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The acromelic dysplasias comprise short stature, hands and feet, and stiff joints. Three disorders are ascribed to this group, namely Weill-Marchesani syndrome, geleophysic dysplasia, and acromicric dysplasia, although similar in phenotype, can be distinguished clinically. Weill-Marchesani syndrome, on the basis of microspherophakia and ectopia lentis; geleophysic dysplasia by progressive cardiac valvular thickening, tracheal stenosis, and/or bronchopulmonary insufficiency, often leading to early death. Microspherophakia has not been reported previously in geleophysic dysplasia. Mutations in FBN1, ADAMTS10, or ADAMTS17 cause Weill-Marchesani syndrome by disrupting the microfibrillar environment, while geleophysic dysplasia is associated with enhanced TGF-β signaling mediated through mutations in FBN1 or ADAMTSL2. We studied a 35-year-old woman with geleophysic dysplasia, with short stature, small hands and feet, limitation of joint mobility, mild skin thickening, cardiac valvular disease, restrictive pulmonary disease, and microspherophakia. Sequencing of ADAMTSL2 demonstrated two changes: IVS8-2A>G consistent with a disease-causing mutation, and IVS14-7G>A with potential to generate a new splice acceptor site and result in aberrant mRNA processing. The unaffected mother carries only the IVS8-2A>G transition providing evidence that the two changes are in trans-configuration in our patient., (© 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.)

Objectives: The objective of our study was to review the long-term outcomes of patients undergoing surgical repair of aortic coarctation., Background: Surgical repair of aortic coarctation has been performed at the Mayo Clinic, Rochester, Minnesota, for over 60 years., Methods: Between 1946 and 2005, 819 patients with isolated coarctation of the aorta underwent primary operative repair. Medical records were reviewed and questionnaires mailed to the patients., Results: Mean age at repair was 17.2 ± 13.6 years. The majority (83%) had pre-operative hypertension. Operations included simple and extended end-to-end anastomosis (n = 632), patch angioplasty (n = 72), interposition grafting (n = 49), bypass grafting (n = 30), and subclavian flap or "other" (n = 35). Overall early mortality (<30 days) was 2.4%. In the previous 30 years (n = 225), there were no operative deaths. Mean follow-up was 17.4 ± 13.9 years, with a maximum of 59.3 years. Actuarial survival rates were 93.3%, 86.4%, and 73.5% at 10, 20, and 30 years, respectively. When compared to an age- and sex-matched population, long-term survival was decreased (p < 0.001). Older age at repair (>20 yrs) and pre-operative hypertension were associated with decreased survival (p < 0.001). Patients age <9 years age at repair had significantly less hypertension at 5 to 15 years of follow-up (p < 0.001). Rates of freedom from re-intervention on the descending aorta were 96.7%, 92.2%, and 89.4% at 10, 20, and 30 years, respectively. Younger age at time of repair (p < 0.001) and an end-to-end anastomosis technique (p < 0.001) were independently associated with lower rates of re-intervention on the descending aorta., Conclusions: Primary repair of isolated coarctation of the aorta was performed with a low rate of mortality. However, long-term survival was reduced compared with that in an age- and sex-matched population, and many patients required further reoperation. These findings emphasize that patients with aortic coarctation need early recognition and intervention, as well as lifelong informed follow-up., (Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

A young female diabetic patient is reported, who presented with a double foot lesion. She presented with a first metatarsal head exposure concomitant with a heel wet gangrene. Magnetic resonance demonstrated osteomyelitis of the rear portion of the calcaneus. Transmetatarsal amputation was performed and a wide debridement was required to remove all gangrenous tissue from the heel wound. The pedal artery was palpable; the posterior tibial pulse was present, but weak.Transcutaneous oximetry (TcPO2) at the dorsum of the foot was TcPO2 = 56 mmHg despite significant oedema. Nevertheless, TcPO2 on the perilesional area of the heel ulcer (TcPO2 = 24mmHg) was suggestive for critical chronic ischemia. At angiographic examination, anterior tibial and peroneal arteries were patent, but the posterior tibial artery that showed severe stenosis then percutaneous angioplasty (PTA) was performed. Just the day after PTA, values of TcPO2 at the perilesional area of the heel ulcer increased to 41 mmHg. Heel osteomyelitis was subsequently treated by partial calcanectomy. The patient was discharged after a 21-day hospital stay. In the treatment of heel ulcers, it is clinically useful to use the angiosomic concept. The majority of the blood supply to the heel is provided by the posterior tibial artery, and only to a small extent by the posterior branch of peroneal artery. If the decrease in blood flow to this region is not detected, and direct flow based on the angiosome concept is not obtained, the healing of a heel ulcer may be delayed or impaired.

