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Diagnosis and treatment of sick sinus syndrome.

Sick sinus syndrome comprises a variety of conditions involving sinus node dysfunction and commonly affects elderly persons. While the syndrome can have many causes, it usually is idiopathic. Patients may experience syncope, pre-syncope, palpitations, or dizziness; however, they often are asymptomatic or have subtle or nonspecific symptoms. Sick sinus syndrome has multiple manifestations on electrocardiogram, including sinus bradycardia, sinus arrest, sinoatrial block, and alternating patterns of bradycardia and tachycardia (bradycardia-tachycardia syndrome). Diagnosis of sick sinus syndrome can be difficult because of its nonspecific symptoms and elusive findings on electrocardiogram or Holter monitor. The mainstay of treatment is atrial or dual-chamber pacemaker placement, which generally provides effective relief of symptoms and lowers the incidence of atrial fibrillation, thromboembolic events, heart failure, and mortality, compared with ventricular pacemakers.

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Advances in pacing for the patient with sick sinus syndrome.

Sick sinus syndrome, the significant clinical manifestation of progressive sinus node dysfunction, is the most frequent indication for the implantation of permanent pacing systems in the United States. Revolutionary advances in pacemaker hardware and programmability now allow a careful tailoring of device, mode, and program for the individual patient and underlying electrophysiologic abnormalities. Evidence indicates that appropriate mode selection in this group of patients not only ameliorates symptoms but may decrease the incidence of complications and help maintain an acceptable quality of life. Most available data is retrospective and uncontrolled, therefore controversy remains regarding mode recommendations. More conclusive data may be produced by clinical trials currently in progress. This article reviews the latest innovations and recommendations regarding permanent pacing for sick sinus syndrome.

Cardiac Pacing, Artificial↗

[Sick sinus syndrome].

Sick sinus syndrome is an association of sinus node dysfunction with bradycardia, pauses, sino-atrial block, chronotropic insufficiency and atrial arrhythmias. The diagnosis is essentially clinical and electrocardiographic. Prolonged ECG recordings allow a better assessment of the severity of the condition whereas electrophysiological investigations only provide complementary information which is rarely decisive in the therapeutic indications. Cardiac pacing is the treatment of choice for symptomatic patients: significant improvement in symptoms may be expected but there is no significant change in survival. Single chamber atrial stimulation or dual chamber pacing is preferable to single chamber ventricular pacing, especially with regards to the incidence of atrial fibrillation and the impact on quality of life.

Bradycardia↗

[Carotid sinus syndrome and sick sinus syndrome: 2 frequent and distinct indications for pacemaker implantation].

UNLABELLED: At present, patients affected by carotid sinus syndrome and, more general by speaking, by vasovagal syncopes, are being considered more and more for pacemaker implant. However, neither the real incidence of the carotid sinus syndrome or its relation with the sick sinus syndrome with which it is often associated or even confused is precisely known. Of a total of 298 consecutive pacemaker implants, 36 (12%) were executed for carotid sinus syndrome, 33 (11%) for sick sinus syndrome and 24 (8%) for both. The annual incidence was respectively of 40, 37 and 26 implants per year/million of inhabitants. Patients affected by carotid sinus syndrome, when compared with those affected by sick sinus syndrome, showed: a higher prevalence of syncopes (97 vs 42%) more syncopal episodes per patient (2.9 +/- 2 vs 1.8 +/- 0.9), a lower prevalence of associated cardiac diseases (53 vs 100%), cardiac enlargement (36 vs 88%), heart failure (6 vs 36%) and paroxysmal atrial fibrillation (0 vs 97%), a more frequent indication to VVI pacing (75 vs 3%). Generally, when both syndromes were present, the patients showed intermediate characteristics. IN CONCLUSION: carotid sinus syndrome is a frequent indication of cardiac pacing as sick sinus syndrome; even if an association is present in about one third of patients, a distinction between carotid sinus syndrome and sick sinus syndrome is justified by theoretical and practical considerations.

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Pacing for carotid sinus syndrome and sick sinus syndrome.

