May

Tetralogy of Fallot or Pulmonary Atresia with Ventricular Septal Defect after the Age of 40 Years: A Single Center Study

Tetralogy of Fallot or Pulmonary Atresia with Ventricular Septal Defect after the Age of 40 Years: A Single Center Study   Hock J, Schwall L, Pujol C, Hager A, Oberhoffer R, Ewert P, Tutarel O.J Clin Med. 2020 May 19;9(5):E1533. doi: 10.3390/jcm9051533.PMID: 32438748 Free article.   Take Home Points   Managing the older adult with ACHD is increasingly common in clinical practice but there is limited data available for this particular group This was a retrospective analysis of patients aged >40 years old between 2005 and 2018 Combined primary endpoint was death, prevention of sudden cardiac death, aborted sudden cardiac death, pacemaker implant, arrhythmia or new-onset heart failure 184 patients were identified who were aged > 40 years – 86% with tetralogy of Fallot and 14% with pulmonary atresia/VSD Median follow up was 3.1 years (IQR 0.6-6.5 years) The combined primary endpoint was defined as any cause death, prevented sudden death, implanted pacemaker, new onset heart failure or new arrhythmia – this was occurred in 19% (n=35). The combined secondary end point was defined as all cause death or prevented sudden cardiac death – this occurred in 8% (n=13) An acquired co-morbidity was present in over two thirds of patients (n=126) and nearly half of the cohort (n=72) had 2 or more co-morbidities NYHA class (HR 2.1, 95% CI 1.2-3.6, p=0.009) and age were independent risk predictors of the primary outcome in multivariable analysis     Commentary from Dr. Damien Cullington (Liverpool, UK), section editor of ACHD Journal Watch: Collective efforts to improve the care of patients with ACHD presents new challenges of the care of adults who are increasingly aged. Over the last few decades, these changes in demographics have resulted in the ‘new norm’ in most of our practices. It now means that acquired heart disease needs to be considered increasingly frequently when assessing symptoms. Often overlooked in a busy outpatient clinic, but closer consideration of risk factor modifications to avoid the development of acquired heart disease needs more of our attention i.e. weight management advice, blood pressure control, lipid modification, exercise advice – a non-exhaustive to-do list and this is just for starters!   There is limited data/analysis which has focused primarily on outcomes of the older adult with ACHD. The authors nicely present a table of other comparative analyses to their own (Table 1). Hock et al. identified 184 patients > 40 years old and followed up at the German Heart Centre over a 15-year period (mean age 45.3 7.2 years). Surgical repairs were performed between 1958 and 2000 with a mean age of repair at 10 7.8 yrs. The primary combined endpoint was classified as the occurrence of a major cardiovascular event and the secondary endpoint as either all cause death or aborted SCD. All patients had previously undergone total corrective repair – patients with a ‘palliative repair’ or who had not undergone surgery (n=15) were excluded from the final analysis (n-169) although it would have been of interest to the reader understand the outcomes of this minority cohort too. The baseline demographics of the cohort are shown in Table 2.   Table 1. Hock et al. study data vs comparable datasets   Table 2. Patient demographics at baseline In 2005, 31 patients were aged >40 years old, increasing to 93 patients in 2017. Approximately 80% of patients included (n=149) were aged in the range 40-50 years. LV and RV function were qualitatively assessed – 78% had normal RV function and 92% normal LV function.   Patient co-morbidities are listed in Table 3. This cohort had high rates of co-incident co-morbidity – only about a third of patients had none (n=58). Of interest, 78 patients (n=42%) had at least one interventional procedure after their 40th birthday. Similarly, 48 patients (26%), had an EP procedure. Almost a third of patients (n=55) had experienced arrythmias – mostly atrial.   Table 3 – Co-morbidity prevalence                                 Primary and Secondary outcomes   During a median follow up of 3.1 years (IQR 0.6-6.5 years), 3 patients died. The primary endpoint occurred in 35 patients – death (n=3), prevented sudden cardiac death (n=10); pacemaker implant (n=9); new onset heart failure (n=8) and clinically significant arrhythmias (n=5).   Univariable and multivariable predictors of primary endpoint are shown in Table 4.   Table 4 - Univariable and multivariable analyses Conclusions   This analysis of older patients with TOF or PA/VSD showed that acquired co-morbidities are common and likely to be increasingly so as the individual ages. Although co-morbidity of any kind was not an independent risk predictor of outcome, this is perhaps a reflection of the duration of follow up – some co-morbidities require decades to manifest pathologically. Assessment of primary prevention risk factors as part of a holistic strategy to modify risk needs to be made more systematically than is likely to be done at present – one hopes that modification will translate into better longer term outcomes. This is something which needs closer attention by all of us involved in the care of ACHD patients – too often this takes a backseat to our concentrated focus on the gross anatomy.    

