Pulmonary Extraintestinal Manifestation of Crohn's Disease Treated Successfully with Adalimumab / 대한소화기학회지

Pulmonary extraintestinal manifestation is rare in Crohn's disease and has been reported in only a few cases. Despite the presence of pulmonary abnormalities in a significant proportion of patients with inflammatory bowel disease, there are only few case reports, due to complicated diagnosis and low recognition by clinicians. Currently, treatment guidelines for pulmonary Crohn's disease have not been established. There are some case reports of pulmonary Crohn's disease that achieved remission after infliximab treatment. Clinical and radiological remission of pulmonary extraintestinal involvement in Crohn's disease after adalimumab therapy has not been reported yet. Here, we report one case of lung involvement of Crohn's disease, which shows radiological and clinical remission after adalimumab treatment.

Spontaneous regression of cardiac sarcoidosis resulting in total occlusion of coronary artery and ventricular aneurysm

No abstract available.

Spontaneous regression of cardiac sarcoidosis resulting in total occlusion of coronary artery and ventricular aneurysm

No abstract available.

A Case of Thymic Carcinoma with Behcet's Disease Combined with Immunoglobulin A Nephropathy

Behcet's disease is a systemic inflammatory disorder of unknown etiology, characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesions. Renal involvement is rare in patients with Behcet's disease particularly immunoglobulin A (IgA) nephropathy. Other autoimmune diseases have been associated with increased risk of malignancy, but not Behcet's disease. Some cases of Behcet's disease accompanied by bladder cancer, thyroid cancer, stomach cancer, or hematologic malignancies have been reported. However, to the best of our knowledge, co-occurrence of Behcet's diseases with thymic carcinoma has not yet been reported. We experienced a 49-year-old male patient who had been treated for Behcet disease and IgA nephropathy, who presented with a large mediastinal mass on chest x-ray. After thymectomy, he was diagnosed with thymic carcinoma with complete resection.

A Case of Pleural Metastasis from Papillary Tthyroid Carcinoma / 결핵및호흡기질환

Lung cancer, breast cancer and lymphoma are the common oncologic causes of malignant pleural effusion, comprising more than the half of the causes. However, an endocrinologic carcinoma associated malignant effusion is very rare. Recently, we encountered a case of papillary thyroid carcinoma causing malignant effusion. An 83-year-old female patient presented with dyspnea due to massive pleural effusion in her left side. The pleural biopsy, pleural fluid cytology and breast needle aspiration biopsy results were consistent with a metastatic papillary thyroid carcinoma. Thyroid ultrasonography showed two thyroid masses, but the patient refused a thyroid biopsy. This case highlights the need for considering the possibility of papillary thyroid carcinoma when the cause of malignant pleural effusion cannot be found because one of the rare clinical manifestations of a papillary thyroid carcinoma can be dyspnea due to malignant effusion.

A Case of Pleural Metastasis from Papillary Tthyroid Carcinoma / 결핵및호흡기질환

Lung cancer, breast cancer and lymphoma are the common oncologic causes of malignant pleural effusion, comprising more than the half of the causes. However, an endocrinologic carcinoma associated malignant effusion is very rare. Recently, we encountered a case of papillary thyroid carcinoma causing malignant effusion. An 83-year-old female patient presented with dyspnea due to massive pleural effusion in her left side. The pleural biopsy, pleural fluid cytology and breast needle aspiration biopsy results were consistent with a metastatic papillary thyroid carcinoma. Thyroid ultrasonography showed two thyroid masses, but the patient refused a thyroid biopsy. This case highlights the need for considering the possibility of papillary thyroid carcinoma when the cause of malignant pleural effusion cannot be found because one of the rare clinical manifestations of a papillary thyroid carcinoma can be dyspnea due to malignant effusion.

Localized Fibrosing Mediastinitis with Superior Vena Caval(SVC) Syndrome / 결핵및호흡기질환

Fibrosing mediastinitis is a rare disease that is characterized by the proliferation of dense fibrous tissue of the mediastinum. The pathogenesis of fibrosing mediastinitis is unknown in most cases. However, histoplasmosis, tuberculosis, autoimmune disease, radiation therapy, and other idiopathic fibroinflammatory diseases have been implicated in some cases. Most clinical features are related to an obstruction or compression of the mediastinal structure. Fibrosing mediastinitis is often progressive and occurs diffusely throughout the mediastinum. We encountered a case of fibrosing mediastinitis of a very focal lesion without evidence of mediastinal involvement. The condition was confirmed by biopsy and graft bypass surgery was performed because of SVC syndrome.

