Real-World Patent Foramen Ovale (PFO) Closure in Japan　- 30-Day Clinical Outcomes From the AmplatzerTM PFO Occluder Japan Post-Marketing Surveillance Study.

BACKGROUND: The AmplatzerTM PFO Occluder was approved for marketing in Japan in May 2019, and the Amplatzer PFO Occluder Japan Post-marketing Surveillance (PFO Japan PMS) study was initiated in December 2019. This analysis presents 30-day clinical outcomes for PFO Japan PMS study patients. METHODS AND RESULTS: PFO Japan PMS is a prospective single-arm non-randomized multicenter clinical study. Eligible patients were indicated for patent foramen ovale (PFO) closure and underwent an implant attempt with the AmplatzerTM PFO Occluder. Technical success was defined as successful delivery and release of the occluder; procedural success was defined as technical success with no serious adverse events (SAEs) within 1 day of the procedure. The primary safety endpoint includes predefined device- and/or procedure-related SAEs through 30 days after the procedure. From December 2019 to July 2021, 500 patients were enrolled across 53 Japanese sites. The mean (±SD) patient age was 52.7±15.4 years, and 29.8% of patients were aged >60 years. Technical and procedural success rates were both high (99.8% and 98.8%, respectively). Further, there was only one primary safety endpoint event (0.2%): an episode of asymptomatic paroxysmal atrial fibrillation that occurred 26 days after the procedure. CONCLUSIONS: In this real-world Japanese study with almost one-third of patients aged >60 years, PFO closure with the AmplatzerTM PFO Occluder was performed successfully and safely, with a low incidence of procedure-related atrial arrhythmias.

Practice Patterns in the Management of Pressure Restrictive Perimembranous Ventricular Septal Defects: A Multinational Survey.

Indications for the closure of pressure restrictive perimembranous ventricular septal defects (pmVSD) are not well established in the pediatric population. We sought to assess practice variability among pediatric cardiologists in the United States (US), Canada, Australia, and New Zealand. A survey ascertaining practice patterns, including case vignettes with incremental progression of disease severity, was designed and administered through representative professional cardiac organizations and email listservs in the designated countries. Among the 299 respondents, 209 (70.0%) were from the US, 65 (21.7%) were from Canada and 25 (8.3%) were from Australia and New Zealand. Indications for pressure restrictive pmVSD closure included the presence of left ventricular (LV) dilation for 81.6% (244/299) (defined as z-score ≥ 2 for 59.0% (144/244) and ≥ 3 for 40.2% (98/244)) and significant pulmonary-systemic flow ratio (QP:QS) for 71.2% (213/299) [defined as ≥ 1.5:1 for 36.2% (77/213) and ≥ 2 for 62% (132/213)]. US pediatric cardiologists elected to close restrictive pmVSD at lower LV z-score and QP:QS ratio cut-offs (p-value 0.0002 and 0.013, respectively). In a case vignette, 63.6% (173/272) chose to intervene if there was right coronary cusp prolapse with stable mild aortic regurgitation. Of the remaining cardiologists, 93% (92/99) intervened if the aortic regurgitation was progressive (from trivial to mild). Commonly identified indications with variable thresholds for closure of pressure restrictive pmVSDs included the presence or progression of LV dilation, significant volume loading, and aortic valve prolapse with regurgitation. US pediatric cardiologists may have a lower threshold for pmVSD closure.

Mechanical Complications of Acute Myocardial Infarction: A Scientific Statement From the American Heart Association.

Over the past few decades, advances in pharmacological, catheter-based, and surgical reperfusion have improved outcomes for patients with acute myocardial infarctions. However, patients with large infarcts or those who do not receive timely revascularization remain at risk for mechanical complications of acute myocardial infarction. The most commonly encountered mechanical complications are acute mitral regurgitation secondary to papillary muscle rupture, ventricular septal defect, pseudoaneurysm, and free wall rupture; each complication is associated with a significant risk of morbidity, mortality, and hospital resource utilization. The care for patients with mechanical complications is complex and requires a multidisciplinary collaboration for prompt recognition, diagnosis, hemodynamic stabilization, and decision support to assist patients and families in the selection of definitive therapies or palliation. However, because of the relatively small number of high-quality studies that exist to guide clinical practice, there is significant variability in care that mainly depends on local expertise and available resources.

