Original Articles
When the bi-directional Glenn is an unfavourable option: primary extracardiac inferior cavopulmonary connection as an alternative palliation
- Ali Dodge-Khatami, Avichal Aggarwal, Mary B. Taylor, Douglas Maposa, Jorge D. Salazar
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- Published online by Cambridge University Press:
- 28 April 2015, pp. 1247-1249
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The superior cavopulmonary anastomosis – bi-directional Glenn – is the standard palliation for single ventricle physiology. When upper body systemic venous anatomic concerns such as superior caval vein stenosis, hypoplasia, or inadequate collateral tributaries are present, a Glenn may be precluded or have a high risk of poor outcome. A primary inferior cavopulmonary connection with an extracardiac conduit is an alternative palliation that provides a generous pathway for pulmonary blood flow, with the additional benefit of including hepatic venous return. We report a case of primary extracardiac inferior cavopulmonary connection in a patient unsuitable for Glenn, with successful post-operative outcome and early follow-up.
Health-related quality of life of patients with pulmonary arterial hypertension associated with CHD: the multicentre cross-sectional ACHILLE study
- Pascal Amedro, Adeline Basquin, Virginie Gressin, Pierre Clerson, Xavier Jais, Jean-Benoit Thambo, Patrice Guerin, Sarah Cohen, Damien Bonnet
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- Published online by Cambridge University Press:
- 16 March 2016, pp. 1250-1259
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Background
The aim of this study was to assess health-related quality of life in patients with pulmonary arterial hypertension associated with CHD and correlations with clinical status.
MethodsThis prospective cross-sectional observational study included CHD patients with pulmonary arterial hypertension in 14 tertiary-care centres in France. We used two health-related quality of life questionnaires – SF-36 and Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) – and one anxiety/depression Hospital Anxiety and Depression Scale (HADS) questionnaire.
ResultsClinical data were collected for the 208 included patients (mean age: 42.6 years, range from 15.1 to 85.8 years, 69.7% female). Most patients were in NYHA functional class II (48.1%) and III (37.5%). Patients’ phenotype was classified as Eisenmenger syndrome (70.7%), pulmonary arterial hypertension associated with systemic-to-pulmonary shunts (12.0%), with small defects (3.4%), or after corrective cardiac surgery (13.9%). In total, 76.4% of the patients were receiving pulmonary arterial hypertension-specific treatments. SF-36 scores showed impairment compared with normalised data. Health-related quality of life scores were significantly lower in females than in males for most dimensions of both questionnaires and were independent of the patients’ phenotype, even after gender adjustment – except for CAMPHOR functioning – but significantly depended on NYHA functional class. The Hospital Anxiety and Depression Scale (HADS) scores suggested anxiety and depression associated with increasing NYHA functional class but independent of patients’ phenotype. NYHA functional class, 6-minute walk distance, HADS, gender, and recent stressful event significantly affected quality of life in the multivariate analysis.
ConclusionsThis study showed impairment of quality of life in a large cohort of patients with pulmonary arterial hypertension associated with CHD with both generic and specific questionnaires. NYHA functional class and HADS scores were predictive of most quality of life scores.
Transcatheter stenting of the right ventricular outflow tract augments pulmonary arterial growth in symptomatic infants with right ventricular outflow tract obstruction and hypercyanotic spells
- Eimear McGovern, Conall T. Morgan, Paul Oslizlok, Damien Kenny, Kevin P. Walsh, Colin J. McMahon
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- Published online by Cambridge University Press:
- 04 March 2016, pp. 1260-1265
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We retrospectively reviewed all the children with right ventricular outflow tract obstruction, hypoplastic pulmonary annulus, and pulmonary arteries who underwent stenting of the right ventricular outflow tract for hypercyanotic spells at our institution between January, 2008 and December, 2013; nine patients who underwent cardiac catheterisation at a median age of 39 days (range 12–60 days) and weight of 3.6 kg (range 2.6–4.3 kg) were identified. The median number of stents placed was one stent (range 1–4). The median oxygen saturation increased from 60% to 96%. The median right pulmonary artery size increased from 3.3 to 5.5 mm (−2.68 to −0.92 Z-score), and the median left pulmonary artery size increased from 3.4 to 5.5 mm (−1.93 to 0 Z-scores). Among all, one patient developed transient pulmonary haemorrhage, and one patient had pericardial tamponade requiring drainage. Complete repair of tetralogy of Fallot +/− atrioventricular septal defect or double-outlet right ventricle was achieved in all nine patients. Transcatheter stent alleviation of the right ventricular outflow tract obstruction resolves hypercyanotic spells and allows reasonable growth of the pulmonary arteries to facilitate successful surgical repair. This represents a viable alternative to placement of a systemic-to-pulmonary artery shunt, particularly in small neonates.