Objectives: The aim of this study was to investigate the prevalence of mitral valve prolapse (MVP) and its association with ventricular arrhythmias in a cohort with "unexplained" out-of-hospital cardiac arrest., Background: Ventricular arrhythmias are an important cause of sudden unexpected death in the young. The role of MVP in sudden unexpected death remains controversial., Methods: Of 1,200 patients evaluated between July 2000 and December 2009 in the Mayo Clinic's Long QT Syndrome/Genetic Heart Rhythm Clinic, all 24 (16 women, median age 33.5 years) with idiopathic out-of-hospital cardiac arrest (i.e., negative for ischemia, cardiomyopathy, and channelopathy) were reviewed., Results: All 24 patients had implantable cardioverter-defibrillators (ICDs). Out-of-hospital cardiac arrest was the sentinel event in 22 (92%). Bileaflet MVP was found in 10 (42%). Compared with patients with normal mitral valves, patients with bileaflet MVP: 1) were over-represented by women (9 of 10 [90%] vs. 7 of 14 [50%], p = 0.04); 2) had a higher prevalence of biphasic or inverted T waves (7 of 9 [77.8%] vs. 4 of 14 [29%], p = 0.04); and 3) on Holter interrogation had higher prevalence of ventricular bigeminy (9 of 9 [100%] vs. 1 of 10 [10%], p < 0.0001), ventricular tachycardia (7 of 9 [78%] vs. 1 of 10 [10%], p = 0.006), and premature ventricular contractions originating from the outflow tract alternating with the papillary muscle or fascicular region (7 of 9 [78%] vs. 2 of 10 [20%], p = 0.02). Over a median 1.8 years (range: 0.1 to 11.9 years) from ICD placement, 13 of 24 patients (54%) received appropriate ventricular fibrillation-terminating ICD shocks. Only bileaflet MVP was associated with ventricular fibrillation recurrences requiring ICD therapy on follow-up (logistic regression odds ratio: 7.2; 95% confidence interval: 1.1 to 48; p = 0.028)., Conclusions: The authors describe a "malignant" subset of patients with MVP who experienced life-threatening ventricular arrhythmias. This phenotype is characterized by bileaflet MVP, female sex, and frequent complex ventricular ectopic activity, including premature ventricular contractions of the outflow tract alternating with papillary muscle or fascicular origin., (Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objective: We characterized the histologic liver changes that occur after the Fontan operation and identified the factors associated with worsening hepatic disease., Methods: From 1979 to 2009, 33 patients who died after the Fontan operation had a postmortem analysis at our institution. The liver histologic findings were graded for sinusoidal and portal fibrosis., Results: The median interval from the Fontan operation to death was 14 days (range, 0 days to 25.6 years). The median age at death was 7.7 years (range, 1.9-52.9 years). Hepatic sinusoidal fibrosis occurred in 25 of 33 patients (76%), with significant fibrosis (grade 2 or greater) in 15 of 25 patients (60%). Portal fibrosis occurred in 17 of 33 patients (52%). The degree of sinusoidal (P < .003) and portal fibrosis (P < .002) correlated with an increased period from the Fontan operation to death. Sinusoidal fibrosis was also associated with increased age at the initial Fontan operation (P < .03). Of the patients who died early (< 35 days postoperatively) after the Fontan operation, sinusoidal fibrosis was present in 15 of the 23 patients (65%) and portal fibrosis in 7 of 23 patients (30%). Significant (grade 2 or greater) sinusoidal fibrosis was seen in 7 patients, and 4 of the 7 had heterotaxy syndrome. Significant portal fibrosis was observed in only 2 patients; both had heterotaxy syndrome., Conclusions: The interval from the initial Fontan operation to death is associated with increased sinusoidal and portal fibrosis. In this small cohort, older age at Fontan operation was associated with sinusoidal fibrosis. Significant sinusoidal fibrosis was observed in patients who died early after the Fontan operation, with more severe disease present in patients with heterotaxy syndrome., (Copyright © 2013 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Background: Successful tricuspid repair for Ebstein malformation (EM) is challenging. Cone repair has recently emerged as the most anatomic repair technique. There are no data on tricuspid re-repair in EM. We reviewed our experience with tricuspid valve (TV) re-repair using a cone technique., Methods: Between June 2007 and October 2012, 134 patients underwent cone repair. Prior TV repair was performed in 20 patients (10 female, 50%). Median age was 15 years (range, 4 to 68 years). Four patients (20%) had prior bidirectional cavopulmonary anastomosis (BDCPA). Preoperative heart failure was present in 8 patients (40%). Recurrent tricuspid regurgitation (TR) was due to incomplete leaflet coaptation with tethered anterior leaflet in all patients; and 10 patients (50%) had diminutive septal leaflet. Prior repair techniques consisted of annuloplasty maneuvers in all patients with no or incomplete surgical delamination in all patients., Results: The TV re-repair using cone technique was possible in all patients. Plication of atrialized right ventricle was done in 6 patients (30%). Modifications included annuloplasty band in 16 patients (80%) and TV leaflet augmentation in 8 (40%). The BDCPA was performed in 3 patients (15%) and modified Cox-maze procedure was done in 10 (50%). There were no mortalities or reoperations. Mean follow-up was 7.7 ± 10.7 months; during follow-up, 18 patients had no or mild TR and 2 had moderate TR., Conclusions: TV re-repair in EM using the cone technique is feasible, particularly when there has been no surgical leaflet delamination at initial operation. Leaflet augmentation facilitates and annuloplasty band stabilizes a competent TV re-repair. Longer follow-up is required to assess durability., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: The purpose of this study was to report the results of percutaneous valve-in-valve therapy using the Melody valve (Medtronic, Minneapolis, Minnesota) for patients with degenerated mitral and tricuspid bioprosthetic valves., Background: Open surgery for replacement of degenerated bioprosthetic valves is associated with morbidity and mortality., Methods: Nineteen patients (median age 65 years, range 10 to 88 years; 7 males) with degenerated mitral (n = 9) or tricuspid (n = 10) bioprosthetic valves underwent transvenous valve-in-valve implantation of the Melody valve., Results: In the mitral patients, the mean Society of Thoracic Surgeons mortality score was 13.3 ± 5.6%. All patients had a prosthetic valve mean diastolic inflow gradient ≥5 mm Hg. Moderate or worse regurgitation was present in 7 of 9 mitral and 7 of 10 tricuspid patients. Implantation of a Melody valve was successful in all. Among the mitral patients, mean diastolic gradient decreased from 12.3 ± 4.6 mm Hg to 5.2 ± 2 mm Hg (p < 0.01). Residual regurgitation was trivial to mild in 6, mild to moderate in 2, and moderate in 1 patient. Among the tricuspid patients, mean diastolic gradient decreased from 10.0 ± 4.3 mm Hg to 5.6 ± 2.5 mm Hg (p < 0.01). Residual regurgitation was trivial to mild in 9 and mild to moderate in 1 patient. New York Heart Association functional class improved in 17 of 19 patients (p < 0.01). No periprocedural deaths, myocardial infarctions, strokes, or valve embolizations occurred. Vascular access site complications occurred in 4 patients., Conclusions: Percutaneous valve-in-valve implantation of the Melody valve in the mitral or tricuspid position for treatment of bioprosthetic valve dysfunction is feasible and can lead to significant symptomatic improvement in carefully selected high-risk patients., (Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: In recent years, percutaneous device closure has become commonplace for treatment of atrial septal defect (ASD) and patent foramen ovale (PFO). However, the effect of device closure on aortic valve regurgitation (AR) has been questioned., Objective: The purpose of this study was to define changes in AR using echocardiography, before and after device closure of ASD or PFO., Methods: We retrospectively reviewed all patients who had percutaneous device closure of ASD or PFO at our institution between 2005 and 2009. We compared the preprocedure echocardiogram to the most recent follow-up study. All investigators were blinded to referral diagnosis, patient demographics, and size and type of the device placed. Aortic regurgitation was graded as none/trivial, mild, moderate, or severe., Results: Overall, 322 patients qualified for inclusion; 204 with PFO and 118 with ASD. Mean age at time of closure was 48 ± 20 years (range 3-85 years). Mean duration of follow-up was 1.2 years (range 2 months to 5 years). Only two patients (0.6%) demonstrated any appreciable increase in AR severity. However, both of these patients manifested a clinically insignificant change from a trivial to mild grade of aortic regurgitation., Conclusions: Percutaneous device closure of PFO or ASD does not increase the incidence or severity of AR over medium-term follow-up., (Copyright © 2012 Wiley Periodicals, Inc.)

