsustained ventricular tachycardia (NSVT), syncope, exercise blood pressure response (BPR), family history of sudden death (FHSD) and left ventricular wall thickness (LVWT). RESULTS: During follow-up (3.6+/-2.5 years [range 2 days to 9.6 years]), 36 patients (9.8%) died, 22 of them suddenly. Two patients received heart transplants. The six-year SD-free survival rate was 91% (95% confidence interval [CI] 87% to 95%). In the Cox model, there was a significant pairwise interaction between FHSD and syncope (p = 0.01), and these were subsequently considered together. The multivariate SD risk ratios (with 95% CIs) were 1.8 for BPR (0.7 to 4.4) (p = 0.22); 5.3 for FHSD and syncope (1.9 to 14.9) (p = 0.002); 1.9 for NSVT (0.7 to 5.0) (p = 0.18) and 2.9 for LVWT (1.1 to 7.1) (p = 0.03). Patients with no risk factors (n = 203) had an estimated six-year SD-free survival rate of 95% (95% CI 91% to 99%). The corresponding six-year estimates (with 95% CIs) for one (n = 122), two (n = 36) and three (n = 7) risk factors were 93% (87% to 99%), 82% (67% to 96%) and 36% (0% to 75%), respectively. Patients with two or more risk factors had a lower six-year SD survival rate (95% CI) compared with patients with one or no risk factors (72% [56% to 88%] vs. 94% [91% to 98%]) (p = 0.0001). CONCLUSIONS: This study demonstrates that patients with multiple risk factors have a substantially increased risk of SD sufficient to warrant consideration for prophylactic therapy.

Adult↗
PubMed2000

Can the assessment of dynamic QT dispersion on exercise electrocardiogram predict sudden cardiac death in hypertrophic cardiomyopathy?

Premature sudden cardiac death (SD) is a critical event in the natural history of hypertrophic cardiomyopathy (HCM), and occurs during or just after physical exertion in approximately 60% of instances. Abnormalities in ventricular repolarization may not be present at rest in some patients but may become apparent under certain conditions. This study was performed to examine whether dynamic QT dispersion during exercise is associated with SD in HCM. Twenty-four HCM patients with catastrophic events (group I; 18 SD, 6 ventricular fibrillation) and 24 event-free survivors (group II) were studied. The two groups were pair-matched for age, gender, and maximum left ventricular wall thickness. QT intervals were manually measured from 12-lead exercise electrocardiogram (ECG) with a digitizing board. A custom-developed program was used to calculate QT and JT dispersion. The QT/RR relationship was evaluated by the slope of linear regression analysis. Before exercise, significant differences in heart rate and JT dispersion were found between group I and II. During exercise, heart rate increased and QT decreased significantly in both groups. QT and JT dispersion decreased in both groups, though the magnitude of reduction was greater in group I than in group II. No significant differences in QTc interval and QT or JT dispersion were found between the groups at any stages. At 3 minutes of recovery, heart rate had decreased but remained higher than before exercise, and all measurements of QT components remained shorter compared with those made before exercise in both groups. There was a strong correlation between QT and RR interval during exercise in all study patients (r = 0.95). No difference in the slope of QT against RR intervals was found between the groups (0.317 vs 0.319). In conclusion, exercise reduced QT dispersion in patients with HCM. The dynamic changes in QT dispersion examined by this method on exercise ECG did not make additional contributions in their risk stratification.

Adolescent↗
PubMed2000

Left ventricular opacification during selective intracoronary injection of echocardiographic contrast in patients with hypertrophic cardiomyopathy.

Percutaneous alcohol ablation of the interventricular septum via the first septal perforator branch of the left anterior descending artery can successfully treat dynamic left ventricular outflow tract obstruction in patients with hypertrophic cardiomyopathy. Increasingly, echocardiographic contrast agents are used before alcohol injection to identify the perfusion bed of the septal perforator vessels. This study describes the unexpected opacification of the left ventricular cavity in three of five consecutive patients following selective injection of the first septal perforator with Optison. This case study demonstrates that direct communication between the first septal perforator vessel and the left ventricle is common, an observation that may have considerable relevance to the technique of alcohol septal reduction.

Adult↗
PubMed2000

Abnormal cardiopulmonary exercise variables in asymptomatic relatives of patients with dilated cardiomyopathy who have left ventricular enlargement.

