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1.
Int J Cardiol ; 415: 132445, 2024 Nov 15.
Artículo en Inglés | MEDLINE | ID: mdl-39134124

RESUMEN

BACKGROUND: The Fontan circulation procedure is the palliative surgery of choice for patients with single ventricle physiology, many of whom are now reaching childbearing age due to advances in care. Our study examines the impact of pregnancy on women with Fontan circulation, assessing both short and long-term outcomes. METHODS: We retrospectively analysed pregnancies in women with Fontan circulation at our centre from 2005 to 2023, including a matched non-pregnant, nulliparous cohort for comparison. Pregnancies lost before 18 weeks were analysed separately. RESULTS: Among 26 pregnancies in 18 women, preterm births were common (73.1%), with 3 fetal losses and no maternal deaths. Neonatal complications included a 50% incidence of babies small for gestational age (SGA). Cardiac events occurred in 19.2% of pregnancies, primarily supraventricular arrhythmias, and 23% experienced postpartum haemorrhage. Over a median 6.1 [5.2-10.6]-year follow-up, no deaths, heart failure (HF), or Fontan-associated liver disease (FALD) developed, and functional status remained stable. CONCLUSIONS: Women with a Fontan-type circulation undergoing pregnancy had a few serious maternal cardiac events, though there was a high rate of post-partum haemorrhage. The rates of neonatal complications, particularly related to restricted growth and pre-term birth, were also high. Our findings indicate a generally positive medium-term outlook for these patients, though the impact of pregnancy on long-term survival remains unclear. Careful selection of patients with Fontan circulation during pre-pregnancy counselling may help to reduce complications during and after pregnancy.


Asunto(s)
Procedimiento de Fontan , Complicaciones Cardiovasculares del Embarazo , Resultado del Embarazo , Humanos , Femenino , Procedimiento de Fontan/efectos adversos , Procedimiento de Fontan/tendencias , Embarazo , Estudios Retrospectivos , Adulto , Resultado del Embarazo/epidemiología , Complicaciones Cardiovasculares del Embarazo/epidemiología , Estudios de Seguimiento , Adulto Joven , Recién Nacido , Cardiopatías Congénitas/cirugía , Factores de Tiempo
2.
Eur Heart J ; 45(23): 2066-2075, 2024 Jun 14.
Artículo en Inglés | MEDLINE | ID: mdl-38743452

RESUMEN

BACKGROUND AND AIMS: Many adult patients with congenital heart disease (ACHD) are still afflicted by premature death. Previous reports suggested natriuretic peptides may identify ACHD patients with adverse outcome. The study investigated prognostic power of B-type natriuretic peptide (BNP) across the spectrum of ACHD in a large contemporary cohort. METHODS: The cohort included 3392 consecutive ACHD patients under long-term follow-up at a tertiary ACHD centre between 2006 and 2019. The primary study endpoint was all-cause mortality. RESULTS: A total of 11 974 BNP measurements were analysed. The median BNP at baseline was 47 (24-107) ng/L. During a median follow-up of 8.6 years (29 115 patient-years), 615 (18.1%) patients died. On univariable and multivariable analysis, baseline BNP [hazard ratio (HR) 1.16, 95% confidence interval (CI) 1.15-1.18 and HR 1.13, 95% CI 1.08-1.18, respectively] and temporal changes in BNP levels (HR 1.22, 95% CI 1.19-1.26 and HR 1.19, 95% CI 1.12-1.26, respectively) were predictive of mortality (P < .001 for both) independently of congenital heart disease diagnosis, complexity, anatomic/haemodynamic features, and/or systolic systemic ventricular function. Patients within the highest quartile of baseline BNP (>107 ng/L) and those within the highest quartile of temporal BNP change (>35 ng/L) had significantly increased risk of death (HR 5.8, 95% CI 4.91-6.79, P < .001, and HR 3.6, 95% CI 2.93-4.40, P < .001, respectively). CONCLUSIONS: Baseline BNP and temporal BNP changes are both significantly associated with all-cause mortality in ACHD independent of congenital heart disease diagnosis, complexity, anatomic/haemodynamic features, and/or systolic systemic ventricular function. B-type natriuretic peptide levels represent an easy to obtain and inexpensive marker conveying prognostic information and should be used for the routine surveillance of patients with ACHD.


