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Pulmonary Insufficiency: Advantage of Pulmonary Regurgitation Volume Versus Pulmonary Regurgitation Fraction in a Congenital Heart Disease Mixed Population.

J Thorac Imaging. 2019 Nov;34(6):380-386

Authors: Secchi F, Chessa M, Petrini M, Monti CB, Alì M, Cannaò PM, Di Leo G, Sardanelli F

Abstract
OBJECTIVE: The objective of this study was to compare the use of pulmonary regurgitation volume (PRV) or indexed PRV (PRVi) with that of pulmonary regurgitation fraction (PRF) in the assessment of patients with pulmonary regurgitation (PR) undergoing cardiac magnetic resonance (CMR) imaging.
MATERIALS AND METHODS: CMR of 176 patients with PR were retrospectively evaluated. Their right ventricular diastolic (end-diastolic volume index [EDVi]) and systolic (end-systolic volume index) volume indexes, stroke volume, and ejection fraction were obtained from cine CMR sequences, whereas phase-contrast flow sequences were analyzed to obtain PRV, PRVi, and PRF. Patients were divided into subgroups, according to underlying pathology and according to PR severity. Correlations between PRV or PRF and RV parameters were studied through Spearman ρ, both in the main group and subgroups. Follow-up examinations were analyzed, and correlations between PRV or PRF from the first CMR examination and volume data from the second were calculated.
RESULTS: Tetralogy of Fallot was the main setting of PR (98/179). Overall, EDVi strongly correlates with PRV (ρ=0.592, P<0.001) than with PRF (ρ=0.522, P<0.001), and end-systolic volume index strongly correlates with PRV (ρ=0.454, P<0.001) and PRF (ρ=0.406, P <0.001). As regards subgroup analysis, in moderate or severe PR patients, EDVi strongly correlates (P=0.043) with PRV (ρ=0.499, P<0.001) than with PRF (ρ=0.317, P<0.001). Follow-up EDVi correlates with PRV (ρ=0.450, P=0.031), but not with PRF.
CONCLUSIONS: Especially when assessing moderate to severe PR, PRV and PRVi may be better indicators of right ventricular dysfunction than PRF. Moreover, PRV may be a predictor of worsening RV dilation.

PMID: 30870306 [PubMed - indexed for MEDLINE]

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Outcomes of Repair of Kommerell Diverticulum.

Ann Thorac Surg. 2019 12;108(6):1745-1750

Authors: Vinnakota A, Idrees JJ, Rosinski BF, Tucker NJ, Roselli EE, Pettersson GB, Vekstein AM, Stewart RD, Raja S, Svensson LG

Abstract
BACKGROUND: Kommerell diverticulum (KD) is an uncommon congenital vascular anomaly. This study assessed outcomes of open and endovascular repair of this disorder.
METHODS: Between 1997 and 2016, 152 adult patients presented with KD at Cleveland Clinic (Cleveland, OH), 87 had no intervention, and 65 underwent open repair (n = 55; 19 elephant trunk [ET] procedures [9 with aortic dissection], including 7 frozen ET, and completions with 5 thoracic endovascular aortic repairs, 2 open, and 5 lost to follow-up) or endovascular procedures (n = 10). Non-ET open KD repairs consisted of resection (n = 15), interposition graft (n = 16), patch (n = 4), or aortopexy (n = 1). Maximum KD diameter was 2.1 cm for nonsurgical patients and 3.2 cm for surgical patients. Among surgical patients, 51 of 65 had dysphagia or dyspnea. A total of 608 patient-years of follow-up were available.
RESULTS: For patients who underwent open and endovascular procedures, after multivariable adjustment, symptoms of dysphagia and hypertension predicted the likelihood of surgery (P < .05, all). There was no operative mortality. Complications included nondisabling stroke (n = 5; 8%), tracheostomy (n = 3; 4.6%), vocal cord paralysis (n = 2; 3%), and reoperation for bleeding (n = 3; 4.6%). During follow-up, 3 of 10 patients treated with hybrid or endovascular procedures required reinterventions for endoleaks. One patient had residual symptoms after aortopexy. Among nonsurgical patients, 2 patients refused surgery, and 1 died of aortic rupture, with a 4.7-cm descending aorta and a 3.4-cm KD. Seven additional patients died of nonaortic comorbidities. The remaining patients were asymptomatic, with an aortic diameter smaller than 4.5 cm.
CONCLUSIONS: Open and endovascular approaches have a high success rate and low mortality risk. Selection of the specific type of intervention should be based on patient anatomy, additional needed procedures, and comorbid conditions.

