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

Steinert's disease (Dystrophia myotonica type 1) is an autosomal dominant neuromuscular disease characterized by myotonia, muscle weakness, frontal balding, cataracts, cardiac conduction abnormalities, especially long PR interval and wide QRS complex. Although subclinical mild myocardial dysfunction may be detected in this syndrome, overt myocardial dysfunction with heart failure called as "myotonic heart disease" is not frequent. Cardiac resynchronization therapy is an effective treatment modality to improve morbidity and mortality in patients with intraventricular conduction delay and congestive heart failure. We report improvement of cardiac dyssynchrony and symptoms of heart failure with biventricular pacing in a 37-year-old male patient with overt myotonic heart disease, PR segment prolongation, and complete left bundle branch block.
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PMID:Cardiac resynchronization therapy in a case of myotonic dystrophy (Steinert's disease) and dilated cardiomyopathy. 1758 76

Cardiac disorders complicate less than 1% of all pregnancies. Physiologic changes in pregnancy may mimic heart disease. In order to differentiate these adaptations from pathologic conditions, an in-depth knowledge of cardiovascular physiology is mandatory. A comprehensive history, physical examination, electrocardiogram, chest radiograph, and echocardiogram are sufficient in most cases to confirm the diagnosis. Care of women with cardiac disease begins with preconception counseling. Severe lesions should be taken care of prior to contemplating pregnancy. Management principles for pregnant women are similar to those for the non-pregnant state. A team approach comprised of a maternal fetal medicine specialist, cardiologist, neonatologist, and anesthesiologist is essential to assure optimal outcome for both the mother and the fetus. Although fetal heart disease complicates only a small percentage of pregnancies, congenital heart disease causes more neonatal morbidity and mortality than any other congenital malformation. Unfortunately, screening approaches for fetal heart disease continue to miss a large percentage of cases. This weakness in fetal screening has important clinical implications, because the prenatal detection and diagnosis of congenital heart disease may improve the outcome for many of these fetal patients. In fact, simply the detection of major heart disease prenatally can improve neonatal outcome by avoiding discharge to home of neonates with ductal-dependent congenital heart disease. Fortunately, recent advances in screening techniques, an increased ability to change the prenatal natural history of many forms of fetal heart disease, and an increasing recognition of the importance of a multidisciplinary, team approach to the management of pregnancies complicated with fetal heart disease, together promise to improve the outcome of the fetus with congenital heart disease.
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PMID:Pregnancy: maternal and fetal heart disease. 1764 25

The X-linked McLeod syndrome is defined by absent Kx red blood cell antigen and weak expression of Kell antigens, and this constellation may be accidentally detected in routine screening of apparently healthy blood donors. Most carriers of this McLeod blood group phenotype have acanthocytosis and elevated serum creatine kinase levels and are prone to develop a severe neurological disorder resembling Huntington's disease. Onset of neurological symptoms ranges between 25 and 60 years, and the penetrance of the disorder appears to be high. Additional symptoms of the McLeod neuroacanthocytosis syndrome that warrant therapeutic and diagnostic considerations include generalized seizures, neuromuscular symptoms leading to weakness and atrophy, and cardiopathy mainly manifesting with atrial fibrillation, malignant arrhythmias and dilated cardiomyopathy. Therefore, asymptomatic carriers of the McLeod blood group phenotype should have a careful genetic counseling, neurological examination and a cardiologic evaluation for the presence of a treatable cardiomyopathy.
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PMID:McLeod syndrome: a neurohaematological disorder. 1768 54

Rigid spine syndrome (RSS) is a group of childhood-onset muscle disorders characterized by marked limitation of flexion of the spine. Various cardiac changes have been documented in case reports. This study reports on a cardiac evaluation of nine patients with the "vacuolar variant" of RSS. Noninvasive cardiac evaluation entailed creatine kinase levels, full-inspiration chest roentgenograms, standard 12-lead ECG, and 24-h ambulatory ECG recording, as well as M-mode and two-dimensional echocardiography with Doppler study. Heart auscultation was abnormal in five patients. Creatine kinase MB fraction was normal in all patients. Chest roentgenogram showed scoliosis (five of nine), kyphosis (one of nine), severe anterior-posterior flattening of the chest cavity (two of nine), elevated hemidiaphragm (one of nine), caved-in appearance of upper lobes (two of nine), and symmetry of lung volumes (one of nine). Twelve-lead ECG abnormalities indicated right-sided heart disease (three of nine). Echocardiogram showed mitral valve prolapse (five of nine) with regurgitation (three of five) and evidence of pulmonary hypertension (three of nine). Ambulatory ECG recorded paroxysmal tachyarrhythmias in hypoxic or hypercapnic patients (three of nine). There was no correlation between any cardiac abnormalities and patient weakness. Mitral prolapse/regurgitation may have a developmental association with this congenital myopathy. Findings of cor pulmonale were due to the restrictive chest wall defect and respiratory muscle weakness. Paroxysmal tachyarrhythmias were due to hypoxia or hypercapnia. There was no evidence of a primary cardiomyopathy.
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PMID:Rigid spine syndrome: a noninvasive cardiac evaluation. 1782 62

Polymyositis and dermatomyositis are autoimmune inflammatory myopathies characterized by muscle weakness and inflammation. Current recommended therapy includes corticosteroids as mainstay treatment in addition to immunosuppressant. We present herein a 25year-old female with dermatomyositis and cardiac involvement resistant to disease modifying anti-rheumatic drugs and anti-tumor necrosis factor-alpha. She was treated with anti-CD20 monoclonal antibody, rituximab. The patient demonstrated a remarkable clinical and laboratory response. B-cell depletion therapy with rituximab may be a viable option in patients with dermatomyositis and heart disease.
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PMID:Successful treatment of cardiac involvement in dermatomyositis with rituximab. 1798 19

