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Necrotizing enterocolitis in patients with congenital heart disease: A single center experience.

J Pediatr Surg. 2018 May;53(5):914-917

Authors: Lau PE, Cruz SM, Ocampo EC, Nuthakki S, Style CC, Lee TC, Wesson DE, Olutoye OO

PURPOSE: The purpose of this study was to evaluate the characteristics of patients with congenital heart disease (CHD) who developed necrotizing enterocolitis (NEC).
METHODS: A retrospective review of neonates with CHD at a tertiary care center between January 2006 and January 2016 was performed. Diagnosis of NEC was based on modified Bell's criteria. Patients were grouped by Risk Adjustment for Congenital Heart Surgery (RACHS-1) or by ductal-dependent (DD) lesions that require a patent ductus arteriosus to supply pulmonary or systemic circulation.
RESULTS: Of 1811 neonates with CHD, 3.4% (n=61) developed NEC. Eighteen (30%) of these required surgical management. The rate of NEC among DD patients was 5% (n=33/653), compared to 2.4% (n=28/1158) in the non-DD group (p=0.003). RACHS-1 score>2 had a higher rate of NEC 6.2% (41/658) compared to RACHS-1≤2 cases, 1.7% (20/1153) (p=0.005). DD patients and complex patients with RACHS-1>2 were more likely to develop NEC after cardiac surgery. Hypoplastic left heart syndrome patients had a rate of 9% (n=16/185). Surgical NEC was more prevalent in the non-DD group. Mortality was similar among groups.
CONCLUSION: CHD patients with ductal-dependent lesions or complex cases (RACHS-1 score>2) have higher rates of NEC than non-ductal-dependent patients or RACHS-1 score of 2 or less. Mortality is similar regardless of ductal dependence, but surgical NEC was more prevalent in non-DD patients.

PMID: 29526349 [PubMed - indexed for MEDLINE]

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Identification of clinically relevant phenotypes in patients with Ebstein anomaly.

Clin Cardiol. 2018 Mar;41(3):343-348

Authors: Cabrera R, Miranda-Fernández MC, Huertas-Quiñones VM, Carreño M, Pineda I, Restrepo CM, Silva CT, Quero R, Cano JD, Manrique DC, Camacho C, Tabares S, García A, Sandoval N, Moreno Medina KJ, Dennis Verano RJ

BACKGROUND: Ebstein anomaly (EA) is a heterogeneous congenital heart defect (CHD), frequently accompanied by diverse cardiac and extracardiac comorbidities, resulting in a wide range of clinical outcomes.
HYPOTHESIS: Phenotypic characterization of EA patients has the potential to identify variables that influence prognosis and subgroups with distinct contributing factors.
METHODS: A comprehensive cross-sectional phenotypic characterization of 147 EA patients from one of the main referral institutions for CHD in Colombia was carried out. The most prevalent comorbidities and distinct subgroups within the patient cohort were identified through cluster analysis.
RESULTS: The most prevalent cardiac comorbidities identified were atrial septal defect (61%), Wolff-Parkinson-White syndrome (WPW; 27%), and right ventricular outflow tract obstruction (25%). Cluster analysis showed that patients can be classified into 2 distinct subgroups with defined phenotypes that determine disease severity and survival. Patients in cluster 1 represented a particularly homogeneous subgroup with a milder spectrum of disease, including only patients with WPW and/or supraventricular tachycardia (SVT). Cluster 2 included patients with more diverse cardiovascular comorbidities.
CONCLUSIONS: This study represents one of the largest phenotypic characterizations of EA patients reported. The data show that EA is a heterogeneous disease, very frequently associated with cardiovascular and noncardiovascular comorbidities. Patients with WPW and SVT represent a homogeneous subgroup that presents with a less severe spectrum of disease and better survival when adequately managed. This should be considered when searching for genetic causes of EA and in the clinical setting.

PMID: 29569399 [PubMed - indexed for MEDLINE]

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Novel oral anticoagulant use in adult Fontan patients: A single center experience.

