Publications by authors named "Chet R Villa"

38 Publications

Cardiac medication management in Duchenne muscular dystrophy.

Pediatr Pulmonol 2021 Apr 1;56(4):747-752. Epub 2021 Mar 1.

The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.

There have been significant improvements in the skeletal muscle and respiratory care for patients with Duchenne muscular dystrophy (DMD) over the last two decades. This has resulted in longer expected survival as many patients will live into their 20s and 30s. This timeline has resulted in a greater proportion of patients experiencing heart failure and cardiac-related mortality. Herein, we describe the current indications for medical therapy for patients with DMD.
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http://dx.doi.org/10.1002/ppul.25175DOI Listing
April 2021

ABCs of Stroke Prevention: Improving Stroke Outcomes in Children Supported With a Ventricular Assist Device in a Quality Improvement Network.

Circ Cardiovasc Qual Outcomes 2020 12 15;13(12):e006663. Epub 2020 Dec 15.

Department of Cardiology, Boston Children's Hospital, Harvard School of Medicine, MA (C.J.V.).

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http://dx.doi.org/10.1161/CIRCOUTCOMES.120.006663DOI Listing
December 2020

Commentary: Rejuvenation of a trusted tool.

J Thorac Cardiovasc Surg 2021 04 25;161(4):1466-1467. Epub 2020 May 25.

Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. Electronic address:

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http://dx.doi.org/10.1016/j.jtcvs.2020.05.039DOI Listing
April 2021

Ventricular Assist Device Therapy and Fontan: A Story of Supply and Demand.

Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2020 ;23:62-68

The Heart Institute, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, Ohio. Electronic address:

The last 10 years have seen an increase in the number of Fontan patients with heart failure. There has been a coincident rapid evolution in the field of pediatric and congenital heart disease ventricular assist device therapy. Herein, we describe the existing body of literature regarding the use of ventricular assist device therapy in the Fontan circulation as well as the current approach to clinical decision-making and device implantation within the field.
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http://dx.doi.org/10.1053/j.pcsu.2020.02.007DOI Listing
March 2021

The total artificial heart in pediatrics: outcomes in an evolving field.

Ann Cardiothorac Surg 2020 Mar;9(2):104-109

The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.

Background: The use of the SynCardia temporary total artificial heart (TAH-t) in adults has increased with time. The development of the smaller, 50 cc TAH-t has expanded the potential applications of the device in children. We sought to describe the evolving use of the TAH-t over time and describe outcomes in the current era.

Methods: The SynCardia database was queried to identify all pediatric patients ≤18 years of age implanted with the device between December 1985 and October 2019. Patient demographics, clinical outcome and support characteristics collected.

Results: Fifty-one children were supported, 36 with the 70 cc TAH-t and 15 with the 50 cc TAH-t with a total support time of 6,243 days. The number of implants has increased with time (19 between 2015 and 2019). A total of 13 patients have been converted to Freedom Driver support, seven 50 cc TAH-t and six 70 cc TAH-t. The majority of implants in the last 5 years (15/19, 79%) have been with the 50 cc TAH-t. The most common diagnosis was dilated cardiomyopathy [24 (47%)] and the average age at the time of implant was 16±2 years old. Overall survival for the patient cohort was 71%.

Conclusions: The use of the SynCardia TAH-t to support children with end-stage heart failure has increased over time. Clinical outcomes with both the 50 cc and 70 cc TAH-t are similar to reported outcomes in adults who require TAH-t or other methods of biventricular support.
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http://dx.doi.org/10.21037/acs.2020.02.15DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7160627PMC
March 2020

The Creation of a Pediatric Health Care Learning Network: The ACTION Quality Improvement Collaborative.

ASAIO J 2020 04;66(4):441-446

Division of Cardiology, Stanford University School of Medicine, Palo Alto, California.

Improving the outcomes of pediatric patients with congenital heart disease with end-stage heart failure depends on the collaboration of all stakeholders; this includes providers, patients and families, and industry representatives. Because of the rarity of this condition and the heterogeneity of heart failure etiologies that occur at pediatric centers, learnings must be shared between institutions and all disciplines to move the field forward. To foster collaboration, excel discovery, and bring data to the bedside, a new, collaborative quality improvement science network-ACTION (Advanced Cardiac Therapies Improving Outcomes Network)-was developed to meet the needs of the field. Existing gaps in care and the methods of improvement that will be used are described, along with the mission and vision, utility of real-world data for regulatory purposes, and the organizational structure of ACTION is described.
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http://dx.doi.org/10.1097/MAT.0000000000001133DOI Listing
April 2020

Implantable Cardioverter Defibrillator Use in Males with Duchenne Muscular Dystrophy and Severe Left Ventricular Dysfunction.

