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1.
Pediatr Cardiol ; 45(5): 976-985, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38485760

ABSTRACT

Adults with congenital heart disease (CHD) benefit from cardiology follow-up at recommended intervals of ≤ 2 years. However, benefit for children is less clear given limited studies and unclear current guidelines. We hypothesize there are identifiable risks for gaps in cardiology follow-up in children with CHD and that gaps in follow-up are associated with differences in healthcare utilization. Our cohort included children < 10 years old with CHD and a healthcare encounter from 2008 to 2013 at one of four North Carolina (NC) hospitals. We assessed associations between cardiology follow-up and demographics, lesion severity, healthcare access, and educational isolation (EI). We compared healthcare utilization based on follow-up. Overall, 60.4% of 6,969 children received cardiology follow-up within 2 years of initial encounter, including 53.1%, 58.1%, and 79.0% of those with valve, shunt, and severe lesions, respectively. Factors associated with gaps in care included increased drive time to a cardiology clinic (Hazard Ratio (HR) 0.92/15-min increase), EI (HR 0.94/0.2-unit increase), lesion severity (HR 0.48 for shunt/valve vs severe), and older age (HR 0.95/month if < 1 year old and 0.94/year if > 1 year old; p < 0.05). Children with a care gap subsequently had more emergency department (ED) visits (Rate Ratio (RR) 1.59) and fewer inpatient encounters and procedures (RR 0.51, 0.35; p < 0.05). We found novel factors associated with gaps in care for cardiology follow-up in children with CHD and altered health care utilization with a gap. Our findings demonstrate a need to mitigate healthcare barriers and generate clear cardiology follow-up guidelines for children with CHD.


Subject(s)
Heart Defects, Congenital , Humans , Heart Defects, Congenital/therapy , Male , Female , Child, Preschool , Risk Factors , Infant , Child , North Carolina/epidemiology , Health Services Accessibility , Retrospective Studies , Patient Acceptance of Health Care/statistics & numerical data , Infant, Newborn , Follow-Up Studies
2.
Catheter Cardiovasc Interv ; 103(4): 565-569, 2024 03.
Article in English | MEDLINE | ID: mdl-38344889

ABSTRACT

Coronary angioplasty in patients with a right-sided heart may be difficult due to challenges in engaging the coronary arteries, interpreting angiogram, and further delivering intracoronary therapies. We present our experience of percutaneous coronary intervention in two cases and propose a practical algorithm to approach cardiac catheterization in these patients.


Subject(s)
Angioplasty, Balloon, Coronary , Dextrocardia , Percutaneous Coronary Intervention , Humans , Treatment Outcome , Percutaneous Coronary Intervention/adverse effects , Coronary Angiography
3.
Pediatr Cardiol ; 44(7): 1520-1528, 2023 Oct.
Article in English | MEDLINE | ID: mdl-37289278

ABSTRACT

The objective of this study was to assess the relationship of prenatal diagnosis of critical congenital heart disease (CHD) to preoperative and postoperative patient findings. Retrospective analysis of neonates with critical CHD who underwent cardiothoracic surgery at one of four centers in North Carolina between 2008 and 2013. Surgical data collected by sites for submission to the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) and the North Carolina CHD Lifespan Database were queried. There were 715 patients with STS records; 558 linked to the NC-CHD database. Patients with prenatal diagnosis had a lower incidence of preoperative risk factors, including need for mechanical ventilation and presence of shock. However, prenatally diagnosed patients had worse short-term outcomes, including higher operative mortality, higher incidence of select postoperative complications, and longer LOS. There was no difference in one-year mortality. Our findings are consistent with current literature which suggests that prenatal diagnosis of critical CHD is associated with a more optimized preoperative clinical status. However, we found that patients with prenatal diagnoses had less favorable postoperative outcomes. This needs to be investigated further, but may be secondary to patient-specific factors, such as CHD disease severity.


