Permanent paralysis of the atrium in a patient with facioscapulohumeral muscular dystrophy

Baldwin, Brian J.; Talley, Robert C.; Johnson, Charles E.; Nutter, Donald O.

doi:10.1016/0002-9149(73)90337-8

Cited by 65 publications

(16 citation statements)

References 10 publications

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“…However, long before major studies were published on heart disturbances and FSHD [15, 16, 19], possible cardiac alterations in patients affected by this degenerative myopathy were discussed in anecdotal cases. Some authors [17, 18] described ‘atrial paralysis’ in a few FSHD subjects. Later, the clinical diagnosis of these patients was considered inconsistent [8, 15, 16] and this specific heart alteration was no more reported in subsequent studies on FSHD.…”

Section: Discussionmentioning

confidence: 99%

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Facioscapulohumeral Muscular Dystrophy and Occurrence of Heart Arrhythmia

Trevisan¹,

Pastorello²,

Armani³

et al. 2006

Eur Neurol

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Background: Subjects with facioscapulohumeral muscular dystrophy (FSHD) do not generally suffer from significant cardiac symptoms. Although with heterogeneous results, studies reported to date indicate that heart alterations unrelated to cardiomyopathy are possible in FSHD. Patients and Methods: We describe the findings of a multicenter investigation aimed at detecting cardiac abnormalities in 83 FSHD patients, 44 males and 39 females with a mean age of 47 years. All patients underwent clinical heart examination, 12-lead electrocardiography and 24-hour Holter monitoring; echocardiography was also performed on most patients. Results: Among the 83 patients, 62 with no cardiovascular risk factors were identified. Ten of them manifested clinical or subclinical cardiac involvement: 5 reported symptoms represented mostly by frequent palpitations secondary to supraventricular arrhythmia and another 5 exhibited electrocardiographic signs of short runs of supraventricular paroxysmal tachycardia. In the absence of cardiovascular risk factors, we found symptoms or signs of heart involvement of mainly arrhythmic origin in 10 of our 83 FSHD patients (12%). Conclusions: Considering our data and those available in the literature as a whole, arrhythmic alterations seem to be detected more frequently than expected in FSHD patients.

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Section: Discussionmentioning

confidence: 99%

“…At a time when DNA molecular diagnosis was not available, older anecdotal reports on patients apparently affected by FSHD described ‘atrial paralysis’ [17, 18]. Subsequently, however, major clinical studies on the issue yielded different, sometimes even controversial, results.…”

Section: Introductionmentioning

confidence: 99%

Facioscapulohumeral Muscular Dystrophy and Occurrence of Heart Arrhythmia

Trevisan¹,

Pastorello²,

Armani³

et al. 2006

Eur Neurol

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“…[1][2][3][4][5][6][7] There also may be an association with cardiomyopathy. [11][12][13] To our knowledge, the current case is the first detailed electrophysiological assessment of this condition.…”

Section: Discussionmentioning

confidence: 87%

Congenital Sick Sinus Syndrome With Atrial Inexcitability and Coronary Sinus Flutter

