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Atrioventricular block after congenital heart surgery: Analysis from the Pediatric Cardiac Critical Care Consortium.

J Thorac Cardiovasc Surg. 2019 03;157(3):1168-1177.e2

Authors: Romer AJ, Tabbutt S, Etheridge SP, Fischbach P, Ghanayem NS, Reddy VM, Sahulee R, Tanel RE, Tweddell JS, Gaies M, Banerjee M, Retzloff L, Zhang W, Patel AR

OBJECTIVES: Our primary aims were to describe the contemporary epidemiology of postoperative high-grade atrioventricular block (AVB), the timing of recovery and permanent pacemaker (PPM) placement, and to determine predictors for development of and recovery from AVB.
METHODS: Patients who underwent congenital heart surgery from August 2014 to June 2017 were analyzed for AVB using the Pediatric Cardiac Critical Care Consortium registry. Predictors of AVB with or without PPM were identified using multinomial logistic regression. We used these predictors to model the probability of PPM for the subgroup of patients with intraoperative complete AVB.
RESULTS: We analyzed 15,901 surgical hospitalizations; 422 (2.7%) were complicated by AVB and 162 (1.0%) patients underwent PPM placement. In patients with transient AVB, 50% resolved by 2 days, and 94% resolved by 10 days. In patients who received a PPM, 50% were placed by 8 days and 62% were placed by 10 days. Independent risk factors associated with PPM compared with resolution of AVB were longer duration of cardiopulmonary bypass (relative risk ratio, 1.04; P = .023) and a high-risk operation (relative risk ratio, 2.59; P < .001). Among patients with complete AVB originating in the operating room, those with the highest predicted probability of PPM had a PPM placed only 77% of the time.
CONCLUSIONS: In this cohort, postoperative AVB complicated almost 3% of congenital heart surgery cases and 1% of patients underwent PPM placement. Because almost all patients (94%) with transient AVB had resolution by 10 days, our results suggest there is limited benefit to delaying PPM placement beyond that time frame.

PMID: 30917883 [PubMed - indexed for MEDLINE]

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The role of echocardiography for quantitative assessment of right ventricular size and function in adults with repaired tetralogy of Fallot.

Congenit Heart Dis. 2019 Sep;14(5):700-705

Authors: Egbe AC, Pislaru SV, Kothapalli S, Jadav R, Masood M, Angirekula M, Pellikka PA

BACKGROUND: Quantitative assessment of right ventricular (RV) systolic function by echocardiography is challenging in patients with congenital heart disease because of the complex geometry of the RV and the iatrogenic structural abnormalities resulting from prior cardiac surgeries. The purpose of this study was to determine the correlation between echocardiographic indices of RV systolic function and cardiac magnetic resonance imaging (CMRI) derived RV ejection fraction (RVEF) in adults with repaired tetralogy of Fallot (TOF).
METHODS: Quantitative assessment of RV function was performed with RV tissue Doppler systolic velocity (RV s'), tricuspid annular plane systolic excursion (TAPSE), and fractional area change (FAC). These echocardiographic indices were compared to RVEF from CMRI performed on the same day as echocardiogram.
RESULTS: Of 209 patients, the mean RV FAC was 39 ± 9%, TAPSE was 18 ± 4 mm, RV s' was 10 ± 2 cm/s, and RVEF was 40 ± 10%. There was a good correlation between TAPSE and RVEF (r = 0.79, P < .001), good correlation between RV s' and RVEF (r = 0.71, P < .001), and modest correlation between FAC and RVEF (r = 0.66, P < .001). TAPSE < 17 mm effectively discriminated between patients with RV systolic dysfunction defined as RVEF < 47% (sensitivity 81%, specificity 79%, area under the curve [AUC] 0.805). FAC < 40% was associated with RVEF < 47% (sensitivity 72%, specificity 63%, AUC 0.719). RV s' < 11 cm was associated with RVEF < 47% (sensitivity 83%, specificity 68%, AUC 0.798).
CONCLUSION: Despite the structural and functional abnormalities of the RV in patients with repaired TOF, quantitative assessment of RV systolic function by echocardiography is feasible and had good correlation with CMRI-derived RVEF.

