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.

Maternal congenital complete heart block in pregnancy: a rare case report

Complete heart block comprises complete absence of AV conduction - none of the supraventricular impulses are conducted to the ventricles. Perfusing rhythm is maintained by a junctional or ventricular escape rhythm. Typically, the patient will have severe bradycardia with independent atrial and ventricular rates. The incidence is 1 in 15,000 to 20,000 live births. Authors present the case report of a primigravida, aged 30 years who presented with amenorrhoea of 35 weeks. She was diagnosed as complete heart block by cardiologist. She had no history of syncopal attacks during childhood or antenatal period. She had a heart rate of 50-52 bpm. ECG showed ventricular escape rhythm with narrow QRS complexes. Echocardiography showed no structural defects. Her elective LSCS was done at 39 weeks after consultation with cardiologist. Patient underwent temporary pacemaker insertion just prior to surgery and she delivered a healthy female baby of weight 3 kg with Apgar 9/10 at 5 mins. She remained asymptomatic during the postoperative period and pacemaker was removed after 30 hours. She was discharged under satisfactory condition on day 6. Patient was counselled for follow up with cardiologist. This case lays emphasis on proper history taking, vitals monitoring, early recognition of undiagnosed cardiac disorders and team work for good maternal and fetal outcome.

Unmasking Brugada Syndrome with Oral Flecainide Provocation. A Case Series of Three Patients

The present case series discuss three patients who had brugada type 2/ type 3 like ECG pattern that was converted to type 1 pattern with oral flecanide challenge test. Brugada syndrome is associated with a high incidence of sudden cardiac death, typical ECG pattern being ST-segment elevation in the right precordial leads with T wave inversion. Pharmacological provocation should only be performed when the baseline ECG is not diagnostic of Brugada Syndrome. PR prolongation in the baseline ECG is also a contraindication because of the risk of inducing AV block. Drug challenge is performed under strict monitoring of BP and 12-lead ECG and facilities for cardio version and resuscitation are available. Atypical RBBB pattern/type 2/3 Brugada pattern on ECG in patients of syncope or family history of sudden cardiac arrest is commonly encountered by a cardiologist. This can be performed to provoke type 1 brugada pattern on ECG. Diagnosed cases of Brugada may be treated with ICD with proper indication if needed and thus prevent sudden cardiac death.

Mobitz Type 1 Second‐degree Atrioventricular Block in Anterior Wall Myocardial Infarction and Its Reversal with Successful PCI of Left Anterior Descending Artery – An Extremely Rare Association

Arrhythmias can complicate the course of patients with ST-elevation myocardial infarction. These arrhythmias can include both tachyarrhythmias and bradyarrhythmias. Tachyarrhythmias range from Ventricular premature complexes to life-threatening ventricular tachycardia/ fibrillation. Bradyarrhythmias range from sinus bradycardia to complete heart block. These arrhythmias have the ability to provoke hemodynamic consequences and increase mortality. Tachyarrhythmias are more common with Anterior wall myocardial infarction and bradyarrhythmias are more common with Inferior wall myocardial infarction. We report a case of Mobitz Type 1 (Wenkebach) second-degree atrioventricular block in a patient with Anterior wall myocardial infarction. Angiography showed a significant lesion in Left anterior descending artery, after the first septal and diagonal branch. After the successful percutaneous coronary intervention, this second degree AV block reverted to first degree AV block. To the best of our knowledge, there is no case report describing this association separately.

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.

Importance of atropine challenge test in the current era of electrophysiology.

Mobitz II block is misunderstood more than any other abnormality of rhythm or conduction”. The concept of 2:1 AV block remains poorly understood by many physicians even after so many years of advancement in the field of electrophysiology. It cannot be classified into type I or type II second-degree AV block because there is only one PR interval to examine before the blocked P wave A 46 year male admitted with chief complaints of effort intolerance and non anginal chest pain of fifteen days duration. His resting ECG revealed 2:1 conduction of P wave, before and after non conducted P wave PR interval was constant and of normal duration. So in order to define the site of block we performed the atropine challenge test as the patient was not able to walk. On administration of atropine ECG revealed worsening of AV block in a 3:1 to 4:1 conduction of P wave with narrow complex QRS. This finding suggesting the block is in bundle of his or branches. This patient underwent Electrophysiological study. Electro physiological tracings showed normal PR interval, QRS duration, with 2:1 AV block. The non conducted P wave was blocked at the level of distal His bundle. This case illustrated the importance of localisation of site of block in 2:1 AV block in order to manage the case appropriately. Both vagal manoeuvres and exercise can help in localising the site of block, which will be confirmed by electrophysiology study.

