Wiley: Congenital Heart Disease: Table of Contents

Pulmonary artery pulsatility index predicts prolonged inotrope/pulmonary vasodilator use after implantation of continuous flow left ventricular assist device

Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Objective

Predictors of right ventricle (RV) dysfunction after continuous-flow left ventricular assist device (CF-LVAD) implantation in children are not well described. We explored the association of preimplantation Pulmonary Artery Pulsatility index (PAPi) and other hemodynamic parameters as predictors of prolonged postoperative inotropes/pulmonary vasodilator use after CF-LVAD implantation.

Design

Retrospective chart review.

Setting

Single tertiary care pediatric referral center.

Patients

Patients who underwent CF-LVAD implantation from January 2012 to October 2017.

Interventions

Preimplantation invasive hemodynamic parameters were analyzed to evaluate the association with post-CF-LVAD need for prolonged (>72 hours) use of inotropes/pulmonary vasodilators.

Measurements and main results

Preimplantation cardiac catheterization data was available for 12 of 44 patients who underwent CF-LVAD implant during the study period. Median (IQR) age and BSA of the cohort were 15.3 years (10.2, 18) and 1.74 m² (0.98, 2.03). Group 1 (n = 6) included patients with need for prolonged inotropes/pulmonary vasodilator use after CF-LVAD implantation and Group 2 (n = 6) included those without. Baseline demographic parameters, cardiopulmonary bypass time, and markers of RV afterload (pulmonary vascular resistance, PA compliance and elastance) were similar among the two groups. PAPi was significantly lower in group 1 compared to group 2 (0.96 vs 3.6, respectively; P = .004). Post-LVAD stay in the intensive care unit was longer for patients in group 1 (46 vs 23 days, P = .52). Brain natriuretic peptide was significantly higher at 3 months after implantation in group 1; P = .01.

Conclusions

The need for inotropes/pulmonary vasodilators in the postoperative period can be predicted by the preimplantation intrinsic RV contractile reserve as assessed by PAPi rather than the markers of RV afterload. Further investigation and correlation with clinical outcomes is needed.

Echocardiography vs cardiac magnetic resonance imaging assessment of the systemic right ventricle for patients with d‐transposition of the great arteries status post atrial switch

Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Objective

Patients with Dextro-transposition of the great arteries status post atrial switch (dTGA s/p atrial switch) are “at-risk” for systemic right ventricular (RV) dysfunction. Due to complex RV geometry, echocardiography (Echo) does not allow accurate determination of ejection fraction (EF), but cardiac magnetic resonance imaging (CMR) allows quantitative right ventricular assessment. Measures of ventricular deformation may be precursors to global ventricular dysfunction. The primary aim of this study was to characterize imaging and clinical findings for adult patients with dTGA s/p atrial switch.

Design

This was a retrospective cohort study of patients with dTGA s/p atrial switch operation (February 1966 to August 1988) with CMR performed at Children’s Hospital of Wisconsin (from September 2005 to May 2015). Eligible patients had clinic visit, Echo, and exercise stress test within 1 year of CMR.

Results

This study enrolled twenty-seven patients (16 males, 11 females) with dTGA s/p atrial switch (18 with Mustard operation and 9 with Senning operation; median age 30 years; 74% New York Heart Association class 1 and 26% class 2). Seventy-four percentage had normal RV systolic function (RV EF >45% by CMR). No correlation was observed between Echo strain data and clinical status (EF, exercise endurance, VO₂ max, or New York Heart Association class). Cardiac magnetic resonance imaging RV global circumferential strain GCS and RV EF had moderate negative correlation (r = −0.65, P < .001). Global circumferential strain was significantly different for those with RV EF above and below 45%, while global peak longitudinal strain (GLS) was not. Patients had reduced CMR myocardial strain values compared with healthy controls.

Conclusions

Reduced RV CMR GCS (for those with RV EF <45%) suggests that CMR evaluation may enhance early detection of detrimental changes in the systemic RV myocardium.

Baseline tubular biomarkers in young adults with congenital heart disease as compared to healthy young adults: Detecting subclinical kidney injury

Dana Y. Fuhrman, Lan Nguyen, Morgan Hindes, John A. Kellum — Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Background

There are significant implications for kidney disease in young adults with congenital heart disease. Prior investigations have not focused on the use of urinary tubular biomarkers for the early identification of kidney disease in this growing patient group.

Objective

Determine if young adults with congenital heart disease have differences in the baseline concentration of urinary tubular biomarkers when compared to healthy young adults.

Design/Methods

In a pilot case control study, 30 patients from 18 to 35 years of age with congenital heart disease and a normal serum creatinine were recruited during a routine follow-up visit. In the same age group, 30 control subjects without history of heart or kidney disease were recruited. Urine samples were obtained to measure beta 2-microglobin, alpha 1-microglobin, N-acetyl-B-D-glucosaminidase, liver fatty acid binding protein, kidney injury molecule-1, insulin-like growth factor binding protein 7, and tissue inhibitor of metalloproteinases-2. Comparisons were done using Wilcoxon rank-sum or Fisher’s exact test.

Results

No study participants had proteinuria on urine dipstick. Median concentrations of kidney injury molecule-1 were higher (P = .01) and concentrations of insulin-like growth factor binding protein 7 (P = .001) and tissue inhibitor of metalloproteinases-2 (P = .009) were lower in the subjects with congenital heart disease when compared to the control subjects. There were no significant differences between the groups with respect to the other biomarkers.

Conclusion

Our data suggest that young adults with congenital heart disease may have subclinical kidney dysfunction. Lower levels of insulin-like growth factor binding protein 7 and tissue inhibitor of metalloproteinases-2 may indicate an impaired ability to respond to injury, while higher levels of kidney injury molecule-1 may reflect early tubular injury.

Infundibular sparing versus transinfundibular approach to the repair of tetralogy of Fallot

Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Introduction

The right ventricular infundibular sparing approach (RVIS) to the repair of tetralogy of Fallot (TOF) avoids a full-thickness ventricular incision, typically utilized in the transinfundibular (TI) method.

Methods

We performed a retrospective, age-matched cohort study of patients who underwent RVIS at Texas Children’s Hospital or TI at Children’s Hospital Medical Center in Nebraska and subsequently underwent cardiac magnetic resonance imaging (CMR). We compared right ventricular end-diastolic and systolic volumes indexed to body surface area (RVEDVi and RVESVi) and right ventricular ejection fraction (RVEF) as primary endpoints. Secondary endpoints were indexed left ventricular diastolic and systolic volume (LVEDVi and LVESVi), left ventricular ejection fraction (LVEF), right ventricular (RV) sinus ejection fraction (EF) and RV outflow tract EF (RVOT EF).

Results

Seventy-nine patients were included in the analysis; 40 underwent RVIS and 39 underwent TI repair. None of the patients in the TI repair group had an initial palliation with a systemic to pulmonary arterial shunt compared to seven (18%) in the RVIS group (P < .01). There was no appreciable difference in RVEDVi (122 ± 29 cc/m² vs 130 ± 29 cc/m², P = .59) or pulmonary regurgitant fraction (40 ± 13 vs 37 ± 18, P = .29) between the RVIS and TI groups. Compared to the TI group, the RVIS group had higher RVEF (54 ± 6% vs 44 ± 9%, P < .01), lower RVESV (57 ± 17 cc/m² vs 67 ± 25 cc/m², P = .03), higher LVEF (61 ± 11% vs 54 ± 8%, P < .01), higher RVOT EF (47 ± 12% vs 41 ± 11%, P = .03), and higher RV sinus EF (56 ± 5% vs 49 ± 6%, P < .01)

Conclusions

In this selected cohort, patients who underwent RVIS repair for TOF had higher right and left ventricular ejection fraction compared to those who underwent TI repair.

