Aortico-left ventricular tunnel and left ventricular non-compaction are rare congenital cardiac anomalies with varied clinical presentations and sparsely described co-existence in the medical literature. Owing to the limited information about these diagnoses in tandem, we present four cases of aortico-left ventricular tunnel and left ventricular non-compaction from our institution and discuss the clinical presentation, management, and follow-up.

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An infant with coarctation of the aorta and Williams syndrome was noted to have petechiae in cardiology clinic prior to planned surgical intervention. Workup revealed acquired von Willebrand syndrome secondary to the high shear force generated by the aortic coarctation. He was treated with intra- and postoperative Humate P; there were no postoperative bleeding complications. His acquired von Willebrand syndrome resolved postoperatively.

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