2024 AHA/ACC/AMSSM/HRS/PACES/SCMR Guideline for the Management of Hypertrophic Cardiomyopathy: A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines.

AIM: The "2024 AHA/ACC/AMSSM/HRS/PACES/SCMR Guideline for the Management of Hypertrophic Cardiomyopathy" provides recommendations to guide clinicians in the management of patients with hypertrophic cardiomyopathy. METHODS: A comprehensive literature search was conducted from September 14, 2022, to November 22, 2022, encompassing studies, reviews, and other evidence on human subjects that were published in English from PubMed, EMBASE, the Cochrane Library, the Agency for Healthcare Research and Quality, and other selected databases relevant to this guideline. Additional relevant studies, published through May 23, 2023, during the guideline writing process, were also considered by the writing committee and added to the evidence tables, where appropriate. STRUCTURE: Hypertrophic cardiomyopathy remains a common genetic heart disease reported in populations globally. Recommendations from the "2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy" have been updated with new evidence to guide clinicians.

Utility of routine follow-up defibrillation safety margin testing in young patients with epicardial implantable cardioverter-defibrillators.

BACKGROUND: Routine defibrillation threshold testing (DFT) of transvenous implantable defibrillators (ICDs) has largely been in decline. In patients with non-transvenous ICDs that utilize subcutaneous and pleural ICD leads, serial DFT testing can detect a significant number of failures. Data about the utility of follow-up defibrillation safety margin testing (DSM) testing in pediatric patients and young adults with an epicardial ICD are lacking. METHODS: Patients aged < 25 years old who underwent epicardial ICD placement at Mayo Clinic from 2014 to 2023 with at least one follow-up DSM test were included. The patients were divided into a "routine" (R) and "clinically indicated" (CI) group based on the index of clinical concern. Inadequate DSM was defined as unsuccessful defibrillation at an output of less than 10 J below the maximum output of the device. The purpose of this study was to assess the utility of follow-up DSM testing. RESULTS: An epicardial ICD system was placed in 122 patients. A total of 26 patients met inclusion criteria and underwent a total of 47 DSM follow up tests. Inadequate DSM occurred in 1/33 (3%) in the R group and 2/14 (14%) DSM tests in the CI group. The median follow-up period was 54 and 36 months for the R and CI group, respectively. CONCLUSIONS: Our data suggest that epicardial ICDs are reliable and routine follow-up DSM testing may not be necessary for all patients. DSM testing should be performed in individuals with epicardial ICD systems when there is clinical concern about lead or coil performance.

Hypertrophic Cardiomyopathy: Preadolescence, Mitral Valve Disease, and Midventricular Obstruction.

Septal myectomy is indicated in patients with obstructive hypertrophic cardiomyopathy (HCM) who have persistent symptoms despite medical therapy, intolerance of medication side effects, or severe resting or provocable gradients. Septal myectomy at high volume centers is safe, with low operative mortality (1%) and low rates of complications such as complete heart block or ventricular septal defect (3% and 0.5%, respectively). Additionally, improved survival following myectomy has been observed when compared to patients with obstructive HCM managed medically or those with nonobstructive HCM. As a longstanding, quaternary referral center for septal myectomy, our institution has built significant experience and expertise in the surgical and medical management of HCM, including atypical HCM, defined as preadolescent patients, those with mitral valve disease, and those with isolated midventricular obstruction. The most important factor of septal myectomy in achieving complete resolution of obstruction and avoiding recurrence is the apical extent of the myectomy trough, which must extend to the septum opposite the papillary muscles. If this cannot be fully achieved via a transaortic exposure, especially in preadolescents and patients with midventricular obstruction, then a transapical approach may be needed. Mitral valve repair is rarely necessary as SAM-mediated MR resolves with adequate myectomy alone, but mitral repair is performed in cases of intrinsic valvular disease. In this manuscript we provide a summary of current operative techniques and outcomes data from our institution on the management of these various categories of HCM.

Cardiac Catheterization in Adults with Ebstein Anomaly: Pathophysiologic Insights Regarding Surgical Repair and Prognosis.

