Review Articles
Electrocardiac effects associated with lithium toxicity in children: an illustrative case and review of the pathophysiology
- Dhiraj Singh, Akinbolaji Akingbola, Nancy Ross-Ascuitto, Robert Ascuitto
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- Published online by Cambridge University Press:
- 14 September 2015, pp. 221-229
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Lithium is a potent psychotherapeutic agent that has gained wide acceptance in paediatrics, especially as adjunct treatment for severe behavioural, anxiety, and attention-deficit hyperactivity disorders, along with bipolar conditions. Its cardiac toxicity has been well-documented in adults; however, information is limited regarding lithium’s effects on the heart in children. Therefore, paediatric cardiologists following-up children on lithium therapy should be cognizant of the cardiac side-effects and pathophysiology associated with this drug. In this manuscript, we used an illustrative case of a child who presented with lithium poisoning, in order to highlight adverse clinical manifestations that can arise from this medication. The cardiac cell membrane is thought to be the primary site of lithium’s action. Thus, we reviewed lithium’s effects on membrane electrogenic pumps and channels involved in the distribution and passage of sodium, potassium, and calcium across the sarcolemma, as these ions, and their associated currents, are the primary determinates of the action potentials underlying auto-rhythmicity and contractile activity of the heart.
A 16-year-old with ST elevation myocardial infarction: case report and review of the literature
- Dustin Hill, Adam Waldman, Deepak Vivek
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- Published online by Cambridge University Press:
- 02 October 2015, pp. 230-236
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Chest pain in young adults presents a unique diagnostic challenge, placing young patients at an increased risk to be misdiagnosed, as this patient population typically does not demonstrate the traditional risk factors associated with cardiovascular disease. This study details the case of a 16-year-old male who presented with new-onset chest pain and ST elevation on electrocardiogram. His history was unremarkable for known cardiac risk factors, but laboratory evaluation demonstrated markedly elevated troponins and electrocardiographic findings confirmed ST-segment elevation myocardial infarction. Coronary angiography demonstrated 100% occlusion of the left anterior descending artery, which was managed with percutaneous transluminal coronary angioplasty, thrombectomy, and bare-metal stenting. The patient had an uneventful recovery. This study examines the major causes of ST elevation myocardial infarction in young adults and reviews the major differences between younger and older myocardial infarction populations with emphasis on risk factor profile, pathophysiological mechanisms, clinical presentation, angiographic findings, and prognosis. This review highlights the need for consideration of a wide differential in younger subsets of the population presenting with chest pain and ST elevation. The implementation of current adult management protocols and guidelines for ST elevation myocardial infarction should not be overlooked due to age. Given the potential for premature death and long-term disability with resulting individual and societal consequences, it is crucial to understand the importance of correct diagnostic evaluation in this clinical scenario.
Original Articles
Association between polymorphisms in IL27 and risk for CHD in a Chinese population
- Danyan Zhang, Mingfu Ma, Yuyou Yang, Ling Wan, Zhixi Yang, Jing Lv, Xinsheng Li, Hao Yang, Pei Huang, Lianbing Li
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- Published online by Cambridge University Press:
- 09 February 2015, pp. 237-243
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Background
IL-27, a member of the IL-12 family, has been involved in maternal tolerance to the foetus and successful pregnancy. Growing evidences indicate that IL-27 plays a crucial role in pregnancy.
AimWe carried out the present study in order to investigate whether polymorphisms in the IL27 are associated with the risk for CHDs, including atrial septal defect and ventricular septal defect.
Patients and methodsWe conducted this case–control study among 247 atrial septal defect patients, 150 ventricular septal defect patients, and 368 healthy controls in a Chinese population using polymerase chain reaction-restriction fragment length polymorphism assay.
ResultsSignificantly increased risk for atrial septal defect (p=0.001, OR=1.490, 95% CI=1.178–1.887) and ventricular septal defect (p=0.004, OR=1.502, 95% CI=1.139–1.976) was observed to be associated with the allele G of rs153109. In a dominant model, we have also observed that increased susceptibilities for atrial septal defect (p<0.01, OR=1.89, 95% CI=1.35–2.63) and ventricular septal defect (p<0.01, OR=2.50, 95% CI=1.67–3.85) were statistically associated with rs153109; however, no association was found between CHD risk and rs17855750 in the IL27 gene.
