Despite right-to-left shunt, not all patients with so-called cyanotic congenital heart disease (CHD) are cyanosed at all times. Moreover, despite undisputed clinical utility, cyanosis is unreliable for the detection of arterial desaturation. Pulse oximetry, on the other hand, provides a much easier, reliable, and accurate method for detecting arterial desaturation. For optimal detection, therefore, it is perhaps sensible to replace cyanosis with pulse oximetry-based detection of arterial desaturation in all cases with suspected CHD.

The hemodynamics of anomalous origin of the pulmonary artery (PA) from the aorta is challenging. Different sources of blood supply to the lungs lead to a unique state of differential flow, pressure, and pulmonary vascular resistance in each lung. The decision for surgical reimplantation of the anomalous PA during infancy is easy. The assessment of operability beyond infancy, however, is perplexing. In this report, we describe stepwise multimodal hemodynamic evaluation and successful surgical management in a 15-year-old boy with an isolated anomalous origin of the right PA from the aorta. We also report 5-year hemodynamic data confirming sustained benefit over the long term, thus providing much-needed clinical validation of often cited Poiseuille’s and Ohm’s laws.

Despite optimal medical management, an 8-year-old boy had persistent pleural drainage following total cavopulmonary connection. Detailed evaluation, including computed tomography angiography, confirmed obstruction at the lower end of the circuit due to infolding of the polytetrafluoroethylene graft. Balloon dilation of the obstruction resulted in prompt resolution of pleural effusion with sustained relief at 1-year follow-up. The case demonstrates the importance of careful assessment in the diagnosis and successful nonsurgical management of an unusual cause of obstruction in the Fontan circuit.

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