Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The CardioWest TAH was created and initially tested at the same time as the Thoratec, Novacor, and HeartMate devices. It was designed as a permanent artificial heart and was the first-ever mechanical circulatory device to be used as destination therapy. Twenty years have passed since that early experience. Pneumatic technology is still current and being developed as in existing or new implantable Thoratec VADs the pneumatic HeartMate, and the Abiomed BVS 5000 pumps. Portable pneumatic drivers have been available since 1982, and in recent times have allowed discharge to home of substantial numbers of patients, thus reducing the length of hospital stays and making mechanical device support less expensive to society and more tolerable to patients. Within months, a portable driver for the CardioWest will be available. The documented benefits of the CardioWest TAH include rescue of: critically ill patients with advanced heart failure; patients with biventricular failure especially those with significant right heart failure, elevated pulmonary vascular resistance, or pulmonary edema; patients with renal or hepatic failure secondary to low cardiac output; patients with massive myocardial damage such as those with post-\infarction VSD or irreversible cardiac graft rejection; patients with mechanical valves or native valve disease; and patients with intractable arrhythmias and heart failure. High device outputs with restoration of normal filling pressures result in high perfusion pressures that have led to dramatic recoveries, convalescence, and return to levels of activity compatible with normal life. The average device output with the CardioWest TAH is higher than any other approved or investigational device. The reason for this resides in design simplicity this device has the shortest and largest inflow pathway. Stroke, in the authors' own series, is rare with a linearized rate of 0.068 events per patient year. If the experiences of La Pitie and the University of Arizona are combined, there has been one stroke in 25 patient years (0.04 events/patient year). Serious infections have been rare (12% of patients). No clinical mediastinitis has occurred. Drivelines have healed in tightly and never caused an "ascending" infection. There has not been a case of device endocarditis. Using a broad definition of bleeding, including takeback reoperation for bleeding, bleeding more than 8 units in the first postoperative 24 hours or 5 units over any other 48-hour period, a 25% to 36% incidence has been documented. No cases of fatal exsanguination have resulted, as there have been with the HeartMate. The incidence of bleeding as an adverse event is about 17% lower than the rate reported for the HeartMate VE LVAD, and it is about the same as that reported for Novacor and for Thoratec. Implantation of this device is relatively easy and often done (with attending help) by the authors' residents. If one follows the guidelines for fitting the device, and takes the recommended advice for implantation, hemostasis is excellent and restoration of immediate cardiac function with high flows is nearly automatic. Use of a neopericardium of 0.1 mm EPTFE at the time of implantation assures atraumatic and relatively quick re-entry for transplantation and prevents the normal inflammatory mediastinal reaction that might be desirable in a destination application. In selected patients the CardioWest TAH is the device of choice for bridge to transplantation. When a portable driver becomes available, out of hospital management of CardioWest TAH patients will be feasible and consideration of use of this device for longer term applications, (e.g., "destination therapy,") will be reasonable. A wearable driver, even smaller than a portable, will improve quality of life and expand the patient population that may be therapeutically served with this system. In short, the CardioWest TAH has come nearly full circle. It was first used as a destination device. It has since been used as a bridge to transplantation in nearly 200 patients as the Jarvik-7/Symbion TAH and, since 1993, in over 225 patients as CardioWest. The results have improved with time. Thromboembolism and infection rates have been competitive with currently available devices. Device reliability and durability have been excellent. Survival rates have been very high in a group of perhaps the sickest patients to be supported with any pulsatile device. Pneumatic technology has improved with portability and miniaturization, and there is reason to believe that it will become even better. Application of modern manufacturing techniques to this very simple device raises the possibility of significant manufacturing cost reduction, in an era of prohibitive cost for other devices. All of this establishes the CardioWest as a valuable device for any program that is seriously interested in end-stage heart disease and a likely device for permanent use in appropriately selected patients.
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PMID:Total artificial hearts: bridge to transplantation. 1279 49