Pediatr Cardiol 2020 Jun 10;41(5):925-931. Epub 2020 Mar 10.

The Children's Hospital of Philadelphia, Philadelphia, PA, USA.

Duchenne muscular dystrophy (DMD) is characterized by myocardial fibrosis and left ventricular (LV) dysfunction. Implantable cardioverter defibrillator (ICD) use has not been characterized in this population but is considered for symptomatic patients with severe LV dysfunction (SLVD) receiving guideline-directed medical therapy (GDMT). We evaluated ICD utilization and efficacy in patients with DMD. Retrospective cohort study of DMD patients from 17 centers across North America between January 2, 2005 and December 31, 2015. ICD use and its effect on survival were evaluated in patients with SLVD defined as ejection fraction (EF) < 35% and/ or shortening fraction (SF) < 16% on final echocardiogram. SLVD was present in 57/436 (13.1%) patients, of which 12 (21.1%) died during the study period. Of these 12, (mean EF 20.9 ± 6.2% and SF 13.7 ± 7.2%), 8 received GDMT, 5 received steroids, and none received an ICD. ICDs were placed in 9/57 (15.8%) patients with SLVD (mean EF 31.2 ± 8.5% and SF 10.3 ± 4.9%) at a mean age of 20.4 ± 6.3 years; 8/9 received GDMT, 7 received steroids, and all were alive at study end; mean ICD duration was 36.1 ± 26.2 months. Nine ICDs were implanted at six different institutions, associated with two appropriate shocks for ventricular tachycardia in two patients, no inappropriate shocks, and one lead fracture. ICD use may be associated with improved survival and minimal complications in DMD cardiomyopathy with SLVD. However, inconsistent GDMT utilization may be a significant confounder. Future studies should define optimal indications for ICD implantation in patients with DMD cardiomyopathy.
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http://dx.doi.org/10.1007/s00246-020-02336-9DOI Listing
June 2020

Risk Factors for Cardiac and Non-cardiac Causes of Death in Males with Duchenne Muscular Dystrophy.

Pediatr Cardiol 2020 Apr 3;41(4):764-771. Epub 2020 Feb 3.

Mayo Clinic Children's Center, Rochester, MN, USA.

As survival and neuromuscular function in Duchenne muscular dystrophy (DMD) have improved with glucocorticoid (GC) therapy and ventilatory support, cardiac deaths are increasing. Little is known about risk factors for cardiac and non-cardiac causes of death in DMD. A multi-center retrospective cohort study of 408 males with DMD, followed from January 1, 2005 to December 31, 2015, was conducted to identify risk factors for death. Those dying of cardiac causes were compared to those dying of non-cardiac causes and to those alive at study end. There were 29 (7.1%) deaths at a median age of 19.5 (IQR: 16.9-24.6) years; 8 (27.6%) cardiac, and 21 non-cardiac. Those living were younger [14.9 (IQR: 11.0-19.1) years] than those dying of cardiac [18 (IQR 15.5-24) years, p = 0.03] and non-cardiac [19 (IQR: 16.5-23) years, p = 0.002] causes. GC use was lower for those dying of cardiac causes compared to those living [2/8 (25%) vs. 304/378 (80.4%), p = 0.001]. Last ejection fraction prior to death/study end was lower for those dying of cardiac causes compared to those living (37.5% ± 12.8 vs. 54.5% ± 10.8, p = 0.01) but not compared to those dying of non-cardiac causes (37.5% ± 12.8 vs. 41.2% ± 19.3, p = 0.58). In a large DMD cohort, approximately 30% of deaths were cardiac. Lack of GC use was associated with cardiac causes of death, while systolic dysfunction was associated with death from any cause. Further work is needed to ensure guideline adherence and to define optimal management of systolic dysfunction in males with DMD with hopes of extending survival.
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http://dx.doi.org/10.1007/s00246-020-02309-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7328368PMC
April 2020

Commentary: The tortoise and the hare: Does speed matter in pediatric VAD therapy?

J Thorac Cardiovasc Surg 2020 04 27;159(4):1528-1529. Epub 2019 Nov 27.

Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. Electronic address:

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http://dx.doi.org/10.1016/j.jtcvs.2019.10.207DOI Listing
April 2020

Investigation of de novo variation in pediatric cardiomyopathy.

Am J Med Genet C Semin Med Genet 2020 03 8;184(1):116-123. Epub 2020 Jan 8.

The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.

