Introduction
Septal defects are the most prevalent types of CHDs. Nonetheless, the simultaneous presence of atrial septal defect, ventricular septal defect, and patent ductus arteriosus is rarely observed. Transcatheter interventions present a viable alternative to surgical methods. Although multiple defects are generally managed through surgery, when all defects are suitable for an interventional approach, simultaneous transcatheter device closure can be performed. Reference Narin, Pamukcu and Baykan1–Reference Tripathi8 However, reports on simultanous closure of atrial, ventricular septal defect, and patent ductus arteriosus are scarce. Reference Tripathi8
Here, we present a case of a successful closure of both septal defects and ductus arteriosus during the same procedure.
Case report
A 3-year and 5 months old girl weighing 15.4 kg was referred for recent onset tiredness and breathlessness. On examination, she had a dynamic precordium with normal heart sounds and a harsh pansystolic murmur at the lower left sternal edge and apex. There was no hepatomegaly. Twelve-lead electrocardiogram showed normal sinus rhythm with a superior axis. There was RSR pattern in V1 and juvenile T waves in leads V1–V3. Transthoracic echocardiogram showed a 7 mm diameter secundum atrial septal defect (Figure 1 b), a 4 mm diameter perimembranous ventricular septal defect with left to right shunt and a peak gradient up to 70 mmHg (Figure 1 a), and a small patent ductus arteriosus measuring 2 mm (Figure 1 c). There was dilatation of left heart cavities due to the ventricular septal defect and to the patent ductus arteriosus, with good biventricular function. During subsequent multidisciplinary discussion, it was not possible to reach a consensus regarding which defect(s) to close. However, transcatheter intervention was decided to investigate the haemodynamic consequences of the CHDs and treat the most relevant defect(s). The patient’s parents were informed and consented for transcatheter closure of the three CHDs.
Echocardiographic images of the 3 CHDs with a) transthoracic echocardiography in 4-chamber view showing a 4 mm diameter perimembranous ventricular septal defect with color Doppler flow (arrow) b) transesophageal echocardiography showing 2D and color Doppler flow of a 7 mm diameter secundum atrial septal defect (arrow) and c) transthoracic echocardiography in suprasternal short axis view showing color Doppler flow of a 2 mm diameter patent ductus arteriosus (arrow).
Under general anaesthesia, using ultrasound guidance, two 5-French short sheaths were inserted in the right femoral vein and artery. The pulmonary artery pressure was normal with a mean of 11 mmHg. Under transoesophageal echocardiography guidance, the perimembranous ventricular septal defect was measured at maximal diameter of 7 mm, partially covered by tricuspid valve tissue. It was then decided to close this one first. A 5-French multipurpose catheter was used to cross it, using retrograde approach. After the multipurpose catheter was placed at the apex of the right ventricle, a 0.035 hydrophilic wire (Terumo, Tokyo, Japan) was advanced, and the multipurpose catheter was removed. The 5-French arterial short sheath was then removed over the wire, and a 5-French-55 cm long Cook Flexor sheath (Bloomington, Indiana, USA) was advanced over the Terumo wire into the right ventricle. A 10/7 mm Amplatzer Vascular Plug II (Abbott, Chicago, Illinois, USA) was then advanced through the long sheath and deployed across the defect under echocardiography and fluoroscopy guidance. Post-deployment echocardiogram showed a tiny residual shunt with peak velocity of 4 m/s. Following that, an aortic angiogram was performed using a 4F pigtail catheter to visualise the PDA, which was small to moderate, favourable for retrograde transcatheter closure, measuring around 2 mm diameter and 10 mm length. A 4/4 mm Amplatzer Duct Occluder II (Abbott, Chicago, Illinois, USA) was then advanced through a 4-French Amplatzer TorqVue (Abbott, Chicago, Illinois, USA) and deployed across the PDA under fluoroscopy guidance. Post-deployment aortic angiogram showed no residual shunt.
