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1.
Int J Cardiol ; 322: 129-134, 2021 Jan 01.
Artigo em Inglês | MEDLINE | ID: mdl-32805330

RESUMO

BACKGROUND: Patients with systemic right ventricle (sRV), including transposition of great arteries (TGA) after atrial switch procedure and congenitally corrected transposition of great arteries (ccTGA), may require anticoagulation for thromboembolism (TE) prevention. In the absence of data on non-vitamin K antagonist oral anticoagulants (NOACs), vitamin K antagonists (VKAs) remain the agent of choice. We investigated the safety, efficacy and feasibility of NOACs treatment in adults with sRV in a worldwide study. METHODS: This is an international multicentre prospective study, using data from the NOTE registry on adults with sRV taking NOACs between 2014 and 2019. The primary endpoints were TE and major bleeding (MB). The secondary endpoint was minor bleeding. RESULTS: A total of 76 patients (42.5 ±â€¯10.0 years, 76% male) with sRV (74% TGA, 26% ccTGA) on NOACs were included in the study. During a median follow-up of 2.5 years (IQR1.5-3.9), TE events occurred in 3 patients (4%), while no MB episodes were reported. Minor bleeding occurred in 9 patients (12%). NOAC treatment cessation rate was 1.4% (95%CI:0.3-4%) during the first year of follow-up. All the patients with TE events had a CHA2DS2-VASc score ≥ 2 and impaired sRV systolic function at baseline. The total incidence of major events during follow-up was significantly lower compared to historical use of VKAs or aspirin before study inclusion (1.4% (95%CI:0.29-4%) vs 6,9% (95%CI:2.5-15.2%); p = .01). CONCLUSIONS: In this prospective study, NOACs appear to be well-tolerated, with excellent efficacy and safety at mid-term in patients with sRV.

2.
Can J Cardiol ; 2020 Nov 24.
Artigo em Inglês | MEDLINE | ID: mdl-33246004

RESUMO

BACKGROUND: Atrial tachyarrhythmias (AAs) are the main source of morbidity and mortality in adults with congenital heart disease (ACHD). Direct current cardioversion (DCCV) is an effective method to acutely terminate AAs, however, many patients require repeat DCCV. Little is known about the impact of radiofrequency catheter ablation (RFCA) of AAs on the incidence of repeat DCCV in ACHD. The purpose of this study was to evaluate the impact of RFCA on the incidence of DCCV in ACHD patients. METHODS: A total of 157 ACHD undergoing DCCV in our hospital from 2011 to 2018 (female n=76 [48.4%], mean age 37.8±12.5), were reviewed. The median follow-up period was 31.8 months (IQR, 16.3-55.1). RESULTS: Out of the total of 157 patients, 102 (65.0%) underwent RFCA for AAs, and 55 (35.0%) patients were treated without RFCA. Successful RFCA with termination of AAs during ablation was 62.7%. More than half of the patients had complex forms of congenital heart disease (62.4%). During follow-up, 57 (55.9%) patients who had RFCA developed recurrence of AAs, and 36 (35.2%) patients underwent repeat DCCV. Thirty-three (60.0%) out of 55 patients without RFCA required repeat cardioversion. Compared to patients without RFCA, RFCA significantly reduced the need for repeat DCCV by 40% (hazard ratio [HR] 0.60, 95% CI 0.23-0.80 P=0.009). In multivariate analysis, successful RFCA was associated with reduced risk of DCCV (HR 0.41, 95% CI 0.19-0.92, P=0.031). CONCLUSION: AAs remain common despite RFCA in ACHD patients. Nevertheless, RFCA is associated with a marked reduction in the need for repeat DCCV.

