Ventrikuläre Tachykardie         Zurück                                 


Titel

VT = ventrikuläre Tachykardie = Kammertachykardie

Krankheitsnummer ( ICD )

I47.2 Ventrikuläre Tachykardie

engl Bezeichnung + Abkürzungen

VT = Ventrikuläre Tachykardie

englische Begriffe:

wide complex tachycardia

LBNA ventricular tachycardia = ?

Definition

VT = ventrikuläre Tachykardie = Kammertachykardie

= Beschleunigter Herzschlag mit einer Frequenz > 100 dessen Reizursprung in der Kammer liegt.

Eine Salve konsekutiver ventrikulärer Schläge mit einer Frequenz > 100 Schlägen/min

Kriterien der Kammertachykardie

Frequenz :

3 oder mehr aufeinanderfolgende QRS Komplexe ventrikulären Ursprungs mit einer Frequenz > 100/min

Obere Frequenzgrenze unscharf (Kammerflattern 250 - 400 / min)

Dauer:

QRS-Breite > 0,12 msek

Bei Tachykardie mit QRS > 0,12

Vorhof:

entweder unabhängig langsam mit 60 - 100/min

Capture Beats und durchwandernde P-Wellen zeigen die AV Dissoziation an

Einer Kammertachykardie muß nicht unbedingt eine kreisende Erregung zu Grunde liegen.

Ein Kammerrhythmus mit einer Frequenz unter < 100 heißt idioventrikulärer Rhythmus .

Einteilungen

Dauer:

EKG Morphologie

Ventrikuläre Tachykardien können

Ätiologie

Meist auf dem Boden einer schweren strukturellen kardialen Erkrankung.

Anhaltende ventrikuläre Tachykardien komplizieren eine Vielzahl von Herzerkrankungen, am häufigsten:

Epidemiologie

Kosten

Pathologie

Pathophysiologie

Symptome und Klinik

Diagnostik

Jede Tachykardie mit breitem QRS-Komplex (QRS über 120 msec.) sollte als ventrikuläre Tachykardie angesehen werden, bis das Gegenteil bewiesen ist.

EKG:

Das Vorhandensein von

Hinweise für eine VT

Diagnose.

Eine Tachykardie, die nicht ventrikulären Ursprungs ist (z.B. eine echte Vorhoftachykardie, eine Intra- oder Post-AV-Knoten-Tachykardie und die Reentry-Tachykardien, die ein akzessorisches Leitungsbündel benutzen), kann aberrierend geleitet werden und so eine Tachykardie mit breitem QRS- Komplex herbeiführen. Dies ist sehr selten, jedoch wird die Diagnose wiederholt und irrtümlich bei Patienten mit ventrikulärer Tachykardie gestellt.

         Anamnesefragen

Hatten Sie schon einmal einen Herzinfarkt ?

Haben Sie eine Herzerkrankung ?

Seit wann besteht die Rhythmusstörung ?

Wie oft ist sie schon aufgetreten ?

       Körperliche Untersuchung

         Labor

Elektrolyte

Herzenzyme

         Sonstige Verfahren

Echo

Herzkatheter

EPU

Spezial Untersuchungen

         Differential Diagnostik

Bilder

Nicht anhaltende VT

Therapie

AkuteTherapie

Chronische Therapie

Akute Therapie

Eine ventrikuläre Tachykardie mit Blutdruckabfall erfordert eine Soforttherapie mit einem synchronisierten Elektroschock. Häufig kann mit einer Stromstärke von 50 Wattsekunden ein Sinusrhythmus wiederhergestellt werden. Eine programmierte Stimulation ist eine Alternative, aber gewöhnlich zu zeitaufwendig.

Die medikamentöse Therapie ventrikulärer Tachykardien wird, mit Lidocain 100 mg i.v. durchgeführt, was über 2 Minuten gegeben wird mit einer weiteren Dosis von 50 mg i.v. 5 Minuten später, wenn die Arrhythmie nicht umgeschlagen ist.

Verlauf

Fälle

VT nach großem VWI

Frau S ,

Herr Pr.

Arrhythmogenic Right Ventricular Dysplasia

by Jonathan A. Bradlow M.D. and Steven L. Zweibel M.D.

Montefiore Medical Center, Bronx, NY 10467

Zusammenfassung:

A 31 year old white female with a family history of arrhythmogenic right ventricular dysplasia (ARVD) presents with palpitations and a wide complex tachycardia. Coronary angiograms and left ventriculography were unrevealing, whereas cardiac MRI and right ventriculography were suggestive of ARVD. EP studies found easily inducible ventricular tachycardia with a left bundle, normal axis configuration. Two separate RF ablations were attempted in the region of the right ventricular outflow tract, however the patient still had inducible VT by EP and exercise testing. She was begun on beta blocking agents and ultimately discharged with no exercise-induced arrhythmia. The patient was readmitted for VT and is now arrythmia free on Sotolol.

Case Presentation

F.P. is a 31 year old female without significant past medical history who was transferred to our hospital for evaluation of ventricular tachycardia.

The patient first noted near-syncopal episodes not associated with palpitations approximately 7 years prior to this admission. Work-up at that time including exercise stress test, Holter monitoring, and echocardiogram were all within normal limits. The patient was asymptomatic until three years prior to this admission when she noted episodes of rapid, irregular heart beat while she was exercising on a treadmill. She noted the symptoms to last for about half an hour before resolving abruptly and spontaneously. She again underwent an exercise stress test to attempt to provoke the arrhythmia but that test was negative. Two years prior to her admission the patient noted "skipped beats" during an episode of emotional stress and an electrocardiogram at that time revealed normal sinus rhythm with atrial premature contractions.