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.

Study of Hypothyroid Related Atrioventricular Block in Elderly Hypothyroid Patients and Assessment of Any Reversal After L-Thyroxin Therapy.

We intend to study the pattern of atrioventricular conduction blockage in hypothyroidism in elderly population and assessment of any reversal after Lthyroxin therapy. Aims : 1) To detect hypothyroid related AV block in elderly hypothyroid patients. 2) To supplement L-thyroxin for 6 weeksand check whether the AV conduction is restored or not. Settings and design : The proposed study is a hospital based case control study followed by interventional one. Hypothyroid elderly subjects attending OPD over a period of one year were considered for the purposes of observation. Age and gender matched controls were selected simultaneously. Methods and materials : 42 elderly (>60 years) hypothyroid patients were selected as case and 45euthyroid sage and gender matched subjects as control. ECG was done to asses the degree of AV block. Any degree of AV conduction block noted, was supplemented with L-thyroxin for 6 weeks and later resting ECG was done to assess whether the normal AV conduction was restored or not. Statistical analysis : Chi square test done between the case and control group and paired T test done before and after L-thyroxin supplementation. Results : Chi square test revealed that out of 42 cases, a significant proportion of 62% had increased P-R interval (P<.001), whereas 76% out of 45 control had normal P-R interval.After L-thyroxin supplementation in 69% of the cases, normal P-R interval was restored (P< .001, paired T test value 13.484). Conclusion : In hypothyroid patients AV block detected in resting ECG should be a matter of concern, because if the conduction block is reversed to normal sinus rhythm by thyroxin supplementation, unnecessary pacemaker implantation can be avoided.

Case of Thyrotoxic Periodic Paralysis Accompanied by Atrioventricular Block Associated with Hypokalemia and Hypophosphatemia

Thyrotoxic periodic paralysis (TPP) occurs in 2% of the asian patients with hyperthyroidism and is characterized by bilateral flaccid paralysis of the extremity, especially lower limbs. It is well-known that hypokalemia is usually accompanied by TPP. However, hypophosphatemia is usually mild and well neglected. Although paralysis is generally recovered without treatment, in some cases, patients with TPP may die due to cardiopulmonary complications, such as cardiac arrhythmia. Therefore, proper and rapid replacement of potassium is essential. But it should be acknowledged that replacement may cause a rebound. TPP is often unrecognized and over-treated in the emergency room due to its non-specific symptoms. This is why clinicians must be familiar with this disease and its diagnostic clues such as Echocardiography change and clinical features. This is a case report of a 29-year-old male presenting with TPP accompanied by hypokalemia, hypophosphatemia and second degree atrioventricular block, who showed rebound hyperkalemia and hyperphosphatemia after rapid replacement of electrolytes. EKG changed to the normal sinus rhythm in the end after the correction of the electrolytes.

Catheter cryoablation of atrio-ventricular nodal reentrant tachycardia. A clinical review / Crioablación con catéter de la taquicardia por reentrada intranodal. Una revisión clínica

Cryoablation is a new method in interventional cardiac electrophysiology for percutaneous catheter ablation of cardiac arrhythmias. Cryothermal mapping enables the functional assessment of a particular site before permanent ablation. In this way, the targeted tissue may be confirmed as safe for ablation. This is useful in high-risk ablation, for example, next to the His bundle or the compact AV node. In the last decade, several studies have been addressed to AV-nodal reentry tachycardia (AVNRT) cryoablation. Current experiences indicate that cryoablation for AV-nodal reentry tachycardia is effective and safe. However, its wide use seems to be somewhat limited by a slightly lower efficacy when compared to radiofrequency. Further studies evaluating long-term success of cryothermal ablation versus radiofrequency are warranted. However, for high-risk ablations, cryoenergy is very helpful and should be systematically used. This article is a review of acute and long-term effects of cryoablation in patients suffering of AV-nodal reentry tachycardia episodes.

