Adult Congenital Heart Disease

Nationwide Registry-Based Analysis of Infective Endocarditis Risk After Pulmonary Valve Replacement. Stammnitz C, Huscher D, Bauer UMM, Urban A, Nordmeyer J, Schubert S, Photiadis J, Berger F, Klaassen S; German Competence Network for Congenital Heart Defects Investigators.J Am Heart Assoc. 2022 Mar;11(5):e022231. doi: 10.1161/JAHA.121.022231. Epub 2022 Feb 18.PMID: 35179045   Take Home Points: Bovine jugular vein valves (Contegra/Melody) have the highest risk of infective endocarditis, irrespective of the mode of deployment (surgical or percutaneous) Male sex and higher number of previous pulmonary valve replacements increase risk of Infective Endocarditis Commentary from Dr. Shailendra Upadhyay (Connecticut, USA), section editor of ACHD Journal Watch: This study is the largest retrospective analysis of the German NR-CHD (National Register for Congenital Heart Defects) comparing the risk of infectious endocarditis (IE) after percutaneous pulmonary valve implantation or surgical pulmonary valve replacement in congenital heart disease. All patients with CHD with at least 1 surgical pulmonary valve replacement (SPVR) or percutaneous pulmonary valve replacement (PPVI) before January 1, 2018 [January 2007-Dec 2017] were included and followed up for the combined end point (grafts infected or explanted, or the patient was deceased). The study included 1170 patients (median age 12, 5-20 years, 56% males, 68% < 18 years) that included 1598 surgical pulmonary valve replacement (SPVR) including - aortic/pulmonary homograft, heterograft including Contegra, bio-prosthetic valved conduits using Bovine/Porcine materials, mechanical valves and percutaneous pulmonary valve implantation (PPVR) including Melody and Edward Sapien valves. Diagnoses included: Tetralogy of Fallot (TOF)- commonest, Truncus Arteriosus, Aortic stenosis and s/p Ross operation. Total follow-up was 9397 years (per patient median 10 years). Clinical characteristics of patients is noted in table 2. IE occurred in 4.8% during a 10-year median follow-up (6-10 years), tables 1 and 2. IE After homograft 1.3%; IE After heterograft 4.3% [Heterograft non Contegra 2.5% and Contegra 5.4%]; IE After Melody 7.5%.     IE showed no significance influence on the overall survival of the patients ([HR], 3.57; P=0.20). Bovine jugular vein valves (Contegra and Melody) had the highest risk of IE [Table S4], irrespective of the mode of deployment, either surgical or percutaneous. In the multivariable analysis, the risk of IE was increased in the male sex, in patients with a higher number of previous pulmonary valve replacement and for bovine jugular vein valves (with a similar risk for Melody versus Contegra valves). [Table 3].     Conclusion: Homograft or non Contegra heterograft replacement has the least risk of IE. Bovine jugular valves (Contegra/Melody) have the highest risk of IE whether deployed surgically or percutaneously. Male sex and higher number of previous PVR add significant IE risk. Other significant risk factors for IE are male sex and higher numbers of previous PVR. The Edwards-Sapien valve may be useful for PPVI in high risk subgroups for IE, however data are limited.

Young adults after arterial switch operation for transposition of the great arteries in Switzerland: a growing population. Ruperti-Repilado FJ, Affolter J, Bouchardy J, Gabriel H, Stämpfli SF, Engel R, Schwerzmann M, Greutmann M, Tobler D.Swiss Med Wkly. 2022 Feb 23;152:w30114. doi: 10.4414/smw.2022.w30114. eCollection 2022 Feb 14.PMID: 35230031   Take Home Points: Patients with d- transposition of the great arteries (d-TGA) post arterial switch generally have a good biventricular function and a normal exercise capacity. Some of these patients suffer from cardiac events or require reinterventions, and they are likely patients with complex d-TGA. Pregnancy within this cohort is largely safe with favourable outcomes. Commentary from Dr. MC Leong (Kuala Lumpur), section editor of ACHD Journal Watch: Previous studies have reported the long-term outcomes of patients with d-transposition of the great arteries (d-TGA) after the arterial switch operations (1-4). These studies, which were retrospective in nature, were usually flawed with issues inherent in retrospective studies, e.g., missing data, patients lost to follow up and recall errors. In this study, the authors attempted to overcome these deficiencies by examining data prospectively. Data were extracted from a prospectively collected Swiss Adult Congenital HEart disease Registry (SACHER). The registry was established in 2013 and was webbed-based (5). In this study, the authors examined the outcomes of patients with d-TGA post arterial switch operation in the hope of raising awareness among general practitioners and general cardiologists regarding this growing population and their potential complications.   