Outcomes following aortic valve procedures in 201 complex congenital heart disease cases—results from the UK National Audit

Abstract OBJECTIVES Some patients with complex congenital heart disease (cCHD) also require aortic valve (AoV) procedures. These cases are considered high risk but their outcome has not been well characterized. We aim to describe these scenarios in the current practice, and provide outcome data for counselling and decision-making. METHODS This was a retrospective study using the UK National Congenital Heart Disease Audit data on cCHD patients undergoing aortic valve replacement, balloon dilation (balloon aortic valvuloplasty) or surgical repair (surgical aortic valve repair) between 2000 and 2012. Coarsened exact matching was used to pair cCHD with patients undergoing AoV procedures for isolated valve disease. RESULTS A total of 201 patients with a varied spectrum of cCHD undergoing 242 procedures were included, median age 9.4 years (1 day–65 years). Procedure types were: balloon aortic valvuloplasty (n = 31, 13%), surgical aortic valve repair (n = 57, 24%) and aortic valve replacement (n = 154, 63%). Mortality at 30 days was higher in neonates (21.8% vs 5.3%, P = 0.02). Survival at 10 years was 83.1%, freedom from aortic valve replacement 83.8% and freedom from balloon aortic valvuloplasty/surgical aortic valve repair 86.3%. Neonatal age (P < 0.001), single ventricle (P = 0.08), concomitant Fontan/Glenn (P = 0.002) or aortic arch procedures (0.02) were associated with higher mortality. cCHD patients had lower survival at 30 days (93% vs 100%, P = 0.003) and at 10 years (86.4% vs 96.1%, P = 0.005) compared to matched isolated AoV disease patients. CONCLUSIONS AoV procedures in cCHD can be performed with good results outside infancy, but with higher mortality than in isolated AoV disease. Neonates and patients with single ventricle defects, especially those undergoing concomitant Fontan/Glenn, have worse outcomes.

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Abstract 17049: Outcomes of Bioprosthetic and Mechanical Aortic Valve Replacement in Patients With Congenital Heart Disease

Circulation ◽

10.1161/circ.142.suppl_3.17049 ◽

2020 ◽

Vol 142 (Suppl_3) ◽

Author(s):

Reilly D Hobbs ◽

Megan Schultz ◽

Catherine Wagner ◽

Richard G Ohye ◽

Edward Bove ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Aortic Valve ◽

Aortic Valve Replacement ◽

Valve Replacement ◽

Congenital Heart ◽

Valve Repair ◽

Long Term Outcomes ◽

Long Term Mortality

Introduction: Aortic valve replacement (AVR) is required in patients with congenital heart disease who are not candidates for Ross procedure or valve repair. The long-term outcomes of prosthetic AVR in patients with congenital heart disease are unknown. Methods: A single-institution, retrospective review of all patients treated with mechanical (M) and bioprosthetic (BP) AVR between 12/1985 to 2/2020 was undertaken. Results: One-hundred and ninety-three patients underwent BP (n=134) or M (n=59) AVR. Indications for AVR were insufficiency (91/193), stenosis (61/194), mixed (38/193), aneurysm (2/193), and unknown (1/193). Patients receiving M valves were more likely to be younger (18.5 years versus 29.0 years, p < 0.01) and less likely to require subsequent valve reintervention (p < 0.01). Average time to first reintervention was 76 and 71 months in the BP and M groups respectively (p = 0.785). Survival between the M and BP groups was similar (p = 0.120). Twenty-four patients who received a M valve and thirteen patients who received a BP valve died during the study period. Causes of death were cardiac (16/37), stroke (3/37), non-cardiac (4/37), and unknown (14/37). Conclusions: Long-term freedom from death or valve reintervention is poor in congenital heart disease patients requiring AVR. Survival is similar between patients treated with M and BP valves, however, BP valves more frequently required reintervention during the study period. Both BP and M valves are associated with significant long-term mortality. These results highlight the need for the development of robust aortic valve repair techniques so that AVR can be avoided.

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Shedding more light on the use of mechanical aortic valve replacement in patients with congenital heart disease

Journal of Thoracic and Cardiovascular Surgery ◽

10.1016/j.jtcvs.2018.08.047 ◽

2019 ◽

Vol 157 (1) ◽

pp. 341

Author(s):

Paul J. Chai

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Aortic Valve ◽

Aortic Valve Replacement ◽

Valve Replacement ◽

Congenital Heart ◽

Mechanical Aortic Valve

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Aortic Valve Repair for Adult Congenital Heart Disease

Circulation ◽

10.1161/circ.102.suppl_3.iii-40 ◽

2000 ◽

Vol 102 (suppl_3) ◽

Cited By ~ 1

Author(s):

