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

Tetralogy of Fallot with absent pulmonary valve (TOF/APV) is a rare form of congenital heart disease with a high risk of perinatal mortality, particularly when diagnosed before birth. We aimed to assess whether global left and right ventricular function in fetal TOF/APV, using the Tei index, correlate with outcome. We reviewed the fetal echocardiogram and clinical outcome of eight fetuses diagnosed with TOF/APV. Of the eight cases, four developed cardiovascular compromise, leading to intrauterine death in two fetuses and neonatal demise in two fetuses, and four fetuses survived the neonatal period. The right ventricular (RV) Tei index was significantly greater and the left ventricular (LV) Tei index tended to be greater in nonsurvivors compared with survivors with TOF/APV (RV Tei, 0.90 +/- 0.17 versus 0.30 +/- 0.28, p < 0.05; LV Tei, 0.97 +/- 0.42 versus 0.54 +/- 0.21). The global LV and RV function can be affected in TOF/APV. Furthermore, more severe pulmonary insufficiency and worse biventricular function as assessed by Tei index likely contribute to the high perinatal mortality associated with this disease.
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PMID:Left and right ventricular function in fetal tetralogy of Fallot with absent pulmonary valve. 1590 13

The clinical presentation of right ventricular (RV) dysfunction due to congenital heart disease (CHD) is similar to that of cor pulmonale. RV volume and pressure loads, and primary RV myocardial dysfunction are mechanisms by which CHD affects right heart function. RV volume load may arise from pre-tricuspid left to right shunts (e.g., atrial septal defect) or regurgitant lesions in the right heart (e.g., Ebstein's anomaly of the tricuspid valve and pulmonary insufficiency after repair of tetralogy of Fallot). RV pressure load may be caused by anatomic obstruction to RV outflow and by pulmonary arteriolar hypertension. The latter can result from Eisenmenger syndrome secondary to congenital and postoperative left to right shunts or from defects that cause pulmonary venous hypertension (e.g., pulmonary vein stenosis, cor triatriatum, or mitral stenosis). The RV myocardium may be affected by a primary cardiomyopathy or by congenital abnormalities of the coronary vessels. Finally, CHD may be associated with airway obstruction, scoliosis, or pulmonary emboli, which, in turn, may lead to the development of cor pulmonale. Congenital heart disease, therefore, must be included in the differential diagnosis of patients who present with right ventricular dysfunction.
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PMID:The right heart in congenital heart disease. 1608 51

Congenital aortic stenosis is a relatively common cardiac anomaly encountered in approximately 5% of all children with heart disease. The Ross procedure is increasingly used for replacement of the aortic valve in children. We report a 12-year-old boy who was born with congenital aortic stenosis secondary to a bicommissural aortic valve. The patient underwent open valvotomy in infancy and aortic valvuloplasty 2 years later. Residual/recurrent stenosis prompted referral for aortic valve replacement, and he underwent an autologous Ross procedure, in which the aortic root was replaced with a pulmonary autograft and the repaired aortic valve was used to restore right ventricular-to-pulmonary artery continuity. The postoperative course was unremarkable. Nitroprusside, esmolol, and labetolol were used to control postoperative hypertension. He was discharged 4 days after surgery on oral furosemide and aspirin, and he has had no cardiovascular symptoms during follow-up. Recent echocardiography demonstrated mild right ventricular outflow tract obstruction with a peak velocity of 3.6 m/sec, with a gradient of 42 mmHg and moderate pulmonary insufficiency. There was no left ventricular outlet tract obstruction or aortic insufficiency.
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PMID:Autologous Ross operation for congenital aortic stenosis. 1703 18

Long-term hyperserotoninemia induces heart valve disease in rats, and cases of cardiac valvulopathies have been reported in patients using ergolines, possibly through activation of the 5-hydroxytryptamine(2B) (5HT(2B)) receptor. The ergoline terguride (transdihydrolisuride) is a 5HT(2B/2C) receptor antagonist. Using a rat model, we have investigated whether terguride could prevent serotonin-induced changes in general and heart disease specifically. During 4 months, twelve Sprague-Dawley rats were given daily subcutaneous serotonin injections; twelve rats received a combination of serotonin injections and terguride by gavage, whereas ten rats were untreated controls. Using echocardiography, rats with aortic insufficiency were found in all 3 groups, while pulmonary insufficiency was only found in two rats injected with serotonin alone. Animals given serotonin alone had significantly higher heart weights compared to the controls (p=0.029) and rats given terguride (p=0.034). Rats injected with serotonin alone developed macroscopic skin changes at the injection sites, histologically identified as orthokeratosis and acanthosis. Terguride completely prevented these changes (p=0.0001, p=0.0003). Liver weights were higher in the animals given serotonin alone compared to controls (p=0.014) and terguride treated animals (p=0.009). Stomach weights were higher in animals given serotonin alone compared to rats given terguride (p=0.012). In the mesenchymal cell-line MC3T3-E1, terguride almost completely inhibited serotonin-induced proliferation (p<0.01). Serotonin increases heart, liver and stomach weights, possibly through enhanced proliferation. Terguride inhibits these effects. We propose that terguride may have beneficial effects in the treatment of diseases such as carcinoid syndrome, where serotonin plays an important pathogenic role.
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PMID:Long-term serotonin effects in the rat are prevented by terguride. 1739 82

