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1.
World J Pediatr Congenit Heart Surg ; 14(2): 155-160, 2023 03.
Article in English | MEDLINE | ID: mdl-36866598

ABSTRACT

Background: Regionalization of care for children with congenital heart disease has been proposed as a method to improve outcomes. This has raised concerns about limiting access to care. We present the details of a joint pediatric heart care program (JPHCP) which utilized regionalization and actually improved access to care. Methods: In 2017, Kentucky Children's Hospital (KCH) launched the JPHCP with Cincinnati Children's Hospital Medical Center (CCHMC). This unique satellite model was the product of several years of planning, leading to a comprehensive strategy with shared personnel, conferences, and a robust transfer system; "one program-two sites." Results: Between March 2017 and the end of June 2022, 355 operations were performed at KCH under the auspices of the JPHCP. As of the most recent published Society of Thoracic Surgeons (STS) outcome report (through the end of June 2021), for all STAT categories, the JPHCP at KCH outperformed the STS overall in postoperative length of stay, and the mortality rate was lower than expected for the case mix. Of the 355 operations, there were 131 STAT 1, 148 STAT 2, 40 STAT 3, and 36 STAT 4 operations, with two operative mortalities: an adult undergoing surgery for Ebstein anomaly, and a premature infant who died from severe lung disease many months after aortopexy. Conclusions: With a select case mix, and by affiliating with a large volume congenital heart center, the creation of the JPHCP at KCH was able to achieve excellent congenital heart surgery results. Importantly, access to care was improved for those children at the more remote location utilizing this one program-two sites model.


Subject(s)
Cardiac Surgical Procedures , Ebstein Anomaly , Heart Defects, Congenital , Infant , Infant, Newborn , Adult , Child , Humans , Cardiac Surgical Procedures/methods , Heart Defects, Congenital/surgery , Infant, Premature , Databases, Factual , Health Services Accessibility
2.
J Am Soc Echocardiogr ; 31(10): 1146-1150, 2018 10.
Article in English | MEDLINE | ID: mdl-30076010

ABSTRACT

BACKGROUND: Supraventricular tachycardia (SVT) is the most common sustained arrhythmia in children. Infants with SVT and ventricular preexcitation (Wolff-Parkinson-White syndrome) are known to have up to 30% prevalence of congenital heart disease (CHD). Infants without ventricular preexcitation who present with SVT at <1 year of age have a similar prevalence of CHD. However, for children without ventricular preexcitation who present with SVT at older ages, the prevalence of CHD is not known. The aim of this study was to determine the prevalence of CHD in older children and adolescents presenting with SVT without ventricular preexcitation, with the goal of providing guidance regarding the usefulness of echocardiography in this patient population. METHODS: Children aged 2 to 18 years presenting with confirmed SVT between January 2011 and December 2015 were included in this retrospective review. Patients with any history of ventricular preexcitation or preexisting heart disease were excluded. Medical records were reviewed, and electrocardiographic and echocardiographic findings were classified as normal, incidental, or abnormal. RESULTS: Two hundred ninety patients met the inclusion criteria. Echocardiographic examinations were completed on 224 patients. Only one patient was found to have CHD, a moderate primum atrial septal defect. This patient was noted to have electrocardiographic abnormalities consistent with primum atrial septal defect. CONCLUSIONS: For older children and adolescents with no known heart disease presenting with SVT without ventricular preexcitation, echocardiography may not be a necessary part of initial evaluation when the results of physical examination and electrocardiography are normal.


