Long-term Outcome and Quality of Life after Arterial Switch Operation: A Prospective Study with a Historical Comparison

Ruys, Titia P.E.; Bosch, Annemien E. van der; Cuypers, Judith A.A.E.; Witsenburg, Maarten; Helbing, Willem A.; Bogers, Ad J.J.C.; Domburg, Ron van; McGhie, Jacky; Geleijnse, Marcel L.; Henrichs, Jens; Utens, Elisabeth M. W. J.; Zwaan, Heleen B. van der; Takkenberg, Johanna J.M.; Roos‐Hesselink, Jolien W.

doi:10.1111/chd.12033

Cited by 39 publications

(35 citation statements)

References 33 publications

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“…However, 84% of included children underwent an atrial balloon septostomy procedure in the first days of life. This proportion contrasts with the reported incidence of approximately 40% atrial balloon septostomy procedures in infants with TGA,27 suggesting that incomplete mixing and prenatal haemodynamic changes were more prevalent in these children. Also, the occurrence of PPHN, a condition of disturbed adaptation of the pulmonary vasculature to postnatal life, is more frequent in children with TGA than in the normal population, supporting this concept of altered prenatal pulmonary haemodynamics.…”

Section: Discussioncontrasting

confidence: 61%

Pulmonary arterial hypertension in children after neonatal arterial switch operation

Zijlstra

Elmasry

Pepplinkhuizen

et al. 2017

Heart

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Objectives Paediatric pulmonary arterial hypertension (PAH) after neonatal arterial switch operation (ASO) for transposition of the great arteries (TGA) is a clinically recognised entity with an estimated incidence of 0.6%-1.0%. Nevertheless, a clinical characterisation is lacking. We present an international cohort of children with PAH after neonatal ASO for TGA and describe epidemiology and clinical course. Methods Data were collected of children with PAH after neonatal ASO (≤6 weeks after birth) for simple TGA without residual shunt defects, identified in four national paediatric PAH networks in Europe and one US referral centre. Results Twenty-five children were identified between 1989 and 2014. In 17 children (68%), PAH was detected <1 year after ASO. In the remaining children, PAH was detected after median 64 months (IQR 19.5, 94.5). Nineteen children (96%) received PAH-targeted therapies. During follow-up after ASO (median 5.2 years), eight children died, four underwent lung transplantation and two received a Potts shunt. 1-year and 5-year Potts shunt-and transplantation-free survival after ASO was 100% and 73%. From first PAH detection, this was 100% and 58%, respectively, which did not differ between children with early (<1 year after ASO) or late PAH detection. Conclusions The occurrence of PAH after ASO for TGA represents a specific association. PAH onset may be early or late after ASO, with similar fatal course from first PAH detection. Mechanisms leading to PAH in this association are unknown, but may include abnormal prenatal pulmonary haemodynamics and/or genetic susceptibility. Routine, lifelong follow-up for children who undergo ASO for TGA should include screening for PAH.

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Section: Discussioncontrasting

confidence: 61%

Pulmonary arterial hypertension in children after neonatal arterial switch operation

Zijlstra

Elmasry

Pepplinkhuizen

et al. 2017

Heart

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“…A retrospective search of the Boston Children's Heart Center database identified all patients who fulfilled the following criteria: (1) underwent an ASO at our institution during the first year of life; (2) had at least 1 CMR examination at our laboratory from January 1998 through December 2014; and (3) had an anatomic diagnosis of normal visceral-atrial situs and ventricular D-loop. Patients with inadequate image data for measurements of at least 1 of the following key structures were excluded: (1) great vessel dimension imaged either by a contrast-enhanced 3D magnetic resonance angiography or a noncontrast electrocardiographically gated isotropic 3D steady-state free precession (SSFP) sequence, or (2) ventricular size and function assessed by cine SSFP.…”

Section: Patientsmentioning

confidence: 99%

Cardiovascular Magnetic Resonance Findings Late After the Arterial Switch Operation

Shepard

Germanakis

White

et al. 2016

Circ: Cardiovascular Imaging

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Because the arterial switch operation (ASO) became the standard treatment for infants and children with D-loop transposition of the great arteries (TGA) in the early-to-mid 1980s, survival has improved with the majority of patients now reaching adulthood.1 Although late mortality is rare, complications involving the implanted coronary arteries, pulmonary arteries, and neoaortic valve and root are increasingly recognized as sources of morbidity.2,3 Although echocardiography is capable of providing much of the clinically relevant information for noninvasive assessment of most ASO patients during infancy and childhood, its ability to adequately assess the key anatomic and functional elements in older patients is hindered by deteriorating acoustic windows.4 Cardiovascular magnetic resonance (CMR) has been shown to be a robust imaging modality in adolescents and adult patients with a wide variety of congenital heart disease, including those after the ASO. 5,6 Specifically, unlike echocardiography, CMR is not hindered by body size and is considered a reliable technique for assessment of the great vessels, biventricular size and function, myocardial viability, and valve regurgitation. See Editorial by de Roos See Clinical PerspectiveAlthough CMR can provide comprehensive anatomic and functional assessment in ASO patients, relatively sparse information has been published to date, and the ranges of ventricular and arterial dimensions and functional parameters have not been characterized. The goal of the present study, therefore, was to describe the range of biventricular size and function, frequency and location of focal myocardial fibrosis evaluated by late gadolinium enhancement (LGE) imaging, spectrum of great vessel dimensions, and frequency and severity of neoaortic valve regurgitation (neo-AR) in a large cohort of ASO patients evaluated at our center. In addition, we sought to explore factors associated with left ventricular (LV) enlargement and dysfunction, as well as neo-AR.Background-Despite its robust diagnostic capabilities in adolescents and adult patients after the arterial switch operation, little information is available on the cardiovascular magnetic resonance findings in this population. Methods and Results-The cardiovascular magnetic resonance findings of 220 consecutive patients evaluated in our center were retrospectively reviewed (median age at cardiovascular magnetic resonance, 15.4 years; 66.8% male sex). Compared with published normal values, left and right ventricular end-diastolic volume z scores were mildly enlarged (0.48±1.76 and 0.33±1.5; P=0.0003 and 0.0038, respectively), with 26% of patients having left ventricular dilatation and 20% having right ventricular dilatation. Left ventricular dysfunction was present in 21.5% of patients (mild in most), and only 5.1% of patients had mild right ventricular dysfunction. Myocardial scar was found in 1.8% of patients. Dilatation of the neoaortic root was common (76%), and root z score increased at an average rate of 0.03 points per year. By multivar...

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“…4 The initial short follow-up showed satisfactory results of ASO in comparison with the Mustard and Senning correction, but long-term results have only recently been reported. [5][6][7][8] The patient population with Mustard and Senning correction is now well into adulthood, making longterm follow-up possible. The population of patients with Mustard and Senning correction represents a clinical challenge and can provide us with important insight into the consequences of having a morphological right ventricle as a systemic ventricle.…”

mentioning

confidence: 99%

Long-Term Outcome of Mustard/Senning Correction for Transposition of the Great Arteries in Sweden and Denmark

et al. 2015

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Long-term Outcome and Quality of Life after Arterial Switch Operation: A Prospective Study with a Historical Comparison

Cited by 39 publications

References 33 publications

Pulmonary arterial hypertension in children after neonatal arterial switch operation

Pulmonary arterial hypertension in children after neonatal arterial switch operation

Cardiovascular Magnetic Resonance Findings Late After the Arterial Switch Operation

Long-Term Outcome of Mustard/Senning Correction for Transposition of the Great Arteries in Sweden and Denmark

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