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Whereas the prevalence and impact of atrioventricular valve (AVV) regurgitation in patients with single ventricle physiology has become increasingly apparent, the optimal timing for valve intervention is unclear. To investigate this, we performed a retrospective review of all 1,167 patients from the Mayo Clinic Fontan database. Thirteen percent (153 patients) had AVV repair or replacement during their staged single ventricle palliation. We found that patients with right ventricular morphology and common AVV were at increased risk for AVV intervention. Patients who underwent AVV intervention had increased risk of death/transplant compared with those who did not (hazards ratio [HR] = 1.75, 95% CI 1.37 to 2.23, p <0.001). With respect to valve intervention timing, whereas AVV intervention before Fontan presented similar risk for death/transplant compared with no AVV intervention (HR = 0.85, 95% CI 0.32 to 2.27, p = 0.74), intervention at time of Fontan had a significantly higher risk (HR = 1.46, 95% CI 1.09 to 1.97, p = 0.01), and intervention after Fontan had a much more substantial risk (HR = 3.83, 95% CI 2.54 to 5.79, p <0.001). AVV repair failure occurred in 11% of patients. In terms of relative risk of valve repair versus replacement, in post-Fontan AVV intervention patients, AVV replacement carried a 2.9 fold risk of death/transplant compared with AVV repair. In conclusion, AVV disease remains a considerable challenge for durable Fontan physiology. This data demonstrates that earlier intervention on valve pathology improves survival with the Fontan circulation. Continued surveillance of single ventricle patients and prompt referral of those with valve pathology can improve outcomes in this challenging population., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: Lambl's excrescences (LE) are fibrous extensions that can be found along the lines of closure of the aortic valve. Due to improvements in ultrasound technology, LE are frequently imaged during transthoracic echocardiography (TTE) in adults., Objective: The purpose of this study was to determine the prevalence of LE among children from two eras (2004-2006 and 2011-2012) and the effect of technological advancements on LE detection., Methods: TTE from 700 subjects (age 18 years old or younger) were reviewed. All parasternal long and short axis images of the aortic valve were reviewed by a board certified echocardiographer, and the positive studies were then reviewed by two additional observers to confirm the presence of LE. A two-sample t test with 95% significance was used to analyze the presence of LE in the cohorts. Median follow-up duration was 66 months., Results: Of the 700 subjects, 12 (1.7%) children were found to have LE. No significant difference in prevalence was found between the two eras (.9% vs. 2.6%, P = .08) and the presence of LE was not related to age (P = .36). The youngest subject with an LE was 5 months old. During long-term follow-up there were no clinical events in the 12 children identified with a LE., Conclusions: The prevalence of LE in children is lower than that reported in adults, this supports the age-related "wear and tear" process that has been described in previous studies. LE do not require intervention or more aggressive invasive imaging in children., (© 2017 Wiley Periodicals, Inc.)

Background: There are limited long-term, single-cohort, follow-up studies available about patients after the Fontan operation., Objectives: This study sought to determine the long-term outcome of all patients who had a Fontan operation at the Mayo Clinic., Methods: Records of all patients who had a modified Fontan operation between 1973 and 2012 were reviewed. A follow-up questionnaire was mailed to all patients alive at the time of the study., Results: Overall, 10-, 20-, and 30-year survival for 1,052 patients was 74%, 61%, and 43%, respectively. Factors associated with decreased overall or late survival in multivariate analysis included pre-operative diuretic use, longer cardiopulmonary bypass time, operation prior to 1991, atrioventricular valve (AVV) replacement at the time of Fontan operation, elevated post-bypass Fontan (>20 mm Hg) or left atrial (>13 mm Hg) pressures, prolonged chest tube drainage (>21 days), post-operative ventricular arrhythmias, renal insufficiency, and development of protein-losing enteropathy (PLE). Pre-operative and intraoperative sinus rhythm were associated with improved survival. Long-term survival was similar for patients regardless of ventricular morphology. The most common reoperations were pacemaker insertion/revision in 212 patients (20%), Fontan revision/conversion in 117 patients (11%), and AVV repair/replacement in 66 patients (5%). Clinically significant late atrial or ventricular arrhythmias occurred in 468 patients (44%). Ninety-five patients (9%) developed PLE, and 5-, 10-, and 20-year survival after diagnosis of PLE was 50%, 35%, and 19%, respectively., Conclusions: As the surgical techniques for the Fontan operation have changed over the last 40 years, survival has improved. However, development of PLE and arrhythmias and the need for reoperation during long-term follow-up pose significant management challenges., (Copyright © 2015 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objective: After Fontan operation, hemodynamically significant veno-venous collateral (VVC) vessels can lead to systemic arterial desaturation. Outcomes after embolization of VVCs have not been determined. We sought to determine the frequency of and outcomes for patients undergoing VVCs embolization after Fontan operation., Design: We retrospectively analyzed clinical and hemodynamic data of patients who underwent cardiac catheterization after the Fontan operation from 1995 to 2014 at Mayo Clinic. Clinical, imaging, and hemodynamic data from patients with VVCs were compared based on intervention (embolization) vs. nonintervention., Results: Four hundred ninety-six patients with prior Fontan operation had catheterization; 109 VVCs were identified in 72 patients (37 males, mean age 26 ± 12 years). Embolization was performed in 31/72 patients (43%). Following embolization, no improvement was demonstrated in oxygen saturation or hemoglobin concentration at ambulatory follow-up (2.5 ± 2 years), and cardiac index declined at follow-up catheterization (5.6 ± 3 years). Overall, 15 patients (21%) died at a mean of 2.8 ± 4 years after embolization. Fifty percent of deaths after embolization occurred in patients with Fontan pressures ≥18 mm Hg. Five-year survival of patients with VVCs undergoing embolization was 74% compared with 92% in those patients who did not undergo embolization (P < .01). In multivariate analysis, significant predictors of death on follow-up were embolization [hazard ratio (HR) = 9.3 (95% confidence interval [CI], 2.8-42), P = .0001], atrio-pulmonary Fontan (HR = 4.2 [95% CI, 1.4-15], P = .01), and heterotaxy (HR = 3.7 [95% CI, 1.0-15], P = .05)., Conclusion: We observed decreased 5-year survival in patients who had VVC embolization. Embolization of VVC in patients after Fontan should be evaluated very carefully in patients with atrio-pulmonary type Fontan, heterotaxy, and those with Fontan pressure above 18 mm Hg. These patients may benefit from the "natural" fenestration that VVCs provide., (© 2015 Wiley Periodicals, Inc.)

