Asian Cardiovasc Thorac Ann 2004;12:266-269
© 2004 Asia Publishing EXchange Ltd
Cusp Extension Technique for Bicuspid Aortic Valve in Turner-like Stigmata
Yoshihiro Oshima, MD,
Keiju Koto, MD,
Chikashi Shimazu, MD,
Takuro Misaki, MD,
Fukiko Ichida, MD1,
Ikuo Hashimoto, MD1
Department of Surgery
1 Department of Pediatrics, Toyama Medical & Pharmaceutical University, Toyama, Japan
For reprint information contact: Yoshihiro Oshima, MD Tel: 81 76 434 2281 Fax: 81 76 434 5032 Email: oshima{at}ms.toyama-mpu.ac.jp Department of Surgery, Toyama Medical & Pharmaceutical University, 2630 Sugitani, Toyama 930– 0194, Japan.
 |
ABSTRACT
|
|---|
A 19-year-old girl with bicuspid aortic valve and Turner-like stigmata underwent a successful repair with a cusp extension technique using fresh autologous pericardium for aortic insufficiency. She also had a simultaneous artificial graft replacement for ascending aortic dilatation. Aortic valve function showed no significant stenosis and slight insufficiency 2 years postoperatively. This technique is recommended to avoid a Ross or Ross/Konno procedure because of its ready availability, simplicity and excellent midterm results.
|
INTRODUCTION
|
|---|
Recently, a revival of the aortic cusp extension technique has been reported.1,2 This technique is recommended to avoid a Ross or Ross/Konno procedure because of its ready availability, simplicity and excellent midterm results. We performed a successful repair with a cusp extension technique using fresh autologous pericardium for aortic insufficiency in a 19-year-old girl with bicuspid aortic valve and Turner-like stigmata.
A 19-year-old girl weighing 40 kg (body surface area of 1.27 m2) was referred to us due to nonspecific chest pain and progressing cardiomegaly. She had had a ligation of the patent ductus arteriosus at 2 years of age. Her condition was monitored due to her bicuspid aortic valve having a mild aortic insufficiency (AI). Although she exhibited symptoms of the Turner phenotype, including congenital hearing loss, a webbed neck and primary amenorrhea with hypoplastic uterus, the chromosomal anomaly associated with the Turner phenotype was not detected. On admission of the patient, echocardiography showed moderate AI and left ventricular dilatation (120% of normal). Catheterization demonstrated a 20 mm Hg peak pressure gradient across the aortic valve. Aortography showed grade III AI, ascending aortic dilatation with a diameter of 40 mm (3.1cm•m–2) and an aortic arch of 34 mm in diameter (Figure 1
). The aortic sinus, sino-tubular junction and descending aorta were of normal size.
View larger version (241K):
[in this window]
[in a new window]
|
Figure 1. (A) Lateral view of left ventriculogram shows left ventricular dilation and aortic dilatation. (B) Lateral view of aortogram shows moderate to severe aortic insufficiency.
|
|
We performed an ascending aortic graft replacement and an aortic valve repair on cardiopulmonary bypass with moderate hypothermia and cold blood cardioplegia. Aortic cannulation through an expanded polytetrafluoroethylene graft sutured on the brachiocephalic artery allowed us to clamp the ascending aorta just proximal to the brachiocephalic artery. A mild degree of cusp thickening and retraction without calcification was found. A 17 mm dilator passed tightly through the aortic orifice. A usual aortic valve replacement seemed to lead to obstruction of the right coronary ostium as the positioning of the ostium was very close to the right side of the commissure. Following the aortic commissurotomy without cusp shaving, each cusp was extended using a strip of fresh autologous pericardium with a height of 7 mm. The pericardial commissural area was sutured to the aortic wall above the native commissures using 5-0 polypropylene U stitches (Figure 2). The aortic orifice was enlarged enough for a 19 mm dilator to pass through easily. The dilated ascending aorta was resected, and the aorta was reconstructed with a 22 mm Dacron conduit (Hemashield, Meadox Medical Inc. Oakland, NJ). Microscopic examination of the aortic wall revealed disruption of the elastic media and accumulation of mucopolysaccharide (Figure 3). The patient recovered uneventfully. A subsequent follow-up echocardiogram showed no significant aortic stenosis, slight AI, normal LV dimension (Figure 4) and a normal reconstructed ascending aorta.
View larger version (165K):
[in this window]
[in a new window]
|
Figure 2. Each cusp was extended using a strip of fresh autologous pericardium with a height of 7 mm. The "U" stitches were passed through both commissural extensions of the pericardial patch and aortic wall above both commissures.
|
|
View larger version (257K):
[in this window]
[in a new window]
|
Figure 3. Cystic medial degeneration in this patient has accumulation of mucopolysaccharide (A, alciane blue stain, x 400) and disruption of the elastic media (B, elastic tissue stain, x 400).
|
|
View larger version (57K):
[in this window]
[in a new window]
|
Figure 4. Parasternal long-axis view shows no significant aortic stenosis, slight aortic insufficiency, and normal left ventricular dimension.
