Dr Luigi Camporota

  • Specialist Registrar in Intensive Care Medicine
  • Department of Adult Intensive Care
  • Guy? and St Thomas?NHS Foundation Trust
  • London, UK

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At cardiac catheterization muscle relaxant for dogs purchase skelaxin 400 mg on line, a typical finding is the similarity of pressure wave contours obtained from the pulmonary artery, right ventricle, and right atrium. Endocardial potentials, if recorded during catheterization, show normal transition between the ventricular and atrial complexes, helping to rule out Ebstein anomaly (59). Angiocardiography demonstrates dilated right cardiac chambers with delayed filling of the pulmonary artery and also may demonstrate a small right-to-left atrial shunt. Although a few patients have been described who lived into adulthood, most patients die in infancy or childhood. The typical pathologic finding is the markedly dilated right ventricle, which is described as parchment-like. Histologically, the endocardium is thickened, and there are few if any true myocardial cells in the right ventricular free wall. Indeed, the right atrial wall may be thicker than the right ventricular anterior wall. The tricuspid valve arises normally from a dilated valve annulus and may be dysplastic but is not displaced into the right ventricular cavity as is typical for Ebstein anomaly. Various surgical procedures have been performed, including atrial septal defect closure, Potts anastomosis, and Glenn anastomosis. However, procedures that create total cavopulmonary anastomosis and right ventricular exclusion, with or without resection of the dilated right ventricular anterior wall, have resulted in survival into later years (6365). Although this lesion is extremely rare, at least one patient has undergone successful heart transplantation (66) and it has been considered in others (67). This form of treatment seems to be an option for long-term survival in symptomatic patients with findings of Uhl anomaly. Die Krankheiten des Herzens: Systematisch Bearbeitet und Durcheigne Beobachtungen Erlautert. The electrocardiogram in tricuspid atresia and pulmonary atresia with intact ventricular septum. The surgical treatment of malformations of the heart in which there is pulmonary stenosis or pulmonary atresia. Shunt between superior vena cava and distal right pulmonary artery: Report of clinical application in thirty-eight cases. Increasing the accuracy of lung perfusion scintigraphy in children with bidirectional Glenn circulation. In vitro flow experiments for determination of optimal geometry of total cavopulmonary connection for surgical repair of children with functional single ventricle. Effect of ventricular volume before unloading in a systemic ventricle supporting the Fontan circulation. Impact of age at Fonran completion on postoperative hemodynamics and long-term aerobic exercise capacity in patients with dominant left ventricle.

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Risk factors for poor outcome include young age at diagnosis muscle relaxant metabolism order 400 mg skelaxin with amex, septicemia, tamponade, delay in diagnosis, inadequate drainage, concurrent myocarditis, and a staphylococcal etiology (37,39,42). Constrictive pericarditis can be a late complication (36,39), most commonly associated with Staphylococcus aureus, Haemophilus influenzae, or Streptococcus pneumoniae infections. Other Causes of Pericarditis and Pericardial Renal Failure Effusion Pericarditis occurs in 10% of patients with chronic renal failure (50,51). Care should be taken with heparinization in these patients, as a pericardial hemorrhage can occur with resultant tamponade. Pericardiocentesis should be performed for the usual indications, including patients suspected of having bacterial pericarditis and in those with hemodynamic compromise. If the effusion fails to resolve with dialysis, or if constriction develops, pericardiectomy may be necessary (51). Tuberculous Pericarditis Once common throughout the world, Mycobacterium tuberculosis pericarditis now occurs most frequently only in developing nations. The typical onset is insidious with symptoms of low-grade fever, night sweats, weight loss, malaise, dyspnea, and chest pain. Tuberculous pericarditis often occurs due to direct extension of miliary tuberculosis or lymphatic spread into the pericardium. The pericardial fluid, when aspirated, is serosanguineous or hemorrhagic with lymphocyte predominance. Iatrogenic hemopericardium also can occur during placement of central venous lines and invasive cardiac procedures. Potentially metastatic tumors include Hodgkin disease, nonHodgkin lymphoma, leukemia, malignant melanoma, Wilms tumor, neuroblastoma, Kaposi sarcoma, and bone/soft tissue sarcomas (62-65). Nonmalignant congenital intrapericardial lesions may occur, including pericardial cysts, extralobar pulmonary sequestration, cystic lymphangioma, bronchogenic cysts, and pericardial teratomas (66-70). Pericarditis can occur as a complication of certain chemotherapy agents, listed in Table 62. Diagnosis of the cause of a malignant effusion can be made using cytologic analysis and culture of pericardia I fluid (72). Patients who have received mediastinal irradiation are at increased risk of pericardia I involvement after chemotherapy. Up to 5% of patients receiving mediastinal irradiation will develop pericarditis, typically from 2 months to 2 years after treatment (73). The patient may present with only mild symptoms or may progress to fulminant constrictive pericarditis Kawasaki Disease During the acute phase of Kawasaki disease, one-third of patients have a pericardial effusion, which typically resolves within 2 weeks. Effusions related to this inflammatory process do not commonly progress to tamponade, but this has been reported (52,53). Drug-Induced Pericarditis Drug-induced pericarditis may occur in patients treated with the medications listed in Table 62. Patients with hypersensitivity reactions to penicillin and cromolyn sodium in association with pericardial effusion have been reported (55,56).