Subject(s)
Cardiac Surgical Procedures , Heart Defects, Congenital , Infant, Newborn , Pregnancy , Female , Humans , Cardiac Surgical Procedures/adverse effects , Heart Defects, Congenital/diagnosis , Heart Defects, Congenital/surgery , Retrospective Studies , Prenatal Diagnosis , Risk Factors
4.
Pediatr Cardiol ; 44(2): 472-478, 2023 Feb.
Article in English | MEDLINE | ID: mdl-36454266

ABSTRACT

Children with congenital heart defects (CHDs) are at risk for poor academic performance. The degree to which receipt of health care services is associated with adverse academic outcomes is not known. We examined the association between episodes of cardiac care and third-grade performance in children with CHD. We identified subjects between 1/1/2008 and 4/30/2012 among 5 centers in North Carolina. We classified children by CHD type and linked subjects to the state educational records. Any inpatient or outpatient cardiac encounter on a date of service was considered an encounter. We calculated the number of encounters by adding the number of inpatient or outpatient cardiac visits prior to the date of the end-of-grade (EOG) tests. We estimated the odds of failing third-grade reading or math EOG tests by episodes of care stratified at the 50th percentile, controlling for CHD type, maternal education, sex, race/ethnicity, birth weight, and gestational age. A total of 184 children had third-grade EOG scores linked to health care records. The median number of episodes of care was 4 (range: 1-60). Those with visits ˃ 50th percentile (> 4 encounters/year over the 4.3 year observation period) had 2.09 (95% CI 1.04, 4.21) greater odds of failing the math EOG compared to those ≤ 50th percentile (1-4 encounters). The third-grade math score declined by 1.5 points (P < 0.008) for every 10 episodes of care. There was no association of episodes of care on third-grade reading performance. Children with CHD with > 4 episodes of cardiac care/year may be at risk for delays in third-grade academic performance. Strategies to minimize school absenteeism may improve academic success in this population.


Subject(s)
Academic Performance , Heart Defects, Congenital , Humans , Child , Educational Status , Heart Defects, Congenital/epidemiology , Heart Defects, Congenital/therapy , Schools , North Carolina/epidemiology
5.
World J Pediatr Congenit Heart Surg ; 13(6): 707-715, 2022 11.
Article in English | MEDLINE | ID: mdl-36300264

ABSTRACT

BACKGROUND: The AHA/ACC Adult Congenital Heart Disease guidelines recommend that most adults with congenital heart disease (CHD) follow-up with CHD cardiologists every 1 to 2 years because longer gaps in care are associated with adverse outcomes. This study aimed to determine the proportion of patients in North Carolina who did not have recommended follow-up and to explore predictors of loss to follow-up. METHODS: Patients ages ≥18 years with a healthcare encounter from 2008 to 2013 in a statewide North Carolina database with an ICD-9 code for CHD were assessed. The proportion with cardiology follow-up within 24 months following index encounter was assessed with Kaplan-Meier estimates. Cox regression was utilized to identify demographic factors associated with differences in follow-up. RESULTS: 2822 patients were identified. Median age was 35 years; 55% were female. 70% were white, 22% black, and 3% Hispanic; 36% had severe CHD. The proportion with 2-year cardiology follow-up was 61%. Those with severe CHD were more likely to have timely follow-up than those with less severe CHD (72% vs 55%, P < .01). Black patients had a lower likelihood of follow-up than white patients (56% vs 64%, P = .01). Multivariable Cox regression identified younger age, non-severe CHD, and non-white race as risk factors for a lower likelihood of follow-up by 2 years. CONCLUSION: 39% of adults with CHD in North Carolina are not meeting AHA/ACC recommendations for follow-up. Younger and minority patients and those with non-severe CHD were particularly vulnerable to inadequate follow-up; targeted efforts to retain these patients in care may be helpful.