Echocardiographic parameters for assessing successful balloon mitral valvuloplasty in juvenile age groups
- Ajith Ananthakrishna Pillai, Geofi George, Harichandrakumar Kottyen Thazhath, Sreekanth Yerram
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- Published online by Cambridge University Press:
- 16 November 2015, pp. 1266-1273
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Background
Different echocardiographic parameters have been studied and validated for assessing the severity of mitral stenosis; however, scant data are available for these markers in the context of balloon mitral valvuloplasty in juvenile age groups (ages ⩽20 years).
ObjectivesThe aim of the present study was to find out the utility of echocardiographic parameters such as mitral valve separation index, left atrial volume, right ventricular systolic pressure, tricuspid annular plane systolic excursion, tricuspid annular systolic velocity, and right ventricular Tei index in predicting success of balloon mitral valvuloplasty and their relation to mitral valve area in juvenile mitral stenosis.
MethodsWe carried out a prospective single-centre study involving 52 juvenile mitral stenosis patients undergoing elective valvuloplasty. Success was defined as an increase in mitral valve area ⩾50% or ⩾1.5 cm2. Echocardiographic measurements were taken before and 24 hours after the procedure and statistical analyses were carried out.
ResultsThe mean age of the study population was 14.3 years (SD ±4.55), ranging from 7 to 20 years. Valvuloplasty was successful in 49 out of 52 patients. The mean valve area improved from 0.89 (SD ±0.16) to 1.73 (SD ±0.22) cm2/m2 (p<0.01), and the mean mitral valve gradient decreased from 19.87 (SD ±7.89) to 7.45 (SD ±2.07) (p=0.021). All the surrogate parameters improved favourably after valvuloplasty (p<0.01). The decrease in right ventricular systolic pressure was a better indicator of the success followed by the increase in valve separation index (area under the curve 0.81 and 0.76, respectively).
ConclusionsAll the surrogate markers studied showed favourable improvement, and right ventricular systolic pressure reduction and improved mitral valve separation index were better indicators of successful valvuloplasty.
Abnormal heart rate recovery and deficient chronotropic response after submaximal exercise in young Marfan syndrome patients
- Paulo Peres, Antônio C. Carvalho, Ana Beatriz A. Perez, Wladimir M. Medeiros
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- Published online by Cambridge University Press:
- 02 November 2015, pp. 1274-1281
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Background
Marfan syndrome patients present important cardiac structural changes, ventricular dysfunction, and electrocardiographic changes. An abnormal heart rate response during or after exercise is an independent predictor of mortality and autonomic dysfunction. The aim of the present study was to compare heart rate recovery and chronotropic response obtained by cardiac reserve in patients with Marfan syndrome subjected to submaximal exercise.
MethodsA total of 12 patients on β-blocker therapy and 13 off β-blocker therapy were compared with 12 healthy controls. They were subjected to submaximal exercise with lactate measurements. The heart rate recovery was obtained in the first minute of recovery and corrected for cardiac reserve and peak lactate concentration.
ResultsPeak heart rate (141±16 versus 155±17 versus 174±8 bpm; p=0.001), heart rate reserve (58.7±9.4 versus 67.6±14.3 versus 82.6±4.8 bpm; p=0.001), heart rate recovery (22±6 versus 22±8 versus 34±9 bpm; p=0.001), and heart rate recovery/lactate (3±1 versus 3±1 versus 5±1 bpm/mmol/L; p=0.003) were different between Marfan groups and controls, respectively. All the patients with Marfan syndrome had heart rate recovery values below the mean observed in the control group. The absolute values of heart rate recovery were strongly correlated with the heart rate reserve (r=0.76; p=0.001).
ConclusionMarfan syndrome patients have reduced heart rate recovery and chronotropic deficit after submaximal exercise, and the chronotropic deficit is a strong determinant of heart rate recovery. These changes are suggestive of autonomic dysfunction.
