Images in cardiovascular medicine. Scimitar syndrome: complete diagnosis by transthoracic echocardiography.
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Echocardiography, Transesophageal↗
Biomedical subjects
A Jamil Tajik
Publications and source records attributed to A Jamil Tajik.
Surgical correction of mitral regurgitation in the elderly: outcomes and recent improvements.
BACKGROUND: In the elderly, mitral regurgitation (MR) is frequent, but surgery risks are considered high. Benefits and indications of MR surgery are uncertain in the elderly. METHODS AND RESULTS: Baseline characteristics, outcome, and trends for surgical results improvement were analyzed in elderly patients (> or = 75 years of age; n=284) operated on for MR in 1980 to 1995 compared with younger patients (65 to 74 years of age, n=504; and <65 years of age, n=556). Preoperatively, class III to IV symptoms, atrial fibrillation, coronary disease, creatinine, and comorbidity index were more severe in elderly patients (all P<0.002). In the long term after surgery, observed survival stratified by age (> or = 75, 65 to 74, <65 years) was lower in elderly than in younger patients (at 5 years, 57+/-3%, 73+/-2%, and 85+/-2%, respectively; P<0.001), but ratios of observed to expected survival were similar (83%, 85%, and 88%, respectively). In multivariate analysis adjusted to expected survival, elderly patients showed no difference in life expectancy restoration compared with younger patients (adjusted hazard ratio, 0.89; 95% confidence interval, 0.73 to 1.30; P=0.54). Temporal trends showed that risk of operative mortality, although higher in elderly patients (P<0.001), declined markedly for all ages (27% to 5% in those > or = 75 years of age, P<0.01; 21% to 4% in those 65 to 74 years of age, P<0.01; and 7% to 2% in those <65 years of age, P=0.06), with a parallel decline in low cardiac output and length of hospital stay. Over time, valve repair feasibility increased in all age groups (30% to 84% overall and 31% to 93% in degenerative MR; P<0.0001). CONCLUSIONS: Elderly patients undergoing MR surgery display more severe preoperative characteristics and incur higher operative risks than younger patients. However, restoration of life expectancy after surgery is similar in elderly and younger patients, and outstanding recent surgical improvements particularly benefited elderly patients. Thus, elderly patients with MR can now carefully be considered for surgery before refractory heart failure is present.
Age Factors↗
Left atrial size: physiologic determinants and clinical applications.
Left atrial (LA) enlargement has been proposed as a barometer of diastolic burden and a predictor of common cardiovascular outcomes such as atrial fibrillation, stroke, congestive heart failure, and cardiovascular death. It has been shown that advancing age alone does not independently contribute to LA enlargement, and the impact of gender on LA volume can largely be accounted for by the differences in body surface area between men and women. Therefore, enlargement of the left atrium reflects remodeling associated with pathophysiologic processes. In this review, we discuss the normal size and phasic function of the left atrium. Further, we outline the clinically important aspects and pitfalls of evaluating LA size, and the methods for assessing LA function using echocardiography. Finally, we review the determinants of LA size and remodeling, and we describe the evidence regarding the prognostic value of LA size. The use of LA volume for risk stratification is an evolving science. More data are required with respect to the natural history of LA remodeling in disease, the degree of LA modifiability with therapy, and whether regression of LA size translates into improved cardiovascular outcomes.
Atrial Function↗
Transient midventricular ballooning syndrome: a new variant.
We describe a new variant of transient left ventricular (LV) ballooning in North American Caucasian patients in which only the midventricle is affected. The patients described in this case series initially presented with emotional or physical stress and had similarities to transient apical ballooning syndrome; however, this variant is unique in that the transient ballooning involves the midventricle with hypercontractility of the apical and basal segments. The presentation, clinical features, and transient nature of the reported cases in this series are similar to transient LV apical ballooning and suggest a shared pathophysiologic etiology. Sparing of the apical segment with involvement of midventricle only supports etiologies not related to an epicardial coronary artery distribution. Although the pathophysiologic mechanism of the transient ventricular ballooning syndromes and other cases of catecholamine-associated transient ventricular dysfunction are not well understood, the emergence of this new variant raises further questions in the understanding of the "brain-heart" relationship.
Aged↗
Cardiopulmonary exercise testing determination of functional capacity in mitral regurgitation: physiologic and outcome implications.
