Original Article
Outcomes of closure of doubly committed subarterial ventricular septal defects in adults
- Zhaoyang Chen, Wanhua Chen, Hang Chen, Zhenmei Liao, Qiang Chen, Lianglong Chen
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- Published online by Cambridge University Press:
- 20 April 2020, pp. 599-606
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Background:
Outcome data of doubly committed subarterial ventricular septal defect closure in adults are limited.
Methods:A review was made of the inpatients >18 years of age who underwent doubly committed subarterial ventricular septal defect closure between June 2010 and June 2017.
Results:The patients were categorised into two groups: The valve intervention group consisted of 31 patients who underwent aortic valvuloplasty, aortic valve replacement, or repair of sinus Valsalva aneurysm in addition to doubly committed subarterial ventricular septal defect closure; non-valvular intervention group comprised 58 patients who underwent only doubly committed subarterial ventricular septal defect closure. The groups did not differ by sex and age. Patients in the valve intervention group, with a larger ventricular septal defect size, were shorter and tended to be lighter. The valve intervention group had more patients with pneumonia perioperatively. No infective endocarditis and reoperation were noted during the study period. At last follow-up, 91 and 96.6% of the studied patients were free from left ventricle dilation and pulmonary hypertension. In patients without pre-operative aortic regurgitation, 12 developed new mild aortic regurgitation during the follow-up.
Conclusions:About 34.8% of adult patients with doubly committed subarterial ventricular septal defect required concurrent intervention on aortic valve or sinus Valsalva aneurysm. The midterm results of doubly committed subarterial ventricular septal defect closure in adult patients were favourable. However, the incidence of new mild aortic regurgitation after ventricular septal defect closure was high (27.3%). Long-term follow-up of aortic regurgitation progression is needed.
Outcomes of paediatric cardiac patients after 30 minutes of cardiopulmonary resuscitation prior to extracorporeal support
- Pilar Anton-Martin, Axel Moreira, Paul Kang, Michael L. Green
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- Published online by Cambridge University Press:
- 31 March 2020, pp. 607-616
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Objectives:
To characterise the mortality and neurological outcomes of paediatric cardiac patients requiring cardiopulmonary resuscitation for more than 30 minutes prior to extracorporeal membrane oxygenation cannulation and to identify risk factors associated with adverse outcomes in this population.
Materials and methods:Observational retrospective cohort study in paediatric cardiac patients undergoing cardiopulmonary resuscitation for greater than 30 minutes prior to cannulation in a tertiary children’s hospital, from July 2000 to July 2013.
Results:Seventy-three paediatric cardiac patients requiring cardiopulmonary resuscitation for more than 30 minutes prior to cannulation were included in the study. Survival to hospital discharge was 43.8%, with 75% of survivors having either normal neurologic function or only mild disability. Multivariable logistic regression analysis demonstrated that increased use of calcium during resuscitation (odds ratio 14.5, p 0.01), cardiopulmonary resuscitation duration >50 minutes (odds ratio 4.12, p 0.03), >6 interruptions of chest compressions during cannulation (odds ratio 6.40, p 0.03), the need for continuous renal replacement therapy (odds ratio 11.1, p 0.001), and abnormal pupillary response during extracorporeal membrane oxygenation (odds ratio 33.9, p 0.006) were independent predictors for hospital mortality.
Conclusion:Survival after cardiopulmonary resuscitation for more than 30 minutes prior to extracorporeal membrane oxygenation cannulation in our paediatric cardiac cohort was 43.8%. Factors associated with mortality included calcium use during resuscitation, longer cardiopulmonary resuscitation, increased chest compression pauses during cannulation, the use of continuous renal replacement therapy, and abnormal pupils during extracorporeal membrane oxygenation support. A prospective assessment of these factors in paediatric cardiac patients may be beneficial in improving outcomes.
Aetiological evaluation of chest pain in childhood and adolescence
- Emre Aygun, Sibel Tugce Aygun, Taciser Uysal, Fatih Aygun, Hasan Dursun, Ahmet Irdem
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- Published online by Cambridge University Press:
- 05 May 2020, pp. 617-623
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Background:
Chest pain, as a common cause of hospital admissions in childhood, necessitates detailed investigations due to a wide range of differential diagnoses. In this study, we aimed to determine the distribution of diseases causing chest pain in children and investigate the clinical characteristics of children with chest pain.
