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Andersons pediatric cardiology 1359

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Management Large natural history studies on ruptured sinus of Valsalva aneurysms are unavailable in the literature due to the relatively low incidence, and early reports consisted of isolated cases or case series However, most patients were found to have died within a year of rupture from congestive heart failure or endocarditis or prior to the era of surgical repair.75 The management of sinus of Valsalva aneurysms associated with other lesions is based on the underlying disease process (e.g., connective tissue or infectious process) In addition, this may consist of β-blockers, angiotensin-converting enzyme inhibitors, angiotensin II receptor antagonists, and lifestyle modifications with respect to sports restrictions Surgical management strategies are dictated by progression in the size of the aneurysm, in addition to the absolute size of the aortic sinuses In general, although medical management in patients with ruptured sinus of Valsalva aneurysms may help to control symptoms to some extent (with diuretics, angiotensin-converting enzyme inhibitors, and cardiorespiratory support in extreme cases), definitive correction of the defect is warranted in a timely fashion Surgical treatment consists of primary closure of the ruptured aneurysm or patch closure of the defect and can be approached from the aortic end, the end of the chamber into which the rupture has occurred, or both ends.67,68,76–79 Concomitant aortic valve surgery/replacement may also need to be performed In follow-up, progression of aortic insufficiency can occur, necessitating aortic valve replacement.68,77–79 Residual fistulous communications may be present in follow-up and in some instances also require repair.67,68,76–79 Since the first report from Cullen and colleagues,80 there have been numerous reports and series of closure of ruptured sinus of Valsalva communications in the cardiac catheterization laboratory The defect can be closed from a retrograde approach or antegrade approach facilitated by an arteriovenous guidewire loop The majority of these ruptured aneurysms can be occluded in the cardiac catheterization laboratory with the many options of devices available at the interventional cardiologist's disposal in the current era (e.g., VSD, patent ductus arteriosus and atrial septal defect occluder devices) In four relatively large transcatheter series reporting outcomes with patient numbers ranging from,17–25 successful occlusion was achieved in 84% to 94% of patients.81–85 Residual shunt (in most instances resolving) and device-related aortic regurgitation occurred in a minority of patients In a large review of 877 patients undergoing correction of ruptured sinus of Valsalva aneurysms (741 surgical, 136 transcatheter closure), transcatheter closure was favorable with regard to efficacy and safety profile.83 Given our current knowledge, it would seem that transcatheter closure of ruptured sinus of Valsalva aneurysms is an acceptable alternative to surgery and should be considered unless concomitant lesions need to be surgically addressed Regardless of whether transcatheter or surgical closure is performed, patients should be followed for life, with particular emphasis on aortic regurgitation, residual fistulous communications, and coronary artery compromise Triple Outlets From the Cardiac Base These lesions are very rare They are also most unexpected morphologic findings,86 although the finding of three arterial trunks is the usual arrangement in reptiles and crocodilians.8 As congenital malformations, we are aware of descriptions of malformations of the outflow tract of the right ventricle such that each PA has a direct origin from the ventricular roof This can seemingly produce a triple-outlet ventricle when all arterial roots arise from the right ventricle.87 As was explained when discussing the developmental issues, we have now associated a comparable arrangement of the aortic root, whereby duplicated roots fed the aortic trunk, again in the setting of a patient with doubleoutlet right ventricle (Fig 51.21).9 FIG 51.21 Duplication of the aortic root (A) At first look, there is a double outlet from the right ventricle (arrow) (B) However, the aortic root is duplicated Therefore the patient exhibited triple outlet from the right ventricle (Courtesy Dr Ashish Katewa, Chhatishgarh, India.) The first description of triarterial heart, also termed tritruncal heart, was by Diaz-Gongora in 1982.86 This heart was described to have a pulmonary trunk arising from the right ventricle and giving rise to the left PA and arterial duct, while the aorta arose from the left ventricle in normal location posterior to the

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