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The introduction of the ventricular inhibited pulse generator with the feature of rate hysteresis has been associated with a variety of rhythm disturbances, some causing serious concern. This pulse generator has two different pacing rates: (1) the automatic rate, which is the interval between two successive paced beats (usually 860 msec or 70/min), and (2) the hysteresis interval, which results in a 1,000 msec delay between a sensed cardiac contraction and the next pacemaker discharge. The hysteresis interval after a sensed signal may result in long pauses that may predispose to the development of serious cardiac arrhythmias. Two examples of this type of complication were recently observed. One patient had bigeminal rhythm with mechanically ineffective cardiac contractions and an effective cardiac rate of 35/min; he experienced dyspnea and weakness during these prolonged episodes. Another patient had repeated episodes of ventricular fibrillation. The cardiac arrhythmias were not controlled by antiarrhythmic agents, and both patients required replacement of the pulse generator. The proposed advantages of pulse generator hysteresis are (1) prolongation of battery life, and (2) maintenance of effective atrial transport; these advantages may be outweighed by undesirable cardiac arrhythmias that may be associated with this mode of pacemaker function. Rate hysteresis cardiac pacemakers should be reserved for patients having predominantly sinus rhythm without ventricular irritability. In patients with frequent ectopic ventricular activity, atrial fibrillation or high degree atrioventricular block, the rate hysteresis pacemaker offers no advantage over the conventional demand pacemaker. For patients with frequent ectopic ventricular activity not easily controlled by antiarrhythmic agents, consideration should be given to the use of a permanent demand pacemaker with external rate control, which may provide greater flexibility in arrhythmia management.
Am J Cardiol 1976 Nov 23
PMID:Undesirable cardiac arrhythmias associated with rate hysteresis pacemakers. 99 5

In arterial hypertension, casual blood pressure seems to be weakly related to the level of cardiac involvement. The aim of the present study was to assess if blood pressure during ambulatory monitoring, and during different stress tests, is a stronger predictor of anatomical and functional changes observed in hypertensive heart disease. To this aim, 29 untreated patients with borderline-to-moderate essential hypertension underwent an echo-Doppler evaluation to determine left ventricular thickness and mass. From transmitral flow, the ratio between late and early filling velocities (A/E ratio) was used to assess left ventricular diastolic behaviour. On the same day that ultrasonic study was carried out, we also measured a set of casual blood pressures; conducted a mental arithmetic test (standardized series of mental subtractions); a handgrip test (30% of maximum voluntary contraction for 3 minutes); and performed noninvasive ambulatory monitoring of blood pressure (Spacelabs 5200). Significant relationships were observed between left ventricular mass and both night-time systolic blood pressure (r = 0.46, P less than 0.02) and peak systolic blood pressure during mental stress (r = 0.39, P less than 0.05). The A/E ratio was significantly associated with casual systolic and diastolic blood pressure (r = 0.45, P less than 0.02; r = 0.38, P less than 0.05, respectively); day-time diastolic blood pressure (r = 0.47, P less than 0.02); night-time systolic and diastolic blood pressure (r = 0.44, P less than 0.05; r = 0.42, P less than 0.05 respectively); and peak systolic blood pressure during the mental arithmetic test (r = 0.44, P less than 0.05). Our results seem to confirm the presence of a relationship between causal blood pressure and left ventricular filling. Moreover, the transmitral flow seems to be dependent on both mean levels of blood pressure on ambulatory monitoring and systolic blood pressure during mental stress. As concerns left ventricular mass, the correlations observed support the weakness of the links between blood pressure and left ventricular anatomy.
Int J Cardiol 1991 Apr
PMID:Casual, ambulatory and stress blood pressure: relationships with left ventricular mass and filling. 183 35

