Successful repair of supravalvular aortic stenosis using Steinberg 3 sinuses reconstruction; report of a case

Steinberg method is a modification of Doty extended aortoplasty for supravalvular aortic stenosis (SAS). This modification entails placement of an additional patch in the left coronary sinus. A 3-year-old boy was diagnosed as SAS with aortic valvular stenosis. He was noticed a systolic murmur from 1 month after his birth. Echocardiography showed left ventricular hypertrophy, and pressure gradient of 80 mmHg was measured between the ascending aorta and the left ventricle. Cardiac catheterization revealed severe aortic stenosis at the sino-tubular (ST) junction. We adopted Steinberg 3 sinuses reconstruction. After this operation, there was no pressure gradient at ST junction although aortic valvular stenosis remained and mild aortic valve regurgitation newly developed. As this method can produce a symmetric aortic root, it may reduce aortic valve deformity especially on the left coronary cusp.     

Reconstruction of the ascending aorta and aortic root: experience in 45 consecutive patients

BACKGROUND: Patients with aneurysms of the ascending aorta or aortic root may have associated aortic insufficiency (AI). We reviewed our experience with aortic root remodeling and reconstruction of the sino-tubular junction. METHODS: Forty-five patients were operated on between July 1995 and September 1998. Transesophageal echocardiography showed AI grade III or IV in 15 patients. Twenty-seven patients had replacement of all three sinuses, 10 of one or two sinuses. Reconstruction of the sino-tubular junction alone was performed in 8 patients. RESULTS: There was one death at 28 days. Perioperative transesophageal echocardiography showed no or discrete AI in all patients. There has been one aortic valve replacement at day 4 postoperatively for cusp repair failure. Transesophageal echocardiography in 40 patients at a mean time of 12.5 months showed no progression of AI in 38 patients, and a grade II in 2. Clinical follow-up averaged 14.5 months. There have been three late, not procedure-related deaths. Thirty-six patients are in New York Heart Association functional class I. There have been no cases of endocarditis. CONCLUSIONS: Aortic remodeling is successful in eliminating AI in patients with aortic root disease with minimal mortality and morbidity. Early echocardiography (1 year) has shown no progression of AI in 95% of cases.     

New technique of aortic root reconstruction with aortic valve annuloplasty in ascending aortic aneurysm

We describe a new technique of aortic valve conservation for ascending aortic aneurysm with aortic valvular insufficiency. This technique allows a total anatomic aortic root reconstruction associated with an aortic annuloplasty preventing late annulus dilation and reoperation. Preliminary results demonstrate the feasibility and the safety of this new original procedure.     

Surgical treatment of valvular and supravalvular aortic stenosis in homozygous familial hypercholesterolemia

A 61-year-old male with homozygous familial hypercholesterolemia presented with dyspnea and syncope. He had been treated with low-density lipoprotein apheresis for 26 years. Echocardiography and computed tomography showed severe valvular and supravalvular aortic stenosis. Computed tomography and cardiac catheterization revealed a severely calcified narrowed aortic root and an occlusion in the proximal right coronary artery. During surgery, the ascending aorta was replaced under deep hypothermic circulatory arrest without aortic cross-clamping. After that, the aortic root from the annulus to the sino-tubular junction was enlarged with a two-ply bovine pericardial patch. An aortic valve replacement with a 17 mm mechanical valve and coronary artery bypass grafting to the right coronary artery were performed. The patient recovered from the surgery without any cerebrovascular complications.     

