Adult congenital heart disease in Greece: Preliminary data from the CHALLENGE registry.

BACKGROUND: The majority of patients with congenital heart disease (CHD), nowadays, survives into adulthood and is faced with long-term complications. We aimed to study the basic demographic and clinical characteristics of adult patients with congenital heart disease (ACHD) in Greece. METHODS: A registry named CHALLENGE (Adult Congenital Heart Disease Registry. A registry from Hellenic Cardiology Society) was initiated in January 2012. Patients with structural CHD older than 16years old were enrolled by 16 specialized centers nationwide. RESULTS: Out of a population of 2115 patients with ACHD, who have been registered, (mean age 38years (SD 16), 52% women), 47% were classified as suffering from mild, 37% from moderate and 15% from severe ACHD. Atrial septal defect (ASD) was the most prevalent diagnosis (33%). The vast majority of ACHD patients (92%) was asymptomatic or mildly symptomatic (NYHA class I/II). The most symptomatic patients were suffering from an ASD, most often the elderly or those under targeted therapy for pulmonary arterial hypertension. Elderly patients (>60years old) accounted for 12% of the ACHD population. Half of patients had undergone at least one open-heart surgery, while 39% were under cardiac medications (15% under antiarrhythmic drugs, 16% under anticoagulants, 16% under medications for heart failure and 4% under targeted therapy for pulmonary arterial hypertension). CONCLUSIONS: ACHD patients are an emerging patient population and national prospective registries such as CHALLENGE are of unique importance in order to identify the ongoing needs of these patients and match them with the appropriate resource allocation.

Valve repair using biodegradable ring annuloplasty: from bench to long-term clinical results.

  Annulus remodeling and stabilization with a ring is a necessary step in mitral and tricuspid valve repair to maintain effective leaflet coaptation and improve long-term results. Although conventional rings meet the basic needs of adults, they do not preserve the changes in shape and size occurring during the cardiac cycle, and do not allow growth of the native annulus in children. The bioring annuloplasty ring was developed to allow for annular stabilization, while remaining biodegradable and allowing for growth. It is a curved "C" segment of poly-1,4-dioxanone polymer located on a non-degradable polyvinyl monofilament suture equipped with a stainless steel needle at each extremity. This ring is inserted subendocardially directly into the mitral or tricuspid annulus, away from blood contact. Animal model experiments have shown that it degrades within 12 months of implantation and is replaced by fibrous tissue, which stabilizes the annulus durably, while allowing for annular growth in children. We review the published data, from bench to bedside, as well as the early, mid and long-term clinical outcomes using the biodegradable ring, which shows that biodegradable rings remodel the annulus, reinforce the repair, restore the function of the atrioventricular valve and maintain the three dimensional dynamic motion and geometry of the mitral and tricuspid valves annulus. Growth potential is preserved in children. The mid- and long-term results showed that degradation of the device occurred without negative observable consequences.

[Abdominal aortic aneurysms: diagnosis and endovascular therapy]. / Anévrismes de l'aorte abdominale: connaissances actuelles et traitement endovasculaire.

Abdominal aortic aneurysms are a common condition whose prognosis without treatment is poor because of the lethality in case of breakage. An early diagnosis is thus necessary by screening or symptoms recognition. Surgery is not the only option then, continuous monitoring and medical treatment being sometimes possible. If curative treatment is undertaken, endovascular technique is an alternative to open surgical repair but its indications are not yet fully established and endovascular grafts have not the same longevity as open surgery. A review of the indications for endovascular repair in patients operated at the Geneva University Hospital has been used to illustrate the importance of individualized care for each patient.

[Selection and preoperative follow-up of heart transplantation candidates in the French part of Switzerland]. / Sélection et suivi prétransplantation des patients candidats à la greffe cardiaque en Suisse romande.

Heart transplantation (HTx) started in 1987 at two university hospitals (CHUV, HUG) in the western part of Switzerland, with 223 HTx performed at the CHUV until December 2010. Between 1987 and 2003, 106 HTx were realized at the HUG resulting in a total of 329 HTx in the western part of Switzerland. After the relocation of organ transplantation activity in the western part of Switzerland in 2003, the surgical part and the early postoperative care of HTx remained limited to the CHUV. However, every other HTx activity are pursued at the two university hospitals (CHUV, HUG). This article summarizes the actual protocols for selection and pre-transplant follow-up of HTx candidates in the western part of Switzerland, permitting a uniform structure of pretransplant follow-up in the western part of Switzerland.

