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Darshan Dalal

Publications and source records attributed to Darshan Dalal.

At least 19 recordsLinked to original sources
PubMed2006

Magnetic resonance imaging of arrhythmogenic right ventricular dysplasia: sensitivity, specificity, and observer variability of fat detection versus functional analysis of the right ventricle.

OBJECTIVES: The purpose of this study was to determine interobserver agreement for interpretation of magnetic resonance imaging (MRI) examinations of arrhythmogenic right ventricular dysplasia (ARVD) and to determine sensitivity and specificity of fat detection versus functional parameters measured by MRI. BACKGROUND: The interobserver variability of MRI and the relative importance of different MRI parameters (fat detection, regional and global right ventricular [RV] function) for ARVD diagnosis is unknown. METHODS: Two experienced observers blinded to the clinical history independently analyzed MRI datasets obtained from 40 patients evaluated for ARVD. Twenty normal subjects underwent MRI and served as control subjects. The MRI scans were performed according to a standard protocol on a 1.5-T scanner. The observers reported on fat infiltration, global and regional RV function, myocardial thinning, and chamber dilatation qualitatively. The RV volumes were measured on the cine sequences. RESULTS: Interobserver kappa scores for fat infiltration, global and regional RV function, wall thinning, and RV outflow dilatation were 0.74, 0.94, 0.89, 0.93, and 0.93, respectively. Correlation coefficients between observers for RV end-diastolic volume, end-systolic volume, and ejection fraction were 0.93, 0.94, and 0.95, respectively (p < 0.001). Fifteen patients were diagnosed with ARVD using Task Force criteria. Sensitivity of fat infiltration, RV enlargement, and regional RV dysfunction for diagnosing ARVD was 84%, 68%, and 78%, and specificity was 79%, 96%, and 94%, respectively. CONCLUSIONS: Qualitative assessment of RV structure and function is highly reproducible for experienced observers. Among the qualitative parameters, fat infiltration is less reproducible and lacks specificity compared with RV kinetic abnormalities.

Adipose Tissue↗
PubMed2006

Penetrance of mutations in plakophilin-2 among families with arrhythmogenic right ventricular dysplasia/cardiomyopathy.

OBJECTIVES: The purpose of our study was to characterize the penetrance of PKP2 mutations among family members of people with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) and to examine clinical features and predictors of disease among PKP2 mutation carriers. BACKGROUND: Arrhythmogenic right ventricular dysplasia/cardiomyopathy is an inherited cardiomyopathy characterized by fatty-fibrous myocardial replacement of the right ventricle, ventricular arrhythmias, and right ventricular dysfunction. Mutations in PKP2, the gene encoding plakophilin-2, are found in 11% to 43% of ARVD/C probands. METHODS: The study population was composed of 64 individuals in 9 families with an ARVD/C proband previously shown to carry a pathogenic PKP2 mutation. The diagnosis of ARVD/C was established based on task force criteria (TFC) set by the European Society of Cardiology. RESULTS: In addition to the probands, PKP2 mutations were present in 52% of relatives screened. Forty-nine percent of PKP2 mutation carriers met TFC. Among mutation carriers who did not meet full TFC, 50% met at least some TFC criteria besides family history. Pedigrees showed wide intra-familial variability, ranging from severe disease with early death to individuals who were completely asymptomatic late in life. Male PKP2 mutation carriers were more likely to have structural and conduction abnormalities as determined by imaging studies, signal-averaged electrocardiography, and 24-h ambulatory electrocardiography (p < 0.05). CONCLUSIONS: PKP2 mutations in a group of North American families with ARVD/C have both reduced penetrance and variable expressivity. Gender may have an influence on penetrance of PKP2 mutations, with male mutation carriers more likely to develop specific phenotypic manifestations of this disease.

Adolescent↗
PubMed2006

Long-term single procedure efficacy of catheter ablation of atrial fibrillation.

