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By U. Sigmor. Notre Dame de Namur University. 2019.

Left atrial size in children with hypertension: the influence of obesity discount 25mg clomiphene amex, blood pressure order generic clomiphene pills, and left ventricular mass buy cheap clomiphene 50 mg on line. Effect of lean body mass discount clomiphene american express, fat mass, blood pressure, and sexual maturation on left ventricular mass in children and adolescents. Improvement of left ventricular function after cardiac resynchronization therapy is predicted by tissue Doppler imaging echocardiography. Cardiac resynchronization therapy tailored by echocardiographic evaluation of ventricular asynchrony. Tissue Doppler imaging predicts improved systolic performance and reversed left ventricular remodeling during long-term cardiac resynchronization therapy. A novel tool to assess systolic asynchrony and identify responders of cardiac resynchronization therapy by tissue synchronization imaging. American Society of Echocardiography recommendations for use of echocardiography in clinical trials. Calcineurin Abeta gene targeting predisposes the myocardium to acute ischemia-induced apoptosis and dysfunction. Hypertrophic defect unmasked by calcineurin expression in asymptomatic tropomodulin overexpressing transgenic mice. End-systolic stress-velocity and pressure-dimension relationships by transthoracic echocardiography in mice. Comprehensive transthoracic cardiac imaging in mice using ultrasound biomicroscopy with anatomical confirmation by magnetic resonance imaging. Differences between echocardiographic measurements of left ventricular dimensions and function by local investigators and a core laboratory in a 2-year follow-up study of patients with an acute myocardial infarction. Use of hand-carried ultrasound devices to augment the accuracy of medical student bedside cardiac diagnoses. Physician-performed point-of-care echocardiography using a laptop platform compared with physical examination in the cardiovascular patient. Usefulness of a hand-carried cardiac ultrasound device to detect clinically significant valvular regurgitation in hospitalized patients. Will a handheld ultrasound scanner be applicable for screening for heart abnormalities in newborns and children? Use of a hand-carried ultrasound device by critical care physicians for the diagnosis of pericardial effusions, decreased cardiac function, and left ventricular enlargement in pediatric patients. Impact of telemedicine on the practice of pediatric cardiology in community hospitals. Application of a low cost telemedicine link to the diagnosis of neonatal congenital heart defects by remote consultation. Effectiveness of echocardiographic imaging by nurses to identify left ventricular systolic dysfunction in high-risk patients. Hand-carried cardiac ultrasound enhances healthcare delivery in developing countries. This paper identifies indications for outpatient pediatric echocardiography and graded “appropriateness” level for echocardiography in each indication. The impact of procedural sedation on diagnostic errors in pediatric echocardiography. These two papers investigate the factors that lead to diagnostic errors in pediatric echocardiography. The authors conclude that sedation is associated with lower likelihood of errors and that overnight or weekend studies are risk factors for poor quality. Exercise echocardiography demonstrates biventricular systolic dysfunction and reveals decreased left ventricular contractile reserve in children after tetralogy of Fallot repair. Linking pediatric obesity to subclinical alterations in cardiac structure and function. Friedberg Quantification of Chamber Dimensions and Cardiac Structures Accurate measurements of valves, chambers, and vessels are essential to the diagnosis and management of patients with congenital and pediatric heart disease. The adult guidelines were recently revised to include three- dimensional (3-D) echocardiography and myocardial deformation imaging (4). One of the important differences between measurements in adult and pediatric patients is the effect of growth or body size on measurements. To overcome this limitation, the use of z-scores has been proposed as a practical alternative (5). Z-scores for different cardiovascular structures have been published (6,7) but the effect of gender and race on cardiovascular measurements may necessitate establishing normal values based on population mix seen in a specific laboratory. Detailing the measurement of each individual cardiovascular structure is beyond the scope of this chapter. In this chapter, the evaluation of cardiac function and chamber quantification are discussed in more detail. M-mode measurements have a very high temporal resolution albeit at the expense of low spatial resolution. If the measurements are not well standardized, M-mode measurements can be highly variable. This study showed poor agreement between the core laboratory and local laboratory measurements with relatively wide limits of agreement. Especially at high heart rates, the identification of the frame representing end systole and end diastole can be more variable. Another limitation is that currently the majority of published normal z-score data are based on M-mode measurements and not on 2-D imaging. M-mode measurement is obtained from a parasternal short-axis view of the left ventricle just inferior to the mitral valve leaflets. Three-dimensional echocardiography overcomes this problem as full 3-D volumetric data sets can