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Resynchronization therapy in pediatric and congenital heart disease patients: an international multicenter study buy silagra 50 mg on-line causes of erectile dysfunction and premature ejaculation. Regression of tricuspid regurgitation after two-stage arterial switch operation for failing systemic ventricle after atrial inversion operation cheap 50mg silagra amex erectile dysfunction doctor called. The transfer of injurious energy is most commonly associated with blunt trauma purchase silagra 100 mg with visa erectile dysfunction washington dc, penetrating trauma discount silagra 50 mg otc erectile dysfunction treatment with diabetes, or contact with electrical current. Most medical knowledge about cardiac trauma in children is extrapolated from studies of adults. Relatively little direct information is available regarding cardiac injuries in children. Blunt Cardiac Injury Blunt trauma accounts for the vast majority of injuries to children and is the chief cause of cardiac trauma in the pediatric age group. The incidence of cardiac injury in blunt trauma among children has been reported to be from 0% to 43% (1). Blunt cardiac injuries are often unsuspected injuries associated with multiple system trauma. Clinical manifestations of cardiac trauma vary depending on the location of injury. They are often nonspecific and include shock, cardiovascular instability, dysrhythmias, chest pain, and changes in mentation. In victims of multiple trauma, all of these findings can be easily attributed to other serious injuries to the head, abdomen, or extremities. Having a high level of suspicion for blunt cardiac injury is necessary for early diagnosis and intervention. Most blunt cardiac injuries from direct chest impact among children are the result of motor vehicle crashes (3). Blunt blows with weapons, fists, and animal kicks; blunt collisions during sports; and falls from heights also cause direct-impact cardiac injuries. Because the heart is suspended from the great vessels, acceleration–deceleration injuries occur as the heart moves like a pendulum in the thorax. Compression of the chest can crush the heart or cause damage through increased intrathoracic and intracardiac pressures. Cardiac rupture is more likely if compression occurs during maximum filling of the chambers. Rib fractures and contusions of the chest wall are not always seen, especially in children owing to their highly compliant chest walls. Abdominal and lower extremity compression also can force blood back to the heart, causing damage through a hydraulic ram effect. Commotio cordis is a specific form of cardiac trauma that has become an increasingly recognized cause of sudden cardiac death in otherwise healthy young athletes (4). While the National Commotio Cordis Registry in Minneapolis lists almost 250 cases of commotio cordis, the frequency of such cases is most certainly underestimated (5). Commotio cordis is a form of nonpenetrating chest trauma insufficient to result in significant myocardial or chest wall injury. The most likely mode of death appears to be an induction of a malignant arrhythmia (i. The two critical components appear to be precordial impact location and the timing of the impact occurring during the upstroke of the T wave (6,7,8). The impact site for victims of commotio cordis is specifically located at or near to the center of the cardiac silhouette. Recent animal model studies suggest that the trauma must occur within an electrical vulnerability period during the cardiac cycle, namely, within 20 ms of the T-wave upstroke. As reported recently by Maron and Estes (4), commotio cordis is seen most frequently in the second decade of life, peaking at 15 years of age. Baseball is the most common sport in which commotio cordis is observed followed by softball, hockey, and football. It is more commonly experienced during organized sporting events but by no means exclusively. In younger victims (<10 years of age) commotio cordis may occur during activities unrelated to sports. Commotio cordis: the single most common cause of traumatic death in youth baseball. Victims of commotio cordis most commonly experience instantaneous cardiovascular collapse but in approximately 20% of cases victims are able to stay erect for several seconds after the trauma occurs. Survival from resuscitative efforts is low but has increased to 35% in recent years most likely due to increasing availability of automatic external defibrillators and bystanders knowledgeable in the use of these devices. According to data from the above-mentioned national registry, nearly one-third of cases of commotio cordis that occurred during competitive sports were victims who were wearing a commercially