|Year : 2016 | Volume
| Issue : 2 | Page : 136-139
An eventful second natural history of a congenital bicuspid aortic valve followed up for 40 years
Ramachandra Barik1, Ramesh C Mishra2, Lalita Nemani1
1 Department of Cardiology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India
2 Department of Cardiothoracic Surgery, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India
|Date of Web Publication||4-Aug-2016|
Department of Cardiology, Nizam's Institute of Medical Sciences, Hyderabad - 500 082, Telangana
Source of Support: None, Conflict of Interest: None
The second natural history of a congenital bicuspid aortic valve with the early symptomatic presentation is eventful. The spectrum takes it start from intrauterine life. The cumulative history may include valvotomy by balloon or surgery, valve replace replacement, stuck valve, thromboembolic complications, thrombolysis, anticoagulation-related bleeding diathesis, redo of replacement, and transcathetor aortic valve implantation. In addition, a retained foreign body granuloma in such cases further lengthens the story. We report a 48-year-old male with congenitally single kidney who underwent redo aortic valve replacement (AVR) and resection textiloma after 15 years of AVR. The eventful second natural history included surgical valvotomy in pre-balloon valvotomy era, AVR, thrombolysis for stuck valve for six times, thromboembolic episode, redo AVR, and excision of paracardiac textiloma.
Keywords: Cardioembolic stroke, mechanical valve, recurrent stuck valve, redo aortic valve replacement, textiloma, thrombolysis
|How to cite this article:|
Barik R, Mishra RC, Nemani L. An eventful second natural history of a congenital bicuspid aortic valve followed up for 40 years. Nig J Cardiol 2016;13:136-9
|How to cite this URL:|
Barik R, Mishra RC, Nemani L. An eventful second natural history of a congenital bicuspid aortic valve followed up for 40 years. Nig J Cardiol [serial online] 2016 [cited 2020 Dec 1];13:136-9. Available from: https://www.nigjcardiol.org/text.asp?2016/13/2/136/187713
| Introduction|| |
Natural history of congenital aortic stenosis is eventful as its second natural history. ,,, Some of the landmark achievements in the treatment history congenital aortic stenosis include surgical valvotomy, balloon valvuloplasty 1983,  valve replacement using mechanical or bioprosthesis, sutureless valve,  fetal balloon valvuloplasty,  and transcathetor aortic valve  replacement for critical aortic stenosis reported recently. Although the valve replacement ushered a golden era with an added inherent disease called prosthetic valve diseases (thromboembolism, stuck valve, thrombolysis-related hemorrhage, bleeding diathesis related to anticoagulation, endocarditis, structural valve disease [SVD], and redo surgery).  Bioprosthesis improved the adverse events but with more frequent SVD.  However, it has not been reported earlier any patient undergoing redo cardiac surgery for resection of textiloma and stuck valve replacement together.
| Case report|| |
A 48-year-old man presented with sixth-time thrombosis of the mechanical aortic valve with New York Heart Association (NYHA) Class IV for 2 days. He was a nondiabetic and nonsmoker. The diagnosis of the stuck valve was based on clinical profile, history of recurrent episodes of stuck valve [Table 1], transesophageal echocardiography, fluoroscopy, and international normalized ratio (INR) of 1.6 in this presentation. Chest X-ray showed pulmonary venous hypertension. Twelve-lead electrocardiogram suggested a score of six for left ventricular hypertrophy (LVH) by Rohm Hilt Estes' point criteria. The past medical history revealed he had undergone surgical valvotomy in 1981 (35 years back) in pre-PBAV era. He had normal functioning solitary right kidney. Aortic valve was replaced using St. Jude's bileaflet mechanical valve of size 19 mm in 2000 for symptomatic SVD (aortic stenosis and regurgitation). He was on regular follow-up with warfarin with anticoagulation monitoring. However, all the reports of prothrombin time (PT) and INR were not available. The follow-up medications were warfarin dose titrated to PT/INR of 2-3, Ecosprin 150 mg/daily, statin, and angiotensin receptor blocker. He had multiple lacunar infarct of the brain in 2012 which was due to thromboembolism. At the time of