Long-term outcome and cardiac function after anatomic repair of congenitally corrected transposition.

OBJECTIVES: There is limited information on long-term outcomes and trajectories of ventricular and valvular functions in patients with congenitally corrected transposition of the great arteries after anatomic repair according to the operative strategy with a median follow-up period of more than 10 years. METHODS: Twenty-nine patients who underwent anatomic repair in Okayama University Hospital between January 1994 and December 2020 were reviewed. Outcomes were compared between patients who underwent a double switch operation (DS group) and patients with an atrial switch with a Rastelli operation (Rastelli-Senning/Mustard group). RESULTS: Fifteen (52%) were in the DS group and 14 (48%) were in the Rastelli-Senning/Mustard group. The median follow-up period after anatomic repair was 12.7 (interquartile range 4.2-18.8) years. There were 3 (10%) early deaths and 3 (10%) late deaths. Survival rates for the entire cohort at 10 and 20 years were 86% and 71%, respectively, and were not different between the 2 groups. Using competing risk analysis, risks of heart failure, cardiac rhythm device implantation and atrial arrhythmia showed no significant differences between the 2 groups, whereas risk of reoperation was higher in the Rastelli-Senning/Mustard group than that in the DS group. Four patients after a DS operation and 1 patient after a Rastelli technique developed more than moderate aortic regurgitation. CONCLUSIONS: During a median follow-up period of more than 10 years, mortality rate and ventricular and valvular functions after anatomic repair were acceptable, though the incidences of late complications were relatively high, especially in the Rastelli-Senning/Mustard group.

Relationship between patent foramen ovale anatomical features and residual shunt after patent foramen ovale closure.

Transcatheter closure of patent foramen ovale (PFO) is an effective strategy for preventing recurrence of paradoxical embolism. However, PFO closure is often associated with residual shunt, which is a risk of recurrent stroke. This study aimed to evaluate the relationship between the anatomical features of PFO and residual shunt. The degree of residual shunt and its relationship with the anatomical features of PFO were evaluated in 106 patients who underwent PFO closure at our institution between March 2011 and January 2022 and in whom contrast transthoracic echocardiography was performed 1 year later. The mean PFO tunnel length was 9.3 ± 3.6 mm and the mean PFO height was 3.2 ± 2.2 mm. Atrial septal aneurysm (ASA) was found in 37 patients. After PFO closure, residual shunt was observed in 28 patients (grade 1, n = 8; grade 2, n = 16; grade 3, n = 3; grade 4, n = 1). Univariate logistic analysis identified ASA to be associated with residual shunt (odds ratio 2.78, 95% confidence interval 1.14 to 6.79; p = 0.024). There was no association of residual shunt with the size of the PFO, the length of PFO tunnel, or the size of the device used for closure. Two of four patients with a large residual shunt of grade 3 or grade 4 were found to have device size mismatch. Residual shunt after PFO closure was observed in a quarter of patients and was related to the presence of ASA. A few patients had a large residual shunt due to the device size mismatch.

Association Between Aortic Valve Calcification and Severity of Concomitant Aortic Regurgitation in Patients With Severe Aortic Stenosis.

BACKGROUND: Patients with severe aortic stenosis (AS) frequently have concomitant aortic regurgitation (AR), but the association between aortic valvular calcification (AVC) and the severity of AR remains unclear.Methods and Results: We retrospectively reviewed patients with severe AS who underwent transthoracic echocardiography and multidetector computed tomography (MDCT) within 1 month. The patients were divided into 3 groups according to the degree of concomitant AR. The association between AVC and the severity of concomitant AR was assessed in patients with severe AS. The study population consisted of 95 patients: 43 men and 52 women with a mean age of 82±7 years. Of the 95 patients with severe AS, 27 had no or trivial AR, 53 had mild AR, and 15 had moderate AR. The AVC score (AVCS) and AVC volume (AVCV) significantly increased as the severity of concomitant AR increased (P=0.014 for both), and similar findings were obtained for the AVCS and AVCV indexes (P=0.004 for both). CONCLUSIONS: The severity of AR correlated with AVCS and AVCV measured by MDCT in patients with severe AS. AVC may cause concomitant AR, leading to worsening of disease condition.

