Search

Aims: Trials of rate control vs. rhythm control for atrial fibrillation or flutter included few patients with new-onset arrhythmia. Our objective was to assess the relapse rate and the effect of the relapse of new-onset atrial arrhythmias on mortality after direct-current cardioversion (DCCV)., Methods and Results: A cohort of 351 patients with atrial fibrillation (new onset in 179) and 126 patients with atrial flutter (new onset in 78) was followed-up after DCCV. Cox proportional hazard models were used. Median age was 74.6 years. Mean follow-up for relapse was 7.7 months; for death, 29.4 months. Patients with new-onset atrial flutter [adjusted hazard ratio (HR) = 1] were more likely to maintain sinus rhythm than the patients with recurrent atrial flutter (adjusted HR = 2.5, P < 0.01), new-onset atrial fibrillation (adjusted HR = 2.4, P < 0.01), or recurrent atrial fibrillation (adjusted HR = 2.7, P < 0.01). Patients with new-onset atrial fibrillation were as likely to have relapses as patients with recurrent atrial fibrillation or flutter. Relapse of atrial arrhythmia after DCCV was associated with increased mortality (adjusted HR= 3.1, P < 0.01)., Conclusion: DCCV is more successful in maintaining sinus rhythm in patients with new-onset atrial flutter than in patients with new-onset atrial fibrillation. Relapse of atrial arrhythmia after cardioversion is associated with increased mortality.

Background: In patients undergoing transesophageal echocardiography-guided cardioversion, we evaluated the use and safety of an expedited in-hospital anticoagulation regimen that incorporates shorter-than-standard durations of precardioversion intravenous unfractionated heparin and postcardioversion bridging therapy with a low-molecular-weight heparin., Methods: Adult patients who underwent successful transesophageal echocardiography-guided cardioversion for atrial fibrillation or atrial flutter between May 2000 and August 2003 were classified into 2 groups by duration of intravenous unfractionated heparin therapy (<24 h or > or =24 h) before transesophageal echocardiography and cardioversion. Safety end points evaluated included all-cause death, stroke or other thromboembolic events, and major bleeding complications within 1 month after successful cardioversion., Results: The study population of 386 patients included 199 (52%) who received expedited intravenous unfractionated heparin (<24 h; minimum duration, <4 h) and 193 patients (50%) who were discharged on low-molecular-weight heparin therapy. The adverse event rates at 1-month follow-up were not significantly different between the 2 unfractionated heparin patient groups, and the rate of stroke among patients dismissed on low-molecular-weight heparin was less than 1%. No adverse events occurred among patients who received intravenous unfractionated heparin for less than 12 hours and who were dismissed on low-molecular-weight heparin bridging therapy., Conclusions: The use of an expedited heparin anticoagulation regimen in patients with atrial fibrillation or atrial flutter undergoing transesophageal echocardiography-guided cardioversion appears to be safe. Cardioversion can be performed as early as a few hours after initiation of intravenous unfractionated heparin, and bridging therapy with a low-molecular-weight heparin can be used after cardioversion until the international normalized ratio is therapeutic.

Objectives: We sought to test the hypothesis that C-reactive protein (CRP) can predict the recurrence of atrial fibrillation (AF) after successful electrical cardioversion (CV)., Background: In patients with AF, CRP levels are predictive of immediate failure of CV., Methods: We prospectively measured high-sensitivity CRP in 67 patients with AF or atrial flutter who underwent successful electrical CV., Results: At one-month follow-up, 22 patients (33%) had recurrence of their arrhythmia. Arrhythmia recurrence was associated with significantly higher pre-CV CRP levels (odds ratio [OR] 1.84; 95% confidence interval [CI] 1.14 to 2.98; p = 0.013) even after adjusting for age (OR 2.22; 95% CI 1.25 to 3.93; p = 0.006), for gender (OR 1.89; 95% CI 1.16 to 3.09; p = 0.011), or duration of arrhythmia (OR 1.86; 95% CI 1.13 to 3.07; p = 0.015). On multivariate analysis, CRP was the only independent predictor of arrhythmia recurrence (OR 2.19; 95% CI 1.05 to 4.55; p = 0.036)., Conclusions: Our data suggest that high levels of CRP are associated with an increased risk of recurrence of AF within one month. These data support the hypothesis that anti-inflammatory interventions may help in maintenance of normal sinus rhythm after CV. These data also may have implications for the identification of patients who are most likely to experience substantial benefit from CV therapy for AF.

