Standing on the shoulders of Giants: a citation analysis of the pediatric congenital heart disease literature

Abstract

Objective

The citation history of a published article reflects its impact on the literature over time. We conducted a comprehensive bibliometric analysis to identify the most cited papers on congenital heart disease in children.

Methods

190 journals listed in Journal Citation Reports were accessed via Web of Science. Publications with 250 or more citations were identified from Science Citation Index Expanded (1900-2020) and those relating to structural congenital heart disease in children were reviewed. Articles were ranked by citation count and the 100 most cited were analyzed.

Results

The number of citations ranged from 2,522 to 309 (median 431, IQR 356-518), with 35 published since 2000. All were written in English, most originated from United States (74%), and were published in cardiovascular journals, with Circulation (28%) the most frequent. There were 86 original research articles, including 50 case series, 14 cohort studies and 10 clinical trials. The most cited paper was by Hoffman JI and Kaplan S on the incidence of congenital heart disease. Thirteen authors had four or more publications in the top 100, all of whom had worked in Boston, Philadelphia, San Francisco, or Dallas, and the most prolific author was Newburger JW (9 articles).

Conclusions

Citation analysis provides an historical perspective on scientific progress by assessing the impact of individual articles. Our study highlights the dominant position of US-based researchers and journals in this field. Most of the highly cited articles remain case series, with few randomized controlled trials in congenital heart disease appearing in recent years.

Introduction

Over the last century, the diagnosis and management of children with congenital heart disease has changed rapidly, founded on the work of visionary pioneers, and improved incrementally through advances in knowledge, technique, and technology. Developments in pediatric cardiology, cardiac surgery, imaging, intensive care, genetics, and other areas have radically altered the natural history of congenital heart disease, especially for those needing intervention at an early stage in their life trajectory who have the most to gain. Today, we can offer the prospect of ‘cure’ or successful palliation in almost all structural congenital heart conditions.

Progress can be plotted through landmark articles that have made a major impact on the literature [1,2], providing an insight into the evolution of a specialty [3]. We therefore conducted a comprehensive bibliometric analysis to identify the most-cited papers in the field of structural congenital heart disease in children.

Methods

Journals listed in the 2019 Journal Citation Reports of the Web of Science (Clarivate Analytics, Philadelphia, PA) under the following subject categories were evaluated for inclusion: Cardiac & Cardiovascular Systems (138 journals); Critical Care Medicine (12); Genetics & Heredity (9); Medicine, General & Internal (14); Multidisciplinary Science (4); Pediatrics (13); Respiratory System (4); Surgery (11); and Transplantation (11). To identify the most cited articles, each journal was searched individually using the Science Citation Index Expanded (1900-2020) accessed via the Web of Science portal on May 16, 2020. Articles identified by each journal search were ranked according to the number of times cited, from highest to lowest. An additional search was conducted using key words on congenital heart disease as the topic or title (see supplement).

Publications with 250 or more citations were identified and the abstract and/or full text screened independently by both authors to determine whether it was relevant to structural congenital heart disease in children; articles in any language were included and any discrepancies resolved by discussion. Articles containing only non-human species, adult patients or focusing primarily on arrhythmias, vascular disease, normal cardiac function, anesthesia, cardiomyopathies, or primary pulmonary hypertension were excluded. Relevant articles were ranked according to their citation count and the one hundred most-cited papers were included in the analysis. Data were extracted independently by both authors from the full text publication, sourced online or via national libraries, including the title, journal, year, authors, center and country of origin, language, type of study, level of evidence [4], field of study, topic, and funding source.

Results

One hundred and ninety journals listed in one or more categories of the Journal Citation Reports were included in the search (see supplement) and 134 articles with 250 or more citations which met the criteria were identified. Articles were ranked according to their citation count and the 100 most-cited papers, published in 24 journals, were included in the analysis (table 1).

Table 1. The top 100 most cited publications in structural congenital heart disease in children.

