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Pre- and posttransplantation allosensitization in heart allograft recipients: major impact of de novo alloantibody production on allograft survival buy kamagra oral jelly master card losartan causes erectile dysfunction. Trends and outcomes in transplantation for complex congenital heart disease: 1984 to 2004 buy kamagra oral jelly with american express diabetic erectile dysfunction pump. Cardiac transplantation in pediatric patients: fifteen-year experience of a single center cheap kamagra oral jelly line erectile dysfunction vitamin. Heart transplantation to a physiologic single lung in patients with congenital heart disease. Evidence of pulmonary vascular disease after heart transplantation for Fontan circulation failure. The International Society of Heart and Lung Transplantation Guidelines for the care of heart transplant recipients. Perioperative management in pediatric heart transplantation from 1988 to 2001: anesthetic experience in a single center. Renal insufficiency and end-stage renal disease in the heart transplant population. Donor-recipient size matching in pediatric heart transplantation: a word of caution about small grafts. Sinus node dysfunction after orthotopic cardiac transplantation: postoperative incidence and long-term implications. Anti-T-cell-antibody prophylaxis in children: success with a novel combination strategy of mycophenolate mofetil and antithymocyte serum. Infection and malignancy after pediatric heart transplantation: the role of induction therapy. Calcineurin inhibitor minimization using sirolimus leads to improved renal function in pediatric heart transplant recipients. Empiric switch from calcineurin inhibitor to sirolimus-based immunosuppression in pediatric heart transplantation recipients. Decline in rejection in the first year after pediatric cardiac transplantation: a multi-institutional study. The current state of, and future prospects for, cardiac transplantation in children. Infection after pediatric heart transplantation: results of a multiinstitutional study. Trends in invasive disease due to Candida species following heart and lung transplantation. Noninvasive markers for acute heart transplant rejection in children with the use of automatic border detection. Prospective evaluation of echocardiography for primary rejection surveillance after infant heart transplantation: comparison with endomyocardial biopsy. Diastolic performance assessed by tissue Doppler after pediatric heart transplantation. The yield of surveillance endomyocardial biopsies as a screen for cellular rejection in pediatric heart transplant patients. Evaluation of a noninvasive index of global ventricular function to predict rejection after pediatric cardiac transplantation. Risk factors for recurrent rejection in pediatric heart transplantation: a multicenter experience. Endomyocardial biopsy in pediatric heart transplant recipients: a useful exercise? Is biopsy-proven cellular rejection an important clinical consideration in heart transplantation? Identifying cardiac transplant rejection in children: diagnostic utility of echocardiography, right heart catheterization and endomyocardial biopsy data. An improved echocardiographic rejection-surveillance strategy following pediatric heart transplantation. Value of plasma B- type natriuretic peptide as a marker for rejection in pediatric heart transplant recipients. B-type natriuretic peptide is a sensitive screening test for acute rejection in pediatric heart transplant patients. The International Society for Heart and Lung Transplantation grading system for heart transplant biopsy specimens: clarification and commentary. Association of viral genome with graft loss in children after cardiac transplantation. Viral endomyocardial infection is an independent predictor and potentially treatable risk factor for graft loss and coronary vasculopathy in pediatric cardiac transplant recipients. The 2013 International Society for Heart and Lung Transplantation Working Formulation for the standardization of nomenclature in the pathologic diagnosis of antibody-mediated rejection in heart transplantation. Controversies in defining cardiac antibody-mediated rejection: need for updated criteria. A clinical correlation study of severity of antibody-mediated rejection and cardiovascular mortality in heart transplantation. National conference to assess antibody-mediated rejection in solid organ transplantation. The role of proteasome inhibition with bortezomib in the treatment of antibody-mediated rejection after kidney-only or kidney-combined organ transplantation. Rapid reduction in donor-specific anti-human leukocyte antigen antibodies and reversal of antibody-mediated rejection with bortezomib in pediatric heart transplant patients. The pharmacokinetics of a microemulsion formulation of cyclosporine in primary renal allograft recipients. Randomized clinical trial of tacrolimus- vs cyclosporine-based immunosuppression in pediatric heart transplantation: preliminary results at 15-month follow-up. Sirolimus in de novo heart transplant recipients reduces acute rejection and prevents coronary artery disease at 2 years: a randomized clinical trial. Sirolimus immunosuppression in pediatric heart transplant recipients: a single-center experience. Sirolimus as primary immunosuppression attenuates