Annals of Pediatric Cardiology最新文献

Introduction: Congenital heart disease (CHD)-associated malnutrition is a systemic consequence of CHD. Dietary recommendations to fulfill nutritional requirements are lacking. This randomized controlled trial (RCT) was conducted to determine the efficacy of early enteral feeding with supplemented expressed breast milk (suppl-EBM) versus expressed breast milk (EBM) in improving the weight of postoperative cardiac surgical infants.

Objective: The primary objective was the weight change between the EBM group and the suppl-EBM group at the 15^th postoperative day (POD) or intensive care unit (ICU) discharge. The secondary objectives were to compare the ventilation duration (VD), length of ICU stay (LOICUS), length of hospital stay (LOHS), macronutrient consumption, adverse events, sepsis, and mortality between the two groups.

Materials and methods: This study was a parallel-group, open-labeled, single-blinded, variable block size RCT conducted at a tertiary care teaching hospital in northern India. Full-term breastfed infants ≤6 months, weighing ≥2.5 kg at birth, and undergoing congenital cardiac repair were enrolled in this study. The infants were fed either EBM or supplemented EBM in control and intervention groups, respectively. Weight and length were measured at baseline and 15^th POD or at ICU discharge. Biochemical parameters at baseline and every alternate day, sepsis parameters every third POD and VD, LOICUS, LOHS, macronutrient consumption, and adverse events were assessed daily.

Results: The mean weight, weight change percentage, and weight for age z score were significantly higher in the supplemented EBM group (P < 0.05). The macronutrient consumption was significantly higher in the intervention group (P < 0.05). No significant difference was found between the two groups for VD, LOICUS, and LOHS (P > 0.05). The sepsis was higher in the EBM group. However, the mortality rate did not differ between the two groups (P > 0.05).

Conclusion: Supplemented feeding may improve the weight of postoperative cardiac infants with no serious adverse events.

The right venous valve is critical to the proper development of the fetal heart. As the right venous valve degenerates later in fetal development, residual structures can remain, such as the terminal crest, Eustachian valve, Thebesian valve, and a prominent Chiari network, with a sail-like appearance resembling a spinnaker. While these structures are often incidentally found on adult echocardiograms, we present a case of a term neonate with hypoxia secondary to a persistent right valve of the sinus venosus.

Coronary sinus (CS) defects are rare congenital cardiac anomalies that occur in isolation or with other congenital heart diseases. Persistent left superior vena cava (LSVC) is a relatively common entity that usually drains into the CS, is of no hemodynamic consequence, and is easily diagnosed on echocardiography by a dilated CS and an antegrade flow toward the heart. However, a combination of LSVC and CS defect may reverse its flow direction and CS dilation may be absent. In the absence of echocardiographic clues, the reversed flow can be easily misdiagnosed for other structures such as the more common vertical vein of anomalous pulmonary venous connection or the rarer levoatrial cardinal vein. Here, we report a 2-year-old boy with ventricular septal defect, patent ductus arteriosus, absent CS, and a persistent LSVC with retrograde flow producing a hemodynamically significant pretricuspid left-to-right shunt along with its diagnostic challenges. He underwent successful surgical closure of these defects and had been asymptomatic on follow-up.

Background: Branch pulmonary artery (PA) stenosis must be addressed early to prevent right ventricular scarring and establish lung blood flow. Balloon-mounted stents are more useful in managing right ventricular outflow tract (RVOT) obstruction and PA stenosis.

Materials and methods: We studied the clinical and angiographic data of children with congenital heart disease who underwent stenting for RVOT obstruction and branch PA stenosis using the Formula stent (Cook Medical, Limerick, Ireland) between 2018 and 2024 in a tertiary pediatric cardiac center in southern India.

Results: Our patient cohort included 65 patients with a median age of 3 years (range: 3 months to 17 years). The median weight of patients was 11.5 kg (4.8-60.4 kg), with 40% weighing <10 kg. Out of the 87 stents implanted, four stents were implanted in RVOT, while the rest were branch PA stenting. The median stent length was 20 mm (12-60 mm), and the median stent diameter was 10 mm (5-10 mm). All stents were deployed using 5Fr to 8Fr sheaths or guiding catheters. The median procedure time was 62 min (20-170 min), with a median fluoroscopy time of 15 min (4-110 min). A total of 17 (19%) stents in 14 patients required further dilatation at a mean interval of 25 ± 4 months from the time of stenting. Ballooning resulted in an increase in diameter from 7.8 ± 2.2 mm to 10.2 ± 1.7 mm. Right ventricular systolic pressure had reduced from 70.3 ± 8 to 40.1 ± 1 mmHg after balloon dilatation in patients with a biventricular heart.

Conclusion: The Formula stent provides a comprehensive stent size option, requiring smaller sheaths, reduced procedural time, minimum complications, and an acceptable rate of reinterventions.

