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Pulmonary arterial hypertension AND congenital heart disease AND pediatric

Lijiang Ma, Wendy K Chung
Group 1 pulmonary hypertension or pulmonary arterial hypertension (PAH) is a rare disease characterized by proliferation and occlusion of small pulmonary arterioles, leading to progressive elevation of pulmonary artery pressure and pulmonary vascular resistance, and right ventricular failure. Historically it has been associated with a high mortality rate, although over the last decade, treatment has improved survival. PAH includes idiopathic PAH (IPAH), heritable PAH (HPAH), and PAH associated with certain medical conditions...
October 22, 2016: Journal of Pathology
Willemijn M H Zijlstra, Johannes M Douwes, Mark-Jan Ploegstra, Usha Krishnan, Marcus T R Roofthooft, Hans L Hillege, D Dunbar Ivy, Erika B Rosenzweig, Rolf M F Berger
Congenital heart disease (CHD) is a frequent cause of pediatric pulmonary arterial hypertension (PAH), with diverse etiology and outcome. We aimed to describe phenotypic heterogeneity in pediatric PAH associated with CHD (PAH-CHD), assess the applicability of the Nice CHD classification, and explore whether this classification accurately reflects patient/disease characteristics and survival. All children with CHD from a contemporary cohort of consecutive pediatric PAH patients followed in three major referral centers (Denver, New York, the Netherlands) were characterized and classified on the basis of the latest proposed clinical classification for PAH-CHD (World Symposium on Pulmonary Hypertension, Nice, 2013)...
September 2016: Pulmonary Circulation
Hala Mahmoud Koura, Neamat M Abdalla, Mona Hamed Ibrahim, Maha M A Abo Hashish, Sherif Mohamed Zaki
BACKGROUND: B-type natriuretic peptide (BNP) levels are elevated in children with congenital heart disease involving a left-to-right shunt (LRS) and are also raised in dilated cardiomyopathy (DCM). As far as we know, there are few reports in the literature comparing the change of the NT-proBNP in LRS and DCM especially in the pediatric age group. OBJECTIVES: The aim of the study was to compare the changes of the NT-proBNP in pediatric patients with LRS and DCM. Correlation between the levels of NT-proBNP and the echocardiographic parameters in both groups was determined...
June 2016: Iranian Journal of Pediatrics
Steven H Abman
PURPOSE OF REVIEW: Pediatric pulmonary vascular disease contributes to morbidities and death in diverse clinical settings, ranging from idiopathic or heritable forms of pediatric arterial hypertension, congenital heart disease, developmental lung disorders, chronic lung disease, left heart disease, sickle cell disease, oncologic disease, and systemic disorders. Despite its impact on the clinical courses in so many diseases, information is limited on how to best approach the diagnosis and evaluation of pediatric pulmonary hypertension...
October 2016: Current Opinion in Pediatrics
I-Chen Chen, Zen-Kong Dai
UNLABELLED: Compared with adult patients with pulmonary hypertension (PH), pulmonary vascular disease is characterized by complex heterogeneity in pediatric patients. The Nice PH classification does not completely characterize or individualize any subgroup of pediatric PH. This is in contrast to the Panama classification, in which prenatal and fetal origins of many pulmonary vascular diseases in neonates and children, perinatal pulmonary vascular maladaptation, prenatal and postnatal pulmonary vascular mal-development, and pulmonary vascular hypoplasia are included...
November 2015: Acta Cardiol Sin
Johannes M Douwes, Tilman Humpl, Damien Bonnet, Maurice Beghetti, D Dunbar Ivy, Rolf M F Berger
BACKGROUND: In pulmonary arterial hypertension (PAH), acute vasodilator response testing (AVT) is considered important to identify adult patients with favorable prognosis using calcium-channel blocker (CCB) therapy. However, in pediatric PAH, criteria used to identify acute responders and CCB use are insufficiently studied. OBJECTIVES: This study sought to describe current clinical practice of AVT and subsequent treatment decisions in pediatric PAH. METHODS: From January 2008 to May 2013, 529 consecutive children with confirmed pulmonary hypertension were enrolled in an international registry...
March 22, 2016: Journal of the American College of Cardiology
Mohammed H Alghamdi, Muna I Ismail, Talat Mesud Yelbuz, Fahad Alhabshan
AIM: To determine if a transthoracic echocardiography (TTE) can be used as the sole diagnostic imaging modality to evaluate children with congenital heart disease (CHD) undergoing cardiac surgery. METHODS: A retrospective study was carried out at the King Abdulaziz Cardiac Center. We reviewed all pediatric patients who underwent cardiac surgery during the period January 2011 to December 2011. RESULTS: Three hundred ninety-two pediatric patients with CHD fulfilled the inclusion criteria...