Asunto(s)
Biomarcadores , Cardiopatías Congénitas , Péptido Natriurético Encefálico , Humanos , Péptido Natriurético Encefálico/sangre , Péptido Natriurético Encefálico/metabolismo , Cardiopatías Congénitas/mortalidad , Cardiopatías Congénitas/sangre , Femenino , Masculino , Adulto , Pronóstico , Biomarcadores/sangre , Persona de Mediana Edad , Causas de Muerte , Estudios de Seguimiento
3.
Eur J Prev Cardiol ; 31(11): 1336-1344, 2024 Aug 22.
Artículo en Inglés | MEDLINE | ID: mdl-38669446

RESUMEN

Improved survival rates for patients with a Fontan circulation has allowed more women with this complex cardiac physiology to contemplate pregnancy. However, pregnancy in women with a Fontan circulation is associated with a high risk of adverse maternal and foetal outcomes, high rates of miscarriage, and preterm delivery. Factors associated with a successful pregnancy outcome are younger age, normal body weight, absence of significant functional limitation, no Fontan-related complications, and well-functioning single ventricle physiology. Appropriate care with timely preconception counselling and regular, frequent clinical reviews by a multidisciplinary team based at a tertiary centre improves the chance of a successful pregnancy. Empowerment of patients with education on their specific congenital cardiac condition and its projected trajectory helps them make informed choices regarding their health, reproductive choices, and assists them to achieve their life goals.


Pregnancy in women with Fontan is feasible but associated with a high risk of adverse maternal/foetal outcomes. Younger age, normal body weight, absence of significant functional limitation, and no Fontan-related complications are favourable factors. Timely preconception counselling and regular clinical reviews in a tertiary centre with an MDT improves pregnancy outcomes. Empowerment of patients with education helps them make informed choices on health, family planning, and the future.


Asunto(s)
Procedimiento de Fontan , Cardiopatías Congénitas , Complicaciones Cardiovasculares del Embarazo , Humanos , Procedimiento de Fontan/efectos adversos , Femenino , Embarazo , Complicaciones Cardiovasculares del Embarazo/fisiopatología , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/fisiopatología , Resultado del Embarazo , Factores de Riesgo , Resultado del Tratamiento , Medición de Riesgo
4.
Eur J Prev Cardiol ; 31(11): 1316-1323, 2024 Aug 22.
Artículo en Inglés | MEDLINE | ID: mdl-38306409

RESUMEN

AIMS: To evaluate the late outcomes of adults (above 35 years) with a Fontan-type circulation, for whom current data on morbidity and mortality are lacking. METHODS AND RESULTS: Data were collected retrospectively on consecutive patients with Fontan circulation above the age of 35 years followed in three European specialist centres. Overall, 115 Fontan patients were included [median age 35 (range 35-48) years, 47.8% female]. The most common underlying congenital heart disease diagnosis was tricuspid atresia (n = 58, 50.4%), and the age at first Fontan completion was 9.1 (interquartile range 5.0-15.8) years. Almost two-thirds (61.7%) of patients had undergone an atriopulmonary Fontan, and 23.5% had received a total cavopulmonary connection. One-third required repeat surgery or intervention. Most patients (55.9%) were in New York Heart Association functional class II or class I (30.6%), 76 (66.1%) patients had experienced at least one arrhythmia, and eight (7.0%) protein-losing enteropathy. At a median follow-up of 5.0 (2.4-10.3) years, 15 (13.0%) patients were referred for transplantation assessment and 19 (16.5%) patients died, mainly from heart failure (84.2%). Univariable predictors of death or transplantation included lower serum albumin level [hazard ratio (HR) 1.09 per g/L decrease, 95% confidence interval (CI): 1.04-1.15, P = 0.0009], prior heart failure admission (HR 4.28, 95% CI:1.75-10.44, P = 0.001), prior atrial tachycardia or flutter (HR 3.02, 95% CI: 1.23-7.38, P = 0.02), and baseline pulmonary vasodilator therapy (HR 8.59, 95% CI:1.05-70.13, P = 0.04). Lower serum albumin and prior atrial tachycardia or flutter remained significant on bivariable analysis. CONCLUSION: Our study highlights the significant morbidity and mortality in older adults with a Fontan-type circulation, emphasizing the need for lifelong specialist surveillance with frequent risk stratification, close monitoring, and early consideration for transplantation assessment.