The underlying invasive hemodynamics and physiology in Ebstein anomaly (EA) are poorly understood. Moreover, the hemodynamic impact of tricuspid valve intervention in EA has not been well studied. Retrospective cohort of 52 adults with repaired and 36 with unrepaired EA undergoing right heart catheterization at Mayo Clinic, MN between 1993 and 2021. "Repaired" EA was defined as prior tricuspid valve repair and/or replacement (83% post-tricuspid valve replacement). Repaired patients were younger than those with unrepaired EA (41.3 ± 16.0 versus 50.6 ± 15.6 years, p = 0.008) and had a lower prevalence of ≥ moderate native or prosthetic tricuspid regurgitation (67% versus 81%, p = 0.01). Right atrial (RA) pressure was higher among patients with repaired EA than in unrepaired disease [13 (11; 18) versus 10 (8; 15) mmHg; p = 0.02], but these differences were no longer present when adjusting for ≥ moderate right ventricular systolic dysfunction and ≥ moderate tricuspid regurgitation. Cardiac index (Qs) was lower among those with unrepaired EA than in repaired EA (1.9 ± 0.7 versus 2.3 ± 0.6 l/min/m2, p = 0.01), even after adjusting for similar confounders. During a follow-up of 8.6 (3.2-13.3) years, 16 (18%) patients died. Systolic pulmonary artery pressure was independently associated with all-cause mortality. In summary, higher cardiac indices were found in those with repaired EA compared to those with unrepaired disease. RA hypertension was prevalent in both groups and no differences in right filling pressures were found between groups after adjusting for potential confounders. Elevation in pulmonary pressures was independently associated with survival. The use of pulmonary vasomodulators in EA requires further investigation.

Hemodynamics in Adults with Systemic Right Ventricles: Differences Between Congenitally Corrected and Complete Transposition of the Great Arteries.

Despite their anatomical differences, congenitally corrected (ccTGA) and complete transposition of the great arteries (d-TGA) post-atrial switch are frequently studied together and managed similarly from a medical standpoint due to the shared systemic right ventricle (sRV). The aim was to assess differences in their underlying hemodynamics. The study is a retrospective review of 138 adults with ccTGA or d-TGA post-atrial switch undergoing cardiac catheterization at Mayo Clinic, MN between 2000 and 2021. ccTGA was categorized into isolated or complex ccTGA depending on concomitant ventricular septal defect and/or left ventricular outflow obstruction. There were 53 patients with d-TGA (91% post-Mustard procedure), 51 with complex and 34 with isolated ccTGA. Isolated ccTGA patients were older (51.8 ± 13.1 years) than those with d-TGA (37.5 ± 8.3 years) or complex ccTGA (40.8 ± 13.4 years). There were no differences in sRV or left ventricular size and function across groups. The ccTGA group more commonly had ≥ moderate tricuspid regurgitation than those with d-TGA; ≥ moderate mitral and ≥ moderate pulmonary regurgitation were most prevalent in complex ccTGA. There were no differences in sRV end-diastolic pressure (sRVEDP) or PAWP between groups. However, the ratio of PAWP:sRVEDP was higher in those with d-TGA compared to those with ccTGA. Cardiac index was higher in the d-TGA group than both groups of ccTGA patients with the latter showing higher indices of ventricular afterload. In conclusion, despite sharing a sRV, adults with d-TGA and ccTGA have substantial differences in hemodynamics and structural/valvular abnormalities. Further investigation regarding disease-specific responses to heart failure therapy in those with d-TGA and ccTGA is warranted.

Infant Mitral Valve Replacement: Current State of the Art.

Mitral valve replacement (MVR) in the very young is an imposing clinical challenge. Early and late mortality risk is substantial, severe adverse events are common, and redo mitral valve replacement is inevitable. Therapeutic options are limited. In the older infant with an annulus of 17mm or larger, mechanical MVR is associated with low risk of mortality and predictable durability. For the very young with annular hypoplasia, bovine jugular vein conduit MVR appears to offer equivalent or better early outcomes with the possibility of subsequent valve expansion, potentially prolonging the interval to redo MVR. Experience with cylinder MVR and other forms of surgeon-manufactured MVR is quite limited, and there is currently no information on late outcomes or durability.

Coagulation Tests and Bleeding Classification After Cardiopulmonary Bypass: A Prospective Study.