Rank	Publication	Citations
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2	Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax 1971;26(3):240-8.	1631
3	Hagen PT, Scholz DG, Edwards WD. Incidence and size of patent foramen ovale during the first 10 decades of life: an autopsy study of 965 normal hearts. Mayo Clin Proc 1984;59(1):17-20.	1540
4	Van der Linde D, Konings EEM, Slager MA, Witsenburg M, Helbing WA, Takkenberg JJM, Roos-Hesselink JW. Birth prevalence of congenital heart disease worldwide: a systematic review and meta-analysis. J Am Coll Cardiol 2011;58(21):2241-7.	990
5	Gatzoulis MA, Balaji S, Webber SA, Siu SC, Hokanson JS, Poile C, Rosenthal M, Nakazawa M, Moller JH, Gillette PC, Webb GD, Redington AN. Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study. Lancet 2000;356(9234):975-81.	909
6	Schott JJ, Benson DW, Basson CT, Pease W, Silberbach GM, Moak JP, Maron BJ, Seidman CE, Seidman JG. Congenital heart disease caused by mutations in the transcription factor NKX2-5. Science 1998;281(5373):108-11.	874
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99	Niwa K, Perloff JK, Bhuta SM, Laks H, Drinkwater DC, Child JS, Miner PD. Structural abnormalities of great arterial walls in congenital heart disease: light and electron microscopic analyses. Circulation 2001;103(3):393-400.	310
100	Rosenzweig EB, Kerstein D, Barst RJ. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation 1999;99(14):1858-65.	309

The number of citations ranged from 2,522 to 309 (median 431, IQR 356-518) and articles were published between 1939 and 2014, with the most cited paper originating from 2002. The most represented decade was the 2000s (28) followed by the 1990s (20), with 35 published since 2000 (figure 1). All the top 100 papers were published in the English language and the most frequent journals were: Circulation (28), Journal of the American College of Cardiology (10), New England Journal of Medicine (8), Journal of Pediatrics and Journal of Thoracic and Cardiovascular Surgery (both 7); there were seven papers in non-clinical scientific journals, including four in Nature. Most articles originated from the United States (74), followed by Canada (10), United Kingdom (7), Japan (5), France (4), Germany, Italy, and Sweden (2 each), and one each from Austria, the Netherlands, and New Zealand, including seven international collaborations.

Figure 1. Number of articles in the top 100 most cited, by decade of publication.

258x168mm (330 x 330 DPI)

There were 542 individuals listed as authors, of whom 81 appeared more than once. Thirteen authors had four or more publications included in the list, as shown in table 2; all these authors were based at four leading US centers for most or all their publications: Boston Children’s Hospital and Harvard University; Children’s Hospital of Philadelphia and University of Pennsylvania; University of California San Francisco; and/or University of Texas Southwestern Medical Center. The leading pediatric cardiologists were Dr Jane W Newburger (9) and Dr Gil Wernovsky (8) and the leading surgeons were Dr Richard A Jonas (6), Dr Thomas L Spray and Dr J William Gaynor (5 each). 59 papers reported specific funding, with the US National Institutes of Health cited as the sole or a contributing funder in 28 articles.

Table 2. Authors with four or more articles in the 100 most cited.

Author	Publications (n)
Jane W Newburger, Boston Children’s Hospital and Harvard Medical School	9
Gil Wernovsky, Boston Children’s Hospital and Harvard Medical School, and Children’s Hospital of Philadelphia and University of Pennsylvania	8
Richard A Jonas, Boston Children’s Hospital and Harvard Medical School, and Children’s National Medical Center	6
J William Gaynor, Children’s Hospital of Philadelphia and University of Pennsylvania	5
Thomas L Spray, Children’s Hospital of Philadelphia and University of Pennsylvania	5
David Wypij, Boston Children’s Hospital and Harvard School of Public Health	5
David C Bellinger, Boston Children’s Hospital and Harvard Medical School	4
Frank L Hanley, Boston Children’s Hospital and Harvard Medical School, and University of California San Francisco	4
Paul R Hickey, Boston Children’s Hospital and Harvard Medical School	4
Julien I E Hoffman, University of California San Francisco	4
John E Mayer Jr, Boston Children’s Hospital and Harvard Medical School	4
Norman H Silverman, University of California San Francisco, and Stanford University	4
Deepak Srivastava, University of Texas Southwestern Medical Center, and University of California San Francisco	4

