Tilt Training Increases Vasoconstrictor Reserve in Patients with Neurocardiogenic Syncope

Neurocardiogenic syncope (NCS) is a common clinical entity resulting from an excessive reflex autonomic response, particularly during orthostatism. Treatment options are controversial and of limited effectiveness. Tilt training (TT) is a promising option to treat these patients. However, its mechanism of action and clinical impact remain unclear. OBJECTIVE: To characterize hemodynamic and autonomic responses during a TT program in patients with NCS refractory to conventional measures. METHODS: We studied 28 patients (50% male, mean age 41±14 years) without structural heart disease, with NCS documented by tilt testing. The TT program included 9 tilt sessions (3 times a week, 30 min) (60° - 6 sessions, 70° - 3 sessions), under ECG and blood pressure monitoring combined with home orthostatic self-training and 10° head-up during sleep. Systolic volume, cardiac output, total peripheral resistance, baroreflex sensitivity and heart-rate variability were computed. Patients were reassessed at 1 month and every 6 months for a maximum of 36 months (24±12 months). RESULTS: Over the course of the TT program there was a significant increase in total peripheral resistance (1485±225 vs. 1591±187 dyn·s·cm(-5), p<0.05), with a decrease in standard deviation (206±60 vs. 150±42, p<0.05). During follow-up, syncope recurred in five patients (19%), with a significant reduction in the number of episodes (4.0±3.2/patient in the 12 months before TT vs. 1.4±0.8/patient post-TT, p<0.05). CONCLUSION: In refractory NCS, TT may be an effective therapeutic option, with long-term benefits. These results appear to be due to an increase in vasoconstrictor reserve combined with a reduction in its variance.

Volumoso Pseudoaneurisma do Ventrículo Esquerdo após Cirurgia Cardíaca

A 75-year old female patient, with previous inferior acute myocardial infarction (AMI) in December 2000, was admitted in April 2001 with angina and heart failure. Transthoracic echocardiography (TTE) was suggestive of a postero-inferior pseudoaneurysm (PA) of the left ventricle (LV), with 61x49 mm. of size and mitral regurgitation. Cardiac catheterization was suspected of a PA of the LV and revealed a three vessels coronary artery disease. On 20th April she was submitted to cardiac surgery with resection of a large LV aneurysm (AN) and triple coronary artery bypass surgery. Afterwards, she was on NYHA class III and subsequent TTE and transesophagic echocardiography (TEE) were suggestive of a 90x60 mm LV posterior PA (confirmed by nuclear magnetic resonance) and severe mitral regurgitation, with good LV systolic function. She underwent a new cardiac surgery on 31st May 2002, with resuturing of the LV postero-inferior wall patch and removal of the PA. The patient is in good condition and on NYHA functional class I-II.

Results of Surgery for Ebstein Anomaly: a Multicenter Study from the European Congenital Heart Surgeons Association

OBJECTIVE: Since most centers' experience with Ebstein anomaly is limited, we sought to analyze the collective experience of participating institutions of the European Congenital Heart Surgeons Association with surgery for this rare malformation. METHODS: The records of all 150 patients (median age 6.4 years) who underwent surgery for Ebstein anomaly in the 13 participating Association centers between January 1992 and January 2005 were reviewed retrospectively. Patients with congenitally corrected transposition were excluded. RESULTS: Most patients (81%) had Ebstein disease type B or C and significant functional impairment (61% in New York Heart Association class III or IV) and 16% had prior operations. Surgical procedures (n = 179) included valve replacement (n = 60, 33.5%), valve repair (n = 49, 27.3%), 1(1/2) ventricle repair (n = 46, 25.6%), palliative shunt (n = 13, 7.26%), and other complex procedures (n = 11, 6.14%). There were 20 hospital deaths (operative mortality 13.3%) after valve replacement in 5 patients, valve repair in 3, 1(1/2) ventricle repair in 7, palliative procedures in 3, and miscellaneous procedures in 2. Younger age and palliative procedures were univariate risk factors for operative death, but only age was an independent predictor on multivariable analysis. CONCLUSIONS: Most patients coming to surgery presented in childhood and were significantly symptomatic. More than half underwent valve replacement or repair, but a considerable proportion had severe disease necessitating 1(1/2) ventricle repair or palliative procedures. Operative mortality did not differ significantly among repair, replacement, and 1(1/2) ventricle repair but was associated with palliative procedures for severe disease early in life, young age being the only independent predictor of operative death.

