Left-ventricular noncompaction in a pediatric population: predictors of survival.

Abstract:

:Left-ventricular noncompaction (LVNC) is an echocardiographic finding of increasing frequency in pediatrics; however, predictors of outcomes have been difficult to identify. We conducted a retrospective review of pediatric patients at the Morgan Stanley Children's Hospital of New York from January of 1993 to September of 2009 to identify predictors of the primary outcome of death or heart transplantation. LVNC was identified in 50 patients, 34 of them < 1 year of age. Death or transplantation occurred in 26 patients, with a median survival of 1.17 years after presentation. Patients surviving 1 year after presentation had 75% conditional survival, and patients surviving 2 years after presentation had 92% conditional survival. Hemodynamic instability, poor ventricular function, and LV dilatation were each independent predictors of poor outcome. Of the 21 patients who presented with hemodynamic instability, 17 died or underwent transplantation at a median of 0.08 years after presentation. In conclusion, LVNC is recognized more in younger patients; however, age is not a predictor of outcome. Patients who present with hemodynamic instability and poor ventricular function have decreased transplant-free survival, and most poor outcomes occur within the first year after presentation. Therefore, early listing for transplant may lead to better outcomes in this population.

journal_name

Pediatr Cardiol

journal_title

Pediatric cardiology

authors

Zuckerman WA,Richmond ME,Singh RK,Carroll SJ,Starc TJ,Addonizio LJ

doi

10.1007/s00246-010-9868-5

subject

Has Abstract

pub_date

2011-04-01 00:00:00

pages

406-12

issue

4

eissn

0172-0643

issn

1432-1971

journal_volume

32

pub_type

杂志文章
  • Percutaneous closure of ventricular septal defect associated with tunnel-shaped aneurysm using the Amplatzer duct occluder.

    abstract::Different devices have been used for percutaneous closure of ventricular septal defects (VSDs). Over the past 6 months, we have used the Amplatzer duct occluder for VSDs associated with tunnel-shaped aneurysm in three patients here at our institution. Due to the anatomic resemblance between such VSDs and the duct occl...

    journal_title:Pediatric cardiology

    pub_type: 杂志文章

    doi:10.1007/s00246-007-9092-0

    authors: Dilawar M,Numan M,El-Sisi A,Gendi SM,Ahmad Z

    更新日期:2008-03-01 00:00:00

  • Restricting sports for athletes with heart disease: are we saving lives, avoiding lawsuits, or just promoting obesity and sedentary living?

    abstract::Sudden cardiac death in young competitive athletes is tragic and usually due to unsuspected cardiovascular disease. Screening programs for athletes remain debatable, and restriction of athletes from sports can have physical, emotional, and legal ramifications. In this article, we review the epidemiology of the more co...

    journal_title:Pediatric cardiology

    pub_type: 杂志文章,评审

    doi:10.1007/s00246-012-0170-6

    authors: Vaseghi M,Ackerman MJ,Mandapati R

    更新日期:2012-03-01 00:00:00

  • Malignant fibrosarcoma with features of myxoma.

    abstract::Left atrial tumor, diagnosed as myxoma at first investigation, was later demonstrated to be a malignant fibrosarcoma with myxoid change. Myxoma should be diagnosed and managed carefully because of its wide range of histopathologic features. ...

    journal_title:Pediatric cardiology

    pub_type: 杂志文章

    doi:10.1007/s002460010218

    authors: Hishitani T,Ogawa K,Hoshino K,Kido S,Nakamura Y,Ogawa Y

    更新日期:2001-05-01 00:00:00

  • Isolated junctional tachycardia in a patient with pheochromocytoma: an unusual presentation of an uncommon disease.

    abstract::Pheochromocytoma is a rare disease, which often presents a diagnostic challenge due to a variable symptom presentation complex. We report the case of a child who presented with isolated symptoms of palpitations and was found to have junctional tachycardia with subsequent development of cardiomyopathy. Further evaluati...

    journal_title:Pediatric cardiology

    pub_type: 杂志文章

    doi:10.1007/s00246-007-9134-7

    authors: Kim JJ,Valdes SO,Kertesz NJ,Cannon BC

    更新日期:2008-09-01 00:00:00

  • Anomalous origin of the left coronary artery from the pulmonary artery in a premature infant with preserved left ventricular function.

