Effects of Atrial Tachypacing on Symptoms and Blood Pressure in Severe Orthostatic Hypotension

The treatment of severe orthostatic hypotension (OH) is currently unsatisfactory and usually includes various pharmaceuticals to expand the blood volume and promote peripheral vasoconstriction. This study examined the short- and intermediate-term effects of atrial tachypacing (ATP) in patients with severe OH. We implanted dual chamber pacemakers in five patients (mean age 64 ± 7 years; four men), presenting with drug refractory, recurrent syncope, and OH due to panautonomic failure with severe chronotropic incompetence and absence of rate acceleration upon assuming the upright posture. The blood pressure (BP) was measured in the supine and passive upright postures, during sinus rhythm, and during atrial pacing at 90, 100, and 110 ppm, at 1 week and at discharge and/or 3 months after pacemaker implantation. Alleviation of symptoms and a delay in the fall in upright BP were observed in a single patient at 1 week, while at discharge and/or 3 months, all patients were markedly improved. The mean fall in systolic/diastolic BP between supine and upright position decreased from 73 ± 17/46 ± 13 mmHg before, to 56 ± 27/41 ± 30 mmHg during ATP. Although these changes did not reach statistical significance, the time required for the fall in BP lengthened significantly from 2.1 ± 0.2 minutes during sinus rhythm to 9.3 ± 1.5 minutes during ATP (P < 0.001).
Conclusions: At discharge and/or 3 months of follow-up, ATP conferred beneficial effects on orthostatic BP and alleviated symptoms in patients with severe OH. The short-term effects of ATP did not reflect its longer-term effects in four of the five patients.     

Functional Communication Between Cardiac ATPSensitive K+ Channel and Na/K ATPase

K_ATP Channel and Na/K ATPase. Introduction: Functional interaction between K_ATP channel and Na/K ATPase was studied in single guinea pig ventricular myocytes because both membrane molecules are known to he involved in ischemic episodes. Methods and Results: K_ATP channel currents were recorded at 36°C by using whole cell, cell attached, inside-out, and open cell-attached modes of patch clamp techniques on enzymatically isolated ventricular myocytes. In the whole cell mode, ouabain (1 μM) reversibly inhibited the K_ATP currents induced by metabolic stress (ATP-free pipette solution and 1 mM NaCN), but not those activated by cromakalim (100 μM), a K_ATP channel opener. In the cell-attached mode, ouabain concentration dependently inhibited K_ATP, channel opening induced by metabolic suppression (5.5 μM 2-deoxyglucose and 1 mM CN). Half-inhibition concentration for ouabain was 21.0 ± 5.5 nM and the Hill coefficient was 0.8 ± 0.1 (n = 26). However, ouabain did not have an effect on the channel activity induced by cromakalim (100 μM). In the inside-out mode, ouabain applied to the internal side of membrane did not affect the channel. In the open cell-attached mode made by preincubation with streptolysin-0 (0.08 U/mL), the K_ATP channels were not activated by the metabolic inhibitors but were by reducing extracellular ATP concentrations, because subsarcolenimal ATP concentration could he controlled through tiny membrane holes. The channels thus activated were not suppressed by ouabain. Conclusion: The inhibition of Na/K ATPase by ouahain appeared to block the K_ATP channels by accumulating subsarcolemmal ATP caused by a decrease of the transition from ATP to ADP. In the presence of ischemic episodes, the administration of digitalis compounds may affect the opening of K_ATP channels, which is primarily protective against the development of irreversible myocardial damage.     

Familial occurrence of congenital bile duct cysts

Congenital bile duct cysts are now a well-documented anomaly of the biliary tree, and have become more common in Japan. Familial occurrence of congenital bile duct cysts, however, is extremely rare, with only six reported cases in the literature. We report a familial pattern of congenital bile duct cysts in a mother and her daughter. A 33-year-old female was admitted for evaluation of right upper quadrant abdominal pain and fever 6 days after an uneventful delivery of her second child. A com- puted tomography (CT) and ultrasound scan (US) revealed an obstructed biliary tract. Percutaneous transhepatic biliary drainage was then performed, and a cholangiogram revealed a Scholtz type B choledochocele without an anomalous connection of the pancreaticobiliary ducts. Endoscopic US demonstrated that the choledochocele was associated with a stone in the cyst. A pylorus-preserving pancreatoduodenal resection was performed, and a histological study revealed that the choledochocele was lined by biliary mucosa without evidence of malignancy. The newborn infant had an abdominal tumour. An US and CT revealed a congenital bile duct cyst. An operation was performed and the intraoperative cholangiogram showed an Alonso-Lej type I congenital bile duct cyst with an anomalous connection of the pancreaticobiliary ducts. Whether congenital bile duct cysts are hereditary remains to be elucidated.     

