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2.
Cardiovasc Res ; 119(3): 857-866, 2023 05 02.
Article in English | MEDLINE | ID: mdl-35727948

ABSTRACT

AIMS: The present study aims to characterize the genetic risk architecture of bicuspid aortic valve (BAV) disease, the most common congenital heart defect. METHODS AND RESULTS: We carried out a genome-wide association study (GWAS) including 2236 BAV patients and 11 604 controls. This led to the identification of a new risk locus for BAV on chromosome 3q29. The single nucleotide polymorphism rs2550262 was genome-wide significant BAV associated (P = 3.49 × 10-08) and was replicated in an independent case-control sample. The risk locus encodes a deleterious missense variant in MUC4 (p.Ala4821Ser), a gene that is involved in epithelial-to-mesenchymal transformation. Mechanistical studies in zebrafish revealed that loss of Muc4 led to a delay in cardiac valvular development suggesting that loss of MUC4 may also play a role in aortic valve malformation. The GWAS also confirmed previously reported BAV risk loci at PALMD (P = 3.97 × 10-16), GATA4 (P = 1.61 × 10-09), and TEX41 (P = 7.68 × 10-04). In addition, the genetic BAV architecture was examined beyond the single-marker level revealing that a substantial fraction of BAV heritability is polygenic and ∼20% of the observed heritability can be explained by our GWAS data. Furthermore, we used the largest human single-cell atlas for foetal gene expression and show that the transcriptome profile in endothelial cells is a major source contributing to BAV pathology. CONCLUSION: Our study provides a deeper understanding of the genetic risk architecture of BAV formation on the single marker and polygenic level.


Subject(s)
Bicuspid Aortic Valve Disease , Heart Valve Diseases , Animals , Humans , Bicuspid Aortic Valve Disease/metabolism , Bicuspid Aortic Valve Disease/pathology , Aortic Valve/pathology , Heart Valve Diseases/pathology , Genome-Wide Association Study , Zebrafish/genetics , Endothelial Cells/metabolism
3.
J Thorac Cardiovasc Surg ; 166(2): 422-431.e3, 2023 Aug.
Article in English | MEDLINE | ID: mdl-34657715

ABSTRACT

OBJECTIVES: The study aim was to evaluate long-term results after anatomic restoration of the aortic root. METHODS: During an 18-year period, a total of 669 patients underwent valve-sparing root repair (aneurysm 554, dissection 115) using selective sinus replacement. None/trivial, mild, moderate, and severe (grades 3+ and 4+) insufficiency were present in 57, 146, 204, and 262 patients, respectively. RESULTS: The anatomic repair was adjusted to the existing aortic annulus diameter, which was 27.0 ± 3.0 mm on average. Replacement of 1, 2, or 3 sinuses of Valsalva was performed in 209, 234, and 226 patients, respectively. Altogether, 454 additional procedures on the cusps were performed, mostly as cusp patch plasty with pericardium (210). Thirty-day mortality was 0.6%. The mean follow-up duration was 7.1 ± 4.1 years (range, 0.01-19.1 years). The estimated freedom from relevant aortic insufficiency grade 3+ or greater (15 events) was 98% ± 1%, 97% ± 1%, and 94% ± 3% at 5, 10, and 15 years, respectively. On echocardiogram, no patient revealed a considerable change of the form or size of the repaired root, which was confirmed radiologically in 160 patients who received computed tomography angiography for any reason. Multivariate logistic regression analysis identified cusp prolapse/pseudo-prolapse as the only independent risk factor for the development of recurrent insufficiency grade 2+ or greater (41 occurrences), with a hazard ratio of 3.258 (95% confidence interval, 1.658-6.403; P = .001). An association between aortic annulus size and functional results could not be demonstrated. CONCLUSIONS: Patient-tailored root repair using isolated sinus replacement offers excellent functional long-term results regardless of underlying root pathology or annulus size. Aortic cusp pathology was decisive for long-term valve function.