This study sheds light on the complex medical journey of adults living with the outcomes of Fontan surgery­a procedure performed in early childhood. These individuals have reached the milestone of their forties and beyond, yet they confront an array of significant health challenges that necessitate lifelong, individualized congenital heart disease care. The key findings are as follows:While adults with Fontan circulation are living longer, they are at high risk of death, mainly due to heart failure. They also face a host of other health issues, including the need for additional surgeries or interventions. Nearly two-thirds have experienced some form of heart rhythm problem, and a substantial number eventually require evaluation for a heart transplant.Heart transplants within this group were rare, which may be linked to the various barriers to transplantation in the Fontan population. Moreover, those with multiple indicators of advanced disease have a heightened risk of life-threatening events, reinforcing the critical need for personalized and continuous specialist care designed to meet their distinct health requirements.


Asunto(s)
Procedimiento de Fontan , Cardiopatías Congénitas , Humanos , Procedimiento de Fontan/efectos adversos , Procedimiento de Fontan/mortalidad , Femenino , Masculino , Adulto , Estudios Retrospectivos , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/mortalidad , Persona de Mediana Edad , Factores de Riesgo , Factores de Tiempo , Resultado del Tratamiento , Factores de Edad , Europa (Continente)/epidemiología , Medición de Riesgo
5.
J Am Heart Assoc ; 12(18): e029251, 2023 09 19.
Artículo en Inglés | MEDLINE | ID: mdl-37721152

RESUMEN

Background Establishing surgical criteria for aortic valve replacement (AVR) in severe aortic regurgitation in young adults is challenging due to the lack of evidence-based recommendations. We studied indications for AVR in young adults with severe aortic regurgitation and their outcomes, as well as the relationship between presurgical echocardiographic parameters and postoperative left ventricular (LV) size, function, clinical events, and valve-related complications. Methods and Results Data were collected retrospectively on 172 consecutive adult patients who underwent AVR or repair for severe aortic regurgitation between 2005 and 2019 in a tertiary cardiac center (age at surgery 29 [22-41] years, 81% male). One-third underwent surgery before meeting guideline indications. Postsurgery, 65% achieved LV size and function normalization. LV ejection fraction showed no significant change from baseline. A higher presurgical LV end-systolic diameter correlated with a lack of LV normalization (odds ratio per 1-cm increase 2.81, P<0.01). The baseline LV end-systolic diameter cut-off for predicting lack of LV normalization was 43 mm. Pre- and postoperative LV dimensions and postoperative LV ejection fraction predicted clinical events during follow-up. Prosthetic valve-related complications occurred in 20.3% during an average 5.6-year follow-up. Freedom from aortic reintervention was 98%, 96.5%, and 85.4% at 1, 5, and 10 years, respectively. Conclusions Young adult patients with increased baseline LV end-systolic diameter or prior cardiac surgery are less likely to achieve LV normalization after AVR. Clinicians should carefully balance the long-term benefits of AVR against procedural risks and future interventions, especially in younger patients. Evidence-based criteria for AVR in severe aortic regurgitation in young adults are crucial to improve outcomes.


Asunto(s)
Insuficiencia de la Válvula Aórtica , Adulto Joven , Humanos , Masculino , Adulto , Femenino , Insuficiencia de la Válvula Aórtica/diagnóstico por imagen , Insuficiencia de la Válvula Aórtica/cirugía , Estudios Retrospectivos , Corazón , Aorta , Catéteres
7.
Expert Rev Respir Med ; 17(5): 413-423, 2023 05.
Artículo en Inglés | MEDLINE | ID: mdl-37159412

RESUMEN

INTRODUCTION: Pulmonary arterial hypertension (PAH) is defined as a mean pulmonary artery pressure >20 mmHg and pulmonary vascular resistance >2 Wood Units (WU) on right-heart catheterization. Pregnancy is generally contraindicated in PAH, it is associated with high maternal mortality. Despite current recommendations, the number of women with PAH wishing to become pregnant is increasing. Specialist care is essential for preconception counseling, and the management of pregnancy and delivery in such patients. AREAS COVERED: We cover the physiology of pregnancy, and its effects on the cardiovascular system in PAH. We also discuss optimal management based on available evidence and guidance. EXPERT OPINION: Pregnancy should be avoided in most patients with PAH. Counseling on appropriate contraception should be offered routinely. Education of women with childbearing potential is essential and should start at the time of diagnosis of PAH, or the time of transition from pediatric to adult services in patients developing PAH in childhood. Women wishing to become pregnant should receive individualized risk assessment and optimization of PAH therapies via a dedicated specialist pre-pregnancy counseling service, to minimize risk and improve outcomes. Pregnant PAH patients should receive expert multidisciplinary management in a PH center, including close monitoring and early initiation of therapies.


