Pediatric vascular ring outcomes for surgically repaired unoperated children: a single-center experience.

Background: Vascular rings represent 1% of congenital cardiovascular abnormalities. Phenotypic expression varies from asymptomatic to severe forms related to either oesophageal or tracheal compression. While refinement in prenatal screening led to an increase in fetal diagnosis, optimal management in asymptomatic neonates and infants is currently a matter of debate.

Atrial fibrillation in a pediatric patient caused by an unusual malignant etiology: A case report.

This case report describes a 15-year-old patient with a known congenital malformation syndrome and immune deficiency, presenting with new-onset atrial fibrillation (AF) after a recent diagnosis of an intrathoracic mass. Transthoracic echocardiography showed a structurally and functionally normal heart and workup confirmed a primary diffuse large B-cell lymphoma, with pericardial and left atrial involvement on cardiac magnetic resonance imaging. Electrical cardioversion was successfully performed to convert the AF and chemotherapy was promptly started.

Leftward deviation of the primary septum or dividing left atrial shelf?

Isolated leftward prolapse or deviation of the primary atrial septum is a rare CHD that can mimic abnormal pulmonary venous return at first sight. We present a case of a newborn infant, referred for surgical correction of totally anomalous pulmonary venous return into the right atrium, with the peri-operative finding of a leftward deviation of the superior margin of the primary atrial septum. The distinction with a dividing atrial shelf could not be confirmed with certainty.

Case Report: Disseminated Systemic Embolism of Lipiodol After Lymphography for Plastic Bronchitis After Fontan Repair.

Lipiodol-based lymphangiography is not only a diagnostic tool for visualization of lymphatic disorders such as plastic bronchitis (PB), but also aims a therapeutic effect by embolizing lymph leakages. We performed such percutaneous lymphatic embolization for PB in a Fontan patient with proven absence of right-to-left shunt, and demonstrated important lymphatic abnormalities in the mediastinum. Shortly after the procedure, the patient developed severe convulsive seizures, revealing multiple cerebral embolisms of Lipiodol.

Direct EBUS-guided transtracheal lymphosclerosis for plastic bronchitis after Fontan.

We report on a new puncture technique with direct transtracheal mediastinal lymphatic access to treat plastic bronchitis after Fontan repair. High resolution contrast-enhanced spiral CT identified enlarged lymph nodes in the paratracheal region. Inguinal intranodal Gadolinium Dynamic Contrast-enhanced Magnetic Resonance lymphangiography (DCMRL) confirmed the pathologic centrifugal lymph flow passing through these lymph nodes before leaking into the bronchial tree.

Percutaneous embolization of lymphatic fistulae as treatment for protein-losing enteropathy and plastic bronchitis in patients with failing Fontan circulation.

Background: To determine the feasibility and clinical result of selective embolization of hepatoduodenal or paratracheal lymphatics in Fontan patients with protein-losing enteropathy (PLE) or plastic bronchitis (PB).

Methods: Dilated lymph vessels in periportal (PLE) or paratracheal (PB) position were percutaneously punctured with a 22G Chiba needle. Intralymphatic position was confirmed by water soluble contrast injection with drainage to hepatoduodenal or tracheal fistulae.

Percutaneous obliteration of the right ventricle to avoid coronary damage by sinusoids in patients with pulmonary atresia intact ventricular septum during staged single ventricle palliation.

Background And Aims: Suprasystemic pressure waves can damage the coronary arteries resulting in myocardial ischemia and excess early mortality. We aimed to reduce the coronary pressure wave through the sinusoids by abolishing RV volume with percutaneous devices.

Methods And Results: Four patients with PA-IVS and coronary sinusoids from the hypertensive rudimentary RV were evaluated at a median age 26.