presentation, there was no residual neurological deficit. However, between 2001 and 2015, he had suffered six attacks of stuck valve. All the stuck valve episodes were thrombolysed successfully using streptokinase (STK) but sixth one. A look at his past medical record did not reveal adequate evaluation for recurrent thrombosis. This patient was with us for the treatment of the sixth spell of stuck valve. A battery of routine test was done before thrombolysis. Chest X-ray in anterior-posterior view showed a rounded right paracardiac mass and lateral view showed obliteration of retrosternal space. Transthoracic echocardiogram and transesophageal echo showed compression of the right atrial free wall by a paracardiac mass, stuck aortic valve with a peak gradient of 128 mmHg and a mean of 80 mmHg, good biventricular function with concentric LVH. Other valves were normal. Fluoroscopy suggested reduced movement of aortic valve leaflets. Therefore, thrombolysis was not done for the sixth spell of stuck valve. Multiple episodes of stuck valve with NYHA III/IV shortness of breath and cardioembolic stroke in 2012 were IIA level of evidence for redo aortic valve replacement (AVR). Computed tomography (CT) confirmed normal coronaries. There was a heterogeneous spherical soft tissue mass of size 5.2 cm × 4.6 cm × 6.5 cm and density of 44 HU, in the region of right paracardiac region leading to obliteration of the right cardiophrenic angle. Some calcific plaques were seen in peripheral part of this mass. There was minimal contrast enhancement. The opening inferior vena cava was effaced. There was a well-defined plane between this mass and neighboring structures. The restricted movement of leaflet of mechanical aortic valve was clearly defined.
|Table 1: The second natural history from 1981 to 2015 (chronological snippet) |
Click here to view
Serum fibrinogen level was 306 mg/dl. Plasma homocysteine level was 11 mg/dl. Antibody G and M for antiphospholipid antibody was negative. The level of thyroid-stimulating hormone was 4 μIU/ml. Glomerular filtration rate estimated using Modification of Diet in Renal Disease equation was 72 ml/min/1.73 m 2 . The solitary kidney size was normal in size with normal resistive index at hilum, middle segment, and distal segment of renal arteries (approximately 0.6-0.7). Carotid Doppler was normal. The differential leukocyte count showed normal absolute count of 603/cubic mm. Protein C and protein S levels were within normal limit.
Genotyping for warfarin function evaluation revealed no polymorphism of CYP450 2C9.2, 2C9.3, and VKORC1 promoter region. The daily recommended dose of warfarin based on this report was 5.1 mg daily. Karyotyping ruled out Turner syndrome.
The surgical note
Under cardiopulmonary support, the stuck valve was replaced using 19 mm St. Jude's bileaflet mechanical valve. Our patient could not afford stentless bioprosthesis or homograft which is suitable because of repeated thrombosis, and our patient was almost nearing 60 years. The removed valve was studded with pannus [Figure 1]. A smooth-walled right paracardiac mass abutting on the right border of right atrium was seen. It was firmly attached the wall right atrium. It was dissected completely from neighboring structures. A partially decomposed retained intrathoracic surgical swab was inside the mass [Figure 2]. The damaged right atrial wall was repaired using bovine pericardial patch.
|Figure 1: Rounded right paracardiac mass of size 6 cm in diameter with irregular surface was densely attached to the right atrial free wall. Hemorrhagic patches on the surface of the granuloma were apparent. On resection yellowish degenerated cotton threads (degenerated gauze swab) was seen|
Click here to view
|Figure 2: Pannus was quite apparent in the hinges of the bileaflet mechanical (St. Jude) valve, almost restricting valve opening to 10°|
Click here to view
Gross examination of the resected specimen showed a cystic mass of size 6 cm × 5 cm, irregular surface with hemorrhagic patches, wall thickness of 0.5 cm, and yellowish central stuff studded with fragmented cotton threads, suggesting "textiloma." Histopathology concluded it chronic nonspecific granuloma consists of lymphomononuclear cells, histiocytic foreign body giant cells and flattened mesothelial cells lining. There was no evidence of malignant cells.
