Browsing by Subject "Heart Defects, Congenital"

Now showing 1 - 17 of 17
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    A Multifaceted Approach to Pulmonary Hypertension in Adults With Congenital Heart Disease.
    (Progress in cardiovascular diseases, 2018-09) Fathallah, Mouhammad; Krasuski, Richard A
    Advances in the management of congenital heart disease (CHD) in children have resulted in growing numbers of adults with CHD. Pulmonary arterial hypertension related to CHD (PAH-CHD) is a common complication, affecting up to 10% of patients; and can arise even after successful and complete defect repair, with severe and potentially fatal consequences. Careful work-up in these patients is essential, particularly hemodynamic assessment, and can help define the most appropriate therapeutic approach. Management can be challenging, but the therapeutic armamentarium is continually expanding and now includes surgical, transcatheter and medical options. Timely correction of defects along with early treatment with advanced medical therapies appears to improve quality of life and possible even improve survival. Interestingly most studies of PAH-CHD have focused on its most severely afflicted patients, those with Eisenmenger Syndrome, making it less certain how to manage PAH-CHD of milder degrees. This review summarizes our current understanding of PAH-CHD and emphasizes the need for close follow-up in specialized centers of care where close collaboration is common practice.
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    Adult Congenital Heart Disease Care Provision: the Need for a Jack/Jackie of All Trades.
    (Progress in cardiovascular diseases, 2018-09) Krasuski, Richard A
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    Aortic dissection and ruptures in adult congenital heart disease in Texas from 2009 to 2019.
    (European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery, 2022-01) Well, Andrew; Mizrahi, Michelle; Johnson, Gregory; Patt, Hanoch; Fraser, Charles D; Mery, Carlos M; Beckerman, Ziv

    Objectives

    Acute thoracic aortic dissection and rupture (TADR) has an incidence of 5-7 per 100 000-person years. Today, most children with congenital heart disease (CHD) survive to become adults with congenital heart disease (ACHD). This study evaluates TADR in patients with ACHD in a large, hospitalized patient population over 11 years to evaluate the incidence, risk factors and outcomes associated with TADR.

    Methods

    This was a retrospective review of the Texas Inpatient Discharge Data Set from 1 January 2009 to 31 December 2019. All non-trauma discharges of patients ≥18 years were included. ACHD discharges were identified by International Classification of Diseases, 9th edition (ICD-9)/10 diagnosis codes. TADR were identified using 2 definitions: TADR1 is an ICD-9/10 code for TADR, and TADR2 is TADR1 with an ICD-9/10 procedure code for aortic intervention. Descriptive, univariate and logistic regression statistics were used.

    Results

    A total of 22 154 664 eligible discharges were identified, of which 12 584 (0.06%) were TADR1 and a subgroup of 5699 (0.03%) were TADR2. CHD was more prevalent in TADR1 (0.2% vs 0.05%; P < 0.001) and TADR2 (0.3% vs 0.04%; P < 0.001). Adjusting for known TADR risk factors, CHD had an odds ratio of 1.69 (95% confidence interval: 1.09-2.63; P = 0.020) for TADR1 and an odds ratio of 1.69 (95% confidence interval: 0.99-2.88; P = 0.056) for TADR2. No in-hospital deaths were found in patients with CHD with TADR.

