Morphology of the patent ductus arteriosus does not preclude successful patent ductus arteriosus stent implantation in high-risk patients undergoing hybrid stage I palliation: recommendations to optimize ductal stent positioning.

OBJECTIVE: To describe patent ductus arteriosus (PDA) stenting regardless of ductal morphology in high risk patients with hypoplast physiology undergoing hybrid stage I palliation (PDA stenting and placement of bilateral pulmonary artery bands). BACKGROUND: Hybrid palliation is an accepted alternative for patients with hypoplastic left heart syndrome. Patients weighing less than 2.5 kg, history of prematurity, intracranial hemorrhage, and chromosomal abnormality belong to a high-risk group who otherwise might not be ideal candidates for traditional surgical repair (Norwood Operation). METHODS: Between May 2005 and February 2013, a series of 13 high-risk patients with hypoplast physiology with varying types of ductal morphology underwent PDA stenting as part of hybrid stage I palliation. RESULTS: Three major types of ductal morphology were identified: (1) short and semi horizontal, (2) long and semi horizontal, and (3) tortuous. All patients underwent successful PDA stenting. One patient developed proximal coarctation from inadequate coverage of the pulmonary end of the PDA and was successfully treated with a balloon expandable stent 69 days after the initial procedure. CONCLUSIONS: Mid- to long-term follow-up indicates that PDA stents remain widely patent regardless of ductal morphology until comprehensive stage II repair.

Right pulmonary artery thrombosis after hybrid stage 1 palliation: resolution after direct tissue plasminogen activator infusion.

A patient with unbalanced right ventricular dominant atrioventricular septal defect with ascending and transverse arch hypoplasia underwent hybrid stage 1 palliation. On postoperative day 7, he experienced irritability and acute cyanosis. Echocardiography demonstrated a thrombus occluding the right pulmonary artery band. Emergency cardiac catheterization confirmed thrombosis of the right pulmonary artery at the site of the band. Direct infusion of tissue plasminogen activator successfully lysed the thrombus.

A Case of Trisomy 13 Mosaicism With Aortic Root Dilatation.

We present a 6-year-old female child, with prenatal diagnosis of trisomy 13 mosaicism and nondisjunction, a bicuspid aortic valve, and severe aortic root dilatation. This patient is only the third reported case of aortic dilatation in the setting of trisomy 13 mosaicism. (Level of Difficulty: Intermediate.).

Occlusive Pulmonary Artery Thrombosis in a Healthy Neonate With No Identifiable Risk Factors.

Pulmonary artery thrombosis is reported in neonates with risk factors for hypercoagulability. No consensus exists regarding standard therapy for this condition. We present a neonate, with no risk factors for thrombosis, who was admitted after birth to the Pediatric Cardiac Intensive Care Unit with an occlusive left pulmonary artery thrombus. (Level of Difficulty: Intermediate.).

Ventricular septal avulsion and ventricular septal defect after blunt trauma.

Traumatic ventricular septal defect is an uncommon complication of blunt chest trauma. We report a case of ventricular septal avulsion, associated with traumatic ventricular septal defect and the associated management concerns.

Culture negative endocarditis caused by Bartonella henselae in a child with congenital heart disease.

A 9-year-old white male with congenital aortic and subaortic stenosis palliated by the Ross-Konno procedure presented with culture-negative endocarditis. Serologic studies and polymerase chain reaction testing of resected homograft valvular tissue provided evidence of Bartonella henselae as the etiology. B. henselae can cause endocarditis in children, particularly those with underlying valvular disease. Serologic testing for B. henselae should be considered in children with culture-negative endocarditis.

Expression and developmental regulation of the NMDA receptor subunits in the kidney and cardiovascular system.

Antagonists to the N-methyl-D-aspartate (NMDA) receptor bind to various extraneuronal tissues. We therefore assessed the expression of the main NMDA subunit, NR1, in various tissues. We demonstrate that NR1 appears to be most abundant in the rat kidney and heart. NR1 is present in total rat kidney, cortex, and medulla. Of the NR2 subunits, only the NR2C subunit protein is present in the kidney. The abundance of the NR1 subunit protein increases with kidney development. Both NR1 and NR2C are present in opossum kidney, Madin-Darby canine kidney, and LLC-PK(1) cells. Immunohistochemistry studies show that the NR1 subunit is present in the renal proximal tubule. NR1 is abundant in the atrium and ventricle but is also expressed in the aorta and pulmonary artery. The NR2 subunits are not expressed in the heart. NR1 subunit protein expression is constant throughout heart development. Finally, the NR1 subunit protein is expressed in heart cells (H9c2) grown in culture. These studies reveal the presence of the NMDA receptor in the kidney and the cardiovascular system.