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Primary hyperparathyroidism with cardiac abnormalities: a case report.

Primary hyperparathyroidism, characterized by hypersecretion of parathyroid hormone (PTH) leading to hypercalcemia and relative hypophosphatemia, is quite common in the elderly. Most patients with primary hyperparathyroidism have only mild hypercalcemia and are symptomless. But others experience various other organ diseases. Primary hyperparathyroidism is also associated with cardiovascular abnormalities, including QT interval shortening, heart block, cardiac arrhythmias, hypertension, myocardial hypertrophy, myocardial calcification and, though rarely, with valvular heart disease. We described a case of primary hyperparathyroidism associated with cardiac abnormalities. An 82-year-old male presented with the complaints of chest discomfort, fatigue, general weakness, nausea and vomiting over a period of months and was admitted in July 1996. Physical examination with heart auscultation showed a pansystolic murmur over the right sternal border and apex region, and a blowing diastolic murmur over the left sternal border. Biochemistry profiles revealed elevations of serum calcium (14.3 mg/dl) and chloride/phosphate ratio (> 33). Endocrinological studies showed elevations of serum PTH-C (4.8 ng/ml) and PTH-intact (705 pg/ml) concentrations. Kidney ultrasonography revealed a left renal stone. A spine X-ray revealed spondylosis and a compression fracture of the lumbar-spine with osteoporotic change. Thyroid ultrasonography and Thallium (Tl201)-technetium (Tc99m) subtraction scan showed parathyroid adenoma in the low pole of the right thyroid bed. Parathyroid aspiration cytology revealed few and discrete cells. Echocardiogram revealed moderate to severe aortic valvular calcification as well as stenosis with moderate aortic regurgitation, mitral regurgitation and myocardial calcification. The patient received parathyroidectomy one month later. During his postoperative days, he suffered from muscle twitching with positive Trousseau's sign and Chvostek's sign. The patient received calcium carbonate and vitamin D for hypocalcemia, diltiazem and capoten for his heart problems. A repeated echocardiogram two months after surgery showed no improvement of valvular calcification.

Aged↗

Severe hypoglycaemia and cardiovascular autonomic dysfunction in type I diabetic children treated with intensified conventional insulin therapy.

To assess the relationship between severe hypoglycaemias and autonomic dysfunction, five cardiovascular tests (resting heart rate, hyperventilatory arrhythmia, standing/lying heart rate ratio, orthostatic decrease in blood pressure, and increase in blood pressure during sustained handgrip) were performed in a 1-yr prospective study of 34 insulin-dependent diabetic children treated with intensified conventional insulin therapy (ICIT). There were twelve severe episodes in 7 diabetic children, and the remaining 27 patients had no severe hypoglycaemia. The hypoglycaemic group had a longer duration of diabetes than the nonhypoglycaemic group (5.4 SD 2.5 years vs. 2.8 SD 2.2 years, p less than 0.02). The hyperventilatory arrhythmia in the hypoglycaemic group in comparison with the nonhypoglycaemic group was significantly decreased (before ICIT: 16.1 SD 3/min vs. 24.4 SD 5/min, p less than 0.01; 1 yr thereafter: 17.3 SD 3/min vs. 26.0 SD 5/min, p less than 0.01). The hypoglycaemic group showed a pronounced orthostatic decrease in blood pressure compared to the nonhypoglycaemic group (before ICIT: 13.2 SD 4 mmHg vs. 6.0 SD 4 mmHg, p less than 0.01; 1 yr thereafter: 12.3 SD 4 mmHg vs. 5.6 SD 4 mmHg, p less than 0.01). Three or more abnormal cardiovascular test results were found in patients of the hypoglycaemic group who showed abnormal hyperventilatory arrhythmia and abnormal orthostatic decrease in blood pressure simultaneously, whereas such a coexistence was not found in the nonhypoglycaemic group. These observations may support the view that diabetic children and adolescents with autonomic dysfunction are susceptible to severe hypoglycaemia during ICIT.

Adolescent↗

Cardiovascular drugs for the newborn.

This article reviews the various cardiovascular drugs for newborns, including antiarrhythmics, antihypertensives, inotropes, and pulmonary vasodilators. Antiarrhythmic drugs are classified according to their mechanisms of action, such as effects on ion channels, duration of repolarization, and receptor interaction, which help with understanding the effects of individual antiarrhythmic drugs and selection of drugs for specific arrhythmias. Drug treatment for hypertension should start with a single drug from one of the following classes: ACE inhibitors, angiotensin-receptor antagonists, beta-receptor antagonists, calcium channel blockers, or diuretics. The inotropic drug should be selected according to its specific pharmacologic properties and the specific cardiovascular abnormality to be corrected. An effective pulmonary vasodilator must dilate the pulmonary vasculature more than the systemic vasculature.

