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Congenital heart disease in adults: Assessmentof functional capacity using cardiopulmonary exercise testing.

Rev Port Cardiol. 2018 May 15;:

Authors: Aguiar Rosa S, Agapito A, Soares RM, Sousa L, Oliveira JA, Abreu A, Silva AS, Alves S, Aidos H, Pinto FF, Ferreira RC

AIM: The aim of the study was to compare functional capacity in different types of congenital heart disease (CHD), as assessed by cardiopulmonary exercise testing (CPET).
METHODS: A retrospective analysis was performed of adult patients with CHD who had undergone CPET in a single tertiary center. Diagnoses were divided into repaired tetralogy of Fallot, transposition of the great arteries (TGA) after Senning or Mustard procedures or congenitally corrected TGA, complex defects, shunts, left heart valve disease and right ventricular outflow tract obstruction.
RESULTS: We analyzed 154 CPET cases. There were significant differences between groups, with the lowest peak oxygen consumption (VO2) values seen in patients with cardiac shunts (39% with Eisenmenger physiology) (17.2±7.1ml/kg/min, compared to 26.2±7.0ml/kg/min in tetralogy of Fallot patients; p<0.001), the lowest percentage of predicted peak VO2 in complex heart defects (50.1±13.0%) and the highest minute ventilation/carbon dioxide production slope in cardiac shunts (38.4±13.4). Chronotropism was impaired in patients with complex defects. Eisenmenger syndrome (n=17) was associated with the lowest peak VO2 (16.9±4.8 vs. 23.6±7.8ml/kg/min; p=0.001) and the highest minute ventilation/carbon dioxide production slope (44.8±14.7 vs. 31.0± 8.5; p=0.002). Age, cyanosis, CPET duration, peak systolic blood pressure, time to anaerobic threshold and heart rate at anaerobic threshold were predictors of the combined outcome of all-cause mortality and hospitalization for cardiac cause.
CONCLUSION: Across the spectrum of CHD, cardiac shunts (particularly in those with Eisenmenger syndrome) and complex defects were associated with lower functional capacity and attenuated chronotropic response to exercise.

PMID: 29776810 [PubMed - as supplied by publisher]

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Arrhythmias in adult patients with congenital heart disease and pulmonary arterial hypertension.

Heart. 2018 May 18;:

Authors: Drakopoulou M, Nashat H, Kempny A, Alonso-Gonzalez R, Swan L, Wort SJ, Price LC, McCabe C, Wong T, Gatzoulis MA, Ernst S, Dimopoulos K

OBJECTIVES: Approximately 5%-10% of adults with congenital heart disease (CHD) develop pulmonary arterial hypertension (PAH), which affects life expectancy and quality of life. Arrhythmias are common among these patients, but their incidence and impact on outcome remains uncertain.
METHODS: All adult patients with PAH associated with CHD (PAH-CHD) seen in a tertiary centre between 2007 and 2015 were followed for new-onset atrial or ventricular arrhythmia. Clinical variables associated with arrhythmia and their relation to mortality were assessed using Cox analysis.
RESULTS: A total of 310 patients (mean age 34.9±12.3 years, 36.8% male) were enrolled. The majority had Eisenmenger syndrome (58.4%), 15.2% had a prior defect repair and a third had Down syndrome. At baseline, 14.2% had a prior history of arrhythmia, mostly supraventricular arrhythmia (86.4%). During a median follow-up of 6.1 years, 64 patients developed at least one new arrhythmic episode (incidence 3.47% per year), mostly supraventricular tachycardia or atrial fibrillation (78.1% of patients). Arrhythmia was associated with symptoms in 75.0% of cases. The type of PAH-CHD, markers of disease severity and prior arrhythmia were associated with arrhythmia during follow-up. Arrhythmia was a strong predictor of death, even after adjusting for demographic and clinical variables (HR 3.41, 95% CI 2.10 to 5.53, p<0.0001).
CONCLUSIONS: Arrhythmia is common in PAH-CHD and is associated with an adverse long-term outcome, even when managed in a specialist centre.