Pediatric cardiomyopathies can be caused by variants in genes encoding the sarcomere and cytoskeleton in cardiomyocytes. Variants are typically inherited in an autosomal dominant manner with variable expressivity. De novo variants have been reported, however their overall frequency is largely unknown. We sought to determine the rate of de novo, pathogenic and likely pathogenic (P/LP) variants in children with a diagnosis of hypertrophic, dilated, or restrictive cardiomyopathy (HCM, DCM, or RCM), and to compare disease outcomes between individuals with and without a de novo variant. A retrospective record review identified 126 individuals with HCM (55%), DCM (37%), or RCM (8%) ≤18 years of age who had genetic testing. Overall, 50 (40%) had positive genetic testing and 18% of P/LP variants occurred de novo. The rate of de novo variation in those with RCM (80%) was higher than in those with HCM (9%) or DCM (20%). There was evidence of germline mosaicism in one family with RCM. Individuals with de novo variants were more likely than those without to have a history of arrhythmia (p = .049), sudden cardiac arrest (p = .024), hospitalization (p = .041), and cardiac transplantation (p = .030). The likelihood of de novo variation and impact on family risk and screening should be integrated into genetic counseling.
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http://dx.doi.org/10.1002/ajmg.c.31764DOI Listing
March 2020

Optimizing Postcardiac Transplantation Outcomes in Children with Ventricular Assist Devices: How Long Should the Bridge Be?

ASAIO J 2020 07;66(7):787-795

From the Department of Cardiothoracic Surgery, Heart Institute, Cincinnati Children's Hospital, Cincinnati, Ohio.

Ventricular assist devices (VADs) decrease waitlist mortality and improve end-organ function. Therefore, we sought to determine the duration of VAD support that could allow for optimal posttransplant outcomes. Pediatric transplant recipients were identified from the United Network for Organ Sharing database. Inflection points were determined using hazard of mortality associated with support time fitted by cubic spline method. Of 685 patients, those supported for >2 months had a significant decrease in inotrope use and mechanical ventilation and an increase in functional status compared with those supported for <2 months (all p < 0.001). Those supported for 2-4 months experienced better posttransplant survival than <2 months (p = 0.031). In durable pulsatile devices, similar improvement was seen in mechanical ventilation and functional status for the 2-4 month group with superior survival compared with <2 months (p = 0.008) and >4 months (p = 0.012). In continuous flow devices, used in patients overall less ill, the inflection point was >3 weeks with improvement in most end-organ function (p < 0.001) and posttransplant survival (p = 0.014) compared with <3 weeks. In general, a period of VAD support is associated with improvement in pretransplant risk factors and better posttransplant survival. This suggests that most patients bridged to transplantation by VADs should be supported for some time before listing to optimize posttransplant outcomes.
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http://dx.doi.org/10.1097/MAT.0000000000001075DOI Listing
July 2020

Use of advanced heart failure therapies in Duchenne muscular dystrophy.

Prog Pediatr Cardiol 2019 Jun 11;53:11-14. Epub 2019 Jan 11.

Arkansas Children's Hospital, University of Arkansas for Medical Sciences, Little Rock, AR, United States of America.

Background: As survival and neuromuscular function in Duchenne Muscular Dystrophy (DMD) improve with glucocorticoid therapy and respiratory advances, the proportion of cardiac deaths is increasing. Little is known about the use and outcomes of advanced heart failure (HF) therapies in this population.

Methods: A retrospective cohort study of 436 males with DMD was performed, from January 1, 2005-January 1, 2018, with the primary outcome being use of advanced HF therapies including: implantable cardioverter defibrillator (ICD), left ventricular assist device (LVAD), and heart transplantation (HTX).

Results: Nine subjects had an ICD placed, 2 of whom (22.2%) had appropriate shocks for ventricular tachycardia; 1 and 968 days after implant, and all of whom were alive at last follow-up; median 18 (IQR: 12.5-25.5) months from implant. Four subjects had a LVAD implanted with post-LVAD survival of 75% at 1 year; 2 remaining on support and 1 undergoing HTX. One subject was bridged to HTX with ICD and LVAD and was alive at last follow-up, 53 months after HTX.

Conclusion: Advanced HF therapies may be used effectively in select subjects with DMD. Further studies are needed to better understand risk stratification for ICD use and optimal candidacy for LVAD implantation and HTX, with hopes of improving cardiac outcomes.
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http://dx.doi.org/10.1016/j.ppedcard.2019.01.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6663084PMC
June 2019

How small can you go? A 2.5-kg infant with pulmonary atresia and coronary atresia bridged to cardiac transplantation with a paracorporeal-continuous flow ventricular assist device.

J Thorac Cardiovasc Surg 2019 08 5;158(2):e67-e69. Epub 2018 Oct 5.

