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Progressive familial heart block type I in a korean patient.

Lee CK, Shin DH, Jang JK, Jang KH, Kim EK, Cheong SS, Yoo SY - Korean Circ J (2011)

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Division of Cardiology, Gangneung Asan Hospital, University of Ulsan College of Medicine, Gangneung, Korea.

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.

No MeSH data available.


Related in: MedlinePlus

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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Figure 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.

Mentions: A 29-year-old man was referred to the emergency department with a complaint of abdominal pain and dizziness. He had experienced two episodes of syncope during exertion, one episode 7 years ago and the other 25 years ago. Family history revealed that his mother had received a permanent pacemaker implantation at 51 years of age (Fig. 2B). In addition, his two uncles had also received a permanent pacemaker implantation. On physical examination, his blood pressure was 120/80 mmHg, heart rate was 49 beats per minute, respiratory rate was 20 times per minute and temperature was 36.8℃. His ECG showed atrial flutter with variable ventricular response and bifascicular block (RBBB and LAFB) (Fig. 1A). The echocardiogram showed a mild left ventricular systolic dysfunction (ejection fraction=50%), enlarged left and right atrium. On the third day of hospitalization, the 24-hour Holter showed ventricular pause up to 16 seconds during syncope (Fig. 1B). At this time a temporary pacemaker was inserted. Radio frequency catheter ablation (RFCA) with bidirectional cavotricuspid isthmus block of atrial flutter was performed. After RFCA, the ECG revealed bifascicular block (RBBB and LAFB) and marked first-degree AV block (Fig. 1C) and the 24-hour Holter showed an intermittent second-degree AV block. He received a permanent pacemaker implantation with DDD type of pacemaker because of frequent episodes of symptomatic high-degree AV block (Fig. 1D).


Progressive familial heart block type I in a korean patient.

Lee CK, Shin DH, Jang JK, Jang KH, Kim EK, Cheong SS, Yoo SY - Korean Circ J (2011)

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.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3116107&req=5

Figure 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.
Mentions: A 29-year-old man was referred to the emergency department with a complaint of abdominal pain and dizziness. He had experienced two episodes of syncope during exertion, one episode 7 years ago and the other 25 years ago. Family history revealed that his mother had received a permanent pacemaker implantation at 51 years of age (Fig. 2B). In addition, his two uncles had also received a permanent pacemaker implantation. On physical examination, his blood pressure was 120/80 mmHg, heart rate was 49 beats per minute, respiratory rate was 20 times per minute and temperature was 36.8℃. His ECG showed atrial flutter with variable ventricular response and bifascicular block (RBBB and LAFB) (Fig. 1A). The echocardiogram showed a mild left ventricular systolic dysfunction (ejection fraction=50%), enlarged left and right atrium. On the third day of hospitalization, the 24-hour Holter showed ventricular pause up to 16 seconds during syncope (Fig. 1B). At this time a temporary pacemaker was inserted. Radio frequency catheter ablation (RFCA) with bidirectional cavotricuspid isthmus block of atrial flutter was performed. After RFCA, the ECG revealed bifascicular block (RBBB and LAFB) and marked first-degree AV block (Fig. 1C) and the 24-hour Holter showed an intermittent second-degree AV block. He received a permanent pacemaker implantation with DDD type of pacemaker because of frequent episodes of symptomatic high-degree AV block (Fig. 1D).

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Division of Cardiology, Gangneung Asan Hospital, University of Ulsan College of Medicine, Gangneung, Korea.

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.

No MeSH data available.


Related in: MedlinePlus