Background: The management of the zone of apposition (ZOA) in patients with atrioventricular septal defect (AVSD) and parachute left atrioventricular valve (LAVV) is controversial., Methods: Between 1977 and 2010, 28 patients with parachute LAVV associated with AVSD were reviewed. The median age at operation was 10 months (range, 36 days to 14 years). Sixteen (57%) patients had complete AVSD and 12 (43%) had partial AVSD. Thirteen (46%) patients had moderate to severe LAVV regurgitation., Results: The ZOA was managed with complete closure in 6 (22%), partial closure in 10 (36%), and no closure in 11 (39%) patients One patient underwent LAVV replacement for dysplastic leaflets. Dismissal echocardiogram demonstrated moderate LAVV regurgitation in 10 (36%) patients; 7 patients had no closure of ZOA, and 3 had partial closure. Mild or moderate LAVV stenosis was present in all 6 patients with complete closure of ZOA and 1 patient with partial closure. Median follow-up was 9 years (maximum, 22 years). Eight patients had progression of LAVV regurgitation through the unsutured ZOA; 6 patients subsequently underwent LAVV replacement. Of the 7 patients who had LAVV stenosis, 1 patient required opening of ZOA 1 month after surgery. The other 6 patients had a decrease in mean gradient. There was 1 late death after the fourth redo LAVV replacement., Conclusions: Progression of LAVV regurgitation from the unsutured ZOA was the main indication for reoperation in parachute LAVV with AVSD. The ZOA in parachute LAVV should be partially or completely closed at the time of AVSD repair. Although mild LAVV stenosis appeared to improve with time, life-long surveillance is essential., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Reproducible repair of Ebstein's malformation is challenging and numerous surgical techniques have been described. We reviewed our experience with the cone reconstruction., Methods: Between June 2007 and December 2011, 89 patients (47 female; 53%) underwent cone reconstruction (median age 19 years; range, 19 days to 68 years). Indication for operation was progressive cardiomegaly in 43 (48%), cyanosis in 29 (33%), and heart failure in 13 (15%). Prior tricuspid valve repair was performed in 12 patients (13%). Severe tricuspid regurgitation (TR) was present in 75 patients (84%)., Results: All patients underwent cone reconstruction (360-degree leaflet tissue repair anchored at true annulus). Modifications included ringed annuloplasty in 57 patients (64%), leaflet augmentation in 28 patients (31%), and autologous chordae in 17 patients (19%). Bidirectional cavopulmonary anastomosis was performed in 21 patients (24%). Early mortality occurred in 1 patient (1%). Early reoperation for recurrent TR occurred in 12 patients (13%); re-repair was performed in 6 patients (50%), and 6 (50%) required replacement. Mean follow-up was 19.7 ± 24.7 months. There was no late mortality or reoperation. At follow-up, 72 patients (87%) had no or mild TR, 9 (11%) had moderate TR, and 2 patients (2%) had severe TR. Ringed annuloplasty was associated with less than moderate TR at dismissal (p = 0.01)., Conclusions: The learning curve for cone reconstruction is steep, but early mortality is low. Cone reconstruction with ringed annuloplasty results in less TR and should be used whenever possible. Longer follow-up is essential to determine late durability of cone reconstruction., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: In patients with a modified Fontan connection, particularly the right atrial to right ventricular connection, the pulmonary ventricle may enlarge with time., Methods: Between January 1990 and December 2006, 10 patients (median age, 24 years) underwent Fontan conversion to a one and one half ventricle repair. Tricuspid atresia was the most common diagnosis (n=7). The right atrial to right ventricular connection was present in 8 patients; 3 patients had a prior bidirectional cavopulmonary anastomosis. Preoperative median right atrial pressure was 14 mm Hg (range, 12 to 20 mm Hg). Indications for surgery were exercise intolerance, arrhythmias, and conduit obstruction. Echocardiography showed moderate right ventricular hypoplasia, with right atrial to right ventricular regurgitation. The conversion included closure of septal defects, tricuspid valve replacement (n=8), bidirectional cavopulmonary anastomosis (n=7), valved right ventricular outflow tract reconstruction (n=2), and arrhythmia surgery (n=6)., Results: There was no early mortality. There was one reoperation for residual ventricular septal defect. Prolonged chest tube drainage occurred in 2 patients. Postoperative right atrial pressure ranged from 8 to 14 mm Hg (median, 10 mm Hg; p=0.02). Median follow-up was 8 years. There was no late mortality. Nine patients are in New York Heart Association class I or II, and 1 patient has biventricular failure and was awaiting transplantation. There were two late reoperations, 1 for thrombosis of the mechanical tricuspid valve prosthesis and 1 for obstruction of the valved right ventricular outflow tract conduit. Three patients had recurrent atrial arrhythmias requiring cardioversion., Conclusions: Fontan conversion to one and one half ventricle repair is feasible in selected patients with a failing Fontan circulation. Operation can be performed with low early mortality. Arrhythmia surgery should be performed routinely. Quality of life is excellent., (Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Introduction: The aim of the present study was to compare the restaging results obtained by transrectal ultrasound (TRUS), computed tomography (CT), and magnetic resonance imaging (MRI) performed after preoperative chemoradiation with pathologic staging of the operative specimen., Methods: From January 2008 to December 2009, all the consecutive patients with locally advanced rectal cancer that underwent neoadjuvant therapy at our department were evaluated. The results of diagnostic examinations and the definitive pathological examination were considered and compared., Results: Thirty-seven patients were included in the study (27 males, 73%), mean age was 65.5 years (range 45–82 years). In all the patients TRUS and CT and in 20 patients MRI were performed before and after the treatment. Concerning the depth of invasion after treatment TRUS agreed with histopathology in 25/37 patients (67.5%), CT agreed in 22/ 37 cases (59.5%), and MRI in 12/20 cases (60%). Considering only neoplasia with stage T3, TRUS agreed in 23/24 cases (96%), CT in 19 cases (79%), and MRI in 10/12 cases (83.5%). Considering the tumors that did not exceed the rectal wall (T0, T1, and T2), TRUS agreed with histology in 2/13 cases (15.5%),CTin 3/13 cases (23%), andMRI 2/8 cases (25%). Concerning the presence of positive lymph nodes TRUS agreed with histology in 28/37 cases (75.5%), while CT agreed in 21/37 cases (56.5%) and MRI in 11/20 cases (55%). The concordance between the techniques was found to be low., Conclusions: Transrectal ultrasonography resulted as the most accurate method to determine neoplastic wall infiltration and lymph node involvement even after radiochemotherapy. In most cases, considering the poor correlation between the diagnostic procedures and the disagreement of the results, a restaging performed only with TRUS could be proposed, limiting the use of the other imaging methods to selected cases.