Concomitant surgery for double-chambered right ventricle and coronary stenosis.

BACKGROUND AND AIMS: The clinical data of coronary artery disease in patients with double-chambered right ventricle are limited. We report an adult double-chambered right ventricle case with three-vessel coronary artery disease that was successfully treated with concomitant double-chambered right ventricle repair and coronary artery bypass grafting. MATERIALS AND METHODS: The patient case data was extracted from hospital records. RESULTS: The patient was the case of a 60-year-old man with a double-chambered right ventricle and three-vessel coronary artery disease. He underwent concomitant surgery comprising double-chambered right ventricle repair and coronary artery bypass grafting. Achieving cardiac arrest allowed us to obtain a good surgical view of the heavy and severely hypertrophied heart. Postoperative computed tomography revealed a feasible running course of the sequential graft, indicating that the path of the sequential graft should be clockwise (aorto-right coronary-left circumflex artery) in this unusual anatomical condition. DISCUSSION AND CONCLUSION: We report this rare disease combination and highlight the need for careful preoperative planning in such cases.

[Drop in oxygen saturation and blood pressure as well as increase in central venous pressure during mitral valve clipping in an 81-year-old female patient]. / Abfall von Sauerstoffsättigung und Blutdruck sowie Anstieg des zentralen Venendrucks im Rahmen eines Mitralklappenclippings bei einer 81-Jährigen.

BACKGROUND: Atrial septal defects (ASD) following endovascular mitral valve clipping are potentially hemodynamically relevant complications. Immediate closure with an occluder can represent a safe and effective treatment. An 81-year-old female patient suffering from severe dyspnea due to previously known severe mitral valve regurgitation was scheduled for elective mitral valve clipping. The clip was successfully implanted. Removal of the transseptal cannula resulted in a sudden drop in oxygen saturation and systolic blood pressure as well as an immediate increase in central venous pressure. An iatrogenic left-right shunt was observed at the atrial level with a relevant shunt volume. Immediate closure using an atrial septal occluder successfully restored the oxygen saturation and hemodynamic parameters. CONCLUSION: An increase in central venous pressure, reduction of systolic blood pressure or oxygen saturation after withdrawal of the transseptal cannula during mitral valve clipping should always be further investigated regarding a possible ASD.

Mirror-image dextrocardia: why is the apex impulse not on the right?

This article presents the case of an 11-year-old girl with a history of Fortan surgery who presented to the authors' department with shortness of breath, orthopnea, and cyanosis. Electrocardiography (ECG) was indicative of mirror-image dextrocardia despite location of the apex impulse on the left. Echocardiography suggested mirror-image dextrocardia accompanied by levoversion, a large atrial septal defect and left ventricular atresia (functional single atrium and single ventricle), and right ventricular hypertrophy. ECG with corrected leads placement showed a sinus rhythm, biatrail enlargement, and right ventricular hypertrophy. Based on echocardiography and medical history, the case was rediagnosed as mirror-image dextrocardia with levoversion.

The Application and Teaching Value of a Ventricular Septal Defect Canine Model Established by Transcatheter Puncture.

This study aimed to improve and further explore a ventricular septal defect (VSD) canine model on the basis of the transcatheter puncture method and to evaluate its application and teaching value.In order to lessen the complications of VSD closure, it is necessary to improve the currently available treatment devices using appropriate animal models.In this study, we used 16 healthy adult canines as our models. After anesthesia, the VSD puncture was performed, followed by balloon dilatation of the perforation. VSD was confirmed by angiography. The venous-artery orbit was established, and the VSD was then closed once the catheter and occluder were across the defect.Of the experimental canines, 14 of the 16 canines were successfully modeled, giving a success rate of 87.5%. The canines underwent an immediate creation of a venous-artery orbit for teaching practice and were implanted with an occluder during the procedure. After 4 weeks, 13 canines survived. As per our findings, most VSD types established by the puncture were perimembranous (10 of 13, 77%).The current model has a high success rate. The model can not only avoid the risk of infection and hemodynamic disorders associated with an open thoracotomy, but can also be effectively used in evaluating the impact of occluders. It can also directly measure the parameters of the devices during the procedure, thus having a very high experimental and teaching value.

