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Circulation | 2009

Randomized comparison of strategies for type B aortic dissection: the INvestigation of STEnt Grafts in Aortic Dissection (INSTEAD) trial.

Christoph Nienaber; Hervé Rousseau; Holger Eggebrecht; Stephan Kische; Rossella Fattori; Tim C. Rehders; Günther Kundt; Dierk Scheinert; Martin Czerny; Tilo Kleinfeldt; Burkhart Zipfel; Louis Labrousse; Hüseyin Ince

Background— Thoracic endovascular aortic repair (TEVAR) represents a novel concept for type B aortic dissection. Although life-saving in acute emergencies, outcomes and survival of TEVAR in stable dissection are unknown. Methods and Results— One hundred forty patients in stable clinical condition at least 2 weeks after index dissection were randomly subjected to elective stent-graft placement in addition to optimal medical therapy (n=72) or to optimal medical therapy alone (n=68) with surveillance (arterial pressure according to World Health Organization guidelines ≤120/80 mm Hg). The primary end point was all-cause death at 2 years, whereas aorta-related death, progression (with need for conversion or additional endovascular or open surgery), and aortic remodeling were secondary end points. There was no difference in all-cause deaths, with a 2-year cumulative survival rate of 95.6±2.5% with optimal medical therapy versus 88.9±3.7% with TEVAR (P=0.15); the trial, however, turned out to be underpowered. Moreover, the aorta-related death rate was not different (P=0.44), and the risk for the combined end point of aorta-related death (rupture) and progression (including conversion or additional endovascular or open surgery) was similar (P=0.65). Three neurological adverse events occurred in the TEVAR group (1 paraplegia, 1 stroke, and 1 transient paraparesis), versus 1 case of paraparesis with medical treatment. Finally, aortic remodeling (with true-lumen recovery and thoracic false-lumen thrombosis) occurred in 91.3% of patients with TEVAR versus 19.4% of those who received medical treatment (P<0.001), which suggests ongoing aortic remodeling. Conclusions— In the first randomized study on elective stent-graft placement in survivors of uncomplicated type B aortic dissection, TEVAR failed to improve 2-year survival and adverse event rates despite favorable aortic remodeling. Clinical Trial Registration— URL: http://www.clinicaltrials.gov. Unique identifier: NCT00525356.


Circulation-cardiovascular Interventions | 2013

Endovascular Repair of Type B Aortic Dissection Long-term Results of the Randomized Investigation of Stent Grafts in Aortic Dissection Trial

Christoph Nienaber; Stephan Kische; Hervé Rousseau; Holger Eggebrecht; Tim C. Rehders; Guenther Kundt; Aenne Glass; Dierk Scheinert; Martin Czerny; Tilo Kleinfeldt; Burkhart Zipfel; Louis Labrousse; Rossella Fattori; Hüseyin Ince

Background—Thoracic endovascular aortic repair (TEVAR) represents a therapeutic concept for type B aortic dissection. Long-term outcomes and morphology after TEVAR for uncomplicated dissection are unknown. Methods and Results—A total of 140 patients with stable type B aortic dissection previously randomized to optimal medical treatment and TEVAR (n=72) versus optimal medical treatment alone (n=68) were analyzed retrospectively for aorta-specific, all-cause outcomes, and disease progression using landmark statistical analysis of years 2 to 5 after index procedure. Cox regression was used to compare outcomes between groups; all analyses are based on intention to treat. The risk of all-cause mortality (11.1% versus 19.3%; P=0.13), aorta-specific mortality (6.9% versus 19.3%; P=0.04), and progression (27.0% versus 46.1%; P=0.04) after 5 years was lower with TEVAR than with optimal medical treatment alone. Landmark analysis suggested a benefit of TEVAR for all end points between 2 and 5 years; for example, for all-cause mortality (0% versus 16.9%; P=0.0003), aorta-specific mortality (0% versus 16.9%; P=0.0005), and for progression (4.1% versus 28.1%; P=0.004); Landmarking at 1 year and 1 month revealed consistent findings. Both improved survival and less progression of disease at 5 years after elective TEVAR were associated with stent graft induced false lumen thrombosis in 90.6% of cases (P<0.0001). Conclusions—In this study of survivors of type B aortic dissection, TEVAR in addition to optimal medical treatment is associated with improved 5-year aorta-specific survival and delayed disease progression. In stable type B dissection with suitable anatomy, preemptive TEVAR should be considered to improve late outcome. Clinical Trial Registration—URL: http://www.clinicaltrials.gov. Unique identifier: NCT01415804.


