Renal abscess after the Fontan procedure: a case report

<p>Abstract</p> <p>Introduction</p> <p>The Fontan procedure is an intervention that helps to correct single ventricle physiology. There are many known long-term complications of 'Fontan physiology'. However, the occurrence of renal abscess in such patients has not yet been reported in the literature. The first generation of adults has now undergone the procedure and it is necessary to be aware of the long-term outcomes and complications associated with it.</p> <p>Case presentation</p> <p>We report the case of a 22-year-old South Indian man who had developed a staphylococcal renal abscess against a background of xanthogranulomatous pyelonephritis, nine years after Fontan surgery. He presented to our hospital with a high-grade fever of 25-days duration but with no other symptoms. Physical examination identified costovertebral angle tenderness and pedal edema. An ultrasound scan revealed a mass in his left kidney. The results of a computed tomography scan were consistent with a renal abscess. Despite treatment with the appropriate parenteral antibiotics, there was no change in the size of the abscess and a left nephrectomy was performed as a curative procedure.</p> <p>Conclusions</p> <p>The learning points here are manifold. It is important to be aware of the possibility of renal abscess in a post-procedural patient. The early diagnosis of a septic focus in the kidneymay help to prevent the rare outcome of nephrectomy.</p

The use of Macitentan in Fontan circulation: a case report.

BACKGROUND: The Fontan circulation, a result of a palliative procedure in patients with single systemic ventricles, is defined by chronically elevated pulmonary vascular resistance. When traditional heart failure therapies fail, pharmacological agents that reduce pulmonary artery pressures may be used. These include endothelial-receptor antagonists, prostanoids and phosphodiesterase type 5 inhibitors. We report the first use of macitentan, an endothelin-receptor antagonist, in a patient with a Fontan circulation. CASE PRESENTATION: We describe the case of a 50 year old female with tricuspid atresia and transposition of the great arteries. Following complex surgery as a child, she subsequently underwent a fenestrated modified atrial pulmonary Fontan operation which was later converted to a total cavopulmonary anastomosis Fontan circulation. Due to failure of various medications to relieve her worsening symptoms, she was commenced on macitentan in April 2016. Few months later, she demonstrated a significant symptomatic improvement and associated increase in her incremental shuttle walking test distance. CONCLUSIONS: Macitentan has slower receptor dissociation kinetics compared to other endothelin-receptor antagonists, leading to enhanced pharmacological activity with promising effects in patients with pulmonary arterial hypertension. The patient we report has shown considerable improvement in exercise capacity following introduction of this medication and thus we suggest further randomised trials to establish the role of different endothelin-receptor antagonists in the management of the Fontan circulation

Critical coarctation of the aorta in selective fetal growth restriction and the role of coronary stent implantation

Introduction:Monochorionic twins are at increased risk of congenital heart defects (CHDs). Up to 26% have a birth weight <1,500 g, a CHD requiring neonatal surgery, therefore, poses particular challenges.Objective:The aim of the study was to describe pregnancy characteristics, perinatal management, and outcome of monochorionic twins diagnosed with critical coarctation of the aorta (CoA).Methods:We included monochorionic twins diagnosed with critical CoA (2010-2019) at 2 tertiary referral centers, and we systematically reviewed the literature regarding CoA in monochorionic twins.Results:Seven neonates were included. All were the smaller twin of pregnancies complicated by selective fetal growth restriction. The median gestational age at birth was 32 weeks (28-34). Birth weight of affected twins ranged as 670-1,800 g. One neonate underwent coarctectomy at the age of 1 month (2,330 g). Six underwent stent implantation, performed between day 8 and 40, followed by definitive coarctectomy between 4 and 9 months in 4. All 7 developed normally, except for 1 child with neurodevelopmental delay. Three co-twins had pulmonary stenosis, of whom 1 required balloon valvuloplasty. The literature review revealed 10 cases of CoA, all in the smaller twin. Six cases detected in the first weeks after birth were treated with prostaglandins alone, by repeated transcatheter angioplasty or by surgical repair, with good outcome in 2 out of 6.Conclusions:CoA specifically affects the smaller twin of growth discordant monochorionic twin pairs. Stent implantation is a feasible bridging therapy to surgery in these low birth weight neonates.Research into fetal development and medicin

Infective endocarditis in patients after percutaneous pulmonary valve implantation with the stent-mounted bovine jugular vein valve : clinical experience and evaluation of the modified Duke criteria