Emery-Dreifuss muscular dystrophy (EDMD) is a rare X-linked muscular dystrophy characterized by early contractures, progressive muscle weakness, and atrial arrhythmias. Recent reports suggest that there may be additional cardiac problems in affected males and that carrier females may also show ECG abnormalities. We restudied two large families with EDMD in order to determine the extent of these problems. We examined 10 affected males and interviewed 2 others. The 3 affected males less than 20 years old had no ECG changes. All affected men of 35 years or older had arrhythmias. One had more severe arrhythmias when asleep, indicating the usefulness of continuous 24-h ECG monitoring in the evaluation of males affected with EDMD. Two required pacemakers, 4 had already had a pacemaker placed, and 4 other affected men with pacemakers had died prior to this study. One affected man with a pacemaker developed ventricular bigeminy and another developed congestive heart failure. Thus of 10 affected males with pacemakers, 6 had additional cardiac symptoms and 4 have died. Males with EDMD may survive longer with a ventricular pacemaker, but this may increase the likelihood that they will develop cardiomyopathy and ventricular arrhythmias. Of 34 carrier females examined, 6 had arrhythmias typical of EDMD. Two required a pacemaker. The risk of arrhythmia increased with age. Results from one family should be extrapolated to another with caution, as there appears to be significant interfamilial variation. We suggest careful cardiologic follow-up of EDMD patients and regular cardiac evaluations for older carrier females.
Clin Cardiol 1991 May
PMID:Progression of cardiac disease in Emery-Dreifuss muscular dystrophy. 204 91

A 14-year-old male presented with a one week history of weakness, lightheadedness and vomiting. Bilateral pleural effusions were evident on chest radiography; electrocardiogram revealed decreased voltages. Echocardiogram, abdominal ultrasound and magnetic resonance imaging revealed a mass in an hepatic vein and the inferior vena cava extending up to and filling the right atrium. Under deep hypothermia and extracorporeal circulation the mass was removed en bloc. It originated from the hepatic vein. Pathology revealed a smooth muscle tumour intermediate between benign and malignant (atypical leiomyoma). This is the first reported pediatric primary leiomyoma of the hepatic vein. It caused the Budd-Chiari syndrome, a rare pediatric entity.
Can J Cardiol 1990 Apr
PMID:Atypical hepatic vein leiomyoma extending into the right atrium: an unusual cause of the Budd-Chiari syndrome. 218 75

A diagnosis of endocarditis was made in 37 patients (three days to 21 years old) on the basis of the following: histology in 11; at least two positive blood cultures in patients with underlying cardiac disease in 22; less than two positive blood cultures, vegetations seen at echocardiography and a suggestive clinical syndrome in four. Twenty-six patients had primary endocarditis (17 with pre-existing cardiopathy, nine with normal hearts). The 11 others developed secondary endocarditis following heart surgery (early onset in six, late onset in five). The mean delay before diagnosis was prolonged 35.8 days. The clinical and laboratory findings included weakness in 36 patients, fever in 35, new or modified heart murmur in 14, positive blood cultures in 30, anemia in 12, high white blood cell count in 15, increased sedimentation rate in 14, and positive echocardiogram in 11. Etiologic agents isolated were: streptococci in 17, staphylococci in seven, miscellaneous germs in eight, and aspergillus in two. Mortality was greater in patients less than one year old, infected with aspergillus or without underlying heart disease. The present study suggests that childhood endocarditis remains uncommon but presents a poor prognosis with a mortality of 27% and a morbidity of 85.7%.
Can J Cardiol 1990 May
PMID:The evolving pattern of pediatric endocarditis from 1960 to 1985. 234 61

To characterize the clinical and pathologic features of acute myocardial infarction (AMI) in cardiac transplant recipients, 22 Stanford patients who had 25 documented infarcts at a mean of 3.86 years after transplantation were reviewed. Symptoms included chest pain (2), arm pain (3), weakness (16), dyspnea (11) and palpitations (8). Three episodes were clinically silent, detected only as new electrocardiographic changes during routine follow-up. Of 18 patients hospitalized with symptoms, only 7 had electrocardiographic changes of typical Q-wave AMI; 5 had nonspecific ST-segment changes and 2 had no documented changes. Two had old Q waves. Twelve of the 18 were misdiagnosed at admission as having infection or congestive heart failure. Serial creatine phosphokinase levels were obtained in 13 patients, and values were elevated in 8. Six of 25 AMI episodes were associated with development of congestive heart failure and 4 others led to development of cardiogenic shock. Seven patients died during the acute phase of infarction, 12 were retransplanted from 2 days to 6 months after infarct and 1 died suddenly after discharge. Two healed myocardial infarctions of unknown duration were found at autopsy or on explantation in patients not clinically suspected of having an AMI. All infarcts occurred in patients known to have angiographic evidence of transplant coronary artery disease, based on annual coronary arteriography. At autopsy or explantation all hearts were found to have characteristic diffuse concentric coronary artery narrowing, and 4 (18%) had an unusual pattern of multiple foci of nontransmural AMI.(ABSTRACT TRUNCATED AT 250 WORDS)
Am J Cardiol 1989 Nov 15
PMID:Acute myocardial infarction in cardiac transplant recipients. 281 60

