A six-month-old boy with Down syndrome, complete atrioventricular septal defect with intact atrial septum, and moderate left-sided atrioventricular valve regurgitation underwent surgery. The presence of a common atrioventricular junction could not be diagnosed preoperatively. Postoperative left-sided atrioventricular valve regurgitation remained mild by complete cleft closure, direct closure of incised atrial septum, and patch augmentation of the underside of septum primum.

Anomalous left coronary artery from the pulmonary artery is a rare CHD. It is the most common type of anomalous coronary origin. It may cause myocardial ischaemia or infarction, mitral regurgitation, congestive heart failure, and early death in infancy if left untreated. Surgery is the only treatment for anomalous left coronary artery from the pulmonary artery. In recent years, with advancements in surgical techniques and the widespread utilisation of extracorporeal cardiac assist devices such as extracorporeal membrane oxygenation, the treatment outcomes for anomalous left coronary artery from the pulmonary artery have demonstrated significant improvements. However, the surgical indications and methods of anomalous left coronary artery from the pulmonary artery, especially the surgical methods of anomalous left coronary artery from the pulmonary artery with intramural coronary artery, and whether to treat mitral regurgitation at the same time are still controversial. The long-term complications and prognosis remain discouraging simultaneously, with significant variations in outcomes across different centres. The present review specifically addresses these aforementioned concerns. Based on the literature published at home and abroad, we found that no matter what type of anomalous left coronary artery from the pulmonary artery patients, even asymptomatic patients, regardless of the collateral circulation between the left and right coronary arteries, should immediately undergo surgical treatment to promote the recovery of left ventricular function. Based on different coronary artery anatomical morphology and preoperative cardiac function, the long-term follow-up results of individualised surgical treatment of anomalous left coronary artery from the pulmonary artery children show good prognosis, and most children have significant improvement in cardiac function. Patients with moderate to severe mitral regurgitation should undergo mitral valve operation at the same time as anomalous left coronary artery from the pulmonary artery repair. Mitral valvuloplasty can quickly improve mitral regurgitation and promote the early recovery of cardiac function after operation, and does not increase the risk of operation. Mechanical circulatory support is a safe and effective means of early postoperative transition for children with severe anomalous left coronary artery from the pulmonary artery. Anomalous left coronary artery from the pulmonary artery with intramural coronary artery is a rare anomaly. According to different anatomical types, different surgical methods can be used for anatomical correction, and satisfactory early and mid-term results can be obtained.

Mitral regurgitation in the neonatal period is relatively rare. It can be secondary to a congenital malformation of the valve apparatus or mitral valve dysfunction and deformation secondary to myocardial dysfunction or volume load of the left ventricle. Less commonly, it can be due to coronary artery abnormalities leading to mitral valve papillary muscle ischaemia and subsequent dysfunction. Such coronary artery abnormalities include anomalous left coronary artery from pulmonary artery, left main coronary artery atresia, or a thromboembolic phenomenon. In this study, we describe a newborn with a dysplastic aortic valve causing obstruction of the os of the left coronary artery leading to progressive mitral insufficiency.

The prevalence of congenital left main coronary artery atresia is very low. We report the characteristics and long-term outcomes of four children with left main coronary artery atresia. Three patients had heart murmurs due to mitral regurgitation at less than 1 year old. Their myocardial ischaemia worsened on exercise with aging. In the fourth patient, hypertrophic cardiomyopathy and Noonan syndrome were suspected at 1 year old. The development of communicating arteries between the conus branch and the left anterior descending artery was detected at 7 years old. The left main coronary artery atresia was confirmed by a selective coronary angiogram at 15 years old. Congenital left main coronary artery atresia could not be diagnosed by two-dimensional echocardiography; however, the left coronary arteries were small. Two patients underwent coronary artery bypass grafting of the left anterior descending artery using the left internal thoracic artery at 3 years and 6 years old, respectively. Two patients had an angioplasty with a cut back at the orifice of the left coronary artery at 2 years old and 17 years old, respectively. Two patients had no cardiac events without medication for more than 30 years after the operation. We must differentiate the diagnosis of left main coronary artery atresia in the small left coronary arteries with mitral regurgitation during the first year. Coronary artery revascularisation and mitral annuloplasty are needed. The long-term outcome of both coronary artery bypass grafting and angioplasty were good. The degree of mitral regurgitation after surgery may affect the prognosis.

We report the first successful Glenn procedure for pulmonary atresia with intact ventricular septum in the setting of D-transposition of the great arteries. This case was complicated by hypoplastic left ventricle and severe mitral regurgitation. Partial closure of the mitral valve at the time of Blalock-Taussig shunting resulted in improvement of heart failure.

Mitral valve perforation is an uncommon aetiology of mitral regurgitation in the paediatric population. We present a case where 3-dimensional echocardiography assisted in the diagnosis of the source of mitral regurgitation and the surgical correlation.

A full-term, female presented on her date of birth with severe pulmonary hypertension (PH) and mitral regurgitation (MR), requiring veno-arterial extracorporeal membrane oxygenation. After the treatment, her PH and MR were resolved with no anatomic abnormality present. We propose a positive feedback loop of PH causing right ventricular dilation and interventricular septal shifts, worsening MR, and elevated left atrial, and potentially pulmonary, pressures.

