In patients with CHD after Fontan palliation, there is a lack of a pumping chamber in the pulmonary circulation; thus, pulmonary blood flow and cardiac output are sensitive to increased pulmonary vascular resistance; if obstructive sleep apnoea is present, there are legitimate concerns from continuous positive airway pressure ventilation, which may hinder pulmonary blood flow.

Ischemia of the leg in a peripartum female is an uncommon condition. Paradoxical arterial embolisation, and arterial dissection, are rarely encountered but recognized causes of this clinical condition. Peripartum cardiomyopathy is a rare life-threatening cardiac condition that can foster intracardiac thrombosis and produce peripheral vascular complications through embolization. We present here the case of a young, healthy, postpartum female who developed acute ischemia of the left leg, and asymptomatic arterial insufficiency of the right lower leg, as the presenting symptoms of peripartum cardiomyopathy, highlighting the fact that ischemia threatening the loss of a limb can be the initial manifestation of peripartum cardiomyopathy.

Specific gene polymorphisms are known to be associated with a different arterial physiology in the younger generation. The present study found that young Russians with the matrix metalloproteinase 3 6A/6A and γ-glutamyltransferase 1AA genotypes have lower levels of the cardio-ankle vascular index – a recent measure of arterial stiffness. This observation may serve as an additional tool for cardiovascular disease prevention in the young population.

patients with congenitally malformed hearts characterised by a functionally single ventricle are currently treated using several procedures that bypass the right heart, such as the fontan and hemi-fontan operations, the bidirectional cavopulmonary anastomosis, and the total cavopulmonary connection. all these options are based on the procedures introduced for palliative correction of tricuspid atresia by fontan and baudet in 1971. introduced with the purpose of reducing the pre-operative volume overload, the surgical task mainly consists of separating the pulmonary from the systemic circulation. irrespective of the specific operation performed, we can call the resulting circulation the fontan circulation.

We analysed the occurrence of tetralogy of Fallot and simple transposition in the Hispanic and non-Hispanic populations of Clark County, Nevada, in the United States of America over a 30-year period from 1980 to 2009. We found a downward trend in the incidence of simple transposition of the great arteries in the non-Hispanic population but an upward trend in the incidence in the Hispanic population. For tetralogy of Fallot, we found an upward trend in the incidence in both populations; the trend, however, was more dramatic in the Hispanic population. We also noted differences in the male to female ratios in the different groups. Even though we make no definitive conclusions regarding the causes of these incidence curves or the differences in occurrence between males or females, the data suggest an interplay of genetics and the environment.

Although surgery is the standard treatment for native coarctation in neonates, it carries a high risk of complications. Percutaneous balloon angioplasty may be considered as an alternative treatment. The materials used in the intervention should be selected carefully to reduce complications. We recommended the use of non-compliant balloons in risky babies. They are more effective in the treatment of strick stenosis than compliant balloons.

A 3-year-old boy developed acute obstruction of a left modified Blalock-Taussig shunt following selective power injection of contrast in the shunt. Balloon dilation was ineffective due to rapid recoil of the narrowed segment, but implantation of a stent effectively abolished the obstruction. The obstruction itself may have been produced by an intimal flap caused by the power injection.

Anomalies of the innominate vein are uncommon in congenital cardiac disease. We report a case of duplicate innominate veins forming a vascular ring encircling the ascending aorta. We postulate that this vascular ring represents the failure of both a dorsal and ventral precardinal anastomosis to regress.

We describe an individualised approach in the rare situation of a tunnel between the left aortic sinus of Valsalva and the junction of the superior caval vein with the right atrium. It is necessary, in this rare anomaly, to identify the origin of the left coronary artery prior to intervention.

Atrial dysrhythmia is an important cause of mortality and morbidity in patients with atrial septal defect. Increased P wave duration can predict the risk of atrial dysrhythmia. The aim of this study is to evaluate the risk of atrial dysrhythmia by measuring P wave dispersion, and to observe the effect of surgical and transcatheter closure. Sixty-one patients and 30 controls were investigated. In patient group, before and after closure and in control group at the time of presentation, 12-lead electrocardiography records were evaluated. P wave duration and amplitude, P wave axis, PR interval, P wave dispersion, QRS axis, corrected QT interval, and QT dispersion were calculated. The measurements in patient and control groups, measurements before and after closure, and measurements of surgical and transcatheter group were compared. There were 27 patients in surgical group and 34 patients in transcatheter group. In patient group, signs of right heart volume overload were apparent but there was no significant difference in terms of P wave dispersion between two groups. We compared patient group in itself and found that while the use of medication, cardiothoracic index, ratio of right ventricular dilation, and paradoxical movement in interventricular septum were increased, mean age of closure was younger in surgical group. While P wave dispersion decreased in transcatheter group after closure, it increased in surgical group (p = 0.021). In conclusion, atrial septal defects may cause atrial repolarisation abnormalities and this effect persists even after surgical closure. Transcatheter closure in childhood may decrease dysrhythmia risk in long-term follow-up.

We report a 3-year-old boy with giant and atypical coronary artery aneurysms in the acute phase of Kawasaki disease, despite appropriate therapeutic intervention, in Noonan syndrome with a novel heterozygous PTPN11 mutation, c. 907 G>A (p.Asp303Asn). We hypothesised that this PTPN11 mutation might affect both hyperinflammation caused by Kawasaki disease and vascular fragility in the coronary artery, resulting in coronary artery aneurysms.

An 8-day-old neonate was presented with severe respiratory distress and diagnosed as primary pulmonary hypertension of the newborn on functional echocardiogram. Evaluation showed bounding pulse, enlarged umbilical cord, and bruit over the periumbilical region. Transthoracic echocardiography and CT angiogram showed a large fistulous communication between the umbilical vein and artery suggestive of congenital umbilical arteriovenous malformation leading to high-output cardiac failure and pulmonary artery hypertension. The patient was stabilised with medications and ventilation. Transcatheter closure of communication was done using coils, vascular plug, and KONAR-MFTM device. The patient improved from heart failure soon after the procedure and thriving normally at 6 months of follow-up.

In this study, three patients presenting with early or late postoperative Fontan complications were identified to suffer from restriction of the native atrial septum. This caused significant obstruction to pulmonary venous return and elevated systemic venous pressure. Dobutamine stress testing was used in one patient to identify this lesion. Transcatheter stenting was performed in the other two patients. Patients improved after relief of the obstruction.