保留主动脉瓣主动脉根部置换术治疗主动脉根部瘤的疗效分析

目的评价使用保留主动脉瓣主动脉根部置换术治疗主动脉根部瘤的临床应用疗效。方法 2001年2月至2010年9月阜外心血管病医院对60例主动脉根部瘤患者行保留主动脉瓣主动脉根部置换术,其中男44例,女16例;年龄9～64(37.2±13.0)岁。主动脉夹层15例,升主动脉瘤10例,马方综合征25例,主动脉瓣二瓣化畸形2例。行主动脉根部重建术53例,主动脉瓣瓣叶再植术7例。比较术前、术后心功能及主动脉瓣反流程度等指标。结果全组中无手术死亡和严重并发症发生,呼吸机使用时间中位数为13(2～1 110)h,住ICU时间1～18(2.7±2.5)d。术后复查超声心动图提示:主动脉瓣反流程度均明显减轻,仅3例为中大量反流,其余无反流或微少量反流。术后随访2～122(61.5±35.9)个月,随访56例,失访4例,随访期间死亡9例,生存率83.9%(47/56)。2例因主动脉瓣反流分别于术后13个月和14个月再次入院行主动脉瓣置换术。47例患者心功能较术前明显改善,心功能Ⅰ级35例(74.4%),Ⅱ级8例(17.0%)。免于主动脉瓣中重度反流40例(85.1%)。结论保留瓣叶的主动脉根部置换术治疗主动脉根部瘤的远期疗效满意,瓣膜相关并发症发生率低。     

Aortic valve-sparing operations in patients with aneurysms of the aortic root or ascending aorta

BACKGROUND: Aortic valve-sparing operations are an alternative to aortic root replacement in patients with aortic root aneurysms, or aortic valve replacement and supracoronary replacement of the ascending aorta in patients with ascending aorta aneurysms and dilated sinotubular junctions with consequent aortic insufficiency. METHODS: From 1988 to 2001, 230 patients underwent aortic valve-sparing operations for aortic root aneurysms (151 patients) or ascending aortic aneurysms with aortic insufficiency (79 patients). Two types of aortic valve-sparing operations were performed in patients with aortic root aneurysms: reimplantation of the aortic valve and remodeling of the aortic root. Mean follow-up was 3.8 +/- 2.8 years. RESULTS: Patients with aortic root aneurysms were younger, had less severe aortic insufficiency, less extensive vascular disease, and better left ventricular function than patients with ascending aorta aneurysms. The 8-year survival was 83% +/- 5% for the first group and 36% +/- 14% for the second. The freedom from aortic valve reoperation at 8 years was 99% +/- 1% for the first group and 97% +/- 2% for the second. In patients who had aortic root aneurysms, 3 developed severe aortic insufficiency (AI), and 15 developed moderate AI, for an 8-year freedom from significant AI of 67% +/- 7%. But freedom from AI was 90% +/- 3% after the technique of reimplantation, and 55% +/- 6% after the technique of remodeling (p = 0.02). In patients with ascending aortic aneurysms, the freedom from AI greater than 2+ at 8 years was 67% +/- 11%. CONCLUSIONS: The long-term results of aortic valve sparing for aortic root aneurysms are excellent, and reimplantation of the aortic valve may provide a more stable repair of the aortic valve than remodeling of the aortic root.     

Aortic valve reconstruction in the young infants and children

Considering the structure and function of the aortic root, changes in the aortic valve leaflets and changes in the geometry of the aortic root are the two primary causes of aortic valve dysfunction. In adults, aortic valve sparing reconstruction has a long history beginning in the 1970s, where tensor fascia was used for leaflet repair in patients with isolated aortic regurgitation and ascending aortic replacement was used in patients with ascending aortic aneurysms or aortic ectasia. Subsequent progress in the 1980s and 1990s led to pericardial leaflet replacement and aortic root re-implantation and remodeling. However, it has not been until the last decade that these concepts and techniques have been applied in younger patients focusing on the conotruncus, valvar apparatus, sino-tubular junction, and ascending aorta.     

An experience of a case with perforation of an aneurysm in the anterior mitral cusp developing after aortic valve replacement.

Mitral valve aneurysm is a rare disease and in Japan, cases of perforation which is considered to be mainly caused by infectious endocarditis is usually only encountered through case reports. We experienced a case who received aortic valve replacement and mitral valve annuloplasty for combined valvular heart disease of aortic insufficiency and mitral insufficiency followed by mitral valve replacement for severe mitral valve regurgitation subsequent to perforation of the anterior mitral cusp, leading to recovery.     

