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Background: Stenosis of the venous connections and conduits is a well-known late complication of the Fontan procedure. Currently, data on the outcomes of percutaneous intervention for the treatment of extra- or intracardiac conduits and lateral tunnel baffles obstruction are limited. In an attempt to better define the nature and severity of the stenosis and the results of catheter interventional management, we reviewed Fontan patients with obstructed extra- or intracardiac conduits and lateral tunnel baffles., Methods: Retrospective review of all Fontan patients who had cardiac catheterization from January 2002 to October 2018 was performed. Hemodynamic and angiographic data that assessed extra- or intracardiac conduit, or lateral tunnel baffle obstruction/stenosis were evaluated., Results: Twenty patients underwent catheter intervention because of conduit stenosis, including calcified homografts, stenotic Gore-Tex conduits and obstructed lateral tunnels. Six other patients had Fontan obstruction but were referred for surgical revision. After stenting, there was a significant reduction in the connection gradient [2.0 mm Hg (IQR 2; 3) vs 0 mm Hg (IQR 0; 1), P < .0001]. Fontan conduit/connection diameter increased [10.5 mm (IQR 9; 12) vs 18 mm (IQR 14.9; 18); P < .0001] and New York Heart Association class [III (IQR II; III) vs I (IQR II; III); P = .03) with stent placement., Conclusions: We demonstrated the hemodynamics and angiographic subtypes of conduit stenosis in patients after Fontan, We showed that calcified homografts, stenotic Gore-Tex conduits and lateral tunnels pathways can be safely and effectively stented to eliminate obstruction. Percutaneous stenting is associated with a decrease in connection gradients and improvement in functional capacity., (© 2019 The Authors. Congenital Heart Disease Published by Wiley Periodicals, Inc.)

Background: The transapical approach has been utilized in adult HCM patients with either midventricular obstruction or a small LV cavity; however, there are little data on its use in children., Methods: We retrospectively reviewed all patients (age <21 years) with HCM who underwent a transapical myectomy from January 2002 to December 2016. Indication for surgery was midventricular obstruction in 19/23 (83%) and small LV cavity in 4 (17%). Preoperative symptoms included: dyspnea (96%), chest pain (65%), presyncope (61%), and syncope (35%). The mean age at the time of operation was 14 ± 4.0 years (range, 4-20)., Results: Overall, 23 patients (12 males) underwent transapical myectomy. A concomitant transaortic approach was performed in 16/19 (84%) with obstruction. The intraventricular gradient decreased from 71 mm Hg (IQR 44-92 mm Hg) preoperatively to 18 mm Hg (IQR 8-34 mm Hg, P < .0001) after myectomy. In patients with a small LV cavity, the mean left ventricular end diastolic dimension (LVEDD) increased from 40 ± 3 mm to 46 ± 3 mm (P = .05) after myectomy. There were no early deaths. Postoperative morbidity included complete heart block in 3 patients, 2 of which required pacemakers. Median follow up was 3.5 years (IQR 1.6-5.6). Symptoms improved in 95% of patients; the number of patients in NYHA class 3 or 4 heart failure decreased from 10/23 (43%) preoperatively to 3/23 (13%) postoperatively (P < .0001). Overall survival at 5 years postsurgery was 100%. Transplant-free survival was 91% and 87% at 1 and 5 years, respectively., Conclusion: In children with HCM, transapical myectomy is an effective adjunct to a transaortic approach to abolish midventricular obstruction and it effectively increases LV stroke volume in patients with small LV cavities and nonobstructive HCM. It may be beneficial for these patients with significant symptoms and who have failed medical therapy as a treatment alternative to cardiac transplantation., (© 2018 Wiley Periodicals, Inc.)

