Pregnancy outcomes in women with a systemic right ventricle and transposition of the great arteries results from the ESC-EORP Registry of Pregnancy and Cardiac disease (ROPAC).

OBJECTIVE: Cardiac disease is a major cause of maternal mortality. Data regarding pregnancy outcomes in women with a systemic right ventricle (sRV) are scarce. We studied pregnancy outcomes in women with an sRV after the atrial switch procedure for transposition of the great arteries (TGA) or congenitally corrected TGA (CCTGA). METHODS: The ESC EORP Registry of Pregnancy and Cardiac Disease is an international prospective registry of pregnant women with cardiac disease. Pregnancy outcomes (maternal/fetal) in all women with an sRV are described. The primary end point was a major adverse cardiac event (MACE) defined as maternal death, supraventricular or ventricular arrhythmias requiring treatment, heart failure, aortic dissection, endocarditis, ischaemic coronary event and other thromboembolic events. RESULTS: Altogether, 162 women with an sRV (TGA n=121, CCTGA n=41, mean age 28.8±4.6 years) were included. No maternal mortality occurred. In 26 women, at least one MACE occurred, heart failure in 16 (9.8%), arrhythmias (atrial 5, ventricular 6) in 11 (6.7%) and others in 4 (2.5%). Prepregnancy signs of heart failure as well as an sRV ejection fraction <40% were predictors of MACE. One woman experienced fetal loss, while no neonatal mortality was observed. No significant differences were found between women with CCTGA and TGA. In the subset of women who had an echocardiogram before and after pregnancy, no clear deterioration in sRV was observed. CONCLUSION: The majority of women with an sRV tolerated pregnancy well with a favourable maternal and fetal outcome. Heart failure and arrhythmias were the most common MACE.

Global and regional right ventricular mechanics in repaired tetralogy of Fallot with chronic severe pulmonary regurgitation: a three-dimensional echocardiography study.

BACKGROUND: Data about the right ventricular (RV) mechanics adaptation to volume overload in patients with repaired tetralogy of Fallot (rToF) are limited. Accordingly, we sought to assess the mechanics of the functional remodeling occurring in the RV of rToF with severe pulmonary regurgitation. METHODS: We used three-dimensional transthoracic echocardiography (3DTE) to obtain RV data sets from 33 rToF patients and 30 age- and sex- matched controls. A 3D mesh model of the RV was generated, and RV global and regional longitudinal (LS) and circumferential (CS) strain components, and the relative contribution of longitudinal (LEF), radial (REF) and anteroposterior (AEF) wall motion to global RV ejection fraction (RVEF) were computed using the ReVISION method. RESULTS: Corresponding to decreased global RVEF (45 ± 6% vs 55 ± 5%, p < 0.0001), rToF patients demonstrated lower absolute values of LEF (17 ± 4 vs 28 ± 4), REF (20 ± 5 vs 25 ± 4) and AEF (17 ± 5 vs 21 ± 4) than controls (p < 0.01). However, only the relative contribution of LEF to global RVEF (0.39 ± 0.09 vs 0.52 ± 0.05, p < 0.0001) was significantly decreased in rToF, whereas the contribution of REF (0.45 ± 0.08 vs 0.46 ± 0.04, p > 0.05) and AEF (0.38 ± 0.09 vs 0.39 ± 0.04, p > 0.05) to global RVEF was similar to controls. Accordingly, rToF patients showed lower 3D RV global LS (-16.94 ± 2.9 vs -23.22 ± 2.9, p < 0.0001) and CS (-19.79 ± 3.3 vs -22.81 ± 3.5, p < 0.01) than controls. However, looking at the regional RV deformation, the 3D CS was lower in rToF than in controls only in the basal RV free-wall segment (p < 0.01). 3D RV LS was reduced in all RV free-wall segments in rToF (p < 0.0001), but similar to controls in the septum (p > 0.05). CONCLUSIONS: 3DTE allows a quantitative evaluation of the mechanics of global RVEF. In rToF with chronic volume overload, the relative contribution of the longitudinal shortening to global RVEF is affected more than either the radial or the anteroposterior components.

Incidence and predictors of obstetric and fetal complications in women with structural heart disease.