Detection of hepatocellular carcinoma during Fontan-associated liver disease follow-up: a report of three cases.

We herein demonstrate three patients diagnosed with early hepatocellular carcinoma (HCC) during follow-up for Fontan-associated liver disease (FALD). Case 1: Twenty-one years after undergoing the Fontan procedure, a 26-year-old female was diagnosed with FALD. At the initial consultation, her serum alpha-fetoprotein (AFP) levels were markedly elevated, and dynamic enhanced computed tomography (CT) revealed HCC measuring 40 mm in diameter. She underwent partial hepatectomy. Ten months later, she underwent conventional transcatheter arterial chemoembolization (cTACE) for recurrent HCC near the resected hepatic stump as a curative treatment. Case 2: Twenty-one years after undergoing the Fontan procedure, a 25-year-old male was diagnosed with FALD and underwent HCC surveillance every 6 months. Thirteen months after the initial consultation, dynamic enhanced CT revealed HCC measuring 10 mm in diameter. He received cTACE as a curative treatment. Case 3. Twenty-eight years after undergoing the Fontan procedure, a 37-year-old male was diagnosed with FALD and underwent HCC surveillance every 3 months. Fourteen months later, abdominal ultrasonography (US) revealed HCC measuring 13 mm in diameter. He received radiofrequency ablation. These cases showed that HCC surveillance using abdominal US and AFP measurements in patients with FALD enables the detection of HCC and increases the chance of a cure.

Pulmonary arteriovenous fistula in a rare location: The importance of excluding patent foramen ovale.

A 46-year-old woman with a history of repeated thromboembolic stroke and anti-phospholipid antibody syndrome was referred to our hospital. Saline contrast transthoracic echocardiography showed that microbubbles appeared in the left atrium within 4 heartbeats. Thus, she was initially suspected as having a patent foramen ovale with associated paradoxical embolism. However, no evidence of patent foramen ovale or atrial septal defect could be found using transesophageal echocardiography. Saline contrast transesophageal echocardiography showed that microbubbles flowed into the left atrium through the left superior pulmonary vein. Ultimately, she was diagnosed as having a pulmonary arteriovenous malformation located at the upper left pulmonary lobe using contrast computed tomography and pulmonary artery angiography. Pulmonary arteriovenous malformations are typically located in the lower lobe of either lung and, in bubble studies, contrast appears in the left atrium after 4 heartbeats. Here, the pulmonary arteriovenous malformation was in the upper lobe, and contrast appeared in the left atrium at an earlier time point: one associated with patent foramen ovale. These findings made it difficult to differentiate the two diseases initially. This case suggests that pulmonary arteriovenous malformation should be carefully considered, even if microbubbles appear in the left atrium early on a saline contrast transthoracic echocardiograph. Learning objective: Pulmonary arteriovenous malformation occasionally appears in the upper lobe. In these cases, microbubbles may appear in the left atrium after detection in the right atrium with a time-course that is suggestive of a patent foramen ovale. Therefore, diagnosis should be carefully confirmed by using other multimodal imaging tests, such as transesophageal echocardiography, contrast computed tomography, or pulmonary artery angiography.

Association between Cardiovascular Disease and Liver Disease, from a Clinically Pragmatic Perspective as a Cardiologist.

