Right ventricular outflow tract obstruction after sinus of valsalva aneurysm repair.

Sinus of Valsalva aneurysm (SVA) is a rare cardiac lesion especially in the western countries and older population. We report an unusual case of a 60-year-old Caucasian male with SVA, acute decompensation, and a pressurized prolapsed aortic leaflet cystic remnant via a small supracristal VSD causing recurrent right ventricular outflow tract obstruction following a Bentall procedure

Carpentier edwards porcine valved conduit for right ventricular outflow tract reconstruction.

BACKGROUND: The optimal conduit for right ventricular outflow tract (RVOT) reconstruction is uncertain, with varying degrees of longevity reported for pericardial, homograft, and xenograft valves utilized in this position. METHODS: A retrospective review of children and adults with congenital heart disease who underwent RVOT reconstruction with the Carpentier Edwards™ (CE) porcine valved conduit was conducted from 2001 to 2009 at the University of Rochester and SUNY Upstate Medical Centers. Clinical data were analyzed for each subject according to conduit size, and all of the Doppler derived transconduit gradients from postoperative echocardiograms were analyzed. RESULTS: Two hundred and eighteen patients received a single CE conduit for RVOT reconstruction with conduit size ranging from 12 to 30 mm. Perioperative mortality was 1.8% (4/218). Follow-up data were available for 95% of subjects with duration of follow-up ranging from 1 to 9 years. The increase in transconduit gradient over time was inversely proportional to conduit size. For the entire series, freedom from reoperation was 70.3% at 8.2 years. Patients receiving 25 and 30 mm conduits demonstrated no gradient development over this period of follow-up. CONCLUSIONS: In this series, the CE conduit showed excellent longevity at intermediate term follow-up, with slower progression of conduit stenosis as measured by RVOT gradient change compared with previous reports.

Elevated risk of thrombosis in neonates undergoing initial palliative cardiac surgery.

BACKGROUND: Thrombotic events cause significant morbidity and mortality in children who undergo surgery for complex congenital cardiac disease. We prospectively evaluated the incidence of thrombosis and examined preoperative and postoperative laboratory tests of coagulation and inflammation in neonates experiencing initial surgical palliation for variations of single ventricle physiology. METHODS: Neonates (<30 days) requiring initial surgical palliation were studied. All subjects received aspirin from postoperative day 1 onward. Thromboses were diagnosed by serial transthoracic echocardiograms, vascular imaging, and interstage cardiac catheterizations according to predefined criteria. RESULTS: Twenty-two neonates, age 1 to 11 days (mean 4 +/- 2.5) were studied. Follow-up ranged from three hours to 18 months (median, 212 days). Eight infants died. Four of the 14 subjects who survived (28%), and one of the eight who died (12.5%), had evidence of thrombosis identified over a range of four hours to nine months postoperatively (median 14 days). When compared with reference values established in healthy children, preoperative subject hematocrit (Hct), platelet count, factors II, V, VII, VIII, and X, antithrombin, protein C, and soluble CD40 ligand measures were significantly lower, and the prothrombin time and partial thromboplastin time were significantly higher. Postoperative C reactive protein (CRP) was significantly higher, and Hct and platelet count significantly lower, than preoperative values. Thrombotic events were significantly related to high preoperative CRP (p = 0.02). CONCLUSION: Thrombotic complications occur frequently in neonates undergoing initial palliative surgery, suggesting that aspirin therapy alone may constitute inadequate protection. Elevated preoperative CRP appears to be associated with increased thrombotic risk.

Aortopulmonary Window with Interrupted Aortic Arch and Pulmonary Artery Sling: Diagnosis by Echocardiography and Magnetic Resonance Imaging: Case Report and Literature Review.

We present the pediatric case of a patient who had aortopulmonary window with interrupted aortic arch, intact ventricular septum, and pulmonary artery sling that was imaged with echocardiography and magnetic resonance imaging. This case is unique because this combination of findings has not been previously reported. The diagnosis was made without the need for angiography, and the findings were confirmed at surgery. This case shows that complex congenital heart defects can be accurately imaged by noninvasive methods.