暂无摘要。

UNASSIGNED: Patients with transposition of the great arteries (TGA) and systemic right ventricle often confront significant adverse cardiac events. The prognostic significance of invasive hemodynamic parameters in this context remains uncertain. Our hypothesis is that the aortic pulsatility index and hemodynamic profiling utilizing invasive measures provide prognostic insights for patients with TGA and a systemic right ventricle.
UNASSIGNED: This retrospective multicenter cohort study encompasses adults with TGA and a systemic right ventricle who underwent cardiac catheterization. Data collection, spanning from 1994 to 2020, encompasses clinical and hemodynamic parameters, including measured and calculated values such as pulmonary capillary wedge pressure, aortic pulsatility index, and cardiac index. Pulmonary capillary wedge pressure and cardiac index values were used to establish 4 distinct hemodynamic profiles. A pulmonary capillary wedge pressure of ≥15 mm Hg indicated congestion, termed wet, while a cardiac index <2.2 L/min per m2 signified inadequate perfusion, labeled cold. The primary outcome comprised a composite of all-cause death, heart transplantation, or the requirement for mechanical circulatory support.
UNASSIGNED: Of 1721 patients with TGA, 242 individuals with available invasive hemodynamic data were included. The median follow-up duration after cardiac catheterization was 11.4 (interquartile range, 7.5-15.9) years, with a mean age of 38.5±10.8 years at the time of cardiac catheterization. Among hemodynamic parameters, an aortic pulsatility index <1.5 emerged as a robust predictor of the primary outcome, with adjusted hazard ratios of 5.90 (95% CI, 3.01-11.62; P<0.001). Among the identified 4 hemodynamic profiles, the cold/wet profile was associated with the highest risk for the primary outcome, with an adjusted hazard ratio of 3.83 (95% CI, 1.63-9.02; P<0.001).
UNASSIGNED: A low aortic pulsatility index (<1.5) and the cold/wet hemodynamic profile are linked with an elevated risk of adverse long-term cardiac outcomes in patients with TGA and systemic right ventricle.

UNASSIGNED: Transcatheter edge-to-edge repair (TEER) for the systemic atrioventricular valve has been anecdotally reported as a viable treatment option in symptomatic inoperable adult patients born with congenitally corrected transposition of the great arteries (ccTGA). However, to date, case reports on TEER treatment of both atrioventricular valves are lacking, especially when considering the present availability of specific mitral and tricuspid valve TEER devices.
UNASSIGNED: We present the case of an 84-year-old man with recurrent admissions for acute heart failure due to high-grade regurgitation of both atrioventricular valves. The patient was first diagnosed with ccTGA at this advanced age and underwent a thorough multimodality imaging approach, including transthoracic and transoesophageal echocardiography, cardiac magnetic resonance imaging, cardiac computed tomography, and ventriculography of the systemic ventricle. Due to the high symptom burden despite optimal medical therapy and high doses of diuretics, the heart team recommended TEER, first for the systemic tricuspid valve and later on for the non-systemic mitral valve. Both complex procedures were uneventful and led to considerable improvement in quality of life.
UNASSIGNED: Congenitally corrected transposition of the great arteries mostly manifests itself in adulthood and affects both ventricles and atrioventricular valves. In case of anatomical doubts on transthoracic echocardiography, a thorough multimodality imaging work-up is recommended. Transcatheter treatment of both atrioventricular valves seems to be a safe and effective therapeutic option in these often inoperable patients.

Background: Congenitally corrected transposition of the great arteries (cc-TGA) is a defect characterized by arterio-ventricular and atrioventricular disconcordance. Most patients have co-existing cardiac abnormalities that warrant further treatment. Some patients do not require surgical intervention, but most undergo physiological repair or anatomical surgery, which enables them to reach adulthood. Aims: We aimed to evaluate mortality risk factors in patients with cc-TGA. Results: We searched the PubMed database and included 10 retrospective cohort studies with at least a 5-year follow-up time with an end-point of cardiovascular death a minimum of 30 days after surgery. We enrolled 532 patients, and 83 met the end-point of cardiovascular death or equivalent event. As a risk factor for long-term mortality, we identified New York Heart Association (NYHA) class ≥III/heart failure hospitalization (OR = 10.53; 95% CI, 3.17-34.98) and systemic ventricle dysfunction (SVD; OR = 4.95; 95% CI, 2.55-9.64). We did not show history of supraventricular arrhythmia (OR = 2.78; 95% CI, 0.94-8.24), systemic valve regurgitation ≥moderate (SVR; OR = 4.02; 95% Cl, 0.84-19.18), and pacemaker implantation (OR = 1.48; 95% Cl, 0.12-18.82) to affect the long-term survival. In operated patients only, SVD (OR = 4.69; 95% CI, 2.06-10.71) and SVR (OR = 3.85; 95% CI, 1.5-9.85) showed a statistically significant impact on survival. Conclusions: The risk factors for long-term mortality for the entire cc-TGA population are NYHA class ≥III/heart failure hospitalization and systemic ventricle dysfunction. In operated patients, systemic ventricle dysfunction and at least moderate systemic valve regurgitation were found to affect survival.