The incidence of arrhythmias during exercise stress tests among children with Kawasaki disease: A single‐center case series

Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Objective

Based on 2017 guidelines, participation in competitive sports with prior history of Kawasaki Disease (KD) requires those with coronary artery aneurysms (CAA) z score ≥ 5 to undergo evaluation for evidence of inducible ischemia or arrhythmias. The use of exercise stress testing (EST) to evaluate arrhythmias among KD patients has never been reported. This retrospective single-center case series study sought to describe the presence of inducible arrhythmias during EST in KD patients with or without CAA.

Methods

Single-center retrospective review of medical records of patients diagnosed with KD between 1989-2015 at Texas Children’s Hospital, Houston, Texas who underwent EST were included.

Results

Among 1007 patients diagnosed with KD, 95 (9%) underwent 165 ESTs at a median time of 9.6 years (IQR 5.8-11.3 years) from diagnosis. Of these 95 patients, 37 had normal coronaries, 21 dilated (z score 2 to <2.5), 10 small (5 >z ≥2.5), 12 medium (10>z ≥ 5 absolute dimension <8 mm), 10 large (z ≥10 or absolute dimension ≥8 mm), 5 severe (myocardial infarct or bypass graft). Supraventricular tachycardia was not seen. Ventricular arrhythmias during EST were uncommon and seen only among patients with CAA z ≥5. Ventricular tachycardia occurred in a single patient with a large CAA, known VT and ICD. High-grade ventricular ectopy was seen in one patient who had severe CAA and underwent bypass grafting.

Conclusions

Arrhythmias on EST were noted only among patients with CAA z ≥5. The current guidelines are a reasonable approach to increasing healthy activity among KD patients. Clarification regarding which inducible arrhythmias meet criteria for activity restriction may be helpful to guide sport participation.

Trajectory of right ventricular indices is an early predictor of outcomes in hypoplastic left heart syndrome

Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Background

Children with hypoplastic left heart syndrome (HLHS) have risk for mortality and/or transplantation. Previous studies have associated right ventricular (RV) indices in a single echocardiogram with survival, but none have related serial measurements to outcomes. This study sought to determine whether the trajectory of RV indices in the first year of life was associated with transplant-free survival to stage 3 palliation (S3P).

Methods

HLHS patients at a single center who underwent stage 1 palliation (S1P) between 2000 and 2015 were reviewed. Echocardiographic indices of RV size and function were obtained before and following S1P and stage 2 palliation (S2P). The association between these indices and transplant-free survival to S3P was examined.

Results

There were 61 patients enrolled in the study with 51 undergoing S2P, 20 S3P, and 18 awaiting S3P. In the stage 1 perioperative period, indexed RV end-systolic area increased in patients who died or needed transplant following S2P, and changed little in those surviving to S3P (3.37 vs −0.04 cm²/m², P = .017). Increased indexed RV end-systolic area was associated with worse transplant-free survival. (OR = 0.815, P = .042). In the interstage period, indexed RV end-diastolic area increased less in those surviving to S3P (3.6 vs 9.2, P = .03).

Conclusion

Change in indexed RV end-systolic area through the stage 1 perioperative period was associated with transplant-free survival to S3P. Neither the prestage nor poststage 1 indexed RV end-systolic area was associated with transplant-free survival to S3P. Patients with death or transplant before S3P had a greater increase in indexed RV end-diastolic area during the interstage period. This suggests earlier serial changes in RV size which may provide prognostic information beyond RV indices in a single study.

Prevalence and risk factors for low bone density in adults with a Fontan circulation

Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Objective and Patients

This study aimed to characterize bone mineral density abnormalities and pathophysiological associations in young adults living with a Fontan circulation.

Design

Participants underwent bone mineral density measurement using dual-energy X-ray absorptiometry and serum biochemical analysis, cardiopulmonary exercise and strength testing and transthoracic echocardiography.

Results

In our cohort (n = 28), 29% had osteopenic-range bone mineral density and one patient was osteoporotic (average hip t score: −0.6 ± 1.1; spine t score: −0.6 ± 0.9). Four patients (14%) had z scores < −2.0. Parathyroid hormone levels were increased compared with laboratory median (6.1 ± 3.5 vs 4 pmol/L, P = .01) and 27% had 25-hydroxy-vitamin D < 50 nmol/L. 25-hydroxy-vitamin D negatively correlated with parathyroid hormone (ρ = −0.53, P = .01) suggesting secondary hyperparathyroidism. Atrioventricular valve systolic to diastolic duration ratio, an echocardiographic measure of diastolic dysfunction, inversely correlated with hip t and z scores (P < .01). Hip t scores were positively associated with oxygen saturations (ρ = 0.45, P = .05) and tended to be inversely associated with parathyroid hormone levels (ρ = −0.44, P = .07) and N-Terminal pro b-type natriuretic peptide (ρ = −0.42, P = .08).

Conclusions

Many young adults with a Fontan circulation have abnormal bone mineral density. The underlying pathophysiology is likely multifactorial. Possible contributors include secondary hyperparathyroidism, hypoxemia, diastolic cardiac dysfunction and neurohormonal activation. As low bone mineral density is clinically relevant and potentially treatable, assessment of bone mineral density should be part of routine care in this cohort.

Use of vasoactive agents in postoperative pediatric cardiac patients: Insights from a national database

Rohit S. Loomba, Saul Flores — Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Objective

The main goal of this study is to examine the variation in vasoactive agent prescription patterns across a large cohort of patients. In addition, we sought to determine the association between the number of vasoactive agents used during admissions and characteristics of admissions utilizing varying numbers of vasoactive agents.

Methods

This was a multi-institutional, cross-sectional study of the pediatric health information system database of patients who underwent congenital heart surgery and received vasoactive agents from 2004 to 2015. The international classification of disease-9 (ICD-9) codes were used to select admissions to those only pertaining to cardiac patients. The vasoactive agents investigated included epinephrine, norepinephrine, dopamine, dobutamine, milrinone, and vasopressin.

Results

A total of 43 441 postoperative pediatric cardiac admissions were identified and included in the final analyses. Of these, a majority used at least one vasoactive agent at some point during the admissions with the median being three vasoactives. Each vasoactive was utilized with decreasing frequency throughout the study period except for vasopressin which increased in frequency of use. After adjusting for multiple confounding factors, only milrinone was associated with decreased inpatient mortality in any postoperative subset, while the rest of the vasoactive were associated with increased inpatient mortality in some of the postoperative subsets.

Conclusion

Vasoactive agents have decreased in frequency of use in postoperative pediatric cardiac admissions, except for vasopressin. Only milrinone was found to be associated with decreased inpatient mortality in any subset of these patients, while all other vasoactive agents were found to be associated with increased inpatient mortality at least in one of the subsets.

Three‐dimensional rotational angiography in congenital heart disease: Present status and evolving future

Sok‐Leng Kang, Aimee Armstrong, Gregor Krings, Lee Benson — Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Three-dimensional rotational angiography (3D-RA) enables volumetric imaging through rotation of the C-arm of an angiographic system and real-time 3D reconstruction during cardiac catheterization procedures. In the field of congenital heart disease (CHD), 3D-RA has gained considerable traction, owing to its capability for enhanced visualization of spatial relationships in complex cardiac morphologies and real time image guidance in an intricate interventional environment. This review provides an overview of the current applications, strengths, and limitations of 3D-RA acquisition in the management of CHD and potential future directions. In addition, issues of dosimetry, radiation exposure, and optimization strategies will be reviewed. Further implementation of 3D-RA will be driven by patient benefits relative to existing 3D imaging capabilities and fusion techniques balanced against radiation exposure.

Coronary artery disease screening in adults with congenital heart disease prior to cardiac surgery

Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Objective

As adults with congenital heart disease (CHD) grow older, preoperative screening for coronary artery disease (CAD) may be indicated prior to CHD surgery. Data regarding the indications for preoperative CAD screening in this population are limited. Current practice is to follow guidelines for patients with valvular heart disease; however, the risk for CAD in certain congenital heart diagnoses may be higher than the general population. This study aimed to assess the results of preoperative CAD screening in patients prior to CHD surgery.

