Original Article
Aorto-left ventricular tunnel: case series of a rare disease
- Obayda M. Diraneyya, Fahad Alhabshan, Abdullah Alghamdi, Hussein Moafa, Mohammed Alnasef, Mohamed S. Kabbani
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- Published online by Cambridge University Press:
- 19 October 2020, pp. 47-51
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Introduction:
Aorto-left ventricular tunnel is a rare disease that can cause significant morbidity early in life due to volume overload and left ventricular failure. Surgical intervention is usually curative with minimal early complications. However, long-term effects and outcome are not fully determined.
Objective:We are reporting a case series of this rare CHD with its long-term outcome.
Methods:We conducted a retrospective analysis of all children from birth to 14 years of age who were admitted between 2001 and 2020 with the diagnosis of aorto-left ventricular tunnel. Demographic, echocardiographic, and perioperative data were collected and reviewed. The pre-operative data were compared with data reviewed on the last outpatient follow-up.
Results:Total of five patients fulfilled our inclusion criteria. Three patients were diagnosed after auscultating an incidental murmur, one had symptoms of congestive heart failure, and one had an abnormal fetal echocardiogram. Echocardiography demonstrated stenotic and regurgitant aortic valve with severely depressed left ventricle function in two patients, one of them with also single left coronary artery. The other three patients had normal aortic valve structure and normal ventricular function. All five patients had surgical repair, two by patch closure at aortic end of aorto-left ventricular tunnel, two by patch closure at both aortic and left ventricular ends, and one by aortic root replacement using a homograft. During follow-up, there was no residual aorto-left ventricular tunnel in any of our five cases, two had moderate aortic regurgitation and one had moderate residual aortic stenosis.
Conclusions:Spectrum of presentation for aorto-left ventricular tunnel varies from an occult lesion to frank left heart failure due to volume or less commonly, pressure overload. Early surgical repair is recommended and is usually associated with complete resolution. Long-term follow-up is recommended for aortic root dilatation and aortic valve competency, as valve function need to be addressed in a timely manner to avoid further complications.
Characteristics of antegrade-only accessory pathways in children and adolescents
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- Minh B. Nguyen, Allison C. Hill, Yaniv Bar-Cohen, Michael J. Silka
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- Published online by Cambridge University Press:
- 25 January 2021, pp. 1258-1262
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There is minimal data regarding antegrade-only accessory pathways in young patients. Given evolving recommendations and treatments, retrospective analysis of the clinical and electrophysiologic properties of antegrade-only pathways in patients <21 years old was performed, with subsequent comparison of electrophysiology properties to age-matched controls with bidirectional pathways. Of 522 consecutive young patients with ventricular pre-excitation referred for electrophysiology study, 33 (6.3%) had antegrade-only accessory pathways. Indications included palpitations (47%), chest pain (25%), and syncope (22%). The shortest value for either the accessory pathway effective refractory period or the pre-excited R-R interval was taken for each patient, with the median of the antegrade-only group significantly greater than shortest values for the bidirectional group (310 [280–360] ms versus 270 [240–302] ms, p < 0.001). However, the prevalence of pathways with high-risk properties (effective refractory period or shortest pre-excited R-R interval <250 ms) was similar in both study patients and controls (13% versus 21%) (p = 0.55). Sixteen patients had a single antegrade-only accessory pathway and no inducible arrhythmia. Six patients had Mahaim fibres, all right anterolateral with inducible antidromic reciprocating tachycardia. However, 11 patients with antegrade-only accessory pathways and 3 with Mahaim fibres had inducible tachycardia due to a second substrate recognised at electrophysiology study. These included concealed accessory pathways (7), bidirectional accessory pathways (5), and atrioventricular node re-entry (2). Antegrade-only accessory pathways require comprehensive electrophysiology evaluation as confounding factors such as high-risk conduction properties or inducible Supraventricular Tachycardia (SVT) due to a second substrate of tachycardia are often present.
The different surgical impact of the superior cavoatrial incision in children and adults
- Lamia Ait-Ali, Antonio Ravaglioli, Pierluigi Festa, Alessandro Tamburrini, Chiara Marrone, Magdalena Cuman, Cecilia Farnetani, Vitali Pak, Mario Chiavarelli, Duccio Federici
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- Published online by Cambridge University Press:
- 15 December 2020, pp. 751-755
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Introduction:
The single- and double-patch repairs are undoubtedly the most commonly used techniques for the surgical management of partial anomalous pulmonary venous connection associated with sinus venosus atrial septal defect. The aim of this study was to retrospectively compare early and long-term surgical outcomes in paediatric and adult patients, focusing in particular on the occurrence of ectopic atrial rhythm.
