Poor outcome for patients with totally anomalous pulmonary venous connection and functionally single ventricle.

Totally anomalous pulmonary venous connection, when also associated with a functionally univentricular connection, is known to have a poor outcome. We retrospectively analysed results for 19 patients undergoing surgery for this combination of lesions between 1995 and February 2009.Of the patients, 12 were neonates, with 11 presenting with signs of pulmonary venous obstruction. In 3 patients, a modified Blalock-Taussig shunt had been constructed. The dominant ventricle was of right ventricular morphology in 17 of the 19 patients, and double inlet was present in all bar 1. Pulmonary atresia or stenosis was found in 14 patients, a common atrioventricular junction in 14 patients, and isomerism of the right atrial appendages in 12 patients, respectively. Comprehensive Aristotle scores ranged from 14 to 23.50. The mean was 16.55, with a standard deviation of 2.19. Pulmonary venous rerouting was combined in 6 patients with construction of a modified Blalock-Taussig, in 4 with banding of the pulmonary trunk, in another 4 with a bidirectional Glenn anastomosis, in 3 with creation of a total cavo-pulmonary connection, and in 1 each with enlargement of the right ventricular outflow tract and the Norwood procedure. Of the cohort, 8 patients died early due to pulmonary hypertension, with all patients having Aristotle scores of at least 18 points dying. Among the 11 early survivors, 5 needed mechanical ventilation for over a week, but 5 patients died later, 3 due to pulmonary hypertension and 2 due to infection. Actuarial survival stabilized at 31.6%, with standard error of 10.7%, from one year onwards. Conversion to the Fontan circulation was successful in 5 patients.Our experience confirms that totally anomalous pulmonary venous connection, when associated with the functionally univentricular arrangement, carries one of the worst outcomes in current surgical practice. Use of the Aristotle comprehensive complexity scores effectively discriminates those patients with this condition at particularly high risk.

Amniotic air insufflation during minimally invasive fetoscopic fetal cardiac interventions is safe for the fetal brain in sheep.

BACKGROUND: Amniotic air insufflation during experimental fetoscopic fetal cardiac interventions greatly improves the visualization of intra-amniotic contents. The purpose of this study was to assess any histologically discernible effects from this approach on the fetal brain after short-term studies and long-term survival in sheep. METHODS: Thirty pregnant ewes between 80 and 110 days of gestation underwent amniotic air insufflation during various fetoscopic fetal cardiac interventions. After 18 short-term and 12 long-term studies, the brains of the operated fetuses and-if available-their unoperated siblings were examined for hemorrhage, embolism, infarctions, inflammatory changes, and abnormal cortical maturation. RESULTS: Amniotic air insufflation during minimally invasive fetoscopic fetal cardiac interventions did not result in any histologically discernible damage to the brain in short-term and long-term studies in any but 2 sibling sheep. In the 2 affected siblings, a small area of chronic cortical frontal lobe infarction was observed after long-term survival. CONCLUSIONS: Amniotic air insufflation during minimally invasive percutaneous fetoscopic fetal cardiac interventions is safe for the fetal brain and does not compromise maternal hemodynamics in sheep. These findings encourage further investigation of the role this technique might play during fetoscopic fetal cardiac interventions in humans.

Assessment of subacute inflammatory and proliferative response to coronary stenting in a porcine model by local gene expression studies and histomorphometry.