Objectives: The purpose of this study was to determine if there is a difference in commercially available biphasic waveforms., Background: Although the superiority of biphasic over monophasic waveforms for external cardioversion of atrial fibrillation (AF) is established, the relative efficacy of available biphasic waveforms is less clear., Methods: We compared the effectiveness of a biphasic truncated exponential (BTE) waveform and a biphasic rectilinear (BR) waveform for external cardioversion of AF. Patients (N = 188) with AF were randomized to receive transthoracic BR shocks (50, 75, 100, 120, 150, 200 J) or BTE shocks (50, 70, 100, 125, 150, 200, 300, 360 J). Shock strength was escalated until success or maximum energy dose was achieved. If maximum shock strength failed, patients received the maximum shock of the opposite waveform. Analysis included 141 patients (71 BR, 70 BTE; mean age 66.5 +/- 13.7. Forty-seven randomized patients were excluded because of flutter on precardioversion ECG upon blinded review (n = 25), presence of intracardiac thrombus (n = 7), or protocol deviation (n = 15). Groups were similar with regard to clinical and echocardiographic characteristics., Results: The success rate was similar for the two waveforms (93% BR vs 97 BTE, P = .44), although cumulative selected and delivered energy was less in the BTE group. Only AF duration was significantly different between successful and unsuccessful patients. No significant complications occurred., Conclusions: Biphasic waveforms were very effective in transthoracic cardioversion of AF, and complication rates were low. No significant difference in efficacy was observed between BR and BTE waveforms. Impedance was not an important determinant of success for either biphasic waveform.

Objectives: This study was designed to determine the prevalence and clinical manifestations of 22q11.2 microdeletion in adults with selected conotruncal anomalies and to assess the clinician's ability to predict the presence or absence of 22q11.2 microdeletion on the basis of clinical features., Background: It is known that 22q11.2 microdeletion is a chromosomal anomaly with cardiac and extracardiac manifestations. The prevalence and manifestations in adults have not been well characterized., Methods: A total of 103 consecutive adults with either tetralogy of Fallot (TOF), pulmonary atresia/ventricular septal defect (PA/VSD), or truncus arteriosus (TA) were prospectively screened for 22q11.2 microdeletion using a fluorescence in situ hybridization (FISH) assay. Clinicians were asked to predict 22q11.2 microdeletion status on the basis of clinical features. A geneticist blinded to FISH assay results reviewed photographs of the patients for typical dysmorphic features of 22q11.2 microdeletion., Results: Six patients (prevalence 5.8%, 95% confidence interval 1.3 to 10.3) had 22q11.2 microdeletion (3 with TOF, 2 with PA/VSD, 1 with TA). In two of these patients, the clinician incorrectly predicted absence of the deletion. In three, typical dysmorphic features of 22q11.2 microdeletion were absent., Conclusions: Our work showed that 22q11.2 microdeletion is under-recognized in adults with congenital heart disease. The absence of typical phenotypic features makes it difficult to correctly predict if the deletion is present. Screening for 22q11.2 microdeletion should be considered in adults with high-risk cardiac lesions, as it has important implications in reproductive counseling and surveillance for associated extracardiac manifestations.

Background: Atrial remodeling secondary to atrial fibrillation (AF) may be important in the arrhythmogenic process. Unfortunately, the study of electrophysiologic remodeling in humans has been limited by the invasive nature of most tests of electrophysiologic characteristics. We sought to determine whether changes in atrial electrophysiology occur acutely (within the first hour) after cardioversion and whether these changes could be detected noninvasively by measuring the signal-averaged P-wave., Methods: The filtered P-wave duration (FPD) was measured by signal-averaged electrocardiography (ECG) at 20 and 60 minutes after cardioversion in 46 patients with AF, and the difference between the two values was calculated. The root-mean-square voltage of the terminal 40 ms of the signal-averaged P-wave at 20 and 60 minutes and the difference between them were also determined., Results: The FPD at 20 minutes was significantly different from that at 60 minutes (153.0 +/- 19.1 vs 159.7 +/- 24.8 ms; P = 0.02). In a univariate linear regression model, none of the clinical variables studied was significantly associated with the change in FPD. The root-mean-square voltage at 20 minutes was not significantly different from that at 60 minutes (5.8 +/- 3.0 vs 5.5 +/- 2.7; P = 0.14)., Conclusions: We conclude that significant changes in atrial electrophysiology occur within the first hour after cardioversion of AF. These changes can be detected by measuring the FPD.