Design

Retrospective study.

Setting

Single tertiary center.

Patients

Patients ≥35 years that had CHD surgery from 1/1/2007 to 5/1/2017.

Outcome Measures

Data regarding CAD risk factors and preoperative CAD screening results were obtained. Prevalence and risk factors for CAD were analyzed, along with their relationship to perioperative outcomes.

Results

A total of 73 patients underwent CAD screening with either cardiac catheterization (56%) or computed tomography angiography (34%) prior to CHD surgery. Overall 16 (22%) patients were found to have CAD. Only two patients had severe coronary stenosis and underwent coronary bypass grafting at time of CHD surgery. Patients with CAD were more likely to be older and have history of hypertension, dyslipidemia, and tobacco smoking. CHD diagnosis was not significantly associated with presence of CAD.

Conclusion

CAD is common in asymptomatic older patients referred for screening prior to CHD surgery; however, severe CAD requiring concomitant coronary intervention is uncommon. Preoperative CAD screening should be based on age and traditional CAD risk factors, rather than underlying CHD.

Comprehensive left ventricular myocardial deformation assessment in children with Kawasaki disease

Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Objective

Children with Kawasaki disease (KD) with persistent coronary artery aneurysms (CAAs) can develop chronic vasculopathy and subsequent myocardial ischemia. Early detection of this process is challenging. Myocardial deformation analysis can detect early alterations in myocardial performance. We aim to determine whether there are differences in myocardial deformation between KD patients with and without CAAs.

Design

This is a cross-sectional study of 123 echocardiograms performed on 103 children with KD. Myocardial deformation was measured with two-dimensional speckle tracking (2DSTE). The echocardiograms were divided into groups according to the KD phase in which they were performed: acute, subacute, and convalescent/chronic. The convalescent/chronic phase group was then divided based on the presence or absence of CAAs. Left ventricular (LV) global longitudinal strain (GLS), global longitudinal strain rate (GLSSR), global circumferential strain (GCS), global circumferential systolic strain rate (GCSSR), peak torsion, and torsion rate were measured.

Results

The numbers of echocardiograms analyzed in each of the KD phase groups were: 31 acute, 25 subacute, and 67 convalescent/chronic. Myocardial deformation was within normal limits in all groups. However, GLSSR, GCSSR, peak torsion, and torsion rate were lower in the convalescent/chronic phase group than in the acute phase group (mean, −1.37 ± 0.24 vs −1.55 ± 0.21 1/s; −1.63 ± 0.27 vs −1.84 ± 0.35 1/s; 2.49 ± 1.13 vs 3.41 ± 2.60 °/cm, and 21.97 ± 8.36 vs 26.69 ± 10.86 °/cm/s; P < .05 for all). The convalescent/chronic phase subgroup with CAAs had lower GLSSR and GCSSR than the subgroup without CAAs (mean, −1.23 ± 0.22 vs −1.42 ± 0.22 1/s; −1.46 ± 0.25 vs −1.68 ± 0.26 1/s, P < .05 for both).

Conclusions

Children in the convalescent/chronic phase of KD have a subtle decrease in strain rate when compared to the acute phase, although within the normal range. This decrease is more pronounced in children with CAAs than those without CAAs. Longitudinal studies are needed to discern whether low-normal strain rate predicts decreased myocardial function in the long term.

Long‐term outcomes up to 25 years following balloon pulmonary valvuloplasty: A multicenter study

Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Objective

Evaluate long-term outcomes following balloon pulmonary valvuloplasty (BPV) for pulmonary stenosis (PS).

Background

Long-term data following BPV is limited to small, single center studies.

Methods

BPV from April 12, 1985 to January 7, 2015 from three centers were included. Outcomes studied were ≥ moderate PI by echocardiogram and residual PS ≥ 40 mm Hg. Risk factors for ≥ moderate PI, residual PS, and repeat intervention were assessed by univariate and multivariate analysis.

Results

Among 254 patients, mean age at BPV was 3.8 years (range 1 day-67 years), initial PS catheter gradient was 56 mm Hg (IQR 40-70), 19% had critical PS, and 9% had genetic syndromes. Mean follow-up duration was 7.5 years (maximum 25 years). Sixty-nine (29%) had ≥ moderate PI, 41 patients (17%) had residual PS > 40 mm Hg, and 31 (13%) had re-intervention. In univariate analysis, younger age, lower weight, greater initial PS gradient, greater initial RV/systemic pressure ratio, critical PS, and longer follow-up duration were associated with ≥ moderate PI. Greater initial PS gradient was associated with long-term residual PS or repeat intervention. In multivariate analysis, greater initial gradient and lower weight were independently associated with > moderate PI and greater initial PS gradient and genetic abnormality were independently associated with residual PS and repeat intervention.

Conclusion

Smaller patients with greater initial PS were more likely to develop significant long-term PI. Patients with greater initial PS and genetic abnormalities were more likely to have residual PS or require repeat intervention following BPV.

Exercise responses in children and adults with a Fontan circulation at simulated altitude

Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Background

Traveling to high altitude has become more popular. High-altitude exposure causes hypobaric hypoxia. Exposure to acute high altitude, during air travel or mountain stays, seems to be safe for most patients with congenital heart disorders (CHD). Still, current guidelines for CHD patients express concerns regarding safety of altitude exposure for patients with a Fontan circulation. Therefore, investigating hemodynamic and pulmonary responses of acute high-altitude exposure (±2500 m) at rest and during maximal exercise in patients with Fontan circulation can provide clarity in this dispute and may contribute to improvement of clinical counseling.

Methods

Twenty-one Fontan patients with 21 age-matched healthy controls, aged 8-40 years, were enrolled in an observational study. Participants performed two cardiopulmonary exercise tests on a cycle ergometer with breath-by-breath respiratory gas analyses combined with noninvasive impedance cardiac output measurements: one at sea level (±6 m) and one at simulated high altitude (±2500 m), respectively.

Results

The effect of altitude exposure was different in rest for saturation (−2.3% vs −4.1%) between Fontan patients and healthy controls (P < .05). At peak exercise the effects of high altitude exposure was different on VO₂ (−5.1% vs 9.6%) and AvO₂-diff (−0.3% vs −12.8%) between Fontan patients and healthy controls.

Conclusion

Although, acute high-altitude exposure has a detrimental effect on exercise capacity, the impact on pulmonary and hemodynamic responses of high-altitude exposure is comparable between Fontan patients and healthy controls.

Impact of durable ventricular assist devices on post‐transplant outcomes in adults with congenital heart disease

Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Background

There are no published data on post-transplant outcomes in durable ventricular assist device (VAD)-supported adult congenital heart disease (ACHD) patients.

Methods

We compared post-transplant outcomes in VAD-supported vs non-VAD-supported ACHD patients using the Scientific Registry of Transplant Recipients.

Results

At 1 year, there was no difference in post-transplant mortality between VAD-supported (12 patients) and non-VAD-supported (671 patients) ACHD patients.

Conclusions

In appropriate ACHD patients, VAD use as a bridge to transplant is a reasonable strategy.

Screening performance of congenital heart defects in first trimester using simple cardiac scan, nuchal translucency, abnormal ductus venosus blood flow and tricuspid regurgitation

Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Objective

The objective of this study was to analyze if the addition of simple cardiac scan in cases with increased nuchal translucency (NT) and/or abnormal ductus venosus (DV) blood flow, and/or tricuspid regurgitation (TCR) can improve detection of congenital heart defects (CHD) in chromosomally normal fetuses without non-cardiac defects at 11-13 + 6 gestational weeks in a population of singleton pregnancies.

