Current research suggests that a small pulmonary artery can cause adverse events and reduce exercise capacity after the Fontan procedure. This study aimed to evaluate the impact of pulmonary artery size on early haemodynamic and laboratory variables after total cavopulmonary connection.

We reviewed all patients who underwent staged Fontan between 2012 and 2022. Pulmonary artery index before bidirectional cavopulmonary shunt and before total cavopulmonary connection was calculated according to Nakata and colleagues. We sought to analyse the impact of the pulmonary artery index on early haemodynamic and laboratory variables, including pulmonary artery pressure and mean arterial pressure 12 hours after extubation and lactate levels 6 hours after extubation.

A total of 263 patients were included. Median age and weight at total cavopulmonary connection were 2.2 (interquartile ranges: 1.8–2.7) years and 11.7 (interquartile range: 10.7–13.3) kg, respectively. Before that, all patients underwent bidirectional cavopulmonary shunt at a median age of 4.1 (interquartile range: 3.2–5.8) months. In the multivariable analysis, pre-bidirectional cavopulmonary shunt pulmonary artery index (p = 0.016, odds ratio 0.993), with a cut-off value of 154 mm2/m2 was an independent risk factor for a higher pulmonary artery pressure (> 17 mmHg). No variable was identified as a significant risk factor for lower mean arterial pressure (< 57 mmHg). Regarding lactate levels (> 4.5 mg/dl), pre-bidirectional cavopulmonary shunt right pulmonary artery index (p < 0.001, odds ratio 0.983), with a cut-off value of 70 mm2/m2 was identified as an independent risk factor.

In patients with staged Fontan palliation, a small pulmonary artery size before bidirectional cavopulmonary shunt and total cavopulmonary connection was a determinant factor associated with unfavourable early postoperative haemodynamics after total cavopulmonary connection.

Due to the high postoperative mortality, tools for an adaequate risk stratification are important to identify high-risk patients undergoing the Norwood procedure. As a marker of ventricular wall stress, NT-proBNP might be of particular interest in these children.

This study evaluated whether NT-proBNP’s age-adjusted z-score (“zlog-NT-proBNP”) predicts outcomes after stage I Norwood procedure.

Patients who underwent the Norwood procedure between 1 January 2011 and 31 December 2022, with perioperative NT-proBNP measurements available were enrolled. Since reference intervals of NT-proBNP are highly age-dependent, age-adjusted zlog-NT-proBNP was used. Serial zlog-NT-proBNP values were analysed to predict the occurrence of major adverse cardiovascular events after the Norwood procedure. Major adverse cardiovascular events was defined as death, resuscitation, or mechanical circulatory support. Secondary endpoints were re-operation and re-intervention for shunt.

A total of 139 patients underwent the Norwood procedure and had at least one NT-proBNP measurement available. Preoperative zlog-NT-proBNP measurements (median 3.7, interquartile range 3.1–4.19) showed no association with the occurrence of major adverse cardiovascular events or mortality. Zlog-NT-proBNP early after ICU admission (3.2, interquartile range 2.4–3.8) was predictive of mortality but showed no association with the occurrence of major adverse cardiovascular events. Zlog-NT-proBNP before ICU discharge (3.2, interquartile range 2.8–3.8) was significantly associated with the occurrence of both major adverse cardiovascular events (hazard ratio 1.83, 95% confidence interval 1.25–2.67, P = 0.002) and death (hazard ratio 2.1, 95% CI 1.4–3.2, P < 0.001).

High zlog-NT-proBNP levels after the Norwood surgery were strongly associated with the occurrence of major adverse cardiovascular events and death. Therefore, zlog-NT-proBNP has the potential to identify high-risk patients before life-threatening complications occur.

This study aimed to evaluate veno-venous collaterals between bidirectional cavopulmonary shunt and total cavopulmonary connection.

Patients who underwent staged total cavopulmonary connection between 1995 and 2022 were reviewed. Veno-venous collaterals between bidirectional cavopulmonary shunt and total cavopulmonary connection were depicted using angiograms. The prevalence of veno-venous collaterals, the risks for the development of veno-venous collaterals, and the impact of veno-venous collaterals on outcomes were analysed.

