Transition and transfer from pediatric to adult care of the young adult with complex congenital heart disease.

This article focuses first on the process of transition and transfer of care of young adults with complex congenital heart disease. It defines the transition process and briefly discusses its history. It reviews the important aspects of transition, outlines the key elements of a successful transition program, and provides a curriculum appropriate for the young adult with congenital heart disease. Finally, it identifies the barriers to transfer of care, discusses the importance of a policy on timing, outlines the components of adult provider services that may be needed, and reviews the steps to an orderly transfer process.

Transcatheter device closure of congenital and postoperative residual ventricular septal defects.

BACKGROUND: Our purpose was to describe a 13-year experience with patients undergoing transcatheter device closure of unrepaired congenital or postoperative residual ventricular septal defects (VSDs). METHODS AND RESULTS: Since 1989, 170 patients (median age, 3.9 years) have undergone catheterization for closure of 1 or more congenital (n=92) or postoperative (n=78) residual VSDs using successive generations of STARFlex-type devices. Outcomes included echocardiographic assessment of residual flow and device position, assessment of VSD shunt/severity, and adverse events. Among 168 patients in whom device implantation was performed, between 1 and 7 devices were placed per patient (median, 1), with multiple devices placed in 40%. There was a significant decrease in left-to-right shunting after device implantation (P<0.001) and significant improvement in VSD size/severity, and device position proved stable. Of 332 adverse events, 39 were related to the device and 261 were related to the catheterization; all but 5 occurred in the periprocedural period. At a median follow-up of 24 months (0 to 154 months), 14 patients had died and 18 had device(s) explanted. CONCLUSIONS: Congenital and postoperative VSD closure using STARFlex-type devices resulted in stable improvement in clinical status and decreased interventricular shunting. Although periprocedural events occurred frequently, late events caused by the device were rare. Transcatheter device closure is an effective management option for patients with complex muscular VSDs that are difficult to approach surgically and for postoperative residual VSDs.

Respiratory symptoms secondary to aortopulmonary collateral vessels in tetralogy of Fallot absent pulmonary valve syndrome.

Hemodynamically significant systemic-to-pulmonary artery collaterals may represent an underappreciated cause of cardiorespiratory compromise in tetralogy of Fallot with absent pulmonary valve (TOF/APV). We retrospectively reviewed the angiographic, magnetic resonance imaging, operative, and autopsy reports of the 50 patients with TOF/APV managed at our institution in the past 10 years and demonstrated that at least 7 of 50 patients (14%) had significant aortopulmonary collaterals.

Interatrial communication through the mouth of the coronary sinus.

OBJECTIVES: We describe the structure of, and suggest an etiology for, the interatrial communication which can occur through the mouth of the coronary sinus. Based on the study of human embryos, we propose that the defect is best explained by dissolution of the wall of the coronary sinus adjacent to the left atrium, permitting shunting between the atriums through the right atrial orifice of the sinus. BACKGROUND: An interatrial communication across the mouth of the coronary sinus defect was first described in 1965 by Raghib and colleagues, its existence being predicated on the basis of incomplete formation of the left "atriovenous fold". Their hypothesis implies that the coronary sinus never develops, and thus the atrial septum itself is incomplete. METHODS: We have studied the development of the coronary sinus in a series of human embryos. Based on this work, we present the anatomical findings in 6 specimens with varying degrees of dissolution of the walls of the coronary sinus, and ten specimens with isomerism of the right atrial appendages, in which the sinus has never been formed. RESULTS: The coronary sinus defect is not a hole within the atrial septum, but a communication between the atriums through the mouth of the sinus. There was a range of defects in our series of specimens with usual atrial arrangement, extending from complete absence of the walls which normally separate the coronary sinus from the left atrium, to small fenestrations between this vessel and the left atrial cavity. In the hearts with isomerism of the right atrial appendages, however, we never observed an orifice of the coronary sinus. Thus, a coronary sinus defect cannot exist in this setting. CONCLUSIONS: Our findings indicate that the defect requires initial formation of the walls of the coronary sinus, but with subsequent dissolution of the wall adjacent to the left atrium. This produces a communication between the atriums through the mouth of the sinus.