Charcot-Marie-Tooth disease (CMT) is an inherited neurological disorder that affects the arms and legs, causing muscle weakness and atrophy along with decreased sensation.
The optic nerve is a bundle of nerve fibers that connects the retina (at the back of the eye) and the visual center of the brain.
Optic neuropathy, also called optic atrophy, is a condition that occurs when the optic nerve is damaged. Sickle cell anemia, also referred to as sickle cell disease, is a genetic condition that causes a mutated form of hemoglobin.
Much of the experience of sickle cell disease consists of moving from one health crisis to another. Everyone who has sickle cell disease needs to be under the care of a hematologist, a physician who specializes in diseases of the blood.
Sickle cell disease is caused by the presence of an abnormal gene for the production of hemoglobin, Hb S, rather than the normal gene for the production of hemoglobin, HbA. One of the most common and deadly complications of sickle cell anemia is pulmonary hypertension, resulting from blood clots in the vein that carries oxygen from the lungs back to the heart. Since sickle cell disease is a condition caused by defective red blood cells and blood transfusions supply red blood cells, it would seem logical to treat sickle cell disease with blood tranfusions. Sickle cell disease is a chronic condition affecting one out of every 500 African-American births. Find health and lifestyle advices & Get answers!Share real-life experiences with more than 250,000 community members!
Atrial fibrillation (AF) is a common heart rhythm disorder in which the electrical impulses in the upper cardiac chambers (atria) degenerate from their usual organized rhythm into a rapid chaotic pattern.
The definition and classification has been described and updated in 2011 guidelines published by the American College of Cardiology (ACC), American Heart Association (AHA), and European Society of Cardiology (ESC), with the collaboration of the Heart Rhythm Society (HRS).1 According to these guidelines, an episode of AF is defined as an event lasting greater than 30 seconds in duration. AF is the most common sustained cardiac tachyarrhythmia encountered by clinicians in the United States and world-wide. AF may be acutely associated with physiologic stressors such as surgical procedures, pulmonary embolism, chronic lung diseases, hyperthyroidism, and alcohol ingestion. Pathogenesis can be broadly divided into the categories of triggers, substrate and sustaining mechanisms. Cardiac ganglionic plexuses clustered posteriorly and superiorly to the left atrium are known to play an important role in the initiation and maintenance of AF. AF rotors represent an emerging concept as a sustaining mechanism for AF involving spiral waves detected by spectral analysis of dominant frequencies recorded by intra-cardiac mapping catheters. The clinician must realize that an irregular pulse detected by physical examination or an irregular ventricular rhythm seen on the electrocardiogram (ECG) is not always AF. The management of AF is directed at three basic goals: control of the ventricular rate, minimization of thromboembolism risk (particularly stroke), and restoration and maintenance of sinus rhythm. Warfarin should be continued after cardioversion until sinus rhythm has been maintained for at least 4 weeks to allow the atrial transport mechanism to recover. Nonpharmacologic methods of stroke prevention include surgical left atrial exclusion or percutaneous left atrial appendage occlusion.
The restoration and maintenance of sinus rhythm can be beneficial for patients with bothersome symptoms. The largest study, the Atrial Fibrillation Follow-up Investigation of Rhythm Management (AFFIRM) trial, was a large multicenter randomized study that compared these two treatment strategies for patients with AF.9 Both treatment strategies used appropriate anticoagulation strategies according to established guidelines. Rates of successful immediate cardioversion by pharmacologic means have ranged from 40% to 90%, with success more likely to come for patients with AF of shorter duration. The only IV agents approved in the United States for immediate pharmacologic cardioversion of AF are procainamide, amiodarone, and ibutilide (Table 2).
