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.

Premature ductal constriction was diagnosed in a 30-week gestation fetus with hypoplastic left heart syndrome. The fetus developed right ventricular hypertrophy and mild tricuspid regurgitation. Foetal neuroimaging showed slowed growth of the brain and increased lactate. We describe the imaging and clinical findings. Fetuses with critical CHD and in utero ductal constriction are at increased risk of morbidity and mortality, and require appropriate counselling.

We present the surgical implantation of the Edwards Inspiris Resilia® aortic valve in mitral position for mechanical mitral valve failure in a severely ill infant after valve replacement because of anomalous origin of the left coronary artery from the pulmonary artery. The biological valve was chosen because the child could not receive oral anticoagulation and was for several months on heparin infusion. The procedure was safely performed with good haemodynamic result.

Pulmonary artery stenting may not be possible transcutaneously because of anatomic features. Although intraoperative stenting has been well described, we present a case in which stenting of the left pulmonary artery was performed transthoracically in a separate procedure. Unusual anatomic conditions may require a multi-disciplinary hybrid approach to achieve the desired results.

The Fontan operation is a palliative surgical procedure for patients whose hearts cannot support a biventricular circulation. The haemodynamic changes that occur in pregnancy are particularly challenging for Fontan patients and the outcomes are variable. We present a case where fetal outcome was particularly poor despite a lack of high risk features pre-pregnancy.

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.

Giant coronary artery aneurysms are a complication of Kawasaki disease and can be fatal if associated with thrombosis. We describe the clinical outcome of a boy with Kawasaki disease who exhibited “supergiant” coronary artery aneurysms at the age of 14 months and, despite treatment with anticoagulant and antiplatelet medication, developed a left coronary artery thrombosis and presented following a myocardial infarction at 2 years old. Although his symptoms were minimal, the myocardial infarction was identified by abnormal Q-waves and giant negative T-waves in precordial leads of routine electrocardiography. Intensive anticoagulant therapy combining heparin injections and high-dose warfarin was successful. The abnormal Q-waves and negative T-waves had completely disappeared 2 weeks later, likely in association with confirmed reperfusion. On the basis of prompt identification of abnormal Q-waves by electrocardiography, the patient could avoid thrombolytic therapy and catheter or surgical intervention.

A 15-month-old child underwent percutaneous expansion of a Melody transcatheter pulmonary valve in the mitral position to accommodate growth after initial surgical implantation during infancy, but transiently decompensated after valvuloplasty owing to stent malformation. The Melody valve in the mitral position of small patients can be further expanded by percutaneous dilation, but there are a number of potential complications and technical improvements to consider.

Acquired diaphragmatic hernia is a rare complication of pediatric intervention or surgery. In this study, we report an infant with iatrogenic diaphragmatic hernia following neonatal complex congenital cardiac surgery, and then we review the associated literature.

In approximately 5% of patients with idiopathic recurrent pericarditis, the disease usually follows a chronic relapsing course, and children can develop dependence and side effects of prolonged high-dose corticosteroid regimens. In this setting anakinra, a recombinant human interleukin-1 competitive receptor antagonist that blocks the biologic effects of interleukin-1, thereby reducing systemic inflammatory responses, appears to be one of the most promising strategies. We report an adolescent with steroid-dependent idiopathic recurrent pericarditis that was successfully treated with anakinra, highlighting that this therapeutic option seems to be an effective, rapidly acting, steroid-sparing, and relatively safe agent for the treatment of this entity in children.

Cardiac hydatid cysts are a rare presentation of hydatid cyst disease in the body, with a reported cardiac involvement rate of <2%. The left ventricle is the most common site of cardiac involvement. Here, we report a patient with a hydatid cyst that ruptured into the pericardium after producing an aneurysm on the right ventricular free wall, appearing as fibrinated fluid and a solid mass lesion in the pericardium. Our aim in this case report was to emphasise that the possibility of a hydatid cyst should not be overlooked in the differential diagnosis of pericardial tumours.

A 5-year-old girl presented with chronic fatigue and extreme exercise intolerance. After countless doctor visits, investigations, and hospital admissions, striking desaturation during exercise test pointed to a cardiovascular problem. Desaturation as a result of right-to-left shunting through a patent foramen ovale during upright exercise was hypothesised. A cardiac catheterisation confirmed the presence of an unusually cranially located patent foramen ovale; the defect was subsequently closed. Physical re-evaluation 6 weeks later showed spectacular physical and mental improvement and stabile saturation during exercise.

Kleefstra syndrome (chromosome 9q34.3 deletion) is a rare genetic disorder with less than 110 patients reported till date. We report a 4-month-old Caucasian girl with Kleefstra syndrome and Shone’s complex, an association which has not been previously reported. Surgical planning for patients with Kleefstra syndrome and complex CHD can pose challenges due to an uncertain natural history and a risk of post-operative pulmonary hypertension.

We present a young soldier presenting with aborted sudden cardiac death, who was found to have concomitant hypertrophic cardiomyopathy and Wolff–Parkinson–White syndrome. Along with pathological haemodynamic features of hypertrophic cardiomyopathy, an easily-inducible re-entrant tachycardia was clearly documented in our patient. Given the fatal potential of supraventricular tachycardia in hypertrophic cardiomyopathy, we postulated that his tachyarrhythmia could potentially trigger the event. Upon his refusal to receive implantable cardioverter/defibrillator therapy, we ablated anatomical arrhythmogenic substrate instead, and he remained uneventfully over 3 years on β-blocker.

Coronary compression exclusion during right ventricle outflow tract stenting is recommended and potential oversight may be fatal. Balloon inflation in right ventricle outflow tract with simultaneous aortography can be inconclusive or falsely negative. We present a case of 27-year-old male post Ross operation qualified for percutaneous pulmonary valve implantation. Neither of the conventional views obtained provided a definite exclusion of coronary compression, therefore an intravascular ultrasound of the left coronary artery before and during balloon inflation in right ventricle outflow tract was performed. Acquired images allowed excluding potential constriction, thus a covered stent and pulmonary valve were implanted and the procedure was concluded. Two hours later, the patient complained of chest pain. Transthoracic echocardiography demonstrated a significant pericardial effusion. Retrospective analysis of the final angiogram revealed a possibility of subtle extravasation at the distal part of the homograft. A hybrid procedure consisting of additional covered stent implantation, pericardial drainage, and second pulmonary valve implantation was performed with an acceptable result. To conclude, in case of doubtful or unconvincing images obtained from aortography or selective angiography during balloon inflation in right ventricle outflow tract, intravascular ultrasound might be a feasible and useful technique. Signs of homograft rupture may be subtle, whereas symptoms of cardiac tamponade delayed. In selected patients percutaneous treatment of homograft rupture is achievable and beneficial.