Adult Congenital Heart Disease

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). Tutarel O, Baris L, Budts W, Gamal Abd-El Aziz M, Liptai C, Majdalany D, Jovanova S, Frogoudaki A, Connolly HM, Johnson MR, Maggioni AP, Hall R, Roos-Hesselink JW; ROPAC Investigators Group.Heart. 2021 Apr 28:heartjnl-2020-318685. doi: 10.1136/heartjnl-2020-318685. Online ahead of print.PMID: 33911009 Take Home Points: In women with a systemic right ventricle (sRV) after atrial switch for transposition of the great arteries (121 patients) or congenitally corrected transposition of the great arteries (ccTGA, 41 patients), in this international prospective observational registry, pregnancy was well tolerated with favourable maternal and fetal outcomes with: 26 (21%) having at least 1 major adverse cardiac event (MACE) 16 (9.8%) having heart failure needing treatment 11 (6.8%) having arrthymia (5 atrial and 6 ventricular) needing treatment 4 (2.5%) having other events No deterioration in sRV was seen in those who had pre and post pregnancy echoes Pre-pregnancy signs of heart failure and sRV ejection fraction <40% predicted MACE. Commentary from Dr Simon MacDonald (London, UK) section editor of ACHD Journal Watch: There is uncertainty about how a systemic right ventricle supports the systemic circulation and adapts to the volume changes seen in pregnancy. This is important in patients with transposition of the great arteries (TGA) who have had the atrial switch operation (Mustard or Senning operation) and those who have congenitally corrected transposition of the great arteries (ccTGA), this study helping inform management of pregnancy for these women. They used an international, prospective, observational registry of pregnant patients with cardiac disease called the European Society of Cardiology (ESC) EURObservational Research Program(EORP) Registry on Pregnancy and Cardiac disease (ROPAC). They studied all pregnancies in women with a sRV enrolled in this database from January 2007 to January 2018. Baseline characteristics were collected before pregnancy, including echocardiography where available. The primary combined endpoint taken was major adverse cardiac event (MACE), defined as maternal death, supra-ventricular or ventricular arrhythmias needing treatment, heart failure (requiring hospital admission, new or change in treatment), aortic dissection, endocarditis, ischemic coronary event and other thromboembolic events. 5739 women were in the registry, 162 had a sRV (121 post atrial switch and 41 ccTGA). Baseline characteristics are shown in table 1: Pregnancy outcomes were as in table 2: Obstetric and fetal outcomes were as in table 3. 48.5% had a caesarean section and there was 1 fetal death in a woman with TGA, none in ccTGA women. There were no maternal deaths. 26 of the 79 women having a caesarean did so for cardiac reasons, such as reported heart failure (23%) or severity of cardiac disease (34%). There were 34 premature births with 15 of these induced, 12 spontaneous and 7 unknown. This was not significantly associated with heart failure or NYHA class but was with maternal cardiac medication use (p=0.01). 17.1% of ccTGA women had low birthweight infants, 18.2% with TGA. Pre-pregnancy signs of heart failure, a RVEF <40% were predictors of MACE, being primigravida reduced the risk, as seen in figure 1: The authors conclude that the majority of women with sRV tolerate pregnancy well with low rates of MACE and without maternal or neonatal mortality. This was a large prospective study, compared to previous smaller retrospective studies. Numbers were too small for multivariate analysis and obstetric and fetal complications seemed less than that reported in other smaller studies however. Serial echo data was only available on a limited number as a caution. The authors also note that most of the women studied had TGA with palliative Mustard or Senning operation. This will change as the arterial switch operation is the predominant operation now for this condition, with the left ventricle supporting the systemic circulation, and the proportion of woman with ccTGA and systemic right ventricle will proportionately increase.

