Annals of Pediatric Cardiology最新文献

Obstruction of the superior vena cava (SVC) is a rare complication after cardiac surgery in infants and children. We present the case of an 8-year-old boy who underwent bi-directional Glenn shunt followed by takedown of Glenn shunt and complete repair for cyanotic congenital heart disease. After 4 years of surgery, the child developed features of superior vena caval (SVC) syndrome. Echocardiography and CT angiography revealed complete obstruction of SVC without any forward flow. Transcatheter intervention was performed successfully to re-canalize and stent the SVC to maintain its patency. The patient was doing well at follow-up appointments, with good laminar flow through the stent. In conclusion, transcatheter management of post cardiac surgery SVC obstruction was successful in this patient.

Mitral annular disjunction (MAD) is defined as a separation between the mitral annulus and the left ventricular myocardium and is most often seen in association with mitral valve prolapse (MVP). MAD has been linked to ventricular arrhythmias in adults, independent of MVP. However, it has rarely been reported in children. We, hereby, report two cases of MAD associated with a large atrial septal defect (ASD). Thus far, there are no consensus guidelines for the management of MAD. The additional association of large ASD further complicates the decision-making in these patients. To the best of our knowledge, this is the first report of the association of MAD with ASD. We further discuss the challenges in the management of this condition.

Hypoplastic left heart syndrome (HLHS) is a complex congenital heart defect (CHD) characterized by a spectrum of underdeveloped left-sided cardiac structures. It is a serious defect and warrants either 3-staged surgical palliation or a heart transplant. Despite numerous surgical advancements, long-term outcomes remain challenging and still have significant morbidity and mortality. There have been notable advancements in stem cell therapy for HLHS, including developments in diverse stem cell origins and methods of administration. Clinical trials have shown safety and potential benefits, including improved ventricular function, reduced heart failure, and fewer adverse events. Younger myocardium seems particularly receptive to stem cell signals, suggesting the importance of early intervention. This review explores the potential of emerging stem cell-based therapies as an adjunctive approach to improve the outcomes for HLHS patients.

A neonate born of third-degree consanguineous marriage presented on day 12 of life with congestive cardiac failure. A male sibling died at 3 months of age, cause of which was not known. He was treated with decongestive measures and multiple inotropes. 2D Echocardiogram revealed severe Left ventricular dysfunction with prominent trabeculations and deep recesses in the left ventricle suggestive of Left ventricular non-compaction. He was also found to have horse-shoe kidney. Considering the presence of cardiac left ventricular non compaction, horse-shoe kidney and family history of neonatal death and pregnancy loss clinical exome sequencing was done. It detected a homozygous missense variant in exon 6 of the AGK gene suggestive of Senger's syndrome. Baby was on regular follow-up and was thriving well on diuretics, sacubitril-valsartan and weekly levosimendan infusions. At 8 months of age, cardiac transplantation was successfully done and baby has been doing well post-transplantation. LVNC in children is rare with an estimated incidence of 0.11 per 100,000, the highest incidence being during infancy. Senger's syndrome is autosomal recessive in inheritance. Senger's syndrome associated with Left ventricular non compaction has been reported only once in literature so far. Renal manifestations in the form of horse shoe kidney like in our index baby has not been reported previously with Senger's syndrome.

Background: Open-heart surgery is challenging in preterm neonates and infants, and its feasibility in low-resource settings has not been defined. We describe our institutional experience with open-heart surgeries performed on consecutive preterm infants.