A number of oral agents may be used for long-term maintenance of sinus rhythm for patients with AF (Table 3). Sotalol is a Class III antiarrhythmic that has beta-blocking properties and is generally well tolerated. Dofetilide, another Class III agent, has good efficacy rates and carries the principle advantage of being one of the best tolerated anti-arrhythmic drugs in terms of daily side effect profile. Amiodarone, although an effective antiarrhythmic agent, generally is reserved for patients with AF for whom other antiarrhythmic drugs have been contraindicated, ineffective, or poorly tolerated. With the recognition of PVs as the source of the critical triggering beats of AF in most patients, a standard catheter ablation approach involves achieving electrical PV isolation (PVI).
In virtually all studies involving catheter ablation, efficacy rates are lower among patients with persistent AF and long-standing persistent AF. Experienced centers, such as the Cleveland Clinic, have reported 1-year AF freedom rates between 75% and 80% off anti-arrhythmic drugs for patients with paroxysmal AF following a single ablation procedure, and 85% to 90% following a second catheter ablation procedure. The original Cox maze surgical procedure for the treatment of AF has substantially evolved from its initial form.
Treating AF is centered on quality of life because it is not immediately life threatening in most instances provided that patients receive appropriate thromboembolic stroke prophylaxis.
The prevalence of AF, already at epidemic proportions, is expected to continue to increase as the population ages and more patients with heart disease live longer.
Atrial fibrillation is the most common sustained tachyarrhythmia in the United States and world-wide.
Any unstable patient presenting with atrial fibrillation should undergo immediate electrical cardioversion.
Treatment strategies involving rate control only are comparable to rhythm-control strategies in terms of mortality rates, but many studies support quality of life benefits associated with a rhythm-control approach.
Procedure-based treatment for atrial fibrillation should be considered for symptomatic patients refractory to standard therapies. The Atrial Fibrillation Follow-up Investigation of Rhythm Management (AFFIRM) Investigators.
It is not life-threatening and rarely affects the brain but can diminish a person's quality of life as nerve degeneration reduces the use of the arms and legs. Physical therapy can help strengthen the muscles of the arms and legs and slow the progression of muscle atrophy and nerve deterioration. The deformed hemoglobin causes deformed red blood cells that take on an elongated, sickle shape rather than the usual ovals. The single most common symptom of sickle cell disease is pain caused by the occlusion of veins that can occur anywhere in the body.
When a process called electrophoresis detects two copies of the Hb S gene in red blood cells, a diagnosis of sickle cell disease is made. The costs of treating the disease are enormous, and both quality and length of life are limited.
It is also helpful to make sure the hematologist has experience dealing with many cases of sickle cell disease. People who have two copies of the Hb S gene develop all of the symptoms of the disease, and generally have a very short life expectancy, on average just 27 years, even in the United States.
Even in infancy, sickle cell disease can cause dachtylitis, a condition of painful joints also known as hand-foot syndrome. Some studies have found that 40% of people who have sickle cell have this condition, which worsens with age.
A phase 1 dose-escalation study: httpsafety, tolerability, and pharmacokinetics of FBS0701, a novel oral iron chelator for the treatment of transfusional iron overload. This disruption results in an irregular and often rapid heartbeat that is classically described as "irregularly irregular" and occurs because of the unpredictable conduction of these disordered impulses across the atrioventricular (AV) node into the lower cardiac chambers (ventricles).
Paroxysmal AF refers to patients with spontaneous termination of the arrhythmia within 7 days of its onset.
Disease states commonly associated with AF include hypertension, valvular heart disease, CHF, coronary artery disease, Wolff-Parkinson-White (WPW) syndrome, pericarditis, obstructive sleep apnea and cardiomyopathy. Since the late 1990s, it has been recognized that the initiation of AF in most cases occurs because of premature atrial contractions triggered by beats that arise from the pulmonary veins (PVs), usually from muscular tissue sleeves near the junction with the left atrium.2 These triggers may also fire repetitively and contribute to the maintenance of AF, essentially becoming drivers of AF.