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: The increased incidence of preoperative and postoperative arrhythmia in Ebstein anomaly (EA) prompted some clinicians to perform an electrophysiology study (EPS) in all patients prior to surgery for EA. The cone repair (CR) is the current surgical option of choice for most young patients with EA but the effect of the CR on arrhythmia is not well established., Objectives: To assess the burden of arrhythmia in young patients after CR and to assess the utility of selective preoperative EPS., Materials and Methods: A retrospective review of all patients <21 years of age with EA who had a CR at Mayo Clinic from June 2007 to December 2015 was performed. Surveys were mailed and telephone calls were made to all individuals to assess antiarrhythmic medication use and EP/device procedures performed after CR., Results: There were 143 patients; median age, 10 years (0.1-20.9 years). Thirty-five (24%) patients had a preoperative EPS of which 26 (18%) had a preoperative ablation. Indications for EPS were Wolff-Parkinson-White (WPW), documented arrhythmia, or suspected arrhythmia. Posthospital discharge data were available for 140 (98%) patients. Mean follow-up was 2.9 years (0.1-9.2 years). At follow-up, 7 (5%) patients were receiving antiarrhythmic medications. After CR, only 3 (2%) patients who did not have a preoperative EPS have required an ablation., Conclusions: The risk of arrhythmia after CR for EA in young patients is very low when a preoperative EPS is limited to those with WPW, known arrhythmia, or suspected arrhythmia. In smaller patients, it may be reasonable to defer the EPS., (© 2018 Wiley Periodicals, Inc.)

Background: Bicuspid aortic valve (BAV) occurs both as an isolated cardiac lesion and in association with congenital heart defects (CHD). Their aim was to identify the incidence and morphology of BAV in patients with coexisting CHD and compare their disease progression to patients with isolated BAV., Methods: The Mayo Clinic echocardiography database was retrospectively analyzed to identify pediatric and young adult patients (≤22 years) who were diagnosed with BAV from 1990 to 2015. The morphology of BAV was determined from the echocardiographic studies before any intervention., Results: Overall, 1010 patients with BAV were identified, 619 (61%) with isolated BAV and 391 (39%) with BAV and coexisting CHD. The incidence of BAV was highest in patients with coarctation of the aorta (36%) and interrupted aortic arch (36%). In comparison to patients with isolated BAV, patients with BAV and left-sided obstructive lesions more frequently had right-left cusp fusion (P = .0001). BAV in patients with right-sided obstructive lesions was rare, but they more frequently had right-noncoronary or left-noncoronary cusp fusion (P = .01). No significant progression of aortic stenosis or regurgitation was observed in patients with BAV and coexisting CHD; however in patients with isolated BAV the severity of aortic regurgitation increased with age. In patients with isolated BAV, the ascending aorta diameter (z-score) increased with age, peaked around 8-9 years of age, and was larger in comparison to patients with BAV and coexisting CHD. The sinus of Valsalva diameter (z-score) in patients with BAV and ventricular septal defect was larger than isolated BAV patients after 18 years (P < .04)., Conclusions: The morphology of BAV, the pattern and progression of aortic dilatation, and the severity of aortic valve disease vary in pediatric and young adult patients with BAV and coexisting CHD. However, there was no significant BAV disease progression when associated with these CHD., (© 2016 Wiley Periodicals, Inc.)

The physiological consequences of the Fontan circulation impose risk for hepatic dysfunction and may culminate in hepatic fibrosis, cirrhosis, and hepatocellular carcinoma. Consensus regarding appropriate surveillance modalities to diagnose liver disease in Fontan patients is lacking, in part due to the relative lack of strong evidence and prospective studies in this patient population. The goal of this paper is to critically review the current evidence and provide recommendations for the surveillance of hepatic complications in the post-Fontan patient population., (© 2017 Wiley Periodicals, Inc.)

