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Background Creation of Fontan circulation helps establishing separating pulmonary and systemic circulation in patients born with anatomical/physiological single ventricle. These patients have significantly impaired exercise capacity, mainly due to limited augmentation of cardiac output, predominantly secondary to impaired venous return (pre-load). Studies have suggested that pulmonary vasodilator therapies improve cardiac output by augmenting venous return. Use of such vasodilator therapy is significantly sparse, as many of these patients are reported to have normal pulmonary vascular resistance (PVR). However, the normal range of PVR was defined only in those with subpulmonic ventricle and pulsatile pulmonary flow. Incorporating such an information into routine management of patients with Fontan circulation, who have passive venous filling and non-pulsatile flow is likely to mislead physicians, and delay appropriate therapy in many suitable candidate. We sought to evaluate observed PVR in this cohort. Materials and methods Retrospective review of data from a large tertiary adult congenital heart disease (ACHD) centre in the UK. Results From a cohort of 4454 patients with complex ACHD conditions, 154 had Fontan circulation; 70 patients with failing Fontan were investigated by cardiac catheterization. Thirty-four (48.6%) were male, mean age of 30.1±6.2 years (17–43), and mean body mass index of 24.1±5.3 kg/m2. Mean Fontan pressure was 16±4 mmHg (7–29), mean wedge capillary pressure was 11±3 mmHg (4–19), and mean trans-pulmonary gradient (TPG) was 5±3 mmHg (0–15). Mean cardiac output was 4.1±2.7 L/min and calculated PVR was 1.7±1.2 Wood units (0.25–5.3). Although cardiac output was well maintained, mean Fontan pressure significantly correlated with the mean capillary wedge pressure (p Conclusions In patients with Fontan circulation, rise in PVR above the normal range was observed only after moderate rise in Fontan pressure. Such rise in PVR seems to be a very late phenomenon to occur. Acceptable range of PVR in patients with Fontan circulation should be re-defined, so that patients can be treated in a timely fashion. A large prospective trial or registry should be established to explore this subject further in details.

Background Advances in clinical management have improved life expectancy in the ACHD population; as a result, implantable cardiac devices remain in situ for longer periods than ever before. These patients pose unique challenges, including; young age at implant, multiple device procedures during their lifetime, complex venous and cardiac anatomy, and co-morbidities which may predispose to infection. In the non-ACHD population, there is useful long-term research which helps to guide clinical decision-making; however, small numbers and incomplete outcome data has lead to limited comparability against ACHD patients. Aims This study aimed to examine 20 years of pacing experience in a large ACHD centre, using novel data-mining techniques to generate comprehensive lead survival data. Methodology A retrospective analysis was performed of pacing and defibrillator leads implanted between June 1996 and December 2016 in a 173 patient ACHD cohort. Automated text searching algorithms with manual review of over 1 00 000 pieces of clinical correspondence was used to maximise identification of outcomes. Lead-specific complications, non-elective removal and overall lead survival were compared for surgical and transvenous leads in patients with complex and non-complex ACHD anatomy. Results 340 leads with complete implant and follow-up data were identified of which 53% were in patients with complex ACHD anatomy and 15% were in patients with tricuspid valve abnormalities. Median lead survival time was 13.6 years in transvenous leads and 15.9 years in surgical leads (p=n .s.) Transvenous leads were associated with a higher risk of major infection than were surgically-implanted leads (11.5% vs 0% p Conclusion In this large cohort of patients with ACHD, lead survival appears to be reasonable regardless of whether a transvenous or surgical approach is used. A higher incidence of infection in transvenous leads is offset by a higher electrical failure rate in surgical leads. Further work examining large cohorts is required to determine optimal pacing strategies tailored to patient-specific anatomical challenges.

Background: Infants born with cardiac abnormalities causing dependence on the arterial duct for pulmonary blood flow are often palliated with a shunt usually between the subclavian artery and either pulmonary artery. A so-called modified Blalock-Taussig shunt allows progress through early life to an age and weight at which repair or further more stable palliation can be safely achieved. Modified Blalock-Taussig shunts continue to present concern for postprocedural instability and early mortality such that other alternatives continue to be explored. Duct stenting (DS) is emerging as one such alternative with potential for greater early stability and improved survival. Methods: The purpose of this study was to compare postprocedural outcomes and survival to next-stage palliative or reparative surgery between patients undergoing a modified Blalock-Taussig shunt or a DS in infants with duct-dependent pulmonary blood flow. All patients undergoing cardiac surgery and congenital interventions in the United Kingdom are prospectively recruited to an externally validated national outcome audit. From this audit, participating UK centers identified infants Results: There was an early (to discharge) survival advantage for infants before next-stage surgery in the DS group (odds ratio, 4.24; 95% confidence interval, 1.37–13.14; P =0.012). There was also a difference in the need for postprocedural extracorporeal support in favor of the DS group (odds ratio, 0.22; 95% confidence interval, 0.05–1.05; P =0.058). Longer-term survival outcomes showed a reduced risk of death before repair in the DS group (hazard ratio, 0.25; 95% confidence interval, 0.07–0.85; P =0.026) but a slightly increased risk of reintervention (hazard ratio, 1.50; 95% confidence interval, 0.85–2.64; P =0.165). Conclusions: DS is emerging as a preferred alternative to a surgical shunt for neonatal palliation with evidence for greater postprocedural stability and improved patient survival to destination surgical treatment.

Objectives This study aimed to report our national experience with transcatheter patent ductus arteriosus (PDA) occlusion in infants weighing

Creation of Fontan circulation helps separating pulmonary and systemic circuits in patients born with single ventricle. They have impaired exercise capacity, mainly due to limited augmentation of cardiac output (CO). Studies have suggested that pulmonary vasodilator therapies improve CO by

Trans-catheter pulmonary valve implantation (TPVI) has become a less-invasive alternative to those at higher risk for surgical pulmonary valve replacement. Recent publications have raised concern with increased incidence of infective endocarditis (IE) following TPVI. We evaluated our cohort of