OBJECTIVES: The purpose of this study was to determine if pulmonary vascular capacitance predicts survival in patients with idiopathic pulmonary arterial hypertension (IPAH). BACKGROUND: The prognosis of patients with IPAH is difficult to predict, despite knowledge of clinical and hemodynamic parameters previously identified as predictors. METHODS: We proposed a capacitance index of stroke volume divided by pulmonary pulse pressure (SV/PP) and prospectively gathered data on IPAH patients who underwent a right heart catheterization. SV/PP was analyzed as a predictor of mortality after adjusting for other modifiers of risk. RESULTS: During 4-year follow-up of 104 patients, 21 patients died. When compared with conventional markers, SV/PP was the strongest univariate predictor of mortality (hazard ratio 17.0 per ml.mm Hg(-1) decrease, 95% confidence interval 13.0 to 22.0; p < 0.0001). In successive bivariate analysis, SV/PP was the only predictor of mortality. In quartile analysis, the lowest SV/PP quartile had a 4-year mortality of 61%; the highest SV/PP had no deaths. CONCLUSIONS: The capacitance index (SV/PP) is a strong independent predictor of mortality in patients with IPAH.

Adult↗

PubMed2006

Approach to the patient with aortic stenosis and low ejection fraction.

Aortic stenosis is the most common valvular abnormality in the United States today. It has been well established that most patients with severe symptomatic aortic stenosis should undergo operation with aortic valve replacement. This is particularly true when ventricular function is maintained, as the operative risk is low and the long-term outcome is excellent. In most patients with a reduced ejection fraction, there is an increased risk of operation and poorer long-term outcome. However, operation is still of benefit in most patients, as relief of an afterload mismatch will improve ventricular function and provide symptom relief. There is a subset of patients with left ventricular dysfunction at even higher risk who have a low aortic valve gradient and small valve area, some of whom may not have severe obstruction. Pharmacologic challenge may be necessary to identify those patients with true aortic stenosis and further helps determine those who would benefit from surgical intervention.

Aortic Valve↗

PubMed2006

Determining myocardial viability in chronic ischemic left ventricular dysfunction: a prospective comparison of rest-redistribution thallium 201 single-photon emission computed tomography, nitroglycerin-dobutamine echocardiography, and intracoronary myocardial contrast echocardiography.

BACKGROUND: Detection of viable myocardium (VM) has important therapeutic implications for chronic ischemic left ventricular (LV) systolic dysfunction. We compared the ability of nitroglycerin-dobutamine echocardiography (NTG-DE), intracoronary myocardial contrast echocardiography (MCE), and rest-redistribution thallium 201 single-photon emission computed tomography (RRT-SPECT) to detect VM in this setting. METHODS: Patients with LV ejection fraction (LVEF) <40% and multivessel coronary disease suitable for revascularization underwent NTG-DE, MCE, RRT-SPECT, and radionuclide ventriculography to determine baseline LVEF. Myocardial contrast echocardiography was performed using intracoronary injection of Albunex. Patients who underwent revascularization had 3-month postprocedural radionuclide ventriculography and transthoracic echocardiography to assess functional recovery. RESULTS: Of 512 myocardial segments in the 32 patients studied, 309 were akinetic or dyskinetic at baseline. Nitroglycerin alone increased regional thickening in 20% of segments with contractile reserve. By RRT-SPECT, 93% of nitroglycerin-responsive segments were viable. Myocardial contrast echocardiography had up to 85% sensitivity and 74% specificity for detection of VM diagnosed by RRT-SPECT. In the 23 patients who underwent revascularization, 54% of akinetic segments showed improved contractility, and mean LVEF increased from 32% to 37% (P = .04). Sensitivities and specificities for detecting functional recovery were 95% and 37% for RRT-SPECT, up to 87% and 48% for MCE, and 63% and 83% for a biphasic response during NTG-DE. CONCLUSIONS: In patients with chronic ischemic LV dysfunction, RRT-SPECT had the highest sensitivity, and NTG-DE, the best specificity for detection of VM. Nitroglycerin facilitated detection of VM and may be a useful adjunct to dobutamine stimulation.

Aged↗

PubMed2006

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↗

PubMed2006

The prognostic value of pulmonary vascular capacitance determined by Doppler echocardiography in patients with pulmonary arterial hypertension.

