Publications by authors named "Peter J Bartz"

32 Publications

Identifying self-reported neurocognitive deficits in the adult with congenital heart disease using a simple screening tool.

Congenit Heart Dis 2018 Sep 31;13(5):728-733. Epub 2018 Jul 31.

Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin.

Objective: Children with congenital heart disease (CHD) and adults with acquired heart disease are at an increased risk of neurocognitive impairment. The objective of this study was to determine the prevalence of self-reported neurocognitive impairment and its risk factors in the adult congenital heart disease (ACHD) population.

Design: The Wisconsin Adult Congenital Heart Disease Program recently began screening ACHD patients to identify those with significant self-perceived neurocognitive impairments. Screening consists of using a validated neuro-oncology screening instrument that has been modified for the ACHD population. Patients who answer this survey in a predetermined fashion consistent with significant self-perceived neurocognitive deficits are referred for a formal neurocognitive evaluation. Demographic and clinical information are obtained by chart review.

Results: Three hundred ten patients (49% males) completed the screening process. The average age was 30 years (range: 17-69 years). For the cohort, 57 (18%) patients had no prior cardiac surgeries, 85 (28%) one surgery, 77 (25%) two, and 91 (29%) at least three surgeries. Of those screened, 106 (34%) met criteria for a formal neurocognitive evaluation. Patients who were referred had undergone a greater number of prior cardiac surgeries (2.2 vs 1.7, P = .008) and were more likely to have severe complexity CHD (P = .006). Of those patients who were referred, the worst perceived functioning was in math and attention.

Conclusion: There is a high prevalence of ACHD patients with significant self-perceived neurocognitive deficits. Simple screening questionnaires may help identify those patients at high risk and allow for timely and appropriate referral for formal neurocognitive evaluation, diagnosis, and therapy.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/chd.12646DOI Listing
September 2018

Anatomic Repair of Congenitally Corrected Transposition of the Great Arteries: Single-Center Intermediate-Term Experience.

Pediatr Cardiol 2017 Dec 16;38(8):1696-1702. Epub 2017 Sep 16.

Cincinnati Children's Hospital Medical Center, University of Cincinnati, 3333 Burnet Ave, Cincinnati, OH, 45229, USA.

We present our experience for patients who have undergone an anatomic repair (AR) for congenitally corrected transposition of the great arteries (CCTGA) at the Children's Hospital of Wisconsin. A retrospective chart review of patients who underwent AR for CCTGA from 2001 to 2015 was performed. The cohort consisted of 15 patients (74% male). Median age of anatomic repair was 15 months (range 4.5-45.6 months). Four patients had a bidirectional Glenn (BDG) prior to AR. At the time of AR,-9 (60%) underwent Senning/Rastelli procedure, 4 (26.6%) had double switch operation, and 2 (13.3%) underwent only Senning with VSD closure. Median duration of follow-up was 5.5 years (0.05-14 years). Reoperations prior to discharge included BDG, revision of pulmonary venous baffle, closure of residual VSD, and pacemaker placement. Late reoperations included left ventricular outflow tract obstruction repair, conduit replacement, melody valve placement, and pacemaker implantation. At their most recent follow-up, no patient had heart failure symptoms and only 1 had severely diminished function that improved with cardiac resynchronization therapy. Moderate mitral regurgitation was noted in 15% (2/13), and severe in 7% (1/13). Moderate tricuspid regurgitation was noted in 15% (2/13). One patient, 7% (1/13), developed moderate aortic insufficiency. There was a 100% survival at the time of the most recent follow-up. Patients with CCTGA who have undergone AR have excellent functional status and mid-term survival but reinterventions are common. Longer term studies are needed to determine both the extent and spectrum of reinterventions as well as long term survival.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00246-017-1715-5DOI Listing
December 2017

The prevalence of sexual dysfunction and its association with quality of life in adults with congenital heart disease.

Int J Cardiol 2017 Feb 19;228:953-957. Epub 2016 Nov 19.

Divisions of Pediatric Cardiology and Adult Cardiovascular Medicine, Departments of Pediatrics and Internal Medicine, Medical College of Wisconsin, USA. Electronic address:

Background: The prevalence of sexual dysfunction (SD) and its impact on quality of life (QOL) in adults with congenital heart disease (CHD) is not well known. The aims of this study were to: determine the prevalence of SD, evaluate the risk factors associated with SD, and determine the association between SD and QOL in adults with CHD.

Methods: This was a cross-sectional study of adults (≥18years) with CHD presenting for routine follow-up at our institution. Subjects completed the CDC HRQOL-14 "Health Days Measure" to assess mental and physical health, and either the Sexual Health Inventory for Men or the Female Sexual Function Index to assess sexual function. Baseline characteristics were obtained via chart review.

Results: 105 subjects were enrolled. The mean age was 31.9±11.7years, 53 (51%) were men, 81% were NYHA Functional Class 1, and 76% had moderate or complex CHD. The rates of SD were 28% overall in the cohort, 30% in men and 25% in women. Men with SD were more likely to be taking spironolactone (p<0.001) and digoxin (p=0.002). Men with SD reported a greater number of days of poor mental health (p=0.004), feeling anxious, worried or tense (p=0.003), needing assistance (p=0.042), and inhibited activity of daily living (p=0.009). Women with SD were more likely to have atrial arrhythmias (p=0.002) and to report fewer days feeling healthy and energetic (p=0.031).

Conclusions: SD is highly prevalent and associated with several indicators of worse health-related QOL in this young and well-functioning population.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ijcard.2016.11.192DOI Listing
February 2017

Center Variability in Timing of Stage 2 Palliation and Association with Interstage Mortality: A Report from the National Pediatric Cardiology Quality Improvement Collaborative.

Pediatr Cardiol 2016 Dec 24;37(8):1516-1524. Epub 2016 Aug 24.