Novel approach to mitral valve repair in childhood dilated cardiomyopathy.

Evidence is mounting that mitral valve repair can improve symptoms in adults with dilated cardiomyopathy. Data is currently lacking for children with dilated cardiomyopathy and options for annuloplasty are limited in children. We report on the successful management of a 21 month-old child in heart failure from dilated cardiomyopathy and severe mitral regurgitation. The echocardiogram showed severely dilated left heart cavities, severe mitral regurgitation from a dilated annulus (23 mm, Z-score 1.74) with discrete anterior leaflet tethering, and moderate systolic dysfunction. The mitral valve was repaired using a 16 mm Bioring Kalangos biodegradable annuloplasty ring. The patient was extubated on the third postoperative hour and discharged on the fifth postoperative day with trivial mitral regurgitation and a 5 mmHg mean transvalvular gradient. At 12 months, the patient is in NYHA class I and presents trivial central mitral regurgitation without any transmitral gradient. This represents the first report in successfully managing a child with dilated cardiomyopathy with mitral regurgitation using a novel biodegradable annuloplasty ring, which has the potential to durably remodel the mitral annulus and grow with the patient.

Primary cardiac lymphoma causing right atrial occlusion.

Primary cardiac lymphomas (PCL) are extremely rare, and diffuse large B-cell lymphoma is a highly aggressive subtype. We report a case that was initially diagnosed as chronic right heart dysfunction. Detailed investigations revealed a large lobulated tumour occluding the right atrium, infiltrating the inter-atrial septum, the roof of the left atrium, and the aortic root. Despite adequate surgical debulking and initial successful tricuspid annuloplasty, the patient succumbed to multi-organ failure. Pathological analysis of the resected tumour confirmed a diffuse large B-cell lymphoma with a proliferation rate of 100%. What is unique about this case is the size of this rare cardiac tumour, which we believe to be one of the largest described in the literature for a purely intra-cardiac PCL, its aggressive growth rate, and the relatively mild symptomatology until a late stage of the disease.

Complex aortic coarctation, regurgitant bicuspid aortic valve with VSD and ventricular non-compaction: a challenging combination.

Left ventricular non-compaction is a myocardial disorder characterized by excessive trabeculations and deep recesses that communicate with the ventricular cavity, which is thought to result from a failure of the trabecular regression that occurs during normal embryonic development. It carries a high mortality from heart failure or sudden cardiac death. A 15-year-old female patient was referred to our institution for moderate symptoms of heart failure. Echocardiography and MRI showed a bicuspid aortic valve with severe regurgitation, subaortic VSD, dilated left ventricle and left ventricular non-compaction with a moderately decreased ejection fraction, as well as isthmic coarctation and transverse arch hypoplasia. We elected to perform transaortic VSD closure and aortic valve replacement using a mechanical prosthetic valve on an arrested heart, and to address aortic coarctation and transverse arch hypoplasia using an extra-anatomic ascending-to-descending aorta bypass. Aortic cross-clamping was limited to 41 minutes. The postoperative recovery was rapid and the girl was discharged in NYHA class I with an estimated LVEF of 39%. Although management must be individualized, extra-anatomic bypass is a good single-stage approach for patients with complex coarctation and concomitant cardiovascular or myocardial disorders, reducing ischemic time and offering a better chance of successful weaning from cardiopulmonary bypass.

Respiratory distress after surgery of RVOT pathologies: a word of caution on pseudoaneurysm development.

Pseudoaneurysm of the right ventricle outflow tract (RVOT) is a rare complication in pediatric cardiac surgery. We report a patient who developed a right ventricular pseudoaneurysm 8 months after RVOT enlargement using a pericardial patch for infundibular pulmonary stenosis. Our patient was born with severe pulmonary valvular stenosis and treated with percutaneous balloon valvotomy in the neonatal period. Six months later, she developed infundibular pulmonary stenosis, which required surgical resection of right ventricle infundibular trabeculations and bovine pericardial patch enlargement. The postoperative period was normal. She was readmitted to hospital 5 months later complaining of wheezing, coughing and shortness of breath. Echocardiography showed a huge aneurysmal dilatation of the outflow patch in connection with the right ventricular cavity. The patient underwent resection of the pseudoaneurysm and former patch, followed by interposition of a bovine jugular vein conduit between the RVOT and pulmonary bifurcation. The early postoperative period was uncomplicated. On echocardiography, no significant residual gradient was measured through the conduit and there was no insufficiency of the valve. RVOT reconstruction with patch enlargement, homograft or conduit implantation can be the origin of pseudoaneurysms. Although their incidence is rare, they are often asymptomatic before becoming quite large and causing compression symptoms as in our patient with respiratory complaints due to airway compression. It is important to follow up these patients closely, especially in the first year after surgery since most aneurysms develop within 6 months of surgery.

