Highlights
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Transcatheter closure of PM-VSD in cc-TGA anatomy is extremely rare and infrequently reported.
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Mal-alignment of the interventricular and interatrial septum and conduction abnormalities are major hurdles in cc-TGA.
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Understanding and modification in technique, like projections, crossing, and multimodality imaging, are a must before undertaking these cases.
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Transcatheter closure of VSD in cc-TGA can be a feasible alternative for physiologic repair in selected cases.
Congenitally corrected transposition of great arteries (cc-TGA) is often accompanied by associated lesions with a wide spectrum of presentations. Traditionally, ventricular septal defects (VSDs) in such patients have been managed surgically with either physiologic repair or, more recently, there is a paradigm shift towards anatomical repair, such as the double switch operation. Percutaneous VSD device closure in cc-TGA physiology is extremely rare, with only isolated case reports in the literature. The present case report highlights a 15-year-old female, previously diagnosed with cc-TGA with moderate right atrio-ventricular valve regurgitation (AVVR), who presented to us with a history of shortness of breath for the last 1 year. The presence of perimembranous VSD with VSD jet directed towards the right atrioventricular (AV) valve, leading to moderate right AVVR, was identified during detailed evaluation. Cardiac catheterization was undertaken for better visualization and hemodynamic assessment, which revealed sub-systemic morphological left ventricle pressures and a device suitable VSD anatomy. Considering physiologic repair, VSD device closure was done in the same setting. The procedure was adapted to account for the altered cardiac anatomy by adjusting the fluoroscopic angles and wire-torquing manoeuvres. A KONAR-Multifunctional Occluder device (MFO) 10 × 8 mm was successfully deployed via an antegrade approach without complications utilizing hemodynamic and echocardiographic guidance, with good follow-up outcomes. Our case underscores the unique challenges of this complex cardiac anatomy and suggests technical modifications for successful percutaneous VSD closure. In conclusion, VSD device closure in atypical scenarios like cc-TGA can be a safe and feasible option for physiologic repair in selected cases.
Clinical findings and modes of presentation in cc-TGA are highly variable, largely based on the presence or absence of associated lesions. Ventricular septal defect (VSD), particularly in the perimembranous location, is frequently observed in association with cc-TGA, accounting for approximately 60% to 80% of cases . Surgical intervention remains the primary treatment modality, as these defects are often accompanied by valvular regurgitation and conduction abnormalities. In recent years, the double switch operation has emerged as a comprehensive solution for anatomical repair; however, it is not routinely performed across all medical centres. Transcatheter device closure of a perimembranous VSD, particularly in the absence of associated lesions, offers a viable physiological repair alternative in selected cases, though it remains less reported in the literature. This presentation outlines a case involving the transcatheter closure of a perimembranous VSD using an MFO VSD occluder in a 15-year-old patient diagnosed with cc-TGA. This case exemplifies the potential challenges associated with technical, electrical, and imaging considerations during the procedure.
Case Report
Presentation: A 15-year-old female, diagnosed with cc-TGA with moderate right AVVR, following incidental detection of murmur at a peripheral facility, was referred to us for further evaluation and management of AVVR. She presented to us with a complaint of shortness of breath on exertion for the last 1 year. Cardiovascular examination revealed the apex beat in the 5th intercostal space at midline with a harsh grade 4 pansystolic murmur, best heard at the lower right sternal border.
Investigations: Except for mild anaemia (Hb level of 11.2 gm/dL), all other relevant blood investigations were within normal range. A chest X-ray indicated mesocardia with cardiomegaly (cardiothoracic ratio = 0.67). A conventional 12-lead ECG showed sinus rhythm with dominant left-sided ventricle forces with a baseline PR interval of 180 msec, with no conduction abnormality, which was not typical of cc-TGA ( Figure 1 ). No Q waves in V1-V3 were observed. Two-dimensional echocardiography with color Doppler confirmed cc-TGA physiology with mesocardia, a dilated left and right-sided atrium, and left-sided systemic ventricle (morphological right ventricle). Interestingly, there was a perimembranous VSD (7 mm) shunting from the left-sided systemic ventricle (morphological right ventricle mRV) to the right-sided subpulmonic ventricle (morphological left ventricle mLV) with early restriction by the left-sided AV valve billowing in VSD, and a peak gradient of 42 mm Hg (underestimated) was noted across the VSD ( Figure 2 ). The VSD jet was seen redirecting toward the right AV valve and was responsible for moderate right AVVR (mitral regurgitation) ( Video 1 ). It was difficult to delineate VSD due to the limited parasternal window. This shunt was best visualized in the right parasternal short-axis view at the 2 o’clock position. After obtaining informed consent from parents, the patient was considered for cardiac catheterization and to assess the feasibility of shunt closure. Fortunately, in our case, there was the absence of a significant left-sided valve (tricuspid valve) abnormality with only mild regurgitation and no conduction defect with good biventricular function, which favored us toward a transcatheter approach.
