Fear of cardiac arrest among parents of infants with heart disease can cause stress and anxiety. Literature is scarce on the effects of cardiopulmonary resuscitation training (CPRt) on anxiety and stress of parents. We analysed the impact of CPRt on anxiety, stress, and comfort levels on parents of infants with heart disease.

Cardiopulmonary resuscitation (CPR) and choking relief manoeuvre (CRM) comfort level, Parental State-Trait Anxiety Inventory (STAI), and Parenting Stress Index (PSI) scores were prospectively collected pre-, immediately post-, and 3 months post-CPRt.

There were 97 participants: 80% (n = 78) mothers/grandmothers and 20% (n = 19) fathers. The mean (SD) age of participants was 28.7 (5.6) years old. There was a significant decrease in STAI across the three time points collected; STAI decreased by 12% from baseline to immediately post-CPRt and 19% from baseline to 3 months post-CPRt (p < .0001). There were no significant changes in PSI across the time points. Baseline to immediately post-teaching, we found that CPRt significantly increased comfort performing CPR, CRM, and comfort in knowing what to do (p=< .001, p=< .001, p=< .001, respectively). Comfort levels persisted elevated when comparing pre- to 3 months post-CPRt (p=< .001, p= .002, p= .001, respectively), maintaining at least a 177% average increase up to 3 months post-CPRt for all aspects.

CPRt can aid in improving anxiety and comfort levels of parents of infants with heart disease around hospital discharge. Parental preparedness and reassurance to know what to do in emergency situations can be enhanced by a simple intervention such as CPRt.

Right anterolateral thoracotomy or left anterolateral thoracotomy applied to partial anomalous pulmonary venous connection correction have been reported to obtain cosmetic and less invasive outcomes as alternative approaches to median sternotomy. However, the application of different approaches is still confusing. We compared the perioperative and mid-term outcomes to seek indications of different approaches and guide surgical treatment of partial anomalous pulmonary venous connection.

From July 2019 to August 2023, 44 patients who underwent surgical correction of partial anomalous pulmonary venous connection were recruited. Of these, 13 (29.6%) patients who underwent median sternotomy were separated into M group, 28 (63.6%) patients who underwent right anterolateral thoracotomy were separated into R group, and three (6.8%) patients who underwent left anterolateral thoracotomy were separated into L group. Clinical data were reviewed and compared between each group.

In M group, direct anastomosis accounted for the most (7, 53.8%), in R group, intra-atrial rerouting accounted for the most (22, 78.6%), and in L group, direct anastomosis accounted for the most (3, 100%). R group had shorter cardiopulmonary bypass time (70.0 ± 32.4 vs113.1 ± 83.3, p = 0.029), shorter ICU stay (1.0 ± 0.2 vs 1.9 ± 1.7, p = 0.01), shorter postoperative stay (6.0 ± 1.2 vs 8.3 ± 5.0, p = 0.021), and fewer chest tube drainage (8.4 ± 4.3 vs 13.9 ± 10.2, p = 0.026) compared with M group. Three left-sided partial anomalous pulmonary venous connection in L group adopted off-pump approach.

Median sternotomy S.V.C. can be applicable to all anatomic types of partial anomalous pulmonary venous connection especially for complex types. Considering the minimally invasive advantages, we prefer applying right anterolateral thoracotomy for pulmonary vein connected to right atrium or proximal end of off-pump. Applying left anterolateral thoracotomy for left-sided partial anomalous pulmonary venous connection without atrial septal defect by off-pump.

Cardiomyopathy is the leading cause of death in patients with Duchenne muscular dystrophy. The relationship between cardiac and skeletal muscle progression is unclear. The objective of this study was to evaluate the correlation between muscle activity and cardiomyopathy. We hypothesised that cardiomyopathy and skeletal muscle activity are directly related.

Physical activity was assessed with accelerometers worn for 7 days. Average activity (vector magnitude/min) and percentage of time in different activities were reported. Cardiac MRI was used to assess left ventricular ejection fraction, global circumferential strain (Ecc), late gadolinium enhancement, and cardiac index. Associations were assessed between physical activity and cardiac variables using a Spearman correlation.