ConclusionThe 153109 of the IL27 gene may be associated with the susceptibility to CHD, including atrial septal defect and ventricular septal defect.
Breakfast frequency, adiposity, and cardiovascular risk factors as markers in adolescents
- Suziane U. Cayres, Ismael F. F. Júnior, Maurício F. Barbosa, Diego G. D. Christofaro, Rômulo A. Fernandes
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- Published online by Cambridge University Press:
- 10 February 2015, pp. 244-249
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Objective
To analyse the relationship between skipping breakfast and haemodynamic, metabolic, inflammatory, and cardiovascular risk factors in adolescents.
MethodsA cross-sectional study was carried out with information from an ongoing cohort study in Presidente Prudente, São Paulo, Brazil. The sample comprised of 120 adolescents (11.7±0.8 years old) who met the following inclusion criteria: age between 11 and 14 years; enrolled in the school unit of elementary education; absence of any known disease; and no drug consumption. The parents or legal guardians of the patients signed a formal informed consent. Skipping breakfast was self-reported through face-to-face interviews. Blood pressure, intima-media thickness, trunk fatness, total and fractional cholesterol levels – high-density lipoprotein cholesterol and low-density lipoprotein cholesterol – triacylglycerol levels, and high-sensitivity C-reactive protein levels were measured.
ResultsIn this study, 47.5% (95% CI: 38.5–56.4%) of the adolescents reported skipping breakfast at least 1 day/week. Adolescents who skipped breakfast had higher values of trunk fatness and systolic blood pressure. Breakfast frequency was negatively related to systolic blood pressure (β −1.99 [−3.67; −0.31]) and z score dyslipidaemia (β −0.46 [−0.90; −0.01]), but this relationship was mediated by trunk fatness.
ConclusionSkipping breakfast is related to cardiovascular risk factors in adolescents, and this relationship was mainly mediated by trunk fatness.
Influence of CHDs on psycho-social and neurodevelopmental outcomes in children with Down syndrome
- Jeannie Visootsak, Lillie Huddleston, Allison Buterbaugh, Adrienne Perkins, Stephanie Sherman, Jessica Hunter
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- Published online by Cambridge University Press:
- 16 February 2015, pp. 250-256
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Objective
To evaluate the family psycho-social outcomes of children with Down syndrome and atrioventricular septal defect, and examine the impact of these variables on the child’s neurodevelopmental outcome.
MethodsThis was a cross-sectional study that consisted of 57 children with Down syndrome – 20 cases and 37 controls – of ~12–14 months of age. In both groups, we assessed the development of the child, the quality of the child’s home environment, and parenting stress.
ResultsCompared with the Down syndrome without CHD group, the atrioventricular septal defect group revealed lower scores in all developmental domains, less optimal home environments, and higher parental stress. Significant differences in development were seen in the areas of cognition (p=0.04), expressive language (p=0.05), and gross motor (p<0.01). The Home Observation for Measurement of the Environment revealed significant differences in emotional and verbal responsiveness of the mother between the two groups. The Parenting Stress Index revealed that the Down syndrome with atrioventricular septal defect group had a significantly higher child demandingness subdomain scores compared with the Down syndrome without CHD group.
ConclusionsThe diagnosis of a CHD in addition to the diagnosis of Down syndrome may provide additional stress to the child and parents, elevating parental concern and disrupting family dynamics, resulting in further neurodevelopmental deficits. Finding that parental stress and home environment may play a role in the neurodevelopmental outcomes may prompt new family-directed interventions and anticipatory guidance for the families of children with Down syndrome who have a CHD.
Academic achievement and satisfaction in adolescents with CHD
- Christina J. Schaefer, Ricarda Hoop, Stefanie Schürch-Reith, Dominik Stambach, Oliver Kretschmar, Urs Bauersfeld, Beatrice Latal, Markus A. Landolt
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- Published online by Cambridge University Press:
- 24 February 2015, pp. 257-262
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Objectives
To evaluate academic achievement and satisfaction in adolescents with CHD.