    Conclusions

    ACHD discharges had a higher frequency of TADR versus the general population (0.9-1.2 vs 0.3-0.6 per 1000 discharges). There is an indication that CHD confers an increased adjusted odds of TADR. As the ACHD population continues to grow in number as well as age, it will be important to continue to assess the risk of TADR from CHD and how traditional risk factors impact this risk.
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    Arrhythmias Requiring ECMO in Infants Without Structural Congenital Heart Disease.
    (Pediatric cardiology, 2022-04) Well, Andrew; Fenrich, Arnold; Shmorhun, Daniel; Stromberg, Daniel; Lavinghousez, Preston; Beckerman, Ziv; Fraser, Charles D; Mery, Carlos M
    Arrhythmias account for 55 per 100,000 patient evaluations in pediatric emergency departments. Most arrhythmias in children are amenable to medical management or cardioversion. Rarely, arrhythmias lead to significant hemodynamic instability requiring extracorporeal membrane oxygenation (ECMO) support. This study seeks to evaluate children under 1 year of age with a structurally normal heart requiring ECMO for an arrhythmia. This is a retrospective review of the Extracorporeal Life Support Organization Registry. All patients less than 1 year of age between 2009 and 2019 with a diagnosis of arrhythmia and without a diagnosis of structural heart malformation were included. Demographics, clinical characteristics, and outcomes were assessed with descriptive statistics and univariate and multivariable analyses. A total of 140 eligible patients were identified from the dataset. The most common arrhythmia was supraventricular tachycardia (SVT) in 70 (50%) patients. ECMO complications occurred in 106 (76.3%) patients and survival to discharge was achieved in 120 (85.7%) patients. In-hospital mortality was associated with neuromuscular blockade prior to ECMO [aOR 10.0 (95% CI 2.95-41.56), p < 0.001], neurologic ECMO complication [aOR 28.1 (95% CI 6.6-155.1), p < 0.001], and race with white race being protective [aOR 0.13, (95% CI 0.02-0.21), p = 0.002]. Similar survival and complication rates were found in subgroup analysis of SVT arrhythmias alone. Arrhythmias necessitating ECMO support in infants without structural congenital heart disease is a rare occurrence. However, survival to hospital discharge is favorable at greater than 85%. Given the favorable survival, earlier and more aggressive utilization of ECMO may result in improved outcomes.
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    Commentary: How to avoid early Fontan failure?
    (The Journal of thoracic and cardiovascular surgery, 2021-04) Mizrahi, Michelle; Beckerman, Ziv
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    Congenital heart disease and pulmonary hypertension.
    (Heart Fail Clin, 2012-07) Gupta, Vedant; Tonelli, Adriano R; Krasuski, Richard A
    Many patients with congenital heart disease and systemic-to-pulmonary shunts develop pulmonary arterial hypertension (PAH), particularly if the cardiac defect is left unrepaired. A persistent increase in pulmonary blood flow may lead to obstructive arteriopathy and increased pulmonary vascular resistance, a condition that can lead to reversal of shunt and cyanosis (Eisenmenger syndrome). Cardiac catheterization is crucial to confirm diagnosis and facilitate treatment. Bosentan is the only medication to date to be compared with placebo in a randomized controlled trial specifically targeting congenital heart disease-associated PAH. Lung transplantation with repair of the cardiac defect or combined heart-lung transplantation is reserved for recalcitrant cases.
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    Congenital Heart Disease Epidemiology in the United States: Blindly Feeling for the Charging Elephant.
    (Circulation, 2016-07-12) Krasuski, Richard A; Bashore, Thomas M
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    Coronary Disease and Modifying Cardiovascular Risk in Adult Congenital Heart Disease Patients: Should General Guidelines Apply?
    (Progress in cardiovascular diseases, 2018-09) Awerbach, Jordan D; Krasuski, Richard A; Camitta, Michael GW