PMID: 31070871 [PubMed - indexed for MEDLINE]

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The clinical characteristics of coronary artery fistula anomalies in children and adults: A 24-year experience.

Congenit Heart Dis. 2019 Sep;14(5):772-777

Authors: Huynh KT, Truong VT, Ngo TNM, Dang TB, Mazur W, Chung ES, Tretter JT, Kereiakes DJ, Le TK, Pham VN

OBJECTIVES: The aim of our work is to investigate the clinical characteristics of coronary artery fistula (CAF) anomalies in South Vietnam.
METHODS: This is a retrospective analysis of 119 patients with diagnosis of definite CAF between January 1992 and April 2016. The demographic, clinical, echocardiographic, and angiographic characteristics and management of CAF with short-term outcomes are described.
RESULTS: The median age was 15 years (range, 1-79 years), with 49 male (41%) and 70 female (59%). There were 77 symptomatic patients (64.7%) and 91 patients (76.5%) who presented with a murmur. The electrocardiogram was abnormal in 45.4% and cardiac enlargement or increased pulmonary vasculature were seen in 76 patients (63.9%) on chest X-ray. The sensitivity of echocardiography for CAF diagnosis was 79%. The source of the fistula was most often from the RCA (54%), most commonly to right atrium (34.5%) or right ventricle (31.1%). In comparison with surgery, transcatheter closure had a shorter hospital length of stay (5.4 ± 3.8 days vs 12.6 ± 6.5 days, P = .02) and better postprocedural left ventricular ejection fraction (67.9 ± 8.1% vs 62.9 ± 6.0%, P = .03).
CONCLUSION: The majority of fistula in this study originated from the RCA and terminated in the right atrium or the right ventricle. Transcatheter and surgical closure are both relatively safe and effective, with the potential for shortened length of hospital stay following transcatheter closure.

PMID: 31136097 [PubMed - indexed for MEDLINE]

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Role of Doppler echocardiography for assessing right ventricular cardiac output in patients with atrial septal defect.

Congenit Heart Dis. 2019 Sep;14(5):713-719

Authors: Yogeswaran V, Kanade R, Mejia C, Fatola A, Kothapalli S, Najam M, Sandhyavenu H, Angirekula M, Osma K, Jessey M, Hagler D, Egbe AC

BACKGROUND: Although Doppler echocardiography is routinely used to assess left ventricle cardiac output, there are limited data about the feasibility of Doppler echocardiography for right ventricular (RV) cardiac output assessment in patients with left-to-right shunt. The purpose of the study was to determine the correlation between Doppler-derived and Fick-derived RV cardiac index (CI), and the interobserver correlation in Doppler-derived RV CI assessment.
METHODS: Retrospective study of patients (age ≥18 years) with unrepaired atrial septal defect who underwent cardiac catheterization and echocardiography (within 3 days), 2004-2017. RV CI was calculated using the hydraulic orifice formula: [.785 × (right ventricle outflow tract diameter)2  × right ventricular outflow tract (RVOT) time velocity integral × heart rate]/body surface area.
RESULTS: A total of 128 patients (age 52 ± 17 years; female 88 [69%]) met the inclusion criteria. There was a modest correlation between Doppler-derived and Fick-derived RV CI (r = .57, P < .001), and the mean difference between Doppler-derived and Fick-derived RV CI was -.3 (95% confidence interval of agreement, -.8 to +.9) L/min/m2 . There was also a modest correlation between Doppler-derived RV CI from observer #1 and observer #2 (r = .62, P < .001), and the mean difference between Doppler-derived RV CI from observer #1 and observer #2 was -.2 (95% confidence interval of agreement, -.9 to +.6).
CONCLUSIONS: The current study demonstrated a modest correlation between Doppler-derived and Fick-derived RV cardiac output, and a modest interobserver correlation in Doppler-derived RV cardiac output assessment. Further studies are required to validate these results and to explore other potential applications such as in patients with chronic pulmonary regurgitation.