暂无摘要。

BACKGROUND: Congenitally corrected transposition of the great arteries (CC-TGA) is a rare and complex form of congenital heart disease. Results of physiologic repair proved disappointing due to late right ventricular dysfunction and/or tricuspid regurgitation. The current study was performed to evaluate surgical outcomes in patients undergoing a double switch for CC-TGA.
METHODS: This was a retrospective review of 121 patients who underwent a double switch over a 2-decade time frame (2002-2023). Patients were a median age of 32 months. Before the double switch, 49 of 121 patients (40%) had undergone left ventricular retraining.
RESULTS: Sixty-seven patients underwent an arterial switch, and 54 underwent a Rastelli procedure. There were 4 in-hospital deaths (3.3%), including 3 who had a Rastelli procedure (5.6%) and 1 who had an arterial switch (1.5%). At a median follow-up of 30 months, there were 4 late deaths (2 Rastelli and 2 arterial switch). Combined early and late mortality was 9.3% for the Rastelli and 4.5% for arterial switch. Combined mortality was 2.0% for patients who required left ventricular retraining vs 9.7% for those who did not. For the 117 patients discharged from the hospital, 93% have normal or low-normal left ventricular function, and 96% have mild or less neoaortic insufficiency.
CONCLUSIONS: Surgical outcomes in patients undergoing a double switch procedure have been excellent both in the short- and midterm. However, the Rastelli procedure was associated with a more than 2-fold increase in mortality risk compared with the arterial switch.

UNASSIGNED: The atrio-ventricular and ventricle-arterial double discordance (DD) or corrected transposition of the great arteries is a rare heart disease, it occurs in 0.02-0.07 of every 1,000 live newborns. The objective of the study is to describe the diagnosis, treatment and evolution of a series of patients with DD.
UNASSIGNED: A retrospective and descriptive study was carried out, reviewing the records of patients diagnosed with DD in the last 22 years. Descriptive statistics were performed. Numerical variables were obtained using means and standard deviation and categorical variables using frequencies and percentages.
UNASSIGNED: Thirty patients were studied in 22 years with a ratio of 1.5:1 for men, with a mean age of 20 months. The situs was solitus in 24/29 patients (82.7%). Ventricular septal defect was the most frequent lesion in 25/29 (86.2%) Tricuspid insufficiency in 70%. Four patients diagnosed with pulmonary atrial hypertension. With atrio-ventricular block 20%. One with Wolff-Parkinson-White syndrome. Surgical treatment was carried out in 70% of patients. Eight with Glenn procedure (26.6%) and 4 with Fontan surgery (13.3%). Follow-up ranged from 1 month to 17 years. Five died (16.6%). Of the 25 patients in follow-up, 18 patients (72%) had normal ventricular function, 5 with Grade II Ross classification (20%) and 2 in Grade III (8%).
UNASSIGNED: The quality of life of these patients is improving and there is still controversy in the literature about the ideal time to perform the most appropriate surgical procedure.
UNASSIGNED: La doble discordancia auriculo-ventricular y ventrículo-arterial (DD) o transposición corregida de las grandes arterias, se presenta en 0.02-0.07 de cada 1,000 recién nacidos vivos. El objetivo del estudio es describir el diagnóstico, tratamiento y evolución de pacientes con DD.
UNASSIGNED: Se realizó un estudio retrospectivo y descriptivo, revisando los registros de pacientes con DD en los últimos 22 años. Se realizó estadística descriptiva. Las variables numéricas se obtuvieron mediante medias y desviación estándar y las categóricas mediante frecuencias y porcentajes.
UNASSIGNED: Se estudiaron 30 pacientes con una relación de 1.5:1 para el varón, con una edad media de 20 meses. El situs fue solitus en 24/29 pacientes (82.7%). La comunicación interventricular fue la lesión más frecuente en 25/29 pacientes (86.2%), insuficiencia tricuspídea en el 70%. Cuatro pacientes con diagnóstico de hipertensión arterial pulmonar. Con bloqueo atrio-ventricular un 20%. Uno con síndrome de Wolff-Parkinson-White. El tratamiento quirúrgico se realizó en el 70% de los pacientes. Con procedimiento de Glenn 8 (26.6%) y 4 cirugías de Fontan (13.3%). El seguimiento fue de 1 mes a 17 años. Cinco fallecieron (16.6%). De los 25 restantes, 18 pacientes (72%) con función ventricular normal, 5 con clasificación de Ross grado II (20%) y 2 en G III (8%).
UNASSIGNED: La calidad de vida de estos pacientes está mejorando, aún existe controversia sobre el momento ideal para realizar el procedimiento más adecuado quirúrgico.