OBJECTIVES: We sought to determine if a novel measurement of pulmonary vascular (PV) capacitance (PVCAP) by Doppler echocardiography predicts all-cause mortality in patients with primary pulmonary arterial (PA) hypertension (PPAH). BACKGROUND: The prognosis of patients with PPAH is variable and has been difficult to predict using clinical or hemodynamic parameters. PVCAP is a measure of the workload on the right ventricle (RV) and we recently have shown that PVCAP determined by cardiac catheterization is a strong predictor of survival. This same hemodynamic information to calculate PVCAP can be derived from Doppler echocardiography. Therefore, the purpose of this study was to determine if PVCAP from noninvasive Doppler echocardiography would be a useful measure of survival in patients with PPAH. METHODS: We analyzed clinical and hemodynamic variables on all patients with PPAH who had a right heart catheterization and echocardiogram from January to December 1999. Because capacitance is directly proportional to stroke volume and inversely proportional to PA pulse pressure, PVCAP was defined as stroke volume/pulse pressure. PVCAP was derived noninvasively from a comprehensive 2-dimensional and Doppler echocardiogram. Using the peak systolic tricuspid regurgitation velocity and the end-diastolic pulmonary regurgitation velocity, the modified Bernoulli equation was used to calculate the PA systolic and diastolic pressures, respectively. Stroke volume was obtained using the volumetric flow through the left ventricular outflow tract. PVCAP was then analyzed as a predictor of mortality, adjusting for other known modifiers of risk. RESULTS: In all, 54 patients (13 men) were studied with a mean age of 44 +/- 11 years, ejection fraction of 62 +/- 11%, and RV systolic pressure of 90 +/- 21 mm Hg. In all, 24% were in World Health Organization (WHO) class II, 52% in class III, and 24% in class IV. During follow-up of 1498 +/- 108 days, 12 patients died. The strongest noninvasive predictor of mortality was PVCAP (risk ratio 3.0/mL/mm Hg decrease in PVCAP, 95% confidence interval 1.2-8.0, P = .0212). WHO class, RV index of myocardial performance, RV systolic pressure, and RV ejection time were weaker predictors. PVCAP was also a stronger predictor of mortality than invasively determined PV resistance, right atrial pressure, and mean PA pressure. In multivariate analysis, PVCAP was the only noninvasive predictor of mortality. In quartile analysis the lowest PVCAP quartile had a 4-year mortality of 39% whereas the highest PVCAP had a mortality of 7%. CONCLUSION: The novel measure of PVCAP, as determined by Doppler echocardiography, is a strong noninvasive predictor of mortality in patients with PPAH and adds prognostic value to conventional risk markers.

Adult↗

PubMed2006

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↗

PubMed2005

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↗

PubMed2005

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↗

PubMed2005

Severe symptomatic tricuspid valve regurgitation due to permanent pacemaker or implantable cardioverter-defibrillator leads.

OBJECTIVES: We report a series of patients with severe tricuspid valve regurgitation due to a permanent pacemaker (PPM) or implantable cardioverter-defibrillator (ICD) lead. BACKGROUND: Severe tricuspid regurgitation caused by a PPM or ICD lead is an under-recognized but treatable etiology of severe right heart failure. METHODS: We reviewed the records of 41 patients who underwent tricuspid valve operation for severe tricuspid regurgitation caused by previously placed PPM or ICD leads. RESULTS: During surgery, severe tricuspid regurgitation was found to be caused by the PPM or ICD leads in all 41 patients. There was a perforation of the tricuspid valve leaflet by the PPM or ICD lead in 7 patients, lead entanglement in the tricuspid valve occurred in 4 patients, lead impingement of the tricuspid valve leaflets occurred in 16 patients, and lead adherence to the tricuspid valve occurred in 14 patients. The septal leaflet was most often perforated (6 of 7). In the preoperative evaluation, valve malfunction due to the PPM or ICD lead was diagnosed preoperatively in only 5 of 41 (12%) patients by transthoracic echocardiography. All patients underwent successful tricuspid valve operation (22 tricuspid valve replacement), with one perioperative death occurring. During follow-up (range, 1 to 99 months), there was one patient who died from left-sided heart failure and three patients died of other causes. The remaining patients showed improvement in signs and symptoms of heart failure. CONCLUSIONS: Damage to the tricuspid valve by PPM or ICD leads may result in severe symptomatic tricuspid regurgitation and may not be overtly visualized by echocardiography. This etiology should be considered when evaluating patients with severe right heart failure after PPM or ICD implantation.

