Revista Portuguesa de Cardiologia 41 (2022) 177.e1---177.e3 Revista Portuguesa de Cardiologia Portuguese Journal of Cardiology www.revportcardiol.org CASE REPORT Ruptured sinus of Valsalva aneurysm: A reversible cause of suprasystemic pulmonary hypertension Lluis Asmarats a,b,∗ , Armando Bethencourt c , Antonio Rodríguez a , Marta Noris a , Jaume Maristany a , Andrés Grau a , Alfredo Gómez-Jaume a , Vicente Peral a a Department of Cardiology, Hospital Universitari Son Espases, Palma, Spain b Department of Cardiology, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain c Department of Cardiology, Hospital Quirón Palmaplanas, Palma, Spain Received 15 April 2018; accepted 28 October 2018 Available online 8 December 2021 KEYWORDS Abstract Management of patients with congenital heart defects and associated pulmonary Sinus of Valsalva arterial hypertension remains a major concern. With evolving targeted drug therapies and new aneurysm; iterations of transcatheter devices, treatment of appropriately selected patients with severe Pulmonary pulmonary hypertension, classically considered inoperable, has become feasible. We report the hypertension; case of a patient with concomitant ruptured right sinus of Valsalva aneurysm and ventricular Transcatheter closure septal defect, with early reversal of suprasystemic pulmonary pressures following successful percutaneous closure of ruptured sinus of Valsalva. © 2021 Sociedade Portuguesa de Cardiologia. Published by Elsevier España, S.L.U. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by- nc-nd/4.0/). PALAVRAS-CHAVE Shunt cardíaco e hipertensão pulmonar: uma associação preocupante mas corrigível Aneurisma do seio de Valsalva; Resumo O tratamento de pacientes com defeitos cardíacos congénitos e hipertensão pul- Hipertensão monar associada continua a ser um importante motivo de preocupação. Com o desenvolvimento pulmonar; de estratégias farmacológicas dirigidas e novos dispositivos transcatéter, o tratamento de Encerramento pacientes selecionados com hipertensão pulmonar grave, tradicionalmente considerados inop- percutâneo eráveis, tornou-se viável. Apresentamos o caso de um paciente com aneurisma do seio de Valsalva direito roto e defeito do septo ventricular, com rápida reversão das pressões pulmonares suprassistêmicas após encerramento percutâneo do seio de Valsalva roto. © 2021 Sociedade Portuguesa de Cardiologia. Publicado por Elsevier España, S.L.U. Este é um artigo Open Access sob uma licença CC BY-NC-ND (http://creativecommons.org/licenses/by- nc-nd/4.0/). ∗ Corresponding author. E-mail address: luis asmarats@hotmail.com (L. Asmarats). https://doi.org/10.1016/j.repc.2018.10.016 0870-2551/© 2021 Sociedade Portuguesa de Cardiologia. Published by Elsevier España, S.L.U. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). L. Asmarats, A. Bethencourt, A. Rodríguez et al. Introduction discussion and considering the anatomical suitability of the rupture opening for a transcatheter approach (moderate Sinus of Valsalva aneurysm (SVA) is a rare condition affecting sized defect, optimal proximal and distal margins, no 3% of Asian and <1% of Western patients undergoing cardiac associated aortic regurgitation), the patient was referred surgery, with a 4:1 male predominance.1 Etiology is either for transcatheter ruptured SVA closure, after prior balloon congenital or acquired secondary to infective endocarditis, test occlusion to assess PAH reversibility. syphilis, atherosclerosis, Marfan syndrome, autoimmune dis- The procedure was performed under fluoroscopic and eases or chest trauma. Aneurysms arising from the right TEE guidance, using a retrograde approach, under intra- sinus are the most common, usually rupturing into the venous heparin (80 U/kg) and antibiotic cover (cefazolin). right ventricle. Nearly 50% of SVAs coexist with a long- A Swan-Ganz thermodilution catheter was inserted through standing primary ventricular septal defect (VSD), most often the right cephalic vein. Baseline invasively determined pres- supracristal. Rupture typically occurs in the mid-thirties, sures showed suprasystemic PAH with a mean pulmonary with variable severity of symptom onset according to size, pressure of 59 mmHg and a mean aortic pressure of 50 location, associated lesions and acuity of the rupture. If left mmHg. A pigtail catheter for aortography was advanced untreated, right ventricular overload and pulmonary arterial from the left femoral artery. Through right femoral arte- hypertension (PAH) develop, with a grim prognosis (one- rial access, the SVA defect was crossed retrogradely from year life expectancy).2 While surgery remains the mainstay the aorta into the right ventricular outflow tract with a treatment for ruptured SVA, percutaneous treatment has