Congenital heart disease overall was the most considered disease group (46 articles), followed by the spectrum of single ventricle heart conditions (11), transposition (8), septal defects (7), and patent arterial duct (6). The most common subject was surgical outcomes (19), followed by pathology (18), surgical technique (13), epidemiology (12), and catheter intervention (9) (table 3). There were 86 original research articles, including 50 case series, 14 cohort studies and 10 clinical trials (table 4); one additional case series was published as a conference abstract. The level of evidence of articles by decade of publication is shown in figure 2, with level 4 evidence predominant before the 1980s and level 3 evidence most prevalent in more recent decades; all bar one of the most cited level 1 evidence has been published in the last 30 years.

Table 3. Subjects of studies in the 100 most cited.

Subject	Publications (n)
Surgical outcomes	19
Pathology	18
Surgical technique	13
Epidemiology	12
Catheter intervention	9
Genetics	8
Morphology	8
Medical therapy	6
Fetal	4
Surgical risk modelling	2
Imaging	1

Table 4. Types of studies in the 100 most cited.

Type of study	Publications (n)
Case series	51
Cohort studies	14
Clinical trials	10
Randomized controlled trial	9
Non-randomized trial	1
Single case reports	10
Reviews	7
Narrative reviews	5
Systematic reviews	2
Scientific statements	4
Expert consensus/opinions	2
Case control study	1
Data modelling	1

Figure 2. Level of evidence of articles in the top 100 most cited, by decade of publication.

258x168mm (330 x 330 DPI)

Discussion

The number of times that a published article is cited in the literature reflects its influence and impact over time [1]. Citation counts are being used increasingly as a tool to assess, rank, and compare individuals, institutions, and journals through metrics such as h-index and impact factor [5]. These measures assume that influential researchers and important publications are cited proportionately more often than those of lesser value, providing a measure of impact on the scientific field over time. There is marked variability in the rate of accumulating citations amongst highly cited papers with some peaking early before falling away, whilst others have a long rise to prominence [2]. In this study, we identified the most cited papers on congenital heart disease in children, spanning 75 years, from Robert Gross and John Hubbard’s landmark 1939 study on surgical ligation of a patent arterial duct in Boston, MA [6] to Ariane Marelli and colleagues’ 2014 paper on the lifetime prevalence of congenital heart disease in Quebec, Canada [7]. Whilst many historical classics remain relevant today, one in three of the most highly cited articles was published in the last 20 years.

Types of highly cited study

Much of modern clinical practice in pediatric cardiology and cardiac surgery is based on expert opinion and institutional case series [8], as reflected by the presence of 61 case reports or series amongst the most cited. Many of the older articles were the classic descriptions of syndromes, such as Williams (727 citations), Patau (563), Cantrell (504), Edwards (500), Alagille (475), and Noonan (408). Similarly, seminal reports of eponymous operations or interventions featured prominently, including Fontan (1631 citations), Blalock and Taussig (837), Mustard (527), Rashkind (523), Senning (490), Norwood (473), and Ross (316). Whilst these procedures remain relevant today and continue to attract attention, it is unlikely that such reports published today would have a similar impact.

Twelve of the top 100 articles were epidemiology studies, including five of the top 20 with over 5,700 citations between them. Incidence, prevalence, and natural history are often quoted by authors in their introduction as a way of setting the scene, leading to high numbers of citations for those studies that break through and become the standard reference.

Only nine articles reported the outcomes of randomized controlled trials and five of these were on the early and late findings from the Boston Circulatory Arrest trial [9–13], totaling 2,294 citations and involving 8 of the 12 authors appearing in table 2. The other highly cited randomized controlled trials were the Pediatric Heart Network’s multicenter Single Ventricle Reconstruction trial (463 citations) [14], a national trial of indomethacin in premature infants with a patent arterial duct (427) [15], the multicenter PRIMACORP study of prophylactic milrinone after surgery (404) [16], and the first clinical application of remote ischemic preconditioning for myocardial protection (387) [17]. The low numbers of randomized controlled trials on the list may reflect the lack of late-phase, practice changing trials in pediatric cardiology and cardiac surgery [18,19], or that clinical trials address specific research questions and therefore the findings have a narrower appeal. However, all bar one of these trials were published in the last 30 years yet have garnered sufficient citations to enter the top 100 most cited articles in the field.