Materials methods and results: This was a single-center retrospective cohort from a tertiary hospital in Southern India and included consecutive preterm neonates (<37 weeks) admitted for open-heart surgery. This report is limited to babies who were <3 months at the surgery. The salient features of the 15 preterm included twin gestation: 7 (46.7%); median gestational age at birth: 35 weeks (28-36 weeks); median corrected gestational age at surgery: 37 weeks (33-40 weeks); birth weight: 1.75 kg (1.0-2.6 kg); weight at surgery: 1.8 kg (1.2-2.9 kg); and small for gestational age: 12 (80%). The heart defects included transposition of the great arteries (7), total anomalous pulmonary venous return (3), large ventricular septal defect (VSD) (1), and VSD with coarctation of the aorta (4). Eleven (73%) were mechanically ventilated preoperatively and five had preoperative sepsis. The mean cardiopulmonary bypass time was 169.7 ± 61.5 min, and cross-clamp time was 99.7 ± 43.8 min. There was no inhospital mortality; one baby expired during follow-up at 1 month. Postoperative mechanical ventilation duration was 126.50 h (84.25-231.50 h), and intensive care unit stay was 13.5 days (9-20.8). The total hospital stay was 39 days (11-95 days). Two children (13.3%) had postoperative sepsis.

Conclusion: Through collaborative multidisciplinary management, excellent outcomes are feasible in low-resource environments for selected preterm neonates undergoing corrective open-heart operations.

We describe a unique report of percutaneous closure of multiple secundum atrial septal defects in a child utilizing three Occlutech Figulla septal occluders deployed sequentially. The procedure was performed under live three-dimensional transesophageal echocardiography guidance.

Objective: Anthracycline administration in children is associated with cardiac dysfunction. Speckle-tracking echocardiography (STE) can detect subclinical cardiac damage that may go undetected by conventional two-dimensional (2D) echocardiography. This study aims to investigate medium-term anthracycline cardiotoxicity using STE and determine a safer administrable level of anthracyclines (ACs).

Methods: This observational case-control study enrolled 37 healthy controls and 78 pediatric cancer survivors who received chemotherapy. The patients were divided into two groups: cardiotoxic received (CR) and cardiotoxic free (CF). Data on segmental longitudinal strain (LS), global LS (GLS), and 2D echocardiographic parameters were collected after a drug-free period of at least one year.

Results: A total of 115 children with a mean age of 108 ± 55 months, of whom 66% were males, were included in the study. Both the groups of cancer survivors exhibited significantly reduced GLS compared to healthy controls (CR vs. controls, P = 0.001; CF vs. controls, P = 0.013), but no significant difference in left ventricular ejection fraction (LVEF) was observed (P = 0.75). Overall, cancer survivors treated with ACs demonstrated a significant reduction in strain in 10 left ventricular segments, particularly in the basal segments (P < 0.05). Among CR patients, those with impaired GLS (n = 43, GLS worse than -21.9) had significantly higher mean age and cumulative anthracycline dose compared to CR patients with normal GLS (age, P = 0.024; anthracycline dosage, P = 0.036). Using an anthracycline cutoff of 223 mg/m² resulted in a higher detection rate (49% vs. 25%) and fewer missed cases (51% vs. 74%) compared to the 360 mg/m² anthracycline cutoff.

Conclusion: Childhood cancer survivors demonstrate significantly reduced GLS while preserving a normal LVEF, which does not differ significantly from reference values of healthy children. The reduction in strain appears to be associated with higher anthracycline doses and older age. Lowering the anthracycline threshold to 223 mg/m² may improve the predictability of a decline in cardiac function using strain imaging at medium-term follow-up.

Retrieval of embolized ductal stents from the pulmonary or systemic circulation can be challenging. Most children benefit from surgical shunts in such scenarios. Although early retrieval is advised, stents lodged in the peripheral pulmonary tree can be inaccessible, making the removal complicated. In such patients, stents can be "parked" in the segmental pulmonary arterial branches for retrieval later. In the low-pressure single ventricle pulmonary circulation, partially expanded embolized stents, if left in situ, can precipitate pulmonary arterial thrombosis. This subset of patients may benefit from meticulous anticoagulation and antiplatelet agents. In our case report, we describe the successful extraction of an embolized ductal stent without damage to the right lower lobe pulmonary artery (PA). In the follow-up evaluation, the growth of the right PA is good.