be acquired including the entire ventricle. This results in improved reproducibility as it eliminates observer bias in determining endocardial borders. Hence, the intra- and interobserver variability of 3-D methods are much lower compared to 2-D-based volumetric calculations. In pediatrics, a limitation of 3-D echocardiography is the lower temporal resolution related to the relative low volume rates. Additionally not every vendor has a high-frequency 3-D probe available, which can limit temporal resolution especially in smaller children. The first two images from the left show the apical four-chamber view in end diastole and end systole, respectively. The next two images are obtained from the apical two-chamber view also in end diastole and end systole, respectively. The formula cannot be used in patients with asymmetric hypertrophic cardiomyopathy. Various measurements obtained from the apical four-chamber view have been proposed (Fig. A problem with these measurements is the lack of normal pediatric data precluding calculation of z-scores. Studies have shown that good quality 3-D data sets can only be obtained in 55% to 75% of patients after tetralogy of Fallot repair (20,21,22). Studies have suggested that the larger the ventricle, the more important the underestimation. This is probably related to difficult visualization of the endocardial borders related to the coarse trabeculations. Quantification of the Univentricular Heart Assessing chamber dimensions of the univentricular heart can be challenging due to the variable morphology. First, it is important to know whether the dominant ventricle has left, right, or indeterminate morphology. Moreover, the type of palliation and associated volume loading will influence ventricular size. A shunted single ventricle will generally be larger compared to the same ventricle after total cavopulmonary anastomosis. Three-dimensional volumes can also be calculated based on full volumetric data sets.

These include auto- immune disorders generic clomiphene 100mg with mastercard, such as SlE 25mg clomiphene amex, and in renal diseases cheap 50 mg clomiphene, especially various types of glomerulonephritis generic clomiphene 25 mg overnight delivery. For this reason, complement measurement can also be used to monitor disease activity. Antigenic tests for total serum levels of complement factors C3 and C4 are also available. This is especially true in patients who are pregnant or who have concomitant infammatory disorders, for whom a signifcant decrease in complement levels can signal increased disease activity, even if the complement levels are still within the normal range. Protein electrophoresis is a technique by which serum or urine proteins are separated by application of an electrical feld to proteins loaded on a semisolid matrix. The resolution varies by assay, but proteins typically resolve into fve distinct regions, representing albumin and alpha-1, alpha-2, beta, and gamma globulins. Because the shapes and relative concentrations of each band differ in a variety of pathologic conditions, protein electrophoresis can be a useful adjunctive diag- nostic test for many diseases. However, its primary purpose is the detection, measurement, and moni- toring of monoclonal immunoglobulin generated by plasma cell neoplasms. This so-called “m spike” can be fur- ther analyzed by immunofxation electrophoresis, and this evaluation is required to defne the heavy and light chain constituents of this abnormal band so that they can be followed in subsequently collected specimens. Failure to do so may lead to contami- nation by plasma proteins, such as fbrinogen, that can confound interpretation of the serum protein electrophoresis. A history of stem cell transplant, acute- phase reaction, hemolysis, or iron defciency should be noted to aid in distinguishing bona fde m-spikes from oligoclonal bands and other confounding bands that can mimic m-spikes. Despite equal stoichiometry of heavy and light chains in intact immunoglobulin, plasma cells produce more light chains than heavy chains. Consequently, free light chains circulate in the blood and are excreted into urine. Their absolute values and the ratio between kappa and lambda light chains can be used as surrogate markers for monoclonal immunoglobulin in the diagnosis and monitoring of plasma cell myeloma. However, because they are cleared by the kidney, caution must be exer- cised in the interpretation of light chain concentrations in the urine of patients with renal insuffciency. While the light chain assays are a good screening tool, defni- tive diagnosis of monoclonal gammopathy requires the presence of an m-spike on serum and/or urine electrophoresis. Cryoglobulins are serum immunoglobulins that precipitate at tem- peratures lower than normal body temperature. Type i cryo- globulins are single monoclonal immunoglobulins, often associated with B cell or plasma cell neoplasms. Precipitation of cryoglobu- lins in serum can activate complement, leading to an infammatory vasculitis. Cryoglobulins are measured by allowing them to precipitate at 4°C, quantifying the volume of cryoglobulin (cryocrit), and analyzing the cryoglobulin content by protein electrophoresis. Samples should not be allowed to cool below 37°C until after centrifugation to pre- vent loss of precipitated cryoglobulins from the specimen prior to analysis. Also, samples should be drawn from a periph- eral vein, rather than from an intravenous line. Rheumatoid arthritis classi- fcation criteria: an American College of Rheumatology/ European league Against Rheumatism collaborative initiative. Clinical and immuno- logical features of drug-induced and infection-induced proteinase 3-antineutrophil cytoplasmic antibodies and myeloperoxidase-antineutrophil cytoplasmic antibodies and vasculitis. Color Atlas of Hematology: An Illustrated Field Guide Based on Profciency Testing. Clinical inter- pretation of antinuclear antibody tests in systemic rheu- matic diseases. Effcacy of advanced discriminating algorithms for screening on iron- defciency anemia and β-thalassemia trait. Hypereosinophilic syn- drome and clonal eosinophilia: point of care diagnos- tic algorithm and treatment update. Accurate Results in the Clinical Laboratory: A Guide to Error Detection and Correction. Zinc-induced copper defciency: a report of three cases initially rec- ognized on bone marrow examination. As the initial contact point, processing staff may examine a specimen and detect common preanalytical errors before the specimen is ana- lyzed. Mislabeling, wrong tube types, transportation delays, and other mistakes can affect patient results. By detecting and correcting problems before the specimen is placed on an analyzer, staff can prevent clinical mismanagement based on erroneous results. Clinicians may envi- sion their patient as the only one being analyzed by the laboratory, but in today’s highly automated clinical laboratory, specimens are lined up and analyzed solely based on the label/barcode on the side of the tube. Searching for a specifc specimen among racks of similar specimens can be labor intensive, so automated processes that archive and manage speci- men storage and retrieval can improve the labora- tory’s effciency. These additional processes are also based on information contained on the specimen label. Thus, clinicians must ensure that patients are properly identifed and specimens are uniquely and appropriately labeled before sending them to the laboratory. Samples can be mislabeled with another patient’s name or contain incorrect infor- mation, such as name misspelling or wrong demo- graphics such as age or sex. Partially labeled specimens contain two appropriate identifers, but may be miss- ing important information, such as specimen source or date/time of collection. Some cases may present unique situations that require individual consideration, despite the exist- ence of a labeling policy. Thus, pro- cessing staff need to be diligent of the potential for mistakes and verify the integrity of specimen iden- tifcation with each and every specimen arriving in the laboratory. Some additives prevent clotting and allow the analysis of plasma, while other additives inhibit glycolysis and metabolism. Color-coded tubes may also contain a gel barrier that facilitates sample pro- cessing. These different collection tubes have different intended purposes and are generally not interchange- able. Certain tests may require specifc types of collection tubes, processing, or transport prior to analysis. Errors in Specimen Transportation Delays in transportation or exposure of specimens to extreme temperatures during transit to a laboratory can affect test results. Couriers should monitor environmental conditions to ensure that specimens are maintained within specifed conditions. Specimen Processing Errors The technique and manner of specimen processing can impact the quality of laboratory analysis. Failure to sepa- rate cells from the serum/plasma portion of blood allows for continued cellular metabolism that leads to decreased glucose values. Vigorous mixing of blood prior to analysis can generate foam- ing, which can affect pipette volumes and also induce hemolysis. Specimens are barcoded during col- lection, and usually arrive at the laboratory ready for analysis. This automation greatly reduces the possibility of previously com- mon analytical errors such as mixing up aliquots, ordering incorrect tests within the laboratory, mak- ing dilution errors, and reporting results to the wrong patient. These analyzers fag results to be held by the instrument management system pending review by the technologist prior to release to the ordering physician. Thus, automation of laboratory analyzers is an essential tool that enables the technologist to identify those specimens with unusual characteris- tics and specimens that need repeat testing or sepa- rate handling. Mislabeled and mishandled specimens, inappropriate collection and transport, miscom- munication, and misunderstanding of protocols and procedures can generate erroneous results that are not detectable by automated chemistry instru- mentation. Careful attention to specimen quality is required both within the laboratory as well as out- side of the laboratory. Preanalytical errors in test ordering, specimen collection, transportation, and processing, as well as postanalytical errors in deliv- ery and communication of test results, contribute to overall error rates that are related to laboratory test- ing. Collection of specimens through indwell- ing catheters presents a deviation from routine phle- botomy practice. Clinical staff are tempted to utilize intravenous lines, because lines provide direct access to the patient’s circulation, minimize patient discom- fort from additional needlesticks, and are easier for the staff since there is no need for additional equipment or localization of veins. Metabolites, proteins, drugs, and other molecules can be unstable in a patient sample. This sample will have lower glucose results than the patient’s actual levels at the time of collection.