A Case of Benign Metastasizing Pulmonary Leiomyoma / 결핵및호흡기질환

A benign metastasizing pulmonary leiomyoma (BMPL) is a rare disease that usually occurs in women with a prior or coincident history of uterine leiomyoma. Although leiomyoma is histologically benign, it has the potential to metastasize to a distant site such as the lung. A 35 year old woman who had undergone a hysterectomy due to uterine leiomyoma 5 years prior was admitted for an investigation of multiple pulmonary nodules on a routine chest roentgenogram. An open lung biopsy was taken to make a pathological diagnosis. The microscopic finding of the nodules was leiomyoma and was similar to those of the uterine leiomyoma that had been resected 5 years ago. The woman underwent wedge resections of all pulmonary nodules. This is the first case of BMPL in Korea, which was treated with wedge resections of all multiple pulmonary nodules.

Prevalence of Antibodies to PPD and Lipoarabinomannan of Mycobacterium tuberculosis among Patients with an Indication of Fine Needle Aspiration Biopsy

Recent increase in the incidence of lung cancer often makes it difficult to differentiate between lung cancer and tuberculosis (TB), due to their radiologic similarities. Fine needle aspiration biopsy (FNAB) has been widely employed for the diagnosis of lung cancer and TB, but the diagnostic accuracy of TB is not high enough. As a rapid screening test for tuberculosis, we evaluated serological tests using Mycobacterium tuberculosis PPD and lipoarabinomannan (LAM) antigens. A total of 95 patients with indication of FNAB cytology from initial CT findings were enrolled. 25 patients had TB, 76 thoracic malignancy, and six (7.9%) of the lung cancer patients also had TB, indicating much higher prevalence of TB in thoracic tumor patients. Antibodies to PPD were elevated in 18 (72.0%) of 25 TB patients and in 22 (31.4%) of 70 patients with thoracic malignancy. In contrast, only 3 (4.7%) of 64 healthy controls aged 40 or above were seropositive to PPD antigen. The prevalence of anti-PPD antibodies in thoracic tumor patients was therefore significantly greater than that amongst the healthy controls (p 0.001, chi-square test). However, no significant difference in the prevalence of anti-LAM antibodies was found between study subjects and controls. This study demonstrates that thoracic tumor patients have significantly elevated antibodies to PPD; therefore, high anti-PPD seroreactivity in thoracic tumor patients should be cautiously interpreted. A longitudinal investigation on seropositive thoracic tumor patients is required to determine the role of the serological test for TB in lung cancer patients.

HRCT Findings of Hypersensitivity Pneumonitis: Correlation with Pulmonary Function Test

PURPOSE: To evaluate the HRCT findings of hypersensitive pneumonitis and to correlate the findings with the results of the pulmonary function test (PFT). MATERIALS AND METHODS: Seven patients in whom hypersensitive pneumonitis was histologically confirmed (by transbronchial lung biopsy in two, thoracoscopic lung biopsy in one, open lung biopsy in two, and typical clinical and laboratory findings in two) were involved in this study. Their radiological patterns were assessed by HRCT and the extent of each finding was evaluated semi-quantitatively and correlated with the results of the pulmonary function test. RESULTS: The HRCT findings were as follows: lobular overinflation (n = 7), ground glass attenuation (n = 7), centrilobular nodule (n = 6), reticular opacity (n = 5), interlobular septal thickening (n = 3), consolidation (n = 2), and irregular subpleural line (n = 1). Five patients showed lower lung predominance and two, middle lung predominance. In all, a restrictive pattern and diminished diffusion capacity was noted. The grade score of reticular opacity showed significant correlation with forced vital capacity and forced expiratory volume. There was, however, no significant correlation between other HRCT findings and PFT results. Two patientsin whom lobular overinflation associated with parenchymal fibrosis was noted showed a decreased maximal midexpiratory flow rate of 25 -75. CONCLUSION: Lobular overinflation, ground-glass attenuation and centrilobular nodules are commonly observed in hypersensitive pneumonitis. The only significant correlation between each HRCT finding and the pulmonary function test was that between reticular opacity and both forced expiratory volume and forced vital capacity. In cases of chronic hypersensitive pneumonitis presenting as pulmonary fibrosis, associated lobular overinflation could be helpful for differential diagnosis.