Duchenne muscular dystrophy subjects (n = 46) with an average age of 13 ± 4 years had a mean left ventricular ejection fraction of 57 ± 8%. All physical activity measures showed significant correlations with left ventricular ejection fraction (rho = 0.38, p = 0.01) and left ventricular cardiac index (rho = 0.51, p < 0.001). Less active subjects had lower left ventricular ejection fraction (p = 0.10) and left ventricular cardiac index (p < 0.01). Non-ambulatory patients (n = 29) demonstrated a stronger association between physical activity and left ventricular ejection fraction (rho = 0.40, p = 0.03) while ambulatory patients demonstrated a stronger association between physical activity and left ventricular cardiac index (rho = 0.53, p = 0.03). Ecc did not associate with physical activity in either cohort.

Physical activity correlates with left ventricular ejection fraction and left ventricular cardiac index and is modified by ambulation. Future analysis should assess the temporal relationship between physical activity and cardiomyopathy.

To retrospectively assess the suitability of pulmonary artery banding as a treatment strategy for dilated cardiomyopathy and left ventricular non-compaction cardiomyopathy with depressed left ventricular ejection fraction.

The study was retrospective and included consecutive patients who met the inclusion criteria: diagnosed with dilated cardiomyopathy or left ventricular non-compaction cardiomyopathy and left ventricular ejection fraction less than 35%. Cardiac indices were documented, and clinical outcomes were followed for 5 years.

This study included 21 patients with depressed left ventricular ejection fraction due to dilated cardiomyopathy (n = 11) or left ventricular non-compaction cardiomyopathy (n = 10), treated either with anti-congestion medication alone or in combination with pulmonary artery banding. The groups treated with pulmonary artery banding showed significant improvement in left ventricular ejection fraction compared to controls (ANOVA, p = 0.0002), with no major adverse events. In the subgroup with left ventricular non-compaction, pulmonary artery banding led to significant improvement of the left ventricular ejection fraction (p = 0.00002) and significant reductions in the Z scores of left ventricular end-diastolic diameter (p = 0.0002) and of end-diastolic volume (p = 0.004).

Pulmonary artery banding appears to be a viable strategy for improving heart function in patients with non-compaction and dilated cardiomyopathy and depressed left ventricular ejection fraction. While pulmonary artery banding demonstrated more pronounced benefits in the subgroup with non-compaction cardiomyopathy, significantly enhancing cardiac restoration indices throughout the follow-up period, warranting further investigation in larger studies.

Sudden cardiac death is a significant concern among patients with congenital heart disease (CHD). We assessed the risk of remote sudden cardiac death after congenital heart surgery.

Patients undergoing congenital heart surgery before 21 years of age between 1982 and 2003 in the Pediatric Cardiac Care Consortium registry were linked to National Death Index data through 2019. Sudden cardiac death was defined as death associated with a cardiac arrest or ventricular fibrillation diagnosis code. Standardised mortality ratios relative to the general population were calculated using Centers for Disease Control and Prevention data.

Among 30,566 patients discharged after their initial surgery, 2,718 deaths occurred over a median period of 23 years (IQR 19–27). Of 463 (17%) sudden cardiac deaths, the median age was 1.7 years (IQR 0.5–16.5). The mean incidence was 7 per 10,000 person-years (95% CI: 0.64–0.77), ranging from 2.7 for left-to-right shunt lesions to 37 for single-ventricle physiology. Cardiac comorbidities including heart failure (13.6%) and arrhythmias (7.1%) were more frequent among sudden cardiac death patients. Standard mortality ratios for sudden cardiac death were elevated across all CHD types, ranging from 8.0 (95% CI: 6.3–9.6) for left-to-right shunts to 107.7 (95% CI: 88.9–126.5) for single-ventricle physiology.

Sudden cardiac death risk is higher post-congenital heart surgery compared to the general population. Even patients with mild CHD are at risk, highlighting the need for long-term follow-up for all patients. Heart failure and arrhythmia prevalence suggest potential therapeutic targets to reduce sudden cardiac death risk.

