Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors analyse a series of 46 operated intracardiac tumours (0.4% of all cases of cardiac surgery). The predominant symptoms were episodes of heart failure and pseudo-valvular disease. Peripheral emboli and pericardiac syndromes with tamponade occurred more rarely and constituted surgical emergencies. Non-invasive investigations, dominated by echocardiography, allow early diagnosis. The majority of tumours were situated in the left atrium and consisted, histologically, of myxomas, the surgical treatment of which consists of wide excision of the pedicle. Malignant tumours (17.7%) were dominated by cardiac sarcomas. When possible, extensive resection of these tumours may improve the chances of medium-term and long-term survival. The operative mortality was 8.8%. The delayed mortality was due to malignant tumours (17.3%). Myxomas must be followed in the long-term by systematic echocardiography. The authors observed 11% of recurrences occurring after a interval of 12 to 144 months (3 cases).
 ...
PMID:[Cardiac tumor mass. Diagnostic and therapeutic approach. Apropos of 46 cases]. 218 38

Although cardiac myxomas are histologically benign, they may be lethal because of their strategic position. They can mimic not only every cardiac disease but also infective, immunologic, and malignant processes. Myxomas must therefore be included in the differential diagnosis of valvular heart disease, cardiac insufficiency, cardiomegaly, bacterial endocarditis, disturbances of ventricular and supraventricular rhythm, syncope, and systemic or pulmonary embolism. The symptoms depend on the size, mobility, and location of the tumor. Echocardiography, including the transesophageal approach, is the most important means of diagnosis; CT and MRI may also be helpful. Coronary arteriography in patients over 40 years of age is generally required to rule out concomitant coronary artery disease. Surgical removal of the tumor should be performed as soon as possible; the long-term prognosis is excellent, and recurrences are rare. In follow-up examinations as well, echocardiography is essential.
 ...
PMID:Cardiac myxomas. 861 42

Myxomas rarely are located on the mitral valve. We report the case of a 44-year-old man operated on successfully with mitral valve conservation, which is the 21st case of mitral valve myxoma reported in the western literature. Among the cases reported in the literature, the diagnosis was made at the time of autopsy in 6 cases, with premortem heart failure in 1 case. A clinical diagnosis was made in 15 cases on the basis of the following symptoms: peripheral embolism in 9 cases, cardiac signs in 4 cases, and constitutional manifestations in 2 cases. The clinical presentation of mitral valve myxoma differs slightly from that of other cardiac myxomas in that it has a lower incidence of constitutional manifestations. The gold standard for noninvasive diagnosis and localization of cardiac myxomas is transesophageal echocardiography. It allows for the detection of early, small valvular tumors and may help to characterize better their location and echostructure, facilitating the choice of an optimal surgical approach through preoperative study of the integrity and mobility of the valve. Conservative operative treatment by resection of the area of implantation of the myxoma followed by suture repair of the valve and annuloplasty may be recommended as the most appropriate treatment option for this rare condition.
 ...
PMID:Clinical presentation and treatment options for mitral valve myxoma. 930 3

We report here a tricky case of right atrial myxoma with a pulmonary localization mimicking pulmonary thromboembolism. The diagnosis on imaging investigation was delayed because of its atypical appearance. This case report emphasizes the leading role of transthoracic and transesophageal echocardiography in the management of this condition. In autopsy series, the incidence of primary tumors of the heart is evaluated at 0.0017% to 0.19%.(1) Nearly half of them are myxoma.(1, 2) Myxoma are more frequently observed in adults and are commonly localized in the left atrium. Signs and symptoms are comparable to those arising in other cardiovascular and systemic conditions, including variable cardiac murmur, uneasiness, blackout, systemic embolism, cardiac insufficiency, lasting fever, or sudden death.(3) Rare cases of pulmonary embolism have been described. We report here an atypical case of right atrial myxoma with a pulmonary localization mimicking pulmonary embolus.
 ...
PMID:Fragmentation of a right atrial myxoma presenting as a pulmonary embolism. 1062 33