All 149 patients with d-TGA post arterial switch in the registry were included. Baseline characteristics of the cohort were tabulated in Table 2. In the study, the authors echoed the findings of previous studies, which demonstrated that adults post arterial switch operation generally presented with good biventricular function and normal exercise capacity. However, many had residual lesions, which may or may not require intervention. The usual residua of RVOT obstruction, branch pulmonary artery stenoses, infective endocarditis and etc., were largely similarly to those seen in previous reports. The study also touched on the functional status (VO2max and NT-proBNP) as well as pregnancy outcomes which were largely unremarkable.   Interestingly. the authors showed that patients with complex d-TGA tended to suffer from cardiac events (occurring in 18% of patients with complex d-TGA) and required reintervention (Figure 1). These patients also had a trend towards a lower survival compared to their peers with simple TGA but the difference was not statically significant. The authors argued that this might be due to the overall low number of outcomes. Of the outcome examined by the study, one that particularly interested me was the growth of neo-aortic root and the accompanying neo-aortic valve incompetency. Admittedly, the reported aortic dissection in this population is exceedingly low. However, the study showed that the majority of the patients had at least moderate dilatation of the aortic root, and they demonstrated a marginal but progressive growth in the aortic dimensions over time (Table 2 & S2). Whether many of these patients would eventually require intervention in the distant future is yet to be seen. Only time will tell. Equally interesting was the presence of the increase in the QRS duration over time (Table S2). Its significance is yet to be determined, but one would assume that a progressive increase in the QRS duration may lead to inter-ventricular dyssynchrony, which may cause deterioration of heart function in the long run.   All in all, this is an interesting audit of the Swiss d-TGA cohort. The authors should be commended for establishing the prospectively collected SACHER registry. It would give systematically collected outcome data regarding this cohort of patients in the distant future and help understand this unique cohort. We would wait with much eagerness for the long-term effects of neo-aortic root dilatation as well as the prolonged QRS as they unfold over time. Reference: Tobler D, Williams WG, Jegatheeswaran A, Van Arsdell GS, McCrindle BW, Greutmann M, Oechslin EN, Silversides CK. Cardiac outcomes in young adult survivors of the arterial switch operation for transposition of the great arteries. J Am Coll Cardiol. 2010 Jun 29;56(1):58-64. Khairy P, Clair M, Fernandes SM, Blume ED, Powell AJ, Newburger JW, Landzberg MJ, Mayer JE Jr. Cardiovascular outcomes after the arterial switch operation for D-transposition of the great arteries. Circulation. 2013 Jan 22;127(3):331-9. Santens B, Van De Bruaene A, De Meester P, Gewillig M, Troost E, Claus P, Bogaert J, Budts W. Outcome of arterial switch operation for transposition of the great arteries. A 35-year follow-up study. Int J Cardiol. 2020 Oct 1;316:94-100. Kempny A, Wustmann K, Borgia F, Dimopoulos K, Uebing A, Li W, Chen SS, Piorkowski A, Radley-Smith R, Yacoub MH, Gatzoulis MA, Shore DF, Swan L, Diller GP. Outcome in adult patients after arterial switch operation for transposition of the great arteries. Int J Cardiol. 2013 Sep 10;167(6):2588-93. Tobler D, Schwerzmann M, Bouchardy J, Engel R, Stambach D, Attenhofer Jost C, et al.; On Behalf Of Sacher. Swiss Adult Congenital HEart disease Registry (SACHER) - rationale, design and first results. Swiss Med Wkly. 2017 Oct;147:w14519   

Diagnostic Value of 18F-Fluorodeoxyglucose Positron Emission Tomography Computed Tomography in Prosthetic Pulmonary Valve Infective Endocarditis. Venet M, Jalal Z, Ly R, Malekzadeh-Milani S, Hascoët S, Fournier E, Ovaert C, Casalta AC, Karsenty C, Baruteau AE, Le Gloan L, Selegny M, Douchin S, Bouvaist H, Belaroussi Y, Camou F, Tlili G, Thambo JB. JACC Cardiovasc Imaging. 2022 Feb;15(2):299-308. doi: 10.1016/j.jcmg.2021.07.015. Epub 2021 Sep 15. PMID: 453863   Study Summary and Take-Home Points: In this retrospective study that included 66 patients with suspected endocarditis of a prosthetic pulmonary valve or right sided conduit, 44 patients had definite diagnosis of endocarditis, 10 had possible and 12 a rejected diagnosis. The most frequent pathogens were staphylococcus and streptococcus, both accounting for 68% of overall microbiological findings. The median interval between the initial endocarditis suspicion date and a 18F-Fluorodeoxyglucose Positron Emission Tomography Computed Tomography (18 F-FDG-PET/CT) was 7.5 days. Appropriate antibiotics were started before PET/CT for 83.3% of the patients. Among them, the median delay between antibiotics onset and PET/CT was 7 days. Sufficient myocardial suppression was obtained in 92.5% of the cases. The sensitivity, specificity, positive, and negative predictive values of 18F-FDG PET/CT for diagnosis of PPVE were as follows: 79.1% (95% CI: 68.4%-91.4%), 72.7% (95% CI: 60.4%-85.0%), 91.9% (95% CI: 