Diabetes mellitus is a risk factor for congestive heart failure. Diabetics with congestive heart failure should have good glycemic control, treatment of hypertension and dyslipidemia, and treatment with diuretics, angiotensin-converting enzyme inhibitors, and beta blockers as well as digoxin, if the left ventricular ejection fraction is abnormal. Patients with chronic obstructive pulmonary disease may have left ventricular failure because of a coexistent cardiac disorder or right ventricular failure from pulmonary hypertension. An acute respiratory tract infection may precipitate right ventricular failure and should be treated. Alveolar hypoxia should be corrected by improving alveolar ventilation through relieving airflow obstruction with bronchodilators and by increasing inspired oxygen concentration. Loop diuretics should be used cautiously. Beta blockers may be given to patients with chronic obstructive pulmonary disease and left ventricular failure if bronchospasm is not present. Angiotensin-converting enzyme inhibitors should be used to treat left ventricular failure. Digitalis should not be used in patients with right ventricular failure due to chronic obstructive pulmonary disease. Nonsteroidal anti-inflammatory drugs are contraindicated in patients with congestive heart failure. There are controversial data about the negative interaction between aspirin and angiotensin-converting enzyme inhibitors in patients with congestive heart failure. Patients with arthritis and congestive heart failure needing large doses of aspirin for pain relief may be treated instead with acetaminophen, tramadol, or Percocet if necessary for chronic severe pain.
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PMID:Treatment of heart failure in older persons. Dilemmas with coexisting conditions: diabetes mellitus, chronic obstructive pulmonary disease, and arthritis. 1282 72

RADIOCHROMIUM HAS THESE ADVANTAGES FOR THE MEASUREMENT OF WHOLE BLOOD VOLUME: it remains in the erythrocytes many hours; it can be measured easily and accurately; the amount of radiation from it is very low.As measured by the radiochromium method, the whole blood volume of normal patients was determined to be 65.6 cc. +/- 5.95 cc. per kilogram of body weight or 2.49 +/- 0.28 liters per square meter of body surface. In a majority of a series of patients with heart disease, hypervolemia was found during right ventricular failure but not in those having left ventricular failure or mitral stenosis alone.
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PMID:Blood volume in cardiac decompensation; determinations by use of radiochromium. 1319 3

Primary pulmonary hypertension (PPH) is characterised by sustained elevations of pulmonary arterial pressure without a demonstrable cause, leading to right ventricular failure and death. Hereditary mutations in the bone morphogenetic protein receptor type II (BMPR2) gene result in familial PPH transmitted as an autosomal dominant trait, albeit with low penetrance. The causes in cases without a BMPR2 mutation are unknown, but a syndrome of pulmonary arterial hypertension (PAH) similar to hereditary PPH is associated with systemic connective tissue disease, congenital heart disease, portal hypertension, and human immunodeficiency virus infection, or with the use of appetite-suppressant drugs. The authors identified a BMPR2 gene mutation in a 27-yr-old female who developed PAH after a short course of the appetite-suppressant drug amfepramone (diethylpropion). This allowed molecular genetic counselling and prevention of potentially harmful drug exposure in the patient's son treated for attention deficit disorder with methylphenidate, an amphetamine-related drug. No BMPR2 mutation was found in four additional, unrelated patients with appetite suppressant-related PPH. The findings provide strong evidence that amfepramone can trigger primary pulmonary hypertension in a bone morphogenetic protein receptor type II gene mutation carrier, and indicate that other genes are probably implicated in genetic susceptibility to appetite suppressants.
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PMID:Primary pulmonary hypertension after amfepramone (diethylpropion) with BMPR2 mutation. 1517 99

A 68-year old woman was hospitalised because of isolated right heart failure. Doppler echocardiography revealed severe tricuspid regurgitation with thickened, shortened, hypomobile leaflets. Pulmonary valve was thickened with mild pulmonary regurgitation. Mitral and aortic valves were normal. The patient was finally diagnosed with carcinoid heart disease from an isolated ovarian carcinoid cancer without hepatic metastases. Ovarectomy was performed and the patient was considered cured of her cancer. Because of refractory right heart failure, she underwent tricuspid valve replacement with a bioprosthesis. Such cardiovascular manifestations are rarely the presenting symptoms of carcinoid disease. Carcinoid heart disease from ovarian primary cancer is exceptional. In this circumstance, carcinoid cardiac lesions may develop in the absence of hepatic metastases because the venous blood from the ovaries drains into the inferior vena cava without hepatic first past effect. Surgical resection of primary ovarian carcinoid tumor is often curative and the prognosis depends mainly on the cardiac condition. The diagnosis of carcinoid syndrome should be discussed in patients with organic tricuspid regurgitation without left valvular disease.
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PMID:[Isolated carcinoid tumor of the ovary disclosed by tricuspid insufficiency]. 1462 39