OBJECTIVE: No recent prospective studies have analyzed the accuracy of standard coagulation tests and thromboelastography (TEG) to identify patients with excessive microvascular bleeding following cardiopulmonary bypass (CPB). The aim of this study was to assess the value of coagulation profile tests, as well as TEG, for the classification of microvascular bleeding after CPB. DESIGN: A prospective observational study. SETTING: At a single-center academic hospital. PARTICIPANTS: Patients ≥18 years of age undergoing elective cardiac surgery. INTERVENTIONS: Qualitative assessment of microvascular bleeding post-CPB (surgeon and anesthesiologist consensus) and the association with coagulation profile tests and TEG values. MEASUREMENTS AND MAIN RESULTS: A total of 816 patients were included in the study-358 (44%) bleeders and 458 (56%) nonbleeders. Accuracy, sensitivity, and specificity for the coagulation profile tests and TEG values ranged from 45% to 72%. The predictive utility was similar across tests, with prothrombin time (PT) (62% accuracy, 51% sensitivity, 70% specificity), international normalized ratio (INR) (62% accuracy, 48% sensitivity, 72% specificity), and platelet count (62% accuracy, 62% sensitivity, 61% specificity) displaying the highest performance. Secondary outcomes were worse in bleeders versus nonbleeders, including higher chest tube drainage, total blood loss, transfusion of red blood cells, reoperation rates (p < 0.001, respectively), readmission within 30 days (p = 0.007), and hospital mortality (p = 0.021). CONCLUSIONS: Standard coagulation tests and individual components of TEG in isolation agree poorly with the visual classification of microvascular bleeding after CPB. The PT-INR and platelet count performed best but had low accuracy. Further work is warranted to identify better testing strategies to guide perioperative transfusion decisions in cardiac surgical patients.

Advocacy at the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery.

The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery (WCPCCS) will be held in Washington DC, USA, from Saturday, 26 August, 2023 to Friday, 1 September, 2023, inclusive. The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery will be the largest and most comprehensive scientific meeting dedicated to paediatric and congenital cardiac care ever held. At the time of the writing of this manuscript, The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery has 5,037 registered attendees (and rising) from 117 countries, a truly diverse and international faculty of over 925 individuals from 89 countries, over 2,000 individual abstracts and poster presenters from 101 countries, and a Best Abstract Competition featuring 153 oral abstracts from 34 countries. For information about the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery, please visit the following website: [www.WCPCCS2023.org]. The purpose of this manuscript is to review the activities related to global health and advocacy that will occur at the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery.Acknowledging the need for urgent change, we wanted to take the opportunity to bring a common voice to the global community and issue the Washington DC WCPCCS Call to Action on Addressing the Global Burden of Pediatric and Congenital Heart Diseases. A copy of this Washington DC WCPCCS Call to Action is provided in the Appendix of this manuscript. This Washington DC WCPCCS Call to Action is an initiative aimed at increasing awareness of the global burden, promoting the development of sustainable care systems, and improving access to high quality and equitable healthcare for children with heart disease as well as adults with congenital heart disease worldwide.

Robotic vs. minimally invasive mitral valve repair: A 5-year comparison of surgical outcomes.

BACKGROUND: Minimally invasive mitral valve repair (MVr) is commonly performed. Data on the outcomes of robotic MVr versus nonrobotic minimally invasive MVr are lacking. We sought to compare the short-term and mid-term outcomes of robotic and nonrobotic MVr. METHODS: We reviewed all patients who underwent robotic MVr (n = 424) or nonrobotic MVr via right mini-thoracotomy (n = 86) at Mayo Clinic, Rochester, MN, from January 2015 to February 2020. Data on baseline and operative characteristics, operative and long-term outcomes were analyzed. Patients were matched 1:1 using propensity scores. RESULTS: Sixty-nine matched pairs were included in the study. The median age was 59 years (interquartile range [IQR]: 54-69) and 75% (n = 103) were male. Baseline characteristics were similar after matching. Robotic and nonrobotic MVr had similar operative characteristics, except that robotic had longer cross-clamp times (57 [48-67] vs. 47 [37-58] min, p < .001) and more P2 resections (83% vs. 68%, p = .05) compared to nonrobotic MVr. There was no difference in operative outcomes between groups. Hospital stay was shorter after robotic MVr (4 [3-4] vs. 4 [4-6] days, p = .003). After a median follow-up of 3.3 years (IQR, 2.1-4.5), there was no mortality in either group, and there was no difference in freedom from mitral valve reoperations between robotic and nonrobotic MVr (5 years: 97.1% vs. 95.7%, p = .63). Follow-up echocardiogram analysis predicted excellent freedom from recurrent moderate-or-severe mitral regurgitation at 3 years after robotic and nonrobotic MVr (90% vs. 92%, p = .18, respectively). CONCLUSIONS: Both short-term and mid-term outcomes of robotic and nonrobotic minimally invasive mitral repair surgery are comparable.

