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Quadricuspid Aortic Valve Detected by Three-Dimensional Transesophageal Echocardiography

Published:April 03, 2015DOI:https://doi.org/10.1053/j.jvca.2015.02.006
      To the Editor:
      Quadricuspid aortic valve (QAVs) is a rare congenital heart defect. Two-dimensional (2D) transthoracic echocardiography (TTE) and transesophageal echocardiography (TEE) have been used to capture these abnormalities. Recently introduced live real-time three-dimensional (3D) TEE allows for online assessment of cardiac structures and provides novel views of complex cardiac abnormalities. In this letter, we show 2D and 3D TEE images of QAV in addition to a photograph obtained during intraoperative inspection.

      Case Report

      A 50-year-old- woman was diagnosed with aortic regurgitation approximately 20 years ago but had an otherwise unremarkable medical history. She presented with progressively worsening fatigue and shortness of breath over the previous 3 months. Physical examination revealed a blowing murmur in early diastole along the left sternal border with blood pressure of 128/43 mmHg. TTE demonstrated severe aortic insufficiency with an enlarged left ventricular cavity and normal contraction. The left ventricular end-diastolic diameter was 54 mm, the end-systolic diameter was 33 mm, and the ejection fraction was 63%. Moreover, a suspected QAV was observed but could not be confirmed due to poor image quality. No other pathologic features were noted. The patient was scheduled for elective replacement of the aortic valve. Intraoperative 2D TEE was performed to evaluate the valve and revealed a clear QAV comprising 2 equal larger cusps and 2 equal smaller cusps (Fig 1). On the other hand, 3D TEE showed 1 large cusp and 3 smaller equal cusps and miscoaptation of the accessory cusp between the right coronary cusp and left coronary cusp with moderate-to-severe aortic insufficiency (Fig 2). Holodiastolic reverse flow was observed in the descending aorta. The aortic valve was replaced with a 21-mm St. Jude Medical RegentTM. She was discharged on postoperative day 15 after an uneventful course and is doing well and free from symptoms limiting her quality of life.
      Figure thumbnail gr1
      Fig 1Two-dimensional transesophageal echocardiography image. This image shows 2 equal larger cusps (NCC and LCC) and 2 equal smaller cusps (RCC and accessory cusp). NCC, noncoronary cusp; LCC, left coronary cusp; RC, right coronary cusp.
      Figure thumbnail gr2
      Fig 2Three-dimensional transesophageal echocardiography image. This image shows 1 large cusp (NCC) and 3 equal smaller cusps (LCC, RCC, and accessory cusp) with a regurgitation jet on the accessory cusp. NCC, noncoronary cusp; LCC, left coronary cusp; RCC, right coronary cusp.

      Discussion

      Quadricuspid aortic valve is a very rare congenital abnormality. Systemic autopsy studies estimate that the incidence of QAV is 0.008%, while a more recent echocardiographic review reported an incidence of 0.043%. QAV accounts for 0.55% to 1.46% of aortic valve surgical patients.
      • Tutarel O.
      The quadricuspid aortic valve: A comprehensive review.
      • Jagannath A.D.
      • Johri A.M.
      • Liberthson R.
      • et al.
      Quadricuspid aortic valve: A report of 12 cases and a review of the literature.

      Hayakawa M, Asai T, Kinoshita T, et al: Quadricuspid aortic valve: A report on a 10-year case series and literature review. Ann Thorac Cardiovasc Surg, In press

      • Gouveia S.
      • Martins J.D.
      • Costa G.
      • et al.
      Quadricuspid aortic valve --- 10-year case series and literature review.
      The mechanism of this abnormality is not well known. One cogent hypothesis is the abnormal septation of the embryologic truncus arteriosus. In general, after septation of the arterial trunk, 3 mesenchymal swellings develop into semilunar leaflets of the aortic and pulmonary trunks. In QAV, however, the fourth cusp arises during the early stage of truncal septation, resulting in either a different number of primordial aortic leaflets or abnormal cusp proliferation.
      • Zhu J.
      • Zhang J.
      • Wu S.
      • et al.
      Congenital quadricuspid aortic valve associated with aortic insufficiency and mitral regurgitation.
      Hurwitz and Roberts classified quadricuspid valves into 7 subtypes based on the cusp size and degree of cusp equality.
      • Hurwitz L.E.
      • Roberts W.C.
      Quadricuspid semilunar valve.
      In this case, 2D TEE examination revealed that the valve had 2 equal larger cusps and 2 equal smaller cusps (type C in Hurwitz’s classification). In the 3D TEE image, however, 1 large cusp and 3 equal smaller cusps (type E in Hurwitz’s classification) were revealed. Intraoperative inspection by the surgeon (Fig 3) confirmed the presence of 3 equal smaller cusps and 1 larger cusp. The erroneous finding of the 2D TEE examination was due to an unreliable aortic valve short-axis view. The ultrasonic echo beam of the 2D TEE was not transmitted vertically against the long axis of the aortic valve. The ability of 3D TEE to use a cropping mode allowed us to detect an arbitrary plane of the pyramid-shaped 3D data set. As a result, the real aortic valve short-axis view could be clearly observed. Therefore, the precise configuration of the QAV could be diagnosed by 3D TEE and was confirmed by the surgeon’s intraoperative inspection.
      Figure thumbnail gr3
      Fig 3Intraoperative findings by the surgeon. This image confirmed the diagnosis of 1 large cusp (NCC) and 3 equal smaller cusps (LCC, RCC, and accessory cusp). NCC, noncoronary cusp; LCC, left coronary cusp; RCC, right coronary cusp.
      Aortic regurgitation appears to be the most prevalent hemodynamic abnormality associated with QAV. The patient had aortic regurgitation for more than 20 years and required an aortic valve replacement. QAVs often are associated with other abnormalities, such as displacement of the coronary sinus and ostium, ventricular septal defect, patent ductus arteriosus, subaortic stenosis, cardiomyopathy, Valsalva aneurysm, and mitral valve regurgitation.
      • Tutarel O.
      The quadricuspid aortic valve: A comprehensive review.
      • Jagannath A.D.
      • Johri A.M.
      • Liberthson R.
      • et al.
      Quadricuspid aortic valve: A report of 12 cases and a review of the literature.

      Hayakawa M, Asai T, Kinoshita T, et al: Quadricuspid aortic valve: A report on a 10-year case series and literature review. Ann Thorac Cardiovasc Surg, In press

      • Gouveia S.
      • Martins J.D.
      • Costa G.
      • et al.
      Quadricuspid aortic valve --- 10-year case series and literature review.
      In this patient, the right coronary sinus and ostium were located on the side of the commissure between the right coronary cusp and accessory cusp. We observed no indication of right coronary artery ischemia after surgery.
      TTE has an important role in detecting congenital anomalies preoperatively. TTE may be suboptimal, however, for recognizing QAV and associated malformations due to the poor acoustic window. TEE does, however, provide reliable imaging of the structure in the heart even in complex congenital heart disease. In this patient, we could not diagnose QAV by preoperative TTE examination. In contrast, intraoperative 3D TEE provided clearer visualization and thus definitive diagnosis. Moreover, 3D TEE revealed a jet of regurgitation through the accessory cusp.
      Aortic valve repair for QAV now is being performed, and the short-term results have been good. The 3D TEE, which allows for the detection of complex pathologic anatomic changes, might provide key information to help prevent surgical complications, such as coronary ischemia and residual regurgitation. Thus, 3D TEE will have an important role not only for diagnosis but also for guiding further surgical procedures.

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