International journal of cardiology. Congenital heart disease最新文献

Background

Untreated critical pulmonary stenosis may develop into pulmonary atresia with intact ventricular septum, which is associated with a high risk of morbidity and mortality both in fetuses and neonates. In this meta-analysis, we sought to discover the potential of fetal pulmonary valvuloplasty that might affect patients' survival compared to other available procedures.

Methods

This systematic review and meta-analysis were conducted based on the PRISMA guideline. The authors thoroughly searched the recognized and potential interventions for PA-IVS, including FPV, total ventricular repair, and palliative procedures. The primary outcome was the mortality rate. We used R software (version 4.1.3) to calculate the overall proportion using the random-effects model of proportional meta-analysis.

Results

The FPV procedure was performed at a mean gestational age of 26.28 weeks (95%CI: 24.83–27.73) and was successful in 87.6 % (95 % CI: 78.3–96.3 %) of patients. A total of 52.9 % patients attained biventricular circulation postnatally (95 % CI: 31.2–74.7 %). Successful FPV was associated with a slightly higher overall mortality rate [periprocedural death 4.7 % (95%CI: 0–10.7 %) and postnatal death 8 % (95%CI: 3–13 %)] compared to the three currently available definitive therapies, namely the Fontan procedure [10 % (95%CI: 4–17 %)], 1.5V repair [11 % (95%CI: 5–17 %), and 2V repair [8 % (95%CI: 1–15 %)].

Conclusion

FPV can potentially increase the likelihood of biventricular circulation in fetuses with critical pulmonary valve stenosis.

Background

The relationship between plasma brain natriuretic peptide (NT-proBNP) and soluble suppression of tumorigenicity-2 (sST2) with structural adaptions and exercise capacity remains incompletely described in patients with repaired Tetralogy of Fallot (rTOF).

Methods

Peripheral venous blood samples were drawn for 99 patients with repaired TOF, 59 patients with severe pulmonary regurgitation (PR) and 40 patients with no or mild PR. NT-proBNP was measured using enzyme-linked immunosorbent assays (Roche Diagnostics, Indianapolis, IN). Soluble ST2 levels were assessed on Aspect-plus ST2 quantitative rapid test.

Results

The mean value of NT-proBNP was 160 ± 137 pg/ml, and sST2 was 29 ± 13, ng/ml in the entire population. 58 % had an elevated NT-proBNP, while sST2 was abnormal in 40 %. Mean NT-proBNP was significantly higher in patients with severe PR (169 ± 150 vs145 ± 118, pg/ml, p < 0.001), while similar sST2 levels were observed in both groups (29 ± 14 vs30 ± 12, ng/ml, p > 0.05). NT-proBNP and sST2 levels were higher in patients with transannular patch when compared to other RVOT intervention (174 ± 145 vs 107 ± 100, pg/ml, p < 0.001); (31 ± 13 vs 29 ± 15, ng/ml, p < 0.05). Both biomarkers were significantly associated with exercise capacity, but NT-proBNP (r = −0.60, p < 0.001) was stronger. The optimal cut-off of 90 pg/ml for NT-proBNP had a sensitivity of 74 % and specificity of 63 % for detection of impaired exercise capacity.

Conclusions

Serum levels of sST2 and NT-proBNP are elevated in patients with repaired TOF, with higher values observed in those with severe PR, but also in patients undergoing transannular patch repair. Incorporating both markers in these patients increased the ability to detect impairment in exercise capacity.

Background

After the arterial switch operation (ASO) for transposition of the great arteries (TGA), neo-aortic dilatation and coronary arterial anomalies, especially an interarterial course and acute coronary artery take-off angle, are commonly found. Long-term follow-up data after ASO is scarce. Aim of this study was to determine the prevalence of neo-aortic dilatation and coronary abnormalities, with special emphasis on acute coronary take-off angle, in adult TGA-ASO patients.

Methods

In this retrospective cohort study, all adult TGA-ASO patients with ≥1 CT-angiography (CTA) at the age of ≥16 years were included.

Results

Eighty-one patients, 69 % male and median age 21.0 [18.5–22.8] years, were included. At baseline, maximum neo-aortic diameter was 39.2 ± 5.3 mm; 35 (43 %) patients had neo-aortic dilatation (neo-aortic diameter of >40 mm), 22 (27 %) patients had an acute coronary take-off angle (<30°), and 5 (6 %) patients had an interarterial course of the RCA (2 %) or LCA (4 %). Neo-aortic or coronary artery re-intervention occurred in 10 (12 %) patients. All 10 patients had neo-aortic dilatation or coronary take-off angle of <30° on baseline CTA.

