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.

Intracardiac teratomas are rare primary tumours. We report the case of an infant prenatally diagnosed with an isolated multi-cystic mass developed in the right ventricle causing neonatal refractory ventricular arrhythmia. Despite rescue extracorporeal support and partial surgical resection, he died as almost all the previous reported perinatal intracardiac teratomas whatever the prenatal tolerance and the size of the tumour. The common poor outcome of fetal intracardiac teratomas should be known when counselling parents during pregnancy.

We report of a 26-year-old female patient who was referred to our centre with congestive heart failure (CHF). Acute myocarditis with a high Parvovirus B19 virus load was diagnosed by myocardial biopsy. CHF improved after start of ramipril 5 mg/d, metoprolol, diuretics, immunoglobins, and a 24-hour infusion of levosimendan. Soon after initiation of medical therapy, the patient started to expectorate bronchial casts with varying frequencies (three times per week to five times daily). Thorough pneumological workup, including histology of the casts, microbiology, and a CT scan of the lungs, did not reveal any cause for bronchial cast formation. Inhalative corticoids were started without any benefit. Two years later, cardiac catheterisation demonstrated normalised left ventricular function. LV end-diastolic pressure, however, was still elevated at 14 mmHg. Endomyocardial biopsies at this time were negative for virus genome. Finally, we changed afterload reduction therapy from ramipril to candesartan. Within 24 hours, expectoration of bronchial casts terminated. Four weeks later, re-exposition to ramipril prompted immediate re-appearance of cast formation, which again stopped with switching back to candesartan. Finally, we were to prove that treatment with ramipril resulted in bronchial cast formation in this patient.

We report the case of a 14-year-old female who had tetralogy of Fallot along with anomalous origin of the left pulmonary artery from the ascending aorta with co-dominant double aortic arch forming a complete vascular ring compressing the oesophagus along with a left main coronary artery to right ventricular outflow tract fistula. She underwent surgical correction without conduit placement.

Takotsubo cardiomyopathy is characterised by akinesis and ballooning of the left ventricular apex during contraction of the otherwise normal base of the heart. We describe the case of a 7-month-old previously healthy female who presented with an unwitnessed cardiac arrest. Workup raised suspicion for non-accidental trauma. Despite progression to brain death, the severely decreased ventricular function and apical akinesis of the left ventricle improved within 40 hours of admission. This report will familiarise paediatricians with this rare cardiomyopathy and emphasise the importance of considering non-accidental trauma as an inciting event for patients with unwitnessed cardiac arrest found to have decreased ventricular function.

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.

Congenital complete tracheal rings are usually associated with pulmonary slings. We report a rare association of congenital complete tracheal rings with hypoplastic left heart variant. A term infant with diagnosis of a mildly hypoplastic mitral valve, unicuspid aortic valve, and moderately hypoplastic aortic arch with severe coarctation underwent a hybrid procedure initially. Upon failing extubation attempts, complete tracheal rings were seen on direct laryngoscopy. The combination of the lesions resulted in a poor outcome. In patients with failure of extubation post-cardiac surgery, a diagnosis of complete tracheal rings should be included in the differential and a direct laryngoscopy should be considered.

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.

CHDs occasionally have coronary complications; however, stent implantation is technically difficult in small children. We reported a successful drug-eluting stent implantation into the congenital anomalous coronary artery in a 6-year-old boy. This treatment is useful for rescuing coronary stenosis, and dual antiplatelet therapy is important to prevent stent restenosis.

A newborn with prenatally diagnosed dysplasia of both atrioventricular valves presented after birth with signs and symptoms of low cardiac output, severe regurgitation of both mitral and tricuspid valves. This combination is as rare as challenging, since it regards both the timing and management of this complex cardiac malformation. We report an early surgical repair of both atrioventricular valves in a symptomatic newborn, which improved his clinical status and, so far, delayed valve replacement.

A shorter umbilical venous approach provides an opportunity for balloon atrial septostomy in the younger neonate as opposed to those who present at the end of first week of life. However, the ideal choice of access for a bedside balloon atrial septostomy is not well established. Wouldn’t prostaglandin infusion be a safer option for transport of babies with dextro-transposition in the neonatal period, when the arterial duct can be kept open? A prenatal diagnosis of dextro-transposition facilitates monitoring and planning of septostomy in the early neonatal period explaining why babies underwent bedside procedures more often.