![]() ![]() Congenital sick sinus syndrome without an apparent cause may be related to genetic mutations. So both sinoatrial node dysfunction and cardiac malformations caused by congenital dysplasia should be considered. Furthermore, this patient also has a congenital absence of the vagina and a reverse position of the upper abdominal viscera according to computerized tomography. To the best of our knowledge, herein we have for the first time reported a case of congenital bicuspid aortic valve accompanied by sinoatrial node dysfunction however, there is no direct or definite relationship between them. However, sinoatrial node dysfunction was not previously seen in patients with congenital bicuspid aortic valve. It increases the risk of thoracic aortic aneurysm and dissection, infective endocarditis, and sudden cardiac death. Her increased heart rate came from the junctional area during physical activity, which showed that the autonomic heart rate of the junctional area had sufficient emergency capacity.Ĭongenital bicuspid aortic valve, one of the most common congenital cardiac malformations, is associated with valvular abnormalities and aortopathies. Then the heart rate of junctional rhythm accelerated to 103 bpm to accommodate the body’s needs during exercise, paralleled with premature atrial contractions. ![]() Before exercise, the ECG showed escape beats and atrial capture beats ( Figure 4A). Then we monitored her ECG during exercise ( Figure 4). The results indicated that the autonomy of the junctional pacemaker is significantly higher than that of the sinoatrial node. It might be a sinus P wave, but it was still too slow to conduct to the ventricle compared with the junctional escape beat. We observed the appearance of atrial potential on the EB lead. ![]() A faster artificial atrial stimulation at 60 bpm was applied to capture the heart and to restrain the junctional pacemaker. Then esophageal electrophysiologic examination was performed for further diagnosis ( Figure 3). Echocardiography confirmed the presence of bicuspid aortic valve malformation and that the valve activity was good ( Figure 2).Įchocardiography of the Patient Showed the Presence of Bicuspid Aortic Valve Malformation. The disappearance of the sinus P wave might be caused by sinus arrest or sinus bradycardia. The fastest heart rate during the whole day was 63 bpm ( Figure 1D), and all the beats were accelerated junctional rhythm. As the daily activity changed, the heart rates were dynamically observed to become faster (51 bpm Figure 1B) and stable ( Figure 1C) in some periods of the whole day. Also tens of thousands of premature atrial conductions and escape beats were observed in the dynamic ECG. The ECG at night ( Figure 1A) showed slow sinus beats (arrows) of about 21 per minute during the whole day and junctional escape beats (stars) at a slightly faster frequency of about 29–33 per minute. (D) The Fastest Heart Rate as Junctional Rhythm (63 bpm) in the Dynamic ECG. (C) The Junctional Rhythm Became Stable (45 bpm) in Some Periods of the Dynamic ECG. (B) The Junctional Escape Beats Became Faster (51 bpm) Combined wth Atrial or Sinus Seizures in the Dynamic ECG. (A) The Lowest Heart Rate with Junctional Escape Beats (29–33 bpm) in the Dynamic ECG. ![]()
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