Medium-term follow up of pulmonary autograft aortic valve replacement: technical advances and echocardiographic follow up

BACKGROUND AND AIM OF THE STUDY: The Ross procedure (aortic valve replacement (AVR) with pulmonary autograft and pulmonary homograft replacement of pulmonary valve) was developed as a durable aortic valve substitute that avoids the need for anticoagulation and provides young patients with a long-lasting aortic valve substitute. Our seven-year follow up echocardiography data are reviewed. METHODS: Between May 1993 and March 2000, 40 adult patients (28 males, 12 females; mean age 33.3 years) underwent the Ross procedure at the Brigham and Women's Hospital for congenital aortic stenosis (n = 6), aortic insufficiency (n = 17) and mixed disease (n = 17). All patients had aortic root replacement with the pulmonary autograft and had no regurgitation after operation. Postoperative evaluation was conducted by transthoracic echocardiography, office visit and/or telephone interview. NYHA functional class, aortic and pulmonary valve function and aortic root dimensions were evaluated. RESULTS: One patient died postoperatively as a result of a low output state related to global left ventricular dysfunction. Four patients (10%) developed pulmonary homograft stenosis with a peak gradient >40mmHg; and six developed mild pulmonary stenosis. One patient had aortic insufficiency seven years postoperatively that required valve replacement. Eight patients developed mild dilatation (>37 mm) of the neoaortic root, and five of these had aortic insufficiency. One patient required transplantation at 40 months for restrictive cardiomyopathy. CONCLUSION: The Ross procedure is an effective means of AVR that can be accomplished with low perioperative morbidity and mortality if certain technical modifications are carried out. In this series of 40 patients with mid-term follow up, a significant number developed moderate pulmonary trunk stenosis, though echo characterization demonstrated good valve function.     

Exercise echocardiographic comparison of pulmonary autograft and aortic homograft replacements for aortic valve disease in adults

BACKGROUND AND AIM OF THE STUDY: The pulmonary autograft, or Ross procedure, has theoretical hemodynamic benefits over other aortic valve replacements. The hemodynamic performance of the pulmonary autograft and pulmonary homograft components of this procedure have not been well defined. METHODS: Twenty patients with pulmonary autograft replacement of the aortic valve and six with aortic homografts underwent exercise echocardiography with assessment of exercise duration, left ventricular dimensions, mass, and function. Hemodynamics at rest and maximal exercise, including Doppler gradients and effective orifice area (EOA), were measured across the pulmonary autograft and aortic homograft valves. Doppler gradients across the pulmonary homograft valves were compared to native pulmonary valve gradients at rest and maximal exercise. RESULTS: Both groups of patients had excellent self-reported and measured exercise capacity. In comparison to the aortic homograft, the pulmonary autograft had lower peak Doppler gradients across the neoaortic valve at rest (5 +/- 2 versus 11 +/- 4 mmHg; p = 0.027) and maximal exercise (10 +/- 5 versus 15 +/- 5 mmHg; p = 0.003) and larger indexed EOA. However, the Ross procedure patients had higher gradients across the pulmonary homograft both at rest (14 +/- 10 versus 3 +/- 1 mmHg; p < 0.001) and maximal exercise (25 +/- 22 versus 5 +/- 4 mmHg; p = 0.004). Two patients in the Ross procedure group had significant pulmonary homograft stenosis in short- or mid-term follow up. CONCLUSION: In comparison to aortic homograft replacement of the aortic valve, pulmonary autograft replacement has superior hemodynamics at rest and during exercise. However, the pulmonary homograft replacement may develop hemodynamically significant stenosis after the Ross procedure.     

Fate of the autograft and homograft following Ross aortic valve replacement: reoperative frequency, outcome, and management

