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1.
Korean Journal of Pancreas and Biliary Tract ; : 24-28, 2016.
Article in English | WPRIM | ID: wpr-98133

ABSTRACT

The pancreatic cystic lesions are known to be incidentally found up to 10-15% of patients undergoing cross-sectional imaging. And the prevalence of mucinous cystic neoplasm which has malignant potential is known to be up to 25% of all pancreatic cystic neoplasm in South Korea. The symptoms included abdominal pain, palpable mass, weight loss, loss of appetite, jaundice, asymptomatic and etc. However, spontaneous rupture of pancreatic mucinous cystadenocarcinoma (MCAC) is an extremely rare complication. Here we report a case of spontaneous rupture of pancreatic MCAC in a 72-year-old male with review of the literature. To the best of our knowledge, this is the first ruptured case of pancreatic MCAC in male patient.


Subject(s)
Aged , Humans , Male , Abdominal Pain , Appetite , Cystadenocarcinoma, Mucinous , Jaundice , Korea , Mucins , Pancreas , Pancreatic Cyst , Prevalence , Rupture , Rupture, Spontaneous , Weight Loss
2.
Korean Journal of Medicine ; : 458-467, 2012.
Article in Korean | WPRIM | ID: wpr-101019

ABSTRACT

BACKGROUND/AIMS: Adenosine deaminase (ADA) is a valuable biochemical marker for pericardial effusion (PE) and may be useful for diagnosing tuberculous pericarditis (TPE) in patients with PE. However, no definite cut-off or borderline values for ADA currently exist to distinguish TPE from other PE etiologies. In this study, we identified other useful parameters and characterized their relationship with ADA as a method for diagnosing TPE. METHODS: From June 2004 to November 2011, 42 patients underwent pericardiocentesis due to moderate or severe PE, as confirmed by echocardiography or chest computed tomography (CT). Patients were subdivided into TPE and non-TPE (NTPE) groups. We analyzed ADA (p) (the pericardial ADA) and %Lymph (p)/Glucose (p) (the ratio between the percentage of lymphocytes and glucose levels in PE). RESULTS: We defined the cut-off value of ADA (p) as 48.5 IU/L, and that of %Lymph (p)/Glucose (p) as 0.678%.dL/mg. In a multivariate logistic regression analysis, an odds ratio (OR) of 44.24 and a 95% confidence interval (CI) of 2.85-686.97 were observed in patients with an ADA (p) > or = 48.5 IU/L (p = 0.023). An OR of 20.39 and a 95% CI of 1.06-392.93 were observed in patients with a %Lymph (p)/Glucose (p) > or = 0.678%.dL/mg (p = 0.046). The combination of ADA (p) and %Lymph (p)/Glucose (p) had a higher positive predictive value (PPV, 80.0%) and specificity (Sp, 93.8%) than either ADA (p) (PPV, 47.4%; Sp, 68.8%) or %Lymph (p)/Glucose (p) (PPV, 69.2%; Sp, 87.5%) alone. CONCLUSIONS: %Lymph (p)/Glucose (p) is a useful parameter for distinguishing TPE from other pericardial diseases if combined with an ADA (p) > or = 48.5 IU/L.


Subject(s)
Humans , Adenosine Deaminase , Biomarkers , Echocardiography , Glucose , Logistic Models , Lymphocytes , Odds Ratio , Pericardial Effusion , Pericardiocentesis , Pericarditis, Tuberculous , Sensitivity and Specificity , Thorax
3.
Journal of the Korean Surgical Society ; : 55-57, 2010.
Article in Korean | WPRIM | ID: wpr-19169

ABSTRACT

Mixed squamous cell carcinoma and papillary carcinoma in the thyroid gland is a very rare malignant tumor. Primary squamous cell carcinoma of the thyroid may develop from squamous metaplasia of the follicular epithelium in a variety of pathologic conditions, such as other differentiated carcinomas of the thyroid. A 70-year-old man presented with a palpable mass on the right side of the thyroid gland. The patient underwent a right lobectomy without neck lymph node dissection. Histopathological findings revealed primary squamous carcinoma mixed papillary thyroid carcinoma. He underwent a completion thyroidectomy and radioactive iodine therapy. Adjuvant radiotherapy and chemotherapy were not performed.


