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Benjamin W Eidem

Publications and source records attributed to Benjamin W Eidem.

15 recordsLinked to original sources
PubMed2006

Left ventricular non-compaction cardiomyopathy in children: characterisation of clinical status using tissue Doppler-derived indices of left ventricular diastolic relaxation.

BACKGROUND: Left ventricular non-compaction (LVNC) may manifest an undulating phenotype ranging from dilated to hypertrophic appearance. It is unknown whether tissue Doppler (TD) velocities can predict adverse clinical outcomes including death and need for transplantation in children with LVNC. METHODS AND RESULTS: 56 children (median age 4.5 years, median follow-up 26 months) with LVNC evaluated at one hospital from January 1999 to May 2004 were compared with 56 age/sex-matched controls. Children with LVNC had significantly decreased early diastolic TD velocities (Ea) at the lateral mitral (11.0 vs 17.0 cm/s) and septal (8.9 vs 11.0 cm/s) annuli compared with normal controls (p<0.001 for each comparison). Using receiver operator characteristic curves, the lateral mitral Ea velocity proved the most sensitive and specific predictor for meeting the primary end point (PEP) at 1 year after diagnosis (area under the curve = 0.888, SE = 0.048, 95% CI 0.775 to 0.956). A lateral mitral Ea cut-off velocity of 7.8 cm/s had a sensitivity of 87% and a specificity of 79% for the PEP. Freedom from death or transplantation was 85% at 1 year and 77% at 2 years. CONCLUSIONS: TD velocities are significantly reduced in patients with LVNC compared with normal controls. Reduced lateral mitral Ea velocity helps predict children with LVNC who are at risk of adverse clinical outcomes including death and need for cardiac transplantation.

Adolescent↗
PubMed2006

B-type natriuretic peptide predicts adverse cardiovascular events in pediatric outpatients with chronic left ventricular systolic dysfunction.

BACKGROUND: Plasma B-type natriuretic peptide (BNP) levels are elevated in adults with heart failure and correlate with functional classification and prognosis. The range and predictive power of BNP concentrations in children with chronic heart failure, however, are not known. METHODS AND RESULTS: Whole blood BNP concentrations were measured in 53 consecutive patients with chronic left ventricular (LV) systolic dysfunction (biventricular hearts, ejection fraction < 50%, > 3 months since diagnosis). Children who had been hospitalized within 3 months before potential enrollment and those < 2 months or > 21 years of age were excluded. BNP concentrations were measured with the Triage assay (Biosite Diagnostics, Inc, San Diego, Calif). Echocardiographers and clinicians were blinded to BNP levels. An adverse cardiovascular event was defined as cardiac death, cardiac-related hospitalization, or listing for cardiac transplantation. The median age of patients with LV dysfunction was 9.3 years (interquartile range [IQR], 2.7 to 15.1 years). BNP levels were elevated in children with LV dysfunction compared with healthy controls (median, 78 pg/mL [IQR, 22 to 551 pg/mL] versus median, 7 pg/mL [IQR, 5 to 11 pg/mL]; P < 0.0001). Whole blood BNP concentrations were increased in patients who had a 90-day adverse cardiovascular event compared with those who did not (median, 735 pg/mL [IQR, 685 to 1510 pg/mL] versus median, 37 pg/mL [IQR, 14 to 92 pg/mL]; P < 0.001). Patients with a BNP concentration > or = 300 pg/mL were at increased risk of death, hospitalization, or listing for cardiac transplantation (adjusted hazard ratio, 63.6; P < 0.0001). CONCLUSIONS: BNP concentrations are elevated in children with chronic LV systolic dysfunction and predict the 90-day composite end point of death, hospitalization, or listing for cardiac transplantation.

Adolescent↗
PubMed2006

Rapid ventricular remodeling with left ventricular unloading postventricular assist device placement: new insights with strain imaging.

