A Stunning Left Atrial Appendage Thrombus.

BACKGROUND: Left atrial appendage thrombus formation is a known major complication of atrial fibrillation and atrial flutter which increases the risk of embolism and stroke. This risk of thrombosis is greatly increased with a lack of anticoagulation. After conversion to a normal sinus rhythm in these arrhythmias, the risk of thrombus formation in the left atrium persists through a phenomenon termed atrial myocardial stunning. CASE: We present the case of a patient who previously underwent successful pulmonary vein isolation and was found to be in typical isthmus-dependent atrial flutter with a questionable recurrence of atrial fibrillation. The decision was made to return for atrial flutter ablation and for evaluation of prior pulmonary vein isolation. Initially, a transesophageal echocardiogram showed a normal ejection fraction, biatrial enlargement and no left atrial appendage thrombus. Ablation of the cavotricuspid isthmus was successfully accomplished with documented bidirectional block. A transesophageal echocardiogram probe was still in place prior to planned transseptal puncture for the evaluation of pulmonary veins. A large thrombus was now observed filling the left atrial appendage. Conclusion and Objective: Atrial stunning is a transient atrial contractile dysfunction that occurs whether sinus rhythm is restored spontaneously, electrically, pharmacologically or by ablation. We know after conversion that there is higher propensity to increased spontaneous echogenic contrast and decreased velocities; however, we do not have documented knowledge of exactly how soon after the conversion to a sinus rhythm a thrombus may be seen. We demonstrate a case of acute left atrial appendage thrombus formation immediately following the successful ablation of isthmus-dependent atrial flutter. Our report validates the belief that strategies of not interrupting anticoagulation prior to the conversion of these arrhythmias should be implemented.

Use of methylphenidate in the treatment of patients suffering from refractory postural tachycardia syndrome.

Methylphenidate has been shown to be an effective therapy in patients with refractory neurocardiogenic syncope. However, the role of methylphenidate in patients suffering from postural orthostatic tachycardia (POTS) has not been reported. The study was approved by the institutional review board. A retrospective nonrandomized analysis was preformed on 24 patients evaluated at our autonomic center for POTS from 2003 to 2010. The diagnosis of POTS was based on patient history, physical examination, and response to head up tilt table testing. The mean follow-up period was 9 ± 3 months. The patients were included in the current study if they had a diagnosis of POTS with severe symptoms of orthostatic intolerance and were refractory to the commonly used medications. All of these patients were started on methylphenidate and the response to therapy was considered successful if it provided symptomatic relief. Twenty-four patients (age 28 ± 12, 20 women) met inclusion criterion for this study. The response to treatment was assed subjectively in each patient and was collected in a retrospective fashion from patient charts and physician communications. Four patients reported side effects in the form of nausea and 2 ultimately had to discontinue the treatment. Another 4 patients had a follow-up of less than 6 months. Thus, only 18 patients who received methylphenidate completed the follow-up of 6 months. Out of these 18 patients, 14 (77%) patients reported marked improvement in their symptoms. Nine out of 12 patients who had recurrent episodes of syncope reported no syncope at 6 months of follow-up. Fourteen (77%) patients reported marked improvement in their symptoms of fatigue and presyncope. Four patients continue to have symptoms of orthostatic intolerance and 3 continued to have recurrent episodes of syncope. Methylphenidate may be beneficial in patients with otherwise refractory postural tachycardia syndrome.

Use of octreotide in the treatment of refractory orthostatic intolerance.

