Korean Circ J.  2011 May;41(5):276-279. 10.4070/kcj.2011.41.5.276.

Progressive Familial Heart Block Type I in a Korean Patient

• Lee CK
• Shin DH
• Jang JK
• Jang KH
• Kim EK
• Cheong SS
• Yoo SY

• KMID: 2297936
• DOI: http://doi.org/10.4070/kcj.2011.41.5.276

Abstract

A 29-year-old man was referred to the emergency department with a complaint of abdominal pain and dizziness. He had experienced two previous syncopal episodes. His family history revealed that his mother and his two uncles had received permanent pacemaker implantation. His initial heart rate was 49 beats per minute. The electrocardiography (ECG) showed atrial flutter and right bundle branch block (RBBB) with left anterior fascicular block (LAFB). On admission, 24-hour Holter showed ventricular pause up to 16 seconds during syncope. Radio frequency catheter ablation (RFCA) of atrial flutter was performed. The ECG revealed bifascicular block (RBBB and LAFB) and first-degree atrioventricular block. He received a permanent pacemaker implantation. His brother's and his sister's ECGs also showed trifascicular block and the pedigree showed autosomal dominant inheritance. This patient was diagnosed with a progressive familial heart block (PFHB) type I. This would be the first report of a PFHB type I case documented in Korea.

Keyword

Hereditary bundle branch system defect; Atrial flutter

MeSH Terms

Abdominal Pain
Adult
Atrial Flutter
Atrioventricular Block
Bundle-Branch Block
Catheter Ablation
Dizziness
Electrocardiography
Emergencies
Heart
Heart Block
Heart Rate
Humans
Korea
Mothers
Pedigree
Syncope
Wills
Heart Block

Figure

• 

  Fig. 1 Initial ECG shows atrial flutter with bifascicular block; RBBB and LAFB (A). Twenty four-hour Holter shows asystole up to 16 seconds (B). ECG after RFCA shows trifascicular block; first-degree atrioventricular block, RBBB and LAFB (C). The patient received a permanent pacemaker implantation (D). ECG: electrocardiography, RBBB: right bundle branch block, LAFB: left anterior fascicular block.

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  Fig. 2 The pedigree showed an autosomal dominant inheritance (A). The patient's mother received a permanent pacemaker implantation at the age of 51 years (B). His brother's and his sister's electrocardiography also showed trifascicular block (C and D). Pt.: patient.

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