BACKGROUND AND AIM OF THE STUDY: The optimal hemodynamic performance and potential for growth of the pulmonary autograft has led to expanded indications for the Ross aortic valve replacement (AVR) procedure in some centers. The authors' institutional mid-term experience was reviewed to assess autograft and homograft hemodynamics, growth profile of the autograft, and reoperative frequency following Ross AVR. METHODS: Between June 1993 and June 2005, 167 consecutive patients (mean age 24.9 +/- 15.5 years; range: 1 month to 61 years) underwent Ross AVR: 48% of patients were aged < 19 years. Additional procedures (n = 78) were performed in 55 patients (33%) at the time of the Ross procedure. In total, 151 patients had isolated aortic valve disease and 16 pediatric patients had more complex, multi-level left ventricular outflow tract obstruction. RESULTS: There were two early deaths (1.2%) and one late death (0.6%) over a mean follow up of 5.1 +/- 3.0 years (range: 1 month to 11 years). Actuarial survival at 10 years was 98%. In pediatric patients with Konno procedure (n = 16), the pulmonary autograft mean annulus diameter increased from 10.2 to 19.9 mm. Twelve patients underwent 12 reoperations without mortality for autograft insufficiency or an ascending aortic aneurysm at a median interval of 5 years (range: 2 to 8 years): aortic annuloplasty and ascending aorta replacement (n = 4), composite aortic root replacement (n = 7), and repair of left ventricular pseudoaneurysm (n = 1). Freedom from replacement of the pulmonary autograft was 96% at 10 years. Five of the 164 surviving patients (3%) developed significant obstruction of the pulmonary homograft and required conduit replacement at a median of four years. CONCLUSION: The Ross AVR can be performed with good mid-term results, including the pediatric age group. The potential for development of significant autograft insufficiency and homograft stenosis warrants annual follow up through the intermediate and late terms.     

An evaluation of the Ross operation in adults

BACKGROUND AND AIM OF THE STUDY: Pulmonary autograft replacement of the aortic valve (the Ross operation) is the operation of choice in infants and children. Although this procedure can offer theoretical advantages at any age, its use in adults remains controversial. METHODS: A total of 264 consecutive patients (203 males, 61 females; mean age 35.0 +/- 11.5 years; range: 18-66 years) was studied. These patients underwent the Ross operation at two institutions and were followed up for a total of 1,634 patient-years. The etiology was mainly congenital (52%), degenerative (22%), and rheumatic (8%). Among patients, 21% underwent prior aortic valve replacement. RESULTS: Thirty-day mortality was 2.3% (n = 6), and four more patients died during follow up (mean follow up 6.2 years; range 0-15.4 years). Cumulative survival at five years was 96.8%, and at 10 years was 95.4%. Eleven patients underwent reoperation on the aortic valve; this was due to progressive dilatation and aortic regurgitation in 10 cases, and to dissection of the arterial wall of the autograft in one case. Overall freedom from pulmonary homograft reoperation was 94.9% at 10 years, and for autograft reoperation was 92.9%. Estimated freedom from autograft reoperation at Harefield was 98.6% at five and 10 years, and at Rotterdam 96.0% at five years and 88.2% at 10 years (p = 0.10, Tyrone-Ware). No risk factors for early and late mortality and reoperation were detected. CONCLUSION: In this combined series, the Ross operation in adult patients resulted in excellent survival and acceptable reoperation rates. A prospective randomized trial is proposed to study whether this observation truly reflects the potential advantages of the Ross procedure, or whether it is caused by patient selection.     

Reoperations for aortic aneurysm after the Ross procedure

BACKGROUND AND AIM OF THE STUDY: Among late complications after the Ross operation, autograft dilatation is likely the most common. In order to define prevalence, consequences and management of autograft dilatation, a 10-year clinical experience was reviewed. METHODS: A total of 112 patients (mean age 29 +/- 10 years) underwent cross-sectional echocardiographic follow up. End-points of the study were freedom from autograft dilatation (diameter >4 cm, indexed as 0.21 cm/m2) and from reoperation for dilatation. Risk factors for autograft dilatation were also identified. RESULTS: There were 110 late survivors; average follow up was 5.1 +/- 1.9 years (range: 0.3 to 10.6 years). At 10 years, autograft dilatation was identified in 32 patients (29%), compatible with aortic aneurysm (>5.0 cm) in seven patients (6%). Seven of 32 patients (22%) presented moderate or greater autograft insufficiency. Ten-year freedom from dilatation was 43 +/- 8%, and from regurgitation was 75 +/- 8%. At multivariate analysis, preoperative aneurysm (p = 0.02), root replacement technique (p = 0.03) and absence of root buttressing (p = 0.04) were predictive of dilatation. Reoperation for autograft aneurysm was performed in five patients at a mean of 7.3 +/- 0.8 years after the Ross procedure, while two patients await reintervention. Two patients had root replacement and three remodeling with valve preservation (two root replacements, one sinotubular junction replacement): all survived reoperation. Ten-year freedom from root reoperation was 81 +/- 6%, and from full root replacement was 94 +/- 2%. CONCLUSION: With increasing follow up after the Ross operation, the incidences of root dilatation and reoperation are likely to rise. Graft replacement of coexisting aneurysm, avoidance of root replacement technique and the use of root-stabilization measures may reduce the prevalence of late root pathology. Early replacement of dilated autograft roots may allow preservation of the autologous pulmonary valve.     