    There are >1.4 million adult congenital heart disease (CHD; ACHD) patients living in the United States. Coronary artery disease (CAD) is at least as prevalent in ACHD patients as in the general population and has become a leading cause of their mortality. In the majority of cases, CAD in the ACHD population is driven by the presence of traditional cardiovascular disease (CVD) risk factors. 80% of ACHD patients have at least one CVD risk factor. Hypertension (HTN), obesity and physical inactivity are frequently seen in both pediatric and adult patients with CHD. Many ACHD patients demonstrate abnormal glucose metabolism and are at an increased risk for developing diabetes. Current guidelines for CVD risk assessment and prevention do not specifically mention patients with CHD but are likely applicable to most of these patients. Specific CHD populations have "high-risk" lesions that are associated with an increased risk of CVD complications and may warrant intensified screening and treatment. These include patients with a history of coarctation of the aorta or with prior coronary artery ostial manipulation (patients with a history of d-transposition of the great arteries or anomalous aortic origin of a coronary artery). The physiology of single ventricle patients is also poorly suited for the effects of superimposed CVD; these patients may benefit from intensified treatment of CVD risk factors, particularly HTN and obesity.
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    Development of the morpholino gene knockdown technique in Fundulus heteroclitus: a tool for studying molecular mechanisms in an established environmental model.
    (Aquat Toxicol, 2008-05-30) Matson, Cole W; Clark, Bryan W; Jenny, Matthew J; Fleming, Carrie R; Hahn, Mark E; Di Giulio, Richard T
    A significant challenge in environmental toxicology is that many genetic and genomic tools available in laboratory models are not developed for commonly used environmental models. The Atlantic killifish (Fundulus heteroclitus) is one of the most studied teleost environmental models, yet few genetic or genomic tools have been developed for use in this species. The advancement of genetic and evolutionary toxicology will require that many of the tools developed in laboratory models be transferred into species more applicable to environmental toxicology. Antisense morpholino oligonucleotide (MO) gene knockdown technology has been widely utilized to study development in zebrafish and has been proven to be a powerful tool in toxicological investigations through direct manipulation of molecular pathways. To expand the utility of killifish as an environmental model, MO gene knockdown technology was adapted for use in Fundulus. Morpholino microinjection methods were altered to overcome the significant differences between these two species. Morpholino efficacy and functional duration were evaluated with molecular and phenotypic methods. A cytochrome P450-1A (CYP1A) MO was used to confirm effectiveness of the methodology. For CYP1A MO-injected embryos, a 70% reduction in CYP1A activity, a 86% reduction in total CYP1A protein, a significant increase in beta-naphthoflavone-induced teratogenicity, and estimates of functional duration (50% reduction in activity 10 dpf, and 86% reduction in total protein 12 dpf) conclusively demonstrated that MO technologies can be used effectively in killifish and will likely be just as informative as they have been in zebrafish.
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    Developmental exposure to a complex PAH mixture causes persistent behavioral effects in naive Fundulus heteroclitus (killifish) but not in a population of PAH-adapted killifish.
    (Neurotoxicol Teratol, 2016-01) Brown, DR; Bailey, JM; Oliveri, AN; Levin, ED; Di Giulio, RT
    Acute exposures to some individual polycyclic aromatic hydrocarbons (PAHs) and complex PAH mixtures are known to cause cardiac malformations and edema in the developing fish embryo. However, the heart is not the only organ impacted by developmental PAH exposure. The developing brain is also affected, resulting in lasting behavioral dysfunction. While acute exposures to some PAHs are teratogenically lethal in fish, little is known about the later life consequences of early life, lower dose subteratogenic PAH exposures. We sought to determine and characterize the long-term behavioral consequences of subteratogenic developmental PAH mixture exposure in both naive killifish and PAH-adapted killifish using sediment pore water derived from the Atlantic Wood Industries Superfund Site. Killifish offspring were embryonically treated with two low-level PAH mixture dilutions of Elizabeth River sediment extract (ERSE) (TPAH 5.04 μg/L and 50.4 μg/L) at 24h post fertilization. Following exposure, killifish were raised to larval, juvenile, and adult life stages and subjected to a series of behavioral tests including: a locomotor activity test (4 days post-hatch), a sensorimotor response tap/habituation test (3 months post hatch), and a novel tank diving and exploration test (3months post hatch). Killifish were also monitored for survival at 1, 2, and 5 months