Patients with a significant left-to-right shunt at ventricular level may become inoperable at an early age due to irreversible pulmonary vascular disease. On the other hand, even suprasystemic pulmonary hypertension due to mitral stenosis remains treatable. We report a 24-year-old patient with large ventricular septal defect, severe mitral stenosis and cyanosis who improved after surgical correction of both the lesions. This emphasises the importance of additional post-capillary pulmonary hypertension in Eisenmenger syndrome.

Patients with patent ductus arteriosus and significant left-to-right shunt develop Eisenmenger syndrome at an early age and are not operable after development of irreversible pulmonary artery hypertension. Patients with mitral stenosis, however, are treatable even with suprasystemic pulmonary artery pressures. A combination of these two lesions is rare. We document a patient with differential cyanosis who improved after corrective surgery of both the lesions. The importance of post-capillary pulmonary artery hypertension in shunt lesions needs to be better appreciated.

Borderline left ventricle refers to a spectrum of left ventricular underdevelopment, typically associated with other cardiac anomalies. The left ventricle may be mildly hypoplastic, as is sometimes seen accompanying aortic coarctation, or it can be severely hypoplastic, as is seen in hypoplastic left heart syndrome. For patients with a borderline left ventricle that is at either extreme, the treatment decision is relatively straightforward. Those with the most severe form of left ventricle hypoplasia will require single ventricle palliation or cardiac transplantation, whereas those with the mildest form may not need any intervention. It is the management strategy of children that fall within the grey zone of the spectrum, which continues to be controversial and remains variable within and among different institutions. Cardiac diseases with associated left ventricle hypoplasia include critical aortic stenosis, mitral stenosis, coarctation of the aorta, arch hypoplasia, cor triatriatum, unbalanced common atrioventricular canal, Shone’s complex, total anomalous pulmonary venous return, and complex conotruncal abnormalities. In this review, we will discuss the assessment and management of infants with borderline left ventricle with critical aortic stenosis or arch obstruction and associated mitral anomalies.

This manuscript presents a technically straightforward technique to allow for mitral valve repair in the patient with rheumatic mitral stenosis. This non-resection technique allows for the correction of both mitral stenosis and regurgitation without requiring complex subvalvar procedures and eliminates the concerns for postoperative systolic anterior motion. The authors feel this three-part technique of bi-commissural release, anterior leaflet augmentation, and oversized annuloplasty may allow for a more reproducible approach to repair of the rheumatic mitral valve.

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.

Objectives: Insulin-like growth factor-1 may serve some regulatory function in the immune system. Rheumatic mitral stenosis is related to autoimmune heart valve damage after streptococcal infection. The aim of this study was to assess the level of insulin-like growth factor-1 and its correlation with the Wilkins score in patients with rheumatic mitral stenosis. Methods: A total of 65 patients with rheumatic mitral stenosis and 62 age- and sex-matched control subjects were enrolled in this study. All subjects underwent transthoracic echocardiography. The mitral valve area and Wilkins score were evaluated for all patients. Biochemical parameters and serum insulin-like growth factor-1 levels were measured. Results: Demographic data were similar in the rheumatic mitral stenosis and control groups. The mean mitral valve area was 1.6±0.4 cm2 in the rheumatic mitral stenosis group. The level of insulin-like growth factor-1 was significantly higher in the rheumatic mitral stenosis group than in the control group (104 (55.6–267) versus 79.1 (23.0–244.0) ng/ml; p=0.039). There was a significant moderate positive correlation between insulin-like growth factor-1 and thickening of leaflets score of Wilkins (r=0.541, p<0.001). Conclusions: The present study demonstrated that serum insulin-like growth factor-1 levels were significantly higher in the rheumatic mitral stenosis group compared with control subjects and that insulin-like growth factor-1 level was also correlated with the Wilkins score. It can be suggested that there may be a link between insulin-like growth factor-1 level and immune pathogenesis of rheumatic mitral stenosis.

Three-dimensional echocardiography is an increasingly used method to accurately depict congenital heart disease in children. We report the case of a 2-month-old girl with supramitral ring. Three-dimensional echocardiography allowed perfect visualisation of the fibrous shelf before surgery.

Rheumatic heart disease is the only residual morbidity, and the sole cause of mortality, from rheumatic fever. Echocardiography is ideally suited to confirm and follow the course of rheumatic heart disease. Additionally, both minimal valvar pathology in children, and extensive valvar pathology in adults, may not cause a murmur and can be detected only by echocardiography. Whenever possible, echocardiography should be routinely employed for management of patients with rheumatic fever or suspected rheumatic fever.

Anomalous origin of a coronary artery is occasionally associated with other congenital heart malformations. Presence of the anomalous artery can then complicate surgical repair. We report an unusually complex arrangement in a patient who died 16 hours following complete surgical repair despite appropriate management.

Cervical aortic arch is a rare congenital anomaly. We present a case of left-sided cervical aortic arch, found in a patient with mitral regurgitation, mitral stenosis, and a regurgitant bicuspid aortic valve. There was atypical obstruction proximally within the arch. The obstructive segment was resected, and corrected by performing an end-to-side anostomosis. Mitral valvoplasty was performed in the same surgical procedure. To the best of our knowledge, a cervical arch has not previously been described with such atypical obstruction, and in association with multiple lesions involving the left heart.