Yoshifumi Toyama

Cardiac involvement in the Kugelberg-Welander syndrome

Two cases of the Kugelberg-Welander syndrome (juvenile form of progressive spinal muscular atrophy) associated with cardiomyopathy and cardiomegaly are presented. The first patient, a 24 year old man, had atrial flutter with complete atrioventricular (A-V) block due to A-H block. Echocardiography revealed an increase in the left atrial and right ventricular dimensions. The second patient was a 26 year old man whose electrocardiogram revealed an A-V junctional rhythm, deep Q wave in leads I, aVL and V5 to V6 and an RS pattern in lead V1. Histologic examination of the myocardium in Case 2 showed slight interstitial fibrosis. Review of previously reported cases shows that (1) the atrium, the ventricular myocardium and A-V conducting tissue may be involved, and (2) atrial arrhythmias, A-V conduction disturbances and congestive heart failure may occur in the Kugelberg-Welander syndrome.

Prinzmetal's variant angina associated with subarachnoid hemorrhage: A case report.

Prinzmetals variant of angina oc curred in a 48-year-old man who sus tained two attacks of subarachnoid hem orrhage within 10 days. The first anginal pain started at the same time that the second cerebrovascular accident devel oped, but subsequent anginal episodes were not accompanied by other symp toms or signs that indicated new devel opment of subarachnoid hemorrhage. Twelve days later, when nuchal rigidity was fairly improved, the episodes of chest pain ended. A vasospasm of the large coronary arteries—probably due to the derangement of the autonomic ner vous system caused by subarachnoid hemorrhage—was presumed to contrib ute to the occurrence of the variant an gina. Based on this case and on review of the literature, we propose that coronary arterial spasm is one of several causes of the cardiac changes seen in subarachnoid hemorrhage.

Paroxysmal hypertension in aortitis syndrome.

Three patients with aortitis syndrome ehibited paroxysmal hypertension which seemed to result from baroreceptor dysfunction. All of the patients had signs of active inflammation of aortitis syndrome and stenotic carotid and subclavian arteries. During the attacks, the blood pressure rose to at least 230 mm. Hg systolic and the heart rate exceeded 100. However, with prolonged administration of steroid hormones, the attacks ceased. In two patients with dilated thoracic aortas and aortic regurgitation, the attacks of paroxysmal hypertension occurred without apparent precipitating factors and were followed by anginal pain with marked ST depression. The sympathicotonic state resulting from the disturbance of the baroreceptors was considered to be responsible for the attacks. In another patient, the attacks occurred in the course of treatment with a steroid hormone and were provoked only by voluntary micturition. This post-micturition hypertension was presumed to be an expression of abnormal overshooting following a fall in blood pressure after voiding.

Sympathetically induced atrial tachycardia. Successful treatment by left stellate ganglion block

An unusual type of atrial tachycardia was observed in a 52-year-old woman. The tachycardia occurred only when the patient kept the left upper limb in the lateral and horizontal position. Propranolol, atropine sulfate, carotid sinus massage and ocular compression were not effective in terminating or preventing the arrhythmia. Left stellate ganglion block has stopped the tachycardia for at least two years. The atrial tachycardia in this patient was thought to be caused by mechanical stimulation of the left stellate ganglion resulting in pacemaker shifting, as has been demonstrated in the canine heart.

Echocardiographic Findings in Patients With Aortitis Syndrome

Echocardiography was performed in 18 patients with the aortitis syndrome and in 20 age-matched normal volunteers. The aortic root dimension, the aortic dimension at the level of the sinotubular ridge, the aortic arch dimension, the left ventricular internal dimension, the left atrial dimension, the interventricular septal thickness, and the left ventricular posterior wall thickness were measured. All measurements, except for the left atrial dimension, were significantly greater in patients with aortitis syndrome than in the control subjects. We concluded (1) that the patients with the aortitis syndrome may have an enlarged or narrowed aorta, a dilated left ventricle and left atrium, and a thickened interventricular septum and left ventricular posterior wall; (2) that the incidence and the degree of these abnormalities depend on the presence of complications such as aortic regurgitation and arterial hypertension; and (3) that M-mode as well a cross- sectional echocardiography plays an important role in the assessment of the aorta and heart in the aortitis syndrome.