• Journal of Chest Surgery
• /
• v.48 no.1
• /
• pp.52-54
• /
• 2015

Idiopathic pulmonary arterial hypertension eventually leads to right-sided heart failure and sudden death. Its mortality rate in children is still high, despite improvements in pharmacological therapy, and therefore novel treatments are necessary. The Potts shunt, which creates an anastomosis between the left pulmonary artery and the descending aorta, has been proposed as a theoretically promising palliative surgical technique to decompress the right ventricle. We report the case of a 12-year-old girl with suprasystemic idiopathic pulmonary hypertension and right ventricular failure who underwent a Potts shunt for palliation with good short-term results.

• Tuberculosis and Respiratory Diseases
• /
• v.59 no.2
• /
• pp.170-178
• /
• 2005

Background : Idiopathic pulmonary arterial hypertension (IPAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are rare but significantly imperative in inducing chronic pulmonary hypertension. Clinically, it is difficult to distinguish between IPAH and CTEPH. However, the treatment of pulmonary hypertension is different depending on the disease. The present study was performed to analyze the similarities and differences in clinical features between IPAH and CTEPH. Methods : During a nine-year period, thirty-three patients with IPAH and twenty-two patients with CTEPH were enrolled. Symptoms, physical findings, chest radiograph, electrocardiograph, pulmonary function test, echocardiograph, perfusion lung scan, right heart catheterization results were analyzed between both the groups. Results : The median age of IPAH group was 33 (6~70) years that was lower than that (52(27~80) years) of CTEPH group. Amongst the IPAH patients, there was female predominance (76 %) and there was no sex difference between the patients with CTEPH. Both the groups exhibited similarity in the results of chest radiograph, electrocardiograph, pulmonary function test, and echocardiograph. In the perfusion lung scan, all IPAH patients exhibited findings with normal (28%) or low probability (72%) of pulmonary embolism and all CTEPH patients exhibited findings with high probability of pulmonary embolism. Conclusion : Although IPAH and CTEPH bear similarities in terms of symptoms, physical signs and general investigation results, there were differences in age distribution, sex predominance and results of perfusion lung scan.

• Tuberculosis and Respiratory Diseases
• /
• v.63 no.6
• /
• pp.475-479
• /
• 2007

• The Korean Journal of Physiology and Pharmacology
• /
• v.21 no.3
• /
• pp.353-360
• /
• 2017

Several human diseases have been associated with mitochondrial voltage-dependent anion channel-1 (VDAC1) due to its role in calcium ion transportation and apoptosis. Recent studies suggest that VDAC1 may interact with endothelium-dependent nitric oxide synthase (eNOS). Decreased VDAC1 expression may limit the physical interaction between VDAC1 and eNOS and thus impair nitric oxide production, leading to cardiovascular diseases, including pulmonary arterial hypertension (PAH). In this report, we conducted meta-analysis of genome-wide expression data to identify VDAC1 influenced genes implicated in PAH pathobiology. First, we identified the genes differentially expressed between wild-type and Vdac1 knockout mouse embryonic fibroblasts in hypoxic conditions. These genes were deemed to be influenced by VDAC1 deficiency. Gene ontology analysis indicates that the VDAC1 influenced genes are significantly associated with PAH pathobiology. Second, a molecular signature derived from the VDAC1 influenced genes was developed. We suggest that, VDAC1 has a protective role in PAH and the gene expression signature of VDAC1 influenced genes can be used to i) predict severity of pulmonary hypertension secondary to pulmonary diseases, ii) differentiate idiopathic pulmonary artery hypertension (IPAH) patients from controls, and iii) differentiate IPAH from connective tissue disease associated PAH.

• Neonatal Medicine
• /
• v.18 no.1
• /
• pp.124-129
• /
• 2011

Purpose: To evaluate the effect of oral sildenafil therapy in neonates with persistent pulmonary hypertension of the newborn (PPHN) Methods: We conducted a retrospective review of 32 neonates ${\geq}$35 weeks' gestation and fraction of inspired oxygen ($FiO_2$) 1.0 with PPHN. The first dose (0.5 mg/kg) of oral sildenafil was started and 1 mg/kg was given every 6 hour thereafter. Mean airway pressure (MAP), $FiO_2$, oxygenation index (OI), mean arterial blood pressure (MBP) were documented before and 6, 12, 24, and 48 hours after sildenafil. For adverse effects, gastrointestinal symptoms, brain ultrasound, funduscopy and auditory brainstem response results were evaluated. Results: The underlying diseases of PPHN (n=32) were meconium aspiration syndrome (n=9), respiratory distress syndrome (n=8), pneumonia (n=3), and idiopathic (n=12). Thirty-one neonates survived; 3 neonates were transferred for inhaled nitric oxide (iNO) and all of them survived. In 28 infants, $FiO_2$ and OI improved significantly by 6 hours and MAP improved significantly by 48 hours after initiation of sildenafil. There were no clinically significant adverse effects of sildenafil. Conclusion: Sildenafil may be an effective and safe agent for near-term and term neonates with PPHN, providing significant improvement in oxygenation, and thus may be especially useful in the treatment of PPHN in hospitals without iNO.