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 >

Ranjit Philip¹, Jason Nathaniel Johnson^1,2, Ronak Naik¹, Dai Kimura^1,3, 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 >

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 >

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… 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… 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;… 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… 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 >

Shi-Min Yuan

European Cytokine Network, Vol.28, No.1, pp. 1-7, 2017, DOI:10.1684/ecn.2016.0386

Abstract Pulmonary artery hypertension is a syndrome that shows similar clinical and pathophysiological features characterized by elevated pulmonary arterial pressure and resistance. There have been a series of hypotheses trying to describe the development of pulmonary artery hypertension; however, none of them perfectly explains its pathogenesis. To highlight the pathogenesis, novel vasomotorial cytokines including hypoxia-inducible factor-1α endothelin-1, urotensin II, Krüppel-like factor 4, calcitonin gene-related peptide, angiopoietins and serotonin closely related to pulmonary artery hypertension are discussed. The development of the new agents relating to these cytokines may improve the relevant treatment strategies. 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… More >