Material and methods:Seventy patients (male: 38, 54.2%) underwent surgical repair for partial anomalous pulmonary venous connection with sinus venosus atrial septal defect. Forty-nine patients (70%) underwent surgical repair in paediatric age (<16 years old), while 21 of (30%) patients were operated in adulthood. Thirty patients (42.8%) underwent single-patch repair and 39 patients (55.7%) underwent double-patch repair. In only one patient, the Warden procedure was performed (1.4%). Median follow-up time was 52 months (IQ 15.1–113).
Results:The type of surgical technique didn’t affect the incidence of ectopic atrial rhythm (26.6% in single-patch group and 25.6% in double-patch groups, p = 0.9). At long-term follow-up, ectopic atrial rhythm, as an expression of sinoatrial node disturbance, was however significantly more frequent in the paediatric population (28.8% paediatric group and 4.7% adult group, p = 0.02).
Conclusions:The higher incidence of ectopic atrial rhythm in children is probably related to the closer position of the sinus node to the superior cavoatrial incision, which makes irreversible iatrogenic traumatism more likely to occur. Surgical techniques that avoid any manipulation on the superior cavoatrial junction should, therefore, be preferred for children undergoing partial anomalous pulmonary venous connection repair.
Editorial
How should we measure research impact in paediatric cardiology?
- Kevin D. Hill, Kathy J. Jenkins
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- Published online by Cambridge University Press:
- 08 July 2021, pp. 1205-1206
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Original Article
Does obesity influence ventricular repolarisation in children?
- Nihan Yıldırım Yıldız, Tayfun Uçar, Mehmet G. Ramoğlu, Merih Berberoğlu, Zeynep Şıklar, Ercan Tutar, Semra Atalay
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- Published online by Cambridge University Press:
- 14 December 2020, pp. 568-576
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Objective:
Ventricular repolarisation changes may lead to sudden cardiac death in obese individuals. We aimed to investigate the relationship between ventricular repolarisation changes, echocardiographic parameters, anthropometric measures, and metabolic syndrome laboratory parameters in obese children.
Methods:The study involved 81 obese and 82 normal-weight healthy children with a mean age of 12.3 ± 2.7 years. Anthropometric measurements of participants were evaluated according to nomograms. Obese patients were subdivided into two groups; metabolic syndrome and non-metabolic syndrome obese. Fasting plasma glucose, fasting insulin, and lipid profile were measured. QT/QTc interval, QT/QTc dispersions were measured, and left ventricular systolic and diastolic measurements were performed.
Results:Body weight, body mass index, relative body mass index, waist/hip circumference ratio, and systolic and diastolic blood pressures were significantly higher in obese children. QT and QTc dispersions were significantly higher in obese children and also obese children with metabolic syndrome had significantly higher QT and QTc dispersions compared to non-metabolic syndrome obese children (p < 0.001) and normal-weight healthy children (p < 0.001). Waist/hip circumference ratio, body mass index, and relative body mass index were the most important determinant of QT and QTc dispersions. Left ventricular wall thickness (left ventricular posterior wall thickness at end-diastole, left ventricular posterior wall thickness at end-systole, interventricular septal thickness at end-diastole) and left ventricular mass index were significantly higher and ejection fraction was lower in obese children. Left ventricular mass index and interventricular septal thickness at end-diastole were positively correlated with QT and QTc dispersions.
Conclusions:Our study demonstrated that QT/ QTc interval prolongation and increase in QT and QTc dispersion on electrocardiogram may be found at an early age in obese children.
The Michigan Appropriateness Guide for Intravenous Catheters in children with congenital heart disease: miniMAGIC-CHD
- Tanya Perry, Amanda J Ullman, Ranjit Aiyagari, Stephanie Pitts, Jeffrey P Jacobs, David S Cooper
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- Published online by Cambridge University Press:
- 19 March 2021, pp. 1814-1818
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Background:
The approach to vascular access in children with CHD is a complex decision-making process that may have long-term implications. To date, evidence-based recommendations have not been established to inform this process.