Subject(s)
Aorta, Thoracic , Aortic Valve Insufficiency , Humans , Aorta, Thoracic/surgery , Aortic Valve/diagnostic imaging , Aortic Valve/surgery , Aortic Valve/pathology , Treatment Outcome , Aorta/diagnostic imaging , Aorta/surgery , Aortic Valve Insufficiency/diagnostic imaging , Aortic Valve Insufficiency/surgery , Aortic Valve Insufficiency/pathology , Prolapse , Retrospective Studies
4.
Article in English | MEDLINE | ID: mdl-35461710

ABSTRACT

OBJECTIVES: To evaluate the results after selective sinus replacement (SSR) for aortic root remodeling in bicuspid aortopathy. METHODS: Among 662 patients who underwent root repair using SSR between 2005 and 2020, there were 114 with bicuspid aortopathy. SSR was performed either as an isolated procedure (31) or as an adjunct to aortic valve repair (83) and was adjusted to the existing aortic annulus diameter rather than a downsized diameter. In valves with asymmetrical commissural orientation, the repair aimed for the achievement of a 180°-commissural orientation. RESULTS: Abolishment of aortic insufficiency (AI) ≥2+ using root repair alone was only possible in 2 patients with acute-dissection-related AI, yet isolated root repair was also performed in 29 further patients with no/mild AI. All remaining patients with AI ≥2+ presented cusp-related regurgitation and necessitated an additional valve repair. During the mean follow-up of 91 months (range, 13-196), a relevant valve defect (AI ≥3+ in 8, stenosis in 2) occurred in 10 patients (all after combined repair) resulting in an estimated freedom from a relevant aortic valve defect and/or reoperation of 96 ± 2%, 89 ± 4%, and 82 ± 6% at 5, 10, and 12 years, respectively. Echocardiographically, no patient revealed a considerable change of form or size of the repaired root nor was any root reintervention necessary. CONCLUSIONS: Patient-tailored root repair using SSR is a very effective and durable valve-sparing approach for bicuspid aortopathy. Aortic cusp repair is decisive for both abolishment of AI in bicuspid aortopathy and for the functional durability of the repaired aortic valve.

5.
Eur J Cardiothorac Surg ; 62(5)2022 10 04.
Article in English | MEDLINE | ID: mdl-35385074

ABSTRACT

OBJECTIVES: We evaluate the mid-term results of mitral valve (MV) repair with patch augmentation of the posterior leaflet in secondary mitral regurgitation. METHODS: Patients were included after diagnosis of a severe symptomatic secondary MV insufficiency with grade III and IV according to the Carpentier classification IIIb. Indication for a patch augmentation technique was a dilatation of the left ventricle leading to a displacement of the papillary muscles, causing restricted leaflet motion and a marked leaflet tenting height. Data were collected prospectively between December 2011 and March 2020. RESULTS: In total, 174 patients (mean age: 65 ± 12 years) received an MV repair with patch augmentation of the posterior leaflet and a true-sized remodelling annuloplasty (mean size 30.8 mm). Causes of the MV incompetence were dilatative cardiomyopathy in 126 patients and ischaemic myocardial disease in 48 patients. Concomitant bypass surgery was performed in 28 patients, and the tricuspid valve was repaired in 68 patients. The mean follow-up was 40 ± 28.2 months. There was no 30-day mortality. In-hospital mortality was 1.2% (n = 2); late mortality was 10.9% (n = 19). At 8 years, overall survival was 62.48%, freedom from moderate or severe recurrent mitral regurgitation was 91.9% and freedom from reoperation due to MV insufficiency was 97.1%. CONCLUSIONS: Augmentation of the posterior MV leaflet in addition to remodelling annuloplasty is a safe and reproducible mitral reconstruction technique that renders sustainable MV competence.


Subject(s)
Cardiac Surgical Procedures , Mitral Valve Annuloplasty , Mitral Valve Insufficiency , Humans , Middle Aged , Aged , Mitral Valve Insufficiency/etiology , Mitral Valve Insufficiency/surgery , Mitral Valve Insufficiency/diagnosis , Mitral Valve/diagnostic imaging , Mitral Valve/surgery , Papillary Muscles/surgery , Cardiac Surgical Procedures/methods , Tricuspid Valve , Dilatation, Pathologic , Treatment Outcome , Mitral Valve Annuloplasty/methods
9.
Aorta (Stamford) ; 9(2): 60-66, 2021 Apr.
Article in English | MEDLINE | ID: mdl-34619801

