Brittany Hoffmann-Eubanks, PharmD, MBA

• Clinical Pharmacist, Jewel-Osco Pharmacy, South Holland, Illinois

When the anomalous connection is to the azygous vein cholesterol levels what is normal buy discount rosuvastatin, this structure is enlarged and can be recognized on the chest radiogram as a rounded bulge in the right superior mediastinum at the right cardiac border cholesterol levels in quail eggs 10 mg rosuvastatin overnight delivery. The individual pulmonary veins should be examined in every patient cholesterol on keto rosuvastatin 10 mg sale, particularly at the time of the first echocardiographic evaluation cholesterol vldl purchase rosuvastatin 10mg visa. Chest radiogram in the posteroanterior projection in a patient with totally anomalous pulmonary venous connection to the innominate vein showing the typical "snowman" appearance of the mediastinum cholesterol levels pork vs beef discount rosuvastatin 10 mg line. Peaked P waves and right ventricular hypertrophy of the systolic overload pattern occur in older patients exhibiting pulmonary hypertension test of cholesterol discount 10 mg rosuvastatin free shipping. This can be achieved from the subcostal, apical, parasternal, and suprasternal notch windows. The subcostal window is ideal for evaluating the pulmonary veins in infants and young patients with good subcostal acoustic windows. The parasternal, subclavicular, and suprasternal windows are used in older patients. Typically, the systemic vein distal to the connection of the pulmonary vein is dilated, reflecting the increased flow. The diagnosis can be made from the subcostal window in infants and young children. The wide field of view, excellent spatial orientation, and its inherent three-dimensional (3-D) nature allow unambiguous delineation of the course, connections, and drainage of the pulmonary veins independent of body size and acoustic windows. The anatomy of the pulmonary veins can be evaluated by a series of T1-weighted spin echo images in the axial (transverse), coronal, or oblique planes. Interventional catheterization is indicated to occlude aortopulmonary collaterals in scimitar syndrome. If the anomalous connection is entered, direct injection of radiographic contrast dye will delineate the anatomy. Treatment Medical Management Three-dimensional reconstruction of gadolinium-enhanced magnetic resonance angiogram in a posterior view in a 20-year-old male with scimitar syndrome. Studies based on anatomic material (8) indicate that patients with one pulmonary vein connected anomalously and with an intact septum have an excellent prognosis and rarely present with cardiorespiratory symptoms. It would be erroneous, however, to apply this excellent prognosis to patients who present with symptoms. Patients with atrioventricular concordance in whom a biventricular repair can be achieved. In these patients, the atrial septum is excised, and a new septum is constructed so that the systemic and pulmonary veins drain into their corresponding atria. In these patients, the pulmonary veins are baffled into the systemic ventricle and the systemic veins and then drain into the pulmonary ventricle. These favorable results cannot be anticipated in the patient who has developed pulmonary vascular disease. They had one early operative death in a 31-year-old patient with severe pulmonary hypertension. There was a relatively high incidence of residual scimitar drainage stenosis (freedom from scimitar vein stenosis was 85% at 13 years) that did not appear to vary by surgical approach (33). The postoperative patient should be monitored periodically for development of pulmonary venous obstruction and for arrhythmias. The obstruction can result from occlusion of the anomalous pulmonary veins and can result in no blood flow to the affected lung. Another study recorded 2 % of 800 autopsied cases of congenital cardiac disease in the first year of life (35). The Baltimore-Washington Infant Study, however, showed a possible association with exposure to lead, paint or paint-stripping chemicals, and pesticides (35,37). Among the affected families, the common denominator was the anomalous pulmonary venous connection; the site of the anomalous venous connection was usually not concordant. Gross examination of the heart shows several features common to all cases, regardless of the site of the anomalous connection. Left atrial size usually is diminished because it lacks the contribution of the common pulmonary vein. In a rare case, the right-sided ascending pulmonary venous vessel connects to the azygous vein. Posterior view of heart and lungs specimen showing supracardiac totally anomalous pulmonary venous connection. The W is "sandwiched" between the left main stem bronchus posteriorly and the left pulmonary artery anteriorly. Posterior view of heart and lung specimen showing totally anomalous pulmonary venous connection to the portal vein. Total anomalous pulmonary venous connection: Report of 93 autopsied cases with emphasis on diagnostic and surgical considerations. The ductus venosus normally undergoes constriction, and therefore anomalous connection to this structure results in pulmonary venous obstruction. Finally, when the anomalous connection is to the portal vein or one of its tributaries, the hepatic sinusoids are interposed in the pulmonary venous channel and result in increased resistance to pulmonary venous return. A