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Each transition program should have an established referral relationship (or directly employ) a clinical psychologist with experience in providing services to adolescents and young adults with chronic disease purchase clomiphene 25 mg visa. All transition programs should have access to a social worker with experience in transition discount clomiphene 50mg without a prescription. These providers should have experience regarding how to smoothly transition patients from their adolescent insurance to an adult program cheap clomiphene 25mg with amex. Moreover order clomiphene 100 mg on-line, they should know what social and governmental resources are available for patients with chronic healthcare needs (4,9,16,48). A similar “transition” social worker should be available to the patient after transfer of care to the adult provider as many of these “financial” transitions do not occur until well into adulthood. Barriers to Transition Even in organized healthcare systems, successful transition and transfer of care occurs less than half the time (49). This relates to the multiple barriers that exist that may belie successful transition. Several of these barriers as well as potential ways to prevent their occurrence will be discussed. The main reasons for this gap in care include the patient feeling well or not knowing that follow-up was required (5). In addition, a history of missed cardiology appointments during childhood were predictive of being lost to follow-up as a young adult (50). Solution In order to decrease the number of patients who are lost to follow-up during the transfer period, it is vital to stress to the patient and his/her family the importance of lifelong follow-up. At transfer, the pediatric provider should provide the patient with medical follow-up that includes a specific location (provider), date, and reason (4). A system should be in place for the pediatric provider to follow-up and ensure that their patient went to their first appointment with the adult cardiac provider (4,51). Problem: Communication Issues Excellent communication lies at the foundation of a successful transition and transfer—and poor communication can result in failure. A recurrent theme in the literature is the desire for better communication, especially during the transfer of care from the pediatric to the adult provider (4,5,7,13,17,25,51,52). Both providers and patients desire more thorough communication during this process. These summaries often lack the rationale for the current treatment plan and leave out key events (including adverse events, reactions or failures to previous treatments) that are vital to patient care. When the adult provider does not know these key historic events, it serves to reinforce the skepticism that many patients already carry into these relationships. Solution To improve communication, it is vital that the pediatric provider provide a thorough, written, transition note to the new adult provider (4,7,16,51,52). This note should be individualized, developed by the patient/parent/provider, and include important information regarding their diagnoses, surgical history, treatment history, and rationale for current treatment plan (8,9). For more complex patients, direct communication via telephone or an in-person discussion may further strengthen the transfer process. In some instances, the pediatric cardiologist may choose to not transfer patients if they do not feel that qualified adult providers are available (7). In addition, pediatric providers should recognize the need for transfer of care and its importance in the patient taking on responsibility for his or her healthcare needs. Problem: Loss of Insurance As mentioned previously, one of the key components to the transition process is to insure continuous insurance (or other financial) coverage (4,25). In general, over 25% of young adults in the United States lack health insurance coverage (54). Solution It is extremely important to maintain insurance coverage without a lapse throughout adolescence and into adulthood. The transition social worker should work with family during the transition process to insure plans are in place to insure continuous insurance coverage. If needed, the process to apply for government health care and/or disability should be started well in advance of these programs being required. The importance of maintaining continuous insurance coverage should be stressed to the patient and their family so this can be taken into account when making any changes or employment-related decisions (4). New guidelines and training pathways hope to provide some consistency to the current, uneven system. The prevalence of adult congenital heart disease, results from a systematic review and evidence based calculation. Congenital heart disease in the general population: changing prevalence and age distribution. Best practices in managing transition to adulthood for adolescents with congenital heart disease: the transition process and medical and psychosocial issues: a scientific statement from the American Heart Association. Transition from child-centered to adult health-care system for adolescents with chronic conditions: a position paper of the Society for Adolescent Medicine. Transition and transfer from pediatric to adult care of young adults with complex congenital heart disease. Developing a transition program from pediatric- to adult-focused cardiology care: practical considerations. Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Care of the adult congenital heart disease patient in the United States: a summary of the current system. Attitude toward and current practice of transfer and transition adolescents with congenital heart disease in the United States of America and Europe. The role of the pediatrician in transitioning children and adolescents with developmental disabilities and chronic illnesses from school to work to college. The emerging burden of hospital admissions of adults with congenital heart disease. American Academy of Pediatrics, American Academy of Family Physicians, American College of Physicians-American Society of Internal Medicine. A consensus statement on health care transitions for young adults with special health care needs. Transition to adult health care for adolescents and young adults with congenital heart disease: perspectives of the patient, parents and health care provider. Supporting development of children with chronic conditions: from compliance, toward shared management. What do adult patients with congenital heart disease know about their disease, treatment, and prevention of complications? A cohort study on psychosocial adjustment and psychopathology in adolescents and young adults with congenital heart disease. Biopsychosocial experiences of adults with congenital heart disease: review of the literature. Adults with congenital heart disease: psychological needs and treatment preferences. Health behaviors among adolescents and young adults with congenital heart disease. Facilitators of and barriers to advance care planning in adult congenital heart disease. Perioperative morbidity and mortality after noncardiac surgery in young adults with congenital or early acquired heart disease: a retrospective cohort analysis of the National Surgical Quality Improvement Program database. Decentralization of care for adults with congenital heart disease in the United States: a geographic analysis of outpatient surgery. Reproductive and contraceptive counseling received by adult women with congenital heart disease: a risk-based analysis. Recreational and occupational recommendations for young patients with heart disease: a statement for physicians by the Committee on Congenital Cardiac Defects of the Council on Cardiovascular Disease in the Young, American Heart Association. Adult congenital heart disease incidence and consultation: a survey of general adult cardiologists. Prevalence and correlates of successful transfer from pediatric to adult health care among a cohort of young adults with congenital heart defects. Risk factors for loss to follow-up among children and young adults with congenital heart disease. Recommendations for organization of care for adults with congenital heart disease and for training in the subspecialty of ‘Grown-up Congenital Heart Disease’ in Europe: a position paper of the Working Group on Grown-up Congenital Heart Disease of the European Society of Cardiology. Survey of primary care pediatricians on the transition and transfer of adolescents to adult health care. Update on the challenges facing the adult with congenital heart disease community: for both the patient and provider. Siu In general, pregnancy is well tolerated in women with congenital heart disease.

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When proximal control has been achieved such that there may be a gradient by blood pressure cuff between arms it would be possible to place a controlling clamp if injury and legs order clomiphene no prescription, perhaps because of lower extremity vasoconstric- occurred at the time of dissection of the descending aorta and tion in the setting of systemic hypothermia buy clomiphene cheap online. The posterior wall is sutured frst working inside the lumen (inverting suture line) followed by an everting external suture line across the anterior wall order online clomiphene. However purchase genuine clomiphene, by the time of hospital dis- Radically Extended End-to-End Anastomosis charge, a residual gradient of greater than 14 mm by cuff sug- One technique for dealing with the hypoplastic aortic arch gests a signifcant risk of need for reintervention. The approach is as for simple anastomo- intracardiac left to right shunt is present, e. In is carried along the proximal aortic arch and up to the dis- the infant and small child, a single chest tube is adequate. The proximal innominate artery is also The incision is closed with interrupted pericostal absorbable defned. A particularly important step in this procedure is suture with careful closure of the muscle layers with absorb- to ensure that there is a method for monitoring perfusion of able continuous suture technique and subcutaneous and sub- the innominate artery during the period that clamps will be cuticular absorbable suture completing wound closure. The coarctation segment has been resected and an aortotomy has been incised across the under- surface of the isthmus, distal aortic arch and proximal aortic arch. In addition, it is useful to have a vessels, as well as the descending aorta, it is possible to bring pulse oximeter on the right hand and right ear. Near infra- the toe of the descending aorta up to the ascending aorta in red spectroscopy is also proving to be helpful. A C-clamp is the same way that a direct anastomosis to the ascending aorta applied which partially occludes the distal ascending aorta is fashioned for type B interrupted aortic arch. The aortotomy must be taken with the toe of the anastomosis to ensure