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Percutaneous closure of ruptured noncoronary sinus of Valsalva to right atrium causing severe right heart failure, a case report
G Model
JCCASE-1140; No. of Pages 4 Journal of Cardiology Cases xxx (2019) xxx–xxx
Contents lists available at ScienceDirect
Journal of Cardiology Cases journal homepage: www.elsevier.com/locate/jccase
Case Report
Percutaneous closure of ruptured noncoronary sinus of Valsalva to right atrium causing severe right heart failure, a case report Hesham Abdo Naeim (MD, FASE)*, Lamiaa Khedr (MD), Abeer Mahmoud (SBC), Waleed Saeed (SBC), Elsayed Ali Taha (MBBCH), Reda Abuelatta (MD) Madina Cardiac Center, Madina, Saudi Arabia
A R T I C L E I N F O
A B S T R A C T
Article history: Received 4 May 2019 Received in revised form 9 September 2019 Accepted 25 September 2019
In all young and middle-aged patients presenting with symptoms of acute heart failure and new heart murmurs, sinus of Valsalva aneurysm (SVA) rupture should be considered in the differential diagnosis. Most of SVAs rupture into the right side of the heart. Percutaneous closure is a less invasive alternative to surgery. A 25-year-old man presented with shortness of breath New York Heart Association class III of nine months’ duration with a progressive course. He had a continuous murmur with maximum intensity over the left sternal border and propagated all over the pericardium. Chest radiographs revealed moderate congestion. Transthoracic and transesophageal echocardiograms with 3D imaging revealed a shunt between the ruptured noncoronary SVA and the right atrium. Percutaneous closure decided; the wire passed from superior vena caca through the ruptured sinus to the aorta. The distal disc of the device deployed in the aorta and the proximal disc in the right atrium. The ruptured aneurysm closed with no more flow to the right atrium. The patient was discharged from the hospital after two days. In conclusion, device closure of ruptured coronary sinus to the right atrium is feasible and safe. Surgery should be reserved for patients with failed device closure. © 2019 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.
Keywords: Ruptured Percutaneous Closure Coronary Sinus Right atrium Case report
Introduction Sinus of Valsalva aneurysm (SVA) is a congenital aneurysm of one of the three coronary sinuses. Deficiencies of muscle and elastic fibers in the middle layer generate a weak area, which gradually progresses into an aneurysm [1]. SVA remains asymptomatic until rupture in a cardiac chamber. Rupture of SVA usually happens in the third and fourth decade of life [2]. Men are affected by illness 2–4 times more often than women [2]. SVA occurs mostly in the right coronary sinus, followed by non-coronary sinus, and rarely left coronary sinus [3]. SVA ruptured more frequently in the right ventricle followed by the right atrium (RA), and rarely the left ventricle [4]. Initially, rupture may be silent but later manifests itself as a progressive heart failure according to the size of the
* Corresponding author at: Madina Cardiac Center, Madina, Shoribat PO 1972, Saudi Arabia. E-mail address: [email protected] (H.A. Naeim).
shunt and the draining chamber. We present a case of a big noncoronary SVA ruptured in the RA with significant left to right shunt. Right side dilation and failure manifested in the third decade in this case. Case report A 25-year-old man who was neither diabetic nor hypertensive presented to the emergency room with shortness of breath and New York Heart Association class III of nine months’ duration with progressive course. He had tachypnea, respiratory rate 25 breaths/minute and tachycardia, heart rate 110 beats/minute, blood pressure 95/60 mmHg and no fever, temperature 37 C. Physical examination revealed an engorged jugular vein (8 cm above the Lewis angle) and mild bilateral pitting lower limb edema. There was mild hepatomegaly with mild tenderness. There were no marfanoid features. On auscultation, he had a continuous murmur with maximum intensity over the left sternal border and propagated all over the pericardium. Laboratory
https://doi.org/10.1016/j.jccase.2019.10.005 1878-5409/© 2019 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.