OBJECTIVES: This study was designed to evaluate prevalence, determinants, and clinical outcome implications of reduced functional capacity (FC) in patients with organic mitral regurgitation (MR). BACKGROUND: Evaluation of FC by exercise testing is rarely performed in MR because little is known about the clinical determinants and outcome implications of FC. METHODS: Cardiopulmonary exercise testing (CPET) was prospectively performed in 134 asymptomatic patients with organic MR to assess FC (peak oxygen consumption [VO2]) simultaneously to Doppler-echocardiographic quantitation of MR (effective regurgitant orifice [ERO]) and left ventricular (LV) systolic and diastolic function. RESULTS: Peak VO2 was 26 +/- 6 ml/kg/min (96 +/- 16% of age-predicted), but varied widely (57% to 145% of predicted) and was markedly reduced (< or =84% of predicted) in 19% of patients. Although ERO of MR was univariately associated with reduced FC (26 vs. 9% with ERO > or =40 vs. <40 mm2), independent determinants of reduced FC were LV diastolic function (higher E/E' ratio, p = 0.006), atrial fibrillation (p = 0.01), and lower forward stroke volume (p = 0.03). Clinical events (death, heart failure, new atrial fibrillation) and clinical events or surgery were more frequent with than without reduced FC (3 years, 36 +/- 14% vs. 13 +/- 4%, p = 0.02; and 66 +/- 11% vs. 29 +/- 5%, p = 0.001, respectively), even adjusting (risk ratios 1.80 and 1.54 respectively, both p < or = 0.03) for age and ERO. CONCLUSIONS: In asymptomatic organic MR, FC quantitatively assessed by CPET is unexpectedly markedly reduced in one out of every four to five patients. Reduced FC is independently determined by consequences rather than severity of MR and predicts increased subsequent clinical events. Therefore, CPET frequently reveals functional limitations not detected clinically and is an important tool in managing patients with organic MR.
Exercise Test↗
Diastolic heart failure can be diagnosed by comprehensive two-dimensional and Doppler echocardiography.
There are many myocardial and non-myocardial conditions that cause heart failure with normal left ventricular ejection fraction (LVEF). Among them, diastolic heart failure (heart failure due to diastolic dysfunction) is the most common cause of heart failure with normal LVEF. Diastolic heart failure easily can be diagnosed by comprehensive two-dimensional and Doppler echocardiography, which can demonstrate abnormal myocardial relaxation, decreased compliance, and increased filling pressure in the setting of normal LV dimensions and preserved LVEF. Therefore, diastolic heart failure should always be considered when LVEF is normal on two-dimensional echocardiography in patients with clinical evidence of heart failure. The diagnosis can be confirmed if Doppler echocardiography and myocardial tissue imaging provide evidence for impaired myocardial relaxation (i.e., decreased longitudinal velocity of the mitral annulus during early diastole and decreased propagation velocity mitral inflow), decreased compliance (shortened mitral A-wave duration and mitral deceleration time), and increased filling pressure (shortened isovolumic relaxation time and an increased ratio between early diastolic mitral and mitral annular velocities). Early identification of diastolic dysfunction in asymptomatic patients by the use of echocardiography may provide an opportunity to manage the underlying etiology to prevent progression to diastolic heart failure.
Blood Flow Velocity↗
Use of echocardiography in patients with hypertrophic cardiomyopathy: clinical implications of massive hypertrophy.
We examined the 10-year outcome of 107 patients with hypertrophic cardiomyopathy and massive myocardial hypertrophy as assessed by echocardiography. These patients demonstrated excess mortality in comparison with their expected survival with respect to a general population of individuals (10-year survival 77% vs 95%, P < .001). Adverse events occurred primarily in the youngest and oldest patients. Sudden cardiac death was the most frequent cause of death among the young (overall survival 80%), whereas other causes predominated in older patients (overall survival 31%). For patients aged 30 to 59 years, survival was 97%, no different from their expected survival. Patients with hypertrophic cardiomyopathy and massive hypertrophy identified by echocardiography are at increased risk of death, but this risk is related to age at presentation. Sudden cardiac death is the primary cause in those who are young, whereas other causes predominate in older patients. Middle-aged patients with massive hypertrophy do not experience an excess mortality.