Study designQuestionnaires were sent to all adolescents, aged between 17 and 20 years with CHD, currently treated at our hospital (n=326) in order to assess the patients’ education and satisfaction with their academic career. Results were compared with the official community statistics.
ResultsA total of 207 patients completed the questionnaires (participation rate 63.5%), 113 boys and 94 girls; 50% had completed mandatory school at the highest, 37.3% at the middle, and 12.7% at the lowest educational level. The distribution in the general population was comparable: 57.6, 32.5, and 9.9%, respectively (p=0.8). Adolescents with severe CHD were less likely to attain a higher educational level than those with moderate or mild CHD (p=0.03 for school grades 7–9). None of the other examined medical or socio-demographic factors, such as socio-economic status, foreign language, severity of CHD, cyanosis, and open heart surgery, were found to be associated with lower educational attainment. After the mandatory 9 years of schooling, 21.4% (n=44) of the patients with CHD compared with 16.7% in the general population attended higher school levels heading towards university education (p=0.7). From the 165 patients who provided information on career satisfaction, 79% regarded their job or school situation as being their desired one without a difference for those with severe CHD.
ConclusionSchool education in Swiss adolescents with CHD is very similar to the normal population. In addition, the majority of adolescents are satisfied with their educational career. This fact may be due to the good educational support provided during schooling.
Routine intra-operative trans-oesophageal echocardiography yields better outcomes in surgical repair of CHD
- Erin J. Madriago, Rajesh Punn, Natalie Geeter, Norman H. Silverman
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- Published online by Cambridge University Press:
- 02 March 2015, pp. 263-268
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Objectives
Trans-oesophageal echocardiographic imaging is valuable in the pre- and post-operative evaluation of children and adults with CHD; however, the frequency by which trans-oesophageal echocardiography guides the intra-operative course of patients is unknown.
MethodsWe retrospectively reviewed 1748 intra-operative trans-oesophageal echocardiograms performed between 1 October, 2005 and 31 December, 2010, and found 99 cases (5.7%) that required return to bypass, based in part upon the intra-operative echocardiographic findings.
ResultsThe diagnoses most commonly requiring further repair and subsequent imaging were mitral valve disease (20.9%), tricuspid valve disease (16.0%), atrioventricular canal defects (12.0%), and pulmonary valve disease (14.1%). The vast majority of those requiring immediate return to bypass benefited by avoiding subsequent operations and longer lengths of hospital stay. A total of 14 patients (0.8%) who received routine imaging required further surgical repair within 1 week, usually due to disease that developed over ensuing days. Patients who had second post-operative trans-oesophageal echocardiograms in the operating room rarely required re-operations, confirming the benefit of routine intra-operative imaging.
ConclusionsThis study represents a large single institutional review of intra-operative trans-oesophageal echocardiography, and confirms its applicability in the surgical repair of patients with CHD. Routine imaging accurately identifies patients requiring further intervention, does not confer additional risk of mortality or prolonged length of hospital stay, and prevents subsequent operations and associated sequelae in a substantial subset of patients. This study demonstrates the utility of echocardiography in intra-operative monitoring of surgical repair and highlights patients who are most likely to require return to bypass, as well as the co-morbidities of such manipulations.
The Eisenmenger malformation: a morphologic study
- Angelo Restivo, Cira R. T. di Gioia, Robert H. Anderson, Raffaella Carletti, Pietro Gallo
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- 17 February 2015, pp. 269-279
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We studied 11 autopsied cases of the Eisenmenger malformation, comparing the findings with 11 hearts with intact ventricular septal structures, and nine hearts having perimembranous ventricular septal defects in the absence of aortic overriding. We found variable lengths for the subpulmonary infundibulum in the hearts with Eisenmenger defects. It was well developed in three hearts, of intermediate length in seven, and very short in one of the specimens. The muscular outlet septum was also of variable length compared with the free-standing subpulmonary infundibular sleeve. Except for one, all hearts had fibrous continuity between the aortic and tricuspid valvar leaflets, such that the ventricular septal defect was then perimembranous. In the remaining case, there was a completely subaortic muscular infundibulum, with the ventricular septal defect showing only muscular borders. The medial papillary muscle was absent in the majority of cases, but was well formed in three hearts, all with relatively short muscular outlet septums. We identified mild, intermediate, and severe degrees of rightward rotation of the aortic valve, and these findings correlated with the extent of aortic valvar overriding. In nine of the 11 hearts, the ventriculo-arterial connections were concordant, but there was double-outlet from the right ventricle in the other two specimens. Based on our anatomic and morphometric observations, we conclude that the hearts we have defined as having Eisenmenger defects show marked individual variation in their specific phenotypic anatomy.
