IU Indianapolis ScholarWorks :: Browsing by Subject "congenital heart disease"

Browsing by Subject "congenital heart disease"

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HAND2 Mutation Detection in Tricuspid Atresia Patients
(Office of the Vice Chancellor for Research, 2013-04-05) Barry, Elijah H.; VanDusen, Nathan; Firulli, Anthony B.
Tricuspid Atresia (TA) is a congenital heart disease in which the tricuspid valve is missing or abnormally developed. The defect blocks blood in the right atrium from flowing directly into the right ventricle. It is an uncommon form of congenital heart disease that affects about 5 in every 100,000 live births. While the cause of TA is unknown, the lab data shows that in mice loss of transcription factor Hand2 function within a population of cells that line the inside of the heart (the endocardium) results in a TA phenotype. Hand2 is a protein that belongs to the basic helix-loop-helix family of transcription factors, and has been shown to play many different roles in embryonic development. To test whether loss of Hand2 function in humans results in TA, sequencing the HAND2 gene in 25 TA patients. Polymerase Chain Reaction (PCR) was used to amplify the TA patient Hand2 alleles. A TOPO reaction was then performed to insert the amplicons into a sequencing plasmid, followed by a transformation and minipreps to isolate individual clones. Isolated Hand2 alleles within the TOPO sequencing plasmid were sent to a sequencing core facility. In this manner the Hand2 DNA sequence for several patients was obtained and analyzed for mutations. This project will shed light on the cause of TA. Further research is currently in progress in Dr. Firulli’s Lab.
Hospital Costs Related to Early Extubation after Infant Cardiac Surgery
(Elsevier, 2018) McHugh, Kimberly E.; Mahle, William T.; Hall, Matthew A.; Scheurer, Mark A.; Moga, Michael-Alice; Triedman, John; Nicolson, Susan C.; Amula, Venugopal; Cooper, David S.; Schamberger, Marcus; Wolf, Michael; Shekerdemian, Lara; Burns, Kristin M.; Ash, Kathleen E.; Hipp, Dustin M.; Pasquali, Sara K.; Pediatrics, School of Medicine
Background The Pediatric Heart Network Collaborative Learning Study (PHN CLS) increased early extubation rates after infant Tetralogy of Fallot (TOF) and coarctation (CoA) repair across participating sites by implementing a clinical practice guideline (CPG). The impact of the CPG on hospital costs has not been studied. Methods PHN CLS clinical data were linked to cost data from Children’s Hospital Association by matching on indirect identifiers. Hospital costs were evaluated across active and control sites in the pre- and post-CPG periods using generalized linear mixed effects models. A difference-in-difference approach was used to assess whether changes in cost observed in active sites were beyond secular trends in control sites. Results Data were successfully linked on 410/428 (96%) of eligible patients from 4 active and 4 control sites. Mean adjusted cost/case for TOF repair was significantly reduced in the post-CPG period at active sites ($42,833 vs. $56,304, p<0.01) and unchanged at control sites ($47,007 vs. $46,476, p=0.91), with an overall cost reduction of 27% in active vs. control sites (p=0.03). Specific categories of cost reduced in the TOF cohort included clinical (-66%, p<0.01), pharmacy (-46%, p=0.04), lab (-44%, p<0.01), and imaging (-32%, p<0.01). There was no change in costs for CoA repair at active or control sites. Conclusions The early extubation CPG was associated with a reduction in hospital costs for infants undergoing repair of TOF, but not CoA repair. This CPG represents an opportunity to both optimize clinical outcome and reduce costs for certain infant cardiac surgeries.
