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The P2 component of the second heart sound becomes louder over time and may become palpable buy motilium 10mg line gastritis magnesium. An S1 coincident holosystolic blowing murmur at the left lower sternal border may indicate tricuspid regurgitation and a diastolic decrescendo murmur may indicate pulmonary regurgitation purchase motilium 10 mg with visa gastritis upper gi, both resulting from elevated pulmonary vascular resistance order motilium 10mg fast delivery gastritis dogs. A third heart sound of right ventricular origin may be present at the left lower sternal border buy motilium mastercard gastritis diet vegetarian. The intensity of the murmur is almost always related to the size of the defect: the smaller the defect, the larger the pressure gradient across the defect and the louder and higher frequency the murmur. Infants and children with small defects are essentially asymptomatic because the left-to-right shunt is minimal. Physical examination is also generally unremarkable, with no evidence of failure to thrive, normal heart rate and respiratory rate, and no feeding difficulties. Cardiac examination reveals a normally active precordium with a normal point of maximal impulse. With a high-pressure gradient, a thrill may be palpable along the left sternal border. The first heart sound is normal but often difficult to hear because of the heart murmur. The murmur is typically high frequency and accentuated when auscultation is performed with the diaphragm of the stethoscope. A diastolic rumble is uncommon because there is normal flow across the mitral valve. The second heart sound is normal because the pulmonary artery pressure is not elevated. The first heart sound is normal but may be difficult to hear, and the intensity of the second heart sound is normal or only slightly increased. Gerbode defects cause a loud, holosystolic murmur heard at the left mid-lower sternal border because of the pressure gradient from the left ventricle to the right atrium (27). In those cases, a decrescendo high-pitched diastolic murmur may be heard in the aortic position. In the infant who has a long-standing left-to-right shunt, left atrial enlargement is typically present. Those with elevated pulmonary vascular resistance will have evidence for right ventricular hypertrophy, sometimes with a strain pattern. As pulmonary vascular resistance drops, the chest x-ray of children with a large P. Left ventricular volume overload will result in cardiomegaly, involving dilation of the left atrial and left ventricular segments of the cardiac silhouette (Fig. If pulmonary vascular disease ensues with its associated increase in pulmonary vascular resistance and decrease in pulmonary blood flow, the lung fields on the chest x-ray may appear normal or even dark and underperfused. Cardiomegaly may persist for some time, even after attenuation of the left-to-right shunting across the defect with progressive pulmonary vascular disease. Anatomic evaluation must include identification of the defect type as well as its location using the right ventricular septal surface as reference. Anterior malalignment of the conal septum is best seen in subxiphoid sagittal and right anterior oblique views (see Fig. Posterior malalignment is often more subtle and can be recognized when the aorta sits entirely over the left ventricle (the aorta usually overrides the crest of the muscular ventricular septum in the normal heart) (Fig. This can be demonstrated in a subcostal left anterior oblique view (delineating the left ventricular outflow tract), apical long-axis view, or parasternal long-axis view. The borders or margins of the defect should be delineated, particularly in terms of the area of fibrous continuity between the tricuspid and aortic valves and between the aortic and pulmonary valves. If possible, the conal septum should be identified and characterized, particularly in terms of its alignment (or lack thereof) with the remainder of the muscular ventricular septum. If there is malalignment of the atrial septum and ventricular septum, straddling attachments of the tricuspid valve into the left ventricle should be displayed. In addition, associated lesions such as right or left ventricular outflow tract obstruction (see Fig. Serial measurements of left ventricular size using Z-scores can help determine if the left ventricle is becoming dilated over time. Three-dimensional reconstructions can be created from bright blood data, and these can be sliced in any plane in order to profile the ventricular septum and the defect, thereby helping to plan the optimal intervention strategy if needed. The Qp/Qs ratio can also be estimated with calculation of right and left ventricular stroke volumes from three-dimensional bright blood datasets. The majority of anatomic and physiologic data can be obtained from these modalities without subjecting patients to an invasive procedure associated with radiation exposure. If there is a concern for evolving pulmonary vascular disease, then pressure and oxygen saturation measurements in the superior vena cava, pulmonary artery, left atrium, and aorta during catheterization will allow calculation of pulmonary and systemic vascular