Asunto(s)
Hipertensión Pulmonar , Hipertensión Arterial Pulmonar , Embarazo , Adulto , Humanos , Femenino , Niño , Hipertensión Pulmonar Primaria Familiar/complicaciones , Medición de Riesgo , Consejo
8.
Eur J Prev Cardiol ; 30(13): 1335-1342, 2023 09 20.
Artículo en Inglés | MEDLINE | ID: mdl-36974357

RESUMEN

AIMS: Previous studies in adult congenital heart disease (CHD) have demonstrated a link between renal dysfunction and mortality. However, the prognostic significance of renal dysfunction in CHD and decompensated heart failure (HF) remains unclear. We sought to assess the association between renal dysfunction and outcomes in adults with CHD presenting to our centre with acute HF between 2010 and 2021. METHODS AND RESULTS: This retrospective analysis focused on the association between renal dysfunction, pre-existing and on admission, and outcomes during and after the index hospitalization. Chronic kidney disease (CKD) was defined as an estimated glomerular filtration rate <60 mL/min/1.73 m2. Cox regression analysis was used to identify the predictors of death post-discharge. In total, 176 HF admissions were included (mean age 47.7 ± 14.5 years, 43.2% females). One-half of patients had a CHD of great complexity, 22.2% had a systemic right ventricle, and 18.8% had Eisenmenger syndrome. Chronic kidney disease was present in one-quarter of patients. The median length of intravenous diuretic therapy was 7 (4-12) days, with a maximum dose of 120 (80-160) mg furosemide equivalents/day, and 15.3% required inotropic support. The in-hospital mortality rate was 4.5%. The 1- and 5-year survival rates free of transplant or ventricular assist device (VAD) post-discharge were 75.4% [95% confidence interval (CI): 69.2-82.3%] and 43.3% (95% CI: 36-52%), respectively. On multivariable Cox analysis, CKD was the strongest predictor of mortality or transplantation/VAD. Highly complex CHD and inpatient requirement of inotropes also remained predictive of an adverse outcome. CONCLUSION: Adult patients with CHD admitted with acute HF are a high-risk cohort. CKD is common and triples the risk of death/transplantation/VAD. An expert multidisciplinary approach is essential for optimizing outcomes.


Renal dysfunction was associated with more advanced disease, higher diuretic doses, and a longer hospital inpatient stay. Chronic kidney disease was common and tripled the risk of death, transplantation, or ventricular assist device. Renal dysfunction in adults with congenital heart disease and heart failure should prompt intensified monitoring, optimization of medical therapy, and collaborative management with renal physicians.


Asunto(s)
Cardiopatías Congénitas , Insuficiencia Cardíaca , Insuficiencia Renal Crónica , Femenino , Humanos , Adulto , Persona de Mediana Edad , Masculino , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/terapia , Estudios Retrospectivos , Cuidados Posteriores , Alta del Paciente , Insuficiencia Cardíaca/diagnóstico , Insuficiencia Cardíaca/etiología , Insuficiencia Cardíaca/terapia , Insuficiencia Renal Crónica/complicaciones , Insuficiencia Renal Crónica/diagnóstico , Insuficiencia Renal Crónica/terapia
9.
Eur Heart J Case Rep ; 7(3): ytad079, 2023 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-36895304

