Interaction between ozone and paternal smoking on fetal congenital heart defects among pregnant women at high risk: a multicenter maternal-fetal medicine study.

BACKGROUND: Evidence remains limited on the association between maternal ozone (O3) exposure and congenital heart defects (CHDs) in offspring, and few studies have investigated the interaction and modification of paternal smoking on this association. METHODS: Using a sample including pregnant women at high risk of fetal CHD (with metabolic disease, first-trimester viral infection, family history of CHD, etc.) from a maternal-fetal medicine study covering 1313 referral hospitals in China during 2013-2021, we examined the associations between maternal O3 exposure during 3-8 weeks of gestational age and fetal CHD in offspring and investigated the interaction and modification of paternal smoking on this association. CHD was diagnosed by fetal echocardiograms, maximum daily 8-hour average O3 exposure data at a 10 km × 10 km spatial resolution came from the Tracking Air Pollution in China dataset, and paternal smoking was collected using questionnaires. Logistic regression models were used to estimate adjusted odds ratios (ORs) and 95% confidence intervals (CIs). RESULTS: Among 27,834 pregnant women at high risk of fetal CHD, 17.4% of fetuses were diagnosed with CHD. Each 10 µg/m3 increase in maternal O3 exposure was associated with a 17% increased risk of CHD in offspring (OR = 1.17, 95% CI = 1.14-1.20). Compared with paternal nonsmoking and maternal low O3 exposure, the ORs (95% CI) of CHD for smoking and low O3 exposure, nonsmoking and high O3 exposure, and smoking and high O3 exposure were 1.25 (1.08-1.45), 1.81 (1.56-2.08), and 2.23 (1.84-2.71), respectively. Paternal smoking cessation seemingly mitigated the increased risk of CHD. CONCLUSIONS: Maternal O3 exposure and paternal smoking were interactively associated with an increased risk of fetal CHD in offspring, which calls for effective measures to decrease maternal exposure to O3 pollution and secondhand smoke for CHD prevention.

Cardiac manifestations in a Chinese cohort of fetuses from mothers with anti-Ro and anti-La antibodies.

Objectives: To analyze the clinical characteristics, echocardiographic features, and prognosis of fetuses based on three groups of cardiac manifestations associated with maternal anti-Ro and anti-La antibodies in China. This study included three groups: the isolated-arrhythmia, isolated-endocardial fibroelastosis (EFE), and mixed groups. Methods: We prospectively evaluated 36 fetuses with cardiac manifestations due to maternal anti-Ro and anti-La antibodies from our center between 2016 and 2020 in China. Clinical and echocardiographic data were collected. Results: There were 13 patients (36%) in the isolated-arrhythmia group, eight (22%) in the isolated-EFE group, and 15 (42%) in the mixed group. All patients in the isolated-EFE group presented with mild EFE. Severe EFE was identified in four patients (27%) in the mixed group. Atrioventricular block (AVB) was more common in the isolated-arrhythmia group (13, 100%) than in the mixed group (6, 40%; p = 0.001). Moderate-severe mitral regurgitation (p = 0.006), dilated cardiomyopathy (DCM, p = 0.017), and low cardiovascular profile scores (p = 0.013) were more common in the mixed group than in the other two groups. Twenty-one mothers decided to terminate the pregnancy and 15 fetuses were born with regular perinatal treatment. They all survived at 1 year of age. One patient in the isolated-arrhythmia group and two in the mixed group required a pacemaker due to third-degree AVB or atrioventricular junctional rhythm. Five patients in the isolated-EFE group and five in the mixed group had no DCM or heart failure and the location of mild EFE was significantly reduced. Conclusion: Fetal cardiac manifestations due to maternal anti-Ro and anti-La antibodies can be divided into three groups, i.e., the isolated-arrhythmia, isolated-EFE, and mixed groups. AVB usually occurs in the isolated-arrhythmia group. Severe EFE, moderate-severe mitral regurgitation, and DCM mainly appear in the mixed group. Location of mild EFE significantly reduces after birth and the outcome of fetuses with mild EFE depends on the presence of arrhythmia and its subtypes.

Diagnosis of fetal total anomalous pulmonary venous connection based on the post-left atrium space ratio using artificial intelligence.