BACKGROUND: Two important limitations of the data regarding the outcomes of catheter ablation of atrial fibrillation (AF) are the short-term follow-up used in most published studies and the lack of single-procedure outcomes. OBJECTIVE: The objective was to report the long-term single-procedure outcomes at our center. MATERIALS AND METHODS: The patient population was comprised of 200 consecutive patients who underwent ablation (133 men; age 56 +/- 11 years). Atrial fibrillation was paroxysmal in 92 (46%). Success was defined as absence of symptomatic AF, off antiarrhythmic drug (AAD) after a single procedure. RESULTS: After a follow-up of 26 +/- 11 months, the single-procedure long-term success rate was 28% with an additional 7% of patients demonstrating improvement. After including repeat procedures in 64 patients, the overall long-term success rate was 41% with 11% demonstrating improvement. Further subgroup analysis of 48 paroxysmal AF patients considered to be optimal candidates for the procedure, revealed a long-term success rate of 69% with an additional 4% demonstrating improvement. A major complication occurred in 7.9% of patients. CONCLUSION: The results reveal that the long-term single-procedure success rate of catheter ablation of AF in a cohort of patients with predominantly non-paroxysmal AF is less than 40%. The inclusion of redo procedures resulted in an improvement in outcomes. A much higher success rate of 69% was achieved in patients with paroxysmal AF considered to be optimal candidates for this procedure. These results make it clear that further advances in the technique of catheter ablation of AF are needed to improve the safety and efficacy of this procedure. In order to be able to compare outcomes of various techniques in differing patient populations, we urge investigators to report long-term single procedure outcomes.

Atrial Fibrillation↗
PubMed2006

Prospective randomized study comparing amiodarone vs. amiodarone plus losartan vs. amiodarone plus perindopril for the prevention of atrial fibrillation recurrence in patients with lone paroxysmal atrial fibrillation.

AIMS: The purpose of this trial was to compare the long-term efficacy of low-dose amiodarone with losartan and perindopril (both combined with low-dose amiodarone) for the prevention of atrial fibrillation (AF) recurrence in patients with lone paroxysmal AF. METHODS AND RESULTS: One-hundred and seventy-seven patients with lone paroxysmal AF were randomly assigned to three treatment groups: group 1 received low-dose amiodarone alone, group 2 received low-dose amiodarone plus losartan, and group 3 received low-dose amiodarone plus perindopril. Left atrial diameter was measured with transthoracic echocardiogram at baseline and 6, 12, 18, and 24 months after randomization. The primary endpoint was the incidence of AF documented by 12-lead ECG or Holter after 14 days and within 24 months after randomization. The primary endpoint was reached in 24 patients (41%) in group 1, 11 (19%) in group 2, and 14 (24%) in group 3 (P = 0.02). The Kaplan-Meier survival analysis demonstrated a significant reduction in AF recurrence in group 2 (P = 0.006, log-rank test) as well as in group 3 (P = 0.04, log-rank test) when compared with group 1. No difference in the AF recurrence-free survival was found between group 2 and group 3. After 24 months follow-up, the left atrial diameter in group 2 and group 3 was significantly smaller than that in group 1 (36 +/- 2.3 and 35 +/- 2.4 vs. 38 +/- 2.4 mm, P < 0.001 for both comparisons). CONCLUSION: The results of this study suggest that the combination of perindopril or losartan with low-dose amiodarone is more effective than low-dose amiodarone alone for the prevention of AF recurrence in patients with lone paroxysmal AF. Adding losartan or perindopril to amiodarone can inhibit left atrial enlargement in this group of patients.

Amiodarone↗
PubMed2006

DSG2 mutations contribute to arrhythmogenic right ventricular dysplasia/cardiomyopathy.

Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a disorder characterized by fibrofatty replacement of cardiac myocytes that typically manifests in the right ventricle. It is inherited as an autosomal dominant disease with reduced penetrance, although autosomal recessive forms of the disease also occur. We identified four probands with ARVD/C caused by mutations in DSG2, which encodes desmoglein-2, a component of the cardiac desmosome. No association between mutations in this gene and human disease has been reported elsewhere. One of these probands has compound-heterozygous mutations in DSG2, and the remaining three have isolated heterozygous missense mutations, each disrupting known functional components of desmoglein-2. We report that mutations in DSG2 contribute to the development of ARVD/C.

Amino Acid Sequence↗
PubMed2006

Clinical features of arrhythmogenic right ventricular dysplasia/cardiomyopathy associated with mutations in plakophilin-2.

BACKGROUND: Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is an inherited cardiomyopathy characterized by right ventricular dysfunction and ventricular arrhythmias. A recent study reported mutations in PKP2, encoding the desmosomal protein plakophilin-2, associated with ARVD/C. The purpose of our study was to validate the frequency of PKP2 mutations in another large series of ARVD/C patients and to examine the phenotypic characteristics associated with PKP2 mutations. METHODS AND RESULTS: DNA from 58 ARVD/C patients was sequenced to determine the presence of mutations in PKP2. Clinical features of ARVD/C were compared between 2 groups of patients: those with a PKP2 mutation and those with no detectable PKP2 mutation. Thirteen different PKP2 mutations were identified in 25 (43%) of the patients. Six of these mutations have not been reported previously; 4 occurred in multiple, apparently unrelated, families. The mean age at presentation was lower among those with a PKP2 mutation (28+/-11 years) than in those without (36+/-16 years) (P<0.05). The age at median cumulative symptom-free survival (32 versus 42 years) and at the median cumulative arrhythmia-free survival (34 versus 46 years) was lower among patients with a PKP2 mutation than among those without a PKP2 mutation (P<0.05). Inducibility of ventricular arrhythmias on an electrophysiology study, diffuse nature of right ventricular disease, and presence of prior spontaneous ventricular tachycardia were identified as predictors of implanted cardioverter/defibrillator (ICD) intervention only among patients without a PKP2 mutation (P<0.05). CONCLUSIONS: Our study highlights the clinical relevance of PKP2 mutations in ARVD/C. Presence of a PKP2 mutation in ARVD/C correlates with earlier onset of symptoms and arrhythmia. Patients with a PKP2 mutation experience ICD interventions irrespective of the classic risk factors determining ICD intervention in ARVD/C patients.

Adult↗
PubMed2006

Association of body mass index, metabolic syndrome, and leukocyte count.

Obesity and metabolic syndrome (MS), which often co-exist, are associated with an increased cardiovascular risk. An increased leukocyte count is also associated with an increased cardiovascular risk. However, the role of obesity, independent of MS, has been debated. We sought to assess the influence of MS on the association of obesity and leukocyte count in asymptomatic patients. The data from 431 asymptomatic Brazilian men (mean age 46 +/- 7 years), who presented for cardiovascular risk assessment, were analyzed. MS was defined as the presence of > or =3 of the following risk factors: hypertension (> or =130/85 mm Hg), truncal obesity (> or =102 cm or 40 in), hypertriglyceridemia (> or =150 mg/dl), high-density lipoprotein cholesterol (< or =40 mg/dl), and hyperglycemia (glucose > or =110 mg/dl). Obesity was defined as a body mass index of > or =30 kg/m(2). Confounding variables (age, smoking, lipid-lowering therapy, and physical activity) and leukocyte count (10(9)/L) were recorded. The patients were divided into 4 groups: group1, no obesity and no MS; group 2, obesity but no MS; group 3, no obesity but MS; and group 4, obesity and MS. The mean leukocyte count increased from groups 1 to 4 (6.10 +/- 0.09, 6.42 +/- 0.28, 6.71 +/- 0.21, and 6.96 +/- 0.22 x 10(9)/L, p < 0.001 for trend). Multivariate regression analysis demonstrated that the leukocyte count was significantly higher in groups 3 (coefficient 0.61, p = 0.007) and 4 (coefficient 0.86, p < 0.001) compared with group 1. However, no significant difference was found in the leukocyte count between groups 1 and 2 (coefficient 0.29, p = 0.42) and groups 3 and 4 (coefficient 0.25, p = 0.41). The association between obesity and leukocyte count was highly dependent on the presence of MS.