3.
Heart ; 2020 Oct 28.
Artigo em Inglês | MEDLINE | ID: mdl-33115764

RESUMO

BACKGROUND: Patients with Fontan circulation are known to be at high risk for developing atrial tachyarrhythmias (AAs). Our objective was to examine the efficacy and safety of amiodarone in the management of ATs in adult Fontan patients. METHODS: Primary outcomes of this single-centre, retrospective study included freedom from AAs and incidence of adverse effects of amiodarone on Fontan patients. Heart failure (HF) events and composite outcomes of death from any cause, Fontan revision and heart transplantation were evaluated as secondary outcomes. Predictors of HF and discontinuing amiodarone were also evaluated. RESULTS: A total of 61 patients (mean age 31.6±11.3 years, 40.9% female), who were treated with amiodarone in between 1995 and 2018, were included. AAs free survival at 1, 3 and 5 years were 76.2%, 56.9% and 30.6%, respectively. During a median follow-up of 50.5 months, 34 (55.7%) patients developed side effects, and 20 (32.8%) patients discontinued amiodarone due to side effects. Thyroid dysfunction was the most common side effect (n=26, 76.5%), amiodarone-induced thyrotoxicosis (AIT) (n=16, 27.1%) being most common thyroid dysfunction. Young age (age <28.5 years) was associated with discontinuing amiodarone (HR 5.50, 95% CI 1.19 to 25.4, p=0.029). AIT significantly increased risk of HF (HR 4.82, 95% CI 1.71 to 13.6, p=0.003). CONCLUSIONS: Short-term efficacy of amiodarone in Fontan physiology is acceptable. However, long-term administration is associated with a reduction of efficacy and a significant prevalence of non-cardiac side effects. AIT is associated with exacerbation of HF. The judicious use of amiodarone administration should be considered in this population.

4.
Heart ; 2020 Jul 06.
Artigo em Inglês | MEDLINE | ID: mdl-32748799

RESUMO

OBJECTIVES: Although a life-preserving surgery for children with single ventricle physiology, the Fontan palliation is associated with striking morbidity and mortality with advancing age. Our primary objective was to evaluate the impact of non-invasive, external, thoraco-abdominal ventilation on pulmonary blood flow (PBF) and cardiac output (CO) as measured by cardiovascular magnetic resonance (CMR) imaging in adult Fontan subjects. METHODS: Adults with a dominant left ventricle post-Fontan palliation (lateral tunnel or extracardiac connections) and healthy controls matched by sex and age were studied. We evaluated vascular flows using phase-contrast CMR imaging during unassisted breathing, negative pressure ventilation (NPV) and biphasic ventilation (BPV). Measurements were made within target vessels (aorta, pulmonary arteries, vena cavae and Fontan circuit) at baseline and during each ventilation mode. RESULTS: Ten Fontan subjects (50% male, 24.5 years (IQR 20.8-34.0)) and 10 matched controls were studied. Changes in PBF and CO, respectively, were greater following BPV as compared with NPV. In subjects during NPV, PBF increased by 8% (Δ0.20 L/min/m2 (0.10-0.53), p=0.011) while CO did not change significantly (Δ0.17 L/min/m2 (-0.11-0.23), p=0.432); during BPV, PBF increased by 25% (Δ0.61 L/min/m2 (0.20-0.84), p=0.002) and CO increased by 16% (Δ0.47 L/min/m2 (0.21-0.71), p=0.010). Following BPV, change in PBF and CO were both significantly higher in subjects versus controls (0.61 L/min/m2 (0.2-0.84) vs -0.27 L/min/m2 (-0.55-0.13), p=0.001; and 0.47 L/min/m2 (0.21-0.71) vs 0.07 L/min/m2 (-0.47-0.33), p=0.034, respectively). CONCLUSION: External ventilation acutely augments PBF and CO in adult Fontan subjects. Confirmation of these findings in larger populations with longer duration of ventilation and extended follow-up will be required to determine sustainability of haemodynamic effects.

5.
Can J Cardiol ; 2020 Jun 06.
Artigo em Inglês | MEDLINE | ID: mdl-32522524

RESUMO

BACKGROUND: Implantable cardioverter-defibrillators (ICDs) have been proven to prevent sudden cardiac death in adult congenital heart disease (ACHD) patients. Although the left side is chosen by default, implantation from the right side is often required. However, little is known about the efficacy and safety of right-sided ICDs in ACHD patients. METHODS: In this study we reviewed a total of 191 ACHD patients undergoing ICD/cardioverter resynchronisation therapy-defibrillator (CRT-D) implantation at our hospital between 2001 and 2019 (134 men and 57 women; age [mean ± standard deviation], 41.5 ± 14.8 years). RESULTS: Twenty-seven patients (14.1%) had right-sided devices. The most common causes of right-sided implantation were persistent left superior vena cava and vein occlusion (37.0%). Although procedure time (202.8 ± 60.5 minutes vs 143.8 ± 69.1 minutes, P = 0.008) was longer and the procedural success was lower (92.6% vs 99.4%, P = 0.008) for right-sided devices, no difference in R-wave and pacing threshold were noted. Among the 47 patients (24.6%) who underwent defibrillation threshold testing (DFT), no difference in DFT was observed (25.2 ± 5.3 J vs 23.8 ± 4.1 J, P = 0.460). During the median follow-up of 42.4 months, appropriate ICD therapy was observed in 5 (18.5%) and 30 (18.3%) patients for right- and left-sided ICDs/CRTDs, respectively (P = 0.978). No significant difference was seen in complications between them. CONCLUSIONS: Implantation of an ICD on the right side is technically challenging, but it is feasible as an alternative approach for ACHD patients with contraindications to left-sided device implantation.