May 2016: Congenital Heart Disease
Jennifer E Pogoriler, Thomas J Kulik, Alicia M Casey, Christopher W Baird, Mary P Mullen, Kathy J Jenkins, Sara O Vargas
Pulmonary vein stenosis is a rare progressive narrowing of the extrapulmonary pulmonary veins, presenting predominantly in infancy and virtually always lethal. It typically arises following repair of congenital heart disease, particularly anomalous pulmonary venous return. Histologic characterization of pediatric pulmonary vein stenosis, not previously well described, may provide insight into the disease pathobiology. We retrieved archival lung specimens (biopsy, explant, or autopsy) from patients with pediatric pulmonary vein stenosis...
May 2016: Pediatric and Developmental Pathology
Sang-Yun Lee, Soo-Jin Kim, Jae Sung Son, Seong-Ho Kim, Chang-Ha Lee
BACKGROUND AND OBJECTIVES: Despite remarkable advances in pediatric cardiology, pulmonary arterial hypertension associated with congenital heart disease remains a major problem. In the past decade new vasodilators have been introduced and appear to be effective in reducing pulmonary vascular resistance (PVR). SUBJECTS AND METHODS: From 2000 to 2011, we retrospectively reviewed the records of 22 patients who had congenital septal defects and borderline pulmonary vascular disease (PVD)...
September 2015: Korean Circulation Journal
Gloria Pelizzo, Valeria Calcaterra, Savina Mannarino, Lorenzo Paolo Moramarco, Giovanni Leati, Pietro Quaretti
INTRODUCTION: Aortopulmonary collateral arteries are an uncommon variant of alternative blood supply in cases of complex congenital heart disease. Although surgery may still be the classic approach for this condition, mini-invasive endovascular occlusion has been recently attempted as an alternative less traumatic procedure. Children born to women with epilepsy are at increased risk of congenital malformations. CASE PRESENTATION: A cardiovascular malformation in a 6-year-old white boy with prenatal exposure to carbamazepine is described...
2015: Journal of Medical Case Reports
Kai Ma, Qian Zhao, Weidan Chen, Hao Zhang, Shoujun Li, Xiangbin Pan, Qiuming Chen
OBJECTIVE: Although several microRNAs were reported to play essential roles in pulmonary artery hypertension due to hypoxia or monocrotaline, their potential role in pulmonary arterial hypertension secondary to congenital heart disease is largely unknown. This study aimed to indentify microRNAs implicated in pulmonary arterial hypertension secondary to congenital heart disease in children. METHODS: Using microRNAs microarray, we profiled the microRNAs in the lung specimen from 12 congenital heart disease patients, (6 with pulmonary arterial hypertension and the others without)...
December 2015: Pediatric Pulmonology
D Dunbar Ivy, Steven H Abman, Robyn J Barst, Rolf M F Berger, Damien Bonnet, Thomas R Fleming, Sheila G Haworth, J Usha Raj, Erika B Rosenzweig, Ingram Schulze Neick, Robin H Steinhorn, Maurice Beghetti
Pulmonary hypertension (PH) is a rare disease in newborns, infants, and children that is associated with significant morbidity and mortality. In the majority of pediatric patients, PH is idiopathic or associated with congenital heart disease and rarely is associated with other conditions such as connective tissue or thromboembolic disease. Incidence data from the Netherlands has revealed an annual incidence and point prevalence of 0.7 and 4.4 for idiopathic pulmonary arterial hypertension and 2.2 and 15.6 for pulmonary arterial hypertension, respectively, associated with congenital heart disease (CHD) cases per million children...
October 2014: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
Frédéric Lador, Nicole Sekarski, Maurice Beghetti
INTRODUCTION: Pulmonary hypertension is a hemodynamic condition occurring rarely in pediatrics. Nevertheless, it is associated with significant morbidity and mortality. When characterized by progressive pulmonary vascular structural changes, the disease is called pulmonary arterial hypertension (PAH). It results in increased pulmonary vascular resistance and eventual right ventricular failure. In the vast majority of cases, pediatric PAH is idiopathic or associated with congenital heart disease, and, contrary to adult PAH, is rarely associated with connective tissue, portal hypertension, HIV infection or thromboembolic disease...
