Adult Congenital Heart Disease

Baffle Complications in Adults After Atrial Switch for Transposition of the Great Arteries

Baffle Complications in Adults After Atrial Switch for Transposition of the Great Arteries. Woudstra OI, Alban FTE, Bijvoet GP, de Bruin-Bon RHACM, Planken RN, Leiner T, Boekholdt SM, Warmerdam EG, Sieswerda GT, Mulder BJM, Bouma BJ, Meijboom FJ.Can J Cardiol. 2022 Jan;38(1):68-76. doi: 10.1016/j.cjca.2021.09.034. Epub 2021 Oct 9.PMID: 34634378   Take Home Points: There was a high incidence of baffle interventions for baffle complications in patients with a prior atrial switch operation for TGA These interventions were mainly for worsening symptoms (effort intolerance, fluid retention or desaturation) Combining contrast-enhanced transthoracic echocardiography with CT for screening, markedly increases the diagnostic yield of diagnosing baffle complications (CT found baffle stenosis in almost half) There was little correlation between clinical signs and the presence of baffle complications on CT Pulse-wave Doppler had a low sensitivity to detect systemic venous baffle stenosis Baffle stenosis was associated with lower peak exercise work rates (obstruction to ventricular filling in stiff baffles are clinically relevant) Commentary from Dr. Blanche Cupido (Cape Town, South Africa), chief section editor of ACHD Journal Watch: Baffle complications remain a rather overlooked subset of late complications following atrial switch operations for TGA. Interventions for baffle complications have been done for both baffle leaks and baffle stenosis in symptomatic patients. The use of percutaneous intervention for asymptomatic patients is also increasing. The scope of these complications is poorly studied and it’s diagnosis on conventional transthoracic echocardiography limited. For this reason, guidelines recommend routine interval screening by advanced imaging (CT/MRI). The prevalence on previous studies by these modalities on routine screening was 40-60% (though many study limitations noted in these cohorts). This dual-center study conducted in the Netherlands had 3 aims:   To assess the prevalence of consequences of major baffle-related complications (baffle interventions / strokes) To assess the prevalence of baffle stenoses and baffle leaks in unselected TGA patients after atrial switch surgeries To assess whether clinical signs and symptoms differed between patients with and without these complications. A cross-sectional imaging sub-study (2017-2019) of participants in the Dutch CONCOR registry (2001 onward) was done looking at a standardized screening protocol for baffle complications, instituted in both centers in 2017. All patients had a routine contrast-enhanced transthoracic echo with agitated saline during rest and exercise to assess baffle patency – agitated saline (after a test injection to exclude a large R-L shunt in case of a baffle leak) is performed using 8mL agitated saline and graded upon the amount of bubbles seen in the systemic ventricles within 3 cardiac cycles.   Since 2017, both centers involved in the study instituted CT (arterial and venous phase scanning) as part of routine screening. Systemic venous baffle dimensions were measured and stenosis was defined as a transverse luminal diameter <10mm.   There were 67 patients with arterial switch surgeries for TGA in the CONCOR Registry. Mustard repairs were done in 85% (n=57). Patients were followed up for a median of 9 years. Baffle interventions were performed in 36% (n=24), with 46% having re-interventions. Fourteen had their interventions prior to enrollment in the CONCOR registry with no details available. Most patients presented with increasing symptoms (effort intolerance, fluid retention or desaturation). Indications for intervention included:   1 – baffle leak only 6 – stenosis only 3 – both stenosis and leak No patients experienced strokes, 5 died (3 heart failure, 2 infective endocarditis) and 12 were lost to follow-up. Figure 3 below shows a cumulative risk of re-intervention for baffle complications as 25% at 15 year follow-up:     Fifty patients had CT scans. Of the 29 patients who had both contrast-enhanced transthoracic echo (CE-TTE) and CT, 4 (14%) had no baffle complications; 11 (38%) had baffle leaks only; 5 (17%) had stenosis only and 9 (31%) had both stenosis and leaks. Systemic venous to systemic arterial circulation (R-L) shunting was found in 69% (n=20) of patients – none of them had known or suspected shunting. In 8 patients (40%) the shunting was spontaneous without provocation. Both exercise and Valsalva maneuvers increased the diagnostic yield on CE-TTE.     There was no statistically significant associations between clinical characteristics (including oxygen saturation at rest or exercise) and the presence of baffle leaks.   Baffle stenosis was associated with a lower peak work rate noted on exercise echocardiography (89 ±24W vs 123±21W, p<0.001) – figure 5 below. This remained significant even after correcting for body surface area (p=0.003). Peak Doppler flow over the systemic venous baffle was not associated with the presence of anatomic stenosis (<10mm diameter) on CT (1.1 vs 1.0m/s for those with and without stenosis respectively; p=0.53).   

