Adeline Basquin

Outcomes and safety of transcatheter pulmonary valve replacement in patients with large patched right ventricular outflow tracts

BACKGROUND Although globally accepted, the indication for implantation of the Melody(®) (Medtronic Inc., Minneapolis, MN, USA) transcatheter pulmonary valve is limited to the treatment of haemodynamically dysfunctional right ventricular outflow tract (RVOT) with right ventricle to pulmonary artery (PA) obstruction. The use of the Melody valve for haemodynamically significant isolated pulmonary regurgitation has not been evaluated. AIM We evaluated the outcomes of Melody valve insertion in patients with a large patched RVOT. METHODS We analysed procedural and short-term outcomes data from 13 patients who underwent Melody valve implantation for a large RVOT with significant pulmonary regurgitation as the primary lesion. RVOT preparation was done in all patients using the Russian dolls technique and/or the PA jailing technique. Melody valve insertion was performed concomitantly in 10 patients and after 1 to 3 months in three patients. RESULTS All procedures were successful. The mean follow-up period was 30 ± 4 months after the procedure. There was no incidence of stent fracture, migration or embolization. Only one patient who underwent the jailing technique developed a significant paraprosthetic leak and is scheduled for redilatation of the Melody valve. CONCLUSIONS Careful patient selection, balloon sizing and RVOT preparation with prestenting using the Russian dolls technique and/or the PA jailing technique are required to modify the RVOT for transcatheter valve implantation. Short-term follow-up showed competent valves with no stent fracture or migration and appears promising. Wider experience with long-term outcomes may be required to standardize the procedure in such a subset of patients.

Off-Pump Replacement of the Pulmonary Valve in Large Right Ventricular Outflow Tracts: A Transcatheter Approach Using an Intravascular Infundibulum Reducer

We report our experience of pulmonary valve replacement in animals with large right ventricular outflow tracts (RVOTs) using a percutaneous approach. We intended to implant a device to percutaneously reduce the diameter of the pulmonary artery (PA). Following its insertion, we intended to implant a valved stent inside the restriction. Animals were killed acutely (group 1, n = 6) and after a mean follow-up of 1 (group 2, n = 3) and 2 mo (group 3, n = 9). In group 1, all reducers were successfully deployed and allowed the reduction of the PA to a diameter of 12-mm. In one animal, the proximal part of the reducer did not reach its final configuration. Another reducer embolized when manipulating the stiff wire. The insertion of valves was therefore possible in 17/18 animals. One animal died from an arrhythmia during positioning of the valve. Angiographic evaluations showed no leak between devices and the pulmonary wall. During the follow-up, there was no device migration. At autopsy, reducers were fixed to the pulmonary wall and completely covered by a fibrous tissue. In conclusion, with the use of an intravascular reducer, implantation of a pulmonary valve is possible in sheep through a transcatheter approach when the RVOT exceeds 22-mm in diameter.

Transcatheter valve insertion in a model of enlarged right ventricular outflow tracts

OBJECTIVE Transcatheter pulmonary valve insertion has recently emerged as an alternative to surgery. To extend its indications to patients with a large right ventricular outflow tract, we previously developed an intravascular device that reduces the diameter of the main pulmonary artery, allowing the insertion of available valved stents. Here we report its use in a model of animals with an enlarged right ventricular outflow tract and pulmonary valve incompetence. METHODS AND RESULTS The study comprised 33 sheep that first underwent surgical enlargement of the main pulmonary artery. We then intended to implant a filler percutaneously, followed later by the insertion of a valve. Three animals died during the intermediate stage. The remainder were humanely killed either immediately (group 1, n = 6) or after a mean follow-up of 1 (group 2, n = 12) or 2 months (group 3, n = 12). Animals from groups 2 and 3 were equally divided into 2 subgroups according to the difference between diameters of the device inserted and the main pulmonary artery (A < 5 mm, B > or = 5 mm). Fillers were all inserted successfully (n = 30), although one embolized after its insertion (group 3A). A valved stent was implanted in all animals, but in 1 case a balloon ruptured during inflation of the stent leading to incomplete expansion and the death of the animal. Six animals, 5 of which were from group A, had pulmonary regurgitation after valve insertion. CONCLUSION Pulmonary valve insertion is possible through a transcatheter technique using a pulmonary artery filler. Oversizing the device reduces the risk of embolization and paraprosthetic leak.

Acquired Left Ventricular Submitral Aneurysms in the Course of Takayasu Arteritis in a Child

A 9-year-old black African boy was hospitalized for heart failure revealing a severe left ventricular dysfunction associated with dilated cardiomyopathy, two submitral aneurysms, occlusion of the circumflex artery and a giant coronary artery aneurysm on the proximal left anterior descending artery. The boy was coinfected with human immunodeficiency virus and Mycobacterium tuberculosis. Though rare, association of Takayasu arteritis and submitral aneurysm leads to rethinking the pathogenesis of submitral aneurysm and suggests that some of them may be acquired. In our case, a common inflammatory process, possibly triggered by tuberculosis or HIV, may underlie Takayasu and submitral aneurysms.