Pulmonary Epithelioid Hemangioendothelioma: Radiologic Findings

PURPOSE: To describe the computed tomographic (CT) findings and follow-up changes of pulmonary epithelioidhemangioendothelioma (PEH). MATERIALS AND METHODS: The clinical and serial radiological findings [follow-up,5months-5 years (mean, 26.4 months)] of five patients with histologically proven PEH were retrospectivelyreviewed. Three were men and two were women, and they were aged between 25 and 54(mean, 35.6) years. Initial chestradiographs were available in all cases and HRCT, conventional CT and MRI were available in one, respectively.Follow-up conventional CT (n=3) and HRCT (n=2) were performed, and the size, number and distribution of thenodules, calcification and follow-up changes were analyzed. RESULTS: In four patients, chest radiography and CTrevealed bilateral multiple nodules 1-15mm in size. In two patients, the nodules showed interstitial distribution,and one had a single nodule in the right upper lobe. On follow-up CT images, an endobronchial mass withobstructive pneumonitis (n=1) or consolidation with pleural effusion (n=1) was noted. In three patients, thenodules had increased in size and number, and calcification within the nodules was observed in two. CONCLUSION:PEH usually manifests as widespread nodules, sometimes with calcification. Along with larger and increased numbersof nodules, follow-up images of PEH may show an endobronchial mass with obstructive pneumonitis or aconsolidation-like mass with pleural effusion.

Findings Chest Radiograph and CT in Mediastinitis: Effcacy of CT in Patients with Delayed Diagnosis

PURPOSE: To analyse the causes and radiologic findings in patients with mediastinitis and to evaluate theefficacy of chest CT scanning in patients with delayed diagnosis. MATERIALS AND METHODS: Seventeen patients withhistopathologically(n=15) or cliniclly diagnosed(n=2) mediastinitis were involved in this study. Eleven of theformer group underwent surgery, and in four, tube drainage was performed. All underwent chest radiography and CTscanning, and in seven patients, the causes of delayed diagnosis were analysed. RESULTS: The most common cause ofmediastinitis was esophageal rupture (n=11). Others were extension from neck abscess to the mediastinum(n=3),complications after a Benthall procedure(n=1), tuberculous lymphadenitis (n=1) and mycotic aneurysm(n=1). Patientswith esophageal rupture suffered from underlying diseases such as esophageal cancer(n=2), iatrogenic esophagealrupture(n=2), Boerhaave's syndrome(n=2), and esophagitis(n=1). In patients with neck abscess(n=3), each wassecondary to infected cystic hygroma, Ludwig angina, or deep neck infection, respectively. On chest CT, patientswith esophageal rupture(n=11) had an abscess in the posterior mediastinum; nine abscesses extended to the cervicalarea along the retropharyngeal space, and the patient with Ludwig angina had an abscess involving all compartmentsof the mediastinum. Among the total of 17 patients, diagnostic delays were found in seven, while five hadspontaneous esophageal ruptures and two suffered complications after a Benthall procedure and Tbc lymphadenitis,respectively. The causes of diagnostic delay varied. Among seven patients, pnevmonia was initially diagnosed intwo, who were treated ; one had multiorgan failure, and one was suffering from pericardial effusion and lungabscess. In three other patients, chest radiographs initially showed non-specific findings, leading to delayed CTexamination. CONCLUSION: The most common cause of mediastinitis was esophageal rupture, and in these patients,chest radiographs and clinical symptoms were sometimes not specific. CT was valuable for the detection ofmediastinitis, and for early diagnosis can be the modality of choice.