Methods:This study included 782 patients aged between 3 and 18 years who presented to a paediatric cardiology outpatient clinic with chest pain between April 2017 and March 2018. Aetiological causes and demographic features of the patients were analysed.
Results:Most prevalent causes of chest pain were musculoskeletal system (33%) and psychogenic (28.4%) causes. Chest pain due to cardiac reasons was seen in eight patients (1%). Diseases of musculoskeletal and gastrointestinal systems and psychogenic disorders were significantly more common in male and female patients, respectively (p < 0.001 for all). In winter, patients’ age and the number of patients with ≥12 years were higher than those in other seasons (p < 0.001). Most of the parents (70.8%) and patients (90.2%) thought that chest pain in their children was caused by cardiac causes.
Conclusion:Most of the diagnoses for chest pain in childhood period are benign and include the musculoskeletal system and psychogenic diseases. Although chest pain due to cardiac diseases is rare, a comprehensive analysis of medical history, detailed physical examination and cardiac imaging with echocardiography is needed to reach more accurate diagnoses.
Faecal calprotectin concentrations in neonates with CHD: pilot study
- Graeme O’Connor, Katherine L. Brown, Andrew M. Taylor
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- Published online by Cambridge University Press:
- 03 April 2020, pp. 624-628
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Neonates with CHD are at increased risk of developing necrotising enterocolitis due to mesenteric hypoperfusion. Necrotising enterocolitis results in repeated feed interruptions contributing to poor growth during the early post-operative phase. Poor weight gain and longer hospital stay are risk factors for death in neonates with CHD. Abdominal radiography is used as a diagnostic tool for necrotising enterocolitis; however, its utility is limited in the early stages of necrotising enterocolitis when pneumatosis intestinalis is absent. Calprotectin is a neutrophil activation biomarker, and elevated levels are evident in inflammatory diseases such as necrotising enterocolitis. The aim of this study was to determine whether there is a correlation between faecal calprotectin concentration and gut inflammation in neonates with CHD. This prospective single-centre study recruited newly diagnosed term patients with duct-dependent CHD between March 2018 and March 2019. Faecal calprotectin concentrations were measured in post-surgical patients using enzyme-linked immunosorbent assay methods. A total of 30 patients were included in the analysis. Calprotectin concentration for patients who developed necrotising enterocolitis was 3528 µg/g compared with 390 µg/g without, compared with 1339 µg/g in patients with suspected necrotising enterocolitis (p = 0.0001). Patients with suspected necrotising enterocolitis had a significantly longer length of hospital stay, on average 18 days longer compared to patients without necrotising enterocolitis (p = 0.03). Faecal calprotectin concentrations may reflect severity of gut inflammation in neonates with CHD. Suspected necrotising enterocolitis contributes to longer days nil by mouth and an increase in length of hospital stay.
Fontan completion during winter season is not associated with higher mortality or morbidity in the early post-operative period
- Sarah Nordmeyer, Sabeth Krettek, Johannes Nordmeyer, Marie Schafstedde, Konstantin Rehm, Joachim Photiadis, Felix Berger, Stanislav Ovroutski
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- Published online by Cambridge University Press:
- 13 April 2020, pp. 629-632
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Objectives:
The aim of our study was to compare post-operative outcome after total cavopulmonary connection between patients operated during winter and summer season.
Methods:We retrospectively studied 211 patients who underwent extracardiac total cavopulmonary connection completion at our institution between 1995 and 2015 (median age 4 (1–42) years). Seventy (33%) patients were operated during winter (November to March) and 141 (67%) patients during summer season (April to October).
Results:Patients operated during winter and summer season showed no difference in early mortality (7% versus 5%, p = 0.52) and severe morbidity like need for early Fontan takedown (1% versus 1%, p = 0.99) and need for mechanical circulatory support (9% versus 4%, p = 0.12). The post-operative course and haemodynamic outcome were comparable between both groups of patients (ICU (4 versus 3 days, p = 0.44) and hospital stay (15 versus 14 days, p = 0.28), prolonged pleural effusions (36% versus 31%, p = 0.51), need for dialysis (16% versus 11%, p = 0.37), ascites (37% versus 33%, p = 0.52), supraventricular tachyarrhythmia (16% versus 13%, p = 0.56) and chylothorax (26% versus 16%, p = 0.12), change of antibiotic treatment (47% versus 36%, p = 0.06), prolonged inotropic support (24% versus 14%, p = 0.05), intubation time (15 versus 12 hours, p = 0.33), and incidence of fast-track extubation (11% versus 22%, p = 0.06).
