Transcatheter secundum atrial septal defect closure using intracardiac echocardiography in adult patient with azygos/hemiazygos continuation of the inferior vena cava

A 53-year-old woman came for the onset of dyspnea. Physical examination revealed a fixed splitting second heart sound. Chest radiography showed prominent pulmonary vasculature. Transthoracic echocardiography (TTE) revealed enlarged right heart cavities and transesophageal echocardiography (TEE) confirmed a 23 × 17 mm secundum Atrial Septal Defect (ASD II), with the anterior rim of 2 mm (Fig. 1-A).

Fig. 1.

– A: Three-dimensional transesophageal echocardiography with multiplanar reconstruction; B–C: angiographic demonstration of the IVC draining into the right atrium, and dilated hemiazygos vein draining directly into the SVC; D: balloon sizing of the defect shows the plane of the atrial septum almost perpendicular to the balloon shaft; E: right atrial angiography after successful deployment of the GSO demonstrates no angiographic evidence of any right-to-left shunting; F: ICE imaging of the GSO device, correctly positioned. Ao: aorta; GSO: Gore Septal Occluder; HEAv: hemiazygos vein; ICE: intracardiac echocardiography; IVC: inferior vena cava; SVC: superior vena cava.

After obtaining informed consent, the patient was admitted to catheter laboratory for percutaneous closure of ASD through the right femoral approach. A 6-Fr sheath was inserted into this vein. Pressure measurements and oximetry for shunt volume (Qp:Qs = 1.4) and pulmonary vascular resistance calculation (PVR = 3 WU) were performed with a Swan-Ganz catheter.

Due to TEE intolerance and the patient's low Body Mass Index (BMI = 17.1 Kg/m²), the intracardiac echocardiography (ICE) was instead inserted via 9-F long venous sheath from the left femoral vein and advanced to attempt entry through the Inferior Vena Cava (IVC) into the right atrium. The patient received intravenous heparin (100 IU/Kg), and a venography was performed immediately after (Fig. 1B–C), because of the abnormal course of the catheter and an azygos/hemiazygos continuation of the left iliac vein, up to the Superior Vena Cava (SVC).

For this double IVC with hemiazygos continuation of the left-side IVC and for a very acute angle of the draining azygos/hemiazygos veins into the SVC, it was not possible to advance the ICE into the right atrium through this venous system. The right femoral vein was therefore catheterised twice: proximally with a 7-F sheath, which was used and replaced with 12-F long sheath for device implantation; distally with 9-F sheath for the ICE study in the right atrium.

The interatrial septal defect was passed through using a superstiff-0.035’ Amplatzer wire and directed toward the left superior pulmonary vein. The dimensions of the ASD were confirmed by the sizing balloon (Fig. 1-D), and compared with the ICE. For the small anterior rim, a 37 mm Gore Cardioform ASD occluder (GSO) was loaded and advanced through the sheath, and the defect was closed using the routine protocol. The position and stability of the device were evaluated on ICE. No post-procedure residual shunt was detected (Fig. 1E–F).

Postoperatively, computed tomography angiography was performed (Fig. 2) and the patient was discharged 2 days later with aspirin (at a 6-month follow-up was asymptomatic).

Fig. 2.

– A: Normal systemic venous system schematic and double IVC with hemiazygos continuation of the IVC schematic. B: MDCT 3-dimensional reconstruction - lateral plane. Note “candy cane” appearance of anomalous venous pathway. C: MDCT 3-dimensional reconstruction showing double IVC with hemiazygos continuation of the left-side IVC - coronal plane; D: Contrast-enhanced CT – axial plane. Ao: aorta; Av: azygos vein; CT: computed tomography; HEAv: hemiazygos vein; IVC: inferior vena cava; MDCT: multidetector computed tomography.

Percutaneous closure of an isolated ASD II has become the first-line treatment in patients with sufficient rim and the procedure is mostly performed through the femoral vein. Standard techniques include the use of balloon-size technique and ICE, the latter as an accepted alternative to the TEE. Congenital anomalies of the deep thoracoabdominal venous system are caused by variations in the development during embryogenesis [1].

Anomaly/interruption of the IVC is a rare entity (prevalence in general population is 0.1–0.6%) [2,3], associated primarily with isomerism syndromes. Few cases with anomalies of IVC and no heterotaxy syndrome are reported [4]. However, our patient had isolated ASD II and double IVC without any other congenital abnormality, and in our case the prevalence is 0.3%.

Usually, the presence of this vascular variation alone does not cause clinical problems, however if found incidentally [5,6], can in some cases become clinically important. As previously published, knowledge of this anatomic arrangement is important in percutaneous cardiopulmonary procedures. Transfemoral, transjugular, transhepatic approach (Table 1), or surgery can be an alternative for closure. The study of septal rims and possible angle of delivery catheter/device with respect to the septum is a crucial point for interventional cardiologists.

Table 1.

Studies evaluating the approach for percutaneous ostium secundum atrial septal defect closure in patients with interrupted Inferior Vena Cava.

