Non-invasive assessment of severe liver fibrosis in patients with Fontan-associated liver disease: The VALDIG-EASL FONLIVER cohort.

BACKGROUND & AIMS: Fontan-type surgery is used as a palliation for congenital heart disease with univentricular physiology but may, in the long term, lead to advanced chronic liver disease. This study assessed the accuracy of conventional non-invasive models in assessing liver fibrosis and introduces a new risk score employing non-invasive tools. METHODS: A prospective, cross-sectional, observational study was conducted across five European centers and encompassing all consecutive adult patients with Fontan circulation, liver biopsy and non-invasive tests (elastography, APRI, FIB-4, Fibrosis score, Doha, GUCI, and AAR). The primary outcome was the identification of severe liver fibrosis on biopsy. Multivariable logistic regression identified non-invasive predictors of severe fibrosis, leading to the development and internal validation of a new scoring model named the FonLiver risk score. RESULTS: In total, 217 patients (mean [standard deviation] age, 27.9 [8.9] years; 50.7% males) were included. Severe liver fibrosis was present in 47.9% (95% CI 41.2%-54.5%) and correlated with a lower functional class, protein-losing enteropathy, and compromised cardiopulmonary and systemic hemodynamics. The final FonLiver risk score incorporated liver stiffness measurement using transient elastography and platelet count and demonstrated strong discrimination and calibration (area under the receiver operating curve [AUROC] of 0.81). The FonLiver risk score outperformed conventional prediction models (APRI, FIB-4, Fibrosis score, Doha, GUCI, and AAR), which all exhibited worse performance in our cohort (AUROC < 0.70 for all). CONCLUSION: Severe liver fibrosis is prevalent in adults following Fontan-type palliation and can be effectively estimated using with the novel FonLiver risk score. This scoring system can be easily incorporated into the routine assessment of patients with Fontan circulation.

Point-of-Care Echocardiography: A Useful Tool for Assessing Complex Arrhythmias in the Pediatric Intensive Care Unit.

OBJECTIVE: To demonstrate that the analysis of the atrioventricular, intraventricular, and interventricular asynchrony by point-of-care ultrasound (POCUS) could be an alternative tool for assessing complex arrhythmias in pediatric patients with congenital heart diseases, mainly when an epicardial register or electrophysiology study is not available. DESIGN: Descriptive, retrospective case series study. SETTING: The pediatric intensive care unit of a university-affiliated tertiary hospital in Spain. PATIENTS: The authors included 12 patients with congenital heart disease younger than 18 years admitted to the authors' pediatric intensive care unit (PICU) from January 2018 to December 2019, with complex arrhythmias after surgery, managed by performing a bedside echocardiography when an electrophysiology test or epicardial auriculogram was unavailable. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The authors included a total of 14 complex arrhythmias in 12 postsurgical patients in whom a comprehensive transthoracic electrocardiogram was not conclusive. The presence of atrioventricular, intraventricular, or interventricular asynchrony was assessed in standard echo views by performing M-mode and Doppler mitral inflow analysis, checking the heart motion. The final POCUS diagnoses were atrial flutter (n = 5), postsurgical atrioventricular block (n = 4), asynchrony induced by pacemaker (n = 2), junctional ectopic tachycardia (n = 1), nodal rhythm plus ventricular extrasystole (n = 1), and supraventricular tachycardia (n = 1). In all patients, regardless of the type of arrhythmia, detecting motion asynchrony was crucial for making the correct diagnosis. Offline cardiologist analysis of the POCUS scans showed full agreement. CONCLUSIONS: POCUS is a useful tool for initial diagnosis and management of complex arrhythmias in the PICU, mainly when epicardial auriculogram or electrophysiology studies are unavailable.

Prevalence, features and predictive factors of liver nodules in Fontan surgery patients: The VALDIG Fonliver prospective cohort.

BACKGROUND & AIMS: Fontan surgery is used to treat a variety of congenital heart malformations, and may lead to advanced chronic liver disease in the long-term. This study examines the prevalence, characteristics and predictors of liver nodules in patients following Fontan surgery. METHODS: This was a prospective, cross-sectional, observational study conducted at 8 European centres. Consecutive patients who had undergone Fontan surgery underwent blood tests, abdominal ultrasonography (US), transient elastography (Fibroscan®), echocardiography, haemodynamic assessments, and abdominal MRI/CT scan. The primary outcome measure was liver nodules detected in the MRI/CT scan. Predictors of liver nodules were identified by multivariate logistic regression. RESULTS: One hundred and fifty-two patients were enrolled (mean age 27.3 years). The mean time elapsed from surgery to inclusion was 18.3 years. Liver nodule prevalences were 29.6% (95% CI 23-37%) on US and 47.7% (95% CI 39-56%) on MRI/CT. Nodules were usually hyperechoic (76.5%), round-shaped (>80%), hyperenhancing in the arterial phase (92%) and located in the liver periphery (75%). The sensitivity and specificity of US were 50% (95% CI 38-62%) and 85.3% (95% CI 75-92%), respectively. Inter-imaging test agreement was low (adjusted kappa: 0.34). In the multivariate analysis, time since surgery >10 years was the single independent predictor of liver nodules (odds ratio 4.18; p = 0.040). Hepatocellular carcinoma was histologically diagnosed in 2 of the 8 patients with hypervascular liver nodules displaying washout. CONCLUSION: While liver nodules are frequent in Fontan patients, they may go unnoticed in US. Liver nodules are usually hyperechoic, hypervascular and predominantly peripheral. This population is at risk of hepatocellular carcinoma, the diagnosis of which requires confirmatory biopsy. LAY SUMMARY: Fontan surgery is the standard of care for many patients with univentricular congenital cardiopathies. Recent advances have improved the survival of Fontan patients, and nowadays most of them reach adulthood. In this setting, Fontan-associated liver disease (FALD) is increasingly recognised, and has become a significant prognostic factor. Liver nodules are considered a component of FALD yet their prevalence, imaging features and predictors have hardly been evaluated. In this study, we observed that liver nodules are frequent, typically hyperechoic, hypervascular and predominantly peripheral in patients with FALD. This population is at risk of hepatocellular carcinoma, the diagnosis of which must be confirmed by biopsy.

