Risks and benefits of sharing patient information on social media: a digital dilemma.

Social media (SoMe) has witnessed remarkable growth and emerged as a dominant method of communication worldwide. Platforms such as Facebook, X (formerly Twitter), LinkedIn, Instagram, TikTok, and YouTube have become important tools of the digital native generation. In the field of medicine, particularly, cardiology, attitudes towards SoMe have shifted, and professionals increasingly utilize it to share scientific findings, network with experts, and enhance teaching and learning. Notably, SoMe is being leveraged for teaching purposes, including the sharing of challenging and intriguing cases. However, sharing patient data, including photos or images, online carries significant implications and risks, potentially compromising individual privacy both online and offline. Privacy and data protection are fundamental rights within European Union treaties, and the General Data Protection Regulation (GDPR) serves as the cornerstone of data protection legislation. The GDPR outlines crucial requirements, such as obtaining 'consent' and implementing 'anonymization', that must be met before sharing sensitive and patient-identifiable information. Additionally, it is vital to consider the patient's perspective and prioritize ethical and social considerations when addressing challenges associated with sharing patient information on SoMe platforms. Given the absence of a peer-review process and clear guidelines, we present an initial approach, a code of conduct, and recommendations for the ethical use of SoMe. In conclusion, this comprehensive review underscores the importance of a balanced approach that ensures patient privacy and upholds ethical standards while harnessing the immense potential of SoMe to advance cardiology practice and facilitate knowledge dissemination.

Panic attacks and supraventricular tachycardias: the chicken or the egg?

Panic attacks occur in about 2 % of the population. Symptoms include a racing or pounding heart beat, chest pain, dizziness, light-headedness, nausea, difficulty in breathing, tingling or numbness in the hands, flushes or chills, dreamlike sensations or perceptual distortions. The symptoms of paroxysmal supraventricular tachycardia (PSVT) may be similar. A PSVT is often difficult to document on the ECG since it has often ceased before the patient comes to medical attention. Besides, a tachycardia may still be present and even be documented but interpreted as a phenomenon secondary to the panic attack. In addition, ECG abnormalities between episodes can often not be identified. The evidence that in some patients paroxysmal SVT is the cause, but not the consequence of a panic attack, is based on observations that catheter ablation was able to cure patients presenting with panic disorders. To better establish the prevalence of SVT as the underlying mechanism of a panic attack, there is a need for prospective studies and/or registries. Whereas gastric ulcer has in some patients changed from a psychosomatic disorder to an infectious disease, we may hypothesise that a certain proportion of panic disorders may mutate into an underlying arrhythmia rather than a primary psychiatric disorder.

QT interval prolongation and risk for cardiac events in genotyped LQTS-index children.

Congenital long-QT syndrome (LQTS) is an inherited cardiac disorder with a disturbance in repolarization characterized by a prolonged QT interval on the surface electrocardiogram and life-threatening ventricular tachycardia. Publications from the International LQTS Registry have provided information that the cardiac risk may be influenced by gender, genotype, exposure to arrhythmia triggers, and previous cardiac events. In children, early-onset of disease, changes in life style, and medical treatment is a sensitive issue and significant, gender-related differences of a first life-threatening event were reported. Thus, we investigated the clinical features of a large genotyped population of LQTS-index children (age < or =16 years) upon a single-center experience and determined risk factors for symptoms. Of 83 children [mean corrected QT interval (QTc) 510 +/- 74 ms], 89% had LQT1, -2, or -3. Nine patients (11%) were identified as having Jervell and Lange-Nielsen syndrome. Among symptomatic children (n = 51, 61%), syncope was the most prevalent symptom at initial presentation (49%); however, aborted cardiac arrest (ACA) occurred in 33% and sudden cardiac death (SCD) in 18%, respectively, as the initial manifestation. During a mean follow-up period of 5.9 +/- 4.7 years, 31% of the children developed symptoms while on therapy (86% syncope, 9% ACA, 5% SCD). Statistical analyses of risk factors for cardiac events showed that the QTc >500 ms was a strong and significant predictor for cardiac events during follow-up (p = 0.02). Furthermore, a prior syncope [hazard ratio (HR), 4.05; 95% confidence interval (CI), 1.1 to 15.0; p = 0.03] or an ACA (HR, 11.7; 95% CI, 3.1 to 43.4; p = <0.001) identified children with an increased risk for recurrent cardiac events compared to asymptomatic LQT children. LQTS-index children manifest with a high percentage of severe symptoms. Among presently validated risk factors for LQTS, a QTc interval >500 ms and a history of prior syncope or ACA were strong predictors for recurrent cardiac events.