Divison of Cardiology, Department of Pediatrics, Medical College of Wisconsin, 9000 W Wisconsin Ave, Milwaukee, WI, 53226, USA.

For infants with single-ventricle lesions with aortic arch hypoplasia, the interstage period from discharge following stage 1 palliation (S1P) until stage 2 palliation (S2P) remains high risk. Significant variability among institutions exists around the timing of S2P. We sought to describe institutional variation in timing of S2P, determine the association between timing of S2P and interstage mortality, and determine the impact of earlier S2P on hospital morbidity and mortality. The National Pediatric Cardiology Quality Improvement Collaborative registry was queried. Centers were divided based on median age at S2P into early (n = 15) and late (n = 16) centers using a cutoff of 153 days. Groups were compared using Chi-squared or Wilcoxon rank-sum test. Multivariable logistic regression was used to determine risk factors for interstage mortality. The final cohort included 789 patients from 31 centers. There was intra- and inter-center variability in timing of S2P, with the median age by center ranging from 109 to 214 days. Late centers had a higher mortality (9.9 vs. 5.7 %, p = 0.03) than early centers. However, the event rate (late: 8.2 vs. early: 5.8 deaths per 10,000 interstage days) was not different by group (p = 0.26). Survival to hospital discharge and hospital length of stay following S2P were similar between groups. In conclusion, in a large multi-institution collaborative, the median age at S2P varies among centers. Although optimal timing of S2P remains unclear, centers performing early S2P did not experience worse S2P outcomes and experienced less interstage mortality.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00246-016-1465-9DOI Listing
December 2016

Growth Trajectory in Children with Trisomy 21 with and without Atrioventricular Septal Defect.

Congenit Heart Dis 2016 Jul 30;11(4):348-53. Epub 2016 May 30.

Pediatric Cardiology, Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, Wis, USA.

Objective: Trisomy 21 is associated with poor weight gain and atrioventricular septal defects. The impact of atrioventricular septal defects on weight gain in the setting of Trisomy 21 has not previously been described in the recent era. This study aimed to determine if such an association is present.

Design: Patients with Trisomy 21 with and without atrioventricular septal defects were identified. Clinical, surgical, and postoperative data were collected were for these patients and then compared between patients. Specifically, weight for age z-scores were compared at various time points in a univariate and multivariate fashion. Effect of timing of surgery in those with an atrioventricular septal defect was also studied.

Results: A total of 86 patients were identified, 42 with an atrioventricular septal defect. There was a difference in weight for age z-scores between patients with and without atrioventricular septal defects only at 2 months (P value .038) and 6 months (P value .003) of age. This persisted after multivariate regression which demonstrated atrioventricular septal defects as an independent risk factor. There was no difference noted in weight at 2 years of age in patients undergoing atrioventricular septal defect repair before and 150 days of life.

Conclusion: There was a statistically significant, but not clinically relevant, difference in weight between the patients with Trisomy 21 with and without atrioventricular septal defects in our cohort. Those with atrioventricular septal defects required more nutritional intervention, such as gastrostomy tube placement. Timing of repair did not alter outcomes at midterm follow-up.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/chd.12378DOI Listing
July 2016

The Impact of Obesity on Postoperative Outcomes in Adults with Congenital Heart Disease Undergoing Pulmonary Valve Replacement.

Congenit Heart Dis 2015 Sep-Oct;10(5):E197-202. Epub 2015 Apr 27.

Department of Pediatrics, Division of Pediatric Cardiology, Medical College of Wisconsin, Milwaukee, Wis, USA.

Objective: The impact of obesity on surgical morbidity in adults with congenital heart disease is currently unknown. The aim of our study was to investigate the impact of obesity on postoperative outcomes in adults with congenital heart disease undergoing reoperation for pulmonary valve replacement.

Methods: A retrospective analysis was performed assessing the influence of obesity on surgical outcomes. Obesity was defined as a body mass index ≥30 kg/m2.

Results: The mean body mass index of the cohort was 25.9 ± 6.9 kg/m2 . The cohort included 71 patients with 17 patients (24%) being obese. There was no postoperative mortality. Obese patients had a longer hospital length of stay (6.6 vs. 4.7 days; P < .001) and increased incidence of postoperative arrhythmias (29% vs. 5.6%; P = .003) compared with nonobese patients. Multivariable analysis performed using logistic regression with backwards elimination demonstrated obesity was independently associated with hospital length of stay >5 days (odds ratio [OR] = 5.2; 95% confidence interval [CI]: 1.5-18.2, P = .01) and with increased postoperative arrhythmias (OR = 4.2; 95% CI: 1.7-40, P < .01).

Conclusions: Obesity is associated with increased morbidity in adults with congenital heart disease undergoing pulmonary valve replacement, including longer hospitalization and higher risk for postoperative arrhythmias.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/chd.12266DOI Listing
August 2016

Tricuspid repair at pulmonary valve replacement does not alter outcomes in tetralogy of Fallot.

Ann Thorac Surg 2015 Mar 14;99(3):899-904. Epub 2015 Jan 14.

Department of Pediatrics, Division of Cardiology, Medical College of Wisconsin, Milwaukee, Wisconsin; Department of Internal Medicine, Division of Adult Cardiovascular Medicine, Medical College of Wisconsin, Milwaukee, Wisconsin. Electronic address:

Background: Chronic pulmonary regurgitation after tetralogy of Fallot repair often leads to progressive right ventricle dilation, dysfunction, and frequently, pulmonary valve replacement. For those with significant tricuspid regurgitation at the time of pulmonary valve replacement, it is unknown whether concomitant tricuspid valve repair improves postoperative outcomes.

Methods: This is a retrospective review of patients after tetralogy of Fallot repair who underwent pulmonary valve replacement between 1999 and 2012. Preoperative and postoperative echocardiograms were assessed for tricuspid regurgitation (vena contracta) and right ventricular size and function (Tomtec software).