PMID: 29776964 [PubMed - as supplied by publisher]

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Ambulatory Intravenous Inotropic Support and or Levosimendan in Pediatric and Congenital Heart Failure: Safety, Survival, Improvement, or Transplantation.

Pediatr Cardiol. 2018 May 18;:

Authors: Apostolopoulou SC, Vagenakis GA, Tsoutsinos A, Kakava F, Rammos S

End-stage heart failure (HF) frequently needs continuous inotropic support in hospital and has high morbidity and mortality in absence of heart transplantation. This study reports outcome, efficacy, and safety of continuous ambulatory inotropes (AI) and/or periodic levosimendan (LS) infusions in pediatric HF patients. The study included 27 patients, median age 9.4 (0.1-26.1) years, with severe HF (6 myocarditis, 13 dilated cardiomyopathy, 2 restrictive cardiomyopathy, 6 repaired congenital heart disease). Dobutamine and milrinone AI were administered in 21 patients through a permanent central catheter for median duration 1.0 (0.3-3.7) years. Additionally, 14 AI patients and the remaining 6 study patients received periodic LS infusions for median duration 1.1 (0.2-4.2) years. During median follow-up 2.1 (0.3-21.3) years, 4 patients died of worsening HF after 0.8-2.1 years AI, 6 patients underwent heart transplantation with only 3 survivors, while the rest remained stable out of the hospital with complications 4 line infections treated with antibiotics and 4 catheter reinsertions due to dislodgement. Severe pulmonary hypertension was reversed with AI in 2 patients, allowing successful heart-only transplantation. Therapy with AI was discontinued after 1.4-0.4 years in 6 improved myocarditis and 3 cardiomyopathy patients without deterioration. In conclusion, prolonged AI and/or LS infusions in HF are safe and beneficial even in small infants, allowing stabilization and reasonable social and family life out of the hospital. It may provide precious time for heart transplantation or myocardial remodeling, improvement, and possible discontinuation even after long periods of support.

PMID: 29777282 [PubMed - as supplied by publisher]

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Electrophysiology and structural interventions in adults with congenital heart disease: Comparison of combined versus separate procedures.

J Cardiovasc Electrophysiol. 2018 May 18;:

Authors: Lindsay I, Nik-Ahd F, Aboulhosn JA, Moore JP

BACKGROUND: Electrophysiologic (EP) and structural interventions in adult congenital heart disease (ACHD) are typically completed during separate hospital encounters. With planning/coordination, these cases can be combined.
OBJECTIVES: We hypothesized that this integrated approach would yield patient and health system benefits.
METHODS: Consecutive ACHD patients undergoing combined interventions were matched to controls with identical but separate procedures. Primary endpoints of total hospital length of stay and cost were compared.
RESULTS: Sixty-six combined cases and 120 controls were identified (45% male, mean age 36.2 ± 14.2 years). The most common diagnoses were Fontan (27%), tetralogy of Fallot (23%), and transposition complexes (20%). The most common EP procedure was catheter ablation (n = 30) followed by electrophysiologic study (n = 13); the most common structural intervention was transcatheter valve replacement (n = 16) followed by angioplasty/stenting (n = 14). Compared to controls, cases showed shorter anesthesia duration (323 [IQR 238-405] vs 355 minutes [270-498], p = 0.06), smaller contrast dose (130 [50-189] vs 177 mL [94-228], p = 0.045), fewer venipunctures (4 [3-4] vs 6 [5-7], p < 0.001), and fewer work days missed (2 [2-5] vs 4 [4-6], p < 0.001). There was shorter hospital stay (30 [19-35] vs 38 hours [26-50], p = 0.023) and a 37% reduction in hospital charges ($117,894 vs $187,648; p = 0.039) and 27% reduction in payments ($65,757 vs $88,859; p = 0.016), persisting after adjustment for group differences. There were no significant differences in number of complications or efficacy.
CONCLUSIONS: There appear to be advantages to combining ACHD interventional procedures that include reductions in hospital length of stay and cost, without detectable difference in procedural outcome. This article is protected by copyright. All rights reserved.

