Cardiac Autonomic Modulation of Heart Rate Recovery in Children with Spina Bifida.

We aimed to analyse cardiac autonomic control by assessing the post-exercise heart rate recovery (HRR) and physical fitness in children and adolescents with spina bifida (SB), compared to participants with typical development. A total of 124 participants, 42 with spina bifida (SB group) and 82 typical developmental controls (CO group) performed the arm cranking exercise test with a gas analysis system. HRR was determined at the first (HRR_1) and second (HRR_2) minute at recovery phase. Data are shown as [mean difference (95% CI)]. The SB group showed reduced HR reserve [14.5 (7.1-22.0) bmp, P<0.01], slower HRR_1 [12.4 (7.4-17.5) bpm, P<0.01] and HRR_2 [16.3 (10.6-21.9) bpm; P<0.01], lower VO2peak [VO2peak relative: 7.3 (4.2-10.3) mL·min-1·kg-1, P<0.01; VO2peak absolute: 0.42 (0.30-0.54) L·min-1, P<0.01], and lower O2 pulse [2.5 (1.8-3.2) mL·bpm, P<0.01] and ventilatory responses [13.5 (8.8-18.1) L·min-1, P<0.01] than the CO group. VE/VO2 was not different between groups [-2.82 (-5.77- -0.12); P=0.06], but the VE/VCO2 [-2.59 (-4.40-0.78); P<0.01] and the values of the anaerobic threshold corrected by body mass [-3.2 (-5.8- -0.6) mL·min-1·kg-1, P=0.01] were higher in the SB group than in the CO group. We concluded that children and adolescents with SB have reduced physical fitness and a slower HRR response after maximal effort.

Genetic variants of HIF1α are associated with right ventricular fibrotic load in repaired tetralogy of Fallot patients: a cardiovascular magnetic resonance study.

BACKGROUND: Studies suggest that right ventricular (RV) fibrosis is associated with RV remodeling and long-term outcomes in patients with tetralogy of Fallot (TOF). Pre-operative hypoxia may increase expression of hypoxia inducible factor-1-alpha (HIF1α) and promote transforming growth factor ß1 (TGFß1)-mediated fibrosis. We hypothesized that there would be associations between: (1) RV fibrosis and RV function, (2) HIF1α variants and RV fibrosis, and (3) HIF1α variants and RV function among post-surgical TOF cases. METHODS: We retrospectively measured post-surgical fibrotic load (indexed volume and fibrotic score) from 237 TOF cases who had existing cardiovascular magnetic resonance imaging using late gadolinium enhancement (LGE), and indicators of RV remodeling (i.e., ejection fraction [RVEF] and end-diastolic volume indexed [RVEDVI]). Genetic data were available in 125 cases. Analyses were conducted using multivariable linear mixed-effects regression with a random intercept and multivariable generalized Poisson regression with a random intercept. RESULTS: Indexed fibrotic volume and fibrotic score significantly decreased RVEF by 1.6% (p = 0.04) and 0.9% (p = 0.03), respectively. Indexed fibrotic volume and score were not associated with RVEDVI. After adjusting for multiple comparisons, 6 of the 48 HIF1α polymorphisms (representing two unique signals) were associated with fibrotic score. None of the HIF1α polymorphisms were associated with indexed fibrotic volume, RVEDVI, or RVEF. CONCLUSION: The association of some HIF1α polymorphisms and fibrotic score suggests that HIF1α may modulate the fibrotic response in TOF.

A Systematic Review of Benefits and Risks of Fetal Surgery for Congenital Cardiac Defects Such as Pulmonary Valve Stenosis and Critical Aortic Stenosis.