Exogenous nitric oxide regulates activity and synthesis of vascular endothelial nitric oxide synthase.

BACKGROUND: Nitric oxide (NO) - a major signalling molecule of the vascular system - is constitutively produced in endothelial cells (EC) by the endothelial NO synthase (eNOS). Since a reduced NO synthesis is an early sign of endothelial dysfunction and NO delivering drugs are used to substitute the impaired endothelial NO production, we addressed the effect of exogenous NO on eNOS in human umbilical venous endothelial cell cultures. MATERIALS AND METHODS: The synthetic NO donor DETA/NO (trade name, but in the following we refer to detNO), that releases NO in a strictly first order reaction with a half life of 20 h, was used in our experiments. RESULTS: Short-term (20-30 min) detNO treatment of EC increases the Ser(1177) phosphorylation of the constitutively expressed endothelial NOS and the production of endogenous NO generated by eNOS from [(3)H]arginine. The phosphorylation of eNOS is Akt-dependent and completely reverted by the phosphatidylinositol-3 kinase (PI-3K) inhibitor LY294002. A prolonged continuous exposure of EC to detNO 150 micromol L(-1) over a period of 24-48 h causes a reversible cell cycle arrest at G(1)-phase associated with a larger cell volume and increased cell protein content (hypertrophic phenotype of EC). The eNOS protein and mRNA of the hypertrophic cells and the generation of endogenous NO are reduced but eNOS phosphorylation could still be elevated by stimulation with vascular endothelial growth factor. CONCLUSIONS: Our data explain clinical studies describing a short-term but not a long-term benefit of NO treatment for patients with cardiovascular risk factors. The results could be a rational approach to develop a generation of NO donors accomplishing a retarded release from NO donors that mimic the low continuous pulsatile stress-induced release of endogenous NO.

xIAP induces cell-cycle arrest and activates nuclear factor-kappaB : new survival pathways disabled by caspase-mediated cleavage during apoptosis of human endothelial cells.

Survival of human vascular endothelial cells depends on their ability to activate the transcription factor nuclear factor-kappaB (NF-kappaB), a regulator of antiapoptotic genes, such as the X chromosome-linked inhibitor of apoptosis protein (xIAP). In the present study, we demonstrated expression of xIAP in the endothelial lining of normal human arteries and veins and elevated levels in highly malignant human endothelial tumors. Using retroviral infection of human endothelial cells, we identified two novel survival mechanisms mediated by xIAP in endothelial cells. First, xIAP can activate the transcription factor NF-kappaB, a known survival factor for human endothelial cells. This positive feedback loop induced by xIAP is mediated via phosphorylation and sustained degradation of inhibitor (I) kappaBalpha. Second, xIAP can inhibit cell proliferation via downregulation of cyclins A and D1 and induction of the cyclin-dependent kinase inhibitors p21(Cip1/Waf1) and p27(Kip1). Cleavage of xIAP by caspases during endothelial cell apoptosis disables both of these biological functions of xIAP. Thus, caspase-mediated cleavage of xIAP interrupts a positive regulatory cytoprotective loop between NF-kappaB and xIAP and increases the vulnerability of the cell to apoptosis by releasing it from an xIAP-mediated quiescent state.

Abnormalities of cardiac sympathetic innervation in arrhythmogenic right ventricular cardiomyopathy : quantitative assessment of presynaptic norepinephrine reuptake and postsynaptic beta-adrenergic receptor density with positron emission tomography.