Conclusion:Outcomes after total cavopulmonary connection completion during winter and summer season were comparably related to mortality, severe morbidity, or longer hospital stay in the early post-operative period. These results suggest that total cavopulmonary connection completion during winter season is as safe as during summer season.
A transition clinic intervention to improve follow-up rates in adolescents and young adults with congenital heart disease
- Stephanie S. Gaydos, Shahryar M. Chowdhury, Rochelle N. Judd, Kimberly E. McHugh
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- Published online by Cambridge University Press:
- 13 April 2020, pp. 633-640
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Background:
Children with congenital heart disease (CHD) require lifelong cardiology follow-up. Many experience gaps in care around the age of transition to adult-oriented care with associated comorbidity. We describe the impact of a clinic-based intervention on follow-up rates in this high-risk population.
Methods:Patients ≥11 years seen in a paediatric outpatient CHD Transition Clinic completed self-assessment questionnaires, underwent focused teaching, and were followed on a clinic registry with assessment of care continuation. The cohort “lost to follow-up” rate, defined as absence from care at least 6 months beyond the recommended timeframe, was compared with a control group. Secondary outcomes included questionnaire scores and adult cardiology referral trends.
Results:Over 26 months, 53 participants completed an initial Transition Clinic visit; 43% (23/53) underwent a second visit. Median participant age was 18.0 years (interquartile range 16.0, 22.0). The cohort’s “lost to follow-up” rate was 7.3%, which was significantly lower than the control rate (25.9%, p < 0.01). Multivariable regression analyses demonstrated clinic participation as the only factor independently associated with follow-up rates (p = 0.048). Transition readiness was associated with older age (p = 0.01) but not sex, univentricular heart, interventional history, or surgical complexity. One-third of adult participants transferred to adult care.
Conclusions:A CHD Transition Clinic intervention can improve follow-up rates in adolescents and young adults. Age is an important factor in transition readiness, and retention of adults in paediatric care appears multi-factorial. We postulate that serial assessments of self-management, focused education, and registry utilisation may improve patient outcomes by reducing lapses in care.
Lymphopenia in adults after the Fontan operation: prevalence and associations
- Tarek Alsaied, Mathias Possner, Nicole Brown, Hassan Almeneisi, Cassandra Szugye, Andrew T. Trout, Omar Niss, Joseph J. Palermo, Faizeen Zafar, Jonathan R. Dillman, Gruschen R. Veldtman, Alexander R. Opotowsky, Adam M. Lubert
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- Published online by Cambridge University Press:
- 06 April 2020, pp. 641-648
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Lymphopenia is common in adults who have had a Fontan operation although its aetiology and clinical implications remain unknown. Previous work suggests an association between lymphopenia and both liver disease and splenomegaly. The objective of this study was to assess the prevalence of lymphopenia in adults with a Fontan circulation and evaluate its associations with risk factors and clinical outcomes. Using a retrospective cohort study design, we studied 73 adult Fontan patients (age 25.0 ± 8.4 years) who had a complete blood count and abdominal imaging performed. Patients with protein-losing enteropathy were excluded. Clinical data were extracted from hospital records. The mean white blood cell count was 6580 ± 220/ml with a mean lymphocyte count of 1223 ± 508/ml. Lymphopenia, defined as lymphocyte count <1000/ml, was present in 23 (32%) patients. Patients with lymphopenia had a lower total white blood cell count (5556 ± 2517 versus 7136 ± 1924/ml, p = 0.009) and a lower platelet count (162 ± 69 versus 208 ± 69 k/ml, p = 0.008). Lymphopenia was also associated with findings of portal hypertension, including splenomegaly (36 versus 14%, p = 0.04), varices (22 versus 6%, p = 0.04), and ascites (39 versus 14%, p = 0.02). Lymphopenia did not correlate with any cardiac imaging, haemodynamic or exercise testing variables. In conclusion, lymphopenia is common in adult Fontan patients and is associated with markers of portal hypertension. Larger studies are needed to better define the relationship between lymphopenia and clinical outcomes.
A low threshold for neonatal intervention yields a high rate of biventricular outcomes in pulmonary atresia with intact ventricular septum
- Gareth J. Morgan, Srinivas A. Narayan, Sebastian Goreczny, Henry Chubb, Thomas Krasemann, Eric Rosenthal, Shakeel A. Qureshi
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- 23 April 2020, pp. 649-655
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Aims:
Management strategies for pulmonary atresia with intact ventricular septum are variable and are based on right ventricular morphology and associated abnormalities. Catheter perforation of the pulmonary valve provides an alternative strategy to surgery in the neonatal period. We sought to assess the long-term outcome in terms of survival, re-intervention, and functional ventricular outcome in the setting of a 26-year single-centre experience of low threshold inclusion criteria for percutaneous valvotomy.
