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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The oto-vertebral syndrome is a rare combination of malformations of the ears, spinal anomalies, and congenital heart disease. The syndrome appears to be caused by early embryonic exogenous damage during the 6th or 7th week of embryonic development. The case is reported of a 28-year-old man with bilateral aplasia of the external ear, bilateral aplasia of the ear canal, hypoplasia of the mandibula, severe thoracic scoliosis, and ventricular septal defect with pulmonary hypertension. He was admitted with dyspnea on exertion, syncope, and severe cyanosis. Cardiac catheterization revealed severe pulmonary hypertension with moderate right-to-left and slightly left-to-right shunt (Eisenmenger syndrome). Right and left ventricular function, as evaluated by angiocardiography, was slightly reduced. Because of the severe hemodynamic alterations, symptomatic therapy with digitalis, repeated venesection, and anticoagulation was initiated.
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PMID:[The oto-vertebral syndrome]. 70 8

A 40-year-old woman was admitted because of increasing exertional dyspnea. Right heart failure was suggested by the presence of hepatomegaly, pretibial edema and also echocardiographic findings. Physical examination and echocardiography showed no evidence of valvular disease or congenital heart disease except for right ventricular dilatation and tricuspid regurgitation. The ventricular septum deviated toward the left ventricle throughout the cardiac cycle, but left ventricular function was preserved. Severe pulmonary hypertension averaging 44 mmHg was revealed by cardiac catheterization. Digital subtraction angiography and pulmonary blood flow scintigraphy showed no evidence of pulmonary artery embolism, and no interstitial pulmonary lesions that might have caused pulmonary hypertension were recognized. Hypergammaglobulinemia suggested an autoimmune disorder, and signs of systemic lupus erythematosus (SLE), such as pleural effusion, proteinuria, lymphocytopenia, LE cell phenomenon and antinuclear antibodies were present. Several autoimmune diseases are known to be causative factors of pulmonary hypertension. However, only ten cases of SLE complicated by pulmonary hypertension have been reported the present one. These cases were characterized by a high incidence of Raynaud's phenomenon and positivity for anti-RNP antibody. In our present case, SLE activity was suppressed using prednisolone, but pulmonary hypertension persisted and the patient eventually died due to right cardiac failure. Judging from the clinical course of the ten reported cases of SLE-pulmonary hypertension, there seems to be no hope of improving the pulmonary hypertension once it has become established. Therefore it is important to detect and cure pulmonary hypertension as early as possible.
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PMID:[A case of lupus erythematosus preceded by right heart failure due to pulmonary hypertension]. 174 69

Though breathing pattern is frequently analyzed during clinical exercise testing, there is little information regarding its usefulness in the differential diagnosis of impaired exercise tolerance. This study tested the hypothesis that differences in peak tidal volume during exercise between patients with different cardiorespiratory diseases are related largely to differences in severity of respiratory mechanical impairment (vital capacity), not to differences in disease state. Patients with chronic obstructive pulmonary disease, restrictive lung disease, bronchial asthma, and heart disease (mitral valve disease or left ventricular dysfunction) were studied. Subjects selected had one and only one of the above diagnoses. All subjects performed maximal (symptom-limited) incremental exercise on a cycle ergometer. Multiple linear regression of all subjects (n = 30) in all four groups showed a significant correlation between VTmax and VC: VTmax = 0.55, VC -0.09 L (r = 0.827, p less than 0.0001). The VTmax/VC (x 100) was (mean +/- SD) 44 +/- 15, 54 +/- 11, 56 +/- 11, and 54 +/- 12 for the COPD, RLD, BA and HD patients respectively. There was no significant difference between any of the groups. We concluded that differences in VTmax between different patients are related largely to differences in VC (ie, differences in severity of respiratory mechanical impairment), not to differences in disease state. Measurement of VTmax or the VTmax/VC ratio has little value in the differential diagnosis of exertional dyspnea.
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PMID:Diagnostic value of maximal exercise tidal volume. 224 73

