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. AN 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. AU 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 cave with significant obstruction. Conclusion: In the presence of right atrial isomerism and total anomalous pulmonary venous connection, there may be an intrapulmonary venous connection that may be obstructed. Anastomosing the pulmonary venous confluence to the atrium may be difficult because of hypoplasia of the pryvenous confluence. (CREST 1996; 110:1363-66)
Key words: right atrial isomerism; scimitar syndrome; total anomalous pulmonary venous connection
The clinical manifestations of patients with total anomalous pulmonary venous connection are determined by the presence of pulmonary venous obstruction, the amount of pulmonary blood flow, the size of the atrial septal defect, and associated anomalies. Echocardiography is considered the best means to examine patients with total anomalous pulmonary venous connection. However, for the supracardiac type of total anomalous pulmonary venous connection with a vertical vein buried m the pulmonary parenchyma, imaging the pulmonary venous pathway was generally unsuccessful with echocardiography. We describe experience with four patients having the supracardiac type of total anomalous pulmonary venous connection with a pulmonary venous pathway that traversed pulmonary parenchyma and joined the superior vena cave with significant obstruction.
PATIENTS AND METHODS
During a 9-year period from 1985 to 1994 in National Taiwan University Hospital, a diagnosis of total anomalous pulmonary venous connection was made m 25 patients with right atrial isomerism by review of records from the Pediatric, Surgical, and Pathology Departments. Four patients had an intrapulmonary segment of pulmonary venous pathway. Two were male and two were female with ages ranging from 1 week to 4 years. All four patients underwent echocardiography, cardiac catheterization, and angiography. One patient underwent MRI of the cardiovascular system. Autopsy information was available for all four patients.
Cyanosis with tachypnea was found in all four patients. All had right atrial isomerism, a diagnosis based on juxtaposition of abdominal great vessels on echocardiogram, bilateral morphologic right bronchi on high-kilovoltage-filtered chest film or MRI.[3,4] Their cardiovascular anomalies are summarized in Table 1. On plain chest x-ray films, an abnormal shadow resembling scimitar syndrome at the upper field of the right lung was seen in two (Fig 1). In echocardiographic examinations, a horizontal pulmonary venous confluence was identified behind a common atrium in all patients, but no vertical vein connecting to the pulmonary venous confluence was identified. On a high parasternal view, an abnormal vessel joining to the superior vena cave with a narrow junction, from which a turbulent flow emitted, was imaged in all four patients. This vessel can be a distal segment of a vertical vein. In the angiographic studies, a pulmonary arterial angiogram or right ventricular angiogram failed to delineate the anomalous pulmonary venous pathway in three. On a pulmonary venogram, an intrapulmonary segment of vertical vein traversing the right pulmonary parenchyma with obstruction was clearly shown in two patients (cases 1 and 2 [Fig 2] ). This pathway was well demonstrated by magnetic resonance imaging on coronal sections in one patient (case 3 [Fig 3]). The diagnosis of the intrapulmonary segment was not made during life but in the postmortem autopsy in the remaining one (case 4).
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