ABSTRACT

BACKGROUND: The aim of the study was to assess the indications, surgical strategies, and outcomes after reoperative aortic arch surgery performed generally under mild hypothermia. METHODS: Ninety consecutive patients (60 males, mean age, 55 ± 16 years) underwent open reoperative aortic arch surgery after previous cardiac aortic surgery. The indications included chronic-progressive arch aneurysm (55.5%), chronic aortic dissection (17.8%), contained arch rupture (16.7%), and graft infection (10%). The reoperation was performed through a repeat sternotomy (96%) or clamshell thoracotomy (4%) using antegrade cerebral perfusion under mild systemic hypothermia (28.9 ± 2.5°C) in all except three patients. RESULTS: The surgery comprised hemiarch or total arch replacement in 41 (46%) and 49 (54%) patients, respectively. The distal extension included classic or frozen elephant trunk technique, each in 12 patients, and total descending aorta replacement in 4 patients. Operative mortality was 6 (6.7%) among all patients, with age identified as the only independent predictor of operative mortality (p = 0.05). Permanent and transient neurologic deficits occurred in 1% and 9% of the patients, respectively. Estimated survival at 8 years was 59 ± 8% with advanced heart failure predictive for late mortality (p = 0.014). Freedom from second reoperation or intervention on the aorta was 78 ± 6% at 8 years, with most of these events occurring downstream in patients with chronic degenerative aneurysms. CONCLUSION: Aortic arch reoperations performed using antegrade cerebral perfusion under mild systemic hypothermia offer favorable operative outcomes with an exceptionally low rate of neurologic morbidity without any difference between hemiarch and complex arch procedures.

10.
Eur J Cardiothorac Surg ; 59(4): 758-764, 2021 04 29.
Article in English | MEDLINE | ID: mdl-33284973

ABSTRACT

OBJECTIVES: The study objective was to describe the technique and outcomes of original coronary ostial slide plasty in patients with anomalous coronary artery origin (ACAO) localized in the aneurysmal ascending aorta (AA) being replaced because of its isolated pathology and otherwise non-pathological aortic root. METHODS: A total of 23 patients (median age 52 years) with the ascending phenotype of proximal aorta aneurysm and ACAO of at least 1 coronary artery localized in the AA being replaced underwent ostial slide plasty to transpose the ACAO to the respective sinus of Valsalva and, consequently, to allow an AA replacement with placement of the proximal anastomosis at the level of the sinotubular junction (STJ). In 15 patients, the aortic valve was bicuspid, and all but 3 patients presented with a relevant valve defect. In addition to remodelling the STJs (all patients), valve-sparing repair or replacement was performed in 12 and 8 patients, respectively. RESULTS: No patient died during the entire follow-up (median 72, range 3-183 months). One patient required replacement of a recurrently insufficient valve that was repaired primarily using cusp patch plasty, but there were no further cardiac reoperations nor any re-interventions on the proximal aorta, aortic valve and/or coronary artery ostia. Two patients received peripheral coronary stents (8 and 7 years after surgery, respectively) due to coronary heart disease. CONCLUSIONS: Transposition of the ACAO from the replaced AA into the normal sinus of Valsalva using the ostial slide plasty offers a simple and safe surgical option enabling a recreation of a durable STJ at the level of the anastomosis between the root and the aortic graft.


Subject(s)
Aortic Aneurysm , Aortic Valve Insufficiency , Aorta/surgery , Aortic Aneurysm/diagnostic imaging , Aortic Aneurysm/surgery , Aortic Valve/diagnostic imaging , Aortic Valve/surgery , Humans , Middle Aged , Treatment Outcome
11.
Asian Cardiovasc Thorac Ann ; 29(8): 801-803, 2021 Oct.
Article in English | MEDLINE | ID: mdl-33307714

ABSTRACT

We report a rare case of complex coronary fistula that contained two aneurysms and was connected to the descending aorta. The fistula was also associated with further aortic pathologies necessitating surgery. Preoperative computed tomography angiography, which revealed an abnormal vessel communication, was crucial for choosing the appropriate operative strategy. During a one-stage procedure comprising closure of both arterial (aortic and coronary) fistula origins and repair of the aortic valve and thoracic aorta, the coronary origin of the fistula was ligated and the frozen elephant trunk technique was used to close the aortic origin.


Subject(s)
Aortic Aneurysm, Thoracic , Blood Vessel Prosthesis Implantation , Fistula , Aorta, Thoracic/diagnostic imaging , Aorta, Thoracic/surgery , Aortic Aneurysm, Thoracic/surgery , Blood Vessel Prosthesis , Humans , Stents
12.
13.
J Thorac Cardiovasc Surg ; 159(2): 365-371.e1, 2020 Feb.
Article in English | MEDLINE | ID: mdl-30922634