Subject(s)
Aged , Humans , Carcinoma , Carcinoma, Papillary , Carcinoma, Squamous Cell , Epithelium , Iodine , Lymph Node Excision , Metaplasia , Neck , Radiotherapy, Adjuvant , Thyroid Gland , Thyroid Neoplasms , Thyroidectomy
4.
The Korean Journal of Hepatology ; : 510-516, 2009.
Article in Korean | WPRIM | ID: wpr-161888

ABSTRACT

Primary hepatic epithelioid hemangioendothelioma is a rare neoplasm of endothelial origin. The clinical manifestations are nonspecific, ranging from complete absence of symptoms to hepatic failure and death. Spontaneous rupture of a hepatic epithelioid hemangioendothelioma is an extremely rare presentation. We present a case of primary hepatic epithelioid hemangioendothelioma in a 65-year-old male patient with alcoholic liver cirrhosis. He was hospitalized due to epigastric pain and multiple liver masses on abdominal ultrasound. Dynamic liver CT imaging revealed multiple peripheral nodular enhanced mass lesions with delayed centripetal enhancement, and the adjacent collection of high-attenuation fluid along the liver capsule. Abdominal tapping revealed blood in the peritoneal cavity. Primary hepatic epithelioid hemangioendothelioma with spontaneous rupture was finally diagnosed based on a histopathologic examination revealing positive immunohistochemical staining for CD34.


Subject(s)
Humans , Male , Antigens, CD34/metabolism , Bone Neoplasms/diagnosis , Diagnosis, Differential , Hemangioendothelioma, Epithelioid/diagnosis , Liver Cirrhosis, Alcoholic/complications , Liver Neoplasms/diagnosis , Pancreatic Neoplasms/diagnosis , Rupture, Spontaneous , Tomography, X-Ray Computed
5.
Korean Journal of Medicine ; : 610-615, 2009.
Article in Korean | WPRIM | ID: wpr-151175

ABSTRACT

An osteoclast-like giant cell tumor of the pancreas is a very rare neoplasm, with only three cases reported in Korea. Due to the rarity of this tumor type, few clinical data are available. We present a case of undifferentiated carcinoma with osteoclast-like giant cell tumor arising in the tail of the pancreas in a 72-year-old woman hospitalized to evaluate epigastric pain and a palpable abdominal mass. Magnetic resonance imaging revealed the presence of a large enhancing mass with septation arising from the tail of the pancreas. A distal pancreatectomy with splenectomy was performed. The pathological diagnosis was undifferentiated carcinoma with osteoclast-like giant cell tumor. Here, we describe the histopathological and immunohistochemical findings and review the clinical features of the cases reported in the Korean literature.


Subject(s)
Aged , Female , Humans , Carcinoma , Giant Cell Tumors , Giant Cells , Korea , Magnetic Resonance Imaging , Pancreas , Pancreatectomy , Pancreatic Neoplasms , Splenectomy
6.
Journal of the Korean Surgical Society ; : 254-256, 2007.
Article in Korean | WPRIM | ID: wpr-154000

ABSTRACT

Head and neck surgeons see many congenital cysts of the neck. Most of these cysts are thyroglossal duct cysts and branchial cleft cysts. Bronchogenic cysts are rare congenital malformations of the ventral foregut development. They are usually located in the mediastinum and intrapulmonary regions. Cervical bronchogenic cysts are unusual. Only 70 cases of bronchogenic cysts in the head and neck regions have been reported on and the majority of cases have been found in the pediatric population. We describe here a 61-year-old female who presented a palpable left neck mass. The preoperative diagnostic studies included chest X-ray and sonography-guided fine needle aspiration. The neck sonography showed the mass, but it could not rule out a pathologic lymph node. Aspirated material contained no cellular content. The mass was excised. The neck mass of the patient was diagnosed as a bronchogenic cyst. We suggest that the clinical observation of a lateral neck mass in an adult includes the possibility of a bronchogenic cyst in the differential diagnosis.