There have been reports on the use of octreotide in patients with orthostatic hypotension, postural tachycardia syndrome, and orthostatic syncope. However, there are little if any data on the use of octreotide in patients who have failed multiple other medications. This study was a retrospective chart analysis and was approved by our Institutional Review Board. A total of 12 patients were identified for inclusion in this study. The diagnosis of orthostatic intolerance was based on patient history, physical examination, and response to Head Up Tilt Table testing. These patients had failed multiple medications and were ultimately treated with octreotide. In a retrospective chart review, we collected data, including demographic information, presenting symptoms, laboratory data, tilt-table response, standing heart rate, standing blood pressure before and after treatment (wherever available), and treatment outcomes. Twelve patients aged 33 ± 18 years, eight (66.7%) females, were found to have symptoms of refractory orthostatic intolerance and failed multiple regimens of medication and were ultimately treated with octreotide administration. Five patients (41.7%) had demonstrated a postural tachycardia syndrome pattern, five (41.7%) a neurocardiogenic, and two (16.6%) a dysautonomic response on a Head Up Tilt Table. Symptoms of syncope and orthostatic palpitations improved in six (50%) of the patients. Standing heart rate was significantly reduced after octreotide administration (80 ± 8 versus 108 ± 13; P < 0.05). The standing systolic blood pressure was increased after octreotide administration (107 ± 26 versus 116 ± 22). Three patients (25%) reported complete elimination of syncope, whereas another three had reduction in the frequency of their syncope. However, symptoms of fatigue improved only in two (29%) of the seven patients. Octreotide may improve symptoms in some patients with refractory orthostatic intolerance.

Erythropoietin in the treatment of postural orthostatic tachycardia syndrome.

Erythropoietin has been reported to improve symptoms of orthostatic intolerance in patients suffering from orthostatic hypotension. Previous reports on the use of erythropoietin in patients with postural orthostatic tachycardia (POTS) have included only a very small number of patients. In the current study, we report on the use of erythropoietin in patients with refractory POTS. The study was approved by the institutional review board. A retrospective nonrandomized analysis was performed on 39 patients evaluated at our autonomic center for POTS from 2003 to 2010. The diagnosis of POTS was based on patient history, physical examination, and response to head-up tilt-table testing. The mean follow-up period was 6 months. The patients were included in the current study if they had a diagnosis of POTS with severe symptoms of orthostatic intolerance and were refractory to the commonly used medications. All these patients were started on erythropoietin, and the response to therapy was considered successful if it provided symptomatic relief. We screened 200 patients with POTS and found 39 patients (age 33 ± 12, 37 females) to be eligible for inclusion in the current study. The response to the treatment was assessed subjectively in each patient and was obtained in a retrospective fashion from patient charts and physician communications. Eight (21%) patients demonstrated no improvement in symptoms after administration of erythropoietin. Three (8%) patients showed an improvement in symptoms of orthostatic intolerance of <3 months. Twenty-seven (71%) patients demonstrated sustained improvement in their symptoms of orthostatic intolerance at the mean follow-up of 6 months. Erythropoietin significantly improved sitting diastolic blood pressure but had no effect on other hemodynamic parameters. In a select group of POTS patients who are refractory to commonly used medications, erythropoietin may help improve symptoms of orthostatic intolerance.

Transmyocardial voyage of a temporary epicardial lead: an unusual long-term complication.

Although the majority of temporary epicardial leads used during cardiac surgery are removed in the perioperative period, occasionally, the wires are cut and allowed to retract into the subcutaneous tissue. Complications arising from such retained epicardial wires are rare. We present a case of transmyocardial migration of a retained epicardial wire through the right ventricular myocardium into the pulmonary artery in a patient who had undergone coronary artery bypass graft surgery 13 years ago. We describe the presentation and successful management of this case.

Syncope as an initial manifestation of atypical lipomatous hypertrophy of the interatrial septum.

Lipomatous hypertrophy of the interatrial septum (LHIS) is an incidental echocardiographic abnormality characterized by dumb-bell configuration of the interatrial septum from excess fat deposition. We report a case of syncope in a patient with LHIS obstructing the superior vena cava at the right atrial junction.

Preliminary observations suggesting that treatment with modafinil improves fatigue in patients with orthostatic intolerance.