This study aims to examine the surgical outcome of Kabuki syndrome patients after neonatal congenital heart surgery.

This was a single-centre retrospective study of Kabuki syndrome patients undergoing neonatal congenital heart surgery from 2018 to 2023. Primary outcome was survival to discharge after index surgery. Secondary outcomes were morbidities and complications. Survival and hospital length of stay were compared to neonates with non-Kabuki genetic anomalies undergoing congenital heart surgery in the same time period.

A total of seven patients were reviewed. All Kabuki syndrome patients had left-sided lesions including three with hypoplastic left heart syndrome, three with aortic stenosis and/or aortic arch hypoplasia, and one with an isolated coarctation of aorta. Hospital survival was 5/7 (71% compared to 88% for neonates with non-Kabuki genetic anomalies). To date, four remain alive, including one with hypoplastic left heart syndrome. A higher percentage of Kabuki syndrome patients had unplanned interventions (43% vs 15% in non-Kabuki), abnormal brain imaging (29% vs 5%), and bacteremia (29% vs 9%). Median total ventilator days for Kabuki patients were also longer (16 days vs 6 days in non-Kabuki) as was hospital length of stay (66 days vs 41 days).

Despite survival to discharge after index operation, Kabuki syndrome patients with single ventricle physiology remain at high risk of mortality and morbidity after cardiac surgery. However, they may be discharged without ventilator dependency and survive to toddler years.

Sodium-glucose cotransporter-2 inhibitors reduce cardiovascular outcomes in patients with congestive heart failure and a biventricular circulation. Congestive heart failure in Fontan univentricular circulation is distinctly different. Experience with sodium-glucose cotransporter-2 inhibitors in this group has not yet been well described.

This work describes safety and tolerability of sodium-glucose cotransporter-2 inhibitors in patients with Fontan circulation.

Single-centre review of patients with Fontan circulation prescribed a sodium-glucose cotransporter-2 inhibitors for congestive heart failure. Primary outcome was tolerability or need for discontinuation. Secondary outcomes were changes in New York Heart Association class, congestive heart failure hospitalisation, ventricular function, exercise performance, and laboratory values.

We identified 25 patients with Fontan circulation prescribed an sodium-glucose cotransporter-2 inhibitors, most with a systemic right ventricle. Over a third of subjects had at least moderately reduced baseline ventricular function. Baseline catheterisation showed a mean Fontan pressure of 17.1 ± 3.7 mmHg and pulmonary capillary wedge pressure 11.7 ± 3.2 mmHg at rest; 59% had occult diastolic dysfunction with abnormal pulmonary capillary wedge pressure elevation following volume expansion. Most were on congestive heart failure medications and/or a pulmonary vasodilator prior to sodium-glucose cotransporter-2 inhibitors addition, and three had a congestive heart failure hospitalisation within the previous year. All reported good medication tolerance except one patient was nonadherent to medications and two discontinued sodium-glucose cotransporter-2 inhibitors for perceived side effects. There were no significant differences in secondary outcomes. There was, however, a downward trend of serum brain natriuretic peptide (n = 13) and improved peak VO2 (n = 6), though neither statistically significant (p > 0.05).

This series, the largest published to date, suggests that sodium-glucose cotransporter-2 inhibitors are safe and tolerable congestive heart failure therapy in Fontan circulation. Further research is warranted to explore therapy in this unique population.

To demonstrate safety and efficacy of using the AmplatzerTM vascular plug II device for perimembranous ventricular septal defect closure with retrograde approach and show the follow-up in all patients.

At present, there is no FDA-approved device for transcatheter closure of perimembranous ventricular septal defects. Small studies and case reports have shown the use of various catheter-based devices in an off-label management; however, there are no large studies to show their efficacy. The second generation of AmplatzerTM vascular plug seems to offer a safe and attractive alternative for this procedure. Besides, a retrograde approach might decrease procedure time and radiation exposure time.

