Right ventricular free wall strain predicts functional capacity in patients with repaired Tetralogy of Fallot.

Right ventricular free wall strain predicts functional capacity in patients with repaired Tetralogy of Fallot. Arroyo-Rodríguez C, Fritche-Salazar JF, Posada-Martínez EL, Arías-Godínez JA, Ortiz-León XA, Calvillo-Arguelles O, Ruiz-Esparza ME, Sandoval JP, Sierra-Lara D, Araiza-Garaygordobil D, Picano E, Rodríguez-Zanella H. Int J Cardiovasc Imaging. 2020 Apr;36(4):595-604. doi: 10.1007/s10554-019-01753-z. Epub 2020 Jan 1. PMID: 31894525 Similar articles Select item 31894524 Take home point: Right ventricular free wall longitudinal strain may be useful as a surrogate marker for low functional capacity in patients with repaired tetralogy of Fallot with severe pulmonary regurgitation (PR). Commentary from Dr. Helen Parry (Leeds UK), section editor of ACHD Journal Watch:  Functional capacity can be a useful guide to pulmonary valve replacement in patients with repaired tetralogy of Fallot who are on the borderline for intervention according to current guidelines.  However, many patients are unable to exercise well due to non-cardiac reasons. This study looked at surrogate markers for impaired functional capacity to help guide management in borderline cases for pulmonary valve replacement. Method: Thirty-three patients were included in the study.  Inclusion criteria were previous tetralogy of Fallot repair, NYHA class 1, no other significant cardiac abnormality such as AVSD and severe pulmonary regurgitation (PR).  Severe PR was defined according to the following criteria: Jet: pulmonary annulus diameter >=0.7 Pressure half time <100ms Diastolic flow reversal in the pulmonary branches PR index <0.77 The level of PR was classed as severe if 3 of the above criteria were fulfilled, or 2 if PR index<0.77. Exercise echocardiography was performed using a bike with initial workload of 25W, increasing by 25 W every 2 mins.  Patients who managed <7 METS were categorised as low functional capacity. Strain imaging was performed using a GE machine and analysis was performed offline using the EchoPAC software.  Inter-observer variability was assessed by repeat analysis by the same operator with a 2 week difference period. Statistical analysis was performed by dividing the patients into 2 groups: low and normal functional capacity.  Comparisons were made using the Students t-test for normally distributed variables and Wilcoxon sum rank test for non-parametric variables. Results: Twenty- two patients had normal functional capacity relative to 11 in the low functional capacity group. The following variables were associated with low functional capacity, p value<0.05: Female gender Short stature Previous shunt palliation LV contractile reserve Right ventricular free wall longitudinal strain <17% Conclusions: Right ventricular free wall longitudinal strain may be useful as a surrogate marker for low functional capacity in patients with repaired tetralogy of Fallot with severe PR. Strengths of the study: Timings for pulmonary valve replacement are a continued subject of debate- many clinicians believe we should be doing this at an earlier stage. Limited literature regarding the uses of newer echo techniques here, this study is an important contribution to the literature. Suggests a useful alternative to exercise testing in these borderline patients. Weaknesses of the study: Very small sample size, only 11 patients in the low functional capacity group-can statistically meaningful associations genuinely be found in such a small sample? Strain imaging is both machine specific and supplier specific- many departments do not use GE systems, the cut-off for right ventricular wall strain is likely to be different across manufacturers. Many patients do not have adequate echo windows for reliable RV strain imaging. Inter-observer variability was tested by the same reporter reviewing the scans with 2 weeks difference. Two individuals performing analysis and testing agreement between the 2 would be a better way of looking at inter-observer variability.


Prevalence and Cause of Early Fontan Complications: Does the Lymphatic Circulation Play a Role?