INTRODUCTION: Congenital heart diseases (CHDs) constitute the most prevalent congenital pathology, and they are a consequence of structural and functional abnormalities during fetal development. The etiology of CHD involves the interaction of genetic and environmental factors. Fetal cardiac surgery aims at preventing natural pathways of CHD in utero, mitigating progression to more complex abnormalities. The goal of this review was to demonstrate the benefits and risks of fetal interventions in the two most prevalent CHDs, pulmonary stenosis and pulmonary atresia with an intact ventricular septum, but also critical aortic stenosis and hypoplastic left heart syndrome. METHODS: Original and relevant articles were selected by meta-aggregation to perform a qualitative analysis of fetal cardiac interventions for pulmonary stenosis and critical aortic stenosis. The Joanna Briggs Institute's Qualitative Assessment and Review Instrument (or JBI-QARI) was used for data quality appraisal. RESULTS: Of 61 potential articles, 13 were selected, and nine were finally included. Discussion: The present review demonstrated that fetal cardiac surgery increases right ventricular growth and hemodynamic flow in pulmonary stenosis, whereas in critical aortic stenosis it enables growth of the left ventricle and increases left ventricular pressure. However, it has a high complication rate, along with considerable morbidity and mortality. CONCLUSION: The benefits of fetal cardiac surgery for pulmonary stenosis and critical aortic stenosis are well-described in the literature; however, there is a significant risk of complications which can be reduced by the surgeon's technical expertise and well-structured hospital facilities.

Congenitally Corrected Transposition of the Great Arteries in the Adult.

INTRODUCTION: Congenitally corrected transposition of the great arteries (CCTGA) is a rare anomaly. Current data available regarding adult cases is derived from small series, information simultaneously presented in pediatric publications, and one classical multicenter study. This review, not aimed to exhaust the subject, has the purpose to examine the literature addressing presentation, diagnostic methodology, and management of afflicted adult patients. METHODS: A comprehensive search was undertaken in three major databases (PubMed, Cochrane, SciELO), using the keywords "congenitally corrected transposition of the great arteries" and "adults". Relevant articles in English, Spanish, and Portuguese were extracted and critically appraised in this review. Steps for study selection were: (1) identification of titles of records through databases searching, (2) removal of duplicates, (3) screening and selection of abstracts, (4) final inclusion in the study. RESULTS: Four hundred sixty-five publications on CCTGA in adult patients were retrieved, and 166 were excluded; 299 studies were used for this review including 76 full-text articles, 70 studies related to general aspects of the subject, and, due to the small number of publications, 153 case reports. Sixty-one articles referring to combined experiences in pediatric and adult patients and judged to be relevant, but retrieved from another sources, were also included. CONCLUSION: Albeit clinical presentation and diagnostic criteria have been well stablished, there seems to be room for discussion related to clinical and surgical management of CCTGA in adults. Considering the rarity of the disease, well designed multicenter studies may provide answers.

Central Venous Oxygen Saturation/Lactate Ratio and Prediction of Major Adverse Events After Pediatric Heart Surgery.

INTRODUCTION: Major adverse events (MAE) are unexpected but undesirably frequent after pediatric congenital heart surgery and contribute to poorer outcomes. The aim of this study was to test the predictive value of a ratio between central venous oxygen saturation and arterial lactate (ScvO2/lactate) for MAE after pediatric congenital heart surgery in a Brazilian university hospital. METHODS: We conducted a retrospective observational study in a tertiary care university hospital, including 194 infants and children submitted to surgery for congenital heart disease. The predictive value of ScvO2, lactate, and ScvO2/lactate ratio were assessed by the area under the receiver operating characteristics curve (AUC), sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV). RESULTS: The incidence of MAE was 16% - cardiac arrest/death, unplanned reoperation, and low cardiac output syndrome were the most common events. Overall, ScvO2/lactate ratio discriminated patients with and without MAE very well (AUC 0.842), performing better than either variable alone, with sensitivity of 48%, specificity of 94%, PPV of 60%, and NPV of 91%. CONCLUSION: A ScvO2/lactate ratio > 5 can accurately identify patients at low risk of MAE after pediatric congenital heart surgery, with very good specificity and NPV, but poor sensitivity and PPV.

Evaluation of Coronary Circulation after Arterial Switch Operation. / Avaliação da Circulação Coronariana após a Cirurgia de Jatene.

