Project description:This study investigated the characteristics of congenital rubella syndrome (CRS)-associated cardiac complications, particularly patent ductus arteriosus (PDA). We reviewed the medical records of patients with CRS who were admitted to the Children's Hospital 1 in Vietnam between December 2010 and December 2012, and patients with CRS who underwent PDA transcatheter occlusion therapy at the cardiology department between December 2009 and December 2015. We compared the characteristics of PDA treated with transcatheter closure between children with CRS (CRS-PDA) and those without CRS (non-CRS-PDA) who underwent PDA transcatheter closure between July 2014 and December 2015. One-hundred-and-eight children with CRS were enrolled. Cardiac defects (99%), cataracts (72%), and hearing impairment (7%) were detected. Fifty CRS-PDA and 290 non-CRS-PDA patients were examined. CRS-PDA patients had smaller median birthweight (p < 0.001), more frequent pulmonary (p < 0.001) and aortic stenosis (p < 0.001), higher main pulmonary artery pressure, and higher aortic pressure in systole/diastole (p < 0.001 for each) than did non-CRS-PDA patients. The proportion of tubular-type PDA was higher in CRS-PDA patients (16%) than in non-CRS-PDA patients (3%) (p = 0.020). Tubular-type PDA was frequently seen in patients with CRS and accompanied by pulmonary/systemic hypertension and pulmonary/aortic stenosis; in these patients, more cautious device selection is needed for transcatheter PDA closure.
Project description:Postnatal ductal closure is stimulated by rising oxygen tension and withdrawal of vasodilatory mediators (prostaglandins, nitric oxide, adenosine) and by vasoconstrictors (endothelin-1, catecholamines, contractile prostanoids), ion channels, calcium flux, platelets, morphologic maturity, and a favorable genetic predisposition. A persistently patent ductus arteriosus (PDA) in preterm infants can have clinical consequences. Decreasing pulmonary vascular resistance, especially in extremely low gestational age newborns, increases left-to-right shunting through the ductus and increases pulmonary blood flow further, leading to interstitial pulmonary edema and volume load to the left heart. Potential consequences of left-to-right shunting via a hemodynamically significant patent ductus arteriosus (hsPDA) include increased risk for prolonged ventilation, bronchopulmonary dysplasia, necrotizing enterocolitis or focal intestinal perforation, intraventricular hemorrhage, and death. In the last decade, there has been a trend toward less aggressive treatment of PDA in preterm infants. However, there is a subgroup of infants who will likely benefit from intervention, be it pharmacologic, interventional, or surgical: (1) prophylactic intravenous indomethacin in highly selected extremely low gestational age newborns with PDA (<26 + 0/7 weeks' gestation, <750 g birth weight), (2) early targeted therapy of PDA in selected preterm infants at particular high risk for PDA-associated complications, and (3) PDA ligation, catheter intervention, or oral paracetamol may be considered as rescue options for hsPDA closure. The impact of catheter-based closure of hsPDA on clinical outcomes should be determined in future prospective studies. Finally, we provide a novel treatment algorithm for PDA in preterm infants that integrates the several treatment modalities in a staged approach.
Project description:This is the case of a young female who was seen at our adult congenital heart disease and pulmonary hypertension service (Royal Brompton Hospital, London, UK) at the age of 17 years. She initially presented at the age of 4 years with increasing shortness of breath. At that time, there was differential cyanosis with clubbing and lower oxygen saturations in the toes (82%) compared with her fingers (95%). On echocardiography there was evidence of severe pulmonary hypertension and a large patent ductus arteriosus (PDA) with low velocity bidirectional shunting. She underwent cardiac catheterisation at the time which showed a pulmonary arterial pressure equal to that of the aorta.
Project description:BackgroundInfectious endarteritis associated with patent ductus arteriosus (PDA-IE) is an uncommon complication in the era of antibiotics. However, it implies a clinical challenge in patients with a fever of undetermined origin; Two-dimensional transthoracic echocardiography (TTE) performs a fundamental role in diagnosis and follow-up.MethodsA retrospective analysis was then made of the data of all patients admitted at our center with PDA-IE within 15 years, and a review of the literature regarding diagnosis, TTE findings, and treatment was performed.ResultsA total of 17 patients were identified. The mean age was 17.8 years. The TTE done in all patients confirmed the PDA and PA vegetations diagnosis; in five cases, one vegetation was present; in three cases, two vegetations were found, and in the nine remaining cases, three or more vegetations were observed. In two-thirds of the cases, the vegetations' size was 3 to 28 mm, and the principal morphology was filiform. In all cases, at least one of the vegetations was developed in the DA's lateral wall. Pulmonary valve (PV) was affected in 41% of the patients and caused low to moderate valvular regurgitation. Pulmonary embolism was present in 7 cases and pulmonary aneurism in one case.ConclusionsDecreased incidence of PDA-IE has been currently achieved with early antibiotic therapy. However, today, this complication carries a significant risk of valve damage and other cardiac structures' involvement.
Project description:We describe the case of a 32-year-old man with history of patent ductus arteriosus (PDA) closed with an Amplatzer device 12 years earlier. Imaging investigations revealed a persistent large PDA and the device migrated in the right pulmonary artery. A new transcatheter PDA occlusion was attempted with optimal post-procedural results. (Level of Difficulty: Advanced.).
Project description:While cyclooxygenase inhibitors have been the most common medications used to facilitate earlier closure of patent ductus arteriosus in preterm infants, adverse effects and low efficacy in extremely low gestational age neonates (ELGANs) have highlighted a need for alternative options. Combination therapy with acetaminophen and ibuprofen is a novel strategy for PDA treatment in ELGANs, as it may facilitate higher ductal closure rates via additive action on two separate pathways inhibiting prostaglandin production. Initial small observational studies and pilot randomized clinical trials indicate potentially higher efficacy of the combination regime to induce ductal closure in comparison to treatment with ibuprofen alone. In this review, we examine the potential clinical impact of treatment failure in ELGANs with significant PDA, highlight the biological rationale in support of studying combination therapy, and review the randomized and non-randomized studies to date. With the rising number of ELGANs receiving neonatal intensive care, who are vulnerable to PDA-related morbidities, there is an urgent need for adequately powered clinical trials to systematically investigate the efficacy and safety of combination therapy for PDA treatment.
Project description:ObjectiveTo identify single-nucleotide polymorphisms (SNPs) in specific candidate genes associated with patent ductus arteriosus in term infants.Study designWe conducted an initial family-based, candidate gene study to analyze genotype data from DNA samples obtained from 171 term infants and their parents enrolled in the National Birth Defects Prevention Study (NBDPS). We performed transmission disequilibrium testing (TDT) using a panel of 55 SNPs in 17 genes. Replication of SNPs with P < .1 in the NBDPS trios was performed with a case-control strategy in an independent population.ResultsTDT analysis of the NBDPS trios resulted in 6 SNPs reaching the predetermined cutoff (P < .1) to be included in the replication study. These 6 SNPs were genotyped in the independent case-control population. A SNP in TGFBR2 was found to be associated with term patent ductus arteriosus in both populations after we corrected for multiple comparisons. (rs934328, TDT P = 2 × 10(-4), case-control P = 6.6 × 10(-5)).ConclusionsThese findings confirm the importance of the transforming growth factor-beta pathway in the closure of the term ductus arteriosus and may suggest new therapeutic targets.