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The onset of tapping sounds (phase 1) is the point used for systolic blood pressure and the disappearance of sound (phase 5) is used for the diastolic blood pressure spasms under ribs 400 mg skelaxin overnight delivery. When sounds can be heard to 0 mm Hg, then the onset of the phase 4, or muffing of sound, should be used to determine the diastolic blood pressure. The mercury sphygmomanometer is the optimum device for measurement of blood pressure. An appropriate alternative is the aneroid device, which is a mechanical device that is calibrated against the mercury column. In general, automated oscillometric devices are not recommended for routine measurement of blood pressure in children and adolescents. If an automated oscillometric device is used and elevated blood pressure is identified, this should be confirmed using a mercury manometer or aneroid device (238). Children and Adolescents For older children, the likelihood of secondary hypertension is diminished, but it still may be present. The standard evaluation for children with persistent elevation of blood pressure is presented in Table 71. Findings on history that may suggest other causes of hypertension are presented in Table 71. Physical examination findings that may suggest secondary forms of blood pressure elevation are presented in Table 71. Based on history and physical findings, additional laboratory tests may be performed to evaluate the possibility of an underlying cause for the blood pressure elevation. This means that for a younger child, with greater elevation of blood pressure and little or no family history of hypertension, consideration should be given to possible secondary causes. For the adolescent with mild elevation of blood pressure, over- Evaluation of Target Organ Abnormalities Echocardiography is recommended as the main tool for clinical evaluation of target organ abnormalities. In the older child and adolescent, a history of alcohol or drug abuse may suggest the utility of a drug screen. It is when lifestyle changes have been shown to be insufficient and blood pressure remains elevated that more aggressive treatment with pharmacologic agents is instituted. Obesity may have a pathologic effect resulting in elevation of left ventricular mass (251). A conservative cutpoint for determining elevation of left ventricular mass index is 51 g/rn-". This is >99th percentile for normal children and adolescents and is a level that is associated with increased cardiovascular morbidity and mortality in adults with hypertension (222). Other methods for evaluation of target organ effects such as ultrasound evaluation of the carotid arteries or evaluation or the urine for micro albuminuria are not currently recommended in children and adolescents. Ophthalmologic examination of the retinal vessels may be useful in identifying children and adolescents with retinal vascular changes, which may reflect abnormal changes in other vascular beds. In children with a history of snoring, irregular breathing during sleep, and daytime sleepiness, a polysomnogram may be indicated to lifestyle Modification There is good evidence in adults that modification of lifestyle focused on changing diet and physical activity can have a beneficial effect on blood pressure. From the standpoint of diet, overweight, excess intake of salt and alcohol, and low intake of potassium have all been associated with blood pressure elevation (252).