PMID: 29777556 [PubMed - as supplied by publisher]

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Pulmonary valve replacement after repaired Tetralogy of Fallot.

Gen Thorac Cardiovasc Surg. 2018 May 19;:

Authors: Tatewaki H, Shiose A

In this review article, we describe pulmonary valve replacement (PVR) late after repaired Tetralogy of Fallot (TOF). Since the introduction of surgical intervention for patients with TOF in 1945, surgical management of TOF has dramatically improved early survival with mortality rates, less than 2-3%. However, the majority of these patients continue to experience residual right ventricular outflow tract pathology, most commonly pulmonary valve regurgitation (PR). The patients are generally asymptomatic during childhood and adolescence and, however, are at risk for severe PR later which can result in exercise intolerance, heart failure, arrhythmias, and sudden death. While it has been shown that PVR improves symptoms and functional status in these patients, the optimal timing and indications for PVR after repaired TOF are still debated. This article reviews the current state of management for the patient with PR after repaired TOF.

PMID: 29779123 [PubMed - as supplied by publisher]

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Atrial arrhythmia after transcatheter closure of secundum atrial septal defects in patients ≥40 years of age.

Europace. 2017 Aug 01;19(8):1322-1326

Authors: Duong P, Ferguson LP, Lord S, Murray S, Shepherd E, Bourke JP, Crossland D, O'Sullivan J

Aim: Data on arrhythmia outcome following device closure of atrial septal defect (ASD) are lacking. This study provides medium-term follow-up data on atrial arrhythmias in patients who were ≥40 years of age at the time of transcatheter ASD closure.
Methods and results: It is a retrospective review. Mean age of the 159 patients was 57 years. Median follow-up was 3.6 years (range 6 months-10.9 years). Patients were classified, according to arrhythmia status prior to ASD closure, into Group I, no history of atrial arrhythmia (n = 119, mean age 55.5 years); Group II, paroxysmal atrial arrhythmia (n = 18, mean age 55.7 years); and Group III, persistent atrial fibrillation (n = 22, mean age 65.7 years). Group III patients were significantly older, had larger left atrial size, and had higher mean pulmonary arterial pressure than Group I and II patients (P < 0.001). Prior to closure, radiofrequency ablation was carried out in 12/18 (66%) of Group II and 3/22 (14%) of Group III. After device closure, 7 patients (6%) of Group I developed new atrial fibrillation. Fifty per cent (9/18) of Group II but only 9% (2/22) of Group III were in sinus rhythm on follow-up.
Conclusion: Device closure alone in patients with persistent atrial arrhythmia is not likely to restore sinus rhythm in the medium term. New atrial arrhythmia occurred in 6% of patients who were in sinus rhythm prior to device closure. At least 50% of the patients with paroxysmal atrial arrhythmia continue to have significant atrial arrhythmia following device closure, and the role of ablation prior to closure in patients with a history of arrhythmia requires refinement.

PMID: 27702856 [PubMed - indexed for MEDLINE]

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Initial clinical experience with the GORE® CARDIOFORM ASD occluder for transcatheter atrial septal defect closure.

