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

Whilst the pulmonary valve is a tricuspid valve, very few reports exist of bicuspid pulmonary valves, the majority of which are associated with congenital heart disease. Isolated bicuspid valves not associated with congenital abnormalities are even more rare. Herein, the case is described of a 65-year-old man who died from a stroke, and in whom a bicuspid pulmonary valve was found during post-mortem dissection for tissue donation.
 ...
PMID:Isolated bicuspid pulmonary valve: an unusual finding. 1522 1

Piercing and tattooing enjoy widespread popularity in modern society. Patients with congenital heart disease are at elevated risk for infective endocarditis. However, it is not yet known whether piercing and tattooing are dangerous for these patients.A search of the literature provided 10 published cases of infective endocarditis after piercing or tattooing. Affected patients were adolescents or young adults ranging in age from 13 to 30 years (5 male, 5 female). Four of the patients had a known cardiac risk factor for endocarditis (bicuspid aortic valve, postoperative trans-position of the great arteries, postoperative coarctation, postoperative aortic valve stenosis). Piercing preceded endocarditis in 9 cases (4 times mouth, 2 ear, 1 nose, 1 breast, 1 navel), one tattoo. The following agents were isolated: S. aureus in 4 cases, 2 S. epidermidis, 1 Str. viridans, 1 Neisseria mucosa, 1 Haemophilus aphrophilus, 1 Haemophilus parainfluenzae. All patients were treated with antibiotics. Six patients underwent cardiac surgery (5 of them valve replacement). Patients with congenital heart disease constitute less than 1% of the population. Thus, they are clearly overrepresented in the published literature. Epidemiologic conclusions are not possible from these data. However, patients with congenital heart disease and their parents should be strongly advised against piercing and tattooing with regard to the risk of infective endocarditis.
 ...
PMID:[Piercing and tattoos in patients with congenital heart disease -- is it a problem?]. 1533 48

A 44-year-old woman had tako-tsubo-like ventricular dysfunction with chest pain and ST segment elevation on the ECG. Echocardiography revealed a bicuspid aortic valve with moderate to severe aortic regurgitation. She developed mild heart failure during the clinical course, but the medication (furosemide, enalapril, and asprin) had to be stopped because of skin eruptions. Four weeks after ceasing the antiplatelet agent, she was re-admitted with acute renal infarction. Enhanced chest computed tomography revealed a filling defect in the left ventricle and echocardiography showed a high echogenic mass in the left ventricular apical wall. These findings strongly suggested that the renal infarction was caused by an embolism derived from a left ventricular thrombus that formed during the clinical course of the transient left ventricular apical ballooning. Anticoagulation therapy with urokinase and warfarin successfully lysed the thrombus. Left ventricular thrombus should be considered a complication of transient left ventricular apical ballooning, especially in patients with organic heart disease.
 ...
PMID:Transient left ventricular apical ballooning in a patient with bicuspid aortic valve created a left ventricular thrombus leading to acute renal infarction. 1550 92

The population of patients with adult congenital heart disease is approximately 800,000 in the U.S. Those with prior cardiac surgery often consider themselves "cured," although the majority faces a lifetime of problems including arrhythmias, ventricular dysfunction, and one or more re-operations. Even patients with repaired "simple" lesions such as an atrial septal defect may not have normal survival if they are repaired in adulthood. Patients with repaired coarctation may have premature cardiovascular complications including sudden cardiac death, myocardial infarction, and stroke. They also have aortic complications such as aneurysm and dissection, which result from a diffuse arteriopathy and continued hypertension that may be caused by underlying endothelial dysfunction. In addition, bicuspid aortic valve occurs in more than one-half of the patients with coarctation, so continued surveillance for significant aortic valvular heart disease is necessary. More complex lesions also pose problems after "total correction." Patients with repaired tetralogy of Fallot often have pulmonary regurgitation, which is frequently overlooked on clinical exam and echocardiography. Pulmonary valve replacement should be performed before the development of irreversible right ventricular dysfunction and an increased risk of ventricular tachycardia or sudden cardiac death. Because they are vulnerable to deterioration of systemic ventricular function, those with congenitally corrected transposition require special vigilance, usually with concomitant atrio-ventricular valve regurgitation. Late referral is common with a deleterious effect on long-term survival. These patients need lifelong follow-up and the residua and sequelae of their complex anomalies must be understood in order to provide optimum care.
 ...
PMID:The adult with congenital heart disease: born to be bad? 1599 27

Calcification of the aortic valve is the third leading cause of heart disease in adults. The incidence increases with age, and it is often associated with a bicuspid aortic valve present in 1-2% of the population. Despite the frequency, neither the mechanisms of valve calcification nor the developmental origin of a two, rather than three, leaflet aortic valve is known. Here, we show that mutations in the signalling and transcriptional regulator NOTCH1 cause a spectrum of developmental aortic valve anomalies and severe valve calcification in non-syndromic autosomal-dominant human pedigrees. Consistent with the valve calcification phenotype, Notch1 transcripts were most abundant in the developing aortic valve of mice, and Notch1 repressed the activity of Runx2, a central transcriptional regulator of osteoblast cell fate. The hairy-related family of transcriptional repressors (Hrt), which are activated by Notch1 signalling, physically interacted with Runx2 and repressed Runx2 transcriptional activity independent of histone deacetylase activity. These results suggest that NOTCH1 mutations cause an early developmental defect in the aortic valve and a later de-repression of calcium deposition that causes progressive aortic valve disease.
 ...
PMID:Mutations in NOTCH1 cause aortic valve disease. 1602