Glial fibrillary acidic protein as a biomarker for brain injury in neonatal CHD
- Stephanie L. McKenney, Fahad F. Mansouri, Allen D. Everett, Ernest M. Graham, Irina Burd, Priya Sekar
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- Published online by Cambridge University Press:
- 20 January 2016, pp. 1282-1289
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Neonates with critical CHD have evidence, by imaging, of preoperative brain injury, although the timing is unknown. We used circulating postnatal serum glial fibrillary acidic protein as a measure of acute perinatal brain injury in neonates with CHD. Glial fibrillary acidic protein was measured on admission and daily for the first 4 days of life in case and control groups; we included two control groups in this study – non-brain-injured newborns and brain-injured newborns. Comparisons were performed using the Kruskal–Wallis test with Dunn’s multiple comparisons, Student’s t-test, and χ2 test of independence where appropriate. In aggregate, there were no significant differences in overall glial fibrillary acidic protein levels between CHD patients (n=56) and negative controls (n=23) at any time point. By day 4 of life, 7/56 (12.5%) CHD versus 0/23 (0%) normal controls had detectable glial fibrillary acidic protein levels. Although not statistically significant, the 5/10 (50%) left heart obstruction group versus 1/17 (6%) conoventricular, 0/13 (0%) right heart, and 1/6 (17%) septal defect patients trended towards elevated levels of glial fibrillary acidic protein at day 4 of life. Overall, glial fibrillary acidic protein reflected no evidence for significant peripartum brain injury in neonates with CHD, but there was a trend for elevation by postnatal day 4 in neonates with left heart obstruction. This pilot study suggests that methods such as monitoring glial fibrillary acidic protein levels may provide new tools to optimise preoperative care and neuroprotection in high-risk neonates with specific types of CHD.
Association of IL-4 (intron 3) and IL-10 (-1082) gene polymorphisms with risk of mitral valve disease in children with rheumatic heart disease
- Sherif M. Yousry, Yasser Sedky, Alaa Sobieh
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- Published online by Cambridge University Press:
- 28 October 2015, pp. 1290-1296
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Aim
Rheumatic heart disease is an inflammatory disease of cardiac tissue. The underlying pathogenic mechanisms highlight a complex interplay of immunological, genetic, and environmental factors. The aim of the present study was to investigate whether IL-4 (intron 3) and IL-10 (-1082) gene polymorphisms could be associated with susceptibility and/or severity of rheumatic heart disease among patients from the Egyptian population.
Materials and methodsA cohort of 140 Egyptian children with rheumatic heart disease and 100 healthy controls were enrolled in this case–control study. Genotyping for IL-4 (intron 3) and IL-10 (-1082) gene polymorphisms was carried out for all patients using a polymerase chain reaction-based analysis.
ResultsNo significant difference in the distribution of genotypes and allelic frequencies between rheumatic heart disease cases and controls for IL-4 (intron 3) (p=0.17; OR 1.07, 95% CI 0.82–3.74) and IL-10 (-1082) (p=0.49; OR 1.03, 95% CI 0.65–2.71) gene polymorphisms was observed. Further categorisation of patients into mitral valve disease and combined valve disease subgroups showed that cases with mitral valve disease have significantly higher frequency of the RP2 allele of IL-4 (intron 3) (p=0.03; OR 2.98, 95% CI 1.93–6.15) and the G allele of IL-10 (-1082) (p=0.04; OR 2.14, 95% CI 1.62–4.95) when compared with controls.
DiscussionOur study shows that IL-4 (intron 3) and IL-10 (-1082) gene polymorphisms are not significantly associated with susceptibility to rheumatic heart disease, but they might play a role in the pathogenesis of patients with mitral valve disease.
Use of three-dimensional mapping in young patients decreases radiation exposure even without a goal of zero fluoroscopy
- Cheyenne Beach, Lee Beerman, Sharon Mazzocco, Maria M. Brooks, Gaurav Arora
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- Published online by Cambridge University Press:
- 28 October 2015, pp. 1297-1302
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At present, three-dimensional mapping is often used during cardiac ablations with an explicit goal of decreasing radiation exposure; three-dimensional mapping was introduced in our institution in 2007, but not specifically to decrease fluoroscopy time. We document fluoroscopy use and catheterisation times in this setting. Data were obtained retrospectively from patients who underwent ablation for atrioventricular nodal re-entrant tachycardia from January, 2004 to December, 2011. A total of 93 patients were included in the study. Among them, 18 patients who underwent radiofrequency ablation without three-dimensional mapping were included in Group 1, 13 patients who underwent cryoablation without three-dimensional mapping were included in Group 2, and 62 patients who underwent cryoablation with three-dimensional mapping were included in Group 3. Mean fluoroscopy times differed significantly (34.3, 23.4, and 20.3 minutes, p<0.001) when all the groups were compared. Group 3 had a shorter average fluoroscopy time that did not reach significance when compared directly with Group 2 (p=0.29). An unadjusted linear regression model showed a progressive decrease in fluoroscopy time (p=0.002). Mean total catheterisation times differed significantly (180, 211, and 210 minutes, p=0.02) and were related to increased ablation times inherent to cryoablation techniques. Acute success was achieved in 89, 100, and 97% of patients (p=0.25), and chronic success was achieved in 80, 92, and 93% of patients (p=0.38). Complication rates were similar (17, 23, and 7%, p=0.14). In conclusion, three-dimensional mapping systems decrease fluoroscopy times even without an explicit goal of zero fluoroscopy. Efficacy and safety of the procedure have not changed.