RESUMEN

Background: Coarctation of the aorta (CoA) is one of the more common congenital heart defects affecting up to 5% of patients with congenital heart disease. Pregnant patients with unrepaired or severe re-coarctation are considered to be modified World Health Organisation (mWHO) IV, have the highest risk of maternal mortality and morbidity. The management of unrepaired CoA during pregnancy is influenced by a variety of factors which include the extent of the coarctation and coarctation characteristics, but due to paucity of data, it largely relies on expert opinion. Case summary: A 27 year old multi-gravid woman underwent successful percutaneous stent implantation for severe native CoA due to maternal resistant hypertension and foetal cardiac compromise on echocardiography. After intervention, the remainder of her pregnancy was uneventful with improved arterial hypertension control. The foetal cardiac structures, namely left ventricular size, improved after intervention. This case demonstrates the importance of CoA intervention during pregnancy to optimise both maternal and foetal outcome. Conclusion: Coarctation of the aorta should be considered in pregnant women with poorly controlled hypertension. This case also highlights that, despite associated risks, percutaneous intervention can lead to improved maternal haemodynamics and fetal growth.

10.
Eur Heart J Qual Care Clin Outcomes ; 9(4): 351-357, 2023 06 21.
Artículo en Inglés | MEDLINE | ID: mdl-36792070

RESUMEN

AIMS: Less than one-third of adolescents with congenital heart disease (CHD) successfully transition to adult care, missing out on education of their cardiac condition, and risking loss to follow-up. We assessed the efficacy of our transition clinic on patient education and empowerment and identified correlates of successful transition. METHODS AND RESULTS: Overall, 592 patients were seen at least once in our transition service between 2015 and 2022 (age 15.2 ± 1.8 years, 47.5% female). Most adolescents (53%) had moderate CHD, followed by simple (27.9%) and severe (19.1%) CHD. Learning disability (LD) was present in 18.9% and physical disability (PD) in 4.7%. In patients without LD, knowledge of their cardiac condition improved significantly from the first to the second visit (naming their condition: from 20 to 52.3%, P < 0.0001; describing: 14.4-42.7%, P < 0.0001; understanding: 26.1-60.7%, P < 0.0001), and from the second to the third (naming: 67.4%, P = 0.004, describing: 61.4%, P < 0.001, understanding: 71.1%, P = 0.02;). Patients with LD did not improve their disease knowledge over time (all P > 0.05). Treatment adherence and management involvement, self-reported anxiety, and dental care awareness did not change over time. Successful transition (attendance of ≥ 2 clinics) was achieved in 49.3%. Younger age at the first visit, simpler CHD, and absence of PD were associated with successful transition. CONCLUSION: A transition service positively impacts on patient education and empowerment in most CHD adolescents transitioning to adult care. Strategies to promote a tailored support for patients with LD should be sought, and earlier engagement should be encouraged to minimize follow-up losses.


Asunto(s)
Cardiopatías Congénitas , Transición a la Atención de Adultos , Adulto , Adolescente , Humanos , Femenino , Masculino , Autocuidado , Cardiopatías Congénitas/terapia , Autoinforme
11.
JACC Case Rep ; 28: 102136, 2023 Dec 20.
Artículo en Inglés | MEDLINE | ID: mdl-38204530

RESUMEN

We present 4 patients with Fontan circulation who underwent successful pregnancies, albeit with complications that required close monitoring and timely intervention. Each Fontan patient presents with a unique clinical picture, making risk stratification challenging but all the more important.

12.
Int J Cardiol ; 360: 23-28, 2022 08 01.
Artículo en Inglés | MEDLINE | ID: mdl-35500817

RESUMEN

BACKGROUND: Infective endocarditis (IE) is a potentially lethal disease for adults with congenital heart disease (ACHD). Staphylococcus aureus (SA) is one of the commonest pathogens, identified in patients with ACHD and IE. This study aims to analyse the epidemiology, clinical characteristics and in-hospital management of IE due to SA (SAIE) in ACHD. METHODS: This is a single center retrospective study on documented episodes of SAIE in ACHD patients, treated at the Royal Brompton Hospital between 1999 and February 2020. RESULTS: Thirty-seven ACHD patients had an episode of SAIE (mean age 35.5 ± 14.9 years). More than half (51.3%), had a cardiac lesion of at least moderate complexity, with left ventricular outflow tract lesions being the most frequent. Twenty-four (64.9%) patients had a history of at least one previous open-heart surgery, and prosthetic material was used in 20 patients. A predisposing event was identified in 11 episodes (29.7%). Surgery was performed during the same admission in 24 (64.9%) patients, with early surgery (≤ 7 days from admission) performed in 12 patients. Early operation was associated with a shorter in-hospital stay compared to a later operation during the same admission or medical management [28.5 (IQR 23) vs. 43.5 (IQR 30) days, P = 0.028]. In-hospital mortality was 8.1%. Renal dysfunction at admission was associated with in-hospital multiorgan complications and/or death (OR 7.50, 95% CI, 1.18-47.67, P = 0.03). CONCLUSION: SAIE remains a life-threatening complication, necessitating cardiac surgery in the majority of ACHD patients. Ongoing educational investment on prevention and timely SAIE detection is warranted.


