CPA stenoses and hypoplasia need surgical attention in approximately half of all patients undergoing the NP. The need for reoperation is increased when using the RV-PA conduit technique (although the majority of these are performed as part of the Stage II procedure). Catheter reinterventions are almost exclusively confined to the left CPA and are increased when the arch is shortened by resection of the coarctation tissue at time of NP.
Williams-Beuren syndrome (WBS) is a multisystem genetic disorder comprising of craniofacial, developmental, and cardiac malformations. The most common cardiac defects found are supravalvar aortic stenosis and peripheral pulmonary stenosis. However, WBS should be regarded as a general arteriopathy consisting of stenoses of medium- and large-sized arteries including the coronary arteries. Cardiac manifestations are often the initial reason for referral and careful cardiovascular assessment is important as coronary artery involvement confers a significant anesthetic risk and may be associated with ischemia and resultant ventricular dysfunction. Here we review the literature and describe a 2-year-old boy with evolving clinical features of WBS. He presented to our pediatric cardiology department for a routine assessment of peripheral pulmonary branch stenosis. A 12-lead electrocardiogram showed changes consistent with left ventricular ischemia and a two-dimensional echocardiogram showed reduced left ventricular function and mild supravalvar aortic stenosis. Subsequent cardiac catheterization diagnosed severe left main coronary artery stenosis. Deteriorating ventricular function secondary to acute ischemia postcatheterization required intensive care treatment from which the patient did not recover. This case report highlights the necessity of careful cardiology assessment without delay in patients with a suspicion of WBS. Isolated coronary stenosis though rare in WBS should be considered in the presence of ischemia or reduced ventricular function. Larger case series are needed to further characterize the correlation between WBS and acute coronary events.
We describe a 2-year-old girl with tetralogy of Fallot and pulmonary atresia, palliated as a neonate with a right modified Blalock Taussig shunt, who developed severe cyanosis following total correction in the absence of corresponding evidence of parenchymal lung disease on the chest X-ray. Selective pulmonary angiography showed new intrapulmonary shunting involving only the right middle and lower lobes only. The cyanosis resolved rapidly subsequent to inhalation of nitric oxide. To our knowledge, this is the first documented case of rapid onset of localised intrapulmonary right-to-left shunting, involving only two lung lobes, following biventricular repair for complex congenital heart disease.
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