Arteria lusoria: developmental anatomy, clinical, radiological and surgical aspects.

The left aortic arch with an aberrant right subclavian artery, or arteria lusoria, is the most common aortic arch anomaly, occuring in 0.5-2.5% of individuals. Four vessels arise sequentially from the aortic arch: the right common carotid artery, the left common carotid artery, the left subclavian artery and the aberrant right subclavian artery, which crosses upwards and to the right in the posterior mediastinum. It results from a disruption in the complex remodelling of the paired branchial arches, typically of the right dorsal aorta distal to the sixth cervical intersegmental artery. The diagnosis and differentiation of arch anomalies is based on findings at chest radiography in association with those at esophagography. It is usually asymptomatic. When symptomatic, it produces dysphagia lusoria or dyspnea and chronic coughing. Treatment is indicated for symptomatic relief of dysphagia lusoria and for prevention of complications due to aneurysmal dilatation.

Biodegradable mitral annuloplasty for congenital ischemic mitral regurgitation.

An anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is associated with ischemic mitral regurgitation through a combination of papillary muscle ischemia or permanent fibrosis, left ventricular free wall ischemic dyskinesis and left ventricular dilatation. We report the successful management of a 27-month-old girl with ALCAPA and severe mitral regurgitation using a biodegradable mitral annuloplasty ring. It could represent a novel tool for mitral valve repair in ALCAPA-associated ischemic mitral regurgitation.

Vascular ultrasound studies for the non-invasive assessment of vascular flow and patency in experimental surgery in the pig.

Vascular ultrasound is a reliable non-invasive tool used for the routine assessment of vascular flow and patency in human recipients. We describe the use at three different time points (immediately, 1 week and 4 weeks postsurgery) of ultrasound studies and its validation by angiographic studies in 37 swine undergoing carotid graft replacement. We calculated predictive values (>92%), sensitivity (>85%) and specificity (>92%) with high results at all time points. Ultrasound appeared as an accessible non-invasive technique, providing rapid, safe, repeatable and reliable results. It is an excellent alternative to angiography, avoiding risks inherent to invasive methods and therefore contributing to animal welfare.

[Monitoring and adjustment of immunosuppression after heart transplantation]. / Suivi du patient après transplantation cardiaque: monitoring et adaptation de l'immunosuppression.

Heart transplantation remains the best therapeutic option for the treatment of end-stage heart failure. However, good survival rates can be obtained only if patients are closely monitored, particularly for their immunosuppressive regimens. Currently, a triple-drug regimen usually based on calcineurin-inhibitors (cyclosporin A or tacrolimus), anti-proliferative agents and steroids is used in most recipients. New agents such as the mTOR inhibitors, a more recently developed class of immunosuppressive drugs, can also be used in some patients. The aim of this article is to review currently used immunosuppressive regimens after heart transplantation, and to propose some individualized options depending on specific patient characteristics and recent pharmacological developments in the field.

Use of extracorporeal circulation and selective renal perfusion during the surgical correction of abdominal aortic coarctation.

Coarctation of the abdominal aorta is a rare pathology. Stenosis of visceral and renal arteries may present together with coarctation, which requires specific operation techniques. We present the case of a patient with coarctation of the abdominal aorta associated with stenosis of the celiac trunk, the superior mesenteric and the right renal arteries. Distal aortic perfusion by extracorporeal circulation and selective right renal perfusion techniques were used during the operation to protect the spinal cord and kidney against hypoperfusion and ischemia.

Matching the diameter of ePTFE bypass prosthesis with a native artery improves neoendothelialization.