Asunto(s)
Endocarditis Bacteriana , Endocarditis , Cardiopatías Congénitas , Infecciones Estafilocócicas , Adulto , Endocarditis/epidemiología , Endocarditis Bacteriana/diagnóstico , Endocarditis Bacteriana/epidemiología , Endocarditis Bacteriana/terapia , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/epidemiología , Cardiopatías Congénitas/cirugía , Humanos , Persona de Mediana Edad , Estudios Retrospectivos , Factores de Riesgo , Infecciones Estafilocócicas/diagnóstico , Infecciones Estafilocócicas/epidemiología , Infecciones Estafilocócicas/terapia , Staphylococcus aureus , Adulto Joven
13.
J Am Coll Cardiol ; 78(3): 234-242, 2021 07 20.
Artículo en Inglés | MEDLINE | ID: mdl-34266577

RESUMEN

BACKGROUND: In-hospital mortality is a rare, yet feared complication following cardiac surgery in adult congenital heart disease (ACHD). A risk score, developed and validated in ACHD, can be helpful to optimize risk assessment. OBJECTIVES: The purpose of this study was to assess the performance of EuroSCORE II components and procedure-related Adult Congenital Heart Surgery (ACHS) score, identify additional risk factors, and develop a novel risk score for predicting in-hospital mortality after ACHD surgery. METHODS: We assessed perioperative survival in patients aged >16 years undergoing congenital heart surgery in a large tertiary center between 2003 and 2019. A risk variable-derived PEACH (PErioperative ACHd) score was calculated for each patient. Internal and external validation of the model was undertaken, including testing in a validation cohort of patients operated in a second European ACHD center. RESULTS: The development cohort comprised 1,782 procedures performed during the study period. Re-sternotomy was undertaken in 897 (50.3%). There were 31 (1.7%) in-hospital deaths. The PEACH score showed excellent discrimination ability (area under the curve [AUC]: 0.88; 95% CI: 0.83-0.94), and performed better than the ACHS score in our population (ACHS AUC: 0.69; 95% CI: 0.6-0.78; P = 0.0003). A simple 3-tiered risk stratification was formed: PEACH score 0 (in-hospital mortality 0.2%), 1-2 (3.6%), and ≥3 (17.2%). In a validation cohort of 975 procedures, the PEACH score retained its discriminative ability (AUC: 0.75; 95% CI: 0.72-0.77) and was well calibrated (Hosmer-Lemeshow chi-square goodness-of-fit P = 0.55). There was agreement in expected and observed perioperative mortality between cohorts. CONCLUSIONS: The PEACH score is a simple, novel perioperative risk score developed and validated specifically for ACHD patients undergoing cardiac surgery.


Asunto(s)
Procedimientos Quirúrgicos Cardíacos , Cardiopatías Congénitas/mortalidad , Medición de Riesgo/métodos , Adulto , Femenino , Estudios de Seguimiento , Cardiopatías Congénitas/cirugía , Mortalidad Hospitalaria/tendencias , Humanos , Masculino , Curva ROC , Estudios Retrospectivos , Factores de Riesgo , Tasa de Supervivencia/tendencias , Reino Unido/epidemiología
14.
Heart ; 107(17): 1384-1389, 2021 09.
Artículo en Inglés | MEDLINE | ID: mdl-33958396

