To better understand parents’ accounts of their prenatal and postnatal experience after prenatal diagnosis of CHD – particularly emotional processing and coping mechanisms – to identify strategies to improve support.

This single-centre, longitudinal qualitative study included pregnant mothers and their support persons seen in Fetal Cardiology Clinic at Vanderbilt Children’s Hospital from May through August 2019 for probable complex CHD. Twenty-seven individuals from 17 families participated in 62 phone interviews during pregnancy and postpartum: 27 conducted after the initial prenatal cardiology consultation, 15 after a follow-up prenatal visit, and 20 after birth. Applied thematic analysis approach was used to code and analyse transcribed interviews. Coding and codebook revisions occurred iteratively; intercoder reliability was >80%.

Patients included mothers (16 [59%]), fathers (8 [30%]), and other support persons (3 [11%]). Initial fetal diagnoses included a range of moderate to severe CHD. Prenatally, parents sought to maintain hope while understanding the diagnosis; planning for the future rather than focusing on day-to-day was more common if prognoses were better. Postnatally, with confirmation of prenatal diagnoses, parents’ sense of control expanded, and they desired more active engagement in clinical decision making.

To enhance effective communication and support, understanding how parents conceptualise hope in relation to diagnosis and how that may evolve over time is critical. Expectant parents whose child has a significant risk of mortality may demonstrate hope by focusing on positivity. As prognostic uncertainty diminishes postpartum, the parental role on the team may shift, requiring clinicians to provide different support.

Hypertension following primary coarctation repair affects up to a third of subjects. A number of studies suggest that future hypertension risk is reduced if primary repair is performed at a younger age.

The objective of this study was to evaluate the risk of future medical treatment for hypertension depending on age of primary coarctation repair.

This study was carried out at a tertiary paediatric cardiology referral centre. Retrospective database evaluation of children aged <16 years undergoing primary surgical coarctation repair between October, 2005 and October, 2014 was carried out. Patients with complex heart diseases were excluded. The following age groups were considered: neonate (⩽28 days), infant (>28 days and ⩽12 months), and children (>12 months). Main outcome measure is the need for long-term anti-hypertensive medication. The risk for re-coarctation was also evaluated.

A total of 87 patients were analysed: 60 neonates, 17 infants, 10 children. Among them, 6.7% neonates, 29.4% infants, and 40% children required long-term anti-hypertensive medications. Group differences were statistically significant (p=0.004). After adjustment for type of repair, the risk of long-term anti-hypertensive therapy was 4.5 (95% confidence interval 1.2–16.9, p=0.025) and 10.5 times (95% confidence interval 2.6–42.3, p=0.001) higher if primary repair was carried out in infancy and childhood, respectively, compared with neonates. Among all, 13 patients developed re-coarctation: 21.7% in the neonatal group, 5.9% in the infant group, and 20% in the child group. We could not demonstrate a significant difference between these proportions or calculate a reliable risk for developing re-coarctation.

Risk of medical treatment for hypertension was lowest when primary repair was carried out during the neonatal period, rising 10-fold if first operated on as a child. Knowing the likelihood of hypertension development depending on age of primary repair is useful for long-term surveillance and counselling.

Cardiovascular morbidity is high in patients with coarctation of aorta even after successful repair. This study aimed to assess the frequency of late hypertension and the relationship between ambulatory hypertension and cardiovascular target organ damage in children and adolescents after early and successful repair of coarctation of the aorta.

Twenty-five children and adolescents (mean age 13.5 ± 3.43 years) with repaired coarctation of the aorta (median age at repair 4 months, arm–leg gradient <20 mmHg) and 16 healthy controls were included. Office and ambulatory blood pressure, pulse wave velocity, and left ventricular mass index were assessed.

Both day- and night-time systolic blood pressure standard deviation score and left ventricular mass index were significantly higher in patients compared to controls (p ≤ 0.001 for all), whereas pulse wave velocity did not differ. The prevalence of masked hypertension, isolated nocturnal hypertension, and left ventricular hypertrophy were 40, 28, and 24%, respectively. Left ventricular mass index was higher in patients with sustained hypertension, masked hypertension, and normotension compared to controls (p < 0.05). In multivariate analysis, higher night-time systolic blood pressure standard deviation score was the only independent predictor of left ventricular mass index.

The present study reveals a high prevalence of masked hypertension, isolated nocturnal hypertension, and left ventricular hypertrophy in children and adolescents with coarctation of the aorta even after early and successful repair. Ambulatory blood pressure monitoring should be considered to diagnose hypertension. All coarctation of aorta patients should be followed up lifelong and encouraged to establish a healthy lifestyle starting from childhood.