79.6%-100.0%), and 47.1% (95% CI: 34.8%-59.4%), respectively. The median time between antibiotics starting and PET/CT in the false negative cases was 2.5 days and median CRP level was 56 mg/L, whereas in true positive cases, this delay was 7.0 days and median CRP level was 67.0 mg/L. More atypical causing bacteria were grown in the false negative group. The type of prosthesis was not associated with higher false positives or negatives PET/CT results. Among the 44 cases of definite endocarditis, 27 patients exhibited both positive imaging studies (i.e. TTE/TEE or cardiac CT) and PET/CT, and 13 patients presented a positive PET/CT despite a negative imaging study. PET/CT appeared to provide better diagnostic capabilities when more common causing organisms were involved: when streptococcus or staphylococcus were identified as the causative agent, 80% PET/CTs were positive, whereas when less common microorganisms were involved, PET/CT was positive in 66.7%. Notably, four definite endocarditis patients had both negative PET/CT and imaging studies. In these patients, the causing organisms were atypical (Streptococcus sanguinis, Aggregatibacter actinomycetemcomitans, Candida parapsilosis, and Coxiella burnetii) Embolic and distant lesions were identified by PET/CT in 31 patients. Pulmonary septic emboli were identified by chest CT and PET/CT in 14 patients, 13 of which had definite and 1 had possible endocarditis. During a median follow up period of 26.6 months in the endocarditis group, 75% of the patients required surgery and one 50 year old patient with tetralogy of Fallot and other “severe comorbidities” passed away from the infection. Commentary from Dr. Yonatan Buber (Seattle, USA), section editor of ACHD Journal Watch: The overall incidence of endocarditis in congenital heart disease patients is estimated at 1.33 per 1,000 person-years, and 50% to 60% of the cases involve right-sided valves with a high proportion of pulmonary prosthetic valve or conduit endocarditis. Both transcatheter and surgically implanted valve can be affected. Although the integration of 18F-FDG positron emission PET/CT increased the diagnostic sensitivity by 52%-70% to 91%-97% for endocarditis involving left-sided valve, the data on its applicability for right-sided on endocarditis remains limited.   This was a retrospective study conducted between 2010-2020 in 8 centers in France aimed to evaluate the diagnostic performance of 18F-FDG PET/CT in the diagnosis of prosthetic pulmonary valve or conduit endocarditis in CHD patients. Sixty-six suspected pulmonary prosthetic valve or conduit endocarditis episodes involving 59 patients were included, all of which underwent an 18F-Fluorodeoxyglucose Positron Emission Tomography Computed Tomography (18F-FDG PET/CT) study . Median age was 23.4 years, 10 patients were below 15 years of age, the youngest was 5 years old, and 72.7% were men. The involved RVOT prosthetic substrates included percutaneous pulmonary valved stents (Melody, Medtronic Inc; and Sapien, Edwards Lifesciences devices), surgical pulmonary bioprostheses, right ventricle to pulmonary artery prosthetic conduits, and pulmonary homografts.   Similar to other studies reporting the diagnostic performance of 18F-FDG-PET/CT in left-sided endocarditis, the main strength appears to be its strong positive predictive value, which in this study was 92%, whereas its main limitation remains the very low negative predictive value, which in this study was only 47%. Sensitivity (79%) and specificity (72%) were in the moderate-high range. Noteworthy observations that are of considerable clinical implications are that the diagnostic capabilities of 18F-FDG-PET/CT were lower when the causative organisms were atypical, when the inflammatory markers were lower and when the studies were performed <3 months from the time of the valve or conduit implantation.   The authors provide additional theoretical explanations to the low negative predictive performance, including the fact that many nuclear physicians are less familiar with RVOT features than left-sided valve prostheses, and the potential lower difference between pathological and nonpathological 18F-FDG uptake with some biological tissues such as bovine jugular vein materials or pulmonary homografts.   Notably, the gold standard used for the diagnosis of endocarditis in this study was an expert consensus obtained by an endocarditis group, while only 4 explanted valves or conduits showed evidence of infection. An important limitation is the lack of intracardiac echo studies in this series, a tool that is commonly used in contemporary practice for suspected pulmonary valve endocarditis patients.   18-F FDG PET/CT can thus be a useful tool in the workup of patients with suspected prosthetic pulmonary valve endocarditis. It appears that is should be performed early in the inflammation phase but not in the first 2-3 months after the valve implantation, and that CRP levels could help guide the timing. Negative results should not be used to rule out the diagnosis of endocarditis in this clinical setting.