The real incidence of pacemaker implants for carotid sinus syndrome (CSS) and the relation between CSS and sick sinus syndrome (SSS) is not precisely known. Patients who needed pacing therapy because of atrial bradyarrhythmias were investigated by means of carotid sinus massage, dynamic ECG, and invasive electrophysiological sinus node evaluation. Of 298 consecutive patients receiving a pacemaker implant, 36 (12%) had a severe cardioinhibitory carotid sinus reflex with reproducible spontaneous symptoms (CSS), 33 (11%) had sinus bradycardia less than 50 beats/min or an abnormal electrophysiological evaluation (SSS) and 24 (8%) had both (CSS + SSS). The annual incidence was 40, 37, and 26, respectively, implants per year/million of inhabitants (total incidence 325). Patients affected by CSS, if compared with those affected by SSS, showed: a higher prevalence of syncope (97% vs 42%); more syncopal episodes per patient (2.9 +/- 2 vs 1.8 +/- 0.9); a lower prevalence of associated cardiac diseases (53% vs 100%); cardiac enlargement (36% vs 88%); heart failure (6% vs 36%) and paroxysmal atrial fibrillation (0% vs 42%); and a more frequent indication for VVI pacing (75% vs 3%). In patients with CSS + SSS, intermediate characteristics were present. In conclusion, CSS is as frequent an indication to cardiac pacing as SSS; clinical differences justify a distinction between them, even if they are associated in 26% of cases.

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[Studies on the differential diagnosis of carotid sinus syndrome and sick sinus syndrome (author's transl)].

55 patients suffering from syncopes or dizziness were investigated by atrial pacing and carotid sinus reflex. In 17 pts. the only pathologic finding was a prolonged sinus node recovery time, in 8 pts. a pathologic carotid sinus reflex. In 7 pts. both pathologic findings were present, a prolonged sinus node recovery time and a pathologic carotid sinus reflex. In 3 pts. suffering from atrial fibrillation in addition a pathologic carotid sinus reflex was found. In 20 pts. the findings were normal. It is concluded that sick sinus syndrome and carotid sinus syndrome are different diseases, which can be separated clearly.

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Treatment of sick sinus syndrome with permanent cardiac pacemaker. (Sick sinus syndrome and artificial pacemaker).

Twenty nine patients with sick sinus syndrome were treated from November, 1972 through July, 1976. Six cases had sinus bradycardia, 14 cases had sinoatrial block/sinus arrest and 9 cases had bradycardia-tachycardia syndrome. Twenty five patients received the implantation pacemaker with good result. There was found that an adequate pacing of the heart was effective for the control of the attack of tachyarrhythmia in the case with bradycardia-tachycardia syndrome.

Adolescent↗

[Pathophysiology and diagnosis of bradyarrhythmias in sick sinus syndrome].

The sick sinus syndrome (SSS) is a complex cardiac disturbance involving impulse formation and conduction. Anatomy and physiology of the sinus node and atrial conduction are reviewed and the pathophysiology of bradycardia and tachycardia in the SSS is discussed. Finally, a practical approach to diagnosis is outlined.

Action Potentials↗

Characterisation of heart rate response to exercise in the sick sinus syndrome.

Patients with sick sinus syndrome have abnormalities of the sinoatrial node. We have measured the heart rate response to exercise in 7 patients with sick sinus syndrome without significant associated heart disease (group A) mean age 53.4 years, and compared this with 7 'normal' patients who were age-matched to within 5 years (group B), and 7 younger, well-trained subjects (group C). All underwent maximal treadmill exercise. Although maximum oxygen consumption (VO2max), 1/min per kg, in group A was not significantly different from group B (23.8 +/- 4.7 vs 19.9 +/- 0.8, mean +/- SE) maximum heart rate, beats/min, in group A was significantly lower than in group B (124 +/- 8.9 vs 163 +/- 3.7, P less than 0.001). At the end of 3 minutes of Bruce Stage I exercise, group A patients had a heart rate less than 130/minute (95% confidence level), whereas group B patients had heart rates greater than 134/minute. VO2 was plotted against heart rate (HR). Patients in group A had a significantly lower slope (deltaHR 5.20 +/- 0.33/delta1 ml VO2/kg per min, P less than 0.001). There was no significant difference in the slopes between groups A and C. On exercise patients with sick sinus syndrome have a normal VO2, but a reduced heart rate response as compared with age-matched normal patients. This abnormal heart rate response to the physiological stimulus of exercise may be of help in the evaluation of patients with sick sinus syndrome who do not have significant underlying heart disease.

Arrhythmia, Sinus↗

A case of CREST syndrome associated with sick sinus syndrome.

Cardiac manifestations of the CREST syndrome or limited systemic sclerosis (ISSc) are very rare. We report a case of CREST syndrome associated with sick sinus syndrome. Histopathology of cardiac muscle revealed fibrotic changes, suggesting that such changes may be pathogenetically related to CREST syndrome.

CREST Syndrome↗

Pacemaker treatment of sick sinus syndrome in children.