Subject(s)
Adult , Female , Humans , Middle Aged , Biopsy, Fine-Needle , Branchioma , Bronchogenic Cyst , Diagnosis, Differential , Head , Lymph Nodes , Mediastinum , Neck , Thorax , Thyroglossal Cyst
7.
Korean Journal of Gastrointestinal Endoscopy ; : 287-291, 2007.
Article in Korean | WPRIM | ID: wpr-198770

ABSTRACT

Obstructive jaundice is most commonly attributed to a malignancy or stones affecting the common bile duct. Biliary tuberculosis and lymphadenitis around the periportal area have also been implicated but cases are quite rare. A 24 year old man presented with jaundice and abdominal pain for 3 days. Abdominal CT and ERCP revealed a stricture of the extrahepatic bile duct with multiple enlarged lymph nodes showing necrotic foci located at the periportal area. The colonoscopic biopsy showed evidence of M. tuberculosis. The patient was treated with ERBD insertion and oral anti-tuberculosis therapy. However, the abdominal pain recurred and there was progressive stenosis of the common bile duct. A bile duct resection with choledochojejunostomy was subsequently performed. Frozen sections revealed granulomatous inflammation with caseation necrosis, which was consistent with tuberculosis. We report a case of tuberculous cholangitis and lymphadenitis with obstructive jaundice that was managed surgically due to the progressive stricture of the bile duct.


Subject(s)
Humans , Young Adult , Abdominal Pain , Bile Ducts , Bile Ducts, Extrahepatic , Bile , Biopsy , Cholangiopancreatography, Endoscopic Retrograde , Cholangitis , Choledochostomy , Common Bile Duct , Constriction, Pathologic , Frozen Sections , Inflammation , Jaundice , Jaundice, Obstructive , Lymph Nodes , Lymphadenitis , Necrosis , Tomography, X-Ray Computed , Tuberculosis , Tuberculosis, Lymph Node
8.
Journal of Cardiovascular Ultrasound ; : 82-85, 2007.
Article in English | WPRIM | ID: wpr-141329

ABSTRACT

Spontaneous rupture of a primary cardiac angiosarcoma is a rare condition with a poor prognosis. The authors describe the case of a 48-year-old man with abnormal blood flow from a pericardial mass to the right atrium 3 months after pericardiocentesis. The flow was presumed to have occurred due to rupture of this cardiac angiosarcoma of the right atrial wall and pericardium into the right atrial cavity.


Subject(s)
Humans , Middle Aged , Echocardiography , Heart Atria , Hemangiosarcoma , Pericardiocentesis , Pericardium , Prognosis , Rupture , Rupture, Spontaneous
9.
Journal of Cardiovascular Ultrasound ; : 82-85, 2007.
Article in English | WPRIM | ID: wpr-141328

ABSTRACT

Spontaneous rupture of a primary cardiac angiosarcoma is a rare condition with a poor prognosis. The authors describe the case of a 48-year-old man with abnormal blood flow from a pericardial mass to the right atrium 3 months after pericardiocentesis. The flow was presumed to have occurred due to rupture of this cardiac angiosarcoma of the right atrial wall and pericardium into the right atrial cavity.