She was in her usual state of excellent health except for a mild upper respiratory tract infection one week prior to admission. On the day of admission when the patient was exercising on a treadmill she suddenly noted the onset of fluttering in her chest. She denied dizziness, syncope, or chest pain. The patient drove herself to the Emergency Room of a local hospital where she was found to be in a wide-complex tachycardia with a ventricular rate of 220 bpm (Figure 1). Systolic blood pressure was initially 90 mmHg.

The patient was given 6 mg of adenosine followed by 12 mg of adenosine followed by 100 mg of IV lidocaine without any response. No slowing was noted with carotid sinus massage. After the infusion of 1 gram of procainamide the patient's heart rate decreased to 180 bpm followed by conversion to normal sinus rhythm with occasional premature ventricular contractions. Electrocardiograms post-conversion (Figure 2) revealed normal sinus rhythm with 1-2 mm ST segment depressions in leads I, II, V2-V6 which resolved over the following few hours. The patient was chest pain free throughout. Cardiac enzymes were significant for a peak CPK of 840 with an MB fraction of 10%. The patient was transferred to our hospital for left and right heart catheterization with right ventricular biopsy as well as a cardiac MRI and electrophysiologic studies.

Figure 1. 12 lead electrocardiogram on presentation. LBNA ventricular tachycardia.

Figure 2. 12 lead electrocardiogram after cardioversion.

Medications (before admission): None.

Allergies: None.

Social History: Denies tobacco, alcohol or illicit drug use. The patient works in public relations, is single, and has no children.

Family History: Significant for patient's mother being diagnosed with arrhythmogenic right ventricular dysplasia (ARVD) with recurrent ventricular tachycardia requiring implantation of a cardioverter-defibrillator. She is alive and well after 10 years of follow-up. No other significant family history of heart disease.

Physical Exam: Appearance: Patient is a well developed, well nourished female Vitals: BP 120/70 HR 74 RR 14 afebrile

HEENT: normal Neck: no JVD, brisk carotid upstroke without bruits

Chest: clear to auscultation and percussion

Cardiac: normal PMI; regular rate and rhythm, S1, S2 normal with no S3 or S4; no rubs, murmurs, gallops or thrills

Abdomen: soft, no hepatosplenomegaly, normal bowel sounds were present Extremities: no clubbing, cyanosis, or edema 2+ pulses throughout

Neurologic: alert and oriented x 3, non-focal exam

Skin: No rashes

Labs: WBC 8.6, Hct 39%, Platelets 253,000

Na+ 141, K+ 3.8, Cl- 108, Glucose 86, Creatinine 0.9, Ca++ 8.7 Phos 3.3

CPK peak 840, 10% MB LDH 273 SGOT 138 SGPT 21 Cholesterol 152

beta-HCG: negative Urinalysis: negative

Procainamide 4.9 (therapeutic 2-6), N-Acetyl-Procainamide 1.4 (therapeutic 6-20)

PT 13, PTT 28

CXR: No infiltrate or effusion. Normal cardiac silhouette.

Cardiac MRI: No evidence of fatty displacement of muscle tissue, however, increased signal was noted in small areas of the interventricular septum on the T1 weighted images, possibly suggestive of early ARVD, possibly normal variant.

Endomyocardial biopsy: Endocardium was unremarkable; myocytes were not hypertrophied; no adipose tissue infiltration; mild interstitial fibrosis; no inflammation was noted; blood vessels unremarkable. Limited, small biopsy samples - cannot exclude possibility that an infiltrative process could have been missed.

Cardiac Catheterization:

Left heart - normal left ventricular size with minimal decrease in left ventricular function, LVEDP 8-10, normal coronary arteries

Right heart - normal pressures, low normal cardiac index, right heart ventriculogram showed a small area of dilatation of the right ventricular outflow tract, also possibly suggestive of early ARVD or normal variant

(Figure 3).

Figure 3. Views of the right ventricle:

Figure 3a. Anterior-posterior view in systole.

Figure 3b. Anterior-posterior view in diastole.

Figure 3c. Lateral view in systole.

Figure 3d. Lateral view in diastole.

Hospital Course: EP Study:

Conduction intervals were within normal limits

SA node: normal

Atrium: no atrial arrythmias were inducible with extrastimulus testing

AV Node: dual AV node physiology was demonstrated

Ventricle: Programmed electrical stimulation at the RV apex revealed easily inducible left bundle normal axis (90 degrees in the frontal plane) ventricular tachycardia at rates of 220-230 bpm with triple extrastimuli; easily terminated by burst pacing. Radiofrequency energy was applied to the anteroseptal area of the RV outflow tract with resolution of VT inducibility.

Exercise Testing: showed a normal hemodynamic response to exercise testing with 97% MPHR achieved. Sustained but self terminating VT of the same morphology occurred during cool down. Test performed while the patient was off medicines.

Holter monitor: Three Holter monitors were done without VT on routine ambulation.

After the ablation the patient had recurrent VT and opted to undergo a repeat procedure with more RF energy applied to the anteroseptal area of the RV outflow tract and tricuspid annulus; this time guided by pace mapping and activation sequence mapping. VT (LBNA) was still inducible with sinus and a single extrastimulus while on isoproterenol. After the second ablation the exercise tests revealed VT of the same morphology as previously described.

The patient was then placed on low dose atenolol. However, VT occurred with 8 mets of exercise on repeat exercise testing. On high dose atenolol (75 mg per day) the patient exercised to 11 mets without recurrence of VT.

The patient was discharged on the thirteenth hospital day on a medical regimen of enteric coated aspirin and Atenolol 75 mg per day.

Follow-Up: The patient was re-admitted to the hospital about 3-4 weeks later in VT which was terminated with IV propranolol and IV procainamide. Repeat Holter monitoring revealed normal sinus rhythm with very frequent premature ventricular contractions. Repeat exercise testing was negativ

FALL NEJM 1993 , Vol 329 , P.1639, Weekly Clinicopathological

Exercises : Case 47- 1993 : A 28-Year Old Man with Recurrent VT and Dysfunction of Multiple Organs

Case Records of the Massachusetts General Hospital

AUTHOR(S): Venditti, Ferdinand J., Jr.; Fallon, John T.