resistance (left atrial pressure can also be estimated from pulmonary capillary wedge pressure measurements, and pulmonary venous saturation can be assumed to be 99% if there is no evidence for pulmonary disease). Patients with elevated pulmonary vascular resistance can then be challenged with 100% oxygen supplementation or other pharmacologic intervention to evaluate the reversibility of the pulmonary vascular disease. Aortograms may be helpful to identify and characterize aortic regurgitation as well as the presence of a patent ductus arteriosus, particularly if the right ventricular and pulmonary arterial pressures are at or near systemic levels. Generally a Qp/Qs ratio of less than 2:1 suggests that the residual shunt is small enough to avoid additional surgery. In general, the accessory tricuspid valve aneurysm does not alter tricuspid valve function. Unrecognized, spontaneous closure of the ductus arteriosus results in cardiovascular collapse and death usually in the first few days of life. Central (perimembranous) and outlet (doubly committed subarterial) defects may become partially or completely occluded by a prolapsing aortic valve leaflet. As previously described, aortic valve prolapse may also cause leaflet distortion and aortic regurgitation. Though poorly understood, it is thought that the high-velocity jet that hits the right ventricular free wall causes hypertrophy and development of a prominent right ventricular muscle bundle. The gradient across the muscle bundle may become significant such that hypertension of the proximal segment of the right ventricle develops. In general, large defects may be associated with premature death if closure is not performed in the first 2 years of life. Pulmonary vascular disease and biventricular failure may occur with long-standing large left-to-right shunt and associated systemic right ventricular pressure. Elevated pulmonary vascular resistance that is unresponsive to medical management may occur in as many as one-quarter of patients who have not had an intervention by 2 years of age (52). Alternatively, a moderate-sized defect may behave similarly to a large defect and require surgical or catheter-directed intervention. Over months to years, a large left-to-right shunt will typically lead to pulmonary vascular disease.

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Interestingly order 10mg motilium otc gastritis diet , it is not uncommon even in the best of circumstances may be only one or two for there to be an inverse relationship between the prestige of resignations or frings from collapse discount 10 mg motilium amex chronic gastritis with h pylori. One of the commonest the medical school and the salary paid by its affliated teach- reasons for failure of a congenital heart program is failure of a ing hospital order motilium 10mg overnight delivery gastritis diet spanish, although geographic factors also play a strong junior and politically inexperienced cardiac surgeon to garner role order motilium 10 mg with visa gastritis uptodate, with salary usually being higher in larger and more the necessary resources to ensure the success of the program. In general and despite the low percent- may be a higher salary than in a popular urban location. However, it requires a substantial investment in hospital is similar to that of many private corporations, the both infrastructure and personnel. And just as in building a important difference being that members of the hospital board successful sports franchise, the key element is not just hir- are serving pro bono. Furthermore, these generous men and ing those who appear to have had the necessary training but women also play an important role in fundraising for the hos- hiring individuals who will be a good “ft” with the team. In addition to the administrative outnumbered 10:1 by his cardiology colleagues to understand board, a group of executives performs the day-to-day tasks how the power is structured within the hospital and who his or of running the hospital. Generally, there is a chief executive her allies are within that administrative structure. These individuals are responsible other reAsons Why congenitAl heArt progrAms fAil for the fscal strength of the hospital. They work closely with the chief of surgery, the chief of medicine (in a pediatric hos- The very rapid growth of cardiology programs over the last pital the chief of pediatrics), and the chief of nursing. The 20 years has produced an important imbalance of power hospital executive group is advised not only by the adminis- within the traditional hospital administrative structure. The trative board, but also by the surgical executive council made chief of pediatrics is very likely to do the bidding of the chief up of the chiefs of surgical departments and/or divisions, and of cardiology, while the chief of surgery fnds that cardiac a medical staff executive composed of the heads of medical surgery is directly contributing no more to his or her bottom departments and divisions. Thus, he or she can play only a very limited role in ology and chief of cardiac surgery are staff recruitment and the surgical executive committee. Obviously, there are mul- surgeon has multiple other roles, all of which can be a