common vessel originates from this confluence, descends immediately anterior to the esophagus, and penetrates the diaphragm through the esophageal hiatus. Unobstructed veins often exhibited vein wall atrophy or hypertrophy of intima, mediaadventitia, or both. Obstructed veins usually have mediaadventitial thickening and often have intimal proliferation. The major variable is the state of the pulmonary vascular bed, which initially depends on the presence or absence of pulmonary venous obstruction. Progressive dilation and hypertrophy of the right ventricle and dilation of the pulmonary artery usually occur. Although the muscle fibers were narrow with scanty cytoplasm, they had abundant nuclei. Pulmonary edema and extravasation of red cells into the alveolar spaces are pronounced. Intimal proliferation within the arterioles is common, and necrotizing arteritis rarely is seen. Pulmonary edema results when the hydrostatic pressure in the capillaries exceeds the osmotic pressure of the blood. Mechanisms that tend to prevent pulmonary edema include increased pulmonary lymphatic flow, alternative pulmonary venous bypass channels, altered permeability of the pulmonary capillary wall, and reflex pulmonary arteriolar constriction. The state of the interatrial septum is of primary importance in this distribution. Hence, the stimulus for the development of a large interatrial communication is minimal. The hemodynamic consequences of inadequate interatrial communication include pulmonary venous obstruction. The presence of intrinsic or extrinsic narrowing in the connecting vein also produces pulmonary venous obstruction. Thus, the manifestations may be divided according to whether pulmonary venous obstruction is absent or present. Tachypnea and feeding difficulties were the initial symptoms, usually manifested by the first few weeks of life. From that point on, the infants did not thrive, were subject to repeated respiratory infections, and usually had cardiorespiratory failure by 6 months of age. Cyanosis may be so mild as to be clinically inapparent, except in the presence of cardiac failure and in the patient who survives long enough to acquire secondary pulmonary vascular changes. Of these infants, 75% to 85% die by 1 year of age, most in the first 3 months of life (40). This murmur often is heard well over the xiphoid and at the lower left sternal border; in this case, it is Sl coincident secondary to tricuspid regurgitation. A diastolic tricuspid flow murmur at the lower left sternal border occurs frequently. Unlike the "innocent" venous hum, this murmur is not louder during diastole and is not altered by change in position or pressure on the neck veins. In cardiac failure, hepatomegaly is always present, and peripheral edema is present in about half of the cases. Once identified, each individual pulmonary vein is imaged by 2-D and is interrogated by color Doppler flow mapping. Based upon a recent multicenter study from Europe investigators similarly found hypoplastic/stenotic pulmonary veins to be an independent risk factor for death (46). The individual pulmonary veins should be imaged from multiple windows, but the parasternal, subclavicular, and suprasternal notch views mostly are used. Often the pulmonary venous channel dilates proximal to the site of stenosis, a finding that should prompt a careful search for obstruction. Pulmonary venous flow in an unobstructed vessel is characterized by a low-velocity, phasic laminar flow pattern with brief flow reversal during atrial systole. An increased flow velocity disturbed (turbulent) flow pattern, and loss of the phasic variations characterize obstructed pulmonary venous flow. Doppler interrogation is used to differentiate flow characteristics among the various abdominal vessels. Flow in the descending aorta has a systolic laminar profile in a direction away from the heart. Flow in the common pulmonary vein is characteristic of the venous flow pattern, except the direction is away from the heart toward the abdomen. Suprasternal, parasternal, and subcostal windows, as described previously, should be used. Transesophageal echocardiography provides an alternative approach in patients with poor transthoracic windows. The need for transesophageal imaging in the newborn and young infant, however, is minimal because the transthoracic windows 829 are usually adequate. Identification of the pulmonary venous connections is one goal of a fetal echocardiographic examination. A right atrial pressure:::::2 m Hg in excess of left atrial pressure m is more reliable in predicting a restrictive interatrial communication, but too often it occurs in the face of free communication between the atria. Interpretation of atrial pressures, particularly in an attempt to determine the adequacy of the interatrial communication, is difficult. The presence of equal pressures in the two atria is probably an unreliable sign of a nonobstructive intera- Pulmonary venous obstruction is usual when the venous connection is to the umbilicovitelline venous system. Regardless of the site of pulmonary venous obstruction, the clinical profile is the same. Symptoms usually did not appear in the first 12 hours of life, a finding that helped differentiate these patients from patients with respiratory distress syndrome. Once symptoms began, there was rather rapid progression to dyspnea, feeding difficulties, and