Patients with congenital perimembranous ventricular septal defects who underwent transcatheter closure using Amplatzer vascular plug II devices were included. Primary end point was to determine efficacy and safety of this generation of devices and the incidence of complications at follow-up (complete heart block and aortic/tricuspid/mitral regurgitation). Forty-five patients underwent perimembranous ventricular septal defect closure at a median age of 6 years (9 months–17 years). During the catheterization, there were only minor complications and at follow-up of 48 ± 25.7 months (up to 96 months). Closure rate was high of 93.3% and freedom from atrioventricular block was 100%.

The second generation of the AmplatzerTM vascular plug II seems to offer a safe and attractive alternative for percutaneous closure of the perimembranous ventricular septal defects.

Desbuquois dysplasia type 1 is a rare autosomal recessive chondrodysplasia characterised by distinct skeletal abnormalities and multisystem involvement, including pulmonary, renal, and ocular abnormalities, has also been reported. Cardiac complications, although infrequently discussed in the literature, include aortopathy and atrioventricular valve prolapse, potentially due to defective proteoglycan production.

This case report details a 7-year-old male diagnosed with Desbuquois dysplasia type 1 and a coronary-cameral fistula, both of which are exceedingly rare conditions. Genetic analysis revealed a previously reported homozygous pathogenic variant in the calcium-activated nucleotidase 1 gene, ENST00000c.898C>T; p.Arg300Cys. Echocardiographic findings indicated significant cardiac enlargement, mitral valve prolapse, coronary-cameral fistula, pulmonary hypertension, advanced aortic root enlargement and aneurysmatic ascending aorta, and atrial septal defect, necessitating careful clinical management.

This case underscores the complexity of Desbuquois dysplasia and its associated cardiac anomalies, highlighting the need for further research into the systemic implications of this disorder. To the best of our knowledge, this case has importance as it is the first of its kind in the literature.

The interstage period is a critical phase for single ventricle infants due to their fragile cardiovascular state. Infants often experience medical and feeding challenges during this period, resulting in caregiver stress. We completed a quality improvement project at Children’s Healthcare of Atlanta to understand these challenges to inform targeted interventions.

This single-center project included a medical chart review and a cross-sectional caregiver survey. Data were collected on patient and caregiver demographics and clinical variables. Feeding outcomes were assessed using the Pediatric Functional Oral Intake Scale. Caregiver impact was measured using the Feeding/Swallowing Impact Survey.

The project included 15 single ventricle patients with a mean (standard deviation) age of 151.73(25.92) days at the time of the second-stage palliation. Forty percent of patients experienced at least one readmission, primarily due to feeding intolerance (20%) and desaturations (26.7%). Milk protein allergy (26.9%) was the most common medical complication, followed by interstage unplanned reinterventions. Pediatric Functional Oral Intake Scale scores demonstrated that 33% consumed minimal volumes or no oral intake at the time of the bidirectional Glenn, and 93.3% of patients did not receive outpatient feeding services during the interstage. Caregiver stress scores resulted in mean scores (standard deviation) of 2.23(1.54), with the highest impact on daily activities. All caregivers affirmed the need for a dedicated multidisciplinary clinic.

The interstage period for single ventricle patients poses significant medical and feeding challenges, resulting in caregiver stress. Comprehensive, multidisciplinary feeding support during the interstage period may improve patient outcomes and alleviate caregiver burden.

Primary pulmonary vein stenosis is a rare congenital heart condition and carries a poor prognosis. Various surgical techniques have been introduced to treat primary pulmonary vein stenosis. However, there is no consensus on the optimal approach. This study aimed to evaluate the outcomes of surgical treatment of primary pulmonary vein stenosis, identify the risk factors for pulmonary vein restenosis and reintervention, and determine the optimal strategy for primary pulmonary vein stenosis repair.

From December 2008 to December 2023, 64 patients with primary pulmonary vein stenosis undergoing surgical repair in our institution were included [non-sutureless group, n = 42; sutureless group, n = 22]. The Cox proportional hazard model was used to identify risk factors for pulmonary vein restenosis and pulmonary vein restenosis-related reintervention.