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Roentgenogram of a 1-day-old infant with critical pulmonary stenosis and congestive heart failure spasms just below ribs discount skelaxin 400 mg on-line. Cardiac Catheterization the refinement of 2-D and Doppler echocardiography over the past three decades has had a dramatic impact on the use of cardiac catheterization in the management of pulmonary valve stenosis. Because diagnosis and exclusion of other significant lesions can now be accomplished noninvasively, the role of catheterization has become largely therapeutic. A resting right ventricular pressure >30 to 35 mm Hg and a pressure gradient across the pulmonary valve of >10 mm Hg are considered abnormal. Continuous-wave Doppler recording from the parasternal short-axis view of a patient with severe pulmonary valve stenosis. The right ventricular end diastolic pressure may be normal, but usually it is elevated with severe obstruction or right ventricular failure. The right atrial pressure is normal in mild to moderate obstruction, but tall right atrial "a" waves usually are seen with severe obstruction. Pulmonary artery pressure is normal in mild cases, but it is decreased and dampened in severe cases. This depression of pulmonary artery pressure is more marked in the main pulmonary artery just beyond the valve than further distally because of the Bernoulli effect. In patients with relatively normal cardiac output, classification of severity of pulmonary stenosis is routinely based on measurements of right ventricular pressure and valve gradient. Mild stenosis is characterized by a right ventricular pressure less than half the left ventricular pressure or a valve gradient <35 to 40 mm Hg. Severe stenosis is defined as a right ventricular pressure ~75% of the left ventricular pressure or a gradient >60 to 70 mm Hg. Patients with severe stenosis are unable to increase their stroke volume and rely solely on an increase in heart rate to augment cardiac output during exercise. Decreased right ventricular compliance is further evidenced by elevated right ventricular end diastolic pressure at rest and abnormal increase with exercise observed in patients with severe obstruction (21). Angiocardiography Angiocardiography provides information about the location and severity of pulmonary stenosis that is invaluable for diagnostic and therapeutic purposes. The anatomy of the pulmonary valve and associated features can be shown best by right ventricular angiography in the anteroposterior view with the tube angled cephalad and in the lateral view. The pulmonary valve annulus is typically of normal size, but it may be hypoplastic in infants with severe stenosis. There is often the appearance of tethering of the valve leaflets to the main pulmonary artery wall at the sinotubular junction. Mild pulmonary stenosis should be differentiated from idiopathic dilation of the main pulmonary artery, atrial septal defect, peripheral pulmonary arterial stenosis, mitral valve prolapse, straight back syndrome, mild aortic stenosis, and innocent murmurs. When cyanosis is present in severe stenosis, tetralogy of Fallot and pulmonary atresia with intact ventricular septum must be excluded.

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The right ventricle is at systemic levels of pressure or higher spasms in stomach discount skelaxin 400 mg buy online, and the end-diastolic pressure may be abnormally high, consistent with a noncompliant ventricular chamber. The finding of a subsystemic right ventricular pressure is consistent with a globally disadvantaged right ventricle. The right ventricle is usually thinned, and severe tricuspid regurgitation often is present. The functional disturbance of severe tricuspid regurgitation correlates with an Ebstein-like abnormality of the tricuspid valve or severe tricuspid valve dysplasia. When right ventricular angiography does not demonstrate ventriculocoronary connections, one can be reasonably certain that coronary arterial stenosis or interruption or major fistulae with coronary-cameral flow will not be evident. In some patients with ventriculocoronary connections, a balloon catheter inflated in the right ventricle or catheter-induced tricuspid insufficiency with concurrent observation of the simultaneous electrocardiographic tracing may unmask a right ventricular-dependent coronary circulation. The angiocardiographic investigation of the patient with the hypertensive right ventricle requires right ventricular angiocardiography in frontal and lateral projections. The reported surgical survival of 27% prior to the availability of prostaglandins was poor (57). Rarely an urgent balloon atrial septostomy may be required for a restrictive septum as pulmonary flow improves and should be considered in any neonate that demonstrates persistent low cardiac output with the usual therapeutic maneuvers. For the premature infant or the extremely small-for-gestational-age infant, a prolonged course of an E-type prostaglandin may be necessary before surgery is undertaken, although this is unusual in the current surgical era. Once pulmonary blood flow is established, it is important to recognize that systemic oxygen saturation is related to the amount of flow into the pulmonary circulation. The patient has an obligatory rightto-left shunt at atrial level, hence a very high saturation usually reflects an enormous amount of pulmonary flow. The resulting low diastolic pressure and low cardiac output syndrome can result in compromised splanchnic and renal flow as well as ongoing acidosis and, in the extreme, cardiogenic shock. In addition, this physiology can compromise oxygen delivery to the aortocoronary circulation resulting in myocardial ischemia. The pulmonary outflow tract in this patient with valvar atresia is imaged in the precordial short-axis view with slight clockwise rotation. Lower Panel: the possibility of pulmonary insufficiency (red flow) raises the possibility that the valve is severely stenotic and not atretic. Selective injection of the right ventricular outflow tract can differentiate severe stenosis of the pulmonary valve from membranous atresia (56). Aortography in the neonate defines the laterality of the aortic arch, the caliber of the subclavian arteries, the site of insertion of the arterial duct, the caliber of the pulmonary arteries, and coarctation of the pulmonary artery at the insertion of the arterial duct that may preclude treatment with a stent to permanently maintain its patency or require a surgical patch at the time of placing a Surgical Surgical and other catheter-based interventional therapies have continued to change with an understanding of the heterogeneity of this disorder. The diagnostic and therapeutic algorithms are now quite diverse, depending on a wide number of morphologic variables (5,58-62). Among some centers, primary cardiac transplantation also is considered particularly when the coronary artery anatomy is complex. Functional pulmonary atresia in a newborn with pulmonary atresia and intact ventricular septum. Is the patient ultimately a candidate for a complete biventricular (including the so-called one-and-a-half ventricle) repair or a univentricular total cavopulmonary (Fontan) palliation Ideally, one would like to achieve a biventricular repair in these patients, and the initial approach should allow right ventricular decompression by some form of right ventricular outflow tract reconstruction, whether surgical pulmonary valvotomy, outflow tract patch, or catheter-based perforation and dilation of the pulmonary valve.

