This study investigated the prevalence of malnutrition, time to achieve caloric goals, and nutritional risk factors after surgery for CHD in a cardiac ICU.

This retrospective study included patients with CHD (1 month-18 years old) undergoing open-heart surgery (2021–2022). We recorded nutritional status, body mass index-for-age z-score, weight-for-length/height z-score, cardiopulmonary bypass and aortic cross-clamp time, Paediatric Risk of Mortality-3 score, Paediatric Logistic Organ Dysfunction-2 score, vasoactive inotropic score, total duration of mechanical ventilation, length of stay in the cardiac ICU, mortality, and time to achieve caloric goals.

Of the 75 included patients, malnutrition was detected in 17% (n= 8) based on the body mass index-for-age z-score and in 35% (n= 10) based on the weight-for-length/height z-score. Sex, mortality, cardiopulmonary bypass and aortic cross-clamp time, Paediatric Risk of Mortality-3, Paediatric Logistic Organ Dysfunction-2, and vasoactive inotropic score, duration of mechanical ventilation, and length of cardiac ICU stay were similar between patients with and without malnutrition. Patients who achieved caloric goals on the fourth day and those who achieved them beyond the fourth day showed statistical differences in mortality, maximum vasoactive inotropic score, duration of mechanical ventilation, cardiopulmonary bypass and aortic cross-clamp time, Paediatric Risk of Mortality-3, Paediatric Logistic Organ Dysfunction-2, and length of cardiac ICU and hospital stay (p< 0.05). Logit regression analysis indicated that the duration of mechanical ventilation, Paediatric Logistic Organ Dysfunction-2 and Paediatric Risk of Mortality-3 score was a risk factor for achieving caloric goals (p< 0.05).

Malnutrition is prevalent in patients with CHD, and concomitant organ failure and duration of mechanical ventilation play important roles in achieving postoperative caloric goals.

Data on arterial thromboembolism in children undergoing cardiac surgery are limited. We sought to characterise, and estimate rates of, incident and recurrent arterial thromboembolism, and describe antithrombotic therapies for treatment in a large multinational population of children with CHD undergoing cardiac surgery.

We queried the TriNetX global electronic health record (derived real-world data research platform) from 2017 to 2024 for patients less than 18 years of age and an index arterial thromboembolism within 1 year of congenital cardiac surgery. Data were descriptively analysed.

Of 20,102 children who underwent an index cardiac surgery for CHD, 206 (1.1%) developed an index arterial thromboembolism within 1 year of surgery: 111 (53.9%) had only arterial thromboembolism and 95 (46.1%) had concomitant venous thromboembolism. The most common anatomic site for arterial thromboembolism was the lower extremity (n = 141, 68.4%), and the most common surgery was the Glenn procedure (n = 35, 17%). Unfractionated heparin was utilised in 136 (67 %) and aspirin in 91 (44.2%) patients. Recurrent thromboembolism occurred in 36 (17.5%) patients within 1 year of the index thromboembolism.

Among children undergoing congenital cardiac surgery, arterial thromboembolism was rare (1% of patients), but the 1-year risk of recurrent thromboembolism was high, at 17.5%. Multicentre prospective cohort studies are warranted to further evaluate risk factors for recurrent thromboembolism, to facilitate future risk-stratified interventional trials designed to reduce the high thromboembolism recurrence risk in these children.

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.

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.

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.

Vascular rings cause highly variable clinical presentations. This study assesses the impact of prenatal versus postnatal diagnosis on clinical outcomes.

We conducted a single centre retrospective review of isolated vascular ring patients (without significant CHD) from 2011 to 2022 and compared clinical and operative data between patients with prenatal and postnatal diagnoses.