Background: Transthoracic cardioversion fails to restore sinus rhythm in 6% to 33% of patients with atrial fibrillation. This study sought to determine the relative efficacy of biphasic waveforms compared with monophasic waveforms in the treatment of atrial arrhythmias., Methods: A total of 912 patients underwent 1022 transthoracic cardioversions between May 2000 and December 2001. A monophasic damped sine waveform was used in the first 304 cases, and a rectilinear biphasic defibrillator was used in the next 718 cases., Results: Use of a biphasic waveform was associated with 94% success in conversion to sinus rhythm compared with 84% with a monophasic waveform (P < .001). The cumulative energy required to restore sinus rhythm was lower with biphasic shocks in both atrial fibrillation and atrial flutter groups (554 +/- 413 J for monophasic vs 199 +/- 216 J for biphasic shocks in the atrial fibrillation group, P < .001; 251 +/- 302 J vs 108 +/- 184 J, respectively, in the atrial flutter group, P < .001). In a multivariate analysis, use of a biphasic shock was associated with a 3.9-fold increase in success of cardioversion., Conclusion: When used to cardiovert atrial arrhythmias, the rectilinear biphasic waveform was associated with higher success rates and lower cumulative energies than the monophasic damped sine waveform.

This study evaluated the diagnostic role of early diastolic mitral annular velocity (E') by tissue Doppler echocardiography for differentiating constrictive pericarditis from restrictive cardiomyopathy (primary restrictive cardiomyopathy and cardiac amyloidosis). The study group consisted of 75 patients (53 men, 22 women; mean age 62 years, range 27 to 87). Of these, 23 patients had surgically confirmed constrictive pericarditis, 38 had biopsy-proved systemic amyloidosis and typical echocardiographic features of cardiac involvement, and 14 had primary restrictive cardiomyopathy. Standard mitral inflow characteristics were measured. Tissue Doppler echocardiography was used to measure E' at the septal annulus. E' was significantly higher in patients with constrictive pericarditis than in those with primary restrictive cardiomyopathy or cardiac amyloidosis (12.3 vs 5.1 cm/second, p <0.001). An E' cut-off value > or =8 cm/second resulted in 95% sensitivity and 96% specificity for the diagnosis of constrictive pericarditis. There was no overlap of E' between patients who had constrictive pericarditis and those who had cardiac amyloidosis. In a subgroup analysis of restrictive cardiomyopathy, E' of patients who had cardiac amyloidosis was significantly lower than that of patients who had primary restrictive cardiomyopathy (4.6 vs 6.3 cm/second, p <0.001). Thus, E' velocity can distinguish between constrictive pericarditis and restrictive cardiomyopathy with a specific cut-off value in patients with clinical and echocardiographic evidence of diastolic heart failure.

Objectives: We sought to determine pregnancy outcomes in patients with tetralogy of Fallot (TOF)., Background: Pregnancy outcomes in patients with TOF are incompletely defined., Methods: Clinical, hemodynamic, and obstetric data were reviewed for women with TOF and prior pregnancy., Results: Of 72 respondents, 43 (mean age, 26 years) had 112 pregnancies (range, 1 to 5); 82 pregnancies were successful. Eight women had unrepaired TOF at the time of their 20 successful pregnancies. At first assessment (age > or =18 years), six patients had pulmonary hypertension, three had moderate or severe right ventricular (RV) systolic dysfunction, and 13 had severe RV dilation due to pulmonic regurgitation. Sixteen patients had 30 miscarriages (27%) and one term stillbirth. Mean overall birth weight was 3.2 kg (range, 2.1 to 4.2 kg). Unrepaired TOF (p = 0.05) and morphologic pulmonary artery abnormality (p = 0.03) were independently predictive of infant birth weight. Six patients had cardiovascular complications during pregnancy: supraventricular tachycardia in two, heart failure in two, pulmonary embolism in a patient with pulmonary hypertension, and progressive RV dilation in a patient with severe pulmonic regurgitation. Five infants (6%) had congenital anomalies., Conclusions: Patients with TOF have an increased risk of fetal loss, and their offspring are more likely to have congenital anomalies than offspring in the general population. Adverse maternal events, although rare, may be associated with left ventricular dysfunction, severe pulmonary hypertension, and severe pulmonic regurgitation with RV dysfunction.