Methods and results:Retrospective analysis of patients diagnosed with pulmonary atresia with intact ventricular septum from 1990 to 2016 at a tertiary referral centre, was performed. Of 71 patients, 48 were brought to the catheterisation laboratory for intervention. Catheter valvotomy was successful in 45 patients (94%). Twenty-three patients (51%) also underwent ductus arteriosus stenting. The length of intensive care and hospital stay was significantly shorter, and early re-interventions were significantly reduced in the catheterisation group. There were eight deaths (17%); all within 35 days of the procedure. Of the survivors, only one has required a Fontan circulation. Twenty-eight patients (74%) have undergone biventricular repair and nine patients (24%) have one-and-a-half ventricle circulation. Following successful valvotomy, 80% of patients required further catheter-based or surgical interventions.
Conclusions:A low threshold for initial interventional management yielded a high rate of successful biventricular circulations. Although mortality was low in patients who survived the peri-procedural period, the rate of re-intervention remained high in all groups.
3D advanced imaging overlay with rapid registration in CHD to reduce radiation and assist cardiac catheterisation interventions
- Yousef Arar, Surendranath R. Veeram Reddy, Heidi Kim, V. Vivian Dimas, Thomas M. Zellers, Riad Abou Zahr, Ravi Vamsee, Joshua S. Greer, Animesh Tandon, Antonia Pontiki, Jeanne Dillenbeck, Luis Zabala, Gerald Greil, Alan W. Nugent, Tarique Hussain
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- 15 April 2020, pp. 656-662
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Novel commercially available software has enabled registration of both CT and MRI images to rapidly fuse with X-ray fluoroscopic imaging. We describe our initial experience performing cardiac catheterisations with the guidance of 3D imaging overlay using the VesselNavigator system (Philips Healthcare, Best, NL). A total of 33 patients with CHD were included in our study. Demographic, advanced imaging, and catheterisation data were collected between 1 December, 2016 and 31 January, 2019. We report successful use of this technology in both diagnostic and interventional cases such as placing stents and percutaneous valves, performing angioplasties, occlusion of collaterals, and guidance for lymphatic interventions. In addition, radiation exposure was markedly decreased when comparing our 10–15-year-old coarctation of the aorta stent angioplasty cohort to cases without the use of overlay technology and the most recently published national radiation dose benchmarks. No complications were encountered due to the application of overlay technology. 3D CT or MRI overlay for CHD intervention with rapid registration is feasible and aids decisions regarding access and planned angiographic angles. Operators found intraprocedural overlay fusion registration using placed vessel guidewires to be more accurate than attempts using bony structures.
Clinical significance of family history and bicuspid aortic valve in children and young adult patients with Marfan syndrome
- Emanuele Monda, Adelaide Fusco, Daniela Melis, Martina Caiazza, Felice Gragnano, Alfredo Mauriello, Annapaola Cirillo, Marta Rubino, Augusto Esposito, Angelina Grammegna, Stefano Nistri, Guglielmina Pepe, Paolo Calabrò, Pietro Strisciuglio, Alessandro Della Corte, Guido Oppido, Mariagiovanna Russo, Giuseppe Limongelli
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- 15 April 2020, pp. 663-667
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Background:
Marfan syndrome is an autosomal dominant disorder of the connective tissue, whose cardinal features affect eyes, musculoskeletal, and cardiovascular system. Despite prevalence and natural history of cardiovascular manifestation are well known in adults, little is known about children and young adult patients. The aim of this study was to describe a well-characterised cohort of consecutive children and young patients with marfan syndrome, looking at the impact of family history and presence of bicuspid aortic valve on disease severity.
Methods:A total of 30 consecutive children and young patients with Marfan syndrome were evaluated. All patients underwent a comprehensive clinical–instrumental–genetic evaluation. Particular attention was posed to identify differences in prevalence of cardiovascular abnormalities between patients with and without family history of Marfan syndrome or bicuspid aortic valve.
