Purpose: To explore the use of three-dimensional patient-specific cardiovascular models using rapid prototyping techniques (fused deposition modelling) to improve surgical planning in patients with complex congenital heart disease. Description: Rapid prototyping techniques are used to print accurate three-dimensional replicas of patients' cardiovascular anatomy based on magnetic resonance images using computer-aided design systems. Models are printed using a translucent polylactic acid polymer. Evaluation: As a proof of concept, a model of the heart of a 1.5-year-old boy with transposition of the great arteries, ventricular septal defect and pulmonary stenosis was constructed to help planning the surgical correction. The cardiac model allowed the surgeon to evaluate the location and dimensions of the ventricular septal defect as well as its relationship with the aorta and pulmonary artery. Conclusions: Cardiovascular models constructed by rapid prototyping techniques are extremely helpful for planning corrective surgery in patients with complex congenital malformations. Therefore they may potentially reduce operative time and morbi-mortality.
Despite many advances in recent years for patients with critical paediatric and congenital cardiac disease, significant variation in outcomes remains across hospitals. Collaborative quality improvement has enhanced the quality and value of health care across specialties, partly by determining the reasons for variation and targeting strategies to reduce it. Developing an infrastructure for collaborative quality improvement in paediatric cardiac critical care holds promise for developing benchmarks of quality, to reduce preventable mortality and morbidity, optimise the long-term health of patients with critical congenital cardiovascular disease, and reduce unnecessary resource utilisation in the cardiac intensive care unit environment. The Pediatric Cardiac Critical Care Consortium (PC4) has been modelled after successful collaborative quality improvement initiatives, and is positioned to provide the data platform necessary to realise these objectives. We describe the development of PC4 including the philosophical, organisational, and infrastructural components that will facilitate collaborative quality improvement in paediatric cardiac critical care.
Background: Few data are available on the neuropsychological, behavioural, or structural brain imaging outcomes in adolescents who underwent corrective surgery in infancy for tetralogy of Fallot. Methods: In this single-centre cross-sectional study, we enrolled 91 adolescents (13-16 years old) with tetralogy of Fallot and 87 referent subjects. Assessments included tests of academic achievement, memory, executive functions, visual-spatial skills, attention, and social cognition, as well as brain magnetic resonance imaging. Results: Genetic abnormalities or syndromes were present in 25% of tetralogy of Fallot patients, who had markedly greater neuropsychological morbidities than did patients without a syndrome. However, even patients without a syndrome performed significantly worse than the referent group or population norms in all of the neuropsychological domains assessed. In multivariable regression in those without a genetic/phenotypic syndrome, the strongest predictors of adverse late neurodevelopmental outcomes included a greater number of complications at the first operation, more total surgical complications across all operations, and occurrence of post-operative seizures. The presence of at least one abnormality on structural magnetic resonance imaging was more frequent in tetralogy of Fallot patients than the referent group (42% versus 8%). Conclusions: Adolescents with tetralogy of Fallot are at increased neurodevelopmental risk and would benefit from ongoing surveillance and educational supports even after childhood.
Background At present, the exclusion for percutaneous pulmonary valve implantation for free pulmonary regurgitation after tetralogy of Fallot repair includes an unfavourably large right ventricular outflow tract. Objective To report feasibility and early experience with a recently developed transcatheter heart valve, Venus P-valve(TM), implanted in six patients with severe pulmonary regurgitation with large right ventricular outflow tracts. Patients There were two female patients and four male patients. The median age of the patients was 18.5 years, and the mean body weight was 53.8 kg. All the patients were in NYHA class II and had severe pulmonary regurgitation after previous transannular patch repair of tetralogy of Fallot. The median time after the last surgical operation was 13.5 years. Results The Venus P-valve(TM) was successfully implanted in all the patients with implanted valve diameters ranging from 24 to 32 mm. The mean fluoroscopy time was 29.8 minutes. None of the patients had significant outflow tract gradient or pulmonary regurgitation immediately after valve implantation. Only one patient had unexpected mild proximal valve migration to the right ventricular body during withdrawal of the delivery system. It caused mild paravalvar leak and significant tricuspid regurgitation. At 6 months follow-up, the median of right ventricular end-diastolic volume indices decreased from 146 to 108 ml/m(2) (p-value=0.046). The Doppler systolic peak gradient across the valve ranged from 4 to 40 mmHg, and there was no evidence of stent fracture on fluoroscopy or structural valve failure. Conclusion The Venus P-valve(TM) can be implanted successfully and effectively in patients with severe pulmonary regurgitation and a large right ventricular outflow tract. The early results with this valve are encouraging.
