Right ventricular dysfunction is a major determinant of long-term outcomes after tetralogy of Fallot repair, particularly in the presence of chronic pulmonary regurgitation. Strain analysis may detect early right ventricular dysfunction more sensitively than conventional echocardiographic parameters.

This retrospective single-centre study included 63 patients after tetralogy of Fallot repair who underwent postoperative echocardiographic evaluation during mid-term follow-up. Right ventricular function was assessed using tricuspid annular plane systolic excursion, right ventricular systolic velocity, fractional area change, and right ventricular free-wall longitudinal strain. Pulmonary regurgitation severity was evaluated by colour Doppler, with pulmonary regurgitation jet width expressed as a ratio relative to the pulmonary annulus diameter. Associations between pulmonary regurgitation severity and right ventricular function were analysed using correlation and multivariable linear regression.

Conventional right ventricular systolic parameters were largely preserved, whereas right ventricular free-wall longitudinal strain worsened with increasing pulmonary regurgitation severity (p = 0.036). Pulmonary regurgitation jet width emerged as the sole independently associated determinant of impaired right ventricular strain (β = 0.132, p = 0.038). QRS duration was not independently associated. Pulmonary regurgitation jet width showed moderate discriminatory ability for impaired right ventricular strain (area under the curve = 0.67).

Right ventricular free-wall longitudinal strain is a sensitive marker of pulmonary regurgitation-related right ventricular dysfunction after tetralogy of Fallot repair. Pulmonary regurgitation jet width, expressed relative to the pulmonary annulus, appears to be the dominant determinant of right ventricular mechanical impairment and may complement conventional assessment during follow-up.

This study aimed to identify echocardiographic predictors of successful weaning from extracorporeal membrane oxygenation in paediatric and congenital heart disease patients.

We retrospectively analyzed pediatric patients who underwent venoarterial extracorporeal membrane oxygenation for cardiogenic shock or postoperative support between March 2018 and September 2023. Clinical and echocardiographic variables assessed at the time of weaning evaluation were compared between patients who were successfully weaned and those who were not.

Among the 46 enrolled patients, 31 were successfully weaned from extracorporeal membrane oxygenation. The mean age at extracorporeal membrane oxygenation initiation was 9.6 ± 13.9 years, and the mean duration of support was 12.3 ± 12.1 days. Patients in the successfully weaned group had significantly higher left ventricular ejection fraction (50.9 ± 16.4% vs. 27.3 ± 18.7%, p < 0.001) and higher velocity time integral at the left ventricular outflow tract (12.3 ± 8.0 cm vs. 4.1 ± 3.6 cm, p = 0.001) compared with the unsuccessfully weaned group. The cutoff values for predicting successful weaning were a left ventricular ejection fraction of 43.03% (sensitivity, 74.2%; specificity, 86.7%) and a velocity time integral of 4.45 cm (sensitivity, 92.0%; specificity, 66.7%).

Left ventricular ejection fraction and velocity time integral provide valuable echocardiographic information for predicting successful weaning from extracorporeal membrane oxygenation in pediatric patients and may support clinical decision-making during weaning assessments.

Right atrial appendage aneurysm, or giant right atrial appendage, is extremely rare, with very few cases reported in scientific literature. We sought to systematically review the published cases of right atrial appendage aneurysm in terms of age, sex, clinical presentation, electrocardiography, imaging (chest X-ray, echocardiography, CT/cardiac magnetic resonance), and outcome.

An electronic search for case reports, case series, and related articles published until March 2025 was carried out, and clinical data were extracted and analysed.

Forty-four cases of right atrial appendage aneurysm were identified with a clear male prevalence (68.2%) and commonly presenting in the third decade of life. Palpitation (27.3%) and dyspnoea (18.2%) were the most common clinical presentations, whereas 40.9% of right atrial appendage aneurysm patients were asymptomatic. Electrocardiography was done in 77.3% of the sample. It displayed an atrial arrhythmia (atrial fibrillation or flutter, atrial tachycardia, supraventricular tachycardia) in 31.8%. A chest X-ray was done in 65.9%. Echocardiography was the most common diagnostic modality (93.2%). Right atrial appendage aneurysm diagnosis was confirmed on CT and/or MRI in 79.5%. The mean size of the right atrial appendage aneurysm was 93 × 70 mm. In 12 patients (27.3%), an associated congenital cardiac abnormality was found, mostly in the form of an atrial septal defect/patent foramen ovale (22.7%). Half of the patients (50.0%) were treated surgically, whilst 47.8% were treated medically with close follow-up. One patient experienced right atrial appendage aneurysm reduction in size after atrial septal defect device closure. One death (2.3%) was reported also.