Congenit Heart Dis. 2018 Jul;13(4):541-547

Authors: Georgekutty J, Kazerouninia A, Wang Y, Ermis PR, Parekh DR, Franklin WJ, Lam WW

OBJECTIVE: Adult Fontan patients are at increased risk for thrombosis and thromboembolic complications leading to increased morbidity and mortality. Most are prescribed antiplatelet or anticoagulant therapy for thromboprophylaxis; novel oral anticoagulants (NOACs) are uncommonly used given lack of data on their use in this population and generalized concerns regarding Fontan patients' abnormal coagulation. We report the largest single-center experience with the use of NOACs for treatment and prophylaxis of thrombosis and thromboembolism in adult Fontan patients.
RESULTS: A retrospective chart review identified 21 patients (11 female, 10 male), median age 33 years (18-50) at first initiation, who were prescribed a NOAC on 27 different occasions. The main indications for anticoagulation were arrhythmia (N = 12), thrombosis (N = 8), and persistent right to left shunts (N = 2); one patient was initially on anticoagulation for arrhythmia but restarted for thrombosis. The most common indications for initiation of a NOAC over warfarin were patient/provider preference (N = 11), labile international normalized ratio (INR) (N = 5), initiation of therapy elsewhere (N = 3), and history of poor clinical follow-up (N = 2). Over a cumulative 316 months of patient therapy, one new thrombotic event was noted. No major or nonmajor bleeding events occurred, and 10 patients experienced minor bleeding that did not require the cessation of therapy. One patient died from multiorgan system failure following an unwitnessed, out of hospital arrest. At present, 10 patients remain on NOAC therapy in the setting of ongoing arrhythmia (N = 4), history of stroke (N = 2), history of pulmonary embolism (N = 2), history of deep vein thrombosis (N = 1), and history of right ventricle thrombus (N = 1).
CONCLUSIONS: While our study is limited by size, our results suggest that NOACs may be a non-inferior alternative to traditional anticoagulation and that further study is warranted.

PMID: 29575675 [PubMed - indexed for MEDLINE]

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Outcomes of Ebstein's Anomaly Patients Treated with Tricuspid Valvuloplasty or Tricuspid Valve Replacement: Experience of a Single Center.

Chin Med J (Engl). 2018 May 05;131(9):1067-1074

Authors: Li B, Sun HS, Pan SW, Xu JP

Background: The incidence of Ebstein's anomaly is extremely low, and except for the Mayo Clinic, no cardiac center has reported on a sufficient number of patients. The aim of our study was to report the outcomes of Ebstein's anomaly patients treated with tricuspid valvuloplasty (TVP) or tricuspid valve replacement (TVR).
Methods: TVP or TVR was performed in 245 patients from July 2006 to April 2016. We reviewed patients' records and contacted patients via outpatient service and over the telephone.
Results: The mean follow-up time was 43.6 ± 32.6 months, and 224 (91.4%) patients underwent follow-up. The mean operative age was 31.2 ± 15.7 years. TVR was performed in 23 patients, and TVP was performed in 201 patients. The 30-day mortality rate was 1.3%, and the overall survival rate was 97.9% at 5 and 10 years. The early mortality rate of the TVP group was lower than that of the TVR group (0.5% vs. 8.7%, P = 0.028), and the overall mortality rate of the TVP group was lower than that of the TVR group, without statistical significance (1.0% vs. 8.7%). After propensity score matching, the rates of mortality and New York Heart Association class ≥ III were lower in the TVP group than those in the TVR group without statistical significance. Seven patients with Type B Wolff-Parkinson-White (WPW) syndrome underwent one-stage surgery, and arrhythmias disappeared. Six patients suffered from episodes of left ventricular outflow tract obstruction (LVOTO) during surgery. Severe LVOTO could be treated with reoperation of the atrialized right ventricle.
Conclusions: Ebstein's anomaly patients treated with TVP or TVR can experience optimal outcomes with midterm follow-up. However, TVP should be the first-choice treatment. Optimal outcomes can be obtained from one-stage operation in patients with Type B WPW syndrome. Severe LVOTO during surgery might be related to improper operation of the atrialized right ventricle.