The Journal of Thoracic and Cardiovascular Surgery | 2010

Strategies for subacute/chronic type B aortic dissection: the Investigation Of Stent Grafts in Patients with type B Aortic Dissection (INSTEAD) trial 1-year outcome.

Christoph Nienaber; Stephan Kische; Ibrahim Akin; Hervé Rousseau; Holger Eggebrecht; Rossella Fattori; Tim C. Rehders; Guenther Kundt; Dierk Scheinert; Martin Czerny; Tilo Kleinfeldt; Burkhart Zipfel; Louis Labrousse; Hüseyin Ince

OBJECTIVE Endovascular stent grafting represents a novel concept for type B aortic dissection both in the acute and subacute/chronic setting, with an unknown effect on outcomes. METHODS In a prospective trial 140 patients with stable type B dissection were randomly subjected to elective stent-graft placement in addition to optimal medical therapy (n = 72) or to optimal medical therapy (n = 68) with surveillance (arterial pressure according to World Health Organization guidelines ≤ 120/80 mm Hg). The primary end point was 1-year all-cause mortality, whereas aorta-related mortality, progression (with need for conversion or additional endovascular or open surgical intervention), and aortic remodeling were secondary end points. RESULTS There was no difference in all-cause mortality: cumulative survival was 97.0% ± 3.4% with optimal medical therapy versus 91.3% ± 2.1% with thoracic endovascular aortic repair (P = .16). Moreover, aorta-related mortality was not different (P = .42), and the risk for the combined end point of aorta-related death (rupture) and progression (including conversion or additional endovascular or open surgical intervention) was similar (P = .86). Three neurologic adverse events occurred in the thoracic endovascular aortic repair group (1 paraplegia, 1 stroke, and 1 transient paraparesis) versus 1 episode of paraparesis with medical treatment. Finally, aortic remodeling (with true-lumen recovery and thoracic false-lumen thrombosis) occurred in 91.3% with thoracic endovascular aortic repair versus 19.4% with medical treatment (P < .001), which is suggestive of continued remodeling. CONCLUSIONS In survivors of uncomplicated type B aortic dissection, elective stent-graft placement does not improve 1-year survival and adverse events, despite favorable aortic remodeling.


Clinical Research in Cardiology | 2010

Cardiac manifestation of the hypereosinophilic syndrome: new insights

Tilo Kleinfeldt; Christoph Nienaber; Stephan Kische; Ibrahim Akin; R. G. Turan; Thomas Körber; Henrik Schneider; Hüseyin Ince

Cardiac manifestation is the major cause of morbidity in patients with hypereosinophilic syndrome (HES). Clinical features range from heart failure to arterial embolism, which are caused by thickening of the endocardium and mural left ventricular thrombosis. Modern magnetic resonance imaging and echocardiography are able to detect fibrosis, eosinophilic infiltrate and thrombi to stage the fibrotic evolution of the disease. Treatment of HES involves standard medication for heart failure, anticoagulant therapy, immunosuppressive therapy and potentially surgical resection. The outcome of HES depends on both the progression of endocardial fibrosis and associated complications and the 5-year mortality is estimated at 30%.


Stem Cells and Development | 2011

Improved Mobilization of the CD34+ and CD133+ Bone Marrow-Derived Circulating Progenitor Cells by Freshly Isolated Intracoronary Bone Marrow Cell Transplantation in Patients with Ischemic Heart Disease

Ramazan Gökmen Turan; Ilkay Bozdag-Turan; Jasmin Ortak; Ibrahim Akin; Stephan Kische; Henrik Schneider; Cem Hakan Turan; Tim C. Rehders; Mathias Rauchhaus; Tilo Kleinfeldt; Ester Adolph; M. Brehm; Sedat Yokus; Stephan Steiner; Kurtulus Sahin; Christoph Nienaber; Hüseyin Ince