BACKGROUND: Coronary artery evaluation remains after arterial switch operation a clinical challenge. OBJECTIVE: This study aims to correlate anatomical changes diagnosed by cardiac computed tomography (CCT) with physiological alterations on clinical evaluation to diagnose coronary obstruction in late ASO patients. METHODS: This study included 61 consecutive patients with mean age of 9.4 years who underwent ASO. The patients were submitted to echocardiography, electrocardiography, cardiopulmonary exercise test, and cardiac computed tomography to evaluate functional capacity and coronary artery anatomy. RESULTS: Cardiac computed tomography revealed that only 3.3% of the patients had coronary stenosis. These patients were asymptomatic, and no signs of myocardial ischemia were detected by the tests. CONCLUSION: The incidence of coronary abnormalities in late ASO patients was 3.3% in our cohort. There is no clear guideline as to why, when, and how these patients should be screened or what to propose when a coronary obstruction is diagnosed in asymptomatic patients.

FUNDAMENTO: A avaliação da artéria coronária após a cirurgia de Jatene ainda é um desafio clínico. OBJETIVO: Correlacionar alterações anatômicas identificadas por tomografia computadorizada cardíaca (TCC) com alterações fisiológicas detectadas na avaliação clínica para diagnosticar obstrução coronária no pós-operatório tardio de pacientes submetidos à cirurgia de Jatene. MÉTODOS: Este estudo incluiu 61 pacientes consecutivos com idade média de 9,4 anos que foram submetidos à cirurgia de Jatene. Os pacientes realizaram ecocardiografia, eletrocardiografia, teste cardiopulmonar do exercício, e tomografia computadorizada cardíaca para avaliação da capacidade funcional e anatomia da artéria coronária. RESULTADOS: A tomografia computadorizada cardíaca revelou que somente 3,3% dos pacientes apresentaram estenose da artéria coronária. Esses pacientes eram assintomáticos, e não foram detectados sinais de isquemia miocárdicas pelos exames realizados. CONCLUSÃO: A incidência de anormalidades da artéria coronária é de 3,3% no seguimento tardio de nossa coorte de pacientes submetidos à cirurgia de Jatene. Não existe uma diretriz clara sobre o porquê, quando, e como esses pacientes deveriam ser rastreados, ou o que propor quando pacientes assintomáticos forem diagnosticados com obstrução coronária.

Going home after a child's cardiac surgery: education for safe care.

OBJECTIVES: to validate the content and appearance of the booklet "Going home after a child's cardiac surgery" and assess family members' cognitive learning regarding its use. METHODS: a methodological and quasi-experimental study of before and after type, with semi-structured interview, pre-test and reading of the booklet in a hospital; post-test and validation occurred after hospital discharge. Wilcoxon non-parametric statistics were used. RESULTS: nineteen family members of children with heart disease participated. The average of correct answers increased 14 percentage points from pre- to post-test; most were medication errors. There was a significant difference (p <0.0001) in the comparison between cognitive knowledge prior to using the booklet and learning acquired from its use. All items received a positive rating, except font size. CONCLUSIONS: the booklet helps family members to understand a child's needs after cardiac surgery, and can be used to prevent unwanted occurrences and enable safe care at home.

Functional lymphatic reserve capacity is depressed in patients with a Fontan circulation.

BACKGROUND: Lymphatic abnormalities play a role in effusions in individuals with a Fontan circulation. Recent results using near-infrared fluorescence imaging disclosed an increased contraction frequency of lymphatic vessels in Fontan patients compared to healthy controls. It is proposed that the elevated lymphatic pumping seen in the Fontan patients is necessary to maintain habitual interstitial fluid balance. Hyperthermia has previously been used as a tool for lymphatic stress test. By increasing fluid filtration in the capillary bed, the lymphatic workload and contraction frequency are increased accordingly. Using near-infrared fluorescence imaging, the lymphatic functional reserve capacity in Fontan patients were explored with a lymphatic stress test. METHODS: Fontan patients (n = 33) were compared to a group of 15 healthy individuals of equal age, weight, and gender. The function of the superficial lymphatic vessels in the lower leg during rest and after inducing hyperthermia was investigated, using near-infrared fluorescence imaging. RESULTS: Baseline values in the Fontan patients showed a 57% higher contraction frequency compared to the healthy controls (0.4 ± 0.3 min-1 vs. 0.3 ± 0.2 min-1 , p = 0.0445). After inducing stress on the lymphatic vessels with hyperthermia the ability to increase contraction frequency was decreased in the Fontan patients compared to the controls (0.6 ± 0.5 min-1 vs. 1.2 ± 0.8 min-1 , p = 0.0102). CONCLUSIONS: Fontan patients had a higher lymphatic contraction frequency during normal circumstances. In the Fontan patients, the hyperthermia response is dampened indicating that the functional lymphatic reserve capacity is depressed. This diminished reserve capacity could be part of the explanation as to why some Fontan patients develop late-onset lymphatic complications.