The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.

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http://dx.doi.org/10.1016/j.jtcvs.2018.09.027DOI Listing
August 2019

If I only had a heart: The trials and tribulations of ventricular assist device support when missing a ventricle.

J Thorac Cardiovasc Surg 2018 08 13;156(2):746-747. Epub 2018 Apr 13.

The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. Electronic address:

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http://dx.doi.org/10.1016/j.jtcvs.2018.04.013DOI Listing
August 2018

Transplant Outcomes for Congenital Heart Disease Patients Bridged With a Ventricular Assist Device.

Ann Thorac Surg 2018 08 30;106(2):588-594. Epub 2018 Apr 30.

Division of Cardiothoracic Surgery, The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.

Background: Ventricular assist device (VAD) use as a bridge to transplant (BTT) for children with end-stage heart failure and congenital heart disease (CHD), although challenging, has increased, but its effect on posttransplant outcome is unknown. This study describes posttransplant outcomes of CHD patients BTT with a VAD.

Methods: All heart transplant recipients identified in United Network of Organ Sharing database from 2006 to 2015 (n = 21,865) were divided into four groups by those with (+) and without (-) a diagnosis of CHD and with (+) and without (-) VAD support at transplant: +CHD/+VAD, +CHD/-VAD, +VAD/-CHD, and -VAD/-CHD. Posttransplant survival of +CHD/+VAD was compared with +CHD/-VAD, -CHD/+VAD, and -CHD/-VAD in addition to pretransplant characteristics comparison between +CHD/+VAD and +CHD/-VAD.

Results: Of 1,871 patients (8.6%) with CHD, 1,348 (72%) were younger than 18 years old, and 143 (7.6%) were BTT with a VAD (+CHD/+VAD). At transplant, +CHD/+VAD compared with +CHD/-VAD were more likely to have worse functional status (<50%: 60% vs 46%, p = 0.004), infections (29% vs 14%, p < 0.001), to be sensitized (47% vs 30%, p < 0.001) and on ventilator support (20% vs 13%, p = 0.029) and dialysis (13% vs 2.5%, p < 0.001). Overall, 1-year (84% vs 87%) and 5-year (72% vs 75%) survival was similar for +CHD/+VAD and +CHD/-VAD (p = 0.694). Survival was also similar when +CHD/+VAD were compared with -CHD/+VAD (n = 7,363; p = 0.529) and -CHD/-VAD (n = 12,613; p = 0.097).

Conclusions: Although more ill pretransplant, CHD patients BTT with a VAD have similar posttransplant survival compared with CHD patients without a VAD and with other non-CHD heart transplant patients. VAD support may mitigate certain risk factors for poor posttransplant outcomes in the challenging CHD cohort.
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http://dx.doi.org/10.1016/j.athoracsur.2018.03.060DOI Listing
August 2018

The Right Tool for the Right Job: Bridging a Failing Fontan to Transplant.

Ann Thorac Surg 2018 09 16;106(3):e145-e146. Epub 2018 Apr 16.

Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. Electronic address:

Mechanical circulatory support and heart transplantation are both becoming increasingly common among Fontan patients as this growing cohort of patients is reaching young adulthood. Although device placement may present challenges, the new focus is on selecting the correct cannulation sites and devices at the correct time to successfully bridge these challenging patients to heart transplant. Herein, we report the use of a number of mechanical circulatory support strategies tailored to the clinical setting that culminated in 130 days of support before cardiac transplantation of a young adult with a failing Fontan circulation.
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http://dx.doi.org/10.1016/j.athoracsur.2018.03.029DOI Listing
September 2018

Heart failure after the Norwood procedure: An analysis of the Single Ventricle Reconstruction Trial.

J Heart Lung Transplant 2018 07 16;37(7):879-885. Epub 2018 Feb 16.

Boston Children's Hospital and Department of Pediatrics Cardiology Harvard School of Medicine, Boston, MA (K.PD., J.W.N.).

Background: Heart failure results in significant morbidity and mortality in young children with hypoplastic left heart syndrome (HLHS) after the Norwood procedure.

Methods: We studied subjects enrolled in the prospective Single Ventricle Reconstruction (SVR) Trial who survived to hospital discharge after a Norwood operation and were followed up to age 6 years. The primary outcome was heart failure, defined as heart transplant listing after Norwood hospitalization, death attributable to heart failure, or symptomatic heart failure (New York Heart Association [NYHA] Class IV). Multivariate modeling was undertaken using Cox regression methodology to determine variables associated with heart failure.