Cardiovascular Agents↗

Effects of thyroid hormone and thyroid dysfunction on the cardiovascular system.

Thyroid disease is common in veterinary practice. The heart, especially the myocardium, is sensitive to thyroid hormone, and deficiencies or excesses can alter cardiovascular function. Observed changes result from direct effects upon the myocardium and indirect effects that result from effects upon the vasculature and peripheral tissues. Clinically significant cardiovascular abnormalities related to hypothyroidism are rare. If present, they are primarily manifest as reduced left ventricular pump function, as apparent echocardiographically, or arrhythmias. Hyperthyroidism is common in the cat and infrequently encountered in dogs. Clinically significant cardiovascular manifestations are common and often dramatic. Hyperdynamic systolic function and mild myocardial hypertrophy are common manifestations which may lead to overt congestive and high output heart failure. If signs of congestive heart failure or significant arrhythmias are not evident, specific therapy need only be directed toward restoration of the euthyroid state. In most cases the cardiovascular changes associated with thyroid dysfunction are completely reversible.

Animals↗

Superior mesenteric artery blood flow velocity and estimated volume flow: duplex Doppler US study of preterm and term neonates.

To obtain information about intestinal hemodynamics of healthy neonates, the authors assessed velocity and volume of blood flow with duplex Doppler sonography in the superior mesenteric artery (SMA) in 91 stable preterm and term neonates. Blood flow velocity in the SMA and estimated volume blood flow increased linearly with gestational age and increasing body weight. The mean estimated volume blood flow (+/- standard deviation) was 43 mL/kg/min +/- 13 and did not depend on differences in body weight. The authors also assessed blood flow velocity in the SMA and volume blood flow in 18 infants with conditions that may affect blood supply to the bowel. Twelve infants who were small for gestational age appeared to have an abnormally low resistance of the vascular bed of the SMA during the 1st days of life, as compared with stable appropriate-for-gestational-age infants matched for gestational age. Three of six term neonates with cardiovascular abnormalities had left ventricular outflow obstruction and an abnormal blood flow velocity waveform of the SMA, suggesting a decrease in blood supply to the bowel. The results of this study may help in evaluations of intestinal perfusion in infants with abnormal conditions.

Blood Flow Velocity↗

Effects of thyroid hormone on the cardiovascular system.

Increased or reduced action of thyroid hormone on certain molecular pathways in the heart and vasculature causes relevant cardiovascular derangements. It is well established that overt hyperthyroidism induces a hyperdynamic cardiovascular state (high cardiac output with low systemic vascular resistance), which is associated with a faster heart rate, enhanced left ventricular (LV) systolic and diastolic function, and increased prevalence of supraventricular tachyarrhythmias - namely, atrial fibrillation - whereas overt hypothyroidism is characterized by the opposite changes. However, whether changes in cardiac performance associated with overt thyroid dysfunction are due mainly to alterations of myocardial contractility or to loading conditions remains unclear. Extensive evidence indicates that the cardiovascular system responds to the minimal but persistent changes in circulating thyroid hormone levels, which are typical of individuals with subclinical thyroid dysfunction. Subclinical hyperthyroidism is associated with increased heart rate, atrial arrhythmias, increased LV mass, impaired ventricular relaxation, reduced exercise performance, and increased risk of cardiovascular mortality. Subclinical hypothyroidism is associated with impaired LV diastolic function and subtle systolic dysfunction and an enhanced risk for atherosclerosis and myocardial infarction. Because all cardiovascular abnormalities are reversed by restoration of euthyroidism ("subclinical hypothyroidism") or blunted by beta-blockade and L-thyroxine (L-T4) dose tailoring ("subclinical hyperthyroidism"), timely treatment is advisable in an attempt to avoid adverse cardiovascular effects. Interestingly, some data indicate that patients with acute and chronic cardiovascular disorders and those undergoing cardiac surgery may have altered peripheral thyroid hormone metabolism that, in turn, may contribute to altered cardiac function. Preliminary clinical investigations suggest that administration of thyroid hormone or its analogue 3,5-diiodothyropropionic acid greatly benefits these patients, highlighting the potential role of thyroid hormone treatment in patients with acute and chronic cardiovascular disease.

Cardiovascular Diseases↗

[Propofol-fentanyl anesthesia for a 13-year-old patient with Beckwith-Wiedemann syndrome].