available chest protector. Many of these devices were not designed to prevent commotio cordis and either expose the precordium or do not adequately absorb the impact from a projectile (9). Efforts have been made to create safer baseballs (aka “safety” baseballs) with some evidence of effectiveness. It is important that the public and organizers of sporting events be aware of the risk of commotio cordis. Two other mechanisms of blunt cardiac injury have been described: blast injury and a combination injury, which involves more than one of the above mechanisms. The types of anatomic injuries resulting from these various mechanisms in blunt cardiac trauma include pericardial injury, myocardial contusion, cardiac rupture, septal disruption, ventricular aneurysm, injury to the heart valves and supporting structures, and injury to the great vessels, brachiocephalic arteries, venae cavae, and coronary arteries (Table 27. Pericardial Injury Blunt pericardial injuries range from contusion to rupture and are usually associated with myocardial injury. Pericardial lacerations and pericardial rupture are rarely significant injuries unless cardiac herniation occurs through a pericardial tear. Cardiac tamponade is a common complication of myocardial injury but is not likely with isolated pericardial injury. The clinical features are single or recurrent pericardial or pleural effusions, similar to the postpericardiotomy syndrome. The frequency of pericardial injury associated with blunt chest trauma is unknown. Asymptomatic pericardial effusions have been demonstrated by cardiac ultrasonographic examination following blunt chest trauma. Pericardial lacerations were frequently found in dogs following sublethal blunt chest trauma. Traumatic cardiac tamponade rarely presents with all the classic Beck triad features of hypotension, distant heart sounds, and elevated central venous pressure with neck vein distension. An echocardiogram is the most sensitive diagnostic test for cardiac tamponade and can be used in the emergency department for selected trauma patients. A diagnostic and therapeutic pericardiocentesis also can be used for patients with suspected cardiac tamponade. However, false-negative pericardiocentesis results have been observed in 25% to 80% of patients who have blood in the pericardium (12). If pericardial effusion or associated pleural effusions are clinically significant, pericardiocentesis or thoracocentesis is indicated.

The commonest cause of late death is coronary artery disease followed by sudden death purchase 100 mg silagra fast delivery impotence at 16, heart failure 100 mg silagra fast delivery erectile dysfunction herbs, cerebrovascular accidents discount silagra 50mg without prescription erectile dysfunction ginkgo biloba, and ruptured aortic aneurysm (103) cheap silagra 50 mg line vasculogenic erectile dysfunction causes. Structural abnormalities of the aortic media in coarctation patients have been reported within 24 hours after birth, implying an intrinsic underlying cause (104). Moreover, medial wall abnormalities were identical at sites subjected to high and low pressure (proximal and distal ends of the coarctation site), eliminating the influence of hemodynamics in the described pathology (16). Cardiac structures derived from neural crest cells include the outflow tract of the heart and the aortic arch system, as well as the cervicocephalic arteries. Development of aneurysm at the site of surgical repair is associated with the patch graft technique and repair of coarctation before the age of 14 years (11). Imaging follow-up of both the ascending and descending thoracic aorta is recommended in coarctation patients, irrespective of previous repair, for early detection of aortic wall complications (101,107). Prophylactic treatment with β-blockade as well as appropriate timing of surgical intervention on the dilated ascending aorta in this patient cohort remains uncertain. Nonetheless, the cumulative incidence of grade 2 or higher regurgitation was 9% at 15 years, with pulmonary outflow obstruction reported as the main cause for reoperation. Interestingly, aortic root pathology has also been described in this lesion following the Mustard procedure, but may be largely underestimated due to other serious complications of the technique, such as right ventricular dysfunction, arrhythmias, and sudden cardiac death (1). Importantly, in the majority of patients with neoaortic dilation, a stabilization of Z-scores was observed during late follow-up, suggestive of absence of progressive dilation. Risk factors for neoaortic dilation included previous pulmonary artery banding and older age at operation. Other Lesions Structural abnormalities of the aortic media have been documented