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Cangrelor use in a 6-year-old patient undergoing complex percutaneous coronary intervention after post-surgical myocardial infarction.

Platelets. 2020 Feb 23;:1-4

Authors: Sirico D, Morici N, Soriano F, Marianeschi SM, Pedrazzini G, Leonardi S, Vignati G

Abstract
Cangrelor is an intravenously administered P2Y12 receptor antagonist, which has been approved for adult patients undergoing percutaneous coronary intervention and, due to its unique pharmacokinetics, it allows effective and controllable peri-procedural platelet inhibition. We report the case of a 6-year-old child with anomalous origin of right coronary artery from aortic left coronary sinus, who underwent elective surgical replacement of stenotic and calcified conduit between the right ventricle and the main pulmonary artery. The surgery was complicated by acute myocardial infarction secondary to coronary extrinsic compression. The patient was successfully treated with urgent percutaneous coronary intervention (simultaneous V-stenting) and cangrelor infusion, subsequently switched to clopidogrel therapy.

PMID: 32090666 [PubMed - as supplied by publisher]

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Surgical Management of Heterotaxy Syndrome: Current Challenges and Opportunities.

World J Pediatr Congenit Heart Surg. 2020 Mar;11(2):166-176

Authors: Alongi AM, Kirklin JK, Deng L, Padilla L, Pavnica J, Romp RL, Mauchley DC, Cleveland DC, Dabal RJ

Abstract
INTRODUCTION: Heterotaxy syndrome presents a unique challenge in surgical management, even in the current era. We hypothesized that certain anatomic subsets merit novel strategies.
METHODS: We analyzed morphologic details, surgeries, comorbidities, subsequent admissions, and survival using Kaplan-Meier methods and multivariable risk models from a single-institution experience of 103 consecutive patients with heterotaxy who underwent cardiac surgery between January 1, 1990, and May 31, 2016.
RESULTS: Of the 103 patients (50 males and 53 females), 31 had left atrial isomerism, 64 had right atrial isomerism (RAI), and 8 patients' isomerism was indeterminate (IND), with first cardiac operation at a mean 1.0 year (standard deviation ±3.0 years) of age. Kaplan-Meier overall survival estimate was 83.1% at six months, 77.8% at one year, 65.9% at five years, and 52.1% at ten years. Survival was particularly low among RAI following repair of total anomalous pulmonary venous connection (TAPVC) at first operation, with one- and five-year survival of 57% and 46%, respectively. By multivariable analysis, the only risk factor for death during the early phase (hazard model) was repair of TAPVC at the first cardiac operation (hazard ratio [HR]: 4.4, P = .01), and risk factors during the longer term constant phase were atrioventricular valve (AVV) regurgitation (HR: 4.2, P < .01), male gender (HR: 3.7, P < .01), and two-ventricle repair (HR: 3.0, P = .02). Patients with heterotaxy undergoing the Fontan procedure had excellent subsequent survival (85% at ten years).
CONCLUSIONS: This analysis of over 100 patients with heterotaxy identified TAPVC requiring initial repair as the major risk factor for early death and important AVV regurgitation as the major risk factor in the longer term. Survival with RAI and early repair of TAPVC were poor, with one-year mortality exceeding 40%. Patients with single ventricle completing the Fontan operation enjoyed outstanding ten-year survival (85%). Initial management of RAI requiring early repair of TAPVC remains challenging. For this high-risk subset, alternative strategies such as early referral for cardiac transplantation evaluation warrant consideration.

PMID: 32093563 [PubMed - in process]

Early clinical experience with the straight design of Venus P-valve™ in dysfunctional right ventricular outflow tracts.

Catheter Cardiovasc Interv. 2020 Feb 25;:

Authors: Morgan GJ, Sivakumar K, Promphan W, Goreczny S, Prachasilchai P, Qureshi S

Abstract
OBJECTIVES: To assess the initial procedural and short to medium-term experience with the straight design of the Venus P-valve™ (Venus MedTech, Hangzhou, China) in dysfunctional right ventricular outflow tracts (RVOT).
BACKGROUND: The Melody™ valve (Medtronic, Minneapolis, Minnesota) has been the only percutaneous valve option for smaller RVOT conduits. The straight Venus P-valve™ may provide an alternative to the Melody™ valve.
METHODS: Retrospective data collection of patient characteristics, procedural data, clinical and imaging follow-up of the straight Venus P-valve™.
RESULTS: Nine patients (four female) with a mean age of 23.1 ± 7.5 years and a mean weight of 72.7 ± 29.4 kg underwent straight Venus P-valve™ implantation between 03/2014 and 06/2016. All patients had right ventricle-to-pulmonary artery conduits which were pre-stented before the valve implantation. All valves were deployed successfully without any significant procedural complications. During the mean follow-up of 24 ± 9.1 months, there were no valve related re-interventions or deterioration in valve performance. There was one case of insignificant, single wire frame fracture and no cases of endocarditis. The cohort demonstrated a reduction in pulmonary regurgitation and tricuspid regurgitation, which was sustained throughout the follow-up. Similarly the gradient across the RVOT tract did not significantly increase.
CONCLUSIONS: Implantation of the straight Venus P-valve™ has provided satisfactory short to mid-term results with high success rates and no complications and may be considered as an alternative option in patients with RVOT dysfunction.

