A case report of atrial fibrillation in early adulthood: dig deeper.

Background: Atrial fibrillation (AF) is a common cardiac arrhythmia, which is often associated with underlying risk factors and undiagnosed conditions, including congenital heart disease. Atrial septal defects (ASDs) come to mind, albeit arrhythmias usually present later in life. We present herewith a young patient with cor triatriatum sinister (CTS), with some delay in establishing the diagnosis, following new onset AF in early adulthood. Case summary: A 31-year-old man presented with pre-syncope and coryzal symptoms and was newly diagnosed with AF in the context of an intercurrent viral illness. After treatment with oral anticoagulation and successful outpatient cardioversion, he was discharged from cardiology review. Two years later he re-presented with exercise intolerance and a 12-lead electrocardiogram revealing recurrence of AF. Subsequent investigation with transthoracic echocardiography revealed the underlying congenital cardiac defect of CTS, together with an ASD and patent foramen ovale. After corrective surgery, which involved membrane resection, a Cox-maze procedure and ASD closure, sinus rhythm was restored and at follow-up the patient had returned to baseline function. Discussion: Young patients presenting with new onset AF should undergo thorough cardiovascular assessment to identify treatable causes and reversible risk factors. Cor triatriatum sinister is a rare congenital anomaly that may present in adulthood and give rise to symptomatic AF. Surgical correction including a Cox-maze procedure in our patient resulted in restoration of sinus rhythm and a return of the patient's baseline functional status and improved quality of life.

Surgical Management of Valvular Heart Disease in Mucopolysaccharidoses: A Review of Literature.

Mucopolysaccharidoses are extremely rare diseases that are frequently presenting with structural heart problems of the aortic and mitral valve in combination with myocardial dysfunction. In a substantial proportion, this leads to heart failure and is a leading cause of death in these patients. As this glycosaminoglycan degradation defect is associated with other conditions strongly influencing the perioperative risk and choice of surgical technique, multidisciplinary planning is crucial to improve short- and long-term outcomes. The extensive variance in clinical presentation between different impaired enzymes, and further within subgroups, calls for personalised treatment plans. Enzyme replacement therapies and bone marrow transplantation carry great potential as they may significantly abrogate the progress of the disease and as such reduce the clinical burden and improve life expectancy. Nevertheless, structural heart interventions may be required. We reviewed the existing literature of the less than 50 published cases regarding surgical management, technique, and choice of prostheses. Although improvement in therapy has shown promising results in protecting valvar tissue when initiated in infancy, concerns regarding stability of this effect and durability of biological prostheses remain.

Cardiohelp System use in school age children and adolescents at a center with interfacility mobile extracorporeal membrane oxygenation capability.

Cardiohelp System use for pediatric extracorporeal membrane oxygenation (ECMO) beyond the transport setting is sparsely described in literature. We report the use of Getinge's Cardiohelp System in children and integrated utilization of Mobile ECMO Retrieval Team (MERT) at an all-age specialized cardiorespiratory center. Electronic database of all patients under 16 years of age who received ECMO with use of the Cardiohelp System between January 2018 and March 2020 was retrospectively reviewed and analyzed for demographics, set-up, complications, and outcomes. Out of 41 patients, seven patients (four in middle childhood, three in early teenage) with median age of 10 years (range 8.8-15.6) were supported with use of Cardiohelp System. Median weight and height were 34 kg (range 28-53) and 145 cm (range 134-166) respectively. Initial ECMO deployment was veno-arterial (V-A) in five patients and veno-venous (V-V) in two. There were three interhospital transfers by our MERT, and 12 intrahospital transfers for interventions or imaging. The median ECMO therapy was 7 days (range 4-25), with standard 3/8-inch tubing and ECMO flow rate range at 56-100 mL/kg/min (1.89-5.0 LPM). There were two circuit changes and three reconfigurations of support. Two patients received continuous veno-venous hemofiltration via ECMO circuit. The 90-day and 180-day survival rates were 100% (including two heart transplants at day 7 and day 8). There were no transport-related or circuit-related complications during the 1750 h of Cardiohelp use. Cardiohelp System use is safe in pediatric patients for diverse application of ECMO support including inter- and intrahospital transfers.

Follow the Lead: The Challenges of Cardiogenic Shock in Device-Related Infective Endocarditis.