Cell therapy is a promising novel option for treatment of cardiovascular disease. Because the role of bone marrow-derived circulating progenitor cells (BM-CPCs) after cell therapy is less clear, we analyzed in this randomized, controlled study the influence of intracoronary autologous freshly isolated bone marrow cell transplantation (BMC-Tx) by using a point-of-care system on cardiac function and on the mobilization of BM-CPCs in patients with ischemic heart disease (IHD). Fifty-six patients with IHD were randomized to either receive freshly isolated BMC-Tx or a control group that did not receive cell therapy. Peripheral blood concentrations of CD34/45(+) and CD133/45(+) CPCs were measured by flow cytometry pre-, immediately post-, and at 3, 6, and 12 months postprocedure in both groups. Global ejection fraction and the size of infarct area were determined by left ventriculography. We observed in patients with IHD after intracoronary transplantation of autologous freshly isolated BMCs-Tx at 3 and 12 months follow-up a significant reduction of the size of infarct area and increase of global ejection fraction as well as infarct wall movement velocity. The mobilization of CD34/45(+) and CD133/45(+) BM-CPCs significantly increased at 3, 6, and 12 months after cell therapy when compared with baseline in patients with IHD, although no significant changes were observed between pre- and immediately postintracoronary cell therapy administration. In the control group without cell therapy, there was no significant difference of CD34/45(+) and CD133/45(+) BM-CPCs mobilization between pre- and at 3, 6, and 12 months postcoronary angiography. Intracoronary transplantation of autologous freshly isolated BMCs by using a point-of-care system in patients with IHD may enhance and prolong the mobilization of CD34/45(+) and CD133/45(+) BM-CPCs in peripheral blood and this might increase the regenerative potency in IHD.


International Journal of Cardiology | 2009

Detection of FMR1-gene in Takotsubo cardiomyopathy: A new piece in the puzzle

Tilo Kleinfeldt; Henrik Schneider; Ibrahim Akin; Stefan Kische; R. Gökmen Turan; Christoph Nienaber; Hüseyin Ince

Takotsubo cardiomyopathy (TTC) is a cardiac entity appreciated only recently mimicking acute myocardial infarction, often affects post-menopausal women and is triggered by preceding emotional or physical stress. Pathogenesis of TTC is unknown, recurrence of TTC in one individual and familial predisposition occurs. Expression profiling of cardiac genes in the acute phase of TTC are not enough analyzed and are a component of future research. We report for the first time on a female individual with TTC, who happened to be carrier of an FMR1 gene mutation, alleles of an intermediate size between 40-55 triplet premutations.


International Journal of Cardiology | 2009

Recurrent left ventricular apical ballooning induced by recurrent stress

Tilo Kleinfeldt; Raik Severin; Stefan Lischke; Hueseyin Ince; Christoph Nienaber

This paper reports on a 67-year-old woman with apical ballooning and recurrent dyskinesia of left ventricular wall. During MRI-examination the patient developed severe apical dyskinesia of the left ventricle induced by agoraphobia in the magnet.


Medizinische Klinik | 2009

Quadrikuspide Aortenklappe als ungewöhnliche Genese einer ausgeprägten Aortenklappeninsuffizienz

Ibrahim Akin; Stephan Kische; Jasmin Ortak; Tim C. Rehders; Tushar Chatterjee; Henrik Schneider; Dietmar Bänsch; Gökmen R. Turan; Mathias Rauchhaus; Tilo Kleinfeldt; Esther Adolph; Christoph Nienaber; Hüseyin Ince