over 5-month rearing period. Developmental PAH exposure caused short-term as well as persistent behavioral impairments in naive killifish. In contrast, the PAH-adapted killifish did not show behavioral alterations following PAH exposure. PAH mixture exposure caused increased mortality in reference killifish over time; yet, the PAH-adapted killifish, while demonstrating long-term rearing mortality, had no significant changes in mortality associated with ERSE exposure. This study demonstrated that early embryonic exposure to PAH-contaminated sediment pore water caused long-term locomotor and behavioral alterations in killifish, and that locomotor alterations could be observed in early larval stages. Additionally, our study highlights the resistance to behavioral alterations caused by low-level PAH mixture exposure in the adapted killifish population. Furthermore, this is the first longitudinal behavioral study to use killifish, an environmentally important estuarine teleost fish, and this testing framework can be used for future contaminant assessment.
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    Early experience with intravenous sotalol in children with and without congenital heart disease.
    (Heart rhythm, 2018-12) Valdés, Santiago O; Miyake, Christina Y; Niu, Mary C; de la Uz, Caridad M; Asaki, S Yukiko; Landstrom, Andrew P; Schneider, Andrew E; Rusin, Craig G; Patel, Raajen; Lam, Wilson W; Kim, Jeffrey J
    BACKGROUND:Arrhythmias are common in the pediatric population. In patients unable to take oral medications or in need of acute therapy, options of intravenous (IV) antiarrhythmic medications are limited. Recently IV sotalol has become readily available, but experience in children is limited. OBJECTIVE:The purpose of this study was to describe our initial experience with the use of IV sotalol in the pediatric population. METHODS:A retrospective study of all pediatric patients receiving IV sotalol was performed. Patient demographic characteristics, presence of congenital heart disease, arrhythmia type, efficacy of IV sotalol use, and adverse effects were evaluated. RESULTS:A total of 47 patients (26 (55%) male and 24 (51%) with congenital heart disease) received IV sotalol at a median age of 2.05 years (interquartile range 0.07-10.03 years) and a median weight of 12.8 kg (interquartile range 3.8-34.2 kg), and 13 (28%) received IV sotalol in the acute postoperative setting. Supraventricular arrhythmias occurred in 40 patients (85%) and ventricular tachycardia in 7 (15%). Among 24 patients receiving IV sotalol for an active arrhythmia, acute termination was achieved in 21 (88%). Twenty-three patients received IV sotalol as maintenance therapy for recurrent arrhythmias owing to inability to take oral antiarrhythmic medications; 19 (83%) were controlled with sotalol monotherapy. No patient required discontinuation of IV sotalol secondary to adverse effects, proarrhythmia, or QT prolongation. CONCLUSION:IV sotalol is an effective antiarrhythmic option for pediatric patients and may be an excellent agent for acute termination of active arrhythmias. It was well tolerated, with no patient requiring discontinuation secondary to adverse effects.
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    Essential role of beta-adrenergic receptor kinase 1 in cardiac development and function.
    (Proc Natl Acad Sci U S A, 1996-11-12) Jaber, M; Koch, WJ; Rockman, H; Smith, B; Bond, RA; Sulik, KK; Ross, J; Lefkowitz, RJ; Caron, MG; Giros, B
    The beta-adrenergic receptor kinase 1 (beta ARK1) is a member of the G protein-coupled receptor kinase (GRK) family that mediates the agonist-dependent phosphorylation and desensitization of G protein-coupled receptors. We have cloned and disrupted the beta ARK1 gene in mice by homologous recombination. No homozygote beta ARK1-/- embryos survive beyond gestational day 15.5. Prior to gestational day 15.5, beta ARK1-/- embryos display pronounced hypoplasia of the ventricular myocardium essentially identical to the "thin myocardium syndrome" observed upon gene inactivation of several transcription factors (RXR alpha, N-myc, TEF-1, WT-1). Lethality in beta ARK1-/- embryos is likely due to heart failure as they exhibit a > 70% decrease in cardiac ejection fraction determined by direct in utero intravital microscopy. These results along with the virtual absence of endogenous GRK activity in beta ARK1-/- embryos demonstrate that beta ARK1 appears to be the predominant GRK in early embryogenesis and that it plays a fundamental role in cardiac development.
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    Home is Where the Heart Is: Interstage Home Monitoring in Infants With Single-Ventricle Heart Disease.