We determined the utility of continuous wave (CW) Doppler for quantification of pulmonary insufficiency (PI) confirmed by pulmonary angiography in patients with postoperative adult congenital heart disease. A total of 41 patients with PI were divided into two groups on the basis of PI severity by pulmonary angiography: group A (n = 27) with severe PI and group B (n = 14) with mild or moderate PI. Nine patients in group A had pulmonic valve replacement and reverted to mild PI after surgery. Their pre- and postoperative data were compared. All underwent a two-dimensional/Doppler study with interrogation of the PI jet for jet width by color Doppler and peak flow velocity, deceleration time (DT), pressure half-time (PHT), diastolic period (DP), and PI flow time (FT) by CW Doppler. The no-flow time (NFT), NFT/FT ratio, and NFT/DP fraction were calculated. Group A had a larger right ventricle (4.1 +/- 0.9 vs. 3.5 +/- 0.6 cm, P = .033), higher PI peak velocity (2.1 +/- 0.5 vs. 1.7 +/- 0.5 m/s, P = .04), shorter DT (261 +/- 61 vs. 317 +/- 83 ms, P = .018) and PHT (76 +/- 29 vs. 132 +/- 53, P < .0001), longer NFT (146 +/- 66 vs. 40 +/- 42 ms, P < .0001), and higher ratios of NFT/FT (46% +/- 27% vs. 13% +/- 14%, P < .0001) and NFT/DP (29% +/- 13% vs. 10% +/- 9%, P < .0001). The PHT and DT lengthened, and the NFT shortened in patients who underwent pulmonic valve replacement (all P < .05). By binary logistic regression, NFT and PHT were the best predictors for severe PI. An NFT of 80 ms had 84% sensitivity and 93% specificity, and a PHT of 100 ms had 93% sensitivity and 93% specificity for identifying angiographically severe PI. CW Doppler accurately distinguishes severe from lesser degrees of PI in patients with postoperative adult congenital heart disease.
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PMID:Quantitative assessment of pulmonary insufficiency by Doppler echocardiography in patients with adult congenital heart disease. 1786 58

Tetralogy of Fallot (TOF) is a common form of cyanotic heart disease. Complete surgical correction in younger age group offers good long-term results with reasonable morbidity and improved prognosis in patients with TOF. However, following corrective surgery pulmonary valve replacement (PVR) might be required for residual pulmonary regurgitation in order to avoid irreversible right ventricular remodeling. Otherwise, residual uncorrected pulmonary regurgitation may lead to right ventricular dilatation, impaired biventricular function, ventricular arrhythmias and limited exercise capacity. We report the first case of Freedom Solo stentless valve (Sorin Group, Saluggia, Italy) implantation in the pulmonary position in an adolescent with severe pulmonary insufficiency 12 years after the repair of TOF. Pericardial stentless valves may be an alternative choice for pulmonary valve replacement to improve right ventricular contractile recovery and remodeling after PVR and may have impact on long-term survival.
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PMID:First pulmonary valve replacement with Freedom Solo stentless valve in an adolescent. A case report. 1794 52

This article reviews the most significant developments reported between July 2006 and July 2007 in the fields of pediatric cardiology and congenital heart disease, in diagnosis and treatment in particular. In the area of imaging techniques, the increasing clinical roles of magnetic resonance imaging, tissue Doppler imaging and three-dimensional echocardiography are highlighted, as is the application of these techniques to fetal cardiology and its repercussions. The incidence and treatment of arrhythmias in children are also discussed and, with regard to heart failure, there is an assessment of recent findings in epidemiology and diagnosis, new drugs, ventricular assist systems, and the current status of heart transplantation. In the area of interventional cardiology, present-day techniques for the closure of atrial and ventricular septal defects are reviewed and there is an assessment of early results with partially absorbable devices, percutaneous pulmonary valve implantation, and new types of stent (e.g., premounted, coated and absorbable stents). In cardiac surgery, the focus is on studies that evaluate outcome quality, the search for new biocompatible conduits, experience with new techniques for treating complex transpositions (e.g., the Nikaidoh procedure and its variants), the medium- and long-term results of treating aortic valvular disease with pulmonary autografts (i.e., the Ross and Ross-Konno procedures), and current findings on the treatment of pulmonary insufficiency after correction of either the tetralogy of Fallot or a dysfunctional Fontan circuit.
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PMID:[Advances in pediatric cardiology and congenital heart diseases]. 1834 32

Tetralogy of Fallot (TOF) represents the most common form of cyanotic congenital heart disease, accounting for 6.8% of all congenital heart disease. As surgical techniques and medical management of patients with TOF have improved, most affected patients are reaching adulthood. Though surgical outcomes are favorable (<2% early mortality rate), adults with TOF may experience complications from the long-term sequelae of congenital heart disease and complications related to treatment. We describe common and uncommon findings in adults with TOF, including pulmonary insufficiency, central and peripheral pulmonary artery stenosis and aneurysms, in addition to graft and shunt-related complications. Pulmonary function abnormalities and lung parenchymal imaging findings will be detailed. The diagnostic value of computed tomography and magnetic resonance imaging in adults with complications of TOF will be illustrated.
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PMID:MRI and computed tomography of cardiac and pulmonary complications of tetralogy of fallot in adults. 2021 61