We present the challenging case of a young man with congenital heart disease who survived severe device-related infective endocarditis and new pulmonary hypertension. He required prolonged mechanical circulatory support and had multiple significant complications. His case posed a management dilemma that was successfully resolved by effective multidisciplinary, tertiary center care. (Level of Difficulty: Beginner.).

Enhanced Assessment of Perioperative Mortality Risk in Adults With Congenital Heart Disease.

BACKGROUND: In-hospital mortality is a rare, yet feared complication following cardiac surgery in adult congenital heart disease (ACHD). A risk score, developed and validated in ACHD, can be helpful to optimize risk assessment. OBJECTIVES: The purpose of this study was to assess the performance of EuroSCORE II components and procedure-related Adult Congenital Heart Surgery (ACHS) score, identify additional risk factors, and develop a novel risk score for predicting in-hospital mortality after ACHD surgery. METHODS: We assessed perioperative survival in patients aged >16 years undergoing congenital heart surgery in a large tertiary center between 2003 and 2019. A risk variable-derived PEACH (PErioperative ACHd) score was calculated for each patient. Internal and external validation of the model was undertaken, including testing in a validation cohort of patients operated in a second European ACHD center. RESULTS: The development cohort comprised 1,782 procedures performed during the study period. Re-sternotomy was undertaken in 897 (50.3%). There were 31 (1.7%) in-hospital deaths. The PEACH score showed excellent discrimination ability (area under the curve [AUC]: 0.88; 95% CI: 0.83-0.94), and performed better than the ACHS score in our population (ACHS AUC: 0.69; 95% CI: 0.6-0.78; P = 0.0003). A simple 3-tiered risk stratification was formed: PEACH score 0 (in-hospital mortality 0.2%), 1-2 (3.6%), and ≥3 (17.2%). In a validation cohort of 975 procedures, the PEACH score retained its discriminative ability (AUC: 0.75; 95% CI: 0.72-0.77) and was well calibrated (Hosmer-Lemeshow chi-square goodness-of-fit P = 0.55). There was agreement in expected and observed perioperative mortality between cohorts. CONCLUSIONS: The PEACH score is a simple, novel perioperative risk score developed and validated specifically for ACHD patients undergoing cardiac surgery.

Structural and Functional Properties of Subsidiary Atrial Pacemakers in a Goat Model of Sinus Node Disease.

BACKGROUND: The sinoatrial/sinus node (SAN) is the primary pacemaker of the heart. In humans, SAN is surrounded by the paranodal area (PNA). Although the PNA function remains debated, it is thought to act as a subsidiary atrial pacemaker (SAP) tissue and become the dominant pacemaker in the setting of sinus node disease (SND). Large animal models of SND allow characterization of SAP, which might be a target for novel treatment strategies for SAN diseases. METHODS: A goat model of SND was developed (n = 10) by epicardially ablating the SAN and validated by mapping of emergent SAP locations through an ablation catheter and surface electrocardiogram (ECG). Structural characterization of the goat SAN and SAP was assessed by histology and immunofluorescence techniques. RESULTS: When the SAN was ablated, SAPs featured a shortened atrioventricular conduction, consistent with the location in proximity of atrioventricular junction. SAP recovery time showed significant prolongation compared to the SAN recovery time, followed by a decrease over a follow-up of 4 weeks. Like the SAN tissue, the SAP expressed the main isoform of pacemaker hyperpolarization-activated cyclic nucleotide-gated channel 4 (HCN4) and Na+/Ca2+ exchanger 1 (NCX1) and no high conductance connexin 43 (Cx43). Structural characterization of the right atrium (RA) revealed that the SAN was located at the earliest activation [i.e., at the junction of the superior vena cava (SVC) with the RA] and was surrounded by the paranodal-like tissue, extending down to the inferior vena cava (IVC). Emerged SAPs were localized close to the IVC and within the thick band of the atrial muscle known as the crista terminalis (CT). CONCLUSIONS: SAN ablation resulted in the generation of chronic SAP activity in 60% of treated animals. SAP displayed development over time and was located within the previously discovered PNA in humans, suggesting its role as dominant pacemaker in SND. Therefore, SAP in goat constitutes a promising stable target for electrophysiological modification to construct a fully functioning pacemaker.

Cardiac and Vascular Surgeons for the Treatment of Aortic Disease: A Successful Partnership for Decision-Making and Management of Complex Cases.

