We examined the clinical features, 12-leads ECG, Holter ECG, monitor ECG, and electrophysiologic study (EPS) in nine consecutive paroxysmal atrioventricular block (PAVB) patients treated in our hospital between 1995 and 2000. In some of them, parasympathetic nerve stimulating maneuvers, drug administration and head-up tilt test (HUT) were performed as provocative tests. EPS documented that the sites of AV block were within the His-Purkinje system (H-PSB) in five patients, proximal to the His bundle potential (AHB) in three patients. In the remaining one patient, the His bundle potential was not recorded. The main features of the patients with H-PSB were as follows: 1) often wide QRS complex with or without axis deviation; 2) variable degrees of AV conduction disturbance in a short period; 3) sinus tachycardia, and normal length and constant PQ intervals in a daytime being apt to precede PAVB; 4) rather long ventricular asystoles leading to abrupt syncope; 5) easy induction of the block by intravenousadministration of atropine. In the other hand, the features of the patients with AHB included: 1) narrow QRS; 2) progressively increasing or decreasing in PQ intervals preceding PAVB; 3) ventricular asystole lasting about 3 to 10 seconds; 4) the failure to induce PAVB by any probale provocative tests.
Although we treated all the patients with permanent pacemakers, there might have been other management method for AHB patients who had about 3-second ventricular asystole.

A 73-year-old man was admitted to the hospital on March 31, 2000 because of nonsustained ventricular tachycardia (VT). In 1983, he was diagnosed as having apical hypertrophic cardiomyopathy (APH). Electrocardiograms showed high amplitude of R waves and giant negative T waves (GNT), and left ventriculography (LVG) revealed spade like configuration. He stopped medication without leave six months after the diagnosis. The latest ECG showed a decrease in amplitude of R waves, no signs of GNT, ST elevation in precordial leads, and an increase in QRS duration. LVG demonstrated midventricular obstruction, apical aneurysm, and the intraventricular pressure gradient in systolic phase was 56 mm Hg. No stenotic lesion was observed in coronary arteriography. Early diastolic paradoxic flow from the apical chamber toward the outflow of the left ventricle was detected by pulsed Doppler echocardiography. VT was successfully treated with beta-blocker and amiodarone, but he needed hospital treatment again one month later because of congestive heart failure. During the follow-up of APH, the decrease in amplitude of R waves and disappeasance of GNT, ST elevation, and the increase in QRS duration in electrocardiograms, and the detection of early diastolic paradoxic flow by echocardiography could be the predictors of developing apical aneurysm and/or left ventricular dysfunction.

A19-year-old man was admitted to the hospital because of severe congestive heart failure on 7 April 2000. In the previous year his case had been diagnosed as Churg-Strauss syndrome (allergic granulomatous angiitis, AGA) with bronchial asthma and mononeuritis multiplex. Echocardiography revealed the dilatation of the left ventricle (LVDd 74 mm) and impaired left ventricular systolic function (LVEF 20%). On the 21st hospital day, the irregularity of peripheral branches of left and right coronary arteries was detected by coronary arteriography. Right ventricular endomyocardial biopsy yielded little fibrosis and no infiltration of eosinophil. Although all the laboratory tests showed lower activity of AGA, steroid pulse therapy was tried and the use of steroids was tapered at intervals of two weeks. Left ventricular function was slowly improved (LVDd 60 mm, LVEF 36%). He was discharged on foot on the 71st hospital day.