We report our experience using an originally designed thin walled metal-tipped arterial cannula for neonatal open heart surgery. Its external diameter is 2.1 mm and its internal diameter is 1.7 mm, it has a high internal – external diameter ratio. It was used on 36 patients, who were either neonates or small infants. In 32 of the patients, it was inserted into the ascending aorta for systemic perfusion. In the other 4, it was inserted into the brachiocephalic artery for selective cerebral perfusion during repair of the aortic arch. The relationship between the pressure drop and the bypass flow was obtained for the 7 most recently treated patients. The pressure drop at a bypass flow of 500 ml/min was 150 mmHg. Due to the high internal – external diameter ratio of this cannula, and its excellent flow characteristics, we consider it to be particularly useful in neonatal open heart surgery, while it also allows for successful selective cerebral perfusion during repair of the aortic arch in neonates.

A maternally inherited novel pathogenic non-POU domain-containing octamer-binding gene variant c.767G>T, p.R256I [NM_001145408], manifested in a male infant as dilated cardiomyopathy with severe left ventricular dysfunction and dilation, biventricular non-compaction, tricuspid hypoplasia, and hydrocephaly. To the best of our knowledge, no previous non-POU domain-containing octamer-binding gene variants with biventricular non-compaction have been associated with tricuspid valve hypoplasia. Hence, this case introduces a new pathogenic variant observed in the non-POU domain-containing octamer-binding gene and adds to the range of cardiac phenotypes identified in non-POU domain-containing octamer-binding gene variants.

Introduction: Patients with absent pulmonary valve syndrome often present early with airway compression from aneurysmal pulmonary arteries. This study reviews our experience in managing absent pulmonary valve syndrome in later presenting children, and techniques used for managing airway compression. Methods: This study is a retrospective chart review of all patients who underwent repair of absent pulmonary valve syndrome from 2000 to 2012 at our institution. The primary endpoints were post-operative bronchoscopic and clinical evidence of persistent airway compression and need for reinterventions on the pulmonary arteries. Results: A total of 19 patients were included during the study period. The mean age at repair was 4.1±3.0 years (range 10 months–11 years). In all, seven patients had pre-operative bronchoscopic evidence of airway compression, which was managed by pulmonary artery reduction plasty in four patients and Lecompte manoeuvre in three patients. There were no peri-operative deaths. In patients with pulmonary artery plasty, two had no post-operative airway compression, one patient had improved compression, and one patient had unchanged compression. In patients managed with a Lecompte manoeuvre, two patients had no or trivial airway compression and one had improved compression. There were six late reinterventions or reoperations on the pulmonary arteries – two out of four in the pulmonary artery plasty group and one out of three in the Lecompte group. Conclusions: Most late-presenting patients with absent pulmonary valve syndrome do not have airway compression. Either pulmonary artery reduction plasty or the Lecompte manoeuvre can relieve proximal airway compression, without a significantly different risk of pulmonary artery reintervention between techniques.

A 4-year-old boy presented with intractable atrial tachycardia and heart failure. Antiarrhythmic drugs, such as digoxin, beta-blockers, and amiodarone were ineffective. Although we attempted multiple radiofrequency catheter ablations, the atrial tachycardia arising from left atrial appendage frequently recurred. Finally, we decided to perform atrial appendectomy using the thoracoscopic approach. Immediately after the appendectomy, the atrial tachycardia was terminated and restored to sinus rhythm. Left ventricular ejection fraction increased from 33 to 60% within 1 week. He had no arrhythmia during the subsequent 9-month follow-up period. Minimally invasive thoracoscopic surgery can be applied even in a small child who has focal atrial tachycardia originating from an atrial appendage.

