Study objectives: To describe four patients having total anomalous pulmonary venous connection with an intrapulmonary vertical vein, rendering difficulty in diagnosis and surgery.
Setting: a tertiary referral center.
Patients and methods: By reviewing medical records, 4 of 25 patients with right atrial isomerism and total anomalous pulmonary venous connection were identified to have an intrapulmonary vertical vein. All four patients underwent echocardiography, catheterization, and angiography. One underwent MRI. Two underwent open-heart surgery and one received a modified Blalock-Taussig shunt.
Results: Right atrial isomerism was present in all four patients. On chest x-ray films, an abnormal shadow resembling scimitar syndrome was seen in two patients. Imaging the vertical vein was unsuccessful with an echocardiogram in all four patients. The intrapulmonary course of the vertical vein was depicted with a pulmonary venogram in two patients and with magnetic resonance in one patient. The intrapulmonary segment remained undetected until autopsy in one patient. All four patients died. At autopsy, the pulmonary venous confluence was hypoplastic in all four hearts. The vertical vein was buried in pulmonary parenchyma and drained to superior vena cava with significant obstruction.
Conclusion: In the presence of right atrial isomerism and total anomalous pulmonary venous connection, there may be an intrapulmonary pulmonary venous connection that may be obstructed. Anastomosing the pulmonary venous confluence to the atrium may be difficult because of hypoplasia of the pulmonary venous confluence.