Indications for catheter-based interventions in patent ductus arteriosus (PDA)-dependent pulmonary blood flow have yet to be defined. The aim of this study was to assess the acute and midterm outcome after stent implantation in the PDA. Between 1996 and 2002, ductal stenting was performed in 21 neonates and infants (14 females/7 males) to maintain pulmonary blood supply in cyanotic congenital heart disease (CHD). Balloon-expandable stents were implanted in the PDA with a final diameter of 4-5 mm without procedural deaths. Stent patency was achieved for 8-1,130 days (median, 142). Reintervention was necessary in nine patients. Overall survival rate after 6 years was 86%. Two neonates died a few days after the procedure due to right heart failure not related to PDA stenting. Corrective surgery was possible in six patients. An additional aortopulmonary shunt was needed in three patients; Fontan type operations were performed in six. One patient died after bidirectional Glenn shunt, another five reached palliation by additional perforation of the atretic valve/balloon valvuloplasty, and two are awaiting surgery. We conclude that in many patients with cyanotic CHD, especially in those with ductal pulmonary perfusion and additional forward flow from the right ventricle, ductal stenting is an effective transcatheter approach. Morphology of the PDA predicts the risk of restenosis and necessity of reintervention. Growth of the pulmonary vascular bed allows corrective or palliative surgery, and some patients can be cured by the intervention alone.
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PMID:Stent implantation in the ductus arteriosus for pulmonary blood supply in congenital heart disease. 1475 21

Pulmonary arterial hypertension (PAH) is a rare condition characterised by elevated pulmonary arterial resistance leading to right heart failure. PAH can be sporadic (idiopathic PAH, or primary pulmonary hypertension), familial (caused by germline BMPR2 mutations, a type II member of the TGFbeta receptor superfamily), or related to other conditions including connective tissue disease, congenital heart disease, human immunodeficiency virus infection, portal hypertension, appetite suppressant exposure... Idiopathic PAH has a prevalence of 2 per million per year in France. The lack of specificity of PAH symptoms (mostly dyspnea) presumably lead to underdiagnosis of this condition. Echocardiography is the investigation of choice for non-invasive screening. Measurement of hemodynamic parameters during right-heart catheterism is mandatory to establish the diagnosis (mean pulmonary artery pressure >25 mmHg and pulmonary artery wedge pressure <12 mmHg). Acute pulmonary vasodilator testing should be performed with nitric oxide or prostacyclin during right-heart catheterization. Recent advances in the management of PAH including continuous intravenous prostacyclin infusion and endothelin receptor antagonists have improved markedly the patients' prognosis. Novel treatments such as inhaled iloprost and type 5 phosphodiesterase inhibitors have to be further evaluated in this setting. Lung transplantation is the last option for patients deteriorating despite medical treatment.
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PMID:[Pulmonary arterial hypertension]. 1504 92

Mitral annular velocities are reportedly useful in diagnosing constrictive pericarditis (CP); however, their exact efficacy in larger clinical settings remains unevaluated. This study reexamined the role of longitudinal tissue Doppler imaging in diagnosing CP in clinical practice. Tissue velocity imaging (GE Vingmed System Five) was performed in 122 subjects (87 referred with clinically suspected CP and 35 age- and sex-matched controls). Of the 87 subjects with suspected CP, 45 (51.7%) had CP confirmed at surgery, 11 (12.6%) had restrictive heart disease, 20 (23.0%) had right heart failure due to cor pulmonale, and the other 11 (12.6%) had old pericardial effusions and no hemodynamic evidence of constriction on follow-up echocardiography. Of the 45 patients with CP, mitral early diastolic (Ea) annular velocities from septal and lateral regions were normal (>/=8 cm/s) in 40 (88.9%) and decreased (<8 cm/s) in 1 or both regions in 5 (3 with left ventricular systolic dysfunction, 2 with extensive mitral annular calcification). Of 11 patients with restrictive cardiomyopathy, 8 (72.7%) had reduced Ea (<8 cm/s) and 3 showed normal Ea velocity in 1 or both corners of the mitral annulus. All except 2 patients with right-sided heart failure from cor pulmonale and those with previous pericardial effusion had normal Ea velocities. A normal Ea velocity improved recognition of CP, particularly in the presence of nondiagnostic 2-dimensional or transmitral flow-Doppler imaging. The overall sensitivity and specificity for diagnosing CP using tissue Doppler incrementally with M-mode, 2-dimensional, and transmitral flow Doppler were 88.8% and 94.8%, respectively. Mitral annular velocities help with diagnosis and differentiation of CP in most cases, except in the presence of extensive annular calcification, left ventricular systolic dysfunction, or segmental nonuniformity in myocardial velocities.
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PMID:Accuracy and pitfalls of early diastolic motion of the mitral annulus for diagnosing constrictive pericarditis by tissue Doppler imaging. 1505 Apr 94