Increasingly, paediatric cardiologists are called upon to diagnose cardiac malformations prenatally. In the main, the types of malformation seen during fetal life will be similar to those documented postnatally, but the frequency with which they are encountered, as well as the views that can be used for diagnosis, will be different. This review aims to describe the anatomic spectrum of malformations seen in 917 fetal hearts examined consecutively following prenatal diagnosis. The distribution of anomalies is illustrated in terms of a simple sweep through the fetal thorax passing from the four-chamber plane to the outflow tracts, and then to more cranial views of the mediastinum. Two-thirds of the anomalies described would have been detectable in the four-chamber plane. Some, such as tricuspid valvar abnormalities, will alter the normal appearances of the four-chambers dramatically. In terms of the overall spectrum, however, such obvious abnormalities only form a minor part of the total number. Others, such as atrioventricular septal defect, will often require closer inspection of intracardiac anatomy, but will make up a large proportion of the entire cohort. Up to one third of the anomalies in the series would have required views more cranial to the four-chamber plane of section. In these, it would have been necessary to examine the nature of the left ventricular outflow tract, the crossing of the two outflows, or else the arterial arches in order to secure detection. In the fetus, these and other planes must be considered by the echocardiographer in order completely to detect and document the entire spectrum of cardiac abnormalities likely to be encountered.

Background: Nicotinamide adenine dinucleotide phosphate oxidase of the vascular cell membrane is an important source of reactive oxygen species. The aim of our study was to evaluate the possible influence of the p22phox C242T gene polymorphism on blood pressure and some markers of oxidative stress in children with tetralogy of Fallot. Methods: After surgical repair in early life, we recruited 38 children, aged 11.7 plus or minus 3.2 years, including 185 healthy individuals as controls for the purposes of establishing frequencies of alleles and genotypes. From this latter group, we matched a sub-sample of 53 healthy caucasian children, aged 11.0 plus or minus 1.0 years, in order to compare enzymic activities. Results: The children with tetralogy of Fallot showed significantly lower values of low-molecular-weight protein tyrosine phosphatase, particularly in carriers of CC genotype for the p22phox gene, with values of 145.2 plus or minus 77.4 μmol/g Hb/h, compared to controls, at 344.4 plus or minus 100.4 μmol/g Hb/h (p less than 0.001). Methemoglobin reductase activity in the patients with tetralogy was also lower in those with the CC genotype, at 9.8 plus or minus 3.2 μmol/g Hb−1 min−1 compared to 24.2 plus or minus 11.8 μmol/g Hb−1 min−1 as measured in the controls (p less than 0.01). Lower systolic (p less than 0.05) and diastolic (p less than 0.01) blood pressures were also observed in the patients with tetralogy of Fallot. Conclusions: Patients with tetralogy of Fallot having the CC genotype may be at a higher state of oxidative stress than T allele carriers, a finding which could have prognostic implications. Long term follow-up of these patients, however, may be necessary in order to draw definite conclusions.

We describe an unusual case of congenitally corrected transposition in which left-sided obstructions included a supravalvar tricuspid ring, a parachute deformity of the valve itself, and coarctation of the aorta. Surgical correction was performed in 3 stages. Initially we repaired the coarctation and banded the pulmonary trunk. Then we removed the band and, finally, attempted unsuccessfully to replace the tricuspid valve. When coarctation of the aorta occurs in congenitally corrected transposition, it is always wise to search carefully for abnormalities of the tricuspid valve.

We present the case of a 6-year-old patient with double-orifice mitral valve and severe regurgitation, which was successfully repaired. We demonstrate that mitral valve repair may be attempted even in complex anatomy such as the double-orifice mitral valve, where it can offer excellent results.