Replacement of ascending aorta with aortic valve reimplantation: midterm results

Objective: Aneurysms of the aortic root lead to aortic valve incompetence due to dilatation of the sinotubular junction and annuloaortic ectasia. Reimplantation of the native, structurally intact aortic valve within a Dacron tube graft corrects annular ectasia as well as dilatation of sinotubular junction and aortic sinuses. Durability of this valve repair with respect to increased mechanical stress on valve cusps is discussed controversially and is yet unknown. Methods: Since 7/93, replacement of the ascending aorta with repair of the aortic valve was performed in 48 patients (34 male, 14 female; 47±20 years) with aortic insufficiency and aneurysm of the aortic root. Fifteen patients (31%) had Marfan's syndrome and five patients (10%) had an aortic dissection type A (two acute, three chronic). In 11 patients (23%), concomitant replacement of the aortic arch was necessary utilizing elephant trunk technique in two patients. Additionally, one patient required mitral valve repair and two other patients coronary artery bypass grafts. Clinical and echocardiographic follow-up was performed in 6–12 month intervals for a cumulative study period of 100 patient years. Results: There were no operative deaths. Two patients (4%) died 5 and 20 months postoperatively. One additional patient experienced a TIA within the first postoperative week. Three patients (6%) with an early postoperative aortic insufficiency (AI)>1 required aortic valve replacement after 9, 11, and 14 months due to progressive AI. In these patients, distortion of the aortic root geometry led to valve incompetence. All other patients have no or mild aortic insufficiency. The repair now remains stable for up to 63 months (mean 25±18 months). Other valve related complications did not occur. Conclusions: Our results demonstrate that this type of aortic valve repair achieves excellent results in selected patients. Perfect coaptation of valve cusps during the repair with no or only trace AI at initial echocardiography seems to be essential for durability.     

Aortic prosthetic ring annuloplasty: a useful adjunct to a standardized aortic valve-sparing procedure?

OBJECTIVE: Dilation of aortic annulus, sinuses of Valsalva, and sinotubular junction (STJ) diameters are the characteristic lesions of aortic root aneurysm. The remodeling technique reduces STJ diameter and creates three neosinuses of Valsalva. Alternatively, the reimplantation technique reduces both annulus and STJ diameters to the detriment of aortic root dynamics. Although the remodeling technique is recognized as the most physiological valve-sparing procedure, aortic annulus dilation may jeopardize its results. A standardized approach that combines an external subvalvular aortic prosthetic ring annuloplasty with the remodeling technique is suggested. METHODS: Eighty-three patients underwent an elective aortic root remodeling procedure, either isolated (group 1, n=34) or combined with an external subvalvular aortic prosthetic ring annuloplasty (group 2, n=49). Preoperative aortic regurgitation was 1.59+/-1.1 (group 1) and 1.97+/-1.3 (group 2) (NS). The aortic annulus was more dilated in group 2 than in group 1 (27+/-2.77 mm vs 26.4+/-2.3 mm, p<0.01). Residual aortic regurgitation > or =grade II was the conversion criteria for aortic valve replacement. RESULTS: Operative mortality was 3.6% (n=3). Intraoperative conversion for valve replacement was 32.7% in group 1 (n=11) versus 4.2% in group 2 (n=2) (p<0.001). In group 1, preoperative annulus diameter was larger for converted than for valve-spared patients (27.6+/-1.7 mm vs 25.2+/-1.5 mm, p<0.02). In group 2, implanted aortic ring significantly reduced annulus diameter (20.6+/-1.8 mm) without significant aortic valve gradient (8.3+/-3 mmHg). Follow-up was 17.2+/-13.4 months (group 1) and 10.41+/-7.95 months (group 2). Reoperation for recurrent aortic regurgitation was 13% in group 1 (n=3) versus 4.2% in group 2 (n=2). Echocardiographic follow-up found residual aortic regurgitation < or =grade I in 17 patients in group 1 (90%) versus 43 patients in group 2 (95.5%) and of grade II in two patients in group 1 (10%) and two patients in group 2 (4.5%). CONCLUSION: The addition of external aortic prosthetic ring annuloplasty improves the remodeling technique's operative reproducibility and short-term results. Therefore, its use as a systematical adjunct to the remodeling procedure is suggested. However, further long-term evaluation comparing this valve-sparing procedure to composite graft replacement should define the best surgical strategy for aortic root aneurysm.     

Surgery for calcific aortic root stenosis in homozygous familial hypercholesterolemia.