Methods

During the 10 years period, all singleton pregnancies at 11-13 + 6 weeks were routinely scanned for NT, DV blood flow and TCR assessment and, if a single of these parameters was abnormal, simple cardiac scan with 2D gray scale and color and/or directional power Doppler in 4-chamber (4-CV) and 3 vessel and trachea views (3VTV) was performed.

Results

The sensitivity and specificity of NT ≥ 95th + DV R/A a-wave + TCR in detecting CHD were 77% and 97%, respectively, and of simple cardiac scan, 67% and 98%, respectively. Area under the curve of receiver operating characteristic curve of NT ≥ 95th + DV R/A a-wave + TCR was 0.838, and of NT ≥ 95th + DV R/A a-wave + TCR + simple cardiac scan was 0.915.

Conclusions

In chromosomally normal fetuses without non-cardiac anomalies, addition of simple cardiac scan to the combined first trimester screening parameters improves detection of major CHD during first trimester.

Kidney injury biomarkers after cardiac angiography in children with congenital heart disease

Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Objective

This study aims to investigate the changes in renal function and levels of urinary biomarkers before and after cardiac angiography in children with congenital heart disease (CHD).

Setting

Children with CHD are at a risk for kidney injury during contrast exposure in cardiac angiography.

Outcome Measures

We measured urinary protein, albumin, N-acetyl-β-D-glucosaminidase (NAG), β2-microglobulin (BMG), and liver-type fatty acid-binding protein (L-FABP) levels, as well as serum creatinine and cystatin C levels, before and after cardiac angiography in 33 children with CHD.

Results

No significant decrease was noted in either the creatinine-based or cystatin C-based estimated glomerular filtration rate at 24 hours after angiography compared with that before angiography. Urinary protein, NAG, BMG, and L-FABP levels were significantly increased at 24 hours after angiography, all of which returned to baseline levels at more than 7 days after angiography. An increase in urinary level of protein, albumin, NAG, or BMG was mostly associated with increased urinary L-FABP level. An increase in both urinary BMG and L-FABP, but not that in urinary L-FABP alone, was associated with increased levels of urinary protein and NAG, as well as the greater dose of contrast media.

Conclusions

Transient increases of kidney injury biomarkers following cardiac angiography are not necessarily associated with the impairment of renal function in a short time period; however, the increase in urinary protein, albumin, NAG, or BMG level may indicate greater stresses to the kidneys than the increase in urinary L-FABP alone in children with CHD.

Left cardiac sympathetic denervation in the management of long QT syndrome and catecholaminergic polymorphic ventricular tachycardia: A meta‐regression

Alessandro Sgrò, Thomas M. Drake, Pedro Lopez‐Ayala, Kevin Phan — Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Background

Left cardiac sympathetic denervation (LCSD) has been proposed as useful therapy for long QT syndrome (LQTS) and catecholaminergic polymorphic ventricular tachycardia (CPVT), in addition to anti-arrhythmic agents and implantable cardioverter defibrillators. This study aimed to assess the current evidence for LCSD and compare the open vs the video-assisted thoracoscopic surgery (VATS) approaches.

Methods

MEDLINE, Embase and Cochrane library databases were searched up to December 2018 for studies reporting the long-term outcomes of LCSD in LQTS, CPVT patients. The incidence of cardiac events (CEs) before and after surgery, the change in QTc interval, and surgical complications were pooled to estimate the efficacy of LCSD. Meta-regression was used to estimate the effects of surgical approach (open vs VATS) on outcomes following LCSD.

Results

Twenty-seven papers met our inclusion criteria (647 patients). VATS was used in 408 patients (63.1%), open surgery in 239 (36.9%). Mean follow-up was 32.3 ± 32.5 months. Postsurgery, 398/585 patients (68.0%) were free of CEs and QTc decreased from 522 ± 61.6 ms to 494 ± 52.3 ms. Meta-regression showed no differences between the two approaches in the incidence of CEs and surgical complications. VATS was associated with a smaller reduction in QTc (β-coefficient −20.04, 95% CI −36.82 to −3.27, P = .019).

Conclusions

LCSD was associated with a reduction in the incidence of CEs in LQTS, CPVT patients and in the duration of QTc. Open surgery was associated with a greater reduction in QTc. Due to the limitations that hindered our study, a randomized trial is warranted to fully establish LCSD safety and efficacy.

Atrial fibrillation in adults with congenital heart disease following cardiac surgery in a single center: Analysis of incidence and risk factors

Michael A. Brock, John‐Anthony Coppola, Jana Reid, Diego Moguillansky — Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Objective

The primary aim of our work is to determine the incidence of atrial fibrillation following cardiac surgery in adults with congenital heart disease. Secondary aims include identifying risk factors predictive of developing early postoperative atrial fibrillation and morbidities associated with early postoperative atrial fibrillation.

Design

Retrospective analysis.

Setting

Single center, quaternary care children’s hospital.

Patients

This review included patients at least 18 years of age with known congenital heart disease who underwent cardiac surgery requiring a median sternotomy at our congenital heart center from January 1, 2012 to December 31, 2016.

Interventions

None.

Outcome Measures

The primary outcome was early postoperative atrial fibrillation. Secondary outcomes included preoperative comorbidities, preoperative echocardiographic findings, operative details, and postoperative morbidities, such length of stay, reintubation, stroke, and death.

Results

The incidence of early postoperative atrial fibrillation was 21%. Those who developed early postoperative atrial fibrillation were older (50 years vs 38 years, P =< .001), had a history of atrial fibrillation prior to surgery, had preoperative pulmonary hypertension, and had longer cardiopulmonary bypass times (103 minutes vs 84 minutes, P = .025) when compared to those who did not develop postoperative atrial fibrillation. Multivariate analysis identified age greater than 60, preoperative pulmonary hypertension, mitral valve intervention, and the need for postoperative inotropic support as being independent predictors of postoperative atrial fibrillation. Those who developed postoperative atrial fibrillation remained in the hospital longer (9 days vs 7 days, P =< .001).

Conclusions

Atrial fibrillation is a common complication following cardiac surgery in adults with congenital heart disease. Age, preoperative comorbidities, type of surgical intervention, and the need for perioperative inotropic infusions may predict the risk of atrial fibrillation in this unique patient population.

Parenting stress trajectories during infancy in infants with congenital heart disease: Comparison of single‐ventricle and biventricular heart physiology

Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Objective

Parents of infants with congenital heart disease (CHD) experience increased parenting stress levels, potentially interfering with parenting practices and bear adverse family outcomes. Condition severity has been linked to parenting stress. The current study aimed to explore parenting stress trajectories over infancy in parents of infants with complex CHD, and to compare them by post-operative cardiac physiology.

Design

Data from a larger prospective cohort study was analyzed using longitudinal mixed-effects regression modeling.

Setting

Cardiac intensive care unit and outpatient clinic of a 480-bed children's hospital in the American North-Atlantic region.

Participants

Parents of infants with complex CHD (n = 90).

Measures

Parenting stress was measured via the parenting stress index-long form over four time points during infancy.

Results

Parents of infants with a single-ventricle heart experienced a decrease in total stress over time. Parents of infants with a biventricular heart experienced a decrease in attachment-related stress, and an increase in stress related to infant temperament over time. Parenting stress trajectories over time significantly differed between groups on infant temperamental subscales.

Conclusions

Findings highlight stressful and potentially risky periods for parents of infants with complex CHD, and introduce additional illness-related and psychosocial/familial aspects to the parenting stress concept. Early intervention may promote parental adaptive coping and productive parenting practices in this population.

Concomitant pulmonary vein isolation and percutaneous closure of atrial septal defects: A pilot project

Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Background

Patients with an atrial septal defect (ASD) are at increased risk of developing atrial fibrillation (AF). Currently percutaneous ASD closure is the preferred therapeutic strategy and although pulmonary vein isolation (PVI) for AF is feasible after ASD closure, the transseptal puncture can be technically challenging and probably increases the perioperative risk. A staged approach, with PVI several months before ASD closure, has been recommended for patients already scheduled for closure, but no data are available on combined procedures.