cardiorespiratory failure. A cardiac murmur often is absent, but, when present, it is usually a soft, blowing, systolic ejection murmur in the pulmonary area. Sites of obstruction also may be outlined when the anomalous connection is to other venous channels. If the cardiac catheter enters the anomalous venous channel, it may traverse the area of obstruction, thereby creating high-grade or complete obstruction to venous return. Measures may include mechanical ventilation, inotropic support, diuresis, and correction of acidosis and other metabolic problems. Balloon dilation of obstructed anomalous venous channels was unsuccessful in the patients described by Lock et al. Over time, the age at surgical repair has gradually decreased, as did operative mortality. A more recent, larger multicenter cohort of 422 cases from European centers found the 3-year survival for those operated between 1998 and 2004 to be 85%, with independent risk factors for death in multivariate analysis comprised of earlier age at surgery, hypoplastic/stenotic pulmonary veins, associated complex cardiac lesions, postoperative pulmonary hypertension, and postoperative pulmonary venous obstruction (46). The surgical techniques for the specific anomalies are indicated in the following sections. Far advanced intimal lesions in the pulmonary arterioles have been described as early as 8 months of age. The patients who the long-term prognosis appears to depend mainly on the state of the pulmonary vascular bed at the time of operation and the adequacy of the pulmonary venous-left atrial anastomosis. The late deaths were due to persistent pulmonary hypertension in one and recurrent pulmonary venous obstruction in the other. The mortality rate was affected by the preoperative status of the infant and the postoperative pulmonary artery pressure. Late pulmonary venous obstruction occurred in four patients for whom further therapy was advised. Residual stenosis at the left atrial-pulmonary venous anastomosis created at surgery was present in 8 of 68 patients (12%) reported by Yee et al. These obstructions were relieved by patch pIasty 1 to 24 months following the initial operation. Two of the three patients with recurrent pulmonary venous obstruction were found to have diffuse fibrosis of all lobar pulmonary veins as the cause rather than obstruction at the left atrial-pulmonary venous anastomosis. Sixty (15%) of these infants developed postoperative pulmonary venous obstruction, with 3-year mortality of 41 % in this subgroup; risk factors for the development of postoperative pulmonary venous obstruction included hypoplastic/stenotic pulmonary veins at presentation and the absence of a common pulmonary venous confluence. The usual chest radiographic picture of severe pulmonary venous obstruction is present. Extracorporeal membrane oxygenation was an essential postoperative supportive measure in two of these patients (60). Prognosis Symptoms occur on the first day of life, and these patients follow a progressive downhill course to death within the first month of life when there is no surgical intervention.

Insulin therapy is only used for persistent elevations in serum glucose above 300 mg/dL or when osmotic dieresis is complicating fluid therapy grocery list cholesterol lowering foods purchase rosuvastatin overnight. In addition cholesterol ratio hdl ldl proven 10 mg rosuvastatin, many patients with trisomy 21 have either overt or functional hypothyroidism (up to 30%) cholesterol comparison chart order rosuvastatin no prescription. In these patients and especially infants cholesterol tea purchase rosuvastatin 10mg without a prescription, T3 infusion can be very effective at improving myocardial function and decreasing the need for high-dose inotropic support (302 cholesterol xe2ed rosuvastatin 10 mg fast delivery,303) cholesterol test nyc buy rosuvastatin 10mg free shipping. Strict attention to preventing infection by common measures such as hand washing between every patient contact, disinfecting stethoscopes and other equipment and isolation of known or potentially infective patients is of critical importance. Immediate response, institution of effective chest compressions, assuring oxygenation and ventilation, rapid defibrillation or cardioversion, and administering standard resuscitation drugs such as epinephrine and atropine rapidly and prompt cardioversion or defibrillation are very important. Rapid surgical assistance to reopen the sternum of the arrested patient with cardiac tamponade, for instance, is essential. Such a system requires considerable institutional resources to equip and train personnel. Fever or hypothermia, leukocytosis, hemodynamic or respiratory compromise, coagulopathy or failure to progress in the expected course of illness are common accompaniments of significant infection. A thorough exarrunation, cultures of blood, endotracheal secretions, unne and imaging studies such as echocardiography to evaluate whether a vegetation is present, are necessary in suspected infection. Therapy is narrowed and promptly directed specifically at any" organisms that grow from cultures. These patients often do not have the same hemodynamic monitoring catheters, that is, arterial, right and left atrial catheters, as postsurgery patients. Not only can residual defects be assessed in detail with catheterization but also pulmonary vein saturations are often helpful