This patient has completed 2 years follow-up after second-time AVR with mechanical valve regular follow-up. The follow-up is uneventful.
| Discussion|| |
Recurrent stuck of mechanical valve is not uncommon. The level of anti-STK antibody rises with repeated thrombolysis but does not interfere the benefit of thrombolysis which may explain why our patient had been benefited by thrombolysis for five times with STK because of financial reason.  It may be related to the type of valve, patient-prosthesis mismatch, structural heart valve disease, compliance to oral anticoagulation (OAC), quality of monitoring of PT and INR. Among the rare causes of mechanical valve thrombosis, genetic polymorphism of CYP450 2C9.2, 2C9.3, and VKORC1 promoter region,  hypereosinophilia, , mutation in methylenetetrahydrofolate reductase enzyme,  and antiphospholipid antibody.  The choice of valve varies from patient to patient depending on their age, gender, reproductive age, thrombotic risk, body habitus, geographical territory (infrastructure to support monitoring and developed vs. developing countries), thrombotic risk and affordability and contraindication to OAC. ,, Our patient is an active middle-aged adult (age, 58 years) without contraindication to OAC. Although there was no known risk factor for thrombosis in our patient, the foreign body granuloma (paracardiac textiloma) may a source of thrombotic milieu in the body. Therefore, resection of textiloma and replacement of aortic valve are well-accepted for accepted our patient. The annulus was relatively small (19 mm) in our patient which fairly good to replace with 19 mm mechanical valve with good long-term result.  Because of the financial limitation like most of the developing countries, we could not use homograft, stentless bioprosthesis with or without aortic root enlargement which are good option in this case. , Although our patient had already had an episode cardioembolic stroke consequent to stuck mechanical valve, we preferred mechanical valve in our case to reduce patient-prosthesis mismatch because stented tissue valves are inherently bulky for small size annulus. We opted out aortic annular dilatation because of relative risk and no consent from the patient. , Contemporarily, genotyping based dose of warfarin is not approved by Food and Drug Administration. Additional antiplatelet does reduce minor cardioembolic stroke but not reduces mortality. The adequate anticoagulation with monthly adjustment of PT/INR reduces the incidence of stuck valve.  Though case had bicuspid aortic valve (BAV) and solitary right kidney, there was no feature Turner syndrome in which BAV may be associated with a single kidney. 
| Conclusion|| |
The low incidence, low index of suspicion and nonspecific findings on plain chest X-ray or CT chest in the postoperative period could be the main causes of missing chest textiloma after open heart surgery. Thus, if a thoracic mass is seen in a patient with persistent respiratory symptoms and a history of cardiac surgery, textiloma should be included in the differential diagnose. The second of natural history in this case of BAV with single congenital kidney was very interesting because it included a follow-up period of 40 years, surgical valvotomy, valve replacement, stuck valve, five times thrombolysis, redo open cardiac surgery for AVR, and resection of textiloma.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Campbell M. The natural history of congenital aortic stenosis. Br Heart J 1968;30:514-26.
Gardiner H, Kovacevic A, Tulzer G, Sarkola T, Herberg U, Dangel J, et al.
Natural history of 107 cases of Fetal aortic stenosis from a European multicenter retrospective study. Ultrasound Obstet Gynecol 2016. doi: 10.1002/uog.15876. [Epub ahead of print].
Nishimura RA, Pieroni DR, Bierman FZ, Colan SD, Kaufman S, Sanders SP, et al.
Second natural history study of congenital heart defects. Aortic stenosis: Echocardiography. Circulation 1993;87 2 Suppl: I66-72.
Carabello BA, Paulus WJ. Aortic stenosis. Lancet 2009;373:956-66.
Lababidi Z. Aortic balloon valvuloplasty. Am Heart J 1983;106(4 Pt 1):751-2.
Meuris B, Flameng WJ, Laborde F, Folliguet TA, Haverich A, Shrestha M. Five-year results of the pilot trial of a sutureless valve. J Thorac Cardiovasc Surg 2015;150:84-8.
Freud LR, Tworetzky W. Fetal interventions for congenital heart disease. Curr Opin Pediatr 2016;28:156-62.
Kodali SK, Williams MR, Smith CR, Svensson LG, Webb JG, Makkar RR, et al.