BACKGROUND AND AIM: The undersizing of the bypass graft diameter compared to native artery changes blood flow characteristics and velocity which may affect conduit neo-endothelialization, intimal hyperplasia reaction and patency. The aim of this study was to evaluate conduit neoendothelialization, intimal hyperplasia reaction and patency results between undersized and matched ePTFE grafts. MATERIAL AND METHODS: In 16 male Sprague-Dawley rats, undersized (1-mm internal diameter) and matched (2-mm internal diameter) ePTFE grafts were anastomosed end-to-end in the infrarenal abdominal aorta. Blood flow volume per minute was measured and wall shear stress was calculated for each group. After 3 weeks of follow-up, angiography was performed via the left carotid artery just before sacrifice. Conduit neoendothelialization and intimal hyperplasia reaction were measured by computer-assisted morphometry. RESULTS: Wall shear stress was 8 times higher for the undersized group (840.56 vs. 105.07 mPa). Three weeks after implantation, conduit neoendothelialization was better in matched grafts compared to undersized grafts (441 vs. 574 microm, p = 0.008). Intimal hyperplasia reaction was similar for both groups (8.7 vs. 6.7 microm(2)/microm for undersized and matched grafts, respectively). Patency rate was 7/8 for undersized and 8/8 for matched ePTFE grafts. CONCLUSION: Although the graft patency and the intimal hyperplasia reaction were not different between the two groups after 3 weeks, matched grafts had a significantly better endothelialization compared to undersized grafts. This short-term beneficial effect may influence long-term patency results.

Mitral tendon prolapsing into the left ventricular outflow tract.

Intracardiac masses of the mitral valve are rare. Their differential diagnosis is wide, ranging from tumors (myxomas, lipomas and fibroelastomas), thrombi and abnormal muscular or fibrous bands. We report a case and management. A 68 year-old asymptomatic female who had undergone coronary angioplasty and stent placement in the left anterior descending artery for acute myocardial infarction four years earlier, was shown to have, on routine follow-up, an intracardiac mass originating from the anterior leaflet of the mitral valve and prolapsing into the left ventricular outflow tract (LVOT). The patient underwent surgical excision of the mass under cardiopulmonary bypass, to prevent cerebral or coronary embolization and sudden death due to the highly sensitive location of the mass, in the high-velocity flow LVOT. A transverse aortotomy provided exposure of the ventricular surface of the anterior mitral leaflet and revealed a fusiform mass attached to the medial segment of the anterior leaflet, resembling a secondary cordae, measuring 20 by 3 mm. The implantation was calcified on the ventricular aspect of the anterior mitral leaflet. This mass was completely excised. Postoperative recovery was uneventful. Peroperative and postoperative transesophageal echocardiography were normal. Histological examination showed a partially necrosed and calcified fibrous tissue lined by endothelium. The final diagnosis was that of a mitral tendon. Intracardiac masses of the mitral valve are rare lesions, mostly papillary fibroelastomas and myxomas and more rarely mitral tendons, which require surgical resection for prevention of embolization. The definitive diagnosis is often only obtained on histological analysis.

Coarctation of the aorta: pre and postoperative evaluation with MRI and MR angiography; correlation with echocardiography and surgery.

AIMS: To compare MRI and MRA with Doppler-echocardiography (DE) in native and postoperative aortic coarctation, define the best MR protocol for its evaluation, compare MR with surgical findings in native coarctation. MATERIALS AND METHODS: 136 MR studies were performed in 121 patients divided in two groups: Group I, 55 preoperative; group II, 81 postoperative. In group I, all had DE and surgery was performed in 35 cases. In group II, DE was available for comparison in 71 cases. MR study comprised: spin-echo, cine, velocity-encoded cine (VEC) sequences and 3D contrast-enhanced MRA. RESULTS: In group I, diagnosis of coarctation was made by DE in 33 cases and suspicion of coarctation and/or aortic arch hypoplasia in 18 cases. Aortic arch was not well demonstrated in 3 cases and DE missed one case. There was a close correlation between VEC MRI and Doppler gradient estimates across the coarctation, between MRI aortic arch diameters and surgery but a poor correlation in isthmic measurements. In group II, DE detected a normal isthmic region in 31 out of 35 cases. Postoperative anomalies (recoarctation, aortic arch hypoplasia, kinking, pseudoaneurysm) were not demonstrated with DE in 50% of cases. CONCLUSIONS: MRI is superior to DE for pre and post-treatment evaluation of aortic coarctation. An optimal MR protocol is proposed. Internal measurement of the narrowing does not correspond to the external aspect of the surgical narrowing.

Improved neo-endothelialization of small diameter ePTFE grafts with titanium coating.