BACKGROUND: Left ventricular enlargement with normal systolic function is common in asymptomatic relatives of patients with familial dilated cardiomyopathy, many of whom progress to overt dilated cardiomyopathy at follow up. OBJECTIVE: To examine maximal and submaximal gas exchange variables of cardiopulmonary exercise testing in asymptomatic relatives with left ventricular enlargement. DESIGN AND SETTING: Controlled evaluation of metabolic exercise performance of patients with dilated cardiomyopathy and asymptomatic relatives with left ventricular enlargement identified through prospective family screening in a cardiomyopathy outpatient clinic. METHODS: 23 relatives with left ventricular enlargement, 33 normal controls, 29 patients with dilated cardiomyopathy, and 10 elite athletes with echocardiographic criteria of left ventricular enlargement ("physiological" enlargement) underwent symptom limited upright cycle ergometry using a ramp protocol. RESULTS: Peak oxygen consumption (pVO(2); mean (SD)) was significantly reduced in relatives with left ventricular enlargement (78 (16.3)%) v normal controls (96%, p < 0.01) and athletes (152%, p < 0.001), but was higher than in patients with dilated cardiomyopathy (69%, p < 0.01). pVO(2) was less than 80% of predicted in 75% of patients, 58% of relatives, 22% of controls, and none of the athletes. Oxygen pulse (pVO(2)/heart rate) was less than 80% of predicted in 69% of patients, 35% of relatives, 6% of controls, and none of the athletes. The slope of minute ventilation v CO(2) production (DeltaVE/DeltaVCO(2)) was > 30 in 68% of patients, 50% of relatives, and in none of the controls or athletes. Anaerobic threshold, occurring in relatives at 37 (14)% of the predicted VO(2), was higher than in the patients (32%, p < 0.01) and lower than in the controls (45%, p < 0.05) or in the athletes (55%, p < 0.001). CONCLUSIONS: Maximal and submaximal cardiopulmonary exercise test variables are abnormal in asymptomatic relatives with left ventricular enlargement, in spite of normal systolic function. This provides further evidence that left ventricular enlargement represents subclinical disease in relatives of patients with dilated cardiomyopathy. Metabolic exercise testing can complement echocardiography in identifying relatives at risk for the development of dilated cardiomyopathy.

Adolescent↗
PubMed2000

Hypertrophic cardiomyopathy: the interrelation of disarray, fibrosis, and small vessel disease.

OBJECTIVE: To make a quantitative assessment of the relation between disarray, fibrosis, and small vessel disease in hypertrophic cardiomyopathy. DESIGN: Detailed macroscopic and histological examination at 19 segments of the left and right ventricle and the left atrial free wall. PATIENTS: 72 patients with hypertrophic cardiomyopathy who had suffered sudden death or progression to end stage cardiac failure (resulting in death or heart transplantation). MAIN OUTCOME MEASURES: The presence of scarring, atrial dilatation, and a mitral valve impact lesion were noted, and heart weight, wall thickness, per cent disarray, per cent fibrosis, and per cent small vessel disease quantitated for each heart. RESULTS: Within an individual heart the magnitude of hypertrophy correlated with the severity of fibrosis (p = 0.006) and disarray (p = 0.0002). Overall, however, total heart weight related weakly but significantly to fibrosis (r = 0.4, p = 0.0001) and small vessel disease (r = 0.3, p = 0.03), but not to disarray. Disarray was greater in hearts with mild left ventricular hypertrophy (maximum wall thickness < 20 mm) and preserved systolic function (60.9 (26)% v 43 (20.4)% respectively, p = 0.02) and hearts without a mitral valve impact lesion (26.3% v 18.9%, p = 0.04), but was uninfluenced by sex. Fibrosis was influenced by sex (7% in male patients and 4% in female, p = 0.04), but not by the presence of an impact lesion. No relation was found between disarray, fibrosis, and small vessel disease. CONCLUSIONS: Myocyte disarray is probably a direct response to functional or structural abnormalities of the mutated sarcomeric protein, while fibrosis and small vessel disease are secondary phenomena unrelated to disarray, but modified by factors such as left ventricular mass, sex, and perhaps local autocrine factors.

Adolescent↗
PubMed1999

Survival after cardiac arrest or sustained ventricular tachycardia in patients with hypertrophic cardiomyopathy.

OBJECTIVES: The aim of this study was to evaluate the survival of patients with hypertrophic cardiomyopathy (HCM) after resuscitated ventricular fibrillation or syncopal sustained ventricular tachycardia (VT/VF) when treated with low dose amiodarone or implantable cardioverter defibrillators (ICDs). BACKGROUND: Prospective data on clinical outcome in patients with HCM who survive a cardiac arrest are limited, but studies conducted before the widespread use of amiodarone and/or ICD therapy suggest that over a third die within seven years from sudden cardiac death or progressive heart failure. METHODS: Sixteen HCM patients with a history of VT/VF (nine male, age at VT/VF 19 +/- 8 years [range 10 to 36]) were studied. Syncopal sustained ventricular tachycardia/ventricular fibrillation occurred during or immediately after exertion in eight patients and was the initial presentation in eight. One patient had disabling neurologic deficit after VT/VF. Before VT/VF, two patients had angina, four had syncope and six had a family history of premature sudden cardiac death. After VT/VF all patients were in New York Heart Association class I or II, three had nonsustained VT during ambulatory electrocardiography and 11 had an abnormal exercise blood pressure response. After VT/VF eight patients were treated with low dose amiodarone and six received an ICD. Prophylactic therapy was declined by two patients. RESULTS: Mean follow-up was 6.1 +/- 4.0 years (range 0.5 to 14.5). Cumulative survival (death or ICD discharge) for the entire cohort was 59% at five years (95% confidence interval: 33% to 84%). Thirteen (81%) patients were alive at last follow-up. Two patients died suddenly while taking low dose amiodarone, and one died due to neurologic complications of his initial cardiac arrest. Three patients had one or more appropriate ICD discharges during follow-up; the times to first shock after ICD implantation were 23, 197 and 1,124 days. CONCLUSIONS: This study shows that patients with HCM who survive an episode of VT/VF remain at risk for a recurrent event. Implantable cardioverter defibrillator therapy appears to offer the best potential benefit regarding outcome.

Adolescent↗