Late Onset Postcapillary Pulmonary Hypertension in Patients With Transposition of the Great Arteries and Mustard or Senning Baffles.

Published on Oct 12, 2017in Journal of the American Heart Association4.605
· DOI :10.1161/JAHA.117.006481
Marie-A. Chaix7
Estimated H-index: 7
(MHI: Montreal Heart Institute),
Annie Dore27
Estimated H-index: 27
(MHI: Montreal Heart Institute)
+ 9 AuthorsPaul Khairy70
Estimated H-index: 70
(MHI: Montreal Heart Institute)
BackgroundThere is a paucity of data regarding late‐onset pulmonary hypertension (PH) in patients with transposition of the great arteries and atrial switch surgery. Methods and ResultsA retrospect...
#1Nazzareno GalièH-Index: 98
#2Marc HumbertH-Index: 163
Last. Marius M. HoeperH-Index: 99
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ALAT : alanine aminotransferase ASAT : aspartate aminotransferase APAH : associated pulmonary arterial hypertension BAS : balloon atrial septostomy BMPR2 : bone morphogenetic protein receptor 2 BNP : brain natriuretic peptide BPA : balloon pulmonary angioplasty BREATHE : Bosentan
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#1Niels VejlstrupH-Index: 31
#2Keld E. SørensenH-Index: 51
Last. Peter Eriksson (IT University)H-Index: 2
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Background—The atrial switch operation, the Mustard or Senning operation, for the transposition of the great arteries (TGA) was introduced in the late 1950s and was the preferred surgery for TGA until the early 1990s. The Mustard and Senning operation involves extensive surgery in the atria and leaves the right ventricle as the systemic ventricle. The Mustard and Senning cohort is now well into adulthood and we begin to see the long-term outcome. Methods and Results—All the 6 surgical centers th...
54 CitationsSource
#1Nazzareno GalièH-Index: 98
#2Marc HumbertH-Index: 163
Last. Marius M. HoeperH-Index: 99
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381 CitationsSource
When pulmonary hypertension (PH), defined by mean pulmonary artery pressure (mPAP) ≥25 mm Hg, is associated with an abnormally elevated pulmonary capillary wedge pressure (PCWP) >15 mm Hg or left ventricular end-diastolic pressure (LVEDP) >18 mm Hg [(1)][1], it has been variably termed World
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#1Judith A.A.E. Cuypers (EUR: Erasmus University Rotterdam)H-Index: 17
#2Jannet A. Eindhoven (EUR: Erasmus University Rotterdam)H-Index: 14
Last. Jolien W. Roos-Hesselink (EUR: Erasmus University Rotterdam)H-Index: 70
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Aims To describe long-term survival, clinical outcome and ventricular systolic function in a longitudinally followed cohort of patients after Mustard repair for transposition of the great arteries (TGA). There is serious concern about the long-term outcome after Mustard repair. Methods and results This longitudinal single-centre study consisted of 91 consecutive patients, who underwent Mustard repair before 1980, at age <15 years, and were evaluated in-hospital every 10 years. Survival status wa...
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633 CitationsSource
#1Rick A. Nishimura (Mayo Clinic)H-Index: 128
#2Catherine M Otto (UW: University of Washington)H-Index: 85
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The medical profession should play a central role in evaluating evidence related to drugs, devices, and procedures for detection, management, and prevention of disease. When properly applied, expert analysis of available data on the benefits and risks of these therapies and procedures can improve
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#1Lawrence G. RudskiH-Index: 21
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#1Jean-Luc Vachiery (ULB: Université libre de Bruxelles)H-Index: 54
#2Yochai Adir (Technion – Israel Institute of Technology)H-Index: 24
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Pulmonary hypertension (PH), a common complication of left heart diseases (LHD), negatively impacts symptoms, exercise capacity, and outcome. Although the true prevalence of PH-LHD is unknown, a subset of patients might present significant PH that cannot be explained by a passive increase in left-sided filling pressures. The term “out-of-proportion” PH has been used to identify that population without a clear definition, which has been found less than ideal and created confusion. We propose a ch...
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#1Denise van der Linde (EUR: Erasmus University Rotterdam)H-Index: 11
#2Elisabeth E.M. Konings (EUR: Erasmus University Rotterdam)H-Index: 2
Last. Jolien W. Roos-Hesselink (EUR: Erasmus University Rotterdam)H-Index: 70
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Congenital heart disease (CHD) accounts for nearly one-third of all major congenital anomalies. CHD birth prevalence worldwide and over time is suggested to vary; however, a complete overview is missing. This systematic review included 114 papers, comprising a total study population of 24,091,867 live births with CHD identified in 164,396 individuals. Birth prevalence of total CHD and the 8 most common subtypes were pooled in 5-year time periods since 1930 and in continent and income groups sinc...
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Cited By10
#1Alexander Van De Bruaene (Katholieke Universiteit Leuven)H-Index: 21
#2Walter Droogne (Katholieke Universiteit Leuven)H-Index: 20
Last. Werner Budts (Katholieke Universiteit Leuven)H-Index: 57
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Abstract Being the most common mode of death, heart failure related to adult congenital heart disease (ACHD-HF) will be one of the main challenges for the ACHD community in the coming years. With increasing number of ACHD-HF patients and an increase in ACHD-HF related admissions, there is an increasing demand for advanced HF therapies such as the use of ventricular assist devices and transplantation. Unfamiliarity with the underlying anatomy and pathophysiology, a higher peri-operative transplan...