Adult↗
Echocardiography-guided genetic testing in hypertrophic cardiomyopathy: septal morphological features predict the presence of myofilament mutations.
OBJECTIVE: To examine the relationship among age, septal morphological subtype, and presence of hypertrophic cardiomyopathy (HCM)-associated myofilament mutations. PATIENTS AND METHODS: Comprehensive mutation analysis of the 8 HCM susceptibility genes that encode the myofilaments of the cardiac sarcomere was performed previously in 382 unrelated patients with HCM. Blinded to genotype status, we used echocardiography to characterize the left ventricular morphological features. Multivariate regression was used to assess the relationship among morphological subtypes, clinical data, and genetic variables. RESULTS: The mean +/- SD age of the patients was 41.6+/-19.0 years, with 126 patients 50 years or older at initial diagnosis. The septal morphological subtype was sigmold in 181 (47%), reverse in 132 (35%), apical variant in 37 (10%), and neutral in 32 (8%). The HCM-associated myofilament mutations were Identified in 143 patients (37%). Multivariate analysis showed that the reverse curvature septal morphological subtype was a strong predictor of genotype-positive status (odds ratio, 21; P<.001). Overall, the yield of HCM genetic testing was 79% in the setting of reverse curvature HCM but only 8% in sigmold septal HCM. CONCLUSION: In stark contrast to HCM in young patients, elderly patients with HCM display a predominantly sigmoid septal morphological subtype and uncommonly have perturbations of known HCM susceptibility genes. Independent of age, septal morphological subtype strongly predicts the presence or absence of HCM-associated myofilament mutations and may enable echocardiography-guided genetic testing for HCM.
Actin Cytoskeleton↗
Sinus venosus atrial septal defect: long-term postoperative outcome for 115 patients.
BACKGROUND: Sinus venosus atrial septal defect (SVASD) differs from secundum atrial septal defect by its atrial septal location and its association with anomalous pulmonary venous connection (APVC). Data on long-term outcome after surgical repair are limited. METHODS AND RESULTS: We reviewed outcomes of 115 patients (mean age+/-SD 34+/-23 years) with SVASD who had repair from 1972 through 1996. APVC was present in 112 patients (97%). Early mortality was 0.9%. Complete follow-up was obtained for 108 patients (95%) at 144+/-99 months. Symptomatic improvement was noted in 83 patients (77%), and deterioration was noted in 17 patients (16%). At follow-up, 7 (6%) of 108 patients had sinus node dysfunction, a permanent pacemaker, or both, and 15 (14%) of 108 patients had atrial fibrillation. Older age at repair was predictive of postoperative atrial fibrillation (P=0.033). No reoperations were required during follow-up. Survival was not different from expected for an age- and sex-matched population. Clinical improvement was more common with older age at surgery (P=0.014). Older age at repair (P=0.008) and preoperative New York Heart Association class III or IV (P=0.038) were independent predictors of late mortality. CONCLUSIONS: Operation for SVASD is associated with low morbidity and mortality, and postoperative subjective clinical improvement occurs irrespective of age at surgery. Postoperative atrial fibrillation appears to be related to older age at operation. SVASD repair achieves survival similar to that of a matched population and should be considered whenever repair may impact survival or symptoms.
Adolescent↗
Long-term effects of surgical septal myectomy on survival in patients with obstructive hypertrophic cardiomyopathy.