Unexpected Triggers for Pheochromocytoma-Induced Recurrent Heart Failure

Pheochromocytoma crisis typically presents as paroxysmal episodes of headache, tachycardia, diaphoresis or hypertension. We describe an uncommon case of recurrent non-hypertensive heart failure with systolic dysfunction in a young female due to pheochromocytoma compression. It presented as acute pulmonary oedema while straining during pregnancy and later on as cardiogenic shock after a recreational body massage. Such crisis occurring during pregnancy is rare. Moreover, of the few reported cases of pheochromocytoma-induced cardiogenic shock, recreational body massage has not yet been reported as a trigger for this condition.

The Proarrhythmic Effect of Cardiac Resynchronization Therapy: an Issue that Should Be Borne in Mind

The demonstrated benefits of cardiac resynchronization therapy (CRT) in reducing mortality and hospitalizations for heart failure, improving NYHA functional class and inducing reverse remodeling have led to its increasing use in clinical practice. However, its potential contribution to complex ventricular arrhythmias is controversial.We present the case of a female patient with valvular heart failure and severe systolic dysfunction, in NYHA class III and under optimal medical therapy, without previous documented ventricular arrhythmias. After implantation of a CRT defibrillator, she suffered an arrhythmic storm with multiple episodes of monomorphic ventricular tachycardia (VT), requiring 12 shocks. Subsequently, a pattern of ventricular bigeminy was observed, as well as reproducible VT runs induced by biventricular pacing. Since no other vein of the coronary sinus system was accessible, it was decided to implant an epicardial lead to stimulate the left ventricle, positioned in the left ventricular mid-lateral wall. No arrhythmias were detected in the following six months. This case highlights the possible proarrhythmic effect of biventricular pacing with a left ventricular lead positioned in the coronary sinus venous system.

Long-Term Survival with Heart Transplantation for Fibrosarcoma of the Heart

Primary sarcoma of the heart is a rare disease that has an ominous prognosis with either medical or surgical therapy. We report a case of a 25-year-old woman with sarcoma of the heart who received a transplant and is clinically well after 7 years. We believe that transplantation must be considered in this kind of pathology for selected cases.

Enfarte Agudo do Miocárdio na Criança

A retrospective study was made of 6 children, with nonsurgical-related acute myocardial infarction (AMI), between January 1987 and December 1994. The ratio for gender was 1 and mean age at AMI was 49 days, 4 cases being associated with congenital heart disease (Fallot's tetralogy, truncus arteriosus and DiGeorge syndrome, one case each, and anomalous origin of left coronary artery, 2 cases). Kawasaki disease and coronary embolisation from thrombosis of the renal vein occurred in the other 2 cases respectively. All developed congestive cardiac failure and cardiomegaly. In the ECG pathologic q waves with more than 35 msec occurred in all, and QT prolongation occurred in 3. Five children (83%) all with AMI in the anterior and lateral wall of the left ventricle died, death being related with cardiac mechanical failure and not with arrhythmias.

Risk of Surgery for Congenital Heart Disease in the Adult: a Multicentered European Study

