Browsing by Author "Parent, John J."

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    Current Practices in Treating Cardiomyopathy and Heart Failure in Duchenne Muscular Dystrophy (DMD): Understanding Care Practices in Order to Optimize DMD Heart Failure Through ACTION
    (Springer Nature, 2022) Villa, Chet; Auerbach, Scott R.; Bansal, Neha; Birnbaum, Brian F.; Conway, Jennifer; Esteso, Paul; Gambetta, Katheryn; Hall, E. Kevin; Kaufman, Beth D.; Kirmani, Sonya; Lal, Ashwin K.; Martinez, Hugo R.; Nandi, Deipanjan; O’Connor, Matthew J.; Parent, John J.; Raucci, Frank J.; Shih, Renata; Shugh, Svetlana; Soslow, Jonathan H.; Tunuguntla, Hari; Wittlieb‑Weber, Carol A.; Kinnett, Kathi; Cripe, Linda; Pediatrics, School of Medicine
    Cardiac disease has emerged as a leading cause of mortality in Duchenne muscular dystrophy in the current era. This survey sought to identify the diagnostic and therapeutic approach to DMD among pediatric cardiologists in Advanced Cardiac Therapies Improving Outcomes Network. Pediatric cardiology providers within ACTION (a multi-center pediatric heart failure learning network) were surveyed regarding their approaches to cardiac care in DMD. Thirty-one providers from 23 centers responded. Cardiac MRI and Holter monitoring are routinely obtained, but the frequency of use and indications for ordering these tests varied widely. Angiotensin converting enzyme inhibitor and aldosterone antagonist are generally initiated prior to onset of systolic dysfunction, while the indications for initiating beta-blocker therapy vary more widely. Seventeen (55%) providers report their center has placed an implantable cardioverter defibrillator in at least 1 DMD patient, while 11 providers (35%) would not place an ICD for primary prevention in a DMD patient. Twenty-three providers (74%) would consider placement of a ventricular assist device (VAD) as destination therapy (n = 23, 74%) and three providers (10%) would consider a VAD only as bridge to transplant. Five providers (16%) would not consider VAD at their institution. Cardiac diagnostic and therapeutic approaches vary among ACTION centers, with notable variation present regarding the use of advanced therapies (ICD and VAD). The network is currently working to harmonize medical practices and optimize clinical care in an era of rapidly evolving outcomes and cardiac/skeletal muscle therapies.
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    Echocardiographic Guidance During Neonatal and Pediatric Jugular Cannulation for ECMO
    (Elsevier, 2018-12) Salazar, Paul A.; Blitzer, David; Dolejs, Scott C.; Parent, John J.; Gray, Brian W.; Surgery, School of Medicine
    Background Internal jugular vein extracorporeal membrane oxygenation (ECMO) cannula position is traditionally confirmed via plain film. Misplaced cannulae can result in need for repositioning and increased morbidity. Echocardiography (ECHO) may be used during cannulation as a more accurate means of guiding cannula position. This study reviews the effect of a protocol encouraging the use of ECHO at cannulation. Methods and materials Single institution retrospective review of patients who received ECMO support using jugular venous cannulation. We compared those who underwent ECHO (ECHO+) at the time of cannulation with those who did not (ECHO−). Results Eighty-nine patients were included: 26 ECHO+, 63 ECHO−. Most ECHO+ patients underwent dual-lumen veno-venous (VV) cannulation (65%); 32% of ECHO− patients had VV support (P = 0.003). There was no difference in the rate of cannula repositioning between the two groups: 8% ECHO+ and 10% ECHO−, P = 0.78. In the VV ECMO subgroup, ECHO+ patients required no repositioning (0/17), while 20% (4/20) of ECHO− VV patients did (P = 0.10). After cannulation, there were 0.58 ECHO studies per patient to verify cannula position in the ECHO+ group compared with 0.22 in the ECHO− group (P = 0.02). Each group had a major mechanical complication: atrial perforation from a guidewire during cannulation in ECHO+ and late atrial perforation from a loose cannula in ECHO−, and there was no difference in minor complications. Conclusions ECHO guidance during neonatal and pediatric jugular cannulation for ECMO did not decrease morbidity or reduce the need for cannula repositioning. ECHO may still be a useful adjunct for precise placement of a dual-lumen VV cannula and during difficult cannulations.