We analyzed a series of 112 consecutive cases of left atrial myxoma diagnosed in a single French hospital (72 women and 40 men; age range, 5-84 yr) over 40 years, from 1959 to 1998. Symptoms of mitral valve obstruction, the first arm of the classic triad of myxoma presentation, were present in 75 patients (67%), with mostly cardiac failure or malaise. Symptoms of embolism, the second frequent presentation in the classic triad, were observed in 33 cases (29%) with 1 or several locations, essentially cerebral emboli with stroke. Males are statistically at greater risk than females of developing embolic complications. The third arm of the classic triad consists of constitutional symptoms (34%) with fever, weight loss, or symptoms resembling connective tissue disease, due to cytokine (interleukin-6) secretion. Younger and male patients have more neurologic symptoms, and female patients have more systemic symptoms. Seventy-two patients (64%) had cardiac auscultation abnormalities, essentially pseudo-mitral valve disease (53.5%) and more rarely the suggestive tumor plop (15%). The most frequent electrocardiographic sign was left atrial hypertrophy (35%), whereas arrhythmias were uncommon. The greater number of myxoma patients (98) diagnosed preoperatively after 1977 reflects the introduction of echocardiography as a noninvasive diagnostic procedure. However, there was no significant reduction in the average time from onset of symptoms to operation between patients seen in the periods before and after 1977. The tumor diameter ranged from 1 to 15 cm with a weight of between 15 and 180 g (mean, 37 g). The myxoma surface was friable or villous in 35% of the cases, and smooth in the other 65% cases. Myxomas in patients presenting with embolism have a friable surface; those in patients with cardiac symptoms, pseudo-mitral auscultation signs, tumor plop, and electrocardiogram or radiologic signs of left atrium hypertrophy and dilatation are significantly the larger tumors. The long-term prognosis is excellent, and only 4 deaths occurred among our 112 cases over a median follow-up of 3 years. The recurrence rate is low (5%), but long-term follow-up and serial echocardiography are advisable especially for young patients.
 ...
PMID:Clinical presentation of left atrial cardiac myxoma. A series of 112 consecutive cases. 1138 92

We describe two cases of cardial myxomas. The first had a rare location in the left ventricle presenting with cardiac arrest because of left ventricular outflow obstruction. The other myxoma has a typical localisation in the left atrium with symptoms of heart failure and syncope because of mitral valve occlusion. Myxomas are rare benign tumours with a wide spectrum of symptoms from asymptomatic to very serious, depending of the localisation of the tumours in relation to the heart valves. Eccocardiography is the examination of choice when myxoma is suspected. The treatment and way to final diagnostic is surgery.
 ...
PMID:[Two cases of cardial myxomas with severe course]. 1882 6

The Carney complex is an inherited tumor predisposition caused by activation of the cAMP-dependent protein kinase [protein kinase A (PKA)] resulting from mutation of the PKA-regulatory subunit gene PRKAR1A. Myxomas and tumors in cAMP-responsive tissues are cardinal features of this syndrome, which is unsurprising given the important role played by PKA in modulating cell growth and function. Previous studies demonstrated that cardiac-specific knockout of Prkar1a causes embryonic heart failure and myxomatous degeneration in the heart, whereas limited Schwann cell-specific knockout of the gene causes schwannoma formation. In this study, we sought to determine the role of PKA activation in this phenotype by using genetic means to reduce PKA enzymatic activity. To accomplish this goal, we introduced null alleles of the PKA catalytic subunits Prkaca (Ca) or Prkacb (Cb) into the Prkar1a-cardiac knockout (R1a-CKO) or limited Schwann cell knockout (R1a-TEC3KO) line. Heterozygosity for Prkaca rescued the embryonic lethality of the R1a-CKO, although mice had a shorter than normal lifespan and died from cardiac failure with atrial thrombosis. In contrast, heterozygosity for Prkacb only enabled the mice to survive 1 extra day during embryogenesis. Biochemical analysis indicated that reduction of Ca markedly reduced PKA activity in embryonic hearts, whereas reduction of Cb had minimal effects. In R1a-TEC3KO mice, tumorigenesis was completely suppressed by a heterozygosity for Prkaca, and by more than 80% by heterozygosity for Prkacb. These data suggest that both developmental and tumor phenotypes caused by Prkar1a mutation result from excess PKA activity due to PKA-Ca.
 ...
PMID:Differential role of PKA catalytic subunits in mediating phenotypes caused by knockout of the Carney complex gene Prkar1a. 2185 54

Myxomas are the most common cardiac neoplasm accounting for 50% of all tumours. Usually symptomatic at diagnosis, the advent of routine echocardiogram in clinical practice has enabled earlier diagnosis before onset of symptoms. There however have been few reports in black Africans, and so we are reporting a male patient who presented with heart failure secondary to a large left atrial myxoma mimicking mitral stenosis from diastolic flow obstruction across the valve. He underwent successful resection and to the best of our knowledge is the first reported resected surgical case in Nigeria.
 ...
PMID:Left atrial myxoma: case report and literature review. 2496 94

Cardiac myxomas are the most frequently encountered tumors of the heart. However, they are unusual to be newly diagnosed in the geriatric population. Myxomas are among the great mimickers, with a myriad of clinical presentations related to heart failure, embolic events, and constitutional symptoms. We describe a rare case of a giant atrial myxoma in a nonagenarian presenting with heart failure, which was medically managed.
 ...
PMID:Medical management of a suspected atrial myxoma in a nonagenarian. 3268 52