BACKGROUND: Surgery for congenital heart disease (CHD) has changed considerably during the last three decades. The results of primary repair have steadily improved, to allow treating almost all patients within the pediatric age; nonetheless an increasing population of adult patients requires surgical treatment. The objective of this study is to present the early surgical results of patients who require surgery for CHD in the adult population within a multicentered European study population. METHODS: Data relative to the hospital course of 2,012 adult patients (age > or = 18 years) who required surgical treatment for CHD from January 1, 1997 through December 31, 2004 were reviewed. Nineteen cardiothoracic centers from 13 European countries contributed to the data collection. RESULTS: Mean age at surgery was 34.4 +/- 14.53 years. Most of the operations were corrective procedures (1,509 patients, 75%), followed by reoperations (464 patients, 23.1%) and palliative procedures (39 patients, 1.9%). Six hundred forty-nine patients (32.2%) required surgical closure of an isolated ostium secundum atrial septal defect. Overall hospital mortality was 2%. Preoperative cyanosis, arrhythmias, and NYHA class III-IV, proved significant risk factors for hospital mortality. Follow-up data were available in 1,342 of 1,972 patients (68%) who were discharged home. Late deaths occurred in 6 patients (0.5%). Overall survival probability was 97% at 60 months, which is higher for corrective procedures (98.2%) if compared with reoperations (94.1%) and palliations (86.1%). CONCLUSIONS: Surgical treatment of CHD in adult patients, in specialized cardiac units, proved quite safe, beneficial, and low-risk.

Utilidade de Ecocardiografia da Sobrecarga Farmacológica com Dobutamina nas Cardiomiopatias Dilatadas não Isquémicas

INTRODUCTION: Low-dose dobutamine stress echocardiography is a common and useful technique to assess myocardial viability in patients with ischemic cardiomyopathy. OBJECTIVE: To evaluate the use of low-dose dobutamine stress echocardiography in determining the functional status of patients with idiopathic dilated cardiomyopathy (IDCM). METHODS: Prospective study of 28 patients with IDCM by transthoracic echocardiography (2D), low-dose dobutamine stress echocardiography, cardiopulmonary exercise testing (CPET) and measurement of pro-BNP. RESULTS: The mean age of the population was 50.3 +/- 11.5 years, 9 female and 19 male. Mean ejection fraction was 32.1 +/- 9.8%. All were in sinus rhythm. The following parameters were analyzed in 2D echocardiography and after dobutamine: dimensions of left atrium (LA) and of left ventricle in diastole and systole, shortening fraction (%), left ventricular end-diastolic (EDV) and end-systolic volumes (ESV), ejection fraction (EF), and mitral inflow (E, A, E/A ratio and deceleration time). In CPET, we considered the following parameters: peak VO2 and % maximal peak VO2 attained. We compared echo results with CPET. There was a correlation between age and peak VO2 (r = -0.38 with p = 0.049). In 2D echo, there was a correlation between baseline EF and LA dimensions and peak VO2 (r = 0.45 / p = 0.004 and r = -0.49 / p = 0.014, respectively). After dobutamine echo, there was a correlation between some echo parameters and peak VO2: EF - r = 0.59 / p = 0.001, LA dimensions - r = 0.56 / p = 0.007, and ESV - r = -0.45 / p = 0.026. Percentage maximal peak VO2 attained correlated with LA dimensions measured in 2D echo and after dobutamine (r = -0.398 / p = 0.036 and r = -0.674 / p = 0.02 respectively) and EF after dobutamine (r = -0.389 / p = 0.04). The value of pro-BNP correlated with LA dimensions and baseline EF (r = 0.44 / p = 0.02 and r = -0.57 / p = 0.002, respectively), and the correlation was maintained after inotropic stimulation with dobutamine (r = 0.57 / p = 0.001 and r = -0.55 / p = 0.0039). CONCLUSION: Low-dose dobutamine stress echocardiography showed stronger correlations with cardiopulmonary exercise testing than the parameters evaluated by conventional echocardiography and could be used to determine the functional status of patients with dilated cardiomyopathy; patients with greater ejection fraction after inotropic stimulation had better cardiopulmonary tests.

Variáveis Ecocardiográficas Predizentes de Terapêuticas Apropriadas de Taquidisritmias Ventriculares em Doentes Submetidos a Terapêutica de Ressincronização Cardíaca Combinada