April 2015: Expert Opinion on Pharmacotherapy
Li Jiang, Sheng Ding, Hongtao Yan, Yunming Li, Liping Zhang, Xue Chen, Xiumei Yin, Shunbi Liu, Xiuying Tang, Jinbao Zhang
We hypothesized that postoperative sedation with dexmedetomidine/fentanyl would be effective in infants and neonates with congenital heart disease and pulmonary arterial hypertension (PAH). Children who were <36 months of age, had congenital heart disease with PAH, and had been treated at our hospital between October 2011 and April 2013 (n = 187) were included in this retrospective study. Either dexmedetomidine/fentanyl (Group Dex) or midazolam/fentanyl (Group Mid) was used for postoperative sedation. The main outcome variables included delirium scores, supplemental sedative/analgesic drugs, ventilator use, and sedation time...
June 2015: Pediatric Cardiology
Steven H Abman, Christopher Baker, Jason Gien, Peter Mourani, Csaba Galambos
Pulmonary arterial hypertension (PAH) contributes to poor outcomes in diverse diseases in newborns, infants, and children. Many aspects of pediatric PAH parallel the pathophysiology and disease courses observed in adult patients; however, critical maturational differences exist that contribute to distinct outcomes and therapeutic responses in children. In comparison with adult PAH, disruption of lung vascular growth and development, or angiogenesis, plays an especially prominent role in the pathobiology of pediatric PAH...
September 2014: Pulmonary Circulation
D B Frank, B D Hanna
Pulmonary arterial hypertension (PAH) is an uncommon but serious disease characterized by severe pulmonary vascular disease and significant morbidity and mortality. PAH associated with congenital heart disease (APAH-CHD) is one etiology of PAH that has innate characteristics delineating it from other forms of PAH. The patient with APAH-CHD presents with unique challenges consisting of not only pulmonary vascular disease but also the complexity of the cardiac lesion. Eisenmenger syndrome (ES) represents the severe end of the spectrum for disease in APAH-CHD...
April 2015: Minerva Pediatrica
Mark-Jan Ploegstra, Marcus T R Roofthooft, Johannes M Douwes, Beatrijs Bartelds, Nynke J Elzenga, Dick van de Weerd, Hans L Hillege, Rolf M F Berger
BACKGROUND: The value of echocardiography in assessing disease severity and predicting outcome in pediatric pulmonary arterial hypertension (PAH) is insufficiently defined. The aim of this study was to describe correlations between echocardiography and disease severity and outcome in pediatric PAH. METHODS AND RESULTS: Forty-three consecutive children (median age, 8.0 years; range, 0.4-21.5) with idiopathic/hereditary PAH (n=25) or PAH associated with congenital heart disease (n=18) were enrolled in a prospective single-center observational study...
January 2015: Circulation. Cardiovascular Imaging
Charlien Gabriels, Patrizio Lancellotti, Alexander Van De Bruaene, Damien Voilliot, Pieter De Meester, Roselien Buys, Marion Delcroix, Werner Budts
AIMS: Patients at risk of pulmonary arterial hypertension (PAH) may present with abnormal dynamic pulmonary vascular resistance (PVR) during exercise. However, its clinical significance remains unclear. The present study aimed at analysing the meaning of dynamic PVR in two populations at risk of PAH: secundum atrial septal defect (ASD) and systemic sclerosis (SSc). METHODS AND RESULTS: Adult patients with corrected ASD were consecutively selected from the database of Pediatric and Congenital Heart Disease of the University Hospitals Leuven...
May 2015: European Heart Journal Cardiovascular Imaging
Vicki Mahan, Randy Stevens, Cesar Mesia, Achintya Moulick
SESSION TITLE: Pediatric Global Case ReportsSESSION TYPE: Global Case ReportPRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PMINTRODUCTION: Small pulmonary arteries in patients with congenital heart disease pose technical and physiologic challenges that may not improve with conventional systemic-to-pulmonary artery shunts. The internal mammary artery (IMA) offers advantages over standard shunts and has been used in selected patients. This case report describes use of the IMA for initial palliation and subsequent reconstruction of the pulmonary artery in an infant with an atretic-thrombosed right pulmonary artery (RPA) originating from the ascending aorta...
October 1, 2014: Chest
Hiroshi Kagawa, Kiyozo Morita, Yoshimasa Uno, Yoshihiro Ko, Yoko Matsumura, Katsushi Kinouchi, Kazuhiro Hashimoto
BACKGROUND: Hyperoxemic management during cardiopulmonary bypass (CPB) is still common, and there is no consensus about physiologic oxygen tension strategy (normoxemic management) during pediatric CPB. In this study, we compared the postoperative conditions and measures of inflammatory response among patients with acyanotic congenital heart disease subjected to either hyperoxemic or normoxemic management strategy during CPB. METHODS: We studied 22 patients with a ventricular septal defect and pulmonary artery hypertension...
October 2014: World Journal for Pediatric & Congenital Heart Surgery
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