Results: Sixty-two patients underwent pulmonary valve replacement. Thirty-six (58%) had greater than or equal to moderate tricuspid regurgitation on preoperative echocardiogram. Significant predictors were not identified. Of the 36, 18 (50%) underwent concomitant tricuspid valve repair at the time of pulmonary valve replacement. After surgery, there was a significant reduction in the degree of tricuspid regurgitation (p < 0.001) and measures of right ventricular size (p < 0.05) in both cohorts. Between surgical groups, there was no statistical difference in the grade of tricuspid regurgitation (p = 0.47) or measures of right ventricular size (p > 0.4) at 6-month follow-up.

Conclusions: Tricuspid regurgitation is a common finding in repaired tetralogy of Fallot, although risk factors for its development remain unclear. After pulmonary valve replacement with or without tricuspid valve repair there is significant improvement in the degree of tricuspid regurgitation and right ventricular size. Finally, 6 months after pulmonary valve replacement there were no statistical differences between those patients undergoing concomitant tricuspid valve repair and those undergoing pulmonary valve replacements alone.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.athoracsur.2014.09.086DOI Listing
March 2015

Improving interstage survival after Norwood operation: outcomes from 10 years of home monitoring.

J Thorac Cardiovasc Surg 2014 Oct 14;148(4):1540-7. Epub 2014 Feb 14.

Section of Critical Care, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wis; Herma Heart Center, Children's Hospital of Wisconsin, Milwaukee, Wis.

Objective: Infants who undergo Norwood stage 1 palliation (S1P) continue with high-risk circulation until stage 2 palliation (S2P). Routine care during the interstage period is associated with 10% to 20% mortality. This report illustrates the sustained reduction of interstage mortality over 10 years associated with use of home monitoring.

Methods: Daily monitoring of oxygen saturation and weight was done for all patients discharged to home after S1P. Notification of the care team occurred for oxygen saturation<75% or >90%, weight gain<20 g over 3 days, weight loss>30 g, or intake<100 cc/kg/d. Breach of these criteria marked an interstage event. Interstage outcomes are reported. Patient characteristics and perioperative variables were compared between patients with and without interstage events.

Results: Over 10 years, 157 patients were discharged after S1P with home monitoring. Interstage survival was 98%. Breach of home criteria occurred in 59% (93 out of 157), with violation of oxygen saturation<75% the most common event. Patient characteristics, operative data, and early postoperative morbidity did not differ between patients with and without events.

Conclusions: Home monitoring after S1P is associated with excellent interstage survival. Although a breach of monitoring criteria occurred in more than half of patients, our analysis failed to identify independent predictors of interstage events. Analysis of variables predicting mortality could not be assessed due to the low frequency of death in this cohort. Failure to identify specific variables for interstage events suggests that home monitoring, as part of an interstage surveillance program, should be applied to all S1P hospital survivors.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jtcvs.2014.02.038DOI Listing
October 2014

Effect of feeding modality on interstage growth after stage I palliation: a report from the National Pediatric Cardiology Quality Improvement Collaborative.

J Thorac Cardiovasc Surg 2014 Oct 8;148(4):1534-9. Epub 2014 Feb 8.

Division of Critical Care, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wis.

Objectives: Achieving adequate growth after stage 1 palliation for children with single-ventricle heart defects often requires supplemental nutrition through enteral tubes. Significant practice variability exists between centers in the choice of feeding tube. The impact of feeding modality on the growth of patients with a single ventricle after stage 1 palliation was examined using the multiinstitutional National Pediatric Cardiology Quality Improvement Collaborative data registry.

Methods: Characteristics of patients were compared by feeding modality, defined as oral only, nasogastric tube only, oral and nasogastric tube, gastrostomy tube only, and oral and gastrostomy tube. The impact of feeding modality on change in weight for age z-score during the interstage period, from stage 1 palliation discharge to stage 2 palliation, was evaluated by multivariable linear regression, adjusting for important patient characteristics and postoperative morbidities.

Results: In this cohort of 465 patients, all groups demonstrated improved weight for age z-score during the interstage period with a mean increase of 0.3±0.8. In multivariable analysis, feeding modality was not associated with differences in the change in weight for age z-score during the interstage period (P=.72). Risk factors for poor growth were a diagnosis of hypoplastic left heart syndrome (P=.003), vocal cord injury (P=.007), and lower target caloric goal at discharge (P=.001).

Conclusions: In this large multicenter cohort, interstage growth improved for all groups and did not differ by feeding modality. With appropriate caloric goals and interstage monitoring, adequate growth may be achieved regardless of feeding modality and therefore local comfort and complication risk should dictate feeding modality.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jtcvs.2014.02.025DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4126898PMC
October 2014

Feeding dysfunction in children with single ventricle following staged palliation.

J Pediatr 2014 Feb 22;164(2):243-6.e1. Epub 2013 Oct 22.

Division of Cardiology, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI; Division of Adult Cardiovascular Medicine, Department of Internal Medicine, Medical College of Wisconsin, Milwaukee, WI.

Objective: To determine the prevalence of feeding dysfunction in children with single ventricle defects and identify associated risk factors.

Study Design: Patients aged 2-6 years with single ventricle physiology presenting for routine cardiology follow-up at the Children's Hospital of Wisconsin were prospectively identified. Parents of the patients completed 2 validated instruments for assessment of feeding dysfunction. Chart review was performed to retrospectively obtain demographic and diagnostic data.