Objectives: We sought to examine the incidence and predictors of arrhythmias and sudden cardiac death (SCD) after Fontan operation., Background: Arrhythmias and SCD have been reported following operations for congenital heart disease, but the incidence and risk factors have not been well defined in patients after a Fontan operation., Methods: We reviewed records of all patients who had a Fontan operation from 1973 to 2012 (n = 1052) at our institution. A questionnaire was mailed to patients who were not known to be deceased at the initiation of the study. Late arrhythmias were classified as bradyarrhythmias or tachyarrhythmias requiring treatment >30 days after operation., Results: We included 996/1052 (95%) patients with no arrhythmia diagnosis prior to Fontan. Overall 10-, 20-, and 30-year freedom from arrhythmias was 71%, 42%, and 24%, respectively. Of 864 patients who survived >30 days after Fontan, 304 (35%) had atrial flutter, 161 (19%) had atrial fibrillation, 108 (13%) had atrial tachycardia, 37 (4%) had reentrant supraventricular tachycardia, 40 (5%) had ventricular tachycardia, and 113 (13%) had sinus node dysfunction. Predictors of late arrhythmias included an atriopulmonary Fontan, age at operation (>16 years) or atrial arrhythmias postoperatively. During follow-up, 52/1052 (5%) patients had SCD, with 51 having documentation available; 8 patients died suddenly within 30 days and the remaining 43 had an average time to SCD of 6.9 ± 6.7 years (median was 3.8 years). Arrhythmias were documented in 28/43 (65%) patients prior to SCD. Predictors of SCD included atrioventricular valve replacement and post-bypass Fontan pressures >20 mm Hg; preoperative sinus rhythm was protective., Conclusions: Arrhythmias and SCD are significant concerns among Fontan patients and specific risk factors may warrant closer follow-up and earlier consideration for therapy., (© 2016 Wiley Periodicals, Inc.)

Objective: Atrial tachyarrhythmias frequently develop after the Fontan operation. Patients with Fontan physiology rely on atrial contribution to cardiac output, and thus control of atrial arrhythmias is important. Outcomes after cardioversion in patients after Fontan have not been reported. We sought to determine if cardioversion results in improved echocardiographic parameters or clinical symptomatology; and, discern risk factors for arrhythmia recurrence., Design: We retrospectively analyzed the Mayo Clinic echocardiographic database to capture patients after the Fontan operation who underwent transesophageal echocardiography-guided electrical cardioversion from 2000-2015. Clinical and echocardiographic data were collected and compared at baseline and follow-up., Results: Eight hundred ninety patients with prior Fontan operation underwent echocardiographic evaluation; 341 (38%) developed atrial arrhythmias. Thirty-six patients [20 males, median age 29 (12-51)] underwent transesophageal echocardiography-guided cardioversion of atrial arrhythmias [atrial flutter/intraatrial reentrant tachycardia (75%); atrial fibrillation (25%)]. At follow-up, improvements were noted in ejection fraction by 10% (P < .0001); atrioventricular valve regurgitation grade (39%) (P = .002); New York Heart Association (NYHA) class (61%) (P < .001); and resolution of spontaneous echo contrast in the Fontan circuit (65%) (P < .01). No embolic events occurred following cardioversion. Eighteen patients (50%) developed recurrent atrial arrhythmias at 15 (3-36) months after cardioversion. Five-year freedom from arrhythmia recurrence was 61%. Significant univariate predictors of arrhythmia recurrence were atrial flutter/intraatrial reentrant tachycardia (HR = 4.3, P = .02); NYHA ≥ II (HR = 4.1, P = .03); systemic right ventricle (HR = 5.2; P = .02); and ejection fraction ≤ 40% (HR = 2.8; P = .04). On multivariate analysis, only systemic right ventricle (HR = 3.7; P = .02) remained an independent predictor of arrhythmia recurrence., Conclusion: After the Fontan operation, cardioversion of atrial arrhythmias improves ventricular function, atrioventricular valve regurgitation grade, and NYHA class. Arrhythmia recurrence was common and patients with atrial flutter/intraatrial reentrant tachycardia, systemic right ventricle, or reduced ventricular function may be at risk of arrhythmia recurrence. Further studies are required to identify additional risk factors and protective factors for arrhythmia recurrence., (© 2016 Wiley Periodicals, Inc.)