|
|
|
DISCUSSION
|
|---|
We are still searching for chromosomal abnormalities with Turner-like characteristics in this patient. Bicuspid aortic valve and coarctation of the aorta are commonly associated with Turner syndrome. A review of the up-to-date literature revealed 68 patients with aortic dilation, dissection, or rupture.3 A survey by Lin and colleagues3 detected aortic dilation with or without dissection in approximately 6% of patients with Turner syndrome. Dissection or rupture was reported in 42 out of 68 (62%) cases. Cystic medial necrosis similar to what is seen in aortic dissection in Marfan syndrome has also been reported in Turner syndrome.4 The criteria in diagnosis of aortic dilatation are not precisely known because of the lack of standards for aortic root or ascending aortic diameter in Turner syndrome. Allen and coworkers5 defined a "significant" aortic root dilation as an echocardiogram measurement greater than the 90% based on body surface area. Lin and colleagues4 considered aortic-root/descending-aorta ratios greater than 1.5 as measured by magnetic resonance imaging to be abnormal. Furthermore, aortic dilatation at the levels of the aortic annulus and sinuses of Valsalva is significantly more common in patients with a severely regurgitant bicuspid valve than in those with a functionally normal bicuspid valve.6 Marked or rapidly progressive aortic dilatation, progressing left ventricular enlargement, and unexplained episodes of chest pain are symptoms of a progressing AI and should be considered as indications for elective surgery. Elective composite aortic graft replacement has been recommended for patients with Marfan syndrome when aortic root dilation as determined by echocardiography reaches 55 mm,7 as with Lin and coworkers,4 who reported on aortic dissections in cases of Turner syndrome where the aortic root diameter was 35 mm (2.5 cm•m–2) in one patient and the ascending aorta diameter was 47 mm in another 19-year-old patient. Although the use of nomograms based on body size in adults should maximize sensitivity for aortic dilatation, 98% specificity is attained by the use of an upper normal limit of 2.1 cm•m–2 for aortic diameter at the sinuses of Valsalva in both men and women.8 The vast majority of patients with aortic dilation have an associated risk factor such as a typically bicuspid aortic valve, coarctation or systemic hypertension. These patients represent a higher risk group and should be followed up appropriately. When the ascending aorta diameter measures over 2.5–3.0 cm•m–2 in patients with both Turner syndrome and bicuspid valve, elective aortic graft replacement may be considered.
We considered a prosthetic aortic valve replacement as a first choice since this patient had primary amenorrhoea and quite a low possibility of pregnancy. We performed the aortic valve repair by cusp extension using fresh autologous pericardium because of an anatomical problem with the right coronary artery and small aortic root. Although the Ross/Konno procedure with a Dacron graft extension may be an alternative option, pathological problems, including the possibility of dilatation of the pulmonary artery root, would still remain. Bicuspid aortic valve repair for prolapsing leaflet is also an alternative method. Recently, a revival of the aortic cusp extension technique has been reported.1,2 This technique is recommended for children in whom anticoagulation and small aortic annuls could give rise to complications because of its ready availability, simplicity and excellent mid-term results.2 The long-term results are still unknown, but we believe this procedure may be a viable surgical option for those wishing to avoid a Ross or Ross/Konno procedure.
|
ACKNOWLEDGMENTS
|
|---|
The contributions of Masakiyo Sasahara MD to this report are gratefully acknowledged.
|
REFERENCES
|
|---|
- Duran CM, Gometza B, Kumar N, Gallo R, Bjornstad K. From aortic cusp extension to valve replacement with stentless pericardium. Ann Thorac Surg
1995;60(2 Sppl):S428–32.
- Kalangos A, Beghetti M, Baldovinos A, Vala D, Bichel T, Mermillod B, et al. Aortic valve repair by cusp extension with the use of fresh autologous pericardium in children with rheumatic aortic insufficiency. J Thorac Cardiovasc Surg
1999;118:225–36.[Abstract/Free Full Text]
- Lin AE, Lippe B, Rosenfeld RG. Further delineation of aortic dilation, dissection, and rupture in patients with Turner syndrome. Pediatrics
1998;102:e12.[Abstract/Free Full Text]
- Lin AE, Lippe BM, Geffner ME, Gomes A, Lois JF, Parton CW, et al. Aortic dilation, dissection, and rupture in patients with Turner syndrome. J Pediatr
1986;109:820–6.[Medline]
- Allen DB, Hendricks SA, Levy JM. Aortic dilation in Turner syndrome. J Pediatr
1986;109:302–5.[Medline]
- Hahn RT, Roman MJ, Mogtader AH, Devereux RB. Association of aortic dilation with regurgitant, stenotic and functionally normal bicuspid aortic valves. J Am Coll Cardiol
1992;19:283–8.[Abstract]
- McDonald GR, Schaff HV, Pyeritz RE, McKusick VA, Gott VL. Surgical management of patients with the Marfan syndrome and dilatation of the ascending aorta. J Thorac Cardiovasc Surg
1981;81:180–6.[Abstract]
- Roman MJ, Devereux RB, Kramer-Fox R, O’Loughbin J. Two-dimensional echocardiographic aortic root dimensions in normal children and adults. Am J Cardiol
1989;64:507–12.[Medline]
TOP
ABSTRACT
INTRODUCTION