Study/Year	DOI/PMID	Diagnosis	Device	Approach/Route	TEE	AGE/SEX	Introducer sheath size
Papa M et al., 2004	https://pubmed.ncbi.nlm.nih.gov/15320573/PMID: 15320573	Undetected A continuation	ASO 16 mm	Right IJV (transjugular)	yes	34 y/M	N/A
Emmel M et al., 2006	https://doi.org/10.1007/s00392-006-0382-y PMID: 16598389	Absent IVC, with A continuation	ASO	Transhepatic access	NA	3 y	9 French
Oliveira EC et al., 2006	https://doi.org/10.1590/s0066–782 × 2006001500019 PMID: 16951839	Absent IVC	ASO 30 mm	Transhepatic access	yes	18 y/F	12 French
Kashour TS et al., 2010	https://doi.org/10.1111/j.1747-0803.2010.00391.x PMID: 21106024	IVC interruption	ASO 12 mm	RFV from IVC through the A continuation and SVC	yes	53 y/F	8 French
Lowry AW et al., 2011	https://doi.org/10.1002/ccd.23041 PMID: 21936042	Left isomerism type, polysplenia, dextrocardia and IVC interruption	ASO 20 mm	RFV, through the A continuation of IVC into the SVC	yes	3 y/F	9 French
Ozbarlas N et al., 2012	https://pubmed.ncbi.nlm.nih.gov/22719163/PMID: 22719163	Heterotaxia syndrome with left atrial isomerism, IVC interruption	ASO 10 mm	Transjugular approach	yes	8 y/M	7 French
Flosdorff P et al., 2013	https://doi.org/10.1007/s00246-012-0307-7 PMID: 22453839	Interrupted IVC with A continuation	ASO 9 mm	Transfemoral closure via A continuation	yes	3 y/M	6 French
Seshagiri RD et al., 2013	https://doi.org/10.1007/s12928-012-0113-4 PMID: 22810920	Interrupted IVC with A continuation	Blockaid ASD occluder 30 mm	Transjugular approach	yes	40 y/F	12 French
Narin N et al., 2014	https://doi.org/10.5114/PWKI.2014.46769 PMID: 25489321	Interrupted IVC with A continuation to SVC	ASO 18 mm	Right IJV	yes	6 y/F	N/A
Sadiq N et al., 2014	https://doi.org/10.4172/2329-6607.1000120	Interrupted IVC	27 mm Occluder device	Transhepatic access	yes	24 y/F	12 French
Yücel İK et al., 2016	https://doi.org/10.5543/tkda.2015.75222 PMID: 27138315	Left atrial isomerism, and interrupted IVC with A continuation.	Ceraflex septal occluder 24 mm	Jugular vein	yes	12 y/F	12 French
Bhargava RA et al., 2017	https://doi.org/10.4103/APC.APC_167_16 PMID: 28566830	Interrupted IVC with A continuation	ASO 22 mm	Transjugular approach	yes	49 y/M	9 French
Yarrabolu TR et al., 2017	https://doi.org/10.4103/0974-2069.197056 PMID: 28163447	Interrupted IVC	ASO 20 mm	Right IJV	yes	4 y/M	10 French
Imai M et al., 2018	https://doi.org/10.1016/j.jcin.2018.01.243 PMID: 29605245	Situs inversus, dextrocardia, and interrupted IVC	Occlutech Figulla Flex II 21-mm	Jugular venous approach	yes	31 y/M	12 French
Ozdemir E et al., 2018	http://www.ijcva.com/text.asp?2018/4/1/15/229256 https://doi.org/10.4103/IJCA.IJCA_6_18	Interrupted IVC with A continuation to SVC	ASO 14 mm	Right IJV	Yes	32 y/F	8 French
Truong QB et al., 2018	https://doi.org/10.1016/j.jccase.2018.05.007 PMID: 30279924	Interrupted IVC with A continuation Interrupted IVC, and A continuation	ASO 26 mm ASO 15 mm	RFV through the A vein up to SVC Transjugular approach	yes yes	49 y/F 15 months	11 French 7 French
Oliveira EC et al., 2019	https://doi.org/10.1017/S1047951119002099 PMID: 31475660	Situs inversus along with dextrocardia, agenesis of the infra-hepatic segment of the IVC, with a communication through the left SVC by the A vein	ASO 19	Left IJV	yes	3 y/M	9 French
Alizade E et al., 2020	https://doi.org/10.14744/AnatolJCardiol.2019.70968 PMID: 32120364	Interrupted IVC with A continuation	ASO 20 mm	Femoral vein approach	yes	37 y/F	12 French
Yerram S et al., 2020	https://doi.org/10.1017/S104795112000178X PMID: 32594956	Severe valvular pulmonary stenosis, situs inversus, dextrocardia, interrupted IVC	Ceraflex septal occluder 36 mm	Left IJV	TTE	20 y/F	14 French
Mansour A et al., 2020	https://doi.org/10.1016/J.JCCASE.2021.02.003 PMID: 34354786	Interrupted IVC with A continuation, destrocardia, situs ambiguous, heterotaxia syndrome, left atrial isomerism	Occlutech Figulla Flex II 27 mm	Left IJV	yes	11 y/M	12 French
Piccinelli E et al., 2021	https://doi.org/10.1007/s00246-021-02708-9 PMID: 34561725	Situs inversus, dextrocardia, and interrupted IVC	Occlutech Figulla Flex II 16.5 mm	Left IJV	yes	5y/F	9 French
Olangunju A et al., 2021	https://doi.org/10.1177/23247096211045255 PMID: 34521236	Situs ambiguous (heterotaxia) abdominal anatomy with a left-sided intact IVC	ASO 28 mm	Transhepatic approach	yes	45 y/F	12 French
Firouzi A et al., 2021	https://doi.org/10.1017/S1047951121000755 PMID: 33658101	IVC injection showed segmental interruption of IVC with continuation to the right atrium via multiple collaterals draining to enlarged hepatic veins	Occluder device 33 mm	Transfemoral approach	yes	50 y/M	12 French
Das S et al., 2021	https://doi.org/10.4103/HEARTVIEWS.HEARTVIEWS_197_20 PMID: 34760057	Interruption of the IVC with A continuation	ASO 16 mm	Right IJV	TTE	6 y/M	9 French
Lopes J et al., 2021	https://pubmed.ncbi.nlm.nih.gov/34609330/PMID: 34609330	Interrupted IVC above the renal veins	ASO 15 m	Right basilica vein	yes	55 y/F	N/A
Kong G et al., 2021	https://doi.org/10.1016/S0735-1097(21)03809-2	Interrupted IVC with A continuation to SVC	ASO 10 mm	Transjugular approach	yes	63 y/F	6 French
Enta Y et al., 2022	https://doi.org/10.1177/2150135120983807 PMID: 33956541	Complete transposition of the great arteries type 3, absence of the hepatic segment of the IVC with A continuation	Occlutech Figulla Flex II 13.5-mm	Left subclavian vein	yes	39 y/M	N/A
Dhulipudi B et al., 2022	https://doi.org/10.4103/apc.apc_228_21 PMID: 36246740	IVC interruption draining into the A vein IVC interruption at the infrahepatic level and draining into the A vein through multiple venovenous collaterals	ASO 28 mm ASO 22 mm	Hepatic vein access Hepatic vein access	yes	9 y/F 4 y/M	10 French 10 French

