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Heart Failure in Adult Patients with Congenital Heart Disease.

Anesthesiol Clin. 2019 Dec;37(4):751-768

Authors: Smit-Fun VM, Buhre WF

Adult patients with congenital heart disease are a complex population with a variety of pathophysiologic conditions based on the anatomy and type of surgery or intervention performed, usually during the first years of life. Nowadays, the majority of patients survive childhood and present for a number of noncardiac surgeries or interventions needing appropriate perioperative management. Heart failure is a major contributing factor to perioperative morbidity and mortality. In this review, we present an overview of the most common types of adult patients with congenital heart disease and actual knowledge on therapy and specific risks in this challenging patient population.

PMID: 31677689 [PubMed - in process]

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Impact of a centre and home-based cardiac rehabilitation program on the quality of life of teenagers and young adults with congenital heart disease: the QUALI- REHAB study rationale, design and methods.

Int J Cardiol. 2019 08 01;288:70-71

Authors: Frigiola A

PMID: 30879939 [PubMed - indexed for MEDLINE]

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Centile Curves for Velocity-Time Integral Times Heart Rate as a Function of Ventricular Length: The Use of Minute Distance Is Advantageous to Enhance Clinical Reliability in Children.

J Am Soc Echocardiogr. 2018 01;31(1):105-112.e2

Authors: Solinski A, Klusmeier E, Horst JP, Körperich H, Haas NA, Kececioglu D, Laser KT

BACKGROUND: The generation of velocity-time integrals (VTIs) from Doppler signals is an essential component of standard echocardiographic investigations. The most effective algorithm to compensate for growth in children has, however, not yet been identified. This study was initiated to establish pediatric reference values for VTI and to enhance the interpretability of those values, considering technical and physiological factors.
METHODS: The echocardiographic data sets of healthy children and adolescents (N = 349; age range, 0-20 years) were recorded in a prospective approach and subsequently analyzed. In a pilot study, aortic and pulmonary VTIs were set in relation to the physiologic parameters of heart size as possible influencing parameters in a subgroup of children with comparable physical characteristics. The ratio with the smallest SD was taken as the base to generate centile curves using the LMS method. The clinical utility of the model was tested by examining patients (n = 80) with shunt lesions such as patent ductus arteriosus and atrial septal defect.
RESULTS: Feasibility was 94.6% for aortic VTI and 92.8% for pulmonary VTI. The pilot study identified ventricular length and heart rate as suitable parameters with the lowest relative SDs and high correlations with VTI. Gender differences were not relevant for children <7 years of age, and with increasing age, SD increased because of higher stroke volume variations. The detection of increased aortic VTI was possible with sensitivity of 73% for patients with patent ductus arteriosus with moderate or large hemodynamically significant ductus arteriosus. Patients with atrial septal defects with enlarged right ventricles could be identified as having increased pulmonary VTI with sensitivity of 84%.
CONCLUSIONS: These new reference values for VTI times heart rate as a function of ventricular length may be of specific clinical value to improve the assessment of cardiac function, therapeutic decision making, and follow-up in pediatric patients with heart disease.

PMID: 29158018 [PubMed - indexed for MEDLINE]

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Efficacy of inhalational sevoflurane anesthesia induction on inhibiting the stress response to endotracheal intubation in children with congenital heart disease.

Eur Rev Med Pharmacol Sci. 2018 02;22(4):1113-1117

Authors: Wang CH, Luo J, Li J, Zhang JZ, Huang SY, Shao W, Ma HS

OBJECTIVE: To investigate the efficacy of inhalational sevoflurane anesthesia induction on inhibiting the stress response to endotracheal intubation in pediatric patients with congenital heart disease (CHD).
PATIENTS AND METHODS: Forty ASA physical status I/II pediatric patients scheduled for interventricular septal defect repair or interatrial septal defect repair, were randomly divided into two groups (20 each): intravenous induction group (Group C) and inhalational sevoflurane anesthesia induction group (Group D). In group C, anesthesia was induced with midazolam, pipecuronium bromide and fentanyl, and the children were examined by radial artery monitoring after the consciousness extinction. Also, they were endotracheally intubated after muscle relaxation. In group D, anesthesia was induced with inhalation of 8% sevoflurane and 6 L/min oxygen, and the children were examined by radial artery monitoring after the consciousness extinction and were endotracheally intubated 4 min later. Before anesthesia induction (T0), consciousness extinction (T1), endotracheal intubation (T2), endotracheal intubation (T3), and after endotracheal intubation (T4), 1 and 3 min after intratracheal intubation (T5,6), HR and bispectral index (BIS) were monitored. The MAP of T2-T6 points was recorded. Ulnar vein blood samples were taken for determination of Endothelin (ET) and Thromboxane A2(TXA2) in the points of consciousness extinction, and 5 and 10 min after endotracheal.
RESULTS: All the children were well examined by endotracheal intubation. Compared with the baseline value at T0, there was no significant difference of HR in group D, but the HR of group C was decreased at T2, T3, T4 and T6. The BIS of the two groups were decreased at T1-T6 (p<0.05). Compared with the values at T2, they were increased at T5 and T6 in group C, and increased at T6 in group D (p<0.05). Compared with group C, the MAP of group D was decreased at T5, and the BIS of the two groups was decreased at T2-T6 (p<0.05). There were no significant differences of ET and TXA2 between groups.
CONCLUSIONS: It is well inhibited the endotracheal intubation stress response in children with congenital heart diseases using sevoflurane inhalational anesthesia induction.