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The papillary muscle morphology is also variable muscle relaxant of choice in renal failure generic skelaxin 400 mg visa, in particular with regard to the number of heads. Indeed if they are removed during valve replacement, then this results in left ventricular dysfunction (22). These features are readily appreciated by 3-D echocardiography, both from a left atrial and left ventricular view. This is of particular value for imaging the chordal apparatus, as they are imaged in the axial plane that provides optimal resolution. The left ventricular view is of particular importance for imaging the commissures, because they are imaged more reliably from below than from the left atrial view. This is due to the fact that the normal mitral leaflets billow toward the left atrium, just as a parachute does when seen from the sky. The area of this jet correlates well with absolute volume regurgitation, providing a semiquantitative assessment of the degree of regurgitation. As well, it provides an accurate road map to the location of the regurgitation, providing vital information for the surgeon when contemplating repair. The upper left hand image shows the 2-D appearance of the anterior papillary muscle and leaflet. The restrictive functional orifice is clearly seen in the lower right hand panel (black arrow). Note that the relationship of this to the valve can be appreciated from this image. The mitral orifice and the thickened leaflets can be seen, as well as the tethering of the mural leaflet (black arrow). Unfortunately, in the majority of children, it is not possible to calculate a pressure halftime that can provide an absolute valve area in adults. The leaflet is invariably tethered by shortened chordae, preventing normal coaptation with the anterior leaflet. The regurgitant jet invariably extends along the total length of the valve orifice and may respond to surgical repair by leaflet extension. Three-dimensional echocardiography provides optimal imaging of this entity, as well as an accurate assessment of the severity of regurgitation. Therefore, in the majority of cases, surgical intervention does not cure the problem, but simply provides some relief of the associated stenosis. This entity therefore has a physiologic impact on the mitral annulus, as well as the leaflets and subvalve apparatus. Mitral Arcade this is a rare entity, however one that has a significant impact on outcome. The functional result is often regurgitation, which is due to a tethered valve with deficient zones of leaflet coaptation.

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Surgical results have been excellent (130 muscle relaxant during pregnancy 400 mg skelaxin purchase,131,136,137), with resolution of associated symptoms (130,136,176). Two-dimensional echocardiogram in a 2-yearold child with a left atrial myxoma (M). The use of echocardiography to facilitate a surgical approach has been proposed (177) by preoperatively defining tumor size, location, point of attachment, and presence of concurrent site involvement. Patients require continual reevaluation for recurrence of disease and for later development of peripheral arterial aneurysms (130,136,139,142,146,160,175). The approximate incidence of recurrence is 4% to 7% in most large series (162,164,178,179). Familial occurrence of cardiac myxomas is well established (130,131,136,137,180,181) and accounts for 7% of all myxomas. Cardiac myxomas often are seen in children and adolescents with multiple lentigines syndromes (157) and may be associated with nonneoplastic endocrine abnormalities. The precise gene defects remain unknown (182); however, certain investigators have mapped these syndromes to two loci, on chromosome 2p (183) and chromosome 17q (184). These rare tumors previously were associated with a high mortality rate (185-189). More recently, increased survival is emerging as a result of earlier diagnosis and improvements in surgical care (129,185). Intrapericardia I tumors are seldom malignant or recurrent; therefore, surgery is considered curative for such life-threatening illness. Intrapericardial teratomas are single, encapsulated, grayish tan, bosselated tumors attached to the base of the heart (189-191). Often a broad-based stalk or narrow pedicle firmly attaches the tumor to the root of the aorta or pulmonary artery (185,188,189). The tumor capsule itself can be firmly attached to the aorta (186-200) or to pulmonary artery adventitia (187,189,191,197,200). The tumor has been reported to adjoin the superior vena cava (191), right atrium (187,189,191), right ventricle, left atrium, and left ventricle (189). The tumor blood supply usually emanates as nutrient vessels from the aortic vasa vasorum (187,189,197,198). Single blood vessels from the vicinity of the coronary arteries (190) or multiple small blood vessels from the superior mediastinum also may supply the tumor (191). Intrapericardial teratomas may be three to four times the size of the newborn or infant heart (186,189,199); however, the tumor may be relatively small in asymptomatic older children and adolescents. Critically ill newborns and babies almost always have a large pericardia I effusion (188,189,197). Obstruction and compression of the heart develop due to an essentially solid tumor mass contained within a restrictive fibrous pericardium (188,189,198).