Although very uncommon, right atrial appendage aneurysm can be linked to considerable morbidity. Surgical removal is recommended for patients who are symptomatic.

To evaluate the right ventricle function by conventional echocardiographic methods and strain analysis in the long term after balloon pulmonary valvuloplasty. In addition, we investigated the relationship between pulmonary regurgitation, demographic data at the time of the procedure, and right ventricle dysfunction in late follow-up.

The records of patients submitted to balloon pulmonary valvuloplasty from 2001 to 2015 in a single centre were reviewed. From that sample, a revised cohort was formed, and the patients were submitted to clinical and echocardiographic evaluations.

The retrospective and the revised cohort analyses included 73 and 18 patients, respectively. In the follow-up, pulmonary regurgitation was observed in all patients, and there was a significant worsening of its magnitude over time (p < 0.001); the severity of pulmonary regurgitation was associated with balloon pulmonary valvuloplasty performed in patients with weight < 3 kg (p < 0.03), body surface area < 0.3 m2 (p < 0.04), and < 1 year of age (p < 0.006). Global longitudinal systolic strain of the right ventricle was abnormal in 8 of 18 patients, and conventional methods were abnormal in 2 of 18 patients (p = 0.001). There was a significant relationship between severe pulmonary regurgitation and right ventricle dysfunction detected only by strain evaluation (p = 0.01).

The severity of pulmonary regurgitation was related to the impairment of right ventricle function detected by strain. The predictors of pulmonary regurgitation severity in late follow-up were age < 1 year, weight < 3 kg, and body surface area < 0.3 m2.

Echocardiography is the preferred method for the visual assessment of bubble load in divers. This study evaluates the feasibility of a microteaching program for training combat medics to perform ultrasound measurements using echocardiography for self-monitoring decompression stress on the waterside.

A microteaching was provided to combat medics of the Netherlands Armed Forces. Participants used a handheld ultrasound device connected to a tablet. After two minutes practice time, medics performed and recorded videos on randomly assigned partners while being assessed by an anesthesiologist. Three outcomes were measured: (1) observer-assessed performance adapted from Objective Structured Assessment of Technical Skills (OSATS); (2) self-perceived procedure experience; and (3) video recording quality on a five-point scale.

All 21 combat medics completed the microteaching program. Three out of 21 video recordings were lost due to technical issues. All participants successfully obtained at least a partial cardiac view (median time: 61 seconds). Performance scores indicated near-competence across preparation, time-motion, and procedural flow. Image quality ratings by two reviewers showed near-perfect intra-rater agreement (κ = 0.904 and κ = 0.960) but substantial inter-rater variability (κ = 0.671); the assessor’s median scores were 2.75 and 3.0 out of 5.0, respectively. Most recordings received average scores of 3.0 or higher.

This study demonstrates that combat medics, following a brief microteaching session, were able to acquire cardiac ultrasound images partially suitable for assessing vascular gas emboli (VGE). These findings support microteaching as a feasible first step in echocardiography training for combat medics in austere environments.

Single ventricle pulmonary arteriovenous malformations are poorly understood and variably assessed in published literature. To improve our understanding of single ventricle pulmonary arteriovenous malformations and facilitate multi-centre studies, it will be necessary to have uniform clinical practice patterns among paediatric heart institutions.

The aim of this study was to assess paediatric interventional cardiologists’ clinical perspectives and practice patterns for diagnosing single ventricle pulmonary arteriovenous malformations.

We surveyed paediatric interventional cardiologists using the Congenital Cardiovascular Interventional Consortium listserv. A single survey was distributed electronically with two subsequent reminder emails. Voluntary participants completed the anonymous survey electronically via RedCap.