To evaluate biventricular mechanics by means of echo-derived myocardial work (MW) analysis in patients with a systemic right ventricle (sRV). Comprehensive echo data were collected in all patients with a sRV who underwent transthoracic echocardiography at our tertiary centre between 2020 and 2021 including sRV function indices, global longitudinal strain (GLS) of right and left ventricle (RV/LV), biventricular MW, and atrial strain in those with congenitally corrected transposition of the great arteries (ccTGA). Fifty-six patients (37 [30.97-45.87]years, 59% male) and 49 healthy individuals matched per age and sex were included for comparison. Global work index (GWI:1106 [869.80-1293.10] Vs 314.2 [281.5-358.2]mmHg%, p < 0.0001) and global constructive work(GCW: 1542.50 [1338.9-1718.50] Vs 416.4 [365.70-464]mmHg%, p < 0.0001) were both increased for sRV compared to normal RV, reflecting exposition to a systemic afterload, with a contemporary raise in wasted work (GWW:197 [138.50-322.20] Vs 26.09 [17.80-43.48]mmHg%, p < 0.0001) and impaired efficiency (GWE:89 [83-93.54] Vs 93.67 [91.67-96] %, p < 0.0001). Conversely, sRV showed reduced MW indices in comparison to normal LV(p < 0.0001 for all). Non-systemic LV demonstrated normal GLS values (19.51 ± 3.9%), but reduced GWI (479 [368-665] Vs 2172 [1978-2386]mmHg%, p < 0.0001) and GCW (708 [490-815]mmHg% Vs 86.5 [59.25-118], p < 0.0001). Nevertherless, non-systemic LV showed also impaired efficiency (91 [88-94] Vs 95 [94-97]%, p < 0.0001). LVGLS values were related to RVGLS (R = 0.5, p = 0.00019), suggesting a consistent interventricular dependency. Atrial strain could be assessed in 16 out of 20 (80%) patients with ccTGA: both atria had reduced strain values compared to their normal counterparts. Moreover, pulmonary atrial strain during the reservoir phase was related to LVGWE (R = 0.58, p = 0.047) and inversely related to LVGLS (R = - 0.71, p = 0.0043). MW analysis is feasible in sRV and may provide additional clinical data. In our cohort MW revealed biventricular impairment, in particular for non-systemic LV, in spite of normal GLS values.

Congenitally corrected transposition of the great arteries (ccTGA) is a rare malformation with diverse morphology. We assessed features of fetuses with ccTGA and evaluated neonatal and pediatric outcomes. This was a retrospective review of fetuses with ccTGA at Birmingham Women\'s and Children\'s Hospital born from 2005 to 2019. Of thirty-six fetuses identified, six had unavailable prenatal data, one was postnatally diagnosed with isomerism and 29 fetuses were evaluated. ccTGA without associated cardiac lesions was found in 28% (8/29), ccTGA with significant VSD in 31% (9/29), ccTGA with pulmonary obstruction in 24% (7/29) and ccTGA with complex anomalies in 17% (5/29). Tricuspid regurgitation (TR) was observed in 17% (5/29) and heart block (HB) in 10% (3/29) prenatally. Six, that is 21% underwent genetic testing of which one was abnormal. Five extra-cardiac anomalies were reported prenatally and postnatally. Pregnancy was discontinued in five, of which two had moderate TR. There were thirty-one liveborn. Coarctation of the aorta was found in five postnatally but not suspected prenatally. In one, pulmonary stenosis was underestimated; otherwise, prenatal morphology was confirmed. Cardiac interventions were performed in 77% (24/31) liveborn with 39% (12/31) undergoing neonatal intervention. Overall, 6/31 liveborn died including all three with prenatal heart block and one with TR. Estimated survival for all liveborn at 1, 5 and 10 years was 87% (95% CI 76-100%), 83% (95% CI 72-98%) and 80% (95% CI 66-96%) respectively. Accurate prenatal diagnosis of ccTGA is critical for counseling. Early outcomes are favorable with 77% of liveborn undergoing surgery. Fetuses with prenatal diagnosis of complex associated abnormalities, HB and TR appear to do less well.

OBJECTIVE: There is limited information on long-term outcomes and trajectories of ventricular and valvular functions in patients with congenitally corrected transposition of the great arteries after anatomic repair according to the operative strategy with a median follow-up period of more than 10 years.
METHODS: Twenty-nine patients who underwent anatomic repair in Okayama University Hospital between January 1994 and December 2020 were reviewed. Outcomes were compared between patients who underwent a double switch operation (DS group) and patients with an atrial switch with a Rastelli operation (Rastelli-Senning/Mustard group).
RESULTS: Fifteen (52%) were in the DS group and 14 (48%) were in the Rastelli-Senning/Mustard group. The median follow-up period after anatomic repair was 12.7 (interquartile range 4.2-18.8) years. There were 3 (10%) early deaths and 3 (10%) late deaths. Survival rates for the entire cohort at 10 and 20 years were 86% and 71%, respectively, and were not different between the 2 groups. Using competing risk analysis, risks of heart failure, cardiac rhythm device implantation and atrial arrhythmia showed no significant differences between the 2 groups, whereas risk of reoperation was higher in the Rastelli-Senning/Mustard group than that in the DS group. Four patients after a DS operation and 1 patient after a Rastelli technique developed more than moderate aortic regurgitation.
CONCLUSIONS: During a median follow-up period of more than 10 years, mortality rate and ventricular and valvular functions after anatomic repair were acceptable, though the incidences of late complications were relatively high, especially in the Rastelli-Senning/Mustard group.