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.)

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.)

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.)

Background: Neoadjuvant chemoradiotherapy is recommended in the treatment of locally advanced rectal cancer. Studies have suggested that chemoradiotherapy adversely affects anorectal function. However, the functional implication and the underlying neuromyogenic changes involved in radiation-induced damage are poorly understood., Objective: This study evaluated the functional changes following chemoradiotherapy on the internal anal sphincter., Design and Patients: This article describes an in vitro study on the internal anal sphincter collected from patients undergoing abdominoperineal resection or proctectomy. Five patients were treated by surgery alone (control group), and 6 received preoperative chemoradiotherapy (treatment group). Sphincter strips were mounted in organ bath, and the responses to electrical field stimulation and drugs were monitored., Settings: The study was performed at the University of Oxford., Main Outcome Measures: The end points of this study were to investigate whether chemoradiotherapy has any significant effects on internal anal sphincter function and, subsequently, to establish the type of injury induced., Results: Chemoradiotherapy strips developed similar tone, but significantly lower spontaneous activity (p = 0.001) than controls. Electrical field stimulation induced relaxation, followed by contraction. At 50 Hz, electrical field stimulation produced 25.6 ± 4.9% (mean ± SE) of maximum relaxation followed by a contraction of 5.5 ± 0.9% of basal tone in chemoradiotherapy strips i9n comparison with 47.0 ± 6.2% (p = 0.009) and 17.7 ± 4.0% (p = 0.007) in controls. Relaxation was significantly attenuated by N-nitro-L-arginine. Significant differences were found in responses to carbachol (p = 0.018) and phenylephrine (p = 0.022), but not to sodium nitroprusside., Limitations: This work was limited by the relatively small number of patients enrolled, because of the difficulty of finding human tissue for laboratory studies, and the lack of long-term results., Conclusions: Chemoradiotherapy significantly impairs internal anal sphincter function and intrinsic nerves seem more susceptible than smooth muscle. The exclusion of anal canal from the radiation field is recommended, when oncologically safe.

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.)

We present a newborn patient who had surgical correction of supracardiac total anomalous pulmonary venous connection repair with an unusual cause of residual postoperative pulmonary venous obstruction secondary to compression from the transesophageal echocardiography (TEE) probe.