PRESENTATION OF CASE A 28-year-old man was admitted to the hospital because of recurrent ventricular tachycardia.

There was a 12-year history of chronic active hepatitis with cirrhosis for which the patient took prednisone daily. Nine years before entry ulcerative colitis developed, with frequent exacerbations, especially during the spring.

Four years later eosinophilia was detected; it ranged from 10 to 20 percent on differential counts and decreased during intervals when increased doses of prednisone were given. Anosmia and alopecia universalis developed, with apparent improvement when doses of prednisone were higher.

Fifty-eight months before entry third-degree atrioventricular block occurred, and a subcutaneous dual-chamber pacemaker was implanted. One year later the patient experienced the onset of asthma and allergic rhinitis, which improved with increases in the dose of prednisone. One year thereafter diarrhea recurred, and a test on a stool specimen showed Clostridium difficile toxin. Metronidazole was ineffective; vancomycin relieved the symptoms, but they recurred several times. In the same year warts developed in the perianal region and on the genitalia, hands, and feet; they were managed with locally administered liquid nitrogen. Intermittent fungal infections involved the toes.

A year and a half before admission a diagnosis of idiopathic thrombocytopenic purpura was made; a splenectomy was followed by remission; a liver biopsy confirmed the diagnosis of hepatic cirrhosis. A cardiac ultrasonographic examination was reported to be negative. Nine months before admission the patient was evaluated at another facility, where a tentative diagnosis of systemic lupus erythematosus was rejected. A diagnosis of a "systemic autoimmune disease" was made. Four months before entry another exacerbation of ulcerative colitis developed and was managed elsewhere. Radiographs of the chest were reported to be normal. Seven weeks before admission the patient experienced increasing dyspnea, fatigue, and dizziness, without palpitations or chest pain, and found that he had gained approximately 3 kg in weight. Four weeks before entry a prolonged tachyarrhythmia occurred, with dyspnea. A wide-complex tachycardia was observed on an electrocardiographic monitor while the patient was being transported to a hospital; it resolved after the intravenous administration of lidocaine. An x-ray film of the chest showed cardiac enlargement. Another cardiac ultrasonographic examination revealed a left ventricular ejection fraction of 10 to 15 percent. Electrophysiologic studies disclosed an inducible sustained monomorphic ventricular tachycardia, which was not suppressed by quinidine, mexiletine, or amiodarone and was terminated by overdrive pacing. Dobutamine and dopamine were given by vein, and hydralazine and furosemide were begun by mouth; prednisone  was continued. A diagnosis of nonischemic dilated cardiomyopathy was made. Heart transplantation was considered but rejected, and the patient was transferred to this hospital.

The patient was a student. Twelve years before admission he had worked at bicycle repair and used a silicone lubricant frequently. At the time of admission, he had lost 6.8 kg in weight in recent weeks, with a decrease in food intake and the daily use of furosemide. His current medications included prednisone (25 mg daily), furosemide (40 mg daily), hydralazine (50 mg four times daily), cimetidine, potassium chloride, and magnesium chloride. There was no history of use of tobacco or alcohol, xerophthalmia, xerostomia, rash, photosensitivity, arthralgias, Raynaud's phenomenon, myopathy, sexually transmitted diseases, diabetes mellitus, valvular heart disease, thromboembolic disease, homosexual activity, or intravenous drug abuse or receipt of blood products, and no family history of coronary artery disease, sudden death, asthma, or autoimmune disease. The temperature was 37.7°C, the respirations were 18, and the blood pressure was 95/70 mm Hg; the heart rhythm was electronically paced at 85 beats per minute.

On physical examination the patient was completely hairless and appeared well. No lymphadenopathy or cutaneous abnormality was found except for perianal warts. The head and neck were normal; the jugular venous pressure was less than 5 cm. A subcutaneous pacemaker generator was present in the right pectoral region; the lungs were clear. A paced heart rhythm was regular; a sustained left ventricular impulse was palpated in the sixth intercostal space at the anterior axillary line, and a soft S3 sound was heard; no murmur was detected. The liver was not felt. The extremities and joints were normal, and the peripheral pulses were intact; there was onychomycosis of the toenails; no peripheral edema, cyanosis, or digital clubbing was found. The genitalia and rectum were normal; a stool specimen was negative for occult blood. Neurologic examination was negative. The urine was normal. The hematologic and blood chemical values are presented in and . An electrocardiogram showed atrioventricular sequential pacing at a rate of 87 beats per minute. X-ray films of the chest () revealed an electronic pacemaker in the right anterior chest region, connected to electrodes that terminated in the area of the cardiac apex and right atrial appendage; the heart was slightly enlarged; the aorta, mediastinum, and pulmonary vasculature appeared normal, and the lungs were clear. Another cardiac ultrasonographic examination disclosed dilatation and diffuse hypokinesis of both ventricles; the left ventricular internal diameter was minimally increased, with an estimated ejection fraction of 36 percent; a pacemaker wire was present in the right heart chambers, and there was evidence of slight tricuspid regurgitation on Doppler echocardiography; the estimated right ventricular systolic pressure was 26 mm Hg; the pulmonic, mitral, and aortic valves appeared normal.

Serum immunoelectrophoresis showed a normal IgG precipitin arc, with an increased concentration; the IgA and IgM precipitin arcs and concentrations were normal. The IgG was 1510 mg, the IgA 309 mg, and the IgM 281 mg per deciliter. A test for Bence Jones protein in a 50-fold concentrated specimen of urine was negative; small amounts of albumin and slightly larger amounts of alpha-1 globulin were present. The results of serologic testing are presented in .