OBJECTIVE: To explore whether the post-left atrium space (PLAS) ratio would be useful for prenatal diagnosis of total anomalous pulmonary venous connection (TAPVC) using echocardiography and artificial intelligence. METHODS: We retrospectively included 642 frames of four-chamber views from 319 fetuses (32 with TAPVC and 287 without TAPVC) in end-systolic and end-diastolic periods with multiple apex directions. The average gestational age was 25.6 ± 2.7 weeks. No other cardiac or extracardiac malformations were observed. The dataset was divided into a training set (n = 540; 48 with TAPVC and 492 without TAPVC) and test set (n = 102; 20 with TAPVC and 82 without TAPVC). The PLAS ratio was defined as the ratio of the epicardium-descending aortic distance to the center of the heart-descending aortic distance. Supervised learning was used in DeepLabv3+, FastFCN, PSPNet, and DenseASPP segmentation models. The area under the curve (AUC) was used on the test set. RESULTS: Expert annotations showed that this ratio was not related to the period or apex direction. It was higher in the TAPVC group than in the control group detected by the expert and the four models. The AUC of expert annotations, DeepLabv3+, FastFCN, PSPNet, and DenseASPP were 0.977, 0.941, 0.925, 0.856, and 0.887, respectively. CONCLUSION: Segmentation models achieve good diagnostic accuracy for TAPVC based on the PLAS ratio.

Prenatal diagnosis of isolation of aortic brachiocephalic artery.

BACKGROUND: Isolated aortic brachiocephalic artery (IABA) is a rare congenital aortic arch anomaly. It is difficult to diagnose IABA prenatally and the prevalence in the prenatal population is unknown. PURPOSE: To evaluate the echocardiographic characteristics and associations in fetuses with IABA. MATERIAL AND METHODS: We retrospectively analyzed all cases of prenatal diagnosis of IABA from January 2012 to November 2020 and reviewed the follow-up results. Copy Number Variation Sequencing (CNV-Seq) was performed using the biological specimens of the of the fetuses and family members. RESULTS: Ten cases (10/45652, 0.022%) of IABA were identified in our center. The prevalence of the cases with isolated left subclavian artery (ILSCA) in the right aortic arch (RAA) population was 0.98% (6/613). The ILSCA was the most common isolated arch branch. All the isolated branches were on the opposite side of aortic arch in all the cases. The "ice stick" sign in the coronal section could be seen in most cases of IABA. Of the 10 cases, 8 (8/10, 80%) were associated with tetralogy of Fallot (TOF). Two cases of IABA were combined with 22q11.2 deletion syndrome. CONCLUSION: IABA is a rare aortic anomaly. ILSCA was the most common isolated arch branch and TOF was the most common associated intra-cardiac anomaly. The "ice stick" sign in the coronal section could indicate a diagnosis of the IABA.

Fetal atrial appendage aneurysm: Prenatal diagnosis by echocardiography and prognosis.

BACKGROUND: Congenital atrial appendage aneurysm (AAA) is a rare malformation which can coexist with potentially lethal complications. We aimed to summary echocardiographic characteristics and prognosis of fetal AAA. METHODS: We retrospectively analyzed the echocardiographic data of 17 fetuses with AAA，and their outcomes or pathological reports were also collected. RESULTS: Eight fetuses with left AAA (LAAA) and 9 fetuses with right AAA (RAAA) were identified. Five fetuses were diagnosed with other cardiac defects. Two fetuses with RAAA presented with arrhythmias, including atrial premature beats (n = 1) and bradyarrhythmia (n = 1). LAAA could be detected by four-chamber view (50.0%) and short-axis view (100.0%). RAAA could be detected by four-chamber view (100.0%), and view of right ventricular inflow tract (33.3%). There were three cases with mild pericardial effusion. Three cases with complex cardiac defects were selectively terminated, with confirmation of LAAA by autopsy in one case. Fourteen fetuses were born. After following 2 (range, 1-5) years, the AAA disappeared in one case with LAAA and two cases with RAAA. While, 11 cases were still diagnosed with AAA. Atrial premature beats with RAAA, which appeared in prenatal period, still persisted after birth. CONCLUSION: Congenital AAA is a rare abnormality in utero. The short-axis view and the four-chamber view were the most useful views to detect fetal AAA. Fetal AAA may disappear in childhood. Atrial tachyarrhythmias in utero may exist persistently after birth. Patients with AAA should be followed up closely and appropriate intervention should be taken when complications appeared.

Treatment and follow-up of fetuses that developed congenital heart block due to autoantibody in two cases.

