The patient-specific aortic silicone model was established based on CTA data. The digital particle image velocimetry (DPIV) test method in the modified ViVitro pulsatile flow system was used to investigate the aortic hemodynamic performance and flow field characteristics before and after transcatheter aortic valve replacement (TAVR). The results showed that the hemodynamic parameters were consistent with the clinical data, which verified the accuracy of the model. From the comparative study of preoperative and postoperative effective orifice area (0.33 cm² and 1.78 cm²), mean pressure difference (58 mmHg and 9 mmHg), percentage of regurgitation (52% and 8%), peak flow velocity (4.60 m/s and 1.81 m/s) and flow field distribution (eccentric jet and uniform jet), the immediate efficacy after TAVR is good. From the perspective of viscous shear stress and Reynolds shear stress, the risk of hemolysis and thrombotic problems was low in preoperative and postoperative patient-specific models. This study provides a set of reliable DPIV testing methods for aortic flow field, and provides biomechanical basis for the immediate and long-term effectiveness of TAVR from the perspective of hemodynamics and flow field characteristics. It has important application value in clinical diagnosis, surgical treatment and long-term evaluation.
Humans ; Transcatheter Aortic Valve Replacement/methods* ; Aortic Valve/surgery* ; Heart Valve Prosthesis ; Hemodynamics ; Aortic Valve Stenosis/diagnosis* ; Treatment Outcome

Prenatal intervention of severe fetal aortic valve stenosis by ultrasound-guided percutaneous balloon valvuloplasty has been performed to prevent the progression to hypoplastic left heart syndrome, and achieve biventricular circulation in neonates. Here we report a case of fetal aortic valvuloplasty prenatally diagnosed with aortic stenosis at 24 weeks of gestation and showed worsening features on a follow-up echocardiography. Prenatal aortic valvuloplasty was performed at 29 weeks of gestation, and was a technical success. However, fetal bradycardia sustained, and an emergency cesarean delivery was performed. To the best of our knowledge, this is the first reported case of fetal aortic valvuloplasty which was performed in Asia.
Aortic Valve Stenosis ; Asia* ; Balloon Valvuloplasty ; Bradycardia ; Echocardiography ; Emergencies ; Fetal Heart ; Fetal Therapies ; Follow-Up Studies ; Humans ; Hypoplastic Left Heart Syndrome ; Infant, Newborn ; Pregnancy ; Prenatal Diagnosis

BACKGROUND AND OBJECTIVES: Prior studies indicate that up to 35% of cases of severe aortic stenosis (AS) have paradoxical low flow, low gradient despite preserved left ventricular ejection fraction (LVEF). However, error in left ventricular outflow tract (LVOT) diameter may lead to misclassification. Herein, we determined whether measurement of LVOT diameter by transesophageal echocardiography (TEE) results in reclassification of cases to non-severe AS. SUBJECTS AND METHODS: Patients with severe AS with aortic valve area (AVA) <1 cm2 by transthoracic echocardiography (TTE) within 6 months were studied. Paradoxical low flow, low gradient was defined as mean Doppler gradient (MG) <40 mm Hg and stroke volume index (SVI) ≤35 mL/m². Preserved LVEF was defined as ≥0.50. RESULTS: Among 108 patients, 12 (15%) had paradoxical low flow, low gradient severe AS despite preserved LVEF based on TTE measurement. When LVOT diameter by TEE in 2D was used, only 5 (6.3%) patients had low flow, low gradient severe AS (p<0.001). Coefficients of variability for intraobserver and interobserver measurement of LVOT were <10%. However, the limits of agreement between TTE and TEE measurement of LVOT ranged from 0.43 cm (95% confidence interval [CI]: 0.36 to 0.5) to -0.31 cm (95% CI: -0.38 to -0.23). CONCLUSION: TEE measured LVOT diameter may result in reclassification to moderate AS in some patients due to low prevalence of true paradoxical low flow, low gradient (PLFLG) severe AS.
Aortic Valve ; Aortic Valve Stenosis* ; Diagnosis* ; Echocardiography ; Echocardiography, Transesophageal* ; Humans ; Prevalence ; Stroke Volume