Objective: This study was undertaken to examine the association between bicuspid aortic valve (BAV) and aortic dilatation in the community. The association between BAV and aortic dilatation has been reported predominantly in retrospective studies in patients mostly with valvular dysfunction or selected surgical patients from tertiary referral centers. An independent association of BAV and aortic dilatation in a community-based study has not been demonstrated., Methods and Results: In a geographically defined population of Olmsted County, Minnesota, residents with BAV (n=44, age 35+/-13 years) without hemodynamically significant obstruction or regurgitation and matched controls with normal tricuspid aortic valves were identified by transthoracic echocardiography. The two groups were compared with respect to measurements of the aorta. The BAV and control groups differed with respect to size of the aortic anulus (23.2+/-2.4 versus 21.6+/-2.4 mm; P=0.002), aortic sinus (33.5+/-4.6 versus 30.3+/-4.1 mm; P=0.0001), and proximal ascending aorta (33.3+/-6.5 versus 27.9+/-3.6 mm; P=0.0001). There was no difference in the size of the aortic arch (24.2+/-3.6 versus 25.3+/-3.4 mm; P=0.16). These differences were maintained when the groups were stratified by sex and blood pressure. The relationship between bicuspid aortic valve and aortic dilatation was maintained when adjusting for factors related to fluid mechanics and hemodynamics such as systolic blood pressure, diastolic blood pressure, left ventricular ejection time, and peak aortic valve velocity., Conclusions: In a community-based study, BAV is associated with an alteration of aortic dimensions even in the absence of hemodynamically significant aortic valve stenosis or regurgitation.

Atrial fibrillation is the most common sustained cardiac arrhythmia and an important health concern in the United States because of the increasing aging population. Cardioversion of atrial fibrillation to sinus rhythm to relieve symptoms and to reduce the incidence of thromboembolism is now common practice. Recently, transesophageal echocardiography (TEE)-facilitated cardioversion emerged as an acceptable therapeutic alternative owing to the assumption that early cardioversion can obviate many of the concerns and disadvantages associated with the conventional approach. We review the current standing of TEE-facilitated early cardioversion vis-à-vis the salient cardioversion issues and its potential future role amid evolving cardioversion paradigms.

Objectives: The goal of this study was to determine the presentation and outcome of the unoperated adult with congenitally corrected transposition of the great arteries., Background: The presentation of this disorder and the outcome in unoperated adults have not been well defined., Methods: All unoperated patients > or =18 years old were evaluated for spectrum of disease, hemodynamic severity, timeliness of diagnosis and referral, and outcome., Results: Forty-four patients aged 20 to 79 years (mean, 44) were followed up to 144 months. In 29 (66%), the correct diagnosis was first made at age > or =18 years; the diagnosis was missed in seven of these patients in a prior cardiology consultation, despite cardiac imaging. Systemic atrioventricular valve (SAVV) regurgitation (grade > or =3/4) was noted in 26 patients (59%). Thirty (68%) had surgical intervention, including SAVV replacement in all, with no early mortality. Preoperatively, this subset had significant dysfunction of the systemic ventricle (SV) (ejection fraction [EF], 40 +/- 10%), and most had advanced symptoms (25 with ability index > or =2/4). In 16 (53%), SAVV regurgitation > or =3/4 and ventricular dysfunction had been documented for >6 months. The mean EF of the SV decreased significantly postoperatively (34 +/- 11%, p = 0.006). Four patients (13%) eventually required cardiac transplantation. Poor preoperative EF of the SV predicted eventual need for transplantation (p = 0.001)., Conclusions: Patients with unoperated congenitally corrected transposition of the great arteries are often misdiagnosed in adulthood and are referred late despite symptomatic SAVV regurgitation and significant SV dysfunction. Although excellent early surgical results can be achieved, significant residual dysfunction of the SV is common.