Preoperative 12-lead ECG showing sinus rhythm with dominant left-sided forces with no conduction abnormalities.
Echocardiography, A short-axis image highlighting perimembranous VSD, which measures 7 mm, B-modified 4 chamber view depicting VSD at the exit site, which measures 5.5 mm, C-showing Atrio-ventricular discordance with VSD directed towards the right-sided AV valve causing moderate regurgitation, d – Four chamber view showing perimembranous location of VSD with restriction by septal tissue (AV-atrio-ventricular, LA-left atrium, RA-right atrium, mLV-morphological left ventricle, mRV-morphological right ventricle, VSD-ventricular septal defect)
Procedure: The procedure was performed under local anesthesia and conscious sedation. Cardiac catheterization suggested a significant systemic to pulmonary shunt with a suitable pulmonary vascular resistance/systemic vascular resistance (PVR/SVR) ratio ( Table 1 ). However, the morphological left ventricle pressures were less than systemic ventricular pressure with an LV/RV ratio of 0.41. Considering age, hemodynamically significant shunt, LV unpreparedness, and device suitable VSD with absence of associated lesions and ventricular dysfunction, we opted for physiological repair after parents’ consent and discussion with the cardiac surgeon.
Table 1
Hemodynamic data
| Pressure (mm Hg) | Saturation | |
|---|---|---|
| Ascending aorta | 112/74(94) | 98.4% |
| Right atrium (RA) | 5 | |
| Pulmonary artery (PA) | 17/6(13) | 88.8% |
| RV ESP/EDP | 107/12 | |
| LV ESP/EDP | 44/7 | |
| Qp:Qs | 2.25:1 | |
| PVRi | 0.82 woodunits.m 2 |
ESP, end-systolic pressure; EDP, end-diastolic pressure; LV, left ventricle; PVRI, pulmonary vascular resistance indexed; RV, right ventricle; Qp, pulmonary blood flow; Qs, systemic blood flow.
Following the catheterization study, VSD device closure was performed in the same sitting. With preprocedure planning, a 5F pigtail catheter (Cordis USA) was advanced through right femoral arterial access and an RV angiogram was performed in right anterior oblique (RAO)-cranial 30° to 20° projection to delineate the VSD, which measures around 7 mm on entry and exit site measures around 5.5 mm in angiography ( Figure 3 ). We modify our technique; instead of the traditional wire and catheter technique, we first made twisting of J tip Terumo wire (Terumo Medical Corporation, Somerset, New Jersey) at the RV apex, make a redundant loop, and then the floppy end of the wire is directed towards the IVS, which then crosses the VSD ( Video 2 ). It is complicated in cc-TGA as the VSD is more remote from the aortic valve compared to d -looped ventricles with normally related great arteries, and due to subaortic right ventricular trabeculations. The wire is snared from the inferior vena cava (IVC) and externalized to create a veno-arterial loop over which the closure device is deployed antegrade ( Figure 4 ).
Angiogram, A- depicting cc-TGA connection(aorta from mRV, Pulmonary artery from mLV), B-AP projection and C- RAO-cranial projection showing(arrow) depicting 7 mm VSD remote from aorta, proximal to pulmonary valve and showing coarse trabeculations in RV (AP-antero-posterior, mLV-morphological left ventricle, mRV-morphological right ventricle, PA-pulmonary artery, VSD-ventricular septal defect).
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