RESUMEN

BACKGROUND: To date, clinical trials have been underpowered to demonstrate a benefit from ACE inhibitors (ACEis) or angiotensin II receptor blockers (ARBs) in preventing systemic right ventricle (sRV) failure and disease progression in patients with transposition of the great arteries (TGA). This observational study aimed to estimate the effect of ACEi and ARB on heart failure (HF) incidence and mortality in a large population of patients with an sRV. METHODS: Data on all patients with an sRV under active follow-up at two tertiary centres between January 2007 and September 2018 were studied. The effect of ACEi and ARB on the incidence of HF and mortality was estimated using a propensity score weighting approach to control confounding. RESULTS: Among the 359 patients with an sRV (32.2 (IQR 26.4-38.3) years, 59.3% male, 66% complete TGA with atrial switch repair and 34% congenitally corrected TGA), 79 (22%) had a moderate to severe sRV dysfunction and 138 (38%) were treated with ACEi or ARB. Fourteen (3.6%) patients died, 8 (2.1%) underwent heart transplantation and 46 (11.8%) had a new HF event over a median follow-up of 7.1 (IQR 4.0-9.4) years. On multivariate Cox analysis with adjustment using propensity score weighting approaches, ACEi or ARBs treatment was not significantly associated with a lower HF incidence or mortality in patients with an sRV. CONCLUSIONS: Despite significant neurohormonal activation described in patients with an sRV, there is still no evidence of a beneficial effect of ACEi or ARB on morbidity and mortality in this population.


Asunto(s)
Antagonistas de Receptores de Angiotensina/uso terapéutico , Inhibidores de la Enzima Convertidora de Angiotensina/uso terapéutico , Insuficiencia Cardíaca , Complicaciones Posoperatorias , Transposición de los Grandes Vasos , Disfunción Ventricular Derecha , Adulto , Operación de Switch Arterial/efectos adversos , Operación de Switch Arterial/métodos , Progresión de la Enfermedad , Femenino , Francia/epidemiología , Insuficiencia Cardíaca/tratamiento farmacológico , Insuficiencia Cardíaca/epidemiología , Insuficiencia Cardíaca/etiología , Humanos , Incidencia , Masculino , Complicaciones Posoperatorias/diagnóstico , Complicaciones Posoperatorias/tratamiento farmacológico , Complicaciones Posoperatorias/fisiopatología , Prevención Secundaria/métodos , Transposición de los Grandes Vasos/complicaciones , Transposición de los Grandes Vasos/mortalidad , Transposición de los Grandes Vasos/fisiopatología , Transposición de los Grandes Vasos/cirugía , Resultado del Tratamiento , Reino Unido/epidemiología , Disfunción Ventricular Derecha/tratamiento farmacológico , Disfunción Ventricular Derecha/etiología , Disfunción Ventricular Derecha/fisiopatología
16.
JACC Case Rep ; 2(5): 725-731, 2020 May.
Artículo en Inglés | MEDLINE | ID: mdl-34317336

RESUMEN

A 52-year-old man following surgery for Ebstein's anomaly after repair developed acute hemodynamically significant left ventricular outflow tract obstruction with systolic anterior motion of the mitral valve and severe mitral regurgitation. Fluid resuscitation and weaning of inotropes were unsuccessful. Left ventricular outflow tract obstruction and mitral regurgitation resolved by using esmolol. (Level of Difficulty: Intermediate.).

18.
Front Pediatr ; 5: 39, 2017.
Artículo en Inglés | MEDLINE | ID: mdl-28293551

RESUMEN

INTRODUCTION: Patients with Tetralogy of Fallot (TOF) and pulmonary insufficiency and a dilated right ventricle (RV) may suffer from a reduction in left ventricular (LV) performance. It is not clear whether timely pulmonary valve replacement (PVR) preserves LV mechanics. METHODS: Ten TOF patients who underwent PVR were identified from hospital records, and pre- and postoperative cardiac magnetic resonance images were post-processed with a semi-automatic tissue tracking software. LV circumferential strain, time to peak strain, and torsion were compared before and after PVR. A control group of 10 age-matched normal volunteers was assessed as a comparison. RESULTS: LV circumferential strain did not change before vs. after PVR (basal -18.3 ± 3.7 vs. -20.5 ± 3%, p = 0.082; mid-ventricular -18.4 ± 3.6 vs. -19.1 ± 2%, p = 0.571; apical -22.7 ± 5.2 vs. -22.1 ± 4%; p = 0.703). There was also no difference seen between the baseline strain and normal controls (control basal -18.2 ± 3.3%, p = 0.937; mid -18 ± 3.2%, p = 0.798; apex -24.1 ± 5%, p = 0.552). LV torsion remained unchanged from baseline to post PVR [systolic 2.75 (1.23-9.51) °/cm vs. 2.3 ± 1.2°/cm, p = 0.285; maximum 5.5 ± 3.5°/cm vs. 2.34 (1.37-8.07) °/cm, p = 0.083]. There was no difference in time to measured peak LV circumferential strain before vs. after PVR (basal 0.44 ± 0.1 vs. 0.43 ± 0.05, p = 0.912; mid-ventricular 0.42 ± 0.08 vs. 0.38 ± 0.06, p = 0.186; apical 0.40 ± 0.08 vs. 0.40 ± 0.06, p = 0.995). At the same time, pulmonary regurgitation and RV end-diastolic and end-systolic volume indices decreased and LV end-diastolic volume increased after PVR. RV and LV ejection fractions remained constant. CONCLUSION: PVR allows for favorable remodeling of both ventricular volumes for TOF patients with significant pulmonary regurgitation. In this cohort, LV myocardial functional parameters such as circumferential strain, time to peak strain, and LV torsion were normal at baseline and remain unchanged after PVR.