to distinguish pulmonary parenchymal disease from other causes of arterial desaturation. This also affords the opportunity for intervention, where additional surgery would be high risk. In addition, patients on mechanical support may require catheter therapy to create a larger atrial communication for left heart decompression or correct positioning of intracardiac support catheters. Bloodstream infections are common and a prominent feature for increased mortality (308,309). This problem may be minimized by the routine preoperatively percutaneous placement of central catheters, which have a very low infection rate (310). Common pathophysiology often includes a dilated, poorly functioning left ventricle with acute congestive heart failure. Diagnosis and specific treatment for the underlying cause of heart failure is of paramount importance. Serial echocardiograms, serum lactate, mixed venous oxygen saturation measurements, and serum troponin or B-natriuretic peptide levels can be important modalities to assist in decision making. The melding of state-of-the-art knowledge and techniques in Cardiology, Surgery, Intensive Care, Anesthesia, Nursing, Respiratory Therapy, and Perfusion, continually yields new approaches to address the needs of these complicated patients. How to start and sustain a successful pediatric cardiac intensive care program: a combined clinical and administrative strategy. Assessing the brain using near infrared spectroscopy during postoperative ventricular circulatory support. Pediatric cardiac intensive care: current state of the art and beyond the millennium. A non-invasive estimation of mixed venous oxygen saturation using near infrared spectroscopy by cerebral oximetry in pediatric cardiac surgery patients. Changes in cerebral and somatic oxygenation during stage I palliation of hypoplastic left heart syndrome using continuous regional cerebral perfusion. Risk factors for central lineassociated bloodstream infection in a pediatric cardiac intensive care unit. Catheter-associated bloodstream infection in the pediatric intensive care unit: a multidisciplinary approach. Databases for assessing the outcomes of the treatment of patients with congenital and paediatric cardiac disease-the perspective of critical care. Databases for assessing the outcomes of the treatment of patients with congenital and paediatric cardiac diseasethe perspective of anaesthesia. A qualitative study exploring the experiences of parents of children admitted to seven Dutch pediatric intensive care units. Family-member presence during interventions in the intensive care unit: perceptions of pediatric cardiac intensive care providers. Postoperative lactate concentrations predict the outcome of infants aged 6 weeks or less after intracardiac surgery: a cohort follow-up to 18 months. The monitoring of venous saturations of oxygen in children with congenitally malformed hearts. An experimental and clinical evaluation of a novel central venous catheter with integrated oximetry for pediatric patients undergoing cardiac surgery. Evaluation of a pediatric central venous oximetry catheter in critically ill children. Correlation of abdominal site near infrared spectroscopy with gastric tonometry in infants following surgery for congenital heart disease. Percutaneous pulmonary artery catheterization in pediatric cardiovascular anesthesia: insertion techniques and use. Pulmonary artery catheter placement under transoesophageal echocardiography guidance. Estimation of cardiac index by means of the arterial and the mixed venous oxygen content and pulmonary oxygen uptake determination in the early post-operative period following surgery of congenital heart disease. Cardiac output determination in children; equivalence of the trans pulmonary thermodilution method to the direct Fick principle. Cardiac index monitoring by pulse contour analysis and thermodilution after pediatric cardiac surgery. Arterial pulse wave analysis: an accurate means of determining cardiac output in children. Reactivity of renal systemic circulation to vasoconstrictor agents in normotensive and hypertensive subjects. Systemic and coronary effects of intravenous milrinone and dobutamine in congestive heart failure. Acute hemodynamic effects of dobutamine and isoproterenol in patients with low output cardiac failure. Milrinone in the treatment of low cardiac output states following cardiac surgery. Pharmacokinetics and side effects of milrinone in infants and children after open heart surgery. Population pharmacokinetics of milrinone in neonates with hypoplastic left heart syndrome undergoing stage I reconstruction. Relation between ionized calcium concentration and ventricular pump performance in the dog under hemodynamically controlled conditions. Normal parathyroid hormone responses to hypocalcemia during cardiopulmonary bypass. Epinephrine plasma metabolic clearance rates and physiologic thresholds for metabolic and hemodynamic actions in man. Noradrenaline for management of septic shock refractory to fluid loading and dopamine or dobutamine in full-term newborn infants. Correlation between arterial blood pressure and oxygenation in retralogy of Failor. Intraoperative Doppler echocardiography in hypertrophic cardiomyopathy: correlations with the obstructive gradient. Intravenous arginine-vasopressin in children with vasodilatory shock after cardiac surgery. Vasopressin