Over recent years, clinical guidelines endorsed by national or international bodies have emerged as a key resource for patient management. Four scientific statements from the American Heart Association were included on the list and it is likely that the number of such articles amongst the most cited will increase in the coming decade.

Origins of highly cited studies

We found that the most cited articles on structural congenital heart disease in children were all published in English and dominated by clinical research groups in the United States, as found in other areas of cardiovascular disease [20,21]; only 16 involved authors from non-English speaking countries and four originated from outside Europe or North America. Most articles were publishing in US-based cardiovascular journals, whilst almost a quarter appeared in high-impact general medical (17) or non-clinical scientific (7) journals. It is not surprising that of those rapidly rising studies published in the last 15 years, 14 of 16 (88%) were published in high-impact specialty or general medical journals.

The observation that all authors featuring four or more times had worked at one of four US institutions for most or all their impactful publications reinforces the benefits of ‘team science’. These groups established and sustained successful and collaborative research programs, where success breeds success, although multiple highly cited papers originating from one clinical trial contributed much to this achievement. However, it also raises the possibility of a publication bias in which work from leading US institutions is favored by leading US journals.

The most highly cited papers

At the time of our search, three papers had generated over one thousand citations. Julien Hoffman and Samuel Kaplan’s article on the incidence of congenital heart disease was by far the most cited with 2,522 citations [22]. Published in 2002, it is likely to retain this position for many years, although the systematic review and meta-analysis on worldwide prevalence by Van der Linde and colleagues from 2011 is rising fast, already in fourth place with 990 citations [23]. The second most cited paper was a surgical classic from 1971, Fontan and Baudet’s original description of ventricularization of the right atrium to direct inferior vena caval blood to the left lung, receiving 1,631 citations [24]; the evolution and outcomes of the Fontan procedure have remained the focus of intense research over the last five decades. In third place was Philip Hagen and colleagues’ 1984 autopsy study on the incidence and size of patent foramen ovale by age in children and adults and has grossed over 1,500 citations [25].

Strengths and limitations

This study provides a unique insight into the most influential peer-reviewed publications that have shaped the development of our specialty over the last 100 years, providing a reference list that will be of interest to students, clinicians, and researchers. Our comprehensive search of a wide range of journals listed on the expanded Science Citation Index is likely to have missed few if any of the most cited papers. However, our findings are limited by our use of only one bibliometric tool to determine citation counts; other databases, such as Scopus or Google Scholar, use different data sources and algorithms which may impact on the number of citations detected and therefore which papers appear in the top 100 [26] although as both index fewer articles published prior to the 1990s, they are less suited to historical analyses. Similarly, metrics related to other sources of information, such as number of times accessed online and twitter engagements, were not assessed but are much more relevant to recently published works. Citation analysis demonstrates the overall impact of a paper since publication but is not adjusted to account for its current influence or its quality; infamous papers may receive as much attention as celebrated papers. Like any bibliometric analysis, our findings are a snapshot in time, a now historical view on the most cited articles in the field. Since our search, recently published articles, such as Zaidi et al. in Nature 2013 (426 citations) [27] and Marelli et al. in Circulation 2014 (348) [7], will have continued their rapid rise, whilst others, such as the scientific statement by Pierpont et al. in Circulation 2007 (516) [28], have been superseded by updated reviews [29] and are therefore likely to plateau. As a time-dependent process, other groundbreaking but recently published articles may have had too little time to accrue sufficient citations to make our list.

Conclusions

This study identified the most influential papers on congenital heart disease in children, providing an historical perspective on scientific progress over the last century. Our findings demonstrate that highly cited papers are exclusively published in English and that researchers and journals based in the United States hold a dominant position in this field. Most of the highly cited articles remain case series, with few randomized controlled trials in congenital heart disease appearing in recent years.