PMID: 32096924 [PubMed - as supplied by publisher]

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Evaluation of pulmonary endothelial function in Fontan patients.

J Thorac Cardiovasc Surg. 2019 08;158(2):523-531.e1

Authors: Latus H, Lederle A, Khalil M, Kerst G, Schranz D, Apitz C

Abstract
OBJECTIVES: Pulmonary endothelial dysfunction due to a loss of pulsatile pulmonary blood flow is thought to be a major contributor in the development of increased pulmonary vascular resistance (PVR) in patients with a Fontan circulation. We sought to evaluate pulmonary endothelial dysfunction by vasodilator response to acetylcholine in children and adolescents with Fontan hemodynamics.
METHODS: Twenty-one Fontan patients with a median age of 10.7 years (range, 3.4-30.0 years) underwent invasive cardiac catheterization, including intra-arterial Doppler and pressure measurements in a segmental pulmonary artery. Pulmonary flow reserve (PFR) was quantified as the change of average peak velocity in response to acetylcholine infusion. Local PVR was assessed by the ratio of local pressure and blood flow velocity.
RESULTS: Average peak velocity significantly increased from 16.4 ± 6.6 cm/s to 20.1 ± 6.4 cm/s (P = .002) resulting in a mean PFR of 1.28 ± 0.37. Local PVR dropped from 0.72 ± 0.32 mm Hg/cm/s to 0.57 ± 0.20 mm Hg/cm/s (n = 19; P = .01). A significant relationship was found between baseline local PVR and PFR (r = 0.73; P = .0006) as well as between PFR and the percent decrease in local PVR (r = 0.85; P < .0001). In addition, percentage decrease in local PVR correlated with New York Heart Association functional class, whereas neither PFR nor local PVR correlated with global PVR.
CONCLUSIONS: The assessment of PFR and local PVR may help to evaluate adverse pulmonary vascular remodeling in Fontan patients as an adjunct to the usual catheterization protocol, particularly given the difficulties in assessing global PVR in these patients. Future research is required to study whether impaired local pulmonary endothelial function may be associated with Fontan failure.

PMID: 30954300 [PubMed - indexed for MEDLINE]

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Long-Term Survival of Patients With Coarctation Repaired During Infancy (from the Pediatric Cardiac Care Consortium).

Am J Cardiol. 2019 09 01;124(5):795-802

Authors: Oster ME, McCracken C, Kiener A, Aylward B, Cory M, Hunting J, Kochilas LK

Abstract
Patients who undergo coarctation repair during infancy have excellent early survival but long-term survival is unknown. We aimed to describe the long-term survival of patients with coarctation repaired during infancy and determine predictors of mortality. We performed a retrospective cohort study using data from the Pediatric Cardiac Care Consortium for patients with coarctation who underwent surgical repair before 12 months of age between 1982 and 2003. Long-term transplant-free survival was obtained by linkage with the National Death Index and the Organ Sharing Procurement Network. Kaplan Meier survival plots were constructed, and univariate and multivariable analyses were performed to determine predictors of mortality. We identified 2,424 coarctation patients who met inclusion criteria. At 20 years postoperatively, 94.5% of all patients and 95.8% of those discharged after initial operation remained alive, respectively. Significant multivariable predictors of mortality included surgical weight <2.5 kg (hazard ratio [HR] 3.70, 95% confidence interval [CI] 2.19 to 6.24), presence of a genetic syndrome (HR 2.40, 95% CI 1.13 to 5.10), and repair before 1990 (HR 1.91, 95% CI 1.09 to 3.34). None of the other factors examined including age at repair, gender, coarctation type, or surgical approach were found to be statistically significant. Over half of the deaths were due to the underlying congenital heart disease or other cardiovascular etiology. Overall long-term survival of patients who undergo coarctation repair during infancy is excellent. However, patients do experience small continued survival attrition throughout early adulthood. Ongoing monitoring of this cohort is necessary to assess late mortality risk.

PMID: 31272703 [PubMed - indexed for MEDLINE]

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Stenting of modified Blalock-Taussig shunt in adult with palliated pulmonary atresia and ventricular septal defect: a case report.