The ross procedure for replacement of the bicuspid aortic valve: mid-term results from 55 consecutive cases

BACKGROUND AND AIM OF THE STUDY: A congenitally dysfunctioning bicuspid aortic valve may require surgical treatment in children and young adults. This retrospective study evaluated mid-term clinical results obtained with the Ross procedure in this patient group. METHODS: Between July 1994 and December 2000, 55 patients (48 males, seven females; mean age 27+/-10 years; range: 7-49 years) underwent replacement of a diseased bicuspid aortic valve (stenosis in six cases (11%); insufficiency in 36 (65%); mixed lesion in 13 (24%)) with a pulmonary autograft. Mean NYHA functional class was 1.7. Five patients (9%) had healed endocarditis and six (11%) had previous cardiac surgery. The autograft was inserted as a subcoronary implant in two cases (4%), as a root in 40 (73%), and as a cylinder in 13 (23%). The right ventricular outflow tract was reconstructed with a cryopreserved pulmonary homograft in all cases. Mean cardiopulmonary bypass and aortic cross-clamp times were 207+/-34 min and 162+/-18 min, respectively. RESULTS: No early or late deaths had occurred at a mean follow up of 31+/-19 months. Two patients (4%) were re-explored for bleeding. Four patients (7%) experienced intraoperative coronary complications which resolved without sequelae. Two-dimensional echocardiographic evaluation of neoaortic valve competence at six months revealed no evidence of aortic valve regurgitation in 46 patients (84%), trivial regurgitation in seven (13%), mild regurgitation in one patient (2%), and moderate regurgitation in one patient. The latter patient (subcoronary implant) required reoperation. At six months, the degree of regression of left ventricular mass compared (versus preoperative) was 34+/-13% (p <0.05). Three patients (5%) showed mild dilatation (>4 cm) of the neoaortic root after two years follow up. All patients are currently asymptomatic, in NYHA class I, and enjoy a normal social lifestyle. CONCLUSION: The Ross procedure may be offered as a low-risk alternative in adolescents and young adults with a bicuspid aortic valve. Although the inclusion cylinder technique might help to prevent root dilatation, continued patient evaluation with regard to root sizing, evidence of neoaortic valve degeneration and homograft dysfunction is required in the long term.     

Ross operation in children: late results.

BACKGROUND AND AIM OF THE STUDY: Although the Ross operation has become the accepted aortic valve replacement in children, the long-term fate of the pulmonary autograft valve remains unknown. To assess mid-term and late results of autograft valve durability, patient survival and valve-related morbidity, a retrospective review of patients (age range: 3 days to 17 years) having a Ross operation between November 1986 and May 2001 were reviewed. METHODS: Medical records and patient contacts with all but two of 167 current survivors of 178 consecutive patients having an aortic valve replacement as a Ross operation have been completed during the past two years. The most recent echocardiographic evaluation was reviewed for autograft valve and homograft valve function. RESULTS: Operative mortality was 4.5% (8/178), with three late deaths (two were non-valve-related) for an actuarial survival of 92+/-3% at 12 years. Actuarial freedom from autograft valve degeneration (reoperation or severe insufficiency of autograft valve or valve-related death) was 90+/-4% at 12 years. Autograft valve degeneration was not affected by technique of insertion (141 root replacement, 37 intra-aortic), aortic valve morphology (157 bicuspid or unicuspid, 26 tricuspid), or age at operation. Autograft valve degeneration was worse in patients with a primary lesion of aortic insufficiency than in those with aortic stenosis (p = 0.03). Autograft valve reoperation was required in 12 patients, with autograft valve replacement in seven. Actuarial freedom from autograft replacement was 93+/-3% at 12 years. Homograft valve replacement was required in seven patients, with actuarial freedom from replacement of 90+/-4% at 12 years. Eight additional patients have homograft valve obstruction (gradient > or =50 mmHg), and seven have severe pulmonary insufficiency. CONCLUSION: Survival and freedom from aortic valve replacement are excellent in children. Homograft valve late function remains a concern, and efforts to improve homograft durability should be encouraged.     

