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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 Cardiac output (CO) measurement guides management of various medical conditions, including but not limited to various adult congenital heart diseases (ACHD), and pulmonary hypertension. It is mandatory to calculate patients’ oxygen consumption (VO2), to measure CO. Ideally VO2 consumption can be measured by using a facemask apparatus; however, due to complexity in their routine use, various formulae derives assumed VO2 are incorporated to obtain CO values. The most commonly used formula in catheter laboratories treating adult patients was reported by LaFarge and Miettinen (1970). However, it was based on data from paediatric population, and their use in adult population is not validated. Moreover, these individual formulae were compared with true VO2 consumption; however, limited information exploring agreement between these formulae is available. Such a comparison is very important, as individual catheter laboratories use these formulae at their discretion to derive cardiac output, influencing patients’ management. Materials and methods We sought to compare cardiac output measurement based upon four commonly used formulas, (1) LaFarge and Miettenen, (2) Dehmer, Firth and Hills, (3) Bergstra, Van Dijk, Jillege, and (4) Seckeler, Hirsch, Beekman Methodology, in 112 ACHD patients who underwent diagnostic catheterization at the Manchester Royal Infirmary, UK between 1st January 2015 to 31st March 2017. Their CO derived by using 4 different formulae were compared using a one way repeated measures ANOVA test. Results CO measured by various formulae is reported here with: LaFarge and Miettenen 4.31±1.43 L/min; Dehmer, Firth and Hills – 4.91±1.50 L/min; Bergstra, Van Dijk, Jillege, – 6.1±2.22 L/min; and Seckeler, Hirsch, Beekman Methodology – 2.96±0.88 L/min. LaFarge and Miettenen formula derived CO was lowest in comparison to all other formulae and was significantly lower than Dehmer as well as Bergstra formulae (p Conclusion There is no agreement between the assumed VO2 derived cardiac output measurements, including the most commonly used LaFarge formula, when compared in a cohort of ACHD patients. Using such assumed formulae derived CO may be misleading and such values should be interpreted carefully. Every cardiologist should be cognizant of these limitations. Facemask apparatus should be routinely used in catheter laboratories to obtain true VO2, especially, when such a value is likely to influence major management decisions.

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

Cardiac output (CO) measurement guides management of various medical conditions, including adult congenital heart diseases (ACHD) and pulmonary hypertension. It is mandatory to calculate patients’ oxygen consumption (VO2), to measure CO. Ideally VO2 consumption should be measured by using a

Objective The Blalock–Taussig (BT) shunt is an excellent palliative procedure for cyanotic congenital heart defects. We reviewed two techniques of performing the BT shunt, median sternotomy and thoracotomy, in relation to morbidity and mortality. Methods Forty-five modified BT shunts in 41 patients, mean age 93 days (1–1045 days), were performed between January 2002 and October 2004. Twenty-four (53.3%) shunts in 21 (51.2%) patients were performed through thoracotomy and 21 (46.7%) shunts in 20 (48.8%) patients through median sternotomy. One surgeon preferred thoracotomy and the other sternotomy approach irrespective of age/weight or elective/emergency. Thirty-eight (84.4%) cases underwent elective operation and 7 (15.6%) cases were operated as emergencies. In both groups the most frequent diagnosis was complex Tetralogy of Fallot. Results Postoperative oxygen saturation was same in both groups and there were no significant complications in either group. Patients undergoing BT shunt via median sternotomy approach had longer duration of ventilation (mean 183h vs. 53h, P P P P Conclusions Median sternotomy approach to performing BT shunt seems to carry a higher morbidity than thoracotomy. We recommend a large case series study and longer follow up.

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

Pulmonary atresia with intact septum is itself a rare congenital abnormality, albeit known to be associated with other cardiac and non-cardiac anomalies. The combination of right- and left-sided obstructive lesions, however, is extremely rare. We describe a patient having pulmonary atresia with intact septum associated with aortic coarctation, which to the best of our knowledge has been previously described on but one occasion.

The Blalock-Taussig (BT) shunt is an excellent palliative procedure for cyanotic congenital heart defects. We reviewed two techniques of performing the BT shunt, median sternotomy and thoracotomy, in relation to morbidity and mortality.Forty-five modified BT shunts in 41 patients, mean age 93 days (1-1045 days), were performed between January 2002 and October 2004. Twenty-four (53.3%) shunts in 21 (51.2%) patients were performed through thoracotomy and 21 (46.7%) shunts in 20 (48.8%) patients through median sternotomy. One surgeon preferred thoracotomy and the other sternotomy approach irrespective of age/weight or elective/emergency. Thirty-eight (84.4%) cases underwent elective operation and 7 (15.6%) cases were operated as emergencies. In both groups the most frequent diagnosis was complex Tetralogy of Fallot.Postoperative oxygen saturation was same in both groups and there were no significant complications in either group. Patients undergoing BT shunt via median sternotomy approach had longer duration of ventilation (mean 183 h vs. 53 h, P0.001) and inotropic requirements (33.3% vs. 4.2%, P0.05) leading to longer intensive care unit stay (mean 9.14 days vs. 3.3 days, P0.05) and hospital stay (mean 14.59 days vs. 5 days P0.005).Median sternotomy approach to performing BT shunt seems to carry a higher morbidity than thoracotomy. We recommend a large case series study and longer follow up.

Cardiac diverticula are rare and may have a wide spectrum of presentation ranging from an acute rupture and life threatening arrhythmias to being completely asymptomatic. The treatment options may vary but in view of their life threatening potential, the consensus is towards treating them early when the diagnosis was made. We present a case of an infant who presented in an emergency with cardiac arrest and was ultimately treated successfully on cardiopulmonary bypass and made an uneventful recovery. The literature has also been discussed on left ventricular diverticulum.

Without intervention, babies born with transposed great arteries (TGA) are doomed to a rapid death. Jatene and coworkers deserve the credit for performing the first successful arterial switch operation (ASO) in a patient with TGA and ventricular septal defect (VSD) in 1975. Since then ASO has become the procedure of choice in most medical centers. This review article summarizes the historical aspects of arterial switch operation and assesses this procedure's outcomes.