Growth after ventricular septal defect repair: does defect size matter? A 10-year experience.

AIM: To evaluate whether the ventricular septal defect (VSD) size, along with the degree of preoperative growth impairment and age at repair, may influence postoperative growth, and if VSD size can be useful to identify children at risk for preoperative failure to thrive. METHODS: Sixty-eight children submitted to VSD repair in a Brazilian tertiary-care institution were evaluated. Weight and height measurements were converted to Z-scores. Ventricular septal defect size was normalized by dividing it by the aortic root diameter (VSD/Ao ratio). RESULTS: Twenty-six patients (38%) had significantly low weight-for-height, 10 patients (15%) had significantly low height-for-age and 13 patients (19%) had both conditions at repair. Catch-up growth occurred in 82% of patients for weight-for-age, in 75% of patients for height-for-age and in 89% of patients for weight-for-height. Weight-for-height Z-scores at surgery were significantly lower in patients who underwent repair before 9 months of age. The VSD/Ao ratio did not associate with any other data. On multivariate analysis, weight-for-age Z-scores and age at surgery were independent predictors of long-term weight and height respectively. CONCLUSION: The VSD/Ao ratio was not a good predictor of preoperative failure to thrive. Most patients had preoperative growth impairment and presented catch-up growth after repair. Preoperative growth status and age at surgery influenced long-term growth.

Adult Congenital Heart Disease Outpatient Clinic. Descriptive Analysis of A 12-Year Experience in Brazil.

OBJECTIVE: Description of adult congenital heart disease (CHD) outpatient characteristics has not been reported and several aspects regarding these patients require attention. We describe the 12-year experience of a Brazilian unit. METHODS: The main characteristics of 1168 patients were reviewed annotating for each patient age, gender, city of residence, main diagnosis, functional class at last examination, defect complexity and in-hospital referral pattern. RESULTS: Increasing workload was documented. Among the CHD patients, 663 (57%) were between 14 and 30 years old and 920 (79%) lived in the referral region. Referrals were made by hospital cardiologists for 611 (52%) patients, while 519 (45%) were referred by pediatric cardiologists. Regarding CHD severity, 637 (55%) had a defect of mild complexity. Of the patients analyzed, 616 (53%) had undergone an intervention, mainly atrial septal defect (ASD) closure, correction of tetralogy of Fallot, ventricular septal defect (VSD) closure and relief of coarctation of the aorta (CoAo). The main diagnosis of the 552 (47%) patients not submitted to an intervention were ASD, VSD, aortic stenosis, complex CHD and pulmonary stenosis. Regarding functional class, 1016 (87%) were in class I and 280 (24%) were lost to follow-up. Seventy-three patients had died, mainly due to cardiac death. CONCLUSION: In a unit were complex pediatric congenital heart surgery started twenty years ago, an increasing adult CHD workload was documented. Referral came predominantly from cities around the unit, most patients had low complexity defects and were in functional class I, a significant loss of follow-up was documented, and the death of patients was mainly due to the heart defect.

Scimitar sign in the absence of anomalous pulmonary venous drainage: a case report.

BACKGROUND: Scimitar syndrome consists of anomalous pulmonary vein drainage to the inferior vena cava. Its name derives from the image this anomalous pulmonary vein creates on a chest radiograph. We describe a case of normal venous pulmonary vein drainage that also presented the scimitar sign due to an aorto-collateral vessel. CASE SUMMARY: A 15-month-old girl presented with mild dyspnoea and fever. Control chest X-ray showed an image of cardiac dextroposition, hypoplastic right lung, and the 'scimitar sign'. Although the transthoracic echocardiogram confirmed the initial suspicion of anomalous pulmonary venous drainage, the computed tomography (CT) scan showed normal right pulmonary veins connected to the left atrium and revealed that an aorto-collateral vessel caused the scimitar sign. DISCUSSION: Although the patient had several typical alterations of the scimitar syndrome, the pulmonary venous connection was normal, and the scimitar sign was due to an aorto-collateral vessel. It might be difficult to describe venous pulmonary connections on the basis of echocardiography, so an angio CT scan proved to be a valuable tool in this scenario.

