Abstract
Abstract Despite technological advancements, diagnosis of total anomalous pulmonary venous connection (TAPVC) can be challenging in neonates and infants, particularly in asymptomatic cases. We report a case of a 22-month-old boy who presented to the Emergency department with a history of intermittent fever and cough, initially diagnosed as pneumonia. The presence of an enlarged mediastinal mass was noted in a chest radiograph and was interpreted as a potential malignancy. However, the presence of a supracardiac type of TAPVC with significant right-sided cardiac dilation and a large unobstructed ascending vertical vein was confirmed by echocardiogram. Elective and uneventful surgical correction was performed with subsequent follow-up to confirm cardiac stability and normalization. This case presents the “snowman” or “figure-of-eight” appearance characteristic of TAPVC. Moreover, our patient’s chest radiograph is suggestive of a well-defined, smooth and linear vascular shadow with the presence of a normal lung parenchyma along with increased pulmonary vascularity indicating a left-sided vertical vein in keeping with the supracardiac type of TAPVC. Not commonly reported in previous literature, these unique radiographic findings may hold significant value for pediatricians and imaging specialists as a diagnostic tool for the supracardiac variant of this congenital pathology
Highlights
Identification of a superior mediastinal shadow in a child admitted in the intensive care unit is always of grave concern and warrants urgent attention
Craig et al [4] was the first to classify Total anomalous pulmonary venous connection (TAPVC) into four groups based on the site of drainage of the pulmonary veins (PV): (i) Supracardiac: All the PV meet at the confluence behind the left atrium (LA) and drain via an ascending vertical vein into the innominate vein, superior vena cava (SVC) or the azygous vein
These features resemble a mug shape in a CXR (Figure 2), where the handle represents the ascending vertical vein. This radiographic feature has not been previously described. The novelty of this case lies in the presentation of a new CXR finding which is strongly predictive of a supracardiac TAPVC as a primary differential diagnosis
Summary
Identification of a superior mediastinal shadow in a child admitted in the intensive care unit is always of grave concern and warrants urgent attention. Craig et al [4] was the first to classify TAPVC into four groups based on the site of drainage of the PV: (i) Supracardiac: All the PV meet at the confluence behind the LA and drain via an ascending vertical vein into the innominate vein, superior vena cava (SVC) or the azygous vein This is the most common form and occurs in about 50% of all TAPVC patients; (ii) Cardiac: All the PVs drain directly into the coronary sinus or right atrium (RA). This occurs in about 30% of all TAPVC patients; (iii) Infracardiac: All the PV meet at the confluence and drain via a descending vertical vein into the hepatic or portal vein below the diaphragm. The child is 5 years old and remains in sinus rhythm and good cardiac status
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