PMID: 31254511 [PubMed - indexed for MEDLINE]

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Contemporary Outcomes of the Fontan Operation: A Large Single-Institution Cohort.

Ann Thorac Surg. 2019 11;108(5):1439-1446

Authors: Mery CM, De León LE, Trujillo-Diaz D, Ocampo EC, Dickerson HA, Zhu H, Adachi I, Heinle JS, Fraser CD, Ermis PR

Abstract
BACKGROUND: The Fontan procedure is the accepted standard for single-ventricle palliation. The goal of this study was to determine short- and midterm outcomes of patients undergoing a Fontan operation at a single institution and to identify contemporary risk factors for acute and chronic failure.
METHODS: All patients undergoing a Fontan operation between 1995 and 2016 were included. Failure was defined as death, transplantation, Fontan takedown or revision, fenestration creation or enlargement, plastic bronchitis, protein-losing enteropathy, or major perioperative reintervention. Multivariable logistic and Cox regression models were used to identify risk factors for acute (perioperative) and chronic failure (after hospital discharge or 30 days postoperatively, or both).
RESULTS: The cohort included 610 patients. Median age at surgery was 4 years. Median follow-up was 6.8 years. Trends showed increasing use of extracardiac conduits, nonfenestrated Fontan, and extubation in the operating room. Perioperative mortality was 0.5% (n = 3). Transplant-free survival at 5, 10, and 15 years was 97%, 94%, and 92%, respectively; freedom from failure was 91%, 89%, and 87%, respectively. Extubation in the operating room was associated with lower risk of acute failure (odds ratio, 0.30; 95% confidence interval [CI], 0.11 to 0.87). Independent risk factors for chronic failure included genetic syndrome (hazard ratio [HR], 2.54; 95% CI, 1.11 to 5.83), ventricular dysfunction (HR, 3.86; 95% CI, 1.81 to 8.24), cardiopulmonary bypass time in 30-minute intervals (HR, 1.242; 95% CI, 1.100 to 1.402), and persistent pleural effusions (HR, 4.26; 95% CI, 2.25 to 8.07). Moderate or severe atrioventricular valve regurgitation (HR, 2.61; 95% CI, 1.13 to 6.02) and cardiopulmonary bypass time (HR, 1.22; 95% CI, 1.03 to 1.45) were associated with reduced long-term transplant-free survival.
CONCLUSIONS: Contemporary midterm outcomes for Fontan patients are reassuring. Lifelong follow-up is mandatory to determine long-term outcomes and need for additional surgery as patients reach adulthood.

PMID: 31279788 [PubMed - indexed for MEDLINE]

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Outcomes of Bioprosthetic Valves in the Pulmonary Position in Adults With Congenital Heart Disease.

Ann Thorac Surg. 2019 11;108(5):1410-1415

Authors: Egbe AC, Connolly HM, Miranda WR, Dearani JA, Schaff HV

Abstract
BACKGROUND: There are limited data on the incidence of prosthetic valve dysfunction (PVD) after pulmonary valve replacement (PVR). The purpose of this study was to determine the longevity of bioprosthetic valves in the pulmonary position and the factors associated with bioprosthetic valve longevity in adults with congenital heart disease.
METHODS: This retrospective review of adults with bioprosthetic PVR was conducted at the Mayo Clinic in Rochester, Minnesota from 1990 to 2017. The study end point was PVD, defined as peak velocity greater than 4 m/s, severe prosthetic regurgitation, or both. For the purpose of this study we assessed bioprosthetic valve longevity by using 3 different indices: (1) cumulative incidence of PVD, (2) incidence density of PVD, and (3) time to PVD.
RESULTS: There were 807 bioprosthetic PVRs in 573 patients. PVD occurred in 267 (33%) prostheses. Time to PVD was 12.6 ± 3.9 years, the incidence of PVD was 3.2 (95% confidence interval [CI], 3.0 to 3.4) cases per 100 prosthesis-years, and the 15-year cumulative incidence was 48% (95% CI, 43%- to 53%). No difference in prosthesis longevity by type of prosthesis implanted was observed. The multivariate risk factors for PVD were a history of atrial fibrillation (hazard ratio [HR], 1.36; 95% CI, 1.08 to 2.54; P = .014), and greater than moderate right ventricular dysfunction (HR, 1.21, 95% CI, 1.01 to 1.48; P = .049). Postoperative anticoagulation with a vitamin K antagonist was associated with a lower risk of PVD (HR, 0.83; 95% CI, 0.61 to 0.92; P = .038).
CONCLUSIONS: The limited longevity of bioprosthetic valves poses significant concerns about the cumulative lifetime risk of reinterventions. Prospective studies are required to determine if interventions such as treatment of atrial fibrillation and postoperative anticoagulation will delay the occurrence of PVD.