Of 177 patients, 45% (N = 80) had prenatal diagnosis. Between 2018 and 2022, 78% had prenatal diagnosis compared to 41% from 2013 to 2018 and 4% before 2013 (p < 0.001). 76.3% (N = 135) had a right aortic arch with left ligamentum arteriosum, 22.6% (N = 40) had a double aortic arch, and 1.1% (N = 2) had a left aortic arch with right ligamentum arteriosum. Postnatal diagnosis patients were more likely to have preoperative respiratory symptoms (55.7%), medications (34.0%), or admissions (24.7%) (versus 32.5%, 10.0%, and 11.3% of the prenatal diagnosis patients, p < 0.05) and require surgical repair (68.0% versus 38.8% of prenatal diagnosis patients, p < 0.0001). 54.8% of patients had surgical repair; prenatal diagnosis patients were younger at surgery, 7.5 (3-11) months compared to 16.0 (5-18) months in the postnatal diagnosis patients (p = .0014). Double aortic arch patients were more likely to require surgical repair (90.0%, compared to 44.5% with right aortic arch, p < 1e−4). Postnatal diagnosis patients had more residual postoperative symptoms (40.9% versus 16.1% in prenatal diagnosis patients, p = 0.01).

Prenatal diagnosis of vascular rings improves clinical surveillance, resulting in earlier surgical repair in symptomatic patients and diminished morbidity. Higher risk double aortic arch patients should have a tailored evaluation pathway.

Dysphagia is common in infants born with critical CHD. Thickened liquids are often used to treat dysphagia, but associated risks limit widespread use among feeding specialists. This survey aims to assess dysphagia treatment patterns and thickened liquid use across paediatric cardiac surgical centres.

A 24-question, cross-sectional survey. Convenience and snowball sampling methods were used to engage 52 paediatric cardiac surgical centres affiliated with the Cardiac Newborn Neuroprotective Network. Descriptive statistics were used to analyse and compare responses.

Twenty-six individual respondents represented 21 unique paediatric cardiac surgical centres. Most responses were from experienced, speech–language pathologists (78%) at medium size centres (88%). Ninety-three percent of responding centres used thickened liquids to treat dysphagia and 81% only after formal instrumental assessment of swallowing. Thickened oral feeding was used for single-ventricle patients by 85% versus 69% for two-ventricle patients. Barriers to recommending thickened oral feedings included the cost of thickening agents, parental non-adherence, and gastrointestinal concerns.

This is the first survey to report multi-institutional dysphagia treatment practice variation at United States congenital cardiac surgical centres. Thickened oral feedings are frequently used across centres in high-risk critical CHD patients but treatment benefit remains unclear. This survey highlights a broad scientific community poised to direct dysphagia research in critical CHD to address practice variation, short- and long-term impact of thickened oral feeding on feeding outcomes, and barriers to use and access of thickening agents.

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.

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.

Left bundle branch area pacing is a recent technique gaining rapid acceptance due to its broader target area and excellent electrical parameters. The aim of this study was to demonstrate the feasibility of left bundle branch area pacing in children and share short-term results.

A retrospective study conducted at a single centre between December 2021 and April 2024 involved 19 children who underwent left bundle branch area pacing using Select Secure leads. The study included echocardiographic evaluations, pacing parameters, and follow-up outcomes.

The cohort comprised 10 males and 9 females. Median age was seven years (range 2–18), and median weight was 38 kg (range 13–56). All patients had complete atrioventricular block, with seven having isolated congenital complete atrioventricular block and 12 postoperative complete atrioventricular block. In nine patients, transitioning from epicardial to endocardial pacing resulted in ventricular dysfunction due to chronic right ventricular pacing. The remaining patients received left bundle branch area pacing initially. One patient underwent implantation in a septal position close to the left bundle due to left bundle branch area pacing infeasibility. The median post-procedure QRS duration was 92 msec (range 80–117). Median R wave amplitude, threshold, and impedance values were 14.7 mV (range 13.3–16.8), 0.7 mV (range 0.5–1.1), and 728 ohms (range 640–762), respectively. Atrioventricular (DDD mode) leads were implanted in 10 patients, and ventricular leads (VVIR mode) were implanted in nine patients. Median fluoroscopy dose was 18.7 mGy (13.5–34.52).

Left bundle branch area pacing can be safely conducted in paediatric patients exhibiting a narrow QRS duration and stable pacing parameters.

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.

Children with CHD are at increased risk for neurodevelopmental disabilities and neuropsychological impairments throughout their life span. The purpose of this report is to share our experience building a sustainable, novel, inpatient, interdisciplinary Neurocardiac Critical Care Program to mitigate risks and optimize outcomes during the ICU stay.

A descriptive review was chosen to identify meaningful characteristics, challenges and lessons learned related to the establishment, expansion of and sustainability of Neurocardiac Critical Care Program in a 26-bed pediatric cardiac ICU.