Cardiovascular diseases and liver diseases are closely related. Non-alcoholic fatty liver disease has the same risk factors as those for atherosclerotic cardiovascular disease and may also be a risk factor for atherosclerotic cardiovascular disease on its own. Heart failure causes liver fibrosis, and liver fibrosis results in worsened cardiac preload and congestion. Although some previous reports regard the association between cardiovascular diseases and liver disease, the management strategy for liver disease in patients with cardiovascular diseases is not still established. This review summarized the association between cardiovascular diseases and liver disease. In patients with non-alcoholic fatty liver disease, the degree of liver fibrosis progresses with worsening cardiovascular prognosis. In patients with heart failure, liver fibrosis could be a prognostic marker. Liver stiffness assessed with shear wave elastography, the fibrosis-4 index, and non-alcoholic fatty liver disease fibrosis score is associated with both liver fibrosis in patients with liver diseases and worse prognosis in patients with heart failure. With the current population ageing, the importance of management for cardiovascular diseases and liver disease has been increasing. However, whether management and interventions for liver disease improve the prognosis of cardiovascular diseases has not been fully understood. Future investigations are needed.

Temporary balloon occlusion test can overestimate the risk of acute pulmonary edema after transcatheter atrial septal defect closure.

BACKGROUND: Atrial septal defect (ASD) closure can cause acute pulmonary edema. Before transcatheter closure is performed, temporary balloon occlusion test (BOT) is recommended in patients with left ventricular dysfunction to predict the risk of pulmonary edema. However, the accuracy of BOT has not been verified. This study aimed to compare hemodynamic differences between BOT and transcatheter closure. METHODS: A total of 42 patients with a single ASD over age 18 years who underwent BOT before transcatheter ASD closure between October 2010 and May 2020 were analyzed. Pulmonary capillary wedge pressure (PCWP) was measured using a Swan-Ganz catheter placed in the pulmonary artery at baseline, after 10 min of BOT, and after transcatheter closure. Amplatzer septal occluder was used for all transcatheter closures. RESULTS: Mean patient age was 64 ± 18 years (range, 18-78). Mean ASD diameter and pulmonary to systemic flow ratio were 18 ± 5 and 2.8 ± 1.0 mm, respectively. Mean PCWP at baseline, during BOT, and after transcatheter closure was 8.9 ± 2.9, 13.5 ± 4.2, and 9.5 ± 2.6 mmHg, respectively. The difference between BOT and after transcatheter closure values was significant (p < 0.001). During BOT, PCWP increased ≥18 mmHg in 7 patients, whereas after ASD closure, PCWP was <18 mmHg in all 7 and none developed acute pulmonary edema. CONCLUSION: Temporary balloon occlusion of an ASD and transcatheter ASD closure result in different hemodynamic change. BOT overestimates increase of PCWP after transcatheter ASD closure and requires careful interpretation. Well-designed, larger studies in higher-risk patients are warranted to verify the clinical implications of BOT in more detail.

Survey results: status report on problems caused by sexual mismatch between sonographer and patient during echocardiography-a 2020 report of the Japanese Society of Echocardiography.

OBJECTIVES: Ultrasonography is an essential examination performed in various clinical fields. The number of clinical sonographers has been increasing. However, the working environments and conditions at each facility are different, leading to diverse problems. Among them, the emerging issue is the sexual disagreement between the sonographer and patient at the time of echocardiography. Since the patient must expose their breast during echocardiography, female patients may refuse to undergo the examination when conducted by a male sonographer. This study aimed to conduct a questionnaire survey to understand the measures for sonographer-patient gender mismatch at different facilities. METHODS: A questionnaire on the implementation of echocardiography by male sonographers for female patients was answered by representatives and specialist technicians of the Japanese Society of Echocardiography. RESULTS: Questionnaire responses were obtained from 50 facilities (59 participants). A total of 70% of the facilities restricted male sonographers from conducting echocardiography examinations for female patients. Among them, 81% of the facilities serviced female patients aged 60 years or younger. CONCLUSIONS: It has become clear that the gender selection of echocardiographic examiners varies from facility to facility, and providing a sufficient explanation before echocardiographic examination is necessary to avoid causing uncomfortable situations for female patients.