It can decrease cardiac output by as much as 20% attributable to the contribution of atrial systole, and increase pulmonary capillary wedge pressure resulting in CHF. AF can produce bothersome symptoms that affect quality of life, but patients with AF also have a substantial risk of thromboembolic stroke, as discussed later. It is necessary to consider and exclude other types of irregular rhythm disturbances, including atrial or ventricular ectopy, atrial tachycardia or atrial flutter (Figure 1) with variable AV conduction, multifocal atrial tachycardia (Figure 2), and wandering atrial pacemaker.
Electrocardiographic findings in AF include the absence of P waves, the presence of low amplitude, high frequency atrial fibrillary waves (f waves). Patients with WPW syndrome may be vulnerable to ventricular fibrillation and sudden death because of the development of AF, which can result in extremely rapid conduction over the accessory pathway (Figure 4).
The first two management goals are essential for most patients, but the third management goal may not be necessary in all patients. If the cardioversion was performed using the TEE-guided approach with IV heparin as the method of anticoagulation, it is advisable to continue IV heparin until a therapeutic INR is achieved with warfarin.
Such interventions have shown great promise, but remain an active area of investigation without a proven indication.
However, management of patients with asymptomatic or minimally symptomatic AF has been controversial for many years. This study has demonstrated that a rhythm-control strategy is no better than a ventricular rate control strategy with regard to quality of life, incidence of stroke, or mortality at a follow-up of approximately 5 years. The acute success rate for electrical direct current cardioversion with a biphasic shock is approximately 95%. Contemporary use of pharmacologic cardioversion in the United States now centers around non-elective scenarios such as the emergency department or ICU setting, and also stable outpatients treated with a unique type of rhythm control strategy referred to as a 'pill-in-the-pocket' approach.
Quinidine was once widely prescribed for AF, but its use has significantly decreased in recent years.
Patients may have difficulty tolerating the beta blocker side effects, such as fatigue, and there is a potential risk of excessive bradycardia. Importantly, dofetilide has also been shown to be safe for patients with cardiomyopathy, CHF, and ischemic heart disease. This is primarily because amiodarone has potential time- and dose-dependent organ toxicities that can affect the liver, thyroid, and lungs and the eyes. Certainly, there is a substantial incidence of sinus node and AV node dysfunction in the AF population requiring cardiac pacing. This type of ablation is the ultimate method of ventricular rate control and is often reserved for patients with permanent or paroxysmal AF refractory to medical or ablative therapy.
The end result of this procedure is that spontaneous electrical impulses originating from within any of the four PVs cannot propagate into the atrial body to initiate or 'trigger' AF. Research into the underlying molecular and genetic causes of AF may lead to novel methods of disease prevention. Elective cardioversions are safe and effective with attention to appropriate anticoagulation strategies, including a transesophageal echocardiogram when needed. Such procedures include catheter ablation, surgical approaches and implantable cardiac devices.


Spontaneous initiation of atrial fibrillation by ectopic beats originating in the pulmonary veins. Efficacy of cardiac autonomic denervation for atrial fibrillation: a meta-analysis [published online ahead of print March 19, 2012]. Focal impulse and rotor modulation ablation of sustaining rotors abruptly terminates persistent atrial fibrillation to sinus rhythm with elimination on follow-up: a video case study [published online ahead of print March 28, 2012]. A randomized comparison of pulmonary vein isolation with versus without concomitant renal artery denervation in patients with refractory symptomatic atrial fibrillation and resistant hypertension [published online ahead of print September 5, 2012]. Clinical outcomes after ablation and pacing therapy for atrial fibrillation: a meta-analysis. Validation of clinical classification schemes for predicting stroke: results from the National Registry of Atrial Fibrillation. Refining clinical risk stratification for predicting stroke and thromboembolism in atrial fibrillation using a novel risk factor-based approach: the euro heart survey on atrial fibrillation [published online ahead of print September 17, 2009]. Clopidogrel plus aspirin versus oral anticoagulation for atrial fibrillation in the Atrial Fibrillation Clopidogrel Trial with Irbesartan for prevention of Vascular Events (ACTIVE W): a randomised controlled trial. Effect of clopidogrel added to aspirin in patients with atrial fibrillation [published online ahead of print March 31, 2009].
