Clinical and hemodynamic effect of endothelin receptor antagonists in Eisenmenger Syndrome.

INTRODUCTION: Endothelin receptor antagonists (ERAs) are widely accepted as a specific treatment for pulmonary arterial hypertension. Unfortunately, consensus and recommendations are lacking for the treatment of patients who suffer from pulmonary arterial hypertension and congenital heart disease, including Eisenmenger syndrome. OBJECTIVE: This meta-analysis aimed to compare the effect of ERA on patients with Eisenmenger syndrome. METHODS: Electronic search on PubMed (MEDLINE), EBSCO, EuropePMC, Clinicaltrials.gov, and Google Scholar was done. Studies involving the use of ERAs on Eisenmenger syndrome patients were included. There were 18 studies included. The primary outcome of interest was the 6-min walking test distance before and after exposure to ERA. RESULTS: There were 517 patients with Eisenmenger syndrome. The subjects had Eisenmenger syndrome secondary to congenital heart disorders, with WHO functional Class ranging from Class I-IV. The follow-up ranges from a mean of 4-60 months. Seventeen studies reported a statistically significant difference between pretreatment and the posttreatment result of 6-min walking test distance. Pooled mean difference comparing pre and posttreatment values yielded an increase of 55.24 m (42.15, 68.33) P < 0.001; moderate heterogeneity I 2 51% P = 0.008. Pooled mean pulmonary vascular resistance index difference comparing pre and posttreatment values yielded a decrease of 4.76 woods unit (-6.86, -2.66), P < 0.001 favoring posttreatment; low heterogeneity I2 0%, P = 0.82. Pooled mean mean pulmonary arterial pressure difference comparing pre and posttreatment values yielded a decrease of 5.40 mmHg (-7.53, -3.28), P < 0.001 favoring posttreatment, low heterogeneity I 2 0%, P = 0.65. CONCLUSION: Implementation of ERA in Eisenmenger improves 6-min walking distance and pulmonary vascular pressure indices. Earlier administration of ERA might be beneficial, further studies are needed to assess mortality benefit of this agent.

Concurrent right atrial isomerism, complete atrioventricular septal defect, and single ventricle in an L-transposition of great arteries patient complicated by brain abscess.

We present a case of an 11-year-old Indonesian female who was referred to our facility after surgical excision of brain abscess. The patient has been previously diagnosed with right atrial isomerism, complete atrioventricular septal defect, and L-Transposition of great arteries. Multiple staged surgeries are required for the management of this condition.

Infective endocarditis due to Burkholderia cepacia in a neonate: a case report.

BACKGROUND: Burkholderia is a pathogen that is rarely seen in clinical cases. However, this organism is being found more commonly in hospitals. CASE PRESENTATION: A female Indonesian newborn was referred to our neonatal intensive care unit because of respiratory distress. The newborn had been delivered the previous night via cesarean section. A physical examination revealed intercostal retractions and weak cry. The newborn's gestational history was preterm, small for gestational age, and preterm premature ruptured membrane for 14 hours. Continuous positive airway pressure was administered. A multiple-antibiotic regimen consisting of ampicillin-sulbactam, gentamicin, meropenem, and ceftriaxone was initiated. Insertion of a central catheter was performed. The patient's laboratory results were low blood albumin and globulin, anemia, and leukopenia. A blood culture revealed Burkholderia cepacia that was resistant to multiple antimicrobial agents. A chest x-ray showed infiltrate on both lung fields. Echocardiography showed two vegetations on the tricuspid valve. CONCLUSIONS: B. cepacia is a rare cause of infective endocarditis. With its capability to colonize water and grow on microbicides, the presence of B. cepacia in a patient's blood warrants further investigation in institutions providing care. This might not be the first publication on this topic.