Cacchi Ricci disease

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In general spasmus nutans treatment cheap skelaxin 400 mg amex, this entity is readily recognized by both two and three-dimensional echocardiography. A: these two images are from a double-orifice left atrioventricular valve, where both orifices are of a similar size. The 2-D echo images on the left show the large posterior orifice and what appears to be a large separate anterior papillary muscle. The upper right hand panel is a 3-D image from below and shows the main orifice indicated by the asterisk, and the tension apparatus that supports the imperforate anterior orifice. The aortic or anterior leaflet is always involved with chordal apparatus from that leaflet having variable attachments within the right ventricle. In some instances, they insert into the crest of the interventricular septum while in others, they insert into a papillary muscle on the proximal or distal interventricular septum. A: this montage is from a case with corrected transposition after a double switch. The two lower images show the mitral chords inserting into a papillary muscle; however, the precise location is unclear. The image on the left is with partial cropping of the heart, while the one on the right views the ventricular septal defect in front from the right ventricle. Although 2-D echo cardiography has superior temporal resolution making identification of fine chordal structures more accurate, inferior spatial resolution can impede precise location of the abnormal chordae. Despite this, it is an invaluable measurement in pediatric patients, whereas pressure halftime is difficult to interpret due to the higher heart rates of children compared to adults (42). Certainly, this technique provides superior data with regard to the site of regurgitation and can be incorporated into surgical decision making. The advantage is that the valve can be seen as a whole, and commissural and central regurgitant jets pinpointed. This is understandable as 2-D color techniques presume that the jets are circular in shape. Of interest, for the tricuspid valve, although there is a good correlation between vena contracta width by 2-D echocardiography and vena contracta area by 3-D echocardiography, only the latter has been shown to be predictive of outcome using multivariate analysis (47). In that setting, the papillary muscles are invariably sclerotic, and abnormal flow from associated coronary collaterals also can be identified. Continuous Doppler Regurgitant Jet Profile the more severe the regurgitation, the denser the continuouswave Doppler profile. This must be taken in context as increased gain settings can enhance a Doppler signal. Once again, this will be affected by those cases with associated diastolic dysfunction. In practical terms, the color Doppler Nyquist limit is set to a value at which aliasing occurs near the regurgitant orifice.

Colver Steer Godman syndrome

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Dobutamine Dobutamine is a racemic mixture with complex actions involving a- and spasms pronunciation buy skelaxin 400 mg on-line,8-adrenergic receptors. Dobutamine is often selected in situations for which the primary goal of therapy is to improve ventricular contractility (48,53). Dobutamine may be administered as a single drug or as an adjunct to the infusion of other agents. As the dosage increases, dobutamine may adversely increase heart Isoproterenol Isoproterenol is a synthetic catecholamine with potent nonselective,8-adrenergic agonism and no significant effect on a-adrenergic receptors (47). Isoproterenol increases cardiac contractility and heart rate (,8ceffect) and reduces systemic vascular resistance due to dilation of skeletal muscle renal and splanchnic beds (,82-effect). The drug is a poten~ bron~ chodilator and thus, may be particularly beneficial in patients with pulmonary disease and bronchoconstriction. Since many infants with low cardiac output are already tachycardic, isoproterenol is used rarely. The published experience with levosimendan in children is quite limited and additional studies are necessary to determine the safety and efficacy in pediatric patients with depressed cardiac function. Phenylephrine has been used acutely during hypercyanotic episodes in patients with tetralogy of Fallot to increase systemic resistance, reduce the right-to-left shunt, increase pulmonary blood flow, and thereby improve systemic oxygenation. However, diuretics do not improve the neurohormonal alterations that contribute to the heart failure syndrome. Aggressive diuresis can actually promote activation of the sympathetic nervous system and the renin-angiotensinaldosterone system. Hypovolemia, decreased renal blood flow, reduced glomerular filtration rate, or sodium depletion may reduce diuretic efficacy. The most commonly used drug is furosemide, but ethacrynic acid and bumetanide are also available. Loop diuretics inhibit chloride-sodiumpotassium cotransport in the thick ascending limb of the loop of Henle. This reduces reabsorption of chloride, sodium, and potassium and increases net excretion of free water. Despite these observations, milrinone has gained widespread usage in the pre- and postoperative management of infants and children with ventricular dysfunction (48,57-59). However, whether the apparently beneficial hemodynamic responses are primarily due to increased contractility or to pulmonary and systemic vasodilation remains unclear. Furosemide Furosemide increases renal blood flow, enhances renin release, and reduces renal vascular resistance. It has diuretic and nondiuretic pulmonary effects and appears to reduce pulmonary transvascular fluid filtration. The primary indications for furosemide in children with heart disease include acute and chronic management of congestive circulatory states and diuresis following cardiac surgery. The drug is primarily excreted unchanged by the kidneys and the dosage must be adjusted in renal failure or in infants with immature renal function.