How Pregnancy Impacts Adult Cyanotic Congenital Heart Disease

Survival into adulthood of patients with unrepaired cyanotic congenital heart defects (CHDs) is possible when cyanotic CHDs are deemed unsuitable for radical surgical repair but are compatible with survival. These situations include, for example, complex pulmonary atresia with aortopulmonary collaterals and single-ventricle hearts (with and without earlier palliation). This is also the case when patients with cyanotic CHDs reach adulthood without serious symptoms requiring surgery, such as those with mild tetralogy of Fallot, Ebstein’s anomaly, and some cases of corrected transposition of the great arteries with pulmonary stenosis and ventricular septal defect. Many women with these heart conditions wish to become pregnant, which creates a situation of high maternal and fetal risks of complications.1 Management of these patients before, during, and after pregnancy has improved, with an earlier recognition of the underlying disease, improved understanding of cardiopulmonary physiopathology, better prenatal and peri-partum obstetric/anesthetic management, and the introduction of a multidisciplinary approach.2 We retrospectively reviewed the charts of all pregnant women with cyanotic CHDs (n=51) who were followed in 11 adult CHD referral …

CO 7 Outcomes after protein-losing enteropathy in univentricular hearts: A multicenter study

Suzanne Borrhomée*, Sébastien Hascoët, Alban-Elouen Baruteau, Jérôme Petit, Lucile Houyel, Marielle Gouton, Régine Roussin, Marianne Peyre, Mohammed Ly, Emre Belli, Emmanuel Lebret, Serge Demontoux, Virginie Lambert, Daniela Laux 1 Centre chirurgical Marie-Lannelongue, Pôle des cardiopathies congénitales, Centre de référence M3C des cardiopathies congénitales complexes, Le Plessis-Robinson, France.

0238: Quality of life of patients with pulmonary arterial hypertension associated with congenital heart disease: the multicenter cross-sectional Achille study

Objectives: To assess health-related quality of life (QoL) in patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) and correlations with clinical status. Methods: This cross-sectional study included PAH-CHD patients in 14 centers in France. QoL was self-reported with a generic questionnaire (SF-36) and a PH-specific questionnaire (CAMPHOR). Patients filled out the Hospital Anxiety and Depression Scale (HADS) questionnaire. Main clinical data were collected. Results: 200 patients were included (mean age 43±SD15.7 years, range 15 to 86 years, 70% female), and were classified as Eisenmenger syndromes (73.5%), PAH associated with systemic to pulmonary shunts (13%), PAH associated with small defects (3.5%) and PAH after corrective cardiac surgery (10%). At inclusion, 76.5% patients were receiving PAH-specific treatments. All component scores of both QoL questionnaires showed degradation vs healthy population standard scores. QoL scores were lower in females than in males for all dimensions of both questionnaires (p Conclusion: This study showed degradation of QoL in a large cohort of PAH-CHD patients with both generic and specific questionnaires. QoL was correlated with NYHA class but not with the type of PAH-CHD.

289: Vitamin K antagonists self management with COAGUCHEK XS® in children: an observational study

Introduction The use of COAGUCHEK XS ® (Roche Diagnostics), an INR home measuring device, was authorised by the French Health High Authority on June 2008 for children. Nevertheless, there are few data on safety and effectiveness of patients self management particularly in children. Methods Since 2008, 23 children (mean age: 9 y; 16 males, 7 females) and their families were trained to INR self testing and self management of vitamin K antagonists (VKA) (acenocoumarol: n=10, fluindione : n=9, warfarin: n=4). INR controls (self testing or lab) were made at expected dates established by the french law. Additional measurements were authorised in risky situations (illness, hyperthermia, forgetting VKA treatment, bleeding, etc…). Drug dose adaptation was made either by the family or by the health team if needed. Objectives To evaluate safety and efficacy in families performing self management in our centre. Time in therapeutic range (TTR), severe haemorrhage (needing hospitalization and/or blood transfusion) and thrombosis recurrence were observed. Results Mean follow-up time was 529 days [1.5 y; range 0.3-2.5y]. Mean TTR was 68.53% and was 90.14% when tolerance range was applied. There was no severe haemorrhage or thrombosis recurrence during follow-up and INR >5 were 0.5% (8/1604 measurements). Lastly, 3.5% additional measurements were performed by the families. Conclusion VKA self management using COAGUCHEK XS ® was safe and effective in our centre. Clinical studies with larger sample and cost-effectiveness considerations are required.