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    Impact of Genetic Testing for Cardiomyopathy on Emotional Well-Being and Family Dynamics: A Study of Parents and Adolescents
    (American Heart Association, 2021) Ahimaz, Priyanka; Sabatello, Maya; Qian, Min; Wang, Aijin; Miller, Erin M.; Parrott, Ashley; Lal, Ashwin K.; Chatfield, Kathryn C.; Rossano, Joseph W.; Ware, Stephanie M.; Parent, John J.; Kantor, Paul; Yue, Lisa; Wynn, Julia; Lee, Teresa M.; Addonizio, Linda J.; Appelbaum, Paul S.; Chung, Wendy K.; Pediatrics, School of Medicine
    Background: Genetic testing is indicated for children with a personal or family history of hereditary cardiomyopathy to determine appropriate management and inform risk stratification for family members. The implications of a positive genetic result for children can potentially impact emotional well-being. Given the nuances of cardiomyopathy genetic testing for minors, this study aimed to understand how parents involve their children in the testing process and investigate the impact of genetic results on family dynamics. Methods: A survey was distributed to participants recruited from the Children's Cardiomyopathy Foundation and 7 North American sites in the Pediatric Cardiomyopathy Registry. The survey explored adolescent and parent participants' emotions upon receiving their/their child's genetic results, parent-child result communication and its impact on family functionality, using the McMaster Family Assessment Device. Results: One hundred sixty-two parents of minors and 48 adolescents who were offered genetic testing for a personal or family history of cardiomyopathy completed the survey. Parents whose child had cardiomyopathy were more likely to disclose positive diagnostic genetic results to their child (P=0.014). Parents with unaffected children and positive predictive testing results were more likely to experience negative emotions about the result (P≤0.001) but also had better family functioning scores than those with negative predictive results (P=0.019). Most adolescents preferred results communicated directly to the child, but parents were divided about whether their child's result should first be released to them or their child. Conclusions: These findings have important considerations for how providers structure genetic services for adolescents and facilitate discussion between parents and their children about results.
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    Left ventricular noncompaction in a family with lamin A/C gene mutation
    (Texas Heart Institute Journal, 2015-02) Parent, John J.; Towbin, Jeffrey A.; Jefferies, John L.; Department of Pediatrics, IU School of Medicine
    Left ventricular noncompaction is a rare type of cardiomyopathy, the genetics of which are poorly understood to date. Lamin A/C gene mutations have been associated with dilated cardiomyopathy and diseases of the conduction system, but rarely in left ventricular noncompaction cardiomyopathy. This report describes the cases of 4 family members with a lamin A/C gene mutation, 3 of whom had phenotypic expression of left ventricular noncompaction.
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    Management of Complications Caused By a Massive Left Ventricle Tumor in a Neonate
    (Elsevier, 2018) Yabrodi, Mouhammad; Mastropietro, Christopher W.; Darragh, Robert K.; Parent, John J.; Ayres, Mark D.; Kean, Adam C.; Turrentine, Mark; Pediatrics, School of Medicine
    We report a case of a neonate born with a giant fibroma occupying the entirety of her left ventricle. Due to the extensive resection, her postoperative course was complicated by severely diminished left ventricular function and complete heart block necessitating extracorporeal support. Ultimately, cardiac resynchronization therapy was employed, after which the infant’s ventricular function gradually improved and she was successfully discharged to home.
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    Noncompaction cardiomyopathy and heterotaxy syndrome
    (Elsevier, 2017) Martinez, Hugo R.; Ware, Stephanie M.; Schamberger, Marcus S.; Parent, John J.; Department of Pediatrics, School of Medicine
    Left ventricular noncompaction cardiomyopathy (LVNC) is characterized by compact and trabecular layers of the left ventricular myocardium. This cardiomyopathy may occur with congenital heart disease (CHD). Single cases document co-occurrence of LVNC and heterotaxy, but no data exist regarding the prevalence of this association. This study sought to determine whether a non-random association of LVNC and heterotaxy exists by evaluating the prevalence of LVNC in patients with heterotaxy. In a retrospective review of the Indiana Network for Patient Care, we identified 172 patients with heterotaxy (69 male, 103 female). Echocardiography and cardiac magnetic resonance imaging results were independently reviewed by two cardiologists to ensure reproducibility of LVNC. A total of 13/172 (7.5%) patients met imaging criteria for LVNC. The CHD identified in this subgroup included atrioventricular septal defects [11], dextrocardia [10], systemic and pulmonary venous return abnormalities [7], and transposition of the great arteries [5]. From this subgroup, 61% (n = 8) of the patients developed arrhythmias; and 61% (n = 8) required medical management for chronic heart failure. This study indicates that LVNC has increased prevalence among patients with heterotaxy when compared to the general population (0.014–1.3%) suggesting possible common genetic mechanisms. Interestingly, mice with a loss of function of Scrib or Vangl2 genes showed abnormal compaction of the ventricles, anomalies in cardiac looping, and septation defects in previous studies. Recognition of the association between LVNC and heterotaxy is important for various reasons. First, the increased risk of arrhythmias demonstrated in our population. Secondly, theoretical risk of thromboembolic events remains in any LVNC population. Finally, many patients with heterotaxy undergo cardiac surgery (corrective and palliative) and when this is associated with LVNC, patients should be presumed to incur a higher peri-operative morbidity based on previous studies. Further research will continue to determine long-term and to corroborate genetic pathways.