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According to Richard order levitra soft with mastercard, she was a wonderful teacher discount levitra soft online, chef order cheap levitra soft line, and role model (especially for young female fellows). Willis Potts to clarify the abnormal findings in children who had died of congenital heart disease. He directed congenital heart disease research at the Hektoen Institute from 1957 to 1982. He taught and practiced cardiac pathology for almost all the hospitals and all the medical schools in Chicago and was the unifying force for our discipline in that city for years. He moved to New Jersey and directed laboratories at Deborah Heart and Lung Center from 1982 to 1988. He moved back to Chicago and until his death helped direct the Congenital Heart and Conduction System Center. He is known for coauthoring a major pediatric cardiology textbook, Heart Disease in Children. He was a master teacher and attracted students from all over the world to his laboratory. He was an incredibly meticulous observer, and defined many of the conduction system anatomy and abnormalities seen in various forms of congenital heart disease. Two of his books were published in the 1990s; he authored or coauthored over 500 papers. Anderson (1942-) (information (22) and from personal encounters) was trained as an anatomist, not a pathologist. He was to study ophthalmology, but did his thesis in the Anatomy department (University of Manchester) where he became fascinated with the intricacies of a congenital heart specimen that had been obtained after a surgical suture interrupted the conduction bundle. This led to a career spent carefully and accurately defining conduction areas of the heart and clarifying the deranged anatomy seen in various forms of congenital heart disease, especially the atrioventricular septal defect. These observations have saved many children undergoing congenital heart operations because the surgeon, now aware of Dr. His “Andersonian” terminology is very logical and clarifies much of what we see daily. His wife, Christine, accompanies him on his various trips and is an excellent teacher herself. The Surgeons Ludwig Rehn (1849–1930) was the first surgeon to operate on the heart. Although many years earlier, the Aztecs had perfected cardiectomy, the first heart operation with intent toward saving lives was not done until 1896. He was an excellent observor; a year earlier he had shown that a chemical caused bladder cancer in aniline dye workers. He described a heart operation that he did in a 22 year old who had been stabbed during an altercation in a park the night before. After unsuccessful treatment with rest, icebags, and camphor injection, he began to deteriorate. Proving them wrong, by 1907 he had collected 124 cases of “heart suture” with 60% mortality (vs. Gross (1905–1988) first planned upon becoming a surgical pathologist and trained for 3 years at the Peter Bent Brigham, then went to Harvard for surgical training. At the age of 33 years, while a surgical chief resident on August 26, 1938, he waited until his chief, Dr. William Ladd, was out of town (but had permission from the acting chief), and operated a 7-year-old girl who had a 7-mm patent arterial duct, closing it with a single no. Ladd had no choice but to rehire him under political pressure and against his own wishes. Taussig visited him in the late 1930s and tried to convince him to try the shunt concept that she had developed, he said “Madam, I close ductuses, I do not make new ductuses”. Gross had many honors throughout his career, including being the first president of the American Pediatric Surgical Association and recipient of two Lasker Awards. Clarence Crawfoord (1899–1984) was at the Karolinska institute, Stockholm, Sweden. Crawfoord had done two successful pulmonary embolectomies, and in the 30s he introduced the concept of heparin prophylaxis for pulmonary emboli. He did successful pneumonectomies after he pioneered positive pressure mechanical ventilation in the 40s. Ake Senning did the first atrial switch operation for transposition and he implanted the first pacemaker. He was a talented violinist and did professional training at the Stockholm Musical Academy. He did an internship in urology but his assistant residency in general surgery was not renewed. Part of their work was to create a pulmonary hypertension model that was abandoned because pulmonary hypertension did not result from the subclavian swingdown done in the dogs. He became chief of surgery at Johns Hopkins in 1941 (after several bouts of tuberculosis) and brought Mr. Helen Taussig, he created a ductus-like situation using the previous pulmonary hypertension dog concept—subclavian artery swingdown. Thomas standing over his shoulder and guiding most of the operation, the tetralogy patient survived and became a “lovely colour” (Dr. In 1948 he developed a dilator to open stenotic pulmonary valves in patients with pulmonary stenosis and tetralogy patients. He did many mitral finger fracture valvotomies for rheumatic patients with mitral valve stenosis, a technique first developed by Dr. Henry Souttar in 1925, but Souttar was so roundly criticized by his colleagues that he abandoned the technique (31). Blalock spent time with him and their collaboration continued to the point that Brock developed a bypass pump and used hypothermia, bringing the open heart technique to England. He was known as an exacting person who did not tolerate laziness or unclear thinking. He was knighted in 1954 and received several awards for his expertise and contributions. He completed his degree at Johns Hopkins, interned there, and worked with Blalock whom he assisted along with Longmire on the first Blalock–Thomas–Taussig shunt. His training was interrupted by a stint in the Army Medical Corps where he was Chief of surgical services in Linz, Austria. He came back to Hopkins and finished training and remained as an instructor in surgery. He says that the main reason he was hired by Blalock was to “keep those damn women out of my hair”—Drs. Taussig, Wittemore, Hanson, and Engle made evening rounds on postoperative patients and were calling Dr.