Objective: The feasibility and safety of pregnancy after the Fontan operation is not well understood. We sought to determine contraception practices and early and late outcomes of pregnancy after the Fontan operation., Design: We performed a retrospective review of medical records to identify women of childbearing age from the Mayo Clinic Fontan database. A follow-up questionnaire was mailed to all patients not known to be deceased at the time of study. Patients with available contraception and pregnancy data were included in the study., Results: Of the 138 women with available contraception data, 44% used no contraception, 12% each used barrier methods, combination hormone therapy or sterilization, 8% used Depo-Provera, 7% had intrauterine devices, 4% had a partner with a vasectomy and 1% used progestin pills. Six women had thrombotic complications (only one using oral contraceptives). Thirty-five women had pregnancy data available. Prior to the Fontan operation there were 10 pregnancies (8 miscarriages, 2 therapeutic abortions, and no live births). After the Fontan operation there were 70 pregnancies resulting in 35 miscarriages (50%), 29 live births (41%), and 6 therapeutic abortions (9%). There were no maternal deaths during pregnancy. During long-term follow up (26 ± 6 years since the Fontan), 1 death, and 1 cardiac transplant occurred. Mean gestational age of the newborns (n = 22/29) was 33.1 ± 4.0 weeks; mean birth weight (n = 20/29) was 2086 ± 770 g. There was 1 neonatal death because of prematurity and two children were born with congenital heart disease (one patent ductus arteriosus and one membranous ventricular septal defect)., Conclusions: Pregnancy after the Fontan operation is associated with a high rate of miscarriages, preterm delivery, and low birth weight. Further studies are needed to identify specific variables influencing risk stratification of pregnancy in this patient population., (© 2015 Wiley Periodicals, Inc.)

Background: Initial palliative treatment of congenital aortic valve stenosis consists of either surgical commissurotomy or transcatheter balloon valvuloplasty. The basis for selection of primary approach usually is ill defined. It was hypothesized that aortic valve characteristics may be helpful in directing choice of intervention. This study assesses the impact of valve morphology on time to reintervention after catheter- or surgical-based therapy., Methods: A retrospective review of preprocedural echocardiographic aortic outflow characteristics was compared with outcomes of primary surgical or balloon valve interventions. Characteristics studied included (1) annular dimension; (2) leaflet number; (3) leaflet excursion; and (4) degree of leaflet coaptation. Patients included those <20 years with a primary diagnosis of aortic stenosis (AS) and no other hemodynamically significant lesions at our institutions from 2000 to 2011. A total of 102 patients were included, 31 classified as having critical and 71 as having noncritical aortic stenosis. Of the patients, 79 were male, and 50 underwent primary catheter intervention. Echo parameters were compared with procedural outcome as defined by death or need for reintervention. Receiver operator curves were utilized to determine the point within each morphologic feature where the greatest difference occurred. This was utilized as the distinguishing point within each the morphologic group. Analysis was conducted separately for critical and noncritical aortic stenosis., Results: Kaplan-Meier analysis demonstrated no significant difference in time to reintervention or death whether initial palliation consisted of surgical commissurotomy or balloon valvuloplasty with respect to any of the morphologic characteristics studied., Conclusion: Patients with AS do equally well with surgical commissurotomy or balloon valvuloplasty as initial palliation. This holds true for those with either critical or noncritical aortic stenosis. Valve morphology did not help in selection of initial palliative strategy. Current technologies should enable an improved selection of initial palliative approach through thoughtful, randomized trials., (© 2015 Wiley Periodicals, Inc.)

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.)

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.)