Maternal and Neonatal Outcomes of Pregnancies in Women With CongenitalHeart Disease: A Meta-Analysis. Hardee I, Wright L, McCracken C, Lawson E, Oster ME.J Am Heart Assoc. 2021 Apr 20;10(8):e017834. doi: 10.1161/JAHA.120.017834. Epub 2021 Apr 6.PMID: 33821681 Take Home Points: More severe congenital disease was associated with neonatal mortality. Commentary by Dr. Helen Parry (Leeds, UK), section editor of ACHD Journal Watch: Introduction: Survival to reproductive age in infants with congenital heart disease is higher than ever before. Counseling these patients regarding risk of pregnancy and management throughout the gestational and postpartum period can be challenging as the evidence base is relatively small. Objective: To group data from previously published studies to assess risk of maternal or fetal death according to severity of congenital heart Disease Methods: Studies analyzed were published between 2007 and 2019 and identified from the MEDLINE, EMBASE and Cochrane libraries. Only studies reporting on maternal or fetal mortality with data of the severity of the congenital cardiac lesion were included. Many different study types were included including cohort studies, cross-section studies, randomized trials and case series. Congenital cardiac conditions were categorized into 3 groups for the purpose of analysis: Mild: ASD, VSD, PDA Moderate: coarctation, Ebstein’s anomaly, pulmonary stenosis and Tetralogy of Fallot Severe: double outlet right ventricle, Fontan, pulmonary atresia, transposed great arteries, Eisenmenger syndrome Statistical analysis was performed using Comprehensive Meta-Analysis v3.  Data were summarized according to the total number of patients, total number of events and the event rate.  Event rates were given by percentages with 95% Confidence Intervals by random effects regression. Two methods were used to assess the robustness of the study: Data were reclassified as mild, moderate and severe according to the method published by Stout et al, the analysis was repeated Analyses were repeated removing single studies, if the result then fell outside the 95% confidence interval the results were regarded as biased by that single study Results: Thirty-two studies were included, 27 of those looked at maternal mortality.  There were 9 maternal deaths in total, all due to “uncontrollable heart failure”.  Eight of these 9 women had lesions classed as severe.  Sub-analysis of specific lesions demonstrated mortality was highest in patients with Eisenmenger syndrome (13%) and transposition of the great arteries (1%). Sub-categories were analyzed as summarized in the table below: Complication Event rate mild % Event rate moderate % Event rate severe % Miscarriage/ spontaneous abortion Too few for accuracy 16 34 Small for gestational age 15 14 36 Prematurity 6 14 51 Too few studies looked at therapeutic abortion for the data to be included. Overall recurrence rate (i.e. babies with congenital heart disease born to mothers with congenital heart disease) was 33 in 1075. Conclusions: The most firm conclusion drawn by the authors is the association between more severe congenial heart disease and neonatal mortality. Positive aspects of the study: Data generated can help when counseling women as a broad guide Highlights gaps in the existing literature such as the frequency of therapeutic abortion in women with congenital heart disease Adds to what is a relatively small pool of data in a rapidly growing area of medicine Negative aspects: The authors acknowledge that there was heterogeneity in how certain complications were defined across studies; this was most striking in arrhythmia Many different study designs were used making comparison between studies/ pooling of data less reliable Grouping of disorders as mild, moderate and severe could be criticized, especially since transposition of the great arteries was classed as severe. Some post arterial switch patients are likely to be thought of lower risk than some patients with tetralogy of Fallot repair who have developed multiple complications It is unclear whether the associations with congenital heart disease and some of the sub-group outcomes, such as prematurity, are controlled for other factors that influence prematurity Certain diagnoses which are commonly encountered and produce significant concern in pregnancy do not specifically appear such as bicuspid aortic valve aortopathy (which may not be coarctation) and Marfan’s syndrome.

Usefulness of (18)F-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography in the Diagnosis of Infective Endocarditis in Patients With Adult Congenital Heart Disease. Ishikita A, Sakamoto I, Yamamura K, Umemoto S, Nagata H, Kitamura Y, Yamasaki Y, Sonoda H, Tatewaki H, Shiose A, Tsutsui H.Circ J. 2021 Apr 1. doi: 10.1253/circj.CJ-20-1067. Online ahead of print. PMID: 33790144 Take Home Points: 18F-FDG PET/CT improves diagnostic sensitivity of infective endocarditis from 39% to 88% It helps detect echocardiogram-elusive infective endocarditis in right-sided lesions However, the test was less sensitive in native valve infective endocarditis Commentary from Dr. M.C. Leong (Kuala Lumpur, Malaysia), section editor of ACHD Journal Watch: Fluorine-18 Fluorodeoxyglucose (18F-FDG) is a radioactively labeled glucose analogue that is taken up by the cells. High glycolytic activities which signal high glucose metabolism helps to identify foci of concentrated inflammations. These inflammations are then picked up by the PET scan and when superimposed with the CT scan, detects the location of the inflammation. The 2015 ESC modified diagnostic criteria states that 18F-FDG PET/CT can be performed gives additional diagnostic value when there is (1) positive blood cultures consistent with infective endocarditis (IE); (2) vegetation detected on echocardiography; or (3) minor criteria that suggest the presence of IE.  This article examined the use of the diagnostic accuracy of 18F-FDG PET/CT in various ACHD-associated IE and characterized its advantages. This is a small retrospective study of 22 patients (age: 35 (24, 43) years; 72% male) who have undergone 18F-FDG PET/CT scans for investigation of IE (Table 1). During the clinical course, 18 out of the 22 patients were diagnosed to have IE. Seven patients were diagnosed to have IE on an echocardiogram. Eleven patients who were missed by echocardiogram were diagnosed to have IE by 18F-FDG PET/CT, increasing the detection rate from 39% to 88% (Figure 2). Of interesting note is the number of right-sided infection which was detected (Table 3). The authors reasoned that the right-sided lesions, especially those at the right ventricular outflow tract, were not well delineated by transthoracic echocardiogram and at the same time, too anterior for clear visualization on transoesophageal echocardiogram. 18F-FDG PET/CT scans were negative in 5 patients with IE although echocardiograms detected vegetations in them. Patients who were had a negative 18F-FDG PET/CT scan were found to be older, had a left-sided IE, native valves, less likely to have a history of device implantation, and a lower maximal standardized uptake value (Table 4). The 4 patients who were not diagnosed to have IE had negative echocardiographic and 18F-FDG PET/CT findings. This study is an easy-to-understand study. The main drawback of the study is its small cohort of patients which underpowers analyses. Nevertheless, it provides a real-world experience of utilising 18F-FDG PET/CT to improve the sensitivity of IE detection. One should note, however, that the 18F-FDG PET/CT is less sensitive in detecting IE in native valves.