Transcatheter therapy for Lutembacher's syndrome: The road less travelled
An 18-year-old male, presented with history of dyspnea on exertion New York Heart Association (NYHA) class II. Physical examination revealed loud first heart sound, wide and fixed split second heart sound, mid diastolic murmur at the apex, and lower left sternal border with grade 4/6 ejection systolic murmur in the left second parasternal region. Electrocardiogram (ECG) showed incomplete right bundle branch block with right atrial enlargement. Chest X-ray findings were increased pulmonary vascular markings with prominent right pulmonary artery. Transthoracic echocardiography (TTE) showed a 27 mm ostium secundum ASD with severe rheumatic MS []. The mitral valve area (MVA) was 0.67 cm2 by planimetry. Color flow mapping revealed a left-to-right shunt across the ASD. As both the ASD and MS appeared suitable for percutaneous treatment, concurrent transcatheter therapy was planned.\nPatient received 300 mg of clopidogrel and 325 mg of aspirin 1 day prior to procedure. After antibiotic prophylaxis, right femoral arterial and venous access was obtained, and 5,000 units of heparin administered. Right and left heart catheterization with oximetry run confirmed a left to right shunt with step up at the atrial level with Qp/Qs of 5.37 as measured by Fick's principle. The mean left atrial pressure and transmitral gradients were relatively low, in spite of significant MS.\nBalloon mitral valvotomy (BMV) was first undertaken. Multiple attempts with standard Inoue technique proved futile with repeated jumping of Inoue balloon back into the right atrium (RA) and right ventricle (RV). Hence, an over -the -wire (OTW) technique was adopted with the available hardware on shelf. The mitral valve (MV) was crossed using a balloon floatation catheter (Swan Ganz) and an Inoue wire was placed directly into the LV. However, an attempt to introduce the Inoue balloon over the Inoue wire failed because of poor support. Modifying OTW technique, we recrossed the MV with a balloon floatation catheter, parking an Inoue wire directly into the left ventricle (LV). With the Inoue wire in situ, the Swan Ganz catheter was removed and a Mullins sheath was introduced into the LV. With adequate support provided by Mullins sheath, the Inoue wire was exchanged for a J tip Amplatz extra stiff wire (ESW). After removing the Mullins sheath, an Inoue balloon was successfully introduced into the LV over the ESW. With Inoue balloon stabilized in the LV cavity, the ESW was changed to Inoue wire for better conformation to the LV anatomy and provide safe support to Inoue balloon during mitral valvotomy []. The MV was successfully dilated using a 26 mm Inoue balloon with abolition of the transmitral gradient and a MVA of 1.8 cm2.\nAfter successful BMV, ASD device closure was undertaken. Based on our experience of device closure of ASDs without balloon sizing under trans-thoracic (TTE) guidance in young patients, we decided to deploy a 30 mm Amplatzer (AGA Medical, Golden Valley, MN) ASD device under TTE guidance. Postprocedure TTE showed a well-deployed device across the ASD. Twelve hours postprocedure TTE showed a malpositioned device, which was confirmed later on transesophageal echocardiography (TEE) []. After arranging for full surgical backup, percutaneous retrieval of malpositioned ASD device was undertaken under cinefluroscopic and TEE guidance. Intentional bevel was created at the end of a 12 French Amplatzer sheath and a 1-cm Amplatz GooseNeck snare (Amplatz, ev3 Inc., Phymouth, MN, USA) was chosen with intention to grasp the microscrew of the right atrial disk of the ASD device. Using alternate orthogonal views to align the snare in the plane of the ASD device, the device was successfully pulled from the RA into the inferior vena cava below the kidneys and retrieved through the femoral sheath [Figure –]. Careful reassessment of ASD diameter under TEE guidance showed a procedural diameter of 30 mm and total interatrial septal length of 52 mm. Final ASD device closure [] was done with a 36 mm Heartr™ atrial septal occluder device (Lifetech Scientific Inc., Shenzhen, China). There were no further postprocedural complications and patient has done excellently with no clinical sequelae at 16 months follow-up.