Incidence of Ischemic Optic Neuropathy After Cardiopulmonary Bypass: 20-Year Experience.

OBJECTIVE: This study evaluated the incidence and risk factors for ischemic optic neuropathy (ION) as a complication of cardiac surgery requiring cardiopulmonary bypass (CPB). DESIGN: Retrospective chart review of prospectively collected data at a tertiary care center. SETTING: Single tertiary academic referral center. PARTICIPANTS: This study comprised 44,568 cardiac surgery patients who underwent CPB between January 1, 1995, and January 5, 2017, using the Society of Thoracic Surgeons database and cross-matching it with International Classification of Diseases codes for visual changes. INTERVENTIONS: None; this was a retrospective chart review. MEASUREMENTS AND MAIN RESULTS: Six patients initially were identified as experiencing visual changes. Only 1 patient from 44,568 cardiac surgeries with CPB between January 1, 1995, and January 5, 2017, experienced ION, for an incidence 0.22 per 10,000. Because only 1 patient experienced ION, the authors were unable to determine risk factors for this complication; however, the nadir perioperative hemoglobin in the affected patient was 7.3 g/dL (postoperative). CONCLUSION: The incidence of ION decreased from 6 per 10,000 in the authors' previous study from 1976-1994 to 0.22 per 10,000 in the present study. Because of the low incidence of this dreaded complication, the authors were not able to identify risk factors for ION. Practice improvements (eg, transition to membrane oxygenators, blood transfusion guidelines, less- invasive surgical options for high-risk patients) during the time between the authors' studies likely affected the incidence reduction.

Outcomes of pericardiectomy for constrictive pericarditis following mediastinal irradiation.

BACKGROUND: Pericardiectomy for postradiation constrictive pericarditis has been reported to generally have unfavorable outcomes. This study sought to evaluate surgical outcomes in a large cohort of patients undergoing pericardiectomy for radiation-associated pericardial constriction. METHODS: A retrospective analysis of all patients (≥18 years) who underwent pericardiectomy for a diagnosis of constrictive pericarditis with a prior history of mediastinal irradiation from June 2002 to June 2019 was conducted. There were 100 patients (mean age 57.2 ± 10.1 years, 49% females) who met the inclusion criteria. Records were reviewed to look at the surgical approach, the extent of resection, early mortality, and late survival. RESULTS: The overall operative mortality was 10.1% (n = 10). The rate of operative mortality decreased over the study period; however, the test of the trend was not statistically significant (p = .062). Hodgkin's disease was the most common malignancy (64%) for which mediastinal radiation had been received. Only 27% of patients had an isolated pericardiectomy, and concomitant pericardiectomy and valve surgery were performed in 46% of patients. Radical resection was performed in 50% of patients, whereas 47% of patients underwent subtotal resection. Prolonged ventilation (26%), atrial fibrillation (21%), and pleural effusion (16%) were the most common postoperative complications. The overall 1, 5-, and 10-years survival was 73.6%, 53.4%, and 32.1%, respectively. Increasing age (hazard ratio, 1.044, 95% confidence interval 1.017-1.073) appeared to have a significant negative effect on overall survival in the univariate model. CONCLUSION: Pericardiectomy performed for radiation-associated constrictive pericarditis has poor long-term outcomes. The early mortality, though high (~10%), has been showing a decreasing trend in the test of time.

Impact of postoperative complications after cardiac surgery on long-term survival.

