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The levoatriocardinal vein: morphology and echocardiographic identification of the pulmonary-systemic connection
H S Bernstein1, P Moore, P Stanger
1Department of Pediatrics, University of California, San Francisco 94143-0214, USA.
Insights
The rare levoatriocardinal vein, a pulmonary-systemic connection, aids decompression in infants with left-sided heart obstructions. Echocardiography offers rapid, noninvasive identification of this vein, crucial for diagnosis and surgical planning.
Area of Science:
- Cardiovascular Medicine
- Pediatric Cardiology
- Medical Imaging
Background:
- The levoatriocardinal vein is a rare pulmonary-systemic connection allowing alternative pulmonary venous blood egress in left-sided obstructive lesions.
- Its rarity has limited comprehensive descriptions of morphology and echocardiographic identification.
- It is hypothesized to arise from persistent embryonic anastomotic channels connecting foregut capillary plexus to cardinal veins.
Purpose of the Study:
- To examine the levoatriocardinal vein, its associated lesions, and its role in pulmonary venous return.
- To evaluate echocardiography as a method for rapid identification of the levoatriocardinal vein.
- To compare echocardiographic findings with angiography and necropsy data.
Main Methods:
- Retrospective review of 13 patients with levoatriocardinal vein at UCSF.
- Comparison of echocardiographic findings with angiography or necropsy.
- Review of 12 previously published case reports.
Main Results:
- Patients presented before age 2 years, mostly under 6 months.
- Hypoplastic left heart syndrome variations were the most common primary defects.
- The levoatriocardinal vein typically originated from the left atrium and drained to the superior vena cava or innominate vein.
Conclusions:
- The levoatriocardinal vein acts as a decompression mechanism for pulmonary venous return in left ventricular inflow obstruction.
- It may form when the atrial septum fails to provide an alternative egress for left atrial blood.
- Echocardiography is a valuable noninvasive tool for identifying this vein, which can be mistaken for other anomalies or act as an occult shunt.
Objectives:
This study considers the array of pulmonary-systemic connections made by the levoatriocardinal vein. The primary and associated lesions that play a role in forming this vein are examined, and echocardiography is discussed as a method for its rapid identification.
Background:
The levoatriocardinal vein is a pulmonary-systemic connection that provides an alternative egress for pulmonary venous blood in left-sided obstructive lesions. It is thought to result from the persistence of anastomotic channels that connect the capillary plexus of the embryonic foregut to the cardinal veins. Only 12 cases of levoatriocardinal vein have been reported since its first description in 1926. A comprehensive description of the morphology and echocardiographic identification of this lesion has been unavailable because of its rarity.
Methods:
A retrospective study was performed in 13 patients with a levoatriocardinal vein from the University of California, San Francisco. Echocardiographic findings were compared with those obtained by angiography or at necropsy. In addition, the details of 12 previously published case reports were reviewed. Age at presentation, primary obstruction to pulmonary venous return, integrity of the atrial septum and origin and drainage of the levoatriocardinal vein were compared.
Results:
Patient age at presentation was < 2 years, with most patients presenting before age 6 months. Variations of the hypoplastic left heart syndrome accounted for the majority of primary defects encountered, although multiple but less severe left-sided lesions were seen. The atrial septum was functionally intact in most patients. The levoatriocardinal vein, defined echocardiographically, originated predominantly from the smooth-walled left atrium and drained to the superior vena cava or innominate vein; however, variations of this pattern existed.
Conclusions:
As a physiologic entity, the levoatriocardinal vein provides a mechanism for decompression of pulmonary venous return primarily in patients with left ventricular inflow obstruction. A levoatriocardinal vein is thought to form when the atrial septum fails to provide an alternate egress for left atrial blood. However, when a septal defect or alternative shunt occurs in conjunction with a levoatriocardinal vein, the clinical presentation may be postponed. Echocardiography provides a rapid, noninvasive modality for identifying the pulmonary-systemic connection, which may masquerade as the vertical vein in anomalous pulmonary venous connection or act as an occult source of left to right shunting in patients undergoing surgery for hypoplastic left heart syndrome.