OBJECTIVES: This study sought to determine the impact of surgical myectomy on long-term survival in hypertrophic cardiomyopathy (HCM). BACKGROUND: Left ventricular (LV) outflow tract obstruction in HCM increases the likelihood of heart failure and cardiovascular death. Although surgical myectomy is the primary treatment for amelioration of outflow obstruction and advanced drug-refractory heart failure symptoms, its impact on long-term survival remains unresolved. METHODS: Total and HCM-related mortality were compared in three subgroups comprised of 1,337 consecutive HCM patients evaluated from 1983 to 2001: 1) surgical myectomy (n = 289); 2) LV outflow obstruction without operation (n = 228); and 3) nonobstructive (n = 820). Mean follow-up duration was 6 +/- 6 years. RESULTS: Including two operative deaths (procedural mortality, 0.8%), 1-, 5-, and 10-year overall survival after myectomy was 98%, 96%, and 83%, respectively, and did not differ from that of the general U.S. population matched for age and gender (p = 0.2) nor from patients with nonobstructive HCM (p = 0.8). Compared to nonoperated obstructive HCM patients, myectomy patients experienced superior survival free from all-cause mortality (98%, 96%, and 83% vs. 90%, 79%, and 61%, respectively; p < 0.001), HCM-related mortality (99%, 98%, and 95% vs. 94%, 89%, and 73%, respectively; p < 0.001), and sudden cardiac death (100%, 99%, and 99% vs. 97%, 93%, and 89%, respectively; p = 0.003). Multivariate analysis showed myectomy to have a strong, independent association with survival (hazard ratio 0.43; p < 0.001). CONCLUSIONS: Surgical myectomy performed to relieve outflow obstruction and severe symptoms in HCM was associated with long-term survival equivalent to that of the general population, and superior to obstructive HCM without operation. In this retrospective study, septal myectomy seems to reduce mortality risk in severely symptomatic patients with obstructive HCM.
Adult↗
Outcome of 622 adults with asymptomatic, hemodynamically significant aortic stenosis during prolonged follow-up.
BACKGROUND: This study assessed the long-term outcome of a large, asymptomatic population with hemodynamically significant aortic stenosis (AS). METHODS AND RESULTS: We identified 622 patients with isolated, asymptomatic AS and peak systolic velocity > or =4 m/s by Doppler echocardiography who did not undergo surgery at the initial evaluation and obtained follow-up (5.4+/-4.0 years) in all. Mean age (+/-SD) was 72+/-11 years; there were 384 (62%) men. The probability of remaining free of cardiac symptoms while unoperated was 82%, 67%, and 33% at 1, 2, and 5 years, respectively. Aortic valve area and left ventricular hypertrophy predicted symptom development. During follow-up, 352 (57%) patients were referred for aortic valve surgery and 265 (43%) patients died, including cardiac death in 117 (19%). The 1-, 2-, and 5-year probabilities of remaining free of surgery or cardiac death were 80%, 63%, and 25%, respectively. Multivariate predictors of all-cause mortality were age (hazard ratio [HR], 1.05; P<0.0001), chronic renal failure (HR, 2.41; P=0.004), inactivity (HR, 2.00; P=0.001), and aortic valve velocity (HR, 1.46; P=0.03). Sudden death without preceding symptoms occurred in 11 (4.1%) of 270 unoperated patients. Patients with peak velocity > or =4.5 m/s had a higher likelihood of developing symptoms (relative risk, 1.34) or having surgery or cardiac death (relative risk, 1.48). CONCLUSIONS: Most patients with asymptomatic, hemodynamically significant AS will develop symptoms within 5 years. Sudden death occurs in approximately 1%/y. Age, chronic renal failure, inactivity, and aortic valve velocity are independently predictive of all-cause mortality.
Aged↗
Quantitative determinants of the outcome of asymptomatic mitral regurgitation.
BACKGROUND: The clinical outcome of asymptomatic mitral regurgitation is poorly defined, and the treatment is uncertain. We studied the effect on the outcome of quantifying mitral regurgitation according to recent guidelines. METHODS: We prospectively enrolled 456 patients (mean [+/-SD] age, 63+/-14 years; 63 percent men; ejection fraction, 70+/-8 percent) with asymptomatic organic mitral regurgitation, quantified according to current recommendations (regurgitant volume, 66+/-40 ml per beat; effective regurgitant orifice, 40+/-27 mm2). RESULTS: The estimated five-year rates (+/-SE) of death from any cause, death from cardiac causes, and cardiac events (death from cardiac causes, heart failure, or new atrial fibrillation) with medical management were 22+/-3 percent, 14+/-3 percent, and 33+/-3 percent, respectively. Independent determinants of survival were increasing age, the presence of diabetes, and increasing effective regurgitant orifice (adjusted risk ratio per 10-mm2 increment, 1.18; 95 percent confidence interval, 1.06 to 1.30; P<0.01), the predictive power of which superseded all other qualitative and quantitative measures of regurgitation. Patients with an effective regurgitant orifice of at least 40 mm2 had a five-year survival rate that was lower than expected on the basis of U.S. Census data (58+/-9 percent vs. 78 percent, P=0.03). As compared with patients with a regurgitant orifice of less than 20 mm2, those with an orifice of at least 40 mm2 had an increased risk of death from any cause (adjusted risk ratio, 2.90; 95 percent confidence interval, 1.33 to 6.32; P<0.01), death from cardiac causes (adjusted risk ratio, 5.21; 95 percent confidence interval, 1.98 to 14.40; P<0.01), and cardiac events (adjusted risk ratio, 5.66; 95 percent confidence interval, 3.07 to 10.56; P<0.01). Cardiac surgery was ultimately performed in 232 patients and was independently associated with improved survival (adjusted risk ratio, 0.28; 95 percent confidence interval, 0.14 to 0.55; P<0.01). CONCLUSIONS: Quantitative grading of mitral regurgitation is a powerful predictor of the clinical outcome of asymptomatic mitral regurgitation. Patients with an effective regurgitant orifice of at least 40 mm2 should promptly be considered for cardiac surgery.
