The purpose of this study is to assess the mid-term outcomes of aortic root replacement after repair of CHDs.

This is a single-institutional retrospective, cohort study with consecutive patients undergoing aortic root replacement after surgical repair of CHDs between 1999 and 2022. Operative indications included aortic root dilatation with/without aortic insufficiency, sinus of Valsalva rupture, or aortic dissection involving the root.

Forty-four patients (36 male and 8 female) were enrolled. Mean age at the root replacement was 36.6 ± 11.9 years. The most frequent primary diagnosis was congenital aortic stenosis (n = 10) and ventricular septal defect (n = 10). Mean time from the surgical repair to aortic root replacement was 26.6 ± 13.0 years. Operative indications were aortic root enlargement with or without aortic valve aetiology (n = 40), sinus of Valsalva rupture (n = 2), and aortic dissection (n = 2). Forty-two patients underwent valve-replacing aortic root replacement, and two patients underwent valve-sparing, with 40 concomitant procedures. The median follow-up was 3.5 (1.3–7.6) years. There were one early and five late mortalities and five cardiovascular-related reoperations. Actuarial survival at 5–10 years after root replacement was 81.0 ± 6.6%. The cumulative incidence of cardiovascular-related reoperation and aortic root or valve-related reoperation at 5 years after root replacement was 11.9% and 5.6%, respectively.

The early and mid-term outcomes of aortic root replacement for patients with a history of repair of CHDs were favourable in terms of survival and aortic root or valve-related reoperation.

Congenital junctional ectopic tachycardia is a rare arrhythmia that occurs in patients without previous cardiac surgery. In this report, we wanted to present a 6-hour-old newborn with congenital junctional ectopic tachycardia resistant to conventional anti-arrhythmic medications, who was successfully treated with ivabradine and amiadarone combination.

A six-hour-old newborn girl was hospitalised in neonatal ICU because transient tachypnoea of the newborn. She was tachycardic, and supraventricular tachycardia was noticed. There was no answer to the adenosine esmolol treatment; even synchronised direct cardioversion intravenous amiodarone was started. Junctional ectopic tachycardia was diagnosed. We have added propranolol to the treatment and followed patient for 2 days. On the fourth day, junctional ectopic tachycardia rhythm still persisted; therefore, ivabradine treatment was added to the treatment. On the following day, the heart rhythm was slowed to 110/min, and propranolol was stopped; intravenous amiodarone treatment was changed to the oral form. The rhythm turned into sinus; two days after starting ivabradine and oral amiodarone.

Tachyarrhythmia originating in the atrioventricular node and atrioventricular junction including the bundle of His complex are junctional ectopic tachycardia. Congenital junctional ectopic tachycardia is rare, and it is mostly resistant to the conventional treatment.

Ivabradine is a new anti-arrhythmic agent, used extensively to decrease sinus rate in the treatment of cardiac failure. Ivabradine may be an option for the resistant congenital ectopic tachycardia

In 2020, reports revealed cases called multisystem inflammatory syndrome in children and adolescents temporally related to COVID-19 or multisystem inflammatory syndrome in children. A small proportion of patients suffer from persistent left ventricular dysfunction at discharge. The primary aim was to investigate if myocardial impairment persists during follow-up in these patients.

Children fulfilling the criteria for multisystem inflammatory syndrome in children with cardiac involvement hospitalized between December 2020 and February 2022 were included in this retrospective single centre study. Cardiac MRI was performed six months after the onset of symptoms to evaluate possible persistent myocardial damage.

Fifteen patients (80% male) with a median age of 8 years (interquartile range 4.5 - 13.5 years) were included. Upon admission, eight patients (53%) presented with reduced left ventricular function, with a median left ventricular ejection fraction of 54% (interquartile range 49.5%-61.5%) on transthoracic echocardiography. Elevated levels of cardiac-specific markers were found in 14 patients (93%). Cardiac MRI was performed in 12 patients at a median of 190.5 days after the onset of symptoms. Nine patients (75%) had normal left ventricular function, with a median left ventricular ejection fraction of 59.45%, while the remaining patients showed mildly to moderately reduced values. None of the patients showed signs of late gadolinium enhancement, indicating the absence of persistent myocardial scarring.

During a follow-up of 6.2 months, mild to moderate cardiac impairment was revealed in 25% of patients evaluated by cardiac MRI. Although a majority of patients do not show signs of cardiac impairment, close follow-ups should be performed in a proportion of patients.

This study aimed to describe the perception of Malaysian patients with pulmonary hypertension towards palliative care and their receptivity towards palliative care.

This was a cross-sectional, single-centre study conducted via questionnaire. Patients aged 18 years old and above, who were diagnosed with non-curable pulmonary hypertension were recruited and given the assessment tool – perceptions of palliative care instrument electronically. The severity of pulmonary hypertension was measured using WHO class, N-terminal pro B-type natriuretic peptide and the 6-minute walking test distance.