PURPOSE: Kabuki syndrome is a multiple congenital malformation syndrome, with characteristic facial features, mental retardation, and skeletal and congenital heart anomalies. However, the cardiac anomalies are not well described in the Korean population. We analyzed the cardiac anomalies and clinical features of Kabuki syndrome in a single tertiary center. METHODS: A retrospective analysis was conducted for a total of 13 patients with Kabuki syndrome. RESULTS: The median age at diagnosis of was 5.9 years (range, 9 days to 11 years and 8 months). All patients showed the characteristic facial dysmorphisms and congenital anomalies in multiple organs, and the diagnosis was delayed by 5.9 years (range, 9 days to 11 years and 5 months) after the first visit. Noncardiac anomalies were found in 84% of patients, and congenital heart diseases were found in 9 patients (69%). All 9 patients exhibited left-side heart anomalies, including hypoplastic left heart syndrome in 3, coarctation of the aorta in 4, aortic valve stenosis in 1, and mitral valve stenosis in 1. None had right-side heart disease or isolated septal defects. Genetic testing in 10 patients revealed 9 novel MLL2 mutations. All 11 patients who were available for follow-up exhibited developmental delays during the median 4 years (range, 9 days to 11 years 11 months) of follow-up. The leading cause of death was hypoplastic left heart syndrome. CONCLUSION: Pediatric cardiologist should recognize Kabuki syndrome and the high prevalence of left heart anomalies with Kabuki syndrome. Genetic testing can be helpful for early diagnosis and counseling.
Abnormalities, Multiple ; Aortic Coarctation ; Aortic Valve Stenosis ; Cause of Death ; Counseling ; Diagnosis ; Early Diagnosis ; Follow-Up Studies ; Genetic Testing ; Heart Defects, Congenital ; Heart Diseases ; Heart* ; Humans ; Hypoplastic Left Heart Syndrome ; Intellectual Disability ; Mitral Valve Stenosis ; Prevalence ; Retrospective Studies

INTRODUCTIONThe effects of reduction of left ventricular (LV) systemic afterload following aortic valve replacement (AVR) for severe aortic valve stenosis (AS) were investigated, using echocardiography and tissue Doppler imaging (TDI).

METHODSWe compared the preoperative and postoperative echocardiographic assessments of 23 patients with severe AS who had undergone isolated AVR (n = 13) or concomitant AVR with coronary artery bypass grafting (CABG) (n = 10). Conventional echocardiographic evaluations and TDI at the lateral mitral annulus were performed.

RESULTSEchocardiography was performed at a median of 120 (interquartile range: 66-141) days after AVR. There was significant reduction in aortic transvalvular mean pressure gradient after AVR. Although LV dimensions, mass and ejection fraction remained unchanged, LV diastolic and systolic functions improved (as observed on TDI). Early diastolic (E'), late diastolic (A') and systolic (S') mitral annular velocities increased significantly (p < 0.05). There was significant improvement in TDI-derived parameters among the patients who had isolated AVR, while among the patients who had concomitant AVR with CABG, only S' had significant improvement (p = 0.028).

CONCLUSIONTDI was able to detect improvements in LV systolic and diastolic function after AVR for severe AS. There was less improvement in the TDI-derived diastolic parameters among patients who underwent concomitant AVR with CABG than among patients who underwent isolated AVR.

Aged ; Aortic Valve ; surgery ; Aortic Valve Stenosis ; diagnosis ; surgery ; Coronary Artery Bypass ; Diastole ; Echocardiography ; Echocardiography, Doppler ; Female ; Heart Valve Prosthesis ; Humans ; Male ; Middle Aged ; Postoperative Period ; Systole ; Ventricular Function, Left

Cardiac papillary fibroelastomas (CPF) are benign cardiac tumors and usually discovered incidentally during echocardiography. This report describes the case of a 68-year-old man, referred to cardiology for multiple masses of the left ventricle and left atrium. The transthoracic echocardiography revealed multiple oscillating masses in the left ventricle and aortic valve, non-mobile mass in the left atrium with severe mitral stenosis and moderate aortic regurgitation. The patient underwent surgical resection of the masses with valve replacements. Histopathologic examination confirmed the diagnosis of CPF in the left ventricle and aortic valve, thrombus in the left atrium.
Aged ; Aortic Valve ; Aortic Valve Insufficiency ; Cardiology ; Diagnosis ; Echocardiography ; Heart Atria ; Heart Neoplasms ; Heart Ventricles ; Heart* ; Humans ; Mitral Valve Stenosis ; Thrombosis*

Despite the necessity of surgical aortic valve replacement, many patients with symptomatic severe aortic stenosis (AS) cannot undergo surgery because of their severe comorbidities. In these high-risk patients, percutaneous transcatheter aortic valve implantation (TAVI) can be safely accomplished. However, no study has shown that TAVI can be performed for patients with severe AS accompanied by acute decompensated heart failure. In this case report, 1 patient presented a case of severe pulmonary hypertension with decompensated heart failure after diagnosis with severe AS, and was successfully treated via emergency TAVI. Without any invasive treatment, acute decompensated heart failure with severe pulmonary hypertension is common in patients with severe AS, and it can increase mortality rates. In conclusion, TAVI can be considered one of the treatment options for severe as presented as acute decompensated heart failure patients with pulmonary hypertension.
Aortic Valve Stenosis ; Aortic Valve* ; Comorbidity ; Diagnosis ; Emergencies* ; Emergency Treatment ; Heart Failure ; Heart Valve Prosthesis Implantation ; Humans ; Hypertension, Pulmonary ; Mortality

BACKGROUNDAngina pectoris has been recognized as one of the principal symptoms of aortic valve stenosis (AS), even in patients without significant coronary artery disease (CAD). However, the incidence of angina pectoris and related CAD in such patients is controversial. There is continuing debate as to whether coronary angiography is necessary before aortic valve replacement (AVR) in patients with severe AS. The purpose of this study was to evaluate the incidence and predictors of CAD in patients with severe AS in a Korean population.