Purpose

This pilot study evaluates the feasibility of a combined procedure of PVI and ASD closure in patients with a hemodynamic important ASD and documented AF.

Methods

In one procedure, PVI was performed prior to placement of the ASD closure device. Transseptal access for PVI was obtained via wire passage through the ASD in all patients. Patients were followed with 5-day-holter monitoring at 3, 6, and 12 months. Recurrence of AF was defined as a documented, symptomatic episode of AF.

Results

The study population consisted of five patients (four females, mean age: 58 (±3) years). Acute PVI was achieved in all patients. Only one patient had a small residual ASD after closure. Besides a small groin hematoma in two patients, no complications occurred. After 12-month follow-up, three patients were free of AF recurrence (60%).

Conclusion

This study shows that a combined PVI with ASD closure is feasible with an acceptable success rate of AF free survival. These preliminary results in a small patient group warrants a larger trial.

Transfusion‐related acute hepatic injury following postoperative platelets administration in pediatric patients undergoing the Fontan procedure

Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Objective

The final common pathway of single ventricle patients is the Fontan procedure. Among the immediate postoperative complications is acute hepatic injury presented by marked elevation of liver enzymes (alanine transaminase [ALT] and aspartate transaminase [AST]). We aimed to determine the contribution of blood products transfusion to acute hepatic injury.

Design

Single center retrospective cohort study.

Setting

Pediatric Cardiac Intensive Care Unit at a tertiary medical center.

Patients

Ninety-nine pediatric patients undergoing the Fontan procedure between January 2009 and December 2016.

Interventions

None.

Measurements and Main Results

Out of the four types of blood products, transfusion of platelets was found to significantly affect postoperative levels of ALT and AST. Additional factors included postoperative administration of sodium bicarbonate, decreased flow through the Fontan canal and decreased urine output. Preoperative pulmonary artery pressure and pulmonary vascular resistance, cardiopulmonary bypass time, aortic cross-clamp time, amount of postoperative bleeding, and vasoactive-inotropic score did not influence liver enzymes levels

Conclusions

In pediatric Fontan patients, platelets transfusions contribute to an acute hepatic injury. The relation between platelets and transfusion-related acute lung injury (TRALI) has been well described, but this is the first time it is being described in regard to acute hepatic injury (TRAHI). Changing platelet transfusion strategy could decrease morbidity in Fontan patients but further research is needed.

Impact of feeding mode on neurodevelopmental outcome in infants and children with congenital heart disease

Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Objective

To investigate the impact of feeding mode on neurodevelopmental outcomes in children with congenital heart defects.

Design

A retrospective cohort study of 208 children with congenital heart disease (CHD), who had surgery from 1 January 2013 until 31 December 2016 at Texas Children’s Hospital, Houston, TX, US.

Settings

University Hospital, Developmental Outcome Clinic.

Outcomes measures

Standardized cognitive scores were assessed with Capute Scales and motor development with Revised Gesell Developmental Schedules. We analyzed anthropometrics, mode of feeding, surgical complexity, syndrome, and gender as predictors of developmental outcomes at four time points: hospital discharge, and 6, 12, and 24 months of age.

Results

Mode of feeding is associated with neurodevelopmental outcome in children with CHD. Children on enteral feeding tubes had significantly lower developmental quotient (DQ) scores in cognition, communication, and motor function at 12 and 24 months compared to orally fed children. There were greater proportions of developmental delays (DQ < 70) in enteral tube fed children at the 6, 12, and 24 months visits. Further, there was a strong association between presence of enteral feeding tube, syndrome, and developmental outcome. Greater surgical complexity, weight gain and ethnicity were not associated with the developmental outcomes.

Conclusions

Our findings suggest that the presence of an enteral feeding tube following corrective congenital heart surgery are at increased risk of neurodevelopmental delays at 12 and 24 months.

Arteriovenous fistula creation for hypoxia after single ventricle palliation: A single‐institution experience and literature review

Andrew D. Spearman, Steven J. Kindel, Ronald K. Woods, Salil Ginde — Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Background

Hypoxia is a common and sometimes severe morbidity of single ventricle congenital heart disease (CHD). Creation of an arteriovenous fistula (AVF) is occasionally performed for patients after superior or total cavopulmonary connection (SCPC or TCPC) in an attempt to improve oxygen saturations. Despite previous reports, AVF creation is a rare palliation with inadequately defined benefits and risks. We sought to determine changes in peripheral oxygen saturation (SpO₂) and risk of adverse event after AVF creation in children with single ventricle CHD at our institution.

Methods

We conducted a retrospective chart review of patients with a history of single ventricle palliation and history of surgical AVF creation who were seen at our tertiary care center from 1996 to 2017.

Results

A total of seven patients were included in our study. SpO₂ for the overall cohort did not significantly increase after AVF creation (pre-AVF 79.1 ± 6.9%, post-AVF 82.7 ± 6.0% [P = .23]). SpO₂ trended up for large shunts (>5 mm) (pre-AVF 75.0 ± 7.6%, post-AVF 84.0 ± 5.3% [P = .25]). SpO₂ did not improve for small shunts (≤5 mm) (pre-AVF 82.3 ± 6.5%, post-AVF 81.0 ± 8.5% [P = .50]). The 12-month overall and transplant-free survival were 85.7% and 71.4%, respectively. Freedom from AVF-related complication (cephalic edema, thrombotic occlusion) was 51.4% at 12 months.

Conclusion

Palliative AVF creation for patients with single ventricle CHD and hypoxia does not universally improve SpO₂ and is prone to early complications. Despite a lack of durable benefit and known risks, AVF creation remains a reasonable palliation for a subset of patients after SCPC who are not candidates for TCPC, or potentially as a bridge to heart transplantation.

Short‐term results in infants with multiple left heart obstructive lesions

Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Objective

Deciding on a surgical pathway for neonates with ≥2 left heart obstructive lesions is complex. Predictors of the successful biventricular (2V) repair in these patients are poorly defined. The goal of our study was to identify patients who underwent the 2V repair and assess anatomic and echocardiographic predictors of success.

Design

Infants born between July 2015 and August 2017 with ≥2 left heart obstructive lesions with no prior interventions were identified (n = 19). Patients with aortic or mitral valve (MV) atresia and critical aortic stenosis were excluded. Initial echocardiograms were reviewed for aortic, MV, tricuspid valve annulus size, and left (LV) and right (RV) ventricle diastolic longitudinal dimensions. The valve morphology and presence of a ventricular septal defect (VSD) and coarctation were assessed. Clinical outcomes included successful 2V repair, complications, and repeat interventions or surgeries. Failed 2V repair was defined as a takedown to single ventricle (1V) physiology, cardiac transplantation, or death.

Results

For 2V repair, 14/19 patients were selected and for 1V, 5/19 patients were selected. Initial surgical procedures of the 2V group were simple coarctation repair (5), complex coarctation/arch reconstruction +/− septal defect closure (6), hybrid stage 1 (2), and none (1). Three of the 2V patients required reintervention in the first 90 days. The LV to RV diastolic longitudinal ratio >0.75 and mitral/tricuspid ratio of <0.8 were observed in 13/14 of the 2V patients. The LV:RV ratio and the aortic valve z score were significantly larger in the 2V group compared to the 1V group. All patients in the 1V group had a nonapex forming LV. There was no mortality with follow-up to three years of age.

Conclusions

This study showed excellent short-term and midterm surgical results in the 2V population. The LV:RV diastolic longitudinal ratio may be a useful tool in the risk stratification of a successful 2V repair even in cases with a small MV.