OBJECTIVE: Autoantibody-related congenital heart block (ACHB) is a passively acquired autoimmune disease. This study aimed to examine the pathogenesis, clinical manifestations, and treatment of ACHB. METHOD: The clinical data of two fetuses with first-degree ACHB were retrospectively analyzed. RESULTS: Two pregnant women were strongly positive for anti-Sjögren's syndrome-related antigen A (SSA) antibody. Among these two cases, one had a prolonged atrioventricular (AV) interval at 28+3 weeks in utero, while the other had a prolonged AV interval at 24+6 weeks in utero. After prenatal intervention, one patient recovered to normal, while one fetus continued to have ACHB after treatment with dexamethasone and intravenous immunoglobulin. Furthermore, the two neonates were positive for anti-SSA antibody and were diagnosed with ACHB. CONCLUSION: The pathogenesis of ACHB is closely correlated with anti-SSA/Ro antibody and anti-SSB/La antibody from the mother, and is affected by fetal susceptibility. Early screening and early intervention for ACHB are important.

Prenatal diagnosis of Marfan syndrome by fetal echocardiography: A case report and review of cardiovascular manifestations.

Neonatal Marfan syndrome (nMFS), phenotypically and genotypically distinct from the classical syndrome, is rarely diagnosed prenatally, and the cardiovascular prognosis is poor. This case report described one fetus diagnosed with nMFS by fetal echocardiography. The main features were cardiomegaly, and atrioventricular valves prolapse with moderate regurgitation and dilated great vessels. Extracardiac malformations included right diaphragmatic eventration, bilateral pyelectasis, and lengthy femur. Pathological examination confirmed the findings of fetal echo, and all cardiac valves were dysplastic. Sanger sequencing revealed a deletion mutation affecting exon 30 of the fibrillin 1 (FBN1) gene. Echocardiography is essential for prenatal diagnosis, and multivalve dysplasia is common among those patients.

Prenatal diagnosis of Berry syndrome by fetal echocardiography: A report of four cases.

Berry syndrome is a rare congenital cardiac malformation. We describe 4 cases of Berry syndrome diagnosed by fetal echocardiography. Based on our experience, the three-vessel view is important for diagnosing the aortopulmonary window and aortic origin of the right pulmonary artery. Furthermore, the true cross-sectional and sagittal views obtained by continuously scanning from the three-vessel-trachea view to the long-axis view of the aortic arch are required to image the interruption or coarctation of the aortic arch. An early and accurate prenatal diagnosis of Berry syndrome is feasible and helps to improve patient outcomes.

Association of peak atrial longitudinal strain with atrial fibrillation recurrence in patients with chronic lung diseases following radiofrequency ablation.

BACKGROUND: Strain was shown associated with atrial fibrillation (AF) recurrence after radiofrequency ablation (RFA), but data on AF patients complicated with chronic lung diseases (CLD) were rare. AIM: This study was designed to evaluate the relationship of baseline atrial function with AF recurrence in these patients using speckle-tracking echocardiography. METHODS: Average strain values (median: 2 days before RFA) were calculated for 87 AF patients (Mean age: 61.91 years, male: 71.26%) with CLD undergoing RFA from 2013 to 2014. Of these patients, 25 (28.74%) experienced AF recurrence during a mean follow up of 10.3 months. RESULTS: Peak right atrial longitudinal strain (R-PALS) was associated with peak left atrial longitudinal strain (L-PALS, Standardised ß = 0.45, P < 0.001) in multivariate linear regression. Multivariate Cox regression analysis showed R-PALS was associated with AF recurrence (hazard ratio, 0.86; 95% confidence interval (CI), 0.78-0.96, P = 0.005) in CLD. Patients with R-PALS ≥14.69% had higher AF free rate compared with R-PALS<14.69% using Kaplan-Meier analysis (log-rank, P < 0.001). R-PALS had similar C-index compared to L-PALS (difference: 0.03, 95%CI: -0.06-0.12, P = 0.53) and combined R-PALS and L-PALS (difference: 0.005, 95%CI: -0.04-0.05, P = 0.84) associated with AF recurrence in CLD. CONCLUSION: R-PALS, L-PALS and combined R-PALS and L-PALS are important factors associated with AF recurrence following RFA in patients with CLD.

The effect of statins therapy in aortic stenosis: Meta-analysis comparison data of RCTs and observationals.