reversal of phenoxybenzarnine-induced hypotension after the Norwood procedure. Hemodynamic effects of intravenous nitroglycerin in pediatric patients after heart surgery. The impact of afterload reduction on the early postoperative course after the Norwood operation-a 12-year single-centre experience. The safety, efficacy, and pharmacokinetics of esmolol for blood pressure control immediately after repair of coarctation of the aorta in infants and children: a multicenter, double-blind, randomized trial. Prostaglandin E1: a new therapy for refractory right heart failure and pulmonary hypertension after mitral valve replacement. Prostaglandin E1: an effective treatment of right heart failure after orthotopic heart transplantation. Kermode], Butt W, Shann E Comparison between prostaglandin E1 and epoprostenol in infants after heart surgery. Use of inhaled iloprost in a case of pulmonary hypertension during pediatric congenital heart surgery. Intravenous sildenafillowers pulmonary vascular resistance in a model of neonatal pulmonary hypertension. Effects of escalating doses of sildenafil on hemodynamics and gas exchange in children with pulmonary hypertension and congenital cardiac defects. Very low dose inhaled nitric oxide: a selective pulmonary vasodilator after operations for congenital heart disease. Perioperative effects and safety of nesiritide following cardiac surgery in children. Effect of nitric oxide on oxygenation and hemodynamics in infants after cardiac surgery. Milrinone and nitric oxide: combined effect on pulmonary artery pressures after cardiopulmonary bypass in chlldren. Pharmacokinetics and safety of intravenously administered citrulline in children undergoing congenital heart surgery: potential therapy for postoperative pulmonary hypertension. Fenoldopam for controlled hypotension during spinal fusion in children and adolescents. A review of calcium channel antagonists in the treatment of pediatric hypertension. Intravenous nicardipine for treatment of postcoarctectorny hypertension in children. Magnesium supplementation during cardiopulmonary bypass to prevent juncrional ectopic tachycardia after pediatric cardiac surgery: a randomized controlled study. Amiodarone as a first-line therapy for postoperative junctional ectopic tachycardia. Decreased catecholamine sensitivity and beta receptor densiry in failing human hearts. Beta-receptor downregulation in congenital heart disease: a risk factor for complications after surgical repair Carvedilol as therapy in pediatric heart failure: an initial multicenter experience. Esmolol for the treatment of hypercyanotic spells in infants with terralogy of Fallot. Levosimendan enhances cardiac performance after cardiopulmonary bypass: a prospective, randomized placebo-controlled triaL] Cardiouasc PharmacoI1999;34:219-228. The change of plasma endorhelin-l levels before and after surgery with or without Down syndrome. Assessment of rhe pulmonary circulation in patients with functionally univentricular physiology. Early postoperative care of patients with pulmonary hypertension associated with congenital cardiac disease. Inhaled nitric oxide and prevention of pulmonary hypertension after congenital heart surgery: a randomised double-blind study. The effects of inhaled nitric oxide on postoperative pulmonary hypertension in infants and children undergoing surgical repair of congenital heart disease. Inhaled prostacyclin following surgical repair of congenital heart disease-a pilot study. Changes in cerebral saturation profile in response to mechanical ventilation alterations in infants with bidirectional superior cavopulmonary connection. Effect of carbon dioxide on systemic oxygenation, oxygen consumption, and blood lactate levels after bidirectional superior cavopulmonary anastomosis. Hyperventilation versus standard ventilation for infants in postoperative care for congenital heart defects with pulmonary hypertension. Have changes in ventilation practice improved outcome in children with acute lung injury Intravenous nesiritide, a natriuretic peptide, in the treatment of decompensated congestive heart failure. Hemodynamic and renal excretory effects of human brain natriuretic peptide infusions in patients with congestive heart failure: a double blind, placebo controlled, randomized crossover trial. Ventilator-associated pneumonia in the pediatric intensive care unit: characterizing the problem and implementing a sustainable solution. Perioperative risk factors for prolonged mechanical ventilation following cardiac surgery in neonates and young infants. Corticosteroids for the prevention and treatment of post-extubation stridor in neonares, children and adults. Children with corrected or palliated congenital heart disease on home mechanical ventilation. Factors affecting the decision to defer endotracheal extubation after surgery for congenital heart disease: a prospective observational study. Insights in pediatric ventilation: timing of intubation, ventilatory strategies, and weaning. Handover after pediatric heart surgery: a simple tool improves information exchange. Principles of antegrade cerebral perfusion during arch reconstruction in newborns/infants. Tissue factor-activated rhrornboelastograrns in children undergoing cardiac surgery: baseline values and comparisons. Fresh whole blood versus reconstituted blood for pump priming in heart surgery in infants.