Many patients who suffer from orthostatic intolerance (OI) may also have severe fatigue and extreme exercise intolerance. In some of these patients, fatigue may be so severe that they are unable to maintain employment. In some, even the activities of the daily living may be compromised. We report on the use of modafinil in a subgroup of patients who failed therapy with commonly used medication for fatigue in patients with OI. The study was approved by the institutional review board. A retrospective nonrandomized analysis was preformed on 60 patients evaluated at our autonomic center for OI from 2003 to 2010. The diagnosis of OI was based on patient history, physical examination, and reponse to head up tilt table testing. All these patients had fatigue as their predominant symptom. Multiple trials of stimulants including methylphenidate, amphetamine, or dextroamphetamine failed to provide symptomatic relief of fatigue in these patients. Each patient received modafinil (100-200 mg daily). The mean follow-up period was 9 ± 3 months. A treatment was considered successful if it provided symptomatic relief from fatigue for the patient. Sixty patients, age 29 ± 15, 52 women were included in the analysis. Migraine (57%) and joint hypermobility syndrome (33%) were common comorbidities. Out of 60 patients, 40 patients reported initial improvement with initiation of modafinil therapy. Twenty patients reported no change in their symptoms of fatigue. Of the 40 patients who showed initial improvement in their symptoms 4 had eventual recurrence of fatigue after 3 months of modafinil therapy. Thirty-six patients continued to demonstrate symptom relief from fatigue for more than 6 months. In a selective group of patients of OI, modafinil may improve fatigue.

Neurocardiogenic syncope coexisting with postural orthostatic tachycardia syndrome in patients suffering from orthostatic intolerance: a combined form of autonomic dysfunction.

INTRODUCTION: There is anecdotal evidence that one or more forms of orthostatic intolerance (OI) subgroups may coexist in the same patients. However, there is a paucity of published data on the clinical features and management of patients who suffer from coexisting features of postural tachycardia syndrome (POTS) and neurocardiogenic syncope (NCS). We herein present our experience of 18 patients who we found displayed evidence of coexisting NCS and POTS. METHODS: We reviewed charts of 300 POTS patients seen at the University of Toledo Syncope and Autonomic Disorders Center from 2003 to 2010 and found 18 patients eligible for inclusion in this study. Patients were included in this study if they reported clinical symptoms consistent with both POTS and NCS and then demonstrated a typical POTS pattern (a rise in heart rate without change in blood pressure [BP]) on head up tilt table (HUTT) within the first 10 minutes of upright posture followed by a neurocardiogenic pattern (a sudden fall in heart rate and/or fall in blood pressure) reproducing symptoms that were similar to the patients spontaneous episodes. RESULTS: We found 18 patients, mean age (30 ± 12), with 15 (84%) women and three (16%) men, who met the inclusion criterion for this study. Each of these 18 patients demonstrated a typical POTS pattern within the first 10 minutes on initial physical exam and on a HUTT. Continued tilting beyond 10 minutes resulted in a sudden decline in heart rate (which in some patients manifested as an asystole that lasted anywhere between 10 and 32 seconds [mean of 18 seconds]) and/or a fall in BP in each of these patients demonstrating a pattern consistent with neurocardiogenic subtype of OI. The mean time to the NCS pattern of a fall in BP and heart was 15 minutes with a range of 13-20 minutes. This group of patients was highly symptomatic and reported frequent clinical symptoms that were suggestive of OI. Recurrent presyncope, syncope, orthostatic palpitations, exercise intolerance, and fatigue were the principal symptoms reported. CONCLUSION: NCS may coexist with POTS in a subgroup of patients suffering from OI.

Pyridostigmine in the treatment of postural orthostatic tachycardia: a single-center experience.