Results:Of these 30 patients, family history of Marfan syndrome and bicuspid aortic valve were present in 76 and 13%, respectively. Compared to patients with family history of Marfan syndrome, those without showed higher prevalence of aortic sinus dilation (87 versus 32%, p-value = 0.009), greater aortic sinus diameters (4.2 ± 2.1 versus 1.9 ± 1.1 z score, p-value = 0.002), and higher rate of aortic surgery during follow-up (37 versus 0%, p-value = 0.002). Compared to patients with tricuspid aortic valve, those with bicuspid aortic valve were younger (3.2 ± 4.3 versus 10.7 ± 6.8 years old, p-value = 0.043), showed greater aortic sinus diameters (4.2 ± 0.9 versus 2.2 ± 1.6 z score, p-value = 0.033), and underwent more frequently aortic root replacement (50 versus 4%, p-value = 0.004).
Conclusions:In our cohort of patients with Marfan syndrome, the absence of family history and the presence of bicuspid aortic valve were associated to severe aortic phenotype and worse prognosis.
Exercise capacity, physical activity, and health-related quality of life in adults with CHD
- Linda Ashman Kröönström, Åsa Cider, Anna-Klara Zetterström, Linda Johansson, Peter Eriksson, Lars Brudin, Mikael Dellborg
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- Published online by Cambridge University Press:
- 15 April 2020, pp. 668-673
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Objectives:
The aim of this study was to assess exercise capacity, physical activity, and health-related quality of life within a broad and unselected group of adults with CHD.
Design:From April 2009 to February 2014, 1310 patients were assessed for suitability to participate in this single-centre cross-sectional study. Seven hundred and forty-seven (57%) patients were included, performed a submaximal bicycle test, and answered questionnaires regarding physical activity and health-related quality of life. Exercise capacity, physical activity, and health-related quality of life were compared with reference values and correlations were studied.
Results:The exercise capacities of men and women with CHD were 58.7 and 66.3%, respectively, of reference values. Approximately, 20–25% of the patients did not achieve the recommended amount of physical activity. In addition, men scored significantly less points on 7 out of 10 scales of health-related quality of life and women in 6 out of 10 scales, compared with reference values. The strongest correlation was between exercise capacity and the Short Form-36 (physical function).
Conclusions:Exercise capacity was impaired in all adults with CHD, including those with less complicated CHD. One-quarter of the patients did not achieve the recommended levels of physical activity. Exercise tests followed by individualised exercise prescriptions may be offered to all patients with CHD aiming to increase exercise capacity, levels of physical activity, improve health-related quality of life, and reduce the risk of acquired life-style diseases.
Appropriate heart rate during exercise in Fontan patients
- Eva R. Hedlund, Liselott Söderström, Bo Lundell
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- 17 April 2020, pp. 674-680
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Objective:
To evaluate heart rate against workload and oxygen consumption during exercise in Fontan patients.
Method:Fontan patients (n = 27) and healthy controls (n = 25) underwent cardiopulmonary exercise testing with linear increase of load. Heart rate and oxygen uptake were measured during tests. Heart rate recovery was recorded for 10 minutes.
Results:Heart rate at midpoint (140 ± 14 versus 153 ± 11, p < 0.001) and at maximal effort (171 ± 14 versus 191 ± 10 beats per minute, p < 0.001) of test was lower for patients than controls. Heart rate recovery was similar between groups. Heart rate in relation to workload was higher for patients than controls both at midpoint and maximal effort. Heart rate in relation to oxygen uptake was similar between groups throughout test. Oxygen pulse, an indirect surrogate measure of stroke volume, was reduced at maximal effort in patients compared to controls (6.6 ± 1.1 versus 7.5 ± 1.4 ml·beat−1·m−2, p < 0.05) and increased significantly less from midpoint to maximal effort for patients than controls (p < 0.05).
Conclusions:Heart rate is increased in relation to workload in Fontan patients compared with controls. At higher loads, Fontan patients seem to have reduced heart rate and smaller increase in oxygen pulse, which may be explained by inability to further increase stroke volume and cardiac output. Reduced ability to increase or maintain stroke volume at higher heart rates may be an important limiting factor for maximal cardiac output, oxygen uptake, and physical performance.
Clinical efficacy and safety of switch from bosentan to macitentan in children and young adults with pulmonary arterial hypertension: extended study results
- Ebru Aypar, Dursun Alehan, Tevfik Karagöz, Hakan Aykan, İlker Ertugrul
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- 15 April 2020, pp. 681-685
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Background:
Macitentan is an orally active, potent, dual endothelin receptor antagonist and is the only registered treatment for pulmonary arterial hypertension that significantly reduced morbidity and mortality in a long-term study.