in a wide range of congenital heart defects, including ventricular septal defect, truncus arteriosus, double outlet right ventricle, tricuspid atresia, and double aortic arch (16). These changes may be present as early as the neonatal period, raising the question of a common genetic substrate which is yet to be elucidated. Despite the exponential research in this field during the last decade, there is a paucity of data in terms of available genetic screening and development of new therapies. However, recently recognized molecules promoting aneurysm formation are being targeted in animal models. Marfan syndrome in children and adolescents: predictive and prognostic value of aortic root growth for screening for aortic complications. Progressive dilation of the ascending aorta in children with isolated bicuspid aortic valve. Aortic root dilation and aortic elastic properties in children after repair of tetralogy of Fallot. Massive aortic aneurysm and dissection in repaired tetralogy of Fallot; diagnosis by cardiovascular magnetic resonance imaging. Thoracic aortic dissection and rupture in conotruncal cardiac defects: a population-based study. Predictors of aneurysmal formation after surgical correction of aortic coarctation. Long-term predictors of aortic root dilation and aortic regurgitation after arterial switch operation. Structural abnormalities of great arterial walls in congenital heart disease: light and electron microscopic analyses. Vascular matrix remodeling in patients with bicuspid aortic valve malformations: implications for aortic dilatation. Expression of a mutant human fibrillin allele upon a normal human or murine genetic background recapitulates a Marfan cellular phenotype. Evidence for a critical contribution of haploinsufficiency in the complex pathogenesis of Marfan syndrome. Comprehensive genetic analysis of relevant four genes in 49 patients with Marfan syndrome or Marfan-related phenotypes. Marfan syndrome: 30 years of research equals 30 years of additional life expectancy. The Marfan syndrome in early childhood: analysis of 15 patients diagnosed at less than 4 years of age. Comparison of clinical characteristics and frequency of adverse outcomes in patients with Marfan syndrome diagnosed in adulthood versus childhood. Two-dimensional echocardiographic aortic root dimensions in normal children and adults. Marfan syndrome in children and adolescents: an adjusted nomogram for screening aortic root dilatation. What is the best treatment for patients with acute type B aortic dissections–medical, surgical, or endovascular stent-grafting? Aortic stiffness and diameter predict progressive aortic dilatation in patients with Marfan syndrome. Phenotypic features and impact of beta blocker or calcium antagonist therapy on aortic lumen size in the Marfan syndrome. Losartan added to beta-blockade therapy for aortic root dilation in Marfan syndrome: a randomized, open-label pilot study. Losartan reduces aortic dilatation rate in adults with Marfan syndrome: a randomized controlled trial. Recommendations for physical activity and recreational sports participation for young patients with genetic cardiovascular diseases. Surgery for aortic root aneurysm in children: a 21-year experience in 50 patients. Aortic valve-sparing operations in patients with aneurysms of the aortic root or ascending aorta. A prospective study to assess the frequency of familial clustering of congenital bicuspid aortic valve. Frequency by decades of unicuspid, bicuspid, and tricuspid aortic valves in adults having isolated aortic valve replacement for aortic stenosis, with or without associated aortic regurgitation. Coarctation of the aorta, bicuspid aortic valve and abnormal ascending aortic wall. Ascending aortic dilatation associated with bicuspid aortic valve: pathophysiology, molecular biology, and clinical implications. Abnormal aortic valve development in mice lacking endothelial nitric oxide synthase. Identification of vascular endothelial genes differentially responsive to fluid mechanical stimuli: cyclooxygenase-2, manganese superoxide dismutase, and endothelial cell nitric oxide synthase are selectively up-regulated by steady laminar shear stress. A larger aortic annulus causes aortic regurgitation and a smaller aortic annulus causes aortic stenosis in bicuspid aortic valve. Spatiotemporal patterns of smooth muscle cell changes in ascending aortic dilatation with bicuspid and tricuspid aortic valve stenosis: focus on cell-matrix signaling. Bicuspid aortic valve: clinical approach and scientific review of a common clinical entity. Reduced aortic elasticity and dilatation are associated with aortic regurgitation and left ventricular hypertrophy in nonstenotic bicuspid aortic valve patients.

