UNASSIGNED: Congenital heart disease is a risk factor for mortality after orthotopic heart transplantation; however, the impact of preoperative circulation type and primary congenital heart disease diagnosis remains poorly delineated.
UNASSIGNED: We retrospectively reviewed patients with adult congenital heart disease aged 16 years or more who underwent orthotopic heart transplantation at our institution between 2008 and 2022. Patients were categorized as having single-ventricle or biventricular circulation. The primary end point was 5-year post-transplant survival.
UNASSIGNED: Sixty-one patients with adult congenital heart disease (single-ventricle: n = 26 [42.6%], biventricular: n = 35 [57.4%]) underwent orthotopic heart transplantation at 33.7 [interquartile range, 19.1-48.7] years. The most common congenital heart disease diagnosis was hypoplastic left heart syndrome (n = 11, 42.3%) in the single-ventricle group and congenitally corrected transposition of the great arteries (n = 7, 20.0%) in the biventricular group. Twenty-four patients previously underwent Fontan palliation. At transplant, patients in the single-ventricle group were younger (18.5 [interquartile range, 17.6-32.3] years vs 45.0 [interquartile range, 33.0-52.2] years, P < .001) and more likely to have biopsy-proven cirrhosis (46.2% vs 14.3%, P = .01) and protein-losing enteropathy (42.3% vs 2.9%, P < .001). Patients in the single-ventricle group also had longer bypass times (223.4 ± 65.3 minutes vs 187.4 ± 59.5 minutes, P = .03) and longer durations of mechanical ventilatory support (3.5 [interquartile range, 2.0-6.0] days vs 1.0 [interquartile range, 1.0-2.0] days, P < .001). Operative mortality was comparable (11.5% vs 8.6%, P = 1). Median follow-up was 6.0 [interquartile range, 2.4-10.0] years. Five-year survival was worse in the single-ventricle group (66.0% ± 10.0% vs 91.3% ± 4.8%, P = .03), as was freedom from major rejection (58.3% ± 10.2% vs 84.0% ± 6.6%, P = .02). In univariable analysis, hypoplastic left heart syndrome and Fontan circulation were risk factors for post-transplant mortality (hypoplastic left heart syndrome: hazard ratio, 5.0, P < .001; Fontan: hazard ratio, 3.5, P = .03).
UNASSIGNED: Adult patients with congenital heart disease undergoing heart transplant with single-ventricle physiology experienced a more complicated post-transplant course, with worse long-term survival and freedom from rejection. Multicenter studies are required to guide orthotopic heart transplantation decision-making in this complex cohort.

UNASSIGNED: Abnormal left ventricular (LV) rotational mechanics in biventricular hearts are associated with adverse outcomes; however, these are less well characterized for hearts with functionally single ventricles.
UNASSIGNED: The purpose of this study was to characterize ventricular rotational mechanics in the Fontan circulation and their relationship to outcomes.
UNASSIGNED: Single-center, retrospective analysis of magnetic resonance examinations for 329 Fontan patients (15 [IQR: 10-21] years) and 42 controls. The ventricular cine short-axis stack was analyzed to derive torsion metrics. Torsion calculated as the difference between apical and basal rotation normalized to ventricular length.
UNASSIGNED: Fontan patients had higher indexed ventricular end-diastolic volume (97 mL/body surface area1.3 vs 72 mL/body surface area1.3), lower ejection fraction (53% vs 60%), and lower proportion of basal clockwise rotation (62% vs 93%), apical counterclockwise rotation (77% vs 95%), and positive torsion (82% vs 100%); P < 0.001 for all. A composite outcome of death or heart transplant-listing occurred in 31 (9%) patients at a median follow-up of 3.9 years. Torsion metrics were associated with the outcome; although, on multivariate analysis only right ventricular (RV) morphology and indexed ventricular end-diastolic volume were independently associated. LVs with negative torsion, and RVs regardless of torsional pattern, had worse outcomes compared to LVs with positive torsion (P = 0.020).
UNASSIGNED: Single ventricles in a Fontan circulation exhibit abnormal torsional mechanics, which are more pronounced for RV morphology. Abnormal torsion is associated with death or need for heart transplantation. Fontan patients with LV morphology and preserved torsion exhibit the highest transplant-free survival and torsion may offer incremental prognostic data in this group of patients.