We successfully launched, expanded, and sustained an interdisciplinary Neurocardiac Critical Care Program. Here, we share the foundation, framework, challenges, and lessons learned as we established and sustained the Neurocardiac Critical Care Program. The key elements of our program are (1) consistent engagement by pediatric neurologists in the cardiac ICU, (2) comprehensive education initiatives, (3) evidence-based clinical practice changes, and (4) quality improvement and research projects.

The development of a pediatric Neurocardiac Critical Care Program is feasible and sustainable. This program was informed by recent research related to perioperative and psychosocial risk factors that impact brain development and neurodevelopmental outcomes in this vulnerable population. By aligning our efforts, our multidisciplinary team is helping shift the paradigm in pediatric cardiac critical care to actively manage complex heart disease, while simultaneously and proactively mitigating risks to the developing brain and family unit.

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.

Vitamin D is crucial for normal organ function, vascular health and exercise performance, yet its deficiency is widespread. Patients with CHD often exhibit reduced exercise capacity. Limited research exists on vitamin D in CHD.

This study investigates serum 25-hydroxy vitamin D levels in 55 adult CHD patients (median age 31 years) compared to 55 age- and gender-matched controls without cardiac disease and examines associations with exercise capacity, peripheral microvascular function, muscle strength and biventricular function in CHD. Therefore, patients underwent fingertip arterial tonometry, transthoracic echocardiography, muscle strength measurements and cardiopulmonary exercise testing.

Results indicated that 93% of CHD patients and 91% of controls had 25-hydroxy vitamin D levels <30 ng/ml, with both groups showing varying values depending on the season in which the studies were conducted. No significant difference in 25-hydroxy vitamin D levels was found between patients and controls. While vitamin D levels in CHD patients did not significantly correlate with age, body mass index, blood pressure, peripheral microvascular function, high-sensitivity C-reactive protein, cholesterol levels, N-terminal-pro hormone B-type natriuretic peptide, ventricular function or muscle strength, a significant correlation was found with percent-predicted peak oxygen consumption (ρ=0.41, p = 0.005 and ρ=0.34, p = 0.02 for reference values following Wasserman and the LowLands registry, respectively), even after adjusting for season (p = 0.03 and 0.05, respectively).

In conclusion, vitamin D levels were similar between CHD patients and controls, but vitamin D insufficiency is common and linked to reduced exercise capacity in CHD. Further research is needed to determine whether vitamin D supplementation combined with exercise could be beneficial in CHD with vitamin D insufficiency.

Neurodevelopmental follow-up programmes for children with CHD help identify neurodevelopmental impairments and support the delivery of recommended interventions. The Cardiac Neurodevelopmental Outcome Collaborative, Diversity, Equity, and Inclusion Special Interest Group surveyed members to assess perceived patient barriers to neurodevelopmental follow-up, previous diversity and inclusion education, and confidence in caring for historically marginalised populations.

A link to a Redcap online survey was emailed to Cardiac Neurodevelopmental Outcome Collaborative members on 23 April 2022, with 4 weeks given to complete.

Eighty-four participants from 37 institutions in North America completed the survey. Respondents acknowledged that education on the importance of neurodevelopmental follow-up and frequent accommodations for cancellations or rescheduling clinic visits is essential. Language interpretation and written materials were available in languages other than English, but a limited number provided fully translated evaluation reports. Driving distance and the caregiver’s lack of understanding of the rationale for neurodevelopmental follow-up were the top perceived barriers to programme attendance. At the institutional level, training for cultural competency was typically provided, and most respondents felt comfortable caring for patients from diverse backgrounds. However, many agreed their programmes could do more to make evaluations accessible to historically marginalised/underserved populations.

Multiple barriers exist to cardiac neurodevelopmental follow-up, particularly for patients from under-represented minorities and for those whose primary language is not English. Surveying families will be valuable to understand how we may overcome these barriers. Further education about the importance of neurodevelopmental follow-up programmes continues to be essential.

The treatment for proximal aortic arch hypoplasia in paediatric patients is still controversial. While some authors favours direct tissue anastomosis, others state that patch augmentation may also be a good alternative. The aim of this study is to compare the results of arch reconstructions using bovine pericardium with the direct anastomosis technique.

