Portopulmonary hypertension is a rare but serious complication of portal hypertension or portosystemic shunting. Portopulmonary hypertension is an indication for liver transplantation or shunt closure. However, liver transplantation is contraindicated in patients with severe pulmonary arterial hypertension. Reported mortality rates are high in children with portopulmonary hypertension and there are scarce recommendations on its management. Our aim was to report on our real-world experience of managing portopulmonary hypertension in a specialised centre. We describe a series of 6 children with portopulmonary hypertension. Their median age at diagnosis was 13 years (range 10-15). The underlying liver conditions were cirrhosis of unknown origin (1), congenital portocaval shunts (3), biliary atresia (1), and portal vein cavernoma with surgical mesenterico-caval shunt (1). Median mean pulmonary arterial pressure was 47 mmHg (range 32-70), and median pulmonary vascular resistance was 6.6 Wood units (range 4.3-15.4). All patients except one were treated with a combination of pulmonary arterial hypertension-specific therapy (phosphodiesterase type 5 inhibitors and/or endothelin receptor antagonists and/or prostacyclin analogues). Three patients then benefited from shunt closure and the others underwent liver transplantation. Five patients showed improvement or stabilisation of pulmonary arterial hypertension with no deaths after a mean follow-up of 39 months. Based on our limited experience, early and aggressive treatment with a combination of pulmonary arterial hypertension-specific therapy significantly improves patients' haemodynamic profile and enables the performance of liver transplantation and shunt closure with satisfactory outcomes.

The population of patients with congenital heart disease is constantly growing with an increasing number of individuals reaching adulthood. A significant proportion of these children and young adults will suffer from tachyarrhythmias due to the abnormal anatomy, the hemodynamic burden, or as a sequela of surgical treatment. Depending on the underlying mechanism, arrhythmias may arise in the early postoperative period (hours to days after surgery) or in the late postoperative period (usually years after surgery). A good understanding of the electrophysiological characteristics and pathophysiological mechanisms is therefore crucial to guide the therapeutic approach. Here, we synthesize the current state of knowledge on epidemiological features, risk factors, pathophysiological insights, electrophysiological features, and therapy regarding tachyarrhythmias in children and young adults undergoing reparative surgery for congenital heart disease. The evolution and latest data on treatment options, including pharmacological therapy, ablation procedures, device therapy decision, and thromboprophylaxis, are summarized. Finally, throughout this comprehensive review, knowledge gaps and areas for future research are also identified.

OBJECTIVES: To determine factors associated with brain death in children treated with extracorporeal cardiopulmonary resuscitation (E-cardiopulmonary resuscitation). DESIGN: Retrospective database study. SETTINGS: Data reported to the Extracorporeal Life Support Organization (ELSO), 2017–2021. PATIENTS: Children supported with venoarterial extracorporeal membrane oxygenation (ECMO) for E-cardiopulmonary resuscitation. INTERVENTION: None. MEASUREMENTS AND MAIN RESULTS: Data from the ELSO Registry included patient characteristics, blood gas values, support therapies, and complications. The primary outcome was brain death (i.e., death by neurologic criteria [DNC]). There were 2,209 children (≥ 29 d to < 18 yr of age) included. The reason for ECMO discontinuation was DNC in 138 patients (6%), and other criteria for death occurred in 886 patients (40%). Recovery occurred in 1,109 patients (50%), and the remaining 76 patients (4%) underwent transplantation. Fine and Gray proportional subdistribution hazards’ regression analyses were used to examine the association between variables of interest and DNC. Age greater than 1 year (p < 0.001), arterial blood carbon dioxide tension (Paco ₂) greater than 82 mm Hg (p = 0.022), baseline lactate greater than 15 mmol/L (p = 0.034), and lactate 24 hours after cannulation greater than 3.8 mmol/L (p < 0.001) were independently associated with greater hazard of subsequent DNC. In contrast, the presence of cardiac disease was associated with a lower hazard of subsequent DNC (subdistribution hazard ratio 0.57 [95% CI, 0.39–0.83] p = 0.004). CONCLUSIONS: In children undergoing E-cardiopulmonary resuscitation, older age, pre-event hypercarbia, higher before and during ECMO lactate levels are associated with DNC. Given the association of DNC with hypercarbia following cardiac arrest, the role of Paco ₂ management in E-cardiopulmonary resuscitation warrants further studies.