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Pulmonary Hypertension in Adults with Congenital Heart Disease: Real-World Data from the International COMPERA-CHD Registry

Pulmonary Hypertension in Adults with Congenital Heart Disease: Real-World Data from the International COMPERA-CHD Registry.   Kaemmerer H, Gorenflo M, Huscher D, Pittrow D, Apitz C, Baumgartner H, Berger F, Bruch L, Brunnemer E, Budts W, Claussen M, Coghlan G, Dähnert I, D'Alto M, Delcroix M, Distler O, Dittrich S, Dumitrescu D, Ewert R, Faehling M, Germund I, Ghofrani HA, Grohé C, Grossekreymborg K, Halank M, Hansmann G, Harzheim D, Nemes A, Havasi K, Held M, Hoeper MM, Hofbeck M, Hohenfrost-Schmidt W, Jurevičienė E, Gumbienè L, Kabitz HJ, Klose H, Köhler T, Konstantinides S, Köestenberger M, Kozlik-Feldmann R, Kramer HH, Kropf-Sanchen C, Lammers A, Lange T, Meyn P, Miera O, Milger-Kneidinger K, Neidenbach R, Neurohr C, Opitz C, Perings C, Remppis BA, Riemekasten G, Scelsi L, Scholtz W, Simkova I, Skowasch D, Skride A, Stähler G, Stiller B, Tsangaris I, Vizza CD, Vonk Noordegraaf A, Wilkens H, Wirtz H, Diller GP, Grünig E, Rosenkranz S.J Clin Med. 2020 May 13;9(5):E1456. doi: 10.3390/jcm9051456.PMID: 32414075 Free article.   Take Home Points:   Adults with congenital heart disease and pulmonary hypertension have better survival compared to those with idiopathic pulmonary arterial hypertension Treatment patterns changed over time, with more patients receiving combination targeted pulmonary arterial hypertension therapy Despite the lack of evidence, some patients with congenital heart disease and pulmonary hypertension continued to receive anticoagulant and antiplatelets     Commentary from Dr. MC Leong (Kuala Lumpur), section editor of ACHD Journal Watch: The COMPERA (Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension) registry is a prospective European registry of all patients with various forms of pulmonary hypertension. It started in Dresden, Germany in July 2007 and soon grew to include 49 pulmonary hypertension centers across 11 European countries. Understandably, the majority of the centers are from Germany. The registry has since collected some 8200 adult patients and become one of the largest pulmonary hypertension registries.   Baseline demographics   This study looks at the subset of 680 (8.3% from the total patients in the registry) adult patients (median age: 44 years, range 18-87 years and 66.6% females) in the registry with congenital heart disease. The median age of the patient is comparatively higher than the mean age of other registries of patients with congenital heart disease (CHD). This registry included 7 patients who were palliated with a Fontan circuit and received targeted pulmonary arterial hypertension (PAH) medications. Patient demographics and baseline characteristics of these patients were summarized in Table 1. Of the 680 patients, 487 (71.6%) patients had cardiac catheterization to confirm the diagnosis of pulmonary hypertension. The rest of the patients were diagnosed through non-invasive investigations.     Figure 1, showed the age distribution of this cohort of patients.     Types of congenital heart disease   Simple lesions such as ventricular septal defects (29.3%) and patent ductus arteriosus (5.9%) constitute the majority of the cases. Atrial septal defects constitute 27.4% of all patients. 264 patients had their congenital heart disease operated. A summary of the types of congenital heart disease was tabulated in table 4.       Medication and treatment strategies   600 (88.2%) patients received targeted PAH therapy. 389 (65%) received endothelin receptor antagonists while 353 (59%) received phosphodiesterase type-5 inhibitors. Tables 2 & 3 showed the treatment characteristics and the distribution of targeted PAH medications used in different types of PAH in this cohort. 80 patients with Eisenmenger syndrome were treatment-naïve. These patients, according to the authors, were younger and had milder clinical symptoms. Treatment pattern changed over time. In the initial period, monotherapy was seen in the majority of patients (70% of total patients). After a median observation time of 45.3 months, more patients (50%) were on combination therapy in all groups except for patients with Fontan palliation (Figure 3). This was in line with the emergence of recent studies demonstrating the effects of goal-targeted therapy as well as combination therapies towards improved survival.     The use of anticoagulants or platelet was varied. It was interesting to note that 13.4% of patients with Eisenmenger were on antiplatelets as there is no reliable data to support the beneficial effects of antiplatelet in this group of patients (Figure 4). There was also a substantial amount of patients receiving vitamin K or novel anticoagulants.   Survival   Of the 511 patients who received targeted PAH therapy, 91 died during the 5-year follow-up period, compared to 41 deaths in 1326 patients with idiopathic PAH. Patients with CHD had a better 5-year survival compared to patients with idiopathic PAH (Figure 5). Among patients with Eisenmenger syndrome, those with a simple CHD had a better survival compared to those with complex CHD (81% vs 64% survival at 5-year, p=0.063). These findings were in accord with previous studies.   This is an audit of a large registry. Like many multi-center registries, it is a mammoth task to validate all the data that is being entered and hence there may be inherent error in the data entry. The authors should be commended for being able to verify registry data in up to 70% of the participating centers. Pulmonary hypertension in CHD is often not easy to group and analyze. For example, patients with atrial septal defect and PAH may be classified as a simple lesion but the underlying pathogenesis is complex and often associated with idiopathic form of PAH. To include these patients in the simple lesion group and analyze their survival with other truly simple lesions changes the survival trajectory. Also, patients with single ventricle physiology with and without palliation may have a different cause of death. Patients with fenestrated Fontan circuits have complex physiology preventing accurate assessment of its pulmonary hypertension. Lastly, time 0 in survival curves in patients with CHD and pulmonary hypertension usually arbitrarily taken as the date the patients are included into the registry. Often, patients with bad disease would have died prior to being included into the registry and by natural selection, the survived patients are the ones with better and more stable disease. This gives the false impression that patients with CHD and pulmonary hypertension have better survival than patients of other causes of pulmonary hypertension. It will be interesting to see the “real” survival curve of this group of patients if time 0 be taken during infancy or childhood when the pulmonary hypertension starts to develop.    