The aim of the study was to analyse inflammatory and proliferative response early after coronary stenting by angiography, histomorphometry and local gene expression analysis using quantitative rt-PCR. Therefore, eight German domestic pigs underwent stenting of the left coronary artery. Selective coronary angiography was performed after 14 days. Explanted coronary arteries were examined histomorphometrically after methacrylate-embedding. Snap-frozen samples were examined for local gene expression of TGF-beta, TNF-alpha, GM-CSF, VEGF, PDGF and Fas Ligand (FasL) by real-time quantitative rt-PCR normalized to the housekeeping gene GAPDH and compared to unstented coronary arteries. All stented coronaries were patent with only little neointima formation. The median vessel diameter was 2.55 mm (range 2.43-2.68 mm). Histopathology revealed little inflammatory response limited to the tissue surrounding the stent struts; luminal area ranged from 84% to 91%. Compared to unstented control arteries, no significant differences in local gene expression were detected for VEGF, PDGF, TGF-beta, TNF-alpha and GM-CSF. Expression of FasL was upregulated as little as 1.7-fold (p=0.01). We conclude that, in native coronary arteries, no significant upregulation of investigated genes regulating vascular remodelling, inflammation or fibrogenesis was demonstrated 14 days after stenting. Whether upregulation of FasL as a marker gene of apoptosis is transient and biological significant requires further investigation.

Transcatheter closure of a multiperforated atrial septal defect extending from the oval fossa to the mouth of the inferior caval vein.

We report a novel technique using an Amplatzer atrial septal occluder to close a defect located in the inferior-posterior portion of the interatrial septum that extended into the mouth of the inferior caval vein. Because of the close relation of the defect to the inferior caval vein, the right atrial disc was opened into the inferior caval vein and pushed toward the right atrium by use of the delivery cable. There was no residual shunting immediately and 3 months after the intervention. We conclude that even defects located infero-posteriorly within the oval fossa may be successfully closed by transcatheter techniques using the Amplatzer device.

Secondary embolization of a Helex occluder implanted into a secundum atrial septal defect.

We report on a 2-year-old patient with atrial septal defect with a stretched diameter of 11 mm. The defect was closed uneventfully by use of a 25 mm Helex device. There was only trivial residual shunting (1 mm at the cranial margin of the device). After 36 hr, routine postinterventional echocardiography confirmed device embolization into the pulmonary artery. The child was asymptomatic. Transcatheter device retrieval with snares of different sizes, bioptomes, and retrieval forceps failed due to the mismatch of the diameter of the device and the small diameter of the pulmonary artery. Therefore, the device was retrieved surgically and the ASD closed by primary sutures. Secondary embolization of a Helex device complicated the closure of an uncomplicated atrial septal defect with a device-to-defect ratio according to the manufacturer's suggestion due to a mechanism not yet understood.

[Interventional therapy of large veno-venous collaterals after univentricular palliation for congenital heart disease using the Starflex- and Amplatzer- PDA occluder]. / Interventioneller Verschluss von grosslumigen veno-venösen Kollateralen nach univentrikulärer Palliation angeborener Herzfehler mittels Starflex- und Amplatzer-PDA-Occluder.

UNLABELLED: Arterial hypoxemia is frequently observed after Fontan-like palliation of congenital heart defects. Whereas small systemic-to-pulmonary venous collaterals can easily be occluded by use of metal spirals, large collateral vessels may be therapeutically challenging. We report on two patients with arterial hypoxemia (transcutaneous oxygen saturations 72% and 82%, respectively) after Fontan-like operation. One patient was diagnosed with double inlet left ventricle, malposition of the great arteries and subaortic obstruction; another patient was diagnosed with mitral atresia, double-outlet right ventricle, malposition of the great arteries and hemiazygos continuity. In the first patient, a large venous collateral (measuring 16 mm in diameter) was observed connecting the superior vena cava and the right upper pulmonary vein. In the latter patient, hepatic fistulas drained systemic venous blood into an excluded hepatic vein. There was an associated patent fenestration of the conduit connecting the hepatic veins to the pulmonary artery. After occlusion of the supracardiac venous collateral with a 33 mm Starflex device, arterial oxxgen saturation rose to levels above 95%. The excluded hepatic vein was occluded with of an 10/8 mm Amplatzer PDA occluder and the fenestration was closed with a 17 mm Starflex device. Arterial oxygen saturations rose to 98%. CONCLUSION: Occlusion of large collateral vessels after Fontan-like palliation of congenital heart defects can be effectively performed using the Amplatzer PDA device and the Starflex device. Different occlusion devices are needed to address the morphological differences of the collateral vessels.