Early diagnosis and β-blocker therapy for high-risk patients with LQTS are important for prevention of cardiac events during pregnancy and the postpartum period, and β-blocker therapy may be tolerated for babies in LQT-P cases.
Loss of consciousness (LOC) in long QT syndrome (LQTS) patients can be caused by torsade de pointes (TdP) or vasovagal syncope (VVS). On genetic testing and head-up tilt testing (HUTT), we diagnosed three young patients with both genotyped LQTS and autonomic dysregulation. According to grade of prolongation of QT interval and LOC status, syncope episodes in two patients were classified as due to VVS, while those of the other patient were due to TdP. We also diagnosed one patient with postural orthostatic tachycardia syndrome. Syncope in LQTS patients should not automatically be labeled TdP. If there is a possibility of VVS, HUTT should be performed, but careful observation is required because TdP cannot be completely ruled out.
The present method of analyzing QTc on Holter ECG, in which automatic and manual measurements are combined, is practical and may be useful for diagnosis and risk stratification of LQTS.
Background: Secondary hypertension is more common in children than in adults. Although the number of patients with renal artery stenosis is relatively small, renal artery stenosis is an important clinical condition because of the potential for a complete cure. Methods: We reviewed the clinical outcomes of patients with renovascular hypertension who underwent percutaneous transluminal angioplasty or surgery at a single center between 2005 and 2011. The hypertensive response was defined as (1) cured: normal blood pressure with no antihypertensive treatment, (2) improved: improved blood pressure with identical or reduced treatment, (3) unchanged: no change in blood pressure despite angiographic success, or (4) failed. Results: The first surgical or endovascular intervention was performed between 1.8 and 13 years of age. Nineteen renal angioplasty procedures and four surgeries were done. The outcomes were as follows: cured in one patient who underwent surgery, improved in 14 patients who underwent renal angioplasty and three who underwent surgery, unchanged in four patients who underwent renal angioplasty, and failed in one patient who underwent renal angioplasty. Conclusions: The results as a whole were satisfactory. The treatment of small children and patients with double renal arteries is difficult because of their small vessels.
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