Paediatric patients who underwent arch reconstruction via median sternotomy between 2019 and 2023 were evaluated. Patients were divided into two groups according to the repair method of arch reconstructions: direct native tissue anastomosis and bovine pericardial patch augmentation. Using perioperative data, the relationship between the surgical method and postoperative morbidity, in-hospital mortality, and the risks for early reintervention was investigated.

Between August 2019 and August 2023, 38 paediatric patients underwent arch reconstruction. The average age and weight of the patients were 40 days (15–157.5 days, interquartile) and 3.78 kg (3.2–6.0 kg, interquartile range), respectively. While completely native tissue anastomosis was applied in 18 of the patients (47.4%), bovine pericardial patch was used in arch reconstruction in 20 patients (52.6%). Cross-clamp time was found to be significantly longer in patients using bovine patches (p = .016). No difference was detected between the two surgical methods in terms of postoperative mortality and morbidity factors (p > .05). There was no significant difference between the two surgical procedures in terms of reintervention in the early period after discharge (p = .177).

Although early results of both reconstruction techniques may be promising, their reliability needs to be evaluated in detail with large-scale prospective studies.

Duchenne muscular dystrophy is a devastating neuromuscular disorder characterized by the loss of dystrophin, inevitably leading to cardiomyopathy. Despite publications on prophylaxis and treatment with cardiac medications to mitigate cardiomyopathy progression, gaps remain in the specifics of medication initiation and optimization.

This document is an expert opinion statement, addressing a critical gap in cardiac care for Duchenne muscular dystrophy. It provides thorough recommendations for the initiation and titration of cardiac medications based on disease progression and patient response. Recommendations are derived from the expertise of the Advance Cardiac Therapies Improving Outcomes Network and are informed by established guidelines from the American Heart Association, American College of Cardiology, and Duchenne Muscular Dystrophy Care Considerations. These expert-derived recommendations aim to navigate the complexities of Duchenne muscular dystrophy-related cardiac care.

Comprehensive recommendations for initiation, titration, and optimization of critical cardiac medications are provided to address Duchenne muscular dystrophy-associated cardiomyopathy.

The management of Duchenne muscular dystrophy requires a multidisciplinary approach. However, the diversity of healthcare providers involved in Duchenne muscular dystrophy can result in variations in cardiac care, complicating treatment standardization and patient outcomes. The aim of this report is to provide a roadmap for managing Duchenne muscular dystrophy-associated cardiomyopathy, by elucidating timing and dosage nuances crucial for optimal therapeutic efficacy, ultimately improving cardiac outcomes, and improving the quality of life for individuals with Duchenne muscular dystrophy.

This document seeks to establish a standardized framework for cardiac care in Duchenne muscular dystrophy, aiming to improve cardiac prognosis.

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.

We aimed to evaluate the clinical and laboratory characteristics and aetiological factors of patients who underwent pericardiocentesis for moderate to large pericardial effusion.

A total of 38 patients who underwent pericardiocentesis due to moderate-severe pericardial effusion and not related to cardiac surgery were included in the study.

The male-to-female ratio was 2.16, and found to be 7.5 in patients over 3 years of age. Mean age and body weight of the patients were 69.4 ± 74.9 months and 22.5 ± 22.4 kg. Dyspnoea (51.7%) was the most common complaint, followed by chest pain (37.9%). Tamponade was present in 23.7% of the patients. The largest diameter of effusion was 24.4 ± 10.4 mm. The amount of fluid drained was 279.24 ± 279 ml. Macroscopic appearance was serous in 12 (34.3%), and haemorrhagic in 18 (51.4%). No complication related to procedure was seen. Aetiology for efusion was infectious in 26%, idiopathic in 18%, iatrogenic in 11%, rheumatological in 11%, malignancy in 8%, cardiomyopathy in 8%, and other factors related in 18%. Of the 38 patients, 16 received nonsteroidal anti-inflammatory drugs (NSAID), and colchicine and corticostreoid were added in nine and two patients, respectively. A total of eight (21%) patients died during follow-up.

In conclusion, percutaneous pericardiocentesis can be applied safely and the underlying aetiology is decisive in the prognosis of the patient. Although pericardial effusion in children is often due to inflammation of the pericardium, it can develop as a finding of many local or systemic diseases that should be kept in mind.