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The superior sagittal sinus runs in the upper area cheap 100 mg silagra with visa circumcision causes erectile dysfunction, anterior limb of the internal capsule buy 100 mg silagra with amex impotence juice recipe, anteroinferior part border of the falx cerebri whereas the inferior sagittal sinus of the striatum silagra 100 mg fast delivery erectile dysfunction weed, and anterior hypothalamus buy 50mg silagra fast delivery erectile dysfunction medication reviews. Large from the second half of A1 are smaller and join the arterial anterior skull base tumors may have large veins draining plexus of the optic nerve, chiasm, and optic tract. Defcits associated with injury to these arteries the trunk of A1 before entering the subarachnoid space and include serious and incapacitating memory defcits, personal- penetrating the brain at the junction of the medial and lateral ity changes, and electrolyte imbalances. It is an important vessel to preserve because it can result in paraplegia of the lower extremities with sparing supplies the head of the caudate and the anteroinferior inter- of the upper limbs, incontinence, abulic or motor aphasia, and nal capsule, anterior hypothalamus, and olfactory region. It is vitally important that both members of this skull base team have endoscopic skills. Here the neuro- surgeon learns how to manage the endoscope and how to work from the video monitor in two dimensions rather than with the microscope in three dimensions. The sinus surgeon learns how to manipulate intracranial tumors and surround- ing neural and vascular structures. These hours spent on pituitary tumor resection build confdence within the skull base team enabling benign or malignant nasal tumors with intracranial extension to be tackled. The frst steps for access are complete sphenoethmoidec- tomy with exposure of the entire skull base. If the tumor is relatively posteriorly sited with a relatively small intracranial extension, skull base resection can be performed without a modifed Lothrop procedure. In such cases the intracranial extension of tumor must be small and the resection of this extension should be possible without it being necessary to resect across the midline. It then swings anteriorly before should be clearly delineated and then enlarged to expose un- becoming­the­paraclival­carotid­in­the­foor­of­the­sphenoid­and­pro- involved dura on all sides of the defect. The orbitofrontal usually originates from the frst is required then the next step is to perform an endoscopic 5 mm of A2 at the junction of the lamina terminalis and callo- modifed Lothrop procedure allowing the anterior aspect of sal cisterns and has a downward and forward course crossing the skull base to be delineated (Fig. The frontopolar branch is separated from the skull base allowing visualization of the originates after the orbitofrontal branch and travels anteri- entire skull base from the frontal sinuses anteriorly to the orly more medially across the subfrontal sulcus. Before the skull base can be resected the anterior to respect the optic nerve canals as their lateral landmarks. The technique of removing residual attachment holding the skull base is the attachment lamina papyracea to fnd the anterior artery as it enters the of the falx cerebri to the crista galli (Figs. An easier and safer technique is to run the diamond burr over the region of the anterior ethmoidal artery removing the bone until the artery is exposed in its canal. This is done bilaterally before the posterior eth- moidal arteries are also identifed using the diamond drill on the skull base. The arteries usually enter the skull base at the junction of the posterior ethmoids and sphenoid and the drill is run over this region of the skull base until the artery is clearly identifed, cauterized, and cut on both sides (Fig. The next step is to perform an endoscopic modifed Lothrop procedure as set out in Chapter 9. A septal perfo- ration (window) is performed and the frontal sinus opened bilaterally and communicated by removal of the intersinus septum (Fig. A straight through-cutting Blakesley is used to cut the nasal septum at its insertion on the skull base. This isolates the skull base and allows the osteotomies to be made so that the skull base can be dropped into the nasal cavity (Fig. The falx cerebri often has vessels running in it and should dissecting them free from the tumor. The falx can extend posteriorly for some plane is identifed and the tumor wall delivered into the distance (often more than 1 cm) so care needs to be taken created cavity. This can be done superiorly looking for the not to damage the vessels lying on each side of the falx. With either technique once the base can then be dropped into the nasal cavity and removed vessels have been identifed, the vessel is dissected free from (Figs. Alternatively, if it attachments under stretch, thereby allowing the second is felt that this cannot