METHODSData from all consecutive patients with severe AS undergoing AVR at a major tertiary cardiac and vascular center in Korea were entered in a prospective registry beginning in 1995. Clinical and echocardiographic follow-up data were recorded into the database annually. Significant CAD was defined as one or more major coronary arteries having an estimated narrowing of ≥70% and left main coronary arteries having an estimated narrowing of ≥50% on coronary angiography. We excluded patients with multiple valve disease, significant aortic regurgitation, or prior CAD or valve surgery.

RESULTSTotally 574 patients with severe AS (mean age, (65.9±9.6) years) were enrolled in this study. Significant CAD was found in 61 patients (10.6%). Factors associated with increased likelihood of CAD were age, hypertension, diabetes mellitus, chronic renal failure, carotid disease, and aorta calcification. In Logistic regression analysis, the independent predictor of the presence of CAD was age (P = 0.011). The incidence of CAD increased significantly at 69.2 years of age. Having two risk factors for cardiovascular disease was the most useful cutoff to predict whether a patient was going to have significant CAD.

CONCLUSIONSThere was a low incidence of significant CAD in a population of Korean patients with severe AS. Therefore, coronary angiography before AVR will be considered in patients with multiple risk factors for cardiovascular disease or in patients more than 69 years of age without risk factors for cardiovascular disease.

Aged ; Angina Pectoris ; diagnosis ; epidemiology ; Aortic Valve ; surgery ; Aortic Valve Stenosis ; epidemiology ; surgery ; Coronary Angiography ; Coronary Artery Disease ; diagnosis ; epidemiology ; Echocardiography ; Humans ; Incidence ; Middle Aged

PURPOSE: Transcatheter aortic valve implantation (TAVI) has become an attractive therapeutic strategy for severe aortic stenosis (AS) in elderly patients due to its minimally-invasive nature. Therefore, early results of its clinical outcomes in elderly Korean patients were evaluated. MATERIALS AND METHODS: We compared early clinical outcomes of TAVI, surgical aortic valve replacement (SAVR), and optimal medical therapy (OMT) in patients aged > or =80 years with symptomatic severe AS. Treatment groups were allocated as follows: TAVI (n=10), SAVR (n=14), and OMT (n=42). RESULTS: Baseline clinical characteristics including predicted operative mortality were similar among the three groups. However, patients with New York Heart Association functional class III or IV symptoms and smaller aortic valve area were treated with TAVI or SAVR rather than OMT. In-hospital combined safety endpoints (all-cause mortality, major stroke, peri-procedural myocardial infarction, life-threatening bleeding, major vascular complication, and acute kidney injury) after TAVI or SAVR were significantly lower in the TAVI group than in the SAVR group (10.0% vs. 71.4%, respectively, p=0.005), along with an acceptable rate of symptom improvement and device success. During the follow-up period, the TAVI group showed the lowest rate of 3-month major adverse cardiovascular and cerebrovascular events, a composite of all-cause mortality, myocardial infarction, major stroke, and re-hospitalization (TAVI 0.0% vs. SAVR 50.0% vs. OMT 42.9%, p=0.017). CONCLUSION: Treatment with TAVI was associated with lower event rates compared to SAVR or OMT. Therefore, TAVI may be considered as the first therapeutic strategy in selected patients aged > or =80 years with symptomatic severe AS.
Age Factors ; Aged, 80 and over ; Aortic Valve/*surgery ; Aortic Valve Stenosis/diagnosis/*surgery ; Female ; Humans ; Male ; Prognosis ; Risk Factors ; Treatment Outcome

Isolated left ventricular (LV) apical hypoplasia is a rare congenital cardiac anomaly which is not accompanied by other cardiac abnormalities, with the exception of two cases. We report a case of a 33-year-old male patient with isolated LV apical hypoplasia combined with infundibular pulmonary stenosis and aortic stenosis. We review a literature focusing on the characteristic magnetic resonance features and combined cardiac abnormalities.
Adult ; Aortic Valve Stenosis/*complications/diagnosis ; Diagnosis, Differential ; Echocardiography ; Electrocardiography ; Humans ; Hypoplastic Left Heart Syndrome/*complications/diagnosis ; Magnetic Resonance Imaging, Cine ; Male ; Pulmonary Valve Stenosis/*complications/diagnosis