Correlation Between Obstructive Coronary Artery Disease and Electron Beam Tomography Coronary Artery CalciumScan

PURPOSE: To determine the correlation between obstructive coronary artery disease and electron beamtomography coronary artery calcium(EBT CAC) scan and to measure the difference in calcium score according tosymptoms. MATERIALS AND METHODS: Fifty-six patients underwent EBT CAC scanning and either coronary angiography orstress thallium 201 scanning or the treadmill test. When the results were positive, coronary artery obstructivedisease(CAOD) was assumed to be present. The patients were divided into three groups : symptomatic CAOD,asymptomatic CAOD, and asymptomatic non- CAOD; those with a previous history of myocardial ischemia or who showedpositive results in any of the three tests relating to typical symptoms of angina were assigned to the symptomaticgroup. RESULTS: The number of cases assigned to group to group 1,2 and 3 was 19, 16 and 21, respectively; totalCAC scores were 571+/-751, 600+/-726 293+/-401, respectively. The difference in CAC score between asymptomatic CAODand asymptomatic non- CAOD was not statistically significant(p=0.079) but in asymptomatic CAOD, the score tendedto be higher. The CAC score was not different between symptomatic and asymptomatic CAOD(p>0.1). When the CACthreshold was 1, sensitivity was 89% and specificity was 14%;when the threshold was 200, sensitivity was 60% andspecificity was 67%. CONCLUSION: When the EBT CAC score is high, further evaluation provides early evidence ofcoronary artery obstructive disease.

Analysis of Mediatinal Lymph Nodes with Internal Low Density on Contrast Enhanced CT Scan / 결핵

BACKGROUND: To analyze the morphologic characteristics of low density lymph node in etiologic differentiation of lymphadenopathy, emphasizing the different features between tuberculosis and lung cancer, on contrast enhanced CT scan,. METHOD: A total of 64 patients who showed low density lymph nodes on chest CT scan were analyzed. Primary causes were tuberculosis (n=28), lung cancer (n=27), malignant lymphoma (n=5) and metastasis from extrathoracic malignancies (n = 4). CT scan was performed with 10mm slice thickness and 7 characteristic features were evaluated: location,size, presence or absence of the nonnecrotic lymph node, calcification, perinodal fat obliteration, thickness and evenness of the enhancing rim. RESULTS: In patients with tuberculous lymphadenopathy, lymph nodes with uneven (68.0%) and thick (62.1%) enhancing rim were more common than lung cancer (p<0.05). Low density lymph nodes with less than 1cm in size were found only in tuberculous lymphadenopathy(n=10). In 48.2% of patients with lung cancer, more than 1 nonnecrotic enlarged lymph node were coexisted, whereas 21.4% in patients with tuberculous lymphadenopathy(p=0.06). However, the size, location and calcification were not statistically significant between tuberculous lymphadenopathy and lung cancer. CONCLUSION: Tuberculous lymphadenopathy is strongly suggested when enhancing rim of enlarged lymph nodes is uneven and thick, when the coexisting nonnecrotic lymph nodes are few in number and when central low density is encountered in normal sized lymph nodes.

The Role of MR Imaging in Determination of Atrial Situs in Congenital Heart Disease with Situs Ambiguus

PURPOSE: to assess the role of MR imaging in determining of the atrial situs in complicated congenital heart disease with situs ambiguus. MATERIALS AND METHODS: In order to classify the situs, the morphology of atrial appendages, on bronchial length ratio, the superior-inferior relation of the pulmonary artery (PA) and main bronchi on each side, and splenic abnormality were evaluated by MR imaging in 22 patients (12 boys and 10 girls), and the results were compared. RESULTS: In all patients, the superior-inferior relation of the PA and main bronchi tended to lateralize, and in one, bronchial length ratio was not consistent with the relation between the PA and bronchus. Bronchial and atrial situs, as determined by appendage morphology, were consistent in ten of 13 right isomerism patients, and in only three of nine of these with left isomerism. All 13 right isomerism patients, classified by the relation of the PA and main bronchi, showed asplenia, whereas eight of nine of these with left isomerism had polysplenia. CONCLUSION: In the assessment of atrial situs by MR imaging, the positional relation of a bronchus and the PA, bronchial length ratio, and splenic abnormality are constant and reliable. The accuracy of classification of situs on the basis of atrial appendage morphology is, however, limited.

