Channelopathies




(1)
Pediatric Cardiology, Policlinico S.Orsola-Malpighi, Bologna, Italy

 





A419998_1_En_16_Fig1_HTML.gif


Fig. 16.1
Family with Brugada due to SCN5A mutation . All members are asymptomatic. (a) 9-year-old male. A saddleback pattern can be noted in V1 (Brugada type 2). Note also the first-degree AV block: PR is 215 ms, identical to the father (b) and similar to the brother (c). Regardless of the pattern in the right precordial leads, in young family members of Brugada syndrome, a first-degree AV block is highly suggestive of the disease. Also the left axis deviation should be considered an ancillary sign in Brugada patient


A419998_1_En_16_Fig2_HTML.gif


Fig. 16.2
(a) An 8-year-old girl admitted for pneumonia . There is a suggestive Brugada pattern. (b) During fever the right precordial leads are even more suggestive. The fever is known for its ability to unmask the Brugada pattern (type 1, coved). The genetic test was negative, as in more than 65% of people with Brugada pattern


A419998_1_En_16_Fig3_HTML.gif


Fig. 16.3
(a) An 11-year-old male asymptomatic with pre-sport participation ECG. The right precordial leads present an atypical RBBB . (b) We did the ECG of the father (first ECG in his life), and the result was impressive, with a didactic Brugada pattern type 1 (Coved, ST elevation with at least 2 mm of elevation in at least 1 right precordial lead). (c) Moving V1 and V2 in the upper intercostal space (up to the second), the Brugada pattern is even more clear


A419998_1_En_16_Fig4_HTML.gif


Fig. 16.4
(a) A 10-year-old asymptomatic male referred for Brugada pattern type 2 (saddleback ST elevation in right precordial leads). (b, c) Moving V1–V2 to the second intercostal space, the picture is that of the usual benign right conduction delay. All the family members have a normal ECG, and no genetic tests were performed


A419998_1_En_16_Fig5_HTML.gif


Fig. 16.5
(a) Standard right precordial leads of a 16-year-old male with profound sinus bradycardia and first-degree AV block. (b) Moving V1–V2 to the second intercostal space, a saddleback pattern in V2 can be noted. (c) Synopsis with another 11-year-old male, both are followed for bradycardia and long PR interval. In such cases, a red flag for Brugada or other channelopathies has to be raised


A419998_1_En_16_Fig6_HTML.gif


Fig. 16.6
A 22-year-old woman with Brugada syndrome and atrial fibrillation . As we said for bradycardia, unusual tachycardia must be seen with suspicion in children and young persons. AF in the young may be idiopathic, but channelopathies should be excluded


A419998_1_En_16_Fig7a_HTML.gifA419998_1_En_16_Fig7b_HTML.gif


Fig. 16.7
(a) A 12-year-old male. The QT interval corrected by the Bazett is 465 ms. The patient is a LQT1 carrier due to mutation of the potassium channel-related gene. The T wave in V5 has a wide base and is tall; this can be the characteristic ECG phenotype of LQT1 but also compatible with the normal pattern for the age. (b) Close-up. (c) A 20-year-old female with LQT1 syndrome. A J wave due to benign early repolarization (arrow) can be noted: so an early repolarization is not incompatible with the late repolarization. Both patients are on treatment with nadolol

Only gold members can continue reading. Log In or Register to continue

Stay updated, free articles. Join our Telegram channel

Dec 17, 2017 | Posted by in CARDIOLOGY | Comments Off on Channelopathies

Full access? Get Clinical Tree

Get Clinical Tree app for offline access