In total, 586 patients were included. Veno-venous collaterals were found in 72 (12.3%) patients. Majority of veno-venous collaterals originated from the superior caval vein and drained into the inferior caval vein. Before bidirectional cavopulmonary shunt, mean pulmonary artery pressure (16.3 vs. 14.5 mmHg, p = 0.018), and trans-pulmonary gradient (9.5 vs. 8.0 mmHg, p = 0.030) were higher in patients with veno-venous collaterals compared to those without. Veno-venous collaterals intervention was performed in 32 (5.5%) patients, in a median of 29 (16–152) days after bidirectional cavopulmonary shunt. Before total cavopulmonary connection, pulmonary artery pressure (10.3 vs. 9.4 mmHg, p = 0.015) and ventricular end-diastolic pressure (8.4 vs. 7.6 mmHg, p = 0.035) were higher, and arterial oxygen saturation (SaO2, 80.6 vs. 82.6 %, p = 0.018) was lower in patients with veno-venous collaterals compared to those without. More palliations before total cavopulmonary connection (p < 0.001, odds ratio: 1.689) were an independent risk for the development of veno-venous collaterals. Veno-venous collaterals did not affect survival after total cavopulmonary connection (92.8 vs. 92.7% at 10 years, p = 0.600).

The prevalence of veno-venous collaterals between bidirectional cavopulmonary shunt and total cavopulmonary connection was 12%. Veno-venous collaterals may be induced by the elevated pulmonary artery pressure and trans-pulmonary gradient, and also by more previous palliations. However, they had no impact on clinical outcomes following total cavopulmonary connection.

This study aims to assess the surgical outcome of borderline hypoplastic left ventricle before and after the induction of the left ventricle rehabilitation strategy.

A retrospective review investigated patients with borderline hypoplastic left ventricle who underwent surgical intervention between 2012 and 2022. The patient cohort was stratified into two groups based on the initiation of left ventricle rehabilitation: an early-era group (E group, 2012–2017) and a late-era group (L group, 2018–2022). Left ventricle rehabilitation was defined as palliation combined with other procedures aimed at promoting left ventricular growth such as restriction of atrial septal defect, relief of inflow/outflow obstructive lesions, and resection of endocardial fibroelastosis.

A total of 58 patients were included. Primary diagnosis included 12 hypoplastic left heart syndromes, 11 critical aortic valve stenosis, and others. A total of 9 patients underwent left ventricle rehabilitation, 8 of whom underwent restriction of atrial septal defect. As for clinical outcomes, 9 of 23 patients achieved biventricular repair in the E group, whereas in the L group, 27 of 35 patients achieved biventricular repair (39% vs. 77%, p = 0.004). Mortality did not differ statistically between the two groups (log-rank test p = 0.182). As for the changes after left ventricle rehabilitation, left ventricular growth was observed in 8 of 9 patients. The left ventricular end-diastolic volume index (from 11.4 to 30.1 ml/m2, p = 0.017) and left ventricular apex-to-right ventricular apex ratio (from 86 to 106 %, p = 0.014) significantly increased after left ventricle rehabilitation.

The introduction of the left ventricle rehabilitation strategy resulted in an increased proportion of patients achieving biventricular repair without a concomitant increase in mortality. Left ventricle rehabilitation was associated with enhanced left ventricular growth and the formation of a well-defined left ventricle apex. Our study underscores the significance of left ventricle rehabilitation strategies facilitating successful biventricular repair. The data suggest establishing restrictive atrial communication may be a key factor in promoting left ventricular growth.

Failing Fontan poses a significant clinical challenge. This study aims to improve patients’ outcomes by comprehensively understanding the incidence, pathophysiology, risk factors, and treatment of failing Fontan after total cavopulmonary connection.

We performed a retrospective analysis of patients who underwent total cavopulmonary connection at the German Heart Center Munich between 1994 and 2022. The onset of failing Fontan was defined as: protein-losing enteropathy, plastic bronchitis, NYHA class IV, NYHA class III for > one year, unscheduled hospital admissions for heart failure symptoms, and evaluation for heart transplantation.