Objectives: The study sought to determine the outcome of pregnancy in women with coarctation of the thoracic aorta., Background: Patients with coarctation of the thoracic aorta are expected to reach childbearing age, but data on the outcome of pregnancy in this population are limited., Methods: The Mayo Clinic database was reviewed for women of childbearing age (> or =16 years old) with a diagnosis of aortic coarctation evaluated from 1980 to 2000. Spectrum of cardiovascular disease, surgical history, and obstetrical and neonatal outcomes were determined., Results: Fifty women with coarctation had pregnancies: 30 had coarctation repair before pregnancy, 10 had repair after pregnancy, 4 had repair both before and after pregnancy, and 6 had no history of repair. The 50 women had 118 pregnancies resulting in 106 births. There were 11 miscarriages (9%), 4 premature deliveries (3%), and 1 early neonatal death; 38 deliveries (36%) were by cesarean section. Of the 109 offspring, 4 (4%) had congenital heart disease. A patient with Turner syndrome died of a Stanford type A dissection at 36 weeks of pregnancy. Nineteen women (38%) were known to have hemodynamically significant coarctation during pregnancy (gradient > or =20 mm Hg). Fifteen women (30%) had hypertension during their pregnancy, 11 of whom (73%) had hemodynamically significant coarctation during that time (8 with native and 3 with residual/recurrent coarctation)., Conclusions: Major cardiovascular complications were infrequent but continue to be a source of concern for patients with coarctation who become pregnant. Systemic hypertension during pregnancy was common and related to the presence of a significant coarctation gradient.

Because congenital ventricular septal defects are of different sizes and locations, their clinical presentation, natural history, and treatment vary greatly. This review discusses the different types of ventricular septal defects commonly seen in adults in the authors' experience and in published literature. Ventricular septal defects are either isolated small defects or larger defects associated with pulmonary stenosis, pulmonary hypertension, or aortic regurgitation. These associations play an important role in the pathophysiologic consequences of the defect, its long-term complications, and treatment options. Knowledge of the different clinical presentations in adulthood and the specific features pertinent to these defects will help in the assessment and the care of adult patients with one of the most common congenital cardiac malformations.

A 31-year-old male with pulmonary atresia, ventricular septal defect presented with exercise intolerance and severe cyanosis. A restrictive coronary-pulmonary artery fistula was identified as the main source of pulmonary blood flow. We report transcatheter stent implantation in the fistula to augment pulmonary flow as a palliative management option in the adult patient with complex congenital heart disease., (Copyright 2001 Wiley-Liss, Inc.)

Implications: Atrial septal defect is usually diagnosed and surgically repaired in childhood. We present a case of previously asymptomatic atrial septal defect that presented during total hip arthroplasty during general anesthesia in a 72-yr-old woman. Intraoperative transesophageal echocardiography assisted in the diagnosis and in determining the appropriate treatment.

Primary cardiac osteosarcomas are rare and usually originate in the left atrium. In contrast, osteosarcomas metastatic to the heart most commonly involve the right cardiac chambers. This case report describes an unusual primary cardiac osteosarcoma, initially observed as a slowly growing, densely calcified mass of the left ventricle with subsequent secondary pulmonary metastasis. Although cardiac tumors may be asymptomatic, this patient had recurrent bouts of ventricular tachyarrhythmia. We describe the clinical, echocardiographic, and radiological observations spanning 6 years and the gross and microscopic features at autopsy.

Background: Idiopathic restrictive cardiomyopathy is a poorly recognized entity of unknown cause characterized by nondilated, nonhypertrophied ventricles with diastolic dysfunction resulting in dilated atria and variable systolic function., Methods and Results: Between 1979 and 1996, 94 patients (61% women) 10 to 90 years old (mean, 64 years) met strict morphological echocardiographic criteria for idiopathic restrictive cardiomyopathy, mainly dilated atria with nonhypertrophied, nondilated ventricles. None had known infiltrative disease, hypertension of >5 years' duration, or cardiac or systemic conditions associated with restrictive filling. Nineteen percent were in NYHA class I, 53% in class II, and 28% in class III or IV. Atrial fibrillation was noted in 74% of patients and systolic dysfunction in 16%. Follow-up (mean, 68 months) was complete for 93 patients (99%). At follow-up, 47 patients (50%) had died, 32 (68%) of cardiovascular causes. Four had heart transplantation. The death rate compared with actuarial statistics was significantly higher than expected (P<0.0001). Kaplan-Meier 5-year survival was 64%, compared with expected survival of 85%. Multivariate analysis using proportional hazards showed that the risk of death approximately doubles with male sex (hazard ratio [HR] = 2.1), left atrial dimension >60 mm (HR = 2.3), age >70 years (HR = 2.0), and each increment of NYHA class (HR = 2.0)., Conclusions: Idiopathic restrictive cardiomyopathy or nondilated, nonhypertrophic ventricles with marked biatrial dilatation, as defined morphologically by echocardiography, affects predominantly elderly patients but can occur in any age group. Patients present with systemic and pulmonary venous congestion and atrial fibrillation and have a poor prognosis, particularly men >70 years old with higher NYHA class and left atrial dimension >60 mm.