This pathophysiology is in distinction from that occurring in the other well-recognized cardiopulmonary complication serum cholesterol ratio uk cheap rosuvastatin 10 mg line, portopulmonary hypertension cholesterol lowering diet plan pdf buy rosuvastatin, which is characterized by abnormal pulmonary vasoconstriction and obliterative vascular remodeling cholesterol hdl ratio too low buy rosuvastatin with a visa. Alteration in the hepatic synthesis or metabolism of vasoactive pulmonary substances cholesterol in dried shrimp order 10 mg rosuvastatin, nitric oxide and possibly endothelin-1 cholesterol ratio scale order 10 mg rosuvastatin mastercard, are believed to be integral to the development of intrapulmonary vascular dilation in the hepatopulmonary syndrome (126 cholesterol levels by food discount rosuvastatin 10 mg overnight delivery,128). A particularly interesting congenital etiology of the hepatopulmonary syndrome is the Abernethy Malformation because its initial presentation is one of dyspnea and cyanosis rather than of frank liver disease (127). The malformation is due to congenital absence of the portal vein, which results in a diversion of portal blood away from the liver and directly into the vena cava. For example, a right antecubital injection is sufficient if one needs to opacify the right heart for investigation of an intracardiac right-to-Ieft shunt. In the case of unusual Glenn and Fontan connections it may be necessary to inject into a lower extremity vein to preferentially fill the left pulmonary artery. Sometimes agitated saline contrast should be administered through central catheters during cardiac catheterization, for example, to pinpoint the exact location of a Fontan leak. Two syringes (one empty, the other filled with saline [-3 mL for newborn, -20 mL for adult]) are connected to a stopcock. The stopcock is connected to the intravenous line close to its entry into the body. Agitation is achieved by turning the stopcock off to the patient and forcefully pushing the saline (or saline/blood mixture) alternately between the two syringes for approximately 30 seconds. The sonographer should obtain a view (usually the apical four-chamber view) in which both right and left heart structures are imaged simultaneously so that the contrast can be visualized passing (a) through the right heart to verify that the contrast injection was indeed adequate and (b) through the left heart to verify if a right-to-left shunt is indeed present. The stopcock is then turned on to the patient and the contrast is pushed rapidly into the vein. In older patients, a Valsalva maneuver may be performed to enhance the ability to detect a right-to-Ieft shunt. Indications Agitated saline contrast is helpful whenever a right-to-Ieft intrapulmonary or intracardiac shunt is suspected but cannot be detected or definitively diagnosed by standard echocardiographic modalities. The micro spheres consist of an internal gas (air or fluorocarbon) encapsulated by an external shell (aggregated albumin, galactose, or lipid). After an intravenous injection, the micro spheres will follow the downstream course of the blood into the right heart and pulmonary vasculature. The microspheres are sufficiently small and the diffusion of the gas is sufficiently limited by its low partition coefficient that the micro spheres pass through the capillary bed into the left heart. Because the acoustic impedance of the microspheres is much lower than that of the blood, the ultrasound waves are scattered and reflected at the microsphere-blocd interface. Now, the digital laboratory of our present uses technology enabling gray scale storage of each pixel in the echocardiographic image by conversion into a series of binary digits. For example, an 8-bit depth binary system stores the image as series of eight digits. This level is more than sufficient when one considers that the human eye can distinguish only 50 shades of gray. Typically, a freeze-frame ultrasound image has 512 lines of information with 512 samples per line, resulting in approximately 262,000 pixels per frame. Each pixel is described by an 8-bit binary system, resulting in approximately 2 million bits of data to describe a single frame. Guidelines for the management of this massive amount of digital echocardiographic data have been developed by the American Society of Echocardiography (132). Lossless compression ensures that no data are lost but provides only a modest 3:1 reduction in data storage. Although pediatric dosages have not been established, 50% of the adult dose produces excellent left heart opacification without side effects (130). The ultrasound system should be set to low power or mechanical index so that bubble destruction is minimized. With this setting, the myocardium will appear black and the contrast-filled cavity will be white. In adults, adverse reactions are extremely rare and when present consist of allergic reaction, headache, flushing, and nausea. In the presence of an intracardiac shunt, the microspheres can bypass filtering by the lung and enter the arterial circulation directly. It is believed that the larger (up to 32 fl) microspheres which constitute a very small percentage of the total suspension and which are normally filtered by the lungs can pass into the left heart and produce arterial occlusions in this setting. Therefore, no transpulmonary contrast agent should ever be administered to a patient with a known or suspected intra cardiac shunt. Therefore, they are indicated when traditional echocardiographic modalities yield suboptimal myocardial and endocardial