19.
J Cardiovasc Magn Reson ; 19(1): 32, 2017 Mar 08.
Artículo en Inglés | MEDLINE | ID: mdl-28270208

RESUMEN

BACKGROUND: Pulse wave velocity (PWV) is a biomarker for the intrinsic stiffness of the aortic wall, and has been shown to be predictive for cardiovascular events. It can be assessed using cardiovascular magnetic resonance (CMR) from the delay between phase-contrast flow waveforms at two or more locations in the aorta, and the distance on CMR images between those locations. This study aimed to investigate the impact of different distance measurement methods on PWV. We present and evaluate an algorithm for automated centreline tracking in 3D images, and compare PWV calculations using distances derived from 3D images to those obtained from a conventional 2D oblique-sagittal image of the aorta. METHODS: We included 35 patients from a twin cohort, and 20 post-coarctation repair patients. Phase-contrast flow was acquired in the ascending, descending and diaphragmatic aorta. A 3D centreline tracking algorithm is presented and evaluated on a subset of 30 subjects, on three CMR sequences: balanced steady-state free precession (SSFP), black-blood double inversion recovery turbo spin echo, and contrast-enhanced CMR angiography. Aortic lengths are subsequently compared between measurements from a 2D oblique-sagittal plane, and a 3D geometry. RESULTS: The error in length of automated 3D centreline tracking compared with manual annotations ranged from 2.4 [1.8-4.3] mm (mean [IQR], black-blood) to 6.4 [4.7-8.9] mm (SSFP). The impact on PWV was below 0.5m/s (<5%). Differences between 2D and 3D centreline length were significant for the majority of our experiments (p < 0.05). Individual differences in PWV were larger than 0.5m/s in 15% of all cases (thoracic aorta) and 37% when studying the aortic arch only. Finally, the difference between end-diastolic and end-systolic 2D centreline lengths was statistically significant (p < 0.01), but resulted in small differences in PWV (0.08 [0.04 - 0.10]m/s). CONCLUSIONS: Automatic aortic centreline tracking in three commonly used CMR sequences is possible with good accuracy. The 3D length obtained from such sequences can differ considerably from lengths obtained from a 2D oblique-sagittal plane, depending on aortic curvature, adequate planning of the oblique-sagittal plane, and patient motion between acquisitions. For accurate PWV measurements we recommend using 3D centrelines.


Asunto(s)
Algoritmos , Aorta/diagnóstico por imagen , Interpretación de Imagen Asistida por Computador/métodos , Imagenología Tridimensional/métodos , Angiografía por Resonancia Magnética/métodos , Análisis de la Onda del Pulso/métodos , Rigidez Vascular , Adulto , Anciano , Aorta/fisiopatología , Aorta/cirugía , Coartación Aórtica/diagnóstico por imagen , Coartación Aórtica/fisiopatología , Coartación Aórtica/cirugía , Automatización , Velocidad del Flujo Sanguíneo , Bases de Datos Factuales , Femenino , Humanos , Masculino , Persona de Mediana Edad , Variaciones Dependientes del Observador , Valor Predictivo de las Pruebas , Flujo Sanguíneo Regional , Reproducibilidad de los Resultados , Estudios Retrospectivos , Adulto Joven
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