Cardiovascular and metabolic risk markers are related to parasympathetic indices in pre-pubertal adolescents
- Suziane U. Cayres, Luiz Carlos M. Vanderlei, Danilo R. P. Silva, Manoel Carlos S. Lima, Maurício F. Barbosa, Rômulo A. Fernandes
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- Published online by Cambridge University Press:
- 24 February 2015, pp. 280-287
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Objective
To analyse the relationship between different heart rate variability indices, resting heart rate, and cardiovascular markers in adolescents.
MethodsA cross-sectional study was carried out with information from an ongoing cohort study. The sample was composed of 99 adolescents who complied with the following inclusion criteria: aged between 11 and 14 years; enrolled in a school unit of elementary education; absence of any known diseases; no drug consumption; and a formal consent signed by the parents or legal guardians. Weight, height, heart rate variability, lipid profile, inflammatory markers, blood pressure, resting heart rate, intima-media thickness, blood flow, and trunk fatness were measured. Partial correlation and linear regression (expressed by β and 95% confidence intervals [95%CI]) analyses were used to analyse the relationships between the variables.
ResultsIn the linear regression analysis, even after adjustments for sex, age, trunk fatness, and somatic maturation, parasympathetic activity presented significant correlations with maximum carotid artery blood flow (β=−0.111 [95%CI=−0.216; −0.007]), systolic blood pressure (β=−0.319 [95%CI=−0.638; −0.001]), and resting heat rate (β=−0.005 [95%CI=−0.009; −0.002]).
ConclusionParasympathetic activity at rest is inversely related to maximum and minimum blood flow, triacylglycerol levels, and systolic blood pressure. These findings suggest that heart rate variability has the potential to discriminate pre-pubertal adolescents at increased risk.
Current outcomes of the bi-directional cavopulmonary anastomosis in single ventricle patients: analysis of risk factors for morbidity and mortality, and suitability for Fontan completion
- Katrien François, Kristof Vandekerckhove, Katya De Groote, Joseph Panzer, Daniel De Wolf, Hans De Wilde, Thierry Bové
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- Published online by Cambridge University Press:
- 23 February 2015, pp. 288-297
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Objectives
The bi-directional cavopulmonary anastomosis forms an essential staging procedure for univentricular hearts. This review aims to identify risk factors for morbidity, mortality, and suitability for Fontan completion.
MethodsA total of 114 patients undergoing cavopulmonary anastomosis between 1992 and 2012 were reviewed to assess primary – mortality and survival to Fontan completion – and secondary outcome endpoints – re-intubation, new drain, and ICU stay. Median age and weight were 8 months and 6.9 kg, respectively. In 83% of patients, 1–3 interventions had preceded. Norwood-type procedures became more prevalent over time.
ResultsExtubation occurred after a median of 4 hours, median ICU stay was 2 days; 10 patients (8.8%) needed re-intubation and 18 received a new drain. Higher central venous pressure and transpulmonary gradient were risk factors for new drain insertion (p<0.01). Higher pre-operative pulmonary pressure correlated with increased inotropic support and prolonged intubation (p=0.01). Need for re-intubation was significantly affected by younger age at operation (p=0.01). Hospital and pre-Fontan mortality were 11.4 and 5.3%, respectively. Operative mortality was independently affected by younger age (p=0.013), lower weight (p=0.02), longer bypass time (p=0.04), and re-intubation (p=0.004). Interstage mortality was mainly influenced by moderate ventricular function (p=0.03); 82% of survivors underwent or are candidates for Fontan completion.
