Andrew H. Van Bergen

Inadvertent stenting of patent ductus arteriosus with Amplatzer Vascular Plug

We report a 12‐month old patient who presented for murmur evaluation after percutaneous closure of type C patent ductus arteriosus (PDA) using a 10 mm Amplatzer Vascular Plug (AGA Medical Corporation, Golden Valley, MN) at an outside institution. Echocardiography revealed a large left‐to‐right shunt through the implanted device, inadvertently stenting the PDA instead of closing it. The patient underwent repeat catheterization with successful coil implantation within the Amplatzer Vascular Plug, completely eliminating the large residual ductal shunt. Although challenging, this case illustrates the technique of implanting coils within this occlusion device. This case also illustrates that occlusion of type C PDA utilizing the Amplatzer Vascular Plug may not only result in incomplete occlusion but also create a potentially worse clinical situation in which the PDA is stretched larger and stented open. Without consideration of simultaneous coil implantation within this device, use of the Amplatzer Vascular Plug might be contraindicated in type C PDA, because there may be no way to ensure successful closure by the Vascular Plug alone.

Mitral stenosis and aortic atresia in hypoplastic left heart syndrome: survival analysis after stage I palliation.

BACKGROUND Mitral stenosis with aortic atresia (MS-AA) has been implicated as a risk factor for decreased survival after stage 1 palliation for hypoplastic left heart syndrome. Conflicting results were reported in the literature evaluating the association of anatomic subtypes and mortality. Our objective was to determine whether MS-AA is associated with increased mortality after stage 1 palliation. METHODS Between January 2005 and May 2009 100 consecutive neonates with hypoplastic left heart syndrome underwent stage 1 palliation. Echocardiograms were reviewed for patency of the mitral and aortic valves as well as presence of ventriculocoronary connections (VCC). Patients were divided into (1) mitral and aortic atresia (MA-AA), (2) MS-AA, and (3) mitral and aortic stenosis groups. Survival analysis was performed, and impact of MS-AA and VCC on early and midterm survival was assessed. RESULTS Of the 100 patients, 31 had MA-AA, 42 had mitral stenosis and aortic stenosis, and 27 had MS-AA (15 with VCC and 12 without). Stage 1 palliation 30-day survival was 90% for the entire cohort and 88.9% for the MS-AA subtype (not significantly different). Six-month survival was 70% overall and 70.4% for MS-AA (not significantly different). When VCC was taken into account, MS-AA with VCC reached 93% survival (versus 50% for MS-AA without VCC; p < 0.01). CONCLUSIONS Stage 1 palliation for hypoplastic left heart syndrome is associated with satisfactory hospital and midterm survival regardless of anatomic subtype. The variants MS-AA and MA-AA are associated with smaller ascending aorta. Mitral stenosis with aortic atresia or VCC did not adversely influence survival.

CASE REPORTS: Transcatheter Closure of Ruptured Sinus of Valsalva Aneurysm and Secundum Atrial Septal Defect with Limited Inferior Rim

We report a case of a 33‐year‐old man with Down syndrome and ruptured sinus of Valsalva aneurysm (SOVA) plus large ostium secundum atrial septal defect (ASD) with attenuated inferior rim. He underwent successful transcatheter closure of both defects at two separate procedures with transesophageal echocardiography (TEE) guidance. The combination of these two procedures has not been previously reported. Transgastric echocardiographic views which mimic transthoracic subcostal echocardiographic views provide high quality useful images of the anatomy, hemodynamics, and device placement.

Coronary Artery-to-Pulmonary Artery Fistula in a Case of Pulmonary Atresia With Ventricular Septal Defect

Pulmonary atresia with ventricular septal defect (PA-VSD) can be associated with varying pulmonary artery connections. The origin of pulmonary blood flow can vary greatly among patients, and some case reports have described the presence of left coronary artery-to-pulmonary artery fistula. Two cases of patients found to have coronary artery-to-pulmonary artery fistula in the settings of PA-VSD are reported.

Extensive Left Ventricular to Coronary Artery Connections in Hypoplastic Left Heart Syndrome

We demonstrate the echocardiographic features of a rare case of very extensive left ventricular to coronary arterial connections (VCC) to both the right and left coronary arteries in a fetus and subsequent neonate with hypoplastic left heart syndrome (HLH). The right coronary artery, after receiving multiple VCC supplied the only antegrade ascending aortic flow. The left main coronary artery was very hypoplastic and the left anterior descending coronary artery was dilated and tortuous with multiple large VCC. The left circumflex coronary artery arose exclusively from its own VCC. To our knowledge this is the first such echocardiographic demonstration of HLH with extensive bilateral VCC and coronary artery stenosis and interruption.