PMID: 31207173 [PubMed - indexed for MEDLINE]

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More than the heart: Hepatic, renal, and cardiac dysfunction in adult Fontan patients.

Congenit Heart Dis. 2019 Sep;14(5):765-771

Authors: Byrne RD, Weingarten AJ, Clark DE, Huang S, Perri RE, Scanga AE, Menachem JN, Markham LW, Frischhertz BP

SETTING: Fontan-associated liver disease universally affects adults with single ventricle heart disease. Chronic kidney disease is also highly prevalent in adult Fontan patients. In this study, we evaluate the relationship of Fontan hemodynamics invasively and noninvasively with extra-cardiac dysfunction as measured by MELD and MELD-XI.
OBJECTIVE: We hypothesize that invasive and noninvasive measures of Fontan circuit congestion and ventricular dysfunction are associated with increased MELD and MELD-XI scores.
DESIGN: Single-center data from adults with Fontan palliation who had ongoing care, including cardiac catheterization, were retrospectively collected. Hemodynamic data from cardiac catheterization and echocardiographic assessment of ventricular and atrioventricular valve function were tested for association with serum creatinine, MELD, and MELD-XI. Linear regression was used to perform multivariable analysis in the echocardiogram cohort.
RESULTS: Fifty-seven patients had congruent lab and catheterization data for analysis. Sixty-three and sixty-nine patients had congruent lab and echocardiogram data for MELD and MELD-XI, respectively. Of the hemodynamic variables analyzed, only decreased systemic oxygen saturation had significant correlation with elevated MELD and MELD-XI (P = .045). Patients with moderately or severely reduced ejection fraction by echocardiogram had significantly higher MELD and MELD-XI scores compared to those with normal or mildly depressed systolic ventricular function (P = .008 and P < .001 for MELD and MELD-XI, respectively). Significant differences in creatinine were also found among the ventricular dysfunction groups (P = .02).
CONCLUSIONS: In adults following Fontan palliation, systolic ventricular dysfunction and decreased oxygen saturation were associated with hepatic and renal dysfunction as assessed by elevated serum creatinine, MELD, and MELD-XI scores.

PMID: 31282062 [PubMed - indexed for MEDLINE]

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Postoperative and short-term atrial tachyarrhythmia burdens after transcatheter vs surgical pulmonary valve replacement among congenital heart disease patients.

Congenit Heart Dis. 2019 Sep;14(5):838-845

Authors: Wadia SK, Lluri G, Aboulhosn JA, Laks H, Biniwale RM, Van Arsdell GS, Levi DS, Salem MM, Shannon KM, Moore JP

OBJECTIVE: We examined the atrial tachyarrhythmia (AT) burden among patients with congenital heart disease (CHD) following transcatheter (TC-) or surgical (S-) pulmonary valve replacement (PVR).
DESIGN/SETTING: This was a retrospective observational study of patients who underwent PVR from 2010 to 2016 at UCLA Medical Center.
PATIENTS: Patients of all ages who had prior surgical repair for CHD were included. Patients with a history of congenitally corrected transposition of the great arteries, underwent a hybrid PVR procedure, or had permanent atrial fibrillation (AF) without a concomitant ablation were excluded.
OUTCOME MEASURES: The primary outcome was a time-to-event analysis of sustained AT. Sustained ATs were defined as focal AT, intra-atrial reentrant tachycardia/atrial flutter, or AF lasting at least 30 seconds or terminating with cardioversion or antitachycardia pacing.
RESULTS: Two hundred ninety-seven patients (TC-PVR, n = 168 and S-PVR, n = 129) were included. During a median follow-up of 1.2 years, nine events occurred in TC-PVR group (5%) vs 23 events in S-PVR group (18%). In the propensity adjusted models, the following factors were associated with significant risk of AT after PVR: history of AT, age at valve implantation, severe right atrial enlargement, and S-PVR. In the secondary analysis, TC-PVR was associated with lower adjusted risk of AT events in the postoperative epoch (first 30 days), adjusted IRR 0.31 (0.14-0.97), P = .03, but similar risk in the short-term epoch, adjusted IRR 0.64 (0.14-2.94), P = .57.
CONCLUSION: There was an increased risk of AT in the first 30 days following S-PVR compared to TC-PVR. Additional factors associated with risk of AT events after PVR were a history of AT, age at valve implantation, and severe right atrial enlargement.