BACKGROUND: The long-term efficacy of pyridostigmine, a reversible acetyl cholinesterase inhibitor, in the treatment of postural orthostatic tachycardia syndrome (POTS) patients remains unclear. We report our retrospective, single-center, long-term experience regarding the efficacy and adverse effect profile of pyridostigmine in the treatment of POTS patients. METHODS: This retrospective study included an extensive review of electronic charts and data collection in regards to patient demographics, orthostatic parameters, side-effect profile, subjective response to therapy, as well as laboratory studies recorded at each follow-up visit to our institution's Syncope and Autonomic Disorders Center. The response to pyridostigmine therapy was considered successful if patient had both symptom relief in addition to an objective response in orthostatic hemodynamic parameters (heart rate [HR] and blood pressure). Three hundred patients with POTS were screened for evaluation in this study. Of these 300, 203 patients with POTS who received pyridostigmine therapy were reviewed. Of these 203 patients, 168 were able to tolerate the medication after careful dose titration. The mean follow-up duration in this group of patients was 12 ± 3 (9-15) months. Pyridostigmine improved symptoms of orthostatic intolerance in 88 of 203 (43%) of total patients or 88 of 172 (51%) who were able to tolerate the drug. The symptoms that improved the most included fatigue (55%), palpitations (60%), presyncope (60%), and syncope (48%). Symptom reduction correlated with a statistically significant improvement in upright HR and diastolic blood pressure after treatment with pyridostigmine as compared to their baseline hemodynamic parameters (standing HR 94 ± 19 vs 82 ± 16, P < 0.003, standing diastolic blood pressure 71 ± 11 vs 74 ± 12, P < 0.02). Gastrointestinal problems were the most common adverse effects (n = 39, 19%) reported. The overall efficacy of pyridostigmine in our study was seen in 42% of total patients or 52% of patients who could tolerate taking the drug. CONCLUSION: The subgroup of POTS patients who can tolerate oral pyridostigmine may demonstrate improvement in their standing HR, standing diastolic blood pressure, and clinical symptoms of orthostatic intolerance.

Postural orthostatic tachycardia syndrome: a rare complication following electrical injury.

We report on two previously healthy patients who developed severe form of postural orthostatic tachycardia syndrome (POTS) following an electric injury. Both the patients developed symptoms of orthostatic intolerance in the form of dizziness, fatigue, lightheadedness, and palpitations, weeks to months after electrical injury. Orthostatic intolerance produced considerable functional impairment in these patients. Early recognition of POTS when it occurs after an electrical injury allows for prompt evaluation and management to occur.

An anatomic-based approach for the placement of implantable loop recorders.

INTRODUCTION: Placement of the Reveal implantable loop recorder (ILR; Medtronic Inc., Minneapolis, MN, USA) has previously involved preoperative cutaneous mapping to determine the optimal location. We describe an anatomic-based approach to ILR placement that does not require cutaneous mapping. METHOD: A total of 63 patients (40 women, 23 men, mean age 38 ± 15 years) were included in the study. Each underwent implantation of a Reveal ILR in the left upper chest area midway between the supraclavicular notch and the left breast area. Thirty-two patients received a Medtronic Reveal DX ILR and 31 received Reveal XT device. RESULTS: In all 62 patients, adequate electrocardiographic tracings were obtained at implant without the need for preoperative cutaneous mapping, and all were followed for a period of 10 ± 4 months afterwards. The mean P wave amplitude was 0.12 ± 0.20 mV at implant and at follow-up (6-14 months postimplant); the amplitude was 0.11 ± 0.19 mV. The peak-to-peak QRS amplitude was 0.48 ± 0.15 mV at implant and 0.44 ± 0.16 mV at a follow-up of 6-14 months. The P waves were not detected in two patients at follow-up. In one patient, decreased amplitude of QRS complex resulted in the autoactivation of the device and in one other patient noise was inappropriately oversensed and recorded. CONCLUSION: A simple anatomic approach can be used for reveal ILR placement.

Underutilization of implantable cardioverter defibrillators post coronary artery bypass grafting in patients with systolic dysfunction.

BACKGROUND: Evaluation of the need for prophylactic internal cardiac defibrillators among patients with ischemic cardiomyopathies should be deferred until at least 3 months after revascularization procedures to allow adequate time for recovery of ventricular function. METHODS: Among patients with left ventricular systolic dysfunction (LVSD) who undergo coronary artery bypass grafting (CABG), the proportion of patients who are risk stratified postoperatively with reassessment of left ventricular ejection fraction (LVEF) is unknown. RESULTS: One hundred and six patients with LVSD (LVEF < 40%) who underwent CABG during 2004-2006 and survived 3 months post CABG were evaluated. Follow-up was assessed by chart review and telephone contact. LVEF was not reassessed in 24% (25/106) of the population, none of whom underwent internal cardioverter defibrillator (ICD) implantation. Of those with LVEF reassessed, persistent LVSD was present in 20/81 (25%), 12 of whom were referred for prophylactic ICD placement. CONCLUSION: One-fourth of patients with LVSD who undergo CABG do not have LVEF reassessed postoperatively which may lead to underutilization of ICDs.