ConclusionThe cavopulmonary anastomosis remains associated with adverse outcomes. Age at operation decreases with rising prevalence of complex univentricular hearts. Considering the important impact of re-intubation on hospital mortality, peri-operative management should focus on optimising cardio-respiratory status. Once this selection step is taken, successful Fontan completion can be expected, provided that ventricular function is maintained.
Exercise-induced ventricular re-polarisation changes in moderate congenital aortic valve stenosis
- Louis-Simon Beauséjour, Hugo Gravel, Wadi Mawad, Nagib Dahdah, Daniel Curnier
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- 23 February 2015, pp. 298-305
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Introduction
Pressure overload increases in patients with moderate aortic valvular stenosis during exercise. In the absence of symptoms, it remains difficult, however, to discriminate patients for surgery based only on pressure overload. Other parameters, such as the dispersion of ventricular re-polarisation (d-QT), which reportedly increases with the transvalvular pressure gradient, have not been fully studied in this condition.
ObjectiveTo determine the pattern of QT and d-QT response to exercise testing in children with moderate aortic valve stenosis in order to evaluate the impact of pressure overload from an electrophysiological perspective.
Materials and methodsIn all, 15 patients were compared with 15 controls paired for age (14.8±2.5 versus 14.2±1.5 years old) and gender (66.7% male). All the patients underwent exercise stress testing with 12-lead electrocardiograph recording. QT was measured from the onset of QRS to the apex (QTa) at rest, at peak exercise, and at 1 and 3 minutes upon recovery. QT was corrected using the Fridericia equation, and d-QT was calculated.
ResultsResting QTc was similar among the study groups, but increased significantly in study patients compared with the control group at maximal effort (p=0.004) and after 1 (p<0.001) and 3 (p<0.001) minutes of recovery. A significant association was identified between groups for d-QT (p=0.034), and post-hoc tests revealed a significant difference only at rest (p=0.001).
ConclusionsVentricular re-polarisation abnormalities can be unmasked and highlighted by the assessment of electrical re-polarisation during exercise challenge in patients with asymptomatic moderate aortic valve stenosis. Using QT response to exercise could be beneficial for better optimisation of risk stratification in these patients.
Cardiac MRI in patients with complex CHD following primary or secondary implantation of MRI-conditional pacemaker system
- Nadya Al-Wakeel, Darach O h-Ici, Katharina R. Schmitt, Daniel R. Messroghli, Eugénie Riesenkampff, Felix Berger, Titus Kuehne, Bjoern Peters
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- Published online by Cambridge University Press:
- 23 February 2015, pp. 306-314
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Objectives
In patients with CHD, cardiac MRI is often indicated for functional and anatomical assessment. With the recent introduction of MRI-conditional pacemaker systems, cardiac MRI has become accessible for patients with pacemakers. The present clinical study aims to evaluate safety, susceptibility artefacts, and image reading of cardiac MRI in patients with CHD and MRI-conditional pacemaker systems.
Material and methodsCHD patients with MRI-conditional pacemaker systems and a clinical need for cardiac MRI were examined with a 1.5-T MRI system. Lead function was tested before and after MRI. Artefacts and image readings were evaluated using a four-point grading scale.
ResultsA total of nine patients with CHD (mean age 34.0 years, range 19.5–53.6 years) received a total of 11 cardiac MRI examinations. Owing to clinical indications, seven patients had previously been converted from conventional to MRI-conditional pacemaker systems. All MRI examinations were completed without adverse effects. Device testing immediately after MRI and at follow-up showed no alteration of pacemaker device and lead function. Clinical questions could be addressed and answered in all patients.
ConclusionCardiac MRI can be performed safely with high certainty of diagnosis in CHD patients with MRI-conditional pacemaker systems. In case of clinically indicated lead and box changing, CHD patients with non-MRI-conditional pacemaker systems should be considered for complete conversion to MRI-conditional systems.