We report the case of a 6-year old girl who presented with severe dilated cardiomyopathy. Cross-sectional echocardiography demonstrated a severely dilated hypokinetic left ventricle with an ejection fraction less than 10%. She developed intractable ventricular tachyarrhythmias and subsequently had a left ventricular assist device implanted. Within 1 week of mechanical support, echocardiography demonstrated dramatic reverse remodeling of the left ventricle with marked improvement in myocardial deformation using strain rate imaging. This report further highlights the potential for rapid remodeling after mechanical support of the failing myocardium.

Cardiomyopathy, Dilated↗
PubMed2005

Genetic predictors and remodeling of dilated cardiomyopathy in muscular dystrophy.

BACKGROUND: Dystrophin gene mutations cause 2 common muscular dystrophies, Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD). Both are frequently associated with dilated cardiomyopathy (DCM) and premature death. We hypothesized that early diagnosis and treatment of DCM in DMD/BMD patients would lead to ventricular remodeling and that specific dystrophin gene mutations would predict cardiac involvement. METHODS AND RESULTS: Sixty-nine boys with DMD (n=62) and BMD (n=7) (mean age, 12.9 and 13.7 years, respectively) were referred to our Cardiovascular Genetics Clinic for evaluation, including echocardiography and DNA analysis. Follow-up evaluations were scheduled yearly until the first abnormal echocardiogram indicative of DCM and quarterly thereafter. After the first abnormal echocardiogram, angiotensin-converting enzyme inhibitor or beta-blocker therapy was started. beta-Blockers were added if echocardiography showed no ventricular remodeling in angiotensin-converting enzyme inhibitor-treated patients after 3 months. DCM was diagnosed in 31 subjects (DMD, 27/62, 44%; BMD, 4/7, 57%) (mean age at onset, 15.4+/-2.8 years; range, 10.4 to 21.2 years). All 31 subjects were begun on pharmacological therapy after diagnosis. On follow-up (n=29), 2 subjects (both DMD) showed stable DCM, 8 subjects (all DMD) showed improvement, and 19 subjects (16 DMD; 3 BMD) showed normalization of left ventricular size and function (total improvement, 27/29 [93%]). DNA analysis in 47 cases (68%) revealed a significant association between DCM and exon 12 and 14 to 17 mutations, possible protection against DCM by exon 51 to 52 mutations, and a trend toward significant association between onset of DCM and exon 31 to 42 mutations. Statistical significance was based on nominal probability values. CONCLUSIONS: Early diagnosis and treatment of DCM may lead to ventricular remodeling in DMD/BMD patients. Specific dystrophin gene mutations appear to be predictive of cardiac involvement, while other mutations may protect against or inhibit development of DCM. Further studies evaluating the impact of early intervention strategies on left ventricular geometry and function in muscular dystrophy patients seem warranted.

Adolescent↗
PubMed2005

Characteristics and outcomes of fetuses with pericardial effusions.

Little is known about the characteristics and outcomes of fetuses with pericardial effusions (PEs); therefore, this study sought to identify factors associated with fetal PEs and the natural histories and outcomes of fetuses with PEs. Large PEs are associated with a greater likelihood of structural heart disease, impaired cardiac function, and chromosomal abnormalities, and PEs with hydrops or extracardiac malformations are associated with death. Most fetal PEs resolve, and fetuses with isolated PEs have a very good prognosis.

Adolescent↗
PubMed2005

Mitral valve morphology and morbidity/mortality in Shone's complex.

Early echocardiographic studies of 50 patients with Shone's complex were retrospectively examined to identify left-sided cardiac features associated with progressive mitral valve (MV) disease requiring intervention, as well as mortality. Thickened MV leaflets, shortened MV chordae coupled with either thickened MV leaflets or turbulence at or below the MV noted by color Doppler, left ventricular outflow tract obstruction without coarctation of the aorta, and mild or moderate aortic insufficiency were associated with a poor prognosis.

Child↗
PubMed2005

Neonates with aortic coarctation and cardiogenic shock: presentation and outcomes.