Subject(s)
Echocardiography/methods , Heart Ventricles/growth & development , Tachycardia, Supraventricular/diagnosis , Ventricular Function, Left/physiology , Adolescent , Child , Child, Preschool , Electrocardiography , Female , Follow-Up Studies , Heart Defects, Congenital/complications , Heart Defects, Congenital/diagnosis , Heart Defects, Congenital/physiopathology , Heart Ventricles/physiopathology , Humans , Male , Reproducibility of Results , Retrospective Studies , Tachycardia, Supraventricular/etiology , Tachycardia, Supraventricular/physiopathology
3.
Clin Cardiol ; 38(7): 401-6, 2015 Jul.
Article in English | MEDLINE | ID: mdl-26072711

ABSTRACT

The Intersocietal Accreditation Commission (IAC) accredits vascular, echocardiography, nuclear medicine, computed tomography, and magnetic resonance imaging laboratories. How facilities involved in the accreditation process view accreditation is unknown. The objective of this study was to examine the perception of laboratory accreditation from those who had undergone the process. An electronic survey request was sent to all facilities that had received IAC accreditation at least once. Demographic information, as well as opinions on the perceived value of accreditation as it relates to 15 quality metrics was acquired. Responses were obtained from 2782 facilities. Of the 15 quality metrics examined, the process was perceived as leading to improvements by a majority of respondents for 10 (67%) metrics including: report standardization, adherence to guidelines, test standardization, report completeness, identification of deficiencies, improved staff knowledge, report timeliness, distinguished facility, correction of deficiencies, and image quality. Overall, the perceived improvement was greater for hospital-based facilities (global 66% vs 59%; P < 0.001). Survey data demonstrate that the accreditation process has a positive perceived impact on the majority of examined metrics. These findings suggest that those undergoing the process find value in accreditation.


Subject(s)
Accreditation , Ambulatory Care Facilities/standards , Diagnostic Imaging/standards , Perception , Quality of Health Care , Humans , Internet , Quality Improvement , Surveys and Questionnaires
4.
J Nucl Cardiol ; 22(3): 496-503, 2015 Jun.
Article in English | MEDLINE | ID: mdl-25352528

ABSTRACT

BACKGROUND: The Medicare Improvements for Patients and Providers Act requires accreditation for all non-hospital suppliers of nuclear cardiology, nuclear medicine, and positron emission tomography (PET) studies as a condition of reimbursement. The perceptions of these facilities regarding the value and impact of the accreditation process are unknown. We conducted an electronic survey to assess the value of nuclear cardiology accreditation. METHODS: A request to participate in an electronic survey was sent to the medical and technical directors (n = 5,721) of all facilities who had received Intersocietal Accreditation Commission (IAC) Nuclear/PET accreditation. Demographic information, as well as, opinions on the value of accreditation as it relates to 16 quality metrics was obtained. RESULTS: There were 664 (11.6%) respondents familiar with the accreditation process of which 26% were hospital-based and 74% were nonhospital-based. Of the quality metrics examined, the process was perceived as leading to improvements by a majority of all respondents for 10 (59%) metrics including report standardization, report completeness, guideline adherence, deficiency identification, report timeliness, staff knowledge, facility distinction, deficiency correction, acquisition standardization, and image quality. Overall, the global perceived improvement was greater for hospital-based facilities (63% vs 57%; P < .001). Ninety-five percent of respondents felt that accreditation was important. Hospital-based facilities were more likely to feel that accreditation demonstrates a commitment to quality (43% vs 33%, P = .029), while nonhospital-based facilities were more likely to feel accreditation is important for reimbursement (50% vs 29%, P≤ .001). CONCLUSION: Although the accreditation process is demanding, the results of the IAC survey indicate that the accreditation process has a positive perceived impact for the majority of examined quality metrics, suggesting the facilities find the process to be valuable.