Subject(s)
Cardiology , Heart Defects, Congenital , Adult , Humans , Female , Adolescent , Male , Heart Defects, Congenital/epidemiology , Heart Defects, Congenital/surgery , Follow-Up Studies , North Carolina/epidemiology , Risk Factors
6.
Eur Heart J Case Rep ; 6(6): ytac204, 2022 Jun.
Article in English | MEDLINE | ID: mdl-35668844

ABSTRACT

Background: Loeys-Dietz syndrome (LDS) is a connective tissue disorder that commonly presents with vascular abnormalities. Owing to the rarity and severity of the condition, consensus guidelines for aortic surgery thresholds vary. In addition, evaluation of coronary arteries in patients with LDS (either routinely or before aortic root surgery) remain undefined. In this case report, we discuss a patient with LDS who found to have an ectatic aortic root and a coronary artery aneurysm and discuss guidelines for evaluation and management in this patient population. Case summary: A 48-year-old woman was incidentally found to have a 45 mm ectatic aortic root during evaluation for a neck mass. As part of pre-operative evaluation for aortic root replacement, left heart catheterization revealed a left main coronary artery aneurysm. Family history revealed aortic aneurysms, sudden cardiac death, and tall height. Physical examination was notable for pectus excavatum and elongated limbs. Workup for inflammatory aetiologies of aortic root dilation was negative. Genetic testing revealed a heterozygous pathogenic TGBF3 variant, consistent with LDS Type 5. She subsequently underwent two-vessel coronary artery bypass, excision of her left main coronary artery aneurysm, and ascending aortic replacement. Discussion: In this case, we describe a patient with LDS who was noted to have a coronary artery aneurysm, a rare finding in the initial presentation of disease. In addition, we examine guidelines regarding evaluation of management of aortic root disease and coronary aneurysms.

7.
JPEN J Parenter Enteral Nutr ; 46(6): 1270-1282, 2022 08.
Article in English | MEDLINE | ID: mdl-34822187

ABSTRACT

BACKGROUND: We aimed to assess safety, tolerability, and improvement in weight gain with an energy- and protein-enriched formula (EPEF) in infants with poor growth. METHODS: Infants aged 1-8 months with poor growth received EPEF for 16 weeks. Our primary objective was improvement in weight as measured by change in weight-for-age z-score (WAZ) and weight gain velocity (grams per day) ≥ median for age. Secondary objectives included improvement in other anthropometric z-scores, formula tolerance, and safety. RESULTS: Twenty-six patients with poor growth due to congenital heart disease (n = 15), other organic causes (n = 9), and nonorganic causes (n = 2) completed the study per protocol. Mean daily energy intake was 123 ± 32 kilocalories per kilogram of body weight, with >90% of energy coming from EPEF. Weight gain velocity exceeded the median for 83% (20 of 24) and 67% (16 of 24) of infants at ≥1 time point and for the overall study period, respectively. Mean ± SD WAZ improved from -2.92 ± 1.04 at baseline to -2.01 ± 1.12 at 16 weeks (P = 0.0001). Z-scores for weight-for-length and head circumference (P = 0.0001) and for length-for-age (P = 0.003) improved significantly at 16 weeks. Compared with baseline, stool consistency was different at 2, 4, and 16 weeks (P < 0.05). There were no significant differences in vomiting, fussiness, or daily number of stools while there was a decrease or no change in spit-up, flatulence, crying, or gassiness. CONCLUSION: EPEF is safe, well tolerated, and improves weight gain in infants with poor growth.


Subject(s)
Malnutrition , Weight Gain , Anthropometry , Humans , Infant , Infant Formula
8.
Child Obes ; 18(6): 361-368, 2022 09.
Article in English | MEDLINE | ID: mdl-34871088