Background: Approximately 5% to 15% of patients develop protein-losing enteropathy (PLE) after the Fontan operation. Oral controlled release (CR) budesonide has been used as a treatment strategy, but its use in the older Fontan population has not been described., Methods: Seven patients with refractory PLE after the Fontan operation were started on oral CR-budesonide at 9 mg. After 3 to 9 months, the dose was weaned to 3 mg. Response to treatment was assessed by clinical evaluation, serum albumin levels, and fecal α-1 antitrypsin clearance when available., Results: Median age at last evaluation was 20 years (range, 16 to 32 years). Six patients had increases in serum albumin levels but only 4 patients had symptomatic improvement. Systemic side effects included: cushingoid features (5), adrenal insufficiency (4), and new-onset type 2 diabetes mellitus (2). One patient had improvement in cushingoid features after weaning CR-budesonide to 3 mg. Older patients (ages 27 to 32 years) had the worst side effect profiles and were the most refractory to treatment. These patients had sonographic evidence of hepatic cirrhosis but normal serum liver function tests. Two deaths occurred: 1 from sepsis 1 month after CR-budesonide initiation and 1 from respiratory arrest 5 months after CR-budesonide discontinuation., Conclusions: CR-budesonide can be used to treat PLE in certain patients, but careful assessment of hepatic function should be performed before initiation of therapy as systemic side effects can limit treatment. Normal serum liver function tests do not preclude hepatic dysfunction in the Fontan patient, and it is important to perform radiographic assessments as well., (Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

The tumor suppressor p53 and its negative regulator MDM2 have crucial roles in a variety of cellular functions such as the control of the cell cycle, senescence, genome stability and apoptosis, and are frequently deregulated in carcinogenesis. Previous studies have highlighted the contribution of the common functional polymorphisms p53 p.Arg72Pro and MDM2 309SNP to the risk of both common cancers and Li-Fraumeni syndrome. Their possible role in retinoblastoma has recently been addressed by Castéra et al, who however only studied the MDM2 309SNP. Here, for the first time, we analyzed both single nucleotide polymorphisms (SNPs) in a case-control study of 111 Italian hereditary retinoblastoma patients. We found a significant association of the p53 Pro/Pro genotype with the disease (odds ratio=3.58, P=0.002). The MDM2 309SNP showed a weak negative association of allele G that deserves further investigation. These findings further support the hypothesis that genetic variability of the p53 pathway contributes to the individual susceptibility to retinoblastoma, as shown for Li-Fraumeni syndrome and a variety of non-hereditary cancers.

In this study we evaluated the results of stapled hemorrhoidopexy considering the histological features of the resected tissue obtained after the intervention, the staple line height and the improvement of the quality of life after the treatment. From January 2003 to December 2006, 72 patients with symptomatic grade 3 and 4 hemorrhoid that underwent stapled hemorrhoidopexy in our clinic were enroled in the study. Preoperative, intraoperative and postoperative characteristics of the patients were evaluated, including demographics, staple line height, specimen histology, complications, recurrence, day to return to work, quality of life (QoL) score and use of analgesics. Staple line height was always above 2.5 cm from the dentate line. Smooth muscle fibers were observed in 97.2%. The complication rate was 18%. Bleeding was observed in 5.5%. Fecal urgency was referred in 6.9%. Recurrence rate was 6.9%. Mean follow-up was 68 months. Hemorrhoidopexy is safe and effective but extreme attention must be paid to some critical details. We consider the placement of the purse string suture as a critical factor in the outcome of the patients. We find that some amount of the muscle fibers would invariably be involved in the resected specimen, without any significant impact in the clinical outcome or in the quality of life improvement.

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.)

Background: Tissue Doppler Imaging (TDI) is a sensitive index of myocardial function. Its role in the fetus has not been extensively evaluated., Objective: To compare myocardial tissue Doppler velocities in fetuses with hypoplastic left heart syndrome (HLHS) to those of normal fetuses (matched for gestational age.), Methods: Cross-sectional retrospective study conducted at 2 large perinatal centers (2003-2007). Fetuses with HLHS (n = 13) were compared with normal fetuses (n = 207) in 5 gestational age groups. TDI data included peak systolic (s'), peak early (e'), and late diastolic velocities (a'). Linear regression was used to compare TDI parameters in fetuses with HLHS to normal fetuses matched for gestational age., Results: Fetuses with HLHS had significantly reduced lateral tricuspid annular e' as compared to normal fetuses. Both normal fetuses and those with HLHS had linear increase in TDI velocities with advancing gestational age., Conclusions: TDI velocities are abnormal in fetuses with HLHS. TDI can be useful in serial follow-up of cardiac function in fetuses with HLHS.

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.)

Although the Fontan procedure has been enormously successful in palliation of single-ventricle patients, many seem to experience progressive failure of the Fontan circulation over time. Ventricular assist devices (VADs) have developed into stable platforms for long-term support of adult patients with heart failure. Given the success of axial flow devices, it was hypothesized that the technology could provide clinical benefit to failing Fontan patients. The aim of this study was to use a computer model to evaluate VAD support in failing Fontan physiology. A computer model of Fontan circulation with heart failure was developed and the HeartMate II (HM II) (Thoratec Corp) axial flow ventricular assist device was connected to this model in a systemic configuration to examine its impact. Cardiac catheterization data from 7 patients (8 catheterization studies) with failing Fontan physiology were applied to this model to evaluate the impact of using the HM II in this manner. When the HM II was used in a systemic configuration at 8000 rpm, there was a 35% decrease in the systemic venous pressure in the Fontan circuit and a 63% decrease in pulmonary capillary wedge pressure with a resultant 41% increase in CI. The model also predicted patient-specific parameters where the VAD may not benefit the patient, such as fixed elevated pulmonary vascular resistance, low systemic ventricular end-diastolic pressure, and high unresponsive systemic vascular resistance. These data suggest a potential benefit from application of axial flow VAD technology in the management of failing Fontan physiology. Clinical correlation will allow for refinement of this model as a predictive tool in discerning which patients may benefit from placement of a VAD and what issues must be addressed prior to implanting the device.