A case of mistaken identity: asystole causing convulsions identified using implantable loop recorder.

We present herein an interesting tracing of a patient who suffered from recurrent episodes of transient loss of consciousness (TLOC) associated with convulsive activity thought to be due to epilepsy or conversion disorder.

Autonomic dysfunction presenting as postural orthostatic tachycardia syndrome in patients with multiple sclerosis.

BACKGROUND: Autonomic dysfunction is common in patients suffering from multiple sclerosis (MS) and orthostatic dizziness occurs in almost 50% of these patients. However, there have been no reports on postural orthostatic tachycardia syndrome (POTS) in patients suffering from MS. METHODS: The patients were included for analysis in this study if they had POTS with either a prior history of MS or having developed MS while being followed for POTS. Postural orthostatic tachycardia (POTS) is defined as symptoms of orthostatic intolerance(>6 months) accompanied by a heart rate increase of at least 30 beats/min (or a rate that exceeds 120 beats/min) that occurs in the first 10 minutes of upright posture or head up tilt test (HUTT) occurring in the absence of other chronic debilitating disorders. We identified nine patients with POTS who were suffering from MS as well. Each of these patients had been referred from various other centers for second opinions. RESULTS: The mean age at the time of diagnosis of POTS was 49+/-9 years and eight of the 9 patients were women. Five patients (55%) had hyperlipidemia, 3 (33%) migraine and 2 (22%) patients had coronary artery disease and diabetes each. Fatigue and palpitations (on assuming upright posture) were the most common finding in our patients (9/9). All patients also had orthostatic dizziness. Syncope was seen in 5/9(55%) of patients. Four patients (44%), who did not have clear syncope, were having episodes of near syncope. The presence of POTS in our study population resulted in substantial limitation of daily activities. Following recognition and treatment of POTS, 6/9(66%), patients were able to resume daily activities of living. Their symptoms (especially fatigue and orthostatic intolerance) improved. The frequency and severity of syncope also improved. Three (33%) patients failed to show a good response to treatment. CONCLUSION: Patients suffering from MS may manifest autonomic dysfunction by developing POTS. Early recognition and proper management may help improve the symptoms of POTS.

Comparative clinical profile of postural orthostatic tachycardia patients with and without joint hypermobility syndrome.

BACKGROUND: Autonomic dysfunction is common in patients with the joint hypermobility syndrome (JHS). However, there is a paucity of reported data on clinical features of Postural orthostatic tachycardia syndrome (POTS) in patients suffering from JHS. METHODS: This retrospective study was approved by our local Institutional Review Board (IRB). Over a period of 10 years, 26 patients of POTS were identified for inclusion in this study. All these patients had features of Joint Hypermobility Syndrome (by Brighton criterion). A comparison group of 39 patients with other forms of POTS were also followed in the autonomic clinic during the same time. We present a descriptive report on the comparative clinical profile of the clinical features of Postural Orthostatic Tachycardia patients with and without Joint Hypermobility syndrome. The data is presented as a mean+/-SD and percentages wherever applicable. RESULTS: Out of 65 patients, 26 patients (all females, 20 Caucasians) had POTS and JHS. The mean age at presentation of POTS was 24+/-13 (range 10-53 years) vs 41+/-12 (range 19-65 years), P=0.0001, Migraine was a common co morbidity 73 vs 29% p=0,001. In two patients POTS was precipitated by pregnancy, and in three by surgery, urinary tract infection and a viral syndrome respectively. The common clinical features were fatigue (58%), orthostatic palpitations (54%), presyncope (58%), and syncope (62%). CONCLUSIONS: Patients with POTS and JHS appear to become symptomatic at an earlier age compared to POTS patients without JHS. In addition patients with JHS had a greater incidence of migraine and syncope than their non JHS counterparts.

A tale of two atria.

We present an interesting intracardiac electrogram of a dissimilar atrial rhythm in a patient of bi-atrial orthotopic cardiac transplant.

Passing sheaths and electrode catheters through inferior vena cava filters: safer than we think?