Among 253 Congenital Cardiovascular Interventional Consortium members, a total of 55 (21.7%) paediatric cardiology interventional attending physicians completed the survey. There was near unanimity (98%) that pulmonary arteriovenous malformations develop due to lack of hepatic vein blood flow to the lungs; however, there was wide variation among practice patterns. A minority (20%) of respondents perform bubble contrast echocardiograms (bubble studies) more than half the time pre-Fontan, whereas many (31%) almost never (< 5% of cases) perform bubble studies pre-Fontan. Most respondents reported that they did not perform bubble studies because results do not impact clinical decision making pre-Fontan (56%) or post-Fontan (60%). Many respondents (49%) do not have a typical volume of agitated saline that they inject for bubble studies.

Clinical practice patterns vary widely among paediatric cardiology interventionalists. A standardised clinical approach, new diagnostic tools, or both are needed to standardise our field’s approach to diagnosing, studying, and treating single ventricle pulmonary arteriovenous malformations.

With more than 1 million children in the United States living with a heart defect or condition, it is important to identify interventions that may minimise the long-term impacts of repeated medical surveillance and care. Thus, the purpose of this quasi-experimental study was to examine relationships between facility dog intervention and young children’s anxiety during outpatient echocardiogram.

Participants were seventy children aged 18 months to 8 years undergoing echocardiogram in a paediatric cardiology clinic. Child anxiety was scored by a trained nurse observer pre- and post-procedure using the modified Yale Preoperative Anxiety Scale. Facility dog intervention included individualised play, positioning, therapeutic conversation and touch, and emotional support throughout to promote coping and compliance. Parents and staff completed a post-procedural perceptions survey about their experiences.

Paired samples t-tests demonstrated child anxiety levels were significantly lower post-procedure compared to pre-procedure (Z = −3.974, p < .001). This direction held for nearly all participants; however, those with prior echocardiogram history demonstrated significantly higher anxiety levels at the pre-procedural timepoint (z = −2.442, p = .015). Caregivers (97.2%) and staff (87.9%) agreed or strongly agreed that facility dog intervention was helpful in this context.

Facility dog intervention was associated with a significant reduction in young children’s anxiety across procedural timepoints in outpatient echocardiography. The intervention was perceived as helpful by families and staff; no workflow changes or barriers were noted. Thus, facility dog intervention may be a well-received and promising care innovation for this vulnerable chronic population.

Congenital aortic valvar disease represents a heterogeneous population with suboptimal surgical repair or replacement outcomes. We assess our approach and short-term outcomes in this population using cardiac CT evaluation for personalised surgical planning and execution.

We assessed patients who underwent aortic valvar surgery from February 2022 to August 2024. Pre-surgical evaluation included cardiac CT with quantitative assessment of the leaflet geometry and measures of leaflet coaptation. A standardised approach towards surgical execution guided by this assessment was established and followed.

Seventy-three patients underwent surgery at a median age of 26.0 years (interquartile range 19–44), 65.8% males. Forty-eight patients (65.8%) underwent some form of aortic valvar repair, with 22 of these 48 patients undergoing a valve-sparing aortic root replacement. The remaining 25 patients (34.2%) underwent some form of aortic valvar replacement. Mean post-surgical follow-up was 4.2 ± 6.1 months. Moderate or greater aortic regurgitation was present in 45 patients (61.6%) pre-operatively versus 2 patients (2.7%) post-operatively (p-value < 0.001). The peak and mean gradient improved from 33.2 ± 31.3 mmHg and 16.9 ± 10.7 mmHg pre-operatively, to 16.9 mmHg±10.7 mmHg and 9.5 ± 6.4 mmHg post-operatively (p-value < 0.001).

The heterogeneity and complexity of the dysfunctional and/or dilated (neo-)aortic root encountered in those presenting for surgery necessitates a methodical, detailed three- and four-dimensional assessment. By applying such an approach, we have aimed to standardise not only the assessment, but also description and surgical execution in this challenging patient population. Excellent short-term results have been achieved, necessitating long-term follow-up to understand the potential benefits towards this personalised approach.