The Ross procedure in the acute phase of infectious endocarditis in childhood]

The Ross procedure of aortic valve replacement with a pulmonary autograft has several advantages in childhood over mechanical prostheses or homografts, especially in infectious endocarditis requiring early surgery. Between January 1997 and July 1998, 3 children with no known previous cardiac disease, aged 14 months, 10 and 11 years, had aortic valve infectious endocarditis. The causal organism was not identified in 1 case and the other two were due to staphylococcus aureus and corynebacterium diphteriae. All children had severe, rapidly progressive aortic regurgitation complicated by pulmonary oedema in the baby and systemic emboli in the two older children. Surgery was performed within 9 days, 1.5 month and 2 months after the onset of the disease. The postoperative course was uncomplicated in the 3 cases. Postoperative Doppler echocardiography showed absence of autograft dysfunction or stenosis, with the presence of pulmonary regurgitation in 1 case. Pulmonary autograft has the advantages of not requiring anticoagulation, of allowing growth of the aortic ring, of not being limited by the age of the patient and of having a low risk of degeneration and infectious endocarditis. Therefore, it seems particularly indicated for cases of complicated infectious endocarditis requiring early aortic valve replacement. The early (4.8%) and late (4.3%) mortality rates were comparable to those of other techniques and are lower than those associated with valve replacement with mechanical prostheses in cases of endocarditis (8.5% versus 40%). The secondary morbidity is 18.8% with dysfunction of the autograft and/or stenosis of the pulmonary homograft. Despite a limited follow-up, aortic valve replacement by a pulmonary homograft seems better than aortic valve replacement with a homograft or mechanical prosthesis, especially in cases of complicated infectious endocarditis requiring surgery in the acute phase. Further studies are required to confirm these encouraging results.     

应用自体肺动脉瓣置换病变主动脉瓣（附4例报告）

应用自体肺动脉瓣置换病变主动脉瓣、同种肺动脉瓣原拉重建右室流出道（Ross手术）治疗4例主动脉瓣病变患者,成功3例。1例主动脉瓣二瓣化畸形术后存在轻度主动脉瓣返流。超声心动图均提示主动脉根部及同种瓣良好。1例术中误伤自体肺动脉瓣,改机械瓣置换。认为用自体肺动脉瓣置换病变主动脉瓣效果满意,术中预防自体肺动脉瓣损伤和主动脉瓣返流是手术成功的关键,同种肺动脉瓣原位重建右室流出道可为常规选择的管道。     

Saccular aneurysm of pulmonary autograft after the Ross procedure.

The Ross procedure, which includes removal of the malformed aortic valve and replacement of a pulmonary autograft in the aortic position, has increased the number of available treatment options. Recently, dilatation of the autograft pulmonary root after the Ross procedure has been reported as a complication. We report a patient with bicuspid aortic valve malformations and aortic annulus dilatation, who had a saccular-form, true-type, aneurysm in the pulmonary autograft seven months after the Ross procedure. These changes have not been described so far as complication. Pathologically, marked mucoid degeneration was noted in the tunica media of the aneurysm, as well as in the original aortic root. These findings may suggest similar pathological characteristics between the aorta and pulmonary arteries. Hence, the surgical risks accompanying vascular characteristics in patients with congenital aortic valve malformations should be considered.     

