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Ventricular tachyarrhythmias in a patient with Andersen-Tawil syndrome.

Pyo JY, Joh DH, Park JS, Lee SJ, Lee H, Kim W, Joung B - Korean Circ J (2013)

Bottom Line: Andersen-Tawill syndrome (ATS), a rare autosomal dominant disorder, is characterized by periodic paralysis, dysmorphic features and cardiac arrhythmias.She was diagnosed with ATS, based on dysmorphic features, ventricular arrhythmia, and periodic paralysis.This is the first case to be reported in Korea who experienced a fatal cardiac arrest and respiratory failure caused by ATS.

View Article: PubMed Central - PubMed

Affiliation: Division of Cardiology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.

ABSTRACT
Andersen-Tawill syndrome (ATS), a rare autosomal dominant disorder, is characterized by periodic paralysis, dysmorphic features and cardiac arrhythmias. This syndrome is caused by mutations of KCNJ2 gene, which encodes inward rectifying potassium channel. Here, we report an 18-year-old girl who was presented with life-threatening cardiac arrhythmia and acute respiratory distress. She was diagnosed with ATS, based on dysmorphic features, ventricular arrhythmia, and periodic paralysis. This is the first case to be reported in Korea who experienced a fatal cardiac arrest and respiratory failure caused by ATS.

No MeSH data available.


Related in: MedlinePlus

The 12 lead ECG without wide QRS tachycardia. This ECG shows prolonged QT interval with prominent U wave. And some premature ventricular contractions are presented at the end of T wave, consistent with R on T phenomenon. ECG: electrocardiogram.
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Figure 4: The 12 lead ECG without wide QRS tachycardia. This ECG shows prolonged QT interval with prominent U wave. And some premature ventricular contractions are presented at the end of T wave, consistent with R on T phenomenon. ECG: electrocardiogram.

Mentions: Her initial electrocardiogram (ECG) showed sinus rhythm with prolonged QT interval of 657 ms and frequent premature ventricular contractions (Fig. 2). Her initial laboratory tests showed Na+ 147 mEq/L, K+ 2.0 mEq/L and Cl- 123 mEq/L. Blood gas analysis revealed pH 6.85, pCO2 126 mm Hg, pO2 216 mm Hg, and HCO3- 22.5 mmol/L revealing severe respiratory acidosis, and she was intubated for acute respiratory difficulty. Other lab findings were not significant, including the thyroid function test. Until the serum potassium was corrected to above 2.5 mmol/L, several episodes of wide and narrow QRS tachycardia were observed (Fig. 3). Interestingly, the wide QRS tachycardia was sometimes changed to narrow QRS tachycardia with the same cycle length of 360 ms. Another ECG, taken after the cardioversion, prolonged QT interval with prominent U wave and right bundle branch block pattern premature ventricalaric contractions are seen at the end of the T wave (Fig. 4).


Ventricular tachyarrhythmias in a patient with Andersen-Tawil syndrome.

Pyo JY, Joh DH, Park JS, Lee SJ, Lee H, Kim W, Joung B - Korean Circ J (2013)

The 12 lead ECG without wide QRS tachycardia. This ECG shows prolonged QT interval with prominent U wave. And some premature ventricular contractions are presented at the end of T wave, consistent with R on T phenomenon. ECG: electrocardiogram.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3569570&req=5

Figure 4: The 12 lead ECG without wide QRS tachycardia. This ECG shows prolonged QT interval with prominent U wave. And some premature ventricular contractions are presented at the end of T wave, consistent with R on T phenomenon. ECG: electrocardiogram.
Mentions: Her initial electrocardiogram (ECG) showed sinus rhythm with prolonged QT interval of 657 ms and frequent premature ventricular contractions (Fig. 2). Her initial laboratory tests showed Na+ 147 mEq/L, K+ 2.0 mEq/L and Cl- 123 mEq/L. Blood gas analysis revealed pH 6.85, pCO2 126 mm Hg, pO2 216 mm Hg, and HCO3- 22.5 mmol/L revealing severe respiratory acidosis, and she was intubated for acute respiratory difficulty. Other lab findings were not significant, including the thyroid function test. Until the serum potassium was corrected to above 2.5 mmol/L, several episodes of wide and narrow QRS tachycardia were observed (Fig. 3). Interestingly, the wide QRS tachycardia was sometimes changed to narrow QRS tachycardia with the same cycle length of 360 ms. Another ECG, taken after the cardioversion, prolonged QT interval with prominent U wave and right bundle branch block pattern premature ventricalaric contractions are seen at the end of the T wave (Fig. 4).

Bottom Line: Andersen-Tawill syndrome (ATS), a rare autosomal dominant disorder, is characterized by periodic paralysis, dysmorphic features and cardiac arrhythmias.She was diagnosed with ATS, based on dysmorphic features, ventricular arrhythmia, and periodic paralysis.This is the first case to be reported in Korea who experienced a fatal cardiac arrest and respiratory failure caused by ATS.

View Article: PubMed Central - PubMed

Affiliation: Division of Cardiology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.

ABSTRACT
Andersen-Tawill syndrome (ATS), a rare autosomal dominant disorder, is characterized by periodic paralysis, dysmorphic features and cardiac arrhythmias. This syndrome is caused by mutations of KCNJ2 gene, which encodes inward rectifying potassium channel. Here, we report an 18-year-old girl who was presented with life-threatening cardiac arrhythmia and acute respiratory distress. She was diagnosed with ATS, based on dysmorphic features, ventricular arrhythmia, and periodic paralysis. This is the first case to be reported in Korea who experienced a fatal cardiac arrest and respiratory failure caused by ATS.

No MeSH data available.


Related in: MedlinePlus