Please cite this article in press as: Naeim HA, et al. Percutaneous closure of ruptured noncoronary sinus of Valsalva to right atrium causing severe right heart failure, a case report. J Cardiol Cases (2019), https://doi.org/10.1016/j.jccase.2019.10.005
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JCCASE-1140; No. of Pages 4 H.A. Naeim et al. / Journal of Cardiology Cases xxx (2019) xxx–xxx
2
investigations showed his hemoglobin was 11.5 g/dL, normal renal function (creatinine 0.8 mg/dL), and normal cardiac enzymes: creatine kinase-MB (CK-MB) 9 ng/mL and troponin-I, 0.01 ng/mL. Chest X-rays revealed mild pulmonary congestion. Transesophageal echocardiography (TEE) with 3D imaging shortaxis view on the aortic root showed normal left and right coronary sinuses, but the noncoronary sinus had aneurysmal tissue with two large and small fenestrations as shown in Fig. 1A and Supplementary Video S1. TEE mid esophageal view 2D with color showed the turbulent color flow from the aortic root through the ruptured coronary sinus to the right atrium (RA) below the tricuspid valve (Fig. 1B). 3D imaging from the right atrial side revealed the clear aneurysmal tissues bulging in the RA, as shown in Fig. 1C and Supplementary Video S2. Transthoracic echocardiogram revealed severe right side dilation with the color flow to the RA and moderate tricuspid regurgitation (Fig. 1D and Supplementary Video S3). Diagnostic cardiac catheterization with aortography confirmed the diagnosis and showed the flow from the aorta to RA (Supplementary Video S4). We decided on percutaneous closure of the ruptured aneurysm. For successful percutaneous closure, the aortic valve should be healthy with no or only mild aortic regurgitation. The procedure was performed in the catheterization room under general anesthesia with TEE guidance. The femoral vein and artery were accessed. The ruptured noncoronary SVAwas measured at the aortic end as well as at the rupture site both on TEE and angiography. The larger diameter at the aortic side was 10 mm. The size of the Amplatzer duct occluder (ADO) selected to be 2 mm more, so, 12 mm device was accepted for closure. The defect was crossed from the aortic side using a 4 F Judkins right coronary catheter and a 0.035 in. angled tip glide wire (Terumo Inc., Tokyo, Japan). The wire was exchanged for a 300-cm long noodle wire (AGA Medical, Plymouth, MN, USA) that was snared with an
Fig. 1.
Amplatzer gooseneck snare (Microvena, White Bear Lake, MN, USA) from the vena cava and exteriorized from the femoral vein. A stable arteriovenous wire loop was established. Amplatzer delivery sheath (AGA Medical) was introduced from the femoral vein and placed into the ascending aorta across the defect (Fig. 2A). ADO 12
mm was inserted through the delivery sheath, and its aortic disk was deployed in the ascending aorta. The whole assembly was pulled back until the aortic drive blocked the aortic end of the SVA (Fig. 2B and Supplementary Videos S5 and S6). The proximal part of the ADO was deployed in the RA side of the defect. After TEE showed no more color flow through the defect, the aortic valve was normal, the tricuspid valve was healthy and there was no evidence of coronary affection, the ADO was released from the delivery cable (Fig. 3B and Supplementary Video S7). Fig. 3C shows the aorta long-axis view post device closure with the disappearance of color flow through the defect ( Supplementary Videos S8–S10). The patient was discharged home after two days. At follow up after six months, there was a dramatic improvement in symptoms, and TTE revealed no residual flow. Discussion Edwards first described the SVA as a deficiency of normal elastic tissue in the media of the aortic sinuses. A blind-ended diverticulum
(A) 3D zoom short axis on AV, three coronary sinuses were seen, the noncoronary sinus had aneurysmal tissues with significant filling defects. (B) Mid-esophageal view showed the flow from ascending aorta to the RA above the TV. (C) 3D zoom showed the aneurysmal tissues of the noncoronary sinus protruding to the RA above the TV. (D) TTE apical four-chamber view showed color flow from the aorta to RA.
Please cite this article in press as: Naeim HA, et al. Percutaneous closure of ruptured noncoronary sinus of Valsalva to right atrium causing severe right heart failure, a case report. J Cardiol Cases (2019), https://doi.org/10.1016/j.jccase.2019.10.005
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JCCASE-1140; No. of Pages 4 H.A. Naeim et al. / Journal of Cardiology Cases xxx (2019) xxx–xxx
Fig. 2.
