Unicuspid aortic valve: a rare congenital anomaly

Unicuspid aortic valve (UAV) is a rare congenital aortic valve anomaly. It has two subtypes and often is associated with aortic valve dysfunction and corresponding clinical presentations. Echocardiography is the first choice of diagnostic method for UAV. Three-dimensional echocardiography has played an increasingly important role in diagnosis, intraprocedural guidance, and post-procedural assessment in recent years. There remain challenges in distinguishing UAV from bicuspid aortic valve (BAV) or tricuspid aortic valve (TAV). Misdiagnosis is most resulted from raphes or leaflet calcifications. Multi-modality imaging has obvious advantages over single-modality imaging and is crucial for UAV diagnosis. Accurate identification of aortic valve morphology is important to choose the best treatment. Aortic balloon valvoplasty, surgical valvotomy, commissurotomy, Ross procedure, surgical valve repair and surgical replacement are most common treatment modalities of UAV. In this review, we aim to discuss UAV including epidemiology, definition, classification, diagnostic importance and necessity, valvular function, clinical presentation, associated disorders, non-invasive diagnostic modalities and therapeutic approaches.

Comparative assessment of aortic valve stenosis using two-dimensional, three-dimensional transesophageal echocardiography, computed tomography and cardiac catheterization

Aim. To compare effectiveness of ultrasound, radiological and invasive methods for assessing aortic valve (AV) stenosis.Material and methods. This study included 33 patients with AV stenosis. The mean age of the patients was 71,8±6,8 years. All patients underwent standard and three-dimensional echocardiography, computed tomography, and cardiac catheterization.Results. According to two-dimensional echocardiography, the AV area averaged 0,58±0,21 mm2, according to cardiac catheterization — 0,61±0,17 mm2, according to three-dimensional transesophageal echocardiography — 1,13±0,42 mm2, and according to multislice computed tomography 0,88±0,48 mm2. The difference between the values was significant (p<0,05).Conclusion. For routine diagnosis of AV stenosis, two-dimensional echocardiography is the optimal research method. With indications for radical treatment methods, three-dimensional echocardiography or multislice computed tomography should be performed.

Feasibility and Accuracy of a Fully Automated Right Ventricular Quantification Software With Three-Dimensional Echocardiography: Comparison With Cardiac Magnetic Resonance

Background: A novel, fully automated right ventricular (RV) software for three-dimensional quantification of RV volumes and function was developed. The direct comparison of the software performance with cardiac magnetic resonance (CMR) was limited. Therefore, the aim of this study was to test the feasibility, accuracy, and reproducibility of a fully automated RV quantification software against CMR imaging as a reference.Methods: A total of 170 patients who underwent both CMR and three-dimensional echocardiography were enrolled. RV end-diastolic volume (RVEDV), RV end-systolic volume (RVESV), and RV ejection fraction (RVEF) were obtained using fully automated three-dimensional RV quantification software and compared with a CMR reference. For inter-technical agreement, Spearman correlation and Bland–Altman analysis were used.Results: The fully automated RV quantification software was feasible in 149 patients. RVEDV and RVESV were underestimated, and RVEF was overestimated compared with CMR values. RV measurements obtained from the manual editing method correlated better with CMR values than that without manual editing (RVEDV, 0.924 vs. 0.794: RVESV, 0.955 vs. 0.854; RVEF, 0.941 vs. 0.781 respectively, all p &lt; 0.0001) with less bias and narrower limit of agreement (LOA). The bias and LOA for RV volumes and EF using the automated software without and with manual editing were greater in patients with severely impaired RV function or low frame rate than those with normal and mild impaired RV function, or high frame rate. The fully automated RV three-dimensional measurements were highly reproducible.Conclusion: The novel fully automated RV software shows good feasibility and reproducibility, and the measurements had a high correlation with CMR values. These findings support the routine application of the novel 3D automated RV software in clinical practice.

The impact of intraoperative pericardial three-dimensional echocardiography for the atrioventricular valve repair in the patient with congenital heart disease

Background Atrioventricular valve (AVV) regurgitation enormously affected the survival outcome of the patients with congenital heart disease (CHD). However, the image quality by use of transthoracic echocardiography has not reached a level that is sufficient, and also, three-dimensional echocardiography, which is useful to clarify complex AVV anatomy, cannot be applied for the patients less than 15kg, to guide for the AVV repair in pediatric patients. We try to show surgeons more precise three-dimensional images about an AVV by using intraoperative pericardial three-dimensional echocardiography (IP3DE) and improve the surgical outcome. Purpose To determine the efficacy of IP3DE by assessing the surgical outcome of an AVV repair and re-intervention rate. Method Eighty-five patient with CHD who underwent atrioventricular repair with significant regurgitation (Grade 2–4+) before operation were divided into two groups imaged IP3DE or not, in our hospital from 1993 to 2020. We assessed the surgical outcome and re-intervention rate between two arms and re-evaluate AVV images before surgery compared to the IP3DE. Result IP3DE was performed in forty-six patients (IP3DE group) and thirty-nine patients were not (control group). Median age at AVV repair was 3.0/2.8 years, respectively. The AVV was tricuspid (n=25), mitral (n=41), or common (n=19). The IP3DE group had a significantly higher improvement in regurgitation of AVV (IP3DE: Grade 3.2±0.3 → 1.7±0.3 vs Control: Grade 2.8±0.3 → 1.8±0.3, p&lt;0.05). Fifty-nine percent of the IP3DE group was successful outcome (Grade&lt;1+ after repair). There was no significant difference in the rate of re-intervention after surgery between two groups. In multivariate analysis, using IP3DE contributed to successful outcome for AVV repair (OR: 4.66, 95% CI: 1.46–14.8, p&lt;0.01). The different and/or additional anatomical AVV findings were obtained in sixty-one percent of patients by the IP3DE. Conclusion IP3DE contributes to successful outcome for AVV repair by obtaining further information on complicated AVV anatomy in congenital heart disease. IP3DE also enables both cardiovascular surgeons and cardiologists to share the accurate and detail “surgeon's view” in the operating room for planning of AVV repair. FUNDunding Acknowledgement Type of funding sources: None.