Cerebrovascular accidents in Ebstein’s anomaly

Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Introduction

Mechanisms and risk factors for cerebrovascular accidents (CVAs) in Ebstein’s anomaly (EA) are not well understood; hence, we aimed to clarify these in a large cohort of EA patients.

Methods

Patients with a confirmed diagnosis of EA were retrospectively reviewed. Baseline characteristics were compared between patients with and without a prior history of CVA using logistic regression modeling. Cox regression analysis was used to identify predictors of CVA following initial evaluation. CVA incidence from birth and following tricuspid valve surgery were estimated using the Kaplan-Meier method.

Results

Nine hundred sixty-eight patients (median age 21.1 years, 41.5% male) were included, in which, 87 patients (9.0%) had a history of CVA (54 strokes, 33 transient ischemic attacks; 5 associated with brain abscesses) prior to their initial evaluation. The odds of atrial septal defect/patent foramen ovale (odds ratio [OR] 4.91; 95% CI 2.60-21.22; p = .0002) and migraines/headaches (OR 2.38; 95% CI 1.40-4.04; p = .0013) but not atrial arrhythmias (OR 0.75; 95% CI 0.44-1.30; p = .31) were significantly higher among patients with prior CVA following multivariable adjustment. Seventeen patients experienced CVA following initial evaluation; no examined variables including atrial arrhythmias (HR 2.38; 0.91-6.19; p = .076) were predictive of CVA risk. The 10-year, 50-year, and 70-year incidences of CVA were 1.4%, 15.9%, and 23.5%, respectively, with paradoxical embolism heavily implicated.

Conclusion

Patients with EA are at substantive risk for CVA. Histories of migraines/headaches and interatrial shunts should prompt concern for paradoxical embolic CVAs. This has significant implications for all patients with atrial-level shunting.

Longitudinal growth in patients with single ventricle cardiac disease receiving tube‐assisted feeds

Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Objective

Children with single ventricle cardiac disease (SVCD) have poor growth in early life. Tube-assisted feeding (TF) is used to improve weight gain, but its impact on long-term growth remains unknown. We sought to compare the longitudinal growth of SVCD patients receiving TF after initial cardiac surgery with those fed entirely by mouth.

Design

We conducted a retrospective cohort study of SVCD patients who underwent initial surgical palliation between 1999 and 2009. We defined TF as the use of nasogastric, gastrostomy, or jejunostomy TF. We compared maximal attained growth z-scores for each year of life between TF and non-TF patients. A secondary analysis compared surgical and clinical factors between groups.

Results

A total of 134 patients were included; 64% were male and 68% underwent the Norwood operation. One third of patients (44) received TF. Adjusting for age, TF patients had an average of 0.56 lower weight-for-age z-score (WAZ) than non-TF patients (P = 0.007) through the age of 6 years. Longitudinal height was not affected by TF status (P = 0.15). In a subanalysis of Norwood patients, TF patients had lower WAZ at initial hospital discharge despite longer LOS. TF patients had diminished WAZ after adjusting for complications, interstage hospitalizations, and timing of subsequent operations.

Conclusions

In this single-center study, patients with SVCD requiring TF at discharge from initial surgical palliation had diminished WAZ at discharge and on long-term follow-up, despite controlling for other identifiable risk factors. Further investigation is needed to understand the mechanisms underlying this phenomenon and to risk stratify infants who go home on TF.

Immediate and short‐term effects of transcatheter device closure of large atrial septal defect in senior people

Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Objectives

We sought to evaluate the safety and efficacy in improving cardiac function and functional capacity with device closure of large atrial septal defects (ASD) in senior adults.

Background

Atrial septal defect accounts for about 10% of all congenital heart disease. It still remains unclear whether large ASD closure in senior people should be performed or not. Hence we aim to prospectively assess the safety and clinical status of senior patients after transcatheter closure in large ASD.

Patients and interventions

This was a prospective study of all patients aged over 50 years who underwent device closure of a secundum large ASD between January 2013 and January 2018. Investigations including brain natriuretic peptide level, electrocardiography, chest X-ray, transthoracic echocardiogram, transesophageal echocardiogram, and 6-minute walk test were performed before and at 2 days and 4 weeks and 6 months after the procedure.

Results

Twenty patients (median age 68 years, 10 women) had transcatheter device closure of large ASD successfully. Median ASD size was 32 mm (range 30-39 mm). Median pulmonary artery pressure was 58 mm Hg (range 47-67 mm Hg). At 6 months, there was a significant change in right atrium size (P < .001) and right ventricle size (P < .01) and left ventricle size (P < .001) and also pulmonary artery pressure (P < .0001), New York Heart Association functional class improved (P = .03) in 19 patients and also significant improvement in 6-minute walk test distance (P < .001). There were no major complications.

Conclusions

Our data showed that large ASD closure at senior people results in satisfactory cardiac remodeling and cardiac function improvement.

Metabolic syndrome in adults with congenital heart disease and increased intima‐media thickness

Anna‐Luisa Häcker, Renate Oberhoffer, Alfred Hager, Peter Ewert, Jan Müller — Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Aims

Age-related cardiovascular diseases are a relevant risk in the aging population of adults with congenital heart diseases (ACHD). Risk factors such as the metabolic syndrome (MetS) impact the risk of increased carotid intima-media thickness (cIMT) and thereby long-term cardiovascular diseases. The aim of the study was to assess MetS in ACHD and outline a possible association to cIMT.

Methods and Results

In total, 512 ACHD (43.0 ± 9.6 years, 48.9% female) were screened for MetS by the standards of the International Diabetes Federation, and their cIMT by ultrasound from January 2017 to June 2019. MetS was prevalent in 72 (14.1%) of the ACHD population (34 female, 15.5%). Regarding severity class, patients with simple forms of CHD had a MetS prevalence of 11.8%, moderate 16.7%, and severe 13.8%. ACHD with MetS had significantly increased cIMT compared to ACHD without MetS (ACHD with MetS: 0.587 ± 0.079 mm, ACHD without MetS: 0.560 ± 0.087 mm, mean difference: 0.028 mm, P = .013). Such a difference in vascular structure corresponds to roughly five years of normal vascular aging of the vessels.

Conclusion

ACHD with MetS have a thicker cIMT compared to ACHD without MetS. Screening for MetS and targeting risk factors in ACHD might help to prevent structural alterations of the vessels at an early stage.

Risk stratification models for congenital heart surgery in children: Comparative single‐center study

Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Objective

Three scores have been proposed to stratify the risk of mortality for each cardiac surgical procedure: The RACHS-1, the Aristotle Basic Complexity (ABC), and the STS-EACTS complexity scoring model. The aim was to compare the ability to predict mortality and morbidity of the three scores applied to a specific population.

Design

Retrospective, descriptive study.

Setting

Pediatric and neonatal intensive care units in a referral hospital.

Patients

Children under 18 years admitted to the intensive care unit after surgery.

Interventions

None.

Outcome measures

Demographic, clinical, and surgical data were assessed. Morbidity was considered as prolonged length of stay (LOS > 75 percentile), high respiratory (>72 hours of mechanical ventilation), and high hemodynamic support (inotropic support >20).

Results

One thousand and thirty-seven patients were included, in which 205 were newborns (18%). The category 2 was the most frequent in the three scores: In RACHS-1, ABC, 44.9%, and STS-EACTS, 40.8%. Newborns presented significant higher categories. Children required cardiopulmonary bypass in more occasions (P < .001) but the times of bypass and aortic cross-clamp were significantly higher in newborns (P < .001 and P = .016). Thirty-two patients died (2.8%). A quarter of patients had a prolonged LOS, 17%, a high respiratory support, and 7.1%, a high hemodynamic support. RACHS-1 (AUC 0.760) and STS-EACTS (AUC 0.763) were more powerful for predicting mortality and STS-EACTS for predicting prolonged LOS (AUC 0.733) and the need for high respiratory support (AUC 0.742).