The ascending aorta or pulmonary trunk in congenital heart disease may dilate out of proportion to hemodynamic or morphogenetic expectations, may become aneurysmal, and may rupture. A bicuspid aortic valve and/or coarctation of the aorta are consistently associated with ascending aortic and para-coarctation medial abnormalities. Congenital heart diseases such as single ventricle, truncus arteriosus, transposition of the great arteries and tetralogy of Fallot are also associated with aortic medial abnormalities. Aortic regurgitation in unrepaired tetralogy of Fallot imposes volume overload on both ventricles. A significant subset of adults late after repair of tetralogy of Fallot exhibits progressive aortic root dilatation which may lead to regurgitation and predispose to dissection and rupture which can be fatal, and necessitating aortic valve and aortic root surgery. The aortic dilatation relates medial abnormalities coupled with previous long-standing volume overload of the ascending aorta. Risk factors for aortic dilatation and regurgitation in tetralogy of Fallot relate to specific hemodynamic abnormalities such as pulmonary atresia, right aortic arch and a history of an aorto-pulmonary shunt, and patient demographics such as male sex and the association of chromosome 22q11 deletion. There is no current consensus on beta-blocker administration for limiting progressive dilatation of the aortic root in patients with congenital heart disease and repaired tetralogy of Fallot. Aortic root surgery should be considered for these patients and address aortic regurgitation and or prevent the risk of aortic dissection. Meticulous follow-up of the aortic root after repair, tetralogy of Fallot are thus recommended.
 ...
PMID:Aortic root dilatation in tetralogy of Fallot long-term after repair--histology of the aorta in tetralogy of Fallot: evidence of intrinsic aortopathy. 1608 Sep 67

The use of valvuloplasty in low birth weight infants with valvular aortic stenosis has not been widely reported. We report a 1556 g 3-day-old female infant with complex heart disease including critical valvular aortic stenosis, bicuspid aortic valve, mitral stenosis, patent ductus arteriosus, and secundum type atrial septal defect. She also had esophageal atresia with a distal tracheoesophageal fistula and trisomy 18. Balloon valvuloplasty was considered to be more dangerous in this patient than in infants with simple critical aortic stenosis without other associated anomalies or low birth weight. Retrograde aortic balloon valvuloplasty was done carefully with the patient intubated and mechanically ventilated. We successfully dilated the stenotic aortic valve, which resulted in a dramatic decrease of the left ventricular pressure from 139/12 mm Hg to 81/11 mm Hg. Despite the high operative risk and poor prognosis, balloon valvuloplasty may be the first feasible step towards the survival of low birth weight infants with valvular aortic stenosis.
 ...
PMID:Balloon valvuloplasty for critical aortic stenosis in a low birth weight baby with tracheoesophageal fistula. 1609 32

Hypoplastic left heart syndrome (HLHS) accounts for nearly 25% of deaths among neonates with congenital heart disease. The essential feature of HLHS is a small left ventricle (LV) incapable of supporting the circulation. The etiology of HLHS is unknown. A hypothesis is proposed implicating an immune mechanism involving maternal antibodies produced in response to pharyngitis caused by group A beta-hemolytic streptococci (GABHS) ("strep throat"). After crossing the placenta, the antibodies injure the developing fetal heart, leading to HLHS either because of direct injury to the LV or secondary to reduced blood flow through affected aortic and mitral valves. Analogy is drawn to rheumatic heart disease (RHD), a known sequela of strep throat. In RHD a misdirected immune response originally intended for GABHS leads to cardiac injury through "molecular mimicry"; the normal heart antigens supposedly mimic the GABHS antigens. A similar pathogenesis is proposed for HLHS and related heart defects. HLHS may represent an extreme form of injury, while a milder insult may present as only mild aortic stenosis or a bicuspid aortic valve, conditions with wide prevalence among the general population. The injury may indeed superimpose on many other congenital heart defects, leading to a variable presentation of these other diseases. Beside remarkable likenesses between HLHS and RHD, the hypothesis is also supported by increasing evidence for the role of deleterious transplacental antibodies in the pathogenesis of other fetal diseases. Implications for other congenital heart diseases and the broader picture of global public health are discussed.
 ...
PMID:Hypoplastic left heart syndrome: Rheumatic heart disease of the fetus? 1624 53

From 1988 to 2005, seven patients were operated at our hospital because of infectious endocarditis (IE) with congenital heart disease (CHD). Underlying CHD included ventricular septal defect (VSD) in 4 (2 previous operations with residual region), atrial septal defect (ASD) in 2 and bicuspid aortic stenosis (AS) in 1. No cases had preventive antibiotic prophylaxis for dental procedures. We could confirm bacteria origin from blood culture in all cases, but two patients had operations without gaining control of the infection. VSD or ASD closure and valve surgery were performed in four patients. One patient had a VSD closure, two patients had valve surgery. There were no operative or hospital deaths and there were no recurrences of IE during the study period. We successfully treated IE with CHD by enough debridement of the infective focus of IE, and valve surgery. It is important for patients with CHD to have preventive antibiotic prophylaxis for dental procedures.
 ...
PMID:Infective endocarditis with congenital heart disease. 1689 44

Bicuspid aortic valve is the most common form of congenital heart disease. It is associated with both valvular pathology and aortic disease. Aortic regurgitation caused by a bicuspid aortic valve can be corrected by surgical valve repair, which has good short-term results. However, the significant rate of aortic regurgitation recurrence found on long-term follow-up remains a problem, partly because of the progressive aortic dilatation that is characteristic of this disease. We report three different cases of bicuspid aortic valve treated by surgical valve repair at our centre.
 ...
PMID:[Valve repair for bicuspid aortic valve regurgitation: an option?]. 1733 88


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>