Kawasaki disease, traditionally classified as medium vessel vasculitis, is known for the preferential involvement of coronary arteries. Infrequently, large systemic arteries might be involved and may affect the prognosis. Here, we present an infant with Kawasaki disease who had extensive involvement of large vessels such as the abdominal aorta and medium vessels of the extremities, along with giant coronary artery aneurysms.

Background and objective: Respiratory distress is the most common cause requiring neonatal intensive care unit admission. As respiratory and cardiac functions are closely interrelated, some cardiac dysfunction is expected in respiratory distress. The myocardial performance index (MPI) is an index to assess global myocardial function, easily measurable by bedside echocardiography and reliable. Here, we conducted this study to determine the change in cardiac function in neonates with respiratory distress before initiating respiratory support and after weaning from the support.

Methodology: The study was carried out in 92 neonates with a gestational age of more than 32 weeks who required invasive or noninvasive respiratory support. The tissue Doppler left ventricular MPI (LV MPI) was calculated before the initiation of respiratory support and after weaning from respiratory support. The data were analyzed using a paired t-test and a Wilcoxon signed-rank test.

Results: This study comprised 92 neonates with a median (interquartile range) LV MPI value of 0.56 (0.10) before initiation of respiratory support and 0.47 (0.04) after weaning from respiratory support with P < 0.001. The isovolumetric contraction time, isovolumetric relaxation time, and ejection time increased after weaning from respiratory support (all P < 0.0001). The severity of respiratory distress determined by invasive mode of ventilation and longer duration of respiratory support caused higher initial LV MPI before initiation of respiratory support compared with recovery and P < 0.001, suggesting subclinical ventricular dysfunction with respiratory distress in neonates.

Conclusion: LV MPI was higher in neonates with respiratory distress and normalized after weaning from respiratory support, which indicates that neonates requiring respiratory support may have subclinical ventricular dysfunction and should be followed up carefully.

Generalized arterial calcification of infancy is a rare entity with poor fetal and postnatal outcomes and high morbidity in survivors. Half of the cases are diagnosed intrauterine due to hemodynamic compromise, and the associated manifestations pose unique management challenges. We hereby report an account of a fetal diagnosis in a referral for hydrops with postnatal evaluation and management.

Absent pulmonary valve syndrome, commonly linked with tetralogy of Fallot and ventricular septal defect, is a rare congenital condition. It is exceedingly rare to have an isolated absent pulmonary valve with an intact ventricular septum without cardiovascular shunt lesions, such as an atrial/ventricular septal defect or patent ductus arteriosus. This report presents a case of such rarity involving a young child with recurrent lower respiratory tract infections.

Residual defect after surgical closure of atrial septal defect is extremely uncommon. This communication reports four cases encountered in a tertiary care center during the last three decades. Clinical diagnosis was challenging, and the diverse presentations included acute ischemic stroke, cyanosis, and right ventricular volume overload. The morphology of the residual defects was complex, and multimodality imaging (transesophageal echocardiography, peripheral venous contrast studies, computed tomography, and balloon occlusion) enabled accurate recognition. Percutaneous device closure was feasible in one but required repeat surgery owing to unfavorable anatomy in the others. The communication focuses on difficulties in diagnosis and management.

Introduction: 22q11.2 deletion is associated with conotruncal anomalies and immunological aberrations. Given the common embryonic origin of conotruncus and thymus, conotruncal anomalies may be associated with immunological aberrations irrespective of 22q11.2 deletion. We planned to study the prevalence of immunological aberrations and 22q11.2 deletion among patients with conotruncal anomaly to understand the impact of their interplay.

Patients and methods: Preoperative children (age <12 years) with conotruncal anomalies were evaluated for clinical dysmorphism, lymphocyte subsets by flowcytometry, immunoglobulin levels by nephelometry, and 22q11.2 deletion by multiplex ligand-dependent probe amplification (January 2021-June 2022). Patients with asplenia and polysplenia were excluded from immunological studies.

Results: Major cardiac defects ([n = 101], [median age, 32 days]) included dextro-transposition of great arteries (d-TGA) - 41.6%, tetralogy of Fallot - 37.6%, double outlet right ventricle (DORV) - 13.9%, and truncus arteriosus - 4.9%. Four patients had polysplenia with situs inversus, while 17 had clinical dysmorphism. Flow cytometry (n = 82) revealed low absolute counts of lymphocytes (33%), T-cells (51.2%), CD4+ cells (50%), and CD8+ cells (51.2%), while only 14.1% had low IgG levels. Eight patients (8/95, 8.4%) had 22q11.2 deletion, with universal deletion of TBX1-2 and TBX1-7 genes; the other 19 genes were deleted in various combinations. Two patients with 22q11.2 deletion had normal T-cell subsets, while none had a complete absence of T-cells.

Conclusion: Immunological aberrations, especially T-cell abnormalities, were present in almost half of the patients, irrespective of 22q11.2 deletion. Only 8.4% of patients had 22q11.2 deletion. The high incidence of d-TGA among 22q11.2 deletion patients needs further exploration.