Surgical repair of partial atrioventricular septal defects (AVSD) has been successful for more than 60 years. However, recent data from the Pediatric Heart Network show that 31% of patients have moderate or severe left atrioventricular valve regurgitation (LAVVR) at follow-up. Previously, our institution found that only 9% of patients had more than moderate LAVVR at the last follow-up. Our objective was to determine the long-term outcomes after repair of partial AVSD in the current era. We reviewed all patients with partial AVSD who had primary biventricular repair from January 1995 to June 2011 at our institution. The Kaplan-Meier method was used to estimate the survival free of an event, and factors were evaluated for an association with each outcome using the log-rank test. All 105 patients with partial AVSD who had surgery during this period were evaluated. The median age at surgery was 7.9 years. The overall survival rate at 1 year was 97%. Median follow-up was 5.3 years (interquartile range 1.7 to 11.1). At 3 years, the survival rate free from reoperation was 89%. Thirteen patients required reoperations with the most common reason being LAVVR. A total of 10 patients developed more than moderate LAVVR with a cumulative incidence of 8% by 2 years. The discrepancy with the Pediatric Heart Network data may be due to the later age of operation for patients in our cohort suggesting that elective repair of partial AVSD should be deferred until children are somewhat older (ages 5 to 8 years). Neither patient age (p = 0.11) nor severity of preoperative LAVVR (p = 0.16) were identified as statistically significant risk factors. In conclusion, there is less morbidity and mortality after surgical repair for partial AVSD., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Objective: Percutaneous coronary intervention (PCI) is commonly used in adult patients with coronary artery disease, but data on PCI in children and adolescents remain limited. Herein, we describe our experience with the use of PCI in pediatric and adolescent patients., Design: This is a retrospective review., Setting: The study was conducted at Mayo Clinic, Rochester, MN., Patients: All patients ≤18 years old who underwent PCI from 2004 to 2012 were included., Interventions: Intravascular ultrasound and balloon angioplasty were performed in all patients, with subsequent stent placement in 6/7 (86%) procedures., Outcome Measures: Residual postintervention stenosis, early procedural morbidity and mortality were the outcome measures., Results: Overall, seven unique PCI procedures were performed in five patients (four males, mean age 13.2 ± 3.8 years, range 8-18 years). Mean follow-up interval was 2 (0.6-5.5) years. Indications for the procedures included transplant coronary vasculopathy (n = 1), coronary dissection (n = 1), and acute coronary thrombosis/myocardial infarction (n = 1). Additionally, there were two patients (n = 2) who experienced coronary compression as a sequelae of prior heart surgery. Intravascular ultrasound and balloon angioplasty were performed in all patients, with subsequent stent placement in 6/7 (86%) procedures. A total of eight stents were placed (average stent diameter 3 ± 0.5 mm), including six (75%) drug-eluting stents. The targeted coronary artery lesions were successfully treated in all seven procedures. There was no early procedural morbidity or mortality. Two patients were noted to have angiographic evidence of in-stent restenosis at 3 and 15 months postdeployment, respectively, despite treatment with aspirin and clopidogrel., Conclusion: PCI in children and adolescents can be utilized to improve coronary blood flow in a variety of clinical situations. It may be particularly effective in cases of postsurgical coronary compression. Close angiographic follow-up is critical as these patients are at risk for in-stent restenosis., (© 2013 Wiley Periodicals, Inc.)

