Pulmonary arterial hypertension (PAH) leads to right ventricular failure (RVF) as well as an increase in pulmonary vascular resistance. Our purpose was to study the effect of sildenafil on right ventricular remodeling in a rat model of monocrotaline (MCT)-induced RVF. The rats were distributed randomly into 3 groups. The control (C) group, the monocrotaline (M) group (MCT 60 mg/kg) and the... |
Tumor necrosis factor (TNF)-α is thought to contribute to pulmonary hypertension. We aimed to investigate the effect of infliximab (TNF-α antagonist) treatment on pathologic findings and gene expression in a monocrotaline-induced pulmonary hypertension rat model. Six-week-old male Sprague-Dawley rats were allocated to 3 groups: control (C), single subcutaneous injection of normal saline (0.1 mL/kg); monocrotaline (M), single subcutaneous injection of monocrotaline... |
Rubinstein-Taybi syndrome (RTS) is characterized by peculiar facies, mental retardation, broad thumbs, and great toes. Approximately one-third of the affected individuals have a variety of congenital heart diseases. They can also have upper airway obstruction during sleep, due to hypotonia and the anatomy of the oropharynx and airway, which make these patients susceptible to obstructive sleep apnea (OSA). In our... |
An increase in the number of preterm infants and a decrease in the gestational age at birth have resulted in an increase in the number of patients with significant bronchopulmonary dysplasia (BPD) and secondary pulmonary hypertension (PH). PH contributes significantly to the high morbidity and mortality in the BPD patients. Therefore, regular monitoring for PH by using echocardiography and B-type... |
Purpose : Despite recent advances in pulmonary hypertension management and surgery, appropriate guidelines remain to be developed for operability in congenital heart disease with pulmonary artery hypertension (PAH). Our aim was to evaluate clinical outcomes of patients with severe PAH who underwent surgical closure of left-to-right shunt lesions (LRSL) on the basis of pulmonary reactivity. Methods : We retrospectively reviewed 21... |
Transient tachypnea of the newborn (TTN), respiratory distress syndrome (RDS), and persistent pulmonary hypertension (PPHN) are the three most common disorders that cause respiratory distress after birth. An understanding of the pathophysiology of these disorders and the development of effective therapeutic strategies is required to control these conditions. Here, we review recent papers on the pathogenesis and treatment of neonatal... |
Purpose : To examine the effect of bosentan, a dual endothelin receptor (ER) antagonist, on the development of monocrotaline (MCT)-induced pulmonary hypertension in rats by especially focusing on the pulmonary vascular morphology changes. Methods : Sprague-Dawley rats were treated as follows: controls received a subcutaneous saline injection, MCT-treated rats received a subcutaneous MCT injection, and bosentan-treated rats received a MCT... |
Purpose : This study aims to describe the clinical characteristics of severe meconium aspiration syndrome (MAS) which required mechanical ventilation over 48 h and to delineate the progress of respiratory failure and radiographic findings in severe MAS. Methods : Twelve infants admitted to the Neonatal Intensive Care Unit (NICU) of the Seoul National University Bundang Hospital diagnosed with severe MAS from... |
Fetal alcohol syndrome can be suspected in infants born to mothers with a prenatal history of alcohol abuse if the child exhibits characteristic facial features, together with intrauterine growth retardation, multiple neurological abnormalities, and multiorgan defects. If only a few of the above criteria are satisfied, the term fetal alcohol effects is used. We experienced a neonate who presented with... |
Congenital pulmonary vein stenosis(CPVS) with anatomically normal connection, a rare anomaly, usually leads to progressive pulmonary hypertension, cardiac failure in infancy, and death if untreated. Most are combined with other anomalies, particularly left to right shunt lesions. Very often, the detection of CPVS is overlooked on the initial cardiac echocardiogram, because it may be mild in its severity initially, but... |
Persistent pulmonary hypertension of the newborn(PPHN) is a disorder characterized by persistence of the pattern of fetal circulation after birth due to a sustained elevation of pulmonary vascular resistance. The two primary events in the pathophysiology of neonatal pulmonary hypertension are hypoxic pulmonary vasoconstriction(HPV) and hypoxic pulmonary vascular remodeling(HPR). Chronic hypoxemia may cause increased muscularity of the pulmonary arteries and... |