INTRODUCTION: The significant risk of sudden arrhythmic death in patients with congestive heart failure and electromechanical ventricular dyssynchrony has led to increased use of combined cardiac resynchronization therapy defibrillator (CRT-D) devices. OBJECTIVES: To evaluate the echocardiographic variables in patients undergoing CRT-D that predict the occurrence of appropriate therapies (AT) for ventricular tachyarrhythmia. METHODS: We analyzed 38 consecutive patients (mean age 60 +/- 12 years, 63% male) with echocardiographic evaluation before and 6 months after CRT-D implantation. Patients with AT were identified in a mean follow-up of 471 +/- 323 days. A standard echocardiographic study was performed including tissue Doppler imaging (TDI). Responders were defined as patients with improvement in NYHA class of < or = 1 in the first six months, and reverse remodeling as a decrease in left ventricular end-systolic volume of < or = 15% and/or an increase in left ventricular ejection fraction of > 25%. RESULTS: The responder rate was 74%, and the reverse remodeling rate was 55%. AT occurred in 21% of patients, who presented with greater left ventricular end-diastolic internal diameter (LVEDD) before implantation (86 +/- 8 vs. 76 +/- 11 mm, p = 0.03) and at 6 months (81 +/- 8 vs. 72 +/- 14 mm, p = 0.08), and increased left ventricular end-systolic internal diameter (66 +/- 14 vs. 56 +/- 14 mm, p = 0.03) and lower ejection fraction (24 +/- 6 vs. 34 +/- 14%, p = 0.08) at 6 months. In the group with AT, the responder rate was lower (38 vs. 83%, p = 0.03), without significant differences in reverse remodeling (38% for the AT group vs. 60%, p = 0.426) or in the other variables. By univariate analysis, predictors of AT were LVEDD before implantation and E' after implantation. Age, gender, ischemic etiology, use of antiarrhythmic drugs, reverse remodeling and the other echocardiographic parameters did not predict AT. In multivariate logistic regression analysis, both LVEDD before implantation (OR 1.24, 95% CI 1.04-1.48, p = 0.019) and postimplantation E' (OR 0.27, 95% CI 0.09-0.76, p = 0.014) remained as independent predictors of AT. CONCLUSIONS: In patients undergoing CRT-D, episodes of ventricular tachyarrhythmia occur with high incidence, independently of echocardiographic response, with LVEDD before implantation and E' after implantation as the only independent predictors of AT in the medium-term. These results highlight the importance of combined devices with defibrillation capability.

Utilidade do Doppler Tecidular na Predição de Eventos Arrítmicos em Adultos com Tetralogia de Fallot Corrigida

INTRODUCTION: Adults with repaired tetralogy of Fallot (TOF) may be at risk for progressive right ventricular (RV) dilatation and dysfunction, which is commonly associated with arrhythmic events. In frequently volume-overloaded patients with congenital heart disease, tissue Doppler imaging (TDI) is particularly useful for assessing RV function. However, it is not known whether RV TDI can predict outcome in this population. OBJECTIVE: To evaluate whether RV TDI parameters are associated with supraventricular arrhythmic events in adults with repaired TOF. METHODS: We studied 40 consecutive patients with repaired TOF (mean age 35 +/- 11 years, 62% male) referred for routine echocardiographic exam between 2007 and 2008. The following echocardiographic measurements were obtained: left ventricular (LV) ejection fraction, LV end-systolic volume, LV end-diastolic volume, RV fractional area change, RV end-systolic area, RV end-diastolic area, left and right atrial volumes, mitral E and A velocities, RV myocardial performance index (Tei index), tricuspid annular plane systolic excursion (TAPSE), myocardial isovolumic acceleration (IVA), pulmonary regurgitation color flow area, TDI basal lateral, septal and RV lateral peak diastolic and systolic annular velocities (E' 1, A' 1, S' 1, E' s, A' s, S' s, E' rv, A' rv, S' rv), strain, strain rate and tissue tracking of the same segments. QRS duration on resting ECG, total duration of Bruce treadmill exercise stress test and presence of exercise-induced arrhythmias were also analyzed. The patients were subsequently divided into two groups: Group 1--12 patients with previous documented supraventricular arrhythmias (atrial tachycardia, fibrillation or flutter) and Group 2 (control group)--28 patients with no previous arrhythmic events. Univariate and multivariate analysis was used to assess the statistical association between the studied parameters and arrhythmic events. RESULTS: Patients with previous events were older (41 +/- 14 vs. 31 +/- 6 years, p = 0.005), had wider QRS (173 +/- 20 vs. 140 +/- 32 ms, p = 0.01) and lower maximum heart rate on treadmill stress testing (69 +/- 35 vs. 92 +/- 9%, p = 0.03). All patients were in NYHA class I or II. Clinical characteristics including age at corrective surgery, previous palliative surgery and residual defects did not differ significantly between the two groups. Left and right cardiac chamber dimensions and ventricular and valvular function as evaluated by conventional Doppler parameters were also not significantly different. Right ventricular strain and strain rate were similar between the groups. However, right ventricular myocardial TDI systolic (Sa: 5.4+2 vs. 8.5 +/- 3, p = 0.004) and diastolic indices and velocities (Ea, Aa, septal E/Ea, and RV free wall tissue tracking) were significantly reduced in patients with arrhythmias compared to the control group. Multivariate linear regression analysis identified RV early diastolic velocity as the sole variable independently associated with arrhythmic history (RV Ea: 4.5 +/- 1 vs. 6.7 +/- 2 cm/s, p = 0.01). A cut-off for RV Ea of < 6.1 cm/s identified patients in the arrhythmic group with 86% sensitivity and 59% specificity (AUC = 0.8). CONCLUSIONS: Our results suggest that TDI may detect RV dysfunction in patients with apparently normal function as assessed by conventional echocardiographic parameters. Reduction in RV early diastolic velocity appears to be an early abnormality and is associated with occurrence of arrhythmic events. TDI may be useful in risk stratification of patients with repaired tetralogy of Fallot.