Objective: The objective of this study was to evaluate the early and late results of atrioventricular valve surgery after Fontan operation., Background: Atrioventricular valve regurgitation is a known perioperative risk factor for Fontan operation. There are limited data on the outcomes of late atrioventricular valve surgery following Fontan operation., Methods: Patients who underwent atrioventricular valve surgery following Fontan procedure were identified from the Mayo Clinic Fontan database. Medical records were reviewed for pre-operative, operative, and post-operative clinical and haemodynamic data. All patients not known to be deceased were sent health status questionnaires., Results: A total of 61 patients (28 females) underwent atrioventricular valve surgery following Fontan procedure. The median age at atrioventricular valve surgery was 14 years. The median duration between Fontan and atrioventricular valve surgery was 4.7 years. Median follow-up was 9 years. There were a total of 32 (52%) deaths with 8 (13%) within 30 days of surgery. The 5-, 10-, and 15-year survival rates were 67%, 57%, and 45%, respectively. On follow-up, 44 of 61 (72%) had arrhythmias, 21 of 29 (72%) were symptomatic, and 12 of 61 (20%) developed protein-losing enteropathy. On multivariate analysis, reduced ventricular function and development of protein-losing enteropathy were associated with decreased survival., Conclusion: Atrioventricular valve surgery after Fontan procedure is associated with substantial late morbidity and mortality. Atrioventricular valve surgery in this cohort of patients portends poor long-term outcome and is associated with a high incidence of protein-losing enteropathy. Reduced ventricular function and development of protein-losing enteropathy were associated with decreased survival.

A 25-year-old man had complained of sudden fever spikes for two years and his blood tests were within the normal range. In 1993, a surgical biopsy of swollen left inguinal lymph nodes was negative for malignancy, but showed reactive lymphadenitis and widespread sinus histiocytosis. A concomitant needle biopsy of the periaortic lymph nodes and a bone marrow aspirate were also negative. In 1994, after an emergency hospital admission because of a sport-related thoracic trauma, a right inguinal lymph node biopsy demonstrated Hodgkin's lymphoma Stage IVB (scleronodular mixed cell subtype). Although it was improved by chemotherapy, the disease suddenly relapsed, and a further lymph node biopsy was performed in 1998 confirming the same diagnosis. Despite further treatment, the patient died of septic shock in 2004, at the age of 38 years. Retrospective analysis of the various specimens showed intracellular heavy metal nanoparticles within lymph node, bone marrow, and liver samples by field emission gun environmental scanning electron microscopy and energy dispersive spectroscopy. Heavy metals from environmental pollution may accumulate in sites far from the entry route and, in genetically conditioned individuals with tissue specificity, may act as cofactors for chronic inflammation or even malignant transformation. The present anecdotal report highlights the need for further pathologic ultrastructural investigations using serial samples and the possible role of intracellular nanoparticles in human disease.

In adults, the left atrial (LA) volume has been shown to reflect diastolic function and is a powerful predictor of cardiac morbidity and mortality. Normative LA volume values in children and the effect of loading conditions on the LA volume in those with congenital heart disease are lacking. The purposes of the present study were to (1) establish normal LA volume values for children, (2) assess the effect of left ventricular volume loading conditions on LA volume, and (3) describe the effect of abnormal myocardial relaxation on the LA volume. We retrospectively reviewed the echocardiograms from 3 pediatric cohorts: group N (n = 522), children with normal echocardiographic findings; group VSD/PDA (n = 71), children with ventricular septal defect (VSD; n = 50) or patent ductus arteriosus (PDA; n = 21); and group HC (n = 63), children with hypertrophic cardiomyopathy (HC). In group N, we identified the LA volume indexed to the body surface area (LA volume index) as a consistent measure of the LA volume in children 3 to 23 months old (mean 16 ± 3 ml/m(2)) and 2 to 17 years old (mean 22 ± 4 ml/m(2)). LA dilation was more common in group VSD/PDA than in group N (27% vs 2%, p <0.0001) and in children with moderate or large shunts than in those with smaller shunts (61% vs 5%, p <0.0001). In group HC, the LA volume index correlated with the mitral valve E/e' ratio (p <0.0001). In conclusion, this is the first study to establish normal pediatric LA volume values. The LA volume index is a reproducible measure of LA size in children ≥ 3 months old. The LA volume index reflects a chronically increased left ventricular volume load in children with VSD or PDA and chronically elevated left ventricular filling pressures in children with HC., (Copyright © 2010 Elsevier Inc. All rights reserved.)