The patient's usual medications were continued. The temperature ranged between 38.3 and 36.9°C at all times, and the blood pressure was 90/60 to 95/70 mm Hg on most occasions. The patient remained comfortable at rest, with constant electronic pacing.

A diagnostic procedure was performed.

DIFFERENTIA L DIAGNOSIS

Dr. Ferdinand J. Venditti, Jr. *: May we review the x-ray film of the chest and the echocardiographic study?

[* Chief, Section of Cardiology, and director, Arrhythmia Service, Lahey Clinic, Burlington, Mass.; clinical instructor in medicine, Harvard Medical School.]

Dr. Stephen W. Miller: A film of the chest () obtained on the recent admission shows moderate cardiac enlargement and the permanent cardiac pacer, with its wires in the right atrium and right ventricle. The lungs are clear. There is no evidence of mediastinal lymphadenopathy.

Dr. Mary Etta King: The parasternal views from the echocardiographic study demonstrate left ventricular dilatation with normal wall thickness and no increased reflectivity of the ventricular myocardium. The left atrium is of normal size. There is slight thickening of the aortic-valve cusps but no stenosis. There is no pericardial effusion. The cross-sectional views of both ventricles show diffuse hypokinesis. The estimated left ventricular ejection fraction was 36 percent. There was no evidence of an intracardiac thrombus.

The Doppler inflow pattern across the mitral and tricuspid valves was normal, with normal diastolic filling. There was no evidence of mitral regurgitation on color-flow Doppler study. There was a small jet of tricuspid insufficiency, with a peak velocity of 2 m per second, indicating a right ventricular pressure of 26 mm Hg.

Dr. Venditti: In summary, this young man had a multisystem disorder. Cardiac manifestations developed over a five-year period and included complete heart block, congestive heart failure, and sustained ventricular tachycardia. I shall begin by reviewing the classification of cardiomyopathy and myocarditis. Disease in either category could have been responsible for this patient's illness.

Cardiomyopathies have been divided into hypertrophic, dilated, and restrictive types. The normal thickness of the ventricular walls in this case excludes hypertrophic cardiomyopathy.

Restrictive cardiomyopathy is the least common of the three groups in the United States. Its hallmark is a "stiff" ventricle, with abnormal diastolic function and often with normal systolic function. Myocardial hypertrophy, fibrosis, or infiltration can be the underlying pathologic process. Restrictive cardiomyopathy may accompany amyloidosis, hemochromatosis, glycogen storage diseases, collagen-vascular diseases, eosinophilia, neoplastic disease, and fibroplastic disorders, among others. Since most of these disorders result in only a restrictive cardiomyopathy, I shall exclude them, although several of them deserve comment. Amyloidosis is characterized by an abnormal deposition of amyloid fibrils in almost any organ. Cardiac involvement can result in systolic dysfunction and congestive heart failure, restrictive cardiomyopathy, orthostatic hypotension, and arrhythmias . Up to 45 percent of the patients have atrioventricular block; complex ventricular arrhythmias and sudden death are also common . The diagnosis can be suspected clinically but is usually made by biopsy of the liver, kidney, rectum, gingiva, or bone marrow. I would have expected the diagnosis to have been made by liver biopsy in this case, if amyloidosis was present.

Hemochromatosis is characterized by iron overload in many organs, causing dysfunction. Typically, the liver is the first organ affected, with hepatomegaly, frequently occurring without elevation of liver enzyme values. Alopecia, skin pigmentation, diabetes, arthropathy, testicular atrophy, gynecomastia, and palmar erythema are also seen. Cardiac involvement can result in dilated as well as restrictive myopathy, but conduction-system abnormalities and ventricular arrhythmias are uncommon . The diagnosis is important to make, since therapy can arrest or reverse cardiac dysfunction. Again, a liver biopsy would be diagnostic.

Dilated cardiomyopathy is the most common type and is characterized by increased ventricular size, reduced systolic function, and as in this case congestive heart failure. In most cases the cause is unknown. The  syndrome may be the outcome of many toxic, metabolic, infectious, and inflammatory insults to the myocardium. The course of dilated cardiomyopathy is usually progressive, with worsening heart failure. The ventricles are generally uniformly involved, but segmental wall-motion abnormalities can be seen early in the course of the disease. As ventricular dilatation progresses, mitral and tricuspid valvular insufficiency can develop. The course can also be complicated by atrial tachyarrhythmias, conduction-system abnormalities, ventricular tachycardia, and fibrillation. In view of the acuteness of this patient's presentation, acute myocarditis is more likely. Its causes include infectious, inflammatory, and toxic agents. I shall exclude toxins because of the lack of a history of exposure to them.

Viral myocarditis has to be considered. Symptomatic acute viral myocarditis can be characterized by chest pain, congestive heart failure, bradyarrhythmias, tachyarrhythmias, and even sudden death. The long-term outcome after an episode of myocarditis varies; frequently, there is no residual myocardial dysfunction. Some authors have speculated that idiopathic dilated cardiomyopathy is the result of a remote viral myocarditis that causes immunologic damage in the heart .

The viruses that are most commonly implicated in acute myocarditis are the coxsackie group B viruses, or enteroviruses, which often involve the heart, presumably because of an affinity of myocardial-membrane receptors for viral particles . The course is usually acute, and other symptoms precede the development of myocarditis, including myalgias, fever, nausea, vomiting, upper respiratory tract symptoms, arthralgias, and pleurodynia, none of which were present in this case. Hepatitis B virus has also been implicated as a cause of acute myocarditis. Lymphocytic infiltrates, petechiae, fatty infiltration, and edema can be seen on microscopical examination of the myocardium. The presentation is usually during the acute phase of viral hepatitis, with congestive heart failure and arrhythmias. The course is frequently fulminant and fatal . The results of viral serologic tests are not reported in this case.