Impact of postoperative complications on hospital costs following the Norwood operation
- Kimberly E. McHugh, Sara K. Pasquali, Matthew A. Hall, Mark A. Scheurer
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- Published online by Cambridge University Press:
- 30 December 2015, pp. 1303-1309
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Introduction
Patients undergoing the Norwood operation consume considerable healthcare resources; however, detailed information regarding factors impacting hospitalisation costs is lacking. We evaluated the association of postoperative complications with hospital costs.
MethodsIn the present study, we utilised a unique data set consisting of prospectively collected clinical data from the Pediatric Heart Network Single Ventricle Reconstruction trial linked at the patient level with cost data for 10 hospitals participating in the Children’s Hospital Association Case Mix database during the trial period. The relationship between complications and cost was modelled using linear regression, accounting for the skewed distribution of cost, adjusting for within-centre clustering and baseline patient characteristics.
ResultsA total of 334 eligible Norwood records (97.5%) were matched between data sets. Overall, 82% suffered from at least one complication (median 2; with a range from 0 to 33). Those with complications had longer postoperative length of stay (25 versus 12 days, p<0.001), more total ventilator days (7 versus 5 days, p<0.001), and higher in-hospital mortality (17.6 versus 3.4%, p<0.006). Mean adjusted hospital cost in those with a complication was $190,689 (95% CI $111,344–$326,577) versus $120,584 (95% CI $69,246–$209,983) in those without complications (p=0.002). Costs increased with the number of complications (1–2 complications=$132,800 versus 3–4 complications=$182,353 versus ⩾5 complications=$309,372 [p<0.001]).
ConclusionsThis merged data set of clinical trial and cost data demonstrated that postoperative complications are common following the Norwood operation and are associated with worse clinical outcomes and higher costs. Efforts to reduce complications in this population may lead to improved outcomes and cost savings.
Usefulness of maximal oxygen pulse in timing of pulmonary valve replacement in patients with isolated pulmonary regurgitation
- Antoine Legendre, Ruddy Richard, Florence Pontnau, Jean-Philippe Jais, Marc Dufour, Olivier Grenier, Elie Mousseaux, Magalie Ladouceur, Laurence Iserin, Damien Bonnet
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- Published online by Cambridge University Press:
- 22 December 2015, pp. 1310-1318
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Patients with pulmonary regurgitation after tetralogy of Fallot repair have impaired aerobic capacity; one of the reasons is the decreasing global ventricular performance at exercise, reflected by decreasing peak oxygen pulse. The aims of our study were to evaluate the impact of pulmonary valve replacement on peak oxygen pulse in a population with pure pulmonary regurgitation and with different degrees of right ventricular dilatation and to determine the predictors of peak oxygen pulse after pulmonary valve replacement.
The mean and median age at pulmonary valve replacement was 27 years. Mean pre-procedural right ventricular end-diastolic volume was 182 ml/m2. Out of 24 patients, 15 had abnormal peak oxygen pulse before pulmonary valve replacement. We did not observe a significant increase in peak oxygen pulse after pulmonary valve replacement (p=0.76). Among cardiopulmonary test/MRI/historical pre-procedural parameters, peak oxygen pulse appeared to be the best predictor of peak oxygen pulse after pulmonary valve replacement (positive and negative predictive values, respectively, 0.94 and 1). After pulmonary valve replacement, peak oxygen pulse was well correlated with left ventricular stroke and end-diastolic volumes (r=0.67 and 0.68, respectively).
Our study confirms the absence of an effect of pulmonary valve replacement on peak oxygen pulse whatever the initial right ventricular volume, reflecting possible irreversible right and/or left ventricle lesions. Pre-procedural peak oxygen pulse seemed to well predict post-procedural peak oxygen pulse. These results encourage discussions on pulmonary valve replacement in patients showing any decrease in peak oxygen pulse during their follow-up.
Systemic rapamycin to prevent in-stent stenosis in peripheral pulmonary arterial disease: early clinical experience
- Anna Hallbergson, Jesse J. Esch, Trang X. Tran, James E. Lock, Audrey C. Marshall
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- Published online by Cambridge University Press:
- 28 December 2015, pp. 1319-1326
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Objectives
We have taken a novel approach using oral rapamycin – sirolimus – as a medical adjunct to percutaneous therapy in patients with in-stent stenosis and high risk of right ventricular failure.