    abstract::Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare condition. The majority of cases present with impaired left ventricular function. We report on a premature infant who was diagnosed at a gestational age near term with ALCAPA during routine cardiac examination. The patient showed...

    journal_title:Pediatric cardiology

    pub_type: 杂志文章

    doi:10.1007/s00246-004-0878-z

    authors: Grosse-Wortmann L,Wenzl T,Hoevels-Guerich HH

    更新日期:2006-03-01 00:00:00

  • Double-outlet single ventricle and an abdominal vascular mass: in utero diagnosis with pathological confirmation.

    abstract::A fetal echocardiographic scan was performed when routine prenatal ultrasound screening failed to identify four cardiac chambers. The scan showed a single ventricle with an associated circoid varicosity. Because of these anomalies, amniocentesis was suggested and trisomy 18 confirmed. The presence of major cardiac str...

    journal_title:Pediatric cardiology

    pub_type: 杂志文章

    doi:10.1007/BF00794843

    authors: Duncan WJ,George D,Ezzat W,Wallace K,Van den Beuken B

    更新日期:1993-01-01 00:00:00

  • Interrupted aortic arch in infancy: a 10-year experience.

    abstract::Fifty infants with interrupted aortic arch (IAA), admitted between 1979 and 1988, were reviewed. They usually presented early in severe cardiac failure or shock. In the initial 5-year period, 17 of the 21 infants underwent diagnostic or confirmatory cardiac catheterization, in contrast with the latter 5 years when onl...

    journal_title:Pediatric cardiology

    pub_type: 杂志文章

    doi:10.1007/BF00838779

    authors: Menahem S,Rahayoe AU,Brawn WJ,Mee RB

    更新日期:1992-10-01 00:00:00

  • Lipoprotein (a): Examination of Cardiovascular Risk in a Pediatric Referral Population.

    abstract::Atherosclerotic cardiovascular disease (CVD), a leading cause of death globally, has origins in childhood. Major risk factors include family history of premature CVD, dyslipidemia, diabetes mellitus, and hypertension. Lipoprotein (a) [Lp(a)], an inherited lipoprotein, is associated with premature CVD, but its impact o...

    journal_title:Pediatric cardiology

    pub_type: 杂志文章

    doi:10.1007/s00246-018-1927-3

    authors: Qayum O,Alshami N,Ibezim CF,Reid KJ,Noel-MacDonnell JR,Raghuveer G

    更新日期:2018-12-01 00:00:00

  • Intra-utero diagnosis of anomalous right ventricular muscle bundles in association with a ventricular septal defect: a case report.

    abstract::The morphology and natural history of anomalous right ventricular muscle bundles (ARVMB) have been described in a number of postnatal studies. Whether this is a congenital or acquired cardiac lesion remains obscure. A fetal echocardiogram performed in a 32-week gestation mother showed a large ventricular septal defect...

    journal_title:Pediatric cardiology

    pub_type: 杂志文章

    doi:10.1007/BF00795736

    authors: Leandro J,Dyck JD,Smallhorn JF

    更新日期:1994-09-01 00:00:00

  • Reintervention Burden and Vessel Growth After Surgical Reimplantation of a Pulmonary Artery During Childhood.

    abstract::Children requiring reimplantation of a branch pulmonary artery (PA) are at risk for postoperative stenosis and impaired growth of the reimplanted PA. Outcomes and risk factors for reintervention and impaired growth are incompletely described. We reviewed data on patients who underwent reimplantation of a branch PA bet...

    journal_title:Pediatric cardiology

    pub_type: 杂志文章

    doi:10.1007/s00246-017-1767-6

    authors: Wu SJ,Downing T,Mascio C,Gillespie MJ,Dori Y,Rome JJ,Glatz AC

    更新日期:2018-02-01 00:00:00

  • Outcomes following electroanatomic mapping and ablation for the treatment of ectopic atrial tachycardia in the pediatric population.

    abstract::Ectopic atrial tachycardia (EAT) is often resistant to medical therapy, with radiofrequency ablation (RFA) being a preferred treatment option. Three-dimensional (3-D) electroanatomic mapping was introduced as a tool for improved substrate localization, although there are no published data with this technology in pedia...