Superior vena cava obstruction after the Glenn procedure.

Acute obstruction of superior vena cava anastomosis right after the Glenn procedure may lead to tragic consequences. We describe the case of a one-year-old child with tricuspid atresia and a previous Blalock-Taussig shunt procedure, who presented severe low cardiac output syndrome right after the Glenn procedure and died forty-four hours after the procedure. The autopsy showed obstruction of the superior vena cava anastomosis. Patients that present superior vena cava syndrome and low cardiac output right after the Glenn procedure should have the surgical anastomosis revised immediately.

A Systematic Review of Benefits and Risks of Fetal Surgery for Congenital Cardiac Defects Such as Pulmonary Valve Stenosis and Critical Aortic Stenosis

ABSTRACT Introduction: Congenital heart diseases (CHDs) constitute the most prevalent congenital pathology, and they are a consequence of structural and functional abnormalities during fetal development. The etiology of CHD involves the interaction of genetic and environmental factors. Fetal cardiac surgery aims at preventing natural pathways of CHD in utero, mitigating progression to more complex abnormalities. The goal of this review was to demonstrate the benefits and risks of fetal interventions in the two most prevalent CHDs, pulmonary stenosis and pulmonary atresia with an intact ventricular septum, but also critical aortic stenosis and hypoplastic left heart syndrome. Methods: Original and relevant articles were selected by meta-aggregation to perform a qualitative analysis of fetal cardiac interventions for pulmonary stenosis and critical aortic stenosis. The Joanna Briggs Institute's Qualitative Assessment and Review Instrument (or JBI-QARI) was used for data quality appraisal. Results: Of 61 potential articles, 13 were selected, and nine were finally included. Discussion: The present review demonstrated that fetal cardiac surgery increases right ventricular growth and hemodynamic flow in pulmonary stenosis, whereas in critical aortic stenosis it enables growth of the left ventricle and increases left ventricular pressure. However, it has a high complication rate, along with considerable morbidity and mortality. Conclusion: The benefits of fetal cardiac surgery for pulmonary stenosis and critical aortic stenosis are well-described in the literature; however, there is a significant risk of complications which can be reduced by the surgeon's technical expertise and well-structured hospital facilities.

Isolated Left Ventricular Apical Hypoplasia without Lamin A/C Gene Mutation

Abstract Isolated left ventricular apical hypoplasia is a rare cardiomyopathy, with a broad range of clinical presentations. Since this entity was already described in association with osteomuscular diseases, mutation in the Lamin A/C gene has been regarded as a possible cause of this disease. This study describes the case of an asymptomatic teenager with isolated left ventricular apical hypoplasia and arthrogriposis but with no mutations in the entire Lamin A/C gene.

Collaborative Quality Improvement in the Congenital Heart Defects: Development of the ASSIST Consortium and a Preliminary Surgical Outcomes Report.

OBJECTIVE: ASSIST is the first Brazilian initiative in building a collaborative quality improvement program in pediatric cardiology and congenital heart disease. The purposes of this manuscript are: (a) to describe the development of the ASSIST project, including the historical, philosophical, organizational, and infrastructural components that will facilitate collaborative quality improvement in congenital heart disease care; (b) to report past and ongoing challenges faced; and (c) to report the first preliminary data analysis. METHODS: A total of 614 operations were prospectively included in a comprehensive online database between September 2014 and December 2015 in two participating centers. Risk Adjustment for Congenital Heart Surgery (RACHS) 1 and Aristotle Basic Complexity (ABC) scores were obtained. Descriptive statistics were provided, and the predictive values of the two scores for mortality were calculated by multivariate logistic regression models. RESULTS: Many barriers and challenges were faced and overcome. Overall mortality was 13.4%. Independent predictors of in-hospital death were: RACHS-1 categories (3, 4, and 5/6), ABC level 4, and age group (≤ 30 days, and 30 days - 1 year). CONCLUSION: The ASSIST project was successfully created over a solid base of collaborative work. The main challenges faced, and overcome, were lack of institutional support, funding, computational infrastructure, dedicated staff, and trust. RACHS-1 and ABC scores performed well in our case mix. Our preliminary outcome analysis shows opportunities for improvement.