6.
Int J Cardiol ; 301: 127-134, 2020 02 15.
Artigo em Inglês | MEDLINE | ID: mdl-31604655

RESUMO

INTRODUCTION: Arrhythmia contributes significantly to morbidity and mortality of patients with congenital heart disease (CHD) or cardiomyopathy (CMP). It also has the potential to worsen symptoms and is particularly detrimental to patients with advanced heart failure awaiting cardiac transplantation. We report our experience using catheter ablation to treat recurrent arrhythmia in patients with CHD or CMP considered for transplantation. METHODS: Five consecutive patients (3 female, mean age 47.8 ±â€¯12.8 years) with complex CHD or CMP (tricuspid atresia, mitral atresia, double inlet left ventricle, arrhythmogenic right ventricular cardiomyopathy, left ventricular non-compaction) presented with either atrial (n = 3) or ventricular (n = 2) arrhythmias. All ablations were guided by three-dimensional (3D) electro-anatomical mapping, plus remote magnetic navigation in 3 patients. RESULTS: Patients underwent a median of 2 ablation procedures for a total number of 26 tachycardias. None of the 5 patients experienced further arrhythmia at a median of 939 days (range 4-1375) from their last ablation. During a median follow up of 31 months (range 1-70), three patients underwent successful transplantation at 1375, 1062 and 321 days following their last ablation. One patient with a Fontan circulation died from hepatic cancer and one from end-stage heart failure despite urgent transplant listing. CONCLUSIONS: Catheter ablation is feasible in complex cardiac patients considered for heart transplantation and should be offered for rhythm management and patient optimization until a suitable donor is found.

7.
Heart ; 106(6): 455-461, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-31444268

RESUMO

OBJECTIVES: Cardiac surgery or catheter interventions are nowadays commonly performed to reduce volume loading of the right ventricle in adults with congenital heart disease. However, little is known, on the effect of such procedures on pre-existing tricuspid regurgitation (TR). We assessed the potential reduction in the severity of TR after atrial septal defect (ASD) closure and pulmonic valve replacement (PVR). METHODS: Demographics, clinical and echocardiographic characteristics of consecutive patients undergoing ASD closure or PVR between 2005 and 2014 at a single centre who had at least mild preoperative TR were collected and analysed. RESULTS: Overall, 162 patients (mean age at intervention 41.6±16.1 years, 38.3% male) were included: 101 after ASD closure (61 transcatheter vs 40 surgical) and 61 after PVR (3 transcatheter vs 58 surgical). Only 11.1% received concomitant tricuspid valve surgery (repair). There was significant reduction in the severity of TR in the overall population, from 38 (23.5%) patients having moderate or severe TR preoperatively to only 11 (6.8%) and 20 (12.3%) at 6 months and 12 months of follow-up, respectively (McNemar p<0.0001). There was a significant reduction in tricuspid valve annular diameter (p<0.0001), coaptation distance (p<0.0001) and systolic tenting area (p<0.0001). The reduction in TR was also observed in patients who did not have concomitant tricuspid valve (TV) repair (from 15.3% to 6.9% and 11.8% at 6 and 12 months, respectively, p<0.0001). On multivariable logistic regression including all univariable predictors of residual TR at 12 months, only RA area remained in the model (OR 1.2, 95% CI 1.04 to 1.37, p=0.01). CONCLUSIONS: ASD closure and PVR are associated with a significant reduction in tricuspid regurgitation, even among patients who do not undergo concomitant tricuspid valve surgery.