The acute management of strokes in children with infective endocarditis is limited by the paucity of published data on their clinical course and outcomes. Our retrospective study at an urban tertiary-care academic center characterized the clinical course of seven pediatric patients with endocarditis and subsequent cerebral infarcts. Among 115 patients with endocarditis, a stroke occurred in seven. Four patients manifested congenital heart disease. In six patients, the stroke occurred in the distribution of the middle cerebral artery, with no preference for the left or right hemisphere. The most common presenting sign was focal weakness. Three patients manifested mycotic aneurysms, all of which were successfully repaired. Two patients received aspirin therapy, with no adverse effects. All patients survived, but neurologic recovery was variable. The two youngest patients (aged 3 and 14 weeks) demonstrated the longest periods of hospitalization, with the most severe neurologic impairment. These findings suggest that children may have better outcomes than adults after a stroke secondary to bacterial endocarditis. Routine surveillance for mycotic aneurysms in patients with new neurologic deficits, and the use of aspirin, should be considered in the medical management.
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PMID:Pediatric endocarditis and stroke: a single-center retrospective review of seven cases. 1835 1

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease, generally treated with total correction within the first two years of life. Occasionally, some unoperated cases can reach older ages. A 68-year-old woman with diabetes mellitus presented with swelling in legs and abdomen, weakness, exertional dyspnea, and orthopnea. On physical examination, she had mild cyanosis with clubbing. Her blood pressure was 110/60 mmHg and pulse rate was 79 beat/min. She had a systolic ejection murmur and bilateral rales on basal lung areas. Massive edema was noted in both lower limbs. Electrocardiography showed atrial fibrillation with normal ventricular response. Chest radiography showed an increased cardiothoracic ratio and bilateral minimal pleural effusion. Echocardiography showed biatrial dilatation (right atrium 62 mm, left atrium 49 mm) and thickening of left ventricular walls. There was right ventricular hypertrophy with decreased systolic function. A very large ventricular septal defect and severe pulmonary stenosis were noted. The patient did not accept any interventional procedure. To our knowledge, this is the oldest unoperated TOF case reported from our country.
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PMID:Unoperated tetralogy of Fallot in a 68-year-old patient. 1862 10

Chronic arsenic toxicity (arsenicosis) due to drinking of arsenic contaminated ground water is a major environmental health hazard throughout the world including India. A lot of new information is emerging from extensive research on health effects of chronic arsenic toxicity (CAT) in humans during the last two decades. Available literature has been reviewed to highlight the problem including its malignancies. Pigmentation and keratosis are the specific skin lesions characteristics of CAT. CAT also produces various systemic manifestations over and above skin lesions, important ones being chronic lung disease like chronic bronchitis, chronic obstructive pulmonary disease and bronchiectasis, liver disease like non-cirrhotic portal fibrosis and other diseases like polyneuropathy, peripheral vascular disease, hypertension and ischeamic heart disease, diabetes mellitus, non-pitting oedema of feet/hands, weakness and anaemia. Cancer of skin, lung and urinary bladder are important cancers associated with chronic arsenic toxicity. Stoppage of drinking of arsenic contaminated water is the main stay in the management of arsenicosis as specific chelation therapy has limited value. Early skin cancer, detectable by regular active surveillance, is curable. In addition to dermatological features, CAT produces protean clinical manifestations. Treatment of arsenicosis is unsatisfactory and is mostly symtomatic.
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PMID:Chronic arsenic toxicity & human health. 1910 39

Familial hyperkalemic periodic paralysis (HYPP) is a rare genetic disorder in which the sodium channels in skeletal muscle cells have altered structure and function. Small elevations in serum potassium lead to inactivation of sodium channels, causing episodic weakness or paralysis. Exposure to cold, anesthesia, fasting, emotional stress, potassium ingestion, and rest after exercise can stimulate an attack. This case report describes a 65-year-old man with HYPP who was admitted for a right total knee arthroplasty. He had a history of arteriosclerotic heart disease and stenting 8 years earlier, previous inferior wall myocardial infarction with ejection fraction of 65%, anxiety, degenerative joint disease, well-controlled type 2 diabetes mellitus, and a body mass index of 53.3 kg/m2. A combined spinal/general anesthetic with a femoral nerve block for postoperative pain control was chosen. Careful attention was given to monitoring and maintenance of core temperature, use of insulin and glucose to maintain normokalemia, and carbohydrate loading the night before surgery. The patient recovered from the anesthetic without complication and had pain relief for approximately 22 hours postoperatively because of the femoral nerve block. The patient was without weakness or paralysis related to HYPP in the postanesthesia care unit or throughout his hospitalization.
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PMID:Combined spinal/general anesthesia with postoperative femoral nerve block for total knee replacement in a patient with familial hyperkalemic periodic paralysis: a case report. 2057 4

This case report describes congestive heart failure with pleural effusion in two middle-aged, pet house rabbits. Both had a history of acute onset dyspnoea, weakness and weight loss. Bi-atrial enlargement was seen on echocardiography in both rabbits. One rabbit had atrial fibrillation and ventricular premature complexes identified on electrocardiography. There was a radiographically evident pleural effusion in both rabbits and thoracocentesis was undertaken in one rabbit. These findings were confirmed on post-mortem examination. The aetiology for the underlying heart disease was not found, but the potential types of cardiomyopathies are discussed.
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PMID:Congestive heart failure in two pet rabbits. 2117 25


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