distrac- tiple other roles including oversight of the care delivered by tion to fnding adequate time to compete for resources within physicians within the department, supervision of the teach- the traditional hospital administrative structure. All of these ing and training aspect of the department, and oversight of factors can lead to failure of a congenital heart program. Caring for the Patient and Family with Congenital Heart Disease 5 rebUilding the fAiled congenitAl heArt progrAm There are challenges, however, in developing a heart institute structure. The chief of surgery and/or the chief of Once failure of a congenital cardiac program has occurred, pediatrics may be unwilling to cede the power or revenue there are three main directions in which the failed program involved, and this can be exacerbated by a fscal segregation can move in order to restructure (Box 1. Hospital adminis- First, the congenital cardiac surgical program can be con- tration may not be willing to change and break with tradition, solidated into a major regional center with maintenance of the the chief of cardiology may be unwilling to share prestige, local cardiology program and with total outsourcing of surgi- power, and dollars with a junior surgeon, and the political cal patients. Second, a heart institute structure can be devel- inexperience of a junior surgeon advocating within the heart oped with a horizontal political administration rather than a institute structure may lead to instability. Finally, administrator model, which has been pioneered by Frank there can be oversight by a traveling senior surgeon. The senior of a surgical program, include (1) the presence of a successful traveling surgeon recruits less experienced surgeons who regional center within 100 miles and within the same state, remain on site and perform simple and moderate complex- (2) that it is the preference of the cardiologists and intensivists ity cases alone. The senior surgeon acts as gram, (4) the lack of a strong medical school, and (5) specifc a surgical administrator and advocate for the pediatric pro- challenges, whether they be geographic, cultural, or fnancial, gram and performs more complex cases on site. The challenge outsourcing usually does not happen principally because of for the traveling surgeon scenario is the stigma of itinerant fnancial issues. In addition, there are the home and important factors of regional pride as well as the desires of family stresses of frequent travel. With this structure, the most senior adminis- AmericAn medicAl AssociAtion trators within the group advocate for cardiac surgery in a way that is not possible for a chief of surgery or chief of cardiac The stated mission of the American Medical Association is surgery, who have too many competing allegiances with sur- to “promote the art and science of medicine for the better- geons from other specialties. This is a particular problem if ment of the public health, to advance the interests of physi- the pediatric cardiac surgical program is administered with cians and their patients, to promote public health, to lobby for an adult cardiothoracic surgery program, where revenue and legislation favorable to physicians and patients, and to raise volume (but not income expectations) have declined substan- money for medical education. In the United States, the role of spe- cardiothoracic surgeons including congenital cardiac sur- cialty certifcation is taken by the American Board of Medical geons. The American Board of Thoracic Surgery over- stringent requirements including the need to have made a sees certifcation of general cardiothoracic surgery and the signifcant contribution to the specialty. Most members have recently developed subspecialty certifcation in congenital published at least 40 or 50 scientifc papers and are usually cardiac surgery. There a non-government nonproft organization separate from the is an annual scientifc meeting, usually in early May, at which Accreditation Council for Graduate Medical Education that some of the most important research breakthroughs are pre- oversees training positions in teaching hospitals. It member and supporting letters of recommendation by at least plays a strong political advocacy role including a perma- two additional surgeon members. The two major cardiology societies play important roles in advocating for patients with heart disease and increasing aware- ness of heart disease in the community. The American Heart congenitAl heArt sUrgeons’ society Association has an important focus on fundraising and support The Congenital Heart Surgeons’ Society began as a small of research. Both societies have annual scientifc meetings and club that met annually at a Chicago airport hotel. They senior surgeons in the emerging feld of congenital cardiac each have small subspecialty “councils” representing pediatric surgery to discuss challenging cases in an informal atmo- heart disease and cardiac surgeons, but they are overwhelm- sphere. The society has grown to more than 100 members ingly about acquired heart disease in adults and adult cardi- representing all of the major congenital cardiac surgical ologists. Membership requires nomination, letters of reference, programs in North America as