Conclusions

STS-EACTS seems to stratify better risk of mortality, prolonged LOS, and need for respiratory support after surgery.

Progressive loss of bone mass in children with Fontan circulation

Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Objective

We investigated bone mineral density (BMD) at different ages after the Fontan completion, and we evaluated the relationship between BMD, vitamin D levels, and pertinent patient variables.

Methods

A cross-sectional sample of 64 patients was examined with dual-energy X-ray absorptiometry (DXA) scans to determine BMD. Of these patients, 24 were also examined with BoneXpert software to determine bone mass density (BMX), expressed as the bone health index (BHI). Blood samples from all patients were analyzed. Patients were divided into three different age groups; A: 4-9 years old (n = 22), B: 10-15 years old (n = 21), and C: 16-18 years old (n = 21).

Results

Overall, BMD z scores were (mean ± SD): −1.0 ± 1.3 for the lumbar spine and −0.2 ± 1.2 for the total body. Groups B and C had significantly lower z score values compared to group A. Of patients in group C, 35% had z score values ≤−2 SD of the mean of the healthy population. There was no difference related to systemic ventricular anatomy (left or right); however, patients with lateral tunnels had lower BMD than patients with extra cardiac conduits. Overall, the BHI z score was (mean ± SD): −1.2 ± 0.9, but low BMX did not correlate with low BMD. The 25-hydroxy vitamin D level was 58 ± 30 nmol/L. Vitamin D levels decreased with age: in group C, 33.3% of patients exhibited vitamin D deficiencies. Vitamin D levels were not correlated with bone mineral densities.

Conclusion

BMD levels decreased with age in patients with Fontan circulation. Different bone components were involved. Vitamin D levels also decreased with age, but they were not consistently associated with bone mineral densities. The single factor most predictive of low BMD was a lateral tunnel Fontan, compared to an extra cardiac Fontan.

Assessing hepatic impairment in Fontan‐associated liver disease using the HepQuant SHUNT test

Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Background & Aims

Fontan surgery for single ventricle congenital heart disease leads to Fontan-associated liver disease (FALD). Typical laboratory tests, imaging, and histopathology cannot predict clinical severity in FALD. HepQuant SHUNT is a proprietary serum test of hepatic function and physiology that has not yet been evaluated in FALD.

Methods

Fourteen adult FALD patients at a single urban tertiary care center who underwent a Fontan procedure in childhood received HepQuant SHUNT testing between September 2015 and April 2018. The HepQuant SHUNT disease severity index (DSI) assesses global liver function and physiology from systemic and portal hepatic filtration rates (HFRs, clearances adjusted for body mass) of orally and intravenously administered cholates labeled with deuterium or 13C. The SHUNT parameter of the test measures portal systemic shunting from the ratio of Systemic HFR to Portal HFR. Chart review included laboratory tests, imaging, and clinical findings. Data from FALD patients were compared with data from healthy controls.

Results

The average DSI and SHUNT values for the FALD patients were 17.5% and 36.1%, respectively, compared to 9.2% and 24.1%, respectively, for controls. Twelve (85.7%) FALD patients had a DSI >15 (upper limit of normal). Seven (50.0%) FALD patients had SHUNT values >30% (upper limit of normal), while three FALD patients (21.4%) had SHUNT values >49%. One FALD patient with preoperative SHUNT of 69%, who underwent a combined heart-liver transplant, had confirmed cirrhotic morphology within the liver explant.

Conclusions

This pilot study demonstrated that most FALD patients had hepatic impairment detected by abnormal DSI, with a smaller number having markedly elevated SHUNT values >49% suggesting intrinsic liver disease. The HepQuant SHUNT test may be useful in detecting and quantifying liver disease severity in FALD patients.

Improvement in ventricular function with rhythm control of atrial arrhythmias may delay the need for atrioventricular valve surgery in adults with congenital heart disease

Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Objective

Atrial arrhythmias and atrioventricular valve regurgitation (AVVR) are common causes of morbidity among adults with congenital heart disease (ACHD). The impact of rhythm control on AVVR in this population is unknown. We sought to determine whether a rhythm control strategy is associated with greater freedom from AV valve surgery than a rate control strategy.

Design

Patients evaluated by both ACHD and electrophysiology specialists at a single academic center were screened for atrial arrhythmias and at least moderate-severe AVVR. Clinical and electrographic data were abstracted. All echocardiograms were interpreted by a single echocardiographer blinded to treatment strategy. Patients were followed until AV valve surgery, heart transplantation, death, or last clinical follow-up.

Results

Rhythm control was attempted in 9 of 24 identified patients. Among these nine patients, arrhythmias were eliminated in three and reduced from persistent to paroxysmal in another three. In the rhythm control group, mean left ventricular ejection fraction improved from 54.4 ± 12.4% to 60.0 ± 11.5% (P = .02) and mean right ventricular systolic function increased nearly one grade (P = .02). AVVR did not decrease significantly. No significant change in left or right ventricular systolic function, or AVVR was observed among the 15 patients treated with rate control. Four-year survival free of AV valve operation and heart transplant was 88% in the rhythm control group and 31% in the rate control group (P = .04).

Conclusions

In ACHD patients with atrial arrhythmias and at least moderate-severe AVVR, a rhythm control strategy was associated with improved biventricular systolic function. This improvement in ventricular function and symptoms may allow valve surgery to be deferred.

Surveillance and screening practices of New England congenital cardiologists for patients after the Fontan operation

Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Introduction

Surveillance and management guidelines for Fontan patients are lacking due to the paucity of evidence in the literature of screening efficacy on outcome measures.

Methods

The Fontan Working Group within the New England Congenital Cardiology Association designed an electronic survey to assess surveillance practices for patients with Fontan procedures among New England congenital cardiologists and to explore variability in screening low-risk vs high-risk Fontan patients across regional programs.

Results

Fifty-six cardiologists representing 12 regional programs responded to the survey, comprising ~40% of the total New England congenital cardiac physicians. The majority of desired testing and consultation was available within 50 miles of the patient's home institution with some limitations of cardiac catheterization and cardiac magnetic resonance imaging availability. Surveillance and screening were less frequent in low-risk Fontan patients compared to high-risk Fontan patients. Counseling practices were similar for both low-risk and high-risk Fontan patients. Aspirin monotherapy was recommended by 82% of providers for low-risk Fontan patients, while anticoagulation regimens were more varied for the high-risk population. Practitioners with ≤15 years of experience were more likely to provide quality of life testing in both low-risk and high-risk Fontan patients. There were no other major differences in testing frequencies by years of practice, quaternary vs nonquaternary care facility, or the number of Fontan patients in a practice.

Conclusion

This survey provides insight into regional practices of screening and surveillance of Fontan patients. These data may be used to design future research studies and evidence-based guidelines to streamline the approach to manage these complex patients.

Outcomes in adults with congenital heart disease and heterotaxy syndrome: A single‐center experience

Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Background

Heterotaxy syndrome (HS) is a condition in which the thoracoabdominal organs demonstrate an abnormal lateral arrangement and is often associated with congenital heart disease (CHD). Little is known about the adult HS population with CHD.

Objective

To describe the outcomes and sociodemographics of the adult CHD population with HS.

Methods

Records of patients 18 years of age or older with diagnoses of both CHD and HS at Texas Children's Hospital from 1964 to 2018 were reviewed.