BACKGROUND: The frequent provocation of ventricular tachycardia by stress or catecholamines and the efficacy of antiarrhythmic drugs with antiadrenergic properties suggest an involvement of the cardiac adrenergic system in arrhythmogenesis in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). Previous studies demonstrated abnormalities of the presynaptic uptake-1 assessed by (123)I-MIBG-single-photon emission computed tomography. METHODS AND RESULTS: This study investigated neuronal reuptake of norepinephrine (uptake-1) and beta-adrenergic receptor density in 8 patients with ARVC and 29 age-matched control subjects. All subjects underwent positron emission tomography with the volume of distribution (V(d)) of [(11)C]hydroxyephedrine ((11)C-HED) used to assess presynaptic norepinephrine reuptake, the maximum binding capacity (B(max)) of [(11)C]CGP-12177 ((11)C-CGP-12177) to assess postsynaptic beta-adrenergic receptor density, and [(15)O]H(2)O for quantification of myocardial blood flow. Patients with ARVC demonstrated a highly significant global reduction in postsynaptic beta-adrenergic receptor density compared with that in control subjects (B(max) of (11)C-CGP-12177: 5.9+/-1.3 vs 10.2+/-2.9 pmol/g tissue, P<0.0007), whereas the presynaptic uptake-1 tended toward reduction only (V(d) of (11)C-HED: 59.1+/-25.2 vs 71.0+/-18.8 mL/g tissue, NS). There were no differences in myocardial blood flow between the groups, and plasma norepinephrine was within normal limits in patients and control subjects. CONCLUSIONS: The findings demonstrate a significant reduction of myocardial beta-adrenergic receptor density in patients with ARVC. This may result from a secondary downregulation after increased local synaptic norepinephrine levels caused by increased firing rates of the efferent neurons or as the result of impaired presynaptic catecholamine reuptake. These findings give new insights into the pathophysiology of arrhythmogenesis in ARVC, with potential impact on diagnostic evaluation and therapeutic management.

Fetoscopic transesophageal electrocardiography and stimulation in fetal sheep: a minimally invasive approach aimed at diagnosis and termination of therapy-refractory supraventricular tachycardias in human fetuses.

BACKGROUND: Therapy-refractory supraventricular tachycardia commonly results in hydrops and death in human fetuses. The purpose of this study in fetal sheep was to assess the feasibility of a minimally invasive fetoscopic approach for fetal transesophageal electrocardiography and stimulation aimed at diagnosis and termination of these tachycardias. METHODS AND RESULTS: We studied a total of 10 fetal sheep (87 to 103 days of gestation; term=145 days). We entered the amniotic cavity using a percutaneous fetoscopic approach and placed various electrophysiology catheters into the fetal esophagus. We recorded the number of animals in which fetoscopic transesophageal electrocardiography and stimulation were successful and assessed pacing success and thresholds for different catheters. In addition, we monitored for potential adverse effects from stimulation and for other complications of the operation. Recording of transesophageal electrocardiograms was successful in all fetal sheep. Capture during stimulation was successfully documented by additional fetal bipolar surface electrocardiograms in 7 fetuses. In fetuses in which fetal surface electrocardiograms were not recorded, pacing stimulus artifacts interfered with documentation of capture. Although stimulation thresholds were high, the maternal rhythm was not affected by fetal stimulation. CONCLUSIONS: Fetoscopic fetal transesophageal electrocardiography and stimulation are feasible in fetal sheep. This minimally invasive approach might have the potential to improve diagnosis and management of therapy-refractory supraventricular tachycardias in human fetuses.

De novo mutation in the SCN5A gene associated with early onset of sudden infant death.