Hypoplastic Left Heart Syndrome
(StatPearls, 2020) Kritzmire, Stacy M.; Cossu, Anne E.; Anesthesia, School of Medicine
Hypoplastic Left Heart Syndrome
(StatPearls Publishing, 2021) Kritzmire, Stacy M.; Cossu, Anne E.; Anesthesia, School of Medicine
Hypoplastic left heart syndrome (HLHS) is a type of congenital heart disease that results in the underdevelopment of the left-sided structures of the heart, including the mitral valve, left ventricle, aortic valve, ascending aorta, and aortic arch. HLHS was first described as a syndrome in 1958 by Nadas and NoonanFontanwho referred to it as combined aortic and mitral atresia.[1] HLHS affects 1 in 5,000 neonates or 3% of all infants born with congenital heart disease.[2][3] Thirty years ago, there were no treatment options for these neonates, and mortality was 100% within the first week of life.[4] Neonates born with HLHS are dependent on a patent ductus arteriosus and an interatrial communication for survival until surgical intervention. A continuous infusion of prostaglandin E1 (PGE1) is needed to maintain ductal patency. Today, several treatment options are available in the prenatal or neonatal period, including the Norwood procedure, hybrid stage 1, heart transplantation, palliative care, and fetal intervention. A series of three palliative surgical operations (Norwood/Hybrid, Hemi-Fontan/Bidirectional Glenn, and Fontan) are typically necessary for survival beyond the neonatal period and infancy. Though rare, a diagnosis of HLHS is responsible for 23% of all cardiac deaths in the first week of life.[1]
Isolated congenital cleft mitral valve leaflet: a rare cause of refractory cardiogenic shock complicating acute myocardial infarction
(BMC, 2021-06) Bahrami, Mubashir H.; Ansari, Hamza Z.; Guglin, Maya; Ephrem, Georges; Revtyak, George E.; Medicine, School of Medicine
We report a unique presentation of isolated congenital cleft mitral valve complicating cardiogenic shock from acute myocardial infarction. Isolated cleft mitral valve is an uncommon diagnosis that can have significant clinical implications, especially if not recognized in patients presenting to the catheterization lab with acute myocardial infarction and cardiogenic shock. A review of this rare diagnosis including the options and timing of therapeutic interventions, which can include MitraClip, is important for publication. The case is of a patient who presented with an anterior acute ST elevation myocardial infarction. Despite early coronary revascularization and conventional support, refractory cardiogenic shock ensued requiring mechanical circulatory support escalation to Veno-arterial extracorporeal membrane oxygenation. Subsequently, left ventriculography revealed a massively dilated left atrium and severe mitral regurgitation raising concerns for a mechanical mitral valve complication. The patient was taken to the operating room for possible mitral valve surgery, but a preoperative transesophageal echocardiogram revealed an isolated posterior cleft mitral valve. Since the patient had stabilized on mechanical circulatory support, emergent surgery was deferred. The patient successfully recovered during index hospitalization with mechanical circulatory support and discharged on guideline directed medical therapy. In conclusion, isolated cleft mitral valve is a rare diagnosis that can often be underrecognized without comprehensive 3-dimensional transesophageal echocardiography evaluation. If diagnosed early with significant regurgitation, surgical treatment results in good outcomes and preservation of LV systolic function. Percutaneous correction of a CMVL with MitraClip has been described and may offer an alternative approach for high risk surgical patients.
Learning to Crawl: Determining the Role of Genetic Abnormalities on Postoperative Outcomes in Congenital Heart Disease
(AHA, 2022-10) Landis, Benjamin J.; Helm, Benjamin M.; Herrmann, Jeremy L.; Hoover, Madeline C.; Durbin, Matthew D.; Elmore, Lindsey R.; Huang, Manyan; Johansen, Michael; Li, Ming; Przybylowski, Leon F.; Geddes, Gabrielle C.; Ware, Stephanie M.; Pediatrics, School of Medicine
Background Our cardiac center established a systematic approach for inpatient cardiovascular genetics evaluations of infants with congenital heart disease, including routine chromosomal microarray (CMA) testing. This provides a new opportunity to investigate correlation between genetic abnormalities and postoperative course. Methods and Results Infants who underwent congenital heart disease surgery as neonates (aged ≤28 days) from 2015 to 2020 were identified. Cases with trisomy 21 or 18 were excluded. Diagnostic genetic results or CMA with variant of uncertain significance were considered abnormal. We compared postoperative outcomes following initial congenital heart disease surgery in patients found to have genetic abnormality to those who had negative CMA. Among 355 eligible patients, genetics consultations or CMA were completed in 88%. A genetic abnormality was identified in 73 patients (21%), whereas 221 had negative CMA results. Genetic abnormality was associated with prematurity, extracardiac anomaly, and lower weight at surgery. Operative mortality rate was 9.6% in patients with a genetic abnormality versus 4.1% in patients without an identified genetic abnormality (P=0.080). Mortality was similar when genetic evaluations were diagnostic (9.3%) or identified a variant of uncertain significance on CMA (10.0%). Among 14 patients with 22q11.2 deletion, the 2 mortality cases had additional CMA findings. In patients without extracardiac anomaly, genetic abnormality was independently associated with increased mortality (P=0.019). CMA abnormality was not associated with postoperative length of hospitalization, extracorporeal membrane oxygenation, or >7 days to initial extubation. Conclusions Routine genetic evaluations and CMA may help to stratify mortality risk in severe congenital heart disease with syndromic or nonsyndromic presentations.