Subject(s)
Humans , Middle Aged , Echocardiography , Heart Atria , Hemangiosarcoma , Pericardiocentesis , Pericardium , Prognosis , Rupture , Rupture, Spontaneous
10.
Infection and Chemotherapy ; : 323-326, 2007.
Article in Korean | WPRIM | ID: wpr-722271

ABSTRACT

Infections of the intervertebral disc and adjacent vertebrae may present with spondylitis, discitis and spondylodiscitis and are hematogenous origin in most cases. Potential sources of hematogenous infection are skin and soft tissue infection, genitourinary tract infection, infective endocarditis, intravenous drug abuse, respiratory tract infection and infected intravenous injection site. We have experienced a case of pyogenic spondylitis caused by methicillin-resistant Staphylococcus aureus (MRSA) bacteremia with peripheral injection site infection. He was successfully treated with surgical debridement and antibiotics.


Subject(s)
Anti-Bacterial Agents , Bacteremia , Debridement , Discitis , Endocarditis , Injections, Intravenous , Intervertebral Disc , Methicillin Resistance , Methicillin-Resistant Staphylococcus aureus , Respiratory Tract Infections , Skin , Soft Tissue Infections , Spine , Spondylitis , Substance Abuse, Intravenous
11.
Infection and Chemotherapy ; : 323-326, 2007.
Article in Korean | WPRIM | ID: wpr-721766

ABSTRACT

Infections of the intervertebral disc and adjacent vertebrae may present with spondylitis, discitis and spondylodiscitis and are hematogenous origin in most cases. Potential sources of hematogenous infection are skin and soft tissue infection, genitourinary tract infection, infective endocarditis, intravenous drug abuse, respiratory tract infection and infected intravenous injection site. We have experienced a case of pyogenic spondylitis caused by methicillin-resistant Staphylococcus aureus (MRSA) bacteremia with peripheral injection site infection. He was successfully treated with surgical debridement and antibiotics.


Subject(s)
Anti-Bacterial Agents , Bacteremia , Debridement , Discitis , Endocarditis , Injections, Intravenous , Intervertebral Disc , Methicillin Resistance , Methicillin-Resistant Staphylococcus aureus , Respiratory Tract Infections , Skin , Soft Tissue Infections , Spine , Spondylitis , Substance Abuse, Intravenous
12.
Korean Circulation Journal ; : 66-71, 2006.
Article in Korean | WPRIM | ID: wpr-80342

ABSTRACT

BACKGROUND AND OBJECTIVES: The use of drug-eluting stent implantation for in-stent restenosis (ISR) has shown some promising results. This study investigated the clinical and angiographic results of performing sirolimuseluting stent (SES) implantation for the treatment of patients with ISR as compared with treatment with cutting balloon angioplasty (CBA). SUBJECTS AND METHODS: Forty one patients with ISR (43 lesions) were treated with SES implantation and they were retrospectively compared with a group of patient with matched lesions that were treated with CBA (57 patients with 61 lesions). Routine angiographic follow-up was obtained at about 6 months after treatment and the incidence of major adverse cardiovascular events was evaluated. RESULTS: The baseline clinical characteristics, the patterns of ISR and the minimal luminal diameters (MLD) were not different between the two groups. There was no procedural failure and no immediate major cardiovascular adverse events (MACE) in the two groups. There were two target lesion revascularizations during the clinical follow-up in the CBA group and no MACE was found in the SES group (4% vs. 0%, respectively p<0.001). On the follow-up angiogram, the MLD and the stenosis diameter were significantly lower in the SES group than in the CBA group (2.82+/-0.33 vs. 1.80+/-0.82 mm, p<0.001; and 11.3+/-9.6 vs. 41.2+/-24.8%, p<0.001, respectively). The acute gain was significantly higher and the late loss was significantly lower in the SES group than in the CBA group (2.64+/-0.38 vs. 2.02+/-0.44 mm, p<0.001; and 0.21+/-0.28 vs. 0.71+/-0.73, p<0.001, respectively). CONCLUSION: The sirolimuseluting stent was safe, feasible and highly effective for treating the patients suffering with in-stent restenosis as compared with cutting balloon angioplasty.