Aged↗

PubMed2005

Task Force 1: preparticipation screening and diagnosis of cardiovascular disease in athletes.

Explore the source record for details and available documents.

Adaptation, Physiological↗

PubMed2005

Task Force 4: HCM and other cardiomyopathies, mitral valve prolapse, myocarditis, and Marfan syndrome.

Explore the source record for details and available documents.

Cardiomyopathies↗

PubMed2005

Alcohol septal ablation for hypertrophic cardiomyopathy: indications and technique.

Alcohol septal ablation for the treatment of hypertrophic cardiomyopathy has been the subject of great interest, and the number of procedures performed is increasing despite an absence of randomized trial data. Although straightforward in concept, alcohol septal ablation may be considerably more difficult in actual practice. To optimize the results and prevent complications, the anatomy of the septal arcade architecture must be understood and the anatomic relationship between the septal artery and the specific portion of the septum to be ablated must be carefully delineated. For the latter, during the procedure, an echocardiographic contrast medium injection into the septal artery of interest is essential. Selection of the volume and amount of alcohol to be injected varies depending on the size and distribution of the septal artery. Specific complications such as conduction defects, hemodynamic compromise, ventricular arrhythmias, and inadequate gradient reduction can be minimized by specific technical approaches. After ablation, protocols are needed for periprocedural guidelines because some complications may occur late during the next several days. For optimal results, patients need to be selected after catheter assessment and combined echocardiography and angiography, and ablation techniques need to be scientific and rigorous.

Cardiomyopathy, Hypertrophic↗

PubMed2005

Effectiveness of a computerized system for intravenous heparin administration: using information technology to improve patient care and patient safety.

To overcome errors in prescribing, calculating doses, and monitoring intravenous heparin, a computerized heparin nomogram system (HepCare) was developed to improve heparin safety using interactive cues between the prescriber, nurse, pharmacist, and the laboratory. The frequency of deviations decreased from 0.5 per patient before HepCare with the protocol to 0.006 per patient with HepCare and the protocol. The goal activated partial thromboplastin time results of the HepCare system (group I) were compared with patients who were not treated using the HepCare system (group II). There was a higher mean percentage of activated partial thromboplastin times within goal range in group I vs. II-44% vs. 27% (p<0.01). There were reminders of a drop in platelet count in 6% of patients, hemoglobin drop in 0.7%, and validation activated partial thromboplastin time values in 7% of patients by HepCare. HepCare-guided intravenous heparin resulted in significant improvements in safety, quality assurance, and targeted activated partial thromboplastin time values.

Anticoagulants↗

PubMed2004

Alcohol septal ablation versus surgical septal myectomy: comparison of effects on atrioventricular conduction tissue.

OBJECTIVES: This study was designed to evaluate the effect of septal reduction therapies on the conduction system for patients with hypertrophic cardiomyopathy (HCM). BACKGROUND: Heart block is a potential complication of both catheter-based and surgical procedures to relieve left ventricular outflow tract obstruction in HCM, but it is important to understand the different effects of these treatments on the conduction system. METHODS: The electrocardiograms and postoperative course of patients who underwent percutaneous alcohol septal ablation or surgical myectomy at Mayo Clinic between 1999 and 2003 were reviewed. RESULTS: For the 58 patients who underwent alcohol septal ablation, 21 (36%) developed right bundle branch block. Six patients (12%) developed complete heart block requiring permanent pacing, three of whom had left bundle branch block before the procedure. Among the 117 patients who underwent surgical septal myectomy, 47 (40%) developed left bundle branch block. Four patients (3%) developed heart block requiring permanent pacing after the procedure, three of whom had right bundle branch block preoperatively. CONCLUSIONS: Percutaneous septal ablation selectively produces transmural infarction of the basal mid-septum and adjacent right bundle tissue, whereas surgical myectomy affects the endocardial portion of the basal anterior septum and adjacent left bundle tissue. These observations may help identify patients at risk for complete heart block after septal reduction procedures for HCM.

Atrioventricular Node↗