BACKGROUND: Congenital long QT syndrome (LQTS), a cardiac ion channel disease, is an important cause of sudden cardiac death. Prolongation of the QT interval has recently been associated with sudden infant death syndrome, which is the leading cause of death among infants between 1 week and 1 year of age. Available data suggest that early onset of congenital LQTS may contribute to premature sudden cardiac death in otherwise healthy infants. METHODS AND RESULTS: In an infant who died suddenly at the age of 9 weeks, we performed mutation screening in all known LQTS genes. In the surface ECG soon after birth, a prolonged QTc interval (600 ms(1/2)) and polymorphic ventricular tachyarrhythmias were documented. Mutational analysis identified a missense mutation (Ala1330Pro) in the cardiac sodium channel gene SCN5A, which was absent in both parents. Subsequent genetic testing confirmed paternity, thus suggesting a de novo origin. Voltage-clamp recordings of recombinant A1330P mutant channel expressed in HEK-293 cells showed a positive shift in voltage dependence of inactivation, a slowing of the time course of inactivation, and a faster recovery from inactivation. CONCLUSIONS: In this study, we report a de novo mutation in the sodium channel gene SCN5A, which is associated with sudden infant death. The altered functional characteristics of the mutant channel was different from previously reported LQTS3 mutants and caused a delay in final repolarization. Even in families without a history of LQTS, de novo mutations in cardiac ion channel genes may lead to sudden cardiac death in very young infants.

Genotype-phenotype correlation in the long-QT syndrome: gene-specific triggers for life-threatening arrhythmias.

BACKGROUND: The congenital long-QT syndrome (LQTS) is caused by mutations on several genes, all of which encode cardiac ion channels. The progressive understanding of the electrophysiological consequences of these mutations opens unforeseen possibilities for genotype-phenotype correlation studies. Preliminary observations suggested that the conditions ("triggers") associated with cardiac events may in large part be gene specific. METHODS AND RESULTS: We identified 670 LQTS patients of known genotype (LQT1, n=371; LQT2, n=234; LQT3, n=65) who had symptoms (syncope, cardiac arrest, sudden death) and examined whether 3 specific triggers (exercise, emotion, and sleep/rest without arousal) differed according to genotype. LQT1 patients experienced the majority of their events (62%) during exercise, and only 3% occurred during rest/sleep. These percentages were almost reversed among LQT2 and LQT3 patients, who were less likely to have events during exercise (13%) and more likely to have events during rest/sleep (29% and 39%). Lethal and nonlethal events followed the same pattern. Corrected QT interval did not differ among LQT1, LQT2, and LQT3 patients (498, 497, and 506 ms, respectively). The percent of patients who were free of recurrence with ss-blocker therapy was higher and the death rate was lower among LQT1 patients (81% and 4%, respectively) than among LQT2 (59% and 4%, respectively) and LQT3 (50% and 17%, respectively) patients. CONCLUSIONS: Life-threatening arrhythmias in LQTS patients tend to occur under specific circumstances in a gene-specific manner. These data allow new insights into the mechanisms that relate the electrophysiological consequences of mutations on specific genes to clinical manifestations and offer the possibility of complementing traditional therapy with gene-specific approaches.

Ectopic atrial tachycardia after transvenous catheter ablation of a posteroseptal accessory pathway.

Closed chest electrode catheter ablation of a posteroseptal accessory pathway was performed on an 18 year old patient with recurrent supraventricular tachycardia. After the procedure, ectopic atrial tachycardia was observed for the first time. Detailed endocardial atrial mapping revealed that this ectopic atrial tachycardia originated from the first site of catheter ablation. As retrograde conduction resumed, a second ablation procedure was performed, resulting in successful ablation of both the ectopic atrial tachycardia and the accessory pathway conduction.

High frequency alternating current ablation of an accessory pathway in humans.