Subject(s)
Cardiology/organization & administration , Nuclear Medicine/organization & administration , Accreditation , Algorithms , Attitude of Health Personnel , Cardiology/methods , Guideline Adherence , Hospitals , Medicare , Nuclear Medicine/methods , Positron-Emission Tomography , Quality Improvement , Quality of Health Care , Surveys and Questionnaires , United States
5.
J Am Coll Cardiol ; 57(1): 76-85, 2011 Jan 04.
Article in English | MEDLINE | ID: mdl-21185505

ABSTRACT

OBJECTIVES: The aim of this study was to investigate the possible effects of antiretroviral therapy (ART) in utero on cardiac development and function in human immunodeficiency virus (HIV)-negative children. BACKGROUND: ART reduces vertical HIV transmission. Long-term cardiotoxicity after in utero exposure to ART is unknown in children but has occurred in young animals. METHODS: Using a prospective multisite cohort study design, echocardiograms taken between birth and 24 months were compared in 2 groups of HIV-negative infants of HIV-positive mothers: 136 infants exposed to ART (ART+) and 216 unexposed infants (ART-). RESULTS: Mean left ventricular (LV) mass z-scores were consistently lower in ART+ girls than in ART- girls: differences in mean z-scores were -0.46 at birth (p = 0.005), -1.02 at 6 months (p < 0.001), -0.74 at 12 months (p < 0.001), and -0.79 at 24 months (p < 0.001). Corresponding differences in z-scores for boys were smaller: 0.13 at 1 month (p = 0.42), -0.44 at 6 months (p = 0.01), -0.15 at 12 months (p = 0.37), and -0.21 at 24 months (p = 0.21). Septal wall thickness and LV dimension were smaller than expected in ART+ infants, but LV contractility was consistently about 1 SD higher at all ages (p < 0.001). In ART+ infants, LV fractional shortening was higher than in ART- infants; girls showed a greater difference. CONCLUSIONS: Fetal exposure to ART is associated with reduced LV mass, LV dimension, and septal wall thickness z-scores and increased LV fractional shortening and contractility up to age 2 years. These effects are more pronounced in girls than in boys. Fetal ART exposure may impair myocardial growth while improving depressed LV function.


Subject(s)
Anti-Retroviral Agents/adverse effects , HIV Seropositivity/drug therapy , HIV/immunology , Heart/drug effects , Maternal Exposure/adverse effects , Pregnancy Complications, Infectious/drug therapy , Ventricular Function, Left/drug effects , Adult , Anti-Retroviral Agents/therapeutic use , Antiretroviral Therapy, Highly Active/adverse effects , Child, Preschool , Female , Follow-Up Studies , Heart/embryology , Humans , Infant , Infant, Newborn , Infectious Disease Transmission, Vertical , Male , Pregnancy , Prospective Studies , Risk Factors
6.
Am J Perinatol ; 25(5): 277-81, 2008 May.
Article in English | MEDLINE | ID: mdl-18401841

ABSTRACT

Cardiac malposition is a rare but important finding when detected on fetal ultrasound. The purpose of this study was to evaluate the incidence of fetal cardiac malposition, associated abnormalities, and clinical outcome in a tertiary-care medical center. Records of fetuses (1993 to 2006) with dextroposition, dextrocardia, mesocardia, ectopia cordis, or heterotaxy were reviewed. The presence of congenital heart disease (CHD), extracardiac anomalies, and outcome were noted. Cardiac malposition was present in 101 fetuses among a total of 3313 (3%) pregnancies. In 78 (78%) patients, the heart was positioned in the right hemithorax. Of those, 26 (33%) had dextrocardia (CHD = 21), and 52 (67%) had dextroposition (CHD = 14). Sixteen (16%) patients had mesocardia (CHD = 8), and 7 (7%) had ectopia cordis (CHD = 6). The majority (58%) of fetal cardiac malposition was caused by intrathoracic masses. Concomitant CHD occurred in 50%. Outcome was available in 97%. The perinatal and neonatal mortality rate was 30%; the elective termination rate was 4%. Patients with CHD had higher mortality rates. The highest mortality rates occurred in ectopia cordis and combined disease of diaphragmatic hernia and CHD.