BACKGROUND: Some neonates with coarctation of the aorta (COA) present with cardiogenic shock and secondary end-organ injury. The management of this subgroup imposes unique challenges. We review our perioperative strategy and outcomes for neonates with COA who presented with cardiogenic shock. METHODS: Neonates (younger than 30 days) with isolated COA or COA with aortic arch hypoplasia were identified. Retrospective review was performed to identify and characterize patients who presented with cardiogenic shock, defined as impaired left ventricular (LV) or right ventricular (RV) systolic function, or both, respiratory failure requiring tracheal intubation, and metabolic acidosis. RESULTS: Thirteen neonates presented in cardiogenic shock and underwent surgical repair. No patients required catheter or surgical reintervention for recoarctation. There were no deaths at a mean follow-up of 54 months. Group I neonates (isolated COA, n = 7) underwent end-to-end anastomosis through left thoracotomy. The mean age and pH at presentation were 9 (+/-1.1) days and 7.07 (+/-0.21), respectively. The mean preoperative and postoperative LV myocardial performance indices (MPI) were 0.81 (+/-0.22) and 0.37 (+/-0.16), respectively (p = 0.002). Group II neonates (COA with arch hypoplasia +/- ventricular septal defect, n = 6) underwent aortic arch advancement and ventricular septal defect closure through median sternotomy. The mean time from diagnosis to surgery in group II was 5.5 (+/-1.9) days versus 2.4 (+/-1.5) days in group 1 (p = 0.01). The mean age and pH at presentation were 11.8 (+/-9.3) days and 7.02 (+/-0.21), respectively. The mean preoperative and postoperative LV MPI were 0.46 (+/-0.13) and 0.35 (+/-0.11), respectively (p = 0.02). The total hospital length of stay in group II patients was 18 (+/-6.23) days versus 11.3 (+/-5. 7) days in group I (p = 0.04). CONCLUSIONS: Timely intervention with a strategy individualized to the patient anatomy can be performed with excellent outcomes in neonates with COA and cardiogenic shock. Neonates with isolated COA had worse preoperative LV MPI, which reflects more significant global left ventricular systolic dysfunction in this subgroup. The elapsed time from diagnosis to surgery was decreased in neonates with isolated COA.

Aortic Coarctation↗
PubMed2005

Impact of chronic left ventricular preload and afterload on Doppler tissue imaging velocities: a study in congenital heart disease.

BACKGROUND: Doppler tissue imaging (DTI) velocities have been reported to be relatively independent of changes in ventricular loading conditions in adult studies. The clinical impact of altered left ventricular (LV) preload and afterload on DTI velocities in children with congenital heart disease has not been adequately evaluated. The purpose of this study was to evaluate the impact of chronic LV preload and afterload on DTI velocities in children with isolated ventricular septal defect and aortic valve stenosis compared with age-matched normal and abnormal (dilated cardiomyopathy) control groups. METHODS: From an apical 4-chamber view, DTI velocities were obtained at the cardiac base at the lateral mitral annulus, lateral tricuspid annulus, and interventricular septum in early diastole, late diastole, and ventricular systole. RESULTS: The majority of DTI velocities did not change significantly in patients with increased LV preload. Patients with increased LV afterload had significantly decreased systolic and early diastolic DTI velocities at both the lateral mitral annulus and ventricular septum compared with control subjects. Children with dilated cardiomyopathy had significantly decreased DTI velocities at all myocardial annular locations. CONCLUSIONS: We conclude that increases in chronic LV preload do not significantly affect the majority of DTI velocities in children with ventricular septal defects. In addition, significantly increased chronic LV afterload in children with aortic valve stenosis is associated with decreased DTI velocities in the absence of other identifiable abnormalities of LV function. Decreased DTI velocity may be secondary to increased afterload or may alternatively be an early marker of subclinical LV longitudinal dysfunction.

Adolescent↗
PubMed2004

Characterization of left ventricular diastolic function by tissue Doppler imaging and clinical status in children with hypertrophic cardiomyopathy.