High frequency alternating current ablation of an accessory pathway was performed in a patient with incessant circus movement tachycardia using a right-sided, free wall accessory pathway. Antiarrhythmic drugs, antitachycardia pacing and transvenous catheter ablation using high energy direct current shocks could not control the supraventricular tachycardia. A 7F bipolar electrode catheter with an interelectrode distance of 1.2 cm was positioned at the site of earliest retrograde activation during circus movement tachycardia. At this area, two alternating current high frequency impulses were delivered with an energy output of 50 W through the distal tip of the bipolar catheter, while the patient was awake. After the first shock supraventricular tachycardia terminated and accessory pathway conduction was absent without altering anterograde conduction in the normal atrioventricular (AV) conduction system. No reports of pain or other complications were noted. In short-term follow-up of 5 months, the patient had been free of arrhythmias without antiarrhythmic medication. Thus, high frequency alternating current ablation was performed for the first time in the treatment of an arrhythmia incorporating an accessory pathway in a human. This technique may be an attractive alternative to the available transcatheter ablation techniques and to antitachycardia surgery.

Reappraisal of criteria for assessing drug efficacy in patients with ventricular tachyarrhythmias: complete versus partial suppression of inducible arrhythmias.

To test whether increased difficulty in inducing ventricular tachycardia during antiarrhythmic therapy can be considered a sufficient criterion for predicting long-term efficacy of such therapy in patients with ventricular tachyarrhythmias, 95 patients were studied with a graded stimulation protocol (single and double premature stimuli during sinus rhythm and ventricular drives of 120, 140, 160 and 180 beats/min). After a control study, the effects of oral antiarrhythmic drugs on the ability to induce ventricular tachycardia were assessed. The median number of drug trials was four per patient. After antiarrhythmic therapy, four subgroups of patients were identified. In 36 patients, there was no change in inducibility (group 1), whereas in 18 patients ventricular tachycardia was rendered more difficult to induce; that is, a sustained ventricular tachycardia was inducible at a basic drive at least 40 beats/min faster than during the control study (group 2). In 34 patients, ventricular tachycardia induction was suppressed (group 3) and in 7 patients with nonsustained ventricular tachycardia, only 3 to 5 repetitive ventricular responses were induced after treatment (group 4). During follow-up of 15.5 +/- 11.5 months, 10 patients of group 1 had a recurrence of ventricular tachycardia and 6 died suddenly, whereas in group 2 only 1 patient died suddenly and in group 3, 2 patients had a recurrence of ventricular tachycardia (group 1 versus 2 and 3, p less than 0.001, Mantel-Cox and Breslow; group 2 versus 3, no difference). Thus, increased difficulty in inducing ventricular tachycardia is a sufficient criterion for predicting long-term efficacy of an antiarrhythmic drug regimen.

Characteristics of local electrogram predicting successful transcatheter radiofrequency ablation of left-sided accessory pathways.

OBJECTIVES: The purpose of this study was to analyze and compare the local electrograms recorded at successful and unsuccessful sites of ablation to identify the criteria that may predict successful sites and minimize unnecessary radiofrequency delivery. BACKGROUND: Transcatheter ablation of accessory pathways using radiofrequency energy requires extremely precise localization of an accessory pathway. METHODS: Local electrograms from 50 consecutive patients with left-sided accessory pathways who underwent transcatheter radiofrequency ablation were analyzed. During catheter ablation, localization of accessory pathways was performed in 39 pathways during pre-excited sinus rhythm and in 14 pathways during orthodromic tachycardia. A total of 429 local electrograms at target sites obtained before delivery of radiofrequency current was analyzed. A prospective study was performed in another 20 patients using the criteria derived from the retrospective study. RESULTS: Accessory pathway conduction block was achieved in 36 (92%) of 39 pathways in which mapping was performed during pre-excited sinus rhythm and in 9 (64%) of 14 pathways in which mapping was performed during orthodromic tachycardia (p less than 0.05). When mapping was performed during pre-excited sinus rhythm, a combination of four variables (that is, an accessory pathway potential, stability of local electrograms, atrial activation greater than 1 mV and ventricular activation preceding the onset of the delta wave) showed a 62% probability of success. In contrast, excluding these variables resulted in a 95% probability of failure (noneffective or transiently effective). The prospective study shows that the use of these criteria can significantly reduce the number of current applications. When mapping was performed during orthodromic tachycardia, recording the earliest atrial activation was the most powerful predictor of success. A stable local electrogram with a small notch on the ventricular potential, presumed to be an accessory pathway potential, may add predictive value. CONCLUSIONS: Transcatheter radiofrequency ablation is highly effective in the treatment of patients with left-sided accessory pathways. Specific characteristics of local electrograms can be important predictors of success or failure. Mapping during pre-excited rhythm renders ablation more effective than does mapping during orthodromic tachycardia.