visualization. Most often this is necessary during stress echocardiography when visualization of all myocardial segments is required to adequately assess integrity or compromise of coronary perfusion (130,131). Although the noncontrast image (A) is reasonably clear and most wall segments can be identified, the contrast image (B) makes identification of all wall segments (particularly those at the apex) easy. Despite the large amount of data created with digital echocardiographic image acquisition, image management has been relatively facile and has indeed improved efficiency in the pediatric echocardiography laboratory (136). Computer technology has continued to improve so that images have been routinely attached to web-based journal articles (137). Importantly, images can be transferred easily through web-based networks and onto personal digital devices allowing for reading of echocardiograms from and in remote sites as long as web access is available (138). Acquiring and reading echo cardiograms in this manner has been shown to be diagnostically accurate (139). Further, this technology has had profound impact on (a) more timely diagnosis of critically ill patients, (b) better determination of need for cardiology consult, and (c) preventing unnecessary transfers for the purpose of merely obtaining an echocardiogram (140-142). Echocardiography has also been instrumental in animal research, most notably in the phenotyping of transgenic mice (154-159). This prohibited not only acquisition of longitudinal data and also further breeding. Parasternal and apical images are obtained and quantitative data include left ventricular dimensions, function, mass, and aortic and mitral valve Doppler velocities. If the animal has undergone microcatheterization of the left ventricle or a systemic artery, pressure data can be coupled with simultaneous echocardiographic left ventricular dimensional data to create pressure-dimension loops or end-systolic wall stress data, powerful indices of ventricular function (see above). The American Society of Echocardiography has established guidelines for the appropriate use of echocardiography in clinical trials (143). Echocardiography has been used successfully to provide mechanistic insights into disease processes and therapeutic outcomes, to provide both cross-sectional and longitudinal data in large epidemiologic studies, and to measure functional and structural changes now considered to be end points. The Framingham Heart Study was the first epidemiologic study to use echocardiographic measurements (144). The single largest application of echocardiography in epidemiologic studies has been the measurement of left ventricular mass and its change with antihypertensive therapy (145-149). Recently, the use of echocardiography has been expanded to clinical trials investigating cardiac resynchronization therapy for congestive heart failure. Echocardiography has been instrumental to demonstrate that remodeling due to resynchronization therapy is associated with less risk of subsequent ventricular tachyarrhythmia (150). In addition, echocardiography is the major tool for providing detailed phenotypes for large human genetic studies (151). A minimum number of sonographers and readers should be used to limit the effects of interobserver variability. Left ventricular pressure-dimension loops in control mice (thin lines) and hypothyroid mice (thick lines) derived from simultaneous M-mode echocardiography and catheterization of the left ventricle. Afterload was manipulated with phenylephrine and nitroprusside infusion to create a family of pressure-dimension loops from which end-systolic relations can be measured. At any given end-systolic dimension,the end-systolic pressure is less in the hypothyroid mice versus the control mice indicating depressed contractility in the hypothyroid mice. This echocardiographic technique, more appropriately called ultrasound biomicroscopy, involves mounting the anesthetized pup or pregnant ewe on a microscope stage embedded with heating elements to keep the animal warm and which can be moved spatially in x, y, and z planes using three micromanipulator knobs similar to using a microscope. Embryonic mice imaging is extremely valuable since many transgenic mice models are embryonic lethal. Doppler signals are apparent as early as the eighth embryonic day, essentially the heart tube stage (gestational age of mouse = 18. Resolution has improved significantly with these higher frequency transducers such that it will now be possible to phenotype transgenic mice models targeted for maldevelopment of the cardiac valves. Using such equipment, investigators have studied the origins of adult cardiovascular disease. Specifically, it has been shown in transgenic studies, for example, that prenatal diet has profound influences on adult cardiovascular mechanics as assessed by echocardiography (167). Now, echocardiography shares the stage of noninvasive cardiac imaging with a variety of other modalities, including cardiac magnetic resonance imaging, positron emission tomography, metabolic imaging, and perfusion imaging. Echocardiographers and other pediatric cardiologists need to understand that these modalities complement, rather than replace, echocardiography. For example, cardiac magnetic resonance imaging excels in imaging both extracardiac anatomy and also the spatial relationships of cardiovascular anatomy in relation to other structures in the thoracic cavity. The former has been difficult and the latter impossible for the pediatric echocardiographer. Because of these