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Quadricuspid Aortic Valve Plasty: Is it Worth it to Repair as an Alternative to Substitution?
Quadricuspid Aortic Valve Plasty: Is it Worth it to Repair as an Alternative to Substitution? Andrea Daprati, MD, Tommaso Generali, MD, Francesco Arlati, MD, and Maurizio Roberto, MD Department of Cardiovascular Disease, Cardiac Surgery Unit, IRCCS Centro Cardiologico Fondazione Monzino, Milano, Italy
We present the case of a 49-year-old man with an abnormal aortic valve formed by four cusps (three equal large cusps and one smaller cusp; type 1 according to Hurwits and Roberts classification) with a marked lack of coaptation that caused a severe aortic valve insufficiency. The patient underwent a repair of the defect with a tricuspidalization of the aortic valve, restoring the normal coaptation of the cusps and the normal function of the aortic valve. Postoperative course was unremarkable. The early follow-up showed a mild aortic valve insufficiency. (Ann Thorac Surg 2013;95:e7– 8) © 2013 by The Society of Thoracic Surgeons
T
he quadricuspid aortic valve is a rare congenital heart defect. The incidence, which ranges from 0.008% to 0.033%, is nearly equal in men and women, although some authors have described a slight male predilection [1]. The first quadricuspid aortic valve was reported in 1862 by Balington [1]. A recent review cited 186 cases of quadricuspid aortic valve in the world literature, with the patients’ mean age at the time of diagnosis being 51 years [2]. Previously, this congenital defect was diagnosed at the time of surgery or at postmortem examination. With advances in echocardiography, more cases are being diagnosed before surgery. Echocardiography series incidence rates range from 0.013% to 0.043% [3]. During embryogenesis, the formation of the three leaflets of the aortic valve derives from the presence of three small pads of connective forming the three aortic cusps. Any further anomalus division leads to a variable number of the cusps [1, 3]. Hurwits and Roberts classified this kind of valves into seven types according to the relationship in size of the four aortic cusps [4]. In this pattern of anomalous anatomy, an aortic valve dysfunction such as an alteration in hemodynamic is not
Accepted for publication June 21, 2012. Address correspondence to Dr Daprati, Department of Cardiovascular Disease, Cardiac Surgery Unit, IRCCS Centro Cardiologico Fondazione Monzino, Via Parea 4, Milan, Italy; e-mail: [email protected].
rare. Aortic valve insufficiency is the most common finding, although rarely aortic stenosis or endocarditis have been reported [1]. The aortic valve insufficiency is the most common finding in these patients because an unequal juxtaposition of the cusps in diastole and distribution of stress can cause a deterioration of valve function over the years [1]. Quadricuspid aortic valve can be associated with other congenital heart defects. In approximately 18% of the cases reviewed, an additional malformation was found, including anomalies of the coronary arteries, ventricular septal defect, patent ductus arteriosus, subaortic fibromuscular stenosis, and mitral valve defects [1–3]. The past surgical treatment of choice for those who have an aortic valve dysfunction owing to a supernumerary cusp was usually the aortic valve replacement (AVR), especially in the patients in whom the valve is markedly calcified or is subject to an endocarditis process. Obviously the replacement with the implantation of a biological or mechanical prosthesis can lead to valve-related risks such as thromboembolism, prosthetic valve endocarditis, and bleeding. For this reason, in the absence of significant calcification or endocarditis process, the valve repair is considered the standard treatment; however, long-term results are still not described [1,4]. The most common repair technique is the aortic valve tricuspidalization—that is, the conversion of a quadricuspid aortic valve into a tricuspid valve by anastomosing the commissures of a coronary, or noncoronary, cusp and the supranumerary cusp, thereby restoring normal coaptation [1]. We present the case of a 49-year-old man (NYHA class I, AHA class B) with a history of hypertension and hyperlipidemia. He was referred to our institution with an accidental finding at a transthoracic echocardiogram performed during cardiologic routine screening of an abnormal aortic valve formed by four cusps (three equal large cusps and one smaller cusp, type 1 according to Hurwits and Roberts classification) with a marked lack of coaptation, which caused a severe aortic valve insufficiency (Figs 1A, 1B, 2A). He was then examined with a transesophageal threedimensional echocardiogram (Fig 1. B) that confirmed the presence of an abnormal aortic valve with a supranumerary cusp and an important coaptation defect causing a severe regurgitation. No other congenital heart defects have been shown. Considering the clinical condition and the age of the patient, we decided to surgically repair the aortic valve instead to replace it. Fig 1. (A) Two-dimensional echocardiographic image showing the presence of four aortic valve cusps, three normal cusps, and one small accessory cusp. (B) Three-dimensional intraoperative transesophageal echocardiographic image that confirms the presence of four aortic cusps with a marked defect of coaptation causing a severe aortic valve regurgitation. (A ⫽ accessory cusp; L ⫽ left coronary cusp; N ⫽ noncoronary cusp; R ⫽ right coronary cusp.)