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Global gene expression profiling identifies new therapeutic targets in acute Kawasaki disease cheap levitra soft 20mg line. A genome-wide association study identifies three new risk loci for Kawasaki disease order levitra soft 20mg on-line. Role of activating FcγR gene polymorphisms in Kawasaki disease susceptibility and intravenous immunoglobulin response purchase levitra soft toronto. Transforming growth factor-beta signaling pathway in patients with Kawasaki disease. Cyclosporin A treatment for Kawasaki disease refractory to initial and additional intravenous immunoglobulin. Calcineurin inhibitor treatment of intravenous immunoglobulin-resistant Kawasaki disease. Three linked vasculopathic processes characterize Kawasaki disease: a light and transmission electron microscopic study. Kawasaki disease patients with redness or crust formation at the Bacille Calmette-Guérin inoculation site. Erythrocyte sedimentation rate and C- reactive protein discrepancy and high prevalence of coronary artery abnormalities in Kawasaki disease. Elevated gamma- glutamyltransferase concentrations in patients with acute Kawasaki disease. Immune hemolysis, disseminated intravascular coagulation, and serum sickness after large doses of immune globulin given intravenously for Kawasaki disease. Hemolytic anemia following intravenous immunoglobulin therapy in patients treated for Kawasaki disease: a report of 4 cases. Incidence rate of recurrent Kawasaki disease and related risk factors: from the results of nationwide surveys of Kawasaki disease in Japan. Systemic onset juvenile idiopathic arthritis with macrophage activation syndrome misdiagnosed as Kawasaki disease: case report and literature review. Coronary artery abnormalities in children with systemic-onset juvenile idiopathic arthritis. Performance of 2004 American Heart Association recommendations for treatment of Kawasaki disease. Clinical characteristics and serum N-terminal pro-brain natriuretic peptide as a diagnostic marker of Kawasaki disease in infants younger than 3 months of age. Changes in cardiac troponin I in Kawasaki disease before and after treatment with intravenous gammaglobulin. Noncoronary cardiac abnormalities are associated with coronary artery dilation and with laboratory inflammatory markers in acute Kawasaki disease. Clinical manifestations associated with Kawasaki disease shock syndrome in Mexican children. Repeated quantitative angiograms in coronary arterial aneurysm in Kawasaki disease. Long-term prognosis of giant coronary aneurysm in Kawasaki disease: an angiographic study. Coronary artery involvement in Kawasaki syndrome in Manhattan, New York: risk factors and role of aspirin. Correlates of coronary artery aneurysm formation in patients with Kawasaki disease. Assessment of Kawasaki disease risk scores for predicting coronary artery aneurysms at a North American Center. Long-term prognosis of patients with Kawasaki disease complicated by giant coronary aneurysms: a single-institution experience. Prevalence of coronary artery lesions on the initial echocardiogram in Kawasaki syndrome. Mucocutaneous lymph node syndrome (Kawasaki disease): delayed aortic and mitral insufficiency secondary to active valvulitis. Sensitivity, specificity and predictive value of two-dimensional echocardiography in detecting coronary artery aneurysms in patients with Kawasaki disease. Report of Subcommittee on Standardization of Diagnostic Criteria and Reporting of Coronary Artery Lesions in Kawasaki Disease. Improved classification of coronary artery abnormalities based only on coronary artery z-scores after Kawasaki disease. Coronary artery caliber in normal children and patients with Kawasaki disease but without aneurysms: an echocardiographic and angiographic study. Dobutamine stress echocardiography for detection of coronary artery stenosis in children with Kawasaki disease. Dobutamine stress echocardiography in the assessment of suspected myocardial ischemia in children and young adults. Assessment of ischemic heart disease using magnetic resonance first-pass perfusion imaging. Microvasculature in acute myocardial ischemia: part I: evolving concepts in pathophysiology, diagnosis, and treatment. Is there a role for intravenous transpulmonary contrast imaging in pediatric stress echocardiography? Assessment of the ability of myocardial contrast echocardiography with harmonic power Doppler imaging to identify perfusion abnormalities in patients with Kawasaki disease at rest and during dipyridamole stress. Discordance between thallium-201 scintigraphy and coronary angiography in patients with Kawasaki disease: myocardial ischemia with normal coronary angiogram. Long-term prognostic impact of dobutamine stress echocardiography in patients with Kawasaki disease and coronary artery lesions: a 15-year follow-up study. Arterial complications associated with cardiac catheterization in pediatric patients with a previous history of Kawasaki disease. Coronary magnetic resonance angiography in adolescents and young adults with Kawasaki disease. Magnetic resonance angiography is equivalent to X- ray coronary angiography for the evaluation of coronary arteries in Kawasaki disease. Detection of active coronary arterial vasculitis using magnetic resonance imaging in Kawasaki disease. Coronary computed tomographic angiographic findings in patients with Kawasaki disease. Assessment of coronary artery abnormalities by multislice spiral computed tomography in adolescents and young adults with Kawasaki disease. Carotid intima-media thickness and pulse wave velocity after recovery from Kawasaki disease. Novel and traditional cardiovascular risk factors in children after Kawasaki disease: implications for premature atherosclerosis. Are patients after Kawasaki disease at increased risk for accelerated atherosclerosis?