Background: Patients undergoing repair of partial atrioventricular septal defect (AVSD), are reportedly more likely to require reoperation for subaortic obstruction and left atrioventricular valve abnormalities when compared with repair of complete AVSD. Our objective was to examine reoperations after initial repair of partial AVSD, and determine late outcome., Methods: Between 1962 and 2006, 96 patients (63 female) underwent reoperation at our institution after prior repair of partial AVSD (median interval, 10 years; range, 8 days to 48 years). Median age at first reoperation was 26 years (range, 10 months to 71 years). Indications for the first reoperation included left atrioventricular valve (LAVV) regurgitation in 64 patients (67%), subaortic stenosis in 24 (25%), right atrioventricular valve regurgitation in 21 (22%), residual ASD in 11 (11%), and other in 6 (6%)., Results: The first reoperation included LAVV repair in 38 patients, LAVV replacement in 35, subaortic fibrous resection-myectomy in 22, right atrioventricular valve regurgitation surgery in 19, and other in 14. There were five early deaths (5.2%) after first reoperation; three were prior to 1983. There was no significant difference in 20-year survival after LAVV repair or replacement (69% vs 55%, p = 0.20). At last follow-up (median, 5.2 years; max, 34 years), 81 of 89 late survivors were in New York Heart Association functional class I or II., Conclusions: The most common indication for reoperation after initial repair of partial AVSD is LAVV pathology, followed by recurrent left ventricular outflow tract obstruction. The need for further reoperation and late survival is similar between patients undergoing valve repair or replacement. When reoperation is required overall late survival is significantly reduced., (Copyright (c) 2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

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.

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.

Background: Pericarditis requiring pericardiectomy is uncommon in the pediatric population. The aim of this study is to characterize our experience with this subset of patients., Methods: Between February 1978 and May 2008 pericardiectomy was performed on 27 pediatric patients (25 male). The indication for surgery was inflammatory pericarditis in 16 and constrictive pericarditis in 11. Mean age was 16.7 years (range, 3 to 21 years). Chest pain was the most common presenting complaint. Median duration of symptoms prior to operation was 1 year. Most patients had aggressive pharmacologic treatment prior to operation. Before pericardiectomy, 10 patients were hospitalized for treatment of symptoms, 15 underwent pericardiocentesis, and 3 had a prior partial pericardiectomy., Results: Twenty-one patients underwent complete pericardiectomy, 3 a biventricular pericardiectomy, and 3 a completion pericardiectomy. Pathologic histology of all specimens was positive for pericarditis. Pericardial cultures were obtained in 13 cases with bacteria retrieved from only 2 specimens. Median length of stay was 7 days, and the majority had an uneventful postoperative course. The one early mortality was due to acute hepatic failure in a patient with radiation-induced heart disease, 155 days after operation. After median follow-up of 1 year, complete resolution of symptoms was achieved in 89% of patients., Conclusions: In properly selected pediatric patients, complete pericardiectomy can be performed with good outcomes. Although the etiology of pericardial irritation is frequently elusive, resolution of symptoms can be expected in most patients. Confronted with medically refractory pericarditis, earlier consideration for pericardiectomy may be warranted.

Pulmonary venous pathway obstruction is a late complication of the atrial switch operation for transposition of the great arteries. Gaining peripheral access to the pulmonary venous baffle obstruction to treat the obstruction with stent deployment is difficult if not impossible. We present three patients in which we used hybrid procedures in the operating room to relieve the pulmonary venous pathway obstructions.

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.

Background: Excellent surgical results have been reported after repair of complete atrioventricular septal defects (CAVSD); however, 5% to 10% require reoperation. We examine causes leading to reoperation and evaluate long-term outcome., Methods: Between 1972 and 2007, 50 patients (26 male) underwent reoperation at our institution after initial repair of CAVSD (median interval, 15 months; range, 3 days to 29 years). Median age at first reoperation was 4.5 years (range, 53 days to 38 years). Indications for first reoperation included left atrioventricular valve (LAVV) regurgitation in 41 patients, subaortic stenosis in 5, and LAVV stenosis, residual atrial septal defect (ASD), pulmonary artery (PA) stenosis, and aortic coarctation in 1 each., Results: The first reoperation included LAVV repair in 21 patients and replacement in 21, modified Konno procedure in 3, septal myectomy in 2, and PA reconstruction, coarctation repair, and ASD re-repair in 1 each. After LAVV repair (n = 21) 5 patients required a second reoperation, and after LAVV replacement (n = 21) 6 patients required a second reoperation. Overall freedom from further reoperation after the first reoperation was 63%, 48%, and 42% at 5, 10, and 15 years, respectively. There were 2 early deaths (4%) after first reoperation, and none after subsequent reoperations. During late follow-up (median 10.7 years, maximum 30 years), actuarial overall survival was 91%, 91%, and 86% at 5, 10, and 15 years, respectively., Conclusions: The most common indication for reoperation after CAVSD repair is LAVV regurgitation. LAVV re-repair offers good durability, and LAVV replacement does not preclude additional reoperations. Long-term survival is very good despite need for multiple reoperations in some.

Objectives: We sought to determine safety, recurrence rates, and novel risk factors for recurrence in patients with cryptogenic stroke/transient ischemic attack (TIA) after patent foramen ovale closure., Background: Patent foramen ovale closure in patients with cryptogenic stroke/TIA remains highly controversial. There are limited data on long-term recurrence rates and their predictors in these patients., Methods: The records of all patients who underwent patent foramen ovale device closure between December 2001 and June 2006 were reviewed. Patients were seen for clinical follow-up at 3 months then followed annually via telephone. Primary end points were recurrent stroke/TIA. Kaplan-Meier methods were used to estimate recurrent event rates. Cox regression analysis was used to identify risk factors for recurrences., Results: There were 352 patients with cryptogenic stroke (n = 225) or TIA (n = 118) with a mean age of 53.4 years. The procedural complication rate was 3.4%. Recurrent events occurred in 8 patients: 7 strokes and 2 TIA, 1 patient had 2 recurrent strokes. The recurrence rate was 0.6% and 2.1% for stroke and 0.3% and 0.7% for TIA at 1 and 4 years, respectively. The combined end point of recurrent stroke/TIA occurred at a rate of 0.9% and 2.8% at 1 and 4 years, respectively. Risk factors for recurrences were elevated pulmonary artery pressure (hazard ratio [HR]: 1.12, p = 0.009), elevated right ventricular pressure (HR: 1.09, p = 0.04), factor V Leiden mutation (HR: 7.42, p = 0.014), and protein S deficiency (HR: 12.2, p = 0.002). Residual shunt and atrial septal aneurysm were not associated with recurrences., Conclusions: Patent foramen ovale device closure is safe and is associated with a low recurrence of stroke/TIA. Factors associated with recurrence are thrombophilia and elevated intracardiac pressures.