The morphology of the coronary sinus in patients with congenitally corrected transposition: implications for cardiac catheterisation and re-synchronisation therapy
- Vera D. Aiello, Flávia C. N. Ferreira, Mauricio I. Scanavacca, Robert H. Anderson, André D’Avila
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- Published online by Cambridge University Press:
- 03 March 2015, pp. 315-320
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Patients with congenitally corrected transposition frequently benefit from re-synchronisation therapy or ablation procedures. This is likely to require catheterisation of the coronary sinus. Its anatomy, however, is not always appreciated, despite being well-described. With this caveat in mind, we have evaluated its location and structure in hearts with congenitally corrected transposition in order to reinforce the guidance needed by the cardiac interventionist. We dissected and inspected the coronary sinus, the oblique vein of the left atrium, and the left-sided-circumflex venous channel in eight heart specimens with corrected transposition and eight controls, measuring the orifice and length of the sinus and the atrioventricular valves. In two-thirds of the malformed hearts, the sinus deviated from its anticipated course in the atrioventricular groove, ascending obliquely on the left atrial inferior wall to meet the left oblique vein. The maximal deviation coincided in all hearts with the point where the left oblique vein joined the left-sided-circumflex vein to form the coronary sinus. We describe a circumflex vein, rather than the great cardiac vein, as the latter venous channel is right-sided in the setting of corrected transposition. The length of the sinus correlated positively with the diameter of the tricuspid valve (p=0.02). Compared with controls, the left oblique vein in the malformed hearts joined the circumflex venous channel significantly closer to the mouth of the sinus. The unexpected course of the coronary sinus in corrected transposition and the naming of the cardiac veins have important implications for venous cannulation and interpretation of images.
The angulation of the septal structures impacts ventricular imbalance in atrioventricular septal defects with a common atrioventricular junction
- Zaheer Ahmad, Zek Lim, Kevin Roman, Marcus Haw, Robert H. Anderson, Joseph Vettukattil
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- 03 March 2015, pp. 321-326
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Objective
Multiplanar re-formatting of full-volume three-dimensional echocardiography data sets offers new insights into the morphology of atrioventricular septal defects. We hypothesised that distortion of the alignment between the atrial and ventricular septums results in imbalanced venous return to the ventricles, with consequent proportional ventricular hypoplasia.
MethodsA single observer evaluated 31 patients, with a mean age of 52.09 months, standard deviation of 55, and with a range from 2 to 264 months, with atrioventricular septal defects, of whom 17 were boys. Ventricular imbalance, observed in nine patients, was determined by two-dimensional assessment, and confirmed at surgical inspection in selected cases when a univentricular strategy was undertaken. Offline analysis using multiplanar re-formatting was performed. A line was drawn though the length of the ventricular septum and a second line along the plane of the atrial septum, taking the angle between these two lines as the atrioventricular septal angle. We compared the angle between 22 patients with adequately sized ventricles, and those with ventricular imbalance undergoing univentricular repair.
ResultsIn the 22 patients undergoing biventricular repair, the septal angle was 0 in 14 patients; the other eight patients having angles ranging from 1 to 36, with a mean angle of 7.4°, and standard deviation of 11.1°.The mean angle in the nine patients with ventricle imbalance was 28.6°, with a standard deviation of 3.04°, and with a range from 26 to 35°. Of those undergoing univentricular repair, two patients died, with angles of 26 and 30°, respectively.
ConclusionsThe atrioventricular septal angle derived via multiplanar formatting gives important information regarding the degree of ventricular hypoplasia and imbalance. When this angle is above 25°, patients are likely to have ventricular imbalance requiring univentricular repair.
Challenges of intra-institutional transfer of care from paediatric to adult congenital cardiology: the need for retention as well as transition
- Claudine M. Bohun, Patricia Woods, Christiane Winter, Julie Mitchell, Joel McLarry, Joseph Weiss, Craig S. Broberg
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- Published online by Cambridge University Press:
- 13 April 2015, pp. 327-333
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Background
Transferring patients with CHD from paediatric to adult care has been challenging, especially across institutions. Within a single institution, some issues such as provider interaction, information exchange, or administrative directives should not play a significant role, and should favour successful transfer.