Methods:The RAND/UCLA Appropriateness Method was used to develop miniMAGIC, including sequential phases: definition of scope and key terms; information synthesis and literature review; expert multidisciplinary panel selection and engagement; case scenario development; and appropriateness ratings by expert panel via two rounds. Specific recommendations were made for children with CHD.
Results:Recommendations were established for the appropriateness of the selection, characteristics, and insertion technique of intravenous catheters in children with CHD with both univentricular and biventricular physiology.
Conclusion:miniMAGIC-CHD provides evidence-based criteria for intravenous catheter selection for children with CHD.
Marked skeletal muscle deficits are associated with 6-minute walk distance in paediatric pulmonary hypertension
- Catherine M. Avitabile, Sofia Saavedra, Nithya Sivakumar, Elizabeth Goldmuntz, Stephen M. Paridon, Babette S. Zemel
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- Published online by Cambridge University Press:
- 11 February 2021, pp. 1426-1433
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Background:
Poor growth is common in children with pulmonary hypertension; however, skeletal muscle deficits have not been described and the association between muscle deficits and functional status is unknown.
Methods:Patients aged 8–18 years with pulmonary hypertension (diagnostic Groups 1, 2, or 3) and World Health Organization functional class I or II underwent dual-energy absorptiometry to measure leg lean mass Z-score (a surrogate for skeletal muscle). Muscle strength was assessed using dynamometry. Physical activity questionnaires were administered. Clinical data, including 6-minute walk distance, were reviewed. Relationships between skeletal muscle, physical activity score, and 6-minute walk distance were assessed by correlations and linear regression.
Results:Sixteen patients (12.1 ± 3.2 years, 50% female, 56% Group 1, 56% functional class II) were enrolled. Leg lean mass Z-score was significantly less than reference data (−1.40 ± 1.12 versus 0.0 ± 0.9, p < 0.001) and worse in those with functional class II versus I (−2.10 ± 0.83 versus −0.50 ± 0.73, p < 0.01). Leg lean mass Z-score was positively associated with right ventricular systolic function by tricuspid annular plane systolic Z-score (r = 0.54, p = 0.03) and negatively associated with indexed pulmonary vascular resistance (r = −0.78, p < 0.001). Leg lean mass Z-score and forearm strength were positively associated with physical activity score. When physical activity score was held constant, leg lean mass Z-score independently predicted 6-minute walk distance (R2 = 0.39, p = 0.03).
Conclusions:Youth with pulmonary hypertension demonstrate marked skeletal muscle deficits in association with exercise intolerance. Future studies should investigate whether low leg lean mass is a marker of disease severity or an independent target that can be improved.
Ventricular septal defect: diagnosis and treatments in the neonates: a systematic review
- Ahmed Adan, Loay Eleyan, Mariam Zaidi, Amr Ashry, Ram Dhannapuneni, Amer Harky
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- 17 December 2020, pp. 756-761
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Background:
Medical advancements have encouraged minimally invasive surgical repair of congenital heart defects such as ventricular septal defects (VSDs), and the diagnostic process can now be carried out using non-traditional techniques such as pulse oximetry. This, in turn, has improved clinical outcomes with reduced complication rates post-surgery. However, the variations in type of VSDs, age of patient, comorbidities, and access to closure devices may limit the efficacy of surgical advancements.
Methods:Articles were identified amongst Scopus, MEDLINE, and PubMed using various relevant search strings using PRISMA guidelines. Of the 115 articles initially extracted, 10 were eventually reviewed after duplicates and irrelevant studies were removed.
Results:Of the 24 eligible articles, 10 papers were selected for analysis. Minimally invasive approaches to VSD repair was associated with satisfactory short-term outcomes when compared to open repair. For diagnosis of congenital VSD, whilst recent advances such as pulse oximetry method and genome analysis are more sensitive, the limited availability and access to such investigatory methods must be recognised.
Conclusion:Pulse oximetry and fetal echocardiography are established non-invasive diagnostic tools for VSD. The recent advances in minimally invasive treatment options including periventricular approach and transcatheter techniques have improved patient outcomes, yet at the expense of higher residual rates. Careful patient selection for each technique and follow-up should be planned through multidisciplinary team meetings.