A 35-year-old female with homozygous familial hyperlipidemia (IIa) was referred to our hospital for an operation against supravalvular and valvular aortic stenosis. She had been treated with low-density lipoprotein apheresis for 20 years, and total cholesterol ranged between 200 and 400 mg/dl under this treatment. She had undergone percutaneous coronary intervention for ostial stenosis of the right coronary artery three times since the age of 19. Unenhanced three-dimensional computed tomography showed supravalvular stenosis, funnelling and heavily calcified aorta. An operation was performed under deep hypothermic circulatory arrest without aortic cross clamping. After the ascending aorta had been replaced with a one-branched vascular graft, arterial perfusion was resumed. The stenosed ascending aorta was resected at the sinotubular junction. Because the aortic root was still extremely small, the noncoronary sinus and the commissure between left and right coronary cusp were incised, and the aortic root was enlarged with linguiform vascular-graft patches. A 21-mm mechanical valve was implanted. The postoperative course was uneventful.     

Endoscopic observation following aortic cusp plication

In valve-sparing operations for aortic root aneurysms, the dilated aortic root is replaced by a vascular graft. However, cusp disorders remain in some cases. We observed closed cusps endoscopically, and an improvement in the cusp coaptation was seen following plication. One of our cases of aortic valve plication is reported here. A rigid thoracoscope and cannula for cardioplegia delivery were inserted from the distal end of the aortic graft, and the graft was inflated with cardioplegic solution, providing an endoscopic view of the closed aortic cusps. We believe that endoscopic observation of closing cusps in this way may help surgeons to learn and master the techniques used in cusp repair more rapidly.     

Endovascular stent-grafting through the aortic arch: an alternative approach for distal arch aortic aneurysm

BACKGROUND: Endovascular stent-grafting is an innovative procedure; we have developed a novel approach to treat distal arch aortic aneurysm through a small incision in the aortic arch. METHODS: Eight patients with thoracic aortic aneurysms were treated with an endovascular stent-graft that was introduced into the thoracic aorta through a small incision in the aortic arch. Of these patients, 7 had distal arch aortic aneurysms, and 1 had chronic aortic dissection of Stanford type B. Four of these patients had received concomitant coronary artery bypass grafting, and 1 patient had undergone tricuspid valvular annuloplasty. The stent-graft was introduced into the distal arch aorta and descending aorta through a small incision in the aortic arch, under selective cerebral perfusion and hypothermic circulatory arrest. RESULTS: The selective cerebral perfusion time ranged from 52 to 86 minutes (mean, 68 minutes) and the operating time from 289 to 422 minutes (mean, 318 minutes). There was no endoluminal leakage into the aneurysm. Seven patients survived and were discharged, but 1 patient suffered a cerebral infarction and died during the follow-up period. CONCLUSIONS: Placing an endovascular stent-graft through the aortic arch is an acceptable alternative treatment for distal arch aortic aneurysms.     

Effects of ascending aorta replacement on aortic root dilatation.

OBJECTIVE: Because of an increase of aortic root wall stress, prosthetic replacement of the ascending aorta might be a risk factor for the progressive increase of the aortic root dimension. Aim of the present study was to evaluate the aortic root diameter change and the progression of aortic valve regurgitation late after ascending aorta replacement for different ethiology. METHODS: Sixty-three late survivors after supracoronary ascending aortic replacement were evaluated. Forty-one patients were operated on for acute aortic dissecting aneurysm (group I) and 22 for chronic atherosclerotic non-dissecting aneurysm (group II). Aortic root diameter and aortic valve regurgitation were assessed echocardiografically after a mean follow-up of 63+/-31 months and were compared with those early after surgery. RESULTS: Seven patients of group I (17%) needed reoperation for aortic root dilatation or dissection. Twenty-five percent of the patients (15 of group I and 1 of group II) showed at least a 10% increase in aortic root diameter at follow-up (46.8+/-6.1 vs. 38.1+/-6.1mm, P<0.0001). Aortic root diameter increased almost exclusively in patients operated on for acute dissecting aneurysm. A significant worsening of aortic valve insufficiency with time was evident only in patients operated on for acute dissecting aneurysm with an higher incidence in those with progressive root dilatation. CONCLUSIONS: Both the increase of aortic root diameter and the progressive worsening of aortic valve insufficiency seem to justify a more aggressive treatment of the aortic root at the time of surgery for acute aortic dissecting aneurysm but not for chronic atherosclerotic non-dissecting aneurysms.     