Aged↗
Electrocardiographic findings in primary systemic amyloidosis and biopsy-proven cardiac involvement.
The electrocardiograms of 127 patients with primary systemic amyloidosis and biopsy-proved cardiac involvement were analyzed. Low voltage (46%) and a pseudoinfarct pattern (47%) were the most common findings. Criteria for left ventricular hypertrophy were present in 16% of patients.
Adult↗
Diastolic stress echocardiography: a novel noninvasive diagnostic test for diastolic dysfunction using supine bicycle exercise Doppler echocardiography.
Left ventricular filling pressures can be estimated reliably by combining mitral inflow early diastolic velocity (E) and annulus velocity (E'). An increased E/E' ratio reflects elevated filling pressures and may be useful in assessing an abnormal increase in filling pressures for patients with diastolic dysfunction. The purpose of this study was to evaluate the feasibility of supine bicycle exercise Doppler echocardiography for assessing left ventricular diastolic pressure during exercise. Mitral inflow and septal mitral annulus velocities were measured at rest and during supine bicycle exercise (25-W 3-minute increments) in 45 patients (19 men; mean age, 59 years) referred for evaluation of exertional dyspnea. None had echocardiographic or electrocardiographic evidence of myocardial ischemia with exercise. Patients were classified according to E/E' ratio at rest: 26 had E/E' < or = 10 at rest (group 1) and 19 had E/E' > 10 (group 2). For group 1, 17 had no increase in E/E' during exercise (group 1A) and 9 did (group 1B). For group 2, E/E' did not increase during exercise. Despite different responses of E/E', there was no significant difference in changes of mitral inflow indices (E, A, E/A, deceleration time) between groups. Although the percentage of dyspnea as a primary reason for stopping exercise was similar for the groups, exercise duration was significantly shorter for groups 1B (7.2 +/- 2.5 minutes) and 2 (7.1 +/- 3.3 minutes) than in group 1A (10.4 +/- 3.7 minutes, P = .0129). Diastolic stress echocardiography using a supine bicycle is technically feasible for demonstrating changes in E/E' (filling pressure) with exercise. Our preliminary results suggest the hemodynamic consequences of exercise-induced increase in diastolic filling pressure can be demonstrated noninvasively with exercise Doppler echocardiography.
Adult↗
Clinical, laboratory, and transesophageal echocardiographic correlates of interatrial septal thickness: a population-based transesophageal echocardiographic study.
BACKGROUND: The determinants of interatrial septal (IAS) thickening ("lipomatous hypertrophy"), a common echocardiographic finding in the elderly, are poorly defined. The objective of this study was to determine the clinical, laboratory, and transesophageal echocardiographic correlates of IAS thickening in the general population. METHODS: The thickness of the IAS was measured by transesophageal echocardiography in 384 patients (median age: 66 years; range: 51-101 years; 53% men) participating in a population-based study (Stroke Prevention: Assessment of Risk in a Community). The associations between atherosclerosis risk factors, clinical cardiovascular disease, aortic atherosclerotic plaques, and IAS thickness were examined. RESULTS: Age and body surface area (BSA) were significantly associated with IAS thickness (median: 6 mm; range: 2-17 mm). IAS thickness increased by 12.6% per 10 years of age (95% confidence interval: 9.0-16.4%) adjusting for sex and BSA, and increased by 7.0% per 0.1 m 2 BSA (confidence interval: 5.0-9.2%) adjusting for age and sex. Overall, age, sex, and BSA accounted for 22.5% of the variability in IAS thickness. Current smoking (20.4% increase in IAS thickness in current smokers) and hypertension treatment (8.5% increase in treated patients) were associated with increased IAS thickness, adjusting for age, sex, and BSA ( P < .05), but these two risk factor variables jointly explained only an additional 2.3% of the variability in IAS thickness beyond the variability explained by age, sex, and BSA. Clinical coronary artery and cerebrovascular disease, atrial arrhythmias, and aortic atherosclerotic plaques were not associated with IAS thickness, adjusting for age, sex, and BSA ( P > .3). CONCLUSIONS: IAS thickening is an age-associated process. Atherosclerosis risk factors are weakly associated with IAS thickening, whereas atherosclerotic vascular disease is not.