Objective: Valve repair for pediatric patients with Ebstein anomaly has historically yielded varied results. The cone reconstruction (CR) first described by Da Silva has revolutionized the surgical approach to these patients. This study reports our recent experience with CR in children and young adults with Ebstein anomaly., Design: Electronic medical records were reviewed for all patients < 21 years old who had surgery to repair Ebstein anomaly at Mayo Clinic Rochester between June 2007 and December 2012. Clinical data including preoperative demographics, intraoperative procedures, and postoperative outcomes were recorded., Results: Eighty-four patients initially had a cone reconstruction (54% male, mean age 10.1 ± 5.9 years). Indications for operation included cardiomegaly (42%), cyanosis (19%), and heart failure (19%). The preoperative echocardiogram demonstrated severe tricuspid regurgitation in 91% of patients. There was one early death and 3 early CR breakdowns requiring reoperation (2 re-repair, 1 tricuspid replacement). Eighty-two patients (98%) had successful CR at the time of hospital discharge. Patient age, gender, cardiopulmonary bypass time, and aortic cross-clamp time were not associated with early CR failure. Use of a partial or eccentric annuloplasty ring correlated with successful initial CR (P = .01). There have been no early CR breakdowns since 2010. Follow-up information was available for 77 patients (longest follow-up 6.5 years; mean 0.8 ± 0.2 years). The most recent postoperative echocardiogram demonstrated mild or no tricuspid regurgitation in 83%. Tricuspid stenosis (mean gradient > 5 mm Hg) was present in 6 patients. There was one late death (motor vehicle accident) and one late re-repair of the tricuspid valve 4 years after initial operation., Conclusions: CR in children and young adults with Ebstein anomaly can be performed with low early mortality and excellent durability at short-term follow-up. CR represents an important surgical option for young patients. It is applicable to patients with a broad range of anatomic variability and precludes valve replacement in the vast majority. CR should be considered prior to the deleterious effects of chronic right ventricular volume overload and the development of systolic dysfunction, which hamper long-term prognosis. Therefore, early referral for surgical evaluation is recommended., (© 2013 Wiley Periodicals, Inc.)

Headaches and blurred vision in patients with cyanotic congenital heart disease and secondary erythrocytosis may be attributed to hyperviscosity and traditionally were treated with phlebotomy. In the current era, phlebotomy is rarely performed in these patients except in cases of hemoptysis or hyperviscosity symptoms. We report a case of a patient with a history of complex cyanotic congenital heart disease and secondary erythrocytosis who presented with headache and visual changes. He was found to have bilateral papilledema and increased intracranial pressure. Reduction of intracranial pressure with acetazolamide therapy led to alleviation of headache and visual changes. This demonstrates the need for formal ophthalmologic evaluation of these patients to assess other treatable causes of headache and visual changes before considering phlebotomy., (© 2011 Wiley Periodicals, Inc.)

Octreotide has had limited use for the treatment of protein-losing enteropathy following the Fontan operation. We describe three cases where subcutaneous octreotide was successfully used to treat refractory protein-losing enteropathy following the Fontan operation. Patients received octreotide therapy for a period of 14-28 months. Octreotide was discontinued in one patient due to symptomatic cholelithiasis; this patient died 7 months after treatment was discontinued. Octreotide may be a useful treatment in these patients, but further study on its efficacy and mechanism of action is needed., (© 2011 Wiley Periodicals, Inc.)

Valve thrombosis is a potentially dangerous complication for right sided prosthetic valves in adults with congenital heart disease. Thrombolytic therapy is an important alternative to surgery and may be of benefit in patients with a history of multiple surgical interventions. We present two cases of successful thrombolytic therapy for right sided valve thrombosis in patients with congenital heart disease., (© 2011 Copyright the Authors. Congenital Heart Disease © 2011 Wiley Periodicals, Inc.)

Objective: Fibrosing mediastinitis is a rare disorder characterized by an excessive fibrotic reaction in the mediastinum which can result in compromise of mediastinal structures. We sought to evaluate short- and midterm results of intravascular thoracic vessel stent placement for patients with fibrosing mediastinitis., Design: We reviewed all records of fibrosing mediastinitis patients who were referred for stent placement to treat mediastinal vascular compression over a 7-year period. Catheterization reports and digital angiography were assessed to determine vessel dimension and stent characteristics., Patients: Thirteen catheterizations were performed in six patients (five females, mean age 39 years, range 23-63) with a range of 1-4 per patient., Interventions: Four patients were treated with intravascular stents placed percutaneously. One patient underwent surgical intravascular stent placement, and one patient declined surgical therapy. The right pulmonary artery was treated in three patients, the superior vena cava was treated in one patient, and three pulmonary veins were treated in one patient., Outcome Measures: Pertinent vessel and hemodynamics including immediate short-, and mid-term results were assessed. Procedural complications, midterm follow-up (up to 7 years), and overall survival were determined., Results: Each intervention resulted in hemodynamic improvement with subsequent clinical improvement. Reintervention was required within 12 months in two of four percutaneously treated patients. One death occurred 4 days after cutting balloon angioplasty in a preexisting pulmonary vein stent., Conclusions: Percutaneous therapy for vessel compression secondary to fibrosing mediastinitis is an option that is effective in improving short-term vascular patency. In-stent stenosis was a frequent complication in patients with fibrosing mediastinitis, particularly when pulmonary veins were involved. Short- and midterm success can be achieved, but progressive fibrosing mediastinitis remains a difficult clinical problem with repeat dilation of stents and/or additional stent placement necessary to maintain optimal stent patency and improvement in clinical symptomatology.

