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Original research article
Anatomical and clinical predictors of valve dysfunction and aortic dilation in bicuspid aortic valve disease
  1. Arturo Evangelista1,
  2. Pastora Gallego2,
  3. Francisco Calvo-Iglesias3,
  4. Javier Bermejo4,
  5. Juan Robledo-Carmona5,
  6. Violeta Sánchez6,
  7. Daniel Saura7,
  8. Roman Arnold8,
  9. Amelia Carro1,
  10. Giuliana Maldonado1,
  11. Augusto Sao-Avilés1,
  12. Gisela Teixidó1,
  13. Laura Galian1,
  14. José Rodríguez-Palomares1,
  15. David García-Dorado1
  1. 1Servei de Cardiologia, Hospital Universitari Vall d'Hebron, CIBER-CV, Barcelona, Spain
  2. 2Servicio de Cardiología, Hospital Universitario Virgen Macarena, CIBER-CV, Sevilla, Spain
  3. 3Servicio de Cardioloxía, Complexo Hospitalario Universitario de Vigo, Vigo, Spain
  4. 4Servicio de Cardiología, Hospital Gregorio Marañón, CIBER-CV, Madrid, Spain
  5. 5Unidad de Gestión Clínica del Corazón, Hospital Universitario Virgen de la Victoria de Málaga, CIBER-CV, Málaga, Spain
  6. 6Servicio de Cardiología, Hospital Universitario Doce de Octubre, CIBER-CV, Madrid, Spain
  7. 7Servicio de Cardiología, Hospital Clínico Universitario Virgen de la Arrixaca, IMIB-Arrixaca, CIBER-CV, Murcia, Spain
  8. 8Servicio de Cardiologia (ICICOR), Hospital Clínico Universitario, CIBER-CV, Valladolid, Spain
  1. Correspondence to Dr Arturo Evangelista, Servei de Cardiologia, Hospital Universitari Vall d’Hebron, Pº Vall d’Hebron 119, Barcelona 08036, Spain; arturevangelistamasip{at}


Objective Bicuspid aortic valve (BAV) is associated with early valvular dysfunction and proximal aorta dilation with high heterogeneity. This study aimed to assess the determinants of these complications.

Methods Eight hundred and fifty-two consecutive adults diagnosed of BAV referred from cardiac outpatient clinics to eight echocardiographic laboratories of tertiary hospitals were prospectively recruited. Exclusion criteria were aortic coarctation, other congenital disorders or intervention. BAV morphotype, significant valve dysfunction and aorta dilation (≥2 Z-score) at sinuses and ascending aorta were established.

Results Three BAV morphotypes were identified: right–left coronary cusp fusion (RL) in 72.9%, right–non-coronary (RN) in 24.1% and left–non-coronary (LN) in 3.0%. BAV without raphe was observed in 18.3%. Multivariate analysis showed aortic regurgitation (23%) to be related to male sex (OR: 2.80, p<0.0001) and valve prolapse (OR: 5.16, p<0.0001), and aortic stenosis (22%) to BAV-RN (OR: 2.09, p<0.001), the presence of raphe (OR: 2.75, p<0.001), age (OR: 1.03; p<0.001), dyslipidaemia (OR: 1.77, p<0.01) and smoking (OR: 1.63, p<0.05). Ascending aorta was dilated in 76% without differences among morphotypes and associated with significant valvular dysfunction. By contrast, aortic root was dilated in 34% and related to male sex and aortic regurgitation but was less frequent in aortic stenosis and BAV-RN.

Conclusions Normofunctional valves are more prevalent in BAV without raphe. Aortic stenosis is more frequent in BAV-RN and associated with some cardiovascular risk factors, whereas aortic regurgitation (AR) is associated with male sex and sigmoid prolapse. Although ascending aorta is the most commonly dilated segment, aortic root dilation is present in one-third of patients and associated with AR. Remarkably, BAV-RL increases the risk for dilation of the proximal aorta, whereas BAV-RN spares this area.

  • bicuspid aortic valve
  • valvular heart disease
  • aortic dilation
  • echocardiography
  • aortic stenosis
  • aortic regurgitation

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  • Contributors AE planned, conducted the study and wrote the manuscript. PG, FC-I, JB, JR-C, VS, DS, RA and AC collected and acquired echocardiographic studies. GM included information in database. AS-A and JR-P contributed in statistical analysis. GT and LG, expert echocardiographers, analysed and measured all studies. DG-D helped in writing the final version of the manuscript.

  • Funding Supported by grants from the Fondo de Investigación Sanitaria, Red de Investigación Cooperativa de las Enfermedades Cardiovasculares, CIBER-CV Instituto de Salud Carlos III, Ministerio de Sanidad y Consumo and cofunded by European Union (ERDF/ESF) FIS-PI11/01081.

  • Competing interests None declared.

  • Patient consent Obtained.

  • Ethics approval CEIC Hospital Universitari Vall d'Hebron.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Collaborators Hospital Universitari Vall d'Hebron, Barcelona: Laura Gutiérrez, Ruben Fernández and Teresa González-Alujas. Hospital Universitario Virgen Macarena, Sevilla: Irene Mendez; Complexo Hospitalario Universitario de Vigo: Mireya Castro Verdes and Cristina Victoria Iglesia Carreño. Hospital Gregorio Marañón, Madrid: María Eugenia Vázquez and Ana González-Mansill. Hospital Universitario Virgen de la Victoria de Málaga: Isabel Rodríguez-Bailón. Hospital Universitario Doce de Octubre, CIBER cardiovascular, Madrid: Sagrario Fernández Casares and Carmen Jiménez López-Guarch. Hospital Clínico Universitario Virgen de la Arrixaca, Murcia: Gonzalo de la Morena and M. José Oliva. Hospital Clínico Universitario, Valladolid: Teresa Sevilla.

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