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Congenital Portal

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This video shows a simple operative technique for the management of scimitar syndrome with hypoplasia of the right lung and a ventricular septal defect in a six-month-old infant.
March 21, 2013
Brom’s three-patch aortoplasty is indicated for the repair of supravalvar aortic stenosis, which can occur as an isolated defect or as a part of Williams syndrom.  This syndrome is characterized by supravalvar aortic stenosis, elfin craniofacial features, peripheral pulmonary artery stenosis, and hypercalcemia. 
March 21, 2013
The completion Fontan operation is, as the name implies, the final stage of palliation for patients who have had previous surgery to address various types of single ventricle congenital heart disease.
March 21, 2013
The surgical management of patients with tetralogy of Fallot and significant right ventricular outflow tract obstruction (RVOTO) remains controversial. The authors describe the use of a monocusp in patients with right ventricular outflow tract obstruction.
March 21, 2013
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly that is one of the most common causes of myocardial ischemia and infarction in children. If untreated, it results in a mortality rate of up to 90% within the first year of life. In the current era, establishment of a two coronary artery system is considered the goal for repair of ALCAPA.
March 21, 2013
The Aortic Translocation procedure was introduced for the management of patients with complex transposition of the great arteries (TGA) with a ventricular septal defect (VSD) and pulmonary stenosis (PS). The technique has proven especially useful in the subset of patients with anatomic variables that complicate the performance of a Rastelli repair.
March 21, 2013
The superior cavopulmonary anastomosis is the first of two operations used in a staged Fontan approach for definitive palliation of functionally single ventricle hearts.
March 17, 2013
In one-third of patients with congenitally corrected transposition, there can be co-existing pulmonary stenosis. As explained, although it is stated that no heart exists in the Idriss archive with this lesion, this is not the case, and such a heart in shown in the next video clip.
March 17, 2013
By  Robert H. Anderson, MD, FRCPath Introductory Remarks
March 17, 2013
In the second videoclip exploring congenitally corrected transposition, we show a specimen in which the potentially corrected circulatory patterns are uncorrected to a degree by the presence of a ventricular septal defect.
March 17, 2013
In this last video in the series, we examine three specimens with rarer lesions associated with congenitally corrected transposition. The first is an example of a heart with discordant atrioventricular, but concordant ventriculo-arterial connections.

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