Aim:We have recently reported that switch from bosentan to macitentan significantly improved exercise capacity in children and young adults with pulmonary arterial hypertension in a 24-week prospective study and well tolerated without adverse events. We now aimed to evaluate clinical efficacy, safety of switch in a larger patient population, in a 24-month prospective study.
Methods:This is a single-institution, 24-month prospective study. Patients ≥12 years with idiopathic/heritable, pulmonary arterial hypertension, or related to CHD or residual pulmonary arterial hypertension due to repaired congenital systemic-to-pulmonary shunts and on bosentan treatment were included. Concomitant treatment with oral phosphodiesterase type 5 inhibitors/inhaled prostanoids was allowed. Outcome measures included change from baseline to 24 months, in the 6-minute walk distance, functional class, oxygen saturation at rest/after walk distance test, and natriuretic peptide levels. Safety end points included adverse events, laboratory abnormalities.
Results:Twenty-seven patients (19 adults/8 children, mean age: 21.1 ± 6.3 years (12–36), weight: 53.1 ± 15.7 kgs (26–87)) were included. Mean duration of macitentan treatment: 22.3 ± 3.9 months (9–24). Six-minute walk distance significantly improved from baseline (mean: 458 ± 79 m (300–620)) at 6 months (mean: 501 ± 73 m (325–616) + 43 m) (p < 0.05), at 12 months (mean: 514 ± 82 m (330–626) + 56 m) (p < 0.05), and at 24 months (mean: 532 ± 85 m (330–682) + 74 m) (p < 0.05). We observed a significant improvement during the first 6 months but no incremental improvement after 6 months (p > 0.05). Macitentan did not significantly change functional class, oxygen saturation, and natriuretic levels (p > 0.05). None of the patients had anaemia, hepatotoxicity, and peripheral edema.
Conclusions:Our study is the first study which showed that switch from bosentan to macitentan improved exercise capacity in children and young adults with pulmonary arterial hypertension significantly in the first 6 months and compared to baseline in 24 months and well tolerated without adverse events.
Fetal CHD and perinatal outcomes
- Christina J. Ge, Amanda C. Mahle, Irina Burd, Eric B. Jelin, Priya Sekar, Angie C. Jelin
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- 20 April 2020, pp. 686-691
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Objective:
To evaluate delivery management and outcomes in fetuses prenatally diagnosed with CHD.
Study design:A retrospective cohort study was conducted on 6194 fetuses (born between 2013 and 2016), comparing prenatally diagnosed with CHD (170) to those with non-cardiac (234) and no anomalies (5790). Primary outcomes included the incidence of preterm delivery and mode of delivery.
Results:Gestational age at delivery was significantly lower between the CHD and non-anomalous cohorts (38.6 and 39.1 weeks, respectively). Neonates with CHD had a significantly lower birth weights (p < 0.001). There was an approximately 1.5-fold increase in the rate of primary cesarean sections associated with prenatally diagnosed CHD with an odds ratio of 1.49 (95% CI 1.06–2.10).
Conclusions:Our study provides additional evidence that the prenatal diagnosis of CHD is associated with a lower birth weight, preterm delivery, and with an increased risk of delivery by primary cesarean section.
The impact of physical activity modification on the well-being of a cohort of children with an inherited arrhythmia or cardiomyopathy
- Susan Christian, Martin Somerville, Sherry Taylor, John C. Spence, Michael Giuffre, Joseph Atallah
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- 14 April 2020, pp. 692-697
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Background:
We evaluated a cohort of 35 children diagnosed with long QT syndrome, catecholaminergic polymorphic ventricular tachycardia, hypertrophic cardiomyopathy, or arrhythmogenic right ventricular cardiomyopathy with regard to physical and psychosocial well-being.
Material and Methods:Patients wore an accelerometer to record their time involved in moderate- to vigorous-intensity physical activity and completed the Pediatric Quality of Life Inventory and the Pediatric Cardiac Quality of Life Inventory. Parents were also asked to describe if their child had changed their physical activity because of their diagnosis and how difficult and upsetting it was for the child to adapt to the physical activity recommendations.
Results:Patients were involved in less moderate- to vigorous-intensity physical activity per day (35 min/day versus 55 min/day) and had lower Pediatric Quality of Life Inventory total health scores (79 versus 84) compared to normative data. Overall, 51% of the cohort modified their physical activity in some way because of their diagnosis and changing physical activity was associated with lower Pediatric Quality of Life Inventory and Pediatric Cardiac Quality of Life Inventory scores.