ObjectiveWe studied patients who were eligible for transfer to the adult congenital heart disease service within our institution in order to identify factors associated with successful transfer to adult care providers versus failure to transfer.
MethodsPatients above18 years of age with CHD who were seen by paediatric cardiologists before January, 2008 were identified through a patient-care database. Records were reviewed to determine follow-up between 2008 and 2011 and to determine whether the patient was seen in the adult congenital cardiology clinic, paediatric cardiology clinic, or had no follow-up, and statistical comparisons were made between groups.
ResultsAfter reviewing 916 records, 229 patients were considered eligible for transition to adult congenital cardiology. Of these, 77 (34%) were transferred successfully to adult congenital cardiology, 47 (21%) continued to be seen by paediatric cardiologists, and 105 (46%) were lost to follow-up. Those who transferred successfully differed with regard to complexity of diagnosis, insurance, and whether a formal referral was made by a paediatric care provider. Only a small fraction of the patients who were lost to follow-up could be contacted.
ConclusionWithin a single institution, with shared information systems, administrations, and care providers, successful transfer from paediatric to adult congenital cardiology was still poor. Efforts for successful retention are just as vital as those for transfer.
Absent right superior caval vein in situs solitus
- Rebekka Lytzen, Karin Sundberg, Niels Vejlstrup
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- Published online by Cambridge University Press:
- 26 March 2015, pp. 334-339
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Introduction
In up to 0.07% of the general population, the right anterior cardinal vein obliterates and the left remains open, creating an absent right superior caval vein and a persistent left superior caval vein. Absent right superior caval vein is associated with additional congenital heart disease in about half the patients. We wished to study the consequences of absent right superior caval vein as an incidental finding on prenatal ultrasonic malformation screening.
Material and methodsThis is a retrospective case series study of all foetuses diagnosed with absent right superior caval vein at the national referral hospital, Rigshospitalet, Denmark, from 2009 to 2012.
ResultsIn total, five cases of absent right superior caval vein were reviewed. No significant associated cardiac, extra-cardiac, or genetic anomalies were found. Postnatal echocardiographies confirmed the diagnosis and there were no postnatal complications. All children were found to have healthy hearts at follow-up.
ConclusionsIn all cases, the findings proved to be a benign condition with no clinical manifestations or complications. Although isolated absent right superior caval vein does not seem to affect the outcome, associated anomalies may be serious. Absent right superior caval vein should, therefore, prompt a search for additional malformations. Furthermore, the diagnosis of an isolated absent right superior caval vein is important, because knowledge of the anomaly can prevent future problems when invasive procedures are necessary.
Duplicated left pulmonary artery: an unknown disease? Three case reports and review of the literature
- Valentina Giudici, Mazyar Kanani, Nagarajan Muthialu, Michelle Carr, Alistair D. Calder, Catherine M. Owens, Andrew C. Cook, Jan Marek
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- Published online by Cambridge University Press:
- 05 March 2015, pp. 340-346
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We report three cases of an abnormal finding of duplicated left pulmonary artery: two of these occurring in children with Kabuki syndrome and configuring the setting of a pseudo-pulmonary sling without any clinical or cardiac cross-sectional evidence of tracheal compression. The other case instead represents duplicated left pulmonary artery with pulmonary sling caused by the retro-tracheal course of the lower left pulmonary artery associated with “Christmas Tree” arrangement of the tracheo-bronchial system.
In both patients with pseudo-pulmonary sling and Kabuki syndrome, the abnormal finding was incidental during echocardiographic examination and neither of the patients required surgical repair for the condition. To the best of our knowledge, they represent the third and fourth cases in which such an anomaly of the pulmonary artery branches not forming a sling is seen in association with Kabuki syndrome. Another case represents our second experience and the second case reported in literature with duplicated left pulmonary artery in the setting of a complex tracheal anatomy. In this symptomatic patient, surgical repair of atrial septal defect and relief of the vascular ring were indicated, and the surgical repair was performed successfully at the age of 3 years.