Left atrial appendage morphology with the progression of atrial fibrillation.

Left atrial appendage (LAA) size is crucial for determining the indication of transcatheter LAA closure. The aim of this study was to evaluate the differences in LAA morphology according to the types of atrial fibrillation (AF). A total of 299 patients (mean age: 67 ± 13 years) who underwent transesophageal echocardiography (TEE) were included. Patients were classified into non-AF (n = 64), paroxysmal AF (n = 86), persistent AF (n = 87), or long-standing persistent AF (n = 62). LAA morphology, including LAA ostial diameter and depth, was assessed using TEE. Patients with long-standing persistent AF had larger LAA ostial diameter and depth and lower LAA flow velocity. The maximum LAA ostial diameter was 19 ± 4 mm in patients with non-AF, 21 ± 4 mm in patients with paroxysmal AF, 23 ± 5 mm in patients with persistent AF, and 26 ± 5 mm in patients with long-standing persistent AF. LAA ostial diameter was increased by 2 or 3 mm with the progression of AF. LAA ostial diameter was correlated with LA volume index (R = 0.37, P < 0.01) and the duration of continuous AF (R = 0.30, P < 0.01), but not with age or the period from the onset of AF. In conclusion, LAA size was increased with the progression of AF.

Overview of the 86th Annual Scientific Meeting of the Japanese Circulation Society　- Cardiology Spreading Its Wings.

The 86thAnnual Scientific Meeting of the Japanese Circulation Society was held in a web-based format on March 11-13, 2022. In accordance with the internationalization policy of the JCS, the meeting was held with the Asian Pacific Society of Cardiology Congress 2022. The main theme was "Cardiology Spreading its Wings". The number of patients with heart failure and other cardiovascular diseases is increasing dramatically, and the fields dealt with by cardiovascular medicine are also greatly expanding. This conference was both intellectually satisfying and exciting for all participants, who numbered over 14,900. The meeting was completed with great success, and the enormous amount of cooperation and support from all involved was greatly appreciated.

Vascular occlusion with 0.035-inch hydrogel expandable coils in congenital heart diseases and vascular anomalies.

BACKGROUND: We present our experience with transcatheter vascular occlusion using 0.035-inch hydrogel expandable coils, which has been reported only in a few cases in the pediatric cardiology fields. METHODS: This study is a retrospective analysis of all patients who underwent transcatheter embolization with 0.035-inch hydrogel coils at the Department of Pediatrics, Okayama University Hospital, between October 2018 and September 2020. RESULTS: Twenty patients with a median age of 5.1 years (0.05-26.0 years) and a median weight of 13.8 kg (3.0-56.8 kg) were included. A total of fifty-four 0.035-inch hydrogel coils, including 35 Azur 35 and nineteen Azur CX 35 coils (Terumo, Tokyo, Japan), were successfully deployed in 22 target vessels. The target vessels consisted of 10 aortopulmonary collaterals, 8 veno-venous collaterals, and 4 pulmonary arteriovenous malformations. We achieved technical success in all the target vessels. In total, the mean target vessel diameter was 4.4 mm, the mean number of 0.035-inch hydrogel coils was 2.5 per vessel. The mean device to vessel ratio was 1.6 for the anchor coil and 1.2 for the additional coil. Post-implantation angiograms revealed that the primary occlusion rate was 18/22 (82%). There were no periprocedural complications. CONCLUSIONS: The 0.035-inch hydrogel expandable coils are effective and safe in patients with congenital heart disease and vascular anomalies. These occlusion devices could be valuable options for interventional pediatric cardiologists.

Efficacy of shear wave elasticity for predicting clinical outcomes in patients with significant tricuspid regurgitation.