Nonviral infectious causes, such as bacteria, including mycobacteria, and rickettsia, are unlikely in this case in view of the absence of typical associated signs and symptoms. Cardiac involvement in Lyme disease, a spirochete infection transmitted by ticks, may take the form of complete atrioventricular block, myopericarditis, ventricular tachycardia, and left ventricular dysfunction . However, the absence of reported exposure, arthritis, and erythema chronicum migrans and the development of complete heart block during prednisone therapy make this diagnosis improbable. I do not believe that acute infectious myocarditis would explain all the findings in this case.

The multisystem involvement in this patient suggests a systemic disease, with a chronic course punctuated by acute myocardial involvement. In a young patient with complete heart block and sustained ventricular tachycardia, sarcoidosis deserves consideration. It frequently affects the lungs, eyes, liver, spleen, lymph nodes, nervous system, kidneys, skin, and heart; involvement of the gut is very uncommon . The hallmark of sarcoidosis is a noncaseating granuloma, which can heal with fibrosis. Pulmonary involvement is characteristic, but isolated cardiac involvement has been reported . Sudden death, conduction-system disturbances, ventricular tachycardia, congestive heart failure, pericardial effusions, papillary-muscle dysfunction, and acute myocardial infarction have been reported in cardiac sarcoidosis . Granulomatous replacement of the myocardium can result in a restrictive or dilated cardiomyopathy. Congestive heart failure can be the result of either acute or chronic myocardial involvement . Variable amounts of myocardium can be involved, and localized involvement of the conduction system can occur without extensive myocardial damage ; conversely, massive cardiac involvement can be seen in the absence of manifest involvement of other organs . The base of the interventricular septum and the left ventricular free wall are frequently affected . Involvement of the septum explains the propensity for conduction-system disease, whereas involvement of the left ventricle, if extensive, can result in sustained ventricular tachycardia. In one report 48 percent of the patients had conduction-system disturbances, 32 percent had tachyarrhythmias, and 24 percent died suddenly . In another series 67 percent of the deaths were sudden, and in 17 percent of the patients sudden death was the initial manifestation of the disease . Ventricular free-wall involvement may progress to aneurysm formation, particularly during steroid treatment . Although steroid therapy is indicated in the presence of myocardial involvement, the cardiac disease may progress during treatment. Although some of the immunologic abnormalities seen in this case can occur in sarcoidosis, eosinophilia of longstanding duration and asthma are uncommon. Also, the systems involved in this patient are not typically affected in sarcoidosis. Although sarcoidosis is a possibility, another syndrome better fits the clinical presentation in this case. I believe that the chronic eosinophilia was important in assessing the cause of this patient's disease.

There are many causes of eosinophilia (). Bronchial asthma, allergic rhinitis, and urticaria can be accompanied by moderate eosinophilia, but these disorders do not usually affect other organs and therefore do not explain the cardiac syndrome in this patient. Mycotic infections, such as bronchopulmonary aspergillosis, coccidioidomycosis, and  oromucocutaneous candidiasis, can be associated with eosinophilia . Although all three causative fungi can involve the heart, the usual presentation is during systemic mycotic infections in immunocompromised hosts and results principally from endocardial and pericardial involvement .

Persistent severe eosinophilia of infectious cause usually occurs in tissue- invasive parasitic diseases, such as strongyloidiasis, trichinosis, schistosomiasis, filariasis, and toxocariasis . There is usually an associated elevated IgE level. Although the participation of eosinophils in the immune response remains enigmatic, they may have a role in the host response to these parasites . Eosinophil-derived proteins have toxic effects on helminths, although the clinical importance of this effect is unclear . Several of the helminths, such as Toxocara canis, rarely involve the heart primarily during the tissue-invasive phase of the infestation. The presentation is usually that of an acute myocarditis, with fever, dyspnea, and involvement of other organ systems, frequently resulting in death . The diagnosis should be suspected whenever there is evidence of exposure, which was lacking in this case.

Other parasitic infestations, such as strongyloidiasis, would result in overwhelming infection in the setting of prednisone therapy. Therefore, I do not think that the eosinophilia and cardiac disease in this case were due to parasitic disease.

Idiopathic or autoimmune chronic active hepatitis has been reported to be associated with eosinophilia . This syndrome typically affects boys and young men and is very responsive to steroid treatment. Other autoimmune diseases have been absent in most patients with this disorder. In the case under discussion the serologic type of the hepatitis is unknown, but in view of the lack of risk factors for viral hepatitis this patient probably had autoimmune chronic active hepatitis. Although antinuclear antibodies, antithyroid antibodies, thrombocytopenia, and ulcerative colitis can all be associated with chronic active hepatitis, primary cardiac disease is not part of the syndrome.

Eosinophilia is infrequent in patients with collagen-vascular diseases, such as scleroderma, systemic lupus erythematosus, and polyarteritis nodosa. Systemic lupus erythematosus can cause pericarditis, noninfectious endocarditis, and mitral regurgitation . Rarely, myocarditis and myocardial infarction in patients with antiphospholipid antibodies have been reported . Although this patient had a positive test for antinuclear antibodies, the absence of other specific antibodies and of typical manifestations of systemic lupus erythematosus makes that diagnosis unlikely. Polyarteritis nodosa is a necrotizing vasculitis involving medium-sized and small arteries and adjacent veins. Because of the prominent renal involvement, the most common cardiac presentation is that of congestive heart failure and hypertension caused by renal failure. Myocardial infarction with subsequent ventricular dysfunction can also occur because of coronary artery vasculitis . A variant of polyarteritis nodosa is the Churg-Strauss syndrome, in which prominent eosinophilia and allergic disease antedate the vasculitic process. The heart is frequently involved in the vasculitic stage of this disease. I shall return to this entity later.