complementary niches of the various imaging modalities, patient care has improved to unprecedented levels. Nevertheless, the myriad of diagnostic tests provides echocardiographers with at least two challenges. There may be a tendency to become less rigorous during the echocardiographic exam knowing that other imaging modalities may later be employed. If difficult anatomy can be diagnosed accurately with echocardiography, the expense, the inconvenience, and potential risk of other imaging modalities will be avoided. We need to remain compulsive and true to our specialty and continue to bring the same rigor and compulsiveness to the exam that we have employed in the past. This will involve not only efforts at the bedside and in the echocardiography laboratory but also efforts at a more communal level such as the continued development of guidelines and standards for the performance of quality echocardiographic examinations (168,169). The second challenge is one of "imaging responsibility" to our patients and our health care system. We must realize that we are responsible for recognizing and resisting the lure of employing all of the diagnostic armamentarium available to us. The challenge is to partner with our colleagues managing these other imaging modalities to develop pathways in the diagnostic approach for our patients. Such an approach will necessarily be diagnosis- and age- specific and, indeed, in some instances may not utilize echocardiography. In all cases, however, imagers must join together to develop strategies that bring the greatest value to the patient. Because of continued miniaturization of computer and ultrasound equipment, hand-carried ultrasound devices have been used for medical diagnostics. Most of these devices now have the capabilities of even the most technologically advanced ultrasound systems but are the size of a small laptop computer or even a mobile phone. Using such devices, echocardiographers can provide point-of-service care more effectively. More importantly, these devices expand the community that can now receive and benefit from the diagnostic power of echocardiography. In addition, the devices improve diagnostic accuracy by complementing the cardiac physical examination in even tertiary care centers. Although auscultation has been the traditional foundation of the cardiac physical examination, many primary care physicians and even some cardiologists have imperfect auscultatory skills. For example, diagnostic accuracy of current resident physicians using auscultation alone is notoriously poor (170-172). It is likely that these skills will continue to decline as resident physicians cope with the competing forces of having to learn vast amounts of newer medical information within the time constraints imposed by resident work hour regulations. Handcarried ultrasound devices extend the diagnostic accuracy of echocardiography from the ultrasound laboratory to the time and place of the physical examination. By complementing stethoscope use with a hand-held system, an examiner can not only hear the heart but see it as well, thereby improving diagnostic accuracy (173). Implementation of a hand-carried ultrasound device program into the medical school curriculum significantly increases the diagnostic accuracy of the physical examination performed by medical students (174). Even the accuracy of the cardiovascular examination performed by board-certified cardiologists is enhanced by their use of a hand-carried device (175,176). In pediatrics, hand-held ultrasound has been shown to have similar diagnostic accuracy as traditional ultrasound systems and its use is speculated to only increase (177). The increased availability of echocardiography made possible by hand-carried devices has tempted other noncardiac specialists to practice cardiac ultrasound (178,179). While this has the potential benefit of enhancing overall patient care by improving diagnostic accuracy, it also emphasizes the need for responsible practice of ultrasound. It is the duty of the echocardiography community to develop standards for the practice of hand-carried ultrasound and ensure that they are met (180). Most physicians who use a stethoscope are not cardiologists; the diagnostic accuracy of the stethoscope varies according to its user, and cardiologists have greater expertise in its use. Likewise, hand-carried ultrasound devices are currently being used by a variety of health care providers, for example, emergency room physicians, neonatologists, and even robots (181-184). However, as with the stethoscope, it would be expected and need to be indoctrinated as standard of care, that when a noncardiologist identified a patient with suspected pathology using a hand-held device, the patient would be referred to a cardiologist for further evaluation. The development and expansion of hand-held ultrasound devices speaks to us as physicians, specifically to the reasons as to why many of us chose medicine as our career. We have a powerful, robust tool in echocardiography; a tool with which we can do much good by providing very advanced medical care to an even vaster population. There is great value in the fact that hand-held devices allow us to provide increased availability to our tertiary care populations at surrounding satellite clinics improving medical care by obviating the need for lengthy, stressful and time-consuming journeys to the central facility. Echocardiographic assessment of juxtaposition of the right atrial appendage in children with congenital heart disease.

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