PMID: 29692378 [PubMed - indexed for MEDLINE]

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Modifiable cardiovascular risk factors in adolescents and adults with congenital heart disease.

Congenit Heart Dis. 2018 Jul;13(4):563-570

Authors: Harris KC, Voss C, Rankin K, Aminzadah B, Gardner R, Mackie AS

OBJECTIVE: Individuals with congenital heart disease (CHD) may be at higher risk of acquired cardiovascular disease than the general population due to their underlying physiology and/or surgical sequelae. We sought to assess the prevalence of cardiovascular disease risk factors in youth and adults with CHD.
METHODS: We assessed cardiovascular health as per the Cardiovascular Health in Ambulatory Care Research Team (CANHEART) health index in patients with CHD aged 15+ years who attended cardiology outpatient clinics. Participants self-reported smoking behavior, fruit and vegetable consumption, physical activity, and whether they had diabetes and hypertension. Individual health indices were categorized into ideal/not ideal, and sum of individual health indices was categorized as poor, intermediate or ideal cardiovascular health as per CANHEART criteria.
RESULTS: We included n = 102 adults (35.4 ± 12.9 years, 46% female) and n = 88 youth (17.2 ±1.1 years, 41% female). Most individuals reported to be nonsmokers (88% youth vs 86% adults) and to consume ≥5 servings of fruit and vegetables per day (83% vs 85%, respectively). More adults than youth were overweight/obese (52% vs 22%, p < 0.001) though more adults than youth reported meeting age-specific physical activity guidelines (84% vs 55%, p < 0.001). According to CANHEART health index criteria, 32% of youth and 27% of adults were in ideal cardiovascular health.
CONCLUSIONS: A low proportion of individuals with CHD are in ideal cardiovascular health, suggesting a need to promote healthy lifestyles during adolescence and throughout adulthood in these individuals.

PMID: 29938932 [PubMed - indexed for MEDLINE]

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Perception scores of siblings and parents of children with hypoplastic left heart syndrome.

Congenit Heart Dis. 2018 Jul;13(4):528-532

Authors: Caris EC, Dempster N, Wernovsky G, Miao Y, Moore-Clingenpeel M, Neely T, Fonseca R, Miller-Tate H, Allen R, Fichtner S, Stewart J, Cua CL

OBJECTIVES: Siblings of children with chronic medical conditions endorse a lower quality of life compared to age-matched peers. Caregiver and sibling-self report of adjustment are often discordant. Congenital heart disease significantly affects family life. To date, there have been no studies addressing the functioning of siblings of children with hypoplastic left heart syndrome, one of the most severe forms of congenital heart disease. The goal of this study was to assess the impact of hypoplastic left heart syndrome on sibling's quality of life as well as the caregiver's perception of this effect.
STUDY DESIGN: Cross-sectional study using a web-based survey distributed via various listservs targeted towards families of children with hypoplastic left heart syndrome. Employed the Sibling Perception Questionnaire, designed to assess sibling and caregiver perceptions of adjustment to chronic illness. A Negative Adjustment Composite Score was calculated for each respondent, with higher values representing more negative adjustment.
RESULTS: Thirty-five caregivers responded. Majority of caregivers were female (74%), white (86%) and college educated (54%). Thirty-two siblings participated, ranging in age from 7 to 30 years of age (12.5 ± 6.3). Most children with hypoplastic left heart syndrome (73%) had undergone the third stage of palliation. Forty-two caregiver-sibling pairs were examined. Caregiver Negative Adjustment Composite Scores were significantly higher than sibling scores, with caregivers reporting more adjustment problems (2.4 ± 0.4) than siblings (2.3 ± 0.3, P < .05). Sibling age was correlated with worse caregiver and sibling scores (r 0.35, P < .05).
CONCLUSIONS: Caregivers of children with hypoplastic left heart syndrome perceive their siblings as struggling more than the children self-report. Siblings tend to report worse adjustment as they get older. These data suggest that programs should include support for the entire family through all ages to optimize quality of life.