PMID: 31282099 [PubMed - indexed for MEDLINE]

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Postoperative and long-term outcomes in children with Trisomy 21 and single ventricle palliation.

Congenit Heart Dis. 2019 Sep;14(5):854-863

Authors: Peterson JK, Setty SP, Knight JH, Thomas AS, Moller JH, Kochilas LK

OBJECTIVE: Patients with Trisomy 21 (T21) and single ventricle (SV) physiology present unique challenges compared to euploidic counterparts. This study reports postoperative and long-term outcomes in patients with T21 and SV palliation.
DESIGN: This retrospective cohort study from the Pediatric Cardiac Care Consortium (PCCC) included patients with T21 (<21 years old) that underwent surgical palliation for SV between 1982 and 2008 and control patients without known genetic anomaly following Fontan palliation for similar diagnoses. Kaplan-Meier survival plots were created based on death events obtained from the PCCC and by linkage with the National Death Index (NDI) and the Organ Procurement and Transplantation Network (OPTN) through 2014 for patients with adequate identifiers.
RESULTS: We identified 118 children with T21 who underwent initial surgical SV palliation. Among 90 (75.6%) patients surviving their first surgery, 66 (73.3%) underwent Glenn anastomosis and 25 (27.8%) completed Fontan palliation with in-hospital survival of 80.3% and 76.0%, respectively. Fifty-three patients had sufficient identifiers for PCCC-NDI-OPTN linkage. Ten-year survival, conditioned on discharge alive after the Fontan procedure, was 66.7% compared to 92.2% for 51 controls without genetic anomaly (P = .001). Median age at death for T21 patients following initial surgical SV palliation was 2.69 years (IQR 1.34-7.12) with most deaths (89.2%) attributed to the underlying congenital heart disease (CHD).
CONCLUSIONS: Children with T21 and SV are at high risk for procedural and long-term mortality related to their genetic condition and underlying CHD. Nevertheless, a select group of patients can successfully complete Glenn or Fontan palliation, reaching satisfactory long-term survival.

PMID: 31332952 [PubMed - indexed for MEDLINE]

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Vancomycin induced DRESS syndrome (drug reaction with eosinophilia and systemic symptoms) in a patient with tricuspid endocarditis.

BMJ Case Rep. 2019 Sep 16;12(9):

Authors: Hewitson LJ

A 57-year-old man presented for an elective pacemaker upgrade, complicated by the discovery of device infection. He had a background of complex congenital heart disease, including replacement of heart valves, and was treated for presumed infective endocarditis that was later confirmed by echocardiography. Antibiotic treatment, with intravenous vancomycin, was given as per the tissue sample sensitivities. On day 24 of treatment he deteriorated clinically, with the evolution of recurrent fever, epigastric pain, diarrhoea, widespread pruritic rash, lymphadenopathy and severe hypoxia over the subsequent 7-10 days. Blood tests revealed development of a marked eosinophilia, transaminitis and rising inflammatory markers. Further radiological imaging was non-diagnostic. On the basis of these clinical and biochemical features a diagnosis of drug reaction with eosinophilia and systemic symptoms syndrome was made. This led to the cessation of vancomycin, the offending agent and the referral for specialist immunology advice. He was subsequently treated with oral prednisolone and made a full recovery.