Inferior vena cava (IVC) filters are being inserted with increasing frequency for the prevention of pulmonary embolus. Previous case reports have documented the passage of up to three electrode catheters or an individual long sheath through an IVC filter. The current report expands on prior series with regard to the number of devices used. We describe our experience in 10 patients in whom up to five electrode catheters and/or sheaths were placed through an IVC filter using a transfemoral approach under fluoroscopic guidance without routine venography. Devices were successfully introduced and withdrawn in each case without filter dislodgment. Our series illustrates the feasibility and safety of passing multiple electrode catheters and long sheaths through an IVC filter. Evidence is accumulating to suggest that an IVC filter should not be considered an absolute contraindication to performing diagnostic or therapeutic procedures.

Psychogenic syncope? A cautionary note.

INTRODUCTION: In some patients with recurrent syncope, the etiology may remain unclear despite extensive evaluation. These patients may sometimes be labeled as having a "psychogenic" cause for their syncope. METHODS: We report on three patients with recurrent unexplained syncope (despite extensive evaluation) who were labeled as having a psychogenic cause for their events. In each patient following placement of an implantable loop recorder, their syncopal events were found to be due to periods of prolonged asystole and/or complete heart block. One patient had prolonged asystole for 44 seconds. In each patient, episodes of syncope were eliminated following permanent pacemaker implantation. CONCLUSION: We conclude that physicians should exercise great caution before labeling any patient's syncope as psychogenic and that prolonged monitoring may be necessary to exclude a potential cardiac rhythm-related etiology.

Outcomes of pregnancy in patients with preexisting postural tachycardia syndrome.

BACKGROUND: Postural orthostatic tachycardia syndrome (POTS) occurs more commonly in women than in men and often affects women of childbearing age. Many of these women wish to have children, yet there are little reported data on the outcomes of pregnancy in patients with POTS. To date there has been one report of two patients with POTS who successfully completed pregnancy. We report the outcomes of 22 women with preexisting POTS who became pregnant. OBJECTIVE: To assess the outcome of pregnancy in patients with preexisting POTS. METHODS AND RESULTS: Twenty-two patients, age 30 +/- 7 years, with POTS became pregnant. Migraine was the common comorbidity found in 40% of patients. Medications used were beta-blockers (18%), midiodrine (31%), selective serotonin reuptake inhibitors (31%), fludrocortisone (13%), combination (40%), and none (18%). During pregnancy, symptoms of POTS remained unchanged in three (13%), improved in 12 (55%), and worsened in seven (31%) patients. One patient who had recurrent episodes of syncope without aura was found to have complete heart block and received a cardiac pacemaker. All patients completed pregnancy successfully. There were no stillbirths. One patient developed hyperemesis. Eighteen patients had vaginal delivery and four patients delivered by cesarian section. No other complications of pregnancy were encountered. Congenital abnormalities were encountered in the form of one atrial septal defect, one ventricular septal defect, and one Down's syndrome. Postpartum symptoms of POTS remained stable in 15 (69%) patients and worsened in seven (31%) patients. CONCLUSION: Based on our observation, patients with POTS can safely complete pregnancy if they desire to do so. POTS should not be considered a contraindication to pregnancy per se.

Differentiation of convulsive syncope from epilepsy with an implantable loop recorder.

INTRODUCTION: Not all convulsive episodes are due to epilepsy and a number of these have a cardiovascular cause. Failure to identify these patients delays the provision of adequate therapy while at the same time exposes the individual to the risk of injury or death. METHODS: We report on three patients who suffered from recurrent convulsive episodes, thought to be epileptic in origin, who were refractory to antiseizure therapy. Although each patient had undergone extensive evaluation, no other potential cause of his or her seizure like episodes had been uncovered. In each patient placement of an implantable loop recorder (ILR) demonstrated that their convulsive episodes were due to prolonged periods of cardiac asystole and/or complete heart block. In all patients their convulsive episodes were eliminated by permanent pacemaker implantation. CONCLUSION: In patients with refractory "seizure' like episodes of convulsive activity of unknown etiology a potential cardiac rhythm disturbance should be considered and can be easily evaluated by ILR placement.