ABSTRACT

Background: Lifestyle behaviors (LB), defined by diet and physical activity, are associated with cardiometabolic health among adults. The association of LB with cardiometabolic health among middle-school children is uncertain. Methods: An abbreviated version of the School Physical Activity and Nutrition survey was used to examine LB among students participating in a wellness program between 2004 and 2018. Students were incorporated into three groups determined by self-reported healthy LB (≥6, 4-5, ≤3 behaviors), including; ≤1 serving/day sugary foods/beverages; ≤1 serving/day fried/fatty foods; ≥1 serving/day fruits and vegetables; ≤2 hours of screen time/day; ≥1 day/week of physical education; ≥1 team sport/year; and ≥1 session/week of moderate to vigorous activity. Baseline cardiometabolic parameters [BMI, lipids, glucose, and blood pressure (BP)], resting heart rate (HR), and HR recovery were examined in association with LB groups. Results: Of 2538 children, 488 (19.2%) reported ≥6, 1219 (48.0%) reported 4-5, and 831 (32.7%) reported ≤3 LB. White or Asian race and higher socioeconomic status were associated with ≥6 LB (p < 0.001). Students performing ≤3 LB exhibited higher BMI (p < 0.001), BP (p = 0.001), resting HR (p < 0.001), and HR recovery (p < 0.001). Students performing ≥6 LB were less likely to be overweight (p < 0.001), obese (p < 0.001), or have low high-density lipoprotein (p = 0.05); however, more likely to have elevated triglycerides (p < 0.01). Conclusions: Among middle-school students, baseline BMI, BP, resting, and recovery HR were higher among children reporting fewer healthy LB. Students performing more healthy LB were less likely to be overweight or obese. Efforts to improve LB among middle-school children may be important for primordial cardiovascular prevention efforts.


Subject(s)
Cardiovascular Diseases , Pediatric Obesity , Cardiovascular Diseases/epidemiology , Cardiovascular Diseases/prevention & control , Child , Health Behavior , Humans , Life Style , Overweight , Pediatric Obesity/epidemiology
9.
Cardiovasc Pathol ; 54: 107370, 2021.
Article in English | MEDLINE | ID: mdl-34273507

ABSTRACT

BACKGROUND: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is commonly associated with myocardial injury and heart failure. The pathophysiology behind this phenomenon remains unclear, with many diverse and multifaceted hypotheses. To contribute to this understanding, we describe the underlying cardiac findings in fifty patients who died with coronavirus disease 2019 (COVID-19). METHODS: Included were autopsies performed on patients with a positive SARS-CoV-2 reverse-transcriptase-polymerase-chain reaction test from the index hospitalization. In the case of out-of-hospital death, patients were included if post-mortem testing was positive. Complete autopsies were performed according to a COVID-19 safety protocol, and all patients underwent both macroscopic and microscopic examination. If available, laboratory findings and echocardiograms were reported. RESULTS: The median age of the decedents was 63.5 years. The most common comorbidities included hypertension (90.0%), diabetes (56.0%) and obesity (50.0%). Lymphocytic inflammatory infiltrates in the heart were present in eight (16.0%) patients, with focal myocarditis present in two (4.0%) patients. Acute myocardial ischemia was observed in eight (16.0%) patients. The most common findings were myocardial fibrosis (80.0%), hypertrophy (72.0%), and microthrombi (66.0%). The most common causes of death were COVID-19 pneumonia in 18 (36.0%), COVID-19 pneumonia with bacterial superinfection in 12 (24.0%), and COVID-19 pneumonia with pulmonary embolism in 10 (20.0%) patients. CONCLUSIONS: Cardiovascular comorbidities were prevalent, and pathologic changes associated with hypertensive and atherosclerotic cardiovascular disease were the most common findings. Despite markedly elevated inflammatory markers and cardiac enzymes, few patients exhibited inflammatory infiltrates or necrosis within cardiac myocytes. A unifying pathophysiologic mechanism behind myocardial injury in COVID-19 remains elusive, and additional autopsy studies are needed.


Subject(s)
COVID-19/pathology , Heart Diseases/pathology , Myocardium/pathology , SARS-CoV-2/pathogenicity , Adult , Aged , Aged, 80 and over , Atherosclerosis/mortality , Atherosclerosis/pathology , Autopsy , COVID-19/immunology , COVID-19/mortality , COVID-19/virology , Comorbidity , Female , Heart Diseases/immunology , Heart Diseases/mortality , Heart Diseases/virology , Host-Pathogen Interactions , Humans , Hypertension/mortality , Hypertension/pathology , Inflammation Mediators/analysis , Male , Middle Aged , Myocardium/immunology , Necrosis , SARS-CoV-2/immunology , Up-Regulation
10.
Pediatr Cardiol ; 42(6): 1308-1315, 2021 Aug.
Article in English | MEDLINE | ID: mdl-33890132