Prenatal diagnosis of CHDs: a simple ultrasound prediction model to estimate the probability of the need for neonatal cardiac invasive therapy
- Pablo Marantz, Sofía Grinenco, Fabio Pestchanker, César H. Meller, Gustavo Izbizky
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- Published online by Cambridge University Press:
- 27 April 2015, pp. 347-353
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Objectives
To develop a prediction model based on echocardiographic findings to estimate the probability of the need for neonatal cardiac invasive therapy, including cardiac surgery or catheter-based therapy, in foetuses with CHD.
MethodsRetrospective cohort study: a prediction model was developed based on echocardiographic findings on the examination of the four-chamber, the three-vessel, and the three-vessel and tracheal views. We assessed performance using the area under the curve of the receiver operating characteristic.
ResultsAmong 291 patients with prenatal diagnosis of CHD and complete follow-up, 175 (60.1%) required neonatal cardiac invasive therapy. The variables “functionally single ventricle”, “great artery reverse flow”, and “congenital heart block” had a discrimination value of 100% and were excluded from the model. In univariate and multivariate analysis, “non-visualisation of a great vessel”, “asymmetry of the great vessels”, “visualisation of one atrioventricular valve”, and “ventricular asymmetry” were significantly associated with the need for neonatal cardiac invasive therapy. The area under the receiver operating characteristic curve was 0.9324 (95% CI 0.92–0.97).
ConclusionsA prediction model based on echocardiographic findings in foetuses with CHD, even without a definite diagnosis, allows an accurate estimation of the probability of requiring neonatal cardiac invasive therapy. This can modify patient care, especially in regions where a Foetal Medicine Specialist or a Paediatric Cardiologist is not available and referral may be extremely difficult due to social and economic barriers.
Pharmacokinetics of intravenous sildenafil in children with palliated single ventricle heart defects: effect of elevated hepatic pressures
- Kevin D. Hill, Mario R. Sampson, Jennifer S. Li, Robert D. Tunks, Scott R. Schulman, Michael Cohen-Wolkowiez
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- Published online by Cambridge University Press:
- 01 April 2015, pp. 354-362
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Aims
Sildenafil is frequently prescribed to children with single ventricle heart defects. These children have unique hepatic physiology with elevated hepatic pressures, which may alter drug pharmacokinetics. We sought to determine the impact of hepatic pressure on sildenafil pharmacokinetics in children with single ventricle heart defects.
MethodsA population pharmacokinetic model was developed using data from 20 single ventricle children receiving single-dose intravenous sildenafil during cardiac catheterisation. Non-linear mixed effect modelling was used for model development, and covariate effects were evaluated based on estimated precision and clinical significance.
ResultsThe analysis included a median (range) of 4 (2–5) pharmacokinetic samples per child. The final structural model was a two-compartment model for sildenafil with a one-compartment model for des-methyl-sildenafil (active metabolite), with assumed 100% sildenafil to des-methyl-sildenafil conversion. Sildenafil clearance was unaffected by hepatic pressure (clearance=0.62 L/hour/kg); however, clearance of des-methyl-sildenafil (1.94×(hepatic pressure/9)−1.33 L/hour/kg) was predicted to decrease ~7-fold as hepatic pressure increased from 4 to 18 mmHg. Predicted drug exposure was increased by ~1.5-fold in subjects with hepatic pressures ⩾10 versus <10 mmHg (median area under the curve=533 versus 792 µg*h/L).
DiscussionElevated hepatic pressure delays clearance of the sildenafil metabolite – des-methyl-sildenafil – and increases drug exposure. We speculate that this results from impaired biliary clearance. Hepatic pressure should be considered when prescribing sildenafil to children. These data demonstrate the importance of pharmacokinetic assessments in patients with unique cardiovascular physiology that may affect drug metabolism.
Images in Congenital Cardiac Disease
A classical case of the Gasul phenomenon
- Girish R. Sabnis, Milind S. Phadke, Prafulla G. Kerkar
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- Published online by Cambridge University Press:
- 28 October 2015, pp. 363-364
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This case demonstrates the development of secondary infundibular stenosis in a 10-year-old male child with documented large non-restrictive perimembranous ventricular septal defect in infancy – the classical Gasul phenomenon.