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Cardiac MRI predictors of adverse outcomes in adults with a systemic right ventricle

Cardiac MRI predictors of adverse outcomes in adults with a systemic right ventricle. Lewis MJ, Van Dissel A, Kochav J, DiLorenzo MP, Ginns J, Zemer-Wassercug N, Groenink M, Mulder B, Rosenbaum M.ESC Heart Fail. 2022 Apr;9(2):834-841. doi: 10.1002/ehf2.13745. Epub 2022 Jan 20.PMID: 35048545   Commentary by Dr. Helen Parry (Leeds, UK), section editor of ACHD Journal Watch:   Introduction: The anatomical right ventricle re-models in order to adapt to function as a systemic ventricle. However, a point is reached where this is maladaptive.   Study question: Do MRI measurements of the volume of the systemic right ventricle predict clinical course?   Method: This was a retrospective case-control study. Two centres were involved: the Columbia University Irving Medical Centre and the Amsterdam University Medical Centre.   Inclusion criteria for cases: Age >= 18 years Congenitally corrected transposition of the great vessels (CCTGA) D-TGA with mustard or Senning (atrial switch) Ability to safely undergo MRI scanning Three primary outcomes studied were death, referral for cardiac transplant and use of ventricular assist device. Degree of tricuspid regurgitation (TR) and medications were analysed. The MRI images were analysed by manual tracing in end diastole and end systole. The degree of inter rater variability was assessed by 2 reporters blindly analysing the same 10 scans. Receiver operating curves and area under the curve (AUC) were calculated for: Right ventricular end diastolic volume, indexed (RVEDVI) Right ventricular end systolic volume, indexed (RVESVI) Right ventricular ejection fraction, RVEF (RVEF= RVEDV-RVESV)/100) Right ventricular mass The AUCs were compared using Cox proportional hazard regression analysis using STATA. Adult patients with a systemic right ventricle who were unable to undergo MRI scanning due to presence of a non-MRI conditional device were used as controls.   Results: Baseline cardiac MRI scanning was performed between December 1999 and November 2020 on patients who met the inclusion criteria: 101 DTGA with atrial switch 57 CCTGA N=158 A total of 21 patients (13%) met the primary end point during a follow up period of 1320 patient years. Those with CCTGA were more likely to meet the endpoint (9% versus 21%, p= 0.029). There were 15 deaths, 4 referrals for cardiac transplant and 2 patients were given a ventricular assist device as destination therapy.   Patients with CCTGA and DTGA with atrial switch were analysed separately by univariable and multi variable analysis. Multivariable analysis was performed according to tricuspid regurgitation of moderate severity or more and age at MRI. Separate multivariable analysis was performed in patients with adjustment for tricuspid valve replacement following MRI. In all the above analyses, RVEDVI, RVESVI, RVEF and RV mass remained predictive of the primary end point at the 1 in 20 level (p<0.05). No association between the presence of tricuspid regurgitation of moderate severity or greater and the primary end points were found.   Thirty nine adult patients with systemic right ventricles had non-MRI conditional devices. Eight of these (21%) met the primary end point during follow up and there was no statistically significant difference with the MRI group.   Discussion and conclusion: MRI measurements outlined above predict end stage heart failure in patients with systemic right ventricles. This is superior to the use of estimated right ventricular function on 2D transthoracic echo alone when compared with the previous literature.   Positive aspects of the study: Two centres were used to increase confidence in findings Inter rater variability was very small Novel: this study is the first looking at RV size and function according to MRI assessment as predictors of outcome in systemic right ventricle Negative aspects: The sample size meant a relatively small number of patients met the primary end point, making the statistical power of the study questionable Patients with tricuspid regurgitation were divided into a) less than moderate and b) moderate or more. It may be that there is a significant difference between patients with moderate and patients with severe TR, especially since TR is so common in patients with systemic right ventricles. The clinical application requires further work. Knowing these patients are more likely to develop end stage heart failure does not necessarily mean interventions can be made at this point that would prevent that from happening.   

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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)

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. 2022 Jan;108(2):117-123. doi: 10.1136/heartjnl-2020-318685. Epub 2021 Apr 28. 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 Prepregnancy signs of heart failure and sRV ejection fraction <40% predicted MACE.   Commentary by 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 Programme (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, supraventricular or ventricular arrhythmias needing treatment, heart failure (requiring hospital admission, new or change in treatment), aortic dissection, endocarditis, ischaemic 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. Prepregnancy 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.   

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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)

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,...

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Comparison of Patent Ductus Arteriosus Stent and Blalock-Taussig Shunt as Palliation for Neonates with Sole Source Ductal-Dependent Pulmonary Blood Flow: Results from the Congenital Catheterization Research Collaborative

Comparison of Patent Ductus Arteriosus Stent and Blalock-Taussig Shunt as Palliation for Neonates with Sole Source Ductal-Dependent Pulmonary Blood Flow: Results from the Congenital Catheterization Research Collaborative. Bauser-Heaton H, Qureshi AM, Goldstein BH,...

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