ABSTRACT

With improved surgical outcomes, infants and children with congenital heart disease (CHD) may die from other causes of death (COD) other than CHD. We sought to describe the COD in youth with CHD in North Carolina (NC). Patients from birth to 20 years of age with a healthcare encounter between 2008 and 2013 in NC were identified by ICD-9 code. Patients who could be linked to a NC death certificate between 2008 and 2016 were included. Patients were divided by CHD subtypes (severe, shunt, valve, other). COD was compared between groups. Records of 35,542 patients < 20 years old were evaluated. There were 15,277 infants with an annual mortality rate of 3.5 deaths per 100 live births. The most frequent COD in infants (age < 1 year) were CHD (31.7%), lung disease (16.1%), and infection (11.4%). In 20,265 children (age 1 to < 20 years), there was annual mortality rate of 9.7 deaths per 1000 at risk. The most frequent COD in children were CHD (34.2%), neurologic disease (10.2%), and infection (9.5%). In the severe subtype, CHD was the most common COD. In infants with shunt-type CHD disease, lung disease (19.5%) was the most common COD. The mortality rate in infants was three times higher when compared to children. CHD is the most common underlying COD, but in those with shunt-type lesions, extra-cardiac COD is more common. A multidisciplinary approach in CHD patients, where development of best practice models regarding comorbid conditions such as lung disease and neurologic disease could improve outcomes in this patient population.


Subject(s)
Cause of Death/trends , Heart Defects, Congenital/mortality , Adolescent , Child , Child, Preschool , Female , Humans , Infant , Infant, Newborn , Male , North Carolina/epidemiology , Young Adult
11.
Cureus ; 13(1): e13015, 2021 Jan 30.
Article in English | MEDLINE | ID: mdl-33665045

ABSTRACT

The diagnosis of vascular rings is challenging and may be delayed as symptoms overlap with more common conditions associated with childhood. Underlying genetic associations of this condition remain largely undiscovered. In this report, we present a patient with a double aortic arch and highlight the importance of diagnostic imaging. We also engage in a review of the important genetic considerations.

12.
Cardiol Young ; 31(6): 1030-1033, 2021 Jun.
Article in English | MEDLINE | ID: mdl-33504403

ABSTRACT

Anomalous coronary arteries from the pulmonary artery are uncommon causes of heart failure in the adult population. This case demonstrates the unusual presentation in a patient with anomalous right coronary artery from the pulmonary artery and discusses the complex pathophysiology of this lesion and the role of guideline-directed medical therapy in the management of these patients.


Subject(s)
Coronary Vessel Anomalies , Heart Failure , Adult , Aged , Coronary Angiography , Coronary Vessel Anomalies/diagnosis , Coronary Vessel Anomalies/diagnostic imaging , Heart Failure/diagnosis , Heart Failure/etiology , Humans , Male , Pulmonary Artery
13.
Eur Heart J Case Rep ; 4(FI1): 1-5, 2020 Oct.
Article in English | MEDLINE | ID: mdl-33089057

ABSTRACT

BACKGROUND: Myocardial injury is associated with excess mortality in severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infections, and the mechanisms of injury are diverse. Coagulopathy associated with this infection may have unique cardiovascular implications. CASE SUMMARY: We present a case of 62-year-old male who presented after experiencing syncope and cardiac arrest. Given the clinical presentation and electrocardiographic findings, there was concern for acute coronary syndrome. However, coronary angiogram did not reveal significant coronary obstruction. Due to the unclear nature of his presentation, a bedside echocardiogram was rapidly performed and was indicative of right ventricular strain. Due to these findings, a pulmonary angiogram was performed that revealed massive pulmonary embolism. He successfully underwent catheter-directed thrombolysis and, after a prolonged hospital stay, was discharged home on lifelong anticoagulation. DISCUSSION: The impact of coronavirus disease-2019 (COVID-19) on the cardiovascular system has been prominent and multifaceted. COVID-19 can have wide-ranging effects on the cardiovascular system due to coagulopathy with resultant venous and arterial thrombo-embolism. Due to the critical condition of many patients affected by COVID-19, imaging for thrombo-embolic events is often delayed. With the use of bedside echocardiogram, observation of right ventricular strain may be critical in raising suspicion for pulmonary embolism, especially when atypical features are noted on electrocardiogram.