An internationally approved and globally used classification scheme for the diagnosis of CHD has long been sought. The International Paediatric and Congenital Cardiac Code (IPCCC), which was produced and has been maintained by the International Society for Nomenclature of Paediatric and Congenital Heart Disease (the International Nomenclature Society), is used widely, but has spawned many "short list" versions that differ in content depending on the user. Thus, efforts to have a uniform identification of patients with CHD using a single up-to-date and coordinated nomenclature system continue to be thwarted, even if a common nomenclature has been used as a basis for composing various "short lists". In an attempt to solve this problem, the International Nomenclature Society has linked its efforts with those of the World Health Organization to obtain a globally accepted nomenclature tree for CHD within the 11th iteration of the International Classification of Diseases (ICD-11). The International Nomenclature Society has submitted a hierarchical nomenclature tree for CHD to the World Health Organization that is expected to serve increasingly as the "short list" for all communities interested in coding for congenital cardiology. This article reviews the history of the International Classification of Diseases and of the IPCCC, and outlines the process used in developing the ICD-11 congenital cardiac disease diagnostic list and the definitions for each term on the list. An overview of the content of the congenital heart anomaly section of the Foundation Component of ICD-11, published herein in its entirety, is also included. Future plans for the International Nomenclature Society include linking again with the World Health Organization to tackle procedural nomenclature as it relates to cardiac malformations. By doing so, the Society will continue its role in standardising nomenclature for CHD across the globe, thereby promoting research and better outcomes for fetuses, children, and adults with congenital heart anomalies.
Children with single-ventricle disease experience high mortality and complex care. In other life-limiting childhood illnesses, paediatric palliative care may mitigate maternal stress. We hypothesised that early palliative care in the single-ventricle population may have the same benefit for mothers. In this pilot randomised trial of early palliative care, mothers of infants with prenatal single-ventricle diagnoses completed surveys measuring depression, anxiety, coping, and quality of life at a prenatal visit and neonatal discharge. Infants were randomised to receive early palliative care - structured evaluation, psychosocial/spiritual, and communication support before surgery - or standard care. Among 56 eligible mothers, 40 enrolled and completed baseline surveys; 38 neonates were randomised, 18 early palliative care and 20 standard care; and 34 postnatal surveys were completed. Baseline Beck Depression Inventory-II and State-Trait Anxiety Index scores exceeded normal pregnant sample scores (mean 13.76 +/- 8.46 versus 7.0 +/- 5.0 and 46.34 +/- 12.59 versus 29.8 +/- 6.35, respectively; p=0.0001); there were no significant differences between study groups. The early palliative care group had a decrease in prenatal to postnatal State-Trait Anxiety Index scores (-7.6 versus 0.3 in standard care, p=0.02), higher postnatal Brief Cope Inventory positive reframing scores (p=0.03), and a positive change in PedsQL Family Impact Module communication and family relationships scores (effect size 0.46 and 0.41, respectively). In conclusion, these data show that mothers of infants with single-ventricle disease experience significant depression and anxiety prenatally. Early palliative care resulted in decreased maternal anxiety, improved maternal positive reframing, and improved communication and family relationships.
Recurrent aortic arch obstruction following the Norwood procedure is recognised as an important complication. Balloon arch angioplasty is associated with a high recoarctation rate. We sought to evaluate the prevalence and outcome of stent implantation for recoarctation in children following Norwood or Damus-Kaye-Stansel procedure over the past decade at a single national cardiology centre. Of 114 children who underwent Norwood procedure or Damus-Kaye-Stansel procedure between January 2003 and June 2013, 80 patients survived. Of these 15 children underwent stent implantation for recoarctation. Six of these patients had previous balloon angioplasty. The median age at stent implantation was 4.4 months (range 2-82 months). The median peak aortic arch gradient at catheterisation decreased from 26mmHg (range 10-70mmHg) to 2mmHg (range 0-20mmHg). The median luminal diameter increased from 4.7 mm (range 3.2-7.9 mm) to 8.6 mm (range 6.2-10.9 mm). The median coarctation index increased by 0.49 (range = 0.24-0.64). A Valeo stent was employed in 11 children, a Palmaz Genesis stent in 2 patients, a MultiLink stent in 1 child, and a Jomed covered stent in 1 child. Two factors were associated with the need for stent placement: previous arch angioplasty (p valve < 0.001, χ-square 11.5) and borderline left ventricle (p = 0.04, χ-square = 4.1). Stent migration occurred in one child. There were two deaths related to poor right ventricular systolic function and severe tricuspid regurgitation. Six patients underwent redilation of the stent with no complications. The prevalence of recurrent aortic arch obstruction following Norwood/Damus-Kaye-Stansel procedure was 18%. Stent implantation is safe and reliably eliminates the aortic obstruction. Redilation can be successfully achieved to accommodate somatic growth or development of stent recoarctation.