Catheter Ablation of Accessory Atrioventricular Connection between Right Atrial Appendage to Right Ventricle

Accessory AV Connection Between RAA and RV. A 24-year-old woman had experienced frequent attacks of orthodromic AV reciprocating tachycardia. The polarity of the delta waves suggested a right anterior or anterolateral accessory pathway. After ablation at the tricuspid annulus was unsuccessful, earliest retrograde atrial activation was recorded on the floor of the right atrial appendage, 2 cm above the tricuspid ring. Application of radiofrequency en-ergy at this site aholished accessory pathway conduction. This unusual accessory pathway, located between the floor of the right atrial appendage and the right ventricle, was amenable to radiofrequency catheter ablation from within the right atrial appendage.     

Albumin gene encoding free fatty acid and β-amyloid transporter is genetically associated with Alzheimer disease

Abstract  Alzheimer disease (AD), the major cause of dementia in the elderly, is characterized by β-amyloid deposition in senile plaques and hyperphosphorylated tau in neurofibrillary tangles. Since albumin, binding and transporting free fatty acids is also the major transporter of β-amyloid, we examined the association between the albumin ( ALB ) gene and the occurrence of late-onset AD (LOAD). We found that the allele distribution of the intron 4 microsatellite of the ALB gene showed a significant difference ( P  < 0.05) between LOAD ( n  = 285) and control group ( n  = 656). An allele with 11 CA repeat, termed (CA)₁₁ allele, was significantly predominant in the control group ( P  < 0.005), and the odds ratio carrying the (CA)₁₁ allele was 0.43 ( P  < 0.01, 95% CI = 0.24–0.79). Logistic regression indicated that this effect was independent of age and the ɛ4 dose of the apolipoprotein E gene ( P  < 0.01), while the ALB gene was supposed to be related to aging. Our results indicate that the ALB gene is genetically related with the occurrence of LOAD, supporting the link between fatty acid and β-amyloid transport in the development of LOAD.     

Treatment of Malignant Neurocardiogenic Vasovagal Syncope with a Rate Drop Algorithm in Dual Chamber Cardiac Pacing

A 29-year-old man with malignant vasovagal syncope presented with episodes of abrupt loss of consciousness associated with an aura, totaling more than 10 episodes over 3 months. Holter monitoring showed cardiac arrest with a duration of 15 seconds. Oral propranolol and disopyramide therapy failed to prevent the syncope. A dual chamber pacemaker with a rate drop response algorithm successfully prevented the syncope but not the aura. There may be multifactors involved in the mechanism of this syndrome. The patient has returned to a normal active life. This rate drop algorithm is an effective therapy for the prevention of syncope in malignant vasovagal syncope.     

A Case of Widely Split Double P Waves with Marked Intra-Atrial Conduction Delay

Widely Split Double P Wave. We report a 78-year-old man as the first documented case of double P waves separated by 400 msec on 12-lead ECG. These P waves had different polarities on lead V₁. The first P wave represented activation of the lateral wall of the right atrium, and the latter P wave represented activation of the nudial right atrium and the left atrium. Widely spaced double potentials were recorded craniocaudally along the line, presumably corresponding to the crista terminalis during sinus rhythm. For this to occur, conduction disturbance has to be present both in the upper and lower right atrium. Conduction disturbance in the upper right atrium would interrupt excitation from the sinus node to the medial wall, and conduction disturbance in the lower right atrium would interrupt excitation spreading from the lower lateral right atrium to the isthmus area where fragmented potentials were recorded. These multiple discrete lesions appear to constitute a unique electrical atriopathy in this patient.