Management of right ventricular outfl ow tract obstruction: Evolution to revolution

Percutaneous pulmonary valves have opened up new avenues of treatment in patients requiring pulmonary valve replacement. The management of tetralogy of Fallot demonstrated the evolution of treatment: from palliativecare to modern day early complete surgical repair. Use of trans-annular patches to treat right ventricle outfl ow obstruction gave rise to signifi cant pulmonary regurgitation. Clinicians considered this a benign condition until,three decades later patients started dying unexpectedly. Surgical pulmonary valve replacement was the only treatment, but these showed dysfunction after some years. Clear guidelines for intervention do not exist. Current guidelines recommend treatment at upper limits of tolerance. Arguments for earlier intervention are presented, but none of the guidelines have been scientifically validated

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

Report of the Coding Committee of the Association for European Paediatric Cardiology

IN 1999 AND EARLY 2000, THE ASSOCIATION FOR European Paediatric Cardiology published the European Paediatric Cardiac Code as independent but linked Short and Long Lists, containing 650 and 3876 primary terms respectively. The historical background and rationale for development of this coding system has been previously detailed, but essentially it followed a series of meeting of the coding committee of the Association between 1997-1999, during which a pre-existing Long List was adopted and then used to create the condensed Short List. The system was published as the recommended standard coding system for use across Europe, covering the diagnosis and therapy of children with congenital and acquired cardiac disease. The scope of the lists was to encompass the needs of all those involved with such patients, from the fetal cardiologist through to the specialist in adult congenital heart disease; and from the general paediatric cardiologist and cardiac surgeon, to those specialising in transcatheter interventions, paediatric electrophysiology, and paediatric echocardiographers. In addition, the code was crossmapped to the 9th and 10th revisions of the International Classification of Diseases ("ICD-9” and "ICD-10”) provided by the World Health Organisation in order to facilitate returns to central government, a requirement in most countries. In so doing, it was hoped to address the concerns of many centres that such information submitted by professional coding staff was often inaccurate due to the complex nature of congenital cardiac disease, together with the limited scope and vague terminology of the International listing

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

Preterm birth is associated with adverse cardiac remodeling and worse outcomes in patients with a functional single right ventricle

ObjectiveTo assess the effects of preterm birth on cardiac structure and function and transplant-free survival in patients with hypoplastic left heart syndrome and associated anomalies throughout the staged palliation process.Study designData from the Single Ventricle Reconstruction trial were used to assess the impact of prematurity on echocardiographic measures at birth, Norwood, Stage II, and 14 months in 549 patients with a single functional right ventricle. Medical history was recorded once a year using medical records or telephone interviews. Cox regression models were applied to analyze transplant-free survival to age 6 years. Causal mediation analysis was performed to estimate the mediating effect of birth weight within this relationship.ResultsOf the 549 participants, 64 (11.7%) were born preterm. Preterm-born participants had lower indexed right ventricle end-diastolic volumes at birth but higher volumes than term-born participants by age 14 months. Preterm-born participants had an increased risk of death or heart transplantation from birth to age 6 years, with an almost linear increase in the observed risk as gestational age decreased below 37 weeks. Of the total effect of preterm birth on transplant-free survival, 27.3% (95% CI 2.5-59.0%) was mediated through birth weight.ConclusionsPreterm birth is associated with adverse right ventricle remodeling and worse transplant-free survival throughout the palliation process, in part independently of low birth weight. Further investigation into this vulnerable group may allow development of strategies that mitigate the impact of prematurity on outcomes in patients with hypoplastic left heart syndrome.Developmen