BackgroundPeripheral pulmonary artery stenosis can result in right ventricular hypertension, dysfunction, and death. Percutaneous pulmonary artery angioplasty and stent placement acutely relieve obstructions, but patients frequently require re-interventions due to re-stenosis. In patients with tetralogy of Fallot or arteriopathy, the problem of in-stent stenosis contributes to the rapidly recurrent disease.
MethodsRapamycin was administered to 10 patients (1.5–18 years) with peripheral pulmonary stenosis and in-stent stenosis and either right ventricular hypertension, pulmonary blood flow maldistribution, or segmental pulmonary hypertension. Treatment was initiated around the time of catheterisation and continued for 1–3 months. Potential side-effects were monitored by clinical review and blood tests.
ResultsTarget serum rapamycin level (6–10 ng/ml) was accomplished in all patients; eight of the nine patients who returned for clinically indicated catheterisations demonstrated reduction in in-stent stenosis, and eight of the 10 patients experienced no significant side-effects. Among all, one patient developed diarrhoea requiring drug discontinuation, and one patient experienced gastrointestinal bleeding while on therapy that was likely due to an indwelling feeding tube and this patient tolerated rapamycin well following tube removal.
ConclusionsOur initial clinical experience supports that patients with peripheral pulmonary artery stenosis can be safely treated with rapamycin. Systemic rapamycin may provide a novel medical approach to reduce in-stent stenosis.
Does superior caval vein pressure impact head growth in Fontan circulation?
- Tina Trachsel, Christian Balmer, Håkan Wåhlander, Roland Weber, Hitendu Dave, Andrea Poretti, Oliver Kretschmar, Anna Cavigelli-Brunner
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- Published online by Cambridge University Press:
- 15 January 2016, pp. 1327-1332
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Background
Patients with bidirectional cavopulmonary anastomosis have unphysiologically high superior caval vein pressure as it equals pulmonary artery pressure. Elevated superior caval vein pressure may cause communicating hydrocephalus and macrocephaly. This study analysed whether there exists an association between head circumference and superior caval vein pressure in patients with single ventricle physiology.
MethodsWe carried out a retrospective analysis of infants undergoing Fontan completion at our institution from 2007 to 2013. Superior caval vein pressures were measured during routine catheterisation before bidirectional cavopulmonary anastomosis and Fontan completion as well as head circumference, adjusted to longitudinal age-dependent percentiles.
ResultsWe included 74 infants in our study. Median ages at bidirectional cavopulmonary anastomosis and Fontan were 4.8 (1.6–12) and 27.9 (7–40.6) months, respectively. Head circumference showed significant growth from bidirectional cavopulmonary anastomosis until Fontan completion (7th (0–100th) versus 20th (0–100th) percentile). There was no correlation between superior caval vein pressure and head circumference before Fontan (R2=0.001). Children with lower differences in superior caval vein pressures between pre-bidirectional cavopulmonary anastomosis and pre-Fontan catheterisations showed increased growth of head circumference (R2=0.19).
ConclusionsPatients with moderately elevated superior caval vein pressure associated with single ventricle physiology did not have a tendency to develop macrocephaly. There is no correlation between superior caval vein pressure before Fontan and head circumference, but between bidirectional cavopulmonary anastomosis and Fontan head circumference increases significantly. This may be explained by catch-up growth of head circumference in patients with more favourable haemodynamics and concomitant venous pressures in the lower range. Further studies with focus on high superior caval vein pressures are needed to exclude or prove a correlation.
Assessment of the carotid artery intima-media complex through ultrasonography and the relationship with Pathobiological Determinants of Atherosclerosis in Youth
- Thacira D. A. Ramos, Tatianne M. E. Dantas, Mônica O. S. Simões, Danielle F. Carvalho, Carla C. M. Medeiros
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- Published online by Cambridge University Press:
- 11 November 2015, pp. 1333-1342
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Objective
To evaluate the presence of carotid thickening and its relationship with the Pathobiological Determinants of Atherosclerosis in Youth score.
MethodsWe carried out a cross-sectional study involving 512 brazilian adolescents. Variables such as sex, body mass index, concentrations of non-high-density lipoprotein and high-density lipoprotein cholesterol, blood pressure, blood glucose and glycated haemoglobin A1c levels that make up the score, and carotid thickening through the intima-media complex measured by ultrasound were evaluated. We adopted two cut-off points to evaluate carotid thickening, being considered altered for those higher or equal to the z-score 2+ and ⩾75th percentile. The association was assessed using the χ2 test and univariate and multivariate logistic regression analyses.
