Timing and type of surgery for severe pulmonary regurgitation after repair of tetralogy of Fallot.

Repaired tetralogy of Fallot (rTOF) has an excellent long-term prognosis; however, survival is somewhat less than normal. Of all the residual lesions and sequellae after rTOF, pulmonary regurgitation (PR) is the most important, correlating with right ventricular (RV) size, exercise intolerance and serious ventricular arrhythmias. Pulmonary valve replacement (PVR) has beneficial effects on RV size and function, provided it is performed early, before irreversible RV dysfunction ensues.

Enzyme replacement therapy in severe Fabry disease with renal failure: a 1-year follow-up.

We present here the course of clinical response of a 53-year-old haemodialysed Fabry patient who received recombinant human alpha-galactosidase A at a dose of 1 mg/kg every other week over a period of 1 year. The therapy was well tolerated by the patient, who revealed an impressive favourable cutaneous, gastrointestinal, neurological and psychiatric response and a dramatic improvement in his quality of life, but no improvement in cardiac and renal function.

Mechano-electrical interaction late after Fontan operation: relation between P-wave duration and dispersion, right atrial size, and atrial arrhythmias.

Background: The growing population with Fontan operation surviving into adulthood has significant morbidity and mortality rates from recurrent atrial tachyarrhythmias. We hypothesized that the structural characteristics and electrical behavior of atria may differ in these patients compared with those without arrhythmias.

Methods And Results: We studied 33 consecutive patients (age, 25.

Doppler-echocardiographic assessment of pulmonary regurgitation in adults with repaired tetralogy of Fallot: comparison with cardiovascular magnetic resonance imaging.

Aims: The purpose of this study was to compare the noninvasive assessment of severity of pulmonary regurgitation with Doppler echocardiography versus cardiovascular magnetic resonance imaging (CMR) in adult patients with repaired tetralogy of Fallot (rTOF).

Methods: We studied 52 (22 females) consecutive patients (aged 32 +/- 2 years, 23 +/- 5 years after rTOF) using Doppler echocardiography and compared these findings with CMR. From the continuous-wave Doppler trace, the duration of pulmonary regurgitation and of total diastole was measured and the ratio between the 2 was defined as pulmonary regurgitation index (PRi).

Global impairment of cardiac autonomic nervous activity late after the Fontan operation.

Background: Atrial tachyarrhythmia is a common cause of morbidity and mortality in patients with univentricular physiology undergoing the Fontan operation. We examined cardiac autonomic nervous activity, a predictor of arrhythmia and sudden death in other cardiovascular disease, in patients late after the Fontan operation, employing heart rate variability (HRV) and baroreflex sensitivity.

Methods And Results: We measured HRV and baroreflex sensitivity in 22 consecutive patients (8 male, age 26+/-9 years) who had undergone the Fontan operation 13+/-6 years previously, and 22 age- and sex-matched healthy controls.

Comparison of equilibrium radionuclide ventriculography with cardiovascular magnetic resonance for assessing the systemic right ventricle after Mustard or Senning procedures for complete transposition of the great arteries.

We assessed the utility of equilibrium radionuclide ventriculography for assessing function of the systemic right ventricle by comparing it with cardiovascular magnetic resonance (CMR) in subjects who had undergone the Mustard or Senning operations for complete transposition of the great arteries. The 95% limits of agreement for right ventricular ejection fraction by the 2 techniques were 15.8% to -16.

Elevated circulating levels of inflammatory cytokines and bacterial endotoxin in adults with congenital heart disease.

Chronic heart failure is a state of immune activation, and endotoxin is a potential trigger for cytokine production. Our aim was to study whether immune activation and endotoxemia occur in adults with congenital heart disease. We prospectively measured tumor necrosis factor (TNF)-alpha, soluble TNF receptors (sTNFR-1, sTNFR-2), interleukin-6, interleukin-10, endotoxin, and soluble CD14 levels in 52 consecutive adults with congenital heart disease (age 34 +/- 2 years [mean +/- SEM]) and 18 healthy controls (age 31 +/- 1 years).

Prognostic significance of coronary artery calcium in asymptomatic subjects with usual cardiovascular risk.

Background: Coronary calcium detected noninvasively is an attractive way to diagnose atherosclerosis before the development of symptoms. This study examines the prognostic value of coronary calcium in asymptomatic subjects with usual cardiovascular risk.

Methods And Results: In 425 asymptomatic subjects, 229 men (aged 45.

Prednisolone and azathioprine in IgA nephropathy - a ten-year follow-up study.