N-terminal pro-B-type-natriuretic peptide as a screening tool for pulmonary hypertension in the paediatric population
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- Soham Dasgupta, Erika Bettermann, Michael Kelleman, Usama Kanaan, Ritu Sachdeva, Christopher Petit, Dennis Kim, Robert Vincent, Holly Bauser-Heaton
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- Published online by Cambridge University Press:
- 02 March 2021, pp. 1595-1607
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Background:
Although cardiac catheterisation (cath) is the diagnostic test for pulmonary hypertension, it is an invasive procedure. Echocardiography (echo) is commonly used for the non-invasive diagnosis of pulmonary hypertension but maybe limited by lack of adequate signals. Therefore, emphasis has been placed on biomarkers as a potential diagnostic tool. No prior paediatric studies have simultaneously compared N-terminal pro-B-type-natriuretic peptide (NTproBNP) with cath/echo as a potential diagnostic tool. The aim of this study was to determine if NTproBNP was a reliable diagnostic tool for pulmonary hypertension in this population.
Methods:Patients were divided into Study (echo evidence/established diagnosis of pulmonary hypertension undergoing cath) and Control (cath for small atrial septal defect/patent ductus arteriosus and endomyocardial biopsy post cardiac transplant) groups. NTproBNP, cath/echo data were obtained.
Results:Thirty-one patients met inclusion criteria (10 Study, 21 Control). Median NTproBNP was significantly higher in the Study group. Echo parameters including transannular plane systolic excursion z scores, pulmonary artery acceleration time and right ventricular fractional area change were lower in the Study group and correlated negatively with NTproBNP. Receiver operation characteristic curve analysis demonstrated NTproBNP > 389 pg/ml was 87% specific for the diagnosis of pulmonary hypertension with the addition of pulmonary artery acceleration time improving the specificity.
Conclusions:NTproBNP may be a valuable adjunctive diagnostic tool for pulmonary hypertension in the paediatric population. Echo measures of transannular plane systolic excursion z score, pulmonary artery acceleration time and right ventricular fractional area change had negative correlations with NTproBNP. The utility of NTproBNP as a screening tool for pulmonary hypertension requires validation in a population with unknown pulmonary hypertension status.
The intraoperative use of recombinant activated factor VII in arterial switch operations
- Jessica Zink, Zachary A. Spigel, Christopher Ibarra, Erin A. Gottlieb, Iki Adachi, Carlos M. Mery, Michiaki Imamura, Jeffrey S. Heinle, Emmett Dean McKenzie, Charles D. Fraser, Ziyad M. Binsalamah
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- Published online by Cambridge University Press:
- 19 November 2020, pp. 386-390
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Background:
The rate of bleeding complications following arterial switch operation is too low to independently justify a prospective randomised study for benefit from recombinant factor VIIa. We aimed to evaluate factor VIIa in a pilot study.
Methods:We performed a retrospective cohort study of patients undergoing arterial switch operation from 2012 to 2017. Nearest-neighbour propensity score matching on age, gender, weight, and associated cardiac defects was used to match 27 controls not receiving recombinant factor VIIa to 30 patients receiving recombinant factor VIIa. Fisher’s exact test was performed to compare categorical variables. Wilcoxon’s rank-sum test was used to compare continuous variables between cohorts.
Results:Post-operative thrombotic complications were not associated with factor VIIa administration (Odds Ratio (OR) 0.28, 95% CI 0.005–3.77, p = 0.336), nor was factor VIIa administration associated with any re-explorations for bleeding. No intraoperative transfusion volumes were different between the recombinant factor VIIa cohort and controls. Post-operative prothrombin time (10.8 [10.3–12.3] versus 15.9 [15.1–17.2], p < 0.001) and international normalised ratio (0.8 [0.73–0.90] versus 1.3 [1.2–1.4], p < 0.001]) were lower in recombinant factor VIIa cohort relative to controls.
Conclusions:In spite of a higher post-bypass packed red blood cell transfusion requirement, patients receiving recombinant factor VIIa had a similar incidence of bleeding post-operatively. With no difference in thrombotic complications, and with improved post-operative laboratory haemostasis, a prospective randomised study is warranted to evaluate recombinant factor VIIa.