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Skeletal muscle index determined by bioelectrical impedance analysis is a determinant of exercise capacity and a prognostic predictor in patients with congenital heart disease

Skeletal muscle index determined by bioelectrical impedance analysis is a determinant of exercise capacity and a prognostic predictor in patients with congenital heart disease.   Sato M, Inai K, Asagai S, Harada G, Shimada E, Sugiyama H.J Cardiol. 2020 May 18:S0914-5087(20)30159-3. doi: 10.1016/j.jjcc.2020.04.011. Online ahead of print.PMID: 32439338   Take Home Messages   Bioelectrical impedance analysis (BIA) has been introduced in several clinical fields, such as cardiology, nephrology, hepatology, nutrition, and rehabilitation. BIA is a non-invasive, rapid, and safe assessment method that involves the application of alternating currents to the body to acquire eight-polar tactile-electrode impedance. Skeletal muscle Index (SMI) determined by BIA is a determinant of exercise capacity and can be used as a prognostic predictor in patients with CHD.     Commentary from Dr. Soha Romeih (Aswan, Egypt), section editor of ACHD Journal Watch:   Patients with congenital heart disease (CHD) reportedly have reduced exercise capacity. Underlying cardiac anatomy, a sedentary lifestyle, and chronotropic incompetence are thought to be associated with exercise impairment (Figure 1). Bioelectrical impedance analysis (BIA) has been introduced in several clinical fields, such as cardiology, nephrology, hepatology, nutrition, and rehabilitation. BIA is a non-invasive, rapid, and safe assessment method that involves the application of alternating currents to the body to acquire eight-polar tactile-electrode impedance. The first signs of heart failure (HF) are thought to appear during exercise. If BIA parameters can predict impaired exercise capacity and detect unbalanced body composition, it could be used to evaluate the early stages of HF in patients with CHD.                 The present study aimed to clarify the correlation between BIA parameters and exercise capacity as well as the prognostic importance of the skeletal muscle index (SMI) in patients with CHD.   This retrospective single-center study included 305 consecutive patients aged > 12 years with CHD. Patients with a cardiac pacemaker were excluded because the electric current generated by BIA could interfere with device function. Additionally, pregnant patients were excluded because BIA in pregnancy is unreliable.   Blood samples were obtained to analyze the hemoglobin, albumin, creatinine, sodium, and brain natriuretic peptide (BNP) levels. Using the M-mode of the parasternal short-axis view, the left ventricular fraction shortening (LVFS) was determined. Similarly, they measured the tricuspid annual plane systolic excursion using the M-mode of the apical 4-chamber view. The early mitral inflow velocity (E) was measured using pulsed Doppler imaging with the sample volume at the mitral tip. The early diastolic mitral annular velocity (e’) was measured at the septal annulus using TDI, and the E/e’ ratio was calculated.   The 6-minute walking test (6MWT) was performed. A cardio-pulmonary exercise test (CPX) on a treadmill using the ramp protocol performed to assess peak oxygen uptake (peakVO2) and oxygen uptake at the aerobic threshold (ATVO2).   Bioelectrical impedance analysis (BIA)   The BIA was performed in patients 2–4 h after lunch and the oral administration of medicines. In the present study, to compare skeletal muscle and mineral levels in a wide range of body sizes, skeletal muscle mass was indexed to height squared (that is, the Skeletal muscle index - SMI), and mineral content was indexed to height squared (that is, the mineral index, MI). Additionally, the Extracellular Index (EI), which is the ratio of Extra-cellular water (ECW) divided by the total body water, was assessed. The BIA parameters were blinded to physicians who determined the HF related admissions and those who performed the 6MWT and CPX.   