#1Lasya Gaur (Johns Hopkins University)H-Index: 3
#2Ari Cedars (Johns Hopkins University)H-Index: 2
Last. Stefan OrwatH-Index: 18
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Dextro-transposition of the great arteries (D-TGA) has undergone a significant evolution in surgical repair, leading to survivors with vastly different postsurgical anatomy which in turn guides their long-term cardiovascular morbidity and mortality. Arterial switch repair survivors are limited by a right ventricle in the systemic arterial position, arrhythmia and atrial baffles prone to obstruction or leak. Functional assessment of the systemic right ventricle is complex, requiring multimodality...
#1William R. Miranda (Mayo Clinic)H-Index: 14
#2C. Charles Jain (Mayo Clinic)H-Index: 4
Last. Donald J. Hagler (Mayo Clinic)H-Index: 88
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Objective To assess the prevalence of elevated systemic right ventricular (sRV) end-diastolic pressure and pulmonary arterial hypertension in adults with transposition of the great arteries (TGA) who have undergone atrial switch operation. Methods Forty-two adults (aged ≥18 years) with complete TGA and atrial switch palliation undergoing cardiac catheterisation between 2004 and 2018 at Mayo Clinic, MN, were identified. Clinical, echocardiographic and invasive haemodynamic data were abstracted fr...
#1Yiu-fai Cheung (HKU: University of Hong Kong)H-Index: 37
#2Wendy W. M. Lam (Queen Mary Hospital)H-Index: 14
Last. Pak-Cheong Chow (HKU: University of Hong Kong)H-Index: 9
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Abstract Background This study aimed to assess diffuse myocardial fibrosis of the systemic right ventricle and subpulmonary left ventricle in patients after Senning or Mustard operation for complete transposition of the great artery (TGA) using cardiac magnetic resonance (CMR) T1 mapping. Methods Thirty-one adult TGA patients after Senning (n = 24) or Mustard (n = 7) operation were studied at the age of 33.3 ± 4.0 years. Systemic right ventricular (RV) and subpulmonary left ventricular (LV) volu...
#1Ana Moya (Katholieke Universiteit Leuven)
#2Pieter De Meester (Katholieke Universiteit Leuven)H-Index: 12
Last. Alexander Van De Bruaene (Katholieke Universiteit Leuven)H-Index: 21
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Introduction: Despite right ventricular (RV) dysfunction being a major concern in Senning patients, long-term follow-up data is lacking. This study aimed (1) at evaluating regional (base-mid-apex) ...
#1Jonathan N. Menachem (VUMC: Vanderbilt University Medical Center)H-Index: 7
#2Kelly H. Schlendorf (VUMC: Vanderbilt University Medical Center)H-Index: 12
Last. JoAnn Lindenfeld (VUMC: Vanderbilt University Medical Center)H-Index: 60
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Abstract As a result of improvements in care for patients with congenital heart disease (CHD), >90% of children born with CHD are expected to survive to adulthood. For those adults, heart failure (HF) is the leading cause of death. Advances in recognition of, and treatments for, these patients continue to improve. Specifically, adults with CHD are candidates for both heart transplantation and mechanical circulatory support. However, challenges remain that require investigation to improve outcome...
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#1Meryl S. Cohen (Children's Hospital of Philadelphia)H-Index: 43
#2Luc Mertens (U of T: University of Toronto)H-Index: 68
Echocardiographic assessment of patients with transposition of the great arteries and congenitally corrected transposition requires awareness of the morphology and commonly associated lesions. The pre-operative echocardiography should include a full segmental and sequential analysis. Post-operative assessment is not possible without awareness of the type of surgical procedure performed and consists of assessing surgical connections and residual lesions.
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#2Norihisa Toh (UHN: University Health Network)H-Index: 1
Last. S. Lucy RocheH-Index: 12
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Objective This study sought to determine the prevalence, predictors, prognostic relevance and evolution of pulmonary hypertension (PH) (mean pulmonary artery pressure ≥25 mm Hg) in adult patients with a subaortic right ventricle (RV) in a biventricular circulation (2V-RV). Methods We analysed retrospective data from patients with 2V-RV undergoing cardiac catheterisation in our centre between 2000 and 2018. Echocardiographic assessment of subpulmonary ventricular pressures (left ventricular systo...
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#1Yuli Y. Kim (Children's Hospital of Philadelphia)H-Index: 14
#2Katherine Awh (Children's Hospital of Philadelphia)H-Index: 2
Last. Joyce Wald (HUP: Hospital of the University of Pennsylvania)H-Index: 18
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BACKGROUND: Systemic right ventricular (RV) failure may progress necessitating referral for orthotropic heart transplantation (OHT). Pulmonary hypertension (PH) frequently coexists in adult congenital heart disease and can complicate the assessment for OHT. METHODS: Single-center case series of six patients (median age 34.9 years [IQR, 31.9-42.4]) with systemic RV physiology with PH referred for OHT evaluation from 2008 to 2017. RESULTS: One-third (n = 6) of 18 patients with systemic RV physiolo...
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#1Marie-A. Chaix (MHI: Montreal Heart Institute)H-Index: 7
#2Annie Dore (MHI: Montreal Heart Institute)H-Index: 27
Last. François-Pierre Mongeon (MHI: Montreal Heart Institute)H-Index: 17
view all 13 authors...
Abstract Background Echocardiographic evaluation of the systemic right ventricle (sRV) remains challenging in patients with transposition of the great arteries (TGA) corrected by an atrial switch (AS) and with congenitally corrected TGA (ccTGA). The aim of this study was to determine the interobserver and intraobserver variability of echocardiographic parameters for sRV size and systolic function. Methods Six independent observers retrospectively interpreted 44 previously acquired echocardiogram...
3 CitationsSource