Prevalence and Cause of Early Fontan Complications: Does the Lymphatic Circulation Play a Role? Ghosh RM, Griffis HM, Glatz AC, Rome JJ, Smith CL, Gillespie MJ, Whitehead KK, O'Byrne ML, Biko DM, Ravishankar C, Dewitt AG, Dori Y. J Am Heart Assoc. 2020 Apr 7;9(7):e015318. doi: 10.1161/JAHA.119.015318. Epub 2020 Mar 30. PMID: 32223393 Free Article Similar articles Select item 32200729   Take Home Points:   Nearly a third of patients developed complications in the first 6 months post Fontan completion. This was mainly driven by prolonged pleural effusions, readmission or unplanned cardiac catheterization. Prolonged cross-clamp time and prolonged bypass time emerged as risk factors for early Fontan morbidity. The presence and severity of AV valve regurgitation did not influence early outcomes. The presence of a type 3 or type 4 lymphatic drainage pattern on MRI (T2 -weighted) was associated with higher early failure rates (Odds ratio 6.28). Commentary from Dr. Blanche Cupido (Cape Town, South Africa), section editor of ACHD Journal Watch:  Recent studies implicated the role of lymphatic congestion in the pathogenesis of both protein-losing enteropathy and plastic bronchitis in patients with the Fontan circulation resulting in late Fontan failure. The role of lymphatic drainage in the development of early Fontan complications is not well described. This is a single center retrospective study at a tertiary paediatric center in the US, describing the prevalence and cause of early post-Fontan morbidity.   All patients who underwent a Fontan operation from June 2012 to July 2017 were included. Those presenting for a Fontan take-down, those for Fontan revision or those with a Kawashima operation, were excluded. Lymphatic patterns were characterized using T2-weighted images. Patterns included 4 types: Type 1: Little or no abnormalities of the thoracic duct Type 2: Enhancement of the supraclavicular area and dilatation and/or tortuosity of the thoracic duct Type 3: Enhancement of mediastinal and supraclavicular area Type 4: enhancement extending from the mediastinum into the lung parenchyma   The primary outcome was a composite of early Fontan complications (within 6 months of surgery) including death, Fontan take-down, ECMO, chest drain >14 days, cardiac catheterization, re-admission, heart transplant listing. Fontan failure is characterized into 4 groups: structural failure, pump failure, pleural (non-chylous) effusions despite lack of pump or structural failure, and lastly lymphatic failure – usually presenting with chylothorax or plastic bronchitis. Two hundred and thirty-eight patients were included in the study; 58 developed early Fontan complications (25.7%) – only 2 deaths occurred. Mean age at surgery was 3.4±1.7 years. An extra-cardiac fenestrated Fontan was present in 81% of the cohort. The presence of a systemic RV (81% vs 67%, p0.047), a longer bypass time (median time 69.5 vs 64 minutes, p=0.025) and a longer cross-clamp time (median time 29 vs 25 min, p=0.002) was associated with a higher rate of early post-Fontan complications.  The presence and severity of atrio-ventricular valve regurgitation did not have an effect on early outcomes. Fontan failure was attributed to structural failure in 20 patients (35%), and to pump failure in 8 patients (14%). The presence of severe Fontan complications was only 4%.  Fifteen patients (21.5%) had prolonged effusions and 15 patients (21.5%) had lymphatic failure as evidenced by plastic bronchitis or chylous effusions. One hundred and ninety-five patients (82%) had pre-Fontan MRI’s. The systemic-pulmonary arterial collateral (APC) burden was quantified and expressed as a percentage of the total aortic flow. Of the 51 patients with a >35%  APC burden, 43 had collateral embolization prior to Fontan completion. Only 126 patients had the correct T2 weighted sequences to establish lymphatic flow patterns: 39 had type 1, 41 had type 2, 35 had type 3. Only 7 had type 4 – thus rather rare. Forty- three percent of those with type 3 pattern and all of those with the type 4 pattern developed early complications. Type 3 and type 4 patterns were combined and regarded as high-grade lymphatic abnormalities. Type 3 and 4 combined subtended an odds-ratio of 6.05 (95% CI 2.10-17.46, p=0.001) for developing complications compared to those with a type 1 pattern. When controlling for a morphological RV, bypass and cross-clamp time, the odds ratio was 6.28 (95% CI 2.13 – 18.5, p=0.001) for developing early Fontan complications.