Assuntos
Comunicação Interatrial/complicações , Comunicação Interatrial/cirurgia , Valva Pulmonar/cirurgia , Insuficiência da Valva Tricúspide/complicações , Adulto , Procedimentos Cirúrgicos Cardíacos , Feminino , Doenças das Valvas Cardíacas/complicações , Doenças das Valvas Cardíacas/cirurgia , Implante de Prótese de Valva Cardíaca , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Índice de Gravidade de Doença , Insuficiência da Valva Tricúspide/cirurgia
8.
Int J Cardiol ; 299: 131-135, 2020 01 15.
Artigo em Inglês | MEDLINE | ID: mdl-31371115

RESUMO

BACKGROUND: Pulmonary arterial hypertension (PAH), is a rare and progressive disease with a high morbidity and mortality. Prostanoid pulmonary vasodilators are the most effective treatment for idiopathic and connective tissue associated PAH. Nonetheless, data examining their safety and efficacy in patients with Eisenmenger syndrome the most severe form of PAH, that is, related to cyanotic congenital heart disease (CHD-PAH) remains limited. AIM: To evaluate safety and the clinical efficacy of nebulised iloprost in patients with Eisenmenger syndrome who are on maximum background oral PAH therapy. METHODS: This pilot study was a randomised, double-blind, placebo-controlled, cross-over study. Patients were randomised to receive nebulised placebo or iloprost for 12 weeks and were then crossed over, with a 7-14-day washout. The primary endpoint was a change in 6-minute walk distance (6MWD). RESULTS: Sixteen patients (11 females, aged 47.3 ±â€¯9.8 year) were recruited, twelve completed the study. All were in WHO-FC III, with a resting oxygen saturation of 84 [81-87] % and a median 6MWD of 290 [260-300] m. There was no significant difference in the primary endpoint between nebulised iloprost (0[-4-9]m) and placebo (10 [-15-51]m), p = 0.58. There were no safety concerns with nebulised iloprost. CONCLUSIONS: Our pilot study provides preliminary evidence that the addition of nebulised iloprost to maximum oral PAH therapy did not improve the primary endpoint of 6MWD. Nebulised iloprost was well tolerated with no significant safety concerns in CHD-PAH.


Assuntos
Complexo de Eisenmenger/tratamento farmacológico , Complexo de Eisenmenger/fisiopatologia , Iloprosta/administração & dosagem , Nebulizadores e Vaporizadores , Vasodilatadores/administração & dosagem , Adulto , Estudos Cross-Over , Método Duplo-Cego , Complexo de Eisenmenger/diagnóstico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Projetos Piloto , Teste de Caminhada/métodos
11.
Heart ; 105(12): 932-937, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-30700516

RESUMO

OBJECTIVE: Pulmonary valve replacement (PVR) is often required in patients with congenital heart disease. We aimed to describe temporal trends in PVR in a nationwide English cohort between 1997 and 2014, survival and the need for re-PVR. METHODS: Patients were identified in the Hospital Episode Statistics Database. Survival data were retrieved from the UK Office for National Statistics. RESULTS: A total of 2733 patients underwent PVR (2845 procedures) over the study period. Median age at first procedure increased from 20.1 years in 1997-2005 to 24.7 years in 2006-2014. The annual number of PVRs increased from 23 in 1997 to 251 in 2014. Homografts were the most common choice in the early years, but the use of xenografts increased after 2005. During a median follow-up of 5.8 years, 176 patients died and 108 required redo PVR. Early (30 day) survival was 98% for all PVRs and was similar for all types of prostheses but longer-term mortality dropped to 92% at 10 years and 90% at 15 years. Age >16 years and percutaneous PVR were risk factors for death. The cumulative incidence for re-PVR at 10 years was 8% for all PVRs and 11% at 15 years. Risk factors for re-PVR were complex diagnosis, male gender and black ethnicity. CONCLUSION: There was a significant increase in the number of PVRs performed in England over the last two decades and a significant change in the type of prosthesis employed. While early mortality was low across the board, longer-term mortality was not negligible in this young population.