Role of transesophageal echocardiography-guided cardioversion of patients with atrial fibrillation. Radiofrequency ablation vs antiarrhythmic drugs as first-line treatment of symptomatic atrial fibrillation: a randomized trial. Preliminary report: effect of encainide and flecainide on mortality in a randomized trial of arrhythmia suppression after myocardial infarction. Cryoballoon ablation of pulmonary veins for paroxysmal atrial fibrillation: first results of the North American Arctic Front (STOP AF) pivotal trial [published online ahead of print March 21, 2013]. Characteristics and prognosis of lone atrial fibrillation: 30-year follow-up in the Framingham Study. Occupational therapy helps the person adapt to reduced limb function by teaching him new ways to perform everyday tasks like buttoning clothes and turning doorknobs. Sickle cells don't carry oxygen as efficiently, so someone who has the disease is effectively anemia. It may present as dachtylitis, pain in the fingers and toes, or as pain in the joints, or as excruciating pain in the abdomen when the liver is clotted. It is important to get the definitive test, because two other diseases cause some of the same symptoms as sickle cell disease, HbSC and Hb-beta thalassemia. There is some hope for the children of sickle cell carriers, however, in the form of stem cell therapies. Many people born with two copies of the Hb S gene develop kidney failure which cannot be permanently corrected with a transplant by the age of 24. As a child with sickle cell disease grows older, the pain involves the long bones of the legs and arms, and later the lower back. Another commmon complication of sickle cell is avascular necrosis, which causes death of the tissues surrounding the hip joint as circulation fails. Sickle cell patients receive so many transfusions that they can develop immune reactions to some of the lesser-known proteins in blood, such as the C, E, JKB (Kidd), Kell, and Fya (Duffy) antigens, which are not a concern in people who do not receive transfusions on a regular basis.
Its prevalence increases with age, and it has been diagnosed at some point in up to 10% of the population older than 80 years.
Lone AF refers to patients without overt structural heart disease, or identifiable risk factors. Deleterious hemodynamic effects also include non-physiologic tachycardia, increased valvular regurgitation, and irregularity in ventricular systole. It should be emphasized that for any unstable patient presenting with AFa€”for example, a patient with chest pain, pulmonary edema, or hypotensiona€”the recommended therapy is rapid electrical cardioversion.
If these medications are ineffective or their effectiveness is prohibited by the development of excessive bradycardia, then other measures may need to be considered.
The decision to initiate and continue anticoagulation for AF shorter than a duration of 48 hours should be based on the presence of other risk factors for thromboembolism. Selecting appropriate patients for a rhythm-controlling strategy are well articulated in the 2011 updated clinical practice guidelines. Direct-current cardioversion should be administered with the patient under deep sedation, with cardiac and hemodynamic monitoring, and in the presence of personnel skilled in airway management. Elective pharmacologic cardioversion is uncommon in the United States given the superiority of a planned electrical cardioversion under sedation with appropriate airway management personnel on hand.
The pill-in-the-pocket treatment approach may be useful for select outpatients in order to terminate recent-onset episodes of AF. In fact, all of the Class IA antiarrhythmic drugsa€”quinidine, procainamide, and disopyramidea€”have become less popular for the long-term treatment of AF. As with other Class III antiarrhythmic agents, sotalol causes QT prolongation and may result in ventricular proarrhythmia, such as torsades de pointes.