If an exhaustive workup reveals no cause of eosinophilia and if signs and symptoms of organ-system dysfunction are present, the diagnosis of the idiopathic hypereosinophilia syndrome must be considered. The cause of organ-system dysfunction in this syndrome may be directly related to the abundant eosinophils. Eosinophil major basic protein and eosinophil cationic protein, both present in the granules of eosinophils, have been detected in high concentrations in patients with organ involvement, and their levels seem to correlate with the extent of the dysfunction . Eosinophil major basic protein has been demonstrated to cause cell damage in vitro in concentrations frequently present in patients with the hypereosinophilia syndrome . Also, there appears to be an association between cardiac disease and degranulated eosinophils in the periphery in that disorder, with more extensive involvement noted with higher concentrations of degranulated eosinophils .

Virtually any organ system can be affected in the hypereosinophilia syndrome. Involvement of the neurologic, cutaneous, pulmonary, cardiac, and hepatic systems is common . Cardiac involvement is characterized by endomyocardial fibrosis, often referred to as Loffler's endocarditis. Hemodynamics are restricted by endomyocardial fibrosis . There may be valvular insufficiency because of the propensity for involvement of the support structures of the mitral and tricuspid valves . In addition, there is a characteristic obliteration of the apical segments of the right and left ventricles . These areas can be filled with clot, which can become organized, reducing the chamber size, and give rise to emboli . There is an initial acute myocarditis, which progresses with endocardial damage and thromboembolic events . The cellular infiltrate during the acute myocarditis is dominated by eosinophils ; ultimately, endomyocardial fibrosis ensues . Conduction abnormalities might be expected in this syndrome because of the frequent inflammation and fibrosis of the subendocardium. Indeed, 18 of 64 patients in one report had conduction abnormalities . Ventricular tachyarrhythmias have also been reported, but sustained arrhythmias seem uncommon . Both types of arrhythmia occur in persons with far advanced disease . This case has many features consistent with the hypereosinophilia syndrome. The cardiac abnormalities could have been due to the acute necrotic phase of myocardial involvement. However, some aspects of the history, such as the asthma, would remain unexplained if we attribute this patient's presentation to the hypereosinophilia syndrome.

The Churg-Strauss syndrome, or allergic granulomatosis and angiitis, best explains this patient's constellation of findings. This syndrome may be very similar to the hypereosinophilia syndrome clinically but is characterized, in addition, by asthma or allergic rhinitis and evidence of a necrotizing vasculitis involving at least two organ systems . Asthma is seldom a component of the hypereosinophilia syndrome, and there is no associated vasculitis . The Churg-Strauss syndrome is characterized typically by three phases -- asthma or atopic disease, peripheral eosinophilia, and ultimately vasculitis. The heart, lungs, skin, gastrointestinal tract, and nervous system are frequently involved. Peripheral eosinophilia is the hallmark of the disease, but because of the wide and rapid fluctuations of the eosinophil count it can be missed . In addition, steroids are potent suppressors of eosinophilia, and if they are administered for other reasons, as in this case, eosinophilia may be less prominent.

In the series reviewed almost all patients with the Churg-Strauss syndrome had a history of asthma, which is unusual in other vasculitic syndromes, such as polyarteritis nodosa and Wegener's granulomatosis . Asthma typically antedates eosinophilia by three years and vasculitis by five years . There is frequently a dissociation between asthma and the onset of vasculitis, with bronchospastic symptoms abating during the period of acute vasculitis . The characteristic pathological findings in this disease are tissue infiltration by eosinophils, extravascular granulomas, and necrotizing vasculitis involving small arteries and venules . Eosinophilic infiltrates are seen, particularly early in the development of the lesions.

Interstitial eosinophilic infiltrates and scattered fibrosis can be seen in the myocardium in addition to granulomas and vasculitis. Pulmonary involvement results in infiltrates, which have been reported in 72 percent of the cases of the Churg-Strauss syndrome . These infiltrates may be symmetric, involving the periphery and axillary zones, or they may radiate from the hilus, like the infiltrates of pulmonary edema . Upper respiratory tract disease is typified by allergic rhinitis, which commonly occurs early in the disease. Eosinophilic infiltrates can involve any part of the gut.

With mucosal involvement of the large bowel, diarrhea, abdominal pain, and bleeding can occur. Up to 33 percent of the patients have diarrhea, and 18 percent have gastrointestinal bleeding during their clinical course . Colitis closely resembling ulcerative colitis can occur, with a relapsing and remitting course . This type of presentation results in the inclusion of eosinophilic colitis in the differential diagnosis of inflammatory bowel disease. Although gastrointestinal and pulmonary involvement can occur during the prodrome, skin, nervous-system, renal, and cardiac involvement is usually limited to the vasculitic phase. Malaise, fatigue, and lassitude often herald the onset of this phase. Cutaneous involvement is seen in 70 percent of the patients during the vasculitic phase . The manifestations include a macular or papular erythematous rash, urticaria, subcutaneous nodules, or purpura, which can be palpable. Purpura is by far the most common skin manifestation, occurring in almost half the patients. Biopsy of the subcutaneous nodules frequently reveals granulomas. The nervous system is involved in up to 75 percent of the cases of the Churg-Strauss syndrome . Mononeuritis multiplex is the most common lesion, but diffuse, symmetric peripheral neuropathy is also seen.

Central nervous system involvement usually occurs late in the course of fulminant disease. Renal involvement is limited, with only occasional examples of severe disease, in contrast to polyarteritis nodosa, in which most of the patients have clinically important renal involvement . Cardiac manifestations of the Churg-Strauss syndrome are the most serious, since half the mortality due to this disease is directly related to cardiac involvement . At autopsy granulomas are commonly found in the heart, most prominently in the epicardium but also diffusely in the myocardium, which may be extensively replaced by granulomas and fibrosis.