ZusammenfassungHintergrund:Eine Aortenklappeninsuffizienz, unterteilbar in eine akute sowie eine chronische Form, kann unterschiedlicher Genese sein. Während bei der akuten Form häufig eine Endokarditis, eine Typ-A-Aortendissektion sowie Traumata eine Rolle spielen, kann die chronische Form u.a. durch eine angeborene Klappenanomalie bedingt sein. Hierbei stellt die quadrikuspide Aortenklappe die weitaus seltenste Form dar. Die Diagnose wird meist zufällig während der echokardiographischen Untersuchung, im Rahmen von kardiochirurgischen Eingriffen oder postmortal gestellt.Fallbeschreibung:Berichtet wird über eine 44-jährige Patientin mit seit 2 Jahren bestehender progredienter Belastungsdyspnoe. In der klinischen Untersuchung fand sich ein lautes Diastolikum im zweiten Interkostalraum rechts bei einem Blutdruck von 170/60 mmHg. Die echokardiographische Untersuchung ergab die Diagnose einer Aortenklappeninsuffizienz III° bei angeborener quadrikuspider Aortenklappe. Nach operativem Aortenklappenersatz nach Bentall und anschließender oraler Antikoagulation wurde die Patientin im weiteren Verlauf beschwerdefrei.Schlussfolgerung:Das Auftreten einer quadrikuspiden Aortenklappe, häufig assoziiert mit einer Aortenklappeninsuffizienz, ist sehr selten. Bei klinischem Verdacht sollte eine echokardiographische Untersuchung veranlasst werden. Die spezielle Therapie kann anhand der klinischen Beschwerden und der objektiven Parameter festgelegt werden. Bei geringer Aortenklappeninsuffizienz und leichten klinischen Beschwerden ist ein medikamentös-konservatives Vorgehen gerechtfertigt. Bei Auftreten einer höhergradigen Aortenklappeninsuffizienz sowie klinischen Zeichen einer kardialen Dekompensation wird eine chirurgische Therapie empfohlen.AbstractBackground:Both, acute and chronic aortic insufficiency, can be caused by various conditions. While the acute form is often associated with an endocarditis, type A aortic dissection and trauma, one possible cause of chronic aortic insufficiency is a congenital malformation of the aortic valve. Among these malformations, the quadricuspid aortic valve is the most rare form. The diagnosis is often made by Doppler echocardiography, during cardiac surgery, or postmortem.Case Report:A 44-year-old female patient presented with a 2-year history of increasing dyspnea on exertion. The physical examination revealed a diastolic heart murmur in the second right intercostal space and a blood pressure of 170/60 mmHg. Transthoracic and transesophageal Doppler echocardiography disclosed a quadricuspid aortic valve with severe aortic insufficiency. After implantation of a mechanical aortic valve, the patient was clinically well.Conclusion:Quadricuspid aortic valve, often associated with aortic insufficiency, is a rare condition. The diagnosis can be made by Doppler echocardiography. The special treatment should be based on objective and subjective findings. In the absence of severe aortic regurgitation or clinical signs, a medical treatment with close follow-up is justified. In case of severe aortic regurgitation or clinical signs of cardiac decompensation, surgical treatment is recommended.BACKGROUND Both, acute and chronic aortic insufficiency, can be caused by various conditions. While the acute form is often associated with an endocarditis, type A aortic dissection and trauma, one possible cause of chronic aortic insufficiency is a congenital malformation of the aortic valve. Among these malformations, the quadricuspid aortic valve is the most rare form. The diagnosis is often made by Doppler echocardiography, during cardiac surgery, or postmortem. CASE REPORT A 44-year-old female patient presented with a 2-year history of increasing dyspnea on exertion. The physical examination revealed a diastolic heart murmur in the second right intercostal space and a blood pressure of 170/60 mmHg. Transthoracic and transesophageal Doppler echocardiography disclosed a quadricuspid aortic valve with severe aortic insufficiency. After implantation of a mechanical aortic valve, the patient was clinically well. CONCLUSION Quadricuspid aortic valve, often associated with aortic insufficiency, is a rare condition. The diagnosis can be made by Doppler echocardiography. The special treatment should be based on objective and subjective findings. In the absence of severe aortic regurgitation or clinical signs, a medical treatment with close follow-up is justified. In case of severe aortic regurgitation or clinical signs of cardiac decompensation, surgical treatment is recommended.


Medizinische Klinik | 2009

Quadricuspid aortic valve as an unusual cause of severe aortic regurgitation

Ibrahim Akin; Stephan Kische; Jasmin Ortak; Tim C. Rehders; Tushar Chatterjee; Henrik Schneider; Dietmar Bänsch; Gökmen R. Turan; Mathias Rauchhaus; Tilo Kleinfeldt; Esther Adolph; Christoph Nienaber; Hüseyin Ince