Utility of transesophageal echocardiography for intra-operatively assessing pulmonary artery pressure across an isolated ventricular septal defect in children.

BACKGROUND: The magnitude of pulmonary hypertension (PH) is extremely important with respect to the intra-operative management of children and infants with an isolated ventricular septal defect (VSD). This study aimed to assess the feasibility and accuracy of transesophageal echocardiography for estimating pulmonary arterial systolic pressure (PASP) across isolated VSD. METHODS: We compared the results of transesophageal echocardiography vs invasive PASP measured simultaneously. This study included 40 patients (age: 6 months to 6 years; weight: >5 kg) who were undergoing elective surgery for isolated VSDs. Flow signals across the VSDs were identified as high velocity turbulent signals in systole via continuous wave Doppler at 0-120° at the mid-esophageal level. Peak velocities were recorded. Radial artery systolic pressures were assessed invasively, and PASPs were obtained after exposing the pulmonary artery intra-operatively. RESULTS: After excluding five patients because of unusable measurements, invasive PASP measurements were obtained in 35 patients (87.5%). There were no significant biases between echocardiographic and catheterization measurements of PASP, with a tight confidence interval measuring, on average, up to 2.6 mmHg. However, the ± 2 standard deviation limits of agreement for mean PASP were -3.8 and 10.6 mmHg. CONCLUSION: PASP measurements via transesophageal echocardiography in cardiac surgical patients under general anesthesia are recommended for use as a screening and monitoring tool for PH in children and infants, but cannot be used as a diagnostic tool.

Left ventricular rotation and torsion in neonates and infants younger than three months with symptomatic ventricular septal defect: Acute effects from open heart surgery.

PURPOSE: We evaluated ventricular rotation, torsion, and strain changes in infants using preoperative and postoperative M-mode echocardiography after early surgery for symptomatic ventricular septal defects (VSD). METHODS: Thirty-five patients with VSD underwent vector velocity imaging echocardiography before and after open heart surgery. Their rotational variables were compared with 18 controls. RESULTS: All the patients (19 boys and 16 girls; median age: 44.4 days; range: 13-84 days) showed normal septal motion preoperatively; however, septal motion changed into flat septum or paradoxical septal motion after surgery. Left ventricular end-diastolic internal dimension and fractional shortening significantly decreased after surgery (P = .001 and P = .000). Patients showed significant postoperative reduction of peak systolic apical rotation and maximal torsion (P = .010 and P = .000). Peak systolic basal rotation decreased after surgery but it was not significantly (P = .106). No significant differences were found in longitudinal and circumferential systolic strains between patients and controls. CONCLUSION: Abnormal motion of the ventricular septum was confirmed by postoperative M-mode echocardiography. Decreased rotation/torsion variables may reflect postoperative changes of ventricular loading conditions. Because systolic strain was preserved, postoperative echocardiographic results should not be interpreted as abnormal or decreased ventricular function.

First reported adult patient with TARP syndrome: A case report.

TARP syndrome (talipes equinovarus, atrial septal defect, Robin sequence, and persistence of the left superior vena cava) is a rare X-linked syndrome often resulting in pre- or post-natal lethality in affected males. In 2010, RBM10 was identified as the disease-causing gene, and we describe the first adult patient with TARP syndrome at age 28 years, hereby expanding the phenotypic spectrum. Our patient had Robin sequence, atrial septal defect, intellectual disability, scoliosis, and other findings previously associated with TARP syndrome. In addition, he had a prominent nose and nasal bridge, esotropia, displacement of lacrimal points in the cranial direction, small teeth, and chin dimple, which are the findings that have not previously been associated with TARP syndrome. Our patient was found to carry a hemizygous c.273_283delinsA RBM10 mutation in exon 4, an exon skipped in three of five protein-coding transcripts, suggesting a possible explanation for our patient surviving to adulthood. Direct sequencing of maternal DNA indicated possible mosaicism, which was confirmed by massive parallel sequencing. One of two sisters were heterozygous for the mutation. Therefore, we recommend sisters of patients with TARP syndrome be carrier tested before family planning regardless of carrier testing results of the mother. Based on our patient and previously reported patients, we suggest TARP syndrome be considered as a possible diagnosis in males with severe or profound intellectual disability combined with septal heart defect, and Robin sequence, micrognathia, or cleft palate.