Pediatric cardiac transplantation is currently an accepted option for end-stage heart disease and congenital cardiac malformations. This report focuses on the anesthetic perioperative management in 12 yr. From 1988 to 2001 we performed 90 heart transplantations in 88 children, infants and neonates. The pediatric heart transplant program of the children's heart center at our university hospital started in June 1988 with the transplantation of a 2-yr-old boy who was suffering from congenital heart disease. Since then, 88 transplants have been performed. We divided our patients into two groups. Group 1 ranged from 1988 to 1996 and Group 2 from 1997 to 2001. The patient characteristics have not significantly changed over the years in our institution. At the time of transplantation, mean age of the patients was 2.6 +/- 4.3 yr from the period of 1988-1996 and 2.5 +/- 4.1 yr from 1997 to 2001. Since 1988, 90 transplants (Tx) in 88 patients have been performed. Two patients needed re-Tx within 2 days after the initial operation because of primary graft failure. Indications for Tx were congenital heart disease (n = 67) and cardiomyopathy (n = 21). In the subgroup of the patients suffering from congenital heart disease there were 46 with the diagnosis of HLHS, followed by endocardial fibroelastosis (n = 7); the remaining 14 patients had other complex cardiac malformations and some underwent corrective palliative cardiac surgery before Tx. Sixty-three patients were younger than 1 yr of age and only five children were older than 10 yr. Twenty-three percent of the patients on the waiting list died before Tx was possible. The overall survival rate was 79% at 1 yr and 73% at 5 and 10 yr. Infants with HLHS had a lower probability of survival after 5 yr compared with other diagnosis (69% vs. 84%). Until now 21 patients have died after Tx. The duration of anesthesia, time of CPB and the age at the time of surgery decreased over the years. It is always a challenge for the anesthesiologist to treat these patients with pulmonary hypertension as one of the most critical risks in this group of patients. The preventive therapy with vasodilators as well as the availability of mechanical assist devices before and after heart transplantation reduces the effects of transitional pulmonary hypertension and prevents the development of post-operative right heart failure.
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PMID:Perioperative management in pediatric heart transplantation from 1988 to 2001: anesthetic experience in a single center. 1517 60

Eisenmenger syndrome is the most common consequence of congenital cyanotic heart disease seen in adults; survival to the fifth decade of life is rare. Death is very difficult to predict: it is related to sudden cardiac ventricular arrhythmia, massive hemoptysis and right heart failure. In this paper, a patient with ventricular septal defect and Eisenmenger reaction is described. The patient was relatively well until 48 years of age, when she underwent surgery because of a cerebral abscess without cerebral complications but with some deterioration of her cardiac function. After discharge, the patient was readmitted to the hospital because the electrocardiogram showed persistent ST inferior elevation. Echocardiography demonstrated poor contractility and inferior akinesia. Sudden ventricular tachycardia occurred and the patient became unconscious. She was successfully resuscitated and, following a period of ventilation, the hemodynamics stabilized and she was discharged 17 days later. She remained well two years later.
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PMID:Eisenmenger syndrome: a case of survival after ventricular tachycardia due to inferior myocardial infarction in a 48-year-old patient with congenital large ventricular septal defect. 1522 66


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