International journal of cardiology. Congenital heart disease最新文献

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.

Background

Heterotaxy syndromes encompass left and right atrial isomerism (LAI and RAI respectively) and are associated with variable cardiac and non-cardiac anomalies which greatly influence outcomes. RAI is usually associated with complex congenital heart disease (CHD), early surgical intervention and increased mortality. LAI is less commonly associated with complex CHD but can be associated with heart block. The objective of this study was to review the clinical features and outcomes of patients with heterotaxy syndromes in Western Australia (WA).

Methods

A retrospective review was performed of live born patients diagnosed with heterotaxy from 2003 to 2022 in a statewide tertiary cardiac service, representing all cases in WA with a view to compare the outcomes between LAI and RAI at our centre.

Results

30 patients (53% male) were diagnosed with heterotaxy; 16 (53%) with LAI and 14 (47%) with RAI. Overall incidence was 0.48 per 10,000 live births over the defined period. RAI patients were significantly more likely to have an antenatal diagnosis (81.8% versus LAI 28.6%, p = 0.03). Overall, 5-year survival was 56% for RAI and 87% for LAI. No deaths occurred after the first 12 months of life with a median follow-up of 65 months (IQR 114.8). RAI was associated with asplenia (91%), atrioventricular septal defect (91%) and a functionally univentricular circulation (71%). LAI was associated with polysplenia (100%) and complete heart block in 3 patients (19%). Surgical pathways included repair of anomalous pulmonary venous return (45%), Blalock Taussig shunt (60%), bidirectional cavopulmonary connection (50%) and Fontan completion (30%).

Conclusions

Patients with RAI suffer high mortality and early surgical intervention, with few making it to Fontan completion. By comparison patients with LAI have less morbidity and mortality. The management of heterotaxy continues to be challenging due to widely associated cardiac and extracardiac manifestations.

Long-term effects of systemic hypertension (HTN) and HTN-mediated damages have been largely studied in non-congenital adult populations. By contrast, robust data about the predisposing factors, prevalence, consequences, and treatment of HTN in adults with congenital heart diseases (ACHD) is still scarce. Different mechanisms including the underlying cardiac disease, cardiac surgery and its consequences, the development of metabolic syndrome and secondary forms seem to play a role in HTN in ACHDs. To mitigate the potential long-term effects of HTN in this complex population, a meticulous follow-up is mandatory to identify patients who should receive treatment, and tailored strategies should be applied to obtain the best as possible result.

Thus, this review will investigate risk factors, effects, and treatments of HTN in ACHD patients.