Finally, by transoesophageal echocardiography, the atrial septal defect was measured at 10 mm diameter, with adequate rims for transcatheter closure. The defect was then crossed with the multipurpose catheter which was positioned into the left upper pulmonary vein. An Amplatz Super Stiff short taper exchange wire (Abbott, Chicago, Illinois, USA) was then advanced over the multipurpose catheter that was subsequently removed. After removing the 5-French short sheath in the femoral vein, an 8-French Occlutech delivery system (Occlutech Inc., Helsingborg, Sweden) was advanced over the Super Stiff wire. A 12 mm Figulla Flex Occluder (Occlutech Inc., Helsingborg, Sweden) was then successfully implanted in the atrial septal defect and released, with no residual shunt on echocardiogram.
The total fluoroscopy time was 19.4 minutes, and total radiation dose was 175cGycm2. Given the suspicion of a small filamentous thrombus attached to the Amplatzer Vascular Plug II, the patient underwent intravenous heparin administration for 24 hours and was then discharged on Rivaroxaban. However, comparison with pre-procedure echocardiogram demonstrated that this was an accessory chorda, and rivaroxaban was replaced by aspirin at 5 mg/Kg once a day. At 3 months post-procedure, the patient was asymptomatic and transthoracic echocardiogram showed a tiny residual ventricular septal defect, with no other residual shunts (Figure 2).
Discussion
In our case, the cumulative effect of all three defects lead to breathlessness, consistent with a large left-to-right shunt and left heart dilatation, due to the patent ductus arteriosus and the ventricular septal defect, whereas the atrial septal defect was causing right atrial dilatation. Although there was no firm plan to close the three CHDs simultaneously, this was not excluded. After transcatheter closure of the more complex perimembranous ventricular septal defect, the two other CHDs were easily occluded, relieving all symptoms.
Addressing multiple defects in one session is potentially challenging in children. Several simultaneous procedures such as balloon angioplasty or valvuloplasty were reported alongside atrial septal, ventricular septal, and patent ductus arteriosus closure. Reference Shu M.Q. and Hu2–Reference Ho, Fu and Jan4 Interestingly, we found only four reported cases of combined atrial and ventricular septal defect transcatheter closures, including three infants, Reference Narin, Pamukcu and Baykan1,Reference Kumar, Banerjee and Aggarwal5,Reference Basappa, Rao and Srinidhi6 and one adult. Reference Iyisoy, Demirkol and Celik7 Narin et al reported atrial septal and ventricular septal device closure in a 4-month-old child using 9 mm Amplatzer Septal Occluder (Abbott, Chicago, Illinois, USA) and a 4/4 mm Amplatzer Duct Occluder II. Reference Narin, Pamukcu and Baykan1 . Ho et al reported successful closure of atrial septal defect and patent ductus arteriosus in two children. Reference Ho, Fu and Jan4
Song et al analysed transcatheter closure of compound defects in 36 patients, including 11 combined ventricular septal defect and patent ductus arteriosus closure, 8 combined ventricular septal and atrial septal defect device closure, 7 atrial septal defect and pulmonary stenosis, and 1 patient with atrial septal defect and mitral stenosis. Reference Shu M.Q. and Hu2 Finally, we only found one case reporting simultaneous closure of all three defects in a patient with Down syndrome. Reference Tripathi8
Conclusion
To our knowledge, this is only the second successful simultaneous closure of atrial, ventricular septal defect, and patent ductus arteriosus. Although this is feasible and effective in our experience, more studies are needed on the feasibility of transcatheter treatment in patients with this rare condition.
Postprocedure transthoracic echocardiography in parasternal short axis and in 4-chamber view showing the patent ductus arteriosus device (a), and the atrial septal (gray arrow) and ventricular septal defect (white arrow) devices (b).
Supplementary material
The supplementary material for this article can be found at https://doi.org/10.1017/S1047951124036643.
Competing interests
Other authors have no conflict of interest.
Statements and declarations
Alain Fraisse is consultant and proctor for Occlutech Inc., ICardiology and Abbott Inc.