    journal_title:Pediatric cardiology

    pub_type: 杂志文章,多中心研究

    doi:10.1007/s00246-007-9137-4

    authors: Cummings RM,Mahle WT,Strieper MJ,Campbell RM,Costello L,Balfour V,Burchfield A,Frias PA

    更新日期:2008-03-01 00:00:00

  • Bartonella henselae endocarditis in a child.

    abstract::14-year-old white male with a past medical history of congenital bicuspid aortic valve, Streptococcus viridans endocarditis, and pulmonary valve homograft presented with culture-negative endocarditis. Molecular studies identified the causative organism as Bartonella henselae and subsequent serologic studies supported ...

    journal_title:Pediatric cardiology

    pub_type: 杂志文章

    doi:10.1007/s00246-006-1383-3

    authors: Pitchford CW,Creech CB 2nd,Peters TR,Vnencak-Jones CL

    更新日期:2006-11-01 00:00:00

  • Acute occlusion of a simple aortic coarctation presenting as abdominal angina.

    abstract::A 9-year-old boy whose past history was remarkable for a heart murmur was diagnosed with abdominal angina due to acute occlusion of a simple coarctation of the aorta. Using a single cross-clamp, we accomplished an end-to-end anastomosis after resection of the coarctation. Paradoxical hypertension and abdominal angina ...

    journal_title:Pediatric cardiology

    pub_type: 杂志文章

    doi:10.1007/s00246-002-0381-3

    authors: Ingu A,Morikawa M,Fuse S,Abe T

    更新日期:2003-09-01 00:00:00

  • Interparametric correlation between echocardiographic markers in preterm infants with patent ductus arteriosus.

    abstract::Various echocardiographic parameters are determined in the assessment and evaluation of a patent ductus arteriosus. In isolation, many of these parameters have a low sensitivity and specificity for ductal hemodynamic significance compared with ductal size. This study aimed to correlate various echocardiographic parame...

    journal_title:Pediatric cardiology

    pub_type: 杂志文章

    doi:10.1007/s00246-013-0640-5

    authors: Sehgal A,Menahem S

    更新日期:2013-06-01 00:00:00

  • Ventricular aneurysm or diverticulum? Clinical differential diagnosis.

    abstract::Intrathoracic ventricular aneurysms and diverticula can be differentiated by several criteria. Contractility is the only reliable parameter: aneurysms expand, whereas diverticula contract during ventricular systole. ...

    journal_title:Pediatric cardiology

    pub_type: 杂志文章

    doi:10.1007/s002460010264

    authors: Krasemann T,Gehrmann J,Fenge H,Debus V,Loeser H,Vogt J

    更新日期:2001-09-01 00:00:00

  • Multiple risk factors before pediatric cardiac transplantation are associated with increased graft loss.

    abstract::Identification of heart transplant recipients at highest risk for a poor outcome could lead to improved posttransplantation survival. A chart review of primary heart transplantations from 1993 to 2006 was performed. Analysis was performed to evaluate the risk of graft loss for those with a transplantation age less tha...

    journal_title:Pediatric cardiology

    pub_type: 杂志文章

    doi:10.1007/s00246-011-0077-7

    authors: Auerbach SR,Richmond ME,Chen JM,Mosca RS,Quaegebeur JM,Addonizio LJ,Hsu DT,Lamour JM

    更新日期:2012-01-01 00:00:00

  • Epidemiology, etiology, and management of Kawasaki disease: state of the art.

    abstract::Since its first description in Japan 30 years ago, Kawasaki disease has been reported worldwide. Although an infectious etiology is suspected based on the epidemiology and clinical features, a causative agent has not been identified. The majority of the morbidity and mortality associated with this condition is attribu...

    journal_title:Pediatric cardiology

    pub_type: 杂志文章,评审

    doi:10.1007/s002469900436

    authors: Laupland KB,Dele Davies H

    更新日期:1999-05-01 00:00:00

  • Two-dimensional echocardiographic visualization of the aortic arch by right parasternal scanning in neonates and infants.

    abstract::Echocardiography has previously been of limited use in visualizing the aorta in neonates and infants. Using a new technique--right parasternal scanning--the aortic arch and brachiocephalic vessels were studied in 50 children under 18 months of age. Complete visualization was accomplished in 47 patients including those...