allograft vasculopathy with improved late survival and decreased cardiac events after cardiac transplantation. Everolimus initiation and early calcineurin inhibitor withdrawal in heart transplant recipients: a randomized trial. Indications, tolerance and complications of a sirolimus and calcineurin inhibitor immunosuppression regimen: intermediate experience in pediatric heart transplantation recipients. Sirolimus as primary immunosuppressant reduces left ventricular mass and improves diastolic function of the cardiac allograft. Recommendations for the use of everolimus (Certican) in heart transplantation: results from the second German-Austrian Certican Consensus Conference. Prospective study of everolimus with calcineurin inhibitor-free immunosuppression in maintenance heart transplant patients: results at 2 years. A randomized active-controlled trial of mycophenolate mofetil in heart transplant recipients. Three-year results of a randomized, double-blind, controlled trial of mycophenolate mofetil versus azathioprine in cardiac transplant recipients. Methotrexate or total lymphoid radiation for treatment of persistent or recurrent allograft cellular rejection: a comparative study.
Increasing the pitch purchase cheap kamagra oral jelly line erectile dysfunction recovery time, and reducing the rotation time can offset the increased scan time and radiation exposure associated with the large volume of coverage buy cheap kamagra oral jelly line encore vacuum pump erectile dysfunction. Coronal minimum intensity projection images during inspiration (B) and expiration (C) demonstrate moderately severe long segment narrowing of distal trachea (yellow arrow) and right and left main- stem bronchi (blue arrows) in inspiration (B) and near complete collapse of the distal trachea (yellow arrow) and left main-stem bronchus (white arrow) on expiration purchase 100 mg kamagra oral jelly with amex erectile dysfunction treatment doctors in bangalore, indicative of severe tracheal stenosis and tracheobronchomalacia. Temporal Resolution A high temporal resolution is needed to freeze cardiac motion and avoid artifacts, especially with 64-detector scanners. With single sector reconstruction, only data from the prescribed time range during one cardiac cycle are used for partial scan reconstruction of images. Multisector reconstruction (18) (segmented reconstruction) can increase the temporal resolution by using scanned data from more than one heart cycle for image reconstruction. The more sectors that are used, the higher the overlap during data acquisition has to be. Multisector reconstruction yields the shortest effective scan time, but also needs the highest radiation dose to accomplish this goal. Temporal padding overcomes this limitation by increasing acquisition time before and after the predicted acquisition window. This technique permits retrospective modification of the reconstruction window to ensure image reconstruction within low motion and identical cardiac phases from one cardiac cycle to another. The acquisition of temporally uniform volumetric data sets with 320-detector scanner enables retrospective half scan reconstruction within different cardiac phases in raw data. This post-acquisition data processing technique, called Target Mode, finds and reconstructs the best motion-free cardiac phase in raw data without increasing the radiation dose to the patient (1). This boosts the radiation dose three to four times compared to a nongated examination. The scans are performed with breath holding and suspended inspiration in cooperative patients. Scans are obtained during quiet respiration in children who are unable to cooperate with breath holding instructions. With the advent of 16-slice scanners, with temporal resolutions of about 50 msec, patients with heart rates of up to 120 bpm can be scanned without premedication. When contraindications have been ruled out, beta-blockers are generally recommended for patients with a heart rate above 110 bpm, when 64-detector or lesser generation scanners are used. In spite of this, sedation is required for evaluation of infants and children who are 5 years of age or younger to prevent gross motion artifacts during scanning. The benefit of scanning without sedation must always be evaluated against the risk of having to repeat the scan due to motion artifact. Many intensive care patients are relatively immobile, and can be scanned without sedation. Children older than 5 years can generally cooperate after vocal reassurance and explanation of the procedure and will not need sedation. With these scanners, sedation is restricted to young patients who are combative and unsafe to restrain, or to indications such as coronary assessment. Since osmolality is a measure of the number of dissolved particles (including ions, molecules or aggregates) per liter of water, ionic agents are considered as high osmolar contrast media, and have five to eight times the osmolality of human serum. Examples of ionic media include sodium and/or meglumine diatrizoate and iothalamate. The nonionic contrast media are considered as low osmolar contrast agents, and have one to two times the osmolality of human serum. Examples of nonionic contrast media include iohexol, iopamidol, ioversol, and iodixanol, and are currently the only type of contrast agents used in children, as they are safer and