Severe valvulitis owing to acute Kawasaki disease leading to severe mitral regurgitation is a rare event in infants. Further, there is less information about underlying ruptured mitral chordae tendineae causing severe mitral regurgitation. We encountered ruptured mitral chordae tendineae in three female patients after Kawasaki disease. The age at the onset of Kawasaki disease ranged from 3 to 8 months, and detection of ruptured mitral chordae tendineae was from 24 to 90 days. Two patients had acute heart failure, and one was asymptomatic. One patient underwent mitral annuloplasty, and the others responded to medication. These ruptured mitral chordae tendineae occurred after the remission of the initial acute Kawasaki disease, in the early course and the convalescent of acute Kawasaki disease. Further, the recurrent fever was also detected in them. The ruptured mitral chordae tendineae in an infant within 6 months can be detected by systolic heart murmur around the convalescent stage of acute Kawasaki disease, although the prevalence is very low.

An infant with residual severe mitral regurgitation following mitral commissurotomy developed cardiogenic unilateral pulmonary oedema and subsegmental atelectasis that resolved with mechanical mitral valve replacement.

We aimed to elucidate the relationship between severity of secondary mitral regurgitation and mitral valve geometry in children with dilated cardiomyopathy. The medical records of 16 children with dilated cardiomyopathy (median age, 1.2 years; range, 0.4–12.3 years) were reviewed. Mitral valve geometry was evaluated by measuring coaptation depth using echocardiographic apical four-chamber views at the initial presentation. Patients were dichotomised according to the mitral regurgitation severity: patients with moderate or severe secondary mitral regurgitation (n=6) and those with mild secondary mitral regurgitation (n=10). A total of 58 healthy children were considered as normal controls, and a regression equation to predict coaptation depth by body surface area was derived: coaptation depth [mm]=4.37+1.34×ln (body surface area [m2]) (residual standard error, 0.49; adjusted R2, 0.68; p<0.0001). Compared with patients with mild secondary mitral regurgitation, those with moderate or severe secondary mitral regurgitation had significantly larger coaptation depth z-scores (6.4±2.3 versus 1.9±1.4, p<0.005), larger mitral annulus diameter z-scores (3.6±2.6 versus 0.9±1.8, p<0.05), higher left ventricular sphericity index (0.89±0.07 versus 0.79±0.06, p<0.005), and greater left ventricular fraction shortening (0.15±0.05 versus 0.09±0.05, p<0.05). In conclusion, geometric alteration in the mitral valve and the left ventricle is associated with the severity of secondary mitral regurgitation in paediatric dilated cardiomyopathy, which would provide a theoretical background to surgical intervention for secondary mitral regurgitation in paediatric populations.

Valvar cardiac disease accounts for a large percentage of heart lesions in the paediatric population. There are a variety of congenital and acquired valvar lesions produced by different mechanisms that produce unique physiologies. With the development of more advanced cardiac interventional procedures and congenital cardiac surgery techniques, most of these lesions can be repaired in a single or multi-stage approach. Given the potential for significant symptomatology before intervention, it is imperative to review the critical care management of paediatric valvar heart disease. The purpose of this article is to provide an overview of the current critical care management of valvar cardiac disease. We approach each cardiac valve individually in order to review the pathophysiology caused by valve dysfunction (obstruction and incompetence) and elaborate on the most frequent diagnoses, specific cardiac physiology, potential for other organ system associations, and perioperative management.

Williams syndrome is caused by a gene deletion of chromosome 7. A majority of the cases are sporadic with typical facial appearance, cardiac anomalies, and mental retardation. We report a rare case of Williams syndrome associated with supravalvular aortic stenosis, subvalvular aortic membrane, mitral regurgitation, aortic coarctation, and patent ductus arteriosus. The patient had undergone a single-stage surgical repair with satisfactory results at 5 months of follow-up.

Percutaneous edge-to-edge mitral valve repair using the MitraClipTM has not been used in children. The patient in this reported case was a 15-year-old male adolescent who presented postnatally with severe aortic valve stenosis and dysplasia of the mitral valve. The boy underwent surgical valvuloplasty at the age of 3 months and an aortoventriculoplasty with three re-operations. At the age of 15 years, he developed severe mitral valve regurgitation. Owing to high surgical risks, a MitraClipTM was implanted with a reduction of mitral regurgitation from grade 4+ to 2+, translating into a rapid clinical improvement.

Anomalous origin of the anterior descending coronary artery from the pulmonary trunk is very rare. We suspected such anomalous origin in a boy aged 2.5 years owing to the echocardiographic demonstration of mitral regurgitation and abnormal diastolic flow in the ventricular septum. The diagnosis was confirmed by angiography. The anomalous artery was successfully reimplanted into the aorta.

A 24-year-old female without previous symptoms or known heart disease consulted a general practitioner with dyspnoea which had slowly worsened over a period of two years. She was treated for asthma, without investigations, until she presented with rapid atrial fibrillation, chest pain and pulmonary oedema necessitating admission to hospital, where she was found to have “mitral” regur-gitation (of the left-sided atrioventricular valve) and congenitally corrected transposition. At operation the grossly regurgitant left-sided morphologically tricuspid valve was replaced by a “top-hat” inverted aortic valve homograft. The procedure resulted in intermittent heart block, needing a pacemaker, and continued myocardial failure which progressed with new right-sided valvar regurgitation. She died a few months later. Post mortem examination confirmed the double discordance, and showed a fistula around the tricuspid valvar prosthesis, left atrial thrombus and changes in the mitral valve. There was also an unsuspected area of old myocardial ischaemia, associated with severe hypoplasia of the circumflex branch of the right-sided coronary artery. This was considered probably to have been of embolic origin.