This study aimed to evaluate the efficacy of shear wave (SW) elasticity for assessing clinical outcomes in patients with significant tricuspid regurgitation (TR). Significant TR develops right heart failure (HF) with organ congestion, resulting in adverse outcomes, but appropriate therapeutic strategies remain unclear. The assessment of the degree of hepatic congestion using SW elasticity may be effective for determining therapeutic strategies. We prospectively enrolled 77 patients with moderate or severe TR who underwent SW elastography. Patients were divided into three groups according to the value of SW elasticity: low group (SW elasticity < 6.4 kPa, n = 26), medium group (6.4 ≤ SW elasticity < 9.5 kPa, n = 26), and high group (SW elasticity ≥ 9.5 kPa, n = 25). The endpoint was cardiovascular death or hospitalization for HF. During the median follow-up period of 17 months (range 7-39 months), cardiovascular death or hospitalization for HF occurred in seven patients of high group, in three patients of medium group, and in no patients of low group. In high group, three patients died and seven patients were hospitalized for HF. In medium group, two patients died and one patient was hospitalized. Kaplan-Meier analysis showed that the event-free survival rate was worse in high group than in other groups (log-rank test, p = 0.02). High SW elasticity was independently related to cardiac events as well as right ventricular and left ventricular dysfunction. SW elasticity was a predictor of cardiac events in patients with significant TR by assessing hepatic congestion. SW elasticity can be valuable for determining therapeutic strategies for TR.

Assessment of congestion and clinical outcomes in patients with chronic heart failure using shear wave elasticity.

AIMS: The relief of congestion is essential for the prevention of worsening heart failure (HF) resulting in hospitalizations. Assessment of the degree of organ congestion in the chronic phase of HF is important for determining therapeutic strategies. The aim of this study was to evaluate the efficacy of shear wave (SW) elasticity for assessing congestion and clinical outcomes in patients with chronic HF. METHODS AND RESULTS: We prospectively enrolled 345 consecutive patients with chronic HF who underwent SW elastography at outpatient clinic. Patients were divided into two groups according to the median value of SW elasticity: low group (SW elasticity <6.4 kPa, n = 176) and high group (SW elasticity ≥6.4 kPa, n = 169). The endpoint was cardiovascular death or hospitalization for HF. During the median follow-up period of 19 months (range: 7-36 months), cardiovascular death or hospitalization for HF occurred in 4 patients of low group and 27 patients of high group. In high group, 8 patients died, and 19 patients were hospitalized for HF. In low group, 3 patients died, and 1 patient was hospitalized. Kaplan-Meier analysis showed that the event-free survival rate was worse in high group than in low group (log-rank test, P = 0.004). After adjusting for variables, high SW elasticity was independently related to cardiac events. In multivariate regression analysis, SW elasticity was correlated with left atrial volume index, early diastolic mitral inflow velocity to mitral annular velocity ratio, and inferior vena cava diameter. CONCLUSIONS: The SW elasticity reflected haemodynamic congestion in patients with chronic HF, which was related to cardiac events.

Importance of saline contrast transthoracic echocardiography for evaluating large right-to-left shunt in patent foramen ovale associated with cryptogenic stroke.

Transcatheter closure of patent foramen ovale (PFO) is an effective therapy for preventing recurrent stroke in very specific patient cohorts, such as cryptogenic stroke (CS). The identification of high-risk PFO, which is more likely to be linked to CS, is essential. This study aimed to assess the accuracy of saline contrast transthoracic echocardiography (TTE) for evaluating large right-to-left (RL) shunt. We enrolled 119 patients with or without CS who were confirmed to have PFO by transesophageal echocardiography (TEE) or catheterization. The severity of RL shunt evaluated by TTE and TEE was classified as follows: small (< 10 microbubbles), moderate (10-20 microbubbles), and large (> 20 microbubbles). With TTE, large RL shunt was observed in 94 (79%) of 119 patients, including 66 of 74 with CS and 28 of 45 without CS. With TEE, large RL shunt was observed in 33 (28 %) patients, including 26 with CS and 7 without CS. TTE showed large RL shunt more frequently than TEE (p < 0.01). Large RL shunt evaluated by TTE had a sensitivity of 89 % and an accuracy of 70 % for the association with CS, whereas large RL shunt evaluated by TEE had a sensitivity of 35% and an accuracy of 56 %. Accuracy was significantly greater in TTE than in TEE (p = 0.02). In conclusion, TTE identified large RL shunt associated with CS with higher sensitivity and accuracy compared to TEE. Our findings suggest that the decision for device closure should be made based on the severity of RL shunt by TTE.