Assuntos
Implante de Prótese de Valva Cardíaca/métodos , Implante de Prótese de Valva Cardíaca/estatística & dados numéricos , Valva Pulmonar/cirurgia , Adolescente , Adulto , Inglaterra , Feminino , Implante de Prótese de Valva Cardíaca/tendências , Humanos , Masculino , Fatores de Tempo , Adulto Jovem
12.
Heart ; 105(8): 596-602, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30636220

RESUMO

BACKGROUND: Increased longevity in patients with congenital heart disease (CHD) is associated with late complications, mainly heart failure, which may not be amenable to redo surgery and become refractory to medical therapy and so, trigger referral for transplantation. We assessed the current role and future prospects of heart and heart-lung transplantation for patients with CHD in England. METHODS: We performed a retrospective analysis of hospital episodes for England for 1997-2015, identifying patients with a CHD code (ICD-10 'Q2xx.x'), who underwent heart or heart-lung transplantation. RESULTS: In total, 469 transplants (82.2% heart and 17.8% heart-lung) were performed in 444 patients. Half of patients transplanted had mild or moderate CHD complexity, this percentage increased with time (p=0.001). While overall, more transplantations were performed over the years, the proportion of heart-lung transplants declined (p<0.0001), whereas the proportion of transplants performed in adults remained static. Mortality was high during the first year, especially after heart-lung transplantation, but remained relatively low thereafter. Older age and heart-lung transplantation were strong predictors of death. While an increase in CHD transplants is anticipated, actual numbers in England seem to lag behind the increase in CHD patients with advanced heart failure. CONCLUSIONS: The current and future predicted increase in the numbers of CHD transplants does not appear to parallel the expansion of the CHD population, especially in adults. Further investment and changes in policy should be made to enhance the number of donors and increase CHD transplant capacity to address the increasing numbers of potential CHD recipients and optimise transplantation outcomes in this growing population.


Assuntos
Cardiopatias Congênitas , Insuficiência Cardíaca , Transplante de Coração , Transplante de Coração-Pulmão , Complicações Pós-Operatórias , Reoperação , Adulto , Criança , Bases de Dados Factuais/estatística & dados numéricos , Progressão da Doença , Inglaterra/epidemiologia , Feminino , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/epidemiologia , Cardiopatias Congênitas/terapia , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/cirurgia , Transplante de Coração/efeitos adversos , Transplante de Coração/métodos , Transplante de Coração/estatística & dados numéricos , Transplante de Coração-Pulmão/efeitos adversos , Transplante de Coração-Pulmão/métodos , Transplante de Coração-Pulmão/estatística & dados numéricos , Humanos , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Mortalidade , Determinação de Necessidades de Cuidados de Saúde , Avaliação de Processos e Resultados em Cuidados de Saúde , Complicações Pós-Operatórias/mortalidade , Complicações Pós-Operatórias/cirurgia , Reoperação/métodos , Reoperação/estatística & dados numéricos , Estudos Retrospectivos , Fatores de Risco
13.
Int J Cardiol ; 280: 61-66, 2019 04 01.
Artigo em Inglês | MEDLINE | ID: mdl-30477927

RESUMO

OBJECTIVES: To describe the characteristics and long-term outcome of a large adult cohort with pulmonary atresia. BACKGROUND: Patients with pulmonary atresia (PA) are a heterogeneous population in terms of anatomy, physiology and surgical history, and their management during adulthood remains challenging. METHODS: Data on all patients with PA followed in our center between January 2000 and March 2015 were recorded. Patients were classified into the following groups: PA with ventricular septal defect (PA-VSD, 1), PA with intact ventricular septum (PA-IVS, 2) and other miscellaneous PA (PA-other, 3). RESULTS: Two-hundred twenty-seven patients with PA were identified, 66.1% female, mean age 25.5 ±â€¯8.7 years. Over a median follow-up of 8.8 years, 49 (21.6%) patients had died: heart failure (n = 21, 42.8%) and sudden cardiac death (n = 8, 16.3%) were the main causes. There was no significant difference in mortality between the 3 Groups (p = 0.12) or between repaired and unrepaired patients in Group 1 (p = 0.16). Systemic ventricular dysfunction and resting oxygen saturations were the strongest predictors of mortality. Additionally, 116 (51%) patients were hospitalized, driven mainly by the need for invasive procedures, heart failure and arrhythmias. CONCLUSIONS: Adult survivors with pulmonary atresia have a high morbidity and mortality irrespective of underlying cardiac anatomy and previous reparative or palliative surgery. We present herewith predictors of outcome in adult life that may assist with their tertiary adult congenital care.