A: azygos; ASO: Amplatzer Septal Occluder; F: female; IJV: internal jugular vein; IVC: inferior vena cava; M: male; N/A: not available; RFV: right femoral vein; SVC: superior vena cava; TEE: transesophageal echocardiography; TTE: transthoracic echocardiography.

Rarely, transfemoral closure can be possible through the azygos continuation of the interrupted IVC [7,8]; however navigation with large calibre delivery sheaths through the azygous vein may be difficult and result in vessel injury (catastrophic pleural or mediastinal haemorrhage due to a sharp bend of the azygos vein).

Transhepatic access can be properly used in children and slim adults, but may cause higher incidence of complications (retro or intraperitoneal bleeding, injury of the gallbladder, pneumothorax, pleural effusions, and liver abscess or peritonitis) [9].

As described in a few case reports, alternatively the transjugular approach is a potentially safer procedure in adults, due to the larger size of internal jugular vein [10]. However, placing and maintaining the guidewire in the left upper or lower pulmonary veins may be more difficult than the transfemoral route. In transjugular approach, the tip of the delivery system tends to point toward the left ventricular inflow or left atrial appendage and the tip of the catheter may easily fall into the left ventricular cavity before or after the guidewire removal. Complications are air embolism and bleeding (compressing is more difficult).

In the literature, all procedures were performed with TEE monitoring or TTE to prevent injury of the cardiac structures and to ensure the opening of the left-right atrial disc of the device in atrial cavities.

Most atrial defect closures are reported with the Amplatzer devices or similar self-centering devices. To our knowledge, we submit this first report on the closure of an ASD with the GSO device under intracardiac ultrasound guidance, in a patient with double IVC with azygos/hemiazygos continuation. This device, less rigid and more conformable, allowed us to perfectly close the atrial defect, despite the small anterior rim. We released the device with the intracavitary vision, keeping it attached through the retrieval cord, watching the movement and positioning of the device until the last moment. The approach with ICE allowed us to perform the procedure without sedation or an anesthetist's help, in a single session.

In conclusion, a comprehensive, multidisciplinary, and tailored preoperative approach should be applied, to choose the best route, the optimal device, and timing of operation.

The interventional manoeuvres coded for vascular access, materials, and technique, should be carefully re-evaluated in cases of congenital anomalies involving the systemic venous return to the right atrium. These anomalies are usually asymptomatic and incidentally found, and the major clinical significance of their recognition is to prevent misdiagnosis, with implications on invasive procedures and highly feared complications.

Probably in the future more flexible and navigable ICEs could allow all approaches. Based on our experience, intravenous heparin would be best administered only after checking the drainage of both femoral veins.

The management of venous return anomalies to the right atrium associated with congenital anomalies remains challenging and clearly requires expertise.

Declaration of competing interest

The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper and that they have secured informed consent from the patient for publishing this case report.