Sinus Venosus Atrial Septal Defects Workup
- Author: Gary M Satou, MD, FASE; Chief Editor: Howard S Weber, MD, FSCAI more...
General laboratory studies are rarely helpful in sinus venosus atrial septal defect (ASD). However, imaging studies help in the evaluation and diagnosis of suspected sinus venosus ASD.
Findings on chest radiography may include the following:
Prominent right atrium
Prominent main pulmonary artery
Increased heart size and pulmonary vascularity
Echocardiography (ECHO) reveals ASD and most of the pulmonary vein connections in most patients and is the diagnostic modality of choice.
Two-dimensional ECHO with color flow Doppler reveals the position and size of the defect and the presence of anomalous pulmonary venous drainage (in many of these cases). It also helps identify associated anomalies and reveals the left-to-right (or right-to-left) direction of flow and the degree of right ventricular overload.
In children with difficult transthoracic windows, or in older or larger patients, transesophageal echocardiography may be helpful in imaging the defect and pulmonary vein connections. In the current era, cardiac magnetic resonance angiography (MRA)/magnetic resonance imaging (MRI) may be alternatively used to complete the diagnostic information needed prior to surgery.
Features that may be seen with MRI/MRA include the following:
Atrial septal defect size and location are shown.
Excellent delineation of individual pulmonary vein connections can be identified.
Right ventricle enlargement and indexing to body surface area (BSA) is available if helpful.
Flow-quantification may also be performed.
Note the following:
Right ventricular hypertrophy predominates, with a lengthened PR interval and incomplete right bundle branch block secondary to right ventricular dilation (small rSR').
Peaked P waves in lead II may demonstrate right atrial enlargement.
Abnormal P-wave axis (negative in lead AVF) would indicate a displaced sinus node inferiorly (non sinus atrial rhythm).
Cardiac catheterization is usually not required in the preoperative assessment of patients with sinus venosus atrial septal defect, but it may be considered in the following circumstances:
In any child in whom associated lesions are suspected or in whom pulmonary hypertension is suspected, catheterization is performed to measure pulmonary artery pressure and, if pulmonary resistance is elevated, the response to pulmonary vasodilators.
Adults who have the potential for associated coronary atherosclerotic lesions should undergo catheterization to exclude these abnormalities before surgical repair of the sinus venosus atrial septal defect.
Patients with pulmonary hypertension and advanced pulmonary vascular obstructive disease may exhibit histologic changes similar to those seen in pulmonary vascular disease. Specifically, these include intimal and medial hypertrophy and, in more advanced lesions, luminal occlusion.
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