Hemodynamic Profiling Using a Cardiac Index–Systemic Vascular Resistance Plot in Patients with Fontan Circulation

Background: Elevated Fontan pressure (FP) alone cannot fully predict clinical outcomes. We hypothesized thathemodynamic profiling using a cardiac index (CI)-systemic vascular resistance (SVR) plot could characterize clinicalfeatures and predict the prognosis of post-Fontan patients. Methods: We included post-Fontan patients whounderwent cardiac catheterization at age < 10 years. Patients were classified into four categories: A, CI ≥ 3, SVRindex (SVRI) ≥ 20;B, CI < 3, SVRI ≥ 20;C, CI ≥ 3, SVRI < 20;and D, CI < 3, SVRI < 20. The primary outcome wasfreedom from the combined endpoint: new onset of protein-losing enteropathy or plastic bronchitis, heart transplant,and death. Clinical and hemodynamic variables and freedom from the endpoint were compared betweenthe hemodynamic categories and outcome predictors were evaluated. Results: Eighty-three patients wereincluded. Median follow-up was 5.3 years. Category A/B/C/D consisted of 4/15/53/11 patients, respectively. Allthe patients in category A were New York Heart Association I/II and had a significantly lower pulmonary vascularresistance index (PVRI). Patients in category C had lower pulmonary/systemic blood flow. Patients in category Dhad a higher PVRI and had the poorest freedom from the endpoint (44% at 5 years). Elevated FP and category Dwere outcome predictors. Conclusions: CI-SVR plots was a novel adjunctive method for Fontan hemodynamicprofiling.

Emergency Cesarean Delivery in a Parturient with Fontan Circulation and Reduced Platelets: A Case Report

NOWADAYS,Fontan circulation always refers to the hemodynamic status after total cavo-pul-monary connection, which was first created in the 1990s. There are two modern surgeryforms, one creating a channel within the right atrium and another making an extra-cardiac channel to connect infe-rior venous cava directly to the right pulmonary artery. Parturients with Fontan circulation are at increased risk of cardiac morbidity and thrombotic complications. We pre-sented a parturient with reduced platelet status of un-known reason undergoing an emergent cesarean delivery.

Veno-Venous Extra-Corporeal Membrane Oxygenation (ECMO) in a Child with Hemoptysis and Fontan Circulation

Background: Extracorporeal membrane oxygenation is a rescue life support technique used in life threatening conditions of refractory respiratory and/or cardiac distress. Indication for extracorporeal life support in children depends on age and varies from pulmonary to cardiac pathologies. In some cases, it may be used as a bridge to a therapeutic procedure. We described here the management of respiratory failure due to hemoptysis in a child with a Fontan circulation and veno-venous extracorporeal membrane oxygenation which served as a bridge to angio-embolization. Hemoptysis can be a life threatening condition which can lead to hypovolemic shock and impaired alveolar gas exchange. The latter can result in respiratory failure and consequent asphyxia. When hemoptysis occurs in a patient with a univentricular heart and a Fontan circulation, management of this clinical situation can be challenging due to the particular physiology of the latter. Total cavopulmonary connection is a palliative surgical repair which constitutes Fontan circulation as a definitive treatment in patients with a univentricular heart. Methods: Case report description of a 16 year-old boy with a univentricular heart and a Fontan circulation who presented hemoptysis managed with a veno-venous extracorporeal membrane oxygenation (ECMO) as a bridge to angio-embolization. Results: Hemoptysis due to diffuse intra-alveolar hemorrhage from collateral circulation was successfully treated in this young patient with pulmonary vascular embolization. This allowed to wean the patient from extra-corporeal membrane oxygenation. Conclusion: Veno-venous ECMO can be life-saving as a bridge to angio-embolization for severe hemoptysis in patients with Fontan circulation. The reported case allows to underline that our multidisciplinary approach in this complex pediatric patient surely participated to improve outcome.

Ultrasound liver elastography beyond liver fibrosis assessment

Several guidelines have indicated that liver stiffness(LS)assessed by means of shear wave elastography(SWE)can safely replace liver biopsy in several clinical scenarios,particularly in patients with chronic viral hepatitis.However,an increase of LS may be due to some other clinical conditions not related to fibrosis,such as liver inflammation,acute hepatitis,obstructive cholestasis,liver congestion,infiltrative liver diseases.This review analyzes the role that SWE can play in cases of liver congestion due to right-sided heart failure,congenital heart diseases or valvular diseases.In patients with heart failure LS seems directly influenced by central venous pressure and can be used as a prognostic marker to predict cardiac events.The potential role of LS in evaluating liver disease beyond the stage of liver fibrosis has been investigated also in the hepatic sinusoidal obstruction syndrome(SOS)and in the Budd-Chiari syndrome.In the hepatic SOS,an increase of LS is observed some days before the clinical manifestations;therefore,it could allow an early diagnosis to timely start an effective treatment.Moreover,it has been reported that patients that were successfully treated showed a LS decrease,that reached pre-transplantation value within two to four weeks.It has been reported that,in patients with Budd-Chiari syndrome,LS values can be used to monitor short and long-term outcome after angioplasty.

Arrhythmic Risk in Paediatric Patients Undergoing Surgical Repair for Pulmonary Atresia with Intact Ventricular Septum

Introduction:While previous studies only focused on the arrhythmic risk associated with specific correction strategies,this study evaluates this risk in a large cohort of paediatric patients with all phenotypes of PA-IVS after surgical repair.Methods:In this single centre observational cohort study,we retrospectively evaluated 165 patients with a diagnosis of PA-IVS and we excluded those with an exclusively percutaneous treatment,patients lost or with insufficient follow-up and those affected by other arrhythmic syndromes.Surgical history and clinical outcomes were reviewed.Results:86 patients were included in the study(54 male[62.8%],mean age 16.4±6.1 years),with median follow-up from definitive repair of 12.8 years(6.4–18.9 years).They underwent three different final repairs:23 patients(26.7%)univentricular palliation,43(50%)biventricular correction,and 20(23.3%)one and a half ventricle correction.Thirteen patients(15%)developed arrhythmia:6 patients(all the subgroups)sinus node disfunction(SND);2(biventricular repair)premature ventricular complexes;2(one and a half ventricle repair)non-sustained ventricular tachycardia;1(biventricular repair)intra-atrial re-entrant tachycardia;1(one and a half ventricle repair)supraventricular tachyarrhythmia;1(biventricular repair)atrial fibrillation.Three patients with SND needed a pacemaker implantation.Only Fontan circulation showed an association with SND,while the other two groups heterogeneous types of arrhythmias.Conclusions:The low arrhythmic risk is related to surgical repair,it does not appear to be associated with native cardiomyopathy,and it appears to increase with length of follow up.Continuous follow-up in specialized centres is necessary to make an early diagnosis and to manage the potential haemodynamic impact at medium-long term.