Varma

Helms

Benson

et al. 2011

Circ: Arrhythmia and Electrophysiology

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ongenital sick sinus syndrome has been described sporadically. The syndrome may comprise various components of sinus bradycardia, atrial fibrillation, and right atrial inexcitability. [1][2][3][4][5][6][7] In the current case, detailed electrophysiological evaluation was undertaken with electroanatomic mapping of the right atrium and coronary sinus (CS) and of associated supraventricular arrhythmias. CaseA 37-year-old asymptomatic male physician from Mexico without any previous medical problems was referred after routine physical examination for evaluation of atrial fibrillation with a slow ventricular rate. Arrhythmia duration was unknown. He stated that bradycardia had been consistently noted on previous clinical examinations and had been attributed to competitive long-distance running since childhood. Heart rates in the "40s" had been noted since age 10 to 11 years when he had been informed of a "heart murmur." Subsequent echocardiographic examination had been normal. The patient denied any family history of sudden death or arrhythmias. He had no siblings and no children. His prior records (and those of his parents) were unavailable.Physical examination was normal except for a regular bradycardia of 45 beats/min. Twelve-lead ECG showed fixed R-R intervals. No clear P-wave activity was identified. However, there was small-amplitude baseline electric activity of debatable origin, interpreted variously by experienced electrocardiographers as standstill, artifact, or atrial fibrillation waves ( Figure 1A). A transthoracic echocardiogram showed normal ventricular volume and function (left ventricular end-diastolic diameter, 52 mm; left ventricle end-systolic diameter, 34 mm) and mild tricuspid regurgitation with estimated pulmonary artery systolic pressure of 40 mm Hg. Both atria were severely enlarged (left atrial volume index, 58 mL; right atrial area, 41 cm 2 ). Doppler echocardiographic examination of both tricuspid and mitral atrioventricular (AV) inflow showed complete absence of A waves, indicating lack of left and right atrial contractile activity (Figure 2). A transesophageal echocardiogram did not reveal any evidence of an intracardiac shunt. A Bruce protocol exercise treadmill test was stopped at 10 minutes and 26 s because of patient shortness of breath. Heart rate accelerated gradually to a maximum of 131 beats/min and always remained regular. Baseline atrial activity (if any) was not interpretable because of exercise artifact. Isolated premature ventricular contractions were observed during exercise. A 24-hour Holter monitor showed regular rhythm (mean heart rate, 47 beats/ min; range, 35-95 beats/min; maximum R-R interval, 1.8 s) with 56 ventricular bigeminal cycles. The only symptom during recording periods was an abnormal lateral chest sensation that corresponded with heart rates between 30 and 40 beats/min. The patient underwent an external cardioversion without appreciable change in heart rate (44 beats/min), although electrocardiographic intervals between QRS-T complexes became completely isoel...

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“…Whether there is CI in FSHMD or not has been controversially discussed during the past. There are reports which clearly demonstrated CI in FSHMD [1, 2, 3, 4, 5, 6, 9, 10, 11, 12, 13], whereas other reports found no CI in FSHMD [14, 15]. Concerning CI-positive early reports, some authors are skeptical about the neurological diagnosis and believe that these investigations describe cases with Emery-Dreifuss muscular dystrophy, scapuloperoneal syndromes with cardiomyopathy, or FSHMD with ischemic cardiac disease [8].…”

Section: Discussionmentioning

confidence: 99%

“…Manifestations of CI in FSHMD on ECG so far described were multifocal atrial contractions, permanent paralysis of both atria [3, 9, 10], bradycardia [1, 4, 5, 9, 12], sinus node dysfunction [3, 4], tall P waves [2, 3, 4], supraventricular arrhythmia [1], short PR interval [1], abnormal atrioventricular conduction with complete atrioventricular block [1, 4, 6], abnormal Q waves [12], intraventricular conduction delay [1, 4, 12], ventricular tachycardia [1], abnormal ST segment [4], high T waves [1, 4, 13], prolonged QT interval [5], increased R/S ratio in V1 [12], and hypertrophy signs [1]. Echocardiographic abnormalities so far described in patients with FSHMD were restricted right ventricular movement due to thoracic deformities, enlarged right cardiac cavities [2, 3, 12], and hypertrophic cardiomyopathy [5, 7].…”

Section: Discussionmentioning

confidence: 99%

Cardiac Involvement in Facioscapulohumeral Muscular Dystrophy

2005

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Cardiac involvement (CI) in form of myocardial thickening in a patient with genetically confirmed facioscapulohumeral muscular dystrophy (FSHMD) has not been reported. The patient is a 50-year-old male with a tandem repeat size of 17 and 14 kb in the D4Z4 locus on chromosome 4q35. The clinical cardiologic investigation was normal. Blood pressure was 150/90 mm Hg. Funduscopy, 24-hour ambulatory ECG, and 24-hour blood pressure monitoring were normal. ECG showed incomplete right bundle branch block, ST elevation in V2–V4, tall T waves in V3–V5, and hypertrophy signs. Echocardiography revealed left ventricular myocardial thickening of the posterior wall (11.7 mm) and the septum (15.5 mm). In conclusion, CI in genetically confirmed FSHMD may manifest not only as ECG abnormalities but also as left ventricular myocardial thickening.

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Permanent paralysis of the atrium in a patient with facioscapulohumeral muscular dystrophy

Cited by 65 publications

References 10 publications

Facioscapulohumeral Muscular Dystrophy and Occurrence of Heart Arrhythmia

Facioscapulohumeral Muscular Dystrophy and Occurrence of Heart Arrhythmia

Congenital Sick Sinus Syndrome With Atrial Inexcitability and Coronary Sinus Flutter

Cardiac Involvement in Facioscapulohumeral Muscular Dystrophy

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