Body Composition in Young Adults Living With a Fontan Circulation: The Myopenic Profile.

Body Composition in Young Adults Living With a Fontan Circulation: The Myopenic Profile. Tran D, D'Ambrosio P, Verrall CE, Attard C, Briody J, D'Souza M, Fiatarone Singh M, Ayer J, d'Udekem Y, Twigg S, Davis GM, Celermajer DS, Cordina R. J Am Heart Assoc. 2020 Apr 21;9(8):e015639. doi: 10.1161/JAHA.119.015639. Epub 2020 Apr 15. PMID: 32290749 Free Article Similar articles Select item 32315058 Take Home Points: • In this relatively healthy group of Fontan patients, low skeletal muscle mass was associated with reduced exercise capacity, ventricular dysfunction, and compensatory erythrocytosis as a marker of cyanosis. • BMI overestimates skeletal muscle mass and underestimates adiposity in Fontan patients. Commentary by Dr. Maan Jokhadar (Atlanta), section editor of ACHD Journal Watch: The Fontan circulation is associated with elevated central venous pressure, low cardiac output, and cyanosis. These abnormalities increase the risk of heart failure, arrhythmias, thromboembolic events, hepatic fibrosis, and protein using the neuropathy, to name a few. Prior studies have described role of skeletal muscle as a “muscle pump” that increases venous return and augments pulmonary blood flow, which improves cardiac output and improves exercise capacity in Fontan patients. Cardiac dysfunction can cause neurohormonal derangement and associated skeletal muscle loss and myopenia. There is also a complicated and often paradoxical interrelationship between obesity and heart failure (obesity paradox) with obese patients more likely to develop heart failure but obese patients with heart failure having improved survival. Data are mixed regarding the presence of the obesity paradox failure in Fontan patients. Derek Tran and colleagues from Sydney, Australia performed a cross-sectional study of 28 Fontan patients who were prospectively recruited. The mean age was 26 with a near even split between male and female and 57% had a systemic left ventricle. Extracardiac Fontan was present in 50%, lateral tunnel in 39%, and 11% (3 patients) with atriopulmonary Fontan. The median BMI was 22.4 kg/m2. Participants had dual energy x-ray absorptiometry (DXA) to assess Appendicular lean mass index (ALMI) Z score and total percent body fat (%BF). They also underwent cardiopulmonary stress testing, echocardiography, handgrip strength assessment, and biochemical assessments. This was a relatively healthy group with exclusion criteria that included NYHA class III-IV, major intellectual or physical disability, or current pregnancy. - Fontan associated myopenia ( Z score: -2 or lower) : 11 patients (39%) - Less pronounced skeletal muscle mass deficit (Z score: between -2 and -1) : 8 patients (29%) - Normal range muscle mass (Z score: higher than -1) was present in only 9 patients (32%) All participants with normal range skeletal muscle mass had normal ventricular systolic function. Whereas 80% participants with ventricular dysfunction had skeletal myopenia. Males had lower %BF. High adiposity was present in 32%, moderate adiposity and 14%, 50% had normal range adiposity, and 4% (1) had low adiposity. There were 3 patients who had both Fontan associated myopenia and high adiposity. Vitamin D deficiency was not associated with myopenia. Above normal range PTH was present in 40%, even though only 7 patients had low vitamin D. Blood leptin was increased and 70% of patients, reflecting elevated adiposity. ALMI was strongly associated with exercise capacity as measured by peak VO2. Fontan associated myopenia was strongly associated with reduced peak handgrip. There was no difference in spirometry measures between normal and reduced muscle mass groups. This is the first study to characterize body composition using DXA in Fontan patients. This clinically stable group showed low skeletal muscle mass and adiposity predisposition, which can be unrecognized when looking at BMI alone. BMI may overestimate skeletal muscle mass and underestimate adiposity in Fontan patients. Low skeletal muscle mass was associated with reduced exercise capacity, ventricular dysfunction, and compensatory erythrocytosis as a marker of cyanosis. About two thirds of participant had reduced muscle mass. ALMI was independently associated with absolute peak VO2. Grip strength was positively associated with muscle mass and was lower in patients with Fontan associated myopenia. Ventricular systolic dysfunction was associated with low muscle mass, which could be due to peripheral “muscle pump” impairment reducing venous return, pulmonary blood flow, and cardiac output. Ventricular dysfunction can also cause myopenia due to myriad physiologic and neurohormonal mechanisms. Based in DXA analysis of Fontan patients, reduced muscle mass and increased adiposity is common. Given that reduced muscle mass is associated with ventricular dysfunction and reduced exercise capacity, additional study is needed to determine the therapeutic strategies and potentially substantial benefits of building lean muscle mass in these patients.