PMID: 30019479 [PubMed - indexed for MEDLINE]

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Pheochromocytoma and paraganglioma in Fontan patients: Common more than expected.

Congenit Heart Dis. 2018 Jul;13(4):608-616

Authors: Song MK, Kim GB, Bae EJ, Lee YA, Kim HY, Min SK, Kim JH, Won JK

OBJECTIVE: Pheochromocytoma and paraganglioma (extra-adrenal pheochromocytoma) are rare neuroendocrine tumors that arise from the neuroendocrine cells. Chronic hypoxia is known as a possible cause, and a strong link between cyanotic congenital heart disease and these tumors has been reported. However, reports of phechromocytoma/paraganglioma in Fontan patients were scarce. We herein report seven cases of phechromocytoma/paraganglioma after Fontan operation at a single tertiary center.
METHODS: We retrospectively reviewed medical records and imaging studies who diagnosed as phechromocytoma/paraganglioma after Fontan operation in Seoul National University Children's Hospital.
RESULTS: Seven patients were identified during follow-up after Fontan operation, and the prevalence was 2.5% among Fontan patients greater than 10 years old on active follow-up. Three patients were diagnosed as phechromocytoma and 4 patients as paraganglioma. Median time interval between Fontan operation and diagnosis of pheochromocytoma/paraganglioma was 21.4 years (range, 10.4-29.7 years). Resting percutaneous oxygen saturation varied from 77% to 94%. All patients underwent complete tumor resection. Phechromocytoma recurred in two patients, of whom one patient died at the age of 18 years due to the tumor progression with multiple metastasis and aggravation of heart failure with profound cyanosis. Pheochromocytoma/paraganglioma developed after hepatocellular carcinoma in two patients.
CONCLUSION: Phechromocytoma/paraganglioma could occur in Fontan patients more than expected. Because it is curable by tumor resection during its early phase, early diagnosis and treatment of pheochromocytoma are crucial in Fontan patients not to make hemodynamic deterioration and aggravation of heart failure.

PMID: 30033572 [PubMed - indexed for MEDLINE]

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Outcomes in patients with cor triatriatum sinister.

Congenit Heart Dis. 2018 Jul;13(4):628-632

Authors: Fuchs MM, Connolly HM, Said SM, Egbe AC

OBJECTIVE: To describe outcomes in patients with cor triatriatum sinister (CTS).
DESIGN: Retrospective review of patients with CTS followed at Mayo Clinic Rochester from 1990 to 2016. Clinical notes, operative reports, and baseline imaging studies were reviewed including echocardiogram, magnetic resonance imaging, computed tomography, and cardiac catheterization.
RESULTS: Fifty-seven patients (median age 34 years; men 32 (56%)) were enrolled. Definitive or suspected CTS diagnosis was made by transthoracic echocardiogram in 41 (72%) patients, and additional multimodality imaging was required in 39 (68%) patients. Of these 57 patients, initial diagnosis was made in adulthood in 35 (61%) patients, and 33 of 57 (58%) patients had additional congenital heart disease (CHD) diagnosis. A total of 27 (47%) patients required surgical resection of CTS membrane during median follow-up of 76 months, and these patients were younger at the time of CTS diagnosis (26 vs 41, P = 0.01) and more likely to have associated CHD (55% vs 45%, P = 0.02). There was one perioperative mortality and no late mortality. There was no recurrence of CTS membrane obstruction in the patients that underwent surgery. Similarly there was no significant increase in CTS membrane gradient in the patients that were managed conservatively.
CONCLUSIONS: The natural history of CTS is stability without progressive left atrial obstruction, especially in patients with isolated CTS and in those with initial CTS diagnosis made in adulthood. In patients requiring surgical membrane resection due to flow obstruction, surgery is safe and effective with very low risk of recurrence.

PMID: 30033682 [PubMed - indexed for MEDLINE]

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Renal dysfunction is associated with higher central venous pressures in patients with Fontan circulation.