PMID: 29509264 [PubMed - indexed for MEDLINE]

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Illness Uncertainty and Posttraumatic Stress in Young Adults With Congenital Heart Disease.

J Cardiovasc Nurs. 2018 Jul/Aug;33(4):356-362

Authors: Moreland P, Santacroce SJ

BACKGROUND: Young adults with congenital heart disease (CHD) are at risk for chronic illness uncertainty in 4 domains: ambiguity about the state of their illness; lack of information about the disease, its treatment, and comorbidities; complexity of the healthcare system and relationship with healthcare providers; and unpredictability of the illness course and outcome. Chronic uncertainty has been associated with posttraumatic stress symptoms (PTSS) and posttraumatic stress disorder (PTSD).
OBJECTIVE: The aims of this study were to explore how young adults with CHD experience uncertainty and to describe the relationship between PTSS and the appraisal and management process.
METHODS: An exploratory, mixed methods design was used. Data were collected in person and via Skype from 25 participants (19-35 years old), who were diagnosed with CHD during childhood and able to read and write English. In-depth interviews and the University of California at Los Angeles Posttraumatic Stress Disorder Reaction Index were used to collect data. Qualitative data were analyzed using the constant comparative method.
RESULTS: The 4 domains of uncertainty were evident in the narratives. The PTSD mean (SD) score was 31.3 (7.7). Six participants met criteria for PTSD. Narrative analysis revealed a relationship between severity of PTSS and the appraisal and management of uncertainty. Participants with PTSD used management strategies that included avoidance, reexperiencing, and hyperarousal.
CONCLUSIONS: Young adults with CHD may be at risk for the development of long-term psychological stress and PTSD in the setting of chronic uncertainty. Regular monitoring to identify PTSS/PTSD may be a means to promote treatment adherence and participation in healthcare.

PMID: 29601372 [PubMed - indexed for MEDLINE]

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J Cardiopulm Rehabil Prev. 2018 11;38(6):374-379

Authors: Wagers TP, Stevens CJ, Ross KV, Leon KK, Masters KS

PURPOSE: Spontaneous coronary artery dissection (SCAD) is a nonatherosclerotic type of acute myocardial infarction that primarily affects young, healthy women without typical risk factors for heart disease. Few investigations have examined psychosocial variables in this population and none have looked at patient perceptions of the experience and stresses associated with having a SCAD event or the resources currently available to SCAD survivors. This investigation describes survivors' subjective experiences of SCAD. Participants also provided information about types and helpfulness of resources available to them post-SCAD, including cardiac rehabilitation.
METHODS: Participants were recruited online and completed a one-time questionnaire.
RESULTS: Participants (n = 409) completed a questionnaire concerning their experiences with their SCAD event in the 1 y and 2 wk prior to the SCAD event. Their responses reflected moderate to high perceptions of stress. Participants experienced the SCAD event as highly stressful and frightening and their heart health presents as a moderate-severe source of current, post-diagnosis stress. Spontaneous coronary artery dissection-based informational support was frequently rated as inadequate, whereas other supportive resources varied in their helpfulness and accessibility. Participants reported positive experiences in cardiac rehabilitation and strong interest in SCAD-specific, professionally led, online patient education and support groups.
CONCLUSIONS: This study is the largest to date investigation of SCAD survivors and their experiences in this understudied and perhaps underrecognized condition. Opportunities for researchers and providers to develop, tailor, and disseminate SCAD-specific interventions are discussed.

PMID: 29939880 [PubMed - indexed for MEDLINE]

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Prenatal echocardiographic classification and prognostic evaluation strategy in fetal pulmonary atresia with intact ventricular septum.