PURPOSE: The impact of postoperative complications on long-term survival is not well characterized. We sought to study the prevalence of postoperative complications after cardiac surgery and their impact on long-term survival. METHODS: Operative survivors (n = 26,221) who underwent coronary artery bypass grafting (CABG) (n = 13,054, 49.8%), valve surgery (n = 8667, 33.1%) or combined CABG and valve surgery (n = 4500, 17.2%) from 1993 to 2019 were included in the study. Records were reviewed for postoperative complications and long-term survival. Propensity-match analysis was performed between patients who did and did not have a postoperative complication. The associations between postoperative complications and survival were assessed using a Cox-proportional model. RESULTS: Complications occurred in 17,463 (66.6%) of 26,221 operative survivors. A total of 17 postoperative complications were analyzed. Postoperative blood product use was the commonest (n = 12,397, 47.3%), followed by atrial fibrillation (n = 8399, 32.0%), prolonged ventilation (n = 2336, 8.9%), renal failure (n = 870, 3.3%), reoperation for bleeding (n = 859, 3.3%) and pacemaker/ICD insertion (n = 795, 3.0%). Stroke (hazard ratio [HR]: 1.55; 95% confidence interval [CI]: 1.36-1.77), renal failure (HR: 1.45; 95% CI: 1.33-1.58) and pneumonia (HR: 1.23; 95% CI: 1.11-1.36) had the strongest impact on long-term survival. Long-term survival decreased as the number of postoperative complications increased. CONCLUSIONS: Postoperative complications after cardiac surgery significantly impact outcomes that extend beyond the postoperative period. Stroke, renal failure, and pneumonia are particularly associated with poor long-term survival.

Sequential Minimally Invasive Fetal Interventions for Two Life-Threatening Conditions: A Novel Approach.

INTRODUCTION: In utero interventions are performed in fetuses with "isolated" major congenital anomalies to improve neonatal outcomes and quality of life. Sequential in utero interventions to treat 2 anomalies in 1 fetus have not yet been described. CASE PRESENTATION: Here, we report a fetus with a large left-sided intralobar bronchopulmonary sequestration (BPS) causing mediastinal shift, a small extralobar BPS, and concomitant severe left-sided congenital diaphragmatic hernia (CDH). At 26-week gestation, the BPS was noted to be increasing in size with a significant reduction in right lung volume and progression to fetal hydrops. The fetus underwent ultrasound-guided ablation of the BPS feeding vessel leading to complete tumor regression. However, lung development remained poor (O/E-LHR: 0.22) due to the left-sided CDH, prompting fetal endoscopic tracheal occlusion therapy at 28-week gestation to allow increased lung growth. After vaginal delivery, the newborn underwent diaphragmatic repair with resection of the extralobar sequestration. He was discharged home with tracheostomy on room air at 9 months. DISCUSSION/CONCLUSION: Sequential in utero interventions to treat 2 severe major anomalies in the same fetus have not been previously described. This approach may be a useful alternative in select cases with otherwise high morbidity/mortality. Further studies are required to confirm our hypothesis.

Transcatheter closure of coronary artery fistula: A 21-year experience.

OBJECTIVES: To evaluate the outcomes of transcatheter coronary artery fistula (CAF) closure and to identify anatomic/procedural factors that may impact outcomes. BACKGROUND: Due to the rarity of CAF, reported experience with transcatheter closure remains limited and anatomic and procedural factors that may lead to unsuccessful closure, complications, or recanalization of CAF are unclear. METHODS: All patients who underwent transcatheter CAF closure at Mayo Clinic from 1997 to 2018 were retrospectively reviewed. CAF anatomic characteristics, procedural techniques, and clinical/angiographic outcomes were assessed. RESULTS: A total of 45 patients underwent transcatheter closure of 56 CAFs. The most commonly used devices were embolization coils in 40 (71.4%) CAFs, vascular occluders in 10 (17.8%), or covered stent in 2 (3.6%). Acute procedural success with no or trivial residual flow occurred in 50 (89.3%) CAFs. Residual flow was small in three (5.4%) and large in three (5.4%). Eight (17.8%) patients had complications, including device migration in three, intracranial hemorrhage from anticoagulation in one, and myocardial infarction (MI) in four. MI was a result of covered stent thrombosis or stagnation of flow after closure of large distal CAF. Twenty-two patients with 27 CAFs had follow-up angiography after successful index procedure at median time of 423 (IQ 97-1348) days. Of these, 23 (85.2%) had no/trace flow and 4 had large flow from recanalization. CONCLUSIONS: Transcatheter CAF closure is associated with a favorable acute procedural success and complication rate in selected patients. Procedural success and risk for complication are highly dependent on CAF anatomy and closure technique.

Thrombin Generation and Antithrombin Activity in Infants Undergoing Cardiopulmonary Bypass-An Exploratory Study.