ZusammenfassungHintergrund:Eine Aortenklappeninsuffizienz, unterteilbar in eine akute sowie eine chronische Form, kann unterschiedlicher Genese sein. Während bei der akuten Form häufig eine Endokarditis, eine Typ-A-Aortendissektion sowie Traumata eine Rolle spielen, kann die chronische Form u.a. durch eine angeborene Klappenanomalie bedingt sein. Hierbei stellt die quadrikuspide Aortenklappe die weitaus seltenste Form dar. Die Diagnose wird meist zufällig während der echokardiographischen Untersuchung, im Rahmen von kardiochirurgischen Eingriffen oder postmortal gestellt.Fallbeschreibung:Berichtet wird über eine 44-jährige Patientin mit seit 2 Jahren bestehender progredienter Belastungsdyspnoe. In der klinischen Untersuchung fand sich ein lautes Diastolikum im zweiten Interkostalraum rechts bei einem Blutdruck von 170/60 mmHg. Die echokardiographische Untersuchung ergab die Diagnose einer Aortenklappeninsuffizienz III° bei angeborener quadrikuspider Aortenklappe. Nach operativem Aortenklappenersatz nach Bentall und anschließender oraler Antikoagulation wurde die Patientin im weiteren Verlauf beschwerdefrei.Schlussfolgerung:Das Auftreten einer quadrikuspiden Aortenklappe, häufig assoziiert mit einer Aortenklappeninsuffizienz, ist sehr selten. Bei klinischem Verdacht sollte eine echokardiographische Untersuchung veranlasst werden. Die spezielle Therapie kann anhand der klinischen Beschwerden und der objektiven Parameter festgelegt werden. Bei geringer Aortenklappeninsuffizienz und leichten klinischen Beschwerden ist ein medikamentös-konservatives Vorgehen gerechtfertigt. Bei Auftreten einer höhergradigen Aortenklappeninsuffizienz sowie klinischen Zeichen einer kardialen Dekompensation wird eine chirurgische Therapie empfohlen.AbstractBackground:Both, acute and chronic aortic insufficiency, can be caused by various conditions. While the acute form is often associated with an endocarditis, type A aortic dissection and trauma, one possible cause of chronic aortic insufficiency is a congenital malformation of the aortic valve. Among these malformations, the quadricuspid aortic valve is the most rare form. The diagnosis is often made by Doppler echocardiography, during cardiac surgery, or postmortem.Case Report:A 44-year-old female patient presented with a 2-year history of increasing dyspnea on exertion. The physical examination revealed a diastolic heart murmur in the second right intercostal space and a blood pressure of 170/60 mmHg. Transthoracic and transesophageal Doppler echocardiography disclosed a quadricuspid aortic valve with severe aortic insufficiency. After implantation of a mechanical aortic valve, the patient was clinically well.Conclusion:Quadricuspid aortic valve, often associated with aortic insufficiency, is a rare condition. The diagnosis can be made by Doppler echocardiography. The special treatment should be based on objective and subjective findings. In the absence of severe aortic regurgitation or clinical signs, a medical treatment with close follow-up is justified. In case of severe aortic regurgitation or clinical signs of cardiac decompensation, surgical treatment is recommended.BACKGROUND Both, acute and chronic aortic insufficiency, can be caused by various conditions. While the acute form is often associated with an endocarditis, type A aortic dissection and trauma, one possible cause of chronic aortic insufficiency is a congenital malformation of the aortic valve. Among these malformations, the quadricuspid aortic valve is the most rare form. The diagnosis is often made by Doppler echocardiography, during cardiac surgery, or postmortem. CASE REPORT A 44-year-old female patient presented with a 2-year history of increasing dyspnea on exertion. The physical examination revealed a diastolic heart murmur in the second right intercostal space and a blood pressure of 170/60 mmHg. Transthoracic and transesophageal Doppler echocardiography disclosed a quadricuspid aortic valve with severe aortic insufficiency. After implantation of a mechanical aortic valve, the patient was clinically well. CONCLUSION Quadricuspid aortic valve, often associated with aortic insufficiency, is a rare condition. The diagnosis can be made by Doppler echocardiography. The special treatment should be based on objective and subjective findings. In the absence of severe aortic regurgitation or clinical signs, a medical treatment with close follow-up is justified. In case of severe aortic regurgitation or clinical signs of cardiac decompensation, surgical treatment is recommended.