better tolerated than high osmolar contrast media. Contrast Reaction Patients receiving intravascular contrast media can have a wide variety of reactions including nausea, vomiting, urticaria, bronchospasm, laryngeal edema, vasovagal reactions, hypotension, seizures, and exacerbation of myocardial ischemia or congestive heart failure. Adverse contrast reactions may be classified as idiosyncratic or nonidiosyncratic. Idiosyncratic reactions are dose-independent systemic reactions, which produce signs and symptoms that mimic true anaphylaxis. While their etiology remains unknown, the currently favored mechanism is activation of the complement system. Nonidiosyncratic reactions are thought to result from direct chemotaxic or hyperosmolar effects of contrast material. Symptoms include nausea, vomiting, cardiac arrhythmias, renal failure, pulmonary edema, and cardiovascular collapse (22,23). Previous reaction to contrast media is the most important risk factor in prediction of reaction. Premedication should be considered in a patient with previous allergic-like reaction to iodinated contrast material; multiple (usually four or more) allergies or a severe allergy to another substance; or asthma with frequent, recent, or severe attacks. Premedication for prevention of idiosyncratic reactions usually consists of prednisone 50 mg administered 13 hours, 7 hours, and 1 hour before, and diphenhydramine administered 1 hour before contrast media infusion, along with the use of low osmolar contrast media (24). Usually, the rise in serum creatinine is seen within 1 to 2 days after contrast injection. The current evidence suggests that low osmolar contrast media reduce, but do not eliminate the incidence of contrast-induced renal failure. Hence, an alternative diagnostic modality that does not involve the use of systemic contrast media is recommended in high-risk patients. When contrast media injection is essential for a diagnostic workup, a reduced volume of nonionic contrast may be utilized. Intravenous Contrast Injection The usual dose of intravenous iodinated contrast media is 2 mL/kg with an iodine concentration of 240 to 370 mg/mL. The greater the iodine concentration, the better the vascular enhancement, but this also increases the contrast viscosity (30,31). In general, the more rapid the rate of contrast injection, the greater will be the level of vascular enhancement. The degree of enhancement is inversely related to body weight, which is an advantage when imaging small infants. Contrast injection rate is limited by the size and location of the peripheral intravenous line. The antecubital location is the preferred location in upper extremity due to larger vein size. Rates of 2 to 3 mL/s, which are optimal for infants and young children, require the use of a power injector and at least a 22-gauge peripheral angiocatheter. Rates of 3 to 5 mL/s require a 20-gauge catheter or a closed system fenestrated catheter (32,33,34). Rates of 5 mL/s are usually used for coronary artery evaluation or to detect pulmonary emboli in adult-sized patients (35). Contrast Injection Protocols While high enhancement is required for evaluation of coronary arteries, pulmonary vessels, aortopulmonary collaterals and stent patency, a lower and more constant enhancement level through the heart is required for evaluation of cardiac morphology. Monophasic contrast material injection protocols fulfill the former need, while biphasic injection protocols are more suited to the latter indication (38). Monophasic contrast material injection is not ideally suitable for evaluating cardiac morphology because it leads to a gradual increase in intravascular contrast with time, until a maximum is reached and enhancement rapidly decreases thereafter. This effectively occurs at different points in time for the right and left side of the heart and will therefore make homogeneous enhancement of the heart chambers difficult.
In his classic 1970 study order kamagra oral jelly us erectile dysfunction treatment can herbal remedies help, Campbell reported natural history data obtained from necropsy and clinical records on 465 patients with coarctation (40) 100 mg kamagra oral jelly amex impotence unani treatment in india. Only subjects who survived the first year of life were evaluated generic kamagra oral jelly 100mg with mastercard erectile dysfunction 19 year old male, excluding infants with critical coarctation. The most common causes of death were congestive heart failure (26%), aortic rupture (21%), endocarditis (18%), and intracranial hemorrhage (12%). Given such a poor prognosis untreated, it is apparent that intervention is indicated in virtually all patients with coarctation of the aorta. Presentation in Infancy Coarctation presenting with heart failure in infancy requires immediate treatment. Medical management consists of initially stabilizing the patient with inotropic support. A critically ill newborn also may benefit from prostaglandin E1 to promote ductal patency and improve perfusion of the descending aorta, renal, and mesenteric beds (41). Metabolic disturbances such as acidosis, hypothermia, hypoglycemia, or anemia must be treated promptly. After a brief period of medical management to stabilize the child, definitive repair should be performed (42). In experienced centers, the mortality rate for surgical repair of isolated coarctation