A 10-year-old boy with idiopathic scoliosis presented with exertional dyspnea, lower leg edema since two weeks prior to admission. Physical examination revealed neck venous engorgement, moist rales, mild hepatomegaly, ascites and lower leg edema in addition to the scoliosis. Chest x-ray films showed cardiomegaly, pulmonary congestion and pneumonic infiltration of both lower lobes with bilateral pleural effusion. Electrocardiograms showed right axis deviation and right atrial hypertrophy. Two-dimensional echocardiography revealed dilatation of the right heart chambers and pulmonary artery. He was treated initially with mechanical ventilation, antibiotics, bronchodilators, diuretics, vasodilators, and digoxin with improvement. Unfortunately the patient became comatose after an episode of cardiopulmonary arrest. He did not recover from this tragedy and was discharged against medical advice in comatose state. Scoliosis may be complicated with pneumonia and cor pulmonale in children. Early surgical correction of the scoliosis may prevent the development of cor pulmonale. Combined medical and surgical managements should be carried out aiming at scoliotic heart disease.
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PMID:[Idiopathic scoliosis and pneumonia complicated with cor pulmonale. Report of one case]. 227 31

From January 1972 to April 1982, 125 adults underwent operation for congenital heart disease at the Medical University of South Carolina. Age ranged from 18 to 73 years, with an average age of 31 years. A variety of defects were seen. Atrial septal defect (59 patients), ventricular septal defect (15 patients), patent ductus arteriosus (14 patients), tetralogy of Fallot (14 patients), and coarctation of the aorta (9 patients) were the most common. There were 48 men and 77 women in the study. Most patients presented with recent onset of dyspnea on exertion or at rest, but 16 patients were asymptomatic. Eight patients died after surgery for an overall operative mortality rate of 6 per cent. The most common postoperative complication was arrhythmia (13 patients, 10%). Despite recent advances in the diagnosis and treatment of congenital heart disease in infants, a significant number of patients apparently escape detection and first present with congenital heart disease as adults. Prompt diagnosis and surgical intervention will usually produce excellent results and relief of symptoms in these patients.
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PMID:Congenital heart disease in adults. 669 33

It is not the purpose of this paper to dismiss the possibility that tricyclics may somehow cause cardiac function to deteriorate, with resulting congestive heart failure. Any patient with heart disease should receive intense clinical scrutiny for the development of cardiac symptoms--dyspnea on exertion, ankle edema, orthopnea, paroxysmal nocturnal dyspnea, rales, and the presence of an S3 are never taken lightly. These parameters should be monitored because the patient has cardiac disease, not because he or she is on a tricyclic antidepressant.
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PMID:Cardiovascular effects of antidepressants. 681 88

To clarify the clinical and electrophysiological characteristics of atrial standstill (AS) we studied 11 patients (7 males and 4 females), whose average age was 62 years and who were followed over a period of 4-179 months. Underlying heart disease was present in nine patients and two cases were idiopathic. Major clinical symptoms in the 11 cases included Adams-Stokes attacks, and dyspnea on exertion. In the standard 12-lead ECGs obtained on admission, the P wave was absent in six cases. Atrial flutter (AF) was noted in 3, atrial fibrillation (Af) in 1, and multifocal atrial tachycardia in 1. In some cases, the ECG initially showed AF or Af, and was transformed after several years into ectopic atrial tachycardia or an ectopic atrial rhythm with a markedly decreased amplitude of the P wave. Finally, the P wave disappeared over a prolonged period. When intracardiac mapping was performed, the atrial electrograms tended to diminish at the site of high, mid-lateral right atrium (RA). Electrograms were remained present in the vicinity of the tricuspid valve (TV) annulus. A repeated mapping and pacing study conducted in two patients revealed that the "silent" area spread toward the lower site of RA. During the average follow-up period of 64 months, four patients died. The interval until death in one patient with myocarditis was 6 months, and in another with dilated cardiomyopathy (DCM) it was 8 months. It appears that the atrial muscular lesion starts in the high lateral RA and progresses toward the lower RA, then to the vicinity of the TV annulus. A diffuse and progressive disturbance may occur not only in the atrial muscle, but also in the atrioventricular conduction system in patients with AS who had progressive myocarditis or DCM.
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PMID:Clinical and electrophysiological characteristics of atrial standstill. 765 78