© 2013 by The Society of Thoracic Surgeons Published by Elsevier Inc
0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2012.06.036
e8
CASE REPORT DAPRATI ET AL QUADRICUSPID AORTIC VALVE REPAIR
Ann Thorac Surg 2013;95:e7– 8
Fig 2. (A) Intraoperative view of the quadricuspid aortic valve. We performed the tricuspidalization of the quadricuspid aortic valve anastomosing with separated stitches with felts, the commissures of the noncoronary, and the supranumerary small cusp (white arrows). (B) Intraoperative image of the aortic valve after tricuspidalization (black arrow). We restored a normal aortic tricuspid valve and with a normal cusps coaptation. We also have positioned three subcommissural stitches with felt over the leaflets, gaining an annular reduction and an elevation of the valve, improving leaflet to leaflet coaptation (black arrow). (A ⫽ accessory cusp; L ⫽ left coronary cusp; N ⫽ noncoronary cusp; R ⫽ right coronary cusp.)
Through a median sternotomy, on cardiopulmonary bypass with aortic cross clamping, we performed the tricuspidalization of the aortic valve, which appeared to be a real quadricuspid valve, anastomosing by separated stitches with felts the commissures of the noncoronary and the supranumerary small cusp, so creating a new bigger noncoronary cusp and restoring a normal tricuspid valve and a good coaptation surface (Figs 2A, 2B). To reduce the risk of insufficiency, we then performed a commissural plication by positioning three subcommissural stitches with felt over the leaflets, gaining an annular reduction and an elevation of the valve, improving leaflet to leaflet coaptation, and stabilizing it (Fig 2B). On the hydrodynamic testing, the resulting new tricuspid valve was competent, with no evident signs of regurgitation. No further annular dilation or commissural plication was performed in order to avoid the risk of a significant stenosis.
Comment Another common repair technique we considered is accessory leaflet exclusion with annular reduction. The rationale for this technique is the same as for the Kay technique for tricuspid valve repair in tricuspid valve insufficiency. In our case, there was no critical annular dilatation. The problem was the altered coaptation of the cusps due to the supernumerary leaflet. We did not choose this technique because we prefer the “respect rather than resect” approach. As a result, we have been conservative regarding the native valve anatomy, with the result of an effective fixation of the anatomic defect. Postoperative course was unremarkable. The patient was discharged home on day 6 in good general condition.
Postoperative transthoracic echocardiography showed a residual mild aortic regurgitation with a good coaptation of the cusps. Transthoracic echocardiogram 3 months after surgery showed a residual mild-moderate aortic insufficiency. Perhaps a mild stenosis would be a better result than a permanence of insufficiency, but we think that a further commissural plication, even possible, would have generates more than a mild stenosis. for this reason, we preferred not to proceed on with a further plication. Postoperative transthoracic echocardiography also showed a significant reduction of ventricular volumes compared with the preoperative evaluation. In particular, the end-diastolic volume was reduced from 161 to 114 mL, and the end-systolic volume decreased from 70 to 55 mL. During the whole period, the patient remained asymptomatic (NYHA class I, AHA class A). In patients without endocarditis or aortic valve stenosis, the repair of the aortic abnormal valves can be performed with good early results. Midterm and long-term follow-up is still needed to confirm or disconfirm these results.
References 1. James KB, Centorbi LK, Novoa R. Quadricuspid aortic valve. Case report and review of the literature. Tex Heart Inst J 1991;18:141–3. 2. Tutarel O. The quadricuspid aortic valve: a comprehensive review. J Heart Valve Dis 2004;13:534 –7. 3. Tang YF, Xu JB, Han L, et al. Congenital quadricuspid aortic valve: analysis of 11 surgical cases. Chin Med J 2011;124:2779 – 81. 4. Hurwitz LE, Roberts WC. Quadricuspid semilunar valve. Am J Cardiol 1973;31:623– 6.