Pheochromocytoma/paraganglioma is an exceedingly rare tumour, thought to share an association with cyanotic CHD. This association is thought to be a result of chronic hypoxaemia (Antonio et al, Revista Española de Cardiología (English Edition) 2017; 70: 673-675; Folger et al, Circulation 1964; 29: 750-757; Opotowsky et al, J Clin Endocrinol Metab 2015; 100: 1325-1334) We report two cases of paraganglioma over a 4-year period in patients with hypoplastic left heart syndrome who had undergone Fontan completion by ages 2 and 4. Based on a very small number of reported cases of CHD, the mechanism of tumourigenesis is unclear. It is imperative that cases associated with CHD continue to be reported so that we may learn more about the pathogenesis and epidemiology of this entity.
The Melody valve, designed for implantation into the pulmonary outflow tract, can also be used to treat the pathology of atrioventricular (AV) valves. Increasing gradients are seen as an indication for re-dilating the valve. Our case demonstrates the heart rate dependency of the gradient across a Melody implanted in the left AV valve position in an infant. Beta blockers were used to lower both heart rate and gradient.
Purulent pericarditis leading to constrictive pericarditis is a rare but serious complication following invasive pneumococcal infection. Early recognition of this complication is crucial to prevent mortality. Here, we report a previously healthy child who developed constrictive pericarditis due to purulent pericarditis following necrotising pneumococcal pneumonia, which is not common in this current antibiotic and pneumococcal vaccine era. The child was successfully treated with pericardiectomy.
Pulmonary embolism is frequently under-recognised in children and, therefore, a high index of suspicion should be exerted on patients with exertional dyspnoea, presyncope/syncope and unexplained cardiopulmonary arrest. We discuss a 10-year-old previously healthy girl who presented with syncope and subsequent cardiac arrest related to massive pulmonary embolism and was salvaged successfully by emergent pulmonary embolectomy.
A right aortic arch with an isolated left innominate artery is a rare form of aortic arch anomaly. We present a case of neonatal diagnosis of this anomaly with concerning findings of global cerebral white matter atrophy at 13 months of age.
Splanchnic circulation constitutes a major portion of the vasculature capacitance and plays an important role in maintaining blood perfusion. Because patients with asplenia syndrome lack this vascular bed as a blood reservoir, they may have a unique blood volume and distribution, which may be related to their vulnerability to the haemodynamic changes often observed in clinical practice. During cardiac catheterisation, the mean circulatory filling pressure was calculated with the Valsalva manoeuvre in 19 patients with Fontan circulation, including 5 patients with asplenia syndrome. We also measured the cardiac output index and circulatory blood volume by using a dye dilution technique. The blood volume and the mean circulatory filling pressure and the venous capacitance in patients with asplenia syndrome were similar to those in the remaining patients with Fontan circulation (85 ± 14 versus 77 ± 18 ml/kg, p = 0.43, 31 ± 8 versus 27 ± 5 mmHg, p = 0.19, 2.8 ± 0.6 versus 2.9 ± 0.9 ml/kg/mmHg, p = 0.86). Unexpectedly, our data indicated that patients with asplenia syndrome, who lack splanchnic capacitance circulation, have blood volume and venous capacitance comparable to those in patients with splanchnic circulation. These data suggest that (1) there is a blood reservoir other than the spleen even in patients with asplenia; (2) considering the large blood pool of the spleen, the presence of a symmetrical liver may represent the possible organ functioning as a blood reservoir in asplenia syndrome; and (3) if this is indeed the case, there may be a higher risk of hepatic congestion in patients with Fontan circulation with asplenia syndrome than in those without.