Echocardiographic follow-up after Ross procedure in 100 patients

The Ross procedure could provide an ideal aortic valve replacement method in children and young adults. We evaluated midterm echocardiographic results to assess pulmonary homograft function as well as pulmonary autograft dimensions and function. In all, 105 patients (26 women and 79 men) underwent the Ross procedure; median age at implant was 29 years. All patients underwent free root replacement. Transvalvular gradients and autograft dimensions were measured at 3 levels (annulus, sinuses of Valsalva, and proximal aorta) at discharge, at 6 months, and annually thereafter. Perioperative mortality was 4.7%. The mean period for echocardiographic follow-up in 100 patients was 32.7 months (range 0.5 to 7 years), during which 4 noncardiac-related deaths occurred. Two patients underwent late reintervention. No moderate or severe regurgitation was recorded. There was 1 case of mild homograft regurgitation and 4 of mild autograft regurgitation at late follow-up. Autograft peak gradients were low and reproducible (5 +/- 2.8 mm Hg at discharge vs 5.5 +/- 3.5 mm Hg at last follow-up, p = NS). Homograft peak gradients increased significantly without severe obstruction (7.8 +/- 5.7 mm Hg at discharge vs 15.8 +/- 9.2 mm Hg at last follow-up). The diameter of the autograft annulus was stable during follow-up, whereas autograft dimensions at sinuses and proximal aorta increased significantly. One group of patients was identified with sinus diameter increases >20% (group A). The 90 remaining patients were classified into group B. The only parameter significantly different between the 2 groups was the sinus diameters measured at discharge (1.74 cm/m2 (group A) vs 1.92 cm/m2 (group B); p = 0.036). In 100 patients and with echocardiographic follow-up for up to 7 years, the Ross procedure showed excellent results. For 10% of patients, we observed a 20% dilation of sinus diameters, but in only 3 patients (3%) was this beyond the upper normal limit.     

Annuloplasty and aortoplasty as modifications of the Ross procedure for the correction of geometric mismatch

BACKGROUND AND AIM OF THE STUDY: Pulmonary autograft replacement of the aortic valve (the Ross procedure) is reliable and durable; however, geometric mismatch of the autograft and systemic outflow tract may lead to poor results. Manipulation of the aortic annulus and sinotubular diameters to match the autograft can prevent geometric mismatch, and improve results. METHODS: Annuloplasty and/or aortoplasty were combined with the Ross procedure in 26 of 44 patients (median age 42.5 years; range: 3 days to 62 years) undergoing surgery between April 1994 and July 1998. Plication of the aortic annulus at either two or three of the commissures was done in five cases, aortic annulus fixation with an external pericardial pledget incorporated in the proximal suture line in 12 cases, and tailoring aortoplasty in nine patients. RESULTS: There was one operative death. Two patients required reoperation; one for progressive autograft dysfunction and one for homograft dysfunction. Annular fixation was performed on the patient requiring reoperation for autograft dysfunction. Doppler echocardiography during the follow up (median 9 months; range: 1-50 months) revealed 10 patients with trace 1+ and one patient with 2+ aortic insufficiency. Trace 1+ and 2+ aortic stenosis were present in one patient each. None of the patients undergoing commissural plication had significant regurgitation or stenosis. Both patients with stenosis underwent annular fixation. Aortoplasty was associated with 1+ insufficiency in two patients. CONCLUSION: Prevention of geometric mismatch between the autograft and systemic outflow tract at the annulus and sinotubular junction by plication techniques allows better performance of the autograft, and extends the Ross procedure to patients who otherwise may be unable to undergo such surgery. Fixation may provide similar benefit, but appears to be more susceptible to insufficiency and stenosis.     

Intermediate-term results of Medtronic freestyle valve for right ventricular outflow tract reconstruction in the Ross procedure

BACKGROUND AND AIM OF THE STUDY: The Ross procedure has become the first choice for aortic valve replacement in children and young adults at many institutions. Since 1997, a lack of availability of homograft valves in Turkey has prompted the use of alternative substitutes for right ventricular outflow tract (RVOT) reconstruction during the Ross procedure. METHODS: Before April 2005, among 20 patients (age range: 14 months to 45 years) at the present authors' institution, the Ross procedure was performed in 14 and a Ross-Konno procedure in six. Sixteen patients underwent RVOT repair using alternative methods for homograft valve replacement. Fourteen patients received a Medtronic Freestyle valve and one patient a Medtronic Contegra bovine jugular vein conduit. An autologous RVOT repair was used in one patient. Ten of the Medtronic Freestyle valve patients were aged <16 years. In all patients who received a Medtronic Freestyle valve echocardiographic evaluations were conducted shortly after surgery and during follow up. RESULTS: There was no early mortality. One patient died from pneumonia after six months, and another (asymptomatic) patient died suddenly at 34 months after surgery. Before hospital discharge the mean peak pressure gradient across the Freestyle valve was 12.1 +/- 11.0 mmHg, and this increased to 24.1 +/- 20.0 mmHg after a mean follow up of 51.2 +/- 6.9 months (range: 6 to 101 months) (p <0.002). Mild pulmonary regurgitation was seen in two patients. One asymptomatic adult patient was reoperated on at another center because of a 60-mmHg echocardiographic peak gradient at four years postoperatively. CONCLUSION: Although long-term follow up is required to explain the durability of the Medtronic Freestyle valve, the present results show that the valve can be used with intermediate-term success in the Ross procedure - and even in children as an alternative - if homograft valves are not available.     