be safely done either due to the consis- surgeon to either dissect free or cut any small residual at- tency of the tumor, position of the tumor, or endoscopic skill tachments under visualization with magnifcation. If the levels of the surgeons, then residual tumor should be left and posterior osteotomy is not 100% complete, this attachment the vessels not endangered. In most cases from the tumor but vessels supplying the tumor are cauter- limited extension of tumor through the skull base will be re- ized with bipolar forceps and divided. If the dura or a large vessel such as the intrasphenoid carotid Such a dissection is again very delicate requiring great endo- artery is exposed then coverage of this area is considered im- scopic skill from both surgeons. When the A1-A2 complex is engulfed of the thigh for the harvesting of fascia lata during the pro- in tumor there are two routes to identifying the vessels and cedure and pituitary tumor cases have either the thigh or 262 Endoscopic Sinus Surgery A B Fig. We do not use the balloon intracranially and used to support the frst underlay fascia routinely as it is important to do an immediate postopera- lata graft or dural substitute graft. We now tend to secure this underlay graft anteriorly by placing two 1-mm holes through the skull base and either passing tails of the graft through these holes and “riveting” the graft or by placing a suture through the underlay graft and passing it through the holes and tying the two sutures together thereby fxing the graft anteriorly (Fig. It must not have any folds and must be smoothly adherent to the inner surface of the skull base. A thin ring of fat is placed at the defect edges on the underlay graft as a gasket to increase the seal of this layer. In the past a second layer of fascia lata was placed as an overlay on the skull base, again ensuring that there were no folds and that it lay closely approximated to Fig. If a free mucosal graft is available been made through the anterior table anterior to the defect. In our experience this of the defect by ,10 mm in all directions and be placed onto was­the­most­common­place­for­postoperative­cerebrospinal­fuid­leak. In the cadaver dissection (B) the postnasal artery vision and the packing force is at all times is controlled by the the incisions have not gone through all the layers the sec- surgeon. To close the defect either intradural un- substantially altered the way that intra-arachnoid skull derlay fascia lata graft or a dural substitute such as the colla- base defects are closed today (Figs. This branch leaves the the bone edges of the defect and, as above, maintain smooth sphenopalatine foramen in its posterior superior aspect contact with the bony defect edges. The pedicled septal fap and travels along the anterior face of the sphenoid below needs to be larger than the defect and overlap the mucosa the natural ostium of the sphenoid before reaching the pos- free bony margins of the defect by at least 5 mm. Here it divides into two main tal fap from one side is not large enough, consideration can branches which, through the anastomosis with the ante- be given to using the opposite septal fap and resecting the rior and posterior ethmoidal artery branches, supply the septum. In some patients this usually performed after the sinuses have been fully opened may still not allow complete coverage of the defect and a sec- and the middle turbinate resected. Be aware that the poste- ond layer of fascia lata is then used to create the second layer rior middle turbinate resection should not compromise the and may be bolstered by thin strips of fat on joining areas. Performing the sinus surgery The faps are secured with Surgicel and sealed with fbrin before raising the fap gives extra space and makes the pro- glue before being covered with Gelfoam and supported by cedure much simpler. One of the morbidities associated with using the pedicled My preference is to use a scalpel blade to make the inci- nasal septal fap is the crusting that happens on the donor site sion for the fap although others use a Bovey unipolar needle. To overcome this problem we make a U-shaped The superior incision starts at the lower edge of the natural incision on the opposite septal fap based anteriorly so that sphenoid ostium and proceeds toward the skull base then the posterior portion of this mucosa can be swung around coming anteriorly until just behind the mucocutaneous onto the opposite nostril and used to cover the donor site. It turns vertically onto the foor of the nose and This fap is secured with sutures at the beginning of the pro- then proceeds posteriorly to the posterior choana where it cedure (Fig. At times the nose and nasal vestibule can is taken along the inferior edge of the choana to the lateral dry out during the procedure making the passage of instru- nasal wall (Fig. Where a rectangular piece of silastic over the columella with each 264 Endoscopic Sinus Surgery A B C D Fig.