We report anesthetic management of a 13-year-old boy with Beckwith-Wiedemann syndrome for urethral fistulectomy using propofol-fentanyl anesthesia. The syndrome has three major symptoms; exomphalos, macroglossia and gigantism. Other complications including neonatal hypoglycemia, visceromegaly, metabolic disorders, malignant tumors, and cardiovascular abnormalities may also be seen. This patient was complicated with gigantism, exomphalos, hypospadias, cryptorchidism, hepatoblastoma, accessory mammae, and left axis deviation with left anterior fascicular block. A comprehensive preoperative examination should include assessment of the airway, cardiovascular conditions, endocrine status, blood glucose, and electrolytes. In this patient, airway management was easy by laryngeal mask airway and the anesthetic course was uneventful.

Adolescent↗

Cardiovascular autonomic dysfunction in familial Mediterranean fever.

OBJECTIVE: To compare the hemodynamic responses to autonomic challenge evoked by upright tilt table testing in patients with familial Mediterranean fever (FMF). METHODS: Forty consecutive patients with FMF and 25 age and sex matched healthy controls were evaluated using the head-up tilt test (HUTT). The main outcome measures were the values of blood pressure (BP) and heart rate (HR) recorded during recumbence and tilt. The endpoints of vasodepressor and cardioinhibitory reactions, orthostatic tachycardia, and postural tachycardia syndrome were recorded. RESULTS: Patients with FMF exhibited significantly higher diastolic BP during supine and tilt measurements (p = 0.003 and 0.04, respectively). In response to tilt, patients showed significant increases in HR compared to healthy subjects (p = 0.02). Pathological endpoints on tilt were observed in the FMF group in 7 patients (17%) and in no controls. FMF severity, genotype, duration of illness, response to therapy, and associated amyloidosis did not correlate with pathological reactions on HUTT. CONCLUSION: FMF patients exhibit an abnormal cardiovascular reactivity, which is clinically occult, but can be detected on autonomic challenge. The abnormal autonomic activity in FMF is similar to dysautonomia described in a variety of rheumatic disorders.

Adolescent↗

Preparticipation echocardiographic screening for cardiovascular disease in a large, predominantly black population of collegiate athletes.

Sudden death in young competitive athletes is most commonly due to underlying cardiovascular disease. Echocardiography has the potential to identify structural cardiovascular abnormalities, such as hypertrophic cardiomyopathy (HC), that have been incriminated in such events. In this study, echocardiography (2-dimensional and M-mode) was used as a primary screening test to assess 265 Howard University collegiate athletes for cardiovascular disease; 262 (99%) were black. Most athletes (234, 88%) had no definitive echocardiographic evidence of HC or other major cardiovascular diseases, but 30 (11%) had mitral valve prolapse, and 1 other athlete had a small atrial septal defect. In addition, 4 athletes were identified as having mild systemic hypertension. Most athletes (236 of 265) showed normal left ventricular wall thickness of less than or equal to 12 mm, but an important minority (29, 11%) had maximal ventricular septal thicknesses of greater than or equal to 13 mm that could not always be distinguished (by morphology alone) from mild anatomic expressions of nonobstructive HC. Based on this experience, preparticipation athletic screening using echocardiography as the primary test does not appear to be justified on a cost-effective basis. In addition, the substantial minority of subjects with increased wall thickness made clinical interpretation of the echocardiographic findings difficult in individual athletes.

Adult↗

Sonographic differential diagnosis of fetal cardiac abnormalities.

Ultrasonographic evaluation of the fetal heart for structural and functional abnormalities is vital for prenatal diagnosis and perinatal management. Even more important than up-to-date diagnostic equipment is keen observation and an understanding of fetal cardiac abnormalities. One diagnostic approach begins with the four-chamber view with particular attention to the normal symmetric sizes of all four cardiac chambers. Ventricular disproportion, inequality in ventricular size, is often an easily detectable sign of a cardiovascular abnormality. Segmental diagnosis of cardiac and visceral situs, ventricular outflow tracts for conotruncal abnormalities and the aortic and ductal arches is also vital for diagnosing affected fetuses.

Diagnosis, Differential↗

[Relapsing polychondritis: report of a case and review of the literature].