BACKGROUND: Conventional transmitral Doppler indices are unreliable in assessing clinical status in patients with hypertrophic cardiomyopathy (HCM) because they are affected by loading conditions. This study sought to determine whether tissue Doppler velocities are predictive of adverse clinical outcomes including death, cardiac arrest, ventricular tachycardia (VT), significant cardiac symptoms, and exercise capacity in children with HCM. METHODS AND RESULTS: We studied 80 consecutive children (median age 12 years, median follow-up 26 months) evaluated at 1 hospital from January 1999 to August 2003 compared with 80 age- and gender-matched controls. Patients underwent echocardiography, ambulatory Holter monitoring, and upright exercise testing. Children with HCM had significantly decreased early diastolic tissue Doppler velocities at the lateral mitral (13.2 versus 19.3 cm/s), tricuspid (13.3 versus 16.3 cm/s), and septal (9.4 versus 13.5 cm/s) annuli compared with controls (P<0.001 for each comparison). By forward stepwise regression analysis, early transmitral left ventricular filling velocity (E)/septal Ea ratio predicted death, cardiac arrest, or VT (r=0.610, R2=0.37, P<0.001). Peak oxygen consumption (VO2) was most predictive of children who developed symptoms (r=0.427, R2=0.182, P<0.001). Peak VO2 correlated inversely with E/Ea septal ratio (r=-0.740, P<0.01). CONCLUSIONS: Transmitral E/septal Ea ratio predicts children with HCM who are at risk of adverse clinical outcomes including death, cardiac arrest, VT, and significant cardiac symptoms. Peak VO2 correlated with peak exercise capacity in HCM patients.

Adolescent↗
PubMed2004

Impact of cardiac growth on Doppler tissue imaging velocities: a study in healthy children.

BACKGROUND: Doppler tissue imaging (DTI) is a useful modality to quantitatively assess regional myocardial function. Studies attempting to establish reference values for DTI velocities in healthy children have been limited by small sample sizes and limited age distribution. In addition, the clinical effect of cardiac growth and other demographic and echocardiographic parameters on DTI velocities during childhood has not been adequately evaluated. METHODS: Pulsed wave DTI velocities were obtained in 325 healthy children at the lateral mitral annulus, interventricular septum, and lateral tricuspid annulus during early diastole, late diastole, and ventricular systole and were compared with demographic and echocardiographic study variables. RESULTS: In healthy children, parameters of cardiac growth, most notably left ventricular end-diastolic dimension, have the most significant correlation with the majority of DTI velocities. Age was also significantly correlated with most DTI velocities whereas sex, heart rate, and other echocardiographic parameters demonstrated minimal or no correlation. CONCLUSIONS: This study establishes reference values for DTI velocities and demonstrates the important clinical effects of cardiac growth and age on DTI velocities in neonates and children.

Adolescent↗
PubMed2004

Myocardial performance index with sevoflurane-pancuronium versus fentanyl-midazolam-pancuronium in infants with a functional single ventricle.