Subject(s)
Fetal Diseases/epidemiology , Heart Defects, Congenital/epidemiology , Abortion, Eugenic , Adolescent , Adult , Female , Fetal Diseases/mortality , Heart Defects, Congenital/complications , Heart Defects, Congenital/mortality , Hernia, Diaphragmatic/complications , Hernia, Umbilical/complications , Humans , Infant , Infant, Newborn , Pregnancy , Pregnancy Outcome , Retrospective Studies , Ultrasonography, Prenatal
8.
J Am Coll Cardiol ; 47(11): 2283-8, 2006 Jun 06.
Article in English | MEDLINE | ID: mdl-16750697

ABSTRACT

OBJECTIVES: The aim of this work was to evaluate myocardial contractility using the end-systolic wall stress (ESSm)-velocity of circumferential fiber shortening (VCFc) relationship in sickle cell anemia (SCA) patients compared with a similar age group of African-American (AA) control patients. BACKGROUND: Abnormalities of myocardial function have been documented in SCA patients using load-dependent echocardiographic indexes. Whether the systolic dysfunction results from impaired myocardial contractility or altered loading conditions is unknown because controlled studies using a load-independent measure of contractility have not been performed. METHODS: Fifty healthy AA patients and 57 SCA patients age 3 months to 18 years were studied. Simultaneous indirect arterial pulse tracing, phonocardiogram, electrocardiogram, and M-mode tracing of the left ventricular (LV) short-axis were recorded. The LV dimensions, corrected ejection time (ETc), percent fractional shortening (%FS), VCFc, and ESSm were determined. The ESSm-VCFc relationship was calculated and compared between groups. Duration and severity of anemia and effects of exchange transfusion on the ESSm-VCFc relationship were determined. RESULTS: The SCA patients had increased LV dimensions in systole and diastole, and increased indexed LV mass. Load-dependent measurements of LV function (ETc, %FS, and VCFc) were lower in SCA patients, and afterload, as measured by ESSm, was increased. The ESSm-VCFc relationship demonstrated reduced contractility in SCA patients compared with control subjects. Degree and duration of anemia along with exchange transfusions did not impact contractility. CONCLUSIONS: Sickle cell anemia patients have significant LV dilatation and increased LV mass due to abnormal loading conditions. Contractility, measured by the ESSm-VCFc index, is lower in SCA patients and was not negatively impacted by severity or duration of anemia, or exchange transfusions. The underlying mechanism explaining these findings requires further investigation.


Subject(s)
Anemia, Sickle Cell/physiopathology , Myocardial Contraction , Myofibrils , Systole , Ventricular Function, Left , Adolescent , Black or African American , Anemia, Sickle Cell/blood , Anemia, Sickle Cell/diagnostic imaging , Case-Control Studies , Child , Child, Preschool , Echocardiography , Female , Humans , Infant , Male , Prospective Studies , Severity of Illness Index , Time Factors , Ventricular Function
9.
Pediatr Blood Cancer ; 47(6): 831-3, 2006 Nov.
Article in English | MEDLINE | ID: mdl-16078213

ABSTRACT

We recently cared for a patient with sickle cell disease (SCD) who presented with cardiac arrest and was found to have a prolonged corrected QT interval (QTc). This prompted us to perform a retrospective review of all electrocardiograms performed in patients with SCD during the last two years. Among 142 patients with ECG results, we identified 12 patients (8 males and 4 females), who had one or more documented measurements of prolonged QTc intervals. The relatively high prevalence of borderline or moderately prolonged QTc intervals in our patient population warrants further investigation.