PMID: 31323213 [PubMed - indexed for MEDLINE]

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The Quest for Precision Medicine: Unmeasured Patient Factors and Mortality After Congenital Heart Surgery.

Ann Thorac Surg. 2019 12;108(6):1889-1894

Authors: Pasquali SK, Gaies M, Banerjee M, Zhang W, Donohue J, Russell M, Gaynor JW

Abstract
BACKGROUND: Emerging data across many fields suggest that unique patient characteristics can impact disease manifestation and response to therapy, supporting "precision medicine" approaches and more individualized and targeted therapeutic strategies. In children undergoing congenital heart surgery, current risk models primarily focus on the population level, and their utility in understanding precise characteristics that place individual patients at risk for poor outcome remains unclear.
METHODS: We analyzed index surgeries in the Pediatric Cardiac Critical Care Consortium (PC4) registry (August 2014 to May 2016) and utilized a previously constructed model containing patient factors typically included in in-hospital mortality risk models (age, weight, prematurity, chromosomal anomalies/syndromes, preoperative factors, The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery score). Partitioned variances based on a hierarchical generalized linear model were used to estimate the proportion of variation in mortality explained by these factors.
RESULTS: A total of 8406 operations (22 hospitals) were included. We found that only 30% of the total between-patient variation in mortality in our cohort was explained by the patient factors included in our model. Age, The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery score, and preoperative mechanical ventilation explained the greatest proportion of variation. Of the variation that remained unexplained, 95% was attributable to unmeasured patient factors. In stratified analyses, these results were consistent across patient subgroups.
CONCLUSIONS: Patient factors typically included in congenital heart surgery risk models explain only a small portion of total variation in mortality. A better understanding of other underrecognized factors is critical in further defining risk profiles and in developing more individualized and tailored therapeutic strategies.

PMID: 31398358 [PubMed - indexed for MEDLINE]

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Prognostic value of von Willebrand factor in adult patients with congenital heart disease.

Heart. 2020 Mar 18;:

Authors: Ohuchi H, Hayama Y, Miike H, Suzuki D, Nakajima K, Iwasa T, Konagai N, Sakaguchi H, Miyazaki A, Shiraishi I, Kurosaki KI, Nakai M

Abstract
OBJECTIVES: von Willebrand factor (vWF) has prognostic value in patients with heart failure (HF) and in those with liver disease. Liver congestion, due to right-sided HF (RHF), is one of the major clinical pathophysiologic manifestations in adults with congenital heart disease (ACHD). The present study's purpose was to clarify the prognostic value of plasma levels of vWF antigen (vWF:Ag) in ACHD.
METHODS: We measured vWF:Ag (%) in 382 consecutive patients (20 unrepaired cyanotic ACHD, 172 Fontan patients and 190 ACHD after biventricular repair) and compared the results with the clinical profiles and prognosis.
RESULTS: The plasma vWF:Ag level was 130±53 (normal range: 55%-190%), and 48 patients (13%) showed high levels of vWF:Ag (≥190%). Older age, Fontan circulation, higher central venous pressure, lower arterial oxygen saturation and lower plasma levels of albumin were independently associated with high log (vWF:Ag) (p<0.05-0.0001). During the follow-up of 2.4±1.4 years, 15 patients died. High log (vWF:Ag) predicted the all-cause mortality (HR 1.63 per 0.1, 95% CI 1.40 to 1.96, p<0.0001). Specifically, patients with high vWF:Ag (≥165%) had a substantially higher risk of all-cause mortality (HR 56.4, 95% CI 11.4 to 1020, p<0.0001), and this prognostic value was independent of plasma levels of brain-type natriuretic peptide.
CONCLUSIONS: High vWF:Ag may reflect RHF severity and related liver dysfunction with a strong prognostic value of all-cause mortality in ACHD. Thus, vWF:Ag might be an excellent biomarker for monitoring ACHD with RHF.