Aortic valve replacement with a pulmonary autograft (the Ross operation) in adult and pediatric patients. A preliminary study

INTRODUCTION AND OBJECTIVES: Aortic valve replacement with the patients own pulmonary autograft (the Ross procedure) is by now, the best surgical method for the replacement of the diseased aortic valve in certain groups of patients, this is particularly true for young adults and children or neonates with complex left ventricular outflow tract obstructions. The procedure was described by Donald Ross in 1967, and many years have passed. So in view of the accumulated experience the indications have extended to a wide group of patients which include children, neonates and young adults with formal contraindications for anticoagulation. In this publication we present our experience and our preliminary results in a group of fifteen patients which include adult and pediatric. MATERIAL AND METHODS: In six patients the etiology of lesion was congenital and in the remainder nine the valve had an acquired lesion. Two patients had an open heart procedure before this operation both of them to relieve an obstruction to the left ventricular outflow tract. In this group of patients the Ross procedure was carried out inserting the pulmonary autograft in the aortic position as a total root which was always reconstructed with cryopreserved pulmonary homograft, the mean homograft diameter was 26.1 +/- 4 mm (19-35). RESULTS: In all patients a transesophageal echocardiogram was performed in the operating room and postoperative, 1 or 2 months later. Only in one patient a mild aortic regurgitation was detected, no significant transaortic or transpulmonary gradients were detected postoperative. One patient was reoperated for bleeding in the postoperative course, there was no hospital mortality in our group and all the patients had an uneventful postoperative period. In the short term follow-up (41-155 days). All the patients are free of anticoagulant therapy, all them are in New York Heart Association Functional Class I. CONCLUSIONS: The patients presented in this publication which include adult and pediatric, are the first group of patients operated in our country with some excellent preliminary results. We hope that this procedure will become popular and that other surgical groups will adopt it as another surgical tool to replace a diseased aortic valve.     

Acceptance and versatility of the Ross procedure

During the 31 years since the initial Ross procedure, data have been collected that have been helpful in assessing long-term performance of the autograft. The ongoing study of the pulmonary autograft supports the use of the Ross procedure in young patients, in females of childbearing age, and in patients with congenital aortic stenosis and complex left ventricular outflow tract obstruction. We continue to see little or no thromboembolism despite no anticoagulation therapy. The remarkable ability of the autograft to grow in children is extremely beneficial. Additionally, excellent results have been obtained in some series for the treatment of endocarditis. Recently, the autograft has performed similarly to a normal aortic valve under high stress. Changes in implantation techniques transitioning from subcoronary to root replacement and performing annular narrowing has decreased the incidence of early regurgitation. A potential for an immune response with resulting pulmonary stenosis and possible early explanation of the pulmonary homograft exists; however, overall, results of the Ross procedure are excellent and highly reproducible.     

Morquio's syndrome: severe aortic regurgitation and late pulmonary autograft failure

A 44-year-old woman underwent a Ross procedure for severe aortic regurgitation at the age of 32 years. She had been diagnosed in childhood with spondyloepiphyseal dysplasia and a bicuspid aortic valve. At surgery, a tricuspid aortic valve with chondroid metaplasia and fibrosis was reported. Biochemical and genetic evaluation in this patient confirmed the diagnosis of mucopolysaccharidosis IV type B (MPS IV), otherwise known as Morquio's syndrome. This autosomal-recessive inherited syndrome is characterized by the accumulation of keratin sulfate in connective tissue and various other organs. Cardiac (notably valvular) involvement has been well described in the literature. To the authors' knowledge, this is the first reported case of valve replacement or Ross procedure for this condition. This woman presented 12 years after her initial valve surgery with progressive dyspnea. Echocardiographic examination revealed severe pulmonic autograft regurgitation without a dilated aortic root, together with severe stenosis of the pulmonary homograft. It is postulated that the underlying metabolic abnormality may have led to progressive pulmonary autograft failure and to accelerated dysfunction and stenosis of the pulmonary homograft. It is likely that a mechanical prosthesis would have been a better therapeutic option if the preoperative diagnosis of MPS IV had been made.     