Results: Of the 461 subjects discharged home following a Norwood procedure, 66 (14.3%) met the criteria for heart failure. Among these, 15 died from heart failure, 39 were listed for transplant (22 had a transplant, 12 died after listing, and 5 were alive and not yet transplanted), and 12 had NYHA Class IV heart failure but were never listed. The median age at heart failure identification was 1.28 (interquartile range 0.30 to 4.69) years. Factors associated with early heart failure included post-Norwood lower fractional area change, need for extracorporeal membrane oxygenation, non-Hispanic ethnicity, Norwood perfusion type, and total support time (p < 0.05).

Conclusions: By 6 years of age, heart failure developed in nearly 15% of children after the Norwood procedure. Although transplant listing was common, many patients died from heart failure before receiving a transplant or without being listed. Shunt type did not impact the risk of developing heart failure.
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http://dx.doi.org/10.1016/j.healun.2018.02.009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6015519PMC
July 2018

Is there an optimal organ acceptance rate for pediatric heart transplantation: "A sweet spot"?

Pediatr Transplant 2018 05 30;22(3):e13149. Epub 2018 Jan 30.

Department of Cardiothoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.

Despite a limited supply of donors, potential donor hearts are often declined for subjective concerns regarding organ quality. This analysis will investigate the relationship between donor heart AR and patient outcome at pediatric transplant centers. The UNOS database was used to identify all match runs for pediatric candidates (age < 18 years) from 2008 through March 2015 in which a heart offer was ultimately placed. Centers which received ≥10 offers/y were included (10 634 offers, 38 centers). Transplant centers were stratified based on their AR: low (<20%, n = 13), medium (20%-40%, n = 16), or high (>40%, n = 9). Low AR centers experienced worse negative WL outcome compared with medium (P = .022) and high (P = .004) AR centers. Low AR centers had similar post-transplant graft survival to medium (P = .311) or high (P = .393) AR centers; however, medium AR centers had better post-transplant graft survival than high AR centers (P = .037). E-F survival from listing regardless of transplant was worse for low AR centers compared with medium (P < .001) or high (P = .001) AR centers. Low AR centers experience worse WL outcomes without improvement in post-transplant outcomes. High AR centers experience higher post-transplant graft failure than medium AR centers. AR of 20%-40% appears to have optimal WL and post-transplant outcomes.
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http://dx.doi.org/10.1111/petr.13149DOI Listing
May 2018

Obesity class does not further stratify outcome in overweight and obese pediatric patients after heart transplantation.

Pediatr Transplant 2018 03 29;22(2). Epub 2018 Jan 29.

Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.

The effect of obesity stratification on pediatric heart transplant outcomes is unknown. The UNOS database was queried for patients ≥2-<18 years listed for heart transplant and stratified by BMI: normal (BMI>5%-≤85 percentile), overweight (BMI=86%-95 percentile), class 1 (BMI=100%-120% of 95 percentile), class 2 (BMI=121%-140% of 95 percentile), and class 3 obesity (BMI>140% of 95 percentile). A total of 5056 individuals were listed for transplant, with 71% normal, 13% overweight, 10% class 1, 4% class 2, and 2% class 3 obesity. Waitlist survival was not different between groups. Post-transplant survival was decreased in overweight and combined obese groups vs normal, with no further difference between overweight and obese classes. Overweight and obese patients had higher listing status and were more likely to have ventilator, inotrope, and mechanical circulatory support at listing. After transplant, there was an association of overweight-obese patients with diabetes and rejection requiring hospitalization. Stricter definition of normal weight reveals overweight-obese status was an independent risk factor for poorer post-transplant survival, without further effect by stratification of weight class. However, because there is no difference in waitlist survival, this study does not allow the selection of absolute weight-based criteria regarding transplant listing and suggests the need to look further for modifiable risk factors post-transplant.
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http://dx.doi.org/10.1111/petr.13161DOI Listing
March 2018

Inferior Transplant Outcomes of Adolescents and Young Adults Bridged with a Ventricular Assist Device.

ASAIO J 2018 May/Jun;64(3):295-300

From the Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.