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Life cycle 50mg silagra amex impotence natural home remedies, sites of predilection and relation to clinical course of rheumatic fever purchase silagra 100 mg on-line erectile dysfunction causes alcohol. Lesions in the auriculoventricular conduction system occurring in rheumatic fever purchase 100mg silagra fast delivery impotence and prostate cancer. Mechanisms of mitral valvar insufficiency in children and adolescents with severe rheumatic heart disease: an echocardiographic study with clinical and epidemiological correlations cheap 50 mg silagra best herbal erectile dysfunction pills. Acute severe mitral regurgitation during first attacks of rheumatic fever: clinical spectrum, mechanisms and prognostic factors. Postinflammatory mitral and aortic valve prolapse: a clinical and pathological study. Special Writing Group of the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease of the Council on Cardiovascular Disease in the Young of the American Heart Association. Rheumatic fever in a high incidence population: the importance of monoarthritis and low grade fever. Diagnosis of rheumatic fever: current status of Jones Criteria and role of echocardiography. The initial attack of acute rheumatic fever during childhood in North India; a prospective study of the clinical profile. Rheumatic fever and rheumatic heart disease: clinical profile of 550 cases in India. Clinical profile of rheumatic fever and rheumatic heart disease: a study of 2,500 cases. Acute rheumatic fever in New York City (1969 to 1988): a comparative study of two decades. Acute rheumatic fever and rheumatic heart disease in Fiji: prospective surveillance, 2005–2007. Rheumatic fever diagnosis, management, and secondary prevention: a New Zealand guideline. Consensus guidelines on pediatric acute rheumatic fever and rheumatic heart disease. Australian Guideline for Prevention, Diagnosis, and Management of Acute Rheumatic Fever and Rheumatic Heart Disease. New Zealand guidelines for the diagnosis of acute rheumatic fever: small increase in the incidence of definite cases compared to the American Heart Association Jones criteria. Review of 609 patients with rheumatic fever in terms of revised and updated Jones criteria. No increased risk of valvular heart disease in adult poststreptococcal reactive arthritis. Prevention of rheumatic fever and diagnosis and treatment of acute Streptococcal pharyngitis: a scientific statement from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee of the Council on Cardiovascular Disease in the Young, the Interdisciplinary Council on Functional Genomics and Translational Biology, and the Interdisciplinary Council on Quality of Care and Outcomes Research: endorsed by the American Academy of Pediatrics. Review of the literature and long-term evaluation with emphasis on cardiac sequelae. Are all recurrences of “pure” Sydenham chorea true recurrences of acute rheumatic fever? Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections: clinical description of the first 50 cases. Therapeutic plasma exchange and intravenous immunoglobulin for obsessive-compulsive disorder and tic disorders in childhood. Streptococcal infection and exacerbations of childhood tics and obsessive- compulsive symptoms: a prospective blinded cohort study. Inflammatory valvular prolapse produced by acute rheumatic carditis: echocardiographic analysis of 66 cases of acute rheumatic carditis. Anterior mitral leaflet prolapse as a primary cause of pure rheumatic mitral insufficiency. Evidence against a myocardial factor as the cause of left ventricular dilation in active rheumatic carditis. Left ventricular mechanics during and after acute rheumatic fever: contractile dysfunction is closely related to valve regurgitation. Echocardiographic evaluation of patients with acute rheumatic fever and rheumatic carditis. The clinical picture of rheumatic fever: diagnosis, immediate prognosis, course, and therapeutic implications. Evaluation of the streptococcal desoxyribonuclease B and diphosphopyridine nucleotidase antibody tests in acute rheumatic fever and acute glomerulonephritis. Congestive heart failure and rheumatic activity in young patients with rheumatic heart disease. A comparison of atrio-ventricular conduction in normal children and in patients with rheumatic fever, glomerulonephritis, and acute febrile illnesses. Role of echocardiography in the diagnosis and follow-up evaluation of rheumatic carditis. Recommendations for evaluation of the severity of native valvular regurgitation with two-dimensional and Doppler echocardiography. Prevalence of rheumatic fever and rheumatic heart disease in school children of Kathmandu city. Epidemiology of rheumatic fever and rheumatic heart disease in a rural community in northern India. The role of echocardiography in diagnosing carditis in the setting of acute rheumatic fever. Optimising echocardiographic screening for rheumatic heart disease in New Zealand: not all valve disease is rheumatic. Idiopathic (degenerative) and rheumatic mitral valve prolapse: historical aspects and an overview. A common color flow Doppler finding in the mitral regurgitation of acute rheumatic fever. Doppler echocardiography distinguishes between physiologic and pathologic “silent” mitral regurgitation in patients with rheumatic fever. Prospective comparison of clinical and echocardiographic diagnosis of rheumatic carditis: long term follow up of patients with subclinical disease. Doppler echocardiography and the early diagnosis of carditis in acute rheumatic fever. Cardiac auscultatory skills of physicians-in-training: a comparison of three English-speaking countries. Usefulness of antimyosin antibody imaging for the detection of active rheumatic myocarditis. Insights from three-dimensional echocardiography into the mechanism of functional mitral regurgitation: direct in vivo demonstration of altered leaflet tethering geometry. Progression of mitral regurgitation: a prospective Doppler echocardiographic study.