A case of allergic bronchopulmonary asperogillosis with atypical asthma / 알레르기

The tests for sputum acid fast bacilli and sputum cytology for malignancy were negative. The PC20 of bronchial methacholine challenge test was 20.2 mg/ml. Transbronchial lung biopsy showed bronchial inflammatory change with infiltration of eosinophils and the existence of fungal hypae. Antibody index for serum IgE-Af and IgG-Af was 10.2 and 2.1 respectively, comparing with Af-sensitive asthma patients. Recently we experienced an allergic bron-chopulmonary aspergillosis with atypical respiratory symptoms. Forty-seven years old female was admitted to our hospital because of cough, sputum, right pleuritic pain for 2 weeks. She had experienced pneumonia several times, but had been not confirmed any causative organism. On physical examination, breathing sound was decreased over right upper lung, but wheezing and crackle were not heard over both lungs. The chest X-ray showed segmental consolidation in right upper lobe, and HRCT showed tubular bronchiectasis and obstruction of right upper lobal bronchus due to mucoid impaction. Allergic skin prick test against Aspergillus fumigatus(Af) and serum preeipitin were negative, but intrader mal skin test was positive for Af. Specific IgE for Af was positive with class I. Total eosinophil count was 650/mm3, total IgE level was more than 3000 IU/ml, ESR was 62mm/hr, and eosinophils in induced sputum was 35%. The test for sputum acid fast bacilli and spu-Tum cytology for malignancy were negative. The PC20 of bronchial methacholine challenge-Test was 20.2 mg/ml. Transbronchial lung bi-Opsy showed bronchial inflammatory change With infiltration of eosinophils and the exis-Serum lgE-Af and lgG-Af was 10.2 and 2.1 Respectively, comparing with Af-sensitive Asthma patients.

MR Imagine of Systemic and Pulmonary Venous Return in Congential Cardiac Defects with Situs Ambiguus

BACKGROUND: Preoperative identification of systemic and pulmonary venous return is essential for surgical design in situs ambiguus. This study was carried out to evaluate anatomy of systemic and pulmonary venous return and to assess clinical efficacy of magnetic resonance imagine(MR) by comparing with results of cardiac catheterization(Cath) and echocardiography(Echo). MATERIALS AND METHODS: MR performed on 22 patients with cardiac situs ambiguus(right isomerism ; 13, left isomerism ; 9). MR findings were compared with the findings of Cath and Echo for the assessment of diagnostic accuracy of MR in 19 patients. RESULTS: 1) Interruptions of IVC with azygous continuation were found in all patients of left isomerism. But IVC was drained to right of left sided atria in right isomerism. 2) Brlateral SVC were found in 12 of 22 situs ambiguus(left isomersm ; 5, right isomerism ; 8). 3) Total anomalous pulmonary venous returns(TAPVR) were found in 7 of 12 right isomerism. Location of vertical veins were as follows ; prearterial(n=1), retroarterial-prebronchial(n=3), retrobronchial(n=3). In 5 patients of remained 6 right isomerism, pulmonary venous returns(PAPVR) in which right and left pulmonary veins entered to right and left atrium respectively, were found in 5 of 9 left isomerism. 4) Compared with Cath and Echo findings(n=19) in which MR, Cath and Echo were performed simultaneously, TAPVR were found in 6 cases on MR but 2 cases on Cath and Echo. The cases that were not detected by Cath showed severe decrement of pulmonary flow due to hypoplasia of pulmonary artery or obstruction of pulmonary vein. On MR, accurate anatomy of PAPVR were found only in 4 cases. CONCLUSIONS: MR can provide accurate and complete imaging of systemic and pulmonary venous return in sitrs ambiguus. Especially, MR is superior to Cath or Echo in depiction of TAPVR with severe decrement of pulmonary flow of obstruction of pulmonary vein, PAPVR and bilateral SVC.

Hepatic Venous Return in Atrial Isomerism Evaluated by MR / 대한흉부외과학회지

We performed this study to evaluate hepatic venous drainage in atrial isomerism by MR and the clinical significance of anomalous hepatic venous return in total cavopulmonary shunt operation. Numbers and locations of hepatic veins in twenty-two patients with isomerism(thirteen with right isomerism and nine with left isomerism) were evaluated by MR. Operative procedure of hepatic veins and postoperative arterial oxygen saturation were compared with hepatic vein connection in six patients after total cavopulmonary shunt operation. Among nine patients with left isomerism, hepatic venous return was totally anomalous via a single opening in eight, and via two separate openings in one. Among thirteen patients with right isomerism, partial anomalous hepatic venous connection directly to the atrium was seen in four. One showed total anomalous hepatic venous connection to atrium through one opening. Total cavopulmonary shunt operation was performed in 6 patients. Hepatic veins were connected to pulmonary arteries in four patients who had one atrial opening of hepatic vein and/or IVC, or two ipsilateral atrial opening of hepatic veins and IVC. In conclusion, hepatic vein drainage to atrium is variable in atrial isomerism. MR is useful for evaluation of hepatic vein drainage in atrial isomerism and surgical planning.

