Transcatheter closure of ruptured sinus of Valsalva: A report of first five cases in Tehran Heart Center, Iran

Abstract

Ruptured sinus of Valsalva (RSOV) is a potentially lethal lesion requiring prompt intervention. Transcatheter Closure of RSOV is a novel alternative to the standard treatment, which is open-heart surgery. In this case series, we have reported our center's first five cases of RSOV patients undergoing Transcatheter Closure.

INTRODUCTION

Aneurysms of the sinus of Valsalva (SOVA) affect 0.09% of the population.^1,2 It mainly occurs when the proximal aortic flow is high, accompanied by a congenital or acquired weakness in the aortic wall. The ruptured sinus of Valsalva (RSOV) occurs when the aneurysmal sinus ruptures into an adjacent chamber. Due to the proximity of the right, left, and noncoronary sinuses to the interventricular septum, left ventricular free wall, and interatrial septum, respectively, rupture roots can be detected based on the origin of SOVA.³

Most SOVAs are asymptomatic before they rupture into adjacent structures. They usually arise in the right coronary sinus (RCS).^4,5 As a result of the rupture, heart failure progresses, and if left untreated, patients with RSOV have a poor prognosis and high mortality rates. In these cases, prompt intervention is necessary to close the ruptured fistula.^2,6

In recent years, the Transcatheter Closure (TCC) of RSOV has emerged as a viable, less invasive alternative to surgery.⁷ However, clinical researches regarding the TCC of RSOV are only limited to small-scale studies.⁸ Thus, further studies are required for a complete delineation of patient selection, imaging modalities, device selection, and potential complications of this intervention.

In this case series, we report five cases of RSOV that underwent transcatheter closure in our center. To the best of our knowledge, this is the first case series reporting patients undergoing this procedure in Iran.

CASE REPORTS

Five cases of SVA were treated by transcatheter intervention at Tehran Heart Center between 2014 and 2018. A summary of patient characteristics and their outcomes is demonstrated in Table 1. All patients were male, aged 24–35 years old.

TABLE 1 Summary of first five patients undergoing Transcatheter Closure of Ruptured Sinus of Valsalva in Tehran Heart Center.

Patient	Age	Gender	Symptoms	Associated heart lesions	Defect Site	Defect dimensions	Device brand	Device size	Immediate Residual Shunt	FU duration (months)
1	33	M	Dyspnea- Palpation	BAV	RCS to RA	10	Cardiofix DO	18/16	Present- resolved in 1 month	1 m
2	31	M	Palpation	Small VSD	RCS to RVOT	8/2	Occlutech PDAO	14/16	None	52 m
3	33	M	Dyspnea- Palpation	BAV	RCS to RA	11/6	Occlutech PDAO	12/15	None	42 m
4	24	M	None	None	RCS to RVOT	3.7/8.6	Occlutech PDAO	10/12	None	26 m
5	35	M	None	Surgical VSD closure and AVR	RCS to RA	5	Occlutech PDAO	4/6	None	31 m

Abbreviations: AR, Aortic Regurgitation; AVR, Aortic Valve Replacement; BAV, Bicuspid aortic valve; DO, Duct Occluder; FU, Follow-Up; LCS, Left Coronary sinus; M, Male Gender; PDAO, Patent Ductus Arteriosus Occluder; RA, Right Atrium; RCS, Right Coronary sinus; RV, Right Ventricle; RVOT, Right Ventricle Outflow Tract; VSD, Ventricular Septal Defect.

Three of these patients were symptomatic and diagnosed with RSOV in echocardiographic evaluations. Their symptoms were as follows: Palpation (three patients) and dyspnea (two patients with New York Heart Association (NYHA) Functional Class II). Two other patients were diagnosed during a routine checkup and mentioned no relevant symptoms. One of the patients previously reported⁹ had a history of Surgical VSD closure and aortic valve replacement due to severe AI (Aortic Insufficiency), two had Bicuspid Aortic Valves, and one had a small VSD.

