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Advances in pediatric cardiology 2018

Bernstein, Daniel

doi: 10.1097/MOP.0000000000000659
CARDIOVASCULAR MEDICINE: Edited by Daniel Bernstein

Department of Pediatrics (Cardiology), Stanford University, Stanford, California, USA

Correspondence to Daniel Bernstein, Department of Pediatrics (Cardiology), Stanford University, 750 Welch Road Suite 325, Palo Alto, CA 94304, USA. Tel: +1 650 723 7913; e-mail:

In this issue, we introduce a new series of reviews that should be of special interest to the practicing pediatrician, family physician, and pediatric cardiologist. We shift our focus from congenital and acquired heart disease in isolation to the unique aspects of children's cardiovascular disease in the context of pediatric genetic syndromes and two forms of acquired heart disease: Kawasaki disease and the cardiotoxicity associated with the anticancer agent, doxorubicin.

In the introductory article, Mary Ella Pierpont from the University of Minnesota (pp. 601–608) and Maria Cristina Digilio from Bambino Gesù Children's Hospital in Rome (pp. 601–608) provide an update of the current state of our understanding of the genetics of Noonan syndrome, marked by clinical findings of facial dysmorphism, congenital heart disease, and short stature. We learn that the scope of cardiac disease in Noonan syndrome is highly variable depending on the specific gene mutation, with some mutations associated with a high incidence of congenital heart defects and others with hypertrophic cardiomyopathy. New opportunities for treatment based on an understanding of basic mechanisms are discussed.

Moving next to focus on William's syndrome, Tom Collins (pp. 609–615) at Stanford provides an update on our understanding of and treatment options for patients with this multisystem disorder of elastin deficiency. Cardiovascular abnormalities, which occur in 80% of patients with William's syndrome, are the leading cause of morbidity and mortality. Dr Collins reviews the unique aortic and pulmonary arterial pathophysiology in William's syndrome and recent developments in multilevel surgical interventions, which have led to a marked improvement in clinical outcomes compared with balloon angioplasty procedures. Dr Collins demonstrates how careful periprocedural management and planning hold great promise for significant improvements in the cardiovascular outcomes of these patients.

Bruno Marino and colleagues from Bambino Gesu Hospital in Rome then review the congenital heart defects and other cardiovascular issues associated with Down syndrome. With improvements in cardiac surgery, life expectancy in these patients has dramatically increased, and practitioners are brought up to date on issues these patients face as they grow from childhood to adolescence to young adulthood.

Next, Jane Burns from the University of California, San Diego and Tomoyo Matsubara from Dokkyo Medical University, Tokyo, (pp. 623–627) describe the challenges still inherent in the cardiovascular care of patients with Kawasaki Disease, where the search for a cause continues. They review recent progress being made toward improved our understanding of disease pathogenesis, treatment, and long-term outcomes. Epidemiologic studies suggest that there has been a true increase in patient numbers rather than an increase due to increased case ascertainment. Long-term consequences of damage to the coronary arterial wall and myocardium are emerging and these authors suggest that both longitudinal observational studies and clinical trials of adjunctive therapies are a major unmet need.

In the next article, Neha Bansal, Kelley K. Hutchins, and Steven E. Lipshultz from Children's Hospital, Michigan and Shahnawaz M. Amdani from Washington University, St. Louis (pp. 628–638) review chemotherapeutic agents which result in cardiotoxicity. They discuss the clinical and subclinical presentations and the temporal progression of cardiotoxicity, as well as the management of asymptomatic and symptomatic cardiovascular disease in survivors of childhood cancer. Various preventive measures are reviewed including dexrazoxane. Critically, an understanding of the mechanisms of cardiotoxicity induced by these drugs suggests that there is likely no totally ‘safe’ dose of anthracyclines. The authors review several risk factors, including higher cumulative dose, higher anthracycline dose rates, female sex, longer follow-up, younger age at anthracycline treatment, and cardiac irradiation as factors associated with more severe cardiotoxicity.

Finally, Mark Lindsay from Massachusetts General Hospital (pp. 639–644) reviews cardiovascular disease and current management strategies for patients with Marfan syndrome, the most common cause of aortic aneurysm in children. Whereas surgical therapy remains the primary treatment to prevent aortic complications, medical therapy may slow or prevent aortic growth. The authors provide the background required for pediatricians and pediatric cardiologists to manage children and adolescents with Marfan syndrome, including the most important life-threatening manifestations. Several large scale clinical trials and one meta-analysis have compared AT1 blockers to beta-blockers, concluding that these drugs show clinical equivalence and that either class of agent may be an appropriate choice for the medical management of aortic growth in Marfan's syndrome.

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Conflicts of interest

There are no conflicts of interest.

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