Intracardiac total anomalous pulmonary venous return or septum primum malposition? Complete repair in left atrial isomerism.

Total anomalous pulmonary venous return due to septum primum malposition is a poorly understood condition despite being very common in left atrial isomerism or polysplenia syndrome. Due to the leftward displacement of the septum primum, either the two right pulmonary veins or all four pulmonary veins can drain abnormally into the right atrium, despite their correct position. In other words, the four pulmonary veins (or the two right pulmonary veins), looking from outside the heart, return at the back of the atrium in the normal position. Nevertheless, from the inside of the heart, two or all four pulmonary veins drain into the right atrium due to the leftward displacement of the septum primum. As an example, we report a 5-month-old patient with severe malposition of the septum primum and consequent total anomalous pulmonary venous drainage into the right atrium. The patient underwent surgical correction with resection of the malpositioned septum primum and reconstruction of a normal interatrial septation with a pericardial patch.

The Norwood operation performed with the heart beating and no prosthetic materials.

The Norwood procedure performed with the heart beating is a useful and attractive way to perform the stage I palliation for hypoplastic left heart syndrome. It allows completion of the surgical procedure without stopping the heart and without using deep hypothermia and circulatory arrest. Therefore it could be an attractive solution for reducing the adverse effects of the operation and the rate of postoperative complications. Moreover, this technique avoids the use of prosthetic materials, thus minimizing long-term complications and reoperations, and promotes a more physiological growth of the anatomical structures. However, the high level of expertise required to perform this type of complex neonatal procedure with the heart beating may be limited to only a few centers in the world thus far.

Type A Aortic Dissection in an 11-month-old Infant With Loeys-Dietz Syndrome.

Type A aortic dissection is a very uncommon finding in children, especially among infants, where no cases seem to have been described. We report the case of an 11-month-old boy with Loeys-Dietz syndrome and asymptomatic type A aortic dissection involving the aortic root that was successfully repaired.

Tirofiban administration and percutaneous coronary intervention with stenting of saphenous vein graft thrombosis.

Distal embolization during percutaneous coronary intervention (PCI) of saphenous vein graft (SVG) lesions is associated with a high risk of myonecrosis and myocardial infarction. PCI guidelines advocate the use of distal embolic protection devices, when technically feasible, in patients undergoing PCI for SVG disease. To date, alternative management strategies are not fully investigated. We report a case of an 84-year-old male patient with acute coronary syndrome who underwent PCI for a quite occlusive stenosis of an SVG on the first diagonal branch of the left anterior descending artery complicated by wide endoluminal thrombosis with poor antegrade coronary blood flow and absent opacity of the distal first diagonal vessel. A strategy of delayed PCI after upstream, 48 h long tirofiban administration in order to obtain a thrombus burden reduction was decided. After tirofiban administration, a high-level thrombus resolution was obtained, with a significant improvement in coronary flow, and a successful PCI with stenting was performed. There was neither clinical nor instrumental periprocedural sign of ischemia, and the patient remained asymptomatic throughout his hospital stay. Preprocedural tirofiban administration followed by PCI with stenting of an SVG thrombotic lesion without a distal protection device might be a well-tolerated and feasible option for patients with degenerated SVG disease. Further studies are needed to further expand our findings.

Acute aortic syndromes at high surgical risk: the endovascular approach.

AIMS: Acute aortic syndromes (AAS) still represent life-threatening conditions. The aim of this study was to describe our experience in the management of patients (pts) with AAS and to evaluate the safety and feasibility of endovascular treatment (EVT) in high surgical risk patients. METHODS AND RESULTS: One hundred and four patients underwent EVT. We selected 56 pts with AAS: 17 complicated type B aortic dissections, five traumatic aortic ruptures at the isthmus, 11 thoracic aneurysms and 23 pts with large AAA with impending rupture. All these pts were at high surgical risk because of their comorbidities and/or their emergency situation. They were clinically followed during hospitalisation and they underwent a 2 mm-interval CT-scan two weeks, six and 12 months after discharge and every year after. Death, paraplegia, open surgical conversion did not occur. Two pts underwent a successful secondary EVT for type I endoleak. One patient with thoracic aortic aneurysm died of septic shock from pneumonia 78 days after discharge and two pts with AAA suffering from a severe three-vessel coronary disease experienced sudden death at one year follow-up. CONCLUSIONS: EVT seems to be a safe and effective therapeutic option with good short- and midterm results in patients with AAS at high surgical risk. Thus, it can be considered as a less-invasive alternative in patients considered otherwise unsuitable for conventional surgery, even though a careful, continued follow-up is still necessary to confirm the long-term safety and effectiveness of EVT in AAS.