Membranous subaortic stenosis is a CHD with high recurrence-rate despite surgical treatment. This study investigated the outcome of operated patients and possible predictors for recurrence.

Retrospective review of all patients (n = 38) ≤ 18 years of age operated for membranous subaortic stenosis between 1994–2019 at Sahlgrenska University Hospital. The primary outcomes were recurrence, reintervention, and mortality. Predictors of recurrence and reintervention were secondary outcomes.

Median age (range) at diagnosis, initial intervention, and last follow-up were 2.3 (0.003–17.2), 5.3 (0.03–17.5) and 17.5 (3.6–20.4) years, respectively. Median follow-up time was 9.9 (0.01–19.5) years. 61% were males, and 53% had other associated CHD. 19 patients (56%) developed recurrence and 7 (21%) underwent reintervention. One patient died peri-operatively. Age <5 years at first intervention increased the likelihood of reintervention. Postoperative peak/mean gradients were higher in patients with disease recurrence.

The median echocardiographic peak-/mean gradients at initial diagnosis, pre-, postoperative, and at last follow-up were 61/36, 83/50, 16/8, and 19/17 mmHg respectively (p < 0.0001 pre/post). Pre-/postoperative peak gradients were linearly correlated, decreasing by 80% pre-/postoperatively (p < 0.01). Presence of symptoms and the preoperative peak gradient were positively associated (p < 0.001) with a peak gradient threshold value of > 90 mmHg. The distance between the subaortic stenosis membrane and the aortic valve was inversely correlated to the preoperative peak-gradient (p < 0.01).

Reintervention following surgical intervention of membranous subaortic stenosis is common. A positive correlation exists between high pre- and postoperative peak-gradient. A low postoperative peak gradient may be important in avoiding recurrence.

Left ventricular function after arterial switch operation for d-transposition of the great arteries is notoriously compromised because of abnormal coronary artery anatomy or altered loading conditions. We sought to longitudinally investigate the performance of the left ventricle in a cohort of d-transposition of the great artery patients after arterial switch operation, by using advanced echocardiographic deformation imaging and grouping patients according to pre- and post-surgery variables, labelled as risk factors.

Longitudinal single-centre study involving 53 d-transposition of the great artery patients (81.1% male) after arterial switch operation, the latter being performed as unique surgical procedure in 39 patients (76.5%). Median follow-up was 59 months [23.5–72].

Selected patients were split into two groups according to risk factors. Fifteen patients (30.6%) were grouped into high-risk class (<3 risk factors). Echocardiographic variables such as tricuspid annular plane systolic excursion, ejection fraction, and global longitudinal strain were compared between the two groups. Only global longitudinal strain reached statistical significance (−17.56 ± 2.26 versus −19.82 ± 1.97 %; p < 0.001). To discriminate high- versus low-risk patients, a receiver operating characteristic (ROC) curve identified a global longitudinal strain cut-off value of −17.75% (sensitivity 57.1%, specificity 97%, AUC 80%).

Several neonatal and post-surgical variables might conditionate long-term follow-up of d-transposition of the great artery patients after arterial switch operation, and global longitudinal strain best conveys the overall risk profile of these patients.

To summarise the characteristics and postoperative outcomes in paediatric patients with coronary sinus septal defect.

This retrospective study recruited paediatric patients diagnosed with coronary sinus septal defect from the Guangdong Cardiovascular Institute between 2011 and 2023. Clinical characteristics, echocardiographic parameters, surgical procedures, and postoperative outcomes were collected from electronic health records.

Among the 68 patients, 50% were male, with a median age of 1.0 years. Four cases (5.9%) were diagnosed during the prenatal period. The proportions of patients with type I, II, III, and IV coronary sinus septal defect were 51.5%, 5.9%, 16.1%, and 26.5%, respectively. The most common coexisting cardiac anomalies were persistent left superior caval vein. Twenty-seven cases were either missed or misdiagnosed by echocardiogram, accounting for 39.7% of the overall cases, with type I being the most frequently missed diagnosis. Fifty-four patients underwent surgery, two patients received transcutaneous intervention, while the remaining patients did not undergo any surgery or intervention. At follow-up, two patients with type I coronary sinus septal defect died from multiorgan dysfunction, and one patient underwent reoperation due to narrowing of the extracardiac tunnel. The remaining patients did not experience any major events and recovered well.

