Percutaneous Closure of a Ventricular Septal Defect Using Intracardiac Echocardiography

Abstract

Ventricular septal defects (VSD) comprise the most common congenital heart defect at birth. Although a majority of them close spontaneously by adulthood, transcatheter closure has become the preferred strategy for isolated symptomatic defects. Herein, we present a case of percutaneous device closure of a symptomatic muscular VSD with intracardiac echocardiography.

Case report

A 49-year-old female with a known history of congenital heart disease, including a ventricular septal defect (VSD) diagnosed since birth, presented with progressive dyspnea on exertion. Physical examination was unremarkable except for a holosystolic murmur at the left lower sternal border. Transthoracic echocardiogram confirmed a 10mm muscular VSD with significant left-to-right shunt through the defect (peak velocity - 4.5 cm/s; peak gradient - 81 mmHg). Right heart catheterization and shunt run revealed mild step-up in the right ventricle with an oxygen saturation of 81%. 

Coronary angiography demonstrated non-obstructive coronary artery disease.  Left ventriculography in orthogonal projections confirmed VSD with significant angiographic shunting (Figure 1).In the setting of clinical symptoms, and both echocardiographic and angiographic shunting, the decision was made for percutaneous closure of the VSD.

Under conscious sedation, a JR 4 catheter was advanced from the right femoral arterial access across the aortic valve into the left ventricular (LV) cavity and a Glidewire (Terumo) used to cross the VSD into the right ventricle and beyond into the pulmonary artery after systemic intravenous anticoagulation. Intracardiac echocardiography (ICE) using the AcuNav ultrasound catheter (Biosense Webster) confirmed the muscular VSD measuring 10mm (Figure 2), with the wire across the defect.

A 35mm Gooseneck snare (Covidien) was advanced from the right femoral venous access into the pulmonary artery to capture the Glidewire (Figure 3) and externalize it to create an arteriovenous loop across the VSD (Figure 4). An 8 French (Fr) TorqVue delivery sheath (Amplatzer, St. Jude Medical) was then advanced from thevenous access across the VSD into the LV.

 Using ICE imaging, a 10mm VSD occluder device (Amplatzer, St Jude Medical) was advanced, and the left-sided disk deployed and brought back against the left ventricular septum. The sheath was then withdrawn to deploy the right-sided disk within the tunneled track of the VSD (Figure 5). ICE imaging demonstrated no impingement of the aortic or tricuspid valves, with no significant regurgitation.

 Final ventriculogram (Figure 6) and ICE imaging (Figure 7) showed no residual shunting. Limited echocardiogram the following day showed a well-seated VSD occluder device and no residual shunt, and the patient was discharged home without complications. A follow-up study at one month was unchanged, with significant improvement in symptoms.  

Discussion

VSDs account for only 10 percent of congenital heart defects in adults, since many close spontaneously.^1,2 VSDs are of various sizes and locations, may be single or multiple, and in adults, may be complicated by subpulmonary stenosis, pulmonary hypertension, and/or aortic regurgitation, making their clinical presentation, natural history, and treatment quite variable and sometimes challenging. Although VSDs are associated with other congenital heart defects including atrial septal defect (35 percent), patent ductus arteriosus (22 percent), right aortic arch (13 percent), subpulmonic stenosis, and even more complex defects, such as transposition of the great arteries and tetralogy of Fallot, the majority of congenital VSDs in adults present as an isolated defect.³ 

The ventricular septum is a three-dimensional partition of the left and right ventricles, composed of five segments: membranous, muscular (also known as trabecular), infundibular, inlet, and atrioventricular segments, therefore being classified into five types based on anatomical location of the defect. Infundibular VSD (type 1; also referred to as surpacristal, subarterial, subpulmonary, conal, or doubly committed juxta-arterial) results from deficiency in the septum above and anterior to the crista supraventricularis, beneath the aortic and pulmonary valves. Membranous VSD (type 2; also known as conoventricular) results from deficiency of the membranous septum and is the most common type of VSD. Inlet defects (type 3; also referenced as atrioventricular [AV] canal) result from deficiency of the inlet septum, located beneath both mitral and tricuspid valves. Muscular defects (type 4), which account for 5-20% of VSDs, are bordered only by muscle within the trabecular septum, away from the cardiac valves. The least common VSD is the atrioventricular VSD or Gerbode defect, caused by deficiency of the membranous septum separating the left ventricle from the right atrium, causing a left ventricular to right atrial shunt. 

VSDs become symptomatic when: 

• The defect constitutes a physiologically significant shunt; 
• The aortic valve prolapses from shunting through perimembranous or outlet defects, resulting in aortic regurgitation; 
• The associated valves become a source of infective endocarditis; or
• The right ventricular outflow tract develops obstruction due to magnitude of left to right shunting.

Transcatheter device VSD closure is a treatment option for isolated uncomplicated muscular VSDs and certain membranous VSDs, in patients with suitable anatomy. Appropriate anatomy for transcatheter closure includes a VSD location remote from the tricuspid and aortic valves with an adequate rim. Globally, the most widely used device for closure of VSDs is the Amplatzer VSD occluder, a self-expanding, self-centering device made of nitinol wire mesh and two ventricular retention discs. The technical success rate of transcatheter closure of selected muscular and membranous VSDs is high and the mortality rate is low.⁴ In a large series of 848 patients, the technical success rate of the procedure was 98.1%, with an adverse event rate of only 12.1%, of which only 0.9% were categorized as major.⁵ 

Transesophageal echocardiography (TEE) is the imaging modality of choice to guide transcatheter device VSD closure. However, intracardiac echocardiography (ICE) has become increasingly popular because it offers clearer imaging, shorter procedure times, and a reduced radiation dose to the patient, while maintaining comparable financial costs to TEE.⁶

Herein, we present a patient with a symptomatic muscular VSD that was successfully managed using transcatheter device closure with ICE imaging.  

Dr. Jon George can be contacted at: jcgeorgemd@gmail.com.

Disclosure: Dr. George reports he is a consultant for Biosense Webster and Covidien. Dr. Desai reports no conflicts of interest regarding the content herein. 

References

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