Aortic root dilatation may alter the dimensions of the valve leaflets

Objective: Valve-sparing surgery can be used in patients with dilated aortic roots and aortic insufficiency (AI) but has not become a common practice, in part because the spared valve may be incompetent. Our goal was to study how the dimensions of the aortic root and leaflets have changed in such patients. Methods: Fourteen patients with dilated aortic root and AI were examined by transesophageal echocardiography. The annulus diameter, sinotubular junction (STJ) diameter, sinus height, leaflet free-edge length, and leaflet height were measured. Correlations among these dimensions and with the AI grades were explored. Measurements were also made in 19 normal human aortic valves from silicone molds. Results: There was no evident change in the average diameter of the annulus between the normal valves and those in the dilated aortic roots. The STJ diameter was obviously increased in the dilated aortic roots; the aortic sinuses also appeared to be taller and the leaflets larger than normal. The leaflet free-edge length, the leaflet height, and the sinus height were found to increase with the dilated STJ diameter. The degree of AI was not found to correlate well with any of the dimensions measured. Conclusions: The dimensions of the leaflets may change parallel to aortic root dilatation with AI. Therefore, during valve sparing, it may be necessary to correct both the dilatation of the root and the leaflet free-edge length to achieve a competent valve.     

Aortic valve sparing operations

Aortic valve sparing operations were developed to preserve the aortic valve in patients with ascending aortic aneurysm and aortic insufficiency or patients with aortic root aneurysm. There are 2 types of aortic valve sparing operations, remodeling of the aortic root and reimplantation of the aortic valve. The author believes that remodeling of the aortic root is more appropriate for older patients with ascending aortic aneurysm, dilated aortic sinuses, and normal aortic annulus, whereas reimplantation of the aortic valve is more appropriate for young patients with aortic root aneurysm in whom dilation of the aortic annulus is commonly associated. Although remodeling of the aortic root has been extensively used in patients with aortic root aneurysm, the long-term results are somewhat inferior to reimplantation in most series. The late results of aortic valve sparing operations have been excellent, and these operations have become an important addition to the surgical armamentarium to treat patients with proximal aortic aneurysms.     

Recurrence of aortic insufficiency after aortic root remodeling with valve preservation

Background. Aortic root remodeling (ARR) has recently been proposed for patients with aortic aneurysms and valve insufficiency (AI). To define factors associated with a favorable functional outcome, a review of the mid-term results with ARR was undertaken.

Methods. Between March 1994 and October 1997, 17 consecutive patients (11 men, 6 women), aged 57 ± 11 years (range 35–71), had elective ARR for aortic aneurysm with or without annuloaortic ectasia (13), sinus of Valsalva aneurysm (3), or chronic aortic dissection (1). Moderate or severe AI was present in 11 patients (65%). Preoperative aortic root diameter was 58 ± 5 mm (range 51–70). ARR involved replacement of all three aortic sinuses and coronary button reimplantation, using grafts with a mean diameter of 28 ± 2 mm (range 24–30).

Results. There was one early death (6%) due to multiple organ failure. Survivors were followed for 16 ± 12 months (range 1–44). Actuarial 3-year survival was 94% ± 6%. Discharge echocardiogram showed a decrease in AI in all patients: AI was absent in 11 (69%) and mild in 5 (31%). Recurrence of moderate or severe AI after a mean of 16 ± 9 months (range 9–28) was noted in 6 patients (37%), 3 of whom had no AI at discharge. Five of 6 patients required aortic valve replacement. Comparison of demographic and operative variables showed that severe preoperative AI (67% vs 20%, p = 0.06), annuloaortic ectasia (100% vs 20%, p = 0.002), and cystic medial necrosis (100% vs 20%, p = 0.002) were significantly more prevalent in patients developing severe AI at follow-up. The 10 patients (63%) with absent AI showed durable competence of the valve and relief from symptoms at follow-up.

Conclusions. Despite early restoration of valve competence, AI may recur and progress after ARR at medium-term follow-up in a proportion of patients. The severity of preoperative AI and the nature of aortic root disease may negatively influence the durability of repair. Continued observation of results with ARR appears mandatory to identify the appropriate surgical candidates.     