ABSTRACT

OBJECTIVES: The aim of the study was to evaluate the surgical and neurological outcomes after aortic arch surgery using unilateral cerebral perfusion. METHODS: Between June 2004 and February 2017, a total of 1000 patients (mean age 63 ± 12; range, 14-88 years) with nonacutely dissected aortic pathology (aneurysm, porcelain aorta, chronic dissection, infection, and injury in 89.1%, 4.9%, 4.1%, 1.6%, and 0.3%, respectively) underwent aortic arch surgery using unilateral cerebral perfusion for brain protection using mild hypothermia. A previous neurological event with residuals was documented in 3.6% of the patients and 12.2% had received previous cardiovascular surgery. The surgery comprised total/subtotal arch repair (with involvement of at least 1 supra-aortic artery) or hemiarch replacement in 346 and 654 patients, respectively. The aortic valve was replaced in 521 (including 190 valve composite grafts) and repaired in 380 patients (284 valve-sparing root repairs). RESULTS: The unilateral cerebral perfusion (mean duration 23.3 ± 17.2; range, 6-105 minutes) was performed via cannulated common carotid or innominate artery and aimed for a pressure-controlled (70-100 mm Hg) flow (mean flow, 1.4 ± 0.3 L/min; mean pressure, 90.1 ± 20.1 mm Hg) at a constant blood temperature of 28°C for ensuring the patency of collateral pathways. The circulatory arrest of the lower body (mean duration 18.4 ± 9.9 minutes) was performed at a rectal temperature of 31.2 ± 1.8°C. Early (30-day) and in-hospital mortality was 1.3% and 2.1%, respectively; the rates of permanent neurological deficit and transient neurological dysfunctions were 1.0% and 4.9%, respectively. CONCLUSIONS: Unilateral cerebral perfusion performed in the described conditions is highly effective for cerebral protection in aortic arch surgery.


Subject(s)
Aorta, Thoracic/surgery , Brain Ischemia/prevention & control , Cerebrovascular Circulation/physiology , Perfusion/methods , Adolescent , Adult , Aged , Aged, 80 and over , Aortic Diseases/surgery , Female , Humans , Male , Middle Aged , Postoperative Complications/prevention & control , Vascular Surgical Procedures/adverse effects , Young Adult
14.
Gen Thorac Cardiovasc Surg ; 68(5): 530-533, 2020 May.
Article in English | MEDLINE | ID: mdl-30868369

ABSTRACT

Anatomical aortic root restoration in a patient with a giant aneurysm of the right Sinus of Valsalva accompanied by severe AI and compression of the right-ventricular outflow is described and the entire case series (5) is reported.


Subject(s)
Aortic Aneurysm/surgery , Aortic Valve Insufficiency/surgery , Sinus of Valsalva/surgery , Aortic Aneurysm/complications , Aortic Valve Insufficiency/complications , Heart Ventricles/pathology , Humans , Male , Middle Aged
16.
J Thorac Cardiovasc Surg ; 159(4): 1176-1184.e1, 2020 04.
Article in English | MEDLINE | ID: mdl-31128903

ABSTRACT

OBJECTIVES: To evaluate long-term results after valve-sparing surgery of acutely dissected aortic root in which a restoration of root anatomy adjusted to the existing aortic annulus diameter, rather than any kind of downsizing, was performed. METHODS: Among 286 consecutive patients operated on because of type A aortic dissection during the last 17 years were 100 patients (35.0%), mean age 63 ± 13 years (range, 29-88 years), in whom an anatomical restoration of the involved root was performed. Twenty-eight patients presented with severe (3+ or 4+), 37 with mild to moderate (2+), and 24 with mild (1+) insufficiency. In 5 patients, the valve was bicuspid. In all patients, a curative root repair with replacement of all dissected aortic wall was performed, containing a selective replacement of pathologic/dissected aortic sinuses, which were 1, 2, or all 3 sinuses in 62, 32, and 6 patients, respectively. Concomitant cusp repair was necessary in 18 patients. RESULTS: Thirty-day mortality was 1.0%. Survival was estimated starting with surgery and was 89.4 ± 3.4% (95% confidence interval, 80.5-94.4) and 68.8 ± 6.4% (95% confidence interval, 54.4-79.5) at 5 and 12 years, respectively. No patient required reoperation on the aortic root and/or valve during the follow-up period of 70 ± 50 (range, 3-202) months. A freedom from any aortic valve/root reintervention and/or relevant (>2+) aortic insufficiency at 12 years was 100%. CONCLUSIONS: Selective replacement of pathologic sinuses offers an anatomophysiological albeit curative restoration of acutely dissected aortic root resulting in excellent and durable outcomes in selected patients with acute aortic dissection.