Adolescents, who are thought to have compliance issues, are well known to have poor heart transplant (HTx) outcomes. This "effect" has recently been demonstrated to extend to age 29. The study sought to investigate whether the poor outcomes for HTx related to adolescent age are also observed in recipients who are bridged to transplant (BTT) with a ventricular assist device (VAD) and whether this effect extends beyond the standard definition of adolescent age 12-18 years. All HTx BTT with a VAD in recipients 8-39 years were identified in the United States Organ Sharing (UNOS) database (1 January 2005 to 30 June 2016). Based on the Kaplan-Meier survival comparison for age year, patients were divided into three groups: Group 1 (8-14 years), group 2 (15-29 years), and group 3 (30-39 years). A total of 1,848 HTx were bridged with a VAD. A decline in post-HTx 5 years survival was noted after 14 years of age, which improved at around 30 years of age. Group 1 had 237 (13%) HTx, group 2 had 787 (43%) HTx, and group 3 had 823 (44%) HTx. Group 2 (15-29 years) had worse post-HTx survival compared with group 1 (p < 0.001) and group 3 (p = 0.005). On subdividing group 2 (15-29 years) into "older adolescents" (15-17 years) and "young adults" (18-29 years), post-HTx survival was similar between the two subgroups (p = 0.353). In conclusion, older adolescents and young adults, both, have similarly poor post-HTx survival when BTT with a VAD compared with other age groups. These groups are generally categorized into different broad pediatric and adult age groups; however, these similarities should be carefully considered when formulating treatment protocols for older adolescents and young adults.
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http://dx.doi.org/10.1097/MAT.0000000000000685DOI Listing
March 2019

Ventricular assist device use in single ventricle congenital heart disease.

Pediatr Transplant 2017 Nov 15;21(7). Epub 2017 Sep 15.

The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.

As VAD have become an effective therapy for end-stage heart failure, their application in congenital heart disease has increased. Single ventricle congenital heart disease introduces unique physiologic challenges for VAD use. However, with regard to the mixed clinical results presented within this review, we suggest that patient selection, timing of implant, and center experience are all important contributors to outcome. This review focuses on the published experience of VAD use in single ventricle patients and details physiologic challenges and novel approaches in this growing pediatric and adult population.
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http://dx.doi.org/10.1111/petr.13031DOI Listing
November 2017

Elevated Myocardial Extracellular Volume Fraction in Duchenne Muscular Dystrophy.

Pediatr Cardiol 2017 Oct 31;38(7):1485-1492. Epub 2017 Jul 31.

The Heart Institute, Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave, Cincinnati, OH, 45229, USA.

Duchenne muscular dystrophy (DMD) is a genetic, X-linked recessive disease with an associated cardiomyopathy characterized by myocardial fibrosis leading to heart failure, arrhythmias, and death. Earlier detection and treatment of cardiac involvement in DMD hold potential to improve outcomes. Cardiovascular magnetic resonance (CMR) extracellular volume (ECV) quantification using T1 mapping is a histologically validated, non-invasive marker of diffuse fibrosis. This study aims to determine the ECV in a pediatric DMD population, and correlate it with metrics of left ventricular function. A retrospective review of pediatric DMD subjects who underwent CMR at a single institution. A total of 47 DMD patients (mean age 14 ± 2 years) were included for analysis. Global myocardial ECV was significantly higher in the DMD group (29 ± 6%) compared with published normal values (24 ± 2%, p = 0.0001). Higher ECV values correlate with indices of left ventricular function, including decreased left ventricular ejection fraction (r = -0.46, p = 0.001) and indexed left ventricular end diastolic volume (r = 0.41, p = 0.004). ECV was not significantly higher in DMD patients with late gadolinium enhancement (LGE) (30 ± 7%) compared to DMD patients without LGE (27 ± 5%, p = 0.0717). CMR T1 mapping is a feasible method for quantification of ECV in patients with DMD. Global myocardial ECV is significantly higher in the DMD population compared to healthy controls and correlates with other metrics of myocardial function. Global myocardial ECV may serve as an important tool to determine cardiac involvement in DMD population and help guide medical management.
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http://dx.doi.org/10.1007/s00246-017-1690-xDOI Listing
October 2017

Pediatric continuous-flow left ventricular assist devices: No longer just a bridge? The changing of a mindset!

J Thorac Cardiovasc Surg 2017 10 17;154(4):1362-1363. Epub 2017 May 17.

The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. Electronic address:

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http://dx.doi.org/10.1016/j.jtcvs.2017.05.014DOI Listing
October 2017

The Total Artificial Heart in End-Stage Congenital Heart Disease.

Front Physiol 2017 9;8:131. Epub 2017 May 9.

Cincinnati Children's Hospital Medical Center, Heart InstituteCincinnati, OH, USA.