We describe a 63-year old female patient presenting with increasing dyspnea on exertion. The chest X-ray was suggestive but not compelling for Ebstein's anomaly. Although Ebstein's anomaly is most often discovered first in childhood, rare cases, such as ours, are described in late adulthood, however. Obesity may be one reason why it was not possible in our patient to detect the characteristic features in transthoracic echocardiography. The diagnosis of Ebstein's anomaly could be established only by transesophageal echocardiography: There the right atrium was grossly enlarged due to the distal insertion of the septal tricuspid valve leaflet. Colour flow echocardiography clearly demonstrated severe tricuspid valve incompetence. No additional congenital or acquired cardiac disorder could be detected. Cardiac catheterization confirmed the echocardiographical findings, whereas magnetic resonance tomography did not show the abnormal insertion of the tricuspid valve leaflet. If Ebstein's anomaly is suspected and cannot be ascertained by transthoracic echocardiography, transesophageal echocardiography can be employed to definitely diagnose this fairly rare form of congenital heart disease in an adult population.
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PMID:[Diagnosis of Ebstein's anomaly with transesophageal echocardiography]. 792 29

A 32-year-old woman was hospitalized with recurrent left-sided chest pain and dyspnea on exertion, which had progressed for approximately 10 years. Since age 18 she had been spending more than twelve hours per day in a predominantly seated position on a floor mat, engaged in Japanese dressmaking. A chest roentgenogram showed marked dilation of the main pulmonary arteries, bilateral oligemia in the upper lung fields and a peripheral infiltration in the middle field of the left lung. The (99m)Tc-MAA perfusion lung scan showed multiple defects in both lungs, but no abnormal findings were detected on a 133Xe ventilation scan. A pulmonary angiogram showed multiple occlusions of pulmonary arteries in both lungs. Because recurrent chest pain and dyspnea had been present for a long time, and because ultrasonic cardiography revealed pulmonary hypertension repeatedly for several years, pulmonary thromboembolism was considered to be chronic and recurrent. The patient had none of the following risk factors for pulmonary emboli: malignancy, neurological disease, heart disease, obesity, pregnancy, or a congenital coagulative abnormality such as deficiency of AT-III, protein C, protein S, or plasminogen. Because no other cause could be found, the chronic recurrent pulmonary thromboembolism most likely resulted from extensive sedentary work that caused stagnation of venous return and deep vein thrombosis.
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PMID:[Chronic recurrent pulmonary thromboembolism associated with sedentary work]. 862 76

Cardiac diseases are well known among patients on maintenance hemodialysis (HD), and carnitine deficiency may be an important factor in cardiac morbidity. We studied the effects of low-dose L-carnitine treatment (500 mg/day) on chest symptoms (dyspnea on exertion, chest pain, palpitation), cardiac function, and left ventricular (LV) mass in 9 HD patients with reduced ejection fraction (EF). After 6 months of L-carnitine treatment, most patients had at least some improvement in chest symptoms, while LVEF was increased and LV mass was decreased. Carnitine fractions increased and reached plateaus at 2-3 times the baseline levels. These results suggest that prolonged low-dose L-carnitine treatment can improve the cardiac morbidity by restoring decreased carnitine tissue levels and impaired oxidation of FFA.
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PMID:Effects of L-carnitine supplementation on cardiac morbidity in hemodialyzed patients. 1087 1


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