Medizinische Klinik | 2009

Quadrikuspide Aortenklappe als ungewöhnliche Genese einer ausgeprägten Aortenklappeninsuffizienz@@@Quadricuspid Aortic Valve as an Unusual Cause of Severe Aortic Regurgitation

Ibrahim Akin; Stephan Kische; Jasmin Ortak; Tim C. Rehders; Tushar Chatterjee; Henrik Schneider; Dietmar Bänsch; Gökmen R. Turan; Mathias Rauchhaus; Tilo Kleinfeldt; Esther Adolph; Christoph Nienaber; Hüseyin Ince

ZusammenfassungHintergrund:Eine Aortenklappeninsuffizienz, unterteilbar in eine akute sowie eine chronische Form, kann unterschiedlicher Genese sein. Während bei der akuten Form häufig eine Endokarditis, eine Typ-A-Aortendissektion sowie Traumata eine Rolle spielen, kann die chronische Form u.a. durch eine angeborene Klappenanomalie bedingt sein. Hierbei stellt die quadrikuspide Aortenklappe die weitaus seltenste Form dar. Die Diagnose wird meist zufällig während der echokardiographischen Untersuchung, im Rahmen von kardiochirurgischen Eingriffen oder postmortal gestellt.Fallbeschreibung:Berichtet wird über eine 44-jährige Patientin mit seit 2 Jahren bestehender progredienter Belastungsdyspnoe. In der klinischen Untersuchung fand sich ein lautes Diastolikum im zweiten Interkostalraum rechts bei einem Blutdruck von 170/60 mmHg. Die echokardiographische Untersuchung ergab die Diagnose einer Aortenklappeninsuffizienz III° bei angeborener quadrikuspider Aortenklappe. Nach operativem Aortenklappenersatz nach Bentall und anschließender oraler Antikoagulation wurde die Patientin im weiteren Verlauf beschwerdefrei.Schlussfolgerung:Das Auftreten einer quadrikuspiden Aortenklappe, häufig assoziiert mit einer Aortenklappeninsuffizienz, ist sehr selten. Bei klinischem Verdacht sollte eine echokardiographische Untersuchung veranlasst werden. Die spezielle Therapie kann anhand der klinischen Beschwerden und der objektiven Parameter festgelegt werden. Bei geringer Aortenklappeninsuffizienz und leichten klinischen Beschwerden ist ein medikamentös-konservatives Vorgehen gerechtfertigt. Bei Auftreten einer höhergradigen Aortenklappeninsuffizienz sowie klinischen Zeichen einer kardialen Dekompensation wird eine chirurgische Therapie empfohlen.AbstractBackground:Both, acute and chronic aortic insufficiency, can be caused by various conditions. While the acute form is often associated with an endocarditis, type A aortic dissection and trauma, one possible cause of chronic aortic insufficiency is a congenital malformation of the aortic valve. Among these malformations, the quadricuspid aortic valve is the most rare form. The diagnosis is often made by Doppler echocardiography, during cardiac surgery, or postmortem.Case Report:A 44-year-old female patient presented with a 2-year history of increasing dyspnea on exertion. The physical examination revealed a diastolic heart murmur in the second right intercostal space and a blood pressure of 170/60 mmHg. Transthoracic and transesophageal Doppler echocardiography disclosed a quadricuspid aortic valve with severe aortic insufficiency. After implantation of a mechanical aortic valve, the patient was clinically well.Conclusion:Quadricuspid aortic valve, often associated with aortic insufficiency, is a rare condition. The diagnosis can be made by Doppler echocardiography. The special treatment should be based on objective and subjective findings. In the absence of severe aortic regurgitation or clinical signs, a medical treatment with close follow-up is justified. In case of severe aortic regurgitation or clinical signs of cardiac decompensation, surgical treatment is recommended.BACKGROUND Both, acute and chronic aortic insufficiency, can be caused by various conditions. While the acute form is often associated with an endocarditis, type A aortic dissection and trauma, one possible cause of chronic aortic insufficiency is a congenital malformation of the aortic valve. Among these malformations, the quadricuspid aortic valve is the most rare form. The diagnosis is often made by Doppler echocardiography, during cardiac surgery, or postmortem. CASE REPORT A 44-year-old female patient presented with a 2-year history of increasing dyspnea on exertion. The physical examination revealed a diastolic heart murmur in the second right intercostal space and a blood pressure of 170/60 mmHg. Transthoracic and transesophageal Doppler echocardiography disclosed a quadricuspid aortic valve with severe aortic insufficiency. After implantation of a mechanical aortic valve, the patient was clinically well. CONCLUSION Quadricuspid aortic valve, often associated with aortic insufficiency, is a rare condition. The diagnosis can be made by Doppler echocardiography. The special treatment should be based on objective and subjective findings. In the absence of severe aortic regurgitation or clinical signs, a medical treatment with close follow-up is justified. In case of severe aortic regurgitation or clinical signs of cardiac decompensation, surgical treatment is recommended.

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