    journal_title:Pediatric cardiology

    pub_type: 杂志文章

    doi:10.1007/BF02426973

    authors: George L,Waldman JD,Kirkpatrick SE,Turner SW,Pappelbaum SJ

    更新日期:1982-01-01 00:00:00

  • The Action of Smooth Muscle Cell Potassium Channels in the Pathology of Pulmonary Arterial Hypertension.

    abstract::Many different types of potassium channels with various functions exist in pulmonary artery smooth muscle cells, contributing to many physiological actions and pathological conditions. The deep involvement of these channels in the onset and exacerbation of pulmonary arterial hypertension (PAH) also continues to be rev...

    journal_title:Pediatric cardiology

    pub_type: 杂志文章,评审

    doi:10.1007/s00246-016-1491-7

    authors: Hayabuchi Y

    更新日期:2017-01-01 00:00:00

  • Diagnostic and Prognostic Value of Plasma Levels of Cardiac Myosin Binding Protein-C as a Novel Biomarker in Heart Failure.

    abstract::Heart failure (HF) has high morbidity and mortality in children. This study aimed to investigate the value of cardiac myosin binding protein-C (cMyBP-C) as a diagnostic and prognostic biomarker in children with heart failure. This study was a prospective case-control study that involved 50 children with acute HF and 2...

    journal_title:Pediatric cardiology

    pub_type: 杂志文章

    doi:10.1007/s00246-016-1532-2

    authors: El Amrousy D,Hodeib H,Suliman G,Hablas N,Salama ER,Esam A

    更新日期:2017-02-01 00:00:00

  • Vertically oriented intramural right coronary artery in complete transposition of the great arteries: prospective recognition and surgical implications.

    abstract::This report describes a rare case of complete transposition of the great arteries (TGA) with an intramural right coronary artery arising well above the sinotubular junction. Additionally in this case, the left circumflex artery arose anomalously as a branch from the right coronary. Identification of this anatomic anom...

    journal_title:Pediatric cardiology

    pub_type: 杂志文章

    doi:10.1007/s00246-011-0108-4

    authors: Johnson JA,Burkhart HM,O'Leary PW

    更新日期:2012-01-01 00:00:00

  • A multicenter, long-term study on arrhythmias in children with Ebstein anomaly.

    abstract::To assess the prevalence, history, and treatment of arrhythmias, in particular preexcitation and Wolff-Parkinson-White (WPW) syndrome, in patients with Ebstein anomaly (EA) during childhood and adolescence, we performed a multicenter retrospective study of all consecutive live-born patients with EA, diagnosed, and fol...

    journal_title:Pediatric cardiology

    pub_type: 杂志文章,多中心研究

    doi:10.1007/s00246-009-9590-3

    authors: Delhaas T,Sarvaas GJ,Rijlaarsdam ME,Strengers JL,Eveleigh RM,Poulino SE,de Korte CL,Kapusta L

    更新日期:2010-02-01 00:00:00

  • Warfarin therapy for giant aneurysm prevents myocardial infarction in Kawasaki disease.

    abstract::We retrospectively investigated the effect of warfarin therapy in improving the clinical outcome of Kawasaki disease (KD) patients with giant coronary aneurysms (GAs). We followed 2350 KD patients from 1973 to 2004. The GAs (> or =8 mm in diameter) were diagnosed by coronary angiography. Sixty-eight patients (54 males...

    journal_title:Pediatric cardiology

    pub_type: 杂志文章

    doi:10.1007/s00246-007-9132-9

    authors: Sugahara Y,Ishii M,Muta H,Iemura M,Matsuishi T,Kato H

    更新日期:2008-03-01 00:00:00

  • The Significance of Crochetage on the R wave of an Electrocardiogram for the Early Diagnosis of Pediatric Secundum Atrial Septal Defect.

    abstract::The purpose of this study was to test the hypothesis that the incidence of crochetage on the R wave in inferior limb leads can be used for the diagnosis of pediatric secundum atrial septal defect (ASD). Two hundred fifty-six children with secundum ASD (case cohort) and 256 age- and gender-matched children without hear...