well as a small number of and review by a membership committee. Membership requires a signifcant contribution to the feld through clinical activity, teaching of thorAcic sUrgery societies: AmericAn surgical trainees, and research. The society maintains a data center, formerly at the University of Alabama where it was maintained by Dr. John Kirklin (a) not only pioneered many cardiac surgical procedures, but was also responsible for establishing the data center of the Congenital Heart Surgeons Society currently located in Toronto, Canada. He was very ably assisted by his long-term colleague at the University of Alabama, Dr. Eugene Blackstone (b), who has been instrumental in developing rigorous statistical analysis of cardiac surgical outcomes. Constantine “Gus” Mavroudis, formerly from Chicago and now from Orlando, Florida, and (c) Dr. Bove (front row center), Austin (back row right), and the author are pictured with their wives at the gala dinner in Warsaw, Poland, of the combined meeting in 2008 organized by Dr. The society meets every fourth year with the European Congenital Heart The World Society brings together congenital cardiac sur- Surgeons Association (Fig. The society publishes the only journal exclu- pital’s administrative structure as well as advocating for the sively devoted to surgery for congenital heart disease, the care of patients with heart disease both in the local commu- World Journal for Pediatric and Congenital Heart Surgery, nity and globally. The print journal is available through membership, while the online journal is presently available for no charge. Prevalence of congeni- that cares for the child or adult with congenital heart disease.

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In these instances purchase motilium 10mg gastritis kefir, the lumen within the stent “remodels” with neointima to the size of the normal adjacent vessel generic motilium 10mg without prescription gastritis diet cabbage. These patients have continued to survive into adulthood order motilium 10mg on-line gastritis kaffee, often with very minimal early limitations in their overall cardiopulmonary exercise capacity motilium 10mg lowest price gastritis diet menu plan. However, it has gradually become very clear that the “atrial switch” procedures have not “corrected” these patients, but instead have created a long-term palliation with very favorable early results. As a result of their often fairly normal overall cardiovascular status, several patients have been “lost to follow-up” or only been followed up in large intervals without very detailed diagnostic studies being performed on a regular basis. However, as these patients have survived into adulthood, more patients have become symptomatic from problems that are related to the early palliation and which are frequently amenable to transcatheter and/ P. In addition, the right ventricular function is frequently found to be reduced, often manifesting itself in an increasing amount of tricuspid regurgitation. The workup prior to any transcatheter intervention should include an exercise study with documentation for potential arrhythmias or desaturations on exercise, a transthoracic or preferably transesophageal echocardiographic evaluation with documentation of any potential baffle leak or baffle obstruction, as well as a Holter recording. The electrophysiologic team has to be closely involved in preparing these procedures, deciding upon the best physical location (adult or pediatric hospital) as well as the order in which each individual part of the procedure is performed. This may not only include the pediatric, but in some patients also the adult electrophysiologic team who may have a higher expertise in, for example, complex pacing lead extractions. For example, a patient with a permanent pacemaker and a lead fracture may benefit from lead extraction within an adult center. In general, most patients will usually undergo a combined procedure, during which the interventional cardiologist initially performs a full left- and right-heart hemodynamic evaluation (ideally obtaining jugular venous as well as femoral venous access), which will give some idea of potential pulmonary or systemic baffle limb stenoses. If multiple abnormalities are identified it is very important to involve the cardiosurgical team during the procedure. That allows determining, which lesions may require surgical revision, while deciding at the same time, whether other lesions could/should be addressed during the transcatheter procedure. Many cardiologists have in the past adopted a “wait and see” approach when faced with baffle limb obstructions or baffle leaks. Baffle leaks can also lead to desaturation with and without exercise and thereby may contribute to a reduced exercise tolerance while moderate L-R shunts add unwanted volume loading to the already reduced right ventricular function. At this institution we have therefore adopted an approach that attempts to eliminate any residual anatomic or morphologic abnormalities as much as possible through transcatheter therapy, which has been documented to be an excellent form of therapy for these lesions with very favorable results (118). Stents across a baffle leak should only be placed after the