All patients underwent TEE to assess the exact size and location of the defect mentioned in Tables 1 and 2. Preprocedural CT angiography of the aorta was performed only in one patient with a history of aortic valve replacement as a follow-up for the previous surgery. The right coronary sinus aneurysm ruptured to the right atrium in three patients (Figure 1; Video S1) and the right ventricle outflow tract (RVOT) in the other two patients (Figure 2; Video S2). All patients had only one shunt site from the aorta.

TABLE 2 Transesophageal echocardiography characteristics of patients undergoing Transcatheter Closure of Ruptured Sinus of Valsalva before the intervention.

Patient	Left Atrium	Aortic Root	LVD d	LVD s	Global EF	IVS	PWd	RVD d	MV	TV	AV	PV
1	38	30	60	42	50	8	9	36	Mild MR	Normal	BAV, Trivial AI	Trivial PI
2	36	34	48	28	55–60	8	8	31	Mild MR	Normal	Trivial AI	Normal
3	44	38	56	42	50	10	9	32	Mild MR	Normal	BAV, No AI	Mild PI
4	39	36	58	39	45–50	9	9	25	Mild MR	Trivial TR	Normal	Mild PI
5	42	43	58	42	45	9	8	34	Normal	Trivial TR	Normally functioning mechanical bileaflet prosthesis	Trivial PI

Abbreviations: AI, Aortic Insufficiency; AV, Aortic Valve; BAV, Bicuspid aortic valve; IVS, InterVentricular Septum; LVD d, Left Ventricle diastolic diameter; LVD s, Left Ventricle systolic diameter; MR, Mitral Regurgitation; MV, Mitral Valve; PI, Pulmonary Insufficiency; PV, Pulmonary Valve; PW d, Pulmonary capillary Wedge Pressure; RVD d, Right Ventricle diastolic diameter; TV, Tricuspid Valve; TR, Tricuspid Regurgitation.

FIGURE 1. Ruptured right coronary sinus to the right atrium. (A, B) Transesophageal echocardiography in mid-esophageal short axis view demonstrates wind sock of the ruptured right coronary sinus to the right atrium (arrow) adjacent to the tricuspid valve with a left to right shunt in color doppler flow study (arrow). (C) Aortic root injection in left anterior oblique view demonstrating flow from the aorta filling right atrium and right ventricle (arrow). (D) The patent ductus arterioles (PDA) occluder device occluded the aforementioned flow (arrow). (E) Transthoracic echocardiography in parasternal short axis view after rupture occlusion demonstrating PDA occluder device (arrow) at the appropriate site. LA, Left atrium; RA, Right atrium; RV, Right ventricle.

FIGURE 2. Ruptured right coronary sinus to distal of the right outflow tract. (A, B) Transesophageal echocardiography in mid-esophageal short axis view demonstrates wind sock of the ruptured right coronary sinus to distal of the right outflow tract (arrow) with a left to right shunt in color doppler flow study (arrow). (C) Aortic root injection in the left lateral view shows a flow from the aorta filling the right outflow tract (arrow). (D) The patent ductus arteriousus (PDA) occluder device occluded the aforementioned flow (arrow). (E, F) Transthoracic echocardiography in parasternal short axis view after ruptured sinus Valsalva occlusion demonstrated the PDA occluder device at the appropriate site (arrow). LA, Left atrium; RA, Right atrium; RV, Right ventricle.

PROCEDURE

Local anesthesia was induced in all patients to conduct the intervention procedure. Venus and arterial accesses were obtained from the femoral vessels, followed by the administration of Heparin and Cefazolin through the venous access. TEE was simultaneously employed during interventions to re-assess the diameter of the ruptured aneurysms (Figures 1 and 2A,B).

Angiography of the aortic root was conducted in the next step utilizing a left anterior oblique view with a cranial tilt projection and a left lateral view in patients with aortic shunts to the RA and RVOT, respectively (Figures 1 and 2C). The dimensions of the aortic and atrial/ventricular rupture roots obtained from the angiography were compared with TEE, and the larger dimensions were considered for device size selection. A compatible device size was chosen according to device type and rupture size, ranging from 4/6 to 18/12. Occlutech PDA Occluders were employed in four cases, and Cardiofix Ductal Occluder in one case was used in one of the cases.

