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In this report, a unique case of a symptomatic vascular ring formed by right aortic arch, aberrant left subclavian artery, and left ligamentum arteriosus in which there is atresia of the proximal left subclavian artery is described. Imaging modalities were non-diagnostic and the patient was sent to surgery based on strong clinical suspicion. Her anatomy was delineated in the operating room and the ring was successfully repaired.
We had two cases of neonates with hypoplastic left heart syndrome and intact atrial septum who had unique levoatriocardinal veins. Contrast-enhanced CT and angiography revealed that previously unknown communicating vessels ran from the top of the left atrium and drained into the right atrium. We emphasise that transcatheter atrial septostomy should be performed not through these communicating vessels but using the trans-septal approach in neonates with hypoplastic left heart syndrome and an intact atrial septum.
Truncus arteriosus is a rare cyanotic congenital heart defect that involves septation failure of the heart’s main arterial outflow tract. Varying morphologies of the truncal valve and aorta have been reported; however, the ascending aorta is typically supplied via anterograde blood flow through the truncal valve. We present the first reported case of neonatal truncus arteriosus with the ascending aorta being supplied entirely by retrograde flow.
An asymptomatic 6-year-old boy with a history of right lung hypoplasia was referred for cardiology evaluation. Echocardiography demonstrated right pulmonary artery hypoplasia with flow reversal in that vessel. The right pulmonary veins were not visualised in the echocardiogram. Cardiac catheterisation confirmed the diagnosis of scimitar syndrome with a characteristic large vertical vein; however, the right pulmonary veins were found to be atretic with no connection to the heart with decompression through the azygos vein. In all, four systemic to pulmonary arterial collaterals were identified, supplying the right lung, which were occluded using embolization coils. This case demonstrates the potential for progressive stenosis and atresia of the so-called “scimitar vein” without previous surgical instrumentation, and that this can occur without haemodynamic embarrassment or development of pulmonary vascular disease.
We present the case of a 10-month-old female with a right coronary sinus of Valsalva aneurysm with rupture into the right atrial appendage who presented with a murmur. Surgical repair was performed shortly after diagnosis with pericardial patch closure from within the aorta and closure of the right atrial rupture site. To our knowledge, this is the youngest child with sinus of Valsalva aneurysm with rupture to be identified in the literature.
Known as a benign arrhythmia and normally requiring no specific treatment, accelerated idioventricular rhythm can rarely degenerate to a life-threatening arrhythmia. Here, we present a child with left coronary cusp-originating accelerated idioventricular rhythm, degenerating into torsades de pointes and resulting in cardiac arrest, which was ablated with a cryocatheter. An 11-year-old boy, followed due to asymptomatic accelerated idioventricular rhythm before, was referred to our department because he had experienced an aborted cardiac arrest during sleep. He had been resuscitated for 5 minutes. Twenty-four-hour Holter-ECG revealed incessant accelerated idioventricular rhythm, consisting up to 90% of the whole record and two torsades de pointes attacks, triggered by accelerated idioventricular rhythm-induced “R on T” phenomenon, and resulting in syncope and cardiac arrest. Transthoracic echocardiography revealed no structural cardiac defect but mild left ventricular systolic dysfunction with an ejection fraction of 45% and shortening fraction 23%. An electrophysiologic study was conducted, and accelerated idioventricular rhythm focus was mapped to left aortic coronary cusp. A cryocatheter with an 8-mm tip was preferred for successful ablation of the accelerated idioventricular rhythm focus, due to close neighbourhood to coronary ostium. The patient was discharged in 3 days without any premature ventricular contractions or accelerated idioventricular rhythm and with normalised cardiac functions. After 9 months on follow-up, he was still asymptomatic, without any premature ventricular contractions or accelerated idioventricular rhythm and with normal cardiac functions. Although the clinical course of accelerated idioventricular rhythm is known as benign, accelerated idioventricular rhythm can rarely degenerate to a life-threatening arrhythmia. In such cases, electrophysiologic study and catheter ablation are a good option in such cases with accelerated idioventricular rhythm for an ultimate cure.