Two-year outcomes after transcatheter or surgical aortic-valve replacement. N Engl J Med 2012;366:1686-95.
Zhao DF, Seco M, Wu JJ, Edelman JB, Wilson MK, Vallely MP, et al.
Mechanical versus bioprosthetic aortic valve replacement in middle-aged adults: A Systematic Review and Meta-Analysis. Ann Thorac Surg 2016. pii: S0003-497501764-6.
Rahimtoola SH. Choice of prosthetic heart valve for adult patients. J Am Coll Cardiol 2003;41:893-904.
Singh S, Doshi S, Salahuddin S, Tarik M, Barwad P, Ramakrishnan L, et al.
Antistreptokinase antibodies and outcome of fibrinolytic therapy with streptokinase for left-sided prosthetic valve thrombosis. Am Heart J 2015;169:170-4.
Wolkanin-Bartnik J, Pogorzelska H, Szperl M, Bartnik A, Koziarek J, Bilinska ZT. Impact of genetic and clinical factors on dose requirements and quality of anticoagulation therapy in Polish patients receiving acenocoumarol: Dosing calculation algorithm. Pharmacogenet Genomics 2013;23:611-8.
Awasthy N, Bhat Y, Radhakrishnan S, Sharma R. Recurrent stuck mitral valve: Eosinophilia an unusual pathology. Pediatr Cardiol 2015;36:692-3.
Zakhama L, Slama I, Boussabah E, Harbegue B, Mimouni M, Abdelaali N, et al
. Recurrent native and prosthetic mitral valve thrombosis in idiopathic hypereosinophilic syndrome. J Heart Valve Dis 2014;23:168-70.
Kalcik M, Gursoy MO, Karakoyun S, Yesin M, Astarcioglu MA, Ozkan M. Potential inherited causes of recurrent prosthetic mitral valve thrombosis in a pregnant patient suffering from recurrent miscarriage. Korean Circ J. 2014;44:268-70.
Aykan AÇ, Gökdeniz T, Kalçik M, Astarcioglu MA, Gündüz S, Karakoyun S, et al.
Role of anticardiolipin antibodies in the pathogenesis of prosthetic valve thrombosis: An observational study. Herz 2015;40:528-33.
Khalpey Z, Myers PO, McGurk S, Schmitto JD, Nauta F, Borstlap W, et al.
Nineteen-millimeter bioprosthetic aortic valves are safe and effective for elderly patients with aortic stenosis. Ann Thorac Surg 2016;101:650-7.
Wu JJ, Seco M, Edelman JB, Eslick GD, Wilson MK, Vallely MP, et al
. Mechanical versus bioprosthetic aortic valve replacement in patients aged 40 to 70 years: A systematic review and meta-analysis. Ann Thorac Surg 2016. p. S0003-4975(15)01764-6. doi: 10.1016/j.athoracsur.2015.10.092. [Epub ahead of print].
Alameddine AK, Alimov VK, Pluchino F. A technique for avoiding patient-prosthesis mismatch in the small aortic root. J Card Surg 2016;31:309-10.
Wollersheim LW, Li WW, Kaya A, Bouma BJ, Driessen AH, van Boven WJ, et al
. Stentless versus stented aortic valve bioprostheses in the small aortic root. In: Seminars in Thoracic and Cardiovascular Surgery. WB, Saunders; 2016.
Beckmann E, Martens A, Alhadi F, Hoeffler K, Umminger J, Kaufeld T, et al.
Aortic valve replacement with sutureless prosthesis: Better than root enlargement to avoid patient-prosthesis mismatch? Interact Cardiovasc Thorac Surg 2016;22:744-9.
Yoon IK, Lee KE, Lee JK, Chang BC, Gwak HS. Adequate intensity of warfarin therapy for Korean patients with mechanical cardiac valves. J Heart Valve Dis 2013;22:102-9.
Al Alwan I, Khadora M, Amir I, Nasrat G, Omair A, Brown L, et al
. Turner Syndrome Genotype and phenotype and their effect on presenting features and timing of Diagnosis. International journal of health sciences 2014;8:195.
[Figure 1], [Figure 2]