Results: Instruments were completed for 56 patients; median age was 39 months. Overall, 28 (50%) patients had some form of feeding dysfunction. Compared with a normal reference population, patients with single ventricle had statistically significant differences in dysfunctional food manipulation (P < .001), mealtime aggression (P = .002), choking/gagging/vomiting (P < .001), resistance to eating (P < .001), and parental aversion to mealtime (P < .001). Weight and height for age z-scores were significantly lower in subjects with feeding dysfunction (-0.84 vs -0.33; P < .05 and -1.46 vs -0.56; P = .001, respectively). Multivariable analysis identified current gastrostomy tube use (P = .02) and a single parent household (P = .01) as risk factors for feeding dysfunction.

Conclusion: Feeding dysfunction is common in children with single ventricle defects, occurring in 50% of our cohort. Feeding dysfunction is associated with worse growth measures. Current gastrostomy tube use and a single parent household were identified as independent risk factors for feeding dysfunction.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jpeds.2013.09.030DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3946833PMC
February 2014

The spectrum of congenital heart disease and outcomes after surgical repair among children with Turner syndrome: a single-center review.

Pediatr Cardiol 2014 Feb 10;35(2):253-60. Epub 2013 Aug 10.

Medical College of Wisconsin, Milwaukee, WI, USA,

Turner syndrome (TS), a genetic abnormality affecting 1 in 2,500 people, is commonly associated with congenital heart disease (CHD). However, the surgical outcomes for TS patients have not been well described. This study reviewed the spectrum of CHD in TS at the authors' center. The authors report outcomes after coarctation of the aorta (CoA) repair or staged palliation of hypoplastic left heart syndrome (HLHS) and then compare the surgical outcomes with those of non-TS patients undergoing like repair. This retrospective chart review was conducted at the Children's Hospital of Wisconsin from 1999 to 2011. Of the 173 patients with TS, 77 (44.5 %) were found to have CHD. Left-sided obstructive lesions were the most common. However, the spectrum of CHD was wide and included systemic and pulmonary venous abnormalities as well as abnormalities of the coronary arteries. In the comparative analysis of CoA repair, the TS patients younger than 60 days had longer aortic cross-clamp times (24 vs. 16 min; p = 0.001) and longer hospital stays (12 vs. 6 days; p ≤ 0.0001) than the non-TS patients. At the follow-up assessment after 8.8 ± 9.1 years, 17 % of the TS patients had hypertension, but no patient had required reintervention, and no deaths had occurred. Finally, three of the four TS patients with HLHS died within the first year. The spectrum of CHD within TS is wide and not limited to bicuspid aortic valve or CoA. Additionally, patients with TS undergoing CoA repair may have a more challenging early postoperative course but experience outcomes similar to those of non-TS patients. Finally, patients who have TS combined with HLHS remain a challenging population with generally poor survival.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00246-013-0766-5DOI Listing
February 2014

Extracardiac complications in adults with congenital heart disease.

Congenit Heart Dis 2013 Sep-Oct;8(5):370-80. Epub 2013 May 13.

The Wisconsin Adult Congenital Heart Disease Program (WAtCH), Medical College of Wisconsin, Milwaukee, Wis, USA.

With the increasing number of adults living with repaired, or unrepaired, congenital heart disease, there is a growing incidence of extracardiac comorbidities. These comorbidities can affect various organ systems in complex ways, and may have a significant impact on a patient's quality of life and survival. Many of these potential complications may go undiagnosed until there is already a significant bearing on the patient's life. Therefore, it is important for physicians who care for the adult congenital patient to be mindful of these potential extracardiac complications, and actively assess for these complications in their adult congenital practice. Continued research to identify modifiable risk factors is needed so that both preventative and therapeutic management options for these extracardiac complications may be developed.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/chd.12080DOI Listing
May 2014

Significant response to oral Etoposide in the treatment of an unresectable cardiac sarcoma.

J Pediatr Hematol Oncol 2014 May;36(4):e237-40

*Department of Pediatrics, Division of Hematology-Oncology-BMT †Department of Pediatrics, Division of Cardiology ‡Department of Surgery, Division of Pediatric Surgery §Department of Pathology ∥Department of Surgery, Division of Cardiothoracic Surgery, Medical College of Wisconsin, Milwaukee, WI.

Primary cardiac sarcomas are rare and carry a poor prognosis. The standard of care is complete resection. Outcomes for patients without complete resection are dismal, and the benefit of adjuvant therapy is uncertain. A 9-year-old girl presented with a large right-sided cardiac mass. After biopsy, the tumor was classified as an undifferentiated sarcoma. Resection was not feasible due to apparent invasion of the right ventricle and atrioventricular groove. Treatment with oral etoposide resulted in a 97% reduction in tumor volume and allowed for complete resection of residual tumor. She is alive with no evidence of disease 25 months from diagnosis.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1097/MPH.0b013e318290c3b8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4479394PMC
May 2014

Feeding dysfunction in single ventricle patients with feeding disorder.

Congenit Heart Dis 2014 Jan-Feb;9(1):26-9. Epub 2013 May 7.

Department of Pediatrics, Division of Cardiology, Medical College of Wisconsin, Milwaukee, Wis, USA.

Purpose: To determine whether caregivers of children with single ventricle heart defects identified as having feeding disorder will report more frequent feeding dysfunction, or maladaptive mealtime behavior and/or interactions, when compared with reference populations.

Methods: As part of routine evaluation, parents of children evaluated at the Feeding, Swallowing, and Nutrition Center at the Children's Hospital of Wisconsin completed previously validated questionnaires to assess feeding dysfunction and parental stress. Parental responses for single ventricle patients were compared with all other children evaluated with a feeding disorder.

Results: Questionnaires were completed in eight patients with single ventricle heart defects. The mean age was 36 ± 23 months, with five females (63%). Mean weight-for-age z-score was -1.4 ± 0.9. Compared with noncardiac feeding clinic children, there was more reported child resistance to eating (83 ± 15% vs. 44 ± 2%; P = .05). Single ventricle parents were more likely to report distress (50 ± 18% vs. 21 ± 2%; P = .04) and a difficult child (63 ± 17% vs. 31 ± 2%; P = .05). There was also more defensive responding among parents of single ventricle children (63 ± 17% vs. 29 ± 2%; P = .04).