Acute myocarditis, constrictive pericarditis, cardiac tamponade, acute myocardial infarction, congestive heart failure, ventricular tachyarrhythmias, and complete heart block have all been reported as consequences of cardiac involvement . The obliterative and restrictive endomyocardial involvement that typifies hypereosinophilic endomyocarditis is seen rarely in this syndrome .

Patients with this syndrome are usually treated with corticosteroids or cytotoxic agents . Although involvement of the heart as well as other organs has progressed despite therapy, substantial improvement in myocardial function has been achieved with aggressive early treatment . Acute congestive heart failure can develop as doses of steroids are tapered, with reactivation of vasculitis, but responds to an increase in the dose, with resolution of the symptoms.

Therefore, I believe that this patient had the Churg-Strauss syndrome, with cutaneous, colonic, and cardiac involvement. The findings of eosinophilia and asthma support this interpretation. The purpura noted at the time that "idiopathic thrombocytopenic purpura" was diagnosed may have been due to skin involvement, and the accompanying thrombocytopenia may have been related to hypersplenism caused by cirrhosis. The recent exacerbation of ulcerative colitis may have been the result of colonic involvement. Early granulomatous involvement of the conduction system probably caused complete heart block. Recurrent involvement probably resulted in more extensive cardiac disease, with acute myocarditis. This situation was presumably complicated by systolic dysfunction and ventricular tachycardia. I believe that the procedure performed was a right ventricular biopsy at the time of cardiac catheterization, which could have demonstrated granulomas, eosinophilic infiltrates, necrotizing vasculitis, or fibrosis or a combination of these findings.

Dr. Robert E. Scully: Dr. Brooks, you took care of this patient. Will you tell us your impressions?

Dr. D. Ross Brooks: We thought that the patient had nonischemic dilated cardiomyopathy, possibly related to active myocarditis, with resultant congestive heart failure and recurrent ventricular tachycardia.

Dr. Scully: Dr. Merkel, will you comment on your impressions?

Dr. Peter A. Merkel: Some of the many abnormalities of his immune function are consistent with lupus. Immune thrombocytopenia is frequent and may precede the diagnosis by years. Myocarditis is seen in approximately 10 percent of patients with the disease, with the prevalence higher in autopsy series . Complete heart block occurs in infants who acquire maternal antibodies to Ro antigen, but in adults with systemic lupus erythematosus complete heart block is a manifestation of underlying myocardial disease. However, lupus-related myocarditis is usually seen in the presence of other obvious manifestations of the disease. The most interesting aspect with regard to the possibility of systemic lupus erythematosus is the high titer of antinuclear antibodies. Over 90 percent of patients with idiopathic systemic lupus erythematosus have a positive test for antinuclear antibodies, but the finding is not specific . Antinuclear antibodies are seen in patients with many other diseases thought to have an autoimmune basis as well as in some healthy persons. A high titer of antinuclear antibodies, as seen in this patient, increases the likelihood of a true positive test. To define better the antigen or antigens resulting in the presence of antinuclear antibodies in this case, testing for extractable nuclear antigens was done. No antibodies to Ro (SSA), La (SSB), Smith (Sm), or ribonuclear protein (RNP) antigens or to double- stranded DNA were found in this patient's serum. The presence of antibodies to Sm antigens or to double-stranded DNA would have been highly suggestive of a diagnosis of lupus in this patient . Therefore, although the patient had some features of systemic lupus erythematosus, it would not explain all his gastrointestinal, infectious, dermatologic, and hematologic manifestations.

Could this patient's positive test for antinuclear antibodies have resulted from his hydralazine use? Hydralazine is well known to cause tests for antinuclear antibodies to be positive. It is important to differentiate drug- induced seroconversion to positivity for antinuclear antibodies from drug- induced lupus in which the patient has clinical findings of systemic lupus erythematosus. Histone antibodies develop in over 90 percent of patients with hydralazine-induced autoimmunity . A test of this patient's serum for the presence of histone antibodies was negative, but the result was not available until after his discharge. Although the absence of histone antibodies does not exclude hydralazine as a cause of the positive test for antinuclear antibodies, it makes it considerably less likely. Furthermore, it is usually months between the start of hydralazine and the development of antinuclear antibodies rather than several weeks, as in this case. If the test for antinuclear antibodies in this patient had been positive because of hydralazine, the titer would have fallen and the test would eventually have become negative after discontinuation of the drug. Therefore, it seems possible that the patient had a yet undefined idiopathic autoimmune disease, but the positive test for antinuclear antibodies does not define his illness more precisely. Finally, the possibility that this patient was extensively exposed to silicone about the time that his clinical problems began raises the controversial issue of silicone-induced autoimmune disease. This is an area of intense current investigation in view of the multiple reports of the development of clinical features of autoimmune disease in patients with silicone-based breast implants .

CLINICAL DIAGNOSIS Dilated cardiomyopathy; ? active myocarditis.

DR. FERDINAND J. VENDITTI, JR.'S, DIAGNOSIS Churg-Strauss syndrome, with acute myocarditis and cutaneous and colonic  involvement.

PATHOLOGICAL DISCUSSION Dr. John T. Fallon: The diagnostic procedure was an endomyocardial biopsy. Microscopical examination revealed no amyloid or iron deposition, no eosinophils, and no granulomas or vasculitis. Thus, there was no evidence of sarcoidosis or the Churg-Strauss syndrome. There were, however, three fragments that were obtained at the time of the cardiac biopsy. Examination of one of the fragments showed relatively intact myocardium and focal areas of inflammation characterized by an interstitial mononuclear-cell infiltration, with loss of myocardial cells (), consistent with active lymphocytic myocarditis. Immunoperoxidase staining for UCHL-1 revealed that large numbers of the mononuclear cells were T lymphocytes.

