Cardiology in the Young最新文献

Background: 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.

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

Methods: 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.

Results: 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).

Conclusion: 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.

Background: 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.

Methods: 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.

Results: 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.

Conclusion: 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.

Purpose: 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.

Case report: 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.

Conclusion: 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.

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

Background: 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 Amplatzer^TM 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.

Methods and results: 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%.

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

Coronary artery fistulas are rare cardiac anomalies, often asymptomatic and frequently discovered incidentally during routine cardiac imaging. Transcatheter closure with coil embolisation is increasingly recognised as a preferable alternative to surgical intervention due to its lower complication rates. In this report, we present three paediatric cases in which coronary artery fistulas were successfully embolised using the Azur® CX Peripheral Coil System. This system demonstrates low-profile suitability for occluding highly tortuous vessels in children, enabling controlled coil delivery via a microcatheter. Its hydrogel-coated coils allow for expansion between gaps, making it highly effective as a minimally invasive treatment.

Sinus tachycardia due to hyperthyroidism is generally treated with beta-blockers. But some patients do not respond to beta-blockers or may have non-tolerable side effects or have contraindications. We presented a case with persistent sinus tachycardia secondary to hyperthyroidism refractory to maximal doses of propranolol. After ivabradine treatment, her heart rate was < 100 bpm within 24 hours. There were no electrocardiogram changes or side effects. The use of ivabradine is promising and can be considered in cases where tachycardia cannot be controlled in children with hyperthyroidism.

We present the case of a 9-year-old girl with a distal aortopulmonary window (APW) with anomalous origin of right coronary artery from the main pulmonary artery and an isolated left subclavian artery arising from the main pulmonary artery. This report highlights the unusual combination of the three anomalies and late presentation of APW.

Kawasaki disease is an idiopathic vasculitis that involves the participation of various physiological systems and the inflammation of small-to medium-sized arteries, resulting in the formation of aneurysms. It most commonly affects children under 5 years. The development of coronary artery aneurysms is a significant concern in the context of Kawasaki disease, rendering it the prevailing acquired cardiac condition among children. It has been observed that 1-30% of Kawasaki illness patients experience neurological complications. However, facial nerve palsy is uncommon, with a prevalence of only 0.9%. Infants with Kawasaki disease and facial nerve palsy tend to be under 6 months of age and had a higher chance to develop coronary artery aneurysms compared to those without facial nerve palsy whose condition was never treated.

Approximately 90% of primary paediatric cardiac tumours are benign lesions. Depending on their location and size, benign cardiac tumours may cause inflow and outflow obstructions, cyanosis, valvular insufficiencies, myocardial ischaemia, associated dysfunction, systemic embolisation, arrhythmias, and even sudden death. Decision-making and timing for surgery can be challenging in children. Herein, we present an asymptomatic 11-year-old girl with papillary fibroelastoma in the mitral, tricuspid, and pulmonary valves, discussing the decision-making process and successful surgical management.

Kawasaki disease is a childhood vasculitic disorder that has a special predilection for coronary arteries. Kawasaki disease has been reported from all regions of the world, with an increasing incidence in several countries. Kawasaki disease is now the most common cause of acquired heart disease in children all over the world. However, it is concerning that the estimated vast majority of Kawasaki disease cases in low- and middle-income countries are not getting diagnosed and treated. The World Health Organization acknowledges cardiovascular disease in their priority of actions. The World Health Organization is invited to acknowledge the reality of Kawasaki disease in its list of cardiovascular diseases and take steps to facilitate the diagnosis and treatment of Kawasaki disease, especially in low- and middle-income countries. It is a disease of public health importance and needs urgent prioritisation by the World Health Organization.