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It may become necessary to return on bypass silagra 50mg on line erectile dysfunction venous leak, take approach employing preliminary shunting and later repair buy silagra 100mg erectile dysfunction drugs stendra. When this is lonG-term results after early primary repair undertaken buy silagra 50 mg cheap erectile dysfunction garlic, it is useful to use interrupted pledgetted sutures with the pledgets lying on the endocardial surface of the free In 2001 purchase 100 mg silagra with amex erectile dysfunction and testosterone injections, Bacha et al. Retraction of the main pulmonary artery to view the up was obtained for 45 of the 49 long-term survivors. Although there were eight early Retrograde fow in the left main, as well as evidence of papil- deaths in this early timeframe, there was only one late death lary muscle fbrosis, may alert the echocardiographer to the 24 years after the initial repair. There was no infuence of a transannular patch on Transatrial Approach to Repair of late survival (Fig. The majority of reintervention procedures which obstruction is secondary to moderately hypertrophied muscle 10 patients underwent were for recurrent right ventricular out- bundles rather than generalized hypoplasia of the infundibu- fow tract obstruction which was necessary in eight patients. There is a trend toward a higher rate of reintervention for patients who did not have transannular patch. Other reinterventions included one patient who had a homo- graft pulmonary valve replacement 20 years postoperatively primary versus two-staGe repair performed at another institution and one patient who required a defbrillator for inducible ventricular tachycardia. A small number of centers continue to support an approach Long-term follow-up studies from other centers have of initial palliation with a modifed Blalock shunt in the suggested that residual or recurrent right ventricular out- frst 6–12 months of life followed by subsequent repair3,4 fow tract obstruction is a more serious late problem and a and have been able to achieve excellent early results with more common cause of need for reoperation than pulmonary this strategy. Chen and Moller67 followed 144 patients for 10 underwent a right ventricular infundibular sparing strat- years. They found that patients with right ventricular outfow egy between 1995 and 2008 at Texas Children’s Hospital. Postoperative morbidity reviewed 106 patients who underwent repair of tetralogy at included arrhythmias (3% [10]), postoperative bleeding the University of Minnesota between 1954 and 1960. Similar (2% [7]), temporary renal failure (1% [3]), and neurologic to the experience from Boston, the commonest cause for reop- injury (<1% [2]). Overall eration was recurrent right ventricular outfow tract obstruc- 1- and 7-year Kaplan–Meier survivals were 97 and 96%. For with the mandatory reintervention required with a two- example, this was true in the two institutional study reported stage approach. For exam- of Alabama with an approach of early primary repair as prac- ple, in a 1997 report by Gladman et al. In fact, a high postrepair patients who had a two-stage approach at the Hospital for Tetralogy of Fallot with Pulmonary Stenosis 363 Sick Children in Toronto was 90%, while in patients who had replacement. The latter when indexed to body surface area is currently con- sidered the best measure for proceeding with valve replace- transatrial versus transventricular repair 2 ment. Currently, an indexed volume of 150–165 mL/m or A number of studies have presented excellent results using a greater is considered an indication to operate. Reoperation for right ventricular outfow obstruction was more common in the latter phase of the study, presumably Although some centers have expressed enthusiasm for place- as the strategy of infundibular sparing was more aggres- ment of a monocusp valve at the time of initial tetralogy sively applied. These authors have focused attention on the postoperative echocardiographic fndings of 24 patients who length of the ventriculotomy as an important determinant of had undergone transannular patch repair with a monocusp late right ventricular function. However, it is important to valve with 17 patients who had undergone patch repair with- remember that centers which mainly use a ventriculotomy out a monocusp valve and 20 patients who had undergone approach to the repair of tetralogy focus attention on a num- repair without a transannular patch. The authors found no ber of other factors which determine late right ventricular signifcant differences in the degree of early postoperative function. These factors include: pulmonary regurgitation or in clinical outcomes, such as mortality, number of reoperations, or hospital stay. Although • avoidance of excessive division of right ventricu- 16 of 19 patients had competent monocusp valves immedi- lar muscle ately postoperatively by 24 months only one of seven patients • careful preservation of tricuspid valve function by had a competent valve. No patient had monocusp stenosis, avoidance of sutures snaring tricuspid chords or although this is likely to be an important late consequence of leafet tissue monocusp valve insertion. This can result in severe compres- fracture