Fragmented QRS as a predictor of cardiac events in patients with cardiac sarcoidosis.

BACKGROUND: Multiple spikes within the QRS complex, known as fragmented QRS (fQRS), are associated with the occurrences of ventricular arrhythmic events (VAEs) in patients with Brugada syndrome and hypertrophic cardiomyopathy. However, the association between fQRS and occurrence of VAEs in patients with cardiac sarcoidosis (CS) has not been elucidated. METHODS: We evaluated the associations between fQRS and cardiac events including VAEs [non-sustained ventricular tachycardia (NSVT), sustained ventricular tachycardia (VT), and ventricular fibrillation (VF)], hospitalization for heart failure, and all-cause death in 68 patients with CS (30 patients with fQRS vs. 38 patients without fQRS) over a 5-year period. RESULTS: Cardiac events occurred in 22 patients with fQRS and 18 patients without fQRS (73% vs. 47%, p=0.009). Of the cardiac events that occurred in CS patients, VAEs occurred more frequently in patients with fQRS than in patients without fQRS (VAEs: 70% vs. 45%, p=0.017; NSVT: 70% vs. 45%, p=0.010; VT: 43% vs. 18%, p=0.011, and VF: 6.7% vs. 2.6%, p=0.34), whereas there was no significant difference in hospitalization for heart failure or all-cause death between patients with and those without fQRS (hospitalization for heart failure: 6.7% vs. 5.3%, p=0.75; all-cause death: 6.7% vs. 5.3%, p=0.64). Multivariate analysis showed that fQRS in the baseline electrocardiogram was independently associated with VAEs (hazard ratio: 2.21, 95% confidence interval: 1.15-4.25, p=0.017). CONCLUSION: fQRS is a predictor of VAEs in patients with CS.

Importance of direct right-to-left shunt as high-risk patent foramen ovale associated with cryptogenic stroke.

BACKGROUND: Because transcatheter closure of patent foramen ovale (PFO) has become effective for preventing cryptogenic stroke (CS), it is necessary to determine high-risk PFO associated with CS. This study aimed to clarify the importance of direct right-to-left (RL) shunt through the PFO for identifying high-risk PFO. METHODS: We analyzed 137 patients with and without CS who were confirmed to have PFO. The timing of RL shunt through the PFO was evaluated by cardiac cycles after right atrium (RA) opacification on saline contrast transesophageal echocardiography. Direct RL shunt was defined as microbubbles crossing the PFO before and at the same time of RA opacification. RESULTS: Cardiac cycles of microbubbles crossing the PFO were shorter in patients with CS than in those without CS (2.0 ± 2.2 vs .5 ± 1.1, p < 0.01). Direct RL shunt was more frequently observed in patients with CS than in those without CS (77% vs 29%, p < 0.01), with a sensitivity of 79% and a specificity of 71% for the association with CS. Multivariate analysis revealed that direct RL shunt was related to atrial septal aneurysm and low-angle PFO. Regarding functional features of PFO, the detection rate of CS was 50% for large RL shunt alone, and was increased to 83% when direct RL shunt was added. CONCLUSION: Direct RL shunt was associated with CS and had the incremental value in detecting PFO associated with CS for large RL shunt. The timing of RL shunt can be valuable for identifying high-risk PFO.