Subject(s)
Humans , Angioplasty , Angioplasty, Balloon , Constriction, Pathologic , Coronary Restenosis , Drug-Eluting Stents , Follow-Up Studies , Incidence , Phenobarbital , Retrospective Studies , Sirolimus , Stents
13.
Korean Circulation Journal ; : 817-819, 2006.
Article in English | WPRIM | ID: wpr-197266

ABSTRACT

Among all the congenital coronary anomalies, an anomalous origin of the left coronary artery (LCA) from the right sinus of Valsalva is rare. A 48-year-old male patient suffering with lateral acute myocardial infarction was referred for primary percutaneous coronary intervention. The initial angiogram failed to show the LCA, which originated from the right coronary sinus. A critical stenotic lesion was observed in the distal left circumflex artery. The lesion was treated successfully with stenting. We report here on a case of an anomalous origin of the left coronary artery from the right sinus of Valsalva, and the patient presented as acute myocardial infarction. He was successfully treated with primary percutaneous intervention.


Subject(s)
Humans , Male , Middle Aged , Arteries , Coronary Sinus , Coronary Vessel Anomalies , Coronary Vessels , Myocardial Infarction , Percutaneous Coronary Intervention , Sinus of Valsalva , Stents
14.
Journal of the Korean Society of Neonatology ; : 93-99, 2005.
Article in Korean | WPRIM | ID: wpr-94006

ABSTRACT

Twin-twin transfusion syndrome is attributed to an unbalanced blood flow between the donor and the recipient twin, but the exact pathophysiology remains incompletely understood. Despite active prenatal management, fetal twin-twin transfusion syndrome is still associated with a substantial residual perinatal mortality and morbidity. The donor twin progressively becomes anemic, growth restricted, oliguric and has oligohydramnios, where as the recipient becomes plethoric, polyuric and has polyhydramnios and in the most severe cases develops cardiomegaly, congestive heart failure and hypertension. We report a case of severe systemic hypertension developed in recipient twin.


Subject(s)
Female , Humans , Pregnancy , Cardiomegaly , Heart Failure , Hypertension , Oligohydramnios , Perinatal Mortality , Polyhydramnios , Tissue Donors
15.
Korean Circulation Journal ; : 702-705, 2005.
Article in English | WPRIM | ID: wpr-128182

ABSTRACT

Rheumatoid arthritis patients have an increased risk of subclinical cardiovascular disease, and they also have a high prevalence of carotid disease and peripheral arterial disease as a form of vasculitis. Rheumatoid arthritis patients have an increased cardiovascular mortality rate and an increased premature death rate, and they have a higher incidence of atherosclerosis. Myocardial infarction due to vasculitis is a rare complication for patients with rheumatoid vasculitis. We report here on a case of a patient with multiorgan involvement who developed myocardial infarction, right carotid artery occlusion and left renal artery occlusion secondary to his rheumatoid vasculitis.


Subject(s)
Humans , Arthritis, Rheumatoid , Atherosclerosis , Cardiovascular Diseases , Carotid Arteries , Carotid Stenosis , Incidence , Mortality , Mortality, Premature , Myocardial Infarction , Peripheral Arterial Disease , Prevalence , Renal Artery , Rheumatoid Vasculitis , Vasculitis
16.
Journal of the Korean Surgical Society ; : 153-158, 2004.
Article in Korean | WPRIM | ID: wpr-173612