Percutaneous pulmonary valve implantation (PPVI) has proven good hemodynamic results. As infective endocarditis (IE) remains a potential complication with limited available clinical data, we reviewed our patient records to improve future strategies of IE prevention, diagnosis and treatment. Medical records of all patients diagnosed with Melody® valve IE according to the modified Duke criteria were retrospectively analyzed in three Belgian tertiary centers. 23 IE episodes in 22 out of 240 patients were identified (incidence 2.4% / patient year) with a clear male predominance (86%). Median age at IE was 17.9 years (range 8.2-45.9 years) and median time from PPVI to IE was 2.4 years (range 0.7-8 years). Streptococcal species caused 10 infections (43%), followed by Staphylococcus aureus (n = 5, 22%). In 13/23 IE episodes a possible entry-point was identified (57%). IE was classified as definite in 15 (65%) and as possible in 8 (35%) cases due to limitations of imaging. Echocardiography visualized vegetations in only 10 patients. PET-CT showed positive FDG signals in 5/7 patients (71%) and intracardiac echocardiography a vegetation in 1/1 patient (100%). Eleven cases (48%) had a hemodynamically relevant pulmonary stenosis at IE presentation. Nine early and 6 late percutaneous or surgical re-interventions were performed. No IE related deaths occurred. IE after Melody® valve PPVI is associated with a relevant need of re-interventions. Communication to patients and physicians about risk factors is essential in prevention. The modified Duke criteria underperformed in diagnosing definite IE, but inclusion of new imaging modalities might improve diagnostic performance

Validation and application of single breath-hold cine cardiac MR for ventricular function assessment in children with congenital heart disease at rest and during adenosine stress

To validate the accuracy of ventricular function analysis using a single breath-hold real-time steady sate free precession (SSFP) cine MR method and demonstrate its application during adenosine stress imaging in children with congenital heart disease. Materials and Methods. Twenty-eight subjects with congenital heart disease were studied (mean age 12.4 ± 2.3 years) with MR imaging at 1.5 T. Short-axis images covering the entirety of both ventricles were acquired at rest, with a conventional segmented SSFP cine sequence acquired over multiple breath-holds and a single breath-hold real-time SSFP sequence. Seventeen subjects were given an infusion of adenosine, and the single breath-hold real-time short-axis stack was repeated during stress. Two independent observers performed the ventricular function analysis. Data was compared between the 2 acquisition methods at rest and between the single breath-hold acquisition at rest and during adenosine stress. Results. There was good agreement between the multiple breath-hold and single breath-hold methods for measurement of end-diastolic volume (r = 0.95 and 0.96, p < .0001) and end-systolic volume (ESV) (r = 0.76 and 0.90, p < .0001) for the left and right ventricles respectively, and the left ventricular mass (r = 0.97, p < .0001). Adenosine was administered safely to all subjects. During stress there were significant changes in the heart rate and ESV, which led to a significant increase in cardiac output (mean 1.5 ± 1.0 L/min, p < .001). Conclusion. Single breath-hold real-time SSFP cine imaging is robust and accurate in assessing cardiac function in children with congenital heart disease. An application of this method is the rapid assessment of cardiac function during adenosine stress

Common Genetic Variants Contribute to Risk of Transposition of the Great Arteries

Rationale: Dextro-transposition of the great arteries (D-TGA) is a severe congenital heart defect which affects approximately 1 in 4,000 live births. While there are several reports of D-TGA patients with rare variants in individual genes, the majority of D-TGA cases remain genetically elusive. Familial recurrence patterns and the observation that most cases with D-TGA are sporadic suggest a polygenic inheritance for the disorder, yet this remains unexplored. Objective: We sought to study the role of common single nucleotide polymorphisms (SNPs) in risk for D-TGA. Methods and Results: We conducted a genome-wide association study in an international set of 1,237 patients with D-TGA and identified a genome-wide significant susceptibility locus on chromosome 3p14.3, which was subsequently replicated in an independent case-control set (rs56219800, meta-analysis P=8.6x10-10, OR=0.69 per C allele). SNP-based heritability analysis showed that 25% of variance in susceptibility to D-TGA may be explained by common variants. A genome-wide polygenic risk score derived from the discovery set was significantly associated to D-TGA in the replication set (P=4x10-5). The genome-wide significant locus (3p14.3) co-localizes with a putative regulatory element that interacts with the promoter of WNT5A, which encodes the Wnt Family Member 5A protein known for its role in cardiac development in mice. We show that this element drives reporter gene activity in the developing heart of mice and zebrafish and is bound by the developmental transcription factor TBX20. We further demonstrate that TBX20 attenuates Wnt5a expression levels in the developing mouse heart. Conclusions: This work provides support for a polygenic architecture in D-TGA and identifies a susceptibility locus on chromosome 3p14.3 near WNT5A. Genomic and functional data support a causal role of WNT5A at the locus