A total of 84 patients [mean age: 35 ±11 years, female: 83.3%, median N-terminal pro B-type natriuretic peptide: 491 pg/ml (interquartile range: 155,1317.8), median 6-minute walking test distance: 420m (interquartile range: 368.5, 480m)] completed the questionnaires. Patients with a higher WHO functional class and negative feelings (r = 0.333, p = 0.004), and cognitive reaction to palliative care: hopeless (r = 0.340, p = 0.003), supported (r = 0.258, p = 0.028), disrupted (r = 0.262, p = 0.025), and perception of burden (r = 0.239, p = 0.041) are more receptive to palliative care. WHO class, N-terminal pro B-type natriuretic peptide, and 6-minute walking test distance were not associated with higher readiness for palliative care. In logistic regression analyses, patients with positive feelings (β = 2.240, p = < 0.05), and practical needs (β = 1.346, p = < 0.05), were more receptive to palliative care.

Disease severity did not directly influence patients’ readiness for palliative care. Patients with a positive outlook were more receptive to palliative care.

The NKX2.5 gene is an important cardiac developmental transcription factor, and variants in this gene are most commonly associated with CHD. However, there is an increased need to recognise associations with conduction disease and potentially dangerous ventricular arrhythmias. There is an increased risk of arrhythmia and sudden cardiac death in patients with NKX2.5 variants, an association with relatively less attention in the literature.

We created a family pedigree and reconstructed familial relationships involving numerous relatives with CHD, conduction disease, and ventricular non-compaction following the sudden death of one family member. Two informative but distantly related family members had genetic testing to determine the cause of arrhythmias via arrhythmia/cardiomyopathy gene testing, and we identified obligate genetic-positive relatives based on family relationships and Mendelian inheritance pattern.

We identified a novel pathogenic variant in the NKX2.5 gene (c.437C > A; p. Ser146*), and segregation analysis allowed us to link family cardiac phenotypes including CHD, conduction disease, left ventricular non-compaction, and ventricular arrhythmias/sudden cardiac death.

We report a novel NKX2.5 gene variant linking a spectrum of familial heart disease, and we also encourage recognition of the association between NKX2.5 gene and potentially dangerous ventricular arrhythmias, which will inform clinical risk stratification, screening, and management.

This is the first study to report on the impact of race on differences in the prevalence of echocardiographic left ventricular hypertrophy and left ventricular adaptation at the time of diagnosis of essential hypertension in children.

This cross-sectional, single-centre study included patients aged 3–18 years who had newly diagnosed essential hypertension. Echocardiography was used to assess left ventricular mass index and left ventricular relative wall thickness. An left ventricular mass index > the 95th percentile for age and gender, and an left ventricular relative wall thickness > 0.42, were used to diagnose left ventricular hypertrophy and concentric adaptation. Various echocardiographic parameters were compared between African Americans and Caucasians.

The study included 422 patients (289 African Americans and 133 Caucasians) diagnosed with essential hypertension at a median age of 14.6 (interquartile range; 12.1–16.3) years. Eighty-eight patients (20.9%) had left ventricular hypertrophy. There was no statistically significant difference in the prevalence of left ventricular hypertrophy between African Americans and Caucasians (22.5% versus 17.3%, p=0.22). The median left ventricular relative wall thickness was 0.35 (0.29–0.43), and 114 patients (27.0%) had an left ventricular relative wall thickness > 0.42. The presence of an left ventricular relative wall thickness > 0.42 was significantly higher among African Americans compared to Caucasians (30.1% versus 20.3%, p = 0.04). The African American race was a strong predictor for an left ventricular relative wall thickness > 0.42 (odds ratio 1.7, p = 0.04), but not for left ventricular mass index > the 95th percentile (p = 0.22). Overweight/obesity was a strong predictor for an left ventricular mass index > the 95th percentile.

There was no difference in the prevalence of left ventricular hypertrophy in children with essential hypertension of different races. Obesity, rather than being African American, is associated with left ventricular hypertrophy.

This meta-analysis aimed to consolidate existing data from randomised controlled trials on hypoplastic left heart syndrome.

Hypoplastic left heart syndrome specific randomised controlled trials published between January 2005 and September 2021 in MEDLINE, EMBASE, and Cochrane databases were included. Regardless of clinical outcomes, we included all randomised controlled trials about hypoplastic left heart syndrome and categorised them according to their results. Two reviewers independently assessed for eligibility, relevance, and data extraction. The primary outcome was mortality after Norwood surgery. Study quality and heterogeneity were assessed. A random-effects model was used for analysis.

Of the 33 included randomised controlled trials, 21 compared right ventricle-to-pulmonary artery shunt and modified Blalock–Taussig-Thomas shunt during the Norwood procedure, and 12 regarded medication, surgical strategy, cardiopulmonary bypass tactics, and ICU management. Survival rates up to 1 year were superior in the right ventricle-to-pulmonary artery shunt group; this difference began to disappear at 3 years and remained unchanged until 6 years. The right ventricle-to-pulmonary artery shunt group had a significantly higher reintervention rate from the interstage to the 6-year follow-up period. Right ventricular function was better in the modified Blalock–Taussig-Thomas shunt group 1–3 years after the Norwood procedure, but its superiority diminished in the 6-year follow-up. Randomised controlled trials regarding medical treatment, surgical strategy during cardiopulmonary bypass, and ICU management yielded insignificant results.

Although right ventricle-to-pulmonary artery shunt appeared to be superior in the early period, the two shunts applied during the Norwood procedure demonstrated comparable long-term prognosis despite high reintervention rates in right ventricle-to-pulmonary artery shunt due to pulmonary artery stenosis. For medical/perioperative management of hypoplastic left heart syndrome, further randomised controlled trials are needed to deliver specific evidence-based recommendations.