14.
J Am Heart Assoc ; 9(14): e016400, 2020 07 21.
Article in English | MEDLINE | ID: mdl-32654582

ABSTRACT

Background Little is known about the contemporary mortality experience among adults with congenital heart disease (CHD). The objectives of this study were to assess the age at death, presence of cardiovascular comorbidities, and most common causes of death among adults with CHD in a contemporary cohort within the United States. Methods and Results Patients with CHD who had a healthcare encounter between 2008 and 2013 at 1 of 5 comprehensive CHD centers in North Carolina were identified by International Classification of Diseases, Ninth Revision (ICD-9), code. Only patients who could be linked to a North Carolina death certificate between 2008 and 2016 and with age at death ≥20 years were included. Median age at death and underlying cause of death based on death certificate data were analyzed. The prevalence of acquired cardiovascular risk factors was determined from electronic medical record data. Among the 629 included patients, the median age at death was 64.2 years. Those with severe CHD (n=157, 25%), shunts (n=202, 32%), and valvular lesions (n=174, 28%) had a median age at death of 46.0, 65.0, and 73.3 years, respectively. Cardiovascular death was most common in adults with severe CHD (60%), with 40% of those deaths caused by CHD. Malignancy and ischemic heart disease were the most common causes of death in adults with nonsevere CHD. Hypertension and hyperlipidemia were common comorbidities among all CHD severity groups. Conclusions The most common underlying causes of death differed by lesion severity. Those with severe lesions most commonly died from underlying CHD, whereas those with nonsevere disease more commonly died from non-CHD causes.


Subject(s)
Cause of Death , Heart Defects, Congenital/mortality , Aged , Aged, 80 and over , Cohort Studies , Comorbidity , Female , Humans , Male , North Carolina/epidemiology , Risk Factors
15.
Cardiol Young ; 30(8): 1081-1085, 2020 Aug.
Article in English | MEDLINE | ID: mdl-32611471

ABSTRACT

BACKGROUND: Adults with CHD have reduced work participation rates compared to adults without CHD. We aimed to quantify employment rate among adult CHD patients in a population-based registry and to describe factors and barriers associated with work participation. METHODS: We retrospectively identified adults with employment information in the North Carolina Congenital Heart Defects Surveillance Network. Employment was defined as any paid work in a given year. Logistic regression was used to examine patients' employment status during each year. RESULTS: The registry included 1,208 adult CHD patients with a health care encounter between 2009 and 2013, of whom 1,078 had ≥1 year of data with known employment status. Overall, 401 patients (37%) were employed in their most recent registry year. On multivariable analysis, the odds of employment decreased with older age and were lower for Black as compared to White patients (odds ratio = 0.78; 95% confidence interval: 0.62, 0.98; p = 0.030), and single as compared to married patients (odds ratio = 0.50; 95% confidence interval: 0.39, 0.63; p < 0.001). CONCLUSION: In a registry where employment status was routinely captured, only 37% of adult CHD patients aged 18-64 years were employed, with older patients, Black patients, and single patients being less likely to be employed. Further work is needed to consider how enhancing cardiology follow-up for adults with CHD can integrate support for employment.


Subject(s)
Employment , Heart Defects, Congenital , Adult , Aged , Heart Defects, Congenital/epidemiology , Humans , North Carolina/epidemiology , Registries , Retrospective Studies
16.
Circ Cardiovasc Interv ; 10(8)2017 Aug.
Article in English | MEDLINE | ID: mdl-28801537