Eur Heart J Case Rep. 2019 Dec;3(4):1-4

Authors: Illner J, Reinecke H, Baumgartner H, Kaleschke G

Abstract
Background: Adults with complex congenital heart disease palliated with systemic-to-pulmonary artery shunts have become rare and represent a particularly challenging patient group for the cardiologist. One of the complications and causes of severe clinical deterioration during long-term follow-up are progressive obstruction or total occlusion of the shunt. The risk for surgical intervention is frequently high and catheter intervention may be complicated by complex anatomy and shunt calcification.
Case summary: We report the case of a 47-year-old man with uncorrected (palliated) pulmonary atresia and ventricular septal defect who presented with progressive cyanosis (oxygen saturation 69%) and decreasing exercise capacity. Computed tomography revealed a totally occluded modified left Blalock-Taussig (BT) shunt and a severely stenosed central shunt (Waterston-Cooley) in a patient with confluent but hypoplastic pulmonary arteries and multiple major aortic pulmonary collaterals. Due to a high operative risk, an interventional, percutaneous approach was preferred to re-do surgery. From a radial access the calcified BT shunt could be crossed with a hydrophilic guidewire. Then, a rotational thrombectomy, balloon dilatation, and bare-metal stenting at the proximal and distal anastomoses were performed. Post-interventionally, peripheral oxygen saturation increased from 69% to 82%. Clopidogrel was administered for 1 month after bare-metal stenting. At 1-year follow-up, the BT shunt was still patent on echocardiography and exercise tolerance markedly improved.
Discussion: This case highlights the benefit of percutaneous rotational thrombectomy followed by stenting of chronically occluded systemic-to-pulmonary artery shunts for further palliation in adult patients with complex congenital heart disease not suitable for surgical repair.

PMID: 32099958 [PubMed]

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Emergency department evaluation of chest pain among adult congenital heart disease patients.

Am Heart J. 2020 Feb 05;222:191-198

Authors: Gales J, Krasuski RA, Awerbach JD

Abstract
BACKGROUND: Data regarding emergency department (ED) assessment of acute chest pain (CP) and incidence of myocardial infarction (MI) among adult congenital heart disease (ACHD) patients, relative to the non-congenital population, is lacking.
OBJECTIVES: To describe MI risk in ACHD patients presenting to the ED with chest pain and to compare clinical characteristics, diagnostic testing patterns, and outcomes to controls.
METHODS: We retrospectively identified a cohort of ACHD patients presenting with acute CP and matched them with non-ACHD controls at a large tertiary-level ED during the period 1998-2018.
RESULTS: The congenital and control cohorts comprised 297 patients respectively. While MI was less common among ACHD patients (5.2%) than controls (19.7%), P = .01, arrhythmia (14% vs 6%, P < .001) and acute heart failure (3% vs 0.3%, P = .02) were more often the cause of symptoms. Despite more often presenting with non-anginal CP (81% vs 66%, P < .001) and having fewer CAD risk factors (P = .03), ACHD patients underwent more frequent stress testing (22% vs 14%, P < .001) and underwent invasive coronary angiography with equal frequency (7% vs 8%, P = .99). The trend of greater diagnostic scrutiny for acute coronary disease, in the absence of increased risk, strongly correlated with degree of congenital complexity. Both CP character and HEART Score reliably predicted MI for ACHD patients and controls (both P < .001).
CONCLUSION: MI is an uncommon cause of CP among ACHD patients presenting to the ED and occurs less frequently than seen in the general population. Established MI predictors, CP character and HEART Score, can reliably identify MI in ACHD patients.

PMID: 32105985 [PubMed - as supplied by publisher]

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Heart Failure in Single Right Ventricle Congenital Heart Disease: Physiologic and Molecular Considerations.

Am J Physiol Heart Circ Physiol. 2020 Feb 28;:

Authors: Garcia AM, Beatty JT, Nakano SJ

Abstract
Due to remarkable surgical and medical advances over the past several decades, there are growing numbers of infants and children living with single ventricle congenital heart disease (SV), where there is only one functional cardiac pumping chamber. Nevertheless, cardiac dysfunction and ultimately heart failure is a common complication in the SV population and pharmacologic heart failure therapies have largely been ineffective in mitigating the need for heart transplantation. Given that there are several inherent risk factors for ventricular dysfunction in the setting of SV in addition to probable differences in molecular adaptations to heart failure between children and adults, it is perhaps not surprising that extrapolated adult heart failure medications have had limited benefit in children with SV heart failure. Further investigations into the molecular mechanisms involved in pediatric SV heart failure may assist with risk stratification as well as development of targeted, efficacious therapies specific to this patient population. In this review, we present a brief overview of SV anatomy and physiology, with a focus on patients with a single morphologic right ventricle requiring staged surgical palliation. Additionally, we discuss outcomes in the current era, risk factors associated with the progression to heart failure, present state of knowledge regarding molecular alterations in end-stage SV heart failure and current therapeutic interventions. Potential avenues for improving SV outcomes, including identification of biomarkers of heart failure progression, implications of personalized medicine and stem cell-derived therapies and applications of novel models of SV disease, are proposed as future directions.