Conclusion:Our cohort was involved in less moderate- to vigorous-intensity physical activity and had lower Pediatric Quality of Life Inventory total health scores compared to normative paediatric data. Modifying one’s physical activity was associated with worse health-related quality of life scores, highlighting a vulnerable sub-group of children. These findings are useful for families and healthcare professionals caring for children who are adjusting to a new cardiac diagnosis of an inherited arrhythmia or cardiomyopathy.
Treatment strategies for protein-losing enteropathy in Fontan-palliated patients
- Anastasia Schleiger, Stanislav Ovroutski, Björn Peters, Stephan Schubert, Joachim Photiadis, Felix Berger, Peter Kramer
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- Published online by Cambridge University Press:
- 04 May 2020, pp. 698-709
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Objective:
Protein-losing enteropathy is an infrequent but severe condition occurring after Fontan procedure. The multifactorial pathogenesis remains unclear and no single proposed treatment strategy has proven universally successful. Therefore, we sought to describe different treatment strategies and their effect on clinical outcome and mortality.
Material and Methods:We performed a retrospective observational study. From the total cohort of 439 Fontan patients treated in our institution during the study period 1986–2019, 30 patients (6.8%) with protein-losing enteropathy were identified. Perioperative, clinical, echocardiographic, laboratory, and invasive haemodynamic findings and treatment details were analysed.
Results:Median follow-up after disease onset was 13.1 years [interquartile range 10.6]. Twenty-five patients received surgical or interventional treatment for haemodynamic restrictions. Medical treatment, predominantly pulmonary vasodilator and/or systemic anti-inflammatory therapy with budesonide, was initiated in 28 patients. In 15 patients, a stable remission could be achieved by medical or surgical procedures (n = 3 each), by combined multimodal therapy (n = 8), or ultimately by cardiac transplantation (n = 1). Phrenic palsy, bradyarrhythmia, Fontan pathway stenosis, and absence of a fenestration were significantly associated with development of protein-losing enteropathy (p = 0.001–0.48). Ten patients (33.3%) died during follow-up; 5-year survival estimate was 96.1%. In unadjusted analysis, medical therapy with budesonide and pulmonary vasodilator therapy in combination was associated with improved survival.
Conclusions:Protein-losing enteropathy is a serious condition limiting survival after the Fontan procedure. Comprehensive assessment and individual treatment strategies are mandatory to achieve best possible outcome. Nevertheless, relapse is frequent and long-term mortality substantial. Cardiac transplantation should be considered early as treatment option.
Exercise echocardiography demonstrates potential myocardial damage in patients with repaired tetralogy of Fallot using layer-specific strain analysis
- Kana Yazaki, Ken Takahashi, Maki Kobayashi, Mariko Yamada, Takeshi Iso, Satoshi Akimoto, Sachie Shigemitsu, Kotoko Matsui, Katsumi Akimoto, Masahiko Kishiro, Keisuke Nakanishi, Shiori Kawasaki, Toshiaki Shimizu
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- 04 May 2020, pp. 710-716
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Introduction:
Exercise stress echocardiography and layer-specific strains are emerging as important tools for cardiac assessment. This study was aimed to evaluate layer-specific strains and torsion parameters during exercise in order to investigate the characteristics of cardiac dysfunction in patients with repaired tetralogy of Fallot and to detect subclinical left ventricular dysfunction.
Materials and Methods:Thirteen patients with repaired tetralogy of Fallot (median age, 17.3 [interquartile range, 14.5–22.9] years; 6 males) and 13 controls (median age, 28.5 [interquartile range, 27.6–31.6] years; 13 males) underwent echocardiography at rest and during supine exercise. Layer-specific longitudinal strain and circumferential strain of three myocardial layers (endocardium, midmyocardium, and epicardium), torsion, and untwisting rate were measured using two-dimensional speckle-tracking echocardiography.
Results:Peak endocardial papillary circumferential strain (−21.1 ± 2.6% vs. −25.8 ± 3.8%, p = 0.007), midmyocardial apical circumferential strain (−11.1 ± 4.0% vs. −15.6 ± 3.2%, p = 0.001), epicardial apical circumferential strain (−11.1 ± 4.0% vs. −15.6 ± 3.2%, p = 0.021), and torsion (8.9 ± 6.0 vs. 14.9 ± 4.8 degree, p = 0.021) were significantly lower in the repaired tetralogy of Fallot group than in the control group during exercise, though no significant difference was found between patients and controls at rest.