Subject(s)
Anemia, Sickle Cell/complications , Long QT Syndrome/complications , Long QT Syndrome/diagnosis , Adolescent , Child , Child, Preschool , Electrocardiography/methods , Female , Heart Arrest/etiology , Humans , Long QT Syndrome/physiopathology , Male , Retrospective Studies , Sensitivity and Specificity
10.
J Pediatr Hematol Oncol ; 27(12): 654-8, 2005 Dec.
Article in English | MEDLINE | ID: mdl-16344670

ABSTRACT

The prevalence of pulmonary hypertension (PHT) among adolescents with sickle cell disease (SCD) is unknown. A tricuspid regurgitant (TR) jet peak velocity of 2.5 m/s or more is a screening test for PHT. The authors retrospectively reviewed echocardiograms and clinical data of adolescents followed at the Texas Children's Sickle Cell Center. Of 80 evaluable adolescents with SCD and echocardiogram data, 21 (26%) had a TR jet velocity of 2.5 m/s or more. Of these 21 patients with PHT, 12 (57%) had an echocardiogram performed during an inpatient stay for vaso-occlusive crisis (n = 6), acute chest syndrome (n = 4), fever (n = 1), or seizures (n = 1), and 9 (43%) had an echocardiogram performed as an outpatient in a baseline state of health. Elevation of pulmonary artery pressures was common in this adolescent cohort, but clinical symptoms were rare. Prospective study is warranted to determine the prevalence and course of elevated pulmonary artery pressures in this age group.


Subject(s)
Anemia, Sickle Cell/physiopathology , Hypertension, Pulmonary/etiology , Pulmonary Artery , Adolescent , Adult , Anemia, Sickle Cell/complications , Cohort Studies , Echocardiography, Doppler, Color , Female , Follow-Up Studies , Humans , Hypertension, Pulmonary/diagnostic imaging , Male , Pulmonary Artery/diagnostic imaging , Retrospective Studies , Sensitivity and Specificity
11.
J Am Soc Echocardiogr ; 18(8): 830-8, 2005 Aug.
Article in English | MEDLINE | ID: mdl-16084335

ABSTRACT

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.


Subject(s)
Echocardiography, Doppler , Heart Defects, Congenital/physiopathology , Heart Ventricles/physiopathology , Ventricular Function, Left/physiology , Adolescent , Blood Flow Velocity , Child , Child, Preschool , Female , Heart Defects, Congenital/diagnostic imaging , Heart Ventricles/diagnostic imaging , Humans , Infant , Infant, Newborn , Male , Observer Variation , Reproducibility of Results , Retrospective Studies
12.
Am J Cardiol ; 96(4): 599-601, 2005 Aug 15.
Article in English | MEDLINE | ID: mdl-16098320

ABSTRACT

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.


Subject(s)
Fetal Diseases/diagnostic imaging , Pericardial Effusion/etiology , Adolescent , Adult , Chromosome Aberrations/embryology , Echocardiography, Doppler , Female , Fetal Death/epidemiology , Fetal Diseases/embryology , Fetal Diseases/mortality , Follow-Up Studies , Gestational Age , Heart Defects, Congenital/complications , Heart Defects, Congenital/diagnostic imaging , Heart Defects, Congenital/embryology , Humans , Hydrops Fetalis/complications , Hydrops Fetalis/diagnostic imaging , Hydrops Fetalis/embryology , Pericardial Effusion/diagnostic imaging , Pericardial Effusion/mortality , Pregnancy , Retrospective Studies , Survival Rate , Ultrasonography, Prenatal
13.
J Am Soc Echocardiogr ; 18(7): 743-8, 2005 Jul.
Article in English | MEDLINE | ID: mdl-16003272

ABSTRACT

BACKGROUND: The end-systolic wall stress (ESS)-heart rate-corrected velocity of circumferential fiber shortening (VCFc) relationship provides a load-independent assessment of systolic function. Previously published indices may not be appropriate for studies where ethnicity may introduce bias. OBJECTIVE: We sought to investigate potential differences in the ESS-VCFc relationship between the African American (AA) and Caucasian population. METHODS: In all, 50 AAs and 72 Caucasians, age 3 months to 17 years, were studied. Arterial pulse tracing, phonocardiogram, electrocardiogram, and M-mode of the left ventricle were recorded. Left ventricular dimensions, functional indices, ESS, and ESS-VCFc relationships were compared between groups. RESULTS: AAs had decreased indexed left ventricular end-systolic dimensions and increased septal and posterior wall thickness. AAs had increased percent fractional shortening, VCFc, and decreased ESS. Meridional ESS-VCFc relationships for the groups were similar. CONCLUSIONS: Young AAs have slightly thicker ventricles with increased VCFc, lower systolic volumes, and diminished ESS compared with control subjects. Despite differences, the meridional ESS-VCFc relationships were similar and correlated closely to previously reported normal indices.