Paediatric patients with coronary sinus septal defect often do not exhibit specific clinical manifestations. Enhancing our understanding of the anatomic and haemodynamic characteristics of coronary sinus septal defect can improve the diagnostic accuracy of echocardiography. If diagnosis is suspected, confirmation can be obtained by cardiac CT and cardiac magnetic resonance. Accurate preoperative and intraoperative diagnosis of coronary sinus septal defect contributes to high surgical success rates and favourable treatment outcomes.

Aortic coarctation can occur isolated or associated with ventricular septal defect. This study evaluated aortic stiffness in normotensive patients surgically treated for aortic coarctation and ventricular septal defect and in those who underwent simple aortic coarctation repair. Both groups were compared with healthy controls. Again, the two pathological groups were compared with each other regarding aortic stiffness and left ventricular diastolic function. A possible relationship between aortic stiffness and left ventricular diastolic function was investigated.

Twenty-two isolated aortic coarctation patients and 17 aortic coarctation and ventricular septal defect patients were enrolled. Aortic root distensibility and aortic stiffness index were calculated from echocardiography and blood pressure. E wave to A wave (E/A) ratio was measured from mitral valve inflow profile.

Aortic root distensibility and aortic stiffness index in simple aortic coarctation vs healthy controls: both p < 0.0001. Aortic root distensibility and aortic stiffness index in aortic coarctation/ventricular septal defect vs healthy controls: both p < 0.0001. Aortic root distensibility and aortic stiffness index were similar in the two pathological groups (both p = ns). No statistically significant difference was detected in relation to left ventricular diastolic function (p = ns). No correlation was detected between aortic stiffness and diastolic function in simple aortic coarctation and aortic coarctation/ventricular septal defect groups (both p = ns).

In both normotensive isolated aortic coarctation and aortic coarctation/ventricular septal defects subgroups, aortic stiffness is increased in a similar way in comparison with controls. Diastolic function was normal and similar in both groups. Aortic stiffness was not related to left ventricular diastolic function in this specific setting.

Syncope is common among pediatric patients and is rarely pathologic. The mechanisms for symptoms during exercise are less well understood than the resting mechanisms. Additionally, inert gas rebreathing analysis, a non-invasive examination of haemodynamics including cardiac output, has not previously been studied in youth with neurocardiogenic syncope.

This was a retrospective (2017–2023), single-center cohort study in pediatric patients ≤ 21 years with prior peri-exertional syncope evaluated with echocardiography and cardiopulmonary exercise testing with inert gas rebreathing analysis performed on the same day. Patients with and without symptoms during or immediately following exercise were noted.

Of the 101 patients (15.2 ± 2.3 years; 31% male), there were 22 patients with symptoms during exercise testing or recovery. Resting echocardiography stroke volume correlated with resting (r = 0.53, p < 0.0001) and peak stroke volume (r = 0.32, p = 0.009) by inert gas rebreathing and with peak oxygen pulse (r = 0.61, p < 0.0001). Patients with syncopal symptoms peri-exercise had lower left ventricular end-diastolic volume (Z-score –1.2 ± 1.3 vs. –0.36 ± 1.3, p = 0.01) and end-systolic volume (Z-score –1.0 ± 1.4 vs. −0.1 ± 1.1, p = 0.001) by echocardiography, lower percent predicted peak oxygen pulse during exercise (95.5 ± 14.0 vs. 104.6 ± 18.5%, p = 0.04), and slower post-exercise heart rate recovery (31.0 ± 12.7 vs. 37.8 ± 13.2 bpm, p = 0.03).

Among youth with a history of peri-exertional syncope, those who become syncopal with exercise testing have lower left ventricular volumes at rest, decreased peak oxygen pulse, and slower heart rate recovery after exercise than those who remain asymptomatic. Peak oxygen pulse and resting stroke volume on inert gas rebreathing are associated with stroke volume on echocardiogram.