Implantable LENUS pro pump for treprostinil infusion in three pediatric patients.

BACKGROUND: Prostanoid treatment in patients with severe pulmonary arterial hypertension (PAH) has been proven safe and effective. Subcutaneous administration of treprostinil has side effects, which limits their use and acceptance. An implantable pump for continuous intravenous treprostinil infusion has been recently approved. We describe our experience with the implantable pump in three pediatric patients. DESCRIPTION OF CASES: The LENUS pro pump was implanted in three adolescents with severe PAH, who were treated with tadalafil, ambrisentan, and subcutaneous treprostinil. The indication of the Lenus pro pump implantation was the local side effects of subcutaneous treprostinil (pain, inflammation, and local infection) that were not well tolerated and that severely decreased their quality of life. The pump was surgically implanted under general anesthesia.One patient, in functional class IV, suffered postoperative hemodynamic instability and small pneumothorax, requiring an increase in treprostinil dose up to 85 ng/kg/min and a decrease 9 days after the pump implantation. The second patient who was discharged 4 days after surgery with treprostinil at 60 ng/kg/min reported improvement in his quality of life, but the dose requirement increased up to 92 ng/kg/min. After a 21-month follow-up, this patient received a lung transplant. The third patient presented a hematoma at the pump site with no other complications and had a follow-up of 9 months with an improvement in her quality of life. COMMENTS: Implantable pumps for continuous parenteral prostanoid infusion in pediatric patients are an alternative to external pumps, especially when familiar psychological or psychomotor issues hinder the use of external pumps.

Experiencia con la prótesis de pericardio bovino Carpentier-Edwards® en posición pulmonar en pacientes con cardiopatías congénitas / Experience with Carpentier Edwards® bovine pericardium implantations in pulmonary position in patients with congenital heart diseases

Introducción. El número de pacientes con cardiopatías complejas corregidas en la infancia que necesitan una sustitución valvular pulmonar para restaurar la competencia o solucionar la estenosis del tracto de salida de ventrículo derecho ha aumentado en los últimos años. El injerto ideal continúa siendo motivo de controversia. En el servicio de cardiocirugía del Hospital Ramón y Cajal de España, se comenzó a utilizar prótesis de pericardio bovina de Carpentier-Edwards siendo el objetivo de este estudio su evaluación a corto y medio plazo. Materiales y Métodos. Entre enero de 2004 y mayo de 2010 fueron intervenidos 42 pacientes para sustitución valvular pulmonar mediante prótesis de pericardio bovino. El estudio fue ambispectivo con prospección durante los dos últimos años. Resultados. La mediana de la edad fue de 20,96 años (amplitud intercuartil 10,5 años). El número medio de cirugías previas fue de 1,9±0,9 siendo el tiempo medio entre la última cirugía y la implantación de la prótesis de 17,2±7 años. Las indicaciones quirúrgicas fueron: disfunción del ventrículo derecho (45%), su dilatación progresiva (38%), arritmias ventriculares (14%) y síncopes (3%). La mortalidad precoz de causa cardiológica fue de dos pacientes. El tiempo medio de seguimiento fue de 2,1±1,4 años (rango entre 0,1 y 6,3 años) estando el 94,3% de ellos en clase funcional I de la New York Heart Association. El gradiente Doppler pico transprotésico por ecocardiografía fue de 18,5±17 mm Hg. No se observaron cambios degenerativos ni ningún tipo de deterioro estructural de la prótesis. Conclusiones. La prótesis de pericardio bovino en posición pulmonar presenta excelentes resultados a corto y medio plazo. Sin embargo, es necesario un seguimiento mayor para confirmar lo resultados iniciales respecto a su durabilidad y hemodinamia a largo plazo...

Introduction. In recent years the number of patients with complex congenital heart disease previously corrected in infancy who need a pulmonary valve replacement has increased dramatically. The ideal substitute remains a source of dispute. Nowadays, in the unit of heart surgery of the Hospital Ramon y Cajal in Spain, its being implanting in this position the bovine pericardium Carpentier-Edwards prosthesis. The aim of the study is its short and mediumtermassessment. Material and methods. Between January 2004 and May of 2010, 42 patients have been operated for pulmonary valve replacement with pericardium prosthesis. The study was ambispective, being prospective in its last two years. Results. The median age of the patients was 20.96 years (interquartile 10.5 years). The mean number of surgeries prior to the pulmonary valve replacement was 1.9±0.9, being the mean time between the “corrective” surgery and the prosthetic implantation 17.2±7 years. The main indications for this surgery were: right ventricle dysfunction (45%), progressive dilation of the same ventricle (38%), ventricular arrhythmias (14%) and syncopes (3%). Two patients died in the immediate postoperative due to cardiological causes. The mean follow-up time has been 2.1±1.4 years (0.1-6.3). The 94.3 % of the surviving patients are in functional class I, according to the New York Health Association. The peak Doppler transprosthetic gradient determined by echocardiography was 18.5±17 mm Hg. In the echocardiograpic follow-up there have been neither degenerative changes nor any type of structural deterioration of the prosthesis. Conclusions. The bovine pericardium prosthesis in pulmonary position presents excellent results in the short and medium-term. However, it is necessary a longer follow-up to confirm our initial results regarding its durability and haemodynamics long-term...