that is extended across the entire surface of the aortic arch into this area is not stenosed since there will be at least moderate Coarctation of the Aorta 299 tension on the anastomosis (Fig. It is opened longitudinally with important to ensure that retraction on the proximal C-clamp the incision being carried along the isthmus of the aorta does not importantly interfere with perfusion of the innomi- and several millimeters beyond the coarctation shelf (Fig. The dependent on fow through the circle of Willis from the right toe of the fap is sutured into the most distal extent of the vertebral artery and the right common carotid artery. Although some authors the left common carotid artery and left subclavian arteries have recommended excision of the coarctation shelf, there must be occluded during the clamp period. Mild hypother- is concern that this increases the risk of subsequent aneu- mia, e. It is usually performed Approach is as for resection and end-to-end anastomo- in conjunction with resection and end-to-end anastomosis. The left subclavian artery is mobilized to In the neonate with a patent ductus arteriosus receiving a the level of the frst rib. In theory, the left vertebral artery prostaglandin infusion, it is possible to perform the sub- should be ligated in order to prevent a subsequent left sub- clavian fap component of the combined procedure without clavian steal phenomenon. This is a good important to avoid ligating multiple branches of the distal example of the way in which correct sequencing of an oper- subclavian artery as this can increase the risk of left limb ation can minimize the stress of the surgery for the patient ischemia. Clamps are applied across the distal aortic arch and described for the antegrade subclavian fap procedure. In proximal descending aorta following ligation of the duc- addition, the aortic arch is dissected free to a point proxi- tus or ligamentum. The aorta is controlled between clamps and the left subclavian artery is retracted. A longitudinal incision is extended along the full length of the left subclavian artery and across the area of coarctation opposite the ductus. The dashed line indicates the incision along the right side of the subclavian artery, the superior surface of the dis- tal aortic arch, and the left side of the origin of the left common carotid artery. This allows continuing perfusion of the lower body through the patent ductus (dashed arrow). The left subclavian artery is turned back in a reverse direction as a fap to complement the hypoplastic distal arch. Following a period of reperfusion, clamps are applied as indicated and the area of coarctation is resected. Coarctation of the Aorta 301 carotid artery is also dissected free over at least 5–6 mm. The isthmus is controlled with a Synthetic Patch Aortoplasty straight or slightly angled neonatal DeBakey clamp. This Although this approach was popular in the 1970s and was par- allows continuing fow through the ductus to perfuse the ticularly championed at that time by Ebert and Mavroudis,38 lower body in the neonate with a patent ductus (Fig. The subclavian artery is opened longitudinally along under certain circumstances of unusual anatomy, for exam- its rightward aspect with the incision extended across the ple a very long tubular narrowing of the aorta, perhaps in superior surface of the distal aortic arch and then distally the setting of recurrent coarctation, a choice must be made along the left common carotid artery for approximately between this procedure and interposition of a tube graft. The fap is In the growing child, it is probably preferable to perform a turned back retrograde toward the left common carotid synthetic patch aortoplasty rather than placing a nongrow- artery with the toe being sutured into the common carotid ing tube graft. After mobilization of the aorta proximal and across the incision in the distal aortic arch, thereby supple- distal to the coarctation area, clamps are applied above and menting the circumference of the distal arch. A longitudinal incision is made on the anterior and continuous 6/0 prolene or an absorbable 6/0 suture, such leftward face of the aorta across the coarctation area (Fig. Once again there is some controversy as to whether clamps and having secured hemostasis, attention can now the coarctation shelf should be resected since it is believed be directed to the coarctation area itself. If a Gore-Tex patch is employed, it is gen- cedure has the advantage that there is less tension on the erally wise to use a Gore-Tex suture since bleeding through anastomosis relative to an extended end-to-end anastomosis needle holes at aortic pressure can be persistent. The cross-clamp time during which descending aor- which is judged unlikely to close spontaneously. One approach is to use a left thoracotomy incision and to place a pulmonary artery band at the time of coarctation repair. The distal extended hospitalization, the risks of two operations rather subclavian artery is controlled with a small bulldog clamp or than one, the expense of two operations rather than one, addi- with a fne tourniquet during the cross-clamp period. The left tional psychological stress for the family, and the cosmetic subclavian artery is divided at its origin from the aorta. Immediately after commencing bypass, because there is a risk that clamp manipulation may obstruct the ductus arteriosus is suture ligated. During cooling, the fow in the innominate artery although bypass fow is con- arch vessels are thoroughly