PMID: 32188625 [PubMed - as supplied by publisher]

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Permanent pacemaker implantation in pediatric heart transplant recipients: A systematic review and evidence quality assessment.

Pediatr Transplant. 2020 Mar 18;:e13698

Authors: Mylonas KS, Repanas T, Athanasiadis DI, Voulgaridou A, Sfyridis PG, Bakoyiannis C, Kapelouzou A, Avgerinos DV, Tzifa A, Kalangos A

Abstract
Bradyarrhythmias are a common complication following pediatric OHT and may require permanent pacemaker implantation (PPM). The purpose of this study was to investigate the incidence, predictors, and outcomes of children undergoing PPM implantation following OHT. A PRISMA-compliant systematic literature review was performed using the PubMed database and the Cochrane Library (end-of-search date: January 27, 2019). The Newcastle-Ottawa scale and the Joanna Briggs Institute tool were used to assess the quality of cohort studies and case reports, respectively. We analyzed data from a total of 11 studies recruiting 7198 pediatric patients who underwent heart transplant. PPM implantation was performed in 1.9% (n = 137/7,198; 95% CI: 1.6-2.2) of the patients. Most patients underwent dual-chamber pacing (46%, 95% CI: 32.6-59.7). Male-to-female ratio was 1.3:1. Mean patient age at the time of OHT was 10.1 ± 6.3. Overall, biatrial anastomosis was used in 62.2% (95% CI: 52.8-70.6) of the patients. The bicaval technique was performed in the remaining 37.8% (95% CI: 29.4-47.1). Sinus node dysfunction was the most frequent indication for PPM implantation (54.4%; 95% CI: 42.6-65.7) followed by AV block (45.6%; 95% CI: 34.3-57.3). The median time interval between OHT and PPM implantation ranged from 17 days to 12.5 years. All-cause mortality was 27.9% (95% CI: 18.6-39.6) during a median follow-up of 5 years. PPM implantation is rarely required after pediatric OHT. The most common indication for pacing is sinus node dysfunction, and patients undergoing biatrial anastomosis may be more likely to require PPM.

PMID: 32189417 [PubMed - as supplied by publisher]

Abdominal Skeletal Muscle Index as a Potential Novel Biomarker in Adult Fontan Patients.

CJC Open. 2020 Mar;2(2):55-61

Authors: Possner M, Alsaied T, Siddiqui S, Morales D, Trout AT, Veldtman G

Abstract
Background: Fontan palliation results in a chronic multisystem disorder with diminished exercise capacity and increased risk of muscle wasting. The aims of this study were to assess the feasibility of skeletal muscle mass measurements in Fontan patients undergoing magnetic resonance imaging liver surveillance to compare muscle mass with a historic control and to assess its correlation with cardiorespiratory fitness.
Methods: Skeletal muscle area (SMA) and skeletal muscle index (SMI) were measured at T12 and L3. A young, healthy historic cohort was used as a comparison group.
Results: Forty patients with a Fontan circulation (mean age, 25.5 ± 7.9 years; 50% were men) were included. Measurements of SMA and SMI were feasible and highly reproducible. Mean SMA and SMI were significantly lower in women compared with men at both T12 (SMA: 25.1 ± 4.9 cm2 vs 33.5 ± 8.4 cm2, P < 0.001; SMI: 9.7 ± 2.1 cm2/m2 vs 11.3 ± 2.7 cm2/m2, P = 0.045) and L3 (SMA: 121 ± 12 cm2 vs 162 ± 24 cm2, P < 0.001; SMI: 46.9 ± 7.0 cm2/m2 vs 54.5 ± 7.4 cm2/m2, P = 0.002). Mean SMI at L3 was significantly lower in the male Fontan population compared with the healthy historic cohort (54.5 ± 7.4 cm2/m2 vs 60.9 ± 7.8 cm2/m2, P < 0.001), but was similar for women (46.9 ± 7.0 cm2/m2 vs 47.5 ± 6.6 cm2/m2, P = 0.692). SMI at L3, but not at T12, was positively correlated with peak oxygen consumption, oxygen pulse, and workload. Four patients (10%) met criteria for muscle wasting in the sarcopenic range based on L3 measurements.
Conclusions: Abdominal skeletal muscle mass can be reproducibly determined on surveillance liver magnetic resonance imaging scans. Muscle wasting appears to occur commonly in Fontan patients. Further research is needed to better define the value of SMI as a biomarker in the Fontan population.