Conclusions:Analysis of layer-specific strains and torsion parameters during exercise could detect subclinical left ventricular dysfunction in patients with repaired tetralogy of Fallot, which might reflect potential myocardial damage, at a stage where these parameters have normal values at rest. This finding provides new insight into the mechanisms of cardiac dysfunction in patients with repaired tetralogy of Fallot.
Are there any novel markers in acute rheumatic fever: neutrophil-to-lymphocyte ratio, platelet-to-lymphocyte ratio, and monocyte-to-lymphocyte ratio: Novel Indexes in Acute Rheumatic Fever?
- Dilek Giray, Olgu Hallioglu
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- 04 May 2020, pp. 717-721
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Objective:
The aim of this study was to investigate the relationship between C-reactive protein and erythrocyte sedimentation rate and neutrophil-to-lymphocyte, platelet-to-lymphocyte, and monocyte-to-lymphocyte ratios in acute rheumatic fever in children.
Method:In this retrospective study, 182 patients with acute rheumatic fever and 173 controls were included. Complete blood count parameters, and neutrophil-to-lymphocyte, monocyte-to-lymphocyte, and platelet-to-lymphocyte ratios were recorded for all the patients underwent transthoracic echocardiography.
Results:Neutrophil-to-lymphocyte, monocyte-to-lymphocyte, and platelet-to-lymphocyte ratios were significantly higher in patients with rheumatic heart disease than patients without cardiac involvement (p < 0.05). C-reactive protein and erythrocyte sedimentation rate levels were found to have a positive correlation with neutrophil-to-lymphocyte (r = 0.228, p = 0.001; r = 0.355, p = 0.001), platelet-to-lymphocyte (r = 0.227, p = 0.01; r = 0.149, p = 0.005), and monocyte-to-lymphocyte ratios (r = 0.117, p = 0.005; r = 0.107, p = 0.044). Cardiac involvement was present in 152 (83.5%) of the patients. Neutrophil-to-lymphocyte, monocyte-to-lymphocyte, and platelet-to-lymphocyte ratios were significantly higher in patients with rheumatic heart disease than patients without cardiac involvement (p < 0.05). Patients with carditis were grouped according to mitral, aortic, or both valve involvement but there was no significant difference between the groups with respect to neutrophil-to-lymphocyte, monocyte-to-lymphocyte, and platelet-to-lymphocyte ratios. In addition, neutrophil-to-lymphocyte and monocyte-to-lymphocyte ratios were significantly higher in patients with Sydenham’s chorea than without chorea (p < 0.05).
Conclusion:Neutrophil-to-lymphocyte, platelet-to-lymphocyte, and monocyte-to-lymphocyte ratios may help make the diagnosis of acute rheumatic fever and its prognosis by serial measurements in follow-up but none of them tell us the severity of carditis. Also, this is the first study showing the positive correlation between Sydenham’s chorea and neutrophil-to-lymphocyte and monocyte-to-lymphocyte ratios. Further studies are needed to confirm this hypothesis, as this is the first study in the literature on this topic.
Images in Congenital Cardiac Disease
Mediastinal mass after a Blalock–Taussig shunt: utility of CT angiography
- Valeria Peña-Trujillo, Sebastian Gallo-Bernal, Julian F Forero Melo
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- Published online by Cambridge University Press:
- 28 April 2020, pp. 722-723
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Pseudoaneurysms are rare complications of Blalock–Taussig fistulas. We present the case of an abscessed right pulmonary aneurysm after a Blalock–Taussig fistula in the context of a Salmonella bacteremia.
Brief Report
Post-operative Brugada electrocardiographic pattern, polymorphic ventricular tachycardia, and sudden death in a child after administration of propofol anaesthesia
- Duygu Uzun, Nimra Hassan, Utkarsh Kohli
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- Published online by Cambridge University Press:
- 27 March 2020, pp. 724-727
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A 9-year-old African-American girl presented with sudden cardiac arrest a few hours after adenotonsillectomy. She received anaesthesia which included propofol during the procedure. Her electrocardiogram (EKG) showed type 1 Brugada pattern, and genetic testing revealed a variant of unknown significance in desmoplakin (DSP) gene. We discuss the association between propofol, Brugada EKG pattern, and malignant ventricular arrhythmias.