Enlarged coronary-bronchial artery anastomosis in cystic bronchiectasis: A case report / 대한내과학회지

Anastomoses between a coronary artery and bronchial or mediastinal arteries have been described since the 19th century. Although coronary-to-bronchial artery communication has been well described, it remains an unusual finding. We report one adult case of abnormally enlarged two coronary-to-bronchial artery communications in a severe cystic bronchiectasis patient. In this case, the bronchial arteries were also supplied from the multiple systemic arteries (the internal mammary, subclavian, inferior phrenic and intercostal arteries) because of severe cystic bronchiectasis. We treated the patient by embolization of the bronchial artery with the abnormal anastomoses and then by surgical resection of the cystic bronchiectatic lung lesion. To date, the patient remains free of symptoms.

Cardiac Dysrhythmias, Cardiomyopathy and Muscular Dystrophy in Patients with Emery-Dreifuss Muscular Dystrophy and Limb-Girdle Muscular Dystrophy Type 1B

Emery-Dreifuss muscular dystrophy (EDMD) and limb-girdle muscular dystrophy type 1B (LGMD1B) are characterized by cardiac dysrhythmias, late-onset cardiomyopathy, slowly progressive skeletal myopathy and contractures of the neck, elbows and ankles. The causative mutation is either in the emerin gene (X-linked recessive EDMD) or lamin A/C gene (autosomal dominant EDMD2 or LGMD1B). We report three cases of EDMD, EDMD2 and LGMD1B. A 14-yr-old boy showed limitation of cervical flexion and contractures of both elbows and ankles. Sinus arrest with junctional escape beats was noted. He was diagnosed as X-linked recessive EDMD (MIM 310300). A 28-yr-old female showed severe wasting and weakness of humeroperoneal muscles. Marked limitation of cervical flexion and contractures of both elbows and ankles were noted. Varying degrees of AV block were noted. She was diagnosed as autosomal dominant EDMD2 (MIM 181350). A 41-yr-old female had contractures of both ankles and limb-girdle type muscular dystrophy. ECG revealed atrial tachycardia with high grade AV block. She was diagnosed as autosomal dominant LGMD1B (MIM 159001). Cardiac dysrhythmias in EDMD and LGMD1B include AV block, bradycardia, atrial tachycardia, atrial fibrillation, and atrial standstill, causing sudden death necessitating pacemaker implantation. Cardiologists should know about these unusual genetic diseases with conduction defects, especially in young adults.

A Case of Emery-Dreifuss Muscular Dystrophy by Emerin Gene Mutation

Emery-Dreifuss muscular dystrophy (EDMD) is a degenerative myopathy characterized by mild, slowly progressing weakness, muscle atrophy, and early contracture of the neck, ankle and elbow. Heart involvement becomes apparent during the teenage years and is characterized by cardiac conduction defects and the infiltration of the myocardium by fibrous and adipose tissues. Heart block can eventually lead to sudden death, and therefore, early treatment with a cardiac pacemaker may improve symptoms and be lifesaving in patients with heart block. We describe our experience of pacemaker implantation in a 14-year old boy with X-linked recessive EDMD and emerin gene mutation. His electrocardiogram findings showed junctional escape beats, and his clinical features, i.e., ECG, nerve conduction test, electromyography and muscle biopsy findings were compatible with EDMD. He was implanted with a VVI type permanent pacemaker following an electrophysiologic study.

