A CASE REPORT OF SYMPTOMATIC COMPLETE HEART BLOCK DIAGNOSED AS KEARNS–SAYRE SYNDROME(KSS)

Kearns-Sayre syndrome (KSS) is a rare syndrome characterized by the triad of progressive external ophthalmoplegia, pigmentary retinopathy and cardiac conduction system disturbances; it is a mitochondrial encephalomyopathy with which usually presents before the patient reaches the age of 20. Here we present a case report of KSS who presented with symptomatic complete heart block.

Kearns-sayre Syndrome Treated with Permanent Pacemaker Insertion for Complete Atrioventricular Block / 고신대학교의과대학학술지

Kearns-Sayre syndrome (KSS) is a rare multisystem mitochondrial disorder associated with progressive external ophthalmoplegia, atypical pigmentary degeneration of the retina, and complete heart block. KSS can lead to a risk of sudden death because of the potential progression of conduction abnormalities such as right or left bundle branch block or complete atrioventricular (AV) block. Here we describe the case of a KSS patient with type I diabetes who experienced syncope in the presence of complete AV block, confirmed by muscular biopsy.

A Typical Case of L-Transposition of the Great Arteries Initially Presented as Complete Atrioventricular Block in Middle-Aged Man

L-transposition of the great arteries (L-TGA) is a rare congenital anomaly and could cause complete atrioventricular (AV) block at relatively younger age. We present a case of 43-year-old male who complained of dizziness due to complete AV block. We confirmed L-TGA using transthoracic echocardiography and cardiac computed tomography. Permanent pacemaker was inserted without complications. No invasive treatment including corrective surgery was performed because patient's cardiac function was almost normal and the symptom was completely resolved after pacemaker insertion.

A Case of Quadricuspid Aortic Valve Complicated by Infectious Endocarditis / 日本心臓血管外科学会雑誌

We report a case of quadricuspid aortic valve concomitant with infective endocarditis. A 73-year-old woman was admitted to our hospital because of general fatigue, loss of body weight and high fever. Transthoracic echocardiography showed moderate aortic regurgitation and left ventricle-right atrium fistulae with vegetation. Infectious endocarditis was diagnosed. Since her fever and hemolytic anemia were not controlled by antibiotics, we operated and the aortic valve had four cusps and there were vegetations on the aortic valve and left ventricle outflow tract. Perforation of the membranous septum was observed. Complete debridement and aortic valve replacement with patch repair of a left ventricle-right atrium (LV-RA) fistula was performed. Although she needed a permanent pacemaker due to complete AV block, her postoperative course was uneventful.

Pacemaker malfunction related to insulation break according to position change in a patient with complete AV block / 대한내과학회지

A 53 year-old woman presented with intermittent dizziness and palpitation. She had received VVI type pacemaker due to complete AV block in 1990, and exchanged by VVIR type pacemaker thirteen years later. 1 year later, she suffered intermittent dizziness and palpitation in erect position, not in supine position. Intermittent pacing failure and sensing failure was observed in pacemaker test, especially in erect position, not in supine position. Pacing threshold was increased in erect position, but lead impedance was not changed. Insulation break was observed in bipolar lead by fluoroscope. Initially we tried a new lead implantation by cephalic access to prevent lead related complication, but failed. So, we implanted a new bipolar lead by subclavian access. Finally, she was treated by a new bipolar lead implantation.

Two Neonatal Cases of Pacemaker Implantation in Congenital Complete Atrioventricular Block

Congenital complete atrioventricular heart block(CCAVB) is a rare disease of the newborn that carries significant mortality and has a heterogenous etiology. It may occur as a result of the presence of maternal autoantibodies that are transferred to the fetus and affect the fetal heart or be associated with a congenital structural abnormality of the heart. Infants with CCAVB are at risk of diminished cardiac output and the subsequent development of congestive heart failure. We report two cases of CCAVB in newborns treated with pacemaker implantation after birth. The first case revealed CCAVB with patent ductus arteriosus and anti- Ro(SS-A) antibody in both of the mother and the newborn. The second case was accompanied with mitral regurgitation and tricuspid regurgitation, but anti-Ro(SS-A) antibody was absent in both of the mother and the newborn. Pacemaker implantation was performed for both cases and the result was favorable. We concluded that our experience supports that the pacemaker implantation in the newborn is feasible and beneficial in the treatment of CCAVB.