Infants with single ventricle physiology have arterial oxygen saturations between 75 and 85%. Home monitoring with daily pulse oximetry is associated with improved interstage survival. They are typically sent home with expensive, bulky, hospital-grade pulse oximeters. This study evaluates the accuracy of both the currently used Masimo LNCS and a relatively inexpensive, portable, and equipped with Bluetooth technology study device, by comparing with the gold standard co-oximeter. Prospective, observational study. Single institution, paediatric cardiac critical care unit, and neonatal ICU. none. Twenty-four infants under 12 months of age with baseline oxygen saturation less than 90% due to cyanotic CHD. Pulse oximetry with WristOx2 3150 with infant sensors 8008 J (study device) and Masimo LCNS saturation sensor connected to a Philips monitor (hospital device) were measured simultaneously and compared to arterial oxy-haemoglobin saturation measured by co-oximetry. Statistical analysis evaluated the performances of each and compared to co-oximetry with Schuirmann's TOST equivalence tests, with equivalence defined as an absolute difference of 5% saturation or less. Neither the study nor the hospital device met the predefined standard for equivalence when compared with co-oximetry. The study device reading was on average 4.0% higher than the co-oximeter, failing to show statistical equivalence (p = 0.16). The hospital device was 7.4% higher than the co-oximeter and also did not meet the predefined standard for equivalence (p = 0.97). Both devices tended to overestimate oxygen saturation in this patient population when compared to the gold standard, co-oximetry. The study device is at least as accurate as the hospital device and offers the advantage of being more portable with Bluetooth technology that allows reliable, efficient data transmission. Currently FDA-approved, smaller portable pulse oximeters can be considered for use in home monitoring programmes.
Elevated left ventricular end diastolic pressure is a risk factor for ventricular arrhythmias in patients with tetralogy of Fallot. The objective of this retrospective study was to identify echocardiographic measures associated with left ventricular end diastolic pressure >12 mmHg in this population. Repaired tetralogy of Fallot patients age ≥13 years, who underwent a left heart catheterisation within 7 days of having an echocardiogram were evaluated. Univariate comparison was made in echocardiographic and clinical variables between patients with left ventricular end diastolic pressure >12 versus ≤12 mmHg. Ninety-four patients (54% male) with a median age of 24.6 years were included. Thirty-four (36%) had left ventricular end diastolic pressure >12 mmHg. Patients with left ventricular end diastolic pressure >12mmHg were older (median 32.9 versus 24.0 years, p = 0.02), more likely to have a history of an aortopulmonary shunt (62% versus 38%, p = 0.03), and have a diagnosis of hypertension (24% versus 7%, p = 0.03) compared to those with left ventricular end diastolic pressure ≤12 mmHg. There were no significant differences in mitral valve E/A ratio, annular e' velocity, or E/e' ratio between patients with left ventricular end diastolic pressure >12 versus ≤12 mmHg. Patients with left ventricular end diastolic pressure >12mmHg had larger left atrial area (mean 17.7 versus 14.0 cm2, p = 0.03) and larger left atrium anterior-posterior diameter (mean 36.0 versus 30.6 mm, p = 0.004). In conclusion, typical echocardiographic measures of left ventricular diastolic dysfunction may not be reliable in tetralogy of Fallot patients. Prospective studies with the use of novel echocardiographic measures are needed.
Pulmonary vein stenosis (PVS) is a condition with challenging treatment and leads to severe cardiac failure and pulmonary hypertension. Despite aggressive surgical or catheter-based intervention, the prognosis of PVS is unsatisfactory. This study aimed to assess the prognosis and to establish appropriate treatment strategies. We retrospectively reviewed endovascular treatments for PVS (2001-2017) from the clinical database at the Okayama University Hospital. A total of 24 patients underwent PVS associated with total anomalous pulmonary venous connection and 7 patients underwent isolated congenital PVS. In total, 53 stenotic pulmonary veins were subjected to endovascular treatments; 40 of them were stented by hybrid (29) and percutaneous procedures (11) (bare-metal stent, n = 34; drug-eluting stent, n = 9). Stent size of hybrid stenting was larger than percutaneous stenting. Median follow-up duration from the onset of PVS was 24 months (4-134 months). Survival rate was 71 and 49% at 1 and 5 years, respectively. There was no statistically significant difference between stent placement and survival; however, patients who underwent bare-metal stent implantation had statistically better survival than those who underwent drug-eluting stent implantation or balloon angioplasty. Early onset of stenosis, timing of stenting, and small vessel diameter of pulmonary vein before stenting were considered as risk factors for in-stent restenosis. Freedom from re-intervention was 50 and 26% at 1 and 2 years. To improve survival and stent patency, implantation of large stent is important. However, re-intervention after stenting is also significant to obtain good outcome.