Traditionally, cardiac and vascular surgeons have been treating diseases of the aorta as individual specialists. Neither cardiac nor vascular surgeons have ever considered the aorta as a whole, which can be diseased throughout its length at the same time requiring a more thoughtful and different approach. Aortic dissection and aneurysmal disease may well benefit from a multidisciplinary approach. In the context of this review, we discuss examples of joint operating between cardiac and vascular surgeons that may well become a more routine approach in more units in the future.

Umbilical artery catheter, aortic dissection, carotid cannulation, and pseudoaneurysm in a neonate: A tale of propagating pathology.

Arterial dissections are uncommon in neonates. Complications include thrombosis, bleeding, dissection, aneurysm and pseudoaneurysm. We report an unusual case of multisite pathology (dissection and pseudoaneurysm) following common vascular interventions. A term neonate with antenatal diagnosis of congenital heart block secondary to maternal lupus deteriorated clinically at 5 days of life. He was found to have an abdominal aortic thrombus secondary to abdominal aortic dissection, following umbilical arterial catheter placement. Attempted percutaneous treatment was complicated by dissection of the left common carotid artery and formation of a large pseudoaneurysm. Neonatal lupus is associated with weakened vessel wall which may be vulnerable to injury from line placement and endovascular interventions. Various options are available to manage arterial dissection, thrombus, and pseudoaneurysm, but consequences of these options need to be carefully weighed to minimize further complications.

Prognostic value of echocardiographic parameters in pediatric patients with Ebstein's anomaly.

BACKGROUND: Accurate risk stratification of patients with Ebstein's anomaly (EA) is crucial. Aim of the study was to assess the prognostic value of echocardiography, including 2D speckle tracking (STE) derived myocardial deformation indices, for predicting outcome in pediatric and young adult unrepaired EA patients. METHODS: Fifty consecutive EA patients (1 day-18 years, 52% males) underwent echocardiography and were followed for a mean follow-up of 60 ±â€¯41 months for clinical outcome (ventricular tachyarrhythmia, heart failure, need for surgery and/or death). Clinical and instrumental features of EA patients with stable disease were compared with those of EA patients with progressive disease. RESULTS: Twenty-four (48%) EA patients had progressive disease. A more severe grade of tricuspid valve (TV) displacement [59.7 mm/m2 (IQR 27.5-83) vs 28.4 mm/m2 (IQR 17.5-47); p = 0.002], a lower functional right ventricle (RV) fractional area change (FAC) (29.2 ±â€¯7.7% vs 36.7 ±â€¯9.6%; p = 0.004), a higher Celermajer index [0.8 (IQR 0.7-0.98) vs 0.55 (IQR 0.4-0.7); p = 0.000], a lower functional RV-longitudinal strain (-10.2 ±â€¯6.2% vs -16.2 ±â€¯7.3%; p = 0.003) and a lower right atrium peak systolic strain (RA-PALS) (25.2 ±â€¯13.5% vs 36.3 ±â€¯12.5%; p = 0.004) were detected in progressive disease group compared to stable one, respectively. Functional RV-FAC and RA-PALS were independent predictors of progressive disease at multivariate analysis. CONCLUSION: Our study demonstrated for the first time the prognostic role of RV-FAC and RA-PALS in a long-term follow-up of EA young patients. A complete echocardiographic evaluation should be regular part in the evaluation and risk-stratification of EA children.

A meta-analysis of platelet gel for prevention of sternal wound infections following cardiac surgery.

Deep sternal wound infection and bleeding are devastating complications following cardiac surgery, which may be reduced by topical application of autologous platelet gel. Systematic review identified seven comparative studies involving 4,692 patients. Meta-analysis showed significant reductions in all sternal wound infections (odds ratio 3.48 [1.08-11.23], p=0.04) and mediastinitis (odds ratio 2.69 [1.20-6.06], p=0.02) but not bleeding. No adverse events relating to the use of topical platelet-rich plasma were reported. The use of autologous platelet gel in cardiac surgery appears to provide significant reductions in serious sternal wound infections, and its use is unlikely to be associated with significant risk.

Benefit of endovascular stenting for aortic coarctation on systemic hypertension in adults.