Pulmonary hypertension associated with congenital heart disease (PAH-CHD) represents one of the leading causes of pediatric pulmonary hypertension. Within this entity, patients can be classified into distinct subgroups, each characterized by specific clinical features, pathophysiological mechanisms, and therapeutic approaches. This review provides an update of the current PAH-CHD classification and outlines management strategies in accordance with the most recent international recommendations. Particular attention is given to the ongoing debate regarding operability in patients with open shunts and some degree of increased pulmonary vascular resistance, and key gaps in knowledge are highlighted. In PAH-CHD, treatment strategies are relatively straightforward in patients with low PVR, where shunt closure is recommended, and in Eisenmenger syndrome, where shunt patency is maintained and pulmonary vasodilators are indicated; however, the management of patients with open shunts and moderately elevated PVR remains highly challenging. Operability in this intermediate group is currently determined by invasive hemodynamic assessment, despite methodological limitations, highlighting the urgent need for reliable noninvasive markers and prospective clinical studies in late-referred patients. Emerging therapies such as sotatercept, along with the development of novel biomarkers to assess pulmonary vascular disease severity may have the potential to redefine operability criteria and expand therapeutic options.

Kingella kingaeis a gram-negative coccobacillus belonging to the HACEK group (Haemophilusspecies,Aggregatibacter actinomycetemcomitans, Cardiobacterium hominis, Eikenella corrodens, andKingellaspecies) and is a common oropharyngeal colonizer of healthy young children. Osteoarticular infection is the most commonly reported invasiveKingella kingaeinfection in children, usually presenting a mild clinical picture. However, it can also cause severe invasive infections, especially infective endocarditis, with a high complication rate. We report the case of a 6-year-old girl, with no past medical history, who presented with fulminant infective endocarditis due toKingella kingae. She received emergency venoarterial extracorporeal membrane oxygenation support, rapidly underwent cardiac surgery, and was then treated using ceftriaxone for 4 weeks as recommended by the American Heart Association. The patient's postoperative course was marked by a cerebral ischemic stroke consistent with septic embolism. She also presented with a para-aortic pseudoaneurysm that required a secondary surgical procedure, with a good postoperative result. This report illustrates a case of fulminant infective endocarditis due toKingella kingaeand responsible for two major complications. We also describe the preventive valve surgery performed to ensure the preservation of valve function and its capacity for growth.

Shock is a life-threatening condition, and its timely recognition is essential for adequate management. Pediatric patients with congenital heart disease admitted to a cardiac intensive care unit (CICU) after surgical corrections are particularly at risk of low cardiac output syndrome (LCOS) and shock. Blood lactate levels and venous oxygen saturation (ScVO₂) are usually used as shock biomarkers to monitor the efficacy of resuscitation efforts, but they are plagued by some limitations. Carbon dioxide (CO₂)-derived parameters, namely veno-arterial CO₂ difference (ΔCCO₂) and the VCO₂/VO₂ ratio, may represent a potentially valuable addition as sensitive biomarkers to assess tissue perfusion and cellular oxygenation and may represent a valuable addition in shock monitoring. These variables have been mostly studied in the adult population, with a strong association between ΔCCO₂ or VCO₂/VO₂ ratio and mortality. In children, particularly in CICU, few studies looked at these parameters, while they reported promising results on the use of CO₂-derived indices for patients’ management after cardiac surgeries. This review focuses on the physiological and pathophysiological determinants of ΔCCO₂ and VCO₂/VO₂ ratio while summarizing the actual state of knowledge on the use of CO₂-derived indices as hemodynamical markers in CICU.