Midterm outcomes of pulmonary artery sling repair with and without tracheoplasty
- Ziyad M. Binsalamah, Alyssa Thomason, Christopher Ibarra, Zachary Spigel, Iki Adachi, Katherine E. Barton, Evan Edmunds, Christopher A. Caldarone, Michiaki Imamura, Jeffrey S. Heinle
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- Published online by Cambridge University Press:
- 13 October 2020, pp. 52-59
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Objective:
Review a single-centre experience with pulmonary artery sling repair and evaluate risk factors for re-intervention.
Methods:Patients with surgically repaired pulmonary artery sling at a single institution between 1996 and 2018 were retrospectively reviewed. A univariate Cox regression analysis was used to evaluate variables for association with freedom from re-intervention.
Results:Eighteen patients had pulmonary artery sling repair. At operation, median age and weight were 6.9 months (interquartile range 4.1–18.1) and 9.5 kg (interquartile range 6.5–14.5), respectively. A median hospital length of stay was 12 days (interquartile range 5.8–55.3). Twelve patients (67%) had complete tracheal rings, of whom six (50%) underwent tracheoplasty (five concurrently with pulmonary artery sling repair). Airway re-intervention was required in five (83%) of the six patients who underwent tracheoplasty. One patient had intraoperative diagnosis and repair of pulmonary artery sling during unrelated lesion repair and required tracheoplasty 24 days post-operatively. One patient died 55 days after pulmonary artery sling repair and tracheoplasty following multiple arrests and re-interventions. Median post-operative follow-up for surviving patients was 6.3 years (interquartile range 11 months–13 years), at which time freedom from re-intervention was 61%. When controlling for patient and tracheal size, initial tracheoplasty was associated with decreased freedom from re-intervention (hazard ratio 21.9, 95% confidence interval 1.7–284.3, p = 0.018).
Conclusions:In patients with pulmonary artery sling, tracheoplasty is associated with decreased freedom from re-intervention. In select patients with pulmonary artery sling and complete tracheal rings, conservative management without tracheoplasty is feasible. Further study is necessary to delineate objective indications for tracheoplasty.
Reproductive health conversations in adolescents with CHD
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- Shannon M. Lyon, Amy J. Katz, Anne G. Farrell, Marcia L. Shew
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- 15 February 2021, pp. 1263-1268
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Background:
Reproductive issues as related to CHD must be discussed in the clinic and at home. Providers can ensure that correct information is imparted to the adolescent and encourage mothers to provide support and guidance to the adolescent. The level to which these conversations occur is unknown.
Methods:A survey distributed to female adolescent/mother dyads assessed self-reported conversations with the healthcare provider and between each other about reproductive health topics. A clinician survey was completed to assess CHD diagnosis, risk of hormonal contraception, and pregnancy risk.
Results:Among 91 dyads, 33.0% of adolescents and 42.9% of mothers reported discussing recurrence risk of CHD with the provider. In regard to the cardiac lesion affecting a baby, 30.7% of adolescents and 28.7% of mothers reported discussing this with a provider. Significantly less adolescents and mothers reported discussing the risks of hormonal contraception and pregnancy with a provider. In assessing conversations between adolescents and mothers, only 44.2% of adolescents and 52.3% of mothers reported discussing with each other the safety of using birth control and 46.5% of adolescents and 64.0% of mothers reported discussing the safety of pregnancy.
Conclusions:Adolescents with CHD and their mothers report low rates of reproductive health-related conversations with the healthcare provider, and mothers report low rates of having these conversations with their daughters. These topics should be discussed at each appointment with the cardiologist and must be encouraged to continue at home.
Differences in perceptions of transition readiness between parents and teens with congenital heart disease: do parents and teens agree?
- David Harrison, Michelle Gurvitz, Sunkyung Yu, Ray E. Lowery, Katherine Afton, Angela Yetman, Jonathan Cramer, Nancy Rudd, Scott Cohen, Russell Gongwer, Karen Uzark
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- Published online by Cambridge University Press:
- 11 January 2021, pp. 957-964
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Background:
Amongst patients with CHD, the time of transition to adulthood is associated with lapses in care leading to significant morbidity. The purpose of this study was to identify differences in perceptions between parents and teens in regard to transition readiness.
Methods:Responses were collected from 175 teen–parent pairs via the validated CHD Transition Readiness survey and an information request checklist. The survey was distributed via an electronic tablet at a routine clinic visit.