Objectives: The purpose of this study was to evaluate the morbidity and mortality associated with noncardiac surgery (NCS) in patients (pts) with Eisenmenger syndrome., Background: Noncardiac surgery in pts with Eisenmenger syndrome is associated with increased cardiovascular complications., Methods: Fifty-eight pts with Eisenmenger syndrome (17M, 41F aged 18 to 69 years (mean 41 years) who had been followed for up to 41.5 years (mean 9.3 years) were retrospectively evaluated for any NCS done at > or = 17 years of age., Results: Twenty-four pts had a total of 28 NCSs at an age of 17 to 55 years (mean 29 years) including 9 tubal ligations, 3 neurosurgeries, 3 cholecystectomies, 3 hysterectomies, 3 vasectomies, and 1 each spinal fusion, appendectomy, eye enucleation, hernia repair, hand surgery, tonsillectomy and therapeutic abortion. There were two deaths (7%), one following spinal fusion and the other following appendectomy at another institution. Fourteen of these NCSs were performed at our institution, including 11 under general anesthesia. The duration of anesthesia varied from 75 to 525 min (mean 165 min). All pts remained in sinus rhythm. The lowest systolic blood pressure (BP) ranged from 78 to 125 mm Hg. Of those 11 pts, 9 were extubated immediately after surgery and 2 needed dopamine. Ten patients were discharged without any complications, including 3 within 1 day of surgery. One death occurred 10 days following spinal fusion. This pt had the longest anesthesia (525 min) and an intraoperative systolic BP as low as 78 mm Hg. She also needed the largest fluid administration (6,475 cc) in addition to postoperative mechanical ventilation and dopamine., Conclusions: Adult pts with Eisenmenger syndrome are at increased risk with NCS, but with current/modern techniques, the risk of death is less than previously thought. In the vast majority of cases, NCS can be undertaken without substantial morbidity, and early extubation is achievable. However, even with relatively minor surgery, significant complications, including death, can occur. Referral to major centers with expertise in the care of pts with Eisenmenger syndrome is advisable.

Partial anomalous pulmonary venous connection, a rare congenital anomaly, most commonly involves the right lung, with one or more pulmonary veins anomalously connecting most frequently to the superior vena cava and less commonly to the right atrium or inferior vena cava. This article describes an unusual case of anomalous pulmonary venous connection of the right lung to the azygos vein in an adult. This anomaly was clearly delineated with angiography, computed tomography of the chest, and transesophageal echocardiography. The transesophageal echocardiographic features of the anomaly are described as a means to prevent further diagnostic misinterpretation.

The purpose of this study was to determine the most discriminating clinical and echocardiographic features that are most helpful in correctly identifying Ebstein's anomaly of the tricuspid valve from other causes of tricuspid regurgitation. Ebstein's anomaly is an uncommon malformation of the tricuspid valve with diagnostic echocardiographic features. Other cardiac disorders associated with tricuspid valve regurgitation and predominate right-sided heart chamber enlargement can be misdiagnosed as Ebstein's anomaly. All patients who were referred to our institution between 1982 and 1995 with the diagnosis of Ebstein's anomaly but were found to have other abnormalities of the tricuspid value or right ventricle were identified. Their clinical, echocardiographic, and surgical records were reviewed retrospectively. Twenty-two patients (12 males and 10 females), aged 7 to 68 years (mean 33 years), were referred to our institution with the diagnosis of Ebstein's anomaly but were found to have another abnormality that mimicked clinical and diagnostic features of Ebstein's anomaly. The most common initial symptom was exercise intolerance (13 [59%] patients) followed by atrial arrhythmia (seven [32%] patients). Two patients had cyanosis. Three patients had paroxysmal and six had chronic atrial fibrillation/flutter. Cardiomegaly on chest x-ray film was noted in 18 (82%) patients. Referral diagnosis of Ebstein's anomaly had been made by echocardiography (12 patients), cardiac catheterization (four patients), both techniques (five patients), and echocardiography and magnetic resonance imaging (one patient). All 22 patients had predominate right atrial and right ventricular enlargement, and 18 (82%) of 22 patients also had right ventricular dysfunction. However, Ebstein's anomaly was confidently ruled out with repeat comprehensive echocardiography at our institution by establishing (1) absence of significant apical displacement of the septal tricuspid valve leaflet (> or = 8 mm/m2) and (2) lack of a redundant, elongated, anterior tricuspid valve leaflet in all 22 patients (100%). All had significant tricuspid regurgitation caused by tricuspid valve dysplasia (nine patients), tricuspid valve prolapse (four patients), trauma (four patients), right ventricular dysplasia (three patients), endocarditis (one patient), and annular dilation caused by free pulmonary regurgitation (one patient). In all 15 patients who subsequently underwent surgery (tricuspid valve repair [seven patients] or replacement [eight patients]), the absence of Ebstein's anomaly was confirmed. Echocardiographic absence of the characteristic degree of displacement of the septal leaflet of the tricuspid valve (> or = 8 mm/m2) and the presence of a nonelongated, nonredundant anterior tricuspid valve leaflet consistently excluded the diagnosis of Ebstein's anomaly. Under such circumstances, other anomalies of the tricuspid valve or right ventricle were consistently identified. Recognition of the mimics of Ebstein's anomaly had important surgical implications.