PMID: 31527200 [PubMed - indexed for MEDLINE]

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Newly Developed Adult Congenital Heart Disease Anatomic and Physiological Classification: First Predictive Validity Evaluation.

J Am Heart Assoc. 2020 Mar 03;9(5):e014988

Authors: Ombelet F, Goossens E, Van De Bruaene A, Budts W, Moons P

Background Risk stratification for adults with congenital heart disease is usually based on the anatomic complexity of the patients' defect. The 2018 American Heart Association/American College of Cardiology guidelines for the management of adults with congenital heart disease proposed a new classification scheme, combining anatomic complexity and current physiological stage of the patient. We aimed to investigate the capacity of the Adult Congenital Heart Disease Anatomic and Physiological classification to predict 15-year mortality. Methods and Results Data on 5 classification systems were collected for 629 patients at the outpatient clinic for a previous study. After 15 years, data on mortality were obtained through medical record review. For this assessment, we additionally collected information on physiological state to determine the Adult Congenital Heart Disease Anatomic and Physiological classification. Harrell's concordance statistics index, obtained through a univariate Cox proportional hazards regression, was 0.71 (95% CI, 0.63-0.78) for the Adult Congenital Heart Disease Anatomic and Physiological classification. Harrell's concordance statistics index of the congenital heart disease anatomic component only was 0.67 (95% CI, 0.60-0.74). The highest Harrell's concordance statistics index was obtained for the anatomic complexity in combination with the Congenital Heart Disease Functional Index (0.79; 95% CI, 0.73-0.84). Conclusions This first investigation of the Adult Congenital Heart Disease Anatomic and Physiological classification system provides empirical support for adding the physiological component to the anatomic complexity in the prediction of 15-year cardiac mortality.

PMID: 32089057 [PubMed - in process]

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Septal Flash-like Motion of the Earlier Activated Ventricular Wall Represents the Pathophysiology of Mechanical Dyssynchrony in Single-Ventricle Anatomy.

J Am Soc Echocardiogr. 2020 Feb 20;:

Authors: Hayama Y, Miyazaki A, Ohuchi H, Miike H, Negishi J, Sakaguchi H, Kurosaki K, Shimizu S, Kawada T, Sugimachi M

BACKGROUND: In biventricular physiology, abnormal septal motion is a hallmark of mechanical dyssynchrony in the left bundle branch block. However, in single-ventricle (SV) physiology, morphologic variations in systemic ventricles pose a challenge in evaluating the negative impact of mechanical dyssynchrony. The present study aimed to characterize the pathologic dyssynchronous contraction patterns in patients with SV.
METHODS: In this retrospective study, 70 consecutive postoperative patients with SV anatomy with prolonged QRS duration (25 female patients; median age, 14 years) were enrolled. We divided each SV into two regions and analyzed independent strains using two-dimensional speckle-tracking echocardiography. From an earlier activated ventricular wall, we calculated the strain ratio (Rstrains) of two values (%) during the QRS period and the ejection period: (100 + Strainejection)/(100 + StrainQRS). We reviewed the clinical profiles, B-type natriuretic peptide plasma levels, exercise capacity, and morbidity. Six patients who underwent cardiac resynchronization therapy (CRT) were analyzed regarding changes in strain patterns and ventricular volume.
RESULTS: Higher Rstrains, indicating a preceding contraction and subsequent dyskinetic dilation of the earlier activated ventricular wall, was associated with increased B-type natriuretic peptide, reduced exercise capacity, and poor outcome. However, delayed contraction of the later activated ventricular wall was not associated with the effects. Decreases in Rstrains and ventricular volume reductions were observed in all patients after CRT.
CONCLUSIONS: A specific strain pattern in an earlier activated ventricular wall indicates mechanical dyssynchrony in patients with SV. This pattern is very similar to the septal flash in adult patients with left bundle branch block. This strategy might be a promising approach for selecting appropriate candidates for CRT in patients with SV.

PMID: 32089381 [PubMed - as supplied by publisher]