mobilized, as well as the proxi- tinued. Considerable care is taken to preserve cooling to moderate hypothermia such as 25°C will still pro- the left recurrent laryngeal and vagus nerves, as well as vide adequate protection of both the spinal cord and brain. A fne neonatal vascular clamp is placed Alternatively, if there is extensive hypoplasia of the aortic across the proximal aortic arch and a C-clamp is placed on arch, either a radical extended end-to-end anastomosis can the descending aorta. The left common carotid and left sub- be fashioned or a patch plasty can be performed using glutar- clavian arteries are controlled with fne tourniquets. These procedures bypass continuing, the coarctation area can be excised and will necessitate the use of a period of hypothermic circula- an extended end-to-end anastomosis performed. In small baby, it is probably wise to cool to deep hypothermia this case, the patient should be cooled to deep hypothermia. A patch of crimped Dacron has been sutured into the aortotomy using a continuous suture technique. Usually fow continues through all but the smallest babies so that partial rewarming can be the right subclavian and vertebral arteries, as well as the right undertaken following the arch repair. An aberrant right subclavian artery (which arises from the proximal descending aorta), however, is included within the clamped segment thereby increasing the risk of compromise CompliCatioNs oF CoarCtatioN surgery of blood fow to the anterior spinal artery. Brewer’s article aNd how to miNimize them emphasizes the rare anatomical variant where the anterior spinal artery is discontinuous as a cause of spinal cord isch- Early Complications emia. In this setting, if multiple intercostal vessels and most Paraplegia particularly the artery of Adamkiewicz are occluded dur- By far the most devastating complication reported with ing the cross-clamp period, then the risk of paraplegia will coarctation surgery is paraplegia. Accordingly, it is important to preserve fow a survey of 12,000 cases of coarctation in 1972 and found a through as many collateral vessels as possible and to avoid 0. In the current era, however, the risk of paraplegia One factor which is not discussed in Brewer’s article is is almost certainly much less. It is a common practice in the pedi- of paraplegia is less when surgery is undertaken at a younger atric operating room to maintain the patient’s temperature age when there is less likely to be major hemorrhage second- aggressively with heating lamps and warming blankets. This ary to injury of the fragile collateral vessels which develop almost certainly increases the risk of neuronal injury in the with age. It is our practice to use a mild degree of systemic necessitates a very much shorter suture line and therefore hypothermia to 34°C or 35°C by use of a cooling blanket quicker cross-clamp time.

Advantages of this technique include an autogenous floor with a pericardial roof that does not form obstructive peels buy 25mg clomiphene visa, and the diameter of the pathway can be as large as desired buy cheap clomiphene 100 mg online, allowing a large bioprosthetic valve to be inserted clomiphene 50 mg on-line. We have examined the freedom from reoperation for conduit failure in an age-matched group of patients who have received a Hancock conduit discount 25mg clomiphene with visa, a homograft conduit, and a valved peel reconstruction (Fig. The peel operation had statistically significant better freedom from reoperation compared with the homograft ( p = 0. Although the peel operation had more favorable durability than the Hancock conduit, this did not reach statistical significance ( p = 0. Percutaneous pulmonary valve therapy is now an alternative and complementary therapy for the postoperative truncus patient with a failing conduit (87,88,89,90). Although the need for reoperation is inevitable for most patients, the risk of reoperation is low and most patients enjoy a good quality of life. Presently, the peel operation provides the most favorable freedom from reoperation and is our procedure of choice when conduit replacement is required. Late results of the peel operation for replacement of failing extracardiac conduits. Truncus arteriosus is considered to be a complex form of congenital heart disease so that care of these patients in an experienced regional center is preferred (91). The primary issues for these patients include long-term function of the right ventricular-to-pulmonary artery conduit and of the truncal (aortic) valve, the potential for branch pulmonary artery abnormalities, development of significant tricuspid regurgitation, and aortic root dilation. In addition, genetic testing should be considered in patients who have not had evaluation for 22q11 microdeletion syndrome (92). Echocardiography is recommended to assess the function of the right ventricle-to-pulmonary artery conduit and valve, paying close attention to stenosis and/or regurgitation. A mean gradient of over 35 mm Hg or a peak gradient of over 50 mm Hg in the pulmonary conduit generally is considered to be indicative of significant stenosis, as is a right ventricular systolic pressure P. There may be evidence of decreased right ventricular function and/or an increase in the degree of tricuspid regurgitation concurrent with a deterioration of conduit valve function. One should assess the degree of truncal or aortic valve insufficiency and consider replacing this valve if significantly incompetent. Cardiac catheterization