ResultsHigh cardiovascular risk was present in 10.2% of the adolescents; carotid thickness was present in 4.3% determined by the z-score 2+ and in 25.0% determined by the 75th percentile. When measured by the z-score, carotid thickening was associated with high systolic blood pressure (p=0.024), high-non-high density lipoprotein cholesterol (p=0.039), and high cardiovascular risk assessed by the score and by the 75th percentile, with body mass index >30 (p=0.005). In the multivariate analysis, high cardiovascular risk was found to be independently associated with the presence of carotid thickness evaluated by the z-score, with risk four times greater (p=0.010) of presenting with this condition compared with individuals with low risk, and this fact was not observed when factors were analysed alone.
ConclusionThe presence of high cardiovascular risk in adolescents assessed by the Pathobiological Determinants of Atherosclerosis in Youth score was associated with marked thickening of the carotid artery in healthy adolescents.
Screening for language delay after life-saving therapies in term-born infants
- Brenda G. Clark, Bryan V. Acton, Gwen Y. Alton, Ari R. Joffe, Irina A. Dinu, Charlene M. T. Robertson
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- Published online by Cambridge University Press:
- 28 December 2015, pp. 1343-1351
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Background
Strong recommendations have been made for the periodic developmental surveillance, screening, and evaluation of children with CHD. This supports similar calls for all at-risk children in order to provide timely, structured early developmental intervention that may improve outcomes. The aim of this study was to determine the accuracy of screening for language delay after life-saving therapies using the parent-completed vocabulary screen of the language Development Survey, by comparing screening with the individually administered language scores of the Bayley Scales of Infant and Toddler Development, Third edition.
MethodIn total, 310 (92.5%) of 335 eligible term-born children, born between 2004 and 2011, receiving complex cardiac surgery, heart or liver transplantation, or extracorporeal membrane oxygenation in infancy, were assessed at 21.5 (2.8) months of age (lost, 25 (7.5%)), through developmental/rehabilitation centres at six sites as part of the Western Canadian Complex Pediatric Therapies Follow-up Group.
ResultsVocabulary screening delay was defined as scores ⩽15th percentile. Language delay defined as scores >1 SD below the mean was calculated for language composite score, receptive and expressive communication scores of the Bayley-III. Delayed scores for the 310 children were as follows: vocabulary, 144 (46.5%); language composite, 125 (40.3%); receptive communication, 98 (31.6%); and expressive communication, 124 (40%). Sensitivity, specificity, positive predictive values, and negative predictive values of screened vocabulary delay for tested language composite delay were 79.2, 75.7, 68.8, and 84.3%, respectively.
ConclusionHigh rates of language delay after life-saving therapies are concerning. Although the screening test appears to over-identify language delay relative to the tested Bayley-III, it may be a useful screening tool for early language development leading to earlier referral for intervention.
A novel approach to ductal spasm during percutaneous device occlusion of patent ductus arteriosus
- Rik De Decker, George Comitis, Jenny Thomas, Elmarie van der Merwe, John Lawrenson
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- Published online by Cambridge University Press:
- 22 December 2015, pp. 1352-1358
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Ductal spasm is a rare yet important complication of device occlusions of patent ductus arteriosus. Spasm may result in failure of the procedure, under-sizing of the device, or embolisation of the implanted device as the spasm resolves after the procedure. We describe a novel protocol that rapidly and completely reversed the spasm in eight prematurely born infants who experienced ductal spasm during cardiac catheterisations for patent ductus arteriosus occlusion.
In total, eight infants born between 25 and 34 weeks of gestation presented for transcatheter patent ductus arteriosus occlusion between 13 and 87 months of age. All eight patients experienced ductal spasm either immediately before, during, or soon after induction of anaesthesia or only after entering the ductus arteriosus with a catheter. After detection of the spasm, the anaesthetist, in each case, changed the mode of anaesthesia from inhaled sevoflurane to total intravenous anaesthesia with propofol, reduced the inhaled oxygen fraction to 21%, and initiated a continuous intravenous infusion of prostaglandin E1.
The first two steps (total intravenous anaesthesia and FiO2 0.21) resulted in only partial relaxation of the spasm. Complete relaxation was attained after intravenous prostaglandin E1 infusions of only 10–15 minutes’ duration. While maintaining this protocol, six ducti were successfully occluded and two were considered to be unsuitable for device occlusion and were referred for surgery.
Ductal spasm during transcatheter occlusion may be reliably resolved and the procedure safely completed by a simple anaesthetic protocol, including the continuous infusion of intravenous prostaglandin E1.