暂无摘要。

We sought to evaluate the potential clinical role of 4D-flow cardiac magnetic resonance (CMR)-derived energetics and flow parameters in a cohort of patients\' post-Fontan palliation. In patients with Fontan circulation who underwent 4D-Flow CMR, streamlines distribution was evaluated, as well a 4D-flow CMR-derived energetics parameters as kinetic energy (KE) and energy loss (EL) normalized by volume. EL/KE index as a marker of flow efficiency was also calculated. Cardiopulmonary exercise test (CPET) was also performed in a subgroup of patients. The population study included 55 patients (mean age 22 ± 11 years). The analysis of the streamlines revealed a preferential distribution of the right superior vena cava flow for the right pulmonary artery (62.5 ± 35.4%) and a mild preferential flow for the left pulmonary artery (52.3 ± 40.6%) of the inferior vena cave-pulmonary arteries (IVC-PA) conduit. Patients with heart failure (HF) presented lower IVC/PA-conduit flow (0.75 ± 0.5 vs 1.3 ± 0.5 l/min/m2, p = 0.004) and a higher mean flow-jet angle of the IVC-PA conduit (39.2 ± 22.8 vs 15.2 ± 8.9, p < 0.001) than the remaining patients. EL/KE index correlates inversely with VO2/kg/min: R: - 0.45, p = 0.01 peak, minute ventilation (VE) R: - 0.466, p < 0.01, maximal voluntary ventilation: R:0.44, p = 0.001 and positively with the physiological dead space to the tidal volume ratio (VD/VT) peak: R: 0.58, p < 0.01. From our data, lower blood flow in IVC/PA conduit and eccentric flow was associated with HF whereas higher EL/KE index was associated with reduced functional capacity and impaired lung function. Larger studies are needed to confirm our results and to further improve the prognostic role of the 4D-Flow CMR in this challenging population.

BACKGROUND: Complex CHDs are life threatening, and surgical treatment is needed for survival. Fontan palliation led to a significant increase in survival rates during the last decades. Consequently, quality of life became more essential. While a reduced quality of life compared to healthy children has been reported, detailed knowledge about individual quality of life and particular areas is lacking. Furthermore, the effect of different risk factors on quality of life is only rarely evaluated.
RESULTS: Database of the department for pediatric cardiology, Heart Center Leipzig, was screened for children after total cavopulmonary connection palliation. n = 39 patients were included in the study, the outcome after total cavopulmonary connection was analysed in detail and quality of life data were collected and analysed using the standardised questionnaire \"Pediatric quality of life inventory\", version 4.0. We compared the total health score of our patients to the mean score of healthy children in the literature. The mean follow-up time was 6.4 ± 3.2 years, the overall survival was 100% after maximal follow-up time of 11.1 years. We could not find any age or gender dependence, nor an influence of age at total cavopulmonary connection on the later quality of life. Yet, patients with three-staged surgery exhibited a worse quality of life than patients with two-staged palliation. Late complications might influence quality of life, but patient number is too small, to find universal results.
CONCLUSIONS: The total cavopulmonary connection palliation affects physical and psychological quality of life as well as cardiac health independently from age and gender. More patients and longer observation should be examined to confirm the results.

Completing 3-stage palliation for hypoplastic left heart syndrome requires significant resources. An analysis of recent data has not been performed. We aimed to determine total charges necessary to complete all 3 stages of single-ventricle palliation, including interstage encounters. We also aimed to determine overall resource utilization, including hospital days, interstage admissions, and interstage procedures. We performed a retrospective cohort study using data from the Pediatric Health Information System database between 2016 and 2021, including all patients who completed 3-stage palliation for hypoplastic left heart syndrome. We identified 199 patients who underwent 3-stage palliation of hypoplastic left heart syndrome between 2016 and 2021. Median total adjusted charges (interquartile range, IQR) over the course of 3-stage palliation were $1,475,800 ($1,028,900-2,191,700). Median adjusted charges (IQR) for stage 1, 2, and 3 hospitalizations were $604,300 ($419,000-891,400), $234,000 ($164,300-370,800), and $256,260 ($178,300-345,900), respectively. Median hospital length of stay (IQR) for stages 1, 2, and 3 was 36 (26,53), 9 (6,17), and 10 (7,14) days, respectively. Pulmonary artery stenosis was the most common admitting diagnosis for interstage hospitalizations (3.4% of hospitalizations). Cardiac catheterization (24.1% of procedures) and feeding tube placement (10.0% of procedures) were the most common principal procedures during interstage hospitalizations. Total inpatient charges incurred throughout 3-stage palliation of hypoplastic left heart syndrome are substantial and have risen since prior studies. Gastrointestinal comorbidities and feeding optimization contribute considerably to this resource utilization.