Objectives: To compare procedural outcomes of transcatheter balloon pulmonary valvuloplasty (BPV) in adults to a concurrent pediatric cohort and to identify whether BPV leads to improvement in symptoms among adults., Background: BPV has long been utilized to relieve right ventricular outflow tract (RVOT) obstruction in neonates and young children. More recently, BPV has been performed in adults, but outcome data are limited in this population., Methods: We reviewed all cases of BPV performed at Mayo Clinic's Congenital Cardiac Cath Lab from December 1993 to July 2012. Patients were excluded if they had additional congenital heart defects, other than simple left-to-right shunt lesions., Results: 89 children and 40 adults underwent 132 BPV procedures. Among the adults, 78% were symptomatic at the time of BPV. The most common symptoms were dyspnea and fatigue. RVOT systolic gradient was 42 ± 19 mmHg (children) and 54 ± 26 mmHg (adults) prior to BPV (P = 0.002). Adults experienced a greater reduction in gradient following BPV (32 ± 21 mmHg vs. 23 ± 19 mmHg; P = 0.007). No patients exhibited "suicide right ventricle" physiology after BPV. Among adults, mean follow-up was 1.9 years (30 days-11 years). Symptoms improved in 20 of the 24 previously symptomatic adults for whom follow-up data were available. Mean NYHA score improved from 2.2 to 1.4 following BPV. At last follow-up, 52% of adults were free of any symptoms, a significant improvement from pre-BPV (P = 0.0003). Only one patient described worsening of symptoms after BPV., Conclusions: BPV is an effective procedure for symptomatic adults with pulmonary valve stenosis. In addition to immediate relief of RVOT gradient, adult patients may experience symptomatic improvement following BPV., (Copyright © 2013 Wiley Periodicals, Inc.)

Objective: To determine the epidemiology of infective endocarditis (IE) presenting in pediatric patients during a 60-year period at our institution., Patients and Methods: In this retrospective medical record review, we extracted demographic characteristics, diagnostic variables, and outcomes for patients less than 20 years of age diagnosed with IE from January 1, 1980, to June 30, 2011. We compared this cohort with a previously reported cohort of pediatric patients with IE from our institution diagnosed from 1950 to 1979., Results: We identified 47 patients (24 males; mean ± SD age at diagnosis, 12.3±5.5 years [range, 1 day to 18.9 years]) who had 53 episodes of IE. The most common isolated organisms were viridans streptococci (17 of 53 episodes [32%]) and Staphylococcus aureus (12 of 53 episodes [23%]). Of the 47 patients, 36 (77%) had congenital heart disease, 24 of whom had cardiac surgery before their first episode of IE (mean ± SD time to IE diagnosis after surgery, 4.2±3.2 years [range, 64 days to 11.3 years]). Fourteen patients (30%) required valve replacement because of valvular IE, and 16 (34%) had complications, including mycotic aneurysm, myocardial abscess, or emboli. Vegetations were identified using echocardiography in 37 of the 53 unique episodes of IE (70%). Endocarditis-related mortality occurred in 1 patient. Compared with the historical (1950-1979) cohort, there were no differences in patient demographic characteristics, history of congenital heart disease, or infecting organisms. One-year mortality was significantly lower in the modern cohort (4%) compared with the historical cohort (38%) (P<.001)., Conclusion: Most pediatric episodes of IE occur in patients with congenital heart disease. Mortality due to endocarditis has decreased in the modern era., (Copyright © 2012 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved.)