Aortic stenosis has been shown to share the same risk factors as atherosclerosis which suggested a potential benefit from statins therapy. Fourteen studies which provided the effect of statins treatment on aortic stenosis (AS) were meta-analyzed, including 5 randomized controlled trials (RCTs) and 9 observational studies. In the RCTs, statins did not have any influence on peak aortic valve velocity, peak valve gradient, mean valve gradient, aortic valve area and aortic calcification compared to controls. In the observational studies, the peak valve velocity, peak gradient and aortic valve area showed less progression in the statins group compared to controls. This article describes data related article title "The effect of statins on valve function and calcification in aortic stenosis: a meta-analysis" (Zhao et al., 2016) [1].

The effect of statins on valve function and calcification in aortic stenosis: A meta-analysis.

BACKGROUND: Aortic calcification has been shown to share the same risk factors as atherosclerosis which suggested a potential benefit from statins therapy. In view of the existing conflicting results, we aimed to provide objective evidence on the effect of statins in aortic stenosis (AS). METHODS AND RESULTS: A meta-analysis of eligible studies that used statins in AS was performed. Fourteen studies were identified, 5 randomized controlled trials (RCTs) and 9 observational studies. In the 14 studies as a whole, no significant differences were found in all cause mortality (OR = 0.98, p = 0.91), cardiovascular mortality (OR = 0.80, P = 0.23) or the need for valve replacement (OR = 0.93, p = 0.45) between the statins and the control groups. LDL-cholesterol dropped in the statins groups in both <24 months and ≥ 24 months follow-up (p < 0.001 for both) but not in controls (p = 0.35 and p = 0.33, respectively). In the <24 months statins group, the annual increase in peak aortic velocity and peak gradient was less (p < 0.0001 and p = 0.004, respectively), but the mean gradient, valve area and calcification score were not different from controls. In the ≥ 24 months statins group, none of the above parameters was different from controls. CONCLUSIONS: Despite the consistent beneficial effect of statins on LDL-cholesterol levels, the available evidence showed no effect on aortic valve structure, function or calcification and no benefit for clinical outcomes.

Intramyocardial Dissecting Hematoma after Acute Myocardial Infarction-Echocardiographic Features and Clinical Outcome.

OBJECTIVES: Intramyocardial dissecting hematoma (IDH) after acute myocardial infarction (MI) is a rare form of subacute cardiac rupture and hence management uncertainties. The objective of this study was to describe the clinical course of a small series of IDH patients and to review the available evidence for managing similar cases. METHODS: Eight IDH patients from our center had echocardiographic, coronary angiographic and clinical outcome data reviewed. PubMed was also searched for IDH following MI. Cases were divided into three groups and compared according to the dissection location. RESULTS: In our 8 patients, 3 had septal, 1 right ventricular (RV), and 4 left ventricular (LV) dissection. Five were medically treated and 3 surgically repaired. Reviewing the literature revealed 68 IDH patients, of mean age 66 ± 10 years, 43 males. The percentage of IDH involving the LV free wall, septal, and RV free wall were 47%, 26.5%, and 26.5%, respectively. In the cohort as a whole, mortality was not different between surgically and medically treated patients (33.3% vs. 54.3%, P = 0.08), neither based on the IDH location (P = 0.49). While surgical and medical treatment of the LV free wall (20.0% vs. 40.9%, P = 0.25) and septal (46.2% vs. 60.0%, P = 0.60) were not different, surgical repair of RV free wall had significantly better survival (30.0% vs. 87.5%, P = 0.015). The LVEF (P = 0.82), mitral regurgitation (P = 0.49) failed to predict mortality. CONCLUSION: While survival following medical and surgical treatment of LV IDH is not different, patients with RV free wall dissection benefit significantly from surgical repair.

Analysis of etiology, chromosome and prognosis for small left heart system development in 69 fetuses.