Body Mass Index↗
PubMed2006

Feasibility and variability of three dimensional echocardiography in arrhythmogenic right ventricular dysplasia/cardiomyopathy.

Arrhythmogenic right ventricular dysplasia (ARVD/C) is a genetic cardiomyopathy characterized by fibrous fatty replacement of the right ventricular (RV) myocardium, leading to progressive RV failure and ventricular arrhythmias in young athletes. This study evaluated whether transthoracic, real-time, 3-dimensional echocardiography (3DE) can adequately assess RV morphology and function in ARVD/C by comparing 3DE with cardiac magnetic resonance (CMR), the current reference standard. Three-dimensional echocardiography was prospectively performed in 58 patients (23 with ARVD/C, 20 first-degree relatives with no ARVD/C, 8 with idiopathic ventricular tachycardia with no ARVD/C, and 7 healthy volunteers). All patients, except 15 patients with ARVD/C with implanted defibrillators, also underwent CMR. Three-dimensional echocardiography and CMR-derived RV volumes and ejection fractions were obtained by offline data analysis by blinded, independent observers. The mean age of the study group was 37 +/- 11 years (30 men). The feasibility of 3DE was high, and analyzable images were obtained in all subjects. Three-dimensional echocardiography revealed a wide variety of RV morphologic abnormalities in ARVD/C. There was a good correlation between 3DE and CMR for RV end-systolic volume (r = 0.72, p = 0.0001), RV end-diastolic volume (r = 0.50, p = 0.0001), and the RV ejection fraction (r = 0.88, p = 0.001). We found high intraobserver and moderate interobserver correlations for 3DE estimations of volumes and ejection fractions. In conclusion, 3DE measurements of RV volumes and ejection fractions closely correlate with CMR values and may be useful in the follow-up of patients with ARVD/C.

Adult↗
PubMed2006

Integrated electroanatomic mapping with three-dimensional computed tomographic images for real-time guided ablations.

BACKGROUND: New ablation strategies for atrial fibrillation or nonidiopathic ventricular tachycardia are increasingly based on anatomic consideration and require the placement of ablation lesions at the correct anatomic locations. This study sought to evaluate the accuracy of the first clinically available image integration system for catheter ablation on 3-dimensional (3D) computed tomography (CT) images in real time. METHODS AND RESULTS: After midline sternotomy, 2.3-mm CT fiducial markers were attached to the epicardial surface of each cardiac chamber in 9 mongrel dogs. Detailed 3D cardiac anatomy was reconstructed from contrast-enhanced, high-resolution CT images and registered to the electroanatomic maps of each cardiac chamber. To assess accuracy, targeted ablations were performed at each of the fiducial markers guided only by the reconstructed 3D images. At autopsy, the position error was 1.9+/-0.9 mm for the right atrium, 2.7+/-1.2 mm for the right ventricle, 1.8+/-1.0 mm for the left atrium, and 2.3+/-1.1 mm for the left ventricle. To evaluate the system's guidance of more complex clinical ablation strategies, ablations of the cavotricuspid isthmus (n=4), fossa ovalis (n=4), and pulmonary veins (n=6) were performed, which resulted in position errors of 1.8+/-1.5, 2.2+/-1.3, and 2.1+/-1.2 mm, respectively. Retrospective analysis revealed that a combination of landmark registration and the target chamber surface registration resulted in <3 mm accuracy in all 4 cardiac chambers. CONCLUSIONS: Image integration with high-resolution 3D CT allows accurate placement of anatomically guided ablation lesions and can facilitate complex ablation strategies. This may provide significant advantages for anatomically based procedures such as ablation of atrial fibrillation and nonidiopathic ventricular tachycardia.