PMID: 32190826 [PubMed]

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Flexible Modeling of the Association Between Cumulative Exposure to Low-Dose Ionizing Radiation From Cardiac Procedures and Risk of Cancer in Adults With Congenital Heart Disease.

Am J Epidemiol. 2019 08 01;188(8):1552-1562

Authors: Danieli C, Cohen S, Liu A, Pilote L, Guo L, Beauchamp ME, Marelli AJ, Abrahamowicz M

Abstract
Adults with congenital heart disease are increasingly being exposed to low-dose ionizing radiation (LDIR) from cardiac procedures. In a recent study, Cohen et al. (Circulation. 2018;137(13):1334-1345) reported an association between increased LDIR exposure and cancer incidence but did not explore temporal relationships. Yet, the impact of past exposures probably accumulates over years, and its strength may depend on the amount of time elapsed since exposure. Furthermore, LDIR procedures performed shortly before a cancer diagnosis may have been ordered because of early symptoms of cancer, raising concerns about reversal causality bias. To address these challenges, we combined flexible modeling of cumulative exposures with competing-risks methodology to estimate separate associations of time-varying LDIR exposure with cancer incidence and all-cause mortality. Among 24,833 patients from the Quebec Congenital Heart Disease Database, 602 had incident cancer and 500 died during a follow-up period of up to 15 years (1995-2010). Initial results suggested a strong association of cancer incidence with very recent LDIR exposures, likely reflecting reverse causality bias. When exposure was lagged by 2 years, an increased cumulative LDIR dose from the previous 2-6 years was associated with increased cancer incidence, with a stronger association for women. These results illustrate the importance of accurate modeling of temporal relationships between time-varying exposures and health outcomes.

PMID: 31107497 [PubMed - indexed for MEDLINE]

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Transcatheter Correction of Superior Sinus Venosus Atrial Septal Defects as an Alternative to Surgical Treatment.

J Am Coll Cardiol. 2020 Mar 24;75(11):1266-1278

Authors: Hansen JH, Duong P, Jivanji SGM, Jones M, Kabir S, Butera G, Qureshi SA, Rosenthal E

Abstract
BACKGROUND: The superior sinus venosus atrial septal defect (SVASD) is characterized by deficiency of the common wall between the superior vena cava (SVC) and the right upper pulmonary vein (RUPV), which is no longer committed to the left atrium.
OBJECTIVES: This study sought to evaluate the potential for redirecting the SVC and RUPV flow to the right and left atria, respectively, by implantation of a covered stent in the SVC.
METHODS: Review of 48 consecutive adult SVASD patients undergoing assessment for correction. Pre-procedural evaluation included cross-sectional imaging and ex vivo simulation using printed or virtual 3-dimensional models.
RESULTS: Transcatheter correction was performed in 25 patients, with a further 6 awaiting stent implantation. Only 8 patients were deemed technically unsuitable. The procedure involved balloon test inflation in the anticipated stent landing zone with simultaneous transesophageal echocardiography and pulmonary venography to confirm defect closure and unobstructed pulmonary venous drainage, followed by deployment of a 10-zig covered Cheatham platinum stent. Stents of lengths between 5 and 8 cm were implanted. A second, uncovered stent was used for anchoring in 9 patients. The RUPV was protected with a high-pressure balloon during stent implantation to prevent pulmonary venous obstruction in 4 patients. The median follow-up period was 1.4 (interquartile range: 0.8 to 1.7) years, with no mortality. Stent embolization occurred in 1 patient; another required drainage of hemopericardium. Cardiac computed tomography after 3 months confirmed unobstructed pulmonary venous return. At latest follow-up, a residual shunt was present in 1 patient.
CONCLUSIONS: Transcatheter correction of SVASD may be considered as an alternative to surgery in a substantial proportion of patients.

PMID: 32192652 [PubMed - in process]

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