Relapsing polychondritis (RP) is a rare disease of unknown etiology, characterized by episodic and progressive chondritis, ocular and audiovestibular involvement, and occasional cardiovascular abnormalities. The inflammation typically involves the cartilage of the ears, nose, trachea, larynx, ribs, joints, and Eustachian tubes. The major clinical features include auricular chondritis, arthritis, nasal chondritis, ocular inflammation, chondritis of the respiratory tract, audiovestibular damage, and cardiovascular disorders. RP occurs predominantly in Caucasians and is uncommon in Oriental patients. Two cases of RP have previously been reported in Taiwan. We report one Taiwanese patient who was diagnosed with RP according to the criteria proposed by Damiani and Levine. The patient has bilateral auricular chondritis, episcleritis, and uveitis. Successful treatment was accomplished with systemic corticosteroid and topical corticosteroid eye drops. At an outpatient visit one year and ten months after discharge, there was no evidence of recurrence.

Adult↗

Oxygen pulse during exercise is related to resting systolic and diastolic left ventricular function in older persons with mild hypertension.

BACKGROUND: The mechanisms responsible for impaired cardiovascular hemodynamics during exercise among persons with milder forms of hypertension are not well documented. We examined the relationship of oxygen pulse during exercise, a correlate of stroke volume, with echocardiographic indices of resting left ventricular function to determine whether abnormal contractility and relaxation are related to abnormal cardiovascular dynamics during exercise among such persons. METHODS: Subjects were 44 men and 55 women ages 55 to 75 years with mild hypertension but who were otherwise healthy. Resting left ventricular systolic and diastolic functions were assessed with 2-dimensional Doppler echocardiography and tissue Doppler imaging. Oxygen pulse (millimeters per beat) at rest and during multistage treadmill testing was derived from measurements of oxygen consumption and heart rate. The slope of oxygen pulse between successive exercise stages was calculated. RESULTS: After a steep rise in oxygen pulse from rest to stage 1 of exercise, a markedly diminished oxygen pulse slope was seen between subsequent exercise stages. In stepwise regression analysis, the increase in the slope of oxygen pulse from rest to stage 1 was explained by a greater lean body mass (57%, P < .001) and a larger left atrial size (2%, P < .001). After exercise stage 1, the increase in the slope of oxygen pulse was explained by sex (24%, P < .001), higher mitral E/A ratio (6%, P < .001), and higher mitral annular systolic velocity (6%, P < .001). CONCLUSIONS: These results suggest that a blunted oxygen pulse response to exercise among older persons with milder forms of hypertension may reflect impaired left ventricular stroke volume changes during exercise secondary to subtle abnormalities in both systolic and diastolic left ventricular functions.

Aged↗

Triiodothyronine in cardiac surgery.

Thyroid hormones have profound cardiovascular effects. Chronic hypothyroidism is associated with cardiovascular abnormalities that include diminished cardiac output and increased systemic vascular resistance. Acute hypothyroidism, frequently referred to as the "euthyroid sick syndrome," is present in diverse clinical situations such as brain death, sepsis, congestive heart failure, and cardiopulmonary bypass. Significant cardiovascular dysfunction often complicates each of these clinical situations. This article reviews the laboratory experiments and clinical trials that have evaluated triiodothyronine (T3) repletion in cardiac surgery. Animal experiments have shown that T3 repletion ameliorates postischemic cardiovascular dysfunction. While anecdotal clinical experience suggests that T3 repletion should be of clinical benefit, rigorous clinical trials have failed to support routine repletion of T3 in cardiac surgery. Based on the results of these clinical trials, we do not recommend routine administration of T3 to patients undergoing cardiac surgery. However, anecdotal experience suggests that T3 may help in weaning patients from cardiopulmonary bypass who are unable to be weaned from bypass despite maximal inotropic support. In use as a "rescue" agent, we administer the 0.8 microgram/kg dose that has been demonstrated to safely improve cardiac output and decrease systemic vascular resistance in the postischemic cardiopulmonary bypass patient.

Animals↗

Cardiopulmonary rehabilitation in a patient with Noonan syndrome.

Noonan syndrome, an autosomal dominant disease occurring with an incidence of 1 in 1,000 to 1 in 2,500 live births, is characterized by its particular cardiovascular abnormalities, including pulmonic valve stenosis, pulmonary artery stenosis, and, more rarely, septal defects and coarctation of the aorta. The case of a 20-year-old man admitted for inpatient cardiopulmonary rehabilitation after pulmonic valve repair, left pulmonary artery angioplasty, and pectus excavatum repair is presented. His endurance was markedly decreased, thus limiting his ability to perform activities of daily living and reducing his exercise tolerance. With participation in a comprehensive cardiopulmonary rehabilitation program, he experienced marked improvement with independence in his activities of daily living and an increase in his metabolic equivalent levels from to 2.8 to 5.4. After inpatient rehabilitation, he underwent left pulmonary stent placement before being discharged home. Subsequent outpatient cardiopulmonary rehabilitation has continued to improve significantly his overall exercise tolerance. Given that Noonan syndrome is viewed as the most common syndrome associated with congenital heart disease after Down syndrome, physiatrists must be familiar with its presentation, its associated abnormalities, and the treatment approach to optimize the patient's cardiopulmonary, musculoskeletal, and psychological status.

