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Special Features: Carolyn A. Bondy for The Turner Syndrome Consensus Study Group Care of Girls and Women with Turner Syndrome: A Guideline of the Turner Syndrome Study Group J Clin Endocrinol Metab 2007; 92: 10-25 [Abstract] [Full text] [PDF]

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Electronic letters published:

Monitoring of electrocardiograms in Turner syndrome

Carolyn Bondy, Angela Lin, Melissa Loscalzo,   (19 December 2006)

E-Letter to the Editor: QTc prolongation in Ullrich-Turner syndrome deserves particular attention

Robert Dalla Pozza, Susanne Bechtold, Heinrich Netz, Hans-Peter Schwarz   (30 November 2006)

Monitoring of electrocardiograms in Turner syndrome 19 December 2006
Carolyn Bondy NICHD, NIH, Angela Lin, Melissa Loscalzo, Send letter to journal: Re: Monitoring of electrocardiograms in Turner syndrome bondyc{at}mail.nih.gov Carolyn Bondy, et al.	The Turner Syndrome Study Group that met in April 2006 drafted guidelines based, as much as possible, on peer-reviewed, published data (1). Before submission of the guidelines, only the paper documenting ECG abnormalities in adults (2) was published and referred to in the text. However, our Table 1 clearly includes an ECG in cardiologic screening for all girls and women with Turner syndrome (1). Now that two independent studies have shown ECG abnormalities in children with Turner syndrome (3, 4) it is appropriate to emphasize the need for monitoring the ECG in girls as well as adults. We certainly agree with the recommendation to avoid medications known to prolong the QT interval in patients with a prolonged QTc. However, it does not seem appropriate to suggest the modest QT prolongation seen in some Turner syndrome patients is equivalent to the long-QT syndrome. There is currently no evidence that the QTc prolongation in Turner syndrome contributes to arrhythmia or sudden death. References 1. Bondy CA, for The Turner Syndrome Consensus Study Group 2006 Care of Girls and Women with Turner Syndrome: A Guideline of the Turner Syndrome Study Group. J Clin Endocrinol Metab:jc.2006-1374 2. Bondy CA, Van PL, Bakalov VK, Sachdev V, Malone CA, Ho VB, Rosing DR 2006 Prolongation of the cardiac QTc interval in Turner syndrome. Medicine (Baltimore) 85:75-81 3. Dalla Pozza R, Bechtold S, Kaab S, Buckl M, Urschel S, Netz H, Schwarz HP 2006 QTc interval prolongation in children with Ulrich-Turner syndrome. Eur J Pediatr 165:831-837 4. Bondy CA, Ceniceros I, Van PL, Bakalov VK, Rosing DR 2006 Prolonged Rate-Corrected QT Interval and Other Electrocardiogram Abnormalities in Girls With Turner Syndrome. Pediatrics 118:e1220-1225
E-Letter to the Editor: QTc prolongation in Ullrich-Turner syndrome deserves particular attention 30 November 2006
Robert Dalla Pozza, Pediatric Cardiologist Department of Pediatric Cardiology, Ludwig-Maximilians-University, Munich, Germany, Susanne Bechtold, Heinrich Netz, Hans-Peter Schwarz Send letter to journal: Re: E-Letter to the Editor: QTc prolongation in Ullrich-Turner syndrome deserves particular attention Robert.DallaPozza{at}med.uni-muenchen.de Robert Dalla Pozza, et al.	We appreciate the excellent guidelines presented by Dr. Bondy on behalf of the Turner Syndrome Study Group (1). We’d like to add some further details regarding the cardiovascular system in Ullrich-Turner syndrome. As reported, patients with UTS show several ECG abnormalities such as right axis deviation or accelerated AV conduction. An important finding can be of the QTc interval prolongation, as a risk factor for ventricular arrhythmias and sudden death (2). Among children and adolescents in the US, the Long-QT syndrome is estimated to account for 3000-4000 deaths annually (3). Several drugs known to affect cardiac repolarization are contraindicated in the long-QT syndrome (4) and should be avoided in affected persons. Contrary to the guideline statement, prolongation of the QTc interval occurs in children with UTS as well as adult patients (5, 6). Patients with 45,X0 karyotype seem to be more affected than mosaics, suggesting a protective influence from the second sex chromosome. The gap between the QTc interval in UTS patients and healthy controls begins to widen when puberty is induced or occurs spontaneously. Consequently, we believe that an electrocardiogram should be performed to detect QTc prolongation in all UTS patients in childhood and also during puberty. When QTc prolongation is present, patients and the family should be informed of the condition, its implications and the risk associated with certain drugs, including several antibiotics and antiasthmatic medication. References 1. Bondy CA for the Turner Syndrome Study Group. 2006. Care of girls and women with Turner Syndrome: a guideline of the Turner Syndrome Study Group. J Clin Endocrin Metab, October 25, epub ahead of print 2. Schwartz PJ. 1997. The long QT syndrome. Curr Prob Cardiol 226:302-351 3. Vincent GM. 1998. The molecular genetic of long QT syndrome: genes causing fainting and sudden death. Annu Rev Med 49:263-274 4. Crouch MA, Limon L, Cassano AT. 2003. Clinical relevance and management of drug-related QT interval prolongation. Pharmacotherapy 23:881-908 5. Bondy CA, Ceniceros I, Van PL, Bakalov UK, Rosing DR. 2006. Prolonged rate-corrected QT interval and other electrocardiogram abnormalities in girls with Turner syndrome. Pediatrics 118:e1220-1225 6. Dalla Pozza R, Bechtold S, Kaab S, Buckl M, Urschel S, Netz H, Schwarz HP. 2006. QTc interval prolongation in children with Ullrich-Turner syndrome. Eur J Pediatr 165:831-837