We present the case of a 49-year-old man with an abnormal aortic valve formed by four cusps (three equal large cusps and one smaller cusp; type 1 according to Hurwits and Roberts classification) with a marked lack of coaptation that caused a severe aortic valve insufficiency. The patient underwent a repair of the defect with a tricuspidalization of the aortic valve, restoring the normal coaptation of the cusps and the normal function of the aortic valve. Postoperative course was unremarkable. The early follow-up showed a mild aortic valve insufficiency. (Ann Thorac Surg 2013;95:e7– 8) © 2013 by The Society of Thoracic Surgeons
T
he quadricuspid aortic valve is a rare congenital heart defect. The incidence, which ranges from 0.008% to 0.033%, is nearly equal in men and women, although some authors have described a slight male predilection [1]. The first quadricuspid aortic valve was reported in 1862 by Balington [1]. A recent review cited 186 cases of quadricuspid aortic valve in the world literature, with the patients’ mean age at the time of diagnosis being 51 years [2]. Previously, this congenital defect was diagnosed at the time of surgery or at postmortem examination. With advances in echocardiography, more cases are being diagnosed before surgery. Echocardiography series incidence rates range from 0.013% to 0.043% [3]. During embryogenesis, the formation of the three leaflets of the aortic valve derives from the presence of three small pads of connective forming the three aortic cusps. Any further anomalus division leads to a variable number of the cusps [1, 3]. Hurwits and Roberts classified this kind of valves into seven types according to the relationship in size of the four aortic cusps [4]. In this pattern of anomalous anatomy, an aortic valve dysfunction such as an alteration in hemodynamic is not
Accepted for publication June 21, 2012. Address correspondence to Dr Daprati, Department of Cardiovascular Disease, Cardiac Surgery Unit, IRCCS Centro Cardiologico Fondazione Monzino, Via Parea 4, Milan, Italy; e-mail: [email protected].
rare. Aortic valve insufficiency is the most common finding, although rarely aortic stenosis or endocarditis have been reported [1]. The aortic valve insufficiency is the most common finding in these patients because an unequal juxtaposition of the cusps in diastole and distribution of stress can cause a deterioration of valve function over the years [1]. Quadricuspid aortic valve can be associated with other congenital heart defects. In approximately 18% of the cases reviewed, an additional malformation was found, including anomalies of the coronary arteries, ventricular septal defect, patent ductus arteriosus, subaortic fibromuscular stenosis, and mitral valve defects [1–3]. The past surgical treatment of choice for those who have an aortic valve dysfunction owing to a supernumerary cusp was usually the aortic valve replacement (AVR), especially in the patients in whom the valve is markedly calcified or is subject to an endocarditis process. Obviously the replacement with the implantation of a biological or mechanical prosthesis can lead to valve-related risks such as thromboembolism, prosthetic valve endocarditis, and bleeding. For this reason, in the absence of significant calcification or endocarditis process, the valve repair is considered the standard treatment; however, long-term results are still not described [1,4]. The most common repair technique is the aortic valve tricuspidalization—that is, the conversion of a quadricuspid aortic valve into a tricuspid valve by anastomosing the commissures of a coronary, or noncoronary, cusp and the supranumerary cusp, thereby restoring normal coaptation [1]. We present the case of a 49-year-old man (NYHA class I, AHA class B) with a history of hypertension and hyperlipidemia. He was referred to our institution with an accidental finding at a transthoracic echocardiogram performed during cardiologic routine screening of an abnormal aortic valve formed by four cusps (three equal large cusps and one smaller cusp, type 1 according to Hurwits and Roberts classification) with a marked lack of coaptation, which caused a severe aortic valve insufficiency (Figs 1A, 1B, 2A). He was then examined with a transesophageal threedimensional echocardiogram (Fig 1. B) that confirmed the presence of an abnormal aortic valve with a supranumerary cusp and an important coaptation defect causing a severe regurgitation. No other congenital heart defects have been shown. Considering the clinical condition and the age of the patient, we decided to surgically repair the aortic valve instead to replace it. Fig 1. (A) Two-dimensional echocardiographic image showing the presence of four aortic valve cusps, three normal cusps, and one small accessory cusp. (B) Three-dimensional intraoperative transesophageal echocardiographic image that confirms the presence of four aortic cusps with a marked defect of coaptation causing a severe aortic valve regurgitation. (A ⫽ accessory cusp; L ⫽ left coronary cusp; N ⫽ noncoronary cusp; R ⫽ right coronary cusp.)