Medicine (Baltimore). 2019 Oct;98(42):e17492

Authors: Liu L, Wang H, Cui C, Li Y, Liu Y, Wang Y, Fan T, Peng B

Fetal pulmonary atresia with intact ventricular septum (PA/IVS) is a rare congenital heart disease. The present study aimed to classify PA/IVS and determine the relationship between prenatal echocardiographic characteristics and postnatal biventricular or univentricular repair strategies.A total of 51 fetuses with PA/IVS were examined from 2012 to 2019. Data on prenatal echocardiography, associated anomaly, karyotype, and outcome were collected. Two-dimensional measurements included tricuspid valve (TV) z-score, mitral valve (MV) z-score, TV/MV ratio, and ratio of right to left ventricle (RV/LV) length, whereas color Doppler measurements included degree of tricuspid regurgitation (TR), ventriculo-coronary artery communication (VCAC), tricuspid inflow duration (TID), cardiac cycle duration (CCD), middle cerebral artery pulsatility index (MCA PI), and umbilical artery pulsatility index (UA PI). Diagnostic classification was based on the development of RV and the presence or absence of VCAC. Postnatal evaluation was divided according biventricular or univentricular repair.Of the 51 fetuses with PA/IVS, 20 were type I, 17 were type II, and 14 were type III. Only one fetus exhibited right aortic arch. The karyotype of all the fetuses was normal. Of the 28 patients who underwent postnatal surgery, 13 (46%) underwent biventricular repair and 15 (54%) underwent univentricular repair. TV z-score was significantly higher for the biventricular repair group compared with univentricular repair group (-1.20 ± 0.98 vs -4.33 ± 0.80, P = .000). TV/MV, RV/LV length, and TID/CCD were significantly higher for the biventricular repair group than the univentricular repair group (0.81 ± 0.14 vs 0.54 ± 0.09, 0.71 ± 0.11 vs 0.49 ± 0.09, 39.20 ± 3.84 vs 29.16 ± 4.58, P = .000). Moderate or severe TR and VCAC were significantly different between the 2 groups (P = .000). Gestational age, MCA PI, and UA PI did not differ between the 2 groups (P = .72, P = .36, P = .06). The cutoff values for the biventricular repair characteristic curves were TV z-score >-3.28, TV/MV ratio >0.71, RV/LV length >0.62, and TID/CCD >33.95%. The sensitivities of the TV z-score, TV/MV, RV/LV length, and TID/CCD were 100%, 77%, 85%, and 92%, respectively. The specificities of the TV z-score, TV/MV, RV/LV length, and TID/CCD were 94%, 100%, 100%, and 94%, respectively.Fetal echocardiography was able to classify PA/IVS according to variable degree of RV and VCAC. In fetal PA/IVS, TV z-score >-3.28, TV/MV >0.71, RV/LV length >0.62, TID/CCD >33.95%, moderate and severe TR, and the absence of VCAC were associated with postnatal biventricular repair strategy. These findings may have implications for prenatal counseling and prediction of fetal outcome.

PMID: 31626103 [PubMed - indexed for MEDLINE]

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Exercise testing for assessment of heart failure in adults with congenital heart disease.

Heart Fail Rev. 2019 Nov 04;:

Authors: Burstein DS, Menachem JN, Opotowsky AR

Congenital heart disease (CHD)-related heart failure is common and associated with significant morbidity, mortality, and resource utilization. In adults with CHD (ACHD), exercise limitation is often underestimated. Quantitative assessment with cardiopulmonary exercise testing (CPET) provides a comprehensive evaluation of exercise capacity and can help risk stratify patients, particularly across serial testing. CPET parameters must be interpreted within the context of the underlying anatomy, specifically for patients with either single ventricle physiology and/or cyanosis. Acknowledging differences in CPET parameters between ACHD and non-ACHD patients with heart failure are also important considerations when evaluating the overall benefit of advanced heart failure therapies. CPET testing can also guide safe exercise recommendation, including those with ACHD-related heart failure.

PMID: 31686282 [PubMed - as supplied by publisher]

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Prevalence of selected cardiotropic pathogens in the myocardium of adult dogs with unexplained myocardial and rhythm disorders or with congenital heart disease.