Objective: We reviewed our experience with the single-patch, 2-patch, and caval division techniques for repair of a partial anomalous pulmonary venous connection., Methods: From 1990 to 2009, 124 patients (65 male patients; 52%) were identified. The single-patch, two-patch, and caval division technique was used in 60 (49%), 24 (19%), and 40 (32%) patients, respectively. The median age was 34.5 years (range, 1-76). Of the 124 patients, 99 (80%) had an atrial septal defect., Results: There was no early mortality. Survival at 5, 10, and 15 years was 99%, 95%, and 82%, respectively. One patient (3%) in the caval division group required early reoperation for superior vena caval obstruction. All patients, exept for 3, had regained sinus rhythm at discharge. Late superior vena caval obstruction developed in 3 patients (7.5%) in the caval division group, 3 (5%) in the single-patch group, and 1 (4%) in the 2-patch group (Р = .5). Two patients underwent reoperation for late pulmonary vein obstruction: 1 (2%) in the single-patch group and 1 (4%) in the 2-patch group (P = .08). A permanent pacemaker was required in 3 patients (3%)., Conclusions: Surgical treatment of partial anomalous pulmonary venous connections is associated with excellent outcomes. The overall incidence of late superior vena caval or pulmonary vein stenosis is low. Although not significant, the 2-patch technique might be associated with a greater incidence of sinus node dysfunction and late pulmonary venous stenosis. The late development of superior vena caval obstruction is a concern with all techniques, necessitating close follow-up., (Copyright © 2012 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objectives: To compare pre- and post-procedure electrocardiograms (ECGs) in a large cohort of patients after percutaneous closure of atrial septal defect (ASD) and patent foramen ovale (PFO)., Background: Percutaneous device closure of ASD or PFO is commonplace. Conduction and rhythm anomalies associated with percutaneous device placement have been reported., Methods: We reviewed records for all patients who underwent percutaneous device closure of ASD or PFO at our institution from 1999 to 2008. Pre-procedure ECG and Holter studies were compared to available short term (<2 months after placement) and intermediate follow-up (>2 months) ECG or Holter., Results: Pre- and post-procedural ECGs were available in 610 patients (305 females, average age 50 ± 18.1 years, range 1-91 years, 384 PFO, 184 ASD, 42 with multiple defects, mean device size 16 mm, range 5-38 mm). We report an incidence of 5.2% (32/610) of arrhythmias in the 4 months following device placement, including 29 patients with atrial tachyarrhythmias (ATs, 22 fibrillation, 7 flutter), 1 with junctional tachycardia, and 2 with heart block. Among other findings, the average P-wave duration was increased on intermediate follow-up as compared to early follow-up (P < 0.001). Development of new-onset 1st degree AV Block after the procedure was associated with an increased risk of ATs post-procedure (P < 0.0001)., Conclusion: We report a low risk of clinically significant post-procedure arrhythmias after device placement. Clinically significant heart block occurred in only two patients (0.3%). Changes in several markers of atrial conduction were found, suggesting an effect of device closure on intra-atrial conduction., (Copyright © 2011 Wiley-Liss, Inc.)

Objectives: The objective of this study was to review the long-term functional outcome of patients with Ebstein anomaly who had cardiac operation at our institution., Background: Ebstein anomaly is a spectrum of tricuspid valvular and right ventricular dysplasia. Many patients will require operation in an attempt to improve quality of life., Methods: From April 1, 1972, to January 1, 2006, 539 patients with Ebstein anomaly underwent 604 cardiac operations at the Mayo Clinic in Rochester, Minnesota. Patient records were reviewed, and all patients known to still be alive were mailed a medical questionnaire or contacted by telephone., Results: At the initial operation at our institution, the mean age of the patients was 24 years (range 8 days to 79 years) and 53% were female patients. Survival at 5, 10, 15, and 20 years was 94%, 90%, 86%, and 76%, respectively. Survival free of late reoperation was 86%, 74%, 62%, and 46% at 5, 10, 15, and 20 years, respectively. Surveys were returned by 285 of 448 (64%) patients known to be alive at the time of this study. Two hundred thirty-seven (83%) patients were in New York Heart Association functional class I or II, and 34% were taking no cardiac medication. One hundred three patients (36%) reported an incident of atrial fibrillation or flutter, 5 patients (2%) reported having had endocarditis, and 1 patient (<1%) reported having a stroke. There were 275 pregnancies among 82 women. The recurrence of congenital heart disease was reported in 9 of 232 (3.9%) liveborn children., Conclusions: Patients have good long-term survival and functional outcomes after undergoing surgery for Ebstein anomaly. Atrial arrhythmias are common both before and after surgery. Many patients have had one or more successful pregnancies with a low-recurrence risk of congenital heart disease.

The objective of this study was to assess growth patterns of hyperlipidemic children enrolled in a preventive cardiovascular health clinic. A retrospective chart review of hyperlipidemic children enrolled in the Mayo Clinic Cardiovascular Health Clinic for the Young was performed. All participants were counseled to eat an American Heart Association Step-One Diet and exercise regularly. Weight and height were measured every 3 months. Growth was assessed using attained heights and weights and body-mass index Z scores compared to standard distributions for North American children. Sixty-three patients (33 males and 30 females) were enrolled in the study. Mean age at clinic entrance was 7.8 +/- 3.5 years (range: 2 to 16 years). We conclude that participation in a preventive health clinic is generally safe for hyperlipidemic children. However, medical management of hyperlipidemic children must include meticulous surveillance to detect the infrequent occurrence of excessive weight loss or weight stabilization resulting from inappropriate response to dietary counseling.