To assess the efficacy and safety of captopril, simvastatin, and L-carnitine as cardioprotective drugs in children with type 1 diabetes mellitus on different echocardiographic parameters, electrocardiographic parameter, lipid profile, and carotid intima–media thickness.

This randomised controlled trial was conducted on 100 children with type 1 diabetes mellitus for more than 3 years during the period from September 2018 to June 2020. Fifty healthy children of matched age and sex served as a control group. The patients were randomly assigned into four groups (25 children each): no-treatment group who received no cardioprotective drug, simvastatin group who received simvastatin (10–20 mg/day), captopril group who received captopril (0.2 mg/kg/day), and L-carnitine group who received L-carnitine (50 mg/kg/day) for 4 months. Lipid profile, serum troponin I, carotid intima–media thickness, and echocardiographic examinations were performed on all included children before and after the treatment.

Total cholesterol and low-density lipoprotein were significantly decreased in children who received simvastatin or L-carnitine. Triglycerides significantly decreased only in children who received simvastatin. High-density lipoprotein significantly increased in simvastatin and L-carnitine groups only. Serum troponin I decreased significantly in all the three treatment groups. Carotid intima–media thickness showed no significant change in all treatment groups. Echocardiographic parameters significantly improved in simvastatin, L-carnitine, and captopril groups.

Captopril, simvastatin, and L-carnitine have a significant beneficial effect on cardiac functions in children with type 1 diabetes mellitus. However, only simvastatin and L-carnitine have a beneficial effect on the lipid profile. The drugs were safe and well tolerated.

Clinical trial registration: The clinical trial was registered at www.clinicaltrial.gov (NCT03660293).

Indexing left ventricular mass to body surface area or height2.7 leads to inaccuracies in diagnosing left ventricular hypertrophy in obese children. Lean body mass predictive equations provide the opportunity to determine the utility of lean body mass in indexing left ventricular mass. Our objectives were to compare the diagnostic accuracy of predicted lean body mass, body surface area, and height in detecting abnormal left ventricle mass in obese children.

Obese non-hypertensive patients aged 4–21 years were recruited prospectively. Dual-energy X-ray absorptiometry was used to measure lean body mass. Height, weight, sex, race, and body mass index z-score were used to calculate predicted lean body mass.

We enrolled 328 patients. Average age was 12.6 ± 3.8 years. Measured lean body mass had the strongest relationship with left ventricular mass (R2 = 0.84, p < 0.01) compared to predicted lean body mass (R2 = 0.82, p < 0.01), body surface area (R2 = 0.80, p < 0.01), and height2.7 (R2 = 0.65, p < 0.01). Of the clinically derived variables, predicted lean body mass was the only measure to have an independent association with left ventricular mass (β = 0.90, p < 0.01). Predicted lean body mass was the most accurate scaling variable in detecting left ventricular hypertrophy (positive predictive value = 88%, negative predictive value = 99%).

Lean body mass is the strongest predictor of left ventricular mass in obese children. Predicted lean body mass is the most accurate anthropometric scaling variable for left ventricular mass in left ventricular hypertrophy detection. Predicted lean body mass should be considered for clinical use as the body size correcting variable for left ventricular mass in obese children.

Right ventricular dysfunction in patients with tetralogy of Fallot and significant pulmonary regurgitation may lead to systolic dysfunction of the left ventricle due to altered ventricular interaction. We were interested in determining whether chronic pulmonary regurgitation affects the preload of the left ventricle. In addition, we wanted to study whether severe chronic pulmonary regurgitation would alter the preload of the left ventricle when compared with patients having preserved pulmonary valve annulus.

The study group comprised 38 patients with tetralogy of Fallot who underwent surgical repair between 1990 and 2003. Transannular patching was required in 21 patients to reconstruct the right ventricular outflow tract. Altogether, 48 age- and gender-matched healthy volunteers were recruited. Cardiac MRI was performed on all study patients to assess the atrial and ventricular volumes and function.

Severe pulmonary regurgitation (>30 ml/m2) was present in 13 patients, of whom 11 had a transannular patch, but only two had a preserved pulmonary valve annulus. The ventricular preload volumes from both atria were significantly reduced in patients with severe pulmonary regurgitation, and left ventricular stroke volumes (44.1±4.7 versus 58.9±10.7 ml/m2; p<0.0001) were smaller compared with that in patients with pulmonary regurgitation <30 ml/m2 or in controls.

In patients with tetralogy of Fallot, severe pulmonary regurgitation has a significant effect on volume flow through the left atrium. Reduction in left ventricular preload volume may be an additional factor contributing to left ventricular dysfunction.

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.

A 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.

In 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.

The 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.