OBJECTIVE: Women with cardiac disease becoming pregnant have an increased risk of obstetric and fetal events. The aim of this study was to study the incidence of events, to validate the modified WHO (mWHO) risk classification and to search for event-specific predictors. METHODS: The Registry Of Pregnancy And Cardiac disease is a worldwide ongoing prospective registry that has enrolled 2742 pregnancies in women with known cardiac disease (mainly congenital and valvular disease) before pregnancy, from January 2008 up to April 2014. RESULTS: Mean age was 28.2±5.5 years, 45% were nulliparous and 33.3% came from emerging countries. Obstetric events occurred in 231 pregnancies (8.4%). Fetal events occurred in 651 pregnancies (23.7%). The mWHO classification performed poorly in predicting obstetric (c-statistic=0.601) and fetal events (c-statistic=0.561). In multivariable analysis, aortic valve disease was associated with pre-eclampsia (OR=2.6, 95%CI=1.3 to 5.5). Congenital heart disease (CHD) was associated with spontaneous preterm birth (OR=1.8, 95%CI=1.2 to 2.7). Complex CHD was associated with small-for-gestational-age neonates (OR=2.3, 95%CI=1.5 to 3.5). Multiple gestation was the strongest predictor of fetal events: fetal/neonatal death (OR=6.4, 95%CI=2.5 to 16), spontaneous preterm birth (OR=5.3, 95%CI=2.5 to 11) and small-for-gestational age (OR=5.0, 95%CI=2.5 to 9.8). CONCLUSION: The mWHO classification is not suitable for prediction of obstetric and fetal events in women with cardiac disease. Maternal complex CHD was independently associated with fetal growth restriction and aortic valve disease with pre-eclampsia, potentially offering an insight into the pathophysiology of these pregnancy complications. The increased rates of adverse obstetric and fetal outcomes in women with pre-existing heart disease should be highlighted during counselling.

[Percutaneous closure of patent foramen ovale, as secondary prevention of cryptogenic cerebral event]. / Nyitott foramen ovale katéteres záráisa, a cryptogen agyi történés szekunder prevenciója.

In patients with patent foramen ovale and cryptogenic stroke, the risk of recurrent event is about 4-5% yearly, despite of the antithrombotic treatment. Transcatheter closure of patent foramen ovale has been shown to decrease risk of recurrence. This report summarizes the first experiences in Hungary and the medium term follow up of our patients. Thirty-three patients were selected for closure. All the patients had at least one ischemic stroke, transitory ischemic attack or peripheral embolus. In 31 of them the stroke was confirmed by computer tomographic or magnetic resonance imaging. Patent foramen ovale with right-to-left shunt was diagnosed by transesophageal echocardiography. Transcatheter closure was performed under fluoroscopic and transesophageal echocardiographic control. Closure was successful in 31 patients. Passage of atrial septum did not succeed in two cases. An AV fistula at puncture site was the only early complication. There was no late complication. No device dislocation, periprocedural arrhythmia or embolic event was detected. No residual shunt could be found. During follow up of 11.3 (1-30) months no recurrent embolus was detected. Transcatheter closure of patent foramen ovale is a safe and efficient procedure to decrease the risk of recurrent ischemic events.

[Adult congenital heart program in a tertiary care facility of pediatric cardiology]. / Felnottkori congenitalis szívsebészet gyermekkardiológiai és szívsebészeti központban.

INTRODUCTION: Owing to excellent survival following primary repair over 80% of congenital cardiac patients reach adulthood, half of them requiring continuous specialist care and one-third needing further reoperation. The ample variety and complexity of lesions warrant individualised treatment strategy. OBJECTIVE: This study focuses on grown-up congential heart (GUCH) programme in the settings of a tertiary pediatric cardiac centre. METHODS: Patients underwent corrective surgical procedures in pediatric facilities (theatre, ICU, wards) with a close involvement of adult cardiology/anaesthetic team. Patients were divided into simple/complex groups. RESULTS: Simple group of comprised patients (n = 20) having ASD-II (18/20) sinus venosus ASD (2/20) repair without morbidity/mortality from right subaxillary thoracotomy in 17/20. Complex group (n = 20): corrective surgery for tetralogy of Fallot (6), LVOT-aortic valve repairs (4), allograft conduit exchange (3), TCPC (3), miscellaneous procedures (4) were performed as reoperations in 16/20 at 16.1 +/- 8.1 years following previous operations (median: 1.96, range 1-4). Postoperative right ventricle- (1), acute renal (1) failure and ARDS (1) fully recovered. One patient was lost for multi-organ-failure due to low cardiac output syndrome caused by chronic RV failure. Preoperative cyanosis was a risk factor for postoperative complications (p = 0.01). All survivors are symptom-free. No significant difference in ITU stay/LOS was observed between simple and complex groups. CONCLUSIONS: This study represents the initial experience of authors with GUCH. The number of GUCH patients is expected to rise with an upgrade shift in surgical complexity and severity requiring a multidisciplinary approach. It is advocated that complex GUCH cases should be performed by teams experienced in congenital reconstructive surgery. Patients following complex GUCH procedures have a comparable hospital course to patients undergoing simple cardiac operations.