Congenital Familial Nodal Rhythm

Bacos, James M.; Eagan, John T.; Orgain, Edward S.

doi:10.1161/01.cir.22.5.887

Cited by 33 publications

(9 citation statements)

References 7 publications

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“…14 = episodes of sinus arrest, intermittent second degree atrioventricular block (2: 1). families showed persistent atrial standstill, sinus bradycardia, atrial fibrillation, atrioventricular nodal rhythm with atrioventricular dissociation, or complete heart block (Bacos et al, 1960;Phair, 1963;Khorsandian et al, 1964;Gazes et al, 1965;Allensworth et al, 1969;Gault et al, 1972). Electrocardiograms of first degree relatives of unselected patients with complete atrioventricular block have been examined and conduction abnormalities found in 10 per cent (Morgans et al., 1974).…”

mentioning

confidence: 99%

Familial sinus node dysfunction with autosomal dominant inheritance.

Lehmann¹,

Klein²

1978

Heart

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mentioning

confidence: 99%

Familial sinus node dysfunction with autosomal dominant inheritance.

Lehmann¹,

Klein²

1978

Heart

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“…17 An AV nodal escape rhythm is characteristic of this condition (Online Mendelian Inheritance of Man database [OMIM] 163800). 29 The current patient had a robust escape and remained asymptomatic until incidental discovery. However, an association with syncope and sudden death indicates that the syndrome may not always be benign.…”

Section: Varma Et Al Sinus Node Dysfunction and Atrial Standstill E55mentioning

confidence: 72%

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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“…Bacos, Eagan, and Orgain (1960) reported the first series of familial nodal rhythm. No apparent cause was found in these patients.…”

Section: Caralis and Varghesementioning

confidence: 99%