Septum primum malposition defect is an extremely rare CHD and is often found in patients with visceral heterotaxy, particularly of the polysplenia type. We describe a unique patient with dextrocardia, situs inversus totalis, and bilateral superior vena caval veins without heterotaxy syndrome who was diagnosed with an interatrial defect and partial anomalous pulmonary venous drainage due to malposition of the septum primum.
Ductal arterial spasm is a very potentially dangerous incidence during percutaneous device closure of patent ductus arteriosus (PDA), which, otherwise, is a very safe catheter intervention. It is essential to notice its occurrence before device sizing and deploying. Without awareness, it can mislead device selection and can result in serious complication. In this report, we shared our nightmare of ductal spasm during transcatheter closure of PDA in two children which had led to death in one patient.
An isolated left common carotid artery is very rare, and only 13 cases have been reported thus far. All those cases were accompanied by a right aortic arch and aberrant left subclavian artery, and the connecting vessel between the pulmonary artery and left common carotid artery was thought to be ductal tissue. However, there have been no reports that have followed the natural closure of this vessel. We present a case in whom we could observe the closing process of this vessel at the connection between the left common carotid artery and main pulmonary artery in association with a tetralogy of Fallot.
The incidence of paediatric venous thromboembolism has steadily increased in the past decade, by nearly 10% per year. Deep venous thrombosis may remain completely asymptomatic during the acute phase and symptoms may occur later, due to complications. We related the case of a 9-month-old child with increasing cyanosis. A computed tomography (CT) angiography showed a thrombosis of the superior vena cava (SVC) with the development of collateral flow from the systemic to the pulmonary veins. Transcatheter shunt occlusion after SVC recanalization was successfully performed. We discussed the characteristics of these cases and the consequence on our practice in term of treatment (anticoagulation, transcatheter, intervention) and screening.
Cor triatriatum sinister is a rare congenital abnormality, with varying signs and symptoms related to the anatomic features and the components of the accompanying cardiac abnormalities. We present a 3-year-old girl who underwent successful simultaneous operation with complete loss of the membrane and ligation of vertical vein by anomalous venous return. The principles for surgical management of cor triatriatum can be summarised as radical resection of the membrane and correction of other intracardiac anomalies.
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.
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.
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.
In this study, we report a patient with pulmonary atresia with intact ventricular septum (PA/IVS), confluent pulmonary arteries supplied by an arterial duct, and chromosome 22q11.2 microdeletion. The 22q11.2 deletion syndrome has been associated with anomalies of the outflow tracts, such as tetralogy of Fallot with either pulmonary stenosis or atresia, but we are aware of a solitary case described with pulmonary atresia when the ventricular septum is intact. The presence of genetic malformations can have long-term co-morbidities. By describing our patient, we aim to create awareness of this rare association.
Respiratory symptoms are prevalent in vascular ring anomalies, including double aortic arch, which is considered the most common type of vascular ring. However, respiratory failure owing to critical airway obstruction in the neonatal period is rare with few reported cases in the literature. We report a case of a newborn patient who required emergent intubation within the first few hours of life and was later discovered to have a double aortic arch.
We present a case of a 57-year-old man with congenitally corrected transposition of great vessels who was found to have coronary ostial atresia with cranial flow through left superior vena cava on CT with contrast injection in coronary tributaries. As such, he was ineligible for cardiac resynchronization therapy.
We describe the case of a 21-year-old patient who underwent repairs for multiple lesions including aortic and pulmonary valve replacements, right ventricular outflow tract reconstruction, revision of the right pulmonary artery route, and a repair of partial anomalous pulmonary venous drainage, which was diagnosed during this fourth sternotomy. For these patients with adult CHD, it is most important to address all underlying factors as much as possible at the redo surgery.
We report a rare case of anomalous aortic origin of the right coronary artery from the posterior/non-coronary sinus of Valsalva in a 9-year-old male diagnosed during the workup of premature ventricular contractions. The finding was initially noted on transthoracic echocardiogram and further confirmed with computed tomography. The anomalous coronary artery shows a wide ostium with no intramural or interarterial course.
We present the second reported case of left pulmonary artery sling with dextrocardia, right pulmonary hypoplasia, and total pulmonary venous connection in a fetus. This case highlights the importance of the determination of pulmonary artery arrangement by fetal echocardiography if right pulmonary hypoplasia and/or congenital heart disease is suspected.