Objective: We sought to identify complications that occurred during congenital cardiac catheterization (CCC) and determine factors that could improve the quality of care provided to patients with congenital heart disease during this procedure., Design: We reviewed the electronic medical record for 903 CCC cases, (455 female; mean age = 29 +/- 22 years, range = birth to 91 years) performed in our catheterization laboratory from 2005 to 2007. Included in this cohort are 342 cases performed on patients less than 18 years of age. Clinical follow-up data were reviewed for 3 months postcatheterization. Complications were assigned a grade from 1 to 4 based on severity., Results: The indication for catheterization was diagnostic in 459 (51%) patients, interventional in 386 (43%) patients, and endomyocardial biopsy in 58 (6%) patients. Mean intravenous contrast dose = 1.9 +/- 1.8 mL/kg. Mean fluoroscopy exposure = 22 +/- 13 minutes. Mean procedure duration = 122 +/- 42 minutes. Although 806 cases (89%) were performed without complication, 102 complications were observed in 97 cases. There were no deaths. Emergent surgery was performed in four patients. One patient notified us 16 days after catheterization that she was pregnant. The result of that pregnancy was normal. Thirty complications occurred during the CCC procedure and a first year fellow was involved in 17. Patient age, weight, gender, attending physician, or type of procedure (diagnostic vs. intervention) did not impact risk of complications., Conclusions: Patients of all ages with congenital heart disease can expect a safe procedure with minimal risk of serious complications. Procedural changes that have been implemented include pregnancy testing on all menstruating females prior to CCC regardless of history of sexual activity, and first-year fellows are now directly supervised by the attending physician rather than a more senior fellow throughout the procedure.

Coronary artery anomalies are not uncommon, occurring in 1.3% (range = 0.3-5.6%) of the population, and are often an incidental finding in asymptomatic patients. Approximately 20% of coronary anomalies have potential for life-threatening complication, including myocardial infarction, arrhythmia, or sudden death early in life or during adulthood. Coronary artery anomalies are composed of a wide variety of disorders. Some, such as anomalous location of a coronary ostium, duplication of coronary arteries, or multiple ostia, become clinically significant only when another cardiac surgical procedure is necessary, and generally, surgical correction is not required in these patients. On the other hand, the diagnosis of anomalous origin of left coronary artery from pulmonary artery or from the opposite sinus with inter-arterial course is an indication for operation. Some anomalies like coronary artery fistulas, myocardial bridging, and coronary aneurysm require operation only when they cause clinical symptoms. Coronary artery anomalies should be included in the differential diagnosis of anginal symptoms, myocardial infarction, arrhythmia, or heart failure, especially in young patients. Increased awareness of these pathologies will lead to earlier diagnosis and treatment of a potentially life-threatening condition.

Objective: There are situations in which standard echocardiography does not adequately define the aortic arch. We sought to determine what additional information could be gained by analyzing abdominal aortic Doppler flows in coarctation., Design: Previously recorded echocardiographic data were reviewed in 70 controls and 248 patients with coarctation, including abdominal aortic values for pulsatility indices, pulse delay, and presence of early diastolic reversal. Ability of these variables to distinguish controls from coarctation patients and to assess coarctation severity was assessed., Results: Corrected maximum instantaneous gradient and all abdominal aortic flow variables were associated with severity of obstruction. Early diastolic reversal was universally absent in significant coarctation. Threshold values for other parameters associated with significant obstruction were: corrected pulse delay >or=3.4 msec(1/2), pulsatility index <2.0, and systolic to diastolic velocity ratio <3.6. A combined abdominal aortic "variable" (absence of early diastolic reversal and corrected pulse delay >or=2.8 msec(1/2)) was found to be the best predictor of clinical coarctation status (positive predictive value = 93%, negative predictive value = 88%)., Conclusions: In the absence of a ductus arteriosus, abdominal aortic Doppler parameters can reliably predict the presence of significant coarctation. When early diastolic reversal was present, obstruction was always absent. Lack of early diastolic reversal with a prolonged pulse delay was the best predictor of significant obstruction. Abdominal aortic Doppler evaluation should become a routine part of the evaluation of patients with known or suspected coarctation.