(A) The wire passed from SVC through the ruptured sinus to aorta. Deployment of the distal disc to aorta in short-axis view (B) and long-axis view (C). (D) 3D zoom showed the aortic disc.
Fig. 3.
(A) Fluoroscopy deployed device. (B) Fluoroscopy released device. (C) Post closure of the fistula, no more flow seen.
formed which gradually increases in size due to high pressure until it ruptures [5]. SVA is accompanied by aortic regurgitation in 30-50% of cases and bicuspid aortic valve in 9% [2]. Operative mortality is between 1.9% and 3.6%, and 15-year survival is around 90% [2]. Complications of percutaneous SVA closure include failure to deployment of the device due to significant size defect, residual shunting that may close at follow up, and procedure-related aortic regurgitation (AR) due to deformity of the cusps related to the device [6]. Kerkar et al. [6] reported 20 cases of percutaneous closure of ruptured SVA; they concluded that transcatheter closure of ruptured SVA was a promising alternative to surgery in selected cases in the immediate and mid-term period. Percutaneous SVA closure should be directly compared with surgery to evaluate its safety and efficacy.
3
Preoperative TEE is essential to decide if the ruptured SVA is amenable for device closure or not. In this case, we want to emphasize the need for careful intraprocedural TEE for accurate measurement of the diameter of the defect both in the aortic side and RA side to determine the size of the device needed for closure. TEE is crucial for the assessment of pre-, intra-, and post-procedure AR. The relation of the device to aortic leaflets and whether the residual AR is due to the deformity of the leaflets because of the device is a significant role of TEE. Also, TEE is essential for assessment of tricuspid valve (TV), the relation of RA part of the device toTV leaflets, and the residual TR.
Please cite this article in press as: Naeim HA, et al. Percutaneous closure of ruptured noncoronary sinus of Valsalva to right atrium causing severe right heart failure, a case report. J Cardiol Cases (2019), https://doi.org/10.1016/j.jccase.2019.10.005
G Model
JCCASE-1140; No. of Pages 4 H.A. Naeim et al. / Journal of Cardiology Cases xxx (2019) xxx–xxx
4
Conclusion Percutaneous closure of isolated ruptured SVA without significant AR is feasible and relatively safe. TEE is crucial for guiding the procedure and detecting any complications.
Appendix A. Supplementary data
[1] Takach TJ, Reul GJ, Duncan JM, Cooley DA, Livesay JJ, Ott DA, et al. Sinus of Valsalva aneurysm or fistula: management and outcome. Ann Thorac Surg 1999;68:1573–7. [2] Weinreich M, Yu PJ, Trost B. Sinus of Valsalva aneurysm: review of the literature and an update on management. Clin Cardiol 2015;38:185–9. [3] Yan F, Abudureheman M, Huo Q. Surgery for sinus of Valsalva aneurysm: 33-year of a single center experience. Chin Med J 2014;127:4066–70. [4] Chang C-C, Chin C-H, Chen M-L, Chen T-H, Lo H-S. Sinus of Valsalva aneurysm with rupturing into the right atrium - a case report and review of the literature. Acta Cardiol Sin 2006;22:96–101. [5] Edwards JE, Burchell HB. The pathological anatomy of deficiencies between the aortic root and the heart, including aortic sinus aneurysms. Thorax 1957;12(2):125–39. [6] Kerkar PG, Lanjewar CP, Mishra N, Nyayadhish P, Mammen I. Transcatheter closure of ruptured sinus of Valsalva aneurysm using the Amplatzer duct occluder: immediate results and mid-term follow-up. Eur Heart J 2010;31:2881–7.