Conclusion: Single ventricle patients evaluated for disordered feeding more frequently showed resistance to eating and parental distress than noncardiac feeding clinic patients. Parents of these children underestimated the degree of feeding difficulty by defensive responding and had more parental distress. These findings suggest that feeding dysfunction can contribute to longer-term feeding and growth problems in single ventricle patients with feeding disorder.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/chd.12071DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4422377PMC
September 2014

Atrial tachyarrhythmias and the Cox-maze procedure in congenital heart disease.

Congenit Heart Dis 2013 Sep-Oct;8(5):434-9. Epub 2012 Dec 27.

Department of Pediatrics and Internal Medicine, Medical College of Wisconsin, Milwaukee, Wis, USA.

Introduction: Atrial tachyarrhythmias, particularly atrial flutter and fibrillation, are commonly associated with congenital heart disease and are a major cause of morbidity and mortality. The Cox-maze procedure, introduced by Dr. James Cox in 1987, is effective at controlling atrial fibrillation in structurally normal hearts. Though the Cox-maze procedure has been used for atrial tachyarrhythmias in patients with congenital heart disease, few studies have looked at its effectiveness.

Methods: A retrospective chart review was performed on 24 patients with congenital heart disease who underwent the Cox-maze procedure at the Medical College of Wisconsin from 2004 through 2010.

Results: Mean age at time of Cox-maze procedure for the cohort was 40.9 years (range, 14 to 66 years). The most common congenital heart diseases among the patients included tetralogy of Fallot (n = 8) and atrioventricular septal defect (n = 4). All patients had concomitant cardiac procedures with the most common being right ventricular outflow tract reconstruction (n = 10), tricuspid valve repair (n = 8), and atrial septal defect repair (n = 7). Prior to the Cox-maze procedure, arrhythmias consisted of atrial flutter or intratrial reentrant tachycardia (n = 19) and atrial fibrillation (n = 5). There were three early postoperative deaths and one late postoperative death. Follow-up was available for 19 of 21 (90%) survivors with a mean length to follow-up from Cox-maze procedure of 2.8 years (range, 0.14-5.7 years). At last follow-up, 14 (74%) of the survivors remained arrhythmia-free.

Conclusions: In patients with congenital heart disease and atrial tachyarrhythmias, the majority were rendered arrhythmia-free by the Cox-maze procedure.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/chd.12031DOI Listing
May 2014

Long-term outcomes of the neoaorta after arterial switch operation for transposition of the great arteries.

Ann Thorac Surg 2013 May 6;95(5):1654-9. Epub 2012 Dec 6.

Department of Pediatrics, Division of Pediatric Cardiology, Milwaukee, Medical College of Wisconsin, Wisconsin.

Background: After the arterial switch operation (ASO) for transposition of the great arteries (TGA), the native pulmonary root and valve function in the systemic position, and the long-term risk for neoaortic root dilation and valve regurgitation is currently undefined. The aim of this study was to determine the prevalence and progression of neoaortic root dilation and neoaortic valve regurgitation in patients with TGA repaired with the ASO.

Methods: Measurements of the neoaortic annulus, neoaortic root at the level of the sinuses of Valsalva, and the degree of neoaortic regurgitation were assessed by serial transthoracic echocardiograms on 124 patients with TGA at a median follow-up of 7.2 years (range, 1 to 23 years) after the ASO at our institution.

Results: Neoaortic root dilation with z scores of 2.5 or greater was identified in 66%, and the root diameter z score increased at an average rate of 0.08 per year over time after ASO. Freedom from neoaortic root dilation at 1, 5, 10, and 15 years after ASO was 84%, 67%, 47%, and 32%, respectively. Risk factors for root dilation include history of double-outlet right ventricle (p = 0.003), previous pulmonary artery banding (p = 0.01), and length of follow-up (p = 0.04). Neoaortic valve regurgitation of at least moderate degree was present in 14%. Neoaortic root dilation was a significant risk factor for neoaortic valve regurgitation (p < 0.0001). No patient required reintervention on the neoaorta or neoaortic valve during follow-up.

Conclusions: Progressive neoaortic root dilation is common in patients with TGA after the ASO. Continued surveillance of this population is required.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.athoracsur.2012.10.081DOI Listing
May 2013

Myocardial infarction due to a retained epicardial pacing wire.

Ann Thorac Surg 2012 Nov;94(5):1724-6

Adult Congenital Heart Disease Program of Wisconsin, Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, WI 53213, USA.

A 20-year-old man with a temporary epicardial pacing wire retained after infant repair of an atrioventricular septal defect presented with cardiopulmonary arrest. After resuscitation with extracorporeal membranous oxygenation, a coronary angiogram demonstrated a retained epicardial pacing wire compressing the left anterior descending artery and obtuse marginals. Owing to poor prognostic findings on a computed tomography scan of his head, he was terminally withdrawn from mechanical support. This case demonstrates a potential devastating complication of a retained temporary epicardial pacing wire.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.athoracsur.2012.04.055DOI Listing
November 2012

Restrictive lung disease is an independent predictor of exercise intolerance in the adult with congenital heart disease.

Congenit Heart Dis 2013 May-Jun;8(3):246-54. Epub 2012 Oct 18.

Division of Cardiology, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI 53226, USA.

Background/objectives: Following repair of congenital heart disease (CHD), adult patients are at risk for reduced exercise capacity. Restrictive lung disease (RLD) may contribute to reduced exercise capacity in this population. The aim of this study was to determine the prevalence of RLD and its impact on exercise tolerance in the adult with CHD.