There were three in-hospital deaths and one late death. The survival rates at 1, 5, and 15 years were 91.9%, 84.2%, and 69.8%, respectively, in the whole cohort. Compared with the non-sutureless group, the sutureless group did not significantly improve the long-term survival of primary pulmonary vein stenosis patients (P = 0.484). Pulmonary vein restenosis occurred in 15 patients. Multivariable analysis showed that Involvement of the left inferior pulmonary vein (P < 0.001) was the only independent risk factor for pulmonary vein restenosis, while sutureless repair (P = 0.037) was a protective factor. Pulmonary vein restenosis-related reintervention occurred in 12 patients. On multivariate analysis, three or four pulmonary veins involvement (P = 0.001) and preoperative severity score exceeding 5 (P = 0.050) were found to be independent risk factors associated with pulmonary vein restenosis-related reintervention; however, sutureless repair (P = 0.021) was a protective factor.

Management of infants with primary pulmonary vein stenosis is challenging. Surgical repair of primary pulmonary vein stenosis can be safely achieved using different techniques, with similar long-term mortality. Compared with non-sutureless repair, sutureless repair was significantly associated with decreased pulmonary vein restenosis and decreased pulmonary vein restenosis-related reintervention, respectively. Preoperative severity score exceeding 5, 3, or 4 pulmonary veins involvement, and left inferior pulmonary vein involvement are independent risk factors for adverse outcomes.

Congenital heart Disease (CHD) is a significant cause of morbidity and mortality. Pulse-oximetry is a good non-invasive simple tool for critical CHD screening. Implications of this tool may be possible in certain areas and non-practical in others. We aim to report on the preliminary results of a recent ongoing protocol concerning the use of pulse-oximetry in detecting critical CHD in newborn in Egypt.

All neonates born in or transferred to 10 university hospitals during the period between February and November 2023 and fulfilled the criteria of inclusion were screened for critical CHD by pulse-oximetry using Granelli protocol in the first 24–72 hours after birth.

During a 10-month pilot period, a total of 2392 neonates were screened. A total of 549 neonates (23%) tested positive (failed) screening. Among the positive cases, 213 neonates (42%) died during their hospital stay, while the remaining were discharged or scheduled for intervention. The positive cases underwent echocardiography that revealed CHD in the majority of cases (80.3%). Only 40 cases of those cases had cardiac defects that are classified as critical CHD with a prevalence of 16.7 per 1000 live births, while the rest of the cases have either simple (non-critical CHD) or persistent pulmonary hypertension. About 19.7% of positive cases have completely free echocardiograms without cardiac defects.

The prevalence of critical CHD in Egypt is higher than the mean worldwide prevalence. The introduction of pulse-oximetry as a mass screening tool for critical CHD is possible and effective in low-income countries.

Infants who require cardiopulmonary bypass for surgical repair of CHD are at high risk for secondary infections, which cause significant death and disability. The risk of secondary infection is increased by immune dysfunction. The intestinal microbiome calibrates immune function. Infants with CHD have substantial changes in their intestinal microbiome. We performed this scoping review to describe the current understanding of the relationship between the intestinal microbiome and immune function after pediatric cardiac surgery with cardiopulmonary bypass.

We searched the PubMed, Cumulative Index to Nursing and Allied Health Literature, Cochrane, and Scopus databases with the assistance of a medical librarian. We included trials that analysed intestinal microbiome composition and immune function after cardiac surgery with cardiopulmonary bypass in infants.

We found two observational cohorts and two interventional trials describing composition of intestinal microbiome and some measures of immune function after heart surgery with cardiopulmonary bypass in infants. A total of 114 children were analysed. Three trials were exclusively in infants, and one was in older children and infants. All trials found a differential composition of the intestinal microbiome in infants with CHD compared to those without CHD, and one described a robust correlation between composition of the intestinal microbiome with cytokine profile and adverse outcomes.

Despite robust preclinical data and data from other disease states, there is minimal data about the correlation between immune function and intestinal microbiome composition in infants with CHD after cardiopulmonary bypass.