Ventricular tachycardias above the initially programmed tachycardia detection interval in patients with implantable cardioverter-defibrillators: incidence, prediction and significance.

OBJECTIVES: This retrospective study was performed to provide data on ventricular tachycardias (VT) with a cycle length longer than the initially programmed tachycardia detection interval (TDI) in patients with implantable cardioverter defibrillators (ICDs). BACKGROUND: It has been clinical practice to program a safety margin of 30 to 60 ms between the slowest spontaneous or inducible VT and the TDI. METHODS: Baseline characteristics of 659 consecutive patients with ICDs were prospectively; follow-up information was retrospectively collected. RESULTS: During a mean follow-up of 31+/-23 months, 377 patients (57.2%) had at least one recurrent VT or ventricular fibrillation; 47 patients (7.1%) suffered 61 VTs above the TDI. The risk of a VT above the TDI ranged between 2.7% and 3.5% per year during the first four years after ICD implantation. The difference between the cycle length of the slowest VT before ICD implantation, spontaneous or induced, and the first VT above TDI was 108+/-58 ms. Fifty-four VTs (88.5%) above the TDI were associated with significant clinical symptoms (angina or palpitation 63.9%, heart failure 6.6% and syncope 8.2%). Six patients (9.8%) had to be resuscitated. Kaplan-Meyer analysis identified New York Heart Association class II or III (p = 0.021), ejection fraction < 0.40 (p = 0.027), spontaneous (p<0.001) or inducible (p<0.001) monomorphic VTs and the use of class III antiarrhythmic drugs (amiodarone, p<0.001; sotalol, p = 0.004) as risk predictors of VTs above the TDI. The risk of recurrent VTs above TDI was 11.8%, 12.5% and 26.6% during the first, second and third year after first VT above TDI, respectively. CONCLUSIONS: The risk of VTs above the TDI is significantly increased in some patients, and many VTs above TDI cause significant clinical symptoms. A larger safety margin between spontaneous or inducible VTs and the TDI seems to be necessary in selected patients. This is in conflict with an increased risk of inadequate episodes and demands highly specific and sensitive detection algorithms in these patients.

Clusters of ventricular tachycardias signify impaired survival in patients with idiopathic dilated cardiomyopathy and implantable cardioverter defibrillators.

OBJECTIVES: This retrospective study was undertaken to provide data on occurrence, significance and therapy of ventricular tachyarrhythmia (VT) clusters (VTCs) in patients with dilated cardiomyopathy (DCM) and implantable cardioverter defibrillators (ICDs). BACKGROUND: Data on the clinical significance of VTCs are lacking in patients with DCM and ICDs. METHODS: Baseline characteristics of 106 consecutive patients with DCM and ICDs were prospectively collected, and chart reviews and episode data retrospectively analyzed. A VTC was defined as > or =3 sustained VTs/24 h. RESULTS: During a mean follow-up of 33+/-23 months, 73 patients (68.9%) had recurrent VT or ventricular fibrillation (VF), 43 patients (40.6%) suffered only single VTs and 30 patients (28.3%) experienced 52 clusters of VTs. Actuarial survival free of VT or VF was 44.6%, 33.0% and 26.5%, and survival free of VTC was 77.3%, 72.2% and 67.1% after one, two and three years, respectively. Independent predictors of VT clusters were heart failure before ICD implantation (p = 0.033), presenting monomorphic VT (p = 0.044), EF <0.40 (p = 0.014) and inducible mVT, especially with right bundle branch block and superior axis configuration (p<0.001). Survival free of recurrent VTCs was 50.8%, 38.1% and 19.0% after one, two and three years, respectively. Once a VTC had occurred, only 56.7%, 46.4%, 30.9% and 15.5% of patients survived and were not transplanted after one, two, three and four years, respectively. Survival was even more reduced if a VTC was associated with cardiac decompensation: 65.6% and 21.9% after one and two years, respectively. CONCLUSIONS: Despite antiarrhythmic intervention, clusters of VTs occur and recur frequently in patients with DCM. They signify impaired survival, especially if they are associated with cardiac decompensation, and may be a harbinger of progressive myocardial deterioration rather than a primarily arrhythmic problem. The benefit of ICD therapy may therefore be low in these patients.