Background: Immunoglobulin A nephropathy (IgAN) is one of the most common primary glomerular diseases. Although its clinical course is usually benign, some patients develop end-stage renal failure (ESRF). The role of immunosuppressive drugs in the treatment of IgAN remains controversial.

Coronary calcium detected by digital cinefluoroscopy and coronary artery disease in patients undergoing coronary arteriography: effects of age and sex.

Background: Coronary artery calcium, detected non-invasively, correlates well with angiographically documented coronary artery disease (CAD). This study was conducted to evaluate the diagnostic efficacy of coronary artery calcium detected by digital cinefluoroscopy for CAD and assess the effects of age and sex on it.

Methods: In 242 patients who underwent coronary angiography, coronary calcium status was determined and related to angiographic findings.

Right ventricular function in adults with repaired tetralogy of Fallot assessed with cardiovascular magnetic resonance imaging: detrimental role of right ventricular outflow aneurysms or akinesia and adverse right-to-left ventricular interaction.

Objectives: We examined the relationship among biventricular hemodynamics, pulmonary regurgitant fraction (PRF), right ventricular outflow tract (RVOT) aneurysm or akinesia, and baseline and surgical characteristics in adults with repaired tetralogy of Fallot (rTOF).

Background: The precise relationship of pulmonary regurgitation with biventricular hemodynamics has been hampered by limitations of right ventricular (RV) imaging.

Methods: We assessed 85 consecutive adults with rTOF and 26 matched healthy controls using cardiovascular magnetic resonance imaging.

Global impairment of cardiac autonomic nervous activity late after repair of tetralogy of Fallot.

Background: Sustained ventricular tachycardia (VT) and sudden cardiac death (SCD) remain devastating late complications after repair of Tetralogy of Fallot (ToF). Although heart rate variability (HRV) and baroreflex sensitivity (BRS) are recognized as independent markers of autonomic activity and strong predictors of SCD in major cardiovascular disease, little is known about their role in patients with tetralogy.

Methods And Results: We measured HRV and BRS in 45 ToF patients (27 male, age 35+/-12 years, 26+/-7 years after repair) and 45 matched healthy controls.

In-vivo testing of digital cellular telephones in patients with implantable cardioverter-defibrillators.

Aims: To investigate the susceptibility of implantable cardioverter defibrillators to electromagnetic interference generated by digital cellular telephones, functioning in both international transmission technologies: the Global System for Mobile Communication (GSM) and the Digital Cellular System (DCS 1800).

Methods And Results: In 36 patients with transvenous implantable cardioverter defibrillators from two manufacturers (Medtronic and Guidant/CPI), cellular telephones with different levels of minimal and maximal power output were tested in the transmitting and receiving mode. Evaluation was performed in activated implantable defibrillators during spontaneous cardiac activity and continuous VVI or DDD pacing to assess possible electromagnetic interference.

Intravenous magnesium sulfate versus diltiazem in paroxysmal atrial fibrillation.

Background: Drugs currently available for the acute treatment of paroxysmal atrial fibrillation have significant limitations. We assessed the safety and effectiveness of intravenous magnesium sulfate versus diltiazem therapy in patients with prolonged episodes of paroxysmal atrial fibrillation.

Methods: In a prospective randomized trial, 46 symptomatic patients presenting with paroxysmal atrial fibrillation were given intravenous magnesium sulfate (n=23) or diltiazem (n=23) therapy.

Diagnostic superiority of continuous-loop electrocardiographic recording over other testing in a patient with recurrent syncope and underlying coronary artery disease with severe left ventricular dysfunction.

Dysrhythmic causes of syncope may elude diagnosis in postinfarction patients despite elaborate testing, including electrophysiologic study. For a correct diagnosis, capture of cardiac rhythm during patient's typical symptoms is crucial. This report describes a patient with coronary artery disease and decreased left ventricular ejection fraction who experienced recurrent syncopal episodes without obvious precipitating factors.

Significance of R-on-T phenomenon in early ventricular tachyarrhythmia susceptibility after acute myocardial infarction in the thrombolytic era.

We investigated the clinical significance and mechanism of the R-on-T phenomenon in the current thrombolytic era as potential precipitant of R-on-T-induced early ventricular tachyarrhythmias in patients with a thrombolysed acute myocardial infarction. We also examined the role of QT dispersion on ventricular vulnerability and its association with R-on-T-initiated ventricular tachyarrhythmias. A total of 93 patients underwent 24-hour Holter monitoring starting at hospital admission before thrombolysis.