Computer-aided auscultation of murmurs in children: evaluation of commercially available software
- Cecilia Lee, Kathryn N. Rankin, Kevin J. Zuo, Andrew S. Mackie
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- Published online by Cambridge University Press:
- 20 January 2016, pp. 1359-1364
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Background
Heart murmurs are common in children and may represent congenital or acquired cardiac pathology. Auscultation is challenging and many primary-care physicians lack the skill to differentiate innocent from pathologic murmurs. We sought to determine whether computer-aided auscultation (CardioscanTM) identifies which children require referral to a cardiologist.
MethodsWe consecutively enrolled children aged between 0 and 17 years with a murmur, innocent or pathologic, being evaluated in a tertiary-care cardiology clinic. Children being evaluated for the first time and patients with known cardiac pathology were eligible. We excluded children who had undergone cardiac surgery previously or were unable to sit still for auscultation. CardioscanTM auscultation was performed in a quiet room with the subject in the supine position. The sensitivity and specificity of a potentially pathologic murmur designation by CardioscanTM – that is, requiring referral – was determined using echocardiography as the reference standard.
ResultsWe enrolled 126 subjects (44% female) with a median age of 1.7 years, with 93 (74%) having cardiac pathology. The sensitivity and specificity of a potentially pathologic murmur determination by CardioscanTM for identification of cardiac pathology were 83.9 and 30.3%, respectively, versus 75.0 and 71.4%, respectively, when limited to subjects with a heart rate of 50–120 beats per minute. The combination of a CardioscanTM potentially pathologic murmur designation or an abnormal electrocardiogram improved sensitivity to 93.5%, with no haemodynamically significant lesions missed.
ConclusionsSensitivity of CardioscanTM when interpreted in conjunction with an abnormal electrocardiogram was high, although specificity was poor. Re-evaluation of computer-aided auscultation will remain necessary as advances in this technology become available.
Clinical implications of mitral valve geometric alterations in children with dilated cardiomyopathy
- Taiyu Hayashi, Ryo Inuzuka, Takahiro Shindo, Hiroshi Ono, Yukihiro Kaneko, Hitoshi Kato
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- Published online by Cambridge University Press:
- 28 December 2015, pp. 1365-1372
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We aimed to elucidate the relationship between severity of secondary mitral regurgitation and mitral valve geometry in children with dilated cardiomyopathy. The medical records of 16 children with dilated cardiomyopathy (median age, 1.2 years; range, 0.4–12.3 years) were reviewed. Mitral valve geometry was evaluated by measuring coaptation depth using echocardiographic apical four-chamber views at the initial presentation. Patients were dichotomised according to the mitral regurgitation severity: patients with moderate or severe secondary mitral regurgitation (n=6) and those with mild secondary mitral regurgitation (n=10). A total of 58 healthy children were considered as normal controls, and a regression equation to predict coaptation depth by body surface area was derived: coaptation depth [mm]=4.37+1.34×ln (body surface area [m2]) (residual standard error, 0.49; adjusted R2, 0.68; p<0.0001). Compared with patients with mild secondary mitral regurgitation, those with moderate or severe secondary mitral regurgitation had significantly larger coaptation depth z-scores (6.4±2.3 versus 1.9±1.4, p<0.005), larger mitral annulus diameter z-scores (3.6±2.6 versus 0.9±1.8, p<0.05), higher left ventricular sphericity index (0.89±0.07 versus 0.79±0.06, p<0.005), and greater left ventricular fraction shortening (0.15±0.05 versus 0.09±0.05, p<0.05). In conclusion, geometric alteration in the mitral valve and the left ventricle is associated with the severity of secondary mitral regurgitation in paediatric dilated cardiomyopathy, which would provide a theoretical background to surgical intervention for secondary mitral regurgitation in paediatric populations.
The comparative role of echocardiography and MRI for identifying critical lesions in patients with single-ventricle physiology, before bidirectional cavopulmonary connection
- Sylvia Krupickova, Michael A. Quail, Robert Yates, Roman Gebauer, Marina Hughes, Jan Marek
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- Published online by Cambridge University Press:
- 04 February 2016, pp. 1373-1382
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Background
In the era of multi-modality imaging, this study compared contemporary, pre-operative echocardiography and cardiac MRI in predicting the need for intervention on additional lesions before surgical bidirectional cavopulmonary connection.
MethodsA total of 72 patients undergoing bidirectional cavopulmonary connection for single-ventricle palliation between 2007 and 2012, who underwent pre-operative assessment using both echocardiography and MRI, were included. The pre-determined outcome measure was any additional surgical or catheter-based intervention within 6 months of bidirectional cavopulmonary connection. Indices assessed were as follows: indexed dimensions of right and left pulmonary arteries, coarctation of the aorta, adequacy of interatrial communication, and degree of atrioventricular valve regurgitation.