Supplementary material related to this article can be found, in the online version, at doi:https://doi.org/10.1016/j.jccase.2019.10.005. References
Please cite this article in press as: Naeim HA, et al. Percutaneous closure of ruptured noncoronary sinus of Valsalva to right atrium causing severe right heart failure, a case report. J Cardiol Cases (2019), https://doi.org/10.1016/j.jccase.2019.10.005
JCCASE-1140; No. of Pages 4 Journal of Cardiology Cases xxx (2019) xxx–xxx
Contents lists available at ScienceDirect
Journal of Cardiology Cases journal homepage: www.elsevier.com/locate/jccase
Case Report
Percutaneous closure of ruptured noncoronary sinus of Valsalva to right atrium causing severe right heart failure, a case report Hesham Abdo Naeim (MD, FASE)*, Lamiaa Khedr (MD), Abeer Mahmoud (SBC), Waleed Saeed (SBC), Elsayed Ali Taha (MBBCH), Reda Abuelatta (MD) Madina Cardiac Center, Madina, Saudi Arabia
A R T I C L E I N F O
A B S T R A C T
Article history: Received 4 May 2019 Received in revised form 9 September 2019 Accepted 25 September 2019
In all young and middle-aged patients presenting with symptoms of acute heart failure and new heart murmurs, sinus of Valsalva aneurysm (SVA) rupture should be considered in the differential diagnosis. Most of SVAs rupture into the right side of the heart. Percutaneous closure is a less invasive alternative to surgery. A 25-year-old man presented with shortness of breath New York Heart Association class III of nine months’ duration with a progressive course. He had a continuous murmur with maximum intensity over the left sternal border and propagated all over the pericardium. Chest radiographs revealed moderate congestion. Transthoracic and transesophageal echocardiograms with 3D imaging revealed a shunt between the ruptured noncoronary SVA and the right atrium. Percutaneous closure decided; the wire passed from superior vena caca through the ruptured sinus to the aorta. The distal disc of the device deployed in the aorta and the proximal disc in the right atrium. The ruptured aneurysm closed with no more flow to the right atrium. The patient was discharged from the hospital after two days. In conclusion, device closure of ruptured coronary sinus to the right atrium is feasible and safe. Surgery should be reserved for patients with failed device closure.
Keywords: Ruptured Percutaneous Closure Coronary Sinus Right atrium Case report
Introduction Sinus of Valsalva aneurysm (SVA) is a congenital aneurysm of one of the three coronary sinuses. Deficiencies of muscle and elastic fibers in the middle layer generate a weak area, which gradually progresses into an aneurysm [1]. SVA remains asymptomatic until rupture in a cardiac chamber. Rupture of SVA usually happens in the third and fourth decade of life [2]. Men are affected by illness 2–4 times more often than women [2]. SVA occurs mostly in the right coronary sinus, followed by non-coronary sinus, and rarely left coronary sinus [3]. SVA ruptured more frequently in the right ventricle followed by the right atrium (RA), and rarely the left ventricle [4]. Initially, rupture may be silent but later manifests itself as a progressive heart failure according to the size of the
* Corresponding author at: Madina Cardiac Center, Madina, Shoribat PO 1972, Saudi Arabia. E-mail address: [email protected] (H.A. Naeim).
shunt and the draining chamber. We present a case of a big noncoronary SVA ruptured in the RA with significant left to right shunt. Right side dilation and failure manifested in the third decade in this case. Case report A 25-year-old man who was neither diabetic nor hypertensive presented to the emergency room with shortness of breath and New York Heart Association class III of nine months’ duration with progressive course. He had tachypnea, respiratory rate 25 breaths/minute and tachycardia, heart rate 110 beats/minute, blood pressure 95/60 mmHg and no fever, temperature 37 C. Physical examination revealed an engorged jugular vein (8 cm above the Lewis angle) and mild bilateral pitting lower limb edema. There was mild hepatomegaly with mild tenderness. There were no marfanoid features. On auscultation, he had a continuous murmur with maximum intensity over the left sternal border and propagated all over the pericardium. Laboratory
https://doi.org/10.1016/j.jccase.2019.10.005 1878-5409/© 2019 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.