and the huge variability of the shape and size of the sion of the trachea, mainstem, and peripheral bronchi. In postoperative infundibulum following placement of a trans- addition, the small pulmonary arteries can be affected. We have also seen late left main coronary normal pattern of single segmental arteries is replaced by a obstruction and development of an aorto-pulmonary fstula network of intertwining arteries which compress intrapul- in the setting of a previous Ross-Konno procedure. At the severe end of the for determining optimal timing for late pulmonary valve spectrum, the outlook is dismal. The pulmonary arteries are 364 Comprehensive Surgical Management of Congenital Heart Disease, Second Edition dilated along their entire length to the periphery of the lung, pathophysioloGy anD clinical presentation and airway compression is severe occurring along the length Hypoxemia in this syndrome is the result of both intrapul- of the bronchial tree and into the small airways. Pulmonary venous hand, at the mild end of the spectrum, there is minimal or desaturation can result from ventilation-perfusion mismatch no tracheal bronchial involvement, the small airways are not and intrapulmonary shunting. Air-trapping is encountered leading right ventricular outfow tract obstruction, including pulmo- to overinfated lungs. Hypercarbia commonly ensues with a about the absent pulmonary valve syndrome is that the ductus resultant respiratory acidosis which can be severe enough to arteriosus is never present when the pulmonary arteries are in reduce pH below 7. It is felt that in the presence of a ductus arteriosus, 79 sion is less severe, the child may present later in infancy with this lesion is incompatible with fetal survival to birth. This has led to speculation that the absence of a pulmonary valve promotes DiaGnostic stuDies premature closure and subsequent obliteration of the ductus80 Chest X-Ray or that only those fetuses without a ductus survive to birth. The dilated central pulmonary arteries are thought to enlarge Individual lobes or the entire lung may be involved depend- secondary to the to-and-fro fow between the pulmonary ing on the site of bronchial obstruction (Fig. Histological study of obstruction commonly leads to a considerable mediastinal the central pulmonary arteries has failed to defne the pres- shift to the contralateral side. The pulmonary Computed Tomography and Magnetic annulus is mildly to moderately hypoplastic. There are ves- Resonance Imaging tigial nubbins of primitive myxomatous tissue at the level If there is any doubt that respiratory symptoms are related of the valve annulus, but no true formation of valve leafets. Sedation or general anesthesia may be necessary to ies which are normally 4–5 mm in diameter are commonly obtain a diagnostic quality scan. The origin of the right main bronchus may be compressed at the point where it lies just posterior to the Bronchoscopy dilated right pulmonary artery. Similarly, the left main bron- Bronchoscopy can provide a baseline measure of the degree chus may be compressed at the point where the dilated left of airway compression. The carina itself dilation is mild, bronchoscopy can be used to rule out tra- may be compressed by the distal main pulmonary artery and cheal and bronchial compression as a source of respiratory the origin of the right pulmonary artery. Symptoms Profound Respiratory Compromise in the Neonate Severe respiratory compromise shortly after birth is an indi- cation for emergent or urgent surgical correction. Wheezing, Frequent Respiratory Infections in Infancy In this circumstance, surgical correction should be under- taken shortly after the diagnosis has been established. Elective Repair In the child who is free of symptoms, surgery should be scheduled electively in the frst months of life or shortly after diagnosis has been established. Use of a pulmonary homograft to replace the dilated main and branch pulmonary arteries and the dys- Cardiac Catheterization plastic pulmonary valve following their resection is almost Angiography at the time of cardiac catheterization provides 81 always indicated. However, because Anesthesia and General Setup such dilation is not medically or surgically treatable and because children with such dilation are likely to have severe Method of Cardiopulmonary Bypass In the small, sick respiratory compromise, cardiac catheterization is rarely neonate who is diffcult to ventilate, surface cooling is a use- indicated for absent pulmonary valve syndrome. The room temperature should be lowered and the cooling blan- ket on which the child lies should be activated during the meDical anD interventional therapy placement of monitoring and intravenous lines. It is likely to If the child has respiratory distress when breathing spon- be preferable to use standard ascending aortic cannulation taneously, intubation and positive pressure ventilation are and a single venous cannula inserted through the right atrial indicated. There will be severe mediastinal crowding by the of the most important maneuvers in the medical palliation hyperinfated lung felds making it diffcult to achieve bicaval of this condition is to place the child in the prone position.

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