Assuntos
Hospitalização/tendências , Atresia Pulmonar/mortalidade , Atresia Pulmonar/cirurgia , Adolescente , Adulto , Feminino , Seguimentos , Humanos , Masculino , Morbidade , Mortalidade/tendências , Atresia Pulmonar/diagnóstico , Resultado do Tratamento , Adulto Jovem
14.
J Am Coll Cardiol ; 72(22): 2778-2788, 2018 12 04.
Artigo em Inglês | MEDLINE | ID: mdl-30497564

RESUMO

Echocardiography is the mainstay in screening for pulmonary hypertension (PH). International guidelines suggest echocardiographic parameters for suspecting PH, but these may not apply to many adults with congenital heart disease (ACHD). PH is relatively common in ACHD patients and can significantly affect their exercise capacity, quality of life, and prognosis. Identification of patients who have developed PH and who may benefit from further investigations (including cardiac catheterization) and treatment is thus extremely important. A systematic review and survey of experts from the United Kingdom and Ireland were performed to assess current knowledge and practice on echocardiographic screening for PH in ACHD. This paper presents the findings of the review and expert statements on the optimal approaches when using echocardiography to assess ACHD patients for PH, with particular focus on major subgroups: patients with right ventricular outflow tract obstruction, patients with systemic right ventricles, patients with unrepaired univentricular circulation, and patients with tetralogy of Fallot with pulmonary atresia.


Assuntos
Ecocardiografia/métodos , Cardiopatias Congênitas/diagnóstico por imagem , Hipertensão Pulmonar/diagnóstico por imagem , Programas de Rastreamento/métodos , Ecocardiografia/tendências , Cardiopatias Congênitas/epidemiologia , Humanos , Hipertensão Pulmonar/epidemiologia , Imagem Cinética por Ressonância Magnética/métodos , Imagem Cinética por Ressonância Magnética/tendências , Programas de Rastreamento/tendências
16.
Int J Cardiol ; 272: 64-69, 2018 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-30153993

RESUMO

BACKGROUND: Measures of left ventricular (LV) systolic and diastolic function are known predictors of mortality after repair of tetralogy of Fallot. We aimed to characterise LV reverse remodelling achievable with ramipril therapy. METHODS AND RESULTS: A blinded post-hoc analysis of baseline and 6-month follow-up echocardiograms from the APPROPRIATE (ISRCTN: 97515585) randomised double-blinded placebo-controlled trial of ramipril therapy was performed in 64 patients: 32 in ramipril and 32 in placebo group. Tissue Doppler systolic and diastolic myocardial velocities, mitral inflow velocities and time intervals were measured. Left atrial area and left atrial emptying fraction were calculated. There was significant increase in long axis shortening mean (standard deviation); MAPSE [1.9 (4.2) mm vs -0.2 (3.7) mm; p = 0.030], peak lateral systolic velocity; S' lateral [1.0 (2.0) cm/s vs -0.3 (2.2) cm/s; p = 0.025], peak lateral early diastolic velocity; E' lateral [0.57 (2.4) cm/s vs -3.3 (3.9) cm/s; p < 0.001], transmitral to lateral mitral annular early diastolic velocity ratio; E/E' lateral [-0.7 (1.9) vs 1.5 (1.9); p < 0.001] over the study period in the ramipril compared to the placebo group. Significantly higher measurements were observed in the ramipril arm of the subgroup of patients with right ventricular restrictive physiology in terms of peak late diastolic velocity; A [5.9 (13.5) cm/s vs -5.8 (12.5) cm/s; p = 0.041] and early to late diastolic transmitral velocity ratio; E/A [-0.18 (0.42) vs 0.23 (0.48); p = 0.037]. CONCLUSION: Six months' ramipril treatment appears to limit progression of both diastolic and systolic LV function in adults late after tetralogy of Fallot repair. With increased appreciation that even subtle LV disease predicts tetralogy of Fallot outcomes, further clinical trials of drug therapies are justified.


Assuntos
Inibidores da Enzima Conversora de Angiotensina/uso terapêutico , Complicações Pós-Operatórias/tratamento farmacológico , Insuficiência da Valva Pulmonar/tratamento farmacológico , Ramipril/uso terapêutico , Tetralogia de Fallot/tratamento farmacológico , Função Ventricular Esquerda/efeitos dos fármacos , Adulto , Inibidores da Enzima Conversora de Angiotensina/farmacologia , Método Duplo-Cego , Feminino , Seguimentos , Humanos , Masculino , Complicações Pós-Operatórias/diagnóstico por imagem , Complicações Pós-Operatórias/epidemiologia , Estudos Prospectivos , Insuficiência da Valva Pulmonar/diagnóstico por imagem , Insuficiência da Valva Pulmonar/epidemiologia , Ramipril/farmacologia , Método Simples-Cego , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/cirurgia , Disfunção Ventricular Esquerda/diagnóstico por imagem , Disfunção Ventricular Esquerda/epidemiologia , Disfunção Ventricular Esquerda/prevenção & controle , Função Ventricular Esquerda/fisiologia , Adulto Jovem
17.
Int J Cardiol ; 267: 79-83, 2018 Sep 15.
Artigo em Inglês | MEDLINE | ID: mdl-29807779