ResultsMedian age at bidirectional cavopulmonary connection was 160 days (interquartile range 121–284). The following MRI parameters predicted intervention: Z score for right pulmonary artery (odds ratio 1.77 (95% confidence interval 1.12–2.79, p=0.014)) and left pulmonary artery dimensions (odds ratio 1.45 (1.04–2.00, p=0.027)) and left pulmonary artery report conclusion (odds ratio 1.57 (1.06–2.33)). The magnetic resonance report predicted aortic arch intervention (odds ratio 11.5 (3.5–37.7, p=0.00006)). The need for atrioventricular valve repair was associated only with magnetic resonance regurgitation fraction score (odds ratio 22.4 (1.7–295.1, p=0.018)). Echocardiography assessment was superior to MRI for predicting intervention on interatrial septum (odds ratio 27.7 (6.3–121.6, p=0.00001)).
ConclusionFor branch pulmonary arteries, aortic arch, and atrioventricular valve regurgitation, MRI parameters more reliably predict the need for intervention; however, echocardiography more accurately identified the adequacy of interatrial communication. Approaching bidirectional cavopulmonary connection, the diagnostic strengths of MRI and echocardiography should be acknowledged when considering intervention.
Dynamics of heart rate variability analysed through nonlinear and linear dynamics is already impaired in young type 1 diabetic subjects
- Naiara M. Souza, Thais R. Giacon, Francis L. Pacagnelli, Marianne P. C. R. Barbosa, Vitor E. Valenti, Luiz C. M. Vanderlei
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- Published online by Cambridge University Press:
- 03 February 2016, pp. 1383-1390
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Background
Autonomic diabetic neuropathy is one of the most common complications of type 1 diabetes mellitus, and studies using heart rate variability to investigate these individuals have shown inconclusive results regarding autonomic nervous system activation.
AimsTo investigate the dynamics of heart rate in young subjects with type 1 diabetes mellitus through nonlinear and linear methods of heart rate variability.
MethodsWe evaluated 20 subjects with type 1 diabetes mellitus and 23 healthy control subjects. We obtained the following nonlinear indices from the recurrence plot: recurrence rate (REC), determinism (DET), and Shanon entropy (ES), and we analysed indices in the frequency (LF and HF in ms2 and normalised units – nu – and LF/HF ratio) and time domains (SDNN and RMSSD), through analysis of 1000 R–R intervals, captured by a heart rate monitor.
ResultsThere were reduced values (p<0.05) for individuals with type 1 diabetes mellitus compared with healthy subjects in the following indices: DET, REC, ES, RMSSD, SDNN, LF (ms2), and HF (ms2). In relation to the recurrence plot, subjects with type 1 diabetes mellitus demonstrated lower recurrence and greater variation in their plot, inter-group and intra-group, respectively.
ConclusionYoung subjects with type 1 diabetes mellitus have autonomic nervous system behaviour that tends to randomness compared with healthy young subjects. Moreover, this behaviour is related to reduced sympathetic and parasympathetic activity of the autonomic nervous system.
Effectiveness of cardiac surgery in patients with trisomy 18: a single-institutional experience
- Yosuke Nakai, Miki Asano, Norikazu Nomura, Hidekazu Matsumae, Akira Mishima
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- Published online by Cambridge University Press:
- 28 January 2016, pp. 1391-1396
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Background
Surgical repair for cardiac lesions has rarely been offered to patients with trisomy 18 because of their very short lifespans. We investigated the effectiveness of cardiac surgery in patients with trisomy 18.
Patients and methodsWe performed a retrospective analysis of 20 consecutive patients with trisomy 18 and congenital cardiac anomalies who were evaluated between August, 2003 and July, 2013. All patients developed respiratory or cardiac failure due to excessive pulmonary blood flow. Patients were divided into two subgroups: one treated surgically (surgical group, n=10) and one treated without surgery (conservative group, n=10), primarily to compare the duration of survival between the groups.
ResultsAll the patients in the surgical group underwent cardiac surgery with pulmonary artery banding, including patent ductus arteriosus ligation in nine patients and coarctation repair in one. The duration of survival was significantly longer in the surgical group than in the conservative group (495.4±512.6 versus 93.1±76.2 days, respectively; p=0.03). A Cox proportional hazard model found cardiac surgery to be a significant predictor of survival time (risk ratio of 0.12, 95% confidence interval 0.016–0.63; p=0.01).
ConclusionsCardiac surgery was effective in prolonging survival by managing high pulmonary blood flow; however, the indication for surgery should be carefully considered on a case-by-case basis, because the risk of sudden death remains even after surgery. Patients’ families should be provided with sufficient information to make decisions that will optimise the quality of life for both patients and their families.