OBJECTIVES: Coagulopathic bleeding is a major complication of pediatric cardiac surgery. Investigating perioperative dynamics of thrombin generation and antithrombin (AT) activity might provide more insight into the underlying mechanisms of coagulopathy. This can help develop a targeted hemostatic approach in the future. The authors hypothesized that there is a decline in both thrombin generation and AT activity in infants undergoing cardiopulmonary bypass (CPB). DESIGN: Prospective observational study. SETTING: Single academic medical center. PARTICIPANTS: Infants <10 kg of weight undergoing cardiac surgery with CPB. INTERVENTIONS: Blood specimen collection and testing. MEASUREMENTS AND RESULTS: The authors performed assays of thrombin generation and AT activity on the samples of platelet-poor plasma of 25 infants, repeating them at 3 points: before CPB and heparinization, after separation from CPB and protamine administration, and after chest closure. The authors observed a statistically significant decline in thrombin generation shortly after separation from CPB compared with baseline. The geometric mean for lag time was prolonged (4.0 v 5.5 minutes, p = 0.013), and peak thrombin and the net amount of generated thrombin declined almost 3-fold (80.7 v 25.1 nmol, p < 0.001; 1264 v 476 nmol, p < 0.001, respectively). This was accompanied by a decline in AT activity (59.8 v 50.1, p = 0.001). After platelet and cryoprecipitate transfusion, at the case conclusion AT activity had recovered marginally (59.8 v 55.4, p = 0.042), but thrombin generation remained reduced. CONCLUSIONS: In pediatric patients <10 kg undergoing cardiac surgery with CPB, thrombin generation and AT activity decline and do not recover completely after transfusion of platelets and cryoprecipitate.

Intraoperative Plasma Transfusion Volumes and Outcomes in Cardiac Surgery.

OBJECTIVE: Assess outcomes after intraoperative plasma transfusion in patients undergoing cardiac surgery. DESIGN: Retrospective study of adult cardiac surgical between 2011 and 2015. Relationships between plasma transfusion volume, coagulation test values, and a primary outcome of early postoperative red blood cell (RBC) transfusion were assessed via multivariable regression analyses. Secondary outcomes included hospital mortality, intensive care unit and hospital-free days, intraoperative RBCs, estimated blood loss, and reoperation for bleeding. SETTING: Academic tertiary referral center. PARTICIPANTS: A total of 1,794 patients received intraoperative plasma transfusions during the study period. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Higher plasma transfusion volumes were associated with worse clinical outcomes, with each 1-unit increase being associated with greater odds for postoperative RBCs [odds ratio (OR) 1.12 (confidence interval [CI] 1.04-1.20); p = 0.002], intraoperative [OR 1.85 (CI 1.69-2.03); p < 0.001], and fewer hospital-free days [mean -0.20 (-0.39, -0.01); p = 0.04]. Each 0.1 increase in pretransfusion International Normalized Ratio (INR) was associated with increased odds of postoperative and intraoperative RBCs, reoperation for bleeding, and fewer intensive care unit and hospital-free days. For given plasma volumes, patients achieving greater reduction in elevated pretransfusion INR values experienced more favorable outcomes. CONCLUSIONS: In patients undergoing cardiac surgery who received intraoperative plasma transfusion, higher plasma transfusion volumes were associated with inferior clinical outcomes. Higher pretransfusion INR values also were associated with worse outcomes; however, those achieving a greater degree of INR correction after plasma transfusion demonstrated more favorable outcomes. Prospective studies related to plasma transfusion are needed to address this important topic.

Placement of Reveal LINQ Device in the Left Anterior Axillary Position.

Implantable loop recorders (ILR) are utilized for long-term rhythm monitoring. Typical placement of the Medtronic Reveal LINQ along the left parasternal border may compromise the quality and/or feasibility of future imaging studies. We sought to evaluate the utility of placing an ILR in the left anterior axillary position and the impact on the quality of cardiac imaging. We reviewed patients from May 2017 to June 2018 who had placement of a Reveal LINQ device in the left anterior axillary position. Demographic, procedural, and clinical data were collected via retrospective review. Cardiac magnetic resonance imaging (MRI) studies were reviewed for image quality after ILR placement. Eight patients met inclusion criteria for this study (median age 6 years, 50% female). Six patients (75%) had an ILR placed in the operating room, while all others were placed in the electrophysiology lab. All patients demonstrated acceptable R waves for diagnostic evaluation (median = 0.85 mV, range 0.24-1.7 mV). Cardiac MRI was obtained in 7 patients following ILR placement with diagnostic image quality and no adverse events. One device was explanted 28 days after placement due to concern for possible infection. No other devices required removal or revision (median follow up duration 11 months, IQR 8-13.5). ILR placement in the left anterior axillary position can record adequate signals in pediatric patients. In addition, axillary ILR device position may allow for completion of cardiac imaging, particularly cardiac MRI, without significant artifacts which is critical for patients with congenital heart disease.