Results:Parents reported a perceived knowledge gap of 29.2% (the percentage of survey items in which a parent believes their teen does not know), compared to teens self-reporting an average of 25.9% of survey items in which they feel deficient (p = 0.01). Agreement was lowest for long-term medical needs, physical activities allowed, insurance, and education. In regard to self-management behaviours, agreement between parent and teen was slight to moderate (weighted κ statistic = 0.18 to 0.51). For self-efficacy, agreement ranged from slight to fair (weighted κ = 0.16 to 0.28). Teens were more likely to request information than their parents (79% versus 65% requesting at least one item) particularly in regard to pregnancy/contraception and insurance.
Conclusion:Parents and teens differ in several key perceptions regarding knowledge, behaviours, and feelings related to the management of heart disease. Specifically, parents perceive a higher knowledge deficit, teens perceive higher self-efficacy, and parents and teens agree that self-management is low.
T1 mapping and conditional survival in paediatric dilated cardiomyopathy with advanced heart failure
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- Shashi Raj, Richa Kothari, N Arun Kumar, Alben Sigamani, Vimal Raj
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- 08 April 2021, pp. 1938-1942
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Myocardial fibrosis is associated with adverse events in idiopathic dilated cardiomyopathy. Cardiac MRI with late gadolinium enhancement can detect myocardial fibrosis. We evaluated the conditional survival of children and adolescents based on native T1 mapping (combined proton signal from myocytes and interstitium prior to contrast administration by the measurement of myocardial and blood relaxation time) as a means to assess myocardial fibrosis. This retrospective case–cohort over a 3-year period included all consecutive patients (aged ≤ 21 years) with advanced heart failure from dilated cardiomyopathy (echocardiographic left ventricular ejection fraction ≤ 45% and NYHA class ≥ 2) who underwent cardiac MRI.
Conditional survival (follow-up ≥ 6 months after cardiac MRI) was assessed to include NYHA functional class and time to event (death or heart transplantation). A total of 57 patients (mean age 11.7 ± 6.1 years; 58% male) had a median NYHA Class III (31/57) and median left ventricular ejection fraction 25% (20–38%). Survival data were available in 82% patients (46/57) and the crude mortality rate was 24% (11/46) and one patient (2%) underwent heart transplantation. The median native T1 was elevated at 1351 ms (95% CI 1332, 1394) and it showed no difference between the groups who survived to those who died. Performing a multilevel regression analysis on prognosis failed to predict 6-month conditional survival.
Impact of peak respiratory exchange ratio on the prognostic power of symptoms-limited exercise testing using Bruce protocol in patients with Fontan physiology
- Jianli Niu, Aliana Godoy, Talya Kadish, Bibhuti B. Das
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- Published online by Cambridge University Press:
- 19 November 2020, pp. 216-223
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Objectives:
We evaluated the impact of peak respiratory exchange ratio on the prognostic values of cardiopulmonary exercise variables during symptoms-limited incremental exercise tests in patients with Fontan physiology.
Methods:Retrospective single-centre chart review study of Fontan patients who underwent exercise testing using the Bruce protocol between 2014 and 2018 and follow-up.
Results:A total of 34 patients (age > 18 years) had a Borg score of ≥7 on the Borg 10-point scale, but only 50% of patients achieved a peak respiratory exchange ratio of ≥ 1.10 (maximal test). Peak oxygen consumption, percent-predicted peak oxygen consumption, and peak oxygen consumption at the ventilatory threshold was reduced significantly in patients with a peak respiratory exchange ratio of < 1.10. Peak oxygen consumption and percent-predicted peak oxygen consumption was positively correlated with peak respiratory exchange ratio values (r = 0.356, p = 0.039). After a median follow-up of 21 months, cardiac-related events occurred in 16 (47%) patients, with no proportional differences in patients due to their respiratory exchange ratio (odds ratio, 0.62; 95% CI: 0.18–2.58; p = 0.492). Multivariate Cox proportional hazard analysis showed percent-predicted peak oxygen consumption, peak heart rate, and the oxygen uptake efficient slope were highly related to the occurrence of events in patients only with a peak respiratory exchange ratio of ≥ 1.10.
Conclusions:The value of peak cardiopulmonary exercise variables is limited for the determination of prognosis and assessment of interventions in Fontan patients with sub-maximal effort. Our findings deserve further research and clinical application.