BACKGROUND: Patency of small synthetic bypass grafts is inferior compared to autologous grafts for revascularization procedures. Titanium coating of foreign surfaces has shown to decrease thrombogenicity, enhance biocompatibility and promote adhesion of endothelial cells. The aim of this study was to test the effect of titanium coating of small diameter ePTFE grafts on short term patency, neo-endothelialization and neointimal proliferation. METHODS: Bilateral carotid graft interposition was performed in 5 pigs with uncoated (n=5) and titanium-coated (n=5) ePTFE grafts (internal diameter=4 mm, length=5 cm), thus each pig served as its own control. At the end of the study (30 +/- 3 days), patency and stenosis severity was assessed by carotid angiography. Animals were sacrificed and grafts were excised for histology and scanning electron microscopy. Morphometry of histologic sections was carried out to determine neointimal proliferation and percentage of neo-endothelial coverage. RESULTS: Patency rate was 80% for uncoated and titanium-coated grafts. Quantitative angiography did not show any significant difference in lumen size between two groups. Morphometry revealed a significantly higher cellular coverage with CD31 positive endothelial cells for titanium-coated (84 +/- 19%) than uncoated grafts (48 +/- 26%, p<0.001). There was a non significant trend (p=0.112) towards increased neointimal proliferation in titanium-coated (94 +/- 61 micron2/micron) compared to uncoated grafts (60 +/- 57 micron2/micron). CONCLUSIONS: Patency rate in uncoated and titanium-coated ePTFE grafts is similar at one month. However, titanium coated grafts show a significant improvement in neo-endothelialization compared to uncoated grafts.

PPS-PEG surface coating to reduce thrombogenicity of small diameter ePTFE vascular grafts.

AIMS: Patency failure of small vascular synthetic grafts is still a major problem for coronary and peripheral revascularization. Thus, three new surface coatings of small synthetic grafts were tested in an acute pig model to evaluate their thrombogenicity (extracorporeal arterio-venous shunt) and in a chronic rat model to evaluate the tissue reaction they induced (subcutaneous implantation). METHODS: In five domestic pigs (25-30 kg) an extracorporeal femoro-femoral arterio-venous shunt model was used. The study protocol included first a non-heparinized perfusion sequence followed by graft perfusion after 10,000 UI iv heparin. Grafts were perfused for 3 and 9 minutes. The following coatings were tested on ePTFE grafts: poly-propylene sulphide (PPS)--poly-ethylene glycol (PEG) (wet and dry applications) as well as carbon. Two sets of control were used, one dry and one wet (vehicle only). After perfusion grafts were examined by scanning electron microscopy for semi-quantitative assessment (score 0-3) of cellular and microthrombi deposition. To assess tissue compatibility, pieces of each material were implanted subcutaneously in 16 Wistar rats. At 2, 4, 8, 12 weeks four animals each were sacrificed for semi-quantitative (score 0-3) histologic evaluation of tissue reaction. RESULTS: In the pig model, cellular deposition and microthrombi formation increased over time. In non- heparinized animals, the coatings did not improve the surface characteristics, since they did not prevent microthrombi formation and cellular deposition. In heparinized animals, thrombogenicity was lowest in coated grafts,especially in PPS -PEG dry (p<0.05), and highest in controls. Cell deposition was lowest in PPS-PEG dry, but this difference was not statistically significant vs.controls. In the rat model,no significant differences of the tissue reaction could be shown between materials. CONCLUSION: While all coatings failed to add any benefit for lowering tissue reaction, surface coating with PPS -PEG (dry application) reduced thrombogenicity significantly (in heparinized animals) and thus appears to be promising for improving graft patency of small synthetic vascular prostheses.

Autologous fibrin glue reinforced by platelets in surgery of ascending aorta.

OBJECTIVES: Fibrin sealants are popular for the improvement of perioperative hemostasis and reducing blood transfusion needs. Biological glues prepared from pooled human donor plasma have an inherent risk of transmission of blood-borne disease and are quite expensive to use. A system for the production of autologous fibrin sealant re-enforced by platelets has been developed. Its efficacy, safety and economic benefits have been evaluated in a prospective, randomized study. MATERIAL: 20 consecutive patients undergoing replacement of the ascending aorta by the same surgical team had local application of either Tissucol (Group A) or autologous fibrin glue (Group B) for hemostasis. RESULTS: No adverse effects of either glue were recorded. The volume of produced autologous fibrin glue was 25 cc PRP. Platelet yield was 72 %. The two groups were comparable. Efficacy was group comparable. Average cost for sealants in Group A was 470 +/- 100 Euros compared to 273 Euros in Group B, p = 0.004. CONCLUSIONS: Autologous fibrin glue re-enforced by autologous platelets can be safely produced in the operating room in a large volume, with an comparable efficacy at a lower cost than commercial sealants.