Multicenter Analysis of Early Childhood Outcomes Following Repair of Truncus Arteriosus
(Elsevier, 2018) Buckley, Jason R.; Amula, Venu; Sassalos, Peter; Costello, John M.; Smerling, Arthur J.; Iliopoulos, lias; Jennings, Aimee; Riley, Christine M.; Cashen, Katherine; Narasimhulu, Sukumar Suguna; Gowda, Keshava Murthy Narayana; Bakar, Adnan M.; Wilhelm, Michael; Badheka, Aditya; Moser, Elizabeth A. S.; Mastropietro, Christopher W.; Biostatistics, School of Public Health
Background Literature describing morbidity and mortality following truncus arteriosus repair is predominated by single-center reports. We created and analyzed a multicenter dataset to identify risk factors for late mortality and right ventricle-to-pulmonary artery (RV-PA) conduit reintervention for this patient population. Methods We retrospectively collected data on children who underwent repair of truncus arteriosus without concomitant arch obstruction at 15 centers between 2009 and 2016. Cox regression survival analysis was conducted to determine risk factors for late mortality, defined as death occurring after hospital discharge and greater than 30 days after surgery. Probability of any RV-PA conduit reintervention was analyzed over time using Fine-Gray modelling. Results We reviewed 216 patients, with median follow-up of 2.9 years (range:0.1-8.8). Operative mortaility occurred in 15 patients (7%). Of the 201 survivors, there were 14 (7%) late deaths. DiGeorge syndrome (HR:5.4; 95%CI:1.6-17.8) and need for postoperative tracheostomy (HR:5.9; 95%CI:1.8-19.4) were identified as independent risk factors for late mortality. At least one RV-PA conduit catheterization or surgical reintervention was performed in 109 patients (median time to reintervention:23 months, range:0.3-93). Risk factors for reintervention included use of pulmonary or aortic homografts versus Contegra® bovine jugular vein conduits (HR:1.9; 95%CI:1.2,3.1) and smaller conduit size (HR per mm/m2:1.05; 95%CI:1.03,1.08). Conclusions In a multicenter dataset, DiGeorge syndrome and need for tracheostomy postoperatively were found to be independent risk factors for late mortality after repair of truncus arteriosus, while risk of conduit reintervention was independently influenced by both initial conduit type and size.
Multicenter Validation of the Vasoactive-Ventilation-Renal Score as a Predictor of Prolonged Mechanical Ventilation After Neonatal Cardiac Surgery
(Wolters Kluwer, 2018-11) Cashen, Katherine; Costello, John M.; Grimaldi, Lisa M.; Gowda, Keshava Murty Narayana; Moser, Elizabeth A. S.; Piggott, Kurt D.; Wilhelm, Michael; Mastropietro, Christopher W.; Pediatrics, School of Medicine
Objectives: We sought to validate the Vasoactive-Ventilation-Renal score, a novel disease severity index, as a predictor of outcome in a multicenter cohort of neonates who underwent cardiac surgery. Design: Retrospective chart review. Setting: Seven tertiary-care referral centers. Patients: Neonates defined as age less than or equal to 30 days at the time of cardiac surgery. Interventions: Ventilation index, Vasoactive-Inotrope Score, serum lactate, and Vasoactive-Ventilation-Renal score were recorded for three postoperative time points: ICU admission, 6 hours, and 12 hours. Peak values, defined as the highest of the three measurements, were also noted. Vasoactive-Ventilation-Renal was calculated as follows: ventilation index + Vasoactive-Inotrope Score + Δ creatinine (change in creatinine from baseline × 10). Primary outcome was prolonged duration of mechanical ventilation, defined as greater than 96 hours. Receiver operative characteristic curves were generated, and abilities of variables to correctly classify prolonged duration of mechanical ventilation were compared using area under the curve values. Multivariable logistic regression modeling was also performed. Measurements and Main Results: We reviewed 275 neonates. Median age at surgery was 7 days (25th–75th percentile, 5–12 d), 86 (31%) had single ventricle anatomy, and 183 (67%) were classified as Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery Congenital Heart Surgery Mortality Category 4 or 5. Prolonged duration of mechanical ventilation occurred in 89 patients (32%). At each postoperative time point, the area under the curve for prolonged duration of mechanical ventilation was significantly greater for the Vasoactive-Ventilation-Renal score as compared to the ventilation index, Vasoactive-Inotrope Score, and serum lactate, with an area under the curve for peak Vasoactive-Ventilation-Renal score of 0.82 (95% CI, 0.77–0.88). On multivariable analysis, peak Vasoactive-Ventilation-Renal score was independently associated with prolonged duration of mechanical ventilation, odds ratio (per 1 unit increase): 1.08 (95% CI, 1.04–1.12). Conclusions: In this multicenter cohort of neonates who underwent cardiac surgery, the Vasoactive-Ventilation-Renal score was a reliable predictor of postoperative outcome and outperformed more traditional measures of disease complexity and severity.