Melody valve in mitral position: Complete fracture causing acute mitral stenosis in a child.

We present a case of a 7-year-old boy with a history of multiple mitral valve interventions and subsequent Melody valve placement in the mitral position, who presented with acute mitral stenosis due to complete fracture of the Melody stent. He was born early with severe mitral and tricuspid insufficiency due to valvular dysplasia, and ended up with 4 sternotomies before the age of 2 due to mitral valve dysfunction and recurrent prosthetic valve thrombosis. He then developed mixed stenosis and regurgitation at age 6, and to avoid another sternotomy, valve-in-valve therapy with off-label use of a 20-mm Melody valve was done with hybrid procedure via trans-apical approach. Eight months later he presented with acutely worsened mitral stenosis (mean gradient 20 mm Hg), due to fracture of the proximal stent. While the safety and efficacy of the Melody valve has been well established especially in the pulmonary position, stent fracture is a known and potentially serious complication. As with any novel valve therapy, close follow-up and frequent imaging may be warranted to watch for loss of stent integrity, particularly if clinical symptoms of valve dysfunction occur.

Current Interventional and Surgical Management of Congenital Heart Disease: Specific Focus on Valvular Disease and Cardiac Arrhythmias.

Successful outcome in the care of patients with congenital heart disease depends on a comprehensive multidisciplinary team. Surgery is offered for almost every heart defect, despite complexity. Early mortality for cardiac surgery in the neonatal period is ≈10% and beyond infancy is <5%, with 90% to 95% of patients surviving with a good quality of life into the adult years. Advances in imaging have facilitated accurate diagnosis and planning of interventions and surgical procedures. Similarly, advances in the perioperative medical management of patients, particularly with intensive care, has also contributed to improving outcomes. Arrhythmias and heart failure are the most common late complications for the majority of defects, and reoperation for valvar problems is common. Lifelong surveillance for monitoring of recurrent or residual structural heart defects, as well as periodic assessment of cardiac function and arrhythmia monitoring, is essential for all patients. The field of congenital heart surgery is poised to incorporate new innovations such as bioengineered cells and scaffolds that will iteratively move toward bioengineered patches, conduits, valves, and even whole organs.

Exercise Capacity After Repair of Ebstein Anomaly in Adults.

Repair of Ebstein anomaly has evolved over the last decade, and timing of repair remains variable. There have been no studies of exercise or functional capacity in patients who have had tricuspid valve surgery for Ebstein anomaly in adulthood. We aimed to compare exercise capacity before and after tricuspid valve repair or replacement for Ebstein anomaly in adults at Mayo Clinic. We performed a retrospective chart review of all patients with Ebstein anomaly who underwent tricuspid valve surgery at Mayo Clinic between June 2007 and January 2015. We compared pre- and postoperative echocardiograms, exercise tests, and clinic visits. Tricuspid valve surgery was done for 322 patients, and 32 patients met criteria of native tricuspid valve repair or replacement at age 18 or older and had maximal pre- and postoperative exercise tests. Nineteen patients had valve repair, and 13 had valve replacement. Surgery for Ebstein anomaly resulted in significant reduction in tricuspid regurgitation and right ventricular size. There was a significant improvement in NYHA functional class after surgery; however, there was no significant improvement in functional aerobic capacity (FAC), metabolic equivalents (METs), exercise time, or [Formula: see text] after surgery. Patients who had an atrial shunt closed during surgery had improved minimum blood oxygen saturations during exercise, though no improvement in exercise capacity. In our cohort, patients who had tricuspid valve repair or replacement for Ebstein anomaly reported an improvement in functional capacity; however, this did not reflect improvement in measured exercise capacity, despite excellent surgical results by echocardiography.