Ultrastructural findings in endomyocardial biopsy of patients with Kearns-Sayre syndrome.

Kearns-Sayre syndrome is clinically defined by progressive external ophthalmoplegia, atypical retinitis pigmentosa and the potential occurrence of complete atrioventricular (AV) block. Right septal endomyocardial biopsy specimens from nine patients (four men and five women with a mean [+/- SD] [corrected] age of 36.3 +/- 14.4 years) with chronic progressive external ophthalmoplegia and mitochondrial skeletal myopathy were studied. Three patients had atypical retinal pigmentation. An atrioventricular or intraventricular conduction defect was observed in five patients. A pacemaker was prophylactically implanted in one patient because of abnormal conduction distal to the His bundle. Ultrastructural investigations revealed mitochondriosis in many heart muscle cells and an increased variability of mitochondrial form and size in all patients. In seven patients, 0.4 to 2.1% of all examined myocytes contained exclusively abnormal mitochondria. Three main types were observed: huge, mainly round mitochondria with concentric cristae; large, round or oval mitochondria with transverse or curved cristae; and small, vacuolated mitochondria. The volume density of myofibrils was reduced (41.9 +/- 11.1 compared with the normal value of 56.5 +/- 2.5 volume density [in percent], p less than 0.01) in these myocytes. Increasing numbers of vacuolated mitochondria correlated significantly with a reduction of myofibrils (r = -0.64, p less than 0.01). The data suggest that the ventricular myocardium of most patients with complete and even incomplete Kearns-Sayre syndrome is affected by disseminated mitochondrial cytopathy.

Efficacy and safety of d,l-sotalol in patients with ventricular tachycardia and in survivors of cardiac arrest.

OBJECTIVE: The aim of this study was to assess the antiarrhythmic efficacy and safety of d,l-sotalol in patients with ventricular tachycardia (VT) or ventricular fibrillation (VF) and in survivors of cardiac arrest and to identify the factors that are associated with arrhythmia suppression and therefore might be helpful in predicting drug efficacy. BACKGROUND: Despite increasing use of the class III antiarrhythmic agent d,l-sotalol, data on its short- and long-term efficacy in a large patient cohort are lacking. Information on its long-term tolerability and safety is limited. METHODS: A total of 396 patients with inducible sustained VT or VF (VT/VF) underwent programmed stimulation before and after receiving oral d,l-sotalol (240 to 640 mg/day). Patients in whom VT/VF was rendered either noninducible or more difficult to induce (more extrastimuli or faster drive cycle length needed for VT/VF induction) were discharged on a regimen of oral d,l-sotalol. RESULTS: d,l-Sotalol suppressed VT/VF in 151 patients (38.1%) and rendered the arrhythmia more difficult to induce in 76 patients (19.2%). The extent of drug-induced prolongation of right ventricular refractoriness and a shorter VT cycle length at baseline were independent predictors of immediate drug efficacy. Torsade de pointes developed in seven patients (1.8%). Two hundred ten patients (53%) continued to receive d,l-sotalol and were followed up for 34 +/- 18 months (mean +/- SD). The actuarial rates for the absence of arrhythmic recurrence (either VT/VF or sudden death) at 1 and 3 years were 89% and 77%, respectively. Actuarial rates for overall survival at 1 and 3 years were 94% and 86%, respectively. VT/VF suppression by d,l-sotalol was an independent discriminant variable that separated patients with and without arrhythmia recurrence. However, noninducibility of VT/VF did not predict freedom from sudden death. CONCLUSION: Oral d,l-sotalol is effective and safe in patients with VT/VF. However, sudden cardiac death develops in a significant proportion of patients, and programmed stimulation seems to be of limited value for its prediction.