Age Factors↗
Ventricular septal hamartoma mimicking hypertrophic cardiomyopathy in a 41-year-old woman presenting with paroxysmal supraventricular tachycardia.
We report an unusual case of cardiac hamartoma involving the ventricular septum. The patient presented with palpitations and exertional chest pain. Echocardiography indicated a large homogeneous mass occupying the superior two-thirds of the ventricular septum. This report confirms the use of transthoracic echocardiography as a screening tool in the diagnosis and management of an uncommon case of a benign cardiac tumor.
Adult↗
Yield of genetic testing in hypertrophic cardiomyopathy.
OBJECTIVE: To determine the clinical parameters of hypertrophic cardiomyopathy (HCM) that correlated significantly with the presence of an identifiable sarcomeric mutation. PATIENTS AND METHODS: Previous comprehensive mutational analyses of all protein-coding exons of 8 sarcomeric genes revealed pathogenic mutations in 147 (38%) of 389 unrelated patients seen at the HCM outpatient clinic at the Mayo Clinic in Rochester, Minn, between April 1997 and December 2001. Clinical data, extracted from patient records and blinded to patient genotype, were maintained in a custom database. RESULTS: In 389 unrelated patients, younger age at diagnosis, family history of HCM, and Increasing left ventricular wall thickness were all associated with Increased likelihood of identifying an HCM-associated sarcomeric mutation. In contrast, family history of sudden cardiac death, myectomy status, and anatomical subtype did not correlate significantly with genotype-positive status. With use of a simple scoring system based on age at diagnosis, left ventricular wall thickness, and family history of HCM, the likelihood of a sarcomeric mutation could be estimated. CONCLUSION: Clinical predictors of positive genotype, such as the presence of an implantable cardioverter-defibrillator, age at diagnosis, degree of left ventricular wall hypertrophy, and family history of HCM, may aid in patient selection for genetic testing and increase the yield of cardiac sarcomere gene screening.
Adolescent↗
Sarcomeric genotyping in hypertrophic cardiomyopathy.
OBJECTIVE: To pool results from studies of patients with hypertrophic cardiomyopathy (HCM) to elucidate important phenotypic differences among genotypes. MATERIAL AND METHODS: Data published from November 1998 through November 2004 were gathered and compared from unrelated study population genotyping studies from the Mayo Clinic (Rochester, Minn), Harvard Medical School (Boston, Mass), France, Germany, Sweden, Finland, and Spain. Standard statistical analysis techniques were used to pool and compare data across genotypes with respect to frequency of mutations, age at diagnosis, and degree of hypertrophy (left ventricular wall thickness). RESULTS: The French study population harbored the highest frequency of mutations (61%), followed by the Mayo Clinic (38%), Harvard Medical School (36%), and Swedish (30%) study populations. For every study population, mutations in myosin binding protein C (MYBPC3) were the most common cause of HCM. Patients with a family history of HCM had mutations more frequently than those without. This pooled analysis revealed no statistically significant differences in left ventricular wall thickness or in mean age at diagnosis across all genotypes. CONCLUSIONS: Differentiation of sarcomeric genotypes, such as MYBPC3-HCM and MYH7-HCM, is not possible on the basis of currently reported phenotypic data. A myriad of genetic and/or environmental modifiers in addition to the primary disease-causing genetic substrate must play an important role in determining a patient's particular phenotype.
Cardiomyopathy, Hypertrophic↗