The development of durable ventricular assist devices (VADs) has improved mortality rates and quality of life in patients with end stage heart failure. While the use of VADs has increased dramatically in recent years, there is limited experience with VAD implantation in patients with complex congenital heart disease (CHD), despite the fact that the number of patients with end stage CHD has grown due to improvements in surgical and medical care. VAD use has been limited in patients with CHD and end stage heart failure due to anatomic (systemic right ventricle, single ventricle, surgically altered anatomy, valve dysfunction, etc.) and physiologic constraints (diastolic dysfunction). The total artificial heart (TAH), which has right and left sided pumps that can be arranged in a variety of orientations, can accommodate the anatomic variation present in CHD patients. This review provides an overview of the potential use of the TAH in patients with CHD.
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http://dx.doi.org/10.3389/fphys.2017.00131DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5422510PMC
May 2017

United States Trends in Pediatric Ventricular Assist Implantation as Bridge to Transplantation.

ASAIO J 2017 Jul/Aug;63(4):470-475

From the *The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH; and †Department of Family Medicine, University of Oklahoma, Tulsa, OK.

Ventricular assist devices (VADs) are increasingly used to support children to heart transplant, however, it is unclear whether this trend is broadly applicable across the age/size spectrum. Children (≤18 years) listed for transplant were identified from the United Network of Organ Sharing (UNOS) database between January 2006 and May 2014. Patients were stratified by era (Early: 2006-2010, Current: 2011-2014), size (<25, 25-50, and >50 kg), and device type. Of 3,986 patients, 520 (13%) were supported by a VAD. The proportion of patients supported with a VAD has increased in the current era (16% vs. 11%, p < 0.001). The increase has occurred in children <25 kg (11% vs. 7%, p < 0.001) and >50 kg (26% vs. 17%, p < 0.002). Although VAD utilization was similar across eras in patients 25-50 kg (14% vs. 13%, p = 0.6), continuous flow VAD (cfVAD) use increased dramatically from 7% to 52% (p < 0.001). Waitlist outcome (p = 0.6) and posttransplant survival (p = 0.53) were similar between eras despite the shift to cfVADs in patients ≥25 kg. The emergence of cfVAD technology in an era of increasing waitlist times raises the prospect of an increasing population of children with cfVADs at home and in our communities.
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http://dx.doi.org/10.1097/MAT.0000000000000524DOI Listing
February 2018

Strategies to Prevent Cast Formation in Patients with Plastic Bronchitis Undergoing Heart Transplantation.

Pediatr Cardiol 2017 Jun 19;38(5):1077-1079. Epub 2017 Jan 19.

The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, USA.

Plastic bronchitis, a rare complication after Fontan palliation, carries a high morbidity and mortality risk. Heart transplantation is an effective treatment option, but casts may occur in the early post-operative period. We present a case series detailing peri-operative management strategies to minimize morbidity and mortality related to plastic bronchitis in patients undergoing heart transplantation. Patient 1 received no treatment pre-, intra-, or post-transplant for prevention of bronchial casts and developed severe respiratory acidosis 18 h following transplant. Emergent bronchoscopy was performed and a large obstructive cast was removed. The patient recovered and received inhaled tissue plasminogen activator (tPA) for 5 days. Patient 2 received inhaled tPA before, during, and for 5 days after transplantation and no bronchial casts developed. Patient 3 underwent intraoperative bronchoscopy just prior to implantation revealing no casts. The patient underwent non-urgent, preemptive bronchoscopy on post-transplant days 1, 3, and 4, removing several partially obstructive bronchial blood clots/casts, with no casts thereafter. Heart transplantation results in eventual resolution of plastic bronchitis. Residual bronchial casts can still be problematic in the peri-operative period. Airway clearance with inhaled tPA or bronchoscopy may prevent the need for prolonged mechanical ventilation and reduce post-operative morbidity in this unique population.
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http://dx.doi.org/10.1007/s00246-017-1568-yDOI Listing
June 2017

Worldwide Experience with the Syncardia Total Artificial Heart in the Pediatric Population.

ASAIO J 2017 Jul/Aug;63(4):518-519

From the *The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio; and †Cedars-Sinai Heart Institute, Los Angeles, California.

Individual centers have documented the use of the Syncardia Total Artificial Heart (TAH) in adolescents with heart failure; however, the number of patients at any given center is small. Herein, we describe the worldwide experience for all patients ≤21 years old supported with the TAH between May 2005 and May 2015 (n = 43). The number of patients experiencing a positive outcome at 60, 90, and 120 days were 30 (70%), 27 (63%), and 25 (58%), respectively. Successful bridge to transplantation varied by diagnosis, but outcomes reported are similar to adults supported with the TAH or biventricular assist devices.
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http://dx.doi.org/10.1097/MAT.0000000000000504DOI Listing
February 2018

The 50/50 cc Total Artificial Heart Trial: Extending the Benefits of the Total Artificial Heart to Underserved Populations.

Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2017 Jan;20:16-19

Department of Pediatric Cardiothoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH. Electronic address:

While use of the total artificial heart (TAH) is growing, the use of the device is not uniform across the gender and age spectrum because the vast majority of implants are in adult males. SynCardia has recently developed a smaller 50 cc TAH that was designed to accommodate patients with a body surface area as low as 1.2 m (potentially even lower using virtual implantation). Herein, we describe the early use of the 50 cc TAH (10 implants in the US and 18 outside the US). Twenty-eight devices have been implanted worldwide. Nineteen (68%) patients were female, 4 (14%) were 21 years of age or younger, and 2 (7%) had a diagnosis of congenital heart disease (1 Fontan). The smallest patient, by body surface area, was 1.35 m. Six patients (21%) have been placed on the Freedom Driver, all of whom have survived. Fourteen patients (50%) have had a positive outcome to date. The development of the 50 cc TAH has expanded the population of patients who may benefit from TAH support and thus may help improve outcomes for patients who have had limited biventricular support options to date.
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http://dx.doi.org/10.1053/j.pcsu.2016.09.004DOI Listing
January 2017

Does Small Size Matter With Continuous Flow Devices?: Analysis of the INTERMACS Database of Adults With BSA ≤1.5 m.

JACC Heart Fail 2017 02 2;5(2):123-131. Epub 2016 Nov 2.

Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. Electronic address:

Objectives: This study investigated how small patient size affects clinical outcomes in patients implanted with a continuous flow left ventricular assist device (CFLVAD).

Background: The development of smaller CFLVADs has allowed ventricular assist device (VAD) use in anatomically smaller patients; however, limited outcome data exist regarding CFLVAD use in patients with a body surface area (BSA) ≤1.5 m.

Methods: All CFLVAD patients entered in the Interagency Registry for Mechanically Assisted Circulatory Support registry April 2008 to September 2013 and with BSA data were included. Biventricular VAD patients were excluded. Patient characteristics and clinical outcomes were compared between patients with BSA ≤1.5 m (small patients) and those >1.5 m.

Results: Of 10,813 CFLVAD recipients, 231 had a BSA ≤1.5 m. Small patients were more commonly female patients (68% vs. 20%; p < 0.01), Hispanic (10% vs. 6%; p < 0.03), and on intravenous inotropes (88% vs. 80%; p < 0.01). Small patients had higher bleeding (p < 0.01) and driveline infection (p < 0.01) rates, while exhibiting lower rates of right heart failure (p < 0.01) and renal dysfunction (p < 0.01). Device malfunction rate (p > 0.05), overall survival (p > 0.05), and 1-year competing outcomes (p > 0.05) were similar between BSA groups.

Conclusions: Patients with a BSA ≤1.5 m supported with a CFLVAD have similar survival to larger patients. These data support the use of CFLVAD in anatomically small patients.
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http://dx.doi.org/10.1016/j.jchf.2016.09.009DOI Listing
February 2017

Identifying evidence of cardio-renal syndrome in patients with Duchenne muscular dystrophy using cystatin C.

Neuromuscul Disord 2016 10 27;26(10):637-642. Epub 2016 Jul 27.

Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.

Patients with Duchenne muscular dystrophy (DMD) develop dilated cardiomyopathy and are at risk for kidney injury. Creatinine based estimated glomerular filtration rate (eGFR) is limited by low muscle mass with low serum creatinine levels in DMD. We assessed the relationship between cardiac function, modified Schwartz eGFR and cystatin C eGFR in patients with DMD. Ninety-three patients with DMD were screened for renal dysfunction in an outpatient neuromuscular clinic. Patients with new nephrotoxic medications, recent hospitalization or decompensated heart failure were excluded from the analysis. Eleven (12%) patients had evidence of renal dysfunction identified by cystatin C eGFR, while no patients had renal dysfunction by Schwartz eGFR. There was no significant correlation between cystatin C eGFR and age (r = -0.2, p = 0.11), prednisone dose (r = 0.06, p = 0.89) or deflazacort dose (r = -0.01, p = 0.63). There was a significant correlation between left ventricular ejection fraction and cystatin C GFR among patients with chronic left ventricular dysfunction (r = 0.46, p < 0.01), but not normal function (r = -0.07, p = 0.77). There was no significant correlation between left ventricular ejection fraction and Schwartz eGFR among patients with (r = 0.07, p = 0.59) or without (r = -0.27, p = 0.07) chronic left ventricular dysfunction. Cystatin C eGFR correlates with cardiac dysfunction in patients with DMD, thus providing novel evidence of cardio-renal syndrome in this population. Routine monitoring of renal function is recommended in patients with DMD.
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http://dx.doi.org/10.1016/j.nmd.2016.07.010DOI Listing
October 2016