ABSTRACT

Hepatoid adenocarcinoma is a rare variant of adenocarcinoma of the stomach. The tumor has been found to be an alpha-fetoprotein (AFP) producing carcinoma arising in extrahepatic organs, and it mimics hepatocellular carcinoma in terms of morphology and function. Vascular invasion, usually prominent, is often complicated by extensive liver metastases, and vascular permeation, especially in the veins, has been described as a characteristic finding of this tumor. A patient with hepatoid adenocarcinoma of the stomach with a huge mass is described. Gastrofiberscopy revealed an elevated lesion with a central depression on the greater curvature of the antrum and with extrinsic compression on the lesser curvature and the duodenum. Computed tomography revealed a large lobulated mass in the lesser curvature of the stomach, attached from the liver, gall bladder and porta hepatitis to the pancreas. The AFP serum level was markedly elevated. After a diagnosis was made of AFP-producing stomach carcinoma with huge lymph node metastasis, subtotal gastectomy with wedge resection of the liver, and cholecystectomy including the huge mass ware performed. Microscopically, the tumor and intraabdominal huge mass showed mainly hepatoid differentiation. The tumor showed immunohistochemical positivity for AFP and huge lesser omental metastasis with adhesion to the liver and extensive venous invasion. Lymph node metastasis was not found. According to these histopathological findings, the tumor was diagnosed as hepatoid adenocarcinoma of the stomach with venous invasion. We report this rare variant of adenocarcinoma of the stomach, which mimics hepatocellular carcinoma in its propensity for venous permeation.


Subject(s)
Humans , Adenocarcinoma , alpha-Fetoproteins , Carcinoma, Hepatocellular , Cholecystectomy , Depression , Diagnosis , Duodenum , Hepatitis , Liver , Lymph Nodes , Neoplasm Metastasis , Pancreas , Stomach , Urinary Bladder , Veins
17.
Journal of the Korean Surgical Society ; : 354-357, 2004.
Article in Korean | WPRIM | ID: wpr-174971

ABSTRACT

A case of a mucin-producing intrahepatic cholangiocellular carcinoma (MPCC) is reported. A 58-year old female presented with epigastric discomfort of several years duration. The physical examination and laboratory findings were normal. Abdominal ultrasonography (US) and computed tomography (CT) showed a focal dilatation of the right posterior intrahepatic bile duct. There was no abnormal mass in the liver parenchyma. Endoscopic retrograde cholangiopancreaticography (ERCP) showed a filling defect in the right posterior hepatic duct. There was no anatomical abnormality and abnormal staining on the heaptic angiography. At the operation, the right posterior hepatic duct was filled with mucin. The patient had a right posterior segmentectomy. Histologically, a 2.5 X 0.6 X 0.6 cm sized mucin-producing intrahepatic cholangiocellular carcinoma was found in segment 6 of the liver. The postoperative recovery was good, and the patient has had a good social life for the last 3 years, with no evidence of tumor recurrence. In patients with a focal dilatation of the intrahepatic bile duct on CT or US with no underlying cause, an intrahepatic malignancy has to be suspected.


Subject(s)
Female , Humans , Middle Aged , Angiography , Bile Ducts, Intrahepatic , Cholangiocarcinoma , Dilatation , Hepatic Duct, Common , Liver , Mastectomy, Segmental , Mucins , Physical Examination , Recurrence , Ultrasonography
18.
Journal of the Korean Society of Echocardiography ; : 31-34, 2003.
Article in Korean | WPRIM | ID: wpr-81464

ABSTRACT

Primary cardiac tumors in infancy and childhood are rare, with fibromas being the second most common tumor after rhabdomyomas. Although cardiac fibromas are characteristically benign intramural tumors, they may exhibit expansile growth resulting in obstruction, valvular dysfunction, as well as other problems so early diagnosis and successful surgical excision are important. We experienced a case of cardiac fibroma in right ventricle that diagnosed by transthoracic echocardiography, chest CT, open heart tissue biopsy etc.


Subject(s)
Biopsy , Early Diagnosis , Echocardiography , Fibroma , Heart , Heart Neoplasms , Heart Ventricles , Rhabdomyoma , Tomography, X-Ray Computed
19.
Korean Circulation Journal ; : 196-204, 2003.
Article in Korean | WPRIM | ID: wpr-211564