PMID: 32108525 [PubMed - as supplied by publisher]

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Features associated with myocardial ischemia in anomalous aortic origin of a coronary artery: A Congenital Heart Surgeons' Society study.

J Thorac Cardiovasc Surg. 2019 09;158(3):822-834.e3

Authors: Jegatheeswaran A, Devlin PJ, McCrindle BW, Williams WG, Jacobs ML, Blackstone EH, DeCampli WM, Caldarone CA, Gaynor JW, Kirklin JK, Lorber RO, Mery CM, St Louis JD, Molossi S, Brothers JA

Abstract
OBJECTIVES: We sought to determine anatomic features associated with evidence of myocardial ischemia and sudden cardiac events (arrest or death) for patients with anomalous aortic origin of a coronary artery.
METHODS: We enrolled 560 patients, less than or equal to 30 years, at diagnosis from 40 institutions. Ischemia was defined as the presence of exertional syncope, a sudden cardiac event (arrest/death), or abnormal investigation results. Data on detailed anatomic features were abstracted from echocardiography, computed tomography, magnetic resonance imaging, operative, and/or surgeon-completed reports.
RESULTS: There were 236 patients with negative ischemia test results, and 49 with evidence of ischemia (including 18 who presented with a sudden cardiac event); 275 asymptomatic patients who had not undergone provocative ischemia testing were excluded from primary analyses. Patients with ischemia (vs without), were more likely to have left anomalous coronary arteries (28/49 vs 46/236; P < .0001). Of patients with ischemia (vs without), those with anomalous left coronary arteries were more likely to have an intramural coronary artery course, or a high or slit-like coronary artery orifice. Of patients with ischemia (vs without), those with anomalous right coronary arteries were more likely to have a longer intramural course. Among patients with ischemia, the occurrence of sudden cardiac events was not shown to have any associated anatomic features.
CONCLUSIONS: Anatomic features including coronary artery involved, intramural course and length, and orifice anomalies were associated with evidence of myocardial ischemia for patients with anomalous aortic origin of a coronary artery. These features might importantly inform risk stratification and decisions regarding surgical management.

PMID: 31235351 [PubMed - indexed for MEDLINE]

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Single- and dual-site ventricular pacing entirely through the coronary sinus for patients with prior tricuspid valve surgery.

J Interv Card Electrophysiol. 2019 Oct;56(1):79-89

Authors: Lee CC, Do K, Patel S, Carlson SK, Konecny T, Chang PM, Doshi RN

Abstract
PURPOSE: Transvenous right ventricular pacing has traditionally been avoided after surgical tricuspid valve repair or replacement because of possible valvular dysfunction. Epicardial pacing has been used but it requires surgical thoracotomy and has higher lead failure rates when compared to transvenous pacing. We evaluated the lead stability and clinical outcomes in patients with isolated coronary sinus (CS) lead due to relative contraindication to transvenous pacing from prior tricuspid valve (TV) surgery.
METHODS: We retrospectively examined a single-center cohort of 34 patients with TV disease and/or surgery who underwent permanent pacemaker implantation with a left ventricular CS lead as the only ventricular pacing lead (to avoid crossing the TV). The clinical outcome, echocardiographic data, and pacing thresholds were evaluated at follow-up.
RESULTS: We implanted 19 patients with a single-CS lead and 15 patients with dual-CS leads. The average left ventricular ejection fraction was 56 ± 13% prior to lead implantation and remained stable at 2-year follow-up. The tricuspid regurgitation remained mild at follow-up. The average lead pacing threshold was 1.2 ± 0.6 V × ms at implant and 1.1 ± 0.4 V × ms at 2-year follow-up (P = 0.39). For patients with dual-CS leads, the pacing threshold was 1.2 ± 0.7 V × ms at implant and 1.1 ± 0.5 V × ms at 2-year follow-up (P = 0.52).
CONCLUSIONS: The use of ventricular pacing entirely through the CS is an effective and minimally invasive method that provides stable pacing for patients with prior TV surgery in whom transvenous lead placement either is not possible or is relatively contraindicated.

PMID: 31432385 [PubMed - indexed for MEDLINE]

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