Echocardiographic follow-up study of the Ross procedure in older versus younger patients

OBJECTIVES: This study was designed to determine echocardiographic follow-up results of the Ross procedure in older adult patients with aortic valve disease. BACKGROUND: The excellent long-term results of the Ross procedure from several institutions have indicated that the pulmonary autograft may be the best available substitute for the diseased aortic valve in children and adolescents. The advantages of this operation include optimal hemodynamics and elimination of thromboembolic complications. These features may benefit older adult patients as well. METHODS: We reviewed data from 49 consecutive patients who had a Ross procedure between 1991 and 1996. Preoperative and postoperative Doppler echocardiographic studies were available for 44 patients (22 men, 22 women; mean [+/-SD] age 36 +/- 14 years) who were grouped into <40 (n = 25) and > or =40 years old (n = 19). Measurements included left ventricular diastolic volume (LVDV), mass, and ejection fraction (EF); a peak pressure gradient across autograft in the aortic position and homograft in the pulmonary position; and valvular regurgitation. RESULTS: The mean length of echocardiographic follow-up was 36 +/- 16 months. Postoperatively, there was a reduction in LVDV and left ventricular mass in both age groups: 153 +/- 99 mL to 111 +/- 72 mL (P =.015) and 210 +/- 93 g to 152 +/- 54 g (P =.002) for younger patients, 174 +/- 115 mL to 126 +/- 43 mL (P =.17) and 233 +/- 71 g to 215 +/- 65 g (P =.19) for older patients. No significant change in EF was noted in the younger age group. However, in the older age group a significant decrease to EF <25% was found in 2 patients 1 year after surgery. Moderate autograft regurgitation was also detected in 2 patients: 1 in each age group. Pressure gradients across the autograft remained within the normal range in both age groups. Two younger patients had severe homograft stenosis with peak gradients of 100 and 62 mm Hg. The older patients did not demonstrate homograft dysfunction. CONCLUSIONS: The Ross procedure can be performed in selected older adults with aortic valve disease and provides durable valves in both aortic and pulmonic positions for at least 3 years after surgery but may result in less favorable left ventricular remodeling compared with that in the younger patients. Further follow-up will be necessary to determine the long-term outcome of the Ross procedure in this older adult patient population.     

The ross operation in 225 patients: a five-year experience in aortic root replacement.

BACKGROUND AND AIM OF THE STUDY: The Ross operation, introduced more than 30 years ago, has recently undergone several modifications to improve both technical feasibility and results. At the authors' institution, the Ross operation, performed as root replacement in all cases, was commenced in February 1995. METHODS: A total of 225 patients (177 males, 48 females; mean age 39+/-15 years; range: 2 to 67 years) were operated on up to December 2000. Aortic regurgitation was present in 80 patients, stenosis in 69, and combined disease in 73; prostheses were replaced in three patients. Combined procedures were performed in 51 patients. Nine patients had active endocarditis. Follow up was 98% complete and totaled 471 patient-years. RESULTS: There was no early mortality, and no thromboembolic or hemorrhagic events. Complications included prolonged ventilation in two patients, perioperative myocardial infarction in three, pacemaker implants in three and perioperative bleeding in six. One patient died at 25 months from hemoptysis, and one at five months of unknown cause. In the long term, four patients required reoperation due to autograft regurgitation (one autograft repair, three autograft replacements). Routine aortic annulus support, a lowered threshold in replacing all dilated ascending aorta and keeping the autograft short to the level of the sinotubular junction seems to have prevented further autograft failure. Pulmonary homograft stenosis led to reoperation in one patient. Six patients with elevated gradients are currently under observation. Echocardiography revealed autograft median peak gradients of 5.1+/-2.8 mmHg, pulmonary homograft gradients of 14.2+/-11.5 mmHg, and no significant regurgitation, except in one additional patient with recently diagnosed aortic insufficiency (grade >2). CONCLUSION: Mid-term excellent hemodynamic results, low morbidity and reoperation requirement support the evolved root replacement technique and justify its further utilization.     