How many versus how much: comprehensive haemodynamic evaluation of partial anomalous pulmonary venous connection by cardiac MRI.

OBJECTIVES: The objective of this study was to investigate the effect of location and number of anomalously connected pulmonary veins and any associated atrial septal defect (ASD) on the magnitude of left-to-right shunting in patients with partial anomalous pulmonary venous connection (PAPVC), and how that influences right ventricular volume loading. METHODS AND RESULTS: The cardiac magnetic resonance (CMR) and echocardiography examinations of 26 paediatric patients (mean age, 11.2 ± 5.1 years) with unrepaired PAPVC were analysed. Fourteen patients had right-sided, 11 left-sided and 1 patient bilateral PAPVC. An ASD was present in 11 patients, of which none had a Qp/Qs < 1.5 and 8 had a Qp/Qs≥ 2.0. No patient with isolated left upper PAPVC experienced a Qp/Qs ≥ 2.0 compared to 9/12 patients with right upper PAPVC. Qp/Qs correlated with indexed right ventricle (RV) end-diastolic volume (RVEDVi, r = 0.59, p = 0.002) by CMR and with echocardiographic right ventricular end-diastolic dimension (RVED) z-score (r = 0.68, p = 0.003). A RVEDVi >124 ml/m2 by CMR and a RVED z-score >2.2 by echocardiography identified patients with a Qp/Qs ≥1.5 with good sensitivity and specificity. CONCLUSIONS: An asymptomatic patient with a single anomalously connected left upper pulmonary vein and without an ASD is unlikely to have a significant left-to-right shunt. On the other hand, right-sided PAPVC is frequently associated with a significant left-to-right shunt, especially when an ASD is present. KEY POINTS: • Patients with PAPVC and ASD routinely have a significant left-to-right shunt. • Patients with right PAPVC are likely to have a significant left-to-right shunt. • Patients with left PAPVC are unlikely to have a significant left-to-right shunt. • CMR is helpful in decision-making for patients with PAPVC.

[Feasibility and effectiveness of percutaneous ventricular septal defect closure under solely guidance of echocardiography].

Objective: To compare the efficacy and safety of percutaneous ventricular septal defect (VSD) closure under solely guidance of echocardiography and fluoroscopy. Methods: The retrospective study was conducted at Fuwai Hospital, between February 2014 and February 2015. The patients were divided into two groups. VSD closure was conducted in 42 patients under solely guidance of echocardiography, and 100 patients who were treated with percutaneous catheter closure under fluoroscopy guidance were selected as a control group. The baseline characteristics, procedural time and complications were recorded and assessed. Results: There were no significant differences in terms of age, gender and pre-operative echocardiographic characteristics (all P>0.05). Percutaneous VSD closure under traditional fluoroscopy guidance was successful in 95 patients (95%). The procedural time was (54.7±12.5) minutes. The symmetrical occluders diameter was (6.9±1.8) mm. Four patients had postoperative residual shunt, one patient developed left bundle branch block, and 6 patients developed new tricuspid regurgitation. Percutaneous VSD closure under only transthoracic echocardiography (TTE) guidance was successful in 39 patients (93%). Because of delivery catheter passage failure through the defect, one case required conversion to perventricular closure via a small transthoracic incision under transesophageal echocardiography (TEE) guidance. The other two cases underwent surgical repair because of residual shunt with more than 2 mm after closure. The procedural time was (40.3±13.2) minutes. The symmetrical occluders diameter was (6.5±1.2) mm. Four patients had postoperative residual shunt that disappeared after 1 month follow-up, and one patient developed right bundle branch block which disappeared 3 days later. During (9.3±3.6) months follow-up, there were no other complications, such as pericardial effusion, occluder malposition, atrioventricular block, aortic valve regurgitation, and aggravating tricuspid regurgitation in each group. However, the total operation time, diameter of VSD occluder, total mild complications, and the equipment cost in the study group were less than that in the control group (P<0.05). Conclusion: Percutaneous VSD closure can be successfully performed under sole guidance of echocardiography with outcomes similar to those achieved with fluoroscopy guidance.