Pediatric Cardiac Tumors: A 45-year, Single Institution Review
(Sage, 2015-04) Linnemeier, Laura; Benneyworth, Brian D.; Turrentine, Mark; Rodefeld, Mark; Brown, John; Department of Pediatrics, IU School of Medicine
Background: Cardiac tumors in children are rare. Of the cases reported in the literature, nearly all are benign and managed conservatively. Methods: This is a retrospective, observational study of pediatric patients <18 years who presented for surgical evaluation of a cardiac tumor, between 1969 and 2014 at a tertiary care children’s hospital. Presentation, pathology, management, and outcomes were evaluated. Results: Over the last 45 years, 64 patients were evaluated for surgical resection of a cardiac tumor. Rhabdomyoma was the most common neoplasm (58%), and 17% of the tumors had malignant pathologies. While 42% of benign cardiac neoplasms required surgical intervention for significant hemodynamic concerns, 73% of malignant neoplasms underwent radical excision, if possible, followed by adjuvant chemotherapy. Despite a 37% mortality in patients with malignant pathology, an aggressive surgical approach can yield long-term survival in some patients. There were no deaths among patients with benign tumors and 17% had postoperative complications mostly related to mitral regurgitation. Conclusion: Cardiac tumors in children are rare but can be managed aggressively with good outcomes. Benign tumors have an excellent survival with most complications related to tumor location. Malignant tumors have a high mortality rate, but surgery and adjuvant chemotherapy allow for prolonged survival in selected patients.
Performance of SynerGraft Decellularized Pulmonary Allografts Compared With Standard Cryopreserved Allografts: Results From Multiinstitutional Data
(Elsevier, 2017-03) Bibevski, Steve; Ruzmetov, Mark; Fortuna, Randall S.; Turrentine, Mark W.; Brown, John W.; Ohye, Richard G.; Surgery, School of Medicine
Background Structural deterioration of allografts over time is believed to be at least partly related to an immune response mounted against human leukocyte antigen specific to the transplanted tissue. SynerGraft (SG) processing (CryoLife, Kennesaw, GA) is a technology that decellularizes an allograft leaving only connective tissue, therefore, reducing immunogenicity and potentially increasing durability of the implant. Methods We performed a retrospective review of 163 SG patients and 124 standard allograft controls from 3 medical centers. Patient demographics were tabulated, and conduit stenosis and insufficiency were measured by echocardiography. Results There were 28 deaths (15 of 163 [9%] SG patients vs 13 of 124 [11%] standard patients; p = 0.72), but no deaths were attributed to structural failure of the conduit. The actuarial survival for SG vs standard cohorts was not different at 5 and 10 years. Among the 274 hospital survivors, 17% SG vs 42% standard had evidence for significant conduit dysfunction at the most recent follow-up or before conduit replacement. Freedom from conduit dysfunction was significantly worse at 10 years in the standard group (58%) than in the SG group (83%, p < 0.001). Conclusions This study represents a multiinstitutional retrospective comparison of SG and standard cryopreserved allografts used in right ventricular outflow tract reconstruction in a broad range of patient ages. Our results demonstrate that at an intermediate-term to long-term follow-up, conduit dysfunction and pulmonary insufficiency and stenosis are higher among patients receiving standard allografts. We postulate that the improved durability of SG is related to decreased immunogenicity of the SG technology.

Browsing by Subject "congenital heart disease"

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