© 2013 by The Society of Thoracic Surgeons Published by Elsevier Inc
0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2012.06.036
e8
CASE REPORT DAPRATI ET AL QUADRICUSPID AORTIC VALVE REPAIR
Ann Thorac Surg 2013;95:e7– 8
Fig 2. (A) Intraoperative view of the quadricuspid aortic valve. We performed the tricuspidalization of the quadricuspid aortic valve anastomosing with separated stitches with felts, the commissures of the noncoronary, and the supranumerary small cusp (white arrows). (B) Intraoperative image of the aortic valve after tricuspidalization (black arrow). We restored a normal aortic tricuspid valve and with a normal cusps coaptation. We also have positioned three subcommissural stitches with felt over the leaflets, gaining an annular reduction and an elevation of the valve, improving leaflet to leaflet coaptation (black arrow). (A ⫽ accessory cusp; L ⫽ left coronary cusp; N ⫽ noncoronary cusp; R ⫽ right coronary cusp.)
Through a median sternotomy, on cardiopulmonary bypass with aortic cross clamping, we performed the tricuspidalization of the aortic valve, which appeared to be a real quadricuspid valve, anastomosing by separated stitches with felts the commissures of the noncoronary and the supranumerary small cusp, so creating a new bigger noncoronary cusp and restoring a normal tricuspid valve and a good coaptation surface (Figs 2A, 2B). To reduce the risk of insufficiency, we then performed a commissural plication by positioning three subcommissural stitches with felt over the leaflets, gaining an annular reduction and an elevation of the valve, improving leaflet to leaflet coaptation, and stabilizing it (Fig 2B). On the hydrodynamic testing, the resulting new tricuspid valve was competent, with no evident signs of regurgitation. No further annular dilation or commissural plication was performed in order to avoid the risk of a significant stenosis.
Comment Another common repair technique we considered is accessory leaflet exclusion with annular reduction. The rationale for this technique is the same as for the Kay technique for tricuspid valve repair in tricuspid valve insufficiency. In our case, there was no critical annular dilatation. The problem was the altered coaptation of the cusps due to the supernumerary leaflet. We did not choose this technique because we prefer the “respect rather than resect” approach. As a result, we have been conservative regarding the native valve anatomy, with the result of an effective fixation of the anatomic defect. Postoperative course was unremarkable. The patient was discharged home on day 6 in good general condition.
Postoperative transthoracic echocardiography showed a residual mild aortic regurgitation with a good coaptation of the cusps. Transthoracic echocardiogram 3 months after surgery showed a residual mild-moderate aortic insufficiency. Perhaps a mild stenosis would be a better result than a permanence of insufficiency, but we think that a further commissural plication, even possible, would have generates more than a mild stenosis. for this reason, we preferred not to proceed on with a further plication. Postoperative transthoracic echocardiography also showed a significant reduction of ventricular volumes compared with the preoperative evaluation. In particular, the end-diastolic volume was reduced from 161 to 114 mL, and the end-systolic volume decreased from 70 to 55 mL. During the whole period, the patient remained asymptomatic (NYHA class I, AHA class A). In patients without endocarditis or aortic valve stenosis, the repair of the aortic abnormal valves can be performed with good early results. Midterm and long-term follow-up is still needed to confirm or disconfirm these results.
References 1. James KB, Centorbi LK, Novoa R. Quadricuspid aortic valve. Case report and review of the literature. Tex Heart Inst J 1991;18:141–3. 2. Tutarel O. The quadricuspid aortic valve: a comprehensive review. J Heart Valve Dis 2004;13:534 –7. 3. Tang YF, Xu JB, Han L, et al. Congenital quadricuspid aortic valve: analysis of 11 surgical cases. Chin Med J 2011;124:2779 – 81. 4. Hurwitz LE, Roberts WC. Quadricuspid semilunar valve. Am J Cardiol 1973;31:623– 6.