Judkins right catheter (Cordis Corp., Miami, FL), which was emerged as a valid alternative for selected patients.3 exchanged for a Mullins sheath (Cook Inc., Bloomington, IN) through a stiff exchange wire. A 10/5-mm Amplatzer Vas- cular Plug III (Abbott Vascular, Santa Clara, CA) was first Case report advanced through the Mullins sheath to occlude the ruptured SVA for 30 min with incomplete shunt sealing, and there- A 39-year-old man presented with a two-month history after replaced by a 12-mm Amplatzer Muscular VSD device, of worsening dyspnea and rapidly progressive orthopnea, resulting in successful shunt occlusion (Figure 2A). Rapid in New York Heart Association (NYHA) functional class hemodynamic improvement was seen during the transient III. Physical examination revealed a continuous systolic- occlusion test, with a marked reduction in mean pulmonary diastolic murmur in the pulmonary area and raised jugular pressure (from 59 to 49 mmHg), increase in mean aortic pres- venous pressure, but no cyanosis. The electrocardiogram sure (from 50 to 73 mmHg), and normalization of oxygen showed sinus tachycardia with right bundle branch block, saturation (SpO2 at 4 l/min from 86% to 100%) (Table 1). and the chest X-ray revealed cardiomegaly with pulmonary After confirmation of the suitability for closure of the rup- venous congestion. Transthoracic echocardiography (TTE) tured SVA, the 12-mm Amplatzer Muscular VSD device was revealed right-sided volume overload with right ventricular released, with minimal residual peri-device leak, no aortic enlargement (45 mm), dilated pulmonary trunk (38 mm) regurgitation and inversion of the VSD shunt (Figure 2B). with near-systemic PAH (>90 mmHg), and the presence The small VSD was considered hemodynamically insignifi- of a perimembranous ventricular septal defect (VSD), cant (restrictive) and was therefore untreated. The patient with normal left ventricular size and function (Figure 1A). was discharged with no complications on dual antiplatelet Transesophageal echocardiography (TEE) confirmed the therapy for six months, and under sildenafil 20 mg/8 h presence of a 3-mm perimembranous VSD with bidirec- and furosemide 20 mg/24 h. At one-year follow-up, the tional shunting (predominantly right-to-left) (Figure 1B), patient remained without events, in NYHA functional class while also revealing a 7-mm right SVA ruptured into the I, with minimal resting left-to-right shunt, and complete right ventricular outflow tract adjacent to the pulmonary regression of suprasystemic PAH, allowing discontinuation valve (Figure 1C). Following multidisciplinary heart team of furosemide and sildenafil (Figure 2C). Figure 1 (A) Transthoracic echocardiography showing a perimembranous ventricular septal defect (VSD, asterisk) with severe pul- monary hypertension; (B) two- and three-dimensional transesophageal echocardiography confirming VSD with bidirectional shunting, and unmasking a circular rupture opening of the right sinus of Valsalva aneurysm (6.5 mm×6.7 mm, arrow) into the right ventricular outflow tract (C). 177.e2 Revista Portuguesa de Cardiologia 41 (2022) 177.e1---177.e3 Figure 2 Fluoroscopic and echocardiographic images of the ruptured sinus of Valsalva aneurysm (arrow) during transient percuta- neous occlusion testing (A) and after definitive closure (B) with the 12-mm Amplatzer Muscular VSD device, with trace peri-device leak and trivial left-to-right shunt through the VSD; (C) one-year echocardiographic follow-up showing appropriate apposition and sealing of the device and absence of pulmonary hypertension. Table 1 Changes in hemodynamic parameters during transient occlusion test. SpO2 , % at 4 l/min PAP, mmHg AoP, mmHg PCWP, mmHg HR, bpm CO, l/min a Baseline 86 92/46/59 61/40/50 53/39/50 75 ---b 30 minutes 100 79/40/49 88/68/73 30/19/16 68 2.47 a Probable artefactual (damped pulmonary pressure). b Not reliable by thermodilution technique in the context of a large systemic-to-pulmonary shunt. AoP: aortic pressure; CO: cardiac output; HR: heart rate; PAP: pulmonary arterial pressure; PCWP: pulmonary capillary wedge pressure; SpO2 : blood oxygen saturation. Discussion right heart failure and hypertensive crisis immediately after closure. However, the rationale for closure in our patient The present case demonstrates the reversal of severe flow- was the subacute onset of symptoms leading to rapid clini- mediated PAH related to systemic-to-pulmonary shunting cal deterioration and the presence of bidirectional shunting with pulmonary overcirculation following transcatheter clo- through the VSD, along with marked hemodynamic improve- sure of a ruptured SVA into the right ventricle. ment after the transient occlusion test, making the hypoth- SVA are generally silent until rupture