Postural orthostatic tachycardia syndrome encompasses multiple disabling symptoms that interfere with daily activities. Non-pharmacologic approaches can be insufficient and can require adjunctive medications to manage symptoms. Minimal data exist in the literature on medication outcomes in these patients. We reviewed our database for medication management outcomes.

Patients aged 18 years and younger at initial diagnosis met the inclusion criteria. All prescribed patient medications were extracted from the electronic health record, excluding medications for unrelated symptoms or comorbid diseases. Medications were grouped by symptom class consistent with our programme utilisation protocol. Within symptom classification, therapy was deemed successful when a specific dose was prescribed at least five consecutive times without changes; this was confirmed by chart review. Individual medications and overall percentage of successful therapies within symptom classifications were assessed, with further analysis by gender. t-Test, χ2, and Mann–Whitney U-test were used to assess for differences in specific variables, as appropriate.

A total of 708 patients met the study criteria. The percentage of patients with effective therapy by symptom includes light-headedness (52.2%), headache (48.2%), nausea (39.1%), dysmotility (43.4%), pain (53.4%), and insomnia (42.8%). Insomnia therapy was better for females; all other therapies showed no gender difference. The median number of therapies prescribed per patient per symptom was 2 for light-headedness, headache, and insomnia, and 1 for nausea, dysmotility, and pain.

Symptoms associated with this disorder can be effectively managed with various medications. Further randomised studies are needed to better ascertain true efficacy compared with placebo.

Following stage 1 palliation, delayed sternal closure may be used as a technique to enhance thoracic compliance but may also prolong the length of stay and increase the risk of infection.

We reviewed all neonates undergoing stage 1 palliation at our institution between 2010 and 2017 to describe the effects of delayed sternal closure.

During the study period, 193 patients underwent stage 1 palliation, of whom 12 died before an attempt at sternal closure. Among the 25 patients who underwent primary sternal closure, 4 (16%) had sternal reopening within 24 hours. Among the 156 infants who underwent delayed sternal closure at 4 [3,6] days post-operatively, 11 (7.1%) had one or more failed attempts at sternal closure. Patients undergoing primary sternal closure had a shorter duration of mechanical ventilation and intensive care unit length of stay. Patients who failed delayed sternal closure had a longer aortic cross-clamp time (123±42 versus 99±35 minutes, p=0.029) and circulatory arrest time (39±28 versus 19±17 minutes, p=0.0009) than those who did not fail. Failure of delayed sternal closure was also closely associated with Technical Performance Score: 1.3% of patients with a score of 1 failed sternal closure compared with 18.9% of patients with a score of 3 (p=0.0028). Among the haemodynamic and ventilatory parameters studied, only superior caval vein saturation following sternal closure was different between patients who did and did not fail sternal closure (30±7 versus 42±10%, p=0.002). All patients who failed sternal closure did so within 24 hours owing to hypoxaemia, hypercarbia, or haemodynamic impairment.

When performed according to our current clinical practice, sternal closure causes transient and mild changes in haemodynamic and ventilatory parameters. Monitoring of SvO2 following sternal closure may permit early identification of patients at risk for failure.

The aim of this study is to determine early changes in cardiac function of children with chronic kidney disease by using 2D-speckle tracking echocardiography.

The study included 38 children – 16 girls and 22 boys – diagnosed as having chronic kidney disease in the nephrology department with a glomerular filtration rate of <90 ml/minute/1.73 m2 for at least 3 months. A total of 37 – 15 girls and 22 boys – age- and sex-matched healthy children were included as the control group. 2D-Speckle tracking echocardiography was performed in all subjects.

