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Limited Accuracy of Administrative Data for the Identification and Classification of Adult Congenital Heart Disease.

J Am Heart Assoc. 2018 Jan 12;7(2):

Authors: Khan A, Ramsey K, Ballard C, Armstrong E, Burchill LJ, Menashe V, Pantely G, Broberg CS

Abstract
BACKGROUND: Administrative data sets utilize billing codes for research and quality assessment. Previous data suggest that such codes can accurately identify adults with congenital heart disease (CHD) in the cardiology clinic, but their use has yet to be validated in a larger population.
METHODS AND RESULTS: All administrative codes from an entire health system were queried for a single year. Adults with a CHD diagnosis code (International Classification of Diseases, Ninth Revision, (ICD-9) codes 745-747) defined the cohort. A previously validated hierarchical algorithm was used to identify diagnoses and classify patients. All charts were reviewed to determine a gold standard diagnosis, and comparisons were made to determine accuracy. Of 2399 individuals identified, 206 had no CHD by the algorithm or were deemed to have an uncertain diagnosis after provider review. Of the remaining 2193, only 1069 had a confirmed CHD diagnosis, yielding overall accuracy of 48.7% (95% confidence interval, 47-51%). When limited to those with moderate or complex disease (n=484), accuracy was 77% (95% confidence interval, 74-81%). Among those with CHD, misclassification occurred in 23%. The discriminative ability of the hierarchical algorithm (C statistic: 0.79; 95% confidence interval, 0.77-0.80) improved further with the addition of age, encounter type, and provider (C statistic: 0.89; 95% confidence interval, 0.88-0.90).
CONCLUSIONS: ICD codes from an entire healthcare system were frequently erroneous in detecting and classifying CHD patients. Accuracy was higher for those with moderate or complex disease or when coupled with other data. These findings should be taken into account in future studies utilizing administrative data sets in CHD.

PMID: 29330259 [PubMed - in process]

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Takotsubo cardiomyopathy in a young adult with transplanted heart: what happened to denervation?

ESC Heart Fail. 2018 Jan 13;:

Authors: Chinali M, Formigari R, Grutter G

Abstract
This manuscript describes the first report of takotsubo cardiomyopathy in a young heart transplant recipient following angry debate. Our patient is a 21-year-old woman with cardiac transplant performed owing to right ventricular failure in congenital heart disease. Positive echocardiography with typical asymmetry of regional function, positive enzymes, and negative biopsy and angiography met the criteria for the diagnosis of takotsubo cardiomyopathy. Patient was discharged after 1 week in good clinical conditions and fully recovered cardiac function. The development of takotsubo cardiomyopathy in transplanted heart suggests that re-innervation occurs, thus representing a target for catecholamine-induced cardiac dysfunction.

PMID: 29330935 [PubMed - as supplied by publisher]

Preoperative Thromboelastographic Profile of Patients with Congenital Heart Disease: Association of Hypercoagulability and Decreased Heparin Response.

J Cardiothorac Vasc Anesth. 2017 Dec 12;:

Authors: Fang ZA, Bernier R, Emani S, Emani S, Matte G, DiNardo JA, Faraoni D, Ibla JC

Abstract
OBJECTIVE: To describe the demographic and thromboelastographic characteristics of patients with congenital heart disease presenting with decreased heparin response before cardiac surgery.
DESIGN: Retrospective, observational study.
SETTING: Single institution, tertiary, academic, university hospital.
PARTICIPANTS: The study comprised 496 pediatric and adult patients undergoing cardiac surgery for congenital heart disease.
INTERVENTIONS: Retrospective review of medical records.
MEASUREMENTS AND MAIN RESULTS: Data on preoperative thromboelastography (TEG), demographics, and response to heparin were collected retrospectively. Logistic regression analysis was used to study the association between TEG and response to heparin. Decreased heparin response (defined as activated clotting time <480 s initial bolus of 300 U/kg heparin) was observed in 23.6% of patients presenting for surgery. Age distribution and preoperative coagulation profiles were similar for both nonresponders and responders to heparin. Preoperatively, nonresponders demonstrated all thromboelastrographic characteristics consistent with a hypercoagulable profile (shorter reaction time, K value, wider angle, and maximum amplitude). Univariate logistic regression identified all TEG variables significantly associated with decreased heparin response. After adjustment for age, procedure type, and the presence of cyanosis, a multivariate logistic regression model identified the TEG variable K (≤1.3 min) as being significantly associated with decreased heparin response (odds ratio 3.7; confidence interval 2.3-5.8; p < 0.0001).
CONCLUSIONS: Decreased response to heparin before cardiac surgery in patients with congenital heart disease is associated with preoperative hypercoagulability identified using a viscoelastic test. Additional studies are needed to better understand the etiology of decreased heparin response and potential clinical strategies to improve anticoagulation management.