Subject(s)
Aortic Aneurysm, Thoracic/surgery , Aortic Dissection/surgery , Aortic Valve/surgery , Plastic Surgery Procedures/methods , Vascular Surgical Procedures/methods , Acute Disease , Adult , Aged , Aged, 80 and over , Female , Humans , Male , Middle Aged , Severity of Illness Index , Survival Rate , Treatment Outcome
18.
Ann Thorac Surg ; 108(1): 115-121, 2019 07.
Article in English | MEDLINE | ID: mdl-30690022

ABSTRACT

BACKGROUND: The aim of the study was to evaluate operative and long-term results after acute type A aorta dissection (AAAD) operation, in which complete resection of all dissected aortic segments (curative repair) was achieved. METHODS: Among 205 consecutive patients operated on between 2002 and 2014 because of AAAD were 88 patients (42.9%), in whom the dissection did not extend into the downstream aorta. The distal extension of the dissection ended before the origin of the innominate artery in 50 patients of the study cohort (56.8%) or extended throughout the arch, necessitating a total/subtotal arch replacement to achieve a curative distal repair in 38 remaining patients (43.2%). The aortic root was involved in 52 patients (59.1%) and was repaired using valve-sparing repair (31) or replacement with a valve composite graft (21). Combination of root and open arch surgery was reported in 46 patients (52.3%). RESULTS: Thirty-day and in-hospital mortalities were 3.4% and 5.7%, respectively. Survival was estimated starting with the operation and was 81.9% ± 4.5% and 56.6% ± 8.7% at 5 and 10 years, respectively. No patient required reoperation on the aortic root and/or distal thoracoabdominal aorta; however 2 cardiac reoperations were unrelated to the primary surgical procedure. Moreover, the freedom of aortic and/or sudden/unknown death was 100%. CONCLUSIONS: Curative aortic repair can be achieved in a relevant share of AAAD patients and is mostly limited by the distal extension of dissection. This kind of repair is advisable, whenever possible, because it can provide very low risk of aortic complications and/or reoperations over time.


Subject(s)
Aorta/surgery , Aortic Aneurysm/surgery , Aortic Dissection/surgery , Vascular Surgical Procedures/methods , Acute Disease , Adult , Aged , Aged, 80 and over , Aortic Dissection/diagnostic imaging , Aortic Dissection/mortality , Aortic Aneurysm/diagnostic imaging , Aortic Aneurysm/mortality , Female , Humans , Male , Middle Aged , Morbidity , Reoperation , Retrospective Studies , Suture Techniques
19.
Eur J Cardiothorac Surg ; 55(2): 351-357, 2019 02 01.
Article in English | MEDLINE | ID: mdl-30085032

ABSTRACT

OBJECTIVES: The aim of the study was to evaluate the incidences of innominate artery (IA) involvement in aortic arch pathology necessitating surgery, the surgical strategies and the operative results. METHODS: Among the 366 patients who underwent total/subtotal arch replacement because of non-acutely dissected aortic pathology between 2004 and June 2017, there were 46 (12.6%) patients (29 males; mean age 69 ± 10, range 35-84 years) with IA involvement. Pathologies necessitating replacement of the IA were chronic aneurysm including progression of chronic dissection, severe atherosclerosis with or without an aneurysmatic dilatation, and inflammatory vasculitis in 34, 11 and 1 patient, respectively. All data were collected prospectively, and intention-to-treat analysis was performed. RESULTS: All patients underwent total/subtotal aortic arch replacement using unilateral cerebral perfusion (mean duration 44.6 ± 15.7 min) under mild hypothermia (30.6 ± .4°C). In addition to arch and IA replacement, repair of one or two further supra-aortic arteries was performed in 20 and 23 patients, respectively. One patient underwent complete thoracic aorta replacement via clamshell thoracotomy. The aortic valve, which was the most frequent object of concomitant surgery, was replaced in 18 (including 3 valve composite-grafts) and repaired in 20 (18 valve-sparing root repairs) patients. Early (30-day and/or in-hospital) mortality was 0. The rate of permanent neurological deficit was 2.2% (1 patient with a huge, partially thrombosed arch and innominate aneurysm). Transient neurological dysfunctions such as agitation were observed in 6 patients. CONCLUSIONS: Involvement of the IA in aortic arch surgery is not infrequent, and its concomitant replacement using well-considered cannulation, perfusion and surgical strategy offers excellent operative outcomes.


Subject(s)
Aorta, Thoracic/surgery , Aortic Diseases , Blood Vessel Prosthesis Implantation , Brachiocephalic Trunk , Adult , Aged , Aged, 80 and over , Aorta, Thoracic/diagnostic imaging , Aortic Diseases/diagnostic imaging , Aortic Diseases/epidemiology , Aortic Diseases/surgery , Blood Vessel Prosthesis , Blood Vessel Prosthesis Implantation/methods , Blood Vessel Prosthesis Implantation/mortality , Brachiocephalic Trunk/pathology , Brachiocephalic Trunk/surgery , Female , Humans , Incidence , Male , Middle Aged , Postoperative Complications , Practice Guidelines as Topic , Treatment Outcome
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