generally is reserved for assessment of abnormalities detected by noninvasive imaging techniques and may involve additional interventions, such as balloon angioplasty of pulmonary branch stenosis or implantation of endovascular stents in the branch pulmonary arteries. Percutaneous implantation of a bioprosthetic pulmonary valve is now available for appropriate patients (88,89,94). Any surgical intervention should be carried out in an institution that specializes in the care of adults with congenital heart disease and performed by a surgeon trained in congenital heart disease (91). Long-Term Issues In summary, patients with repaired truncus arteriosus will need lifelong cardiovascular follow-up. Primary issues that will require attention, ongoing evaluation, and potentially further treatment after neonatal repair include truncal valve dysfunction (stenosis and/or insufficiency), function of the pulmonary homograft/conduit in the right ventricular outflow tract, and the development of branch pulmonary artery stenosis. Left and right ventricular function, both systolic and diastolic, must be evaluated in an ongoing fashion. Seamless transition from the pediatric cardiologist to the adult congenital heart disease specialist is clearly warranted as this patient group ages. Cardiovascular anomalies in DiGeorge syndrome and importance of neural crest as a possible pathogenetic factor. DiGeorge anomaly associated with a de novo Y; 22 translocation resulting in monosomy del(22)(q11. Cardiac malformations in relatives of children with truncus arteriosus or interruption of the aortic arch. Familial recurrence of nonsyndromic interrupted aortic arch and truncus arteriosus with atrioventricular canal. Truncus arteriosus: ten-year experience with homograft repair in neonates and infants. Pathogenesis of persistent truncus arteriosus in light of observations made in a dog embryo with the anomaly. The anatomy of common aorticopulmonary trunk (truncus arteriosus communis) and its embryologic implications: a study of 57 necropsy cases. Truncus arteriosus communis: truncal valve anomalies associated with small conal or truncal septal defects. Selection of patients with truncus arteriosus for surgical correction: anatomic and hemodynamic considerations. Congenital heart disease among 160,480 liveborn children in Liverpool 1960 to 1969. Fatal pulmonary artery banding in truncus arteriosus with anomalous origin of circumflex coronary artery from right pulmonary artery. Anatomical study of truncus arteriosus communis with embryological and surgical considerations. Does the dilated ascending aorta in an adult with congenital heart disease require intervention? Accuracy of prenatal echocardiographic diagnosis and prognosis of fetuses with conotruncal anomalies. Echocardiographic diagnosis alone for the complete repair of major congenital heart defects. Truncus arteriosus with unilateral absence of pulmonary artery: criteria for operability and surgical results. Truncus arteriosus with single pulmonary artery: influence of pulmonary vascular obstructive disease on early and late operative results. Morphologic and functional evaluation of congenital heart disease by magnetic resonance imaging. Truncus arteriosus and previous pulmonary arterial banding: clinical and hemodynamic assessment. Homograft of ascending aorta and aortic valve as a right ventricular outflow: an experimental approach to the repair of truncus arteriosus. Repair of the truncal valve and associated interrupted arch in neonates with truncus techniques. Repair of conotruncal abnormalities with the use of the valved conduit: improved early and midterm results with the cryopreserved homograft. Long-term follow-up of truncus arteriosus repaired in infancy: a twenty-year experience. Truncus arteriosus with interrupted aortic arch: Successful correction in a neonate. Late follow-up of 1095 patients undergoing operation for complex congenital heart disease utilizing pulmonary ventricle to pulmonary artery conduits. Performance of right ventricle to pulmonary artery conduits after repair of truncus arteriosus: a comparison of Dacron-housed porcine valves and cryopreserved allografts. Right ventricular outflow reconstruction with cryopreserved homografts in pediatric patients: intermediate term follow-up with serial echocardiographic assessment. Performance of allografts and xenografts for right ventricular outflow tract reconstruction. Late results of the peel operation for replacement of failing extracardiac conduits. Improving early and intermediate results of truncus arteriosus repair: a new technique of truncal valve repair. Surgical management of severe truncal insufficiency: experience with truncal valve remodeling techniques. Outcomes of repair of common arterial trunk with truncal valve surgery: a review of the society of thoracic surgeons congenital heart surgery database. Pathogenesis of nonobstructive fibrous peels in right-sided porcine-valved extracardiac conduits. Late results of reconstruction of the right ventricular outflow tract with porcine xenografts in children. Surgical pathology of obstructed, right- sided, porcine-valved extracardiac conduits. Evaluation of long-term results of homograft and heterograft valves in extracardiac conduits. Allograft implantation in pediatric cardiac surgery: surgical experience from 1982 to 1994. Repair of truncus arteriosus: a considered approach to right ventricular outflow tract reconstruction.

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