Methods: One hundred consecutive adult patients with CHD, who underwent routine cardiopulmonary exercise testing with spirometry, were evaluated. Clinical data were obtained by retrospective chart review.

Results: Patients from 10 major diagnostic groups were identified. The median age for the cohort was 31 years (range 18-63) and included 43 males and 57 females. Most patients, 79%, had at least one previous surgical procedure. Based on spirometry and flow/volume loops, 50 patients were classified as normal pulmonary function, 44 patients had patterns suggestive of RLD, 4 suggestive of mixed (obstructive and restrictive), and 2 indeterminate. Risk factors associated with RLD include history of multiple thoracotomies (odds ratio = 9.01, P =.05) and history of atrial arrhythmias (odd ratio = 4.25, P =.05). Overall, 56% of the patients had abnormal exercise capacity. Spirometry suggestive of RLD was a significant risk factor for decreased exercise capacity (odds ratio = 3.65, P =.03). Patients with spirometry suggesting RLD also had lower exercise duration (P =.004) and a higher New York Heart Association Functional Class (P =.02). History of previous surgery and decreased heart rate reserve were also significant risk factors for decreased exercise capacity.

Conclusion: Abnormal spirometry suggestive of RLD is common in the adult with CHD and is a significant risk factor for decreased exercise tolerance in this population. Further studies are needed to evaluate the relationship between RLD and exercise intolerance and its relationship to mortality in the adult with CHD.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/chd.12010DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4832600PMC
January 2014

Predicting left ventricular recovery after replacement of a regurgitant aortic valve in pediatric and young adult patients: is it ever too late?

Pediatr Cardiol 2013 Mar 9;34(3):694-9. Epub 2012 Oct 9.

Division of Cardiology, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI, USA.

The management of pediatric and adolescent patients with pure aortic valve regurgitation remains challenging and controversial (Christos et al., Eur J Cardiothorac Surg 17:125-133, 2000; Gersony and Sommerville, ACC Curr J Rev 31:97-98, 2000; Hasaniya et al., J Thorac Cardiovasc Surg 127:970-974, 2004; Sabet et al., Mayo Clin 74:14-26, 1999; Tweddell et al., J Thorac Cardiovasc Surg 129:551-558, 2005). We evaluated pediatric and young adult patients who underwent aortic valve replacement (AVR) primarily for aortic regurgitation in an effort to identify preoperative echocardiographic variables that are predictive of left ventricular (LV) recovery following AVR. Twenty-one patients with severe aortic valve regurgitation who underwent AVR were identified. Retrospective chart review for each patient was performed and transthoracic echocardiograms prior to and 6-months after AVR were analyzed. Improvement in LV size based on preoperative LV end-systolic dimension index when compared to 6-months post-AVR was observed in 68% of the patients. Patients with persistent dilation of their left ventricles had a greater preoperative LV end-systolic dimension index (p ≤ 0.05), a greater preoperative LV end-systolic dimension z-score (p ≤ 0.002), and a lower preoperative ejection fraction (EF) (p ≤ 0.001). A similar trend was present between the two cohorts in regards to LV end-diastolic parameters (LV end-diastolic dimension index and z-score), with patients with abnormal LV size at 6-month follow-up having larger preoperative dimensions. Increasing LV systolic dimensions and declining EF appear to be predictors of poor LV recovery following AVR in pediatric and young adult patients. LV end-systolic indices appear to be more predictive than LV end-diastolic indices. AVR should be performed prior to severe LV enlargement defined as an LV end-systolic dimension z-score >4.5.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00246-012-0529-8DOI Listing
March 2013

Aortic aneurysms remain a significant source of morbidity and mortality after use of Dacron(®) patch aortoplasty to repair coarctation of the aorta: results from a single center.

Pediatr Cardiol 2013 Feb 28;34(2):296-301. Epub 2012 Jul 28.

Department of Pediatrics, Division of Cardiology, Medical College of Wisconsin, Children's Hospital of Wisconsin, Herma Heart Center, 9000 W Wisconsin Avenue, Milwaukee, WI 53226, USA.

Aortic aneurysm formation after coarctation repair is a serious and life-threatening complication. Repairs using synthetic materials such as Dacron(®) may carry the highest risk of aneurysm formation and rupture. The authors sought to determine the prevalence of aneurysm formation in patients who previously underwent coarctation repair using Dacron(®) patch aortoplasty at their institution. Between 1977 and 1994, 63 patients underwent isolated coarctation repair using Dacron(®) patch aortoplasty. Aneurysms were defined as an aortic dimension 1.5 times that of the aorta at the level of the diaphragm as shown by angiography, computed tomography (CT) scan, or magnetic resonance imaging (MRI). Of 61 early survivors, 29 (47 %) experienced an aneurysm in the area of previous repair. Nine patients (31 %) had spontaneous rupture of the aneurysm, which caused death in seven cases. Elective or emergent aneurysm repair was performed for 20 patients without complication, and 2 patients are being monitored at this writing. The mean interval from patch placement to aneurysm repair was 15 years (range, 4-27 years). Overall freedom from the development of an aortic aneurysm was 97 % at 5 years, 90 % at 10 years, 69 % at 20 years, and 42 % at 25 years. After repair of coarctation using Dacron(®) patch aortoplasty, the risk for aneurysm formation in the area of repair and death from rupture is extremely high. Therefore, in accordance with the 2008 American Heart Association/American College of Cardiology (AHA/ACC) guidelines, all patients with repaired aortic coarctation should undergo either CT or MRI imaging at least every 5 years to assess for aortic aneurysm formation. More frequent imaging should be obtained for patients previously repaired with Dacron(®) patch aortoplasty.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00246-012-0442-1DOI Listing
February 2013

Valve-sparing aortic root reconstruction in children, teenagers, and young adults.