A 45 year old Japanese woman with an aneurysm of the left aortic sinus is described. The main trunk of the left coronary artery was displaced upward, and the proximal portion of the circumflex branch was markedly compressed and displaced posteriorly, causing subendocardial infarction and angina. The diagnosis of Takayasu's arteritis was made based on: 1) age, sex and nationality of the patient; 2) inflammatory signs followed by weakness of the right radial pulse; and 3) typical angiographic findings. Five previously reported cases are reviewed.
J Am Coll Cardiol 1986 Mar
PMID:Aneurysm of the left aortic sinus caused by Takayasu's arteritis: compression of the left coronary artery producing coronary insufficiency. 286 78

The hemodynamic and clinical profiles of gallopamil, a new calcium antagonist, were evaluated in 20 patients with severe coronary artery disease in a placebo-controlled, single-blind study. The patients were divided into 2 groups depending on baseline ejection fraction (greater than 45 or less than or equal to 45%) and underwent nuclear ventriculography, both at rest and during bicycle exercise under electrocardiographic monitoring, after 3 weeks of therapy (50 mg 3 times daily) and the 1-week run in and washout placebo periods. The mean anginal weekly frequency per patient was significantly reduced, from 3.4 to 0.5 (p less than 0.001). The left ventricular ejection fraction, cardiac volumes, ejection and filling indexes at rest and for the same workload were not altered in the population as a whole or in each of the 2 groups. The rate pressure product during exercise was reduced for the same workload from 18.0 +/- 5.0 X 10(3) to 16.8 +/- 4.7 X 10(3), while the regional ejection fraction in ischemic regions was not significantly changed. Individual variations of ventriculographic parameters in both groups were not related to basal values. Gallopamil increased the total duration of exercise from 432 +/- 201 to 537 +/- 188 s (p less than 0.001). Six patients did not complain of angina and their exercise was interrupted because of muscular weakness. The hemodynamic and clinical responses did not differ when the results in the population as a whole and in each of the 2 groups were compared. Gallopamil was effective and well tolerated, even in patients with very depressed cardiac function.
Am J Cardiol 1989 Feb 01
PMID:Clinical and hemodynamic effects of long-term administration of gallopamil in patients with coronary artery disease and normal or impaired left ventricular function. 291 30

A case of glycogenosis type II infantile onset (Pompe's disease) is presented, and the literature is reviewed in order to establish the basis of the diagnostic suspect. We studied an 8-month old female with muscular weakness--and cardiac failure. The chest X-ray: cardiomegaly; electrocardiogram: PR interval of 0.06 seconds, gigant QRS complexes, biventricular hypertrophy, and the echocardiogram and cineangiography: left ventricle hypertrophy and hypokinesia. The patient died because of refractory heart failure. Histochemical examination demonstrated excessive intracellular accumulation of glycogen. Also, in the muscle, the kidneys and the liver we did not find any alpha 1,4 glucosidase. Then, the diagnosis of glycogenosis type II infantile onset was established. Finally, we conclude that this disease should be suspected in every infant with muscular weakness, cardiac failure, cardiomegaly, electrocardiogram with shortness of PR interval, gigant QRS complexes and biventricular hypertrophy, and that needs to be confirmed by muscle biopsy. At the moment, there has not been reported any case of Pompe's disease in the Mexican literature.
Arch Inst Cardiol Mex
PMID:[Glycogenosis type II, infantile variant. Report of a case and review of the literature]. 294 26

A 67 year old woman developed a fatal febrile illness 8 years after mitral valve replacement for rheumatic valvular heart disease. The final disease persisted for 1 year and was characterized clinically by weakness, weight loss, congestive heart failure and multiple embolic events to the central nervous system and abdominal organs. The source of the emboli was a tumor, malignant fibrous histiocytoma of the left atrium, originating from the anulus fibrosus around the covered base of the prosthetic valve. This unique case suggests the possibility that chronic exposure to materials, such as Dacron, covering prosthetic valves may induce local malignant tumors.
J Am Coll Cardiol 1986 Apr
PMID:Malignant fibrous histiocytoma complicating mitral valve replacement. 300 98


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