    journal_title:Pediatric cardiology

    pub_type: 杂志文章

    doi:10.1007/s00246-018-1857-0

    authors: Shen L,Liu J,Li JK,Xu M,Yuan L,Zhang GQ,Wang JY,Huang YJ

    更新日期:2018-06-01 00:00:00

  • Latent rheumatic heart disease: outcomes 2 years after echocardiographic detection.

    abstract::Screening with portable echocardiography has uncovered a large burden of latent rheumatic heart disease (RHD) among asymptomatic children in endemic regions, the significance of which remains unclear. This study aimed to determine the 2-year outcomes for children with latent RHD diagnosed by echocardiographic screenin...

    journal_title:Pediatric cardiology

    pub_type: 杂志文章

    doi:10.1007/s00246-014-0925-3

    authors: Beaton A,Okello E,Aliku T,Lubega S,Lwabi P,Mondo C,McCarter R,Sable C

    更新日期:2014-10-01 00:00:00

  • Ebstein's anomaly and tricuspid valve dysplasia: prognosis after diagnosis in utero.

    abstract::Tricuspid valve malformation is a rare congenital heart disease. Prenatal diagnosis of Ebstein's anomaly (EA) and tricuspid valve dysplasia (TVD) is associated with high mortality. There are conflicting reports concerning accurate prognostication after diagnosis in utero. The aim of our study was to assess prognostic ...

    journal_title:Pediatric cardiology

    pub_type: 杂志文章

    doi:10.1007/s00246-012-0355-z

    authors: Barre E,Durand I,Hazelzet T,David N

    更新日期:2012-12-01 00:00:00

  • Prevalence of Coronary Artery Disease Risk Factors and Metabolic Syndrome in Children with Heart Disease.

    abstract::Children with acquired and congenital heart disease (CHD) are increasingly surviving to adulthood. Our aim was to determine the prevalence of coronary artery disease (CAD) risk factors in children at known risk for early CAD or with severe CHD. We recruited children (8-19 years) at risk for early CAD-Kawasaki disease ...

    journal_title:Pediatric cardiology

    pub_type: 杂志文章

    doi:10.1007/s00246-017-1750-2

    authors: Ware AL,Young PC,Weng C,Presson AP,Minich LL,Menon SC

    更新日期:2018-02-01 00:00:00

  • Aneurysm of the ventricular membranous septum: serial echocardiographic studies.

    abstract:OBJECTIVES:The aim of this article is to examine the frequency, timing of formation of aneurysm of the ventricular membranous septum (AVMS), and prognosis in patients with a perimembranous ventricular septal defect (VSD). PATIENTS:One hundred forty-eight patients were diagnosed with a perimembranous VSD within 90 days...

    journal_title:Pediatric cardiology

    pub_type: 杂志文章

    doi:10.1007/s00246-003-0572-6

    authors: Miyake T,Shinohara T,Nakamura Y,Fukuda T,Tasato H,Toyohara K,Tanihira Y

    更新日期:2004-07-01 00:00:00

  • Echocardiographic detection of coronary air embolism as the cause of cardiac arrest in a neonate with transposition of the great arteries.

    abstract::A hemodynamically stable neonate with transposition of the great arteries suddenly went into cardiac arrest during preparation for transport to the operating room. Emergency echocardiography during cardiac massage detected coronary air embolism as the presumed cause of arrest. After about 15 minutes of resuscitation, ...

    journal_title:Pediatric cardiology

    pub_type: 杂志文章

    doi:10.1007/s00246-011-0088-4

    authors: Weidenbach M,Riede FT,Dähnert I

    更新日期:2011-12-01 00:00:00

  • Quadrivalvar replacement in infantile Marfan syndrome.

    abstract::Marfan syndrome (MS) is a connective tissue disease involving the cardiovascular, ocular, and the musculoskeletal systems. MS has variable phenotypic expression and is most often diagnosed in adult life. Infantile-onset MS is rare and is associated with severe cardiovascular manifestations; there is an extremely high ...

    journal_title:Pediatric cardiology

    pub_type: 杂志文章

    doi:10.1007/s00246-006-0066-4

    authors: Strigl S,Quagebeur JM,Gersony WM

    更新日期:2007-09-01 00:00:00