PMID: 29331555 [PubMed - as supplied by publisher]

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Associations of Age and Sex With Marfan Phenotype: The National Heart, Lung, and Blood Institute GenTAC (Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions) Registry.

Circ Cardiovasc Genet. 2017 Jun;10(3):

Authors: Roman MJ, Devereux RB, Preiss LR, Asch FM, Eagle KA, Holmes KW, LeMaire SA, Maslen CL, Milewicz DM, Morris SA, Prakash SK, Pyeritz RE, Ravekes WJ, Shohet RV, Song HK, Weinsaft JW, GenTAC Investigators*

Abstract
BACKGROUND: The associations of age and sex with phenotypic features of Marfan syndrome have not been systematically examined in a large cohort of both children and adults.
METHODS AND RESULTS: We evaluated 789 Marfan patients enrolled in the National Heart, Lung, and Blood Institute GenTAC (Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions) Registry (53% male; mean age 31 [range: 1-86 years]). Females aged ≥15 and males aged ≥16 years were considered adults based on average age of skeletal maturity. Adults (n=606) were more likely than children (n=183) likely to have spontaneous pneumothorax, scoliosis, and striae but were comparable in revised Ghent systemic score, ectopia lentis, and most phenotypic features, including prevalence of aortic root dilatation. Prophylactic aortic root replacement and mitral valve surgery were rare during childhood versus adulthood (2% versus 35% and 1% versus 9%, respectively, both P<0.0001). Adult males were more likely than females to have aortic root dilatation (92% versus 84%), aortic regurgitation (55% versus 36%), and to have undergone prophylactic aortic root replacement (47% versus 24%), all P<0.001. Prevalence of previous aortic dissection tended to be higher in males than females (25% versus 18%, P=0.06); 44% of dissections were type B. Type B dissection was strongly associated with previous prophylactic aortic root replacement.
CONCLUSIONS: Pulmonary, skeletal, and aortic complications, but not other phenotypic features, are more prevalent in adults than children in Marfan syndrome. Aortic aneurysms and prophylactic aortic surgery are more common in men. Aortic dissection, commonly type B, occurs in an appreciable proportion of Marfan patients, especially in men and after previous prophylactic aortic root replacement.

PMID: 28600386 [PubMed - indexed for MEDLINE]

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Catheter ablation for atrioventricular nodal reentrant tachycardia in patients with congenital heart disease.

Heart Rhythm. 2016 Jun;13(6):1228-37

Authors: Upadhyay S, Marie Valente A, Triedman JK, Walsh EP

Abstract
BACKGROUND: Variability in atrioventricular (AV) node location in congenital heart disease (CHD) can make catheter ablation for atrioventricular nodal reentrant tachycardia (AVNRT) challenging.
OBJECTIVE: The purpose of this study was to describe institutional technique and outcomes for slow pathway modification in a cohort with CHD.
METHODS: The study consisted of a retrospective review of CHD patients who underwent study from 2001 to 2013 with a diagnosis of AVNRT. Outcomes for slow pathway modification were recorded. In cases in which ablation was deferred, the reasons for this choice were examined.
RESULTS: Forty-nine patients (median age 19 years) were included. CHD anatomy involved d-transposition of the great arteries (n = 6), "congenitally corrected" transposition of the great arteries (n = 4), Ebstein anomaly (n = 4), tetralogy of Fallot (n = 5), venous anomalies (n = 8), single ventricle (n = 16), and miscellaneous (n = 6). Ablation was attempted in 39 patients, using radiofrequency energy in 24, cryoablation in 8, and both in 7. Acute success rate was 92% (36/39). One patient had first-degree block in response to cryoablation, but no other complications occurred. At median follow-up 32 months, 1 patient had AVNRT recurrence. Most of the 10 patients in whom ablation was deferred had single-ventricle anatomy with uncertain AV node location.
CONCLUSION: Ablation for AVNRT in CHD can be accomplished successfully with attention to underlying anatomy and prior surgery. Patients with single ventricle are a difficult subgroup, and a pharmacologic approach may be indicated in some cases if node localization is ambiguous.