RESUMO

BACKGROUND: Secondary erythrocytosis is common in patients with cyanosis secondary to congenital heart disease (CHD) and/or pulmonary hypertension (PH). This compensatory mechanism aims at increasing oxygen delivery to the tissues, but it requires adequate iron stores. Optimal methods of iron supplementation in this setting remain controversial, with fears of excessive erythropoiesis and hyperviscosity symptoms. We describe our experience using intravenous ferrous carboxymaltose. METHODS AND RESULTS: 142 consecutive cyanotic patients were treated over 5.7 years (201 administrations). Mean age was 51.3 ±â€¯17.6 years and 55 (38.7%) were male. Eisenmenger syndrome (ES) was present in 41 (28.8%), other pulmonary arterial hypertension (PAH) related to CHD (PAH-CHD) in 27 (19.0%), cyanotic CHD without PAH in 16 (11.3%) and PH without CHD in 58(40.8%). Baseline haemoglobin (Hb) concentration was 14.6 ±â€¯3.0 g/dL and haematocrit 0.45 ±â€¯0.09. A 500 mg dose of intravenous (IV) iron carboxymaltose was given in 163 (81.1%) of administrations and a 1000 mg dose in 37 (18.4%). A significant improvement in average Hb, haematocrit, ferritin and transferrin saturation was observed after a median follow-up of 100.0 [70.0-161.0] days (p ≤ 0.0001 for all). There were no cases of excessive erythropoiesis resulting in new hyperviscosity symptoms and/or requiring venesection. A minor transient rash was observed in 2 patients and one patient experienced an air embolus causing a transient ischemic attack. CONCLUSIONS: Intravenous ferrous carboxymaltose appears to be safe in iron deficient patients with cyanosis due to CHD and/or PH, as long as care is taken to avoid air emboli. Further randomised studies are needed to confirm the safety and efficacy of intravenous iron in this setting.


Assuntos
Compostos Férricos , Cardiopatias Congênitas , Hipertensão Pulmonar , Ferro , Maltose/análogos & derivados , Policitemia , Administração Intravenosa , Adulto , Idoso , Monitoramento de Medicamentos/métodos , Eritropoese/efeitos dos fármacos , Feminino , Compostos Férricos/administração & dosagem , Compostos Férricos/efeitos adversos , Cardiopatias Congênitas/sangue , Cardiopatias Congênitas/complicações , Hematínicos/administração & dosagem , Hematínicos/efeitos adversos , Testes Hematológicos/métodos , Humanos , Hipertensão Pulmonar/sangue , Hipertensão Pulmonar/complicações , Ferro/administração & dosagem , Ferro/efeitos adversos , Ferro/deficiência , Masculino , Maltose/administração & dosagem , Maltose/efeitos adversos , Pessoa de Meia-Idade , Policitemia/diagnóstico , Policitemia/etiologia , Policitemia/terapia , Estudos Retrospectivos , Resultado do Tratamento , Reino Unido
18.
Heart ; 104(23): 1963-1969, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-29776964

RESUMO

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.


Assuntos
Arritmias Cardíacas , Cardiopatias Congênitas , Hipertensão Pulmonar , Efeitos Adversos de Longa Duração/mortalidade , Qualidade de Vida , Adulto , Arritmias Cardíacas/classificação , Arritmias Cardíacas/epidemiologia , Arritmias Cardíacas/etiologia , Arritmias Cardíacas/terapia , Feminino , Grécia/epidemiologia , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/diagnóstico , Humanos , Hipertensão Pulmonar/complicações , Hipertensão Pulmonar/diagnóstico , Incidência , Expectativa de Vida , Masculino , Pessoa de Meia-Idade , Administração dos Cuidados ao Paciente/estatística & dados numéricos , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Índice de Gravidade de Doença
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