The clinical detection and quantification of tricuspid valve disease, although important, is not entirely accurate. Diagnostic evaluation is based on echocardiography, and color flow Doppler is useful for quantifying tricuspid regurgitation. Echocardiography provides information on heart chamber dimensions, right ventricular function, and the degree of pulmonary hypertension. In addition, tricuspid stenosis can be accurately assessed using mean and end-diastolic pressure gradient measurements. The treatment options for tricuspid stenosis include balloon valvuloplasty and surgical valve repair. Functional tricuspid regurgitation associated with left heart disease may require surgical attention during an operation to treat the left heart disease. Severe tricuspid regurgitation usually requires surgery to be performed in association with mitral valve surgery. Mild-to-moderate tricuspid regurgitation requires surgery when annular dilatation or severe pulmonary hypertension is present. The surgical options include tricuspid valve repair, with or without an annuloplasty ring. In patients with a primary anatomic deformity of the tricuspid valve, replacement of the valve with a bioprosthesis or mechanical valve may be considered. Intermediate and long-term results favor annuloplasty valve repair over valve replacement. Pulmonary valve disease is predominantly congenital, and generally takes the form of pulmonary stenosis. Pulmonary regurgitation often results from surgical or balloon valvuloplasty and is associated with deleterious long-term sequelae. The recent development of percutaneous valve replacement was a major advance.
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PMID:Tricuspid and pulmonary valve disease evaluation and management. 2107 Jul 30

Improved surgical outcomes in children have led to a growing population of adults with congenital heart disease. Rhythm disturbances in the adult congenital heart disease (ACHD) patient can be intrinsic to the anomaly or acquired after palliation. Tachyarrhythmias, either supraventricular or ventricular, and bradyarrhythmias, either sinus node dysfunction or atrioventricular block, may occur frequently. Technological advances in intervention and surgical approaches have led to prophylactic and therapeutic reduction in arrhythmias. In order of escalation, this article addresses medical management, catheter ablation, device therapy for antitachycardia pacing and defibrillation, and surgical intervention. There are now an estimated one million-plus ACHD patients living in the United States. An estimated 45% of those have simple defects (e.g., atrial septal defects), 40% have moderately complex defects (e.g., tetralogy of Fallot, or ToF), and 15% have severely complex defects (e.g., single ventricle anatomy or surgical palliations for transposition of the great arteries [TGA]). The moderate and severe categories have a high incidence of arrhythmia. Of older repaired ToF patients, 34% develop symptomatic atrial or supraventricular tachycardias, 8.5% develop high-grade ventricular tachycardia (VT), and there are an increasing number of implantable defibrillators due to a sudden-death estimate of 2% per decade. Thus, an estimated 50,000 adults with repaired ToF will require electrophysiology follow-up with 100 sudden deaths per year nationally. Roughly 3% of all congenital heart surgeries requiring cardiopulmonary bypass have post-operative AV block, and 1% will require permanent pacing. Older atriopulmonary Fontans have up to an estimated 50% incidence of atrial tachycardia within a decade of palliation due to suture lines and elevated atrial pressures. Patients who have undergone an atrial switch operation (e.g., Mustard or Senning operations) are rarely exclusively in sinus rhythm a decade after repair, thus posing problems for rhythm control and anticoagulation. Catheter ablation in congenital heart disease is often more challenging than structurally normal hearts because of abnormal anatomy (congenital and/or post-surgical) and thicker chamber walls due to unfavorable hemodynamics. In preparation, review of noninvasive imaging, previous catheterization angiography, and surgical palliation reports are paramount prior to a procedure. Fortunately, to meet the growing challenge, newer software with 3-D packages allow for improved mapping. In addition, advances with larger-tipped and irrigated-tip catheters allow for deeper, more effective lesions to be placed. Several advances in surgical approach have led to a reduction in arrhythmias. For example, the arterial switch operation for d-transposition of the great arteries instead of the aforementioned atrial switch operation eliminates the use of a systemic right ventricle that can later develop intra-atrial reentrant tachycardia and sinus node dysfunction. Other advances include paying careful attention to minimizing ventriculotomies in ToF and ventricular septal defect (VSD) repairs, earlier complete repairs, and valve sparing to reduce pulmonary insufficiency. Finally, completion of the extra-cardiac Fontan procedure (e.g., total cavopulmonary connection) for single ventricles avoids extensive suture lines in the right atrium, thereby reducing scarring and higher pressures that lead to IART and sinus node dysfunction. Extracardiac (EC) conduits and lateral tunnel (LT) Fontans are preferred today, and the Fontan conversion procedure (converting prior atriopulmonary Fontans to the EC or LT type) can be performed to reduce arrhythmia and thromboembolic events.
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PMID:Electrophysiology issues in adult congenital heart disease. 2168 42


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