La crioablación es un nuevo método en la electrofisiología cardiaca intervensionista para la ablación percutánea de las arritmias cardiacas. El mapeo criotérmico permite la evaluación funcional de un sitio en particular antes de la ablación permanente; de esta manera, el tejido blanco puede confirmarse como seguro para el procedimiento. Esto es útil en la ablación de alto riesgo, por ejemplo, cerca del haz de His o del nodo AV compacto. En la última década, varios estudios se han orientado a la crioablación para la taquicardia de reentrada del nodo AV (TRNAV). Las experiencias actuales indican que la crioablación de la taquicardia de reentrada del nodo AV es efectiva y segura. Sin embargo, la apertura para ampliar su uso está parcialmente limitada por su eficacia ligeramente menor al compararla con el empleo de la radiofrecuencia. Se justifican ensayos clínicos futuros con objeto de evaluar el éxito a largo plazo de la ablación criotérmica en comparación con la radiofrecuencia. Para las ablaciones de alto riesgo, la crioenergía es muy útil y debería ser usada sistemáticamente. Este artículo consiste en una revisión sobre los efectos inmediatos y a largo plazo de la crioablación en pacientes que presentan episodios de taquicardia por reentrada del nodo AV.

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.

A Case of Third Degree Complete Atrio-Ventricular Block During Catheterization of an Internal Jugular Vein for Hemodialysis / 대한신장학회잡지

Cardiac arrhythmias are a potential complication in the placement of central venous catheter for hemodialysis. These arrhythmias are typically benign and can be resolved by withdrawing the offending guide-wire or repositioning the catheter tip. We report a case of unusual arrhythmia, a complete 3rd degree atrioventricular block (3rd AVB). A 47-year-old man was admitted with clinical findings suggestive of end stage renal disease. His electrocardiography revealed a complete left bundle branch block. During the placement of the wire for a hemodialysis catheter via the right internal jugular vein, he developed 3rd AVB and hypotension. Despite the withdrawal of the wire, the hemodynamic instability and 3rd AVB had been sustained, leading to the insertion of a temporary pac emaker. On the 7th day after the insertion of the temporary pacemaker, the 3rd AVB was spontaneously resolved. To the best of our knowledge, a 3rd AVB related to the insertion of a hemodialysis catheter has not been reported in Korea.

A Case of Severe Hypokalemia Induced by Chronic Furosemide Abuse / 대한신장학회잡지

A 46-year-old woman was admitted to emergency room for muscle weakness and repiratory difficulty. She was taking furosemide (4 tablets per day : 160 mg/day) without physician's prescription. Her blood pressure was 90/50 mmHg and her heart rate 74/ min. Her laboratory finding showed a low serum potassium concentration (0.9 mmol/L), CK 368 IU/L, creatine 1.6 mg/dL. The result was rechecked and confirmed. Electrocardiography showed a dip in the ST segment, prolonged QTc, 1st degree AV block and 2 : 1 AV block. Echocardiography was normal. Abdominal CT scan show right lower pole calyceal stones without nephrocalcinosis. Treatment was initiated consisting of intravenous potassium chloride. Hypokalemia was overcome in 3 days. Kidney biopsy showed hypokalemic nephropathy - interstitial nephrosis, vacuolar change of tubule, proliferation of juxtaglomerular apparatus. Paralytic ileus, rebound pleural effusion, glucose intolerance, elevation of CK were associated findings. After correction of hypokalemia, her symptoms and electrocardiographic findings returned to normal.

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.

Two Cases of 2:1 Atrioventricular Block in Infants with Idiopathic Long QT Syndrome

Idiopathic long QT syndrome(LQTS) is an important cause of syncope, seizures, serious ventricular arrhythmias and sudden death in children. The abnormalities of AV conduction such as 2:1 AV block in children with LQTS is rarely reported. We reported two cases of 2:1 atrioventricualr block in infants with LQTS. Patient 1, a female newborn, revealed bradycardia(60 beats/min) and QTc of 0.65sec with 2:1 AV block at birth. She died of polymorphic ventricular tachycardia at 26days of age. Patient 2, a 2-month-old female, exhibited prolonged QT interval (QTc=0.54sec), 2:1 AV block, and intermittent Wenckebach conduction. After she received propranolol, sinus rhythm with 1:1 AV conduction and QTc of 0.5sec was seen. After a follow-up of 6 months, the infant is still doing well.

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.