into a cardiac cham- esis of fixed (irreversible) PAH highly unlikely, and thus high- ber. In our patient with concomitant SVA and VSD, the right lighting a potential benefit from permanent shunt closure. coronary cusp aneurysm probably occluded the VSD until the In recent years, percutaneous closure of ruptured SVAs sinus ruptured into the right ventricle, resulting in a large has emerged as a valid alternative to surgery. Although systemic-to-pulmonary shunt through the ruptured SVA and direct comparisons between the two approaches have causing systemic systolic pressure overload in the pulmonary been somewhat limited by selection bias (more severe artery and systemic diastolic pressure and volume overload aortic regurgitation and more complex associated lesions in the right ventricle, with predominant right-to-left shunt- in patients undergoing surgery), transcatheter closure of ing through the VSD and desaturation. Interestingly, closure ruptured SVAs has been shown to be safe and effective.3 of the ruptured SVA led to resolution of the aortocardiac Suitability for percutaneous closure of intracardiac commu- shunt, with inversion of the VSD shunt to left-to-right shunt- nications with associated PAH relies on anatomic features --- ing and normalization of saturation. magnitude of the shunt, presence of adequate rim to secure The degree of reversibility of the pulmonary circulation the device --- and potential for PAH reversal. In our patient, can be assessed using short-acting pulmonary vasodilators, three-dimensional TEE was crucial to precisely delineate or through transient balloon test occlusion, particularly two closely adjacent congenital defects, the shunt flows of in patients with persistently elevated pulmonary vascular which overlapped on TTE, showing anatomical suitability resistance despite a positive response to vasodilators, and for percutaneous closure of the ruptured SVA. Although those with bidirectional shunting.4 Although somewhat arbi- lacking solid scientific evidence, transcatheter closure of trary, a drop in pulmonary pressures of ∼20% without a a ruptured SVA might be a reasonable option for patients significant decrease in systemic pressure during test occlu- with a rupture opening of less than 9 mm,6 in the absence sion would suggest a high likelihood of benefiting from of secondary complications (fistulous ruptured SVA or permanent closure. Closure of cardiac shunts is generally not multiple rupture sites, significant aortic regurgitation) recommended when pulmonary artery pressures are >2/3 of or pre-existing conditions requiring surgical correction systemic values,5 due to increased risk of sustained PAH, (endocarditis, large VSD or other complex congenital 177.e3 L. Asmarats, A. Bethencourt, A. Rodríguez et al. defects).3 Of note, large lesions (>12 mm) should prompt References preprocedural multimodality imaging assessment (including computed tomography or magnetic resonance imaging) to 1. Chu SH, Hung CR, How SS, et al. Ruptured aneurysms of the elucidate the best therapeutic approach.3 Most importantly, sinus of Valsalva in oriental patients. J Thorac Cardiovasc Surg. while transcatheter occlusion of ruptured SVAs has emerged 1990;99:288---98. as a well-established alternative to surgical closure for 2. Sakakibara S, Konno S. Congenital aneurysms of sinus of Valsalva. highly selected patients, caution is warranted in the setting A clinical study. Am Heart J. 1962;63:708---19. of associated severe PAH. Transient occlusion testing 3. Kuriakose EM, Bhatla P, McElhinney DB. Comparison of reported outcomes with percutaneous versus surgical closure of ruptured during cardiac catheterization is critical in determining the sinus of Valsalva aneurysm. Am J Cardiol. 2015;115:392---8. safety of permanent shunt closure, enabling differentiation 4. Dimopoulos K, Peset A, Gatzoulis MA. Evaluating operability in between dynamic PAH and irreversible obstructive PAH, and adults with congenital heart disease and the role of pretreat- providing reliable assessment of the reversibility of pul- ment with targeted pulmonary arterial hypertension therapy. Int monary vascular disease. To our knowledge, this is the first J Cardiol. 2008;129:163---71. case report of suprasystemic flow-induced PAH regression 5. Baumgartner H, Bonhoeffer P, De Groot NM, et al. ESC Guidelines following percutaneous closure of a ruptured SVA. for the management of grown-up congenital heart disease (new version 2010). Eur Heart J. 2010;31:2915---57. 6. Zhong L, Tong SF, Zhang Q, et al. Clinical efficacy and safety Conflicts of interest of transcatheter closure of ruptured sinus of valsalva aneurysm. Catheter Cardiovasc Interv. 2014;84:1184---9. The authors have no conflicts of interest to declare. 177.e4