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  • Open Access

    ARTICLE

    Pulmonary Hemodynamics and Outcome in a Large Cohort of Patients with Sinus Venosus Septal Defect

    Liesbeth van Dessel1, Béatrice Santens2, Els Troost2, Pieter De Meester2,3, Leen Roggen2, Filip Rega3,4, Bart Meyns3,4, Bjorn Cools5, Marc Gewillig5, Philip Moons6,7,8, Lukas Meier9, Werner Budts2,3, Alexander Van De Bruaene2,3,*

    Congenital Heart Disease, Vol.15, No.2, pp. 69-78, 2020, DOI:10.32604/CHD.2020.011512 - 23 June 2020

    Abstract Background: Left-to-right shunt in sinus venosus septal defect (SVSD) may affect resistive (pulmonary vascular resistance–PVR) and elastic (pulmonary artery compliance-PAC) pulmonary artery properties. This study aimed at evaluating (1) impact of age, (2) pulmonary hemodynamics, and (3) outcome in a large cohort of SVSD patients. Methods: This study included 136 patients with SVSD (median age at diagnosis 14 (IQR 5–48) years, 47% male) of which 87 underwent catheterization. Pressures were measured and cardiac output was evaluated using the Fick principle at diagnosis. PVR, PAC and their product (RC time) were calculated. Results: Surgical repair was performed in… More >

  • Open Access

    ARTICLE

    Cardiovascular risk factors in adults with coarctation of the aorta

    Maria Fedchenko, Zacharias Mandalenakis, Helena Dellborg, Görel Hultsberg‐Olsson, Anna Björk, Peter Eriksson, Mikael Dellborg

    Congenital Heart Disease, Vol.14, No.4, pp. 549-558, 2019, DOI:10.1111/chd.12785

    Abstract Background: The aging patient with adult congenital heart disease (ACHD) faces the risk of developing atherosclerotic disease. Patients with coarctation of the aorta (CoA) are especially vulnerable because of an inherent high risk of developing hy‐ pertension. However, data on the prevalence of other cardiovascular risk factors are scarce. Therefore, this study aimed to describe the prevalence of traditional cardio‐ vascular risk factors (diabetes, hypertension, hyperlipidemia, smoking, obesity, and sedentary lifestyle) in adult patients with CoA.
    Methods: Patients with CoA who were registered at the ACHD clinic in Gothenburg were asked to participate in a comprehensive cardiovascular… More >

  • Open Access

    ARTICLE

    The acute effects of 30 mg vs 60 mg of intravenous Fasudil on patients with congenital heart defects and severe pulmonary arterial hypertension

    Hongyun Ruan1, Yigang Zhang2, Ru Liu3, Xiangjun Yang1

    Congenital Heart Disease, Vol.14, No.4, pp. 645-650, 2019, DOI:10.1111/chd.12764

    Abstract Objective: The optimal dose of Fasudil is still controversial in congenital heart disease accompanied with severe pulmonary hypertension (CHD‐PAH). This study aimed to compare acute hemodynamic changes after different doses of Fasudil in 60 consecu‐ tive adult patients with CHD‐PAH.
    Design: Prospective randomized controlled trial.
    Setting: Tertiary cardiology center.
    Patients: Adult patients with CHD‐PAH.
    Interventions: Patients were randomized to Fasudil 30 or 60 mg.
    Outcome Measures: The hemodynamic parameters were measured at baseline and after 30 minutes of Fasudil through right cardiac catheterization. Blood gas results were obtained from the pulmonary artery, right ventricle, right atrium, superior and inferior vena cava,… More >

  • Open Access

    ARTICLE

    Transvenous implantation of the Occlutech Atrial Flow Regulator: Preliminary results from swine models

    Daniel McLennan, Dunbar Ivy, Gareth J. Morgan

    Congenital Heart Disease, Vol.14, No.5, pp. 819-831, 2019, DOI:10.1111/chd.12816

    Abstract Aims: To evaluate in domestic pigs the histopathological processes after implanting the Occlutech Atrial Flow Regulator (AFR).
    Methods and Results: Eleven pigs were chosen and had successful implantation of the AFR. Five pigs were sacrificed at 28 days, and 5 pigs at 90 days. One pig was sacrificed at day 3 after device embolization. Each pig had echocardiography performed at 3 weeks to check patency. Post mortem evaluation included Gross evaluation, radiographic evaluation, histology, and electron microscopy. Nine of the 10 devices implanted remained patent at time of autopsy with no thrombus and minimal inflammation. One device More >

  • Open Access

    ARTICLE

    Exploration of the Notch3‐HES5 signal pathway in monocrotaline‐induced pulmonary hypertension using rat model

    Xing Chen, Wu Zhou, Qinghua Hu, Lingjin Huang

    Congenital Heart Disease, Vol.14, No.3, pp. 396-402, 2019, DOI:10.1111/chd.12733

    Abstract Objective: This study explores the role of the Notch3‐HES5 signal pathway in mono‐ crotaline‐induced pulmonary hypertension (PH) using rat models.
    Method: Sprague Dawley rats (n = 45) were randomly grouped into normal group, control group, and model group. Rats in the model group were used to establish the PH rat model. Four weeks after model establishment, right catheterization was used to measure the mean pulmonary arterial pressure (mPAP) and right ventricular sys‐ tolic pressure (RVSP) to analyze hemodynamic changes. The severity of PH was as‐ sessed by the right ventricular hypertrophy index (RVHI) and percentage of media… More >