Objective: We sought to define what clinical parameters were related to the ultimate outcome in Fontan patients who had developed protein-losing enteropathy (PLE)., Background: PLE is a serious complication of the Fontan operation. Several preoperative and perioperative findings are associated with later PLE. However, there is limited information regarding postoperative abnormalities contributing to or influencing outcome in PLE., Methods: We evaluated 44 consecutive Fontan patients with PLE. A matched control group of Fontan patients without PLE was used for comparison (matched-pair analyses). Kaplan-Meier and Cox proportional hazard methods were used for survival analyses., Results: Median age was 18 years (range 4-48 years). Short-axis and apical fractional area change (Delta FA) were less in PLE patients than in controls (50 vs. 57% [P < .0001] and 49 vs. 54% [P = .01]). Five and 10-year actuarial survival rates for the PLE group were 49 +/- 9% and 30 +/- 11%. Deceleration time <120 milliseconds (hazard ratio [HR] = 9.2, P = .04), New York Heart Association classification III or IV (HR = 4.0, P = .01), and lower serum albumin (HR = 0.30, P = .04) were independent predictors of mortality in those with PLE., Conclusion: Mild reduction in Delta FA was the only late echocardiographic finding associated with development of PLE. However, short deceleration time, poor New York Heart Association (NYHA) class, and low serum albumin identify a group of patients at the greatest risk for death. Presence of these findings in a PLE patient should lead to aggressive management strategies and may warrant early consideration of transplantation.

Objective: The purpose of this study was to assess the outcome of pulmonary valve replacement (PVR) in adults with moderate/severe pulmonary regurgitation after tetralogy repair, with particular emphasis on patient outcome, durability of valve repair, and improvement in symptomatology., Design/setting/patients: The project committee of the International Society of Congenital Heart Disease undertook a retrospective multi-institutional analysis of PVR. Seven centers participated in submitting data on 93 patients >18 years of age who had the operation performed and follow-up obtained. The average age of PVR was 26+/- years (median 27 years). Time of follow-up after replacement was 3 years (range 4 days-28 years). OUTCOMES/MEASURES/RESULTS: Kaplan-Meier estimates of durability of PVR showed approximately 50% replacement at 11 years. There were two deaths at 6 and 12 months after valve replacement. Right ventricular (RV) size estimated by echocardiography from pre- to postoperative studies decreased in 81% (P < 0.001 testing for equal proportions), but RV systolic function increased in only 36% (P = 0.09). Ability index improved in 59% (P < 0.001) and clinical heart failure status improved in 57% with this problem before PVR. PVR did not improve arrhythmia status in a small group of patients., Conclusions: PVR is associated with low mortality, decrease in RV size and improvement in ability index, and uncertain effects on RV systolic function. Average valve durability was approximately 11 years. Criteria for PVR that will preserve RV function are not clearly identified, and management of these patients remains a difficult enterprise.

Aorto-left atrial fistula is a rare entity in which the integrity of the aortic root bordering the left atrium is disrupted. The clinical presentation is highly variable, depending predominantly on the size of the fistula and the pressure difference between the aorta and the left atrium. Surgical repair was the standard treatment. Recently, however, there have been reports of successful transcatheter closure. We report a 32-year-old male with Shone's syndrome who had multiple prior surgical procedures including aortic and mitral valve replacements. He presented with an aorto-left atrial fistula that was successfully closed percutaneously using an Amplatzer atrial septal defect device.