Animals↗
PubMed2006

Recessive arrhythmogenic right ventricular dysplasia due to novel cryptic splice mutation in PKP2.

Arrhythmogenic right ventricular dysplasia (ARVD) is a genetic disorder resulting in fibro-fatty replacement of right ventricular myocytes and consequent ventricular arrhythmias. Heterozygous mutations in PKP2 encoding plakophilin-2 have previously been reported to cause dominant ARVD with reduced penetrance. We report the first case of recessive ARVD caused by mutations in PKP2. Candidate gene analysis in a typical proband with this disorder identified a novel homozygous mutation in PKP2 (c.[2484C>T]+[2484C>T]), which is predicted to be translationally silent (p.Gly828). Analysis of the proband's mRNA, however, shows that this mutation causes predominantly cryptic splicing, with a 7-nucleotide deletion in exon 12. The ensuing frame shift disrupts the last 54 amino acids of plakophilin-2 and extends the open reading frame by 145 nucleotides (48 amino acids) into the 3' untranslated region. Haplotype analysis demonstrates the absence of remote consanguinity. Heterozygous family members produce approximately 60% of properly spliced PKP2 and do not have manifestations of ARVD. Further analysis of PKP2 mRNA sequence revealed two additional alternatively spliced transcripts. The possibility of cryptic or alternative splicing should be considered with identification of apparently synonymous nucleotide substitutions in this gene.

Adult↗
PubMed2006

Incidence of atherosclerotic cardiovascular disease among HIV patients receiving dialysis.

BACKGROUND: Atherosclerotic cardiovascular disease (ASCVD) is increasing in incidence in persons with human immunodeficiency virus (HIV). It is not known whether the morbidity and mortality rate of ASCVD is similarly increasing among patients with HIV end-stage renal disease (ESRD), who are living longer since the initiation of newer therapeutic regimens in 1997. METHODS: We conducted a population-based study of 8,312 HIV ESRD patients enrolled in the US Renal Data System from 1987 to 2001. We determined the incidence of fatal and nonfatal ASCVD events stratified by date of dialysis therapy initiation. RESULTS: There were 3,112 HIV ESRD patients (77.9% men; mean age, 39.6 +/- 9.6 years) who initiated dialysis therapy before 1997 and 2,371 patients (71.5% men; mean age, 41.3 +/- 9.7 years) who initiated dialysis therapy after 1997. Cumulative incidences of ASCVD for those initiating dialysis therapy before 1997 compared with after 1997 were 10.6% versus 13.5% at 12 months, 18.8% versus 27.6% at 24 months, 26.2% versus 42.4% at 36 months, and 33.7% versus 65.9% at 48 months (P < 0.0001 by log rank). Those who initiated dialysis therapy after 1997 had a greater risk for developing ASCVD (adjusted hazard ratio, 1.57; 95% confidence interval, 1.29 to 1.92) than those who initiated dialysis therapy before 1997 after adjusting for demographics and ASCVD risk factors. CONCLUSION: There is an increased risk for ASCVD in patients with HIV who initiated dialysis therapy after 1997. This may reflect the long-term complications of multidrug regimens, as well as improved survival from these regimens leading to prolonged exposure to ASCVD risk factors.

Adult↗
PubMed2006

Symptomatic and asymptomatic atrial fibrillation in patients undergoing radiofrequency catheter ablation.