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At the level of regional or segmental function cheap levitra soft 20mg overnight delivery, regional force development within a segment will result in regional myocardial deformation buy discount levitra soft 20mg line. At the segmental level generic 20mg levitra soft visa, myocardial force is better described as regional wall stress that is influenced by active contractile force development, pressure, wall geometry (wall thickness, regional wall curvature), and segmental interaction. Current echocardiographic techniques allow quantification of regional myocardial deformation as segment shortening, thickening, and rotation (also called regional myocardial strain or deformation). Global pump function is the product of interaction between the different contractile segments resulting in ventricular pressure generation and, when the outlet valve opens, ejection of blood from the ventricle. On the pump level, ventricular performance is determined by myocardial function (influenced by preload, afterload, and heart rate) and efficient segment interaction (synchronicity of contraction). For interpretation of measurements, it is important to know which physiologic parameters influence the echocardiographic parameters. All too often, measurements are determined to be indices of “contractility,” while there are very few, if any, that are not influenced by loading conditions. Knowledge on the reliability, reproducibility, and accuracy of the methods to assess ventricular function will also influence interpretation of the results. On the segmental level, regional wall stress is the composite of regional force development and loading on the regional segment. Echocardiographically, regional wall motion and deformation can be studied by tissue Doppler and myocardial-deformation imaging. On the pump level, generation of ventricular pressure results in ejection of blood. This can be assessed using ejection parameters like ejection fraction by echocardiography. A wide variety of different echocardiographic parameters and indices has been developed for assessing ventricular function. This in itself indicates that no single parameter adequately provides all the necessary information. The echocardiographer needs to integrate information from different parameters to comprehensively describe systolic function. In this chapter, the most commonly used indices will be discussed with a description of their measurement, reproducibility, accuracy, availability of normal values, and the influence of loading conditions. Percent shortening fraction was traditionally measured using M-mode echocardiography from either the parasternal long-axis or short-axis view just below the level of the mitral valve leaflets. It can also be difficult to identify end diastole and end systole on 2-D short- axis views. Values <28% suggest reduced systolic function, while values >38% indicate hyperdynamic function. After adequate standardization of acquisition and analysis, variability should be between 10% and 15%. This assumes that there are no regional differences in wall motion while, in reality, different conditions are associated with regional wall motion abnormalities. This can cause paradoxical septal motion with the septum moving away from the inferolateral wall during systole (Fig. This does not reflect an acute decrease in myocardial contractility but rather the increased loading on the heart. When hypertrophy of the wall occurs, as happens in the context of chronic arterial hypertension or hypertrophic cardiomyopathy, endocardial changes and chamber dimension changes are influenced by the thickened wall resulting in an overestimation of systolic function. When corrected for afterload, the measurement becomes a good parameter for contractility. As “fiber shortening” is calculated by measuring ventricular dimensional changes, the same assumptions can be made to calculate “wall stress. Higher ventricular pressure and larger ventricular size increase wall stress while a thicker wall reduces wall stress. Assumptions for both meridional and circumferential wall stress can be derived from M-mode measurements, pressure measurements, and initially, carotid pulse tracing was used to estimate end-systolic wall stress. While peak stress determines the degree of hypertrophy, end-systolic stress is the most important parameter determining systolic shortening (26). The formula that is used to calculate meridional (longitudinal) end-systolic wall stress is 2 where 1. Simplified versions include using mean or peak systolic pressures instead of end-systolic estimated pressures (27). This seems logical as higherc afterload can be expected to reduce the velocity of fiber shortening for the same myocardial contractility. In younger children, the linearity of the relationship was questioned and it was shown that wall stress as calculated in the formula misrepresents afterload in children and young adults with abnormal left geometry (30). The method has been applied in a number of different clinical conditions, especially for the evaluation of cardiac contractility in pediatric patients exposed to anthracyclines. As an alternative to measuring geometrical changes, Doppler data have been used to quantify ventricular systolic function. Initially, blood pool