Subject(s)
Black or African American/statistics & numerical data , Heart Ventricles/diagnostic imaging , Myocardial Contraction/physiology , Stroke Volume/physiology , Ventricular Function, Left/physiology , White People/statistics & numerical data , Adolescent , Age Distribution , Child , Child, Preschool , Elasticity , Female , Heart Rate/physiology , Humans , Infant , Male , Sex Distribution , Stress, Mechanical , Ultrasonography , United States/ethnology
14.
Am J Cardiol ; 95(4): 541-3, 2005 Feb 15.
Article in English | MEDLINE | ID: mdl-15695151

ABSTRACT

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.


Subject(s)
Heart Defects, Congenital/mortality , Mitral Valve/abnormalities , Mitral Valve/diagnostic imaging , Ventricular Outflow Obstruction/mortality , Child , Child, Preschool , Echocardiography, Doppler, Color , Female , Heart Defects, Congenital/diagnostic imaging , Heart Defects, Congenital/therapy , Humans , Infant , Infant, Newborn , Male , Mitral Valve/surgery , Prognosis , Retrospective Studies , Texas/epidemiology , Ventricular Outflow Obstruction/diagnostic imaging , Ventricular Outflow Obstruction/therapy
15.
Anesthesiology ; 101(6): 1298-305, 2004 Dec.
Article in English | MEDLINE | ID: mdl-15564936

ABSTRACT

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.


Subject(s)
Anesthetics, Inhalation , Anesthetics, Intravenous , Fentanyl , Heart Septal Defects, Ventricular/physiopathology , Heart Septal Defects, Ventricular/surgery , Heart/drug effects , Methyl Ethers , Midazolam , Neuromuscular Nondepolarizing Agents , Pancuronium , Coronary Angiography , Dose-Response Relationship, Drug , Echocardiography , Echocardiography, Doppler , Female , Heart Bypass, Right , Heart Function Tests , Hemodynamics/drug effects , Humans , Infant , Male , Oxygen/blood , Sample Size , Sevoflurane
16.
Cardiovasc Toxicol ; 4(2): 187-97, 2004.
Article in English | MEDLINE | ID: mdl-15371634

ABSTRACT

Seroreverters (uninfected children of HIV-infected mothers) have exhibited left ventricular (LV) dysfunction. Mitochondrial toxicity associated with in utero or postnatal exposure to highly active antiretroviral therapy (HAART) is a possible mechanism. Adult and animal models have demonstrated associations between LV abnormalities, cardiomyopathy, and components of HAART. Yet, outcomes in children are poorly understood. In this study, we explore HAART-associated LV abnormalities in seroreverters exposed to HAART (n = 144) or never exposed (n = 252). Subjects are drawn from the Women and Infants Transmission Study and the Pediatric Pulmonary and Cardiovascular Complications of HIV Study, respectively. Data include (1) echocardiographic studies of LV structure and function and (2) serologic cardiac biomarkers (cardiac troponin, probrain natriuretic peptide, high-sensitivity C reactive protein), both collected during the first month of life, and again at 6, 12, 24, 36, and 48 months postnatally. Planned analyses include several regression models. At this time, we have access to data for all 252 unexposed children, and 53 exposed subjects are enrolled. The cohorts are similar in terms of gender and race and the recruited subjects are representative of all eligible subjects in terms of exposure to HAART. Recruitment will continue into 2006.