Objective: This study sought to demonstrate that with proper technique, identification of the normal and abnormal pulmonary venous connection can be made with confidence using transesophageal echocardiography (TEE)., Background: Partial anomalous pulmonary venous connection (PAPVC) is an uncommon congenital anomaly whose diagnosis has classically been made using angiography., Methods: We performed a retrospective review of all patients of all ages with PAPVC diagnosed at the Mayo Clinic who had undergone TEE because of either right ventricular volume overload or suspected intracardiac shunting by transthoracic echocardiography or intraoperatively., Results: A total of 66 PAPVCs were detected in 43 patients (1.5/patient); in 2 additional patients, TEE suggested, but did not diagnose, PAPVCs. Shortness of breath was the most common presenting symptom (42.2%), followed by heart murmur and supraventricular tachycardia. Right-sided anomalous veins were identified in 35 patients (81.4%), left-sided in 7 (16.3%) and bilateral in 1 (2.3%). There was a single anomalous connecting vein in 23 patients (53.5%), two in 18 (41.9%), three in 1 (2.3%) and four in 1 (2.3%). The connecting site was the superior vena cava (SVC) in 39 veins (59.1%), right atrial-SVC junction in 6 (9.1%), right atrium in 8 (12.1%), inferior vena cava in 1 (1.5%) and the coronary sinus in 2 (3.0%). Ten anomalous left pulmonary veins were connected by a vertical vein to the innominate vein (15.1%). Sinus venosus atrial septal defect (ASD) was the most common associated anomaly in 22 patients (49%), followed by ostium secundum ASD in 6 and patent foramen ovale in 4. Fifteen patients had an intact atrial septum. Thirty-one patients (68.8%) underwent surgical repair. PAPVC was confirmed in all patients, including the two whose TEE results were suggestive of PAPVC. All 49 PAPVCs detected by TEE preoperatively were confirmed at the time of operation., Conclusions: TEE is highly diagnostic for PAPVC and can obviate angiography. Accurate anatomic diagnosis may influence the need for medical and surgical management. TEE should be performed in patients with right ventricular volume overload when the precordial examination is inconclusive.

Adult patients with double-inlet left ventricle and perfectly balanced circulation may survive into the sixth decade with good functional capacity and preserved ventricular function. This should be considered before such patients are referred for a Fontan repair.

