Fig. 32.1
Example of postsurgical muscular VSD closure. Transesophageal echocardiography of a postsurgical muscular VSD in a 73-year-old male after recurrent aortic valve surgery and myectomy (Qp/Qs ratio 2, evidence of pulmonary hypertension, (a) color Doppler long axis and (b) short axis view visualizing the defect). A maximum defect diameter of 7 mm was measured by echo and angiography, and direct retrograde closure could be achieved with an Amplatzer™ Muscular VSD Occluder 10 mm without residual shunting (c: long axis view, d: short axis view with implanted occluder). LA = left atrium, LV = left ventricle, VSD = ventricular septal defect, RV = right ventricle, AV = aortic valve
Baffle leaks may be present after repair of anomalous pulmonary venous drainage and atrial switch operation. While obstruction of systemic or pulmonary venous return after the Mustard or Senning operation has been reported in up to 16 % of patients, baffle leaks in this setting were found in approximately 10 %. Both lesions may be present in combination [1]. Shunt direction and shunt portion depend on defect size and hemodynamics determined by ventricular filling characteristics and associated pathologies. Additional obstructions have particular impact and may cause right-to-left shunt. These patients are at risk for paradoxical embolic events or may be cyanotic and present with secondary erythrocytosis, but the latter findings are rare. Given the increased risk of thromboembolism after permanent pacemaker implantation, every patient after atrial switch operation should meticulously be examined with echocardiography and catheterization because smaller leakages may easily be missed. Defects can virtually occur at all sites of central venous return [2], but are more often located at superior or inferior caval connection [3]. They can however also be found in the central part of the baffle, where posteriorly the pulmonary veins may be accessible and anteriorly the adjacency to the AV valves has to be considered. Reoperation is associated with higher risk and mortality; hence, interventional therapy advanced to primary treatment option in most cases.
32.2 Clinical Scenario
Residual defects may be diagnosed in asymptomatic patients during routine follow-up visits. Patients may present with symptoms such as reduced exercise capacity, shortness of breath, and arrhythmias in case of significant left-to-right shunt with ventricular volume overload and eventually pulmonary hypertension. Symptoms at late stages may include signs of right heart failure. Patients with right-to-left shunt may present with cyanosis and/or paradoxical embolism.
32.3 Indications and Patient Selection for Defect Closure
Patients with symptoms related to residual shunt.
Asymptomatic patients with significant left-to-right shunt defined by signs of volume overload with enlargement of the ventricles (LV enlargement in defects on ventricular level, RV enlargement in defects on atrial level) or shunt ratio (Qp/Qs) >1.5
Asymptomatic patients with elevated pulmonary pressure (see Chap. 34 for specific considerations when severe pulmonary hypertension precludes defect closure)
Otherwise unexplained stroke or other systemic embolism, likely due to paradoxical embolism
Cyanosis not caused by pulmonary hypertension (residual ASDs with specific anatomic features causing right-to-left shunt, baffle leaks in combination with baffle obstruction)
Baffle leaks in patients with indication for pacemaker implantation
32.4 Treatment Options
For most residual leaks after surgical ASD or VSD closure, self-centering double-disk devices or their derivatives (e.g., AmplatzerTM, St. Jude Medical Inc. MN, USA) are suitable. For residual ASDs, ASD occluders will be the devices of choice, while VSD and PDA occluders as well as vascular plugs may be chosen for residual VSDs depending on the specific anatomy. In some cases with long or tortuous tunnels or aneurysm formation after VSD closure, nitinol spiral systems may have an advantage over the more rigid meshed nitinol devices (e.g., Nit-Occlud®, pfm medical ag Köln, Germany). Multiple baffle leaks or leaks with concomitant obstructive lesions can be treated with covered stents such as covered CP StentsTM (NuMed Inc. NY, USA).
32.5 Pre-procedural Imaging
Most appropriate information can be gained from transesophageal echocardiography. In residual ASDs native size of the defects, rims, and proximity to atrial wall, veins, and valves can easily be assessed. Residual VSDs should be addressed in terms of tunnel configuration (e.g., funnel shaped), maximum diameter on left/right ventricular side, distance to the valves, and accessibility of the defect from right/left ventricle.
The arcuated course of baffles accounts for difficulties in uncovering and defining the location of leaks, which can also be missed by angiography or MRI. Color Doppler may detect even small defects and is more sensitive than angiography. 3D echocardiography may help to understand the orientation of the defect, because this can be – as mentioned before – very variable. In some patients, bubble studies help to understand the course of shunt defects and shunt direction. MRI and MSCT may be particularly helpful for the evaluation of baffle anatomy and venous connections. MRI allows calculation of ventricular volume overload and shunt flow.
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