Usefulness of ischemia-modified albumin for assessment of the effects of small ventricular septal defects on the pulmonary vascular bed
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- Pinar Dervisoglu, Taliha Oner
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- Published online by Cambridge University Press:
- 22 March 2021, pp. 1207-1212
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Background:
Pulmonary vascular damage may be associated with oxidative stress in congenital heart diseases. We investigated whether small ventricular septal defects have an effect on the pulmonary bed.
Methods:This prospective cohort study included 100 patients with small ventricular septal defects and 75 healthy controls. Ischemia-modified albumin, high-sensitivity C-reactive protein, and various cardiovascular parameters were assessed in both groups.
Results:The mean ischemia-modified albumin level was significantly higher in patients with small ventricular septal defects (0.62 ± 0.17 absorbance units) than in the control group (0.51 ± 0.09 absorbance units; p < 0.001). The mean high-sensitivity C-reactive protein level was significantly higher in the ventricular septal defects group (3.72 ± 1.57) than in the control group (2.45 ± 0.89; p < 0.001). The ischemia-modified albumin levels in patients with left ventricular internal diameter end diastole and end sistole and main pulmonary artery z-scores ≥ 2 were significantly higher than patients whose z-scores were <2. The ischemia-modified albumin and high-sensitivity C-reactive protein levels were positively correlated in the small ventricular septal defects group (rho = 0.742, p < 0.001). Receiver operating characteristic analyses showed that at the optimal cut-off value of ischemia-modified albumin for the prediction of pulmonary involvement was 0.55 absorbance units with a sensitivity of 60%, specificity of 62% (area under the curve = 0.690, p < 0.001).
Conclusions:We demonstrated the presence of oxidative stress and higher ischemia-modified albumin levels in small ventricular septal defects, suggesting that ischemia-modified albumin might be a useful biomarker for evaluating the effects of small ventricular septal defects on the pulmonary bed.
Echinococcosis in both heart and lungs, the first case reported in Albania
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- Numila Maliqari, Loreta Teneqexhi, Albert Koja, Altin Veshti, Arben Baboci, Albana Haxhiu, Saimir Kuci, Alfred Ibrahimi, Durim Cela, Vittorio Vanini
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- Published online by Cambridge University Press:
- 23 March 2021, pp. 1819-1822
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An 11-year-old male was admitted with cough and fever for the last 4 days and also complained of pain in the right lung for some weeks. The boy did not show any other symptoms and his past medical history was unremarkable as well. The radiologist findings showed an aspect that suggested for echinococcosis.
At first, it was realised the heart intervention. About a 2-month period later, the child underwent another cyst removal in lung. He had begun taking albendazole 5 days before the heart intervention. The therapy was continued until the lung intervention and for 12 weeks post-operatively. The patient had an uneventful recovery and after about 4 years.
Individual and joint effects of genetic polymorphisms in microRNA-machinery genes on congenital heart disease susceptibility
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- Andrea Borghini, Cecilia Vecoli, Antonella Mercuri, Stefano Turchi, Maria Grazia Andreassi
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- Published online by Cambridge University Press:
- 11 January 2021, pp. 965-968
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Single-nucleotide polymorphisms in miRNA-machinery genes may alter the biogenesis of miRNAs affecting disease susceptibility. In this case–control study, we aimed to evaluate the impact of three single-nucleotide polymorphisms (DICER rs1057035, DROSHA rs10719, and XPO5 rs11077) and their combined effect in a genetic risk score model on congenital heart disease (CHD) risk. A total of 639 participants was recruited, including 125 patients with CHD (65 males; age 9.2 ± 10 years) and 514 healthy controls (289 males; age 15.8 ± 18 years). Genotyping of polymorphisms in miRNA-machinery genes was performed using a TaqMan®SNP genotyping assay. A genetic risk score was calculated by summing the number of risk alleles of selected single-nucleotide polymorphisms. There was a significantly increased risk of CHD in patients with XPO5 rs11077 CC genotype as compared to AC heterozygote and AA homozygote patients (ORadjusted = 1.7; 95% CI: 1.1–2.8; p = 0.018). A clear tendency to significance was also found for DROSHA rs10719 AA genotype and CHD risk for both codominant and recessive models (ORadjusted = 1.8; 95% CI: 0.91–3.8; p = 0.09 and ORadjusted = 1.9; 95% CI: 0.92–4; p = 0.08, respectively). The resulting genetic risk score predicted a 1.73 risk for CHD per risk allele (95% CI: 1.2–2.5; p = 0.002). Subjects in the top tertile of genetic risk score were estimated to have more than three-fold increased risk of CHD compared with those in the bottom tertile (ORadjusted = 3.52; 95% CI: 1.4–9; p = 0.009). Our findings show that the genetic variants in miRNA-machinery genes might participate in the development of CHD.