BACKGROUND: Patients with congenital heart disease characterized by a functional single ventricle make up an increasing number of patients presenting for cardiac or noncardiac surgery. Conventional echocardiographic methods to measure left ventricular function, i.e., ejection fraction, are invalid in these patients because of altered ventricular geometry. Two recently described Doppler echocardiographic modalities, the myocardial performance index and Doppler tissue imaging, can be applied to single-ventricle patients because they are independent of ventricular geometry. This study assessed the changes in myocardial performance index and Doppler tissue imaging in response to two anesthetic regimens, fentanyl-midazolam-pancuronium and sevoflurane-pancuronium. METHODS: Thirty patients aged 4-12 months with a functional single ventricle were randomized to receive fentanyl-midazolam or sevoflurane. Myocardial performance index and Doppler tissue imaging were measured by transthoracic echocardiography at baseline and two clinically relevant dose levels. RESULTS: Sixteen patients receiving sevoflurane and 14 receiving fentanyl-midazolam were studied. Myocardial performance index was unchanged from baseline with either agent (fentanyl-midazolam: 0.50 +/- 15 baseline vs. 0.51 +/- 0.15 at dose 2; sevoflurane: 0.42 +/- 0.14 baseline vs. 0.46 +/- 0.09 at dose 2). Doppler tissue imaging S (systolic)- and E (early diastolic)-wave velocities in the lateral ventricular walls at the level of the atrioventricular valve annulus were unchanged in the sevoflurane group; however, both Doppler tissue imaging S- and E-wave velocities were decreased significantly from baseline at dose 1 and dose 2 with fentanyl-midazolam, consistent with decreased longitudinal systolic and diastolic ventricular function. CONCLUSIONS: Myocardial performance index, a global measurement of combined systolic and diastolic ventricular function, is not affected by commonly used doses of fentanyl-midazolam or sevoflurane in infants with a functional single ventricle.

Anesthetics, Inhalation↗
PubMed2004

Kawasaki disease in the adult: a case report and review of the literature.

Kawasaki disease, predominantly a disease of childhood, includes such symptoms as acute vasculitis, mucosal inflammation, rash, cervical adenopathy, and edema. Its most severe forms are associated with coronary artery aneurysms. We report a rare case of this disease in an asymptomatic adult and review its epidemiology, etiology, diagnosis, treatment, and prognosis.

Adult↗
PubMed2003

Is cardiac catheterization a prerequisite in all patients undergoing bidirectional cavopulmonary anastomosis?

Traditionally, all patients undergo cardiac catheterization before bidirectional cavopulmonary anastomosis (BCPA). The purpose of this study was to determine if preoperative catheterization is necessary when echocardiographic parameters appear favorable. A retrospective review was performed of all patients who underwent BCPA (n = 142) between February 1996 and May 2001. Echocardiographic criteria defining a favorable BCPA candidate included good ventricular function, moderate or less atrioventricular and semilunar valve regurgitation, absence of ventricular outflow tract obstruction, normal proximal branch pulmonary artery (PA) size, and low PA pressures estimated by PA band gradient or systemic PA shunt velocity. The median age at operation was 7 months (range: 2-11) and weight was 6.2 kg (range: 2.7-7.1). There were 73 unfavorable candidates. Patients with hypoplastic left heart syndrome (n = 23) and pulmonary atresia with intact septum (n = 15) predominated among the unfavorable group. All patients were catheterized. This provided additional information on PA pressures in 3 patients in the favorable group but did not defer operation or influence outcome (no mortality, prolonged pleural drainage, or longer intensive care department stay). All 3 patients are alive at a mean follow-up of 51 months. The 30-day mortality was 2% (4 of 151 patients), all in the unfavorable group. Overall, 20 patients (13%) required arterioplasty of PAs at the time of BCPA. BCPA can be performed with a low risk of morbidity and mortality in a wide range of patients. By using commonly acquired echocardiographic parameters, a low-risk subgroup of patients can be identified who can safely avoid preoperative cardiac catheterization.

Cardiac Catheterization↗
PubMed2003

Heparin-induced thrombocytopenia with associated thrombosis in children after the Fontan operation: report of two cases.

Heparin-induced thrombocytopenia is a widely recognized clinical disorder. The spectrum of disease ranges from clinically insignificant to severe thrombosis (heparin-induced thrombocytopenia with associated thrombosis). Overall, thrombosis occurs in approximately 33% of adults diagnosed with heparin-induced thrombocytopenia and has been associated with high morbidity and mortality rates. Diagnostic testing for this disorder is not standard in children with thrombocytopenia who are receiving heparin, despite the fact that children with congenital heart disease may be exposed to heparin frequently. There are few reported cases of heparin-induced thrombocytopenia with associated thrombosis in children; herein, we describe the cases of 2 children who developed this disorder after undergoing a Fontan operation.

Anticoagulants↗