ANOMALOUS PULMONARY VENOUS PATHWAY TRAVERSING PULMONARY PARENCHYMA - DIAGNOSIS AND IMPLICATION

Study objectives: To describe four patients having total anomalous pul monary venous connection with an intrapulmonary vertical vein, renderi ng difficulty in diagnosis and surgery. Setting: a tertiary referral c enter. Patients and methods: By reviewing medical records, 4 of 25 pat ients with right atrial isomerism and total anomalous pulmonary venous connection were identified to have an intrapulmonary vertical vein. A ll four patients underwent echocardiography, catheterization, and angi ography. One underwent MRI. Two underwent open-heart surgery and one r eceived a modified Blalock-Taussig shunt. Results: Right atrial isomer ism was present in all four patients. On chest x-ray films, an abnorma l shadow resembling scimitar syndrome was seen in two patients, Imagin g the vertical vein was unsuccessful with an echocardiogram in all fou r patients. The intrapulmonary course of the vertical vein was depicte d with a pulmonary venogram in two patients and with magnetic resonanc e in one patient. The intrapulmonary segment remained undetected until autopsy in one patient. All four patients died, At autopsy, the pulmo nary venous confluence was hypoplastic in all four venous hearts. The vertical vein was buried in pulmonary parenchyma and drained to superi or vena cava with significant obstruction. Conclusion: In the presence of right atrial isomerism and total anomalous pulmonary venous connec tion, 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.