The sick sinus syndrome is being recognized with increasing frequency in children. Although it is sometimes benign, it can be serious or have fatal consequences. Fifty-one patients (mean age 10.5 years) underwent permanent cardiac pacing for sick sinus syndrome. Twenty patients had epicardial ventricular pacing and 12 had an epicardial atrial implant. Seven had endocardial atrial pacing, six epicardial atrioventricular (AV) sequential pacing, four epicardial universal pacing and two endocardial universal pacing. Of the 49 symptomatic patients, 45 had relief of symptoms. Eleven of 18 patients with associated tachyarrhythmias had amelioration of their tachycardia. There were no early but two late deaths unrelated to the pacemakers. Seven patients during a mean follow-up period of 26 months required reoperation for pacing lead or sensing problems. Permanent pacing for sick sinus syndrome in children is a safe and symptomatically effective procedure.

Adolescent↗

Sick sinus syndrome: mechanisms and management.

Sick sinus syndrome has become a frequent and challenging diagnosis for the practicing physician (Fig. 6). Increased longevity will create even more patients in need of effective therapy. Multiple etiologies have been described, but the common final pathway in most instances appears to be chronic fibrosis of the sinus node. The multifaceted features of the syndrome have delayed an organized diagnostic approach until recently. Noninvasive diagnosis often suffices, particularly with the availability of Holter monitoring. Invasive studies help to confirm sinus node dysfunction and possible associated conduction defects in these patients. The prognosis is variable and at times unpredictable. Nonetheless, it is best to establish the diagnosis promptly and to institute appropriate therapy. Cardiac pacing has had a particularly beneficial impact on the long-term management of the sick sinus syndrome.

Cardiac Pacing, Artificial↗

An elderly man with Klinefelter syndrome associated with hypertrophic cardiomyopathy, sick sinus syndrome, and coronary arteriovenous fistula.

Hypertrophic cardiomyopathy (HCM) is a rare cardiac complication in patients with Klinefelter syndrome. We report the case of a 67-year-old Japanese man with Klinefelter syndrome, HCM, sick sinus syndrome, and coronary arteriovenous fistula, in whom the 47XXY/46XY mosaic pattern was revealed by chromosomal study. Echocardiography revealed HCM with an interventricular septum thickness of 17 mm and a left ventricular posterior wall thickness of 10 mm. Sick sinus syndrome type III was diagnosed by paroxysmal atrial fibrillation (longest sinus arrest 9.0 sec) on 24-h Holter ECG recording. Coronary arteriovenous fistula was detected from the left anterior descending artery to the right ventricle by coronary arteriography. To our knowledge, this is the first case report of Klinefelter syndrome with HCM. As there have been a few reports of patients with Klinefelter syndrome in association with skeletal muscular diseases such as Becker-type muscular dystrophy or myotonic dystrophy, the gene mutation that causes Klinefelter syndrome may occur in the cardiac muscle. HCM may represent another variable expression of this chromosomal abnormality.

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Baroreflex sensitivity measured by the phenylephrine pressor test in patients with carotid sinus and sick sinus syndromes.

Baroreflex sensitivity was measured by the heart rate response to the transient rise in blood pressure induced by phenylephrine, in 11 patients with carotid sinus syndrome, 6 patients with sick sinus syndrome and nine age and blood pressure matched controls. Patients with carotid sinus syndrome were divided into those with a hypersensitive carotid sinus reflex manifest as sinus arrest (n = 9) and those with a reflex manifest as atrioventricular block (n = 2). The mean gain of the baroreflex sensitivity was significantly increased (p less than 0.001) in patients with carotid syndrome manifest as sinus arrest (12.7 +/- 5.1 ms . mmg-1) compared to that in patients with sick sinus syndrome (3.8 +/- 2.6 ms . mmHg-1) and controls (4.2 +/- 2.1 ms . mmHg-1). Patients with carotid sinus syndrome manifest as atrioventricular block did not have increased baroreflex sensitivity measured by their sinus node response (2.1 +/- 0.5 ms . mmHg-1). There was significant correlation (p less than 0.001) between the corrected carotid sinus inhibitory response to carotid sinus massage and the measurement of baroreflex sensitivity in the carotid sinus syndrome patients. Patients with carotid sinus syndrome manifest as reflex sinus arrest have an increased gain in baroreflex sensitivity for their age, which is not seen in patients with symptomatic sinoatrial disease. This study provides further evidence of a different mechanism of bradycardiac symptoms in patients with carotid sinus and sick sinus syndromes.

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