in infancy is very low (43,44,45,46,47,48,49,50,51). The surgical risks are higher for infants with coarctation who have associated major intracardiac anomalies. Nevertheless, after a period of medical stabilization, early coarctation repair is indicated in these children. The surgical mortality rate for these infants ranges from 2% to 10% and is highest for children with the most complex intracardiac defects (43,44,45,46,48,51). The need for intracardiac repair (or pulmonary artery banding) at the time of coarctation repair is not always clear. In some circumstances, coarctation repair alone is sufficient and may improve the pathophysiology of the associated lesion. For example, following repair of coarctation an infant with a ventricular septal defect may demonstrate a diminished left-to-right shunt and resolution of heart failure. Subsequently, some will experience spontaneous diminution in size of the ventricular septal defect sufficient to avoid future intracardiac surgery. Modest hypoplasia of the aortic arch, aortic or mitral valves may improve in follow-up after repair of coarctation in the newborn (52). Intracardiac repair at the time of coarctation repair is appropriate in infants with a large ventricular septal defect, or more complex lesions such as d-transposition or double-outlet right ventricle (53,54). Presentation in Childhood Coarctation more commonly presents in childhood or adolescence as upper extremity hypertension and/or a heart murmur without overt symptoms. Coarctation repair is commonly recommended at 6 months to 2 years of age in asymptomatic children without severe upper-extremity hypertension. First, the risk for late recurrence of coarctation appears to be increased when repair is performed on young infants (43,44,45,55,56,57,58,59). The influence of age at repair on restenosis is explained in part by the smaller diameter of the surgical anastomosis when repair is performed in younger children (60). Second, even in the absence of residual stenosis, there is an increased risk for persistent hypertension and early atherosclerotic cardiovascular disease if coarctation repair is delayed into late childhood and adolescence (61). In a long-term follow-up study of 234 patients, the prevalence of late hypertension was 6% in patients who underwent coarctation repair between 1 and 5 years of age compared with 30% to 50% in patients whose coarctation was repaired at an older age (62). An informative, retrospective follow-up study (58) used multivariate analysis to assess influence of age at operation on composite outcomes of survival, residual hypertension, and recurrent stenosis. The study concluded that these outcomes are optimized if elective coarctation repair is performed at approximately 1. Surgical Repair Several surgical techniques have been used to repair aortic coarctation, and each has advantages and disadvantages. Surgical approaches to coarctation include resection and end-to-end anastomosis, subclavian flap aortoplasty, prosthetic patch aortoplasty, and bypass grafts between the ascending and descending aorta. Surgical repair of isolated coarctation generally is performed through a left lateral thoracotomy incision. If necessary, as when combined with repair of an intracardiac lesion, coarctation repair can be performed from an anterior approach. Regardless of the surgical technique used, most children with a discrete coarctation will have a residual resting systolic pressure gradient under 10 mm Hg immediately after repair. The mortality rate for surgical coarctation repair varies depending on patient age and associated lesions. The surgical mortality for repair of isolated coarctation in infants and older children currently approaches 0% (48,49,59), rises to 2% to 10% for infants with an associated large ventricular septal defect, and is higher in the presence of more complex intracardiac lesions (51). Surgical morbidity includes postoperative paradoxical hypertension, spinal cord ischemia and paralysis, recurrent laryngeal or phrenic nerve injury, chylothorax, bleeding, and infection. Paradoxical hypertension (the postcoarctectomy syndrome) may occur during the first 2 to 5 days following coarctation repair, with systolic and diastolic pressures rising above pretreatment levels (63). The mechanism is related to rebound activation of the sympathetic nervous system and the renin–angiotensin system which leads to mesenteric arterial vasoconstriction. Postoperative paradoxical hypertension can be prevented with beta-blocker therapy (64) and by aggressive antihypertensive therapy during the immediate postoperative period. Spinal cord injury and subsequent paralysis may occur if aortic cross-clamping severely compromises perfusion to the descending aorta and spinal arteries. It is avoided by ensuring adequate descending aorta perfusion when the aorta is cross-clamped, limiting total cross-clamp time to under 30 minutes, minimizing the number of intercostal arteries sacrificed, avoiding hyperthermia, and using hypothermia if necessary (65). Left heart bypass may be necessary in some patients to maintain adequate descending aorta perfusion when the aorta is cross-clamped. Surgical repair of coarctation of the aorta was first reported in 1945 by Crafoord and Nylin (66), who described the technique of resection and end-to-end anastomosis (Fig. In most centers, this