ABSTRACT

BACKGROUND: Prospective data on the medium-term safety and effectiveness of the AMPLATZER Septal Occluder in clinical practice are not available. The objective of this study was to prospectively evaluate the risk of hemodynamic compromise and obtain medium-term survival data on patients implanted with the AMPLATZER Septal Occluder for percutaneous closure of secundum atrial septal defects. METHODS AND RESULTS: Subjects were enrolled prospectively at 50 US sites and followed for 2 years. Between 2008 and 2012, atrial septal defect closure with the AMPLATZER Septal Occluder was attempted in 1000 patients (aged 0.3-83.6 years, mean 21±22 years). Procedural closure occurred in 97.9%, with 1-month and 2-year closure 98.5% and 97.9%, respectively. Hemodynamic compromise occurred in 6 subjects (0.65%), because of dysrhythmia in 2, device embolization in 1, and cardiac erosion in 3. The rate of cardiac erosion was 0.3% (average 83, range 12-171 days from implant). CONCLUSIONS: Closure of atrial septal defect with the AMPLATZER Septal Occluder is safe and effective. The rate of hemodynamic compromise and cardiac erosion is rare. The risk factors for cardiac erosion after device closure are not yet clear. CLINICAL TRIAL REGISTRATION: URL: http://www.clinicaltrials.gov. Unique identifier: NCT00650936.


Subject(s)
Heart Septal Defects, Atrial/therapy , Septal Occluder Device/adverse effects , Adolescent , Adult , Aged , Aged, 80 and over , Child , Child, Preschool , Female , Humans , Infant , Male , Middle Aged , Prospective Studies , Septal Occluder Device/statistics & numerical data , Young Adult
17.
Am Heart J ; 162(1): 125-30, 2011 Jul.
Article in English | MEDLINE | ID: mdl-21742098

ABSTRACT

BACKGROUND: The practice of coiling aortopulmonary collaterals (APCs) before Fontan completion is controversial, and published data are limited. We sought to compare outcomes in subjects with and without pre-Fontan coil embolization of APCs using the Pediatric Heart Network Fontan Cross-Sectional Study database which enrolled survivors of prior Fontan palliation. METHODS: We compared hospital length of stay after Fontan in 80 subjects who underwent APC coiling with 459 subjects who did not. Secondary outcomes included post-Fontan complications and assessment of health status and ventricular performance at cross-sectional evaluation (mean 8.6 ± 3.4 years after Fontan). RESULTS: Centers varied markedly in frequency of pre-Fontan APC coiling (range 0%-30% of subjects, P < .001). The coil group was older at Fontan (P = .004) and more likely to have single right ventricular morphology (P = .054) and pre-Fontan atrioventricular valve regurgitation (P = .03). The coil group underwent Fontan surgery more recently (P < .001), was more likely to have a prior superior cavopulmonary anastomosis (P < .001), and more likely to undergo extracardiac Fontan connection (P < .001) and surgical fenestration (P < .001). In multivariable analyses, APC coiling was not associated with length of stay (hazard ratio for remaining in-hospital 0.91, 95% CI 0.70-1.18, P = .48) or postoperative complications, except more post-Fontan catheter interventions (hazard ratio 1.74, 95% CI 1.04-2.91, P = .03), primarily additional APC coils. The groups had similar outcomes at cross-sectional evaluation. CONCLUSION: Management of APCs before Fontan shows marked practice variation. We did not find an association between pre-Fontan coiling of APCs and shorter postoperative hospital stay or with better late outcomes. Prospective studies of this practice are needed.


Subject(s)
Embolization, Therapeutic/methods , Fontan Procedure , Heart Defects, Congenital/surgery , Preoperative Care/methods , Pulmonary Artery , Thoracic Arteries , Ventricular Function, Left , Adolescent , Cardiac Catheterization , Child , Child, Preschool , Collateral Circulation , Cross-Sectional Studies , Echocardiography , Female , Follow-Up Studies , Heart Defects, Congenital/diagnosis , Heart Defects, Congenital/physiopathology , Heart Ventricles/abnormalities , Humans , Length of Stay , Male , Retrospective Studies , Treatment Outcome
18.
Cardiol Young ; 17 Suppl 2: 44-53, 2007 Sep.
Article in English | MEDLINE | ID: mdl-18039398