Please cite this article in press as: Naeim HA, et al. Percutaneous closure of ruptured noncoronary sinus of Valsalva to right atrium causing severe right heart failure, a case report. J Cardiol Cases (2019), https://doi.org/10.1016/j.jccase.2019.10.005
G Model
JCCASE-1140; No. of Pages 4 H.A. Naeim et al. / Journal of Cardiology Cases xxx (2019) xxx–xxx
2
investigations showed his hemoglobin was 11.5 g/dL, normal renal function (creatinine 0.8 mg/dL), and normal cardiac enzymes: creatine kinase-MB (CK-MB) 9 ng/mL and troponin-I, 0.01 ng/mL. Chest X-rays revealed mild pulmonary congestion. Transesophageal echocardiography (TEE) with 3D imaging shortaxis view on the aortic root showed normal left and right coronary sinuses, but the noncoronary sinus had aneurysmal tissue with two large and small fenestrations as shown in Fig. 1A and Supplementary Video S1. TEE mid esophageal view 2D with color showed the turbulent color flow from the aortic root through the ruptured coronary sinus to the right atrium (RA) below the tricuspid valve (Fig. 1B). 3D imaging from the right atrial side revealed the clear aneurysmal tissues bulging in the RA, as shown in Fig. 1C and Supplementary Video S2. Transthoracic echocardiogram revealed severe right side dilation with the color flow to the RA and moderate tricuspid regurgitation (Fig. 1D and Supplementary Video S3). Diagnostic cardiac catheterization with aortography confirmed the diagnosis and showed the flow from the aorta to RA (Supplementary Video S4). We decided on percutaneous closure of the ruptured aneurysm. For successful percutaneous closure, the aortic valve should be healthy with no or only mild aortic regurgitation. The procedure was performed in the catheterization room under general anesthesia with TEE guidance. The femoral vein and artery were accessed. The ruptured noncoronary SVAwas measured at the aortic end as well as at the rupture site both on TEE and angiography. The larger diameter at the aortic side was 10 mm. The size of the Amplatzer duct occluder (ADO) selected to be 2 mm more, so, 12 mm device was accepted for closure. The defect was crossed from the aortic side using a 4 F Judkins right coronary catheter and a 0.035 in. angled tip glide wire (Terumo Inc., Tokyo, Japan). The wire was exchanged for a 300-cm long noodle wire (AGA Medical, Plymouth, MN, USA) that was snared with an
Fig. 1.
Amplatzer gooseneck snare (Microvena, White Bear Lake, MN, USA) from the vena cava and exteriorized from the femoral vein. A stable arteriovenous wire loop was established. Amplatzer delivery sheath (AGA Medical) was introduced from the femoral vein and placed into the ascending aorta across the defect (Fig. 2A). ADO 12
mm was inserted through the delivery sheath, and its aortic disk was deployed in the ascending aorta. The whole assembly was pulled back until the aortic drive blocked the aortic end of the SVA (Fig. 2B and Supplementary Videos S5 and S6). The proximal part of the ADO was deployed in the RA side of the defect. After TEE showed no more color flow through the defect, the aortic valve was normal, the tricuspid valve was healthy and there was no evidence of coronary affection, the ADO was released from the delivery cable (Fig. 3B and Supplementary Video S7). Fig. 3C shows the aorta long-axis view post device closure with the disappearance of color flow through the defect ( Supplementary Videos S8–S10). The patient was discharged home after two days. At follow up after six months, there was a dramatic improvement in symptoms, and TTE revealed no residual flow. Discussion Edwards first described the SVA as a deficiency of normal elastic tissue in the media of the aortic sinuses. A blind-ended diverticulum
(A) 3D zoom short axis on AV, three coronary sinuses were seen, the noncoronary sinus had aneurysmal tissues with significant filling defects. (B) Mid-esophageal view showed the flow from ascending aorta to the RA above the TV. (C) 3D zoom showed the aneurysmal tissues of the noncoronary sinus protruding to the RA above the TV. (D) TTE apical four-chamber view showed color flow from the aorta to RA.
Please cite this article in press as: Naeim HA, et al. Percutaneous closure of ruptured noncoronary sinus of Valsalva to right atrium causing severe right heart failure, a case report. J Cardiol Cases (2019), https://doi.org/10.1016/j.jccase.2019.10.005
G Model
JCCASE-1140; No. of Pages 4 H.A. Naeim et al. / Journal of Cardiology Cases xxx (2019) xxx–xxx
Fig. 2.