In Nigeria, access to open heart surgery (OHS) is adversely affected by insufficient blood and blood products, including the challenges because of the lack of patient-focused blood management strategies owing to the absent requisite point-of-care tests in the operating theatre (OR)/ICU. In addition, the limited availability of altruistic blood donors including the detection of transfusion transmitted infections more commonly among non-altruistic blood donors is another burden affecting the management of excessive bleeding during and after open heart surgery in our country.

The objective of this study was to review our local experience in the use of blood and blood products during open heart surgery and compare the same with the literature.

In a period of 3 years (March, 2013–February, 2016), we performed a retrospective review of those who had open heart surgery in our institution. The data were obtained from our hospital health information technology department. The data comprised demography, types of operative procedures and units of blood and blood products transfused per procedure, including the details regarding the usage of the cell saver, as well as those who had severe bleeding requiring excessive blood transfusion.

During the study period, 102 patients had open heart surgery, an average of 34 cases in a year. Among them, there were 75 (73.53%) males and 37 (36.27%) females, giving a ratio of 2:1. The ages of the patients were from 0.6 (7/12) to 74 years. Mitral valve procedure was the most common (n=22, 21.6%) surgery type. Transfusion requirements averaged 1.9 units of fresh frozen plasma, 0.36 units of platelet concentrate, and 1.68 units of packed cells per procedure. The least common surgical procedure was common atrium repair (n=1, 0.01%).

Open heart procedure is a very complex procedure requiring cardiopulmonary bypass with associated severe perioperative bleeding. The attendant blood loss and haemostatic challenges are combated by intricate and selective transfusions of allogeneic blood and or blood products.

The aim of the present study was to describe the clinical course, laboratory tests, and the cardiac involvement in rheumatic carditis patients in functional class III and IV, submitted to pulse therapy combined with oral prednisone.

A total of 120 patients with severe carditis due to acute rheumatic fever were treatment with three cycles of pulse therapy combined with oral corticosteroids. The patients were followed up from the hospital admission until the end of the treatment and returned after 30, 60, and 90 days to control. The patients were evaluated by clinical, laboratory, and transthoracic echocardiogram.

In total, 23 (19.2%) patients at first attack of rheumatic fever and 97 (80.8%) with recurrent carditis were evaluated. Cardiac surgery was performed in 8 (6.6%) patients. The patients showed improved laboratory and radiological parameters (p<0.001) and were discharged, 74 (61.7%) in functional class I and 46 (38.3%) in functional class II. Hospitalisation time ranged from 21 to 176 days, with a mean of 69.1 days. Reduction of left atrium and ventricle diameters was observed, measured by means of transthoracic echocardiography, at hospital admission and discharge (p<0.001). None of the patients experienced rebound.

The pulse therapy was effective in controlling severe rheumatic carditis and the oral corticosteroid prevented rebound episodes. Prolonged hospital stay was required for the clinical stabilisation of patients and to avoid the interruption of medication.

The objectives of this study were to evaluate the plasma levels of asymmetric dimethyl-L-arginine in children with pulmonary hypertension due to CHD before and after treatment with sildenafil and to evaluate its diagnostic and prognostic value as a biomarker in such children.

A total of 60 children with CHD and 30 healthy control children matched for age and sex were recruited. Children with CHD were divided into two equal groups: the normal pulmonary pressure group (n=30) and the pulmonary hypertension group (n=30). Children with pulmonary hypertension were treated with sildenafil and were followed up for 6 months. Clinical data, haemodynamic parameters, echocardiographic examination, and asymmetric dimethyl-L-arginine levels were evaluated before and after treatment.

Asymmetric dimethyl-L-arginine levels were significantly higher in patients with pulmonary hypertension than in those with CHD-only or the control group, and this increase was positively correlated with increased severity of pulmonary hypertension. Asymmetric dimethyl-L-arginine levels, mean pulmonary artery pressure, and pulmonary vascular resistance were significantly decreased after treatment with sildenafil. Moreover, asymmetric dimethyl-L-arginine level was significantly lower in patients who responded to sildenafil treatment compared with those who did not. At a cut-off point of more than 0.85 nmol/ml, asymmetric dimethyl-L-arginine has a sensitivity of 83% and a specificity of 80% to diagnose pulmonary hypertension-CHD. Asymmetric dimethyl-L-arginine has a sensitivity of 100% and a specificity of 94% to predict poor prognosis in pulmonary hypertension-CHD children at a cut-off point of 1.3 nmol/ml.

Asymmetric dimethyl-L-arginine level has a good diagnostic and prognostic value as a biomarker in children with pulmonary hypertension-CHD and can be used for following up patients with pulmonary hypertension and predicting response to treatment.