INTRODUCTION: The goals of this study were to (i) evaluate the feasibility and results of monitoring patients prior to and following catheter ablation of AF with the mobile cardiac outpatient telemetry (MCOT) system and to (ii) correlate symptoms and the presence or absence of atrial fibrillation (AF). METHODS AND RESULTS: A total of 19 consecutive patients (12 men [63%]; age 60 +/- 6 years) with highly symptomatic drug refractory AF underwent catheter ablation. Each was provided with a MCOT monitor (CardioNet, USA) and asked to wear it 5 days immediately before the ablation, and 5 days per month starting with the ablation for 6 consecutive months. When patients experienced any symptoms, they were asked to activate the system and to record associated symptoms. A total of 494 days (11,856 hours) were monitored. Out of the total 390 events triggered by patient's symptoms, 40% were confirmed as AF events (156) and 60% were confirmed as non-AF events (234). Only shortness of breath and chest discomfort were highly associated with AF (P < 0.05). At the end of 6 months of follow-up, out of 10 patients that completed the study, 7 (70%) patients were free of symptomatic AF recurrences whereas only 5 (50%) patients achieved success when asymptomatic AF recurrences were included in the outcome. CONCLUSIONS: The results of this study demonstrate the potential utility of wireless monitoring systems in the follow-up of AF ablation patients, while also identifying poor patient compliance with an intensive monitoring protocol as an important limitation.

Atrial Fibrillation↗
PubMed2006

Initial experience in the use of integrated electroanatomic mapping with three-dimensional MR/CT images to guide catheter ablation of atrial fibrillation.

INTRODUCTION: No prior studies have reported the use of integrated electroanatomic mapping with preacquired magnetic resonance/computed tomographic (MR/CT) images to guide catheter ablation of atrial fibrillation (AF) in a series of patients. METHODS AND RESULTS: Sixteen consecutive patients with drug-refractory AF underwent catheter ablation under the guidance of a three-dimensional (3D) electroanatomic mapping system (Carto, Biosense Webster, Inc., Diamond Bar, CA, USA). Gadolinium-enhanced MR (n = 8) or contrast-enhanced high-resolution CT (n = 8) imaging was performed within 1 day prior to the ablation procedures. Using a novel software package (CartoMerge, Biosense Webster, Inc.), the left atrium (LA) with pulmonary veins (PVs) was segmented and extracted for image registration. The segmented 3D MR/CT LA reconstruction was accurately registered to the real-time mapping space with a combination of landmark registration and surface registration. The registered 3D MR/CT LA reconstruction was successfully used to guide deployment of RF applications encircling the PVs. Upon completion of the circumferential lesions around the PVs, 32% of the PVs were electrically isolated. Guided by a circular mapping catheter, the remaining PVs were disconnected from the LA using a segmental approach. The distance between the surface of the registered 3D MR/CT LA reconstruction and multiple electroanatomic map points was 3.05 +/- 0.41 mm. No complications were observed. CONCLUSIONS: Three-dimensional MR/CT images can be successfully extracted and registered to anatomically guided clinical AF ablations. The display of detailed and accurate anatomic information during the procedure enables tailored RF ablation to individual PV and LA anatomy.

Atrial Fibrillation↗
PubMed2006

Long-term safety and efficacy of circumferential ablation with pulmonary vein isolation.

BACKGROUND: Each of the two main approaches to catheter ablation of atrial fibrillation (AF, segmental and circumferential) is associated with moderate long-term efficacy. OBJECTIVE: To report the long-term outcomes of a modified technique that combines circumferential ablation with pulmonary vein (PV) isolation, determined by a circular mapping catheter and to determine the relationship between complete PV isolation and long-term efficacy. METHODS: The patient population was composed of 64 consecutive patients (47 men [73%]; age 59 +/- 11 years) with AF who underwent catheter ablation. AF was paroxysmal in 29 (45%) and nonparoxysmal in 35 (55%). Each patient was followed for a minimum of 12 months. RESULTS: After a mean follow-up of 13 +/- 1 months, the long-term single-procedure success rate was 45% (n = 29) with an additional 4% (n = 3) of patients demonstrating improvement. With repeat procedures in 19 patients, the overall long-term success rate was 62% (n = 40) with 9% (n = 6) demonstrating improvement. All the patients who underwent repeat ablations had recovered PV conduction. Incomplete PV isolation was the only independent predictor of failure. A major complication occurred in four (6%) patients, including three patients with vascular complications and one with cardiac tamponade. CONCLUSION: Our results suggest that the long-term single-procedure efficacy of circumferential ablation with PV isolation in a cohort of patients with predominantly nonparoxysmal AF approaches 50%. Repeat procedures involving re-isolation of the PVs result in a significant improvement in outcomes. Complete electrical isolation of the PVs has a significant impact on the long-term efficacy of the procedure.