Among 634 patients, 76 patients presented with failing Fontan, and the incidence was 1.48 per 100 patient-years. Manifestations included protein-losing enteropathy (n = 34), hospital readmission (n = 28), NYHA III (n = 18), plastic bronchitis (n = 16), evaluation for heart transplantation (n = 14), and NYHA IV (n = 4). Risk factors for the onset of failing Fontan were dominant right ventricle (p = 0.010) and higher pulmonary artery pressure before total cavopulmonary connection (p = 0.004). A total of 72 interventions were performed in 59 patients, including balloon dilatation/stent implantation in the total cavopulmonary connection pathway (n = 49) and embolization of collaterals (n = 24). Heart transplantation was performed in four patients. The survival after the onset of Fontan failure was 77% at 10 years. Patients with failing Fontan revealed significantly higher zlog-NT-proBNP levels after onset compared to those without (p = 0.021)

The incidence of Fontan failure was 1.5 per 100 patient years. Dominant right ventricle and higher pulmonary artery pressure before total cavopulmonary connection were significant risks for the onset of failing Fontan. Zlog-NT-proBNP is only a late marker of Fontan failure.

We have left antegrade pulmonary blood flow (APBF) at bidirectional cavopulmonary shunt (BCPS) only for high-risk patients. This study evaluates the indication and the outcomes of patients with APBF, compared to those without APBF.

Patients with APBF after BCPS were identified among patients who underwent BCPS between 1997 and 2022. Outcomes of patients with and without APBF after BCPS were compared.

APBF was open in 38 (8.2%) of 461 patients. Median age (7.7 versus 6.3 months, p = 0.55) and weight (5.6 versus 6.1 kg, p = 0.75) at BCPS were similar in both groups. The most frequent indication for APBF was high pulmonary artery pressure (PAP) in 14 patients, followed by hypoxaemia in 10, and hypoplastic left pulmonary artery in 8. The source of APBF was the pulmonary trunk in 10 patients and the aortopulmonary shunt in 28. Median hospital stay after BCPS was longer (22 versus 14 days, p = 0.018) and hospital mortality was higher (10.5 versus 2.1%, p = 0.003) in patients with APBF compared to those without APBF. However, 448 hospital survivors showed similar survival after discharge following BCPS (p = 0.224). Survival after total cavopulmonary connection (TCPC) was similar between the groups (p = 0.753), although patients with APBF were older at TCPC compared to those without (3.9 versus 2.2 years, p = 0.010).

APBF was left in 8% following BCPS in high-risk patients, mainly due to preoperative high PAP. Hospital survivors after BCPS demonstrated comparable survival in patients with and without APBF. Adding APBF at BCPS might be a useful option for high-risk patients.

Delirium is a potential emergency with serious consequences. Little attention has been paid to residents of nursing homes, although they are at extreme risk for developing delirium. Health Care Professionals (HCPs) such as nurses and general practitioners are assumed to know little about delirium in nursing homes.

The German project DeliA (delirium in nursing homes) comprises three sub- studies and two reviews. The sub-studies have the following objectives: (1) to determine the prevalence of delirium and its sub-types in German nursing homes; (2) to describe and assess the quality of delirium care practices (prevention, diagnosis, therapy) of HCPs in nursing homes; and (3) to develop a Technology Enhanced Learning (TEL) to increase the delirium- specific knowledge of HCPs in nursing homes. The reviews aim to (a) summarize the prevalence of delirium reported in international studies and (b) to find out how, why and under what context education for HCPs in nursing homes works.

A systematic review of the reported prevalence of delirium in nursing homes will be conducted (a). The prevalence study (1) will assess delirium and its proposed associated factors in at least 50 nursing homes using validated measurements. Medication schedules of participating residents will be analyzed to determine potential for delirium. To describe current practice, process-oriented semi-structured guided interviews will be conducted with 30 representatives of the (nursing home) medical service and the nursing service of nursing homes (2). As a theoretical basis for the TEL, a realist review will be conducted to understand the active ingredients of educational interventions and to develop an initial program theory (b). The curriculum for the proposed TEL will be developed based on a synthesis of existing curricula and evaluated by Delphi experts for relevance, comprehensiveness, and content. A final feasibility study will assess the potential increase in knowledge about delirium among HCPs (n = 50) in nursing homes (3).

It is expected that the project and the dissemination of its findings will raise awareness among HCPs and the public about delirium in nursing homes. The developed TEL and its underlying program theory will be further tested.