[Feasibility of device closure for multiple atrial septal defects using 3D printing and ultrasound-guided intervention technique].

Objective: To investigate the feasibility of trans-catheter closure of multiple atrial septal defects (ASD) monitored by trans-thoracic echocardiography (TTE) under the guidance of 3D printing heart model. Methods: Between April and August 2016, a total of 21 patients (8 male and 13 female) with multiple ASD in Fuwai Hospital of Chinese Academy of Medical Sciences underwent CT scan and 3-dimensional echocardiography for heart disease model produced by 3D printing technique. The best occlusion program was determined through the simulation test on the model. Percutaneous device closure of multiple ASD was performed follow the predetermined program guided by TTE. Clinical follow-up including electrocardiogram and TTE was arranged at 1 month after the procedure. Results: The trans-catheter procedure was successful in all 21 patients using a single atrial septal occluder. Mild residual shunt was found in 5 patient in the immediate postoperative period, 3 of them were disappeared during postoperative follow-up. There was no death, vascular damage, arrhythmia, device migration, thromboembolism, valvular dysfunction during the follow-up period. Conclusion: The use of 3D printing heart model provides a useful reference for transcatheter device closure of multiple ASD achieving through ultrasound-guided intervention technique, which appears to be safe and feasible with good outcomes of short-term follow-up.

[Surgical treatment and early-mid follow-up results of complete atrioventricular septal].

Objective: To discuss the surgical treatment and early-mid follow-up results of complete atrioventricular septal defect (CAVSD). Methods: The clinic data of 235 cases of CAVSD underwent surgical treatment at Department of Cardiac Surgery, Guangdong General Hospital from June 2009 to June 2016 was analyzed retrospectively (104 male and 131 female patients). The patients were aged 18 days to 58 years (mid age 8.9 months) at surgery, and 2 cases of newborns (0.9%), 24 cases of 1 to 3 months (10.2%), 56 cases of 4 to 6 months (23.8%), 66 cases of 7 to 12 months (28.1%), 36 cases of 1 to 3 years (15.3%), 37 cases of 4 to 18 years (15.7%), and 14 cases above 18 years (6.0%). There were 129 cases of Rastelli type A (54.89%), 16 cases of type B (6.8%), 25 cases of type C (10.6%) and 65 cases of transitional type (27.7%). The patients were combined with 7 cases of unroofed coronary sinus syndrome, 5 cases of coarctation of aorta, 4 cases of tetralogy of Fallot, 3 cases of double outlet right ventricle, 1 case of transposition of the great arteries, 1 case of multiple ventricular septal defect, 1 case of pulmonary artery sling. The CAVSD correction operations were performed in 199 cases with modified single patch technique (84.7%), 26 cases with two patch technique (11.1%), 10 cases with conventional single patch technique (4.3%), and the corrective surgeries of other anomalies were performed in the meantime. Generalized Estimated Equation was used to statistical analysis of postoperative regurgitation level of mitral valve and tricuspid valve. Results: Followed-up for 1 to 7 years, 18 cases (7.7%) died overall and 15 cases (6.4%) died within 30 days post-operation.The mortality of little infant (operation age ≤3 months) was much highest(26.9%). The mortality of different operation age had significant difference(P=0.007). There was no difference among the mortality with three techniques. Seven cases (3.0%) suffered from the re-operation for severe mitral regurgitation. A total of 217 patients survived more than 1 year after initial surgery, and there were 11 cases (5.1%) with severe mitral regurgitation and 7 cases (3.2%) with severe tricuspid valve regurgitation at 1 year post-operation.The degree of mitral regurgitation after operation decreased at 1 week to 6 months, and slightly higher after 1 year postoperatively.The ratios of severe mitral regurgitation in different operative age group was statistically significant difference(P=0.017), mainly from 3 months group and 1 to 3 years group.The degree of tricuspid regurgitation decreased significantly after operation, and decreased gradually in the follow-up period. The ratio of postoperative residual severe tricuspid regurgitation was the highest in surgical aged >18 years old, less in 3 years group, the lowest in 3 to 18 years group. Conclusions: The mortality and the ratio of patients with serious mitral regurgitation were higher in infants less 3 months than elder patients. The infants need more frequently follow-up and medicine treatment after operation. The modified one patch technique is effective for all age groups.