A coronary Catheter (Judkins right coronary catheter) was passed through the aortic rupture site utilizing an angled tip guide wire. The device delivery sheath was implanted through the shunt site across the arteriovenous loop from the vena cava through the femoral vein. The device was subsequently introduced through the delivery sheath. The aortic side of the device was fixed on the orifice of the rupture site in the Valsalva sinus, blocking the shunt. After that, the blockade was confirmed by the TEE, heart chamber side of the device was installed without disrupting the blockade of the aortic shunt orifice by employing slight traction. Lastly, the presence of residual shunt, AR, TR, or coronary artery stenosis was assessed by performing a control angiography and TEE before the final release of the device within the defect (Figures 1 and 2D,E).

All procedures were performed successfully (100% success rate). Dual antiplatelet treatment was started for the patients with Asprin and Clopidogrel. In a 6- to 52-month follow-up, no patient developed new symptoms, and no residual shunt was detected. Apart from a trivial AI in one of the patients, other follow-up echocardiograms were unremarkable.

DISCUSSION

A SOVA can accompany several symptoms or be detected as an incidental finding without any symptoms (as in 2 of our cases). However, as soon as it ruptures, there could be lethal complications if left untreated. Thus, prompt intervention is required following the diagnosis of this lesion. The TCC of RSOV can be essential in providing a treatment alternative without needing open-heart surgery. However, due to the limited studies regarding this procedure, the patient selection is usually limited to low-risk cases with only simple and small lesions and without any other heart defects.

The first report on the TCC of RSOV was by Cullen et al. in 1994.¹⁰ Several other experiences have been reported since then, utilizing different approaches, techniques, and devices.^7,8 While the arterial approach was mainly used in the first TCC cases, the venous approach is currently preferred due to its easier access and increased maneuverability.¹¹ Rashkind umbrella was the first device utilized in the TCC of RSOV.¹⁰ However, several other device types by various manufacturers have been employed, such as Amplatzer Ductal occluders, PDA occluders, Muscular VSD Occluders, Septal occluders, and Coils.¹¹ PDA occluders (the device type used in our cases) are the most common device utilized in TCC of RSOV, although the availability of devices and familiarity of interventionists significantly affect the device selection.¹²

Regarding various imaging modalities, Transesophageal echocardiography is one of the essential modalities in treating these patients. TEE was also employed during the intervention to provide live visualization of the device, defect location, and measurements.

Routine follow-ups can be essential after the intervention to detect possible rare complications. Residual shunts are the most common complication of TCC of RSOV.⁷ New onset or progression of existing aortic regurgitation can occur following transcatheter device implantation. Other rare complications include device embolization, coronary artery compression, hemolysis, and cardiac conduction disturbances.^11,13–15

CONCLUSION

RSOV is an uncommon but potentially lethal disease requiring rapid intervention. TCC of RSOV can be a safe and effective treatment reducing the need for surgical intervention, although much more research is needed in order to standardize various aspects of this treatment.

AUTHOR CONTRIBUTIONS

Aryan Ayati: Writing – original draft; writing – review and editing. Neda Toofaninezhad: Data curation; methodology. Ali Hosseinsabet: Investigation; methodology; project administration; supervision; writing – review and editing. Kaveh Hosseini: Investigation; supervision; writing – review and editing. Alimohammad Hajizeinali: Conceptualization; investigation; validation.

ACKNOWLEDGMENTS

None.

CONFLICT OF INTEREST STATEMENT

All authors declare that there were no competing interests.

DATA AVAILABILITY STATEMENT

All data associated with the article is available if required.

CONSENT FOR PUBLICATION

All authors have consent for publication of the article.

CONSENT

Written informed consent was obtained from the patients to publish this report in accordance with the journal's patient consent policy.