Congenit Heart Dis. 2018 Jul;13(4):602-607

Authors: Broda CR, Sriraman H, Wadhwa D, Wang Y, Tunuguntla H, Akcan-Arikan A, Ermis PR, Price JF

PURPOSE: Elevated central venous pressure (CVP) has deleterious effects on several organ systems in patients with Fontan circulation. However, the relationship between CVP and estimated glomerular filtration rate (eGFR) has not been assessed in patients with Fontan circulation.
METHODS: Patients with Fontan circulation whose hemodynamics were assessed by catheterization between 1987 and 2015 and had a serum creatinine measured within 72 hours prior to the procedure were included for analysis. Patients with primary kidney disease were excluded. Renal function was calculated by "bedside Schwartz" equation in children (< 18 years) and Modification of Diet in Renal Disease equation in adults. Renal dysfunction (RD) was defined by eGFR < 90mL/min/1.73m2 . Fontan patients with and without RD were compared based on demographics, co-morbidities, medication use, echocardiographic findings, hemodynamics assessed at time of catheterization, and laboratory testing values.
RESULTS: Sixty-seven patients with Fontan circulation met inclusion criteria and 15 patients (22%) had RD; eGFR (mL/min/1.73m2 ) was 60-89in 13 (87%), 45-59in 1 (7%), and 30-45in 1 (7%). Compared to patients with eGFR equal to or greater than 90, patients with RD had higher CVP (18.0 [15.0-21.0] mm Hg vs 13.5 [12.3-16.0] mm Hg (P = 0.001), lower pulmonary blood flow 2.2 [1.9-2.6] L/min/m2 vs 2.8 [2.3-3.7] L/min/m2 , higher ventricular end-diastolic pressure 10.5 [7.0-17.3] mm Hg vs 8.0 [6.0-10.0] mm Hg (P = 0.050), were more likely to have worse atrioventricular valve regurgitation (P = 0.02) and were more likely to be African American (P = 0.009).
CONCLUSIONS: In this study population, renal dysfunction in patients with Fontan circulation is associated with increased CVP and factors that affect CVP. African Americans with Fontan circulation may be at particular risk for renal dysfunction. Continued investigation of the effects of venous congestion on kidneys and other factors associated with renal dysfunction in patients with Fontan circulation is warranted.

PMID: 30079627 [PubMed - indexed for MEDLINE]

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Treatment of Chagas Disease in the United States.

Curr Treat Options Infect Dis. 2018;10(3):373-388

Authors: Meymandi S, Hernandez S, Park S, Sanchez DR, Forsyth C

Purpose of Review: Chagas disease (CD) is endemic to much of Latin America, but also present in the United States (U.S.). Following a lengthy asymptomatic period, CD produces serious cardiac or gastrointestinal complications in 30-40% of people. Less than 1% of the estimated six million cases in the Americas, including 326,000-347,000 in the U.S., are diagnosed. Infected persons are typically unaware and the bulk of clinicians are unfamiliar with current treatment guidelines. This review provides U.S. and other clinicians with the latest knowledge of CD treatment.
Recent Findings: Chagas cardiomyopathy (CCM) causes severe fibrosis and autonomic damage in the myocardium. Eliminating the parasite through antitrypanosomal therapy with benznidazole, a nitroimidazole derivative or nifurtimox, a nitrofuran compound, potentially prevents heart failure and other sequelae of advanced CCM. Benznidazole, recently approved by the U.S. Food and Drug Administration (FDA) for children 2-12 years old, is the first-line therapy; optimal dosages are currently being studied. Antitrypanosomal therapy prevents congenital transmission; produces high cure rates for acute, congenital, and early chronic cases; and improves clinical outcomes in adult chronic indeterminate cases. However, this benefit was not observed in a large clinical trial that included patients with advanced CCM.
Summary: Treatment with antitrypanosomal drugs can cure CD in acute, congenital, and early chronic cases and provides improved clinical outcomes for chronic indeterminate cases. This treatment should be offered as early as possible, before advanced CCM develops.

PMID: 30220883 [PubMed]