ABSTRACT

BACKGROUND AND OBJECTIVES: The optimal treatment for in-stent restenosis(SR) s controversial, although intracoronary radiation therapy(CRT) as provided the most consistent results to date. This study was designed to assess the early and late angiographic results, and to find independent predictors of recurrent restenosis, following cutting balloon angioplasty(BA) or ISR. SUBJECTS AND METHODS: Eighty patients(7 lesions) ith first time ISR underwent CBA and systematic follow-up(U) ngiography. A conventional balloon was used before, or after, the CBA, if required. ICRT was used in 18 lesions(1%). A multivariate logistic regression analysis was performed.(why?) RESULTS: he ISR was focal(n=2, 37%), diffuse or proliferative(n=1, 58%) nd occlusive(n=4, 5%). Procedural success was achieved in all 87 lesions(00%). No significant edge dissection occurred. The pre- and post-procedural diameter stenoses(S) ere 81.5+/-10.8% and 6.7+/-6.0%, respectively, and the pre- and post-procedural MLD(efine MLD?) .71+/-0.44 mm and 2.85+/-0.32mm, respectively, with 2.14+/-0.44mm of acute gain. A FU angiography was performed in 54(8%) f the 69 lesions treated with CBA alone. The overall angiographic restenosis rate was 24%(3/54), with 9%(/22) n the focal ISR and 34%(1/32) n the diffuse or occlusive ISR. The FU DS and MLD were 32.0+/-23.4% and 2.1+/-0.7mm, respectively, with 0.79+/-0.69mm of late loss. The length of a restenotic lesion(R 12.2, 95% CI:1.3-115.2, p=.0286) as an independent predictor of recurrent restenosis. CONCLUSION: CBA is a simple and efficient first line treatment for ISR, with an acceptable restenosis rate, and the length of a restenotic lesion is an independent predictor of recurrent restenosis. In diffuse or occlusive ISR, more definite treatment modalities, such as ICRT combined with CBA or debulking techniques, might be required to reduce recurrent restenosis.


Subject(s)
Angiography , Angioplasty , Angioplasty, Balloon , Coronary Restenosis , Logistic Models , Stents
20.
Korean Circulation Journal ; : 507-512, 2003.
Article in Korean | WPRIM | ID: wpr-219221

ABSTRACT

BACKGROUND AND OBJECTIVES: Primary (idiopathic) pulmonary hypertension is a rare, progressive and fatal disease. It has been defined, by the World Health Organization, as a mean pulmonary arterial pressure greater than 25 mmHg at rest, or greater than 30 mmHg during exercise, without the apparent cause of secondary pulmonary hypertension. This study was performed to better understanding the clinical presentation, natural history and prognosis of primary pulmonary hypertension. SUBJECTS AND METHODS: A total of 18 patients, who were diagnosed as primary pulmonary hypertension, at three University Hospitals, were retrospectively reviewed. All patients had undergone echocardiography and cardiac catheterization. RESULTS: With the patients there was a male: female ratio of 1:8, ranging in age between 10 and 50 years. The most common presenting symptom was dyspnea on exertion, with other symptoms comprising of fatigue in 11, chest pain in 5, syncope in 3 and hemoptysis in 2. The ECG & echocardiography reflected the presence of right-sided heart enlargement. The average right ventricular systolic pressure, from Doppler echocardiography, was 73.6+/-18.8 mmHg. The mean pulmonary artery pressure and pulmonary capillary wedge pressure were 52.9+/-18.4 and 9.2+/-3.1 mmHg, respectively. The survival times were within 30 and 21 to 60 months in 9 and the remaining patients, respectively. CONCLUSION: We conclude that primary pulmonary hypertension is common in female patients in their third to fifth decades. This study also showed a poor prognosis, as in other reports.


Subject(s)
Female , Humans , Male , Arterial Pressure , Blood Pressure , Cardiac Catheterization , Cardiac Catheters , Cardiomegaly , Chest Pain , Dyspnea , Echocardiography , Echocardiography, Doppler , Electrocardiography , Fatigue , Hemoptysis , Hospitals, University , Hypertension, Pulmonary , Natural History , Prognosis , Pulmonary Artery , Pulmonary Wedge Pressure , Retrospective Studies , Syncope , World Health Organization
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