Neuroprotective Anesthesia Regimen and Intensive Management for Pediatric Cardiac Surgery with Cardiopulmonary Bypass: a Review and Initial Experience.

This article describes our proposal for routine anesthesia, intraoperative medical management, cerebral and physiological monitoring during pediatric cardiac surgery with cardiopulmonary bypass that intend to provide appropriate anesthesia (analgesia, hypnosis), neuroprotection, adequate cerebral and systemic oxygen supply, and preventing against drugs neurotoxicity. A concise retrospective data is presented.

Congenitally Corrected Transposition of the Great Arteries in the Adult

ABSTRACT Introduction: Congenitally corrected transposition of the great arteries (CCTGA) is a rare anomaly. Current data available regarding adult cases is derived from small series, information simultaneously presented in pediatric publications, and one classical multicenter study. This review, not aimed to exhaust the subject, has the purpose to examine the literature addressing presentation, diagnostic methodology, and management of afflicted adult patients. Methods: A comprehensive search was undertaken in three major databases (PubMed, Cochrane, SciELO), using the keywords "congenitally corrected transposition of the great arteries" and "adults". Relevant articles in English, Spanish, and Portuguese were extracted and critically appraised in this review. Steps for study selection were: (1) identification of titles of records through databases searching, (2) removal of duplicates, (3) screening and selection of abstracts, (4) final inclusion in the study. Results: Four hundred sixty-five publications on CCTGA in adult patients were retrieved, and 166 were excluded; 299 studies were used for this review including 76 full-text articles, 70 studies related to general aspects of the subject, and, due to the small number of publications, 153 case reports. Sixty-one articles referring to combined experiences in pediatric and adult patients and judged to be relevant, but retrieved from another sources, were also included. Conclusion: Albeit clinical presentation and diagnostic criteria have been well stablished, there seems to be room for discussion related to clinical and surgical management of CCTGA in adults. Considering the rarity of the disease, well designed multicenter studies may provide answers.

Acompanhamento a longo prazo de paciente do sexo masculino com fibroma cardíaco assintomático / Long-Term follow-up of an asymptomatic man with a cardiac fibroma

Descreve-se o caso de um homem de 19 anos assintomático com fibroma de ventrículo esquerdo em acompanhamento por 15 anos, sem tratamento.(AU)

Here we describe a case of a 19-year-old asymptomatic man with a left ventricular fibroma on follow-up for 15 years with no treatment required.(AU)

Central venous oxygen saturation/lactate ratio and prediction of major adverse events after pediatric heart surgery

Abstract Introduction: Major adverse events (MAE) are unexpected but undesirably frequent after pediatric congenital heart surgery and contribute to poorer outcomes. The aim of this study was to test the predictive value of a ratio between central venous oxygen saturation and arterial lactate (ScvO2/lactate) for MAE after pediatric congenital heart surgery in a Brazilian university hospital. Methods: We conducted a retrospective observational study in a tertiary care university hospital, including 194 infants and children submitted to surgery for congenital heart disease. The predictive value of ScvO2, lactate, and ScvO2/lactate ratio were assessed by the area under the receiver operating characteristics curve (AUC), sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV). Results: The incidence of MAE was 16% — cardiac arrest/death, unplanned reoperation, and low cardiac output syndrome were the most common events. Overall, ScvO2/lactate ratio discriminated patients with and without MAE very well (AUC 0.842), performing better than either variable alone, with sensitivity of 48%, specificity of 94%, PPV of 60%, and NPV of 91%. Conclusion: A ScvO2/lactate ratio > 5 can accurately identify patients at low risk of MAE after pediatric congenital heart surgery, with very good specificity and NPV, but poor sensitivity and PPV.