Atrial Fibrillation↗
PubMed2005

Arrhythmogenic right ventricular dysplasia: a United States experience.

BACKGROUND: Arrhythmogenic right ventricular dysplasia (ARVD) is an inherited cardiomyopathy characterized by right ventricular dysfunction and ventricular arrhythmias. The purpose of our study was to describe the presentation, clinical features, survival, and natural history of ARVD in a large cohort of patients from the United States. METHODS AND RESULTS: The patient population included 100 ARVD patients (51 male; median age at presentation, 26 [interquartile range {IQR}, 18 to 38; range, 2 to 70] years). A familial pattern was observed in 32 patients. The most common presenting symptoms were palpitations, syncope, and sudden cardiac death (SCD) in 27%, 26%, and 23% of patients, respectively. Among those who were diagnosed while living (n=69), the median time between first presentation and diagnosis was 1 (range, 0 to 37) year. During a median follow-up of 6 (IQR, 2 to 13; range, 0 to 37) years, implantable cardioverter/defibrillators (ICD) were implanted in 47 patients, 29 of whom received an appropriate ICD discharge, including 3 patients who received the ICD for primary prevention. At follow-up, 66 patients were alive, of whom 44 had an ICD in place, 5 developed signs of heart failure, 2 had a heart transplant, and 18 were on drug therapy. Thirty-four patients died either at presentation (n=23: 21 SCD, 2 noncardiac deaths) or during follow-up (n=11: 10 SCD, 1 of biventricular heart failure), of whom only 3 were diagnosed while living and 1 had an ICD implanted. On Kaplan-Meier analysis, the median survival in the entire population was 60 years. CONCLUSIONS: ARVD patients present between the second and fifth decades of life either with symptoms of palpitations and syncope associated with ventricular tachycardia or with SCD. Diagnosis is often delayed. Once diagnosed and treated with an ICD, mortality is low. There is a wide variation in presentation and course of ARVD patients, which can likely be explained by the genetic heterogeneity of the disease.

Adolescent↗
PubMed2005

Regional differences in systolic and diastolic function in arrhythmogenic right ventricular dysplasia/cardiomyopathy using magnetic resonance imaging.

Global and regional biventricular functions were analyzed in 14 patients diagnosed with arrhythmogenic right ventricular dysplasia/cardiomyopathy using cine magnetic resonance imaging and compared with similar data from 18 age-matched controls. In this study, we report results of quantitative evaluation of biventricular global and regional function using peak ejection rate and peak filling rate as measures of systolic and diastolic function, respectively (volumetric method).

Adolescent↗
PubMed2005

Relation of serum uric acid with metabolic risk factors in asymptomatic middle-aged Brazilian men.

This study in 352 asymptomatic middle-aged Brazilian men demonstrated that serum uric acid increases linearly with an increasing number (0 to >/=3) of metabolic risk factors (5.78 +/- 1.1, 6.14 +/- 1.0, 6.27 +/- 1.1, and 6.79 +/- 1.3, p <0.001). In patients who had >/=3 metabolic risk factors, there was a higher prevalence of serum uric acid in the highest quartile (7.2 to 10.3 mg/dl) than in the lowest quartile (2.6 to 5.4 mg/dl, 35% vs 12%, p <0.001). Mean serum levels of uric acid were higher in those who had an abnormal ratio of >/=3 for triglyceride to high-density lipoprotein (suggesting insulin resistance) than in those who had a normal ratio (6.6 +/- 1.2 vs 5.87 +/- 1 mg/dl, p <0.001).

Adult↗