Assessments   Correlation between exercise capacity and bioelectrical impedance parameters   To clarify the factors associated with exercise capacity, the correlation between peak VO2 and BIA parameters in patients with biventricular as well as single ventricular morphology was determined. Reduced exercise capacity was defined as peak VO2 under 20 ml/kg/min.   Comparison of bioelectrical impedance parameters and exercise capacity with regard to ventricular morphology   To elucidate any influence that ventricular morphology might have, the BIA and exercise capacity (6MWT, peak VO2, and AT VO2) parameters of patients with biventricular morphology as well as those with single ventricular morphology were compared.   Skeletal muscle index as a predictor of HF-related admission   To clarify the prognostic role of SMI, the ratio of HF-related admission in patients with SMI above the median value in the present study was compared with that of patients with SMI equal to or less than the median value using a Kaplan–Meier analysis. HF-related admissions were identified as new-onset decompensated HF or decompensation of chronic HF with symptoms that warranted admission   Results   The multivariate analysis revealed a significant correlation between peak VO2 and EI (r = -0.55) and peak VO2 and SMI (r = 0.49) Figure 2. The receiver operating characteristic curve analysis showed that the EI cut-off for peak VO2 <20 ml/kg/min was 0.386 [area under the curve (AUC), 0.77; sensitivity, 0.67; specificity 0.76], and the SMI cut-off was 7.6 kg/m2 (AUC, 0.78; sensitivity, 0.76; specificity 0.75).   Compared with patients who had biventricular morphology, patients with single ventricular morphology had a higher EI (mean, 0.381 vs. 0.387, respectively) and lower SMI (8.5 vs. 7.7, respectively), resulting in a lower peakVO2 (27.1 vs. 20.8, respectively).   Fig. 2. Receiver operating characteristic curve analysis of bioelectrical impedance parameters for predicting reduced exercise capacity (defined as peak VO2 under 20 ml/kg/ min) (A, edema index; B, skeletal muscle index). AUC, area under the receiver operating characteristic curve.   The Kaplan–Meier analysis showed that a low SMI was associated with an increased risk of future heart failure-related admissions. (Figure 3 below)   Fig. 3. The Kaplan–Meier analysis of the incidence of heart failure-related admissions of patients with biventricular (left) or single ventricular (right) morphology by SMI. The red line shows the patients with a high SMI. The blue line shows those with a low SMI. HF, heart failure; SMI, skeletal muscle index.   Conclusions:   SMI determined by BIA is a determinant of exercise capacity and can be used as a prognostic predictor in patients with CHD.          

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Accessing Femoral Arteries Less than 3 mm in Diameter is Associated with Increased Incidence of Loss of Pulse Following Cardiac Catheterization in Infants

Accessing Femoral Arteries Less than 3 mm in Diameter is Associated with Increased Incidence of Loss of Pulse Following Cardiac Catheterization in Infants Sachin Tadphale 1, Thomas Yohannan 2, Travis Kauffmann 2, Vinod Maller 3, Vijaykumar Agrawal 3, Hannah Lloyd 4, B...

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Skeletal muscle index determined by bioelectrical impedance analysis is a determinant of exercise capacity and a prognostic predictor in patients with congenital heart disease

Skeletal muscle index determined by bioelectrical impedance analysis is a determinant of exercise capacity and a prognostic predictor in patients with congenital heart disease.   Sato M, Inai K, Asagai S, Harada G, Shimada E, Sugiyama H.J Cardiol. 2020 May...

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Modified Lung Ultrasound Examinations in Assessment and Monitoring of Positive End-Expiratory Pressure-Induced Lung Reaeration in Young Children With Congenital Heart Disease Under General Anesthesia.

Wu L, Hou Q, Bai J, Zhang J, Sun L, Tan R, Zhang M, Zheng J. Pediatr Crit Care Med. 2019 May;20(5):442-449. doi: 10.1097/PCC.0000000000001865. PMID:  31058784 Similar articles   Take Home Points: The most prevalent region of post-intubation atelectasis in...

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