We present a case of pulmonary venous baffle obstruction in a child with a history of congenitally corrected transposition status post double switch repair. We highlight two forms of volume rendering three-dimensional reconstructions from computed tomographic data which allowed for detailed pre-surgical planning. These reconstructions emphasise the concept of maximizing previously obtained two-dimensional data in a time-efficient and cost-effective manner. The benefits of these reconstructions are reviewed, highlighting the relatively novel virtual dissection reconstruction technique that appeared identical to what the surgeon encountered in the operating theatre. This technique allowed the surgeon to quickly advance a preconceived detailed surgical repair.
Anomalous single coronary artery from pulmonary artery is a very rare congenital heart anomaly. Anomalous single coronary artery from pulmonary artery has high mortality rates and poor surgical outcome despite advanced surgical techniques. We report a 4-month-old infant presented by congestive heart failure findings and diagnosed with anomalous single trunk coronary arteries arising from right pulmonary artery.
There are limited outcome data in adults with tetralogy of Fallot and pulmonary atresia. The purpose of this study was to describe re-operations and all-cause mortality in adults with tetralogy of Fallot and pulmonary atresia. Retrospective review of adults with repaired tetralogy of Fallot and pulmonary atresia who received care at the Mayo Adult Congenital Heart Disease Clinic, 1990-2016. All-cause mortality was calculated as events per 100 patient-years from the time of first presentation to the Adult Congenital Heart Disease Clinic. Of the 221 patients, the age at initial tetralogy of Fallot repair was 6 (5-13) years, and the age at first presentation to the clinic was 27 - 8 years. All patients had at least one right ventricular to pulmonary artery conduit re-operation. There were 31 deaths (14%) at mean age of 41 - 14 years. The causes of death were end-stage heart failure (n = 17), sudden cardiac death (n=9), post-operative death after cardiac surgery (n = 2), sepsis with multi-system organ failure (n = 2), and unknown (n = 1). All-cause mortality rate was 1.7 per 100 patient-years. The risk factors for all-cause mortality were older age (>12 years) at the time of repair (hazard ratio 1.41, 95 confidence interval 1.06-2.02, p = 0.033), non-sustained ventricular tachycardia (hazard ratio 1.36, 95 confidence interval 1.17-2.47, p = 0.015), and left ventricular ejection fraction <50% (hazard ratio 1.39, 95 confidence interval 1.08-2.31, p = 0.031). Based on a review of 221 adults with repaired tetralogy of Fallot and pulmonary atresia, all patients had re-operations and all-cause mortality rate was 1.7 events per 100 patient-years. The current study provides important outcomes data for risk stratification in adults with tetralogy of Fallot and pulmonary atresia.
To describe the impact of CHD surgery in early childhood on quality of life in children aged 10-16 years with surgically corrected Ventricular Septal Defect, Transposition of the Great Arteries, and Tetralogy of Fallot. A cross-sectional survey study of quality of life survey on 161 children and adolescents aged 10-16 years with surgically corrected Ventricular Septal Defect, Transposition of the Great Arteries, and Tetralogy of Fallot. The international Paediatric Quality of Life 4.0 quality of life questionnaires were applied and collected for assessment from patients and parents. The endpoints were total, physical, emotional, social, and school quality of life scores. The quality of life total and school scores was significantly lower in children with CHD than their healthy peers. There was no significant difference in quality of life between the three CHD groups. All three CHD groups had a significantly lower total (7.7-13.2%, p<0.001) and school scores (21.1-31.6%, p<0.001) than the control group. The tetralogy of Fallot group was the only group that had significantly lower scores in the physical subscale (p<0.001) than the controls. Children and adolescents with surgically corrected CHD show losses in quality of life in total and school scores compared to healthy controls. The tetralogy of Fallot group was the only CHD group that had significantly lower physical score than the controls.