Catheter Cardiovasc Interv. 2017 Sep 01;90(3):495-503

Authors: de Hemptinne Q, Horlick EM, Osten MD, Millán X, Tadros VX, Pighi M, Gonzalez Barlatey F, Alnasser SM, Miró J, Asgar AW, Ibrahim R

OBJECTIVES: To report the initial clinical experience with a novel atrial septal defect (ASD) closure device, the GORE® CARDIOFORM ASD Occluder (GCO).
BACKGROUND: Transcatheter closure has become the treatment of choice for secundum ASD. A wide range of occluder devices are available, but concern has been raised about the risk of cardiac erosion associated with rigid devices and future access to the left atrium METHODS: Retrospective chart review of patients treated with the GCO at three Canadian centers. Primary outcomes were procedural success and residual shunting at follow-up, as well as 30-day major adverse events. Secondary outcomes included new onset atrial arrhythmias, wire frame fractures (WFF), and all cause mortality. Clinical, echocardiographic, procedural data, and follow-up outcome variables were collected in each participating hospital.
RESULTS: Between February and December 2015, 26 patients (5 children and 21 adults) underwent transcatheter ASD closure with the GCO and were included in the study cohort. Procedural success was achieved in 22 of 26 patients (85%) and no major procedural complications were observed. Two patients (8%) presented new onset atrial tachyarrhythmia during early follow-up (0-30 days). Follow-up echocardiography (median of 119 days [IQR: 92-146]) demonstrated no residual shunt in all implanted patients. After a median clinical follow-up of 174 days (IQR: 135-239), one patient died of an unrelated cause, there were no documented major adverse cardiovascular events. Fluoroscopic imaging of the device was performed in 20 patients (91%), and WFF was noted in five cases. No clinical consequence or device dysfunction was observed in these patients.
CONCLUSIONS: In this first-in-man multicenter study, the GCO was safe and effective for ASD closure, with no major adverse events or residual shunt at follow-up. © 2017 Wiley Periodicals, Inc.

PMID: 28128523 [PubMed - indexed for MEDLINE]

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Right ventricular remodelling after transcatheter pulmonary valve implantation.

Catheter Cardiovasc Interv. 2017 Sep 01;90(3):407-417

Authors: Pagourelias ED, Daraban AM, Mada RO, Duchenne J, Mirea O, Cools B, Heying R, Boshoff D, Bogaert J, Budts W, Gewillig M, Voigt JU

OBJECTIVES: To define the optimal timing for percutaneous pulmonary valve implantation (PPVI) in patients with severe pulmonary regurgitation (PR) after Fallot's Tetralogy (ToF) correction.
BACKGROUND: PPVI among the aforementioned patients is mainly driven by symptoms or by severe right ventricular (RV) dilatation/dysfunction. The optimal timing for PPVI is still disputed.
METHODS: Twenty patients [age 13.9 ± 9.2 years, (range 4.3-44.9), male 70%] with severe PR (≥3 grade) secondary to previous correction of ToF, underwent Melody valve (Medtronic, Minneapolis, MN) implantation, after a pre-stent placement. Full echocardiographic assessment (traditional and deformation analysis) and cardiovascular magnetic resonance evaluation were performed before and at 3 months after the intervention. 'Favorable remodelling' was considered the upper quartile of RV size decrease (>20% in 3 months).
RESULTS: After PPVI, indexed RV effective stroke volume increased from 38.4 ± 9.5 to 51.4 ± 10.7 mL/m2 , (P = 0.005), while RV end-diastolic volume and strain indices decreased (123.1 ± 24.1-101.5 ± 18.3 mL/m2 , P = 0.005 and -23.5 ± 2.5 to -21 ± 2.5%, P = 0.002, respectively). After inserting pre-PPVI clinical, RV volumetric and deformation parameters in a multiple regression model, only time after last surgical correction causing PR remained as significant regressor of RV remodelling [R2  = 0.60, beta = 0.387, 95%CI(0.07-0.7), P = 0.019]. Volume reduction and functional improvement were more pronounced in patients treated with PPVI earlier than 7 years after last RV outflow tract (RVOT) correction, reaching close-to-normal values.
CONCLUSIONS: Early PPVI (<7 years after last RVOT operation) is associated with a more favorable RV reverse remodelling toward normal range and should be considered, before symptoms or RV damage become apparent. © 2017 Wiley Periodicals, Inc.

PMID: 28296032 [PubMed - indexed for MEDLINE]

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Impact on survival of warfarin in patients with pulmonary arterial hypertension receiving subcutaneous treprostinil.