procedure remains the surgical treatment of choice for patients with a discrete coarctation. An extended end-to-end anastomosis using a broader longitudinal incision across the proximal aorta (Fig. The advantages of resection include removal of the coarcted segment and adjacent areas of ductal tissue, avoidance of prosthetic materials, and sparing the left subclavian artery in most instances. Disadvantages of resection relate primarily to the presence of a circumferential suture line, which led to a high incidence of restenosis in early studies. The use of interrupted and absorbable sutures anteriorly and an extended anastomosis in patients with isthmus or arch hypoplasia have improved clinical outcomes after resection in more recent studies (48,49,53,59,67). For infants with arch hypoplasia a technique referred to as aortic arch advancement, similar to that used in interrupted aortic arch repairs, also has led to improved outcomes in some centers (68). Prosthetic patch aortoplasty was the second surgical technique described for coarctation repair, by Vosschulte (69) in 1961. A longitudinal incision is made across the coarctation and the area is enlarged with a patch of prosthetic material such as Dacron or Gore-Tex (Fig.
Immediately after commencing all patients with the aim to preserve as much myocardium bypass order genuine kamagra oral jelly on-line erectile dysfunction at age 64, the tourniquets that have already been placed around as possible 100mg kamagra oral jelly with mastercard erectile dysfunction pump australia. It is possible that an extremely small subset of the right and left pulmonary arteries should be tightened patients with profoundly depressed ventricular function and (Fig 100mg kamagra oral jelly amex erectile dysfunction pills in india. This very important step was frst described relatively recently and serves several important functions. With early diag- First, there is always some collateral connection between the nosis and application of the techniques described below this two coronary systems. In addition, blood passing into the left coronary system and pulmonary artery will pass through the pulmonary veins History of Surgery into the left atrium and left ventricle. Distention of the heart must be assiduously avoided early palliative operations was to increase pulmonary artery during the procedure. A vent should be inserted through the pressure and thereby increase coronary perfusion pressure. If there is any diffculty achieving this an an aortopulmonary window, were attempted. Unfortunately, there was a ten- visualizing the external course of the anomalous coronary. Cardioplegia solution is infused Anomalies of the Coronary Arteries 667 into the root of the aorta only. Although some have recom- wall fap, are sutured together using continuous 7/0 Prolene mended simultaneous infusion of cardioplegia solution into (Fig. It should not be necessary to mobilize any more the pulmonary artery root, we have observed satisfactory than 2–3 mm of the actual left main coronary artery, which blanching of the left ventricle using aortic delivery of car- is frequently surrounded by enlarged venous and arterial col- dioplegia only. In fact, the pulmonary artery root is soon seen lateral vessels that can be an important source of bleeding if to fll with clear cardioplegia solution and equilibrate with they are divided as part of the mobilization. The donor site in aortic root pressure, the latter phenomenon also having been the anterior wall of the ascending aorta is reconstructed with observed during the cooling phase on bypass. If Pulmonary Artery Reconstruction adequate visualization is not achieved at any time during the In the neonate it is generally possible to reconstruct the procedure because of collateral return, deep hypothermic divided main pulmonary artery by direct reanastomosis. This necessitates double suture ligation and division of the ductus as well as mobilization of the branch pulmonary Coronary Reimplantation arteries as for an arterial switch procedure. A direct anasto- The surgeon should have carefully studied the preopera- mosis is fashioned using continuous 6/0 Prolene (Fig. If the ostium is close to the aortic side of the pulmo- sionally, it is advisable to use a very short tube of homograft nary artery it may be possible to simply rotate the button and of appropriate diameter (e. However, the ostium often lies toward the Careful venting of the left heart throughout the rewarming leftward edge of the main pulmonary artery and is therefore period is just as important as during the cooling phase. When a considerable distance, that is, the width of the main pul- cardiac action is re-established, generally coincident with monary artery from the ascending aorta. The ostium is also the administration of calcium, venting may be cautiously not uncommonly below the tops of the commissures of the discontinued as left atrial pressure is carefully monitored. Therefore, faps of aortic and pulmonary Appropriate inotropic support, generally with dopamine, artery wall should be developed in order to allow a tension- should already be established. If the left ventricle is unable free anastomosis to the aorta using a coronary extension to handle the left heart return on bypass, as indicated by a developed from the aortic and pulmonary artery faps (Fig. The reconstructed left main