  • Open Access

    ARTICLE

    Phosphodiesterase type 5 inhibitors improve microvascular dysfunction markers in pulmonary arterial hypertension associated with congenital heart disease

    Mariana M. Clavé1, Nair Y. Maeda2, Ana M. Thomaz1, Sergio P. Bydlowski3, Antonio A. Lopes1

    Congenital Heart Disease, Vol.14, No.2, pp. 246-255, 2019, DOI:10.1111/chd.12688

    Abstract Background: Ideally, vasodilator therapies for pulmonary arterial hypertension (PAH) should have a favorable impact on markers of vascular dysfunction, in addition to their known effects on hemodynamics, cardiac function, and patient’s physical capacity.
    Methods: We analyzed circulating (plasma) markers of endothelial and platelet activation/dys‐ function (enzyme‐linked immunoassays) in the specific setting of advanced PAH associated with congenital heart disease, during the course of sildenafil and tadalafil therapies. Thirty‐one patients were enrolled (age 10‐54 years), most of them with chronic hypoxemia and elevated hematocrit. Drugs were administered orally for 6 months (sildenafil [n = 16], 20 mg t.i.d.;… More >

  • Open Access

    ARTICLE

    Effect of Patent Ductus Arteriosus on Pulmonary Vascular Disease

    Ranjit Philip1, Jason Nathaniel Johnson1,2, Ronak Naik1, Dai Kimura1,3, Umar Boston1, Sandeep Chilakala1, Benjamin Hendrickson1, Benjamin Rush Waller1, Shyam Sathanandam1

    Congenital Heart Disease, Vol.14, No.1, pp. 37-41, 2019, DOI:10.1111/chd.12702

    Abstract The hemodynamic effects of a patent ductus arteriosus (PDA) are well known including systemic hypoperfusion and volume overload on the left ventricle. This article aims to provide a review of the long-standing effect of a hemodynamically significant PDA on the pulmonary vasculature and the role of cardiac catheterization in preterm infants with a PDA and pulmonary hypertension. More >

  • Open Access

    ABSTRACT

    Dependency of Nuclear Deformation of Smooth Muscle Cells on Tissue Stretch Direction May Explain Anisotropic Response of Aortic Wall to Hypertension

    Takeo Matsumoto1,*, Chizuru Hirooka1, Yong Fan1, Junfeng Wang1, Naoki Mori1, Eijiro Maeda1

    Molecular & Cellular Biomechanics, Vol.16, Suppl.2, pp. 81-81, 2019, DOI:10.32604/mcb.2019.07102

    Abstract Aortic wall thickens in response to hypertension. Many studies reported that the wall thickening occurs to maintain the wall stress in the circumferential direction at a constant level. In case of the longitudinal direction, however, there are few studies suggesting the constancy of the stress. Such anisotropic response may be attributable to the circumferential alignment of the smooth muscle cells (SMCs) in the wall [1]. However, to the authors’ knowledge, there are no study discussing the underlying mechanism of the anisotropic response. It has been reported that mechanical deformation of the nuclei causes transcription upregulation… More >

  • Open Access

    ABSTRACT

    Reliability and Variability of Hepatic Venous Pressure Gradient as a Surrogate of Portal Pressure Gradient: Insights from a Computational Model-Based Study

    Fuyou Liang1,*, Tianqi Wang1

    Molecular & Cellular Biomechanics, Vol.16, Suppl.1, pp. 25-26, 2019, DOI:10.32604/mcb.2019.05710

    Abstract Hepatic venous pressure gradient (HVPG) measurement has been increasingly accepted as a useful means for indirectly measuring portal venous pressure in patients with portal hypertension (PHT) caused by chronic liver diseases. Despite the existence of numerous studies addressing the clinical utility of HVPG measurement, it is as yet unclear how the accuracy of measured HVPG as a surrogate of portal pressure gradient (PPG) is influenced by the pathological status of the hepatic circulation that not only changes with the progression of liver disease but also differs considerably among patients. In addition, it remains unclear whether… More >

  • Open Access

    ARTICLE

    Hemodynamic effects of ketamine in children with congenital heart disease and/or pulmonary hypertension

    Rohit S. Loomba1, Seth B. Gray2, Saul Flores3

    Congenital Heart Disease, Vol.13, No.5, pp. 646-654, 2018, DOI:10.1111/chd.12662

    Abstract Introduction: Ketamine is a drug often used for procedural sedation or as adjunct agent for general sedation in children with congenital heart disease. In the clinical realm, there is often confusion regarding the effects of ketamine on hemodynamics, particularly pulmonary vascular resistance and systemic vascular resistance. We per‐ formed a meta‐analysis of studies investigating the effects of ketamine on hemodynamics.
    Methods: A systematic review was conducted to identify studies characterizing the hemodynamic effects of ketamine in children with congenital heart disease. Studies were assessed for quality and those of satisfactory quality with pre‐ and postketa‐ mine hemodynamics… More >

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