leak itself has been occluded using a transcatheter device (Fig. Where approved and available, a combination of baffle leak and obstruction can be more readily treated using covered stents. Pulmonary venous baffle limb stenoses can be addressed either transseptal, or using a retrograde aortic approach through right ventricle and tricuspid valve. The short length of these lesions usually makes them less amenable to intravascular stent therapy, but some improvement can be achieved using high-pressure balloon angioplasty. An overview of a single-center experience of managing these patients using a combined team approach was presented by Holzer and colleagues in 2006 (119). Between December 2002 and April 2006, 81% of all patients that were followed after atrial switch procedure underwent transcatheter evaluation. Electrophysiologic or pacing procedures were performed in the same setting in 15 patients. Seven percent of patients had a pulmonary venous baffle limb stenosis while 57% of patients had baffle leaks. Devices for baffle leaks were implanted in 11 patients, while 5 patients received a covered stent for a combination of baffle limb stenosis and leak. After stent implantation, the gradient across the baffle limb was reduced significantly from a mean of 3 mm Hg to a mean of 1 mm Hg, while the mean diameter improved significantly from 8. Transcatheter baffle leak occlusion was performed or attempted in 17 patients, while 3 patients had unsuitable locations of the baffle leak, thereby requiring surgical revision. Trivial residual leaks were seen in two patients, usually around small right atrial trabeculations. This data demonstrates clearly that patients after atrial switch have significant pathology that can be treated in the catheterization laboratory. Transcatheter and electrophysiologic therapy have an important role within the multidisciplinary management of these patients. However, prospective clinical trials that compare this device with other available devices in a controlled and randomized fashion have not been conducted. Vascular access is usually obtained in the right femoral vein, placing an additional arterial monitoring cannula in the right femoral artery. In patients with bilateral occluded femoral veins, device deployment and delivery should usually be performed using transhepatic access, which may even provide for better alignment of the device to the atrial septum (124). Internal jugular venous access is the least preferable access route and is unsuitable for device delivery in most patients. A standard hemodynamic left and right heart catheter evaluation should be performed prior to device closure. This should include evaluation of pulmonary artery pressures, pulmonary vascular resistance, and estimation of the atrial-level shunt. Therefore tube angulations may have to be adjusted after initial angiography to achieve a better profile of the atrial septum. Closure of large intra-atrial communications in “unprepared” patients with left ventricular diastolic dysfunction can lead to a significant increase in left atrial pressure due to the loss of “pop-off” via the atrial septum, with resulting pulmonary edema and ventilator dependency (125). The left atrial/wedge pressure should be evaluated at baseline as well as after test occlusion of the defect. In many patients, placing the device across the defect without release and then recording pulmonary artery wedge pressure via a second catheter eliminates the potential of the balloon size interfering with the obtained pressure tracing. While a small increase (<5 mm Hg) in left atrial pressures may be acceptable and reasonably well tolerated, an increase of left atrial pressure by a mean of 10 mm Hg or more is clearly prohibitive to occlusion of the septal defect (Fig. Preferably, the treatment of any patient in whom these physiologic changes are expected, such as P. This should include aggressive diuretic therapy as well as afterload reducing agents. If a patient, despite appropriate pretreatment, still develops significant left atrial hypertension after test occlusion, the placement of a fenestrated device may be necessary. Alternatively, if these fenestrations need to be maintained for a longer period of time, placement of a short endovascular stent through the device may be beneficial 127). E, F: Short-axis view without and with color flow mapping after release, documenting excellent device position and no significant residual shunt. It is extremely important to expand the balloon until the atrial-level shunt is just abolished. The dimensions are then recorded on echocardiography as well as on cine recording. However, unless a mild waist is visible, cine recordings can be misleading at times, P. Many centers now completely avoid balloon sizing, especially in small children, and instead use an averaged maximum diameter taken from the three standard views to determine the appropriate device size. In patients with a deficient retroaortic rim where the two discs are expected to hug the aortic root on either side, the device size may have to be increased further. Once the device size has been determined, the appropriate delivery sheath is placed over the guidewire into the mouth of the left upper pulmonary vein.