OBJECTIVE: To provide a basis for evaluating the prognosis of small left heart system development in fetuses, we analyzed its related factors. METHODS: The fetal echocardiogram was performed in 3859 pregnant women, and then small left heart system development was identified in 69 fetuses. The data of prenatal and postnatal echocardiograms, postnatal cardiac surgical treatment, chromosome and autopsy after induced labor were analyzed in the 69 fetuses. RESULTS: Except 1320 cases losing follow-up, 2539 cases had complete data. Among the 2539 cases, small left heart system development was identified in 69 fetuses. Of the 69 fetuses, 12 had hypoplastic left heart syndrome, 20 premature closure of foramen ovale, 13 total anomalous pulmonary venous drainage, 2 common pulmonary vein lumen atresia, 21 aortic coarctation or interruption and 1 right pulmonary hypoplasia. Among the 69 fetuses, chromosome abnormality was found in 7. CONCLUSION: There are many etiological factors causing small left heart system development. The prognosis is poor in the fetuses with hypoplastic left heart syndrome, common pulmonary vein lumen atresia, pulmonary hypoplasia, other malformations or/and chromosome abnormality. Fetal echocardiography combined with chromosome examination can provide important bases for making diagnosis and evaluating the prognosis regarding small left heart system development.

Analysis of Renal Artery Stenosis in Patients with Heart Failure: A RASHEF Study.

BACKGROUND: Previous data are controversial about the association of renal artery stenosis (RAS) with clinical outcome in patients with heart failure. Definition of RAS in previous studies might not be appropriate. By definition of RAS with renal duplex sonography, we investigated the association of RAS with clinical outcome in patients with heart failure. METHODS: In this retrospective study, we identified 164 patients with heart failure (New York Heart Association classification ≥II; left ventricular ejection fraction <50%) who had received renal duplex sonography during hospital stay. RAS was defined as renal-aortic ratio ≥3.5 or a peak systolic velocity ≥200 cm/s (or both), or occlusion of the renal artery. Categorical data of patients were compared using the Chi-square test or Fisher's exact test. Cox proportional hazards regression modeling technique was used to investigate the prognostic significance of possible predictors. RESULTS: Finally, 143 patients were enrolled. Median follow-up time was 32 months (1-53 months). Twenty-two patients were diagnosed as RAS by renal duplex sonography, including 13 unilateral RAS (3 left RAS, 10 right RAS) and 9 bilateral RAS. There were more all-cause mortality and cardiovascular death in patients with RAS than patients without RAS. By multivariate analysis, RAS was a significant predictor for all-cause death and cardiovascular death (hazard ratio [HR] = 4.155, 95% confidence interval [CI]: 1.546-11.164, P = 0.005; and HR = 3.483, 95% CI: 1.200-10.104, P = 0.022, respectively). As for composite endpoint events, including death, nonfatal myocardial infarction, ischemic stroke or intracranial hemorrhage, rehospitalization for cardiac failure, and renal replacement therapy, only angiotensin-converting enzyme inhibitor or angiotensin-receptor blocker was significant predictor. RAS was not a significant predictor for composite endpoint events. CONCLUSIONS: Our data suggested that RAS is associated with a poorer clinical outcome in patients with heart failure.

Right atrial cardiac rhabdomyoma with premature foramen ovale restriction: A case report.

Fetal cardiac rhabdomyoma is the most common cardiac tumor in fetuses. However, this benign tumor can cause hemodynamic repercussions and intrauterine fetal mortality. The present study reports a case of rare fetal cardiac rhabdomyoma located in the right atrium, accompanied by premature restriction of the foramen ovale and moderate pericardial effusion, as determined by tomographic ultrasound imaging (TUI). Fetal mortality subsequently occurred late in the second trimester of pregnancy and the diagnosis was confirmed by pathology. The present study discusses the occurrence and diagnosis of this rare abnormality. TUI mode with spatio-temporal image correlation offline imaging provides the physician with clear views of abnormal intracardiac structures in the beating heart. With improvements in sonographic technology, the diagnosis of fetal cardiac rhabdomyoma may be easier and more accurate in the clinical arena.

Utility of echocardiographic tissue synchronization imaging to redirect left ventricular epicardial lead placement for cardiac resynchronization therapy.