Subject(s)
Anti-HIV Agents/adverse effects , HIV Infections/prevention & control , Heart Diseases/chemically induced , Pregnancy Complications, Infectious/drug therapy , Adult , Anti-HIV Agents/administration & dosage , Anti-HIV Agents/therapeutic use , Antiretroviral Therapy, Highly Active/adverse effects , Biomarkers/blood , Child, Preschool , Cohort Studies , Electrocardiography , Female , HIV Infections/drug therapy , HIV Infections/transmission , HIV Seronegativity , Heart Diseases/embryology , Heart Diseases/physiopathology , Humans , Infant , Infant, Newborn , Infectious Disease Transmission, Vertical/prevention & control , Male , Pregnancy , Time Factors , Ventricular Dysfunction, Left/chemically induced , Ventricular Dysfunction, Left/physiopathology
17.
Pediatrics ; 114(3): 691-6, 2004 Sep.
Article in English | MEDLINE | ID: mdl-15342840

ABSTRACT

OBJECTIVE: Left ventricular outflow tract obstructive (LVOTO) malformations are a leading cause of infant mortality from birth defects. Genetic mechanisms are likely, and there may be a higher rate of asymptomatic LVOTO anomalies in relatives of affected children. This study sought to define the incidence of cardiac anomalies in first-degree relatives of children with congenital aortic valve stenosis (AVS), coarctation of the aorta (CoA), and hypoplastic left heart syndrome (HLHS). METHODS: A total of 113 probands with a nonsyndromic LVOTO malformation of AVS (n = 25), BAV (n = 3), CoA (n = 52), HLHS (n = 30), and aortic hypoplasia with mitral valve atresia (n = 2) were ascertained through chart review or enrolled at the time of diagnosis. Echocardiography was performed on 282 asymptomatic first-degree relatives. RESULTS: Four studies had poor acoustic windows, leaving 278 studies for analysis. BAV were found in 13 (4.68%) first-degree relatives. The relative risk of BAV in the relatives was 5.05 (95% confidence interval: 2.2-11.7), and the broad sense heritability was 0.49, based on a general population frequency of 0.9%. BAV was more common in multiplex families compared with sporadic cases. An additional 32 relatives had anomalies of the aorta, aortic valve, left ventricle, or mitral valve. CONCLUSIONS: The presence of an LVOTO lesion greatly increases the risk of identifying BAV in a parent or sibling, providing additional support for a complex genetic cause. The parents and siblings of affected patients should be screened by echocardiography as the presence of an asymptomatic BAV may carry a significant long-term health risk.


Subject(s)
Aortic Valve/abnormalities , Heart Defects, Congenital/diagnostic imaging , Heart Defects, Congenital/genetics , Aortic Coarctation/diagnostic imaging , Aortic Coarctation/genetics , Aortic Valve/diagnostic imaging , Aortic Valve Stenosis/congenital , Aortic Valve Stenosis/diagnostic imaging , Aortic Valve Stenosis/genetics , Echocardiography, Doppler , Female , Humans , Hypoplastic Left Heart Syndrome/diagnostic imaging , Hypoplastic Left Heart Syndrome/genetics , Male , Parents , Risk , Siblings
18.
Ann Thorac Surg ; 77(4): 1334-40, 2004 Apr.
Article in English | MEDLINE | ID: mdl-15063262