J Am Vet Med Assoc. 2019 Nov 15;255(10):1150-1160

Authors: Santilli RA, Grego E, Battaia S, Gianella P, Tursi M, Di Girolamo N, Biasato I, Perego M

OBJECTIVE: To determine the prevalence of nucleic acid from selected cardiotropic pathogens in endomyocardial biopsy samples from dogs with unexplained myocardial and rhythm disorders (UMRD) and compare prevalence with that for a group of control dogs with congenital heart disease (CHD).
ANIMALS: 47 client-owned dogs.
PROCEDURES: Right ventricular endomyocardial biopsy was performed in dogs with UMRD (dilated cardiomyopathy [n = 25], atrioventricular block [6], and nonfamilial ventricular [4] and supraventricular arrhythmias [2]) or CHD (10) that required right ventricular catheterization. Biopsy samples were evaluated histologically, and PCR assays were used for detection of nucleic acid from 12 pathogens.
RESULTS: 197 biopsy samples were collected from dogs with UMRD (n = 172) or CHD (25). At least 1 pathogen was detected in 21 of 37 (57%; 95% confidence interval [CI], 41% to 71%) dogs with UMRD, and canine coronavirus was detected in 1 of 10 (10%; 95% CI, 2% to 40%) dogs with CHD. Dogs with UMRD were significantly more likely than dogs with CHD to have pathogens detected in biopsy samples (OR, 11.8; 95% CI, 1.3 to 103.0). The most common pathogens in dogs with UMRD were canine distemper virus, canine coronavirus, canine parvovirus 2, and Bartonella spp. No pathogens were detected in available blood samples from dogs with pathogens detected in biopsy samples.
CONCLUSIONS AND CLINICAL RELEVANCE: Detection of nucleic acids from selected cardiotropic pathogens in myocardial tissue from dogs with UMRD suggested a possible association between the 2. Further studies are needed to explore whether this association is causative or clinically important. (J Am Vet Med Assoc 2019;255:1150-1160).

PMID: 31687895 [PubMed - in process]

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Prevalence and Outcomes of Pediatric In-Hospital Cardiac Arrest Associated With Pulmonary Hypertension.

Pediatr Crit Care Med. 2019 Nov 05;:

Authors: Morgan RW, Topjian AA, Wang Y, Atkin NJ, Kilbaugh TJ, McGowan FX, Berg RA, Mercer-Rosa L, Sutton RM, Himebauch AS

OBJECTIVES: In adult in-hospital cardiac arrest, pulmonary hypertension is associated with worse outcomes, but pulmonary hypertension-associated in-hospital cardiac arrest has not been well studied in children. The objective of this study was to determine the prevalence of pulmonary hypertension among children with in-hospital cardiac arrest and its impact on outcomes.
DESIGN: Retrospective single-center cohort study.
SETTING: PICU of a quaternary care, academic children's hospital.
PATIENTS: Children (<18 yr old) receiving greater than or equal to 1 minute of cardiopulmonary resuscitation (cardiopulmonary resuscitation) for an index in-hospital cardiac arrest with an echocardiogram in the 48 hours preceding in-hospital cardiac arrest, excluding those with cyanotic congenital heart disease.
MEASUREMENTS AND MAIN RESULTS: Of 284 in-hospital cardiac arrest subjects, 57 (20%) had evaluable echocardiograms, which were analyzed by a cardiologist blinded to patient characteristics. Pulmonary hypertension was present in 20 of 57 (35%); nine of 20 (45%) had no prior pulmonary hypertension history. Children with pulmonary hypertension had worse right ventricular systolic function, measured by fractional area change (p = 0.005) and right ventricular global longitudinal strain (p = 0.046); more right ventricular dilation (p = 0.010); and better left ventricular systolic function (p = 0.001). Children with pulmonary hypertension were more likely to have abnormal baseline functional status and a history of chronic lung disease or acyanotic congenital heart disease and less likely to have sepsis or acute kidney injury. Children with pulmonary hypertension were more likely to have an initial rhythm of pulseless electrical activity or asystole and were more frequently treated with inhaled nitric oxide (80% vs 32%; p < 0.001) at the time of cardiopulmonary resuscitation. On multivariable analysis, pulmonary hypertension was not associated with event survival (14/20 [70%] vs 24/37 [65%]; adjusted odds ratio, 1.30 [CI95, 0.25-6.69]; p = 0.77) or survival to discharge (8/20 [40%] vs 10/37 [27%]; adjusted odds ratio, 1.17 [CI95, 0.22-6.44]; p = 0.85).
CONCLUSIONS: Pulmonary hypertension physiology preceding pediatric in-hospital cardiac arrest may be more common than previously described. Among this cohort with a high frequency of inhaled nitric oxide treatment during cardiopulmonary resuscitation, pulmonary hypertension was not associated with survival outcomes.

PMID: 31688674 [PubMed - as supplied by publisher]