BACKGROUND: Cardiac resynchronization therapy (CRT) with biventricular pacing has demonstrated cardiac function improvement for treating congestive heart failure (HF). It has been documented that the placement of the left ventricular lead at the longest contraction delay segment has the optimal CRT benefit. This study described follow-up to surgical techniques for CRT as a viable alternative for patients with heart failure. METHODS: Between April 2007 and June 2012, a total of 14 consecutive heart failure patients with New York Heart Association (NYHA) Class III-IV underwent left ventricular epicardial lead placements via surgical approach. There were eight males and six females, aged 36 to 79 years ((59.6 ± 9.2) years). The mean left ventricular ejection fraction (LVEF) was (33.6 ± 7.4)%. All patients were treated with left ventricular systolic dyssynchrony and underwent left ventricular epicardial lead placements via a surgical approach. Tissue Doppler imaging (TDI) and intraoperative transesophageal echocardiography were used to assess changes in left heart function and dyssynchronic parameters. Also, echo was used to select the best site for left ventricular epicardial lead placement. RESULTS: Left ventricular epicardial leads were successfully implanted in the posterior or lateral epicardial wall without serious complications in all patients. All patients had reduction in NYHA score from III-IV preoperatively to II-III postoperatively. The left ventricular end-diastolic diameter (LVEDD) decreased from (67.9 ± 12.7) mm to (61.2 ± 7.1) mm (P < 0.05), and LVEF increased from (33.6 ± 7.4)% to (42.2 ± 8.8)% (P < 0.05). Left ventricular intraventricular dyssynchrony index decreased from (148.4 ± 31.6) ms to (57.3 ± 23.8) ms (P < 0.05). CONCLUSIONS: Minimally invasive surgical placement of the left ventricular epicardial lead is feasible, safe, and efficient. TDI can guide the epicardial lead placement to the ideal target location.

Diagnostic value of an ROC curve of the size of the antepartum foramen ovale in the prediction of puerperal atrial septal defect.

The aim of this study was to determine the diagnostic value of an ROC curve of the antepartum foramen ovale (AFO) size and the ratio of FO size to aorta (AO) size (FO/AO) for the prediction of puerperal atrial septal defect in different gestational weeks (DGWs). A total of 958 cases were divided into five groups according to number of gestational weeks. Comparisons of FO size, AO size and FO/AO were determined by variance analysis. The correlations between FO size, AO size and gestational age were determined using regression analysis and comparisons between atrial septal defect (ASD) diagnosed in DGWs and normal cardiac FO size and FO/AO were analyzed by t-test. ROC curve analysis was used for FO size and FO/AO to predict the demarcation point of puerperal ASD (pASD). The differences between FO size and AO size in the five groups at DGWs were statistically significant (P=0.000). The sizes of FO and AO increased with gestational age. The differences among pASD, normal cardiac FO size and FO/AO were statistically significant (P=0.000). FO size in the five DGW groups (18-22, 23-26, 27-30, 31-34 and 35-40 weeks) was able to predict the demarcation points of pASD, which were 5.02, 5.15, 6.55, 8.55 and 7.90 mm, respectively. The prediction of pASD with AFO size and FO/AO was accurate and may provide reliable reference values in the clinic.

Premature closure or restriction of the foramen ovale: prenatal diagnosis by directional enhanced flow imaging.

Premature closure or restriction of the foramen ovale may occur at any time during pregnancy and may be due to various causes. We describe 2 patients with premature closure or restriction of the foramen ovale during the third trimester. In both patients the foramen ovale was detected by directional enhanced flow imaging technology (DeFLOW; Hitachi-Aloka Medical, Ltd, Tokyo, Japan), a novel method of imaging blood flow dynamics. Our findings indicate that D-eFLOW can display blood flow information with higher sensitivity and resolution than conventional methods, helping obstetricians and pregnant women make timely decisions about delivery.

Parachute mitral valve accompanied by bicuspid aortic valve on three-dimensional transesophageal echocardiography.

We report the findings of three-dimensional (3D) transesophageal echocardiography (TEE) in a patient with a parachute mitral valve (MV) accompanied by aortic valve (AV) malformation. The results indicated an enhanced echo in MV anterior leaves, incrassate, and shortened subvalvular chordae tendineae, and posteromedial papillary muscle that had echo reinforcement, calcification, retroposition, and a significant decrease compared with anterolateral papillary muscle. In addition, the anterolateral papillary muscle was huge, with the bilateral papillary muscles fused partly, and the posterior subvalvular chordae tendineae incrassate, shortened, and attached parachute-like to the anterolateral papillary muscle. The MV appeared dome-shaped for the open limit in diastole with an MV area of 1.6 cm. Moreover, the left ventricle increased in size and the bicuspid AV was malformed. Continuous wave Doppler angiograph showed that the flow rate increased to 398 cm/seconds at the AV orifice area. A 3D form of the MV structure was observed from the left ventricle using 3D-TEE inspection. The anterolateral papillary muscle was fused with its posteromedial homologue. The chordae tendineae was attached to the anterolateral papillary with the parachute-like structure, indicating dome movement.