Cirurgias de Derivação do Ventrículo Pulmonar

Cavopulmonary connections have been extensively used in the palliation of complex forms of congenital heart disease requiring some form of right heart bypass. We examine the mid term outcomes of pulmonary ventricle bypass operations in a single institution and performed by the same surgical team. POPULATION: Between March 1999 and April 2006, 62 patients underwent pulmonary ventricle bypass operations: bidirectional cavopulmonary anastomosis (Glenn procedure), total cavopulmonary connections (Fontan procedure) and one and a half ventricle correction in two cases. Age at operation averaged three years (range: 0.42-25 years) for the Glenn procedure and seven years (range: 3-14 years) for the Fontan procedure. There were 36 male patients (58%) and 26 female patients (42%). The most common indication for surgery was the single ventricle defect, present in 66% of patients. Associated lesions included: transposition of the great arteries in 16 patients (35.6%), bilateral superior vena cava in four patients (8.9%), situs ambigus in five patients (11%), situs inversus in another patient (2.2%), Ebstein disease in one patient (2.2) and coronary fistula in another patient (2.2%). Sub-aortic stenosis was present in one patient (2.2%). Palliative surgery was performed in all, but three patients (5%), before the Fontan procedure. RESULTS: Thirty two patients underwent bidirectional cavopulmonary anastomosis and thirty patients underwent cavopulmonary connections, total or 2nd stage. Mean cardiopulmonary bypass times were 50.6+/-21.9 minutes for the Glenn procedure and 88.5+/-26.3 minutes for the Fontan procedure. There was no intra-operative mortality, but two patients (3.2% (died in the first month after surgery; one due to failure of the Glenn circuit and sepsis and the other due to a low cardiac output syndrome and multi-organ dysfunction. Mean ventilation time was 5.2+/-1.7 hours for the Glenn operation and 6.2+/-3.2 hours for the Fontan operation. The mean length of stay in ICU was 3.4+/-2.8 days for patients undergoing the Glenn operation and 4.6+/-3.1 days for patients undergoing the Fontan operation and the mean length of hospital stay was 10.6+/-5.8 days for the Glenn operation and 19.1+/-12.6 days for the Fontan operation respectively. The mean follow up time was 4+/-2.1 years (minimum 0 years and maximum seven years), most patients being in NYHA class I. Epicardiac pacemakers were implanted in three patients due to arrhythmias. Two re-operations (6.7%) were needed, both in the same patient, after the Fontan procedure, this patient eventually died a few years after surgery. CONCLUSIONS: The immediate and mid term outcomes of pulmonary ventricle bypass operations can have excellent results. From our point of view there has been an improvement, namely in the use of the extracardiac conduit technique in the 2nd stage of the Fontan operation.