Pacemakers have several purposes, including bradycardia pacing support, ventricular response regularization, and AF suppression or termination. The potential benefits of this type of approach extend beyond simply controlling ventricular response, because there is evidence that regularization of the ventricular rhythm also confers hemodynamic or symptomatic benefits, particularly in the heart failure population in conjunction with the use of a bi-ventricular pacemaker. PVI is thus a stand-alone treatment approach, but has also been incorporated into larger ablative efforts aimed at non-PV triggers and also substrate modification. Cryoballoon catheter ablation represents an alternative to traditional RF energy ablation associated with similar outcomes for paroxysmal AF patients but with shorter procedure times and less radiation exposure.
Outcomes for patients with persistent and long-standing persistent AF are lower than patients with paroxysmal disease with reported 1-year efficacy rates between 50% and 70% following a single procedure and 70% and 80% following a second procedure.
These are carefully placed to compartmentalize the atrial tissue to channel atrial activity and prevent the re-entry required for the maintenance of AF.
Follow-up data from the Framingham Heart Study25,26 and the Antiarrhythmics Versus Implantable Defibrillators (AVID) trial7 have shown that AF is an independent predictor of increased mortality. The rapid growth of catheter-based and surgical ablation procedural capability is promising and has already relieved many patients of the burden of AF and the side effects and toxicities of antiarrhythmic medications.
Both types result in the disruption of signals between the brain and the limbs, causing one to lose the ability to move them as desired. Many persons who have this condition experience autosplenectomy, in which the diseased spleen "removes" itself, and the pain from the spleen can be referred to other parts of the body so that they hurt, too. HbSC, which occurs in about 1 in 1100 African-Americans, results from having just one of the genes for sickle cell anemia along with a mutant gene for another red blood cell trait.
Some researchers are seeking methods to correct sickle cell disease shortly after or even before birth.
It is necessary to be sure to stay hydrated and to get enough rest, often bed rest, to avoid the "clotting crises" that can come with dehyration or overexertion. People who have only one copy of the defective gene develop some of the symptoms of the disease, such as blood in the urine, but do not have to deal with the worst symptoms of the disease. Where malaria is no longer a problem, however, sickle cell disease is an unmitigated health problem. About 30% of people who have sickle cell disease develop avascular necrosis by the age of 30. Multiple transfusions can also cause iron to build up in the bloodstream; this iron may have to be removed with a class of drugs known as chelating agents. Thus, experts agree that the prevalence of AF will continue to grow, in particular, in the United States and other western countries with aging population demographics. Considerable research had been devoted to the disease mechanisms and pathogenesis, and this remains an area of active investigation. Focal triggers, especially the PVs, are felt to be very important early in the disease process and, in particular, among patients with paroxysmal AF.
For example, AF can manifest with a regular ventricular response in the presence of AV block or with a ventricular paced rhythm. Treatment with AV node-blocking medications such as verapamil or digoxin can facilitate rapid conduction over the accessory pathway and result in ventricular fibrillation. One option suitable for some patients is catheter ablation of the AV node and pacemaker implantation (so-called 'ablate and pace').
In recent years, novel oral anticoagulant drugs have emerged including direct thrombin inhibitors such as dabigatran (Pradaxa), or Factor Xa inhibitors such as rivaroxaban (Xarelto) or apixaban (Eliquis). It is worthwhile to note that the previously mentioned novel oral anticoagulant drugs (dabigatran, rivaroxaban and apixaban) have not been FDA approved for direct current cardioversion, and for other AF-related procedures.
In addition to improving symptoms, the potential benefits of restoring and maintaining sinus rhythm include avoidance of the development of atrial cardiomyopathy from ongoing AF, improvement in heart failure and improved overall quality of life in some studies. For the most part, catheter ablation is considered a second-line treatment for patients who have failed medical anti-arrhythmic drug therapy, or those with drug intolerance. The administration of an antiarrhythmic drug may promote more successful direct current cardioversion and subsequent maintenance of sinus rhythm. This approach has the potential to reduce emergency department visits and hospitalizations, but must be carefully initiated and supervised.