    (Journal of cardiothoracic and vascular anesthesia, 2021-10) Andrews, Jon S; Machovec, Kelly A
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    Long Term Consequences of the Fontan Procedure and How to Manage Them.
    (Progress in cardiovascular diseases, 2018-09-17) Kay, W Aaron; Moe, Tabitha; Suter, Blair; Tennancour, Andrea; Chan, Alice; Krasuski, Richard A; Zaidi, Ali N
    In 1971, Fontan and Baudet described a surgical technique for successful palliation of patients with tricuspid atresia. Subsequently, this technique has been applied to treat most forms of functional single ventricles and has become the current standard of care for long-term palliation of all patients with single ventricle congenital heart disease. Since 1971, the Fontan procedure has undergone several variations. These patients require lifelong management including a thorough knowledge of their anatomic substrate, hemodynamic status, management of rhythm and ventricular function along with multi organ evaluation. As these patients enter middle age, there is increasing awareness regarding the long-term complications and mortality. This review highlights the long-term outcomes of the Fontan procedure and management of late sequelae.
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    Mechanical circulatory support for end-stage heart failure in repaired and palliated congenital heart disease.
    (Current cardiology reviews, 2011-05) Clark, Joseph B; Pauliks, Linda B; Myers, John L; Undar, Akif
    Approximately one in one hundred children is born with congenital heart disease. Most can be managed with corrective or palliative surgery but a small group will develop severe heart failure, leaving cardiac transplantation as the ultimate treatment option. Unfortunately, due to the inadequate number of available donor organs, only a small number of patients can benefit from this therapy, and mortality remains high for pediatric patients awaiting heart transplantation, especially compared to adults. The purpose of this review is to describe the potential role of mechanical circulatory support in this context and to review current experience. For patients with congenital heart disease, ventricular assist devices are most commonly used as a bridge to cardiac transplantation, an application which has been shown to have several important advantages over medical therapy alone or support with extracorporeal membrane oxygenation, including improved survival to transplant, less exposure to blood products with less immune sensitization, and improved organ function. While these devices may improve wait list mortality, the chronic shortage of donor organs for children is likely to remain a problem into the foreseeable future. Therefore, there is great interest in the development of mechanical ventricular assist devices as potential destination therapy for congenital heart disease patients with end-stage heart failure. This review first discusses the experience with the currently available ventricular assist devices in children with congenital heart disease, and then follows to discuss what devices are under development and may reach the bedside soon.
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    Transcatheter Valve Replacement for Right-sided Valve Disease in Congenital Heart Patients.
    (Progress in cardiovascular diseases, 2018-09-17) Gales, Jordan; Krasuski, Richard A; Fleming, Gregory A
    Pulmonary and/or tricuspid valve dysfunction is common among individuals with congenital heart disease, and surgical intervention often carries prohibitive risks. Transcatheter valve replacement (TVR) of the right-sided cardiac valves has become a viable treatment option over the past two decades, while continued technological development aims to broaden its applicability to an even larger portion of those with repaired congenital heart disease. To date, two transcatheter valves have been approved for use in patients with dysfunctional right ventricular to pulmonary artery conduits as well as those with failing pulmonic bioprosthetic valves, and are also used off-label in the "native" RVOT and within surgically repaired/replaced but failing tricuspid valves. TVR has demonstrated comparable safety and short-term outcomes to that of surgical valve replacement. This article aims to review current available devices, focusing on their safety, efficacy and on and off label usage, while briefly describing some of the emerging devices and novel procedural techniques that will likely lead to significant expansion of transcatheter treatment of right sided valve disease in the future.