Objectives: The objective of this study was to determine the frequency of left atrial thrombus in patients with acute atrial fibrillation., Background: It is commonly assumed but unproved that left atrial thrombus in patients with atrial fibrillation begins to form after the onset of atrial fibrillation and that it requires > or = 3 days to form. Thus, patients with acute atrial fibrillation (i.e., < 3 days) frequently undergo cardioversion without anticoagulation prophylaxis., Methods: Three hundred seventeen patients (250 men, 67 women; mean [+/- SD] age 64 +/- 12 years) with acute (n = 143) or chronic (n = 174) atrial fibrillation were studied by two-dimensional transesophageal echocardiography., Results: Left atrial appendage thrombus was present in 20 patients (14%) with acute and 47 patients (27%, p < 0.01) with chronic atrial fibrillation. In patients with a recent embolic event, the frequency of left atrial appendage thrombus did not differ between those with acute (5 [21%] of 24) and those with chronic (12 [23%] of 52, p = NS) atrial fibrillation. Patients with acute versus chronic atrial fibrillation, respectively, did not differ (p = NS) in mean age (64 +/- 13 vs. 65 +/- 11 years), frequency of concentric left ventricular hypertrophy (32% vs. 26%), hypertension (32% vs. 41%), coronary artery disease (35% vs. 39%), congestive heart failure (43% vs. 48%), mitral stenosis (4% vs. 7%) or mitral valve replacement (1.4% vs. 6%). The minimally detectable difference in proportions between patients with acute and chronic atrial fibrillation based on a power of 0.80 and base proportion of 0.20 was 14%., Conclusions: Left atrial thrombus does occur in patients with acute atrial fibrillation < 3 days in duration. The frequency of left atrial thrombus in patients with recent emboli is comparable between those with acute and chronic atrial fibrillation. These data suggest that patients with acute atrial fibrillation for < 3 days require anticoagulation prophylaxis or evaluation by transesophageal echocardiography before cardioversion and should not be assumed to be free of left atrial thrombus.

Atrial pacing and dipyridamole transesophageal echocardiography have been shown to be sensitive and specific tests for the detection of coronary artery disease. However, the sensitivity and specificity of dobutamine transesophageal echocardiography have not been reported. The purpose of this study was to determine the feasibility, sensitivity, and specificity of dobutamine transesophageal echocardiography for the detection of coronary artery disease. Transesophageal echocardiographic assessment of left ventricular function was performed in 81 adult patients aged 62 +/- 12 years during stepwise infusion of dobutamine from 5.0 to 40 micrograms/kg/min. Ischemia was diagnosed by the development of severe hypokinesis, akinesis, or dyskinesis of a previously contractile left ventricular segment. Coronary artery disease was defined by angiography as a reduction in luminal diameter of > or = 70% of an epicardial or > or = 50% of the left main coronary artery. In patients who had undergone coronary artery bypass graft surgery, a stenotic bypass graft was defined as a reduction in luminal diameter of > or = 70%. In patients without previous CABG, significant coronary artery disease was present in 21 patients: 5 with single-vessel disease, 7 double-vessel disease, 8 triple-vessel disease, and 1 left main coronary disease. Dobutamine transesophageal echocardiography had a sensitivity of 90% (19 of 21) and specificity of 94% (49 of 52) for the detection of coronary artery disease. In patients with previous CABG (n = 8), the sensitivity and specificity for the detection of bypass graft stenosis were 100% (4 of 4) and 75% (3 of 4), respectively.(ABSTRACT TRUNCATED AT 250 WORDS)

Two-dimensional transthoracic echocardiography has been shown to be a reliable and accurate method of measuring stenotic mitral valve orifice area. Little data exist on the role of two-dimensional transesophageal echocardiography for this purpose. Thus in 45 adult patients with mitral stenosis mitral valve area was determined by direct planimetry with the use of two-dimensional transesophageal and transthoracic echocardiography. Transesophageal was less feasible than transthoracic echocardiography in the 45 patients (69% vs. 89%, p < 0.025). In 14 patients, two-dimensional transesophageal echocardiography was not feasible, primarily because of leaflet dropout. In 30 patients, transesophageal and transthoracic echocardiography were feasible, and measurements of mitral valve area by the two techniques correlated well (r = 0.91, SEE = 0.33 cm2, p < 0.0001). Mean mitral valve orifice area determined by transesophageal echocardiography (1.54 +/- 0.75 cm2; range 0.56 to 3.49 cm2) and by transthoracic echocardiography (1.55 +/- 0.78 cm2; range 0.62 to 3.68 cm2) did not differ (p = NS). The absolute (0.24 +/- 0.22 cm2) and percent (19% +/- 21%) differences between mitral valve area determined by transesophageal versus transthoracic echocardiography were small. These data show that mitral valve area in patients with mitral stenosis can be accurately measured by direct planimetry with two-dimensional transesophageal echocardiography. Technical refinements such as lateral-gain-compensation features may improve the feasibility of two-dimensional transesophageal echocardiography for measurements of mitral stenosis area, and this technique may become an adjunct to transthoracic echocardiography in the assessment of severity of mitral stenosis.