Prediction of the site and severity of obstruction in hypertrophic cardiomyopathy by color flow mapping and continuous wave Doppler echocardiography.

OBJECTIVE: We investigated whether the site and severity of an obstruction in hypertrophic cardiomyopathy can be accurately predicted by the combined use of color-coded and continuous wave Doppler echocardiography. BACKGROUND: Predicting the site of obstruction by end-systolic cavity shape is not reliable. Therefore, hemodynamic localization of the obstruction is required before surgery is performed. Such localization should be possible with color flow imaging, which provides two-dimensional velocity mapping reflecting the distribution of pressures within the left ventricle. Discrepancies in assessment of the pressure gradient by Doppler echocardiography and cardiac catheterization (which are usually not performed simultaneously) may be due to spontaneous variation of the dynamic obstruction in addition to technical factors related to both methods. METHODS: Twenty consecutive patients with hypertrophic cardiomyopathy were examined 1 day before transseptal left heart catheterization. The obstruction site was defined by color flow mapping. The pressure gradient was determined by continuous wave Doppler echocardiography. Measurements were also performed simultaneously in 10 patients during cardiac catheterization. RESULTS: Midventricular obstruction was correctly identified in 4 patients and subvalvular obstruction in 15 patients. One patient had no obstruction at rest. Invasively and noninvasively determined pressure gradients correlated well (r = 0.89, SEE = 16.3 mm Hg). Multiple single-beat analysis in 10 patients, also simultaneously examined with Doppler echocardiography and catheterization, yielded an excellent correlation (r = 0.97, SEE = 13.1 mm Hg). Comparing the simultaneous (r = 0.96, SEE = 12.5 mm Hg) and nonsimultaneous (r = 0.81, SEE = 23.8 mm Hg) recordings in these patients, we found that the spontaneous variation of the dynamic obstruction mainly accounted for discrepancies (p less than 0.05). CONCLUSION: The combined use of color-coded and continuous wave Doppler echocardiography provides the relevant hemodynamic information required for decision-making in patients with hypertrophic cardiomyopathy who are considered for transaortic myectomy.

Diagnostic value of electrocardiographic variables to predict the presence of ventricular late potentials.

To test the hypothesis that the presence of ventricular late potentials in the highly amplified, averaged and filtered surface electrocardiogram (ECG) can be predicted from the conventional surface ECG, 211 patients with and without previously documented sustained ventricular tachycardia outside the acute phase of myocardial infarction were studied. The presence of left ventricular akinesia or aneurysm was significantly correlated with the ECG score (based on Q wave duration, R wave duration and amplitude ratio). The mean ECG score in patients without ventricular tachycardia was 3.4 +/- 3.5 points compared with 5.5 +/- 3.9 points (p less than 0.001) in patients with ventricular tachycardia. The presence of late potentials was positively correlated with the ECG score in the whole cohort of patients. This was also the case in the subgroup of patients without a history of sustained ventricular tachycardia. In contrast, in patients with ventricular tachycardia, the presence of late potentials was independent of their ECG score. Using linear discriminant function analyses to predict the presence of late potentials, a history of ventricular tachycardia alone and the ECG score alone had a high predictive power (high standardized coefficients). If combinations of variables were analyzed including estimates of left ventricular function (presence of aneurysm or akinesia; ejection fraction), the ECG score and a history of ventricular tachycardia still ranked highest. The influence of ejection fraction if used in combination with other variables for the prediction of late potentials was relatively small (standardized coefficient of 0.4). In conclusion, the surface ECG can be used in patients previously free of sustained ventricular tachycardia to predict the presence of ventricular late potentials.(ABSTRACT TRUNCATED AT 250 WORDS)