Other antiarrhythmic drugs, such as the Class IC agents flecainide and propafenone, have more favorable side effect profiles. Like sotalol, this drug causes QT prolongation that may result in ventricular proarrhythmia and rarely death if excessive and is restricted to patients without advanced renal disease.
These tests include an ophthalmologic examination, pulmonary spirometry and diffusion capacity tests, and blood tests to assess liver and thyroid function.
Clinical practice guidelines detail the recommended uses of implantable pacemakers and antitachycardia devices.
This approach has been shown to be effective and leads to improved quality of life for patients. Implantable atrial defibrillators have been developed, either as a stand-alone device or in combination with a ventricular defibrillator.
To a certain extent, there has been a confluence with some of the lesions sets delivered during catheter ablation techniques. It is not clear whether this higher risk is a reflection of the proarrhythmic complications of antiarrhythmic therapy, a failure to comply with prescribed medical therapy, or the presence of other factors such as stroke, worsening CHF, or unknown factors that were not recognized.
However, these approaches are invasive and inherently destructive, with a small but important risk of serious complications.
This signal disruption also interferes with pain sensation so one may have an injury and not feel it, resulting in further injury or infection. Clotting frequently interferes with the germ-clearing function of the spleen, so people who have sickle cell disease are at greater risk for infections.
Sickle cell disease often causes severe bone pain, as they too are affected by occluded blood vessels. Its symptoms are much milder than sickle cell anemia's, and a more normal life is possible. Other researchers are looking at bone marrow transplants for infants using marrow donated by the father or mother of the child.


Many people who have sickle cell disease receive frequent transfusions, but it is essential that the doctor monitor for signs of iron overload resulting from the breakdown of blood cells. Everyone who has sickle cell disease suffers anemia, which causes severe and often disabling fatigue. About 8% of all African-Americans carry at least one gene for sickle cell disease, and about 1 in 625 African-Americans has both of the genes for the disease, with the full range of symptoms. Varying degrees of kidney failure in people who have sickle cell anemia and in people who just carry one copy of the gene for sickle cell anemia. Most patients in this category require the use of a therapeutic intervention to restore normal sinus rhythm. Achieving a complete understanding is limited by the complexity of this disorder and the heterogeneous patient population it affects. Over time, myocardial fibrosis develops within the atrial tissue in association with AF to support its maintenance by shortening affected tissue refractory periods. That said, some patients with AF are genuinely asymptomatic, even at rapid heart rates for unclear reasons. The baseline on the ECG strip often is undulating and occasionally has coarse irregular activity (Figure 3). When intravenous (IV) pharmacologic therapy is required, the drug of choice is procainamide or amiodarone.
Meta-analysis of studies involving the ablate-and-pace approach8 has shown demonstrated improvements in a number of clinical parameters, including symptoms, quality of life, exercise function, cardiac performance and even longevity among patients with CHF receiving a bi-ventricular pacemaker.
Warfarin has been shown to reduce the annual average relative risk of stroke by 68%, whereas the reduction with aspirin ranges from 0% to 44% (mean, approximately 20%).
In clinical studies, these agents have generally proven themselves as equally effective, if not more effective, than warfarin but with lower bleeding rates with the added convenience of not requiring INR monitoring. A rhythm control strategy often requires the use of antiarrhythmic drugs that may have significant and even life-threatening side effects, and procedures that carry uncommon but potentially life-threatening or disabling complications.
Acutely, restoration of sinus rhythm may be achieved with either pharmacologic or electrical cardioversion.
Similarly, it is reasonable to add an antiarrhythmic drug for any patient who develops an early AF recurrence after direct-current cardioversion and to consider a repeat attempt after the drug has been initiated and reaches steady state blood levels.
Its use has been restricted by the FDA to certified prescribers and requires monitored initiation in a hospital setting followed by structured outpatient follow-up. The blood tests are often repeated at regular intervals, approximately every 6 to 12 months, and the ophthalmologic examination should be performed yearly. Pacemakers may be implanted simply for pacing support in patients with post-AF conversion pauses, or symptomatic bradycardia while in AF.