Conclusion

This study reports a prevalence of 43 % of neo-aortic dilatation, 6 % of interarterial coronary course and 27 % for acute coronary take-off angle (<30°) at a median term of 21.0 years post ASO. All patients with a neo-aortic re-intervention or coronary artery re-intervention during follow-up had a maximum neo-aortic diameter of >40 mm or a coronary take-off angle of <30° at baseline CTA. This hypothesis generating study suggests that an active surveillance in patients with neo-aortic dilation and/or an acute angulation of < 30° post ASO might be considered and requires prospective evaluation.

Background

The Fontan operation resulted in improved survival of patients with congenital heart defects not equipped to sustain biventricular circulation. Long-term complications are common, such as veno-venous collaterals (VVC). The aim of this study was to evaluate patient characteristics, percutaneous treatment strategy and (short-term) outcomes in adult Fontan patients with VVC, and review literature to date.

Methods

In this single-centre retrospective observational cohort study, patients who underwent percutaneous VVC closure between 2017 and 2023 were identified.

Results

Thirteen patients underwent percutaneous VVC closure (77 % female, age at intervention 24 ± 4 years, 77 % systemic left ventricle, 77 % extracardiac tunnel, median conduit size 16 [16–20]mm). Indications for closure were symptoms and/or significant exercise-related hypoxia. Mean Fontan pressure was 10±4 mmHg. The VVC originated from tributaries of the vena cava superior (VCS) and connected to pulmonary veins (8 VVC, 32 %), VCS to systemic atrium (3 VVC, 12 %), VCS to coronary sinus (3 VVC, 12 %) and tributaries of vena cava inferior to pulmonary veins (11 VVC, 44 %). Twenty-three VVC were occluded using coils and/or plugs. No periprocedural complications occurred. At first follow-up at least 6 months after closure (n = 11), 9 patients (82 %) reported symptom reduction. Saturation at rest and peak exercise increased significantly (96 ± 3 to 98 ± 1 %, p = 0.040; 89 ± 3 to 93 ± 5 %, p = 0.024, respectively). Exercise capacity remained unchanged.

Conclusions

VVC typically connect the tributaries of the vena cava inferior and/or superior with the pulmonary veins. Low Fontan pressures do not exclude the presence of VVC. Percutaneous closure of VVC is technically feasible, safe, and associated with symptom reduction and a significant rise in resting and exercise oxygen saturation.

Aim

Transcatheter Pulmonary Valve Implantation (TPVI), when feasible, is the first-line approach to pulmonary valve replacement. Our aim was to obtain a picture of current TPVI practice in Italy.

Methods

After conducting a literature review on TPVI, online surveys were devised by an Advisory Board of 10 experts from the three Italian reference centers for congenital heart diseases and sent electronically to physicians working either in implanting center or in referral non-implanting cardiologic centers.

Results

Approximately 450 physicians across Italy were invited to contribute. 82 (18%) physicians answered. EchoColorDoppler, electrocardiogram and cardiac magnetic resonance were considered the first line approach to monitor these patients, before and after TPVI.

For non-implanting centers, reasons for non-referral of patients for PVR were: paucisymptomatic disease (67%) and patients’ poor adherence to disease management programs (41%), but also the lack of connections with specialized centers (33%). For implanters, the main reasons for refraining from TPVI were: high risk for coronary compression (67% first rank), the need for concomitant cardiac surgical procedures (39% first rank) and the unsuitable anatomy of the conduit (39% first rank). The availability of new larger valves of a self-expandable nature was indicated as a key technological development for expanding the cohort of patients currently eligible for TPVI.

Conclusions

Despite a non-invasive imaging protocol for the follow up and selection of patients candidate to TPVI is well implemented in Italy, there is still a lack in connections between non-implanting and implanting centers.

Catheter interventions can offer patient tailored solutions in high-risk congenital heart disease patients. A 21-year-old male with a Fontan circulation in the setting of unbalanced atrioventricular septal defect with a hypoplastic left ventricle and an aortic homograft connecting the right ventricular outflow tract to the ascending aorta, developed failure of the heavily calcified homograft with severe regurgitation and stenosis. He underwent three sequential transcatheter aortic valve-in-valve implantations to address the homograft failure and the subsequent paravalvular regurgitation, with satisfactory result and improved hemodynamics.