ABSTRACT

BACKGROUND: Children born with heterotaxy syndromes have poorer outcomes compared with children born with comparable cardiac lesions requiring similar surgical palliation. Heterotaxy has been reported as a separate risk factor for mortality and increased morbidity in a series of Fontan operations reported from single centres. Little is known, however, about the functional state of surviving patients with heterotaxy following a Fontan operation. METHODS: In the multicentric cross-sectional study carried out by the Pediatric Heart Network of 546 survivors of the Fontan procedure, the patients, aged from 6 to 18 years, underwent evaluation by echocardiography, exercise testing, electrocardiography, magnetic resonance imaging, and functional health status questionnaires compiled by the patients and their parents. Heterotaxy was identified in 42 patients (8%). Medical and patient characteristics were compared between those with heterotaxy and the remaining 504 patients who did not have heterotaxy. RESULTS: Patients with heterotaxy had their Fontan procedure performed at a later age, with a median of 3.9 years versus 2.8 years (p=0.001) and had volume-unloading surgery performed later, at a median age of 1.4 versus 0.9 years (p=0.008). These patients had significantly different ventricular and atrioventricular valvar morphology, as well as a higher incidence of systemic and pulmonary venous abnormalities. They had a higher incidence of prior surgery to the pulmonary veins, at 21 versus 0.4%. The type of Fontan procedure was different, but no difference was detected in length of stay in hospital, or the number of postoperative complications. Sinus rhythm was less common, at 44 versus 71%, (p=0.002), and history of atrial arrhythmias more common, at 19 versus 8%, (p=0.018) in those with heterotaxy. Echocardiography revealed a greater degree atrioventricular valvar regurgitation, lower indexed stroke volume, and greater Tei index. Exercise performance, levels of brain natriuretic peptide in the serum, and summary and domain scores from health status questionnaires, were not different from those not having heterotaxy. CONCLUSIONS: The study illustrates a profile of characteristics, medical history, functional health state, and markers of ventricular performance in patients with heterotaxy after the Fontan procedure. Despite obvious anatomic differences, and some differences in echocardiography and heart rhythm, there were no important differences in exercise performance or functional health state between these patients and other survivors of the Fontan procedure.


Subject(s)
Abnormalities, Multiple , Fontan Procedure/methods , Health Status , Heart Atria/abnormalities , Heart Defects, Congenital/physiopathology , Heart Ventricles/abnormalities , Situs Inversus/physiopathology , Adolescent , Child , Child, Preschool , Cross-Sectional Studies , Echocardiography , Electrocardiography , Exercise Test , Female , Follow-Up Studies , Heart Defects, Congenital/diagnosis , Heart Defects, Congenital/surgery , Heart Rate/physiology , Humans , Magnetic Resonance Imaging , Male , Situs Inversus/diagnosis , Situs Inversus/surgery , Surveys and Questionnaires , Syndrome , Treatment Outcome
19.
Pharmacotherapy ; 22(6): 766-70, 2002 Jun.
Article in English | MEDLINE | ID: mdl-12066967

ABSTRACT

Calcium channel blockers as a group are responsible for significant morbidity and mortality with toxic exposures. Isradipine, a cardioselective dihydropyridine calcium channel blocker, rarely has been implicated, with only two reports in the literature of significant toxic reactions, one in an adult and another in a child. To our knowledge, we describe the first case of life-threatening isradipine poisoning in a child and provide documentation of serum drug levels. On arrival at the hospital, a 5-year-old girl had abdominal distention and bradycardia that rapidly progressed to asystole. She received 73 minutes of cardiopulmonary resuscitation and transvenous cardiac pacing and survived with an intact neurologic recovery. Serum concentrations of isradipine were 30-100 times those found with therapeutic use.


Subject(s)
Calcium Channel Blockers/adverse effects , Isradipine/adverse effects , Bradycardia/chemically induced , Calcium Channel Blockers/blood , Calcium Channel Blockers/therapeutic use , Cardiac Pacing, Artificial , Cardiopulmonary Resuscitation , Child, Preschool , Electrocardiography , Female , Humans , Hypertension, Renal/complications , Hypertension, Renal/drug therapy , Hypertension, Renal/physiopathology , Isradipine/blood , Isradipine/therapeutic use , Liver Transplantation/physiology , Respiration, Artificial
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