Effect of prior aortic valve intervention on results of the Ross operation

BACKGROUND AND AIM OF THE STUDY: Patient-related factors, aortic insufficiency, bicuspid aortic valve, aortic annulus dilatation, ascending aortic dilatation or aneurysm, and aortic valve endocarditis have been suggested as affecting the results of the Ross operation. The study aim was to assess the impact of prior aortic valve intervention on early and late results of a Ross operation. METHODS: A total of 399 patients who underwent surgery between August 1986 and September 2000 were reviewed retrospectively. The patients were grouped as: no prior aortic valve intervention (NOAVI, n = 219); prior aortic valvuloplasty (AVP, n = 106); prior balloon aortic valvuloplasty (AVB, n = 40); and prior aortic valve replacement (AVR, n = 34). Details of operative and late mortality, autograft valve function, and homograft valve function were analyzed. RESULTS: Operative mortality was higher for AVB (10%; three deaths in neonates) than the other groups (from 2.3% to 5.9%) (p = 0.084). Freedom from autograft valve degeneration, defined as severe autograft valve insufficiency, non-endocarditis autograft valve reoperation or valve-related death, ranged from 93 +/- 3% for AVP to 76 +/- 8% for NOAVI at 10 years (p = 0.43). Freedom from homograft reoperation in the pulmonary position was 100% for AVB at six years, and 99 +/- 1% for AVP, 82 +/- 8% for NOAVI, and 70 +/- 13% for AVR at 10 years (p = 0.0026). CONCLUSION: There appears to be no significant difference between patients with and without prior aortic valve surgery, with respect to operative mortality or late autograft function. However, patients with prior AVR appear to have a significantly higher homograft reoperation rate after a Ross operation, the reasons for which are uncertain.     

Serial echocardiographic assessment of neo-aortic regurgitation and root dimensions after the modified Ross procedure

BACKGROUND AND AIM OF THE STUDY: Concern exists regarding progressive root dilatation after the modified Ross procedure. The present prospective echocardiographic study aimed to provide further insight into neo-aortic regurgitation (nAR) and neoaortic root dimensions over time in adult Rotterdam Ross root patients, and to study potential risk factors for nAR and dilatation. METHODS: All Rotterdam Ross patients aged > or = 16 years at surgery were subjected to a prospective biennial standardized echocardiographic protocol. Analysis over time of nAR according to the jet length and jet diameter method, autograft annulus and sinotubular junction (STJ) diameters was carried out using a multilevel linear model in 90 patients who had two or more echocardiographic measurements (mean 5; range 2-9; total 458) up to 14 years (mean 7 years) after surgery. RESULTS: The mean (+/- SE) initial postoperative jet length nAR was grade 0.9 +/- 0.09, and the annual increase 0.1 +/- 0.02 (p < 0.001). Initial annulus and STJ diameters were 25 +/- 0.5 mm and 36 +/- 0.6 mm, while annual increases were 0.4 +/- 0.07 mm and 0.5 +/- 0.09 mm, respectively (p < 0.001). Patients who eventually underwent an autograft reoperation (n = 10) had significantly greater initial nAR and greater progression of nAR, and a greater initial annulus diameter. The annual annulus and STJ diameter increase was greater in patients who underwent autograft reoperation. Compared to freestanding root replacement, patients with inclusion cylinder aortic root replacement had smaller initial annulus and STJ diameters that did not increase over time. Female gender was associated with a greater initial jet length and jet diameter nAR and a greater increase over time in jet diameter nAR. Preoperative aortic regurgitation or combined aortic stenosis and regurgitation were associated with greater initial annulus and STJ diameters. Neither bicuspid valve disease, patient age, preoperative ascending aorta aneurysm, prior aortic valve surgery nor hypertension had an effect on initial or progression of nAR, annulus, and STJ diameter. CONCLUSION: The annual increase in nAR and root dimensions is small, but persistent, after autograft aortic root replacement in adults, and further reoperations should be anticipated. Use of the inclusion cylinder root replacement technique seems to prevent neo-aortic dilatation.