Schmitt Gillenwater Kelly syndrome

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Lewis and Chabot (114) suggested that left ventricular end-diastolic pressure >25 mm Hg is a predictor of poor outcome and therefore an indication for earlier transplantation muscle relaxant at walgreens skelaxin 400 mg for sale. The greatest risk of death or transplantation was associated with age younger than 1 year or older than 12 years, and female sex. If the cause for the cardiomyopathy is identified and is treatable, such as carnitine deficiency,one should expect a high survival rate. The actuarial survival rate in this group of patients is 63% to 90% at 1 year and 20% to 80% at 5 years (7,113-116). The cause of death usually involves ventricular arrhythmias and progressive intractable ventricular failure or, more rarely, complications of heart transplantation. However, patients with treatable arrhythmias, supraventricular or ventricular, had a higher survival rate when the arrhythmia was adequately controlled (114,118). Intracardiac thrombi were identified in 16% to 23% of patients by thorough 2-D echocardiograms (119). Although thrombus is not a predictor of survival, it does point to the importance of anticoagulation therapy and the potential morbidity in this group of patients. Mutations that alter the surface charge of alpha-tropomyosin are associated with dilated cardiomyopathy. Familial dilated cardiomyopathy: cardiac abnormalities are common in asymptomatic relatives and may represent early disease. Impairment of the myocardial ultrastructure and changes of the cytoskeleton in dilated cardiomyopathy. The structural correlate of reduced cardiac function in human dilated cardiomyopathy. Progression of familial and nonfamilial dilated cardiomyopathy: long term follow up. X-linked dilated cardiomyopathy: molecular genetic evidence of linkage to the Duchenne muscular dystrophy (dystrophin) gene at the Xp21 locus. Deletion of the dystrophin musclepromoter region associated with x-linked dilated cardiomyopathy. Clinical and genetic issues in dilated cardiomyopathy: a review for genetics professionals. Severe disease expression of cardiac troponin C and T mutations in patients with idiopathic dilated cardiomyopathy. Mutations in the human a-sarcoglycan gene in familial and sporadic dilated cardiomyopathy.

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Flint, 40 years: Ambulatory blood pressure monitoring in children and adolescents: recommendations for standard assessment: a scientific statement from the American Heart Association Atherosclerosis, Hypertension and Obesity in Youth Committee of the Council on Cardiovascular Disease in the Young and the Council for High Blood Pressure, 256. Natural history and risk stratification of discrete subaortic stenosis in children: an echocardiographic study.

Dawson, 63 years: Mortality following pulmonary artery banding may be as high as 10%, although the early postoperative course following the arterial switch seems comparable to that of primary repair. Rarely, mechanical devices such as intra -aortic balloon pumps or ventricular assist devices may be required to maintain cardiac output until ventricular function improves.

Owen, 26 years: Unless surgery is contraindicated for other reasons, such patients should be referred for surgery (194,292). A realistic aim statement often requires that it be "bounded" or narrowly focused.

Ramon, 64 years: It is mandatory that genetic testing be performed in conjunction with experienced genetic counselors before and after the test results (87). Our discussion of cardiac malposition includes abnormalities of overall cardiac position (dextrocardia, mesocardia, levocardia [isolated]), as well as pericardial defects, and the complex anomaly of ectopia cordis (1,2).

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