Pulmonary hypertension may be associated with variable conditions such as the hyperkinetic state or pulmonary vascular obstruction. In these, stenosis of the individual pulmonary veins without any cardiac or vascular malformation is very rare. We experienced stenosis of individual pulmonary veins in a 10 months old boy who was admitted with recurrent dyspnea and cyanosis and then underwent angiogram and... |
Purpose : Inhaled nitric oxide(iNO) is an excellent method for the postoperative pulmonary hypertension in congenital heart disease. But more detailed care is needed because of the development of rebound pulmonary hypertension after NO withdrawal. We performed this study in order to discontinue the iNO successfully by way of presenting the adequate weaning and supplying methods. Methods : Between January, 1998... |
Purpose : Endothelin-1(ET-1) is a potent vasoconstrictor peptide. It has potent contractile and proliferative effects on vascular smooth muscle cells. Congenital heart diseases are often accompanied by pulmonary hypertension, and the severity of pulmonary hypertension is important in the prognosis. The aim of this study was to elucidate changes of ET-1 after pulmonary venous stenosis in a dog model, and... |
Purpose : The ratio of time to peak velocity(AT) and right ventricular ejection time(ET) as measured from the pulmonary artery Doppler waveform showed a close inverse correlation with pulmonary arterial pressure. The purpose of this study was to see the pattern of change in pulmonary arterial pressure assessed by AT/ET in preterm infants with respiratory distress syndrome (RDS). Methods : The... |
Purpose : Persistent pulmonary hypertension(PPHN) of the newborn has been treated with some vasodilators including tolazoline. But these drugs have many side effects, especially systemic hypotension . To investigate the usefulness of the nitroglycerin as a specific vasodilator with few side effects, this study was done. Methods : Nitroglycerin was administered within 1st one day after birth in 8 newborn infants who were diagnosed as... |
Purposes : Nitric oxide(NO) is classified as toxic gas in native states, but in most biologic systems NO acts as a messenger molecule. NO is known as endothelium derived relaxing factor that modulates tone of vascular smooth muscle. Inhaled NO has been reported to act as a selective pulmonary vasodilator and we expect that NO inhalation can be used as a successful treatment modality... |
Nitric oxide, an endothelium-derived relaxing factor, is a selective pulmonary vasodilator. We have built and settled down the delivery system of nitirc oxide gas inhalation for the first time in Korea. Two newborn babies delvelped near fatal pulmonary hypertension after surgical repair of a congenital diaphragmatic hernia. All conventional therapeutic measures failed. So we decided to attempt to use of nitric oxide gas for... |
Purpose : Endothelin-1 is the most potent vasoconstrictor. This study was investigated the correlation of plasma endothelin-1 and pulmonary hypertension associated with congenital heart defect. Methods : We evaluated the concentration of ET-1 in 24 cases of congenital heart disease diagnosed at the Department of Pediatrics, Pusan National University, from December 1993 to September 1994. The patients were divided into 2 groups according to mean... |
We report four cases of unilateral pulmonary vein atresia without associated congenital intracardiac anomalies to illustrate a part of the clinical and radiological characteristics and its diagnosis. Pulmonary vein atresia was in right side in all cases. Narrowing of contralateral pulmonary vein and pure red cell anemia were combined in one cases. In four cases, initial presentations were hemoptysis and... |
We have experienced a 14 year old female patient who had suffered from headache, dizziness, exertional dyspnea and chest pain during 6 months. She was diagnosed as primary pulmonary hypertension by ultrasonogram and cardiac catheterization. On the cardiac catheterization, there was elevated pulmonary artery pressure and normal pulmonary wedge pressure. We report this case with related literature review. |
Persistent pulmoanry hypertension of the newborn (PPHN) is a syndrome consisting of severe hypoxemia and pulmoanry hypertension that appears within hours of birth. The essential pathophysiologic features of this syndrome is pulmonary artery hypertension causing right-to-left shunt through the patent ductus arteriosus or foramen ovale or both and clinically manifested by central cyanosis and respiratory distress. We recently have experienced 20 hour old female and... |
Total anomalous pulmonary venous connection (TAPVC) is a rare congenital defect in which all the pulmonary veins are connected to the right atrium or its tributary veins. Without surgical intervention, most patients with TAP VC have grave prognosis, with cardiac failure and death in infancy. But owing to the advances of surgical technique and postoperative care, complete correction has been accomplished with good results. Sixteen... |