Dr. Brooks: Because of his drug-refractory ventricular arrhythmias the patient received an implantable cardioverter-defibrillator system while he was in this hospital. In view of the finding of lymphocytic myocarditis, he received a course of prednisone and azathioprine. I have seen him regularly over the last five months. For the first four months he had no cardiac symptoms. Approximately three weeks ago weakness and ascites developed, and he was admitted to another hospital, where he was found to have acute renal- vein and portal-vein thrombosis and a myopathy due to the steroids. He has become dialysis-dependent and is doing poorly. A repeated echocardiographic examination showed very poor left ventricular function.

Dr. Scully: Dr. Venditti, do you have a final comment?

Dr. Venditti: I approached this case with the law of parsimony in mind. In my attempt to make a unifying diagnosis I considered the constellation of clinical findings most consistent with the Churg-Strauss syndrome, although the pathological findings do not support that diagnosis. Acute lymphocytic myocarditis is seen in many autoimmune disorders, but the accompanying clinical findings in this case are not consistent with the known entities.

Therefore, I agree with Dr. Merkel that this patient probably had an as yet undefined autoimmune disorder.

ANATOMICAL DIAGNOSIS Diffuse lymphocytic myocarditis (progressing to endomyocardial fibrosis).

ADDENDUM Dr. Fallon: The patient died six months after the endomyocardial biopsy was performed. An autopsy was performed elsewhere. The heart weighed 480 g, and all four chambers were dilated. The valves were normal. The ventricular myocardium appeared flabby and yellow- brown throughout. Microscopical examination of the left ventricular myocardium revealed circumferential, subendocardial fibrosis and an ongoing diffuse lymphocytic myocarditis (). There was no infiltrate of eosinophils, although the pathological findings were similar to those seen in endomyocardial fibrosis secondary to Loffler's myocarditis, suggesting a possible link to the history of eosinophilia.

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Experten + Krankenhäuser

Selbsthilfegruppen

Literatur

Internetquellen

Geschichte der Krankheit

Diskussion und Fragen,Anmerkungen

Die VT ist in V1 bis V6 immer monophasisch entweder ganz positiv oder ganz negativ ? Stimmt diese Aussage ??

Stichworte

Orginaltexte:

Device or drug? Study answers questions about treating dangerous heart irregularities

DALLAS, April 11 - A new rating scale may help doctors make better medical decisions about whether a person should receive a drug or a device to fix a potentially deadly heart irregularity, according to researchers reporting in today's Circulation: Journal of the American Heart Association.

Ventricular tachycardia (VT) is a rapid heartbeat that affects the lower chambers of the heart. Ventricular fibrillation (VF) is a quivering of the heart muscle. Both conditions reduce the heart's ability to pump blood and can result in collapse, cardiac arrest and sudden death, unless prompt medical attention is given.

Robert Sheldon, M.D., Ph.D., professor of medicine at the University of Calgary, Alberta, Canada, who headed the study, says that implantable defibrillators (ICDs) are better than medication for the oldest and sickest patients with VT or VF. Medication may be a better choice for those who are younger.

Implantable defibrillators are placed underneath the skin near the collarbone. They are programmed to react when a patient's "danger" level occurs and deliver an electric shock to the heart, speeding up the heart rate and stopping the arrhythmia.

The "gold standard" for drug treatment of VT/VF is amiodarone, says Sheldon. The medication changes the chemistry that creates electrical currents in the heart and prevents many recurrences of VT or VF. However, the drug can have serious side effects, including scarring of the lungs or liver, disruption of thyroid function and tremors. This is one reason why implantable defibrillators are being studied so intensely.

Compared to medication, ICDs reduced the death rate by half among individuals with VT or VF if they were 70 years or older, had poor left ventricular function (weaker contractions) and were bedridden or easily winded.

"These findings were quite unexpected," Sheldon says. "Not only do they suggest that older, sicker patients are most likely to benefit from receiving an ICD as first-line therapy, but they suggest that younger, healthier patients with VT/VF -- which include a larger segment of the population -- would have better treatment results with medication. If confirmed, these findings may have broad implications for the provision of therapy for patients with VT/VF."

Three large international studies found that implantable defibrillators reduced the death rate by 30 percent among VT/VF patients in general, compared to amiodarone. Patients with ICDs experienced improvement of quality of life provided they only need a few interventions from the device. Once patients needed shocks from their defibrillators, a sensation described by one of Sheldon's patients as "like being kicked by a mule," they may become dissatisfied with the devices.

In the latest study of 659 patients, the researchers again compared the two treatments with roughly half of the patients receiving the drug, and the other half receiving an implantable defibrillator. But this time, the subjects were divided into four groups based on risk factors such as being age 70 or older, having significantly decreased left ventricular function (ejection fraction), and having symptoms of heart failure such as a tendency to become short of breath while sitting or lying down.

The top quarter had the highest ratings on risk factors. This group that received an implantable defibrillator had 50 percent fewer deaths, compared to the high-risk group that received the medication. In those with at least two of the risk factors, the risk of dying in the year following treatment was about 14 percent in patients with the defibrillator and 30 percent in those receiving the drug.

Conversely, the youngest VT/VF patients with the least amount of left ventricular or functional impairment derived the least benefit from an implantable defibrillator compared to those who received the drug.

In an accompanying editorial, Arthur J. Moss, M.D., of the Heart Research Follow-Up Program at the University of Rochester Medical Center in Rochester, New York, wrote that because the sickest patients apparently benefit the most from defibrillators, the technique will become "increasingly targeted for patients with more severe heart disease."

Both Sheldon and Moss agree that further study is needed to confirm the findings.

Co-authors are Stuart Connolly, M.D.; Andrew Krahn, M.D.; Robin Roberts, M.Tech; Michael Gent, D.Sc. and Martin Gardner, M.D.

NR00-1130 (Circ/Sheldon)

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