Liesbeth van Dessel¹, Béatrice Santens², Els Troost², Pieter De Meester^2,3, Leen Roggen², Filip Rega^3,4, Bart Meyns^3,4, Bjorn Cools⁵, Marc Gewillig⁵, Philip Moons^6,7,8, Lukas Meier⁹, Werner Budts^2,3, Alexander Van De Bruaene^2,3,*

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

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 128 (94%) at… More >

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 risk assessment. This… More >

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… More >

Shankar Baskar¹, Philippa Horne², Samantha Fitzsimmons³, Philip R. Khoury¹, Joseph Vettukattill⁴, Koichiro Niwa⁵, Teiji Agaki⁶, Mark Spence⁷, Hisanori Sakazaki⁸, Gruschen Veldtman¹

Congenital Heart Disease, Vol.12, No.4, pp. 512-519, 2017, DOI:10.1111/chd.12481

Abstract Background: Patients with Eisenmenger syndrome have a shorter lifespan than the general population. A significant proportion develop arrhythmia and some, sudden death.
Objective: The aims of this study were to characterize the frequency, type and effects of arrhythmias in adult patients with Eisenmenger’s syndrome and to identify risk factors for arrhythmias.
Methods: This retrospective study included patients aged ≥18 years of age with Eisenmenger’s syndrome from three institutions. Arrhythmias were noted from electrocardiograms and Holter study reviews.
Results: A total of 167 patients, 96 females, 63 males (gender not available in 9 patients) were included in this study. The mean… More >

Chih-Hsin Hsu^1,2, Jun-Neng Roan^1,3, Jieh-Neng Wang⁴, Chien-Chi Huang⁵, Chao-Jung Shih³, Jyh-Hong Chen^2,6, Jing-Ming Wu⁴, Chen-Fuh Lam^7,8

Congenital Heart Disease, Vol.12, No.4, pp. 533-539, 2017, DOI:10.1111/chd.12479

Abstract Objectives: Atrial septal defects may result in pulmonary hypertension and right heart remodeling. We analyzed improvements in patients with flow-induced pulmonary hypertension and the activation of endothelial progenitor cells after flow reduction.
Design: This prospective cohort study included 37 patients who were admitted for an occluder implantation. Blood samples were collected before and after the procedure. We determined the number of endothelial progenitor cells in outgrowth colonies and serum Hsp27 concentrations. Daily performance and cardiothoracic ratio were reevaluated later.
Results: Closure of the defect significantly reduced the pulmonary pressure and B-type natriuretic peptide levels. The cardiothoracic ratio and daily performance… More >

Sarah A. Twichell¹, Corinna J. Rea¹, Patrice Melvin², Andrew J. Capraro¹, Joshua C. Mandel¹, Michael A. Ferguson¹, Daniel J. Nigrin¹, Kenneth D. Mandl¹, Dionne Graham², Justin P. Zachariah³

Congenital Heart Disease, Vol.12, No.4, pp. 484-490, 2017, DOI:10.1111/chd.12469

Abstract Background: Recognition of high blood pressure (BP) in children is poor, partly due to the need to compute age-sex-height referenced percentiles. This study examined the change in abnormal BP recognition before versus after the introduction of an electronic health record (EHR) app designed to calculate BP percentiles with a training lecture.
Methods and results: Clinical data were extracted on all ambulatory, non-urgent encounters for children 3–18 years old seen in primary care, endocrinology, cardiology, or nephrology clinics at an urban, academic hospital in the year before and the year after app introduction. Outpatients with at least 1 BP above the… More >

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 for each patient… More >

Timur Mese¹, Baris Guven², Murat Muhtar Yilmazer¹, Cem Karadeniz³, Rahmi Ozdemir¹, Onder Doksoz¹

Congenital Heart Disease, Vol.13, No.4, pp. 506-511, 2018, DOI:10.1111/chd.12616

Abstract Background: Mean platelet volume (MPV), platecrit, and platelet distribution width (PDW) are markers of platelet activation. Previous studies have found that platelet activation occurs in patients with pulmonary arterial hypertension. Platelet indices including MPV, PDW, and platecrit have not been studied in children with congenital heart disease associated pulmonary arterial hypertension (APAH-CHD) who survived and those who died.
Objective: The objective of this study to investigate the value of platelet indices with clinical and hemodynamic indicators predicting the disease severity and survival in children with APAH-CHD.
Methods: This was a nested case-control study. MPV, platecrit, and PDW levels measured in… More >

Jonathan N. Menachem¹, Edo Y. Birati², Payman Zamani², Anjali T. Owens², Pavan Atluri³, Christian A. Bermudez³, David Drajpuch², Stephanie Fuller⁴, Yuli Y. Kim², Christopher E. Mascio⁴, Vikram Palanivel², J. Eduardo Rame², Joyce Wald², Michael A. Acker³, Jeremy A. Mazurek²

Congenital Heart Disease, Vol.13, No.4, pp. 492-498, 2018, DOI:10.1111/chd.12606

Abstract Background: Heart failure (HF) is the leading cause of death in adults with congenital heart disease (ACHD). Identification of disease progression and timing of referral for advanced therapies is often delayed. However, increased awareness and understanding of ACHD and improvements in the approach to treatment have led to improved outcomes. Pulmonary hypertension (PH) is a common barrier to HT. In ACHD, the approach to PH and HT is quite complicated, given the anatomic heterogeneity and lower prevalence and experience. However, in some cases, PH is a result of elevated systemic filling pressures and low output.
Methods: We describe the approach… More >

Shahnawaz M. Amdani¹, Muhammad Umair M. Mian², Ron L. Thomas³, Robert D. Ross⁴

Congenital Heart Disease, Vol.13, No.4, pp. 499-505, 2018, DOI:10.1111/chd.12601

Abstract Aim: To evaluate predictors of morbidity and mortality in pediatric patients with pulmonary hypertension (PH), laboratory and echocardiographic measures of PH were analyzed.
Methods: A retrospective review of all infants and children< 2 years of age with PH from January 2011 to August 2016 was conducted. Correlations were determined using Spearman’s rank correlation coefficients. Differences in characteristics between survivors and nonsurvivors were analyzed and Kaplan-Meier survival curves were generated.
Results: Of 56 patients, the majority were extremely premature; of African American ethnicity; and had bronchopulmonary dysplasia. Patients who died were more likely to have underlying congenital heart disease; have a… More >