The mean age was 13.45±2.8 years in patients and 12.89±3.07 years in controls. Systolic and diastolic blood pressures and left ventricular mass index were significantly higher in patients (p<0.05). The values of mitral e, mitral a, mitral e/a ratio, and mitral deceleration time were not different between the groups. Tricuspid annular plane systolic excursion values were lower in patients (p<0.01). Global strain values in apical long-axis 3-chamber and 2-chamber views were significantly lower in patients (p<0.05). Longitudinal, radial, and circumferential peak systolic strain values were lower in patients, but the difference was statistically significant in all segments of longitudinal view and basal segment of circumferential view (p<0.05). Radial and circumferential systolic strain rates were significantly lower in patients in all three segments (p<0.05). Moreover, early diastolic strain rate was significantly lower in longitudinal and radial apical segments and in all segments of circumferential measurements in patients. Besides, strain rate e/a ratio was significantly lower in all longitudinal segments of patients (p=0.01).

The study concluded that 2D-speckle tracking echocardiography method can determine cardiac involvement earlier than conventional echocardiography in children with chronic kidney disease having preserved ejection fraction.

Dilated Cardiomyopathy is a serious heart disorder that may induce sudden cardiac death and heart failure. Significant progress has been made in understanding the molecular basis of dilated cardiomyopathy. In previous studies, mutations in more than fifty genes have been identified in dilated cardiomyopathy patients. The purpose of this study was to detect the genetic lesion in a family from the central south of China affected by severe dilated cardiomyopathy.

Whole-exome sequencing combined with cardiomyopathy-related genes list were used to analyse the mutations of the proband. Co-segregation analysis was performed by Sanger sequencing.

Two novel heterozygous mutations – Myosin Binding Protein C: p.L1014RfsX6 and Titin: p.R9793X – were identified in the proband. The deletion mutation c.3041delT/p.L1014RfsX6 caused a premature stop codon at position 1020 in exon 28 of the Myosin Binding Protein C. The nonsense mutation, c.29377 C>T/ p. R9793X, of Titin was located in the highly evolutionarily conserved domain, resulting in truncation of the Titin protein as well. Co-segregation analysis further revealed that the Myosin Binding Protein C mutation came from his mother and the Titin mutation came from his father. Both mutations are reported in dilated cardiomyopathy patients for the first time. Our study not only provides a unique example of the genes and molecular mechanisms involved in dilated cardiomyopathy but also expands the spectrum of Myosin Binding Protein C and Titin mutations and contributes to the genetic diagnosis and counselling of dilated cardiomyopathy patients.

Severe fatigue and cognitive dysfunction are frequent symptoms in patients with postural orthostatic tachycardia syndrome. They can be debilitating, and often do not resolve despite improvement in haemodynamic symptoms. Our analysis was intended to assess clinical outcomes of medication treatment for these symptoms in a large, single-centre paediatric programme.

We performed a retrospective review of patients treated for fatigue and cognitive dysfunction. Patients aged 18 years or younger at the time of initial diagnosis were included. Patients who had a specific medication ordered five or more times for these symptoms were confirmed by chart review for clinical improvement. Percentage of patients with clinical improvement for each medication and overall for all medications, as well as the number of medications per patient required to achieve improvement, were assessed. Data were analysed based on gender as well. t-Test and χ2 analyses were used to assess for differences between means in variables, or specific variables.

A total of 708 patients met study criteria, of whom 517 were treated for fatigue or brain fog. Overall efficacy was 68.8%, with individual medication effectiveness ranging from 53.1 (methylphenidate) to 16.5% (atomoxetine). There was no significant difference in efficacy with respect to gender. The median number of medications used per patient was 2, without gender difference. Therapy was limited by side effects or lack of efficacy.

Medications are effective in the improvement of fatigue and cognitive dysfunction in these patients. However, trials of multiple medications may be needed before achieving clinical improvement.

Balloon atrial septostomy is performed in infants with dextro-transposition of the great arteries to improve oxygenation before surgery. It is performed in the catheterisation laboratory with fluoroscopy or at the bedside using echocardiography. It is unclear whether procedural safety and efficacy is superior in one location versus the other, although the bedside procedure may improve resource utilisation and present an opportunity for reducing cost. This study compares safety and efficacy of atrial septostomy performed at the patient’s bedside versus the catheterisation laboratory.