The Role of Intraoperative Transesophageal Echocardiography

BACKGROUND AND OBJECTIVES: Intraoperative echocardiography (IOE), mostly done by transesophageal methods, provides an important means of accessing cardiac structure and function during cardiac and noncardiac surgery. The purpose of this study was to determine the role of IOE by identifying the frequency of the use of IOE during cardiac surgery, the rate of second pump operations determined by IOE findings, and the results of the operations. SUBJECTS AND METHODS: Sixty-five patients (93 lesions) underwent cardiac surgery with the concomitant IOE done by a cardiologist in a single institution between March 1997 and October 1998. The data was collected retrospectively by reviewing the hospital records and IOE videotapes. IOE was applied to 18.9% of total cardiac operations, primarily being used in MV surgery (48.4%). A pre-pump IOE was done in 2 cases and the post-pump procedure was done in the remainder. A second pump operation was performed in 4 cases (6.25%) and all of these were found to be successful after immediate re-operation. CONCLUSION: IOE was primarily used in valve operations, particularly in procedures involving the mitral valve. IOE appears to be useful in determining the immediate results following cardiac surgery and may contribute to determining the prognosis of the patient.

A Case of High Degree AV Block Treated by Implantation of Permanent Pacemaker in Emery-Dreifuss Muscular Dystrophy

Emery-Dreifuss muscular dystrophy is characterized by 1) early contractures of the elbows, Achilles tendons, and postcervical muscles, 2) slowly progressive muscle wasting and weakness with a humeroperoneal distribution in the early stages, and 3) cardiomyopathy with conduction defects and risk of sudden death. The inheritance is usually X-linked recessive but can be autosomal dominant and recessive. We report a case of 28-year old woman who presented with dizziness, palpitation, and progressive muscular weakness. Her ECG revealed high degree AV block and muscle biopsy demonstrated diffuse degenerative change consistent with Emery-Dreifuss muscular dystrophy. She was diagnosed as autosomal dominant Emery-Dreifuss muscular dystrophy by characteristic clinical features, and findings of ECG, nerve conduction test, electromyography and muscle biopsy findings. A VVI-type permanent pacemaker was implanted.

Heart Rate Variability in Patients with Neurocardiogenic Syncope or Presyncope

BACKGROUND AND OBJECTIVES: Neurocardiogenic syncope is believed to be caused by a transient imbalance of autonomic nervous system. Actually, there were significant differences in heart rate variability (HRV) indices during head-up tilt test between patients with neurocardiogenic syncope and normal controls. But there was no definite evidence for it during daily activity. So, we tried to evaluate HRV during daily activity with 24-hour ambulatory electrocardiography monitoring. MATERIALS AND METHODS: 27 patients with neurocardiogenic syncope or presyncope (mean age 45+/-3) and 25 normal volunteers (mean age 47+/-2) comparable for age and sex underwent 24-hour ambulatory electrocardiography. Head-up tilt test was used to diagnose neurocardiogenic syncope or presyncope in patients group. HRV was analysed over the whole 24 hours, using time and frequency domain parameters. Student's t-test was applied. ResultsThere were no significant differences in HRV measures between two groups, over 24-hour period and day-time and night-time period. But the hourly HRV measures showed a transient decrease of LF, LFnorm and LF/HF ratio in patients group compared to normal control group. CONCLUSIONS: These results indicate that patients with neurocardiogenic syncope or presyncope suffer from temporarily decreased sympathetic tone with normal parasympathetic tone. So, transient additive change of autonomic nervous tone may cause syncope or presyncope in these patients. (Korean Circulation J 2000;30 (6):716-723)

A Case of Tsutsugamushi Disease Presented with Granulomatous Pleural Effusion / 감염

Tsutsugamushi disease is one of the acute febrile diseases caused by Orientia tsutsugamushi that is transmitted to human by the bite of larval-stage trombiculid mite (chigger). The clinical illness is characterized by abrupt onset of fever, headache, rashes, myalgia and eschar. Chest radiologic findings show reticulonodular infiltration, cardiomegaly, lymphadenopathy and in a minority, pleural effusion. About pleural effusion, it is supposed to be caused by tsutsugamushi disease itself in most cases and no case has been reported that the pleural effusion confirmed by pleural biopsy and revealed granulomatous lesions. We experienced a case of tsutsugamushi disease with pleural effusion which was also confirmed to granulomatous lesion by pleural biopsy. So we report this case with a brief review of literatures.