coronary artery will run re-established, and arrangements should be made to assist posterior to the reconstructed main pulmonary artery. Likewise, if weaning from bypass is only initial transverse incision anteriorly in the main pulmonary possible with very high levels of inotropic support and high artery is made a millimeter or two above the anticipated level left atrial pressure, left ventricular assistance should be seri- of the anomalous ostium. However, tial that a left ventricular vent be connected into the venous for the reimplantation procedure a button is excised. If the drainage system to ensure that the left heart remains decom- ostium lies below the top of the leftward commissure of the pressed. The vent can be placed directly into the left heart pulmonary valve it may be necessary to detach this commis- from the right pulmonary veins or across the atrial septum sure and subsequently to reattach it to reconstruct the pulmo- from the right atrium. It is generally best to divide the main pulmonary ventricular function can be observed within 48–72 hours, artery completely at the level that the fap has been developed at which time the left heart vent can be clamped and then in order to minimize distortion of the pulmonary artery fol- removed. A second fap of aortic wall based on the leftward this should be possible within 5–6 days at most. It is important The technique of repair of the anomalous left coronary artery that the initial transverse incision be the higher of the two by reimplantation in the aorta was introduced by Neches et so that the tops of the commissures of the aortic valve are al. The two faps, that is the innovative methods for achieving aortic implantation of the anterior pulmonary artery wall fap and the posterior aortic anomalous left coronary artery in 11 patients. There were no 668 Comprehensive Surgical Management of Congenital Heart Disease, Second Edition operative or late deaths. Follow-up for a mean period of 46 true coronary circulation, almost all patients recovered to a months revealed no new angina or infarction, improved ven- normal end-diastolic volume reaching near normal values tricular function and decreased mitral regurgitation. In a follow-up report from in the reconstructed coronary systems was demonstrated by Children’s Hospital Boston, Schwartz et al. The authors as noted in the title of their article – that “aortic implantation concluded that the degree of preoperative mitral regurgita- is possible in all cases of anomalous origin of the left coro- tion was predictive of outcome whereas the severity of pre- nary artery to the pulmonary artery. Hospital survival was had direct aortic implantation with a 13% hospital mortal- 92%. It is cant improvement in mean ejection fraction from 33% preop- important to note that the median age at surgery in this series eratively to 64% postoperatively. Left ven- before corrective surgery, at long-term follow-up scar tissue tricular shortening Z score was not normal at discharge but 31 is relatively scarce. Others reports have also documented excellent results, Use of Ventricular Assist Device including Backer et al. Seven patients were placed on mechanical required repair or replacement a mean of 3. There were no deaths among 12 patients operated on at a Associated Anomalies median age of 3. In patients who were able to achieve a increase pulmonary artery saturation are likely to be asso- Anomalies of the Coronary Arteries 669 ciated with an improved natural history relative to patients while 21 had associated lesions, including coronary artery with normal pulmonary artery pressure and saturation. This rare variation requires par- Coronary artery fstulas are an important component of ticularly careful preoperative diagnosis and surgical manage- pulmonary atresia with intact ventricular septum, in which ment. At Children’s National Medical Center we encountered they can be associated with proximal coronary artery steno- three such patients in a 6-month period. In this setting, the fstulas may provide the only blood had associated tetralogy of Fallot with minimal outfow tract supply (derived from the hypertensive right ventricle) to a obstruction so that there were minimal signs of left ventricu- signifcant portion of the left ventricle. Intraoperative diagnosis was suggested that a similar situation may be present in children particularly challenging because by direct inspection the left with mitral stenosis and aortic atresia as part of the hypoplas- main coronary artery appeared to run in the usual location tic left heart syndrome. Surgical transfer from the right the signifcance of associated coronary artery fstulas, are pulmonary artery to the aorta was also compromised by the discussed in greater detail in other chapters. We also encountered this The regenerative capacity of immature myocardium by anomaly in several patients, always in the setting of severe hyperplasia in the frst months of life mandates early diag- hypoplasia of the true pulmonary arteries and dependency on nosis and establishment of a dual coronary system for multiple aortopulmonary collaterals. Coronary artery translocation with aortic implan- tation is today the procedure of choice. Patients seen after the age of 25 years are almost always symptomatic with angina, congestive Isolated congenital coronary fstulas are exceedingly rare.