Risk of coronary artery disease in adults with congenital heart disease: A comparison with the general population

Kuijpers JM, Vaartjes I, Bokma JP, van Melle JP, Sieswerda GT, Konings TC, Bakker-de Boo M, van der Bilt I, Voogel B, Zwinderman AH, Mulder BJM, Bouma BJ. Int J Cardiol. 2020 Apr 1;304:39-42. doi: 10.1016/j.ijcard.2019.11.114. Epub 2019 Nov 18. PMID: 31767384 Similar articles Select item 31894525   Abstract Background: Coronary artery disease (CAD) will increasingly determine outcome in the aging adult congenital heart disease (CHD) population. We aimed to determine sex-specific incidence of CAD in adult CHD patients throughout adulthood, compared to the general population. Methods and results: We followed 11,723 adult CHD patients (median age 33 years; 49% male; 57% mild, 34% moderate, 9% severe CHD) from the Dutch CONCOR registry, and two age-sex-matched persons per patient from the general population for first CAD event in national registers (period 2002-2012). Incidence rates were estimated using smoothed hazard functions. CAD risk during follow-up, stratified by CHD severity, was compared using proportional subdistribution hazards regression. In ACHD patients, 103 CAD events (43 women) occurred over 60,456 person-years. Rates per 1000person-years increased from 0.3(95% confidence interval: 0.1-0.6) at age 20 to 5.8(3.7-8.9) at 70 years in female, and from 0.5(0.3-1.0) to 7.8(5.1-11.8) in male patients. Compared to the general population, relative risk was 12.0(2.5-56.3) in women and 4.6(1.7-12.1) in men aged 20 years. Relative risk declined with age, remaining significant up to age ~65 years in women and ~50 years in men. In patients with mild, moderate and severe CHD, CAD risk was 1.3(0.9-1.9), 1.6(1.0-2.5) and 2.9(1.3-6.9) times increased compared to the general population, respectively. Conclusions: We found increased CAD risk in adult CHD patients, with greater relative risk at younger age, in women and those with more severe CHD. These results underline the importance of screening for and treatment of CAD risk factors in these patients.   source:https://pubmed.ncbi.nlm.nih.gov/31767384


Incidence and fate of device-related left pulmonary artery stenosis and aortic coarctation in small infants undergoing transcatheter patent ductus arteriosus closure.

Incidence and fate of device-related left pulmonary artery stenosis and aortic coarctation in small infants undergoing transcatheter patent ductus arteriosus closure. Tomasulo CE, Gillespie MJ, Munson D, Demkin T, O'Byrne ML, Dori Y, Smith CL, Rome JJ, Glatz AC....

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