Adult↗

Autonomic and sensory nerve dysfunction in primary biliary cirrhosis.

AIM: Cardiovascular autonomic and peripheral sensory neuropathy is a known complication of chronic alcoholic and non-alcoholic liver diseases. We aimed to assess the prevalence and risk factors for peripheral sensory nerve and autonomic dysfunction using sensitive methods in patients with primary biliary cirrhosis (PBC). METHODS: Twenty-four AMA M2 positive female patients with clinical, biochemical and histological evidence of PBC and 20 age matched healthy female subjects were studied. Five standard cardiovascular reflex tests and 24-h heart rate variability (HRV) analysis were performed to define autonomic function. Peripheral sensory nerve function on median and peroneal nerves was characterized by current perception threshold (CPT), measured by a neuroselective diagnostic stimulator (Neurotron, Baltimore, MD). RESULTS: Fourteen of 24 patients (58%) had at least one abnormal cardiovascular reflex test and thirteen (54%) had peripheral sensory neuropathy. Lower heart rate response to deep breathing (P = 0.001), standing (P = 0.03) and Valsalva manoeuvre (P = 0.01), and more profound decrease of blood pressure after standing (P = 0.03) was found in PBC patients than in controls. As a novel finding we proved that both time domain and frequency domain parameters of 24-h HRV were significantly reduced in PBC patients compared to controls. Each patient had at least one abnormal parameter of HRV. Lower CPT values indicated hyperaesthesia as a characteristic feature at peroneal nerve testing at three frequencies (2000 Hz: P = 0.005; 250 Hz: P = 0.002; 5 Hz: P = 0.004) in PBC compared to controls. Correlation of autonomic dysfunction with the severity and duration of the disease was observed. Lower total power of HRV correlated with lower CPT values at median nerve testing at 250 Hz (P = 0.0001) and at 5 Hz (P = 0.002), as well as with those at peroneal nerve testing at 2000 Hz (P = 0.01). CONCLUSION: Autonomic and sensory nerve dysfunctions are frequent in PBC. Twenty-four-hour HRV analysis is more sensitive than standard cardiovascular tests for detecting of both parasympathetic and sympathetic impairments. Our novel data suggest that hyperaesthesia is a characteristic feature of peripheral sensory neuropathy and might contribute to itching in PBC. Autonomic dysfunction is related to the duration and severity of PBC.

Aged↗

Treatment with drugs to lower blood pressure and blood cholesterol based on an individual's absolute cardiovascular risk.

In this review, we outline the rationale for targeting blood pressure and blood cholesterol lowering drug treatments to patients at high absolute cardiovascular risk, irrespective of their blood pressure or blood cholesterol levels. Because the specific levels of blood pressure and cholesterol are of little clinical relevance when considered in isolation from other risk factors, terms such as hypertension or hypercholesterolaemia have limited value. Separate management guidelines for raised blood pressure and blood cholesterol need to be replaced by integrated cardiovascular risk management guidelines, and absolute cardiovascular risk prediction scores should be used routinely. Since cardiovascular risk factors interact with each other, moderate reductions in several risk factors can be more effective than major reductions in one. An affordable daily pill combining low doses of various drugs could be useful for the many individuals with slightly abnormal cardiovascular risk factors.

Adult↗

Cardiovascular autonomic function in multiple sclerosis.

In this study blood pressure (BP) and heart rate (HR) responses to standing and HR responses to deep breathing were assessed in 34 patients with clinically definite multiple sclerosis (MS) and 63 healthy subjects. Normal ranges, which were clearly age related for both HR responses, were obtained. The BP response to standing was abnormal in 13% of the MS patients, these patients demonstrating significant postural hypotension. The HR response to standing was abnormal in 28% of the MS patients, with a normal initial increase in heart rate and a significantly reduced reflex bradycardia. On deep breathing 36% of MS patients showed abnormal HR changes. The resting HR did not differ between both groups. Abnormalities of one or more tests were found in 53% of the MS patients. No relationship was found between abnormal cardiovascular autonomic responses and the symptoms, duration, severity and progression of the disease. Based on clinical and magnetic resonance imaging findings no indications were found for localisation of the autonomic disturbances in the brainstem. It is suggested that at least a part of the cardiovascular autonomic lesions in MS is located outside the brainstem, i.e. in supramedullary reflex pathways or in the spinal cord.

Adult↗