BACKGROUND: Endovascular stenting is a recognised treatment strategy for aortic coarctation (CoA) in adults. We assessed systemic hypertension control and the need for antihypertensive therapy after CoA stenting in adults. METHODS: Data were collected prospectively on 54 patients (36 men; mean age: 34 ± 16 years) who underwent endovascular stenting for CoA over a 7-year period. Five patients were excluded as they did not attend follow-up appointments. Patients underwent clinical examination, including right arm systolic blood pressure (SBP) and 24-hour ambulatory blood pressure monitoring at baseline, 6-12 weeks and 9-12 months. RESULTS: There was a significant fall in mean peak-to-peak systolic gradient (PG) across the CoA after stenting (26 ± 11 mmHg vs. 5 ± 4 mmHg; P<0.01). There were successive reductions in right arm SBP and ambulatory SBP at baseline, 6-12 weeks and 9-12 months post-procedure (right arm: 155 ± 18 mmHg vs. 137 ± 17 mmHg vs. 142 ± 16 mmHg, respectively; all P-values <0.01; ambulatory: 142 ± 14 mmHg vs. 132 ± 16 mmHg vs. 131 ± 15 mmHg, respectively; all P-values <0.01). Twenty-four patients had severe CoA (PG >25 mmHg before stenting); baseline SBP was significantly higher in severe versus non-severe patients (160 mmHg vs. 148 mmHg; P=0.02). The absolute reduction in PG after stenting was significantly higher in the severe group (31 ± 7 mmHg vs. 14 ± 5 mmHg; P<0.0001), but there was no significant difference in SBP between groups at 6-12 weeks (141 mmHg vs. 135 mmHg; P=0.21) or 9-12 months (139 mmHg vs. 139 mmHg; P=0.96). CONCLUSION: Endovascular stenting of CoA results in a significant reduction in SBP at 6-12 weeks, which is sustained at 9-12 months, with similar outcomes in severe and non-severe CoA groups.

Urgent resection of a giant left atrial appendage aneurysm and mitral valve replacement in a complex case of Hurler-Scheie syndrome.

Hurler-Scheie syndrome is a rare lysosomal storage disease affecting the cardiovascular system. Besides the cardiac manifestations, it presents with complications from abnormal proteoglycan deposition in soft tissues in many locations, resulting in joint contractures, paraplegia, impaired vision, airway narrowing and restrictive lung function, to name a few. There are very few reports of surgical management of valvular heart disease due to mucopolysaccharidosis (MPS). We describe the successful management of a patient with an extremely challenging case of mitral valve stenosis and a giant left atrial appendage aneurysm due to MPS type 1 (Hurler-Scheie syndrome). The patient underwent mitral valve replacement and excision of the giant left atrial appendage aneurysm; a similar case has not been previously reported.

Outcomes following the Kawashima procedure for single-ventricle palliation in left atrial isomerism.

OBJECTIVES: Patients with left atrial isomerism and interrupted inferior vena cava palliated with a superior cavopulmonary connection or Kawashima procedure (KP) have a high incidence of developing pulmonary arteriovenous malformations. The necessity for hepatic vein redirection (HVR) and its timing remains a controversy. We aimed to assess the clinical outcome of patients with left atrial isomerism following a KP. The main end points were death, requirement for HVR and the impact of HVR on oxygen saturation. METHODS: Retrospective review of 21 patients with a diagnosis of left atrial isomerism, interruption of the inferior vena cava and single-ventricle physiology managed with a KP at a single centre between January 1990 and March 2010. RESULTS: Twenty-one patients had a KP, with 12 subsequently undergoing HVR. There was relatively a constant monthly decrement in the proportion of patients who were free from death or HVR up until 60 months following the KP, with a dramatic increase in the hazard after this time. The Cox proportional hazards regression model demonstrated a reduced early risk for HVR or death in patients who underwent pulmonary artery banding versus arterial shunt as the primary procedure (hazard ratio: 0.10; P = 0.01), and an increased risk with bilateral superior vena cavas (SVCs) (hazard ratio: 3.4; P = 0.04) and age at KP (hazard ratio: 1.02 per month increase in age at KP; P = 0.02). HVR mortality was relatively high with 3 of 12 patients dying in the early postoperative period with profound cyanosis. The timing of HVR after the KP did not influence the postoperative rate of increase in oxygen saturation. CONCLUSION: These findings confirm that the majority of patients who undergo a KP will require HVR. Patients who are older at the time of the KP or having an initial arterial shunt or bilateral SVCs are at higher risk of HVR or death. The relatively high mortality at HVR was characterized by severe postoperative cyanosis.