Aortic valve sparing operations: an update

Background. Aortic valve sparing operations in patients with ascending aorta and/or aortic root aneurysms have been performed for a decade in our institution. Initially only patients with normal aortic valve leaflets had these operations, but more recently we utilized them in patients with prolapse of a single leaflet and in those with a bicuspid aortic valve. This article is an update on the clinical results of these operations.

Methods. From May 1988 to December 1997, 126 patients with ascending aorta and/or aortic root aneurysms and aortic insufficiency underwent replacement of the ascending aorta with reconstruction of the aortic root and preservation of the native aortic valve. There were 85 men and 41 women, with a mean age of 54 years (range, 14 to 84). Thirty-two patients had the Marfan syndrome; 17 patients had acute and 10 had chronic type A aortic dissection; 23 had a transverse arch aneurysm; 26 had coronary artery disease, and 8 had mitral regurgitation. The aortic valve sparing operation consisted of simple adjustment of the sinotubular junction in 33 patients, adjustment of the sinotubular junction and replacement of one or more aortic sinuses in 60, and reimplantation of the aortic valve in a tubular Dacron (C.R. Bard, Haverhill, PA) graft in 33. Fifteen patients also had repair of aortic leaflet prolapse. Only 4 patients had a bicuspid aortic valve.

Results. There were 3 operative deaths due to cardiac failure. Patients were followed from 2 to 117 months, with a mean of 31. There were 11 late deaths: 7 cardiovascular and 4 from unrelated causes. The actuarial survival was 72 ± 8% at 7 years. Two patients required aortic valve replacement; the freedom from aortic valve replacement was 97 ± 2% at 7 years. Doppler echocardiography revealed absent, trivial or mild aortic insufficiency in most patients; only 9 patients had moderate aortic insufficiency.

Conclusions. Aortic valve sparing operations are feasible in most patients with ascending aorta and/or aortic root aneurysms who have normal or near normal aortic leaflets. The functional results of the repaired aortic valve are excellent, and the repair appears to be durable.     

主动脉根部CTA影像评估在主动脉瓣成形术中的应用价值

目的探索主动脉根部CT血管造影(CTA)在术前评估中的应用,并与二维超声数据对比,评价两者与术中外科测量数据的相关程度。方法回顾性分析2018年1月至2020年8月期间,在我院行主动脉瓣成形术的53例主动脉瓣病变患者的临床资料,其中男38例、女15例,年龄10～77(42.9±18.3)岁。收集术前经胸二维超声和主动脉根部CTA对主动脉根部测量的数据,包括主动脉瓣环(AVA)、主动脉窦(Sinus)、窦管交界(STJ)。通过与术中实际测量的数据比较,做一致性分析。结果术前心脏超声AVA测量值和术前CT AVA测量值与术中AVA测量值均呈正相关(P<0.001)。与术前心脏超声AVA测量值相比[相关系数(ρ)=0.74,均方误差(MSE)=12.78],术前CT AVA测量值保持了较高准确性,以及与术中AVA测量值的一致性(ρ=0.95,MSE=2.72)。在与术中AVA测量值的相关系数方面,术前CT高于经胸二维超声(P<0.001)。结论和经胸二维超声相比,主动脉根部CTA应用于主动脉瓣成形的术前评估数据,与外科术中实际测量的数据具有更高的一致性和准确性。     

Aortic valve sparing operations: a review

Aortic valve sparing operations were developed to preserve the native aortic valve during surgery for aortic root aneurysm as well as surgery for ascending aortic aneurysms with associated aortic insufficiency. There are basically two types of aortic valve sparing oprations: remodeling of the aortic root and reimplantation of the aortic valve. These operations have been performed for over two decades and the clinical outcomes have been excellent in experienced hands. Although remodeling of the aortic root is physiologically superior to reimplantation of the aortic valve, long-term follow-up suggests that the latter is associated with lower risk of developing aortic insufficiency. Failure of remodeling of the aortic root is often due to dilatation of the aortic annulus. Thus, this type of aortic valve sparing should be reserved for older patients with ascending aortic aneurysm and normal aortic annulus whereas reimplantation of the aortic valve is more appropriate for young patients with inherited disorders that cause aortic root aneurysms. This article summarizes the published experience with these two operations. They are no longer experimental procedures and should be part of the surgical armamentarium to treat patients with aortic root aneurysm and ascending aortic aneurysms with associated aortic insufficiency.