PMID: 26804568 [PubMed - in process]

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Decellularized Cryopreserved Allografts as Off-the-Shelf Allogeneic Alternative for Heart Valve Replacement: In Vitro Assessment Before Clinical Translation.

J Cardiovasc Transl Res. 2017 Apr;10(2):93-103

Authors: Iop L, Paolin A, Aguiari P, Trojan D, Cogliati E, Gerosa G

Abstract
Cryopreserved allogeneic conduits are the elective biocompatible choice among currently available substitutes for surgical replacement in end-stage valvulopathy. However, degeneration occurs in 15 years in adults or faster in children, due to recipient's immunological reactions to donor's antigens. Here, human aortic valves were decellularized by TRICOL, based on Triton X-100 and sodium cholate, and submitted to standard cryopreservation (TRICOL-human aortic valves (hAVs)). Tissue samples were analyzed to study the effects of the combined procedure on original valve architecture and donor's cell removal. Residual amounts of nucleic acids, pathological microorganisms, and detergents were also investigated. TRICOL-hAVs proved to be efficaciously decellularized with removal of donor's cell components and preservation of valve scaffolding. Trivial traces of detergents, no cytotoxicity, and abrogated bioburden were documented. TRICOL-hAVs may represent off-the-shelf alternatives for both aortic and pulmonary valve replacements in pediatric and grown-up with congenital heart disease patients.

PMID: 28281241 [PubMed - in process]

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Pregnant women with heart disease: Placental characteristics and their association with fetal adverse events.

Acute Card Care. 2016 Sep;18(3):56-64

Authors: Lima FV, Koutrolou-Sotiropoulou P, Parikh PB, Avila C, Butler J, Stergiopoulos K

Abstract
BACKGROUND: Pregnant women with heart disease (HD) have higher rates of adverse fetal outcomes. We describe placental pathologic characteristics and their association with fetal events.
METHODS: In pregnant women, known HD were categorized into: (1) cardiomyopathy (CM) or (2) other HD (congenital, coronary, arrhythmia, or valvular). Outcomes were maternal major adverse cardiac events (MACE), fetal adverse clinical events (FACE), a composite of infant death, prematurity, underweight status, intracranial hemorrhage, and respiratory distress. Only pathologically reported placental analyses were included.
RESULTS: We studied 86 pregnancies in women with CM and HD, with pathologic analyses on 35 CM and 52 HD placentas. CM placentas, compared with those with HD, were more likely to have ischemic changes (65.7% vs. 37%, p 0.008), demonstrate immaturity (62.90% vs. 10%, p < 0.001), and have a lower weight (p < 0.001), despite similar gestational age. CM was independently associated with increased risk for MACE (OR 7.38, 95%CI 2.20-24.76). Ischemic placental changes were associated with increased odds of FACE (OR 24.78, 95%CI 2.37-259.03).
CONCLUSIONS: Women with CM were more likely to have ischemic placentas, with lower placental and fetal weights, and evidence of immaturity compared with those with other forms of HD, and an increased odds of MACE.

PMID: 29166205 [PubMed - in process]

Filling pressures in Fontan revisited: Comparison between pulmonary artery wedge, ventricular end-diastolic, and left atrial pressures in adults.

Int J Cardiol. 2017 Dec 28;:

Authors: Miranda WR, Egbe AC, Hagler DJ, Taggart NW, Nishimura RA, Connolly HM, Warnes CA

Abstract
BACKGROUND: Pulmonary artery wedge pressure (PAWP) has been shown to correlate better with left atrial pressure (LAP) than ventricular end-diastolic pressure (VEDP) in acquired heart disease. The correlation between VEDP and PAWP and their performance as surrogates for LAP in Fontan patients is unknown.
METHODS: Offline single-beat simultaneous measurement of PAWP and VEDP was performed in 50 adult Fontan patients and non-simultaneous hemodynamic data abstracted for calculation of pulmonary vascular resistance (PVR). For the evaluation of PAWP and VEDP as surrogates for LAP, 14 fenestrated adult Fontan patients were included.
RESULTS: Mean age was 34.2±10years and 54% of patients were female. Tricuspid atresia and double inlet left ventricle were the most common congenital defects (44% and 20%, respectively). Simultaneous mean VEDP was 10.8±4.6mmHg and mean PAWP was 11±4.6mmHg; the PAWP-VEDP correlation was 0.91 (p<0.001). Using non-simultaneous data, right-sided (mean difference 0.6WU·m2, 95% CI 0.2-1.0; p=0.005) and left-sided (mean difference 0.5WU·m2, 95% CI 0.1-0.9; p=0.02) PVRs were significantly higher when PAWP rather than VEDP was used. In fenestrated patients, LAP-right PAWP and LAP-left PAWP correlations were 0.97 and 0.95 (p<0.0001 for both), respectively, whereas the correlation between LAP-VEDP was 0.76 (p=0.007).
CONCLUSIONS: PAWP and VEDP correlate reasonably well in adult Fontan patients but PAWP is a better surrogate for LAP. The use of VEDP instead of PAWP appears to significantly underestimate PVR in these patients.