Publishing patterns and citation performance of manuscripts relating to paediatric cardiology and congenital heart disease: comparison of paediatric and adult cardiology journals
- Rohit S. Loomba, Danielle Sheikholeslami, Aaron Dyson, Saul Flores, Enrique Villarreal, Juan Torres, Jeffrey P. Jacobs, Robert H. Anderson
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- Published online by Cambridge University Press:
- 24 February 2021, pp. 1608-1612
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Background:
Manuscripts pertaining to paediatric cardiology and CHD have been published in a variety of different journals. Some of these journals are journals dedicated to paediatric cardiology, while others are focused on adult cardiology. Historically, it has been considered that manuscripts published in journals devoted to adult cardiology have greater citation potential. Our objective was to compare citation performance between manuscripts related to paediatric cardiology and CHD published in paediatric as opposed to adult cardiology journals.
Methods:We identified manuscripts related to paediatric cardiology and CHD published in five journals of interest during 2014. Of these journals, two were primarily concerned with adult cardiology, while the other three focused on paediatric cardiology. The number of citations for these identified manuscripts was gathered from Google Scholar. We compared the number of citations (median, mean, and 25th, 75th, 90th, and 95th percentiles), the potential for citation, and the h-index for the identified manuscripts.
Results:We identified a total of 828 manuscripts related to paediatric cardiology and congenital heart as published in the 5 journals during 2014. Of these, 783 (95%) were published in journals focused on paediatric cardiology, and the remaining 45 (5%) were published in journals focused on adult cardiology. The median number of citations was 41 in the manuscripts published in the journals focused on adult cardiology, as opposed to 7 in journals focused on paediatric cardiology (p < 0.001). The h-index, however, was greater for the journals dedicated to paediatric cardiology (36 versus 27).
Conclusion:Approximately one-twentieth of the work relating to paediatric cardiology and CHD is published in journals that focus predominantly on adult cardiology. The median number of citations is greater when manuscripts concerning paediatric cardiology and CHD are published in these journals focused on adult cardiology. The h-index, however, is higher when the manuscripts are published in journals dedicated to paediatric cardiology. While such publications in journals that focus on adult cardiology tend to generate a greater number of citations than those achieved for works published in specialised paediatric cardiology journals, the potential for citation is no different between the journals. Due to the drastically lower number of manuscripts published in journals dedicated to adult cardiology, however, median performance is different.
Vasoactive-ventilation-renal score: a reliable prognostic index for perioperative outcomes following congenital heart surgery in adults
- Diana M. Torpoco Rivera, Richard U. Garcia, Sanjeev Aggarwal
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- Published online by Cambridge University Press:
- 17 December 2020, pp. 762-768
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Introduction:
The number of adults requiring surgeries for CHD is increasing. We sought to evaluate the utility of the vasoactive-ventilation-renal (VVR) score as a predictor of prolonged length of stay in adults following CHD surgery.
Methods:This is a retrospective review of 158 adult patients who underwent CHD surgery involving cardiopulmonary bypass. VVR score was calculated upon arrival to ICU and every 6 hours for the first 48 hours post-operatively. Our primary outcome was prolonged length of stay defined as hospital length of stay greater than 75th percentile for the cohort (≥8 days).
Results:The study cohort had a median age of 25.6 years (18–60 years), and 83 (52.5%) were male. The groups with and without prolonged length of stay were comparable in age, gender, race, and surgical severity score. VVR score was significantly higher at all time points in the group with prolonged length of stay. The first post-operative day peak VVR score ≥13 had a sensitivity of 81% and specificity of 75% for predicting prolonged length of stay (p = 0.0001). On regression analysis, peak VVR score during the first day was independently associated with prolonged length of stay.
Conclusions:Peak VVR score during the first post-operative day was a strong predictor of prolonged length of stay in adults following CHD surgery.