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 >

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 >

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 >

Amir-Reza Hosseinpour¹, Marie-Hélène Perez², David Longchamp², Jacques Cotting², Nicole Sekarski³, Michel Hurni¹, René Prêtre¹, Stefano Di Bernardo³

Congenital Heart Disease, Vol.13, No.2, pp. 210-216, 2018, DOI:10.1111/chd.12545

Abstract Objective: Congenital cardiac malformations with high pulmonary blood flow and pressure due to left-to-right shunts are usually repaired in early infancy for both the benefits of early relief of heart failure and the fear that the concomitant pulmonary hypertension may become irreversible unless these defects are corrected at an early age. Age, however, has been a poor predictor of irreversibility of pulmonary hypertension in our experience, which is presented here.
Design: A retrospective observational study. We defined “late” as age ≥2 years. We examined clinical, echocardiographic, and hemodynamic data from all patients aged ≥2 years with such malformations referred to… More >

Jason F. Goldberg¹, Craig L. Jensen², Rajesh Krishnamurthy³, Nidhy P. Varghese⁴, Henri Justino¹

Congenital Heart Disease, Vol.13, No.1, pp. 124-130, 2018, DOI:10.1111/chd.12537

Abstract Objective: We describe the long-term follow-up of a child with recurrent hemoptysis due to severe pulmonary vein stenosis decompressing via collaterals to esophageal varices.
Design: Case report
Setting: Tertiary children’s hospital
Patient: Single child through ages 2- to 11-year old
Interventions: The child underwent cutting balloon angioplasty, bare metal stenting, and implantation of a PTFE-covered stent, all of which failed rapidly. Only after placement of a paclitaxel drug eluting stent did he have prolonged relief from hemoptysis and long-term patency of the treated vein. The stents were serially dilated to keep up with somatic growth of the child, eventually culminating… More >

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 placed in the… More >