Aims: The purpose of our study was to ascertain the outcome of the construction of a Blalock–Taussig shunt in patients aged 12 years and over. Patients and methods: We identified 21 patients in whom a Blalock–Taussig shunt had been constructed subsequent to the age of 12 years. Of the patients, 9 were female, and their median age was 18.5 years, with a range from 12 to 46 years. All had usual atrial arrangement, and the atrioventricular connections were concordant in 11, with univentricular atrioventricular connection in 10. Pulmonary atresia was present in 8 (38 per cent), and pulmonary stenosis, either valvar or subvalvar, in 13. An interposition graft had been placed between the subclavian and pulmonary arteries in 16 patients, and an end-to-side anastomosis between the arteries in 5. Results: One patient had died in hospital, while 4 patients had died during the period of follow-up after initial construction of the shunt. Long-term follow-up was available in 86 per cent of patients. In the 3 patients lost to follow-up, the shunt had been known to be functioning at periods of 4, 8, and 10 years, respectively. Actuarial freedom from death after a period of 17 years was 76 per cent. In 8 patients, a period of 10 years had elapsed with the shunt patent, and a further 10 had a patent shunt after 5 years follow-up. Symptomatic improvement was reported in 16 (76 per cent) patients, although adverse cardiac events had occurred during follow-up in 17, including congestive heart failure in 3, atrial fibrillation in 3, and endocarditis in 2. In 2 patients, it had been possible to proceed to biventricular repair, one with tetralogy of Fallot, and the other having a Rastelli procedure. Further in 3 patients, it had been possible to construct the Fontan circulation, or one of its variants. One patient has undergone cardiac transplantation, while 2 are awaiting transplantation. Conclusions: Symptomatic improvement can be achieved by construction of a Blalock–Taussig shunt in older subjects, and the risks of surgery are low. Later repair may be feasible in some patients, but adverse cardiac events may follow the increased volume load on the systemic ventricle.

Cardiac rhabdomyoma is a rare tumor. Because of its rate of regression, mainly in the first year of life, conservative therapy is generally suggested when diagnosis occurs in infancy. We report surgical ablation of a rhabdomyoma in a 2-month-old boy in whom the tumor extended into the subaortic outflow tract. The tumor, which had a dual attachment to the aortic and mitral valves, was safely removed without any valvar damage. We suggest immediate surgical intervention for those rhabdomyomas which are life-threatening because of their size, location, or arrhythmogenicity.

Introduction: The diagnosis of trisomy 21 in children has been associated with failed extubation after CHD surgery. Dexmedetomidine may be a useful agent to improve postoperative outcomes in these patients, such as ventilator time, ICU length of stay, or hospital length of stay. Materials and methods: The Pediatric Health Information System database was queried from January, 2008 to December, 2010 for patients with trisomy 21 who underwent CHD surgery. Patients who received dexmedetomidine were matched to patients who did not by propensity score. The primary outcome was ventilator days charged, and secondary outcomes included ICU and hospital length of stay. Results: A total of 1088 patients (544 matched pairs) met inclusion criteria. Patient characteristics were similar, with the exception of more patients in the dexmedetomidine group undergoing repair of complete atrioventricular canal and fewer undergoing mechanical valve replacement (p<0.01). More patients in the dexmedetomidine group were administered milrinone, epinephrine, vasopressin, benzodiazepines, opiates, and adjunct pain and sedative medications (p<0.01). The dexmedetomidine group had greater time on the ventilator [7 (4.5–11) versus 6 (4–10) days (median, interquartile range) p<0.01] and similar ICU length of stay, hospital length of stay, and mortality compared with controls. Mixed-effects modelling clustered on institution did not show beneficial effect of dexmedetomidine on ventilator time, ICU stay, or hospital length of stay. Conclusions: The use of dexmedetomidine was not associated with the decreased ventilatory time. Routine use of dexmedetomidine is not warranted in this patient population.

Twisted atrioventricular connections usually occur in hearts with biventricular artioventricular connections. Here, we describe a case of twisted atrioventricular valves associated with double inlet left ventricle and discordant ventriculo-arterial connections. Color Doppler echocardiography, and cine magnetic resonance imaging, clearly demonstrated that the right atrioventricular valve was located anterior and superior to the left atrioventricular valve, and that the axes of the two atrioventricular valves crossed each other within the dominant left ventricle.