Intramural Ventricular Septal Defect Is a Distinct Clinical Entity Associated With Postoperative Morbidity in Children After Repair of Conotruncal Anomalies.

BACKGROUND: Intramural ventricular septal defects (VSDs) are interventricular communications through right ventricular free wall trabeculations that can occur after repair of conotruncal anomalies. We assessed the prevalence of residual intramural VSDs and their effect on postoperative course. METHODS AND RESULTS: Children who underwent biventricular repair of a conotruncal anomaly from January 1, 2006, to June 30, 2013, and had a postoperative transthoracic echocardiogram were included. Images were reviewed for residual intramural or nonintramural VSDs. The primary outcome was a composite of mortality, extracorporeal membrane oxygenation use, and need for subsequent catheter or surgical VSD closure. The secondary outcome was postoperative hospital length of stay. A residual VSD was present in 256 of the 442 subjects (58%), of which 231 (90%) were <2 mm in size. Forty-nine patients (11%) had intramural VSDs, and 207 (47%) had nonintramural VSDs. Patients with intramural VSDs were more likely to reach the primary composite outcome compared with those with nonintramural VSDs or no residual VSD (14 of 49 [29%] versus 15 of 207 [7%] versus 6 of 186 [3%]; P<0.0001). In addition, those with intramural VSDs had longer postoperative hospital length of stay compared with those with nonintramural VSDs or no residual VSD (20 days [interquartile range, 11-42 days] versus 7 days [interquartile range, 5-14 days] versus 6 days [interquartile range, 4-11 days]; P=0.0001). These associations remained significant after adjustment for known risk factors for poor outcomes, including residual VSD size and operative complexity. CONCLUSIONS: Among residual VSDs after repair of conotruncal anomalies, intramural VSDs are uniquely associated with postoperative morbidity, mortality, and longer postoperative hospital length of stay. It is important to recognize intramural VSDs in the postoperative period.

HIC2 is a novel dosage-dependent regulator of cardiac development located within the distal 22q11 deletion syndrome region.

RATIONALE: 22q11 deletion syndrome arises from recombination between low-copy repeats on chromosome 22. Typical deletions result in hemizygosity for TBX1 associated with congenital cardiovascular disease. Deletions distal to the typically deleted region result in a similar cardiac phenotype but lack in extracardiac features of the syndrome, suggesting that a second haploinsufficient gene maps to this interval. OBJECTIVE: The transcription factor HIC2 is lost in most distal deletions, as well as in a minority of typical deletions. We used mouse models to test the hypothesis that HIC2 hemizygosity causes congenital heart disease. METHODS AND RESULTS: We created a genetrap mouse allele of Hic2. The genetrap reporter was expressed in the heart throughout the key stages of cardiac morphogenesis. Homozygosity for the genetrap allele was embryonic lethal before embryonic day E10.5, whereas the heterozygous condition exhibited a partially penetrant late lethality. One third of heterozygous embryos had a cardiac phenotype. MRI demonstrated a ventricular septal defect with over-riding aorta. Conditional targeting indicated a requirement for Hic2 within the Nkx2.5+ and Mesp1+ cardiovascular progenitor lineages. Microarray analysis revealed increased expression of Bmp10. CONCLUSIONS: Our results demonstrate a novel role for Hic2 in cardiac development. Hic2 is the first gene within the distal 22q11 interval to have a demonstrated haploinsufficient cardiac phenotype in mice. Together our data suggest that HIC2 haploinsufficiency likely contributes to the cardiac defects seen in distal 22q11 deletion syndrome.