However, this approach does not address the fibrillating atria, and such patients still require systemic anticoagulation for thromboembolism and stroke prevention. Furthermore, there are no prospective data to support cryoballoon use among patients with persistent AF as this technique is purely aimed at achieving PVI without significant substrate modification. Beta-thalassemia occurs in persons of Mediterranean as well as African descent, and is also generally easier to treat than sickle cell disease.
This drastic treatment, if successful, would create a new source of healthy red blood cells so that the child never experiences the disease.
A medication for chronic pain called hydroxyurea is available in the United States, but much of the medical attention for sickle cell disease consists of crisis management. Children who have the condition are especially susceptible to infections with parvovirus or strep throat, and adults who have the condition are especially susceptible to food poisoning with Salmonella.
In some parts of Africa up to 30% of the population carries at least one gene for the disease, and the condition is also found in Sicily, Turkey, and India, where malaria was once prevalent. The kidneys lose their ability to concentrate waste products, the urine is unusually watery, and dehydration is a constant threat. Since the patient's bone has to be irradiated and new bone marrow injected, the procedure is expensive, painful, and risky. This has led to the long-held adage that 'AF begets AF.' The multiple wavelet model has suggested that AF is sustained by multiple simultaneous wavelets meandering throughout the atria. More often, however, patients report nonspecific symptoms such as fatigue, dyspnea, dizziness, and diaphoresis. This activity may resemble atrial flutter, but it is not as uniform from wave to wave as atrial flutter. However, these drugs have not yet been established in an extended period of post-FDA approval to rival decades of clinical experience associated with the use of warfarin. Some nonrandomized trials have reported an increase in mortality among patients who were on long-term antiarrhythmic therapy for AF, presumably from the proarrhythmic effects of the drugs.
It is important to remember that electrical and pharmacologic cardioversion are no different with regard to the risk of thromboembolic stroke. Unlike sotalol, however, it does not cause excessive bradycardia and thus can be administered to patients without concern for exacerbating pre-existing bradycardia. Sinus node dysfunction in association with AF is often referred to as tachycardia-bradycardia syndrome (tachy-brady for short). In general, patients have difficulty tolerating even the low-energy internal cardioversion shocks or frequent anti-tachycardia pacing sequences without the deep sedation provided during conventional external cardioversion. Outcomes data suggest that PVI alone without substrate modification works best in patients with paroxysmal AF, and that patients with persistent AF may derive additional benefit from additional substrate-based approaches.
For this reason, utilization of cryoablation appears to have plateaued, and traditional RF ablation remains the overall more commonly applied method.
Procedures typically take 4 to 6 hours, involve the use of radiation X-ray, and have an expected hospital course of overnight observation with planned discharge the next day.
Non-incisional lesions may be placed using bipolar radiofrequency, cryothermy, or microwave energy. It is important that he use a stabilizing device like a cane to prevent falls as he becomes more unstable on his feet. Sickle cell disease often causes ptosis, enlargement of the eyes, gallbladder pain, wounds on the legs that will not heal, and, in males, sometimes as early as the age of 12, priapism, continued and uncontrolled erection. Permanent AF refers to patients in which efforts to restore normal sinus rhythm have either failed or been forgone.
Atrial tissue with abnormal electrical propagation recorded by mapping catheters has been referred to as complex fractionated electrograms (CFEs). In addition, several randomized studies have compared the treatment strategies of ventricular rate control or rhythm control with restoration and maintenance of sinus rhythm, albeit in older patients (mean age, 65-70 years) with minimal or no symptoms during AF. Therefore, the requirements for anticoagulation apply equally to either treatment strategy and are largely dictated by the patient-specific thromboembolic risk profile discussed previously.
