Download Familial Trifascicular Block with Autosomal Dominant Inheritance

ECG & EP CASES
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Familial Trifascicular Block with
Autosomal Dominant Inheritance
Dae-Hee Shin, MD, PhD
Division of Cardiology, Gangneung Asan Hospital, University of Ulsan College of Medicine, Gangneung, Korea
ABSTRACT
A 29-year-old man presented to our hospital with dizziness and a history of recurrent syncopal episodes. His
mother and 2 uncles had undergone permanent pacemaker implantation several years ago. At presentation,
his heart rate was 49 bpm. Electrocardiography (ECG) indicated atrial flutter with right bundle branch block
(RBBB) and left anterior fascicular block (LAFB). Twenty-four hour Holter monitoring showed ventricular
pause up to 16 seconds during syncope. Radiofrequency catheter ablation was performed for atrial flutter. An
additional ECG indicated a trifascicular block (RBBB, LAFB, and first-degree AV block). He then underwent
permanent pacemaker implantation. ECG results of the patient's brother and sister also indicated a
trifascicular block, and the family pedigree showed autosomal dominant inheritance.
Key words: ■ atrial flutter ■ familial heart block ■ trifascicular block
Introduction
“
The term
Type I PFHB is characterized by a right bundle
”
trifascicular block
is confusing
branch block (RBBB), left anterior fascicular block
(LAFB), prolonged PR interval, or complete AV
1,2
because the involvement of the right bundle branch
block with broad QRS complexes.
and both fascicles of the left bundle branch would
Case
generally manifest as a complete heart block.
Moreover, the term trifascicular block is often
inaccurately applied for cases with a bifascicular
A 29-year-old man presented to our hospital
block and prolonged PR interval. Progressive
with a complaint of dizziness. He had experienced 2
familial heart block (PFHB) type I is an autosomal
episodes of syncope 7 and 25 years ago. Examination
dominant cardiac conduction disorder that may
of his family history indicated that his mother had
progress to a complete atrioventricular (AV) block.
undergone permanent pacemaker implantation at
the age of 51 years (Figure 1). In addition, 2 of his
uncles had also undergone permanent pacemaker
Received: August 3, 2012
Revision Received: September 3, 2012
Accepted: October 13, 2012
Correspondence: Dae-Hee Shin, MD, PhD, Assistant Professor, Ulsan
University College of Medicine, Department of Cardiology, Cardiovascular
Center, Gangneung Asan Hospital Gangneung, South Korea,
Tel: 82-33-610-3372, Fax: 82-10-6271-1291, E-mail: [email protected]
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The Official Journal of Korean Heart Rhythm Society
implantation. On examination, the patient's blood
pressure was 120/80 mmHg, heart rate was 49 bpm,
respiratory rate was 20 times per minute, and body
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Figure 1. The patient's mother underwent permanent pacemaker implantation at the age of 51.
℃
temperature was 36.8 . An initial electrocardiography
bifascicular block (RBBB and LAFB) and marked
(ECG) indicated atrial flutter with variable
first-degree AV block (Figure 5), and 24-hour
ventricular response and a bifascicular block (RBBB
Holter monitoring indicated an intermittent
and LAFB, Figure 2). On echocardiography, a mild
second-degree AV block. Following frequent
left ventricular systolic dysfunction (ejection
episodes of symptomatic high-degree AV block, he
fraction = 50%) with enlargement of both atria was
underwent permanent pacemaker implantation
observed. At admission, he exhibited a seizure-like
(DDD type). The ECG results of the patient's brother
motion with syncope, and showed a ventricular
and sister also indicated a trifascicular block
pause up to 16 seconds on 24-hour Holter
(Figure 6, 7). However, the ECG results of his
monitoring (Figure 3, 4). Subsequently, a temporary
sister's daughters were normal. A few months later,
pacemaker was inserted. Radiofrequency catheter
his brother underwent permanent pacemaker
ablation (RFCA) with bidirectional cavotricuspid
implantation for complete AV block at another
isthmus block was then performed for atrial flutter.
hospital. The patient's family pedigree showed an
Following RFCA, an additional ECG revealed a
autosomal dominant inheritance (Figure 8).
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Figure 2. Initial ECG showing atrial flutter with variable ventricular response and bifascicular block (RBBB and LAFB).
Figure 3. ECG monitor during syncope showing atrial flutter waves with two ventricualr escape thythms.
3
Discussion
“
”
hemiblock. The term trifascicular block is confusing,
as the involvement of the 3 fascicles in the ventricle
would generally manifest as a complete heart block.
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In 1968, Rosenbaum and his colleagues described
Therefore, the trifascicular block is often inaccurately
the trifascicular nature of the intraventricular
applied to cases with alternating RBBB and LBBB
conduction system and the trifascicular block and
or prolonged PR interval and bifascicular block.
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Figure 4. Twenty-four hour Holter monitor showing a ventricular pause up to 16 seconds during syncope.
In 1977, Brink and Torrington described a new
These ECG features can help differentiate type I
autosomal dominant familial heart disease
PFHB from progressive familial heart block type II
(progressive familial heart block), which primarily
(type II PFHB), wherein the onset of complete heart
1,2
affects the conduction tissue of the heart.
2
The
block is associated with narrow QRS complexes.
ECG features of type I PFHB are defined by the
Type I PFHB manifests symptomatically when
evidence of RBBB, LAFB, prolonged PR interval, or
complete heart block develops, and dyspnea,
2
syncopal episodes, or sudden death are noted.
complete heart block with broad QRS complexes.
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Figure 5. The ECG shows a bifascicular block (RBBB and LAFB) and marked first-degree AV block following RFCA (CTI
block for atrial flutter).
Figure 6. The ECG result of the patient's sister, 34-year-old shows a bifascicular block (RBBB and LAFB) and marked
first-degree AV block. However, her daughters' ECGs are still normal.
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Prompt implantation of a permanent pacemaker is
with type I PFHB. The use of a prophylactic
vital for the successful management of patients
pacemaker in these conditions is controversial.
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Figure 7. The ECG result of the patient's brother, 31-year-old shows a bifascicular block (RBBB and LAFB) and marked
first-degree AV block. He underwent permanent pacemaker implantation for complete AV block a few months after the
patient's procedure.
Pacemaker
Trifasicular block
or arrjythmia history
Pt.
Figure 8. The patient's family pedigree showing autosomal dominant inheritance for progressive familial heart block.
Follow-up visits and ECGs at 6-month intervals, at
degree of heart block, and an annual examination is
least, are recommended for patients with any
recommended for the patient's family members
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with normal ECGs. Although the global incidence
of type I PFHB is not known, this disease may not
only be confined to South Africa. A few reports
have indicated a familial tendency of bradyarrhythmia
6
in the Asian population as well.
References
1. Brink AJ, Torrington M. Progressive familial heart block-two
types. S Afr med J. 1977;52:53-59.
2. Van der Merwe P-L, Weymar HW, Torrinton M, Brink AJ.
Progressive familial heart block, part II: clinical and ECG
confirmation of progression-report on 4 cases. S Afr med J.
1986;70:356-357.
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The Official Journal of Korean Heart Rhythm Society
3. Rosenbaum MB, Elizari MV, Lázzari JO. The Hemiblocks.
Oldsmar, Florida: Tampa Tracings; 1970.
4. Brink PA, Moolman JC, Ferreira, A, Dejager T, Weymar HW,
Martell RW, Torrington M, Vandermerwe PL, Corfield, VA.
Genetic linkage studies of progressive familial heart block, a
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Progressive familial heart block (type I): a follow-up study after
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2003;33:1155-1160.