velocity measurements were made, and more recently tissue Doppler was introduced to measure the velocity of myocardial motion. The advantage of these methods is that they can be obtained independently of ventricular geometry. Practically, dt is calculated between 1 m/s and 3 m/s; dP between those two time points calculated by the Bernoulli equation is 32 mm Hg. As dP/dt is measured before aortic valve opening, it is independent from changes in afterload but its measurement is influenced by preload changes. As the time interval measured on the Doppler trace is very short and the settings used to obtain the spectral Doppler tracings can be variable, the reliability and accuracy of the method are limited. Assessment of cardiac function by measuring blood flow velocity during ventricular ejection is another logical approach. Doppler signals across the aortic and pulmonary valves can provide timing intervals that are used to assess ventricular function. A logical next step is to combine inflow and outflow Doppler measurements in the assessment of ventricular function. Nevertheless, in certain diseases like pulmonary hypertension, amyloid heart disease, and pulmonary hypertension, it has strong predictive value (34,35). Apart from measuring flow velocities, Doppler has also been used for measuring myocardial velocities or tissue Doppler velocities. Tissue velocities are lower than most blood pool velocities but have higher amplitudes. Thus by adjusting Doppler filter settings, tissue Doppler velocities can be selectively measured (Fig. Pulsed- wave tissue Doppler was developed first, followed by color tissue Doppler. Pulsed Doppler typically measures velocities in a single segment while color tissue Doppler measures velocities in an entire wall or chamber. Therefore, color tissue Doppler velocities are approximately 15% to 20% lower than pulsed Doppler velocities. Color Doppler has the advantage of measuring velocities in different myocardial segments simultaneously while pulsed Doppler samples a single segment in a given time.

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Surface cooling before bypass is almost certainly use- In addition to improving vision buy 20 mg levitra soft fast delivery, loupes are an important ful order levitra soft 20 mg mastercard. Therefore discount levitra soft 20 mg with visa, the room temperature should be maintained aid to optimal use of the surgical headlight. The feld of at less than 17–18°C until all clamps have been released and vision through the loupes must be carefully and accurately the patient is being rewarmed at full fow. Since almost all congenital cardiac surgery is in a sense ‘minimally invasive’ in that the cardiac incisions must be limited, it is often diffcult for all members of the team to be able to follow the progress of the procedure. These instruments are designed to Furthermore, the picture from the headlight camera can be be controlled by the fngers rather than by the arms. Unlike adults, children rarely complain of back, era, as well as an overhead camera mounted in the overhead neck or interscapular pain following a sternotomy. This composite sion does not require stretching, cutting or tearing of any image can be connected via the Internet to viewing stations muscles, unlike so many other incisions. The blood supply elsewhere in the hospital such as the senior surgeon’s offce of the bone is excellent in children so that healing is usually or remotely allowing a junior surgeon to call for input from rapid and complete. For example, the Norwood procedure with should be performed using small instruments. While it is a Blalock shunt involves work on the great vessels and right true that many of the instruments used for congenital car- subclavian artery so that the incision should extend up to the diac surgery need to be delicate, they should not necessarily sternal notch. They need to be long enough to allow three pairs sion can be limited and does not need to extend to the bot- of hands (the surgeon and two assistants) to simultaneously tom of the xiphoid process. On the other hand, the depth procedures, however, the top end of the skin incision can be of the surgical feld in neonates and infants is very much limited to end some distance below the sternal notch. This is an important cosmetic consideration since the major dis- is an important advantage for the surgeon in that it allows advantage of the standard incision is the fact that it can be the hand to be stabilized on the chest wall. By limiting the diac surgeons can use time in the dentist’s chair proftably upper end of the incision it is possible to conceal the incision by analyzing the methods by which dentists and hygienists with most clothing. As with It is critically important that the bone incision be exactly dental instruments, most of the movement of instruments in the midline. The width of the sternum varies tremendously used by the congenital cardiac surgeon should be controlled between children and it may be very narrow. If the incision by the fne muscles of the hand and not by the forearm and is made off midline there is a real risk that the sternal wires shoulder girdle muscles. Microvascular instruments, such as will cut through the delicate cartilaginous bone resulting in the Castro–Viejo needle holder are specifcally