A Case of Ruptured Aneurysm of the Sinus of Valsalva into the Left Ventricle with Complete AV Block in Behcet's Syndrome

Behcet's disease is an inflammatory condition of multiple organ systems in witch recurrent oral and genital ulcers are the most typical signs. Less common clinical features include cerebral vasculitis, arterial aneurysm, deep vein phlebitis, aseptic meningitis, and discrete bowel ulcers. The most serious complication of Behcet's disease is arterial involvement especially ruptured arterial aneurysm. The aneurysm of sinus of Valsalva in Behcet's disease is a rare condition. There is no report about it in Korea. We report a case of a aneurysm of the sinus of valsalva with Behcet's disease. The patient had complete AV block and a right coronary sinus of Valsalva aneurysm which ruptured into the left ventricle. The diagnosis was made with transesophageal echocardiography. The patient was implanted with permanent pacemaker for relief of congestive heart failure due to complete atrioventricular (AV) block. He discharged and he is still follow-up in outpatient clinic.

Permanent pacemaker implantation via coronary sinus

We report a case of successful ventricular pacing via the coronary sinus in a 34 year-old female patient admitted because of repetitive dizziness and syncope. She had rheumatic valvular disease with mitral valve replacement 14 years earlyer. and the mitral, aortic and tricuspid valves were subsequently replaced with prosthetic mechanical valves 4 years ago. Two years after the triple valve replacement, complete AV block developed with the symptoms of dizziness and syncope. A permanent pacemaker was implanted epicardially. Six months later the epicardial lead was replaced because of increased pacing threshold. A year later the epicardial lead had to be replaced because of increased threshold and capture failure to pace. To avoid further thoracotomy, a 'Medtronic 2188' electrode was implanted in the posterior left ventricular vein via the coronary sinus. Pacing threshold was 1.2 volt/0.4 msec. Five days later, the pacing threshold increased to 3.0 volt/0.4 msec. Prednisolone had been given for 10 months. The new system has been functioning well and the pacing threshold was 1.0 volt/0.4 msec at 11 months after implantation. Ventricular pacing via the coronary sinus can be an alternative to the epicardial pacemaker system in patient whose tricuspid valve have been replaced with mechanical prosthetic valve.

Permanent pacemaker implantation via coronary sinus

We report a case of successful ventricular pacing via the coronary sinus in a 34 year-old female patient admitted because of repetitive dizziness and syncope. She had rheumatic valvular disease with mitral valve replacement 14 years earlyer. and the mitral, aortic and tricuspid valves were subsequently replaced with prosthetic mechanical valves 4 years ago. Two years after the triple valve replacement, complete AV block developed with the symptoms of dizziness and syncope. A permanent pacemaker was implanted epicardially. Six months later the epicardial lead was replaced because of increased pacing threshold. A year later the epicardial lead had to be replaced because of increased threshold and capture failure to pace. To avoid further thoracotomy, a 'Medtronic 2188' electrode was implanted in the posterior left ventricular vein via the coronary sinus. Pacing threshold was 1.2 volt/0.4 msec. Five days later, the pacing threshold increased to 3.0 volt/0.4 msec. Prednisolone had been given for 10 months. The new system has been functioning well and the pacing threshold was 1.0 volt/0.4 msec at 11 months after implantation. Ventricular pacing via the coronary sinus can be an alternative to the epicardial pacemaker system in patient whose tricuspid valve have been replaced with mechanical prosthetic valve.

Acute Myocarditis Complicated by Permanent Complete AV Block Associated with Salmonella Group B Gastroenteritis

Acute infectious myocarditis in children can be caused by many pathogens, including bacteria, viruses, fungi and protozoa. Patients may be asymptomatic or may have 'flu-like symptoms' to life-theratening arrhythmias or sudden death. We herein report a case of acute myocarditis complicated by complets and permanent AV block, associated with Salmonella group B gastroenteritis in a previously healthy 5-year-old boy. Presenting symptoms were dyspnea, cyanosis, vomiting, and diarrhea. Electrocardiography on admission showed very slow ventricular escape rhythm at 37 beats/minute. He was treated with antibiotics, inotropic agents, ventilatory support, and transvenous temporary pacemaker. Ten days later, permanent dual chamber pacemaker was implanted. All three consecutive stool cultures done after admission grew Salmonella group B. His ventricular function recovered rapidly and completely and he became fully active 2 months after the onset. However, his conduction system was totally destroyed and at last follw up evaluation 2 years after the onset, he is still totally dependent on the pacemaker without any escape rhythm.