Importância Prognóstica do Doppler Tecidular nos Doentes com Miocardiopatia Dilatada

Previous studies have shown that a ratio of early transmitral flow velocity to early diastolic velocity of the mitral annulus (E/E') of > 15, obtained by tissue Doppler imaging (TDI), correlates with left ventricular filling pressure. OBJECTIVE: The aim of our study was to assess whether E/E' provides prognostic information in patients with dilated cardiomyopathy. METHODS: We studied 33 patients with dilated cardiomyopathy and mean ejection fraction of 31%. All the patients underwent routine two-dimensional and Doppler echocardiographic examination and TDI to determine early peak velocity of the mitral annulus. Pro-B-type natriuretic peptide (pro-BNP) and peak oxygen consumption (VO2max) were also measured. Patients were divided into two groups according to the value of E/E': Group I (n = 15 patients) with E/E' > or = 15 and Group II (n = 18 patients) with E/E' < 15. Patients were followed for 12+/-4 months; new hospital admission due to heart failure, heart transplantation and death were considered as cardiac events. RESULTS: There were significant differences between the two groups in conventional two-dimensional echocardiographic measurements (dimensions and ejection fraction) and Doppler parameters (mitral inflow). With regard to mitral annular velocities obtained by TDI at two different points (septum and lateral wall), the E', A' and S' velocities differed significantly between the two groups, with lower velocities in Group I. Systolic velocity measured in the lateral portion of the mitral annulus showed the most significant difference: Group I - 4.46 cm/sec versus Group II - 7.19 cm/sec, p < 0.00001. Pro-BNP was 5622 pg/ml in Group I, and 1254 pg/ml in Group II, p = 0.004. VO2 max was significantly different between the two groups: Group I - 17.6 ml/kg/min versus Group II - 22.8 ml/kg/min, p = 0.004. During follow-up, events were more common in Group I, with 9 patients (60%) having events, while in Group II, the event rate was 11.1% (2 patients), p = 0.004. CONCLUSION: The ratio of early transmitral flow velocity to early diastolic velocity of the mitral annulus is a powerful predictor of clinical outcome. Lower velocities of mitral annulus on TDI are expected in patients with E/E' > or = 15. Systolic velocities of under 5 cm/sec measured in the lateral portion of the mitral annulus appeared to be strongly related to prognosis.

The Arterial Switch Operation in Europe for Transposition of the Great Arteries: a Multi-Institutional Study from the European Congenital Heart Surgeons Association

OBJECTIVES: This study analyzes the results of the arterial switch operation for transposition of the great arteries in member institutions of the European Congenital Heart Surgeons Association. METHODS: The records of 613 patients who underwent primary arterial switch operations in each of 19 participating institutions in the period from January 1998 through December 2000 were reviewed retrospectively. RESULTS: A ventricular septal defect was present in 186 (30%) patients. Coronary anatomy was type A in 69% of the patients, and aortic arch pathology was present in 20% of patients with ventricular septal defect. Rashkind septostomy was performed in 75% of the patients, and 69% received prostaglandin. There were 37 hospital deaths (operative mortality, 6%), 13 (3%) for patients with an intact ventricular septum and 24 (13%) for those with a ventricular septal defect (P < .001). In 36% delayed sternal closure was performed, 8% required peritoneal dialysis, and 2% required mechanical circulatory support. Median ventilation time was 58 hours, and intensive care and hospital stay were 6 and 14 days, respectively. Although of various preoperative risk factors the presence of a ventricular septal defect, arch pathology, and coronary anomalies were univariate predictors of operative mortality, only the presence of a ventricular septal defect approached statistical significance (P = .06) on multivariable analysis. Of various operative parameters, aortic crossclamp time and delayed sternal closure were also univariate predictors; however, only the latter was an independent statistically significant predictor of death. CONCLUSIONS: Results of the procedure in European centers are compatible with those in the literature. The presence of a ventricular septal defect is the clinically most important preoperative risk factor for operative death, approaching statistical significance on multivariable analysis.