Neonates with dextro-transposition of the great arteries who underwent balloon atrial septostomy from October, 2000 to January, 2014 were included. Medical and procedural records, echocardiograms, and catheterisation data were reviewed. Comparisons between the two procedural locations included patient demographics, pre- and post-procedure oxygen saturations, and outcomes. Complications reviewed included bleeding, arrhythmia, cardiac trauma, stroke, and death. Coronary artery evaluations were recorded. T-tests were used for continuous variables, and Fisher’s exact tests were used for all categorical variables. Wilcoxon rank sum and analysis of covariance modelling were used for time variables and oxygen saturation, respectively.

A total of 88 infants met the inclusion criteria. Among them, 53 underwent septostomy at the bedside and 35 underwent septostomy in the catheterisation laboratory. No safety or outcome benefit was identified between the two procedural locations.

Septostomy performed at the bedside and in the catheterisation laboratory had similar outcomes and efficacy. Further, bedside septostomy has the advantage of no radiation exposure, and obviating risks with patient transfer from the ICU to the catheterisation laboratory.

Catheter interventions for residual lesions in the early postoperative period after CHD operations are still not established as a reliable treatment option.

We retrospectively reviewed our institutional experience of cardiac catheterisations and catheter interventions performed in the early postoperative period. We classified our patients into two groups. The “hyper” acute phase group – operation to cardiac catheterisation of ⩽7 days – and acute phase group – operation to cardiac catheterisation from 7 to 30 days.

Of the 47 patients, catheter interventions were performed in 38 patients (81%). The success rate of the intervention was 96% in the acute phase group and 90% in the “hyper” acute phase group. The overall success rate was 95%. There were two self-limited complications in the acute phase group, but not in the “hyper” acute phase group. There were four cases of catheter interventions performed for a newly reconstructed aortic arch, and those procedures were also safe and effective.

Cardiac catheterisations and catheter interventions were safe and effective not only in the early postoperative period but also in the very early postoperative period. Catheter interventions for the left-sided heart in the early postoperative period were also safe and effective.

The objectives of this study were to describe the approach to stenting arterial shunts in adult congenital patients with single-ventricle physiology and to assess the medium-term clinical and haemodynamic outcomes following stent insertion.

Adult patients with single-ventricle physiology and pulmonary blood flow dependent on a surgically placed arterial shunt who did not progress to venous palliation are extremely challenging to manage. Progressive cyanosis secondary to narrowing of the shunt has a marked impact on exercise tolerance and results in intolerable well-being for these patients. Stenting arterial shunts in adult patients is one method that can help improve pulmonary blood flow. There is very limited information in the literature about this patient.

This is a retrospective study for arterial shunts stenting conducted between 2008 and 2016. The peripheral oxygen saturations, the NYHA status, the haemoglobin, and the degree of atrio-ventricular valve regurgitation as assessed on transthoracic echo were compared before and 6–12 months after procedures.

There was a short-term improvement in oxygen saturations; the pre-procedure mean was 75.8 (SD 2.55)% (range 70–85%) and post-procedure mean was 83 (SD 2.52)% (range 78–87%), with a p value of 0.04. Haemoglobin level decreased from a pre-procedure mean of 22.06–20.28 g/L 6 months post procedure (range 18.1–24.4 to 13–23.3 g/L), with a p value of 0.44. NYHA class decreased from a mean of 3.2–2.2 post procedure. Left atrial volume for four of the cases did not change (22.6–76.6 ml [mean 48.4 ml] to 29.6–72.9 ml [mean 52 ml], p value: 0.83).

Stenting stenotic arterial shunts is a useful method to gain a medium-term improvement on the oxygen saturation and clinical symptoms, and may act as a useful intermediate step for further management plans.

The aim of this study was to assess the impact of age at bidirectional cavopulmonary anastomosis on haemodynamics after total cavopulmonary connection.

We conducted a retrospective analysis of 100 consecutive patients who underwent total cavopulmonary connection from 2010 to 2014. All patients had previously undergone bidirectional cavopulmonary anastomosis. These patients were classified into two groups according to age at bidirectional cavopulmonary anastomosis: younger group, <6 months (n=33), and older group, >6 months (n=67).