Flecainide and propafenone are usually well tolerated and are appropriate first-line options for the treatment of AF in patients without structural heart disease, particularly cardiomyopathy, including hypertrophic cardiomyopathy. This agent does have many potentially lethal drug-to-drug interactions, including many commonly prescribed antibiotics and anti-hypertensive drugs.
Dronedarone is a newer anti-arrhythmic drug designed to function similarly to amiodarone but without the molecular iodine interface associated with some of the previously described amiodarone toxicities. Outcomes associated with surgical approaches are comparable with catheter ablation (reported higher in some series) and offer the advantage of concomitant exclusion of the left atrial appendage.23,24 However, surgical approaches are more invasive than catheter ablation and requires either a sternotomy or a thoracotomy, plus general anesthesia and a longer post-operative recovery period.
CMT is progressive so the symptoms and loss of function worsen and the person eventually becomes disabled. These categories are not mutually exclusive and it is common for patients with one type of AF to exhibit overlapping features of another type. Expression of specific connecting protein channels at the cellular level are also felt to be important contributors to the disease substrate and sustaining mechanisms. Occasionally, patients present with extreme manifestations of hemodynamic compromise, such as chest pain, pulmonary edema, or syncope.
Pacemaker implantation without AV nodal ablation should be considered if the problem is simply excessive bradycardia that prohibits the effectiveness of rate-controlling medication.
For patients who have been in AF for more than 48 hours and are not adequately anticoagulated, electrical or pharmacologic cardioversion should be delayed until appropriate measures are taken to reduce the thromboembolic risk. There is a sustained-release formulation of propafenone that offers the advantage of twice-daily dosing rather than thrice-daily dosing, as for the immediate-release formulation.
Despite all of these limitations and drawbacks, many patients enjoy improved AF control on this agent without the nuisance of the daily side effects that limit use of some of the other anti-arrhythmic drugs.
Early enthusiasm for this drug, based on results from the initial studies, was later tempered by safety concerns and limitations. Refinement in techniques have resulted in a lower incidence of complications, notably PV stenosis, which used to be very common in the early era of catheter ablation. The incidence of perioperative complications has been low, but perhaps greater than catheter ablation.
In particular, the distinction between 'long-standing persistent' and 'permanent' AF largely reflect a treatment intention to restore normal sinus rhythm, or accept AF respectively among patients who have been in AF for more than 1 year. Strategies for suppression or cure of AF should be considered for appropriate patients before pursuing ablation of the AV node. There are two approaches for patients being considered for cardioversion of AF longer than a duration of 48 hours. Experienced centers have reported high rates of successful AF ablation resulting in discontinuation of antiarrhythmic drug therapy.20,21 The ideal candidate is a patient with paroxysmal AF in the absence of structural heart disease.
There is a potential need for a permanent postoperative pacemaker in as many as 7% to 10% of cases.
It is contraindicated for patients with advanced (NYHA functional class IV failure) and was found to increase cardiovascular death rates when given to patients with permanent AF in the Permanent Atrial fibriLLAtion Outcome Study Using Dronedarone on Top of Standard Therapy (PALLAS) study.19 This drug also has some GI side effects that can be partially mitigated in many patients by taking with food. It also has some important drug-to-drug interactions, including with the anticoagulant drug dabigatran. In some cases, cardioversion cannot be postponed for 3 or 4 weeks; in other cases, the patient, clinician, or both may prefer an expedited approach to achieving sinus rhythm. That said, it is a reasonable treatment option for patients without structural heart disease, or advanced liver disease with the principal advantage of not requiring hospital-based initiation like dofetilide.
In such cases, once a therapeutic level of anticoagulation has been achieved with warfarin or IV heparin, TEE may be performed to exclude the presence of an intracardiac thrombus.
Several centers have been using minimally invasive incisions and even thoracoscopic approaches with robotic equipment. TEE can detect the presence of a thrombus in the left atrium, particularly in the left atrial appendage, which is poorly seen on transthoracic echocardiography.



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