Novel approach to the newborn with hypoplastic left heart syndrome and intact atrial septum

A prenatally diagnosed fetus with hypoplastic left heart syndrome and intact atrial septum was delivered in the cardiac catheterization suite. Using radio frequency energy, a transseptal perforation of the thickened and intact atrial septum was immediately performed following transcatheter cannulation of the right atrium via the umbilical vein. Serial cutting balloon septostomies followed by static balloon septostomies resulted in effective left atrial decompression, atrial mixing, and optimal pulmonary and systemic perfusion. The child is now thriving after both stage I Norwood and bidirectional Glenn procedures.

Management of hypoplastic left heart syndrome with intact atrial septum: a two-centre experience

UNLABELLED Introduction Hypoplastic left heart syndrome with an intact atrial septum is a poor predictor of outcomes. Prenatal assessment of pulmonary venous Doppler and emergent postnatal cardiac intervention may be associated with better outcomes. Materials and methods A retrospective review of all hypoplastic left heart syndrome patients in two centres over a 5-year period was performed. Group 1 included patients with adequate inter-atrial communication. Group 2 included patients with prenatal diagnosis with an intact atrial septum who had immediate transcatheter intervention. Group 3 included patients with intact atrial septum who were not prenatally diagnosed and underwent either delayed intervention or no intervention before stage 1 palliation. Primary outcome was survival up to stage 2 palliation. RESULTS The incidence of hypoplastic left heart syndrome with a restrictive atrial communication was 11.2% (n=19 of 170). Overall survival to stage 2 or heart transplantation was 85% and 67% for Groups 1 and 2, respectively (n=129/151, n=8/12; p=0.03), and 0% (n=0/7) for Group 3. Survival benefits were observed between Groups 2 and 3 (p<0.001). Foetal pulmonary vein Doppler reverse/forward velocity time integral ratio of ⩾18% (sensitivity, 0.99, 95% CI, 0.58-1; specificity, 0.99, 95% CI, 0.96-1) was predictive of the need for emergent left atrial decompression. CONCLUSION Using a multidisciplinary approach and foetal pulmonary vein Doppler, time-saving measures can be instituted by delivering prenatally diagnosed neonates with hypoplastic left heart syndrome with intact atrial septum close to the cardiac catheterisation suite where left atrial decompression can be performed quickly and safely that may improve survival.

Technical considerations for closing secundum atrial septal defect in the small child with the HELEX septal occluder via transhepatic access

We describe closure of an atrial septal defect in a symptomatic 4.6 kg child with the HELEX Septal Occluder (W.L. Gore and Associates, Flagstaff, AZ) via transhepatic approach. Technical considerations are discussed.

Improved Results of Aortic Arch Reconstruction in the Norwood Procedure.

BACKGROUND Despite several surgical modifications, the reported incidence of neoaortic coarctation after stage 1 palliation (S1P) of hypoplastic left heart syndrome remains high. A modification of aortic arch reconstruction that may decrease the incidence of neoaortic coarctation after S1P is described. METHODS Between January 2008 and May 2013, 114 patients with hypoplastic left heart syndrome underwent S1P. The hospital records of the 101 surviving patients (89%) were reviewed. Demographic data and perioperative variables for S1P were collected. A modified technique for aortic arch reconstruction that contains a bovine pericardial patch tailored and sutured in a specific way was used in 51 patients (group MT), whereas the traditional technique was used in 50 patients (group TT). Clinical echocardiographic and cardiac catheterization data were analyzed to determine the incidence and location of neoaortic coarctation. RESULTS There were no significant differences in the baseline characteristics, the intraoperative course, or hospital mortality between the two groups of subjects. Duration of ventilation, intensive care unit stay, and hospitalization were significantly shorter in group MT. The mean follow-up was 2.02 ± 0.63 years and 3.98 ± 0.66 years in each group, respectively. Coarctation developed in 4 of 51 patients (7.8%) in group MT compared with 10/50 patients (20%) in group TT. Multivariable logistic regression analysis showed that the estimated odds of coarctation for group MT were 0.41 (95% confidence interval: 0.04, 4.32) times the estimated odds of coarctation for group TT. CONCLUSIONS The described technique may decrease the incidence of recoarctation after S1P by minimizing aortic arch and descending aorta distortion and providing ample enlargement of the aorta at its narrowest diameter.