designed to an unstable sternum and poor healing. An unstable sternum be controlled by the fngers rather than by the arms and are will increase the risk of mediastinal infection. Relative to most other surgical incisions, it has There are a number of options to improve the cosmetic less postoperative pain, particularly in young children who appearance of the standard sternotomy incision. Opening the been possible to prove that minimally invasive incisions sternum requires hinging of the ribs at the costovertebral reduce pain or speed convalescence. It should be is suffciently fexible in children that there seems to be little exceedingly rare that axillary artery cannulation is required, or no advantage in ‘T-ing’ off the incision to one or other side. Carotid artery cannulation is not recommended other can be kept entirely below the level of the nipples. While this than in extreme situations because there is a risk that cerebral limited incision allows for safe closure of septal defects, we blood fow will be compromised. ReopeRaTiVe sTeRnoTomy The previous skin scar is usually excised and the sternal Reopening a sternal incision can be done safely as long as wires are cut and removed. Planning begins with and the linea alba is opened to allow a plane to be developed the preoperative studies which should document the distance behind the lower end of the sternum. The used to elevate the lower end of the sternum off the heart and sternal wires are quite helpful as markers on a direct antero- to provide a counter pressure to the oscillating sternal saw. If a conduit is known to be close to the left side of the status of the femoral and iliac vessels. This knowledge the sternum it may be advisable to free up only the right half may be available from the preoperative catheterization, from of the sternum until the retractor is placed. However, ulti- femoral ultrasound studies or simply from careful palpation mately dissection should extend to the pleural cavities bilat- of the femoral pulses and observation for evidence of previ- erally. At least one groin should be prepped completion of the procedure, but more importantly it allows into the surgical feld. Injury to the right heart can gener- the heart to be moved around more freely, thereby improving ally be dealt with easily by cannulating the femoral artery exposure without having to retract the chambers of the heart and placing a pump sucker in the injured structure. Even emergency cannulation of the femoral ves- nal retractor is in place, dissection is begun using the elec- sels after an aortic injury has occurred will not be helpful trocautery. Dissection should be begun in the space between because blood pumped into the arterial system from any the diaphragm and the inferior surface of the heart which is cannulation site will simply exit via the site of aortic injury. Grasping the diaphragm with for- Certain anomalies carry a higher risk of injury to the aorta, ceps and moving it up and down helps to identify the correct most notably d-transposition of the great arteries. The space is traced rightwards until the right atrium is erative catheterization should include lateral images which identifed. Suffcient inferior right atrial free wall is cleared have a suffciently large frame size to show both the ster- to allow placement of at least one venous cannula. In fact, it may be the surgeon’s choice to proceed with of the sternum and particularly if there is obvious adhesion cannulation at this stage. The remainder of the dissection can which will be apparent because of absence of relative move- proceed during the cooling phase of bypass. Decompression ment between the two structures, then femoral cannulation of the right atrium allows dissection in this area to proceed is required of both the femoral artery and vein before the more rapidly and safely. The child should be cooled on femoral bypass before the bone is cut in the vicinity of the aorta. If sTeRnaL cLosuRe the preoperative studies demonstrate that a right heart struc- ture is very close or adherent to the sternum, at a minimum The sternum is closed with wires of an appropriate gauge the femoral artery should be cannulated before the bone is for the child’s size. It is often wise to cannulate a femoral vein also with a advisable to place the wires through the costal cartilage to thin-walled cannula, such as the Biomedicus® cannula. Ischemic necrosis caused by cannula should be advanced to the level of the right atrium the encircling wires is rarely, if ever, seen. If the femo- wires with an inadequate depth of bite will often cut through ral arteries are occluded bilaterally, it may be necessary to the thin and delicate bone of the child’s sternum. Rather than following the femo- eight wires reduce the tension on each bite and do not appear ral vessels up under the inguinal ligament, it is preferable to interfere with sternal growth. Bleeding can be reduced by that is diffcult to control without leaving a large amount of accurate dissection in the less vascular planes that almost packing, it may be wise to leave the sternum open. This situ- always exist between structures, no matter how many previ- ation most commonly arises in neonates, but rarely beyond ous procedures have been performed.

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