In this case report we describe the delayed diagnosis of a very rare congenital anomaly - isolated partial anomalous pulmonary venous connection. This congenital anomaly should be suspected at any age in the clinical setting of right heart volume overload, especially in the absence of a large atrial septal defect. Tomographic imaging modalities (computed tomography or cardiovascular magnetic resonance) not only allow the comprehensive structural and functional assessment of this anomaly, but also help assess the patient’s suitability for surgical treatment. Surgery is the definitive treatment of a patient with a significant left-to-right shunt due to partial anomalous pulmonary venous connection.
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