OBJECTIVE: The normal (i.e. expected) haemodynamics in adults post-Fontan remain poorly delineated. Moreover, the definitions of elevated exercise pulmonary artery (PA) and PA wedge pressure (PAWP) for this population have not been described.
RESULTS: Seventy-two adults post-Fontan undergoing exercise catheterization were categorized into abnormal (Group I, n = 59; defined as resting mean PA ≥14 mmHg and/or PAWP ≥12 mmHg, ΔPAWP/Δsystemic flow [Qs] >2 mmHg/L/min, and/or ΔPA/Δpulmonary flow >3 mmHg/L/min) and normal (Group II, n = 13) haemodynamics. Thirty-nine patients with non-cardiac dyspnoea (NCD) were included as controls. There was no difference in exercise arterial oxygen saturation (87% [81-92] vs. 89% [85-93], p = 0.29), while exercise PA pressure (27 [23-31] vs. 16 [14.5-19.5] mmHg, p < 0.001) and PAWP were higher (21 [18-28] vs. 12 [8-14] mmHg, p < 0.001) in Group I. At peak exercise, Group I had lower heart rate (97 [81-120] vs. 133 [112.5-147.5] bpm, p < 0.001) and Qs response (67.3 [43.8-93.1] vs. 105.9 (82-118.5) % predicted, p < 0.001) than Group II. Exercise superior vena cava pressures were higher (16 [14-22.5] vs. 5.5 [3-7.3] mmHg, p < 0.001) and arterial oxygen saturation lower (89% [85-93] vs. 97% [96-98], p < 0.001) in Group II compared to NCD, while no differences in PAWP, stroke volume index, heart rate, or Qs response were seen. If defined as two standard deviations above mean values for Group II, elevated PAWP and mean PA pressure post-Fontan would correspond to 20.6 mmHg and 25.8 mmHg, respectively.
CONCLUSIONS: PAWP >20 mmHg and mean PA pressure >25 mmHg could be used to define elevated values during exercise in adults post-Fontan. The major discrepancy in exercise haemodynamics among Group II compared to controls appears to be the degree of systemic venous hypertension and arterial desaturation.

暂无摘要。

Atrial arrhythmias are a common late manifestation after Fontan palliation and are known to contribute to significant morbidity and mortality. Atrial volume by cardiac magnetic resonance imaging has been increasingly used in patients with congenital heart disease with no reports in those with Fontan palliation. In acquired heart disease, left atrial volume has been shown to be a strong predictor of outcomes of sustained atrial arrhythmias, including recurrence of atrial fibrillation. We hypothesized that combined atrial volume (CAV) in patients with total cavopulmonary connection (TCPC) Fontan palliation may be associated with increased risk of significant atrial arrhythmias (SAA). This is a single center retrospective case-control study. Cases were defined as patients with TCPC Fontan palliation ≥ 18 years of age, with SAA requiring intervention. Only those with advanced imaging for 3D rendering between 2013 and 2022 were included. CAV was analyzed from a 3-dimensional (3D) data set, including both the left and right atria, excluding the Fontan baffle. Seventeen TCPC Fontan case patients and 17 control patients were included. There was no difference in age between the two groups. There was no difference between gender, type of Fontan palliation, atrio-ventricular valve regurgitation, or combined ventricular function between the two groups. CAV was higher in SAA group compared to controls, and all control patients had indexed CAV ≤ 80 mL/kg. This is the first data suggesting CAV is associated with SAA in TCPC Fontan patients. Indexed CAV ≥ 80 mL/kg may be a valuable marker for SAA risk.

暂无摘要。