(A) The wire passed from SVC through the ruptured sinus to aorta. Deployment of the distal disc to aorta in short-axis view (B) and long-axis view (C). (D) 3D zoom showed the aortic disc.
Fig. 3.
(A) Fluoroscopy deployed device. (B) Fluoroscopy released device. (C) Post closure of the fistula, no more flow seen.
formed which gradually increases in size due to high pressure until it ruptures [5]. SVA is accompanied by aortic regurgitation in 30-50% of cases and bicuspid aortic valve in 9% [2]. Operative mortality is between 1.9% and 3.6%, and 15-year survival is around 90% [2]. Complications of percutaneous SVA closure include failure to deployment of the device due to significant size defect, residual shunting that may close at follow up, and procedure-related aortic regurgitation (AR) due to deformity of the cusps related to the device [6]. Kerkar et al. [6] reported 20 cases of percutaneous closure of ruptured SVA; they concluded that transcatheter closure of ruptured SVA was a promising alternative to surgery in selected cases in the immediate and mid-term period. Percutaneous SVA closure should be directly compared with surgery to evaluate its safety and efficacy.
3
Preoperative TEE is essential to decide if the ruptured SVA is amenable for device closure or not. In this case, we want to emphasize the need for careful intraprocedural TEE for accurate measurement of the diameter of the defect both in the aortic side and RA side to determine the size of the device needed for closure. TEE is crucial for the assessment of pre-, intra-, and post-procedure AR. The relation of the device to aortic leaflets and whether the residual AR is due to the deformity of the leaflets because of the device is a significant role of TEE. Also, TEE is essential for assessment of tricuspid valve (TV), the relation of RA part of the device toTV leaflets, and the residual TR.
Please cite this article in press as: Naeim HA, et al. Percutaneous closure of ruptured noncoronary sinus of Valsalva to right atrium causing severe right heart failure, a case report. J Cardiol Cases (2019), https://doi.org/10.1016/j.jccase.2019.10.005
G Model
JCCASE-1140; No. of Pages 4 H.A. Naeim et al. / Journal of Cardiology Cases xxx (2019) xxx–xxx
4
Conclusion Percutaneous closure of isolated ruptured SVA without significant AR is feasible and relatively safe. TEE is crucial for guiding the procedure and detecting any complications.
Appendix A. Supplementary data
[1] Takach TJ, Reul GJ, Duncan JM, Cooley DA, Livesay JJ, Ott DA, et al. Sinus of Valsalva aneurysm or fistula: management and outcome. Ann Thorac Surg 1999;68:1573–7. [2] Weinreich M, Yu PJ, Trost B. Sinus of Valsalva aneurysm: review of the literature and an update on management. Clin Cardiol 2015;38:185–9. [3] Yan F, Abudureheman M, Huo Q. Surgery for sinus of Valsalva aneurysm: 33-year of a single center experience. Chin Med J 2014;127:4066–70. [4] Chang C-C, Chin C-H, Chen M-L, Chen T-H, Lo H-S. Sinus of Valsalva aneurysm with rupturing into the right atrium - a case report and review of the literature. Acta Cardiol Sin 2006;22:96–101. [5] Edwards JE, Burchell HB. The pathological anatomy of deficiencies between the aortic root and the heart, including aortic sinus aneurysms. Thorax 1957;12(2):125–39. [6] Kerkar PG, Lanjewar CP, Mishra N, Nyayadhish P, Mammen I. Transcatheter closure of ruptured sinus of Valsalva aneurysm using the Amplatzer duct occluder: immediate results and mid-term follow-up. Eur Heart J 2010;31:2881–7.
Supplementary material related to this article can be found, in the online version, at doi:https://doi.org/10.1016/j.jccase.2019.10.005. References
Please cite this article in press as: Naeim HA, et al. Percutaneous closure of ruptured noncoronary sinus of Valsalva to right atrium causing severe right heart failure, a case report. J Cardiol Cases (2019), https://doi.org/10.1016/j.jccase.2019.10.005