Our objective was to determine the relative merits of using a bioprosthetic porcine valve (BPV) versus a mechanical valve (MechV) when tricuspid valve (TV) replacement is required in patients with Ebstein anomaly. From 1972 to 2006, 333 patients received a BPV and 45 received a MechV. Patient records were reviewed, vital status ascertained, and all patients not known to be deceased were mailed a medical questionnaire or contacted by telephone. Early mortality was not statistically higher for patients who had a MechV (11%) than for those who had a BPV (5%) inserted in the TV position (p = 0.173). The only independent preoperative predictor of operative mortality was moderate to severe left ventricular dysfunction (odds ratio 3.1, p = 0.03); 20-year survival was better in patients who had a BPV (75%) than for those who had a MechV (43%, p = 0.003). On multivariate analysis, after adjusting for ablation of accessory pathways, sinus rhythm at dismissal, and concomitant repair of pulmonary valve stenosis, a BPV remained a predictor of late survival (hazard ratio 0.42, p = 0.004). Survival free of reoperation on the TV at 20 years postoperatively was similar for patients who had a MechV (49%) compared with those who had a BPV (42%) inserted (p = 0.941). A greater percentage of patients who had a MechV reported endocarditis (12% vs 2%), bleeding requiring hospitalization (6% vs 3%), and thrombosis (12% vs 6%); however, none of these differences were statistically significant. In conclusion, a BPV in the tricuspid position was an independent predictor of improved survival. This may be related to the higher incidence of bleeding and thrombotic complications in the patients with MechVs or may be related to differences between the 2 groups. A BPV may offer superior late survival when compared with a MechV when TV replacement is required in patients with Ebstein anomaly, but patient selection must be individualized.

Our objective was to examine the outcomes of patients with left ventricular (LV) dysfunction who underwent operation for Ebstein anomaly. From April 1, 1972 to January 1, 2006, 539 patients with Ebstein anomaly underwent operation at Mayo Clinic. LV function was determined by echocardiography. Of the 495 patients with preoperative echocardiographic assessment of LV function, 50 had moderate or severe LV systolic dysfunction. In patients with LV dysfunction, the tricuspid valve (TV) was repaired in 12 patients and replaced in 36 patients; 1 patient had a 1.5 ventricle repair, and 1 patient had cardiac transplantation. There were 5 early deaths (10%). LV function improved in all but 4 patients after operation. In no patient did LV function worsen after operation. The 1-, 5-, and 10-year survival was 86%, 77%, and 67%, respectively. On univariate analysis, absence of sinus rhythm at dismissal (p = 0.003) was associated with greater overall mortality. For the entire cohort of 539 patients, LV dysfunction was independently predictive of late mortality (hazard ratio 3.76, p <0.001). At late follow-up (mean 6.9 years), 86% of patients were in New York Heart Association class I or II. In conclusion, LV systolic dysfunction occurs infrequently in patients with Ebstein anomaly and is a risk factor for increased late mortality. Although early mortality is greater in patients with LV dysfunction, the late results are favorable. Decreasing LV function should be an indication to promptly restore TV competence rather than a contraindication to TV operation.

Percutaneous branch pulmonary artery (PA) stenting can be challenging, especially in patients with stenosis of the right ventricular (RV) outflow tract or tortuous PA branches. In these cases, a hybrid procedure deploying PA stent(s) during cardiac surgery provides an alternative to relieve branch PA stenosis. The Mayo Clinic Congenital Cardiac surgical database was used to identify all patients having hybrid PA stent procedures. Retrospective analysis of clinical data, procedural details, and outcomes was performed. Between January 1997 and November 2006, 24 patients (15 females), median age 15 years (range 3 to 67 years), had hybrid PA stent procedures. A total of 27 stents were deployed. A left PA stent was placed in 13, right PA stent in 8; 3 patients had bilateral PA stents. Primary cardiac diagnoses were pulmonary atresia (9), tetralogy of Fallot (7), tricuspid atresia (2), and others (6). Maximum balloon diameters ranged from 8 to 16 mm (median = 12 mm). Concomitant surgical procedures performed were RV to PA conduit replacement or RV outflow tract reconstruction (14), pulmonary valve replacement (7), and others (3). Two procedures were performed following complications of percutaneous procedure. There were no deaths or PA damage. There were 2 cases of distal stent migration. Repeat stent dilations within 6 months were performed in 3 patients. In conclusion, hybrid PA stenting can play an important role in the management of congenital heart disease with complex branch PA anatomy. It also can be used as an emergency rescue procedure following complications of percutaneous transcatheter procedures, such as stent embolization. Hybrid procedures were safe and effective in most patients, although stent positioning remains critical. Intraoperative fluoroscopy and active suture fixation of the proximal stent may reduce the need for late reintervention.

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.