ABSTRACT

BACKGROUND: A growing number of adults with functional single ventricles are presenting as candidates for first-time and redo-Fontan operations. This study describes the clinical presentation and early operative results of adults who have undergone Fontan modifications. METHODS: Between July 1995 and April 2003, 23 patients (>18 years old) had Fontan operations. We retrospectively reviewed their perioperative courses. RESULTS: Twenty-three Fontan operations (first-time [n = 8] and redo [n = 15]) were performed with no early or late deaths. No patient has required reoperation. One patient has been listed for orthotopic heart transplantation. The overall mean age is 23 years (18 to 41 years); mean follow-up, 30 months; median postoperative hospital stay, 8 days (4 to 34 days); and median duration of chest tube drainage, 4 days (2 to 12 days). The postoperative New York Heart Association (NYHA) functional class was improved in 22 of 23 patients. Eight first-time Fontan operations (7 of 8 nonfenestrated) were performed; lateral tunnel (n = 7) and extracardiac conduit (n = 1). Two patients had preoperative arrhythmias. New onset arrhythmias (ventricular tachycardia and sinus node dysfunction), requiring treatment, occurred in two patients. Fifteen redo-Fontan operations (all nonfenestrated) were performed; lateral tunnel (n = 5) and extracardiac conduit (n = 10). Fifteen patients had preoperative arrhythmias, thirteen of which had intraatrial reentry tachycardia (IART) and required antiarrhythmic medications. Concomitant intraoperative radiofrequency ablation (RFA) (n = 11) and cryoablation (n = 1) procedures were performed. In the immediate postoperative period, there was IART recurrence in five patients (post-RFA [n = 4] and postcryoablation [n = 1]). At latest follow-up, no patient is being treated with antiarrhythmic medications. Two patients had new onset atrial arrhythmias that required treatment. CONCLUSIONS: The Fontan operation can be performed in adults with minimal morbidity and improved NYHA functional class. New onset arrhythmias requiring treatment are sources of perioperative morbidity. Complete arrhythmia resolution of the preoperative arrhythmia may not be achieved in the immediate postoperative period in redo-Fontan patients. However, modification (intraoperative radiofrequency ablation-right atrial debulking) of the atrial tachycardia circuits in the redo-Fontan patients can result in complete resolution of preoperative atrial tachyarrhythmias at early follow-up.


Subject(s)
Fontan Procedure , Heart Ventricles/abnormalities , Adolescent , Adult , Arrhythmias, Cardiac/complications , Echocardiography , Heart Defects, Congenital/complications , Heart Defects, Congenital/pathology , Heart Defects, Congenital/surgery , Humans , Postoperative Complications , Reoperation , Retrospective Studies , Treatment Outcome
19.
Circulation ; 109(14): 1756-62, 2004 Apr 13.
Article in English | MEDLINE | ID: mdl-15023880

ABSTRACT

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.


Subject(s)
Cardiomyopathy, Hypertrophic/diagnostic imaging , Ventricular Dysfunction, Left/diagnostic imaging , Adolescent , Cardiomyopathy, Hypertrophic/complications , Cardiomyopathy, Hypertrophic/physiopathology , Chest Pain/etiology , Child , Child, Preschool , Electrocardiography, Ambulatory , Exercise Test , Exercise Tolerance , Female , Follow-Up Studies , Heart Arrest/etiology , Humans , Infant , Male , Oxygen Consumption , Prognosis , Prospective Studies , Single-Blind Method , Tachycardia, Ventricular/etiology , Ultrasonography, Doppler , Ventricular Dysfunction, Left/etiology , Ventricular Function, Left , Ventricular Outflow Obstruction/etiology , Ventricular Outflow Obstruction/physiopathology
20.
J Am Soc Echocardiogr ; 17(3): 212-21, 2004 Mar.
Article in English | MEDLINE | ID: mdl-14981417

ABSTRACT

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.


Subject(s)
Blood Flow Velocity/physiology , Echocardiography, Doppler , Heart/growth & development , Adolescent , Age Factors , Body Surface Area , Child , Child Welfare , Child, Preschool , Female , Heart Rate/physiology , Heart Ventricles/diagnostic imaging , Humans , Infant , Infant Welfare , Infant, Newborn , Male , Mitral Valve/diagnostic imaging , Mitral Valve/physiology , Myocardial Contraction/physiology , Observer Variation , Reference Values , Reproducibility of Results , Retrospective Studies , Statistics as Topic , Stroke Volume/physiology , Texas/epidemiology , Tricuspid Valve/diagnostic imaging , Tricuspid Valve/physiology , Ventricular Function , Ventricular Function, Left/physiology
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