Vivek Rao ◽

Glen S. Van Arsdell ◽

Tirone E. David ◽

Anthony Azakie ◽

William G. Williams

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Aortic Valve ◽

Congenital Heart ◽

Septal Defect ◽

Aortic Valve Repair ◽

Subaortic Stenosis ◽

Valve Repair ◽

Aortic Valve Leaflets

Background —Aortic valve–preserving procedures have resulted in excellent outcomes in selected patients, particularly those with normal aortic valve leaflets and dilated aortic roots. However, several congenital heart lesions are associated with abnormal aortic valve leaflets. The long-term results of aortic valve repair for these lesions are not well defined. Methods and Results —We reviewed the clinical records of 54 adult (age >18 years) patients who underwent repair of congenital abnormalities of the aortic valve between 1976 and September 1999. Follow-up data were available on 52 (96%) patients (mean 50±67 months, range 1 to 266). Patients underwent repair at a mean age of 34±14 years with associated diagnoses of subaortic stenosis (n=10), ventricular septal defect with prolapsing aortic valve (n=17), bicuspid aortic valve (n=23), sinus of Valsalva aneurysm (n=10), and bacterial endocarditis (n=2). There was 1 operative death (1.9%) and 3 late deaths. Survival at 5 and 10 years was 98±2% and 74±12%, respectively. Freedom from reoperation was 74±9% and 51±15% at 5 and 10 years, respectively. The presence of a ventricular septal defect predicted failure of valve repair (59% versus 22%, P =0.01). A bicuspid aortic valve, subaortic stenosis, or the requirement for mitral valve surgery did not affect outcomes. Conclusions —Aortic valve repair in adult patients with congenital heart disease can be performed with minimal morbidity and mortality rates. The medium-term results of repair are acceptable, regardless of valvular or associated pathology. However, only 31 patients (57%) demonstrated long-term competence of the aortic valve, suggesting that most adult patients with congenital aortic valve disease will eventually require aortic valve replacement.

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Outcomes after mechanical aortic valve replacement in children and young adults with congenital heart disease

Journal of Thoracic and Cardiovascular Surgery ◽

10.1016/j.jtcvs.2018.08.077 ◽

2019 ◽

Vol 157 (1) ◽

pp. 329-340 ◽

Cited By ~ 11

Author(s):

Patrick O. Myers ◽

Suyog A. Mokashi ◽

Edward Horgan ◽

Michele Borisuk ◽

John E. Mayer ◽

...

Keyword(s):

Congenital Heart Disease ◽

Young Adults ◽

Heart Disease ◽

Aortic Valve ◽

Aortic Valve Replacement ◽

Valve Replacement ◽

Congenital Heart ◽

Mechanical Aortic Valve ◽

Children And Young Adults

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ADAPT-treated pericardium for aortic valve reconstruction in congenital heart disease: histological analysis of a series of human explants

European Journal of Cardio-Thoracic Surgery ◽

10.1093/ejcts/ezz228 ◽

2019 ◽

Vol 56 (6) ◽

pp. 1170-1177

Author(s):

Sarah Nordmeyer ◽

Johanna Kretzschmar ◽

Peter Murin ◽

Mi-Young Cho ◽

Rudi Foth ◽

...

Keyword(s):

Extracellular Matrix ◽

Congenital Heart Disease ◽

Heart Disease ◽

Aortic Valve ◽

Aortic Valve Stenosis ◽

Congenital Heart ◽

Aortic Valve Repair ◽

Valve Repair ◽

Extracellular Matrix Components ◽

Matrix Components

Abstract OBJECTIVES Different types of patch materials are used for aortic valve repair in children with congenital aortic valve disease to avoid early valve replacement. CardioCel© (Admedus, Toowong, QLD, Australia) consists of bovine pericardium treated with the ADAPT method (Admedus’ proprietary tissue engineering process). METHODS Our goal was to describe tissue reactions in 12 explanted aortic valve leaflet specimens (augmented or replaced with CardioCel patch material) (11 explanted surgically, 1 autopsy). Explantation was performed during reoperation after aortic valve repair, necessitated by aortic valve stenosis in 5, aortic valve insufficiency in 2, combined aortic valve lesions in 3 patients and endocarditis in 1 patient. One patient died of sudden left heart failure 28 months after aortic valve repair. At the last documented follow-up of this patient at 22 months, he showed mild aortic valve stenosis and insufficiency. Implantation time (time between implantation and explantation) of CardioCel patches was a median of 25 (range 11–47) months. Explants were examined using a uniform protocol with methylmethacrylate and/or paraffin embedding after fixation in formalin. Besides standard histological staining, von Kossa (for identification of calcifications) and immunohistochemical stains were applied with antibodies specific for muscular, inflammatory and connective tissue component antigens. Findings regarding the extent of appositional growth on top of the patch consisting of fibroblasts and extracellular matrix components, calcification, and inflammation were rated using a 4-grade scale (G0 no/G1 few/G2 moderate/G3 massive). RESULTS Superficial endothelialization was demonstrated in all patients by immunohistochemical analysis. Nine specimens showed mild inflammatory cell infiltration (G1) (G2: n = 3). Significant appositional growth on top of the patch due to addition of fibroblasts and extracellular matrix components, was seen in all specimens (G1: n = 1; G2: n = 7; G3: n = 4). Ten of 12 samples with implant times of 23 months or longer revealed calcifications (G1: n = 6; G2: n = 3; G3: n = 1). Two specimens with the shortest implant times (11 and 20 months) showed no calcifications (G0). Thrombus apposition with granulocyte infiltration was demonstrated in the specimen of the patient with endocarditis. CONCLUSIONS In our cohort, all CardioCel patches used for aortic valve repair in patients with congenital heart disease demonstrated appositional growth of fibroblasts and extracellular matrix components, and calcification after an implant time of at least 23 months.

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