CT Findings and Types of Tuberculous Chest Wall Abscess / 결핵

BACKGROUND: Tuberculous chest wall abscess is a rare complication of tuberculosis. However, there have been few reports about the variable extents and shapes of tuberculous chest wall abscesses. We analyzed the extent and shape of tuberculous chest wall abscesses and grouped them according to combined pleuroparenchymal lesions by CT scans. MATERIALS AND METHODS: CT fadings were evaluated in 20 patients of tuberculous chest wall abscesses. We classified 29 abscesses in 20 patients into three types according to pleuroparnechymal lesions. Type 1 was defined when there was no active pleuroparenchymal lesion, Type 2, when intrathoracic tuberculosis was contacted with chest wall abscess, Type 3, when ipsilateral subpleural nodules were not contacted with chest wall abscess. RESULTS: The type 1 included 6 abscesses in 6 patients. They showed rib and/or postal cartilage destruction in their center. They were relatively large and round. The type 2 included 13 abscesses in 10 patients. The abscesses in contact with pleural lesion or mediastinal lesion were mainly located in the outer muscle layer, and they were relatively large in size, However, the abscesses in contact with parenchymal lesion were mainly located in extrapleural space. They were relatively small and they were longest along the long axis of ribs. The type 3 included 10 abscesses in 6 patients. They were located mainly in the extrapleural space. CONCLUSION: Tuberculous chest wall abscesses showed variable extents and shapes according to pleuroparenchymal lesions. CT is a good diagnostic modality to visualize the extent of tuberculous chest wall abscess and combined pleuroparenchymal lesion.

Post-exercise response of ventricular ejection fraction after total repair of congenital heart disease with left to right shunt

A radioisotope first pass study was done on patients over a period of 1 to 15 years (average 4.6 years) after repair for ventricular septal defect or arterial septal defect with a left to right shunt. The age of the patients ranged from 6 to 32 years (average 14.2 year) at the time of the study. The total work of exercise and the right and left ventricular ejection fraction(EF) were evaluated at rest and after exercise. The results were compared with the preoperative hemodynamic findings and with the age of patient at the time of the operation. 1) When the total work of exercise was divided with the maximal exercise capacity of the normal individual corresponding to the patients' height and body surface area (the percentage of total work), it were very low with the average of 40% of normal. There was no sexual difference, but the percentage of total work of exercise had significant correlation with the patients' age at the time of operation (r = -0.52,p<0.01) and post-exercise left ventricular ejection fraction (LVEF)(r = -0.39,p<0.05). 2) LVEF at rest had some correlation with the preoperative mean pulmonary arterial pressure (r = -0.29, p = 0.05), but showed no relationship with Qp/Qs or Rp/Rs ratios. The right ventricular ejection fraction (RVEF) at rest had no relations with the preoperative hemodynamic findings with maximal workload. 3) The post-exercise RVEF showed linear correlation with the preoperative Rp/Rs ratio (r = -0.49,p<0.005), and mean pulmonary arterial pressure (r = -0.37,p<0.05). The post-exercise LVEF had no significant correlation with any preoperative hemodynamic factors. 4) When greater than 5% increase in ventricular EF after exercise is considered normal, the group with the normal right and left ventricular responses (n = 11) showed normal preoperative Rp/Rs ratio (7.6 +/- 4.1). In the group with normal left, but abnormal right ventricular response (n = 9) and the group with abnormal biventricular response (n = 11), both demonstrated incremental increase in Rp/Rs ratio (20.1 +/- 11.3, 26.3 +/- 19.8 respectively). Normal right, but abnormal left ventricular reaction (n = 2) was noted in patients with residual aortic valvular insufficiency and residual ventricular septal defect. In conclusion, post-operative ventricular response was much more sensitive and informative than that of ventricular function at rest and to detect subclinical cardiac dysfunction. Post-exercise RVEF was closely correlated with preoperative pulmonary vascular hemodynamics, while post-exercise LVEF seemed to be a majo determinant of working capacity after repair.