Ann Thorac Surg 2012 Aug 28;94(2):587-90; discussion 590-1. Epub 2012 Jun 28.

Division of Cardiothoracic Surgery, Department of Surgery, Children's Hospital of Wisconsin, Milwaukee, Wisconsin 53226, USA.

Background: We reviewed our experience with valve-sparing aortic root reconstruction (VSARR) using the sinus of Valsalva graft in children, teenagers, and young adults with connective tissue disorders.

Methods: Results of a single-center experience with VSARR in children, teenagers, and young adults were retrospectively analyzed. End points were death, freedom from reintervention, and freedom from valve dysfunction.

Results: Between 2003 and 2010, 16 patients (Marfan, 9; Loeys Dietz syndrome, 6; conotruncal, 1) underwent VSARR. The mean age was 20±7.4 (range, 9 to 36 years). Indications for VSAAR were aortic root enlargement in 14 (sinus of Valsalva Z-score, 6.2±2) and aortic dissection in 2. Additional procedures included replacement of the ascending aorta in 7, with additional replacement of the aortic arch in 2. No early or late deaths occurred. One patient required a pacemaker. One patient with Loeys-Dietz syndrome required reoperation for aneurysmal dilatation of the coronary buttons. Two patients underwent replacement of the thoracoabdominal aorta for chronic dissection. Follow-up by echocardiography or magnetic resonance imaging at a mean of 33±29 months showed more than mild aortic regurgitation in 2 patients. Both patients with moderate aortic insufficiency also had a bicuspid aortic valve.

Conclusions: VSARR using the sinus of Valsalva graft is a reproducible technique that achieves acceptable early and intermediate results. It is suitable for children, teenagers, and young adults. Anticoagulation is avoided. The procedure is appropriate for emergency operations but should be used with caution in patients with a bicuspid aortic valve.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.athoracsur.2011.12.097DOI Listing
August 2012

Renal dysfunction is common among adults after palliation for previous tetralogy of Fallot.

Pediatr Cardiol 2013 Jan 7;34(1):165-9. Epub 2012 Jun 7.

Department of Pediatrics, Medical College of Wisconsin, 9000 W Wisconsin Avenue, Milwaukee, WI 53226, USA.

Long-term survival after tetralogy of Fallot (TOF) repair is excellent. However, little is published regarding late noncardiac complications. This study aimed to determine the prevalence and risk factors for renal dysfunction among adults after TOF repair. For this study, 56 adult patients with complete repair of TOF were identified, and their charts were retrospectively reviewed. An estimated glomerular filtration rate (eGFR) for each patient was calculated using the Modification of Diet in Renal Disease formula (MDRD). Using each patient's eGFR, he or she was classified into stages based on the National Kidney Foundation chronic kidney disease (CKD) staging. Clinical parameters were compared among patients with and those without renal dysfunction to identify risk factors for renal impairment. The median estimated eGFR rate for the cohort was 78 ml/min/1.73 m(2). Based on the National Kidney Foundation CKD staging system, 54 % of the patients had at least stage 2 chronic renal disease. The risk factors identified were hypertension (p < 0.01), type 2 diabetes mellitus (p < 0.05), longer follow-up evaluation (p < 0.005), older age at complete repair (p < 0.05), and use of daily diuretics (p < 0.05). After repair of TOF, renal dysfunction is common at late follow-up evaluation. The study findings show the importance of routine assessment of renal function and the need to limit or avoid future episodes of acute kidney injury in this at-risk population.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00246-012-0408-3DOI Listing
January 2013

Urinary interleukin-18 and urinary neutrophil gelatinase-associated lipocalin predict acute kidney injury following pulmonary valve replacement prior to serum creatinine.

Congenit Heart Dis 2012 Sep-Oct;7(5):441-7. Epub 2012 Apr 27.

Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI 53226, USA.

Background: It is becoming increasingly recognized that manifestations of congenital heart disease (CHD) extend beyond the cardiovascular system. The factors contributing to renal dysfunction in patients with CHD are multifactorial, with acute kidney injury (AKI) at time of cardiac surgery playing a major role. AKI is often diagnosed based on changes in serum creatinine and estimated glomerular filtration rate (eGFR). Such measurements are often late and imprecise. Recent data indicate that urinary biomarkers interleukin-18 (IL-18) and neutrophil gelatinase-associated lipocalin (NGAL) are earlier markers of AKI. We sought to determine the efficacy of urinary IL-18 and NGAL for detecting early AKI in patients undergoing surgical pulmonary valve replacement (PVR).

Methods: Twenty patients presenting for surgical PVR with a history of previous repair of a conotruncal anomaly were enrolled. Preoperative clinical data were measured and urine samples and serum creatinine were collected at 6, 12, 24, and 72 hours post bypass. Urine was evaluated for NGAL and IL-18. AKI was determined using the Risk, Injury, Failure, Loss and End Stage Renal Disease (RIFLE) classification system.

Results: Using the RIFLE classification system, seven patients (35%) were found to have AKI defined as a drop in the eGFR or an increase in serum creatinine. All seven patients with AKI had marked increase from preoperative baseline in urine IL-18 (sixfold) and NGAL (26-fold). Using NGAL and IL-18, AKI was detected at 6 hours postoperatively, resulting in AKI being identified 12-36 hours prior to detection by conventional methods. No preoperative predictors for AKI were identified.

Conclusion: Both NGAL and IL-18 are early predictive biomarkers of AKI, and both increase in tandem after surgical PVR. Importantly, both rise before an increase in creatinine or a decrease in eGFR is present. Monitoring both biomarkers may allow for earlier detection and subsequent interventions to prevent AKI at time of surgery for CHD.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/j.1747-0803.2012.00662.xDOI Listing
February 2013

Late complications after Takeuchi repair of anomalous left coronary artery from the pulmonary artery: case series and review of literature.