Estudo do comportamento autonômico dos ritmos de escape no bloqueio atrioventricular congênito / Study of the Autonomic Behavior of the Escape Rhythms in Complete Congenital Heart Block

PURPOSE--To study the autonomic behavior of the escape rhythm in congenital complete heart block (CCHB), using heart rate variability (HRV). METHODS--A group of 10 asymptomatic patients with CCHB and narrow QRS was studied, 7 females; mean age = 14 +/- 9 years. The following time domain indexes were analyzed from a 24 hour Holter monitoring. The mean of all RR intervals (NN); the standard deviation of the mean (CLV5); the mean of all standard deviations (SDNN); the percent of successive differences longer than 50 ms (pNN50); the shortest cycle (CC) and the longest cycle (CL). The results were compared with a control group, using the Wilcox test for statistical analysis. RESULTS--The results were: NN = 1016 +/- 276 ms in CHB and 725 +/- 121 ms in control (p < 0.01); CLV5 = 184 +/- 97 ms in CHB and 125 +/- 38 ms in control (p = NS); SDNN = 102 +/- 32 ms in CHB and 88 +/- 29 ms in control (p = NS); rMSSD = 113 +/- 69 ms in CHB and 78 +/- 28 ms in control (p = NS); pNN50 = 43 +/- 26 per cent in CHB and 33 +/- 12 per cent in control (p = NS); CC = 582 +/- 129 ms in CHB and 333 +/- 49 ms in control (p = 0.05). CONCLUSION--No statistical difference was noted by comparing HRV indexes in CHB with control subjects, showing that the autonomic behavior of the escape rhythm in CCHB is similar to the sinus node in asymptomatic patients. The differences in NN, CC and CL are probably related to intrinsic properties of each command

Diastolic Regurgitation of Atrioventricular Valves in Patients with Complete Atrioventricular Block

BACKGROUND: The presence of diastolic mitral regurgitation has been described in patients with complete atrioventricular(AV) block, aortic valve regurgitation, hypertrophic and restrictive cardiomyopathy, and in patients with long diastolic filling period in atrial fibrillation. However, because of rare incidence and difficulty in making diagnosis of this phenomenon, the frequency and hemodynamic effects of diastolic AV valve regurgitation(DAVVR) and relationship of electrocardiographic(ECG) parameters with DAVVR in patients with complete heart block have not been reported in Korea. METHODS: To evaluate the frequency, hemodynamic effects of DAVVR and relation of ECG parameters with DAVVR in patients with complete AV block, we studied 14 consecutive patients with complete AV block by means of two dimensional and Doppper echocardiography. RESULTS: The DAVVR was observed in all cases of complete AV block except 3 cases on temporary pacemaker. The peak velocy of diastolic mitral and tricuspid valve regurgitaton were 105+/-23cm/sec and 98+/-30cm/sec, respectively. The peak and mean pressure gradient of diastolic mitral regurgitation were 4.7+/-1.7mmHg and 3.1+/-1.5mmHg respectively, and the peak and mean pressure gradient of diastolic tricuspid regurgitation were 4.1+/-2.6mmHg and 2.7+/-2.1mmHg, respectively. There was regular interval between p-wave of ECG and onset of diastolic AV valve regurgitation, which was 215+/-12msec, Diastolic AV valve regurgitation disappeared immediately after recovery of complete AV block to sinus rhythm or insertion of DDD-type permanent pacemaker in all cases. CONCLUSION: In Conclusion, the DAVVR was observed in all cases of complete AV block except cases on temporary pacemaker insertion and its hemodynamic effect was mild. There was regular interval between p-wave of ECG and the onset of diastolic AV valve regurgitation.