Results

Sixty-two patients met inclusion criteria. Median age was 22.7 [IQR 19.6-30.0] years; 26 (42%) were female; and 13 (21%) of patients had a gap in care of >3 years. Median follow-up time in adulthood was 2.9 [IQR 1.3-8.2] years. Forty-three (69%) of patients had single ventricle heart disease, 31 (71%) of whom completed Fontan circulation. A total of 36 interventions occurred in 24 patients which included 16 cardiac catherization interventions, 13 electrophysiology-related procedures, and 18 surgical procedures including 2 orthotopic heart transplants. The median age for death or heart transplant was 45.3 (95%CI 34.3-56.1) years. Heart failure-free survival was 80.8 ± 5.2%, 58.7 ± 11.0%, and 31.1 ± 15.7% at 20, 30, and 40 years old, respectively. Cerebrovascular accident-free survival was 84.3 ± 5.1%, 54.2 ± 11.3%, and 40.6 ± 14.5% at 20, 30, and 40 years old, respectively. Tachyarrhythmia-free survival was 54.0 ± 7.1%, 29.2 ± 8.3%, and 19.5 ± 9.7% at 20, 30, and 40 years old and bradyarrhythmia-free survival was 66.0 ± 6.3%, 41.7 ± 9.4%, and 33.4 ± 10.6% at ages 20, 30, and 40 years, respectively.

Conclusions

At a tertiary referral center, adult patients with CHD and HS have high rates of comorbidities and early death or heart transplant. Longitudinal surveillance and further exploration into factors associated with improved survival in this population are warranted.

Prolonged Tpeak‐Tend interval is a risk factor for sudden cardiac death in adults with congenital heart disease

Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Objective

Adult congenital heart disease (ACHD) patients are at risk of sudden cardiac death (SCD). However, methods for risk stratification are not yet well-defined. The T_peak-T_end (TpTe) interval, a measure of dispersion of ventricular repolarization, is a risk factor for SCD in non-ACHD patients. We aim to evaluate whether TpTe can be used in risk stratification for SCD in ACHD patients.

Design

From an international multicenter cohort of 25 790 ACHD patients, we identified all SCD cases. Cases were matched to controls by age, gender, congenital defect, and (surgical) intervention.

Outcome Measures

TpTe was measured on a standard 12-lead ECG. The maximum TpTe of all ECG leads (TpTe-max), mean (TpTe-mean), and TpTe dispersion (maximum minus minimum) were obtained. Odds ratios (OR) for SCD cases vs controls were calculated using conditional logistic regression analysis.

Results

ECGs were available for 147 cases (median age at death 33.5 years (quartiles 26.2, 48.7), 66% male) and 267 controls. The mean TpTe-max was 97 ± 24 ms in cases vs 84 ± 17 ms in controls (P < .001); TpTe-mean was 70 ± 16 vs 63 ± 10 ms (P < .001); and dispersion was 51 ± 22 ms vs 41 ± 16 ms (P = .02), respectively. Assessing each ECG lead separately, TpTe in lead aVR predicted SCD most accurately. TpTe in lead aVR was 71 ± 23 ms in cases vs 61 ± 13 ms in controls (P < .001). After adjusting for impaired ventricular function, heart failure symptoms, and prolonged QRS duration, the OR of SCD of TpTe in lead aVR at an optimal cutoff of 80 ms was 5.8 (95% CI 2.7-12.4, P < .001).

Conclusions

The TpTe interval is associated with SCD in ACHD patients. Particularly, TpTe in lead aVR can be used as an independent risk factor for SCD in ACHD patients and may, therefore, add precision to current risk prediction models.

Utilization of inhaled nitric oxide after surgical repair of truncus arteriosus: A multicenter analysis

Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Background

Elevated pulmonary vascular resistance (PVR) is common following repair of truncus arteriosus. Inhaled nitric oxide (iNO) is an effective yet costly therapy that is frequently implemented postoperatively to manage elevated PVR.

Objectives

We aimed to describe practice patterns of iNO use in a multicenter cohort of patients who underwent repair of truncus arteriosus, a lesion in which recovery is often complicated by elevated PVR. We also sought to identify patient and center factors that were more commonly associated with the use of iNO in the postoperative period.

Design

Retrospective cohort study.

Setting

15 tertiary care pediatric referral centers.

Patients

All infants who underwent definitive repair of truncus arteriosus without aortic arch obstruction between 2009 and 2016.

Interventions

Descriptive statistics were used to demonstrate practice patterns of iNO use. Bivariate comparisons of characteristics of patients who did and did not receive iNO were performed, followed by multivariable mixed logistic regression analysis using backward elimination to identify independent predictors of iNO use.

Main Results

We reviewed 216 patients who met inclusion criteria, of which 102 (46%) received iNO in the postoperative period: 69 (68%) had iNO started in the operating room and 33 (32%) had iNO initiated in the ICU. Median duration of iNO use was 4 days (range: 1-21 days). In multivariable mixed logistic regression analysis, use of deep hypothermic circulatory arrest (odds ratio: 3.2; 95% confidence interval: 1.2, 8.4) and center (analyzed as a random effect, p = .02) were independently associated with iNO use.

Conclusions

In this contemporary multicenter study, nearly half of patients who underwent repair of truncus arteriosus received iNO postoperatively. Use of iNO was more dependent on individual center practice rather than patient characteristics. The study suggests a need for collaborative quality initiatives to determine optimal criteria for utilization of this important but expensive therapy.

A fond farewell

Douglas S. Moodie — Thu, 09 Jan 2020 05:32:24 -0800

Congenital Heart Disease, Volume 14, Issue 6, Page 884-884, November/December 2019.

Issue Information

Thu, 09 Jan 2020 05:32:24 -0800

Congenital Heart Disease, Volume 14, Issue 6, Page 879-883, November/December 2019.

Corrigendum

Thu, 09 Jan 2020 05:32:24 -0800

Congenital Heart Disease, Volume 14, Issue 6, Page 1214-1214, November/December 2019.

Pharmacologic stress cardiovascular magnetic resonance in the pediatric population: A review of the literature, proposed protocol, and two examples in patients with Kawasaki disease

Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Pharmacologic stress cardiovascular magnetic resonance (PSCMR) is a well-established and reliable diagnostic tool for evaluation of coronary artery disease in the adult population. Stress imaging overall and PSCMR in particular is less utilized in the pediatric population with limited reported data. In this review, we highlight the potential use of PSCMR in specific pediatric cohorts with congenital and acquired heart disease, and we review the reported experience. A suggested protocol is presented in addition to two case examples of patients with Kawasaki disease where PSCMR aided decision making.

A case series of left main coronary artery ostial atresia and a review of the literature

Thu, 09 Jan 2020 05:32:24 -0800

Abstract

Left main coronary artery ostial atresia (LMCAOA) is a rare congenital anomaly of the coronary arteries. The published literature regarding the current diagnostic and management recommendations are limited. We present three case series of LMCAOA from our institution, including one with a unique association with anomalous origin of left coronary artery (LCA) from pulmonary artery. In addition, this report includes a review of 50 pediatric and 43 adult cases from literature. The majority of the patients were symptomatic. Sudden cardiac death occurred in 10% of pediatric patients and 7% of adult patients. Almost half of pediatric patients had additional cardiac lesions. At the time of diagnosis, 82% of patients had abnormal exercise stress test and 73% had abnormal myocardial perfusion imaging (MPI). The diagnosis of LMCAOA was suspected by echocardiography in 47% of pediatric patients, while 26% were initially misdiagnosed as anomalous origin of LCA from pulmonary artery. Coronary angiography confirmed the diagnosis in most cases and 70.5% of pediatric patients had small collaterals, while 80.5% of adult patients had large collaterals. Nine pediatric patients had no revascularization surgery with five deaths. Revascularization surgery was performed in 39 pediatric patients with four deaths. After 2005, there is a gradual shift toward performing coronary osteoplasty rather than coronary artery bypass grafting. Eighteen adult patients had revascularization surgery and all survived. Fifteen adult patients had no revascularization surgery, of which there were five deaths. In patients with LMCAOA, revascularization surgery is currently recommended in the presence of symptoms, ischemic changes on electrocardiogram or exercise stress test, myocardial perfusion defect on MPI, global left ventricular systolic dysfunction on echocardiogram, severe mitral regurgitation, or small-sized collaterals in coronary angiography. Short-term and mid-term outcomes are encouraging.