Cardiovasc Ther. 2017 Oct;35(5):

Authors: Ascha M, Zhou X, Rao Y, Minai OA, Tonelli AR

INTRODUCTION: Anticoagulation is a common treatment modality in patients with pulmonary arterial hypertension (PAH). Further studies are needed to appropriately assess the risk/benefit ratio of anticoagulation, particularly in PAH patients receiving PAH-specific therapies.
AIMS: We use observational long-term data on PAH patients treated with subcutaneous (SQ) treprostinil from a large open-label study. Patients were followed for up to 4 years. The use of warfarin and bleeding events were recorded.
RESULTS: At total of 860 patients (age [mean±SD] 46±15 years, 76% female, 83% Caucasian, 49% idiopathic PAH, and 76% New York Heart Association [NYHA] functional class III) were included. All patients received SQ treprostinil (15% also other pulmonary hypertension [PH]-therapies) and 590 (69%) received warfarin during the study. The proportions of women, African American, and idiopathic pulmonary hypertension (IPAH) patients were higher in the group receiving warfarin. A higher proportion of patients with congenital heart disease and portopulmonary hypertension did not receive warfarin. There were no differences in unadjusted long-term survival between PAH patients receiving warfarin or not (log-rank test, P value=.69), even when only considering idiopathic PAH (P=.32). In addition, no difference was found in adjusted long-term survival both in PAH (P=.84) and idiopathic PAH patients (P=.44) based on the use of warfarin. Furthermore, no survival difference based on the use of warfarin were noted between propensity score-matched PAH patients (P=.37).
CONCLUSIONS: Long-term anticoagulation with warfarin was not associated with any significant effect on survival in PAH or idiopathic PAH patients treated with SQ treprostinil.

PMID: 28643420 [PubMed - indexed for MEDLINE]

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Midterm to long-term safety and efficacy of self-expandable nitinol stent implantation for coarctation of aorta in adults.

Catheter Cardiovasc Interv. 2017 Sep 01;90(3):425-431

Authors: Haji Zeinali AM, Sadeghian M, Qureshi SA, Ghazi P

PURPOSE: Endovascular treatment of coarctation of aorta (CoA) by self-expandable Nitinol stents is one of the recognized treatment methods and may be an alternative to surgery or balloon-expandable stent implantation for CoA but there is little information about midterm to long term results of self-expandable stents.
METHODS: Sixty-two patients with CoA (40 men), with a mean age of 30.7 ± 11 years, (range 17-63 years) underwent stent implantation with Optimed self-expandable Nitinol stents between 2005 and 2014. Successful outcome was defined as peak systolic pressure gradient ≤20 mmHg after stent implantation. The patients were followed-up clinically and by echocardiography and in patients, in whom there was suspicion of recoarctation, CT angiography or recatheterization was performed.
RESULTS: 65 stents were successfully implanted in all 62 patients. Peak systolic pressure gradient decreased from mean 62.4 ± 18 mmHg (range 35-100 mmHg) to mean 2.8 ± 5 mmHg (range 0-15 mmHg; P < 0.001). Stent displacement occurred in 3 patients during the procedure. These were managed successfully by an overlapping second stent. None of the patients had major complications such as aortic dissection, rupture, or vascular access problems. In follow up, only three patients had recoarctation, and two of these were managed successfully by balloon redilation or further stenting 16 and 18 months after the first procedure and one patient refused reintervention. There were two deaths, unrelated to the procedure, 12 and 78 months after the initial intervention. Follow-up of a mean of 45.5 ± 17 months (range 12-105 months) demonstrated no evidence of aneurysm formation or stent fracture.
CONCLUSIONS: Self-expandable nitinol stents for the treatment of native and recurrent CoA is safe and has good efficacy with acceptable midterm to long-term outcome.

PMID: 28707350 [PubMed - indexed for MEDLINE]