Pediatr Cardiol 2012 Oct 22;33(7):1115-23. Epub 2012 Mar 22.

Division of Pediatric Cardiology, Department of Pediatrics, Medical College of Wisconsin, 9000 W. Wisconsin Avenue, MS 713, Milwaukee, WI 53226, USA.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart lesion that if left untreated, results in high mortality. Surgical treatment options include reimplantation of the anomalous coronary ostia, use of extracardiac arterial blood supply, or the Takeuchi procedure. The Takeuchi procedure involves creation of an aortopulmonary window and an intrapulmonary tunnel that baffles the aorta to the ostium of the anomalous left coronary artery. The incidence of late complications following the Takeuchi repair is unknown. The goal of our study was to determine the long-term outcome after palliation of ALCAPA using the Takeuchi Procedure. A total of 9 patients with a history of ALCAPA palliated with the Takeuchi procedure were identified from our surgical database. Chart review was performed. The mean age at time of Takeuchi procedure was 49.6 months (range 5 weeks-14.6 years). There was one late death, of unknown cause. Of the remaining 8 patients, the mean length of follow-up after surgery was 15.9 years (13.5-19.7 years). All 8 survivors had some degree of main pulmonary artery stenosis in the area of the intrapulmonary baffle, with moderate stenosis in 2 and severe stenosis in 1. Three late survivors (38 %) had a baffle leak. Two patients (25 %) had decreased left ventricular systolic function and 3 (38 %) had developed at least moderate mitral valve regurgitation. Three of the 8 late survivors (38 %) required a reoperation for repair of mitral valve regurgitation, baffle leak, and main pulmonary artery (MPA) stenosis. Review of literature demonstrated similar complication rates and need for reoperation following the Takeuchi procedure. Compared with patients after direct reimplantation for ALCAPA at our institution, there was no significant difference in late survival or freedom from reoperation. The Takeuchi procedure is a method to establish a two-coronary repair for ALCAPA. Late complications are common, necessitating lifelong care in a center experienced with caring for adults with congenital heart disease.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00246-012-0260-5DOI Listing
October 2012

Timing and technique of pulmonary valve replacement in the patient with tetralogy of Fallot.

Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2012 ;15(1):27-33

Department of Surgery, Division of Cardiothoracic Surgery, Medical College of Wisconsin, Milwaukee, WI, USA.

Residual right ventricular (RV) outflow tract pathology is universal among patients with repaired tetralogy of Fallot, and pulmonary regurgitation (PR) is also commonly present. Although tolerated in early life, by the second decade of life PR is associated with an increased risk of death because of ventricular arrhythmias. Pulmonary valve replacement (PVR) is a safe procedure that will eliminate PR, but timing and indications are evolving. Patients with arrhythmias or prolonged QRS duration are candidates for PVR. Patients with symptomatic exercise intolerance are likely to have improvement in symptoms and quality of life and should be offered PVR. Cardiac magnetic resonance has become an essential component of the management of the patient with tetralogy of Fallot with PR, and has identified the potential for and limitations of RV remodeling following PVR. Among patients with severe RV enlargement, particularly those with diminished RV or left ventricular function, there is an increased risk of adverse events and even asymptomatic patients with severe PR should be considered for PVR. Valve replacement is accomplished with homografts or heterografts, either stented bioprosthetic valves or valved conduits. In a retrospective analysis of the Children's Hospital of Wisconsin experience with PVR, there was no difference in survival or freedom from reintervention between heterografts and homografts.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1053/j.pcsu.2012.01.007DOI Listing
August 2012

Noninvasive assessment of liver fibrosis in adult patients following the Fontan procedure.

Congenit Heart Dis 2012 May-Jun;7(3):235-42. Epub 2012 Feb 23.

Departments of Pediatrics, Division of Pediatric Cardiology Radiology Internal Medicine, Division of Cardiovascular Medicine Cardiothoracic Surgery, Medical College of Wisconsin, Milwaukee, WI 53226, USA.

Objective:   Recent data indicate that patients after the Fontan procedure are at risk for significant liver dysfunction; however, the prevalence and extent of liver disease in the Fontan population remains unknown. Furthermore, limited data exist in regard to screening for liver disease in adult Fontan patients. We sought to determine the prevalence of liver disease in adult patients following the Fontan procedure using computed tomography (CT) and serum biomarkers of liver fibrosis.

Design:   Adult Fontan patients underwent screening for liver disease as part of their annual evaluation. Screening consisted of laboratory evaluation and dual-phase liver CT scan. Laboratory evaluation included analysis of liver function, viral hepatitis serologies, and FibroSURE panel (LabCorp), a test that analyzes the results of serum biomarkers to provide a quantitative surrogate marker for liver fibrosis.

Results:   Sixteen patients, mean age 30.3 (range 20-41) years, were enrolled in the study. Mean length of follow-up from time of Fontan palliation was 20.5 (range 11-33) years. No patients had serologic evidence of viral hepatitis or synthetic liver dysfunction. Twelve patients (75%) had abnormal FibroSURE scores, seven (44%) had elevated FibroSURE scores predictive of Metavir fibrosis stage F2 or greater on liver biopsy, and one (6%) had a FibroSURE score predictive of cirrhosis on biopsy. All 16 patients had abnormal radiologic liver findings identified on CT, including heterogeneous enhancement in 11 (69%), varices in six (38%), and liver nodules in five patients (31%). Length of time since Fontan surgery correlated significantly with an elevated FibroSURE score (P = .05) and having more CT scan abnormalities (P = .04).

Conclusions:   Liver fibrosis detected by serum biomarkers and dual phase CT scan is common in adult patients following the Fontan procedure. Further studies are needed to determine the long-term clinical significance of these findings.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/j.1747-0803.2012.00632.xDOI Listing
September 2012