The proportion of hypoplastic left heart syndrome was higher in the younger group (48 versus 4%). After total cavopulmonary connection, the chest tube period was longer in the younger group (10.1±6.6 versus 6.7±4.5 days; p=0.009). Catheterisation 6 months after total cavopulmonary connection revealed that pulmonary artery pressure was higher (11.5±1.9 versus 10.4±2.1 mmHg; p=0.017) and Nakata index was lower (219±79 versus 256±70 mm2/m2; p=0.024) in the younger group. In patients with a non-hypoplastic left heart syndrome, there was no difference in post-operative haemodynamics between two groups, but the total amount of chest drainage after total cavopulmonary connection was larger in the younger group (109±95 versus 55±40 ml/kg; p=0.044).

Early bidirectional cavopulmonary anastomosis did not affect the outcome of total cavopulmonary connection. Longer chest tube period, smaller pulmonary artery, and higher pulmonary artery pressure after total cavopulmonary connection were recognised in early bidirectional cavopulmonary anastomosis patients, especially in hypoplastic left heart syndrome.

We aimed to investigate the incidence and causes of readmission of infants with single-ventricle and shunt-dependent biventricular CHD following routine, outpatient cardiac catheterisation.

Cardiac catheterisation is commonly performed in patients with single-ventricle and shunt-dependent biventricular CHD for haemodynamic assessment and surgical planning. Best practices for post-procedural care in this population are unknown, and substantial variation exists between centres. Outpatient catheterisation reduces parental anxiety and decreases cost. Our institutional strategy is to discharge patients following a 4- to 6-hour post-procedure observation period.

Retrospective cohort study using the Society of Thoracic Surgeons Database identified patients <1 year of age with pre-stage II single-ventricle CHD or shunt-dependent biventricular CHD who underwent cardiac catheterisation between 2007 and 2015. Readmission was defined as admission to the hospital within 48 hours after discharge after catheterisation.

A total of 92 patients were included in the analysis. Among them, 62 patients (67%) were discharged after a 4- to 6-hour observation period with only 3% readmission, 18 patients (20%) stayed for a 23-hour observation period, and 12 patients (13%) were admitted for >23 hours. There were no differences in baseline characteristics between discharged and admitted patients. Patients who underwent intervention were more likely to be admitted. Patients with hypoplastic left heart syndrome did not have major adverse events or readmissions. No intra- or peri-procedural deaths occurred.

Outpatient cardiac catheterisation may be a safe option for infants with single-ventricle and shunt-dependent biventricular CHD, with low readmission rates and minimal morbidity.

Neurodevelopmental disabilities in children with CHD can result from neurologic injury sustained in the cardiac ICU when children are at high risk of acute neurologic injury. Physicians typically order and specify frequency for serial bedside nursing clinical neurologic assessments to evaluate patients’ neurologic status.

We surveyed cardiac ICU physicians to understand how these assessments are performed, and the attitudes of physicians on the utility of these assessments. The survey contained questions regarding assessment elements, assessment frequency, communication of neurologic status changes, and optimisation of assessments.

Surveys were received from 50 institutions, with a response rate of 86%. Routine clinical neurologic assessments were reported to be performed in 94% of institutions and standardised in 56%. Pupillary reflex was the most commonly reported assessment. In all, 77% of institutions used a coma scale, with Glasgow Coma Scale being most common. For patients with acute brain injury, 82% of institutions reported performing assessments hourly, whereas assessment frequency was more variable for low-risk and high-risk patients without overt brain injury. In all, 84% of respondents thought their current practice for assessing and monitoring neurologic status was suboptimal. Only 41% felt that the Glasgow Coma Scale was a valuable tool for assessing neurologic function in the cardiac ICU, and 91% felt that a standardised approach to assessing pre-illness neurologic function would be valuable.

Routine nursing neurologic assessments are conducted in most surveyed paediatric cardiac ICUs, although assessment characteristics vary greatly between institutions. Most clinicians rated current neurologic assessment practices as suboptimal.