PMID: 29338918 [PubMed - as supplied by publisher]

Related Articles

MiR-328 targeting PIM-1 inhibits proliferation and migration of pulmonary arterial smooth muscle cells in PDGFBB signaling pathway.

Oncotarget. 2016 Aug 23;7(34):54998-55011

Authors: Qian Z, Zhang L, Chen J, Li Y, Kang K, Qu J, Wang Z, Zhai Y, Li L, Gou D

Abstract
MicroRNAs (miRNAs) have been recognized to mediate PDGF-induced cell dysregulation, but their exact functions remain to be elucidated. By using a sensitive S-Poly(T) Plus qRT-PCR method, the expression profiling of 1,078 miRNAs were investigated in pulmonary artery smooth muscle cells (PASMCs) with or without PDGFBB stimulation. MiR-328 was found as a prominent down-regulated miRNA, displaying a specific dose- and time-dependent downregulation upon PDGFBB exposure. Functional analyses revealed that miR-328 could inhibit PASMCs proliferation and migration both with and without PDGFBB treatment. The Ser/Thr-protein kinase-1 (PIM-1) was identified as a direct target of miR-328, and functionally confirmed by a rescue experiment. In addition, the decrease of miR-328 by PDGFBB might be due to the increased expression of DNA methylation transferase 1 (DNMT1) and DNA methylation. Finally, serum miR-328 level was downregulated in PAH patients associated with congenital heart disease (CHD- PAH). Overall, this study provides critical insight into fundamental regulatory mechanism of miR-328 in PDGFBB-activited PASMCs via targeting PIM- 1, and implies the potential of serum miR-328 level as a circulating biomarker for CHD- PAH diagnosis.

PMID: 27448984 [PubMed - indexed for MEDLINE]

Related Articles

Catheter ablation of supraventricular tachyarrhythmia after extracardiac Fontan surgery.

Heart Rhythm. 2016 Sep;13(9):1891-7

Authors: Moore JP, Shannon KM, Fish FA, Seslar SP, Garnreiter JM, Krause U, Tanel RE, Papez AA, Pilcher TA, Balaji S

Abstract
BACKGROUND: Extracardiac total cavopulmonary connection (E-TCPC) is widely performed for single ventricle palliation, yet there is little experience with catheter ablation in this population.
OBJECTIVES: We hypothesized that atrial tachycardia substrates after primary E-TCPC would be similar to those in other forms of congenital heart disease and that catheter ablation could be performed effectively using a primarily transconduit approach.
METHODS: Catheter ablation characteristics of patients with E-TCPC from 9 centers were collected. Acute procedural success was defined as elimination of all sustained supraventricular tachyarrhythmias. Procedural complications, acute success, and recurrences were assessed.
RESULTS: Forty-six catheter ablation procedures were performed in 36 patients. Access to the atrium was by transconduit puncture in 29 procedures (63%). The most common supraventricular tachyarrhythmia mechanism was intra-atrial reentrant tachycardia (IART) in 21 patients (58%); and for all patients with primary E-TCPC and IART, an isthmus between the atrioventricular valve annulus and the oversewn inferior vena cava was critical for maintenance of tachycardia. Overall, acute success was achieved in 38 procedures (83%). There were 8 complications, with only 1 requiring intervention (epicardial pacemaker) and none related to conduit puncture. Recurrence after the final procedure occurred in 6 patients (17%) over a median follow-up duration of 0.4 years (interquartile range 0.1-1.5 years).
CONCLUSION: Catheter ablation could be performed effectively in this group of patients with E-TCPC, and the underlying IART substrate after primary E-TCPC appears to be reproducible. Catheter ablation may be a reasonable alternative to long-term antiarrhythmic therapy in this patient group.

PMID: 27236028 [PubMed - indexed for MEDLINE]

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